Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP)
https://doi.org/10.14412/2074-2711-2026-4-98-102
Abstract
Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP) is a rare condition associated with the presence of antibodies against an intermediate protein located between the finer microfilaments and the larger microtubules in astrocytes. The condition remains poorly understood, necessitating further research to improve diagnosis and treatment. This article presents a clinical case of encephalomyelitis associated with antibodies to GFAP. In a 40-year-old female patient, the disease presented with the onset of numbness and weakness in the lower limbs, accompanied by pelvic organ dysfunction. The detection of antibodies to GFAP in serum and cerebrospinal fluid, the clinical picture of encephalomyelitis, and an MRI pattern of linear radial perivascular enhancement on T1-weighted images, along with an extensive (extending from TI to TXI) hyperintense lesion on T2-weighted images, led to a diagnosis of autoimmune anti-GFAP astrocytopathy. By the time the diagnosis was confirmed, the patient presented with marked gait disturbance requiring the use of bilateral support, as well as neurogenic bladder dysfunction necessitating intermittent catheterisation. No clinical or radiological progression of the disease was observed during anti-B-cell therapy with rituximab. This case report adds to the existing evidence on the clinical features of anti-GFAP astrocytopathy and demonstrates the importance of timely diagnosis and early initiation of pathogenetic therapy for the disease.
About the Authors
I. Yu. OganesyanRussian Federation
Irina Yurikovna Oganesyan
A.Ya. Kozhevnikov Clinic of Nervous Diseases and Department of Nervous Diseases, N.V. Sklifosovsky Institute of Clinical Medicine
11, Rossolimo St., Build. 1, Moscow 119021
S. A. Dzhukkaeva
Russian Federation
A.Ya. Kozhevnikov Clinic of Nervous Diseases and Department of Nervous Diseases, N.V. Sklifosovsky Institute of Clinical Medicine
11, Rossolimo St., Build. 1, Moscow 119021
Sh. R. Nabiev
Russian Federation
A.Ya. Kozhevnikov Clinic of Nervous Diseases and Department of Nervous Diseases, N.V. Sklifosovsky Institute of Clinical Medicine
11, Rossolimo St., Build. 1, Moscow 119021
O. N. Voskresenskaya
Russian Federation
A.Ya. Kozhevnikov Clinic of Nervous Diseases and Department of Nervous Diseases, N.V. Sklifosovsky Institute of Clinical Medicine
11, Rossolimo St., Build. 1, Moscow 119021
References
1. Fang B, McKeon A, Hinson SR, et al. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Novel Meningoencephalomyelitis. JAMA Neurol. 2016;73(11):1297-307. doi: 10.1001/jamaneurol.2016.2549
2. Kimura A, Takekoshi A, Yoshikura N, et al. Clinical characteristics of autoimmune GFAP astrocytopathy. J Neuroimmunol. 2019;332:91-8. doi: 10.1016/j.jneuroim.2019.04.004
3. Chekanova EO, Shabalina AA, Zakharova MN. Clinical characteristics and short-term outcomes of autoimmune encephalitis in adults. S.S. Korsakov Journal of Neurology and Psychiatry. 2023;123(7-2):103-15 (In Russ.). doi: 10.17116/jnevro2023123072103
4. Gklinos P, Athanasopoulos F, Giatrakou V, et al. Unveiling GFAP Astrocytopathy: Insights from Case Studies and a Comprehensive Review of the Literature. Antibodies (Basel). 2024;13(4):79. doi: 10.3390/antib13040079
5. Flanagan EP, Hinson SR, Lennon VA, et al. Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients. Ann Neurol. 2017;81(2):298-309. doi: 10.1002/ana.24881
6. Shan F, Long Y, Qiu W. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Review of the Literature. Front Immunol. 2018;9:2802. doi: 10.3389/fimmu.2018.02802
7. Kunchok A, Zekeridou A, McKeon A. Autoimmune glial fibrillary acidic protein astrocytopathy. Curr Opin Neurol. 2019;32(3):452-8. doi: 10.1097/WCO.0000000000000676
8. Dubey D, Hinson SR, Jolliffe EA, et al. Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1/year. J Neuroimmunol. 2018;321:157-63. doi: 10.1016/j.jneuroim.2018.04.016
9. Kotov AS, Ovchinnikova EO. Autoimmune GFAP astrocytopathy. Lecture and clinical cases. Russian Journal of Child Neurology. 2025;20(4):77-83 (In Russ.). doi: 10.17650/2073-8803-2025-20-4-77-83
