Edit an article: Myelin Oligodendrocyte Glycoprotein (MOG) Optic Neuritis

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= Disease Entity = == Disease == Myelin Oligodendrocyte Glycoprotein (MOG) Optic Neuritis is an antibody mediated demyelinating disease of the central nervous system (CNS) that is a distinct entity from other demyelinating processes of the CNS such as Multiple Sclerosis (MS) or AQP4-Ab-associated neuromyelitis optica spectrum disorder (NMOSD).<ref name="reindl1">Reindl M, Waters P. Myelin oligodendrocyte glycoprotein antibodies in neurological disease. Nat Rev Neurol. 2019;15(2):89-102. doi:10.1038/s41582-018-0112-x</ref> Typical optic neuritis (ON) presents with acute, unilateral, onset of variable visual acuity/visual field loss, retrobulbar pain (worse with eye movement), loss of color vison, a relative afferent pupillary defect (RAPD), and a normal fundus exam (retrobulbar ON).<ref name="in2">in cooperation with the Neuromyelitis Optica Study Group (NEMOS), Jarius S, Ruprecht K, 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. doi:10.1186/s12974-016-0718-0</ref><ref name="chen3">Chen JJ, Flanagan EP, Jitprapaikulsan J, et al. Myelin Oligodendrocyte Glycoprotein Antibody–Positive Optic Neuritis: Clinical Characteristics, Radiologic Clues, and Outcome. Am J Ophthalmol. 2018;195:8-15. doi:10.1016/j.ajo.2018.07.020</ref> ON in MOG however is often “atypical” and may be markedly steroid responsive, bilateral rather than unilateral, and may be associated with optic disc edema rather than a retrobulbar ON.<ref name="chen3"/><ref name="jurynczyk4">Jurynczyk M, Messina S, Woodhall MR, et al. Clinical presentation and prognosis in MOG-antibody disease: a UK study. Brain. 2017;140(12):3128-3138. doi:10.1093/brain/awx276</ref><ref name="in5">in cooperation with the Neuromyelitis Optica Study Group (NEMOS), Jarius S, Ruprecht K, 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;13(1):279. doi:10.1186/s12974-016-0717-1</ref> Although ON is the most common symptom in MOG-Ab seropositive disease it can present with acute disseminated encephalomyelitis, myelitis, or an NMOSD like presentation.<ref name="reindl1"/><ref name="in2"/> Children less than 9 years old who are positive for MOG-Antibodies (Ab) more frequently present with acute disseminated encephalomyelitis (ADEM) that may be relapsing or recurrent and may present with ON later in life.<ref name="reindl1"/><ref name="jurynczyk4"/> == Etiology == MOG related ON may be associated with certain HLA haplotypes.<ref name="reindl1"/> There is wide variability in the epidemiology, severity of acute vision loss, extraocular neurologic deficits, and prognosis seen in various different studies of MOG however.<ref name="in2"/><ref name="chen3"/><ref name="ducloyer6">Ducloyer J-B, Caignard A, Aidaoui R, et al. MOG-Ab prevalence in optic neuritis and clinical predictive factors for diagnosis. Br J Ophthalmol. October 2019:bjophthalmol-2019-314845. doi:10.1136/bjophthalmol-2019-314845</ref> == Risk Factors == MOG ON can be seen in any age,<ref name="reindl1"/><ref name="in2"/><ref name="jurynczyk4"/> any ethnicity, and in either gender. In contrast to NMOSD which is more frequently seen in African and Asian ancestry.<ref name="in2"/><ref name="in7">in cooperation with the Neuromyelitis Optica Study Group (NEMOS), Pache F, Zimmermann H, et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 4: Afferent visual system damage after optic neuritis in MOG-IgG-seropositive versus AQP4-IgG-seropositive patients. J Neuroinflammation. 2016;13(1):282. doi:10.1186/s12974-016-0720-6</ref> Children with MOG ADEM had up to an 80% chance of developing MOG ON in adolescence or adulthood in one study. <ref name="reindl1"/> Other studies have shown contradictory associations between sex and MOG ON.<ref name="in2"/><ref name="chen3"/><ref name="ducloyer6"/> One study found that 50% of pregnant women with MOG-ab’s developed their first episode of MOG-ON during or shortly after pregnancy.<ref name="in2"/> There was no association between MOG-Ab ON and other autoimmune disorders and MOG ON is not typically associated with oligoclonal bands, a test specific for MS.