Chapter 6: Introduction to Neuro-

ophthalmology
Timothy Root, MD

A third of the brain is devoted to the visual system, so neurology is

an important topic! While the rest of ophthalmology involves image
recognition (your ability to recognize corneal abrasions, disk
cupping, and infections under the microscope), neuroophthalmology
requires a more “cerebral” analysis. This makes learning the subject
painful at first – trust me, it becomes more entertaining as you
progress! For our purposes, I’m going to keep things simple and only
cover topics that you should know as a medical student.

FUN FACT:
Phrenology is the study of the morphology of
the skull, and was developed by Franz Josef
Gall (1758 – 1828). Gall felt there was a direct
link between the shape of the skull and human
character and intelligence. While complete
bunk, Gall was one of the first to consider the
brain the source of all mental activities.

Phrenology was very popular in America throughout the 1800’s and

its influence can still be seen in our language. For example, people
with “high brows” were considered more intelligent than those with
“low brows.”

Diplopia:
A common complaint you’re going to be faced with is “double vision.”
Patients frequently complain of doubling — and sometimes they
actually mean it! Often, however, they just mean that their vision
looks blurry. Technically, the phrase diplopia describes the symptom
of seeing two different images of the same object, and that’s what
we are going to discuss!

When faced with a diplopic patient, there is an important question

you must immediately answer: before breaking out your arsenal of
neuroophthalmic flags, prisms, and muscle lights, you must
distinguish whether the diplopia is monocular or binocular. If the
double-vision remains when you cover an eye then you have a
monocular diplopia. You should breath a sigh of relief at this point –
because monocular double vision isn’t a neurologic problem at all
and your exam just got easier!

Monocular doubling is often caused by a refractive problem in the

front part of the eye. There aren’t any mechanisms of monocular
doubling that occur at the retina or further back in the neuro
pathway. The most common cause of monocular diplopia is
astigmatism, an abnormal curvature of the corneal surface. New
onset astigmatism could occur from corneal deformation from an
overlying lid lesion or after surgery with tight corneal stitches
through the cornea. Other causes of monocular diplopia include
cataract irregularities, lens displacement, or primary problems with
corneal curvature such as keratoconus.

Binocular diplopia, on the other hand, occurs when the eyes do not
move in synchrony with each other. This can occur from nerve
lesions (a palsy of CN3, CN4, or CN6), extraocular muscle
abnormalities (such as the muscle-fibrosis that occurs with Grave’s
Disease), or derangements at the neuromuscular junction
(myasthenia gravis). Let’s explore the cranial nerve palsies first.

Cranial Nerves and EOMs

Three cranial nerves control the
movements of the eyeballs. The
relationships of these muscles can be
quite complex as the eyeballs are
neurologically “yoked” together and every
muscle has multiple vectors of force,
depending upon the direction that the eye
is looking. Nerve palsies can be challenging to work out!

There are numerous causes for the individual nerve palsies, including
microvascular disease, strokes, tumors, and aneurysms. If you’re like
myself, you’re probably not up-to-speed on your neuroanatomy. I’ve
drawn this cartoon picture of the brainstem for you to reference over
the next few pages.

Third Nerve Palsy

Oculomotor nerve pasly is the easiest to detect because a complete
3rd nerve palsy looks dramatic. The majority of the extraocular
muscles are innervated by CN3, so when knocked-out the eye
deviates down and out because of the still functioning abducens and
superior oblique muscles. In addition, the levator palpebrae (the main
lid retractor) is innervated by CN3 and its paralysis gives you a
severe eyelid ptosis. Finally, the parasympathetic pupil-constrictor
fibers from the Edinger-Westphal nucleus travel within CN3, and
their loss gives you a “blown pupil.”
Most third nerve palsies are caused by ischemic
events at the nerve secondary to hypertension or
diabetes. The one thing you really need to worry
about in these patients is a compressive aneurysm
pushing on the nerve. These aneurysms occur at
the junction of the posterior communicating artery
and the internal carotid artery. Compressive lesions
usually affect the parasympathetic nerve
component: a blown pupil is a potential emergency. Whenever you
have pupillary involvement, you need an MRI and angiography to rule
out a dangerous aneurysm or tumor.

Pupil Involvement with CN3:

Oculomotor palsies often have pupillary involvement because the
parasympathetic nerves innervating the iris travel with the third
nerve. Pupillary involvement is a crucial diagnostic sign —
compressive lesions tend to involve the pupil, while vascular lesions
actually spare it! This picture isn’t drawn to scale, but graphically
demonstrates what I’m talking about.

