Professional Documents
Culture Documents
Question 1 of 130
Which of the following statements does not fully describe chiasmal lesions?
Patients with an empty sella syndrome without a mass or active pseudotumor cerebri may develop chiasmal field
defects.
Meningiomas that affect the chiasm are more often seen in males.
A patient who has had pituitary apoplexy may have normal visual fields.
Question 2 of 130
Which of the following exogenous agents/drugs is not a typical cause of toxic-nutritional optic neuropathy?
Chloramphenicol
Methanol
Ethambutol
Please select an 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.
Question 3 of 130
Which of the following accurately describes optic nerve sheath fenestration and lumboperitoneal shunting in pseudotumor
cerebri?
Optic nerve sheath fenestration should always be performed before lumboperitoneal shunting in pseudotumor cerebri.
Following optic nerve sheath fenestration, low-pressure headaches are never a problem.
Lumboperitoneal shunting should always be performed before optic nerve sheath fenestration in pseudotumor cerebri.
Please select an 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 pressure headaches, but these can occur with lumboperitoneal shunting.
Question 4 of 130
Which of the following is optic nerve sheath fenestration not a useful therapeutic intervention for?
Pseudotumor cerebri
Cavernous sinus
Question 6 of 130
A 3-month-old child is admitted to the hospital because of drooling and difficulty swallowing. You are asked to see the
patient in consultation, because the child is noted to have complete ophthalmoplegia, ptosis, and pupils that are 9 OU and
unreactive to light or near. What is the most likely diagnosis?
Infantile botulism
Atropine toxicity
It may give the appearance of images persisting after they are gone.
Question 7 of 130
Which of the following is not typical of palinopsia?
It may give the appearance of images persisting after they are gone.
Question 8 of 130
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 examination is normal. What is the most appropriate test for this patient?
MRI
A fluorescein angiogram
A lumbar puncture
Please select an 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, along with fogging, OKN testing, and color vision testing with red-green glasses (green in front of the "good"
eye) will all potentially give information about how well the patient actually sees 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.
Question 9 of 130
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?
Acalculia
Right-left confusion
A right homonymous hemianopsia, worse in the superior hemifield than in the inferior hemifield
Please select an 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 10 of 130
Which of the following is least consistent with toxic-nutritional optic neuropathy?
Observation
Question 12 of 130
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 examination is normal. Which of the following clinical tests would not be
helpful for the evaluation of the patient?
Fogging
Please select an 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, along with fogging, OKN testing, and color vision testing with red-green glasses (green in front of the "good"
eye) will all potentially give information about how well the patient actually sees 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.
Question 13 of 130
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?
Tolosa-Hunt syndrome
Dysthyroid orbitopathy
Occult tumor
Please select an 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.
Question 14 of 130
A 32-year-old woman complains of intermittent diplopia, usually lasting about half an hour when she first wakes up in the
morning, with a few months' duration. 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. Several months later, the left eyelid of
this patient becomes slightly ptotic, and she develops a 12-PD exotropia at distance, with a moderate underaction of
adduction of the right eye. Horizontal forced testing is normal. What is the most likely diagnosis?
Internuclear ophthalmoplegia
Myasthenia gravis
Question 15 of 130
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?
Cerebral angiogram
Please select an 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.
Question 16 of 130
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?
Ophthalmoplegic migraine
Pituitary apoplexy
Question 18 of 130
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?
MRI or CT scan
Fluorescein angiogram
Please select an 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.
Question 19 of 130
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?
Temporal arteritis
Postherpetic neuralgia
Trigeminal neuralgia
Please select an 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.
Question 20 of 130
A 32-year-old woman complains of intermittent diplopia, usually lasting about half an hour when she first wakes up in the
morning, with a few months' duration. 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. Which of the following is the most
appropriate next test?
FTA-ABS
Question 22 of 130
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?
Weight reduction
Lumboperitoneal shunt
Please select an 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.
Lumboperitoneal shunting could be performed, although given the potential risks and problems associated with this
procedure, 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.
Question 23 of 130
A 32-year-old woman complains of intermittent diplopia, usually lasting about half an hour when she first wakes up in the
morning, with a few months' duration. 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?
Dysthyroid orbitopathy
Myasthenia gravis
Pituitary tumor
Please select an 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 24 of 130
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?
