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Prader-Willi Syndrome (updated March 2003)

What information do you have on Prader-Willi Syndrome ?

The following information is from the Prader-Willi Syndrome Association:

Prader-Willi Syndrome (PWS) is an uncommon, noninherited birth defect, lifelong and life-threatening, affecting all races and both sexes. Prevalence is estimated to be 1:12,000 to 15,000. Its characteristics include hypotonia, insatiable appetite, obesity if food intake is uncontrolled, hypogonadism and incomplete sexual development, developmental delays, variable degrees of mental retardation or functional retardation, short stature (adult), small hands and feet, mild dysmorphology, and behavior problems which can be severe.

The cause of this condition is unclear. Approximately 70% have an interstitial deletion or other abnormal finding on chromosome 15, using high resolution (prometaphase) analysis. The remainder have two maternal chromosome 15s and no paternal 15 (maternal uniparental disomy). Diagnosis of infants with PWS is difficult. Newborns are hypotonic, lethargic, exhibit genital hypoplasia, and often require gavage or other special feeding techniques. Prenatally, fetal movement is decreased. Diagnosis in adults is more certain if classic characteristics are present: short stature, small hands and feet, CNS dysfunction, underdeveloped sexual characteristics, poor large muscle strength.

Motor development is delayed typically one to two years, as are most milestones. For example, walking usually occurs around age two. Poor gross motor performance and balance improve slowly and lag behind normal individuals of the same age. Speech and language problems are common. Cause is unclear, possibly poor muscle tone affecting vocal muscles and/or decreased saliva production. Speech therapy is recommended to relieve frustration associated with non-communication. Though language development is delayed, verbal ability is often a strength. Articulation may remain poor. The average IQ is around 70, with a range from 40 to 105. Typically, functioning is below IQ level. Abstract thinking and concepts are weaknesses. Even with a lower IQ, cleverness in food seeking can exist.

Behavior problems, ranging from stubbornness to violent temper tantrums and increasing with age, usually begin to appear during the preschool years when most are pleasant and cooperative. Intervention therapy can be used to modify severity (e.g., fluexitine serotonin uptake inhibitors have been particularly beneficial in some cases). True depression and psychotic episodes are reported.

Compulsive eating and obsession with food begin usually between the ages of 2 - 4, though they may start later. Some learn to eat at fixed times and may refuse certain foods, but the insatiable drive for food persists. Sneaking or stealing extra food is common. In most situations, all sources of food must be kept under lock and key. Avoidance of temptation is helpful. Obesity occurs in 95% if there are no environmental controls. An increasing number are being diagnosed before onset of obesity. Whether obese at diagnosis or not, more and more have their weight managed within acceptable levels. Calorie utilization is decreased and diets should offer fewer calories than unual (often 1,000-1,200 per day). Exercise is critical to weight control.

Sports activities are limited. Running and jumping can cause joint injuries due to poor muscle strength and poor coordination. Incidence of bone fractures is increased, probably related to osteoporosis and decreased muscle bulk. Adaptive physical exercise is necessary in school. Walking, swimming, and stationary exercise equipment are recommended. Medications, e.g., anorexic agents or psychoactive drugs, in most cases have not been found generally useful to control appetite. Drug treatment is essential at times but is not recommended for continuing usage. Consult the national PWSA office for updates. For some medications, increased sensitivity to normal doses is found.

Adolescents and adults can function well in group living programs if they have adequate calorie control and structured living. Their rigid personalities and explosive tempers make supervision and programming difficult. Sheltered workshops generally provide the best employment possibilities.

Stress on families is often extreme. The constant pressure of food control and behavior management affects all family members, e.g., higher divorce rates and siblings with emotional problems. The child with PWS can become a tyrant who rules the family and, with age, becomes more difficult to handle. Awareness of these stresses and dealing with them early on is essential to the family structure.

Following are links to related Internet resources and Internet discussion groups, as well as selected citations from the ERIC database and the search terms we used to find the citations.

You can search the ERIC database yourself on the Internet through either of the following web sites:

NOTE: The primary focus of the ERIC system is education. While the ERIC database contains information on some medical disorders, you may want to search a medical database such as MEDLINE/MEDLARS at the National Library of Medicine, http://www.nlm.nih.gov, or PubMed http://www.ncbi.nlm.nih.gov/PubMed/ or MedWeb at Emory University, http://WWW.MedWeb.Emory.Edu/MedWeb/.

