Optic Nerve Disorders - part 10

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254 D.C. Hood and K. Holopigian

records. First, compare the mfERG responses Figure 11.8 illustrates an example in which
to the visual field. In this case, her field depres- the effects of a fixation error are subtler. These
sion extended at least to 25° (Figure 11.7F) and mfERGs are from a young woman with a very
clearly did not agree with the location of the small central defect in her left visual field. Her
depression of the mfERG (circle in Figure acuity was good, and her fixation appeared
11.7B). Based on this evidence alone, the hypo- steady. It was initially thought that her problem
thetical retinal deficit in this patient should be was retinal because a few of the paracentral
considered suspicious. Second, the 3D plot in responses (see responses in rectangle) appeared
Figure 11.7D can be examined. Notice that both reduced in amplitude. However, an examina-
the foveal peak and the optic disc depression tion of the 3D plot indicated that she was fixat-
are displaced compared to the 3D plot from the ing slightly off center; this is easy to see when
control subject with normal fixation (see Figure the 3D plot is compared to the plot from her
11.1A, bottom). The patient appears to be fixat- unaffected right eye.
ing eccentrically, and all the apparent abnor- In sum, if care is not taken in the recording
malities seen in the trace array in Figure 11.7B and interpretation of mfERGs, then depressed
are based on poor fixation. The left column of responses caused by fixation errors can be mis-
Figure 11.7 illustrates the point. Here an indi- interpreted as a retinal problem.
vidual with normal vision was asked to fixate
down and to the left 8.5° from the center. Notice
Ruling Out Functional or
how the pattern of the patient’s mfERG resem-
Nonorganic Causes
bles that of the results from the control in Figure
11.7A and 11.7C, except that the patient was When diagnosing optic nerve disorders, it is
fixating up and to the left of the target. often important to rule out functional or non-



mfERG responses. (B) 3D plots for the mfERGs in
Figure 11.8. The problem of eccentric fixation.
A. The 3D plot for the left eye indicates that the
(A) mfERG from the two eyes of a patient. The left
patient was fixating slightly off center, which could
eye had a small central defect on the visual field and
account for the reduced mfERG amplitudes in that
the right eye had a normal visual field result. The
black circle indicates an area of apparently decreased
11. The Use of Multifocal ERGs and VEPs in Diagnosing Optic Nerve Disorders 255

organic causes. The advantage of the mfERG The Multifocal Visual Evoked
technique over the conventional ERG is that
it provides a topographical representation
that can be compared to the patient’s visual
The VEP has long been used to diagnosis disor-
fields. If the mfERG is abnormal in the same
ders of the optic nerve. For example, delayed
location as the field defect, then a nonorganic
VEP responses in patients with optic neuritis/
cause can be ruled out. If, on the other hand,
multiple sclerosis (ON/MS) were reported
the mfERG is normal, then further tests
almost 25 years ago.31,32 While the conventional
(e.g., the mfVEP) are needed to rule out a
VEP, elicited by either a pattern-reversal stimu-
nonorganic cause.
lus or bright flash, is still used to help in the
diagnosis of ON/MS or to rule out nonorganic
(functional) causes, the conventional VEP has
Special Techniques for Detecting its limitations. First, conventional VEPs are
Ganglion Cell Damage with dominated by responses from the lower field in
the mfERG most individuals.33–35 Therefore, in some cases,
large defects in the upper field will be missed
The effectiveness of the human mfERG for
with the conventional VEP. Second, the conven-
detecting local ganglion cell damage is currently
tional pattern reversal VEP is recorded to a
under debate. Although some contradictory
display at least 15° in diameter.36 Thus, local
findings can be found in the literature, the evi-
defects can easily be missed. In general, the lack
dence is relatively clear on the following points.
of spatial information can be a problem for the
First, there is a component generated at the
conventional VEP.
optic nerve head that appears to reflect local
The multifocal visual evoked potential
ganglion cell activity. Sutter and Bearse23 first
(mfVEP), developed by Baseler, Sutter, and
identified this component in the human mfERG
colleagues,37,38 allows the recording of local
and called it the optic nerve head component
VEP responses from the visual field by combin-
(ONHC). Second, a component similar to the
ing conventional VEP recording techniques
ONHC has been identified in the monkey
with multifocal technology. As in the case of the
mfERG, and it appears to depend upon gang-
mfERG, each region of the display is an inde-
lion cell activity.24 Thus far, attempts to detect
pendent stimulus and from a single, continuous
glaucomatous damage with standard mfERG
EEG signal, the software extracts the VEP
recordings show relatively poor sensitivity and/
responses generated to each of the independent
or specificity.8,25–27 However, the relatively small
regions. Typically, local VEP responses are gen-
ONHC in humans can be enhanced with speci-
erated simultaneously from 60 regions of the
alized paradigms of mfERG stimulation28,29
central 20° to 25° (radius) of the visual field to
and/or methods of analysis.23 Finally, although
create a topographic profile of the visual field.
clear evidence of local damage has been
reported in a few patients, in general the results
Recording the mfVEP
published to date have been disappointing.29,30
Thus, it remains unclear whether specialized For recording the mfVEP, the same electrodes
mfERG recordings can be used to detect early and amplifiers employed for conventional VEP
damage in patients with glaucoma. If the results recordings are used. However, the parameters
of future studies are more encouraging, then of the stimulus and display and the analysis of
the mfERG technique still needs to be compa- the raw records are different. Although new
paradigms are being developed,39 most of the
red to other objective tests of ganglion cell fun-
ction, such as the pattern ERG (PERG), the published mfVEP data have been recorded
photopic negative response (PhNR), and the with pattern reversal stimulation and a display
multifocal VEP. For now, the mfERG cannot be similar to the one shown in Figure 11.9.
considered a useful clinical tool for studying This display, first introduced by Baseler,
Sutter, and colleagues,37,38 contains 60 sectors
ganglion cell damage.
256 D.C. Hood and K. Holopigian

There is currently no agreement regarding stan-
dard placement for the electrodes. However, all
mfVEP recordings include at least one midline
electrode placement. For example, for our
midline channel we use two electrodes. One is
placed at the inion plus 4 cm and serves as the
“active,” and the other, on the inion, serves as
the “reference”; a third electrode, the ground,
is placed on the forehead. It is not uncommon
to record from more than one channel at a
time.40–42 For example, we use three “active”
electrodes, one placed 4 cm above the inion and
two placed 1 cm above and 4 cm lateral to the
inion on each side of the midline.40,42 Every
Figure 11.9. The multifocal VEP stimulus. This
active electrode is referenced to the inion.
display contains 60 sectors approximately scaled to
account for cortical magnification. Each sector con-
Presentation and Analysis of the
tains 16 checks, 8 black and 8 white.
mfVEP Responses
Figure 11.10 shows software-derived mean
approximately scaled to account for cortical mfVEP responses from 30 control subjects. The
magnification. Each sector contains 16 checks, black traces are the responses for monocular
8 black and 8 white. stimulation of the right eye and the gray traces
The mfVEP is recorded monocularly with are the responses from the left eye. As in the
electrodes placed over the occipital region. case of the mfERG, each of the individual

OD: black
OS: gray

200 nV

100 ms

Figure 11.10. The software-derived mean mfVEP right eye (OD) and the gray traces are the responses
from the left eye (OS). (Reprinted from Hood,10 with
responses from 30 control subjects. The black traces
are the responses for monocular stimulation of the permission from Elsevier.)
11. The Use of Multifocal ERGs and VEPs in Diagnosing Optic Nerve Disorders 257

mfVEP waveforms in the array is not, techni- is changing rapidly, and the analyses shown
cally speaking, a “response.” Rather, each here, based upon our software, soon should be
waveform is derived via a correlation between generally available in commercial software.To
the stimulation and the continuously recorded illustrate these analyses, consider the patient
signal. It is important to note that when the whose visual field (probability plot) is shown
mfVEPs are displayed in an array, as in Figure in Figure 11.11A. This patient had unilateral
11.10, the responses are positioned arbitrarily glaucomatous damage in the left eye; the visual
so they do not overlap. The spatial scale for field from his right eye was normal. The defects
this array is not linear, which can be seen in the left eye are circled in gray and black. The
in a comparison of the iso-degree circles in mfVEP responses obtained from the patient’s
Figure 11.10 to the display in Figure 11.9. For left eye (red) and right eye (blue) are shown
more details about the mfVEP technique, see in Figure 11.11B. Iso-degree contours repre-
recent reviews.42,43 senting the same areas of visual space are
shown for both the visual field and the
mfVEP responses.
Nearly Identical mfVEP Responses To determine which of the responses from
the left eye (red records in Figure 11.11B) are
from the Two Eyes
abnormal, mfVEP probability plots analogous
There is considerable intersubject variability to the visual field probability plot in Figure
in the amplitudes and the waveforms of the 11.11A were developed. Monocular mfVEP
mfVEP responses. This variability is caused probability plots (left two panels in Figure
by individual differences in the location and 11.11C) were obtained by comparing the
folding of the visual cortex.21,42 However, the patient’s monocular mfVEPs to the averaged
responses of the two eyes from any individual mfVEPs from the left and right eyes of a group
with normal vision are nearly identical, as can of control subjects (see Figure 11.10). For each
be seen in the mean responses of Figure 11.10. sector, the amplitude of the patient’s mfVEP
These mean responses from the two eyes are was determined and compared to the results
nearly identical. The reason for this is that they from a control group.40,42,44,45 Each square is
are generated in the same general cortical plotted at the physical center of one of the
regions. The responses from the two eyes do sectors of the mfVEP display (see Figure
deviate in relatively minor ways. First, there is 11.9A). A colored square indicates that the
a small amplitude asymmetry along the hori- mfVEP was statistically significantly different
zontal meridian. Second, there is a small inter- from the control data at either the 5% (desatu-
ocular latency difference (of 4 or 5 ms) across rated color) or 1% (saturated color) level, and
the midline. These small differences can be the color indicates whether it was the left (red)
seen in the insets in Figure 11.10. The responses or right (blue) eye that was significantly smaller
from the left eye are smaller, but are slightly than normal.
faster, than the responses from the right eye Because the visual field (Figure 11.11A) and
in the left visual field, and the reverse is mfVEP (Figure 11.11C) probability plots are
true in the right visual field. (See Hood and shown on the same linear scale, a direct compa-
Greenstein42 for a discussion of the reasons for rison can be made. To aid in this comparison,
these differences.) the black and gray ellipses from Figure 11.11A
were overlaid onto Figure 11.11C. Notice that
the mfVEP results confirm the visual field
Topographical Representation
defect within the black ellipse but not the defect
of the mfVEP within the gray ellipse.
To detect local damage to the ganglion cells/ In some patients, especially those with unila-
optic nerve requires specialized software, and teral damage, an interocular comparison of the
the current analyses available with commerical mfVEP results is a more sensitive indicator of
equipment are limited. However, this situation damage than is the monocular comparison to
258 D.C. Hood and K. Holopigian


OD/OS ratio

OD/OS ratio
>4.5 S.D.

