AAO Neuro

AAO Neuro-Ophthalmology/Orbit 01 Short
Total Questions: 7 (7 Points)

Correctly Answered Questions: 0 (0 Points)
Percentage Score 0%
Questions
Question: Which of the following is least consistent with toxic-nutritional optic neuropathy? (1 Points)
Answer:
A. A low serum B12 and thiamine (0 Points)

B. Temporal pallor of the optic discs (0 Points)
C. A 1.2 log unit relative afferent pupillary defect (RAPD) (1 Points)
Answer Feedback:
Toxic-nutritional optic neuropathy is a symmetric process whose clinical profile consists of
central or cecocentral visual field defects, matopsia, and often focal temporal pallor with loss
of the nerve fiber layer in the papillomacular bundle. Because it is a symmetric process,
significant RAPD will not occur. This neuropathy may be caused by agents such as methanol,
ethambutol, rifampin, chloramphenicol, and deficiencies in vitamins such as B12, folate, and
thiamine. Hydroxychloroquine sulfate (Plaquenil) causes a maculopathy that may give
symmetric central scotomas and cause color vision loss but does not cause an optic
neuropathy.
D. Bilateral cecocentral scotomas (0 Points)
Your answer: (0 Points)
Question: Which of the following exogenous agents/drugs is not a typical cause of toxic-nutritional
optic neuropathy? (1 Points)
Answer:
A. Methanol (0 Points)
B. Hydroxychloroquine sulfate (1 Points)
Answer Feedback:
Toxic-nutritional optic neuropathy is a symmetric process whose clinical profile consists of
central or cecocentral visual field defects, matopsia, and often focal temporal pallor with loss
of the nerve fiber layer in the papillomacular bundle. Because it is a symmetric process,
significant RAPD will not occur. This neuropathy may be caused by agents such as methanol,
ethambutol, rifampin, chloramphenicol, and deficiencies in vitamins such as B12, folate, and
thiamine. Hydroxychloroquine sulfate (Plaquenil) causes a maculopathy that may give
symmetric central scotomas and cause color vision loss but does not cause an optic
neuropathy.
C. Ethambutol (0 Points)
D. Chloramphenicol (0 Points)
Question: A 63-year-old right-handed man has an isolated infarct of the entire left parietal lobe. Which
of the following would you least expect to find on examination? (1 Points)
Answer:
A. Impaired optokinetic nystagmus with the tape going to the left (0 Points)
B. Acalculia (0 Points)
C. Right-left confusion (0 Points)
D. A right homonymous hemianopsia, worse in the superior hemifield than in the inferior
hemifield (1 Points)
Answer Feedback:
Parietal lesions typically cause visual field defects that are worse in the inferior quadrants, as
opposed to temporal lobe lesions, which cause field defects that are worse above (eg, pie-in-
the-sky scotomas). Gerstmann's syndrome (ie, acalculia), right-left confusion, finger agnosia
(inability to identify one's own fingers), and agraphia, occur with lesions involving the parietal-
occipital junction in the dominant hemisphere. With a left parietal lobe lesion, optokinetic
nystagmus is impaired, because pursuit to the left is impaired.
Question: Optic nerve sheath fenestration is not a useful therapeutic intervention for which of the
following conditions? (1 Points)
Answer:
A. Traumatic optic nerve sheath hematoma (0 Points)

B. Pseudotumor cerebri (0 Points)
C. Acute anterior ischemic optic neuropathy (1 Points)
Answer Feedback:
Nerve sheath fenestration remains a very useful procedure for visual loss associated with
optic nerve head swelling or compression in all the listed choices except anterior ischemic
optic neuropathy. The Ischemic Optic Neuropathy Decompression Trial demonstrated no
benefit with this procedure for anterior ischemic optic neuropathy. With pseudotumor cerebri,
optic nerve sheath fenestration is a procedure of choice from a visual standpoint and is used
frequently along with or in addition to lumboperitoneal shunting. Traumatic optic neuropathy
with nerve sheath hematoma, especially in cases where progressive visual loss is
demonstrated, may benefit from optic nerve sheath. With cryptococcal meningitis, it is
important to determine whether the visual loss is due to swelling from increased intracranial
pressure (where the procedure is likely to be beneficial) or due to visual loss secondary to
direct cryptococcal invasion of the optic nerve vasculature with secondary ischemic
neuropathy and infarction (where the patients do not benefit from nerve sheath fenestration).
D. Papilledema with visual loss from cryptococcal meningitis (0 Points)
Question: Which of the following statements most accurately describes the role of optic nerve sheath
fenestration and lumboperitoneal shunting in pseudotumor cerebri? (1 Points)
Answer:
A. Lumboperitoneal shunting should always be performed before optic nerve sheath fenestration
in pseudotumor cerebri. (0 Points)
B. Optic nerve sheath fenestration should always be performed before lumboperitoneal shunting
in pseudotumor cerebri. (0 Points)
C. Following optic nerve sheath fenestration, low-pressure headaches are rarely a problem. (1
Points)
Answer Feedback:
With pseudotumor cerebri, optic nerve sheath fenestration will relieve papilledema in the
operated eye and, in a significant percentage of patients, in the contralateral eye. However, it
may not reliably reduce intracranial pressure, headaches, or diplopia. Thus, the degree to
which these various symptoms are active and bothersome to the patient will influence which
procedure should be performed. Optic nerve sheath fenestration does not lower intracranial
pressure enough to cause intracranial pressure headaches, but these can occur with
lumboperitoneal shunting.
D. Optic nerve sheath fenestration substantially lowers intracranial pressure in pseudotumor

cerebri. (0 Points)
Question: Duane syndrome is associated with a genesis of which of the following structures? (1
Points)
Answer:
A. Medial longitudinal fasciculus (0 Points)

B. Cavernous sinus (0 Points)
C. Sixth nerve nucleus (1 Points)
Answer Feedback:
In the rare cases where tissue has been obtained, the problem appears to arise from an
agenesis of the sixth nerve nucleus, with misdirected innervation from the third nerve.
D. Third nerve nucleus (0 Points)
Question: A 40-year-old taxi driver complains of loss of vision in his right eye following a whiplash
injury. On examination, vision is NLP OD and 20/15 OS. External and slit-lamp examination are normal.
Extraocular movements are full. Pupils are equally reactive without relative afferent defect, and the
remainder of the examination is normal. What is the most appropriate test for this patient? (1 Points)
Answer:
A. Stereo acuity testing (1 Points)
Answer Feedback:
This patient has nonorganic visual loss. Profound subjective monocular visual impairment in
the presence of normally reacting pupils bilaterally and no relative afferent pupillary defect,
with normal vision in the contralateral eye, all suggest that the right eye is normal. Thus, there
is no reason to do MRI, a fluorescein angiogram, or a spinal tap. The stereo acuity testing will
potentially give information about the visual acuity in the reportedly affected eye.
B. A lumbar puncture (0 Points)

C. MRI (0 Points)
D. A fluorescein angiogram (0 Points)
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Questions
Question: A 40-year-old taxi driver complains of loss of vision in his right eye following a whiplash
injury. On examination, vision is NLP OD and 20/15 OS. External and slit-lamp examination are normal.
Extraocular movements are full. Pupils are equally reactive without relative afferent defect, and the
remainder of the examination is normal. Which of the following clinical tests would not be helpful for the
evaluation of the patient? (1 Points)
Answer:
A. Testing with neutral density filters (1 Points)
Answer Feedback:
This patient has nonorganic visual loss. Profound subjective monocular visual impairment in
the presence of normally reacting pupils bilaterally and no relative afferent pupillary defect,
with normal vision in the contralateral eye, all suggest that the right eye is normal. Thus,
fogging, OKN testing, and color vision testing with red-green glasses (green in front of the
good eye) will all potentially give information about the visual acuity in the reportedly affected
eye. Neutral density filters are useful for quantifying relative afferent pupillary defects;
however, as this patient has no such defect, the filters would not add anything diagnostically.
B. Color vision testing with red-green glasses (0 Points)

C. Monocular optokinetic nystagmus (OKN) testing (0 Points)
D. Refraction with fogging of the left eye (0 Points)
Question: A 59-year-old man complains of decreased vision immediately upon emerging from
anesthesia following cardiac bypass surgery. On examination, the patient reports bare light perception
vision OU. External examination, extraocular movements, pupillary reaction, and examination are all
normal. Which of the following is the most likely cause of the visual loss? (1 Points)
Answer:
A. Pituitary apoplexy (0 Points)

B. Bilateral occipital infarcts (1 Points)
Answer Feedback:
The two most common causes of visual loss following bypass surgery are occipital infarction
and ischemic optic neuropathy, with the former being much more common. With bilateral
anterior ischemic optic neuropathy (AION) and pituitary apoplexy, both of which affect the
anterior visual pathway, such severe visual loss would cause poorly reactive pupils. In
addition, with AION, on ophthalmoscopic examination the optic nerve heads would appear
swollen. With pituitary apoplexy, there may also be complaints of headache and limitation of
extraocular movements due to involvement of the cranial nerves in the cavernous sinus. To
consider a functional or nonorganic visual loss, you would need to demonstrate that the vision
is actually better than reported. For example, you would need to demonstrate optokinetic
nystagmus response or see the patient perform purposeful, visually directed tasks. In this
patient, occipital infarction (bilaterality should be assumed because of the decreased visual
acuity), should be presumed.
C. Bilateral anterior ischemic optic neuropathy (0 Points)

D. Functional or nonorganic visual loss (0 Points)
Question: A 59-year-old man complains of decreased vision immediately upon emerging from
anesthesia following cardiac bypass surgery. On examination, the patient reports bare light perception
vision OU. External examination, extraocular movements, pupillary reaction, and examination are all
normal. Which of the following is the diagnostic procedure of choice? (1 Points)
Answer:
A. Erythrocyte sedimentation rate (0 Points)

B. MRI or CT scan (1 Points)
Answer Feedback:
MRI is the procedure of choice with occipital infarction. However, patients may not be
medically stable enough to undergo this, in which case CT should be performed. Fluorescein
angiography would not likely be helpful with normal optic nerves and fundi. Visual loss in
patients with occipital infarction is usually due to hypoperfusion of the occipital lobes during
the bypass procedure, which involves the vertebrobasilar system; thus a carotid duplex study
is not indicated. Patients poststernotomy and post-bypass surgery have high erythrocyte
sedimentation rates. Therefore, this test would be too unreliable and potentially misleading
regarding the possibility of temporal arteritis as the cause, and is not indicated. This clinical
scenario could also be seen with bilateral posterior ischemic optic neuropathy, which has also
been associated with cardiac surgery, especially if there is prolonged systemic hypotension,
anemia, or both. However, in this case, pupillary reactions would be sluggish, rather than
normal, as presented in the case scenario.
C. Carotid duplex study (0 Points)

D. Fluorescein angiogram (0 Points)
Question: A 32-year-old woman presents with a 3-4 month history of intermittent diplopia. She also
notes some irritation and pressure around her eyes. Acuity, color plates, and visual fields are all
normal. Externally, the right upper eyelid is at the upper limbus. The left upper eyelid covers the upper
2 mm of the cornea. There is slight injection OU with some chemosis on the left. Versions are normal,
but cover testing demonstrates a small left hypertropia on up gaze. Corneal sensation is normal. Which
of the following is the most appropriate next test? (1 Points)
Answer:
A. MRI of the brain (0 Points)

