Moyamoya disease beyond Japan and East Asia

Moyamoya disease (MMD) was first described in Japan and is primarily recognized in East Asia. Although rare, it is increasingly diagnosed in Western countries. A Western phenotype - characterized by a lower risk of hemorrhage - has been proposed in a previous review article in this journal.

This narrative review explores MMD from a Western perspective.

Drawing partly on studies from a Danish MMD cohort, the author suggests that MMD exhibits similar features across different regions. Nordic national registries and well-maintained clinical records provide valuable data for gaining a deeper understanding of this disease.

In our data, the overall Kaplan-Meier 5-year stroke risk was 10%. We observed a trend toward higher stroke risk in females and in those with angiographic progression.