Maria B. Weimer, MD Louisiana State University Health Sciences Center, Department of Neurology, 200 Henry Clay Suite 3314, New Orleans, LA 70118, USA.
Curr Treat Options Neurol. 2009 Mar;11(2):77-84. doi: 10.1007/s11940-009-0010-z.
Lambert Eaton myasthenic syndrome (LEMS) is a rare neuromuscular junction disease. Often, the signs and symptoms of LEMS are mistaken for myasthenia gravis and therefore the workup is misdirected. A physician must look for an occult malignancy when the diagnosis is made and then continue to search for a malignancy for at least 5 years after diagnosis. The diagnosis of LEMS can be confirmed with electrophysiologic studies or with serum calcium channel antibodies. In most patients with LEMS, 3,4-diaminopyridine will improve strength. In patients without malignancy, immunosuppressants do have a role in the treatment of LEMS. Patients and physicians must be aware that certain situations and drugs may exacerbate weakness.
Lambert Eaton 肌无力综合征(LEMS)是一种罕见的神经肌肉接头疾病。通常,LEMS 的体征和症状被误诊为重症肌无力,因此检查方向有误。当诊断为 LEMS 时,医生必须寻找隐匿性恶性肿瘤,然后在诊断后至少 5 年内继续寻找恶性肿瘤。LEMS 的诊断可以通过电生理研究或血清钙通道抗体来确认。在大多数 LEMS 患者中,3,4-二氨基吡啶可改善肌力。在没有恶性肿瘤的患者中,免疫抑制剂在 LEMS 的治疗中确实有一定作用。患者和医生必须意识到某些情况和药物可能会使肌无力恶化。