Optic Neuritis
Optic neuritis refers to the inflammation of the optic nerve (cranial nerve II), classically associated with multiple sclerosis.
This updated UKMLA guide to optic neuritis covers causes, risk factors, symptoms, diagnosis, and management.
Causes and Risk Factors
Optic neuritis is most commonly caused by inflammatory demyelination of the optic nerve
- Multiple sclerosis – classic and most common association
- Optic neuritis is often the first sign of multiple sclerosis (see the Multiple Sclerosis (MS) article for more information)
- ~50% of multiple sclerosis patients experience at least 1 episode of optic neuritis over a 15-year period
- Neuromyelitis optica spectrum disorder
- Systemic autoimmune diseases (e.g. sarcoidosis, SLE)
Other causes:
- Infection
- Viral: HSV, VZV, EBV, measles, CMV
- Bacterial: syphilis, Lyme disease, tuberculosis
- Medications
- Ethambutol
- Amiodarone
- Linezolid
- Vitamin B12 deficiency
Risk factors (broadly overlap with those for multiple sclerosis):
- Young adults 20-50 y/o
- Females
- Caucasians
- HLA-B27 and HLA DRB1
Clinical Features
Typically presents with an acute / subacute onset of unilateral symptoms.
CRAP is a useful mnemonic:
| Letter | Corresponding category | Description |
|---|---|---|
| C | Central scotoma |
|
| R | RAPD (Marcus Gunn pupil) | Elicited by the swinging torch test:
Note that RAPD is not diagnostic of one specific disease, it simply indicates reduced afferent input from one eye, usually due to retinal or optic nerve pathology |
| Red desaturation |
|
|
| A | Acuity (visual) |
|
| P | Pain |
|
Fundoscopic appearance depends on the type of optic neuritis:
- The optic disc appears normal in most cases (retrobulbar optic neuritis)
- 1/3 cases will exhibit optic disc swelling and hyperaemia (papillitis)
Disease course and progression:
- After the acute onset, optic neuritis typically stabilises in a subacute stage. Vision often recovers spontaneously within a few weeks (near 90% of patients achieve near-normal vision by 6 months, irrespective of treatment)
- Optic neuritis can recur, entering a chronic or recurrent stage – esp. when associated with MS or NMOSD
Investigation and Diagnosis
The diagnosis of optic neuritis itself is primarily a clinical diagnosis (see above)
If optic neuritis is suspected, further investigations are necessary to check for any underlying causes:
- Gold standard imaging: MRI brain and orbits with gadolinium contrast
- Used to assess the risk of diagnosing multiple sclerosis
- If the diagnosis of optic neuritis is clinically uncertain, enhancement of the optic nerve can confirm the diagnosis
- CSF analysis (via lumbar puncture) – presence of oligoclonal bands in the CSF, but not in the serum, is pathognomonic for MS
- Serology
- Antiaquaporin-4 IgG antibodies to exclude NMOSD
- Myelin oligodendrocyte glycoprotein IgG antibodies to exclude MOGAD
- Autoimmune panel including ANA, if SLE is suspected
- Infection work-up (if an infectious trigger is suspected – syphilis, Lyme disease, tuberculosis testing)
Management
If optic neuritis is suspected → refer to both ophthalmology and neurology.
Acute management of optic neuritis:
- 1st line: high-dose IV methylprednisolone, followed by an oral prednisolone taper
- Oral corticosteroids alone should be avoided, as this increases the risk of recurrent attacks
- If severe optic neuritis does NOT respond to IV steroids → plasma exchange
Technically, corticosteroids are mainly used for inflammatory / autoimmune optic neuritis, especially those associated with multiple sclerosis, NMOSD, and systemic inflammatory causes (e.g. SLE).
Infective causes must be excluded or treated appropriately, as giving steroids alone in infection may worsen the underlying infection.
Long-term management involves identifying and managing any underlying causes:
- Multiple sclerosis → disease-modifying therapies (see the Multiple Sclerosis (MS) article for more information)
- NMOSD and autoimmune causes → immunosuppressants and monoclonal antibodies
- SLE → see the Systemic Lupus Erythematosus (SLE) article
- Sarcoidosis → see the Sarcoidosis article
References
Related Articles
Systemic Lupus Erythematosus (SLE)