As you can see, the parasympathetic nerves course along the

surface of the oculomotor nerve making them susceptible to
compressive lesions from the outside such as an aneurysm from the
posterior communicating artery, boney structures, or the uncal
portion of the temporal lobe. Ischemic lesions (caused by HTN and
diabetes) occur deeper within the oculomotor nerve and thus spare
the superficial parasympathetic fibers.

If you have a patient with CN3 loss and pupillary involvement you
should order an MRI and an angiogram to look for the compressive
site. If there isn’t pupillary involvement, they are probably suffering
from a vaso-occlusive problem, so you should check their glucose
and blood pressure.

Abducens (VI):
The abducens nerve controls the lateral rectus
muscle. Loss of CN6 renders the eye unable to
abduct (turn out). Patients will go cross-eyed, so to
compensate they may turn their head to avoid
double vision.

If you look back in that drawing of the brainstem,

you’ll see that the abducens nerve is located further down the
brainstem, “all by its lonesome” down in the pons. The nerve root
exits the brainstem even further down at the ponto-medulary
junction and has to run up the floor of the skull to get to the
cavernous sinus and into the orbit. Where the nerve enters the
cavernous sinus, it makes an abrupt 90-degree bend. Something
about this abrupt turn makes the 6th nerve susceptible to high
intracranial pressure. Patients with high ICP from pseudotumor
cerebri commonly have their 6th nerve(s) knocked out – an
abducens palsy is actually incorporated into the Dandy criteria for
diagnosing PTC.

FUN FACT:
Crocodiles shed tears, but this isn’t a sign of grief. These secretions
help shed salt-water from the eye. Thus, the term “crocodile tears” is
used to describe false tears. In ophthalmology, we use the term to
describe aberrant regeneration after 7th nerve injury – nerves that
normally control salivation are routed to the lacrimal gland. This
makes you “cry” when you see food. This can be treated by injecting
botox into the lacrimal gland.

Aberrant regeneration occurs with other cranial nerve palsies as well

— the most commonly seen is after a 3rd nerve palsy. As the
oculomotor nerves grow back to their muscles they can get mixed
up. For example, a patient could look medially (activating their medial
rectus) and their eyelid can shoot up (inappropriate co-contraction
of the levator palpebrae).

You only get this kind of synkinesis with trauma or mass lesions that
disrupt the nerve sheath. Microvascular events occur deeper in the
nerve and don’t cause aberrant regeneration. If you find aberrant
degeneration in a patient you previously assumed was from
diabetes, you better get imaging to rule out something more
dangerous.

Trochlear Nerve (IV):

The trochlear nerve (CN4) innervates the
superior oblique muscle. Trochlear paralysis is
the hardest cranial nerve palsy to diagnose and
many ophthalmologists and neurologists will
miss these! These patients have an upward
deviation of the affected eye and a
“cyclotorsion” twisting of the eye that makes
them tilt their head away from the lesion. Don’t
try to memorize these deviations: in a few
paragraphs I’ll cover the anatomy of the superior oblique muscle
which will make it easier to conceptualize these findings.
A trochlear nerve lesion is caused by either trauma, an ischemic
event, or can be congenitally present with later decompensation. The
fourth cranial nerve is the skinniest nerve and runs the longest
distance inside the cranial vault. This long passage makes it more
susceptible to injury if the brain sloshes around and bounces against
the tentorium. The fourth nerve is also susceptible to being pulled
from the root where it exits from the back of the brainstem. The long
course also makes it susceptible to neoplasm. If we break down
trochlear palsy by cause:

1/3rd Trauma
1/3rd Congenital
1/3rd Ischemic (diabetic)
1/3rd Tumor

That’s a lot of thirds, I know. That’s because reports differ depending

upon what age-group you look at: certainly more 4th palsies occur in
elderly males from trauma and more congenital palsies are found in
the pediatric population. Ask about history of closed-head injuries
and check old photographs for head-tilt – this would indicate an
old/congenital palsy that has recently decompensated.

Troclear Muscle Action:

The superior oblique muscle runs from the back
of the orbit, forward through a trochlear “pully”
located next to the upper nasal bridge, before
inserting at the back of the eye. This pulley
system completely changes the direction of
force of the superior oblique … you can think of
the trochlear pulley as the “functional origin” of
this muscle.

