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Glial Fibrillary Acidic Protein Autoimmunity A French Cohort Study

Alice Gravier-Dumonceau 1 Roxana Ameli 2 Veronique Rogemond 3, 4, 5, 6 Anne Ruiz 1 Bastien Joubert 4, 7 Sergio Muniz-Castrillo 3, 4 Alberto Vogrig 3, 4 Geraldine Picard 3, 4, 5, 6 Aditya Ambati 3, 4, 5, 6 Marie Benaiteau 8 Florence Rulquin Jonathan Ciron 9, 8 Kumaran Deiva 10 Thomas De Broucker Laurent Kremer 11, 12 Philippe Kerschen 13 François Sellal 14 Bastien Bouldoires Roxana Genet Julien Biberon 15 Adrien Bigot 16 Fanny Duval 17 Nahema Issa 18 Elena-Camelia Rusu Mathilde Goudot 19 Anais Dutray 20 Jean Louis Devoize 21 Lucie Hopes 19 Anne-Laure Kaminsky 19 Marion Philbert 22 Eve Chanson 23 Amelie Leblanc 24 Erwan Morvan 25 Daniela Andriuta 26, 27 Philippe Diraison Gabriel Mirebeau 28 Celine Derollez Veronique Bourg 29 Quentin Bodard Clementine Fort Irina Grigorashvili-Coin Guillaume Rieul 30 Daniela Molinier-Tiganas Mickael Bonnan 31 Thierry Tchoumi Jerome Honnorat 4, 7 Romain Marignier 3 
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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Soumis le : mercredi 15 juin 2022 - 10:22:10
Dernière modification le : samedi 24 septembre 2022 - 15:04:06

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

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