Progressive multifocal leukoencephalopathy and spectrum of predisposing conditions: a 20-year retrospective cohort study in a tertiary center in São Paulo, Brazil.

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Title: Progressive multifocal leukoencephalopathy and spectrum of predisposing conditions: a 20-year retrospective cohort study in a tertiary center in São Paulo, Brazil.
Authors: Mari, Julia Ferreira (AUTHOR), de Miranda, Érique José Farias Peixoto (AUTHOR), Mendes-Correa, Maria Cassia (AUTHOR), Chow, Felicia C. (AUTHOR), Vidal, José Ernesto (AUTHOR)
Source: Neurological Sciences. Jan2025, Vol. 46 Issue 1, p365-379. 15p.
Subjects: Progressive multifocal leukoencephalopathy, Proportional hazards models, John Cunningham virus, HIV infections, Medical sciences
Geographic Terms: Brazil
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. [ABSTRACT FROM AUTHOR]
Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: Progressive multifocal leukoencephalopathy and spectrum of predisposing conditions: a 20-year retrospective cohort study in a tertiary center in São Paulo, Brazil.
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  Data: <searchLink fieldCode="AR" term="%22Mari%2C+Julia+Ferreira%22">Mari, Julia Ferreira</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22de+Miranda%2C+Érique+José+Farias+Peixoto%22">de Miranda, Érique José Farias Peixoto</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Mendes-Correa%2C+Maria+Cassia%22">Mendes-Correa, Maria Cassia</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Chow%2C+Felicia+C%2E%22">Chow, Felicia C.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Vidal%2C+José+Ernesto%22">Vidal, José Ernesto</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22Neurological+Sciences%22">Neurological Sciences</searchLink>. Jan2025, Vol. 46 Issue 1, p365-379. 15p.
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  Data: <searchLink fieldCode="DE" term="%22Progressive+multifocal+leukoencephalopathy%22">Progressive multifocal leukoencephalopathy</searchLink><br /><searchLink fieldCode="DE" term="%22Proportional+hazards+models%22">Proportional hazards models</searchLink><br /><searchLink fieldCode="DE" term="%22John+Cunningham+virus%22">John Cunningham virus</searchLink><br /><searchLink fieldCode="DE" term="%22HIV+infections%22">HIV infections</searchLink><br /><searchLink fieldCode="DE" term="%22Medical+sciences%22">Medical sciences</searchLink>
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  Data: <searchLink fieldCode="DE" term="%22Brazil%22">Brazil</searchLink>
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  Data: 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. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of Neurological Sciences is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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      – TitleFull: Progressive multifocal leukoencephalopathy and spectrum of predisposing conditions: a 20-year retrospective cohort study in a tertiary center in São Paulo, Brazil.
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              Text: Jan2025
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