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KEY POINTS

106

When examining
patients with
functional
visual loss, it
is crucial for
the physician
to remain
empathetic, lest
the office visit
deteriorate into
an adversarial
event.
It is important
that the
physician
discuss the
examination
findings and
explain their
implications to
the patient with
nonorganic
symptoms. In
addition, the
patient must
always be given
a way out or an
opportunity to
save face in this
very awkward
situation.

FUNCTIONAL
NEURO-OPHTHALMIC
CONDITIONS
Michael S. Vaphiades, Lanning B. Kline

ABSTRACT
The term functional or nonorganic indicates that the patients visual acuity, ocular
motility, or pupillary impairment has no underlying anatomic or physiologic basis.
The diagnosis of a functional syndrome is one of exclusion. This chapter will define
the different types of functional visual disorders and characterize the testing used
to diagnose them.
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INTRODUCTION
The physician-patient relationship is a
unique one. The physicians role is to
acquire the history, examine the patient, diagnose a disease process, and
initiate treatment. The patients role is
even more difficult, because dysfunction is often accompanied by feelings
of fear of the unknown and loss of
control. The physician and the patient
are allies in the achievement of a common goal: identifying the physiologic
or organic cause of the disease process.
When the patients reported symptoms
are suspected not to be based on an
organic process, the physician-patient
dynamic may change from one of trying
to defeat a common enemy (the disease
process) to the physician and patient
now working contrary to one another.
When this occurs, it is crucial for the
physician to remain empathic, lest the
office visit deteriorate into an adversarial event. It is important that the physician discusses the examination findings
and explains their implications to the
patient. In addition, the patient must

always be given a way out or an opportunity to save face in this very awkward situation.
The term functional indicates that
the patients impairment has no underlying anatomic or physiologic basis.
Visual complaints that have no physiologic or organic basis are also referred to as nonorganic. The terms
functional and nonorganic will be
used interchangeably in this text.
Recognition of nonorganic disease
dates back to antiquity. The term
hysterical (from the Greek) literally
means a uterine condition and suggests a process unique to women.
Plato thought the uterus was endowed
with its own will. If it wandered below
the ribs, it produced hypochondria.
In the Middle Ages, if a womans behavior was unusual in any way, she
was felt to be possessed by a demon,
and labeled hysterical (Thompson,
1985). In the late 1800s, Charcot
demonstrated that hysteria was not
unique to women but also occurred in
men. Charcots students, Freud and

Relationship Disclosure: Drs Vaphiades and Kline have nothing to disclose.


Unlabeled Use of Products/Investigational Use Disclosure: Drs Vaphiades and Kline have nothing to disclose.

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Babinski, defined hysteria as a subconscious expression of nonorganic symptoms caused by suggestion and cured
by persuasion. Freud felt that hysteria stemmed from emotional disturbances of sexual origin and that visual
loss was a conversion from emotional
to physical manifestations, which relieved emotional turmoil; hence the
term conversion reaction. Such hysterical patients may exhibit la belle
indifference or lack of concern regarding their symptoms, ie, an emotional
functional mismatch (Thompson, 1985).
The term malingering indicates a
willful feigning of symptoms for personal gain, ie, financial or emotional
improvement. The symptoms of a malingering patient are not psychogenic,
for they are dependent on volition;
true psychogenic disturbances are involuntary. The economic impact stemming from malingering patients is
staggering, costing society millions of
dollars in fraudulent claims (Keltner
et al, 1985). Patients with malingering
are aware that their symptoms do not
exist, while hysterical patients believe
that their symptoms truly exist. An additional form of functional disorder is
that of factitious disease. In this setting,
patients intentionally induce real symptoms. These patients are often said to
have Mu
nchhausen syndrome. Patients
with Mu
nchhausen syndrome harbor
a psychological need to adopt the role
of an ill person and therefore manifest
factitious physical symptoms and signs
to fulfill this need (Miller, 2005). Use of
the terms functional, nonphysiologic,
or nonorganic avoids the negative
aspects of terms such as malingering,
hysteria, or Mu
nchhausen syndrome,
as well as avoids potentially false statements expressed to a third party that
may do harm to a patients reputation.
Functional visual disturbances may
include a variety of symptoms. The
most common functional ophthalmologic symptom is that of visual loss. In

