Typical Optic Neuritis

    • Vision loss caused by demyelination of the optic nerve
    • Idiopathic or part of multiple sclerosis (MS)
    • No evidence of other pertinent systemic disease
    • Afferent pupil defect may be the only objective finding
    • Core features
      • Acute or subacute visual loss—usually in one eye
      • Periocular pain in the affected eye that is worsened by eye movement (90%)
      • Reduced visual acuity and/or nerve fiber bundle visual field defects in the affected eye
      • Trap: visual acuity may be 20/20 or better and visual field defects may be extremely subtle
      • Afferent pupil defect in the affected eye unless the fellow eye has equivalent optic nerve damage
      • Normal or minimally elevated optic disc and no other optic fundus abnormalities
    • Possible accompanying features
      • Clinical manifestations of MS
      • Previous diagnosis of MS
      • MRI enhancement of the affected optic nerve (80%)
      • Characteristic high T2/FLAIR focal MRI signal abnormalities in cerebral white matter, often oriented perpendicular to the long axis of the lateral ventricle and in the corpus callosum (50%)
      • Cerebrospinal fluid lymphocytic pleiocytosis, elevated immunoglobulins, oligoclonal bands, and/or myelin basic protein
      • Prolonged latencies in visual evoked potentials
      • Retinal nerve fiber layer thinning on optical coherence tomography when the optic neuropathy is chronic
    • Atypical optic neuritis
    • Ischemic optic neuropathy
    • Compressive optic neuropathy
    • Infiltrative (neoplastic) optic neuropathy
    • Leber hereditary optic neuropathy
    • Radiation-induced optic neuropathy
    • Order orbit-based MRI
    • Trap: although active optic neuritis causes enhancement of the affected nerve in 80% of patients, the enhancement may be subtle and inapparent unless high-definition, fat suppressed “orbital” MRI is performed; in typical optic neuritis, the enhancement will be in the nerve, not in its dural coating
    • Order serum aquaporin antibody to rule out neuromyelitis spectrum disorder (NMOSD) and serum myelin oligodendrocyte glycoprotein antibody to rule out myelin oligodendrocyte glycoprotein (MOG)-associated optic neuritis, especially if vision loss is marked, rapidly recurrent, or associated with longitudinally-extensive or perioptic enhancement and lack of abnormal cerebral hemispheric white matter signals on MRI
    • Order spine MRI and possibly lumbar puncture if the diagnosis of MS is in doubt
    • Offer intravenous methylprednisolone 1 gm/day for 3 days or equivalent oral doses, followed by prednisone 1 mg/kg for 11 days, acknowledging that…
    • Tip: corticosteroid treatment may very slightly hasten visual recovery, but will not improve ultimate visual outcome
    • Consider prophylactic disease-modifying treatment for patients who have white matter MRI abnormalities or other clinical features of MS
    • Tip: visual evoked potential testing and optical coherence tomography have limited diagnostic value in acute optic neuritis
    • Substantial recovery of vision commonly occurs within 6 months with or without corticosteroid treatment, but…
    • Subtle visual deficits and an afferent pupil defect commonly persist (80%)
    • Recurrent optic neuritis may occur in the previously affected or fellow eye within 5 years (30%)
    • Clinically definite MS will commonly develop within 15 years in patients with at least one typical MRI white matter lesion at the onset of optic neuritis (75%)
    • Clinically-definite MS will uncommonly develop within 15 years in patients with a normal MRI at the onset of optic neuritis (25%)
    • Severe long-term visual or neurological impairments will uncommonly develop (10%)
    • Prophylactic treatment for MS will often be recommended if there are MS-like MRI signal abnormalities

    Optic Nerve And Chiasm Disorders

    Drusen Optic Neuropathy Colobomatous Optic Neuropathy Optic Pit Neuropathy Morning Glory Optic Neuropathy Hypoplastic Optic Neuropathy Typical Optic Neuritis Atypical Optic Neuritis Papillitis (Neuroretinitis) Non-arteritic Ischemic Optic Neuropathy Arteritic Ischemic Optic Neuropathy Posterior Ischemic Optic Neuropathy Hypotensive Ischemic Optic Neuropathy Radiation-induced Optic Neuropathy Diabetic Papillopathy Hypertensive Optic Disc Edema Papilledema Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) Compressive Optic Neuropathy: Overview Optic Neuropathy of Graves Disease Optic Nerve Sheath Meningioma Sphenoid Meningioma Craniopharyngioma Pituitary Adenoma Pilocytic Astrocytoma (Optic Glioma) Carotid Aneurysm Suprasellar Germinoma Infiltrative (Neoplastic) Optic Neuropathy Paraneoplastic Optic Neuropathy Traumatic Optic Neuropathy Toxic Optic Neuropathy Nutritional Deficiency Optic Neuropathy Dominantly-Inherited Optic Neuropathy Leber Hereditary Optic Neuropathy Primary Open Angle Glaucoma