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Cognitive functioning of adults with Noonan syndrome: a case-control study
E Wingbermühle1, R L Roelofs, I van der Burgt
1Vincent van Gogh Institute for Psychiatry, Venray, The Netherlands.pwingbermuhle@vvgi.nl
Genes, Brain, and Behavior
|July 13, 2012
Summary
Adults with Noonan syndrome (NS) show slower information processing speeds but maintain other cognitive functions. Patients reported more subjective cognitive complaints than controls, unlike findings in children with NS.
Area of Science:
- Neuroscience
- Genetics
- Psychology
Background:
- Noonan syndrome (NS) is a genetic disorder with known physical and intellectual characteristics.
- Cognitive functioning in NS has been primarily studied in children, with limited research on adults.
- Existing research on NS cognition has focused less on specific cognitive domains.
Purpose of the Study:
- To systematically evaluate cognitive functioning in adults with Noonan syndrome.
- To compare the cognitive performance of adults with NS to a matched healthy control group.
- To investigate subjective cognitive complaints in adults with NS.
Main Methods:
- Neuropsychological assessment of 42 adults with NS and 42 controls, matched for age, sex, and education.
- Evaluation of cognitive domains including intelligence, processing speed, memory, executive function, and visuoconstruction.
- Self-report questionnaires to assess subjective cognitive complaints.
Main Results:
- Adults with NS exhibited significantly slower information processing speeds compared to controls.
- No significant differences were found in intelligence, working memory, immediate recall, or executive function between groups.
- Patients with NS reported more subjective cognitive complaints, despite generally intact cognitive performance in most domains.
Conclusions:
- The cognitive profile of adults with Noonan syndrome is characterized by slowed information processing speed.
- Unlike children with NS who may have generalized deficits, adults show relatively preserved functioning in other cognitive domains.
- Subjective cognitive complaints in adults with NS may not always align with objective neuropsychological findings.
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