Table 3 Histological features of inherited and sporadic late-onset nemaline myopathies

Proportion of fibers with nemaline rods, mean (range)

53% (39–99%)

11% (1–47%)

Size ratio of fibers with and without rods, mean (range)

1.12 (0.84–1.36)

0.60 (0.30–1.03)

Atrophic fibers filled with rods, % of samples

11%

77%

Necrotic fibers, % of samples

11%

62%

Regenerating fibers, % of samples

22%

77%

Increased endomysial connective tissue, % of samples

11%

62%

Increased perimysial connective tissue, % of samples

56%

93%

Inflammation, % of samples

0%

8%

Distribution of nemaline rods within muscle fibers, % of samples*

Diffuse

11%

77%

Central aggregates

67%

23%

Subsarcolemmal aggregates

78%

23%