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:

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)

Experts

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 07/01/1973 - "Myotonic periodic paralysis improved by negative sodium balance." 10/01/2015 - "Hyperkalemic periodic paralysis (hyperKPP) is a muscle sodium-ion channelopathy characterized by recurrent paralytic attacks. " 07/01/2012 - "To assess whether myoplasmic ionic sodium (Na+) is increased in muscles of patients with hyperkalemic periodic paralysis (HyperPP) with 3-T sodium 23 (23Na) magnetic resonance (MR) imaging and to evaluate the effect of medical treatment on sodium-induced muscle edema. " 05/01/1987 - "Adynamia episodica hereditaria with myotonia: a non-inactivating sodium current and the effect of extracellular pH." 10/01/1996 - "The delayed relaxation or sustained contraction of skeletal muscle-myotonia-is frequently seen in myotonic dystrophy and sodium channelopathies (hyperkalemic periodic paralysis, paramyotonia congenita). "
2.	Phenytoin (Dilantin)FDA LinkGeneric 11/01/1996 - "[Hyperkalemic periodic paralysis or congenital paramyotonia: an attempt of treatment with phenytoin]." 08/01/1992 - "Results suggest phenytoin is useful in modifying disordered ion regulation in the sarcolemma and sarcoplasmic reticulum of skeletal muscle in equine hyperkalemic periodic paralysis." 08/01/1992 - "Effects of phenytoin in two myotonic horses with hyperkalemic periodic paralysis." 07/01/1993 - "Since phenytoin is clinically effective in several skeletal muscle disorders, triacylglycerol metabolism in skeletal muscle from four normal Quarter horses and four Quarter horses with hyperkalemic periodic paralysis was examined. " 07/01/1993 - "These findings suggest a potential involvement of triacylglycerol metabolism in the clinical efficacy of phenytoin in hyperkalemic periodic paralysis."
3.	Triglycerides (Triacylglycerol)IBA 07/01/1993 - "Since phenytoin is clinically effective in several skeletal muscle disorders, triacylglycerol metabolism in skeletal muscle from four normal Quarter horses and four Quarter horses with hyperkalemic periodic paralysis was examined. " 07/01/1993 - "These findings suggest a potential involvement of triacylglycerol metabolism in the clinical efficacy of phenytoin in hyperkalemic periodic paralysis." 10/15/1998 - "In equine hyperkalemic periodic paralysis (HyperPP), there is evidence suggesting that the primary defect in the sodium channel is associated with a secondary alteration in triacylglycerol-associated fatty acid metabolism (TAFAM) in skeletal muscle. " 07/01/1993 - "Triacylglycerol levels were increased in skeletal muscle from seven (three controls, four hyperkalemic periodic paralysis) of the eight horses on treatment with oral phenytoin for one week. " 07/01/1993 - "The horses with hyperkalemic periodic paralysis had low levels of 18:3 in the phospholipids, low levels of 16:0, 16:1 and 18:3 in the free fatty acids and low levels of 20:4 in triacylglycerols. "
4.	Acetazolamide (Diamox)FDA LinkGeneric 08/01/2000 - "Pharmacokinetic data generated by this study should facilitate estimation of appropriate dosages for acetazolamide use in horses with hyperkalemic periodic paralysis." 01/15/1977 - "Studies on the mechanism of action of acetazolamide in the prophylaxis of hyperkalemic periodic paralysis." 05/01/2013 - "This data show the remarkable benefit of acetazolamide on permanent weakness of hyperkalemic periodic paralysis in association with the T704M mutation." 05/01/2013 - "Long-term effectiveness of acetazolamide on permanent weakness in hyperkalemic periodic paralysis." 08/01/2002 - "There was no familial predisposition to hyperkalemic periodic paralysis, which may cause similar laryngopharyngeal signs, and the horse did not respond to administration of acetazolamide. "
5.	PotassiumIBA 12/01/1979 - "Regulation of plasma potassium in hyperkalemic periodic paralysis." 08/01/1979 - "There appears to be a continuous alteration in potassium regulation in our patients with hyperkalemic periodic paralysis." 08/01/1979 - "We studied venous potassium throughout a 36-hour period in two patients with hyperkalemic periodic paralysis and in nine normals under rigidly controlled conditions. " 08/01/1979 - "Hyperkalemic periodic paralysis is frequently considered a disorder in which episodes of weakness and an attendant rise in plasma potassium interrupt a baseline of normal strength and potassium. " 08/01/1979 - "Regulation of plasma potassium in hyperkalemic periodic paralysis."
6.	Sodium Channels (Sodium Channel)IBA 05/01/2013 - "We report a 65 year-old man with hyperkalemic periodic paralysis and late-onset permanent weakness in association with the common T704M mutation in α-subunit, skeletal muscle voltage-gated sodium channel gene. " 03/19/2013 - "In vitro contraction tests were performed on soleus muscle isolated from mice with knock-in missense mutations that result in HypoPP (sodium channel NaV1.4-R669H) or hyperkalemic periodic paralysis (HyperPP; sodium channel NaV1.4-M1592V). " 11/01/2011 - "Familial hyperkalemic periodic paralysis caused by a de novo mutation in the sodium channel gene SCN4A." 06/01/2010 - "Familial hyperkalemic periodic paralysis (HYPP) is a rare genetic disorder in which the sodium channels in skeletal muscle cells have altered structure and function. " 08/01/2002 - "Retrospective clinical and molecular analysis has confirmed suspicions that most normoKPP families actually have a variant of hyperkalemic periodic paralysis (hyperKPP) due to a mutation of the muscle-specific sodium channel gene (SCN4A). "
7.	N- (4'- fluorobutyrophenone)- 4- (4- chlorophenyl)pyridinium (HPP(+))IBA 07/01/1979 - "An electrophysiologic study of peripheral nerve excitability was performed in patients with hyperkalemic periodic paralysis (HPP) and in normal controls. " 12/15/1999 - "A 24-year-old man with sporadic hyperkalemic periodic paralysis (HPP) presented with moderate excessive daytime sleepiness and transitory episodes of weakness which occurred during and after sleep. " 01/01/1999 - "Mutations in the human skeletal muscle Na+ channel underlie the autosomal dominant disease hyperkalemic periodic paralysis (HPP). " 03/15/1994 - "Thirty offspring of a Quarter Horse sire, affected by hyperkalemic periodic paralysis (HPP), were examined electromyographically. " 01/01/1992 - "Electromyography (EMG) was used to detect myotonic discharges in Quarter Horse breeding stock and to follow the results of mating horses with hyperkalemic periodic paralysis (HPP). "
8.	Potassium aggravated myotoniaIBA 10/01/2011 - "It is best described by a combination of potassium-aggravated myotonia and hyperkalemic periodic paralysis. " 10/10/2006 - "Measurements on native muscle fibers revealed provocation-induced, intracellular Na+ accumulation and membrane depolarization by -41 mV for paramyotonia, by -30 mV for hyperkalemic periodic paralysis, and by -20 mV for potassium-aggravated myotonia. " 09/04/2012 - "To electrophysiologically characterize the Na(v)1.4 mutant N440K found in a Korean family with a syndrome combining symptoms of paramyotonia congenita, hyperkalemic periodic paralysis, and potassium-aggravated myotonia. " 10/10/2006 - "Muscle channelopathies such as paramyotonia, hyperkalemic periodic paralysis, and potassium-aggravated myotonia are caused by gain-of-function Na+ channel mutations. " 12/01/2000 - "Missense mutations of the human skeletal muscle voltage-gated Na channel (hSkM1) underlie a variety of diseases, including hyperkalemic periodic paralysis (HyperPP), paramyotonia congenita, and potassium-aggravated myotonia. "
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 04/01/1991 - "Electrophysiological studies on muscle fibres from patients with hyperkalemic periodic paralysis with myotonia have shown that the episodes of weakness are caused by a sustained depolarization of the sarcolemma to potentials between -40 and -60 mV. In muscle fibre segments from three such patients this sustained depolarization was caused by noninactivating Na+ channels with reduced single-channel conductance blocked by TTX and procainamide. "

