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    Periodic Paralysis

    Periodic Paralysis is a topic covered in the 5-Minute Emergency Consult.

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    Basics

    Description

    • Periodic paralysis (PP): Disorder of muscle metabolism usually inherited that leads to flaccid extremity weakness. Exacerbated by hyperkalemia, hypokalemia, thyrotoxicosis
    • Primary: Familial AD mutation skeletal muscle calcium, sodium, or potassium channel
    • Secondary: Thyrotoxic, hypokalemia, hyperkalemia

    Epidemiology

    Incidence and Prevalence Estimates
    • Hypokalemic PP (HypoPP):
      • MC, 1:100,000 prevalence
      • 1/3 new AD mutations
    • Hyperkalemic PP (HyperPP):
      • 1:200,000 prevalence
      • 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

    • 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
    • M>F
    • Age of onset:
      • HypoPP:
        • 1st or 2nd decade
      • HyperPP:
        • 1st decade
      • Andersen–Tawil:
        • 1st or 2nd decade
      • ThyroPP:
        • 2nd–5th decade

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    Citation

    Rosen, Peter, et al., editors. "Periodic Paralysis." 5-Minute Emergency Consult, 5th ed., Lippincott Williams & Wilkins, 2016. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis.
    Periodic Paralysis. In: Rosen P, Shayne P, Barkin AZ, et al, eds. 5-Minute Emergency Consult. 5th ed. Lippincott Williams & Wilkins; 2016. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis. Accessed April 25, 2019.
    Periodic Paralysis. (2016). In Rosen, P., Shayne, P., Barkin, A. Z., Wolfe, R. E., Hayden, S. R., Barkin, R. M., & Schaider, J. J. (Eds.), 5-Minute Emergency Consult. Available from https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis
    Periodic Paralysis [Internet]. In: Rosen P, Shayne P, Barkin AZ, Wolfe RE, Hayden SR, Barkin RM, Schaider JJ, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2016. [cited 2019 April 25]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis.
    * Article titles in AMA citation format should be in sentence-case
    TY - ELEC T1 - Periodic Paralysis ID - 307662 ED - Rosen,Peter, ED - Shayne,Philip, ED - Barkin,Adam Z, ED - Wolfe,Richard E, ED - Hayden,Stephen R, ED - Barkin,Roger M, ED - Schaider,Jeffrey J, BT - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307662/all/Periodic_Paralysis PB - Lippincott Williams & Wilkins ET - 5 DB - Emergency Central DP - Unbound Medicine ER -