TY  - JOUR
A1  - Zeiner, Pia Susan
A1  - Mann, Leonhard
A1  - Filipski, Katharina Johanna
A1  - Starzetz, Tatjana
A1  - Forster, Marie-Thérèse
A1  - Ronellenfitsch, Michael Wilfried
A1  - Steinbach, Joachim Peter
A1  - Mittelbronn, Michel Guy André
A1  - Wagner, Marlies
A1  - Harter, Patrick Nikolaus
T1  - Immune profile and radiological characteristics of progressive multifocal leukoencephalopathy
T2  - European journal of neurology
N2  - Background and purpose: Progressive multifocal leukoencephalopathy (PML) constitutes a severe disease with increasing incidence, mostly in the context of immunosuppressive therapies. A detailed understanding of immune response in PML appears critical for the treatment strategy. The aim was a comprehensive immunoprofiling and radiological characterization of magnetic resonance imaging (MRI) defined PML variants.
Methods: All biopsy-confirmed PML patients (n = 15) treated in our department between January 2004 and July 2019 were retrospectively analysed. Data from MRI, histology as well as detailed clinical and outcome data were collected. The MRI-defined variants of classical (cPML) and inflammatory (iPML) PML were discriminated based on the intensity of gadolinium enhancement. In these PML variants, intensity and localization (perivascular vs. parenchymal) of inflammation in MRI and histology as well as the cellular composition by immunohistochemistry were assessed. The size of the demyelinating lesions was correlated with immune cell infiltration.
Results: Patients with MRI-defined iPML showed a stronger intensity of inflammation with an increased lymphocyte infiltration on histological level. Also, iPML was characterized by a predominantly perivascular inflammation. However, cPML patients also demonstrated certain inflammatory tissue alterations. Infiltration of CD163-positive microglia and macrophage (M/M) subtypes correlated with PML lesion size.
Conclusions: The non-invasive MRI-based discrimination of PML variants allows for an estimation of inflammatory tissue alterations, although exhibiting limitations in MRI-defined cPML. The association of a distinct phagocytic M/M subtype with the extent of demyelination might reflect disease progression.
KW  - biomarker
KW  - immune response
KW  - MRI
KW  - neuroimaging
KW  - PML
Y1  - 2021
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/75066
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-750666
SN  - 1468-1331
VL  - 29
IS  - 2
SP  - 543
EP  - 554
PB  - Wiley-Blackwell
CY  - Oxford [u.a.]
ER  -