{"title":"兰伯特-伊顿肌无力综合征的识别、生理和治疗。","authors":"David P Randall","doi":"10.1016/j.disamonth.2025.101967","DOIUrl":null,"url":null,"abstract":"<p><p>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).</p>","PeriodicalId":51017,"journal":{"name":"Dm Disease-A-Month","volume":" ","pages":"101967"},"PeriodicalIF":4.3000,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"The recognition, physiology, and treatment of Lambert-Eaton myasthenic syndrome.\",\"authors\":\"David P Randall\",\"doi\":\"10.1016/j.disamonth.2025.101967\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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).</p>\",\"PeriodicalId\":51017,\"journal\":{\"name\":\"Dm Disease-A-Month\",\"volume\":\" \",\"pages\":\"101967\"},\"PeriodicalIF\":4.3000,\"publicationDate\":\"2025-06-20\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Dm Disease-A-Month\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1016/j.disamonth.2025.101967\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Dm Disease-A-Month","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.disamonth.2025.101967","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
The recognition, physiology, and treatment of Lambert-Eaton myasthenic syndrome.
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).
期刊介绍:
Designed for primary care physicians, each issue of Disease-a-Month presents an in-depth review of a single topic. In this way, the publication can cover all aspects of the topic - pathophysiology, clinical features of the disease or condition, diagnostic techniques, therapeutic approaches, and prognosis.