Chapter 4 Neuro-Ophthalmology
4.1 Questions
Easy | Medium | Hard |
1. (Easy) A 43-year-old woman complains of blurred vision over the past month. Best corrected visual acuity is 20/50 in the right eye and 20/25 in the left eye. Photostress recovery test was found to be prolonged in the right eye at 50 seconds. What is the best next test?
Humphrey’s visual field 30–2
MRI of the brain and orbits
Retinal nerve fiber layer analysis
Optical coherence tomography (OCT) of the macula
2. (Medium) A 13-year-old adolescent girl is seen urgently for sudden vision loss. She was at school when she experienced painless loss of vision in both eyes. She states she can only see a bright light when it is shown in each eye. The pupils are equal, round, and reactive to light without afferent pupillary defect. Intraocular pressures are normal, as is the remainder of the anterior and posterior dilated examination. What test should be performed next?
Optokinetic testing
MRI of the brain and orbits
Fluorescein angiography
The Amsler grid testing
3. (Medium) A 32-year-old woman with past medical history of multiple sclerosis presents with blurred vision on her left side. Confrontation visual fields reveal a left homonymous hemianopia. MRI is obtained and reveals a right optic tract demyelinating lesion. Examination may have shown what other finding?
Right relative afferent pupillary defect
Left relative afferent pupillary defect
Right optic nerve head edema
Left optic nerve head edema
4. (Hard) A 63-year-old man is sent to you for preoperative evaluation of a known left parietal meningioma causing chronic headaches. What findings may be noted on examination?
An incongruous left homonymous hemianopia
A right superior quadrantanopia
An abnormal optokinetic response with the drum rotated to the right
An abnormal optokinetic response with the drum rotated to the left
5. (Hard) A 72-year-old man complains of right-sided vision loss after a cardiac surgery. Humphrey’s visual field is performed with the results shown below. An embolus to which artery most likely explains the findings?
Right middle cerebral artery
Right posterior cerebral artery
Left middle cerebral artery
Left posterior cerebral artery
6. (Easy) A 42-year-old man presents with the complaint that his vision being “off.” His visual acuity is 20/20 in both eyes. Pupils are equal, round, reactive to light, and without relative afferent pupillary defect. Intraocular pressures are normal. The anterior and dilated fundus examinations are within normal limits. Optical coherence tomography of the macula is performed and found to be normal. The patient is reassured that his eyes and vision are normal. The patient returns 6 months later with progressive vision loss in both eyes over the past few weeks. Examination shows a visual acuity of 20/60 in both eyes and a dense bitemporal hemianopia. MRI reveals a 15-cm sellar mass compressing the optic nerve chiasm. The mass is resected, but the patient is unable to recover his vision. What steps should have been taken to avoid this missed diagnosis?
Check color vision.
Check red desaturation.
Visual field testing.
All of the above.
7. (Hard) A 23-year-old woman presents to the emergency department with 2 days of blurred vision in the left eye. She has pain when performing extraocular movements. There is a 1+ relative afferent pupillary defect on the left. MRI is shown below. The remainder of the MRI is within normal limits. What is this patient’s risk of developing multiple sclerosis within the next 15 years?
23%
56%
74%
Cannot be determined from the given information
8. (Medium) A 34-year-old woman presents to the emergency department with 2 days of blurred vision in the right eye. She has pain when performing extraocular movements. There is a 1+ relative afferent pupillary defect on the right. Orbital MRI shows enhancement of the right optic nerve. Fluid-attenuated inversion recovery (FLAIR) sequence is shown below. What is this patient’s risk of developing multiple sclerosis by 15 years?
23%
56%
74%
Cannot be determined from the given information
9. (Easy) A 72-year-old hypertensive man presents to the emergency department with acute vision loss in the right eye upon awakening in the morning. He denies any headaches, jaw claudication, night sweats, fevers, or unintentional weight loss. Examination shows visual acuity of counting fingers in the right eye with a relative afferent pupillary defect. The optic nerve is diffusely edematous. The left eye has visual acuity of 20/25 with a healthy, cupless optic nerve. Which of the following is the next best step in this patient’s management?
Start topical brimonidine for neuroprotection.
Obtain complete blood count (CBC), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP).
Start oral prednisone 20 mg daily.
Reassure the patient with outpatient follow-up for automated visual field testing.
10. (Medium) A 58-year-old woman with past medical history of hypertension, diabetes mellitus type 2, and obstructive sleep apnea awoke with blurred vision in her inferior visual field from the right eye. Examination found a relative afferent pupillary defect in the right eye, superior segmental optic nerve edema with few peripapillary hemorrhages, and automated perimetry as shown below. Which of the following is not indicated for this patient?
