Skip to main content
Top
Published in: Journal of Translational Medicine 1/2008

Open Access 01-12-2008 | Commentary

Practical aspects in the management of hypokalemic periodic paralysis

Author: Jacob O Levitt

Published in: Journal of Translational Medicine | Issue 1/2008

Login to get access

Abstract

Management considerations in hypokalemic periodic paralysis include accurate diagnosis, potassium dosage for acute attacks, choice of diuretic for prophylaxis, identification of triggers, creating a safe physical environment, peri-operative measures, and issues in pregnancy. A positive genetic test in the context of symptoms is the gold standard for diagnosis. Potassium chloride is the favored potassium salt given at 0.5–1.0 mEq/kg for acute attacks. The oral route is favored, but if necessary, a mannitol solvent can be used for intravenous administration. Avoidance of or potassium prophylaxis for common triggers, such as rest after exercise, high carbohydrate meals, and sodium, can prevent attacks. Chronically, acetazolamide, dichlorphenamide, or potassium-sparing diuretics decrease attack frequency and severity but are of little value acutely. Potassium, water, and a telephone should always be at a patient's bedside, regardless of the presence of weakness. Perioperatively, the patient's clinical status should be checked frequently. Firm data on the management of periodic paralysis during pregnancy is lacking. Patient support can be found at http://​www.​periodicparalysi​s.​org.
Literature
1.
go back to reference Lehmann-Horn F, Rudel R, Jurkat-Rott K: Non-dystrophic myotonias and periodic paralyses. Myology. Edited by: Engel AG. 2004, New York: McGraw-Hill, 1257-1300. 3 Lehmann-Horn F, Rudel R, Jurkat-Rott K: Non-dystrophic myotonias and periodic paralyses. Myology. Edited by: Engel AG. 2004, New York: McGraw-Hill, 1257-1300. 3
2.
go back to reference Venance SL, Jurkat-Rott K, Lehmann-Horn F, Tawil R: SCN4A-associated hypokalemic periodic paralysis merits a trial of acetazolamide. Neurology. 2004, 63 (10): 1977-CrossRefPubMed Venance SL, Jurkat-Rott K, Lehmann-Horn F, Tawil R: SCN4A-associated hypokalemic periodic paralysis merits a trial of acetazolamide. Neurology. 2004, 63 (10): 1977-CrossRefPubMed
3.
go back to reference Editorial Staff: Potassium Supplements (Systemic). USP DI® Drug Information for the Health Care Professional. Edited by: Klasco RK. 2006, Greenwood Village, Colorodo: Thomson Micromedex, 2483-90. 26 Editorial Staff: Potassium Supplements (Systemic). USP DI® Drug Information for the Health Care Professional. Edited by: Klasco RK. 2006, Greenwood Village, Colorodo: Thomson Micromedex, 2483-90. 26
4.
go back to reference Rose BD: Clinical physiology of acid-base and electrolyte disorders. 1994, New York: McGraw-Hill, Inc, 808-812. 4 Rose BD: Clinical physiology of acid-base and electrolyte disorders. 1994, New York: McGraw-Hill, Inc, 808-812. 4
5.
go back to reference Griggs RC, Resnick J, Engel WK: Intravenous treatment of hypokalemic periodic paralysis. Arch Neurol. 1983, 40 (9): 539-40.CrossRefPubMed Griggs RC, Resnick J, Engel WK: Intravenous treatment of hypokalemic periodic paralysis. Arch Neurol. 1983, 40 (9): 539-40.CrossRefPubMed
6.
go back to reference Tawil R, McDermott MP, Brown R, Shapiro BC, Ptacek LJ, McManis PG, Dalakas MC, Spector SA, Mendell JR, Hahn AF, Griggs RC: Randomized trials of dichlorphenamide in the periodic paralyses. Working Group on Periodic Paralysis. Ann Neurol. 2000, 47 (1): 46-53. 10.1002/1531-8249(200001)47:1<46::AID-ANA9>3.0.CO;2-H.CrossRefPubMed Tawil R, McDermott MP, Brown R, Shapiro BC, Ptacek LJ, McManis PG, Dalakas MC, Spector SA, Mendell JR, Hahn AF, Griggs RC: Randomized trials of dichlorphenamide in the periodic paralyses. Working Group on Periodic Paralysis. Ann Neurol. 2000, 47 (1): 46-53. 10.1002/1531-8249(200001)47:1<46::AID-ANA9>3.0.CO;2-H.CrossRefPubMed
