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髓鞘少突胶质细胞糖蛋白抗体相关急性脱髓鞘综合征患儿发生学习困难的危险因素。

Risk factors for academic difficulties in children with myelin oligodendrocyte glycoprotein antibody-associated acute demyelinating syndromes.

机构信息

Pediatric Neurology Department, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Bicêtre Hospital, University Paris-Saclay, Le Kremlin-Bicêtre, France.

National Referral Center for Rare Inflammatory Brain and Spinal Diseases, Le Kremlin-Bicêtre, France.

出版信息

Dev Med Child Neurol. 2020 Sep;62(9):1075-1081. doi: 10.1111/dmcn.14594. Epub 2020 Jun 22.

DOI:10.1111/dmcn.14594
PMID:32567093
Abstract

AIM

To describe cognitive abilities through the evaluation of academic difficulties in children with acute demyelinating syndromes (ADS) and myelin oligodendrocyte glycoprotein (MOG) antibodies.

METHOD

This was an observational, retrospective study of a French paediatric cohort that included children aged 18 years and younger. Clinical, biological, and imaging data were collected and academic outcome was measured.

RESULTS

Seventy-six children were included in the study with a mean (SD) follow-up of 4 years 7 months (6y 4mo). Median age at disease onset was 9 years 1 months (interquartile range=4y 7mo-13y 11mo; 36 females, 40 males). Thirty-six children relapsed and 20 had academic difficulties at the last follow-up. Academic difficulties, as well as deep grey matter and putaminal lesions (p=0.047 and p=0.006 respectively), were significantly more prevalent in children aged 10 years and younger (p=0.02). Using univariate binary regression analysis, we found that age at disease onset of 10 years and younger (odds ratio [OR] 3.72 [95% confidence interval {CI} 1.19-11.64]; p=0.024), acute disseminated encephalomyelitis at disease onset (OR 52.5 [95% CI 5.97-461.4]; p<0.001), and deep grey matter lesions (OR 17.33 [95% CI 3.87-77.72]; p<0.001) were associated with academic difficulties.

INTERPRETATION

MOG antibody-associated ADS have distinct clinical and radiological patterns that are age-dependent. Indirect cognitive evaluation through academic difficulties was prevalent in younger children and is associated with specific clinical and magnetic resonance imaging factors that need to be considered earlier on when assessing this patient population.

摘要

目的

通过评估急性脱髓鞘综合征(ADS)和髓鞘少突胶质细胞糖蛋白(MOG)抗体患儿的学习困难来描述认知能力。

方法

这是一项法国儿科队列的观察性回顾性研究,纳入了 18 岁及以下的儿童。收集了临床、生物学和影像学数据,并测量了学习成绩。

结果

共纳入 76 名患儿,平均(标准差)随访时间为 4 年 7 个月(6y 4mo)。疾病发作的中位年龄为 9 岁 1 个月(四分位距=4y 7mo-13y 11mo;36 名女性,40 名男性)。36 名患儿复发,20 名患儿在最后一次随访时有学习困难。学习困难以及大脑深部灰质和壳核病变(p=0.047 和 p=0.006)在 10 岁及以下的患儿中更为常见(p=0.02)。使用单变量二项回归分析,我们发现 10 岁及以下发病年龄(比值比[OR] 3.72[95%置信区间{CI} 1.19-11.64];p=0.024)、发病时急性播散性脑脊髓炎(OR 52.5[95% CI 5.97-461.4];p<0.001)和大脑深部灰质病变(OR 17.33[95% CI 3.87-77.72];p<0.001)与学习困难相关。

结论

MOG 抗体相关的 ADS 具有与年龄相关的独特临床和影像学模式。通过学习困难进行间接认知评估在年龄较小的儿童中较为常见,并且与特定的临床和磁共振成像因素相关,在评估该患者群体时需要更早考虑这些因素。

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