Periodic Paralysis
Basics
Description
Description
- Periodic paralysis (PP): Disorder of muscle metabolism, usually inherited, that leads to flaccid extremity weakness
- Primary: Familial autosomal dominate (AD) mutation in skeletal muscle calcium, sodium, or potassium channels
- Secondary: Due to thyrotoxicosis, hypokalemia, or hyperkalemia
Epidemiology
Incidence and Prevalence EstimatesEpidemiology
- Hypokalemic PP (HypoPP):
- Prevalence: 1:100,000
- 1/3 are new AD mutations
- Hyperkalemic PP (HyperPP):
- Prevalence: 1:200,000
- 90% of people with mutation will have clinical symptoms
- Thyrotoxic PP (ThyroPP):
- Incidence: 2% in patients with thyrotoxicosis
- Higher in Asians
- Subset of HypoPP, clinically identical
- Andersen–Tawil:
- Subset of HypoPP
- Rare
- Prevalence unknown
Etiology
Etiology
- Mutation of skeletal muscle Na channel gene:
- SCN4A
- HypoPP, HyperPP:
- AD inheritance
- Spontaneous mutation
- Mutation of skeletal muscle calcium channel gene CACN1AS:
- HypoPP
- Mutation of KCNJ2 gene:
- Andersen–Tawil:
- AD inheritance
- 50% spontaneous
- Andersen–Tawil:
- M > F
- Age of onset:
- HypoPP:
- First or second decade
- HyperPP:
- First decade
- Andersen–Tawil:
- First or second decade
- ThyroPP:
- Second–fifth decade
- HypoPP:
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Citation
Schaider, Jeffrey J., et al., editors. "Periodic Paralysis." 5-Minute Emergency Consult, 6th ed., Lippincott Williams & Wilkins, 2020. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis.
Periodic Paralysis. In: Schaider JJJ, Barkin RMR, Hayden SRS, et al, eds. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis. Accessed November 3, 2024.
Periodic Paralysis. (2020). In Schaider, J. J., Barkin, R. M., Hayden, S. R., Wolfe, R. E., Barkin, A. Z., Shayne, P., & Rosen, P. (Eds.), 5-Minute Emergency Consult (6th ed.). Lippincott Williams & Wilkins. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis
Periodic Paralysis [Internet]. In: Schaider JJJ, Barkin RMR, Hayden SRS, Wolfe RER, Barkin AZA, Shayne PP, Rosen PP, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. [cited 2024 November 03]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis.
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