Progressive Myopathy in Hyperkalemic Periodic Paralysis

Walter G. Bradley, Reid Taylor, David R. Rice, Irena Hausmanowa Petruzewicz, Lester S. Adelman, Margaret Jenkison, Hanna Jedrzejowska, H. Drac, William W. Pendlebury

Research output: Contribution to journalArticle

61 Scopus citations

Abstract

A progressive degenerative myopathy has been well described in hypokalemic periodic paralysis but is not as widely recognized in hyperkalemic periodic paralysis. We studied four families with the latter disease in which some members developed a progressive myopathy. Episodes of paralysis were prolonged, lasting for months in some cases, and in one case paralysis was sufficiently severe to require ventilatory support. The progressive myopathy tended to develop at a time when attacks of paralysis were decreasing in frequency. Muscle biopsy specimens showed variability in fiber size, internal nuclei, and fibers with vacuoles. Electron microscopy showed myofibrillary degeneration and tubular aggregates. An abnormal biopsy specimen was more common in older patients. Our experience suggests that a progressive myopathy is as common in hyperkalemic periodic paralysis as it is in the hypokalemic disorder.

Original languageEnglish (US)
Pages (from-to)1013-1017
Number of pages5
JournalArchives of neurology
Volume47
Issue number9
DOIs
StatePublished - Sep 1990
Externally publishedYes

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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    Bradley, W. G., Taylor, R., Rice, D. R., Hausmanowa Petruzewicz, I., Adelman, L. S., Jenkison, M., Jedrzejowska, H., Drac, H., & Pendlebury, W. W. (1990). Progressive Myopathy in Hyperkalemic Periodic Paralysis. Archives of neurology, 47(9), 1013-1017. https://doi.org/10.1001/archneur.1990.00530090091018