[Marinesco-Sjögren syndrome]

The case of a severely mentally-retarded man who congenitally presents a cerebellar syndrome and a serious myopia, is discussed: his unilateral cataract showed at 25, and two presenile cataracts among his relatives. Besides, this case involve an epilepsy, an insufficient somato-genital growth and an hyperostosis frontalis interna. A dyslipoïdosis was called up by d'ANGELO and al. (1967) in the MARINESCO-SJOGREN syndrome; an excess of lipids seems actual in the CSF of our case. So the rare MARINESCO-SJOGREN syndrome's diagnosis criteria may remain no restrictive.