adults, functional visual loss is more


common in women. Many patients
(over 50% in one study) also have a
concomitant psychological disorder,
and care must be taken to separate
the two (Kathol et al, 1983). Examples
include somatization disorder, affective
disorder, anxiety disorder, and various
personality disorders such as histrionic,
antisocial, schizoid, and passive aggressive. Exaggeration of an organic process as a manifestation of a functional
disorder is one of the most difficult
problems faced by the physician, as the
real component may make it impossible to demonstrate that the patients
vision is entirely normal. Similarly, the
real component tends to be ignored
once evidence suggests functional overlay. As will be discussed, dealing with
the underlying organic problem can
often result in marked improvement
of the entire syndrome.
Functional visual loss in children is
estimated to occur at a rate of 1.4 per
1000 per year (Brodsky et al, 1996). It
is more commonly seen in girls between the ages of 9 and 11 (Clarke
et al, 1996). As in adults, functional
visual loss in children is a diagnosis of
exclusion. Up to 25% of children with
nonorganic visual loss have a superimposed organic disorder (eg, maculopathy) (Brodsky et al, 1996). Common
associated symptoms include headaches,
diplopia, and micropsia. Functional ocular motility disorders, including spasm
of the near reflex and voluntary nystagmus, also occur more commonly in
children.
The first requirement for identifying functional visual disorders is a high
index of suspicion. A patients affect
may provide a strong clue to a nonorganic process. Patients may be clearly
depressed, indifferent, anxious, hostile, or excessively cooperative. Historical inconsistencies in the pattern or
the degree of the visual dysfunction
are often the first clue to a nonorganic
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107

" FUNCTIONAL CONDITIONS

process. The nature of the history itself may also provide clues as to motives for secondary gain. The key issue
for the examiner is to assess for inconsistencies between subjective patient concerns and objective findings
on clinical examination. The physician
must be patient, persistent, and facile with the testing techniques used,
and the patient must remain unaware
of the examiners goal: demonstrating better function (visual acuity, visual fields, ocular motility, etc) than
claimed. The concept of misdirection
(ie, the perceived testing of the normal eye when in reality the impaired
eye is being tested) is important, as
the physician often must employ a certain amount of showmanship and
creativity to achieve this goal. Most
importantly, the physician must adopt
an attitude of empathy toward the patient and avoid confrontation, which
will undermine the goal of the office
visit.
Functional visual complaints can
be separated into four categories: (1)
afferent (visual acuity and field), (2)
efferent (ocular motility and alignment), (3) pupil and accommodation,
and (4) eyelid position and function.
Methods to diagnose each will be discussed separately.

108

TABLE 7-1

"

Testing Techniques
for Functional
Visual Loss

Binocular Complete Visual Loss


Mirror
Finger-touching (Figure 7-1)
Optokinetic nystagmus
(Figure 7-2)
Evaluation of ambulation

"

Monocular Complete Visual


Loss
Swinging flashlight (relative
afferent pupillary defect)
Stereoacuity test
Finger-touching (Figure 7-1)
Optokinetic nystagmus
(Figure 7-2)
Mirror technique
Prism shift and prism
dissociation tests (Figure 7-3)

"

Monocular Reduced Visual


Acuity
Stereoacuity (Figure 7-2)
Prism shift and prism
dissociation tests (Figure 7-3)
Polarizing lens test (Figure 7-4)
Duochrome test (Figure 7-5)
Fogging techniques (Figure 7-6)
Bottom-up acuity

TESTING TECHNIQUES
Functional Disease Affecting
the Afferent Visual Pathway
Testing techniques for functional visual loss are outlined in Table 7-1.
Binocular complete visual loss.
Finger touching is a technique in which
the patient is asked to touch the fingertips of each hand together. Patients
who are truly blind, or sighted persons
with closed eyes, can easily touch their
fingertips together because this requires
only proprioception, not vision. Patients
with functional monocular blindness
may touch their fingertips together when

Near-distance discrepancies
Electrophysiology
(visual-evoked potentials,
electroretinogram)

"

Binocular Reduced Visual


Acuity
Stereoacuity test
Prism tests
Polarizing test
Duochrome test

continued on next page

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TABLE 7-1

Continued

Fogging techniques
Bottom-up acuity

"

Some Organic Disorders


Frequently Misdiagnosed
as Functional
Occult maculopathy
Paraneoplastic-associated
retinopathies/optic
neuropathy
Retinitis pigmentosa sine
pigmento
Cone-rod dystrophy
Stargardt disease

mirror must be large enough (approximately 35 cm  67 cm) to prevent the


patient from looking around it. If the
patient states that he or she sees
nothing in the mirror, but the patients eyes move in response to the
moving image as it is rocked back and
forth, a subjectiveobjective mismatch
has been documented (Kramer et al,
1979).
In addition, careful observation of
the way the patient ambulates into and
out of the examination area, avoiding
various obstacles (eg, furniture, other
individuals), and assessment of his/her
affect (fear, indifference, or anger) may
also provide valuable clues as to the

