Hyperkal periodic paralysis triggers

Clinical - Neuromuscular Diseases and Disorders

Hyperkalemic periodic paralysis is an autosomal dominant  disease leading to intermittent weakness associated with hyperkalemia and often precipitated by a potassium-rich meal, rest after exercise, or stressful situations. The paralysis lasts up to an hour

Caused (at least partly) by mutations in sodium channel NaV1.4

Cholinesterase inhibitors (ex. neostigmine), depolarizing muscle relaxants (SCh), and potassium can aggravate hyperkalemic periodic paralysis. Hyperkalemic periodic paralysis patients may remain paralyzed for several hours after anesthesia/surgery. Maintain normal body temperature, avoid hyperkalemia and hypoglycemia.  Most sources report that there is no increased risk for MH, which makes sense because the NaV1.4 gene is encoded on chromosome 17 whereas the ryanodine receptor 1 (RYR-1, implicated in MH) is encoded on chromosome 19. Nevertheless, some sources (i.e., https://www.mhaus.org/healthcare-professionals/be-prepared/associated-conditions/) report an association.  Thus, patients are best treated with a non-trigger anesthetic in the absence of a clear answer.

Treat a hyperkalemic episode with glucose, insulin, epinephrine, and calcium. β-agonists may also be helpful

Hyperkalemic Periodic Paralysis

  • Etiology: mutations in sodium channel NaV1.4
  • Precipitated By: hyperkalemia, potassium-rich meal, rest after exercise, stressful situations, possibly hypoglycemia
  • Anesthesia Management: avoid cholinesterase inhibitors, SCh, and potassium. Maintain normothermia and normoglycemia
  • Treatment: glucose, insulin, epinephrine, β-agonists, and calcium

Also note – Hypokalemic periodic paralysis is (decrease in serum potassium) can be triggered by stress, exercise, high-carbohydrate or salty meals, hypothermia, menstruation, pregnancy, or insulin. Unlike in hyperakalemic periodic paralysis, EMG does not usually show myotonia. Attacks are more severe than in hyperakalemic periodic paralysis. Autosomal dominant disorder of CaV1.1 and NaV1.4 channels, most commonly affecting muscle groups in the arms and legs (but can affect swallowing and respiratory muscles). General anesthesia, stress, glucose additive (to IVF), and long-acting NMBs are associated with postoperative paralysis. Consider epidural analgesia

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2014

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