Skip to main content
Top
Published in: Journal of Neurology 9/2020

01-09-2020 | Magnetic Resonance Imaging | Original Communication

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

Authors: Jiaqi Ding, Kaixi Ren, Jun Wu, Hongzeng Li, Tangna Sun, Yaping Yan, Jun Guo

Published in: Journal of Neurology | Issue 9/2020

Login to get access

Abstract

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.
Appendix
Available only for authorised users
Literature
1.
go back to reference Lennon VA, Wingerchuk DM, Kryzer TJ et al (2004) A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 364:2106–2112CrossRef Lennon VA, Wingerchuk DM, Kryzer TJ et al (2004) A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 364:2106–2112CrossRef
2.
go back to reference Reindl M, Di Pauli F, Rostásy K et al (2013) The spectrum of MOG autoantibody-associated demyelinating diseases. Nat Rev Neurol 9(8):455–461CrossRef Reindl M, Di Pauli F, Rostásy K et al (2013) The spectrum of MOG autoantibody-associated demyelinating diseases. Nat Rev Neurol 9(8):455–461CrossRef
3.
go back to reference Fang B, McKeon A, Hinson SR et al (2016) Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol 73(11):1297–1307CrossRef Fang B, McKeon A, Hinson SR et al (2016) Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol 73(11):1297–1307CrossRef
4.
go back to reference Miya K, Takahashi Y, Mori H (2014) Anti-NMDAR autoimmune encephalitis. Brain Dev 36(8):645–652CrossRef Miya K, Takahashi Y, Mori H (2014) Anti-NMDAR autoimmune encephalitis. Brain Dev 36(8):645–652CrossRef
5.
go back to reference Liu T, Chen B, Yang H et al (2019) Screening for autoantibodies in inflammatory neurological syndrome using fluorescence pattern in a tissue-based assay: cerebrospinal fluid findings from 793 patients. Mult Scler Relat Disord 28:177–183CrossRef Liu T, Chen B, Yang H et al (2019) Screening for autoantibodies in inflammatory neurological syndrome using fluorescence pattern in a tissue-based assay: cerebrospinal fluid findings from 793 patients. Mult Scler Relat Disord 28:177–183CrossRef
6.
go back to reference Titulaer MJ, Höftberger R, Iizuka T et al (2014) Overlapping demyelinating syndromes and anti-N-methyl-d-aspartate receptor encephalitis. Ann Neurol 75(3):411–428CrossRef Titulaer MJ, Höftberger R, Iizuka T et al (2014) Overlapping demyelinating syndromes and anti-N-methyl-d-aspartate receptor encephalitis. Ann Neurol 75(3):411–428CrossRef
7.
go back to reference Ren Y, Chen X, He Q et al (2019) Co-occurrence of anti-N-methyl-d-aspartate receptor encephalitis and anti-myelin oligodendrocyte glycoprotein inflammatory demyelinating diseases: a clinical phenomenon to be taken seriously. Front Neurol 10:1271CrossRef Ren Y, Chen X, He Q et al (2019) Co-occurrence of anti-N-methyl-d-aspartate receptor encephalitis and anti-myelin oligodendrocyte glycoprotein inflammatory demyelinating diseases: a clinical phenomenon to be taken seriously. Front Neurol 10:1271CrossRef
8.
go back to reference Brunner C, Lassmann H, Waehneldt TV et al (1989) Differential ultrastructural localization of myelin basic protein, myelin/oligodendroglial glycoprotein, and 2',3'-cyclic nucleotide 3'-phosphodiesterase in the CNS of adult rats. J Neurochem 52(1):296–304CrossRef Brunner C, Lassmann H, Waehneldt TV et al (1989) Differential ultrastructural localization of myelin basic protein, myelin/oligodendroglial glycoprotein, and 2',3'-cyclic nucleotide 3'-phosphodiesterase in the CNS of adult rats. J Neurochem 52(1):296–304CrossRef
9.
go back to reference Pham-Dinh D, Allinquant B, Ruberg M et al (1994) Characterization and expression of the cDNA coding for the human myelin/oligodendrocyte glycoprotein. J Neurochem 63(6):2353–2356CrossRef Pham-Dinh D, Allinquant B, Ruberg M et al (1994) Characterization and expression of the cDNA coding for the human myelin/oligodendrocyte glycoprotein. J Neurochem 63(6):2353–2356CrossRef
10.
go back to reference Johns TG, Bernard CC (1999) The structure and function of myelin oligodendrocyte glycoprotein. J Neurochem 72(1):1–9CrossRef Johns TG, Bernard CC (1999) The structure and function of myelin oligodendrocyte glycoprotein. J Neurochem 72(1):1–9CrossRef
11.
go back to reference Denève M, Biotti D, Patsoura S et al (2019) MRI features of demyelinating disease associated with anti-MOG antibodies in adults. J Neuroradiol 46(5):312–318CrossRef Denève M, Biotti D, Patsoura S et al (2019) MRI features of demyelinating disease associated with anti-MOG antibodies in adults. J Neuroradiol 46(5):312–318CrossRef
12.
