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Cardiomyopathy and Atrioventricular Block in Emery-Dreifuss Muscular Dystrophy

A Case Report

Mehmet Kanadai, MD

Balcali-Adana, Turkey

Mustafa Demirta, MD

Balcali-Adana, Turkey

Rengin Güzel, MD

Balcali-Adana, Turkey

Mustafa an, MD

Balcali-Adana, Turkey

Ilhan Tuncer, MD

Balcali-Adana, Turkey

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 cardiomy opathy. The biopsy specimen of skeletal muscle showed dystrophic character; 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 inheri tance pattern with severe cardiac involvement.

Angiology, Vol. 53, No. 1, 109-112 (2002)
DOI: 10.1177/000331970205300116


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