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Peripheral neuropathy in mitochondrial encephalomyopathies

C C Chu1, C C Huang, W Fang

  • 1Department of Neurology, Chang Gung Memorial Hospital, Taipei, Taiwan.

European Neurology
|January 1, 1997
PubMed
Summary

Peripheral neuropathy is common in mitochondrial encephalomyopathies, often presenting with sensorimotor symptoms. This condition is primarily caused by axonal degeneration, as confirmed by clinical and pathological studies.

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Area of Science:

  • Neurology
  • Genetics
  • Pathology

Background:

  • Mitochondrial encephalomyopathies are a group of inherited metabolic disorders.
  • Peripheral neuropathy can be a significant clinical manifestation in these disorders.
  • Understanding the underlying mechanisms is crucial for patient management.

Purpose of the Study:

  • To investigate the prevalence and characteristics of peripheral neuropathy in patients with mitochondrial encephalomyopathies.
  • To determine the pathological basis of neuropathy in affected individuals.
  • To correlate clinical findings with electrophysiological and genetic data.

Main Methods:

  • Clinical assessment of neurological symptoms.
  • Electrophysiological studies including nerve conduction studies.

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  • Pathological examination of sural nerve biopsies.
  • Mitochondrial DNA analysis.

    • Main Results:

    • Peripheral neuropathy was observed in seven patients, with varied clinical presentations.
    • Five patients exhibited polyneuropathy with reduced nerve action potential amplitudes, indicative of axonal degeneration.
    • Sural nerve biopsy confirmed axonal degeneration and loss of large myelinated fibers in one patient with MERRF.
    • Mitochondrial DNA analysis revealed an 8344 A-to-G point mutation in the sural nerve of the MERRF patient.

    Conclusions:

    • Peripheral neuropathy is a frequent complication of mitochondrial encephalomyopathies.
    • Axonal degeneration is the predominant pathological finding in neuropathy associated with these conditions.
    • Genetic mutations in mitochondrial DNA are implicated in the pathogenesis of neuropathy.