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Year : 2021  |  Volume : 9  |  Issue : 1  |  Page : 45

Chronic relapsing inflammatory optic neuropathy: A rare entity

Department of Neurology, Dr. RML Hospital, Delhi, India

Date of Submission18-Jul-2020
Date of Decision25-Aug-2020
Date of Acceptance08-Sep-2020
Date of Web Publication10-Apr-2021

Correspondence Address:
Abhishek Juneja
A-15, Old Quarters, Ramesh Nagar, New Delhi - 110 015
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcor.jcor_106_20

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How to cite this article:
Juneja A, Anand KS. Chronic relapsing inflammatory optic neuropathy: A rare entity. J Clin Ophthalmol Res 2021;9:45

How to cite this URL:
Juneja A, Anand KS. Chronic relapsing inflammatory optic neuropathy: A rare entity. J Clin Ophthalmol Res [serial online] 2021 [cited 2022 Jul 1];9:45. Available from: https://www.jcor.in/text.asp?2021/9/1/45/313468


We report the case of 28-year-old female with right-eye vision loss with painful eye movements for 4 days without a preceding history of fever, headache, double vision, seizures, or head trauma. The patient had three such similar attacks of subacute progressive painful vision loss over 1–2 weeks involving either eye in the last 4 years, each attack lasting 4–6 months, with near-complete recovery following the administration of corticosteroids. There was no history of diabetes, hypertension, thyroid illness, or substance abuse in the past. On examination, the vitals were normal, right eye relative afferent pupillary defect was present, and bilateral fundi were normal on ophthalmoscopy. Visual acuity was 6/6 in the left eye by Snellen chart, while only hand movements could be perceived in the right eye with reduced color vision as tested by Ishihara plates. Formal visual field mapping was normal in the left eye with right-eye complete visual field loss. Rest of the neurological examination was unremarkable, without any sensorimotor, cerebellar, or autonomic dysfunction. Routine blood investigations including blood counts; hepatic, renal, thyroid, and glycemic profile; and serum Vitamin B12 levels were within normal limits. Human immunodeficiency virus test by enzyme-linked immunosorbent assay, antinuclear antibody, and anti-neutrophil cytoplasmic antibody by indirect immunofluorescence assay were negative. Serum angiotensin-converting enzyme and Venereal Disease Research Laboratory tests were also negative. Chest radiograph, bilateral temporal artery color duplex ultrasound, and magnetic resonance imaging (MRI) of the brain and spinal cord were unremarkable. Cerebrospinal fluid examination was normal in cell count; glucose and protein levels; oligoclonal bands; aquaporin-4 immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein antibodies were also negative. Pattern reversal-visual-evoked potential suggested prolonged P-100 latency in the right eye (138.6 ± 4.6 ms; N – 98.4 ± 4.7 ms), while normal in the left eye (102.2 ms). The patient was treated with intravenous (IV) methylprednisolone 1 g/day for 5 days followed by tapering doses of oral prednisolone for 4 weeks. The patient regained complete vision over the next 4 months.

Optic neuritis (ON) is characterized by subacute progressive loss of visual acuity and color vision with painful extraocular movements, responsive to corticosteroids. Recurrent attacks may occur in 3%–5% of patients with ON, affecting one or both eyes without evidence of central nervous system demyelination or other identifiable cause.[1] This condition is known as chronic relapsing inflammatory optic neuropathy (CRION). The diagnostic criteria proposed for CRION include at least two attacks of ON with objective evidence of vision loss, seronegative for neuromyelitis optica (NMO) IgG antibody, MRI contrast enhancement of optic nerves, and response to immunosuppressive therapy with features of relapse on drug withdrawal or dose reduction.[2] Treatment mainly relies on corticosteroids and other steroid-sparing immunosuppressive agents including IV immunoglobulin.[3] We ruled out other common causes of ON including multiple sclerosis, NMO, sarcoidosis, and Vitamin B12 deficiency by clinical evaluation and relevant investigations. We suggest that CRION should be kept as a differential diagnosis in recurrent episodes of ON to prevent permanent blindness.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Pirko I, Blauwet LA, Lesnick TG, Weinshenker BG. The natural history of recurrent optic neuritis. Arch Neurol 2004;61:1401-5.  Back to cited text no. 1
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. 2
Stiebel-Kalish H, Hammel N, van Everdingen J, Huna-Baron R, Lee AG. Intravenous immunoglobulin in recurrent-relapsing inflammatory optic neuropathy. Can J Ophthalmol 2010;45:71-5.  Back to cited text no. 3


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