Randall David P
Neuromuscular Neurology, Advocate Lutheran General Hospital, Park Ridge, IL, 1875 Dempster St, 60068, United States.
Dis Mon. 2025 Aug;71(8):101967. doi: 10.1016/j.disamonth.2025.101967. Epub 2025 Jun 21.
Lambert-Eaton myasthenic syndrome (LEMS) is a rare disease affecting the neuromuscular junction. It causes weakness due to the failure of acetylcholine transmission at the neuromuscular junction. It is also responsible for autonomic symptoms due to the loss of acetylcholine release at autonomic nerves resulting in most often dry mouth, but also impotence, sweating disturbance and orthostatic hypotension. The underlying pathology is split nearly evenly as either paraneoplastic or autoimmune. The challenges in making a diagnosis result in a delay which may correlate with a later initiation of cancer therapy in those with cancers, predominantly small cell lung cancer. There are effective treatments for symptoms of LEMS that work directly at the neuromuscular junction, as well as treatments for the cancer if present. Various types of immunosuppression can be beneficial for both paraneoplastic (T LEMS) and non-tumor LEMS (NT LEMS).
兰伯特-伊顿肌无力综合征(LEMS)是一种影响神经肌肉接头的罕见疾病。它因神经肌肉接头处乙酰胆碱传递失败而导致肌无力。由于自主神经处乙酰胆碱释放减少,它还会引发自主神经症状,最常见的是口干,也会导致阳痿、出汗障碍和体位性低血压。其潜在病理几乎平均分为副肿瘤性或自身免疫性。诊断方面的挑战会导致诊断延迟,这可能与癌症患者(主要是小细胞肺癌患者)癌症治疗的延迟启动相关。对于LEMS症状有直接作用于神经肌肉接头的有效治疗方法,对于存在的癌症也有相应治疗方法。各种类型的免疫抑制对副肿瘤性LEMS(T LEMS)和非肿瘤性LEMS(NT LEMS)都可能有益。