Moyamoya Disease Epidemiology

For an unknown reason, Moyamoya Disease is relatively common in people living in East Asian countries such as Korea and Japan, as compared to those in the Western Hemisphere.

According to a survey performed in Japan in 1995, the prevalence of MMD was approximately 3.16/100,000, with an estimated incidence of 0.35/100,000 ¹⁾.

A subsequent survey in 2004 showed that the number of patients considerably increased, with an estimated incidence of 0.54/100,000, and prevalence of 6.03/100,000 in 2003

Another study performed in Hokkaido, Japan ²⁾, showed that the incidence and prevalence were 0.94/100,000 and 10.5/100,000, respectively, based on 267 newly diagnosed MMD patients between 2002 and 2006. The male-to-female ratio was 1:1.8 or 1:2.2 in these surveys, and approximately 10%-15% of patients had a family history. The risk of having MMD in family members is about 30-40 times higher than the general population ³⁾ ⁴⁾.

There were two peaks of incidence, at 10-20 and 35-50 years old.

Sometimes as a conditions associated with aneurysms

Moyamoya Disease Epidemiology outside of Asia.

¹⁾ , ³⁾

Wakai K, Tamakoshi A, Ikezaki K, et al. Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg. 1997;99 Suppl 2:S1-S5. doi:10.1016/s0303-8467(97)00031-0

²⁾

Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry. 2008;79(8):900-904. doi:10.1136/jnnp.2007.130666

⁴⁾

Kuriyama S, Kusaka Y, Fujimura M, et al. Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey. Stroke. 2008;39(1):42-47. doi:10.1161/STROKEAHA.107.490714