Figure 2

Electron microscopy for group G1-severe NM. A. P1. Sarcomeric structure is completely disrupted. Fragment of sarcomeres are intermingled with amorphous material containing organelles and glycogen granules. B. P2. Scattered globular ovoidal nemaline bodies are found inside a fiber showing partial sarcomeric alteration. C. P3. Presence of three muscle fibres presenting different degree of alterations. Nemaline bodies are found in subsarcolemmal areas (indicated by an arrow). The fibre above the first one shows sarcomeric disarray and thicknened Z-lines probably leading to globular rods formation (indicated by an asterisk). A small atrophic fibre is completely invaded by rounded nemaline bodies (indicated by a star). D. P4. Globular nemaline bodies present thin filaments coming out from the thinner edges. Original magnification: A. 11,000x. B. 7,000x C. 8,400x D. 51,000x.