Ensure blood pressure medication is not taken at night.
Obtain carotid Doppler ultrasound.
Order sleep study.
Control blood sugar level.
11. (Easy) A 54-year-old man complains of painless loss of vision from the right eye. He is in the postanesthesia care unit after having undergone a spinal fusion. Ophthalmic examination shows no light perception vision in the right eye with relative afferent pupillary defect on the right and otherwise normal examination. The below MRI is urgently obtained. What is the cause of this patient’s vision loss?
Ischemic infarction of the posterior orbital optic nerve
Embolic ophthalmic artery occlusion
Compressive retrobulbar hemorrhage
None of the above
12. (Medium) A 26-year-old man presents with blurred vision in both eyes. He states it began 2 weeks ago in the right eye, and 2 days ago in his left eye. Examination shows mild optic nerve edema bilaterally with multiple fine tortuous peripapillary vessels. After diagnosis, the patient should be referred for which of the following tests?
Carotid Doppler ultrasound
Echocardiogram
Chest CT
Electrocardiogram
13. (Easy) A 45-year-old man with past medical history of diabetes mellitus type 2, hypertension, and tuberculosis is seen for slowly progressive blurred vision in both eyes. Symptoms began 1 week ago. Color vision is decreased in both eyes. Visual acuity is 20/60 on the right and 20/50 on the left. Which of the following should be the next step?
MRI of orbits and brain.
Perform automated perimetry.
Review medications.
Check color vision.
14. (Hard) A 5-year-old child is seen for slowly progressive proptosis of the left eye. A relative afferent pupillary defect is noted. Imaging is obtained as shown below. Examination may also demonstrate which one of the following?
Flat hyperpigmented skin lesions
Hearing loss
Hypopigmented skin lesions
Facial capillary malformation
15. (Medium) A 46-year-old woman presents with progressive vision loss in the right eye. A trace relative afferent pupillary defect is noted. Imaging is obtained as shown below. Visual acuity is 20/30 in the affected eye. Humphrey’s visual field shows nonspecific changes. What is the best next step?
Biopsy
Surgical resection
Radiotherapy
Observation
16. (Medium) A 15-year-old adolescent boy is referred for optic nerve edema. Examination findings are shown in the images below. What is the most likely recommended treatment plan?
Obtain QuantiFERON gold and chest X-ray.
Order orbital MRI.
Reassurance.
Refer for genetic testing.
17. (Hard) A 6-month-old female infant is brought in as the parents do not feel their child is tracking objects well. A dilated fundus examination reveals bilateral optic nerve hypoplasia. MRI is obtained and shown below. What should be further evaluated in this patient?
CT chest
Electrocardiogram
Urinalysis
Endocrine evaluation
18. (Medium) A patient is found to have a serous macular detachment secondary to an optic pit. Management options include all of the following except which one?
Observation
Corticosteroids
Pars plana vitrectomy
Laser photocoagulation
19. (Medium) A 56-year-old overweight woman complains of worsening headaches over the past month. Examination demonstrates bilateral optic nerve edema. Imaging findings are shown below. What is the best treatment?
Intravenous antibiotics
Intravenous steroids
Anticoagulation
Diuresis
20. (Medium) A 34-year-old woman is sent to the emergency room for worsening headache and bilateral optic nerve edema. Imaging is obtained and shown. Lumbar puncture is performed with opening pressure of 32 cm H2O and normal cerebrospinal fluid analysis. What is the next best step in management?
Intravenous antibiotics
Intravenous corticosteroids
Anticoagulation
Diuresis
21. (Hard) A patient with Balint’s syndrome shows all of the following clinical signs except which one?
Simultagnosia
Hemineglect
Ocular apraxia
Optic ataxia
22. (Medium) You are seeing a 22-year-old woman with suspected right optic neuritis. A pocket watch is swung from its chain back in forth in a horizontal plane. The patient states the watch is moving in an elliptical path. What is this perception called?
Prosopagnosia
Ocular apraxia
The Pulfrich phenomenon
The Riddoch phenomenon
23. (Easy) A patient is blind due to bilateral occipital lesions. However, she insists that she is able to see the room. What is this patient exhibiting?
Anton’s syndrome
Charles Bonnet syndrome
Visual allesthesia
Metamorphopsia
24. (Hard) A 43-year-old woman is noted to have a relative afferent pupillary defect on the right side. The remainder of her ophthalmic examination is within normal limits, including visual field testing. Where is the lesion located?
