Friday, 7 September 2018

HYPERKALEMIC PP VS HYPOKALEMIC PERODIC PARALYSIS

  1. HYPERKALEMIC PERIODIC PARALYSIS MUTATED GENE SCN4A CHROMOSOME 17q DEFECTIVE CHANNEL SODIUM MODE OF INHERITENCE AUTOSOMAL DOMINANT 
  2.  Hyperkalemic Periodic Paralysis • term hyperkalemic is misleading since patients are often normokalemic during attacks. • Onset first decade • M : F 1:1 • Attacks are brief and mild, usually lasting 30 minutes to 4 hours. • Weakness affects proximal muscles, sparing bulbar muscles. • Attacks are precipitated by rest following exercise and fasting. 
  3.  In a variant of this disorder, the predominant symptom is myotonia without weakness (potassium-aggravated myotonia). • The symptoms are aggravated by cold, and myotonia makes the muscles stiff and painful. • Clinically apparent myotonia is seen less than 20% of patients, but electrical myotonia may be found in 50-75%. 
  4. Pathophysiology  In hyperKPP, Na+ channels fail to inactivate and prolonged openings and depolarization result.  Increased extracellular K+ levels worsen the inactivation of Na+ channels.

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