Screening for autoantibodies in inflammatory neurological syndrome using fluorescence pattern in a tissue-based assay: Cerebrospinal fluid findings from 793 patients

Liu, Tianni; Chen, Baikeng; Yang, Huiting; Huang, Jiehong; Liu, Si; Yang, Xiaoshan; Huang, Li; Yao, Hanyu; Qiu, Wei; Zhuang, Honghua; Long, Youming; Gao, Cong

doi:10.1016/j.msard.2018.12.036

Cited by 16 publications

(13 citation statements)

References 24 publications

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“…Brain MRI generally shows a typical pattern of striking radial linear periventricular Gd-enhancement [13]. Although MOG-IgG and GFAP-IgG have been found to coexist with other autoantibodies targeting the CNS respectively [5][6][7]14], the simultaneous presence of the two antibodies in both serum and CSF has not been reported. Herein we present the first case of overlapping syndrome of MOG-AD and autoimmune GFAP astrocytopathy in one juvenile patient mainly presenting brainstem encephalitis and optic neuritis, in which double positivity for MOG-IgG and GFAP-IgG in both serum and CSF contributed to the definite diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…In recent years, increasing autoimmune disease entities of the central nervous system (CNS) have been established with the discovery of novel pathogenic autoantibodies against proteins and components located in the CNS such as aquaporin 4 (AQP4) [1], myelin oligodendrocyte glycoprotein (MOG) [2], glial fibrillary acidic protein (GFAP) [3] and N-methyl-d-aspartate receptor (NMDAR) [4]. Interestingly and notably, coexistence of different types of antibodies including AQP4-IgG and GFAP-IgG, GFAP-IgG and NMDAR-IgG [5], NMDAR-IgG and AQP4-IgG [5,6], and NMDAR-IgG and MOG-IgG [6,7] have been found thereafter, even though the underlying mechanisms still remain unknown. However, coexistence of MOG-IgG and GFAP-IgG has been rarely reported to date and only one patient has been identified with simultaneous presence of serum MOG-IgG and CSF GFAP-IgG.…”

Section: Introductionmentioning

confidence: 99%

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Overlapping syndrome of MOG-IgG-associated disease and autoimmune GFAP astrocytopathy

Ding¹,

Ren²,

et al. 2020

J Neurol

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Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been considered to be closely relevant to an inflammatory demyelinating disease of the central nervous system (CNS). Glial fibrillary acidic protein (GFAP) immunoglobulin G (IgG) has been identified as a biomarker for a novel autoimmune astrocytopathy. However, coexistence of MOG-IgG and GFAP-IgG is extremely unusual and only one patient has been described with simultaneous presence of MOG-IgG in serum and GFAP-IgG in cerebrospinal fluid (CSF). Herein, we reported the first case of overlapping syndrome of MOG-IgG-associated disease (MOG-AD) and autoimmune GFAP astrocytopathy in whom MOG-IgG and GFAP-IgG were detected both in serum and CSF. A 20-year-old male patient was referred to our department with the presentation of decreased vision, diplopia and weakness of right limb with unknown reasons. Magnetic resonance imaging (MRI) revealed multiple intracranial lesions presenting hypointensity on T1-weighted images, hyperintensity on T2-weighted and FLAIR images and patchy contrast enhancement. MOG-IgG and GFAP-IgG were detected both in serum and CSF, and the titers of both antibodies fluctuated with the severity of disease. Treatment strategy employing intravenous methylprednisolone pulse therapy followed by oral prednisone with slow tapering resulted in an improvement of his symptoms and a sustained remission. Coexistence of MOG-IgG and GFAP-IgG with distinct underlying pathogeneses necessitates the recommendations to screen all recognized pathogenic antibodies against CNS antigens when an autoimmune disease is suspected, since it shows great significance for definite diagnosis of disease and treatment strategy options.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Overlapping syndrome of MOG-IgG-associated disease and autoimmune GFAP astrocytopathy

