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Year : 2017  |  Volume : 5  |  Issue : 2  |  Page : 100-104

Sequential bilateral retrobulbar optic neuritis in a young female

Department of Ophthalmology, IGMC, Shimla, Himachal Pradesh, India

Date of Submission11-Mar-2016
Date of Acceptance28-Nov-2016
Date of Web Publication25-Apr-2017

Correspondence Address:
Deepti Mahajan
Indira Gandhi Medical College, Shimla, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-3897.205182

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Optic neuritis (ON) is the demyelinating inflammation of the optic nerve head usually associated with multiple sclerosis. It is usually associated with sudden onset loss of vision with dyschromatopsia and retro-orbital pain. Atypical features for a demyelinating ON include the absence of pain, severe visual loss, progression of visual loss, pain for more than 2 weeks, and lack of recovery after 3 weeks. Atypical features in fundus examination include marked swelling of the nerve with retinal exudates, peripapillary hemorrhages and bilateral presentation, which may occur either simultaneously or sequentially. Atypical ON requires careful consideration and differentiation from demyelinating ON and ischemic optic neuropathy since the treatment is different. We describe a patient who presented with recurrent, sequential episode of visual loss over 2 years, without evidence of any additional neurological deficits, sarcoidosis, or systemic autoimmune disease. A diagnosis of chronic relapsing inflammatory optic neuropathy was made, and she was given intravenous steroids for 3 days, which improved the vision from hand movement close to face to 5/60 in the right eye. She was given immunosuppressants for 3 months, and oral steroids were gradually tapered. Her present vision is the right eye is 6/6 with normal intraocular pressure and color vision.

Keywords: Bilateral, immunosuppressant, optic neuropathy, recurrent, steroids

How to cite this article:
Sharma V, Mahajan D, Sharma S, Chaudhary KP. Sequential bilateral retrobulbar optic neuritis in a young female. J Clin Ophthalmol Res 2017;5:100-4

How to cite this URL:
Sharma V, Mahajan D, Sharma S, Chaudhary KP. Sequential bilateral retrobulbar optic neuritis in a young female. J Clin Ophthalmol Res [serial online] 2017 [cited 2022 Aug 13];5:100-4. Available from: https://www.jcor.in/text.asp?2017/5/2/100/205182

Optic neuritis (ON) is defined as inflammation of the optic nerve. Typical ON presents with sudden onset loss of vision with dyschromatopsia and retro-orbital pain. It is usually associated with multiple sclerosis (MS), whereas atypical causes of ON differ in their clinical presentation, management, and prognosis. Clinical signs and symptoms that deviate from the typical presentation should prompt consideration of less common etiologies. Atypical features include lack of pain, simultaneous or near-simultaneous onset, lack of response or relapse on tapering from corticosteroids, optic nerve head or peripapillary hemorrhages.[1] Atypical causes of ON include neuromyelitis optica (NMO), autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy (CRION), idiopathic recurrent neuroretinitis and optic neuropathy associated with systemic diseases. The visual outcome in these cases is poor if untreated.[2]

  Case Report Top

A 32-year-old female from a rural background presented to the eye clinic on November 06, 2014, with the chief complaint of sudden onset painless decreased vision in the right eye for 1 week accompanied with a headache and difficulty in perceiving the color of objects. Visual acuity (VA) of the right eye and left eye was hand movement close to the face. Pupil of the right eye was sluggishly reacting, and the left eye pupil was mid–dilated and nonreacting. Fundus examination of the right eye revealed hyperemic disc with well-defined disc margins. Left eye fundus examination revealed a chalky white optic disc, with pale neuroretinal rim [Figure 1] and [Figure 2]. Fundus fluorescein angiography revealed a normal disc in the right eye and hypofluroscent disc in the left eye [Figure 3] and [Figure 4]. Visual fields revealed centocaecal scotomas in the right eye and total field defect in the left eye [Figure 5] and [Figure 6].
Figure 1: Fundus right eye

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Figure 2: Fundus left eye

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Figure 3: Fundus fluorescein angiography right eye

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Figure 4: Fundus fluorescein angiography left eye

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Figure 5: Visual field of the right eye

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Figure 6: Visual field of the left eye

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Magnetic resonance imaging (MRI) brain revealed atrophy of the left optic nerve and there was loss of differentiation between optic nerve and nerve sheath near the orbital apex on the right side. There was postcontrast enhancement seen in orbital apex region and intraorbital region, suggesting retrobulbar ON of the right eye. Brain parenchyma was normal [Figure 7]. MRI spine was normal. Visually evoked potential revealed prolonged p100 latency left > right eye.
Figure 7: Magnetic resonance imaging brain showing postcontrast enhancement seen on the right side in orbital apex region and intraorbital region

