Hyperkalemic periodic paralysis (HyperPP)

Definition:

The periodic paralyses are a group of autosomal-dominant disorders of skeletal muscle sodium, potassium, and calcium channel genes. They are characterized by episodes of muscle weakness associated with variations in serum potassium concentration. Hyperkalemic periodic paralysis (HyperPP) is caused by gain-of-function mutations in the alpha-subunit of the skeletal muscle voltage-gated sodium channel, Nav1.4. However, 20% of cases remain genetically undefined, suggesting genetic heterogeneity. HyperPP is characterized by attacks of flaccid limb paralysis or, rarely, weakness of the eye and throat muscles. Triggers for these attacks include ingestion of potassium-rich food, rest after strenuous exercise, and cold exposure.

References:

[1]. Chad R Heatwole, et al. The nondystrophic myotonias. Neurotherapeutics. 2007 Apr;4(2):238-51. [Content Brief]

[2]. Daniel Platt, et al. Skeletal muscle channelopathies: new insights into the periodic paralyses and nondystrophic myotonias. Curr Opin Neurol. 2009 Oct;22(5):524-31. [Content Brief]

[3]. Dipa L Raja Rayan, et al. Skeletal muscle channelopathies: nondystrophic myotonias and periodic paralysis. Curr Opin Neurol. 2010 Oct;23(5):466-76. [Content Brief]

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