Unilateral Optic Disc Swelling: Differential Diagnosis & Evaluation Karl C. Golnik, MD, MEd Professor & Chairman Department of Ophthalmology University of Cincinnati & the Cincinnati Eye Institute
Objectives • List the differential diagnosis of unilateral optic disc swelling. • Outline the diagnostic approach to the patient with unilateral optic disc swelling.
Unilateral Optic Disc Swelling* Inflammation Ischemia • • Typical optic neuritis (idiopathic, NAION • AAION demyelinating) • Atypical Optic Neuritis • * This does not represent an Hypoperfusion (anemia, blood (perineuritis, neuromyeltis optica, loss, hypotension, DM) sarcoid, autoimmune (AON), chronic exhaustive list of causes! Infiltration relapsing inflammatory (CRION), other systemic (SLE, Wegeners) • Lymphoma, Leukemia, other Infection Compression • Syphilis, Lyme, Bartonella, TB, HZV, • Tumor, Graves, ICP, drusen CMV, sinusitis Miscellaneous Hereditary – Leber? hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO
Unilateral Optic Disc Swelling* Inflammation Ischemia • • Typical optic neuritis (idiopathic, NAION • AAION demyelinating) • Atypical Optic Neuritis • Hypoperfusion (anemia, blood (perineuritis, neuromyeltis optica, loss, hypotension, DM) sarcoid, autoimmune (AON), chronic Infiltration relapsing inflammatory (CRION), other systemic (SLE, Wegeners) • Lymphoma, Leukemia, other Infection Compression • Syphilis, Lyme, Bartonella, TB, HZV, • Tumor, Graves, ICP, drusen CMV, sinusitis Miscellaneous Hereditary – Leber? hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO
Optic Neuritis Nonspecific term describing optic nerve involvement by inflammation infection, or demyelination.
Typical Optic Neuritis • Demyelinating, Idiopathic • Mild disc swelling (no exudate/hemorrhage) • Not steroid dependent Atypical Optic Neuritis • Disc swelling may be severe (with exudate/hemorrhage) • May be steroid dependent
Atypical Optic Neuritis Inflammatory • Neuromyeltis Optica (Devics) • Sarcoid • Autoimmune Optic Neuropathy (AON) • Chronic Relapsing Inflammatory Optic Neuropathy (CRION) • Other systemic (SLE, Wegeners) Infectious • Syphilis, Lyme, Bartonella, TB Chikungunya virus Optic Neuritis Associated With Chikungunya Virus Infection in South India. Apoorva M. et al. Arch Ophthalmol 2007; 125:1381.
• VA 20/200 OD 20/20 OS • Pupils RAPD OD • VF OD shown • VF OS normal Diagnosis? Evaluation?
spectrum of VF defects in the ONTT Keltner et al. Arch Ophthalmol 1994;112:946.
Typical Optic Neuritis (Demyelinating/Idiopathic) retrobulbar neuritis papillitis 65% 35%
ONTT - Visual Outcome Visual acuity 20/40 or better: placebo 94.3% IV steroids 93.7% oral steroids 92.6% IV only affects rate of recovery - not final vision!
ONTT: Development of MS at 15 years • 50 % regardless of MRI • 25% if MRI normal at onset • 72% if ≥ 1 plaque at onset Arch Neurol 2008 65:727.
ANN NEUROL 2011; 69:292-302.
• VA 20/200 OD 20/20 OS • Pupils RAPD OD • VF OD shown • VF OS normal Diagnosis? Evaluation?
Atypical Optic Neuritis Inflammatory • Neuromyeltis Optica (Devics) • Sarcoid • Autoimmune Optic Neuropathy (AON) • Chronic Relapsing Inflammatory Optic Neuropathy (CRION) • Other systemic (SLE, Wegeners) Infectious • Syphilis, Lyme, Bartonella, TB Chikungunya virus Optic Neuritis Associated With Chikungunya Virus Infection in South India. Apoorva M. et al. Arch Ophthalmol 2007; 125:1381.
Atypical Optic Neuritis • Important to diagnose because they are usually responsive to steroids and/or antibiotics and need treatment to improve vision. • May also be steroid dependent, relapsing as steroid dose is reduced sometimes requiring immunosuppression for many years.
Neuromyelitis Optica (Devic) • Idiopathic inflammatory demyelinating CNS disease • Unilateral or Bilateral optic neuritis • Transverse myelitis • Monophasic or polyphasic course
Neuromyelitis Optica - Diagnosis • Serum Biomarkers – NMO-IgG • Targets aquaporin-4, dominant water channel protein • Specificity reported 95-100% • Diagnostic Criteria: • Optic neuritis and acute myelitis + 2/3 of the following: • Spinal cord lesion extending >3 vertebral segments • Brain MRI findings not satisfying MS criteria • NMO-IgG seropositive Wingerchuk et al. Neurology 2006; 66(1), 1485-1489
NMO Treatment • IV Corticosteroids • Potentially decreases attack severity and speeds recovery • Therapeutic Plasmapheresis • Effective rescue treatment if steroid unresponsive • Chronic Immunosuppressive Treatment (if recurrent) • Azathioprine ± prednisone – most common treatment • Mitoxantrone • Mycophenolate Mofetil • Rituximab
Sarcoid can cause optic neuropathy with or without disc swelling. Check angiotensin converting enzyme, CXR if disc swelling is more than mild.