go back to reference Jarius S, Paul F, Aktas O et al (2018) MOG encephalomyelitis: international recommendations on diagnosis and antibody testing. J Neuroinflammation 15(1):134CrossRef Jarius S, Paul F, Aktas O et al (2018) MOG encephalomyelitis: international recommendations on diagnosis and antibody testing. J Neuroinflammation 15(1):134CrossRef
13.
go back to reference Flanagan EP, Hinson SR, Lennon VA et al (2017) Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients. Ann Neurol 81(2):298–309CrossRef Flanagan EP, Hinson SR, Lennon VA et al (2017) Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients. Ann Neurol 81(2):298–309CrossRef
14.
go back to reference Yang X, Xu H, Ding M et al (2018) Overlapping autoimmune syndromes in patients with glial fibrillary acidic protein antibodies. Front Neurol 9:251CrossRef Yang X, Xu H, Ding M et al (2018) Overlapping autoimmune syndromes in patients with glial fibrillary acidic protein antibodies. Front Neurol 9:251CrossRef
15.
go back to reference Uzawa A, Mori M, Kuwabara S (2018) MOG antibody disorders and AQP4 antibody NMO spectrum disorders share a common immunopathogenesis. J Neurol Neurosurg Psychiatry 89(9):100CrossRef Uzawa A, Mori M, Kuwabara S (2018) MOG antibody disorders and AQP4 antibody NMO spectrum disorders share a common immunopathogenesis. J Neurol Neurosurg Psychiatry 89(9):100CrossRef
16.
go back to reference Berer K, Mues M, Koutrolos M et al (2011) Commensal microbiota and myelin autoantigen cooperate to trigger autoimmune demyelination. Nature 479(7374):538–541CrossRef Berer K, Mues M, Koutrolos M et al (2011) Commensal microbiota and myelin autoantigen cooperate to trigger autoimmune demyelination. Nature 479(7374):538–541CrossRef
17.
go back to reference Hacohen Y, Wong YY, Lechner C et al (2018) Disease course and treatment responses in children with relapsing myelin oligodendrocyte glycoprotein antibody–associated disease. JAMA Neurol 75(4):478–487CrossRef Hacohen Y, Wong YY, Lechner C et al (2018) Disease course and treatment responses in children with relapsing myelin oligodendrocyte glycoprotein antibody–associated disease. JAMA Neurol 75(4):478–487CrossRef
18.
go back to reference Ramanathan S, Mohammad S, Tantsis E et al (2018) Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. J Neurol Neurosurg Psychiatry 89(2):127–137CrossRef Ramanathan S, Mohammad S, Tantsis E et al (2018) Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. J Neurol Neurosurg Psychiatry 89(2):127–137CrossRef
19.
go back to reference Jurynczyk M, Messina S, Woodhall MR et al (2017) Clinical presentation and prognosis in MOG-antibody disease: a UK study. Brain 140(12):3128–3138CrossRef Jurynczyk M, Messina S, Woodhall MR et al (2017) Clinical presentation and prognosis in MOG-antibody disease: a UK study. Brain 140(12):3128–3138CrossRef
20.
go back to reference Jarius S, Ruprecht K, Kleiter I et al (2016) MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 1: Frequency, syndrome specificity, influence of disease activity, long-term course, association with AQP4-IgG, and origin. J Neuroinflamm 13(1):279CrossRef Jarius S, Ruprecht K, Kleiter I et al (2016) MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 1: Frequency, syndrome specificity, influence of disease activity, long-term course, association with AQP4-IgG, and origin. J Neuroinflamm 13(1):279CrossRef
21.
go back to reference Tea F, Lopez JA, Ramanathan S et al (2019) Characterization of the human myelin oligodendrocyte glycoprotein antibody response in demyelination. Acta Neuropathol Commun 7(1):145CrossRef Tea F, Lopez JA, Ramanathan S et al (2019) Characterization of the human myelin oligodendrocyte glycoprotein antibody response in demyelination. Acta Neuropathol Commun 7(1):145CrossRef
22.
go back to reference Yang X, Huang Q, Yang H et al (2019) Astrocytic damage in glial fibrillary acidic protein astrocytopathy during initial attack. Mult Scler Relat Disord 29:94–99CrossRef Yang X, Huang Q, Yang H et al (2019) Astrocytic damage in glial fibrillary acidic protein astrocytopathy during initial attack. Mult Scler Relat Disord 29:94–99CrossRef
Metadata
Title
Overlapping syndrome of MOG-IgG-associated disease and autoimmune GFAP astrocytopathy
Authors
Jiaqi Ding
Kaixi Ren
Jun Wu
Hongzeng Li
Tangna Sun
Yaping Yan
Jun Guo
Publication date
01-09-2020
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 9/2020
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-020-09869-2

Other articles of this Issue 9/2020

Journal of Neurology 9/2020 Go to the issue