Right optic nerve tract
Left optic nerve tract
Right pretectal nucleus
Left pretectal nucleus
25. (Easy) A 62-year-old man presents with double vision over the past week. Examination shows −4 abduction of the right eye. Also noted is anisocoria with the right pupil 2 mm smaller than the left, and margin to reflex distance 1 of 2 mm on the right and 4 mm on the left. Dilation lag of the right pupil in the dark is noted. There is no anhidrosis. Which one of the following is most likely the possible diagnosis?
Demyelinating brainstem lesion
Apical lung tumor
Internal carotid artery dissection
Intracavernous carotid aneurysm
26. (Medium) A 6-month-old female infant is seen for a droopy eyelid for the past week. You note a mild ptosis on the right along with anisocoria—the right pupil is smaller than the left. What is the next best step?
Discuss the likely benign etiology, and follow-up in 1 month.
Order an MRI of the brain, orbits, neck, and chest.
Order an MRI of the abdomen.
None of the above.
27. (Medium) A 46-year-old woman hospitalized for chronic obstructive pulmonary disease (COPD) exacerbation is seen for anisocoria. She has insulin-dependent diabetes mellitus and hypertension controlled with amlodipine. Her COPD has been under improved control on nebulizers. Examination shows visual acuity at near 20/20 on the right and 20/60 on the left. Pupils are round with the right pupil 4 mm in dark and 2 mm in light. The left pupil is 8 mm and nonreactive to both light and accommodation. Extraocular motilities are full, and the patient is orthophoric. Anterior examination is within normal limits. What is the most likely cause of anisocoria?
Pharmacological
Adie’s tonic pupil
Third cranial nerve palsy
Traumatic mydriasis
28. (Hard) A 34-year-old 30-week gravid woman with history only significant for headaches comes to the emergency room for sudden onset of severe headache this morning. She also states she has double vision. Examination shows anisocoria with the left pupil larger than the right, and a bigger difference in light than dark. There is left ptosis and limited adduction, supraduction, and infraduction. Which of the following is the next best step?
Pharmacological pupil testing
Lumbar puncture
MRI of the brain
Intravenous corticosteroids
29. (Medium) A 62-year-old man presents with diplopia for the past month. Examination demonstrates a right hypertropia consistent with right fourth cranial nerve palsy. Which of the following is most consistent with the diagnosis?
Right hypertropia worse in right gaze and right head tilt
Right hypertropia worse in right gaze and left head tilt
Right hypertropia worse in left gaze and right head tilt
Right hypertropia worse in left gaze and left head tilt
30. (Easy) A 43-year-old woman presents with proptosis and double vision that began 2 months ago. MRI was performed as seen below. What is the most likely diagnosis?
Inflammatory myositis
Thyroid eye disease
Orbital cellulitis
None of the above
31. (Easy) A 35-year-old man presents with acute-onset right eye pain with vertical diplopia, worse in left gaze. Examination shows poor elevation of the right eye when looking up and to the left. Inflammation of which muscle’s tendon is responsible for these findings?
Superior rectus
Inferior rectus
Superior oblique
Inferior oblique
32. (Easy) A 73-year-old woman presents with 2 weeks of double vision that comes and goes. Examination shows normal visual acuity, full extraocular motilities, and orthophoria. There is mild keratopathy. You recommend artificial tears and a follow-up in 1 month. She returns in 1 week with count finger vision in the right eye. What testing should have been performed at the initial visit?
Acetylcholine receptor antibodies
C-reactive protein
Thyroid-stimulating immunoglobulin
Antineutrophil cytoplasmic antibody
33. (Medium) A 46-year-old man presents with 1 month of slowly developing enophthalmos on the right. What is the most likely finding on imaging?
Agenesis of the sphenoid wing
Hypertrophied extraocular muscles
Atrophy of the maxillary sinus
Orbital fat stranding
34. (Hard) A 56-year-old man complains of difficulty with reading. When he attempts to read the newspaper, everything appears doubled. Examination shows bilateral ptosis and severely decreased extraocular movements in all directions. What testing must be obtained in this patient?
MRI of the brain and orbits
CT chest
Renal ultrasound
Electrocardiogram
35. (Medium) A 43-year-old man presents with blurred vision in both eyes. You note the patient has frontal balding, a long face, and temporal wasting. You shake his hand, but he is unable to immediately release. Examination reveals bilateral ptosis and reduced extraocular motility. Slit lamp examination reveals a cataract. What type of cataract is likely in this patient?
Oil droplet
Christmas tree
Sunflower
Cerulean
36. (Hard) A 72-year-old French Canadian woman presents with progressive droopy eyelids. Examination reveals bilateral ptosis and severely restricted extra-ocular movements. This patient should undergo what type of testing?