Psychiatric consultation
Fluorescein angiogram
Please select an 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 26 of 130
A 20-year-old nurse in an ophthalmology clinic 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?
Uveitis
Please select an 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 27 of 130
Which of the following features is most consistent with pharmacologic pilocarpine use in a patient with anisocoria?
Hyperopic shift
Question 28 of 130
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 might the visual field examination be expected to show?
Bitemporal hemianopia
Binasal hemianopia
Please select an 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 29 of 130
A 20-year-old nurse in an ophthalmology clinic complains of right-sided brow ache. Examination of both eyes is normal,
except for the presence of a 1.5 mm, nonreactive right pupil to light or near stimuli. The pupil 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 and flare. What is the most likely diagnosis?
Horner's syndrome
Adie's pupil
Question 30 of 130
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?
Parasympathomimetic
Prostaglandin inhibitors
Sympathomimetic
CT of the orbit
Please select an 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 32 of 130
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. Assuming all testing proved
negative, daily prophylactic therapy would not include which of the following?
A beta blocker
Dihydroergotamine (DHE)
Amitriptyline
Downbeat nystagmus
Myasthenia gravis
Please select an 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.
Question 34 of 130
Which of the following statements relates to a patient who has acute optic neuritis in the right eye?
With the information given, the results of the relative afferent pupillary defect test cannot be predicted.
It would be rare for the patient to have a new visual field defect on automated perimetry in the left eye.
There must be a relative afferent pupillary defect present in the right eye.
There cannot be a better pupillary response in the right eye than in the left.
Please select an answer
Feedback: Optic neuritis frequently is a sign of multiple sclerosis. Many patients with optic neuritis have a prior history of
contralateral optic neuritis. If this patient had had a prior optic neuritis in the left eye, a relative afferent pupillary defect
(RAPD) might not be present when acute optic neuritis occurred in the right eye. Indeed, if optic nerve function were worse
in the left eye than in the right at the time of examination, the RAPD might be in the left eye and the pupil might respond
better in the right eye. In the case described, therefore, with no information about the contralateral eye, the results of the
RAPD testing cannot be predicted. At the time of acute optic neuritis, contralateral asymptomatic visual field defects are
commonly seen.
Question 35 of 130
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?
The patient has a history of motion sickness and has just returned from a cruise.
The patient says that the right eye with the larger pupil has always had a lighter colored iris than the left eye.
The patient is aware of an inability to read with the right eye only.
Question 36 of 130
Which of the following statements does not accurately describe optokinetic response testing?
In a patient with a complete right adduction deficit, ability to cause the right eye to fully adduct in response to an
optokinetic drum indicates that the ophthalmoparesis is not infranuclear in origin.
Absence of response with the tape moving to the patient's left may indicate a left frontal lobe lesion.
Presence of optokinetic nystagmus response to an optokinetic drum at a test distance of 3 feet indicates a visual acuity
of 20/60 or better.
Absence of response with the tape moving to the patient's right may indicate a right parietal lesion.
Please select an answer
Feedback: The optokinetic response may be thought of as a pursuit movement in the direction of the movement of the drum
or tape, and a refixational saccadic movement in the direction away from the movement of the drum or tape. In the case of a
drum moving to the patient's right, the following pursuit movement toward the right is mediated by the right parietal lobe. The
refixational leftward saccade is mediated by the right frontal lobe. Thus, optokinetic response testing may be thought of as
testing the saccade and pursuit initiation in the frontal and parietal lobes on the side toward which the tape or drum is
moving. As with caloric or oculocephalic maneuvers, optokinetic response testing may be used to demonstrate that the final
common pathway from the nucleus to nerve to extraocular muscle is intact, or that the ocular rotation defect is supranuclear
in origin. An optokinetic drum may also be used to estimate visual acuity (as in cases of functional visual loss), but the
minimum acuity requisite to obtain a response to a drum at 3 feet is only counting fingers at 3 to 5 feet.
Question 37 of 130
An emergent consultation is requested on a comatose man with significant periocular trauma to the right eye. A CT scan of
the head is essentially normal. The patient's pupils are 8 mm OD, 4 mm OS; the right pupil does not react to direct light. The
right eye is exodeviated and has complete ptosis, and oculocephalic maneuver indicates that the right eye does not cross
the midline in the field of action of the right medial rectus muscle. There is no enophthalmos or proptosis. Intraocular
pressures are 19 mm Hg OD and 12 mm Hg OS. Both fundi are unremarkable. For the purpose of guiding emergent
treatment, which of the following is the most appropriate test to perform?
