Neurologic disorders associated with MOG antibodies …

Neuroimmunology

Updated 08.03.2023
Released 07.14.2018
Expires For CME 08.03.2026

Neurologic disorders associated with MOG antibodies

Authors: Romana Hoftberger MD, Tobias Zrzavy PhD

See Contributor Disclosures

Editor: Francesc Graus MD PhD

Neurologic disorders associated with MOG antibodies

In This Article

Quick Link

Introduction

Overview

In the last decades, evidence for a critical involvement of a humoral immune response against myelin oligodendrocyte glycoprotein (MOG) in a subgroup of inflammatory demyelinating CNS diseases arose. Antibodies are present in monophasic and recurrent non-multiple sclerosis diseases with a predominance in acute disseminated encephalomyelitis (ADEM) and optic neuritis. As a result, the term “MOG antibody-associated spectrum diseases” (MOGAD) was coined, and new diagnostic criteria have been proposed based on recent cumulating evidence. This article discusses the spectrum of MOG antibody-associated diseases, the implementation of MOG antibodies in clinical routine, and immunopathogenetic findings.

Key points

	• Critical involvement of a humoral immune response against myelin oligodendrocyte glycoprotein exists in a subgroup of inflammatory demyelinating CNS diseases.
	• Antibodies are predominately present in monophasic or recurrent non-multiple sclerosis-acquired demyelinating inflammatory CNS diseases.
	• Although MOG antibodies were first described in pediatric patients, they are also found in a subgroup of adult patients.
	• The clinical phenotype associated with MOG antibodies is age-dependent, with ADEM in children and an optic neuritis presentation in older children and adults.
	• MOG antibodies are associated with a distinct neuropathology.

Historical note and terminology

Apart from multiple sclerosis as the most common inflammatory demyelinating central nervous system disease, other rare entities, such as neuromyelitis optica spectrum diseases or ADEM, must be considered as differential diagnosis in patients with a first demyelinating event. As early treatment initiation is recommended in multiple sclerosis, diagnosis at disease onset is important for patient management and treatment stratification. Despite typical clinical features and paraclinical markers (MRI and cerebrospinal fluid), diagnosis is often challenging. Therefore, there is a need for reliable biological markers for diagnostic and prognostic purposes. In inflammatory demyelinating CNS diseases, it is suggested that an autoimmune reaction finally leading to the cascade of inflammation, demyelination, and axonal loss initiates the development of the disease. Due to diagnostic role of autoantibodies in other autoimmune diseases and evidence for crucial involvement of B cells and antibodies in multiple sclerosis pathogenesis, an international research focus has been directed to antibodies against CNS antigens. A breakthrough was the detection of aquaporin-4 (AQP-4) antibodies in neuromyelitis optica spectrum disorder (30). Knowledge of immunopathogenesis and the diagnostic management changed, and now antibodies against AQP-4 are well integrated in the clinical diagnostic procedures. However, there is still a lack of biological markers for the identification of multiple sclerosis subtypes, AQP-4-negative neuromyelitis optica spectrum disorder cases, ADEM, and other atypical CNS demyelinating diseases.

Numerous potential antigen targets were studied. The most promising results came from studies using cell-based assays expressing full-length, conformationally correct folded myelin oligodendrocyte glycoprotein, which is a protein exclusively present in the CNS at the outermost surface of myelin sheaths and oligodendrocyte membranes. MOG, a protein of 245 amino acids, exposed an immunoglobulin domain in the extracellular space and, therefore, seems to be a potential target structure for an autoimmune reaction against myelin. The exact biological role of MOG remains unclear. It is well-known that in experimental autoimmune encephalomyelitis, MOG is a dominant antigen structure after immunization with CNS tissue homogenates (29). Furthermore, in animal models, antibodies against MOG can enhance demyelination and inflammation. Monoclonal MOG antibodies can cause demyelination in the presence of complement, can induce relapses, and are crucial for myelin phagocytosis of macrophages (36).

