Moussa Taha, Afzal Khalid, Cooper Joseph, Rosenberger Ryan, Gerstle Karyn, Wagner-Weiner Linda
Section of Pediatric Rheumatology, Department of Pediatrics, University of Chicago Medical Center, 5841 S. Maryland Avenue, MC 5044, Chicago, 60637, IL, USA.
Section of Pediatric Psychiatry, Department of Psychiatry, University of Chicago Medical Center, Chicago, USA.
Pediatr Rheumatol Online J. 2019 Feb 18;17(1):8. doi: 10.1186/s12969-019-0310-0.
Anti-NMDA receptor encephalitis, an autoimmune disease associated with antibodies against N-methyl-D-aspartate (NMDA) receptors, is being diagnosed more frequently, especially in children and young adults. Acute neurological and psychiatric manifestations are the common presenting symptoms. Diagnosing anti-NMDA receptor encephalitis is often challenging given the wide range of clinical presentation, and may be further complicated by its overlap of symptoms, brain MRI changes, and CSF findings with other entities affecting the brain. Even though diagnosis can be made by identifying antibodies in immune-mediated encephalitis, the diagnosis may be delayed by weeks to months. Delay in initiation of treatment with immune suppressive therapies is shown to be associated with adverse outcomes. Malignant catatonia is a severe and life-threatening state associated with anti-NMDA receptor encephalitis. It is often inadequately assessed and may not respond to immunosuppressive treatment.
We present a confirmed case of anti-NMDA receptor encephalitis in a 16 year old girl who had severe critical neurological and psychiatric manifestations, including malignant catatonia and autonomic instability. Our patient continued to manifest malignant catatonia despite the initiation of prompt, aggressive immune suppressive therapies, including corticosteroids, plasmapheresis, intravenous gammaglobulin and rituximab, as well as treatment with high-dose benzodiazepines. Once electroconvulsive therapy (ECT) began, she had a robust response with resolution of her catatonia. Six weeks after treatment with eight ECT cycles, she had returned to her normal baseline cognitive and motor function.
ECT was an effective and well-tolerated therapy in our patient, and should be considered for the treatment of children with anti-NMDA receptor encephalitis whose catatonia does not respond to immunosuppression and benzodiazepines.
抗N-甲基-D-天冬氨酸（NMDA）受体脑炎是一种与抗NMDA受体抗体相关的自身免疫性疾病，目前诊断越来越频繁，尤其是在儿童和青年成人中。急性神经和精神症状是常见的临床表现。鉴于临床表现范围广泛，诊断抗NMDA受体脑炎往往具有挑战性，其症状、脑部MRI变化和脑脊液检查结果与其他影响脑部的疾病重叠可能会使情况更加复杂。尽管通过在免疫介导的脑炎中识别抗体可以做出诊断，但诊断可能会延迟数周甚至数月。免疫抑制治疗开始延迟与不良预后相关。恶性紧张症是一种与抗NMDA受体脑炎相关的严重且危及生命的状态。它常常未得到充分评估，且可能对免疫抑制治疗无反应。
我们报告一例确诊的抗NMDA受体脑炎病例，患者为一名16岁女孩，有严重的神经和精神症状，包括恶性紧张症和自主神经功能不稳定。尽管迅速启动了积极的免疫抑制治疗，包括使用皮质类固醇、血浆置换、静脉注射免疫球蛋白和利妥昔单抗，以及大剂量苯二氮䓬类药物治疗，但我们的患者仍持续表现出恶性紧张症。一旦开始进行电休克治疗（ECT），她的紧张症迅速缓解，反应良好。经过八个ECT周期治疗六周后，她恢复到了正常的基线认知和运动功能。
ECT对我们的患者是一种有效且耐受性良好的治疗方法，对于抗NMDA受体脑炎且紧张症对免疫抑制和苯二氮䓬类药物无反应的儿童患者，应考虑使用ECT进行治疗。
