2021/11/26 更新

写真a

コバヤシ カツヒロ
小林 勝弘
KOBAYASHI Katsuhiro
所属
医歯薬学域 教授
職名
教授
外部リンク

学位

  • 医学博士 ( 岡山大学 )

研究キーワード

  • 小児神経学

  • Child Neurology

研究分野

  • ライフサイエンス / 胎児医学、小児成育学

学歴

  • 岡山大学    

    - 1987年

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    国名: 日本国

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  • 岡山大学   Graduate School, Division of Medicine   Child neurology

    - 1987年

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  • 岡山大学   Medical School  

    - 1983年

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    国名: 日本国

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  • 岡山大学   Faculty of Medicine  

    - 1983年

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経歴

  • - 岡山大学医歯薬学総合研究科 教授

    2015年

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  • - Professor,Graduate School of Medicine, Dentistry and Pharmaceutical Sciences,Okayama University

    2015年

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  • Senior Assistant Professor,University Hospital of Medicine and Dentistry,Okayama University

    1997年 - 2015年

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  • 岡山大学   Okayama University Hospital

    1997年 - 2015年

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  • Researcher,McGill University, Montreal Neurological Institute (Canada)

    1997年 - 1998年

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  • マッギル大学、モントリオール神経研究所 (カナダ) 研究員

    1997年 - 1998年

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所属学協会

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委員歴

  • 日本臨床神経生理学会   第52回日本臨床神経生理学会学術大会プログラム委員  

    2021年9月 - 現在   

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  • 日本てんかん学会   第55回日本てんかん学会学術集会プログラム委員  

    2021年9月 - 現在   

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  • 日本臨床神経生理学会   第51回日本臨床神経生理学会学術大会プログラム委員  

    2020年10月 - 2021年12月   

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  • 日本てんかん学会   第54回日本てんかん学会学術集会プログラム委員  

    2020年7月 - 2021年9月   

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  • てんかん治療研究振興財団   研究助成選考委員  

    2020年4月 - 現在   

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  • 日本小児神経学会   Brain & Development 編集長  

    2019年4月 - 現在   

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  • 乳幼児けいれん研究会   世話人副代表  

    2018年10月 - 現在   

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  • 国際抗てんかん連盟   脳波タスクフォース  

    2017年6月 - 現在   

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  • 1988- 日本てんかん学会 第52回日本てんかん学会学術集会プログラム委員(2017-2018)  

    2017年 - 2018年   

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  • 日本小児科学会岡山地方会   第90日本小児科学会岡山地方会 会長  

    2017年   

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    団体区分:学協会

    日本小児科学会岡山地方会

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  • 日本小児科学会   日本小児科学会学術委員会委員  

    2016年 - 2018年   

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    団体区分:学協会

    日本小児科学会

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  • 日本小児科学会   日本小児科学会症例要約評価委  

    2016年   

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    団体区分:学協会

    日本小児科学会

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  • 日本小児科学会   代議員(岡山県)  

    2015年 - 現在   

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    団体区分:学協会

    日本小児科学会

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  • 日本小児神経学会   理事  

    2015年 - 現在   

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    団体区分:学協会

    日本小児神経学会

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  • 日本てんかん学会   理事  

    2015年 - 現在   

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    団体区分:学協会

    日本てんかん学会

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  • 日本臨床神経生理学会   評議員  

    2005年 - 現在   

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    団体区分:学協会

    日本臨床神経生理学会

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  • 日本てんかん学会   評議員  

    2005年 - 現在   

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    団体区分:学協会

    日本てんかん学会

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  • 日本小児神経学会   評議員  

    2004年 - 現在   

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    団体区分:学協会

    日本小児神経学会

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  • - The Japan Epilepsy Society  

    1988年   

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  • - The Japan Epilepsy Society  

    1988年   

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  • - The Japan Epilepsy Society  

    1988年   

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  • - The Japan Epilepsy Society  

    1988年   

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  • - The Japan Epilepsy Society  

    1988年   

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  • - 日本てんかん学会 てんかん専門医委員会委員長(2015-)  

    1988年   

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  • - 日本てんかん学会 日本てんかん学会専門医試験委員会委員(2016-)  

    1988年   

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  • - 日本てんかん学会 日本てんかん学会選挙委員会委員(2014-)  

    1988年   

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  • - 日本てんかん学会 てんかん専門医委員会委員(2014-)  

    1988年   

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論文

  • てんかん専門医の現状とこれから 招待

    小林勝弘

    小児科   62 ( 12 )   1530 - 1534   2021年11月

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    担当区分:筆頭著者   記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)  

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  • Case Report: High-Gamma Oscillations on an Ictal Electroencephalogram in a Newborn Patient With Hypoxic–Ischemic Encephalopathy 査読

    Akihito Takeuchi, Takushi Inoue, Makoto Nakamura, Misao Kageyama, Tomoyuki Akiyama, Katsuhiro Kobayashi

    9   2021年10月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.3389/fped.2021.679771

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  • 次世代のてんかん専門医育成に向けた取り組み 招待 査読

    小林勝弘

    クリニシアン   68 ( 8-9 )   405 - 408   2021年8月

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    担当区分:筆頭著者, 責任著者   記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)  

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  • Monozygotic twins with DYT-TOR1A showing jerking movements and levodopa responsiveness 査読 国際誌

    Hanaoka Y, Akiyama T, Yoshinaga H, Kobayashi K

    Brain Dev   43 ( 7 )   783 - 788   2021年7月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.braindev.2021.03.005

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  • Exclusion of the possibility of “false ripples” from ripple band high-frequency oscillations recorded from scalp electroencephalogram in children with epilepsy 査読 国際誌

    Kobayashi K, Shibata T, Tsuchiya H, Akiyama T

    Front Hum Neurosci   15   696882   2021年6月

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    担当区分:筆頭著者, 責任著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.3389/fnhum.2021.696882

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  • A study on the relationship between non-epileptic fast (40 - 200 Hz) oscillations in scalp EEG and development in children. 査読 国際誌

    Makio Oka, Katsuhiro Kobayashi, Takashi Shibata, Hiroki Tsuchiya, Yoshiyuki Hanaoka, Mari Akiyama, Teruko Morooka, Masao Matsuhashi, Tomoyuki Akiyama

    Brain Dev   43 ( 9 )   904 - 911   2021年5月

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    担当区分:責任著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.braindev.2021.05.004

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  • Current medico-psycho-social conditions of patients with West syndrome in Japan. 査読 国際誌

    Yoshitomi S, Hamano SI, Hayashi M, Sakuma H, Hirose S, Ishii A, Honda R, Ikeda A, Imai K, Jin K, Kada A, Kakita A, Kato M, Kawai K, Kawakami T, Kobayashi K, Matsuishi T, Matsuo T, Nabatame S, Okamoto N, Ito S, Okumura A, Saito A, Shiraishi H, Shirozu H, Saito T, Sugano H, Takahashi Y, Yamamoto H, Fukuyama T, Kuki I, Inoue Y

    Epileptic Disord   23 ( 4 )   579 - 589   2021年4月

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    記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1684/epd.2021.1301

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  • 和文・英文機関紙に求められる使命と役割分担 招待

    山本俊至, 小林勝弘

    脳と発達   53 ( 3 )   203 - 206   2021年3月

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    担当区分:最終著者   記述言語:日本語  

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  • Effects of a stable concentration of propofol upon interictal high-frequency oscillations in drug-resistant epilepsy 査読 国際誌

    Inada T, Kobayashi K, Kikuchi T, Matsuhashi M, Matsumoto R, Takahashi Y, Nake T, Shibata S, Yamao Y, Daifu-Kobayashi M, Togawa J, Yoshida K, Kunieda K, Kobayashi K, Ikeda A, Miyamaoto S

    Epileptic Disord   23 ( 2 )   299 - 312   2021年2月

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    記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1684/epd.2021.1264

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  • 初のWeb学会開催―開催の要点と今後への提言 招待

    小林勝弘, 佐々木征行, 加賀佳美, 藤井克則

    脳と発達   53 ( 2 )   95 - 104   2021年2月

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    担当区分:筆頭著者   記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)  

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  • Pyridoxal in the cerebrospinal fluid may be a better indicator of vitamin B6-dependent epilepsy than pyridoxal 5'-phosphate. 査読 国際誌

    Tomoyuki Akiyama, Yuki Hyodo, Kosei Hasegawa, Taikan Oboshi, Katsumi Imai, Naoko Ishihara, Yuri Dowa, Takayoshi Koike, Toshiyuki Yamamoto, Jun Shibasaki, Hiroko Shimbo, Tetsuhiro Fukuyama, Kyoko Takano, Hiroshi Shiraku, Saoko Takeshita, Tohru Okanishi, Shimpei Baba, Masaya Kubota, Shin-Ichiro Hamano, Katsuhiro Kobayashi

    Pediatric Neurology   113   33 - 41   2020年12月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.pediatrneurol.2020.08.020

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  • Longitudinal correspondence of epilepsy and scalp EEG fast (40-200 Hz) oscillations in pediatric patients with tuberous sclerosis complex. 査読 国際誌

    Hiroki Tsuchiya, Fumika Endoh, Tomoyuki Akiyama, Masao Matsuhashi, Katsuhiro Kobayashi

    Brain Dev   42 ( 9 )   663 - 674   2020年10月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.braindev.2020.06.001

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  • Vitamin B6 in acute encephalopathy with biphasic seizures and late reduced diffusion. 査読 国際誌

    Tomoyuki Akiyama, Soichiro Toda, Nobusuke Kimura, Yukiko Mogami, Yoshiyuki Hanaoka, Chiho Tokorodani, Tomoshiro Ito, Hiroyuki Miyahara, Yuki Hyodo, Katsuhiro Kobayashi

    42 ( 5 )   402 - 407   2020年5月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.braindev.2020.02.002

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  • Metabolic profiling of the cerebrospinal fluid in pediatric epilepsy. 査読

    Tomoyuki Akiyama, Daisuke Saigusa, Yuki Hyodo, Keiko Umeda, Reina Saijo, Seizo Koshiba, Katsuhiro Kobayashi

    Acta Medica Okayama   74 ( 1 )   65 - 72   2020年2月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.18926/AMO/57955

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  • 片側のジストニアを主症状とする抗NMDA受容体脳炎の1例

    兵頭 勇紀, 花岡 義行, 金 聖泰, 秋山 倫之, 三谷 納, 佐久間 啓, 小林 勝弘

    日本小児科学会雑誌   124 ( 2 )   365 - 365   2020年2月

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    記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)   出版者・発行元:(公社)日本小児科学会  

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  • Laboratory changes during ACTH therapy associated with renal calcified lesions. 査読 国際誌

    Hiroyuki Miyahara, Tomoyuki Akiyama, Kosei Hasegawa, Mari Akiyama, Makio Oka, Katsuhiro Kobayashi, Hirokazu Tsukahara

    Pediatrics International   62 ( 5 )   587 - 592   2020年1月

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    記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1111/ped.14158

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  • 脳波を見ること・読むこと 招待

    小林勝弘

    てんかん研究   38 ( 1 )   1 - 2   2020年1月

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    担当区分:筆頭著者, 責任著者   記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)  

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  • ペランパネルが発作および行動異常に共に著効した Lennox-Gastaut症候群の1例 査読

    間浦奈央子, 遠藤文香, 秋山麻里, 花岡義行, 秋山倫之, 小林勝弘

    てんかん研究   38 ( 1 )   36 - 42   2020年1月

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    担当区分:最終著者   記述言語:日本語   掲載種別:研究論文(学術雑誌)  

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  • High-frequency oscillations in a spectrum of pediatric epilepsies characterized by sleep-activated spikes in scalp EEG 査読 国際誌

    Ohuchi Y, Akiyama T, Matsuhashi M, Kobayashi K

    Clin Neurophysiol   130 ( 10 )   1971 - 1980   2019年10月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.clinph.2019.08.001

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  • Novel AVPR2 variant in a male infant with nephrogenic diabetes insipidus who showed delayed head control 査読

    Kosei Hasegawa, Hiromi Ihoriya, Natsuko Futagawa, Yousuke Higuchi, Hiroki Tsuchiya, Takashi Shibata, Yumiko Hayashi, Katsuhiro Kobayashi, Hirokazu Tsukahara

    Clinical Pediatric Endocrinology   28 ( 4 )   155 - 158   2019年10月

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    記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1297/cpe.28.155

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  • Genomic backgrounds of Japanese patients with undiagnosed neurodevelopmental disorders 査読 国際誌

    Yamamoto T, Imaizumi T, Yamamoto-Shimojima K, Lu Y, Yanagishita T, Shimada S, Chong PF, Kira R, Ueda R, Ishiyama A, Takeshita E, Momosaki K, Ozasa S, Akiyama T, Kobayashi K, Oomatsu H, Kitahara H, Yamaguchi T, Imai K, Kurahashi H, Okumura A, Oguni H, Seto T, Okamoto N

    Brain Dev   41 ( 9 )   776 - 782   2019年9月

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    記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.braindev.2019.05.007

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  • Infantile spasmsにおける初回ACTH療法後の再発に関する検討

    金 聖泰, 小林 勝弘, 兵頭 勇紀, 藤代 定志, 水野 むつみ, 道上 理絵, 宮原 大輔, 花岡 義行, 柴田 敬, 秋山 麻里, 岡 牧郎, 遠藤 文香, 秋山 倫之

    てんかん研究   37 ( 2 )   574 - 574   2019年9月

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    記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)   出版者・発行元:(一社)日本てんかん学会  

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  • West症候群における髄液中ビタミンB6化合物の解析

    秋山 倫之, 兵頭 勇紀, 花岡 義行, 秋山 麻里, 小林 勝弘, 池本 智, 松浦 隆樹, 小一原 玲子, 浜野 晋一郎, 岡西 徹

    てんかん研究   37 ( 2 )   590 - 590   2019年9月

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    記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)   出版者・発行元:(一社)日本てんかん学会  

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  • Action of antiepileptic drugs on neurons. 査読

    Kobayashi K, Endoh F, Ohmori I, Akiyama T

    Brain & development   42 ( 1 )   2 - 5   2019年7月

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    担当区分:筆頭著者, 責任著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.braindev.2019.07.006

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  • 医療安全に関するWGからの提言 脳波等神経生理検査時の鎮静における医療安全に関する提言・指針の作成 査読

    是松 聖悟, 宮本 雄策, 村松 一洋, 山中 岳, 白石 秀明, 吉永 治美, 中川 栄二, 稲垣 真澄, 金村 英秋, 小林 勝弘

    脳と発達   51 ( Suppl. )   S172 - S172   2019年5月

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    記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)   出版者・発行元:(一社)日本小児神経学会  

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  • West症候群を発症した結節性硬化症症例のてんかんの経過と幼児期早期の発達について

    遠藤 文香, 土屋 弘樹, 兵頭 勇紀, 水野 むつみ, 金 聖泰, 花岡 義行, 柴田 敬, 秋山 麻里, 小林 勝弘

    脳と発達   51 ( Suppl. )   S279 - S279   2019年5月

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    担当区分:最終著者   記述言語:日本語   掲載種別:研究論文(その他学術会議資料等)   出版者・発行元:(一社)日本小児神経学会  

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  • 幼児期から成人期への長期経過で特異なてんかんの病像変容を認めたBPANの臨床・脳波学的報告 査読

    西本静香, 吉永治美, 遠藤文香, 才津浩智, 松本直通, 小林勝弘

    脳と発達   51 ( 5 )   323 - 327   2019年5月

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    担当区分:最終著者   記述言語:日本語   掲載種別:研究論文(学術雑誌)  

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  • A case of dihydropyrimidinase deficiency incidentally detected by urine metabolome analysis 査読

    Hiroki Tsuchiya, Tomoyuki Akiyama, Tomiko Kuhara, Yoko Nakajima, Morimasa Ohse, Hiroki Kurahashi, Takema Kato, Yasuhiro Maeda, Harumi Yoshinaga, Katsuhiro Kobayashi

    Brain Dev   41 ( 3 )   280 - 284   2019年3月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.braindev.2018.10.005

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  • A case of vitamin B6-responsive West syndrome caused by severe traumatic brain injury 査読 国際誌

    Inoue T, Akiyama T, Hanaoka Y, Oka M, Kobayashi K

    Epilepsy & Seizure   10 ( 1 )   114 - 119   2019年2月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.3805/eands.10.114

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  • Catch-up growth and behavioral development among preterm, small-for-gestational-age children: a nationwide Japanese population-based study 査読

    Takeuchi A, Yorifuji T, Hattori M, Tamai K, Nakamura K, Nakamura M, Kageyama M, Kubo T, Ogino T, Kobayashi K, Doi H

    Brain and Development   41 ( 5 )   397 - 405   2019年

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    記述言語:英語   掲載種別:研究論文(学術雑誌)  

    DOI: 10.1016/j.braindev.2018.12.004

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  • Pyridoxal 5′-phosphate and related metabolites in hypophosphatasia: Effects of enzyme replacement therapy 査読

    Tomoyuki Akiyama, Takuo Kubota, Keiichi Ozono, Toshimi Michigami, Daisuke Kobayashi, Shinji Takeyari, Yuichiro Sugiyama, Masahiro Noda, Daisuke Harada, Noriyuki Namba, Atsushi Suzuki, Maiko Utoyama, Sachiko Kitanaka, Mitsugu Uematsu, Yusuke Mitani, Kunihiro Matsunami, Shigeru Takishima, Erika Ogawa, Katsuhiro Kobayashi

    Molecular Genetics and Metabolism   125 ( 1-2 )   174   2018年9月

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    担当区分:最終著者   出版者・発行元:Elsevier {BV}  

    DOI: 10.1016/j.ymgme.2018.07.006

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  • Detection of fast (40-150 Hz) oscillations from the ictal scalp EEG data of myoclonic seizures in pediatric patients. 査読

    Kobayashi K, Ohuchi Y, Shibata T, Hanaoka Y, Akiyama M, Oka M, Endoh F, Akiyama T

    Brain & development   40 ( 5 )   397 - 405   2018年5月

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    担当区分:筆頭著者, 責任著者  

    DOI: 10.1016/j.braindev.2018.01.004

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  • Novel heterozygous mutation in TBX1 in an infant with hypocalcemic seizures. 査読

    Kosei Hasegawa, Hiroyuki Tanaka, Yousuke Higuchi, Yumiko Hayashi, Katsuhiro Kobayashi, Hirokazu Tsukahara

    Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology   27 ( 3 )   159 - 164   2018年

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    記述言語:英語   掲載種別:研究論文(学術雑誌)  

    Patients with 22q11.2 deletion syndrome have characteristic facial appearance, palate abnormalities, hypoparathyroidism, thymic hypoplasia, and congenital heart disease. The 22q11.2 region includes TBX1 and 30 other genes. Analysis of Tbx1 transgenic mice showed that TBX1 was associated with the 22q11.2 deletion syndrome. In humans, TBX1 mutations have been reported in 22q11.2 deletion-negative patients with velocardiofacial syndrome or DiGeorge syndrome. Genotype-phenotype correlations are not fully understood in these patients. We report the case of an infant with a novel heterozygous TBX1 mutation who experienced hypocalcemic seizures. This patient had no palate abnormalities, cardiac anomalies, or the typical facial appearance observed in 22q11.2 deletion syndrome. The presence of thymic hypoplasia prompted us to perform G-banding, fluorescent in situ hybridization, and subsequent TBX1 analysis. We emphasize the importance of diagnosing thymic hypoplasia in hypocalcemic infants without 22q11.2 deletion for detecting TBX1 mutations.

    DOI: 10.1297/cpe.27.159

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  • 結節性硬化症におけるてんかんの長期予後についての検討

    遠藤 文香, 吉永 治美, 土屋 弘樹, 西本 静香, 兵頭 勇紀, 小林 勝弘

    脳と発達   49 ( Suppl. )   S338 - S338   2017年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • Simultaneous measurement of monoamine metabolites and 5-methyltetrahydrofolate in the cerebrospinal fluid of children. 査読

    Akiyama Tomoyuki, Hayashi Yumiko, Hanaoka Yoshiyuki, Shibata Takashi, Akiyama Mari, Nakamura Kazuyuki, Tsuyusaki Yu, Kubota Masaya, Yoshinaga Harumi, Kobayashi Katsuhiro

    Clin Chim Acta   465   5 - 10   2017年2月

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    担当区分:最終著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    BACKGROUND: We describe a new method for simultaneous measurement of monoamine metabolites (3-O-methyldopa [3-OMD], 3-methoxy-4-hydroxyphenylethyleneglycol [MHPG], 5-hydroxyindoleacetic acid [5-HIAA], and homovanillic acid [HVA]) and 5-methyltetrahydrofolate (5-MTHF) and its use on cerebrospinal fluid (CSF) samples from pediatric patients. METHODS: Monoamine metabolites and 5-MTHF were measured by high-performance liquid chromatography with fluorescence detection. CSF samples were prospectively collected from children according to a standardized collection protocol in which the first 1-ml fraction was used for analysis. RESULTS: Monoamine metabolites and 5-MTHF were separated within 10min. They showed linearity from the limit of detection to 1024nmol/l. The limit of quantification of each metabolite was sufficiently low for the CSF sample assay. In 42 CSF samples after excluding cases with possibly altered neurotransmitter profiles, the concentrations of 3-OMD, MHPG, 5-HIAA, HVA, and 5-MTHF showed significant age dependence and their ranges were comparable with the reference values in the literature. The metabolite profiles of aromatic l-amino acid decarboxylase deficiency, Segaw

    DOI: 10.1016/j.cca.2016.12.005

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  • West syndrome associated with epileptic negative myoclonus 査読

    Takashi Shibata, Harumi Yoshinaga, Makio Oka, Katsuhiro Kobayashi

    No To Hattatsu   46 ( 5 )   354 - 358   2014年9月

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    記述言語:日本語   掲載種別:研究論文(学術雑誌)   出版者・発行元:Japanese Society of Child Neurology  

    We report a 10-month-oIcI girl who had brief epileptic negative myoclonus during the course of West syndrome. She began to have epileptic spasms in series at the age of 8 months. Video-elcctroencephalograph (EEG) monitoring revealed that she also had brief epileptic negative myoclonus when she was 10 months old. Brief atonia of limbs occurred in isolation or in a cluster during drowsiness or sleep. The ictal EEG exhibited diffuse polyspikes and waves or diffuse high-voltage slow waves that were overlapped by low-voltage fast waves. 3 to 4 hundred milliseconds of silent periods were observed in the bilateral deltoid electromyograms, which correspond to the EEG patterns.
    The occurrence of other types of seizures, partial seizures in particular, accompanied by epileptic spasms has been fully investigated. This is the first case report of a patient with West syndrome whose coexisting epileptic negative myoclonus was confirmed by a silent electromyogram pattern.

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  • Clinical analysis of catastrophic epilepsy in infancy and early childhood: results of the Far-East Asia Catastrophic Epilepsy (FACE) study group. 査読

    Oguni H, Otsuki T, Kobayashi K, Inoue Y, Watanabe E, Sugai K, Takahashi A, Hirose S, Kameyama S, Yamamoto H, Hamano S, Baba K, Baba H, Hong SC, Kim HD, Kang HC, Luan G, Wong TT

    Brain & development   35 ( 8 )   786 - 792   2013年9月

  • Peculiar involuntary movements in premature babies with specific cerebellar injuries 査読

    Harumi Yoshinaga, Katsuhiro Kobayashi, Fumika Endoh, Yumiko Ishizaki, Takashi Shibata, Yoko Ohtsuka

    No To Hattatsu   44 ( 3 )   239 - 243   2012年5月

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    記述言語:日本語   掲載種別:研究論文(学術雑誌)  

    We observed characteristic involuntary movements in premature babies during early infancy. These movements consisted of asymmetrical irregular banging of the extremities, similar to chorea, ballisms, or jitteriness. We investigated the clinical characteristics and neuroimaging findings of the patients with these peculiar involuntary movements to clarify their pathophysiological mechanisms and to find a treatment. In our sequential follow-up study on 90 premature infants with various pre-and perinatal brain insults, we found various types of cerebellar injuries in 28 patients. In 19 of these, the prominent injuries were observed in the inferior cerebellar hemispheres. These cerebellar injuries were often observed in patients born before the gestational age of 27 weeks. Fourteen of the 28 patients with cerebellar injuries displayed the above-mentioned characteristic involuntary movements. Twelve of these 14 patients with both cerebellar injury and involuntary movements were born before the gestational age of 27 weeks. On the contrary, 10 patients with cerebellar injury bom after the gestational age of 27 weeks did not display these peculiar involuntary movements. It is noteworthy that cerebral injuries were not associated with the occurrence of these involuntary movements. Two patients with asymmetrical cerebellar deformity caused by compression due to a cystic lesion did not show these involuntary movements. The movements appeared around the corrected age of 3 months, and they disturbed the patients' acquisition of sitting ability. Nine patients with these involuntary movements developed severe athetotic cerebral palsy. These movements showed drug resistance, however, benzodiazepines had a partial effect in some patients. Recently, cerebellar injury in premature infants has received a lot of attention. We believe that the peculiar involuntary movements we observed in the present patient group may be caused by a particular type of cerebellar damage specific to premature infants born before 27 weeks of gestational age.

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  • Lamotrigine add-on therapy for childhood-onset refractory epilepsy: Comparison of efficacy between 3 months and 6 months after initiation 査読

    Kiyoko Watanabe, Katsuhiro Kobayashi, Fumika Endoh, Harumi Yoshinaga, Yoko Ohtsuka

    No To Hattatsu   43 ( 6 )   453 - 458   2011年11月

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    記述言語:日本語   掲載種別:研究論文(学術雑誌)  

    We investigated the effect of lamotrigine (LTG) add-on therapy in 50 patients with childhood-onset refractory epilepsy (25 males and 25 females): 15 with localization-related epilepsy, 33 with generalized epilepsy, and 2 with undetermined epilepsy. Twenty-four patients had experienced a period of West syndrome during their clinical course. Age at the start of LTG therapy ranged from 2 years 6 months to 41 years 2 months: &lt
    16 years in 43 and ≥ 16 years in 7. Seizure frequency was ≥1 per day in 36 patients (72%) and ≥1 per week in 14 (28%). We increased the LTG dosage every two weeks in accordance with usage recommendations. We evaluated efficacy at two points : 3 and 6 months after the start of LTG. At the 6-month point, seizure freedom was achieved in 2 patients (4%), ≥50% seizure reduction in 14 (28%), 25 to 50% seizure reduction in 20 (40%), no effect in 6 (12%), and aggravation in 4 (8%). Only 4 patients (8%) stopped LTG therapy within 6 months due to LTG-related mild skin rash in 2 and suspicion of seizure aggravation in the other 2. In terms of seizure types, seizure freedom or ≥50% seizure reduction was achieved in 29% for epileptic spasms, 32% for tonic seizures, and 29% for partial seizures. A comparison between the 3- and 6-month points revealed that the efficacy level was increased or maintained in 77% of the patients and decreased in 23%. In most cases, the highest level of efficacy appeared within 3 months with doses that were smaller than maintenance doses. Observed CNS-related adverse effects included somnolence in 16 patients, irritability in 14, and sleep disturbance in 11. Positive psychotropic effects in daily activities were seen in 28 patients (56%). These effects appeared regardless of the change in seizure frequency with doses that were smaller than maintenance doses.

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  • Symptomatic generalized epilepsies: Clinical significance and problems 査読

    Yoko Ohtsuka, Katsuhiro Kobayashi, Harumi Yoshinaga, Makio Oka, Fumika Endoh

    NEUROLOGY ASIA   15   1 - 2   2010年

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    記述言語:英語   掲載種別:研究論文(学術雑誌)   出版者・発行元:ASEAN NEUROLOGICAL ASSOC  

    We investigated electroclinical features in patients with symptomatic or cryptogenic generalized epilepsies except for West syndrome. They were divided into a tonic group and a myoclonic group. As a result these groups exhibited different features with some overlap between them. They included Lennox-Gastaut syndrome, severe epilepsy with multiple independent spike foci (SE-MISF), Doose syndrome and other unclassified cases. Thus the boundary between these epilepsy syndromes is sometimes blurred. In patients with SE-MISF, main seizure types were minor generalized seizures though the main EEG findings were multifocal spikes. The role of both cortical and subcortical mechanisms in the occurrence of symptomatic generalized epilepsies should be further clarified.

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  • Source estimation of spikes by a combination of independent component analysis and RAP-MUSIC 査読

    Katsuhiro Kobayashi, Tomoyuki Akiyama, Tomoyuki Nakahori, Harumi Yoshinaga, Yoko Ohtsuka, Jean Gotman, Eiji Oka

    International Congress Series   1232 ( C )   311 - 316   2002年4月

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    担当区分:筆頭著者, 責任著者   記述言語:英語   掲載種別:研究論文(学術雑誌)  

    We designed a combination of independent component analysis (ICA) and recursively applied and projected multiple signal classification (RAP-MUSIC) as a new approach to dipole source estimation of epileptiform discharges. The estimation is minimally influenced by subjective decisions in this analysis. A simulation study was performed by generating 10 EEG data matrices by means of a computer: each matrix included real background activity from a normal subject to which an array of simulated unaveraged spikes was added. Each discharge was a summation of two transients originating from slightly different “original dipole sources”. The simulated spikes in the unaveraged EEG data were extracted from the background by ICA as spatiotemporal components, each component having fixed potential field distribution and maximally independent waveform. The complete separation between spikes and background was objectively proven by reconstruction of the EEG from the decomposed components. RAP-MUSIC was performed based on the spatial information included in the epileptic ICA components. RAP-MUSIC does not involve subjective decisions such as selection of multiple local score peaks, determination of the number of dipoles or initial guesses of dipole parameters. In every simulated EEG data matrix, two dipoles close to the original sources were estimated. Their activities were also similar to those of the original sources. For comparison, the same simulated EEG data were averaged and subjected to RAP-MUSIC using eigen-decomposition of the covariance matrices. Only one dipole was estimated in every simulation by this conventional method and therefore the result of the present method was better. RAP-MUSIC based on ICA, thus, proved promising for source estimation of unaveraged epileptiform discharges. © 2002, Elsevier B.V.

    DOI: 10.1016/S0531-5131(01)00713-0

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  • Childhood-onset epilepsy associated with polymicrogyria 査読

    Yoko Ohtsuka, Akio Tanaka, Katsuhiro Kobayashi, Hodaka Ohta, Kiyoko Abiru, Kohsuke Nakano, Eiji Oka

    Brain and Development   24   758 - 765   2002年

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    記述言語:英語  

    DOI: 10.1016/S0387-7604(02)00099-2

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  • てんかん診療の最近の進歩 てんかんと睡眠 徐波睡眠中連続的きょく徐波を示す非けいれん性てんかん重積状態を中心に:徐波睡眠中連続的棘徐波を示す非痙攣性てんかん重積状態を中心に

    小林 勝弘, 大塚 頒子, 大田原 俊輔

    脳と発達   22 ( 2 )   136 - 142   1990年

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    記述言語:日本語   出版者・発行元:The Japanese Society of Child Neurology  

    てんかんと睡眠の関連につきこれまでの知見を概観し, 特に徐波睡眠中広汎性棘徐波の連続する非痙攣性てんかん重積状態すなわちepilepsy with electrical status epilepticus duringslow sleep, peculiar type of non convulsive status epilepticus in childhood, atypical benignpartialepilepsyにつき臨床的脳波学的に検討した. 前2者において高率に知的退行がみられ, これは上記脳波像の最終抑制年齢および徐波睡眠でのspike-wave indexと相関していた. 3病型とも思春期前は難治であるが, 16歳までに寛解し年齢依存性の病態が示唆された. またcoherenceand phase analysisによりsecondary bilateral synchronyが多数を占めることが示された.

    DOI: 10.11251/ojjscn1969.22.136

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書籍等出版物

  • てんかん専門医ガイドブック : てんかんにかかわる医師のための基本知識 改訂第2版 査読

    小林勝弘( 担当: 共編者(共編著者) ,  範囲: 大田原症候群、小児の脳波とその異常、発達性てんかん性脳症)

    診断と治療社  2020年12月  ( ISBN:9784787824172

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    総ページ数:xxxi, 433p   担当ページ:33-36,95-99,234-235   記述言語:日本語 著書種別:教科書・概説・概論

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  • 今日の小児治療指針

    水口, 雅, 市橋, 光( 担当: 分担執筆 ,  範囲: てんかん症候群)

    医学書院  2020年12月  ( ISBN:9784260039468

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    総ページ数:22, 980p   担当ページ:657-659   記述言語:日本語 著書種別:教科書・概説・概論

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  • 今日の診断指針

    永井, 良三, 浅利, 靖( 担当: 分担執筆 ,  範囲: 小児の痙攣)

    医学書院  2020年3月  ( ISBN:9784260038089

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    総ページ数:xxvii, 2079p   担当ページ:22-25   記述言語:日本語 著書種別:教科書・概説・概論

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  • モノグラフ「臨床脳波を基礎から学ぶ人のために」第2版 査読

    日本臨床神経生理学会( 担当: 分担執筆 ,  範囲: ワイドバンド脳波:高周波成分の発生機構、記録および検出)

    診断と治療社  2019年12月  ( ISBN:9784787824462

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    総ページ数:x, 110p   担当ページ:231-238   記述言語:日本語 著書種別:教科書・概説・概論

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  • 指定難病ペディア

    水澤, 英洋, 五十嵐, 隆, 北川, 泰久, 高橋, 和久(内科学), 弓倉, 整( 担当: 分担執筆 ,  範囲: 64 大田原症候群 [指定難病146])

    日本医師会,診断と治療社 (発売)  2019年7月  ( ISBN:9784787824165

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    総ページ数:365p   担当ページ:S126   記述言語:日本語 著書種別:教科書・概説・概論

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  • Human pathobiochemistry : from clinical studies to molecular mechanisms 査読 国際共著

    Kobayashi K, Akiyama T, Go C( 担当: 共著 ,  範囲: Epilepsy)

    Springer  2019年  ( ISBN:9789811329760

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    総ページ数:xi, 349 p.   担当ページ:295-310   記述言語:英語 著書種別:教科書・概説・概論

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  • 抗てんかん薬TDM標準化ガイドライン

    日本TDM学会

    金原出版  2018年11月  ( ISBN:9784307470483

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    総ページ数:xiii, 111p   記述言語:日本語 著書種別:教科書・概説・概論

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  • 稀少てんかんの診療指標

    診断と治療社  2017年  ( ISBN:9784787823090

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  • 稀少てんかんの診療指標

    診断と治療社  2017年  ( ISBN:9784787823090

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  • 小児神経専門医テキスト

    診断と治療社  2017年  ( ISBN:4787822772

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  • Annual Review神経2017

    中外医学社  2017年  ( ISBN:4498228766

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  • てんかん白書―てんかん医療・研究のアクションプラン

    南江堂  2016年 

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  • ポケット版神経内科検査・処置マニュアル

    新興医学出版社  2016年 

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  • 臨床てんかん学

    医学書院  2015年 

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  • 臨床てんかん学

    医学書院  2015年 

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  • 臨床てんかん学

    医学書院  2015年 

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  • てんかん専門医ガイドブック―てんかんにかかわる医師のための基礎知識―

    診断と治療社  2014年  ( ISBN:9784787820341

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  • 希少難治てんかん診療マニュアル 疾患の特徴と診断のポイント

    診断と治療社  2013年  ( ISBN:9784787819864

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  • フローチャートでわかる小児てんかん診療ガイド

    診断と治療社  2011年 

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  • Pediatric Electroencephalography

    2008年 

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  • 小児脳波―判読のためのアプローチ

    診断と治療社  2008年 

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  • Recent Advances in Human Brain Mapping

    Elsevier,Amsterdam  2002年 

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  • 領域別症候群シリーズ No. 37 神経症候群 VI てんかん症候群

    日本臨牀社,大阪  2002年 

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  • Recent Advances in Human Brain Mapping

    Elsevier,Amsterdam  2002年 

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  • 厚生省精神・神経疾患研究委託費「新技術を用いたてんかん等の診断法と治療法の開発」平成12年度報告書

    2001年 

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▼全件表示

MISC

  • 里吉病の1例

    兵頭 勇紀, 秋山 麻里, 秋山 倫之, 平井 陽至, 小林 勝弘

    日本小児科学会雑誌   123 ( 7 )   1198 - 1199   2019年7月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • ACTH療法中に尿路結石を生じた乳幼児例における尿沈渣所見の経時的変化

    宮原 宏幸, 水野 むつみ, 兵頭 勇紀, 西本 静香, 花岡 義行, 松田 奈央子, 金 聖泰, 柴田 敬, 遠藤 文香, 秋山 麻里, 岡 牧郎, 秋山 倫之, 小林 勝弘, 塚原 宏一

    日本小児科学会雑誌   123 ( 7 )   1199 - 1199   2019年7月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • West症候群を発症した結節性硬化症症例のてんかんの経過と幼児期早期の発達について

    遠藤 文香, 土屋 弘樹, 兵頭 勇紀, 水野 むつみ, 金 聖泰, 花岡 義行, 柴田 敬, 秋山 麻里, 小林 勝弘

    脳と発達   51 ( Suppl. )   S279 - S279   2019年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 非典型的な臨床像を呈したDYT-TOR1Aの一卵性双胎例

    花岡 義行, 秋山 倫之, 秋山 麻里, 岡 牧郎, 遠藤 文香, 柴田 敬, 小林 勝弘

    日本小児科学会雑誌   123 ( 2 )   328 - 328   2019年2月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • 脳葉酸欠乏症を合併し、folinic acidで治療しているミトコンドリア病の1例

    兵頭 勇紀, 秋山 麻里, 秋山 倫之, 小林 勝弘

    脳と発達   50 ( 6 )   444 - 444   2018年11月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 過去7年間におけるてんかん外科症例の治療成績

