4.4 Article

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

Journal

BMC NEUROLOGY
Volume 22, Issue 1, Pages -

Publisher

BMC
DOI: 10.1186/s12883-022-02625-1

Keywords

Lambert-Eaton myasthenic syndrome (LEMS); Autonomic dysfunction; Skin sympathetic response (SSR)

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This case report presents a patient with Lambert-Eaton myasthenic syndrome (LEMS) and autonomic disorder, providing detailed results of the skin sympathetic response (SSR) test. Due to the significant impact of autonomic dysfunction on clinical management, SSR test is recommended as an effective detection method.
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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