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May 1938

THE LAURENCE-BIEDL SYNDROME OCCURRING IN A BROTHER AND SISTER

Author Affiliations

Norristown, Pa.; Philadelphia

From the Laboratory of Pathology, the Norristown State Hospital; the Department of Neurology, the Jefferson Medical College Hospital and the Laboratory of Neuropathology, the Philadelphia General Hospital.

Arch NeurPsych. 1938;39(5):1033-1042. doi: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.

Biedl1 suggested that the syndrome is a familial form of dystrophia adiposogenitalis of cerebral rather than pituitary origin. This is generally accepted. Raab2 expressed 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 King3 disproved the theory of pituitary drainage into the hypothalamus. Ornsteen4 explained the syndrome on the basis of a developmental defect of

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