    松田 奈央子, 秋山 麻里, 佐々木 達也, 伊達 勲, 小林 勝弘

    脳と発達   50 ( 6 )   443 - 443   2018年11月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 運動により両側下肢の律動性異常運動が誘発される2症例

    水野 むつみ, 秋山 麻里, 遠藤 文香, 村上 暢子, 三谷 納, 小林 勝弘

    臨床神経生理学   46 ( 5 )   431 - 431   2018年10月

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    記述言語:日本語   出版者・発行元:(一社)日本臨床神経生理学会  

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  • 小児てんかんのミオクロニー発作の頭皮発作時脳波における速波(40-150 Hz)振動の検出

    小林 勝弘, 大内 勇児, 柴田 敬, 花岡 義行, 秋山 麻里, 岡 牧郎, 遠藤 文香, 秋山 倫之

    臨床神経生理学   46 ( 5 )   494 - 494   2018年10月

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    記述言語:日本語   出版者・発行元:(一社)日本臨床神経生理学会  

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  • ペランパネルが発作と異常行動に共に著効したLennox-Gastaut症候群の1例

    松田 奈央子, 遠藤 文香, 秋山 倫之, 小林 勝弘

    臨床神経生理学   46 ( 5 )   431 - 431   2018年10月

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    記述言語:日本語   出版者・発行元:(一社)日本臨床神経生理学会  

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  • West症候群および類縁病型の新たな治療戦略における活性型ビタミンB6の有効性の検討

    花岡 義行, 秋山 倫之, 岡 牧郎, 遠藤 文香, 秋山 麻里, 金 聖泰, 西本 静香, 兵頭 勇紀, 小林 勝弘

    てんかん研究   36 ( 2 )   460 - 460   2018年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 左心低形成症候群の児に好発したPanayiotopoulos症候群様のてんかん

    柴田 敬, 秋山 麻里, 福嶋 遥佑, 松田 奈央子, 水野 むつみ, 諸岡 輝子, 遠藤 文香, 大月 審一, 笠原 真悟, 小林 勝弘

    てんかん研究   36 ( 2 )   452 - 452   2018年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 本邦におけるピリドキシン依存性てんかん診断システムの開発

    秋山 倫之, 大星 大観, 今井 克美, 道和 百合, 椎原 隆, 福山 哲広, 兵頭 勇紀, 土屋 弘樹, 久保田 雅也, 浜野 晋一郎, 岡西 徹, 小林 勝弘

    てんかん研究   36 ( 2 )   479 - 479   2018年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 髄液中ピリドキサールリン酸とてんかんとの関連性についての検討

    金 聖泰, 秋山 倫之, 兵頭 勇紀, 松田 奈央子, 水野 むつみ, 西本 静香, 小林 勝弘

    てんかん研究   36 ( 2 )   478 - 478   2018年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 小児けいれん性疾患における活性型ビタミンB6の有用性

    花岡 義行, 吉永 治美, 秋山 倫之, 柴田 敬, 小林 勝弘

    てんかん研究   36 ( 1 )   67 - 67   2018年6月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 難治性てんかんで発症したinsular pilocytic astrocytomaの一例

    佐々木 達也, 細本 翔, 岡崎 三保子, 水野 むつみ, 柴田 敬, 秋山 麻里, 亀田 雅博, 安原 隆雄, 小林 勝弘, 伊達 勲

    てんかん研究   36 ( 1 )   88 - 89   2018年6月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 視床下部過誤腫に伴う笑い発作の検討

    秋山 麻里, 岡 牧郎, 土屋 弘樹, 西本 静香, 兵頭 勇紀, 遠藤 文香, 吉永 治美, 小林 勝弘

    てんかん研究   36 ( 1 )   72 - 72   2018年6月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • Landau-Kleffner症候群の1例

    秋山 麻里, 諸岡 輝子, 藤田 隼平, 岡 牧郎, 小林 勝弘

    てんかん研究   36 ( 1 )   67 - 67   2018年6月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • GABRA1遺伝子異常を認めた難治てんかんの一例

    柴田 敬, 竹内 章人, 秋山 麻里, 岡 牧郎, 遠藤 文香, 秋山 倫之, 山本 俊至, 小林 勝弘

    てんかん研究   36 ( 1 )   84 - 85   2018年6月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 病巣切除により発作抑制が得られた乳児結節性硬化症の2例

    佐々木 達也, 上利 崇, 西本 静香, 秋山 麻里, 遠藤 文香, 岡 牧郎, 秋山 倫之, 小林 勝弘, 伊達 勲

    てんかん研究   36 ( 1 )   76 - 76   2018年6月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • てんかんの原因となる先天性代謝疾患の診断に対する当院の取り組み

    秋山 倫之, 秋山 麻里, 林 裕美子, 柴田 敬, 花岡 義行, 吉永 治美, 小林 勝弘

    てんかん研究   36 ( 1 )   74 - 74   2018年6月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 運動により両側下肢の律動性異常運動が誘発される2症例

    水野 むつみ, 秋山 麻里, 遠藤 文香, 村上 暢子, 三谷 納, 小林 勝弘

    脳と発達   50 ( Suppl. )   S424 - S424   2018年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 小児期脳炎・脳症患者における急性期脳波の重症度と予後の関連

    秋山 麻里, 岡 牧郎, 遠藤 文香, 秋山 倫之, 小林 勝弘

    脳と発達   50 ( Suppl. )   S325 - S325   2018年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • てんかんを合併した結節性硬化症児の幼児期の発達について

    遠藤 文香, 土屋 弘樹, 兵頭 勇紀, 秋山 麻里, 岡 牧郎, 秋山 倫之, 吉永 治美, 小林 勝弘

    脳と発達   50 ( Suppl. )   S305 - S305   2018年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • West症候群に対するビガバトリンの使用経験

    柴田 敬, 秋山 麻里, 松田 奈央子, 兵頭 勇紀, 土屋 弘樹, 花岡 義行, 遠藤 文香, 秋山 倫之, 小林 勝弘

    脳と発達   50 ( Suppl. )   S295 - S295   2018年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 小児期の睡眠時頭皮脳波の棘波に伴う高周波振動の追跡研究

    大内 勇児, 秋山 倫之, 兵頭 勇紀, 土屋 弘樹, 花岡 義行, 柴田 敬, 秋山 麻里, 遠藤 文香, 岡 牧郎, 小林 勝弘

    脳と発達   50 ( Suppl. )   S333 - S333   2018年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 脊髄性筋萎縮症I型に対しヌシネルセンを使用した1例

    水野 むつみ, 秋山 麻里, 高橋 伸方, 藤原 倫昌, 小林 勝弘

    日本小児科学会雑誌   122 ( 4 )   840 - 841   2018年4月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • 小児期脳炎・脳症患者における急性期脳波像と予後の関連

    秋山 麻里, 岡 牧郎, 小林 勝弘

    日本小児科学会雑誌   122 ( 4 )   841 - 841   2018年4月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • 難聴と糸球体腎炎を伴うCharcot-Marie-Tooth病の男児例

    花岡 義行, 宮井 貴之, 秋山 麻里, 林 裕美子, 岡 牧郎, 塚原 宏一, 小林 勝弘

    日本小児科学会雑誌   122 ( 2 )   264 - 264   2018年2月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • Catch-Up Growth and Neurobehavioral Development among Full-Term, Small-for-Gestational-Age Children: A Nationwide Japanese Population-Based Study. 国際誌

    Akihito Takeuchi, Takashi Yorifuji, Kazue Nakamura, Kei Tamai, Shigehiro Mori, Makoto Nakamura, Misao Kageyama, Toshihide Kubo, Tatsuya Ogino, Katsuhiro Kobayashi, Hiroyuki Doi

    The Journal of pediatrics   192   41 - 46   2018年1月

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    記述言語:英語  

    OBJECTIVE: To examine the relationship between catch-up growth of full-term, small for gestational age (SGA) children and their neurobehavioral development. STUDY DESIGN: Data were obtained from a population-based nationwide Japanese longitudinal survey that started in 2001. Study participants were full-term children with information on height at 2 years of age (n = 32 533). Catch-up growth for SGA infants was defined as achieving a height at 2 years of age of more than -2.0 standard deviations for chronological age. Logistic regression analyses were used to estimate ORs and 95% CIs for the associations of SGA and catch-up growth status with neurobehavioral development at 2.5 and 8 years of age, adjusting for potential infant- and parent-related confounding factors. RESULTS: Fifteen percent of term SGA infants failed to catch up in height. At 2.5 years of age, SGA children without catch-up growth were more likely to be unable to climb stairs (OR, 10.42; 95% CI, 5.55-19.56) and unable to compose a 2-word sentence (OR, 3.58; 95% CI, 1.81-7.08) compared with children with normal growth at birth. Furthermore, SGA children without catch-up growth were at increased risk for aggressive behaviors (OR, 3.85; 95% CI, 1.19-12.47) at 8 years of age. CONCLUSIONS: Continuous follow-up for full-term SGA infants with failure of catch-up growth or poor postnatal growth may be beneficial for early detection and intervention for behavioral problems.

    DOI: 10.1016/j.jpeds.2017.09.002

    PubMed

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  • トピラマート(TPM)が影響したと思われる幻覚妄想状態を呈した腫瘍切除後の側頭葉てんかんの1例

    松本洋輔, 石川亮, 稲垣正俊, 川田清宏, 小林勝弘, 上利崇, 山田了士, 山田了士

    てんかん研究   36 ( 1 )   65‐66   2018年

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    記述言語:日本語  

    J-GLOBAL

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  • 【もう慌てないけいれん・てんかん 発作時の対応・治療・日常生活支援までバッチリわかる!】日常生活支援 小児てんかん患者

    秋山 麻里, 小林 勝弘

    Brain Nursing   33 ( 12 )   1201 - 1203   2017年12月

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    記述言語:日本語   出版者・発行元:(株)メディカ出版  

    <check point>てんかん発作に遭遇した場合、看護師は患者の安全面に配慮し、まずは見守ります。てんかん発作は自然に止まることが多いですが、処置を要する状況は知っておきましょう。日常生活に過度な制限を強いることのないように、患者家族・医療者・学校間の情報共有が重要です。(著者抄録)

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  • GABRG2 de novo変異を有し難治てんかんと四肢麻痺を示す1女子例

    兵頭 勇紀, 秋山 麻里, 小林 勝弘, 山本 俊至

    脳と発達   49 ( 6 )   429 - 430   2017年11月

  • Another Case of Glucose Transporter 1 Deficiency Syndrome with Periventricular Calcification, Cataracts, Hemolysis, and Pseudohyperkalemia

    Takashi Shibata, Katsuhiro Kobayashi, Harumi Yoshinaga, Hiroaki Ono, Michiko Shinpo, Kuriko Kagitani-Shimono

    NEUROPEDIATRICS   48 ( 5 )   390 - 393   2017年10月

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    記述言語:英語   出版者・発行元:GEORG THIEME VERLAG KG  

    Glucose transporter 1 (GLUT1) deficiency syndrome (GLUT1DS) is a disorder resulting from shortage of energy in the brain caused by reduced GLUT1 activity. Its common clinical symptoms include seizures, microcephaly, intellectual disability, abnormal ocular movements, ataxia, and dystonia. We report a case of GLUT1DS with unusual symptoms, including periventricular calcification. The patient is a Japanese girl, whose seizures had always evolved into status epilepticus since she was 4 months old. She also had cataracts and horizontal nystagmus. Neuroimaging studies showed periventricular calcification and brain atrophy. Laboratory data revealed pseudohyperkalemia, reticulocyte increase, and hypoglycorrhachia. A mutation of c1306_1308delATC (p.Ile436-del) was identified in the SLC2A1 gene, and she was thus diagnosed with GLUT1DS. A case with the identical SLC2A1 gene mutation and similar clinical findings was previously reported by Bawazir et al (2012). The leak of monovalent cations through the red cell membrane causes hemolysis in such patients, and a similar phenomenon may occur at the blood-brain barrier and the lens epithelium. After commencing ketogenic diet therapy, the electroencephalogram (EEG) abnormalities improved markedly and the patient's development advanced. Clinicians should be aware of atypical GLUT1DS.

    DOI: 10.1055/s-0037-1603520

    Web of Science

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  • Pyridoxal 5'-phosphate, pyridoxal, and 4-pyridoxic acid in the paired serum and cerebrospinal fluid of children

    Tomoyuki Akiyama, Yumiko Hayashi, Yoshiyuki Hanaoka, Takashi Shibata, Mari Akiyama, Hiroki Tsuchiya, Tokito Yamaguchi, Katsuhiro Kobayashi

    CLINICA CHIMICA ACTA   472   118 - 122   2017年9月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Background We quantified pyridoxal 5'-phosphate (PLP), pyridoxal (PL), and 4-pyridoxic acid (PA) in paired serum and cerebrospinal fluid (CSF) samples from children and investigated the effect of age on the concentrations and CSF-to-serum ratios of these vitamers.
    Methods: Serum and CSF samples prospectively collected from 49 pediatric patients were analyzed. PLP, PL, and PA were measured using high-performance liquid chromatography with fluorescence detection, using pre column derivatization by semicarbazide. Effects of age on these vitamers, the PLP-to-PL ratio, CSF-to-serum PLP ratio, and CSF-to-serum PL ratio were evaluated using correlation analysis.
    Results: The PLP, PL, and PA concentrations in the serum and CSF were higher at younger ages, except for CSF PA concentrations that were mostly below the limit of detection (< 1.2 nmo1/1). The PLP-to-PL ratios in the serum and CSF correlated positively with age. The CSF-to-serum PLP ratio and CSF-to-serum PL ratio were independent of age.
    Conclusions: Age-related changes in PLP, PL, and PA in serum and in CSF from pediatric patients and CSF-toserum ratios of PLP and PL demonstrated in this study will provide valuable information for evaluating PLP supply to the central nervous system from the peripheral blood.

    DOI: 10.1016/j.cca.2017.07.032

    Web of Science

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  • けいれん重積型(二相性)急性脳症(AESD)後にepileptic spasmを発症した症例の検討

    兵頭 勇紀, 秋山 麻里, 花岡 義行, 柴田 敬, 岡 牧郎, 秋山 倫之, 小林 勝弘

    てんかん研究   35 ( 2 )   454 - 454   2017年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 結節性硬化症の乳児期におけるてんかんの特徴

    土屋 弘樹, 遠藤 文香, 岡 牧郎, 花岡 義行, 柴田 敬, 林 裕美子, 秋山 麻里, 秋山 倫之, 吉永 治美, 小林 勝弘

    てんかん研究   35 ( 2 )   453 - 453   2017年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 尿中メタボローム解析により偶発的に発見されたジヒドロピリミジナーゼ欠損症の1例

    土屋 弘樹, 秋山 倫之, 久原 とみ子, 中島 葉子, 柴田 敬, 秋山 麻里, 岡 牧郎, 遠藤 文香, 吉永 治美, 小林 勝弘

    日本先天代謝異常学会雑誌   33   228 - 228   2017年9月

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    記述言語:日本語   出版者・発行元:日本先天代謝異常学会  

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  • 早期手術を行い発作抑制が得られた乳児結節性硬化症の2例

    佐々木 達也, 上利 崇, 西本 静香, 秋山 麻里, 遠藤 文香, 岡 牧郎, 秋山 倫之, 小林 勝弘, 伊達 勲

    てんかん研究   35 ( 2 )   588 - 588   2017年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 難治てんかんにおけるペランパネルの有効性について

    遠藤 文香, 水野 むつみ, 松田 奈央子, 大内 勇児, 秋山 倫之, 小林 勝弘

    てんかん研究   35 ( 2 )   485 - 485   2017年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • High-frequency oscillations: The state of clinical research

    Birgit Frauscher, Fabrice Bartolomei, Katsuhiro Kobayashi, Jan Cimbalnik, Maryse A. Van't Klooster, Stefan Rampp, Hiroshi Otsubo, Yvonne Hoeller, Joyce Y. Wu, Eishi Asano, Jerome Engel, Philippe Kahane, Julia Jacobs, Jean Gotman

    EPILEPSIA   58 ( 8 )   1316 - 1329   2017年8月

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    記述言語:英語   出版者・発行元:WILEY  

    Modern electroencephalographic (EEG) technology contributed to the appreciation that the EEG signal outside the classical Berger frequency band contains important information. In epilepsy, research of the past decade focused particularly on interictal high-frequency oscillations (HFOs) > 80 Hz. The first large application of HFOs was in the context of epilepsy surgery. This is now followed by other applications such as assessment of epilepsy severity and monitoring of antiepileptic therapy. This article reviews the evidence on the clinical use of HFOs in epilepsy with an emphasis on the latest developments. It highlights the growing literature on the association betweenHFOs and postsurgical seizure outcome. A recent meta-analysis confirmed a higher resection ratio for HFOs in seizure-free versus non-seizure-free patients. Residual HFOs in the postoperative electrocorticogram were shown to predict epilepsy surgery outcome better than preoperative HFOrates. The review further discusses the different attempts to separate physiological from epileptic HFOs, as this might increase the specificity of HFOs. As an example, analysis of sleep microstructure demonstrated a different coupling between HFOs inside and outside the epileptogenic zone. Moreover, there is increasing evidence that HFOs are useful to measure disease activity and assess treatment response using noninvasive EEG and magnetoencephalography. This approach is particularly promising in children, because they show high scalp HFO rates. HFO rates in West syndrome decrease after adrenocorticotropic hormone treatment. Presence of HFOs at the time of rolandic spikes correlates with seizure frequency. The time-consuming visual assessment of HFOs, which prevented their clinical application in the past, is now overcome by validated computer-assisted algorithms. HFO research has considerably advanced over the past decade, and use of noninvasive methods will make HFOs accessible to large numbers of patients. Prospective multicenter trials are awaited to gather information over long recording periods in large patient samples.

    DOI: 10.1111/epi.13829

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  • 乳児神経軸索ジストロフィーの1例

    柴田 敬, 岡 牧郎, 秋山 麻里, 吉永 治美, 下島 圭子, 山本 俊至, 小林 勝弘

    日本小児科学会雑誌   121 ( 8 )   1444 - 1444   2017年8月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • 結節性硬化症の乳児期における中枢神経系の画像変化

    土屋 弘樹, 遠藤 文香, 岡 牧郎, 秋山 倫之, 吉永 治美, 小林 勝弘

    日本小児科学会雑誌   121 ( 8 )   1443 - 1443   2017年8月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • 髄液中モノアミン類と5-メチルテトラヒドロ葉酸の同時測定法の開発(Simultaneous measurement of monoamines and 5-methyltetrahydrofolate in the cerebrospinal fluid)

    秋山 倫之, 林 裕美子, 花岡 義行, 柴田 敬, 秋山 麻里, 中村 和幸, 露崎 悠, 久保田 雅也, 遠山 潤, 吉永 治美, 小林 勝弘

    脳と発達   49 ( Suppl. )   S334 - S334   2017年5月

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    記述言語:英語   出版者・発行元:(一社)日本小児神経学会  

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  • 小児期の強い過眠に関する臨床的検討

    花岡 義行, 柴田 敬, 林 裕美子, 秋山 麻里, 岡 牧郎, 吉永 治美, 小林 勝弘

    脳と発達   49 ( Suppl. )   S343 - S343   2017年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • A ten-year follow-up cohort study of childhood epilepsy: Changes in epilepsy diagnosis with age

    Yoshiyuki Hanaoka, Harumi Yoshinaga, Katsuhiro Kobayashi

    BRAIN & DEVELOPMENT   39 ( 4 )   312 - 320   2017年4月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: To elucidate all of the characteristics of childhood epilepsy, we performed a long-term follow-up study on the patients who visited Okayama University Hospital.
    Subjects and methods: We retrospectively investigated the patients who were involved in the previous epidemiological study and visited Okayama University Hospital for a period of 10 years after December 31, 1999.
    Results: Overall, there were 350 patients' medical records that were evaluated, and 258 patients with complete clinical information available for a 10-year period were enrolled. Ten patients died and the remaining 82 were lost to follow-up. Of 258 patients with complete information, 153 (59.3%) were seizure-free for at least 5 years. One hundred thirty (50.4%) had intellectual disabilities and 77 (29.8%) had motor disabilities, including 75 (29.1%) with both disabilities on December 31, 2009. Thirty-four patients of 350 (9.7%) changed the epilepsy classification during follow-up. With regard to ten patients who died, nine of them had symptomatic epilepsy, particularly those with severe underlying disorders with an onset during the first year of life.
    Conclusion: Clinical status considerably changed during the decade-long follow-up period in childhood epilepsy. Changes in the epilepsy diagnosis are especially important and should be taken into account in the long-term care of children with epilepsy. (C) 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2016.10.011

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  • Measurement of pyridoxal 5 '-phosphate, pyridoxal, and 4-pyridoxic acid in the cerebrospinal fluid of children

    Tomoyuki Akiyama, Mari Akiyama, Yumiko Hayashi, Takashi Shibata, Yoshiyuki Hanaoka, Soichiro Toda, Katsumi Imai, Shin-ichiro Hamano, Tohru Okanishi, Harumi Yoshinaga, Katsuhiro Kobayashi

    CLINICA CHIMICA ACTA   466   1 - 5   2017年3月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Background: We quantified pyridoxal 5'-phosphate (PLP), pyridoxal (PL), and 4-pyridoxic acid (PA) in the cerebrospinal fluid (CSF) of children and to investigate the effect of age, sex, epilepsy, and anti-epileptic drug (AED) therapy on these vitamers.
    Methods: CSF samples prospectively collected from 116 pediatric patients were analyzed. PLP, PI, and PA were measured using high-performance liquid chromatography with fluorescence detection, using pre-column derivatization by semicarbazide. Effects of age, sex, epilepsy, and AEDs on these vitamers and the PLP/PL ratio were evaluated using multiple linear regression models.
    Results: The PLP, PL, and PA concentrations were correlated negatively with age and the PLP/PL ratio was correlated positively with age. Multiple regression analysis revealed that the presence of epilepsy was associated with lower PLP concentrations and PLP/PL ratios but sex and AED therapy had no influence on these values. The observed ranges of these vitamers in epileptic and non-epileptic patients were demonstrated.
    Conclusions: We showed the age dependence of PLP and PL in CSF from pediatric patients. Epileptic patients had lower PLP concentrations and PLP/PL ratios than non-epileptic patients, but it is unknown whether this is the cause, or a result, of epilepsy. (C) 2016 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.cca.2016.12.027

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  • Behavioral outcomes of school-aged full-term small-for-gestational-age infants: A nationwide Japanese population-based study

    Akihito Takeuchi, Takashi Yorifuji, Kyohei Takahashi, Makoto Nakamura, Misao Kageyama, Toshihide Kubo, Tatsuya Ogino, Katsuhiro Kobayashi, Hiroyuki Doi

    BRAIN & DEVELOPMENT   39 ( 2 )   101 - 106   2017年2月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Background: Small for gestational age (SGA) birth is linked with neurological deficits among children at pre-school age, but the evidence is still limited on whether such deficits are still observable at school age. We investigated the association between SGA birth and behavioral development at school age among full-term infants.
    Methods: We analyzed data from a large, Japanese, nationwide, population-based longitudinal survey that started in 2001. We restricted the study participants to children born at 37-41 weeks of gestation with information on birth weight and behavioral outcomes at 8 years of age (n = 33,795). Behavioral outcomes including three attentional problems and four aggressive behaviors queried at 8 years of age by survey questions were used as outcome indicators. We then used logistic regression to estimate odds ratios (OR) and 95% confidence intervals (95% CI) for the associations between SGA birth and each outcome, adjusting for potential infant- and parent-related confounding factors.
    Results: Among full-term children, SGA children were more likely to interrupt people (OR 1.10, 95% CI 1.01, 1.20), unable to wait his/her turn (OR 1.17, 95% CI 1.00, 1.38), and destroy toys and/or books (OR 1.15, 95% CI 1.00, 1.31).
    Conclusions: This is the largest study ever conducted on this issue. SGA birth is negatively associated with some attentional problems and aggressive behavior at school age among full-term children. Appropriate long-term developmental follow-up and support may be needed for full-term SGA infants. (C) 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2016.08.007

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  • 結節性硬化症の乳児期における中枢神経症状の展開

    土屋 弘樹, 岡 牧郎, 花岡 義行, 柴田 敬, 林 裕美子, 秋山 麻里, 遠藤 文香, 秋山 倫之, 吉永 治美, 小林 勝弘

    日本小児科学会雑誌   121 ( 2 )   257 - 257   2017年2月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • A Japanese case of β-ureidopropionase deficiency with dysmorphic features

    Akiyama T, Shibata T, Yoshinaga H, Kuhara T, Nakajima Y, Kato T, Maeda Y, Ohse M, Oka M, Kageyama M, Kobayashi K

    Brain Dev   39 ( 1 )   58 - 61   2017年1月

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  • A case of Dravet syndrome with cortical myoclonus indicated by jerk-locked back-averaging of electroencephalogram data

    Yoshinori Kobayashi, Yoshiyuki Hanaoka, Tomoyuki Akiayma, Iori Ohmori, Mamoru Ouchida, Toshiyuki Yamamoto, Makio Oka, Harumi Yoshinaga, Katsuhiro Kobayashi

    BRAIN & DEVELOPMENT   39 ( 1 )   75 - 79   2017年1月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    We report a female patient with Dravet syndrome (DS) with erratic segmental myoclonus, the origin of which was first identified in the cerebral cortex by the detection of myoclonus-associated cortical discharges. The discharges were disclosed through jerk locked back-averaging of electroencephalogram (EEG) data using the muscle activity of myoclonus as triggers. The detected spikes on the contralateral parieto-central region preceded myoclonic muscle activity in the forearms by 28-46 ms. The patient was six months old at the time of examination, and was developing normally before seizure onset at two months of age. She suffered from recurrent afebrile or febrile generalized tonic clonic seizures that often developed into status epilepticus. Interictal EEG and brain magnetic resonance imaging (MRI) showed no significant findings. The amplitudes of the somatosensory-evoked potentials were not extremely large. She has a chromosomal microdeletion involving SCN1A and adjacent genes. (C) 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2016.07.005

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  • Significance of High-frequency Electrical Brain Activity

    Katsuhiro Kobayashi, Tomoyuki Akiyama, Takashi Agari, Tatsuya Sasaki, Takashi Shibata, Yoshiyuki Hanaoka, Mari Akiyama, Fumika Endoh, Makio Oka, Isao Date

    ACTA MEDICA OKAYAMA   71 ( 3 )   191 - 200   2017年

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    記述言語:英語   掲載種別:書評論文,書評,文献紹介等   出版者・発行元:OKAYAMA UNIV MED SCHOOL  

    Electroencephalogram (EEG) data include broadband electrical brain activity ranging from infra-slow bands (<0.1 Hz) to traditional frequency bands (e.g., the approx. 10 Hz alpha rhythm) to high-frequency bands of up to 500 Hz. High-frequency oscillations (HFOs) including ripple and fast ripple oscillations (80-200 Hz and >200/250 Hz, respectively) are particularly of note due to their very close relationship to epileptogenicity, with the possibility that they could function as a surrogate biomarker of epileptogenicity. In contrast, physiological high-frequency activity plays an important role in higher brain functions, and the differentiation between pathological/epileptic and physiological HFOs is a critical issue, especially in epilepsy surgery. HFOs were initially recorded with intracranial electrodes in patients with intractable epilepsy as part of a long-term invasive seizure monitoring study. However, fast oscillations (FOs) in the ripple and gamma bands (40-80 Hz) are now noninvasively detected by scalp EEG and magnetoencephalography, and thus the scope of studies on HFOs/FOs is rapidly expanding.

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  • 前頭葉に発生し,てんかん原性を示した髄膜腫合併髄膜血管腫症の1例

    河原明奈, 伏見聡一郎, 板倉淳哉, 藤澤真義, 小林勝弘, 林裕美子, 伊達 勲, 上利崇, 柳井広之, 松川昭博

    診断病理   34 ( 1 )   46 - 50   2017年

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  • Complex observation of scalp fast (40-150 Hz) oscillations in West syndrome and related disorders with structural brain pathology 国際誌

    Kobayashi K, Endoh F, Agari T, Akiyama T, Akiyama M, Hayashi Y, Shibata T, Hanaoka Y, Oka M, Yoshinaga H, Date I

    Epilepsia Open   2 ( 2 )   260 - 266   2017年

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    記述言語:英語  

    DOI: 10.1002/epi4.12043

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  • 結節性硬化症における多臓器フォローアップの実態

    吉永治美, 岡牧郎, 秋山倫之, 遠藤文香, 秋山麻里, 林裕美子, 柴田敬, 花岡義行, 小林勝弘

    脳と発達   49 ( 1 )   5 - 9   2017年

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  • Importance of the multisystem follow-up in patients with tuberous sclerosis complex

    Harumi Yoshinaga, Makio Oka, Tomoyuki Akiyama, Fumika Endoh, Mari Akiyama, Yumiko Hayashi, Takashi Shibata, Yoshiyuki Hanaoka, Katsuhiro Kobayashi

    No To Hattatsu   49 ( 1 )   5 - 9   2017年

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  • これからの小児てんかんの治療―新規抗てんかん薬を巡る模索―

    小林勝弘

    小児科臨床   70 ( 8 )   1201 - 1209   2017年

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  • Fast (40-150 Hz) oscillations are associated with positive slow waves in the ictal EEGs of epileptic spasms in West syndrome

    Katsuhiro Kobayashi, Tomoyuki Akiyama, Makio Oka, Fumika Endoh, Harumi Yoshinaga

    BRAIN & DEVELOPMENT   38 ( 10 )   909 - 914   2016年11月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: To elucidate the generative mechanisms of epileptic spasms (ESs) in West syndrome, we investigated the temporal relationship between scalp fast (40-150 Hz) oscillations (FOs) and slow waves in the ictal electroencephalograms (EEGs) of ESs.
    Methods: In 11 infants with WS, ictal FOs were detected in a bipolar montage based on spectral and waveform criteria. Their temporal distribution was analyzed in terms of the positive peaks (trough point, TT) of identical EEG data in a referential montage. Among six EEG data sections defined according to TT, the number of FOs, peak power values, and peak frequencies were compared.
    Results: We identified a total of 1014 FOs (946 gamma and 68 ripple oscillations), which clustered closely at TT. The number of gamma oscillations in the 1 s epoch including TT was significantly higher than those in the prior and subsequent phases. Peak power values and frequencies tended to be higher in these positive phase sections.
    Conclusions: The temporal association of FO clustering and positive slow waves in the ictal EEGs of ES indicated that active neuronal firing related to FOs underlies the generation of ESs and their ictal slow waves. (C) 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights

    DOI: 10.1016/j.braindev.2016.05.005

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  • SSADH deficiency possibly associated with enzyme activity-reducing SNPs

    Tomoyuki Akiyama, Hitoshi Osaka, Hiroko Shimbo, Tomiko Kuhara, Takashi Shibata, Katsuhiro Kobayashi, Kenji Kurosawa, Harumi Yoshinaga

    BRAIN & DEVELOPMENT   38 ( 9 )   871 - 874   2016年10月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Background: Succinic semialdehyde dehydrogenase (SSADH) deficiency is a rare autosomal recessive disorder that affects the degradation of gamma-aminobutyric acid and leads to the accumulation of gamma-hydroxybutyric acid (GHB) in body fluids. Diagnosis of SSADH deficiency is challenging, since the neurological symptoms are non-specific.
    Case: The patient is a nine-year-old Japanese boy who presented with developmental delay, autism, epilepsy, and episodic gait disturbance. Brain magnetic resonance imaging showed hyperintense lesions in the bilateral thalami, globus pallidi, substantia nigra, and dentate nuclei. Urine metabolome analysis revealed elevated GHB, which led to a biochemical diagnosis of SSADH deficiency. Genetic analysis of the ALDH5A1 gene revealed a novel missense mutation c.1586G>A inherited from his father. It also demonstrated three single nucleotide polymorphisms (SNPs) (c.106G>C, c.538C>T, and c.545C>T), all of which were inherited from his mother and are known to reduce SSADH enzyme activity. There were no duplications or deletions in other exons in the patient or his parents. No variants in the upstream, intronic, or downstream regions of the ALDH5A1 gene were found in the patient. Enzymatic assay revealed a marked reduction of SSADH enzyme activity (approximate to 2% of the lower limit of the normal range).
    Conclusion: Although other mechanisms cannot be fully excluded, the clinical manifestation of SSADH deficiency in this patient may be attributed to the combined effect of the mutation and the three enzyme activity-reducing SNPs. Urine metabolome analysis effectively detected his elevated GHB and is thus considered to be a good screening method for this underdiagnosed and potentially manageable metabolic disorder. (C) 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2016.03.008

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  • Cytokine-related and sodium channel polymorphism as candidate predisposing factors for childhood encephalopathy FIRES/AERRPS

    M. Saitoh, K. Kobayashi, I. Ohmori, Y. Tanaka, K. Tanaka, T. Inoue, A. Horino, K. Ohmura, A. Kumakura, Y. Takei, S. Hirabayashi, M. Kajimoto, T. Uchida, S. Yamazaki, T. Shiihara, T. Kumagai, M. Kasai, H. Terashima, M. Kubota, M. Mizuguchi

    JOURNAL OF THE NEUROLOGICAL SCIENCES   368   272 - 276   2016年9月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Febrile infection-related epilepsy syndrome (FIRES), or acute encephalitis with refractory, repetitive partial seizures (AERRPS), is an epileptic encephalopathy beginning with fever-mediated seizures. The etiology remains unclear. To elucidate the genetic background of FIRES/AERRPS (hereafter FIRES), we recruited 19 Japanese patients, genotyped polymorphisms of the IL1B, IL6, IL10, TNFA, IL1RN, SCN1A and SCN2A genes, and compared their frequency between the patients and controls. For IL1RN, the frequency of a variable number of tandem repeat (VNTR) allele, RN2, was significantly higher in the patients than in controls (p = 0.0067), and A allele at rs4251981 in 5' upstream of IL1RN with borderline significance (p = 0.015). Haplotype containing RN2 was associated with an increased risk of FIRES (OR 3.88, 95%CI 1.40-10.8, p = 0.0057). For SCN1A, no polymorphisms showed a significant association, whereas a missense mutation, R1575C, was found in two patients. For SCN2A, the minor allele frequency of G allele at rs1864885 was higher in patients with borderline significance (p = 0.011).
    We demonstrated the association of IL1RN haplotype containing RN2 with FIRES, and showed a possible association of IL1RN rs4251981 G > A and SCN2A rs1864885 A > G, in Japanese patients. These preliminary findings suggest the involvement of multiple genetic factors in FIRES, which needs to be confirmed by future studies in a larger number of FIRES cases. (C) 2016 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.jns.2016.07.040

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  • Determination of CSF 5-methyltetrahydrofolate in children and its application for defects of folate transport and metabolism

    Mari Akiyama, Tomoyuki Akiyama, Kaoruko Kanamaru, Mutsuko Kuribayashi, Hiroko Tada, Tsugumi Shiokawa, Soichiro Toda, Katsumi Imai, Yu Kobayashi, Jun Tohyama, Takafumi Sakakibara, Harumi Yoshinaga, Katsuhiro Kobayashi

    CLINICA CHIMICA ACTA   460   120 - 125   2016年9月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: To describe an assay of 5-methyltetrahydrofolate (5MTHF) in the cerebrospinal fluid (CSF) of children, to determine reference values, and to report the clinical significance of this assay in metabolic disorders affecting folate transport and metabolism.
    Methods: CSF 5MTHF was determined by high-performance liquid chromatography with fluorescent detection in pediatric patients including one with FOLR1 gene mutation and one with methylenetetrahydrofolate reductase (MTHFR) deficiency. CSF total folate was measured using an automated analyzer.
    Results: 5MTHF and total folate were determined in 188 and 93 CSF samples, respectively. CSF 5MTHF was high throughout the first six months of life and subsequently declined with age. Reference values of CSF 5MTHF and total folate were determined from 162 and 82 samples, respectively. The patient with FOLR1 gene mutation had extremely low CSF 5MTHF and total folate, though these values normalized after folinic acid supplementation. The patient with MTHFR deficiency had extremely low 5MTHF and moderately low total folate; these values were not associated and showed no significant change after folic acid supplementation.
    Conclusions: This 5MTHF assay is simple, rapid, sensitive, reliable, and cost-effective. It will aid in the diagnosis and therapeutic monitoring of metabolic disorders affecting folate transport and metabolism. (C) 2016 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.cca.2016.06.032

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  • Idiopathic focal epilepsies: the "lost tribe"

    Deb K. Pal, Colin Ferrie, Laura Addis, Tomoyuki Akiyama, Giuseppe Capovilla, Roberto Caraballo, Anne de Saint-Martin, Natalio Fejerman, Renzo Guerrini, Khalid Hamandi, Ingo Helbig, Andreas A. Ioannides, Katsuhiro Kobayashi, Dennis Lal, Gaetan Lesca, Hiltrud Muhle, Bernd A. Neubauer, Tiziana Pisano, Gabrielle Rudolf, Caroline Seegmuller, Takashi Shibata, Anna Smith, Pasquale Striano, Lisa J. Strug, Pierre Szepetowski, Thalia Valeta, Harumi Yoshinaga, Michalis Koutroumanidis

    EPILEPTIC DISORDERS   18 ( 3 )   252 - 288   2016年9月

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    記述言語:英語   掲載種別:書評論文,書評,文献紹介等   出版者・発行元:JOHN LIBBEY EUROTEXT LTD  

    The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community. Most neurologists would recognise the core syndromes of IFE to comprise: benign epilepsy of childhood with centro-temporal spikes or Rolandic epilepsy (BECTS/RE); Panayiotopoulos syndrome; and the idiopathic occipital epilepsies (Gastaut and photosensitive types). The Landau-Kleffner syndrome and the related (idiopathic) epilepsy with continuous spikes and waves in sleep (CSWS or ESES) are also often included, both as a consequence of the shared morphology of the interictal discharges and their potential evolution from core syndromes, for example, CSWS from BECTS. Atypical benign focal epilepsy of childhood also has shared electro-clinical features-warranting inclusion. In addition, a number of less well-defined syndromes of IFE have been proposed, including benign childhood seizures with affective symptoms, benign childhood epilepsy with parietal spikes, benign childhood seizures with frontal or midline spikes, and benign focal seizures of adolescence.
    The term "benign" is often used in connection with the IFEs and is increasingly being challenged. Certainly most of these disorders are not associated with the devastating cognitive and behavioural problems seen with early childhood epileptic encephalopathies, such as West or Dravet syndromes. However, it is clear that specific, and sometimes persistent, neuropsychological deficits in attention, language and literacy accompany many of the IFEs that, when multiplied by the large numbers affected, make up a significant public health problem. Understanding the nature, distribution, evolution, risk and management of these is an important area of current research.A corollary to such questions regarding comorbidities is the role of focal interictal spikes and their enduring impact on cognitive functioning. What explains the paradox that epilepsies characterised by abundant interictal epileptiform abnormalities are often associated with very few clinical seizures? This is an exciting area in both clinical and experimental arenas and will eventually have important implications for clinical management of the whole child, taking into account not just seizures, but also adaptive functioning and quality of life. For several decades, we have accepted an evidence-free approach to using or not using antiepileptic drugs in IFEs. There is huge international variation and only a handful of studies examining neurocognitive outcomes. Clearly, this is a situation ready for an overhaul in practice.
    Fundamental to understanding treatment is knowledge of aetiology. In recent years, there have been several significant discoveries in IFEs from studies of copy number variation, exome sequencing, and linkage that prompt reconsideration of the "unknown cause" classification and strongly suggest a genetic aetiology. The IFE are strongly age-related, both with regards to age of seizure onset and remission. Does this time window solely relate to a similar age-related gene expression, or are there epigenetic factors involved that might also explain low observed twin concordance? The genetic (and epigenetic) models for different IFEs, their comorbidities, and their similarities to other neurodevelopmental disorders deserve investigation in the coming years. In so doing, we will probably learn much about normal brain functioning. This is because these disorders, perhaps more than any other human brain disease, are disorders of functional brain systems (even though these functional networks may not yet be fully defined). In June 2012, an international group of clinical and basic science researchers met in London under the auspices of the Waterloo Foundation to discuss and debate these issues in relation to IFEs. This Waterloo Foundation Symposium on the Idiopathic Focal Epilepsies: Phenotype to Genotype witnessed presentations that explored the clinical phenomenology, phenotypes and endophenotypes, and genetic approaches to investigation of these disorders. In parallel, the impact of these epilepsies on children and their families was reviewed. The papers in this supplement are based upon these presentations. They represent an updated state-of-the-art thinking on the topics explored.
    The symposium led to the formation of internationalworking groups under the umbrella of "Luke's Idiopathic Focal Epilepsy Project" to investigate various aspects of the idiopathic focal epilepsies including: semiology and classification, genetics, cognition, sleep, high-frequency oscillations, and parental resources (see www.childhood-epilepsy.org). The next sponsored international workshop, in June 2014, was on randomised controlled trials in IFEs and overnight learning outcome measures.