Examination of the relative magnitude of the pupillary response of the left eye, when the left and then the right eye is
illuminated
Question 38 of 130
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. What is the most likely diagnosis?
Classic migraine
Cluster headache
Please select an 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.
Question 39 of 130
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?
He has optic nerve hypoplasia OD, and his mother has insulindependent (juvenile-onset) diabetes mellitus.
The patient had an episode of marked acute visual loss OD with pain on ocular rotation 2 years before, with partial
recovery.
Please select an 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.
Question 40 of 130
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?
Observation only
Echocardiogram
Carotid ultrasound
MRI
Please select an 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 41 of 130
A mass causing a relative afferent pupillary defect and a homonymous hemianopia would most likely be found in which one
of the following?
Optic nerve
Optic chiasm
Optic tract
Occipital lobe
Please select an 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.
Question 42 of 130
Which one of the following is a contraindication to magnetic resonance imaging?
Iodine allergy
Acute hemorrhage
Cochlear implant
Please select an 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.
Question 43 of 130
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?
Retinopathy
Chiasmal syndrome
Retinopathy
Chiasmal syndrome
Question 44 of 130
Which of the following findings would most likely suggest a supranuclear gaze palsy in a 72-year-old man who cannot look
down?
Downbeating nystagmus
Please select an 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 doll’s head
maneuver suggests the nuclear and peripheral pathways for eye movement are functional.
Question 45 of 130
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?
Optic neuritis
Occipital stroke
Please select an 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. The only choice for an optic neuropathy listed is
posterior ischemic optic neuropathy, which indicates that the optic disc is normal acutely and develops pallor weeks after the
ischemia.
Question 47 of 130
Which one of the following disorders of facial activity is most commonly bilateral?
Essential blepharospasm
Eyelid myokymia
Hemifacial spasm
Question 48 of 130
Eye pain with ipsilateral ptosis would be most likely to be seen in a patient with which one of the following?
Carotid dissection
Horner syndrome
Nyctalopia
Carotid stenosis
Please select an answer
Feedback: Ocular ischemic syndrome, or venous stasis retinopathy, is characterized by venous dilation, typically in the
absence of tortusoity, retinal hemorrages, and decreased vision after exposure to light, likely secondary to prolonged
recovery of the retinal photoreceptors due to ischemia. Internal carotid stenosis (typically 90% or more) is the most common
cause of ocular ischemic syndrome, but Horner syndrome would not be an expected finding.
Question 49 of 130
Which one of the following would be expected in a patient with ocular ischemic syndrome?
Horner syndrome
Nyctalopia
Carotid stenosis
Please select an answer
Feedback: Ocular ischemic syndrome, or venous stasis retinopathy, is characterized by venous dilation, typically in the
absence of tortusoity, retinal hemorrages, and decreased vision after exposure to light, likely secondary to prolonged
recovery of the retinal photoreceptors due to ischemia. Internal carotid stenosis (typically 90% or more) is the most common
cause of ocular ischemic syndrome, but Horner syndrome would not be an expected finding.
Question 50 of 130
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?
Slit-lamp biomicroscopy
Carotid ultrasonography
Please select an 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.
Question 51 of 130
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 normal except for enlarged blind spots on visual field testing and marked bilateral papilledema. What is the
most reasonable next step in her management?
Start her on acetazolamide 500 mg q6h, and refer her to a neurologist on Tuesday.
Obtain an MRI that day; if it is negative, begin her on acetazolamide 500 mg q6h and see her in 3 days to determine
whether the papilledema has improved.
Start her on acetazolamide 500 mg q6h, and refer her to a neurologist on Thursday.
Obtain an MRI on her that day; if it is negative, arrange for a spinal tap shortly thereafter.
Please select an 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.
Question 52 of 130
Which of the following best describes Leber's hereditary optic neuropathy?
Cervicomedullary/cerebellar area
Parasellar/third ventricle
Question 54 of 130
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 image moves and is double only
when viewed with the right eye. The results of his examination are normal. Which of the following is the most appropriate
action to take?
