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Optic Neuropathy Part 2:Ischemic, Compressive, Hereditary
Karl C. Golnik, MD
Professor & ChairmanDepartment of Ophthalmology
University of Cincinnati & The Cincinnati Eye Institute
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Objectives
1. Differentiate different forms of ischemic optic neuropathy.
2. Identify presenting signs and symptoms of compressive optic neuropathy.
3. Describe at least 2 forms of hereditary optic neuropathy.
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Differential Diagnosis of Optic NeuropathyInflammation• Typical optic neuritis (idiopathic,
demyelinating)• Atypical Optic Neuritis
(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)
Infection• Syphilis, Lyme, Bartonella, TB, HZV,
CMV, sinusitis
Hereditary – Leber, Dominant
Ischemia• NAION• AAION• Hypoperfusion (anemia, blood
loss, hypotension, DM)
Infiltration• Lymphoma, Leukemia, other
Compression• Tumor, Graves
MiscellaneousNutritional, Toxic
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Differential Diagnosis of Optic NeuropathyInflammation• Typical optic neuritis (idiopathic,
demyelinating)• Atypical Optic Neuritis
(perineuritis, neuromyeltis optica, sarcoid, autoimmune (AON), chronic relapsing inflammatory (CRION), other systemic (SLE, Wegeners)
Infection• Syphilis, Lyme, Bartonella, TB, HZV,
CMV, sinusitis
Hereditary – Leber, Dominant
Ischemia• NAION• AAION• Hypoperfusion (anemia, blood
loss, hypotension, DM)
Infiltration• Lymphoma, Leukemia, other
Compression• Tumor, Graves
MiscellaneousNutritional, Toxic
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68-yo-WM c/o blurred vision OD x 1 week.
Exam: VA-20/60 (6/18) OD, +R-RAPD
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Most likely diagnosis?
1. Ischemic2. Compressive3. Nutritional4. Hereditary5. Inflammatory
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AIONAnteriorIschemic Optic Neuropathy
Blood supply problem to optic disc causes sudden, painless, visual loss
Diffuse Sectoral
Normal disc below
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Non-Arteritic AION (NAION)Age, HTN, DM, cholesterol
Disc-at-Risk
Sleep apnea
Nocturnal Hypotension?
Not carotid stenosis
15% sequential (5yrs)50% no change43% improve (some)
7% worsen
cup to big
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Giant Cell (Temporal) Arteritis
Always need to consider & ask the right questions:
jaw claudication, scalp tenderness, HA, fatigue
Check ESR, CRP, CBC/platelets
pallid swelling suspicious
Arteritic (AAION) vs. Non-Arteritic (NAION)
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AION - CausesSildenafil (Viagra)?
Pomeranz et al. J Neuro-Ophthalmol 2005; 25:9-13.
• 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
• 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
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Amiodarone or NAION?
• same risk factors as NAION• 1.3-1.8% optic neuropathy vs 0.3% NAION• $23,000,000 awarded (1997)
Treatment• stop or reduce dose (half-life 100 days!)
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AION - MimicAmiodarone?
(Cordone, Pacerone)
From: Chen D, Hedges DR. Sem Ophthalmol 2003;18:169-73. Based on review of 73 patients.
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Posterior Ischemic Optic Neuropathy (PION)
• relatively rare• severe vasculopath• post dialysis most common• usually need imaging
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Perioperative Visual Loss• 41-yo-WM injured in MVA. 10 hr low
back surgery done to relieve spinal cord compression.
• VA: NLP OU• Amaurotic pupils• Exam otherwise normal PION
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Perioperative Visual Loss• 26-yo-WM underwent low back surgery.
Procedure was uncomplicated but took 9 hrs. Required several units PRBC. Blood pressure kept 90/50-60.
• VA: 20/20• L RAPD
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Ischemic Optic Neuropathy• Most common cause of periop visual loss• 1/60,965 (0.0016%) all anesthetics• 1/1000 spine surgeries• Often bilateral blindness• Medical-legal issues
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Perioperative Visual Loss• case-control study:
• Only spine procedures• ION cases predominated• No difference in Hct or BP• Longer procedures• Greater blood loss
Myers MA, et al. Visual loss as a complication of spine surgery: a review of 37 cases. Spine 1997;22:1325-1329
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Perioperative Visual Loss
Proposed Factors• Hypotension or relative hypotension• Blood loss• Anemia• Hypoxia • Duration
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Perioperative Visual Loss
Proposed factors:• Hemodilution• Large crystalloid administration• Hypovolemia• Use of vasopressors• Venous congestion• Head position• Ocular compression
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Practice Advisory for Perioperative Visual Loss
• Etiology incompletely understood• Likely multifactorial• “High-risk patient”
• Prolonged prone spine surgery+/- substantial blood loss
Anesthesiology 2006; 104:1319-28.
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37-yo-WM c/o mild blurriness but notes visual loss left eye with L gaze.
ExamVA: 20/15, 20/20 HRR: 10/10, 4/10 P: L RAPDFundus: normal
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Most likely diagnosis?
