Hülsbrink Robert, Hashemolhosseini Said
Institut für Biochemie, Friedrich-Alexander-Universität Erlang en-Nürnberg, Fahrstrasse 17, 91054 Erlangen, Germany.
Institut für Biochemie, Friedrich-Alexander-Universität Erlang en-Nürnberg, Fahrstrasse 17, 91054 Erlangen, Germany.
Clin Neurophysiol. 2014 Dec;125(12):2328-36. doi: 10.1016/j.clinph.2014.06.031. Epub 2014 Jul 4.
Lambert-Eaton myasthenic syndrome (LEMS) describes a rare human autoimmune disorder of the neuromuscular junction (NMJ). Clinically, LEMS patients suffer from characteristic muscle weakness that is caused by the presence of antibodies directed against their voltage-gated calcium channels (VGCC). These channels are localized in the presynaptic membrane of their motor nerve terminals. Binding of autoimmune antibodies to the VGCCs leads to reduced neuromuscular transmission. In approximately 50% of the patients, LEMS is reflected by a paraneoplastic manifestation and most commonly associated with a small cell lung carcinoma (SCLC) whose cells also express VGCCs in their plasma membrane. Better understanding of the pathophysiological mechanisms of LEMS has helped with the development of new diagnostic approaches and has led to targeted symptomatic and immunosuppressive therapy. For LEMS patients with an underlying malignancy, tumor therapy is the first choice to date.
兰伯特-伊顿肌无力综合征（LEMS）是一种罕见的人类神经肌肉接头（NMJ）自身免疫性疾病。临床上，LEMS患者患有特征性肌肉无力，这是由针对其电压门控钙通道（VGCC）的抗体所致。这些通道位于运动神经末梢的突触前膜。自身免疫抗体与VGCC结合导致神经肌肉传递减少。在大约50%的患者中，LEMS表现为副肿瘤综合征，最常见于小细胞肺癌（SCLC），其细胞在质膜中也表达VGCC。对LEMS病理生理机制的更好理解有助于开发新的诊断方法，并导致了有针对性的对症和免疫抑制治疗。对于患有潜在恶性肿瘤的LEMS患者，迄今为止肿瘤治疗是首选。
