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Year : 2021  |  Volume : 59  |  Issue : 1  |  Page : 77-79

Recurrent bilateral optic neuritis associated with neuromyelitis optica spectrum disorder in a child

Department of Neuro-Ophthalmology, Aravind Eye Hospital, Salem, Tamil Nadu, India

Date of Submission06-Sep-2020
Date of Acceptance01-Jan-2021
Date of Web Publication27-Mar-2021

Correspondence Address:
Dr. Alap Jayesh Bavishi
Department of Neuro-Ophthalmology, Aravind Eye Hospital, Sankagiri Main Road, Nethimedu, Salem - 636 002, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_132_20

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Reports of bilateral optic neuritis in children associated with viral infection or postimmunization are relatively common. Neuromyelitis optica spectrum disorders (NMOSD) is a rare syndrome of severe inflammatory immune-mediated demyelination of central nervous system, with median age of presentation being 37.5 years reported in south Indian population. NMOSD associated with optic neuritis in children is very rare. We present a case of 10-year-old girl with bilateral recurrent optic neuritis, seropositive for NMO-IgG (anti-AQP4) supporting the diagnosis of NMOSD. With prompt treatment, patient made a remarkable visual recovery without neurologic deficit. This report highlights prompt diagnosis, leading to better visual outcomes in NMOSD.

Keywords: Immune-mediated demyelination, neuromyelitis optica spectrum disorder, NMO-IgG, optic neuritis

How to cite this article:
Bavishi AJ, Sinha S, Balasundaram M. Recurrent bilateral optic neuritis associated with neuromyelitis optica spectrum disorder in a child. TNOA J Ophthalmic Sci Res 2021;59:77-9

How to cite this URL:
Bavishi AJ, Sinha S, Balasundaram M. Recurrent bilateral optic neuritis associated with neuromyelitis optica spectrum disorder in a child. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Jul 28];59:77-9. Available from: https://www.tnoajosr.com/text.asp?2021/59/1/77/312289

  Introduction Top

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system characterized by severe, immune-mediated demyelination predominantly targeting optic nerves and spinal cord. It can be monophasic (single episode) or polyphasic (recurrent). Currently, three entities are proposed based on the presence of autoantibodies thought to produce the disease: anti-AQP4, anti-MOG, and anti-NF. Hence, now, NMO is called as “Neuromyelitis optica spectrum disorders (NMOSD).”[1] In pediatric age group, only a few cases of recurrent bilateral optic neuritis associated with neuromyelitis optica spectrum disorder (NMOSD) with positive NMO-IgG (anti-AQP4) are reported till date.

  Case Report Top

We present a case of a 10-year-old girl with bilateral recurrent optic neuritis diagnosed as neuromyelitis optica spectrum disorder (NMOSD). The patient presented with best-corrected visual acuity (BCVA) right eye (RE) hand motion and left eye (LE) 20/200 at the age of 8. She was diagnosed with bilateral optic neuritis based on her initial presentation with bilateral disc edema [Figure 1] and supportive imaging evidence on magnetic resonance imaging (MRI) orbit. Treatment with intravenous methylprednisolone acetate 250 mg once daily for 3 days led to a remarkable improvement, following which she was asymptomatic and lost to follow-up. A couple of years later, at the age of 10, she presented with pain and blurred vision in LE with BCVA of 20/200 in BE. Ocular findings include a relative afferent pupillary defect Grade III with grayish optic disc pallor suggestive of secondary optic atrophy in RE. LE presented with a sluggish ill-sustained reaction of the pupil with blurred nasal disc margins and temporal pallor [Figure 2] She did not report any other neurological deficits. Basic blood investigations were within normal limits with nil systemic history. MRI brain and orbit suggests bilateral optic neuritis [Figure 3]. Spinal MRI was also done which was normal. A neurologist opinion was sought for a detailed evaluation and serological testing. She was diagnosed as bilateral optic neuritis secondary to NMOSD with MRI findings of optic neuritis and positive test for NMO-IgG (anti-AQP4) without any other neurological deficits. She was treated with 250 mg once daily intravenous methylprednisolone acetate for 5 days, followed by oral prednisolone 15 mg once a day for 11 days with further taper. A remarkable improvement in LE was noted with a BCVA of 20/20 without any neurologic deficit. The patient was again followed up and referred to neurologist for further treatment to prevent recurrence.
Figure 1: (a) Color fundus photograph of right eye showing swollen optic disc with blurred margins. (b) Color fundus photograph of left eye showing swollen optic disc with blurred margins. Both suggestive of optic disc edema

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Figure 2: Color fundus photograph of right eye showing greyish optic disc pallor suggestive of secondary optic atrophy (a). Color fundus photograph of left eye showing swollen disc with blurred disc margins nasally and temporal pallor (b)

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Figure 3: Magnetic resonance imaging brain and orbit revealed hyperintensity in intraconal segments of bilateral optic nerves in the coronal section of T2 weighted image suggestive of bilateral optic neuritis

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  Discussion Top

Neuromyelitis optica spectrum disorder (NMOSD) previously considered as NMO or Devic's disease is a rare disorder. Our patient reported here was diagnosed as bilateral optic neuritis secondary to NMOSD. Core clinical characteristics of NMOSD include MRI findings related to optic neuritis or acute myelitis or area postrema syndrome and positive test for NMO-IgG (anti-AQP4) according to the latest diagnostic criteria for NMOSD with AQP4-IgG by the International Panel for NMO Diagnosis.[1]

