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September 1967

Hereditary Myopathy Limited to Females

Author Affiliations

From the Department of Neurology and the Division of Neuropathology, Indiana University Medical Center, Indianapolis.

Arch Neurol. 1967;17(3):238-247. doi:10.1001/archneur.1967.00470270016003

HEREDITARY occurrence of various myopathies is well established.1-3 Duchenne, in the 1860's, observed a preponderance of affected males, 11 out of 13 cases,4 and the eponym "Duchenne muscular dystrophy" has come to epitomize a disease limited to young boys.4,5 However, familial cases, similar to this type of muscular dystrophy, have also been seen in girls,6-11 and some 40 cases12 are known.

The mode of inheritance of muscular dystrophy of later onset is not as clear. Although families with sex-linked inheritance are known,3,13,14 simple autosomal recessive inheritance usually prevails. There are also many sporadic cases.12,15

Though a slight preponderance of females has been reported16 in facioscapulohumeral dystrophy, exclusive involvement of females in one kindred has not been recorded. A family with limb-girdle dystrophy exhibiting precisely such a pattern of inheritance is here presented.

Report of Cases  The following Anglo-Saxon family, residing in

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