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Emery–Dreifuss muscular dystrophy

muscular dystrophy that chiefly affects muscles used for movement (skeletal) and heart (cardiac) muscle

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Record originEnglish Wikipedia
Text licenseCC BY-SA 4.0
Source revisionAug 25, 2026
Entity authorityQ1335642 ↗
Source-derived summary

Emery–Dreifuss muscular dystrophy (EDMD) is a type of muscular dystrophy, a group of heritable diseases that cause progressive impairment of muscles. EDMD affects muscles used for movement (skeletal muscles), causing atrophy, weakness, and contractures. It almost always affects the heart, causing abnormal rhythms, heart failure, or sudden cardiac death. It is rare, affecting 0.39 per 100,000 (1 per 250,000) people. It is named after Alan Eglin H. Emery and Fritz E. Dreifuss.

Classification

Emery–Dreifuss muscular dystrophy can be sub-classified by pattern of inheritance: X-linked, autosomal dominant, and autosomal recessive.

Autosomal dominant: heart problems with weakness (and wasting) of skeletal muscles and Achilles tendon contractures.

X-linked: result of the EMD gene mutation, characterized by cardiac involvement.

Autosomal recessive: characterized by cardiac issues, such as arrhythmia.

Signs and symptoms

The classic triad of EDMD consists of early contractures, muscle weakness, and heart involvement, typically manifesting in adolescence.

Editorial summary

Begin with the source’s own compact description: “Emery–Dreifuss muscular dystrophy” is muscular dystrophy that chiefly affects muscles used for movement (skeletal) and heart (cardiac) muscle. The dossier treats that line as a proposition to test through Emery, Dreifuss and muscular, not as a finished interpretation.

Editorial reviewA strong contextual entry point for chronology, institutions and public events when official records are distinguished from later interpretation. The current 145-word lead offers orientation but no explicit four-digit date, so chronology should not be assumed. The selected authority fields contribute no independent date. For this dossier, Emery, Dreifuss and muscular is the immediate research focus.
Editorial analysis

Why this record matters

The phrase “muscular dystrophy that chiefly affects muscles used for movement (skeletal) and heart (cardiac) muscle” supplies a clear boundary for inquiry. It also exposes the unanswered questions: who defined that boundary, when it became stable and which sources sit outside it.

Evidence profile

Contemporary correspondence, administrative files and participant testimony can test how later narratives organized the event or institution. The source revision retrieved here is dated Aug 25, 2026. The linked authority identifier is Q1335642.

Critical limits

Official terminology may obscure informal participation, dissent or communities documented only indirectly. The lead is largely declarative, so disagreement and counter-evidence require a deliberate search beyond the opening account. Authority statements aid reconciliation but still require their own references, qualifiers and ranks to be checked.

How to read it

Compare institutional narratives with records created by participants and affected communities. Dates and formal titles are useful anchors, but not substitutes for context.

Best used for
  • Event chronology
  • Institutional context
  • Locating named record creators
Verify next

Contemporary correspondence, government or organizational records, oral histories and cited historical scholarship.

Three-step research path

  1. Establish the record: confirm the title “Emery–Dreifuss muscular dystrophy”, its source revision and the description used here.
  2. Expand the search: follow Emery–Dreifuss muscular dystrophy primary sources, Emery–Dreifuss muscular dystrophy archive and Emery research across catalogues and specialist indexes.
  3. Test the account: compare the strongest cited source with the responsible institution’s current record and note any disagreement.

Questions for further research

  1. Which source most directly establishes the central claim about “Emery–Dreifuss muscular dystrophy”?
  2. Who created the surviving record, and for what administrative purpose?
  3. Which voices are present, absent or mediated by the institution?
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Source & attribution

This entry incorporates text from “Emery–Dreifuss muscular dystrophy” on English Wikipedia. Contributors are listed in the page history. Text is available under the Creative Commons Attribution-ShareAlike 4.0 License. Selected authority identifiers and statements are retrieved from Wikidata under CC0; their references and qualifiers remain part of the verification path.