Abstract

ALVAREZ L, Alberto José; CESPEDES, Ghislaine; GONZALEZ, Jesús Enrique  and  LARA, Carmen. Atrofia muscular neurogénica en miopatía nemalínica: ¿Fenómeno primario o secundario? A propósito de un caso. Gac Méd Caracas [online]. 2003, vol.111, n.4, pp.308-312. ISSN 0367-4762.

Nemaline myopathy is a congenital non-progressive myopathy which shows rod-shaped structures or "nemalines" in the muscle fibers. We present a severe neonatal form of nemaline myopathy with neurogenic muscle atrophy and some motors neurons with simple degeneration in the spinal cord without other evidence of denervation. Some authors have shown similar cases and evidence has been obtained that neurogenic muscle atrophy could be an alteration of innervation or that the muscle damage by nemalines may produce retrograde neurogenic lesion.

Keywords : Nemaline; Muscle; Myopathy; Denervation.