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. To study the etiology, varied presentations, and outcome after therapy of patients with hypokalemic paralysis. Hyperkalemic periodic paralysis is a genetic disease that causes episodes of extreme muscle weakness and an increase of the potassium levels in the blood. Approval was based on 2 randomized, doubleblinded placebocontrolled studies that included 8 patients. Laboratory evaluation revealed a markedly low potassium level.
Periodic paralyses hyperkalemic, hypokalemic, andersentawil syndrome what is periodic paralysis. Hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness. Acquired nonthyrotoxic hypokalemic periodic paralysis edorium. Pp is classified as hypokalemic when episodes occur in association with low potassium blood levels or as. Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Differentiating hypokalemic periodic paralysis hpp from. Hypokalemic periodic paralysis genetics home reference nih. Hyperkalemic periodic paralysis genetics home reference. List of hypokalemic periodic paralysis medications 3. Thyrotoxic periodic paralysis tpp is a rare complication of hyperthyroidism characterized by episodes of muscle weakness and hypokalemia. Periodic paralysis syndrome describes seven to eight different types of rare genetic conditions that cause symptoms of episodic muscle weakness and paralysis. Thyrotoxic periodic paralysis is a condition in which there are episodes of severe muscle weakness. The medical name for low potassium level is hypokalemia. Periodic paralyses hyperkalemic, hypokalemic, andersen.
However, its mechanism of efficacy is not fully understood, and it is not known whether therapy response relates to genotype. Mutations in the scn4a gene can also cause hyperkalemic periodic paralysis hypp. Hypokalemic periodic paralysis hypopp or hypokpp is a rare disorder in which a person experiences episodes of painless muscle weakness and often paralysis. The best tests to diagnose hypokalemic periodic paralysis are measuring the blood potassium level during an attack of paralysis and checking for known gene mutations.
Hypokalemic periodic paralysis is an autosomaldominant disorder caused most commonly by mutations in the alpha subunit of the skeletal muscle calcium channel gene cav1. Only a few cases of phpp have been reported in indian literature in adultsl. Brody and dudley 1969 found no differences from the normal while au and yeung 1972 noted a significant decrease in the amount of calcium bound during the attacks in patients with thyrotoxic hypokalemic periodic paralysis. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. Description periodic paralysis disorders are genetic disorders that affect muscle strength. Hypokalemic paralysis was predominantly seen in younger males. Hypokalemic periodic paralysis and spectrum of thyroid disorders. Periodicparalysis differential diagnosis and important. Clinical profile in hypokalemic periodic paralysis cases electronic.
A clinically useful classification of primary periodic paralyses, shown in table 1, includes hypokalemic, hyperkalemic, and paramyotonic forms. 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. 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. Pdf hypokalemic thyrotoxic periodic paralysis in female. Practical aspects in the management of hypokalemic. Renal tubular acidosis, types 1 and 2 liddles syndrome bartters syndrome gitelmans syndrome hypomagnesemia. Hypokalemic periodic paralysis hpp, a lifethreatening. Hyperthyroid periodic paralysis hpp is a rare life threatening complication of hyperthyroidism commonly occurring in young asian males but sporadically found in other races. Clinical and biochemical spectrum of hypokalemic paralysis. Acetazolamide efficacy in hypokalemic periodic paralysis. Changes to your diet may help reduce the frequency of episodes of paralysis. Attacks are more severe than in hyperakalemic periodic paralysis. Thyrotoxic hypokalemic periodic paralysis in a hispanic male article pdf available in journal of the national medical association 945. Acute hypokalemic paralysis, characterized by acute flaccid paralysis is primarily a calcium channelopathy, but secondary causes like renal tubular acidosis rta, thyrotoxic periodic paralysis tpp, primary hyperaldosteronism, gitelmans syndrome are also frequent.
The first attack usually occurs in childhood or adolescence. Acquired nonthyrotoxic hypokalemic periodic paralysis. 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. 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. Specifically, ekg, tsh, free t3, and free t4 are the minimum indicated labs. 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. Hypokalemic periodic paralysis genetic and rare diseases. Pdf thyrotoxic hypokalemic periodic paralysis tpp is a condition. Episodes typically involve a temporary inability to move muscles in the arms and legs. Biochemical studies measuring the calciumbinding capacity of sr have produced equivocal results. Positive long exercise test see clinical testing available.
Secondary periodic paralysis a demonstrably known causes serum potassium abnormal even in interictal phase 7. Various laboratory examinations outside of ictal and interictal serum potassium measures play an important role in both diagnosis and management of hypokalemic periodic paralysis. The attacks of weakness are similar to those of familial hypokalemic periodic paralysis fhpp except for the presence of hyperthyroidism table 2. Thyrotoxic hypokalemic periodic paralysis thpp is a rare, potentially life threatening endocrine emergency. Hypokalemic periodic paralysis is an autosomal dominant disorder, which means that one abnormal copy of the gene is all that is needed to have symptoms. It occurs in people who have high levels of thyroid hormone in their blood hyperthyroidism, thyrotoxicosis. Hypokalemic periodic paralysis hokpp is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels hypokalemia.
