A: Partly atrophic and atrophic angulated muscle fibers and nuclei clumps indicative for a chronic and slowly progressive neurogenic muscular atrophy. No inflammatory infiltrates in the endomysial tissue. H&E staining, magnification x 400.
B: Myofibrillar ATPase (pH 9.4) shows partly atrophic muscle fibers of both fiber types with predominance of type two fibers. Enzyme histochemistry with mATPase at pH 9.4, magnification x 200.
C: Three T-cells adjacent to a blood vessel in the perimysial tissue. No evidence of endomysial inflammatory infiltrates. Immunohistochemistry with anti-CD3 and slight counterstain with hemalum, magnification x 400.
D: Physiological expression of MHC class I-antigen on endomysial capillaries while sarcolemmal upregulation is not noted. Immunohistochemistry with anti-MHC class I- antigen and slight counterstain with hemalum, magnification x 400.
Figure 2: Biopsy of quadriceps muscle (patient #1)