A review of chronic relapsing inflammatory optic neuropathy

Pushpendra Nath Renjen; Dinesh Mohan Chaudhari; Kamal Ahmad; Shivangi Garg; Anjali Mishra

doi:10.4103/am.am_38_20

REVIEW ARTICLE

Year : 2020 | Volume : 17 | Issue : 4 | Page : 256-258

A review of chronic relapsing inflammatory optic neuropathy

Pushpendra Nath Renjen¹, Dinesh Mohan Chaudhari¹, Kamal Ahmad¹, Shivangi Garg¹, Anjali Mishra²
¹ Department of Neurology, Institute of Neurosciences, Indraprastha Apollo Hospitals, New Delhi, India
² Department of Critical Care, Max Super Speciality Hospital, New Delhi, India

Date of Submission	14-May-2020
Date of Acceptance	05-Oct-2020
Date of Web Publication	24-Nov-2020

Correspondence Address:
Pushpendra Nath Renjen
C-85, Anand Niketan, New Delhi - 110 021
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_38_20

Abstract

Chronic relapsing inflammatory optic neuropathy (CRION), initially described in 2003, is a form of recurrent optic neuritis (ON) that has relatively good response/dependency to steroid treatment. In demyelinating ON, patients present with periorbital ache and pain elicited by eye movement, which usually begins insidiously and worsens, in synchrony with or preceding a reduction in vision, which deteriorates over days. The etiology of CRION is unknown. However, the excellent response to corticosteroids and the need for immunosuppressive therapy to prevent relapse suggests an immunomediated origin.

Keywords: Chronic relapsing inflammatory optic neuropathy, myelin oligodendrocyte glycoprotein, neuromyelitis optica, neuropathy, optic

How to cite this article:
Renjen PN, Chaudhari DM, Ahmad K, Garg S, Mishra A. A review of chronic relapsing inflammatory optic neuropathy. Apollo Med 2020;17:256-8

How to cite this URL:
Renjen PN, Chaudhari DM, Ahmad K, Garg S, Mishra A. A review of chronic relapsing inflammatory optic neuropathy. Apollo Med [serial online] 2020 [cited 2021 May 14];17:256-8. Available from: https://www.apollomedicine.org/text.asp?2020/17/4/256/301472

Introduction

Subacute loss of vision accompanied by pain is most commonly due to some form of inflammatory optic neuropathy; the most common cause of which is demyelinating optic neuritis (ON). In demyelinating ON, patients present with periorbital ache and pain elicited by eye movement, which usually begins insidiously and worsens, in synchrony with or preceding a reduction in vision, which deteriorates over days. The pain tends to diminish as the visual symptoms supervene; the visual loss reaches a variable nadir at around 5 days, then begins to improve. Substantial recovery of vision occurs within 6 weeks and is followed by slower recovery over 12 ± 18 months.^[1] Demyelinating optic neuropathies also occur following viral infections, particularly in children, in whom the disorder is more commonly bilateral. Herpesviruses, hepatitis A, enteroviruses, and exanthemata have all been associated with this complication, as having bacterial infections involving such organisms as Streptococcus, Meningococcus, Brucella, Pertussis, Salmonella typhi, Treponema pallidum, Borrelia, and Mycobacterium tuberculosis. Postvaccinial ON also occurs. Systemic vasculitis may cause optic neuropathy. In the majority of cases, this is associated with an acute painless onset, resembling acute anterior ischemic optic neuropathy, but in other cases, there is subacute, progressive, painful optic neuropathy.^[2] Optic neuropathy is also known in sarcoidosis, in which condition granulomatous masses are often seen to involve the anterior visual pathways but in some cases imaging is normal. Compressive or infiltrative lesions, such as metastatic carcinoma or lymphoreticular disease, meningioma and glioma, and fungal diseases may all present in similar ways.^[3]

Chronic Relapsing Inflammatory Optic Neuropathy

Chronic relapsing inflammatory optic neuropathy (CRION), initially described in 2003 [3], is a form of recurrent ON that has relatively good response/dependency to steroid treatment. Petzold and Plant^[4] reviewed 122 case reports and proposed the diagnostic criteria of CRION by adding radiological and laboratory findings: enhancement of the optic nerve on magnetic resonance imaging (MRI) and the absence of aquaporin-4 antibodies (AQP4-Ab). The antibody to myelin oligodendrocyte glycoprotein (MOG-Ab) has been proposed as a new marker of inflammatory demyelinating diseases (IDDs) of the central nervous system (CNS).^[5],[6] Characteristics of MOG

-IgG-associated diseases include ON as a major symptom, good response to steroid, absence of serum AQP4-Ab, and steroid-dependent relapse or disease exacerbation.^[6],[7]

Etiology

The etiology of CRION is unknown. However, the excellent response to corticosteroids and the need for immunosuppressive therapy to prevent relapse suggests an immunomediated origin.^[8] In their first description, Kidd et al. observed that the clinical picture is similar to other inflammatory optic neuropathies, such as that caused by sarcoidosis. However, in their description, none of the 15 patients followed for an average of 8 years developed systemic symptoms. They hypothesized that CRION could be a localized form of granulomatous disease other than sarcoidosis.^[3]

Wingerchuk et al. described NO within neuromyelitis optica spectrum disorder (NMOSD).^[9] Following this description, a debate arose over whether the CRION was a different entity or a variant of NMOSD. Although controversy remains, what is clear is that the presence of antineuromyelitis optica (NMO) antibodies in patients with recurrent NO directed to a NMOSD; in addition, the CRION criteria require the absence of anti-NMO antibodies.^[4]

Diagnosis

In 2014, Petzold and Plant^[4] proposed the diagnostic criteria for CRION [Table 1]. These criteria are mandatory and are based on the presence of recurrent NO episodes to which are added serological findings and MRI of the brain.^[8]

Table 1: Diagnostic criteria of chronic recurrent inflammatory optic neuropathy

Click here to view

Although CRION should be considered in patients with recurrent NO with response to corticosteroids, this condition can be caused by many pathologies. Systemic diseases, such as sarcoidosis, systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, or Behçet's disease, should be excluded before definitive diagnosis.^[8]

Treatment

The main characteristic of patients with CRION is the rapid and excellent response to corticosteroid therapy, as well as corticodependence, with relapses after the withdrawal or decrease of corticosteroids.^[3] The recommended treatment is intravenous methylprednisolone 1 mg/kg/3–5 days or oral prednisone at a dose of 1 mg/kg with subsequent dose de-escalation.^[4] Abrupt withdrawal of treatment may lead to irreversible worsening of visual acuity.^[10] Because the long-term immunosuppression also produces adverse effects, some authors have suggested treatment with intravenous immunoglobulin to prevent corticodependence.^[11] In another study, treatment with monthly intravenous immunoglobulins allowed the withdrawal of all immunosuppressants in a patient with recurrent NO.^[12] Good results have also been reported with rituximab and natalizumab. However, as these are isolated cases, no conclusion can be drawn.^[10]

Conclusion

CRION is an inflammatory disease of the optic nerve. It is characterized by severe recurrent NOs with a good response to corticosteroids and relapses after its withdrawal
The main characteristic of patients with CRION is the rapid and excellent response to corticosteroid therapy, as well as corticodependence, with relapses after the withdrawal or decrease of corticosteroids
It is a rare entity, making the diagnosis a challenge for the neurologist and ophthalmologist. The main differential diagnoses are demyelinating and systemic diseases.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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