What triggers hyperkalemic periodic paralysis?

Hyperkalemic PP is a muscle disease that has onset in infancy or early childhood and is manifested by transient episodes of paralysis, usually precipitated by cold exposure, rest after exercise, fasting, or the ingestion of small amounts of potassium [2,3].

What happens in hyperkalemic periodic paralysis?

In hyperkalemic periodic paralysis, high levels of potassium in the blood interact with genetically caused abnormalities in sodium channels (pores that allow the passage of sodium molecules) in muscle cells, resulting in temporary muscle weakness and, when severe, in temporary paralysis.

What causes familial periodic paralysis?

In the hypokalemic form, the paralysis is caused by low levels of potassium. In the hyperkalemic form, the paralysis is caused by high levels of potassium in the blood. In the thyrotoxic form, the paralysis is caused by low levels of potassium in the blood and an overactive thyroid gland (hyperthyroidism).

What type of mutation causes hyperkalemic periodic paralysis?

Mutations in the SCN4A gene can cause hyperkalemic periodic paralysis. The SCN4A gene provides instructions for making a protein that plays an essential role in muscles used for movement (skeletal muscles).

How long does periodic paralysis last?

Hyperkalemic PP often starts by age 10. Attacks of skeletal muscle weakness last an average of 30 minutes to 4 hours. The attacks tend to be frequent but less severe than in other PP forms. As you get older, you may get fewer attacks, but you also may have permanent muscle damage that slowly gets worse.

Is there a cure for Hyperkalemic periodic paralysis?

Can hypokalemic periodic paralysis attacks be prevented? Although hypoPP can’t be prevented, you can take steps to reduce how often you experience an episode and help reduce the severity.

Is Hyperkalemic periodic paralysis life threatening?

Hypokalemic periodic paralysis is a rare life-threatening syndrome, potentially reversible when detected at an early stage. Hypokalemia may also occur in other conditions characterized by muscle weakness.

Is there a cure for periodic paralysis?

Although the treatment of choice in periodic paralysis is generally considered to be acetazolamide, there is no standardised treatment regimen and no consensus as to when to start treatment. We do not know if acetazolamide treatment prevents any permanent weakness that may occur.

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 paralysis always permanent?

While paralysis is not always a permanent condition, it can still affect you for a very long time. You may require significant medical treatment and rehabilitation to recover from paralysis, as well as spend a long time out of the workplace.

Is hypokalemic periodic paralysis rare?

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.

Is Hyperkalemic periodic paralysis a genetic disease?

Hyperkalemic periodic paralysis is a genetic disease that causes episodes of extreme muscle weakness and an increase of the potassium levels in the blood.

What causes familiar periodic paralysis?

There are 4 forms of familial periodic paralysis: hypokalemic, hyperkalemic, thyrotoxic, and Andersen-Tawil syndrome. In the hypokalemic form, the paralysis is caused by low levels of potassium. In the hyperkalemic form, the paralysis is caused by high levels of potassium in the blood.

What is the treatment for periodic paralysis?

Treatment depends upon the type of periodic paralysis syndrome you have. Treatment therapies include medications such as dichlorphenamide (Keveyis), oral and/or IV potassium, acetazolamide, and diuretics. Some patients require beta blocker medication, amiodarone (Cordarone), and other drugs.

How is periodic paralysis diagnosed?

Periodic paralysis syndrome is diagnosed by family history, characteristic symptoms, laboratory tests, muscle biopsies, muscle cooling and/or muscle exercise, and repetitive stimulation of the nerves electrically; and in some people, provocative testing.

What is periodic paralysis syndrome?

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.