在患有抗NMDA受体脑炎的儿童中,非常长期的功能结果和依赖性
Li-Wen Chen1, Gemma Olivé-Cirera1, Elianet G Fonseca1
1From the Group of Experimental Neuroimmunology (L.-W.C., G.O.-C., E.G.F., T.A., J.D.), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) and Caixa Research Institute, Barcelona, Spain; Department of Pediatrics (L.-W.C.), National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Pediatric Neurology Unit (G.O.-C.), Hospital Parc Taulí de Sabadell; Neurology Service (E.G.F., J.D.), Hospital Clínic Barcelona; Pediatric Neuroimmunology Unit (E.G.F., T.A.), Neurology Department, Sant Joan de Déu Children's Hospital, Barcelona, Spain; Division of Neurology (M.M.S.), Hospital das Clinicas (HCFMUSP), Faculdade de Medicina, University of São Paulo, Brazil; Department of Neurology (T.I.), Kitasato University School of Medicine, Sagamihara, Japan; Centro de Investigación Biomédica en Red (J.D.), Enfermedades Raras (CIBERER), Spain; Department of Neurology (J.D.), Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA; and University of Barcelona (J.D.), Barcelona, Spain.
大多数患有抗NMDA受体脑炎 (NMDARe) 的儿童康复良好,但有些人面临持久的行为和认知问题. 儿童早期的疾病发病增加了严重的长期缺陷和依赖的风险.
科学领域:
- 儿科神经学 儿科神经学
- 神经免疫学 神经免疫学
- 自免疫性脑炎是什么?
背景情况:
- 抗NMDA受体脑炎 (NMDARe) 是一种严重的自身免疫疾病,影响中枢神经系统.
- 儿科NMDARe幸存者的长期功能结果尚未完全理解.
研究的目的:
- 评估诊断为NMDARe的儿童的长期日常功能.
- 确定影响儿科NMDARe患者功能恢复的因素.
主要方法:
- 对患有NMDARe的儿科患者 (发病年龄≤18岁) 的回顾性研究.
- 医生通过使用15个领域的利物浦结果得分来评估日常功能.
- 最低的随访时间为5年.
主要成果:
- 在76名患者中,68人被跟踪平均7.1年;11%的死亡率.
- 在73%的幸存者中观察到完全康复.
- 行为/学校缺陷18%,多领域缺陷9%.
结论:
- 大多数儿科NMDARe患者在5年内实现了实质性的功能恢复.
- 显著的少数人经历了持续的缺陷,特别是行为和认知方面的缺陷.
- 发病时的年龄较小是较差的预测结果和社会家庭依赖度增加的预测因素.
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