巴西成人和儿科人群自身免疫性脑炎诊断和治疗的共识建议。

Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adult and pediatric populations.

机构信息

Hospital Israelita Albert Einstein, Instituto do Cérebro, São Paulo, São Paulo SP, Brazil.

Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, Rio de Janeiro RJ, Brazil.

出版信息

Arq Neuropsiquiatr. 2024 Jul;82(7):1-15. doi: 10.1055/s-0044-1788586. Epub 2024 Aug 1.

DOI:10.1055/s-0044-1788586

PMID:39089672

Abstract

BACKGROUND

Autoimmune encephalitis (AIE) is a group of inflammatory diseases characterized by the presence of antibodies against neuronal and glial antigens, leading to subacute psychiatric symptoms, memory complaints, and movement disorders. The patients are predominantly young, and delays in treatment are associated with worse prognosis.

OBJECTIVE

With the support of the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, ABN) and the Brazilian Society of Child Neurology (Sociedade Brasileira de Neurologia Infantil, SBNI), a consensus on the diagnosis and treatment of AIE in Brazil was developed using the Delphi method.

METHODS

A total of 25 panelists, including adult and child neurologists, participated in the study.

RESULTS

The panelists agreed that patients fulfilling criteria for possible AIE should be screened for antineuronal antibodies in the serum and cerebrospinal fluid (CSF) using the tissue-based assay (TBA) and cell-based assay (CBA) techniques. Children should also be screened for anti-myelin oligodendrocyte glucoprotein antibodies (anti-MOG). Treatment should be started within the first 4 weeks of symptoms. The first-line option is methylprednisolone plus intravenous immunoglobulin (IVIG) or plasmapheresis, the second-line includes rituximab and/or cyclophosphamide, while third-line treatment options are bortezomib and tocilizumab. Most seizures in AIE are symptomatic, and antiseizure medications may be weaned after the acute stage. In anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, the panelists have agreed that oral immunosuppressant agents should not be used. Patients should be evaluated at the acute and postacute stages using functional and cognitive scales, such as the Mini-Mental State Examination (MMSE), the Montreal Cognitive Assessment (MoCA), the Modified Rankin Scale (mRS), and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE).

CONCLUSION

The present study provides tangible evidence for the effective management of AIE patients within the Brazilian healthcare system.

摘要

背景

自身免疫性脑炎（Autoimmune Encephalitis，AIE）是一组以神经元和神经胶质抗原抗体为特征的炎症性疾病，导致亚急性精神症状、记忆主诉和运动障碍。患者主要为年轻人，治疗延迟与预后较差相关。

目的

在巴西神经病学学会（Academia Brasileira de Neurologia，ABN）和巴西儿童神经病学学会（Sociedade Brasileira de Neurologia Infantil，SBNI）的支持下，采用德尔菲法制定了巴西 AIE 的诊断和治疗共识。

方法

共有 25 名专家小组成员，包括成人和儿童神经病学家，参与了这项研究。

结果

专家小组成员一致认为，疑似 AIE 患者应通过组织基础检测（Tissue-based Assay，TBA）和细胞基础检测（Cell-based Assay，CBA）技术筛查血清和脑脊液（Cerebrospinal Fluid，CSF）中的神经元抗体。儿童还应筛查抗髓鞘少突胶质细胞糖蛋白抗体（Anti-myelin oligodendrocyte glycoprotein antibodies，Anti-MOG）。症状出现后应在 4 周内开始治疗。一线治疗方案为甲泼尼龙联合静脉注射免疫球蛋白（Intravenous Immunoglobulin，IVIG）或血浆置换，二线治疗方案为利妥昔单抗和/或环磷酰胺，三线治疗方案为硼替佐米和托珠单抗。AIE 中的大多数癫痫发作都是症状性的，急性阶段后可逐渐减少抗癫痫药物。在抗 N-甲基-D-天冬氨酸受体（N-methyl-D-aspartate receptor，NMDAR）脑炎中，专家小组成员一致认为不应使用口服免疫抑制剂。患者应在急性和亚急性期使用功能和认知量表（如简易精神状态检查量表（Mini-Mental State Examination，MMSE）、蒙特利尔认知评估量表（Montreal Cognitive Assessment，MoCA）、改良 Rankin 量表（Modified Rankin Scale，mRS）和自身免疫性脑炎临床评估量表（Clinical Assessment Scale in Autoimmune Encephalitis，CASE）进行评估。