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自身免疫性脑炎与免疫治疗:来自阿根廷的经验教训。

Autoimmune encephalitis and immune therapy: lessons from Argentina.

机构信息

Department of Neurology, Neuroimmunology Unit, Ramos Mejía Hospital, School of Medicine, Buenos Aires University, Buenos Aires, Argentina.

出版信息

Acta Neurol Belg. 2020 Jun;120(3):565-572. doi: 10.1007/s13760-018-1013-x. Epub 2018 Sep 4.

DOI:10.1007/s13760-018-1013-x
PMID:30182259
Abstract

Autoimmune encephalitis (AE) is a common cause of noninfectious acute encephalitis. We aimed to provide the first review of immune therapy regimens used for patients with AE in Latin America, as well as the safety and efficacy associated with them, by reviewing the medical records of Argentine patients with AE treated between 2013 and 2018. Data included clinical symptoms, laboratory tests, electroencephalography, magnetic resonance imaging, cerebral spinal fluid, and neoplasm screenings. We examined ten AE patients who received first-line immunotherapy at a median of 2.5 months following symptom onset. Among these patients, five required second-line treatment: three received therapy at a median of 4 months (2-112) after symptom onset and were treated with rituximab, while two received therapy at a median of 4.5 (4-5) months after onset and received methylprednisolone for 6 months and initiated chronic treatment with azathioprine. By the last follow-up, their respective outcomes improved significantly. On the modified Rankin Scale, the median score decreased from 5 to 1 (p ≤ 0.05). Only two of the ten patients in our series experienced relapses; both had been treated with a combination of methylprednisolone and IVIG. The regimen after recurrence included rituximab and corticoids plus azathioprine. Neither patient had experienced another relapse by their last follow-up. Our findings demonstrate the importance of early and aggressive immunosuppressive therapy to achieve a good clinical outcome and a fast recovery without relapses.

摘要

自身免疫性脑炎 (AE) 是一种常见的非传染性急性脑炎的病因。我们旨在通过回顾 2013 年至 2018 年间在阿根廷接受治疗的 AE 患者的病历,提供拉丁美洲 AE 患者免疫治疗方案的使用情况、安全性和有效性的第一份综述,这些方案用于治疗 AE 患者。数据包括临床症状、实验室检查、脑电图、磁共振成像、脑脊液和肿瘤筛查。我们检查了 10 名在发病后中位数 2.5 个月接受一线免疫治疗的 AE 患者。在这些患者中,有 5 名需要二线治疗:3 名在发病后中位数 4 个月(2-112)接受治疗,接受利妥昔单抗治疗,2 名在发病后中位数 4.5 个月接受治疗(4-5)接受甲基强的松龙治疗 6 个月,并开始接受硫唑嘌呤的慢性治疗。在最后一次随访时,他们各自的病情均有显著改善。在改良 Rankin 量表上,中位数评分从 5 分降至 1 分(p≤0.05)。在我们的系列中,只有 2 名患者经历了复发;两者均接受了甲基强的松龙和 IVIG 的联合治疗。复发后的治疗方案包括利妥昔单抗和皮质激素加硫唑嘌呤。到最后一次随访时,两名患者均未再次复发。我们的研究结果表明,早期和积极的免疫抑制治疗对于实现良好的临床结果和快速康复而无复发至关重要。

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