Quantifying disease progression in amyotrophic lateral sclerosis using peripheral nerve sonography

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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 mm2 (forearm) and –0.05 mm2 (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
Funding Information
  • Foundation of Medical Research, Frankfurt/Main, Germany
  • German Research Foundation (SCHR 1418/3-1)

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