Sleep study
Barium swallow
Urinalysis
Lumbar puncture
37. (Hard) A 46-year-old woman presents for evaluation of droopy eyelids. She states it started about 1 year ago and is worse in the evening. Examination shows ptosis with margin to reflex distance 1 of 1 mm on the right and 2 mm on the left. When the patient is asked to maintain upgaze, the eyelids begin to descend around 30 seconds. When looking down and looking straight ahead, the eyelids appear to overshoot before settling back in their prior position. Myasthenia gravis is suspected. You note no systemic signs or symptoms. Acetylcholine receptor antibodies are ordered, but all return negative. What should the patient be told?
Acetylcholine receptor antibodies are positive in up to 90% of patients. There is a small chance you have myasthenia gravis.
Your acetylcholine receptor antibodies are normal; you may proceed with ptosis surgery.
Acetylcholine receptor antibodies are positive in up to 50% of patients. There is still a chance you have myasthenia gravis.
None of the above.
38. (Hard) What additional testing should a patient with ocular or systemic myasthenia gravis undergo?
CT chest
MRI of the brain and orbits
Urinalysis
Electrocardiogram
39. (Hard) A 34-year-old man complains of double vision over the past week worse in left gaze. He also complains of headaches over the past month and intermittent graying out of his vision when he bends over. What would you expect to find on further testing?
Ischemic lesion in posterior midbrain
Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
Elevated intracranial pressure
Thickened left lateral rectus
40. (Medium) A 20-year-old woman presents for double vision in both left and right gaze since childhood. She has no other complaints. Examination shows decreased abduction of the right eye in right gaze and decreased abduction of the left eye in left gaze. Examination additionally reveals decreased orbicularis strength and a flattened smile bilaterally. What other finding may be discovered on examination?
Eyelid ptosis
Light-near dissociation
Papilledema
Difficulty hearing
41. (Medium) A 43-year-old woman states she has difficulty focusing and pain. The episodes started 2 weeks ago and are intermittent. You ask the patient to follow an object. As you move to the left, you note her left eye does not abduct, rather moves toward her nose along with the right eye. What finding hints toward a nonorganic disorder?
Retraction during convergence
Miosis during convergence
Limitation in upgaze
Tonic medial deviation of the left eye
42. (Easy) Which of the following is true about the fourth cranial nerve?
Exits the midbrain ventrally, stays ipsilateral, and enters the orbit outside the annulus of Zinn.
Exits the midbrain dorsally, stays ipsilateral, and enters the orbit outside the annulus of Zinn.
Exits the midbrain dorsally, crosses contralaterally, and enters the orbit outside the annulus of Zinn.
Exits the pons dorsally, crosses contralaterally, and enters the orbit inside the annulus of Zinn.
43. (Hard) A 76-year-old hypertensive man presents with acute onset of double vision. MRI is performed and shows an area of restricted diffusion in the right midbrain at the level of the superior colliculus. What examination findings may be noted?
Compensatory right head tilt
Compensatory left head tilt
Right limitation in supraduction, infraduction and adduction, and bilateral ptosis
Right limitation in infraduction and adduction, bilateral ptosis, and supraduction deficit
44. (Easy) A 68-year-old diabetic woman complains of double vision over the past 3 days with left-sided headache. Examination demonstrates left-sided ptosis with decreased supraduction. All other extra-ocular movements appear intact. Pupils are equal, round, and reactive to light and accommodation. What is the next best step?
Obtain contrasted MRI of the brain and orbits and MRA.
Reassure of likely ischemic nature, and follow-up in 1 month.
Reassure of likely ischemic nature, and follow-up in 1 day.
Either A or C.
45. (Hard) An 86-year-old man complains of double vision. Examination is consistent with a right complete third nerve palsy. Complete neuro-ophthalmic examination also reveals left-sided tremor. The patient states this is new since the double vision started. MRI reveals ischemia in the right midbrain. Which syndrome does this patient have?
Weber’s syndrome
Benedikt’s syndrome
Claude’s syndrome
Nothnagel’s syndrome
46. (Medium) A 54-year-old woman comes to the emergency room with sudden onset of double vision and severe right-sided eye pain. Examination reveals a right dilated and poorly reactive pupil, right-sided ptosis, and decreased supraduction, infraduction, and adduction on the right. MRI and MRA are found to be negative. What is the next best step?
Conventional angiography
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
Lumbar puncture
Acetylcholine receptor antibodies
47. (Medium) A 46-year-old woman develops severe right-sided pain and double vision. Examination shows complete right ophthalmoplegia and ptosis with a dilated pupil. Imaging should be directed to which of the following?
Orbital apex
Cavernous sinus
Brainstem
None of the above
48. (Easy) A 7-year-old girl complains of sudden-onset double vision. Examination demonstrates a right-sided pupil-involving third nerve palsy. MRI is found to be normal. What is the next best step?
