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Related Concept Videos

Diabetic Neuropathy01:22

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Definition A diabetic foot ulcer (DFU) is a chronic, non-healing wound that develops in individuals with diabetes. It typically occurs on pressure-bearing areas such as the heel, metatarsal heads, or hallux, and carries a high risk of infection and amputation.Pathophysiology • The development of DFUs can be explained by four interconnected mechanisms: neuropathy, ischemia, infection, and impaired wound healing. • Neuropathy is the most common factor. Sensory neuropathy reduces pain perception,...

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Related Experiment Video

Updated: Jun 1, 2026

Establishing a Mouse Model of a Pure Small Fiber Neuropathy with the Ultrapotent Agonist of Transient Receptor Potential Vanilloid Type 1
09:39

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Published on: February 13, 2018

Small-fiber neuropathy in patients with ALS.

J Weis1, I Katona, G Müller-Newen

  • 1Institute of Neuropathology, Medical Faculty, RWTH Aachen University, Pauwelsstrasse 30, 52074 Aachen, Germany. jweis@ukaachen.de

Neurology
|June 8, 2011
PubMed
Summary

Amyotrophic lateral sclerosis (ALS) patients show significantly reduced small sensory nerve fibers in the skin, indicating a distal axonopathy. This small-fiber neuropathy affects nearly 80% of ALS patients, suggesting nerve fiber degeneration is a key part of the disease.

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Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease.
  • The role of peripheral sensory fibers in ALS pathogenesis is not fully understood.

Purpose of the Study:

  • To investigate the involvement of epidermal small sensory fibers in the neurodegenerative process of ALS.
  • To evaluate the density of intraepidermal nerve fibers and the prevalence of small-fiber neuropathy in ALS patients.

Main Methods:

  • Skin biopsies were obtained from 28 ALS patients and 17 age-matched controls.
  • Protein gene product 9.5 (PGP9.5) immunohistochemistry was used to assess epidermal nerve fiber density.
  • Comparison of nerve fiber density and prevalence of small-fiber neuropathy between groups.

Main Results:

  • ALS patients exhibited significantly reduced epidermal nerve fiber density in the distal calf compared to controls (4.8 vs 12.2 fibers/mm²).
  • Small-fiber neuropathy was significantly more prevalent in ALS patients (79%) than in controls (12%).
  • Axonal swellings, suggestive of trafficking defects, were observed more frequently in ALS patients.

Conclusions:

  • Distal epidermal small sensory fibers are involved in the neurodegeneration seen in ALS.
  • Findings support the concept of distal axonopathy as a mechanism in ALS pathogenesis.