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儿童炎性获得性脱髓鞘综合征的评估:单中心经验

Evaluation of inflammatory acquired demyelinating syndromes in children: a single-center experience.

作者信息

Kilic Huseyin, Mavi Deniz, Yalcinkaya Beyza Citci, Yildiz Edibe Pembegul, Kizilkilic Osman, Saltik Sema

机构信息

Department of Pediatric Neurology, Cerrahpasa School of Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Department of Neurology, Fulya Acibadem Hospital, Istanbul, Turkey.

出版信息

Acta Neurol Belg. 2022 Dec;122(6):1485-1491. doi: 10.1007/s13760-021-01703-4. Epub 2021 May 10.

DOI:10.1007/s13760-021-01703-4
PMID:33973168
Abstract

To evaluate the clinical and neuroimaging features of pediatric acquired demyelinating syndromes (ADS) in a tertiary pediatric neurology clinic in Turkey. All children diagnosed with any subset of ADS between 2013 and 2018 were included in this retrospective cohort study. Forty-two patients (21 female) with a median follow-up period of 30 months were included. The median age of the patients at disease onset was 11 years (range 1.5-17 years). The most common pediatric ADS categories according to the International pediatric Multiple Sclerosis Study Group consensus classification criteria were acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS), each of which seen in 15 patients, followed by clinically isolated syndrome (CIS) (n = 11) and Neuromyelitis Optica Spectrum Disorder (NMOSD) (n = 1). At the first clinical event, children with ADEM significantly differed from the children affected by MS and CIS in terms of the following parameters: median age at onset (7 vs. 13.5 and 14.5 years; p < 0.001), encephalopathy (93.3 vs 0% and 0%; p < 0.001), and basal ganglia/thalamus lesions (73.3 vs 9.1% and 9.1%; p < 0.001). The frequency of seizure and pleocytosis were higher in ADEM group than MS group (p < 0.05), whereas oligoclonal bands (p < 0.001) and periventricular white matter lesions (p < 0.01) were more frequently observed in MS patients. Rituximab was used with great success in the prevention of relapses in 3 patients: NMOSD (n = 1), MS (n = 1) and ADEM followed by recurrent optic neuritis (n = 1). Our results define the longitudinal disease course of various ADS categories in a single referral center. In addition, this study compares various clinical, laboratory and neuroimaging features between these ADS categories.

摘要

评估土耳其一家三级儿科神经科诊所中儿童获得性脱髓鞘综合征(ADS)的临床和神经影像学特征。2013年至2018年间诊断为任何ADS亚型的所有儿童均纳入这项回顾性队列研究。纳入了42例患者(21例女性),中位随访期为30个月。患者发病时的中位年龄为11岁(范围1.5 - 17岁)。根据国际儿童多发性硬化研究组共识分类标准,最常见的儿童ADS类别为急性播散性脑脊髓炎(ADEM)和多发性硬化(MS),各有15例患者,其次是临床孤立综合征(CIS)(n = 11)和视神经脊髓炎谱系障碍(NMOSD)(n = 1)。在首次临床事件时,ADEM患儿在以下参数方面与MS和CIS患儿有显著差异:发病时的中位年龄(7岁对13.5岁和14.5岁;p < 0.001)、脑病(93.3%对0%和0%;p < 0.001)以及基底节/丘脑病变(73.3%对9.1%和9.1%;p < 0.001)。ADEM组癫痫发作和脑脊液细胞增多的频率高于MS组(p < 0.05),而MS患者中寡克隆带(p < 0.001)和脑室周围白质病变(p < 0.01)更常见。利妥昔单抗在预防3例患者复发方面取得了巨大成功:NMOSD(n = 1)、MS(n = 1)以及ADEM后复发视神经炎(n = 1)。我们的结果确定了单一转诊中心各种ADS类别的纵向病程。此外,本研究比较了这些ADS类别之间的各种临床、实验室和神经影像学特征。

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