Quantifying disease progression in amyotrophic lateral sclerosis using peripheral nerve sonography

Muscle Nerve. 2016 Sep;54(3):391-7. doi: 10.1002/mus.25066. Epub 2016 Jun 9.

Abstract

Introduction: In this study we investigated whether peripheral nerve sonography could be used as a biomarker to monitor disease progression in amyotrophic lateral sclerosis (ALS).

Methods: In 37 patients, ulnar and median nerve cross-sectional area (CSA) was determined in at least 2 ultrasound sessions; mean follow-up was 14.5 months. Linear mixed-effects models were conducted to analyze time effects on CSA.

Results: Ulnar nerve CSA declined significantly at a monthly rate of -0.04 mm(2) (forearm) and -0.05 mm(2) (wrist); the decrease was more pronounced when baseline CSA was greater. To detect a 50% treatment effect on ulnar nerve CSA, 332 patients would need to be entered in a hypothetical randomized, controlled clinical trial. Time had no significant impact on median nerve CSA.

Conclusions: Distal ulnar nerve ultrasound may be a useful biomarker to monitor disease progression in ALS, especially as hypothetical treatment effects on CSA seem to be detectable in manageable cohort sizes. Muscle Nerve 54: 391-397, 2016.

Keywords: amyotrophic lateral sclerosis; biomarker; cross-sectional area; median nerve; ulnar nerve; ultrasound.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Amyotrophic Lateral Sclerosis* / diagnostic imaging
  • Amyotrophic Lateral Sclerosis* / pathology
  • Amyotrophic Lateral Sclerosis* / physiopathology
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Forearm / innervation
  • Humans
  • Male
  • Middle Aged
  • Neural Conduction / physiology
  • Peripheral Nerves / diagnostic imaging*
  • Peripheral Nerves / physiopathology*
  • Ultrasonography / methods*
  • Wrist / innervation