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. 2013 Dec;32(3):154–165.

Table 3.

Muscle histopatology in DM1 and DM2.

Histopathological findings DM1 DM2
Fiber size variation +++ +++
Internal nuclei +++ +++
more in type 2 fibers
Type 1 fiber atrophy ++ -
Type 2 fiber atrophy + ++
Type 2 fiber hypertrophy - +
Nuclear clump fibers +
at advanced stages only
+++
more in advanced stages
Atrophic fibers (diam. ≤ 6μm) ±
type 1 and type 2 fibers at advanced stages only
+++
type 2 fibers
Ring fibers ++ +
Sarcoplasmic masses ++ ±
Fibrosis +++
at late stages only
++
at late stages only
Fatty replacement +++
at late stages only
++
at late stages only

+++ present in >75% of biopsies; ++ present in 20-50% of biopsies; + present in 10-24% of biopsies; ± occasionally present; - absent