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Hypokalemic Periodic Paralysis: Causes, Incidence, and Risk Factors
Hypokalemic Periodic Paralysis: Causes, Incidence, and Risk Factors
Hypokalemic periodic paralysis is an inherited disorder that causes occasional episodes of muscle
weakness.
It is one of a group of genetic disorders that includes hyperkalemic periodic paralysis and thyrotoxic
periodic paralysis.
Hypokalemic periodic paralysis is a condition in which a person has episodes of muscle weakness and
sometimes severe paralysis.
The condition is congenital, which means it is present from birth. In most cases, it is passed down through
families (inherited) as an autosomal dominant disorder. That means only one parent needs to pass the
gene related to this condition on to you in order for you to be affected.
Occasionally, the condition may be the result of a genetic problem that is not inherited.
Unlike other forms of periodic paralysis, persons with congenital hypokalemic periodic paralysis have
normal thyroid function and very low blood levels of potassium during episodes of weakness. This results
from potassium moving from the blood into muscle cells in an abnormal way.
Risks include having other family members with periodic paralysis. The risk is slightly higher in Asian men
who also have thyroid disorders.
Disorders that cause intermittent episodes of paralysis as their primary effect are uncommon.
Hypokalemic periodic paralysis occurs in approximately 1 out of 100,000 people.
Symptoms
The disorder involves attacks of muscle weakness or loss of muscle movement (paralysis) that come and
go. Initially, there is normal muscle strength between attacks.
Attacks usually begin in adolescence, but they can occur before age 10. Attacks that do not begin until
adulthood are rare in people with hypokalemic periodic paralysis and are usually caused by other
disorders.
How often the attacks occur varies. Some people have attacks every day, while others have them once a
year. Episodes of muscle weakness usually last between a few hours and a day.
Occurs intermittently
Eyelid myotonia (a condition in which after opening and closing the eyes, the patient cannot
open them for a short time)
Between attacks, a physical examination shows nothing abnormal. Before an attack, there may be leg
stiffness or heaviness in the legs.
During an attack of muscle weakness, the blood potassium level is low. This confirms the diagnosis.
There is no decrease in total body potassium, and blood potassium levels are normal between attacks.
During an attack, muscle reflexes may be decreased or absent, and muscles go limp rather than staying
stiff. The muscle groups near the body, such as shoulders and hips, are involved more often than the
arms and legs.
An EMG or muscle tracing is usually normal between attacks and abnormal during attacks.
Treatment
Muscle weakness that involves the breathing or swallowing muscles is an emergency situation.
Dangerous heart arrhythmias may also occur during attacks.
The goals of treatment are relief of symptoms and prevention of further attacks.
Potassium that is given during an attack may stop the attack. It is preferred that potassium be given by
mouth, but if weakness is severe, potassium may need to be given through a vein (IV). Note: Potassium,
especially intravenous potassium, should be given with caution, especially in individuals with kidney
disease.
A medicine called acetazolamide prevents attacks in many cases. If you take this medicine, your doctor
may tell you to also take potassium supplements because acetazolamide may cause your body to lose
potassium.
Triamterene or spironolactone may help to prevent attacks in people who do not respond to
acetazolamide.
Expectations (prognosis)
Hypokalemic periodic paralysis responds well to treatment. Treatment may prevent, and even reverse,
progressive muscle weakness. Although muscle strength is initially normal between attacks, repeated
attacks may eventually cause worsening and permanent muscle weakness between attacks.
Complications
Call your health care provider if you have intermittent muscle weakness, particularly if there is a family
history of periodic paralysis.
Go to the emergency room or call the local emergency number (such as 911) if you faint or have difficulty
breathing, speaking, or swallowing. These are emergency symptoms.
Prevention
Hypokalemic periodic paralysis cannot be prevented. Because it can be inherited, genetic counseling may
be advised for couples at risk for the disorder.
Treatment prevents attacks of weakness. Before an attack, there may be leg stiffness or heaviness in the
legs. Performing mild exercise when these symptoms start may help prevent a full-blown attack.
References
1.Barohn RJ. Muscle diseases. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed.
Philadelphia, Pa: Saunders Elsevier; 2007:chap 447.
Review Date: 11/30/2009.
Reviewed by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine,
Department of Medicine, University of Washington School of Medicine; Herbert Y. Lin, MD,
PHD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine,
Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M.,
Inc.
Genetic changes
Inheritance
Treatment
Additional information
Other names
Glossary definitions
You might also find information on the diagnosis or management of hypokalemic periodic
paralysis in Educational resources and Patient support.
To locate a healthcare provider, see How can I find a genetics professional in my area? in the
Handbook.
You may also be interested in these resources, which are designed for healthcare professionals
and researchers.
Gene Reviews - Clinical summary
HOKPP
HypoKPP
HypoPP
Westphall disease
For more information about naming genetic conditions, see the Genetics Home Reference
Condition Naming Guidelines and How are genetic conditions and genes named? in the
Handbook.
What are the different ways in which a genetic condition can be inherited?
If a genetic disorder runs in my family, what are the chances that my children will
have the condition?
Why are some genetic conditions more common in particular ethnic groups?
