Clearing Up The Smoke: Clinical and Radiographic Characterization of a North American Cohort of Childhood Moyamoya

Moyamoya (MMA) is a progressive cerebral steno-occlusive angiopathy which can lead to strokes. Its natural history derives from adult East Asian cohorts with idiopathic disease. Clinical differences in pediatric, non-Asian and syndromic patients are reported but understudied. This study aimed to: 1) characterize clinical and radiographic features of a North American cohort of childhood moyamoya; 2) identify risk factors of ischemic event recurrence (IEC) and poor outcome; and 3) design an IEC predictive model. Idiopathic MMA presents with transient ischemic attacks (TIA) and bilateral disease and have a higher risk of IEC while the neurofibromatosis type 1-related syndromic form tends to be unilateral and indolent. A young age, infarcts, bilateral and advanced disease predict poor outcome. Significant angiographic posterior deep collaterals and lack of leptomeningeal collaterals predict poor outcome, but good revascularization response. Asian ancestry, TIA, ophthalmo-ethmoidal, leptomeningeal, and middle meningeal collaterals and steal phenomenon on functional MRI predict IEC.M.Sc.2021-12-29 00:00:0