Hypokalaemia periodic paralysis liam j stapleton, 2018. Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Hypokalemic periodic paralysis hypopp or hypokpp is a rare disorder in which a person experiences episodes of painless muscle weakness and often paralysis. Pdf hypokalemic periodic paralysis is a rare genetic disorder. Diet for patients with hypokalemic periodic paralysis.
The patients paralysis resolved upon repletion of his low potassium and he was. Hypokalemic periodic paralysis l o w activity o f s a r c o p l a s m i c r e t i c u l u m a n d muscle d u r i n g an i n d u c e d a t t a. Both hypokalemic and hyperkalemic periodic paralysis may respond to small doses of acetazolamide, a diuretic medication. Episodes of paralysis can occur in only certain areas of the body, like an arm or leg, or the whole body.
Primary periodic paralysis ppp is a group of rare diseases that temporarily make muscles stiff, weak, or unable to move. Hypokalemic periodic paralysis genetics home reference nih. Symptoms mimicking those of hypokalemic periodic paralysis. Periodic paralyses hyperkalemic, hypokalemic, andersentawil. Muscle weakness during an attack usually affects the arms and legs and muscles of the eyes, throat, and trunk. In hyperkalemic periodic paralysis, shortterm treatment involves injections of a calcium gluconate solution, and longterm treatment may include insulin and dialysis of the blood. Apr 30, 2018 treatment is often necessary for acute attacks of hypokalemic periodic paralysis but seldom for hyperkalemic periodic paralysis. Acetazolamide treatment of hypokalemic periodic paralysis. Treatment is often necessary for acute attacks of hypokalemic periodic paralysis but seldom for hyperkalemic periodic paralysis. Hypokalaemic periodic paralysis is a rare skeletal muscle channelopathy causing flaccid paralysis, which predominantly presents in adolescents and young adults. Hypokalemic periodic paralysis hpp is a rare autosomal dominant channelopathy characterized by skeletal muscle weakness or paralysis when there is a fall in potassium levels in blood. The sodium and potassium balance in your muscle cells is off. However, you will still need to take your potassium tablets as prescribed by your doctor.
Pp is classified as hypokalemic when episodes occur in association with low potassium blood levels or as. Attacks cause severe weakness or paralysis that usually lasts from hours to days. Hypokalemic periodic paralysis hpp is a rare autosomal dominant channelopathy characterised by muscle weakness or paralysis when there is a fall of potassium level in the blood. Potassium levels in the blood drop during these episodes. In normal persons glucose and insulin lowered serum k 0.
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. In periodic paralysis, these deficits may or may not hold true. Hypokalemic periodic paralysis is an autosomaldominant disorder caused most commonly by mutations in the alpha subunit of the skeletal muscle calcium channel gene cav1. Acute attacks of weakness in patients with hypokalemic periodic paralysis can usually be treated with oral potassium preparations. Management considerations in hypokalemic periodic paralysis. Hypokalemic periodic paralysis hypopp is a disorder that causes occasional episodes of muscle weakness and sometimes a lower than normal level of potassium in the blood. Hypokalemic periodic paralysis is a disorder characterized by episodes of weakness, sparing the respiratory muscles, with a matching fall in serum potassium that lasts anywhere from a few hours to a few days. Hypokalemic periodic paralysis hypopp is caused in 80% of cases by point mutations in the voltagegated calcium channel gene on chromosome 1 fontaine. Review of the diagnosis and treatment of periodic paralysis.
The mechanism of effect of acetazolamide was not discovered. Hypokalemic periodic paralysis case journal of reproductive. Periodic paralysis pp is a muscle disease that causes episodic muscle weakness, in the family of diseases called channelopathies. Common features of pp include autosomal dominant inheritance. Apr 30, 2002 hypokalemic periodic paralysis hypopp is a condition in which affected individuals may experience paralytic episodes with concomitant hypokalemia serum potassium hypokalemic periodic paralysis hypopp is a condition in which affected individuals may experience paralytic episodes with concomitant hypokalemia serum potassium aug 18, 2017 hyperkalemic periodic paralysis is a genetic disease that causes episodes of extreme muscle weakness and an increase of the potassium levels in the blood.
Clinical profile in hypokalemic periodic paralysis cases electronic. Hypokalemic periodic paralysis hokpp is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels hypokalemia. The periodic paralyses are generally divided into hyperkalemic periodic paralysis, hypokalemic periodic paralysis. Hypokalemic periodic paralysis is a rare genetic disorder characterized by recurrent attacks of skeletal muscle weakness with associated hypokalemia which is precipitated by stress, cold, carbohydrate load, infection, glucose infusion, hypothermia, metabolic alkalosis, anesthesia, and steroids. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. Ion channels are tiny pores openings that allow ions molecules like sodium and calcium to move through the cell membrane. Hypokalemic periodic paralysis types 1 and 2 are caused by mutations in the cacnl1a3 and scn4a genes, respectively, and are both inherited in. For the 34 participants having attack rate data for both treatment phases, the mean improvement in attack rate p 0. A medline search was conducted for articles published during the last 40 yr based on the key words thyrotoxic periodic paralysis and hypokalemic periodic paralysis.
