Kuriyama Shinichi, Kusaka Yasuko, Fujimura Miki, Wakai Kenji, Tamakoshi Akiko, Hashimoto Shuji, Tsuji Ichiro, Inaba Yutaka, Yoshimoto Takashi
Division of Epidemiology, Department of Public Health and Forensic Medicine, Tohoku University Graduate School of Medicine, 2-1, Seiryo-machi, Aoba-ku, Sendai, 980-8575, Japan.
Stroke. 2008 Jan;39(1):42-7. doi: 10.1161/STROKEAHA.107.490714. Epub 2007 Nov 29.
The objectives of the present study were to estimate an annual number of patients with moyamoya disease in Japan and to describe the clinicoepidemiological features of the disease.
The study consisted of 2 questionnaire surveys, which were distributed to randomly selected departments of neurosurgery, internal medicine, neurology, cerebrovascular medicine, and pediatrics in hospitals throughout Japan. The first survey inquired about the number of the patients treated in 2003, and the second requested additional detailed clinicoepidemiological information about each patient identified in the first survey.
In 2003, the total number of patients treated in Japan was estimated at 7700 (95% confidence interval, 6300 to 9300). Sex ratio (women to men) of the patients was 1.8. For men, the peak of moyamoya disease was observed in patients aged 10 to 14 years and for women aged 20 to 24 years. Annual rate of newly diagnosed cases in 2003 was 0.54 per 100,000 population. Family history of moyamoya disease was found in 12.1% of the patients. The majority (77.9%) were treated as outpatients.
Although the clinicoepidemiological features of the patients in the present study were almost similar to those obtained in previous ones, the estimated prevalence of moyamoya disease in Japan has almost doubled during the recent decade (3900 in 1994 and 7700 in 2003). The increase could partly be explained by the increase in newly diagnosed cases (0.35 in 1994 and 0.54 in 2003 per 100,000 population).
本研究的目的是估算日本每年患烟雾病的患者数量，并描述该疾病的临床流行病学特征。
该研究包括两次问卷调查，问卷被分发给日本各地医院随机挑选的神经外科、内科、神经内科、脑血管病科和儿科。第一次调查询问了2003年治疗的患者数量，第二次调查要求提供有关在第一次调查中确定的每位患者的更多详细临床流行病学信息。
2003年，日本治疗的患者总数估计为7700例（95%置信区间为6300至9300）。患者的性别比（女性与男性）为1.8。男性烟雾病发病高峰出现在10至14岁的患者中，女性则出现在20至24岁的患者中。2003年新诊断病例的年发病率为每10万人0.54例。12.1%的患者有烟雾病家族史。大多数患者（77.9%）作为门诊患者接受治疗。
尽管本研究中患者的临床流行病学特征与先前研究所得结果几乎相似，但日本烟雾病的估计患病率在最近十年中几乎翻了一番（1994年为3900例，2003年为7700例）。这种增加部分可以用新诊断病例的增加来解释（1994年每10万人中有0.35例，2003年为0.54例）。
