Clinical Case Challenges

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Clinical Case Challenges

• In Neuro-Optometry II
• Thomas J. Landgraf, O.D., F.A.A.O.

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Oops Almost Forgot!

• My Email
• landgraft_at_umsl.edu

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Case 3 Instead Of A Case

• SildenafilDoes the little blue pill cause big
  problems?
• Viagra
• Erectile Dysfunction 30 million men
• Best Seller List
• Association with AION-non-arteritic

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Case 3 Instead Of A Case

• Viagra
• Newer agents
• Levitra (vardenafil hydrochloride)
• Cialis (tadalfil)
• 2 reported cases of NA-AION

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Case 3 Instead Of A Case

• Viagra
• FDA 2005
• Updated labeling for all 3 drugs
• Information on possibility of vision loss

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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

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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

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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

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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 gt 200 mg
• Within hours

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Case 3 Instead Of A Case

• Viagra and NA-AION
• Since 1999, gt 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

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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

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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

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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

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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

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AION-non-arteritic

• Background
• Most common cause of acute optic neuropathy gt 50
• Remains a disease without treatment or
  prophylaxis
• Recently Ischemic Optic Neuropathy Decompression
  Trial and Follow-Up Study

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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

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AION-non-arteritic

• Why per Hayreh
• dysfunctional vascular autoregulatory mechanisms
  at the level of the optic nerve
• Transient nocturnal hypotension
• Overtreatment of systemic hypertension

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AION-non-arteritic

• Diagnosis Who?
• Men and women gt 50 years
• Caucasians
• 1500-5700 new cases / year
• Majority of patients have DM, and / or HTN
• Cigarette smokers
• C/D lt 0.1

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AION-non-arteritic

• Diagnosis Symptoms
• Vision loss gradual over weeks
• Scotoma or blur
• Some more abrupt and without warning
• lt 20/60 within 30 days
• Peri-ocular discomfort in 10
• Usually painless

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AION-non-arteritic

• Diagnosis Signs
• VA ends up between 20/60-20/200
• Inferior altitudinal VF defect
• Inferior nasal and cecocentral too
• APD

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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

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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

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AION-non-arteritic

• Differential Diagnosis
• Optic Neuritis
• Inflammatory
• Slowly progressive and intraocular inflammation
• Infectious
• Infiltrative (papilledema)

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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

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AION-non-arteritic

• Differential Diagnosis
• Dont forget about the demographics of AION
• Everyones 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 dont know it all

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AION-non-arteritic

• Ancillary Tests
• Optometric In-Office
• VFs
• Optic Nerve Imaging
• Photos
• FLAN?
• Unless checking for choroidal perfusion defects
  in arteritic AION

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AION-non-arteritic

• Ancillary Tests
• Referral
• CRP
• ESR
• Probably all that is needed for typical
  AION-non-arteritic

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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

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AION-non-arteritic

• Management
• No effective medical or surgical treatment
• Concentrate on
• DM, HTN, cholesterol
• Smoking cessation

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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

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AION-non-arteritic

• Management
• Neuroprotective Agents
• Menatine benefical in animal models
• Brimonidine in human trials thus far no efficacy

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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

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AION-non-arteritic

• My Clinical Experience
• Not uncommon
• See it sometimes after the fact
• Complicates glaucoma

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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 wrongwas told it
  was sinus-related

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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 overweightwhy mention?

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Case 4BWI

• Exam
• BCVA 20/20, 20/25
• EOMs FROM without diplopia
• Pupils grade 1-2 APD OS
• Confrontation fields FTFC OU
• Amsler normal OU

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Case 4 BWI

• Exam
• SLX essentially normal OU
• IOP 27, 25
• DFE
• .4/.4 with healthy rim OU
• Macula clear OU
• Periphery clear OU

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Case 4 BWI

• Photos

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Case 4 BWI

• Ancillary tests ordered
• Photos
• VFs Humphrey 24-2
• Why no optic nerve imaging?

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Case 4 BWI

• VF OD

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Case 4 BWI The Clincher

• VF OS

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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

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Case 4 BWI

• Planhmmmm
• 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?

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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

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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

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Optic Neuritis

• Background historically
• blindness a divine punishment for sin
• Optic neuritis may have accounted for
  miraculous spontaneous cures

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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 lt 20/40

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MS Additional Ocular Manifestations

• Diplopia
• INO or BINO (bilateral internuclear
  ophthalmoplegia)
• CN VI, III, IV palsy
• Nystagmus

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Optic Neuritis

• Background MS
• Chronic inflammatory condition
• Affects white matter in CNS
• Autoimmune response in genetically predisposed
• Prevalence lt 1
• Leading cause of disability in young adults

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Optic Neuritis

• Why? MS
• Destruction of myelin sheath
• Slows nervous conduction
• Random patches plaques
• Associated with wide range of neurologic sxs

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Optic Neuritis

• Why?
• Demyelinating optic neuropathy ?
• Damages fibers in visual and pupillary pathways ?
• Decreased VA and APD

