Ten easy mistakes to avoid in

your next neuro-ophthalmic

patient

Author: Andrew G. Lee

Review : Khalid A. Al-Mohammadi
MBBs, DO, MSc
Peshawar university–Pakistan
 Professor Andrew G. Lee
Chair of ophthalmology at the Blanton Eye
Institute , Houston Methodist Hospital,
Departments of Ophthalmology, Neurology, and
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Neurosurgery, USA
WE ONLY SEE WHAT WE LOOK
FOR .WE LOOK FOR ONLY WHAT
WE KNOW.
Johann Wolfgang von Goethe
1749-1832
 Introduction

 The busy ophthalmologist is confronted

by many rapid and important clinical
decisions every day, as routine
refractions and `red eyes` and might be
vision –threatening or life-threatening
disease.

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 Introduction

 This presentation will review some

practical recommendations for
avoiding the common mistakes in the
evaluation of the neuro-ophthalmic
patient.

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  A 45 years-old white female
Case study presents with:
- Headache and acute onset loss of
vision in left eye.
 Ocular examination shows :
• Her vision, right eye: 20/15;6/4,8
and the left eye:20/25; 6/9.
• The pupil exam, record `PERRLA`
(Pupils, Equal ,Round Reactive to
Light and Accommodation) and
dilates the patient.
• Slit lamp exam : left eye mild
nuclear cataract

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 • Fundus exam: Normal macula and
Case study optic nerve for both eyes.
• A diagnosis of ` left eye cataract`
is made and given a follow-up
appointment for six months
 the patient returns in three
months with the same complaint
and the exam is unchanged.
• Automated visual field is
`unreliable` because of a high
false positive , high false negative
and multiple fixation losses.
• A diagnosis of `worsening
cataract` is made and given a six
months appointment.

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  The patient seeks a second
Case study opinion………….. NOW:
• Her visual acuity, right eye:
20/15;6/4.8 (same) and the left
eye:20/40; 6/12 (worse).
• Pupillary exam : There is left
eye `RAPD` (Relative Afferent
Pupillary Defect)!!!!
• Slit lamp exam: nuclear
cataract but not compatible
with left eye visual acuity.
• Fundus exam: trace temporal
pallor of the left optic disc.
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 • Visual field shows :
Case study
*right eye, superotemporal visual
field loss.
*left eye , central depression with
breakout to the temporal
periphery.

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 • Magnetic resonance imaging
Case study (MRI) shows: a large suprasellar
mass

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 • The tumor is removed but the
Case study vision does not recover.

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What went wrong???

How can we avoid

making the same
error???

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 • Patient with 6/6 vision
First mistake
who read the Snellen line
ASSUME THAT 20/20;6/6 rapidly in one eye may on
VISUAL ACUITY IS NORMAL other eye read the 6/6
line slowly with lots of
guessing and effort. In this
setting 6/6 vision is not
normal.
• Patients should be
specifically questioned
regarding the exact nature
, circumstance , and
location of their visual
complaint
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First mistake • Many optic neuropathies
can present with normal
ASSUME THAT 20/20;6/6 visual acuity ( e.g.
VISUAL ACUITY IS NORMAL glaucoma, ……..).

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First mistake • Many optic neuropathies
can present with normal
ASSUME THAT 20/20;6/6 visual acuity ( e.g. disc
VISUAL ACUITY IS NORMAL drusen , papilledema)

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First mistake • Patients with bitemporal
or homonymous
ASSUME THAT 20/20;6/6 hemianopia may have 6/6
VISUAL ACUITY IS NORMAL visual acuity in both eyes

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First mistake • Many cases may be
described by the patient
ASSUME THAT 20/20;6/6 VISUAL as ` blurred vision` despite
ACUITY IS NORMAL 6/6 visual acuity
• Double vision (ocular
misalignment )

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First mistake • Many cases may be
described by the patient
ASSUME THAT 20/20;6/6 as ` blurred vision` despite
VISUAL ACUITY IS NORMAL 6/6 visual acuity
• Oscillopsia: vision problem in
which objects appear to jump ,
jiggle or vibrate.
The causes;
*loss of vestibulo-ocular reflex
(VOR) due to meningitis,
medicine like gentamycin, cranial
neuropathy ,severe head injury.
* nystagmus common in multiple
Sclerosis , stroke, brain
inflammation or brain tumors and
head injury.
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First mistake • Many cases may be
described by the patient
ASSUME THAT 20/20;6/6 VISUAL as ` blurred vision` despite
ACUITY IS NORMAL 6/6 visual acuity
• Reading difficulty (e.g.
convergence or
accommodative problems)

