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 c k ribosomes v. It should be excluded in patients presenting after age 20 years or without a family history, by looking for a suppressed thyroid stimulating hormone tsh and elevated free t4 or free t3. The pathophysiology of the paralysis remains unclear. Hyper and hypokalemic periodic paralysis study national. The first attack usually occurs in childhood or adolescence. However, you will still need to take your potassium tablets as prescribed by your doctor. If you have problems viewing pdf files, download the latest version of adobe reader.
Hypokalemic paralysis was predominantly seen in younger males. Changes to your diet may help reduce the frequency of episodes of paralysis. The patient presented to the ed complaining of two days of severe. Hyperkalemic periodic paralysis an overview sciencedirect. Hypokalaemic periodic paralysis is episodic painless muscle weakness associated with areflexia 1.
Oct 21, 2008 hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness. Clinical features hypokalaemic periodic paralysis mim170400 hypopp is the most common pp, with a prevalence of. Clinical profile in hypokalemic periodic paralysis cases electronic. Outline causes of primary periodic paralysis causes of secondary periodic paralysis presentation of hyperkalemic pp comparison between hyper pp vs hypo pp. We report the case of a patient that was hospitalized in neurologys department in habib. It can help shorten the diagnostic journey and expedite clinical management of the disease. This response has been maintained for periods of 16 to 43 months with minimal or no side effects. The payee may view, download, andor print the article for hisher personal, scholarly, research, and.
Hypokalaemic paralysis is occasionaly seen in patients with hyperaldosteronism 1. Hypokalemic periodic paralysis is characterized by a fall in potassium levels in 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. Primary hyperaldosteronism presenting as hypokalemic. We have also a guidance booklet for hypokk hypokalemic periodic paralysis owners manual. Abstract hypokalemic periodic paralysis hypopp is an autosomal dominant disorder belonging to a group of muscle diseases known to.
Hypokalemic periodic paralysis an overview sciencedirect topics. Hypokalemic periodic paralysis in emergency department. Clinical profile in hypokalemic periodic paralysis cases abstract the present article was aimed to study demographic and clinical pattern, periodicity and precipitating events for hypokalemic paralysis and to assess the response to treatment both during acute attacks and as prophylaxis in comparison with available literature. Hypokalemic periodic paralysis genetics home reference nih.
Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Management considerations in hypokalemic periodic paralysis include accurate diagnosis, potassium dosage for acute attacks, choice of. Glucocorticoid induced hypokalemic periodic paralysis. Characterization of hyperkalemic periodic paralysis. The most common form of periodic paralysis in humans is hypokalemic periodic paralysis and is reported to have the prevalence of 1. 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.
We are presenting a case of hypokalemic paralysis in a patient who presented to the emergency department ed with a unique clinical picture that did not fully fit with other causes of periodic paralysis hypokalemic periodic paralysis, thyrotoxic periodic paralysis, hyperkalemic periodic paralysis, and anderson syndrome. The two most common types of periodic paralyses are. Many levels stay the same and should not be used as a guideline for diagnosis of an episode. I report a case of a 33yearold caucasian man who presented with sudden onset paralysis, following previous similar presentations without investigation. Among the hypokalemic periodic paralysis, familial hypokalemic periodic paralysis fpp is the most common cause in western countries, and thyrotoxic periodic paralysis tpp, characterized by the triad of acute hypokalemia without total body potassium deficit, muscle paralysis, and thyrotoxicosis, is the most common cause in asia. It is characterized by muscle hyperexcitability or weakness which, exacerbated by potassium, heat or cold, can lead to uncontrolled shaking followed by paralysis. Printable pdf version download here dec 2017 this article focuses on questions that arise about diagnosis and treatment for people with hypokalemic periodic paralysis. 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. Acute hypokalemic periodic paralysis can be prima ry or familial periodic paralysis, and secondary periodic paralysis.
