Verbal Memory Functioning in Adolescents and Young Adults with Costello Syndrome: Evidence for Relative Preservation in Recognition Memory

dc.contributor.authorSchwartz, David D.
dc.contributor.authorKatzenstein, Jennifer M.
dc.contributor.authorHopkins, Elisabeth
dc.contributor.authorStabley, Deborah L.
dc.contributor.authorSol-Church, Katia
dc.contributor.authorGripp, Karen W.
dc.contributor.authorAxelrad, Marni E.
dc.contributor.departmentNeurology, School of Medicine
dc.date.accessioned2025-05-02T14:42:59Z
dc.date.available2025-05-02T14:42:59Z
dc.date.issued2013
dc.description.abstractCostello 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.
dc.eprint.versionAuthor's manuscript
dc.identifier.citationSchwartz DD, Katzenstein JM, Hopkins E, et al. Verbal memory functioning in adolescents and young adults with Costello syndrome: evidence for relative preservation in recognition memory. Am J Med Genet A. 2013;161A(9):2258-2265. doi:10.1002/ajmg.a.36078
dc.identifier.urihttps://hdl.handle.net/1805/47652
dc.language.isoen_US
dc.publisherWiley
dc.relation.isversionof10.1002/ajmg.a.36078
dc.relation.journalAmerican Journal of Medical Genetics: Part A
dc.rightsPublisher Policy
dc.sourcePMC
dc.subjectCostello syndrome
dc.subjectAcademic skills
dc.subjectMemory
dc.subjectNeuropsychology
dc.subjectVerbal cognition
dc.titleVerbal Memory Functioning in Adolescents and Young Adults with Costello Syndrome: Evidence for Relative Preservation in Recognition Memory
dc.typeArticle
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