Therapies and Procedures

1.	Electrodes (Electrode) 03/01/1988 - "To study the stimulating effect of adrenaline (ADR) on active Na+/K+ transport we used double-barrelled ion-sensitive micro-electrodes to measure the activities of extracellular K+ (aKe) and intracellular Na+ (aNai) in isolated preparations of rat soleus muscle, normal human intercostal muscle and in one case of hyperkalemic periodic paralysis (h.p.p.). "
2.	Anesthesia 01/01/2014 - "ANESTHESIA CHARACTERISTICS OF A PATIENT WITH HYPERKALEMIC PERIODIC PARALYSIS--CASE REPORT." 04/01/1993 - "[Anesthesia in familial hyperkalemic periodic paralysis]." 10/15/1992 - "Anesthesia and surgery in a Quarter Horse affected with hyperkalemic periodic paralysis resulted in euthanasia after 7 days of postoperative recumbency. " 01/01/2014 - "We report a case of a successfully conducted anesthesia, without complications of a patient with hyperkalemic periodic paralysis who underwent elective laparoscopic cholecystectomy for chronic calculous cholecystitis. " 06/01/1996 - "Hyperkalemic periodic paralysis episode during halothane anesthesia in a horse."
3.	General Anesthesia 08/01/1989 - "General anesthesia and hyperkalemic periodic paralysis." 06/01/2010 - "Combined spinal/general anesthesia with postoperative femoral nerve block for total knee replacement in a patient with familial hyperkalemic periodic paralysis: a case report."
4.	Transplants (Transplant) 10/01/2012 - "Management of a patient with hyperkalemic periodic paralysis requiring coronary artery bypass grafts."
5.	Knee Replacement Arthroplasty (Total Knee Replacement) 06/01/2010 - "Combined spinal/general anesthesia with postoperative femoral nerve block for total knee replacement in a patient with familial hyperkalemic periodic paralysis: a case report."