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High resolution computed tomogram chest revealed, minimal pleural thickening with calcified lymph nodes in the mediastinum and calcific parenchymal nodules, suggestive of old healed tuberculosis (TB). Mantoux test was positive, so a TB polymerase chain reaction was ordered, which was negative. The cerebrospinal fluid (CSF) analysis and blood investigations are described in [Table 1] and [Table 2].
Table 1: C.S.F analysis

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Table 2: Blood investigations

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There was a history of similar complaint in the left eye 2 years back in 2012 when she was diagnosed with left eye ON and treated with oral steroids 90 mg/kg, which were tapered subsequently. Her vision was 6/6 at the time of discharge. After 3 months, she consulted a primary health-care center for gradually progressive diminution of vision in the left eye. VA in the left eye had deteriorated to hand movement closed to face. Fundus examination revealed a chalky white optic disc, with pale neuroretinal rim suggestive of primary optic atrophy. History also revealed that she had taken anti-tubercular treatment for 6 months for tubercular osteomyelitis in the left knee joint at 10 years of age and was fully cured after complete treatment.

Considering the history and investigations a diagnosis of chronic sequential retrobulbar ON was made and treatment was started. She was given intravenous (IV) methyl prednisolone 1 mg/kg of body weight in three doses for 3 days. After 3 days, her vision in the right eye improved to 5/60 and the vision in the left eye was Finger countling (FC) at 1/2 feet. The left eye revealed relative afferent pupillary defect. After liver function tests and a complete hemogram, systemic steroids (delsone) 60 mg once daily, pantoprazole (pantop) 40 mg once daily, azathioprine (azorean) 50 mg twice daily and tablet neurobion forte twice daily was started for 2 weeks. After 15 days her vision was 6/9 in the right eye and FC 1/2 m in the left eye. There was a gradual tapering of steroids and immunosuppressants over 3 months as advised by the neurologist. A close follow up every 15 days was advised. Her present vision was 6/6 in the right eye with a normal color vision.

  Discussion Top

ON is essentially a clinical diagnosis. A careful history and examination help to distinguish typical from atypical ON which has different causes and treatment plan. If a complete investigation profile rules out the causes of inflammation of the optic nerve head, CRION may be a possibility.

CRION is a recently described form of recurrent isolated subacute optic neuropathy, without evidence of any additional neurological deficits, sarcoidosis, or systemic autoimmune diseases, with normal brain and spinal MRI. CRION should be considered in the differential diagnosis of MS, however it is a diagnosis of exclusion.[3],[4] CRION was first described in 2003.[5] The disease is rare, with only 122 cases published from 2003 to 2013.[5] Petzold and Plant found that there is a female predominance (48%), as compared to the males (20%), and no gender designation for the rest of the reported cases (32%).[6] The age of the patient's ranges from 14 to 69 years of age and the mean age is 35.6.[6] In contrast with typical ON, this condition is steroid dependent and relapsing, with an interval between episodes of days to 14 years with the involvement of both optic nerves, usually sequentially. Unlike demyelinating ON, it requires treatment to preserve vision.[3]

There are no markers that are diagnostic of CRION five diagnostic criteria have been laid down:[6]

  1. History of ON with one relapse
  2. Objectively measured visual loss
  3. NMO-IgG seronegative
  4. Contrast enhancement on imaging of acutely inflamed optic nerves
  5. Response to immunosuppressive treatment and relapse on withdrawal or dose reduction.

Diagnosis requires exclusion of other neurological, ophthalmological, and systemic conditions. Any cause of optic neuropathy should be ruled out, including demyelinating (MS, and NMO) and systemic disease (diabetic, toxic, nutritional, and infectious causes).[6]

In the acute phase IV steroids (methylprednisolone 1 mg/kg) for 3–5 days or plasmapheresis are given to restore visual function. Oral steroids (prednisone 1 mg/kg) with gradual tapering are given to stabilize vision.[6] To avoid adverse effects of long-term steroids and to avoid relapse of disease steroid-sparing agents such as azathioprine, methotrexate, cyclophosphamide, mycophenolate, IV immunoglobulin, plasma exchange, cyclosporine, and infliximab may be used.[6]

VA is dramatically worse with CRION than other forms of ON. Treatment with corticosteroids induces prompt relief of pain and improved vision.[5] At times, patients obtain complete restoration of vision, although exact success rates are unknown.[5] Recurrence is essentially inevitable in patients without treatment and patients ultimately will require lifelong immunosuppression to prevent relapse.

A total of 3327 patients in the MS Clinic in London, Ontario, specifically searched for clinically isolated syndrome and ON cases. They found three cases compatible with the definition CRION one of which was a male and the other two females (31–46-year-old). They were followed for 12 months and up to 10 years. Oligoclonal bands in CSF were negative. Brain and spine MRI did not meet diagnostic criteria for MS and did not reveal any other pathology. They ruled out Leber's hereditary optic neuropathy, NMO, Vitamin B12 deficiency, sarcoidosis, rheumatological or connective tissue diseases, and MS.[4] Hence, the diagnosis of CRION was made in these cases and further management was done for CRION in all these cases, which successfully restored the vision in the patients.