7.
go back to reference Tawil R, Moxley RT, Griggs RC: Acetazolamide-induced nephrolithiasis: implications for treatment of neuromuscular disorders. Neurology. 1993, 43 (6): 1105-6.CrossRefPubMed Tawil R, Moxley RT, Griggs RC: Acetazolamide-induced nephrolithiasis: implications for treatment of neuromuscular disorders. Neurology. 1993, 43 (6): 1105-6.CrossRefPubMed
9.
go back to reference Liu Z, Braverman LE, Malabanan A: Thyrotoxic periodic paralysis in a Hispanic man after the administration of prednisone. Endocr Pract. 2006, 12 (4): 427-431.CrossRefPubMed Liu Z, Braverman LE, Malabanan A: Thyrotoxic periodic paralysis in a Hispanic man after the administration of prednisone. Endocr Pract. 2006, 12 (4): 427-431.CrossRefPubMed
11.
go back to reference Monnier N, Procaccio V, Stieglitz P, Lunardi J: Malignant-hyperthermia susceptibility is associated with a mutation of the alpha 1-subunit of the human dihydropyridine-sensitive L-type voltage-dependent calcium-channel receptor in skeletal muscle. Am J Hum Genet. 1997, 60 (6): 1316-25. 10.1086/515454.PubMedCentralCrossRefPubMed Monnier N, Procaccio V, Stieglitz P, Lunardi J: Malignant-hyperthermia susceptibility is associated with a mutation of the alpha 1-subunit of the human dihydropyridine-sensitive L-type voltage-dependent calcium-channel receptor in skeletal muscle. Am J Hum Genet. 1997, 60 (6): 1316-25. 10.1086/515454.PubMedCentralCrossRefPubMed
12.
go back to reference Schapira AH, Griggs RC, eds: Blue Books of Practical Neurology Muscle Diseases. 1999, Boston: Butterworth-Heinemann, 24: 135-175. Schapira AH, Griggs RC, eds: Blue Books of Practical Neurology Muscle Diseases. 1999, Boston: Butterworth-Heinemann, 24: 135-175.
13.
go back to reference Marchant CL, Ellis FR, Halsall PJ, Hopkins PM, Robinson RL: Mutation analysis of two patients with hypokalemic periodic paralysis and suspected malignant hyperthermia. Muscle Nerve. 2004, 30 (1): 114-7. 10.1002/mus.20068.CrossRefPubMed Marchant CL, Ellis FR, Halsall PJ, Hopkins PM, Robinson RL: Mutation analysis of two patients with hypokalemic periodic paralysis and suspected malignant hyperthermia. Muscle Nerve. 2004, 30 (1): 114-7. 10.1002/mus.20068.CrossRefPubMed
14.
go back to reference Kim JB, Kim MH, Lee SJ, Kim DJ, Lee BC: The genotype and clinical phenotype of korean patients with familial hypokalemic periodic paralysis. J Korean Med Sci. 2007, 22 (6): 946-51.PubMedCentralCrossRefPubMed Kim JB, Kim MH, Lee SJ, Kim DJ, Lee BC: The genotype and clinical phenotype of korean patients with familial hypokalemic periodic paralysis. J Korean Med Sci. 2007, 22 (6): 946-51.PubMedCentralCrossRefPubMed
15.
go back to reference Robinson JE, Morin VI, Douglas MJ, Wilson RD: Familial hypokalemic periodic paralysis and Wolff-Perkinson-White syndrome in pregnancy. Can J Anaesth. 2000, 47 (2): 160-4.CrossRefPubMed Robinson JE, Morin VI, Douglas MJ, Wilson RD: Familial hypokalemic periodic paralysis and Wolff-Perkinson-White syndrome in pregnancy. Can J Anaesth. 2000, 47 (2): 160-4.CrossRefPubMed
16.
go back to reference Venance SL, Cannon SC, Fialho D, Fontaine B, Hanna MG, Ptacek LJ, Tristani-Firouzi M, Tawil R, Griggs RC, CINCH investigators: The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain. 2006, 129: 8-17. 10.1093/brain/awh639.CrossRefPubMed Venance SL, Cannon SC, Fialho D, Fontaine B, Hanna MG, Ptacek LJ, Tristani-Firouzi M, Tawil R, Griggs RC, CINCH investigators: The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain. 2006, 129: 8-17. 10.1093/brain/awh639.CrossRefPubMed
