Centre de Référence, de Diagnostic et de Traitement des Syndromes Neurologiques Paranéoplastiques, Hospices Civils de Lyon, France.
Curr Opin Oncol. 2009 Nov;21(6):566-72. doi: 10.1097/CCO.0b013e3283306647.
To describe specificities and new advances in treatment of paraneoplastic neurological syndromes (PNS).
PNS are defined as neurological syndromes of unknown cause that often antedate the diagnosis of an underlying cancer that is usually not clinically evident. The clinical signs of PNS are accurately described and 'classical' PNS have been established. Within the context of these syndromes, the concept of limbic encephalitis has evolved dramatically in the last 2 years due to the description of new auto-antibodies. The diagnosis and treatment of the associated cancer remains the key goal of the clinical management of PNS. However, the specific treatment of the neurological symptoms primarily depends on the immunological findings.
There is increasing recognition of an extensive array of PNS and of several paraneoplastic antibodies as biological markers of these disorders. Basic immunological studies support the pathogenic role of some of these antibodies. Others are only markers of the disease.
描述副肿瘤性神经系统综合征(PNS)的治疗特点和新进展。
PNS 定义为原因不明的神经系统综合征,常先于潜在癌症的诊断,而后者通常在临床上并不明显。PNS 的临床特征得到了准确描述,并且已经确立了“经典”的 PNS。在这些综合征的背景下,由于新自身抗体的描述,边缘性脑炎的概念在过去 2 年中发生了巨大变化。诊断和治疗相关癌症仍然是 PNS 临床管理的关键目标。然而,神经症状的具体治疗主要取决于免疫学发现。
人们越来越认识到广泛的 PNS 以及几种副肿瘤性抗体作为这些疾病的生物标志物。基础免疫学研究支持其中一些抗体的致病作用。其他抗体只是疾病的标志物。