Neuroimaging
Question 56 of 130
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. What is the most likely diagnosis?
One-and-a-half syndrome
Dysthyroid orbitopathy
Myasthenia gravis
Please select an 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 57 of 130
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 be most inconsistent with the diagnosis of the patient?
Dementia
Pigmentary retinopathy
Please select an 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
Question 58 of 130
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?
Observation
Restriction of the right eye on forced abduction, no force generated with attempted abduction on forced generation test
No restriction on forced abduction OD, diminished force generated with attempted abduction on forced generation test
Question 60 of 130
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?
Clivus
Question 62 of 130
Which of the following syndromes with neuro-ophthalmic signs is not associated with the endocrinopathy mentioned?
Question 64 of 130
Which of the following findings would be unusual in a patient presenting for an examination before craniotomy for a large
pineal tumor?
Skew deviation
Lid retraction
Please select an 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.
Question 65 of 130
A 7-year-old boy is referred by a pediatrician for evaluation of his visual status. The child is said to be slow in school and is
being placed in special education. The pediatrician wishes to ensure that a refractive error is not contributing to the poor
performance. The child has had three seizures, and a raised papular rash in a butterfly distribution has been noted on his
cheeks. Some patchy hypopigmented areas are also seen on the trunk. The child is otherwise well. What is the most likely
diagnosis?
A positive PPD (purified protein derivative), with the rest of the anergy panel negative (normal cutaneous response was
present to mumps)
Question 67 of 130
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?
The older patient should be treated with 1 g of methylprednisolone sodium succinate (Solumedrol) daily for 3 days.
Question 69 of 130
Which of the following historical points, or physical findings, would most call for MRI in a patient who is having an eleventh
bout of a classic migraine syndrome?
Graves' ophthalmopathy
Myasthenia gravis
Intracavernous meningioma
Question 71 of 130
Which one of the following conditions would most likely be found in a patient with optic disc edema, macular exudate, and
vitritis?
Optic neuritis
Hypertensive retinopathy
Papilledema
Please select an 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 72 of 130
Transient visual loss in one eye associated with eye pain lasting one hour is most consistent with which of the following?
Papilledema
Uveitis-glaucoma-hyphema syndrome
Please select an 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.
Question 73 of 130
Which of the following findings would be expected to be present one year after a stroke involving the right optic tract?
Right dyschromatopsia
Please select an answer
Feedback: Optic tract lesions cause bilateral optic disc pallor in association with a contralateral homonymous hemianopia
and a contralateral relative afferent pupillary defect.
Question 74 of 130
Which of the following would be most suggestive of a compressive lesion causing a right sixth cranial nerve palsy?
Intermittent diplopia
Please select an answer
Feedback: Compressive lesions tend to cause progressive visual symptoms with the continued growth of the mass.
Question 75 of 130
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?
An exophthalmometer
Question 76 of 130
Which one of the following findings would be most likely in a patient with unilateral proptosis and eyelid retraction?
Pars planitis
Choroidal folds
Please select an 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.
Question 77 of 130
Papilledema would be an expected cause of visual loss in a patient with which of the following findings?
Pituitary apoplexy
Bitemporal hemianopia
Downbeating nystagmus
Skew deviation
Question 79 of 130
Which of the following findings would be most suggestive of an optic neuropathy in a 50-year-old woman with decreased
vision in the right eye?
Central scotoma
Question 80 of 130
Which of the following findings would be most suggestive of visual loss from optic disc drusen?
Dyschromatopsia
Central scotoma
Choroidal folds
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?
Question 82 of 130
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?
Levator dehiscence
Myasthenia gravis
Dry eye
Please select an 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.
Question 83 of 130
Temporal arteritis is diagnosed on biopsy when which of the following findings is present?
Intimal hyperplasia
Question 84 of 130
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?
Vitreous hemorrhage
Amblyopia
Please select an 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 85 of 130
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?
Onset of proptosis
Please select an 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.
Question 86 of 130
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?
Question 87 of 130
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?
The patient's erythrocyte sedimentation rate (ESR) of 87 on the second postoperative day is probably a result of
temporal arteritis.
The patient probably experienced blood loss and periods of hypotension during the operation.
