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Clinical Case Challenges . In Neuro-Optometry II Thomas J. Landgraf, O.D., F.A.A.O. Oops Almost Forgot!. My Email landgraft@umsl.edu. Case #3: Instead Of A Case. Sildenafil…Does the “little blue pill” cause big problems? Viagra Erectile Dysfunction: 30 million men Best Seller List

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Clinical Case Challenges


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clinical case challenges

Clinical Case Challenges

In Neuro-Optometry II

Thomas J. Landgraf, O.D., F.A.A.O.

oops almost forgot
Oops Almost Forgot!
  • My Email
  • landgraft@umsl.edu
case 3 instead of a case
Case #3: Instead Of A Case
  • Sildenafil…Does the “little blue pill” cause big problems?
  • Viagra
    • Erectile Dysfunction: 30 million men
    • Best Seller List
    • Association with AION-non-arteritic
case 3 instead of a case4
Case #3: Instead Of A Case
  • Viagra
  • Newer agents
    • Levitra (vardenafil hydrochloride)
    • Cialis (tadalfil)
      • 2 reported cases of NA-AION
case 3 instead of a case5
Case #3: Instead Of A Case
  • Viagra
  • FDA 2005
    • Updated labeling for all 3 drugs
    • Information on possibility of vision loss
case 3 instead of a case6
Case #3: Instead Of A Case
  • Viagra: How does it work?
    • Inhibits phosphodiesterase-5
    • Normally degrades cGMP
    • cGMP normally relaxes smooth muscle and increased blood flow
case 3 instead of a case7
Case #3: Instead Of A Case
  • Viagra: dosage
    • Standard 25-50 mg
      • 100 mg tabs also available
    • Once a day at the most
    • 2-3 x / week
case 3 instead of a case8
Case #3: Instead Of A Case
  • Viagra: systemic side effects
  • Due to effects on phosphodiesterases
  • Potentiates vasodilation
    • HA, flushing, dyspepsia, nasal congestion, cardiovascular, cerebrovascular, vascular
    • Visual disturbances
case 3 instead of a case9
Case #3: Instead Of A Case
  • Viagra: ocular side effects
  • Due to effect on enzyme on photoreceptors and alterates cGMP in the cones
    • Bluish tinge to vision
    • Increased light sensitivity
    • Haloes
    • Infrequent at low dosages
    • 50% if > 200 mg
    • Within hours
case 3 instead of a case10
Case #3: Instead Of A Case
  • Viagra and NA-AION
    • Since 1999, > 20 cases involving ingestion
    • Blur, altitudinal VF loss, edematous disc
    • “disc at risk”: small cup, crowded optic nerve head
    • Ischemia at prelaminar portion of optic nerve
case 3 instead of a case11
Case #3: Instead Of A Case
  • Viagra and NA-AION
  • At risk patients?
    • Small vessel occlusive cerebrovascular disease
    • HTN, DM, elevated cholesterol
    • Smokers
    • After age 50
case 3 instead of a case12
Case #3: Instead Of A Case
  • Viagra and NA-AION
  • Why?
    • Perhaps effect via NO-cGMP pathway
    • Nitric oxide & cyclic gaunosine monophosphate
    • Interferes with autoregulation of ocular blood flow
case 3 instead of a case13
Case #3: Instead Of A Case
  • Viagra: Our Role
    • Include ED drugs in DDx of NA-AION
    • Ask all male patients about use of ED meds
    • Inform about potential risks, especially if already had monocular NA-AION
    • Annual exam with DFE : keep those patient #’s up
case 3 instead of a case14
Case #3: Instead Of A Case
  • Viagra: Bottom Line
    • All over the news and in the literature
    • Patients aware, we must be too!
    • Most recent articles BY FAR
    • Data will continue to develop
aion non arteritic
AION-non-arteritic
  • Background
    • Most common cause of acute optic neuropathy > 50
    • Remains a disease without treatment or prophylaxis
    • Recently: Ischemic Optic Neuropathy Decompression Trial and Follow-Up Study
aion non arteritic16
AION-non-arteritic
  • Why?
    • “a short posterior ciliary artery problem”
      • Small branches occluded or hypoperfused
      • Already have an affected vasculature if small C/D
      • HTN and DM  vaso-occlusion
aion non arteritic17
AION-non-arteritic
  • Why… per Hayreh
    • “dysfunctional vascular autoregulatory mechanisms at the level of the optic nerve”
      • Transient nocturnal hypotension
      • Overtreatment of systemic hypertension
aion non arteritic18
AION-non-arteritic
  • Diagnosis: Who?
    • Men and women > 50 years
    • Caucasians
    • 1500-5700 new cases / year
    • Majority of patients have DM, and / or HTN
