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Home > Diseases and Conditions > Hypokalemic periodic paralysis

Hypokalemic periodic paralysis


Periodic paralysis - hypokalemic

Last reviewed: November 30, 2009.

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.

Causes, incidence, and risk factors

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.

The weakness or paralysis:

Most commonly is located at the shoulders and hips


May also affect the arms, legs, muscles of the eyes, and muscles that help you breathe and
swallow
Occurs intermittently
Most commonly occurs on awakening
Most commonly occurs after sleep or rest
Is rare during exercise, but may be triggered by rest after exercise
May be triggered by heavy, high-carbohydrate, high-salt meals or alcohol consumption
Usually lasts 3 - 24 hours

Other symptoms may include:

Eyelid myotonia (a condition in which after opening and closing the eyes, the patient cannot
open them for a short time)

Note: The patient's thinking remains alert during attacks.

Signs and tests

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 ECG or heart tracing may be abnormal during attacks.


An EMG or muscle tracing is usually normal between attacks and abnormal during attacks.
A muscle biopsy may show abnormalities.

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.

Taking potassium supplements will not prevent attacks.

Avoiding alcohol and eating a low-carbohydrate diet may help.

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

Kidney stones (a side effect of acetazolamide)


Heart arrhythmias during attacks
Difficulty breathing, speaking, or swallowing during attacks (rare)
Progressive muscle weakness

Calling your health care provider

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 Conditions >


Hypokalemic periodic paralysis
On this page:
Description
Genetic changes
Inheritance
Treatment
Additional information
Other names
Glossary definitions

Reviewed April 2007

What is hypokalemic periodic paralysis?


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, repeated episodes can lead to persistent muscle weakness later in life.
People with hypokalemic periodic paralysis 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.

How common is hypokalemic periodic 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 genes are related to hypokalemic periodic paralysis?


Mutations in the CACNA1S and SCN4A genes cause hypokalemic periodic paralysis.
The CACNA1S and SCN4A genes provide instructions for making proteins that play an essential
role in muscles used for movement (skeletal muscles). For the body to move normally, these
muscles must tense (contract) and relax in a coordinated way. Muscle contractions are triggered
by the flow of certain positively charged atoms (ions) into muscle cells. The CACNA1S and
SCN4A proteins form channels that control the flow of these ions. The channel formed by the
CACNA1S protein transports calcium ions into cells, while the channel formed by the SCN4A
protein transports sodium ions.
Mutations in the CACNA1S or SCN4A gene alter the usual structure and function of calcium or
sodium channels. The altered channels cannot properly regulate the flow of ions 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.
A small percentage of people with the characteristic features of hypokalemic periodic paralysis
do not have identified mutations in the CACNA1S or SCN4A gene. In these cases, the cause of
the condition is unknown.
Read more about the CACNA1S and SCN4A genes.

How do people inherit hypokalemic periodic paralysis?


This condition is inherited in an autosomal dominant pattern, which means one copy of the
altered gene in each cell is sufficient to cause the disorder.
In most cases, an affected person has one parent with the condition.

Where can I find information about diagnosis, management, or treatment of


hypokalemic periodic paralysis?
These resources address the diagnosis or management of hypokalemic periodic paralysis and
may include treatment providers.
Gene Review: Hypokalemic Periodic Paralysis
Gene Tests: Hypokalemic Periodic Paralysis Type 1
Gene Tests: Hypokalemic Periodic Paralysis Type 2
MedlinePlus Encyclopedia: Hypokalemic periodic paralysis

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.

Where can I find additional information about hypokalemic periodic paralysis?


You may find the following resources about hypokalemic periodic paralysis helpful. These
materials are written for the general public.
MedlinePlus - Health information (3 links)
Genetic and Rare Diseases Information Center - Information about genetic
conditions and rare diseases
Additional NIH Resources - National Institutes of Health

National Institute of Neurological Disorders and Stroke

Educational resources - Information pages (4 links)


Patient support - For patients and families (2 links)

You may also be interested in these resources, which are designed for healthcare professionals
and researchers.
Gene Reviews - Clinical summary
Gene Tests - DNA tests ordered by healthcare professionals (2 links)
ClinicalTrials.gov - Linking patients to medical research
PubMed - Recent literature
Online Books - Medical and science texts

Scriver's Online Metabolic and Molecular Bases of Inherited Disease (OMMBID):


Channelopathies: Episodic Disorders of the Nervous System

OMIM - Genetic disorder catalog

What other names do people use for hypokalemic periodic paralysis?


Familial Hypokalemic Periodic Paralysis
HOKPP
HypoKPP
HypoPP
Primary Hypokalemic Periodic Paralysis
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 if I still have specific questions about hypokalemic periodic paralysis?


Ask the Genetic and Rare Diseases Information Center .

Where can I find general information about genetic conditions?


The Handbook provides basic information about genetics in clear language.
What does it mean if a disorder seems to run in my family?
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?

These links provide additional genetics resources that may be useful.


Genetics and health
Resources for Patients and Families
Resources for Health Professionals

What glossary definitions help with understanding hypokalemic periodic paralysis?


atom ; autosomal ; autosomal dominant ; calcium ; carbohydrate ; cell ; channel ; contraction ;
familial ; gene ; hypokalemia ; ions ; ion transport ; mutation ; potassium ; prevalence ; protein ;
skeletal muscle ; sodium ; sodium channel ; symptom
You may find definitions for these and many other terms in the Genetics Home Reference
Glossary.
See also Understanding Medical Terminology.
References (8 links)

The resources on this site should not be used as a substitute for professional medical care or
advice. Users seeking information about a personal genetic disease, syndrome, or condition
should consult with a qualified healthcare professional. See How can I find a genetics
professional in my area? in the Handbook.

Reviewed: April 2007


Published: April 3, 2011
0020

Home > Drugs and Supplements > Potassium

Potassium(poe tass' i um)

Last reviewed: September 1, 2008.

Why is this medication prescribed?

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.

How should this medicine be used?

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.
What special precautions should I follow?

Before taking potassium,

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.

What special dietary instructions should I follow?

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).

What should I do if I forget a dose?

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.

What side effects can this medication cause?

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
heaviness or weakness of legs
cold, pale, gray skin
stomach pain
unusual stomach bulging
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].

What storage conditions are needed for this medicine?

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.

What other information should I know?

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

American Society of Health-System Pharmacists, Disclaimer

AHFS Consumer Medication Information. Copyright, 2011. The American Society of Health-System Pharmacists, Inc.,

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:
Brand names of combination products

Neutra-Phos-K (containing Phosphorus and Potassium)

In this Page

Why is this medication prescribed?


How should this medicine be used?
What special precautions should I follow?
What special dietary instructions should I follow?
What should I do if I forget a dose?
What side effects can this medication cause?
What storage conditions are needed for this medicine?
In case of emergency/overdose
What other information should I know?
Other names
Brand names of combination products

Evaluating your options


ACE Inhibitors and ARBs To Protect Your Heart? A Guide for Patients Being Treated for Stable
Coronary Heart Disease

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

Hyperkalemia is higher-than-normal levels of potassium in the blood.

Hypokalemia

Hypokalemia is a lower-than-normal amount of potassium in the blood.

Hypertension

Hypertension is the term used to describe high blood pressure.


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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