Biomarkers of Neurodegeneration in Autoimmune-Mediated Encephalitis
Progranulin (PGRN), Total-Tau (t-tau), and Neurofilament light chain (NfL) are well known biomarkers of neurodegeneration. The objective of the present study was to investigate whether these parameters represent also biomarkers in autoimmune-mediated Encephalitis (AE) and may give us insights into t...
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| Format: | Article |
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Frontiers Media S.A.
2018-09-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fneur.2018.00668/full |
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| author | Peter Körtvelyessy Peter Körtvelyessy Peter Körtvelyessy Peter Körtvelyessy Harald Prüss Harald Prüss Lorenz Thurner Walter Maetzler Walter Maetzler Walter Maetzler Deborah Vittore-Welliong Jörg Schultze-Amberger Hans-Jochen Heinze Hans-Jochen Heinze Hans-Jochen Heinze Dirk Reinhold Frank Leypoldt Stephan Schreiber Daniel Bittner Daniel Bittner |
| author_facet | Peter Körtvelyessy Peter Körtvelyessy Peter Körtvelyessy Peter Körtvelyessy Harald Prüss Harald Prüss Lorenz Thurner Walter Maetzler Walter Maetzler Walter Maetzler Deborah Vittore-Welliong Jörg Schultze-Amberger Hans-Jochen Heinze Hans-Jochen Heinze Hans-Jochen Heinze Dirk Reinhold Frank Leypoldt Stephan Schreiber Daniel Bittner Daniel Bittner |
| author_sort | Peter Körtvelyessy |
| collection | DOAJ |
| description | Progranulin (PGRN), Total-Tau (t-tau), and Neurofilament light chain (NfL) are well known biomarkers of neurodegeneration. The objective of the present study was to investigate whether these parameters represent also biomarkers in autoimmune-mediated Encephalitis (AE) and may give us insights into the pathomechanisms of AE. We retrospectively examined the concentration of PGRN in the cerebrospinal fluid (CSF) and serum of 38 patients suffering from AE in acute phase and/or under treatment. This AE cohort comprises patients with autoantibodies against: NMDAR (n = 18 patients), Caspr2 (n = 8), Lgi-1 (n = 10), GABAB(R) (n = 1), and AMPAR (n = 1). Additionally, the concentrations of NfL (n = 25) and t-tau (n = 13) in CSF were measured when possible. Follow up data including MRI were available in 13 patients. Several age-matched cohorts with neurological diseases besides neuroinflammation or neurodegeneration served as control groups. We observed that PGRN was significantly elevated in the CSF of patients with NMDAR-AE in the acute phase, but normalized at follow up under treatment (p < 0.01). In the CSF of other patients with AE PGRN was in the range of the CSF levels of control groups. T-tau was highly elevated in the CSF of patients with temporal FLAIR-signal in the MRI and in patients developing a hippocampal sclerosis. NfL was exceptionally high initially in Patients with AE with a paraneoplastic or parainfectious cause and also normalized under treatment. The normalizations of all biomarkers were mirrored in an improvement on the modified Rankin scale. The data suggest that the concentration of PGRN in CSF might be a biomarker for acute NMDAR-AE. Pathological high t-tau levels may indicate a risk for hippocampal sclerosis. The biomarker properties of NfL remain unclear since the levels decrease under treatment, but it could not predict severity of disease in this small cohort. According to our results, we recommend to measure in clinical practice PGRN and t-tau in the CSF of patients with AE. |
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| spelling | doaj.art-3e0c2624f809499dad3490264dd7c9542022-12-22T00:13:58ZengFrontiers Media S.A.Frontiers in Neurology1664-22952018-09-01910.3389/fneur.2018.00668393934Biomarkers of Neurodegeneration in Autoimmune-Mediated EncephalitisPeter Körtvelyessy0Peter Körtvelyessy1Peter Körtvelyessy2Peter Körtvelyessy3Harald Prüss4Harald Prüss5Lorenz Thurner6Walter Maetzler7Walter Maetzler8Walter Maetzler9Deborah Vittore-Welliong10Jörg Schultze-Amberger11Hans-Jochen Heinze12Hans-Jochen Heinze13Hans-Jochen Heinze14Dirk Reinhold15Frank Leypoldt16Stephan Schreiber17Daniel Bittner18Daniel Bittner19Department of Neurology, University Hospital Magdeburg, Magdeburg, GermanyGerman Center for Neurodegenerative Diseases Magdeburg, Magdeburg, GermanyDepartment of Neurology, Charité-Universitätsmedizin Berlin, Berlin, GermanyGerman Center for Neurodegenerative Diseases Berlin, Berlin, GermanyDepartment of Neurology, Charité-Universitätsmedizin Berlin, Berlin, GermanyGerman Center for Neurodegenerative Diseases Berlin, Berlin, GermanyJosé Carreras Center for Immuno- and Gene Therapy and Internal Medicine