As you can see in this picture, the superior oblique muscle inserts
onto the back of the eyeball and then yanks the eye down. There is
also an intorsional component that rotates the 12-o’clock corneal
limbus towards the nose. This explains the head-tilt these patients
develop.

A deeper peek at the superior oblique:

To simulate the action of the superior oblique, you can pretend that
your head is a large eyeball. Throw an arm up and wrap it around the
back of your head. Your elbow becomes the trochlear pully … if you
pull your arm, you’re whole head should twist. The direction of head
movement, either up-down or rotational, will depend upon which
direction you’re looking when you start pulling.

The same action occurs in the eyeball such that your patient will see
vertical diplopia when looking medially toward the nose (such as
when reading a book) and will see more rotational doubling when
looking to the side. Think about that one for a minute!

Summary of the EOMs:

Beyond the information above, there isn’t much to localizing a cranial
nerve lesion. Just think about the anatomy: if a single nerve is
affected, then you know the problem is somewhere along that
nerve’s tract. If all three nerves are knocked out, then the lesion is
probably near the cavernous sinus where the three nerves are
bunched together.

The number one reason that any of the cranial nerve gets knocked
out is from a vasculitic event, usually from diabetes. Many of these
isolated cranial nerve palsies don’t need imaging – such as an
isolated 6th nerve palsy in an elderly diabetic. However, you don’t
want to miss an aneurysm or mass lesion, so no one will fault you for
over-imaging. Here are the high-yield facts you should know:

CN3: The eyes are “down and out” with a droopy eyelid. Think
of an aneurym if the pupil is blown.
CN4: Patient tilts their head away from the lesion. Think of
trauma or a congenital head-tilt that has decompensated with
age.
CN6: The patient looks “cross-eyed.” Consider increased
intracranial pressure.

Myasthenia Gravis:
Myasthenia gravis is a rare autoimmune disease in which the body
develops autoimmune antibodies to the nicotinic acetylcholine
receptors located at the neuromuscular junction of striated muscle.
This leads to fatigable muscles and often involves the eye, causing
diplopia and ptosis.

MG patients develop autoantibodies that actually bind to the

receptor and block the receptor binding sites, and eventually destroy
the receptor entirely, leaving patients with decreased numbers of
Ach receptors. Once the number of receptors drops below 30%
normal, then the patient becomes symptomatic, with easily
fatigability. Interestingly, only striated muscle is affected, as both
smooth and cardiac muscle appear to have different antigenicity and
are unaffected with this disease. The bulbar muscles, however, are
quite susceptible, and the majority of patients with MG have ocular
complaints – the ophthalmologist is often the first doctor to diagnose
this disorder.

The diplopia and ptosis is usually worse on

prolonged upgaze: you can test this by
having your patient look at your raised
finger to see who tires out first. More
definitive diagnosis can be made via the
Tensilon test where you give edrophonium chloride (an
anticholineresterase) and look for an improvement in symptoms as
their Ach levels build up. We don’t actually do this test in our office
because of the difficulty of starting IV lines and potential toxic
reactions such as sweating, salivation, bronchospasm, and
bradycardia. More commonly we’ll perform a rest-test or ice-test
where you have the patient hold an icepack over their closed eyes
and then remove it and look for improvement. Neurology can also
perform EMG studies and other tests to help you with this diagnosis.

Systemically, these patients can have problems with mastication,

talking, drinking, and swallowing. Aspiration pneumonia and
respiratory failure from inability to clear secretions is the big killer
with this disease. Remember, if your patient has MG, work them up
for a thymoma and check their thyroid levels.

Neuritis and Neuropathies of the Optic Nerve

Personally, I always found this topic confusing because the terms
“optic neuritis” and “optic neuropathy” sound very similar. After all,
what’s the difference between an “itis” and an “opathy?” There’s
really only three main optic-nerve entities that I think you need to be
aware of, and each has a slightly different presentation and
mechanism:
1. ON (Optic Neuritis)
An “inflammation” of the nerve, often demyelinating. The cardinal
signs in these patients are decreased vision (especially color vision),
pain with eye-movement, enhancement of the optic nerve on MRI,
and potential association with multiple sclerosis. Occurs in younger
patients.

2. ION (Ischemic Optic Neuropathy)

… sometimes called NAION (non-arteritic ischemic optic
neuropathy)
This is a localized ischemic event at the junction of the optic nerve as
it enters the back of the eyeball. This portion of the optic nerve has
no elastic “give” and a small vascular insult here can lead to swelling
and vision loss. The hemispheric vascular supply to the optic nerve
head usually generates an altitudinal visual defect. This entity usually
occurs in middle-age in those with a predisposed crowded optic
disks (the so-called “disk at risk”).