C Monocular Interocular

Figure 11.11. Results from a patient with glaucoma. (C) Monocular and interocular mfVEP probability
(A) The 24–2 HVF (probability plot) for the patient’s plots. Each symbol is in the center of a sector of the
left eye with the defects circled in gray and black. mfVEP display. A black square indicates that there
(B) The mfVEP responses from the patient’s left eye is no significant difference between the two eyes. The
(red) and right eye (blue). The inset shows the results colored squares indicate that there is a significant
of comparing the RMS ratios of two pairs of difference at greater than the 5% (desaturated) or
responses to those from a group of control subjects. 1% (saturated) level. The color denotes whether the
N.S., the ratio of amplitudes is not significantly dif- right (blue) or left (red) eye had the smaller response.
ferent from normal. Iso-degree contours represen- A gray square indicates that the responses from
ting the same areas of visual space are shown for both eyes were too small to allow for a comparison.
(Modified from Fig. 12 in Hood et al.11)
both the visual field and the mfVEP responses.

the control group.42,46 To obtain the interocular within the gray ellipse is still not apparent, but
an arcuate defect is detected in the lower field
mfVEP plot in Figure 11.11C (right-hand
that was not present in the visual field.
panel), the ratio of the mfVEP amplitudes of
Subsequent tests confirmed that this defect was
the two eyes is measured for each sector of the
real. (Hood and Greenstein42 provide a review
display and compared to the ratios from the
group of controls.21,40,42,47,48 The result is coded of the derivation and use of both monocular
and interocular probability plots.)
as in the case of the monocular fields. The defect
11. The Use of Multifocal ERGs and VEPs in Diagnosing Optic Nerve Disorders 259

right eye) as well as one that was 34.2 ms slower
Measuring Latency as
on the monocular comparison (i.e., relative to
Well as Amplitude
the control group).
It is now possible to objectively measure the
latency of individual mfVEP waves.49,50 Figure
The Origins of the mfVEP
11.12A shows the visual field probability plot
There are two lines of evidence that the mfVEP
from the left eye of a patient; her right eye had
is generated largely in V1. First, as originally
a normal visual field. Figure 11.12B shows the
pointed out by Baseler et al.,37 the mfVEP
mfVEPs from the right and left eyes. Figure
waveforms reverse polarity as one crosses the
11.13A shows the amplitude probability plots
horizontal meridian (see the reversal of the
of her mfVEPs are normal on the monocular
waveforms in Figure 11.10).42,51 The mfVEP
plots but that the interocular plot shows a rela-
from the upper visual field is reversed in polar-
tive loss in amplitude for the left eye. Figure
ity as compared to the lower, whereas the con-
11.13B shows the results of the latency analysis
ventional VEP recorded with the same
plotted in an analagous fashion to the ampli-
electrodes positions and on the same subjects
tude plots. In particular, a colored circle indica-
may show the same polarity for upper and
tes that the mfVEP latency was significantly
lower field stimulation.35 Only potentials gener-
longer at either the 5% (desaturated color) or
ated from inside the calcarine fissure should
1% (saturated color) level, whereas the color
behave this way. Second, a mathematical analy-
indicates whether it was the left (red) or
sis of the multifocal VEP sources suggests that
right (blue) eye that was significantly longer
most of the signal is generated in V1.52 Third,
than normal. In this example, the latency of the
using an application of principal-component
left eye was, on average, 7.8 ms slower than
analysis, Zhang and Hood53 provided evidence
the right, as compared to the normal control
that the first principal component of the mfVEP
subjects. An individual point is shown that was
was generated within the calcarine fissure and
15 ms slower on the interocular comparison
thus within V1. The clinical implication is that
(i.e., her left eye was delayed relative to her


Figure 11.12. Results from a patient with vision loss was normal. (B) The mfVEPs from the right (blue)
in the left eye. (A) The visual field probability plot and left (red) eyes of the patient.
from the affected left eye of a patient; the right eye
260 D.C. Hood and K. Holopigian

A Amplitude Probability Plots

Interocular Plot
Monocular Plots

B Latency Probability Plots

15 ms

34.2 ms

Figure 11.13. Monocular and interocular probabi- cantly smaller than normal. (B) Latency results. A
lity plots derived from the VEP results shown in Fig. colored circle indicates that the mfVEP latency was
11.12. (A) Amplitude results. A colored square indi- significantly longer at either the 5% (desaturated
cates that the mfVEP amplitude was significantly color) or 1% (saturated color) level; the color indi-
smaller at either the 5% (desaturated color) or 1% cates whether it was the left (red) or right (blue) eye
(saturated color) level; the color indicates whether it that was significantly longer than normal.
was the left (red) or right (blue) eye that was signifi-

However, before summarizing the uses of the
damage beyond V1 does not necessarily produce
mfVEP, it is important to understand the effects
abnormal mfVEPs.
of local ganglion cell/optic nerve damage on the
mfVEP. Hood et al.46 showed that the signal in
The mfVEP and the Diagnosis of
the mfVEP response was linearly related to the
Optic Nerve Disorders loss in visual field sensitivity. To take a simple
example, this means that a loss of 10 dB in visual
For a number of years we have recorded
field sensitivity will reduce the amplitude of the
mfVEPs from the patients of two neuro-
signal in the mfVEP response by a factor of 10;
ophthalmologists (Drs. M. Behrens and J. Odel)
this will result in an mfVEP response indistin-
and two glaucoma experts (Drs. R. Ritch and
guishable from noise. Therefore, relatively small
J. Liebmann). In this section, we summarize the
visual field sensitivity losses (6 dB or so) caused
most common uses of the mfVEP in diagnosing
by optic nerve damage produce profound losses
optic nerve disorders. Other examples can be
found in recent reviews.42,43 in mfVEP amplitude.
11. The Use of Multifocal ERGs and VEPs in Diagnosing Optic Nerve Disorders 261

this region there are areas with delayed mfVEP
The Diagnosis and Follow-Up of Optic
responses (asterisks) and regions with reasona-
Neuritis/Multiple Sclerosis
bly normal mfVEP responses (plus signs). In
During the acute phase of ON/MS, mfVEP fact, regions with delays can border regions that
amplitudes are depressed in all regions where have responses with normal amplitude and
the visual field sensitivity is decreased.54 Typi- latency. Thus, the mfVEP is able to detect local
cally, optic neuritis shows partial or complete
recovery within 3 months and so does the Therefore, for diagnosing patients with ON/
mfVEP. In fact, those patients with normal MS, the mfVEP is superior to SAP and the
visual fields after recovery have normal or near- conventional VEP. We have seen a number of
normal mfVEP amplitudes, although the latency cases of ON/MS in which the mfVEP was
in some regions will be markedly delayed.54,55 abnormal but the conventional VEP was
These regions with the delayed mfVEP presu- normal. In these patients, whether the conven-
mably correspond to the portions of the optic tional VEP is normal depends upon the relative
nerve that were demyelinated. The mfVEP contributions of the normal and abnormal
records in Figure 11.14B show the range of regions of the visual field. The conventional
findings that can be observed in a patient who VEP is most likely to miss local delays if the
had an attack of optic neuritis in the left eye.54,55 delays involve very small areas or occur in the
In this case, the visual field probability plot upper field, which typically contributes less to
(Figure 11.14A) shows a paracentral defect and the overall VEP signal than does the lower
field.35 Figure 11.15 shows the SAP probability
the amplitude of the mfVEP is depressed in this
region (ellipse in Figure 11.14B). However, the plot (panel A) and mfVEP responses (panel B)
mfVEP (Figure 11.14B) shows that outside of of a 45-year-old man who complained of blurred

24-2 HVF (OS)

black: OD
gray: OS

Figure 11.14. Results from a patient with optic neu- on the visual field (ellipse). However, outside this
ritis in the left eye. (A) The visual field probability region there are areas with delayed mfVEP respon-
plot from the left eye shows shows a paracentral ses (asterisks) as well as regions with reasonably
defect. (B) The mfVEPs from the left eye show normal mfVEP responses (plus signs). (Reprinted
from Hood,10 with permission from Elsevier.)
depressed amplitudes in the area that was affected
262 D.C. Hood and K. Holopigian



