Conflict of interest: none.
Verbal memory functioning in adolescents and young adults with costello syndrome: Evidence for relative preservation in recognition memory
Version of Record online: 5 AUG 2013
Copyright © 2013 Wiley Periodicals, Inc.
American Journal of Medical Genetics Part A
Volume 161, Issue 9, pages 2258–2265, September 2013
How to Cite
2013. Verbal memory functioning in adolescents and young adults with costello syndrome: Evidence for relative preservation in recognition memory. Am J Med Genet Part A 161A:2258–2265., , , , , , .
- Issue online: 14 AUG 2013
- Version of Record online: 5 AUG 2013
- Manuscript Accepted: 3 MAY 2013
- Manuscript Received: 31 OCT 2012
- Nemours Biomedical Research
- NIH COBRE P20 GM. Grant Number: 103464-8
- Costello syndrome;
- verbal cognition;
- academic skills;
Costello syndrome (CS) is a rare genetic disorder caused by germline mutations in the HRAS proto-oncogene which belongs to the family of syndromes called rasopathies. HRAS plays a key role in synaptic long-term potentiation (LTP) and memory formation. Prior research has found impaired recall memory in CS despite enhancement in LTP that would predict memory preservation. Based on findings in other rasopathies, we hypothesized that the memory deficit in CS would be specific to recall, and that recognition memory would show relative preservation. Memory was tested using word-list learning and story memory tasks with both recall and recognition trials, a design that allowed us to examine these processes separately. Participants were 11 adolescents and young adults with molecularly confirmed CS, all of whom fell in the mild to moderate range of intellectual disability. Results indicated a clear dissociation between verbal recall, which was impaired (M = 69 ± 14), and recognition memory, which was relatively intact (M = 86 ± 14). Story recognition was highly correlated with listening comprehension (r = 0.986), which also fell in the low-average range (M = 80 ± 12.9). Performance on other measures of linguistic ability and academic skills was impaired. The findings suggest relatively preserved recognition memory that also provides some support for verbal comprehension. This is the first report of relatively normal performance in a cognitive domain in CS. Further research is needed to better understand the mechanisms by which altered RAS-MAPK signaling affects neuronal plasticity and memory processes in the brain. © 2013 Wiley Periodicals, Inc.