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(Stroke. 2008;39:42.)
© 2008 American Heart Association, Inc.

Original Contributions

Prevalence and Clinicoepidemiological Features of Moyamoya Disease in Japan

Findings From a Nationwide Epidemiological Survey

Shinichi Kuriyama, MD, PhD; Yasuko Kusaka, MD, PhD; Miki Fujimura, MD, PhD; Kenji Wakai, MD, PhD; Akiko Tamakoshi, MD, PhD; Shuji Hashimoto, PhD; Ichiro Tsuji, MD, PhD; Yutaka Inaba, MD, PhD Takashi Yoshimoto, MD, PhD

From the Division of Epidemiology, Department of Public Health and Forensic Medicine (S.K., I.T.), Tohoku University Graduate School of Medicine; the Department of Neurosurgery (Y.K.), Jikei University School of Medicine; the Department of Neurosurgery (M.F., T.Y.), Tohoku University Graduate School of Medicine; the Department of Preventive Medicine/Biostatistics and Medical Decision Making (K.W.), Nagoya University Graduate School of Medicine; the Division of Clinical Trials (A.T.), National Center for Geriatrics and Gerontology; the Department of Hygiene (S.H.), Fujita Health University School of Medicine; and Department of Epidemiology and Environmental Health (Y.I.), Juntendo University School of Medicine.

Correspondence to Shinichi Kuriyama, MD, PhD, 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. E-mail kuriyama-thk{at}umin.ac.jp

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).

Key Words: epidemiology • Japan • moyamoya disease • prevalence