The neuropsychological basis of hypersociability in Williams and Down syndrome

• Published in: Neuropsychologia Vol. 45; no. 12; pp. 2839 - 2849
• Main Authors: Porter, Melanie A., Coltheart, Max, Langdon, Robyn
• Format: Journal Article
• Language: English
• Published: Oxford Elsevier Ltd 01.01.2007; Elsevier Science
• Subjects: Adult and adolescent clinical studies; Amygdala - physiology; Biological and medical sciences; Child; Child, Preschool; Chromosome aberrations; Cognition - physiology; Down Syndrome - physiopathology; Down Syndrome - psychology; Emotion; Executive function; Female; Frontal Lobe - physiology; Humans; Inhibition (Psychology); Male; Medical genetics; Medical sciences; Neuropsychological Tests; Organic mental disorders. Neuropsychology; Psychology. Psychoanalysis. Psychiatry; Psychomotor Performance - physiology; Psychopathology. Psychiatry; Social Behavior; Social disinhibition; Williams Syndrome - physiopathology; Williams Syndrome - psychology; Executive function; Social disinhibition; Emotion; Chromosomal aberration; Human; Affect affectivity; Trisomy; Aneuploidy; Cognition; Emotion emotionality; Down syndrome; Williams syndrome; Social behavior; Social interaction
• ISSN: 0028-3932; 1873-3514
• DOI: 10.1016/j.neuropsychologia.2007.05.006

People with Williams syndrome (WS) display indiscriminate approach toward strangers in everyday life. People with Down syndrome (DS) can also do so, but to a lesser degree. Inappropriate approach behavior is also characteristic of people with acquired amygdala damage and people with acquired frontal lobe impairment; given this, the developmental disorder of social approach seen in WS and perhaps also DS might be due to poor emotion recognition (due to abnormal amygdala functioning) or poor control of behavior (due to frontal lobe abnormality). A third account of this developmental disorder of social cognition can be couched in terms of heightened salience for social stimuli. We explored these three hypotheses by testing emotion recognition, social approach and frontal lobe functioning in people with WS and DS. Overall, our results were inconsistent with predictions from the amygdala and social salience hypotheses. In contrast, results from a battery of neuropsychological tasks suggested that abnormal social approach in WS and DS in everyday life is best explained by frontal lobe impairment, in particular, poor response inhibition.