3. GCA (Giant cell arteritis) i.e. temporal arteritis

Temporal arteritis occurs as a result of a vasculitis within the medium
and small-sized arteries around the head. The vasculitis can lead to a
sudden occlusion of the blood supply to the eye leading to sudden
and permanent vision loss This happens in older patients, usually
over 70 years of age.

Let’s explore each of these entities in more detail …

Multiple Sclerosis and Optic Neuritis:

Multiple sclerosis is a demyelinating disease of the CNS that is
classically described as “lesions occurring at different times and
different places.” It occurs most commonly in young white women
from northern climates. If the lesion hits the optic nerve, then we call
this finding optic neuritis. About 90% of patients with multiple
sclerosis will develop optic neuritis at some point, and conversely,
patients with “optic neuritis” sometimes progress to develop multiple
sclerosis. Think about that one for a moment!

Signs and symptoms of optic neuritis include:

– Sudden vision loss (central scotoma is classic)
– Decreased contrast and color sensitivity
– Pain with eye movement
– Optic nerve head edema
– Afferent pupillary defect

A patient with optic neuritis needs an MRI of the brain and orbits to
look for enhancing lesions. The more demyelinating lesions found on
imaging, the higher the chance of later developing multiple sclerosis.
Patients with optic neuritis are treated with IV steroids, which will
speed recovery, but won’t ultimately affect the outcome of the
disease. WARNING: You treat with IV steroids only, as oral steroids
may actually increase the reoccurrence of MS! If enhancing lesions
are found in the brain, then you can get neurology involved to
discuss possible treatment with interferons like Avonex to decrease
progression.

FUN FACT
People blink, on average, once every 5-6 seconds.
Women blink almost twice as often as men.

Temporal Arteritis:
Temporal arteritis (also known as giant cell arteritis) is an important
syndrome to keep in the back of your head. While not terribly
common, you might save a patient from complete blindness or death
if you treat them appropriately.

Temporal arteritis is an inflammation that affects the medium-sized

blood vessels. This disease process is similar to polymyalgia
rheumatica except that the vasculitis affects the arteries supplying
the head, face, and eyes. If the blood supply to the eye is affected,
then patients can have catastrophic vision loss. These patients are
almost always older (over 60 and more commonly over 80 years of
age) and present with sudden, painless vision loss. Other preceding
systemic complaints (these are pathognomonic) include:

– Scalp tenderness and headache

– Jaw claudication
– Polymyalgias of the arms and shoulders
– Fevers, night sweats, weight loss

If you suspect GCA you need to order an ESR and CRP as these are
sensitive markers for inflammation. Normal ESR is approximately half
the patient’s age (i.e., an 80-year-old man can have ESR up to 40).
Unfortunately, these labs aren’t very specific and more definitive
diagnosis is made via a temporal artery biopsy (dissect out the artery
at the temple and send it to pathology). On pathology you’ll find
disruption of the internal elastic lamina and occasionally giant cells
(the presence of these cells isn’t actually necessary for the
diagnosis).

You treat temporal arteritis with steroids to decrease inflammation.

While steroids won’t regain lost vision, they will decrease potential
vision loss in the remaining eye, which can be affected within days.
Unfortunately, steroids also decrease the diagnostic yield on your
biopsy. This places you in a dilemma: do you hold off steroids until
after the biopsy, or start steroids and potentially mess up your biopsy
results? Well, the answer is that you start the steroids immediately to
keep the other eye from being affected. You don’t want to blind your
patient – the biopsy can be delayed for up to two weeks and still be
ok, despite the steroids.
The Pupil
The pupil is controlled by a steady balance between the
parasympathetic (which constricts the pupil) and the sympathetic
input (which dilates the pupil). I always remember this with the
mneumonic:

“If a grizzly bear attacks me in the dark woods, my sympathetic

fight-or-flight reflex dilates my eyes so I can see better as I run
away.”

