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Lambert-Eaton 肌无力综合征。

Lambert-eaton myasthenic syndrome.

机构信息

Maria B. Weimer, MD Louisiana State University Health Sciences Center, Department of Neurology, 200 Henry Clay Suite 3314, New Orleans, LA 70118, USA.

出版信息

Curr Treat Options Neurol. 2009 Mar;11(2):77-84. doi: 10.1007/s11940-009-0010-z.

Abstract

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.

摘要

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。

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