OD: black,
OS: gray

Figure 11.15. Results from a patient with blurred (gray) and right (black) eyes. The insets show the
vision in the superior field of the left eye. (A) The mfVEPs summed within each quadrant, indicating
visual fields for the left and right eyes were essen- delayed mfVEPs in the upper field for stimulation of
the left eye. (Modified from Fig. 14 in Hood et al.11)
tially normal. (B) mfVEP response arrays for the left

vision in the superior field of his left eye. The visual fields, a small percentage of the patients
diagnosis of MS was confirmed from magnetic with ON can present with swollen discs but
resonance imaging (MRI) studies, which showed without pain. In these cases, it is important
lesions in the left optic nerve. His conventional to distinguish between ON, ischemic optic
pattern VEP, as well as his SAP fields (panel A), neuropathy (ION), or a compressive lesion.
were normal. The insets in panel B show the We have found the mfVEP useful in these
mfVEPs summed within each quadrant. The
mfVEPs are clearly delayed in the upper field Finally, the mfVEP is particularly useful
for the left eye. This change was missed on for following patients with ON/MS, especially
the conventional VEP, presumably because the in cases in which the visual field is normal.
upper field contributed relatively little to the We have recently documented recovery of
conventional VEP. local mfVEP latencies in some patients
Although the diagnosis of ON can usually whose visual field thresholds are normal
and stable.56
be made based upon the patient’s history and
11. The Use of Multifocal ERGs and VEPs in Diagnosing Optic Nerve Disorders 263

whose complaint of a localized visual loss was
Ruling Out Functional or
thought to be nonorganic in nature. His fields
Nonorganic Causes
were unreliable, and he was under emotional
The conventional VEP has been used to rule stress at home and work. However, his mfVEP
out functional or nonorganic causes for visual confirmed a local deficit in the same general
defects. Because multiple, local responses are region as his complaint. The local change in the
obtained, the mfVEP is more effective than the mfVEP can be seen in the records of panel A
conventional VEP for this purpose. For example, and the interocular probability plot of panel B.
a local defect can be identified on the mfVEP The mfVEPs and the corresponding SAP points
and can be missed on the conventional VEP if illustrate the local loss. Subsequent tests revea-
the defect involves a small part of the total field led a diagnosis of Leber’s optic atrophy. In pati-
stimulated. In these cases, the (incorrect) dia- ents such as this one with localized deficits, the
gnosis of a functional cause can be avoided. conventional VEP is often normal.
Figure 11.16 provides an example of a patient Conversely, when faced with normal mfVEP
responses in regions of the field where the
visual field shows a profound defect,57 the oph-
thalmologist will be comfortable making a dia-
gnosis of a nonorganic cause. In fact, the mfVEP,
with its topographical measures, provides more
information and a greater degree of certainty
than does the conventional VEP.
Finally, it is also possible to assess the patient
with “functional overlay.” That is, it is not
uncommon to have a patient with clear indica-
tions of organic disease, but whose visual fields
are too bad to be explained by what appears to
be the organic cause. A careful quantitative
comparison of the mfVEP amplitudes can help
to parcel out the nonorganic contributions from
the organic ones.

Questionable Fields or Fields That
Need Confirmation
B A related category of patients are those
whose visual fields are questionable to the
ophthalmologist even though the reliability
indices are within the normal ranges. That is, the
visual fields do not appear to reflect the other
clinical findings. For example, some patients
produce visual fields on SAP that are repro-
ducible and of good quality (e.g., false positives,
false negatives, and fixation errors are low), but
which are nonetheless not a veridical indicator
of what the patient actually sees. In such cases,
the ophthalmologist often has insufficient or
contradictory evidence, making it difficult to
Figure 11.16. Results from a patient with a localized
diagnose the cause of a defect seen on the SAP.
vision loss. (A) The mfVEP plots for the left (red)
Figure 11.17 shows an example of a 74-year-old
and right (blue) eyes. (B) The mfVEP interocular
woman with abnormal visual fields. These fields
probability plot reveals local losses (red circle).
264 D.C. Hood and K. Holopigian

–15 –9 –25 –10 –27 –23 –25 –24
–28 –24 –24 –15 –20 –25
–9 –9 –11 –10 –17 –4
–9 –3 –6 –3 –5 –3 –4 –10 –28 –30–12 –15 –11–12 –20 –26
–7 –4 0 –2 –3 –2 –8 –14 –27 –25 –12–11 –10 –4 –5 –25
–7 –2 –1 –4 –2 –13 –13 –26 –21 –6 –6 –4 –5 –7 –26
–12 –7 –5 –4 –4 –5 –17 –20
–16 –13 –6 –4 –4 –3 –5 –3
–23–18 –24 –13 –21 –28
–7 –13 –8 –7 –10 –21
–29–25 –30–26
–8 –12 –11 –23

Figure 11.17. Results from a patient with abnormal ation plots for this patient reveal large losses in sen-
visual fields. (A) mfVEP plots for the left (red) and sitivity that do not agree with the mfVEP findings
right (blue) eyes. (B) The Humphrey 24–2 total devi- shown in A.

would not be classified as unreliable based upon work is beyond the scope of this chapter. For-
standard statistics. Notice in Figure 11.17B (24– tunately, reviews on the use of the mfVEP in
2 Humphrey total deviation plots) that both detecting and following glaucoma are availa-
ble.42,58 Our own view is that the mfVEP can be
eyes had regions of sensitivity loss that exceeded
15 dB. Her ophthalmologist questioned the very useful to the glaucoma expert. It can be
fields because her cup-to-disc ratios [0.6 (OD) used to test unreliable field takers and patients
and 0.5 (OS)] were relatively good whereas her with questionable fields or fields that need
fields were very poor. The mfVEPs were confirmation. However, we do not believe
obtained, and they were inconsistent with her that in its current form it will replace SAP.
visual fields. The mfVEP responses from both Although there are conditions under which the
mfVEP can detect damage missed on SAP,42,48,59,60
eyes (Figure 11.17A) were quite robust, which
did not agree with the large visual field sensitiv- there are conditions under which the reverse
is true.42,60
ity losses. Remember that optic nerve damage
produces profound decreases in the mfVEP
(see foregoing discussion; also Hood et al.46). The Problem of Fixation Errors
Other examples of the use of the mfVEP to
confirm qustionable fields can be found in pub- Unsteady fixation can cause diminished
lished reviews.42 responses in the center of the field.42,60 Inaccu-
rate or unsteady fixation will affect the mfVEP
results.42,60 Monitoring the eye will assure that
Unreliable Visual Field Test Takers
fixation is steady, but it will not guarantee that
Many patients cannot or will not reliably the fixation is accurate. Some patients with
perform SAP. For most of these patients, the central visual problems can have eccentric fixa-
mfVEP provides an alternative. tion. Figure 11.18 shows the effects of a 3° fixa-
tion error. A control subject was instructed to
maintain a steady fixation that was down and
Detecting Glaucomatous Damage
to the left by 3° for the right eye while the left
Most of the work with the mfVEP has focused eye was tested with central fixation. Compared
on glaucoma. A detailed description of this to the control condition (Figure 11.18A,B), the
11. The Use of Multifocal ERGs and VEPs in Diagnosing Optic Nerve Disorders 265

fixation OD down & left by 3°
fixation in center OU



subject instructed to fixate down and to the left by
Figure 11.18. The consequences of eccentric fixa-
3° when testing OD and fixating in the center when
tion. Eccentric fixation can give the appearance of an
testing OS. (D) The 60 mfVEP responses corre-
abnormality in an otherwise normal eye. (A) Interoc-
sponding to the probability plot in C. Responses in
ular mfVEP probability plot for a control subject
the inset show clear polarity reversals and amplitude
fixating at the center of the stimulus when testing
differences between the two eyes. (Reprinted from
both eyes. (B) The 60 mfVEP responses correspond-
Hood et al.,43 with permission from Lippincott
ing to the probability plot in A. Responses in the
Williams & Wilkins.)
inset are of the same polarity and appear normal.
(C) Interocular mfVEP probability plot for the same

eccentric fixation condition (Figure 11.18C,D) field. Confirmation that these symmetrical
showed apparent defects in both eyes on the defects are caused by an eccentric fixation can
interocular probability plot. It is relatively easy be obtained by examining the responses from
to tell that these “defects” are caused by eccen- near the midline. Notice that some of these
tric fixation. The probability plot shows a tell- responses (see inset in Figure 11.18D) show a
tale sign. In particular, there are smaller polarity reversal between the two eyes. Thus, it
responses in diagonally opposite parts of the is important to monitor eye position to avoid
266 D.C. Hood and K. Holopigian