B. FTA-ABS (0 Points)
C. Thyroid function test (1 Points)
Answer Feedback:
This patient has the classic symptoms and soft tissue signs of dysthyroid orbitopathy. In the
early stages, it may present with intermittent diplopia, generally worse in the morning,
secondary to orbital congestion. The eyelid findings suggest subtle retraction OD because the
OS position is normal, as described, not ptotic. Myasthenia gravis, although frequently
presenting with intermittent diplopia, would not have all these subjective complaints
(pressure, irritation) or the lid retraction. However, the possibility of coexisting myasthenia
gravis and Graves' disease should at least be considered. Pituitary tumors without visual field
loss wouldn't be expected to cause intermittent diplopia, especially with this morning
predominance, nor would it cause injection and chemosis. A cavernous sinus syndrome may
show injection and chemosis (although it may also show a perfectly white eye), but it wouldn't
show the lid retraction or the diurnal variation. A cavernous sinus syndrome might also have
more motility disturbance, as well as CN V1 sensory complaints. The majority of patients with
dysthyroid orbitopathy will be hyperthyroid on presentation, although they may be hypothyroid
or euthyroid. Thyroid function tests are indicated to evaluate the patient's overall status, in
addition to trying to confirm the diagnosis. A suggested screening panel would include
sensitive TSH, total T, and thyroid-stimulating immunoglobulins. Acetylcholine receptor
antibodies might be indicated if myasthenia gravis were the most likely choice. If an imaging
study were to be undertaken, it should be an orbital study (CT or MRI) rather than a routine
brain study, as listed. As this does not appear to be a true cranial neuropathy or a discrete
inflammatory lesion, FTA-ABS would not be indicated.
D. Acetylcholine receptor antibody test (0 Points)
Question: A 7-year-old child is seen with unilateral ptosis, mydriasis, and underaction of adduction
and vertical movement. Every 2 minutes, the eye adducts, with the lid elevating and the pupil
constricting. The mother notes this has been present since birth. There are no other medical or
neurologic problems. What are these signs representative of? (1 Points)
Answer:
A. Cyclic oculomotor palsy (1 Points)
Answer Feedback:
Cyclic oculomotor palsy is usually present from birth and has a classic repetitive pattern,
generally lasting about 45 seconds, where a cycle of third nerve actions (lid elevation,
adduction, pupillary constriction) is superimposed on what appears to be a third nerve palsy.
Cycloplegic migraine, although generally appearing in childhood, occurs with relatively
infrequent, episodic attacks, as with migraine in general. During an attack, a third nerve palsy
may occur, which may persist; however, when it resolves, functions generally return to
normal. Periodic alternating nystagmus demonstrates a jerk nystagmus that beats in one
direction, slows, and then reverses direction. There is no evidence of cranial nerve palsy with
this condition. Primary aberrant regeneration is the term used for aberrant regeneration
without a history of prior third nerve palsy. This is generally seen with cavernous sinus lesions
such as memingioma or intracavernous carotid aneurysms. It does not demonstrate the
intrinsic rhythmic action seen in our patient.
B. Primary aberrant regeneration (0 Points)

C. Ophthalmoplegic migraine (0 Points)
D. Periodic alternating nystagmus (0 Points)
Question: A 34-year-old man presents with acute onset of right facial pain, right ptosis, and diplopia.
He also complains of a funny feeling over the right side of his face. On examination, visual acuity is
20/15 OU with full color plates and normal visual fields. Motility examination shows marked underaction
of abduction and upgaze with slow saccades, and absent downgaze and adduction of the right eye. No
torsional movement is seen. The left eye movement is full. Exophthalmometry measurements are 18
mm on the right and 16 mm on the left. What would the most appropriate test be? (1 Points)
Answer:
A. CT of orbits, with contrast (0 Points)

B. An edrophonium (Tensilon) test (0 Points)
C. MRI of the head, with gadolinium (1 Points)
Answer Feedback:
The funny feeling over the right side of the face (sensory trigeminal nerves CN V2 to CN V3)
puts the lesion posterior to the orbit. The involvement of cranial nerves III, IV, V, and VI points
to the cavernous sinus. Thus, MRI of the head with attention to the cavernous sinus is
indicated, rather than CT of the orbits. Posterior communication artery aneurysms may cause
pain and a third nerve palsy, but wouldn't involve the fourth, fifth, and sixth cranial nerves;
thus, a cerebral angiogram would not be immediately indicated. An intracavernous carotid
artery aneurysm might have these findings, but would probably be seen on MRI, which should
be done first. A Tensilon test to rule out myasthenia gravis is clearly not indicated, as
myasthenia gravis does not cause pain or lead to sensory complaints.
D. Cerebral angiogram (0 Points)
Question: A 34-year-old man presents with acute onset of right facial pain, right ptosis, and diplopia.
He also complains of a funny feeling over the right side of his face. On examination, visual acuity is
20/15 OU with full color plates and normal visual fields. Motility examination shows marked underaction
of abduction and upgaze with slow saccades, and absent downgaze and adduction of the right eye. No
torsional movement is seen. The left eye movement is full. Exophthalmometry measurements are 18
mm on the right and 16 mm on the left. Assuming radiologic, serologic, and pharmacologic tests are
negative, what is the most likely diagnosis? (1 Points)
Answer:
A. Chronic progressive external ophthalmoplegia (0 Points)

B. Tolosa-Hunt syndrome (1 Points)
Answer Feedback:
Tolosa-Hunt syndrome is the eponym for an idiopathic cavernous sinus inflammatory
syndrome. It is probably a posterior variation of orbital pseudotumor. Tolosa-Hunt is often
described as exquisitely responsive, although it is the pain that responds quickly. The motility
may take weeks or months to improve. An occult tumor might be considered, but a lesion
large enough to involve cranial nerves III-VI completely would be visualized on an imaging
study and would not present so acutely. It would be unlikely for a carcinomatous meningitis to
involve so many contiguous unilateral cranial nerves. A remote effect of malignancy may
cause brain stem findings, not discrete cranial nerve palsies. Dysthyroid orbitopathy may
cause some discomfort, but would not present with pain. It would not have the associated
right facial dysesthesia, especially involving the CN V2 and CN V3 distribution. Chronic
progressive external ophthalmoplegia is a painless, bilaterally symmetric motility problem
without sensory involvement.
C. Occult tumor (0 Points)

D. Dysthyroid orbitopathy (0 Points)
Question: A 68-year-old man complains of severe, brief, episodic, electric shock-like pain on the left
side of his face, mostly over the cheek. It may be precipitated by chewing or shaving. He is otherwise
asymptomatic. Ophthalmologic and neurologic evaluation are normal. What is the most likely
diagnosis? (1 Points)
Answer:
A. Temporal arteritis (0 Points)

B. Postherpetic neuralgia (0 Points)
C. Trigeminal neuralgia (1 Points)
Answer Feedback:
Based on the triggering factors and the nature of the pain, this is a classic presentation of
trigeminal neuralgia. In a younger age group, consideration should be given to a structural
lesion or even demyelinating disease as a cause of trigeminal neuralgia. Temporal arteritis
must be considered as well in this patient and a determination made as to whether the inciting
factors could perhaps represent skin tenderness or jaw claudication. Temporal arteritis,
however, causes headaches, and sometimes systemic symptoms (fever, anorexia,
polymyalgia). Also, the elicited pain would not be so brief or resemble electric shock.
Although cavernous sinus aneurysms may also cause severe pain, the pain is more
persistent and less likely to have inciting factors. In addition, there are no other signs
suggesting a cavernous sinus problem. Postherpetic neuralgia is excluded, as no history of
zoster is given and no skin changes are noted. The pain of postherpetic neuralgia is also
more continuous and deeper in nature and may be associated with numbness.
D. Cavernous sinus aneurysm (0 Points)
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Questions
Question: A 15-year-old girl experiences blurry vision OS and a frontal headache. Her acuities are
20/20 OD and 20/60 without correction OS. With -2.00 D refraction she sees 20/20 OS. In the light,
pupils measure 6 mm OD and 1 mm OS, and in the dark they measure 7 mm OD and 1 mm OS. The
right pupil is briskly reactive; the left does not react to light or near stimuli. Her examination is otherwise
unremarkable. What type of drop did she use? (1 Points)
Answer:
A. Prostaglandin inhibitors (0 Points)

B. Sympathomimetic (0 Points)
C. Alpha adrenergic agonist (0 Points)
D. Parasympathomimetic (1 Points)
Answer Feedback:
A sympathomimetic drop would dilate the pupil, not constrict the pupil. It would not produce a
myopic shift, and the pupil would remain reactive to light and near stimuli. A prostaglandin
inhibitor would have no effect on the pupil, and an alpha adrenergic agent would have the
same effect as a sympathomimetic. Only a parasympathomimetic drop would constrict the
pupil and induce the myopic shift.
Question: Which of the following features describes the pharmacologic effects of pilocarpine? (1
Points)
Answer:
A. Hyperopic shift (0 Points)

B. Sectoral sphincter palsies (0 Points)
C. A small pupil that does not react to light, but does react to near stimulus. (0 Points)
D. A small pupil that does not react to light or near stimulus (1 Points)
Answer Feedback:
Pharmacologic iris blockade with pilocarpine, which is a parasympathomimetic, would
constrict the pupil and produce poor or no reaction to light or near stimuli. A small pupil that is
unreactive to light but reactive to near is a sign of the Adies tonic pupil (i.e., the little old
Adies pupil). Sectoral palsies of the iris are most consistent with Adie's pupil. A myopic rather
than hyperopic shift occurs with pilocarpine use.
Question: A patient's granddaughter incidentally noted that his pupils were unequal. The patient
denies ptosis or diplopia. In bright light, his pupils are 1 mm OD and 3 mm OS. In dim light, his pupils
are 1 mm OD and 4 mm OS. The right pupil is unreactive to light or near stimulus. Which of the
following best explains the clinical findings? (1 Points)
Answer:
A. History of blindness OD secondary to optic neuropathy (0 Points)

