Intellectual Impairment in Muscular Dystrophy

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Abstract
Severe intellectual impairment is a commonly associated feature of progressive muscular dystrophy of the Duchenne-type. It is probably an integral part of the disease process, and any theory of the etiology of the disease should take this into account. It may not be entirely accurate to consider muscular dystrophy as a disease confined to voluntary muscle. It is difficult to explain why some children should have a normal intelligence while others are severely retarded, in spite of similar severity of muscular involvement. A possible explanation in some of these cases is a different gene inherited by an autosomal recessive rather than a sex-linked recessive mechanism, or that separate genes are responsible for the intellectual impairment and the muscular involvement. A more likely explanation is a single biochemical lesion affecting brain and muscle, but with less acute onset in those with normal intelligence. Alternatively, a product of the muscle destruction may affect the developing brain.