[Anesthesia in a Patient with McArdle Disease].

ABSTRACT

McArdle disease, known as type V glycogen storage disease, is a rare skeletal muscle disorder. Patients with McArdle disease lack skeletal muscle specific glycogen phosphorylase, and myophosphorylase. This subsequently leads to an elevation in serum creatine kinase levels, and results in suffering from exercise intolerance. They have such anesthesiological problems as follows; rhabdomyolysis, myoglobinuria, acute renal failure, and a higher risk of developing malignant hyperthermia. We report a case of general anesthesia for a patient with McArdle disease. The patient was a 38-year-old woman who underwent dilation and curettage. Anesthesia was induced by intravenous administrations of fentanyl and propofol. For maintenance of anesthesia, we used total intravenous anesthesia with propofol. We avoided the use of neuromuscular blockades throughout the operation. There were no signs of rhabdomyolysis, myoglobinuria, worsening muscle weakness, or occurrence of malignant hyperthermia in the perioperative period, and the patient recovered uneventfully.