    • Cigarette smokers
    • C/D < 0.1
aion non arteritic19
AION-non-arteritic
  • Diagnosis: Symptoms
    • Vision loss: gradual over weeks
      • Scotoma or blur
      • Some more abrupt and without warning
      • < 20/60 within 30 days
    • Peri-ocular discomfort in 10%
      • Usually painless
aion non arteritic20
AION-non-arteritic
  • Diagnosis: Signs
    • VA ends up between 20/60-20/200
    • Inferior altitudinal VF defect
      • Inferior nasal and cecocentral too
    • APD
aion non arteritic21
AION-non-arteritic
  • Diagnosis: Signs
    • Optic disc edema needed for diagnosis
      • With flame hemorrhages
      • Hyperemic
      • Dilated and tortuous retinal veins
    • Contralateral small C/D or “disc-at-risk”
aion non arteritic22
AION-non-arteritic
  • Differential Diagnosis
  • Dr. Gray “Diagnosing In The Negative”
    • Vs. AION-arteritic
      • Younger
      • Better VA
      • Lack of constitutional symptoms
      • CRP and ESR less likely to be elevated
      • Lack of cotton wool spots, artery occlusions
aion non arteritic23
AION-non-arteritic
  • Differential Diagnosis
    • Optic Neuritis
    • Inflammatory
      • Slowly progressive and intraocular inflammation
    • Infectious
    • Infiltrative (papilledema)
aion non arteritic24
AION-non-arteritic
  • Differential Diagnosis
    • Compressive
      • Slowly progressive
      • Orbital signs: proptosis
      • HA
      • Vision and VF loss
      • Optic nerve edema
      • GIT upset
      • Personality changes
      • Decreased psychomotor function
aion non arteritic25
AION-non-arteritic
  • Differential Diagnosis
      • Don’t forget about the demographics of AION
      • Everyone’s Level Of Comfort Is Different
        • Never Be Afraid To Get A Second Opinion
        • Try and teach the students…
        • The best O.D.’s know they don’t know it all
aion non arteritic26
AION-non-arteritic
  • Ancillary Tests
    • Optometric In-Office
      • VF’s
      • Optic Nerve Imaging
      • Photos
      • FLAN?
        • Unless checking for choroidal perfusion defects in arteritic AION
aion non arteritic27
AION-non-arteritic
  • Ancillary Tests
    • Referral
      • CRP
      • ESR
    • Probably all that is needed for typical AION-non-arteritic
aion non arteritic28
AION-non-arteritic
  • Management
    • Referral to PCP
      • Diagnosis and management of DM, HTN and atherosclerosis
      • But not a direct marker for impending stroke or heart event
    • Stop smoking
    • 1 month follow-up with you
aion non arteritic29
AION-non-arteritic
  • Management
    • No effective medical or surgical treatment
      • Concentrate on:
      • DM, HTN, cholesterol
      • Smoking cessation
aion non arteritic30
AION-non-arteritic
  • Management
    • Ischemic Optic Neuropathy Decompression Trial
      • No role for optic nerve sheath decompression in AION-non-arteritic
      • Poor efficacy and high risk
    • Aspirin often recommended
      • To prevent contralateral involvement
      • Little supportive data
aion non arteritic31
AION-non-arteritic
  • Management
    • Neuroprotective Agents
      • Menatine benefical in animal models
      • Brimonidine in human trials: thus far no efficacy
aion non arteritic32
AION-non-arteritic
  • Management: Patient education
    • Most patients relatively stable vision-wise
      • Give or take a few lines
    • Up to 1/5 develop contralateral disease
    • ONH edema resolves within a month 
      • Atrophic
aion non arteritic33
AION-non-arteritic
  • My Clinical Experience
    • Not uncommon
    • See it sometimes after the fact
    • Complicates glaucoma
case 4 the bwi connection
Case #4 The BWI Connection
  • History
    • 19 yo African-American female
    • Moderate blur OS
    • Associated with HA , pain, and pressure OS about 1 month ago
    • Went to PCP and then referred to eye clinic in DC
    • Doc did not finding anything wrong…was told it was “sinus-related”
case 4 bwi
Case #4: BWI
  • History
    • Back in Memphis to see family
    • Mom recommends The Eye Center at SCO
    • No significant medical or ocular history
    • Pt is overweight…why mention?
case 4 bwi36
Case #4:BWI
  • Exam
    • BCVA: 20/20, 20/25+
    • EOM’s: FROM without diplopia
    • Pupils: grade 1-2 APD OS
    • Confrontation fields: FTFC OU
    • Amsler normal OU
case 4 bwi37
Case #4: BWI
  • Exam
    • SLX essentially normal OU