Potassium is essential for the proper functioning of the heart, kidneys, muscles, nerves, and digestive
system. Usually the food you eat supplies all of the potassium you need. However, certain diseases (e.g.,
kidney disease and gastrointestinal disease with vomiting and diarrhea) and drugs, especially diuretics
('water pills'), remove potassium from the body. Potassium supplements are taken to replace potassium
losses and prevent potassium deficiency.
This medication is sometimes prescribed for other uses; ask your doctor or pharmacist for more
information.
Potassium comes in oral liquid, powder, granules, effervescent tablets, regular tablets, extended-release
(long-acting) tablets, and extended-release capsules. It usually is taken two to four times a day, with or
immediately after meals. Follow the directions on your prescription label carefully, and ask your doctor or
pharmacist to explain any part you do not understand. Take potassium exactly as directed. Do not take
more or less of it or take it more often than prescribed by your doctor.
Take all forms of potassium with a full glass of water or fruit juice.
Add the liquid to water. Dissolve the powder, granules, or effervescent tablets in cold water or fruit juice
according to the manufacturer's directions or the directions on your prescription label; mix the drug well
just before you take it. Cold liquids help mask the unpleasant taste.
Swallow extended-release tablets and capsules whole. Do not chew them or dissolve them in your mouth.
tell your doctor and pharmacist if you are allergic to potassium or any other drugs.
tell your doctor and pharmacist what prescription and nonprescription medications you are
taking, especially angiotensin converting enzyme (ACE) inhibitors such as captopril
(Capoten), enalapril (Vasotec),and lisinopril (Prinivil, Zestril); diuretics ('water pills'); and
vitamins. Do not take potassium if you are taking amiloride (Midamor), spironolactone
(Aldactone), or triamterene (Dyrenium).
tell your doctor if you have or have ever had heart, kidney, or Addison's (adrenal gland)
disease.
tell your doctor if you are pregnant, plan to become pregnant, or are breast-feeding. If you
become pregnant while taking potassium, call your doctor.
if you are having surgery, including dental surgery, tell the doctor or dentist that you are taking
potassium.
If you are using a salt substitute, tell your doctor. Many salt substitutes contain potassium. Your doctor will
consider this source in determining your dose of potassium supplement. Your doctor may advise you to
use a potassium-containing salt substitute and to eat potassium-rich foods (e.g., bananas, prunes, raisins,
and milk).
Take the missed dose as soon as you remember it and take any remaining doses for that day at evenly
spaced intervals. Do not take a double dose to make up for a missed one.
Potassium may cause side effects. Tell your doctor if any of these symptoms are severe or do not go
away:
upset stomach
vomiting
diarrhea
If you experience any of the following symptoms, call your doctor immediately:
mental confusion
listlessness
tingling, prickling, burning, tight, or pulling sensation of arms, hands, legs, or feet
black stools
If you experience a serious side effect, you or your doctor may send a report to the Food and Drug
Administration's (FDA) MedWatch Adverse Event Reporting program online [at
http://www.fda.gov/Safety/MedWatch] or by phone [1-800-332-1088].
Keep this medication in the container it came in, tightly closed, and out of reach of children. Store it at
room temperature and away from excess heat and moisture (not in the bathroom). Throw away any
medication that is outdated or no longer needed. Talk to your pharmacist about the proper disposal of
your medication.
In case of emergency/overdose
In case of overdose, call your local poison control center at 1-800-222-1222. If the victim has collapsed or
is not breathing, call local emergency services at 911.
Keep all appointments with your doctor and the laboratory. Your doctor will order certain lab tests to check
your response to potassium. You may have electrocardiograms (EKGs) and blood tests to see if your
dose needs to be changed.
Do not let anyone else take your medication. Ask your pharmacist any questions you have about refilling
your prescription.
It is important for you to keep a written list of all of the prescription and nonprescription (over-the-counter)
medicines you are taking, as well as any products such as vitamins, minerals, or other dietary
supplements. You should bring this list with you each time you visit a doctor or if you are admitted to a
hospital. It is also important information to carry with you in case of emergencies.
Other names
KCl
7272 Wisconsin Avenue, Bethesda, Maryland. All Rights Reserved. Duplication for commercial use must be authorized by
ASHP.
The following brand names are from RxNorm, a standardized nomenclature for clinical
drugs produced by the National Library of Medicine:
In this Page
In case of emergency/overdose
Other names
You have coronary heart disease, a disease affecting the arteries of your heart. You may have had a heart
attack or suffer from chest pains with exercise, but your symptoms are not changing. Your coronary heart
disease is in stable condition. This means that your symptoms have not changed or become worse.
Your doctor recommends adding a medicine called an ACE Inhibitor or an ARB. You do not take medicine
for high blood pressure (also called hypertension).
Comparing NSAIDs
How do NSAIDs compare in reducing pain?
Conditions of interest
Kidney disease
Kidney disease is any disease or disorder that affects the function of the kidneys. This may include:
Hyperkalemia
Hypokalemia
Hypertension
Ischemic cardiomyopathy
Ischemic cardiomyopathy is a term that doctors use to describe patients who have reduced heart pumping
(squeezing) due to coronary artery disease. These patients often have congestive heart failure.
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