Long-term survival in paraneoplastic Lambert-Eaton myasthenic syndrome
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Abstract
Objective: To establish whether improved tumor survival in patients with Lambert-Eaton myasthenic syndrome (LEMS) and small-cell lung cancer (SCLC) was due to known prognostic risk factors or an effect of LEMS independently, perhaps as a result of circulating factors.
Methods: We undertook a prospective observational cohort study of patients with LEMS attending Nottingham University Hospitals, UK, or via the British Neurological Surveillance Unit. In parallel, patients with a new diagnosis of biopsy-proven SCLC were enrolled, examined for neurologic illness, and followed up until death or study end.
Results: Between May 2005 and November 2014, we recruited 31 patients with LEMS and SCLC and 279 patients with SCLC without neurologic illness. Allowing for known SCLC survival prognostic factors of disease extent, age, sex, performance status, and sodium values, multivariate Cox regression analysis showed that the presence of LEMS with SCLC conferred a significant survival advantage independently of the other prognostic variables (hazard ratio 1.756, 95% confidence interval 1.137–2.709, p = 0.011).
Conclusions: Improved SCLC tumor survival seen in patients with LEMS and SCLC may not be due solely to lead time bias, given that survival advantage remains after allowing for other prognostic factors and that the same degree of survival advantage is not seen in patients with paraneoplastic neurologic syndromes other than LEMS presenting before SCLC diagnosis.
GLOSSARY
- LEMS=
- Lambert-Eaton myasthenic syndrome;
- PNS=
- paraneoplastic neurologic syndromes;
- SCLC=
- small-cell lung cancer;
- SOX2=
- SRY-Box 2;
- TNM=
- tumor, node, metastasis;
- UK-BNSU=
- British Neurological Surveillance Unit;
- VGCC=
- voltage-gated calcium channel
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at Neurology.org
- Received September 29, 2016.
- Accepted in final form January 17, 2017.
- © 2017 American Academy of Neurology
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