    DOI: 10.1684/epd.2016.0839

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  • 多彩な先天異常を認めたβ-ウレイドプロピオナーゼ欠損症の一例

    柴田 敬, 秋山 倫之, 岡 牧郎, 吉永 治美, 久原 とみ子, 中島 葉子, 加藤 武馬, 前田 康博, 影山 操, 小林 勝弘

    日本先天代謝異常学会雑誌   32   201 - 201   2016年9月

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    記述言語:日本語   出版者・発行元:日本先天代謝異常学会  

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  • 脳波におけるCSWSのDZP静注による改善程度と治療予後の検討

    秋山 麻里, 岡 牧郎, 秋山 倫之, 土屋 弘樹, 西本 静香, 森 篤志, 吉永 治美, 小林 勝弘

    てんかん研究   34 ( 2 )   492 - 492   2016年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • 新生児・乳児期発症てんかんにおける髄液中ピリドキサールリン酸測定の意義

    秋山 倫之, 柴田 敬, 吉永 治美, 小林 勝弘

    日本先天代謝異常学会雑誌   32   205 - 205   2016年9月

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    記述言語:日本語   出版者・発行元:日本先天代謝異常学会  

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  • A Japanese case of hereditary chin trembling responsive to arotinolol

    Tomoyuki Akiyama, Hiroyuki Miyahara, Kenji Waki, Harumi Yoshinaga, Katsuhiro Kobayashi

    PARKINSONISM & RELATED DISORDERS   29   133 - 134   2016年8月

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    記述言語:英語   掲載種別:速報,短報,研究ノート等(学術雑誌)   出版者・発行元:ELSEVIER SCI LTD  

    DOI: 10.1016/j.parkreldis.2016.04.021

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  • コハク酸セミアルデヒド脱水素酵素欠損症の1男児例

    柴田 敬, 小林 勝弘, 秋山 倫之, 岡 牧郎, 小坂 仁, 久原 とみ子, 黒澤 健司, 吉永 治美

    日本小児科学会雑誌   120 ( 7 )   1130 - 1130   2016年7月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • A novel HYLS1 homozygous mutation in living siblings with Joubert syndrome

    M. Oka, K. Shimojima, T. Yamamoto, Y. Hanaoka, S. Sato, T. Yasuhara, H. Yoshinaga, K. Kobayashi

    CLINICAL GENETICS   89 ( 6 )   739 - 743   2016年6月

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    記述言語:英語   出版者・発行元:WILEY-BLACKWELL  

    The p.Asp211Gly homozygous HYLS1 mutation is so far known to cause only hydrolethalus syndrome, a lethal malformation syndrome. We report living sibling patients with a homozygous no-stop mutation in exon 4 of HYLS1, NM_145014.2: c.900A>C (p.Ter300TyrextTer11) in the second decade of life. The proband has Joubert syndrome (JS). The younger brother also has JS and an enlarged posterior fossa that was initially diagnosed as Dandy-Walker malformation. The present mutation is unique as it affects the stop codon. The product protein HYLS1 plays an essential role in the formation of the primary cilium. This report provides insight into the spectrum of disorders involving midline brain defects closely related to cilium dysfunction or ciliopathy.

    DOI: 10.1111/cge.12752

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  • Surgical versus medical treatment for children with epileptic encephalopathy in infancy and early childhood: Results of an international multicenter cohort study in Far-East Asia (the FACE study)

    Taisuke Otsuki, Heung-Dong Kim, Guoming Luan, Yushi Inoue, Hiroshi Baba, Hirokazu Oguni, Seung-Chyul Hong, Shigeki Kameyama, Katsuhiro Kobayashi, Shinichi Hirose, Hitoshi Yamamoto, Shin-ichiro Hamano, Kenji Sugai

    BRAIN & DEVELOPMENT   38 ( 5 )   449 - 460   2016年5月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: To compare the seizure and developmental outcomes in infants and young children with epileptic encephalopathy who have undergone surgical and medical treatments.
    Methods: An international, multicenter, observational cohort study was undertaken. A total of 317 children aged <6 years, who had frequent disabling seizures despite intensive medical treatments, were registered. Among the enrolled children, 250 were treated medically (medical group), 31 underwent resective surgery (resective group), and 36 underwent palliative surgery [callosotomy (n = 30) or vagal nerve stimulation (n = 6); palliative group] on admission. Seizure and developmental outcomes were obtained for 230 children during the 3-year follow-up period. Cox proportional hazard model was used to adjust for clinical backgrounds among treatment groups when comparing the seizure-free survival rates.
    Results: At the 3-year follow-up, seizure-free survival was 15.7%, 32.1%, and 52.4% in the medical, palliative, and resective groups, respectively. The adjusted hazard ratios for seizure recurrence in the resective and palliative groups versus the medical group were 0.43 (95% CI, 0.21-0.87, P = 0.019) and 0.82 (95% CI, 0.46-1.46, P = 0.50), respectively; the former was statistically significant. Regarding the developmental outcome, the mean DQs in the resective group increased significantly compared to those in the medical group during the follow-up (P < 0.01). As for subgroup analysis, better seizure and development outcomes were demonstrated in the resective group compared to the medical group in children with nonsyndromic epilepsies (those to which no known epilepsy syndromes were applicable).
    Significance: These results suggest that surgical treatments, particularly resective surgeries, are associated with better seizure and developmental outcomes compared with successive medical treatment. The present observations may facilitate the identification of infants and young children with epileptic encephalopathy who could benefit from surgery. (C) 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2015.11.004

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  • Dravet症候群早期診断におけるスクリーニングテストの有用性

    秋山 麻里, 林 裕美子, 遠藤 文香, 吉永 治美, 小林 勝弘

    脳と発達   48 ( Suppl. )   S274 - S274   2016年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • てんかん患者における長期的ビタミンB6療法は末梢神経に有害な影響を与えるか?

    井上 拓志, 柴田 敬, 遠藤 文香, 岡 牧郎, 秋山 倫之, 小林 勝弘, 吉永 治美

    脳と発達   48 ( Suppl. )   S362 - S362   2016年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • Hereditary chin tremblingの小児例

    秋山 倫之, 柴田 敬, 花岡 義行, 吉永 治美, 小林 勝弘

    脳と発達   48 ( Suppl. )   S267 - S267   2016年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 幼児期早期に退行をきたし、環状22番染色体を認めた自閉症スペクトラム障害の男児例

    岡 牧郎, 小林 由典, 秋山 麻里, 諸岡 輝子, 花房 香, 荻野 竜也, 吉永 治美, 小林 勝弘

    脳と発達   48 ( Suppl. )   S380 - S380   2016年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 結節性硬化症のフォローアップ中にLAMが見過ごされた2例

    吉永 治美, 秋山 麻里, 遠藤 文香, 秋山 倫之, 小林 勝弘

    日本小児科学会雑誌   120 ( 2 )   387 - 387   2016年2月

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  • Predictive factors for relapse of epileptic spasms after adrenocorticotropic hormone therapy in West syndrome

    Yumiko Hayashi, Harumi Yoshinaga, Tomoyuki Akiyama, Fumika Endoh, Yoko Ohtsuka, Katsuhiro Kobayashi

    BRAIN & DEVELOPMENT   38 ( 1 )   32 - 39   2016年1月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Purpose: To investigate whether serial electroencephalographic (EEG) findings can predict relapse of epileptic spasms after synthetic adrenocorticotropic hormone (ACTH) therapy in patients with West syndrome (WS).
    Subjects and methods: Thirty-nine WS patients (8 cryptogenic and 31 symptomatic) were included in this study. These patients received ACTH therapy for the first time and were regularly followed up for more than three years at our hospital. Sixteen patients (41.0%) showed seizure relapse (relapse group) and 23 patients (59.0%) did not show relapse (non-relapse group). We used survival analysis to investigate the influence of etiology and presence of epileptic discharges after the ACTH therapy on seizure outcome.
    Results: Immediately after the ACTH therapy, etiology was associated with seizure outcome (p = 0.003). In the early stage (1 month after the ACTH therapy), only the presence of epileptic discharges (p = 0.001) had a significant association with seizure outcome, regardless of etiology. Because all relapsed patients were in the symptomatic group, we performed the same statistical analysis on symptomatic WS patient data only. We found that the group with no epileptic discharges on EEG showed a significantly higher seizure-free rate than those with epileptic discharges in the early stage (p = 0.0091).
    Conclusion: This study demonstrated that serial EEG findings after ACTH therapy are significantly related to relapse of epileptic spasms. (c) 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2015.05.012

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  • Occurrence of bilaterally independent epileptic spasms after a corpus callosotomy in West syndrome

    Katsuhiro Kobayashi, Fumika Endoh, Yoshihiro Toda, Makio Oka, Hiroshi Baba, Yoko Ohtsuka, Harumi Yoshinaga

    BRAIN & DEVELOPMENT   38 ( 1 )   132 - 135   2016年1月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    We report a patient with intractable West syndrome whose epileptic spasms (ESs) were initially bilaterally synchronous, as is typical; after a complete corpus callosotomy, however, bilaterally independent ESs originated in either hemisphere. Activity of probable cortical origin associated with ESs was detected by observing ictal gamma oscillations. Brain MRI revealed no structural abnormality before surgery. This case suggests that ESs with a hemispheric origin may appear generalized because of synchronizing effects in the corpus callosum in some patients. (c) 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2015.05.003

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  • The identification of distinct high-frequency oscillations during spikes delineates the seizure onset zone better than high-frequency spectral power changes

    Julia Jacobs, Christina Vogt, Pierre Levan, Rina Zelmann, Jean Gotman, Katsuhiro Kobayashi

    CLINICAL NEUROPHYSIOLOGY   127 ( 1 )   129 - 142   2016年1月

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    記述言語:英語   出版者・発行元:ELSEVIER IRELAND LTD  

    Objective: Interictal high-frequency oscillations (HFOs, 80-500 Hz) can predict the seizure onset zone (SOZ), but visual detection of HFOs is time consuming. Time-frequency analysis can reveal large high-frequency (HF) power changes (80-500 Hz) associated with inter-ictal spikes. The present study determines how well the rate of HFOs and spike-related HF power changes were co-localized with SOZ.
    Methods: We analyzed 583 channels (68 in the SOZ) sampled from 14 patients who underwent intracranial EEG recording. We determined if the rate of visually-marked HFOs and spike-related HF power changes differed between SOZ and non-SOZ.
    Results: Significantly higher rates of HFOs were found in SOZ. The degree of spike-related HF power augmentation failed to differ between SOZ and non-SOZ, whereas that of post-spike HF power attenuation was significantly more severe in SOZ compared to in non-SOZ. Regions showing HFOs and large spike-related HF-changes showed a partial overlap in distribution in 7/14 patients.
    Conclusions: Strong HF augmentation during spikes and high HFO rates occurred over different brain locations. The rate of HFOs showed the best performance in identifying SOZ. Post-spike HF power attenuation may represent increased inhibition in these channels and should be investigated further.
    Significance: Strong HF power changes during spikes and HFOs per se seem to reflect distinct phenomena. (C) 2015 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

    DOI: 10.1016/j.clinph.2015.04.053

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  • てんかん

    小林勝弘

    小児内科   48 ( 2016年増刊号 )   404 - 410   2016年

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  • A study on spike focus-dependence of high-frequency activity in idiopathic focal epilepsy in childhood

    Shibata T, Yoshinaga H, Akiyama T, Kobayashi K

    Epilepsia Open   1 ( 2017/03/04 )   121 - 129   2016年

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  • 脳波:小児てんかんにおける古くて新しい検査

    小林勝弘

    岡山医学会雑誌   128 ( 3 )   183 - 189   2016年

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  • Total folate and 5-methyltetrahydrofolate in the cerebrospinal fluid of children: correlation and reference values

    Tomoyuki Akiyama, Hiroko Tada, Tsugumi Shiokawa, Katsuhiro Kobayashi, Harumi Yoshinaga

    CLINICAL CHEMISTRY AND LABORATORY MEDICINE   53 ( 12 )   2009 - 2014   2015年11月

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    記述言語:英語   出版者・発行元:WALTER DE GRUYTER GMBH  

    Background: Cerebral folate deficiency (CFD) may be underdiagnosed, as it manifests with various non-specific neurological symptoms. The diagnosis of CFD requires a determination of 5-methyltetrahydrofolate (5MTHF) in the cerebrospinal fluid (CSF), which is available in a limited number of specialized laboratories. In clinical biochemistry laboratories, total folate (TF) determination in serum or plasma is routinely performed by automated analyzers. The aim of this study is to determine whether the automated assay of CSF TF is a helpful screening tool for CFD.
    Methods: We analyzed CSF samples collected from 73 pediatric patients. We measured CSF TF, serum TF, and CSF 5MTHF in 73, 70, and 48 patients, respectively. The assay of 5MTHF was conducted by a newly developed system utilizing liquid chromatography-tandem mass spectrometry (LC-MS/ MS). We investigated the correlation between TF and 5MTHF in the CSF.
    Results: There was a strong positive correlation between CSF TF and 5MTHF (rho=0.930, p<0.0001, n=48). Age was negatively correlated with CSF TF (rho=-0.557, p<0.0001, n=51), serum TF (rho=-0.457, p=0.0008, n=51), and CSF 5MTHF (rho=-0.387, p=0.0263, n=33), but not with the CSF/serum TF ratio.
    Conclusions: The automated assay of CSF TF is helpful to estimate CSF 5MTHF. The CSF TF assay may have a significant impact on the early diagnosis of CFD, because clinicians have better access to it than the 5MTHF assay.

    DOI: 10.1515/cclm-2015-0208

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  • インフルエンザの治癒・回復期にせん妄、精神症状を呈した2症例

    秋山 麻里, 遠藤 文香, 岡 牧郎, 小林 勝弘, 吉永 治美

    脳と発達   47 ( 6 )   457 - 457   2015年11月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 小児髄液中モノアミン類の測定系の作成

    秋山 倫之, 小林 勝弘, 井上 拓志, 吉永 治美

    脳と発達   47 ( 6 )   457 - 458   2015年11月

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  • Late-onset epileptic spasms in a female patient with a CASK mutation

    Tomoshi Nakajiri, Katsuhiro Kobayashi, Nobuhiko Okamoto, Makio Oka, Fuyuki Miya, Kenjiro Kosaki, Harumi Yoshinaga

    BRAIN & DEVELOPMENT   37 ( 9 )   919 - 923   2015年10月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    We report a female patient with late-onset epileptic spasms (ESs) of a rare form, distinct from those seen in typical West syndrome, in association with a heterozygous frameshift CASK mutation (c.1896dupC (p.C633fs*2)). She has a phenotype of microcephaly with pontine and cerebellar hypoplasia (MICPCH), and has had intractable ESs in clusters since 3 years 8 months of age with multifocal, particularly bifrontal, epileptic discharges in electroencephalogram.
    The available literature on patients with both ESs and CASK mutations has been reviewed, revealing that four of the five female children, including the present girl, had late-onset ESs, in contrast to the four males, who tended toward early-onset ESs. (C) 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2015.02.007

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  • コハク酸セミアルデヒド脱水素酵素欠損症の1男児例

    柴田 敬, 小林 勝弘, 秋山 倫之, 岡 牧郎, 小坂 仁, 久原 とみ子, 黒澤 健司, 吉永 治美

    日本先天代謝異常学会雑誌   31   150 - 150   2015年10月

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    記述言語:日本語   出版者・発行元:日本先天代謝異常学会  

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  • Spatial relationship between fast and slow components of ictal activities and interictal epileptiform discharges in epileptic spasms

    Tomoyuki Akiyama, Mari Akiyama, Katsuhiro Kobayashi, Tohru Okanishi, Cyrus G. Boelman, Dragos A. Nita, Ayako Ochi, Cristina Y. Go, O. Carter Snead, James T. Rutka, James M. Drake, Sylvester Chuang, Hiroshi Otsubo

    CLINICAL NEUROPHYSIOLOGY   126 ( 9 )   1684 - 1691   2015年9月

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    記述言語:英語   出版者・発行元:ELSEVIER IRELAND LTD  

    Objective: We analyzed the spatial distribution and concordance of fast (>10 Hz) and slow (<5 Hz) electroencephalogram (EEG) components of ictal activities and interictal epileptiform discharges (IIED) recorded by intracranial video EEG (IVEEG) in children with epileptic spasms (ES).
    Methods: We studied eight children with ES, who underwent IVEEG before resective surgery for epilepsy. We quantified the root-mean-square (RMS) amplitude of the fast and slow components of ictal activities during ES and IIED. We compared the concordance between the spatial distributions of the fast and slow components of ES and IIED.
    Results: There was a larger concordance between the spatial distributions of the fast and slow components in IIED than in ES (p = 0.0206 and 0.0401).
    Conclusions: The spatial concordance between the fast and slow EEG components was significantly different between ES and IIED.
    Significance: The mechanisms underlying the generation of slow EEG components may differ between ES and IIED. The slow EEG components of ES might indicate an extensive epileptic network involving remote symptomatic zones for ES in either the cortical or subcortical areas. The high spatial concordance between the fast and slow components of IIED suggests the involvement of a local inhibitory process within the epileptic cortex. (C) 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

    DOI: 10.1016/j.clinph.2014.12.005

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  • てんかん性スパズムの機序と治療 スパズムを呈する乳幼児てんかん性脳症の治療予後 FACE studyの解析より

    大槻 泰介, 須貝 研司, 馬場 啓至, 井上 有史, 小林 勝弘, 小国 弘量, 廣瀬 伸一, 山本 仁, 亀山 茂樹, Kim Heung-Dong, Luan Guoming, Hong Sung-Chul, FACE study group

    てんかん研究   33 ( 2 )   372 - 373   2015年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • てんかん患者において長期的ビタミンB6療法が末梢神経に及ぼす影響

    井上 拓志, 柴田 敬, 遠藤 文香, 岡 牧郎, 秋山 倫之, 小林 勝弘, 吉永 治美

    てんかん研究   33 ( 2 )   622 - 622   2015年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • Dravet症候群早期診断におけるスクリーニングテストの有用性

    秋山 麻里, 吉永 治美, 林 裕美子, 花岡 義行, 小林 勝弘

    てんかん研究   33 ( 2 )   513 - 513   2015年9月

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  • Poor toddler-age sleep schedules predict school-age behavioral disorders in a longitudinal survey

    Katsuhiro Kobayashi, Takashi Yorifuji, Michiyo Yamakawa, Makio Oka, Sachiko Inoue, Harumi Yoshinaga, Hiroyuki Doi

    BRAIN & DEVELOPMENT   37 ( 6 )   572 - 578   2015年6月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: Behavioral problems are often associated with poor sleep habits in children. We investigated whether undesirable toddler-age sleep schedules may be related to school-age behavioral problems.
    Methods: We analyzed the data of a nationwide longitudinal survey with available results from 2001 to 2011. The participants were 41,890 children. The predictors were waking time and bedtime at 2 years of age, and the outcomes were assessed by determining the presence or absence of three attention problems and four aggressiveness problems at 8 years of age. In logistic regression models with adjustments for confounding factors, we estimated odds ratios (ORs) and confidence intervals (CIs) for the association between toddler sleep schedules and behavior during primary-school age years.
    Results: The outcomes of attention problems and aggressiveness problems were observed in 1.7% and 1.2% of children, respectively, at 8 years of age. The OR of an irregular or late morning waking time at 2 years of age with the outcome of aggressiveness problems was 1.52 (95% CI, 1.04-2.22) in comparison to an early waking time. The OR of an irregular or late bedtime with attention problems was 1.62 (95% CI, 1.12-2.36), and the OR of an irregular or late bedtime with aggressiveness problems was 1.81 (95% CI, 1.19-2.77) in comparison to an early bedtime.
    Conclusion: Poor toddler-age sleep schedules were found to predict behavioral problems during primary-school age years. Thus, good and regular sleep habits appear to be important for young children's healthy development. (C) 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2014.10.004

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  • Steroid-dependent sensorineural hearing loss in a patient with Charcot-Marie-Tooth disease showing auditory neuropathy

    Yukihide Maeda, Yuko Kataoka, Akiko Sugaya, Shin Kariya, Katsuhiro Kobayashi, Kazunori Nishizaki

    AURIS NASUS LARYNX   42 ( 3 )   249 - 253   2015年6月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI LTD  

    Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of hearing by prednisolone. An 8-year-old boy was referred to the otolaryngology department at the University Hospital. He had been diagnosed with CMT type 1 (demyelinating type) at the Child Neurology Department and was suffering from mild hearing loss due to auditory neuropathy. An audiological diagnosis of auditory neuropathy was confirmed by auditory brainstem response and distortion-product otoacoustic emissions. At 9 years and 0 months old, 9 years and 2 months old, and 10 years and 0 months old, he had experienced acute exacerbations of hearing loss, each of which was successfully rescued by intravenous or oral prednisolone within 2 weeks. Steroid-responsive cases of CMT have been reported, but this is the first case report of steroid-responsive sensorineural hearing loss in CMT. The present case may have implications for the mechanisms of action of glucocorticoids in the treatment of sensorineural hearing loss. (C) 2014 Elsevier Ireland Ltd. All rights reserved.

    DOI: 10.1016/j.anl.2014.11.002

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  • Phenotypic Variability in Childhood of Skeletal Muscle Sodium Channelopathies

    Harumi Yoshinaga, Shunichi Sakoda, Takashi Shibata, Tomoyuld Aldyama, Makio Oka, Jun-Hui Yuan, Hiroshi Takashima, Masanori P. Takahashi, Tetsuro Kitamura, Nagako Murakami, Katsuhiro Kobayashi

    PEDIATRIC NEUROLOGY   52 ( 5 )   504 - 508   2015年5月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE INC  

    BACKGROUND: Mutations of the SCN4A gene cause several skeletal muscle channelopathies and overlapping forms of these disorders. However, the variability of the clinical presentation in childhood is confusing and not fully understood among pediatric neurologists. PATIENTS: We found three different mutations (p.V445M, p.I693L, and a novel mutation, p.V1149L) in SCN4A but not in the CLCN1 gene. The patient with p.V445M showed the clinical phenotype of sodium channel myotonia, but her clear symptoms did not appear until 11 years of age. Her younger sister and mother, who have the same mutation, displayed marked intrafamilial phenotypic heterogeneity from mild to severe painful myotonia with persistent weakness. The patient with p.I693L exhibited various symptoms that evolved with age, including apneic episodes, tonic muscular contractions during sleep, fluctuating severe episodic myotonia, and finally episodic paralyses. The patient with the novel p.V1149L mutation exhibited episodic paralyses starting at 3 years of age, and myotonic discharges were detected at 11 years of age for the first time. CONCLUSION: The present cohort reveals the complexity, variability, and overlapping nature of the clinical features of skeletal muscle sodium channelopathies. These are basically treatable disorders, so it is essential to consider genetic testing before the full development of a patient's condition.

    DOI: 10.1016/j.pediatrneurol.2015.01.014

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  • Trend figures assist with untrained emergency electroencephalogram interpretation

    Katsuhiro Kobayashi, Kosuke Yunoki, Kazumasa Zensho, Tomoyuki Akiyama, Makio Oka, Harumi Yoshinaga

    BRAIN & DEVELOPMENT   37 ( 5 )   487 - 494   2015年5月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: Acute electroencephalogram (EEG) findings are important for diagnosing emergency patients with suspected neurological disorders, but they can be difficult for untrained medical staff to interpret. In this research, we will develop an emergency EEG trend figure that we hypothesize will be more easily understood by untrained staff compared with the raw original traces. Methods: For each of several EEG patterns (wakefulness, sleep, seizure activity, and encephalopathy), trend figures incorporating information on both amplitude and frequency were built. The accuracy of untrained reviewers' interpretation was compared with that of the raw EEG trace interpretation. Results: The rate of correct answers was significantly higher in response to the EEG trend figures than to the raw traces showing wakefulness, sleep, and encephalopathy, but there was no difference when seizure activity patterns were viewed. The rates of misjudging normal or abnormal findings were significantly lower with the trend figures in the wakefulness pattern; in the other patterns, misjudgments were equally low for the trend figures and the raw traces. Conclusion: EEG trend figures improved the accuracy with which untrained medical staff interpreted emergency EEGs. Emergency EEG figures that can be understood intuitively with minimal training might improve the accuracy of emergency EEG interpretation. However, additional studies are required to confirm these results because there may be many types of clinical EEGs that are difficult to interpret. (C) 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2014.08.006

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  • Action potentials contribute to epileptic high-frequency oscillations recorded with electrodes remote from neurons

    Katsuhiro Kobayashi, Tomoyuki Akiyama, Iori Ohmori, Harumi Yoshinaga, Jean Gotman

    CLINICAL NEUROPHYSIOLOGY   126 ( 5 )   873 - 881   2015年5月

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    記述言語:英語   出版者・発行元:ELSEVIER IRELAND LTD  

    Objective: The importance of epileptic high-frequency oscillations (HFOs) in electroencephalogram (EEG) is growing. Action potentials generating some HFOs are observed in the vicinity of neurons in experimental animals. However electrodes that are remote from neurons, as in case of clinical situations, should not record action potentials. We propose to resolve this question by a realistic simulation of epileptic neuronal network.
    Methods: The rat dentate gyrus with sclerosis was simulated in silico. We computed the current dipole moment generated by each granule cell and the field potentials in a measurement area far from neurons.
    Results: The dentate gyrus was stimulated through synaptic input to evoke discharges resembling interictal epileptiform discharges, which had superimposed HFOs <= 295 Hz that were recordable with remote electrodes and represented bursts of action potentials of granule cells. The increase in power of HFOs was associated with the progression of sclerosis, the reduction of GABAergic inhibition, and the increase in cell connectivity. Spectral frequency of HFOs had similar tendencies.
    Conclusions: HFOs recorded with electrodes remote from neurons could actually be generated by clusters of action potentials. Significance: The phenomenon of action potentials recorded with remote electrodes can possibly extend the clinical meaning of EEG. (C) 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

    DOI: 10.1016/j.clinph.2014.08.010

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  • 読字・書字能力の発達 就学前、小学校1年生、2年生の追跡的研究(第二報)

    岡 牧郎, 花房 香, 諸岡 輝子, 秋山 麻里, 荻野 竜也, 小林 勝弘, 吉永 治美

    脳と発達   47 ( Suppl. )   S364 - S364   2015年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • Acute respiratory distress syndrome in a child with severe epileptic disorder treated successfully by extracorporeal membrane oxygenation: a case report

    Nobuyuki Nosaka, Shingo Ichiba, Kohei Tsukahara, Emily Knaup, Kumiko Hayashi, Shingo Kasahara, Yoshinori Kobayashi, Makio Oka, Katsuhiro Kobayashi, Harumi Yoshinaga, Yoshihito Ujike

    BMC PEDIATRICS   15   29 - 29   2015年4月

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    記述言語:英語   出版者・発行元:BIOMED CENTRAL LTD  

    Background: Extracorporeal membrane oxygenation (ECMO) is now a candidate therapy for children with acute respiratory failure.
    Case presentation: We report our experience of using central ECMO therapy for acute respiratory distress syndrome followed by seizure in a 15-month-old girl with a severe epileptic disorder. Her respiratory distress was refractory to standard medical treatment and mechanical ventilatory support. Her condition was complicated by development of a pneumothorax. The patient was successfully weaned off ECMO and discharged without deterioration of her neurological status.
    Conclusion: The successful outcome in this case resulted from the central ECMO, which enabled "lung rest" and adequate cerebral blood flow. In skilled ECMO facilities, early implementation of ECMO would give some advantages to patients such as the one presented here. Given the invasiveness and the ease of the procedure, introduction of dual-lumen catheters adequately sized for pediatric patients in Japan is required.

    DOI: 10.1186/s12887-015-0348-1

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  • High-frequency EEG activity in epileptic encephalopathy with suppression-burst

    Yoshihiro Toda, Katsuhiro Kobayashi, Yumiko Hayashi, Takushi Inoue, Makio Oka, Fumika Endo, Harumi Yoshinaga, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   37 ( 2 )   230 - 236   2015年2月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: We explored high-frequency activity in the suppression-burst (SB) pattern of interictal electroencephalogram (EEG) in early infantile epileptic encephalopathy including Ohtahara syndrome (OS) and early myoclonic encephalopathy (EME) to investigate the pathophysiological characteristics of SB.
    Methods: Subjects included six patients with the SB EEG pattern related to OS or EME (Group SB). The results were evaluated in comparison to trace alternant (TA) observed during the neonatal period in nine patients to rule out possible nonspecific relationships between high-frequency activity and periodic EEG patterns (Group TA). EEG was digitally recorded with a sampling rate of 500 Hz and the analysis was performed in each of the particular bipolar channel-pairs. We visually selected 20 typical consecutive burst sections and 160 inter-burst sections for comparison from the sleep record of each patient and performed the time frequency analysis. We investigated the maximum frequencies of power enhancement in each derivation in both groups.
    Results: In Group SB, a significant increase in power at a frequency of 80-150 Hz was observed in association with the bursts, particularly in the bilateral parieto-occipital. derivations, in all patients. In Group TA, on the contrary, no significant increase in high-frequency power was found. The maximum frequencies of power enhancement were significantly higher in Group SB than in Group TA (p < 0.001 by repeated-measures ANOVA).
    Conclusion: Interictal high frequencies of up to 150 Hz were detected in the suppression-burst EEG patterns in epileptic encephalopathy in early infancy. Further studies will be necessary to identify the role of the interictal high-frequency activity in the pathophysiology of such early epileptic encephalopathy. (C) 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2014.04.004

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  • デジタル脳波の記録・判読指針

    池田 昭夫, 赤松 直樹, 小林 勝弘, 酒田 あゆみ, 末永 和榮, 飛松 省三, 橋本 修治, 松浦 雅人, 重藤 寛史, 寺田 清人, 松本 理器, 日本臨床神経生理学会ペーパレス脳波の記録・判読指針小委員会

    臨床神経生理学   43 ( 1 )   22 - 62   2015年2月

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    記述言語:日本語   出版者・発行元:(一社)日本臨床神経生理学会  

    DOI: 10.11422/jscn.43.22

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  • 合成ACTH療法におけるST合剤の使用と好中球減少症の関連

    秋山 麻里, 岡 牧郎, 小林 勝弘, 吉永 治美

    日本小児科学会雑誌   119 ( 2 )   389 - 389   2015年2月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • 日本人小児髄液中5-HIAA、HVA、遊離GABAの基準値作成の試み

    秋山 倫之, 小林 勝弘, 秋山 麻里, 岡 牧郎, 遠藤 文香, 吉永 治美

    日本小児科学会雑誌   119 ( 2 )   444 - 444   2015年2月

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    記述言語:日本語   出版者・発行元:(公社)日本小児科学会  

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  • Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy

    Harumi Yoshinaga, Katsuhiro Kobayashi, Takashi Shibata, Takushi Inoue, Makio Oka, Tomoyuki Akiyama

    BRAIN & DEVELOPMENT   37 ( 1 )   13 - 17   2015年1月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Purpose: Benign childhood epilepsy with centro-temporal spikes (BECTS) and Panayiotopoulos syndrome (PS) have different pathophysiologies and show different types of seizures, yet they overlap in some important respects. In an attempt to understand the ways in which they differ from each other and overlap each other, we performed a detailed investigation on patients who had both characteristic types of seizure manifestations, namely, sylvian seizures and emetic seizures. Subjects and methods: We recruited consecutive subjects from the EEG database of outpatients who had visited our hospital between 2008 and 2010 and who had been diagnosed with BECTS or PS. As a result, 45 patients with BECTS and 50 patients with PS were selected from the database. Viewing the clinical records of these 95 patients, five patients were selected who had experienced both sylvian seizures and emetic seizures. Next, the clinical features and EEG findings of these five patients were retrospectively observed at the date of investigation: October 1, 2011. Results: We found that all the patients showed rolandic spikes when they had sylvian seizures, and occipital spikes or multifocal spikes when they had emetic seizures. We also report in detail on one patient who showed two different types of ictal EEG patterns: one of which started in the occipital area and the other of which was located in the rolandic area. Conclusion: Based on these findings, we conclude that widespread cortical hyperexcitability that includes the occipital area is necessary to produce the autonomic seizure manifestations seen in PS. (C) 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2014.01.013

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  • A Storm of Fast (40-150Hz) Oscillations during Hypsarrhythmia in West Syndrome

    Katsuhiro Kobayashi, Tomoyuki Akiyama, Makio Oka, Fumika Endoh, Harumi Yoshinaga

    ANNALS OF NEUROLOGY   77 ( 1 )   58 - 67   2015年1月

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    記述言語:英語   出版者・発行元:WILEY-BLACKWELL  

    ObjectiveFast oscillations (FOs) were first explored from scalp electroencephalographic (EEG) data from hypsarrhythmia in West syndrome (infantile spasms) to investigate the meaning of FOs in this epileptic encephalopathy.
    MethodsIn 17 infants with West syndrome, we conservatively detected fast frequency peaks that stood out from the time-frequency spectral background with square root power>1 mu V (spectral criterion) and corresponded to clear FOs with at least 4 oscillations in the filtered EEG traces (waveform criterion) in sleep EEGs.
    ResultsWe found a total of 1,519 interictal FOs that fulfilled both the spectral and waveform criteria. The FOs with a median frequency of 56.6Hz (range=41.0-140.6Hz) were dense, with a median rate of 66 (range=24-171) per minute before adrenocorticotropic hormone (ACTH) treatment, which was significantly higher than that in control infants without seizures (median=1, p<0.001). The FOs were reduced by treatment. The mean gamma and ripple oscillation rates that were detected using the waveform criterion alone were 40.62/min and 15.75/min, respectively, per channel; these results were 112.8 and 98.4 times higher, respectively, than the previously reported corresponding rates in adult epilepsy patients.
    InterpretationThe observed FOs corresponded to epileptogenicity because of their close relation to the severity of hypsarrhythmia during the course of ACTH treatment. The very high epileptic FO rates in hypsarrhythmia are thought to affect the process of neurodevelopment by interfering with physiological functions in West syndrome, taking into account that high frequencies are also important in physiological higher brain functions. ANN NEUROL 2015;77:58-67

    DOI: 10.1002/ana.24299

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  • 頭蓋内脳波における高周波解析を基に切除計画を立てた難治性てんかんの1手術例

    近藤 聡彦, 吉永 治美, 伊達 勲, 秋山 倫之, 上利 崇, 岡 牧郎, 小林 由典, 林 裕美子, 柴田 敬, 新治 有径, 小林 勝弘

    脳外誌   24 ( 1 )   32 - 39   2015年1月

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    記述言語:日本語   出版者・発行元:日本脳神経外科コングレス  

     難治性新皮質てんかんの外科治療の際, 発作焦点の同定のために頭蓋内脳波記録は有用であるが, 脳波の解析法や結果の解釈の相違により切除範囲や手術成績に影響が生じ得る. 今回, われわれは難治性の後頭葉てんかん患者に対して, 慢性頭蓋内電極を留置し, 高周波律動 (HFO) の解析を行った. 独自のプログラムを用いてHFOの脳表カラーマップを作成し, マップ上のHFOの出現部位を参考に切除範囲を決定して, 焦点切除術を行った. 術後1年経過した現在, 発作は完全に抑制されている. 脳表カラーマップは発作時HFOの動的変化の視覚化に有用であり, この結果に基づいてHFO検出部位を切除範囲に含むことが良好な術後成績に寄与するものと考えられた.

