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Progressive multifocal leukoencephalopathy and spectrum of predisposing conditions: a 20-year retrospective cohort study in a tertiary center in São Paulo, Brazil.
Mari, Julia Ferreira; de Miranda, Érique José Farias Peixoto; Mendes-Correa, Maria Cassia; Chow, Felicia C; Vidal, José Ernesto.
Afiliación
  • Mari JF; Departamento de Moléstias Infecciosas e Parasitárias, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
  • de Miranda ÉJFP; Centro de Ensaios Clínicos E Farmacovigilância, Instituto Butantan, São Paulo, Brazil.
  • Mendes-Correa MC; Laboratório de Investigação Médica (LIM 52) do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
  • Chow FC; Weill Institute for Neurosciences, Department of Neurology, University of California, San Francisco, CA, USA.
  • Vidal JE; Department of Medicine (Infectious Diseases), University of California, San Francisco, CA, USA.
Neurol Sci ; 2024 Jul 15.
Article en En | MEDLINE | ID: mdl-39007964
ABSTRACT

BACKGROUND:

Epidemiological studies on predisposing conditions and outcomes of progressive multifocal leukoencephalopathy (PML) cases have been carried out exclusively in high-income countries. We aim to report and compare the main characteristics and outcomes of patients with PML and several underlying diseases in a referral center in a middle-income country.

METHODS:

We performed a retrospective cohort study of PML cases admitted to a tertiary care hospital in São Paulo, Brazil during 2000-2022. Demographic and PML-specific variables were recorded. One-year case-fatality rate and factors associated with death were identified using a multivariate Cox proportional hazards regression model.

RESULTS:

Ninety-nine patients with PML were included. HIV infection (84.8%) and malignancy (14.1%) were the most prevalent underlying conditions. Other predisposing diseases were autoimmune/inflammatory diseases (5.1%) and solid organ transplantation (1.0%). One (1.0%) patient had liver cirrhosis and another (1.0%) patient was previously healthy. Focal motor deficits (64.2%) and gait instability (55.1%) were the most common signs. The one-year case-fatality rate was 52.5% (95% CI 42.2-62.7). The one-year case-fatality rate (95% CI) in patients with or without malignancy (85.7%, 95% CI 57.2-98.2% and 47.1%, 95% CI 36.1-58.2%, respectively) were statistically different (P = 0.009). Crude and adjusted Cox regression models identified malignancy as independently associated with death (adjusted HR = 3.92, 95% CI 1.76-8.73, P = 0.001).

CONCLUSIONS:

HIV/AIDS was the predisposing condition in 84.8% of PML cases. The one-year case-fatality rate was 52.5% and having a malignancy was independently associated with death. This study reports emerging data on the epidemiology and outcome of PML in a middle-income country.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE País/Región como asunto: America do sul / Brasil Idioma: En Revista: Neurol Sci Asunto de la revista: NEUROLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Italia

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE País/Región como asunto: America do sul / Brasil Idioma: En Revista: Neurol Sci Asunto de la revista: NEUROLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Italia