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

XIII. Management: Typical Optic Neuritis

  1. Hospital admission
  2. Corticosteroids
    1. Methylprednisolone 250 mg IV every 6 hours (or 1 g daily) for 3 days
      1. Bioequivalent oral Prednisone 1250 mg in divided doses/day for 3 days is used in some cases
    2. Follow with oral Corticosteroid course
      1. Prednisone 1 mg/kg/day for 11 days, with 3 day taper
    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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Related Studies

Ontology: Optic Neuritis (C0029134)

Definition (NCI) A disorder characterized by inflammation of the optic nerve. Causes include autoimmune disorders, infections, toxins, drugs, and multiple sclerosis. It may manifest with acute loss of vision and pain.
Definition (MSH) Inflammation of the optic nerve. Commonly associated conditions include autoimmune disorders such as MULTIPLE SCLEROSIS, infections, and granulomatous diseases. Clinical features include retro-orbital pain that is aggravated by eye movement, loss of color vision, and contrast sensitivity that may progress to severe visual loss, an afferent pupillary defect (Marcus-Gunn pupil), and in some instances optic disc hyperemia and swelling. Inflammation may occur in the portion of the nerve within the globe (neuropapillitis or anterior optic neuritis) or the portion behind the globe (retrobulbar neuritis or posterior optic neuritis).
Definition (CSP) inflammation of the second cranial nerve or optic nerve; commonly associated conditions include autoimmune disorders such as multiple sclerosis, infections, and granulomatous diseases.
Concepts Disease or Syndrome (T047)
MSH D009902
ICD9 377.30, 377.3
ICD10 H46 , H46.9
SnomedCT 194051001, 267743006, 194052008, 155189007, 194054009, 66760008
English Neuritides, Optic, Optic Neuritides, Optic Neuritis, Neuritis, Optic, OPTIC NEURITIS, Optic neuritis NOS, Optic neuritis, unspecified, optic neuritis (diagnosis), optic neuritis, Neuritis optic, Unspecified optic neuritis, Optic Neuritis [Disease/Finding], neuritis optic, Neuritis;optic, Unspecified optic neuritis (disorder), Optic neuritis NOS (disorder), Optic nerve inflamed, Optic nerve inflammation, Optic neuritis, ON - Optic neuritis, Optic neuritis (disorder), inflammation; nerve, optic, inflammation; optic nerve, n.opticus; inflammation, neuritis; optic, optic nerve; inflammation, optic; neuritis, Optic neuritis, NOS, Optic neuritis [dup] (disorder)
French NEVRITE OPTIQUE, Névrite optique SAI, Névrite optique, non précisée, Névrite optique
Spanish NEURITIS OPTICA, Neuritis óptica NEOM, Neuritis óptica no especificada, neuritis óptica no especificada, neuritis óptica [dup] (trastorno), neuritis óptica, SAI, neuritis óptica, SAI (trastorno), neuritis óptica no especificada (trastorno), neuritis óptica (trastorno), neuritis óptica, Neuritis óptica, Neuritis Óptica
German OPTIKUSNEURITIS, Neuritis optica NNB, Optikusneuritis, unspezifisch, Neuritis optica, Optikusneuritis
Dutch opticus neuritis NAO, neuritis optica, niet-gespecificeerde opticus neuritis, n.opticus; ontsteking, neuritis; optica, ontsteking; nervus, opticus, ontsteking; oogzenuw, oogzenuw; ontsteking, optica; neuritis, opticus neuritis, Neuritis optica
Italian Neurite ottica, non specificata, Neurite ottica NAS, Neurite ottica, Nevrite ottica
Portuguese Nevrite óptica NE, Neurite óptica NE, Nevrite óptica, NEVRITE OPTICA, Papilite Ocular, Neurite óptica, Neurite Óptica
Japanese 視神経炎、詳細不明, 視神経炎NOS, シシンケイエン, シシンケイエンNOS, シシンケイエンショウサイフメイ, 視神経炎
Swedish Synnervsinflammation
Czech nervus opticus - neuritida, Neuritida zrakového nervu, Neuritida optiku NOS, Neuritida optiku, blíže neurčená, Optická neuritida, neuritida optiku, zánět zrakového nervu
Finnish Näköhermon tulehdus
Russian OPTICHESKII NEVRIT, RETROBUL'BARNYI NEVRIT, ZRITEL'NOGO NERVA NEVRIT, NEIROPAPILLIT, NEVRIT OPTICHESKII, ЗРИТЕЛЬНОГО НЕРВА НЕВРИТ, НЕВРИТ ОПТИЧЕСКИЙ, ОПТИЧЕСКИЙ НЕВРИТ, РЕТРОБУЛЬБАРНЫЙ НЕВРИТ, НЕЙРОПАПИЛЛИТ
Korean 시신경염
Croatian OPTIČKI NEURITIS
Polish Zapalenie nerwu wzrokowego
Hungarian Opticus neuritis, Optikus neuritis k.m.n., Optikus neuritis, Optikus neuritis, nem meghatározott
Norwegian Neuritis optica, Optisk nevritt, Optikusnevritt, Synsnervebetennelse

Ontology: Retrobulbar Neuritis (C0085582)

Concepts Disease or Syndrome (T047)
MSH D009902
ICD10 H46.1
SnomedCT 267743006, 155189007, 230507009
English RETROBULBAR NEURITIS, Neuritides, Retrobulbar, Neuritis, Retrobulbar, Retrobulbar Neuritides, Retrobulbar Neuritis, retrobulbar neuritis, retrobulbar neuritis (diagnosis), Optic neuritis retrobulbar, Retro-bulbar neuritis, Neuritis retrobulbar, Neuritides, Posterior Optic, Neuritis, Posterior Optic, Optic Neuritides, Posterior, Optic Neuritis, Posterior, Posterior Optic Neuritides, Posterior Optic Neuritis, Retrobulbar neuritis NOS, neuritis retrobulbar, optic neuritis retrobulbar, retrobulbar optic neuritis, Retrobulbar neuritis, RBN - Retrobulbar neuritis, Retrobulbar neuritis (disorder), neuritis; optic, retrobulbar, optic; neuritis, retrobulbar
Portuguese Neurite Retrobulbar, NEURITE RETROBULBAR, Nevrite retrobulbar, Nevrite óptica retrobulbar
Spanish Neuritis Retrobulbar, NEURITIS RETROBULBAR, Neuritis retrobulbar, Neuritis óptica retrobulbar, neuritis retrobulbar (trastorno), neuritis retrobulbar
French Névrite optique rétrobulbaire, NEVRITE RETROBULBAIRE, Névrite optique rétro-bulbaire, Névrite rétro-bulbaire, Névrite optique postérieure, Névrite rétrobulbaire
German Retrobulbärneuritis, RETROBULBAERNEURITIS, retrobulbaere Neuritis, Neuritis optica retrobulbaris
Dutch retrobulbair neuritis, opticus neuritis retrobulbair, retrobulbaire neuritis, neuritis retrobulbair, neuritis; optica, retrobulbaris, optica; neuritis, retrobulbaris, Retrobulbaire neuritis
Czech Retrobulbární neuritida, Optická retrobulbární neuritida, retrobulbární neuritida, zánět očního nervu
Japanese キュウゴシシンケイエン, 球後視神経炎
Hungarian Neuritis retrobulbaris, Retrobulbaris optikus neuritis
Italian Neurite retrobulbare, Neurite ottica retrobulbare, Neurite ottica posteriore
Norwegian Neuritis optica retrobulbaris, Retrobulbær nevritt, Bakre synsnerve-betennelse