Progressive multifocal leukoencephalopathy, a review and an extended report of five patients with different immune compromised states

• Published in: European journal of internal medicine 2009-05, Vol.20 (3), p.261-267
• Main Authors: Epker, Jelle L, van Biezen, Paula, van Daele, Paul L.A, van Gelder, Teun, Vossen, Ann, van Saase, Jan L.C.M
• Format: Article
• Language: English
• Subjects: Aged; Antiviral Agents - therapeutic use; Cytosine - analogs & derivatives; Cytosine - therapeutic use; Female; GS-504 cidifovir; Humans; HWA-486 leflunomide; Immunocompromised Host; Immunosuppressive Agents - therapeutic use; Internal Medicine; Isoxazoles - therapeutic use; JC polyomavirus; Leukoencephalopathy, Progressive Multifocal - drug therapy; Leukoencephalopathy, Progressive Multifocal - immunology; Male; Middle Aged; Organophosphonates - therapeutic use; Progressive multifocal leukoencephalopathy
• ISSN: 0953-6205; 1879-0828
• DOI: 10.1016/j.ejim.2008.07.032

Abstract Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection of the brain caused by the JC-virus. Both a decreased cellular or humoral immune response can increase the susceptibility for JC-virus induced PML. Not only HIV infected people are at risk, a wide range of otherwise immune compromised patients are a potential target for this virus. This report of five PML patients shows the importance of a clinician's familiarity with this disease and it's presenting symptoms. The presenting symptoms of PML can sometimes mimic worsening of the underlying disease. Although different therapeutic strategies have been tried, the outcomes remain very poor. In this series, treatment with cidofovir appears not to be effective in treating PML, neither in HIV positive nor HIV negative patients. Experimental therapy with leflunomide, after tapering of the immunosuppressive medication, did change the natural course of PML in one patient.