Although the role of MOG is well established in animal models, its relevance in human diseases was less clear. Numerous studies addressed the diagnostic, prognostic, and pathogenic relevance of MOG antibodies in multiple sclerosis and other CNS demyelinating diseases with controversial results. One problem was that many of the initial studies analyzed antibodies against recombinant linear or refolded human MOG expressed in E coli using different test mechanisms, such as enzyme-linked immunosorbent assay (ELISA) or western blot. These assays recognized MOG lacking the correct membrane topology and glycosylation (36).

However, antibodies against MOG and their role in CNS demyelinating diseases were still of interest, and a breakthrough succeeded with the development of assay-detecting antibodies against conformational MOG. First evidence once again came from animal models: only antibodies against conformational correct MOG can mediate demyelination, pathogenic antibodies were directed against correct glycosylated MOG, and antibodies against native MOG correlate with disease severity (33; 35).

Clinical manifestations

Presentation and course

	• Age defines the spectrum of neurologic syndromes associated with MOG antibodies.
	• Proposed diagnostic criteria for MOGAD consist of (I) one of the core clinical demyelinating events; (II) positive MOG IgG test; (III) supportive clinical or MRI features if MOG-IgG levels are low; and (IV) reasonable exclusion of alternative etiologies, including multiple sclerosis.

Neurologic disorders associated with MOG antibodies: clinical presentation and disease course. The first promising results of MOG antibodies in demyelinating inflammatory CNS diseases were achieved by using self-assembling radiolabeled MOG tetramers, which identified a subgroup of mainly pediatric patients with ADEM and MOG antibodies (34).

Following studies confirmed the result, and MOG antibodies were predominantly described in children and associated with an ADEM-like presentation and a monophasic disease (04; 12; 37). However, it is now well-known that MOG antibodies were also present in a subset of adult patients with CNS demyelinating syndromes, including ADEM, neuromyelitis optica spectrum disorder, monophasic and recurrent optic neuritis, and transverse myelitis as well as demyelinating syndromes overlapping with anti-NMDA receptor encephalitis; it is also well-known that MOG antibodies can be associated with a recurrent disease course (13). Further, an encephalitis clinical phenotype with cortical lesions and epileptic seizures has been repeatedly reported (08; 45).

Within the last large observational cohort studies on MOG antibody-positive patients shed light on the clinical and demographic features of these patients.

These data indicated that an age-related phenotype distribution exists with a predominant initial presentation of ADEM-like disease in children, especially before the age of 11 years, either with or without concurrent involvement of the optic nerve followed by transverse myelitis. In summary, about 50% of MOG antibody-positive children presented with ADEM, another 25% to 35% with optic neuritis, and the remainder with myelitis and other syndromes, eg, brainstem syndromes. In contrast, seropositive adults presented with optic neuritis in 50% of cases: 25% with myelitis and the others with ADEM, brainstem, cerebral, or multifocal symptoms (19; 02).

MRI abnormalities typically relate to the clinical affected anatomical area. Typical findings in children reflect ADEM-like changes. Enhancement of brain lesions is common with MOGAD cerebral attacks and often has a nonspecific patchy appearance and rarely persists beyond 3 months (16). Optical nerve involvement frequently affects the anterior part, and often (31% to 58%) bilateral. Spinal cord lesions can be present as longitudinal extensive transverse myelitis or as short lesions, with predominance of the grey matter “H-sign” and are reported to particularly involve the conus. However, importantly, initial MRI can be normal despite severe clinical affection (09; 32).

CSF examination showed in more than 50% of adult seropositive patients elevated white cell count and in about 50% elevated albumin CSF/serum ratio. CSF-restricted oligoclonal IgG bands, which are a hallmark of multiple sclerosis, were only present in 10% of patients and in these cases, oligoclonal bands were often transient. Mononuclear cells prevailed within the CSF; however, neutrophils are not uncommon. Similar CSF changes are found in children (26; 24).

Diagnostic criteria of MOGAD. In 2023, the International MOGAD Panel proposed the following criteria for the diagnosis of MOGAD (02):

	1. The presence of a core clinical demyelinating event
		• Optic neuritis
		• Myelitis
		• ADEM
		• Cerebral monofocal or polyfocal deficits
		• Brainstem or cerebellar deficits
		• Cerebral cortical encephalitis often with seizures
		AND
	2. A positive MOG-IgG test in serum (fixed cell-based assay result with a titre ≥1:100):
		AND
	3. Exclusion of better diagnoses, including multiple sclerosis

If the cell-based assay from serum show low positive MOG-IgG titers, is negative but the CSF is positive, or the serum titer is unknown, the demyelinating event must fulfill supportive clinical or MRI criteria.