    • IOP: 27, 25
    • DFE:
      • .4/.4 with healthy rim OU
      • Macula clear OU
      • Periphery clear OU
case 4 bwi39
Case #4: BWI
  • Ancillary tests ordered
    • Photos
    • VF’s: Humphrey 24-2
    • Why no optic nerve imaging?
case 4 bwi42
Case #4: BWI
  • Assessment
    • 1. Retrobulbar Optic Neuritis OS
      • Eye pain, APD, central scotoma, decreased VA
    • 2. Glaucoma suspect OU
      • Increased IOP
    • 3. CMA OU
case 4 bwi43
Case #4: BWI
  • Plan…hmmmm
    • Patient concerned about cost of visit to neurologist
    • Wanted to see neurologist when she returned to DC in 2 weeks
    • Insurance coverage through college
    • Is this OK?
case 4 bwi44
Case #4: BWI
  • Plan
    • 1. Refer to neurologist for further management as deemed appropriate
      • R/O Multiple Sclerosis
      • Educated patient on possible etiologies, importance of seeing neurologist, and vision prognosis
    • 2. Recall in 6 months
    • 3. No new Rx recommended
optic neuritis
Optic Neuritis
  • Background
    • Acute inflammation of the optic nerve
      • Vs. “Demyelinating Optic Neuropathy”
    • Initial presentation of Multiple Sclerosis (MS)
    • Recognition aids with diagnosis in early course
    • Available treatments may minimize worse effects
optic neuritis46
Optic Neuritis
  • Background: historically
    • “blindness a divine punishment for sin”
    • Optic neuritis may have accounted for “miraculous” spontaneous cures
optic neuritis47
Optic Neuritis
  • Background: as it relates to MS
    • 75% of patients
    • 25% initially
    • Visual prognosis is good
      • 12 months, nearly all have 20/20
      • 5 years, only 6% < 20/40
ms additional ocular manifestations
MS: Additional Ocular Manifestations
  • Diplopia
    • INO or BINO (bilateral internuclear ophthalmoplegia)
    • CN VI, III, IV palsy
  • Nystagmus
optic neuritis49
Optic Neuritis
  • Background: MS
    • Chronic inflammatory condition
      • Affects white matter in CNS
    • Autoimmune response in genetically predisposed
    • Prevalence < 1%
    • Leading cause of disability in “young adults”
optic neuritis50
Optic Neuritis
  • Why? MS
    • Destruction of myelin sheath
      • Slows nervous conduction
    • Random patches: plaques
    • Associated with wide range of neurologic sxs
optic neuritis51
Optic Neuritis
  • Why?
    • Demyelinating optic neuropathy 
    • Damages fibers in visual and pupillary pathways 
    • Decreased VA and APD
optic neuritis52
Optic Neuritis
  • Background: MS
    • Systemic manifestations: variable in severity and duration
      • Muscle weakness: extremities
      • Trouble with coordination, balance
      • Paresthesias
      • Cognitive problems
      • Uhthoff’s sign
optic neuritis53
Optic Neuritis
  • Diagnosis of MS: Who?
    • Females
    • Age of diagnosis: 20-40 years
    • Northern US
optic neuritis54
Optic Neuritis
  • Diagnosis: Symptoms
    • Blur
    • Eye pain with movement initially
    • HA
optic neuritis55
Optic Neuritis
  • Diagnosis: Signs
    • APD
    • VF defects: central, cecocentral, altitudinal, generalized depression
    • Color vision defects
    • Normal optic nerve head appearance
      • Inflammation is retrobulbar majority of time
      • Papillitis in remaining 35%
optic neuritis56
Optic Neuritis
  • Differential Diagnosis: demyelinating most common
    • Infection: syphilis
    • Infiltrative : lupus
    • Ischemic: DM, HTN
    • Compressive
optic neuritis57
Optic Neuritis
  • Ancillary Tests: Optometric In-Office
    • VF
    • Photos
    • Optic nerve imaging
optic neuritis58
Optic Neuritis
  • Ancillary Tests: Referral
    • MRI: gadolinium-enhanced of the brain and orbits
      • Brain lesions on initial MRI increase risk of Clinically Definite MS (CDMS) at 5 years
      • Brain lesions: T2 ovoid high-signal white matter lesions located in perivenular regions perpendicular to ventricles
    • CSF evaluation
    • VEP
optic neuritis59
Optic Neuritis
  • Management
    • Referral: neurologist, neuro-eye doc
    • ONTT (Optic Neuritis Treatment Trial)
      • High dose IV steroids followed by orals
        • IV methylprednisone (Solumedrol) 250 mg qid x 3 days
      • Hastens visual recovery
      • No long term vision benefits
      • Short term reduction in MS development rate
      • Use of oral steroids alone is contraindicated
optic neuritis60
Optic Neuritis
