• Periodic Paralysis Syndrome

    (Familial Periodic Paralysis; Hypokalemic Periodic Paralysis; Anderson-Tawil Syndrome; Paraneoplastic Periodic Paralysis)

    Definition

    Periodic 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.

    Causes

    Periodic paralysis is a congenital condition, meaning it 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.
    The inherited form of the disorder is autosomal dominant, which means that only one affected parent is needed to transmit the gene to the baby. When one parent is affected, the child has a 50% chance of getting the disease. Rarely, the condition occurs as a result of a noninherited genetic defect.
    Genetic Material
    Chromosome DNA
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    Risk Factors

    Factors that increase your chance of developing periodic paralysis include:
    • Family history of periodic paralysis
    • Thyroid disorder (particularly in Asian males)

    Symptoms

    Although muscle strength returns to normal between attacks, repeated bouts of weakness may lead to chronic muscle weakness later in life. The person remains alert and aware during attacks. There is no accompanying loss of sensation.
    Episodic bouts of severe weakness in the arms and legs are the most prominent symptom. Typically, these bouts 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 ( arrhythmias )
    • Difficulty breathing or swallowing—This requires emergency care.
    Some features are specific to the type of periodic paralysis.
      Hypokalemic:
      • 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
      Hyperkalemic:
      • Potassium levels are high or normal during attacks
      • Attacks are usually shorter (lasting one to two hours), 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
    Persons with some types of periodic paralysis are at risk for a condition known as malignant hyperthermia. This can occur during the use of general anesthesia . Anyone with a family history of periodic paralysis needs to notify the anesthesiologist of this history prior to any surgery.

    Diagnosis

    Because this primarily is an inherited condition, the most important aspect 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 prescribe several blood 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:
    • Blood tests to look for the gene mutation or to look for antibodies that may cause these types of symptoms
    • Electrocardiogram (ECG, EKG)—a test that records the heart's activity by measuring electrical currents through the heart muscle
    • Electromyography (EMG)—to test the functioning of nerves and muscles
    If the diagnosis is in question, your doctor may do a muscle biopsy .
    Electromyography (EMG)
    Electromyogram EMG
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    Treatment

    Since there is no cure for periodic paralysis, lifelong treatment is usually required. Treatment focuses on preventing attacks and relieving symptoms.

    Lifestyle Changes

    There are a few behaviors you can adopt to reduce the frequency and severity of attacks:
      Hypokalemic: Hyperkalemic:

    Medications

      Both hypokalemic and hyperkalemic:
      • Acetazolamide (Diamox) may prevent an attack by reducing the flow of potassium from the bloodstream into the cells of the body.
      Hypokalemic:
      • Potassium (pill or liquid form) 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.
      Hyperkalemic:
      • Thiazide diuretics, or water pills, may be prescribed to prevent an attack.
      • Glucose, glucose and insulin, or calcium carbonate may be prescribed to slow or stop an attack.

    Prevention

    Familial 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
    For the hyperkalemic type, attacks may be reduced by:
    • Avoiding high potassium foods, fasting, and drugs known to increase potassium levels
    • Engaging in regular, mild exercise

    RESOURCES

    Muscular Dystrophy Association http://www.mdausa.org/

    National Organization for Rare Disorders (NORD) http://www.rarediseases.org/

    CANADIAN RESOURCES

    Health Canada http://www.hc-sc.gc.ca/index%5Fe.html/

    Muscular Dystrophy Canada http://www.mdac.ca/

    References

    Hyperkalemic periodic paralysis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated March 19, 2012. Accessed October 11, 2012.

    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

    Periodic paralyses. 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 October 11, 2012.

    Patient page: attacks of immobility caused by diet or exercise? The mystery of periodic paralyses. Neurology . 2004;63:E17-E18.

    Ropper AH, Brown RH. Adams and Victor's Principles of Neurology. 8th ed. New York, NY: McGraw-Hill Medical Publishing Division; 2005: chap 54.

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