Introduction hypokalemic periodic paralysis hopp is a wellknown clinical entity, but many etiological and neurophysiological problems remain. A 23 years old man arrives at the emergency department with paralysis of the limbs, trunk and neck, without myotonia. It is classified as hypokalemic or hyperkalemic periodic paralysis according to serum potassium levels during attacks. 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. The mechanism of effect of acetazolamide was not discovered. 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. Periodic paralysis pp is a muscle disease that causes episodic muscle weakness, in the family of diseases called channelopathies. Thyrotoxic periodic paralysis hyperaldosteronism rta villous adenoma cocaine binge diuretics, licorice, steroids, etoh hyperkalemic k5. For a general phenotypic description and a discussion of genetic heterogeneity of hokpp, see hokpp1, which is caused by mutation in the cacnl1a3. Hypokalaemia periodic paralysis liam j stapleton, 2018. Pdf hypokalemic periodic paralysis is a rare genetic disorder characterized by recurrent attacks of skeletal muscle. Attacks are more severe than in hyperakalemic periodic paralysis. There is an increased risk of pre and post anaesthetic paralysis.
Clinical profile in hypokalemic periodic paralysis cases. 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. The medical name for low potassium level is hypokalemia. 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. 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. Acetazolamide produced a mild metabolic acidosis but did not have a demonstrable effect on total body sodium, total body potassium, or thyroid function. Thyrotoxic periodic paralysis genetic and rare diseases. Hypokalaemic paralysis is a relatively uncommon but potentially lifethreatening clinical syndrome.
Approval was based on 2 randomized, doubleblinded placebocontrolled studies that included 8 patients. See also hokpp2, which is caused by mutation in the scn4a gene. Hypertension and hypokalaema are the classic presentation of primary hyperaldoteronism though some patients can be normokalaemic 2. Pubmed is a searchable database of medical literature and lists journal articles that discuss thyrotoxic periodic paralysis. 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. Dietary guidelines for hypokalemic 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 hokpp is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels hypokalemia. Participants will be randomly assigned to one of two treatment groups. Hyperkalemic periodic paralysis genetic and rare diseases. Hypokalemic periodic paralysis is a rare and dramatic complication of hyperthyroidism.
Practical aspects in the management of hypokalemic periodic. The syndrome of hypokalaemic paralysis represents a heterogenous group of disorders characterised clinically by hypokalaemia and acute systemic weakness. Hypokalemic periodic paralysis 5minute clinical consult. Hypokalemic periodic paralysis an overview sciencedirect. Hypokalemic periodic paralysis is a rare heterogeneous disorder characterized by. 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. Clinical and etiological spectrum of hypokalemic periodic paralysis. Approximately onethird of cases may be new dominant mutations m. Click on the link to view a sample search on this topic. Hypokalemic periodic paralysis hypopp or hypokpp is a rare disorder in which a person experiences episodes of painless muscle weakness and often paralysis. Episodes typically involve a temporary inability to move muscles in the arms and legs.
Hypokalemic periodic paralysis is a channelopathy caused by defective sodium, calcium or potassium ionchannels. Elbaz a, valesantos j, jurkatrott k, lapie p, ophoff ra, bady b, et al. Hypokalemic periodic paralysis and influenza jama jama. Pp is classified as hypokalemic when episodes occur in association with low potassium blood levels or as. The associations allocation for research on hyperkalemic and hypokalemic periodic paralysis. The patient presented with sudden onset paralysis of his extremities. Periodic paralysis an overview sciencedirect topics. Genetic heterogeneity in hypokalemic periodic paralysis uni ulm. Hypokalemic periodic paralysis children with permanent myopathic weakness. General information about hypokalemic periodic paralysis. Familial periodic paralyses information page national. We report this case in order to reemphasize this observation and to note its presentation as a form of hypokalemic periodic paralysis. A rare heterogeneous group of disorders which is characterized by a sudden onset of reversible muscle paralysis. Laboratory evaluation revealed a markedly low potassium level.
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. 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. Hypokalaemic paralysis postgraduate medical journal. Diagnosis of familial or primary hypokalaemic periodic paralysis is. 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. Newer discoveries led to the definition of the new categories making hypokalemic periodic paralysis in the category of channelopathies. A periodic paralysis gene panel test can bring you and your patients closer to uncovering the genetic cause behind the attacks of paralysis and muscle weakness caused by hyperkalemic or hypokalemic periodic paralysis. Factors that cause a decrease in serum potassium will precipitate attacks of paralysis. Mutations in the scn4a gene can also cause hyperkalemic periodic paralysis hypp. A 66yearold white woman suffered an influenzalike illness one month prior to admission, with chills, fever, muscleaching, burning of eyes, and cough. 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 genetic and rare diseases. The patients paralysis resolved upon repletion of his low potassium and he was. Journal of contraceptive studies imedpub journals 2016.
