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  Prader-Willi Syndrome

What is Prader-Willi Syndrome

Prader-Willi Syndrome (PWS) is a group of symptoms caused by a genetic defect in Chromosome 15. This uncommon condition occurs in about one in every 15,000 births, and was first described by Drs. Prader, Labhart, and Willi in 1956.

Symptoms of Prader-Willi Syndrome include many but not necessarily all of the following:

  • Central Nervous System malfunction includes impaired body control and mental retardation with an average IQ around 70. Dysfunction of the hypothalamus affects physical growth, sexual development, appetite, temperature control, and emotional stability.

  • Hypotonia during infancy with poor motor control, weak cry and poor sucking ability. Although children get stronger as they grow older, muscle tone usually remains lower than normal.

  • Short stature, with adults reaching about five feet. Small hands and feet, narrow forehead.

  • Insatiable appetite begins somewhere between age 2 and 5 years. Since individuals with PWS have metabolism which is only 60% of normal, they require fewer calories to maintain weight. An uncontrolable preoccupation with food usually leads to obesity, serious health problems, and early death unless access to food is strictly controlled. With adequate supervision and careful control of food intake, persons with PWS can maintain healthy weight.

  • Scratching and skin picking due to increased pain tolerance and decreased sensory input.

  • Behavior difficulties beginning in early childhood and persisting throughout adult life, includes temper tantrums, stubbornness, non-compliance, and resistance to transitions. Most persons with PWS show signs of obsessive-compulsive disorder (OCD), apart from their obsessions with food. OCD symptoms in PWS included ordering and arranging, concerns with symmetry, rewriting and a compulsion to tell or ask the same thing over and over.

#Note: Prader-Willi Syndrome occurs when the deletion is in the PATERNAL chromosome 15. When the deletion occurs in the MATERNAL chomosome 15, the result is a totally differnt disability called Angelman’s Syndrome, with a very different set of symptoms.

Foster and Adoptive Care Association of Minnesota
P.O. box 48716
Minneapolis, MN 55448-0716
612-233-3399



Articles have been reprinted from News and Views of Our Families 1992-2004