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Hypokalemic Periodic Paralysis

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PPS, Periodic Paralysis Syndrome Periodic Muscle Weakness, or Familial Periodic Paralysis

  • This condition is characterized by periodic or intermittent weakness or paralysis of muscles that may last for hours or days.  PPS starts in adolescence and is rare in adults.  It is often present in multiple members of the family.

  • Sudden weakness of proximal (close to body) muscles
  • Shoulder, hips, arms, and legs are weak and flaccid (not stiff but very relaxed).
  • There can be swallowing or breathing difficulty (swallowing muscles, and respiratory muscles).
  • Muscle Cramps can occur.
  • Often follows a heavy carbohydrate meal (pasta, rice, etc.), upon awakening, or resting after exercise.
  • May last hours or days
  • Between attacks the muscle strength and tone is normal.
  • Constipation
  • Fractures can occur during a fall.
  • Heart may race or beat irregularly.
  • Patient is alert during attacks.

  • Genetic -- the diseased gene is passed down from one parent to the child (autosomal dominant).
  • Sporadic -- cases that are not genetic or familial do exist.

  • History:
    1. Symptoms
    2. Family history
    3. Illnesses
    4. Eating and exercise habits
    5. Social habits-drug and alcohol use
    6. Job
    7. Medications
    8. Surgeries
  • Physical exam:
    1. Weak, flaccid muscles during attack-when the patient picks up his arm, it drops without resistance.
    2. Muscle strength and tone is normal between attacks.
    3. Reflexes, such as the knee-jerk reflex, are diminished.
  • Injection of Insulin (hormone) or glucose (sugar) can trigger an attack by decreasing blood Potassium levels (hypokalemia).
  • Blood taken during attack shows low Potassium levels.  After an attack, the levels are normal.
  • During an attack, an EKG, which records the electrical activity of the heart, is abnormal.
  • ECG -- records the electrical activity of the brain, which may be abnormal during attacks.
  • EMG -- measures the electrical activity and function of the muscle, which may also be normal.

  • Family history
  • Sex --males>females
  • Race -- Asian men
  • Age -- rare in adults. Usually occurs after age 10, but can occur earlier.
  • Eating high carbohydrate diet, rest, and sleep can trigger an attack.
  • Thyroid disease-thyrotoxic PPS

  • Potential dangerous arrhythmias can occur.
  • Potassium orally or intravenously (in the veins) can stop attacks.
  • Potassium (especially given through the veins) is dangerous and must be given under close supervision.
  • Oxygen may be needed if there is breathing difficulty.
  • If breathing stops, a patient may need a respirator, i.e., a machine that breathes for the patient.
  • If arrhythmias are present, medications or electric shock may normalize the heart rate.

  • Contact your physician -- if untreated, PSS can lead to progressive muscle weakness.  If you have difficulty breathing, contact 911.

  • Low carbohydrate diet
  • Medications -- acetazolamide, spironolactone, and triamterene can keep Potassium levels in the heart elevated.
  • Routine checking of blood Potassium
  • Potassium supplements may be needed.
  • Family members may want to seek genetic counseling if they suspect this disease.

  • Hypokalemia from other causes:



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