Background and Purpose Patients with moyamoya vasculopathy (MMV) may experience cognitive impairment, but its reported frequency, severity, and nature vary. In a systematic review and meta-analysis, we aimed to assess the presence, severity, and nature of cognitive impairments in children and adults with MMV. Methods We followed the MOOSE guidelines for meta-analysis and systematic reviews of observational studies. We searched Ovid Medline and Embase for studies published between January 1, 1969 and October 4, 2016. Independent reviewers extracted data for mean intelligence quotient (IQ) and standardized z-scores for cognitive tests, and determined percentages of children and adults with cognitive deficits, before and after conservative or surgical treatment. We explored associations between summary measures of study characteristics and cognitive impairments by linear regression analysis. Results We included 17 studies (11 studies reporting on 281 children, six on 153 adults). In children, the median percentage with impaired cognition was 30% (range, 13% to 67%); median IQ was 98 (range, 71 to 107). Median z-score was –0.39 for memory, and –0.43 for processing speed. In adults, the median percentage with impaired cognition was 31% (range, 0% to 69%); median IQ was 95 (range, 94 to 99). Median z-scores of cognitive domains were between –0.9 and –0.4, with multiple domains being affected. We could not identify determinants of cognitive impairment. Conclusions A large proportion of children and adults with MMV have cognitive impairment, with modest to large deficits across various cognitive domains. Further studies should investigate determinants of cognitive deficits and deterioration, and the influence of revascularization treatment on cognitive functioning.

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Keywords Adult, Child, Intelligence, Moyamoya disease, Neuropsychological tests, Review
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Journal Journal of Stroke
Kronenburg, A. (Annick), van den Berg, E, van Schooneveld, M, Braun, K.P.J, Calviere, L. (Lionel), van der Zwan, A. (Albert), & Klijn, C.J.M. (2018). Cognitive functions in children and adults with Moyamoya Vasculopathy: A systematic review and meta-analysis. Journal of Stroke (Vol. 20, pp. 332–351). doi:10.5853/jos.2018.01550