Pathophysiologic and anesthetic considerations for patients with myotonia congenita or periodic paralyses

Authors

  • Oliver Bandschapp,

    Corresponding author
    • Department of Anesthesia, Surgical Intensive Care, Prehospital Emergency Medicine and Pain Therapy, University Hospital Basel, Basel, Switzerland
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  • Paul A. Iaizzo

    1. Departments of Surgery and Integrative Biology & Physiology, The Malignant Hyperthermia Diagnostic Center, University of Minnesota, Minneapolis, MN, USA
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Correspondence

Oliver Bandschapp, Department of Anesthesia, Surgical Intensive Care, Prehospital Emergency Medicine and Pain Therapy, University Hospital Basel, CH-4031 Basel, Switzerland

Email: Oliver.Bandschapp@usb.ch

Summary

Myotonia congenita and periodic paralyses are hereditary skeletal muscle channelopathies. In these disorders, various channel defects in the sarcolemma lead to a severely disturbed membrane excitability of the affected skeletal muscles. The clinical picture can range from severe myotonic reactions (e.g., masseter spasm, opisthotonus) to attacks of weakness and paralysis. Provided here is a short overview of the pathomechanisms behind such wide-ranging phenotypic presentations in these patients, followed by recommendations concerning the management of anesthesia in such populations.

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