Supportive features
	• Optic neuritis
		-- Bilateral simultaneous clinical involvement
		-- Longitudinal optic nerve involvement (> 50% length of the optic nerve)
		-- Perineural optic nerve sheath enhancement
		-- Optic disc edema
	• Myelitis
		-- Longitudinally extensive myelitis
		-- Central cord lesion or axial H-sign on imaging
		-- Conus lesion
	• Brain, brainstem, or cerebral syndrome
		-- Multiple ill-defined T2-hyperintense lesions in supratentorial and often infratentorial white matter
		-- Deep gray matter involvement
		-- Ill-defined T2-hyperintensity involving pons, middle cerebellar peduncle, or medulla
		-- Cortical lesion with or without lesional and overlying meningeal enhancement

Prognosis and complications

Early studies indicated a monophasic disease course in patients with MOG antibodies. However, subsequent studies revealed that MOG antibodies are also present in recurrent non-multiple sclerosis disorders. Although severe disease courses are described (27; 42), overall, MOG antibodies seemed to be associated with fewer relapses and a better functional outcome (14). Risk of relapse has been reported as ranging from around 27% to 36% over short follow-up periods, with one study reporting an 8-year risk of 36% (28; 08; 06).

Of note, most acquired disability results from the first attack (approximately 60% to 70%), indicating the importance of acute attack management.

As MOG antibodies are present in monophasic and multiphasic diseases, possible association of a temporal dynamic of antibody levels from disease onset to follow-up with prognosis has been suggested. A decline of MOG antibody titer was more common in patients with a monophasic disease course, whereas a stable or increasing titer during follow-up indicated a recurrent disease (08), such as multiphasic disseminated encephalomyelitis or ADEM, followed by optic neuritis, neuromyelitis optica spectrum disorder, and, rarely, multiple sclerosis with anti-MOG seropositivity (20). A potential use of antibodies for treatment response was encouraged by a study showing that conversion to seronegativity during immune-directed therapy was a predictor for disease activity-free follow-up (23). Two studies found a median time to convert to a seronegative status of about 1 year (01; 46). Persistence of MOG antibodies is associated with the risk of having a relapse by a factor of 2 of 10 (02).

However, as severe disease courses with insufficient treatment response to disease-modifying therapies in association with fluctuation and reappearance of antibodies has been described, the use of MOG antibodies as predictive or treatment response marker warrants further prospective studies with clear international standardized assays and cut-off values (42; 38).

The prognostic value of CSF MOG antibodies remains unclear but a retrospective observational, multicenter study suggests that CSF MOG antibodies are common in MOGAD and are associated with a more severe clinical presentation (05). In addition, the clinical outcome is worse in those patients in whom MOG antibodies are only detected in the CSF.

Biological basis

Etiology and pathogenesis

• Tissue injury may be MOG antibody mediated or through an additive effect of MOG-T-cell-induced inflammation.

The pathogenic role of autoimmune responses to MOG has been well established in numerous studies (36). In both human diseases and experimental animal models, MOG-mediated encephalomyelitis leads to heterogenous clinical phenotypes. Studies investigating the pathogenic role of human MOG antibodies demonstrated that they can activate complement and cellular-dependent cytotoxicity in vitro but had only subtle effects after intracerebral or peripheral injection in rodents (03). However, studies have demonstrated that human MOG antibodies are pathogenic in rodents if they cross react with rodent MOG and if titers and affinity are sufficiently high. Further, there are two possible mechanisms of tissue injury: antibody mediated and augmentation of MOG-T-cell-induced inflammation.

Pathology. Histopathological findings of anti-MOG antibody-associated diseases have only been available from single case reports and revealed some information on the poorly understood underlying pathomechanism. Cases have been published with variable clinical presentations including neuromyelitis optica spectrum disorder, ADEM, relapsing-remitting multiple sclerosis, and atypical demyelinating syndrome and different MRI features, including fulminant, tumefactive lesions (36; 47).

