What is periodic hypokalemic periodic paralysis?
Summary Summary. Hypokalemic periodic paralysis (HOKPP) is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels (hypokalemia). Episodes typically involve a temporary inability to move muscles in the arms and legs. The first attack usually occurs in childhood or adolescence.
What are the signs and symptoms of periodic paralysis?
These conditions are associated with either recurrent episodes of hypokalemia, episodic attacks of muscle weakness, and weakness or stiffness associated with exercise. Thus they should be in the mind of the treating physician as they can mimic hypokalemic periodic paralysis.
What causes low potassium levels in periodic paralysis?
People with hypokalemic periodic paralysis typically have reduced levels of potassium in their blood (hypokalemia) during episodes of muscle weakness. Researchers are investigating how low potassium levels may be related to the muscle abnormalities in this condition.
Which conditions are associated with hypokalemia?
Hyperkalemic or normokalemic periodic paralysis Thyrotoxic periodic paralysis Andersen-Tawil syndrome Secondary hypokalemia Myasthenia gravis Paramyotonia congenita These conditions are associated with either recurrent episodes of hypokalemia, episodic attacks of muscle weakness, and weakness or stiffness associated with exercise.
How do you diagnose hypokalemic periodic paralysis?
When there is an established family history of hypokalemic periodic paralysis, no further diagnostic investigations are required to confirm the diagnosis of an episode of a paralytic attack. Otherwise, a low serum potassium level during a typical attack of weakness establishes the diagnosis.
Is hypokalemic periodic paralysis painful?
Hypokalemic periodic paralysis (hypoPP or hypoKPP) is a rare disorder in which a person experiences episodes of painless muscle weakness and often paralysis.
What are the signs and symptoms of periodic paralysis?
What are the symptoms of periodic paralysis?Attacks of muscle weakness that may last for minutes to days.Muscle pain in muscles after exercise.Muscle cramping.Feeling tingles.Permanent weakness, more likely later in life.
Is hypokalemic periodic paralysis a neurological disorder?
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 high-carbohydrate meals.
What triggers hypokalemic periodic paralysis?
Mutations in the CACNA1S or SCN4A gene can cause hypokalemic periodic paralysis. These genes provide instructions for making proteins that play essential roles in muscles used for movement (skeletal muscles). For the body to move normally, skeletal muscles must tense (contract) and relax in a coordinated way.
What kind of doctor treats hypokalemic periodic paralysis?
Division Of Nephrology Their top areas of expertise are Low Potassium Level, Thyrotoxic Periodic Paralysis, Familial Periodic Paralysis, and Hypokalemic Periodic Paralysis.
How do you test for periodic paralysis?
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. It can help shorten the diagnostic journey and expedite clinical management of the disease.
How long does hypokalemia take to recover?
Typical patients with hypokalemia have required a mean of 5 days for return of their serum potassium levels to normal (12,13). Our patient required a daily supplementation of potassium of 40 to 80 mEq for 8 days to maintain normal levels.
Is there a cure for periodic paralysis?
Often, making diet and lifestyle changes and avoiding your triggers can keep you from having attacks. TPP is curable if you treat the thyroid problems that caused it. As you get older and have more episodes, your muscles may get weaker over time.
What cancers cause low potassium?
In SCLC, amongst the important causes of low potassium levels in the blood are adrenocorticotrophic hormone (ACTH)-secreting tumors. Ectopic Cushing's syndrome, secondary to lung cancer, is a rare occurrence with a poor prognosis but may manifest with severe hypokalemia, alongside hyperglycemia and muscle weakness.
Is hypokalemic periodic paralysis fatal?
Thyrotoxic hypokalemic periodic paralysis is a rare but potentially fatal emergency: case report and literature review.
Can hypokalemic periodic paralysis cause death?
The most common form of periodic paralysis in humans is hypokalemic periodic paralysis and is reported to have the prevalence of 1 in 100,000 births, but can be fatal if not treated [1]. Patients' waking up in the morning with weakness is typical. Patients had numbness in four-extremities.
Can hypokalemic periodic paralysis cause death?
