Progressive Multifocal Leukoencephalopathy with Immune Reconstitution Inflammatory Syndrome (PML-IRIS): Two Case Reports of Successful Treatment with Mefloquine and a Review of the Literature

TL;DRAbstract

Failure of cell-mediated immunity allows reactivation of the 'JC' polyomavirus (JCV) in oligodendrocytes, and the development of progressive multifocal leukoencephalopathy (PML). This is most commonly associated with advanced human immunodefi ciency virus (HIV) infection, but is also described after biological therapies such as natalizumab, and rarely other conditions. The development of antiretroviral therapy (ART) has greatly improved this prognosis. In the Swiss Cohort Study, incidence fell 4-fold and attributable 1-year mortality fell from 60% to 20%. 2 Despite these advances, PML still has one of the highest mortality rates among acquired immune defi ciency syndrome (AIDS) defi ning illnesses. A rapid early mortality is observed, and less than half of survivors are expected to recover neurologically. While re-establishment of the immune system is important for successful outcomes, it can trigger an acute infl ammatory response to JCV or host antigens. This immune reconstitution in

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