Anesthetic management of a patient with myotonia congenita

Abstract

Myotonia congenita is also known as Becker's disease. It is a genetic disorder in which skeletal muscles are unable to relax quickly after voluntary movements. Symptoms may vary from person to person and may include muscle stiffness and weakness initiated by movement after rest. Myotonia congenita was first described by Thomsen in 1876 as a disorder of skeletal muscles. Patients suffer from muscle contractility and fatigue which occurs as a consequence of the mutation of the chloride channel gene on the 17th chromosome. These patients have a high risk of developing malignant hyperthermia during anesthesia, and call for full preparation to prevent and deal with this complication.