1. Ischemic2. Compressive3. Nutritional4. Hereditary5. Inflammatory
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37-yo-WM c/o mild blurriness but notes visual loss left eye with L gaze.
ExamVA: 20/15, 20/20 HRR: 10/10, 4/10 P: L RAPDFundus: normal
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Compressive Optic Neuropathy
Historical Points
• gradual visual loss (beware sudden awareness)
• usually painless
• associated symptoms - often none
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Compressive Optic Neuropathy
Exam Characteristics
• signs of optic neuropathy (RAPD, visual loss)
• disc swelling if proximal compression
• choroidal folds
• optociliary collateral vessels
• orbital signs (proptosis, ophthalmoplegia, chemosis)
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ExamThe usual: RAPD, visual loss, decreased color vision
Disc swelling may occur if compression is immediately behind globe. Most compressive optic neuropathies have
no disc swelling.
retrobulbar with disc swelling
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Chorioretinal Folds
tumor Graves’papilledemaidiopathic
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Retinal-Choroidal Collateralsold CRVOmeningiomaCOAGidiopathic
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Orbital Signs
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42-yo-WM c/o “vision getting better!”
ExamVA: 20/15 (cc -2.25), 20/20 (plano)HRR: 10/10, 7/10 P: L RAPDFundus:
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Compressive Optic Neuropathy
Neuroimaging Pitfalls
1. No contrast given.
2. No fat suppression.
3. Scan only as good as the radiologist.
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Compressive Optic Neuropathy
Neuroimaging Pitfalls
1st MRI 2nd MRI
DX: metastatic Ewing’s Sarcoma to optic canal
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Compressive Optic Neuropathy
Neuroimaging Pitfalls
Dx: optic nerve sheath meningioma
with fat suppressionwithout fat suppression
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22-yo-wm c/o blurry vision OD x 1 month.
VA: 20/200 (6/36) OD, HRR: 0/10 OD, 10/10 OS
P: R-RAPD
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Most likely diagnosis?
1. Ischemic2. Compressive3. Nutritional4. Hereditary5. Inflammatory
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Leber’s Hereditary ON
• Severe (<20/200), painless, initially monocular visual loss • Associated RAPD & central or cecocentral VFD• Degeneration of ganglion cell layer & ON
• Males 10-30 yrs old• May occur later (reported up to age 65)• Females account 10-20% cases
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Leber’s Hereditary ON
• 2nd eye involved weeks-months• Rarely interval is longer (up to 8 yrs)
• Visual loss usually permanent, remaining <20/400• 10-20% cases report partial visual recovery
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Fundus appearance
http://www.mcw.edu/FileLibrary/Groups/Neurology/mcnamara.jpg&imgrefurl=http://www.mcw.edu/neurology/research/LHON.htm&usg
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Genetics
• Mitochondrial DNA mutation• 11778 position• Less commonly at 3460, 14484 locations
• 14484 mutations higher chance of late spontaneous improvement in VA (up to 65%)
• 11778 mutation have lower chance (~4%)
• Single base-pair nucleotide substitution results in impaired mitochondrial ATP production• Affects energy-dependent tissues (ON)• Can also affect heart, brain
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Genetics
• Transmitted from mitochondrial DNA
No proven treatmentIdebenone???
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Treatment
• No proven treatment with steroids, VB12, CN inhibitors
• Idebenone/Vitamin B2/Vitamin C • No difference in number of eyes overall with
visual recovery• Shortened the time for vision recovery• 11.1 months vs. placebo 17.4 months; p = 0.03
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Usually detected in asymptomatic childrenMutation at OPA 1 and OPA 3 genes
Dominant Optic Atrophy
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Retrobulbar Optic Neuropathy44-yo-wm c/o blurry vision OU x 1 month.
VA: 20/40 (6/12) OU
HRR: 2/10 OU
VF:
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Most likely diagnosis?
1. Ischemic2. Compressive3. Nutritional4. Hereditary5. Inflammatory
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Nutritional Optic Neuropathy(Tobacco-Alcohol Amblyopia)
• Gradual bilateral painless visual loss
• Typically in alcoholics who drink all their calories
• Central or Ceco-central scotomas
• Discs may be normal then atrophic
• Check vitamin B12, folic acid, rbc folate, B1, copper*
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Retrobulbar Optic Neuropathy66-yo-wm c/o blurry vision OU x 1 month.
VA: 20/40 (6/12) OU, HRR: 2/10 OU
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Retrobulbar Optic Neuropathy
20/100 OD, 20/80 OS
20/25 OU
Ethambutol
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Summary• Ischemic optic neuropathy usually is sudden,
painless and disc is swollen (NAION).
• Posterior ischemic optic neuropathy is rare outside dialysis, hypotension, surgery.
• Compressive optic neuropathy often painless – get the correct imaging!
• Leber HON may mimic optic neuritis
• Nutritional/Toxic optic neuropathy should be suggested by good history.
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Thank-you for your attention.