KEY POINTS

Big blind spot syndromes


Bilateral occipital lobe infarcts

Creutzfeldt-Jakob disease

viewing with the normal eye, but not


with this eye occluded (Figure 7-1)
(Miller, 2005). Optokinetic nystagmus
testing involves an optokinetic nystagmus drum or tape moved to induce
horizontal jerk nystagmus. Optimum
responses occur when the rate of succession of images is 3 to 12 per second. In a patient reporting unilateral
blindness, the drum is rotated with
both eyes open. Once nystagmus is
elicited, the unaffected eye is quickly
covered and the blind eye is observed
for continued nystagmus (Figure 7-2).
Although difficult, it is possible to suppress optokinetic nystagmus especially
if objects on the drum or tape are sufficiently large and the patient can avoid
fixating on them (by looking over or
beyond the targets). Children, however,
are generally unaware of these strategies.
The mirror test is based on the
movement of the eyes in response to
the image on a mirror as it is rocked
back and forth. Patients cannot easily
suppress these eye movements. The

Truly blind
persons can
easily touch
their fingertips
together,
because this
requires only
proprioception,
not vision.
Patients with
functional
monocular
blindness may
touch their
fingertips
together when
viewing with
the normal
eye, but not
with this eye
occluded.
With symptoms
of bilateral
blindness, the
absence of a
relative afferent
pupillary defect
does not imply
nonorganic
visual loss, as
the patient may
have bilateral
symmetric
anterior visual
pathway
pathology or
bilateral
posterior visual
pathway
disease.

Finger-touching technique.
The patient is asked to
touch the tips of the index
finger of each hand together. A, A blind
person can easily perform this task. B, A
patient with nonorganic visual loss who
claims inability to touch her fingers together.

FIGURE 7-1

Reprinted with permission from Parrish RK II,


editor. The University of Miami Bascom Palmer Eye
Institute atlas of ophthalmology. 2nd ed.
Philadelphia: Butterworth-Heinemann, 2000.
Copyright # 2000, Elsevier.

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109

" FUNCTIONAL CONDITIONS

Optokinetic nystagmus testing. A, In a


patient with nonorganic visual loss the
drum is first rotated with both eyes open
and once optokinetic nystagmus is elicited, the unaffected eye
is quickly covered (B) and the blind eye is observed for
continued nystagmus.

FIGURE 7-2

Reprinted with permission from Parrish RK II, editor. The University of


Miami Bascom Palmer Eye Institute atlas of ophthalmology. 2nd ed.
Philadelphia: Butterworth-Heinemann, 2000. Copyright # 2000, Elsevier.

110

nonorganic basis of the visual symptoms. The surprise or shock technique


entails showing objectionable photographs to the patient with the examiner
observing for an emotional response,
such as smiling or gasping. This technique is generally too confrontational
to recommend.
With symptoms of bilateral blindness, the absence of a relative afferent
pupillary defect (RAPD) does not imply
nonorganic visual loss, as the patient
may have bilateral symmetric anterior
visual pathway pathology or bilateral
posterior visual pathway disease.

Monocular complete visual loss.


The swinging flashlight test provides
valuable objective evidence of monocular prechiasmal (retina or optic nerve)
damage. An RAPD may be present if a
patient has a monocular optic neuropathy or an extensive retinal disorder.
The absence of an RAPD in a patient
reporting monocular blindness does
not confirm a nonorganic disorder but
greatly increases suspicion of one. Patients with true monocular blindness
and bilateral optic nerve dysfunction
may not have an RAPD. Also, the patient may state, My right eye is blind,
and have no RAPD, when in reality
the patient is erroneously lateralizing a
right homonymous hemianopia to the
right eye because of the fact that the
field loss of the right temporal hemifield is larger than that of the left nasal
hemifield.
Measurement of stereoacuity is very
useful in detecting monocular functional visual loss because stereopsis requires excellent binocular vision. The
use of polarizing lenses is necessary
for the test (Levy and Glick, 1974).
Two clinical techniques include the
Titmus and the random-dot tests. The
Titmus test is a three-dimensional Polaroid vectograph in a book format. Polaroid glasses give the three-dimensional
effect to the images in the book. The
random-dot test consists of several
plates, each containing various shapes
constructed with colored dots. Some
of the images are only apparent when
red-green glasses are worn. Most recently, the Mentor Binocular Visual
Acuity Test, a computer-based test to
evaluate stereoacuity, has been used.
The finger-touching technique, optokinetic nystagmus, and the mirror test
are also useful.
Monocular reduced visual acuity.
Using the following testing techniques,
the examiner may be able to improve
upon the patients stated acuity and
yet fail to establish 20/20 acuity in the

