Transcript Clinical Case Challenges In Neuro-Optometry III

Clinical Case Challenges In
Neuro-Optometry III
Thomas J. Landgraf, O.D., F.A.A.O.
Before We Get Started…..

On our LAST hour
together

Foundations & Support
Groups
Myasthenia Gravis
 Multiple Sclerosis

Case #6: Don’t Assume Anything
 Or
“The Case Of The Chronic Sixth Nerve Palsy”
 70
yo male
 Diplopia with isolated abduction deficit OS
 Other
 No
eye movements intact
other significant neuro-eye findings
Case #6: Don’t Assume Anything

Or “The Case Of The
Chronic Sixth Nerve
Palsy”

Most likely a case of
ischemic sixth nerve palsy
Patient of vasculopathic age
 Sixth nerve palsy is isolated


May follow the patient
without neuro-imaging

Expectation of improvement
or resolution in 8-12 weeks
Case #6: Don’t Assume Anything

Or “The Case Of The Chronic Sixth
Nerve Palsy”

No expected recovery in 6-8 weeks upon reexam
Refer to neurologist
 MRI with gadolinium


DDx includes: a mass lesion

Pons, along the clivus, in the nasopharynx, at the
base of the brain, in the cavernous sinus, in the orbit
Case #6: Don’t Assume Anything
 Or
“The Case Of The Chronic Sixth Nerve Palsy”
 MRI
with gadolinium
 Large
pontine mass consistent with glioma
A death sentence in a young person
 Elderly, grows very slowly

Case #6: Don’t Assume Anything
 Or
“The Case Of The Chronic Sixth Nerve Palsy”
 Neurosurgical
 Monitor
 Prismatic
consult
without intervention
correction
CN VI Palsy
 Background
 Common
cause of horizontal diplopia
 Most commonly affected of the ocular motor nerves
CN VI Palsy
 Background
 Most
emergent
 Age
40 and under
 If non-isolated
 Think
microvascular in elderly
 If isolated, most patients recover fully and may not
require referral
CN VI Palsy
 Background:
Anatomy
 Longest
subarachnoid course
 Nucleus in the pons
 Innervates the ipsilateral lateral rectus
 T2-weighted
MRI in a patient with a chronic CN VI palsy
shows a left pontine glioma 
CN VI Palsy
 Diagnosis:
Who?
 Any
age
 Variety of causes
 Ischemia

most common in adults
Elderly with DM and HTN
CN VI Palsy
 Diagnosis:
 Variety
Who?
of causes
 Neoplasm
Mass lesion of CNS most common in children and young adults
 MRI of large posterior fossa tumor associated with hydrocephalus
and CN VI palsy in a 6 yo

 Inflammation

Post-viral and ear infections in kids
 Aneurysm
 Trauma
CN VI Palsy
 Diagnosis:
Symptoms
 Diplopia
 Binocular
or monocular?
 Horizontal or vertical?
 Pain,
especially if growing lesion in the cavernous
sinus
 Additional neurologic signs
 Depends
on the etiology
CN VI Palsy
 Diagnosis:
Signs
 Abduction
deficit
 Esotropia
 Maximum
palsy
 Head
turn
on gaze to the side of the
CN VI Palsy
 Diagnosis:
additional signs dependent on etiology
 Papilledema
with nausea, vomiting, tinnitus, HA’s
 Proptosis
 Ptosis
 Increased
ESR with HA and jaw claudication
 Retraction of globe and narrowing of lid fissure on
attempted abduction
CN VI Palsy
 Differential
Diagnosis
 Ischemia
 Inflammation
 Neoplasm
 Aneurysm
 Trauma
CN VI Palsy
 Ancillary
 Visual
Tests: Optometric In-Office
fields
 Forced duction?
CN VI Palsy
 Ancillary
Tests: Referral
 Indications
 Emergent
for Neuro-Imaging
if age < 30 years
 Head trauma
 Pain
 Non-isolated
 Other etiologies besides microvascular, myasthenia gravis,
thyroid, Giant Cell, congenital
CN VI Palsy

