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Paralysis, Hyperkalemic Periodic

An autosomal dominant familial disorder which presents in infancy or childhood and is characterized by episodes of weakness associated with hyperkalemia. During attacks, muscles of the lower extremities are initially affected, followed by the lower trunk and arms. Episodes last from 15-60 minutes and typically occur after a period of rest following exercise. A defect in skeletal muscle sodium channels has been identified as the cause of this condition. Normokalemic periodic paralysis is a closely related disorder marked by a lack of alterations in potassium levels during attacks of weakness. (Adams et al., Principles of Neurology, 6th ed, p1481)

Year introduced: 2000

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Tree Number(s): C05.651.701.600, C10.668.491.650.600, C16.320.565.618.711.600, C18.452.648.618.711.600

MeSH Unique ID: D020513

Entry Terms:

  • Adynamia Episodica Hereditaria with or without Myotonia
  • Familial Hyperkalemic Periodic Paralysis
  • Gamstorp Disease
  • Disease, Gamstorp
  • Gamstorp Episodic Adynamy
  • Sodium Channel Muscle Disease
  • Hyperkalemic Periodic Paralysis, Familial
  • HyperKPP
  • HyperPP
  • Myotonic Periodic Paralysis
  • Paralysis, Periodic, Hyperkalemic, Familial
  • Primary Hyperkalemic Periodic Paralysis
  • Adynamia Episodica Hereditaria
  • Hyperkalemic Periodic Paralysis
  • Hyperkalemic Periodic Paralysis Type 2
  • Hyperkaliemic Periodic Paralysis Type 2

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