The most common form of periodic paralysis in humans is hypokalemic periodic paralysis and is reported to have the prevalence of 1 in 100,000 births, but can be fatal if not treated [1]. Patients' waking up in the morning with weakness is typical. Patients had numbness in four-extremities.
Is hypokalemic periodic paralysis fatal?
Thyrotoxic hypokalemic periodic paralysis is a rare but potentially fatal emergency: case report and literature review.
Is hypokalemic periodic paralysis a disability?
If you or a loved one has been diagnosed with Periodic Paralysis, you may be concerned about your future ability to work. The good news is that the Social Security Administration (SSA) makes disability benefits available for those who are physically unable to earn income due to Periodic Paralysis.
Can you feel if you are paralyzed?
If you have paralysis, you are partly or entirely unable to move the affected parts of the body. Paralysis may be accompanied by a loss of sensation depending on the location of the injury. Strokes and spinal cord injuries cause sudden paralysis. Some medical conditions can cause gradual paralysis.
What is hypokalemic periodic paralysis?
Hypokalemic periodic paralysis (HOKPP) is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels ( hypokalemia ). [1] Episodes typically involve a temporary inability to move muscles in the arms and legs. [2] The first attack usually occurs in childhood or adolescence. Attacks can last for hours or days, and the frequency of attacks varies among people with HOKPP. The frequency is usually highest between the ages of 15 and 35, and then decreases with age. Some people with HOKPP also develop late-onset proximal myopathy. [3]
Is hypokalemic paralysis inherited?
Hypokalemic periodic paralysis (HOKPP) is inherited in an autosomal dominant manner. [3] . This means that having a change ( mutation) in only one copy of one of the responsible genes in each cell is enough to cause symptoms of the condition.
What is hypokalemic periodic paralysis?
Hypokalemic periodic paralysis (HypoKPP) is a rare disorder characterized by the occurrence of episodic severe muscle weakness, usually triggered by strenuous exercise or high carbohydrate diets. HypoKPP episodes are associated with low serum potassium levels. Most cases of the HypoKPP are hereditary or familial. The familial form of HypoKPP is a rare channelopathy caused by the mutation in either of the calcium or sodium ion channels, primarily affecting the skeletal muscle cells. Acquired cases of HypoKPP are also identified and are associated with hyperthyroidism. Hypokalemic periodic paralysis was first described in 1727 by Musgrave, in 1853 by Cavare and in 1857 by Romberg.[1] The disease-causing mutation in HypoKPP, CACNA1S gene, was identified by Jurkat-Rott et al. in 1994.[1]
What causes hypokalemic paralysis?
Familial hypokalemic periodic paralysis is caused by a mutation in either of two genes, calcium or sodium ion channel gene mutation. Over the last few decades, various mutations have been identified as a cause of HypoKPP. The most common familial form, type 1 HypoKPP, has a mutation in the dihydropyridine-sensitive, skeletal muscle calcium channel gene, CACNA1S. While the other familial form, type 2 HypoKPP, has mutations in the voltage-sensitive skeletal muscle sodium channel gene, SCN4A. Disease-causing mutations in the gene KCNJ2 and KCNJ18, code for inward rectifier potassium (Kir) channel, have also been identified. [2][3][4][5][6] Acquired HypoKPP has been associated with thyrotoxicosis. The familial form and thyrotoxic HypoKPP constitute the primary HypoKPP. Periodic muscle weakness can also result from hypokalemia secondary to renal and gastrointestinal potassium loss as in renal tubular acidosis, gastroenteritis, or secondary to endocrine causes.
When does thyrotoxic hypokpp onset?
[2][12] However, in the case of thyrotoxic HypoKPP, the onset is usually after age 20.
How to test for channelopathy?
Between attacks, EMG techniques can be used to demonstrate the change in excitability of muscle fibers due to channelopathy, called the "exercise test." In the long exercise test, an attack of focal muscle weakness is induced by vigorously exercising a single muscle for 2-5 minutes, and the change in postexercise CAMP in muscle fibers is measured by the EMG. The reduction of 40% or more in CAMP is considered abnormal and typical for periodic paralysis. The study showed no false-positive results when the reduction is more than 40% or more, and this change was present in greater than 70% of patients. [12][19][20] The abduction range of the little finger measured postexercise, can be a possible alternative parameter to CAMP in a long exercise test for diagnosis of HypoKPP between attacks of muscle weakness. [21]
How often do you get attacks of weakness?