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affected eye. Although this objective


subjective mismatch confirms a functional component to the visual loss, the
examiner is still not off the hook or a
concomitant organic component to the
visual loss may be present. Stereoacuity
as mentioned above is also useful.
By convention, the prism shift test
(as demonstrated by Case 7-1) uses a
4-diopter (D) base-out prism and relies on normal refixation movements

to avoid diplopia. This technique is


helpful if the patient claims severely
diminished acuity (less than 20/400)
but less valuable in patients with only
minimally reduced acuity (Figure 7-3).
The prism dissociation test uses a
5-D or 6-D base-down prism (some
patients have fusional amplitudes up
to 4-D) placed in front of the normal
eye and a 0.5-D prism placed in any
direction over the impaired eye (so

Case 7-1
A 17-year-old adolescent presented with visual loss in the left eye (OS)
noted 2 days earlier. She had developed a headache after the visual loss,
and her vision had not yet improved. She indicated that she was blind in
her left eye. She had no significant past medical history or allergies. She
did not smoke cigarettes or consume alcohol.
On neuro-ophthalmic examination the patients blood pressure was
119/62 mm Hg, and her heart rate was 68 beats/min. Visual acuity without
correction was 20/20 in the right eye (OD) and no light perception OS.
With prism dissociation using an 8-D base-down prism over the good eye
(right eye), the patient read 20/20 in both eyes. Color vision was 9.5/10
Ishihara plates OD, 0/10 Ishihara plates OS. Confrontational visual fields
were full OD, and automated perimetry was full OD. Pupils measured
6 mm in each eye (OU), with normal reactivity OU and no RAPD. Saccadic
and pursuit movements were normal in the vertical and horizontal
plane. With an 8-D base-in prism, a shift occurred with alternate cover
testing at distance. Ductions were full. Eyelids measured 8 mm OU.
Exophthalmometry revealed Hertel readings of 18 mm OU, with a base
of 101 (normal). Trigeminal and facial nerves were intact. Intraocular
pressures were 14 mm Hg OU (normal is 10 mm Hg to 21 mm Hg). The
eyelids, conjunctiva, cornea, and anterior chamber appeared normal OU.
The optic nerves had cup-disc ratios of 0.2 OU (normal). The retinal vessels
and periphery appeared normal OU.
Comment. This patient has monocular visual dysfunction that is functional
or nonorganic in nature. The prism dissociation test is valuable when the
patient feigns visual loss in only one eye. With prism dissociation, the patient
saw 20/20 OU, indicating the patient can see normally with the blind
left eye. It is important that the patient can indeed see the 20/20 line in the
blind eye. Anything less than 20/20 may indicate better acuity than what
was claimed but not necessarily normal vision in the impaired eye. The
pupillary examination did not reveal an RAPD; this would be unusual if
the patient did indeed have an optic neuropathy or pan-retinopathy in the
blind left eye. With an 8-D base-in prism, a shift occurred with alternate
cover testing at distance, indicating the patient can indeed fixate with the
blind left eye. The patient and family should be counseled, indicating
that the patients visual pathways are anatomically intact and that stress
can produce the patients symptoms. The family should be reassured that
the patients vision should improve.

KEY POINT

A variation of the
prism dissociation
test uses an 8-D
base-down prism
placed in front of
the normal eye and
no prism in front of
the impaired eye. A
vertical refixational
movement of the
eyes is observed
when the patient
shifts fixation from
the lower to the
higher image. To
prove normal
acuity, the patient
must read both
(doubled) 20/20
lines. A report of
diplopia also
provides evidence

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111

" FUNCTIONAL CONDITIONS

112

Prism shift test. A, A base-out prism in front of the


normal eye produces a shift of both eyes away from
the prism base, followed by a fusional refixation
movement of the fellow eye (B). C, When the prism is placed over
the normal eye of a patient whose other eye is blind or poorly sighted,
only the first binocular shift occurs, without a compensatory refixation
movement of the opposite eye. D, A truly blind eye does not refixate,
and a prism placed before this eye should result in no movement of
either eye.
f = fovea; OS = left eye.

FIGURE 7-3

Reprinted with permission from Parrish RK II, editor. The University of Miami
Bascom Palmer Eye Institute atlas of ophthalmology. 2nd ed. Philadelphia:
Butterworth-Heinemann, 2000. Copyright # 2000, Elsevier.

the patient will not become suspicious).


