Principles of Rehabilitation Medicine >Myopathies

Raj Mitra+
Table 75–1Muscular Dystrophies

Average age of onset for

Type of Dystrophy Inheritance pattern Protein Affected Age of Onset Rate of Progression Life Expectancy (years)
daily wheelchair use

Duchenne (DMD) X-linked Dystrophin 2–6 years old Moderate-fast 10–12 years old 20–30

Variable, typically a few Variable, after 15 years

Becker (BMD) X-linked Dystrophin Slow to moderate 40–50
years later than DMD old

Emerin (X-linked); other

30s in the X-linked form,
Emery-Dreifuss (EDMD) X-linkedAD/AR proteins in AD and AR Teens Moderate Variable
variable in the other forms
forms

Variable. Note: 20%–30%

Facioscapulohumeral
AD DUX4 Infancy–40s Slow of people with FSHD Generally unaffected
(FSHD)
ultimately use wheelchairs

LimbGirdle (LGMD) AD/AR Variable Variable Moderate Variable Variable

Typically, no wheelchair
Oculopharyngeal (OPMD) AD PABP2 30s–40s Moderate Unaffected
needed

AD, Autosomal Dominant; AR, Autosomal Recessive; BMD, Becker Muscular Dystrophy; DMD, Duchenne Muscular Dystrophy; EDMD, Emery-Dreifuss Muscular Dystrophy; FSHD, Facioscapulohumeral Muscular
Dystrophy; LGMD, Limb Girdle Muscular Dystrophy; MD, Muscular Dystrophy; OPMD, Oculopharyngeal Muscular Dystrophy.
Duchenne and Becker Muscular Dystrophy (DMD and BMD; dystrophinopathies)

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