Presymptomatic electrophysiological tests predict clinical onset and survival in SOD1G93A ALS mice

Renzo Mancuso, Rosario Osta, Xavier Navarro

Research output: Contribution to journalArticleResearchpeer-review

10 Citations (Scopus)

Abstract

© 2014 Wiley Periodicals, Inc. Introduction: We assessed the predictive value of electrophysiological tests as a marker of clinical disease onset and survival in superoxide-dismutase 1 (SOD1)G93A mice. Methods: We evaluated the accuracy of electrophysiological tests in differentiating transgenic versus wild-type mice. We made a correlation analysis of electrophysiological parameters and the onset of symptoms, survival, and number of spinal motoneurons. Results: Presymptomatic electrophysiological tests show great accuracy in differentiating transgenic versus wild-type mice, with the most sensitive parameter being the tibialis anterior compound muscle action potential (CMAP) amplitude. The CMAP amplitude at age 10 weeks correlated significantly with clinical disease onset and survival. Electrophysiological tests increased their survival prediction accuracy when evaluated at later stages of the disease and also predicted the amount of lumbar spinal motoneuron preservation. Conclusions: Electrophysiological tests predict clinical disease onset, survival, and spinal motoneuron preservation in SOD1G93A mice. This is a methodological improvement for preclinical studies.
Original languageEnglish
Pages (from-to)943-949
JournalMuscle and Nerve
Volume50
DOIs
Publication statusPublished - 1 Jan 2014

Keywords

  • Amyotrophic lateral sclerosis
  • Biomarkers
  • Electrophysiology
  • Motoneuron disease
  • SOD1

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