HypoPP
Hypokalemic Periodic Paralysis (HypoPP) is a rare genetic disorder characterized by episodes of muscle weakness or paralysis associated with a drop in potassium levels in the blood (hypokalemia). These episodes can last for hours or days and are usually triggered by factors such as rest after exercise, high carbohydrate meals, stress, and periods of inactivity.
Causes[edit | edit source]
HypoPP is primarily caused by mutations in the CACNA1S or SCN4A genes. These genes encode for the alpha subunits of the L-type calcium channel and the voltage-gated sodium channel, respectively. Mutations in these genes disrupt ion flow across muscle cell membranes, leading to muscle weakness or paralysis.
Symptoms[edit | edit source]
The main symptom of HypoPP is episodic muscle weakness, which typically begins in adolescence. The severity and duration of these episodes can vary widely among individuals. During an episode, muscles in the arms and legs are usually affected first, and muscle weakness can progress to include muscles used for breathing and swallowing in severe cases. Between episodes, muscle strength is normal.
Diagnosis[edit | edit source]
Diagnosis of HypoPP involves a combination of clinical evaluation, family history, and specialized tests. Blood tests may show low levels of potassium during an episode. Electromyography (EMG) and muscle biopsy can provide additional diagnostic information. Genetic testing can confirm the diagnosis by identifying mutations in the CACNA1S or SCN4A genes.
Treatment[edit | edit source]
Treatment of HypoPP focuses on preventing episodes of muscle weakness and treating episodes when they occur. This may include dietary changes to maintain stable potassium levels, potassium supplements, and medications such as carbonic anhydrase inhibitors. Avoiding known triggers is also an important part of managing the condition.
Prognosis[edit | edit source]
With appropriate management, individuals with HypoPP can lead relatively normal lives. However, some may experience progressive muscle weakness over time. Early diagnosis and treatment are crucial to improving outcomes and preventing complications.
Search WikiMD
Ad.Tired of being Overweight? Try W8MD's physician weight loss program.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available.
Advertise on WikiMD
|
WikiMD's Wellness Encyclopedia |
| Let Food Be Thy Medicine Medicine Thy Food - Hippocrates |
Translate this page: - East Asian
中文,
日本,
한국어,
South Asian
हिन्दी,
தமிழ்,
తెలుగు,
Urdu,
ಕನ್ನಡ,
Southeast Asian
Indonesian,
Vietnamese,
Thai,
မြန်မာဘာသာ,
বাংলা
European
español,
Deutsch,
français,
Greek,
português do Brasil,
polski,
română,
русский,
Nederlands,
norsk,
svenska,
suomi,
Italian
Middle Eastern & African
عربى,
Turkish,
Persian,
Hebrew,
Afrikaans,
isiZulu,
Kiswahili,
Other
Bulgarian,
Hungarian,
Czech,
Swedish,
മലയാളം,
मराठी,
ਪੰਜਾਬੀ,
ગુજરાતી,
Portuguese,
Ukrainian
Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.
Contributors: Prab R. Tumpati, MD
