Table 6.

Treatment of Manifestations in Individuals with Hyperkalemic Periodic Paralysis (hyperPP)

Attacks of flaccid muscle weakness Continuing mild exercise &/or oral ingestion of carbohydrates (2 g glucose per kg body weight) at onset of weaknessMay prevent or abort attacks
IV glucocorticoids or inhalation of 2 puffs of 0.1 mg salbutamolMay abort or attenuate attacks
Calcium gluconate (0.5-2 g taken intravenously) 1May terminate attacks in some persons
Myotonia-assoc issues w/general anesthesia 2 An induction sequence incorporating inhalation of oxygen, cricoid pressure, thiopental, & 2x the ED95 dose of an intermediate or short-action non-depolarizing muscle relaxant, followed by intubation, is a reasonable approach to securing the airway in persons w/hyperPP.
  • Avoid opioids or depolarizing agents incl potassium, anticholinesterases, & succinylcholine, which can aggravate a myotonic reaction & induce masseter spasms & stiffness of respiratory muscles.
  • Alterations of serum osmolarity, pH, & hypothermia-induced muscle shivering & mechanical stimuli can exacerbate myotonic reaction.
Inhalational induction may also be possible for hyperPP & is well tolerated in those undergoing elective surgery.
Post anesthesia general & respiratory muscle weakness To prevent this complication the following are recommended: 3
  • Glucose infusion
  • Maintain normal body temperature.
  • Maintain serum potassium at low level.
Myotonia Mexiletine has been used to treat myotonia in this disorder [Modoni et al 2020].

IV = intravenous


One case report suggested that intravenous magnesium is beneficial as well [Mankodi et al 2015].


Because the generalized muscle spasms associated with such attacks may lead to an increase in body temperature, individuals with hyperPP have been considered to be susceptible to malignant hyperthermia. Most likely, anesthesia-related complications suggestive of a malignant hyperthermia crisis result from severe myotonic reactions [Lehmann-Horn et al 2004, Klingler et al 2005].


From: Hyperkalemic Periodic Paralysis

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