ERIC Citations

The full text of citations beginning with an ED number (for example, EDxxxxxx) is available:

  • In microfiche collections worldwide; to find your nearest ERIC Resource Collection, point your web browser to: http://ericae.net/derc.htm.
  • For a fee through the ERIC Document Reproduction Service (EDRS): http://edrs.com, service@edrs.com, or 1.800.443.ERIC. (no longer available)

The full text of citations beginning with an EJ number (for example, EJxxxxxx) is available for a fee from:

ERIC Search Terms Used

Prader-Willi syndrome

EJ593040 EC623098
Prader-Willi Syndrome: A Review and Implications for Educational Intervention.
Scott, Ellen M.; Smith, Tom E. C.; Hendricks, Mary D.; Polloway, Edward A.
Education and Training in Mental Retardation and Developmental Disabilities; v34 n1 p110-16 Mar 1999
ISSN: ISSN-1079-3917
Language: English
Journal Announcement: CIJAPR2000
This review of Prader-Willi Syndrome notes characteristics (mental retardation and excessive overeating). Educational interventions including weight management, cognitive and educational development, behavioral interventions, and transition to adulthood are discussed.
Descriptors: Behavior Change; *Child Development; Cognitive Development; Congenital Impairments; Educational Needs; *Intervention; *Mental Retardation; Obesity; Special Health Problems; Transitional Programs;
Identifiers: *Prader Willi Syndrome

EJ619723 EC626522
Cognitive Strengths and Weaknesses Associated with Prader-Willi Syndrome.
Conners, Frances A.; Rosenquist, Celia J.; Atwell, Julie A.; Klinger, Laura Grofer
Education and Training in Mental Retardation and Developmental Disabilities; v35 n4 p441-48 Dec 2000
ISSN: ISSN-1079-3917
Language: English
Document Type: JOURAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJJUL2001
Nine adults with Prader-Willi syndrome (PWS) and nine age- and IQ-matched adults with PWS completed standardized tests of long-term and short-term memory, visual and auditory processing, and reading and mathematics achievement. Contrary to previous findings, long-term memory in PWS subjects was strong relative to IQ and there was no evidence that visual processing was superior to auditory processing in PWS individuals.
Descriptors: Adults; Auditory Perception; Congenital Impairments; Intelligence Quotient; Long Term Memory; Mathematics Achievement; *Mental Retardation; Reading Achievement; Short Term Memory; Visual Perception;
Identifiers: *Prader Willi Syndrome

EJ629446 EC627782
Excessive Picking in Prader-Willi Syndrome: A Pilot Study of Phenomenological Aspects and Comorbid Symptoms. Wigren, Margareta; Heimann, Mikael
International Journal of Disability, Development and Education; v48 n2 p129-42 Jun 2001
ISSN: ISSN-0156-6555
Language: English
Document Type: JOURAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJJAN2002
Interviews with parents of 37 individuals (ages 12-30) with Prader-Willi syndrome revealed two-thirds displayed skin picking with a frequency ranging from chronic to transient, episodic symptoms. Many individuals with skin picking also exhibited comorbid picking behaviors And individuals with excessive skin picking also had frequent tantrums and violent outbursts.
Descriptors: Adults; *Behavior Patterns; *Behavior Problems; Children; *Individual Characteristics; *Mental Retardation; Multiple Disabilities; Self Injurious Behavior; *Symptoms (Individual Disorders);
Identifiers: *Prader Willi Syndrome

EJ623103 EC626826
Treatment of Covert Food Stealing in an Individual with Prader-Willi Syndrome.
Maglieri, Kristen, A.; DeLeon, Iser G.; Rodriguez-Catter, Vanessa; Sevin, Bart M.
Journal of Applied Behavior Analysis; v33 n4 p615-18 Win 2000
ISSN: ISSN-0021-8855
Language: English
Document Type: JOURAL ARTICLES (080); REPORTS (143)
Journal Announcement: CIJSEP2001
A study found verbal reprimands, delivered contingent upon eating prohibited foods, were sufficient to decease the food stealing of a girl (age 14) with Prader-Willi syndrome. Warning stimuli were then successfully used to help her discriminate between permitted/prohibited foods during sessions in which food stealing was not directly observed.
Descriptors: Adolescents; *Behavior Modification; *Eating Disorders; Females; *Mental Retardation; *Negative Reinforcement; *Obesity; Program Effectiveness; *Self Control; Verbal Operant Conditioning;
Identifiers: *Prader Willi Syndrome

