Landouzy-Dejerine syndrome

Alternative eponyms

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A hereditary from of progressive muscular dystrophy with atrophic changes in the muscles of the face and scapulohumeral group.

Description

A hereditary from of progressive muscular dystrophy with atrophic changes in the muscles of the face and scapulohumeral group. Begins with weakness of the muscles of the face and shoulder girdle, causing inability to raise the arms above the head (in female, noticed while combing hair), myopathic facies, eyelids that remain partly open in sleep, and inability to whistle or to purse the lips (tapir mouth). The condition is slowly progressive and the musculature of other regions is eventually involved. Deafness and tortuosity of the retinal vessels may be associated. Onset usually takes place during childhood and adolescence, most commonly at age 12-14 years, but may be as late as age 30 to 40. Both sexes equally affected. Aetiology unknown. Inheritance is usually autosomal dominant, but hereditary autosomal recessive and X-linked transmission also observed. The course of this muscular dystrophy is usually benign.

In their paper of 1886, Landouzy and Dejerine drew attention to the familial nature of the disorder and mentioned that four generations were affected in the kindred that they had investigated. One of their cases was a female of 9 years who died in 1964 at the age of 86 years.

Bibliography

  • G. B. A. Duchenne de Boulogne:
    Album de Photographies Pathologiques.
    Ballière et fils. Paris, 1862. De l'électrisation localisée et de son application à la physiologie, à la pathologie et à la thérapeutique.
    Paris, J. B. Baillière et fils, 1855; 2nd edition, 1861; 3rd editon, 1872.
  • L. Landoyzy, J. Dejerine:
    De la myopathie atrophique progressive (myopathie héréditaire, débutant dans l’enfance par la face, sans altération du système nerveux).
    Comptes rendus de l’Académie des sciences, Paris, 1884, 98: 53-55. De la myopathie atrophique progressive; myopathie héréditaire, sans neuropathie, debutant d’ordinaire dans l’enfance par la face.
    Paris, F. Alcan, 1885. Contribution à l’étude de la myopathie atrophique progressive (myopathie atrophique progressive, à type scapulo-huméral).
    Comptes rendus de la Société de biologie, 1886, 8 sér, volume 38: 478-481.

  • W. H. Erb:
    Dystrophia musculorum progressiva.
    Deutsche Zeitschrift für Nervenheilkunde, Berlin, 1891; 1: 173.
  • L. Justin-Besançon, H. Pequignot, F. Contamin, P. Delavierre, P. Roland:
    Myopathie du type Landouzy-Dejerine. Rapport d’une observation historique.
    Semaine des hôpitaux de Paris, 1964, 40: 2990.

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