LAUSR

BACKGROUND Moyamoya disease (MMD) is a rare, chronic cardiovascular condition characterized by stenosis and gradual occlusion of internal carotid arteries in the circle of Willis. Research on this disease is largely restricted to its medical implications, without adequate appreciation for its neurocognitive and neuropsychiatric implications. OBJECTIVES The current study presents the neurocognitive profile of a 31-year-old woman diagnosed with MMD, which was further complicated by cerebral vascular accidents (CVAs) and history of bilateral craniotomy aimed at providing maximal revascularization. METHODS Although there was no prior history of speech, motor, and cognitive difficulties, at the time of evaluation, Ms. Doe presented with difficulties in concentration, memory, word-retrieval, planning, and slowed speed of processing of new information. 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 patients. This is further exacerbated by additional vascular incidents 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.