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

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Editor: Francesc Graus MD PhD

Neurologic disorders associated with MOG antibodies

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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

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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.
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Tobias Zrzavy PhD

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

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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

Anti-IgLON5 disease

Anti-LGI1 encephalitis

Giant cell arteritis

Chronic inflammatory demyelinating polyradiculoneuropathy

Autoimmune autonomic neuropathy

HTLV-1 associated myelopathy

Myositis and cancer

Paraneoplastic retinopathy

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

Anti-IgLON5 disease

Anti-LGI1 encephalitis

Giant cell arteritis

Chronic inflammatory demyelinating polyradiculoneuropathy

Autoimmune autonomic neuropathy

HTLV-1 associated myelopathy

Myositis and cancer

Paraneoplastic retinopathy

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