Canadian Anaesthetists’ Society Journal

, Volume 16, Issue 5, pp 372–376 | Cite as

A propos de la dystrophie myotonique

  • Y. Desnoyers
Article

Summary

Dystrophia myotonica is hereditary and causes systemic effects subsequent to myotonia and muscular atrophy. Pathohistological findings in the muscle fibre and the terminal plate are characteristic. Physiopathological theories are numerous and are related to metabolic disturbances of either the muscle or the neuromuscular junction. These patients are hypersensitive to respiratory depressants used during anaesthesia. A new description is presented of atypical heterozygotous pseudocholinesterases found in a case of dystrophia myotonica. Following familial investigation, a new genetic hypothesis is formulated in an attempt to explain another danger in relation to anaesthesia in these patients.

Résumé

La dystrophic myotonique est une maladie héréditaire impliquant une multitude de systèmes, accompagnée de myotome et ďatrophie musculaire. Ľhistologie de la fibre musculaire et celle des plaques terminales est caractéristique. La physiopathologie fait ľobjet de théories multiples impliquant des perturbations du métabolisme de la myofibrille ou de la jonction myoneurale. Ľanesthésie de ces patients présente des problèmes reliés a une sensibilité accrue aux dépresseurs de la respiration. Nous avons présenté la première description ďatypie pseudocho-linestérasique homozygote dans un cas de dystrophic myotonique. Après investigation familiale, nous croyons ajouter une nouvelle hypothèse génétique possiblement reliée à certains de ces cas ou du moins mentionner un nouveau danger relié à ľanesthésie de ces patients.

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Copyright information

© Canadian Anesthesiologists 1969

Authors and Affiliations

  • Y. Desnoyers
    • 1
  1. 1.Départment ďanesthésieHôpital Ste-Jeanne ďArcMontréal, Québec

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