From January 1978 to December 1995, 92 cases of Moyamoya disease were
collected from seven major medical centers in Taiwan. The data gave an
annual incidence rate of 0.048 per 100 000 population. There were 40
males and 52 females and the ages ranged from 2 to 62 years with the p
eak incidence in the 31-40 year age group (23 cases). Cerebral infarct
ion occurred in 20 out of 24 juvenile patients (83%), and in 24 out of
68 adult patients (35%). The difference was statistically significant
. Haemorrhagic stroke was more frequent in adult patients. Computed to
mographic scans following stroke showed cerebral infarction in 44 case
s, ventricular haemorrhage in 26 cases, intracerebral haemorrhage in 1
4 cases and pure subarachnoid haemorrhage in eight. The most frequent
initial symptom was motor disturbance (59%), followed by headache (49%
) and impaired consciousness (35%). This survey showed an incidence ra
te much lower than that in Japan, but comparable with those in other O
riental countries and higher than those in Western countries. The male
-to-female ratio once differed considerably from that of the Japanese
series, but from the present study is now quite similar. (C) 1997 Else
vier Science B.V.