Moyamoya is a rare vasculopathy we come across in practice and here we discuss a rare etiology and steps for management of this.

Moyamoya pattern is a cerebral vasculopathy resulting in proximal intracranial vascular stenosis with distal collateral formation. Incidence of moyamoya disease in the US is 0.086 per 100,000 people .Rarely Graves' disease has been associated with moyamoya vasculopathy.

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45-year-old  female with known past medical history of Graves' disease with orbitopathy (non-adherent to medications), paroxysmal atrial fibrillation (on apixaban), prior ischemic stroke  who was admitted for evaluation of acute onset dysarthria and aphasia. Neurological examination revealed right facial droop, expressive aphasia and dysarthria.

Labs were remarkable for poorly controlled hyperthyroidism [TSH( <0.01); free T4(2.45); free T3 (6.6); Thyrotropin receptor antibody ( 27.50 ); thyroid stimulating immunoglobulin (8.49)].

MRI Brain showed multiple scattered subacute watershed infarcts in the Left MCA territory.

CTA Head and Neck showed chronic left M1 occlusion, chronic right M2 occlusion, concerning for moyamoya vasculopathy. DSA confirmed bilateral Moya Moya vasculopathy.

Patient was treated medically with Methimazole and beta blocker in addition to secondary stroke prophylaxis with Aspirin and atorvastatin. 3 months later, she underwent Left STA - MCA bypass surgery. Since then she has not had any recurrent strokes.

Graves’ disease is a rare etiology of MMS. Close monitoring of thyroid hormones and good control of the disease is the first line of management. Revascularization procedures can be adopted if patient fails medical management or if ischemic attacks have already occurred .  Future research comparing medical management, surgical management and combination will be helpful.