Hypokalemic periodic paralysis
Periodic paralysis - hypokalemic; Familial hypokalemic periodic paralysis; HOKPP; HypoKPP; HypoPPHypokalemic periodic paralysis (hypoPP) is a disorder that causes occasional episodes of muscle weakness and sometimes a lower than normal level of potassium in the blood. The medical name for low potassium level is hypokalemia.
Hypokalemia
Low blood potassium level is a condition in which the amount of potassium in the blood is lower than normal. The medical name of this condition is h...
Read Article Now Book Mark ArticleHypoPP is one of a group of genetic disorders that includes hyperkalemic periodic paralysis and thyrotoxic periodic paralysis.
Hyperkalemic periodic paralysis
Hyperkalemic periodic paralysis (hyperPP) is a disorder that causes occasional episodes of muscle weakness and sometimes a higher than normal level o...
Read Article Now Book Mark ArticleThyrotoxic periodic paralysis
Thyrotoxic periodic paralysis (TPP) is a condition with episodes of severe muscle weakness. It occurs in people who have high levels of thyroid horm...
Read Article Now Book Mark ArticleHypoPP is the most common form of periodic paralysis. It affects males more often.
Causes
HypoPP is congenital. This means it is present at birth. In most cases, it is passed down through families (inherited) as an autosomal dominant disorder. In other words, only one parent needs to pass the gene related to this condition on to their child in order for the child to be affected.
Autosomal dominant
Autosomal dominant is one of many ways that a genetic trait or disorder can be passed down through families. In an autosomal dominant disease, if you...
Read Article Now Book Mark ArticleIn some cases, the condition may be the result of a genetic problem that is not inherited.
Unlike other forms of periodic paralysis, people with hypoPP have normal thyroid function. But they have a very low blood level of potassium during episodes of weakness. This results from potassium moving from the blood into muscle cells in an abnormal way.
Risk factors include having other family members with periodic paralysis. The risk is slightly higher in Asian men who also have thyroid disorders.
Symptoms
Symptoms include attacks of muscle weakness or loss of muscle movement (paralysis) that come and go. There is normal muscle strength between attacks.
Paralysis
Muscle function loss is when a muscle does not work or move normally. The medical term for complete loss of muscle function is paralysis.
Read Article Now Book Mark ArticleAttacks usually begin in the teen years, but they can occur before age 10. How often the attacks occur varies. Some people have attacks every day. Others have them once a year. During attacks the person remains alert.
The weakness or paralysis:
- Most commonly occurs at the shoulders and hips
- May also affect the arms, legs, muscles of the eyes, and muscles that help with breathing and swallowing
- Occurs off and on
- Most commonly occurs on awakening or after sleep or rest
- Is rare during exercise, but may be triggered by resting after exercise
- May be triggered by high-carbohydrate, high-salt meals, stress, pregnancy, heavy exercise, and cold
- An attack usually lasts for several hours up to a day
Another symptom may include eyelid myotonia (a condition in which after opening and closing the eyes, they cannot be opened for a short time).
Myotonia
Myotonia congenita is an inherited condition that affects muscle relaxation. It is congenital, meaning that it is present from birth. It occurs mor...
Read Article Now Book Mark ArticleExams and Tests
The health care provider may suspect hypoPP based on a family history of the disorder. Other clues to the disorder are muscle weakness symptoms that come and go with normal or low results of a blood potassium test.
Potassium test
This test measures the amount of potassium in the fluid portion (serum) of the blood. Potassium (K+) helps nerves and muscles communicate. It also ...
Read Article Now Book Mark ArticleBetween 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. The blood potassium level is normal between attacks.
During an attack, muscle reflexes are decreased or absent. And muscles go limp rather than staying stiff. Muscle groups near the body, such as the shoulders and hips, are involved more often than the arms and legs.
Tests that may be done include:
- Electrocardiogram (ECG), which may be abnormal during attacks
ECG
An electrocardiogram (ECG) is a test that records the electrical activity of the heart.
Read Article Now Book Mark Article - Electromyography (EMG), which is usually normal between attacks and abnormal during attacks
EMG
Electromyography (EMG) is a test that checks the health of the muscles and the nerves that control the muscles.
Read Article Now Book Mark Article - Muscle biopsy, which may show abnormalities
Muscle biopsy
A muscle biopsy is the removal of a small piece of muscle tissue for examination.
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Other tests may be ordered to rule out other causes.
Treatment
The goals of treatment are relief of symptoms and prevention of further attacks.
Muscle weakness that involves the breathing or swallowing muscles is an emergency situation. Dangerous irregular heartbeats (heart arrhythmias) may also occur during attacks. Any of these must be treated right away.
Heart arrhythmias
An arrhythmia is a disorder of the heart rate (pulse) or heart rhythm. The heart can beat too fast (tachycardia), too slow (bradycardia), or irregul...
Read Article Now Book Mark ArticlePotassium given during an attack may stop the attack. Potassium can be taken by mouth. But if weakness is severe, potassium may need to be given through a vein (IV).
Taking potassium supplements may help prevent muscle weakness.
Eating a low-carbohydrate diet may help decrease symptoms.
A medicine called acetazolamide may be prescribed to prevent attacks. Your provider may tell you to also take potassium supplements because acetazolamide may cause your body to lose potassium.
If acetazolamide does not work for you, other medicines may be prescribed.
Outlook (Prognosis)
HypoPP responds well to treatment. Treatment may prevent, and even reverse, progressive muscle weakness. Although muscle strength starts out normal between attacks, repeated attacks may eventually cause worsening and permanent muscle weakness between attacks.
Possible Complications
Health problems that may be due to this condition include:
- Kidney stones (a side effect of acetazolamide)
Kidney stones
A kidney stone is a solid mass made up of tiny crystals. One or more stones can be in the kidney or ureter at the same time.
Read Article Now Book Mark Article - Irregular heartbeat during attacks
- Difficulty breathing, speaking, or swallowing during attacks (rare)
Difficulty breathing
Breathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough air
Read Article Now Book Mark Article - Muscle weakness that worsens over time
When to Contact a Medical Professional
Contact your provider if you or your child has muscle weakness that comes and goes, especially if you have family members who have periodic paralysis.
Go to the emergency room or call the local emergency number (such as 911) if you or your child faints, has trouble breathing, speaking, or swallowing.
Prevention
HypoPP cannot be prevented. Because it can be inherited, genetic counseling may be advised for couples at risk of the disorder.
Treatment prevents attacks of weakness. Before an attack, there may be leg stiffness or heaviness in the legs. Doing mild exercise when these symptoms start may help prevent a full-blown attack.
References
Boegle AK, Narayanaswami P. Treatment and management of disorders of neuromuscular hyperexcitability and periodic paralysis. In: Bertorini TE, ed. Neuromuscular Disorders: Treatment and Management. 2nd ed. St Louis, MO: Elsevier; 2022:chap 18.
Doughty CT, Amato AA. Disorders of skeletal muscle. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 109.
Kang MK, Kerchner GA, Ptácek LJ. Channelopathies: episodic and electrical disorders of the nervous system. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 98.
Weimer M, Reese JJ, Tilton AH. Acute neuromuscular diseases and disorders. In: Zimmerman JJ, Clark RSB, Fuhrman BP, et al. Fuhrman and Zimmerman's Pediatric Critical Care. 6th ed. Philadelphia, PA: Elsevier; 2022:chap 68.
Review Date: 12/31/2023
Reviewed By: Walead Latif, MD, Nephrologist and Clinical Associate Professor, Rutgers Medical School, Newark, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.