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Abstract:
Abstract: Cystinosis is a metabolic disorder in which the
amino acid cystine accumulates in various organs. Children with
cystinosis have normal intelligence and verbal learning but do less
well than controls on visual learning tasks. The purpose of this
study was to examine visual and verbal learning in adults with
cystinosis, as the cumulative effect of this disorder on the brain
is unknown. Subjects were 6 adults with cystinosis (25.90 + 6.00
years) and 37 controls (26.42 + 9.88 years). All subjects were
administered both the California Verbal Learning Test (CVLT) and a
version of the Visual Spatial Learning Task (VSLT) modified to be
analogous to the CVLT, but in a recognition rather than a recall
format. The subject is shown a series of 15 abstract stimulus
designs (one per second), and then must mark all the stimulus
designs on a grid of designs (15 are stimulus designs and 15 are
foils). This is done for 5 consecutive trials and a 20-minute
delayed recall trial; the presentation order is always the same but
the designs are randomized on each grid. Results showed that
cystinotics performed significantly more poorly than controls on a
number of indices on both the visual and the verbal task. This
suggests that although verbal learning and memory may be spared in
the early stages of cystinosis, as the disease progresses more
widespread dysfunction may become apparent.
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