    DOI: 10.7887/jcns.24.32

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  • 小児の髄液中vitamin B6、ピリドキサールリン酸の基準値確立の試み

    秋山 倫之, 小林 勝弘, 遠藤 文香, 岡 牧郎, 吉永 治美

    脳と発達   47 ( 1 )   60 - 60   2015年1月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 乳幼児重症てんかんの診断における脳循環・代謝検査の有用性―岡山大学病院てんかんセンターの半球離断術の症例を中心として―

    小林勝弘, 上利 崇, 佐々田晋, 秋山倫之, 岡牧郎, 遠藤文香, 吉永治美, 伊達 勲

    脳循環代謝   26 ( 2 )   163 - 168   2015年

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  • Dravet 症候群―乳児重症ミオクロニーてんかん―

    秋山麻里, 小林勝弘

    小児内科   47 ( 9 )   1571 - 1574   2015年

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  • てんかんの神経生理学 -数式を使わない脳波分析の基本-

    小林勝弘

    てんかん研究   33 ( 1 )   140 - 143   2015年

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  • 脳波解析の新しい知見

    小林勝弘, 花岡義行

    小児科診療   78 ( 2 )   259 - 264   2015年

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  • Novel PLA2G6 mutations associated with an exonic deletion derived from non-allelic homologous recombination in a patient with infantile neuroaxonal dystrophy 国際誌

    Yamamoto T, Shimojima K, Shibata T, Akiyama M, Oka M, Akiyama T, Yoshinaga H, Kobayashi K

    Hum Genome Var   2   15048 - 15048   2015年

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    記述言語:英語  

    DOI: 10.1038/hgv.2015.48

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  • 小児のてんかんと脳波異常

    小林勝弘

    小児の脳神経   40 ( 5 )   350 - 360   2015年

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  • Five pediatric cases of ictal fear with variable outcomes

    Mari Akiyama, Katsuhiro Kobayashi, Takushi Inoue, Tomoyuki Akiyama, Harumi Yoshinaga

    BRAIN & DEVELOPMENT   36 ( 9 )   758 - 763   2014年10月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Purpose: Ictal fear is an uncommon condition in which fear manifests as the main feature of epileptic seizures. The literature has suggested that ictal fear is generally associated with poor seizure outcomes. We wanted to clarify the variability in seizure outcome of children with ictal fear. Subjects and methods: We identified five pediatric patients with ictal fear who were followed up on at Okayama University Hospital between January 2003 and December 2012. We retrospectively reviewed their clinical records and EEG findings. Results: The onset age of epilepsy ranged from 8 months to 9 years and 10 months. The common ictal symptoms were sudden fright, clinging to someone nearby, and subsequent impairment of consciousness, which were often accompanied by complex visual hallucinations and psychosis-like complaints. Ictal fear, in four patients, was perceived as a nonepileptic disorder by their parents. Ictal electroencephalograms (EEG) of ictal fear were obtained in all patients. Three showed frontal onset, while the other two showed centrotemporal or occipital onsets. Two patients were seizure free at last follow-up, while seizures persisted in the other three. A patient with seizure onset during infancy had a favorable outcome, which was considered to be compatible with benign partial epilepsy with affective symptoms. Conclusion: Ictal fear is not always associated with a symptomatic cause or a poor seizure outcome. It is quite important to make a correct diagnosis of ictal fear as early as possible to optimize treatment. (C) 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2013.11.011

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  • 小児良性部分てんかんの脳波における高周波振動と棘波の分布に関する検討

    柴田 敬, 小林 勝弘, 井上 拓志, 秋山 倫之, 吉永 治美

    臨床神経生理学   42 ( 5 )   285 - 285   2014年10月

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    記述言語:日本語   出版者・発行元:(一社)日本臨床神経生理学会  

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  • Dravet症候群におけるStiripentolの初期および長期効果の検討

    秋山 麻里, 小林 勝弘, 岡 牧郎, 秋山 倫之, 吉永 治美

    てんかん研究   32 ( 2 )   391 - 391   2014年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • Naチャネル異常による筋疾患の臨床多様性

    柴田 敬, 吉永 治美, 秋山 倫之, 岡 牧郎, 小林 勝弘, 村上 暢子, 迫田 俊一

    脳と発達   46 ( Suppl. )   S244 - S244   2014年5月

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    記述言語:日本語   出版者・発行元:(一社)日本小児神経学会  

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  • 緊急少数電極脳波の振幅・周波数統合トレンド表示は脳波非習熟者における判読精度を向上させるか?

    小林 勝弘, 柚木 宏介, 禅正 和真, 秋山 倫之, 吉永 治美

    脳と発達   46 ( Suppl. )   S312 - S312   2014年5月

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  • CSF/plasma ratios of amino acids: Reference data and transports in children

    Tomoyuki Akiyama, Katsuhiro Kobayashi, Akihito Higashikage, Junko Sato, Harumi Yoshinaga

    BRAIN & DEVELOPMENT   36 ( 1 )   3 - 9   2014年1月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: We intended to investigate the effects of age, gender, and medications on amino acid cerebrospinal fluid (CSF)/plasma ratios in children, and to determine whether amino acid transports across the blood CSF barrier in children differ from those in adults. Patients and methods: Amino acid concentrations measured by ion-exchange high-performance liquid chromatography were used (CSF from 99 children, simultaneously collected plasma from 76 children). Influence of age, gender, and medications on the amino acid CSF concentrations and CSF/plasma ratios were analyzed by linear multiple regression. Interactions of amino acid transports were analyzed by correlation analysis of CSF/plasma ratios. Results: CSF/plasma ratios of serine, valine, histidine, and arginine were higher in younger children. The glutamate CSF/plasma ratio was higher in older children. Serine, alanine, threonine, valine, and histidine CSF/plasma ratios were lower in females. Glutamine, methionine, tyrosine, and phenylalanine CSF/plasma ratios were elevated with valproate therapy. Serine, threonine, valine, leucine, and tyrosine CSF/plasma ratios were lower with clobazam therapy. The asparagine CSF/plasma ratio was elevated with pyridoxal phosphate therapy. Transports of most essential neutral amino acids interacted with each other, as did neutral amino acids with low molecular weights. Cationic amino acids interacted with each other and some essential neutral amino acids. Acidic amino acids had no interactions with other amino acids. Conclusions: Age, gender, and anti-epileptic drugs affect amino acid CSF/plasma ratios in children. Transport interactions between amino acids in children showed no remarkable difference from those of adults and generally followed the substrate specificities of multiple amino acid transport systems. (C) 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

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  • 難治小児てんかんの電気生―特に異常高周波活動と病態との関係について

    小林勝弘, 東田好広

    脳と発達   2014年

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  • 大田原症候群

    小林勝弘

    日本臨床 別冊 新領域別症候群シリーズ「神経症候群(第2版)―その他の神経疾患を含めて―」   2014年

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  • 微細な epileptic negative myoclonus を認めた West症候群の1例

    柴田敬, 吉永治美, 岡牧郎, 小林勝弘

    脳と発達   46 ( 5 )   354 - 357   2014年

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  • Panayiotopoulos 症候群における臨床-脳波相関の検討

    吉永治美, 柴田敬, 小林勝弘

    てんかん治療研究振興財団研究   25   55 - 60   2014年

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  • 意識障害を主症状とするてんかん

    小林勝弘

    小児内科   2014年

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  • A Japanese adult case of guanidinoacetate methyltransferase deficiency

    Akiyama T, Osaka H, Shimbo H, Nakajiri T, Kobayashi K, Oka M, Endoh F, Yoshinaga H

    JIMD Rep   12   65 - 69   2014年

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  • Cortical contribution to scalp EEG gamma rhythms associated with epileptic spasms

    Katsuhiro Kobayashi, Kazushi Miya, Tomoyuki Akiyama, Fumika Endoh, Makio Oka, Harumi Yoshinaga, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   35 ( 8 )   762 - 770   2013年9月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    The cortical contribution for the generation of gamma rhythms detected from scalp ictal EEG was studied in unique cases of epileptic spasms and a review of the related literature was conducted. Ictal scalp gamma rhythms were investigated through time frequency analysis in two cases with a combination of focal seizures and spasms and another case with spasms associated with cortical dysplasia. In the two patients with combined seizures, the scalp distribution of ictal gamma rhythms was related to that of focal seizure activity. In the third patient, an asymmetric distribution of the ictal scalp gamma rhythms was transiently revealed in correspondence to the dysplasic cortex during hormonal treatment. Therefore, the dominant region of scalp gamma rhythms may correspond to the epileptogenic cortical area. The current findings have reinforced the possibility of the cortical generation of ictal scalp gamma rhythms associated with spasms The detection of high frequencies through scalp EEG is a technical challenge, however, and the clinical significance of scalp gamma rhythms may not be the same as that of invasively recorded high frequencies. Further studies on the pathophysiological mechanisms related to the generation of spasms involving high frequencies are necessary in the future, and the development of animal models of spasms will play an important role in this regard. (C) 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2012.12.012

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  • Successful treatment of early myoclonic encephalopathy using lidocaine and carbamazepine

    Kousuke Nakano, Katsuhiro Kobayashi, Satoshi Maniwa, Nobuyuki Kodani, Yoko Ohtsuka

    EPILEPTIC DISORDERS   15 ( 3 )   352 - 357   2013年9月

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    記述言語:英語   出版者・発行元:JOHN LIBBEY EUROTEXT LTD  

    We report two female infants with early myoclonic encephalopathy (EME) whose intractable focal seizures were suppressed with lidocaine and carbamazepine (CBZ). Although EME is a form of early-onset epileptic encephalopathy characterised by myoclonus and focal seizures that are highly resistant to treatment, lidocaine and CBZ may prove effective in treating this disorder. Future studies should be performed in order to determine whether there are common specific mechanisms of seizure generation related to the sodium channel in these patients.

    DOI: 10.1684/epd.2013.0593

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  • バルプロ酸にトピラマートを追加した場合、血中アンモニア濃度は上昇するか?

    秋山 麻里, 秋山 倫之, 小林 勝弘, 吉永 治美

    てんかん研究   31 ( 2 )   432 - 432   2013年9月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • Effects of intravenous diazepam on high-frequency oscillations in EEGs with CSWS

    Yoshihiro Toda, Katsuhiro Kobayashi, Yumiko Hayashi, Takushi Inoue, Makio Oka, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   35 ( 6 )   540 - 547   2013年6月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    High-frequency oscillations (HFOs) associated with continuous spike-waves during slow-wave sleep (CSWS) are speculated to be linked to the disturbance of higher brain function. We intended to investigate the generative mechanisms of HFOs in CSWS by clarifying the effects of intravenous injection (IV) of diazepam (DZP), an agonist for the gamma-aminobutyric acid A (GABA(A)) receptor in the GABAergic interneuron system, in patients who had previously been treated with IV DZP. The subjects were three patients with epilepsy with CSWS. For each patient, EEG data before and after IV DZP were separated into consecutive 5-min sections. Time frequency power spectral analysis was performed on the spikes of each section, and peak-power and frequency of detected high-frequency spectral spots were compared before and after IV DZP. Spectral spots with peak-frequencies at 85.9-121.1 Hz in the ripple band were revealed in all three patients. Although the amplitudes of the spikes largely returned to the baseline levels 20-25 min after IV DZP, the recovery of the peak-power levels of HFOs lagged behind that of the spike amplitudes, and the power levels of HFOs were lower than the baseline data within 25 min after the injection of DZP. No consistent changes were found regarding the spectral frequencies of HFOs. The dissociation of the effect of IV DZP in terms of recovery when comparing spike-amplitudes and the power of HFOs may correspond to an already suggested difference in the pathophysiological mechanisms that generate the spikes and HFOs. (C) 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2012.09.002

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  • 小児期に恐怖発作を呈した5症例

    秋山 麻里, 井上 拓志, 小林 勝弘, 吉永 治美

    てんかん研究   31 ( 1 )   89 - 89   2013年6月

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    記述言語:日本語   出版者・発行元:(一社)日本てんかん学会  

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  • Questionnaire-based assessment of behavioral problems in Japanese children with epilepsy

    Katsuhiro Kobayashi, Fumika Endoh, Tatsuya Ogino, Makio Oka, Teruko Morooka, Harumi Yoshinaga, Yoko Ohtsuka

    EPILEPSY & BEHAVIOR   27 ( 1 )   238 - 242   2013年4月

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    記述言語:英語   出版者・発行元:ACADEMIC PRESS INC ELSEVIER SCIENCE  

    Behavioral problems in Japanese children with epilepsy were investigated by means of a questionnaire for parents consisting of three checklists: the Child Behavior Checklist (CBCL)/4-18 Japanese Edition, the High-Functioning Autism Spectrum Screening Questionnaire (ASSQ), and the Attention-Deficit/Hyperactivity Disorder (ADHD) Rating Scale-IV (ADHD-RS) for parents. The participants were the parents of 108 children aged 6-18 years with apparently normal intelligence. The CBCL indicated abnormal behavior in 10.5 to 35.6% of the children, and T scores on both the internalizing and externalizing scales had a significant positive relation with scores on the ASSQ and ADHD-RS. It was revealed through multivariate logistic regression analysis that the persistence of seizures was significantly related with abnormality on the externalizing scale of the CBCL (p = 0.010, odds ratio: 3.48, 95% confidence interval: 1.34-9.02). Future studies are needed to determine whether seizure freedom improves behavior in children with epilepsy. (C) 2013 Elsevier Inc. All rights reserved.

    DOI: 10.1016/j.yebeh.2013.01.020

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  • Clinical implications of preceding positive spikes in patients with benign partial epilepsy and febrile seizures

    Harumi Yoshinaga, Katsuhiro Kobayashi, Tomoyuki Akiyama, Takashi Shibata, Fumika Endoh, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   35 ( 4 )   299 - 306   2013年4月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Purpose: To clarify the clinical implications of the preceding positive spikes (PPSs) observed primarily in rolandic spikes, we analyzed PPSs in the rolandic and occipital spikes observed in the electroencephalograms (EEGs) of patients with two types of benign partial epilepsies (benign childhood epilepsy with centro-temporal spikes [BECT] and Panayiotopoulos syndrome [PS]) and febrile seizures (FS). Subjects and methods: We identified patients from our outpatient EEG database that were seen between 2006 and 2008 that had BECT, PS, and FS with rolandic or occipital spikes. We generated an averaged spike for each patient from the rolandic and occipital spikes that were detected using an automatic spike detection and clustering system. We compared the presence rate of the averaged spikes with the PPS among the three groups (BECT vs. PS vs. FS) using sequential mapping. Results: We identified 25 BECT, 18 PS, and 15 FS patients with rolandic spikes. Fifteen BECT and nine PS patients exhibited a PPS in their averaged rolandic spikes, whereas only four FS patients did. Three of these four FS patients later developed afebrile seizures, and one of them was diagnosed as having PS. We analyzed eight PS and six FS patients with occipital spikes. Five PS patients exhibited a PPS in their averaged occipital spikes, whereas only one FS patient did. This FS patient later developed prolonged autonomic febrile seizures. Conclusion: PPSs are observed not only in rolandic spikes associated with BECT that is related strictly to sylvian seizures, but also in rolandic and occipital spikes associated with PS. Although PPSs are rare in such spikes observed in FS, patients with FS and PPSs may have an increased risk of developing afebrile seizures or prolonged autonomic febrile seizures. Further studies are warranted to determine the diagnostic utility of PPSs as a marker of the future development of epilepsy when they are observed in FS patients. (C) 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2012.06.006

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  • High-frequency EEG oscillations in hyperthermia-induced seizures of Scn1a mutant rats

    Katsuhiro Kobayashi, Iori Ohmori, Keiichiro Hayashi, Yuichiro Kitagawa, Mamoru Ouchida, Takushi Inoue, Yoko Ohtsuka

    EPILEPSY RESEARCH   103 ( 2-3 )   161 - 166   2013年2月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    We examined high-frequency oscillations (HFOs) in the ictal cortical EEGs of hyperthermia-induced seizures in a rat model of febrile seizures with an SCN1A mutation as a means of investigating the pathophysiological mechanisms underlying the generation of febrile seizures. We used 13 male homozygous Scn1a-N1417H mutant rats (F344/NSlc-Scn1a(Kyo811)) and 10 wild-type control rats.
    Generalized tonic-clonic seizures were induced in all mutant rats, and HFOs with frequencies ranging from 200 to 400 Hz were found to precede spikes during the clonic phases of these seizures in the ictal EEGs. The proportion of all spikes in each seizure that were associated with HFOs increased with age. In time-frequency spectra of the EEG data, the HFOs had a mean peak frequency of 301.1 +/- 65.4 Hz (range: 156.3-468.8 Hz) and a mean peak power of 24.6 +/- 3.8 dB (range: 11.4-33.4 dB); the peak power increased with age. Regarding the wild-type rats, a brief seizure without unmistakable HFOs was exceptionally induced in only one rat.
    The generation mechanism of febrile seizures is still an unanswered question. The detection of HFOs from the ictal EEGs of hyperthermia-induced seizures may provide a cue to answering this open question, although in this research we were unable to provide sufficient evidence to prove that the generation of HFOs depended on the mutation. (C) 2012 Elsevier B.V. All rights reserved.

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  • CACNA1A variants may modify the epileptic phenotype of Dravet syndrome

    Iori Ohmori, Mamoru Ouchida, Katsuhiro Kobayashi, Yoshimi Jitsumori, Akiko Mori, Hiroyuki Michiue, Teiichi Nishiki, Yoko Ohtsuka, Hideki Matsui

    NEUROBIOLOGY OF DISEASE   50 ( 2 )   209 - 217   2013年2月

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    記述言語:英語   出版者・発行元:ACADEMIC PRESS INC ELSEVIER SCIENCE  

    Dravet syndrome is an intractable epileptic syndrome beginning in the first year of life. De novo mutations of SCN1A, which encode the Na(v)1.1 neuronal voltage-gated sodium channel, are considered the major cause of Dravet syndrome. In this study, we investigated genetic modifiers of this syndrome.
    We performed a mutational analysis of all coding exons of CACNA1A in 48 subjects with Dravet syndrome. To assess the effects of CACNA1A variants on the epileptic phenotypes of Dravet syndrome, we compared clinical features in two genotype groups: 1) subjects harboring SCN1A mutations but no CACNA1A variants (n = 20) and 2) subjects with SCN1A mutations plus CACNA1A variants (n = 20). CACNA1A variants detected in patients were studied using heterologous expression of recombinant human Ca(v)2.1 in HEK 293 cells and whole-cell patch-clamp recording.
    Nine CACNA1A variants, including six novel ones, were detected in 21 of the 48 subjects (43.8%). Based on the incidence of variants in healthy controls, most of the variants seemed to be common polymorphisms. However, the subjects harboring SCN1A mutations and CACNA1A variants had absence seizures more frequently than the patients with only SCN1A mutations (8/20 vs. 0/20, p = 0.002). Moreover, the former group of subjects exhibited earlier onset of seizures and more frequent prolonged seizures before one year of age, compared to the latter group of subjects. The electrophysiological properties of four of the five novel Ca(v)2.1 variants exhibited biophysical changes consistent with gain-of-function. We conclude that CACNA1A variants in some persons with Dravet syndrome may modify the epileptic phenotypes. (C) 2012 Elsevier Inc. All rights reserved.

    DOI: 10.1016/j.nbd.2012.10.016

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  • 乳児重症ミオクロニーてんかん

    小林勝弘

    救急・集中治療   25 ( 11・12 )   1425 - 1430   2013年

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  • Successful Treatment of Epilepsy by Resection of Periventricular Nodular Heterotopia

    Takashi Agari, Tadahiro Mihara, Koichi Baba, Katsuhiro Kobayashi, Naotaka Usui, Kiyohito Terada, Fumihiro Nakamura, Kazumi Matsuda, Isao Date

    ACTA MEDICA OKAYAMA   66 ( 6 )   487 - 492   2012年12月

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    記述言語:英語   出版者・発行元:OKAYAMA UNIV MED SCHOOL  

    We report on a case of successful surgical treatment of drug-resistant epilepsy associated with a solitary lesion of periventricular nodular heterotopia (PNH). In the reported patient, intracranial ictal electroencephalography disclosed that seizures did not originate from the heterotopic nodules. However, the seizures were completely suppressed by lesionectomy of PNH alone. Epileptogenesis associated with PM-I likely involves a very complex network between PNH and the surrounding cortex, and the disruption of this network may be an effective means of curing intractable, PNH-associated epilepsy.

    DOI: 10.18926/AMO/49045

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  • Acute encephalopathy with a novel point mutation in the SCN2A gene

    Katsuhiro Kobayashi, Hiroki Ohzono, Mayu Shinohara, Makiko Saitoh, Iori Ohmori, Yoko Ohtsuka, Masashi Mizuguchi

    EPILEPSY RESEARCH   102 ( 1-2 )   109 - 112   2012年11月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Mutations of the neuronal voltage-gated sodium channel alpha subunit type II (SCN2A) cause various epileptic syndromes, but have never been reported in association with acute encephalopathy. To validate the involvement of SCN2A mutations in acute encephalopathy, we screened 25 patients and found a novel missense mutation (Met1128Thr) in a patient With acute encephalitis with refractory, repetitive partial seizures (AERRPS). This finding suggests that SCN2A mutation is a predisposing factor for acute encephalopathy. (C) 2012 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2012.04.016

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  • Dravet Syndrome: A Genetic Epileptic Disorder

    Mari Akiyama, Katsuhiro Kobayashi, Yoko Ohtsuka

    ACTA MEDICA OKAYAMA   66 ( 5 )   369 - 376   2012年10月

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    記述言語:英語   掲載種別:書評論文,書評,文献紹介等   出版者・発行元:OKAYAMA UNIV MED SCHOOL  

    Dravet syndrome (DS), or severe myoclonic epilepsy in infancy, is one of the most severe types of genetic epilepsy. It is characterized by the initial occurrence of febrile or afebrile seizures that often evolve into status epilepticus in infants with normal development, and by the subsequent appearance of myoclonic and/or atypical absence seizures as well as complex partial seizures. The key feature that characterizes DS is fever sensitivity, although photosensitivity and pattern-sensitivity are also often seen. The prognosis is unfavorable in most cases. Seizures become drug-resistant and persist, with many patients suffering from motor and cognitive impairment. Mutations of SCN1A, which encodes the voltage-gated sodium channel Na(v)1.1, are the most frequent genetic cause of this syndrome. SCN1A mutations and/or microchromosomal rearrangements involving SCN1A are detected in about 85% of patients. Mutations of PCDH19 have also been reported in female patients with clinical findings compatible with DS. PCDH19 mutations might account for 5% of overall DS cases. Thirty years after its first description, DS is considered as a model of channelopathy. This survey reviews recent developments in the research literature on DS, focusing on the clinical course, as well as its genetic causes.

    DOI: 10.18926/AMO/48961

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  • Efficacy of topiramate for intractable childhood generalized epilepsy with epileptic spasms: With special reference to electroencephalographic changes

    Fumika Endoh, Katsuhiro Kobayashi, Yumiko Hayashi, Takashi Shibata, Harumi Yoshinaga, Yoko Ohtsuka

    SEIZURE-EUROPEAN JOURNAL OF EPILEPSY   21 ( 7 )   522 - 528   2012年9月

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    記述言語:英語   出版者・発行元:W B SAUNDERS CO LTD  

    Purpose: Epileptic spasms (ES) beyond infancy are a highly refractory type of seizures that require the development of an effective treatment. We therefore studied the efficacy and safety of topiramate (TPM), which is a drug that is indicated to be effective for intractable childhood epilepsy, for ES.
    Methods: Out of 58 children with ES, we enrolled 33 patients treated with TPM at <= 12 years of age. The administration of TPM was limited to cases of epilepsies that were resistant to any other potent treatment. We retrospectively investigated the efficacy of TPM for seizures and changes in electroencephalogram (EEG) findings.
    Results: The median age at the start of TPM treatment was 5 years, 8 months. All patients had ES and 28 also had tonic seizures. As for the efficacy of TPM for all seizures, five patients became seizure-free and two had a >= 50% reduction in seizures. Seizure aggravation was observed in six patients. Of 29 patients whose EEG findings were compared before and during TPM treatment, nine showed EEG improvement with reduced epileptic discharges. Adverse effects were observed in 13 patients and included somnolence, anorexia, and irritability. In general, TPM was well tolerated.
    Conclusions: TPM can be effective at suppressing very intractable ES in a proportion of patients who do not respond to any other treatment. The efficacy of TPM may be predictable based on EEG changes observed early in the course of treatment. TPM is promising for the treatment of extremely intractable childhood epilepsy and it has largely tolerable adverse effects. (c) 2012 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

    DOI: 10.1016/j.seizure.2012.05.009

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  • Cryptogenic West syndrome and subsequent mesial temporal lobe epilepsy

    Takashi Agari, Katsuhiro Kobayashi, Kiyoko Watanabe, Isao Date, Yoko Ohtsuka

    EPILEPTIC DISORDERS   14 ( 3 )   334 - 339   2012年9月

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    記述言語:英語   出版者・発行元:JOHN LIBBEY EUROTEXT LTD  

    We report on a male patient who experienced a previously unreported sequence of cryptogenic West syndrome in infancy and subsequent mesial temporal lobe epilepsy. His complex partial seizures were consistently characterised by motionless staring with brief right eye blinking. Scalp electroencephalography (EEG) showed bilateral temporal spikes which were dominant on the right side. Magnetic resonance imaging (MRI) revealed no organic brain lesion. Invasive EEG recording captured seizures with right hippocampal onset. The patient became seizure-free following right temporal lobectomy at 27 years, 8 months of age. Pathological examination of the resected specimen revealed corpora amylacea and gliosis in the temporal cortex but no clear findings of hippocampal sclerosis. It is suggested that an epileptogenic lesion causing MRI-negative mesial temporal lobe epilepsy may give rise to apparent cryptogenic West syndrome in infancy.

    DOI: 10.1684/epd.2012.0518

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  • Intractable Absence Seizures in Hyperinsulinism-Hyperammonemia Syndrome

    Kousuke Nakano, Katsuhiro Kobayashi, Yoshiyuki Okano, Kazuyoshi Aso, Yoko Ohtsuka

    PEDIATRIC NEUROLOGY   47 ( 2 )   119 - 122   2012年8月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE INC  

    A girl with intractable absence seizures and facial myoclonia at age 7 years was eventually diagnosed with hyperinsulinism-hyperammonemia syndrome because of hypoglycemia, hyperinsulinism, hyperammonemia, and the results of an oral L-leucine loading test. Her seizures occurred even during periods of relatively normal blood glucose levels, and were completely suppressed by diazoxide treatment only. Her diagnosis of hyperinsulinism-hyperammonemia syndrome was confirmed by a loss of sensitivity of glutamate dehydrogenase for guanosine 5'-triphosphate. Genetic studies identified the I444M mutation in the GLUD1 gene, which encodes glutamate dehydrogenase. This case illustrates the complex relationship between seizures and hypoglycemia in hyperinsulinism-hyperammonemia syndrome that can create diagnostic difficulties. The possibility of hyperinsulinism-hyperammonemia syndrome should be considered in patients with refractory absence seizures with myoclonia. (C) 2012 Elsevier Inc. All rights reserved.

    DOI: 10.1016/j.pediatrneurol.2012.04.019

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  • Memory Enhancement and Deep-Brain Stimulation of the Entorhinal Area

    Katsuhiro Kobayashi, Harumi Yoshinaga, Yoko Ohtsuka

    NEW ENGLAND JOURNAL OF MEDICINE   366 ( 20 )   1945 - 1945   2012年5月

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    記述言語:英語   掲載種別:速報,短報,研究ノート等(学術雑誌)   出版者・発行元:MASSACHUSETTS MEDICAL SOC  

    DOI: 10.1056/NEJMc1203204

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  • Amelioration of disabling myoclonus in a case of DRPLA by levetiracetam

    Katsuhiro Kobayashi, Akihito Takeuchi, Makio Oka, Mari Akiyama, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   34 ( 5 )   368 - 371   2012年5月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    We report on an 18-year-old male patient with dentatorubral-pallidoluysian atrophy (DRPLA) (number of CAG repeats: 68) with progressive myoclonus epilepsy (PME), who showed a dramatic response to levetiracetam in terms of the intensity of myoclonus. He began to have convulsive seizures and myoclonus at 7 and 10 years of age, respectively, and his intelligence deteriorated from 12 years of age. EEG showed multifocal and diffuse spike-and-wave complexes. His convulsive seizures were suppressed from 13 years of age.
    At 17 years of age, the patient showed gradual intensification of erratic segmental positive myoclonus as well as frequent atonic falls that were probably attributable to negative myoclonus. Back averaging of EEG data revealed cortical discharges associated with positive myoclonus. Photosensitive myoclonic seizures were also observed. The administration of levetiracetam alleviated positive myoclonus and suppressed atonic falls, resulting in a remarkable improvement in the patient's quality of daily life.
    Reports on the efficacy of levetiracetam for myoclonus in DRPLA are still rare, though its effect on PM E is known in the context of other neurological disorders. Thus levetiracetam should be subjected to clinical trials as a means of disabling myoclonus in DRPLA. (C) 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

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  • A novel mutation in SCN4A causes severe myotonia and school-age-onset paralytic episodes

    Harumi Yoshinaga, Shunichi Sakoda, Jean-Marc Good, Masanori P. Takahashi, Tomoya Kubota, Eri Arikawa-Hirasawa, Tomohiko Nakata, Kinji Ohno, Tetsuro Kitamura, Katsuhiro Kobayashi, Yoko Ohtsuka

    JOURNAL OF THE NEUROLOGICAL SCIENCES   315 ( 1-2 )   15 - 19   2012年4月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Mutations in the pore-forming subunit of the skeletal muscle sodium channel (SCN4A) are responsible for hyperkalemic periodic paralysis, paramyotonia congenita and sodium channel myotonia. These disorders are classified based on their cardinal symptoms, myotonia and/or paralysis. We report the case of a Japanese boy with a novel mutation of SCN4A, p.I693L, who exhibited severe episodic myotonia from infancy and later onset mild paralytic attack. He started to have apneic episodes with generalized hypertonia at age of 11 months, then developed severe episodic myotonia since 2 years of age. He presented characteristic generalized features which resembled Schwarz-Jampel syndrome. After 7 years old, paralytic episodes occurred several times a year. The compound muscle action potential did not change during short and long exercise tests. Functional analysis of the mutant channel expressed in cultured cell revealed enhancement of the activation and disruption of the slow inactivation, which were consistent with myotonia and paralytic attack. The severe clinical features in his infancy may correspond to myotonia permanence, however, he subsequently experienced paralytic attacks. This case provides an example of the complexity and overlap of the clinical features of sodium channel myotonic disorders. (c) 2011 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.jns.2011.12.015

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  • Additional information regarding "Dravet syndrome: Inroads into understanding epileptic encephalopathies"

    Katsuhiro Kobayashi, Yoko Ohtsuka, Iori Ohmori

    JOURNAL OF PEDIATRICS   160 ( 3 )   532 - 533   2012年3月

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    記述言語:英語   掲載種別:速報,短報,研究ノート等(学術雑誌)   出版者・発行元:MOSBY-ELSEVIER  

    DOI: 10.1016/j.jpeds.2011.11.058

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  • 早産児にみられる小脳障害に伴う特異な不随意運動に関する検討.

    吉永治美, 小林勝弘, 遠藤文香, 石崎裕美子, 柴田敬, 大塚頌子

    脳と発達   44 ( 3 )   239 - 243   2012年

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  • Q&A23 抗てんかん薬はいつ開始すべきで、いつまで飲み続けるのでしょうか?また、てんかんが治ることはあるのでしょうか?

    小林勝弘

    治療   94 ( 10 )   1764 - 1765   2012年

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  • 最近提唱されている新しいてんかん病型.

    秋山倫之, 小林勝弘

    小児内科   44 ( 9 )   1461 - 1465   2012年

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  • 目でみるてんかん:脳波の高周波振動と小児良性部分てんかん.

    小林勝弘

    Epilepsy   6 ( 1 )   4 - 6   2012年

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  • 脳波における高周波てんかん性異常の時間・周波数分析.

    小林勝弘, 上利崇

    てんかん治療研究振興財団研究年報   23   57 - 66   2012年

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  • Drug-Resistant Epilepsy

    Katsuhiro Kobayashi, Harumi Yoshinaga, Yoko Ohtsuka

    NEW ENGLAND JOURNAL OF MEDICINE   365 ( 23 )   2238 - 2239   2011年12月

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    記述言語:英語   掲載種別:速報,短報,研究ノート等(学術雑誌)   出版者・発行元:MASSACHUSETTS MEDICAL SOC  

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  • Amplitude-integrated EEG colored according to spectral edge frequency

    Katsuhiro Kobayashi, Nobuyoshi Mimaki, Fumika Endoh, Takushi Inoue, Harumi Yoshinaga, Yoko Ohtsuka

    EPILEPSY RESEARCH   96 ( 3 )   276 - 282   2011年10月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objective: To improve the interpretability of figures containing an amplitude-integrated electroencephalogram (aEEG), we devised a color scale that allows us to incorporate spectral edge frequency (SEF) information into aEEG figures. Preliminary clinical assessment of this novel technique, which we call aEEG/SEF, was performed using neonatal and early infantile seizure data.
    Methods: We created aEEG, color density spectral array (DSA), and aEEG/SEF figures for focal seizures recorded in seven infants. Each seizure was paired with an interictal period from the same patient. After receiving instructions on how to interpret the figures, eight test reviewers examined each of the 72 figures displaying compressed data in aEEG, DSA, or aEEG/SEF form (12 seizures and 12 corresponding interictal periods) and attempted to identify each as a seizure or otherwise. They were not provided with any information regarding the original record.
    Results: The median number of correctly identified seizures, out of a total of 12, was 7 (58.3%) for aEEG figures, 8 (66.7%) for DSA figures and 10 (83.3%) for aEEG/SEF figures; the differences among these are statistically significant (p=0.011). All reviewers concluded that aEEG/SEF figures were the easiest to interpret.
    Conclusion: The aEEG/SEF data presentation technique is a valid option in aEEG recordings of seizures. (C) 2011 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2011.06.012

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  • High-frequency oscillations in idiopathic partial epilepsy of childhood

    Katsuhiro Kobayashi, Harumi Yoshinaga, Yoshihiro Toda, Takushi Inoue, Makio Oka, Yoko Ohtsuka

    EPILEPSIA   52 ( 10 )   1812 - 1819   2011年10月

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    記述言語:英語   出版者・発行元:WILEY-BLACKWELL  

    Purpose: We explored high-frequency oscillations (HFOs) in scalp sleep electroencephalography (EEG) studies of patients with idiopathic partial epilepsy (IPE) of childhood in order to obtain a better understanding of the pathologic mechanisms underlying IPE.
    Methods: The subjects were 45 patients, including 32 with benign childhood epilepsy with centrotemporal spikes (BCECTS) and 13 with Panayiotopoulos syndrome (PS). A total of 136 EEG records were investigated through temporal expansion and filtering of traces and time-frequency spectral analysis.
    Key Findings: HFOs with frequency of 93.8-152.3 Hz (mean 126.2 +/- 13.6 Hz) in the band of ripples were detected in association with spikes in 97 records (71.3%). Time from last seizure to the EEG recording was significantly shorter in those with spike-related HFOs than in the EEG recordings with spikes without HFOs (p = 0.006). Although time from last seizure reflects age, age at the time of recording was not significantly different between EEG studies with and without HFOs. Peak-power values of the high-frequency spots in time-frequency spectra were significantly negatively correlated with time from last seizure (R(2) = 0.122, p < 0.001) but not with age at the time of recording. Peak frequencies of the high-frequency spectral spots were not significantly correlated with age at the time of recording or with time from last seizure.
    Significance: The close relationship between the generation of spike-related HFOs and the period of active seizure occurrence indicated that HFOs may tell us more about epileptogenicity in IPE than the spikes themselves. Because there is a spectrum of pediatric epileptic disorders extending from the benign end of BCECTS to the encephalopathic end of epilepsy with continuous spike-waves during slow-wave sleep (CSWS), and HFOs that have already been detected in association with CSWS were more prominent than HFOs in IPE, intense spike-related HFOs may indicate poor prognosis.

