Moyamoya-like vasculopathy (MMV) is a rare, chronic, progressive cerebrovascular disorder characterized by stenosis or occlusion of the terminal portion of the bilateral internal carotid arteries and development of abnormal collateral vessels at the base of the brain. This disorder develops in association with various systemic diseases and conditions, including neurofibromatosis type 1, Down syndrome, thyroid disease, radiation therapy, and autoimmune disease. We report a case of a 51-year-old female patient with low-activity systemic lupus erythematosus (SLE) who had a sudden onset of global aphasia and right hemiplegia. Three months previous, she had been on antiplatelet medication due to a single transient ischemic attack. Brain magnetic resonance imaging demonstrated a massive infarct of the left middle cerebral artery territory. Conventional angiography showed complete occlusion of the left middle cerebral artery with poor development of basal collateral vessels. This case demonstrates that a patient with underlying autoimmune disease such as SLE accompanied by MMV should be considered vulnerable to ischemic stroke.
