To both clarify the current clinico-epidemiological features of Moyamo
ya disease in Korea as well as compare these cases with Japanese patie
nts, 451 cases were collected from 26 Korean major neurosurgical insti
tutes and 296 definite cases were analyzed statistically. Although the
Korean age distribution patterns of Moyamoya disease showed two peaks
, similar to Japanese patients, the Korean pattern was shifted to the
right thus indicating Korean adult population to be 20% higher than th
at of Japanese patients. The female/male ratio was 1:3, which was slig
htly less than that for Japanese. The family occurrence rate in Korean
s was 1.8%. The incidence of cerebral infarction and bleeding in Korea
ns was higher while transient ischemic attack (TIA) and seizure were l
ess than that of Japanese. The incidence of infarction in children and
of hemorrhage in children and adults were also statistically higher i
n Koreans. The incidence of hemorrhage was higher in females than in m
ales. Both the age at onset and sex affected the disease type. Single
encephalo-duro-arterio-synangiosis (EDAS) was performed on 87.6% of al
l surgical cases. Although the incidence of bleeding was higher in Kor
ea, the outcomes of the patients were similar to that of the Japanese
patients. Although Korean Moyamoya disease showed a relatively higher
incidence of hemorrhage and adult onset, the overall clinical backgrou
nd was similar to that of Japanese patients. (C) 1997 Elsevier Science
B.V.