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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
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
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Soumis le : jeudi 17 février 2022 - 10:08:14
Dernière modification le : mardi 10 mai 2022 - 15:04:03

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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, 2022, 98 (6), pp.E653-E668. ⟨10.1212/WNL.0000000000013087⟩. ⟨hal-03578197⟩

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