Periodic Paralysis Syndrome
(Familial Periodic Paralysis; Hypokalemic Periodic Paralysis; Anderson-Tawil Syndrome; Paraneoplastic Periodic Paralysis)
DefinitionPeriodic paralysis is a rare condition that is usually inherited. It causes occasional episodes of severe muscle weakness. The two most common types of periodic paralysis are hypokalemic and hyperkalemic.
CausesPeriodic paralysis is a condition that is present from birth. Familial periodic paralysis is inherited, but may occur without a known family history. Periodic paralysis is caused by abnormalities of the electrolyte channels on muscles.With the inherited form of the disorder, only one affected parent is needed to transmit the gene to the baby. Rarely, the condition occurs as a result of a noninherited genetic defect.
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Risk FactorsFactors that increase your chance of developing periodic paralysis include:
- Family history of periodic paralysis
- Thyroid disorder—high thyroid hormone levels, particularly in Asian males
SymptomsAlthough muscle strength returns to normal between attacks, repeated episodes of weakness may lead to chronic muscle weakness later in life. The person remains alert and aware during attacks. There is no loss of sensation.Episodes of severe weakness in the arms and legs are the major symptom. Typically, these episodes occur during sleep, early morning, or after strenuous activity. Cold, stress, and alcohol may also produce attacks. Other, less common, symptoms may include:
- Weakness in the eyelids and face muscles
- Muscle pain
- Irregular heartbeats
- Difficulty breathing or swallowing—This requires emergency care.
- Potassium levels are low during attacks
- Frequency of attacks varies from daily to yearly
- Attacks usually last between 4-24 hours, but can last for several days
- Attacks usually begin in adolescence, but they can occur before age 10
- Potassium levels are high or normal during attacks
- Attacks are usually shorter, more frequent , and less severe than the hypokalemic form; breathing and swallowing difficulties are extremely rare
- Between attacks, patients often experience muscle spasms or difficulty relaxing their muscles. This condition is known as myotonia
- Attacks usually begin in early childhood
DiagnosisBecause this primarily is an inherited condition, the most important part of diagnosis is obtaining a family history. Your doctor will ask about symptoms and your medical history. A physical exam will be done.Attacks do not usually occur during an office visit. Your doctor may order several blood tests and thyroid tests to check potassium levels during an attack.Your doctor may want to bring on an attack during an office visit. This should only be done under careful monitoring by an experienced neurologist. If an attack is triggered, several tests may be done, including:biopsy .
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TreatmentSince there is no cure for periodic paralysis, lifelong treatment is usually required. Treatment focuses on preventing attacks and relieving symptoms.
Lifestyle ChangesThere are a few behaviors you can adopt to reduce the frequency and severity of attacks:
- Eat a low carbohydrate , low sodium diet
- Avoid strenuous exercise
- Eat a low potassium diet .
- Stay warm.
- Avoid fasting, alcohol, and heavy exercise.
MedicationsThe type of medications prescribed depend on the type of periodic paralysis.
- Medications for both hypokalemic and hyperkalemic include:
- Acetazolamide may prevent an attack by reducing the flow of potassium from the bloodstream into the cells of the body.
- Potassium may stop an attack; intravenous potassium may be prescribed for severe weakness.
- Avoiding certain commonly prescribed medications may help reduce the onset of attacks.
- If you have a thyroid condition, be sure to get treatment for it.
- Thiazide diuretics, or water pills, may be prescribed to prevent an attack.
- Glucose and insulin, or calcium carbonate may be prescribed to slow or stop an attack.
PreventionFamilial periodic paralysis cannot be prevented. Because it can be inherited, genetic counseling may be advised for couples at risk of passing on the disorder. For the hypokalemic type, attacks may be reduced by:
- Avoiding corticosteroids and glucose infusions
- Following a diet low in carbohydrates and sodium and rich in potassium
- Avoiding high potassium foods, fasting, and drugs known to increase potassium levels
- Engaging in regular, mild exercise
Muscular Dystrophy Association
National Organization for Rare Disorders (NORD)
Muscular Dystrophy Canada
Hyperkalemic periodic paralysis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated April 30, 2013. Accessed June 24, 2013.
Jurkat-Ratt K, Lehmann-Horn F. Paroxysmal muscle weakness-the periodic paralyses. J Neurol . 2006;253:1391-1398.
Jurkatt-Rott K, Lerche H, Weber Y, Lehmann-Horn F. Hereditary channelopathies in neurology. Adv Exp Med Biol . 2010;686:305-334.
Miller TM. Correlating phenotype and genotype in the periodic paralyses. Neurology . 2004;63:1647-1655
NINDS familial periodic paralyses information page. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/periodic%5Fparalysis/periodic%5Fparalysis.htm. Updated March 12, 2012. Accessed June 24, 2013.
Patient page: attacks of immobility caused by diet or exercise? The mystery of periodic paralyses. Neurology . 2004;63:E17-E18.
- Reviewer: Rimas Lukas, MD; Michael Woods, MD
- Review Date: 06/2013
- Update Date: 05/11/2013
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