Familial Periodic Paralyses (Normokalemic Periodic Paralysis)

A heterogenous group of inherited disorders characterized by recurring attacks of rapidly progressive flaccid paralysis or myotonia. These conditions have in common a mutation of the gene encoding the alpha subunit of the sodium channel in skeletal muscle. They are frequently associated with fluctuations in serum potassium levels. Periodic paralysis may also occur as a non-familial process secondary to THYROTOXICOSIS and other conditions. (From Adams et al., Principles of Neurology, 6th ed, p1481)

Also Known As:

Networked: 71 relevant articles (0 outcomes, 5 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Hyperkalemic Periodic Paralysis
2.	Renovascular Hypertension (Goldblatt Syndrome)
3.	Bartter Syndrome (Syndrome, Bartter)
4.	Paralysis (Palsy)
5.	Hypokalemic Periodic Paralysis (Periodic Paralysis, Hypokalemic)

Experts

1.	Lin, Shih-Hua: 3 articles (04/2012 - 01/2003)
2.	Yang, Sung-Sen: 2 articles (04/2012 - 06/2010)
3.	Chau, Tom: 2 articles (06/2010 - 01/2003)
4.	Chen, Qiming: 1 article (09/2020)
5.	Hu, SongNian: 1 article (09/2020)
6.	Liu, Yang: 1 article (09/2020)
7.	Mei, Hailiang: 1 article (09/2020)
8.	Sang, Daoqian: 1 article (09/2020)
9.	Shi, Peng: 1 article (09/2020)
10.	Tan, XinYu: 1 article (09/2020)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Familial Periodic Paralyses:

