Ethnic Variation in Moyamoya Angiopathy in a Diverse New Zealand Population: A Population-Based Cohort Study.

Introduction: Moyamoya angiopathy (MMA) has been reported in the ethnically diverse Auckland region of New Zealand, but the sociodemographic burden and clinical outcomes remain poorly characterised. This study aims to determine age, sex, and prevalence of MMA stratified by ethnicity, and to assess clinical outcomes in adults residing in Auckland (population 1.9 million).

Methods: A retrospective review of patient records and radiology reports from 2008 to 2025 was conducted using ICD codes and keyword searches. Prevalence was estimated using national census data. Primary outcomes were functional independence (modified Rankin Score 0-2) and the composite of stroke or transient ischaemic attack (TIA). Associations were assessed using univariate and multivariate Cox regression. A pooled analysis of published cohorts was also performed for context.

Results: A total of 100 patients were identified (73% female; mean age 38.5 years, SD 17). Period prevalence was highest among Pacific peoples (11/100,000), followed by Māori (6/100,000), Asians (4/100,000), and Europeans (2/100,000). Overall prevalence increased from 0.8 to 4.5 per 100,000 between 2001 and 2025 (p<0.0001). During a median follow-up of 4.2 years, 39% experienced a cerebrovascular event, at a median of 647 days from diagnosis. Two-thirds remained functionally independent. Bilateral internal carotid artery involvement (HR 0.52, 95% CI 0.30-0.91) and recurrent cerebrovascular events (HR 0.54, 95% CI 0.31-0.94) were associated with poorer outcomes, while antithrombotic use was protective (HR 2.1, 95% CI 1.11-3.9). Outcomes were consistent with other international cohorts.

Conclusion: This population-based study highlights marked ethnic variation in MMA prevalence, with the highest rates in Pacific peoples, and an elevated risk of early cerebrovascular events. These findings have implications for timely diagnosis and targeted management in diverse populations.