Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands; Department of Neurology, Groene Hart Hospital, Gouda, The Netherlands.
Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
Handb Clin Neurol. 2024;200:307-325. doi: 10.1016/B978-0-12-823912-4.00012-8.
Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disease characterized by proximal muscle weakness, loss of tendon reflexes, and autonomic dysfunction. Muscle weakness usually starts in the upper legs and can progress to oculobulbar and in severe cases respiratory muscles. P/Q-type voltage-gated calcium channels (VGCCs) localized in the presynaptic motor nerve terminal and in the autonomic nervous system are targeted by antibodies in LEMS patients. These antibodies can be detected in about 90% of patients, and the presence of decrement and increment upon repetitive nerve stimulation is also a highly sensitive diagnostic test. Rapid diagnosis is important because of the association with SCLC in 50%-60% of patients, which stresses the need for vigorous tumor screening after diagnosis. Clinical parameters can predict tumor probability and guide frequency of tumor screening. Treatment of the tumor as well as symptomatic treatment and immunosuppression can effectively control symptoms in the majority of patients.
Lambert-Eaton 肌无力综合征（LEMS）是一种罕见的自身免疫性疾病，其特征为近端肌无力、腱反射消失和自主神经功能障碍。肌无力通常始于大腿，可进展至眼外肌和呼吸肌，在严重情况下。P/Q 型电压门控钙通道（VGCCs）定位于突触前运动神经末梢和自主神经系统，是 LEMS 患者抗体的靶标。这些抗体可在约 90%的患者中检测到，重复神经刺激时的递减和递增也是一种高度敏感的诊断测试。快速诊断很重要，因为 50%-60%的患者与小细胞肺癌相关，这强调了诊断后需要进行积极的肿瘤筛查。临床参数可预测肿瘤概率，并指导肿瘤筛查的频率。对肿瘤进行治疗以及对症治疗和免疫抑制治疗可有效控制大多数患者的症状。
