Hypokalemic periodic paralysis may present as progressive muscle weakness with or without episodes of periodic paralysis

A Danish team followed 37 people with hypokalaemic periodic paralysis due to CACNA1S mutations for three years (26 to 52 months):

  • two were asymptomatic
  • 21 had only episodes of periodic paralysis,
  • two had isolated permanent muscle weakness,
  • 12 had episodes of periodic weakness associated with a permanent muscle strength deficit.

During follow-up :

  • two remained asymptomatic,
  • 11 showed a decline in muscle strength on manual testing, four of whom did not experience paralysis during the study;
  • 27 had an increase in muscle fatty infiltration on MRI, eight of whom had no paralytic attacks during the study.

 

Hypokalemic periodic paralysis: a 3-year follow-up study. Holm-Yildiz S, Krag T, Witting N et al. J Neurol. 2023 Sep 1.