false positives from unsteady fixation. In addi- 2. The mfERG and mfVEP are not useful
tion, the mfVEP plot and responses (see Figure for problems in the far periphery. In general,
11.18) should be examined to avoid false posi- these tests assess performance on the central
tives resulting from eccentric fixation. 20° to 30° from fixation (see Figures 11.1A
and 11.9).61
3. These tests do not assess rod system func-
Poor mfVEP Test Takers tion. These techniques test the cone system: the
cone receptors and cone bipolars are assessed
Just as there are patients who are unreliable
when recording the mfERG, and the cone path-
SAP takers, there are also patients who have
ways up to V1 are assessed when recording the
great difficulty being tested on the mfVEP. In a
mfVEP. This is another reason for using the
few cases, these can be the same individuals.
ISCEV standard full-field ERG, which tests rod
Patients who refuse to cooperate or who go to
and cone system function, if panretinal damage
sleep may be difficult to test on either SAP or
is expected.61
the mfVEP. In our experience, however, the
4. These tests are not appropriate if the
overwhelming majority of the patients who are
patient cannot maintain fixation or has nystag-
poor SAP takers are able to perform the mfVEP
mus. Under these conditions, the mfERG and
test. On the other hand, there are a small per-
mfVEP can be a challenge to interpret, whereas
centage of patients who are good SAP takers
the standard ERG and VEP are more immune
but who do not produce usable mfVEP record-
to eye movements and fixation problems.61
ings. In these cases, the responses are difficult
5. If you are going to perform a multifocal
to discern because of a high noise level second-
test, always attempt to obtain a reliable visual
ary to either a large alpha-wave contribution or
field using SAP. We repeat that the power of the
poor signal-to-noise ratios in general.
multifocal technique is that it provides topo-
graphical information. This advantage is poorly
When Is the Multifocal used without a comparison of the deficits seen
on the multifocal test with those seen on
Electroretinogram and/or
Multifocal Visual Evoked
To conclude, when faced with localized
Potential Test Needed? damage of the visual fields in patients with
steady fixation, the mfERG and mfVEP are
The mfERG and mfVEP are not necessarily
powerful tools for diagnosing and studying dis-
the best electrophysiological tests for every
orders of the optic nerve.
patient. In deciding whether an mfERG or
mfVEP is the appropriate test, the following
points should be kept in mind:
1. If there is no advantage to performing a 1. Holopigian K, Hood DC. Electrophysiology.
multifocal test over a full-field test, then the Ophthalmol Clin N Am 2003;16(2):237–51.
2. Sutter EE, Tran D. The field topography of ERG
standard full-field ERG or conventional wide-
components in man. I. The photopic luminance
field VEP should be performed first. In general,
response. Vision Res 1992;32(3):433–46.
the multifocal tests take more time to adminis-
3. Hood DC. Assessing retinal function with the
ter, require more technical expertise to perform
multifocal technique. Prog Retin Eye Res
and analyze, and are less readily available than
the conventional ERG or VEPs.61 For example, 4. Keating D, Parks S, Malloch C, Evans A. A com-
if the problem is panretinal (a large area of the parison of CRT and digital stimulus delivery
visual field is abnormal), and the ophthalmolo- methods in the multifocal ERG. Doc Ophthal-
gist wants to determine if there is retinal mol 2001;102(2):95–114.
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the test of choice. Seeliger MW, Miyake Y. Guidelines for basic
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DC, Carr RE. Local cone and rod system func-
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27. Palmowski AM, Allgayer R, Heinemann-
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28. Shimada Y, Li Y, Bearse MA Jr, Sutter EE, Fung
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W. Assessment of early retinal changes in diabe-
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29. Fortune B, Bearse MA Jr, Cioffi GA, Johnson
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30. Palmowski AM, Allgayer R, Heinemann-Verna-
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vertical and horizontal meridians of the visual
48. Goldberg I, Graham SL, Klistorner AI. Multi-
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34. Michael WF, Halliday AM. Differences between
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A Angiotensin-converting enzyme Anticoagulants
ACE. See Angiotensin-converting (ACE), levels of, in for cerebral venous sinus
enzyme sarcoidosis, 116 thrombosis, 68–69
Acetazolamide, for IIH, 75, 79 Anterior communicating artery for NAION, 35
Acetylsalicylic acid (ASA), for aneurysm, 101 Anti-MBP. See Anti-myelin basic
GCA, 48 Anterior ischemic optic protein
Activated protein C (APC), neuropathy (AION), Anti-myelin basic protein (anti-
resistance to, NAION and, 42–49 MBP), optic neuritis and,
40 GCA in 7
Acute sphenoid sinusitis, optic diagnosis of, 44–46 Antinuclear antibody (ANA), in
neuritis and, 5 incidence of, 42 neuromyelitis optica,
Addison’s disease, increased ICP pathophysiology of, 42–44 15–16
and, 72 symptoms and signs of, 42 Antioxidants, for FA, 187–188
Adrenoleukodystrophy, 190–191 treatment of, 46–48 Antiphospholipid antibody
Adults, optic neuritis in, 1 visual prognosis of, 48 syndrome, optic neuritis
Aicardi syndrome, with congenital OCT for, 240 and, 6
disc pigmentation, 215 optic neuritis v., 3 Anti-phospholipid protein (anti-
AIDS other etiologies of, 49 PLP), optic neuritis and, 7
cryptococcosis and, 118–119 Anterior visual pathway gliomas Anti-PLP. See Anti-phospholipid
tuberculosis and, 117 benign protein
AION. See Anterior ischemic course and prognosis of, APC. See Activated protein C
optic neuropathy 107–108 Apoptosis, with traumatic optic
Alcohol use histopathology of, 106–107 neuropathy, 137
cavernous hemangioma and, incidence of, 103–104 Aqueductal stenosis, in
225 management of, 108–109 papilledema, 66
optic neuropathy with, 154 neuroimaging of, 106 Arcuate defects
Amblyopia, tobacco-alcohol, NF-I association with, 104 in NAION, 31
optic neuropathy with, 154 symptoms and signs of, in suprasellar meningioma, 98
Amiodarone, in optic 104–106 ASA. See Acetylsalicylic acid
neuropathy, 157–159 malignant Ascites, with benign anterior
Amphotericin B, intravenous, for epidemiology of, 109 visual pathway gliomas,
cryptococcosis, 118–119 neuroimaging of, 110 109
ANA. See Antinuclear antibody pathology of, 110 Aspirin, for NAION, 35–36
Anemia prognosis and treatment of, Astigmatism, optic nerve
IIH and, 72–73 111 hypoplasia and, 201
increased ICP and, 72 symptoms and signs of, Astrocytic hamartoma
NAION with, 38, 40 109–110 diagnostic testing for, 222
PION with, 50–51 Anthrax, optic neuritis with, 4 histopathology of, 222

272 Index

Cerebral autosomal dominant
Bromocriptine, for pituitary
Astrocytic hamartoma (cont.)
arteriopathy with
adenoma, 96
overview of, 221–222
subcortical infarcts and
Brucellosis, optic neuritis with, 4
symptoms and signs of,
treatment of, 223
Cabergoline, for pituitary
Atherosclerosis, NAION with, 35
Cerebral venous sinus thrombosis
adenoma, 96
ATP deficiency
clinical presentation of, 68
CADASIL. See Cerebral
in LHON, 152
IIH v., 72
autosomal dominant
in optic neuropathy, 153
in papilledema, 67–69
arteriopathy with
Autoimmune disease, optic
CHAMPS. See Controlled High-
subcortical infarcts and
neuritis and, 5–6
Risk Subjects Avonex
Autosomal dominant progressive
Multiple Sclerosis
Calpain, optic neuritis with, 7
optic atrophy with
Prevention Study
Canavan’s syndrome, 192
congenital deafness, 183
Cantholysis, lateral, for traumatic
Autosomal dominant progressive
for benign anterior visual
optic neuropathy, 139
optic atrophy with
pathway gliomas, 109
Canthotomy, lateral, for
progressive deafness and
for meningeal metastasis, 112
traumatic optic
ataxia, 184
for ONSM, 91
neuropathy, 139
Autosomal recessive optic
for optic disc tumor, 112–113
Capillary hemangioma
atrophy, 181
for paraneoplastic optic
cavernous hemangioma v., 225
neuropathy syndromes, 21
histopathology of, 224
for GCA, 47
for suprasellar meningioma, 99
symptoms and signs of,
for NMO, 16–17
for sarcoidosis, 116
IIH in, 79–80
treatment of, 224
neurodegenerative disorders
Carbidopa, for NAION, 36
of, 189–192
Carboplatin, for benign anterior
Bacterial infection, optic neuritis
optic neuritis in, 1
visual pathway gliomas,
and, 5
MS risk factors with, 11–12
Bariatric surgery
Carotid artery stenosis, NAION
for IIH, 78–79
AION and, 44
with, 34
for papilledema, 72
NAION and, 38
Carotid-ophthalmic artery
BDGF. See Brain-derived growth
Chloramphenicol, in optic
aneurysm, 102
neuropathy, 159
Caspase inhibitors, for traumatic
Behçet’s disease, AION with, 49
Choroidal folds, with elevated
optic neuropathy, 140
Behr’s syndrome, 181–182
ICP, 64
Cataract surgery, traumatic optic
BENEFIT. See Betaseron in
Choroidal melanoma
neuropathies after, 142–143
Newly Emerging MS for
diagnosis of, 229
Cat scratch disease, optic neuritis
Initial Treatment
symptoms of, 229
with, 4
Betaseron in Newly Emerging
treatment of, 229
Cavernous hemangioma
MS for Initial Treatment
Chronic inflammatory
diagnostic testing for, 225
(BENEFIT), interferon
histopathology of, 225
beta-1a in, 14
polyneuropathy (CIDP),
symptoms and signs of,
Blepharoplasty, traumatic optic
papilledema and, 67
neuropathies after, 144
Churg-Strauss angiitis, AION
treatment for, 225
Blurry vision, with ocular
with, 49
CDMS. See Clinical definite
lymphoma, 113
CIDP. See Chronic inflammatory
multiple sclerosis
Bone marrow transplantation,
Central retinal artery occlusion
for MPS, 190
(CRAO), with optic disc
Bradykinin, with traumatic optic
Clinical definite multiple
drusen, 212
neuropathy, 137
sclerosis (CDMS)
Central retinal vein occlusion
Brain-derived growth factor
interferon beta-1a and, 13–14
(CRVO), with optic disc
(BDGF), for traumatic
optic neuritis and, 1
drusen, 212
optic neuropathy, 141
risk of, 8–9
Central scotomas, in NAION, 31
Brimonidine, for NAION, 36
Index 273

