Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, 70403, Taiwan.
Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, 70403, Taiwan; Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan, 70101, Taiwan.
Pediatr Neonatol. 2021 Jul;62(4):361-368. doi: 10.1016/j.pedneo.2021.03.004. Epub 2021 Mar 18.
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is one of the most common autoimmune encephalitis in children. Most children recovered well after anti-NMDA receptor encephalitis. However, the NMDA receptor network functions are critical for the developing brain in children. The long-term consequences in pediatric patients of anti-NMDA receptor encephalitis are very infrequently reported.
This case series study retrospectively enrolled 10 children aged below 18 years old with antibody-proved anti-NMDA receptor encephalitis in a tertiary medical center from 2010 to 2019. Long-term neurological consequences of anti-NMDA receptor encephalitis in children were followed.
One boy and nine girls were enrolled with a median onset age of 3.6 years. The most common initial presentation was verbal reduction and psychiatric symptoms soon after some flu-like prodromal symptoms. Nearly all patients then developed decreased level of consciousness, mutism, seizures and orofacial-lingual dyskinesia. Autonomic instability occurred in 5 patients, particularly in pre-pubertal children. Only one adolescent patient had ovarian teratoma. All patients survived after immunotherapy and were followed for 5.8 ± 3.3 years after discharge. Four had epilepsy within 2 years after encephalitis, four had a cognitive deficit, one had mild psychiatric symptoms of hallucination, and none had residual involuntary movements. Moreover, two pre-pubertal children developed central precocious puberty about 3 years after encephalitis, and one required gonadotropin-releasing hormone agonist treatment.
Central precocious puberty could be a consequence of anti-NMDA receptor encephalitis in the pre-pubertal children. The pediatrician should pay attention to its occurrence at follow-up.
抗 N-甲基-D-天冬氨酸(NMDA)受体脑炎是儿童中最常见的自身免疫性脑炎之一。大多数儿童在抗 NMDA 受体脑炎后恢复良好。然而,NMDA 受体网络功能对儿童的大脑发育至关重要。抗 NMDA 受体脑炎在儿科患者中的长期后果很少有报道。
本病例系列研究回顾性纳入了 2010 年至 2019 年在一家三级医疗中心确诊为抗 NMDA 受体脑炎的 10 名年龄在 18 岁以下的儿童。随访儿童抗 NMDA 受体脑炎的长期神经后果。
共纳入 1 名男孩和 9 名女孩,中位发病年龄为 3.6 岁。最常见的首发症状是在流感样前驱症状后不久出现言语减少和精神症状。几乎所有患者随后均出现意识水平下降、缄默、癫痫发作和口面部运动障碍。5 例患者出现自主神经不稳定,尤其是青春期前儿童。仅 1 例青少年患者有卵巢畸胎瘤。所有患者经免疫治疗后存活,出院后随访 5.8±3.3 年。4 例在脑炎后 2 年内发生癫痫,4 例存在认知缺陷,1 例出现轻度精神症状幻觉,无残留不自主运动。此外,2 例青春期前儿童在脑炎后约 3 年出现中枢性性早熟,1 例需要促性腺激素释放激素激动剂治疗。
中枢性性早熟可能是青春期前儿童抗 NMDA 受体脑炎的后果。儿科医生在随访时应注意其发生。