Journal of the International Neuropsychological Society



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


VERONICA J.  HINTON a1a2c1, DARRYL C.  DE VIVO a2a3, NANCY E.  NEREO a1, EDWARD  GOLDSTEIN a5 and YAAKOV  STERN a1a2a4
a1 Gertrude H. Sergievsky Center at the College of Physicians and Surgeons, Columbia University, New York, NY 10032
a2 Department of Neurology at the College of Physicians and Surgeons, Columbia University, New York, NY 10032
a3 Department of Pediatrics at the College of Physicians and Surgeons, Columbia University, New York, NY 10032
a4 Department of Psychiatry at the College of Physicians and Surgeons, Columbia University, New York, NY 10032
a5 Department of Neurology, Scottish Rite Children's Medical Center, Atlanta, GA

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.)

(Received September 17 1999)
(Revised December 20 1999)
(Accepted January 3 2000)


Key Words: Duchenne muscular dystrophy; Cognitive profile; Dystrophin; Developmental disability; Working memory.

Correspondence:
c1 correspondence to: Veronica J. Hinton, G.H. Sergievsky Center, P&S Box 16, Columbia University, 630 West 168th Street, New York, NY 10032. E-mail: HintonV@Sergievsky.CPMC.Columbia.edu


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