Clinical features and diagnosis of Lambert-Eaton myasthenic syndrome
- David H Weinberg, MD
David H Weinberg, MD
- Associate Professor of Neurology
- Tufts University School of Medicine
- Section Editors
- Lisa M DeAngelis, MD, FAAN, FANA
Lisa M DeAngelis, MD, FAAN, FANA
- Section Editor — Neurooncology
- Chair, Department of Neurology
- Lillian Rojtman Berkman Chair in Honor of Jerome B. Posner
- Memorial Sloan Kettering Cancer Center
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
Lambert-Eaton myasthenic syndrome (LEMS) is an uncommon disorder of neuromuscular junction transmission with the primary clinical manifestation of muscle weakness. Knowledge of subtle clinical features and laboratory abnormalities that accompany LEMS permits the early identification of the disorder. Early recognition is particularly important because of its strong association with small cell lung cancer (SCLC). Although LEMS can occur at any point in the course of SCLC, it may serve as a marker for early disease.
This topic will review the pathophysiology, clinical features, and diagnosis of LEMS. Treatment of LEMS is discussed separately. (See "Treatment of Lambert-Eaton myasthenic syndrome".)
Lambert and Elmqvist, through a series of elegant intracellular muscle recordings, found that patients with what is now called Lambert-Eaton myasthenic syndrome (LEMS) had the following unique features [1,2]:
●Normal miniature endplate potential amplitude, demonstrating normal postsynaptic sensitivity to acetylcholine (ACh)
●Markedly reduced evoked endplate potential amplitude, suggesting a significant reduction in ACh release
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