Progressive multifocal leukoencephalopathy (PML) and PML immune reconstitution inflammatory syndrome (PML-IRIS) can be a devastating neurologic process associated with HIV, but limited knowledge on their characteristics in the established antiretroviral (ART) era is available. We conducted a case series to evaluate the clinical course of PML and PML-IRIS at our urban safety-net hospital in Atlanta, GA.
All HIV-positive individuals with a positive JCV DNA PCR in the spinal fluid between 5/1/2013 to 6/1/2017 were identified through electronic medical records (EMR) query. Demographics, symptom presentation, laboratory data, imaging results, treatment, and outcomes were abstracted from the EMR. PML and PML-IRIS were defined using the American Association of Neurology criteria.
There were 26 patients included in this study, 15 (58%) HIV-positive patients with PML and 11 (42%) with PML-IRIS (2 with an unmasking presentation and 9 with a paradoxical presentation). The average age was 45 years, 23 (88%) were black, and 20 (77%) were male. Mean CD4 and HIV viral load were 65 cells/µL and 4.11 log10 copies/mL, respectively. The most common presenting symptoms were motor weakness (18, 69%), cognitive deficits (15, 58%), and dysarthria (11, 42%). Twenty-four (92%) patients had white matter changes on magnetic resonance imaging (MRI). Enhancement on MRI and presentation with ataxia, dysarthria, or motor or visual deficits were found to be associated with PML-IRIS. Eleven (42%) patients were on ART at the time of diagnosis, and 24 (92%) of patients were on ART afterward. Corticosteroids were used in 9 patients with PML-IRIS and in 3 with PML. Maraviroc was used in 3 patients with PML-IRIS. Presenting with speech deficits or visual changes, having edema on MRI, and developing PML-IRIS were each positively associated with progression to hospice or withdrawal of care, although these values were not statistically significant. Outcomes were dismal with 7/15 (46.7%) patients with PML and 9/11 (81.8%) with PML-IRIS dying or being referred to hospice.
Despite widespread access to ART, patients with PML continue to have poor outcomes, particularly among those who develop PML-IRIS. More research is needed to understand the risks for and prevention of PML-IRIS.
W. Armstrong, None
V. Marconi, ViiV: Investigator , Research support and Salary . Bayer: Investigator , Research support . Gilead: Investigator , Research support .
M. L. Nguyen, None
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