Decremental motor responses to repetitive nerve stimulation in ALS

James M. Killian, Angus A. Wilfong, Leanne Burnett, Stanley H. Appel, Dennis Boland

Research output: Contribution to journalArticlepeer-review

61 Scopus citations

Abstract

Repetitive nerve stimulation (RNS) of the trapezius muscle at slow rates was performed on 192 patients with amyotrophic lateral sclerosis (ALS). Fifty-six patients (29%) showed classical neuromuscular decrement of 10-43% (mean 16.8%) while 44 patients (23%) had a borderline decrement of 5-9%. The trapezius was significantly more sensitive in revealing the defect than the distal hypothenar muscles. In 30 patients followed serially, the decremental response remained constant or increased with time. However, 25% of patients continued to show no decrement in spite of progression of disease. No statistical correlation was found between decrement and clinical severity, disease staging, or disease progression. The finding that at least 50% of ALS patients show some degree of decrement on RNS of the trapezius muscle suggests that functional alterations of the neuromuscular junction accompany this disease.

Original languageEnglish (US)
Pages (from-to)747-754
Number of pages8
JournalMuscle & Nerve
Volume17
Issue number7
DOIs
StatePublished - Jan 1 1994

Keywords

  • amyotrophic lateral sclerosis
  • decremental motor responses
  • repetitive nerve stimulation

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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