Glial Fibrillary Acidic Protein Autoimmunity: A French Cohort Study. (8th February 2022)

• Record Type: Journal Article
• Authors: Gravier-Dumonceau, Alice; Ameli, Roxana; Rogemond, Veronique; Ruiz, Anne; Joubert, Bastien; Muñiz-Castrillo, Sergio; Vogrig, Alberto; Picard, Geraldine; Ambati, Aditya; Benaiteau, Marie; Rulquin, Florence; Ciron, Jonathan; Deiva, Kumaran; de Broucker, Thomas; Kremer, Laurent; Kerschen, Philippe; Sellal, François; Bouldoires, Bastien; Genet, Roxana; Biberon, Julien; Bigot, Adrien; Duval, Fanny; Issa, Nahema; Rusu, Elena-Camelia; Goudot, Mathilde; Dutray, Anais; Devoize, Jean Louis; Hopes, Lucie; Kaminsky, Anne-Laure; Philbert, Marion; Chanson, Eve; Leblanc, Amelie; Morvan, Erwan; Andriuta, Daniela; Diraison, Philippe; Mirebeau, Gabriel; Derollez, Celine; Bourg, Veronique; Bodard, Quentin; Fort, Clementine; Grigorashvili-Coin, Irina; Rieul, Guillaume; Molinier-Tiganas, Daniela; Bonnan, Mickaël; Tchoumi, Thierry; Honnorat, Jérôme; Marignier, Romain;
• Is Part Of: Neurology. Volume 98:Number 6(2022)
• Page Start: e653
• Page End: e668
• Publication Date: 2022-02-08
• Subjects: Neurology -- Periodicals; Neurology -- Periodicals; Neurologie -- Périodiques; 616.8
• DOI: 10.1212/WNL.0000000000013087
• Languages: English
• ISSNs: 0028-3878

Abstract

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 GFAPα 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.