Moyamoya Angiopathy in Southeast Asians: Systematic review of literature with an illustrative case

Abstract

Background: Moyamoya angiopathy (MMA) is a rare cause of stroke, yet its incidence and outcomes are understudied in Southeast Asia, despite being the third most populous geographical region in the world. Our aim was to enhance understanding of MMA in this population and present a case from our experience.

Methodology: A systematic literature search on MMA in the Southeast Asian population was performed via PubMed, Scopus, and Wiley Online Library, including local journals and databases up until May 2024. Descriptive statistics were used to synthesize data. We also present a case of a 36-year-old Filipino female diagnosed with MMA followed by superficial temporal artery-middle cerebral
artery (STA-MCA) bypass and resolution of symptoms.

Results: Out of the 275 articles retrieved, 23 articles detailing 37 patients of Southeast Asian nationality were included in this review. Stroke predominated (76.3%, n=29) as initial presentation, followed by seizures (36.8%, n=14), movement disorders (n=4, 10.5%) and headaches (n=4, 10.5%). Treatment included antiplatelet therapy for 57.9% (n=22), and anti-seizure medications for 26.3%
(n=10) of cases. Only 21% (n=8) underwent surgical intervention, primarily via direct bypass. Outcomes based on the Modified Rankin Scale (MRS) showed 94.7% (n=36) had retained a functional score of MRS ≤2.

Conclusion: Our review underscores the scarcity of data on MMA in Southeast Asians, and highlights the varied clinical presentation. Effective management requires a tailored approach, with surgical management showing favorable outcomes. Future research should focus on longitudinal studies to assess long-term outcomes, explore the genetic and environmental factors, and develop standardized treatment protocols to improve patient care in this ethnically diverse population.