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.
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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