List of hypokalemic periodic paralysis medications 3. Diagnosis can also be made based on the clinical signs, together with elevated blood potassium levels, although potassium levels in the blood may not be outwith the normal range. Hyperkalemic periodic paralysis hyperpp is a disorder that causes occasional episodes of muscle weakness and sometimes a higher than normal level of potassium in the blood. Hyperkalemic periodic paralysis is a genetic disease that causes episodes of extreme muscle weakness and an increase of the potassium levels in the blood. Vice chairman and associate professor of dermatology, the mount sinai medical center, new york, ny. This depolarisation propagates to the ttubules where it triggers the entry of calcium ions via. In the hyperkalemic type hyperkpp, high serum potassium levels cause attacks of temporary muscle weakness that can result in paralysis when severe.
Prolonged qt interval and ventricular arrhythmias are the most common cardiac manifestations. Hyperkalemic periodic paralysis hypp is a dominantly inherited disorder of muscle in quarter horses, american paint horses, appaloosas, and quarter horse crossbred animals that causes episodes of tremors, myotonia, weakness, or paralysis in association with elevated serum potassium naylor, 1997. 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. Genetic disorders account for hypokalemic periodic paralysis, a condition of repeated episodes of reduced potassium levels and severe muscle weakness. Hyperkalemic periodic paralysis is an autosomal dominant disease leading to intermittent weakness associated with hyperkalemia and often precipitated by a.
Hyperkalemic periodic paralysis hypp, hyperkpp is an inherited autosomal dominant disorder that affects sodium channels in muscle cells and the ability to regulate potassium levels in the blood. Episodes of paralysis can occur in only certain areas of the body, like an arm or leg, or the whole body. Symptoms of hyperkalemic periodic paralysis in horses this hereditary disease seems to strike at random, and typically episodes last somewhere around fifteen to sixty minutes. Attacks occur randomly, but are often triggered by factors like foods or exercise. Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Inheritance is autosomal dominant with reduced penetrance in women fontaine, 1994. Hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness. 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. Hypokalemic periodic paralysis identifying triggers exercise habits. 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. Hyperkalemic periodic paralysis genetics home reference. Periodic paralysis pp is a muscle disease that causes episodic muscle weakness, in the family of diseases called channelopathies.
Hypokpp is one of a group of diseases, called inherited myopathies, that causes problems with. Laboratory evaluation revealed a markedly low potassium level. Common psoriasis triggers how psoriasis flares affect the body living with kidney disease adpkd psoriatic arthritis and your sleep why. However, you will still need to take your potassium tablets as prescribed by your doctor. If the diagnosis is indeed periodic paralysis, then one should think about the disease as. The underlying mechanism of these diseases are malfunctions in the ion channels in skeletal muscle cell membranes that allow electrically charged ions to leak in or out of. For more on the periodic paralyses, please see the 2009 report in focus. Those who have hyperkalemic periodic paralysis may find that highpotassium foods like cantaloupe, bananas, dried fruit, nuts etc.
Hypokalemic and normokalemic are two kinds of this genetic problem. Those with hypokalemic periodic paralysis are more apt to be bothered by sweets, starchy foods like baked goods, pasta, rice and salty foods. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Identify and eliminate the triggers of periodic paralysis. Periodic paralysis is a muscle disease that causes episodic muscle weakness, in the family of diseases called channelopathies.
During an attack of weakness, the fiber fails to maintain a normal resting potential and in this depolarized state the fiber is persistently refractory, unable to generate an action potential figure 73. Hypokalemic paralysis often referred to as familial is caused. Management considerations in hypokalemic periodic paralysis. Periodic paralysis, any of the forms of a rare disorder that is characterized by relatively shortterm, recurrent attacks of muscle weakness. It is characterized by muscle hyperexcitability or weakness which, exacerbated by potassium, heat or cold, can lead to uncontrolled shaking followed by paralysis. If associated with myoclonus jerks, consider small dose of benzodiazepine as jerk represents exercise and trigger of paralysis is rest after exercise. The major triggering factors are cessation of effort following strenuous exercise and carbohydraterich evening meals. Hypokalemic periodic paralysis what is hypokalemic periodic paralysis hypokpp. Meyer, department of anatomy and physiology and clinical sciences, kansas state university, manhattan, kansas 665065602, usa. It is classified as hypokalemic when episodes occur in association with low potassium blood levels or as hyperkalemic.
The most relevant are the periodic paralysis association ppa, the periodic paralysis news desk, and the muscular dystrophy association their web addresses can be found in the references section below 18, 19, 20. How to determine what triggers attacks of periodic paralysis. This is due to mutations in ion channels in the muscle membrane in conjunction with other factors such as eating salty or high carbohydrate foods, sleep or rest after exercising, strong emotions or. Periodic paralysis syndrome describes seven to eight different types of rare genetic conditions that cause symptoms of episodic muscle weakness and paralysis. Clinical features hypokalaemic periodic paralysis mim170400 hypopp is the most common pp, with a prevalence of. Its one of several diseases of horses that has a genetic basis. The condition is called hyperkalaemic periodic paralysis or hypp, mercifully. The medical name for high potassium level is hyperkalemia hyperpp is one of a group of genetic disorders that includes hypokalemic periodic paralysis and thyrotoxic periodic paralysis.