The trial consists of two 9week studiesone study will enroll persons with hyperkalemic periodic paralysis and the other study will enroll persons with hypokalemic periodic paralysis. Introduction hypokalemic periodic paralysis hpp is characterized by muscle weakness secondary to low serum potassium levels. Episodes of paralysis can occur in only certain areas of the body, like an arm or leg, or the whole body. Anaesthetic management of a patient with hypokalemic periodic. Review of the diagnosis and treatment of periodic paralysis. Hyperkal periodic paralysis triggers open anesthesia. Hypokalemia is usually not an absolute deficiency of total body potassium but, rather, a rapid and exaggerated intracellular shifting of potassium into muscles during attacks. Thyrotoxic periodic paralysis resembles hypokalemic periodic paralysis, except that it occurs sporadically and the associated hypokaliemia is often profound. Recurrent hypokalemic paralysis is an extremely unusual presentation of hypothyroidism. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. Periodicparalysis differential diagnosis and important.
Hypokalemic periodic paralysis is one form of periodic paralysis, a rare group of disorders that can cause of sudden onset weakness. Hypokalemic periodic paralysis secondary to renal tubular acidosis. 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. Hypokalemic periodic paralysis and the dihydropyridine receptor cacnl1a3. Thyrotoxicosis is the most common cause of secondary hypokalemic periodic paralysis. Hypokalemic periodic paralysis is an autosomaldominant disorder caused most commonly by mutations in the alpha subunit of the skeletal muscle calcium channel gene cav1. Twelve patients with hypokalemic periodic paralysis were treated with acetazolamide in a placebocontrolled trial. Vomiting, diarrhea were important precipitating factors. Novel etiopathophysiological aspects of thyrotoxic periodic. The primary pp is due to inherited ion channel defects while secondary pp is due to other medical conditions like thyrotoxicosis.
These episodes can last from a few minutes to a few days, depending on. He has a serum concentration of potassium 1,6 meql and ecg changes prolonged pr and flattening of t waves. Severe hypokalemic paralysis and rhabdomyolysis occurring. Periodic paralysis definition of periodic paralysis by. The periodic paralysis international site contains a booklet that can help you out. Inheritance is autosomal dominant with reduced penetrance in women fontaine, 1994. Acetazolamide treatment of hypokalemic periodic paralysis. Cureus unusual clinical presentation of periodic paralysis. 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. Apr 30, 2002 hypokalemic periodic paralysis hypopp is a condition in which affected individuals may experience paralytic episodes with concomitant hypokalemia serum potassium mar 27, 2019 the two most common types of periodic paralyses are. Whereas the majority had failed to improve with previous therapy, 10 of the 12 patients were dramatically improved by acetazolamide. 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 paral trigger open anesthesia.
Unlike those with hypokalemic periodic paralysis hypopp, those with hyperpp infrequently experience generalized flaccid paralysis but rather. Hypokalemic periodic paralysis genetic and rare diseases nih. Hypokalemic periodic paralysis, in which serum potassium drops during a paralytic episode, is the. The 2 most common forms of hypokalemic paralysis are thyrotoxic and familial periodic hypokalemic paralysis. Muscle weakness during an attack usually affects the arms and legs and muscles of the eyes, throat, and trunk. Primary periodic paralyses include hypokalemic paralysis hypopp, hyperkalemic paralysis hyperpp, and andersen.
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. 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. Primary periodic paralysis ppp is a group of rare diseases that temporarily make muscles stiff, weak, or unable to move. Hypokalemic periodic paralysis free download as powerpoint presentation. Thyrotoxic periodic paralysis tpp, the most commonly seen disorder among asian men, is characterized by abrupt onset of hypokalemia and. Hypokalemic periodic paralysis hypopp is a condition in which affected individuals may experience paralytic episodes with concomitant hypokalemia serum potassium hypokalemic periodic paralysis. The perioperative management of a patient with hypokalemic periodic paralysis is challenging in view of the increased risk of paralysis. The patients paralysis resolved upon repletion of his low potassium.
Attacks cause severe weakness or paralysis that usually lasts from hours to days. If recognised and treated appropriately, patients recover without any clinical sequellae. Acetazolamide is the most effective treatment available for hypokalemic periodic paralysis. Unlike in hyperakalemic periodic paralysis, emg does not usually show myotonia. Hypokalemic and normokalemic are two kinds of this genetic problem. Anaesthetic management of a patient with hypokalemic.
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