Clinical profile in hypokalemic periodic paralysis cases. Acetazolamide is the most effective treatment available for hypokalemic periodic paralysis. Changes to your diet may help reduce the frequency of episodes of paralysis. Primary periodic paralyses include hypokalemic paralysis hypopp, hyperkalemic paralysis hyperpp, and andersen. I report a case of a 33yearold caucasian man who presented with sudden onset paralysis, following previous similar presentations without investigation. This series summarizes the clinical and metabolic features of 10 patients who presented to the western and sunshine hospitals in melbourne, australia, between 1997 and 2002 with thyrotoxic periodic paralysis tpp. Hypokalemic and normokalemic are two kinds of this genetic problem. Its the most common of several genetic disorders that cause periodic paralysis. Fda approves drug for primary hyperkalemic and hypokalemic periodic paralysis mda admin 08122015 01112016 we couldnt be more pleased for our families affected by hyperkalemic and hypokalemic periodic paralysis, as the first treatment for these diseases has been approved by the u. The first attack usually occurs in childhood or adolescence. Periodic paralysis pp is a rare neuromuscular disorder related to a defect in muscle ion channels, characterized by episodes of painless muscle weakness, which may be precipitated by heavy exercise, fasting, or highcarbohydrate meals. Inhereted disorder usually a genetic defect in the calcium channel. The different types of periodic paralyses are distinguished by what happens to potassium levels in the blood specifically the serum, or fluid, portion of the blood.
The hypokalemia in hypokalemic periodic paralysis is due to potassium shifts rather than deficit. Hypokalemic periodic paralysis hypokpp, also known as familial hypokalemic periodic paralysis fhpp, is a rare, autosomal dominant channelopathy characterized by muscle weakness or paralysis when there is a fall in potassium levels in the blood. Laboratory evaluation revealed a markedly low potassium level. May 18, 2017 periodic paralysis syndrome describes seven to eight different types of rare genetic conditions that cause symptoms of episodic muscle weakness and paralysis. We will focus on the familial form of hypokalemic periodic paralysis that is due to mutations in one of various genes for ion channels. Intravenous treatment of hypokalemic periodic paralysis jama.
Dietary guidelines for hypokalemic periodic paralysis. After administration of acetazolamide, 250 mg four times daily, serum k fell 0. These episodes can last from a few minutes to a few days, depending on. Fda approves drug for primary hyperkalemic and hypokalemic.
Potassium levels in the blood rise during these episodes. Oct 21, 2008 hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness. Treatment consists of diet changes and avoiding things that trigger your attacks. Periodic paralyses hyperkalemic, hypokalemic, andersentawil syndrome what is periodic paralysis. If recognised and treated appropriately, patients recover without any clinical sequellae. Hypokalemic periodic paralysis an overview sciencedirect. She had the history of polymyositis diagnosis and the use of deltacortril and received intensive care treatment in another clinic with a similar complaint about a. Familial periodic paralyses information page national. Dichlorphenamide, a carbonic anhydrase inhibitor, was approved by the fda in august 2015 for the management of primary hyperkalemic periodic paralysis. Thirtyfour of 42 participants with hypokalemic periodic paralysis completed both treatment phases. Following administration of glucose and insulin to three patients with hypokalemic periodic paralysis, serum k fell 1. It is classified as hypokalemic when episodes occur in association with low potassium blood levels or as hyperkalemic when episodes can be induced by elevated potassium. Hypokalaemic paralysis postgraduate medical journal.
Hypokalemic periodic paral trigger open anesthesia. Hypokalemic periodic paralysis is a channelopathy caused by defective sodium, calcium or potassium ionchannels. Jun 12, 2017 hypokalemic periodic paralysis hokpp is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels hypokalemia. Periodic paralysis pp is a rare neuromuscular disorder related to a. The patient presented with sudden onset paralysis of his extremities.
The medical name for low potassium level is hypokalemia. Nov 10, 2017 primary periodic paralyses pps are rare autosomal. In individuals with this mutation attacks often begin in adolescence and are triggered by strenuous exercise, high carbohydrate meals, or by injection of insulin, glucose, or epinephrine. Designed by the national institutes of health this information is based on a diet designed at the national institutes of health in the 1960s for a young man with hypokalemic periodic paralysis hypokpp. The two most common types of periodic paralyses are.
Practical aspects in the management of hypokalemic periodic. Pps include hypokalemic paralysis, hyperkalemic paralysis, and andersentawil syndrome. This article focuses on questions that arise about diagnosis and treatment for people with hypokalemic periodic paralysis. Acetazolamide produced a mild metabolic acidosis but did not have a demonstrable effect on total body sodium, total body potassium, or thyroid function.
The syndrome of hypokalaemic paralysis represents a heterogenous group of disorders characterised clinically by hypokalaemia and acute systemic weakness. Mda is currently funding 11 grants in the periodic paralyses. Hypokalemic periodic paralysis hpp is a relatively common and potentially lifethreating condition that can be either sporadic or recurring and has both inherited and acquired causes. This form of paralysis is associated with low potassium levels. Hypokalemic periodic paralysis genetic and rare diseases. Medically accurate, uptodate information about the periodic paralyses accessible to patients, caretakers and physicians. Hypokalemic periodic paralysis hypopp is a condition in which affected individuals may experience paralytic episodes with concomitant. Hypokalaemic paralysis is a relatively uncommon but potentially lifethreatening clinical syndrome. Pp include hypokalemic paralysis, hyperkalemic paralysis, and andersentawil syndrome. Episodes typically involve a temporary inability to move muscles in the arms and legs.
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