Chronic relapsing inflammatory optic neuropathy

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Chronic relapsing inflammatory optic neuropathy [1]
Other namesChronic relapsing inflammatory optic neuritis
Specialty Ophthalmology, Neurology, Neuro-ophthalmology
Diagnostic method Consensus Diagnostic Criteria [2]
Differential diagnosis Optic 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]

Contents

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]

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:

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

Related Research Articles

<span class="mw-page-title-main">Acute disseminated encephalomyelitis</span> Autoimmune disease

Acute disseminated encephalomyelitis (ADEM), or acute demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the brain and spinal cord to become inflamed, ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which, as a result, destroys the white matter. The cause is often a trigger such as from viral infection or vaccinations.

<span class="mw-page-title-main">Optic neuritis</span> Inflammation of the optic nerve

Optic neuritis describes any condition that causes inflammation of the optic nerve; it may be associated with demyelinating diseases, or infectious or inflammatory processes.

<span class="mw-page-title-main">Demyelinating disease</span> Any neurological disease in which the myelin sheath of neurons is damaged

A demyelinating disease refers to any disease affecting the nervous system where the myelin sheath surrounding neurons is damaged. This damage disrupts the transmission of signals through the affected nerves, resulting in a decrease in their conduction ability. Consequently, this reduction in conduction can lead to deficiencies in sensation, movement, cognition, or other functions depending on the nerves affected.

<span class="mw-page-title-main">Polyneuropathy</span> Any disease affecting peripheral nerves on both sides of the body

Polyneuropathy is damage or disease affecting peripheral nerves in roughly the same areas on both sides of the body, featuring weakness, numbness, and burning pain. It usually begins in the hands and feet and may progress to the arms and legs and sometimes to other parts of the body where it may affect the autonomic nervous system. It may be acute or chronic. A number of different disorders may cause polyneuropathy, including diabetes and some types of Guillain–Barré syndrome.

Neuromyelitis optica spectrum disorders (NMOSD) are a spectrum of autoimmune diseases characterized by acute inflammation of the optic nerve and the spinal cord (myelitis). Episodes of ON and myelitis can be simultaneous or successive. A relapsing disease course is common, especially in untreated patients.

<span class="mw-page-title-main">Relapsing polychondritis</span> Medical condition

Relapsing polychondritis is a systemic disease characterized by repeated episodes of inflammation and in some cases deterioration of cartilage. The disease can be life-threatening if the respiratory tract, heart valves, or blood vessels are affected. The exact mechanism is poorly understood.

<span class="mw-page-title-main">Myelin oligodendrocyte glycoprotein</span>

Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein believed to be important in the myelination of nerves in the central nervous system (CNS). In humans this protein is encoded by the MOG gene. It is speculated to serve as a necessary "adhesion molecule" to provide structural integrity to the myelin sheath and is known to develop late on the oligodendrocyte.

<span class="mw-page-title-main">Neuritis</span> Inflammation of a nerve or generally any part of the nervous system

Neuritis, from the Greek νεῦρον), is inflammation of a nerve or the general inflammation of the peripheral nervous system. Inflammation, and frequently concomitant demyelination, cause impaired transmission of neural signals and leads to aberrant nerve function. Neuritis is often conflated with neuropathy, a broad term describing any disease process which affects the peripheral nervous system. However, neuropathies may be due to either inflammatory or non-inflammatory causes, and the term encompasses any form of damage, degeneration, or dysfunction, while neuritis refers specifically to the inflammatory process.

<span class="mw-page-title-main">Chronic inflammatory demyelinating polyneuropathy</span> Medical condition

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disease of the peripheral nervous system characterized by progressive weakness and impaired sensory function in the legs and arms. The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy. CIDP is closely related to Guillain–Barré syndrome and it is considered the chronic counterpart of that acute disease. Its symptoms are also similar to progressive inflammatory neuropathy. It is one of several types of neuropathy.

<span class="mw-page-title-main">Pemphigoid</span> Autoimmune blistering diseases

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Optic neuropathy is damage to the optic nerve from any cause. The optic nerve is a bundle of millions of fibers in the retina that sends visual signals to the brain.

Inflammatory demyelinating diseases (IDDs), sometimes called Idiopathic (IIDDs) due to the unknown etiology of some of them, are a heterogenous group of demyelinating diseases - conditions that cause damage to myelin, the protective sheath of nerve fibers - that occur against the background of an acute or chronic inflammatory process. IDDs share characteristics with and are often grouped together under Multiple Sclerosis. They are sometimes considered different diseases from Multiple Sclerosis, but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course.

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<span class="mw-page-title-main">Tumefactive multiple sclerosis</span> Medical condition

Tumefactive multiple sclerosis is a condition in which the central nervous system of a person has multiple demyelinating lesions with atypical characteristics for those of standard multiple sclerosis (MS). It is called tumefactive as the lesions are "tumor-like" and they mimic tumors clinically, radiologically and sometimes pathologically.

<span class="mw-page-title-main">Behçet's disease</span> Inflammatory disorder

Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the eye, and arthritis. The sores can last from a few days, up to a week or more. Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness. Often, the symptoms come and go.