Ding¹,

Ren²,

et al. 2020

J Neurol

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“…Antibody detection was performed using an indirect immunofluorescence assay on rat hippocampal and cerebellar tissue, as described by Long et al [7].…”

Section: Methodsmentioning

confidence: 99%

“…The presence of anti-AQP4-M1, AQP4-M23, MOG, CRMP5, Hu, Yo, Ri, amphiphysin, Ma2, NMDAR, AMPA1, AMPA2, CAP2, LG1, GABA receptor, and GAD65 antibodies was assessed in patients who expressed anti-GFAP antibodies. The detection was carried out as described in previous studies [7].…”

Section: Methodsmentioning

confidence: 99%

Overlapping Clinical Syndromes in Patients with Glial Fibrillary Acidic Protein IgG

Han

Huang

Zeng

et al. 2020

Neuroimmunomodulation

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The aim of this paper is to report 2 cases with overlapping syndromes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. Methods: Antibodies were detected by indirect immunofluorescence assay. Patient data were analyzed retrospectively. Results: One patient presented with overlapping neuromyelitis optica spectrum disorder (NMOSD) and positive GFAP-IgG and aquaporin-4-IgG. His main symptoms included vision loss, hiccups, fever, headache, and ataxia. High leukocyte count and protein levels were found in cerebrospinal fluid. Brain magnetic resonance imaging (MRI) revealed abnormalities in the hippocampus, midbrain, pons, medulla, and meninges. Characteristic radial enhancing patterns were seen. The other patient was a male with relapsing polychondritis (RP) and positive GFAP-IgG. His main manifestations were meningoencepha-litis and dementia. MRI showed extensive abnormalities in the white matter around the ventricles, temporal lobe, and thalamus, with enhancement. Both patients responded well to the treatment with steroids and immunosuppressants. Conclusions: Although overlapping syndromes are rare, we report positive GFAP-IgG in 2 cases with NMOSD or RP. Both patients had clinical features of GFAP astrocytopathy, but diagnosis of the condition was very challenging because of the overlapping presentation.

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“…Because studies with large sample sizes are lacking, current in-depth discussion of this issue has not yet been reported. Western blot, which has high specificity for GFAP antibodies, is usually used to confirm experimental results (16). At present, the Mayo Clinic (USA), Second Affiliated Hospital of Guangzhou Medical University (China), and Catholic University (Italy) have reported GFAP-A.…”

Section: Progress In Detection Of Gfap Antibodiesmentioning

confidence: 99%

Detection and significance of glial fibrillary acidic protein antibody in autoimmune astocytopathy and related diseases

Huang¹,

Huang²,

Zhou³

et al. 2023

Ann Transl Med

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Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is an antibody-related astrocytic disease for which a specific GFAP antibody serves as a biological marker. Indeed, cerebral spinal fluid positive and/or seropositivity for GFAP is an important basis for its diagnosis. However, because patients with autoimmune encephalitis or demyelinating diseases can have a similar antibody profile, termed overlapping autoimmune syndrome, it remains a challenge for clinicians to diagnose and suitably classify autoimmune GFAP-A. To further understand the significance of GFAP antibody detection in neuroimmune diseases, this article discusses GFAP antibodies in autoimmune GFAP-A, progress for detection of GFAP antibodies, diagnostic significance of GFAP antibodies in prototypical disease, as well as overlapping syndrome.

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Screening for autoantibodies in inflammatory neurological syndrome using fluorescence pattern in a tissue-based assay: Cerebrospinal fluid findings from 793 patients

Cited by 16 publications

References 24 publications

Overlapping syndrome of MOG-IgG-associated disease and autoimmune GFAP astrocytopathy

Overlapping syndrome of MOG-IgG-associated disease and autoimmune GFAP astrocytopathy

Overlapping Clinical Syndromes in Patients with Glial Fibrillary Acidic Protein IgG

Detection and significance of glial fibrillary acidic protein antibody in autoimmune astocytopathy and related diseases

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