Anesthesia in Hypokalemic Periodic Paralysis
Provided by Frank Lehmann-Horn MD, PhD
Lehmann-Horn F, Rüdel R, Jurkat-Rott K. Chapter 46: Nondystrophic myotonias and periodic paralyses. In: Myology, edited by AG Engel, C Franzini-Armstrong. McGraw-Hill, New York, 3rd edition, 2004, pp. 1257-1300.
Klingler W, Lehmann-Horn, Jurkat-Rott K. Complications of anesthesia in neuromuscular disorders. Neuromuscular Disord, 15:195-206, 2005.
In patients with hypokalemic periodic paralysis (HypoPP), hypothermia, hypokalemia, sodium chloride and glucose infusions as well as myotoxic substances like succinylcholine in the operating room often lead to flaccid muscle weakness and respiratory distress in the recovery room [Siler and Discavage 1975, Melnick et al. 1983, Rollman and Dickson 1985, Lema et al. 1991]. The hypokalemia is induced by operation-induced stress that leads to K+ uptake into muscle via release of catecholamines, insulin, and other hormones. Keeping the patients warm and serum K+ at high level and avoiding hyperglycemia are essential measures in preventing such attacks [Lema et al. 1991].
A malignant hyperthermia crisis has once been convincingly described for a patient supposed to have hypokalemic periodic paralysis [Lambert et al. 1994], but the diagnosis has not been verified by molecular genetics. Hyperthermic responses or results of the in vitro contracture tests for malignant hyperthermia susceptibility were not detailed in other case reports [Rajabally & El Lahawi, 2002].
In summary, typical anesthetic events in HypoPP patients are characterized by hypothermia and hypokalemia-induced flaccid weakness including ventilatory muscles and no signs of hypermetabolism in contrast to malignant hyperthermia which is characterized by a hypermetabolic reaction associated with hyperthermia and hypercontracted stiff muscles.
- Lambert C, Blanloeil Y, Horber RK, et al. Malignant hyperthermia in a patient with hypokalemic periodic paralysis. Anesth Analg 1994; 79:1012-4.
- Lema G, Urzua J, Moran S, Canessa R: Successful anesthetic management of a patient with hypokalemic familial periodic paralysis undergoing cardiac surgery. Anesthesiology 1991; 74:373-5.
- Melnick B, Chang JL, Larson CE, Bedger RC: Hypokalemic familial periodic paralysis. Anesthesiology 58:263-265, 1983.
- Rajabally YA, El Lahawi M: Hypokalemic periodic paralysis associated with malignant hyperthermia. Muscle Nerve 25:453-455, 2002.
- Rollman JE, Dickson CM: Anesthetic management of a patient with hypokalemic familial periodic paralysis for coronary artery bypass surgery. Anesthesiology 63:526-527, 1985.
- Siler JN, Discavage WJ: Anesthetic management of hypokalemic periodic paralysis. Anesthesiology 43:489-490, 1975.