Optic Neuritis

Aka: Optic Neuritis, Retrobulbar Optic Neuropathy, Papillitis, Neuromyelitis Optica Spectrum Disorder, NMOSD, Devic Disease, Myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis, MOG-IgG Optic Neuritis

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II. Definitions

  1. Optic Neuritis
    1. Inflammation of the Optic Nerve

III. Epidemiology

  1. Incidence: 1 to 5 per 100,000 in U.S. per year
  2. Age in typical Optic Neuritis (demyelinating)
    1. Young patients predominate (esp. age 20 to 45 years old)
    2. Most common cause of unilateral Vision Loss in young adults

IV. Types: General

  1. Typical Optic Neuritis (Demyelinating, most common)
    1. Classic triad: Unilateral Vision Loss, painful Extraocular Movement, impaired color Vision
    2. Relative Afferent Pupillary Defect, and typically normal fundoscopic exam (or mild optic disc swelling)
    3. Progresses over days to weeks, and then, regardless of treatment, spontaneously resolves in 90% of cases
    4. Associated with Multiple Sclerosis (or idiopathic)
      1. Immune-mediated inflammatory demyelination of the Optic Nerve
      2. Demyelination results in Retinal Ganglion cell axon degeneration
  2. Atypical Optic Neuritis (not MS associated)
    1. More common in males, age <18 years or over 50 years old
    2. Broad list of potential causes (see below) unrelated to Multiple Sclerosis
    3. Presents with bilateral, acute, painless Vision Loss that progresses over 2 weeks and persists
    4. Severe Vision Loss (20/200 or worse)
    5. Severe optic disc swelling

V. Types: Specific Atypical Optic Neuritis Demyelinating Syndromes (Uncommon)

  1. General
    1. Rare CNS inflammatory disorders distinct from Multiple Sclerosis (MS)
    2. Among the atypical Optic Neuritis causes
  2. Neuromyelitis Optica Spectrum Disorder (NMOSD)
    1. Presents with severe Vision Loss (20/400 or worse), often bilateral
    2. Residual deficits are worse with delay in Glucocorticoid treatment
    3. Aquaporin-4 (AQP4) serum Antibody positive AND at least 1 of the following clinical features
      1. Optic Neuritis (most common)
      2. Acute Myelitis
      3. Area Postrema Syndrome (unexplained Hiccups or Nausea, Vomiting)
  3. Myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis (MOG-IgG Optic Neuritis)
    1. Severe, Acute Vision Loss on presentation (often recurrent attacks)
    2. Optic disc edema is present in all patients
    3. Much better longterm prognosis than NMOSD
      1. MOG-IgG Optic Neuritis final Vision is typically 20/30

VI. Causes

  1. Demyelinating Conditions (see above)
    1. Multiple Sclerosis (typical Optic Neuritis)
      1. Optic Neuritis is the presenting finding in 15-20% of Multiple Sclerosis (overal seen in 50% of MS cases)
    2. Uncommon, atypical demyelinating causes
      1. Neuromyelitis Optica Spectrum Disorder (NMOSD)
      2. Myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis (MOG-IgG Optic Neuritis)
  2. Autoimmune Conditions
    1. Systemic Lupus Erythematosus
    2. Sarcoidosis
    3. Granulomatosis with Polyangiitis
    4. Sjogren Syndrome
    5. Antiphospholipid Antibody Syndrome
    6. Posterior Uveitis
  3. Infections (rare)
    1. Lyme Disease
    2. Syphilis
    3. HIV Infection
    4. Tuberculosis
    5. West Nile Virus
    6. Toxpolasmosis
    7. Fungal infections
    8. Encephalomyelitis
  4. Optic Nerve vascular lesions
    1. See Ischemic Optic Neuritis
    2. Central Retinal Artery Occlusion
    3. Central Retinal Vein Occlusion
    4. Anterior Ischemic Optic Neuropathy
  5. Tumor
    1. Optic Nerve glioma
    2. Neurofibromatosis
    3. Meningioma
  6. Medications
    1. Aminosalicylic Acid
    2. Chloramphenicol
    3. Ethambutol
    4. Isoniazid
    5. Penicillamine
    6. Phenothiazines
    7. Phenylbutazone
    8. Quinine
    9. Streptomycin