B. History of syphilis as a young man (0 Points)
C. History of recent chiropractic manipulation (0 Points)
D. History of prior, treated unilateral glaucoma (1 Points)
Answer Feedback:
Treatment of unilateral glaucoma with pilocarpine will produce a small pupil that is poorly
reactive to light or near. Chiropractic manipulation associated with anisocoria is typically due
to a Horner syndrome from a carotid artery dissection and would produce a small pupil that is
reactive to light but has poor dilation and a greater degree of anisocoria in the dark. Syphilis
can produce a tonic pupil or the Argyll-Robertson pupil but both produce light near
dissociation of the pupil response. A history of blindness secondary to an optic neuropathy
would produce a poor light reaction but the pupil would retain the near reaction if there was
afferent disease only.
Question: A 20-year-old ophthalmology clinic nurse complains of right-sided brow ache. Examination
of both eyes is normal except for the presence of a 1.5 mm, nonreactive right pupil. The pupil does not
dilate in darkness. The left pupil is 4 mm and briskly reactive. She has no ptosis and normal ocular
motility. The anterior chamber has no cell and flare. What is the most likely etiology? (1 Points)
Answer:
A. Uveitis (0 Points)
B. Horner's syndrome in the right eye (0 Points)
C. Exposure to an anticholinergic (atropine-like) substance in the right eye (0 Points)
D. Exposure to a cholinergic (pilocarpine-like) substance in the right eye (1 Points)
Answer Feedback:
A cholinergic exposure will prduce a pupil that is miotic and nonreactive to light or near.
Atropine or any anticholinergic substance will produce a mydriatic pupil, not a miotic pupil.
The Horner syndrome can produce a small pupil, but this pupil will still respond to light and
near stimuli. Uveitis can produce a small and poorly reactive pupil, but the absenece of cell
and flare make this less likely, although this is a potentially correct answer.
Question: A 20-year-old ophthalmology clinic nurse complains of right-sided brow ache. Examination
of both eyes is normal, except for the presence of a 1.5 mm pupil that does not react to light or near
and that does not dilate in darkness. The left pupil is 4 mm and briskly reactive. The patient has no
ptosis and normal ocular motility. The anterior chamber has no cell or flare. What is the most likely
diagnosis? (1 Points)
Answer:
A. Argyll Robertson pupil (0 Points)
B. Instillation of miotic agent (1 Points)
Answer Feedback:
A pupil that is poorly reactive or nonreactive to light or near stimuli and does not dilate
suggests iris damage or pharmacologic miosis with a topical parasympathomimetic. A Horner
syndrome produces a small pupil that remains reactive to light and near. The Adies tonic
pupil will react poorly or not at all to light, but will have a better and tonic near response. The
Argyll Robertson pupil is a bilateral, small, irregular pupil with a poor light reaction, but a
better near reaction (light near dissociation of the pupils).
C. Adie's pupil (0 Points)

D. Horner's syndrome (0 Points)
Question: An obese 26-year-old woman with a 6-month history of pseudotumor cerebri who is on
acetazolamide (Diamox) 500 mg po qid, notes progressive blurring of vision OS over the past few
weeks. She denies headaches or double vision. Examination shows chronic papilledema. Visual fields
are stable on the right and show progressive constriction on the left. Which of the following
interventions is most desirable at this time? (1 Points)
Answer:
A. Optic nerve sheath fenestration OS (1 Points)
Answer Feedback:
Although treatment of choice remains controversial, most ophthalmologists would agree that
optic nerve sheath fenestration should be recommended in this setting. The patient has
progressive visual problems only in one eye. Repeat MRI scan could be performed, but it is
not the first choice here. Because spinal fluid is completely replenished over 8 hours, repeat
spinal taps are not indicated. Weight loss should be encouraged, but in the face of
progressive visual loss, a more definitive intervention, optic nerve sheath fenestration, is
indicated.
B. repeat MRI scan (0 Points)

C. Weight reduction (0 Points)
D. Repeat lumbar punctures (0 Points)
Question: A 64-year-old hypertensive patient complains of difficulty reading. He states, Things are
blurry, I sometimes lose my place when reading, and I still see things after I've looked away.
Examination demonstrates acuity of 20/20 OU with normal motility, pupillary, and fundus evaluation.
What would the most appropriate initial evaluation be? (1 Points)
Answer:
A. Fluorescein angiogram (0 Points)

B. Erythrocyte sedimentation rate (0 Points)
C. Psychiatric consultation (0 Points)
D. Visual field test (1 Points)
Answer Feedback:
Any patient with persistent visual complaints despite a normal routine eye examination should
have visual field testing. The symptom of losing his place while reading should suggest a left
hemianopia. The complaint of still seeing things after looking away represents palinopsia, a
visual release phenomenon seen with hemianopias, most commonly right parietal lesions.
The likelihood of temporal arteritis causing visual loss is unlikely with a normal fundus.
Similarly, fluorescein angiography in the setting of a normal funduscopic examination would
not be the first test recommended. A psychiatric consultation is not indicated unless a
nonorganic visual loss is demonstrated. The other field defects would not cause the
constellation of visual symptoms described.
Question: A 32-year-old female reports that for the last few months, she has noted intermittent
binocular vertical diplopia on awakening. This symptom never lasts more than 30 minutes. She also
notes some irritation and pressure around her eyes. Acuity, color plates, and visual fields are all
normal. Externally, the right upper eyelid is at the upper limbus. The left covers the upper 2 mm of the
cornea. There is slight injection OU with some chemosis on the left. Versions are normal, but cover
testing demonstrates a small left hypertropia (or right hypotropia) on up gaze. Corneal sensation is
normal. What is the most likely diagnosis at this time? (1 Points)
Answer:
A. Pituitary tumor (0 Points)

B. Cavernous sinus syndrome (0 Points)
C. Myasthenia gravis (0 Points)
D. Dysthyroid orbitopathy (1 Points)
Answer Feedback:
This patient has the classic symptoms and soft tissue signs of dysthyroid orbitopathy. In the
early stages, it may present with intermittent diplopia, generally worse in the morning,
secondary to orbital congestion. The eyelid findings suggest subtle retraction OD because the
OS position is normal, as described, not ptotic. Myasthenia gravis, although frequently
presenting with intermittent diplopia, would not have all these subjective complaints
(pressure, irritation) or the lid retraction. However, the possibility of coexisting myasthenia
gravis and Graves' disease should at least be considered. Pituitary tumors without visual field
loss wouldn't be expected to cause intermittent diplopia, especially with this morning
predominance, nor would it cause injection and chemosis.
Question: A 1-year-old child is referred for evaluation of jiggling eyes. The mother notes that this
condition has been present since about 1month of age. On examination, the child fixes and follows well
with either eye. The anterior segment is normal. Retinoscopy is +2.50+0.50 x 90 degrees OD;
+2.75+0.25 x 75 degrees OS. Dilated fundus examination is normal. Extraocular movements are full.
You note binocular horizontal pendular nystagmus that diminishes on right gaze and when the patient
fixates at near. General medical and neurologic evaluation is reportedly normal. What is the most likely
finding on examination? (1 Points)
Answer:
A. Light-near dissociation on pupillary testing (0 Points)

B. An abnormal fundus on subsequent evaluations (0 Points)
C. Normal vertical optokinetic nystagmus response (1 Points)
Answer Feedback:
This child has motor congenital nystagmus demonstrated by a null point and damping at near.
The condition is not always appreciated right at birth. On testing, horizontal optokinetic
nystagmus (OKN) responses may or may not be elicited or may show inversion; that is,
instead of the recovery saccade (the jerk phase of nystagmus) being in the expected
opposite direction to the drum or OKN tape rotation, it is in the same direction. Vertical
saccades, however, are generally preserved, which yields a somewhat oblique nystagmus
when the OKN response is generated, as vertically induced saccades are superimposed on
the horizontal baseline nystagmus. Convergence retraction nystagmus is seen with the dorsal
midbrain syndrome. One would not expect to see a primary horizontal nystagmus with a null
point in this entity. Normal motility, especially vertical, also argues against a dorsal midbrain
syndrome. No other ocular abnormalities were noted. Light-near dissociation may be seen as
part of the dorsal midbrain syndrome, but this is also seen when there is damage to the
afferent visual system. There is no evidence of either optic nerve or retinal disease, and the
child does fix and follow well. The normal anterior segment and retina excludes albinism. A
patient with Leber 's congenital amaurosis may have a normal fundus on early evaluation
although abnormality evolves over time. In addition, one does not get a null point and
damping at near with Leber's.
D. Convergence retraction nystagmus on upgaze (0 Points)
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Questions
Question: A 62-year-old woman presents complaining that everything is jumping. Visual acuity, color
vision, and visual fields are normal. Motility evaluation shows full movement. You note a coarse,
downward beating of both eyes, worse on downgaze and worse in lateral gaze. What would this sign
most likely represent? (1 Points)
Answer:
A. Periodic alternating nystagmus (0 Points)

B. Downbeat nystagmus (1 Points)
Answer Feedback:
Downbeat nystagmus is a bilateral, downward beating nystagmus, classically worse on down
gaze and lateral gaze downward. Superior oblique myokymia is unilateral, usually with very
fine oscillations, and worsens into the field of the superior oblique muscle (down and in).
Periodic alterating nystagmus is a horizontal jerk nystagmus that beats in one direction,
slows, stops, and then reverses direction. Myasthenia gravis rarely presents as nystagmus.
C. Superior oblique myokymia (0 Points)

D. Myasthenia gravis (0 Points)
Question: A 70-year-old woman reports double vision and trouble with my eyes. This apparently
developed suddenly, a few months ago. Visual acuity, color vision, and threshold visual field evaluation
are normal. Extraocular movements show no adduction OD and no abduction or adduction OS; motility
is otherwise normal. Pupillary evaluation is normal. There is no ptosis. External examination is
unremarkable. Which of the following tests is most indicated? (1 Points)
Answer:
A. Aan edrophonium (Tensilon) test (0 Points)
B. MRI of the parasellar area (0 Points)
C. CT of the orbit (0 Points)
D. MRI of the head/pons (1 Points)
Answer Feedback:
This motility pattern describes a one-and-a-half syndrome, which is a gaze palsy (the one)
with a contralateral internuclear ophthalmoplegia (the half). It represents a lesion in the
pons, most commonly with a demyelinating cause in younger patients and an ischemic cause
in older patients, such as this one. MRI to determine the cause is indicated in this patient with
no pertinent history. Myasthenia gravis can mimic any eye movement disorder, although it
would be unusual to have such a complete one-and-a-half syndrome without any other
extraocular muscle or levator involvement. Myasthenia would also not be expected to arise so
suddenly. Thus, a Tensilon test is not indicated. Dysthyroid orbitopathy may cause
underaction of abduction, although having this pattern without other extraocular muscle
involvement would be very unusual. The sudden onset and normal external examination also
argue against this diagnosis; as a result, orbital CT scanning is not indicated. Regarding
bilateral cavernous sinus syndrome, one might view this patient's problem as a sixth nerve
palsy with a partial, bilateral third nerve involvement. However, one does not get complete
involvement of one muscle innervated by the third nerve (medial rectus in this case) without
any involvement of either lid, pupil, or other extraocular muscles on a peripheral basis, only
centrally; thus, MRI with attention to the parasellar area would not be indicated.
Question: A 24-year-old woman complains of multiple episodes of blurred vision with flashing lights
that progress over approximately 30 minutes. These are followed by a throbbing headache and
resolution of her visual symptoms. A complete ophthalmologic evaluation, including visual fields, is
normal. What is the most likely diagnosis? (1 Points)
Answer:
A. Superior oblique myokymia (0 Points)