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 • Many cases may be
First mistake described by the patient as `
blurred vision` despite 6/6
ASSUME THAT 20/20;6/6 VISUAL visual acuity
ACUITY IS NORMAL • Visual processing or
neurocognitive
abnormalities (e.g.
alexia,…..)
Alexia, pure alexia ; word
blindness: is an acquired reading
disorder in which the patient is
unable to read, despite
preservation of other aspects of
language such as spelling and
writing.it is often accompanied
by right hemianopia. Alexia is
due to a left occipitotemporal
lesion (angular gyrus).
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First mistake
ASSUME THAT 20/20;6/6
VISUAL ACUITY IS NORMAL
I’m sorry – who are you again ???
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• Many cases may be described
by the patient as ` blurred
vision` despite 6/6 visual
acuity
• Visual processing or
neurocognitive abnormalities
(e.g. prosopagnosia,..…)
Prosopanosia, facial blindness : a
neurological disorder characterized
by inability to recognize faces. It is
thought to be the result of
abnormalities , damage or
impairment in the right fusiform
gyrus. Could be congenital and
present in children with autism.
Prosopagnosic subjects can also
have cerebral dyschromatopsia
First mistake • Many cases may be
described by the patient as `
ASSUME THAT 20/20;6/6 blurred vision` despite 6/6
VISUAL ACUITY IS NORMAL visual acuity
• Visual processing or
neurocognitive
abnormalities (e.g. cerebral
dyschromatopsia)
Cerebral dyschromatopsia , an
impairment of color perception
whose hallmark is a deficit in
discriminating hues. This has
often been linked to bilateral
damage to the lingual and
posterior fusiform gyri.
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Second mistake • The RAPD can be the only
The pupil exam ; miss to objective sign of optic
check for the Relative neuropathy or occult
Afferent Pupillary Defect retinopathy.
(RAPD) • The PERRLA (Pupils, Equal
,Round Reactive to Light
and Accommodation) does
not include as assessment of
the RAPD.
• The ‘swinging light test’ is
used to detect a relative
afferent pupil defect (RAPD).

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Second mistake • Neutral density filters
The pupil exam ; miss to are used to quantify
check for the Relative the RAPD. Patients
Afferent Pupillary Defect with a subtle or
(RAPD) questionable RAPD can
be ` neutralized` by
placing the smaller
0.3 log
filter in front of the `
0.6 log
good eye` and
0.9 log
performing the
1.5 log swinging flashing light
test.
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Second mistake • A novel portable
The pupil exam ; miss to
check for the Relative
Afferent Pupillary Defect
(RAPD)
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diagnostic device
called Optic Nerve
Glass (O-Glass)
recently under
investigation. It can
perform optic nerve
neuropathy diagnosis ,
chromatic
pupillometry, and
automatic anterior
segment anomaly
detection.
Second mistake • How to test for a relative
afferent pupillary defect
The pupil exam ; miss to
(RAPD)????
check for the Relative The physiology , the steps of
Afferent Pupillary Defect the test, specific situation,
(RAPD) grading and causes of RAPD.
All you can read and
understand it from the simple
article written by: Dr. David C
Broadway, the
Consultant ophthalmic surgeon
from UK.
• This article published in
Community Eye Health Journal,
VolUME 25 ISSUES 79 & 80.
2012.
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Third mistake
Miss to perform a formal
visual field testing in every
unexplained visual loss
• The sensitivity of
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• Visual field testing by
confrontation is a
reasonable screening
test that should be
performed in every
new patient.
confrontation testing
varied from20% to 90%
depending on type of
visual field loss.
Third mistake • Formal visual field should
be performed on every
Miss to perform a formal
patient with unexplained
visual field testing in every
visual loss.
unexplained visual loss
• Should be tested in both
eyes.
• Clinicians should make a
special effort to clinically
correlate the pathologic
finding with the size ,
severity, and location of
the proposed etiology of
visual loss.
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Fourth mistake • Formal visual field testing
of both eyes is important to
Miss to compare the nasal detect subtle abnormalities
and temporal fields for in the fellow eye and to
hemianopic loss allow comparison of the
nasal and temporal
hemifields.
• For example , a lesion at
the junction of the optic
nerve and chiasm can
produce a superotemporal
field defect in the
contralateral(opposite eye )
and asymptomatic fellow
eye.
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 • A monocular hemianopic
Fourth mistake
field loss can also occur at
Miss to compare the nasal the junction (junctional
and temporal fields for scotoma of Traquair).
hemianopic loss.

