Long term memory profile of disorders associated with dysregulation of the RAS-MAPK signaling cascade

Behav Genet. 2011 May;41(3):423-9. doi: 10.1007/s10519-011-9446-5. Epub 2011 Jan 28.

Abstract

In the present study we evaluated long term memory in twenty individuals with molecularly confirmed diagnosis of Noonan syndrome and LEOPARD syndrome, two disorders caused by mutations in genes coding transducers participating in the RAS-MAPK signaling cascade. The profile of explicit long term memory abilities was investigated using PROMEA, which includes a battery of tests specifically developed to assess memory and learning in verbal, visual and spatial domains. Ten individuals (50%) had impaired (≤5th percentile) or below average (≤15th percentile) performance on a delayed verbal free recall memory task, four (20%) on a delayed visual recognition memory task, and only one (5%) on a delayed spatial recognition memory task. Our data suggest that dysregulation of the RAS-MAPK cascade may be associated with a pattern of reduced verbal recall memory performance but relative sparing of visual and spatial recognition memory.

MeSH terms

  • Child
  • Child, Preschool
  • Cohort Studies
  • DNA Mutational Analysis
  • Female
  • Humans
  • LEOPARD Syndrome / diagnosis
  • LEOPARD Syndrome / genetics*
  • Learning Disabilities / diagnosis
  • Learning Disabilities / genetics*
  • MAP Kinase Signaling System / genetics*
  • Male
  • Memory Disorders / diagnosis
  • Memory Disorders / genetics*
  • Memory, Long-Term*
  • Mitogen-Activated Protein Kinases / genetics*
  • Neuropsychological Tests
  • Noonan Syndrome / diagnosis
  • Noonan Syndrome / genetics*
  • Phenotype
  • ras Proteins / genetics*

Substances

  • Mitogen-Activated Protein Kinases
  • ras Proteins