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本文报道一家族中数名成员中年期患发作性局灶性脑机能障碍,部分出现严重运动障碍伴假性球麻痹或皮层性痴呆。该家族共14名成员经头部 CT 和 MRI 检查,3例还作了血管造影。例Ⅱ-6.男,42岁,1967年突发右侧轻偏瘫伴构音障碍,1周内恢复。1976年和1978年出现两次右偏瘫。第3次右偏瘫时 CT 扫描示双侧脑白质和左侧内囊低密度区,但脑血管造影正常。1978年出现进行性智力衰退。1991年神经病学检查示右侧痉挛性偏瘫,双侧 Babinski 征,构音
This article reports that several members of a family have middle-aged onset-onset focal brain dysfunction and some have severe dyskinesias with pseudobulbar palsy or cortical dementia. A total of 14 members of the family were examined by CT and MRI of the head. Three patients underwent angiography. Case Ⅱ-6. Male, 42 years old, 1967 sudden right hemiplegia accompanied by dysarthria, recovery within 1 week. Right hemiparesis occurred twice in 1976 and in 1978. The third right hemiparesis CT scan showed bilateral white matter and left inner capsule low density area, but normal cerebral angiography. In 1978 there was a progressive intellectual decline. Neurological examination in 1991 showed right spastic hemiplegia, bilateral Babinski sign, and consonants