Kidd et al., in their report of 15 patients, described a syndrome presentation of subacute optic neuropathy, prominent pain, prompt response to systemic corticosteroids, and relapse on steroid withdrawal. In certain cases, immunosuppressants such as azathioprine were required in addition to prolonged corticosteroids. In instances where azathioprine was not tolerated, methotrexate was used.[5] The features that differentiated this group from the classical demyelinating ON included persistence of pain after onset of visual loss, the higher degree of visual loss, and frequent relapses. None of these patients had oligoclonal bands in the CSF, and MRI brain was normal. Out of thirty optic nerves, 19 imaged in these 15 patients showed abnormalities on MRI, including thickening, high signal intensity or enhancement.[5]

Till date, there are no systematic studies evaluating the duration and intensity of immunosuppression required in patients with CRION. The data are purely observational, based on personal experience. The experience in our patient indicates that long-term and low-dose corticosteroids and immunosuppresants are an effective and safe option. Currently, there is no population-based data on the natural history; prognosis and treatment plan for CRION.[4]

Myers et al. have described corticosteroid dependent ON, not associated with demyelinating disease.[7] In their report of 48 patients, 32 had evidence of systemic lupus erythematosus SLE, sarcoidosis, or other systemic autoimmune disease. Seventy-nine percent patients benefitted from systemic immunosuppression.

This patient had a similar attack 2 years back, which initially responded to steroids, but as the steroids were not continued for a longer period, relapse occurred leading to optic atrophy of that eye and sequential ON developed in the other eye. This patient might have had subclinical attacks that lead to optic atrophy in the left eye. The fact that she did not receive IV steroids and the oral steroids too were tapered over a short period of time with no follow could not save the left eye. Although no specific laboratory or clinical criteria have been described for the diagnosis of CRION, clinical indicators, including a higher degree of visual loss, persistence of pain after visual loss is established, and relapse on steroid withdrawal is helpful. A follow-up of the patient with the correct treatment is needed, as the condition is highly responsive to steroids and immunosuppressants and with the correct diagnosis and treatment the right eye could be saved.

  Conclusion Top

CRION should be considered in the differential diagnosis atypical ON after exclusion of demyelinating diseases, granulomatous, infectious, inflammatory, toxic, nutritional, and infiltrative causes of ON.[5] A correct and early diagnosis is necessary to ensure optimal further investigations and treatment. The possibility of CRION should be considered only in patients with recurrent and isolated ON. The symptoms and signs respond well to corticosteroid treatment. The importance of identifying these patients has therapeutic implication as CRION is highly responsive to steroids and may mandate long-term steroid use to maintain remission. Long-term immunosuppression is often necessary to preserve vision. In this patient, we could save the right eye of the patient with the correct diagnosis, treatment, and a close follow-up.


All the contributors would like to thank the entire Ophthalmology Department which worked as a team in making the diagnosis and assisting the various procedures done for the patient.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Gaier ED, Boudreault K, Rizzo JF 3rd, Falardeau J, Cestari DM. Atypical optic neuritis. Curr Neurol Neurosci Rep 2015;15:76.  Back to cited text no. 1
Malik A, Ahmed M, Golnik K. Treatment options for atypical optic neuritis. Indian J Ophthalmol 2014;62:982-4.  Back to cited text no. 2
[PUBMED]  [Full text]  
Saini M, Khurana D. Chronic relapsing inflammatory optic neuropathy. Ann Indian Acad Neurol 2010;13:61-3.  Back to cited text no. 3
[PUBMED]  [Full text]  
Guglielmo D, Kremenchutzky M. Chronic relapsing inflammatory optic neuropathy: A differential diagnosis from multiple sclerosis. Nerology;2012;78:2151.  Back to cited text no. 4
Kidd D, Burton B, Plant GT, Graham EM. Chronic relapsing inflammatory optic neuropathy (CRION). Brain 2003;126(Pt 2):276-84.  Back to cited text no. 5
Petzold A, Plant GT. Chronic relapsing inflammatory optic neuropathy: A systematic review of 122 cases reported. J Neurol 2014;261:17-26.  Back to cited text no. 6
Myers TD, Smith JR, Wertheim MS, Egan RA, Shutts WT, Rosenbaum JT. Use of corticosteroid sparing systemic immunosuppressives for the treatment of corticosteroid dependent optic neuritis not associated with demyelinating diseases. Br J Ophthalmol 2004;88:673–80.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]

  [Table 1], [Table 2]


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