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Familial Periodic Paralysis


Michael Rubin

, MDCM, New York Presbyterian Hospital-Cornell Medical Center

Reviewed/Revised Jan 2022 | Modified Sep 2022

Familial periodic paralysis is a rare inherited disorder that causes sudden attacks of weakness and paralysis. There are four different forms, which involve abnormalities in how electrolytes Overview of Electrolytes Well over half of the body's weight is made up of water. Doctors think about the body's water as being restricted to various spaces, called fluid compartments. The three main compartments are... read more , such as sodium and potassium, are moved in and out of cells.

  • Muscles do not respond normally to stimulation, usually when the blood potassium level is too low or high.

  • Weakness is intermittent, affecting mainly the limbs, and is often brought on by exercising or eating too many or too few carbohydrates.

  • The diagnosis is based on the symptoms, a check of the potassium level in the blood, and the results of an exercise test.

  • Avoiding triggers that cause attacks and taking certain drugs can prevent attacks effectively.

Attacks of familial periodic paralysis occur in response to the amount of potassium in a person's blood. Potassium is an electrolyte Overview of Electrolytes Well over half of the body's weight is made up of water. Doctors think about the body's water as being restricted to various spaces, called fluid compartments. The three main compartments are... read more and is necessary for the normal functioning of cells, nerves, and muscles (see Overview of Potassium's Role in the Body Overview of Potassium's Role in the Body Potassium is one of the body's electrolytes, which are minerals that carry an electric charge when dissolved in body fluids such as blood. (See also Overview of Electrolytes.) Most of the body’s... read more ). During an attack of familial periodic paralysis, muscles do not respond to normal nerve impulses or even to artificial stimulation with an electronic instrument.

The precise form that the disorder takes varies among different families. There are four forms:

  • Hypokalemic

  • Hyperkalemic

  • Thyrotoxic

  • Andersen-Tawil syndrome

In Andersen-Tawil syndrome, potassium levels can be high, low, or normal.

Symptoms of Familial Periodic Paralysis

During an attack of weakness, the person remains completely awake and alert. Muscles in the eye and face are not affected. Weakness may affect only certain muscles or all four limbs.

In the hypokalemic form, attacks generally first appear before age 16 but may appear during the 20s and always by age 30. The attacks last up to 24 hours. Often, the person awakens the day after vigorous exercise with an attack of weakness. The weakness may be mild and limited to certain muscle groups or may affect all four limbs. However, eating meals rich in carbohydrates (sometimes hours or even the day before), emotional or physical stress, alcohol ingestion, and exposure to cold can also cause attacks. Eating carbohydrates and exercising vigorously drive sugar into cells. Potassium moves with the sugar, and the result is lowered potassium levels in the blood and urine.

In the hyperkalemic form, attacks often begin by age 10. The attacks last 15 minutes to 1 hour. Weakness tends to be less severe than in the hypokalemic form. Fasting, rest shortly after exercise, or exercise after meals may trigger attacks. Myotonia (very stiff muscles caused by a delayed ability to relax the muscles after contracting them) is common. Myotonia of the eyelids may be the only symptom.

In the thyrotoxic form, attacks of weakness last hours to days and are usually triggered by exercise, stress, or eating foods rich in carbohydrates, similar to the hypokalemic form. People have symptoms of hyperthyroidism, such as anxiety, tremors, palpitations, and heat intolerance.

Diagnosis of Familial Periodic Paralysis

  • Description of an attack

  • Level of potassium in the blood during an attack

  • Exercise test

  • Genetic testing

A doctor’s best clue to the diagnosis of familial periodic paralysis is a person’s description of a typical attack. If possible, the doctor draws blood while an attack is in progress to check the level of potassium. If the level of potassium is abnormal, doctors usually do additional tests to be sure the abnormal level is not the result of other causes.

In the past, doctors based the diagnosis on provocative testing. In provocative testing, doctors give a person drugs by vein (intravenously) that increase or decrease the level of potassium in the blood to see whether an attack results. However, provocative testing may cause serious side effects such as paralysis of the breathing muscles or an abnormal heart rhythm. Thus, provocative testing has been replaced by a safer exercise test. In the exercise test, doctors have the person vigorously exercise a single muscle for 2 to 5 minutes to see if that makes the muscle weak. Doctors record electrical activity from the muscle before and after exercise to see if it was affected.

Prevention and Treatment

  • Depends on the form

People with the hypokalemic form can take potassium chloride in an unsweetened solution or have it injected into a vein while an attack is in progress. Usually symptoms improve considerably within an hour. People with the hypokalemic form should also avoid meals rich in carbohydrates and salt, avoid alcohol after periods of rest, and avoid strenuous exercise. Acetazolamide, a drug that alters the blood’s acidity, may help prevent attacks.

People with the hyperkalemic form can stop a mild attack by doing light exercise and eating a carbohydrate-rich meal. If an attack is persistent, drugs (such as a thiazide diuretic or inhaled albuterol) can help lower the potassium level. If an attack is severe, doctors give calcium or insulin and glucose by vein. People can prevent attacks by eating frequent meals rich in carbohydrates and low in potassium and by avoiding fasting, strenuous activity after meals, and exposure to cold.

People with the thyrotoxic form are given potassium chloride (as in the hypokalemic form), and doctors closely monitor levels of potassium in the blood during severe attacks. To prevent attacks, doctors give people drugs to keep their thyroid gland functioning properly and beta-blockers (such as propranolol).

More Information

The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

Drugs Mentioned In This Article

Generic Name Select Brand Names
Cena K , ED-K+10, Epiklor, K Plus, K Plus Care, K-10 , K-8, Kaon-CL, Kay Ciel , K-Dur, K-Lor, Klor-Con, Klor-Con M10, Klor-Con M15, Klor-Con M20, Klotrix, K-Lyte CL, K-Sol , K-Tab, Micro-K, Micro-K Extencaps, PROAMP, Rum-K, Slow-K, Tri-K
Diamox, Diamox Sequels
Accuneb, ProAir digihaler, Proair HFA, ProAir RespiClick, Proventil, Proventil HFA, Proventil Repetabs, Respirol , Ventolin, Ventolin HFA, Ventolin Syrup, Volmax, VoSpire ER
HEMANGEOL, Inderal, Inderal LA, Inderal XL, InnoPran XL
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