- depressed general cognitive functioning or IQ;
- processing deficits including short term memory;
- language processing deficits; and
- difficulty with higher order processing.
- visual perception,
- organization, and
- puzzle-solving skills.
- need for sameness,
- obsessive-compulsive behavior, and/or
- autistic spectrum disorder.
Recent evidence of clinical and behavioral differences in PWS subjects with the type I (larger) and type II (smaller) 15q11-q13 deletion subtypes in PWS have been reported and also between those with the typical 15q11-q13 deletion and maternal disomy 15 (both 15s from the mother). [Butler: 2004] [Bittel: 2006]
Genetics in Primary Care Institute (AAP)
The goal of this site is to increase collaboration in the care of children with known or suspected genetic disorders. It includes health supervision guidelines and other useful resources; represents a collaboration among the Health Resources & Services Administration, the Maternal and Child Health Bureau, and the American Academy of Pediatrics.
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|Author:||Merlin G. Butler, MD, PhD - 9/2008|
|Reviewing Author:||Kyna Byerly, MS, CGC - 8/2008|
|Content Last Updated:||2/2015|
Bittel DC, Kibiryeva N, Butler MG.
Expression of 4 genes between chromosome 15 breakpoints 1 and 2 and behavioral outcomes in Prader-Willi syndrome.
Pediatrics. 2006;118(4):e1276-83. PubMed abstract
Butler MG, Bittel DC, Kibiryeva N, Talebizadeh Z, Thompson T.
Behavioral differences among subjects with Prader-Willi syndrome and type I or type II deletion and maternal disomy.
Pediatrics. 2004;113(3 Pt 1):565-73. PubMed abstract / Full Text
Butler MG, Lee PDK, Whitman, BY.
Management of Prader-Willi Syndrome.
3rd ed. New York, NY: Springer Verlag Inc.; 2006. 0387253971
Textbook with diagnosis and management information for PWS. Includes clinical, genetic, social, family, and community issues.
Dykens EM, Roof E.
Behavior in Prader-Willi syndrome: relationship to genetic subtypes and age.
J Child Psychol Psychiatry. 2008;49(9):1001-8. PubMed abstract
Holsen LM, Zarcone JR, Brooks WM, Butler MG, Thompson TI, Ahluwalia JS, Nollen NL, Savage CR.
Neural mechanisms underlying hyperphagia in Prader-Willi syndrome.
Obesity (Silver Spring). 2006;14(6):1028-37. PubMed abstract / Full Text
Jauregi J, Arias C, Vegas O, Alén F, Martinez S, Copet P, Thuilleaux D.
A neuropsychological assessment of frontal cognitive functions in Prader-Willi syndrome.
J Intellect Disabil Res. 2007;51(Pt 5):350-65. PubMed abstract
Reddy LA, Pfeiffer SI.
Behavioral and emotional symptoms of children and adolescents with Prader-Willi Syndrome.
J Autism Dev Disord. 2007;37(5):830-9. PubMed abstract
Shapira NA, Lessig MC, He AG, James GA, Driscoll DJ, Liu Y.
Satiety dysfunction in Prader-Willi syndrome demonstrated by fMRI.
J Neurol Neurosurg Psychiatry. 2005;76(2):260-2. PubMed abstract / Full Text
Torrado M, Araoz V, Baialardo E, Abraldes K, Mazza C, Krochik G, Ozuna B, Leske V, Caino S, Fano V, Chertkoff L.
Clinical-etiologic correlation in children with Prader-Willi syndrome (PWS): an interdisciplinary study.
Am J Med Genet A. 2007;143(5):460-8. PubMed abstract
Yamada K, Matsuzawa H, Uchiyama M, Kwee IL, Nakada T.
Brain developmental abnormalities in Prader-Willi syndrome detected by diffusion tensor imaging.
Pediatrics. 2006;118(2):e442-8. PubMed abstract
Zarcone J, Napolitano D, Peterson C, Breidbord J, Ferraioli S, Caruso-Anderson M, Holsen L, Butler MG, Thompson T.
The relationship between compulsive behaviour and academic achievement across the three genetic subtypes of Prader-Willi syndrome.
J Intellect Disabil Res. 2007;51(Pt. 6):478-87. PubMed abstract