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

Published online by Cambridge University Press:  09 February 2001

VERONICA J. HINTON ,
DARRYL C. DE VIVO ,
NANCY E. NEREO ,
EDWARD GOLDSTEIN  and
YAAKOV STERN
VERONICA J. HINTON
Affiliation:
Gertrude H. Sergievsky Center at the College of Physicians and Surgeons, Columbia University, New York, NY 10032 Department of Neurology at the College of Physicians and Surgeons, Columbia University, New York, NY 10032
DARRYL C. DE VIVO
Affiliation:
Department of Neurology at the College of Physicians and Surgeons, Columbia University, New York, NY 10032 Department of Pediatrics at the College of Physicians and Surgeons, Columbia University, New York, NY 10032
NANCY E. NEREO
Affiliation:
Gertrude H. Sergievsky Center at the College of Physicians and Surgeons, Columbia University, New York, NY 10032
EDWARD GOLDSTEIN
Affiliation:
Department of Neurology, Scottish Rite Children's Medical Center, Atlanta, GA
YAAKOV STERN
Affiliation:
Gertrude H. Sergievsky Center at the College of Physicians and Surgeons, Columbia University, New York, NY 10032 Department of Neurology at the College of Physicians and Surgeons, Columbia University, New York, NY 10032 Department of Psychiatry at the College of Physicians and Surgeons, Columbia University, New York, NY 10032
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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. (JINS, 2001, 7, 45–54.)

Keywords

Duchenne muscular dystrophyCognitive profileDystrophinDevelopmental disabilityWorking memory
Type
Research Article
Information
Journal of the International Neuropsychological Society , Volume 7 , Issue 1 , January 2001 , pp. 45 - 54
Copyright
© 2001 The International Neuropsychological Society

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