optic disc dysplasia, 209
Computed tomography (CT)
Clomiphene citrate, in optic
papillorenal syndrome, 209
of benign anterior visual
neuropathy, 161
Congenital disc pigmentation,
pathway gliomas, 106
Coagulopathies, NAION with,
of cerebral venous sinus
Congenitally anomalous disc
thrombosis, 68
Cochrane Database Systematic
of craniopharyngioma, 100
Review, for cerebral
congenital tilted disc
of fibrous dysplasia, 103
venous sinus thrombosis,
syndrome, 204–206
of Grave’s ophthalmopathy, 92
homonymous hemioptic
of idiopathic orbital
Coenzyme Q
hypoplasia, 203
for FA, 187–188
megalopapilla, 203
pseudotumor, 94
for LHON, 177
optic nerve hypoplasia,
of IIH, 70, 74
Color Doppler imaging
of malignant anterior visual
for GCA, 45
segmental optic nerve
pathway gliomas, 110
for traumatic optic neuropathy,
hypoplasia, 203
of ocular lymphoma, 114
Congenital tilted disc syndrome
of ONSM, 89
Color vision, in optic neuritis, 2
clinical features of, 205
of optic disc tumor, 112
residual deficits with, 11
overview of, 204–205
of papilledema, 65–66
Combined hamartoma of retina
symptoms of, 205–206
of pituitary apoplexy, 97
and retinal pigment
Continuous positive airway
of sphenoid wing meningioma,
pressure (CPAP), for IIH,
diagnosis of, 230
of traumatic optic neuropathy,
histopathology of, 230
Contrast sensitivity, in optic
treatment of, 230
neuritis, 2
Congenital disc anomalies,
Complete blood cell count, for
residual deficits with, 11
nutritional optic
Controlled High-Risk Subjects
congenitally anomalous disc
neuropathies, 151
Avonex Multiple Sclerosis
size, 201–206
Compressive optic neuropathies
Prevention Study
congenital tilted disc
of anterior visual pathway,
(CHAMPS), interferon
syndrome, 204–206
beta-1a in, 13–14
homonymous hemioptic
optic nerve sheath
hypoplasia, 203
meningiomas, 88–91
for GCA, 46–47
megalopapilla, 203
of optic nerve, 92–95
for Grave’s ophthalmopathy,
optic nerve hypoplasia,
Grave’s ophthalmopathy,
for IIH, 76, 79
segmental optic nerve
idiopathic orbital
for NAION, 35
hypoplasia, 203
for ocular lymphoma, 114
elevated optic disc anomalies,
pseudotumor, 94–95
for optic neuritis, 12–13
sellar and suprasellar
for pituitary apoplexy, 97
congenital disc
compressive lesions, 95–103
for sarcoidosis, 116
pigmentation, 214–215
anterior communicating
for traumatic optic neuropathy,
hyaloid system remnants,
artery aneurysm, 101
carotid-ophthalmic artery
CPAP. See Continuous positive
myelinated nerve fibers,
aneurysm, 102
airway pressure
craniopharyngioma, 99–101
optic disc drusen, 210–213
fibrous dysplasia, 102–103
epidemiology of, 99–100
excavated optic disc anomalies,
internal carotid aneurysm,
management of, 101
neuroimaging of, 100
morning glory disc anomaly,
pituitary adenoma, 95–97
pathology of, 100–101
pituitary apoplexy, 97
symptoms and signs of, 100
optic disc coloboma,
sphenoid sinus mucocele, 102
CRAO. See Central retinal artery
sphenoid wing meningioma,
optic disc pit, 208–209
C-reactive protein (CRP), in
peripapillary staphyloma,
suprasellar meningioma,
GCA, 44–46
274 Index

Elevated optic disc anomalies
Crohn’s disease Wolfram syndrome with,
congenital disc pigmentation,
AION with, 49 182–183
increased ICP and, 74 Diabetes insipidus, diabetes
hyaloid system remnants,
CRP. See C-reactive protein mellitus, optic atrophy,
CRVO. See Central retinal vein and sensorineural hearing
myelinated nerve fibers, 214
occlusion loss (DIDMOAD). See
optic disc drusen, 210–213
Cryotherapy, for capillary Wolfram syndrome
Elevated sedimentation rate
hemangioma, 224 Diabetic papillopathy, as
(ESR), in GCA, 44–46
Cryptococcosis NAION, 37
ENA. See Extractable nuclear
diagnostic testing for, 118 DIDMOAD. See Diabetes
epidemiology of, 118 insipidus, diabetes
Encephalitogenic antibodies,
management of, 118–119 mellitus, optic atrophy,
optic neuritis and, 6
pathology of, 118 and sensorineural hearing
Endoscopic sinus surgery,
symptoms and signs of, 118 loss
traumatic optic
CT. See Computed tomography Digoxin, for optic neuropathy,
neuropathies after, 144
Cuban epidemic, of optic 157–159
EOG. See Electro-oculogram
neuropathy, 153 Diphenylhydantoin, for NAION,
ERG. See Electroretinogram
Cup, absent, in NAION, 32 35
ESR. See Elevated sedimentation
Cup-to-disc ratio Diplopia, with Grave’s
in DOA, 178 ophthalmopathy, 93–94
in NAION, 31–34, 37 Direct argon laser
for optic neuropathy, 159
in SIAION, 38 photocoagulation, for
for tuberculosis, 117–118
Cyanide, optic neuropathy from, capillary hemangioma, 224
Ethylene glycol, in optic
154–155 Disulfiram, in optic neuropathy,
neuropathy, 156
Cyanocobalamin 159
Excavated optic disc anomalies
for optic neuropathy, 153 Dizocilpine, for traumatic optic
morning glory disc anomaly,
for vitamin B12 deficiency, 153 neuropathy, 140
DOA. See Dominant optic
optic disc coloboma, 207–208
for GCA, 47
optic disc pit, 208–209
Dominant optic atrophy (DOA)
for idiopathic orbital
peripapillary staphyloma, 208
diagnostic testing for, 179
Extractable nuclear antigen
histopathology of, 179
pseudotumor, 94–95
(ENA), in neuromyelitis
incidence of, 177
for sarcoidosis, 116
optica, 15–16
molecular genetics and genetic
Cyclosporin A, for GCA, 47
heterogeneity of, 179–180
NTG v., 180–181
for idiopathic orbital
FA. See Friedreich’s ataxia
pathophysiology of, 179
Farnsworth-Munsell 100-hue test,
symptoms and signs of,
pseudotumor, 94–95
for optic neuritis, 2, 11
for sarcoidosis, 116
Fatal X-linked optic atrophy,
treatment of, 180
Cytomegalovirus, infiltration of
ataxia, and deafness, 185
Doxycycline, for increased ICP,
optic nerve, 119
Fibrous dysplasia
epidemiology of, 102
Drugs, in toxic optic
management of, 103
neuropathies, 151
Danazol, for increased ICP, 74
neuroimaging of, 103
Dandy criteria, for IIH, 70, 74
pathology of, 103
Dapsone, for GCA, 47
symptoms and signs of, 102
Electro-oculogram (EOG), for
Fluconazole, oral, for
congenital tilted disc
cell-mediated damage in, 6–7
cryptococcosis, 118–119
syndrome, 205
optic neuritis and, 6–7
Flucytosine, oral, for
Electroretinogram (ERG). See
Dexamethasone, for tuberculosis,
cryptococcosis, 118–119
also Multifocal
Fluorescein angiography
for astrocytic hamartoma, 222
for congenital tilted disc
diabetic papillopathy with, 37
for cavernous hemangioma, 225
syndrome, 205
NAION with, 34, 40
Index 275

Heparin-induced extracorporeal
Genetic mutations
for choroidal melanoma, 229
low-density lipoprotein/
of DOA, 179–180
for combined hamartoma of
fibrinogen precipitation
of FA, 186–187
retina and retinal pigment
(HELP), for NAION,
of LHON, 175–176
epithelium, 230
of Wolfram syndrome, 182–183
for GCA, 45
Hereditary ataxias
Giant cell arteritis (GCA), 42
for NAION, 34–35
FA, 186–188
diagnosis of, 44–46
OCT v., 242
SCA-1, 188
incidence of, 42
for optic disc drusen, 210–211
Hereditary motor and sensory
pathophysiology of, 42–44
for optic disc tumor, 112
neuropathy VI (HMSN6),
PION from, 50
for papilledema, 65
symptoms and signs of, 42
for racemose hemangioma, 227
Hereditary optic atrophy with
treatment of, 46–48
Fluoroquinolones, for increased
progressive deafness and
visual prognosis of AION in,
ICP, 74
polyneuropathy, 184–185
Folate treatment, for optic
Hereditary optic neuropathy,
Glial-derived growth factor
neuropathy, 154–155
(GDGF), for traumatic
Folic acid deficiency, in optic
autosomal dominant
optic neuropathy, 141
neuropathy, 155
progressive optic atrophy
Glial tumors, of retina, 221–223
Formate accumulation, in optic
with congenital deafness,
Goldenhar’s syndrome, increased
neuropathy, 153
ICP and, 74
Foster Kennedy syndrome
autosomal dominant
Gorlin syndrome, with myelinated
papilledema in, 65
progressive optic atrophy
nerve fibers, 214
suprasellar meningioma in, 98
with progressive deafness
Granulomatous infections, in ICP
Free radicals, with traumatic
and ataxia, 184
and papilledema, 67
optic neuropathy, 137
autosomal recessive optic
Grave’s ophthalmopathy
Friedreich’s ataxia (FA)
atrophy, 181
diagnostic testing for, 92–93
clinical features of, 186
autosomal recessive optic
epidemiology of, 92
diagnosis of, 187
atrophy with progressive
management of, 93–94
management of, 187–188
hearing loss, spastic
pathology of, 93
molecular genetics of, 186–187
quadriplegia, dementia,
symptoms and signs of, 92
overview of, 186
and death, 184–185
Gray matter neurodegenerative
pathophysiology of, 187
Behr’s syndrome, 181–182
disorders, infantile
Funduscopic examination
DOA, 177–180
neuroaxonal dystrophy,
of benign anterior visual
fatal X-linked optic atrophy,
pathway gliomas, 105
ataxia, and deafness, 185
Guillain-Barré syndrome,
of LHON, 172
in hereditary ataxias, 186–188
papilledema and, 67
of ONSM, 88–89
hereditary optic atrophy with
for optic disc drusen, 210
progressive deafness and
of sarcoidosis, 115
polyneuropathy, 184–185
Hamartoma. See Combined
for suprasellar meningioma, 98
in hereditary polyneuropathies,
hamartoma of retina and
for traumatic optic neuropathy,
retinal pigment epithelium
LHON, 171–177
Fundus findings, in optic neuritis,
MGA, 183
in IIH
in neurodegenerative disorders
in children, 79–80
Furosemide, for IIH, 75–76
of children, 189–192
medical treatment for, 75
NTG, 180–181
in papilledema, 62
opticoacoustic nerve atrophy
HELP. See Heparin-induced
Ganglioglioma of optic nerve,
with dementia, 185
extracorporeal low-density
other syndromes, 192
GDGF. See Glial-derived growth
PEHO syndrome, 185–186
SLOS, 192–193
Hemocysteine metabolism,
Gene therapy
Wolfram syndrome or
NAION and, 40
for LHON, 176–177
DIDMOAD, 182–183
Heparin, for cerebral venous
for traumatic optic neuropathy,
X-linked optic atrophy, 181
sinus thrombosis, 69
276 Index