However, two seminal studies described neuropathological changes in large cohort of MOG antibody patients (21; 43). Demyelination occurred by merging of multiple perivenous lesions, with partial axonal preservation. Complement and immunoglobulin deposition is observed at areas of active demyelination. MOG harboring macrophages are located within the perivascular spaces and in demyelinating lesions. AQP4 expression is preserved, and cortical lesions are prevalent with predominance of intracortical and subpial lesions. Importantly, these studies revealed that MOGAD-associated pathology occurs on a background of mainly CD4+ T-cells, in contrast to multiple sclerosis, supporting a humoral-mediated disease mechanism in MOG antibody-associated diseases. These pathoimmunological features strongly suggest that MOGAD is an independent autoimmune demyelinating disease entity, distinct from aquaporin-4+ neuromyelitis optica spectrum disorders and multiple sclerosis (21; 43).

In conclusion, anti-MOG antibodies indicate a distinct pathomechanism in this subgroup of inflammatory demyelinating diseases.

Management

	• The first attack is treated with intravenous methylprednisolone.
	• Chronic immunotherapy is indicated in patients who relapse.

Until now, there have been no prospective studies regarding treatment of MOG antibody-associated diseases. Usually, the first attack is treated with intravenous methylprednisolone, (in case of nonresponder) plasma exchange and IVIg. Restitutio ad integrum after acute attack is reported to be about 50% to over 90% (27; 08; 01).

Because MOG antibodies are often associated with a monophasic disease course and there is a lack of prospective randomized treatment trials, it is unclear whether long-term treatment is required. Assuming a mainly B-cell mediated and humoral immune pathogenesis of the disease, therapy targeting antibodies and B-cells are of special interest. Patients with MOG antibodies are less often treated with a disease-modifying drug compared with patients with AQP-4 antibodies (22). However, it has been shown that after the first demyelinating event, maintenance of immunosuppression or immunomodulatory treatment, such as azathioprine or rituximab, was associated with a decrease of a relapse risk and a reduced annual relapse rate (28; 08; 14). Furthermore, a prospective observational cohort study including 79 MOG antibody-associated spectrum disease patients who received either mycophenolate mofetil or no therapy revealed a reduced risk of relapse of 86% (31).

Overall, prospective studies for the evaluation of use of long-term disease-modifying treatments in patients with MOG antibodies and the value of serial testing for prognosis and treatment monitoring are required.

Outcomes

Limited data are available about the long-term outcome of MOGAD. MOGAD patients seem to have a better disability outcome than those with AQP4 antibodies (40). In one study of 61 patients with a median follow-up of 14 to 15 years, favorable outcomes were achieved in the majority of patients, yet 37.5% had an EDSS score of 3 or greater, an EDSS of 6 or greater occurred in 12.5%, and poor visual outcome (visual acuity of 0.1 or less at least in one eye) occurred in 16% (11).

However, given the published diagnostic MOG-IgG criteria and the limited data available for long-term follow-up, prospective studies about long-term follow-up are needed to determine the true prognosis of the disease (02).

