Prediction of small cell lung cancer in the Lambert-Eaton myasthenic syndrome

Author(s):
Titulaer, M.J.
Published
2010
Publication type
Doctoral Thesis

Abstract

The Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder in which patients have muscle weakness and autonomic dysfunction. Half of the patients have small cell lung cancer (SCLC). SCLC is a very aggressive lung tumour with a median survival of only 9 months. Quick discovery and treatment is of uttermost importance for both patient and physician to improve the chance of cure. SCLC-LEMS is related to smoking, a higher age at onset and weight loss. The clinical symptoms develop more quickly and more severely if the LEMS is related to SCLC. The discovery of SOX antibodies is described specific for SCLC-LEMS. Based on simple epidemiological and clinical parameters, we created the DELTA-P score and validated it in an independent second cohort. The DELTA-P score predicts already shortly after onset of symptoms with 95% accuracy who has or will have an SCLC. The reliability of imaging to detect SCLC is analyzed in 100 patients, creating a new screening protocol. For other paraneoplastic syndromes, screening guidelines are updated. SOX and Hu antibodies are also found in 43% of SCLC patients (without LEMS). Combination of different antibodies might lead to reliable screening strategies in high-risk patients.

Computed tomographyHuLambert-Eaton myasthenic syndromeLogistic modellingParaneoplasticPositron emission tomographySmall cell lung cancerSOX
Defense date
2010-11-24
Language
English
Supervisor(s)
Verschuren, J.J.G.M.; Roos, R.A.C.; Wirtz, P.W.
Qualification
Ph.D.
Degree granting institution
Leiden University Medical Center (LUMC), Leiden University, Leiden
Publisher
Department of Neurology, Faculty of Medicine / Leiden University Medical Center (LUMC), Leiden University
hdl
1887/16174
ISBN(s)
9789053353387

Funding

Sponsorship
The studies described in this dissertation were supported in part by a European Neurological Society fellowship, the Leiden University Medical Center, the European Union research project “PNSEuronetwork”, contract number LSSM-CT 2005-518174, another grant from the European Union (QLG1-CT-2002-01756), PI030028 Fondo de Investigaciones Sanitarias, Spain and by the Prinses Beatrix Fonds, the Netherlands. Printing of this thesis was supported financially by the Stichting het Remmert Adriaan Laan Fonds.