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Peripheral neuropathy associated with mitochondrial myopathy.

C Yiannikas, J G McLeod, J D Pollard

    Annals of Neurology
    |August 1, 1986
    PubMed
    Summary

    Peripheral neuropathy is common in mitochondrial myopathy patients, with half showing abnormal nerve conduction studies. Biopsies revealed nerve fiber damage and abnormal mitochondria, indicating a significant neurological component to this condition.

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    Area of Science:

    • Neurology
    • Genetics
    • Cell Biology

    Background:

    • Mitochondrial myopathies are a group of inherited disorders affecting muscle energy production.
    • Peripheral neuropathy can be a co-occurring condition, but its prevalence and characteristics in mitochondrial myopathy are not fully understood.

    Purpose of the Study:

    • To investigate the presence and characteristics of peripheral neuropathy in patients diagnosed with mitochondrial myopathy.
    • To correlate clinical, electrophysiological, and histopathological findings of neuropathy in this patient cohort.

    Main Methods:

    • Clinical neurological examination of 20 mitochondrial myopathy patients.
    • Nerve conduction studies (NCS) and electromyography (EMG) to assess peripheral nerve function.
    • Sural nerve biopsy with morphometric analysis and electron microscopy for 4 patients with clinical neuropathy.

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    Main Results:

    • Clinical neuropathy was observed in 25% (5/20) of patients, presenting as mild sensorimotor neuropathy.
    • Electrophysiological studies revealed abnormalities in 50% (10/20) of patients, with significant impairment in motor and sensory conduction compared to controls.
    • Nerve biopsies showed reduced myelinated fiber density, axonal degeneration, and abnormal mitochondria with paracrystalline inclusions in Schwann cells.

    Conclusions:

    • Peripheral neuropathy is a frequent complication of mitochondrial myopathy, affecting both nerve structure and function.
    • Findings suggest that axonal degeneration and mitochondrial abnormalities within Schwann cells contribute to neuropathy in these patients.
    • Further research is warranted to elucidate the specific mechanisms and implications of neuropathy in mitochondrial myopathy.