Neuromuscular transmission in amyotrophic lateral sclerosis

Ricardo A Maselli, R. L. Wollman, C. Leung, B. Distad, S. Palombi, David P Richman, E. F. Salazar-Grueso, R. P. Roos

Research output: Contribution to journalArticle

96 Scopus citations

Abstract

The functional and structural characteristics of the neuromuscular junction were studied in anconeus muscle biopsies of 10 patients with amyotrophic lateral sclerosis (ALS). Intracellular recordings revealed decreased amplitudes of miniature endplate potentials (MEPPs). The MEPP frequencies were highly variable in ALS patients but the average MEPP frequency was not different from that of control patients. The mean quantal content of endplate potentials (m), the mean quanta available for immediate release (n), and the mean quantal stores (N) were all decreased. In contrast, the mean probability of quantal release (p) was normal and the mean probability of quantal store release (P) was surprisingly high at the majority of ALS endplates. Histologic evidence of denervation and small or absent nerve terminals were observed in all ALS patients. These functional and structural abnormalities of the neuromuscular junction may explain the fatigability and the electromyographic evidence of impaired neuromuscular transmission often encountered in ALS patients.

Original languageEnglish (US)
Pages (from-to)1193-1203
Number of pages11
JournalMuscle and Nerve
Volume16
Issue number11
DOIs
StatePublished - 1993

Keywords

  • amyotrophic lateral sclerosis
  • MEPPs
  • motor nerve terminals

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Fingerprint Dive into the research topics of 'Neuromuscular transmission in amyotrophic lateral sclerosis'. Together they form a unique fingerprint.

  • Cite this

    Maselli, R. A., Wollman, R. L., Leung, C., Distad, B., Palombi, S., Richman, D. P., Salazar-Grueso, E. F., & Roos, R. P. (1993). Neuromuscular transmission in amyotrophic lateral sclerosis. Muscle and Nerve, 16(11), 1193-1203. https://doi.org/10.1002/mus.880161109