Moyamoya syndrome as an unusual presenting manifestation of systemic lupus erythematosus in a young woman

Objective: To describe a rare case of moyamoya syndrome associated with systemic lupus erythematosus (SLE).Presentation and Intervention: A 22-year-old woman presented with left-sided hemiparesis in addition to fever, fatigue and malar rash. Brain magnetic resonance imaging revealed acute infarction in the right middle cerebral artery territory. Cerebral angiography showed features of moyamoya syndrome. Brain biopsy showed evidence of vasculitis. She responded well to steroid therapy. Conclusion: This case represented a rare co-occurrence of SLE-related vasculitis and moyamoya syndrome. Early vascular imaging is critical in patients with SLE and suspected ischemic stroke.