Avoidance of or potassium prophylaxis for common triggers, such as rest after. For a general phenotypic description and a discussion of genetic heterogeneity of hokpp, see hokpp1, which is caused by mutation in the cacnl1a3. Mutations in the scn4a gene can also cause hyperkalemic periodic paralysis hypp. Hyperkalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness or paralysis, usually beginning in infancy or early childhood. Hypokalemic periodic paralysis is a rare disorder in which a person experiences episodes of painless muscle weakness and often paralysis. Hypokalemic periodic paralysis is a rare autosomal disorder that is characterized by muscle weakness or paralysis that is triggered by a fall in serum potassium. Hypokalemic periodic paralysis hypokpp, also known as familial hypokalemic periodic. Hypokalemic periodic paralysis is characterized by a fall in potassium levels in the blood.
Familial periodic paralyses information page national. Periodic paralysis syndrome types, symptoms, diagnosis. The medical name for low potassium level is hypokalemia. Ask the vet hypp, supplements for all horses, magnesium for horses, and more. Its very common to experience an attack after waking from sleep. Hyperkal periodic paralysis triggers openanesthesia. 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 an overview sciencedirect topics. The ppa is a nonprofit charitable corporation founded in recognition of the need for an organization that fosters awareness of. The patients paralysis resolved upon repletion of his low potassium and he was. Hypokalemic periodic paralysis hypopp is a condition in which affected individuals may experience paralytic episodes with concomitant hypokalemia serum potassium hyperkalemic periodic paralysis hyperpp is characterized by attacks of flaccid limb weakness which may also include weakness of the muscles of the eyes, throat, and trunk, hyperkalemia serum potassium concentration 5 mmoll or an increase of serum potassium concentration of at least 1. Some animals will have more severe and frequent attacks than others on average, and even in the same horse, the intensity of the attack can vary from episode to episode.
Practical aspects in the management of hypokalemic. In the hypokalemic type hypokpp, low serum potassium levels can trigger attacks. Periodic paralyses hyperkalemic, hypokalemic, andersen. A clinically useful classification of primary periodic paralyses, shown in table 1, includes hypokalemic, hyperkalemic, and paramyotonic forms. Episodes tend to increase in frequency until midadulthood, after which they occur less frequently in many people with the condition. Quarter horse hyperkalemic periodic paralysis ufaw. Periodic paralysis is a group of rare genetic diseases that lead to weakness or paralysis from common triggers such as cold, heat, high carbohydrate meals, not eating, stress or excitement and physical activity of any kind.
See also hokpp2, which is caused by mutation in the scn4a gene. I get periodic questions about a condition that affects primarily quarterhorses, but also paint horses and appaloosas. Hyperkalemic periodic paralysis in horses symptoms. The most sensitive test for hyperkalemic periodic paralysis is the gene probe for hyperkalemic periodic paralysistype sodium channel mutation ie dna testing. Practical aspects in the management of hypokalemic periodic. Attacks cause severe weakness or paralysis that usually lasts from hours to days. Understanding and becoming familiar with the symptoms is another important part of completing the chart. The periodic paralysis is not associated with myotonia. Pp is classified as hypokalemic when episodes occur in association with low potassium blood levels or as.
Hypokalemic periodic paralysis genetics home reference nih. Muscle weakness during an attack usually affects the arms and legs and muscles of the eyes, throat, and trunk. Periodic paralysis is caused by a transient failure of muscle fiber excitability 4,5. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. The patient presented with sudden onset paralysis of his extremities. Introduction a group of disorders of different etiologies with episodic, shortlived, and hyporeflexic skeletal muscle weakness with or without myotonia without sensory deficit without loss of consciousness. Hypopp is one of a group of genetic disorders that includes hyperkalemic periodic paralysis and thyrotoxic periodic. Dietary guidelines for hypokalemic periodic paralysis. Changes to your diet may help reduce the frequency of episodes of paralysis.
Hypokalemic periodic paralysis periodic paralysis association. Periodic paralysis dr abdullah ansari pg2 medicine amu aligarh 2. Managing periodic paralysis 101 jacob levitt, md, faad president, periodic paralysis association. In people with familial hypokalemic periodic paralysis, however, the drop in blood potassium often triggers an episode of paralysis. The following are lists of the common possible symptoms of periodic paralysis one may experience as well as the paralysis and muscle weakness and the triggers, which may set them into motion. A missiondriven, independent and notforprofit whose goal is to help create successful partnerships between physicians, researchers, and periodic paralysis patients in order to speed diagnosis and improve therapy and management strategies, thereby improving quality of life and clinical outcomes for patients. Hypokalemic periodic paralysis hypopp is a condition in which affected individuals may experience paralytic episodes with concomitant hypokalemia serum potassium 5 mmoll or an increase of serum potassium concentration of at least 1. Triggers include cold environment, rest after exercise, stress or fatigue, alcohol, hunger, changes in activity level, potassium in food, specific foods. People with periodic paralysis pp have episodes in which their muscles become weak or paralyzed in response to variations in the amount of.
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