TY - JOUR
T1 - Behavioral phenotype in adults with Prader-Willi syndrome
AU - Sinnema, Margje
AU - Einfeld, Stewart L.
AU - Schrander-Stumpel, Constance T. R. M.
AU - Maaskant, Marian A.
AU - Boer, Harm
AU - Curfs, Leopold M. G.
PY - 2011
Y1 - 2011
N2 - 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
AB - 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
KW - Prader-Willi syndrome
KW - Behavior problems
KW - Developmental Behavior Checklist
KW - Genetic subtypes
KW - Ageing
U2 - 10.1016/j.ridd.2010.12.014
DO - 10.1016/j.ridd.2010.12.014
M3 - Article
C2 - 21227640
SN - 0891-4222
VL - 32
SP - 604
EP - 612
JO - Research in Developmental Disabilities
JF - Research in Developmental Disabilities
IS - 2
ER -