Gilhus Nils Erik
Department of Clinical Medicine, University of Bergen, 5020 Bergen, Norway.
Autoimmune Dis. 2011;2011:973808. doi: 10.4061/2011/973808. Epub 2011 Sep 29.
Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare disease with a well-characterized pathogenesis. In 50% of the patients, LEMS is a paraneoplastic manifestation and caused by a small cell lung carcinoma (SCLC). Both LEMS patients with SCLC and those without this tumour have in 85% of cases pathogenetic antibodies of very high LEMS specificity against voltage-gated calcium channels (VGCCs) in the cell membrane of the presynaptic motor nerve terminal. Better understanding of LEMS pathogenesis has lead to targeted symptomatic therapy aimed at the neuromuscular junction and to semispecific immuno-suppression. For SCLC LEMS, tumour therapy is essential.
兰伯特-伊顿肌无力综合征（LEMS）是一种发病机制明确的罕见疾病。50%的患者中，LEMS是一种副肿瘤表现，由小细胞肺癌（SCLC）引起。患有SCLC的LEMS患者和未患该肿瘤的患者中，85%的病例在突触前运动神经末梢细胞膜上存在对电压门控钙通道（VGCCs）具有高度LEMS特异性的致病性抗体。对LEMS发病机制的深入了解已导致针对神经肌肉接头的靶向对症治疗和半特异性免疫抑制。对于SCLC相关的LEMS，肿瘤治疗至关重要。