17.
go back to reference Miller TM, Dias da Silva MR, Miller HA, Kwiecinski H, Mendell JR, Tawil R, McManis P, Griggs RC, Angelini C, Servidei S, Petajan J, Dalakas MC, Ranum LP, Fu YH, Ptácek LJ: Correlating phenotype and genotype in the periodic paralyses. Neurology. 2004, 63 (9): 1647-1655.CrossRefPubMed Miller TM, Dias da Silva MR, Miller HA, Kwiecinski H, Mendell JR, Tawil R, McManis P, Griggs RC, Angelini C, Servidei S, Petajan J, Dalakas MC, Ranum LP, Fu YH, Ptácek LJ: Correlating phenotype and genotype in the periodic paralyses. Neurology. 2004, 63 (9): 1647-1655.CrossRefPubMed
22.
go back to reference Heine R, Pika U, Lehmann-Horn F: A novel SCN4A mutation causing myotonia aggravated by cold and potassium. Hum Mol Genet. 1993, 2 (9): 1349-1353. 10.1093/hmg/2.9.1349.CrossRefPubMed Heine R, Pika U, Lehmann-Horn F: A novel SCN4A mutation causing myotonia aggravated by cold and potassium. Hum Mol Genet. 1993, 2 (9): 1349-1353. 10.1093/hmg/2.9.1349.CrossRefPubMed
23.
go back to reference Rudel R, Lehmann-Horn F: Paramyotonia, potassium-aggravated myotonias and periodic paralyses. 37th ENMC International Workshop, Naarden, The Netherlands, 8–10 December 1995. Neuromuscular Disord. 1997, 7 (2): 127-132. 10.1016/S0960-8966(96)00418-X.CrossRef Rudel R, Lehmann-Horn F: Paramyotonia, potassium-aggravated myotonias and periodic paralyses. 37th ENMC International Workshop, Naarden, The Netherlands, 8–10 December 1995. Neuromuscular Disord. 1997, 7 (2): 127-132. 10.1016/S0960-8966(96)00418-X.CrossRef
24.
go back to reference Ricker K, Moxley RT, Heine R, Lehmann-Horn F: Myotonia fluctuans. A third type of muscle sodium channel disease. Arch Neurol. 1994, 51 (11): 1095-1102.CrossRefPubMed Ricker K, Moxley RT, Heine R, Lehmann-Horn F: Myotonia fluctuans. A third type of muscle sodium channel disease. Arch Neurol. 1994, 51 (11): 1095-1102.CrossRefPubMed
25.
go back to reference Ptacek LJ, Tawil R, Griggs RC, Meola G, McManis P, Barohn RJ, Mendell JR, Harris C, Spitzer R, Santiago F: Sodium channel mutations in acetazolamide-responsive myotonia congenita, paramyotonia congenita, and hyperkalemic periodic paralysis. Neurology. 1994, 44 (8): 1500-1503.CrossRefPubMed Ptacek LJ, Tawil R, Griggs RC, Meola G, McManis P, Barohn RJ, Mendell JR, Harris C, Spitzer R, Santiago F: Sodium channel mutations in acetazolamide-responsive myotonia congenita, paramyotonia congenita, and hyperkalemic periodic paralysis. Neurology. 1994, 44 (8): 1500-1503.CrossRefPubMed
26.
go back to reference Lerche H, Heine R, Pika U, George AL, Mitrovic N, Browatzki M, Weiss T, Rivet-Bastide M, Franke C, Lomonaco M: Human sodium channel myotonia: slowed channel inactivation due to substitutions for a glycine within the III-IV linker. J Physiol. 1993, 470: 13-22.PubMedCentralCrossRefPubMed Lerche H, Heine R, Pika U, George AL, Mitrovic N, Browatzki M, Weiss T, Rivet-Bastide M, Franke C, Lomonaco M: Human sodium channel myotonia: slowed channel inactivation due to substitutions for a glycine within the III-IV linker. J Physiol. 1993, 470: 13-22.PubMedCentralCrossRefPubMed
Metadata
Title
Practical aspects in the management of hypokalemic periodic paralysis
Author
Jacob O Levitt
Publication date
01-12-2008
Publisher
BioMed Central
Published in
Journal of Translational Medicine / Issue 1/2008
Electronic ISSN: 1479-5876
DOI
https://doi.org/10.1186/1479-5876-6-18

Other articles of this Issue 1/2008

Journal of Translational Medicine 1/2008 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.