<ref name="in2"/><ref name="chen3"/><ref name="jurynczyk4"/> == Pathophysiology == The pathophysiology of MOG ON is incompletely understood and the precise mechanism of MOG-Ab remains ill defined. <ref name="reindl1"/> In addition, human MOG-Ab’s isolated from patient serum do not bind to MOG proteins expressed in rodents, therefore an in vivo study of the pathogenic role of these proteins has not been completely established.<ref name="reindl1"/> MOG protein is found on the surface of oligodendrocytes and known to bind to C1q, IgG Ab’s, DC-SIGN, a c-type lectin receptor expressed on the surface of dendritic cells and macrophages, rubella virus, and nerve growth factor. Disease-causing MOG antibodies are IgG1, which are known to activate the classic complement pathway. Complement deposition has also been seen in neuroimaging studies during acute attacks of MOG-Ab ON.<ref name="reindl1"/> = Diagnosis = == Physical examination == Moderate to severe optic disc edema is a classic differentiating feature of MOG ON from MS and other forms of ON seen in up to 80% of patients.<ref name="in2"/><ref name="ducloyer6"/><ref name="shor8">Shor N, Aboab J, Maillart E, et al. Clinical, imaging and follow‐up study of optic neuritis associated with myelin oligodendrocyte glycoprotein antibody: a multicentre study of 62 adult patients. Eur J Neurol. 2020;27(2):384-391. doi:10.1111/ene.14089</ref> Although MOG ON in older patients has some overlapping features consistent w/ non-arteritic anterior ischemic neuropathy (NAION) usually the differentiation can be made clinically or radiographically. Bothe NAION and MOG ON may present with optic disc edema and peripapillary hemorrhage but NAION is much more commonly seen in older, vasculopathic patients compared to MOG-Ab positive ON which is seen in younger and healthier adults.<ref name="in2"/><ref name="chen9">Chen JJ, Bhatti MT. Clinical phenotype, radiological features, and treatment of myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) optic neuritis: Curr Opin Neurol. November 2019:1. doi:10.1097/WCO.0000000000000766</ref> == Signs == Acute unilateral or bilateral, steroid responsive, recurrent episodes of ON are the hallmark of MOG-Ab ON.<ref name="in2"/><ref name="chen3"/><ref name="ducloyer6"/> MOG ON episodes are often recurrent and the presentation with MOG antibody at the first attack had a positive predictive value on the presentation of subsequent attacks.<ref name="in2"/> MOG ON can be associated with rapidly progressive and severe vision loss<ref name="in2"/><ref name="chen3"/> which may or may not recover. Commonly reported other neurologic signs and symptoms include myelitis (e.g., paralysis and paresthesia) as well as encephalopathy, seizures, incontinence, and dysarthria.<ref name="in2"/><ref name="chen3"/><ref name="jurynczyk4"/> == Symptoms == Symptoms that are highly suggestive of MOG-Ab ON include bilateral and recurrent episodes of ON<ref name="in2"/><ref name="chen3"/><ref name="in5"/> especially in the setting of steroid responsiveness and steroid dependence. A recent study demonstrated that children with MOG are most likely to present with ADEM, adults aged 20 to 45 are most likely to present with unilateral ON, and adults older than 45 are most likely to present with bilateral ON. <ref name="jurynczyk4"/> Other neurologic deficits are seen in about 50% of cases but the presentation is highly variable from study to study (e.g., transverse myelitis, sensory nerve loss, incontinence, and ataxia).<ref name="in2"/><ref name="chen3"/> == Clinical diagnosis == Definitive diagnosis of MOG ON is made in the appropriate clinical setting by seropositivity of MOG-Ab (cell-based assays are the current gold standard).<ref name="reindl1"/><ref name="chen3"/> One prospective cohort study of patients with ON showed that by testing all cases of ON with bilateral ON, recurrent ON, or optic disc swelling on fundoscopy for MOG antibody, all cases of MOG ON would be detected and only 50% of ON cases in the cohort would be tested overall.<ref name="ducloyer6"/> An absence of each of the atypical ON features that were considered to be higher risk for MOG had a negative predictive value of over 90%.<ref name="ramanathan10">Ramanathan S, Mohammad S, Tantsis E, et al. Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. J Neurol Neurosurg Psychiatry. 