Prevalence and clinicoepidemiological features of moyamoya disease in Japan - Findings from a nationwide epidemiological survey
Prevalence and clinicoepidemiological features of moyamoya disease in Japan - Findings from a nationwide epidemiological survey
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DOI：
10.1161/strokeaha.107.490714
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发表时间：
2008-01-01
期刊：
影响因子：
8.3
通讯作者：
Yoshimoto, Takashi
中科院分区：
文献类型：
--
作者：
Kuriyama, Shinichi;Kusaka, Yasuko;Yoshimoto, Takashi
背景和目的，本研究的目的是估计每年的烟雾病患者人数在日本和描述的clinicoepidemiological features of the disease. Methods的研究包括2个问卷调查，其中被分配到随机选择的神经外科，内科，神经科，脑血管内科，儿科在日本各地的医院。第一次调查询问了2003年接受治疗的患者人数，第二次调查要求提供更多关于第一次调查中确定的每个患者的详细临床流行病学信息。结果-2003年，在日本接受治疗的患者总数估计为7700（95%置信区间，6300至9300）。患者的性别比（女性与男性）为1.8。对于男性，烟雾病的高峰期在10至14岁的患者和20至24岁的女性患者中观察到。2003年的年新诊断病例率为每10万人0.54例。12.1%的患者有烟雾病家族史。大多数（77.9%）被视为outpatient. Conclusions，虽然在本研究中的患者的clinicoepidemiological功能几乎相似，在以前的那些，烟雾病在日本的估计患病率几乎翻了一番，在最近的十年（3900在1994年和7700在2003年）。增加的部分原因是新诊断病例的增加（1994年每100 000人0.35例，2003年每100 000人0.54例）。
Background and Purpose-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.Methods-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.Results-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.Conclusions-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).