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September 2022; 9 (5) RESEARCH ARTICLEOpen Access

Cerebellar Ataxia With Anti-DNER Antibodies

Outcomes and Immunologic Features

View ORCID ProfileElise Peter, View ORCID ProfileLe Duy Do, View ORCID ProfileSalem Hannoun, View ORCID ProfileSergio Muñiz-Castrillo, View ORCID ProfileAlberto Vogrig, Valentin Wucher, Anne-Laurie Pinto, Naura Chounlamountri, Walaa Zakaria, View ORCID ProfileVeronique Rogemond, Geraldine Picard, Julien-Jacques Hedou, View ORCID ProfileAditya Ambati, Agusti Alentorn, Alexandra Traverse-Glehen, Mario Manto, Dimitri Psimaras, Emmanuel Mignot, View ORCID ProfileFrancois Cotton, Virginie Desestret, Jérôme Honnorat, View ORCID ProfileBastien Joubert
First published August 8, 2022, DOI: https://doi.org/10.1212/NXI.0000000000200018
Elise Peter
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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  • ORCID record for Elise Peter
Le Duy Do
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Salem Hannoun
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Sergio Muñiz-Castrillo
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Alberto Vogrig
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Valentin Wucher
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Anne-Laurie Pinto
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Naura Chounlamountri
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Walaa Zakaria
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Veronique Rogemond
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Geraldine Picard
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Julien-Jacques Hedou
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Aditya Ambati
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Agusti Alentorn
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Alexandra Traverse-Glehen
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Mario Manto
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Dimitri Psimaras
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Emmanuel Mignot
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Francois Cotton
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Virginie Desestret
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Jérôme Honnorat
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Bastien Joubert
From the Centre National de Référence pour les Syndromes Neurologiques Paranéoplasiques (E.P., S.M.-C., A.V., A.-L.P., V.R., G.P., V.D.,J.H., B.J.), Hospices Civils de Lyon, Hôpital Neurologique, Bron, France; Synaptopathies and Autoantibodies (SynatAc) Team (E.P., L.D.D., S.M.-C., A.V., V.W., N.C., V.D., J.H., B.J.), Institut NeuroMyoGène-MeLis, INSERM U1314/CNRS UMR 5284, Université de Lyon, France; Medical Imaging Sciences Program (S.H., W.Z.), Division of Health Professions, Faculty of Health Sciences, American University of Beirut, Lebanon; Center for Sleep Sciences and Medicine (J.-J.H., Aditya Ambati, E.M.), Stanford University, Palo Alto, CA; Service de Neurologie 2-Mazarin (Agusti Alentorn, D.P.), Hôpitaux Universitaires La Pitié-Salpêtrière–Charles Foix, APHP; Inserm U1127 CNRS UMR 7225 (Agusti Alentorn, D.P.), Institut du Cerveau et de la Moelle épinière, ICM, Université Pierre-et-Marie-Curie, Sorbonne Universités, Paris, France; INSERM Unité Mixte de Recherche (UMR) S1052 (A.T.-G.), Centre National de la Recherche UMR 5286, Centre de Recherche en Cancérologie de Lyon, France; Département de Pathologie (A.T.-G.), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre-Bénite Cedex, France; Service des Neurosciences (M.M.), UMons, Mons, Belgium; Service de Neurologie (M.M.), CHU-Charleroi, Charleroi, Belgium; Department of Radiology (F.C.), Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France; and Université Lyon 1 (F.C.), CREATIS-LRMN, CNRS/UMR/5220-INSERM U630, Villeurbanne, France.
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Citation
Cerebellar Ataxia With Anti-DNER Antibodies
Outcomes and Immunologic Features
Elise Peter, Le Duy Do, Salem Hannoun, Sergio Muñiz-Castrillo, Alberto Vogrig, Valentin Wucher, Anne-Laurie Pinto, Naura Chounlamountri, Walaa Zakaria, Veronique Rogemond, Geraldine Picard, Julien-Jacques Hedou, Aditya Ambati, Agusti Alentorn, Alexandra Traverse-Glehen, Mario Manto, Dimitri Psimaras, Emmanuel Mignot, Francois Cotton, Virginie Desestret, Jérôme Honnorat, Bastien Joubert
Neurol Neuroimmunol Neuroinflamm Sep 2022, 9 (5) e200018; DOI: 10.1212/NXI.0000000000200018

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    Figure 1 Longitudinal Volumetric Analysis of the Patients' Cerebellum

    (A) Boxplot showing the volumes of cerebellar white matter, cerebellar gray matter, and whole cerebellum at baseline and follow-up, in 10 patients with anti-DNER antibodies. Volumes are expressed as percentages of intracranial volume. There is a significant decrease of cerebellar gray matter and whole-cerebellum volumes at follow-up, compared with baseline, **p < 0.005. (B) Representative images of sagittal T1-weighted brain MRI at baseline and follow-up of a patient with favorable outcome (follow-up MRI 9 months after disease onset) and a patient with poor outcome (follow-up MRI 6 months after disease onset).

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    Figure 2 Proteomic Analysis of the Cerebrospinal Fluid of Patients With DNER Ataxia

    Heatmap of CSF log2 expression of proteins shown as median Z-scores per protein in patients with anti-DNER antibodies (n = 5) compared with controls (n = 40). Overexpressed proteins were mainly related to immune response. Among them, overexpression of C-X-C motif chemokine 13 (CXCL-13) and CD48 is implicated in B-cell activation. Other immune-related proteins included complement component 2 (C2), interleukin-1 receptor type 2 (IL1R2), chemokine C-X-C motif chemokine 10 (CXCL10), pulmonary surfactant-associated protein D (SFtPD), and hepcidin (HAMP). Proteins of leukocyte migration were also overexpressed, including vascular cell adhesion protein 1 (VCAM-1), and integrin alpha1 and beta1 (ITGB1/ITGA1). Other overexpressed proteins likely reflected tissue lysis, including proteins involved in cell cycle or processing of proteins and RNA, such as heterogeneous nuclear ribonucleoproteins A2/B1 (HNRNPA2B1), SUMO-conjugating enzyme UBC9 (UBE2I), and acidic leucine-rich nuclear phosphoprotein 32 family member B (ANP32B). Underexpressed proteins were interferon gamma (IFNG), and proteins mostly related to neural development (such as neuronal growth regulator 1 or NEGR1 and the neurexins 1 and 3: NRXN1, NRXN3) or cell cycle (e.g., BH3-interacting domain death agonist [BID]).

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    Figure 3 DNER Antibodies Bind DNER at the Surface of Purkinje Cells

    (A) Incubation of nonpermeabilized live organotypic cerebellar cultures with an anti–DNER-positive CSF shows punctuate cell-surface immunolabeling (top) that is not obtained with a control CSF (bottom). Purkinje cells were labeled with an anti-calbindin D-28K antibody (red). (B) Fixed and permeabilized organotypic cerebellar cultures labeled with an anti-DNER-positive CSF (green), a commercial anti-DNER antibody (purple), and calbindin D-28K (red). Commercial anti-DNER and human DNER-specific IgG demonstrate superposable staining of the dendrites and soma of Purkinje cells.

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