RT Journal Article
SR Electronic
T1 Long‐term follow‐up, quality of life, and survival of patients with Lambert‐Eaton myasthenic syndrome
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP e511
OP e520
DO 10.1212/WNL.0000000000008747
VO 94
IS 5
A1 Lipka, Alexander F.
A1 Boldingh, Marion I.
A1 van Zwet, Erik W.
A1 Schreurs, Marco W.J.
A1 Kuks, Jan B.M.
A1 Tallaksen, Chantal M.
A1 Titulaer, Maarten J.
A1 Verschuuren, Jan J.G.M.
YR 2020
UL http://n.neurology.org/content/94/5/e511.abstract
AB Objective To study survival and to characterize long-term functional impairments and health-related quality of life (HRQOL) of patients with Lambert-Eaton myasthenic syndrome (LEMS).Methods In this observational study, survival of patients with LEMS, separately for nontumor (NT) and small cell lung cancer (SCLC), was compared to that of the Dutch general population and patients with SCLC. Disease course in patients with LEMS was recorded retrospectively. Several scales for functional impairments and health-related quality of life were assessed.Results We included 150 patients with LEMS. Survival was similar to that of the general population in 65 patients with NT-LEMS. Tumor survival was significantly longer in 81 patients with SCLC-LEMS compared to patients with non-LEMS SCLC (overall median survival 17 vs 7.0 months, p < 0.0001). At diagnosis, 39 (62%) of 63 patients with complete follow-up data were independent for activities of daily living, improving to 85% at the 1-year follow-up. The physical HRQOL composite score (55.9) was significantly lower than in the general population (76.3, p < 0.0001) and comparable to that of patients with myasthenia gravis (60.5). The mental HRQOL composite score was 71.8 in patients with LEMS, comparable to that of the general population (77.9, p = 0.19) and patients with myasthenia gravis (70.3).Conclusions This study shows that patients with NT-LEMS have normal survival. Patients with SCLC-LEMS have an improved tumor survival, even after correction for tumor stage. A majority of patients with LEMS report a stable disease course and remain or become independent for self-care after treatment.CI=confidence interval; DELTA-P=Dutch-English LEMS Tumour Association Prediction; HRQOL=health-related quality of life; KPS=Karnofsky Performance Scale; LEMS=Lambert-Eaton myasthenic syndrome; MCS=Mental Composite Score; MG=myasthenia gravis; mRS=modified Rankin Scale; PCS=Physical Composite Score; SCLC=small cell lung cancer; SF-36=Short Form-36; VGCC=voltage-gated calcium channel