B. Cluster headache (0 Points)
C. Classic migraine (1 Points)
Answer Feedback:
These are the symptoms of a classic migraine, or migraine with aura. The visual symptoms
are cortical in origin, so this is not a retinal or ophthalmic migraine (both of the latter are truly
monocular events). Cluster headaches are episodes of severe pain in and around one eye
and side of the head, often associated with tearing and nasal congestion. They tend to occur
daily over a limited period of time (the cluster) and then resolve. Transient ischemic attacks
are generally more abrupt at onset, and are less likely to cause a positive phenomenon, such
as flashing lights; rather, they will present with a defect, such as a hemianopia. In addition,
they are not likely to be followed by headache or to follow such a stereotypic course. Superior
oblique myokymia may be perceived as a shimmering of vision, but it occurs monocularly,
doesn't follow a stereotypic time course, and is not associated with headaches.
D. Transient ischemic attack (0 Points)
Question: A 24-year-old woman complains of episodes of blurred vision with flashing lights that
progress over approximately 30 minutes. These are followed by a throbbing headache and resolution
of her visual symptoms. A complete ophthalmologic evaluation, including visual fields, is normal. The
most likely diagnosis was classic migraine. On a follow-up visit, the patient notes eight additional
episodes. In all cases, she reports, the blur and scintillations occurred only in the left visual field, with
headaches on the right side. She denies any other accompanying neurologic symptoms. Which of the
following is the most appropriate course of action? (1 Points)
Answer:
A. Observation only (0 Points)
B. Echocardiogram (0 Points)
C. Carotid ultrasound (0 Points)
D. MRI of the brain (1 Points)
Answer Feedback:
With multiple recurrent episodes of the same system, all on the same side, a fixed, structural
lesion, especially an arteriovenous malformation mimicking migraine, must be ruled out. MRI
is the procedure of choice. With so many episodes all in the same distribution, observation
alone would not be appropriate. The episodic nature might suggest an ischemic cause, but
the purely visual symptoms without other neurologic symptoms would suggest a posterior,
vertebrobasilar system distribution (not the carotids) as the most likely localization; thus, a
carotid ultrasound is not the first choice of test. A cardiac cause as a possible source of
emboli could be considered, but with multiple identical episodes suggesting only one site of
involvement (and no symptoms suggesting other sites of embolization) an echocardiogram
would not be considered appropriate at this time.
Question: A 22-year-old woman exhibits a dilated right pupil that you clinically suspect is an Adie's
tonic pupil. The pupils are 8 mm OD, 5 mm in bright light, and 8 mm OS, 2 mm in bright light. The
motility is normal, and there is no ptosis. While preparing the appropriate pharmacologic solutions, you
take a more detailed history. Which of the following historical points would be of the least significance
in a patient who demonstrates a large pupil that does not constrict? (1 Points)
Answer:
A. The patient has a history of motion sickness and has just returned from a cruise. (0 Points)
B. The patient is an lCU nurse who runs cardiac resuscitation codes. (0 Points)
C. The patient says that the right eye with the larger pupil has always had a lighter colored iris
than the left eye. (1 Points)
Answer Feedback:
The question is, What in the history is consistent with having an ipsilateral dilated pupil? That
the patient is an ICU nurse is relevant because during cardiac resuscitation, ampules of
atropine are used in haste, and a resuscitation team member might become subject to
atropinization by inadvertent hand-eye contact. Inability to read due to loss of ipsilateral
accommodation is a symptom of Adie's tonic pupil. The most effective pharmacologic
treatment of motion sickness is transdermal scopolamine, which may cause ipsilateral
pupillary dilation by direct inoculation from the fingers. A lighter iris in the affected eye is
insignificant for two reasons: pupils in Horner's syndrome do respond to light; furthermore,
even if this patient had a congenital Horner's syndrome, the lighter iris would be ipsilateral to
the smaller pupil.
D. The patient is aware of an inability to read with the right eye only. (0 Points)
Question: A 17-year-old boy receives an ophthalmic examination before beginning driver's education.
The results of the examination are as follows: Uncorrected acuity: 20/20 OU; Pupils: 5 mm OU, round
and reactive; mild RAPD OD; Motility: Normal; Slit lamp: Normal; Intraocular pressures: 16 mm Hg OD,
18mm Hg OS; Visual fields: Inferior altitudinal defect OD, normal OS. Which of the following points in
the history or examination, if elicited, would not aid in arriving at a diagnosis? (1 Points)
Answer:
A. A superior hemiretinal detachment is present OD. (0 Points)

B. A cilioretinal artery is present OD only. (1 Points)
Answer Feedback:
Offspring of mothers with insulin-dependent (juvenile-onset) diabetes mellitus may have optic
nerve head hypoplasia with an associated congenital altitudinal scotoma. Optic neuritis and a
hemiretinal detachment could also cause an altitudinal scotoma. The finding of a cilioretinal
artery has no bearing on the presence of the altitudinal field defect.
C. The patient had an episode of marked acute visual loss OD with pain on ocular rotation 2
years before, with partial recovery. (0 Points)
D. He has optic nerve hypoplasia OD, and his mother has insulindependent (juvenile-onset)
diabetes mellitus. (0 Points)
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Questions
Question: A 22-year-old woman with acute visual loss in the right eye and pain with eye movement
from optic neuritis due to multiple sclerosis is most likely to have which one of the following clinical
findings? (1 Points)
Answer:
A. Uveitis with vitritis (0 Points)

B. Anisocoria greater in light (0 Points)
C. Optic disc edema with macular star (0 Points)
D. Relative afferent pupillary defect (1 Points)
Answer Feedback:
The description would be most consistent with an inflammatory optic neuropathy, when
asymmetric or unilateral, is associated with a relative afferent pupillary defect. An optic
neuropathy does not cause anisocoria. Optic disc edema with a macular star (neuroretinitis)
and uveitis are not typically seen in multiple sclerosis.
Question: Eye pain with ipsilateral ptosis would be most likely to be seen in a patient with which one
of the following? (1 Points)
Answer:
A. Optic nerve sheath meningioma (0 Points)

B. Carotid dissection (1 Points)
Answer Feedback:
Thyroid eye disease is the most common cause of eyelid retraction. Orbital cavernous
hemangioma, optic nerve sheath meningioma, and other orbital disorders most commonly
cause proptosis. Carotid dissection may cause ipsilateral Horner syndrome, which is
characterized by painful ptosis.
C. Orbital cavernous hemangioma (0 Points)

D. Thyroid eye disease (0 Points)
Question: A mass causing a relative afferent pupillary defect and a homonymous hemianopia would
most likely be found in which of the following anatomic locations? (1 Points)
Answer:
A. Occipital lobe (0 Points)

B. Optic chiasm (0 Points)
C. Optic tract (1 Points)
Answer Feedback:
Occipital lobe lesions are not associated with pupillary abnormalities. Optic nerve (unilateral
visual field defect) and chiamsal problems (temporal visual field defects) do not result in a
homonymous hemianopia. Optic tract lesions cause a combination of a homonymous
hemianopia, and a relative afferent pupillary defect.
D. Optic nerve (0 Points)
Question: Which one of the following is a contraindication to magnetic resonance imaging? (1

Points)
Answer:
A. Orbital wood foreign body (0 Points)

B. Acute hemorrhage (0 Points)
C. Cochlear implant (1 Points)
Answer Feedback:
Cochlear implantation is a contraindication to MRI. Iodine allergy may be a contraindication to
CT scan dye injection. Wood foreign bodies and acute hemorrhage are not contraindications
to either type of imaging study.
D. Iodine allergy (0 Points)
Question: A 25-year-old man is found to have a normal visual field in the right eye and a complete
temporal hemianopic defect in the left eye. The visual field is repeated under binocular conditions, and
the left hemianopic visual field defect persists. These findings are most consistent with which one of the
following? (1 Points)
Answer:
A. Nonphysiologic visual loss (1 Points)

Answer Feedback:
An optic tract syndrome would cause a homonymous hemianopia. A nasal retinopathy on the
left may cause a temporal defect, however, the binocular visual field would be normal. The
normal (nasal) visual field of the right eye would still be able to compensate for any potential
temporal visual field loss of the left eye.
B. Retinopathy (0 Points)
C. Optic tract syndrome (0 Points)
D. Chiasmal syndrome (0 Points)
Question: What is the most likely diagnosis in a 48-year-old man who awakens after cardiac bypass
surgery with decreased vision in the right eye, a right relative afferent pupillary defect, and normal
funduscopy? (1 Points)
Answer:
A. Occipital stroke (0 Points)

B. Optic neuritis (0 Points)
C. Cilioretinal artery occlusion (0 Points)
D. Posterior ischemic optic neuropathy (1 Points)
Answer Feedback:
The findings suggest a right optic neuropathy. The presence of a relative afferent pupillary
defect excludes an occipital lobe lesion. Normal funduscopy excludes chorioretinopathy.
Optic neuritis could cause loss of vision with an afferent pupillary defect and a normal fundus.
However, this condition would be unlikely to occur in the setting of cardiac bypass surgery.
Posterior ischemic optic neuropathy has been described in the setting of bypass surgery as
well as other surgical procedures. In the case of both optic neuritis and posterior ischemic
optic neuropathy, disc pallor only develops several weeks after the acute event.
Question: Which of the following findings would most likely suggest a supranuclear gaze palsy in a
72-year-old man who cannot look down? (1 Points)
Answer:
A. Normal dolls head maneuver (1 Points)
Answer Feedback:
Downbeating nystamus may occur irrespective to a gaze palsy. Abduction deficits do not
reveal whether the cause is nuclear or suparnuclear in origin. Light-near dissociation
suggests a problem in the midbrain. A normal dolls head maneuver suggests the nuclear and
peripheral pathways for eye movement are functional.
B. Downbeating nystagmus (0 Points)

C. Pupillary light-near dissociation (0 Points)
D. Bilateral abduction deficits (0 Points)
Question: A 42-year-old woman has anisocoria that is greater in light. The right pupil is sluggishly
reactive to light and she has normal ocular motility, alignment, and normal eyelid position. What would
be the best next step? (1 Points)
Answer:
A. MRI/MRA of the brain (0 Points)
B. Carotid ultrasonography (0 Points)
C. Slit-lamp biomicroscopy (1 Points)
Answer Feedback:
The findings suggest a problem with the pupillary sphincter (greater anisocoria in light).
Normal lid position, alignment, and eyelid position make third nerve palsy unlikely. Cocaine is
useful in the diagnosis of Horner syndrome. Slit-lamp biomicroscopy would be the most
helpful to distinguish abnormalities in the iris that might suggest a tonic pupil or trauma as the
cause of anisocoria.
D. 10% cocaine eye drops (0 Points)
Question: Which of the following disorders is almost always bilateral? (1 Points)

Answer:
A. Essential blepharospasm (1 Points)
Answer Feedback:
Hemifacial spasm is characteristically unilateral and persists during sleep. Idiopathic facial
palsy and eyelid myokymia are most commonly unilateral. Essential blepharospasm is
bilateral and abolished by sleep.
B. Eyelid myokymia (0 Points)

C. Idiopathic (Bells) facial palsy (0 Points)
D. Hemifacial spasm (0 Points)
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Questions
Question: A patient involved in a motor vehicle accident had loss of consciousness for 10 minutes.
She has an abduction deficit of the right eye, with only about 10% of normal amplitude of abduction
beyond the midline remaining. The anterior examination is normal; exophthalmometry readings are
symmetric. Which of the following would be consistent with the presence of a right sixth-nerve palsy
without an entrapped medial rectus? (1 Points)
Answer:
A. Exodeviation on right gaze (0 Points)

