Neuropsychological correlates of moyamoya disease: A clinical case study

OBJECTIVE: Moyamoya disease (MMD) is a rare, progressive occlusive cerebrovascular disorder characterized by stenosis of the internal carotid arteries and compensatory collateral vessel formation, often leading to ischemic or hemorrhagic events. We present the case of a 50-year-old bilingual Filipina woman with a neuroimaging-confirmed diagnosis of MMD (Suzuki grade III) who underwent staged bilateral surgical revascularization procedures (right intracranial bypass and left encephaloduroarteriosynangiosis [EDAS]), with her left EDAS complicated by an acute left anterior cerebral artery (ACA)/middle cerebral artery (MCA) border zone infarct. Post-operatively, she exhibited expressive and receptive aphasia, dysarthria, right hemiparesis, and functional decline requiring extensive rehabilitation. METHODS: A comprehensive medical record review in conjunction with a clinical interview and administration and interpretation of a comprehensive neuropsychological battery was completed approximately 18-months post-surgical intervention. RESULTS: Neuropsychological evaluation revealed global impairments in memory, executive functioning, processing speed, and visuospatial abilities, with particularly severe language deficits, including a language shift from English to Tagalog, further compounding cognitive and functional decline. CONCLUSION: This case report adds to the small body of literature identifying neuropsychological patterns of impairment in MMD. Additionally, in conjunction with prior research, findings emphasize the importance of early detection and timely surgical intervention in order to optimize cognitive and functional outcomes.