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Hyperkalemic Periodic Paralysis

Hyperkalemic Periodic Paralysis is an inherited form of periodic paralysis in which the serum potassium concentration is elevated during attacks. Unlike other forms of periodic paralysis (such as familial periodic paralysis and thyrotoxic periodic paralysis), hyperkalemic periodic paralysis is not associated with low potassium in the bloodstream (serum potassium). In fact, there may be normal or high levels of potassium in the bloodstream during and between attacks.

The disorder involves attacks of muscle weakness or paralysis, alternating with periods of normal muscle function. Attacks usually begin in early childhood. Multiple daily attacks are not uncommon.
Attacks typically last only 1 to 2 hours, but can sometimes last as long as a day. They tend to occur while resting after exercise or exertion. Attacks may also be triggered by cold exposure or eating small amounts of potassium.

Causes of Hyperkalemic Periodic Paralysis

Hyperkalemic Periodic Paralysis caused by inheritance of defective genes. The gene for hyperkalemic Periodic Paralysis affects virtually all who inherit it, with no difference in male-vs.-female expression. The normal gene is responsible for a muscle protein controlling the flow of sodium during muscle contraction.
 
 

Attacks of Hyperkalemic Periodic Paralysis may be prevented with frequent small meals high in carbohydrates, and the avoidance of foods high in potassium such as orange juice or bananas. Acetazolamide or thiazide (a diuretic) may be prescribed.

Diagnosis of Hyperkalemic Periodic Paralysis

Patients often report years wasted with wrong diagnosis, wrong treatments, deadends and multiple doctors, test and clinics. The CMAP (Compound Muscle Amplitude Potential) test, also called the exercise EMG or X-EMG, is diagnostic in 70-80% of cases when done correctly. Besides the patient history or a report of serum potassium low normal or low during an attack, the CMAP is the current standard for medical testing. Genetic diagnosis is often unreliable as only a few of the more common gene locations are tested. Standard EMG testing cannot diagnose a patient unless they are in a full blown attack at the time of testing. Provoking an attack with exercise and diet then trying oral potassium can be diagnostic, but also dangerous as this form of PP has an alternate form known as hyperkalemic periodic paralysis. The symptoms are almost the same, but the treatment is different. The old glucose insulin challenge is dangerous and risky to the point of being life threatening and should never be done when other options are so readily available[ci

Treatment of Hyperkalemic Periodic Paralysis

Treatment of hypokalemic periodic paralysis focuses on preventing further attacks and relieving acute symptoms. Avoiding carbohydrate-rich meals and strenuous exercise, and taking Acetazolamide (Diamox) or another carbonic anhydrase inhibitor, may help prevent attacks of weakness. Paralysis attacks can be managed by drinking effervescent potassium bicarbonate dissolved in water, or potassium gluconate oral solution. Treatment with potassium chloride or use of intravenious sterroids can cause intensification of symptoms. Some patients find positive results with time release potassium tablets. IV potassium is seldom justified unless the patient is unable to swallow. Daily potassium dosage may need to be much higher than for potassium replacement from simple hypokalemia: 100-150 mEqs of potassium bicarbonate is often needed to manage daily fluctuations in muscle strength and function.

 
 
 
 
 
   

This web site is intended for your own informational purposes only. No person or entity associated with this web site purports to be engaging in the practice of medicine through this medium. The information you receive is not intended as a substitute for the advice of a physician or other health care professional. If you have an illness or medical problem, contact your health care provider.

05/27/2011

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