Progressive myopathy in hyperkalemic periodic paralysis.
Arch Neurol
; 47(9): 1013-7, 1990 Sep.
Article
em En
| MEDLINE
| ID: mdl-2396930
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Paralisias Periódicas Familiares
/
Doenças Musculares
Limite:
Adolescent
/
Adult
/
Female
/
Humans
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Male
/
Middle aged
Idioma:
En
Ano de publicação:
1990
Tipo de documento:
Article