1. The Innate Immune Response Characterizes Posterior Reversible Encephalopathy Syndrome
- Author
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Jens Minnerup, Christopher Nelke, Tobias Ruck, Catharina C. Gross, Marc Pawlitzki, Sven G. Meuth, Andreas Schulte-Mecklenbeck, Saskia Räuber, Heinz Wiendl, and Leoni Rolfes
- Subjects
Adult ,CD4-Positive T-Lymphocytes ,Male ,0301 basic medicine ,posterior reversible encephalopathy syndrome ,CD14 ,Immunology ,Lipopolysaccharide Receptors ,Adaptive Immunity ,CD8-Positive T-Lymphocytes ,CD16 ,Monocytes ,PRES ,03 medical and health sciences ,immune cells ,0302 clinical medicine ,Immune system ,medicine ,Humans ,Immunology and Allergy ,Retrospective Studies ,Innate immune system ,business.industry ,flow cytometry ,Multiple sclerosis ,Progressive multifocal leukoencephalopathy ,Receptors, IgG ,Leukoencephalopathy, Progressive Multifocal ,Posterior reversible encephalopathy syndrome ,Middle Aged ,medicine.disease ,Acquired immune system ,Immunity, Innate ,030104 developmental biology ,Female ,Original Article ,Posterior Leukoencephalopathy Syndrome ,business ,030217 neurology & neurosurgery - Abstract
While posterior reversible encephalopathy syndrome (PRES) is often characterized by an inflammatory cerebrospinal-fluid (CSF) profile, knowledge of immune cell patterns in PRES is lacking. Thus, we retrospectively characterized CSF and peripheral blood (PB) from 15 PRES patients, which we analyzed by multidimensional flow cytometry (FC). Results were compared to 72 controls, as well as to 9 patients with progressive multifocal leukoencephalopathy (PML, as a relevant differential diagnosis) and 15 multiple sclerosis patients (MS, as a classical neuroinflammatory disorder), respectively. Total protein level in CSF from PRES patients was elevated compared to that in controls, but not to MS and PML. In-depth FC analysis revealed no differences for adaptive immune cells (B cells, plasma cells, CD4+, and CD8+ T cells) in PB or CSF of PRES compared to controls. In contrast, we observed alterations of the adaptive immune response in CSF of PML and MS compared to PRES, indicating that the adaptive immune response is not a driver of disease in PRES. Indeed, PRES was characterized by an innate immune response with CD14++/CD16+ (intermediate) monocytes elevated in PB and CSF, while CD14++/CD16− (classical) monocytes were decreased in PB from PRES patients as compared to controls. Levels of CD14++/CD16+ monocytes correlated with the duration of hospital stay as a surrogate marker for disease severity in PRES patients. Our findings argue for a role of innate rather than adaptive immunity in the pathophysiology of PRES. The observed shift in monocyte subsets might provide valuable diagnostic clues for the clinical management of these patients.
- Published
- 2021