Cognitive functioning of adults with Noonan syndrome: A case-control study
Number of pages
SourceGenes, Brain and Behavior, 11, 7, (2012), pp. 785-793
Article / Letter to editor
Display more detailsDisplay less details
SW OZ DCC NRP
SW OZ BSI BO
Genes, Brain and Behavior
SubjectDI-BCB_DCC_Theme 3: Plasticity and Memory; NCEBP 8 - Psychological determinants of chronic illness DCN PAC - Perception action and control; NCEBP 8: Psychological determinants of chronic illness; Neuropsychology and rehabilitation psychology; NCEBP 8: Psychological determinants of chronic illness; Neuro- en revalidatiepsychologie
Noonan syndrome (NS) is a genetic disorder characterised by short stature, facial dysmorphia, congenital heart defects and mildly lowered intellectual abilities. Research has mainly focused on genetic and somatic aspects, while intellectual and cognitive functioning has been documented scarcely. Also, to date studies have been primarily performed in children. This is the first study in which functioning within the major cognitive domains is systematically evaluated in a group of adults with NS and compared with a control group. Extensive neuropsychological assessment, including the domains intelligence, speed of information processing, memory (working memory, immediate recall and delayed recall), executive function and visuoconstruction, was performed in a sample of 42 patients with NS and 42 healthy controls, matched on age, sex and education level. In addition, subjective cognitive complaints were assessed with self-report questionnaires. On the domain speed of information processing patients performed worse than controls (P < 0.05). Furthermore, except for slightly better results on delayed recall in the patients with NS (P < 0.05), none of the other cognitive domains showed between-group differences. On the questionnaires, patients reported substantially more complaints about their own cognitive abilities than controls (P?<?0.05). A lowered speed of information processing and relatively intact functioning in other cognitive domains characterises the cognitive profile of adult patients, in contrast to previous findings in children with NS, who seem to have more generalised cognitive deficits.
Upload full text
Use your RU credentials (u/z-number and password) tolog in with SURFconextto upload a file for processing by the repository team.