    DOI: 10.1111/j.1528-1167.2011.03169.x

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  • An epidemiological study of children with status epilepticus in Okayama, Japan: Incidence, etiologies, and outcomes

    Itsuko Nishiyama, Yoko Ohtsuka, Toshihide Tsuda, Katsuhiro Kobayashi, Hideo Inoue, Koji Narahara, Hiroshi Shiraga, Takafumi Kimura, Makoto Ogawa, Tomoyuki Terasaki, Hiromichi Ono, Tsutomu Takata

    EPILEPSY RESEARCH   96 ( 1-2 )   89 - 95   2011年9月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    To clarify the incidence of first-ever episodes of status epilepticus (SE), their etiologies and outcomes among Japanese children, we performed an epidemiological study in Okayama City. One hundred and twenty patients (69 males, 51 females) experienced first-ever SE episodes between 2003 and 2005. Overall, the annual incidence of SE was 42.0 per 100,000 population (95% CI: 34.5-49.5). The highest incidence was seen in patients aged <2 years, especially in the second year of life. Febrile SE accounted for 59 (49.2%) cases, and acutesymptomatic etiologies accounted for 21. Ten were considered to have remote-symptomatic etiologies, and eight to have acute-on-remote-symptomatic etiologies. Ten were classified as cryptogenic/idiopathic epilepsy-related, and 12 were unclassified. Nineteen (15.8%) patients were diagnosed with exanthema subitum, including three with encephalitis/encephalopathy, and 17 (14.2%) patients with influenza, including four with encephalitis/encephalopathy. After SE, eight (6.7%) patients suffered from motor disturbance with or without mental disturbance. One of these died during the follow-up period. Ultimately 34 (28.3%) of the 120 patients had been diagnosed with epilepsy by the end of the follow-up. We conclude that the incidence of SE among Japanese children is higher than the reported incidence among Caucasian children. Febrile SE accounted for approximately half of the cases. Among the etiologies, exanthema subitum was the most important infectious disease, followed by influenza. Both types of infection caused encephalitis/encephalopathy associated with SE as well as febrile SE. (C) 2011 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2011.05.004

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  • Dravet syndrome with an exceptionally good seizure outcome in two adolescents

    Katsuhiro Kobayashi, Iori Ohmori, Mamoru Ouchida, Yoko Ohtsuka

    EPILEPTIC DISORDERS   13 ( 3 )   340 - 344   2011年9月

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    記述言語:英語   出版者・発行元:JOHN LIBBEY EUROTEXT LTD  

    We present two children who exhibited the characteristics of Dravet syndrome during infancy and young childhood, with SCN1A mutation, but nevertheless achieved seizure freedom for at least four years during adolescence. These patients had no episodes of convulsive status epilepticus with a duration of more than 30 minutes and their overall favourable seizure outcome may be related to the prevention of convulsive status epilepticus.

    DOI: 10.1684/epd.2011.0453

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  • Bilaterally independent epileptic spasms in a case of Aicardi syndrome

    Katsuhiro Kobayashi, Kiyoko Watanabe, Harumi Yoshinaga, Yoko Ohtsuka

    EPILEPTIC DISORDERS   13 ( 3 )   326 - 330   2011年9月

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    記述言語:英語   出版者・発行元:JOHN LIBBEY EUROTEXT LTD  

    A girl with Aicardi syndrome was observed to have two distinct types of asymmetric epileptic spasms, as detected by ictal video-EEG recording at three months of age. When the two types of spasm concurred, they showed no mutual interactions based on either clinical or EEG aspects. This observation does not support the hypothesis that the brainstem always plays an initiating role in generating spasms. [Published with video sequences]

    DOI: 10.1684/epd.2011.0456

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  • 急性脳症における遺伝性けいれん素因とSCN1A遺伝子変異

    大守 伊織, 小林 勝弘, 大内田 守, 奥村 彰久, 前垣 義弘, 西山 逸子, 大塚 頌子

    脳と発達   43 ( Suppl. )   S352 - S352   2011年5月

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    記述言語:英語   出版者・発行元:(一社)日本小児神経学会  

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  • Abnormal Fast Activity before the Onset of West Syndrome

    F. Endoh, H. Yoshinaga, Y. Ishizaki, M. Oka, K. Kobayashi, Y. Ohtsuka

    NEUROPEDIATRICS   42 ( 2 )   51 - 54   2011年4月

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    記述言語:英語   出版者・発行元:GEORG THIEME VERLAG KG  

    In our sequential EEG study performed on 68 infants with various pre- and perinatal brain insults, we found peculiar abnormal fast activity (AFAs) in 12 patients. 9 of the 12 patients with AFAs later developed West syndrome (WS) compared with only 3 of the 56 patients without AFAs (p < 0.001, X(2) test). We analyzed these AFAs using EEG topography, and compared them with ictal fast activity (IFA) corresponding to tonic spasms observed later in the same patients after they had developed WS. We also investigated the clinical and EEG features in these patients. AFAs were first observed commonly at 4-5 months of CA, before the onset of WS. AFA topographic maps revealed posterior predominance in 11 of the 12 patients; IFA maps also showed posterior predominance but were more widely distributed. We propose that, though AFAs and IFAs are different, they share certain aspects of their pathophysiology, and that the maturational process of the occipital cortex plays an important role in the shared aspects. Since AFAs are observed before the onset of WS, they can be considered a sign that WS is imminent.

    DOI: 10.1055/s-0031-1277160

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  • High-frequency changes during interictal spikes detected by time-frequency analysis

    Julia Jacobs, Katsuhiro Kobayashi, Jean Gotman

    CLINICAL NEUROPHYSIOLOGY   122 ( 1 )   32 - 42   2011年1月

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    記述言語:英語   出版者・発行元:ELSEVIER IRELAND LTD  

    Objective: High-frequency (HF) changes were analysed in relation to anatomical origin of spikes, shape and occurrence within the seizure onset zone (SOZ). We evaluated whether HF changes are linked to the SOZ, as established for distinct high-frequency oscillations.
    Methods: SEEG was filtered at 500 Hz and sampled at 2000 Hz. Spikes were selected by shape (spike/spike-slow wave) and location (SOZ/non-SOZ and neocortex/amygdala/hippocampus) in 15 patients. About 50 spikes were averaged for each set. Changes compared to baseline were quantified with false discovery rate controlled t-statistics using time-frequency spectra. Counts of increased or decreased time-frequency values were compared across spike categories in the 80-250 and 250-500 Hz bands.
    Results: Seventy-seven spike types were analysed. Differences between spike categories were most prominent between 250 and 500 Hz. HF changes were more frequent and larger in mesial temporal than in neocortical spikes and for spikes with slow waves than spikes alone. HF changes above 250 Hz were more frequent in spikes within than outside the SOZ.
    Conclusions: HF increases above 250 Hz show regional differences and are very prominent in the SOZ. Hippocampal spikes have the strongest HF components.
    Significance: Analysis of HF changes during spikes may provide information on differing pathophysiological mechanisms of spikes and on epileptogenicity of the tissue. (C) 2010 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

    DOI: 10.1016/j.clinph.2010.05.033

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  • 小児期発症の難治てんかんに対するlamotorigineの効果-開始3ヵ月時と6ヵ月時の比較を含めて-.

    渡邊聖子, 小林勝弘, 遠藤文香, 吉永治美, 大塚頌子

    脳と発達   43 ( 6 )   453 - 458   2011年

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  • 成人期に達した Dravet症候群の長期経過に関する研究.

    秋山麻里, 小林勝弘, 吉永治美, 大塚頌子

    岡山医学会雑誌   123 ( 2 )   91 - 95   2011年

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  • Scalp-recorded high-frequency oscillations in childhood sleep-induced electrical status epilepticus

    Katsuhiro Kobayashi, Yoshiaki Watanabe, Takushi Inoue, Makio Oka, Harumi Yoshinaga, Yoko Ohtsuka

    EPILEPSIA   51 ( 10 )   2190 - 2194   2010年10月

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    記述言語:英語   出版者・発行元:WILEY-BLACKWELL  

    P>Because high-frequency oscillations (HFOs) may affect normal brain functions, we examined them using electroencephalography (EEG) in epilepsy with continuous spike-waves during slow-wave sleep (CSWS), a condition that can cause neuropsychological regression. In 10 children between 6 and 9 years of age with epilepsy with CSWS or related disorders, we investigated HFOs in scalp EEG spikes during slow-wave sleep through temporal expansion of the EEG traces with a low-cut frequency filter at 70 Hz as well as through time-frequency power spectral analysis. HFOs (ripples) concurrent with spikes were detected in the temporally expanded traces, and the frequency of the high-frequency peak with the greatest power in each patient's spectra ranged from 97.7 to 140.6 Hz. This is the first report on the detection of HFOs from scalp EEG recordings in epileptic patients. We speculate that epileptic HFOs may interfere with higher brain functions in epilepsy with CSWS.

    DOI: 10.1111/j.1528-1167.2010.02565.x

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  • Electroclinical characterization and classification of symptomatic epilepsies with very early onset by multiple correspondence analysis

    Tomoyuki Akiyama, Katsuhiro Kobayashi, Yoko Ohtsuka

    EPILEPSY RESEARCH   91 ( 2-3 )   232 - 239   2010年10月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Purpose: To characterize very early onset symptomatic epilepsies electroclinically, to identify meaningful factors to delineate epilepsy syndromes under this category, and to test validity of the current syndromic organization of these epilepsies by the International League Against Epilepsy (ILAE).
    Methods: Subjects were 38 epileptic patients with seizure onset before 3 months of age. We investigated the age of seizure onset, seizure types, myoclonus, EEG features, neuroimaging studies, outcome of seizures and development. We performed multiple correspondence analysis (MCA) to reveal associations among these characteristics and to find important characteristics to divide this epilepsy group into subgroups.
    Results: Very early onset epilepsies were characterized by partial seizures and epileptic spasms, especially occurring in combination as a single ictal event (combined seizures), and poor outcome. Suppression-burst (SB), hypsarrhythmia, EEG asymmetry and structural brain abnormalities were common. MCA demonstrated that presence or absence of SB was the most meaningful factor to separate this group into subgroups. MCA on the subgroup with SB revealed a subset with early-onset spasms, partial seizures with single focus, no myoclonus, SB during wakefulness and sleep, no EEG asynchrony, hypsarrhythmia and combined seizures, and another subset with late-onset spasms, partial seizures with multiple foci, myoclonus, EEG asynchrony, no combined seizures, SB during sleep only, no EEG asymmetry, no structural abnormality and no hypsarrhythmia. These corresponded to Ohtahara syndrome and early myoclonic encephalopathy, respectively.
    Conclusions: Very early onset symptomatic epilepsies have the electroclinical characteristics distinct from those with later onset. This study supported the current ILAE organization on this epilepsy group from statistic approach. (C) 2010 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2010.07.018

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  • Genetic seizure susceptibility underlying acute encephalopathies in childhood

    Katsuhiro Kobayashi, Mamoru Ouchida, Akihisa Okumura, Yoshihiro Maegaki, Itsuko Nishiyama, Hideki Matsui, Yoko Ohtsuka, Iori Ohmori

    EPILEPSY RESEARCH   91 ( 2-3 )   143 - 152   2010年10月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    We herein investigated risk factors of pediatric acute encephalopathy (AE) regarding the hitherto uncharacterized genetic background of seizure susceptibility underlying the pathogenesis of AE. The study included 15 patients with a history of various types of AE in childhood. We undertook the mutational analysis of the neuronal sodium channel alpha 1 subunit (SCN1A) gene which is the most representative gene for hyperthermia-induced seizure susceptibility.
    Six patients (40%) had a positive family history of seizures or AE, especially febrile seizures, in first- or second-degree relatives. The SCN1A-R1575C mutation was detected in a patient with a history of acute encephalitis with refractory, repetitive partial seizures (AERRPS) and also in the patient's apparently healthy father.
    In the present study, dense familial seizure predisposition was present in the patients with AE. Although the presence of seizure susceptibility alone is insufficient to cause AE, it can exacerbate seizures and the subsequent development of inflammatory reactions in the brain when environmental factors are included. Genetic seizure susceptibility may contribute to some types of AE in childhood. Crown Copyright (C) 2010 Published by Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2010.07.005

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  • Characteristics of the synchronous occipital and frontopolar spike phenomenon in Panayiotopoulos syndrome

    Harumi Yoshinaga, Katsuhiro Kobayashi, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   32 ( 8 )   603 - 608   2010年9月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Purpose: The synchronous appearance of an occipital and frontopolar spike (the Fp-O spike) is characteristic of Panayiotopoulos syndrome (PS). This phenomenon is also seen in various other types of epilepsy, particularly those that occur in childhood. Using dipole analysis and sequential mapping, we investigated the characteristics of the Fp-O spike observed in seven patients with PS and six patients with symptomatic localization-related epilepsy in childhood (SLE). Methods: We analyzed both one averaged spike and 20 manually selected successive individual Fp-O spikes for each patient through sequential topographical mapping with steps of 10 ms from 40 ms before to 40 ms after the negative maximum peak of each spike. For dipole analysis, a period of 40 ms before the maximum negative peak of the averaged spike in each patient was examined using equivalent current dipole localization software. Results: Sequential mapping revealed that occipital negative peaks preceded frontal negative peaks in all of the PS patients, as well as in two of the six SLE patients. The four remaining SLE patients did not exhibit preceding occipital peaks. In all of the patients with PS, representative dipole locations were in the posterior area, whereas in SLE patients they were scattered over more anterior areas. The estimated sources of the Fp-O and 0 spikes appeared to have the same position and orientation in the two PS patients. Conclusion: We conclude that Fp-O spikes in PS occur as the result of a rapid spread of epileptic activity from the posterior areas to the anterior areas of the brain. Fp-O spikes in PS patients show a uniform topographical pattern and dipole location, whereas those in other patients show more heterogeneity in these features. These findings support the homogeneity of PS and thus its designation as a syndrome. (C) 2009 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2009.09.007

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  • A long-term follow-up study of Dravet syndrome up to adulthood

    Mari Akiyama, Katsuhiro Kobayashi, Harumi Yoshinaga, Yoko Ohtsuka

    EPILEPSIA   51 ( 6 )   1043 - 1052   2010年6月

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    記述言語:英語   出版者・発行元:WILEY-BLACKWELL  

    P>Purpose:
    We intended to elucidate the whole clinical course of Dravet syndrome (DS) comprehensively, from infancy through adulthood.
    Methods:
    Subjects were 31 patients with DS (14 with typical DS, and 17 with borderline DS) who were followed from childhood to at least 18 years of age. Their seizures, abilities, and electroencephalography (EEG) findings were investigated and statistically analyzed.
    Results:
    The clinical findings of the patients with typical DS and those with borderline DS became largely similar in adolescence and adulthood. Seizures were intractable in childhood in all patients, but suppressed in five (16.1%) during follow-up. Thirty-five (87.5%) of the 40 apparently generalized convulsive seizures that were captured by ictal EEG recording at 7 years of age or later were of focal origin. The seizure-free outcomes were significantly correlated with the experience of < 3 episodes of convulsive status epilepticus, and also with disappearance of spikes on the follow-up EEGs. Mental outcomes involving less severe intellectual disability were correlated with the presence of occipital alpha rhythms in the background activity of the follow-up EEGs. Mean age at the recording of the follow-up EEGs was 23.8 years.
    Discussion:
    Prevention of the occurrence of convulsive status epilepticus was indicated to be critically important for the improvement of seizure prognosis in DS.

    DOI: 10.1111/j.1528-1167.2009.02466.x

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  • Detection of seizure-associated high-frequency oscillations above 500 Hz

    Katsuhiro Kobayashi, Takashi Agari, Makio Oka, Harumi Yoshinaga, Isao Date, Yoko Ohtsuka, Jean Gotman

    EPILEPSY RESEARCH   88 ( 2-3 )   139 - 144   2010年2月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    High-frequency oscillations (HFOs) of up to 500Hz in EEG are considered to have close relation with ictogenesis. We had the unique opportunity to record a seizure in EEG with intracerebral macroelectrodes and a sampling frequency of 10 kHz. Considering the notion that faster HFOs are likely more ictogenic, we investigated this ictal EEG data to find if even faster HFOs were present.
    HFOs were investigated in interictal spikes and seizure activity using time-frequency spectra: t values corresponding to frequencies from 100 to 1000 Hz were obtained by comparison to the background and controlled by the false discovery rate (FDR).
    The seizure had a right hippocampal onset. HFOs up to 800 Hz as well as HFOs below 500 Hz built up in the hippocampal discharges more at the beginning of the seizure and during the preictal period than in the interictal period. These HFOs were visually confirmed in temporally expanded EEG traces.
    We demonstrated for the first time the existence of HFOs above 500 Hz and up to 800 Hz with intracerebral macroelectrodes in an epileptic patient; they occurred primarily in association with the seizure discharge. HFOs above 500 Hz possibly reflect facilitation of ictogenic neuronal hypersynchronization. (C) 2009 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2009.10.008

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  • Epileptic spasms の病態生理

    東田好広, 小林勝弘, 大塚頌子

    臨床脳波   52 ( 12 )   724 - 729   2010年

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  • 強直発作の発作時脳波におけるγ律動のスペクトル分析

    小林勝弘, 井上拓志, 渡邊嘉章, 岡牧郎, 大塚頌子

    臨床脳波   52 ( 3 )   145 - 150   2010年

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  • Age-Dependent Spike Localization in Various Epileptic Syndromes

    Harumi Yoshinaga, Katsuhiro Kobayashi, Yumiko Ishizaki, Mari Wakai, Yusuke Tominaga, Takanori Matsuoka, Yoko Ohtsuka

    PEDIATRIC NEUROLOGY   41 ( 6 )   440 - 444   2009年12月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE INC  

    The electroencephalograms from 276 patients with localization-related epilepsy were analyzed to compare the distribution of spike foci in different age groups. Patients were divided into five groups according to spike location in the frontal, central, temporal, occipital, or multiple cortical regions. The age of peak incidence was earliest in patients with occipital foci, followed by those with central foci and then those with frontal foci. A bimodal age distribution of patients with temporal foci was observed. Symptomatic patients frequently exhibited multiple and frontal foci, and a large number of idiopathic patients had central, temporal, and occipital foci. Multiple foci were detected in 27.5% of idiopathic patients. Age-related spike localization was uniformly observed, regardless of the epileptic syndrome. The analysis of these data indicates that there are two types of multiple foci, one correlated with organic lesions and the other with an idiopathic, functional nature. (C) 2009 by Elsevier Inc. All rights reserved.

    DOI: 10.1016/j.pediatrneurol.2009.06.005

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  • DETECTION OF SEIZURE-ASSOCIATED HIGH-FREQUENCY OSCILLATIONS ABOVE 500 HZ

    Katsuhiro Kobayashi, T. Agari, M. Oka, H. Yoshinaga, I. Date, Y. Ohtsuka, J. Gotman

    EPILEPSIA   50   160 - 160   2009年11月

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    記述言語:英語   掲載種別:研究発表ペーパー・要旨(国際会議)   出版者・発行元:WILEY-BLACKWELL PUBLISHING, INC  

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  • Spectral analysis of EEG gamma rhythms associated with tonic seizures in Lennox-Gastaut syndrome

    Katsuhiro Kobayashi, Takushi Inoue, Yoshiaki Watanabe, Makio Oka, Fumika Endoh, Harumi Yoshinaga, Yoko Ohtsuka

    EPILEPSY RESEARCH   86 ( 1 )   15 - 22   2009年9月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Purpose: EEG gamma rhythms, which are found in association with epileptic spasms in infants with West syndrome, were explored in the ictal EEGs of tonic seizures in older patients with Lennox-Gastaut syndrome (LGS) to investigate the pathophysiology of the disease.
    Methods: The subjects were 20 patients with LGS (11 mates, 9 females; age range: 3 years 1 month to 29 years 3 months) who had at least one digitally recorded tonic seizure with minimal artifacts. A time-frequency analysis was applied to each patient's ictal EEG data.
    Results: A total of 54 seizures were analyzed, excluding spasms in clusters. The ictal EEGs of the tonic seizures showed only diffuse desynchronization in 10 seizures, and desynchronization followed by rhythmic activity in 21. The ictal discharges started as rhythmic activity of varying amplitude without initial desynchronization in 23 seizures. In a total of 25 seizures from 13 patients, gamma rhythms with frequencies ranging from 43 to 101.6 Hz were detected by temporal expansion of the ictal EEG traces and spectral analysis. In 24 (96%) of these seizures, gamma rhythms were observed at seizure onset corresponding to visually identified desynchronization. In the remaining seizure, gamma rhythms were found in association with transient suppression of high-amplitude rapid discharges.
    Conclusion: The detection of gamma rhythms in the ictal EEGs of tonic seizures indicated that some tonic seizures might have generative mechanisms in common with epileptic spasms, and that these mechanisms are possibly related to desynchronization at seizure onset. (C) 2009 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2009.03.011

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  • Abnormal fast activity in infancy with paroxysmal downwards gaze

    Harumi Yoshinaga, Katsuhiro Kobayashi, Fumika Endo, Yumiko Ishizaki, Marl Wakai, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   31 ( 6 )   435 - 441   2009年6月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    We report here on 8 infants who showed paroxysmal downwards gaze (PDG). The time of initial appearance of PDG ranged from one month to five months (mean: 2.7 months) of corrected age. Seven out of eight patients showed interictal spikes in EEG, so they were started on prophylactic therapy with antiepileptic drugs. In five of the eight patients, PDG ceased, either spontaneously or with antiepileptic drug treatment, by four to eight months of corrected age. Six out of eight patients showed localized spikes and peculiar abnormal fast activity (AFA) in the occipital area and five of these patients later developed West syndrome. These AFA were observed on EEGs recorded at the time of initial PDG appearance, before hypsarrhythmia was observed and before tonic spasms appeared. We were able to exclude the possibility that PDG was a Subtle epileptic seizure by confirming the temporal discordance between individual episodes of PDG and AFA with video-EEG monitoring. Yet topographic data showed that AFA in these patients was characteristically located in the occipital area, with a distribution similar to that of the fast activity which accompanied the tonic spasms that later developed in these patients. As a risk factor for developing WS, we propose the clinical symptom of PDG with characteristic occipital AFA visible in the EEG, both of which represent damage to the occipital region including the optic radiation. (C) 2008 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2008.08.007

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  • Detection of changes of high-frequency activity by statistical time-frequency analysis in epileptic spikes

    Katsuhiro Kobayashi, Julia Jacobs, Jean Gotman

    CLINICAL NEUROPHYSIOLOGY   120 ( 6 )   1070 - 1077   2009年6月

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    記述言語:英語   出版者・発行元:ELSEVIER IRELAND LTD  

    Objective: A novel type of statistical time-frequency analysis was developed to elucidate changes of high-frequency EEG activity associated with epileptic spikes.
    Methods: The method uses the Gabor Transform and detects changes of power in comparison to background activity using t-statistics that are controlled by the false discovery rate (FDR) to correct type I error of multiple testing. The analysis was applied to EEGs recorded at 2000 Hz from three patients with mesial temporal lobe epilepsy.
    Results: Spike-related increase of high-frequency oscillations (HFOs) was clearly shown in the FDR-controlled t-spectra: it was most dramatic in spikes recorded from the hippocampus when the hippocampus was the seizure onset zone (SOZ). Depression of fast activity was observed immediately after the spikes, especially consistently in the discharges from the hippocampal SOZ. It corresponded to the slow wave part in case of spike-and-slow-wave complexes, but it was noted even in spikes without apparent slow waves. In one patient, a gradual increase of power above 200 Hz preceded spikes.
    Conclusions: FDR-controlled t-spectra clearly detected the spike-related changes of HFOs that were unclear in standard power spectra.
    Significance: We developed a promising tool to study the HFOs that may be closely linked to the pathophysiology of epileptogenesis. (C) 2009 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

    DOI: 10.1016/j.clinph.2009.03.020

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  • Complex partial status epilepticus in children with epilepsy

    Kenichi Kikumoto, Harumi Yoshinaga, Katsuhiro Kobayashi, Makio Oka, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   31 ( 2 )   148 - 157   2009年2月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Purpose: Complex partial status epilepticus (CPSE) is often under-diagnosed, especially in children. The aim of this study was to clarify the characteristics and pathophysiology of CPSE in children with epilepsy. Subjects and methods: We retrospectively reviewed the medical records and EEGs of 17 children with epilepsy who were diagnosed as having CPSE by ictal or postictal EEGs to investigate clinical and EEG features. Results: The ages at diagnosis of CPSE ranged from 3 months to 17 years. At the time of diagnosis of CPSE, 13 patients had symptomatic localization-related epilepsy, two had epilepsy with continuous spike-waves during slow wave sleep, and each patient had cryptogenic localization-related epilepsy and idiopathic localization-related epilepsy. Only subtle symptoms including autonomic ones associated with disturbance of consciousness were the main clinical features in 12 of 44 CPSE episodes. Another 22 episodes showed minor focal motor elements, and the other 10 had major convulsive phase during or immediately before CPSE. Ictal EEGs of CPSE were divided into three types according to the degree of high-voltage slow waves (HVS) and spike components. Ictal EEGs could show spike-dominant or spike and HVS mixed patterns even if patients showed only subtle symptoms. The epileptogenic areas estimated by the ictal or postictal EEGs showed variability with only two cases of temporal origin, Conclusion: The close observation of clinical symptoms such as various subtle symptoms and/or mild convulsive elements and ictal EEGs are absolutely needed for the diagnosis of CPSE in children. (C) 2008 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2008.04.009

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  • A case of reflex epilepsy induced by neuropsychological tasks including writing, calculation, and spatial tasks

    Inoue T, Kobayashi K, Ohtsuka Y

    Clinical Electroencephalography   51 ( 7 )   449 - 453   2009年

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  • Electroencephalographic recording

    Kobayashi K, Watanabe Y, Oka M

    119 ( 41 )   353 - 358   2009年

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  • 書字・計算などの神経心理学的課題により発作が誘発された反射てんかんの1例

    井上拓志, 小林勝弘, 大塚頌子

    臨床脳波   51 ( 7 )   449 - 453   2009年

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  • 脳波検査の注意点と問題点

    小林勝弘, 渡邊嘉章, 岡牧郎

    小児内科   119 ( 41 )   353 - 358   2009年

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  • Magnetoencephalogram in a postoperative case with a large skull defect

    Haruffni Yoshinaga, Katsuhiro Kobayashi, Tohru Hoshida, Kazushi Kinugasa, Yoko Ohtuska

    PEDIATRIC NEUROLOGY   39 ( 1 )   48 - 51   2008年7月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE INC  

    We present a patient in whom magnetoencephalograms were successfully performed in presurgical and postsurgical evaluations. A 12-year-old boy with congenital porencephaly was admitted with refractory adversive seizures and frontal absence seizures. Ictal magnetoencephalographic dipoles with frontal absence seizures were located in the left frontal lobe, anterior to the porencephalic cyst, and concordant with the same area detected by intraoperative electrocorticography. A partial cortical excision was performed, and the patient's cranial bone flap was removed because of an epidural abscess. The frontal absences then disappeared. The magnetoencephalogram revealed that secondary bilateral synchrony of focal discharges from the lesion may have caused the generalized seizures in this patient. Because of residual partial seizures, second and third magnetoencephalograms were performed, and we detected residual spike dipoles clustering in the area posterior to the cavity of cortical excision and anterior to the porencephalic cyst. Another excision of the area between the cavity and frontal edge of the cyst was performed, and seizure frequency diminished dramatically. In this case, despite the failure of dipole estimation by electroencephalogram in the context of a large bone defect, the magnetoencephalogram was useful in detecting the residual epileptogenic zone after failed epilepsy surgery. (C) 2008 by Elsevier Inc. All rights reserved.

    DOI: 10.1016/j.pediatrneuro1.2008.03.010

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  • Spectral characteristics of EEG gamma rhythms associated with epileptic spasms

    Takushi Inoue, Katsuhiro Kobayashi, Makio Oka, Harumi Yoshinaga, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   30 ( 5 )   321 - 328   2008年5月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    To elucidate the pathophysiology of epileptic spasms, unaveraged time-frequency spectra of spasm-associated EEG gamma rhythms were investigated in 15 patients with West syndrome or related disorders. Using these unaveraged spectra, we were able to investigate in detail various aspects of the structure of ictal gamma rhythms that could not be examined using averaged spectra. The characteristics of the ictal gamma peaks (peak frequency, power, duration, and the number of peaks in each brain-region for each spasm) were statistically evaluated with respect to their differences among the brain regions and over the time-course of the clusters. Our findings were as follows: (1) Gamma peaks were clearly detected in most spectra and generally had a similar pattern in each spasm, which repeated in clusters. (2) The mean frequency of gamma peaks was 69.2 +/- 16.8 Hz, and the number of peaks in each brain region of each spasm was 1.83 +/- 1.16. (3) The occipitoparietal gamma peaks had significantly greater power and longer duration than the frontocentral peaks. (4) The frequency of the gamma peaks was higher in the mid phase of clusters than in the ending, and it tended to have a positive correlation with its latency from the preceding beta peak. An analysis of the ictal gamma rhythms might give some insight into the generative mechanism of spasms. (c) 2007 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2007.10.003

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  • Rasmussen encephalitis associated with SCN1A mutation

    Iori Ohmori, Mamoru Ouchida, Katsuhiro Kobayashi, Yoshimi Jitsumori, Takushi Inoue, Kenji Shimizu, Hideki Matsui, Yoko Ohtsuka, Yoshihiro Maegaki

    EPILEPSIA   49 ( 3 )   521 - 526   2008年3月

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    記述言語:英語   出版者・発行元:BLACKWELL PUBLISHING  

    Mutations in the SCN1A gene, encoding the neuronal voltage-gated sodium channel alpha 1 subunit, cause SMEI, GEFS+, and related epileptic syndromes. We herein report the R1575C-SCN1A mutation identified in a patient with Rasmussen encephalitis. R1575C were constructed in a recombinant human SCN1A and then heterologously expressed in HEK293 cells along with the human beta 1 and beta 2 sodium channel accessory subunits. Whole-cell patch-clamp recording was used to define biophysical properties. The R1575C channels exhibited increased channel availability and an increased persistent sodium current in comparison to the wild-type. These defects of electrophysiological properties can result in neuronal hyperexitability. The seizure susceptibility allele may influence the pathogenesis of Rasmussen encephalitis in this case.

    DOI: 10.1111/j.1528-1167.2007.01411.x

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  • 臨床生理:脳波検査のステップアップ・8 小児基礎脳波

    小林勝弘, 大塚頌子

    検査と技術   36 ( 7 )   640 - 647   2008年

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  • Dramatic aggravation of partial seizures induced by gabapentin overdose

    Kobayashi K, Takeuchi A, Oka M, Endoh F, Yoshinaga H, Ohtsuka Y

    Epilepsy & Seizure   1 ( 1 )   40 - 46   2008年

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  • Management of childhood-onset epilepsy evaluated with a long-term follow-up study

    Ohtsuka Y, Kobayashi K, Namba M, Endoh F, Oka M, Yoshinaga H

    Epilepsy & Seizure   1 ( 1 )   21 - 29   2008年

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  • 食事によって誘発されるシリーズ形成性 spasms を認めた小児例

    井上拓志, 秋山倫之, 小林勝弘, 大塚頌子

    臨床脳波   50 ( 4 )   246 - 250   2008年

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  • Pediatric EEG background activity

    Kobayashi K, Ohtsuka Y

    Modern Medical Laboratory   36 ( 7 )   640 - 647   2008年

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  • A case of periodic spasms induced by eating

    Inoue T, Akiyama T, Kobayashi K, Ohtsuka Y

    Clinical Electroencephalography   50 ( 4 )   246 - 250   2008年

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  • SPECTRAL ANALYSIS OF EEG GAMMA RHYTHMS ASSOCIATED WITH EPILEPTIC TONIC SEIZURES

    Katsuhiro Kobayashi, T. Inoue, Y. Watanabe, K. Watanabe, M. Oka, F. Endoh, M. Wakai, Y. Ishizaki, A. Takeuchi, Y. Imai, T. Morooka, T. Nakahori, H. Yoshinaga, Y. Ohtsuka

    EPILEPSIA   49   245 - 245   2008年

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    記述言語:英語   掲載種別:研究発表ペーパー・要旨(国際会議)   出版者・発行元:WILEY-BLACKWELL PUBLISHING, INC  

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  • Electroencephalographic changes before the onset of symptomatic West syndrome

    Fumika Endoh, Harurni Yoshinaga, Katsuhiro Kobayashi, Yoko Ohtsuka

    BRAIN & DEVELOPMENT   29 ( 10 )   630 - 638   2007年11月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    To clarify the characteristics of the mode of appearance and morphology of epileptiform discharges before the onset of West syndrome (WS). The subjects were 25 infants whose electroencephalograms (EEGs) were recorded before the onset of WS and whose first EEG was recorded before 6 months of corrected age (CA). We extensively analyzed the chronological and topographical changes of the epileptiform discharges before the onset of WS. The location of the initial epileptiform discharges was in the posterior areas in 14 (Group O), the multiple areas in 7 (Group M), and areas other than occipital in 4 (Group non-O). Twelve of the 14 patients in Group O were premature infants, and all but one had PVL. Most patients in Group M were full-term infants or near full-term infants who had hypoxic damage. The ages at the appearance of the initial epileptiform. discharges in Group O were significantly later than those in Group M: 3.0-5.9 months of CA in Group O vs. -0.1 to 2.0 months of CA in Group M. These facts suggest that the difference of brain damage is related to both the topographical characteristics and the age at the appearance of initial epileptiform discharges, and around 3 months of CA is a critical period for the appearance of occipital hyperexcitability. Hypsarrhythmia and tonic spasms appeared almost simultaneously from 4 to 6 months of CA in most patients. To predict the occurrence of WS in high-risk infants, EEG follow-ups from early infancy are very useful. (C) 2007 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2007.04.004

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  • Relation of spasms and myoclonus to suppression-burts on EEG in epileptic encephalopathy in early infancy

    K. Kobayashi, T. Inoue, K. Kikumoto, F. Endoh, K. Miya, M. Oka, H. Yoshinaga, Y. Ohtsuka

    NEUROPEDIATRICS   38 ( 5 )   244 - 250   2007年10月

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    記述言語:英語   出版者・発行元:GEORG THIEME VERLAG KG  

    Purpose: This study was intended to clarify the relation between spasms in series and a suppression-burst (SB) EEG pattern which have a common nature of repetitive bursting activity in epileptic encephalopathy in early infancy.
    Methods: The ictal EEG traces of spasms were temporally compressed and expanded to study the beginning and ending phases of series along with their spectral analysis in two patients with Ohtahara syndrome (OS) and one with early myoclonic encephalopathy (EME). The EEG bursts associated with myoclonus were also investigated.
    Results: A mutual transition was indicated between the ictal activity of spasms and the bursts in the peri-series SB on EEG. Gamma rhythm was detected in common in the ictal activity and the peri-series and interictal bursts on EEG, and also in the bursts with myoclonus.
    Conclusion: The relation between the ictal activity of spasms and SB on EEG was shown to be close. The generative mechanisms of spasms and myoclonus might be linked to the bursting tendency intrinsic to immature brain function.

    DOI: 10.1055/s-2008-1062716

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  • 入門講座「小児脳波:最近の話題」 (2) ―小児の行動発達と脳波―.

    小林勝弘, 荻野竜也, 岡牧郎, 大塚頌子

    臨床脳波   49 ( 6 )   373 - 377   2007年

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  • 入門講座「小児脳波:最近の話題」 (1) ―特発性局在関連性てんかんの脳波―.

