Scheer Shelly, John Rita Marie
J Pediatr Health Care. 2016 Jul-Aug;30(4):347-58. doi: 10.1016/j.pedhc.2015.09.004. Epub 2015 Oct 21.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease that is becoming increasingly recognized in the pediatric population. It may be the most common cause of treatable autoimmune encephalitis. The majority of cases of anti-NMDAR encephalitis are idiopathic in etiology, but a significant minority can be attributed to a paraneoplastic origin. Children with anti-NMDAR encephalitis initially present with a prodrome of neuropsychiatric symptoms, often with orofacial dyskinesias followed by progressively worsening seizures, agitation, and spasticity, which may result in severe neurologic deficits and even death. Definitive diagnosis requires detection of NMDAR antibodies in the cerebrospinal fluid. Optimal outcomes are associated with prompt removal of the tumor in paraneoplastic cases, as well as aggressive immunosuppressive therapy. Early detection is essential for increasing the chances for a good outcome. Close follow-up is required to screen for relapse and later onset tumor presentation. The nurse practitioner plays a major role in the research, screening, diagnosis, treatment, follow-up, and rehabilitation of a child or adolescent with anti-NMDAR encephalitis.
抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一种自身免疫性疾病，在儿科人群中越来越受到关注。它可能是可治疗的自身免疫性脑炎的最常见病因。大多数抗 NMDAR 脑炎病例病因不明，但少数病例可归因于副肿瘤起源。患有抗 NMDAR 脑炎的儿童最初表现为神经精神症状前驱期，常伴有口面部运动障碍，随后癫痫发作、烦躁不安和痉挛逐渐加重，这可能导致严重的神经功能缺损甚至死亡。明确诊断需要在脑脊液中检测到 NMDAR 抗体。在副肿瘤性病例中，最佳治疗效果与及时切除肿瘤以及积极的免疫抑制治疗相关。早期检测对于提高良好预后的几率至关重要。需要密切随访以筛查复发和后期出现的肿瘤。执业护士在抗 NMDAR 脑炎儿童或青少年的研究、筛查、诊断、治疗、随访和康复中起着主要作用。
