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Abstract:
Duchenne muscular dystrophy (DMD) is a neurogenetic
developmental disorder due to the absence of specific isoforms that
normally localize to the muscle and CNS. The disease results in
progressive muscular weakness. Boys with DMD present with varying
intellectual function with specific evidence of verbal phonological
processing deficits. For the current study, 25 boys with DMD were
tested two years apart to determine whether the cognitive deficits
are progressive. Subjects were given the Peabody Picture Vocabulary
(PPV), Digit Span (DS), Similarities (S), Story Memory (SM) and
Picture Memory (PM) Tests. Previous results (from time 1) indicated
the DMD subjects to perform more poorly than their siblings on DS
and SM., but not the other measures. Paired t-tests of standard
scores revealed no differences between time one and time two.
Further, when compared to sibling controls on time 2, the cognitive
profile was the same as at time 1. These results indicate that the
cognitive effects in DMD are constant, unlike the progressive
muscle weakness. Subjects with DMD maintain relatively poorer
performance on tests that require phonological processing over
time.
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