Epidemiology

Points of epidemiological note in Moyamoya disease are:

• it is most commonly observed in East Asian populations with an annual incidence of 0.9 –4/100 000 inhabitants (1)
• it can occur at any age, including childhood, with a female to male ratio of 1.5:1. The age of onset of the symptomatic disease has two peak distributions: 5 to 9 years of age and 45 to 49 years of age (2)
• there is no relation to risk factors such as diabetes, hypertension, hyperlipidaemia, vasculitis
• patients have increased concentration of angiogenic factor
• there is a familial occurrence of 7% - this complicates several disorders with a genetic origin, including Down's syndrome and Fanconi's anaemia
• adult Moyamoya disease is rare in non-Japanese populations but the number of patients being diagnosed in Europe is increasing because of increased awareness of the condition (3)
• asymptomatic disease can occur (4)

References

1. Asselman C, Hemelsoet D, Eggermont D, et al; Moyamoya disease emerging as an immune-related angiopathy. Trends Mol Med. 2022 Nov;28(11):939-950
2. Zhang X, Xiao W, Zhang Q, et al. Progression in Moyamoya Disease: Clinical Features, Neuroimaging Evaluation, and Treatment. Curr Neuropharmacol. 2022;20(2):292-308
3. Khan N, Yonekawa Y; Moyamoya angiopathy in Europe: the beginnings in Zurich, practical lessons learned, increasing awareness and future perspectives. Acta Neurochir Suppl. 2008;103:127-30.
4. Kuroda S; Asymptomatic moyamoya disease: literature review and ongoing AMORE study. Neurol Med Chir (Tokyo). 2015;55(3):194-8