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 language | English |
|---|---|
| Pages (from-to) | 1013-1017 |
| Number of pages | 5 |
| Journal | Archives of Neurology |
| Volume | 47 |
| Issue number | 9 |
| State | Published - Jan 1 1990 |
| Externally published | Yes |
Fingerprint
ASJC Scopus subject areas
- Neuroscience(all)
Cite this
Progressive myopathy in hyperkalemic periodic paralysis. / Bradley, Walter G; Taylor, R.; Rice, D. R.; Hausmanowa-Petruzewicz, I.; Adelman, L. S.; Jenkinson, M.; Jedrzejowska, H.; Drac, H.; Pendlebury, W. W.
In: Archives of Neurology, Vol. 47, No. 9, 01.01.1990, p. 1013-1017.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Progressive myopathy in hyperkalemic periodic paralysis
AU - Bradley, Walter G
AU - Taylor, R.
AU - Rice, D. R.
AU - Hausmanowa-Petruzewicz, I.
AU - Adelman, L. S.
AU - Jenkinson, M.
AU - Jedrzejowska, H.
AU - Drac, H.
AU - Pendlebury, W. W.
PY - 1990/1/1
Y1 - 1990/1/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0025062007&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0025062007&partnerID=8YFLogxK
M3 - Article
C2 - 2396930
AN - SCOPUS:0025062007
VL - 47
SP - 1013
EP - 1017
JO - Archives of Neurology
JF - Archives of Neurology
SN - 0003-9942
IS - 9
ER -