MRI features of demyelinating disease associated with anti-MOG antibodies in adults

doi:10.1016/j.neurad.2019.06.001

Journal of Neuroradiology

Volume 46, Issue 5, September 2019, Pages 312-318

https://doi.org/10.1016/j.neurad.2019.06.001 Get rights and content

Abstract

The spectrum of Myelin Oligodendrocytes Glycoprotein (MOG) antibody disease constitutes a recently described challenging entity, referring to a relatively new spectrum of autoimmune disorders with antibodies against MOG predominantly involving the optic nerve and spinal cord. The purpose of this article is to describe MRI features of MOG-AD involvement in the optic nerves, spinal cord and the brain of adults.

Section snippets

Background

MOG is a glycoprotein located on the myelin surface and found exclusively in the central nervous system (CNS). Although its exact role remains unclear, it is thought to act as a cellular adhesive molecule, as a regulator of oligodendrocyte microtubule stability and as a mediator of the complement activation cascade.

In recent years, a new diagnostic role has been discovered for MOG antibodies (MOG-IgG), particularly in adults. While MOG-IgGs were initially thought to play a role in typical cases

Clinical and demographic characteristics

MOG-AD can occur in all decades of life, with a median age of onset in the early to mid-thirties, and it affects slightly more women than men (female: male ratio = 1.1:1) [5], [9], [10], [11], [12], [13].

The most common presenting feature is optic neuritis (ON), occurring in 54–61% of patients, followed by myelitis. More rarely MOG-AD involves the brain, the phenotype in these cases is often similar to acute disseminated encephalomyelitis (ADEM) or an ADEM-like presentation (e.g., brainstem

Optic nerve MRI findings in MOG-SD

MRI features of optical neuritis are highly reproducible among MOG+ patients. During optic neuritis attacks, orbital MRI (ideally acquired with coronal fat-suppressed T2- and post-contrast T1-weighted sequences) shows an extensive optic nerve T2 hyperintensity. This predominantly involves the anterior segments of the optic nerve, with almost routine inclusion of the intra-orbital segments, while chiasm and retro-chiasmatic pathways are generally spared [18], [27]. The optic nerve becomes

Conclusion

MOG antibody disease is now considered a specific entity per se, predominantly affecting the optic nerves, spinal cord and, to some extent, the brain. Although it may resemble AQP4-IgG-mediated NMOSD, or even MS or ADEM from a clinical perspective, MRI could certainly help to highlight anti-MOG disease when key imaging features are demonstrated, such as bilateral extensive oedematous and inflammatory anterior optic neuritis or longitudinally extended myelitis with a predilection to the conus,

Disclosure of interest

The authors declare that they have no competing interest.

References (39)

R. Narayan et al.
MOG antibody disease: a review of MOG antibody seropositive neuromyelitis optica spectrum disorder
Mult Scler Relat Disord
(2018)
J. Zhou et al.
An unusual case of anti-MOG CNS demyelination with concomitant mild anti-NMDAR encephalitis
J Neuroimmunol
(2018)
M. Nakamura et al.
A case of MOG antibody-positive bilateral optic neuritis and meningoganglionitis following a genital herpes simplex virus infection
Mult Scler Relat Disord
(2017)
M. Denève et al.
Caractéristiques IRM des neuromyélites optiques associées aux anticorps anti-MOG
J Neuroradiol
(2018)
M. Umino et al.
3D double inversion recovery MR imaging: clinical applications and usefulness in a wide spectrum of central nervous system diseases
J Neuroradiol
(2019)
Y. Pekcevik et al.
What do we know about brain contrast enhancement patterns in neuromyelitis optica?
Clin Imaging
(2016)
M. Reindl et al.
The spectrum of MOG autoantibody-associated demyelinating diseases
Nat Rev Neurol
(2013)
S. Jarius et al.
Aquaporin-4 antibodies (NMO-IgG) as a serological marker of neuromyelitis optica: a critical review of the literature
Brain Pathol Zurich Switz
(2013)
S.H.M. Hamid 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)
S. Jarius et al.
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 Neuroinflammation
(2016)

J. Kitley et al.

Myelin-oligodendrocyte glycoprotein antibodies in adults with a neuromyelitis optica phenotype

Neurology

(2012)

M. Spadaro et al.

Histopathology and clinical course of MOG-antibody-associated encephalomyelitis

Ann Clin Transl Neurol

(2015)

S. Jarius et al.

Screening for MOG-IgG and 27 other anti-glial and anti-neuronal autoantibodies in “pattern II multiple sclerosis” and brain biopsy findings in a MOG-IgG-positive case

