A clinical comparison of definite Moyamoya disease between South Korea and Japan

Background and Purpose The goal of the present study was to clarify whether South Korean patients with moyamoya disease have clinical features similar to those of Japanese patients. Methods From 26 South Korean neurosurgical institutes, 296 definite cases were collected and analyzed statistically. These cases were then compared with 731 Japanese definite cases registered to the Research Committee on Moyamoya Disease of the Ministry of Health and Welfare, Japan. Results The Korean age distribution patterns showed two peaks that were similar to those seen in Japanese patients. The incidence of adult moyamoya disease in South Korea, however, was 20% higher than that in Japanese patients. The family occurrence rate was 1.8% in Koreans. The incidence of cerebral infarction and bleeding in Koreans was statistically higher, whereas transient ischemic attack and seizure were less than those in Japanese subjects. The incidence of infarction in children and that of hemorrhage in both children and adults were also statistically higher in Koreans. The incidence of hemorrhage was higher in females than in males. Both the age at onset and sex-affected the disease type. Although most Japanese patients underwent direct bypass surgery and/or combined indirect bypass procedures, single encephaloduroarteriosynangiosis was performed on 87.6% of all surgical cases in Koreans. Despite the higher incidence of hemorrhagic type in South Korea, the outcomes of the patients were similar to those of the Japanese patients. Conclusions This study suggests that the clinical background of moyamoya disease in South Korea is essentially similar to that in Japan.