Prevalence and Clinicoepidemiological Features of Moyamoya Disease in Japan. Findings From a Nationwide Epidemiological Survey

TLDR: The estimated prevalence of moyamoya disease in Japan has almost doubled during the recent decade and the clinicoepidemiological features of the patients in the present study were almost similar to those obtained in previous ones.

Abstract: 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. Fam...