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Abstract
Susac syndrome is an inflammatory pathology of the brain endothelium. Here the authors show that the pathology is driven by CD8 T cells attacking the endothelium, and that blocking T cell-endothelial adhesion ameliorates the disease in a mouse model, and associates with improved clinical score in 4 patients.
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1 Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster, Münster, Germany
2 Centre de Physiopathologie Toulouse-Purpan (CPTP), Université de Toulouse, CNRS, Inserm, UPS, CHU Purpan – BP 3028 – 31024, Toulouse, France
3 Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster, Münster, Germany; Department of Neurology, University Hospital Knappschaftskrankenhaus Bochum, Ruhr University Bochum, Bochum, Germany
4 Department of Neuroimmunology, Center for Brain Research, Medical University of Vienna, Vienna, Austria
5 Institute of Neuropathology, University Hospital Münster, University of Münster, Münster, Germany
6 Institute of Experimental and Clinical Pharmacology and Toxicology, University of Lübeck, Lübeck, Germany
7 Institute of Neuropathology, University Medical Center Göttingen, Göttingen, Germany
8 UMR 1064, INSERM, Centre de Recherche en Transplantation et Immunologie, Université de Nantes, CHU Nantes - Hôtel Dieu Bd Jean Monnet, Nantes Cedex 01, France; Service Neurologie, CHU Nantes, Nantes, France
9 Service d’Anatomo-Pathologie, CHU Nantes, Hôtel-Dieu, rez-de-jardin, Nantes Cedex 1, France
10 Department of Neurology, Royal North Shore Hospital, Sydney, Australia; Australia Northern Clinical School, University of Sydney, Sydney, NSW, Australia
11 Brain and Mind Centre, Medical Faculty, University of Sydney, Sydney, NSW, Australia
12 Brain and Mind Centre, Medical Faculty, University of Sydney, Sydney, NSW, Australia; Department of Neuropathology, Royal Prince Alfred Hospital, 94, Sydney, NSW, Australia
13 Brain and Mind Centre, Medical Faculty, University of Sydney, Sydney, NSW, Australia; Department of Neurology, Concord Hospital, University of Sydney, Sydney, NSW, Australia
14 Department of Neurology, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany; Department of Neurology, Center of Neurology und Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf, Düsseldorf, Germany
15 Max Delbrueck Center for Molecular Medicine and Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, NeuroCure, Experimental and Clinical Research Center, Berlin, Germany
16 Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany
17 Department of Neurology, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany; Department of Neurology, Alfried Krupp Hospital, Essen, Germany
18 Institute of Neurology, Medical University of Vienna, Vienna, Austria
19 Institute of Clinical Neuroimmunology, Biomedical Center and Hospital of the Ludwig-Maximilians-University Munich, Munich, Germany
20 Institute of Clinical Neuroimmunology, Biomedical Center and Hospital of the Ludwig-Maximilians-University Munich, Munich, Germany; Munich Cluster for Systems Neurology (SyNergy), Munich, Germany
21 Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster, Münster, Germany; Cells in Motion (CiM), Münster, Germany
22 Centre de Physiopathologie Toulouse-Purpan (CPTP), Université de Toulouse, CNRS, Inserm, UPS, CHU Purpan – BP 3028 – 31024, Toulouse, France; Department of Infectious and Tropical Diseases, Toulouse University Hospital, Toulouse, France
23 Department of Neurology with Institute of Translational Neurology, University Hospital Münster, University of Münster, Münster, Germany; Australia Northern Clinical School, University of Sydney, Sydney, NSW, Australia; Cells in Motion (CiM), Münster, Germany