Autoimmune optic neuropathy (AON), sometimes called autoimmune optic neuritis, may be a forme fruste of systemic lupus erythematosus (SLE) associated optic neuropathy. AON is more than the presence of any optic neuritis in a patient with an autoimmune process, as it describes a relatively specific clinical syndrome. AON is characterized by chronically progressive or recurrent vision loss associated with serological evidence of autoimmunity. Specifically, this term has been suggested for cases of optic neuritis with serological evidence of vasculitis by positive ANA, despite the lack of meeting criteria for SLE. The clinical manifestations include progressive vision loss that tends to be steroid-responsive and steroid dependent.

MOG antibody disease (MOGAD) or MOG antibody-associated encephalomyelitis (MOG-EM) is an inflammatory demyelinating disease of the central nervous system. Serum anti-myelin oligodendrocyte glycoprotein antibodies are present in up to half of patients with an acquired demyelinating syndrome and have been described in association with a range of phenotypic presentations, including acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, and neuromyelitis optica.

Anti-AQP4 diseases, are a group of diseases characterized by auto-antibodies against aquaporin 4.

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References

  1. 1 2 3 4 5 6 7 8 9 10 11 Kidd D, Burton B, Plant GT, Graham EM (February 2003). "Chronic relapsing inflammatory optic neuropathy (CRION)". Brain: A Journal of Neurology. 126 (Pt 2): 276–84. doi: 10.1093/brain/awg045 . PMID   12538397.
  2. 1 2 3 4 Petzold A, Fraser C, Abegg M, Alroughani R (2022). "Diagnosis and Classification of Optic Neuritis". The Lancet Neurology. 21 (12): 1120–1134. doi:10.1016/S1474-4422(22)00200-9. PMID   36179757. S2CID   252564095.
  3. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 Petzold A, Plant GT (January 2014). "Chronic relapsing inflammatory optic neuropathy: a systematic review of 122 cases reported". Journal of Neurology. 261 (1): 17–26. doi:10.1007/s00415-013-6957-4. PMID   23700317. S2CID   31281504.
  4. 1 2 3 4 Lee HJ, Kim B, Waters P, Woodhall M, Irani S, Ahn S, et al. (October 2018). "Chronic relapsing inflammatory optic neuropathy (CRION): a manifestation of myelin oligodendrocyte glycoprotein antibodies". Journal of Neuroinflammation. 15 (1): 302. doi: 10.1186/s12974-018-1335-x . PMC   6208174 . PMID   30382857.
  5. ↑ Kaut O, Klockgether T (September 2008). "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. doi:10.1007/s00415-008-0919-2. PMID   18575925. S2CID   31618770.
  6. ↑ Saini M, Khurana D (January 2010). "Chronic relapsing inflammatory optic neuropathy". Annals of Indian Academy of Neurology. 13 (1): 61–3. doi: 10.4103/0972-2327.61280 . PMC   2859591 . PMID   20436750.
  7. ↑ Petzold A, Plant GT (2014). "Diagnosis and classification of autoimmune optic neuropathy". Autoimmunity Reviews. 13 (4–5): 539–45. doi:10.1016/j.autrev.2014.01.009. PMID   24424177.
  8. ↑ Chalmoukou K, Alexopoulos H, Akrivou S, Stathopoulos P, Reindl M, Dalakas MC (August 2015). "Anti-MOG antibodies are frequently associated with steroid-sensitive recurrent optic neuritis". Neurology: Neuroimmunology & Neuroinflammation. 2 (4): e131. doi:10.1212/NXI.0000000000000131. PMC   4496630 . PMID   26185777.
  9. ↑ Cantó LN, Boscá SC, Vicente CA, Gil-Perontín S, Pérez-Miralles F, Villalba JC, et al. (2019). "Brain Atrophy in Relapsing Optic Neuritis Is Associated With Crion Phenotype". Frontiers in Neurology. 10: 1157. doi: 10.3389/fneur.2019.01157 . PMC   6838209 . PMID   31736862.
  10. ↑ Navarro CE, Arango GJ, Cubides MF (August 2021). "Chronic relapsing inflammatory optic neuropathy in a patient with triple antiphospholipid antibody positivity". Neurological Sciences. 42 (8): 3439–3443. doi:10.1007/s10072-021-05263-6. PMID   33880676. S2CID   233314170.
  11. ↑ Myers TD, Smith JR, Wertheim MS, Egan RA, Shults WT, Rosenbaum JT (May 2004). "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. doi:10.1136/bjo.2003.028472. PMC   1772147 . 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. ↑ Sharma A, Khurana D, Kesav P (February 2013). "MRI findings in chronic relapsing inflammatory optic neuropathy". BMJ Case Reports. 2013: bcr2012008100. doi:10.1136/bcr-2012-008100. PMC   3604485 . PMID   23417378.
  14. ↑ Colpak AI, Kurne AT, Oguz KK, Has AC, Dolgun A, Kansu T (January 2015). "White matter involvement beyond the optic nerves in CRION as assessed by diffusion tensor imaging". The International Journal of Neuroscience. 125 (1): 10–7. doi:10.3109/00207454.2014.896912. PMID   24588222. S2CID   207419720.
  15. ↑ Malik A, Ahmed M, Golnik K (October 2014). "Treatment options for atypical optic neuritis". Indian Journal of Ophthalmology. 62 (10): 982–4. doi: 10.4103/0301-4738.145986 . PMC   4278124 . PMID   25449930.
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