Hypokalemic periodic paralysis

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Background

  • Autosomal dominant channelopathy[1]
  • Acquired hypokalemia from barium poisoning, hyperthyroidism, renal disorders, and GI losses[2]
  • Most first attacks happen by age 16
  • Young males, Asian
  • There is no decrease in total body potassium. Blood potassium level is normal between attacks.[3]
  • Associations
    • Thyrotoxicosis
    • Steroids
    • ETOH
    • Renal disease

Clinical Features

Symptoms

  • Muscle weakness or paralysis with associated hypokalemia (though potassium levels can be normal)
  • Sometimes painful though often painless
  • Weakness can be from hand to leg to complete paralysis

History

  • Strenuous exercise
  • High carbohydrate meal
  • High sodium meals
  • Sudden changes in temperature
  • Emotional stress
  • Attacks can last several hours to several days

Physical Exam

  • Reflexes are decreased or absent[4]
  • Shoulders and hips, are involved more often than the arms and legs.[4]
  • There is flaccid paralysis rather than hypertonia
  • There should not be myoclonus or spasticity

Differential Diagnosis

Weakness

Evaluation

Labs

  • Chemistry (potassium)

ECG Findings

  • Sinus bradycardia
  • ECG signs of hypokalemia may be present but not always diagnostic[5]
    • U waves in leads II, V-2, V-3, and V-4
    • Progressive flattening of T waves and depression of ST segment
    • Prolongation of the PR and QT intervals

Management

  • Replace potassium appropriately - do not try to correct to normal level as once the muscles release potassium you will likely overcorrect
  • Long Term Treatment:
    • Should be initiated by primary physician but often includes eating a low carbohydrate diet and avoiding alcohol
    • Medications to increase potassium: Acetazolamide, Spironolactone, Potassium tablets

Disposition

  • Can be discharged from ED after potassium repletion and resolution of symptoms
  • Consider admission if patient remains symptomatic

See Also

References

  1. June-Bum Kim, MD, PhD. Channelopathies. Korean J Pediatr. 2014 Jan;57(1):1-18 Full Text
  2. Ahlawat SK and Sachdev A. Hypokalemic paralysis. Postgrad Med J. 1999; 75(882):193-197.
  3. Mount DB, Zandi-Nejad K. Disorders of potassium balance. In Taal MW, Chertow GM, Marsden PA, et al., eds. Brenner and Rector’s The Kidney. 9th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 17.
  4. 4.0 4.1 Chinnery PF. Muscle diseases. In: Goldman L, Schafer AI, eds. Goldman’s Cecil Medicine. 24th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 429.
  5. Hypokalemic Periodic Paralysis. Periodic Paralysis International. http://hkpp.org/physicians/hypokalemic_pp