Guérin-Stern syndrome
Related people
- Adolph Wilhelm Otto
- Ettore Rossi
- Henri Gaston Louis Rocher
- Jules René Guérin
- Sir Wilfrid Percy Henry Sheldon
- Walter G. Stern
Historic term for the sequence of intrauterine hypokinesia (arthrogryposis). A clinical syndrome of congenital contracture of numerous joints in a flexed of contracted position with hypoplasia of the attached musculature and multiple pterygia. The usual wrist deformities consist of flexion and ulnar deviation, sometimes in association with extension contractures. Carpal malalignment is followed by narrowing of the intercarpal joint spaces and fusion. Other abnormalities in the hand may include syndactyly, amputation, camptodactyly, and delayed maturation. Usually combined with clubfoot, vertical talus, rocker bottom foot, hip dislocation. Occurs in three types: myopathic, neuropathic, and distal type.
Bibliography
- A. W. Otto:
Monstrorum sexcentorum descriptio anatomica.
Bratislava, 1841. - J. R. Guérin:
Recherches sur les difformitées congénitales chez les monsters.
Paris, 1880. - H. L. Rocher:
Les raideurs articulaires congénitales multiples.
Journal de médecine de Bordeaux, 1913, 43: 772-780. - W. G. Stern:
Arthrygryposis multiplex congenita.
Journal of the American Medical Association, Chicago, 1923, 81: 1507-1510. - W. Sheldon:
Amyoplasia congenita (multiple congenital articular rigidity: arthrogryposis multiplex congenita).
Archives of Disease in Childhood, London, 1932, 7: 117-136. - E. Rossi:
Le syndrome arthromyodysplasique congénital (contribution à l’étude de l’arthrogryposis multiplex congenita).
Helvetica Paediatrica Acta, Basel, 1947, 2: 82-97.