Abstract
BackgroundSodium-channel myotonia (SCM) is a nondystrophic myotonia, characterized by pure myotonia without muscle weakness or paramyotonia. The prevalence of skeletal muscle channelopathies is approximately 1 in 100,000, and the prevalence of SCM is much lower. To our knowledge, this is the first report on anesthetic management of a patient with SCM.Case presentationA 23-year-old woman with congenital nasal dysplasia and SCM was scheduled to undergo rhinoplasty with autologous costal cartilage. Total intravenous anesthesia without muscle relaxants was administered followed by continuous intercostal nerve block. Although transient elevation of potassium level in the blood was observed during surgery, the patient did not show exacerbation of myotonic or paralytic symptoms in the postoperative period.ConclusionTotal intravenous anesthesia and peripheral nerve block can be administered safely to a patient with SCM. However, careful monitoring of the symptoms and electrolytes is recommended.
Highlights
Sodium-channel myotonia (SCM) is a nondystrophic myotonia, characterized by pure myotonia without muscle weakness or paramyotonia
Sodium-channel myotonia (SCM) belongs to a group of nondystrophic myotonias and is caused by mutations in the sodium-channel gene on chromosome 17q, encoding the α-subunit protein of the voltage-gated sodium channel Nav1.4 (SCN4A) expressed in the skeletal muscle [1]. It is characterized by pure myotonia, without muscle weakness or paramyotonia, and is triggered by exercise and the ingestion of potassium-rich food [1]
Skeletal muscle channelopathies are divided into periodic paralyses and nondystrophic myotonias [1]
Summary
Total intravenous anesthesia and peripheral nerve block can be administered safely to a patient with SCM.
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