Okada Akinori, Koike Haruki, Nakamura Tomohiko, Motomura Masakatu, Sobue Gen
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-Cho, Showa-Ku, Nagoya 466-8550, Japan.
Medical Electronic Course Department of Electrical and Electronics Engineering, The Faculty of Engineering, Nagasaki Institute of Applied Science, 536 Abamachi, Nagasaki, Nagasaki Pref. 851-0193, Japan.
Neuromuscul Disord. 2015 Jan;25(1):70-2. doi: 10.1016/j.nmd.2014.08.006. Epub 2014 Sep 10.
We evaluated the efficacy of intravenous immunoglobulin (IVIg) in a patient with Lambert-Eaton myasthenic syndrome (LEMS). Comprehensive clinical and electrophysiological testing was performed on a 34-year-old woman with progressive limb weakness, before and after IVIg treatment. Neurological examination revealed muscle weakness, predominantly in the proximal parts of the limbs. Muscle weakness improved following a short period of maximum voluntary muscle contraction. A repetitive low-rate (3-Hz) nerve stimulation test of the abductor hallucis was normal, but high-rate (20-Hz) stimulation induced an incremental response. Anti-presynaptic P/Q-type voltage-gated calcium channel (P/Q-VGCC) antibodies were absent in the patient's serum. Whole body computed tomography revealed no tumors. We diagnosed seronegative LEMS without tumor and treated the patient with IVIg. Both clinical and electrophysiological indices improved gradually after treatment. This case study indicates that treatment with IVIg is equally effective for LEMS that is seronegative or seropositive for P/Q-VGCC antibodies.
我们评估了静脉注射免疫球蛋白（IVIg）对一名兰伯特-伊顿肌无力综合征（LEMS）患者的疗效。对一名34岁进行性肢体无力的女性患者在IVIg治疗前后进行了全面的临床和电生理测试。神经系统检查发现肌肉无力，主要位于肢体近端。在短时间最大自主肌肉收缩后，肌肉无力有所改善。对拇展肌进行的重复性低频率（3Hz）神经刺激测试正常，但高频率（20Hz）刺激诱发递增反应。患者血清中不存在抗突触前P/Q型电压门控钙通道（P/Q-VGCC）抗体。全身计算机断层扫描未发现肿瘤。我们诊断该患者为无肿瘤的血清阴性LEMS，并给予IVIg治疗。治疗后临床和电生理指标均逐渐改善。该病例研究表明，IVIg治疗对于P/Q-VGCC抗体血清阴性或血清阳性的LEMS同样有效。
