[Follow-up study on the relationship between age at onset of illness and outcome in patients with moyamoya disease].

Follow-up studies over more than one and a half years (average 6.5 years) were performed in 51 patients with "moyamoya disease". Activities of daily living (ADL) was assessed and graded into five levels both at the time of admission into hospital and at the last assessment in the follow-up study, as follows. Grade I: Fully capable of work or study with no or minimal difficulty, II: Capable of limited work or study with difficulty, III: Incapable of work or study but capable of home life alone, IV: Incapable of home life without help, V: Vegetative state or dead. Patients were divided into three groups by the age of clinical onset: younger pediatric, less than 5 years old (21 patients); older pediatric, 5 to 15 years old (14 patients); and adult, 16 years old and over (16 patients). Although analysis of the angiographical and neurological pictures is important, the present paper concerns only the age of clinical onset and whether reconstructive vascular surgery was performed, because of the small number of clinical materials. Seven of 16 adults suffered hemorrhagic attacks and 2 of them died of hemorrhagic insults. In contrast, none of the pediatric cases had hemorrhagic attacks and none died. Both at the time of admission and last assessment, ADL of younger pediatric cases was much lower than that of older ones. These results suggest that clinical course in patients with "moyamoya disease" closely related with the age of the onset of illness, and the border age of onset of illness between patients with good outcome and with poor outcome is around 5 years.(ABSTRACT TRUNCATED AT 250 WORDS)