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. Periodic paralysis an overview sciencedirect topics. Hypokalemic periodic paralysis free download as powerpoint presentation. See also hokpp2, which is caused by mutation in the scn4a gene. Familial periodic paralyses information page national. Pdf thyrotoxic hypokalemic periodic paralysis in a.
Periodic paralysis pp is a muscle disease that causes episodic muscle weakness, in the family of diseases called channelopathies. In children hypokalemic paralysis secondary to gastroenteritis and chronic renal disease is much more com mon than primary disease2. 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 electrolyte disturbance, k, mg, ca hypokalemic periodic paralysis. Hypokalemic paralysis often referred to as familial is caused. Periodic paralysis is caused by a transient failure of muscle fiber excitability 4,5. Acetazolamide has been the most commonly used treatment for hypokalemic periodic paralysis since 1968. Vomiting, diarrhea were important precipitating factors.
Primary hypokalemic periodic paralysis indian pediatrics. There are two major forms, hypokalemic and hyperkalemic, each caused by. The patient presented with sudden onset paralysis of his extremities. The ninds conducts and supports research on neuromuscular disorders such as the familial periodic paralyses. In a subset of patients, hypokalemic periodic paralysis is triggered by high levels of thyroid hormone hyperthyroidism and is called thyrotoxic periodic paralysis tpp.
Part f is the thyroid involved in your patients hypokalemic periodic paralysis. Outline causes of primary periodic paralysis causes of secondary periodic paralysis presentation of hyperkalemic pp comparison between hyper pp vs hypo pp. Hyperkal periodic paralysis triggers open anesthesia. Muscle weakness during an attack usually affects the arms and legs and muscles of the eyes, throat, and trunk. Elbaz a, valesantos j, jurkatrott k, lapie p, ophoff ra, bady b, et al. These studies are aimed at increasing knowledge about these disorders and finding ways to prevent, treat, and cure them. 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 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. Unlike in hyperakalemic periodic paralysis, emg does not usually show myotonia. Periodic paralysis, any of the forms of a rare disorder that is characterized by relatively shortterm, recurrent attacks of muscle weakness. Thyrotoxic periodic paralysis tpp, a disorder most commonly seen in asian men, is characterized by abrupt onset of hypokalemia and paralysis. Typically, this means that one parent has the disease, but it is possible to have the gene as a result of a new mutation not.
It is characterised by hypokalemia and acute onset paraparesis with prevalence of one in one hundred thousand 1 in 00. Surgical treatment for thyrotoxic 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. Dietary guidelines for hypokalemic periodic paralysis. However, you will still need to take your potassium tablets as prescribed by your doctor. Hypokalemic and normokalemic are two kinds of this genetic problem. The patients paralysis resolved upon repletion of his low potassium and he was. Episodes of paralysis can occur in only certain areas of the body, like an arm or leg, or the whole body. Hyperkalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness or paralysis, usually beginning in infancy or early childhood. Conventional classification of periodic paralysis primary or familial periodic paralysis. Treatment for hypokalemic periodic paralysis in gurgaon, find doctors near you. The condition primarily affects the lower extremities and is secondary to thyrotoxicosis.
Nonetheless, whereas fhpp is an autosomal dominant condition that affects mainly caucasians 17, 18, tpp is a sporadic disease found mainly in asian males. Episodes tend to increase in frequency until midadulthood, after which they occur less frequently in many people with the condition. Hypokalemic periodic paral trigger open anesthesia. Paralysis attack hypokalemic periodic paralysis youtube. 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. What tests are used to diagnose hypokalemic periodic paralysis. Attacks cause severe weakness or paralysis that usually lasts from hours to days. Hypokalemic thyrotoxic periodic paralysis htpp is a rare disorder, which is common among male asian descents though hyperthyroidism is commoner among females. It is characterized by muscle hyperexcitability or weakness which, exacerbated by potassium, heat or cold, can lead to uncontrolled shaking followed by paralysis. These episodes can last from a few minutes to a few days, depending on. Many levels stay the same and should not be used as a guideline for diagnosis of an episode.
The proband experienced a first paralytic attack at age 14 on awakening in the morning, and was found to have a serum potassium of 2. Primary hypokalemic periodic paralysis phpp is a rare entity first described by shakanowitch in 1882. Primary periodic paralysis ppp is a group of rare diseases that temporarily make muscles stiff, weak, or unable to move. Exclusion of other causes of hypokalemia renal, adrenal, thyroid. 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.
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