I, Saarland University Medical School, Homburg, GermanyDepartment of Neurodegeneration, Hertie Institute for Clinical Brain Research (HIH), University of Tübingen, Tübingen, GermanyGerman Center for Neurodegenerative Diseases Tübingen, Tübingen, GermanyDepartment of Neurology, University Hospital Schleswig-Holstein, Kiel, GermanyDepartment of Neurology and Epileptology, Universitätsklinikum Tübingen, Universität Tübingen, Tübingen, Germany0Department of Neurology, Median Clinic Kladow, Kladow, GermanyDepartment of Neurology, University Hospital Magdeburg, Magdeburg, GermanyGerman Center for Neurodegenerative Diseases Magdeburg, Magdeburg, Germany1Department of Behavioral Neurology, Leibniz Institute for Neurobiology, Magdeburg, Germany2Department of Immunohistopathology, Institute of Molecular and Clinical Immunology, Magdeburg, GermanyDepartment of Neurology, University Hospital Schleswig-Holstein, Kiel, Germany3Asklepios Department of Neurology, Brandenburg a.d. Havel, GermanyDepartment of Neurology, University Hospital Magdeburg, Magdeburg, GermanyGerman Center for Neurodegenerative Diseases Magdeburg, Magdeburg, GermanyProgranulin (PGRN), Total-Tau (t-tau), and Neurofilament light chain (NfL) are well known biomarkers of neurodegeneration. The objective of the present study was to investigate whether these parameters represent also biomarkers in autoimmune-mediated Encephalitis (AE) and may give us insights into the pathomechanisms of AE. We retrospectively examined the concentration of PGRN in the cerebrospinal fluid (CSF) and serum of 38 patients suffering from AE in acute phase and/or under treatment. This AE cohort comprises patients with autoantibodies against: NMDAR (n = 18 patients), Caspr2 (n = 8), Lgi-1 (n = 10), GABAB(R) (n = 1), and AMPAR (n = 1). Additionally, the concentrations of NfL (n = 25) and t-tau (n = 13) in CSF were measured when possible. Follow up data including MRI were available in 13 patients. Several age-matched cohorts with neurological diseases besides neuroinflammation or neurodegeneration served as control groups. We observed that PGRN was significantly elevated in the CSF of patients with NMDAR-AE in the acute phase, but normalized at follow up under treatment (p < 0.01). In the CSF of other patients with AE PGRN was in the range of the CSF levels of control groups. T-tau was highly elevated in the CSF of patients with temporal FLAIR-signal in the MRI and in patients developing a hippocampal sclerosis. NfL was exceptionally high initially in Patients with AE with a paraneoplastic or parainfectious cause and also normalized under treatment. The normalizations of all biomarkers were mirrored in an improvement on the modified Rankin scale. The data suggest that the concentration of PGRN in CSF might be a biomarker for acute NMDAR-AE. Pathological high t-tau levels may indicate a risk for hippocampal sclerosis. The biomarker properties of NfL remain unclear since the levels decrease under treatment, but it could not predict severity of disease in this small cohort. According to our results, we recommend to measure in clinical practice PGRN and t-tau in the CSF of patients with AE.https://www.frontiersin.org/article/10.3389/fneur.2018.00668/fullprogranulinneurofilament light chainNMDAR encephalitisLgi-1 encephalitisCaspr2 encephalitistau |
| spellingShingle | Peter Körtvelyessy Peter Körtvelyessy Peter Körtvelyessy Peter Körtvelyessy Harald Prüss Harald Prüss Lorenz Thurner Walter Maetzler Walter Maetzler Walter Maetzler Deborah Vittore-Welliong Jörg Schultze-Amberger Hans-Jochen Heinze Hans-Jochen Heinze Hans-Jochen Heinze Dirk Reinhold Frank Leypoldt Stephan Schreiber Daniel Bittner Daniel Bittner Biomarkers of Neurodegeneration in Autoimmune-Mediated Encephalitis Frontiers in Neurology progranulin neurofilament light chain NMDAR encephalitis Lgi-1 encephalitis Caspr2 encephalitis tau |
| title | Biomarkers of Neurodegeneration in Autoimmune-Mediated Encephalitis |
| title_full | Biomarkers of Neurodegeneration in Autoimmune-Mediated Encephalitis |
| title_fullStr | Biomarkers of Neurodegeneration in Autoimmune-Mediated Encephalitis |
| title_full_unstemmed | Biomarkers of Neurodegeneration in Autoimmune-Mediated Encephalitis |
| title_short | Biomarkers of Neurodegeneration in Autoimmune-Mediated Encephalitis |
| title_sort | biomarkers of neurodegeneration in autoimmune mediated encephalitis |
| topic | progranulin neurofilament light chain NMDAR encephalitis Lgi-1 encephalitis Caspr2 encephalitis tau |
| url | https://www.frontiersin.org/article/10.3389/fneur.2018.00668/full |
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