Isolated optic neuritis with positive glial fibrillary acidic protein antibody

Abstract Background and objectives Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) has been reported as a spectrum of autoimmune, inflammatory central nervous system disorders. Linear perivascular radial gadolinium enhancement patterns on brain magnetic resonance imaging (M...

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Main Authors: Nan Jia, Jiawei Wang, Yuhong He, Zhong Li, Chuntao Lai
Format: Article
Language:English
Published: BMC 2023-04-01
Series:BMC Ophthalmology
Subjects:
Online Access:https://doi.org/10.1186/s12886-023-02927-z
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author Nan Jia
Jiawei Wang
Yuhong He
Zhong Li
Chuntao Lai
author_facet Nan Jia
Jiawei Wang
Yuhong He
Zhong Li
Chuntao Lai
author_sort Nan Jia
collection DOAJ
description Abstract Background and objectives Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) has been reported as a spectrum of autoimmune, inflammatory central nervous system disorders. Linear perivascular radial gadolinium enhancement patterns on brain magnetic resonance imaging (MRI) are a hallmark of these disorders. GFAP-A is associated with cerebrospinal fluid (CSF) GFAP antibody (GFAP-Ab), while the association with serum GFAP-Ab is less clear. This study aimed to observe the clinical characteristic and MRI changes of GFAP-Ab-positive optic neuritis (ON). Methods We performed a retrospective, observational case study at the department of neurology, Beijing Tongren Hospital, from December 2020 to December 2021. The serum of 43 patients and CSF samples of 38 patients with ON were tested for GFAP-Ab by cell-based indirect immune-fluorescence test. Results Four patients (9.3%) were detected GFAP-Ab positive, and in three out of the four patients, GFAP-Abs were detected only in serum. All of them demonstrated unilateral optic neuritis. Three patients (1, 2, and 4) experienced severe visual loss (best corrected visual acuity ≤ 0.1). Two patients (2 and 4) had experienced more than one episode of ON at the time of sampling. MRI showed optic nerve hyperintensity on T2 FLAIR images in all GFAP-Ab positive patients, and orbital section involvement was the most common. During follow-up (mean 4.5 ± 1 months), only Patient 1 had a recurrent ON, and no patient developed new other neurological events or systemic symptoms. Conclusion GFAP-Ab is rare in patients with ON and may manifest as isolated, relapsing ON. This supports the notion that the GFAP-A spectrum should comprise isolated ON.
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spelling doaj.art-a2f0f37f32d84c0dac7d3e2f4395c7a92023-04-30T11:10:54ZengBMCBMC Ophthalmology1471-24152023-04-012311710.1186/s12886-023-02927-zIsolated optic neuritis with positive glial fibrillary acidic protein antibodyNan Jia0Jiawei Wang1Yuhong He2Zhong Li3Chuntao Lai4Department of Neurology, Beijing Tongren Hospital, Capital Medical UniversityDepartment of Neurology, Beijing Tongren Hospital, Capital Medical UniversityDepartment of Neurology, Beijing Tongren Hospital, Capital Medical UniversityDepartment of Neurology, Beijing Tongren Hospital, Capital Medical UniversityDepartment of Neurology, Beijing Tongren Hospital, Capital Medical UniversityAbstract Background and objectives Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) has been reported as a spectrum of autoimmune, inflammatory central nervous system disorders. Linear perivascular radial gadolinium enhancement patterns on brain magnetic resonance imaging (MRI) are a hallmark of these disorders. GFAP-A is associated with cerebrospinal fluid (CSF) GFAP antibody (GFAP-Ab), while the association with serum GFAP-Ab is less clear. This study aimed to observe the clinical characteristic and MRI changes of GFAP-Ab-positive optic neuritis (ON). Methods We performed a retrospective, observational case study at the department of neurology, Beijing Tongren Hospital, from December 2020 to December 2021. The serum of 43 patients and CSF samples of 38 patients with ON were tested for GFAP-Ab by cell-based indirect immune-fluorescence test. Results Four patients (9.3%) were detected GFAP-Ab positive, and in three out of the four patients, GFAP-Abs were detected only in serum. All of them demonstrated unilateral optic neuritis. Three patients (1, 2, and 4) experienced severe visual loss (best corrected visual acuity ≤ 0.1). Two patients (2 and 4) had experienced more than one episode of ON at the time of sampling. MRI showed optic nerve hyperintensity on T2 FLAIR images in all GFAP-Ab positive patients, and orbital section involvement was the most common. During follow-up (mean 4.5 ± 1 months), only Patient 1 had a recurrent ON, and no patient developed new other neurological events or systemic symptoms. Conclusion GFAP-Ab is rare in patients with ON and may manifest as isolated, relapsing ON. This supports the notion that the GFAP-A spectrum should comprise isolated ON.https://doi.org/10.1186/s12886-023-02927-zOptic neuritisAutoimmune glial fibrillary acidic protein astrocytopathyAntibodyBehcet’s syndrome
spellingShingle Nan Jia
Jiawei Wang
Yuhong He
Zhong Li
Chuntao Lai
Isolated optic neuritis with positive glial fibrillary acidic protein antibody
BMC Ophthalmology
Optic neuritis
Autoimmune glial fibrillary acidic protein astrocytopathy
Antibody
Behcet’s syndrome
title Isolated optic neuritis with positive glial fibrillary acidic protein antibody
title_full Isolated optic neuritis with positive glial fibrillary acidic protein antibody
title_fullStr Isolated optic neuritis with positive glial fibrillary acidic protein antibody
title_full_unstemmed Isolated optic neuritis with positive glial fibrillary acidic protein antibody
title_short Isolated optic neuritis with positive glial fibrillary acidic protein antibody
title_sort isolated optic neuritis with positive glial fibrillary acidic protein antibody
topic Optic neuritis
Autoimmune glial fibrillary acidic protein astrocytopathy
Antibody
Behcet’s syndrome
url https://doi.org/10.1186/s12886-023-02927-z
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AT jiaweiwang isolatedopticneuritiswithpositiveglialfibrillaryacidicproteinantibody
AT yuhonghe isolatedopticneuritiswithpositiveglialfibrillaryacidicproteinantibody
AT zhongli isolatedopticneuritiswithpositiveglialfibrillaryacidicproteinantibody
AT chuntaolai isolatedopticneuritiswithpositiveglialfibrillaryacidicproteinantibody