Biedl Syndrome

Pediatrics, Neurology

Also known as: Bardet-Biedl Syndrome, Laurence-Moon-Biedl Syndrome

Historical document, translated for reference. It reflects medical knowledge of the 1920s–30s and is not medical advice.

Summary

Biedl distinguishes two forms of dystrophia adiposo-genitalis: purely hypophyseal (so-called Fröhlich's disease) and purely cerebral. The latter form differs from the classic hypophyseal form by the presence of mental deficiency, a number of degenerative stigmata (atresia ani, polydactyly, pigmentary retinitis), and skull deformation, without changes in the hypophysis or signs of brain compression.

Encyclopedia entry (1928–1936)

BIEDL SYNDROME (or Bardet syndrome). Biedl distinguishes two forms of dystrophia adiposo-genitalis: purely hypophyseal (so-called Fröhlich's disease) and purely cerebral. The latter form differs from the classic hypophyseal form in that, in addition to the symptoms characteristic of the classic hypophyseal form - underdevelopment of the sexual organs and obesity - it exhibits the following syndrome: mental deficiency and a number of degenerative stigmata (atresia ani, polydactyly, pigmentary retinitis), as well as skull deformation. Characteristic of this condition is the absence of changes in the hypophysis and signs of compression from the brain. Biedl associates this syndrome with delayed development of the diencephalon.

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Cite this page

“Biedl Syndrome.” Soviet Medical Encyclopedia. English translation of Bolshaya Meditsinskaya Entsiklopediya, 1st ed. (Moscow, 1928–1936), ed. N. A. Semashko. https://sovietmedicalencyclopedia.pages.dev/article/biedl-syndrome/