Behavioral phenotypes in Prader-Willi syndrome

Laura Rosell-Raga¹ and Fernando Mulas²

¹Valencian Community Prader-Willi Association; ² Neuropediatric Service, Children’s University Hospital LA FE. Valencia. Spain;  Valencian Institute Pediatric Neurology (INVANEP) 

The behavioral phenotype of Prader-Willi Syndrome (PWS) is defined by a neurological profile and a characteristic pattern of behavioral disorders that include cognitive deficits, learning difficulties and behavioral problems, which increase with age, both in number and severity, and disturb the socio-familial lifestyle.

We reviewed the behavioral phenotype of the all cases of 35 PWS and their families registered in the Valencian Community, and differentiated them into four age groups:  

6 subjects from birth to 3 years old,
            7 subjects from 4 to 10 years old,
            5 subjects in the adolescent period (11 to 16 years old), and
          13 subjects in the 17 to 28 year old age group 

We will discuss their peculiar behaviors, and analyze how these generate family and social problems.

            The expression of a more problematic behavioral phenotype is relevant in the adolescent age group, when it is often necessary to use pharmacological support.  We recognize and discuss the description of a “peculiar” behavior pattern in earlier stages of life in children with PWS, that can open up news horizons when understanding and treating PWS, both from a pharmacological and neuropsychological perspective.

June 2004