June 1965

Speech Mechanism in Pseudohypertrophic Muscular Dystrophy

Author Affiliations

From the Children's Neurology Service and Hektoen Institute for Medical Research, Cook County Hospital, Chicago, and the Department of Communicative Disorders, Northwestern University, Evanston. Assistant Professor, Department of Communication Disorders, University of Oklahoma Medical School, Oklahoma City (Dr. Sanders) and Associate Professor, Department of Pediatrics, Northwestern University Medical School, Chicago (Dr. Perlstein).

Am J Dis Child. 1965;109(6):538-543. doi:10.1001/archpedi.1965.02090020540008

PSEUDOHYPERTROPHIC muscular dystrophy of Duchenne is the most common form of muscular dystrophy seen in children. It is transmitted as a sex-linked recessive gene, occurs primarily in males, is generally manifest by the 3rd year of life, and results in death, usually before the 25th year. There is a characteristic progression of muscular weakness starting in the pelvic girdle and trunk, and eventually involving virtually all the striated musculature, including the tongue, facial, masticatory, and respiratory muscles.1,2 The visceral musculature is spared. Enlargement of the calf muscles and occasionally of other muscle groups accounts for the name pseudohypertrophic muscular dystrophy. The diagnosis in the advanced stages is not difficult. However, in the early stages it may have to depend on laboratory studies, such as serum enzymology, electromyography, and muscle biopsy, because clinical signs of weakness may not appear until approximately 50% of the maximum muscle strength is lost.

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