Anti-N-methyl-d-aspartate receptor encephalitis in a patient on long-term continuous immunomodulatory therapy

Lina M. Ariza-Serrano , Nicole Andrea Gómez Perdomo , Antonio Schlesinger , Manuel Tapias , Steven Hurtado
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Abstract

Background

Anti-N-methyl-d-aspartate [anti-NMDA] receptor encephalitis is one of the most common types of immune-mediated encephalitis. It is characterized by a neuropsychiatric syndrome associated with immunoglobulin G [IgG]-type antibodies against the NMDA receptor's GluN1 subunit in cerebrospinal fluid [CSF]. To our Knowledge, only six cases of autoimmune encephalitis with positive CSF NMDA receptor IgG autoantibodies have been reported among patients on long-term immunosuppressive therapies. The etiologies of all six cases were related to solid organ transplantation. Therefore, this report is the first to present a case of immunosuppression associated with an etiology other than post-transplant immunosuppression.

Case presentation

We present the case of a 59-year-old female patient who was on a long-term continuous immunomodulatory therapy for rheumatoid arthritis, and she consulted the emergency department several times for headache, symptoms of neurologic deficit, and subsequent progression to neuropsychiatric symptoms. CSF examination of the patient confirmed the presence of antibodies against the NMDA receptor; hence, the diagnosis of anti-NMDA receptor encephalitis was made.

Conclusions

It is important to recognize the clinical features of autoimmune encephalitis and to consider this condition in the differential diagnosis when neuropsychiatric symptoms are manifested in such patients. Patients on long-term immunomodulatory therapy present with atypical manifestations of anti-NMDA receptor encephalitis. The consideration of these manifestations in the differential diagnosis can ensure efficient diagnosis, management, and reduction in the consequences affecting morbidity and mortality of these patients.

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一例长期持续免疫调节治疗的抗N-甲基-d-天冬氨酸受体脑炎患者
背景抗N-甲基-d-天冬氨酸受体脑炎是最常见的免疫介导型脑炎之一。其特征是与脑脊液中针对NMDA受体GluN1亚基的免疫球蛋白G[IgG]型抗体相关的神经精神综合征。据我们所知,在接受长期免疫抑制治疗的患者中,只有6例CSF NMDA受体IgG自身抗体阳性的自身免疫性脑炎病例被报道。所有6例病例的病因均与实体器官移植有关。因此,本报告首次提出了与移植后免疫抑制以外的病因相关的免疫抑制病例。病例介绍我们介绍了一名59岁的女性患者的病例,她正在接受类风湿性关节炎的长期持续免疫调节治疗,她因头痛、神经功能缺损症状以及随后发展为神经精神症状多次咨询急诊科。对患者的CSF检查证实存在针对NMDA受体的抗体;从而诊断为抗NMDA受体脑炎。结论当自身免疫性脑炎患者出现神经精神症状时,认识其临床特征并在鉴别诊断中考虑这种情况很重要。接受长期免疫调节治疗的患者表现为抗NMDA受体脑炎的非典型表现。在鉴别诊断中考虑这些表现可以确保有效的诊断、管理,并减少影响这些患者发病率和死亡率的后果。
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