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Autoimmune encephalitis: clinical spectrum and management
Practical Neurology ( IF 2.4 ) Pub Date : 2021-10-01 , DOI: 10.1136/practneurol-2020-002567
Christopher E Uy 1, 2 , Sophie Binks 1, 2 , Sarosh R Irani 2, 3
Affiliation  

Autoimmune encephalitis defines brain inflammation caused by a misdirected immune response against self-antigens expressed in the central nervous system. It comprises a heterogeneous group of disorders that are at least as common as infectious causes of encephalitis. The rapid and ongoing expansion of this field has been driven by the identification of several pathogenic autoantibodies that cause polysymptomatic neurological and neuropsychiatric diseases. These conditions often show highly distinctive cognitive, seizure and movement disorder phenotypes, making them clinically recognisable. Their early identification and treatment improve patient outcomes, and may aid rapid diagnosis of an underlying associated tumour. Here we summarise the well-known autoantibody-mediated encephalitis syndromes with neuronal cell-surface antigens. We focus on practical aspects of their diagnosis and treatment, offer our clinical experiences of managing such cases and highlight more basic neuroimmunological advances that will inform their future diagnosis and treatments.

中文翻译:

自身免疫性脑炎:临床表现和管理

自身免疫性脑炎定义了由针对中枢神经系统中表达的自身抗原的错误定向免疫反应引起的脑部炎症。它包括一组异质性疾病,这些疾病至少与脑炎的感染性病因一样常见。该领域的快速和持续扩展是由几种导致多症状神经和神经精神疾病的致病性自身抗体的鉴定推动的。这些病症通常表现出高度独特的认知、癫痫和运动障碍表型,使其在临床上可识别。他们的早期识别和治疗改善了患者的预后,并可能有助于快速诊断潜在的相关肿瘤。在这里,我们总结了众所周知的具有神经元细胞表面抗原的自身抗体介导的脑炎综合征。
更新日期:2021-09-17
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