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Neuropediatrics 2023; 54(02): 134-138
DOI: 10.1055/a-1993-3860
Original Article

Characterization of Neuropsychological Outcomes in a Cohort of Pediatric Patients with Moyamoya Arteriopathy

John R. Gatti
1   Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Rachel K. Peterson
2   Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, Maryland, United States
3   Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Alicia Cannon
2   Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, Maryland, United States
,
Vivek Yedavalli
4   Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Laura A. Malone
5   Center for Movement Studies, Kennedy Krieger Institute, Baltimore, Maryland, United States
6   Department of Physical Medicine and Rehabilitation, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
7   Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Lisa R. Sun
7   Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
› Author Affiliations Funding This work is supported by the Laney Jaymes Foundation for Pediatric Stroke, the American Heart Association (Career Development Award [850044]), the D.C. Women's Board, and the Johns Hopkins School of Medicine Office of Medical Student Affairs.
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Abstract

Introduction Moyamoya arteriopathy is a severe, progressive cerebral arteriopathy that places affected children at high risk for stroke. Moyamoya has been associated with a range of neuropsychological deficits in adults, but data on many cognitive domains remain limited in the pediatric population and little is known about the neuropsychological profile of children with syndromic moyamoya.

Methods This is a single-center, retrospective cohort study of children with moyamoya arteriopathy followed at our center who underwent neuropsychological testing between 2003 and 2021. Test scores were extracted from neuropsychological reports. Medical records were reviewed with attention to individual neuropsychological test results, medical comorbidities, presence of infarct(s) on neuroimaging, and history of clinical ischemic stroke.

Results Of the 83 children with moyamoya followed at our center between 2003 and 2021, 13 had completed neuropsychological testing across multiple cognitive domains. Compared to age-based normative data, children in this sample had lower scores in overall intelligence (p = 0.003), global executive functioning (p = 0.005), and overall adaptive functioning (p = 0.015). There was no significant difference in overall intelligence between children with (n = 6) versus without (n = 7) a history of clinical stroke (p = 0.368), though children with any radiographic infarct scored lower in this domain (p = 0.032).

Conclusion In our cohort, children with moyamoya demonstrated impaired intelligence and executive functioning, even in the absence of clinical stroke. Neuropsychological evaluation should be considered standard of care for all children with moyamoya, even those without a history of clinical stroke.

Keywords

moyamoya disease - stroke - pediatric - neuropsychology - cognitive dysfunction


Publication History

Received: 03 October 2022

Accepted: 01 December 2022

Accepted Manuscript online:
06 December 2022

Article published online:
19 January 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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