A 20/20 Snellen letter is projected and
the patient is asked if he or she can see
two vertically placed letters. If the patient can read both letters, the acuity
in the impaired eye is 20/20.
A variation of this test uses an 8-D
base-down prism placed in front of the
normal eye and no prism in front of
the impaired eye. The patient is asked
if he or she can see the top and bottom lines and then asked to read each
line. A vertical refixational movement
of the eyes is observed when the patient shifts fixation from the lower to
the higher image. The higher image
represents visual acuity in the eye with
the prism (normal eye), and the lower
image represents visual acuity in the
impaired eye. To prove normal acuity,
however, the patient must read both
(doubled) 20/20 lines. A report of diplopia also provides evidence of binocular visual function (Slavin, 1990).
The polarizing lens test uses polarized glasses when viewing a ProjectO-Chart, which projects letters seen
alternately (Figure 7-4); one letter is
seen by both eyes, the next by the
right eye, the next by the left eye, and
so on. A variation of this test includes a
polarizer placed on an occluder without the patient wearing polarizing lenses.
In this way, the examiner controls which
eye is examined, and it becomes more
difficult for patients to alternately close
one eye to determine what should be
seen. The goal of the polarizing lens test
is to have the patient believe he or she
is reading with either the right or left
eye only.
During the duochrome test, patients
think they are reading binocularly, but
they are actually reading with only one
eye. A red and green slide is superimposed on the normal Snellen chart.
With this technique, the eye behind the
red lens sees letters on both sides of
the chart; the eye behind the green
lens sees only letters on the green side

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of the chart. The lenses are arranged so


that the red lens is placed over the eye
with the purportedly decreased vision.
The patient is then asked to read the
chart with both eyes open. If the patient reads the entire line, the impaired
eye must be functioning better than the
patient claims (Figure 7-5) (Ing et al,
1995).
A variety of fogging techniques
may be used for functional visual loss.
The paired cylinder test uses trial
frames with a +6-D and 6-D cylinder
lenses placed at parallel (aligned) axes
before the normal eye in the trial frame.
The patients refraction is placed before
the impaired eye; if the patient does
not require correction, equal power
plus and minus spheres can be used.
After the patient begins reading, a small
turn of the cylinder lenses makes the
axes no longer parallel and blurs the
normal eye so the patient is now reading with the impaired eye. The cylinders can be quickly moved so that they
are again aligned before the patient can
recognize what has occurred. On the
sphere side, lens rotation will make no
difference in image quality (Figure 7-6).
Another method of fogging is to place
a high-power (+6 D) spherical lens
before the normal eye in a trial frame
and the patients normal refraction
before the impaired eye. The patient is
asked to read large-print material, which
is gradually moved farther away. If the
patient is able to continue reading, the
impaired eye must be functioning because a high-power spherical lens will
not allow reading at distances greater
than 25 cm with the normal eye. A
strong plus lens can also be used in the
phoropter for fogging the normal eye.
The bottom-up acuity method begins with acuity testing of the impaired
eye on the smallest line on the Snellen
chart (eg, 20/10). If the patient cannot see these letters, the examiner announces the use of a larger line and
then uses the 20/15 line and several dif-

American Optical (Buffalo, NY) polarizing


lens test. A, The appearance of the entire
chart viewed without a polarizing lens.
B, The appearance of the same chart when viewed through
one of the polarizing lenses. C, The appearance of the chart
when viewed through the other polarizing lens. Note certain
letters (V, C, K, N, S, R) can be seen through both lenses;
others can be seen through one lens but not the other.

FIGURE 7-4

Reprinted with permission from Parrish RK II, editor. The University of Miami
Bascom Palmer Eye Institute atlas of ophthalmology. 2nd ed. Philadelphia:
Butterworth-Heinemann, 2000. Copyright # 2000, Elsevier.

ferent 20/20 lines. The examiner continually expresses surprise that such large
letters cannot be identified. If the patient still denies being able to read the
letters, he or she is asked to determine
the number of characters present and
whether they are round, square, etc.
Once the count is established, the examiner might suggest that the characters are letters and the first one is easier
to identify than the others. This may take
time, and the examiner often has to wait
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113

" FUNCTIONAL CONDITIONS

KEY POINT

114

A normal
full-field and
multifocal
electroretinogram
and a normal
pattern visualevoked potential
in a patient who
presents with
severe (but
good enough
to fixate)
monocular or
binocular visual
loss and an
otherwise
normal clinical
examination
supports the
suspicion of a
nonorganic
disturbance of
vision. This is
assuming that
neuroimaging
(contrasted
cranial and
orbital MRI)
has been
performed.

Duochrome test. A, Appearance of chart with superimposed red-green


duochrome slide when viewed without the red-green lenses. With this
technique, the eye behind the red lens sees letters on both sides of the chart
(B), and the eye behind the green lens sees only letters on the green side of the chart (C).
The lenses are arranged so that the red lens is placed over the eye with the decreased vision,
and the patient is then asked to read the chart with both eyes open. If the patient reads the
entire line, the abnormal eye must be functioning better than the patient claims.

