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Lambert-Eaton Syndrome

  • Chapter
Myasthenia Gravis and Related Disorders

Part of the book series: Current Clinical Neurology ((CCNEU))

Abstract

The Lambert-Eaton syndrome* (LES) is an autoimmune presynaptic disorder of peripheral cholinergic neurotransmission characterized by proximal myasthenic weakness and mild autonomic dysfunction. Approximately 60% of cases are associated with small cell lung carcinoma (SCLC), and LES can appear before or after the clinical diagnosis of cancer. The P/Q-type voltage-gated calcium channel in motor nerve terminals [classiifed by structure and function as Cav 2.1(1)] is the presumptive target of autoantibodies that mediate LES. This channel is related antigenically to calcium channels expressed on surface membranes of SCLC tumor cells. When the neuronal Cav2.1 channel’s function is impaired, nerve-stimulated quantal release of the neurotransmitter acetylcholine (ACh) is reduced. This lowers the safety margin for synaptic transmission at the neuromuscular junction and in certain autonomic nerve terminals.

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Authors
  1. C. Michel Harper
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  2. Vanda A. Lennon
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Editors and Affiliations

  1. Departments of Neurology and Neurosciences, University Hospitals of Cleveland, Louis Stokes Cleveland Veterans Affairs Medical Center, Case Western Reserve University, Cleveland, OH, USA

    Henry J. Kaminski MD

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Dedicated to Dr. Edward H. Lambert on the Occasion of His 87th Birthday

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Harper, C.M., Lennon, V.A. (2003). Lambert-Eaton Syndrome. In: Kaminski, H.J. (eds) Myasthenia Gravis and Related Disorders. Current Clinical Neurology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-341-5_12

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  • DOI: https://doi.org/10.1007/978-1-59259-341-5_12

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