Department of Neuro-oncology Hospices Civils de Lyon, Hôpital Neurologique, Bron, France.
Department of Neuro-oncology, Université Claude Bernard Lyon 1 Villeurbanne, Lyon, France.
Postgrad Med J. 2023 Jun 30;99(1173):669-678. doi: 10.1136/postgradmedj-2022-141766. Epub 2022 May 19.
Paraneoplastic neurological syndromes (PNSs) comprise a subset of immune-mediated nervous system diseases triggered by an underlying malignancy. Each syndrome usually shows a distinct clinical presentation and outcome according to the associated neural antibodies. PNSs generally have a subacute onset with rapid progression and severe neurological disability. However, some patients may have hyperacute onset or even show chronic progression mimicking neurodegenerative diseases. Updated diagnostic criteria for PNS have been recently established in order to increase diagnostic specificity and to encourage standardisation of research initiatives related to PNS. Treatment for PNS includes oncological therapy and immunomodulation to halt neurological deterioration although current treatment options are seldom effective in reversing disability. Nevertheless, growing knowledge and better understanding of PNS pathogenesis promise better recognition, earlier diagnosis and novel treatment strategies. Considering that PNSs provide a model of effective anticancer immunity, the impact of these studies will extend far beyond the field of neurology.
副肿瘤性神经系统综合征（PNSs）是由潜在恶性肿瘤引发的免疫介导性神经系统疾病的一个子集。根据相关神经抗体的不同，每种综合征通常表现出不同的临床特征和结局。PNS 通常呈亚急性起病，进展迅速，神经功能障碍严重。然而，一些患者可能呈超急性起病，甚至表现为类似于神经退行性疾病的慢性进展。为了提高诊断的特异性，并鼓励与 PNS 相关的研究计划标准化，最近已经建立了 PNS 的更新诊断标准。PNS 的治疗包括肿瘤治疗和免疫调节以阻止神经功能恶化，尽管目前的治疗选择很少能有效地逆转残疾。然而，对 PNS 发病机制的深入了解有望更好地识别、更早地诊断和制定新的治疗策略。鉴于 PNSs 提供了有效的抗癌免疫的模型，这些研究的影响将远远超出神经病学领域。
