Shuddering attacks(身震い発作)を呈した4例 Shuddering Attacks in Four Children

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著者

    • 金澤 治 KANAZAWA Osamu
    • 国立療養所西新潟中央病院てんかんセンター小児科 Department of Pediatrics, Epilepsy Center, Nishi-Niigata Central National Hospital
    • 白根 聖子 SHIRANE Seiko
    • 国立療養所西新潟中央病院てんかんセンター小児科 Department of Pediatrics, Epilepsy Center, Nishi-Niigata Central National Hospital
    • 早川 さゆり HAYAKAWA Sayuri
    • 国立療養所西新潟中央病院てんかんセンター小児科 Department of Pediatrics, Epilepsy Center, Nishi-Niigata Central National Hospital

抄録

Shuddering attacks (SA, 身震い発作) は乳幼児期に発症する稀な良性疾患といわれ, てんかんとの鑑別を要する.発作は意識消失のない何秒間かの身震いで日単位で反復する.Essential tremor (ET, 本態性振戦) と同様の発現機序といわれ脳の未熟性による表現様式の違いとされるが, 報告は稀で本邦では皆無である.SAを呈する生後8~14カ月の乳幼児4例で, ビデオないしビデオ・脳波同時記録で発作時をとらえ病態を検討した.1例でSAに同期した筋電図より, ETの周波数とほぼ一致した.全例で発作減少ないし消失をみたが, MRI上脳の未熟性, トルコ鞍扁平, てんかんの家族歴, のある例もあった.従来SAは良性疾患で検索不要といわれるが, 神経系発達に関連した何らかの問題が潜む可能性がある.

Shuddering attacks (SA), an uncommon benign disorder occurring during infancy or early childhood, should be distinguished from epileptic seizures. The attacks are shivering movements occurring daily for several seconds without impairment of consciousness. SA are regarded as an early premature manifestation of essential tremor (ET), and the provoking mechanism is the same between them. There are case reports of SA, but none from Japan.<BR>We studied the pathophysiology of SA in four children aged between 8 and 14 months using a video-EEG monitoring system. In one patient, the shuddering movements, as indicated by contaminating electromyogram during electroencephalography, was almost as frequent as that of ET. SA decreased in frequency or disappeared in all our patients. One of them showed immature brain development on MRI and had relatives with epilepsy. Another showed flattened sella turcica. Although previous reports suggest that SAs are benign and require no investigation, children with SA could have borderline problems related to the development of the nervous system.

収録刊行物

  • 脳と発達

    脳と発達 32(5), 424-429, 2000-09-01

    脳と発達

参考文献:  6件中 1-6件 を表示

各種コード

  • NII論文ID(NAID)
    10005656050
  • NII書誌ID(NCID)
    AN0020232X
  • 本文言語コード
    JPN
  • 資料種別
    SHO
  • ISSN
    00290831
  • データ提供元
    CJP書誌  J-STAGE 
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