B. Ability to induce full abduction OD with oculocephalic maneuver (0 Points)
C. Restriction of the right eye on forced abduction, no force generated with attempted abduction
on forced generation test (0 Points)
D. No restriction on forced abduction OD, diminished force generated with attempted abduction
on forced generation test (1 Points)
Answer Feedback:
In significant head trauma, it is possible to see both a neurogenic sixth-nerve palsy from the
closed head injury and medial rectus entrapment from an orbital fracture. In the setting of a
medial orbital fracture with medial rectus entrapment and without sixth-nerve dysfunction, the
forced duction would demonstrate resistance opposite to the field of action of the restricted
muscle. In a nearly complete sixth-nerve paresis, one would not be able to completely
overcome the abduction deficit with oculocephalic maneuver. Also, in an isolated acute right
sixth-nerve paresis, there would be an esodeviation in all fields of gaze. In an acute sixth-
nerve paresis without entrapment, there would be no resistance to forced aductions, but there
would be either no force or reduced force imparted to the right globe in right abduction,
because the right lateral rectus is not receiving normal neural input to induce its contraction.
Question: A 62-year-old woman presents complaining that everything is jumping. Visual acuity, color
vision, and visual fields are normal. Motility evaluation shows full movement. You note a coarse,
downward beating of both eyes, worse on downgaze, especially laterally. Which of the following actions
is most recommended? (1 Points)
Answer:
A. An edrophonium (Tensilon) test (0 Points)

B. Magnetic resonance angiography (MRA) (0 Points)
C. Observation (0 Points)
D. Magnetic resonance imaging (MRI) (1 Points)
Answer Feedback:
Downbeat nystagmus is frequently associated with problems at the cervicocomedullary
junction, such as Arnold-Chiari malformations or cerebellar atrophy. MRI with attention to this
area is indicated. Observation probably is not adequate; one should try to discern whether a
structural basis exists for the problem. MRA is indicated for vascular lesions. Because this
patient's problem generally does not arise from an aneurysm or vascular malformation, MRA
is not indicated in this case. A Tensilon test is also not indicated, as myasthenia would not
present as nystagmus. Dorsal midbrain lesions are associated with convergence retraction
nystagmus, as well as weakness of up gaze, possibly down gaze, and light-near dissociation.
There may also be abduction problems. Parasellar/third ventricle lesions are associated with
see-saw nystagmus. The medial longitudinal fasciculus is involved in internuclear
ophthalmoplegia, which clinically consists of an underaction of adduction with nystagmus in
the contralateral abducting eye.
Question: A 62-year-old woman complains that everything is jumping. Visual acuity, color vision, and
visual fields are normal. Motility evaluation shows full movement. You note a coarse, downward beating
of both eyes, worse on downgaze, especially laterally. What anatomic site is most closely associated
with this condition? (1 Points)
Answer:
A. Medial longitudinal fasiculus. (0 Points)

B. Pontomedullary junction. (0 Points)
C. Dorsal midbrain. (0 Points)
D. Cervicomedullary junction. (1 Points)
Answer Feedback:
Downbeat nystagmus is frequently associated with problems at the cervicocomedullary
junction, such as Arnold-Chiari malformations or cerebellar atrophy. MRI with attention to this
area is indicated. Observation probably is not adequate; one should try to discern whether a
structural basis exists for the problem. MRA is indicated for vascular lesions. Because this
patient's problem generally does not arise from an aneurysm or vascular malformation, MRA
is not indicated in this case. A Tensilon test is also not indicated, as myasthenia would not
present as nystagmus. Dorsal midbrain lesions are associated with convergence retraction
nystagmus, as well as weakness of up gaze, possibly down gaze, and light-near dissociation.
There may also be abduction problems. Parasellar/third ventricle lesions are associated with
see-saw nystagmus. The medial longitudinal fasciculus is involved in internuclear
ophthalmoplegia, which clinically consists of an underaction of adduction with nystagmus in
the contralateral abducting eye.
Question: A 70-year-old woman reports double vision and trouble with my eyes. These symptoms
apparently developed suddenly, a few months ago. Visual acuity, color vision, and threshold visual field
evaluation are normal. Extraocular movements show no adduction OD and no abduction or adduction
OS; motility is otherwise normal. Pupillary evaluation is normal. There is no ptosis. External
examination is unremarkable. What is the most likely diagnosis? (1 Points)
Answer:
A. Dysthyroid orbitopathy (0 Points)

B. Bilateral cavernous sinus syndrome (0 Points)
C. Pontine stroke (1 Points)
Answer Feedback:
This motility pattern describes a one-and-a-half syndrome, which is a gaze palsy (the one)
with a contralateral internuclear ophthalmoplegia (the half). It represents a lesion in the
pons, most commonly with a demyelinating cause in younger patients and an ischemic cause
in older patients, such as this one. MRI to determine the cause is indicated in this patient with
no pertinent history. Myasthenia gravis can mimic any eye movement disorder, although it
would be unusual to have such a complete one-and-a-half syndrome without any other
extraocular muscle or levator involvement. Myasthenia would also not be expected to arise so
suddenly. Thus, a Tensilon test is not indicated. Dysthyroid orbitopathy may cause
underaction of abduction, although having this pattern without other extraocular muscle
involvement would be very unusual. The sudden onset and normal external examination also
argue against this diagnosis; as a result, orbital CT scanning is not indicated. Regarding
bilateral cavernous sinus syndrome, one might view this patient's problem as a sixth nerve
palsy with a partial, bilateral third nerve involvement. However, one does not get complete
involvement of one muscle innervated by the third nerve (medial rectus in this case) without
any involvement of either lid, pupil, or other extraocular muscles on a peripheral basis, only
centrally; thus, MRI with attention to the parasellar area would not be indicated.
D. Myasthenia gravis (0 Points)
Question: A 68-year-old man complains only of recent onset of trouble reading. There is no past
medical history. The results of the patient's examination are as follows: Best-corrected acuity: distance-
20/25 OU, near-20/20 OU; Pupils: 3 mm OU, round and reactive; no RAPD; lid fissures: 10 mm OU;
Motility: Horizontal eye movement normal, volitional down gaze severely and symmetrically limited, up
gaze mildly limited. Oculocephalic maneuver completely overcomes the vertical eye movement
deficiency; Slit lamp: Normal; Intraocular pressures: Normal; Visual fields: Normal; Fundi: Normal.
Which of the following would NOT be consistent with the diagnosis of the patient? (1 Points)
Answer:
A. Dementia (0 Points)
B. Pigmentary retinopathy (1 Points)
Answer Feedback:
Progressive dementia, square wave jerks (small amplitude saccadic jerky movements away
from fixation), ataxia, and axial rigidity are all typical in the course of progressive supranuclear
palsy. Pigmentary retinopathy is a feature of the Kearns-Sayre syndrome, which is in the
differential of a progressive symmetric ophthalmoparesis, but would not have late onset and
would not be likely to respond to the oculocephalic maneuver.
C. Square wave jerks (0 Points)

D. Extensor neck rigidity (0 Points)
Question: A 335-pound 25-year-old woman comes in on a Monday for evaluation of bilateral

synchronous visual obscurations lasting 15 seconds. These have been occurring for 3 weeks. She has
no other medical history and is on no medications. Her examination is remarkable for enlarged blind
spots on visual field testing and marked bilateral papilledema. What is the best next step in her
management? (1 Points)
Answer:
A. Lumbar puncture (0 Points)

B. MRI (1 Points)
Answer Feedback:
Even in the patient whose presentation is classic for pseudotumor cerebri, two critical steps
must be taken before the diagnosis can be made. First, it is imperative to rule out the
presence of an intracranial mass, which can be a neurosurgical emergency and can certainly
occur in the obese patient. MRI is the imaging method of choice; it also serves to establish
that the pseudotumor cerebri is not secondary to dural sinus thrombosis. If there is no mass,
the second requisite for establishing the diagnosis of pseudotumor cerebri is that the spinal
tap show raised intracranial pressure with normal chemical and cellular composition of the
spinal fluid (eg, no evidence of inflammation or infection). Only after these steps are taken is it
appropriate to begin therapy for pseudotumor cerebri.
C. empiric treatment with diet and excercise (0 Points)

D. empiric treatment with acetazolamide (0 Points)
Question: Which of the following statements about Leber's hereditary optic neuropathy is true? (1
Points)
Answer:
A. Females may develop Leber's hereditary optic neuropathy. (1 Points)
Answer Feedback:
Although males are most often affected (80% to 90% of cases in Caucasians, 60% of cases
in Japanese), females are affected more often than was previously reported. Males at genetic
risk have a higher chance of developing visual loss than do females at genetic risk for Leber's
hereditary optic neuropathy (LHON). We now know that LHON is a phenotypic expression of
several different mitochondrial genetic alterations. These mitochondrial genetic sequences
can now be established, allowing for laboratory confirmation in nearly all cases. Worldwide,
the 11778 point mutation accounts for about 50% of all LHON cases. The 3460 point mutation
accounts for 8% to 25% of LHON cases. A third point mutation at locus 14484 may cause
10% of LHON. Many LHON patients have a negative family history. The interval between the
involvement of the first and the second eye is usually days to weeks, but it may extend to
months or years in some cases.
B. It is rare for affected patients to have no family history of visual loss. (0 Points)
C. It is purely a clinical diagnosis, based on the family inheritance pattern. (0 Points)
D. The onset in the two eyes is always simultaneous. (0 Points)
Question: A 45-year-old right-handed male executive who is an avid golfer presents with the
complaint that for the past year, whenever he addresses a golf ball, it appears to be shimmering up and
down, with perhaps a rotatory component to it. Sometimes the ball looks double to him in the vertical
plane. The patient, a good observer, says that the the image only oscillates when viewed with the right
eye. The results of his examination are normal. Which of the following is the most appropriate action to
take? (1 Points)
Answer:
A. Perform an edrophonium (Tensilon) test (0 Points)

B. Obtain a psychiatric consultation (0 Points)
C. Start the patient on carbamazepine (Tegretol) (1 Points)
Answer Feedback:
The patient has given a classic description of superior oblique myokymia. In this disorder,
there is an intermittent rapid oscillopsia, which is largely a cyclorotatory phenomenon, with
intorsional movements. These episodes may be triggered by moving the eye into the field of
action of the affected superior oblique muscle. When a right-handed golfer addresses a golf
ball; the eye moves into a depressed and adducted position; which can trigger these attacks.
Carbamazepine (Tegretol) seems to help most patients with this condition, although some
report that the beneficial effect is transitory.
D. Start the patient on phenytoin (Dilantin) (0 Points)
Question: A 67-year-old Asian man consulted you 3 months ago with an isolated right sixth-nerve
palsy that had been present for 3 months. His CT scan with contrast of the head, edrophonium
(Tensilon) test, glucose tolerance test, sedimentation rate and serologies for Lyme disease and syphilis
were normal. He now presents with a left sixth-nerve palsy to accompany the persistent right sixth-
nerve palsy. The results of the rest of the examination remain normal. There is no proptosis. A spinal
tap performed by his neurologist 1 day earlier was normal. What is the most likely location of the
pathologic process? (1 Points)
Answer:
A. Genu of right facial nerve around abducens nucleus (0 Points)

