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A Case of High Degree AV Block Treated by Implantation of Permanent Pacemaker in Emery-Dreifuss Muscular Dystrophy
Korean Circulation Journal ; : 1316-1322, 2000.
Article Dans Coréen | WPRIM | ID: wpr-145257
ABSTRACT
Emery-Dreifuss muscular dystrophy is characterized by 1) early contractures of the elbows, Achilles tendons, and postcervical muscles, 2) slowly progressive muscle wasting and weakness with a humeroperoneal distribution in the early stages, and 3) cardiomyopathy with conduction defects and risk of sudden death. The inheritance is usually X-linked recessive but can be autosomal dominant and recessive. We report a case of 28-year old woman who presented with dizziness, palpitation, and progressive muscular weakness. Her ECG revealed high degree AV block and muscle biopsy demonstrated diffuse degenerative change consistent with Emery-Dreifuss muscular dystrophy. She was diagnosed as autosomal dominant Emery-Dreifuss muscular dystrophy by characteristic clinical features, and findings of ECG, nerve conduction test, electromyography and muscle biopsy findings. A VVI-type permanent pacemaker was implanted.
Sujets)
Texte intégral: Disponible Indice: WPRIM (Pacifique occidental) Sujet Principal: Tendon calcanéen / Testaments / Biopsie / Faiblesse musculaire / Contracture / Dystrophie musculaire d'Emery-Dreifuss / Mort subite / Sensation vertigineuse / Coude / Électrocardiographie Limites du sujet: Adulte / Femelle / Humains langue: Coréen Texte intégral: Korean Circulation Journal Année: 2000 Type: Article

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Texte intégral: Disponible Indice: WPRIM (Pacifique occidental) Sujet Principal: Tendon calcanéen / Testaments / Biopsie / Faiblesse musculaire / Contracture / Dystrophie musculaire d'Emery-Dreifuss / Mort subite / Sensation vertigineuse / Coude / Électrocardiographie Limites du sujet: Adulte / Femelle / Humains langue: Coréen Texte intégral: Korean Circulation Journal Année: 2000 Type: Article