My opinion

By Dr. Georg Bollig
Corresponding Author Dr. Georg Bollig
Department of Surgical Sciences, University of Bergen, Haukeland University Hospital - Norway 5021
Submitting Author Dr. Georg Bollig

Anaesthesia, McArdles disease, myophosphorylase insuffiency, glycogenosis type V, malignant hyperthermia, caffeine halothane contracture test , myoglobinuria, muscle cramps, exercise intolerance

Bollig G. Can Inhaled Induction Be Considered As Safe In Patients With Mc Ardles Disease?. WebmedCentral ANAESTHESIA 2010;1(11):WMC001220
doi: 10.9754/journal.wmc.2010.001220
Submitted on: 27 Nov 2010 10:49:14 AM GMT
Published on: 29 Nov 2010 08:27:11 PM GMT

My opinion

McArdle’s disease (myophosphorylase deficiency) is a glycogen storage disease, which can present with symptoms as exercise intolerance, muscle cramps, fatigue, rhabdomyolysis, myoglobinuria and has been associated with the possible risks of acute renal failure and malignant hyperthermia [1,2]. Isaacs et al. suggested that all patients with McArdle’s disease should be tested for malignant hyperthermia (MH) prior to surgery [3]. Benca and Hogan [4] suggested that inhaled induction of anaesthesia with “the newer potent inhaled anaesthetics sevoflurane or desflurane” in order to place an IV catheter may be an option in patients with ”rare enzyme defects” (like McArdle’s disease). They stated that the evidence for an association between McArdle’s disease and malignant hyperthermia (MH) is weak although some patients have a positive caffeine halothane contracture test (CHCT) [4]. No cases of malignant hyperthermia in patients with McArdle’s disease have been described so far, but one patient had rhabdomyolysis and hyperthermia intraoperatively [5]. Although the authors of this case report concluded that the rhabdomyolysis and hyperthermia were linked to a reaction to protamine they stated:
“The presence of McArdle’s disease may have rendered this patient more susceptible to
skeletal muscle injury when increased energy demand ensued, thus resulting in massive rhabdomyolysis and renal failure.” [5]
Patients with McArdle’s disease might react with muscle cramps or rhabdomyolysis, myoglobinuria to different causes and have a risk for developing renal failure due to rhabdomyolysis. Therefore one should avoid muscle ischemia as e.g. caused by tourniquets and try to prevent shivering [2]. An own study included a patient with McArdle´s disease and a positive CHCT. This patient had had an episode of tachycardia and hypotonia under general anesthesia with the use of both succinyl, halothane and isoflurane men did not develop MH [2].
At present it is unclear if a positive CHCT test result in McArdle’s disease is associated with the risk for MH or if it is just a reaction of a muscle running out of fuel. Until this question is answered one should treat patients with McArdle’s disease as patients with the risk of developing MH. In general whenever giving anaesthesia to patients with McArdle’s disease the anaesthetist should avoid MH-trigger substances and consider the recommendations given by me, Mohr and Ræder if not otherwise contraindicated [2].
The evidence for a connection between McArdle’s disease and MH is weak and the risk of developing MH is low for patients with McArdle’s disease. Nevertheless we do not know yet how to interpret a positive test result in the CHCT in McArdle patients. Taking into account the present state of scientific knowledge inhaled induction cannot be recommended as a safe option for patients with McArdle’s disease.
Dr. med. Georg Bollig, MAS, DEAA
Department of Surgical Sciences
Haukeland University Hospital
University of Bergen
5021 Bergen


1. McArdle B. Myopathy due to a defect in muscle glycogen breakdown. Clin Sci 1951; 24:13-35.
2. Bollig G, Mohr S, Ræder J. McArdle`s disease and anaesthesia. Case reports, review of potential problems and association with malignant hyperthermia. Acta Anaesthesiol Scand 2005; 49:1077-1083.
3. Isaacs  H, Badenorst ME, Du Sautoy C. Myophosporylase B deficiency and Malignant Hyperthermia. Muscle & Nerve 1989;12: 203-205.
4. Benca P, Hogan K. Malignant Hyperthermia, Coexisting Disorders, and Enzymopathies: Risks and Management Options. Anesth analg 2009;109:1049-53.
5. Lobato EB, Janelle GM, Urdaneta F, Malias MA. Noncardiogenic pulmonary edema and rhabdomyolysis after protamine administration in a patient with unrecognized McArdle’s disease. Anesthesiology 1999; 91: 303-305.

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