[Clinical pathologic studies and genetic analysis of a female Duchenne muscular dystrophy family]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2005 Feb;22(1):65-7.
[Article in Chinese]

Abstract

Objective: To investigate the clinical features of female Duchenne muscular dystrophy(DMD), and find out the onset mechanism.

Methods: The clinical manifestations of a female DMD family were followed; the immunofluorescence studies on muscle system and the genetic analysis were carried out.

Results: The clinical manifestations and results of relevant examinations on the DMD woman in this family were in accordance with the typical characteristics of DMD. The 39-year-old mother of this proband was noted to have a clinical feature resembling that of Becker muscular dystrophy (BMD), and the immunofluorescence analysis revealed that dystrophin positive fibers and negative fibers co-existed in her muscle. The dystrophy genetic analysis of the family indicated non-deletions. The mother's karyotype was found to be normal.

Conclusion: The 39-year-old female patient's clinical manifestations were similar to BMD, and only one third of her fibers were dystrophin-positive. The present authors assume that the skewed pattern of X inactivation is the likely mechanism, because the karyotype is normal.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Dystrophin / genetics
  • Dystrophin / metabolism
  • Family Health
  • Female
  • Fluorescent Antibody Technique
  • Humans
  • Karyotyping
  • Male
  • Muscular Dystrophy, Duchenne / genetics*
  • Muscular Dystrophy, Duchenne / metabolism
  • Muscular Dystrophy, Duchenne / pathology*
  • Pedigree

Substances

  • Dystrophin