Cerebrovascular occlusive disease with and without the moyamoya vascular network in children

Clinical features of cerebrovascular occlusive disease with the moyamoya network (group 1: twenty-nine children) and those without this network (group 2: nine children) are reported herein. Group 1 was characterized by female preponderance, recurrent and transient ischemic attacks, progression of mental deterioration, rebuild-up after hyperventilation on EEG and wide-spread lesions on CT scan. Group 2 had one or two attacks which led to a rather long-lasting hemiplegia but not to mental deterioration, rare rebuild-up findings on EEG and unilateral focal lesion on CT scan. Precipitating factors for ischemic attacks included deep breathing, changes in body temperature or sleep-waking transition were present in both groups. Etiology in some children was considered to be congenital.