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9/7/2021
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The Nerve-Racking Diagnosis: Swollen Optic Nerve Management
Acetazolamide in combo with low sodium diet resulted in decrease in papilledema, a decrease in CSF pressure and improved quality of life
Acetazolamide S/Es
**
Findings:• 9.4% (10/106) of patients with presumed IIH were
found to have CVST on MRV study• In only 1/10 was the CVST detected on MRI alone
What is CVST?A condition in which the balance between prothrombotic and prothrombolytic processes is disturbed leading to venous (blood clots) thrombosis
Symptoms:◦ Headaches (presenting sx in 70-90%)
◦ 18-38% of cases present with headaches, papilledema and visual disturbances resembling IIH
◦ Hemi-sensory disturbance
◦ Seizures
Associations:◦ Oral contraceptives
◦ Coagulation disorders, red blood cell disorders
◦ Head injury
◦ Post surgical
◦ Infection/inflammation
◦ Pregnancy
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CVST◦ Most often affects the larger sinuses (superior sagittal and lateral sinus)
◦ Can be seen with MRI, but potentially missed if MRV not performed
◦ Can be acute, subacute or chronic
◦ Can result in stroke or death
◦ Treatment: blood thinners
MRV in Papilledema WorkupMRV still debated as part of the routine papilledema workup◦ Low diagnostic yield with high cost of procedure vs missing a life-threatening
condition
◦ Particularly in those that are asymptomatic and fit the typical IIH profile
In a case of incidentally detected papilledema (aka no symptoms) and patient fits profile for IIH (overweight/obese female) without risk factors for thrombus -> MRI only (no MRV)
When in doubt…. order MRV
VITT and CVSTVaccine-induced immune thrombotic thrombocytopenia (VITT)
◦ Linked to AstraZenaca and J&J COVID vaccines
◦ likely response to adenoviral vector (AZ: chimpanzee AV 5; J&J: human AV 26)
Attributed as likely response to adenoviral vector ◦ AZ: chimpanzee AV 5; J&J: human AV 26
Of the cases reported, most common thrombotic event was CVST
VITT◦ Who is at risk?
◦ Young females (<60 years old)
◦ Incidence estimated at 4-5 per million doses
◦ Occurs ~7-10 days post-vaccination
◦ Proper diagnosis is key. These patients can’t be given heparin!◦ Tx: immunoglobulins and non-heparin anticoagulants
Incipient NA-ION• Subset of NA-ION• Swollen, hyperemic optic nerve
• Asymptomatic, VA and VF normal
• Often caught on routine exam
• (+)Risk factors for NA-ION
• 75% spontaneously resolve, 25% progress to full-blown NA-ION• study of 54 cases, median time for conversion 5.8 weeks (SS Hayreh, 2007, Ophthlamology)
Arteritic ION• Presenting s & sxs similar to NA-ION • Swollen and pale ON, “chalky” (vs hyperemic swelling)• A-ION more likely to report pain• Initial visual acuity is poor, >20/400• Possible preceding sxs of amaurosis fugax
• Central bright spot sign on MRI (Reymond P, Am J Neuroradiol, 2017)
• ON enhancement
Giant Cell ArteritisVasculitis affecting small to medium-sized arteries• F>M• Age >50, avg 72• Historically thought to be exclusive to Caucasians• Found equal incidence of biopsy proven-GCA between AAs and Caucasians (Guerner, 2019, JAMA)
Subclavian bruits, diminished pulses, aortic regurgitation, or Raynaud’s phenomenon are found in patients with large vessel disease
Treatment IV steroids followed by oral steroids (chronic, x years)• New tx FDA approved Actmera used in conjunction with steroids (allows for lower dose)
Clinical criteria most strongly suggestive of GCA:1) Jaw claudication2) CRP >2.45 mg/dl3) Neck pain4) ESR > 47 mm/hour
Posterior (P-ION)• Retrobulbar ischemia
• Sudden painless visual loss (VA and/or VF) in one or both eyes with a normal optic disc and fundus appearance
• Diagnosis of exclusion
