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
in Korean
| 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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Achilles Tendon
/
Wills
/
Biopsy
/
Muscle Weakness
/
Contracture
/
Muscular Dystrophy, Emery-Dreifuss
/
Death, Sudden
/
Dizziness
/
Elbow
/
Electrocardiography
Limits:
Adult
/
Female
/
Humans
Language:
Korean
Journal:
Korean Circulation Journal
Year:
2000
Type:
Article
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