鏡像運動と症候性てんかんを示した裂脳症の1症例

A Case with Symptomatic Epilepsy and Mirror Movement due to Unilateral Schizencephaly

　症例は47歳男性。主訴は意識消失発作。4歳時，28歳時，47歳時に左上下肢から始まる全身けいれん発作が発現した。独歩・有意語は4歳時。47歳時神経学的所見では顔面を含む左半身の筋肉・骨格の発育不全と左手指と左足趾の鏡像運動（mirror movement），左片麻痺を認めた。脳MRI所見から，一側性（右側）の裂脳症（schizencephaly；open lip type）によるてんかん発作と診断し，バルプロ酸を投与した。脳血流シンチでは，病変部位の異常灰白質に集積を認めた。本症例の鏡像運動について，文献的考察を加え報告した。

Schizencephaly is an extremely rare congenital dis-ease caused by abnormal neuronal migration. The eti-ology of schizencephaly is not established but vascular disturbance in early childhood could cause this condi-tion. We have cared of a patient with schizencephaly. The patient was 47 year old male. He had focal motor seizure with secondary generalization. Neurological examination revealed, mild left hemiparesis, left py-ramidal signs with no sensory impairment, left he-miatrophy, and mirror movement. MRI findings showed schizencephaly, open lip type (type II) in right cerebral hemisphere.