FIGURE 7-5

Reprinted with permission from Parrish RK II, editor. The University of Miami Bascom Palmer Eye Institute atlas of
ophthalmology. 2nd ed. Philadelphia: Butterworth-Heinemann, 2000. Copyright # 2000, Elsevier.

for the patients response. By the time


the very large letters (eg, 20/50) are
reached, the patient often can be cajoled
into reading optotypes much smaller
than those read on initial acuity testing.
The examiner may also have the patient
wear trial frames with multiple lenses
equaling the correct prescription and
suggest that these are special magnifying
lenses that might permit improved
vision.
Distance and near discrepancies
compare visual acuity performance at
distance and near. In patients with
functional visual loss, the two measurements often are not consistent. One
could also move the patient closer to
the Snellen chart and note if improved
acuity is present for the shorter distance. It is important to correct for any
refractive error, including presbyopia,
and make certain no media disturbances occur.
If a definite distance and near
discrepancy exists and no refractive
error occurs, this may be evidence for
functional visual loss.
Electrophysiologic testing may be
utilized to help diagnose nonorganic

visual loss. Pattern-reversal visual-evoked


potentials are widely available as a
noninvasive measure of visual function.
The test is usually performed monocularly and waveforms generated at an
amplitude and latency appearing at
approximately 100 ms (P100) after
visual stimulus. The patient must have
visual acuity sufficient to fixate on the
center target of the computer screen
for the test to be reliable. An abnormal
visual-evoked potential does not necessarily guarantee organic visual loss.
Patients may alter the waveforms volitionally by using a variety of maneuvers, such as meditation, daydreaming,
convergence, defocusing, or other
tricks. It is a useful test if completely
normal (Morgan et al, 1985).
A full-field electroretinogram (ERG) is
a useful test to eliminate occult retinal
dystrophies (retinal dystrophy without
findings on funduscopic examination).
Testing reliability does not depend on
visual fixation so the patient may have
very poor vision and still yield a reliable test. Multifocal ERG is a relatively
new technique that represents a significant advance over conventional full-field

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electroretinography in the evaluation


of macular disease (Sutter and Tran,
1992). Conventional ERG measures the
summed electrical activity of the entire
retina and thus is relatively insensitive to
localized retinal defect. For example, it is
possible to have a completely normal
conventional ERG in an eye with vision
of hand motion or worse. This occurs
because the fovea, which accounts for
acuity, contributes less than 5% of
the total electrical response elicited by
conventional ERG. Multifocal ERG, in
contrast, measures summed responses
from small defined segments of the
posterior pole centered around the
fovea, typically 103 separate responses
from each area. Thus, localized retinal
defects are readily detected by this test,
and multifocal ERG responses correlate
well with visual acuity in outer retinal
disease (Hood, 2000).
A normal full-field and multifocal ERG
and a normal-pattern visual-evoked potential in a patient who presents with
severe (but good enough to fixate) monocular or binocular visual loss and
an otherwise normal clinical examination support the suspicion of a nonorganic disturbance of vision. This is
assuming that neuroimaging (cranial
and orbital MRI with contrast) has been
performed.
Binocular reduced visual acuity.
Testing methods that are helpful include bottom-up acuity, distance and
near discrepancies, the use of special
charts, and the doctor killing refraction. Also, stereoacuity testing, prism
shift and prism dissociation tests, polarizing lenses and duochrome tests,
and fogging techniques may be useful.
Monocular diplopia. Monocular
diplopia does not remit when covering
one eye and is most commonly produced on the basis of high astigmatism
either due to corneal or lens change.
Monocular diplopia may also result
from a corneal scar, cataract, vitreous
opacity, or macular disease. Very rarely

Fogging technique: Paired cylinders. A, Trial


frames with +6-D and 6-D cylinder lenses
are placed at parallel axes before the normal
eye in the trial frame. After the patient begins reading, a
small turn of the cylinder axes makes the axes no longer
parallel (B) and blurs the normal eye so the patient is now
reading with the impaired eye.

FIGURE 7-6

Reprinted with permission from Parrish RK II, editor. The University of Miami
Bascom Palmer Eye Institute atlas of ophthalmology. 2nd ed. Philadelphia:
Butterworth-Heinemann, 2000. Copyright # 2000, Elsevier.

it results from a neuro-ophthalmic cause


(eg, tumor or hemorrhage of the occipital cortex or lesions of the frontal
eye fields). When diplopia has a cortical
origin, it is present in both eyes (bilateral monocular diplopia) and the diplopia is identical in each eye. Cerebral
polyopia is the duplication of the visual image in space with monocular
viewing. Two images or dozens may
be present. All images are seen with
equal clarity; there is no resolution
with a pinhole as occurs with refractive
causes, and the image is unchanged
if viewed monocularly or binocularly.
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115

" FUNCTIONAL CONDITIONS

It is an extremely rare syndrome and


typically associated with a homonymous visual field defect. In general, if

116

Many monocular and binocular visual field


abnormalities may be detected with Goldmann
perimetry in a patient with nonorganic visual loss.
Examples of visual field abnormalities include spiraling isopters (A) and
crossing isopters (B). C, Nonorganic monocular hemianopia with a
temporal defect on binocular field testing. D, E, A patient with true
monocular full-field blindness should demonstrate the absence of the
temporal crescent on binocular visual field testing versus a nonorganic
normal binocular field.

