Relapsing Autoimmune GFAP Astrocytopathy: Case Report

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Abstract

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.

About the authors

Ekaterina O. Chekanova

Research Center of Neurology

Author for correspondence.
Email: 000012309@mail.ru
ORCID iD: 0000-0001-5442-0877

Postgraduate Student, Neurologist, 6th Neurological Department, Institute of Clinical and Preventive Neurology

Russian Federation, Moscow

Аlla А. Shabalina

Research Center of Neurology

Email: kseniya.antonova@mail.ru
ORCID iD: 0000-0001-9604-7775

D. Sci. (Med.), Leading Researcher, Head, Laboratory of Hemorheology, Hemostasis and Pharmacokinetics (with clinical laboratory diagnostics)

Russian Federation, Moscow

Taras O. Simaniv

Research Center of Neurology

Email: tarassimaniv@ya.ru
ORCID iD: 0000-0001-7256-2668

Cand. Sci. (Med.), Senior Researcher, 6th Neurological Department, Institute of Clinical and Preventive Neurology

Russian Federation, Moscow

Rodion N. Konovalov

Research Center of Neurology

Email: kattorina@list.ru
ORCID iD: 0000-0001-5539-245X

Cand. Sci. (Med.), Senior Researcher, Department of Radiation Diagnostics, Institute of Clinical and Preventive Neurology

Russian Federation, Moscow

Larisa A. Dobrynina

Research Center of Neurology

Email: alinakarshieva@yandex.ru
ORCID iD: 0000-0001-9929-2725

D. Sci. (Med.), Chief Researcher, Head, 3rd Neurological Department, Institute of Clinical and Preventive Neurology

Russian Federation, Moscow

Lyudmila A. Kalashnikova

Research Center of Neurology

Email: kalashnikovaNCN@yandex.ru
ORCID iD: 0000-0003-1142-0548

D. Sci. (Med.), Prof., Principal Researcher, 3rd Neurology Department

Russian Federation, Moscow

Maria V. Gubanova

Research Center of Neurology

Email: kalashnikovancn@yandex.ru
ORCID iD: 0000-0002-9893-712X

Cand. Sci. (Med.), Researcher, 3rd Neurological Department, Institute of Clinical and Preventive Neurology

Russian Federation, Moscow

Maria N. Zakharova

Research Center of Neurology

Email: zakharova@neurology.ru
ORCID iD: 0000-0002-1072-9968

D. Sci. (Med.), Principal Researcher, Head, 6th Neurological Department, Institute of Clinical and Preventive Neurology

Russian Federation, Moscow

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Supplementary files

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2. Fig. 1. Patient M.’s brain and spinal MRI (October 2021).

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3. Fig. 2. Patient M.'s brain and spinal cord MRI (December 2021).

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4. Fig. 3. Patient M.'s brain MRI (November 2022).

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5. Fig. 4. GFAP Ab (IgG; indirect immunofluorescence) both in patient M.'s CSF (А) and serum (В) (November 2022).

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Copyright (c) 2023 Chekanova E.O., Shabalina А.А., Simaniv T.O., Konovalov R.N., Dobrynina L.A., Kalashnikova L.A., Gubanova M.V., Zakharova M.N.

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This work is licensed under a Creative Commons Attribution 4.0 International License.

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