Emery-Dreifuss Muscular Dystrophy with Cardiac Involvement
Korean Circulation Journal
;
: 160-165, 1992.
Article
in Korean
| WPRIM
| ID: wpr-211395
ABSTRACT
Emery-Dreifuss muscular dystrophy has become recognized as a distinct neuromuscular disorder with features including X-linked inheritance, insidious onset in childhood of a distinct pattern of muscle contractures and weakness, slow progression without loss of ambulation, and occurrence by mid-childhood of atrial conduction defects, which, if untreated, cause sudden death. We report a case of Emery-Dreifuss dystrophy with cardiac involvement of atrial standstill. The patient was 24 year-old man, who had suffered from dyspnea and bradycardia and was inserted by VVI type permanent pacemaker. Cardiac involvement usually becomes evident as muscle weakness progress and provided that the diagnosis is made sufficiently early, the insertion of a cardiac pacemaker can be life saving.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Bradycardia
/
Walking
/
Muscle Weakness
/
Contracture
/
Muscular Dystrophy, Emery-Dreifuss
/
Death, Sudden
/
Diagnosis
/
Dyspnea
/
Genes, X-Linked
Type of study:
Diagnostic study
Limits:
Humans
Language:
Korean
Journal:
Korean Circulation Journal
Year:
1992
Type:
Article
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