ganglioglioma of optic nerve,
histopathology of, 94
Hereditary polyneuropathies
management of, 94–95
HMSN6 as, 188–189
malignant anterior visual
symptoms and signs of, 94
HSAN3 as, 189
pathway gliomas, 109–111
Idiopathic perioptic neuritis
Hereditary sensory and
secondary tumors, 111–115
diagnostic testing for, 116–117
autonomic neuropathy
leukemic infiltration, 114–115
epidemiology of, 116
type III (HSAN3), 189
lymphomatous infiltration,
management of, 117
Herpes zoster
pathology of, 117
AION with, 49
meningeal metastasis,
symptoms and signs of, 116
PION with, 50
IIH. See Increased intracranial
myelomatous and other
cryptococcosis and, 118
lymphoreticular tumor
IL-6. See Interleukin-6
optic neuritis and, 4
infiltration, 115
IL-10. See Interleukin-10
HMSN6. See Hereditary motor
other metastases, 112–113
IL-12. See Interleukin-12
and sensory neuropathy
Inflammation, in traumatic optic
Immunosuppressive agents
neuropathy, 138
for GCA, 47
Hodgkin’s disease, optic nerve
for Grave’s ophthalmopathy,
infiltration with, 113
for GCA, 47–48
Homonymous hemioptic
in optic neuropathy, 160–161
Increased intracranial
hypoplasia, 203
Interferon-alpha, in optic
hypertension (IIH)
HSAN3. See Hereditary sensory
neuropathy, 160
cerebral venous sinus
and autonomic
Interferon beta-1a
thrombosis v., 72
neuropathy type III
for CDMS, 13–14
in children, 79–80
Hyaloid system remnants,
for NMO, 17
diagnostic criteria for, 70
for optic neuritis, 13–15
epidemiology/genetics of, 70
Hydroxychloroquine retinopathy,
Interleukin-6 (IL-6)
management of, 74–79
OCT in, 243
in GCA, 44–45
medical treatment, 75–76
Hydroxyurea, for ONSM, 91
with ocular lymphoma, 113
surgical treatment, 76–79
Hyperbaric oxygen, for NAION,
Interleukin-10 (IL-10), with
neuroimaging features of, 74
ocular lymphoma, 113
in papilledema, 64, 66, 69–79
Hypertension, NAION with, 34,
Interleukin-12 (IL-12), with
pathogenesis of, 71–72
ocular lymphoma, 113
during pregnancy, 79
Hyperthyroidism, increased ICP
Internal carotid aneurysm, 101
presentation and course of,
and, 72
International Optic Nerve
Trauma Study, traumatic
symptoms of, 69
NAION with, 35, 38
optic neuropathy in,
visual course and prognosis of,
PION with, 50–51
Hypothalamic invasion, by
Intracellular calcium, with
Inducible nitric oxide synthase
benign anterior visual
traumatic optic
(NOS), with traumatic
pathway gliomas, 105–106
neuropathy, 137
optic neuropathy, 137
Hypothalamus, with malignant
Intracranial pressure (ICP),
Infantile neuroaxonal dystrophy,
anterior visual pathway
gliomas, 110
causes of, 66–67
Infiltrative optic neuropathies
Hypothyroidism, increased ICP
disorders and medications
infectious, 117–119
and, 72
with, 72–73
cryptococcosis, 118–119
features of, 62
other etiologies, 119
in IIH, 71–72
tuberculosis, 117–118
Iatrogenic malabsorption
in papilledema, 62–65
inflammatory, 115–117
syndrome, in optic
Intraneural growth, of benign
idiopathic perioptic neuritis,
neuropathy, 155–156
anterior visual pathway
ICP. See Intracranial pressure
gliomas, 107
sarcoidosis, 115–116
Idiopathic orbital inflammatory
Intraocular pressure (IOP),
primary tumors, 103–111
elevated, NAION with,
benign anterior visual
diagnostic testing for, 94
pathway gliomas, 103–109
epidemiology of, 94
Index 277

Magnetic resonance imaging
histopathology of, 174
Intravenous immunoglobulin
molecular genetics and genetic
of benign anterior visual
heterogeneity of, 175–176
for NMO, 17
pathway gliomas, 106
OCT for, 242
for optic neuritis, 13
of craniopharyngioma, 100
optic neuritis v., 3–4
IONDT. See Ischemic Optic
of cryptococcosis, 118
pathophysiology of, 174–175
of fibrous dysplasia, 103
symptoms and signs of,
Decompression Trial
of GCA, 45–46
IOP. See Intraocular pressure
of Grave’s ophthalmopathy, 92
systemic associations of,
Ischemic optic neuropathies, 31–52
of idiopathic orbital
AION, 42–49
visual prognosis of, 173
NAION, 31–42
pseudotumor, 94
Leukemic infiltration
PION, 49–52
of IIH, 70, 74
epidemiology of, 114
Ischemic Optic Neuropathy
of LHON, 172, 174
management of, 114–115
Decompression Trial
of malignant anterior visual
pathology of, 114
(IONDT), NAION in, 31
pathway gliomas, 110
symptoms and signs of, 114
ONDS, 35–36
of multiple sclerosis, 8–9
Levodopa, for NAION, 35–36
optic disc pallor, 33
of ocular lymphoma, 114
LHON. See Leber’s hereditary
visual acuity, 32
of ONSM, 89
optic neuropathy
Ishihara color plates, for optic
of optic disc tumor, 112
Linezolid, in optic neuropathy,
neuritis, 2
of papilledema, 66
Isoniazid, for tuberculosis, 117–118
of pituitary adenoma, 95–96
Lipidoses, with optic neuropathy,
IVIG, Seee Intravenous
of pituitary apoplexy, 97
of sarcoidosis, 116
Lithium, for increased ICP, 74
of sphenoid wing meningioma,
LP shunting. See
Lumboperitoneal shunting
Juvenile optic atrophy. See
of suprasellar meningioma, 98
Lumboperitoneal (LP) shunting
Dominant optic atrophy
of traumatic optic neuropathy,
complications of, 78
for IIH, 77–78
of tuberculosis, 117
ONSD v., 78
Kallidin, with traumatic optic
of Wolfram syndrome, 182
Lupus erythematosus
neuropathy, 137
Magnetization transfer ratio, in
increased ICP and, 72
Kidneys, papillorenal syndrome
NMO, 15
optic neuritis and, 5–6
with, 209
Malidixic acid, for increased ICP,
PION and, 50
Kjer’s. See Dominant optic
Lyme disease, optic neuritis and,
MBP. See Myelin basic protein
Krabbe disease, 191–192
Medulloepithelioma, 221
Lymphomatous infiltration
Kurtzke expanded disability
Megalopapilla, 203
diagnostic testing for, 113
status scale score, optic
Melanocytic tumors
epidemiology of, 113
neuritis with, 6–7
choroidal melanoma, 229
management of, 114
combined hamartoma, 230
neuroimaging of, 114
melanocytoma, 227–229
symptoms and signs of, 113
Laser interferometry, for traumatic
optic neuropathy, 136
diagnosis of, 228
Leber’s hereditary optic
histopathology of, 227
neuropathy (LHON),
symptoms of, 227
in GCA, 43, 45
treatment of, 228–229
with traumatic optic
ATP deficiency in, 152
Memantine, for traumatic optic
neuropathy, 138
diagnostic testing of, 172–173
neuropathy, 140
Macular scans, of OCT, 240
gene therapy, neuroprotection,
Meningeal metastasis
Magnetic resonance angiography
and other treatments for,
diagnostic testing for, 111–112
management of, 112
for GCA, 46
heteroplasmy and
overview of, 111
for suprasellar meningioma,
environmental factors of,
symptoms and signs of, 111
278 Index

retinal venous sheathing with,
retinal and optic nerve
Meningitis, cerebral venous sinus
disorder diagnosis with,
thrombosis and, 68
visual loss with, 1
Mesalazine, for increased ICP, 74
Mycophenolate mofetil, for
fixation errors and,
Metachromatic leukodystrophy,
NMO, 17
Myelinated nerve fibers, 214
functional and nonorganic
Methanol, in optic neuropathy,
Myelin basic protein (MBP), in
causes, 254–255
153, 156–157
optic neuritis, 6–7
outer retina disease and,
Methazolamide, for IIH, 75
Multifocal visual evoked
for GCA, 47
NAION. See Nonarteritic
potential (mfVEP),
for meningeal metastasis, 112
anterior ischemic optic
for sarcoidosis, 116
identical responses with, 257
Methylprednisolone, intravenous
Naloxone, for traumatic optic
measuring latency and
for GCA, 46
neuropathy, 138
amplitude in, 259
for Grave’s ophthalmopathy,
NASCIS-2. See National Acute
need for, 266
Spinal cord Injury Study,
optic nerve disorder diagnosis
for NMO, 16
with, 260–266
for optic neuritis, 12–13
National Acute Spinal cord
field confirmation with,
for traumatic optic neuropathy,
Injury Study, second
(NASCIS-2), traumatic
fixation error problems,
mfERG. See Multifocal
optic neuropathy in, 138
Nerve fiber layer atrophy, multiple
functional or nonorganic
mfVEP. See Multifocal visual
sclerosis with, 2–3
causes, 263
evoked potential
Neurodegenerative disorders of
glaucomatous damage
MGA. See Type III 3-
detection, 264
methylglutaconic aciduria
gray matter neurodegenerative
optic neuritis and multiple
Migraine, NAION in, 41
disorders, 190
sclerosis, 261–262
Minocycline, for increased ICP,
lipidoses, 190
poor producers of, 266
MPS, 189–190
for unreliable field takers,
Mitoxantrone, for NMO, 17
white matter
Morning glory disc anomaly
origins of, 259–260
optic disc coloboma v., 207
disorders, 190–192
overview of, 245, 255
overview of, 206
Neuroetinitis, optic neuritis with,
presentation and analysis of,
peripapillary staphyloma v., 208
symptoms and signs of, 206
Neurofibromatosis type I (NF-I)
recording of, 255–256
MPS. See Mucopolysaccharidosis
diagnostic criteria for, 104
topographical representation
MRI. See Magnetic resonance
management with, 108
of, 257–258
optic gliomas associated with,
Multiple sclerosis (MS). See also
MS. See Multiple sclerosis
Clinical definite multiple
Mucopolysaccharidosis (MPS)
prognosis with, 107–108
diagnosis of, 190
with LHON, 173
in ICP and papilledema, 66–67
of benign anterior visual
mfVEP for, 261–262
with optic neuropathy, 189–190
pathway gliomas, 106
MRI for, 8–9
Multifocal electroretinogram
of craniopharyngioma, 100
OCT for, 242
(mfERG), 245–255
of fibrous dysplasia, 103
optic disc pallor and nerve
ganglion cell damage detection
of IIH, 74
fiber layer atrophy with,
with, 255
of malignant anterior visual
measuring latency and
pathway gliomas, 110
optic neuritis with, 1–3, 6
amplitude in, 247–249
of NAION, 34
development of, 8, 10
need for, 266
of ocular lymphoma, 114
genetic factors of, 7–8
outer retina generation of, 250
of ONSM, 89
prognosis of, 10–11
overview of, 245
of PION, 51
risk factors for, 11–12
presentation of, 247
of pituitary adenoma, 95–96
tests for, 8–10
recording of, 245–247
Index 279

