Disclosures: Charnette Lercara, MD: No financial relationships or conflicts of interest

Case Description: The patient presented with facial droop, right-sided hemiparesis and aphasia followed by episodes of generalized tonic clonic seizures. She was found to have evolving bilateral ACA/MCA ischemic strokes with leptomeningeal hyperintensities on MRI. Cerebral angiogram revealed bilateral ICA/MCA stenosis with moyamoya type vascularization around bilateral MCAs (L>R). Labs revealed an elevated CRP with serology positive for ANA, anti-smith and high titre anti-chromatin antibodies. The patient underwent a left EDAS (encephalo-duro-arterio-synangiosis).

Setting: Tertiary care hospital

Patient: A 34-year-old African American female with past medical history of Graves’ disease

Assessment/Results: Two days post-op, MRI revealed an acute left MCA stroke. Our patient’s most recent stroke coupled with a negative thrombophilia workup and a new diagnosis of systemic lupus erythematosus (SLE), heightened the likelihood of CNS lupus and prompted Rheumatology to start Methylprednisolone, Hydroxychloroquine and Azathioprine. The patient was subsequently discharged to acute rehab for further recovery.

Discussion: Moyamoya disease (MMD) is a rare, chronic, cerebrovascular disorder that is characterized by progressive stenosis or occlusion of the internal carotid artery accompanied by the formation of a fine collateral vascular network around the circle of Willis. It is most commonly seen in East Asian populations. Progressive vessel occlusion results in TIA, ischemic and hemorrhagic strokes, seizures, headache and cognitive impairment. MMD has been described alongside autoimmune conditions such as Graves’ disease and SLE. SLE and MMD both can present in young women with intracerebral events, making diagnosis difficult. In a young African American female with a history of autoimmune disease, it is important to consider CNS lupus as a contributor to stroke even in the setting of diagnosed MMD.

Conclusion: This case represents a rare co-occurrence of CNS lupus and MMD. SLE should be considered in the differential of young women who present with strokes.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/concomitant-moyamoya-disease-and-lupus-cerebritis-in-an-african-american-female-a-case-report/