Table 2. Histopathological Findings.

Variable	20-1	20-2	20-3	20-4
Overall interpretation	Nemaline myopathy	Neurogenic atrophy With myopathic features	Nemaline myopathy with neurogenic atrophy	Nonspecific congenital myopathy
Fiber type predominance	Type I	Type II	Type II	No predominance
Fiber size variation	+	+	+	+
Nemaline rods	+, Occasional	−	+ (1%-5% Fibers)-	−
Lipid accumulation	NA	+	+	+
Endomysial connective tissue	↑	↑	↑	↑
Myofiber degeneration/regeneration	+	−	−	+
Additional findings	Ring and atrophic fibers, internalized nuclei present	First muscle biopsy (in infancy) consistent with neurogenic atrophy; second (at age 11 y) showed grouped atrophy, round and angulated fibers, increased internalized nuclei	Grouped atrophy, angulated and atrophic fibers, nuclear chains	NA
Electron microscopy findings	Few nemaline rods, focal myofibrillar disruption, Z-band smearing	NA	Nemaline rods seen in continuity with Z-bands often associated with disruption of myofibrillar apparatus	NA

Abbreviations: NA, not available; +, present; −, absent; ↑, increasing.