Glial fibrillar acidic protein (GFAP) autoimmunity is a recently identified disease at the interface between autoimmune encephalitis and gliopathies. Results of a French cohort study showed that GFAP autoimmunity was generally associated with a favourable outcome, and monophasic course with low risk of relapse .
GFAP autoimmunity (autoimmune GFAP astrocytopathy) was first described in 2016 by researchers of the Mayo Clinic (USA) . GFAP is a type III intermediate filament protein with several isoforms and is expressed by astrocytes and ependymocytes in the central nervous system. Auto-antibodies against GFAPα are a biomarker of autoimmune GFAP astrocytopathy.
In a French cohort study, all patients from 2 referral centres who tested positive for GFAP antibodies were included (n=46). Clinical, biological, and imaging features were reported, as well as clinical course and outcomes. Median age at onset was 43 years; 65% were male.
Other autoimmune diseases were found in 22%, coexisting neural autoantibodies in 11% (including MOG-IgG and AQP4-IgG). Infectious prodromal symptoms were reported in 82%. The most frequent presentation was meningoencephalitis (61%) and meningoencephalomyelitis (24%). Other/associated clinical presentation included: myelitis (30%), visual tract involvement (35%), and peripheral nervous system involvement (28%).
Cerebrospinal fluid showed pleocytosis (98%), oligoclonal bands (77%), and low glucose level (15%). MRI findings were heterogeneous: radial enhancement was found in 26%, periventricular diffuse T2 hyperintensity in 39%, brainstem involvement in 33%, leptomeningeal enhancement in 23%.
There is no standard treatment regimen for GFAP autoimmunity, which is one of the unmet needs. Immunotherapy was given to 17 patients. Though high severity at presentation was common, 39/46 patients had a monophasic course, associated with a good outcome at last follow-up (Rankin Score ≤2: 89% at 15.5 months).
- Gravier Dumonceau A, et al. GFAP auto-immunity: a French cohort study. MSVirtual 2020, Abstract FC01.05.
- Fang B, et al. JAMA Neurol. 2016 Nov 1;73(11):1297-1307.
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Table of Contents: MS Virtual 2020
Letter from the Editor
MS Virtual 2020 Highlights Podcast
COVID-19 and MS
Treatment Strategies and Results
Management of progressive MS with approved DMT
Novel Treatment Directions
Neuromyelitis Optica Spectrum Disorders
Ocrelizumab benefits maintained in primary progressive MS
Black MS patients have poorer COVID-19 outcomes