MOYAMOYA DISEASE: A STORY OF MULTIPLE STROKES

Abstract

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Moyamoya disease (MMD) is a specific cerebrovascular disease first described in 1957 by Japanese surgeons, characterized by stenosis or occlusion of the terminal portions of bilateral internal carotid arteries (ICA) with development of fragile small vessel collateral blood vessels. It may cause ischemic attacks or cerebral infarction, more frequent in children than adults. Incidence peaks in two different age groups: children who are approximately 5 years of age and adults in their mid 40's. Initial presentation is that of TIA or cerebral ischemia/hemorrhage. CASE PRESENTATION: 46-year-old female with history of hypertension initially presented with right sided tingling and numbness, CT head was negative, CTA head and neck significant for left ICA stenosis, she received tPA. Underwent full neurology work up including MRI no acute stroke or acute bleeding, MRA head and neck showed >80% narrowing of left ICA, etiology at that time was unknown. Followed by neurology clinic with imaging every 6 months; MRI Brain/MRA two years after initial presentation significant for left ICA supraclinoid occlusion and right supraclinoid moderate stenosis, left hemisphere supply via leptomeningeal collaterals from posterior cerebral artery (PCA) and anterior cerebral artery (ACA) consistent with MoyaMoya Disease. She subsequently had left superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis. Remained asymptomatic for two more years until gradual onset of forgetfulness and memory loss. Repeat imaging at that time demonstrated evolving ischemia of frontal region and high-grade stenosis of bilateral internal carotid arteries, she underwent an additional intracranial bypass. DISCUSSION: Moyamoya disease is a chronic, occlusive cerebrovascular disease involving bilateral stenosis of ICA or occlusion of the terminal portions of MCA and ACA. There are four types of MMD: ischemic, hemorrhagic, seizures, or "other;” hemorrhagic being more common in adults. Initial presentation consists of symptoms of TIA or stroke like symptoms including severe headaches, unilateral weakness, or memory changes. On angiography, characteristic stenosis or occlusion of bilateral terminal portion of ICAs as well as the moyamoya vessels are seen at the base of the brain. Cases with milder symptoms can be medically managed, while more severe symptomatic cases are treated with revascularization such as STA-MCA anastomosis. However, these procedures do not prevent progression of disease and patients may require multiple procedures to prevent ischemic episodes. CONCLUSIONS: Moyamoya disease is a chronic, occlusive cerebrovascular disease involving bilateral ICA, initial symptoms are those of TIA or stroke. Milder cases can be managed medically but more severe cases require surgical intervention, however, this does not slow down progression of disease. Reference #1: Burke, G., Burke, A., Sherma, A., Hurley, M., Batjer, H., Bendok, B (2009). Moyamoya Disease: A Summary. Journal of Neurosurgery. https://doi.org/10.3171/2009.1.FOCUS08310 Reference #2: Fukui M, Kono S, Sueishi K, Ikezaki K. Moyamoya disease. Neuropathology. 2000;20 Suppl:S61-S64. https://doi.org/10.1046/j.1440-1789.2000.00300.x Reference #3: Scott, M., Smith, E. (2009). Moyamoya Disease and Moyamoya Syndrome. The New England Journal of Medicine. 2009; 360: 1226-37. http://moya-moya.org/wp-content/uploads/2011/05/moya-moya.pdf DISCLOSURES: no disclosure on file for Victor Montalvan; No relevant relationships by Brandon Rogers, source=Web Response No relevant relationships by Jasmine Sekhon, source=Web Response