EJ491034 EC609564
Prader-Willi Syndrome: Quality of Life Issues in Home, School and Community.
James, Terrance N.; Brown, Roy I.
Australia and New Zealand Journal of Developmental Disabilities, v18 n4 p253- 60 1993
Special Congress Issue II.
ISSN: 0726-3864
Language: English
Journal Announcement: CIJFEB95
Management of psychosocial aspects of Prader-Willi syndrome is considered for several quality of life issues, including social change, increased life expectancy, parent stress, child stress, independence, residential options, and access to services.
Descriptors: *Developmental Disabilities; Normalization (Disabilities); *Quality of Life; Stress Management
Identifiers: *Prader-Willi Syndrome

EJ465483 EC606254
Beneficial Effects of Exercise on Aerobic Capacity and Body Composition in Adults with Prader-Willi Syndrome.
Silverthorn, Kathryn H.; Hornak, James E.
American Journal on Mental Retardation, v97 n6 p654-58 May 1993
ISSN: 0895-8017
Language: English
Journal Announcement: CIJNOV93
Six adults with Prader-Willi syndrome who participated in a six-month walking program showed significant differences in resting heart rate, aerobic capacity, body fat percentage, and weight loss, compared to a control group of five nonparticipants.
Descriptors: Adults; *Aerobics; *Body Composition; Body Weight; Congenital Impairments; *Exercise; Heart Rate; *Mental Retardation; *Obesity; Outcomes of Treatment; Physical Fitness
Identifiers: *Prader-Willi Syndrome

EJ450128 EC604048
Prader-Willi Syndrome: Causes, Characteristics, Interventions, Long-Term Consequences.
Otto, Tracy L.; Barber, William H.
B.C. Journal of Special Education, v16 n1 p38-50 1992
ISSN: 0704-7509
Language: English
Journal Announcement: CIJJAN93
An overview of Prader-Willi syndrome, the most common form of dysmorphic genetic obesity associated with mental retardation, is presented, with an emphasis on associated causes, characteristics, diagnosis and counseling, intervention, and long-term consequences.
Descriptors: Clinical Diagnosis; *Congenital Impairments; Etiology; *Genetics; Intervention; *Mental Retardation; Obesity; Special Health Problems; *Symptoms (Individual Disorders)
Identifiers: *Prader-Willi Syndrome

EJ411819 EC231004
The Speech and Language Characteristics of Children with Prader-Willi Syndrome.
Kleppe, Sallie A.; And Others
Journal of Speech and Hearing Disorders, v55 n2 p300-09 May 1990
Language: English
Journal Announcement: CIJDEC90
Target Audience: Researchers
The study investigated the communicative development of 18 children (ages 8- 17) with Prader-Willi syndrome. A variety of communicative deficiencies were found in the children's speech, language, voice, and fluency. Descriptors: Adolescents; Children; *Communication Skills; *Congenital Impairments; *Special Health Problems; *Speech Handicaps
Identifiers: *Prader-Willi Syndrome

EJ359477 EC200355
Some Considerations in the Education and Management of the Child with Prader-Willi Syndrome in the Special Education Classroom.
Lupi, Marsha H.; Porcella, John E.
Techniques, v3 n3 p230-35 Jul 1987
Language: English
Document Type: JOURNAL ARTICLE (080); DISSERTATION (041)
Journal Announcement: CIJJAN88
Target Audience: Practitioners
The paper summarizes characteristics of the child with Prader-Willi Syndrome (characterized by insatiable appetite and usually mild to moderate mental retardation). Suggestions are offered for the environmental and behavioral management of the Prader-Willi student.
Descriptors: Behavior Change; Body Weight; *Eating Habits; Elementary Secondary Education; *Mental Retardation; *Special Health Problems
Identifiers: *Prader-Willi Syndrome