B. Clivus (1 Points)
Answer Feedback:
Both sixth nerves come together to ascend the clivus. The sequential involvement of both
abducens nerves should alert the ophthalmologist to consider a mass lesion in this location.
Although a process involving both orbital apices could theoretically cause bilateral sixth-nerve
palsies, proptosis and other signs of orbital involvement would be expected. A lesion of the
genu of the right facial nerve might give an ipsilateral sixth- and seventh-nerve paresis, but
not bilateral sixth-nerve palsies. Bilateral nuclear sixth-nerve palsies would give the patient
bilateral gaze paresis.
C. Both orbital apices (0 Points)

D. Bilateral abducens nucleus (0 Points)
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Questions
Question: A patient presents because of eleven migraine headaches preceeded by scintillating

scotomas. Under which of the following circumstances would neuro-imaging be indicated? (1 Points)
Answer:
A. Male gender. (0 Points)

B. Homonymous hemianopia following the aura. (1 Points)
Answer Feedback:
Migraine is not uncommon at age 17, and although migraine headaches are more common in
females, they are not unusual in males. In classic migraine, the visual phenomenon
(scintillating scotoma) is typically homonymous. Although these episodes may be rather
stereotypical, and they most often may be on one particular side of the head, always having
the symptoms on one side raises the question of a fixed lesion causing the migrainous
phenomenon, such as an arteriovenous malformation.
C. Auras are homonymous and always on the same side. (0 Points)

D. Age of 17 years. (0 Points)
Question: Which of the following syndromes with neuro-ophthalmic signs is not associated with the
endocrinopathy mentioned? (1 Points)
Answer:
A. De Morsier's syndrome/growth failure (0 Points)

B. Chiasmal sarcoidosis/Cushing's disease (1 Points)
Answer Feedback:
Due to concomitant trauma to the hypothalamic-pituitary axis, chiasmal trauma is often
associated with diabetes insipidus. Septo-optic dysplasia, or de Morsier's syndrome, consists
of agenesis of the septum pellucidum, optic nerve hypoplasia, and growth failure.
Angiomatosis retinae may have several extraocular manifestations, including
hemangioblastoma of the central nervous system; renal, pancreatic, hepatic, or epididymal
cysts; and pheochromocytoma. Sarcoidosis of the chiasm is often accompanied by diabetes
insipidus, not Cushing's disease, due to the attendant hypothalamic infiltration and
dysfunction.
C. Angiomatosis retinae (von Hippel-Lindau disease)/pheochromocytoma (0 Points)

D. Chiasmal trauma/diabetes insipidus (0 Points)
Question: A 37-year-old woman presents with ptosis and ophthalmoplegia. She first noted ptosis at
age 12, and noted trouble moving her eyes 3 years later. It has been progressive over the years; she
never had any diplopia, and she believes that her vision, once her eyelids are raised, is normal. There
is no family history. She denies other medical problems, and she has not seen a physician for 10 years.
Which of the following would not be an appropriate action to take at the initial visit? (1 Points)
Answer:
A. Schedule muscle biopsy (0 Points)

B. Send off mitochondrial genetic analysis (0 Points)
C. Perform edrophonium (Tensilon) test (1 Points)
Answer Feedback:
Besides ophthalmoplegia and retinopathy, patients with Kearns-Sayre syndrome should also
have at least one of the following other three manifestations for the clinical diagnosis to be
made: cardiac conduction abnormalities (ie, heart block), elevated cerebrospinal fluid protein,
or cerebellar dysfunction. The clinical diagnosis is supported by muscle biopsy demonstrating
ragged red fibers. Mitochondrial genetic analysis shows gene deletions in many cases,
confirming the diagnosis. Spinal fluid analysis is also useful, because elevated cerebrospinal
fluid protein is a criterion for diagnosis. Because cardiac conduction abnormalities (including
heart block, which may cause sudden death) may be a feature of Kearns-Sayre syndrome,
insertion of a pacemaker is often part of the management of these cases. Thus, edrophonium
(which may cause bradycardia) should not be given to a patient in whom Kearns-Sayre
syndrome is in the differential diagnosis until the patient has had a cardiac evaluation.
D. Schedule cardiac evaluation (0 Points)
Question: Which of the following findings would be unusual in a patient presenting for an examination
before craniotomy for a large pineal tumor? (1 Points)
Answer:
A. Skew deviation (0 Points)

B. Convergence-retraction nystagmus only on attempted saccades downward (1 Points)
Answer Feedback:
In Parinaud's syndrome (also known as dorsal midbrain syndrome, or sylvian aqueduct
syndrome) the pupils may not react to light, but will react to near fixation. These pupils tend to
be midsize or large, and of greater size than those seen with syphilis. Lid retraction (Collier's
sign) is typical, and skew deviation may be part of the findings. In this disorder, there is a
selective involvement of up gaze, and, early on, saccades may be affected more than pursuit.
Attempts at upward saccades may induce convergence-retraction nystagmus. Isolated
convergence-retraction nystagmus on attempted down gaze is not a feature of this syndrome.
C. Light-near dissociation of the pupils (0 Points)

D. Lid retraction (0 Points)
Question: Two patients present with bilateral internuclear ophthalmoplegia. One is 20-years-old, and
the other is 65. The younger patient has no other medical history, and the 65-year-old has always been
well, but recently diagnosed with hypertension. Their examinations are otherwise normal. Which of the
following statements regarding either patient would not be appropriate? (1 Points)
Answer:
A. The problem in both patients localizes to the pons. (0 Points)

B. The older patient should be treated with 1 g of methylprednisolone sodium succinate
(Solumedrol) daily for 3 days. (1 Points)
Answer Feedback:
Bilateral internuclear ophthalmoplegia localizes to the pons. In the young, a common cause is
multiple sclerosis. In the older age group, where atherosclerotic lesions are common, the
etiology is often vasculopathic. For a 65-year-old to have this presentation as an isolated first
sign of demyelination would be distinctly unusual, and further evidence for demyelination
would need to be found before embarking on a trial of corticosteroids.
C. The older patient probably has a vasculopathic process. (0 Points)

D. The most likely problem in the younger patient is demyelinating disease. (0 Points)
Question: A 64-year-old woman reports progressive onset of ptosis and diplopia over an 8-month
period. On examination, the left eye is normal, but the right eye reveals the following abnormalities: 4
mm of ptosis, limitation of eye movement in all directions, 4 mm pupil that does not react to light or
dilate in darkness, and loss of corneal sensation. What is the most likely diagnosis? (1 Points)
Answer:
A. Myasthenia gravis (0 Points)

B. Intracavernous meningioma (1 Points)
Answer Feedback:
The patient's findings can best be explained by involvement of the third, sixth, fifth, and
sympathetic nerves. Fourth-nerve involvement cannot be determined from the description. A
mid-dilated pupil that does not react to light or dilate in darkness usually indicates impairment
of both the parasympathetic and the sympathetic innervation of the pupil. This finding, as well
as the other findings, can best be explained by a lesion in the cavernous sinus. Meningioma
and internal carotid artery aneurysm are the most common causes, particularly with a slowly
progressive history. Myasthenia gravis and Graves' ophthalmopathy would not produce the
pupillary or sensory findings noted in this patient. An aneurysm of the posterior
communicating artery could produce a third-nerve palsy, but would not be expected to
produce the other findings. A midbrain lesion typically produces bilateral ptosis, if any, and
could not explain the sensory findings.
C. Graves' ophthalmopathy (0 Points)

D. Aneurysm of the posterior communicating artery (0 Points)

Print

Percentage Score 0%
Questions
Question: Which one of the following conditions would most likely be found in a patient with optic disc
edema, macular exudate, and vitritis? (1 Points)
Answer:
A. Hypertensive retinopathy (0 Points)

B. Papilledema (0 Points)
C. Nonarteritic anterior ischemic optic neuropathy (0 Points)
D. Optic neuritis (1 Points)
Answer Feedback:
Inflammatory optic neuropathy (optic neuritis) would be expected to be associated with other
evidence of ocular inflammation in some cases. None of the other conditions would be
expected to be associated with inflammation.
Question: Which of the following findings would be most suggestive of visual loss from optic disc
drusen? (1 Points)
Answer:
A. Choroidal folds (0 Points)

B. dyschromatopsia (0 Points)
C. Central scotoma (0 Points)
D. Inferonasal visual field defect (1 Points)
Answer Feedback:
Optic disc drusen is associated with central visual loss or dyschromatopsia in most patients.
Inferonasal defects are the most common type of visual field loss from optic disc druen, which
do not cause mass effect resulting in choroidal folds.
Question: Papilledema would be an expected cause of visual loss in a patient with which of the
following findings? (1 Points)
Answer:
A. Pituitary apoplexy (0 Points)

B. Bilateral optic nerve sheath enhancement (0 Points)
C. Meningeal enhancement from intracranial hypotension (0 Points)
D. Hydrocephalus from aqueductal stenosis (1 Points)
Answer Feedback:
Papilledema, by defintion, is optic disc swelling from elevated intracranial pressure. Only
hydrocephalus would be expected to cause papilledema.
Question: Difficulty reading in a patient with a Chiari malformation would be most likely from which of
the following findings? (1 Points)
Answer:
A. Fourth cranial nerve palsy (0 Points)

B. Skew deviation (0 Points)
C. Downbeating nystagmus (1 Points)
Answer Feedback:
Difficulty reading in a patient with a Chiari malformation would be most likely from
downbeating nystagmus.
D. Bitemporal hemianopia (0 Points)
Question: Which of the following would be most suggestive of a compressive lesion causing a right
sixth cranial nerve palsy? (1 Points)
Answer:
A. Progressive diplopia over a 6-month period (1 Points)
Answer Feedback:
Compressive lesions tend to cause progressive visual symptoms with the continued growth of
the mass.
B. Right periorbital pain (0 Points)

C. Esotropia worse in left gaze (0 Points)
D. Intermittent diplopia (0 Points)
Question: An abduction deficit caused by a sixth cranial nerve palsy can be best distinguished from
that due to thyroid eye disease by which one of the following? (1 Points)
Answer:
A. An exophthalmometer (0 Points)
B. Forced duction testing (1 Points)
Answer Feedback:
Restrictive processes can be differentiated from paralytic processes using forced duction
testing. None of the other techniques listed would help differeniate between these two.
C. A double maddox rod (0 Points)

D. Alternate cover testing (0 Points)
Question: Which of the following findings would be expected to be present one year after a stroke
involving the right optic tract? (1 Points)
Answer:
A. Right relatve afferent pupillary defect (0 Points)

B. Right optic disc pallor (1 Points)
Answer Feedback:
Optic tract lesions cause bilateral optic disc pallor in association with a contralateral
homonymous hemianopia and a contralateral relative afferent pupillary defect.
C. Right dyschromatopsia (0 Points)

D. Right homonymous hemianopia (0 Points)
Question: Transient visual loss in one eye associated with eye pain lasting one hour is most
consistent with which of the following? (1 Points)
Answer:
A. Papilledema (0 Points)
B. Vertebrobasilar arterial insufficiency (0 Points)
C. Uveitis-glaucoma-hyphema syndrome (1 Points)
Answer Feedback:
Uveitis-glaucoma-hypema typically causes symptoms which last longer than transient visual
loss from thromboembolic disease. None of the other conditions is typically associated with
eye pain when the transient visual loss occurs.
D. Cardiac embolic disease (0 Points)
Question:
All of the following clinical findings may be associated with an optic neuropathy. Which of these findings
is most specific for an optic neuropathy as opposed to other causes of visual loss?
(1 Points)
Answer:
A. Central scotoma (0 Points)

