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

Abstract

Background and PurposePatients 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. MethodsWe 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. ResultsWe 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. ConclusionsA 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.

Cognitive Performance Profile in Pediatric Moyamoya Disease Patients and Its Relationship With Regional Cerebral Blood Perfusion

“…Longer duration of symptoms, cerebral infarction, and bilateral involvement had been suggested to associate with poorer cognitive performance (21,(24)(25)(26). However, these associations were not significant in a meta-analysis study (3). In our study, only radiologically-evident cerebral infarction was associated with lower cognitive index scores.…”

“…Consecutive pediatric MMD patients who were admitted to Beijing Tiantan Hospital from July 2017 to March 2019 and met the inclusion criteria were prospectively enrolled in the current study. The inclusion criteria were as follows: (1) Patients were aged between 6 and 16 years old; (2) Patients did not accept previous revascularization surgery; (3) Patients were cooperative to WISC-IV examination. The diagnosis of MMD was confirmed by digital subtraction angiography (DSA) or magnetic resonance angiography (MRA) based on a 2012 guideline on MMD (12), and only bilateral MMD were included.…”

“…Moyamoya disease (MMD) is a cerebrovascular disease characterized by idiopathic stenosis or occlusion of the end of internal carotid arteries (1,2). Impaired cognitive function is recognized in both adult and pediatric MMD patients (3), and has been hypothetically ascribed to chronic hypoperfusion in adult MMD patients (4). However, the presence of this phenomenon in pediatric patients has been poorly reported.…”

Quantitative Angiographic Hemodynamic Evaluation After Revascularization Surgery for Moyamoya Disease

“…Therefore, the ΔICA-MTT may be used as a quantitative index during serial observation. In previous studies, ischemic-onset, abundant ICA moyamoya vessels, Suzuki stages 3-4, and preoperative neurological status were reported to be associated with better neoangiogenesis and more favorable long-term outcome after revascularization surgery for MMD [6,[34][35][36]. However, these factors were not quantitative and mainly based on the preoperative features, which would be inappropriate for serial evaluation.…”

Different aspects of cognitive function in adult patients with moyamoya disease and its clinical subtypes

“…Karzmark et al studied cognitive function in 36 cases of MMD and found that the incidence of cognitive impairment was about 31% (11/36), but most degree of them were mild 6. Kronenburg et al found that the incidence of cognitive impairment in adult MMD was about 30%, and the median IQ was 95 (94–99) 29. In our study, the average IQ of adult MMD was 97 (97.27±14.36), while the average value of healthy controls was 106 (106.26±9.44).…”