MRA
CTA
Conventional angiography
Lumbar puncture
49. (Hard) A 36-year-old man is hospitalized for ataxia and double vision. Examination shows bilateral ophthalmoplegia with dilated and poorly reactive pupils. The facial muscles are bilaterally weak. Deep tendon reflexes are absent. What will testing show?
Elevated cerebrospinal fluid (CSF) protein
CSF pleocytosis
Decreased CSF glucose
All of the above
50. (Hard) A 50-year-old man presents to the emergency room after being found on the ground outside. The patient is unkempt. He is unsure of where he is, and needs to be told that he is at the hospital after collapsing multiple times. Examination shows a bilateral vertical ophthalmoplegia and horizontal gaze-evoked nystagmus. Upon having the patient stand up and walk, he is noted to be profoundly ataxic. MRI is performed and finds increased T2 signal in bilateral mammillary bodies. What is the most appropriate treatment?
Intravenous antibiotics
Intravenous steroids
Vitamin B 1
Vitamin B 12
51. (Hard) A 36-year-old woman presents with difficulty looking to the left for the past few days. Examination shows normal extraocular movements in all fields of gaze when the patient is asked to follow a finger. However, the patient is unable to look from the examiner to a stationary finger to the patient’s left. Looking at a finger in other fields of gaze is normal. MRI is consistent with an inflammatory lesion. Where is the lesion located?
Left sixth nerve nucleus
Left paramedian pontine reticular formation
Right medial longitudinal fasciculus
Right third nerve nucleus
52. (Easy) A 25-year-old woman presents with intermittent double vision over the past few days. Double vision only occurs when looking to the left. Examination finds a right hypertropia that is worse in right gaze and right head tilt. Gaze to the right is full. Gaze to the left finds a slowed saccade of the right eye with mild limitation, as well as a horizontal-beating nystagmus of the left eye. Where is the patient’s lesion located?
Left frontal lobe
Right fourth nerve nucleus
Left paramedian pontine reticular formation
Right medial longitudinal fasciculus
53. (Medium) A 76-year-old man presents with decreased eye movements since the morning. Examination demonstrates an inability to perform saccades or smooth pursuit to the right. In left gaze, the right eye slowly adducts while the left eye has an abducting nystagmus. MRI finds a hemorrhage involving which area?
Right sixth nerve nucleus
Right paramedian pontine reticular formation
Right medial longitudinal fasciculus
Both A and C
54. (Medium) A 70-year-old man complains of difficulty reading. Examination shows an increased near point of convergence. What other findings might be seen on examination?
Internuclear ophthalmoplegia
Movement-related tremor
Decreased vertical eye movements
None of the above
55. (Easy) A 54-year-old woman presents with trouble seeing with her glasses. She has seen multiple eye doctors, and had multiple pairs of glasses prescriptions filled. Examination finds mild presbyopia with her current glasses. Her corrected visual acuity is 20/20 in each eye. She states that with both eyes images seem to be doubled. Extraocular motility is normal. There is a comitant 12 prism diopter esotropia at distance. She is orthophoric at near. What is the next best step?
Reassurance to likely benign and idiopathic etiology.
Prescribe prism glasses.
Order MRI brain.
All of the above.
56. (Hard) An 86-year-old hypertensive woman is seen with her eyes deviated to the left. She has no other complaints or findings. MRI would most likely show an ischemic focus in which of the following?
Right frontal cortex
Left frontal cortex
Right brainstem
Left brainstem
57. (Easy) A 46-year-old man is referred for neuro-ophthalmic evaluation for a known mass compressing the dorsal midbrain. Which finding is not likely to be seen on examination?
Supranuclear upward gaze palsy
Convergence-retraction nystagmus on attempted upgaze
Bilateral ptosis
Light-near dissociation on pupillary examination
58. (Hard) A 26-year-old man is seen in the hospital 1 week after having suffered a massive trauma. He has been unable to move his limbs or speak. He is able to look up and down, but unable to look laterally. Eyelid function is normal. MRI shows a large area of ischemia in which of the following?
Frontal cortex
Thalamus
Midbrain
Pons
59. (Easy) A 36-year-old woman is found to have a carotid-cavernous fistula (CCF) on MRI. Which of the following is expected on examination?
“Corkscrew” dilated conjunctival vessels
Elevated intraocular pressure
Proptosis
All of the above
60. (Easy) A 42-year-old man presents with 2 weeks of intermittent eye bulging. He works as a mail carrier, and states his eye bulges when he picks up heavy packages. It goes away as soon as the activity stops. What is expected to be seen on further testing?
