A Case of High Degree AV Block Treated by Implantation of Permanent Pacemaker in Emery-Dreifuss Muscular Dystrophy.
10.4070/kcj.2000.30.10.1316
- Author:
Jong Seo HONG
;
Ji Hyang KANG
;
Goung Sup LEE
;
Chang Seon LEE
;
Hyun Ju CHOI
;
Byung Doo LEE
;
June Soo KIM
;
Yeon Lim SUH
;
Duk Kyung KIM
;
Je Geun CHI
;
Kyoung Ju AHN
- Publication Type:Original Article
- MeSH:
Achilles Tendon;
Adult;
Atrioventricular Block*;
Biopsy;
Cardiomyopathies;
Contracture;
Death, Sudden;
Dizziness;
Elbow;
Electrocardiography;
Electromyography;
Female;
Humans;
Muscle Weakness;
Muscles;
Muscular Dystrophy, Emery-Dreifuss*;
Neural Conduction;
Wills
- From:Korean Circulation Journal
2000;30(10):1316-1322
- CountryRepublic of Korea
- Language:Korean
-
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.