The absence of relative afferent pupillary defect means that vision loss is likely due to a vitreous hemorrhage.
Question 88 of 130
Rebound nystagmus is a sign for a lesion of what structure?
Cerebellar hemisphere
Please select an 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 89 of 130
Compared to patients who have optic neuritis, patients with neuroretinitis are at low risk for developing which of the following
conditions?
Multiple sclerosis
Lyme disease
Question 90 of 130
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?
Multiple sclerosis
Mitochondrial myopathy
Guillain-Barre syndrome
Brainstem glioma
Please select an 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.
Question 91 of 130
Substernal chest pain in a patient with anterior ischemic optic neuropathy (AION) caused by giant cell arteritis could indicate
what condition?
Hepatomegaly
Coronary arteritis
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.
Question 92 of 130
Which of the following therapies is preferred when managing non-arteritic anterior ischemic optic neuropathy (NAION)?
Question 93 of 130
Following injection of intravenous edrophonium (tensilon), what finding would most strongly suggest the diagnosis of
myasthenia gravis?
Nystagmus resolution.
Eyelid myokymia.
Please select an 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.
Question 94 of 130
When managing a patient with thyroid orbitopathy who has developed vision loss, what procedure or intervention should be
included?
Question 95 of 130
When considering a diagnosis of idiopathic intracranial hypertension (IIH or pseudotumor cerebri), treatment with what
antibiotic may be relevant?
Ciprofloxicin
Gentamicin
Fluconazole
Tetracycline
Please select an 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.
Question 96 of 130
A central scotoma in one eye and a superior temporal defect in the other suggests a lesion in what location?
Occipital lobe
Optic tract
Please select an 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.
Question 97 of 130
What is the structural basis for the pupillary light-near dissociation in Adie's tonic pupil?
More fibers innervate the ciliary body than the pupillary sphincter.
More fibers innervate the pupillary sphincter than the ciliary body.
The ciliary ganglion supplies the sympathetic innervation to the pupil and results in a smaller ipsilateral pupil.
Fibers innervating the pupil are aberrantly activated with contraction of the extraocular muscles.
Please select an 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.
Question 98 of 130
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?
Reflex hammer
Lumbar puncture
Dilute pilocarpine
Optokinetic drum
Please select an 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.
Question 99 of 130
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?
Patching
Neovascularization
Neuroretinitis
Please select an 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.
Question 101 of 130
Chronic treatment of giant cell arteritis with corticosteroids can affect mobility by causing what common systemic
complication?
Myopathy
Chronic arthritis
Cerebellar hemangioblastoma
Please select an answer
Feedback: Carotid cavernous fistulas are associated with retinal vein occlusion, presumably mediated by venous
congestion. Midbrain arteriovenous malformations can be associated with arteriovenous malformations in the retina
(Wyburn-Mason syndrome), but not with retinal vein occlusions. Retinal cavernous hemangiomas may hemorrhage but not
typically associated with retinal vein occlusion. Cerebellar hemangioblastomas are associated with retinal angiomas
(hamartomas), which are usually associated with dilated feeder artery(s) and vein(s).
Question 104 of 130
Which of the following conditions would be most likely to cause a homonymous hemianopia in a 83-year-old man?
Stroke
Mass lesion
Infection
Demyelination
Please select an answer
Feedback: While all of the entities listed may cause a homonymous hemianopia, stroke is by far the most common cause in
the elderly. The new onset of a homymous hemianopia, even in the absence of other symptoms, should prompt an urgent
evaluation for stroke in these patients.
How are the trochlear nerves (fourth cranial nerve) related to their origins (nuclei) and targets?
The fourth cranial nerve exits the brain stem ventrally and passes uncrossed to the superior oblique muscle
They decussate in the brainstem, exit ventrally and pass uncrossed to the superior oblique muscles.
The fourth cranial nerve exits the brain stem ventrally and crosses the midline ventral to the brainstem
They exit the brainstem dorsally, cross sides and pass to the superior oblique muscles.
The fourth cranial nerve decussates within the brainstem exits the brain stem dorsally and crosses to the contralateral
superior oblique muscle
They decussate within the brainstem, exit dorsally and pass uncrossed to the superior oblique muscles.
The fourth cranial nerve exits the brain stem dorsally and passes uncrossed to the superior oblique muscle
They exit the brainstem dorsally, cross sides and then pass to the superior oblique muscles.