Harry Moss Traquair (1875-1954)

Was an Edinburgh ophthalmologist,
who was especially involved in the studies
of perimetry and neuro-ophthalmology.
He included the description of the visual
field as “ an island of vision or hill of
vision surrounded by a sea of blindness”
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 • Recognition of these
Fourth mistake
junctional visual field
Miss to compare the nasal defects is important
and temporal fields for because a lesion of the
hemianopic loss. junction is often
compressive in nature
and neuroimaging is
strongly recommended.

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Fourth mistake • Any unexplained
Miss to compare the nasal bitemporal defect to the
and temporal fields for visual field should be
hemianopic loss considered to be
chiasmal in origin until
proven otherwise.
• Any homonymous defect
to the visual loss should
suggest retrochiasmal
involvement.

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Fifth mistake • An `unreliable
Miss to recognize an automated or
`unreliable` visual field Goldmann visual field
does not equal a `normal` provides the same
visual field. information as no
visual field.
• A confrontation visual
field by using a red
test object is important
medically in all
patients with
`unreliable` visual field.
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Sixth mistake • Slit lamp and
Miss to look at the slit
retinoscope
lamp carefully after examination might
dilation demonstrate irregular
astigmatism,
keratoconus, nuclear
or subcapsular
cataract.

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Seventh mistake • The retina look
Assume that a normal ophthalmoscopically
appearing macula equal normal or near normal
a normal functioning and have markedly
macula. poor visual function.
• OCT can be used to
perform an `optical
biopsy` of the retina to
demonstrate a subtle
macular lesion.

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Seventh mistake • Truly ophthalmoscopically
Assume that a normal normal macula can be
appearing macula equal seen in cone dystrophy
a normal functioning ,acute zonal occult outer
macula. retinopathy , resolved
commotio retinae and all
these detected only by
electrophysiology.

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Eighth mistake • Retrobulbar optic
neuropathy is common in
Assume that a normal demyelinating optic neuritis
appearing optic nerve , in this cases the RAPD may
equal a normal be the only objective sign
functioning optic nerve . of an optic neuropathy.
• In these cases there is a
higher likelihood of a
compressive etiology than if
the optic nerve were
atrophic.
• The condition may be truly
described as “ the patient
sees nothing and the
doctor sees nothing”
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Ninth mistake • Optic atrophy is not
Consider optic atrophy is an etiologic
a diagnosis. diagnosis. It is an
ophthalmoscopic
description of a sign
for a possible optic
neuropathy.

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Tenth mistake • Unexplained optic
atrophy should be
Does not evaluate
considered to be a
unexplained optic
compressive optic
atrophy.
neuropathy until proven
otherwise.
• In one study, 20% of cases
of isolated and
unexplained optic atrophy
were due to an
intracranial lesion.

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 Summary for the ten recommendations for
avoiding errors in neuro-ophthalmic patients
1. Don’t assume that 6/6 visual acuity is normal.
2. Check for the RAPD in every patient with
unexplained visual loss.
3. Perform a formal visual field in every patient with
unexplained visual loss and correlate visual field
loss to presumed ocular etiology.
4. Compare the temporal and nasal field carefully.
5. Recognize that an `unreliable` visual field does not
equal a `normal` visual field and perform a careful
confrontation visual field test.
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 Summary for the ten recommendations for
avoiding errors in neuro-ophthalmic patients
6. look at the slit lamp exam after dilation and consider
retinoscopy.
7. Remember that a normal appearing macula does not
equal a normal functioning macula.
8. Be aware that a normal appearing optic nerve does
not equal a normal functioning optic nerve.
9. Recall that optic atrophy is not a diagnosis , it is simply
an ophthalmoscopic description.
10.Evaluate unexplained optic atrophy.
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FINALLY:
Good judgment comes from experience,
and experience comes from bad judgment.

Mulla Nasrudin

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 Thank you
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