Abstract 162: Clinical and Neuroradiological Characteristics of Moyamoya Syndrome: UT Houston Experience

Abstract

Background: Moyamoya (MM) is a rare progressive arteriopathy characterized by progressive stenosis and occlusion of the internal carotid arteries (ICA) and ultimately the arteries of the circle of Willis. It is called moyamoya disease (MMD) when idiopathic and involving bilateral ICA, and moyamoya syndrome (MMS) when associated with well-recognized risk factors, or when idiopathic but unilateral. Given the paucity of literature on MMS, we analyzed the clinical and radiological characteristics of MM patients presenting to our institute. Methods: We retrospectively identified patients admitted with acute neurological deficits and had ‘moyamoya-like’ physiology from our stroke registry (07/04 to 03/11). We collected demographics, clinical and laboratory data, and prior and subsequent hospital admissions. Two clinically blinded neuroradiologists collected radiological data including vessels involved, type of collateralization, and disease stage and progression. Patients were categorized into MMD or MMS based on risk factors and the side of disease. Results: We identified 36 MM patients (mean age 41.3 years), 13 (36.1%) with MMD and 23 (63.9%) with MMS. Both groups had female preponderance and multiracial involvement; hemiparesis was the leading clinical sign, and most patients had low HDL levels (83%). In MMS group, 4 (17.4%) patients had idiopathic unilateral disease, 4 (17.4%) had atherosclerotic disease, 3 (13%) each had vasculitis and intracranial dissection, 2 (8.7%) each had sickle cell disease and APLA syndrome, and 1 (4.3%) each had Down’s syndrome, Lupus, thyrotoxicosis, hypercoagulable disorder and chronic cocaine use. The MMS patients tended to be older, and higher proportion had ischemic stroke (87%) and neurological worsening after acute event (35%). On the other hand, higher proportion of MMD patients had hemorrhagic stroke (38.5%), multiple (>5) hospital admissions (23%) and EEG abnormality (80%) and were discharged home (62%). In MMS group, 2 (8.7%) patients underwent hemicraniectomy. In MMD group, 12.5% had disease progression radiographically. The clinical and radiological characteristics of these patients are summarized in tables 1 and 2 respectively. Conclusion: We present the single-center experience of clinical and neuroradiological characteristics of patients with MMS.