Medicine:Chronic relapsing inflammatory optic neuropathy

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Chronic relapsing inflammatory optic neuropathy[1]
Other namesChronic relapsing inflammatory optic neuritis
SpecialtyOphthalmology, Neurology, Neuro-ophthalmology
Diagnostic methodConsensus Diagnostic Criteria[2]
Differential diagnosisOptic neuritis subgroups[2]
TreatmentCorticosteroids[2]

Chronic relapsing inflammatory optic neuropathy (CRION) is a form of recurrent optic neuritis that is steroid responsive and dependent.[1] Patients typically present with pain associated with visual loss.[1] CRION is a clinical diagnosis of exclusion, and other demyelinating, autoimmune, and systemic causes should be ruled out.[3] An accurate antibody test which became available commercially in 2017 has allowed most patients previously diagnosed with CRION to be re-identified as having MOG antibody disease,[4] which is not a diagnosis of exclusion. Early recognition is crucial given risks for severe visual loss and because it is treatable with immunosuppressive treatment such as steroids[3] or B-cell depleting therapy.[4] Relapse that occurs after reducing or stopping steroids is a characteristic feature.[3]

Signs and symptoms

Pain, visual loss, relapse, and steroid response are typical of CRION.[1][3] Ocular pain is typical, although there are some cases with no reported pain.[3] Bilateral severe visual loss (simultaneous or sequential) usually occurs, but there are reports of unilateral visual loss.[3] Patients can have an associated relative afferent pupillary defect.[5] CRION is associated with at least one relapse, and up to 18 relapses have been reported in an individual.[6] Intervals between episodes can range from days to over a decade.[1] Symptoms will improve with corticosteroids, and recurrence characteristically occurs after reducing or stopping steroids.[3]

Pathogenesis

In 2013, the etiology was unknown.[1] Given that CRION is responsive to immunosuppressive treatment, it was presumed to be immune-mediated,[3] but this was uncertain as at the time there were no known associated autoimmune antibodies.[3][7]

In 2015, some research pointed to CRION belonging to the MOG antibody-associated encephalomyelitis spectrum.[8]

As of 2019, the correlation between CRION and MOG antibody-associated encephalomyelitis is so high that now CRION is considered the most common phenotype related to myelin oligodendrocyte glycoprotein antibodies (MOG-IgG).[9]

As of 2021, some reports point out a second kind of CRION due to anti-phospholipid antibodies.[10]

Diagnosis

In 2018, of 12 patients in a study who fulfilled the then-current diagnostic criteria for CRION, eleven (92%) were positive for MOG-IgG, and the last patient was borderline.[4] Diagnosis requires exclusion of other neurological, ophthalmological, and systemic conditions.[3] Any cause of optic neuropathy should be ruled out, including demyelinating (MOG antibody disease, multiple sclerosis, and neuromyelitis optica) and systemic disease (diabetic, toxic, nutritional, and infectious causes).[3] Corticosteroid responsive optic neuritis not associated with demyelinating disease should also be ruled out, including sarcoidosis, systemic lupus erythematosus, or other systemic autoimmune disease.[11] Hereditary causes such as Leber's hereditary optic neuropathy are also part of the differential diagnosis.[12]

In 2014, there were no diagnostic biomarkers or imaging features typical of CRION.[3] Antinuclear antibodies (ANA), B12, folate, thyroid function tests, anti-aquaporin-4 antibodies (NMO-IgG), and glial fibrillary acidic protein (GFAP) can facilitate ruling out of other diseases.[3] Most patients are seronegative for NMO-IgG and GFAP, biomarkers for neuromyelitis optica.[3] ANA, indicative of autoimmune optic neuropathy, is also generally negative.[3] CSF can also be evaluated for oligoclonal bands typical of multiple sclerosis, which will not be present in CRION.[1] A chest X-ray or CT scan should be ordered if granulomatous optic neuropathy caused by sarcoidosis is suspected.[3]

Magnetic resonance imaging can capture optic nerve inflammation, but this finding is not present in all patients,[1][3][13] Diffusion tensor imaging has been shown to detect widespread white matter abnormalities in CRION patients with normal MRI findings.[14]

Five diagnostic criteria had been proposed in 2014:[3]

  • History of optic neuritis with one relapse
  • Objectively measured visual loss
  • NMO-IgG seronegative
  • Contrast enhancement on imaging of acutely inflamed optic nerves
  • Response to immunosuppressive treatment and relapse on withdrawal or dose reduction.

