THE LAURENCE-BIEDL SYNDROME OCCURRING IN A BROTHER AND SISTER
- 1 May 1938
- journal article
- other
- Published by American Medical Association (AMA) in Archives of Neurology & Psychiatry
- Vol. 39 (5) , 1033-1042
- https://doi.org/10.1001/archneurpsyc.1938.02270050159009
Abstract
The Laurence-Biedl syndrome has been reported many times. Its cardinal characteristics of obesity, genital dystrophy, mental deficiency, retinitis pigmentosa and polydactylism are generally known. The etiology of the symptom complex is still a subject of controversy. Until this disputed point is settled, all variants or true syndromes of this type deserve recording. Biedl1suggested that the syndrome is a familial form of dystrophia adiposogenitalis of cerebral rather than pituitary origin. This is generally accepted. Raab2expressed the belief that it results from dysfunction of the hypothalamic centers, caused by blocking off secretion from the pars intermedia by a high and massive dorsum sellae. It is generally agreed that a dorsum sellae of this type is not always present. The latest studies of Wislocki and King3disproved the theory of pituitary drainage into the hypothalamus. Ornsteen4explained the syndrome on the basis of a developmental defect ofKeywords
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