  • Management
    • Interferon
      • Effective in preventing progression
        • Future neurologic events
        • Worsening of brain MRI
      • Treat at presentation
optic neuritis61
Optic Neuritis
  • Management
    • Interferon
      • CHAMPS: The Controlled High Risk Avonex Multiple Sclerosis Study
      • Initial clinical episode of optic neuritis + at least two demyelinating brain lesions  Avonex
optic neuritis62
Optic Neuritis
  • Management: Prognosis
    • Majority will develop relapsing, remitting MS
    • CDMS
      • 30% of optic neuritis after 5 years
      • Relatively benign course for 10 years though
optic neuritis63
Optic Neuritis
  • Management: Prognosis
    • 15-25 years post diagnosis: mobility assistance, significant neurologic disability
    • “One Day At A Time”
optic neuritis64
Optic Neuritis
  • Management: Education
    • The course of Optic Neuritis is predictable
    • The course is MS is unpredictable
optic neuritis65
Optic Neuritis
  • Management: Follow-up
    • Within a month
      • VA and VF essential
      • Monitor for steroid ocular side effects?
    • Every 3-6 months thereafter seems appropriate
optic neuritis66
Optic Neuritis
  • My SCO Experience
    • Not uncommon
    • Always a young adult female
      • Recent patients all overweight
    • I discuss the good and the bad
case 5 thinking of pco
Case #5: Thinking Of PCO
  • Thanks Dr. Wormington
  • Great article and case: JAOA 1989
    • 36 yo African-American male
    • Routine eye exam
    • Mild HTN under medical control
case 5 thinking of pco68
Case #5: Thinking of PCO
  • Exam
    • BVA
      • OD: 20/25 but missing some letters on lines from 20/60 to 20/25
      • OS: 20/20
    • CF’s
      • OD: depression on temporal side
    • Pupils: PERRL without APD
    • Fundus: slight temporal ONH pallor OD
case 5 thinking of pco69
Case #5: Thinking of PCO
  • Referral to TEI at PCO 11 days later
    • H/O infertility x 10 years, short period of HA’s 2 years prior
    • BVA 20/80 OD, 20/20 OS
    • Goldmann VF’s
      • Essentially complete temporal hemianopia OD
anatomy review
Anatomy Review
  • A. Carotid artery
  • B. Trochlear nerve
  • C. Maxillary nerve
  • D. Abducens nerve
  • E. Sphenoid sinus
  • F. Pituitary gland
  • G. Cavernous sinus
  • H. Ophthalmic nerve
  • I. Oculomotor nerve
case 5 thinking of pco71
Case #5: Thinking of PCO
  • Assessment
    • Suspect compressive lesion
  • Plan
    • Refer for CT and MRI
    • Enhancing mass arising from sella turcica
    • Most likely a pituitary adenoma 20 x 15 mm
case 5 thinking of pco72
Case #5: Thinking of PCO
  • Hospital admission 4 days later
    • Infertility and gynecomastia revealed
    • 3 days later, BVA 20/200 and 20/25
    • Automated perimetry
      • Total right temporal VF loss
      • Superior temporal loss OS
    • Macroadenoma was surgically removed
case 5 thinking of pco73
Case #5: Thinking of PCO
  • 23 days post surgery
    • Marked improvement in VF’s
    • BVA: 20/20 OD and OS
  • Eventual external beam radiation for residual tumor
pituitary adenoma
Pituitary Adenoma
  • Background
    • “Real Estate Neuro”
    • Chiasmal lesions  visual symptoms
    • Discovery of intracranial lesion
      • Neurologic, endocrine, systemic sequelae
pituitary adenoma75
Pituitary Adenoma
  • Background
    • Typically slow-growing and benign
    • Up to 20% on autopsy
    • 10-15% of symptomatic neoplasms
    • Range in size: 1cm and up
pituitary adenoma76
Pituitary Adenoma
  • To secrete or not secrete
    • Secreting (endocrine-active)
      • Usually not detected by us
      • Prolactin, growth hormone, thyroid-stimulating, adrenocorticotropin, multiple-hormone
pituitary adenoma77
Pituitary Adenoma
  • To secrete or not secrete
    • Nonsecreting
      • 25% of pituitary tumors
      • Larger than secretors
pituitary adenoma78
Pituitary Adenoma
  • Why? Ocular signs and symptoms
    • “Location, location, location”
    • Size >= 10 mm
    • Chiasm impinged upon (8-13 mm above pituitary gland)
pituitary adenoma79
Pituitary Adenoma
  • Diagnosis: Who?
    • Early adulthood
    • Both sexes
    • Nonsecreting
      • Later in life, men
pituitary adenoma80
Pituitary Adenoma
  • Diagnosis: Symptoms
    • Visual or VF loss
    • Decrease depth peception
    • Diplopia
    • Headache
    • Photophobia
pituitary adenoma 120 point screening
Bitemporal hemianopsia