B. decreased visual acuity (0 Points)
C. Relative afferent pupillary defect (1 Points)
Answer Feedback:
Of the choices listed, a relative afferent pupillary defect would be the most suggestive of an
optic neuropathy. Cataract and/or macular degeneration could cause the other problems
listed.
D. Decreased color vision (0 Points)

Question: Which one of the following findings would be most likely in a patient with unilateral
proptosis and eyelid retraction? (1 Points)
Answer:
A. Choroidal folds (1 Points)
Answer Feedback:
Thyroid eye disease would not be expected to be associated with inflammation in the eye or
cystoid macular edema. Thyroid eye disease is one of the most common causes of choroidal
folds, which are often horizontal with respect to the macula.
B. Cystoid macular edema (0 Points)

C. Retinal arterial vasculitis (0 Points)
D. Pars planitis (0 Points)
Print

Percentage Score 0%
Questions
Question: A 28-year-old man complains of painless loss of vision in his right eye. On exam he is
20/20 OD with 1 diopter of hyperopic shift, and 20/20 OS. Color saturation is equal between both eyes,
and there is no relative afferent pupillary defect. After static automated perimetry, the patient's central
sensitivities are mildly reduced on the right, and he has a normal visual field on the left. Photo stress
recovery time is 2 minutes on the right and 20 seconds on the left. What is the most likely diagnosis?
(1 Points)
Answer:
A. Retrobulbar optic neuritis (0 Points)

B. Vitreous hemorrhage (0 Points)
C. Amblyopia (0 Points)
D. Central serous retinopathy (1 Points)
Answer Feedback:
Maculopathies are associated with a prolonged (> 50 seconds) photostress recovery, which is
thought to occur because of prolonged recovery of dysfunctional retinal photoreceptors.
Absence of relative afferent pupillary defect supports that it is unlikely that this is due to an
optic neuropathy. Hyperopia may develop as the retina is elevated, and is not typical of an
isolated optic neuropathy or amblyopia.
Question: Compared to patients who have optic neuritis, patients with neuroretinitis are at low risk for
developing which of the following conditions? (1 Points)
Answer:
A. Permanent vision loss (0 Points)

B. Lyme disease (0 Points)
C. Multiple sclerosis (1 Points)
Answer Feedback:
Neuroretinitis, optic disc edema with macular exudate, often in the configuration of a macular
star, is most commonly seen from inflammatory optic neuropathies, including those from
infection such as cat scratch disease or Lyme disease. Often there are vitreous cells, and
visual function typically improves spontaneously. Neuroretinitis, marked optic disc
edema, and marked optic disc hemorrhage or macular exudates are not typically seen in
patients who have demyelinating optic neuritis from multiple sclerosis.
D. Optic disc swelling associated with exudates (0 Points)
Question: Temporal arteritis is diagnosed on biopsy when which of the following findings is present?
(1 Points)
Answer:
A. Granulomatosis inflammation with or without giant cells (1 Points)
Answer Feedback:
The hallmark histopathologic finding in temporal (giant) cell arteritis is granulomatous
inflammation. Giant cells need not be present. There may also be disruption of the internal
elastic lamina and thicking of the intima, but these may also be seen in conditions other than
temporal arteritis. Intimal hyperplasia or lymphocytes and plasma cells are not specific for
temporal arteritis. Calcific plaque with arterial occlusion is characteristic of embolic disease
from atherosclerosis.
B. Calcific plaque with arterial occlusion (0 Points)

C. Lymphocyes and plasma cells (0 Points)
D. Intimal hyperplasia (0 Points)
Question: A 53-year-old woman has difficulty keeping her eyes open. A visit to her primary care
provider reveals nothing. She tells you that it is easier to keep her eyes open if she hums or sings,
touches her temples or her forehead between her eyes, or chews on her glasses. What condition does
this most likely represent? (1 Points)
Answer:
A. Levator dehiscence (0 Points)

B. Myasthenia gravis (0 Points)
C. Benign essential blepharospasm (1 Points)
Answer Feedback:
Benign essential blepharospasm is typically bilateral and often is aggravated by periods of
stress. Myasthenia gravis may cause bilateral ptosis, which would be expected to worsen with
increased activity of the facial muscles, such as during prolonged upgaze, and worsen
throughout the day.
D. Dry eye (0 Points)
Question: Rebound nystagmus is a sign for a lesion of what structure? (1 Points)

Answer:
A. Medial longitudinal fasiculus (MLF) (0 Points)

B. Rostral interstitial nucleus of the MLF (0 Points)
C. Parapontine reticular formation (0 Points)
D. Cerebellar hemisphere (1 Points)
Answer Feedback:
Rebound nystagmus occurs with lesions involving the cerebellum. Lesions involving the
medial longitudinal fasciculus cause internuclear ophthalmoplegia. The rostral interstitial
nucleus of the MLF is involved in coordinating vertical gaze, and the parapontine reticular
formation is involved in horizontal gaze, so lesions at these sites result in gaze palsies.
Question: A 30-year-old woman is referred to you by her neurosurgeon after removal of a cerebellar
hemangioma. Which of the following are you most likely to find? (1 Points)
Answer:
A. Cavernous angioma of the retina (0 Points)

B. Cavernous hemangioma of the choroid (0 Points)
C. Capillary hemangioma of the retina (1 Points)
Answer Feedback:
Capillary hemangioma of the retina is associated with cerebellar and other central nervous
system hemangiomas as part of the Von Hippel-Lindau syndrome. Choroidal hemangiomas
are seen in Sturge-Weber syndrome. Racemous angiomas of the retina and cavernous
hemangiomas of the retina most commonly occur as isolated entities.
D. Racemous angioma of the retina (0 Points)
Question: A 30-year-old female complains of a continuous quivering of the upper and lower lids
around her right eye over the last 2 weeks. On examination, there is a continuous undulation in the
right orbicularis oculi that trails off into adjacent facial muscles. There is also an asymetrically
prominent nasolabial fold on the right, and her palpebral fissure is narrower on the right side than on
the left. She has no facial weakness. Ophthalmoscopy reveals that the patient has bilateral temporal
pallor and nerve fiber thinning in both eyes. Her saccadic eye movements show mild adduction slowing
with abductor overshoot. What is the most likely diagnosis? (1 Points)
Answer:
A. Guillain-Barre syndrome (0 Points)

B. Multiple sclerosis (1 Points)
Answer Feedback:
The description of undulating movements of the eyelid are characteristic of eyelid myokymia,
most commonly a benign and self-limited condition when it occurs in isolation. Extension of
the myokymia to involve other facial muscles indicates more widespread involvement of the
facial nerve, most commonly seen in ponitine lesions. The presence of optic disc pallor, if
caused by the same condition, is likely related to an optic neuropathy, and of the listed
conditions, only mutiple sclerosis would be associated with a combination of facial myokymia
and optic neuropathy.
C. Brainstem glioma (0 Points)

D. Mitochondrial myopathy (0 Points)
Question: A 62-year-old diabetic and hypertensive male wakes the day after cardiac bypass graft and
notices that the vision in his left eye is blurry. That evening his right eye becomes similarly affected. On
examination, vision is 20/40 OD and 20/60 OS. No afferent defect is detected. However, there are
bilateral inferior altitudinal visual field defects, and both optic discs are swollen superiorly with splinter
hemmorhages. Which test or mechanism best characterizes this disorder? (1 Points)
Answer:
A. The absence of relative afferent pupillary defect means that vision loss is likely due to a
vitreous hemorrhage. (0 Points)
B. Visual loss is related to embolic occlusion in the posterior ciliary circulations. (0 Points)
C. The patient probably experienced blood loss and periods of hypotension during the
operation. (1 Points)
Answer Feedback:
Anterior ischemic optic neuropathy (AION), while typically idiopathic and unilateral, can occur
following hypotension, blood loss, or general surgical procedures (most commonly back and
cardiac surgery). Under these circumstances, the anterior ischemic optic neuropathy is
bilateral about half the time. If the severity of AION is symmetric, there may be no relative
afferent pupillary defect. Emboli are not thought to be a cause of anterior ischemic optic
neuropathy in most patients. In the perioperative period, the erythrocyte sedimentation rate
may be elevated, and in the absence of other symptoms, should not, in isolation, be thought
to be caused by temporal cell arteritis.
D. The patient's erythrocyte sedimentation rate (ESR) of 87 on the second postoperative day is
probably a result of temporal arteritis. (0 Points)
Question: A 60-year-old man has intermittent binocular horizontal diplopia. Physical examination
reveals impaired adduction of the left eye with an abducting nystagmus of the right eye. For a single
lesion to explain these findings where is its location? (1 Points)
Answer:
A. Left medial longitudinal fasciculus (1 Points)
Answer Feedback:
Internuclear ophthalmoplegia is characterized by an adduction deficit and contralateral
abducting nystagmus. Damage to the medial longitudinal fasciculus, which extends from the
pons to the midbrain, is responsible for causing an internuclear ophthalmoplegia. Thus, this
constellation of findings would be a LEFT, not a RIGHT, medial longitudinal fasciculus. A
lesion of the 6th or 3rd fasciculus would not be transitory, bilateral, or associated with
nystagmus.
B. Right medial longitudinal fasciculus (0 Points)

C. Left third-nerve fascicle (0 Points)
D. Right sixth-nerve fascicle (0 Points)
Question: A 30-year-old man with chronic sinus disease has endoscopic sinus surgery.
Postoperatively, he develops a retrobulbar hemorrhage. Physical examination reveals no damage to
the globe. What is the most urgent indication for lateral canthotomy/cantholysis? (1 Points)
Answer:
A. Intraocular pressure > 50 mm Hg with a relative afferent pupillary defect (1 Points)
Answer Feedback:
The most pressing indication for surgical intervention in a patient with orbital hemorrhage is
visual loss from optic neuropathy. Elevated intraocular pressure, especially when
unresponsive to medical therapy, may also require a canthotomy/cantholysis. Limited ocular
motility with diplopia, presence of subconjuntival hemorrhage, or onset of proptosis may
suggest orbital hemorrhage, but this alone is not typically require urgent intervention.
B. Presence of subconjunctival hemorrhage (0 Points)

