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. 2001 Jan;7(1):45-54.
doi: 10.1017/s1355617701711058.

Selective deficits in verbal working memory associated with a known genetic etiology: the neuropsychological profile of duchenne muscular dystrophy

V J Hinton  1 , D C De Vivo, N E Nereo, E Goldstein, Y Stern
Affiliations

Selective deficits in verbal working memory associated with a known genetic etiology: the neuropsychological profile of duchenne muscular dystrophy

V J Hinton et al. J Int Neuropsychol Soc. 2001 Jan.

Abstract

Forty-one boys diagnosed with Duchenne muscular dystrophy (DMD) were each compared to an unaffected sibling on a battery of neuropsychological tests. Verbal. visuospatial, attention/memory, abstract thinking, and academic achievement skills were tested. Results indicated the boys with DMD performed similarly to their siblings on the majority of measures, indicating intact verbal, visuospatial, long-term memory, and abstract skills. However, the DMD group did significantly more poorly than their siblings on specific measures of story recall, digit span, and auditory comprehension, as well as in all areas of academic achievement (reading, writing, and math). This profile indicates that verbal working memory skills are selectively impaired in DMD, and that that likely contributes to limited academic achievement. The association between the known impact of the genetic mutation on the development of the central nervous system and boys' cognitive profile is discussed.

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Figures

Fig. 1
Fig. 1
Distribution of proband and sibling scores on the PPVT-R. Only participants with standard scores > 70 were included in this study to ensure a minimum vocabulary.
Fig. 2
Fig. 2
(a,b) Scaled scores on the WISC-III Information and Digit Span Subtests for individual DMD subjects each paired with his own sibling control. Note that for Figure 2a there is great variability among sibling pairs on the Information subtest, while Figure 2b demonstrates on the Digit Span Subtest the majority of probands did worse than their siblings, yet still scored within normal limits.
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