Literature DB >> 1645844

Single motor end-plates in myasthenia gravis and LEMS at different firing rates.

J V Trontelj1, E Stålberg.   

Abstract

The jitter and frequency of blocking was studied at single motor end-plates in 10 patients with myasthenia gravis (MG) and in a patient with Lambert-Eaton myasthenic syndrome (LEMS), using single fiber EMG (SFEMG) with axonal microstimulation at rates varying from 0.5 Hz to 20 Hz. While some myasthenic motor end-plates showed lowest degrees of transmission disturbance at the lowest rates and most pronounced abnormality at the highest rates of stimulation, over one-half were most abnormal at intermediate rates and improved at higher rates. In 1 patient, all end-plates behaved in this way. On the other hand, all end-plates in the LEMS patient showed the expected improvement of the abnormal jitter and blocking on increasing the stimulation rate. It is argued that improvement of jitter and blocking at higher rates, unless dramatic, does not necessarily suggest a presynaptic abnormality.

Entities:  

Mesh:

Year:  1991        PMID: 1645844     DOI: 10.1002/mus.880140305

Source DB:  PubMed          Journal:  Muscle Nerve        ISSN: 0148-639X            Impact factor:   3.217


  4 in total

1.  Impaired neuromuscular transmission and skeletal muscle fiber necrosis in mice lacking Na/Ca exchanger 3.

Authors:  Sophie Sokolow; Mario Manto; Philippe Gailly; Jordi Molgó; Clarisse Vandebrouck; Jean-Marie Vanderwinden; Andre Herchuelz; Stéphane Schurmans
Journal:  J Clin Invest       Date:  2004-01       Impact factor: 14.808

2.  In vivo impact of presynaptic calcium channel dysfunction on motor axons in episodic ataxia type 2.

Authors:  Susan E Tomlinson; S Veronica Tan; David Burke; Robyn W Labrum; Andrea Haworth; Vaneesha S Gibbons; Mary G Sweeney; Robert C Griggs; Dimitri M Kullmann; Hugh Bostock; Michael G Hanna
Journal:  Brain       Date:  2016-02       Impact factor: 13.501

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

Authors:  Vincenzo Todisco; Giovanni Cirillo; Rocco Capuano; Alessandro d'Ambrosio; Gioacchino Tedeschi; Antonio Gallo
Journal:  Clin Neurophysiol Pract       Date:  2018-08-13

Review 4.  Lambert-Eaton Myasthenic syndrome: early diagnosis is key.

Authors:  Trajche Ivanovski; Francesc Miralles
Journal:  Degener Neurol Neuromuscul Dis       Date:  2019-05-13
  4 in total

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