Intellectual functioning in Duchenne muscular dystrophy: A review

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Abstract

Duchenne muscular dystrophy has traditionally been thought to be a primary disease of muscle, but recently it has been suggested that it may be secondary to a neuronal defect or to a generalized disorder of protein synthesis and membrane. However, to date there is no proof to support unequivocally any of these theories. A higher incidence of mental retardation and decreased intellectual functioning has been reported in the medical literature of Duchenne muscular dystrophy patients than for normals or other control groups. Recently there has been strong evidence to suggest that verbal ability, as reflected by the WISC Verbal scale IQ, may be more commonly and significantly impaired in Duchenne muscular dystrophy patients than is nonverbal ability, as reflected by the Performance scale IQ. This article presents a comprehensive review of data from the literature on intellectual functioning in Duchenne muscular dystrophy, as well as methodological issues involved in assessment of intelligence in this population. The intent is to provide a basis for future attempts to relate the intellectual deficit in Duchenne muscular dystrophy to neuropsychological and neurobiological parameters of the disease. (40 ref) (PsycINFO Database Record (c) 2006 APA, all rights reserved)

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