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Glial Fibrillary Acidic Protein Autoimmunity A French Cohort Study

Alice Gravier-Dumonceau Roxana Ameli 1 Veronique Rogemond 2, 3, 4, 5 Anne Ruiz 6 Bastien Joubert 3, 7 Sergio Muniz-Castrillo 2, 3 Alberto Vogrig 2, 3 Geraldine Picard 2, 3, 4, 5 Aditya Ambati 2, 3, 4, 5 Marie Benaiteau 8 Florence Rulquin Jonathan Ciron 9, 10 Kumaran Deiva 11 Thomas Broucker Laurent Kremer 12, 13 Philippe Kerschen 14 François Sellal 15 Bastien Bouldoires Roxana Genet Julien Biberon 16 Adrien Bigot 17 Fanny Duval 18 Nahema Issa 19 Elena-Camelia Rusu Mathilde Goudot 20 Anais Dutray 21 Jean Louis Devoize 22 Lucie Hopes 20 Anne-Laure Kaminsky 20 Marion Philbert 23 Eve Chanson 24 Amelie Leblanc 25 Erwan Morvan 26 Daniela Andriuta 27, 28 Philippe Diraison Gabriel Mirebeau 29 Celine Derollez Veronique Bourg 30 Quentin Bodard Clementine Fort Irina Grigorashvili-Coin Guillaume Rieul 31 Daniela Molinier-Tiganas Mickael Bonnan 32 Thierry Tchoumi Jerome Honnorat 3, 7 Romain Marignier 33
1 Département de Neuroradiologie [Centre Hospitalier Lyon Sud - HCL]
2 HCL - Hospices Civils de Lyon
3 INMG - Institut NeuroMyoGène
4 ICM - Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute
5 IMRB - Institut Mondor de Recherche Biomédicale
6 CRNL - Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center
7 Centre de Référence Maladie Rare "Syndromes neurologiques Paranéoplasiques"
8 CHU Toulouse [Toulouse]
9 Department of Neurology B4 unit, CRC-SEP, Toulouse Purpan University Hospital, France
10 Institut Toulousain des Maladies Infectieuses et Inflammatoires (Infinity) INSERM UMR1291-CNRS UMR5051 - Université Toulouse III, France
11 Service de neurologie [Le Kremlin Bicêtre]
12 IRIM - Institut de Recherche en Infectiologie de Montpellier
13 INSERM - Institut National de la Santé et de la Recherche Médicale
14 ENT - Excitabilité nerveuse et thérapeutique
15 Hôpitaux Civils de Colmar
16 CPN - Centre Psychothérapique de Nancy [Laxou]
17 CHRU Tours - Centre Hospitalier Régional Universitaire de Tours
18 Groupe hospitalier Pellegrin
19 CHU Bordeaux [Bordeaux]
20 Service de neurologie [CHRU Nancy]
21 Centre Hospitalier Saint Jean de Perpignan
22 CH Angoulême - Centre Hospitalier d'Angoulême
23 UA UFR SM - Université des Antilles - UFR des sciences médicales Hyacinthe Bastaraud
24 CHU Clermont-Ferrand
25 Service de Neurologie [Brest]
26 CEA-LETI - Commissariat à l'énergie atomique et aux énergies alternatives - Laboratoire d'Electronique et de Technologie de l'Information
27 Service de neurologie [Amiens]
28 LNFP - Laboratoire de Neurosciences Fonctionnelles et Pathologies - UR UPJV 4559
29 UGA UFRM - Université Grenoble Alpes - UFR Médecine
30 CHU - Hôpital Pasteur [Nice]
31 CHU de Saint-Brieuc
32 CHU Pau
33 Service de neurologie, sclérose en plaques, pathologies de la myéline et neuro-inflammation - Hôpital Neurologique Pierre Wertheimer Hospices Civils de Lyon, France
Abstract : Background and Objectives To report the clinical, biological, and imaging features and clinical course of a French cohort of patients with glial fibrillary acidic protein (GFAP) autoantibodies. Methods We retrospectively included all patients who tested positive for GFAP antibodies in the CSF by immunohistochemistry and confirmed by cell-based assay using cells expressing human GFAP alpha since 2017 from 2 French referral centers. Results We identified 46 patients with GFAP antibodies. Median age at onset was 43 years, and 65% were men. Infectious prodromal symptoms were found in 82%. Other autoimmune diseases were found in 22% of patients, and coexisting neural autoantibodies in 11%. Tumors were present in 24%, and T-cell dysfunction in 23%. The most frequent presentation was subacute meningoencephalitis (85%), with cerebellar dysfunction in 57% of cases. Other clinical presentations included myelitis (30%) and visual (35%) and peripheral nervous system involvement (24%). MRI showed perivascular radial enhancement in 32%, periventricular T2 hyperintensity in 41%, brainstem involvement in 31%, leptomeningeal enhancement in 26%, and reversible splenial lesions in 4 cases. A total of 33 of 40 patients had a monophasic course, associated with a good outcome at last follow-up (Rankin Score <= 2: 89%), despite a severe clinical presentation. Adult and pediatric features are similar. Thirty-two patients were treated with immunotherapy. A total of 11/22 patients showed negative conversion of GFAP antibodies. Discussion GFAP autoimmunity is mainly associated with acute/subacute meningoencephalomyelitis with prodromal symptoms, for which tumors and T-cell dysfunction are frequent triggers. The majority of patients followed a monophasic course with a good outcome.
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https://hal-u-picardie.archives-ouvertes.fr/hal-03578197
Contributeur : Louise Dessaivre Connectez-vous pour contacter le contributeur
Soumis le : jeudi 17 février 2022 - 10:08:14
Dernière modification le : mardi 10 mai 2022 - 15:04:03

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U-PICARDIE | UNIV-TLSE3 | HCL | CHLS | IMRB | ENT | UPEC-UPEM | UPEC | BS | UNIV-AG | CEA | UNIV-ST-ETIENNE | UNIV-LYON1 | ICM | UNIV-MONTPELLIER | DRT | LETI | CEA-GRE | SU-MEDECINE | UDL | INFINITY | CNRS | CRNL | SORBONNE-UNIVERSITE | SU-TI | UGA | LNFP | FORGETTING_CRNL

Citation

Alice Gravier-Dumonceau, Roxana Ameli, Veronique Rogemond, Anne Ruiz, Bastien Joubert, et al.. Glial Fibrillary Acidic Protein Autoimmunity A French Cohort Study. Neurology, 2022, 98 (6), pp.E653-E668. ⟨10.1212/WNL.0000000000013087⟩. ⟨hal-03578197⟩

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