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Related Experiment Videos

Neuropathy and mitochondrial myopathy.

J M Peyronnard, L Charron, A Bellavance

    Annals of Neurology
    |March 1, 1980
    PubMed
    Summary

    Peripheral neuropathy is linked to ophthalmoplegia-plus syndrome. This study details a case with severe sensorimotor neuropathy, showing nerve fiber abnormalities and altered mitochondria in Schwann cells.

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    Area of Science:

    • Neurology
    • Pathology

    Background:

    • Peripheral neuropathy can be a feature of ophthalmoplegia-plus syndrome.
    • Only one prior case of hypertrophic neuropathy in this context has been reported.

    Observation:

    • A 37-year-old man presented with progressive external ophthalmoplegia and ragged-red fiber myopathy.
    • He also exhibited a severe sensorimotor neuropathy.

    Findings:

    • Sural nerve biopsy showed significant loss of large myelinated fibers and axonal degeneration.
    • Residual nerve fibers had thin myelin sheaths relative to axon diameter.
    • Elevated mitochondria in Schwann cells, unlike muscle findings, suggest altered mitochondrial function.

    Implications:

    • Mitochondrial dysfunction may affect both muscle and nerve cells in this syndrome.
    • This could explain the observed axon loss and abnormal myelination in peripheral nerves.

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