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
Department of Psychology, University of Calgary.
Department of Psychology, Alberta Children's Hospital Research Institute, University of Calgary.
Neuropsychology. 2019 May;33(4):462-469. doi: 10.1037/neu0000513. Epub 2019 Feb 14.
To characterize the clinical cognitive phenotypes and severity of cognitive burden according to disease subtype in children with primary central nervous system vasculitis (cPACNS).
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.
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.
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. (PsycINFO Database Record (c) 2019 APA, all rights reserved).
根据原发性中枢神经系统血管炎（cPACNS）患儿的疾病亚型，描述其临床认知表型和认知负担的严重程度。
这项回顾性多中心炎症性脑病数据库研究，调查了1992年至2016年间连续诊断为原发性中枢神经系统血管炎的80名儿童（44名男性；平均年龄 = 7.89岁，标准差 = 4.17）的神经心理学结果。21名儿童患有小血管疾病（AN_cPACNS），59名患有大血管疾病（包括49名非进行性[APNP_cPACNS]和10名进行性[APP_cPACNS]）。神经影像学显示，大血管和小血管血管炎患儿中分别有100%和90%存在MRI异常。主要结局指标为全量表智商（FSIQ）以及韦氏儿童智力量表第三版（WISC-III）、第四版（WISC-IV）和第五版（WISC-V）的分量表得分。分析探讨了疾病亚型的影响。
症状出现后平均2.82年对智力功能进行评估。小血管中枢神经系统血管炎患儿的FSIQ得分显著低于大血管中枢神经系统血管炎患儿（均值分别为81.90和94.82；p = 0.04）。小血管疾病患儿中智力残疾（FSIQ < 70）更为常见（24%对5%）。相对于言语理解和知觉推理分量表得分，所有组的工作记忆和加工速度分量表得分均较低。在控制癫痫存在后，FSIQ的组间差异仍然显著。
与大血管疾病患儿相比，小血管中枢神经系统血管炎患儿更有可能出现智力功能缺陷，并且两种类型的中枢神经系统血管炎患儿均表现出相对较差的工作记忆和加工速度。（PsycINFO数据库记录（c）2019美国心理学会，保留所有权利）
