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Disease
- A typical optic neuritis is often the initial episode of a Multiple Sclerosis (MS) in previously healthy patients
- Incidence in Central Europe: 5 per 100,000/year
Typical Clinical Presentation
- Idiopathic or associated with Multiple Sclerosis
- Age 18 – 45 years, female > male (3:1)
- Unilateral mild to moderate visual impairment
- Deterioration over hours to days for 1-2 weeks, followed by improvement
- Visual acuity rarely <0.1
- Pain with eye movement
- 2/3 normal optic disc, 1/3 optic disc swelling
Atypical Clinical Presentation
- Bilateral involvement
- Severe visual impairment <0.1
- <18 or >45 years of age
- No pain with eye movement
- Optic disc with significant swelling, haemorrhages, hard exudates, cotton wool spots, or initial atrophy
- No improvement within four weeks
- Early recurrence in the same or partner eye
Other Symptome
- Reduced color and contrast vision
- Periorbital pain exacerbated by eye movement
- No diplopia or other neurological symptoms (except in known MS)
- Uthoff phenomenon (symptom exacerbation with heat/physical exertion) during the course
- Approximately 30% experience phosphenes (light flashes triggered by eye movements or sounds)
- Pulfrich phenomenon (pendulum movements appear elliptical)
Work-up
- Visual acuity
- Pupil examination (RAPD?)
- Contrast vision (Pelli-Robson), colour vision (red desaturation, Ishihara)
- Visual field (no specific defect, any defect possible)
- Motility (painful)
- Slit-lamp examination (normal)
- Fundus examination (pupil dilation not mandatory)
- 2/3 normal optic disc, 1/3 mild disc swelling
- Macula normal
- OCT Macula + Optic Disc (typically papillo-macular bundle affected, thinning of RNFL temporally in the course, decrease in ganglion cells)
- Pattern-VEP (optional)
Imaging
- MRI Orbit + Head + FLAIR sequences within 2-3 days
- Demyelinating lesions?
- Active lesions visible in T1 with contrast, older lesions in FLAIR
- Optic nerve lesions with fat suppression
- Findings in typical optic neuritis
- Periventricular hyperintensities in the white matter
- Short lesions of the optic nerve
- Findings in atypical optic neuritis
- Longitudinal enhancement of the optic nerve (> 50% of the length between disc and chiasm)
- Chiasma involvement or posterior part of the optic nerve
- Longitudinally extensive spinal cord lesions ≥3 vertebral segments (LETM)
Treatment
- Steroids (usually only after imaging):
- 1g/d SoluMedrol i.v. for 3 days if visual acuity drops < 0.5/0.6; if only 1 seeing eye, consider treating from 0.8
- Subsequently, consider Spiricort 1mg/kg body weight/d
Follow-up
- Follow-up after 2, 4 weeks, then individually
- No progressive visual deterioration expected after 2 weeks; visual improvement from about 4 weeks, possibly even faster recovery (depending on severity)
Approach to Atypical Optic Neuritis
- Clinically or radiologically atypical?
- Rule out NMOSD
- Laboratory
- Aquaporin-4 antibodies (AQP4-AB)
- Myelin-Oligodendrocyte-Glycoprotein antibodies (MOG-AB)
- Spinal MRI and Head MRI (if not yet performed)
- Laboratory
- Neurological consultation, consider lumbar puncture, initiation of immunotherapy
Differential Diagnoses
- Neuromyelitis-optica-Spectrum-disorders (NMOSD)
- Classification
- Aquaporin-4 antibodies (AQP4-AB) – seropositive (approx. 70%)
- AQP4-seronegative (approx. 30%)
- Myelin-Oligodendrocyte-Glycoprotein antibodies (MOG-AB) associated
- About 25% of AQP4-negative cases
- Often severe optic neuritis, which often leads to blindness without therapy
- Another autoimmune disease is often known
- Symptoms
- Acute or subacute severe visual impairment (<0.1)
- Unilateral, bilateral simultaneously, or sequentially
- Transverse Myelitis
- Work-up: AQP4-AB, MOG-AB, MRI Skull and Spine
- Treatment: Steroids i.v. in the acute stage; as prophylaxis Rituximab/Azathioprine/Mycophenolate mofetil or others
- Classification
- others
- AION, NAION
- Tumors
- LHON
- Toxic Optic Neuropathy
- Neuroretinitis
- other rare causes, e.g., Sarcoidosis, Lupus erythematosus, Syphilis, Borreliosis
Sources
- EyeWiki Demyelinating Optic Neuritis
- EyeWiki Multiple Sclerosis
- EyeWiki Neuromyelitis optica
- AWMF Leitlinie Optikusneuritis
- The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
- Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)
- Wilhelm H, Schabet M: The diagnosis and treatment of optic neuritis. Dtsch Arztebl Int 2015; 112: 616–26. DOI: 10.3238/arztebl.2015.0616