risk factors of, 34 Optical canal decompression, for
of pituitary apoplexy, 97
symptoms and signs of, 31–32 traumatic optic
of traumatic optic neuropathy,
treatment of, 35–37 neuropathy, 137–139
Non-Hodgkin’s lymphoma Optical coherence tomography
of tuberculosis, 117
(NHL), optic nerve (OCT)
Neuromyelitis optica (NMO)
infiltration with, 113 concluding remarks on, 243
clinical course of, 16
Norepinephrine, intravenous, for for LHON, 172–173
diagnosis of, 15–16
NAION, 35 for occult retinal disease,
epidemiology of, 15
Normal-tension glaucoma (NTG) 242–243
with optic neuritis, 15–18
DOA v., 178, 180 for optic disc drusen, 211–212
pathophysiology of, 16
overview of, 180–181 for optic nerve disorder
treatment of, 16–18
treatment of, 181 analysis, 240–242
Neuroprotection, for LHON,
NOS. See Inducible nitric oxide for optic atrophy, 240–242
synthase for optic disc edema, 240
Neurotrophins, in traumatic optic
NTG. See Normal-tension for optic nerve anomalies, 242
neuropathy, 141
glaucoma for optic neuritis, 10
NF-I. See Neurofibromatosis type
Nutritional optic neuropathies, overview of, 235
150–164 techniques of, 235–240
NHL. See Non-Hodgkin’s
Cuban epidemic of, 153 macular scans, 240
evaluation of, 150–152 optic disc analysis, 237–239
NLP. See No light perception
signs of, 150 papillomacular axis line
NMDA receptor blockers, for
symptoms of, 150 scan, 235–237
traumatic optic
types of, 151–155 peripapillary retinal nerve
neuropathy, 140
Nystagmus, in benign anterior fiber layer scan, 235–236
NMO. See Neuromyelitis optica
visual pathway gliomas, for traumatic optic neuropathy,
No light perception (NLP), with
105 135
traumatic optic
Optic disc
neuropathy, 139–140
in AION, 42
Nonarteritic anterior ischemic O
with malignant anterior visual
optic neuropathy Obesity
pathway gliomas, 110
(NAION), 31–42 with IIH, 71
in NAION, 31–32
AION v., 42 with papilledema, 72
in Papilledema, 62
course and prognosis of, 32–33 Occult retinal disease, OCT for,
in PION, 49
diagnostic tests of, 34 242–243
Optic disc analysis, of OCT,
differential diagnosis of, 33–34 OCT. See Optical coherence
atypical features, 33 tomography
Optic disc coloboma
rim pallor, 33–34 Ocular adnexa, in traumatic optic
overview of, 207
incidence of, 31 neuropathy, 135
symptoms and signs of,
OCT for, 240 Ocular motility deficits, with
with optic disc drusen, 212 craniopharyngioma, 100
Optic disc drusen
optic neuritis v., 33–34 ONDS. See Optic nerve
complications of, 212–213
other settings of, 37–42 decompression surgery
diagnostic tests for, 210–212
chlamydia, 38 ONSD. See Optic nerve sheath
NAION with, 37–38
chronic anemias, 40 fenestration
overview of, 210
coagulopathies, 40–41 ONSM. See Optic nerve sheath
symptoms and signs of, 210
diabetic papillopathy, 37 meningiomas
treatment of, 213
elevated introcular pressure, ONTT. See Optic Neuritis
Optic disc dysplasia, 209
39–40 Treatment Trial
Optic disc excavation
in migraine, 41 OPA1 mutations, in DOA,
in DOA, 178
optic disc drusen associated 177–180
in NTG, 178
with, 37–38 OPA2 mutations, in X-linked
Optic disc pallor
shock-induced, 38–39 optic atrophy, 181
multiple sclerosis with, 2–3
sleep apnea syndrome, Ophthalmoplegia, in benign
in NAION, 33
41–42 anterior visual pathway
visual residual deficits with, 11
pathogenesis of, 34–35 gliomas, 105
280 Index

autosomal dominant
neuroimaging features of, 89
Optic disc pit
progressive optic atrophy
prognosis and treatment of,
overview of, 208
with progressive deafness
symptoms and signs of,
and ataxia, 184
symptoms and signs of, 88–89
autosomal recessive optic
Optic neuritis, 1–21
Optic disc tumor, 220–231
atrophy, 181
clinical presentation of, 1–3
diagnostic testing for, 112
autosomal recessive optic
signs, 1–3
glial tumors of retina, 221–223
atrophy with progressive
symptoms, 1
management of, 112–113
hearing loss, spastic
diagnostic and prognostic tests
melanocytic tumors, 227–230
quadriplegia, dementia,
for, 8–10
choroidal melanoma, 229
and death, 184–185
atypical, 10
combined hamartoma, 230
Behr’s syndrome, 181–182
typical, 8–10
melanocytoma, 227–229
DOA, 177–180
differential diagnosis of, 3–6
prognosis of, 113
fatal X-linked optic atrophy,
mfVEP for, 261–262
of sensory retina and
ataxia, and deafness, 185
MS risk factors with, 11–12
medullary epithelium,
in hereditary ataxias,
in children, 11–12
recurrent, 11
medulloepithelioma, 221
hereditary optic atrophy
NAION v., 33–34
retinoblastoma, 220–221
with progressive deafness
neuromyelitis optica with, 15–18
symptoms and signs of, 112
and polyneuropathy,
diagnosis of, 15–16
vascular tumors of retina,
epidemiology of, 15
in hereditary
overview of, 1
Optic nerve
polyneuropathies, 188–189
paraneoplastic optic
anatomy of, 130–132
LHON, 171–177
neuropathy syndromes,
intracanalicular optic nerve,
MGA, 183
in neurodegenerative
pathogenesis of, 6–8
intracranial optic nerve, 132
disorders of children,
demyelination, 6–7
optic nerve head, 130–131
epidemiological factors, 8
orbital optic nerve, 131–132
NTG, 180–181
genetic factors, 7–8
avulsion of, 133
opticoacoustic nerve atrophy
treatment of, 12–15
mfERG diagnosing of 2,
with dementia, 185
corticosteroids, 12–13
other syndromes, 192
interferon beta-1a, 13–15
swelling of, 133
PEHO syndrome, 185–186
IVIG, 13
Optic nerve decompression
SLOS, 192–193
plasmapheresis, 13
surgery (ONDS), for
Wolfram syndrome or
visual prognosis with, 10–11
NAION, 35–36
DIDMOAD, 182–183
visual residual deficits with, 11
Optic nerve hemangioblastoma
X-linked optic atrophy, 181
Optic Neuritis Treatment Trial
overview of, 225
infiltrative, 103–119
symptoms and signs of, 225–226
infectious, 117–119
conclusions from, 13
treatment for, 226
inflammatory, 115–117
MRI and MS with, 8
Optic nerve hypoplasia, 201–203
primary tumors, 103–111
optic neuritis differentiated
diagnosis of, 201
secondary tumors, 111–115
with, 2
neuroimaging of, 202–203
nutritional, 150–164
Optic neuropathies, 88–119
overview of, 201
Cuban epidemic of, 153
compressive, 88–103
Optic nerve sheath fenestration
evaluation of, 150–152
of anterior visual pathway,
folic acid deficiency, 155
benefits of, 76–77
iatrogenic malabsorption
of optic nerve, 92–95
complications of, 77
syndrome-related, 155–156
sellar and suprasellar
for IIH, 76–77, 79
other epidemics of, 154–155
compressive lesions, 95–103
LP shunting v., 78
signs of, 150
hereditary, 171–193
overview of, 76
symptoms of, 150
autosomal dominant
Optic nerve sheath meningiomas
thiamine/B1 deficiency, 155
progressive optic atrophy
with congenital deafness,
epidemiology of, 88 tobacco-alcohol amblyopia,
histopathology of, 89–90 154
Index 281

