Objectives: Moyamoya syndrome (MMS) is a subset of Moyamoya disease (MMD) with a systemic comorbidity. Due to lack of direct comparisons between various MMS subgroups and their MMD counterparts, we aimed to analyze differences in stroke-related outcomes between MMS subgroups and MMD patients through propensity score matching (PSM).
Methods: We analyzed the National Inpatient Sample (NIS) from 2011-2020 for patients with MMD, and common MMS-related comorbidities: Down syndrome (DS), sickle-cell disease (SCD), and type 1 neurofibromatosis (NF1). PSM was performed to match patients of each MMS subgroup to patients with MMD-only, controlling for stroke risk factors. Rates of ischemic strokes, hemorrhagic strokes, and transient ischemic attacks (TIA) were evaluated post-matching.
Results: A total of 10652 patients with a Moyamoya diagnosis were identified: 7971 with MMD-only, 2202 with MMS-SCD, 162 with MMS-NF1, and 317 with MMS-DS. PSM for MMD-only and MMS-SCD resulted in 630 matched pairs. After matching, univariate analysis showed patients with MMS-SCD had significantly lower rates of ischemic strokes (8.9% vs 17.9%; p=0.001), hemorrhagic strokes (5.1% vs 8.1%; p=0.031), and TIAs (2.2% vs 4.6%; p=0.020) than patients with MMD only. For the MMS-DS subgroup, 92 matched pairs showed significantly lower rates of TIAs than MMD-only patients (0% vs 6.5%; p=0.013). Comparing 54 matched pairs of MMD-only and MMS-NF1 patients showed no significant differences in stroke-related outcomes.
Conclusion: Patients with MMS-SCD had significantly lower rates of acute ischemic stroke, hemorrhagic stroke, and TIA compared to MMD-only patients. Future research should consider the source of these differences in improving stroke prevention in MMD patients.
Keywords: down syndrome; moyamoya disease; moyamoya syndrome; neurofibromatosis; sickle cell; stroke.
Copyright © 2024. Published by Elsevier Inc.