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

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

ANGIOLOGY, cilt.53, sa.1, ss.109-112, 2002 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 53 Konu: 1
  • Basım Tarihi: 2002
  • Doi Numarası: 10.1177/000331970205300116
  • Dergi Adı: ANGIOLOGY
  • Sayfa Sayıları: ss.109-112


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.