Emery-Dreifuss muscular dystrophy, abbreviated EDMD, is one type of muscular dystrophy, the group of inherited diseases that cause progressive weakening of muscles. It affects the skeletal muscles used for movement, causing wasting of muscle, weakness and contractures. It almost always involves the heart as well, producing abnormal rhythms, heart failure or sudden cardiac death. The disease is rare, affecting about 0.39 per 100,000 people, or roughly one in 250,000. It is named after Alan Eglin H. Emery and Fritz E. Dreifuss.
Facts
Classification
ICD-10 ChapterVI: Diseases of the Nervous System 2 Communicable or Non-communicable Causative Agent
Causative Agent (category) Prevalence Band Sources
1. Emery-Dreifuss muscular dystrophy (Wikipedia)
Lead, second sentence
EDMD affects muscles used for movement (skeletal muscles), causing atrophy, weakness, and contractures.
Lead, third sentence
It almost always affects the heart, causing abnormal rhythms, heart failure, or sudden cardiac death.
Lead, first sentence
a type of muscular dystrophy, a group of heritable diseases that cause progressive impairment of muscles
View the Source2. World Health Organization: ICD-10 Version:2019, Chapter VI, G70-G73
World Health OrganizationICD-10 2019 category G71.0Quote, ICD-10 2019 category G71.0
G71.0 Muscular dystrophy
3. National Cancer Institute (NCI Thesaurus): Emery-Dreifuss muscular dystrophy - NCI Thesaurus concept C84685
National Cancer Institute (NCI Thesaurus)DefinitionQuote, Definition
An X-linked or autosomal dominant inherited muscular dystrophy.
4. Orphadata (Orphanet): Orphadata epidemiology, ORPHAcode 261
Orphadata (Orphanet)Orphanet epidemiology, ORPHAcode 261Quote, Orphanet epidemiology, ORPHAcode 261
"PrevalenceClass": "1-9 / 1 000 000", "PrevalenceGeographic": "Europe", "PrevalenceQualification": "Value and class", "PrevalenceType": "Point prevalence"
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