Digit Span Performance in Children with Dystrophinopathy: A Verbal Span or Working Memory Contribution?
In a large cohort of boys with dystrophinopathies and their unaffected siblings, we examined whether consistently observed performance on digit span is due primarily to a verbal span or executive deficit. We additionally assessed whether digit span performance contributed to the observed variability in reading performance noted in this population.



Performance of 170 boys with dystrophinopathy was compared to 95 unaffected sibling controls on measures of verbal function, reading, and digit span. Maximum digit span forward (DSF) and backward (DSB) lengths were converted to Z-scores using normative data. Independent sample t tests, analysis of variance, and hierarchical multiple regression were run (α=0.05).



Probands performed worse than controls on digit span, even after accounting for differences in general verbal function (p<.0001). Differences were significant for both DSF (p<.005) and DSB (p<.0001) span length, and an interaction effect yielded significantly worse DSB compared with DSF (p=.01). Reading performance was also lower in probands (p<.0001). The contribution of general level of verbal function, and forward and backward span lengths, did not vary between groups.



In boys with dystrophinopathy, decreased performance on digit span appears to be due to both decreased span forward (measuring verbal span only) and backward (measuring verbal span and working memory). The extent to which sibling controls exhibited better performance compared to the probands was significantly greater for backward span when compared with forward span. Thus, immediate verbal memory and executive control are differentially compromised among boys with dystrophinopathy, and both of these abilities independently contribute to reading performance. (JINS, 2016, 22, 777-784).
1Sergievsky Center & Department of Neurology,Columbia University,New York,New York.
Emily B
EB
Leaffer
1Sergievsky Center & Department of Neurology,Columbia University,New York,New York.
Robert J
RJ
Fee
1Sergievsky Center & Department of Neurology,Columbia University,New York,New York.
Veronica J
VJ
Hinton
D000293:Adolescent; D002648:Child; D060825:Cognitive Dysfunction / Q000209:etiology / Q000503:physiopathology*; D056344:Executive Function / Q000502:physiology*; D006801:Humans; D008297:Male; D008570:Memory, Short-Term / Q000502:physiology*; D009136:Muscular Dystrophies / Q000150:complications / Q000503:physiopathology*; D011932:Reading; D035781:Siblings
Children; Cognition; Duchenne muscular dystrophy; Reading; Verbal span; Working memory