The fourth cranial nerve is unique in that it decussates in the brainstem, so that the fourth nerve nucleus on the right gives
rise to the left fourth nerve fascicle. It is the smallest cranial nerve and has the longest course, making it prone to damage
with head trauma.
Question 106 of 130
A 40-year-old man has developed a central retinal vein occlusion (CRVO) in the right eye. He does not have systemic
hypertension or diabetes mellitus. What additional testing would have the highest likelihood to reveal an underlying
systemic cause?
Serum electrolytes
Genetic testing
Please select an answer
Feedback: Central retinal vein occlusion may be the initial manifestation of a hypercoagulable disorder, especially in
younger patients with no known history of vascular risk factors, such as hypertension or diabetes mellitus. The remaining
tests have little likelihood of detecting a related systemic condition.
For a patient with a unilateral internuclear ophthalmoplegia, in which field of gaze is the ocular misalignment largest?
Gaze ipsilateral to the brainstem lesion within the medial longitudinal fasciculus
Gaze contralateral to the brainstem lesion within the paramedian pontine reticular formation
Gaze contralateral to the brainstem lesion within the medial longitudinal fasciculus (MLF)
Gaze ipsilateral to the brainstem lesion within the paramedian pontine reticular formation (PPRF)
Please select an answer
Feedback: Internuclear ophthalmoplegia occurs with lesions of the MLF, which runs from the PPRF in the pons to the
midbrain to innervate the medial rectus subnucleus. The MLF decussates in the pons so that innervation from the right
PPRF travels to the left medial rectus third nerve subnucleus. Lesions of the PPRF cause an ipsilateral gaze palsy and not
internuclear ophthalmoplegia.
Papilledema (optic disc edema secondary to elevated intracranial pressure) may be associated with findings on
neuroimaging studies including flattening of the posterior portion of the globes and flattening of the pituitary gland with an
empty sella. Optic nerve sheath enhancement is expected with inflammation of the optic nerves or optic nerve
tumors. Hyperdensity consistent with calcium on computed tomography is suggestive of optic disc drusen. Absence of the
posterior pituitary bright spot can be seen in midline developmental abnormalities such as septo-optic dysplasia.
Torsional misalignment
Degree of esotropia
Please select an answer
Feedback: Double maddox rods are typically used to test for torsion. A virtual image, perpendicular to the plane of the
maddox rod, is seen at the source of light, held at distance, where the two rods should be parallel. In patients with abnormal
torsion, the rods are not parallel and the rods can be twisted to make them parallel. The resulting degree of torsion can then
be quantified.
Visual acuity
Color vision
Please select an answer
Feedback: Optic disc drusen most commonly cause visual field defects without affecting central visual acuity or color
vision. Photostress recovery may be abnormal in maculopathy but is not typically affected in optic neuropathy caused by
optic disc drusen.
Lyme disease
Behcet's disease
Syphilis
Please select an answer
Feedback: The incidence of syphilis is higher in those with HIV. Syphilis can cause virtually any type of intraocular
inflammation including vitritis, optic disc edema, retinitis, choroiditis, and vasculitis. Lyme disease, VKH, and Bechet's
disease are rare and not significantly increased in HIV-positive individuals.
Orbital cellulitis
Myositis
Demyelinating optic neuritis, the form associated with multiple sclerosis, commonly causes pain with eye movement.
Periventricular white matter lesions, typical for mutiple sclerosis, are common in optic neuritis and are the most important
risk factor for the development of subsequent neurologic dysfunction. Acutely the optic disc is normal in 2/3 of patients with
demyelinating optic neuritis, and marked hermorrhage and macular exudate are atypical.
Pupillary dysfunction from syphilis is characterized by light-near dissociation. Syphilitic pupils constrict better to a near
target than to direct light stimulation. Pharmacologic constriction with pilocarpine stimulates the iris constrictor muscle.
Therefore activity of the pupil is affected both at near and to light. The appearance of the iris stroma and sphincter may be
normal, or restricted by senechia, in both conditions.