    小林勝弘, 吉永治美, 菊本健一, 大塚頌子

    臨床脳波   49 ( 5 )   315 - 320   2007年

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  • Topics on pediatric EEG (2): Behavior disorders in childhood and EEG

    Kobayashi K, Ogino T, Oka M, Ohtsuka Y

    Clinical Electroencephalography   49 ( 6 )   373 - 377   2007年

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  • Topics on pediatric EEG (1): EEG of idiopathic localization-related epilpesy

    Kobayashi K, Yoshinaga H, Kikumoto K, Ohtsuka Y

    Clinical Electroencephalography   49 ( 5 )   315 - 320   2007年

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  • SCN1A missense mutation associated with infantile partial epilepsy

    Iori Ohmori, Mamoru Ouchida, Katsuhiro Kobayashi, Yoko Ohtsuka, Kenji Shimizu, Teiichi Nishiki, Kazuhito Tomizawa, Hideki Matsui

    NEUROSCIENCE RESEARCH   58   S187 - S187   2007年

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    記述言語:英語   掲載種別:研究発表ペーパー・要旨(国際会議)   出版者・発行元:ELSEVIER IRELAND LTD  

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  • O1-06 Epileptic spasmsの発作時脳波におけるgamma律動の周波数構造に関する研究(脳波2,一般演題(口演),第40回 日本てんかん学会)

    井上 拓志, 小林 勝弘, 菊本 健一, 岡 牧郎, 秋山 倫之, 大塚 頌子

    てんかん研究   24 ( 3 )   2006年8月

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    記述言語:日本語   出版者・発行元:日本てんかん学会  

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  • Diagnostic issues and treatment of cryptogenic or symptomatic generalized epilepsies

    Yoko Ohtsuka, Harumi Yoshinaga, Katsuhiro Kobayashi, Tatsuya Ogino, Makio Oka, Minako Ito

    EPILEPSY RESEARCH   70   S132 - S140   2006年8月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    To clarify the diagnostic issues and treatment of patients with cryptogenic or symptomatic generalized epilepsies, not including West syndrome (WS), we investigated electroclinical change during the clinical course, and treatment effects in these patients. The selection criteria were minor generalized seizures as their main seizure type and diffuse epileptic discharges as their main EEG findings. Regarding EEG, we included EEGs that predominantly displayed multifocal spike-waves because of the inclusion of severe epilepsy with multiple independent spike foci (SE-MISF). We divided the subjects into two groups according to their main seizure types: Group A (54 patients) with brief tonic seizures and Group B (24 patients) with myoclonic seizures and/or atypical absences. The main epileptic syndromes were considered to be Lennox-Gastaut syndrome and SE-MISF in Group A, and epilepsy with myoclonic-astatic seizures in Group B. A history of WS was often seen in Group A, but it was exceptional in Group B. During the clinical course, seizure types did not basically change in Group A. EEG patterns were changeable in both groups. Although there was some overlap in electroclinical manifestations among epileptic syndromes, a transition between the two groups was not seen. High-dose valproate and ethosuximide were the most effective in Groups A and B, respectively. Long-term prognosis was significantly more favorable in Group B than in Group A. (c) 2006 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2005.11.024

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  • A population-based survey of childhood epilepsy in Okayama Prefecture, Japan: Reclassification by a newly proposed diagnostic scheme of epilepsies in 2001

    Tomoyuki Akiyama, Katsuhiro Kobayashi, Tatsuya Ogino, Harumi Yoshinaga, Eiji Oka, Makio Oka, Minako Ito, Yoko Ohtsuka

    EPILEPSY RESEARCH   70   S34 - S40   2006年8月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    The purpose of this study is to clarify the usefulness and problems of the newly proposed classification of epilepsies (International League Against Epilepsy: ILAE, 2001) in the epidemiological studies of epilepsy.
    We previously conducted an epidemiological study in Okayama Prefecture, Japan, in 1999, using the ILAE 1989 classification. Among 250,997 children under 13 years of age, 2220 epileptic patients were ascertained. In this study, we reclassified them according to the ILAE 2001 classification, focusing on axes 2 (seizure types) and 3 (syndromes).
    We were able to classify 1803 (95.0%) seizure types out of 1899 with detailed clinical information. In focal seizures, the most common were secondarily generalized seizures (88.6%), which generally do not represent a unique anatomic substrate. In generalized seizures, topic-clonic seizures (40.7%) and spasms (21.0%) were the most common. We identified only 269 (12.1%) patients with specific epilepsy syndromes out of the 2220. We classified 1761 patients without specific syndromic diagnoses only by axis 2, but the new concept of epileptic seizure types, representing a unique pathophysiologic mechanism and anatomic substrate, was not very meaningful in most cases, even in those with focal seizures. (c) 2006 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.eplepsyres.2005.11.017

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  • Treatment of epilepsy with electrical status epilepticus during slow sleep and its related disorders

    M Inutsuka, K Kobayashi, M Oka, J Hattori, Y Ohtsuka

    BRAIN & DEVELOPMENT   28 ( 5 )   281 - 286   2006年6月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    To elucidate an effective therapeutic strategy for 'ESES syndrome', epilepsy with electrical status epilepticus during slow sleep (ESES) and its related epileptic disorders, we studied the effect of treatment on the EEG pattern of continuous spike-waves during slow wave sleep (CSWS) in 15 afflicted patients. Basically performed in the following order, the employed therapies included (1) high-dose valproate (VPA) therapy (serum level > 100 mu g/ml); (2) a combination therapy of VPA and ethosuximide (ESM); (3) short cycles of high-dose diazepam (oral or intrarectal DZP, 0.5-1 mg/kg per day for 6-7 days); and (4) intramuscular synthetic ACTH-Z therapy (0.01-0.04 mg/kg per day for 11-43 days). Regarding the initial EEG effect, a remission of CSWS was achieved by high-dose VPA therapy in 7 of 15 trials (47%), by the combination therapy of VPA and ESM in 3/7 trials (43%), by short cycles of high-dose DZP in 2/4 trials (50%), and by ACTH-Z therapy in 2/5 trials (40%). A permanent remission of ESES syndrome was achieved by high-dose VPA therapy and/or combination therapy of VPA and ESM in 10 patients (67%). The effects of short cycles of high-dose DZP and ACTH-Z therapy were at best temporary. Our strategy for the treatment of ESES syndrome is therefore considered valid. (c) 2006 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2005.09.004

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  • EEG dipole characteristics in panayiotopoulos syndrome

    H Yoshinaga, M Koutroumanidis, K Kobayashi, A Shirasawa, K Kikumoto, T Inoue, M Oka, Y Ohtsuka

    EPILEPSIA   47 ( 4 )   781 - 787   2006年4月

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    記述言語:英語   出版者・発行元:BLACKWELL PUBLISHING  

    Purpose: Panayiotopoulos syndrome (PS) is a type of benign childhood partial epilepsy that is frequently associated with abundant multifocal spikes other than main occipital spikes on the EEG. In this study, we investigated the characteristic features of dipoles in PS.
    Methods: We performed dipole analysis of the interictal occipital spike discharges seen in 10 children with PS (group A) and in 10 children with other types of symptomatic localization-related epilepsy (group B). We analyzed the dipoles of the averaged spike in each patient.
    Results: In group A, the averaged occipital spikes in each patient showed dense dipole locations in the mesial occipital area; in group B, widely scattered dipole locations were observed. In Group A, the geometric centers of the dipoles at each time point (such as at the main negative peak and before or after the main peak) were estimated in the neighboring locations. In contrast, they tended to be scattered in group B.
    Conclusions: Our study reveals that PS has high dipole stability, similar to that of rolandic epilepsy. From the electroencephalographic view, this seems to indicate a close link between these two syndromes.

    DOI: 10.1111/j.1528-1167.2006.00519.x

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  • Age-related electrical status epilepticus during sleep and epileptic negative myoclonus in DRPLA

    K Kobayashi, H Hata, M Oka, M Ito, H Yoshinaga, K Kashihara, Y Ohtsuka

    NEUROLOGY   66 ( 5 )   772 - 773   2006年3月

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    記述言語:英語   出版者・発行元:LIPPINCOTT WILLIAMS & WILKINS  

    DOI: 10.1212/01.wnl.0000200958.30060.36

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  • Prevalence of childhood epilepsy and distribution of epileptic syndromes: A population-based survey in Okayama, Japan

    E Oka, Y Ohtsuka, H Yoshinaga, N Murakami, K Kobayashi, T Ogino

    EPILEPSIA   47 ( 3 )   626 - 630   2006年3月

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    記述言語:英語   出版者・発行元:BLACKWELL PUBLISHING  

    Methods: Information on patients younger than 13 years with active epilepsy was collected from medical records. Patients diagnosed with epilepsy according to clinical and EEG findings were put on the list even if those patients had had a single seizure or seizures occurring during febrile episodes.
    Results: In total, 2,220 cases were identified from a background population of 250,997. The prevalence rate was 8.8 per 1,000. If we exclude patients who had experienced a single seizure or seizures occurring during febrile episodes to compare our results with previous reports, the prevalence rate was 5.3 per 1,000. Of the 2,220 cases, 2,030 (91.4%) were classified into three major categories by ILAE classification. They consisted of 1,556 (76.7%) with localization-related epilepsy, 453 (22.3%) with generalized epilepsy, and 21 (1.0%) with undetermined epilepsy. Of the 2,030 cases, 309 (15.2%) were classified into epileptic syndrome categories, and 84.8% of the total were nonspecific types of epilepsy.
    Conclusions: The prevalence rate of childhood epilepsy was distributed from 5.3 to 8.8 per 1,000. The appearance rate of various types of epileptic syndromes was low. Most cases could not be classified into the detailed categories of the International Classification (ILAE, 1989).

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  • 脳波検査 A.脳波検査の基礎知識 g.年齢による脳波の変化

    小林勝弘, 岡牧郎, 榎日出夫

    神経内科   65,Suppl. 4,56-63   2006年

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  • Epileptic spasms の発作時脳波における極高周波律動の周波数分析に関する検討

    小林勝弘, 井上拓志, 岡牧郎, 伊藤美奈子, 大塚頌子

    臨床脳波   2006年

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  • Characteristics of slow waves on EEG associated with epileptic spasms

    K Kobayashi, M Oka, T Inoue, T Ogino, H Yoshinaga, Y Ohtsuka

    EPILEPSIA   46 ( 7 )   1098 - 1105   2005年7月

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    記述言語:英語   出版者・発行元:BLACKWELL PUBLISHING  

    Purpose: The high-voltage slow waves (HVSs) on EEG associated with epileptic spasms were investigated to clarify their characteristics and their relation to the pathophysiology of spasms in West syndrome and related disorders.
    Methods: In 14 patients, digitally recorded EEG segments showing the ictal HVSs were extracted and their traces were overlaid by using an average reference. The ictal HVSs were also averaged to build maps for investigation of the pattern of potential distribution over the scalp.
    Results: In a total of 685 recorded spasms, 346 (50.5%) with minimal artifacts were selected to demonstrate that the ictal HVSs had a largely consistent waveform and distribution in each patient. The ictal HVSs were symmetrical in 10 patients and asymmetrical in the other four, and were relatively negative over the posterior region and positive over the frontal or temporal regions in 11 patients. Two symptomatic patients showed a marked deviation of the distribution of HVSs to the pathologically more involved hemisphere. An infant with Aicardi syndrome had two different types of spasms, each type showing a consistent pattern of HVSs with a lateralized distribution.
    Conclusions: The patterns of distribution of the ictal HVS may be related to the abnormal activation of the brain in the generation of spasms.

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  • Dipole Modeling of epileptic spikes can be accurate or misleading

    K Kobayashi, H Yoshinaga, Y Ohtsuka, T Gotman

    EPILEPSIA   46 ( 3 )   397 - 408   2005年3月

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    記述言語:英語   出版者・発行元:BLACKWELL PUBLISHING INC  

    Purpose: We investigated the accuracy and potential for serious error when representing cortical generators of epileptic spikes with the common single-dipole model. Spike generators were realistically simulated with cortical areas of different extents.
    Methods: The source was simulated by using a patch that comprised small triangles on the cortical surface, each triangle having an elementary dipole generator with a moment corresponding to real intracerebral fields of spikes. The source-patch covered various clinically important parts of the temporal and frontal lobes, with an area ranging from 6 to 120 cm(2). The scalp field was computed for each source-patch by using a realistic head model and was fitted by the single-dipole model to determine the best-fit dipole and the intracerebral distribution of residual variance (RV). Dipole modeling also was performed for the simulated scalp field with additional real EEG background.
    Results: The RV after fitting a dipole to the scalp field without noise was at most 1.34%. Scalp spikes arising from sources of 6 cm(2) were of small amplitude, and the dipoles estimated for these spikes were inconsistent. Extension of the source area was associated with increase of scalp potential amplitude, only very small increase of RV, and increased consistency of the estimated dipoles. When the source was very large, the dipoles clustered at very misleading locations.
    Conclusions: Pitfalls in dipole source localization are caused by the procedure of fitting the simplistic dipole model to real cortical sources with spatial extent and complex configuration.

    DOI: 10.1111/j.0013-9580.2005.31404.x

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  • Relationship between severity of epilepsy and developmental outcome in Angelman syndrome

    Y Ohtsuka, K Kobayashi, H Yoshinaga, T Ogino, L Ohmori, K Ogawa, E Oka

    BRAIN & DEVELOPMENT   27 ( 2 )   95 - 100   2005年3月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    To clarify the relationship between the degree of developmental disturbance and the severity of epilepsy in Angelman syndrome, we investigated 11 patients and measured both clinical outcomes and EEG parameters. Seven patients were followed up until after 8 years of age. Eight patients were found to have 15q11-q13 deletions.
    All patients experienced epileptic seizures and all but one displayed nonconvulsive status epilepticus (NCSE) during the period of observation. Epileptic seizures, including NCSE, disappeared by around 8 years of age. In addition, specific epileptic discharges, as measured by EEG, tended to subside with age. Although development seemed almost normal or only slightly delayed during the first 6 months of life, all patients eventually developed severe retardation. Two patients displayed very severe retardation and were unable to comprehend language or walk independently at the last follow-up. Only one patient was able to speak a few meaningful words. In one of the most severely affected patients, who showed the earliest onset of seizures and NCSE, it is possible that the repetitive bouts of NCSE might be responsible for the severe developmental outcome. However, the other patient with particularly severe retardation did not experience NCSE, while the patient with the most favorable outcome had repetitive episodes of NCSE.
    Therefore, we conclude that the severity of developmental disturbance in Angelman syndrome is not necessarily related to the degree of epilepsy. However, intensive therapy for NOSE might still be justified because there are some patients in whom NOSE results in a transient and sometimes permanent decline in mental and motor functioning.
    (C) 2004 Elsevier B.V. All rights reserved.

    DOI: 10.1016/j.braindev.2003.09.015

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  • 独立成分分析の脳波解析への応用―てんかん発射を中心として―

    小林勝弘, 井上拓志, 菊本健一, 遠藤文香, 吉永治美, 大塚頌子

    臨床脳波   2005年

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  • Relation between typical and borderline severe myoclonic epilepsy in infancy.

    Y Ohtsuka, Ohmori, I, T Ogino, K Kobayashi, E Oka

    EPILEPSIA   46   11 - 12   2005年

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    記述言語:英語   掲載種別:研究発表ペーパー・要旨(国際会議)   出版者・発行元:BLACKWELL PUBLISHING INC  

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  • Kinesigenic attacks with ictal electroencephalographic abnormalities

    T Akiyama, Y Ohtsuka, K Kobayashi, E Oka

    PEDIATRIC NEUROLOGY   31 ( 5 )   357 - 359   2004年11月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE INC  

    We report on a 14-year 5-month-old male who had attacks similar to those of paroxysmal kinesigenic choreoathetosis. The attacks were elicited exclusively by sudden movements. On several occasions, these attacks were immediately followed by loss of consciousness or a seizure. Ictal electroencephalograms of his attacks without loss of consciousness or a seizure indicated 1.5-3.0 Hz activity in the left hemisphere. A small dosage of carbamazepine was remarkably effective in stopping the attacks. This case demonstrates that a thorough ictal electroencephalographic examination is indispensable for clarifying the pathophysiology of kinesigenic attacks. The relationship between paroxysmal kinesigenic choreoathetosis and supplementary motor area seizures is also discussed. (C) 2004 by Elsevier Inc. All rights reserved.

    DOI: 10.1016/j.pediatrneurol.2004.05.006

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  • Clinical and electroencephalographic characteristics of children with febrile seizures plus

    K Kobayashi, Y Ohtsuka, L Ohmori, Y Nishio, M Fujiwara, M Ito, E Oka

    BRAIN & DEVELOPMENT   26 ( 4 )   262 - 268   2004年6月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Objectives: Febrile seizures plus (FS +) are attracting attention for their corresponding genetic abnormalities, and are defined as febrile seizures (FS) continuing beyond 6 years of age (late FS) or those associated with afebrile seizures. We tried to elucidate their clinical and EEG characteristics as compared with those of children having only FS. Subjects and methods: We reviewed clinical records in a pediatric neurology clinic to identify 31 patients with FS + (group FS +) and 51 with only FS (group FS). Their family history of seizures, clinical features and EEG findings were compared. Results: A family history of seizures was noted in 14 patients (45.2%) of group FS + and in 24 (47.1%) of group FS. In group FS +, 19 patients had late FS, 11 had afebrile seizures, and the remaining one had both types of seizures. Two patients had seizures induced by TV/video-game as well, and another suffered from absences. Epileptic EEG abnormalities, which included diffuse spike-waves and focal spikes, were noted in 13 patients (41.9%) of group FS + and 12 (23.5%) of group FS. Conclusions: The clinical and EEG characteristics of the children having FS + were diverse, without significant differences from those with FS except for the seizures types. (C) 2003 Elsevier B.V. All rights reserved.

    DOI: 10.1016/S0387-7604(03)00134-7

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  • Very fast rhythmic activity on scalp EEG associated with epileptic spasms

    K Kobayashi, M Oka, T Akiyama, T Inoue, K Abiru, T Ogino, H Yoshinaga, Y Ohtsuka, E Oka

    EPILEPSIA   45 ( 5 )   488 - 496   2004年5月

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    記述言語:英語   出版者・発行元:BLACKWELL PUBLISHING INC  

    Purpose: Very fast activity was investigated on the ictal EEGs of epileptic spasms to elucidate the pathophysiology of West syndrome (WS) and related disorders from a novel point of view.
    Methods: The traces of scalp ictal EEG of spasms temporally were expanded in 11 patients whose clinical diagnosis was symptomatic WS in six, cryptogenic WS in two, Aicardi syndrome in one, and symptomatic generalized epilepsy after WS in the remaining two. Time evolution of averaged power spectra of the ictal fast activity also was analyzed in each patient.
    Results: Rhythmic gamma activity with frequency ranging from 50 to 100 Hz was detected in a total of 345 of 537 spasms. Fast activity was seen bilaterally in nine patients, was lateralized to one hemisphere in another, and appeared independently on each hemisphere in the remaining infant with Aicardi syndrome. Power spectra showed a clear peak corresponding to spasm-associated gamma rhythm, with frequency centering similar to65 Hz and ranging from 51 to 98 Hz. The morphology and spectral characteristics of ictal gamma rhythm were completely different from those of muscle activity or alternating current (AC) artifacts.
    Conclusions: Spasm-associated gamma activity was clearly detected on the scalp. This observation may provide a clue to the pathophysiology of spasms.

    DOI: 10.1111/j.0013-9580.2004.45703.x

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  • A study of spike-density on EEG in West syndrome

    M Oka, K Kobayashi, T Akiyama, T Ogino, E Oka

    BRAIN & DEVELOPMENT   26 ( 2 )   105 - 112   2004年3月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    To elucidate the pathophysiology of spasms in series, the distribution and density of spikes on hypsarrhythmia were studied in 13 patients with West syndrome by excluding slow waves from EEG using a digital filter. (1) Interictal spikes were mostly multifocal and dominant over the bilateral posterior head area with very few diffuse discharges. Therefore, spikes on hypsarrhythmia were demonstrated to occur actually depending on the brain regions although they appeared random. (2) The dominant region of interictal spikes did not correspond to the underlying focal cortical lesion in many patients, and was indicated to reflect the general process of cerebral maturation in infancy. (3) There were far fewer inter-spasm spikes, especially during the middle phase of a series of spasms, than interictal spikes. It was implied that the cortical activity of hypsarrhythmia was interfered with by the abnormal subcortical function, which might be related to the generation of spasms. (4) The dominant region of inter-spasm spikes tended to coincide with a focal cortical lesion shown by MRI and the focus of associated partial seizures. (C) 2003 Elsevier B.V. All rights reserved.

    DOI: 10.1016/S0387-7604(03)00101-3

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  • 側頭葉棘波の双極子分析の精度に関するシミュレーション

    小林勝弘, 吉永治美, 井上拓志, 大塚頌子, 岡鍈次

    臨床脳波   2004年

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  • Initiation of treatment and selection of antiepileptic drugs in childhood epilepsy

    E Oka, T Murakami, T Ogino, K Kobayashi, Ohmori, I, T Akiyama, M Ito

    EPILEPSIA   45   17 - 19   2004年

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    記述言語:英語   出版者・発行元:WILEY-BLACKWELL  

    Purpose: A retrospective study was carried out on 53 cases with childhood epilepsy to evaluate the validity of the initial selection of antiepileptic drug (AED).
    Methods: We investigated the AEDs selected at the beginning of the treatment from the medical records of 53 untreated cases. A follow-up study was undertaken to evaluate the effects of the AEDs. In the second study, we investigated the AEDs of 10 cases with atypical benign partial epilepsy (ABPE), to clarify whether the initial AEDs selected for rolandic epilepsy were related to the appearance of ABPE.
    Results: The AEDs used at the initial stage consisted of carbamazepine (CBZ), valproic acid (VPA), phenobarbital (PB), and vitamin B-6. The main AEDs were CBZ and VPA for localization-related epilepsy, and VPA for generalized epilepsy. The initial selection of AEDs in 41 (85.4%) of 48 cases treated with AEDs were considered to be correct from the results of follow-up. We could not specify any AEDs that related to the appearance of ABPE.
    Conclusions: The selection of AED in this series was considered to be most appropriate. We proposed a criterion to determine whether to begin the AED treatment immediately at the initial seizure.

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  • A simulation study of the error in dipole source localization for EEG spikes with a realistic head model

    K Kobayashi, H Yoshinaga, M Oka, Y Ohtsuka, J Gotman

    CLINICAL NEUROPHYSIOLOGY   114 ( 6 )   1069 - 1078   2003年6月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Objective: We tried to determine the error range of dipole modeling for EEG spikes originating from various clinically important sources by a simulation study employing a realistic head model. The computed error range was also compared with the degree of disturbance of dipole modeling caused by adding background activity to the spike.
    Methods: The scalp fields generated by temporal, frontal and rolandic epileptic sources with spatial extent were simulated, and the corresponding 3-dimensional maps of residual variance (RV) were built by computing the RV for a single dipole at each point on a fine imaginary grid in the brain. Single dipole modeling was also performed for the simulated scalp fields after adding real background activity,
    Results: The brain volume corresponding to a small RV was compact for the frontal sources and the lateral and baso-mesial temporal sources, and large for the anterior and baso-lateral temporal sources. The distribution of dipoles estimated for spikes contaminated with background corresponded to that of the volume of small RV and to spike-amplitude. Estimates were improved by employing inferior temporal electrodes.
    Conclusions: When evaluating dipole models of epileptic spikes, error ranges can be estimated and they vary considerably from region to region.
    Significance: This study illustrates the variability of the error in dipole modeling of epileptic spikes. This variability is important when considering the clinical interpretation of modeling results. (C) 2003 International Federation of Clinical Neurophysiology. Published by Elsevier Science Ireland Ltd. All rights reserved.

    DOI: 10.1016/S1388-2457(03)00064-6

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  • A simulation study of the error in dipole source localization for EEG spikes with a realistic head model

    K Kobayashi, H Yoshinaga, M Oka, Y Ohtsuka, J Gotman

    CLINICAL NEUROPHYSIOLOGY   114 ( 6 )   1069 - 1078   2003年6月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Objective: We tried to determine the error range of dipole modeling for EEG spikes originating from various clinically important sources by a simulation study employing a realistic head model. The computed error range was also compared with the degree of disturbance of dipole modeling caused by adding background activity to the spike.
    Methods: The scalp fields generated by temporal, frontal and rolandic epileptic sources with spatial extent were simulated, and the corresponding 3-dimensional maps of residual variance (RV) were built by computing the RV for a single dipole at each point on a fine imaginary grid in the brain. Single dipole modeling was also performed for the simulated scalp fields after adding real background activity,
    Results: The brain volume corresponding to a small RV was compact for the frontal sources and the lateral and baso-mesial temporal sources, and large for the anterior and baso-lateral temporal sources. The distribution of dipoles estimated for spikes contaminated with background corresponded to that of the volume of small RV and to spike-amplitude. Estimates were improved by employing inferior temporal electrodes.
    Conclusions: When evaluating dipole models of epileptic spikes, error ranges can be estimated and they vary considerably from region to region.
    Significance: This study illustrates the variability of the error in dipole modeling of epileptic spikes. This variability is important when considering the clinical interpretation of modeling results. (C) 2003 International Federation of Clinical Neurophysiology. Published by Elsevier Science Ireland Ltd. All rights reserved.

    DOI: 10.1016/S1388-2457(03)00064-6

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  • 小児難治てんかんの皮質局在性異常に伴う全般性異常についての臨床的研究

    小林勝弘

    てんかん治療研究振興財団研究年報 研究褒賞受賞記念報告   2003年

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  • 小児難治てんかんの皮質局在性異常に伴う全般性異常についての臨床的研究

    小林勝弘

    てんかん治療研究振興財団研究年報   2003年

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  • Systematic source estimation of spikes by a combination of independent component analysis and RAP-MUSIC I: Principles and simulation study

    K Kobayashi, T Akiyama, T Nakahori, H Yoshinaga, J Gotman

    CLINICAL NEUROPHYSIOLOGY   113 ( 5 )   713 - 724   2002年5月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Objectives: We propose a combination of independent component analysis (ICA) and recursively applied and projected multiple signal classification (RAP-MUSIC) as a new approach to dipole source estimation of epileptiform discharges. The method is minimally dependent on subjective decisions.
    Methods: Ten electroencephalographic (EEG) data matrices were generated by computer, each matrix including real background activity from a normal subject and an array of added simulated spikes. Each spike was a summation of two transients originating from slightly different 'original dipole sources'. The unaveraged EEG matrices were decomposed by ICA, and source estimation was performed by applying RAP-MUSIC to the spatial information defined by the ICA components showing epileptiform activity in their waveform. For comparison, dipoles were also estimated from the same matrices using two existing methods: RAP-MUSIC based on eigen-decomposition of the covariance matrices of averaged spikes and common spatial pattern decomposition.
    Results: In every simulated EEG data matrix, two dipoles close to the original sources were estimated by the present method. Their unaveraged activities were also similar to those of the original sources. The two existing methods gave less precise results than the proposed method.
    Conclusions: RAP-MUSIC based on ICA thus proved promising for source estimation of unaveraged epileptiform discharges. (C) 2002 Elsevier Science Ireland Ltd. All rights reserved.

    DOI: 10.1016/S1388-2457(02)00046-9

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  • Systematic source estimation of spikes by a combination of independent component analysis and RAP-MUSIC II: Preliminary clinical application

    K Kobayashi, T Akiyama, T Nakahori, H Yoshinaga, J Gotman

    CLINICAL NEUROPHYSIOLOGY   113 ( 5 )   725 - 734   2002年5月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Objectives: We tried to estimate epileptic sources by a combination of independent component analysis (ICA) and recursively applied and projected multiple signal classification (RAP-MUSIC) in real epileptiform EEG discharges.
    Methods: EEG data including an array of spikes from 3 patients were decomposed by ICA, and source estimation was performed by applying RAP-MUSIC to the spatial information defined by the set of ICA components that showed epileptiform activity in their waveform. Sources were also estimated from the same data using RAP-MUSIC based on eigen-decomposition of the covariance matrix of averaged spikes, and common spatial pattern decomposition for comparison.
    Results: RAP-MUSIC based on ICA could estimate generally correct epileptic sources in the 3 patients, and its results were better than those of the other methods, when compared to intracerebral data. The present analysis proceeded without introduction of subjective decision after data selection. The separation of epileptiform discharges from the background is essential for this analysis, and was successfully performed in the real EEG data.
    Conclusions: RAP-MUSIC based on ICA appears promising for estimation of epileptic sources with minimal dependence on subjective decisions in the process of analysis. In particular, it was not necessary to select the number of sources. (C) 2002 Elsevier Science Ireland Ltd. All rights reserved.

    DOI: 10.1016/S1388-2457(02)00047-0

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  • The relationship between paroxysmal kinesigenic choreoathetosis and epilepsy

    Ohmori, I, Y Ohtsuka, T Ogino, H Yoshinaga, K Kobayashi, E Oka

    NEUROPEDIATRICS   33 ( 1 )   15 - 20   2002年2月

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    記述言語:英語   出版者・発行元:GEORG THIEME VERLAG KG  

    Purpose: To clarify the relationship between paroxysmal kinesigenic choreoathetosis (PKC) and epilepsy, we investigated the clinical and electroencephalographic (EEG) findings of patients with familial PKC and epilepsy, as well as sporadic cases with both PKC and epilepsy.
    Patients and Methods: Patients consisted of 12 familial cases from seven families and three sporadic cases. The period of follow-up ranged from 17 months to 33 years, 7 months (average: 16 years, 8 months). During the follow-up, a total of 163 EEGs (11 EEGs per subject) were studied, including interictal and ictal EEGs.
    Results: Transient epileptic discharges were found in ten of the 15 patients (66.7%) during the clinical course. As for focus, centro-midtemporal and frontal spikes were most often observed. The ictal EEG of an afebrile convulsion in one patient showed a partial seizure with secondary generalization which originated from the frontal area.
    Conclusions: It appears that patients who suffer from both PKC and epilepsy have a functional abnormality of the cerebral cortex, particularly in the perirolandic and frontal regions.

    DOI: 10.1055/s-2002-23594

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  • Spasms in clusters in epilepsies other than typical West syndrome

    Y Ohtsuka, K Kobayashi, T Ogino, E Oka

    BRAIN & DEVELOPMENT   23 ( 7 )   473 - 481   2001年11月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features Group 2: generalized epilepsies with spasms in clusters at ages of 2-3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group I often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox-Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups I and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes. (C) 2001 Elsevier Science B.V. All rights reserved.

    DOI: 10.1016/S0387-7604(01)00289-3

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  • Clinical spectrum of epileptic spasms associated with cortical malformation

    K Kobayashi, Y Ohtsuka, S Ohno, Ohmori, I, T Ogino, H Yoshinaga, A Tanaka, Y Hiraki, E Oka

    NEUROPEDIATRICS   32 ( 5 )   236 - 244   2001年10月

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    記述言語:英語   出版者・発行元:GEORG THIEME VERLAG KG  

    sThe spectrum of clinico-electrical characteristics of epileptic spasms associated with cortical malformation was studied in detail. The subjects were 15 patients suffering from spasms and cortical malformation demonstrated by MRI. The types of cortical malformation causing spasms were various, including hemimegalencephaly, diffuse pachygyria, focal cortical dysplasia, and polymicrogyria. Ohtahara syndrome was diagnosed in 3 patients, and West syndrome in 8. Symptomatic localization-related epilepsy preceded West syndrome in 4 patients, and a transition from Ohtahara syndrome to West syndrome was observed in one. West syndrome was followed by symptomatic generalized epilepsy including Lennox-Gastaut syndrome in 4 patients. Nine patients showed a condition which was labeled "epilepsy with partial seizures and spasms" (EPS) and characterized by the coexistence of partial seizures and spasms, and multifocal epileptic discharges on EEG. Spasms occurred only as EPS in 5 patients. EPS appeared following Ohtahara syndrome or West syndrome in 4 patients, and showed a transition to symptomatic localization-related epilepsy in 4. However, EPS did not evolve into generalized epilepsy, and persisted until the time of last follow-up in 5 patients. Therefore, the clinico-electrical pictures of patients with spasms and cortical malformation were diverse and not always limited within those of typical generalized epilepsy.

    DOI: 10.1055/s-2001-19117

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  • Utility of the scalp-recorded ictal EEG in childhood epilepsy

    H Yoshinaga, J Hattori, H Ohta, T Asano, T Ogino, K Kobayashi, E Oka

    EPILEPSIA   42 ( 6 )   772 - 777   2001年6月

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    記述言語:英語   出版者・発行元:LIPPINCOTT WILLIAMS & WILKINS  

    Purpose: To evaluate the usefulness of the scalp-recorded ictal EEGs in diagnosing childhood epilepsy.
    Methods: We analyzed the ictal EEGs of 259 seizures in 183 patients who visited the department of child neurology, Okayama University Medical School. during the past 6 years.
    Results: We divided all seizures into the following four categories, according to the diagnostic usefulness of ictal EEGs in determining the seizure type: 1. (a) Ictal EEGs confirmed the diagnosis of the seizure type based on seizure symptoms (101 seizures): (b) Ictal EEGs aided in the classification of the seizure type based on the seizure symptoms (101 seizures): (c) Ictal EEGs corrected errors in the classification (37 seizures): and (d) Ictal EEGs revealed previously unreported/undocumented seizure type (20 seizures). 2. Of the 37 misdiagnosed seizures (group C), 11 were nonepileptic seizures misdiagnosed as epileptic seizures, eight were complex partial seizures (CPS) misdiagnosed as the other seizure types, and 10 were other seizure types misdiagnosed as CPSs. 3. Of the 20 previously unreported/undocumented seizures (group D), nine were myoclonic seizures, five were absence seizures, five were CPS, and one was tonic spasms. 4. Seventy-two patients had CPS. Among them, 11 patients showed no epileptic spikes in their interictal EEG recordings. Therefore, ictal recordings confirmed the diagnosis of epilepsy.
    Conclusions: Ictal EEG recording is a very useful diagnostic tool not only for determining seizure types, but also for uncovering the existence of the unsuspected seizure types. It supplies the physician with useful information for the classification and the treatment of epilepsy. In particular, ictal EEGs are useful in diagnosing patients with CPS.

    DOI: 10.1046/j.1528-1157.2001.37100.x

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  • Separation of spikes from background by independent component analysis with dipole modeling and comparison to intracranial recording

    K Kobayashi, Merlet, I, J Gotman

    CLINICAL NEUROPHYSIOLOGY   112 ( 3 )   405 - 413   2001年3月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Objective: Epileptiform discharges can be objectively separated from the EEG background by independent component analysis (ICA) into the discharge's waveform and its spatial distribution. The correspondence between ICA components, including epileptiform transients extracted from the scalp EEG and intracranial epileptic fields, was investigated.
    Methods: In 11 spike patterns from 8 patients, the scalp EEG data were decomposed by ICA. The corresponding averaged intracranial data were compared with the extracted epileptic components regarding the number of source patterns and source locations estimated from ICA maps.
    Results: Clear epileptic components could be separated in 10/11 spike patterns. The number of epileptic components was identical to the number of intracranial field peaks in 7 spike patterns with simple intracranial fields, and was less in the remaining 3 patterns with complex intracranial peaks. The distance between the contact of the maximal intracranial field and the dipole location estimated by the single dipole model for the dearest epileptic component ranged from 4.7 to 31.9 mm.
    Conclusions: The number of epileptic ICA components largely matched the number of intracranial field patterns, and the dipole location estimated for the map of the clearest epileptic component was generally correct. This establishes the validity of epileptic components extracted by ICA from the scalp background. (C) 2001 Elsevier Science Ireland Ltd. All rights reserved.

    DOI: 10.1016/S1388-2457(01)00457-6

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  • Predictors and underlying causes of medically intractable localization-related epilepsy in childhood

    Y Ohtsuka, H Yoshinaga, K Kobayashi, N Murakami, Y Yamatogi, E Oka, T Tsuda

    PEDIATRIC NEUROLOGY   24 ( 3 )   209 - 213   2001年3月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE INC  

    The goal of this study is to clarify the prognostic factors in childhood localization-related epilepsy in a tertiary medical center. Children (n = 113) with symptomatic and cryptogenic localization-related epilepsy were divided into groups of intractable patients (average seizure frequency: one or more per month during the 6 months before the last follow-up; n = 40) and well-controlled patients (no seizures for at least 1 year before the last follow-up; n = 73), Clinical and electroencephalogram (EEG) factors were examined to elucidate prognostic factors. The subtypes of epilepsies and causes were also investigated. Univariate analyses indicated that the following factors were correlated with seizure outcome: (1) seizure type at the first visit; (2) seizure frequency; (3) underlying cause; (4) age at onset of epilepsy; (5) status epilepticus occurring as the first seizure and before the first visit; and (6) diffuse epileptic discharges on first visit interictal EEGs, Multivariate analyses revealed that seizure type at the first visit, seizure frequency, status epilepticus before the first visit, and underlying causes were significant independent predictive factors. The rate of intractable patients was highest in multilobar epilepsy, followed by frontal-lobe epilepsy. Regarding etiologies, the intractable group contained nine patients with encephalitis of unknown origin and three each with localized cortical malformation and mesial temporal sclerosis, (C) 2001 by Elsevier Science Inc. All rights reserved.

    DOI: 10.1016/S0887-8994(00)00269-1

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  • Electroencephalographic changes and their regional differences during pediatric cardiovascular surgery with hypothermia

    T Akiyama, K Kobayashi, T Nakahori, H Yoshinaga, T Ogino, Y Ohtsuka, M Takeuchi, K Morita, S Sano, E Oka

    BRAIN & DEVELOPMENT   23 ( 2 )   115 - 121   2001年3月

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    記述言語:英語   出版者・発行元:ELSEVIER SCIENCE BV  

    Monitoring brain function by EEG is an important means of preventing cerebral insults in pediatric cardiovascular surgery. Vile studied intraoperative EEGs changes and their regional differences associated with hypothermia and brain ischemia. The subjects of this study consisted of 13 children ranging in age from 4 months to 4 years and 6 months. Multi-channel EEGs were recorded using a portable digital EEG system, and the EEG changes were examined by visual inspection and computerized analyses. The results were as follows. (1) During cooling, a discontinuous EEG pattern was transiently observed in four patients, and this phenomenon indicated rapid suppression of cerebral function and subsequent adaptation. (2) Regarding the patterns of change in equivalent potentials induced by hypothermia: there were two different patterns depending on the degree of hypothermia, and the borderline rectal temperature was found to be around 32 degreesC. (3) During cooling, regional differences in the changes in equivalent potentials were observed in nine patients. A decrease in slow waves was marked in the occipital head area, and a decrease in fast waves was prominent in the anterior head area. (4) Arterial hypotension caused transient EEC abnormalities. Of them, bilaterally synchronous rhythmic high voltage slow waves were remarkable and exhibited bifrontal or bicentral dominance. (5) The EEG changes induced by hypothermia were influenced not only by the rectal temperature itself, hut also by the rate of change in rectal temperature, and we speculated that this phenomenon was a result of adaptation. In intraoperative EEG monitoring, these findings constitute the basis fur early detection of a cerebral hypoxic-ischemic stare during pediatric cardiovascular surgery. (C) 2001 Elsevier Science B.V. All rights reserved.

    DOI: 10.1016/S0387-7604(01)00192-9

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  • Age-related clinical and neurophysiologic characteristics of intractable epilepsy associated with cortical malformation

    K Kobayashi, Y Ohtsuka, S Ohno, A Tanaka, Y Hiraki, E Oka

    EPILEPSIA   42   24 - 28   2001年

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    記述言語:英語   出版者・発行元:BLACKWELL PUBLISHING INC  

    Purpose: To elucidate the relationship between the characteristics of cortical malformation (CM) and those of associated epilepsy, and also to investigate the prognostic value of the clinical and magnetic resonance imaging (MRI) findings for the seizure and mental outcome.
    Methods: We studied 41 patients with CM and epilepsy, and the patients were divided according to the age at onset of epilepsy into two groups: one group of 15 patients with very early onset before age 3 months, and the other group of 26 patients with onset at 3 months or later. Statistical relationship was examined between the types of dysplastic lesions demonstrated by MRI and the age at onset of epilepsy. The effects of the onset age and the features of CM on the outcome of seizures and mental or developmental state also were analyzed.
    Results: The very early-onset epilepsy was related to the dysplastic patterns of reduced sulci and blurred cortical-subcortical junction, which suggested focal CM, whereas the later-onset epilepsy was related to polymicrogyria. The age at onset of epilepsy was related to the poor seizure outcome, and both the onset age and wide distribution of CM were related to severe retardation.
    Conclusions: The type of CM influences the expression of associated epilepsy, especially its age-related features. The age at onset of epilepsy plays an important role in the seizure and mental outcome.

