Hopital Neurologique Pierre-Wertheimer

Author Of 1 Presentation

Diagnostic Criteria and Differential Diagnosis Oral Presentation

FC01.05 - GFAP auto-immunity: a French cohort study

Presentation Number
FC01.05
Presentation Topic
Diagnostic Criteria and Differential Diagnosis
Lecture Time
13:48 - 14:00

Abstract

Background

Glial fibrillar acidic protein (GFAP) autoimmunity is a recently identified disease, at the frontier of auto-immune encephalitis and gliopathies. Clinical features associated to this new entity are still not fully evaluated; especially the risk of relapse and the disability outcome.

Objectives

To report the clinical, biological, imaging features, and the clinical course of a French cohort of patients with GFAP autoantibodies.

Methods

We retrospectively included all patients tested positive for GFAP antibodies since 2017, from two French referral centers (auto-immune encephalitis and rare inflammatory disorders of the brain and the spinal cord). GFAP autoantibodies were detected exclusively in the CSF, by immunohistochemistry and their specificity confirmed by cell-based assay using cells expressing human GFAPα.

Results

We identified 46 patients with GFAP antibodies. Median age at onset was 43 years and men were more affected (30/46). Infectious prodromal symptoms were found in 82% of cases. Other autoimmune diseases were found in 22% of cases and coexisting neural autoantibodies in 11% of cases, including MOG-IgG and AQP4-IgG positive cases. Tumors were present in 24%, and T cell dysfunction in 23% of cases, respectively. The most frequent presentation was acute/subacute menigoencephalitis (85%) with cerebellar dysfunction in 57% of cases. Other/associated clinical presentation included: myelitis (30%), visual tract involvement (35%) and peripheral nervous system involvement (28%). CSF 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%, and reversible splenial lesions in 4 cases.

39/46 patients have a monophasic course, associated to a good outcome at last follow-up (Rankin Score≤2: 89% at 15,5 months), despite a frequently severe clinical presentation (intensive care unit hospitalization: 42%). Seventy patients were treated with immunotherapy. 11/22 patients showed negativation of GFAP antibodies (median time: 2 months).

Conclusions

GFAP autoimmunity was generally associated to a good outcome and a low risk of relapse. Identification of factors associated to risk of relapse or disability, to monitor immunotherapy, is needed.

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Presenter Of 1 Presentation

Diagnostic Criteria and Differential Diagnosis Oral Presentation

FC01.05 - GFAP auto-immunity: a French cohort study

Presentation Number
FC01.05
Presentation Topic
Diagnostic Criteria and Differential Diagnosis
Lecture Time
13:48 - 14:00

Abstract

Background

Glial fibrillar acidic protein (GFAP) autoimmunity is a recently identified disease, at the frontier of auto-immune encephalitis and gliopathies. Clinical features associated to this new entity are still not fully evaluated; especially the risk of relapse and the disability outcome.

Objectives

To report the clinical, biological, imaging features, and the clinical course of a French cohort of patients with GFAP autoantibodies.

Methods

We retrospectively included all patients tested positive for GFAP antibodies since 2017, from two French referral centers (auto-immune encephalitis and rare inflammatory disorders of the brain and the spinal cord). GFAP autoantibodies were detected exclusively in the CSF, by immunohistochemistry and their specificity confirmed by cell-based assay using cells expressing human GFAPα.

Results

We identified 46 patients with GFAP antibodies. Median age at onset was 43 years and men were more affected (30/46). Infectious prodromal symptoms were found in 82% of cases. Other autoimmune diseases were found in 22% of cases and coexisting neural autoantibodies in 11% of cases, including MOG-IgG and AQP4-IgG positive cases. Tumors were present in 24%, and T cell dysfunction in 23% of cases, respectively. The most frequent presentation was acute/subacute menigoencephalitis (85%) with cerebellar dysfunction in 57% of cases. Other/associated clinical presentation included: myelitis (30%), visual tract involvement (35%) and peripheral nervous system involvement (28%). CSF 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%, and reversible splenial lesions in 4 cases.

39/46 patients have a monophasic course, associated to a good outcome at last follow-up (Rankin Score≤2: 89% at 15,5 months), despite a frequently severe clinical presentation (intensive care unit hospitalization: 42%). Seventy patients were treated with immunotherapy. 11/22 patients showed negativation of GFAP antibodies (median time: 2 months).

Conclusions

GFAP autoimmunity was generally associated to a good outcome and a low risk of relapse. Identification of factors associated to risk of relapse or disability, to monitor immunotherapy, is needed.

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