This Article Full Text Full Text (PDF) CME: Take the course for this article: Course on Myotonias and Malignant Hyperthermia Susceptibility Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Google Scholar Articles by Parness, J. Articles by Girard, T. PubMed Articles by Parness, J. Articles by Girard, T. Related Collections Complications Patient Safety Genetics Pediatrics

---

PEDIATRIC ANESTHESIOLOGY

The Myotonias and Susceptibility to Malignant Hyperthermia

Jerome Parness, MD, PhD^*, Oliver Bandschapp, MD, and Thierry Girard, MD

From the Departments of *Anesthesiology, Pharmacology, and Chemical Biology, Children's Hospital of Pittsburgh/University of Pittsburgh Medical Center, University of Pittsburgh, School of Medicine, Pittsburgh, Pennsylvania; and Department of Anaesthesia, University Hospital of Basel, Basel, Switzerland.

Address correspondence and reprint requests to Jerome Parness, MD, PhD, Department of Anesthesiology, Children's Hospital of Pittsburgh/University of Pittsburgh Medical Center, Rangos Research Center, Room 7121, 530 45th St., Pittsburgh, PA 15201. Address e-mail to parnessj{at}upmc.edu.

Abstract

Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle in which volatile anesthetics trigger a sustained increase in intramyoplasmic Ca²⁺ via release from sarcoplasmic reticulum and, possibly, entry from the extracellular milieu that leads to hypermetabolism, muscle rigidity, rhabdomyolysis, and death. Myotonias are a class of myopathies that result from gene mutations in various channels involved in skeletal muscle excitation-contraction coupling and sarcolemmal excitability, and unusual DNA sequence repeats that result in the inability of many proteins, including skeletal muscle channels that affect excitability, to undergo proper splicing. The suggestion has often been made that myotonic patients have an increased risk of developing MH. In this article, we review the physiology of muscle excitability and excitation-contraction coupling, the pathophysiology of MH and the myotonias, and review the clinical literature upon which the claims of MH susceptibility are based. We conclude that patients with these myopathies have a risk of developing MH that is equivalent to that of the general population with one potential exception, hypokalemic periodic paralysis. Despite the fact that there are no clinical reports of MH developing in patients with hypokalemic periodic paralysis, for theoretical reasons we cannot be as certain in estimating their risk of developing MH, even though we believe it is low.

This article has been cited by other articles:

				P. J. Davis and B. W. Brandom The Association of Malignant Hyperthermia and Unusual Disease: When You're Hot You're Hot, or Maybe Not Anesth. Analg., October 1, 2009; 109(4): 1001 - 1003. [Full Text] [PDF]