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.
Similar content being viewed by others
References
Astrom K., Mancall E.L., Richardson E.P.:Progressive multifocal leukoencephalopathy: a hitherto unrecognized complication of chronic lymphatic leukemia and Hodgkin's disease. Brain, 88: 93–111, 1958.
Casato M., Bonomo L., Caccavo D., Giorgi A.:Clinical effects of cyclosporine in dermatomyositis. Clin. Exp. Dermatolog. 15: 121–123, 1990.
Cherin P., Herson S., Wechsler B. et al.:Efficacy of intravenous gammaglobulin therapy in chronic refractory polymyositis and dermatomyositis: an open study with 20 adult patients. Am. J. Med. 91: 162–168, 1991.
De Groen P.C., Aksamit A.J., Rakela J. et al.:Central nervous system toxicity after liver transplantation. N. Engl. J. Med. 317: 861–866, 1989.
Ettinger J., Feiden W., Hubner G., Schreiner M.:Progressive multifokale Leukoenzephalopathie bei Wegener'scher Granulomatose unter Therapie mit Cyclosporine A. Klin. Wochenschr. 67: 260–264, 1989.
Familio L., Racusen L., Firush B. et al.:Central nervous system toxicity of cyclosporine in a rat model. Transplantation, 48: 316–321, 1989.
Faulds D., Goa K.L., Benfield P.:Cyclosporine. A review of its pharmacodynamic and pharmacokinetic properties and therapeutic use in immunoregulatory disorders. Drug, 45: 953–1040, 1993.
Gelfand E.W.:The use of intravenous immunoglobulin in collagen vascular disorders: A potentially new modality of therapy. J. Allerg. Clin. Immunol. 84: 613–615, 1989.
Geilleux M.H., Steiner R.E., Young I.R.:MR imaging in progressive multifocal leukoencephalopathy. Am. J. Neuroradiol. 7: 1033–1035, 1986.
Jann S., Beretta S., Moggio M. et al.:High-dose intravenous human immunoglobulin in polymyositis resistant to treatment. J. Neurol. Neurosurg. Psychiatry 55: 60–62, 1992.
Loveras J.J., Larrue V., Suc E. et al.:Leukoencephalopathy after cyclosporine in a liver transplant. Clin. Transplant. 4: 58–62, 1990.
Lueck C.J.H., Trend P., Swash M.:Cyclosporin in the management of polymyositis and dermatomyositis. J. Neurol. Neurosurg. Psychiatry, 54: 1007–1008, 1991.
Mabin D., Fourquet I., Richard P. et al.:Leucoencephalopathie regressive au cours d'un surdosage en cyclosporine A. Rev. Neurol. 149: 576–578, 1993.
Padgett B.L., Walker D.L., ZuRhein G.M., Eckroade R.J.:Cultivation of papova-like virus from human brain with progressive multifocal leukoencephalopathy. Lancet, 1: 1257–1260, 1971.
Palmer B.F., Toto R.D.:Severe neurologic toxicity in duced by cyclosporine A in three renal transplant patients. Am. J. Kidney Dis. 18: 116–121, 1991.
Shah D., Rylance P.B., Rogerson M.E. et al.:Generalized epileptic fits in renal transplant recipients given cyclosporine A. Br. Med. J. 289: 1347–1348, 1984.
Sigurgeirsson B., Lindelof B., Edhag OP., Allander E.:Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study. N. Engl. J. Med. 326: 363–367, 1992.
Silverman I., Rubinstein L.J.:Electron microscopic observations in a case of progressive multifocal leukoencephalopathy. Acta Neuropathol. 5: 215–224, 1965.
Truwit C.L., Denaro C.P., Lake J.R., De Marco T.:MR imaging of reversible cyclosporine A induced neurotoxicity. Am. J. Neuroradiol. 12: 651–659, 1991.
Vogt D.P., Lederman R.J., Carey W.D., Broughan T.A.:Neurologic complications of liver transplantation. Transplantation, 45: 1056–1061, 1988.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
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
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01999899