References

01: Armangue T, Olivé-Cirera G, Martínez-Hernandez E, et al. Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Lancet Neurol 2020;19(3):234-46. PMID 32057303
02: Banwell B, Bennett JL, Marignier R, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. Lancet Neurol 2023;22(3):268-82.** PMID 36706773
03: Bradl M, Reindl M, Lassmann H. Mechanisms for lesion localization in neuromyelitis optica spectrum disorders. Curr Opin Neurol 2018;31(3):325-33. PMID 29465432
04: Brilot F, Dale RC, Selter RC, et al. Antibodies to native myelin oligodendrocyte glycoprotein in children with inflammatory demyelinating central nervous system disease. Ann Neurol 2009;66(6):833-42. PMID 20033986
05: Carta S, Cobo Calvo Á, Armangué T, et al. Significance of myelin oligodendrocyte glycoprotein antibodies in CSF: a retrospective multicenter study. Neurology 2023;100(11):e1095-108. PMID 36526426
06: Chen JJ, Huda S, Hacohen Y, et al. Association of maintenance intravenous immunoglobulin with prevention of relapse in adult myelin oligodendrocyte glycoprotein antibody-associated disease. JAMA Neurol 2022;79(5):518-25.** PMID 35377395
07: Chen JJ, Tobin WO, Majed M, et al. Prevalence of myelin oligodendrocyte glycoprotein and aquaporin-4-igg in patients in the optic neuritis treatment trial. JAMA Ophthalmol 2018;136(4):419-22. PMID 29470571
08: Cobo-Calvo A, Ruiz A, Maillart E, et al. Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: The MOGADOR study. Neurology 2018;90(21):e1858-69.** PMID 29695592
09: Denève M, Biotti D, Patsoura S, et al. MRI features of demyelinating disease associated with anti-MOG antibodies in adults. J Neuroradiol 2019;46(5):312-8. PMID 31228536
10: de Mol CL, Wong YY, van Pelt ED, et al. Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study. J Neurol 2018;265(6):1310-9. PMID 29569176
11: Deschamps R, Pique J, Ayrignac X, et al. The long-term outcome of MOGAD: an observational national cohort study of 61 patients. Eur J Neurol 2021;28(5):1659-64. PMID 33528851
12: Di Pauli F, Mader S, Rostasy K, et al. Temporal dynamics of anti-MOG antibodies in CNS demyelinating diseases. Clin Immunol 2011;138(3):247-54. PMID 21169067
13: Di Pauli F, Reindl M, Berger T. New clinical implications of anti-myelin oligodendrocyte glycoprotein antibodies in children with CNS demyelinating diseases. Mult Scler Relat Disord 2018;22:35-7. PMID 29524760
14: Dos Passos GR, Oliveira LM, da Costa BK, et al. MOG-IgG-associated optic neuritis, encephalitis, and myelitis: lessons learned from neuromyelitis optica spectrum disorder. Front Neurol 2018;9:217. PMID 29670575
15: Duignan S, Wright S, Rossor T, et al. Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specific in children with acquired demyelinating syndromes. Dev Med Child Neurol 2018;60(9):958-62. PMID 29468668
16: Elsbernd P, Cacciaguerra L, Krecke KN, et al. Cerebral enhancement in MOG antibody-associated disease. J Neurol Neurosurg Psychiatry 2023. [Epub ahead of print] PMID 37221051
17: Hacohen Y, Mankad K, Chong WK, et al. Diagnostic algorithm for relapsing acquired demyelinating syndromes in children. Neurology 2017;89(3):269-78. PMID 28615429
18: Hamid SH, Whittam D, Mutch K, et al. What proportion of AQP4-IgG-negative NMO spectrum disorder patients are MOG-IgG positive? A cross sectional study of 132 patients. J Neurol 2017;264(10):2088-94. PMID 28840314
19: Hegen H, Reindl M. Recent developments in MOG-IgG associated neurological disorders. Ther Adv Neurol Disord 2020;13:1756286420945135. PMID 33029200
20: Hennes EM, Baumann M, Schanda K, et al. Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome. Neurology 2017;89(9):900-8. PMID 28768844
21: Höftberger R, Guo Y, Flanagan EP, et al. The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody. Acta Neuropathol 2020;139(5):875-92.** PMID 32048003
22: Hoftberger R, Sepulveda M, Armangue T, et al. Antibodies to MOG and AQP4 in adults with neuromyelitis optica and suspected limited forms of the disease. Mult Scler 2015;21(7):866-74. PMID 25344373
23: Hyun JW, Woodhall MR, Kim SH, et al. Longitudinal analysis of myelin oligodendrocyte glycoprotein antibodies in CNS inflammatory diseases. J Neurol Neurosurg Psychiatry 2017;88(10):811-7. PMID 28684532
24: Jarius S, Lechner C, Wendel EM, et al. Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 2: results from 108 lumbar punctures in 80 pediatric patients. J Neuroinflammation 2020b;17(1):262. PMID 32883358
