Clinical and MRI improvement in a case of progressive multifocal leukoencephalopathy
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Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating CNS disease due to lytic infection of oligodendrocytes by the John Cunningham polyomavirus (JCV), an opportunistic agent requiring immunosuppression to become active, commonly complicating HIV infections, lymphoproliferative disorders or immunosuppressive therapies . PML has been reported in immune-mediated rheumatologic disorders, such as systemic lupus erythematosus (SLE). Currently, no therapies have been approved , but many broad-spectrum nucleoside-analogue chemotherapeutics have been tested to inhibit JCV replication. Among these, cidofovir, an acyclic nucleotide-phosphonate analogue of deoxycytosine-monophosphate, has been employed with various outcomes, alone or in combination with mirtazapine, an agonist of serotonin-receptor-2A (5HT2AR) blocking the JCV entry into glial cells. Although the restoration of host adaptive-immune response is the first purpose of PML therapy, a...
KeywordsSystemic Lupus Erythematosus Systemic Lupus Erythematosus Patient Progressive Multifocal Leukoencephalopathy Mirtazapine Natalizumab
Authors gratefully thank Doctor Daniele Giuseppe Romano for his help in the brain MRI evaluation.
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Conflict of interest
None. All authors have approved the final article. Patient’s anonymity was protected in this case report.