Cognitive Outcomes of Childhood Primary CNS Vasculitis

• Published in: Neuropsychology 2019-05, Vol.33 (4), p.462-469
• Main Authors: Deschamps, Kristen, Yeates, Keith Owen, Fay-McClymont, Taryn B, Twilt, Marinka, Westmacott, Robyn, Dropol, Anastasia, Brooks, Brian L, Kirton, Adam, Gowdie, Peter, Sheikh, Shehla, Benseler, Susanne M
• Format: Article
• Language: English
• Subjects: Cardiovascular Disorders; Central Nervous System; Cognitive Impairment; Cognitive Processing Speed; Female; Human; Magnetic Resonance Imaging; Male; Phenotypes; Reasoning; Short Term Memory; Subtypes (Disorders); Verbal Comprehension
• ISSN: 0894-4105; 1931-1559
• DOI: 10.1037/neu0000513

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: 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. Results: 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: 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. General Scientific Summary Primary central nervous system (CNS) vasculitis is a newly understood childhood inflammatory brain disease with unknown cognitive consequences. This study found that children with primary CNS vasculitis displayed relative deficits in working memory and processing speed, whereas lower intellectual functioning was more common in the small-vessel disease subtype. These findings help delineate the prognosis for children with this disease and offer a basis for targeted rehabilitation programs.