Léri-Weill syndrome

Alternative eponyms

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A skeletal dysplasia combining dorsal subluxation of the distal end of the ulna (Madelung deformity) with mesomelic short stature.

Description

A skeletal dysplasia combining dorsal subluxation of the distal end of the ulna (Madelung deformity) with mesomelic short stature. Bilaterality of lesions characterizing element. For unilateral lesions, see Madelung’s syndrome. According to some authors, the Madelung deformity never occurs isolated, always being a partial symtpom of the Léri-Weill syndrome.

Both sexes affected but prevalent and somewhat more severe expression in females; detection at birth to end of growing period. Wrist pain when lifting object. Forearms shorter with respect to hands and upper arm; distal ulna dorsal dislocation; dislocation easily reduced but unstable. Limited motion of elbows and wrists; legs shorter with respect to thighs. Autosomal dominant inheritance (sex influenced?).

See also Madeluing deformity, under Otto Wilhelm Madelung, German surgeon, 1846-1926.

The terms Léri-Weill mesomelic dwarfism, Léri-Weill dyschondrosteosis, Leri-Weill syndrome and Lamy-Bienefeld syndrome have also been used as synonyms for Langer’s syndrome, another mesomelic dwarfism. This has been entered as Langer’s syndrome, under Leonard O. Langer Jr., US radiologist, born 1928.

Bibliography

  • A. Léri, J. A. Weill:
    Une affection congénitale et symétrique du développement osseux. La dyschondrostéose.
    Bulletins et memoires de la Société medicale des hôpitaux de Paris, 1929, 53: 1491-1494.
  • M. Lamy, C. Bienenfeld:
    La Dyschondreosteose.
    In Gedda (ed): Analecta Genetica, pp 153-164. Rome, Mendel Institute, 1954.

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