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三级医疗中心儿科抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床表现、治疗结果及预后因素:一项多中心回顾性/前瞻性队列研究

Clinical manifestations, treatment outcomes, and prognostic factors of pediatric anti-NMDAR encephalitis in tertiary care hospitals: A multicenter retrospective/prospective cohort study.

作者信息

Pruetarat Napa, Netbaramee Wiracha, Pattharathitikul Siriporn, Veeravigrom Montida

机构信息

Department of Pediatrics, King Chulalongkorn Memorial Hospital, The Thai Red Cross Society, Thailand.

Department of Pediatrics, Chonburi Hospital, Chonburi, Thailand.

出版信息

Brain Dev. 2019 May;41(5):436-442. doi: 10.1016/j.braindev.2018.12.009. Epub 2019 Jan 11.

DOI:10.1016/j.braindev.2018.12.009
PMID:30639077
Abstract

OBJECTIVE

Anti-NMDAR encephalitis is an acute autoimmune neurological disorder that is increasingly recognized in pediatric populations. Several studies of the disorder have been conducted worldwide but there are few publications in Thailand. Here, we describe the clinical manifestations, treatment outcomes, and prognostic factors in children with anti-NMDAR encephalitis.

METHODS

Between January 2007 and September 2017, we conducted a retrospective/prospective cohort study of children diagnosed with anti-NMDAR encephalitis from three tertiary care hospitals in Thailand: King Chulalongkorn Memorial Hospital, Chonburi Hospital, and Prapokklao Hospital. We assessed the Modified Rankin Score (mRS) score for each participant to measure severity of disease and treatment outcome at baseline, 12, and 24 months.

RESULTS

We recruited 14 participants (1-13 years with median age 8.4 years). Participants were followed up for a median of 20.5 months. Clinical manifestations included behavioral dysfunction (100%), movement disorder (93%), speech disorder (79%), sleep disorder (79%), and seizures (79%). All patients received first-line immunotherapy (corticosteroids: 100%, intravenous immunoglobulin: 79%, plasma exchange: 21%). Second-line immunotherapy (cyclophosphamide) was administered to 57% of patients. During the first 12 months, 8 patients (62%) achieved a good outcome (mRS ≤ 2). At 24 months, 9 patients (81%) had achieved a good outcome. Altered consciousness and central hypoventilation were predictors of poor outcome. (p < 0.05).

CONCLUSIONS

We observed similar clinical manifestation of anti-NMDAR encephalitis in Thai children to those reported in other countries. Furthermore, the percentage of patients with good outcomes in our study was comparable with previous studies. Further studies are required to investigate other populations in other regions of Thailand.

摘要

目的

抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎是一种急性自身免疫性神经疾病,在儿科人群中越来越受到认可。全球已对该疾病进行了多项研究,但泰国的相关出版物较少。在此,我们描述抗NMDAR脑炎患儿的临床表现、治疗结果及预后因素。

方法

2007年1月至2017年9月,我们对泰国三家三级护理医院(朱拉隆功国王纪念医院、春武里医院和普拉波克拉奥医院)诊断为抗NMDAR脑炎的儿童进行了一项回顾性/前瞻性队列研究。我们评估了每位参与者的改良Rankin评分(mRS),以测量基线、12个月和24个月时的疾病严重程度和治疗结果。

结果

我们招募了14名参与者(年龄1至13岁,中位年龄8.4岁)。参与者的中位随访时间为20.5个月。临床表现包括行为功能障碍(100%)、运动障碍(93%)、言语障碍(79%)、睡眠障碍(79%)和癫痫发作(79%)。所有患者均接受一线免疫治疗(皮质类固醇:100%,静脉注射免疫球蛋白:79%,血浆置换:21%)。57%的患者接受了二线免疫治疗(环磷酰胺)。在最初的12个月内,8名患者(62%)取得了良好的结果(mRS≤2)。在24个月时,9名患者(81%)取得了良好的结果。意识改变和中枢性通气不足是预后不良的预测因素(p<0.05)。

结论

我们观察到泰国儿童抗NMDAR脑炎的临床表现与其他国家报道的相似。此外,我们研究中预后良好的患者百分比与先前的研究相当。需要进一步研究以调查泰国其他地区的其他人群。

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