Mult Scler Houndmills Basingstoke Engl

(2016)

R. Wynford-Thomas et al.

Neurological update: MOG antibody disease

J Neurol

(2019)

S. Mariotto et al.

Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study

J Neurol

(2017)

A. Cobo-Calvo et al.

Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: the MOGADOR study

Neurology

(2018)

S. Ramanathan et al.

Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination

J Neurol Neurosurg Psychiatry

(2018)

M. Jurynczyk et al.

Clinical presentation and prognosis in MOG-antibody disease: a UK study

Brain

(2017)

S. Salama et al.

MOG antibody-associated encephalomyelitis/encephalitis

Mult Scler Houndmills Basingstoke Engl

(2019)

Cited by (74)

The Role of MRI in Differentiating Demyelinating and Inflammatory (not Infectious) Myelopathies
2023, Seminars in Ultrasound, CT and MRI
Demyelinating and inflammatory myelopathies represent a group of diseases with characteristic patterns in neuroimaging and several differential diagnoses. The main imaging patterns of demyelinating myelopathies (multiple sclerosis, neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, and myelin oligodendrocyte glycoprotein antibody-related disorder) and inflammatory myelopathies (systemic lupus erythematosus-myelitis, sarcoidosis-myelitis, Sjögren-myelitis, and Behçet's-myelitis) will be discussed in this article, highlighting key points to the differential diagnosis.
The Role of MRI in the Diagnosis of Spinal Cord Tumors
2023, Seminars in Ultrasound, CT and MRI
Spinal cord tumors are uncommon, and its multiple representatives not always have pathognomonic characteristics, which poses a challenge for both patients and caring physicians. The radiologist performs an important role in recognizing these tumors, as well as in differentiating between neoplastic and non-neoplastic processes, supporting clinical and surgical decision-making in patients with spinal cord injury. Magnetic Resonance Imaging (MRI) assessment, paired with a deep understanding of the various patterns of cord involvement allied to detailed clinical data can provide a diagnosis or significantly limit the differential diagnosis in most cases. In this article, we aim to review the most common and noteworthy intramedullary and extramedullary spinal tumors, as well as some other tumoral mimics, with an emphasis on their MRI morphologic characteristics.
The Diagnostic Challenge and Prognosis of Autoimmune Encephalitis in Children: A Single-Center Retrospective Study
2023, Pediatric Neurology
Autoimmune encephalitis (AE) is an immune-mediated encephalitis; nevertheless, its diagnosis in children remains challenging. This study aimed to reveal the clinical characteristics, diagnostic processes, and therapeutic outcomes of AE in children.
A total of 18 children with AE were enrolled. Antibody assay was performed in the cerebrospinal fluid (CSF) and serum samples by indirect immunofluorescence. Electroencephalography (EEG) and magnetic resonance imaging (MRI) were monitored to reflect abnormal neural signals. In addition, demographics data, neurological symptoms, therapeutic strategies, and outcomes were recorded and analyzed.
Convulsion (50.00%) and emotional disturbance (44.44%) were common clinical symptoms of AE. The biochemical parameters in the CSF had a relatively low diagnostic value. Antibodies in the CSF were dominant in the diagnosis of AE but those in the serum were limited in the diagnosis of anti-MOG or anti-LGI1 AE. In addition, all children showed abnormalities in EEG (72.22%) or MRI (66.67%). Methylprednisolone combined with sequential oral prednisone (83.33%) and gamma globulin (88.89%) were the dominant drugs, achieving an overall recovery rate of 72.22%. However, there were still two patients who had poor outcomes, including Patient 3 with a young age (two years old) and progressive symptom and Patient 12 with a long disease course before treatment (>120 days).
The clinical manifestations of AE are varied in children. Antibody in the CSF was dominant, and EEG and MRI were instructive in the diagnosis of AE. Young age, progressive symptom, and prolonged disease course before treatment may contribute to poor outcomes.
Multiple cortical lesions with grainy enhancement of magnetic resonance imaging in anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated encephalitis with seizures (FLAMES)
2023, Journal of the Neurological Sciences
Marked central canal T2-hyperintensity in MOGAD myelitis and comparison to NMOSD and MS
2023, Journal of the Neurological Sciences
To assess marked central canal T2-hyperintensity in patients with myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) myelitis compared to myelitis patients with aquaporin-4-antibody-positive neuromyelitis optica spectrum disorder (AQP4 + NMOSD) and multiple sclerosis (MS).
Two blinded raters evaluated spinal cord magnetic resonance imaging (MRIs) of myelitis patients with MOGAD (n = 63), AQP4 + NMOSD (n = 37), and MS (n = 26), assessing for marked central canal T2-hyperintensity and its evolution. If there were conflicting results, a third neurologist assessed the MRI.
Marked central canal T2-hyperintensity was more frequent in patients with MOGAD (18/63[29%]) than MS (1/26[4%]; p = 0.01) myelitis but did not differ from AQP4 + NMOSD (13/37[35%]; p = 0.49). Marked central canal T2-hyperintensity had completely resolved on follow-up axial MRI for most MOGAD (12/14[86%]) and AQP4 + NMOSD (10/10[100%]; p = 0.49) patients.
Marked central canal T2-hyperintensity is a common transient radiologic accompaniment of MOGAD and AQP4 + NMOSD myelitis, but not MS myelitis.
Clinical characteristics and prognosis of optic neuritis in Taiwan - a hospital-based cohort study
2023, Multiple Sclerosis and Related Disorders
Optic neuritis (ON) is an inflammatory disease of optic nerve. The distinct etiologies of ON significantly influence its clinical manifestation, neuroimaging findings, and visual outcomes. However, the clinical characteristics might be influenced by the racial differences. The purpose of this study is to investigate the clinical characteristics of various types of ON at a Taiwanese tertiary center.
This cohort study analyzed 163 patients who received treatment and continued following-up for ON between 2015 and 2022. We selected patients who had been tested for anti-aquaporin-4 antibody (AQP4-Ab) and anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab). The participants were classified into four groups on the basis of their etiologies, specifically (1) multiple sclerosis (MS)-related, (2) AQP4-Ab-positive, (3) MOG-Ab-positive, or (4) idiopathic ON. The researchers recorded the patients’ clinical characteristics, treatment course, magnetic resonance imaging and optical coherence tomography (OCT) findings, and visual outcomes.
MOG-Ab-positive group had higher percentages of disk swelling and pain with eye movement. Long optic nerve and perineural enhancement are the hallmarks of MOG-Ab-related ON. The ON relapse rate was higher in AQP4-Ab-positive group. Although members of AQP4-Ab-positive group received immediate steroid pulse therapy, these patients experienced the worst visual outcomes. Moreover, a thinner retinal nerve fiber layer (RNFL) was noted in AQP4-Ab-positive group. MS group had a higher incidence of extra–optic nerve lesions. Multivariate regression identified pretreatment visual acuity and RNFL thickness as the important factors affecting visual outcomes.
This cohort study identified the clinical features of different types of ON. Patients with AQP4-Ab-positive ON had poorer visual outcomes, which may be attributed to multiple relapses and profound nerve damage, as revealed by OCT findings. Patients with MOG-Ab-positive ON displayed long optic nerve enhancement but had more favorable prognoses. Thus, antibody-based classification facilitates treatment and prognosis in ON.

