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Progressive myopathy in hyperkalemic periodic paralysis.

W G Bradley1, R Taylor, D R Rice

  • 1Department of Neurology, University of Vermont School of Medicine, Medical Center Hospital, Burlington 05405.

Archives of Neurology
|September 1, 1990
PubMed
Summary

Progressive myopathy is common in hyperkalemic periodic paralysis, similar to hypokalemic periodic paralysis. This study found significant muscle degeneration in patients with hyperkalemic periodic paralysis.

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Area of Science:

  • Neurology
  • Muscle Diseases

Background:

  • Progressive myopathy is recognized in hypokalemic periodic paralysis.
  • It is less recognized in hyperkalemic periodic paralysis, despite shared genetic links.

Purpose of the Study:

  • To investigate the prevalence and characteristics of progressive myopathy in hyperkalemic periodic paralysis.
  • To compare the myopathy in hyperkalemic periodic paralysis to that seen in hypokalemic periodic paralysis.

Main Methods:

  • Studied four families with hyperkalemic periodic paralysis.
  • Analyzed clinical data, including paralysis duration and frequency.
  • Examined muscle biopsy specimens using light and electron microscopy.

Main Results:

  • Progressive myopathy developed in some family members, with prolonged paralysis episodes.

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  • Myopathy onset often coincided with decreased paralysis attacks.
  • Biopsies revealed fiber size variability, internal nuclei, vacuoles, myofibrillary degeneration, and tubular aggregates.
  • Abnormal biopsies were more frequent in older patients.

    • Conclusions:

    • Progressive myopathy is as common in hyperkalemic periodic paralysis as in hypokalemic periodic paralysis.
    • Muscle degeneration is a significant feature of hyperkalemic periodic paralysis.
    • Age is a factor in the manifestation of myopathy in these patients.