An audible periorbital bruit
Pulsatile proptosis
Dilated orbital varix on CT during the Valsalva maneuver
Dilation and thrombosis of the cavernous sinus
61. (Medium) A 3-month-old female infant is brought in by her parents after noting shaking eyes for the past week. Examination shows a horizontal nystagmus with a mixed rotary and pendular nystagmus. Nystagmus appears to dampen the most in left gaze. You diagnose an infantile nystagmus. Which of the following is not fitting with this diagnosis?
A normal optokinetic testing response
Abolished during sleep
Retinal dystrophy noted on examination
Diminished in convergence
62. (Medium) A 56-year-old man presents with a droopy eyelid as seen in the figure below. Further testing is performed and shown in the following two panels of the figure. What is the next best step?
Obtain MRI of the brain and orbits.
Offer surgical repair of levator dehiscence.
Obtain MRA.
Check proximal muscle strength.
63. (Medium) A 6-month-old male infant is found to have spasmus nutans. Which of the following is not true of this condition?
MRI is recommended for common anterior visual pathway gliomas.
It is benign and self-limiting with resolution in a few months.
Head nodding is typically seen.
Torticollis is present.
64. (Easy) A 34-year-old woman with insulin-dependent diabetes mellitus, hypertension, and bipolar disorder is seen for a sensation of things moving around, especially when reading. Examination finds a downbeat nystagmus in primary position that is worse in downgaze and diminished in upgaze. Which step is most likely to help this patient?
Discontinuation of lithium
Discontinuation of atenolol
MRI of the midbrain
Base-up prisms in reading glasses
65. (Hard) A 44-year-old woman with multiple sclerosis is seen with a right jerk horizontal nystagmus. Two minutes later, you notice she has a left jerk horizontal nystagmus. What will most likely help this patient?
Start clonazepam.
Start baclofen.
B 12 supplementation.
Iron supplementation.
66. (Hard) A 53-year-old man develops oscillopsia and vertigo over the past month. Nystagmus is noted on neuro-ophthalmic examination. MRI is obtained and shown below. What type of nystagmus is seen?
High frequency, low-amplitude nystagmus in left gaze
Low-frequency, high-amplitude nystagmus in left gaze
Periodic alternating nystagmus
Monocular horizontal jerk nystagmus in abduction with adduction deficit of the fellow eye
67. (Medium) A 43-year-old woman is referred for nystagmus evaluation. An MRI is obtained and shown below. What type of nystagmus is expected?
Brun’s nystagmus
Periodic alternating nystagmus
Seesaw nystagmus
Dissociated jerk nystagmus
68. (Easy) What type of lesion would be expected to cause the visual field defect shown below?
Left optic tract inflammation
Left occipital lobe ischemia
Sellar mass
Left temporal lobe mass
69. (Medium) A 62-year-old man is seen with a vertical pendular nystagmus. MRI is performed and shows an old area of infarct at the inferior olivary nucleus. What else may be noted on examination?
Orthostatic hypotension
Internuclear ophthalmoplegia
Resting tremor of the upper extremities
Tremor of the palate
70. (Hard) A 36-year-old man is seen in the emergency room after being found poorly responsive outside. The patient has become more responsive but is confused. Examination notes pendular convergence and divergence eye movements. Supraduction is limited bilaterally. A rhythmic motion of the jaw is also noted. What is the best treatment for this patient?
Intravenous corticosteroids
Intravenous mannitol
Intravenous ceftriaxone
Intravenous heparin
71. (Easy) A 42-year-old woman complains of peripheral vision loss. She also feels uneasy when she is walking, especially down long corridors or aisles at the grocery store. The Goldmann visual field is obtained and shown below. What is the most likely cause of her loss in peripheral vision?
Glaucoma
Retinitis pigmentosa
Occipital lobe ischemia
Nonorganic vision loss
72. (Medium) A 25-year-old man complains of blurred vision in the left eye. Fundus findings are shown below. The right eye is normal. What is the next best step?
Obtain optical coherence tomography (OCT).
Perform B-scan ultrasound.
Obtain CT of the brain and orbits.
Obtain MRI of the brain and orbits.
73. (Hard) A 36-year-old man is seen for glare when he is driving at night. A previous MRI is reviewed and shown below. What is the most likely cause for this patient’s complaint?
Exposure keratopathy
Anterior uveitis
Nuclear sclerotic cataracts
Posterior subcapsular cataracts
74. (Medium) A 63-year-old woman presents with gradual proptosis and vision loss. MRI is performed and shown below. Which of the following is true regarding this type of lesion?
Incidence is higher in males than females.
They originate from arachnoid cells.
There is an increased risk of these lesions in patients with neurofibromatosis type 1.
Lesions are responsive to steroids, but tend to recur.
