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 .
Periodic paralysis - hypokalemic
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 onto you in order for you to be affected. Ocassionally, the condition may be the result of a genetic problem that is not inherited.
Unlike other forms of periodic paralysis, persons with this type have normal thyroid function and very low blood levels of potassium during episodes of weakness.
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.
The disorder involves attacks of muscle weakness or loss of muscle movement (paralysis) that come and go. 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 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 located at the shoulders and hips
- Involves the arms and legs, but may also affect muscles of the eyes and those 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 less than 24 hours
Other symptoms may include:
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.
A serum potassium blood tests done during an attack of muscle weakness will reveal low potassium levels. 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.
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: intravenous potassium should be given with caution, especially in individuals with kidney disease.
Taking potassium 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. Acetazolamide may cause the body to lose potassium.
Triamterene or spironolactone may help to prevent attacks in people who do not respond to acetazolamide.
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.
Insulin levels may affect the course of the disorder in some people because insulin increases the flow of potassium into cells.
- 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.
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.