Highlights
- •Patients with MOG-AD may have a monophasic or relapsing disease course.
- •57% of our cohort had a relapsing course.
- •The most common relapse phenotype was optic neuritis.
- •Neither age, gender, race, initial attack, or antibody titer predicted relapse risk.
- •50% of patients on disease modifying therapy continued to relapse.
Abstract
Background
While many patients with myelin oligodendrocyte glycoprotein antibody-mediated disease
(MOG-AD) will have a monophasic course, 30-80% of patients will relapse after the
initial attack. It is not known which factors predict relapse. Here we describe our
clinical experience with MOG-AD and evaluate for factors that correlate with relapsing
disease.
Methods
This was a retrospective, multi-institutional study of 54 patients with MOG-AD, including
17 children and 37 adults. Mann-Whitney U and Fischer's Exact tests were used for
comparisons and logistic regression for correlations.
Results
Incident attack phenotype included acute disseminated encephalomyelitis (15%), unilateral
optic neuritis (ON; 39%), bilateral ON (24%), transverse myelitis (TM; 11%) and ON
with TM (11%). Pediatric patients were more likely than adults to present with ADEM
(p = .009) and less likely to present with unilateral ON (p = .04). 31 patients (57%) had a relapsing disease course, with time to first relapse
of 8.2 months and median annualized relapse rate of 0.97 months. In 40% of patients
(n = 22) the first relapse occurred following the withdrawal of treatment for the incident
attack. 5 patients converted to seronegative at follow up, 2 of whom later relapsed.
Logistic regression revealed no significant relationship between age, gender, race,
presentation phenotype, antibody titer, or cerebrospinal fluid results with risk of
relapse. For patients who started disease modifying therapy (DMT) prior to the first
relapse (n = 11), 64% remained monophasic. 50% (n = 15) of patients on DMT continued to have disease activity, requiring treatment adjustment.
Conclusions
It is difficult to predict which patients with MOG-AD will relapse. Research is needed
to determine the optimal timing and choice of treatment.
Keywords
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References
- Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study.Lancet Neurol. 2020; 19: 234-246
- A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group.N. Engl. J. Med. 1992; 326: 581-588
- Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder.Neurology. 2020; 95: e111-e120
- Usefulness of MOG-antibody titres at first episode to predict the future clinical course in adults.J. Neurol. 2019; 266: 806-815
- Evaluation of treatment response in adults with relapsing MOG-Ab-associated disease.J. Neuroinflammation. 2019; 16: 134
- Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specific in children with acquired demyelinating syndromes.Dev. Med. Child Neurol. 2018; 60: 958-962
- Silent new brain MRI lesions in children with MOG-antibody associated disease.Ann. Neurol. 2021; 89: 408-413
- Disease course and treatment responses in children with relapsing myelin oligodendrocyte glycoprotein antibody–associated disease.JAMA Neurol. 2018; 75: 478-487
- Seizures and encephalitis in myelin oligodendrocyte glycoprotein IgG disease vs aquaporin 4 IgG disease.JAMA Neurol. 2018; 75: 65-71
- MOG spectrum disorders and role of MOG-antibodies in clinical practice.Neuropediatrics. 2018; 49: 3-11
- Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome.Neurology. 2017; 89: 900-908
- 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: 280
- 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; 13: 279
- Clinical presentation and prognosis in MOG-antibody disease: a UK study.Brain. 2017; 140: 3128-3138
- Association of MOG-IgG serostatus with relapse after acute disseminated encephalomyelitis and proposed diagnostic criteria for MOG-IgG-associated disorders.JAMA Neurol. 2018; 75: 1355-1363
- Persistent MOG-IgG positivity is a predictor of recurrence in MOG-IgG-associated optic neuritis, encephalitis and myelitis.Mult. Scler. 2019; 25: 1907-1914
- Anti-MOG antibody: The history, clinical phenotype, and pathogenicity of a serum biomarker for demyelination.Autoimmun. Rev. 2016; 15: 307-324
- Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination.J. Neurol. Neurosurg. Psychiatry. 2018; 89: 127-137
- Myelin oligodendrocyte glycoprotein antibodies in neurological disease.Nat. Rev. Neurol. 2019; 15: 89-102
- Encephalitis, myelitis, and acute disseminated encephalomyelitis (ADEM): case definitions and guidelines for collection, analysis, and presentation of immunization safety data.Vaccine. 2007; 25: 5771-5792
- Acute disseminated encephalomyelitis.Neurology. 2007; 68: S23-S36
- Proposed diagnostic criteria and nosology of acute transverse myelitis.Neurology. 2002; 59 (12236201): 499-505https://doi.org/10.1212/WNL.59.4.499
- Serial Anti-Myelin Oligodendrocyte Glycoprotein Antibody Analyses and Outcomes in Children With Demyelinating Syndromes.JAMA Neurol. 2020; 77: 82-93
- Defining distinct features of anti-MOG antibody associated central nervous system demyelination.Ther. Adv. Neurol. Disord. 2018; 111756286418762083
- Treatment of MOG-IgG-associated disorder with rituximab: an international study of 121 patients.Mult. Scler. Relat. Disord. 2020; 44102251
- International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.Neurology. 2015; 85: 177-189
- Neurological update: MOG antibody disease.J. Neurol. 2019; 266: 1280-1286
- MOG-antibody associated demyelinating disease of the CNS: a clinical and pathological study in Chinese Han patients.J. Neuroimmunol. 2017; 305: 19-28
Article info
Publication history
Published online: August 28, 2021
Accepted:
August 26,
2021
Received in revised form:
July 23,
2021
Received:
January 9,
2021
Identification
Copyright
© 2021 Elsevier B.V. All rights reserved.