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Novel epidemiological features of moyamoya disease
  1. Takeo Baba (baba{at}
  1. Shinsapporo Neurosurgical Hospital, Japan
    1. Kiyohiro Houkin (houkin{at}
    1. Sapporo Medical University, Japan
      1. Satoshi Kuroda (skuroda{at}
      1. Hokkaido University Graduate School of Medicine, Japan


        Background: Many clinical features specific to moyamoya disease have been reported and cited in textbooks based on previous data. The purpose of this study is to investigate the present epidemiological features of moyamoya disease based on recently obtained regional all-inclusive data.

        Methods: The authors have performed an all-inclusive survey of moyamoya disease in Hokkaido, one of the major islands in Japan with a population of 5.63 million. The epidemiological features were analyzed based on the data from 267 newly registered patients with moyamoya disease in Hokkaido from 2002 to 2006. These analyzed data were adjusted to whole Japanese population at 2005.

        Results The detection rate of the disease per year was 0.94 patients per 100,000 people, and prevalence was 10.5 patients per 100,000 people. The incidence of ischemia concerned with the disease was 0.53 patients, and hemorrhage was 0.2 patients. The ratio of female to male patients was 2.18. The ratio of patients 10 and above to under 10 years of age at onset was 6.18. Two peaks for age of onset were seen; the highest observed between 45 and 49 years, and the second between 5 and 9 years. Asymptomatic patients comprised 17.8% of the total number of patients.

        Conclusion:The epidemiological features of moyamoya disease determined by this survey varied considerably from previous data. The detection rate and prevalence of the disease were higher than those reported previously. The highest peak of onset age was older than those reported previously. In addition, it was revealed that asymptomatic moyamoya patients are not always rare in Japan.

        • Age distribution
        • Epidemiological features
        • Moyamoya disease

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