25: Jarius S, Paul F, Aktas O, et al. MOG encephalomyelitis: international recommendations on diagnosis and antibody testing; J Neuroinflammation 2018;15(1):134. PMID 29724224
26: Jarius S, Pellkofer H, Siebert N, et al. Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 1: results from 163 lumbar punctures in 100 adult patients. J Neuroinflammation 2020a;17(1):261. PMID 32883348
27: Jarius S, Ruprecht K, Kleiter I, et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome. J Neuroinflammation 2016;13(1):280. PMID 27793206
28: Jurynczyk M, Messina S, Woodhall MR, et al. Clinical presentation and prognosis in MOG-antibody disease: a UK study. Brain 2017;140(12):3128-38. PMID 29136091
29: Lebar R, Baudrimont M, Vincent C. Chronic experimental autoimmune encephalomyelitis in the guinea pig. Presence of anti-M2 antibodies in central nervous system tissue and the possible role of M2 autoantigen in the induction of the disease. J Autoimmun 1989;2(2):115-32. PMID 2765095
30: Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis; Lancet 2004;364(9451):2106-12. PMID 15589308
31: Li S, Ren H, Xu Y, et al. Long-term efficacy of mycophenolate mofetil in myelin oligodendrocyte glycoprotein antibody-associated disorders: a prospective study. Neurol Neuroimmunol Neuroinflamm 2020;7(3):e705. PMID 32170045
32: Mariano R, Messina S, Roca-Fernandez A, Leite MI, Kong Y, Palace JA. Quantitative spinal cord MRI in MOG-antibody disease, neuromyelitis optica and multiple sclerosis Brain 2021;144(1):198-212. PMID 33206944
33: Marta CB, Oliver AR, Sweet RA, Pfeiffer SE, Ruddle NH. Pathogenic myelin oligodendrocyte glycoprotein antibodies recognize glycosylated epitopes and perturb oligodendrocyte physiology. Proc Natl Acad Sci U S A 2005;102(39):13992-7. PMID 16172404
34: O'Connor KC, McLaughlin KA, De Jager PL, et al. Self-antigen tetramers discriminate between myelin autoantibodies to native or denatured protein. Nat Med 2007;13(2):211-17. PMID 17237795
35: Ohtani S, Kohyama K, Matsumoto Y. Autoantibodies recognizing native MOG are closely associated with active demyelination but not with neuroinflammation in chronic EAE. Neuropathology 2011;31(2):101-11. PMID 20573031
36: Peschl P, Bradl M, Hoftberger R, Berger T, Reindl M. Myelin oligodendrocyte glycoprotein: deciphering a target in inflammatory demyelinating diseases. Front Immunol 2017;8:529. PMID 28533781
37: Probstel AK, Dornmair K, Bittner R, et al. Antibodies to MOG are transient in childhood acute disseminated encephalomyelitis. Neurology 2011;77(6):580-8. PMID 21795651
38: Reindl M, Schanda K, Woodhall M, et al. International multicenter examination of MOG antibody assays. Neurol Neuroimmunol Neuroinflamm 2020;7(2):e674. PMID 32024795
39: Sepulveda M, Aldea M, Escudero D, et al. Epidemiology of NMOSD in Catalonia: influence of the new 2015 criteria in incidence and prevalence estimates. Mult Scler 2017:1352458517735191. PMID 28984163
40: Sepulveda M, Armangue T, Sola-Valls N, et al. Neuromyelitis optica spectrum disorders: comparison according to the phenotype and serostatus. Neurol Neuroimmunol Neuroinflamm 2016;3(3):e225. PMID 27144216
41: Soelberg K, Jarius S, Skejoe H, et al. A population-based prospective study of optic neuritis. Mult Scler 2017;23(14):1893-901. PMID 28980518
42: Spadaro M, Gerdes LA, Krumbholz M, et al. Autoantibodies to MOG in a distinct subgroup of adult multiple sclerosis. Neurol Neuroimmunol Neuroinflamm 2016;3(5):e257. PMID 27458601
43: Takai Y, Misu T, Kaneko K, et al. Myelin oligodendrocyte glycoprotein antibody-associated disease: an immunopathological study. Brain 2020;143(5):1431-46. PMID 32412053
44: Thompson AJ, Banwell BL, Barkhof F, et al. Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol 2018;17(2):162-73. PMID 29275977
45: Valencia-Sanchez C, Guo Y, Krecke KN, et al. Cerebral cortical encephalitis in myelin oligodendrocyte glycoprotein antibody-associated disease. Ann Neurol 2023;93(2):297-302.** PMID 36372941
46: Waters P, Fadda G, Woodhall M, et al. Serial anti-myelin oligodendrocyte glycoprotein antibody analyses and outcomes in children with demyelinating syndromes. JAMA Neurol 2020;77(1):82-93. PMID 31545352
47: Weber MS, Derfuss T, Metz I, Bruck W. Defining distinct features of anti-MOG antibody associated central nervous system demyelination. Ther Adv Neurol Disord 2018;11:1756286418762083. PMID 29623106

Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Authors

Romana Hoftberger MD

Dr. Hoftberger of the Medical University of Vienna received a speaker's honorarium from Biogen.
See Profile
Tobias Zrzavy PhD

Dr. Zrzavy of the Medical University of Vienna has no relevant financial relationships to disclose.
See Profile

Editor

Francesc Graus MD PhD

Dr. Graus, Emeritus Professor, Laboratory Clinical and Experimental Neuroimmunology, Institut D’Investigacions Biomédiques August Pi I Sunyer, Hospital Clinic, Spain, has no relevant financial relationships to disclose.
See Profile

Former Authors

Franziska Di Pauli MD
Thomas Berger MD
Markus Reindl PhD

Patient Profile

Age range of presentation: 0 month to 65+ years
Sex preponderance: Female > male
Heredity: None
Population groups selectively affected: None
Occupation groups selectively affected: None

ICD & OMIM codes

ICD-9: Infectious acute disseminated encephalomyelitis (ADEM): 323.61

Multiple sclerosis: 340

Neuromyelitis optica spectrum disorder: 341.0
ICD-10: Acute disseminated encephalitis and encephalomyelitis, unspecified: G04.00

Multiple sclerosis: G35

Neuromyelitis optica spectrum disorder: G36.0

This is an article preview.
Start a Free Account
to access the full version.

Nearly 3,000 illustrations, including video clips of neurologic disorders.
Every article is reviewed by our esteemed Editorial Board for accuracy and currency.
Full spectrum of neurology in 1,200 comprehensive articles.
Listen to MedLink on the go with Audio versions of each article.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com

ISSN: 2831-9125

Neurologic disorders associated with MOG antibodies

Differential Diagnosis

Acute disseminated encephalomyelitis
Multiple sclerosis
Neuromyelitis optica spectrum diseases
Optic neuritis
Transverse Myelitis
- Brainstem encephalitis
- Encephalitis

Also Relevant

Acute disseminated encephalomyelitis
Autoantibodies: disease markers
Multiple sclerosis: neuroimmunology
Optic neuritis
Transverse myelitis

Clinical Categories

Neuroimmunology

Neurologic disorders associated with MOG antibodies …

Neurologic disorders associated with MOG antibodies

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Associated or underlying disorders

Diagnostic workup

Management

Outcomes

References

Contributors

Authors

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview.
Start a Free Account
to access the full version.

Questions or Comment?

Also in This Category

Autoantibodies: mechanism and testing

Multiple sclerosis: treatment of its symptoms

Anti-GQ1b antibody syndrome

Paraneoplastic syndromes

Anti-IgLON5 disease

Balo concentric sclerosis

Anti-LGI1 encephalitis

Progressive encephalomyelitis with rigidity and myoclonus and glycine receptor antibodies

Neurologic disorders associated with MOG antibodies

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Associated or underlying disorders

Diagnostic workup

Management

Outcomes

References

Contributors

Authors

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview. Start a Free Account to access the full version.

Questions or Comment?

Also in This Category

Autoantibodies: mechanism and testing

Multiple sclerosis: treatment of its symptoms

Anti-GQ1b antibody syndrome

Paraneoplastic syndromes

Anti-IgLON5 disease

Balo concentric sclerosis

Anti-LGI1 encephalitis

Progressive encephalomyelitis with rigidity and myoclonus and glycine receptor antibodies

This is an article preview.
Start a Free Account
to access the full version.