Approval was based on 2 randomized, doubleblinded placebocontrolled studies that included 8 patients. 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. Andersentawil syndrome classically comprises three groups of features. Familial periodic paralyses information page national. 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. A kindred in which 7 males and 2 females exhibit hypokalemic periodic paralysis has been intensively studied by chemical analyses of skeletal muscle, serial measurements of body composition, and electromyography.
Phpp have been reported in indian litera ture in adultsl. Recently, after he completed his events, he felt extremely weak, and his legs became like rubber. 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. Jun 12, 2017 hypokalemic periodic paralysis hokpp is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels hypokalemia. Hypokalemic periodic paralysis case journal of reproductive. Hypokalaemic paralysis postgraduate medical journal. Primary hypokalemic periodic paralysis indian pediatrics. Hypokalemic periodic paralysis free download as powerpoint presentation. Outline causes of primary periodic paralysis causes of secondary periodic paralysis presentation of hyperkalemic pp comparison between hyper pp vs hypo pp. Description periodic paralysis disorders are genetic disorders that affect muscle strength. Features of hypokalemic periodic paralysis there are two forms of hypokpp, a.
Changes to your diet may help reduce the frequency of episodes of paralysis. Weakness is a common, albeit nonspecific, presentation the differential diagnosis for the complaint of. The syndrome of hypokalaemic paralysis represents a heterogenous group of disorders characterised clinically by hypokalaemia and acute systemic weakness. The invitae periodic paralysis panel includes analysis for both hyperkalemic and hypokalemic periodic paralysis. We studied the incidence, etiology, clinical profile, and acidbase disturbances in patients presenting with hypokalemic paralysis and analyzed the significance of periodic and non periodic forms. Ricker dept of physiology, university of ulm, ulm, germany. Paralytic attacks are induced by a drop in the serum potassium level and they have been associated with specific triggers. Unlike in hyperakalemic periodic paralysis, emg does not usually show myotonia. Features of hypokalemic periodic paralysis there are two forms of hypokpp, a paralytic form and a myopathic form. Hypokalemic periodic paralysis an overview sciencedirect.
Periodicparalysis differential diagnosis and important. Hypokalemic periodic paralysis hokpp is a rare neuromuscular disorder caused by altered transport of cellular potassium that leads to significant muscle weakness of the extremities. The mechanism responsible for the onset of paralytic attacks, during which serum potassium decreases, and the real nature of the ensuing neurophysiological changes remain debatable. Management considerations in hypokalemic periodic paralysis include accurate diagnosis, potassium dosage for acute. Anaesthetic management of a patient with hypokalemic periodic. Oct 21, 2008 hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness. Attacks are more severe than in hyperakalemic periodic paralysis. 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. Periodic paralysis definition periodic paralysis pp is the name for several rare, inherited muscle disorders marked by temporary weakness, especially following rest, sleep, or exercise. Hypokalemic periodic paralysis periodic paralysis intl. 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. Periodic paralysis pdf document stories from families affected by periodic paralysis. Testing with the invitae periodic paralysis panel includes analysis of the following four most common associated genes.
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. However, you will still need to take your potassium tablets as prescribed by your doctor. Hypokalemic periodic paralysis hypokpp is one of the primary forms of periodic paralysis, caused by one or more mutations in the calcium, sodium or potassium ion channels in muscle membrane. Find out information about hypokalemic periodic paralysis. Hypokalemic periodic paralysis and influenza to the editor.
Attacks cause severe weakness or paralysis that usually lasts from hours to days. Periodic paralyses hyperkalemic, hypokalemic, andersen. Primary periodic paralysis ppp is a group of rare diseases that temporarily make muscles stiff, weak, or unable to move. Weakness is recurrent, affecting mainly the limbs, and is often brought on by exercising or eating too many or too few carbohydrates. The patients paralysis resolved upon repletion of his low potassium. Uncovering periodic paralysis for more information, please visit. The patients paralysis resolved upon repletion of his low potassium and he was. Practical aspects in the management of hypokalemic periodic.
Hypokalemic periodic paralysis in a hyperthyroid black woman. Hypokalemic periodic paralysis periodic paralysis i an ad condition of late onset that is more intense in and occurs following strenuous exercise or carbohydrate meals, affecting the extremities, respiratory and cardiac muscle, potentially causing ventricular tachycardia and premature ventricular contractions treatment kcl, acetazolamide. Episodes of paralysis can occur in only certain areas of the body, like an arm or leg, or the whole body. If recognised and treated appropriately, patients recover without any clinical sequellae. Periodic paralysis pp is a rare neuromuscular disorder related to a. 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. The hypokalemia in hypokalemic periodic paralysis is due to potassium shifts rather than deficit. Introduction hypokalemic periodic paralysis hopp is a wellknown clinical entity, but many etiological and neurophysiological problems remain.
Hypokalemic periodic paralysis is a disorder of muscle whereby. Familial periodic paralysis is a rare autosomal dominant condition with considerable variation in penetrance characterized by episodes of flaccid paralysis with loss of deep tendon reflexes and failure of muscle to respond to electrical stimulation. The primary pp is due to inherited ion channel defects while secondary pp is due to other medical conditions like thyrotoxicosis. In periodic paralysis, these deficits may or may not hold true. Dietary guidelines for hypokalemic periodic paralysis. Emerson, plus many other medical authorities, those with hypokpp should follow a diet which is high in protein, low. Hypokalemic and normokalemic are two kinds of this genetic problem. The two most common types of periodic paralyses are. Familial periodic paralysis genetic and rare diseases. Hypokalemic periodic paralysis article about hypokalemic. Primary hypokalemic periodic paralysis jimmy jaworski is a 16yearold sprinter on the high school track team. 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.
