{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:40:22Z","timestamp":1787499622234,"version":"build-2736575974"},"reference-count":21,"publisher":"Georg Thieme Verlag KG","issue":"02","funder":[{"name":"Johns Hopkins School of Medicine Office of Medical Student Affairs"},{"name":"D.C. Women\u2019s Board"},{"name":"Laney Jaymes Foundation for Pediatric Stroke"},{"DOI":"10.13039\/100000968","name":"American Heart Association","doi-asserted-by":"crossref","award":["Career Development Award (850044)"],"award-info":[{"award-number":["Career Development Award (850044)"]}],"id":[{"id":"10.13039\/100000968","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropediatrics"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Introduction\u2003Moyamoya 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.<\/jats:p>\n                  <jats:p>Methods\u2003This 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.<\/jats:p>\n                  <jats:p>Results\u2003Of 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\u2009=\u20090.003), global executive functioning (p\u2009=\u20090.005), and overall adaptive functioning (p\u2009=\u20090.015). There was no significant difference in overall intelligence between children with (n\u2009=\u20096) versus without (n\u2009=\u20097) a history of clinical stroke (p\u2009=\u20090.368), though children with any radiographic infarct scored lower in this domain (p\u2009=\u20090.032).<\/jats:p>\n                  <jats:p>Conclusion\u2003In 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.<\/jats:p>","DOI":"10.1055\/a-1993-3860","type":"journal-article","created":{"date-parts":[[2022,12,6]],"date-time":"2022-12-06T19:00:23Z","timestamp":1670353223000},"page":"134-138","source":"Crossref","is-referenced-by-count":10,"title":["Characterization of Neuropsychological Outcomes in a Cohort of Pediatric Patients with Moyamoya Arteriopathy"],"prefix":"10.1055","volume":"54","author":[{"given":"John R.","family":"Gatti","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine, Baltimore, Maryland, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel K.","family":"Peterson","sequence":"additional","affiliation":[{"name":"Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, Maryland, United States"},{"name":"Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia","family":"Cannon","sequence":"additional","affiliation":[{"name":"Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, Maryland, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivek","family":"Yedavalli","sequence":"additional","affiliation":[{"name":"Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura A.","family":"Malone","sequence":"additional","affiliation":[{"name":"Center for Movement Studies, Kennedy Krieger Institute, Baltimore, Maryland, United States"},{"name":"Department of Physical Medicine and Rehabilitation, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States"},{"name":"Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa R.","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2023,1,19]]},"reference":[{"issue":"01","key":"ref1","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1161\/01.STR.14.1.104","article-title":"Moyamoya disease\u2014a review","volume":"14","author":"J Suzuki","year":"1983","journal-title":"Stroke"},{"issue":"03","key":"ref2","doi-asserted-by":"crossref","first-page":"e51","DOI":"10.1161\/STR.0000000000000183","article-title":"Management of stroke in neonates and children: a scientific statement from the American Heart Association\/American Stroke Association","volume":"50","author":"D M Ferriero","year":"2019","journal-title":"Stroke"},{"issue":"01","key":"ref3","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1161\/STROKEAHA.115.011173","article-title":"Risk of recurrent arterial ischemic stroke in childhood: a prospective international study","volume":"47","author":"H J Fullerton","year":"2016","journal-title":"Stroke"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.pediatrneurol.2021.03.002","article-title":"Distinct clinical and radiographic phenotypes in pediatric patients with moyamoya","volume":"120","author":"M L Kaseka","year":"2021","journal-title":"Pediatr Neurol"},{"issue":"04","key":"ref5","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1177\/0883073820967160","article-title":"Clinical presentation and stroke incidence differ by moyamoya etiology","volume":"36","author":"J R Gatti","year":"2021","journal-title":"J Child Neurol"},{"issue":"10","key":"ref6","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1007\/s007010050213","article-title":"Intellectual functions and regional cerebral haemodynamics after extensive omental transplantation spread over both frontal lobes in childhood moyamoya disease","volume":"140","author":"M Ohtaki","year":"1998","journal-title":"Acta Neurochir (Wien)"},{"issue":"03","key":"ref7","doi-asserted-by":"crossref","first-page":"332","DOI":"10.5853\/jos.2018.01550","article-title":"Cognitive functions in children and adults with moyamoya vasculopathy: a systematic review and meta-analysis","volume":"20","author":"A Kronenburg","year":"2018","journal-title":"J Stroke"},{"issue":"03","key":"ref8","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1007\/BF00308495","article-title":"Chronic low-perfusion state in children with moyamoya disease following revascularization","volume":"6","author":"H Sato","year":"1990","journal-title":"Childs Nerv Syst"},{"issue":"05","key":"ref9","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1007\/s12975-022-01003-w","article-title":"Fronto-parietal and white matter haemodynamics predict cognitive outcome in children with moyamoya independent of stroke","volume":"13","author":"E J Choi","year":"2022","journal-title":"Transl Stroke Res"},{"issue":"06","key":"ref10","doi-asserted-by":"crossref","first-page":"e219","DOI":"10.1161\/STROKEAHA.122.038771","article-title":"Nonischemic presentations of pediatric moyamoya arteriopathy: a natural history study","volume":"53","author":"J R Gatti","year":"2022","journal-title":"Stroke"},{"key":"ref11","volume-title":"Wechsler Intelligence Scale for Children","author":"D Wechsler","year":"2003"},{"key":"ref12","volume-title":"WISC-V: Wechsler Intelligence Scale for Children","author":"D Wechsler","year":"2014"},{"key":"ref13","volume-title":"Differential Ability Scales","author":"C D Elliott","year":"2007"},{"key":"ref14","volume-title":"Behavior Rating Inventory of Executive Function\u00ae, Second Edition (BRIEF\u00ae2)","author":"G A Gioia","year":"2015"},{"key":"ref15","volume-title":"Behavior Rating Inventory of Executive Function \u00ae - 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