The two most common types of periodic paralyses are. There are two possible mechanism operates in disease process which may lead to hypokalemia are either transcellular shift of potassium hypokalaemic periodic paralysis, thyrotoxic periodic paralysis, barium poisoning, insulin excess, alkalosis or renal loss excessive mineralocorticoids. Laboratory evaluation revealed a markedly low potassium level. Hypokalemic periodic paralysis potassium medicine scribd. 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. 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. 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. Various laboratory examinations outside of ictal and interictal serum potassium measures play an important role in both diagnosis and management of hypokalemic periodic paralysis. Hypokalemic pp may be familial with autosomal dominant inheritance or may be acquired in patients with thyrotoxicosis 27. The medical name for low potassium level is hypokalemia. List of hypokalemic periodic paralysis medications 3.
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. Disease profile hypokalemic periodic paralysis is an inherited. Hypokalemic periodic paralysis free download as word doc. Hyperkalemic periodic paralysis, on the other hand, is associated with an increase in the potassium level. Fda approves drug for primary hyperkalemic and hypokalemic. Hypokalemic periodic paralysis types 1 and 2 are caused by mutations in the cacnl1a3 and scn4a genes, respectively, and are both inherited in.
Chronic consumption of such carbonated beverages offset acidosis. 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. The first attack usually occurs in childhood or adolescence. Correct the k deficit potassium replacement our patients serum k level. Hypokalemic periodic paralysis genetic and rare diseases. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. Familial periodic paralyses information page national. Pp is classified as hypokalemic when episodes occur in association with low potassium blood levels or as. Hypokalemic paralysis due to excessive sweating now that we know what is wrong with our patient, we should treat him in our ward. Case presentation hypokalemia free download as powerpoint presentation. Paralysis attack hypokalemic periodic paralysis youtube. Hypokalemic periodic paralysis hypopp is a condition in which affected individuals may experience paralytic episodes with concomitant hypokalemia serum potassium hypokalemic periodic paralysis has been related to mutation in the cacnl1a3, scn4a, or kcne3 11qq14 gene and its characterized by attacks that tend to occur on awakening after exercise or after a heavy meal and may last for several days. This makes the potassium to shift from the ecf to the muscle cells.
Specifically, ekg, tsh, free t3, and free t4 are the minimum indicated labs, with renal and adrenal function also recommended. 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. He has a serum concentration of potassium 1,6 meql and ecg changes prolonged pr and flattening of t waves. 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.
Episodes typically involve a temporary inability to move muscles in the arms and legs. Statland jm, fontaine b, hanna mg, johnson ne, kissel jt, sansone va, et al. Periodic paralyses hyperkalemic, hypokalemic, andersentawil. Apr 05, 2016 secondary periodic paralysis a demonstrably known causes serum potassium abnormal even in interictal phase 7. Diuretic use and gastrointestinal losses are common. Hypokalemic paralysis often referred to as familial is caused. Hypokalemic periodic paralysis an overview sciencedirect. Morbidity and mortality associated with unrecognized disease include respiratory failure and death. Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Review of the diagnosis and treatment of periodic paralysis.
Oct 21, 2008 hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness. Hypokalemic pp is the most common of the periodic paralyses, but is still quite rare, with an estimated prevalence of 1 in 100,000 1. Hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness. The patient presented with sudden onset paralysis of his extremities. Binder, md acute hypokalemic paralysis is an uncommon cause of acute weakness. Mar 25, 2017 hypokalemic periodic paralysis hypokpp is a rare, autosomal dominant channelopathy characterized by muscle weakness or paralysis with a matching fall in potassium levels in the blood primarily. Journal of translational medicine commentary open access practical aspects in the management of hypokalemic periodic paralysis jacob o levitt1,2 address. Conventional classification of periodic paralysis primary or familial periodic paralysis. Hypokalemic periodic paralysis is characterized by episodes of muscle paralysis lasting from a few to 48 h, associated with a decrease in serum potassium levels, in the absence of myotonia. 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. Attacks cause severe weakness or paralysis that usually lasts from hours to days.
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. 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 characterized by a fall in potassium levels in the blood. Hypokalemic periodic paralysis free download as powerpoint presentation. Pathophysiology hypokalemia free download as word doc. Episodes of paralysis can occur in only certain areas of the body, like an arm or leg, or the whole body. Hypokalemic periodic paralysis hypopp or hypokpp is a rare disorder in which a person experiences episodes of painless muscle weakness and often paralysis. Hypokalemic periodic paralysis weakness nervous system scribd. 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. Hypokalemia and hyperkalemia are common electrolyte disorders caused by changes in potassium intake, altered excretion, or transcellular shifts. Periodic paralysis pp is a muscle disease that causes episodic muscle weakness, in the family of diseases called channelopathies. 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.
In hypokalemic periodic paralysis, the level of potassium in the blood falls during the attack, which also can be precipitated by anything that tends to lower the potassium level. Changes to your diet may help reduce the frequency of episodes of paralysis. Pathophysiology although the mechanism of thyrotoxic periodic paralysis remains uncertain, the two main ingredients that producea paralytic attac. Practical aspects in the management of hypokalemic periodic. However, you will still need to take your potassium tablets as prescribed by your doctor. Elbaz a, valesantos j, jurkatrott k, lapie p, ophoff ra, bady b, et al. The patients paralysis resolved upon repletion of his low potassium and he was. Hypokalemic periodic paralysis hokpp is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels hypokalemia. Hypokalemic periodic paralysis and the dihydropyridine receptor cacnl1a3.
We studied the incidence, etiology, clinical profile, and acidbase disturbances in patients presenting with hypokalemic paralysis and analyzed the significance of periodic and nonperiodic forms. Hypokalemic periodic paralysis genetics home reference nih. Apr 30, 2018 a clinically useful classification of primary periodic paralyses, shown in table 1, includes hypokalemic, hyperkalemic, and paramyotonic forms. Gamstorp disease, also known as hyperkalemic periodic paralysis, is a rare genetic condition that causes episodes of muscle weakness or temporary paralysis. Apr 30, 2018 ryan dp, da silva mr, soong tw, et al. Hypokalemia medical specialties clinical medicine scribd. Primary periodic paralysis ppp is a group of rare diseases that temporarily make muscles stiff, weak, or unable to move. Hypokalemic periodic paralysis pathology britannica.
Hypokalemic and normokalemic are two kinds of this genetic problem. Hypokalemic periodic paralysis in emergency department. Hypokalemic periodic paralysis has been related to mutation in the cacnl1a3, scn4a, or kcne3 11qq14 gene and its characterized by attacks that tend to occur on awakening after exercise or after a heavy meal and may last for several days. Outline causes of primary periodic paralysis causes of secondary periodic paralysis presentation of hyperkalemic pp comparison between hyper pp vs hypo pp. Hypokalemia, one of the most common electrolyte disturbances, is diagnosed if plasma potassium concentration is less than 3. These episodes can last from a few minutes to a few days, depending on.
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. Medically accurate, uptodate information about the periodic paralyses accessible to patients, caretakers and physicians. Periodic paralyses hyperkalemic, hypokalemic, andersentawil syndrome what is periodic paralysis. Dietary guidelines for hypokalemic periodic paralysis. Periodicparalysis differential diagnosis and important. 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. Case presentation hypokalemia clinical medicine medicine scribd. Case presentation hypokalemia free download as powerpoint.
1556 164 550 1325 1553 208 86 1493 7 1497 585 1330 1382 1112 1013 263 900 842 1346 1511 349 538 760 651 1381 1052 1098 768 1026 1175 946 1108 1019 1039 695 235 438