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Paul E. Barkhaus, MD
Neurology Service (127) Minneapolis Veterans Administration Medical Center Minneapolis, MN 55417

James M. Gilchrist, MD
Department of Neurology Brown University Providence, RI 02903

Arch Neurol. 1990;47(9):951-952.

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In Reply.

—Limb-girdle dystrophy is a rubric that is considered "obsolete" by some authors¹ and may be best considered a syndrome. We were unable to find specific discussions on autosomal dominant limb-girdle dystrophy in the citations^1,2 offered by Boylan. We agree that one cannot clinically differentiate the autosomal dominant from the autosomal recessive forms.³ Without adequate family history and pedigree analysis, an autosomal dominant pattern of inheritance, although quite rare,³ obviously cannot be excluded. If an autosomal dominant pattern is established, however, one can virtually eliminate the possibility of a Duchenne dystrophy manifesting carrier since there cannot be male-to-male transmission of Duchenne dystrophy. This explains the focus on autosomal recessive and sporadic limb-girdle dystrophy as the major differential diagnosis for Duchenne dystrophy manifesting carrier in our article.⁴ Perhaps we should simplify this issue and reiterate that females with a limb-girdle syndrome and no family history . . . [Full Text PDF of this Article]

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