J Stroke.  2018 Sep;20(3):332-341. 10.5853/jos.2018.01550.

Cognitive Functions in Children and Adults with Moyamoya Vasculopathy: A Systematic Review and Meta-Analysis

Affiliations
  • 1Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus, University Medical Center (UMC) Utrecht, Utrecht, The Netherlands. A.kronenburg@umcutrecht.nl
  • 2Department of Neurology, Erasmus MC University Medical Center, Rotterdam, the Netherlands.
  • 3Sector of Neuropsychology, Department of Pediatric Psychology, Wilhelmina Children’s Hospital, UMC Utrecht, Utrecht, The Netherlands.
  • 4Department of Vascular Neurology, University Hospital of Toulouse, Toulouse, France.
  • 5Department of Neurology, Donders Institute for Brain, Cognition and Behavior, Center for Neuroscience, Radboud University Medical Center, Nijmegen, the Netherlands.

Abstract

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 metaanalysis, 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.

Keyword

Moyamoya disease; Intelligence; Child; Adult; Neuropsychological tests; Review

MeSH Terms

Adult*
Child*
Cognition Disorders
Cognition*
Humans
Intelligence
Linear Models
Memory
Moyamoya Disease
Neuropsychological Tests
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