Hyperkalemic Periodic Paralysis

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)

Also Known As:

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

Musculoskeletal Diseases: 719
- Muscular Diseases: 10128
  - Familial Periodic Paralyses: 71
    - Hyperkalemic Periodic Paralysis: 170
Nervous System Diseases: 14178
- Neuromuscular Diseases: 2005
  - Muscular Diseases: 10128
    - Familial Periodic Paralyses: 71
      - Hyperkalemic Periodic Paralysis: 170
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Inborn Errors Metabolism: 1162
    - Inborn Errors Metal Metabolism
      - Familial Periodic Paralyses: 71
        Hyperkalemic Periodic Paralysis: 170
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - Inborn Errors Metabolism: 1162
    - Inborn Errors Metal Metabolism
      - Familial Periodic Paralyses: 71
        Hyperkalemic Periodic Paralysis: 170

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Disease

Drug/Important Bio-Agent (IBA)