Advertisement

Springer Nature is making SARS-CoV-2 and COVID-19 research free. View research | View latest news | Sign up for updates

A propos de la dystrophie myotonique

  • 176 Accesses

  • 5 Citations

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.

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.

References

  1. 1.

    Kaufman, L. Anaesthesia in Dystrophia Myotonica. Proc. Roy. Soc. Med.53: 183 (1959)

  2. 2.

    Gillam, P. M.;Heaf, P. J.; &Kaufman, L. Respiration in Dystrophia Myotonica Thorax.19: 112 (1964).

  3. 3.

    Lee, F. I. &Hughes, D. T. Systemic Effects in Dystrophia Myotonica. Brain.87: 521 (1964).

  4. 4.

    MacDermot, V. The Histology of the Neuromuscular Junction in Dystrophia Myotonica. Brain.84: 75 (1961).

  5. 5.

    Rosman, N. P. &Rebeiz, J. J. The Cerebral Defect and Myopathy in Myotonic Dystrophy. Neurology.17: 1106 (1967).

  6. 6.

    Dundee, J. W. Thiopentone in Dystrophia Myotonica. Curr. Res. Anaesth.31: 257 (1952).

  7. 7.

    Geschwind, N. &Simpson, J. A. Procaine Amide in the Treatment of Myotonia. Brain.78: 81 (1955).

  8. 8.

    McClelland, R. M. The Myasthenic State and the Myotonic Syndrome. Brit. J. Anaesth.32: 81 (1960).

  9. 9.

    Welsh, J. D.;Haase, G. R.; &Bynum, T. E. Myotonic Muscular Dystrophy. Arch. Internal. Med.114: 669 (1964).

  10. 10.

    Pruzanski, W. &Profis, A. Pulmonary Disease in Myotonic Dystrophy. Am. Rev. Resp. Dis.91: 874 (1965).

  11. 11.

    Paterson, I. S. Generalized Myotonia Following Suxamethonium. Brit. J. Anaesth.34: 340 (1962).

  12. 12.

    Brodeur, J.;Martin, P.;Authier, L.; &Lebel, E. Pseudocholinestérase atypique et apnée prolongée à la succinylcholine. Union Med.97: 1025 (1968).

  13. 13.

    Wylie &Churchill-Davidson. A Practice of Anaesthesia, 2nd ed. Lloyd-Luke Ltd. (1966).

  14. 14.

    Brown, G. L. &Harvey, A. M. Congenital Myotonia in the Goat. Brain.62: 341 (1939).

  15. 15.

    Pearson, C. M. Serum Enzymes in Muscular Dystrophy. New England J. Med.256: 1069 (1957).

  16. 16.

    Brain, Russel. Diseases of the Nervous System, 5th ed. London: Oxford University Press (1960).

Download references

Author information

Correspondence to Y. Desnoyers.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Desnoyers, Y. A propos de la dystrophie myotonique. Can. Anaes. Soc. J. 16, 372–376 (1969). https://doi.org/10.1007/BF03004481

Download citation

Keywords

  • Succinylcholine
  • Thiopentone
  • Nous Avons
  • Dibucaine
  • Respiratory Depressant