Peripapillary retinal nerve fiber
management, 74–79
vitamin B12 deficiency,
layer scan, of OCT,
neuroimaging features, 74
pathogenesis, 71–72
vitamin E deficiency, 155
Peripapillary staphyloma, 208
during pregnancy, 79
zinc deficiency, 155
Pertussis, optic neuritis with, 4
visual course and prognosis,
toxic, 150–164
amiodarone- and digoxin-
in optic neuritis, 1
with malignant anterior visual
associated, 157–159
in papilledema, 62
pathway gliomas, 110
Photophobia, in optic neuritis, 1
OCT for, 240
Photopsias, in papilledema, 62
pathology of, 66
clomiphene citrate-
Pial capillary plexus, in PION, 49
signs of, 62–65
associated, 161
PION. See Posterior ischemic
chronic, 64–65
Cuban epidemic of, 153
optic neuropathy
early stages, 62–63
disulfiram-associated, 159
Pituitary adenoma
late stages, 63
ethambutol-associated, 159
epidemiology of, 95
symptoms of, 62
ethylene glycol-associated,
management of, 96–97
unilateral, 65
neuroimaging of, 95–96
visual loss mechanisms in, 66
evaluation of, 150–152
pathology of, 96
Papillitis, optic neuritis with, 2, 5,
symptoms and signs of, 95
Pituitary apoplexy
Papillomacular axis line scan, of
epidemiology of, 97
OCT, 235–237
neuroimaging of, 97
Papillorenal syndrome, 209
linezolid-associated, 160
pathogenesis of, 97
Parainfectious optic neuritis,
methanol-associated, 153,
symptoms and signs of, 97
optic neuritis v., 4
treatment of, 97
Paraneoplastic optic neuropathy
methanol-induced, 156–157
other epidemics of, 154–155
for NMO, 18
malignancies associated with,
radiation-induced, 162–164
for optic neuritis, 13
signs of, 150
Platelet-derived growth factor
optic neuritis with, 18–21
sildenafil- and tadalafil-
(PDGF), in GCA, 43
steroids for, 21
associated, 161–162
Polyarteritis nodosa
treatment outcomes for, 19–20
symptoms of, 150
AION with, 49
Paton’s lines, with elevated ICP,
tamoxifen-associated, 161
PION with, 50
tobacco-alcohol amblyopia,
Posterior ischemic optic
PCNSL. See Primary central
neuropathy (PION), 49–52
nervous system lymphoma
toluene associated, 157
incidence of, 49
PDGF. See Platelet-derived
traumatic, 130–144
pathophysiology of, 49
growth factor
of head injury, 130–142
perioperative, 50–52
PEHO syndrome. See
of ocular surgery, 142–144
symptoms and signs of, 49
Opticoacoustic nerve atrophy
in systemic disorders, 49–50
encephalopathy with
with dementia, 185
treatment of, 51–52
edema, hypsarrhythmia,
Oral contraceptives, for increased
Prednisone, oral
and optic atrophy
ICP, 74
for GCA, 46–47
Pelizaeus-Merzbacher disease,
Orbital emphysema, 133
for Grave’s ophthalmopathy,
Perforating diathermy, for
for idiopathic orbital
capillary hemangioma, 224
Papilledema, 62–80
Periarteritis nodosa, AION with,
asymmetric, 65
pseudotumor, 94
cerebral venous sinus
for idiopathic perioptic
Perineural growth, of benign
thrombosis in, 67–69
neuritis, 117
anterior visual pathway
with craniopharyngioma, 100
for NMO, 16–17
gliomas, 107
diagnostic testing for, 65–66
for optic neuritis, 12–13
Periorbital injections, traumatic
IIH and, 64, 67, 69–79
for sarcoidosis, 116
optic neuropathies with,
diagnostic criteria, 70
for tuberculosis, 118
epidemiology/genetics, 70
282 Index

Segmental optic nerve
Primary central nervous system
hypoplasia, 203
for choroidal melanoma, 229
lymphoma (PCNSL), optic
Shock-induced anterior ischemic
for Grave’s ophthalmopathy,
nerve infiltration with, 113
optic neuropathy
Progesterone, for increased ICP,
(SIAION), 38–39
for ocular lymphoma, 114
Short tau inversion recovery
for ONSM, 90
Progressive encephalopathy with
for pituitary adenoma, 96
edema, hypsarrhythmia,
for Grave’s ophthalmopathy,
Relapsing polychondritis, AION
and optic atrophy (PEHO
with, 49
syndrome), 185–186
for sphenoid sinus mucocele,
Retina, glial tumors of, 221–223
Proptosis, in benign anterior
Retinal hemorrhages, with optic
visual pathway gliomas,
SIAION. See Shock-induced
disc drusen, 212
anterior ischemic optic
Retinal nerve fiber layer (RNFL)
Pseudotumor cerebri
OCT measurement of, 10, 235
pathogenesis of, 71
Sickle cell disease, PION in, 50
with traumatic optic
prognosis of, 71
Sildenafil, in optic neuropathy,
neuropathy, 135
Pseudoxanthoma elasticum, with
Retinal venous sheathing, with
optic disc drusen, 212–213
Sleep apnea syndrome
MS, 3
Pulsatile tinnitus, in papilledema,
IIH and, 73–74
Retinitis pigmentosa
NAION and, 41–42
OCT in, 243
Pupillary abnormality, in optic
SLOS. See Smith-Lemli-Opitz
with optic disc drusen, 212
neuritis, 2
Pyrazinamide, for tuberculosis,
Smith-Lemli-Opitz syndrome
histopathology of, 220–221
(SLOS), 192–193
overview of, 220
Pyridoxine, for tuberculosis, 118
Smoking. See Tobacco use
symptoms and signs of, 220
Sphenoid sinus mucocele, 102
treatment of, 221
Sphenoid wing meningioma
Retrobulbar optic neuritis, 2
Quadrantic defects, in NAION, 31
epidemiology of, 99
Rheumatoid arthritis, AION
Quinagolide, for pituitary
management of, 99
with, 49
adenoma, 96
neuroimaging of, 99
Rifampin, for tuberculosis,
pathology of, 99
symptoms and signs of, 99
Rim pallor, in NAION, 33–34
Racemose hemangioma
Spinocerebellar ataxia type 1
Rituximab, for NMO, 17
diagnostic testing for, 227
(SCA-1), 188
RNFL. See Retinal nerve fiber
overview of, 226
Stellate ganglion blocks, for
symptoms and signs of, 226–227
visual prognosis for, 227
Stem cell implantation, for
for ONSM, 91
Radiation therapy
traumatic optic
for suprasellar meningioma, 99
for benign anterior visual
neuropathy, 141–142
pathway gliomas, 109
Steroids, for paraneoplastic optic
for craniopharyngioma, 101
neuropathy syndromes, 21
low-dose, for idiopathic orbital
STIR. See Short tau inversion
diagnostic testing for, 116
epidemiology of, 115
pseudotumor, 94
Strachan’s syndrome, as optic
management of, 116
for malignant anterior visual
neuropathy, 154
optic neuritis in, 4
pathway gliomas, 111
Sulfa drugs, for increased ICP, 74
pathology of, 116
for meningeal metastasis, 112
Suprasellar meningioma
symptoms and signs of, 115
for ocular leukemia, 114–115
course and visual prognosis of,
SCA-1. See Spinocerebellar
for optic disc tumor, 112–113
ataxia type 1
for optic neuropathy, 162–164
epidemiology of, 97
Scleritis, posterior, optic neuritis
for paraneoplastic optic
management of, 99
with, 4
neuropathy syndromes, 21
neuroimaging of, 98
Scotomas, with lymphomatous
for sphenoid wing
pathology of, 98
infiltration of optic nerve,
meningioma, 99
symptoms and signs of, 97–98
for suprasellar meningioma, 99
Index 283

evaluation of, 150–152
OCT for, 242
for benign anterior visual
for cavernous hemangioma,
signs of, 150
pathway gliomas, 108–109
symptoms of, 150
for craniopharyngioma, 101
of optic disc tumor, 112
types of, 156–164
for fibrous dysplasia, 103
Uthohff’s phenomenon
Trabeculectomy, traumatic optic
for pituitary adenoma, 96
cause of, 11
neuropathies after,
for sphenoid wing
with LHON, 173
meningioma, 99
optic neuritis with, 10–11
Transvitreal optic neurotomy, for
for suprasellar meningioma, 99
for traumatic optic neuropathy,
posterior, optic neuritis with,
Traumatic optic neuropathies,
sarcoidosis with, 115
of head injury, 130–142
diagnostic tests for,
Tadalafil, in optic neuropathy,
Vascular endothelial growth
epidemiology of, 130
Takayasu’s arteritis, AION with,
factor (VEGF), in GCA, 43
localization of direct,
Vascular tumors of retina
Tamoxifen, in optic neuropathy,
capillary hemangioma, 223–224
localization of indirect,
cavernous hemangioma,
Tay-Sachs disease, 190
management of, 138–140
T cells
optic nerve hemangioblastoma,
new perspectives for,
in GCA, 43
for traumatic optic neuropathy,
racemose hemangioma,
optic nerve anatomy,
Temporal artery biopsy, for
Vasospasm, NAION with, 35
pathogenesis of, 137–138
GCA, 45
VEGF. See Vascular endothelial
pathology of, 136–137
Temporal lobes, with malignant
growth factor
visual prognosis of, 136
anterior visual pathway
Ventriculoperitoneal (VP)
of ocular surgery, 142–144
gliomas, 110
shunting, for IIH, 77–78
after blepharoplasty, 144
VEP. See Visual evoked potential
after cataract surgery,
in IIH, 75
Vincristine, for benign anterior
for increased ICP, 74
visual pathway gliomas,
after endoscopic sinus
Thiamine deficiency, in optic
surgery, 144
neuropathy, 155
Vision loss
with periorbital injections,
Thiazide diuretics, for IIH, 79
in AION, 42
Thrombocytosis, GCA and, 44
in choroidal melanoma, 229
after trabeculectomy,
Thrombolytic agents, for NAION,
in combined hamartoma of
retina and retinal pigment
after vitrectomy, 143
Thyroid autoantibodies, in
epithelium, 230
Tuberculin skin test, for
neuromyelitis optica, 15–16
in craniopharyngioma, 100
tuberculosis, 117
Thyroid ophthalmopathy, with
in DOA, 177–178
Grave’s ophthalmopathy,
in idiopathic perioptic neuritis,
epidemiology of, 117
management of, 117–118
Tobacco-alcohol amblyopia, optic
in IIH, 70–71
neuroimaging of, 117
neuropathy with, 154
medical treatment for, 75
optic neuritis with, 4
Tobacco use
in LHON, 171
pathology of, 117
with Grave’s ophthalmopathy,
in malignant anterior visual
symptoms and signs of, 117
Tumor-necrosis factor-α, in GCA, pathway gliomas, 109
NAION with, 34, 40
in melanocytoma, 228
optic neuropathy with, 154
in NTG, 181
Type III 3-methylglutaconic
Toluene, in optic neuropathy, 157
in optic disc coloboma, 207
aciduria (MGA), 183
Topiramate, for IIH, 75
in optic neuritis, 1
Typhoid fever, optic neuritis with,
Toxic optic neuropathies,
in papilledema, 66
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