The frequency of attacks of weakness is very variable and infrequent. Some patients might get attack only once in a lifetime, while others may get it several times a week. [2][14] Women tend to have fewer attacks than men. And the duration of each attack also varies, ranging from minutes to days and can last up to several hours before they resolve spontaneously.
Does carbonic anhydrase inhibitor cause muscle weakness?
Carbonic anhydrase inhibitors seem to be potent in decreasing future attacks of muscle weakness, though the mechanism of CAIs in HypoKPP is still unclear. Carbonic anhydrase inhibitors promote urine potassium loss and non-anion gap metabolic acidosis, which reduce the patient's susceptibility to muscle paralysis. It is also suggested that CAIs increase the opening of the calcium-activated potassium channels. Further, CAIs also reduce intracellular sodium accumulation, thus reducing the cellular toxicity and prevent muscle degeneration, which may be effective in the treatment of permanent weakness. [12] 250 mg twice daily dose of acetazolamide has been effective in lessening the frequency of attacks. [2][7]
Is hypokalemic paralysis a rare disease?
In general, hypokalemic periodic paralysis is a rare disorder and has an estimated prevalence of 1 in 100,000.[7] Most familial cases have an autosomal dominant inheritance pattern with incomplete penetrance. This disorder has lower clinical expression in females because of the lower penetrance and attack rate compared to males.[8] And also the women tend to have fewer attacks of muscle weakness than men. Many cases are sporadic, which represents new mutations. [2][9]
What is hypokalemic paralysis?
Description. Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. Attacks cause severe weakness or paralysis that usually lasts from hours to days.
How many people are affected by hypokalemic paralysis?
Although its exact prevalence is unknown, hypokalemic periodic paralysis is estimated to affect 1 in 100,000 people. Men tend to experience symptoms of this condition more often than women.
What is the cause of muscle weakness?
The altered channels are "leaky," allowing ions to flow slowly but continually into muscle cells, which reduces the ability of skeletal muscles to contract. Because muscle contraction is needed for movement, a disruption in normal ion transport leads to episodes of severe muscle weakness or paralysis.
Can you have a muscle weakness in the morning?
Often, a large , carbohydrate-rich meal or vigorous exercise in the evening can trigger an attack upon waking the following morning. Although affected individuals usually regain their muscle strength between attacks, some develop persistent muscle weakness later in life. People with hypokalemic periodic paralysis typically have reduced levels ...
What is hypokalemic periodic paralysis?
Hypokalemic periodic paralysis is an inherited condition characterized by transient episodes of extreme muscle weakness and/or paralysis of the muscles in the arms and legs, along with a decreased serum potassium concentration during an attack. These episodes may last from a few hours to one day.
Common symptoms reported by people with hypokalemic periodic paralysis
Reports may be affected by other conditions and/or medication side effects. We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition.
Treatments taken by people for hypokalemic periodic paralysis
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Compare treatments taken by people with hypokalemic periodic paralysis
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What are the symptoms of familial hypokalemic periodic paralysis?
Some signs and symptoms noted at the PPA Conference that may also be part of familial hypokalemic periodic paralysis (but have not been formally validated) are: muscle pain with and after attacks, mental confusion with attacks, having an off-mood/bad mood/irritability during attacks, non-restorative sleep with attacks,
When do you start having symptoms of hypokalemic paralysis?
People who have hypokalemic periodic paralysis usually begin having symptoms during their childhood or teen years. In many cases, they have never heard of periodic paralysis and are not sure why they are having attacks of muscle weakness.
How to diagnose hypokalemic periodic paralysis?
