The profile of cognitive impairment and hemodynamic compromise in moyamoya: a single-center prospective cohort study

Annick KronenburgDepartment of Neurology and Neurosurgery, UMC Utrecht Brain Center, Utrecht;

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Pieter T. DeckersDepartment of Neurology and Neurosurgery, UMC Utrecht Brain Center, Utrecht;

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Esther van den BergDepartment of Neurology, Erasmus UMC, Rotterdam;

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Monique M. van SchooneveldDepartment of Pediatric Psychology, Wilhelmina Children’s Hospital, UMC Utrecht;

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Evert-Jan VonkenDepartment of Radiology; UMC Utrecht Brain Center, Utrecht;

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Albert van der ZwanDepartment of Neurology and Neurosurgery, UMC Utrecht Brain Center, Utrecht;

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Bart N. M. van BerckelDepartment of Nuclear Medicine & PET Research, Amsterdam UMC, Amsterdam;

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Maqsood YaqubDepartment of Nuclear Medicine & PET Research, Amsterdam UMC, Amsterdam;

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Willem OtteDepartment of Pediatric Neurology, UMC Utrecht Brain Center, Utrecht; and

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Catharina J. M. KlijnDepartment of Neurology and Neurosurgery, UMC Utrecht Brain Center, Utrecht;
Department of Neurology, Donders Institute for Brain, Cognition and Behavior, Center for Neuroscience, Radboud UMC, Nijmegen, The Netherlands

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Kees P. J. BraunDepartment of Neurology and Neurosurgery, UMC Utrecht Brain Center, Utrecht;

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OBJECTIVE

Patients with moyamoya vasculopathy often experience cognitive impairments. In this prospective single-center study, the authors investigated the profile of neurocognitive impairment and its relation with the severity of ischemic brain lesions and hemodynamic compromise.

METHODS

Patients treated in a Dutch tertiary referral center were prospectively included. All patients underwent standardized neuropsychological evaluation, MRI, digital subtraction angiography, and [O]H2O-PET (to measure cerebrovascular reactivity [CVR]). The authors determined z-scores for 7 cognitive domains and the proportion of patients with cognitive impairment (z-score < −1.5 SD in at least one domain). The authors explored associations between patient characteristics, imaging and CVR findings, and cognitive scores per domain by using multivariable linear regression and Bayesian regression analysis.

RESULTS

A total of 40 patients (22 children; 75% females) were included. The median age for children was 9 years (range 1–16 years); for adults it was 39 years (range 19–53 years). Thirty patients (75%) had an infarction, and 31 patients (78%) had impaired CVR (steal phenomenon). Six of 7 cognitive domains scored below the population norm. Twenty-nine patients (73%) had cognitive impairment. Adults performed better than children in the cognitive domain visuospatial functioning (p = 0.033, Bayes factor = 4.0), and children performed better in processing speed (p = 0.041, Bayes factor = 3.5). The authors did not find an association between infarction, white matter disease, or CVR and cognitive domains.

CONCLUSIONS

In this Western cohort, cognitive functioning in patients with moyamoya vasculopathy was below the population norm, and 73% had cognitive impairment in at least one domain. The cognitive profile differed between adults and children. The authors could not find an association with imaging findings.

ABBREVIATIONS

AIS = acute ischemic stroke; BF = Bayes factor; CVR = cerebrovascular reactivity; DSA = digital subtraction angiography; MMD = moyamoya disease; MMS = moyamoya syndrome; MMV = moyamoya vasculopathy; MOPD II = microcephalic osteodysplastic primordial dwarfism type II; mRS = modified Rankin Scale; mSS = modified Suzuki Scale; NIHSS = National Institutes of Health Stroke Scale; pedNIHSS = Pediatric NIHSS; PSOM = Pediatric Stroke Outcome Measure; ROI = region of interest; TIA = transient ischemic attack; WMD = white matter disease.

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Figure from Ramos et al. (pp 95–103).

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