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Abstract:
Duchenne muscular dystrophy (DMD) is a neurogenetic
developmental disorder which results in the absence of specific
isoforms that normally localize to the CNS. Boys with DMD present
with varying intellectual function with specific evidence of verbal
phonological processing deficits. Many parents report attentional
problems, but it is unclear whether the observed problems are
separate from the phonological processing deficits. To examine
attentional processes in boys with DMD, 11 boys with DMD and their
sibling controls of similar age and estimated IQ were administered
Halperin's A-X Continuous Performance Test (CPT) (a
non-phonological test that involves subjects watching individual
letters flash on a computer screen and responding to the A-X
sequence, generating scores for Inattention, Impulsivity and
Dyscontrol), and two measures of attentional span - one that relies
on phonological processing (Digit Span, DS) and one that is
visually based (Finger Windows, FW). Parents rated their child with
DMD as having significantly more Attention problems than his
sibling on the Child Behavior Checklist. Childrens' test
performance showed no significant between group differences on the
CPT Inattention and Dyscontrol measures, but DMD subjects had
higher Impulsivity scores than their sibling controls. Further, DMD
subjects performed more poorly on DS, but not FW, than controls.
These findings highlight the selective nature of the deficits
associated with DMD, and argue that what appears as "inattention"
is related to impulsivity and phonological processing
deficits.
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