75. (Easy) A 60-year-old woman presents with progressive prominence of the right eye over the last 6 months. Examination shows right-sided proptosis. Imaging is obtained and shown below. Which of the following statements is true regarding this lesion?
A dural tail on MRI helps in diagnosis of this lesion.
The lesion arises from a branch of the external carotid artery.
This is the most common location for presentation of these lesions.
The involved vessel is inferior to the optic nerve.
76. (Hard) A 72-year-old man with diabetes mellitus type 2 and coronary artery disease complains of blurred vision that has not resolved after an episode of sudden-onset right-sided hemiparesis, dizziness, and blurred vision. MRI is obtained and shown below. What type of visual field deficit is expected?
No visual field deficit
Complete right homonymous hemianopia
Right homonymous hemianopia sparing the vertical meridian
Right inferior quadrantanopia sparing the horizontal meridian
77. (Hard) A 45-year-old man is seen for painless progressive vision loss in the left eye. MRI is obtained and shown below. Which feature would help to differentiate this lesion from meningioma?
No dural tail compared to meningioma
Higher incidence in males compared to females for meningioma
Diffusely enhances with gadolinium compared to meningioma
None of the above
78. (Hard) A brain mass was biopsied, and pathology is shown below. Which of the following is not true regarding this lesion?
Composed of neoplastic small B-cell lymphocytes
Negative for markers CD5 and CD10, but positive for CD23
Associated with chronic inflammation
Associated with immunosuppression
79. (Medium) A 63-year-old man presents with double vision. His extraocular movements are shown below. These findings are least consistent with which diagnosis?
Ischemia
Aneurysmal compression
Myasthenia gravis
Idiopathic intracranial hypertension
80. (Easy) A 56-year-old woman presents with 2 months of increased thirst, frequent urination, and bilateral proptosis. MRI is performed and shown below. What is the next best step in management?
Intravenous corticosteroids.
Intravenous antibiotics.
Radiation therapy.
Obtain orbital biopsy.
81. (Hard) An orbital mass was biopsied from the right side of a patient with bilateral proptosis. The pathology is shown below. What is the most accurate statement regarding this disorder?
First-line therapy is surgical debulking with adjuvant radiotherapy.
The disease is curable with excellent long-term prognosis.
More than 50% are positive for the BRAF V600E mutation.
Tumors are composed of neoplastic Langerhans’ cells.
82. (Easy) A 53-year-old diabetic woman returns for followup 3 months after an episode of pupil-sparing third nerve palsy. You note miosis on adduction of the affected eye. Which of the following is the next best step?
Reassurance of ischemic cause secondary to diabetes.
Check acetylcholine receptor antibodies.
Order MRI or MRA.
Order carotid Doppler ultrasound.
83. (Medium) A 36-year-old man presents with 3 months right-sided periocular and perioral twitching. Which of the following is the best next step?
Imaging
Chemodenervation
Reassurance to self-resolving course
None of the above
84. (Easy) A 26-year-old woman presents for further evaluation of headaches that began a few years ago. Headaches start with a scintillating scotoma that resolves after 20 minutes and is followed by a severe headache that resolves after 10 hours. Which description would prompt further imaging?
Nausea and vomiting
Intense photophobia
Ptosis and miosis ipsilateral to headache
Sudden-onset headache after exercise
85. (Medium) A 53-year-old man develops right-sided neck pain. Examination demonstrates right-sided ptosis with margin to reflex distance 1 of 1 mm on the right and 3 mm on the left. There is also anisocoria with the right pupil smaller than the left. The anisocoria is more prominent in dark than light. Imaging is obtained and shown below. What is the most appropriate treatment?
Resection of mass
Anticoagulation
Intravenous corticosteroids
Observation
86. (Easy) A 62-year-old woman develops binocular double vision and difficulty closing her eye. Examination reveals a left-sided facial nerve palsy. MRI is obtained and shown below. What is causing the double vision?
Severe exposure keratopathy
Third cranial nerve palsy
Internuclear ophthalmoplegia
Sixth cranial nerve palsy
87. (Medium) A 26-year-old man suffers a motor vehicle accident with vision loss to light perception in the right eye. There is a right relative afferent pupillary defect. Examination is otherwise within normal limits. CT is performed and found to be normal. What is the best statement to tell the patient?
Surgical decompression of your optic canal is indicated and should lead to improved vision.
IV corticosteroids should be started and will improve your vision compared to observation.
No known treatment will definitely improve your vision. We will observe.
None of the above.
88. (Medium) A 72-year-old man presents with right-sided proptosis, periorbital erythema, and ocular injection. Imaging is performed and shown below. Visual acuity is 20/20 and the remainder of the examination is within normal limits. A biopsy is performed with immunoglobulin G4 (IgG4) stain shown below. Which of the treatments is not appropriate for this patient?