However, genetic testing is not perfect and does not always detect all cases. A muscle biopsy is sometimes needed for doctors to diagnose periodic paralysis. This means that the doctor must remove and examine a small piece of the patient’s muscle tissue during or after an attack to see if the muscle tissue is abnormal. Another diagnosis method is the Compound Muscle Action Potential test , which measures the electrical activity of muscles. This test can’t always detect periodic paralysis, but it can do so in a lot of cases.
What is the most common type of periodic paralysis?
Hypokalemic periodic paralysis (HypoPP) is the most common type of periodic paralysis. In this type, low blood potassium levels trigger attacks, and potassium levels usually drop during attacks as well. “Hypo” means that there is a low amount of something, and “kalemic” refers to potassium.
How often do you have a hypokalemic paralysis attack?
Hypokalemic periodic paralysis causes sufferers to have attacks of extreme muscle weakness or paralysis. These attacks may last from a few hours to a few days. The frequency of attacks can vary greatly between different people. Some people have attacks every day, others have them several times per month, while there are others who have them every few months or less often. The strength of the attacks isn’t always consistent — sometimes, the patient feels fatigued, but can still move around slowly. Other times, they aren’t able to move at all and they may seem like they are unconscious (even though they really are conscious). Attacks may affect one area, like an arm or leg, or many areas at once around the body.
What is hypokalemic paralysis?
Hypokalemic Periodic Paralysis. Periodic paralysis is a rare genetic disorder that is characterized by temporary attacks of extreme muscle weakness or paralysis. These attacks can be triggered by a variety of things, with the most common triggers being resting after exercise, cold temperatures, and having too much or too little potassium.
What medications can help with paralysis?
In addition, there are medications that can help, such as acetazolamide, methazolamide, spironolactone, and dichlorphenamide ( KEVEYIS, the only FDA-approved periodic paralysis treatment). Different patients will have different combinations of medications that are right for them.
How to treat hypokalemic paralysis?
Treatment of hypokalemic periodic paralysis focuses on preventing further attacks and relieving acute symptoms. Avoiding carbohydrate-rich meals, strenuous exercise and other identified triggers, and taking acetazolamide or another carbonic anhydrase inhibitor, may help prevent attacks of weakness.
What is hypokalemic periodic paralysis?
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. In individuals with this mutation, ...
How often do you have a hypokalemic paralysis attack?
Attacks cause severe weakness or paralysis that usually lasts from hours to days. Some people may have episodes almost every day, while others experience them weekly, monthly, or only rarely. Attacks can occur without warning or can be triggered by factors such as rest after exercise, a viral illness, or certain medications. Often, a large, carbohydrate-rich meal or vigorous exercise in the evening can trigger an attack upon waking the following morning. Although affected individuals usually regain their muscle strength between attacks, some develop persistent muscle weakness later in life.
How does hypokalemia affect the body?
Hypokalemic periodic paralysis is a condition that causes episodes of extreme muscle weakness typically beginning in childhood or adolescence. Most often, these episodes involve a temporary inability to move muscles in the arms and legs. Attacks cause severe weakness or paralysis that usually lasts from hours to days. Some people may have episodes almost every day, while others experience them weekly, monthly, or only rarely. Attacks can occur without warning or can be triggered by factors such as rest after exercise, a viral illness, or certain medications. Often, a large, carbohydrate-rich meal or vigorous exercise in the evening can trigger an attack upon waking the following morning. Although affected individuals usually regain their muscle strength between attacks, some develop persistent muscle weakness later in life.
How long does a paralysis attack last?
During an attack, reflexes may be decreased or absent. Attacks may last for a few hours or persist for several days. Recovery is usually sudden when it occurs, due to release of potassium from swollen muscles as they recover.
What is the best IV solution for paralysis?
Normal saline is the preferred IV solution for patients with familial hypokalemic periodic paralysis. Glucose containing solutions may cause weakness. Additionally, the high chloride content can cause a mild acidosis which would be preferred over alkalosis.
What happens when calcium is pumped away from the contractile apparatus?
Calcium is pumped away from the contractile apparatus and the muscle relaxes. Mutations altering the usual structure and function of these channels therefore disrupts regulation of muscle contraction, leading to episodes of severe muscle weakness or paralysis.