View all citing articles on Scopus

View full text

ReviewMRI features of demyelinating disease associated with anti-MOG antibodies in adults

Abstract

Section snippets

Background

Clinical and demographic characteristics

Optic nerve MRI findings in MOG-SD

Conclusion

Disclosure of interest

Mult Scler Relat Disord

J Neuroimmunol

Mult Scler Relat Disord

J Neuroradiol

J Neuroradiol

Clin Imaging

The spectrum of MOG autoantibody-associated demyelinating diseases

Nat Rev Neurol

Aquaporin-4 antibodies (NMO-IgG) as a serological marker of neuromyelitis optica: a critical review of the literature

Brain Pathol Zurich Switz

What proportion of AQP4-IgG-negative NMO spectrum disorder patients are MOG-IgG positive? A cross sectional study of 132 patients

J Neurol

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 Neuroinflammation

Myelin-oligodendrocyte glycoprotein antibodies in adults with a neuromyelitis optica phenotype

Neurology

Histopathology and clinical course of MOG-antibody-associated encephalomyelitis

Ann Clin Transl Neurol

Screening for MOG-IgG and 27 other anti-glial and anti-neuronal autoantibodies in “pattern II multiple sclerosis” and brain biopsy findings in a MOG-IgG-positive case

Mult Scler Houndmills Basingstoke Engl

Neurological update: MOG antibody disease

J Neurol

Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study

J Neurol

Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: the MOGADOR study

Neurology

Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination

J Neurol Neurosurg Psychiatry

Clinical presentation and prognosis in MOG-antibody disease: a UK study

Brain

MOG antibody-associated encephalomyelitis/encephalitis

Mult Scler Houndmills Basingstoke Engl

Review
MRI features of demyelinating disease associated with anti-MOG antibodies in adults