Hypokalemic periodic paralysis is characterized by a fall in potassium levels in the blood. In children hypokalemic paralysis secondary to gastroenteritis and chronic renal. Hypokalemic periodic paralysis is a channelopathy caused by defective sodium, calcium or potassium ionchannels. Primary periodic paralysis is not just 1 condition but a group of longterm neuromuscular disorders. Review of the diagnosis and treatment of periodic paralysis. After the most recent meet, he was unable to walk and had to be carried from the track on a stretcher. A rare heterogeneous group of disorders which is characterized by a sudden onset of reversible muscle paralysis. Hypokalaemic periodic paralysis is a rare skeletal muscle channelopathy causing flaccid paralysis, which predominantly presents in adolescents and young adults. I report a case of a 33yearold caucasian man who presented with sudden onset paralysis, following previous similar presentations without investigation. The perioperative management of a patient with hypokalemic periodic paralysis is challenging in view of the increased risk of paralysis. According to the nih physicians and dietician mary r. Apr 30, 2018 dichlorphenamide, a carbonic anhydrase inhibitor, was approved by the fda in august 2015 for the management of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants. There is an increased risk of pre and post anaesthetic paralysis.
The medical name for low potassium level is hypokalemia. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. Hypokalemic periodic paralysis genetics home reference nih. Hypokalemic periodic paralysis the importance of patient. There are two major forms, hypokalemic and hyperkalemic, each caused by. Laboratory evaluation revealed a markedly low potassium level. Hypokalaemic paralysis is a relatively uncommon but potentially lifethreatening clinical syndrome.
In the hypokalemic form, the paralysis is caused by low levels of potassium. Periodic paralysis pp is a muscle disease that causes episodic muscle weakness, in the family of diseases called channelopathies. The clinical syndrome of periodic paralysis is typically described as consisting of tempo rary weakness of skeletal muscle in individuals who are normal. After the most recent meet, he was unable to walk and had. Familial periodic paralysis is a disease characterized by sudden attacks of weakness and paralysis. Hyperkal periodic paralysis triggers open anesthesia.
Get a printable copy pdf file of the complete article 309k. Hypokalemic periodic paralysis is a rare disorder in which a person experiences episodes of painless muscle weakness and often paralysis. Diet for patients with hypokalemic periodic paralysis. Articles from journal of the national medical association are provided here courtesy of national medical association.
The first attack usually occurs in childhood or adolescence. Statland jm, fontaine b, hanna mg, johnson ne, kissel jt, sansone va, et al. Hypokalemic periodic paralysis genetic and rare diseases. 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. Pdf hypokalemic periodic paralysis is a rare genetic disorder characterized by recurrent attacks of skeletal muscle weakness with associated. Hypokalemic periodic paralysis support and info group home. 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. Thyrotoxic periodic paralysis tpp is a condition featuring attacks of muscle weakness in the presence of hyperthyroidism overactivity of the thyroid gland. Familial periodic paralysis msd manual professional edition. Dichlorphenamide, a carbonic anhydrase inhibitor, was approved by the fda in august 2015 for the management of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants.
Hypokalemic periodic paralysis is an autosomaldominant disorder caused most commonly by mutations in the alpha subunit of the skeletal muscle calcium channel gene cav1. 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. Apr 30, 2018 ryan dp, da silva mr, soong tw, et al. Jul, 2017 there are 4 forms of familial periodic paralysis. Hypokalemic periodic paralysis hypopp was considered. Other causes of weakness and paralysis acute intermittent porphyria ascending weakness evaluation labs.
Familial hypokalaemic periodic paralysis occurs as an. Hypokalemic periodic paralysis is a rare and dramatic complication of hyperthyroidism. Firm data on the management of periodic paralysis during pregnancy is lacking. Also note hypokalemic periodic paralysis is decrease in serum potassium can be triggered by stress, exercise, highcarbohydrate or salty meals, hypothermia, menstruation, pregnancy, or insulin. Periodic paralyses hyperkalemic, hypokalemic, andersentawil syndrome what is periodic paralysis. Periodic paralyses hyperkalemic, hypokalemic, andersentawil. Hypokalemic periodic paralysis support and info group. The involvement of the sarcoplasmic reticulum sr in attacks of hypokalemic periodic paralysis was first documented by electronmicroscopic studies of the affected muscles shy, wanko, rowly and engel 1961. Many levels stay the same and should not be used as a guideline for diagnosis of an episode. Hypokalemic periodic paralysis, geneclinics neuromuscular information nondystrophic myotonias and periodic paralyses. The associations allocation for research on hyperkalemic and hypokalemic periodic paralysis. Hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness.
A model for a clinical and research approach to a rare disorder. Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Eating, especially carbohydrates, made him feel worse. Periodic paralysis electrolyte disturbance, k, mg, ca 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. Episodes typically involve a temporary inability to move muscles in the arms and legs. Case 4 primary hypokalemic periodic paralysis jimmy jaworski is a 16yearold sprinter on the high school track team. Hypokalemic periodic paralysis hokpp is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels hypokalemia.
Primary periodic paralyses include hypokalemic paralysis hypopp, hyperkalemic paralysis hyperpp, and andersen. Clinical profile in hypokalemic periodic paralysis cases electronic. Hypokalemic periodic paral trigger open anesthesia. Hypokalaemia periodic paralysis liam j stapleton, 2018. Easily opened, potassium, a plastic or paper cup in which. In the hyperkalemic form, the paralysis is caused by high levels of potassium in the blood. Recently, after he completed his events, he felt extremely weak, and his leg became like rubber. Hypokalemia a decreased potassium level in the blood is usually present during attacks. Hypokalemic periodic paralysis hkpp is a rare genetic disorder with autosomal dominant inheritance and 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. 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. The patient presented with sudden onset paralysis of his extremities.