Quantitative sensory testing and structural assessment of sensory nerve fibres in amyotrophic lateral sclerosis

J Neurol Sci. 2017 Feb 15:373:329-334. doi: 10.1016/j.jns.2017.01.005. Epub 2017 Jan 6.

Abstract

Objective: In this prospective study, involvement of sensory nerve fibres in ALS patients was assessed using functional and structural measures in the form of quantitative sensory testing (QST) and skin and nerve biopsies.

Methods: Thirty-two ALS patients and 32 healthy subjects were evaluated with a QST battery comprising thresholds of mechanical detection, mechanical pain, vibration detection, cold detection, warm detection, heat pain, and pinprick sensation. Skin biopsies were evaluated in 31 ALS patients by intraepidermal nerve fibre density (IENFD) and axonal swelling ratios, and growth-associated protein 43 (GAP-43) antibody staining. Sural nerve biopsies were evaluated using teased fibre analysis in eight patients.

Results: Mean values for QST parameters and IENFD in ALS patients were within normal range. However, the patients had increased axonal swelling ratios and GAP-43 antibody staining was negative in all patients.

Conclusions: Although QST and IENFD were affected in only a small subset of ALS patients, the axonal swellings observed in all patients indicate that the affection is more frequent, and suggests that IENFD count may not be sufficient. The negative GAP-43 staining suggested an insufficiency of regeneration in small sensory nerve fibres.

Keywords: Amyotrophic lateral sclerosis (ALS); Axonal swelling; Nerve biopsy; Quantitative sensory testing (QST); Skin biopsy.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / diagnosis
  • Amyotrophic Lateral Sclerosis / pathology*
  • Amyotrophic Lateral Sclerosis / physiopathology*
  • Axons / pathology
  • Axons / physiology
  • Biopsy
  • Cold Temperature
  • Female
  • GAP-43 Protein / metabolism
  • Hot Temperature
  • Humans
  • Male
  • Middle Aged
  • Neurologic Examination / methods
  • Pain / pathology
  • Pain / physiopathology
  • Pain Threshold / physiology
  • Prospective Studies
  • Sensory Receptor Cells / pathology*
  • Sensory Receptor Cells / physiology*
  • Sensory Thresholds / physiology*
  • Skin / innervation
  • Skin / pathology
  • Skin / physiopathology
  • Sural Nerve / pathology
  • Sural Nerve / physiopathology
  • Vibration

Substances

  • GAP-43 Protein