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Neurology. 2019 Dec 12. pii: 10.1212/WNL.0000000000008747. doi: 10.1212/WNL.0000000000008747. [Epub ahead of print]

Long-term follow-up, quality of life, and survival of patients with Lambert-Eaton myasthenic syndrome.

Author information

1
From the Departments of Neurology (A.F.L., J.J.G.M.V.) and Biostatistics (E.W.v.Z.), Leiden University Medical Center; Department of Neurology (A.F.L.), Groene Hart Hospital, Gouda, the Netherlands; Department of Neurology (M.I.B., C.M.T.), Oslo University Hospital, Norway; Departments of Immunology (M.W.J.S.) and Neurology (M.J.T.), Erasmus University Medical Center, Rotterdam; Department of Neurology (J.B.M.K.), University Medical Center Groningen, the Netherlands; and Faculty of Medicine (C.M.T.), University of Oslo, Norway. a.f.lipka@lumc.nl.
2
From the Departments of Neurology (A.F.L., J.J.G.M.V.) and Biostatistics (E.W.v.Z.), Leiden University Medical Center; Department of Neurology (A.F.L.), Groene Hart Hospital, Gouda, the Netherlands; Department of Neurology (M.I.B., C.M.T.), Oslo University Hospital, Norway; Departments of Immunology (M.W.J.S.) and Neurology (M.J.T.), Erasmus University Medical Center, Rotterdam; Department of Neurology (J.B.M.K.), University Medical Center Groningen, the Netherlands; and Faculty of Medicine (C.M.T.), University of Oslo, Norway.

Abstract

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.

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