Horner Syndrome
Horners occurs when the sympathetic pathway gets knocked out.
Without sympathetic input to the pupillary dilator muscles, the pupil
constricts and stays small. Sympathetic loss also creates a mild
ptosis from decreased Muller’s muscle action in the eyelid and scalp
anhydrosis (decreased sweating) on the affected side. As you can
see in the picture below, the sympathetic chain is long, complex, and
can be damaged at many levels. To localize the lesion we use a
series of eyedrops:

Cocaine Test
The first test we perform is the cocaine
test – just to decide if this patient REALLY
has a Horners pupil or not. Cocaine
stimulates pupillary sympathetics by
decreasing norepinephrine uptake at the
synaptic cleft. If a patient (a Horner’s
patient) has no sympathetic tone inside
the eye, then cocaine won’t have any
norepinephrine to build up and thus has no
effect on that eye. However, the good eye
will dilate like the dickens! It’s not always easy to obtain cocaine in
private practice, but you should be able to get some in most
hospitals as ENT people use it to control nose bleeding during
surgery.

Paradrine Test
Now that we know there’s a sympathetic palsy, we need to localize
the lesion. There are three neurons in the pathway from the brain…
just like the muscles motor-innervation throughout the rest of the
body. Unlike a leg muscle, however, we can’t check the pupillary
reflex by hitting it with a hammer. We can stimulate that final 3rd
order nerve by pharmacologically hammering it with
hydroxyamphetamine. This drug forces the end-nerve to fire-away at
the pupil. If that pupil still won’t dilate, then you know the final “lower
motor neuron” is dead. If the pupil DOES dilate, then you must have a
“higher order” nerve that’s out – lots of bad things can occur along
this upper-pathway (carotid dissections, pancoast tumors, etc.) so
procede to imaging.

This pharmacological testing is a convoluted topic, and you’ll find it

difficult to really remember these drops until you see your first
Horner’s patient. One catch phrase you should remember, though: if
a patient complains of a painful Horners think of a carotid dissection
and move quickly to rule out this diagnosis.

Adie’s Tonic Pupil

An Adie’s pupil is the opposite of a Horner’s – the parasympathetic
(constrictor) pathway gets knocked out on its way to the iris sphinter
muscles. On exam, the eye is dilated and doesn’t constrict to light
(we’re blocking the parasympathetic pathway from the Edinger-
Westphal nucleus). The pupil will constrict with near vision- but very
slowly. That’s why we call it a “tonic pupil,” it’s tonically slow.

Fortunately for us, the parasympathetic pathway is much shorter

than the convoluted sympathetic pathway, so potential causes are
more benign. The parasympathetic plexus sits right behind the eye
and can be damaged after an otherwise benign viral infection.

PIMP QUESTIONS
1. You have a patient with diplopia. His left eye is turned down
and out and his lid is ptotic on that side. What nerve do you
suspect and what should you check next?
This sounds like a CN3 palsy, and you should check his pupillary
reflex. Pupillary involvement suggests the lesion is from a
compressive source such as an aneurysm.

2. Why do diabetic patients with oculomotor paralysis have

“sparing of their pupil?”
The pupil is typically spared with iscemic third nerve palsies caused
by vascular problems. This is because the parasympathetic pupillary
fibers run along the surface of the nerve, making them susceptible to
aneurysm/tumor compression but resistant to deeper infarction.

3. This 32 year old overweight woman complains of several

months of headaches, nausea, and now double vision. What
cranial nerve lesion do you see in this drawing. What other
findings might you expect on fundus exam and what other tests
might you get?

This looks like a bilateral abducens palsy as the patient can’t move
either eye laterally. While the majority of abducens palsies occur
secondary to ischemic events in diabetics and hypertensives, this
etiology seems unlikely in a young patient with bilateral involvement.
Her symptoms sound suspicious for pseudotumor (obese,
headaches). You should look for papilledema of the optic nerve, get
imaging, and possibly send her to neurology for a lumbar puncture
with opening pressure.

4. A patient is sent to your neurology clinic with a complaint of

double vision. Other than trace cataract changes, the exam
seems remarkably normal with good extraocular muscle
movement. On covering the left eye with your hand, the
doubling remains in the right eye. What do you think is causing
this diplopia?
The first question you must answer with any case of diplopia is
whether the doubling is monocular or binocular. This patient has a
monocular diplopia. After grumbling to yourself about this
inappropriate neurology referral, you should look for refractive
problems in the tear film, cornea, lens, etc..

