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May 1969

Mild and Severe Forms of X-Linked Muscular Dystrophy

Author Affiliations

Halifax, Nova Scotia, Canada; Charlottesville, Va
From the Department of Biology, Wayne State University, Detroit (Dr. Shaw) and the Department of Neurology, University of Virginia School of Medicine, Charlottesville (Dr. Dreifuss). Dr. Shaw is now in the Departments of Pediatrics and Preventive Medicine, and the Atlantic Research Center for Mental Retardation, Dalhousie University, Halifax, Nova Scotia, Canada.

Arch Neurol. 1969;20(5):451-460. doi:10.1001/archneur.1969.00480110015001

IT IS generally held that X-linked muscular dystrophy occurs in two forms, mild and severe. The two disorders, however, are not easily distinguished, and we need to know on what basis an individual case can best be assigned to one category or the other. There is no effective therapy, and the victims go unremittingly downhill to death. Since some are only mildly affected and live for many years while others never live to enter their second decade, accurate statements of prognosis are very much to be desired. Equally important are statements of risk to other family members, especially asymptomatic brothers and brothers yet to be born.

The objectives of the present paper are accordingly to show (1) the existence of two types as well as the progression and prognosis characteristic of each, (2) how individuals can best be assigned to one group or the other, and (3) how statements of

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