GFAP Auto-immunity : a French Cohort Study
- Conditions
- Autoimmune GFAP Astrocytopathy
- Interventions
- Other: Description and analysis
- Registration Number
- NCT04463550
- Lead Sponsor
- Hospices Civils de Lyon
- Brief Summary
Glial fibrillary acidic protein (GFAP)-Immunoglobulin G (IgG) have recently been described as a biomarker of a novel inflammatory central nervous system (CNS) disorder, termed autoimmune GFAP astrocytopathy. Thus far, four major clinical series have been published (two from Mayo Clinic USA, one from Italy and one from China). GFAP-IgG detected in serum or in cerebrospinal fluid, by tissue-based assay and confirmed by cell-based assay, are associated with encephalitis or meningoencephalitis of acute or subacute onset, less frequently with myelitis or optic disk edema. The characteristic MRI feature is brain linear perivascular radial gadolinium enhancement in the white matter perpendicular to the ventricle, consistent with the immunohistochemical staining pattern of GFAP in rodent brain sections. Approximately 20% of reported cases are associated with a neoplasm (ovarian teratoma mostly). Coexisting neural autoantibodies are described in some patients, N-methyl-D-aspartate (NMDA)-receptor (R)-IgG mostly, followed by aquaporin 4 (AQP4)-IgG. The disease is usually corticosteroid responsive although relapse can occur. In contrast, Chinese patients display poorer outcomes. Pathophysiology is not well understood but the intracellular antigen location makes GFAP-IgG unlikely pathogenic whereas animal models and neuropathologic data suggest a T-cell immune-mediated disorder.
The aim of the investigators is to report the first French cohort of patients GFAP-IgG positive. Investigators retrospectively assessed clinical, immunological and radiological features, treatment response and outcomes.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- UNKNOWN
- Sex
- All
- Target Recruitment
- 38
- Positive GFAP-Ab in serum and/or CSF tested by immunohistochemistry on mouse brain slices and confirmed by cell-based assay (CBA) of HEK293 cells expressing GFAP.
- Diagnosis and follow-up in France
- No age limit : from 0 to unlimited age
- Patients GFAP-IgG negative in serum and CSF
- Absence of complete clinicopathological data
- Foreign follow-up
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description Patients GFAP-IgG positive in serum and/or CSF Description and analysis Patients developing clinical autoimmune encephalitis or meningoencephalomyelitis with anti-GFAP antibodies, managed by the National Reference Center for Paraneoplastic Syndromes and Autoimmune Encephalitis or the National Reference Center for Centre de référence for Neuro-inflammatory diseases of the brain and the spinal cord at the Neurological Hospital of Bron.
- Primary Outcome Measures
Name Time Method Report retrospectively clinical data of patients GFAP-IgG positive. 13 months Describe prodromes (yes or no), neurologic signs and clinical course (acute/subacute - yes or
no - or progressive onset - yes or no), if admitted in intensive care units (yes or no), retrospectively provided by
the treating physicians using a structured questionnaire.or progressive onset), if admitted in intensive care units, neoplastic and dysimmune associated diseases and T cell dysregulation condition .
- Secondary Outcome Measures
Name Time Method Describe GFAP-antibody test results. 13 months positivity of GFAP α -IgG in cerebrospinal fluid, in serum and isoform type at diagnostic and follow up.
positivity of GFAP α -IgG in cerebrospinal fluid, in serum and isoform type at diagnostic and follow up.demographic data of patients GFAP-IgG positive : age at onset 13 months age at onset (years) retrospectively provided by the treating
physicians using a structured questionnaire
Trial Locations
- Locations (1)
Hospices Civils de Lyon
🇫🇷Bron, France