5. A patient complains of intermittent double vision that seems

to be worse in the evenings. On exam you find a confusing
diplopia that doesn’t seem to map out to any particular nerve
palsy. What else is on your differential as a cause, and what
tests might you perform in the office?
Myasthenia gravis and thyroid orbitopathy are both great
masqueraders that cause diplopia. Graves patients often have lid
retraction and reduced upgaze from inferior rectus muscle
restriction. The double vision in myasthenia patients can look like an
isolated nerve palsy, a mixture of nerve involvement, or may not fall
into any specific nerve combination – a changing palsy is more
indicative of a process like MG. You can check for fatiguable ptosis
by prolonged upgaze (hold your arm up and see who gets tired first).
In addition, you can perform a cold-pack rest test or even a Tensilon
test.
6. You are giving a tensilon test to a suspected myasthenia
gravis patient and he collapses. What do you do?
Your patient may have a reaction to the anticholinersterase such as
bradycardia or asystole. You should have a crash-cart handy and
administer atropine. Hopefully, this scenario never happens to you. In
this day and age, few ophthalmologists perform the tensilon test,
reserving this for neurology (who more often perform EMG studies).

7. A patient with diplopia is finally diagnosed with myasthenia

gravis after a positive ice-pack test and a positive acetylcholine
receptor antibody test. What else should you work up this
patient for.
You should check for a thymoma, which is highly associated with
MG. Also, check their thyroid level as 20% of myasthenia patients
also have Grave’s disease.

8. A 26 year old woman presents with decreased vision in her

left eye that has gotten progressively worse over the past week.
The eye seems to ache and the vision worsens with exercise. On
exam she is found to have 20/200 vision, trace APD, and
markedly decreased color vision in the affected eye. The optic
nerve is mildly swollen on that side. What does this patient most
likely have?
This patients age, color vision, and progression are all classic
symptoms of optic neuritis. She also describes the classic Uthoff
phenomenon of worsening symptoms with body-temperature
(exercise or shower). Many of these patients describe minor pain
with eye-movement: the optic nerve is inflamed and any tugging on
the nerve with eye movement is going to irritate it.

9. A patient develops optic neuritis. Should you treat with

steroids? Would you start with IV or oral steroids? Will the MRI
findings of numerous demyelinating lesions change your
management? Do you tell the patient that she will develop MS?
The ONTT study has shown that steroids can speed recovery from
optic neuritis, but have little effect on long-term visual outcome.
Surprisingly, the study also showed that oral prednisone may actually
increase reoccurrence of optic neuritis. Therefore, you give IV Solu-
Medrol and don’t give oral prednisone!
The presence of optic neuritis does not necessarily mean the patient
will develop multiple sclerosis, especially in the setting of a negative
MRI. The patient’s long-term risk for developing multiple sclerosis
depends upon the number of CNS lesions found on presentation. If
there are no CNS lesions, then the future risk is only about 15%. This
jumps up to 50% or more with 3+ lesions. In these higher-risk
patients, you should get neurology involved to discuss more
aggressive treatment with Avonex.

10. An 84-year-old man was out golfing with his buddies and
developed sudden vision loss in his right eye. He has no past
ocular history, no medical problems. No complaints of flashes or
floaters, just that things “look dimmer” in his right eye. What
other questions should you ask about his symptoms?
There are many questions you should ask … but with any elderly
person with vision loss, be sure to ask about the symptoms of
temporal arteritis. Specifically, scalp tenderness, jaw claudication,
and polymyalgias (muscle aches in the shoulders and arms). This
sounds like a central retinal artery occlusion, and in a patient this old
you need to rule out life- and vision-threatening causes like GCA.

11. The patient admits to “not feeling good” and “it hurts my
head to brush my hair on the right side” for the past week but
denies all other symptoms. Should you order any labs? Start any
medications?
If you have any suspicion for GCA, you pretty much have to order a
ESR and CRP. Start oral prednisone (about 1mg/kg/day) immediately
and set up for temporal artery biopsy within a week or so. Steroids
won’t help much with his lost vision in these cases, but decreases
the risk to the other eye, which can be affected within hours to days.

12. A young man complains of complete vision loss (no light

perception) in one eye, however, he has no afferent pupil defect.
Is this possible? How might you check whether this patient is
“faking it?”

Assuming the rest of the eye exam is normal (i.e. the eye isn’t filled
with blood or other media opacity) this patient should have an
afferent pupil defect if he can’t see light. There are many tests to
check for malingering: you can try eliciting a reflexive blink by
moving your fingers near the eye. One of my favorite techniques is to
hold a mirror in front of the eye. A seeing eye will fixate on an object
in the mirror. Gentle rocking movements of the mirror will result in a
synchronous ocular movement as the eye unconsciously tracks the
object in the mirror.