Bilateral optic neuritis as a presentation of myelin oligodendrocyte glycoprotein antibody associated disease in a child

Sai Lakshmi Raghavan; Rajesh Prabu; Shruti Mohan; Suruthi Nagarajan

doi:10.4103/ijo.IJO_1803_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 1 | Page : 118-120

Bilateral optic neuritis as a presentation of myelin oligodendrocyte glycoprotein antibody associated disease in a child

Sai Lakshmi Raghavan, Rajesh Prabu, Shruti Mohan, Suruthi Nagarajan
Department of Pediatric Ophthalmology and Strabismology, Sankara Eye Hospital, Coimbatore, Tamil Nadu, India

Date of Submission	29-Jul-2022
Date of Acceptance	14-Sep-2022
Date of Web Publication	20-Jan-2023

Correspondence Address:
Sai Lakshmi Raghavan
Department of Pediatric Ophthalmology and Strabismology, Sankara Eye Hospital, Coimbatore - 641 035, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1803_22

Abstract

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), although rare, is now recognized as a distinct demyelinating disorder. The entity differs from multiple sclerosis and neuromyelitis optica spectrum disorder in terms of clinical presentation, radiodiagnostic features, and prognosis. Here, we present a 5-year-old female who presented with bilateral, painless loss of vision since 3 days. There was optic nerve enhancement, but no brain parenchymal lesions. She tested negative for Aquaporin-4 antibodies, and CSF analysis showed no oligoclonal bands. Serum analysis revealed positivity for myelin oligodendrocyte antibodies. There was full visual recovery on treatment with systemic steroids. The report highlights the need for testing for MOG–IgG antibodies, especially in children with bilateral optic neuritis. It is essential to differentiate the entity from other demyelinating conditions. The prognosis is usually benign.

Keywords: Multiple sclerosis, myelin oligodendrocyte glycoprotein, optic neuritis

How to cite this article:
Raghavan SL, Prabu R, Mohan S, Nagarajan S. Bilateral optic neuritis as a presentation of myelin oligodendrocyte glycoprotein antibody associated disease in a child. Indian J Ophthalmol Case Rep 2023;3:118-20

How to cite this URL:
Raghavan SL, Prabu R, Mohan S, Nagarajan S. Bilateral optic neuritis as a presentation of myelin oligodendrocyte glycoprotein antibody associated disease in a child. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Feb 1];3:118-20. Available from: https://www.ijoreports.in/text.asp?2023/3/1/118/368171

Optic neuritis (ON) in children is commonly a presentation of neuromyelitis optica spectrum disorder and multiple sclerosis. Recently, ON has also been attributed to myelin oligodendrocyte antibody disease (MOGAD). MOGAD is an idiopathic, inflammatory, demyelinating disease of the CNS, clinically and prognostically distinct from multiple sclerosis and neuromtelitica spectrum disorder (NMOSD).^[1]

The hallmark of the disease is the detection of antibodies formed against myelin oligodendrocyte protein (MOG). The development of specific cell-based assays has made it possible to identify a subset of patients with MOG antibodies.

The clinical spectrum of MOGAD is varied, including recurrent ON, transverse myelitis, and acute disseminated myelitis (ADEM).^[2]

The incidence of MOGAD is 0.16 per 1,00,000 individuals, with children accounting for half the reported cases.^[3]

Here we present a rare case of bilateral MOG-ON in a 5-year-old child.

Case Report

A 5-year-old girl presented with sudden-onset, painless diminution of vision in both eyes for 2 days. She did not exhibit signs of raised intracranial tension, alteration in consciousness, or limb weakness. There was no history of fever or any prior infection. The corrected distance visual acuity (CDVA) was counting fingers in both eyes. Color vision was also impaired. On examination, the anterior segment showed a clear cornea, anterior chamber of normal depth, sluggishly reacting pupil, and clear lens in both eyes. The posterior segment showed a normal-appearing disc and macula [Figure 1]a and [Figure 1]b. A neurologist's opinion was sought.

