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Kocher-Debré-Semélaigne syndrome or disease

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A syndrome of hypothyroidism associated muscular enlargement to give the appearance of an infant Hercules. The enlarged muscles are weak and slow to contract and relax. There is clinical or subclinical myxoedema, lower extremity or generalized muscular hypertrophy, and typical manifestations of cretinism. Observed in infancy and childhood. Absence of painful spasms and pseudomyotonia is the major point of difference from the (Johann) Hoffmann syndrome.

Bibliography

  • Th. Kocher:
    Zur Verhütung des Cretinismus und cretinoider Zustände nach neueren Forschungen.
    Deutsche Zeitschrift für Chirurgie, Leipzig, 1892, 34: 556-626.
  • R. Debré, G. Sémélaigne:
    Hypertrophie musculaire généralisée du petit enfant.
    Bulletin de la Société de pédiatrie, Paris, 1934, 32: 699-706. Syndrome of diffuse muscular hypertrophy in infants causing athletic appearance: its connection with congenital myxedema.
    American Journal of Diseases of Children, Chicago, 1935, 50: 1351-1361.
  • J. Hoffmann:
    Weiterer Beitrag zur Lehre von der Tetanie.
    Deutsche Zeitschrift für Nervenheilkunde, 1896, 9: 278-290.

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