Progressive multifocal leukoencephalopathy, a review and an extended report of five patients with different immune compromised states.
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.
Epker
Jelle L
JL
Erasmus Medical Centre, Department of Intensive Care Medicine, The Netherlands. j.epker@erasmusmc.nl
van Biezen
Paula
P
van Daele
Paul L A
PL
van Gelder
Teun
T
Vossen
Ann
A
van Saase
Jan L C M
JL
D000368:Aged; D000998:Antiviral Agents / Q000627:therapeutic use*; D000077404:Cidofovir; D003596:Cytosine / Q000031:analogs & derivatives / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007555:Isoxazoles / Q000627:therapeutic use*; D000077339:Leflunomide; D007968:Leukoencephalopathy, Progressive Multifocal / Q000188:drug therapy* / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D063065:Organophosphonates / Q000627:therapeutic use
D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D007555:Isoxazoles; D063065:Organophosphonates; D003596:Cytosine; D000077339:Leflunomide; D000077404:Cidofovir