10. Chekanova EO, Shabalina AA, Simaniv TO, et al. Relapsing autoimmune GFAP astrocytopathy: case report. Annals of Clinical and Experimental Neurology. 2023;17(4):89-96 (In Russ.). doi: 10.54101/ACEN.2023.4.11
11. Ahmed S, K VCC, Kannoth S, et al. Autoimmune GFAP Astrocytopathy-Beyond the Known Horizon, India's First Multifaceted Institutional Experience. Ann Neurosci. 2024;32(4):248-58. doi: 10.1177/09727531241230213
12. Fang H, Hu W, Jiang Z, et al. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy in Children: A Retrospective Analysis of 35 Cases. Front Immunol. 2021 Nov 22;12:761354. doi: 10.3389/fimmu.2021.761354
13. Theuriet J, Cluse F, Gravier-Dumonceau A, et al. Peripheral nervous system involvement accompanies central nervous system involvement in anti-glial fibrillary acidic protein (GFAP) antibody-related disease. J Neurol. 2023;270(11):5545-60. doi: 10.1007/s00415-023-11908-7
14. Shetty D, Brahmbhatt S, Desai A, et al. Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics. AJNR Am J Neuroradiol. 2024;45(10):1394-402. doi: 10.3174/ajnr.A8236
15. Shu Y, Huang R, Li Q, et al. Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Is Associated with HLA-A*3303 and HLADPB1*0501. Ann Neurol. 2024;95(5):901-6. doi: 10.1002/ana.26899
16. Levraut M, Marignier R, Cohen M, et al. Relevance of Kappa and Lambda Free Light Chains in Autoimmune Astrocytopathy Associated With Anti-GFAP Antibodies. Ann Clin Transl Neurol. 2025;12(9):1785-93. doi: 10.1002/acn3.70103
17. Zhang W, Xie Y, Wang Y, et al. Clinical characteristics and prognostic factors for short-term outcomes of autoimmune glial fibrillary acidic protein astrocytopathy: a retrospective analysis of 33 patients. Front Immunol. 2023;14:1136955. doi: 10.3389/fimmu.2023.1136955
18. Marotta C, Rolla E, Fabris M, et al. Iatrogenic NORSE: Immune checkpoint inhibitor-related anti-GFAP autoimmune astrocytopathy. J Neuroimmunol. 2026;410:578805. doi: 10.1016/j.jneuroim.2025.578805
19. Bernard Healey SA, Benjamin L. Epstein-Barr virus reactivation as a trigger in autoimmune GFAP astrocytopathy. J Neurol Neurosurg Psychiatry. 2026;97(2):99. doi: 10.1136/jnnp-2025-337402
20. Hagbohm C, Ouellette R, Flanagan EP, et al. Clinical and neuroimaging phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy: A systematic review and metaanalysis. Eur J Neurol. 2024;31(7):e16284. doi: 10.1111/ene.16284
21. Ding J, Ren K, Wu J, et al. Overlapping syndrome of MOG-IgG-associated disease and autoimmune GFAP astrocytopathy. J Neurol. 2020;267(9):2589-93. doi: 10.1007/s00415-020-09869-2
22. Liu L, Ma B, Peng J, et al. The clinical significance of S100B, APP, and CHI3L1 in autoimmune GFAP astrocytopathy. Front Neurol. 2026;17:1801196. doi: 10.3389/fneur.2026.1801196
23. Arzalluz-Luque J, Dumez P, Picard G, et al. Clinical course and long-term outcomes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. J Neurol. 2025;272(6):421. doi: 10.1007/s00415-025-13159-0
24. Yang X, Jin L, Shi C, et al. The Clinical Characteristics and Treatment of Patients With Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy (GFAP-A): A Retrospective Study of 29 Patients. Brain Behav. 2026 Jan;16(1):e71159. doi: 10.1002/brb3.71159
25. Cao S, Du J, Pan S, et al. Case Report: Ofatumumab treatment in a patient with rituximab-intolerant refractory autoimmune GFAP astrocytopathy. Front Immunol. 2023;14:1164181. doi: 10.3389/fimmu.2023.1164181
26. Lin Y, Diao Z, Low J, et al. Therapeutic efficacy of telitacicept in a patient with GFAP autoimmune astrocytopathy: a case report. Front Immunol. 2025;16:1691029. doi: 10.3389/fimmu.2025.1691029
27. Fang J, Tong Z, Lu W. Case Report: Need for Caution in the Diagnosis of GFAP Astrocytopathy-A Case of GFAP Astrocytopathy Coexistent With Primary Central Nervous System Lymphoma. Front Neurol. 2022;13:806224. doi: 10.3389/fneur.2022.806224
Review
For citations:
Oganesyan IY, Dzhukkaeva SA, Nabiev SR, Voskresenskaya ON. Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP). Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2026;18(4):98-102. (In Russ.) https://doi.org/10.14412/2074-2711-2026-4-98-102
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