[Nephrotoxicity of cyclosporin. Clinical, physiopathological and histological aspects].
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Biomedical subjects
Publications and source records attributed to M Ben Hmida.
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Polysaccharide myopathy is a rare form of storage muscular disorder. The clinical picture of this particular form of myopathy is unspecific. We report a 62-year-old woman with late-onset progressive weakness and wasting, affecting proximal muscles of the four limbs and the girdles. No myalgia, dysphagia nor symptoms of cardiac failure were observed. Muscle biopsy revealed a vacuolar myopathy with accumulation of amylopectin-like polysaccharide. This material was strongly PAS-positive and diastase-resistant. At electron microscopy, the deposits were composed of non-membrane-bound filamentous and granular material surrounded by numerous mitochondria. No enzyme deficiency was found. Clinical presentation of our patient was similar to the 16 cases reported in the literature. She did not have myocardiopathy and her survival is much longer. Hypothetic mechanisms of polysaccharide accumulation are reviewed.
Histiocytosis X is an uncommon disease. Temporal bone involvement is a frequent head and neck manifestation. Most patients are under fifteen years of age. The authors report a case of a bilateral extensive temporal involvement complicated with vertigo and peripheric facial palsy. CT plays a dominant role in the diagnosis because of the ability to identify bone destruction, soft tissue involvement and intra-cranial histiocytosis more accurately.
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