Ancillary Tests: Referral

Indications for Neuro-Imaging
Consult with neuro-eye doc or neurologist reassures:
 “that level of comfort thing again”


Workup if microvascular: DM, HTN
CN VI Palsy
 Management
 Microvascular,
 Resolve
trauma, idiopathic
spontaneously within 6 months
 Comfort: patch, blur, block, Botox for temporary treatment
 If need long-term: prism, surgery
CN VI Palsy
 Management
 Follow-up
VI  every 6 weeks over 6 months
 If you expect improvement?
 Neuro consult if no improvement
 CN
CN VI Palsy
 My
Clinical Experience
 All
isolated (majority) have been:
 Elderly
 Microvascular
 If
 All
it all makes sense, I hold off on the “Neuro-massage”
non-isolated:
 Younger
 Poor
prognosis
Case #7: A Quickie
 On-call
Resident
 Ptosis
OD
 Several months prior Ptosis OS
 Seen
 Don’t
by another resident
assume you are smarter than another resident
 Documentation
 What
was correct
is really going on?
Myasthenia Gravis
 Background
 Autoimmune
Disease
 Autoantibodies
 Abnormal
against acetylcholine receptors
fatigueability of muscles under voluntary
control
 Usually
orbital and facial muscles
Myastenia Gravis
 Background
 Prevalence:
 But
1:20,000
we see it!
 Ocular involvement: 90%
 Account for initial complaint in 75%
Myasthenia Gravis
 Diagnosis:
 Females
Who?
under 50 / 7:3
 Males peak in late 50’s
 Associated conditions: thymoma, thyroid disease,
diabetes, lupus, rheumatoid
Myasthenia Gravis
 Diagnosis:
Symptoms
 Majority
present with ocular
symptoms
 Ptosis:
asymmetric
 Diplopia: any motility defect
 And spread
 Variability of ocular fatigue
Worse at the end of the day
 Hx, Hx, Hx!

Myasthenia Gravis
 Diagnosis:
Symptoms
 Non-Ocular
 Within
two years of ocular
 Limb fatigue
 Facial muscle weakness

Difficulty breathing, chewing, talking, swallowing
Myasthenia Gravis
 Diagnosis:
Signs
 Ptosis
& EOM involvement
 Cogan’s lid twitch
 Exposure keratitis
 Ophthalmoplegia
 Orbicularis
oculi weakness
Myasthenia Gravis
 Differential
 Pupils
Diagnosis
are never affected
 No eye pain
 Thyroid ophthalmopathy,
 INO (Internuclear Ophthalmoplegia), orbital
pseudotumor, botulism, myotonic dystrophy, Chronic
Progressive External Ophthalmoplegia
Myasthenia Gravis
 Ancillary
 Measure
 Pupil
Tests: Optometric In-Office
palpebral apertures
center to upper lid margin
 Sustained
up gaze
 Squeezing of eyelids closed
 Initial VF
Myasthenia Gravis

Ancillary Tests: Optometric InOffice

Ice Pack Test
5 minutes
 Improves neuromuscular transmission 
ptosis
 Safe, speedy, easy and with relatively high
sensitivity and specificity

Sleep Test: eyes closed for 30 minutes
 FAT (Family Album Topography) Scan

Myasthenia Gravis
 Ancillary
Tests: Referral
 Tensilon
(Endrophonium HCL) Test
 IV
10 mg of Tensilon
 Why refer in Tennessee?
Rate of complications low but life-threatening
 Hypotension, bradycardia, cardiac arrest, respiratory arrest, seizures,
vomiting