Figure 1: (a) and (b): Fundus photographs showing optic disc of normal size, color, and well-defined margins in both eyes, suggestive of retrobulbar optic neuritis

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Baseline blood investigations, including erythrocyte sedimentation rate (ESR) levels, were normal. She tested negative for serum antinuclear antibodies (ANA) and syphilis. Liver and renal parameters were within normal limits.

CSF analysis revealed elevated glucose levels, absence of nucleated cells, and no oligoclonal bands.

MRI brain and orbit showed optic nerve enhancement suggestive of ON. There was no involvement of the optic chiasm or the brain parenchyma [Figure 2].

Figure 2: MRI with contrast: Bilateral prominent enhancement of optic nerve and sheath (perineural enhancement)

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However, serum examination showed positivity for MOG antibodies.

She was treated with intravenous methylprednisolone 500 mg twice a day for a duration of 3 days, followed by a tapering dose of oral steroids for a total period of 2.5 months.

The patient reported improved vision starting day 4 of treatment. At the 1-week follow-up, her visual acuity improved to 6/6 in both eyes. She did not show any signs of relapse until the present follow-up, after 3 months following her first episode.

Discussion

ON is the second most common presentation of MOGAD in children after ADEM.

Classically, the presentation is that of severe vision loss with papilledema. However, most patients have complete visual recovery. Neuroimaging in MOG-ON-positive patients shows the involvement of the anterior orbital segment of the optic nerve without chiasmal involvement. There are typically high MOG antibody titers (median- 1:1280), slightly elevated CSF glucose levels, and an absence of oligoclonal bands. Relapses are common with the disease entity. Children with a polysymptomatic disease and higher antibody titers are at greater risk for relapse.^[1],[2],[4]

For prognostication and appropriate management, it is essential to differentiate MOG-ON from MS and NMOSD.

MS ON occurs more commonly in children aged 10 years and above, preferentially presenting with unilateral ON. Unlike MS, brain lesions in MOG-IgG patients are less likely to enhance with gadolinium, are poorly defined, and are not oriented perpendicularly to the major axis of the corpus callosum. Furthermore, the presence of oligoclonal bands on CSF analysis is more in favor of progression to MS in the future. Relapses are treated with intravenous glucocorticoids, along with disease-modifying agents such as interferon beta and glatiramer acetate. The latter do not seem to influence the disease severity or the recurrence in MOGAD.^[5],[6],[7]

Unlike MOG-ON, visual recovery is poorer in NMOSD-ON. Neuroimaging shows longitudinally extensive optic nerve enhancement, along with chiasmal involvement. Another characteristic feature of NMOSD is the presence of transverse myelitis (which is defined as spinal cord involvement of more than three vertebral segments). Brain lesions occur in the areas of high Aquaporin P4 expression, such as the ependymal cells, hypothalamus, and brainstem.^[8]

Although the clinical presentation of MOG-ON can overlap with NMOSD, double positivity is extremely rare. In addition, the detection of anti-MOG antibodies in a patient with MS is coined a “red flag” that prompts testing for alternate diagnoses.

The clinical profile of our patient fits that of MOG-ON, the one differentiating factor being a normal-appearing disc and the relatively young age of presentation, where ADEM is more common. She had complete visual recovery, although there have been reports of persisting visual defect and optic atrophy in recurrent ON.^[2]

This is a case of monophasic ON, where the child did not show any neurological signs. Several case reports have highlighted the occurrence of ON following ADEM.^[9] It is not unusual for MOG-ON to present with seizures and meningoencephalitis in children. [Table 1] gives a summary of various clinical presentations of MOG ON in children.^[10]

Table 1: A summary of the various case reports in literature citing myelin oligodendrocyte-associated optic neuritis

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To the best of our knowledge, this is the first report of a monophasic bilateral ON attributed to antibodies against myelin oligodendrocyte protein in a child in India.

Conclusion

The case report highlights the need for high suspicion of MOG-ON in children who present bilaterally with sudden, acute vision loss. It is essential to differentiate MOG ON from MS and NMOSD for appropriate management. The disease is found to have a benign prognosis despite frequent relapses.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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