Syndrome de Laurence - Biedl: Deux Cas Chez Deux Freres

Abstract

2 cases of Laurence- Moon-Biedl syndrome are described in 2 rothers. They have 5 out of 6 cardinal symptoms of this syndrome i.e. : I) Obesity ,2) genital dystrophia, 3) retinitis pigmentosa, 4)menal deficiency, 5) familial occurrence. Besides, their only sister has ocular signs of beginning of retinitis igmentosa and one of their relatives had polydactylism and another ne cretinism. What is curious in these case is the coexistence of rotatory stagmus in the eyes and a peculiar dark discoloration of the axillar, guinal and pubic regions ressembling acanthosis nigricans. It might be another proof of the syndrome believed by Some rvers to be the region of hypophyse and hypothalamus.