352 Prevalence and Charactersitics of Moyamoya Syndrome in Patients With Down Syndrome

Abstract

INTRODUCTION: Moyamoya arteriopathy can be idiopathic (moyamoya disease, MMD) or associated with other diseases, i.e., Down syndrome (moyamoya syndrome, MMS). The prevalence of Down syndrome in patients admitted for moyamoya has been estimated using the National Inpatient Sample to be 3.8%. However, the prevalence of moyamoya in Down Syndrome is not well-quantified. METHODS: This retrospective study used the Sie Center for Down Syndrome and Stroke Program databases at Children’s Hospital Colorado to identify moyamoya patients aged 30 days to 18 years at diagnosis. Demographics, clinical presentation, treatment, and outcome were compared among patients with DS, those with idiopathic MMD, and the entire group. RESULTS: Sixty-five patients (30 idiopathic, 14 MMS with DS, 21 MMS without DS). Prevalence of MMS in the DS population was 0.4%. Gender and age at presentation did not differ significantly between populations. 47% of idiopathic moyamoya patients initially presented with transient ischemic attacks (TIAs) versus 14% of DS patients (P < 0.05). This is compared to 34% of the total population that presented with TIAs. DS patients were more likely to present with stroke (57%) compared to idiopathic (37%) and total patient populations (40%) although these results were not significant. 23% of idiopathic patients presented with basal ganglia strokes compared to 7% in the DS population (P < 0.05). CONCLUSIONS: In this study, the prevalence of moyamoya in DS was 0.4%. Patients with DS were less likely to present with TIAs and more likely to present with stroke, with different stroke locations, than were other moyamoya populations.