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Functional Neuro Ophthalmic Conditions.9
Functional Neuro Ophthalmic Conditions.9
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.
Continuum Lifelong Learning Neurol 2009;15(4):106120.
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
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
107
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
"
"
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)
"
TABLE 7-1
Continued
Fogging techniques
Bottom-up acuity
"
KEY POINTS
Creutzfeldt-Jakob disease
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
109
FIGURE 7-2
110
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
111
112
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.
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
Continuum Lifelong Learning Neurol 2009;15(4)
113
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.
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.
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.
115
116
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.
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.
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.
117
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.
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