急激な運動開始により誘発される特異な発作症状を有する1家系

SEIZURES INDUCED BY SUDDEN VOLUNTARY MOVEMENT

I．はじめに

　著者らは，茨城県立友部病院を訪れた患者を発端として，急激に開始された運動によつて誘発され，意識障害はなくて主として四肢の硬直する発作症状を有する者が1家系内に多発しているのを発見したので報告する（第1図）。

　まず0-1,I−9の両名が昭和40年1月に来院し，さらにI−10,I−61の計4名を直接診察検査した。I—16はすでに昭和34年に2回受診して転医していることが判明し当時の記載によつた。他の症例については，家庭訪問によりII−4,I−5から症状を聴取した。

Nine patients were found in one genealogical lineage, who had peculiar seizures induced by sudden voluntary movement of the leg after a period of rest, especially when they began to stand up or walk. Unilateral tonic spasm begins from fingers to arm after numb sensation, accompanying in three patients involuntary flapping movements, and the leg of the same side becomes tonic in seven patients. Speech becomes difficult or impossible, though there is no disturbances of con-sciousness. The attacks are always unilateral and mainly right sided. The duration of them is a few seconds mainly and less than thirty seconds, their occurance being a few times a month to several times a day. The attacks begin in the age between eight to fifteen and one old man of this family has seen the disappearance of them in his forties. According to the test of the central nervous system or elswhere, abnor-mal findings were not found and serum electrorites were normal. EEGs in five patients : Four showed normal record at rest and the other showed the irre-gular activity composing of alpha-, beta-, and theta-waves. In three cases spikes or spike & wavecomplexes were provoked by the activation of over-breathing or metrazol injection, among whom two showed grand mal seizure. EEG corresponding to the attacks des-cribed above was not gotten.

Diphenylhydantoin and phenobarbital are effective and the attacks have been greatly reduced or abolish-ed.