FIGURE 7-7

Reprinted with permission from Parrish RK II, editor. The University of Miami Bascom
Palmer Eye Institute atlas of ophthalmology. 2nd ed. Philadelphia: ButterworthHeinemann, 2000. Copyright # Elsevier, 2000.

bilateral monocular diplopia does not


remit with pinhole, cranial neuroimaging is required to evaluate for hemispheric lesions. Once all is ruled out,
the clinician should suspect a functional
cause for the monocular diplopia.
Visual field abnormalities. The
most common functional visual field
abnormality is generalized constriction.
The tangent screen is useful in these
cases. The patient is initially tested with
a stimulus at 1 m. When the patient is
tested at 2 m, the stimulus size is also
doubled so the size of the test object in
relation to the distance of the patient
from the screen remains constant. In a
normal patient, the visual field should
expand (funnel vision) and not stay the
same or get smaller (tunnel vision), as
often occurs in patients with functional
visual loss (Kline, 2000).
If kinetic Goldmann perimetry is
used, patients with functional visual
loss often give inconsistent responses,
with spiraling (Figure 7-7A) or overlapping of isopters (Figure 7-7B). Spiraling of isopters indicates that the
patient is responding to a constant
stimulus but at variable positions, usually responding closer to fixation each
time the stimulus is presented. Overlapping isopters are not physiologic,
because the patients initial response to
stimuli occurs closer to fixation than
that of a smaller or less bright stimulus
(opposite what it should be). In a
functional monocular temporal defect,
the binocular visual field (tested with
both eyes open) may also have a temporal defect that respects the vertical
meridian (Figure 7-7C). The patient
with a true monocular hemianopia demonstrates only loss of the temporal
crescent on binocular visual field testing because of overlap of the intact nasal field from the opposite eye (Keane,
1979).
A patient with true monocular blindness should demonstrate the absence
of a temporal crescent on the side of

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the visual loss with binocular field testing, while a patient with functional
hemianopia may demonstrate a normal binocular field (Figures 7-7D
and 7-7E).
Automated perimetry often confuses,
rather than clarifies, the clinical picture
in patients with functional visual field
loss. Although abnormalities in the reliability indices in these patients may
occur, testing does not always adequately reflect fixation, false-positive,
or false-negative errors. For example,
in patients with feigned visual field depression (eg, no positive responses),
the false-negative rate may not be
high because the patient responds negatively to all stimuli, producing no falsenegative responses. Clinicians must be
aware of this potential pitfall and employ manual methods of visual field
testing to corroborate abnormalities
detected with automated techniques
(Stewart, 1995).
Patients with functional field loss
may demonstrate deficits that respect
the horizontal and/or vertical meridians,
thus mimicking a disorder of the anterior or posterior visual pathways. On
occasion, it may be difficult to differentiate nonorganic from organic visual
field loss, and patients will require further evaluation.
Functional Disorders Affecting
the Efferent Visual Pathway
Voluntary nystagmus is characterized by
irregular brief bursts of rapid frequency,
low-amplitude eye movements. Most
commonly they are horizontal, although
on occasion they may be vertical or torsional. The eye movements are bilateral
and conjugate and are often associated
with convergence, fluttering eyelids,
blinking, or strained facial expression.
Voluntary nystagmus is difficult to maintain for longer than 10 to 12 seconds. It
is actually back-to-back saccades without
an intersaccadic interval. Patients often
report oscillopsia and reduced vision.

These individuals are identified by the


volitional appearance of the ocular movement disorder, absence of nystagmus
when they are distracted, inability to sustain the fast eye movements, and the lack
of other neuro-ophthalmic abnormalities. Voluntary nystagmus can be diagnosed using eye movement recordings.
Patients with functional ocular motility disorders may report an inability to move their eyes horizontally or
vertically. Such gaze palsies may be
overcome by a variety of maneuvers,
including oculocephalic testing (dolls
head maneuver), optokinetic testing,
mirror tracking, and caloric testing.
Spasm of the near reflex, a syndrome characterized by episodes of
intermittent convergence, increased
accommodation, and miosis, is usually
observed in patients with functional
visual loss but has been associated
with Chiari I malformation, posterior
fossa tumors, pituitary tumors, and
head trauma (Dagi et al, 1987). Patients generally report diplopia and, at
times, micropsia. Since the degree of
convergence is variable, some patients
demonstrate marked convergence of
both eyes, resulting in a large esotropia. Others show a lesser degree of
convergence in which one eye remains
relatively straight while the other converges. Typically, the spasm cannot
be maintained and the esotropia resolves with monocular testing. Spasm
of the near reflex may be mistaken for
unilateral or bilateral abducens nerve
palsies, divergence insufficiency, horizontal gaze paresis, or ocular myasthenia. In addition, patients may report
blurred distance vision from up to 8 D
to 10 D of induced myopia. Refraction
without and with cycloplegia during
the period of spasm (dynamic retinoscopy) establishes the presence of the
induced myopia. The variability of
convergent eye movements, lack of
other neuro-ophthalmic abnormalities,
resolution with monocular testing, and

