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  • Title: An unusual case of muscular limb-girdle dystrophy and mitochondrial myopathy.
    Author: Calore EE, Cavaliere MJ, Wakamatsu A, Perez NM, Maeda MY, Russo DH.
    Journal: Pathologica; 1994 Dec; 86(6):649-51. PubMed ID: 7617396.
    Abstract:
    Limb-girdle muscular weakness and wasting could be caused by different diseases (inflammatory and hereditary myopathies, muscular dystrophies and neurogenic atrophies). Among these, Limb-Girdle Muscular Dystrophy (LGMD) is an heterogeneous group of pathologies that have progressive proximal limbs and girdle weakness, with some dystrophic features by the muscle biopsy. We studied a case of LGMD in an adult man with a typical histological and histochemical profile, associated to a mitochondrial deficit characterized by presence of ragged-red fibers, a histochemical Cytochrome Oxidase deficiency and abnormal mitochondria by ultrastructure.
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