Abstract

FOR MANY YEARS the etiology of the Laurence MoonBiedl syndrome has been a question of very unsatisfactory debate. Its establishment as a diagnostic entity, however, is quite secure with over ioo cases recorded in the literature. In 1866, Laurence and Moon (1) reported 4 cases of retinitis pigmentosa occurring in the same family, associated with other unusual abnormalities including hypogenitalism, obesity, mental deficiency, and dwarfism. The symptom complex, although observed occasionally by clinicians in the interim, was not recognized as a disease entity until 1920 when Bardet (2) reported an isolated case of polydactyly, adiposogenitalism, and retinitis pigmentosa. French authors are naturally inclined to associate his name with the syndrome. Biedl (3), in 1922, reported 2 cases in the same family, and he considered that the syndrome in its complete form would include any of such congenital malformations as polydactylism, ret’ initis pigmentosa, skull deformities, and atresia ani, as well as mental de...

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