Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, Kojima M, Lin Y, Ohno Y
Department of Preventive Medicine, Nagoya University School of Medicine, Japan.
Clin Neurol Neurosurg. 1997 Oct;99 Suppl 2:S1-5. doi: 10.1016/s0303-8467(97)00031-0.
To estimate an annual number of patients treated for Moyamoya disease in Japan and to describe the clinico-epidemiological features, a nationwide epidemiological survey was conducted in 1995. The study consisted of two questionnaires, which were distributed to departments randomly selected, of neurosurgery, neurology and pediatrics in hospitals throughout Japan. The first questionnaire inquired the number of the patients treated in 1994 and the second one detailed clinico-epidemiological information of each patient reported. Following major epidemiological findings emerged from the study: (a) The total annual number of patients treated for Moyamoya disease was estimated as 3900 (95% confidence interval (CI) 3500-4400) in Japan 1994, with the prevalence and incidence rates of 3.16 and 0.35 per 100,000 population, respectively; (b) the sex ratio (females to males) of the patients was 1.8; (c) the peak of age distribution of the patients was observed in 10-14 years old and a smaller peak in their forties; (d) the age at onset was under 10 years old in 47.8% of the patients, but some had developed the disease at the age of 25-49 years; (e) family history of Moyamoya disease was found in 10.0% of the patients; and (f) about 75% of the patients had normal activity of daily life or working ability even before treatment. The present findings were quite comparable with those obtained in the previous nationwide epidemiological survey in 1990.
为了估算日本每年接受烟雾病治疗的患者数量并描述其临床流行病学特征，1995年开展了一项全国性的流行病学调查。该研究由两份问卷组成，问卷被分发给日本各地医院随机抽取的神经外科、神经内科和儿科科室。第一份问卷询问了1994年治疗的患者数量，第二份问卷详细记录了所报告的每位患者的临床流行病学信息。该研究得出了以下主要流行病学发现：(a) 1994年日本每年接受烟雾病治疗的患者总数估计为3900例（95%置信区间(CI) 3500 - 4400），患病率和发病率分别为每10万人3.16例和0.35例；(b) 患者的性别比（女性与男性）为1.8；(c) 患者年龄分布的高峰出现在10 - 14岁，四十多岁时出现一个较小的高峰；(d) 47.8%的患者发病年龄在10岁以下，但有些患者在25 - 49岁时发病；(e) 10.0%的患者有烟雾病家族史；(f) 约75%的患者即使在治疗前日常生活活动或工作能力也正常。目前的研究结果与1990年之前全国性流行病学调查的结果相当。