1.	Sodium Channels (Sodium Channel)IBA 08/01/2008 - "In this study, we aim to investigate the clinical features and Mutations of sodium channel alpha-subunit (SCN4A) genes in Chinese patients with normokalemic periodic paralysis (normoKPP). " 07/01/2014 - "Enhanced slow inactivation of the human skeletal muscle sodium channel causing normokalemic periodic paralysis." 08/01/2008 - "Mutations of sodium channel alpha-subunit genes in Chinese patients with normokalemic periodic paralysis." 06/13/2000 - "Defective slow inactivation of sodium channels contributes to familial periodic paralysis." 04/22/1999 - "Defective slow inactivation of sodium channels contributes to familial periodic paralysis."
2.	PotassiumIBA 06/01/1953 - "Recent studies on the role of potassium in hereditary (familial) periodic paralysis." 01/01/2014 - "Two cardinal features in this family were longer paralytic episodes compared to classical hyperkalemic/normokalemic periodic paralysis and the normal potassium value during the paralytic episodes. " 07/01/1983 - "We studied the serum potassium rise during a 30-min 100 W exercise test in 5 controls, 3 familial periodic paralysis (FPP) patients and their 2 symptom-less brothers. " 07/01/1983 - "Serum potassium exercise test in the diagnosis of familial periodic paralysis." 09/01/1957 - "Potassium movement in patients with familial periodic paralysis: relationship to the defect in muscle function."
3.	Captopril (Capoten)FDA LinkGeneric 01/01/1988 - "In the present study, they investigated the diagnosis and differentiation of diseases concomitant with hyperreninism, such as ACEDS, Bartter's syndrome, familial periodic paralysis, and renovascular hypertension treated with captopril for two months, and discussed the pathogenesis of ACEDS."
4.	SodiumIBA 01/01/2014 - "[Normokalemic periodic paralysis lasting for two weeks: a severe form of sodium channelopathy with M1592V mutation]." 08/10/2019 - "Normokalemic periodic paralysis and paramyotonia congenita can occur in different family members with skeletal muscle sodium channelopathies due to c.2078T>C(p.Ile693Thr) variation of SCN4A gene." 02/11/1964 - "[RECURRING ATTACKS OF MUSCULAR WEAKNESS OF HEREDITARY NATURE (FAMILIAL PERIODIC PARALYSIS, ADYNAMIC EPISODICA HEREDITARIA AND NORMOKALEMIC PERIODIC PARALYSIS SENSITIVE TO SODIUM]." 11/01/1983 - "Interictal conduction slowing in muscle fibers is consistent with the prevailing pathophysiologic hypothesis, which considers an increased membrane permeability to sodium ions as the fundamental defect underlying all forms of familial periodic paralysis." 06/01/1983 - "Familial periodic paralysis: low muscle potassium permeability or high sodium permeability?"
5.	Ion Channels (Ion Channel)IBA 01/01/2008 - "Familial periodic paralysis and nondystrophic myotonias are disorders of skeletal muscle excitability caused by mutations in genes coding for voltage-gated ion channels. " 12/01/2005 - "Nondystrophic myotonias and familial periodic paralyses are disorders of muscle excitability caused by mutations in genes coding for voltage-gated ion channels. " 01/01/2002 - "Familial periodic paralyses are typical channelopathies (i.e., caused by functional disturbances of ion channel proteins). " 09/01/2020 - "Normokalemic periodic paralysis (NormoKPP) of skeletal muscle is an autosomal dominant disorder caused by mutations in the gene encoding voltage-gated sodium channel protein type 4 subunit alpha (SCN4A), which leads to ion channel dysfunction. "
6.	Acetazolamide (Diamox)FDA LinkGeneric 05/01/1989 - "A Latin-American teenager was begun on a regimen of acetazolamide for a presumptive diagnosis of familial periodic paralysis. " 02/01/1977 - "This response distinguished this patient's condition from other forms of familial periodic paralysis and suggests that acetazolamide may be deleterious to some patients with periodic paralysis." 09/01/2013 - "In contrast to familial periodic paralysis, regular oral potassium supplementation is ineffective in thyrotoxic periodic paralysis, and acetazolamide precipitates, rather than prevents, attacks." 01/01/2010 - "RTA should be differentiated from familial periodic paralysis (FPP) because acetazolamide used in FPP aggravates RTA and sodium bicarbonate used in RTA aggravates hypokalemic periodic paralysis."
7.	Proteins (Proteins, Gene)FDA Link 01/01/2020 - "These are hypo- and normokalemic periodic paralysis, malignant hyperthermia susceptibility, CaV1.1-related myopathies, and myotonic dystrophy type 1. In addition, the CaV1.1 function in EC coupling is perturbed in Native American myopathy, arising from mutations in the CaV1.1-associated protein STAC3. " 01/01/2002 - "Familial periodic paralyses are typical channelopathies (i.e., caused by functional disturbances of ion channel proteins). " 09/01/2020 - "Normokalemic periodic paralysis (NormoKPP) of skeletal muscle is an autosomal dominant disorder caused by mutations in the gene encoding voltage-gated sodium channel protein type 4 subunit alpha (SCN4A), which leads to ion channel dysfunction. "
8.	Potassium ChlorideFDA LinkGeneric 09/01/2019 - "Further, she noted hypokalaemia during attacks of weakness in familial periodic paralysis, pioneering treatment with potassium chloride. " 08/01/1975 - "[Ulcerous stenosis of the small intestine following prolonged ingestion of potassium chloride in a subject with hypokalemic familial periodic paralysis]." 01/31/1976 - "In fifteen patients with hyperkalaemic familial periodic paralysis, inhalation of salbutamol alleviated hyperkaleamia and paralysis precipitated by exercise or oral administration of potassium chloride. "
9.	Atracurium (Tracrium)FDA Link 05/01/1991 - "We describe a patient with the hypokalemic type of familial periodic paralysis (FPP) who received atracurium for muscle relaxation as required for diagnostic laparoscopy. " 05/01/1991 - "Atracurium use in a patient with familial periodic paralysis." 08/01/1988 - "Atracurium and hypokalemic familial periodic paralysis." 12/01/1989 - "Propofol and atracurium in familial periodic paralysis."
10.	Anesthetics (Anesthetic Agents)IBA 02/01/1991 - "Successful anesthetic management of a patient with hypokalemic familial periodic paralysis undergoing cardiac surgery." 09/01/1977 - "Anesthetic experiences in a family with hypokalemic familial periodic paralysis." 11/01/1985 - "Anesthetic management of a patient with hypokalemic familial periodic paralysis for coronary artery bypass surgery."

Therapies and Procedures

1.	Nursing Care 06/01/1976 - "Nursing care study: familial periodic paralysis associated with hyperkalaemia."
2.	Anesthesia 01/01/1993 - "[Anesthesia in a case of hyperkalemic familial periodic paralysis]." 07/01/2004 - "Propofol-remifentanil target-controlled anesthesia in a patient with hyperkalemic familial periodic paralysis."
3.	Tonsillectomy 01/01/1997 - "Prolonged muscle weakness following emergency tonsillectomy in a patient with familial periodic paralysis and infectious mononucleosis."
4.	Therapeutics 04/01/2000 - "The clinical presentation is similar in most respects to familial periodic paralysis; however, the therapies proven to be effective differ in the two syndromes. "
5.	Intravenous Injections 12/01/1999 - "In the 5 years after Holler's paper there were many reports of deaths resulting from hypokalaemia, as well as several 'near misses', but clinicians were extremely cautious about early replacement probably, as an editorialist in The Lancet suggested, because 'the frightening effects of intravenous injections of potassium made clinicians reluctant to believe in a lack of potassium as a cause of trouble, except in very rare conditions such as familial periodic paralysis'. "