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Stimulated single-fiber electromyography (sSFEMG) in Lambert-Eaton syndrome

OBJECTIVE: To report the clinical features and the neurophysiological approach of a patient with Lambert-Eaton myasthenic syndrome (LEMS), highlighting the diagnostic role of the stimulated single fiber electromyography (sSFEMG). CASE REPORT: A 60-year-old woman presenting with the LEMS triad (proxi...

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Autores principales: Todisco, Vincenzo, Cirillo, Giovanni, Capuano, Rocco, d'Ambrosio, Alessandro, Tedeschi, Gioacchino, Gallo, Antonio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6134173/
https://www.ncbi.nlm.nih.gov/pubmed/30215026
http://dx.doi.org/10.1016/j.cnp.2018.07.001
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author Todisco, Vincenzo
Cirillo, Giovanni
Capuano, Rocco
d'Ambrosio, Alessandro
Tedeschi, Gioacchino
Gallo, Antonio
author_facet Todisco, Vincenzo
Cirillo, Giovanni
Capuano, Rocco
d'Ambrosio, Alessandro
Tedeschi, Gioacchino
Gallo, Antonio
author_sort Todisco, Vincenzo
collection PubMed
description OBJECTIVE: To report the clinical features and the neurophysiological approach of a patient with Lambert-Eaton myasthenic syndrome (LEMS), highlighting the diagnostic role of the stimulated single fiber electromyography (sSFEMG). CASE REPORT: A 60-year-old woman presenting with the LEMS triad (proximal and axial weakness, autonomic dysfunction and areflexia) was evaluated by neurophysiological tests (electroneuromyography, repetitive stimulation test (TSR), voluntary and stimulated SFEMG). We reported: 1) increase of compound muscle action potential (CMAP) amplitude (>60%) following brief isometric exercise compared to the rest (baseline); 2) decremental/incremental response of CMAP amplitude at low- (3 Hz) and high-frequency (30 Hz) repetitive stimulation test (RST), respectively; 3) increased neuromuscular jitter and blocking at voluntary single-fiber electromyography (vSFEMG); 4) stimulation rate-dependent reduction of the neuromuscular jitter and blocking at sSFEMG. Diagnosis was confirmed by serological demonstration of circulating voltage gated calcium channels (VGCC) antibodies. SIGNIFICANCE: The present case highlights the role of the sSFEMG in the diagnosis of LEMS, underling the stimulation rate-dependency of both neuromuscular jitter and blocks.
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spelling pubmed-61341732018-09-13 Stimulated single-fiber electromyography (sSFEMG) in Lambert-Eaton syndrome Todisco, Vincenzo Cirillo, Giovanni Capuano, Rocco d'Ambrosio, Alessandro Tedeschi, Gioacchino Gallo, Antonio Clin Neurophysiol Pract Clinical and Research Article OBJECTIVE: To report the clinical features and the neurophysiological approach of a patient with Lambert-Eaton myasthenic syndrome (LEMS), highlighting the diagnostic role of the stimulated single fiber electromyography (sSFEMG). CASE REPORT: A 60-year-old woman presenting with the LEMS triad (proximal and axial weakness, autonomic dysfunction and areflexia) was evaluated by neurophysiological tests (electroneuromyography, repetitive stimulation test (TSR), voluntary and stimulated SFEMG). We reported: 1) increase of compound muscle action potential (CMAP) amplitude (>60%) following brief isometric exercise compared to the rest (baseline); 2) decremental/incremental response of CMAP amplitude at low- (3 Hz) and high-frequency (30 Hz) repetitive stimulation test (RST), respectively; 3) increased neuromuscular jitter and blocking at voluntary single-fiber electromyography (vSFEMG); 4) stimulation rate-dependent reduction of the neuromuscular jitter and blocking at sSFEMG. Diagnosis was confirmed by serological demonstration of circulating voltage gated calcium channels (VGCC) antibodies. SIGNIFICANCE: The present case highlights the role of the sSFEMG in the diagnosis of LEMS, underling the stimulation rate-dependency of both neuromuscular jitter and blocks. Elsevier 2018-08-13 /pmc/articles/PMC6134173/ /pubmed/30215026 http://dx.doi.org/10.1016/j.cnp.2018.07.001 Text en © 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Clinical and Research Article
Todisco, Vincenzo
Cirillo, Giovanni
Capuano, Rocco
d'Ambrosio, Alessandro
Tedeschi, Gioacchino
Gallo, Antonio
Stimulated single-fiber electromyography (sSFEMG) in Lambert-Eaton syndrome
title Stimulated single-fiber electromyography (sSFEMG) in Lambert-Eaton syndrome
title_full Stimulated single-fiber electromyography (sSFEMG) in Lambert-Eaton syndrome
title_fullStr Stimulated single-fiber electromyography (sSFEMG) in Lambert-Eaton syndrome
title_full_unstemmed Stimulated single-fiber electromyography (sSFEMG) in Lambert-Eaton syndrome
title_short Stimulated single-fiber electromyography (sSFEMG) in Lambert-Eaton syndrome
title_sort stimulated single-fiber electromyography (ssfemg) in lambert-eaton syndrome
topic Clinical and Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6134173/
https://www.ncbi.nlm.nih.gov/pubmed/30215026
http://dx.doi.org/10.1016/j.cnp.2018.07.001
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