[A case of Sturge-Weber syndrome with severe temper tantrum]

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.