Statland, JM;
Fontaine, B;
Hanna, MG;
Johnson, NE;
Kissel, JT;
Sansone, VA;
Shieh, PB;
... Griggs, RC; + view all
(2018)
Review of the Diagnosis and Treatment of Periodic Paralysis.
Muscle and Nerve
, 57
(4)
pp. 522-530.
10.1002/mus.26009.
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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, 2017.
| Type: | Article |
|---|---|
| Title: | Review of the Diagnosis and Treatment of Periodic Paralysis |
| Location: | United States |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1002/mus.26009 |
| Publisher version: | http://doi.org/10.1002/mus.26009 |
| Language: | English |
| Additional information: | © 2017 The Authors Muscle & Nerve Published by Wiley Periodicals, Inc. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. |
| Keywords: | Andersen-Tawil syndrome, acetazolamide, channelopathies, dichlorphenamide, periodic paralyses, review, treatment |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10044958 |
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