Hyperkalemic Periodic Paralysis (Periodic Paralysis, Hyperkalemic)

An autosomal dominant familial disorder which presents in infancy or childhood and is characterized by episodes of weakness associated with hyperkalemia. During attacks, muscles of the lower extremities are initially affected, followed by the lower trunk and arms. Episodes last from 15-60 minutes and typically occur after a period of rest following exercise. A defect in skeletal muscle sodium channels has been identified as the cause of this condition. Normokalemic periodic paralysis is a closely related disorder marked by a lack of alterations in potassium levels during attacks of weakness. (Adams et al., Principles of Neurology, 6th ed, p1481)
Also Known As:
Periodic Paralysis, Hyperkalemic; Paralysis, Hyperkalemic Periodic; Adynamia Episodica Hereditaria; Familial Hyperkalemic Periodic Paralysis; Hyperkalemic Periodic Paralysis, Familial; Periodic Paralysis - Hypokalemic; Primary Hyperkalemic Periodic Paralysis; Hyperkalemic Periodic Paralyses; Myotonic Periodic Paralyses; Paralyses, Hyperkalemic Periodic; Paralyses, Myotonic Periodic; Paralysis, Myotonic Periodic; Periodic Paralyses, Hyperkalemic; Periodic Paralyses, Myotonic; Periodic Paralysis, Myotonic; Myotonic Periodic Paralysis; Paralysis, Periodic, Hyperkalemic, Familial
Networked: 142 relevant articles (2 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Myotonic Disorders (Paramyotonia Congenita)
2. Myotonia
3. Myotonia Congenita (Thomsen Disease)
4. Hypokalemic Periodic Paralysis (Periodic Paralysis, Hypokalemic)
5. Paralysis (Palsy)


1. Kubota, Tomoya: 3 articles (06/2012 - 05/2009)
2. Takahashi, Masanori P: 3 articles (06/2012 - 05/2009)
3. Lehmann-Horn, F: 3 articles (10/2011 - 01/2000)
4. Jurkat-Rott, Karin: 2 articles (11/2015 - 07/2012)
5. Hanna, Michael G: 2 articles (04/2013 - 08/2002)
6. Kim, June-Bum: 2 articles (12/2012 - 11/2011)
7. Sakoda, Saburo: 2 articles (06/2012 - 05/2009)
8. Jurkat-Rott, K: 2 articles (10/2011 - 10/2006)
9. Meola, G: 2 articles (01/2008 - 01/2003)
10. Sansone, V: 2 articles (01/2008 - 01/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hyperkalemic Periodic Paralysis:
1. SodiumIBA
2. Phenytoin (Dilantin)FDA LinkGeneric
3. Triglycerides (Triacylglycerol)IBA
4. Acetazolamide (Diamox)FDA LinkGeneric
5. PotassiumIBA
6. Sodium Channels (Sodium Channel)IBA
7. N- (4'- fluorobutyrophenone)- 4- (4- chlorophenyl)pyridinium (HPP(+))IBA
8. Potassium aggravated myotoniaIBA
9. Ion Channels (Ion Channel)IBA
04/01/2007 - "For a comprehensive review of pertinent research and studies with application to diagnosis and treatment of individuals with nondystrophic myotonic disorders, the present article is best read in the context of other articles in this issue, especially those on ion channel physiology (Cannon) and pharmacology (Conte-Camerino), and on hyperkalemic periodic paralysis (Lehmann-Horn)."
04/01/2000 - "Therefore, we are underway to perform molecular genetic analysis of ion channels since paramyotonia/hyperkalemic periodic paralysis is known to be associated with Na(+)-channel missense mutations, while hypokalemic periodic paralysis is with Ca(++)-channel. "
07/01/1996 - "The fact that three other paroxysmal neurological disorders (periodic ataxia with myokymia and hypo- and hyperkalemic periodic paralysis) are due to mutation in ion-channel genes raises the possibility that PDC is also due to an ion-channel gene mutation. "
10/01/1999 - "Unique among reviews of this topic is that all known human hereditary diseases of voltage-gated ion channels are described covering various fields of medicine such as neurology (nocturnal frontal lobe epilepsy, benign neonatal convulsions, episodic ataxia, hemiplegic migraine, deafness, stationary night blindness), nephrology (X-linked recessive nephrolithiasis, Bartter), myology (hypokalemic and hyperkalemic periodic paralysis, myotonia congenita, paramyotonia, malignant hyperthermia), cardiology (LQT syndrome), and interesting parallels in mechanisms of disease emphasized. "
08/01/2001 - ""Pure" myotonic disorders affect only muscle and can be separated into ion channel disorders affecting either the chloride channel (myotonia congenita Thomsen or myotonia congenita Becker) or those affecting the sodium channel (paramyotonia, hyperkalemic periodic paralysis, and myotonia fluctuans). "
10. Procainamide (Procan)FDA LinkGeneric

Therapies and Procedures

1. Electrodes (Electrode)
2. Anesthesia
3. General Anesthesia
4. Transplants (Transplant)
5. Knee Replacement Arthroplasty (Total Knee Replacement)