 Improves
eyelid / motility defect
 Anticholinesterase
Myasthenia Gravis
 Ancillary
 EMG
Tests: Referral
(Electomyography)
 Acetylcholine antibody receptor test
Myasthenia Gravis
 Management
 Referral
 Neurologist,
neuro-eye doc
 Internist or PCP
 Lab
testing for associated conditions
 CT scan of chest / mediastinum for thymoma
Myasthenia Gravis
 Management:
Medical
 Anticholinesterases,
 Thymectomy
 Plasmapharesis
 IV
gammaglobulin
steroids, immunosuppressants
Myasthenia Gravis
 Management:
Optometric
 Lid
crutches, tape
 Occlusion
 Rarely prism, ptosis or strabismus surgery
 Follow-up as needed post-diagnosis
 Monitor
for steroid side effects
Myasthenia Gravis
 My
Clinical Experience
 Am
I missing this?
 Teaching about it may help…..
Case #8: OK To Not Refer?
 History
 2006
68 yo Caucasian female
 My
patient since 1997
 “my glaucoma drops are too expensive”

Alphagan-P bid OU
 H/O
thinner that normal pachymetry OU
 H/O highest tonometry 20 mm Hg OU
Case #8: OK To Not Refer?
 History:
 1997:
Of interest to us today…..
first visit
 Referred
for pupil and glaucoma work-up
 Anisicoria noted over 10 years ago
Case #8: OK To Not Refer?
 History
 HTN,
hypercholesterolemia
 H/O bypass
 Hyzarr, Metiprolol, Lipitor, Aspirin, Lyrica, vitamins
 No
wonder the glc drop is too expensive
 POAG,
Horner’s Syndrome, ERM
Case #8: OK To Not Refer?
 Exam
 BVA:
20/25+, 20/25+
 Pupils: anisocora
 Dim
illumination: 4, 6 mm
 Bright illumination: 3, 4 mm
 Ptosis:
1 mm upper lid OD
 EOM’s: FROM
 Confrontation fields: FTFC OU
Case #8: OK To Not Refer?
 Exam
 SLX:
essentially normal OU, mild NS OU
 T(a): 16, 15
 DFE
 .7/.7
OD, .6/.6 OS
 Macular pigmentary changes
 periphery clear OU
 Yearly
HRT, VF
Case #8: OK To Not Refer
 Latest
VF OD
Case #8: OK To Not Refer
 Latest
VF OS
Case #8: OK To Not Refer
 Latest
HRT OD
Case #8: OK To Not Refer
 Latest
HRT OS
Case #8: OK To Not Refer?
 Assessment
 1.
POAG OU
 Stable
HRT, VF, ONH appearance, IOP
 Alphagan-P too expensive
 2.
H/O Horner’s Syndrome OD
 Benign
 3.
and stable
Macular pigmentary changes
Case #8: OK To Not Refer?
 Plan:
 1.
Switch Alphagan-P to Brimonidine, RTC 1 month
IOP check, Education potential side effects of
Brimonidine
 2,3. To monitor
Case #8: OK To Not Refer?
 After
preparing this lecture
 Looked
way back in the record again
 10/5/83: “Anisocoria noted 1-2 years ago; neuroopthalmology work-up with no known causes”
 I never got the cocaine in ‘97
 Good idea eventually:
 Iopidine
 Paredrine
or Pholedrine
Horner’s Syndrome
 Background
 1852:
Claude Bernard first noted experimentally
 1869: Swiss ophthalmologist Johann Friedrich Horner
noted
 Should be called?
 Bernard’s
Syndrome
 Bernard-Horner’s Syndrome
Horner’s Syndrome
 Background:
 First
Sympathetic Anatomy of Eye & Face
order sympathetic neuron
 Begins
in ipsilateral hypothalamus
 Descends through midbrain, pons, and medulla
 Ends at ciliospinal center of Budge at levels of C8-T1 in the
spinal cord
Horner’s Syndrome

Background: Sympathetic
Anatomy of Eye & Face

Second order preganglionic fibers
Leave the ciliospinal center
of Budge
 Pass the pulmonary apex
 Travel up along carotid artery
sheath
 End in superior cervical
ganglion near bifurcation of
common carotid

Horner’s Syndrome

Background: Sympathetic Anatomy
of Eye & Face

Third order post-ganglionic neuron
Travels along internal carotid artery to the
cavernous sinus
 Leave the internal carotid, travel with the
abducens and join the ophthalmic division
of the trigeminal
 Enter the orbit with the naso-ciliary branch

