Prader-Willi Syndrome

Prader-Willi syndrome (PWS) is a rare neurobehavioral genetic disorder that affects numerous organ systems. It occurs in approximately 1 in 10,000 to 30,000 births and affects all races and genders. PWS impacts the functioning of the hypothalamus, a regulation center located in the brain, resulting in decreased growth hormone, altered reproductive [Page 395]hormones (e.g., hypogonadism), disruption in appetite control, and altered regulation of the autonomic nervous system (the nervous system associated with involuntary reactions). In infancy, PWS is associated with a poor sucking reflex and weak muscle tone. In later childhood, it is associated with:

• Excessive food seeking (with subsequent morbid obesity)
• Delayed development
• Mental retardation or learning disability (Intelligence test scores can range from 20 to 115; an average intelligence score for an individual with no disabilities is 100.)
• Characteristic physical features (e.g., short stature, small hands, small feet, almond-shaped eyes, small mouth with thin upper lip and downturned corners, fair skin and hair)

Children and adolescents with PWS often have rigid thought processes, perseverative or obsessive thinking, and tenuous emotional control. A regular education classroom setting is preferred, although the child's developmental or emotional status may require a more restricted classroom environment. Teachers should use positive reinforcement for appropriate behavior and avoid emotional overstimulation. The classroom environment should be structured with clear, consistent rules provided. Holistic and spatial teaching approaches, such as showing the end product then breaking down the steps or using manipulatives, can be effective. A multimodal treatment approach is recommended with a combination of behavioral, emotional, linguistic, physical, and pharmacological interventions.