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Title: | Cognitive outcomes of childhood primary cns vasculitis. | Authors: | Kirton A.;Benseler S.M.;Brooks B.L.;Gowdie P. ;Sheikh S.;Deschamps K.;Yeates K.O.;Fay-McClymont T.B.;Twilt M.;Westmacott R.;Dropol A. | Institution: | (Deschamps) Department of Psychology, University of Calgar., Canada (Yeates) Department of Psychology, Alberta Children's Hospital Research Institute, Canada (Yeates, Kirton) Hotchkiss Brain Institute, Cumming School of Medicine, University of Calgary, Canada (Fay-McClymont) Neurosciences Program, Alberta Children's Hospital, Calgary, AB, Canada (Fay-McClymont, Dropol) Department of Pediatrics, Cumming School of Medicine, University of Calgary, Alberta Children's Hospital Research Institute, Canada (Twilt, Dropol) Section of Rheumatology, Department of Pediatrics, Alberta Children's Hospital, Canada (Twilt, Brooks) Alberta Children's Hospital Research Institute, Department of Pediatrics, Department of Clinical Neurosciences, Cumming School of Medicine, Department of Psychology, University of Calgary, Canada (Westmacott) Psychology Department, Hospital for Sick Children, Toronto, ON, Canada (Westmacott) Department of Pediatrics, Faculty of Medicine, University of Toronto, Canada (Brooks) Neurosciences Program, Alberta Children's Hospital, Canada (Kirton) Departments of Pediatrics and Clinical Neurosciences, Alberta Children's Hospital Research Institute, Canada (Gowdie) Rheumatology Service, Department of General Medicine, Royal Children's Hospital, Melbourne, VIC, Australia (Gowdie) Department of Pediatric Rheumatology, Monash Children's Hospital, Melbourne, VIC, Australia (Gowdie) Department of Pediatrics, Monash University, Australia (Sheikh) Information Services, Hospital for Sick Children, Canada (Benseler) Alberta Children's Hospital Research Institute, Department of Pediatrics, Cumming School of Medicine, University of Calgary, Canada (Dropol, Benseler) University of British Columbia, BC Children's Hospital Research Institute, Vancouver, BC, Canada | Issue Date: | 21-May-2019 | Copyright year: | 2019 | Publisher: | American Psychological Association Inc. (E-mail: journals@apa.org) | Place of publication: | United States | Publication information: | Neuropsychology. 33 (4) (pp 462-469), 2019. Date of Publication: May 2019. | Journal: | Neuropsychology | Abstract: | Objective: To characterize the clinical cognitive phenotypes and severity of cognitive burden according to disease subtype in children with primary central nervous system vasculitis (cPACNS). Method(s): This retrospective multicenter inflammatory brain disease database study examined the neuropsychological outcomes of 80 children (44 male; mean age - 7.89 years, SD = 4.17) consecutively diagnosed with primary CNS vasculitis between 1992 and 2016. Twenty-one children had small-vessel disease (AN=cPACNS), and 59 had large-vessel disease (including 49 nonprogressive [APNP-cPACNS] and 10 progressive [APP-cPACNS]). Neuroimaging revealed MRI abnormalities in 100% and 90% of children with large-and small-vessel vasculitis, respectively. The primary outcomes were Full Scale IQ (FSIQ) and the index scores from the Wechsler Intelligence Scale for Children-III (WISC-III, WISC-IV, and WISC-V). Analyses explored the effect of disease subtype. Result(s): Intellectual functioning was assessed on average 2.82 years after symptom onset. Children with small-vessel CNS vasculitis had significantly lower FSIQ scores than did those with large-vessel CNS vasculitis (Ms - 81.90 vs. 94.82; p - .04). Intellectual disability (FSIQ - 70) was more frequent in children with small-vessel disease (24% vs. 5%). All groups displayed lower Working Memory and Processing Speed index scores relative to Verbal Comprehension and Perceptual Reasoning index scores. Group differences in FSIQ remained significant after controlling for the presence of seizures. Conclusion(s): Children with small-vessel CNS vasculitis are more likely to demonstrate deficits in intellectual functioning than are those with large-vessel disease, and children with both types of CNS vasculitis demonstrate relatively poor working memory and processing speed.Copyright © 2019 American Psychological Association. | DOI: | http://monash.idm.oclc.org/login?url=http://dx.doi.org/10.1037/neu0000513 | PubMed URL: | 30762375 [http://www.ncbi.nlm.nih.gov/pubmed/?term=30762375] | ISSN: | 0894-4105 | URI: | https://repository.monashhealth.org/monashhealthjspui/handle/1/36142 | Type: | Article | Subjects: | *childhood disease/di [Diagnosis] article blood vessel diameter *central nervous system disease/di [Diagnosis] *central nervous system disease/dt [Drug Therapy] child *childhood disease/dt [Drug Therapy] *cognitive defect controlled study data base disease burden *disease severity female functional assessment human intellectual impairment/di [Diagnosis] major clinical study male memory disorder/di [Diagnosis] multicenter study (topic) neuroimaging neuropsychological test nuclear magnetic resonance imaging onset age outcome assessment *phenotype preschool child priority journal retrospective study school child scoring system seizure symptom *vasculitis/di [Diagnosis] *vasculitis/dt [Drug Therapy] Wechsler intelligence scale for children working memory antibiotic agent anticonvulsive agent azathioprine/dt [Drug Therapy] corticosteroid/dt [Drug Therapy] cyclophosphamide/dt [Drug Therapy] cyclophosphamide/iv [Intravenous Drug Administration] mycophenolate mofetil/dt [Drug Therapy] mycophenolic acid/dt [Drug Therapy] prednisone/dt [Drug Therapy] prednisone/po [Oral Drug Administration] psychotropic agent *childhood primary central nervous system vasculitis/di [Diagnosis] *childhood primary central nervous system vasculitis/dt [Drug Therapy] Full Scale IQ score Verbal Comprehension and Perceptual Reasoning index score Working Memory and Processing Speed index score female functional assessment intellectual impairment / diagnosis major clinical study male memory disorder / diagnosis multicenter study (topic) neuroimaging neuropsychological test nuclear magnetic resonance imaging onset age outcome assessment *phenotype preschool child priority journal retrospective study school child scoring system seizure symptom *vasculitis / *diagnosis / *drug therapy Wechsler intelligence scale for children working memory *disease severity human Article blood vessel diameter *central nervous system disease / *diagnosis / *drug therapy child *childhood disease / *diagnosis / *drug therapy *cognitive defect controlled study data base disease burden |
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