CRION has been included as a subtype in a 2022 international consensus classification of optic neuritis.[2]

Treatment

Treatment consists of three phases of immunotherapy:

  • 1. Acute phase: IV steroids (methylprednisolone 1 mg/kg) for 3–5 days or plasmapheresis are given to restore visual function.[3]
  • 2. Intermediate phase: Oral steroids (typically prednisone 1 mg/kg) with taper are given to stabilize vision.[3]
  • 3. Long-term phase: To avoid adverse effects of long-term steroids and to avoid relapse of disease, physicians can transition to a steroid-sparing agent. B-cell depleting therapy,[4] azathioprine, methotrexate, cyclophosphamide, mycophenolate, IVIG, plasma exchange, cyclosporine, and infliximab have been used.[3]

Visual acuity is dramatically worse with CRION than other forms of optic neuritis.[3] Treatment with corticosteroids induces prompt relief of pain and improved vision.[1] At times, patients obtain complete restoration of vision, although exact success rates are unknown.[1]

Prognosis

Recurrence is essentially inevitable in patients without treatment, and patients ultimately will require lifelong immunosuppression to prevent relapse.[3][15]

Epidemiology

CRION was first described in 2003.[1] The disease is rare, with only 122 cases published from 2003 to 2013.[3] There is female predominance with 59 females (48%), 25 males (20%), and no gender designation for the rest of the 122 reported cases (32%).[3] Age ranges from 14 to 69 years of age, and the mean age is 35.6.[3] The disease is noted to occur worldwide and across many ethnicities, with reported cases in all continents except Africa and Australia.[3]

See also

References

  1. ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 "Chronic relapsing inflammatory optic neuropathy (CRION)". Brain: A Journal of Neurology 126 (Pt 2): 276–84. February 2003. doi:10.1093/brain/awg045. PMID 12538397. 
  2. ↑ 2.0 2.1 2.2 2.3 "Diagnosis and Classification of Optic Neuritis". The Lancet Neurology 21 (12): 1120–1134. 2022. doi:10.1016/S1474-4422(22)00200-9. PMID 36179757. https://discovery.ucl.ac.uk/id/eprint/10156457/. 
  3. ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 3.16 3.17 3.18 3.19 3.20 3.21 3.22 3.23 3.24 3.25 3.26 "Chronic relapsing inflammatory optic neuropathy: a systematic review of 122 cases reported". Journal of Neurology 261 (1): 17–26. January 2014. doi:10.1007/s00415-013-6957-4. PMID 23700317. https://discovery.ucl.ac.uk/id/eprint/10153214/. 
  4. ↑ 4.0 4.1 4.2 4.3 "Chronic relapsing inflammatory optic neuropathy (CRION): a manifestation of myelin oligodendrocyte glycoprotein antibodies". Journal of Neuroinflammation 15 (1): 302. October 2018. doi:10.1186/s12974-018-1335-x. PMID 30382857. 
  5. ↑ "51-year-old female with steroid-responsive optic neuropathy: a new case of chronic relapsing inflammatory optic neuropathy (CRION)". Journal of Neurology 255 (9): 1419–20. September 2008. doi:10.1007/s00415-008-0919-2. PMID 18575925. 
  6. ↑ "Chronic relapsing inflammatory optic neuropathy". Annals of Indian Academy of Neurology 13 (1): 61–3. January 2010. doi:10.4103/0972-2327.61280. PMID 20436750. 
  7. ↑ "Diagnosis and classification of autoimmune optic neuropathy". Autoimmunity Reviews 13 (4–5): 539–45. 2014. doi:10.1016/j.autrev.2014.01.009. PMID 24424177. 
  8. ↑ "Anti-MOG antibodies are frequently associated with steroid-sensitive recurrent optic neuritis". Neurology: Neuroimmunology & Neuroinflammation 2 (4): e131. August 2015. doi:10.1212/NXI.0000000000000131. PMID 26185777. 
  9. ↑ "Brain Atrophy in Relapsing Optic Neuritis Is Associated With Crion Phenotype". Frontiers in Neurology 10: 1157. 2019. doi:10.3389/fneur.2019.01157. PMID 31736862. 
  10. ↑ "Chronic relapsing inflammatory optic neuropathy in a patient with triple antiphospholipid antibody positivity". Neurological Sciences 42 (8): 3439–3443. August 2021. doi:10.1007/s10072-021-05263-6. PMID 33880676. 
  11. ↑ "Use of corticosteroid sparing systemic immunosuppression for treatment of corticosteroid dependent optic neuritis not associated with demyelinating disease". The British Journal of Ophthalmology 88 (5): 673–80. May 2004. doi:10.1136/bjo.2003.028472. PMID 15090422. 
  12. ↑ Lee MD, Song BJ, Odel JG, Sadun AA. Bilateral vision loss responsive to corticosteroids. Survey of Ophthalmology. Nov-Dec 2013;58(6):634-639
  13. ↑ "MRI findings in chronic relapsing inflammatory optic neuropathy". BMJ Case Reports 2013: bcr2012008100. February 2013. doi:10.1136/bcr-2012-008100. PMID 23417378. 
  14. ↑ "White matter involvement beyond the optic nerves in CRION as assessed by diffusion tensor imaging". The International Journal of Neuroscience 125 (1): 10–7. January 2015. doi:10.3109/00207454.2014.896912. PMID 24588222. 
  15. ↑ "Treatment options for atypical optic neuritis". Indian Journal of Ophthalmology 62 (10): 982–4. October 2014. doi:10.4103/0301-4738.145986. PMID 25449930. 

External links

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