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  • 焦点性てんかん発射の二次性広汎化過程に関する研究

    王紀文, 小林勝弘, 荻野竜也, 服部旬里, 岡牧郎, 吉永治美, 大塚頌子, 岡えい次

    臨床脳波   2001年

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  • 「てんかん症候群の国際分類(1989)」による小児てんかんの神経疫学的研究?岡山県における調査?

    岡えい次, 大塚頌子, 吉永治美, 小林勝弘, 荻野竜也

    てんかん治療研究振興財団研究年報   2001年

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  • Myoclonic seizures combined with partial seizures and probable pathophysiology of secondary bilateral synchrony

    K. Kobayashi, S. Maniwa, T. Ogino, H. Yoshinaga, Y. Ohtsuka, E. Oka

    Clinical Neurophysiology   111 ( 10 )   1813 - 1816   2000年10月

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    記述言語:英語  

    Objective: We report on a patient who showed an unusual transition from complex partial seizures to a combination of myoclonic seizures and partial seizures, and then to isolated myoclonic seizures. The pathophysiological mechanism of the myoclonic seizures in this uncommon condition was studied. Methods: The interhemispheric small time differences were estimated in the ictal EEG discharges of myoclonic seizures for differentiation between primary and secondary bilateral synchrony. The estimation was performed by coherence and phase analysis based on the two-dimensional autoregressive model. Results: The estimated interhemispheric time differences were at most 27.4 ms. The ictal activity of the myoclonic seizures was suggested to originate from a cortical focus in the right hemisphere, which was also the origin of the partial seizures. Conclusion: The patient was considered to have an unusual type of myoclonic seizures with a probable pathophysiological mechanism of secondary bilateral synchrony. © 2000 Elsevier Science Ireland Ltd.

    DOI: 10.1016/S1388-2457(00)00390-4

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  • Myoclonic seizures combined with partial seizures and probable pathophysiology of secondary bilateral synchrony

    K. Kobayashi, S. Maniwa, T. Ogino, H. Yoshinaga, Y. Ohtsuka, E. Oka

    Clinical Neurophysiology   111 ( 10 )   1813 - 1816   2000年10月

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    記述言語:英語  

    Objective: We report on a patient who showed an unusual transition from complex partial seizures to a combination of myoclonic seizures and partial seizures, and then to isolated myoclonic seizures. The pathophysiological mechanism of the myoclonic seizures in this uncommon condition was studied. Methods: The interhemispheric small time differences were estimated in the ictal EEG discharges of myoclonic seizures for differentiation between primary and secondary bilateral synchrony. The estimation was performed by coherence and phase analysis based on the two-dimensional autoregressive model. Results: The estimated interhemispheric time differences were at most 27.4 ms. The ictal activity of the myoclonic seizures was suggested to originate from a cortical focus in the right hemisphere, which was also the origin of the partial seizures. Conclusion: The patient was considered to have an unusual type of myoclonic seizures with a probable pathophysiological mechanism of secondary bilateral synchrony. © 2000 Elsevier Science Ireland Ltd.

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  • Myoclonic seizures combined with partial seizures and probable pathophysiology of secondary bilateral synchrony 査読

    Clin Neurophysiol   111 ( 10 )   1813 - 1816   2000年10月

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    担当区分:筆頭著者, 責任著者   記述言語:英語  

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  • Using weighted linear spatial decomposition to investigate brain activity through a set of fixed current dipoles

    CJ James, K Kobayashi, J Gotman

    CLINICAL NEUROPHYSIOLOGY   111 ( 5 )   773 - 780   2000年5月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Objectives: We developed a method with the aim of decorrelating scalp EEG based on a set of spatial constraints.
    Methods: We assume that the scalp EEG can be modelled by a small number of current dipoles of fixed location and orientation, placed at regions of interest. The algorithm is based on weighted linear spatial decomposition in order to obtain a weighted solution to the inverse problem. An EEG data matrix is first weighted in favour of a single dipole in the set. The dipole moment is then calculated from the weighted EEG by the pseudo-inverse method. This is repeated for each dipole.
    Results: Six seizures were processed from 4 patients using the standard least-squares solution and our weighted version. The average cross-correlation between channels was calculated for each case. The first method resulted in a mean drop in cross-correlation of 16.5% from that of the scalp. Our method resulted in a reduction of 34.5%.
    Conclusions: Our method gives a more spatially decorrelated signal in regions of interest (although it is not intended as an accurate localization tool). Subsequent analysis is more robust and less likely to be dependent on specific recording montages. This is more than could be obtained using a standard least-squares solution using the same model. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.

    DOI: 10.1016/S1388-2457(99)00316-8

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  • The electroencephalogram through a software microscope: Non-invasive localization and visualization of epileptic seizure activity from inside the brain

    K. Kobayashi, C. J. James, H. Yoshinaga, Y. Ohtsuka, J. Gotman

    Clinical Neurophysiology   111 ( 1 )   134 - 149   2000年1月

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    記述言語:英語  

    Objective: We developed a novel non-invasive analysis to localize the source and visualize the time course of electrical activity generated inside the brain but unclear from the scalp. This analysis applies to signals with unique waveform characteristics, such as seizures. Methods: The method extracts activity from an EEG data matrix as a spatiotemporal component having waveforms uncorrelated to the other concurrent activities. The method also provides the location and orientation of the dipole generating this activity. We applied this method to ten scalp seizures in three patients with temporal lobe epilepsy and single-focus seizures confirmed by intracerebral recordings. A realistic head model based on MRI was used for computation of field distributions. Results: When seizure activity was still not visually identifiable on the scalp, the method demonstrated in all scalp seizures a source in the temporal neocortex corresponding clearly to the region of seizure activity in intracerebral recordings. Frequency characteristics of the estimated activities also resembled those of the intracerebral seizures. Conclusions: This method enables estimation of focal brain activity when its effect on scalp EEG is unclear to visual examination. It works in situations where currently available source analyses methods, which require noiseless definite activity, are not applicable. Copyright (C) 2000 Elsevier Science Ireland Ltd.

    DOI: 10.1016/S1388-2457(99)00202-3

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  • The electroencephalogram through a software microscope: non-invasive localization and visualization of epileptic seizure activity from inside the brain

    K Kobayashi, CJ James, H Yoshinaga, Y Ohtsuka, J Gotman

    CLINICAL NEUROPHYSIOLOGY   111 ( 1 )   134 - 149   2000年1月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Objective: We developed a novel non-invasive analysis to localize the source and visualize the time course of electrical activity generated inside the brain but unclear from the scalp. This analysis applies to signals with unique waveform characteristics, such as seizures.
    Methods: The method extracts activity from an EEG data matrix as a spatiotemporal component having waveforms uncorrelated to the other concurrent activities. The method also provides the location and orientation of the dipole generating this activity. We applied this method to ten scalp seizures in three patients with temporal lobe epilepsy and single-focus seizures confirmed by intracerebral recordings. A realistic head model based on MRI was used for computation of field distributions.
    Results: When seizure activity was still not visually identifiable on the scalp, the method demonstrated in all scalp seizures a source in the temporal neocortex corresponding clearly to the region of seizure activity in intracerebral recordings. Frequency characteristics of the estimated activities also resembled those of the intracerebral seizures.
    Conclusions: This method enables estimation of focal brain activity when its effect on scalp EEG is unclear to visual examination. It works in situations where currently available source analyses methods, which require noiseless definite activity, are not applicable. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.

    DOI: 10.1016/S1388-2457(99)00202-3

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  • Refractory childhood epilepsy and factors related to refractoriness

    Y Ohtsuka, H Yoshinaga, K Kobayashi

    EPILEPSIA   41   14 - 17   2000年

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    記述言語:英語   出版者・発行元:LIPPINCOTT WILLIAMS & WILKINS  

    To clarify the characteristics of refractory childhood epilepsy, we compared recent refractory cases and those of approximately 15 years ago, all of which were seen at the Okayama University Hospital. We also analyzed predictive factors related to refractoriness in the recent refractory cases. Among the recent refractory cases, the proportion of localization-related epilepsies increased, and the proportion of generalized epilepsies decreased compared to historical cases. In generalized epilepsies, the proportion of cases with Lennox Gastaut syndrome decreased to less than half. In localization-related epilepsies, the proportion of cases with frontal lobe epilepsy increased. The proportion of cases with unknown causes decreased to less than half. Of the cases with known causes, postencephalitis/postencephalapathy and focal cortical malformation. including tuberous sclerosis, accounted for most of the cases. Mort of the recent refractory cases were treated with high-dose AED monotherapy. compared to more polytherapy in the cases of 15 years earlier. The following factors were related to future refactoriness: less than 1 year of age at onset of seizures, the presence of underlying pathology, status epilepticus, changes in type of epilepsy during the clinical course, and neonatal seizures. Regarding EEG findings of cases that had localization-related epilepsies at the end of follow-up, focal spike-waves associated with diffuse spike-waves on the first EEGs indicated future refractoriness.

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  • Isolation of epileptiform discharges from unaveraged EEG by independent component analysis

    K. Kobayashi, C. J. James, T. Nakahori, T. Akiyama, J. Gotman

    Clinical Neurophysiology   110 ( 10 )   1755 - 1763   1999年10月

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    記述言語:英語  

    Objective: We propose a method that allows the separation of epileptiform discharges (EDs) from the EEG background, including the ED's waveform and spatial distribution. The method even allows to separate a spike in two components occurring at approximately the same time but having different waveforms and spatial distributions. Methods: The separation employs independent component analysis (ICA) and is not based on any assumption regarding generator model. A simulation study was performed by generating ten EEG data matrices by computer: each matrix included real background activity from a normal subject to which was added an array of simulated unaveraged EDs. Each discharge was a summation of two transients having slightly different potential field distributions and small jitters in time and amplitude. Real EEG data were also obtained from three epileptic patients. Results: Through ICA, we could isolate the two epileptiform transients in every simulation matrix, and the retrieved transients were almost identical as the originals, especially in their spatial distributions. Two epileptic components were isolated by ICA in all patients. Each estimated epileptic component had a consistent time course. Conclusion: ICA appears promising for the separation of unaveraged spikes from the EEG background and their decomposition in independent spatio-temporal components.

    DOI: 10.1016/S1388-2457(99)00134-0

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  • Isolation of epileptiform discharges from unaveraged EEG by independent component analysis

    K. Kobayashi, C. J. James, T. Nakahori, T. Akiyama, J. Gotman

    Clinical Neurophysiology   110 ( 10 )   1755 - 1763   1999年10月

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    記述言語:英語  

    Objective: We propose a method that allows the separation of epileptiform discharges (EDs) from the EEG background, including the ED's waveform and spatial distribution. The method even allows to separate a spike in two components occurring at approximately the same time but having different waveforms and spatial distributions. Methods: The separation employs independent component analysis (ICA) and is not based on any assumption regarding generator model. A simulation study was performed by generating ten EEG data matrices by computer: each matrix included real background activity from a normal subject to which was added an array of simulated unaveraged EDs. Each discharge was a summation of two transients having slightly different potential field distributions and small jitters in time and amplitude. Real EEG data were also obtained from three epileptic patients. Results: Through ICA, we could isolate the two epileptiform transients in every simulation matrix, and the retrieved transients were almost identical as the originals, especially in their spatial distributions. Two epileptic components were isolated by ICA in all patients. Each estimated epileptic component had a consistent time course. Conclusion: ICA appears promising for the separation of unaveraged spikes from the EEG background and their decomposition in independent spatio-temporal components.

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  • Isolation of epileptiform discharges from unaveraged EEG by independent component analysis

    K Kobayashi, CT James, T Nakahori, T Akiyama, J Gotman

    CLINICAL NEUROPHYSIOLOGY   110 ( 10 )   1755 - 1763   1999年10月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Objective: We propose a method that allows the separation of epileptiform discharges (EDs) from the EEG background, including the ED's waveform and spatial distribution. The method even allows to separate a spike in two components occurring at approximately the same time but having different waveforms and spatial distributions.
    Methods: The separation employs independent component analysis (ICA) and is not based on any assumption regarding generator model. A simulation study was performed by generating ten EEG data matrices by computer: each matrix included real background activity from a normal subject to which was added an array of simulated unaveraged EDs. Each discharge was a summation of two transients having slightly different potential field distributions and small jitters in time and amplitude, Real EEG data were also obtained from three epileptic patients.
    Results: Through ICA, we could isolate the two epileptiform transients in every simulation matrix, and the retrieved transients were almost identical as the originals, especially in their spatial distributions. Two epileptic components were isolated by ICA in all patients. Each estimated epileptic component had a consistent time course.
    Conclusion: ICA appears promising for the separation of unaveraged spikes from the EEG background and their decomposition in independent spatio-temporal components. (C) 1999 Elsevier Science Ireland Ltd. All rights reserved.

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  • Estimation of obscure ictal epileptic activity in scalp EEG

    Katsuhiro Kobayashi, Tomoyuki Nakahori, Iori Ohmori, Harumi Yoshinaga, Yoko Otsuka, Eiji Oka, Shunsuke Ohtahara

    Brain Topography   9 ( 2 )   125 - 134   1996年

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    記述言語:英語  

    We have produced a method to estimate ictal localized epileptic activity hidden among the background in scalp EEGs. When the visually completely different waveforms of the epileptic and background activities are nearly orthogonal, epileptic activity may be approximately extracted from the EEG data matrix by singular value decomposition with subsequent orthogonal rotation to match the distribution of one component with that of the epileptic source. A simulation study was carried out using a matrix mimicking the scalp EEG with an inconspicuous ictal epileptic activity from a dipole source. This hidden epileptic activity was approximately recovered by matching the dipole of interest with the epileptic dipole, even when the simulated waveforms of the epileptic and background activities were not exactly orthogonal. High linear correlation between these two types of waveforms hampered the recovery of the epileptic activity. In another simulation study employing two epileptic dipoles producing activities with the same waveform and a brief time lag, it was indicated that the temporal relationship between the epileptic activities could be also estimated using the cross-correlation function. In the preliminary clinical application of this method to the ictal EEGs of complex partial seizures, rhythmic activities with seemingly epileptic waveforms were estimated at the dipoles which were located in the vicinity of cortical lesions revealed by neuroimaging studies. These activities were indicated to appear before any change in the scalp EEG. We hope for the clinical application of this method for noninvasive estimation of inconspicuous ictal epileptic activity.

    DOI: 10.1007/BF01200712

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  • 脳波上の両側同期性棘徐波における両半球間微小時間差と '脳梁部分離断術シミュレーション'

    小林勝弘, 小川和則, 大塚頌子, 岡鍈次, 大田原俊輔

    臨床脳波   37 ( 1 )   17 - 21   1995年1月

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    担当区分:筆頭著者, 責任著者  

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  • Epilepsy with Electrical Status Epileptics During Slow Sleep and Secondary Bilateral Synchrony 査読

    Kobayashi K, Nishibayashi N, Ohtsuka Y, Oka E, Ohtahara S

    Epilepsia   35 ( 5 )   1097 - 1103   1994年5月

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    担当区分:筆頭著者, 責任著者   記述言語:英語  

    Summary: In 3 children with “epilepsy with electrical status epilepticus during slow sleep” (ESES), we estimated interhemispheric small time differences (TDs) during spike‐wave activity in EEG by coherence and phase analysis by the two‐dimensional autoregressive model to differentiate their continuous diffuse spike‐waves during slow‐wave sleep (CSWS) between primary bilateral synchrony and secondary bilateral synchrony (SBS). Maximal TDs at onset of apparently bilateral synchronous spike‐wave bursts (BSSWs) during slow‐wave sleep were 12·0–26·5 ms (mean 20·3 ms) with consistent leading hemispheres in eight bursts of the 3 patients, indicating SBS as pathophysiology of their CSWS. This suggestion was supported by their clinico‐EEG findings, including the effect of a single oral dose of clobazam (CLB) on EEG. Three ictal BSSWs of atypical absence seizures in 2 patients were also analyzed to obtain maximal TDs of 17·9–41·7 ms (mean 26·3 ms) at onset, with the same leading sides as in sleep, also indicating SBS. Examination of intraburst TD variations showed no consistent disappearance of TDs during the latter part of the bursts, in either sleep or the ictal EEGs of atypical absences, and a role of the corpus callosum was suggested in the generation of SBS in ESES. Copyright © 1994, Wiley Blackwell. All rights reserved

    DOI: 10.1111/j.1528-1157.1994.tb02561.x

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  • Epilepsy with Electrical Status Epilepticus During Slow Sleep and Secondary Bilateral Synchrony

    Katsuhiro Kobayashi, Naohiro Nishibayashi, Yoko Ohtsuka, Eiji Oka, Shunsuke Ohtahara

    Epilepsia   35 ( 5 )   1097 - 1103   1994年

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    記述言語:英語  

    Summary: In 3 children with “epilepsy with electrical status epilepticus during slow sleep” (ESES), we estimated interhemispheric small time differences (TDs) during spike‐wave activity in EEG by coherence and phase analysis by the two‐dimensional autoregressive model to differentiate their continuous diffuse spike‐waves during slow‐wave sleep (CSWS) between primary bilateral synchrony and secondary bilateral synchrony (SBS). Maximal TDs at onset of apparently bilateral synchronous spike‐wave bursts (BSSWs) during slow‐wave sleep were 12·0–26·5 ms (mean 20·3 ms) with consistent leading hemispheres in eight bursts of the 3 patients, indicating SBS as pathophysiology of their CSWS. This suggestion was supported by their clinico‐EEG findings, including the effect of a single oral dose of clobazam (CLB) on EEG. Three ictal BSSWs of atypical absence seizures in 2 patients were also analyzed to obtain maximal TDs of 17·9–41·7 ms (mean 26·3 ms) at onset, with the same leading sides as in sleep, also indicating SBS. Examination of intraburst TD variations showed no consistent disappearance of TDs during the latter part of the bursts, in either sleep or the ictal EEGs of atypical absences, and a role of the corpus callosum was suggested in the generation of SBS in ESES. Copyright © 1994, Wiley Blackwell. All rights reserved

    DOI: 10.1111/j.1528-1157.1994.tb02561.x

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  • PRIMARY AND SECONDARY BILATERAL SYNCHRONY IN EPILEPSY - DIFFERENTIATION BY ESTIMATION OF INTERHEMISPHERIC SMALL TIME DIFFERENCES DURING SHORT SPIKE-WAVE ACTIVITY

    K KOBAYASHI, Y OHTSUKA, E OKA, S OHTAHARA

    ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY   83 ( 2 )   93 - 103   1992年8月

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    記述言語:英語   出版者・発行元:ELSEVIER SCI IRELAND LTD  

    Estimation of interhemispheric small time differences (TDs) during spike-wave bursts in the EEG by coherence and phase analysis is useful for differentiation between primary bilateral synchrony (PBS) and secondary bilateral synchrony (SBS) in epilepsy. Because the previous method via Fast Fourier Transform needed long bursts for reliable analysis, a method using a 2-dimensional autoregressive model was newly developed to enable estimation of TDs even in 1.2 sec bursts, and applied to 19 epileptic patients with apparently bilaterally synchronous spike-wave bursts.
    At the onsets of bursts, estimated maximal TDs were 5.8 msec or less and inconsistent in leading hemispheres in 10 patients with a clinical diagnosis of idiopathic, cryptogenic or symptomatic generalized epilepsy indicating PBS, while the maximal TDs were 9.3-41.5 msec and consistent in leading in 7 patients with clinically symptomatic partial epilepsy and also in two with idiopathic and symptomatic generalized epilepsy suggesting SBS.
    Among 8 patients with bursts which suggested SBS and long enough for evaluation of intra-burst TD variation, TDs tended to disappear in the middle to end parts of the bursts in 5 cases, but not in the other 3, suggesting 2 different pathophysiological mechanisms in SBS.

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  • Primary and secondary bilateral synchrony in epilepsy: differentiation by estimation of interhemispheric small time differences during short spike-wave activity

    K. Kobayashi, Y. Ohtsuka, E. Oka, S. Ohtahara

    Electroencephalography and Clinical Neurophysiology   83 ( 2 )   93 - 103   1992年

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    記述言語:英語  

    Estimation of interhemispheric small time differences (TDs) during spike-wave bursts in the EEG by coherence and phase analysis is useful for differentiation between primary bilateral synchrony (PBS) and secondary bilateral synchrony (SBS) in epilepsy. Because the previous method via Fast Fourier Transform needed long bursts for reliable analysis, a method using a 2-dimensional autoregressive model was newly developed to enable estimation of TDs even in 1.2 sec bursts, and applied to 19 epileptic patients with apparently bilaterally synchronous spike-wave bursts. At the onsets of bursts, estimated maximal TDs were 5.8 msec or less and inconsistent in leading hemispheres in 10 patients with a clinical diagnosis of idiopathic, cryptogenic or symptomatic generalized epilepsy indicating PBS, while the maximal TDs were 9.3-41.5 msec and consistent in leading in 7 patients with clinically symptomatic partial epilepsy and also in two with idiopathic and symptomatic generalized epilepsy suggesting SBS. Among 8 patients with bursts which suggested SBS and long enough for evaluation of intra-burst TD variation, TDs tended to disappear in the middle to end parts of the bursts in 5 cases, but not in the other 3, suggesting 2 different pathophysiological mechanisms in SBS. © 1992.

    DOI: 10.1016/0013-4694(92)90022-A

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  • Longitudinal Clinicoelectrophysiologic Study of a Case of Lafora Disease Proven by Skin Biopsy

    Katsuhiro Kobayashi, Kuniaki Iyoda, Yoko Ohtsuka, Shunsuke Ohtahara, Michio Yamada

    Epilepsia   31 ( 2 )   194 - 201   1990年

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    記述言語:英語  

    Summary A longitudinal clinicoelectrophysiologic study was undertaken of a 15‐year 2‐month‐old girl with Lafora disease who was diagnosed by skin biopsy and an immunohistochemical method with antisera against Lafora bodies. From age 10 years 5 months, 4 months after onset, EEG disclosed progressive deterioration of background activity and incremental increase in epileptic discharges. Photosensitivity was unique: Occipital spikes and diffuse spike‐wave discharges were provoked by low‐frequency repetitive photic stimuli but without elicitation of myoclonic seizures. Photosensitivity completely disappeared after age 13 years 10 months. High‐voltage somatosensory evoked potentials (SEPs) and high‐voltage flash visual evoked potentials (F‐VEPs) were seen before age 13. After age 13, progressive prolongation of I‐III and I‐V interpeak latencies of auditory brainstem responses (ABRs), progressive prolongation of latencies of photoevoked eyelid microvibrations, delayed latencies of pattern‐reversal visual evoked potentials, and a decrease in the V/I amplitude ratio of ABRs and the previously high F‐VEP amplitudes were observed. Les auteurs ont suivi une jeune fille âgée de 15 ans et 2 mois, Présentant une maladie de Lafora diagnostiquée par biopsie cutanée et étude immunohistochimique au moyen d'un sérum réagissant sur les corps de Lafora. A partir de l'âge de 10 ans et 5 mois, 4 mois après le début de la maladie, l'EEG a mis en évidence une détérioration progressive de l'activité de fond et une augmentation progressive des décharges épileptiques. Une photosensibilité très particulière a été mise en évidence: des Pointes occipitales et des pointes‐ondes diffuses étaient provoquées par la stimulation lumineuse intermittente à basse fréquence, sans provoquer toutefois de crises myocloniques, cette photosensibilité a disparu complètement après l'âge de 13 ans et 10 mois. Des potentiels évoqués somato‐sensitifs de haut voltage et des potentiels évoqués visuels au flash de haut voltage ont été enregistrés avant l'âge de 13 ans. Après cet âge, les auteurs ont constaté un allongement progressif des latences interpics I‐III et I‐IV des potentiels évoqués auditifs précoces, un allongement progressif des latences des microvibrations palpébrales évoquées par la lumière, un allongement des latences des potentiels évoqués visuels par inversion de pattern, et une diminution de l'amplitude du rapport V/I des potentiels évoqués auditifs précoces et des amplitudes des potentiels évoqués visuels au flash. Se ha realizado un estudio longitudinal clínico‐electrofisiológico en una niña de 15 años y 2 meses de edad que padecía la enfermedad de Lafora diagnosticada mediante una biópsia cutánea y un método histoquímico con antisuero anticuerpos de Lafora. Desde la edad de 10 años y 5 meses, 4 meses después del comienzo de la enfermedad, aapareció un deterioro progresivo de la actividad de fondo con un incremento de las descargas epilépticas. La fotosensibilidad fue muy especial: puntas occipitales y descargas difusas punta‐onda fueron provocadas mediante estimulación fótica repetitiva efectiva, de baja frecuencia, pero sin que se observaran ataques mioclónicos. La fotosensibilidad desapareció completamente después de la edad de 13 años y 10 meses. Antes de la edad de 13 años se observaron potenciales evocados somatosensoriales de alto voltaje y potenciales evocados visuales (F‐VEPs) tras flash de alto voltaje. Después de la edad de 13 años se observaron: una progresiva prolongación de las latencias interpicos de I‐III y I‐V de las respuestas auditivas de tronco cerebral (APRs), una prolongación progresiva de las latencias de las vibraciones de los parpados evocadas fóticamente, un retraso de las latencias de los potenciales evocados visuales inducidos por patrones gráficos y una reducción de la relación V‐I de los ABRs y de las previas amplitudes de los F‐VEP. Eine klinisch‐elektrophysiologische Langzeitstudie wurde bei einem jetzt 15 Jahre alten Mädchen mit Lafora‐Erkrankung durch geführt. Die Diagnose war durch Hautbiopsie un durch immunhistochemische Methoden mit Antiseren gegen Laforakörper gestellt worden. Im Alter von 10,5 Jahren, 4 Monate nach Auftreten der Erkrankung, zeigte das EEG einen progredienten Abbau der Grundaktivität und allmähliche Zunahme epileptischer Entladungen. Die Fotosensibilität war auffällig: durch langsame repetitive Stimuli wurden occipitale spikes und diffuse spike‐waves Entladungen provoziert ohne Auslösung einer myoklonischen Reaktion. Im Alter von 13,10 Jahren verschwand die Fotosensibilität vollständig. Amplitudenhohe somatosensorische EP und hohe Blitz‐VEP wurden vor dem Alter von 13 Jahren und danach beobachtet. Die AEP zeigten allmähliche Verlängerung der I‐III und I‐V Latenzen, ferner eine Verlängerung der Licht‐evozierten Augenlid‐Mikrovibrationen und verzögerte Latenzen der VEP bei Musterumkehr. Ein Abfall der Amplitudenkorrelation V/1 der AEP und der Amplituden der F‐VEP wurde ebenfalls festgestellt. 1990 International League Against Epilepsy

    DOI: 10.1111/j.1528-1167.1990.tb06306.x

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  • Longitudinal Clinicoelectrophysiologic Study of a Case of Lafora Disease Proven by Skin Biopsy

    Katsuhiro Kobayashi, Kuniaki Iyoda, Yoko Ohtsuka, Shunsuke Ohtahara, Michio Yamada

    Epilepsia   31 ( 2 )   194 - 201   1990年

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    記述言語:英語  

    Summary A longitudinal clinicoelectrophysiologic study was undertaken of a 15‐year 2‐month‐old girl with Lafora disease who was diagnosed by skin biopsy and an immunohistochemical method with antisera against Lafora bodies. From age 10 years 5 months, 4 months after onset, EEG disclosed progressive deterioration of background activity and incremental increase in epileptic discharges. Photosensitivity was unique: Occipital spikes and diffuse spike‐wave discharges were provoked by low‐frequency repetitive photic stimuli but without elicitation of myoclonic seizures. Photosensitivity completely disappeared after age 13 years 10 months. High‐voltage somatosensory evoked potentials (SEPs) and high‐voltage flash visual evoked potentials (F‐VEPs) were seen before age 13. After age 13, progressive prolongation of I‐III and I‐V interpeak latencies of auditory brainstem responses (ABRs), progressive prolongation of latencies of photoevoked eyelid microvibrations, delayed latencies of pattern‐reversal visual evoked potentials, and a decrease in the V/I amplitude ratio of ABRs and the previously high F‐VEP amplitudes were observed. Les auteurs ont suivi une jeune fille âgée de 15 ans et 2 mois, Présentant une maladie de Lafora diagnostiquée par biopsie cutanée et étude immunohistochimique au moyen d'un sérum réagissant sur les corps de Lafora. A partir de l'âge de 10 ans et 5 mois, 4 mois après le début de la maladie, l'EEG a mis en évidence une détérioration progressive de l'activité de fond et une augmentation progressive des décharges épileptiques. Une photosensibilité très particulière a été mise en évidence: des Pointes occipitales et des pointes‐ondes diffuses étaient provoquées par la stimulation lumineuse intermittente à basse fréquence, sans provoquer toutefois de crises myocloniques, cette photosensibilité a disparu complètement après l'âge de 13 ans et 10 mois. Des potentiels évoqués somato‐sensitifs de haut voltage et des potentiels évoqués visuels au flash de haut voltage ont été enregistrés avant l'âge de 13 ans. Après cet âge, les auteurs ont constaté un allongement progressif des latences interpics I‐III et I‐IV des potentiels évoqués auditifs précoces, un allongement progressif des latences des microvibrations palpébrales évoquées par la lumière, un allongement des latences des potentiels évoqués visuels par inversion de pattern, et une diminution de l'amplitude du rapport V/I des potentiels évoqués auditifs précoces et des amplitudes des potentiels évoqués visuels au flash. Se ha realizado un estudio longitudinal clínico‐electrofisiológico en una niña de 15 años y 2 meses de edad que padecía la enfermedad de Lafora diagnosticada mediante una biópsia cutánea y un método histoquímico con antisuero anticuerpos de Lafora. Desde la edad de 10 años y 5 meses, 4 meses después del comienzo de la enfermedad, aapareció un deterioro progresivo de la actividad de fondo con un incremento de las descargas epilépticas. La fotosensibilidad fue muy especial: puntas occipitales y descargas difusas punta‐onda fueron provocadas mediante estimulación fótica repetitiva efectiva, de baja frecuencia, pero sin que se observaran ataques mioclónicos. La fotosensibilidad desapareció completamente después de la edad de 13 años y 10 meses. Antes de la edad de 13 años se observaron potenciales evocados somatosensoriales de alto voltaje y potenciales evocados visuales (F‐VEPs) tras flash de alto voltaje. Después de la edad de 13 años se observaron: una progresiva prolongación de las latencias interpicos de I‐III y I‐V de las respuestas auditivas de tronco cerebral (APRs), una prolongación progresiva de las latencias de las vibraciones de los parpados evocadas fóticamente, un retraso de las latencias de los potenciales evocados visuales inducidos por patrones gráficos y una reducción de la relación V‐I de los ABRs y de las previas amplitudes de los F‐VEP. Eine klinisch‐elektrophysiologische Langzeitstudie wurde bei einem jetzt 15 Jahre alten Mädchen mit Lafora‐Erkrankung durch geführt. Die Diagnose war durch Hautbiopsie un durch immunhistochemische Methoden mit Antiseren gegen Laforakörper gestellt worden. Im Alter von 10,5 Jahren, 4 Monate nach Auftreten der Erkrankung, zeigte das EEG einen progredienten Abbau der Grundaktivität und allmähliche Zunahme epileptischer Entladungen. Die Fotosensibilität war auffällig: durch langsame repetitive Stimuli wurden occipitale spikes und diffuse spike‐waves Entladungen provoziert ohne Auslösung einer myoklonischen Reaktion. Im Alter von 13,10 Jahren verschwand die Fotosensibilität vollständig. Amplitudenhohe somatosensorische EP und hohe Blitz‐VEP wurden vor dem Alter von 13 Jahren und danach beobachtet. Die AEP zeigten allmähliche Verlängerung der I‐III und I‐V Latenzen, ferner eine Verlängerung der Licht‐evozierten Augenlid‐Mikrovibrationen und verzögerte Latenzen der VEP bei Musterumkehr. Ein Abfall der Amplitudenkorrelation V/1 der AEP und der Amplituden der F‐VEP wurde ebenfalls festgestellt. 1990 International League Against Epilepsy

    DOI: 10.1111/j.1528-1167.1990.tb06306.x

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  • Studies on Photo-Evoked Eyelid Microvibration 1.2. Developmental Change

    Satoshi Sanada, Katsuhiro Kobayashi

    15 ( 1 )   36 - 41   1987年

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講演・口頭発表等

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    72nd American Epilepsy Society Meeting, Special Interest Group Meeting "Temporal Lobe Club SIG: Noninvasive recording of pathological high frequency oscillations"  2018年11月30日 

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  • 小児神経学の進歩と展開 ―小児てんかんを中心として―

    第 207 回倉敷小児科専門医会  2017年 

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  • てんかん診療と連携

    第 10 回 備州 Brain Conference  2017年 

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  • 乳幼児期の完全脳波記録実現への挑戦と課題~岡山大学病院での取り組み~

    全国てんかんセンター協議会2017  2017年 

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  • 外来ビデオ脳波で記録できた一過性言語障害の発作

    全国てんかんセンター協議会2017  2017年 

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  • 岡山県におけるてんかん診療連携の進展

    全国てんかんセンター協議会2017  2017年 

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  • 小児期の睡眠に関わる生理と病態

    第 43 回岡山脳研究セミナー  2017年 

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  • 小児てんかんの病態解明における広帯域脳波の意義

    第 35 回広島小児神経研究会  2017年 

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  • 発作抑制後、Rey複雑図形記憶困難となった前頭葉てんかん男児例

    全国てんかんセンター協議会2016  2016年 

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  • 発作抑制後、Rey複雑図形記憶困難となった前頭葉てんかん男児例

    第 94 回岡山小児てんかん懇話会  2016年 

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  • 岡山大学における小児神経学研究の歴史と展望

    第 94 回岡山小児てんかん懇話会  2016年 

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  • 岡山大学における小児神経学研究の歴史と展望

    第 94 回岡山小児てんかん懇話会  2016年 

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  • 小児けいれん性疾患における活性型ビタミンB6の有用性

    第 10 回日本てんかん学会中国・四国地方会  2016年 

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  • Landau-Kleffner症候群の1例

    第 10 回日本てんかん学会中国・四国地方会  2016年 

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  • 子どものてんかん

    第 10 回日本てんかん学会中国・四国地方会市民公開講座  2016年 

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  • Utility of the screening test for early diagnosis of Dravet syndrome

    70th Annual Meeting of the American Epilepsy Society  2016年 

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  • 小児てんかんの発作症候学と脳波

    静岡県西部てんかんフォーラム  2016年 

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  • 小児のてんかん-診断と治療の基本-

    第 10 回岡山桃太郎会 第23回岡山日赤小児科クリニカルカンファレンス  2016年 

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  • Rey複雑図形で特異な経過を呈した前頭葉てんかん男児例

    第 21 回認知神経科学会  2016年 

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  • PRRT2遺伝子変異を認めた episodic ataxia の女児

    第 27 回日本小児神経学会中国・四国地方会  2016年 

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  • HYLS1遺伝子変異を認めた Joubert 症候群の兄弟例

    第 27 回日本小児神経学会中国・四国地方会  2016年 

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  • 疼痛や異常感覚、脱力のため検査し、身体症状症および転換性障害と診断した8例

    第 24 回岡山小児心身症研究会  2016年 

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  • 小児てんかんを発作時脳波・ビデオから改めて学ぶ

    第 13 回広島けいれんフォーラム  2016年 

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  • Two unique cases of pyridoxalphoshate-resopnsive infantile epileptic encephalopathies

    International Symposium on Acute Encephalopathy in Infancy aned Its Related Disorders  2016年 

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  • 小児てんかんの発作症候学-脳波・ビデオ同時記録による診断-

    桜山てんかん研究会  2016年 

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  • 小児のてんかん -診断と治療の基本-

    国立病院機構 福山医療センター オープンカンファレンス  2016年 

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  • Dravet 症候群早期診断におけるスクリーニングテストの有用性

    第 58 回日本小児神経学会総会  2016年 

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  • てんかん患者における長期的ビタミンB6 療法は末梢神経に有害な影響を与えるか?

    第 58 回日本小児神経学会総会  2016年 

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  • 幼児期早期に退行をきたし、環状22 番染色体を認めた自閉症スペクトラム障害の男児例

    第 58 回日本小児神経学会総会  2016年 

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  • Hereditary chin trembling の小児例

    第 58 回日本小児神経学会総会  2016年 

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  • 小児てんかんとオシロロジー

    第 57 回日本神経学会学術大会  2016年 

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  • 結節性硬化症のフォローアップ中にLAMが見過ごされた2例

    第 119 回日本小児科学会学術集会  2016年 

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  • 複雑脳動脈奇形により乳児期から脳梗塞を繰り返した女児例

    第 119 回日本小児科学会学術集会  2016年 

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  • PCDH19関連てんかんの女児例

    第 46 回中国・四国点頭てんかん研究会  2016年 

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  • 小児難治てんかん-病態生理の新たなる視点-

    第 46 回中国・四国点頭てんかん研究会  2016年 

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  • Scalp HFOs in various childhood epilepsies

    2nd International Workshop on High Frequency Oscillations in Epilepsy-A scientific and teaching workshop  2016年 

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  • Pyridoxal 5'-phosphate levels in the cerebrospinal fluid in neonatal and infantile epilepsy and its diagnostic utility for antiquitin deficiency

    70th Annual Meeting of the American Epilepsy Society  2016年 

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  • 子どものけいれんとてんかん

    第 9 回岡山県病児保育協議会研修会  2016年 

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  • 小児てんかん診療の進歩と実践

    播磨てんかん懇話会  2016年 

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  • 結節性硬化症の診療連携

    第 4 回日本結節性硬化症学会 教育セミナー  2016年 

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  • 小児難治てんかん-病態生理の新たな視点-

    第 41 回 千葉てんかん懇話会  2016年 

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  • てんかんの病態と薬物治療

    第 55 回日本薬学会・日本薬剤師会・日本病院薬剤師会 中国四国支部学術大会  2016年 

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  • A case of beta-ureidopropionase deficiency with dysmorphic features

    第 58 回日本先天代謝異常学会  2016年 

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  • Pyridoxal 5'-phosphate levels in the cerebrospinal fluid in neonatal and infantaile epilepsy

    第 58 回日本先天代謝異常学会  2016年 

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  • 小児てんかんの脳波分析による病態解明

    第 46 回日本臨床神経生理学会学術大会 サテライトシンポジウム第27回小児脳機能研究会  2016年 

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  • 小児のてんかんに関して

    第 46 回日本臨床神経生理学会学術大会  2016年 

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  • CSWS に対しスルチアムが著効した症例における棘徐波に伴う高周波成分の変化

    第 50 回日本てんかん学会  2016年 

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  • 脳波におけるCSWS のDZP 静注による改善程度と治療予後の検討

    第 50 回日本てんかん学会  2016年 

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  • 難治性部分てんかんにおけるペランパネルの使用経験

    第 50 回日本てんかん学会  2016年 

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  • A clinical picture of PRRT2 related epilepsy in Japan.

