Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, Oxford, UK.
Department of Neurology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
Pract Neurol. 2021 Oct;21(5):412-423. doi: 10.1136/practneurol-2020-002567. Epub 2021 Jun 9.
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.
自身免疫性脑炎定义为中枢神经系统表达的自身抗原引起的免疫反应异常导致的脑炎症。它包含一组异质性疾病,至少与脑炎的感染性病因一样常见。这个领域的快速发展是由几种致病性自身抗体的鉴定所驱动的,这些自身抗体可引起多系统的神经和神经精神疾病。这些疾病通常表现出高度独特的认知、癫痫和运动障碍表型,使其具有临床可识别性。早期识别和治疗可改善患者预后,并可能有助于快速诊断潜在的相关肿瘤。在这里,我们总结了具有神经元细胞表面抗原的已知的抗体介导的脑炎综合征。我们重点介绍了它们诊断和治疗的实际方面,分享了我们管理此类病例的临床经验,并强调了更基础的神经免疫学进展,这些进展将为它们的未来诊断和治疗提供信息。