Selective neuropsychological impairments and related clinical factors in children with moyamoya disease of the transient ischemic attack type

Purpose: Moyamoya disease is characterized by progressive narrowing of bilateral internal carotid arteries. Neuropsychological impairments are suspected due to frequent involvement of the frontotemporal areas. The present study thus aimed to investigate the pattern of neuropsychological function in children diagnosed with moyamoya disease. Methods: Thirteen children with moyamoya disease of the transient ischemic attack type received standardized neuropsychological tests that evaluate general intellectual function, verbal comprehension, perceptual organization, working memory, processing speed, episodic memory, category fluency and visuospatial function. Related clinical factors were also analyzed. Results: The results showed single-domain cognitive impairment in around 15 % of patients and multiple-domain cognitive impairments in 23 % of patients. Selective impairments of episodic memory and processing speed were especially noted in those with younger age of onset and prolonged symptom duration. Conclusions: Neuropsychological impairments are not infrequent in children with moyamoya disease despite normal general intellectual functioning. The pattern of cognitive dysfunction is often associated with lesions in frontotemporal areas. Early detection and intervention shall be considered regarding cognitive outcome in pediatric group.