Clinical characteristics and long term follow-up of Lambert-Eaton myasthenia syndrome in patients with and without small cell lung cancer

Abstract

In order to describe the clinical characteristics, treatment response and long-term follow up in Lambert-Eaton myasthenic syndrome (LEMS) patients with and without small cell lung cancer (SCLC) in East China, patients seen in Huashan Hospital from January 1997 to December 2017 were included. Clinical information was collected retrospectively and quantitative MG (QMG) score, manual muscle testing (MMT), activities of daily living (ADL) scale were evaluated when the patients were followed up. Of 50 patients, 23 (46%) were SCLC-LEMS and 20 (40%) were nontumor LEMS (NT-LEMS). The median onset age was 55.5 (18-86) years old and the gender ratio was about 1.8:1. It took less time to make the diagnosis (median time: 6 vs 22.5 months, p = 0.0003) and there were more patients with other paraneoplastic syndromes in SCLC-LEMS group than in NT-LEMS group (8/23 vs 0/20, p = 0.0042). Electrophysiologically, the peroneal compound motor action potential (CMAP) of rest showed difference between SCLC-LEMS and NT-LEMS (0.8 vs 1.6 mV, p = 0.0499). The median survival time of 19 SCLC-LEMS patients since the diagnosis of SCLC was 30 months. According to their survival time, SCLC patients with LEMS showed a more favorable prognosis than those without LEMS. In the time of follow-up, most NT-LEMS showed improvement or obtained status of CSR/PR/MM after immunosuppressive therapy and no significant difference in proportion of achieving CSR/PR/MM was found between SCLC-LEMS and NT-LEMS patients (0/5 vs 6/13, p = 0.114).

Keywords: Electrophysiology; Lambert–Eaton myasthenic syndrome; Small cell lung cancer; Survival.