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Related Experiment Videos

Peripheral neuropathy in Leigh's disease.

J M Jacobs1, B N Harding, B D Lake

  • 1Institute of Neurology, National Hospital, Queen Square, London.

Brain : a Journal of Neurology
|April 1, 1990
PubMed
Summary

Leigh's disease in children can cause peripheral neuropathy, characterized by thin myelin sheaths in sural nerves. This suggests hypomyelination, potentially leading to demyelination in affected nerves.

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Area of Science:

  • Neuroscience
  • Neuropathology
  • Pediatric Neurology

Background:

  • Leigh's disease is a severe genetic neurometabolic disorder affecting infants and children.
  • Peripheral neuropathy is a known, though less studied, complication in Leigh's disease.
  • Electrophysiological studies indicated peripheral neuropathy in the studied cases.

Observation:

  • Sural nerve biopsies were performed on three children diagnosed with Leigh's disease.
  • Two cases were confirmed postmortem, and one had characteristic CT scan findings.
  • Microscopic examination revealed normal myelinated fiber densities without degeneration or regeneration.

Findings:

  • Quantitative analysis confirmed thin myelin sheaths in the sural nerves.
  • While two cases showed no demyelination, one case exhibited some degree of demyelination.
  • The primary finding suggests hypomyelination of peripheral nerves, possibly progressing to demyelination.

Implications:

  • This study highlights a specific peripheral nerve abnormality in Leigh's disease.
  • The findings suggest that hypomyelination is a key feature, potentially preceding demyelination.
  • Understanding these neuropathological changes can aid in diagnosing and managing peripheral neuropathy in Leigh's disease.

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