par Telerman Toppet, Nicole ;Gerard, Jean-Marie ;Coërs, Christian
Référence Journal of the neurological sciences, 19, 2, page (207-223)
Publication Publié, 1973
Article révisé par les pairs
Résumé : Bilateral pes cavus in a 48 yr old woman, with no muscle weakness, was the sole manifestation of central core disease. Characteristic histological changes were found in gastrocnemius and in the clinically unaffected biceps brachii. Histochemical observations confirmed the exclusive location of central cores in type I fibers. In type II fibers there was progressive atrophy and rarefaction, either by longitudinal splitting or by conversion from type II to type I. Increased collateral branching of subterminal motor axons supports the hypothesis of the neurogenic nature of the disease. Both central cores and targets may represent stages of a non specific metabolic disorder occurring within restricted areas of fibers having high mitochondrial enzyme activity.