KEY POINT

If kinetic Goldmann
perimetry is used,
patients with
functional visual
field defects
often give
inconsistent
responses, with
spiraling or
overlapping of
isopters. Spiraling
of isopters
indicates that
the patient is
responding to a
constant stimulus
but at variable
positions, usually
responding closer
to fixation each
time the stimulus
is presented.

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117

" FUNCTIONAL CONDITIONS

KEY POINTS

118

The pilocarpine
test readily
distinguishes
parasympathetic
denervation
from
pharmacologic
blockade. In
the latter, 1%
pilocarpine
cannot
overcome
the receptor
blockage, and
the pupil
remains large.
It is prudent to
allow patients a
way out by
reassuring
them that
although their
disorder does
not suggest
underlying
damage to the
CNS, they do,
in fact, have
a problem that
is expected
to resolve
over time.

occurrence of miosis with associated


esotropia permit the correct diagnosis.
Functional Disease Producing
Pupillary Abnormalities
Few patients provoke more anxiety for
the physician than those with headache and a dilated fixed pupil. The
differential diagnosis can be narrowed
to four basic processes: (1) pharmacologic blockade, (2) trauma (with or
without pupillary sphincter tears) and
inflammation, (3) oculomotor nerve
palsy, and (4) Adie tonic pupil. Evidence of trauma and inflammation may
be sought at the slit lamp looking for
sphincter damage and pigmentation.
Pharmacologic blockade may occur
because of inadvertent or purposeful
application of mydriatic eye drops or
from the use of a scopolamine patch
to prevent motion sickness or postoperative nausea. The pilocarpine test
readily distinguishes parasympathetic
denervation from pharmacologic blockade. In the latter, 1% pilocarpine cannot overcome the receptor blockage,
and the pupil remains large. A fixed
dilated pupil from injury to the third
cranial nerve will constrict in response
to 1% pilocarpine. Adie tonic pupil will
constrict to 0.1% pilocarpine since denervation supersensitivity will be present. Widely dilated pupils may be seen
in young patients, likely due to increased levels of circulating catecholamines. Rarely, patients are able to
voluntarily dilate both pupils.
Intermittent miosis will occur due to
spasm of the near reflex, accompanied
by changes in eye position (esotropia)
and accommodation. Miosis may also
be produced pharmacologically with
pilocarpine.
Functional Disease Affecting
Eyelid Position and Function
Voluntary blepharospasm may be unilateral or bilateral. At times, it may cause

nonorganic ptosis. Most cases of nonorganic blepharospasm occur in children


or young people and may be triggered
by a particularly emotionally traumatic
event. The blepharospasm may respond
to psychotherapy, hypnosis, behavior
therapy, and biofeedback or may spontaneously resolve on its own.
PATIENT MANAGEMENT
In general, patients with symptoms of
functional visual disorders are best
managed with an understanding approach and words of encouragement.
It is prudent to allow patients a way
out by reassuring them that although
their disorder does not suggest underlying damage to the CNS, they do,
in fact, have a problem that is expected
to resolve over time. Often with one or
two follow-up visits the symptoms will
clear, and they can be reassured of an
excellent prognosis.
In patients with both organic and
functional symptoms, it is best to address the former problem and attempt
to downplay the latter. With appropriate management of the organic visual
disturbance, the patients anxiety may
be alleviated and the nonorganic symptoms resolve.
Despite a thorough examination and
discussion, more than half of patients
with functional visual loss continue to
manifest symptoms on follow-up examinations. In addition, functional patients
with associated psychiatric disorders
are less likely to recover. If a functional disorder is proven, patients are
told that there is no anatomic cause
for their visual dysfunction and that
sometimes stress can be a contributor
to their problem. It is suggested that
it may be beneficial to have a psychiatric evaluation to help better manage
the stress. The notes should indicate
that there is a nonorganic component,
and the words malingering or hysterical should never be used. Also, never

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label a patient as having functional


overlay unless normal visual function
can be proven.
Finally, it is always prudent to follow a patient with what appears to be
only a functional visual disturbance
because organic and functional diseases often coexist (Scott and Egan,
2003). The diagnosis of a functional

syndrome is one of exclusion and, ideally, of retrospection.


ACKNOWLEDGMENTS
This work was supported in part by
an unrestricted grant from the Research to Prevent Blindness, Inc., New
York, NY.

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