Lambert–Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies

Summary Lambert–Eaton myasthenic syndrome (LEMS) is a neuromuscular autoimmune disease that has served as a model for autoimmunity and tumour immunology. In LEMS, the characteristic muscle weakness is thought to be caused by pathogenic autoantibodies directed against voltage-gated calcium channels (...

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Bibliographic Details
Published in:Lancet neurology 2011-12, Vol.10 (12), p.1098-1107
Main Authors: Titulaer, Maarten J, Dr, Lang, Bethan, PhD, Verschuuren, Jan JGM, Prof
Format: Article
Language:English
Subjects:
4-Aminopyridine - analogs & derivatives
4-Aminopyridine - therapeutic use
Autoantibodies
Autoantibodies - immunology
Autoimmune diseases
Calcium channels (voltage-gated)
Calcium Channels - immunology
Cancer
Carcinoma, Small Cell - complications
Carcinoma, Small Cell - immunology
Central nervous system
Disease
Epidemiology
Haplotypes
Humans
Lambert-Eaton myasthenic syndrome
Lambert-Eaton Myasthenic Syndrome - complications
Lambert-Eaton Myasthenic Syndrome - diagnosis
Lambert-Eaton Myasthenic Syndrome - drug therapy
Lambert-Eaton Myasthenic Syndrome - immunology
Lung carcinoma
Lung Neoplasms - complications
Lung Neoplasms - immunology
Muscles
Nerve endings
Neurological diseases
Neurology
Neuromuscular junctions
Patients
Prostate
Tumors
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Summary:Summary Lambert–Eaton myasthenic syndrome (LEMS) is a neuromuscular autoimmune disease that has served as a model for autoimmunity and tumour immunology. In LEMS, the characteristic muscle weakness is thought to be caused by pathogenic autoantibodies directed against voltage-gated calcium channels (VGCC) present on the presynaptic nerve terminal. Half of patients with LEMS have an associated tumour, small-cell lung carcinoma (SCLC), which also expresses functional VGCC. Knowledge of this association led to the discovery of a wide range of paraneoplastic and non-tumour-related neurological disorders of the peripheral and central nervous systems. Detailed clinical studies have improved our diagnostic skills and knowledge of the pathophysiological mechanisms and association of LEMS with SCLC, and have helped with the development of a protocol for early tumour detection.
ISSN:1474-4422
1474-4465
DOI:10.1016/S1474-4422(11)70245-9