Presymptomatic electrophysiological tests predict clinical onset and survival in SOD1(G93A) ALS mice

Muscle Nerve. 2014 Dec;50(6):943-9. doi: 10.1002/mus.24237. Epub 2014 Oct 30.

Abstract

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 SOD1(G93A) mice. This is a methodological improvement for preclinical studies.

Keywords: SOD1; amyotrophic lateral sclerosis; biomarkers; electrophysiology; motoneuron disease.

Publication types

  • Evaluation Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / genetics
  • Amyotrophic Lateral Sclerosis / mortality*
  • Amyotrophic Lateral Sclerosis / physiopathology*
  • Animals
  • Disease Models, Animal
  • Disease Progression
  • Electrophysiology
  • Evoked Potentials / physiology
  • Female
  • Male
  • Mice
  • Mice, Mutant Strains
  • Mice, Transgenic
  • Motor Neurons / physiology*
  • Neural Conduction / physiology
  • Predictive Value of Tests
  • Superoxide Dismutase / genetics*
  • Superoxide Dismutase-1
  • Survival Rate

Substances

  • Sod1 protein, mouse
  • Superoxide Dismutase
  • Superoxide Dismutase-1