Approach to adult Moyamoya disease presenting as bilateral anterior circulation infarcts: a case report.

ABSTRACT

Moyamoya disease (MMD) is a rare progressive condition of unknown etiology that affects the blood vessels in the brain and is characterized by narrowing or stenosis of the distal parts of the internal carotid and major intracranial arteries, leading to the development of fragile collateral supplies. This makes it a significant causative factor for both ischemic strokes as well as intracerebral hemorrhages. As per the recommendations by the Research Committee on Moyamoya disease in 2021, the diagnostic criteria for MMD are essentially based on indicative radiological findings on cerebral angiography or MRI Brain and MRA, after excluding possible differentials. In this case report, we present the case of a 45-year-old Asian lady who presented with sudden-onset headaches and right-sided weakness. Her admission non-contrast CT brain scan was undiagnostic. MRI brain and MRA scans revealed bilateral anterior circulation watershed infarcts of variable age along with supra-clinoid stenosis. She was initially treated with steroids for vasculitis after discussions with a multidisciplinary team involving neurologists, stroke physicians and neuro-radiologists; however, further imaging revealed a diagnosis of MMD, and she was subsequently referred for revascularization surgery. Given the limited number of similarly documented cases, we hope this report will contribute to the knowledge base.