Orbitotomy for mass excision
Corticosteroids
Rituximab
Radiation therapy
89. (Medium) A patient with optic neuritis should not be treated with which of the following modalities?
Oral prednisone dosed at 1 mg/kg
Intravenous methylprednisolone dosed at 1g/d
Rituximab
Intravenous immunoglobulin
90. (Easy) A 34-year-old woman is seen with right facial paralysis and pain in her ear. Examination shows an erythematous vesicular rash involving the right pinna. What is the best management for this patient?
Obtain MRI of the brain and orbits with gadolinium.
Start oral antibiotics.
Obtain lumbar puncture.
Start steroids and antivirals.
91. (Medium) A 36-year-old man complains of recent-onset double vision. The patient has a left head tilt. The double vision is worse when looking to the left. Which of the following would point to a more benign etiology for the patient’s double vision?
Uncontrolled hypertension and diabetes
Large fusion amplitudes
New onset of headaches
No head tilt in old photographs
92. (Easy) The patient below is seen for double vision. Which of the following tests will not help in determining the cause?
CT scan of the orbits
Acetylcholine receptor antibodies
Lumbar puncture for opening pressure
Thyroid-stimulating immunoglobulin
93. (Easy) A 15-year-old adolescent boy has a CT scan for double vision in upgaze as shown below. What is the best treatment for this patient?
Corticosteroids
Thyroidectomy
Pyridostigmine
Orbital surgery
94. (Medium) A 62-year-old woman complains of slowly darkening vision on the right side. She has no other complaints. Examination shows visual acuity of 20/60 on the right and 20/25 on the left. There is a trace relative afferent pupillary defect on the right. Examination demonstrates trace anterior chamber cell and flare. Fundus examination shows moderate dot blot hemorrhages and trace optic nerve edema. Lab studies show erythrocyte sedimentation rate (ESR) of 22 and C-reactive protein (CRP) of 1.0. Carotid Doppler ultrasound finds stenosis of 73% on the right. Blood pressure and serum glucose are within normal limits. What is the best management for this patient?
Start intravenous corticosteroids.
Refer for carotid endarterectomy.
MRI of the orbits with contrast.
None of the above.
95. (Hard) A 10-year-old girl is seen for a failed vision screen at school. Examination demonstrates bilateral visual acuity of 20/200. Genetic testing finds a mutation of OPA1. All of the following are true of this disorder except which one?
Prognosis for final visual acuity ranges from 20/200 to 20/400.
Optic nerve examination will show bilateral temporal to diffuse atrophy.
Transmission is autosomal dominant.
Most patients present in the first decade of life.
96. (Hard) A 12-year-old girl with insulin-dependent diabetes mellitus is seen for progressive vision loss. Ophthalmic examination is only significant for bilateral optic nerve atrophy. All of the following are true of this disorder except which one?
About 50% of patients also have diabetes insipidus.
About 10% of patients have sensorineural hearing loss.
Inheritance is autosomal recessive.
A mutation of WFS1 or WFS2 causes this condition.
97. (Easy) A 15-year-old adolescent boy with worsening ataxia is seen with ophthalmic examination only revealing conjunctival telangiectasia. Imaging in this syndrome should reveal which of the following?
Midbrain atrophy
Cerebellar atrophy
Optic nerve atrophy
No atrophic changes
98. (Medium) A 20-year-old woman with history of seizures since childhood is noted to have angiofibromas in the malar area. MRI shows bilateral subependymal nodules. Which of the following is not true of this syndrome?
Half of patients have learning difficulties.
Angiomyolipomas of the kidneys may cause hematuria.
Astrocytic hamartomas are the only ocular involvement.
Inheritance is in an autosomal dominant pattern.
99. (Hard) An 18-year-old man with history of photocoagulation to a retinal angioma 1 year ago comes in for new headaches over the past month. Examination shows new bilateral optic disc edema. MRI of the brain and orbits shows a vascular cerebellar lesion. Which test is not indicated in this condition?
Abdominal ultrasound
Skin biopsy
Urinary catecholamine metabolites
MRI spinal cord
100. (Hard) A 34-year-old woman presents with severe, shooting pain down the right side of her face. Examination reveals atrophy of the right side of the face with enophthalmos and a collapsed malar area. There is linear scar extending from the forehead to the midface on the right side. Which of the following statements regarding this syndrome is incorrect?
Trauma is the underlying mechanism.
It is considered to be a variant of scleroderma.
Horner’s syndrome may be seen in some patients.
May be associated with ipsilateral body hemiatrophy.
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