    第 50 回日本てんかん学会  2016年 

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  • てんかんの病態解明における広帯域脳波の意義

    第 50 回日本てんかん学会  2016年 

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  • 笑い発作により脱力が誘発された1例

    第 95 回岡山小児てんかん懇話会  2016年 

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  • 点頭てんかんと鑑別を要した乳児ミオクローヌス

    第 95 回岡山小児てんかん懇話会  2016年 

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  • 運動により両側下肢の律動性異常運動が誘発される2症例

    第 96 回岡山小児てんかん懇話会  2016年 

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  • 小児てんかんの発作症候学―脳波・ビデオ同時記録による診断―

    第 90 回日本小児科学会高知地方会  2016年 

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  • 岡山大学における結節性硬化症への取り組み

    TSC JOINT SESSION  2016年 

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  • 結節性硬化症の乳児期における中枢神経系の画像変化

    第 89 回日本小児科学会岡山地方会  2016年 

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  • 乳児神経軸索ジストロフィーの1例

    第 89 回日本小児科学会岡山地方会  2016年 

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  • A storm of fast (40-150 Hz) oscillations during hypsarrhythmia in West syndrome

    69th Annual Meeting of the American Epilepsy Society  2015年 

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  • 重症心身障害児者のトランジッションにおける他職種連携-岡山大学病院での小児看護外来の取り組み-

    第 57 回日本小児神経学会 シンポジウム1「医療的ケアを要する重症心身障害児(者)のトランジッションをどうする?」  2015年 

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  • The influence of comorbid attention deficit hyperactivity disorder (ADHD) and learning disabilities (LD) on the emotions and behavior of children: a study among parents and teachers

    2015 International Association for the Scientific Study of Intellectual and Developmental Disabilities Americas Regional Congress  2015年 

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  • A study on spike focus-dependence of high-frequency oscillations in benign childhood partial epilepsy

    13th Asian and Oceanian Congress of Child Neurology  2015年 

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  • Total folate and 5-methyltetrahydrofolate in the cerebrospinal fluid of children

    13th Asian and Oceanian Congress of Child Neurology  2015年 

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  • Spike-associated high-frequency oscillations in scalp EEG in childhood epilepsy

    13th Asian and Oceanian Congress of Child Neurology  2015年 

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  • 合成 ACTH 療法における ST 合剤(バクタ)の使用と好中球減少症の関連

    第 117 回日本小児科学会学術集会  2015年 

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  • 日本人小児髄液中5 HIAA,HVA,遊離GABA の基準値作成の試み

    第 117 回日本小児科学会学術集会  2015年 

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  • 発達障害をともなう学童期小児の実行機能の年齢群別比較

    第 57 回日本小児神経学会総会  2015年 

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  • 読字・書字能力の発達-就学前、小学1年生、2年生の追跡的研究-第二報

    第 57 回日本小児神経学会総会  2015年 

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  • 結節性硬化症における多臓器フォローアップの実態

    第 57 回日本小児神経学会総会  2015年 

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  • 新生児低酸素性虚血脳症

    第 92 回岡山小児てんかん懇話会  2015年 

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  • ラモトリギンが著効した店頭てんかんの1例

    第 92 回岡山小児てんかん懇話会  2015年 

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  • 当院における半球離断術の予後

    第 9 回日本てんかん学会中国・四国地方会  2015年 

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  • エトスクシミドが著効した高度な脳波異常を呈した幼児の1例

    第 9 回日本てんかん学会中国・四国地方会  2015年 

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  • ACTH 療法が無効で新規抗てんかん薬が著効した West 症候群の6例

    第 9 回日本てんかん学会中国・四国地方会  2015年 

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  • Poor toddler-age sleep schedules predict school-age behavioral disorders in a longitudinal survey

    11th Congress of Asian Society for Pediatric Research  2015年 

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  • A variety of clinical presentations in skeletal muscle sodium channelopathies

    11th Congress of Asian Society for Pediatric Research  2015年 

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  • 合成ACTH療法が血球系に与える影響

    第 45 回中国・四国点頭てんかん研究会  2015年 

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  • ACTH 療法が無効で新規抗てんかん薬が著効した West 症候群の6例

    第 9 回岡山市小児科専門医会  2015年 

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  • TBX1遺伝子異常を同定した副甲状腺機能低下症の1例

    第 67 回中国・四国小児科学会  2015年 

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  • コハク酸セミアルデヒド脱水素酵素欠損症の1男児例

    第 67 回中国・四国小児科学会  2015年 

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  • 小児脳波研究の最前線

    第 45 回日本臨床神経生理学会学術大会  2015年 

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  • 頭皮脳波における高周波振動検出のスキル

    第 45 回日本臨床神経生理学会学術大会  2015年 

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  • 頭皮脳波における高周波振動検出のスキル

    第 45 回日本臨床神経生理学会 シンポジウム11 てんかん性DC電位と高周波振動(HFO)のskills workshop  2015年 

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  • Dravet症候群早期診断におけるスクリーニングテストの有用性

    第 49 回日本てんかん学会  2015年 

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  • てんかん患者において長期的ビタミンB6療法が末梢神経に及ぼす影響

    第 49 回日本てんかん学会  2015年 

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  • Occurrence of bilaterally independent epileptic spasms after a corpus callosotomy in West syndrome

    第 49 回日本てんかん学会  2015年 

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  • A study on spike focus-dependence of high-frequency oscillations in benign childhood partial epilepsy

    International Symposium on Benign Infantile Seizures 2015  2015年 

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  • コハク酸セミアルデヒド脱水素酵素欠損症の1男児例

    第 57 回日本先天代謝異常学会  2015年 

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  • Tic障害に対する薬物治療

    第 23 回岡山小児心身症研究会  2015年 

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  • インフルエンザの治療・回復期にせん妄、精神症状を呈した2症例

    第 26 回日本小児神経学会中国・四国地方会  2015年 

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  • 小児髄液中モノアミン類の測定計の作成

    第 26 回日本小児神経学会中国・四国地方会  2015年 

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  • 脳波解析が明かすてんかんの病態―基礎から最前線まで―

    第 8 回日本てんかん学会東海・北陸地方会イブニングセミナー  2015年 

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  • PNESを認め難治な経過をたどった前頭葉てんかんの1例

    第 70 回岡山てんかん懇話会  2015年 

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  • Spatial relationship between fast and slow components of ictal activities and interictal epileptiform discharges in epileptic spasms

    9th International Conference on Complex Medical Engineering  2015年 

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  • Technical advances to relieve people with epilepsy

    9th International Conference on Complex Medical Engineering, Lunch Seminar 4  2015年 

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  • Ethosuximide が有効であった難治てんかんの 3 例

    第 57 回日本小児神経学会総会  2015年 

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  • 結節性硬化症の定期的フォローの重要性

    第 93 回岡山小児てんかん懇話会  2015年 

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  • 脳波分析から探る小児てんかん性脳症の病態

    第 33 回四国小児神経症例検討会  2015年 

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  • West症候群の予測因子としての脳波の基礎波の有用性

    第 88 回日本小児科学会岡山地方会  2015年 

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  • 難治な経過を示す重症筋無力症の1例

    第 88 回日本小児科学会岡山地方会  2015年 

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  • 虐待に酷似したACTH療法後の外傷性慢性硬膜下血腫の1例

    第 87 回日本小児科学会岡山地方会  2014年 

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  • WDR45遺伝子異変による非典型的な臨床経過を呈したSENDAの1例

    第 66 回中国・四国小児科学会  2014年 

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  • てんかんと脳代謝・血流―小児てんかんを中心として―

    第 26 回日本脳循環代謝学会総会  2014年 

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  • 乳児期から特異な経過を示すDravet症候群の1例

    第 25 回日本小児神経学会中国・四国地方会  2014年 

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  • 小児の髄液中ビタミンB6、ピリドキサールリン酸の基準値確率の試み

    第 25 回日本小児神経学会中国・四国地方会  2014年 

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  • てんかん性高周波振動の発生プロセスを探る

    神経オシレーションカンファレンス  2014年 

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  • Differences in behavioral evaluations for boys with AD/HD and/or PDD between parents and teachers with reference to child's age

    4th International Association for the Scientific Study of Intellectual and Developmental Disabilities Europe Congress  2014年 

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  • 脳波分析による小児良性部分てんかんの展開予測は可能か

    第 48 回日本てんかん学会 イブニングセミナー1「特発性部分てんかんのAtypical evolutionをめぐって」  2014年 

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  • Current status of Dravet syndrome in Japan

    第 48 回日本てんかん学会  2014年 

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  • てんかんの神経生理学―数式を使わない脳波分析の基本―

    第 48 回日本てんかん学会  2014年 

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  • 合成 ACTH 療法における ST 合剤(バクタ)の使用と好中球減少症の関連

    第 91 回岡山小児てんかん懇話会  2014年 

     詳細を見る

  • 小児てんかんの診療と研究

    関西医科大学 CNS Forum  2014年 

     詳細を見る

  • てんかん性高周波脳活動―デジタル脳波が拓くてんかん原性の新たなバイオマーカー

    国立病院機構西新潟中央病院てんかんセンター 夏期セミナー2014  2014年 

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  • Spatial relationship between the fast and slow components of ictal activities and interictal epileptiform discharges in children with epileptic spasms

    68th Annual Meeting of the American Epilepsy Society  2014年 

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  • 新生児脳波における ictal fast activity は乳児期てんかん原性の指標になり得るか?

    第 56 回日本小児神経学会総会  2014年 

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  • 難治てんかんにおけるルフィナミドの有効性について

    第 56 回日本小児神経学会総会  2014年 

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  • 緊急少数電極脳波の振幅・周波数統合トレンド表示は脳波非習熟者における判読制度を向上させるか?

    第 56 回日本小児神経学会総会  2014年 

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  • Examining executive function in children with autism with comorbid AD/HD using the Wisconsin card sorting test

    4th International Association for the Scientific Study of Intellectual and Developmental Disabilities Europe Congress  2014年 

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  • 小児神経学の進歩―小児てんかんを中心として―

    山陽小児臨床懇話会  2014年 

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  • 難治性てんかんに対する迷走神経刺激法の効果

    第 69 回岡山てんかん懇話会  2014年 

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  • Ethosuximide で欠神発作が悪化した1例

    第 69 回岡山てんかん懇話会  2014年 

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  • 注意欠陥/多動性障害と広汎性発達障害の子どもにみられる行動や情緒の特徴:年齢による検討

    第 56 回日本小児神経学会総会  2014年 

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  • 発達障害をともなう子どもにおける実行機能と知能検査成績との関連

    第 56 回日本小児神経学会総会  2014年 

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  • Na チャンネル異常による筋疾患の臨床多様性

    第 56 回日本小児神経学会総会  2014年 

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  • Fast activities on inter-ictal and ictal electroencephalograms in a newborn infant with hypoxic-ischemic encephalopathy

    8th International Conference on Brain Monitoring & Neuroprotection in the Newborn  2014年 

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  • 幼児期発症の DRPLA

    第 117 回日本小児科学会学術集会  2014年 

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  • グアニジノ酢酸 メチルトランスフェラーゼ欠損症の 1 症例 – 治療経過報告

    第 117 回日本小児科学会学術集会  2014年 

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  • 極めて難治のてんかん重積状態を来たし、外科的介入が成功した皮質形成異常の1例

    脳疾患救急ミーティング  2014年 

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  • Late onset spasm を来した CASK 遺伝子異常の1例

    第 44 回中国・四国点頭てんかん研究会  2014年 

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  • 乳児期に診断された Angelman 症候群の1例

    第 90 回岡山小児てんかん懇話会  2014年 

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  • 笑い発作を呈した側頭葉てんかんの1例

    第 37 回日本てんかん外科学会  2014年 

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  • 極めて難治のてんかん重積状態を来たし、外科的介入が成功した皮質形成異常の1例

    第 8 回日本てんかん学会中国・四国地方会  2014年 

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  • Levetiracetam(LEV)の著効例について

    第 8 回日本てんかん学会中国・四国地方会  2014年 

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  • 小児良性部分てんかんの脳波における高周波振動と棘波の分布に関する検討

    第 44 回日本臨床神経生理学会  2014年 

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  • 脳波専門問題について

    第 44 回日本臨床神経生理学会学術大会  2014年 

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  • てんかんの診療技術の進歩

    市民公開講座「てんかんを知る・治す」  2014年 

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  • 小児てんかんの診療実態に関する疫学調査(第2報)

    第 48 回日本てんかん学会  2014年 

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  • Dravet症候群におけるStiripentolの初期および長期効果の検討

    第 48 回日本てんかん学会  2014年 

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  • 髄液中5-メチルテトラヒドロ葉酸(5MTHF)の測定法の開発

    第 87 回日本小児科学会岡山地方会  2014年 

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  • 午前中のふらつきや不機嫌が主訴であったglucose transporter type 1(Glut1)欠損症の1例

    第 87 回日本小児科学会岡山地方会  2014年 

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  • 焦点を有する非定型欠神発作の1例

    第 68 回岡山てんかん懇話会  2013年 

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  • てんかん研究の最前線

    公開講座・講演会 命の輝きを守る 障害医学・医療の推進をめざして  2013年 

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  • ICCA症候群および乳児期発症良性痙攣性疾患におけるPRRT2遺伝子解析

    第 55 回日本小児神経学会総会  2013年 

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  • 小児てんかんの診療実態に関する疫学調査(第1報)

    第 55 回日本小児神経学会総会  2013年 

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  • アミノ酸の髄液中濃度、髄液/血漿中濃度比の日本人小児における基準値設定の試み

    第 55 回日本小児神経学会総会  2013年 

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  • CACNA1A遺伝子変異/多型がドラベ症候群の臨床症状に及ぼす影響

    第 55 回日本小児神経学会総会  2013年 

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  • ICCA症候群に片麻痺性片頭痛を合併した1家系

    第 55 回日本小児神経学会総会  2013年 

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  • 症候の異なる発作性ジスキネジアにおけるPRRT2遺伝子変異の有無

    第 55 回日本小児神経学会総会  2013年 

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  • 難治小児てんかんの電気生理‐特に異常高周波活動と病態との関係について‐

    第 55 回日本小児神経学会  2013年 

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  • 脳波異常から診断に至った Angelman 症候群と Rett 症候群の4症例

    第 116 回日本小児科学会学術集会  2013年 

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  • Gamma oscillations superimposed on subclinical seizure activity in a newborn infant with hypoxic- ischemic encephalopathy

    15th Annual Meeting of Infantile Seizure Society(ISS) International symposium on Neonatal Seizures and Related Disorders  2013年 

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  • Development of amplitude-integrated EEG colored according to spectral edge frequency. International Symposium on Neonatal Seizures and Related Conditions

    15th Annual Meeting of the Infantile Seizure Society  2013年 

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  • 特異な言葉で発作が誘発されるてんかんの1例

    第 88 回岡山小児てんかん懇話会  2013年 

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  • 側頭葉てんかんにおける海馬機能評価に対する選択的後大脳動脈ワダテストの有用性

    第 7 回日本てんかん学会中国・四国地方会  2013年 

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  • 小児期に恐怖発作を呈した5症例

    第 7 回日本てんかん学会中国・四国地方会  2013年 

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  • MRIの画像処理による焦点性てんかんの検出―多症例における検討

    第 7 回日本てんかん学会中国・四国地方会  2013年 

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  • 脳波の高周波活動とてんかん

    第 460 回福岡臨床と脳波懇話会  2013年 

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  • 小児科医に必要な脳波の知識

    第 116 回日本小児科学会  2013年 

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  • The generative process of epileptic high-frequency oscillations as shown through neuronal network simulation of the rat dentate gyrus

    30th International Epilepsy Congress  2013年 

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  • West症候群を発症した46, XY, der(16)t(2;16)(p22;p13.3)の1家系

    第 47 回日本てんかん学会  2013年 

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  • Computerized MRI processing to detect focal lesions in intractable epilepsy

    67th Annual Meeting of the Americal Epilepsy Society  2013年 

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  • 緊急少数電極脳波のトレンド表示は脳波非習熟者における判読制度を向上させるか?

    第 86 回日本小児科学会岡山地方会  2013年 

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  • 下肢の運動障害から始まり多彩な症状を呈した転換性障害の1例

    第 86 回日本小児科学会岡山地方会  2013年 

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  • The first Japanese case of guanidinoacetate methyltransferase (GAMT) deficiency

    3rd Asian Congress of Inherited Metabolic Diseases and 55th Annual Meeting of The Japanese Society for Inherited Metabolic Diseases  2013年 

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  • 乳児期早期に皮質性ミオクローヌスを認めたDravet症候群の1例

    第 43 回日本臨床神経生理学会  2013年 

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  • West症候群におけるACTH療法後の再発と脳波所見の関連

    第 43 回日本臨床神経生理学会  2013年 

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  • 脳波の高周波分析の意義と展望

    第 43 回日本臨床神経生理学会  2013年 

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  • 小児てんかんの診療―脳波・ビデオ同時記録による診断を中心として―

    岡山県東部地区てんかん治療講演会  2013年 

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  • 小児期発病の未決定てんかんおよび症候性全般てんかん

    第 47 回日本てんかん学会 第 8 回てんかん学研修セミナー  2013年 

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  • 頭蓋内脳波における高周波解析を基に切除計画を行ったてんかん手術症例3例

    第 76 回日本脳神経外科学会中国四国支部学術集会  2013年 

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  • バルプロ酸にトピラマートを追加した場合、血中アンモニア濃度は上昇するか?

    第 47 回日本てんかん学会  2013年 

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  • 難治てんかんにおける Levetiracetam の有用性について

    第 47 回日本てんかん学会  2013年 

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  • ラット歯状回におけるてんかん性高周波振動の発生過程のシミュレーション

    第 47 回日本てんかん学会  2013年 

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  • Epileptic encephalopathy in childhood and high frequency EEG oscillations

    Shunsuke Ohtahara Memorial International Symposium  2013年 

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  • 極めて難治のてんかん重積状態を来たし、外科的介入が成功した皮質性形成異常の1例

    第 89 回岡山小児てんかん懇話会  2013年 

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  • 就学後から小学校2年生における、読字・書字能力の発達に関する追跡的研究

    第 18 回認知神経科学会  2013年 

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  • 外傷を契機に頭部MRIにより発見されたHunter症候群の1例

    第 24 回日本小児神経学会中国・四国地方会  2013年 

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  • Kleine-Levin 症候群の2例

    第 24 回日本小児神経学会中国・四国地方会  2013年 

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  • CACNA1A variants as a potential genetic modifier of Dravet syndrome

    30th International Epilepsy Congress  2013年 

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  • MRIのコンピュータ画像処理による難治性部分てんかんの脳病変の検出

    第 85 回日本小児科学会岡山地方会  2012年 

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  • Electrophysiological research: epileptic encephalopathy and high-frequency EEG oscillations in childhood

    Korean Epilepsy Congress: JES-KES Symposium "Basic Science"  2012年 

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  • てんかん性脳症の suppression-burst における高周波活動の統計学的時間・周波数分析による検出

    第 54 回日本小児神経学会総会  2012年 

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  • 岡山県における小児破局てんかんの有病率と10年後の追跡調査

    第 115 回日本小児科学会学術集会  2012年 

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  • てんかん関連電位依存性カルシウムチャネルCav2.1 変異は機能獲得特性を示す

    第 89 回日本日本生理学大会  2012年 

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  • てんかん発作を知る

    第 25回中部地区生理検査研修会  2012年 

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  • Clinico-electroencephalographic analysis in epileptic encephalopathies

    Epilepsy Forum in Okayama  2012年 

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  • 就学前から小学校2年生における、読字・書字能力発達に関する追跡的研究

    第 23 回日本小児神経学会中国・四国地方会  2012年 

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  • MRIの画像処理による局在性皮質形成異常の検出の試み

    第 23 回日本小児神経学会中国・四国地方会  2012年 

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  • PTSD により不注意症状が悪化した広汎性発達障害の1例

    第 20 回岡山小児心身症研究会  2012年 

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  • 内側側頭葉てんかん患者における手術前後の神経心理学的評価

    第 66 回岡山てんかん懇話会  2012年 

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  • Epidemiology of catastrophic epilepsy in infancy and early childhood: results of the Far-East Asia Catastrophic Epilepsy (FACE) Study

    14th the annual meeting of ISS International Symposium on Surgery for Catastrophic Epilepsy in Infants (ISCE)  2012年 

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  • EEG of age-dependent epileptic encephalopathy in infancy and early childhood

    14th annual meeting of ISS International Symposium on Surgery for Catastrophic Epilepsy in Infants (ISCE)  2012年 

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  • 小児のけいれん-熱性けいれんを含めた広義のけいれんの対応

    第 7 回岡山県小児救急医療フォーラム  2012年 

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  • 当院で半球離断術を行った乳児期発症の難治てんかん6例の検討

    第 65 回中国・四国小児科学会  2012年 

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  • Ticに対するAripiprazoleの使用実績

    第 65 回中国・四国小児科学会  2012年 

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  • 小児期発症のナルコレプシーの2例

    第 64 回中国・四国小児科学会  2012年 

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  • Eyelid myoclonia with abseces の臨床的脳波学的検討

    第 42 回日本臨床神経生理学会  2012年 

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  • 小児てんかんにおける脳波の高周波活動

    第 42 回日本臨床神経生理学会 シンポジウム17 てんかん脳波学の進歩  2012年 

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  • 小児期のてんかん性脳症と脳波の高周波振動

    神経オシレーション:共振とディスリズミア  2012年 

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  • Epileptic spasms の病態と治療―脳波の高周波活動を中心として―

    第 62回大阪小児てんかん研究会  2012年 

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  • 脳波専門問題について

    第 42 回日本臨床神経生理学会  2012年 

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  • 急激な経過で呼吸不全をきたした軸索型ギラン・バレー症候群の1例

    第 85 回日本小児科学会岡山地方会  2012年 

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  • Spikes with distinct high frequency oscillations identify better the SOZ than HF power changes during spikes

    66th American Epilepsy Society Annual Meeting  2012年 

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  • SCN9A遺伝子異変/多型がドラベ症候群の臨床型に及ぼす影響

    第 46 回日本てんかん学会  2012年 

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  • 非定型欠神発作の多様性

    第 46 回日本てんかん学会  2012年 

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  • 特殊なてんかん症候群

    第 46 回日本てんかん学会  2012年 

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  • 内側側頭葉てんかん患者における手術の神経心理学的影響-改善群と悪化群の比較・検討-

    第 17 回認知神経科学会  2012年 

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  • 読字・書字能力の発達―就学前、小学1年生、小学2年生の追跡的研究―

    第 36 回日本神経心理学会  2012年 

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  • Bening neonatal sleep myoclonus の1例

    第 87 回岡山小児てんかん懇話会  2012年 

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  • 小児てんかんの診断と治療

    セミナー:てんかんの診断から最新の治療まで「てんかん診療における医療連携」  2012年 

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  • てんかん外科治療における術前脳波と神経画像の意義

    脳外科 Epilepsy Seminar  2012年 

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  • バルプロ酸ナトリウムとエトサクシミドが有効であった後天性てんかん性弁蓋症候群の1例

    第 46 回日本てんかん学会  2012年 

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  • 高解像度MRI画像のコンピュータ画像処理による限局性皮質形成異常の検出の試み

    第 46 回日本てんかん学会  2012年 

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  • 難治頻回部分発作重積型急性脳炎(AERRPS)が疑われた複雑部分発作の1例

    第 85 回日本小児科学会岡山地方会  2012年 

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  • Epileptic spasmsの病態―脳波分析を中心として―

    久留米大学医学部小児科学教室グランドラウンズ  2011年 

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  • 小児のてんかん及び非てんかん性発作の症候学 -脳波・ビデオ同時記録による診断-

    岡山市小児科専門医会学術講演会  2011年 

     詳細を見る

  • Extracranial HFOs

    Workshop: High Frequency Oscillations in Cognition and Epilepsy  2011年 

     詳細を見る

  • 大田原症候群の1例における発作時脳波の高周波活動分析

    第 41 回中国・四国点頭てんかん研究会  2011年 

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  • てんかん・けいれん重積状態の治療

    第 13 回岡山日赤小児科カンファレンス  2011年 

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  • 脳波のてんかん性ガンマ・高周波活動

    第 2 回名古屋小児てんかんフォーラム  2011年 

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  • レベチラセタムが著効したDRPLAの1例

    第 5 回日本てんかん学会中国・四国地方会  2011年 

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  • 非定型欠神発作における部分的要素の検討

    第 41 回日本臨床神経生理学会  2011年 

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  • 脳梁離断術後に左右独立性の epileptic spasms を認めた West 症候群の1例

    第 45 回日本てんかん学会  2011年 

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  • 内側側頭葉てんかんの頭皮上および頭蓋内脳波所見と臨床症状

    第 45 回日本てんかん学会  2011年 

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  • Scn1a変異ラットの高体温誘発発作における高周波振動

    第 45 回日本てんかん学会 トランスレーショナルリサーチ関連演題  2011年 

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  • 脳波を治療するか?:可能な限り治療すべきだ

    第 45 回日本てんかん学会 シンポジウム3「診断・治療のゴールデンスタンダードを求めて」  2011年 

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  • 非てんかん患者のてんかん発射についての検討

    第 84 回日本小児科学会岡山地方会  2011年 

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  • Spectral edge frequency により色彩表示したaEEGの開発

    第 114 回日本小児科学会学術集会  2011年 

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  • 睡眠時脳波の持続性棘徐波に対するジアゼパム静注の影響に関する検討

    第 53 回日本小児神経学会総会  2011年 

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  • 急性脳症における遺伝性けいれん素因とSCN1A遺伝子変異

    第 53 回日本小児神経学会総会  2011年 

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  • 良性小児部分てんかんの棘波に伴う高周波振動に関する研究

    第 53 回日本小児神経学会総会  2011年 

     詳細を見る

  • Gamma and high-frequency EEG activity in childhood epilepsy

    てんかんエキスパートミーティング in 岡山  2011年 

     詳細を見る

  • 小児のてんかん及び非てんかん性発作の症候学 -脳波・ビデオ同時記録による診断-

    第 3 回静岡県西部てんかんフォーラム  2011年 

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  • 難治成人てんかんにおけるLevetiracetamの使用経験

    イーケプラ錠発売一周年記念講演会  2011年 

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  • Scalp recorded high frequency oscillations in EOEE

    Genetically determined early onset epileptic encephalopathies: from phenotype to diagnostic algorithms  2011年 

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  • Utility of digital EEG analysis in childhood epilepsy

    The 29th International Congress of Clinical Neurophysiology-Symposium 42 "Digital EEG and EEG standards"  2010年 

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  • Periventricular leukomalacia を有する小児に合併したてんかんの特徴

    第 44 回日本てんかん学会  2010年 

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  • シンポジウム1「Cutting edge of neurodiagnostics for epilepsy」Advancement of EEG analysis techniques to explore high-frequency oscillations

    第 44 回日本てんかん学会  2010年 

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  • 小児のてんかん及び非てんかん性発作の症候学―脳波・ビデオ同時記録による診断―

    第2回 Kagawa Epilepsy Forum  2010年 

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  • 小児てんかんの発作時ビデオ・脳波同時記録による診断

    てんかんカンファレンス in 播磨  2010年 

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  • Analysis of the preceding positive spikes in patients with benign partial epilepsy and febrile seizures

    29th International Congress for of Clinical Neurophysiology  2010年 

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  • Pathophysiology of West syndrome elucidated by EEG analysis

    Taiwan Pediatric Epilepsy Congress  2010年 

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  • 多彩な発作症状を来たした視床下部過誤腫による難治てんかん

    第 21 回日本小児神経学会中国・四国地方会  2010年 

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  • 難治てんかん患者に対するlamotorigine(LTG) の長期効果

    第 52 回日本小児神経学会総会  2010年 

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  • Epileptic spasms の病態―脳波分析を中心にして―

    第 40 回中国・四国点頭てんかん研究会  2010年 

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  • West 症候群を発症した Sturge-Weber 症候群

    第 82 回岡山小児てんかん懇話会  2010年 

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  • Detection of high-frequency EEG oscillations in sleep-induced electrical status epilepticus in childhood

    The 10th Asian & Oceanian Congress of Child Neurology  2009年 

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  • Statistical time-frequency analysis for the detection of changes in high-frequency EEG activity

    63rd Annual Meeting of the American Epilepsy Society  2009年 

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  • West症候群再発の予測因子としての脳波の有用性

    第 39 回日本臨床神経生理学会  2009年 

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  • A long-term follow-up study of Dravet syndrome up to adulthood

    63rd Annual Meeting of the American Epilepsy Society  2009年 

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  • High-frequency changes during interictal spikes detected by time-frequency analysis

    63rd Annual Meeting of the American Epilepsy Society  2009年 

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  • Detection of seizure-associated high-frequency oscillations above 500 Hz

    63rd Annual Meeting of the American Epilepsy Society  2009年 

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  • 成人期に達した Dravet 症候群の長期経過に関する研究

    第 51 回日本小児神経学会総会  2009年 

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  • 徐波睡眠時に持続性棘徐波を示すてんかんにおける高周波活動

    てんかんエキスパートミーティング in Okayama  2009年 

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  • A study on behavioral problems in children with epilepsy

    12th Annual Meeting of Infantile Seizure Society  2009年 

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  • Epileptic spasmsの病態生理

    第 39 回日本臨床神経生理学会 シンポジウム13「神経生理学よりみた小児てんかん発作(日本小児神経学会合同シンポジウム)」  2009年 

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  • 小児の脳波とてんかん

    第 34 回高知県てんかん研究会  2009年 

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  • 500 Hz 以上の発作時高周波振動

    第 43 回日本てんかん学会  2009年 

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  • 小児のてんかん発作の症候学

    第 4 回てんかん学研修セミナー  2009年 

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  • Investigation on the synchronizing appearance of occipital and frontopolar spike in childhood epilepsy

    18th International Congress on Brain Electromagnetic Topography  2009年 

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  • Detection of high-frequency EEG oscillations in sleep-induced electrical status epilepticus in childhood

    18th International Congress on Brain Electromagnetic Topography  2009年 

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  • 有熱時痙攣が群発したサルモネラ腸炎の1例

    第 81 回岡山小児てんかん懇話会  2009年 

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  • Designated discussant

    International Workshop on Neuropsychiatric Issues in Epilepsy  2009年 

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  • 小児てんかんの発作時脳波・ビデオ同時記録による診断

    第 14 回金沢医科大学小児科地域連携症例検討会  2009年 

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  • A patient with Rasmussen encephalitis and SCN1A mutation

    11th Annual Meeting of Infantile Seizure Society  2008年 

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  • Spectral analysis of EEG gamma rhythms associated with epileptic tonic seizures

    62nd Annual Meeting of the American Epilepsy Society  2008年 

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  • 子供の発作時脳波のとり方・見方

    第 4 回てんかん学会近畿地方会  2008年 

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  • 5 歳以上の児童にみられたepileptic spasms の発作時脳波におけるガンマ律動の検討

    第 38 回日本臨床神経生理学会  2008年 

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  • Sequential mapping による先行陽性棘波の検討

    第 38 回日本臨床神経生理学会  2008年 

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  • 脳波波形の正常と異常

    第 38 回日本臨床神経生理学会 教育セミナー5  2008年 

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  • 成人期に達した Dravet 症候群に関する研究

    第 42 回日本てんかん学会  2008年 

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  • 強直発作の発作時脳波におけるγ律動のスペクトル解析

    第 42 回日本てんかん学会  2008年 

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  • ビデオで判断した特異な発作の1乳児例

    第 79 回岡山小児てんかん懇話会  2008年 

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  • 成人期に達した Dravet 症候群の長期観察

    第 79 回岡山小児てんかん懇話会  2008年 

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  • 臨床における自作ソフトウェアによる脳波分析

    第 25 回日本脳電磁図トポグラフィー研究会 シンポジウム1:「自作解析ソフトウェアによる脳波解析」  2008年 

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  • West 症候群とその周辺

    第 50 回日本小児神経学会 シンポジウム1:「小児てんかんの診断と治療―エキスパートオピニオン―」  2008年 

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  • 成人期に達した Dravet 症候群の長期経過に関する研究

    第 50 回日本小児神経学会総会  2008年 

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  • 岡山市における小児のてんかん重責状態の疫学調査(2003年〜2005年)―発生率と特徴―

    第 50 回日本小児神経学会総会  2008年 

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  • SCN1A変異を認めた Rasmussen 脳炎の1例

    第 50 回日本小児神経学会総会  2008年 

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  • 皮質形成異常の多面的3次元MRI表示の試み

    第41回日本てんかん学会  2007年 

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  • 小児の異常皮質脳波の非侵襲的推定

    第37回日本臨床神経生理学会  2007年 

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  • 乳児期のてんかん性脳症におけるてんかん原性と高周波脳波活動

    第40回日本てんかん学会  2006年 

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  • A clinical study of the relation between spasms in series and periodic EEG abnormality in epileptic encephalopathy beginning in early infancy

    The 10th International Child Neurology Congress  2006年 

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  • 小児にみられる FP-O 棘波の動的脳電図と dipole 分析による検討

    第48回日本小児神経学会総会  2006年 

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  • Spasms と部分発作の複合発作時にみられる極高周波律動

    第47回日本小児神経学会総会  2005年 

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  • 早期乳児のてんかん性脳症における spasms のシリーズ形成と脳波異常の群発形成の関連についての研究

    第39回日本てんかん学会  2005年 

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  • Epileptic spasms の発作時脳波における高振幅徐波の再現性と分布に関する研究

    第46回日本小児神経学会総会  2004年 

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  • Epileptic spasms の発作時脳波における極高周波律動のシリーズ中の一定性に関する研究

    第34回日本臨床神経生理学会学術大会  2004年 

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  • シンポジウム3 「脳波分析の新しい展開」 独立成分分析の脳波解析への応用ーてんかん発射を中心としてー

    第33回日本臨床神経生理学会・学術大会  2003年 

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  • Epileptic spasms の発作時脳波における 60-100Hz 律動活動

    第33回日本臨床神経生理学会・学術大会  2003年 

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  • A clinico-electroencephalographic study of children suffering from febrile seizures plus

    Joint Congress of International Child Neurology Association and Asian & Oceanian Child Neurology Association 2002  2002年 

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  • 側頭葉棘波の双極子分析における残差分散の脳内3次元空間分布に関するシミュレーション研究

    第31回日本臨床神経生理学会学術大会  2001年 

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  • Systematic source estimation of epileptiform discharges by a combination of independent component analysis and RAP-MUSIC

    53rd Annual Meeting of the American Epilepsy Society  2001年 

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  • シンポジウム II 「皮質形成異常と難治てんかん」 皮質形成異常に伴う難治てんかんの臨床・神経生理学的特徴

    第 33 回日本てんかん学会  1999年 

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  • シンポジウム「ミオクロニー発作の臨床と神経生理」 ミオクロニー発作と部分発作

    第 32 回日本てんかん学会  1998年 

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受賞

  • 第57回日本小児神経学会学術集会優秀演題賞

    2015年  

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    受賞国:日本国

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  • てんかん治療研究振興財団研究褒彰

    2003年  

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    受賞国:日本国

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  • 日本てんかん学会 第16回J.A.Wada奨励賞(臨床部門)

    1993年  

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    受賞国:日本国

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担当授業科目

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  • 小児医科学(発達神経病態学)II(演習・実習) (2021年度) 特別  - その他

  • 小児医科学(発達神経病態学)II(講義・演習) (2021年度) 特別  - その他

  • 小児科学 (2021年度) 第1学期  - 木5

  • 生体防御医学演習 (2021年度) 特別  - その他

  • 脳卒中特論 (2021年度) 特別  - その他

  • 選択制臨床実習(小児神経学) (2021年度) 特別  - その他

  • 小児医科学(発達神経病態学)I(演習・実習) (2020年度) 特別  - その他

  • 小児医科学(発達神経病態学)I(講義・演習) (2020年度) 特別  - その他

  • 小児医科学(発達神経病態学)II(演習・実習) (2020年度) 特別  - その他

  • 小児医科学(発達神経病態学)II(講義・演習) (2020年度) 特別  - その他

  • 小児科学 (2020年度) 第1学期  - 木5

  • 生体防御医学演習 (2020年度) 通年  - その他

  • 生体防御医学総論 (2020年度) 通年  - その他

  • 選択制臨床実習(小児神経学) (2020年度) 特別  - その他

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