Early diagnosis of ALS: the search for signs of denervation in clinically normal muscles

J Neurol Sci. 2007 Dec 15;263(1-2):154-7. doi: 10.1016/j.jns.2007.07.008. Epub 2007 Jul 25.

Abstract

Aim and methods: We prospectively investigated whether early diagnosis of amyotrophic lateral sclerosis (ALS) could be facilitated by demonstrating signs of denervation in a muscle of a clinical and electromyographical unaffected region. Muscle fibre conduction velocity (MFCV) was determined in 18 patients in whom the diagnosis ALS was considered but not established beyond a level of clinically possible ALS according to the revised El Escorial criteria. A muscle biopsy was obtained from the same muscle, to demonstrate neurogenic changes. The study followed the guidelines from the STARD initiative.

Results and conclusion: Results were analysed with respect to the final diagnosis. After a mean follow-up of 16 months, 9 patients developed probable or definite ALS. Sensitivity of abnormal MFCV for developing ALS was 89%. Muscle biopsy confirmed that denervation was the cause of abnormal MFCV. We concluded that MFCV can be used to detect denervation in muscles that show no clinical or electromyographical signs of lower motor neuron disease, and thus may contribute to early diagnosis of probable laboratory-supported ALS.

MeSH terms

  • Adult
  • Aged
  • Amyotrophic Lateral Sclerosis / diagnosis*
  • Amyotrophic Lateral Sclerosis / pathology
  • Amyotrophic Lateral Sclerosis / physiopathology
  • Electromyography / methods
  • Female
  • Humans
  • Male
  • Middle Aged
  • Muscle Denervation / methods*
  • Muscle Fibers, Skeletal / physiology
  • Neural Conduction / physiology
  • Predictive Value of Tests
  • Prospective Studies
  • Sensitivity and Specificity