VII. Risk Factors: Typical Optic Neuritis

  1. Multiple Sclerosis
  2. White Race
  3. Female Gender
  4. Age 20 to 45 years old

VIII. Symptoms: Typical Optic Neuritis

  1. Pain behind affected eye is variably present
    1. Worse with eye movement
  2. Central Vision Loss
  3. Loss of color Vision
  4. Impaired Vision develops over hours to days
    1. Affects one or both eyes
    2. Rarely results in total blindness
    3. Acuity often worse than 20/100

IX. Signs: Typical Optic Neuritis

  1. Unilateral optic disc swelling and blurring of the disc margin
    1. Optic Nerve pallor, hyperemia or Papillitis present (Papilledema)
    2. Optic disc may appear normal in Retrobulbar Optic Neuropathy
  2. Pupil light reflex abnormal
    1. Afferent Pupillary Defect
  3. Provocative maneuvers
    1. Extraocular Movement painful
    2. Pressure on globe painful
  4. Decreased Visual Acuity amd Visual Field Deficits

X. Labs

  1. Atypical Optic Neuritis Demyelinating Conditions (Uncommon)
    1. Aquaporin-4 (AQP4) serum Antibody (NMOSD)
    2. Myelin Oligodendrocyte Glycoprotein IgG (MOG-IgG Optic Neuritis)
  2. Cerebrospinal Fluid
    1. Consider in findings suggestive of MS and typical Optic Neuritis
    2. Typical Optic Neuritis MS-related findings
      1. WBC: Normal or 10-20 WBC present
      2. Oligoclonal bands may be present

XI. Imaging

  1. Brain MRI and Orbit MRI with gadolinium contrast (and spine in NMOSD)
    1. Typical Optic Neuritis is a clinical diagnosis based on history and exam, and does not require MRI
      1. MRI may confirm acute demyelinating Optic Neuritis (Optic Nerve enhancement)
      2. MRI may show concurrent CNS findings of Multiple Sclerosis (e.g. periventricular white matter deficits)
    2. Neuromyelitis Optica Spectrum Disorder (NMOSD)
      1. Obtain MRI Brain and spinal cord with gadolinium contrast
      2. Optic Nerve lesions (involving >50%), often bilateral, and may involve Optic Chiasm
      3. Spine may demonstrate extensive Transverse Myelitis (>3 levels) and central cord involvement
        1. Contrast with typical Optic Neuritis of MS, in which the peripheral spinal cord is involved
    3. Myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis (MOG-IgG Optic Neuritis)
      1. Extensive Optic Nerve enhancement on MRI Brain, with no other Brain Lesions (unlike MS)

XII. Differential Diagnosis

  1. See Acute Vision Loss

XIII. Management: Typical Optic Neuritis

  1. Hospital admission
  2. IV Glucocorticoids
    1. Methylprednisolone 250 mg IV every 6 hours (or 1 g daily) for 3 days
    2. May be followed by oral Corticosteroid course
    3. Efficacy
      1. May reduces chance of future Multiple Sclerosis
      2. Corticosteroids speed Vision recovery in typical Optic Neuritis
      3. However, Corticosteroids appear to offer no benefit at 6 months in typical Optic Neuritis
      4. Avoid low dose Corticosteroid (no benefit, and higher relapse risk)
      5. Gal (2015) Cochrane Database Syst Rev (8): CD001430 [PubMed]

XIV. Management: Atypical Optic Neuritis

  1. Indications
    1. Neuromyelitis Optica Spectrum Disorder (NMOSD)
    2. Myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis (MOG-IgG Optic Neuritis)
  2. Acute Optic Neuritis
    1. Methylprednisolone 1 g IV daily for 5 days, then oral steroid taper
      1. Best outcomes with early treatment (esp. for NMOSD)
    2. Plasma Exchange
      1. Indicated in refractory cases
  3. Chronic Immunosuppression to prevent relapse
    1. Azathioprine or Rituximab

XV. Course

  1. Isolated, typical Optic Neuritis
    1. Up to 30-50% develop Multiple Sclerosis within 15 years

XVI. References

  1. Yanoff (1999) Ophthalmology, Mosby, p. 6.2-6.4
  2. Yu and Jasani (2024) Crit Dec Emerg Med 38(1): 27-34

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