Poor verbal working memory across intellectual level in boys with Duchenne dystrophy

Hinton, V.J.; De Vivo, D.C.; Nereo, N.E.; Goldstein, E.; Stern, Yaakov

Objective— To determine whether all boys with Duchenne muscular dystrophy (DMD) have a similar verbal and memory profile of skills, or whether only a subset is affected, and to determine whether the weak areas in their profile are substantially different from a control group. Methods— Performance of patients with DMD on neuropsychological tests of verbal and memory skills was examined in two ways. Standardized test scores for 80 boys with DMD (estimated IQ range, 70 to 160) were ranked individually from worst to best, and the individual rankings were compared across the group using Friedman rank analysis. Additionally, performance of 41 boys with DMD was compared with that of their sibling control subjects of similar age and estimated IQ using multivariate analysis of variance. Results— Individual cognitive profiles were significantly similar among the subjects with DMD, such that for most subjects digit span, story recall, and comprehension were the tests on which each performed most poorly. This finding remained true regardless of whether they were of high or low intellectual function. In contrast, no significant cognitive profile was found among their sibling control subjects, and when compared with their siblings, the DMD group scored significantly more poorly on digit span, comprehension, and story recall, but not on other verbal and memory measures. Conclusions— Boys with DMD have a specific cognitive profile, regardless of their general level of cognitive function. Specifically, boys with DMD performed more poorly on tests requiring attention to complex verbal information than they did on other verbal or memory measures. The possibility that the missing dystrophin brain products may contribute to selective cognitive processing is considered.



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February 11, 2022