Relapsing Autoimmune GFAP Astrocytopathy: Case Report
| Autor: | Ekaterina O. Chekanova, Аlla А. Shabalina, Taras O. Simaniv, Rodion N. Konovalov, Larisa A. Dobrynina, Lyudmila A. Kalashnikova, Maria V. Gubanova, Maria N. Zakharova |
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| Jazyk: | English<br />Russian |
| Rok vydání: | 2023 |
| Předmět: | |
| Zdroj: | Анналы клинической и экспериментальной неврологии, Vol 17, Iss 4, Pp 89-96 (2023) |
| Druh dokumentu: | article |
| ISSN: | 2075-5473 2409-2533 |
| DOI: | 10.54101/ACEN.2023.4.11 |
| Popis: | Introduction. Glial fibrillary acidic protein (GFAP) is the main component of intermediate astrocyte filaments. In 2016, anti-GFAP antibodies (Ab) were identified as the specific biomarker for the first established CNS inflammatory disorder subsequently called autoimmune astrocytopathy associated with anti-GFAP Ab (A-GFAP-A). Since GFAP is localized intracellularly, GFAP Ab do not appear to be directly pathogenic though serve as a biomarker of immune inflammation. Although presence of GFAP-Ab in the serum (but not in the CSF) could be observed in various CNS immune-mediated diseases, detection of GFAP-Ab in CSF is only characteristic for A-GFAP-A. A-GFAP-A usually develops after the age of 40 and mostly manifests acutely or subacutely with symptoms of meningoencephalomyelitis or its focal forms. Linear perivascular radial cerebral white matter enhancement is a specific MRI finding of A-GFAP-A. Concomitant neoplasms or autoimmune disorders, as well as co-expression of other antineuronal antibodies are not uncommon in A-GFAP-A. Usually, disease responds well to immunotherapy, and prolonged remission could be achieved, however recurrent disease course and fulminant cases are also described in the literature. In these cases, long-term immunosuppression is required. Data on epidemiology, etiological factors, and precise pathogenesis of A-GFAP-A are still limited. Due to the lack of long-term follow-up data, diagnostic criteria, generally accepted treatment strategies or prognostic risk factors for relapse and outcome of the disease have not yet been established and precised. We present the first description of a case of relapsing A-GFAP-A in Russia and an analysis of the current data on the pathogenesis, clinical features, as well as the diagnostic challenges and treatment approaches for A-GFAP-A. |
| Databáze: | Directory of Open Access Journals |
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