Review of the Diagnosis and Treatment of Periodic Paralysis

Jeffrey M. Statland, Bertrand Fontaine, Michael G. Hanna, Nicholas E. Johnson, John T. Kissel, Valeria A. Sansone, Perry B. Shieh, Rabi N. Tawil, Jaya Trivedi, Stephen C. Cannon, Robert C. Griggs

Research output: Contribution to journalReview article

22 Scopus citations

Abstract

Periodic paralyses (PPs) are rare neuromuscular disorders caused by mutations in skeletal muscle sodium, calcium, and potassium channel genes. PPs include hypokalemic paralysis, hyperkalemic paralysis, and Andersen-Tawil syndrome. Common features of PP include autosomal dominant inheritance, onset typically in the first or second decades, episodic attacks of flaccid weakness, which are often triggered by diet or rest after exercise. Diagnosis is based on the characteristic clinic presentation then confirmed by genetic testing. In the absence of an identified genetic mutation, documented low or high potassium levels during attacks or a decrement on long exercise testing support diagnosis. The treatment approach should include both management of acute attacks and prevention of attacks. Treatments include behavioral interventions directed at avoidance of triggers, modification of potassium levels, diuretics, and carbonic anhydrase inhibitors. Muscle Nerve 57: 522–530, 2018.

Original languageEnglish (US)
Pages (from-to)522-530
Number of pages9
JournalMuscle and Nerve
Volume57
Issue number4
DOIs
StatePublished - Apr 2018

Keywords

  • Andersen-Tawil syndrome
  • acetazolamide
  • channelopathies
  • dichlorphenamide
  • periodic paralyses
  • review
  • treatment

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

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  • Cite this

    Statland, J. M., Fontaine, B., Hanna, M. G., Johnson, N. E., Kissel, J. T., Sansone, V. A., Shieh, P. B., Tawil, R. N., Trivedi, J., Cannon, S. C., & Griggs, R. C. (2018). Review of the Diagnosis and Treatment of Periodic Paralysis. Muscle and Nerve, 57(4), 522-530. https://doi.org/10.1002/mus.26009