Abstract
Moyamoya disease is a chronic occlusive cerebrovascular disease affecting young patients. Its association with systemic lupus erythematosus (SLE) has not been well established though had been reported. Their similar acute neurological presentations can lead to diagnostic dilemma. We present a young Chinese female with known SLE initially presenting with unilateral moyamoya phenomenon, subsequently progressed to typical bilateral moyamoya disease 5 years later. We also compare various clinical parameters with other reported cases in past literatures.
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