Horner’s Syndrome

Background: Sympathetic Anatomy of Eye
& Face




In the orbit, the sympathetic fibers pass through
the ciliary ganglion
Join the two long ciliary branches of the
nasociliary nerve and innervate the iris dilator
muscle
Other sympathetic branches travel with branches
of the ophthalmic artery to innervate the
lacrimal gland, Muller’s muscles, and orbital
vessels
Sympathetic fibers controlling facial sweating
travel with the external carotid artery
Horner’s Syndrome
 Why?
 Dysfunction
of the sympathetic innervation to eye and
parts of face
 Interruption of oculosympathetic nerve supply
somewhere between hypothalamus and the eye
Horner’s Syndrome
 Diagnosis:
 No
Who?
predilection
 Age,
race, gender, geographics
 Congenital
 Presents
by age two with heterochromia
Horner’s Syndrome
 Diagnosis:
 Ptosis
 1-2
Signs
 Mullers muscle
mm upper eyelid
 Reverse
 Slight
 Miosis
ptosis
elevation lower eyelid
 iris dilator muscle
Horner’s Syndrome
 Diagnosis:
Signs
 Anisicoria
 Dilator
 Dilation
> darkness
iris muscle normally more active
lag: prolonged redilation of the pupil after
dimming the light
 Hypochromia Iridis: typical if congenital
 Anhidrosis
Horner’s Syndrome

Differential Diagnosis: not limited
to…..

Pre-Ganglionic ( First and SecondOrder)





LESS COMMON BUT MORE
OMINOUS
Pancoast Tumor
Tuberculosis
Aortic dissection
Internal carotid artery dissection



MRA (MR angiography)
Trauma
Stroke, syphilis, tumor, MS,
lymphadenopathy
Horner’s Syndrome

Differential Diagnosis

Post-Ganglionic (Third-Order)
Aneurysm
 Atherosclerosis
 Herpes Zoster
 Trauma
 Sinusitis


Painful Horner’s

Carotid dissection until proven othewise
Horner’s Syndrome
 Differential
 Vs
Diagnosis
Third Nerve Palsy
 Ptosis
 Anisicoria
exaggerated in dim illumination  Horner’s
Horner’s Syndrome
 Ancillary
 Pupil
Tests: Optometric In-Office?
Testing 1: Horner’s?
 Cocaine
blocks reuptake of norepinephrine at the sympathetic
nerve endings
Dilates normal eye after an hour
 No dilation in Horner’s due to lack of norepinephrine at nerve
endings

 Availability
of cocaine 10% solution?
Horner’s Syndrome

Ancillary Tests: Optometric In-Office
Pupil Testing 1: Horner’s?
 Alternative: Iopidine (Apraclonidine)

Weak, direct action on alpha-1 receptors
 Normal: no dilation
 Horner’s: dennervation supersensitivity to
norepinephrine  increase in alpha-1 receptors in
iris stroma  dilation

Horner’s Syndrome

Ancillary Tests: Optometric
In-Office

Pupil Testing 2: pre- or postganglionic?

Paredrine 1%
(Hydroxyamphetamine) releases
norepinephrine from stores in
nerve endings




Mydriasis in a normal pupil
No mydriasis in a postganglionic Horner’s due to
destroyed nerve endings
“Fail-safe”?
Postive Paredrine Test  OD
did not dilate
Horner’s Syndrome
 Ancillary
Tests: Optometric In-Office
 Pupil
Testing 2: pre- or post-ganglionic?
 Alternative: Pholedrine 5%
 With
third neuron damage
 Horner’s pupil will not dilate
 Post-ganglionic lesion
 If dilates 
Horner’s Syndrome
 Management:
 Neurologist,
Referral unless congenital
Neuro-Eye Doc
 PCP, Internist
 Cardiologist, Oncologist, Vascular Surgeon
 No treatment to improve or reverse the condition
Horner’s Syndrome

My Clinical Experience
Some pharmacist in
Memphis is upset with me!
 Glad we have Iopidine now
