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Published on: November 19, 2020
Post-cART progressive multifocal leukoencephalopathy era in a Brazilian center
Marco A Lima1, Marcus Tulius T Silva2, Livia A Afonso3
1Laboratory for Clinical Research in Neuroinfection, National Institute of Infectious Diseases Evandro Chagas, FIOCRUZ, Rio de Janeiro, Brazil; Neurology Service, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is lytic infection of oligodendrocytes caused by JC virus (JCV). While PML incidence in developing countries has decreased after the introduction of combination antiretroviral therapy (cART), data in developing countries is scarce and limited to few cohorts. We described the epidemiological and clinical profile of a group of Brazilian HIV infected patients with PML in the cART era. A total of 27 patients were included in the study. The median age at PML onset was 42years (range: 27-67years) and 18 (66.7%) were men. The median CD4+ T cell count at the time of diagnosis was 67cells/mm3 and the median HIV viral load was 27,000copies/ml. Motor deficits were the most common early manifestations (44%). Seizures occurred in 37% of the patients and 9 (33.3%) had PML associated with immune reconstitution inflammatory syndrome (IRIS). Mortality was 33% and lower age at PML onset was associated with survival (p: 0.013). Our results are in accordance with previous published series of PML cases. Factors such as genetic background, regional JCV subtype differences, death from other diseases and underdiagnosis may explain the low prevalence of reported PML cases in developing countries.
Insights
Progressive multifocal leukoencephalopathy (PML) in Brazilian HIV patients is characterized by specific clinical features and a 33% mortality rate. Lower age at onset correlated with improved survival during the combination antiretroviral therapy era.
Area of Science:
- Neurovirology
- Infectious Diseases
- Epidemiology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a JC virus (JCV) infection affecting oligodendrocytes.
- PML incidence has declined with combination antiretroviral therapy (cART), but data from developing nations remains limited.
- Understanding PML epidemiology in diverse settings is crucial for patient management.
Purpose of the Study:
- To describe the epidemiological and clinical characteristics of PML in Brazilian HIV-infected patients.
- To analyze outcomes and survival factors in PML patients during the cART era.
- To compare findings with international PML case series.
Main Methods:
- Retrospective analysis of 27 HIV-infected Brazilian patients diagnosed with PML.
- Data collection included demographics, CD4+ T cell counts, HIV viral load, clinical manifestations, and outcomes.
- Statistical analysis to identify factors associated with survival.
Main Results:
- The study included 27 patients (66.7% male), median age 42 years, median CD4+ 67 cells/mm³, and median HIV viral load 27,000 copies/ml.
- Motor deficits (44%) and seizures (37%) were common; 33.3% experienced PML-associated immune reconstitution inflammatory syndrome (IRIS).
- Mortality was 33%, with younger age at PML onset significantly associated with survival (p=0.013).
Conclusions:
- PML in Brazilian HIV patients presents similarly to other cohorts, with significant morbidity and mortality.
- Lower age at onset is a positive prognostic factor for survival in the cART era.
- Genetic background, JCV subtypes, and underdiagnosis may influence reported PML prevalence in developing countries.
