Hyperkalemic Periodic Paralysis (HyperKpp)

What is hyperkalemic periodic paralysis (HyperKPP)?

Hyperkalemic periodic paralysis (HyperKPP) is a rare genetic condition that causes episodes of extreme muscle weakness or paralysis. Attacks can last minutes or up to days in length. Some individuals with HyperKPP can experience only a few attacks in their lifetime while others can experience attacks daily. Symptoms of HyperKPP are usually first evident in infancy. HyperKPP affects approximately one in 200,000 people.

What are the symptoms of HyperKPP?

Symptoms of HyperKPP include:

• Weakness or paralysis which can occur suddenly. Falls are common.
• Muscle stiffness which is known as myotonia. Usually this stiffness can be alleviated with movement.
• Paramytonia Cogenita von Eulenburg. This is muscle stiffness that is made worse with activity.

What causes HyperKPP?

HyperKPP is an inherited genetic disorder. This condition causes an inability of proper movement of sodium and potassium in and out of cells.  This movement is necessary for proper muscle function. During most attacks an individual is found to have high levels of potassium in their blood stream. Occasionally individuals have normal potassium levels. Factors that can lead to attacks include eating high potassium foods or not eating often even, stress, fatigue, alcohol consumption, pregnancy, certain medications, and exposure to cold temperatures.

Are there treatment options for HyperKPP?

Treatment of HyperKPP is directed at decreasing the frequency and severity of the attacks.  Options for treatment include:

• Dietary management such as a low potassium, high carbohydrate diet. Other dietary adjustments including avoidance of alcohol, regular meals. Consumption of high carbohydrate, high sugar food or beverage during an attack can limit severity and duration.
• Medications can be prescribed to treat weakness and muscle stiffness.
• Avoidance to cold temperature.
• Getting adequate sleep/rest.

Where can I find more information about HyperKPP?

• Periodic Paralysis International
• NIH