Biomaterial Database

Personality profiles of youngsters with Prader-Willi syndrome and youngsters attending regular schools. 1995

L M Curfs, and V Hoondert, and C F van Lieshout, and J P Fryns

Clinical Genetics Center, Maastricht, The Netherlands.

For the study of the personality profile of youngsters with Prader-Willi syndrome (PWS), a PWS group was compared with a matched group of youngsters attending regular school. The PWS group consisted of 28 youngsters (12 males and 16 females; mean age 11 years, 11 months). These youngsters were matched on gender and age level with 28 youngsters out of a group of 333 youngsters in regular school. Behaviour and personality characteristics of each youngster in both groups were described by both parents, using a Dutch version of the California Child Q-set (CCQ; Block & Block 1980). The present authors compared the scores of both groups on eight personality dimensions, derived from the aggregated CCQ-descriptions for fathers and mothers. The personality dimensions were Extraversion, Agreeableness, Conscientiousness, Emotional Stability, Openness, Motor Activity, Irritability and Dependency. The authors investigated further individual differences of PWS youngsters on the same eight CCQ personality dimensions, considering gender and age level as well as IQ level and the presence or absence of a 15q11-q13 deletion. The personality profile of PWS youngsters was markedly different from youngsters in regular school. Some personality characteristics were related to gender and IQ. The lower level of physical activity in PWS girls without 15q11-q13 deletion needs further study.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D010554	Personality Disorders	A major deviation from normal patterns of behavior.	Avoidant Personality Disorder,Impulse-Ridden Personality,Inadequate Personality,Avoidant Personality Disorders,Impulse Ridden Personality,Personality Disorder,Personality Disorder, Avoidant,Personality Disorders, Avoidant,Personality, Impulse-Ridden,Personality, Inadequate
D011218	Prader-Willi Syndrome	An autosomal dominant disorder caused by deletion of the proximal long arm of the paternal chromosome 15 (15q11-q13) or by inheritance of both of the pair of chromosomes 15 from the mother (UNIPARENTAL DISOMY) which are imprinted (GENETIC IMPRINTING) and hence silenced. Clinical manifestations include MENTAL RETARDATION; MUSCULAR HYPOTONIA; HYPERPHAGIA; OBESITY; short stature; HYPOGONADISM; STRABISMUS; and HYPERSOMNOLENCE. (Menkes, Textbook of Child Neurology, 5th ed, p229)	Labhart-Willi Syndrome,Royer Syndrome,Labhart-Willi-Prader-Fanconi Syndrome,Prader Labhart Willi Syndrome,Prader-Labhart-Willi Syndrome,Royer's Syndrome,Willi-Prader Syndrome,Labhart Willi Prader Fanconi Syndrome,Labhart Willi Syndrome,Prader Willi Syndrome,Royers Syndrome,Syndrome, Labhart-Willi,Syndrome, Labhart-Willi-Prader-Fanconi,Syndrome, Prader-Labhart-Willi,Syndrome, Prader-Willi,Syndrome, Royer,Syndrome, Royer's,Syndrome, Willi-Prader,Willi Prader Syndrome
D011596	Psychomotor Disorders	Abnormalities of motor function that are associated with organic and non-organic cognitive disorders.	Psychomotor Impairment,Developmental Psychomotor Disorders,Psychomotor Disorders, Developmental,Developmental Psychomotor Disorder,Impairment, Psychomotor,Impairments, Psychomotor,Psychomotor Disorder, Developmental,Psychomotor Impairments
D011597	Psychomotor Performance	The coordination of a sensory or ideational (cognitive) process and a motor activity.	Perceptual Motor Performance,Sensory Motor Performance,Visual Motor Coordination,Coordination, Visual Motor,Coordinations, Visual Motor,Motor Coordination, Visual,Motor Coordinations, Visual,Motor Performance, Perceptual,Motor Performance, Sensory,Motor Performances, Perceptual,Motor Performances, Sensory,Perceptual Motor Performances,Performance, Perceptual Motor,Performance, Psychomotor,Performance, Sensory Motor,Performances, Perceptual Motor,Performances, Psychomotor,Performances, Sensory Motor,Psychomotor Performances,Sensory Motor Performances,Visual Motor Coordinations
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D004644	Emotions	Those affective states which can be experienced and have arousing and motivational properties.	Feelings,Regret,Emotion,Feeling,Regrets
D005120	Extraversion, Psychological	A state in which attention is largely directed outward from the self.	Extraversion (Psychology),Extraversion, Psychology,Extroversion, Psychological,Extroversion, Psychology,Extraversion,Extroversion,Extroversion (Psychology),Psychological Extraversion,Psychological Extroversion,Psychology Extraversion,Psychology Extraversions,Psychology Extroversion
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man