Central sparing OD,central involvement OS

Pituitary Adenoma: 120 Point Screening
pituitary adenoma82
Pituitary Adenoma
  • Diagnosis: Signs
    • Uni- or bitemporal VF defect
      • Depends on location
      • Complete if body of chiasm
      • Monocular if lesion small and affects uncrossed fibers of the ipsilateral optic nerve
      • Always respect vertical midline initially
pituitary adenoma83
Pituitary Adenoma
  • Diagnosis: Signs
    • Decreased VA
      • From anterior chiasm / distal optic nerve involvement
    • Pallor of the optic discs
      • Especially if chiasmal compression for weeks
      • Dependent on location like the VF
pituitary adenoma84
Pituitary Adenoma
  • Diagnosis: Signs
    • CN III, IV, VI palsy
      • If lesion extends into chiasm
      • Or hemifield slide phenomenon

Decreased facial sensation

If lesion extends into cavernous sinus

Seesaw nystagmus

pituitary adenoma85
Pituitary Adenoma
  • Diagnosis: secreting tumors
    • Clinical manifestations
      • Prolactinomas: amenorrhea, impotence
    • Gigantism & acromegaly
pituitary adenoma86
Pituitary Adenoma
  • Differential Diagnosis
    • Tilted Optic Disc Syndrome
      • Bitemporal defects do not respect the vertical midline
      • Nonhereditary congenital anomaly
      • Superotemporal disc elevated and inferiornasal disc posteriorly displaced
    • Bilaterally Enlarged Blind Spots
pituitary adenoma87
Pituitary Adenoma
  • Differential Diagnosis
    • Chronic retrobulbar optic neuritis, nutritional amblyopia, uncorrected refractive error, low-tension glaucoma, age-related maculopathy
    • Pituitary Apoplexy
pituitary adenoma88
Pituitary Adenoma
  • Differential Diagnosis: Chiasmal Disease Causes
    • Neoplasm
    • Inflammation
    • Infectious
    • Vascular
pituitary adenoma89
Pituitary Adenoma
  • Ancillary Tests: Optometric In-Office
    • VF’s
    • Optic nerve imaging
pituitary adenoma90
Pituitary Adenoma
  • Ancillary Tests: Referral
    • MRI indicated for chiasmal lesions
    • Photos
      • With gadolinium
    • Endocrine work-up
      • For hypo- or hyperpituitarism
      • Radioimmunoassay for prolactin hormone
pituitary adenoma91
Pituitary Adenoma
  • Management
    • Referral
      • Internist, endocronologist, oncologist
      • Neurologist, neuro-eye doc, neurosurgeon
    • Follow-up
      • VF’s and VA’s +
      • Monthly
      • As deemed appropriate thereafter: every 3-6 months
pituitary adenoma92
Pituitary Adenoma
  • Management
    • Visual and hormonal status normal
      • Monitored
    • For endocronologically significant
      • Medical treatment
      • Bromocriptine shrinks prolactin secretors
pituitary adenoma93
Pituitary Adenoma
  • Management
    • Evidence of tumor enlargement with vison compromise or hormonal effects
      • Excison
    • Transsphenoid resection
      • Procedure of choice
      • Dramatic improvements in vision within months
pituitary adenoma94
Pituitary Adenoma
  • My Clinical Experience
    • “Peripheral” involvement
    • Knew I needed to talk about the chiasm
    • All the VIP’s (very important parts) in terms of neuro hang out there