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Friday, December 07, 2007

Hypokalemic Periodic Paralysis

Hypokalemic Periodic Paralysis (HypoKPP) is a congenital disorder that occurs within certain families and causes intermittent episodes of muscle weakness or paralysis, It can be inherited but occasionally this condition caused by genetic mutations (CACNA1S and SCN4A) in a voltage-gated calcium channel. The CACNA1S and SCN4A genes provide instructions for making proteins that play an essential role in muscles used for movement (skeletal muscles).

Sporadic cases of Hypokalemic Periodic Paralysis have also been reported, a temporary inability to move muscles in the arms and legs. Attacks can occur without warning or can be triggered by factors such as rest after exercise, a viral illness, or certain medications. The attacks can occur from daily to yearly and may last for a few hours or for several days.

  • Symptom of Hypokalemic Periodic Paralysis
  • Symptoms of Hypokalemic Periodic Paralysis are usually refers to various symptoms known to a patient, and the doctor will get sign of Hypokalemic Periodic Paralysis after they do some test or examination to the patient.

    Most common symptom of Hypokalemic Periodic Paralysis are :
    - Normal muscle strength between attacks
    - Positive Babinski's reflex
    - Eyelid spasms between episodes
    - Normal alertness during attacks
    - Episodic muscle weakness or paralysis.
    Patient will mention some weakness on his body located at the shoulders, hips, involves the arms and legs. Its Occurs intermittently and commonly occurs on awakening. The patient may be triggered by rest after exercise, by exposure to cold, by heavy, high-carbohydrate, high-salt meals or alcohol consumption.

  • Diagnosis for Hypokalemic Periodic Paralysis
  • Hypokalemic Periodic Paralysis usually begin in adolescence, but they can occur before age 10. To diagnose this case, the doctors are needs specific examination such as patient history and confirmed by appropriate evaluation of serum electrolytes during attacks, with the CMAP amplitude test (Exercise EMG)(30), evaluation of the response to provocative testing or by DNA analysis.

    When period of paralysis happened mostly potassium levels are low, a physical examination shows nothing abnormal but muscle reflexes may be decreased or absent and muscles go limp rather than staying stiff. On this period also when ECG or heart tracing done, the result may show an abnormal. Include of muscle biopsy to the patient with Hypokalemic Periodic Paralysis may show abnormalities.

  • Treatment of Hypokalemic Periodic Paralysis
  • There is no cure for periodic paralyses, The goals of treatment are relief of acute symptoms like myotonia and prevention of further attacks. Patient who diagnose by the doctor as Hypokalemic Periodic Paralysis may they will get advice to avoid carbohydrate-rich meals and do the strenuous exercise. The doctor may prescribed some medicine such as acetazolamide or another carbonic anhydrase inhibitor, may help prevent attacks of weakness.

    To the Patients with hypokalemic periodic paralysis, administering potassium may stop an attack and also giving some glucose or other carbohydrates (sugars) may reduce the severity. Potassium is generally taken by mouth, but in a case of severe muscle weakness, the patient would need to receive it intravenously in a hospital. Intravenous calcium or diuretics such as furosemide may be needed to stop sudden attacks. Patients with Hypokalemic Periodic Paralysis they should take a low-carbohydrate diet and avoidance of alcohol may be recommended.

    By taking acetazolamide periodically, its high risk to the patient for potential Kidney stones. Some of complication during phase of paralysis came, patients with hypokalemic periodic paralysis may risk for difficulty breathing, speaking, or swallowing even all of this are rare cases. The other complication that may happen to the patient hypokalemic periodic paralysis is progressive muscle weakness.
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