Autonomic dysfunction detected by skin sympathetic response in Lambert-Eaton myasthenic syndrome: a case report

被引:5
作者
Zhang, Jinghong [1 ]
Huang, Xusheng [1 ]
Shi, Qiang [1 ]
机构
[1] Chinese Peoples Liberat Army Gen Hosp, Med Ctr 1, Dept Neurol, Beijing, Peoples R China
关键词
Lambert-Eaton myasthenic syndrome (LEMS); Autonomic dysfunction; Skin sympathetic response (SSR); AUTOANTIBODIES; ANTIBODIES; MECHANISMS; GRAVIS;
D O I
10.1186/s12883-022-02625-1
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Background: Lambert-Eaton myasthenic syndrome (LEMS) is a type of paraneoplastic syndrome that may initially manifest itself with proximal weakness and gait abnormalities. Approximately up to 50% of LEMS patients have a primary autonomic dysfunction. Case presentation: We present here a case of a 75-year-old male with symmetric proximal muscle weakness, dry mouth and constipation. The cutaneous response to scratch and upright tilt-table testing were positive. A repetitive nerve stimulation test showed that there was a decremental response of compound muscle action potential (CMAP) amplitude at 3 Hz while an incremental response at 20 Hz. The presence of antibodies against voltage-gated calcium channels (VGCC) confirmed the diagnosis. Because of the prominent symptom of autonomic disorder, the patient further underwent the test of skin sympathetic response (SSR). Lower amplitude and longer response duration were found in palms, while it evoked no response in soles. Conclusions: In this case, we present the detailed results of SSR test on a patient suffering LEMS with autonomic disorder. Since autonomic dysfunction has a significant impact on clinical management and SSR test is an effective detection method, we recommend that SSR test be performed on patients with LEMS regularly.
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