Iris sphincter
Cavernous sinus
Ciliary ganglion
Midbrain
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Feedback: Tonic pupils are thought to result from lesions affecting the ciliary ganglion. These pupils react better to light than
near, are tonic to a near stimulus, and often are supersensitive to pilocarpine, constricting to 1/8%. Lesions of the midbrain
and cavernous sinus would be expected to cause a 3rd-nerve palsy, potentially with pupil involvement. Iris sphincter
damage would cause a sluggish pupil at light and near and would not be expected to constrict with 1/8% pilocarpine.
Thrombocytosis
Hypercalcemia
Erythrocytosis
Pseudotumor cerebri
Neurofibromatosis type II
Multiple sclerosis
Joubert's syndrome
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Feedback: This constellation of findings may be found in Usher's syndrome, but one would expect to find other features of
retinitis pigmentosa. Bilateral posterior subcapsular cataracts and decreased hearing may also be found in type II
neurofibromatosis, which has also been associated with bilateral acoustic neuromas. Epiretinal membranes may also be
present in these individuals. Joubert's syndrome is associated with ocular motor apraxia and cerebellar abnormalities.
Pseudotumor cerebri causes optic disc edema because of papilledema. Optic atrophy may be found late in the disorder, but
the other features mentioned are not found in pseudotumor cerebri. Multiple sclerosis is associated with inflammatory optic
neuropathy, but does not commonly affect hearing.
A 56-year-old woman presents with unilateral painful proptosis and diplopia. What additional finding would prompt
consideration of treatment with systemic steroids?
"S" Shaped lid margin profile and fullness of superior temporal orbit
Scintillating scotoma
6th-nerve palsy
Myasthenia gravis
Accomodative spasm
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Feedback:
Normal forced duction testing implies the problem is not due to restriction, making thyroid eye disease less likely.
Accomodative spasm is associated with pupillary miosis and variable esotropia. A 6th-nerve palsy is typically associated
with decreased saccades of the involved lateral rectus muscle. Myasthenia gravis does not typically involve the pupil and
does not cause restriction. The saccadic velocity is most commonly normal clinically, but can be impaired as more severe
ophthalmoplegia develops.
Compressive lesion
Midbrain stroke
Horner syndrome
Tertiary syphilis
Pharmacologic anisocoria
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Feedback:
A pupil that fails to react to light and near is suggestive of an iris sphincter problem. The unusual feature in this case is that
the affected pupil is the smaller one. Anisocoria greater in the dark suggests that the sphincter muscle is tonically constricted
in the right eye, and this is usually due to pharmacologic constriction from pilocarpine or another muscarinic anticholinergic
drug. Posterior synechiae would not be found in a normal examination. Horner syndrome does not cause a poorly reactive
pupil and would be expected to cause other findings such as ptosis. Syphilic pupils are typically bilateral, irregularly shaped,
and associated with light-near dissociation.
A pupil that fails to react to light and near is suggestive of an iris sphincter problem, even though that pupil is the smaller
one. Anisocoria greater in the dark is suggestive that the sphincter muscle is tonically constricted on the right, suggestive of
pharmacologic anisocoria with a muscarinic agent such as pilocarpine, which may also be causing accomodative spasm and
a brow ache, so that reassurance that the anisocoria will resolve is important and neuroimaging unnecessary. Horner
syndrome does not cause a poorly reactive pupil and would be expected to cause other findings such as ptosis, so that
pharmacologic testing would not be necessary. Syphilic pupils are typically bilateral, irregularly shaped, and associated with
light-near dissociation, so that serum testing would not be required.
Question 128 of 130
A 40-year old woman develops blurred vision at near in the left eye. She has a mid-dilated pupil that reacts only to near
stimulus. What is the most likely diagnosis?
Accommodative insufficiency
Adie's pupil
Accommodative spasm
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Feedback:
An isolated dilated pupil that reacts to near better than light is typically from a tonic (Adie) pupil. Loss of accomodation
frequently occurs with pupillary dilation. Accomodative insufficiency and accomodative spasm are not associated with
pupillary dilation. Third nerve palsy would be expected to have other features, including ptosis and an ocular misalignment.
Hypotony maculopathy
Vitritis
Systemic hypotension
Osteoporosis
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Feedback: Osteoporosis, systemic hypertension, cataracts, increased susceptibility to infection, and ocular hypertension
are all potential side effects of systemic corticosteroid therapy. Inflammation, including vitritis, would not be an expected side
effect.