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Poor Phonological Store in Duchenne Dystrophy

 Veronica Hinton, Robert Fee and Yaakov Stern
  
 

Abstract:
Abstract: Duchenne muscular dystrophy (DMD) is a neurogenetic developmental disorder that is caused by a mutation in a single gene which results in the absence of specific isoforms that normally localize to the CNS. Boys with DMD present with a wide range of intellectual function and selective verbal deficits. The current study examined verbal and memory skills more thoroughly in boys with DMD and their unaffected siblings. 20 boys with DMD and their sibling controls of similar age and estimated verbal IQ were administered the Clinical Evaluation of Language Fundamentals (CELF) and the Children's California Verbal Learning Test (CCVLT). On the CELF, the DMD group scored significantly more poorly (p<.01) than their siblings on the "Concepts and Directions" and "Recalling Sentences" subtests, but did not differ on the other subtests. No differences were observed between the two groups on various measures of the CCVLT. These findings highlight the selective nature of the verbal deficits associated with DMD. Subjects with DMD were able to learn and recall a list of words, identify words that categorically go together, name multiple items in semantic categories, and correctly answer questions about a paragraph they had heard. However, when asked to listen to and follow directions or repeat back sentences, they were unable to do this accurately. These findings suggest that immediate phonological store may be limited in children with DMD.

 
 


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