Moyamoya angiopathy – Is there a Western phenotype?

Moyamoya disease (MMD) is a chronic cerebrovascular disease involving progressive bilateral stenosis of the intracranial segments of the internal carotid arteries. It results in the development of a rich, but friable collateral supply, prone to rupture. The disease is well described in Japanese literature and was originally thought to be a predozminantly Eastern disease. However, the recent literature describes a Western phenotype that may present with a different clinical course. This review aims to describe the variations in the epidemiology of the MMD between Eastern and Western populations, the possible reasons for them and highlight their implications for clinical practise and future research.