Prader Willie Syndrome

Prader-Willie syndrome (PWS) occurs evenly across both gender and ethnic lines. Approximately 1 individual in 10,000-15,000 births is impacted. PWS is caused by a microdeletion of chromosomal matter on chromosome 15. One unique feature of PWS is that genes are expressed in a divergent manner depending on whether one inherited them from one's mother or one's father. Physical manifestations include failure to thrive (early in development), characteristic facial features (e.g., narrow nasal bridge, downturnedmouth, almond-shaped eyes, and thin upper lip), hypotonia, hypogonadism, later insatiable appetite and resulting obesity, and a specific behavioral phenotype.

The behavioral phenotype includes impulsivity, aggression, obsessive behaviors, underactivity, anxiety, daytime sleepiness, and perseverative tendencies. In addition, other maladaptive behaviors and psycho-pathology are present in persons with PWS, including tantrums, mood changes, argumentativeness, stubbornness, oppositional behaviors, theft, and rigidity. Clearly, managing obsessive food-seeking behaviors that occur in clever and manipulative manners poses a substantial challenge for those working with and caring for these individuals. Most persons with PWS are functioning within the mild range of mental retardation, with an average IQ score estimated to be approximately 70. Individual variation has been noted, with scatter from the average level to the profound range of mental retardation. In general, cognitive abilities seem to plateau in adolescence or early adulthood. Adaptive behavior scores are generally reported as being lower than scores for their level of cognitive functioning, with steady gains in socialization and daily livings skills as these individuals grow and develop.



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