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. 2019 Jun;35(6):544-552.
doi: 10.1089/AID.2018.0232. Epub 2019 Apr 3.

Not a Disease of the Past: A Case Series of Progressive Multifocal Leukoencephalopathy in the Established Antiretroviral Era

Nathan A Summers  1   2 , Colleen F Kelley  1   2   3 , Wendy Armstrong  1   2 , Vincent C Marconi  1   2   4 , Minh Ly Nguyen  1   2
Affiliations

Not a Disease of the Past: A Case Series of Progressive Multifocal Leukoencephalopathy in the Established Antiretroviral Era

Nathan A Summers et al. AIDS Res Hum Retroviruses. 2019 Jun.

Abstract

Progressive multifocal leukoencephalopathy (PML) and PML immune reconstitution inflammatory syndrome (PML-IRIS) can be devastating neurological processes associated with HIV, but limited knowledge of their characteristics in the established antiretroviral therapy (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 John Cunningham virus DNA polymerase chain reaction in the spinal fluid between May 1, 2013 to June 1, 2017 were identified from the electronic health records (EHRs) using the HIV Disease Registry. Demographics, symptom presentation, laboratory data, imaging results, treatment, and outcomes were abstracted from the EHR. PML and PML-IRIS were defined using the American Association of Neurology criteria. Of the 32 individuals identified, 6 (19%) were felt to have asymptomatic positive results. Of the remainder, 15 (58%) HIV-positive patients had PML and 11 (42%) PML-IRIS (2 with an unmasking presentation and 9 with a paradoxical presentation). The most common presenting symptoms were motor weakness (18, 69%), cognitive deficits (15, 58%), and dysarthria (11, 42%). Corticosteroids were used in 12 patients and maraviroc in 3 patients. Outcomes were dismal with 7 (47%) patients with PML and 9 (82%) with PML-IRIS dying or being referred to hospice, with median survival times of 266 days in the PML group and 109 days in the PML-IRIS group. 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.

Keywords: HIV; IRIS; JCV; PML; immune reconstitution inflammatory syndrome.

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Conflict of interest statement

No competing financial incentives exist.

Figures

<b>FIG. 1.</b>
FIG. 1.
Study design. CFAR, Center for AIDS Research; CSF, cerebrospinal fluid; IRIS, immune reconstitution inflammatory syndrome; JCV, John Cunningham virus; PCR, polymerase chain reaction; PML, progressive multifocal leukoencephalopathy.
<b>FIG. 2.</b>
FIG. 2.
Kaplan–Meier curve comparing survival of PML versus PML-IRIS patients. Log-Rank p value: .05. Survtime, survival time. Color images are available online.
See this image and copyright information in PMC

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