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Related Experiment Videos

Oculoskeletal myopathy with abnormal mitochondria.

V Bril, N B Rewcastle, J Humphrey

    The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques
    |August 1, 1984
    PubMed
    Summary

    Oculoskeletal myopathy involves eye muscle paralysis and mild weakness, with abnormal mitochondria found in muscle biopsies. This condition is distinct from Kearns-Sayre syndrome and carries a lower risk of sudden cardiac death.

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    Area of Science:

    • Neurology
    • Mitochondrial Diseases
    • Ophthalmology

    Background:

    • Oculoskeletal myopathy is characterized by ophthalmoplegia and skeletal muscle weakness.
    • Abnormal mitochondria are implicated in the pathophysiology of certain myopathies.

    Purpose of the Study:

    • To review the clinical, electrophysiological, and pathological features of oculoskeletal myopathy.
    • To differentiate this condition from Kearns-Sayre syndrome.

    Main Methods:

    • Review of 14 patients with oculoskeletal myopathy.
    • Clinical assessment, electromyography, and skeletal muscle biopsy with electron microscopy.

    Main Results:

    • Patients presented with ophthalmoplegia and mild, slowly progressive skeletal muscle weakness.

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  • Electromyography showed myopathic changes; serum enzymes were normal.
  • Skeletal muscle biopsy revealed abnormal mitochondria with crystalloid inclusions.

    • Conclusions:

    • Oculoskeletal myopathy with abnormal mitochondria represents a distinct clinical entity.
    • This condition has a significantly lower risk of sudden cardiac death compared to Kearns-Sayre syndrome.