極めて激しいかん癪（temper tantrum）を呈したSturge-Weber症候群の1例

A Case of Sturge-Weber Syndrome with Severe Temper Tantrum

Ⅰ．は じ め に

　かん癪（temper tantrum）・攻撃性・不機嫌状態といった性格変化は，側頭葉てんかんでしばしばみられる．また，片側巨脳症，Rasmussen脳炎，乳児片麻痺，Sturge-Weber症候群といった半球離断術を要するような半球性病変においても随伴することが報告されている．今回，われわれは強いかん癪と複雑部分発作を伴ったSturge-Weber症候群を経験し，外科的治療で症状は消失した．本例におけるかん癪症状の発現機序につき，文献的考察をまじえて報告する．

　We report a case of Sturge-Weber syndrome with intractable epilepsy presenting complex partial seizures(CPSs) and severe temper tantrum successfully treated with anterior temporal lobectomy. A 23-month-old girl without facial angioma presented with seizures from 8 months after birth. Neuroradiological examination demonstrated angioma in the left temporooccipital lobes and calcification in the left occipital lobe. Thus she was diagnosed as having Sturge-Weber syndrome. Her parents sought medical advice because of intractable and more frequent CPSs and a change in temperament manifesting as severe temper tantrums. Interictal electroencephalogram (EEG) showed relatively mild abnormal slow wave discharges in the left temporal region. The patient underwent left anterior temporal lobectomy based on intraoperative electrocorticography (ECoG) findings. Histopathological examination demonstrated gliotic change in the amygdala. After the surgery,she has remained seizure-free for 14 months while taking anticonvulsants and temper tantrums ultimately showed remission 10 months following surgery. This case may indicate that severe temper tantrum was associated with a change in amygdala structures.