• Arteritic (GCA), Non-arteritic or Perioperative/Surgical
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Case 336 year old AA female
CC: painless, progressive vision loss OS over 2 weeks, smudge in central vision
Med Hx: type II DM x 5 years, HTN, hyperlipidemia
Meds: Lantus, Glipizide, Metformin, Atorvastatin
BCVA: 20/20 OD, CF OS
Pupils: grade 2 APD OS
EOMs: full, denies pain
VF FDT: full OD, general depression OS
HRR: 10/10 OD, unable to see plates OS
IOP: 19/17 goldman
Ant seg: unremarkable
A/PAssessment36 year old AA female with ONH edema and acute vision loss OS, suspect optic neuritis
+ APD
Generalized VF defects
Elevated, swollen L optic nerve with hemorrhage
Vascular risk factors for NA-ION, but age atypical
(-) pain
PlanOrder MRI of brain w/ and w/o contrast to rule out demyelinating disease
3 weeks later
BCVA OS improved to 20/30
ONH swelling resolved
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5 months later
BCVA OS improved to 20/20
Mild ONH pallor appreciable OS
Optic Neuritis Acute optic nerve inflammation presenting with vision loss +/- pain on eye movement◦ Most often linked to demyelinating disease
F>M
Younger age compared to NA-ION pts; typically 20s-40s
+APD
2/3 have normal ONH appearance (aka retrobulbar)
Visual field loss ◦ most common centrocecal scotoma
Optic NeuritisGood prognosis for vision recovery◦ Most fully recover by 6 months
Sxs typically worsen over the first 1-2 weeks, then gradually recover over weeks
Rarely bilateral presentation◦ Except in Asian population, 30% of cases bilateral
Diagnosing demyelinating dzFinding of white matter lesions on MRI is the strongest predictor for development of MS◦ Abnormal MRI (1 or more lesions) -> 15 year risk of MS is 70%
◦ Compared to normal MRI (no lesions) -> 15 year risk of MS is 25%
Early MS Sxs (variable):◦ Numbness in extremities or face
◦ Fatigue
◦ Balance problems
◦ Bladder problems
◦ Uhthoff’s phenomenon: worsening of neurologic symptoms with a rise in body temperature
Optic neuritis treatment trial• Compared oral steroid, IV followed by oral and placebo.
• Steroids do not improve end VA in optic neuritis
• IV (3 days) followed by oral steroids (11 days) help to delay MS sxs up to 5 years
ON vs NA-ION
OPTIC NEURITIS
• Age 20s-40s
• 2/3 retrobulbar, if swelling mild w/o hemorrhage
• Most common VF defect: centroceccalscotoma
• (+) pain, dull, worsened with eye movement
NA-ION
• Age >40
• hyperemic, swollen disc +/- hemorrhage
• Presence of vascular risk factors
• Most common VF defect: altitudinal
• Small C/D ratio in contralateral eye
• (-) pain
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Atypical Optic NeuritisMost common cause of optic neuritis is demyelinating disease◦ But can also be autoimmune or infectious (bacterial, viral etc)
Optic neuritis workup should always include MRI of brain, may also necessitate a serology workup particularly if atypical findings…
Red flags for atypical ON: ◦ Unusual pt demographics◦ Bilateral signs or sxs◦ Vitreous and/or chorioretinal involvement
Post vaccine optic neuritis (non-infectious)◦ Can occur hours to weeks after the shot
Case 4• 46 year old Caucasian male in for routine exam with mild near blur OS
• Ocular Hx: trauma to OS, car accident at age 10, went through windshield, “left eye went out of socket” had reconstructive surgery on orbit, hx of poor vision OS since accident
• Med Hx: healthy, no meds
• BCVA: 20/20 OD, OS
• Pupils: tr-1 APD OS
• Color: HRR 9/10 OD, OS
• VF FDT: Cl OD, diffuse depression OS
•IOP: 15/16 mmHg
•Slit lamp: ant seg unremarkable
•Dilation: normal, ONHs 0.25/.3
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Case 4A: Hx of injury OS with decreased screening visual field
P: return in one month for HVF 30-2
Patient lost to follow up until….
