II. Epidemiology

  1. Young patients predominate (esp. age 20 to 45 years old)

III. Pathophysiology

  1. Immune-mediated inflammatory demyelination of the Optic Nerve
  2. Demyelination results in Retinal Ganglion cell axon degeneration

IV. Causes

  1. Multiple Sclerosis
  2. Encephalomyelitis
  3. Posterior Uveitis
  4. Optic Nerve vascular lesions
    1. Central Retinal Artery Occlusion
    2. Central Retinal Vein Occlusion
    3. Anterior Ischemic Optic Neuropathy
  5. Tumor
    1. Optic Nerve glioma
    2. Neurofibromatosis
    3. Meningioma
  6. Fungal infections
  7. Medications
    1. Aminosalicylic acid
    2. Chloramphenicol
    3. Ethambutol
    4. Isoniazid
    5. Penicillamine
    6. Phenothiazines
    7. Phenylbutazone
    8. Quinine
    9. Streptomycin

V. Risk Factors

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

VI. Associated Conditions

  1. Multiple Sclerosis
    1. Optic Neuritis is the presenting finding in 15-20% of Multiple Sclerosis (overal seen in 50% of MS cases)

VII. Symptoms

  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

VIII. Signs

  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

IX. Labs: Cerebrospinal Fluid

  1. WBC: Normal or 10-20 WBC present
  2. Oligoclonal bands may be present

X. Imaging

  1. Brain MRI and Orbit MRI with gadolinium contrast
    1. Confirm acute demyelinating Optic Neuritis
    2. Identify concurrent CNS findings suggestive of Multiple Sclerosis (e.g. periventricular white matter deficits)

XI. Management

  1. Hospital admit
  2. IV Glucocorticoids
    1. Methylprednisolone 250 mg IV every 6 hours for 3 days
    2. Reduces chance of future MS

XII. Course

  1. 30-50% develop Multiple Sclerosis within 15 years

XIII. References

  1. Yanoff (1999) Ophthalmology, Mosby, p. 6.2-6.4

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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