The incidence and prevalence of NMOSD vary between populations and geographic region. The mean age at NMO presentation ranges from 32.6 to 45.7 years in major case series in world, but median age of presentation being 37.5 years, ranging from 12 to 65 years reported in south Indian population. Population-based studies from Europe, Asia, and South America suggest that the incidence of NMO ranges from 0.05 to 4/100,000 and the prevalence from 0.52 to 4.4/100,000 per year. In southern India, prevalence rate was 2.7/100,000. Nevertheless, most of these studies have not stratified data by pediatric or adult age at onset. Pediatric onset is relatively rare.[2]

In both NMOSD and multiple sclerosis (MS), the body's immune system attacks the myelin surrounding nerve cells. Unlike standard MS, the attacks are not believed to be mediated by the immune system T cells but rather by antibodies called NMO-IgG which is B-cell-mediated immune system.[3] Clinical course of NMOSD is variable. It may occur as a monophasic illness that is either fulminant or associated with varying degrees of recovery. Pediatric cases typically have a monophasic course and many have complete neurological recovery. Polyphasic courses characterized by relapses and remissions also occur; the main symptoms include loss of vision and spinal cord function.[1] Wingerchuk et al. reported NMO-IgG (anti-AQP4) seropositivity in childhood inflammatory demyelinating disorders to be 78% in children with relapsing NMO against 12.5% in monophasic cases.[4] In young children with relapsing disease and central lesions, MS may be a diagnostic consideration. Our patient never developed symptoms implicating other CNS regions outside the optic nerves. More importantly, NMO-IgG titers would not have been identified in cases of pediatric relapsing-remitting MS.[4]

There are no specific guidelines for NMOSD treatment at present.

Specific medications that have been used include intravenous methylprednisolone with oral prednisolone, immunosuppressants such as azathioprine,[5],[6] cyclophosphamide,[6],[7] and rituximab,[8],[9] and plasmapheresis.[10] Rituximab is a murine/human monoclonal antibody directed against CD20 that induces B-cell depletion when administered in vivo. It is approved by the FDA for the treatment of CD20+ lymphomas and rheumatoid arthritis. It is attractive and more effective approach for treating NMOSD.[10]

  Conclusion Top

Neuromyelitis optica spectrum disorder (NMOSD) is a rare neurological disorder, especially in the pediatric age group. Cases with recurrent bilateral optic neuritis should be evaluated thoroughly for systemic demyelinating conditions even in pediatric population. This case report helps to create awareness among general, pediatric and, neuro-ophthalmologist about this rare entity called NMOSD with a poor prognosis and management difficulties. This case report should serve as an example for prompt diagnosis, long-term follow-up, and prevention of recurrence in pediatric NMOSD.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given her consent for images and other clinical information to be reported in the journal. The guardian understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.


We would like to thank Dr. Puneeth C S, Neurologist, Gokulam Hospital, Salem and Dr. Venkatesh, Radiologist, Mithra Scan, Salem, for aiding in prompt diagnosis and management of this patient.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 2015;85:177-89.  Back to cited text no. 1
Pandit L, Asgari N, Apiwattanakul M, Palace J, Paul F, Leite MI, et al. Demographic and clinical features of neuromyelitis optica: A review. Mult Scler 2015;21:845-53.  Back to cited text no. 2
Kliegman R, Stanton B, St. Geme JW, Schor NF, Behrman RE. Nelson Textbook of Pediatrics. 20th ed.. Phialdelphia, PA: Elsevier; 2016.  Back to cited text no. 3
Wingerchuk DM. Neuromyelitis optica. Int MS J 2006;13:42-50.  Back to cited text no. 4
Petelin Gadze Z, Hajnsek S, Basic S, Sporis D, Pavlisa G, Nankovic S. Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: Case report. BMC Neurology 2009;9:56.  Back to cited text no. 5
Djemal N, Ben Salah M, Ben Hlima N, Ben Rhomdhane W, Ben Ammar H, Chaabouni M, et al. Devic's neuromyelitis optica in children: A case report and review of the literature. Arch Pediatr 2007;14:1337-40.  Back to cited text no. 6
Chia WC, Wang JN, Lai MC. Neuromyelitis optica: A case report. Pediatr Neonatol 2010;51:347-52.  Back to cited text no. 7
Pellkofer HL, Krumbholz M, Berthele A, Hemmer B, Gerdes LA, Havla J, et al. Long-term follow-up of patients with neuromyelitis optica after repeated therapy with rituximab. Neurology2011;76:1310-5.  Back to cited text no. 8
Mahmood NA, Silver K, Onel K, Ko M, Javed A. Efficacy and safety of rituximab in pediatric neuromyelitis optica. J Child Neurol 2011;26:244-7.  Back to cited text no. 9
National Institute of Neurological Disorders and Stroke (NINDS), Neuromyelitis Optica Information Page. Available from: http://www.ninds.nih.gov/disorders/neuromyelitis_optica/neuromyelitis_optica.htm. [Last accessed on 2020 Jun 05].  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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