C. Limited ocular motility with diplopia (0 Points)
D. Onset of proptosis (0 Points)
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Percentage Score 0%
Questions
Question: What is the structural basis for the pupillary light-near dissociation in Adie's tonic pupil? (1
Points)
Answer:
A. More fibers innervate the ciliary body than the pupillary sphincter. (1 Points)
Answer Feedback:
Tonic (Adie's) pupils are thought to occur from loss of innervation of the pupillary sphincter
from the ciliary ganglion. Many more fibers are targeted for the ciliary body than for the
pupillary sphincter muscle, and loss of innervation from the ciliary ganglion results in light-
near dissociation, with relative preservation of pupil constriction with near response compared
to light response. The ciliary ganglion provides parasympathetic imput to the pupillary
sphincter. Aberrant pupillary constriction with extraocular movement may be seen after third
nerve palsy due to aberrant regeneration, not with tonic pupils.
B. The ciliary ganglion supplies the sympathetic innervation to the pupil and results in a smaller
ipsilateral pupil. (0 Points)
C. Fibers innervating the pupil are aberrantly activated with contraction of the extraocular
muscles. (0 Points)
D. More fibers innervate the pupillary sphincter than the ciliary body. (0 Points)
Question: Following injection of intravenous edrophonium (tensilon), what finding would most strongly
suggest the diagnosis of myasthenia gravis? (1 Points)
Answer:
A. Nystagmus resolution. (0 Points)

B. Ocular misalignment improves with alternate cover testing. (1 Points)
Answer Feedback:
Intravenous edrophonium is useful diagnostically for myasthenia gravis because it improves
neuromuscular transmission. The most predictive findings for a positive response to
edrophonium are objective improvements in ptosis or ocular misalignment. Saccadic speed is
often normal, and nystagmus does not occur from myasthenia gravis.
C. Saccadic movements return to normal velocity. (0 Points)

D. Eyelid myokymia. (0 Points)
Question: A 65-year-old man presents for a routine examination. The pupils are each 4 millimeters in
diameter, round, and poorly reactive to direct and consensual light. A brisk near response is present
with brisk redilation on refixation from near to far. Which of the following testing devices or procedures
is most likely to yield diagnostic information? (1 Points)
Answer:
A. Lumbar puncture (0 Points)

B. Optokinetic drum (1 Points)
Answer Feedback:
For patients who have pupils that respond sluggishly to light, the pupillary response to near
stimulus should be assessed. Light-near dissociation most commonly occurs from causes of
decreased vision such as bilateral retinal or optic nerve disease. This finding can also be
seen in dorsal midbrain (Parinaud) syndrome, characterized by deficits in upgaze, light near
dissociation, and convergence retraction. Pursuit and saccades can be appreciated using an
optokinetic drum, which can be rotated downward to produce upward saccades. Upward
saccades in patients with dorsal midbrain syndrome often induce convergence-retraction of
the globes. Tonicity to near is suggestive of tonic (Adie) pupils, and otherwise is not
associated with reduced visual function or an upgaze deficit. Dilute pilocaprine is often
associated with supersensitivity with tonic pupils.
C. Dilute pilocarpine (0 Points)

D. Reflex hammer (0 Points)

Question: A 42-year-old woman had endovascular coils placed in a left posterior communicating
aneurysm 3 months ago. She now has a nearly complete, pupil-involving third-nerve palsy. What would
be the preferred management? (1 Points)
Answer:
A. Referral for strabismus surgery (0 Points)

B. Surgical repair of ptosis (0 Points)
C. Trial of corticosteroids to reduce inflammation from coiling (0 Points)
D. Patching (1 Points)
Answer Feedback:
Coiling of intracranial aneurysms has been increasingly used as a primary mode of
treatment. This includes treatment of aneurysms involving the posterior communicating
artery, the most common to cause a pupil-involving third-nerve palsy. Improvement of the
cranial nerve palsy may occur as coils cause thrombosis and decrease pulsations within the
aneurysmal sac. Resolution of cranial nerve palsy may take months to occur, therefore early
surgery for ptosis and ocular misalignment should be avoided. Corticosteroids are not
generally used because coiling is not typically associated with inflammation.
Question: In a patient with papilledema, what do the small, bright, refractile lesions on the optic disc
represent? (1 Points)
Answer:
A. Pseudodrusen of chronic swelling (1 Points)
Answer Feedback:
Chronic papilledema may result in chronic blockage of axoplasmic flow, which results in
visible refractile-appearing areas superificially, within the optic disc. These areas are termed
pseudodrusen because they are not true optic disc drusen, characterized by calcium
deposition. Pseudodrusen typically take months to develop and do not occur with early optic
disc resolution. Neither neovascularization of the optic disc, which causes abnormal blood
vessel formation, nor neuroretinitis, which implies the formation of macular exudate in
combination with optic disc edema, are associated with refractile lesions of the optic disc.
B. Early resolution of the swelling (0 Points)

C. Neuroretinitis (0 Points)
D. Neovascularization (0 Points)
Question: Which of the following therapies is preferred when managing non-arteritic anterior ischemic
optic neuropathy (NAION)? (1 Points)
Answer:
A. Lowering intraocular pressure (IOP) for visual loss. (0 Points)

B. Optic nerve sheath decompression for visual loss. (0 Points)
C. No effective treatment has been proven. (1 Points)
Answer Feedback:
No therapy for NAION has been proven to be effective. The optic nerve decompression trial,
which randomized patients with NAION to observation or surgery, was not only unhelpful, but
potentially harmful. Lowering IOP has not been found to help, and most patients have normal
IOP in the affected and fellow eye. Aspirin has not been proven to be effective acutely or to
prevent involvement of the fellow eye. So far, agents used for neuroprotection have not been
effective in preventing NAION or shown to reduce visual loss.
D. Aspirin protects the contralateral eye. (0 Points)
Question: A central scotoma in one eye and a superior temporal defect in the other suggests a lesion
in what location? (1 Points)
Answer:
A. Both optic nerves (0 Points)

B. Optic tract (0 Points)
C. Junction of the optic nerve and chiasm (1 Points)
Answer Feedback:
Preferential involvement of the temporal visual field suggests chiasmal disease until proven
otherwise. The combination of a central visual field defect in one eye and a superotemporal
defect in the other eye (termed a junctional scotoma) localizes the problem to the anterior
optic chiasm, the junction between the intracranial optic nerve and optic chiasm. Bilateral
optic nerve disease would be expected to cause bilateral central defects or nerve fiber bundle
type defects. Lesions of the optic tract and parietal lobe result in homonymous hemianopias.
D. Occipital lobe (0 Points)
Question: When managing a patient with thyroid orbitopathy who has developed vision loss, what
procedure or intervention should be included? (1 Points)
Answer:
A. Periodic measurement of visual fields and color vision (1 Points)
Answer Feedback:
Thyroid orbitopathy may result in optic neuropathy (estimated to occur in 5% of patients with
thyroid eye disease). Due to the potential for optic neuropathy, color vision and visual fields
should be measured and followed over time. Although CT scans of the orbit help in
diagnosing thyroid eye disease and establishing compressive optic neuropathy as a cause of
visual loss (most commonly from crowding at the orbital apex secondary to enlargement of
the extraocular muscles), repeating them every 3 months serially is not necessary. Systemic
corticosteroids may play a role in decreasing the inflammatory component of thyroid
orbitopathy, but topical corticosteroids are not helpful. Strabismus surgery, if necessary,
should be delayed until after orbital decompression and after the ocular misalignment is
stable.
B. Strabisumus surgery soon after the development of double vision (0 Points)

C. Topical steroid therapy and systemic diuresis (0 Points)
D. Serial computed tomography (CT) scans of the orbit every 3 months (0 Points)
Question: When considering a diagnosis of idiopathic intracranial hypertension (IIH or pseudotumor

cerebri), treatment with what antibiotic may be relevant? (1 Points)
Answer:
A. Ciprofloxicin (0 Points)
B. Gentamicin (0 Points)
C. Tetracycline (1 Points)
Answer Feedback:
Elevated intracranial pressure can result from a number of causes including mass lesions,
hydrocephalus, meningitis, and thrombosis of the cerebral venous sinuses. Some
medications have been associated with elevated intracranial pressure, including tetracycline
and its derivatives. Gentamicin, Ciprofloxin and fluconazole are not implicated as a cause of
elevated intracranial pressure.
D. Fluconazole (0 Points)
Question: Substernal chest pain in a patient with anterior ischemic optic neuropathy (AION) caused
by giant cell arteritis could indicate what condition? (1 Points)
Answer:
A. Referred pain from carotid arteritis (0 Points)

B. Coronary arteritis (1 Points)
Answer Feedback:
Temporal (giant cell) arteritis is a systemic vasculitis that involves medium and small
arteries. Although the most common ophthalmic manifestations involve the ophthalmic
circulation, other circulations including the cerebral arteries and coronary arteries can be
involved. High steriod levels would most likely reduce arteritis. Carotid arteritis does not
cause referred pain. Hepatomegaly is unrelated to ophthalmic arteritis.
C. Hepatomegaly (0 Points)
D. Toxic steroid levels (0 Points)
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AAO Neuro-Ophthalmology/Orbit 01 Short

Total Questions: 7 (7 Points)

A. A low serum B12 and thiamine (0 Points)

D. Bilateral cecocentral scotomas (0 Points)

Your answer: (0 Points)

Your answer: (0 Points)

Your answer: (0 Points)

A. Traumatic optic nerve sheath hematoma (0 Points)

D. Papilledema with visual loss from cryptococcal meningitis (0 Points)

Your answer: (0 Points)

D. Optic nerve sheath fenestration substantially lowers intracranial pressure in pseudotumor

Your answer: (0 Points)

A. Medial longitudinal fasciculus (0 Points)

D. Third nerve nucleus (0 Points)

Your answer: (0 Points)

A. Stereo acuity testing (1 Points)

B. A lumbar puncture (0 Points)

Your answer: (0 Points)

Total Questions: 8 (8 Points)

A. Testing with neutral density filters (1 Points)

B. Color vision testing with red-green glasses (0 Points)

Your answer: (0 Points)

A. Pituitary apoplexy (0 Points)

C. Bilateral anterior ischemic optic neuropathy (0 Points)

Your answer: (0 Points)

A. Erythrocyte sedimentation rate (0 Points)

C. Carotid duplex study (0 Points)

Your answer: (0 Points)

A. MRI of the brain (0 Points)

D. Acetylcholine receptor antibody test (0 Points)

Your answer: (0 Points)

A. Cyclic oculomotor palsy (1 Points)

B. Primary aberrant regeneration (0 Points)

Your answer: (0 Points)

A. CT of orbits, with contrast (0 Points)

D. Cerebral angiogram (0 Points)

Your answer: (0 Points)

A. Chronic progressive external ophthalmoplegia (0 Points)

C. Occult tumor (0 Points)

Your answer: (0 Points)

A. Temporal arteritis (0 Points)

D. Cavernous sinus aneurysm (0 Points)

Your answer: (0 Points)

AAO Neuro-Ophthalmology/Orbit 03 Short

Total Questions: 9 (9 Points)

A. Prostaglandin inhibitors (0 Points)

Your answer: (0 Points)

A. Hyperopic shift (0 Points)

Your answer: (0 Points)

A. History of blindness OD secondary to optic neuropathy (0 Points)

Your answer: (0 Points)

Your answer: (0 Points)

C. Adie's pupil (0 Points)

Your answer: (0 Points)

A. Optic nerve sheath fenestration OS (1 Points)

B. repeat MRI scan (0 Points)

Your answer: (0 Points)

A. Fluorescein angiogram (0 Points)

Your answer: (0 Points)

A. Pituitary tumor (0 Points)

Your answer: (0 Points)

A. Light-near dissociation on pupillary testing (0 Points)

D. Convergence retraction nystagmus on upgaze (0 Points)

Your answer: (0 Points)