The Periodic Paralyses

A basic chart

by Quest Staff on July 1, 2009 - 3:04pm

QUEST Vol. 16, No. 3
The Periodic Paralyses
Disorder Hyperkalemic Periodic Paralysis Hypokalemic Periodic Paralysis Andersen-Tawil Syndrome
Location of problem sodium channel calcium channel (most common) sodium channel potassium channel
Location of gene chromosome 17 chromosome 1
chromosome 17
chromosome 17
Inheritance pattern dominant dominant dominant
Functional defect channel does not close properly; prolonged sodium leak into cell calcium channel on cell surface does not transmit signal for interior calcium release channel does not open properly; potassium can’t leave cell
Average age of onset before age 10 age 5 to 35 age 2 to 18
Average duration of episodes 30 minutes to 4 hours 2 to 24 hours 1 to 36 hours
Maximum weakness mild to severe severe moderate
Development of permanent weakness may occur; increases with age may occur; increases with age may occur; increases with age
Muscle pain may occur in exercised muscles may occur in exercised muscles may occur in exercised muscles
Episode triggers high blood potassium; high potassium intake; fasting; cold temperatures; certain anesthetics; depolarizing muscle relaxants low blood potassium; high carbohydrate intake; rest after exercise; cold temperatures; certain anesthetics; depolarizing muscle relaxants high or low blood potassium, depending on exact genetic mutation; certain anesthetics; depolarizing muscle relaxants; other triggers consistent with either hyper- or hypokalemic PP
Treatment
  • hydrochlorothiazide, furosemide, acetazolamide or dichlorphenamide; glucose-insulin solution; inhaled albuterol; drugs that bind potassium
  • carbohydrate intake, low-potassium diet
  • frequent meals, warmth, keep moving
  • avoid certain anesthetics and depolarizing muscle relaxants
  • potassium supplements, acetazolamide (can harm some patients), dichlorphenamide, spironolactone or eplerenone
  • high-potassium, low-carbohydrate, low-sodium diet; warmth; keep moving
  • avoid certain anesthetics and depolarizing muscle relaxants
  • cardiac medications such as beta blockers and anti-arrhythmics, implanted pacemakerdefibrillator
  • acetazolamide or dichlorphenamide can help or harm
  • high- or low-potassium diet, depending on mutation
  • avoid certain anesthetics and depolarizing muscle relaxants
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