Abstract
A 45-year-old patient suffering from chronic renal insufficiency developed dermatopolymyositis. Since the patient did not respond to treatment with high doses of prednisone and immunoglobulin, concomitant cyclosporine A was added. Four months later, worsening signs of bilateral pyramidal disorder and an altered state of consciousness appeared. Serial computed tomography (CT) and magnetic resonance imaging (MRI) revealed multiple alterations of the cerebral white matter. Cyclosporine was then discontinued. One month later, exitus occurred. Microscopic examination of the brain showed diffuse tumescence; histological examination revealed perivascular and diffuse lymphomonocyte infiltrations, areas of demyelination, astrocytes with bizarre nuclei, and oligodendrocytes with enlarged nuclei due to hyperchromatic inclusion. Morphological examination confirmed the presence of intranuclear icosahedral viral bodies. Progressive multifocal leukoencephalopathy was diagnosed. The literature contains only one report of an analogous case observed during a course of cyclosporine treatment for Wegener's granulomatosis.
Sommario
Una paziente di 45 anni, affetta da insufficienza renale cronica, sviluppa una dermatopolimiosite. La mancata risposta alla terapia con prednisone ed immunoglobuline, ad alte dosi, porta all'aggiunta della ciclosporina. A distanza di 4 mesi compaiono segni ingravescenti di sofferenza piramidale bilaterale e disturbi dello stato di coscienza. Si eseguono TC e TRM encefaliche che evidenziano alterazioni multiple della sostanza bianca. La ciclosporina viene sospesa. A distanza di un mese si verifica l'exitus. All'esame macroscopico, l'encefalo ha un aspetto diffusamente congesto. L'esame istologico rileva infiltrati linfomonocitari perivasali e diffusi, aree di demielinizzazione, astrociti a nucleo bizzarro ed oligodendrociti con nucleo grandemente dilatato da un'inclusione ipercromatica. L'esame ultrastrutturale conferma la presenza di corpi virali intranucleari di forma icosaedrica. Trattasi di una leucoencefalopatia multifocale progressiva. Un solo caso analogo, osservato in corso di terapia con ciclosporina per una granulomatosi di Wegener, ci risulta essere stato segnalato in letteratura.
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Gentile, S., Sacerdote, I., Roccatello, D. et al. Progressive multifocal leukoencephalopathy during cyclosporine treatment. A case report. Ital J Neuro Sci 17, 363–366 (1996). https://doi.org/10.1007/BF01999899
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DOI: https://doi.org/10.1007/BF01999899