The diagnosis of moyamoya disease requires exclusion of a number of conditions including those associated with vasculitis or thrombosis. A patient with systemic lupus erythematosus (SLE) may develop findings of moyamoya syndrome.
Patient selection: systemic lupus erythematosus
Clinical findings:
(1) presentation with headache, seizure, stroke, transient ischemic attack or other neurologic finding
(2) evidence of risk factor for thrombosis (cerebral vasculitis, lupus anticoagulant with thrombosis, etc)
(3) changes on cerebral angiography of moyamoya disease (stenosis or occlusion of major intracranial arteries with development of collateral vessels)
Classification becomes more difficult when the patient has a family history of moyamoya disease.
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