Elsevier

Neuropsychologia

Volume 47, Issues 8–9, July 2009, Pages 1883-1892
Neuropsychologia

Visual and verbal learning in a genetic metabolic disorder

https://doi.org/10.1016/j.neuropsychologia.2009.02.032Get rights and content

Abstract

Visual and verbal learning in a genetic metabolic disorder (cystinosis) were examined in the following three studies. The goal of Study I was to provide a normative database and establish the reliability and validity of a new test of visual learning and memory (Visual Learning and Memory Test; VLMT) that was modeled after a widely used test of verbal learning and memory (California Verbal Learning Test; CVLT). One hundred seventy-two neurologically intact individuals ages 5 years through 50 years were administered the VLMT and the CVLT. Normative data were collected and the results suggested that the VLMT is a reliable and valid new measure of visual learning and memory. The aim of Study II was to examine possible dissociations between verbal and visual learning and memory performances in individuals with cystinosis as well as to assess changes in performance as individuals with the disorder age. Thirty-seven individuals with cystinosis and 37 matched controls were administered a new test of visual learning and memory (Visual Learning and Memory Test; VLMT) and the California Verbal Learning Test (CVLT). Individuals with cystinosis performed at a lower level than controls on almost all indices of visual learning and memory while no differences were found between the groups on the verbal measure. Examination of the results on the VLMT indicated that the visual learning and memory impairment in cystinosis may result from difficulty with processing visual information quickly. Study III aimed to remediate the observed visual learning and memory deficit by implementing an intervention that increased the exposure time for visual stimuli. Fifteen individuals with cystinosis were administered a version of the VLMT in which the stimuli were exposed for 3 s rather than 1 s. Fifteen matched controls were administered the 1-s version of the VLMT. The results of Study III indicated that by increasing the exposure time for each visual stimulus, individuals with cystinosis were able to perform at the same level as control subjects. This is the first study to demonstrate impaired visual learning and spared verbal learning in individuals with cystinosis. These results may provide the foundation for designing cognitive interventions, may lead to further hypotheses regarding the underlying mechanism of the observed visual learning and memory deficit, and have implications for a greater understanding of gene–behavior relationships.

Section snippets

Study I: normative study of the Visual Learning and Memory Test (VLMT)

The purpose of Study I was to provide a normative database for the VLMT by gathering data on a large group of control subjects, as well as to establish the reliability and validity of the VLMT as a new measure of visual learning and memory.

Study II: visual and verbal learning and memory

The aim of Study II was to examine possible dissociations between verbal and visual learning and memory performances in individuals with cystinosis as well as to assess changes in performance as individuals with the disorder age.

Study III—intervention study: use of the Visual Learning and Memory Test-3 s Exposure Time (VLMT-3″)

The current study aimed to remediate the observed visual learning deficit observed in cystinosis with an intervention that increased exposure time to visual information.

Overall discussion

The current study has implications for a better understanding of the behavioral phenotype associated with cystinosis. The results indicate a dissociation between visual and verbal learning and memory such that visual learning and memory is significantly more affected than verbal learning and memory.

These results, taken collectively with the results from prior studies, indicate that individuals with cystinosis have deficits in visual learning and memory, visuospatial abilities (Ballantyne &

Limitations and future directions

The current study yields important information about the cognitive deficits in cystinosis, which can be examined further in future studies. The results of the current study suggest that processing visual information given a short exposure time may be the core deficit in VLMT performance in cystinosis. One potential hypothesis is that, perhaps, individuals with cystinosis have an impairment in visual processing speed. However, the VLMT was intended as a test of visual learning and memory, not of

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    This project was funded by the Cystinosis Foundation, the Oaktree Foundation, and the National Institutes of Health (NINDS 5-P50-NS22343).

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