Moyamoya disease in Korea



In Korea (South Korea), the second largest number of the patients with moyamoya disease is reported. Between 1976 and 1994, 334 patients with definite moyamoya disease were treated in the neurosurgical centers in Korea. There were 213 adults (63.8%) and 121 children (36.2%). Hemorrhagic manifestation was observed in 144 patients while 126 patients were with ischemic manifestation.


There were many similarities between moyamoya disease in Korea and Japan. Age distribution has two peaks at the age of 6-15 and 31-40. In Korea, adults' patients prevail over children's ones. In general, children presented with ischemic symptoms and adults with hemorrhagic ones. In Korea, there was no sexual difference in incidence. Familial incidence was low (1.5%).

Bypass surgery was less common in Korea. Whatever clinical manifestations were, 24% of adults and 62% of children underwent bypass surgery. Eighty two percents of bypass surgery was indirect. Surprisingly, direct anastomosis (STA-MCA anastomosis) was performed only in 9 patients. Various drugs were administered. Among them, anticonvulsants were most common (72.2%), which were followed by steroids, anti-hypertensive drugs, fibrinolytic drugs, anti-fibrinolytic drugs, etc. Fair clinical outcome was observed in 65.6% of the patients at initial presentation, and 73.1% at the follow-up. Eleven patients (10 adults and one child) died.


In summary, moyamoya disease in Korea had the following characteristics: more adults patients, hemorrhagic presentation, no sexual difference, and less bypass surgery.


Ref. Han DH, et al.