Behavioral Phenotype in Adults with Prader-Willi Syndrome

Autor/inn/en	Sinnema, Margje; Einfeld, Stewart L.; Schrander-Stumpel, Constance T. R. M.; Maaskant, Marian A.; Boer, Harm; Curfs, Leopold M. G.
Titel	Behavioral Phenotype in Adults with Prader-Willi Syndrome
Quelle	In: Research in Developmental Disabilities: A Multidisciplinary Journal, 32 (2011) 2, S.604-612 (9 Seiten)Infoseite zur Zeitschrift PDF als Volltext Verfügbarkeit
Sprache	englisch
Dokumenttyp	gedruckt; online; Zeitschriftenaufsatz
ISSN	0891-4222
DOI	10.1016/j.ridd.2010.12.014
Schlagwörter	Check Lists; Behavior Problems; Disability Identification; Genetics; Genetic Disorders; Symptoms (Individual Disorders); Aggression; Correlation; Age Differences; Body Composition; Adults; Foreign Countries; Classification; Netherlands; Developmental Behavior Checklist + Suchen Sie Ihr Suchwort? Checkliste; Humangenetik; Psychiatrische Symptomatik; Korrelation; Age; Difference; Age difference; Altersunterschied; Ausland; Classification system; Klassifikation; Klassifikationssystem; Niederlande
Abstract	Prader-Willi syndrome (PWS) is characterized by temper tantrums, impulsivity, mood fluctuations, difficulty with change in routine, skinpicking, stubbornness and aggression. Many studies on behavior in PWS are limited by sample size, age range, a lack of genetically confirmed diagnosis of PWS and inconsistent assessment of behavior. The aim of this study was to explore systematically the relation between behavioral problems and age groups, genetic subtypes and BMI categories in an adult PWS population. Participants were contacted via the Dutch Prader-Willi Parent Association and through physicians specialized in persons with ID. Behaviors were studied using the Developmental Behavior Checklist for Adults (DBC-A). The forms were completed by the main caregivers of 98 adults with a genetically confirmed diagnosis of PWS. Differences between age groups were statistically significant (ANOVA, p = 0.03). DBC-A total scores were higher in the consecutive age groups, with the most behavioral problems in the oldest age groups. Differences between genetic subtypes were also statistically significant (ANOVA, p less than 0.01). Persons with mUPD had higher total scores on the DBC-A than persons with a deletion. Those with a Type I deletion showed higher total DBC-A scores than persons with a Type II deletion. There were no statistically significant differences in DBC-A total scores between the different BMI categories. Individuals with a BMI less than 25 had higher scores on the self-absorbed subscale compared to persons with a BMI between 25 and 30. Unlike previous descriptions of the behavioral phenotype in adults with PWS, we did not find a reduction in behavioral problems in older adults. Therefore, special attention should be paid to behavioral problems as part of general management of adults with PWS. Longitudinal studies are warranted to gain more insight into the natural history and course of behavioral problems in adults and older people with PWS over the long term and possible risk and preventive factors. (Contains 4 tables.) (As Provided).
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Erfasst von	ERIC (Education Resources Information Center), Washington, DC
Update	2017/4/10