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

@article{Bandschapp2013PathophysiologicAA,
  title={Pathophysiologic and anesthetic considerations for patients with myotonia congenita or periodic paralyses.},
  author={Oliver Bandschapp and Paul Anthony Iaizzo},
  journal={Paediatric anaesthesia},
  year={2013},
  volume={23 9},
  pages={824-33}
}
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… CONTINUE READING
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State of the art in hereditary muscle channelopathies

Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology • 2010

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