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Clinical outcomes of pediatric Anti-NMDA receptor encephalitis
European Journal of Paediatric Neurology ( IF 2.3 ) Pub Date : 2020-11-01 , DOI: 10.1016/j.ejpn.2020.10.001
YoungKyu Shim , Soo Yeon Kim , Hunmin Kim , Hee Hwang , Jong-Hee Chae , Jieun Choi , Ki Joong Kim , Mi-Sun Yum , Tae Sung Ko , Young Ok Kim , Jung Hye Byeon , Jiwon Lee , Jeehun Lee , Jon Soo Kim , Byung Chan Lim

OBJECTIVE To investigate the clinical features and long-term outcomes of pediatric Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS Thirty-two anti-NMDAR encephalitis patients with positive anti-NMDAR antibody test results were recruited. Clinical outcomes were evaluated using the Clinical Assessment Scale in Autoimmune Encephalitis (CASE) and the modified Rankin Scale (mRS). RESULTS The median age of onset was 9.0 years (range, 0.7-17.2 years). Twenty-four patients (75.0%) were female. All patients received first-line immunotherapy including intravenous immunoglobulin and/or steroid therapy. The second-line immunotherapy was administered to 22 patients (68.8%). Clinical outcomes were evaluated in 27 patients who were followed for longer than 6 months after onset, among whom the median follow-up duration was 31.2 months (range, 6.3-82.9 months). The proportion of patients with ≤2 points on the mRS at their 12-month follow-up was 79.2% (19/24). The CASE scores of these 19 patients ranged from 0 to 5, with language and memory deficits accounting for most of these disabilities. When the outcome was assessed according to onset age (<12 years or 12-18 years), the younger group tended to show a slower recovery over their clinical course. CONCLUSIONS Despite overall favorable clinical outcomes, mild cognitive problems, including language and memory, may persist in pediatric anti-NMDAR encephalitis patients. A specific outcome measure, such as CASE, should be adopted to delineate clinical outcomes and aid the development of individualized treatment plans.

中文翻译:

小儿抗NMDA受体脑炎的临床结果

目的探讨小儿抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特征和远期预后。方法 招募抗NMDAR抗体检测阳性的抗NMDAR脑炎患者32例。使用自身免疫性脑炎临床评估量表 (CASE) 和改良的 Rankin 量表 (mRS) 评估临床结果。结果 发病的中位年龄为 9.0 岁(范围,0.7-17.2 岁)。24 名患者 (75.0%) 为女性。所有患者均接受一线免疫治疗,包括静脉注射免疫球蛋白和/或类固醇治疗。22 名患者(68.8%)接受了二线免疫治疗。对 27 名发病后随访时间超过 6 个月的患者进行了临床结局评估,其中中位随访时间为 31.2 个月(范围为 6. 3-82.9 个月)。12 个月随访时 mRS ≤ 2 分的患者比例为 79.2% (19/24)。这 19 名患者的 CASE 评分范围为 0 到 5 分,语言和记忆缺陷占这些残疾的大部分。当根据发病年龄(<12 岁或 12-18 岁)评估结果时,较年轻的组在其临床过程中往往表现出较慢的恢复。结论 尽管总体临床结果良好,但儿童抗 NMDAR 脑炎患者可能会持续存在轻度认知问题,包括语言和记忆。应采用特定的结果衡量标准,例如 CASE,来描述临床结果并帮助制定个性化的治疗计划。这 19 名患者的 CASE 评分范围为 0 到 5 分,语言和记忆缺陷占这些残疾的大部分。当根据发病年龄(<12 岁或 12-18 岁)评估结果时,较年轻的组在其临床过程中往往表现出较慢的恢复。结论 尽管总体临床结果良好,但儿童抗 NMDAR 脑炎患者可能会持续存在轻度认知问题,包括语言和记忆。应采用特定的结果衡量标准,例如 CASE,来描述临床结果并帮助制定个性化的治疗计划。这 19 名患者的 CASE 评分范围为 0 到 5 分,语言和记忆缺陷占这些残疾的大部分。当根据发病年龄(<12 岁或 12-18 岁)评估结果时,较年轻的组在其临床过程中往往表现出较慢的恢复。结论 尽管总体临床结果良好,但儿童抗 NMDAR 脑炎患者可能会持续存在轻度认知问题,包括语言和记忆。应采用特定的结果衡量标准,例如 CASE,来描述临床结果并帮助制定个性化的治疗计划。较年轻的组在其临床过程中往往表现出较慢的恢复。结论 尽管总体临床结果良好,但儿童抗 NMDAR 脑炎患者可能会持续存在轻度认知问题,包括语言和记忆。应采用特定的结果衡量标准,例如 CASE,来描述临床结果并帮助制定个性化的治疗计划。较年轻的组在其临床过程中往往表现出较慢的恢复。结论 尽管总体临床结果良好,但儿童抗 NMDAR 脑炎患者可能会持续存在轻度认知问题,包括语言和记忆。应采用特定的结果衡量标准,例如 CASE,来描述临床结果并帮助制定个性化的治疗计划。
更新日期:2020-11-01
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