EJ343953 EC190883
Description of an Innovative Intervention Program for Individuals with Prader-Willi Syndrome.
Caldwell, Mary Lou; Taylor, Ronald L.
B. C. Journal of Special Education, v10 n2 p183-87 1986
Language: English
Journal Announcement: CIJMAR87
A successful five-week weight reduction program for individuals with Prader-Willi Syndrome is described. The program combines diet and exercise, and participants are taught to make menu choices within the framework of daily food exchange allotments.
Descriptors: Daily Living Skills; *Dietetics; Eating Habits; Exercise; *Obesity; *Special Health Problems
Identifiers: *Prader-Willi Syndrome

ED349770 EC301516
Issues in Nutrition for Adolescents with Chronic Illnesses and Disabilities.
CYDLINE Reviews.
University of Minnesota, Minneapolis, National Center for Youth with Disabilities; Society for Adolescent Medicine, Independence, MO. Aug 1992
42p.; Sponsoring Agency: Health Resources and Services Administration (DHHS/PHS), Rockville, MD. Office for Maternal and Child Health Services.
EDRS Price - MF01/PC02 Plus Postage.
Language: English
Document Type: BIBLIOGRAPHY (131)
Geographic Source: U.S.; Missouri
Journal Announcement: RIEFEB93
This annotated bibliography lists print materials, training and educational materials, and programs concerned with nutrition for youth with chronic illnesses and disabilities. Basic bibliographic information and a brief abstract are provided for each of the 87 bibliographic citations which date from 1980 through 1991. Citations are organized into the following categories: adolescent nutrition; nutrition and disability; mental retardation/developmental disabilities; chronic illnesses (arthritis, asthma, cerebral palsy, cystic fibrosis, diabetes, digestive system diseases, phenylketonuria, and Prader-Willi syndrome); and learning disabilities. Listings for the 16 training materials also include a brief abstract and source information. Materials include booklets, handouts, videotape recordings, and curriculum guides. Finally, abstracts and contact information is given for three nutrition programs.
Descriptors: Adolescent Development; Asthma; Cerebral Palsy; Child Development; *Child Health; *Chronic Illness; Developmental Disabilities; Diabetes; Dietetics; *Disabilities; Instructional Materials; Mental Retardation; *Nutrition; Nutrition Instruction; *Public Health; Resources; *Special Health Problems
Identifiers: Arthritis; Cystic Fibrosis; Digestive System Diseases; Phenylketonuria; Prader-Willi Syndrome

ED252006 EC171162
Prader-Willi Disease: A Case Study.
Forbus, William R., III
1983; 80p.; Master's Thesis, University of Georgia.
EDRS Price - MF01/PC04 Plus Postage.
Language: English
Document Type: THESIS (042); PROJECT DESCRIPTION (141)
Geographic Source: U.S.; Georgia
Journal Announcement: RIEMAY85
A case study focuses on the characteristics and physical management of a 15- year-old with Prader-Willi Syndrome, a birth defect associated with hypotonia, insatiable appetite, hypogonadism, central nervous system dysfunction, and abnormal growth and development. A literature review addresses studies dealing with behavior modification of obesity and continuous eating, nutrition counseling, and physical activity intervention. Interventions in the case study included those programmed by trained personnel in the following service areas: adapted physical education, behavior modification, nutrition, nursing, dormitory living, social work, education, and speech and language. Data are reported for behavior, weight, and adjustment to physical activity. Analysis of data from the S's developmental and medical histories, continuous evaluations, previous staffing summaries, motor proficiency tests, exercise metabolism tests, progress reports and staff and parent survey forms revealed that the S made considerable progress in reaching her program goals. She was found to demonstrate most of the characteristics classic to the Prader-Willi population. Recommendations were made for programming.
Descriptors: *Adapted Physical Education; *Behavior Modification; *Congenital Impairments; Multiple Disabilities; *Nutrition; *Obesity
Identifiers: *Prader-Willi Syndrome

Physical Education for Children with Prader-Willi Syndrome.
Weber, Robert C.
Palaestra; v9 n3 p41-47 Spr 1993
Target Audience: Teachers
This article addresses physical education for children with Prader-Willi syndrome including etiology, characteristics (e.g., mental retardation, obesity, poor muscle tone), teaching considerations and techniques (including dealing with hypotonisity, obesity, mental retardation, visual problems, and behavior modification), and suggested activities.
Descriptors: Mental Retardation; Special Health Problems; Congenital Impairments; Adapted Physical Education; Characteristics; Etiology; Teaching Methods; Physical Activities; Movement Education
Identifiers: Prader-Willi Syndrome

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