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Guérin-Stern syndrome
Also known as:
Otto's syndrome
Rocher-Sheldon syndrome
Rossi's syndrome
Associated persons:
Jules René Guérin
Adolph Wilhelm Otto
Henri Gaston Louis Rocher
Ettore Rossi
Sir Wilfrid Percy Henry Sheldon
Walter G. Stern
Description:
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.
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