Neuropsychological profile associated with Moyamoya disease: A case report

BACKGROUND: Moyamoya disease (MMD) is a rare cardiovascular condition characterized by stenosis and gradual occlusion of the internal carotid arteries near the Circle of Willis. Current research on the disease has primarily been restricted to its medical implications, without adequate appreciation for its neurocognitive and/or neuropsychiatric implications. OBJECTIVES: The current study presents the neurocognitive profile of a 31-year-old woman diagnosed with MMD, further complicated by cerebral vascular accidents (CVAs) and history of bilateral craniotomy aimed at providing maximal revascularization. METHODS: Although speech and motor disturbances experienced by Ms. Doe around the time of her craniotomy and CVA were resolved at the time of current evaluation, she reported experiencing continued difficulties in processing speed, concentration, memory, word-retrieval, and planning. The patient underwent comprehensive neuropsychological evaluation assessing multiple cognitive domains. RESULTS: Neurocognitive evaluation revealed the presence of a lateralized profile as well as impairments in simple auditory attention, processing speed, working memory, verbal learning, verbal fluency, and speeded fine-motor dexterity. CONCLUSIONS: MMD significantly impacts cognition and daily functioning in affected individuals. This is often further exacerbated by additional CVAs requiring surgical intervention. While there is a clear growth of research on MMD, limited information is available on the neurocognitive and neuropsychiatric outcomes of the disease process. Neuropsychological data from the current case study is closely examined to provide a unique example of the lateralized neuropsychological profile and deficit pattern in a historically high functioning individual diagnosed with MMD following a stroke.