Moyamoya disease in Latin America: A systematic literature review with an illustrative case.

BACKGROUND

Moyamoya disease (MMD) is a rare cerebrovascular disorder involving progressive stenosis of the internal carotid arteries and abnormal collateral vessel formation. While more prevalent in East Asia, MMD also affects Latin American populations, where clinical features and outcomes are less well defined. This study provides a systematic review of MMD in Latin America, complemented by an illustrative case.

METHODS

Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. PubMed, Embase, and Scopus were searched for case reports, series, and cohort studies involving Latin American patients. Data on demographics, clinical presentation, diagnostics, treatments, and outcomes were extracted. Risk of bias was assessed using the Joanna Briggs Institute checklist.

RESULTS

Twenty-one studies comprising 47 patients from Brazil, Mexico, Colombia, Cuba, and Peru were included. Ischemic strokes were the most frequent presentation (78%), followed by hemorrhagic events (22%). Digital subtraction angiography (DSA) was the most frequently used diagnostic tool. Treatments ranged from medical therapy to surgical revascularization, mainly encephaloduroarteriosynangiosis. Across studies, most patients showed favorable outcomes, particularly those undergoing surgical intervention, while neurological sequelae were reported in a subset of cases.

CONCLUSION

In Latin America, MMD mainly presents with ischemic symptoms and is typically diagnosed through DSA. Surgical revascularization appears to be associated with improved outcomes. The rarity and heterogeneity of cases highlight the need for further research to better characterize the disease, optimize management, and enhance patient care in the region.