Case Series: Myelin Oligodendrocyte Glycoprotein-Immunoglobulin G-Related Disease Spectrum

Abstract

Introduction:Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)-related disease was initially described as a subtype of neuromyelitis optica spectrum disorder (NMOSD) with antibodies against MOG. However, it has recently been described as a separate disease entity with clinical and radiological features that overlap those of multiple sclerosis (MS) and NMOSD; the clinical features of this disease phenotype remain undetermined. We herein report the clinical presentation of nine MOG-IgG-positive patients, not all of whom fulfill the NMOSD criteria, in order to highlight the features and challenges of this condition. Method:We retrospectively reviewed the records of the London (Ontario) MS clinic to identify patients diagnosed with positive MOG antibodies based on the 2015 NMOSD consensus criteria.Result: Nine patients were identified, all Caucasian. Seven (78%) were female, and the median age of onset was 41 years (range, 28-69 years); the median Expanded Disability Status Scale score at onset was 3.0 (range, 2.0-4.0). A monophasic course was noted in two (22.2%) patients, while the median number of relapse events was 3 (range 2-5) in 77.8% of the patients. Optic neuritis and transverse myelitis contributed equally as initial manifestations in three individuals (33%), while brainstem relapse was reported in two individuals (22%). The brain magnetic resonance imaging findings were compatible with McDonald's 2010 dissemination in space criteria in three cases (33%). Short myelitis and an (H)-sign were each documented in one patient. Conclusion:The phenotypes of MOG Ab-positive cases exhibited overlapping features with MS and NMOSD. This finding highlights the importance of screening for anti-MOG in individuals with demyelinating symptoms, in consideration of the possibility of false-positive MOG Ab results.

Clinical and Radiological Features of Myelin Oligodendrocyte Glycoprotein-Associated Myelitis in Adults

“…Myelitis is the second-most-common presentation in adults with MOGAD, reportedly accounting for 18%–52% of cases, and the proportion increases up to 59% during the entire disease course. 19 27 28 34 35 36 37 38 39 More than half of myelitis attacks occur in an isolated form, and the remaining show a mixed phenotype that includes ON and/or brain involvement. 19 34 35 39 40 When MOG-IgG myelitis does not occur alone, it is mostly accompanied by ON.…”

“… 19 27 28 34 35 36 37 38 39 More than half of myelitis attacks occur in an isolated form, and the remaining show a mixed phenotype that includes ON and/or brain involvement. 19 34 35 39 40 When MOG-IgG myelitis does not occur alone, it is mostly accompanied by ON. 40 41 Isolated myelitis is a relatively unusual phenotype in pediatric patients with MOGAD, and literature focusing on pediatric MOG-IgG myelitis is sparse.…”

Myelin Oligodendrocyte Glycoprotein Antibody–Associated Optic Neuritis—A Review

“…Studies have assessed the relationship between MOG-IgG seropositivity and relapse. Titers at first episode do not correlate with relapse rate, a specific disease phenotype, or disability (18,42–45). The absolute titer level is highly variable between subjects and appears less relevant than a change in titer (30).…”

Central vein sign and other radiographic features distinguishing myelin oligodendrocyte glycoprotein antibody disease from multiple sclerosis and aquaporin-4 antibody-positive neuromyelitis optica

“…1,2 Several attempts have been made to identify imaging features that distinguish MOGAD from other CNS demyelinating illnesses. [3][4][5][6][7][8][9][10] Although some magnetic resonance imaging (MRI) findings can be suggestive of MOGAD rather than MS and NMOSD, 8,11 these findings are not present in all MOGAD patients. T2 hyperintense lesions in periventricular/subcortical brain regions and the optic nerves can be seen in both MOGAD and MS, and longitudinally extensive spinal cord lesions can be seen in both MOGAD and NMOSD, making these findings less useful for determining a diagnosis.…”