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Inflammatory progressive multifocal leukoencephalopathy in human immunodeficiency virus-negative patients
DeRen Huang1, Michael Cossoy, Meizhang Li
1Department of Neurology, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA. huangd2@ccf.org
Objective:
Inflammatory progressive multifocal leukoencephalopathy (iPML) with enhancing magnetic resonance imaging (MRI) lesions and leukocyte infiltration occurs in human immunodeficiency virus (HIV)-infected individuals after highly active antiretroviral therapy (HAART) treatment. MRI diagnostic criteria for PML suggest that iPML does not occur in HIV-negative individuals.
Methods:
We studied pathologically proved PML (12 by biopsy, 9 with MRI, 32 at autopsy).
Results:
HIV-negative (2/5) and -positive (2/4) PML patients had enhancing MRI lesions, correlated with CD3(+) lymphocyte infiltration. Inflammatory infiltrates occurred in the majority of HIV-negative (7/8) and HIV-positive/HAART (17/20) cases (p > 0.2), but in only 2 of 16 HIV-positive/non-HAART cases (p < 0.001).
Interpretation:
iPML showed radiographic and pathological similarity in HIV-positive/HAART and HIV-negative patients. HIV-negative iPML necessitates further consideration of MRI criteria for PML.
Insights
Inflammatory progressive multifocal leukoencephalopathy (iPML) with enhancing MRI lesions occurs in HIV-positive patients on HAART and HIV-negative individuals. This suggests current MRI criteria for PML may need revision.
Area of Science:
- Neurology
- Infectious Diseases
- Radiology
Background:
- Progressive multifocal leukoencephalopathy (PML) with enhancing magnetic resonance imaging (MRI) lesions and leukocyte infiltration is observed in human immunodeficiency virus (HIV)-infected individuals post-highly active antiretroviral therapy (HAART).
- Current MRI diagnostic criteria for PML suggest iPML is exclusive to HIV-positive individuals.
Purpose of the Study:
- To investigate the occurrence and characteristics of iPML in HIV-negative individuals compared to HIV-positive individuals on HAART.
- To evaluate the diagnostic criteria for PML based on MRI findings.
Main Methods:
- Pathologically confirmed cases of PML were analyzed, including those diagnosed via biopsy, MRI, and autopsy.
- Enhancing MRI lesions and CD3(+) lymphocyte infiltration were assessed in both HIV-negative and HIV-positive patients.
Main Results:
- Enhancing MRI lesions, indicative of iPML, were found in both HIV-negative and HIV-positive patients, correlating with CD3(+) lymphocyte infiltration.
- Inflammatory infiltrates were prevalent in most HIV-negative and HIV-positive/HAART cases, but rare in HIV-positive/non-HAART cases.
Conclusions:
- Inflammatory PML (iPML) exhibits radiographic and pathological similarities between HIV-positive patients on HAART and HIV-negative individuals.
- The findings challenge existing MRI criteria for PML, indicating a need for re-evaluation to include HIV-negative cases of iPML.
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