Hoshino Haruhiko, Izawa Yoshikane, Suzuki Norihiro
Department of Neurology and Stroke Center, Tokyo Saiseikai Central Hospital, Tokyo, Japan.
Neurol Med Chir (Tokyo). 2012;52(5):295-8. doi: 10.2176/nmc.52.295.
To clarify the clinical features of moyamoya disease in Japan, 941 patients with definite moyamoya disease were analyzed from the databases constructed by the Research Committee on Moyamoya Disease, established by the Ministry of Health, Labour and Welfare. Moyamoya disease occurs much more frequently among women than men, with a female-to-male ratio of 1.98. A family history of the disease was observed in 14.9%. The age at onset was characterized by two peaks: one at 5-9 years and another lower peak at around 40 years. Initial clinical features were transient ischemic attack in 46%, infarction in 20%, hemorrhage in 21%, headache in 6%, and epilepsy in 4%. The distribution of the age at onset showed one peak at around 40 years in patients with hemorrhage but two peaks in patients with ischemia.
为明确日本烟雾病的临床特征，我们对厚生劳动省设立的烟雾病研究委员会构建的数据库中的941例确诊烟雾病患者进行了分析。烟雾病在女性中的发病率远高于男性，女性与男性的比例为1.98。14.9%的患者有该病家族史。发病年龄有两个高峰：一个在5-9岁，另一个较低的高峰在40岁左右。初始临床特征为短暂性脑缺血发作占46%，梗死占20%，出血占21%，头痛占6%，癫痫占4%。发病年龄分布在出血患者中约40岁有一个高峰，而在缺血患者中有两个高峰。