2 years later…• CC: In for routine exam, no D/N’ blur, wants updated glasses, no change in health or meds
since last visit
• BCVA 20/20 OD, OS
• Pupils 3+ APD OS
• EOMs Full OU
• IOP 14/14 mmHg
• Visual Field FDT screener Cl OD, defects OS
• Color HRR 10/10 OD, 6/10 OS
Con’t• External: proptosis OS
• Ant seg: unremarkable
• DFE ONH: see photos
macula: clear and flat OD
mild ERM OS
periphery: normal OU
• Exophthalmometry: Base 100: 15 OD, 18.5 OS
Visual Fields HVF 30-2
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A/PAssessment
49 year old Caucasian male with unilateral disc edema… suspect compressive lesion
hx of L eye trauma
+ L Proptosis
+ L APD
Significant L VF defects
Elevated, swollen L optic nerve
PlanOrder MRI of brain and orbits w/ and w/o contrast
MRICompressive optic neuropathy
MRI Report
*Compared to head CT dated 9/7/2006
Compressive optic neuropathyInjury to the optic nerve by an extrinsic lesion
Compression can cause ON swelling, cupping or pallor
Any lesion or injury that produces mass effect can cause compression:◦ Tumors
◦ Orbital inflammation
◦ Grave’s/thyroid
◦ Orbital pseudotumor
◦ Traumatic
◦ Infectious
Thyroid Eye DiseaseHyper > hypo or euthyroid
High association with smoking
Severity scale:
0 No signs or sxs
1 Only signs (lid retraction, stare, lid lag)
2 Soft tissue involvement
3 Proptosis
4 EOM involvement
5 Corneal involvement
6 Sight threatening complications (ON involvement)
Thyroid Optic NeuropathyOptic nerve compression◦ Signs: APD, color vision defect, loss of VA, VF defect, ON
edema or pallor
◦ Neuroimaging shows crowding at the apex from muscle enlargement
◦ Treated with oral or IV steroids or orbital decompression surgery
◦ Tepezza (FDA approved Jan 2020) new treatment for TED◦ Reduces muscle and fatty tissue swelling to reduce proptosis
CT scan Radiopedia.org
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Case 531 year old AA female
CC: constant spot in L eye vision x 1 day
Med hx: type I DM (hospitalized one month ago with diabetic ketoacidosis), HTN, anemia
Meds: humalog, amlodipine, metoprolol
VA: sc 20/20 OD, OS
Pupils: sluggish, tr APD OS
EOMs: full OD, OS
VF screener: nonspecific defects, no pattern OD, OS
IOP icare: 22/20
BP: 125/80 mmHg
A/PSummary:31 year old AA female with uncontrolled type 1 DM and bilateral ONH edema and moderate NPDR OU• Hx suggestive for diabetic origin, recent hospitalization for
ketoacidosis, BS was in 600s and quickly stabilized
Plan: Obtain hospital records, must consider neuroimaging to rule out causes of papilledema
Received records. During hospitalization patient had brain MRI, head CT and lumbar puncture (all WNL). Since workup was so recent no further testing ordered. F/u in one month.
Case 5, 4 months laterCC: presents for urgent care, L red eye x 1 week
VA: sc 20/20 OD, OS
Pupils: sluggish, no APD
EOMs: full OD, OS
VF screener: clear OD, OS
IOP GAT: 18/19 mmHg
BP: 118/78 mmHg
Ant seg: WNL OD, temporal wedge of injection OS (episcleritis)
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Resolved ONH edema OD and OS
Diabetic Papillitis• Unilateral or bilateral ON edema
• Type 1 DM more often the type 2
• Often linked to rapid ↓ in BS
• +/- retinopathy
• ON swelling can be present for weeks-months
• Causes minimal impairment (if any) to VA/VF• self-limiting, resolving without need for treatment
• Diagnosis of exclusion• Bilateral ON edema must be treated as papilledema until proven otherwise
• Exact mechanism unclear…but may be similar to NAION?• disc edema persists longer in DP than in NAION
Case 631 year old AA female complaining of progressive vision loss OS, started 3 months ago, vision has declined substantially in last week
• Medical hx: healthy, no meds
• BCVA: 20/25 OD, HM OS
• Pupils: 2+ APD OS
• Color HRR: 7/10 plates OD, unable OS
• FDT VF: cecocentral defect OD, unable OS
• GAT: 16/16 mmHg
• Ant seg: unremarkable OU
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A/PSummary:31 year old AA female with probable LHON• +Fhx of vision impairment of multiple family members
• Brother has genetically confirmed LHON
Plan: No approved tx for LHONHelped pt to investigate active clinical trialsSet up LV consult