Cardiomyopathy and atrioventricular block in Emery-Dreifuss muscular dystrophy - A case report

Kanadasi M., Demirtas M., Guzel R., San M., Tuncer I.

ANGIOLOGY, vol.53, no.1, pp.109-112, 2002 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 53 Issue: 1
  • Publication Date: 2002
  • Doi Number: 10.1177/000331970205300116
  • Journal Name: ANGIOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.109-112
  • Çukurova University Affiliated: Yes


A 32-year-old woman is described as having the following characteristics of Emery-Dreifuss muscular dystrophy: humeroperoneal muscular atrophy and weakness, neck and elbow contractures with sinus bradycardia, first-degree atrioventricular block, and dilated cardiomyopathy. The biopsy specimen of skeletal muscle showed dystrophic characters a cardiac endomyocardial biopsy specimen showed adipose tissue infiltration and deposition of antihuman IgG. Emery-Dreifuss muscular dystrophy is an X-linked recessive myopathy. The patient had no familial background of the disease. This patient might have a sporadic inheritance pattern with severe cardiac involvement.