Hospices Civils de Lyon, Centre de Référence Maladies Rares Syndromes Neurologiques Paranéoplasiques, Hôpital Neurologique, Neurologie B, 59, boulevard Pinel, 69677 Bron, France; INMG (NeuroMyoGene Institute) Inserm U1217/CNRS UMR 5310, 8, avenue Rockfeller, 69372 Lyon, France; Université de Lyon, Université Claude Bernard Lyon 1, 8, avenue Rockfeller, 69372 Lyon, France.
Hospices Civils de Lyon, Centre de Référence Maladies Rares Syndromes Neurologiques Paranéoplasiques, Hôpital Neurologique, Neurologie B, 59, boulevard Pinel, 69677 Bron, France; INMG (NeuroMyoGene Institute) Inserm U1217/CNRS UMR 5310, 8, avenue Rockfeller, 69372 Lyon, France; Université de Lyon, Université Claude Bernard Lyon 1, 8, avenue Rockfeller, 69372 Lyon, France.
Rev Neurol (Paris). 2018 Nov;174(9):597-607. doi: 10.1016/j.neurol.2018.07.006. Epub 2018 Sep 7.
Movement disorders are extremely common and diverse in autoimmune encephalitis (AE) and paraneoplastic neurological syndromes (PNS). They can sometimes represent the main neurological disorder of a given patient, or just be part of a larger neurological syndrome. Early diagnosis of AE or PNS is essential, as the associated abnormal movements can be effectively treated with immunomodulators. Nevertheless, the diagnosis is often delayed because of the large number of differential diagnoses (infections, metabolic disorders, genetic and degenerative diseases) and because the semiology of abnormal movements arising during AE and PNS is often not well known. However, there are highly specific clinical features, depending on the associated autoantibodies, age and gender of the patient, and associated cancers. Such features are likely to rely on specific mechanisms, the knowledge of which could lead to new therapeutic proposals. Also, the growing body of work on AE and PNS provides a better understanding of the links between immunity and neuronal degeneration, and immunity and genetic specificities. Thus, the purpose of this article is to present the current knowledge and different subtypes of movement disorders associated with AE and PNS, as well as the mechanisms that can lead to neuronal dysfunction.
运动障碍在自身免疫性脑炎(AE)和副肿瘤性神经系统综合征(PNS)中极为常见且多样。它们有时可能代表特定患者的主要神经障碍,或者只是更大的神经系统综合征的一部分。早期诊断 AE 或 PNS 至关重要,因为相关的异常运动可以通过免疫调节剂有效治疗。然而,由于大量的鉴别诊断(感染、代谢紊乱、遗传和退行性疾病),以及 AE 和 PNS 期间出现的异常运动的症状通常不为人所知,因此诊断常常被延迟。然而,根据相关自身抗体、患者的年龄和性别以及相关癌症,存在高度特异性的临床特征。这些特征可能依赖于特定的机制,对这些机制的了解可能会带来新的治疗建议。此外,AE 和 PNS 方面不断增加的工作提供了对免疫与神经元变性之间以及免疫与遗传特异性之间联系的更好理解。因此,本文的目的是介绍与 AE 和 PNS 相关的运动障碍的当前知识和不同亚型,以及可能导致神经元功能障碍的机制。