Unilateral Optic Disc Swelling* Inflammation Ischemia • • Typical optic neuritis (idiopathic, NAION • AAION demyelinating) • Atypical Optic Neuritis • Hypoperfusion (anemia, blood (perineuritis, neuromyeltis optica, loss, hypotension, DM) sarcoid, autoimmune (AON), chronic Infiltration relapsing inflammatory (CRION), other systemic (SLE, Wegeners) • Lymphoma, Leukemia, other Infection Compression • Syphilis, Lyme, Bartonella, TB, HZV, • Tumor, Graves, ICP, drusen CMV, sinusitis Miscellaneous Hereditary – Leber? hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO
Neuroretinitis - optic disc swelling and macular star of exudate. cat scratch disease, idiopathic, toxoplasmosis, tuberculosis, syphillis NOT MS!
Syphilis Cases
Infectious Optic Neuropathy Summary • Presents like any subacute/acute optic neuropathy • Must know disease prevalence in your region • Prognosis probably related to treatment timing
Unilateral Optic Disc Swelling* Inflammation Ischemia • • Typical optic neuritis (idiopathic, NAION • AAION demyelinating) • Atypical Optic Neuritis • Hypoperfusion (anemia, blood (perineuritis, neuromyeltis optica, loss, hypotension, DM) sarcoid, autoimmune (AON), chronic Infiltration relapsing inflammatory (CRION), other systemic (SLE, Wegeners) • Lymphoma, Leukemia, other Infection Compression • Syphilis, Lyme, Bartonella, TB, HZV, • Tumor, Graves, ICP, drusen CMV, sinusitis Miscellaneous Hereditary – Lebers? hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO
22-yo-wm c/o blurry vision OS x 1 month. VA: 20/15 OD, 20/200 OS, HRR: 10/10 OD, 0/10 OS P: L-RAPD 2 wks later OD 20/200 Lebers Hereditary Optic Neuropathy
Unilateral Optic Disc Swelling* Inflammation Ischemia • • Typical optic neuritis (idiopathic, NAION • AAION demyelinating) • Atypical Optic Neuritis • Hypoperfusion (anemia, blood (perineuritis, neuromyeltis optica, loss, hypotension, DM) sarcoid, autoimmune (AON), chronic Infiltration relapsing inflammatory (CRION), other systemic (SLE, Wegeners) • Lymphoma, Leukemia, other Infection Compression • Syphilis, Lyme, Bartonella, TB, HZV, • Tumor, Graves, ICP, drusen CMV, sinusitis Miscellaneous Hereditary – Leber? hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO
Anterior Ischemic Optic Neuropathy (AION)
Non-Arteritic AION (NAION) Age, HTN, DM, cholesterol 15% sequential (5yrs) 50% no change Disc-at-Risk 43% improve (some) Nocturnal Hypotension? 7% worsen Not carotid stenosis cup to big
Arteritic (AAION) vs. Non-Arteritic (NAION) Giant Cell (Temporal) pallid swelling suspicious Arteritis • Always need to consider & ask the right questions: • jaw claudication, scalp tenderness, HA, fatigue • Check ESR, CRP, CBC/platelets
AION - Causes Sildenafil (Viagra)? • 7 patients from 1999-2003 at 1 institution • men, age 50-69 • onset within 36 hrs • all had hypertension, dm, hypercholesterolemia • 4/7 disc-at-risk, 3/7 not specified Pomeranz et al. J Neuro-Ophthalmol 2005; 25:9-13. • 7 other previously reported men, age 48-62 • onset within 12 hrs, 5/7 with risk factors • 3/7 disc-at-risk, 4/7 not specified
AION - Mimic Amiodarone? (Cordone, Pacerone) From: Chen D, Hedges DR. Sem Ophthalmol 2003;18:169-73. Based on review of 73 patients.
Unilateral Optic Disc Swelling* Inflammation Ischemia • • Typical optic neuritis (idiopathic, NAION • AAION demyelinating) • Atypical Optic Neuritis • Hypoperfusion (anemia, blood (perineuritis, neuromyeltis optica, loss, hypotension, DM) sarcoid, autoimmune (AON), chronic Infiltration relapsing inflammatory (CRION), other systemic (SLE, Wegeners) • Lymphoma, Leukemia, other Infection Compression • Syphilis, Lyme, Bartonella, TB, HZV, • Tumor, Graves, ICP, drusen CMV, sinusitis Miscellaneous Hereditary – Leber? hypotony, trauma, vit-pap traction, pseudo, papillophlebitis, CRVO
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