[A multidisciplinary study of infantile Steinert's syndrome].
The authors studied from several points of view 10 patients suffering from a neo-natal form of Steinert's disease. They analyse the variety of the family histories, of dysembryoplastic symptoms, of neo-natal difficulties and the subsequent impairment of mental development. They could not find any correlation between this latter evolution and the severity of the neurological damage, judged by the mentioned symptoms. They conclude that the mental retardation is proportional to the low quality of the early socio-emotional environment and believe that this hypothesis is confirmed by the fact that maxillo-facial malformations are mocise speech and mimicry in their early childhood.
D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008607:Intellectual Disability; D008297:Male; D008445:Maxillofacial Development; D009223:Myotonic Dystrophy / Q000235:genetics*; D010375:Pedigree; D010641:Phenotype; D012931:Social Environment
Etude pluridisciplinaire dans la maladie de Steinert infantile