Abstract:
© 2018 International Strategic Management Association. All rights reserved. Aim: The article is devoted to the specificity of cognitive development in three children with Prader–Willi syndrome (PWS) in the period up to 6-year-old. The PWS is a rare hereditary disease caused by the absence of the father's copy of the 15q11-13 chromosome. Methods: Genomic imprinting is involved into the regulation of the genes in this area of chromosome 15. Diagnostic signs of this syndrome are muscular hypotension, hypogonadism, obesity, excessive adiposity, respiratory complications, mental retardation, small brushes and feet, dysplasia of the hip joints, and stigma of disembryogenesis. Currently, specific ways of treating people with this syndrome have not been developed. It is considered that children with PWS suffer from the retardation of cognitive development; however, there is a lack of scientific information about it. The study of PWS requires an interdisciplinary approach and the detailed description of cognitive development. Results: The results showed that in the period up to 6 years in three children with this rare syndrome the most obvious decline is registered predominantly in the development of impressive speech, and other functions do not suffer significantly in spite of the presence of impairments in motor development. Conclusion: The research was conducted at A. Yu. Ratner Pediatic Сlinical Hospital NO. 8 (Kazan, Russia) by the group of clinical linguists, neurologists, and speech therapists.