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Optic Neuritis

• Background MS
• Systemic manifestations variable in severity and
  duration
• Muscle weakness extremities
• Trouble with coordination, balance
• Paresthesias
• Cognitive problems
• Uhthoffs sign

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Optic Neuritis

• Diagnosis of MS Who?
• Females
• Age of diagnosis 20-40 years
• Northern US

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Optic Neuritis

• Diagnosis Symptoms
• Blur
• Eye pain with movement initially
• HA

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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

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Optic Neuritis

• Differential Diagnosis demyelinating most common
• Infection syphilis
• Infiltrative lupus
• Ischemic DM, HTN
• Compressive

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Optic Neuritis

• Ancillary Tests Optometric In-Office
• VF
• Photos
• Optic nerve imaging

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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

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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

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Optic Neuritis

• Management
• Interferon
• Effective in preventing progression
• Future neurologic events
• Worsening of brain MRI
• Treat at presentation

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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

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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

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Optic Neuritis

• Management Prognosis
• 15-25 years post diagnosis mobility assistance,
  significant neurologic disability
• One Day At A Time

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Optic Neuritis

• Management Education
• The course of Optic Neuritis is predictable
• The course is MS is unpredictable

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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

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Optic Neuritis

• My SCO Experience
• Not uncommon
• Always a young adult female
• Recent patients all overweight
• I discuss the good and the bad

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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

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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
• CFs
• OD depression on temporal side
• Pupils PERRL without APD
• Fundus slight temporal ONH pallor OD

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Case 5 Thinking of PCO

• Referral to TEI at PCO 11 days later
• H/O infertility x 10 years, short period of HAs
  2 years prior
• BVA 20/80 OD, 20/20 OS
• Goldmann VFs
• Essentially complete temporal hemianopia OD

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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

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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

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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

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Case 5 Thinking of PCO

• 23 days post surgery
• Marked improvement in VFs
• BVA 20/20 OD and OS
• Eventual external beam radiation for residual
  tumor

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Pituitary Adenoma

• Background
• Real Estate Neuro
• Chiasmal lesions ? visual symptoms
• Discovery of intracranial lesion
• Neurologic, endocrine, systemic sequelae

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Pituitary Adenoma

• Background
• Typically slow-growing and benign
• Up to 20 on autopsy
• 10-15 of symptomatic neoplasms
• Range in size 1cm and up

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Pituitary Adenoma

• To secrete or not secrete
• Secreting (endocrine-active)
• Usually not detected by us
• Prolactin, growth hormone, thyroid-stimulating,
  adrenocorticotropin, multiple-hormone

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Pituitary Adenoma

• To secrete or not secrete
• Nonsecreting
• 25 of pituitary tumors
• Larger than secretors

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Pituitary Adenoma

• Why? Ocular signs and symptoms
• Location, location, location
• Size gt 10 mm
• Chiasm impinged upon (8-13 mm above pituitary
  gland)

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Pituitary Adenoma

• Diagnosis Who?
• Early adulthood
• Both sexes
• Nonsecreting
• Later in life, men

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Pituitary Adenoma

• Diagnosis Symptoms
• Visual or VF loss
• Decrease depth peception
• Diplopia
• Headache
• Photophobia

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Pituitary Adenoma 120 Point Screening

• Bitemporal hemianopsia
• Central sparing OD,central involvement OS

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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

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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

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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

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Pituitary Adenoma

• Diagnosis secreting tumors
• Clinical manifestations
• Prolactinomas amenorrhea, impotence
• Gigantism acromegaly

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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

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Pituitary Adenoma

• Differential Diagnosis
• Chronic retrobulbar optic neuritis, nutritional
  amblyopia, uncorrected refractive error,
  low-tension glaucoma, age-related maculopathy
• Pituitary Apoplexy

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Pituitary Adenoma

• Differential Diagnosis Chiasmal Disease Causes
• Neoplasm
• Inflammation
• Infectious
• Vascular

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Pituitary Adenoma

• Ancillary Tests Optometric In-Office
• VFs
• Optic nerve imaging

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Pituitary Adenoma

• Ancillary Tests Referral
• MRI indicated for chiasmal lesions
• Photos
• With gadolinium
• Endocrine work-up
• For hypo- or hyperpituitarism
• Radioimmunoassay for prolactin hormone

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Pituitary Adenoma

• Management
• Referral
• Internist, endocronologist, oncologist
• Neurologist, neuro-eye doc, neurosurgeon
• Follow-up
• VFs and VAs
• Monthly
• As deemed appropriate thereafter every 3-6 months

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Pituitary Adenoma

• Management
• Visual and hormonal status normal
• Monitored
• For endocronologically significant
• Medical treatment
• Bromocriptine shrinks prolactin secretors

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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

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Pituitary Adenoma

• My Clinical Experience
• Peripheral involvement
• Knew I needed to talk about the chiasm
• All the VIPs (very important parts) in terms of
  neuro hang out there