2018;89(2):127-137. doi:10.1136/jnnp-2017-316880</ref> MRI findings (e.g., OPN, lack of demyelinating white matter lesions for MS, longitudinally extensive enhancement) are also highly suggestive of MOG ON<ref name="in2"/><ref name="chen3"/><ref name="ducloyer6"/> == Imaging== Longitudinally, extensive (involving >50% of the length of the optic nerve) enhancement and /or T2 weighted hyperintensities in the anterior visual pathways can be seen in MOG-Ab ON.<ref name="in2"/><ref name="chen3"/><ref name="ducloyer6"/> Enhancing lesions are usually seen in the orbital and intracranial region of the optic nerve.<ref name="in2"/><ref name="chen3"/> Optic perineuritis (OPN) with enhancement the optic nerve sheath is a finding that is common in MOG ON as compared to MS related ON (parenchymal optic nerve enhancement). <ref name="in2"/><ref name="chen3"/><ref name="ducloyer6"/> Spinal MRI findings include swelling of the spinal cord and contrast enhancement<ref name="jurynczyk4"/> that can resemble the transverse myelitis of NMOSD. In contrast to the ovoid, periventricular, white matter lesions (including corpus callosum) seen in MS, non-specific white matter lesions or a normal brain MRI may be seen in MOG == Laboratory test == Cell based assays are still recommended for a serologic diagnosis of MOG-Ab. Very few patients with MOG ON have been found to have the CSF oligoclonal bands seen with MS.<ref name="reindl1"/><ref name="jurynczyk4"/> == Differential diagnosis == * MS * NMOSD (caused by AQP4 IgG) * NAION * Sarcoidosis * Lyme Disease * Granulomatosis with polyangiitis (Wegner granulomatosis) = Management = == Treatment == The widely accepted treatment for acute attacks has been intravenous, high dose corticosteroid therapy (e.g., methylprednisolone) followed by intravenous immunoglobulin (IVIG) or plasma exchange in patients who do not respond to IV steroid treatment. <ref name="in2"/> Methylprednisolone has been shown to increase recovery by 10-20% compared to no treatment.<ref name="in2"/><ref name="chen9"/> Of the patients who were not responsive to methylprednisolone, 40% showed improvement with IVIG, although partial recovery was more common than full recovery with IVIG.<ref name="in2"/> While treatment efficacy may be low in acute attacks, there has been substantial success in preventing recurrent attacks. In one study, 95% of patients receiving doses of at least 20 mg of prednisone for 6 months following a MOG ON attack had no recurrent episodes of ON at follow up of over a year.<ref name="ramanathan10"/> High dose and longer length of treatment was strongly associated with remittance, and patience who were given a tapered dose, or discontinued therapy earlier had relapse rates comparable to those with no treatment.<ref name="in2"/><ref name="ramanathan10"/> Randomized, controlled, treatment trials are limited for MOG ON, but observational open-label work suggests a role for high-dose steroids and plasma exchange in the treatment of acute attacks, and immunosuppressive therapy (e.g., steroids, oral immunosuppressants and rituximab) as maintenance treatment. == Prognosis == In one study, 25% of patients suffering an acute attack had permanent visual disability.<ref name="reindl1"/><ref name="jurynczyk4"/> In other studies, patients experienced complete or partial recovery in up to 90% of cases.<ref name="in2"/><ref name="chen3"/> Although MOG ON attacks have been show to permanently damage the neural retinal ganglion and peripheral nerve fiber layers of the retina, the majority of patients fully or partially regain vision.<ref name="in2"/><ref name="in7"/> The majority of individuals in all studies have a recurrence within the first year of attack.<ref name="in2"/><ref name="chen3"/> Increased risk of permanent neurologic deficit with subsequent attacks is much worse than after the first attacks.<ref name="jurynczyk4"/> Younger age at first attack is associated with increased risk of permanent vision loss.<ref name="jurynczyk4"/> Persistent MOG-Ab titers have been associated with relapse compared with patients without persistent MOG-Ab. <ref name="reindl1"/><ref name="jurynczyk4"/> = References = <references />
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