PMC6180937_01
MRI of the thighs in axial T2 (a) and coronal T2 (b) sections shows bilateral fibroadipose degeneration of the adductor and biceps femoris muscles.
CRINM2018-4127213.001.jpg
PMC6180937_01_CRINM2018-4127213.001.jpg
fig1
Light microscopy of the left vastus lateralis ((a), resin; (b)-(f), cryostat). (a) Vacuoles filled with polysaccharide material (PAS, x 40); (b) vacuoles stained positively for acid phosphatase reaction (x 20); (c) ATPase histochemistry at pH 4.3 revealed selective atrophy of type 1 fibers (x 40); (d) vacuoles are present predominantly in type 1 fibers (x 40); (e)-(f) atrophic fibers containing numerous nemaline bodies (Gomori trichrome, x 100).
CRINM2018-4127213.002.jpg
PMC6180937_01_CRINM2018-4127213.002.jpg
fig2
Furthermore, many of these atrophic fibers (7% of all fibers contained in transverse sections) displayed numerous, discrete, deposits of basophilic material, located centrally within the fiber cytoplasm or, in some instances, in a subsarcolemmal position (Figure 2).
By EM, nemaline bodies are oriented consistently with the longitudinal axis of the fibers and they are composed of myofibrillar material in apparent continuity with Z-bands ((a), x 28.000; (b), x 45.000).
CRINM2018-4127213.003.jpg
PMC6180937_01_CRINM2018-4127213.003.jpg
fig3
These were composed of myofibrillar material in apparent continuity with Z-bands (Figure 3).