Neuroophthalmology Flashcards

1
Q
  1. The horizontal gaze center is formed by
    which one of the following:
    a. Pontine paramedian reticular formation
    b. Reticular medial longitudinal fasciculus
    c. Preganglionic Erdinger-Westphal nucleus
    d. Brodman Area 6
    e. Superior colliculus
A

a. Pontine paramedian reticular formation

Gaze is a complex eye movement that is mainly controlled by the combination of CNs III, IV, and VI. Gaze can be simply classified as lateral, vertical, or conjugate. The frontal eye field (Area 8) and supplementary eye field (Area 6) are regions of the cerebral cortex that control gaze, and they innervate the contralateral paramedian pontine reticular formation (PPRF). Fibers from the PPRF reach the ipsilateral CN VI nucleus and contralateral CN III nucleus through the contra- lateral MLF to control lateral gaze. Lateral gaze palsies are classified as internuclear ophthalmo- plegia (INO), lateral gaze palsy, or one-and-a- half syndrome, and the corresponding lesions are located in the MLF, PPRF, and both MLF and PPRF, respectively. In INO, lesions in the
MLF cause ipsilateral eye adduction failure. The contralateral eye can abduct, but with nys- tagmus. In lateral gaze palsy, lesions in the PPRF cause ipsilateral eye abduction and contralateral eye adduction failure. Lateral gaze to the contra- lateral side is preserved in these cases. Lesions in both the MLF and the PPRF result in ‘one- and-a-half’ syndrome with ipsilateral eye abduction and adduction failure and contralateral eye adduction failure. The contralateral eye can abduct, but with nystagmus. The control center for vertical gaze is located at the rostral interstitial nucleus of the MLF, near the CN III nucleus in the midbrain. In addition, it has been suggested that a center for convergence is located near CN III nucleus. A lesion in the midbrain can cause vertical gaze palsy and convergence palsy, and this is known as Parinaud syndrome.

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2
Q

A 25-year-old woman presents with a several
week history of diplopia, with an 8 month history of generalized headache which has been
particularly bad over the last 2 months. On
examination sheis obese, has a normal pupillary
light reflex and no RAPD. Appearance of
fundi are shown. Which one of the following
is most likely cause of her complaint?
a. Non-organic disorder
b. Optic nerve drusen
c. Optic neuritis
d. Idiopathic intracranial hypertension
e. Leber’s hereditary optic neuropathy

A

d. Idiopathic intracranial hypertension

the optic nerve head and usually results in bilateral optic disc swelling. The optic disc may be markedly elevated, with hemorrhages of the nerve head and surrounding retina. Usually, there is no visual loss acutely, except for enlargement of the physiologic blind spot from elevation and compression of the peripapillary retina. Chronic papilledema can cause loss of the peripheral visual field, with central vision
affected only very late in its course. IIH typically occurs in obese women between puberty and men- opause. Men and non-obese women frequently harbor identifiable causes of elevated intracranial hypertension (e.g. Chiari malformation, occult dural arteriovenous malformation, dural venous sinus occlusion, sleep apnea) and should not be con- sidered to have IIH until an exhaustive clinical investigation confirms that there is no identifiable cause. Fundoscopic features of papilledema are highlighted below.

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3
Q

A 50-year-old man complains of 2 days’ double vision. Has abducting nystagmus in the
right eye and vertical gaze is preserved bilaterally. Which one of the following is most
likely?
a. Left internuclear ophthalmoplegia
b. Orbital apex syndrome
c. Left incomplete oculomotor palsy
d. Superior orbital fissure syndrome
e. Left one-and-a-half syndrome

A

a. Left internuclear ophthalmoplegia

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4
Q

A 53-year-old woman reports drooping of her eyelids for the last 6 months. She experiences diplopia when driving for any extended period of time. Which one of the following is most likely?

a. Cavernous sinus thrombosis
b. Myasthenia gravis
c. Oculomotor nerve palsy
d. Pituitary apoplexy
e. Thyroid ophthalmopathy

A

b. Myasthenia gravis

Ocular myasthenia gravis can mimic an isolated or combined neuropathy of CN III (except pupil involvement), IV, or VI, but often presents with diplopia or ptosis that is not easily categorized. Ocular involvement is seen early and may be the only system affected in some patients, with an inability to forcefully close their eyes against resis- tance. Patients frequently report worsening symp- toms as the day progresses and relief after a nap or rest. The diagnosis is sometimes difficult to make because the results of edrophonium chloride (Tensilon) testing, serum acetylcholine receptor antibody assay, repetitive electromyographic nerve stimulation, or trials of pyridostigmine bro- mide (Mestinon) may be equivocal. The ice test is also a useful screen. Single-fiber electromyogra- phy has the greatest sensitivity and specificity but is technically difficult. CT of the thorax is per- formed to exclude a thymoma.

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5
Q

Forty minutes after bilateral instillation of
10% cocaine eyedrops, the left pupil dilates,
but the right does not.
a. Horner’s syndrome
b. Myasthenia gravis
c. Oculomotor palsy
d. Trochlear palsy
e. Thyroid ophthalmopathy

A

a. Horner’s syndrome

Right Horner’s syndrome with right ptosis, miosis and facial anhidrosis due to right lateral medullary infarction. Relative miosis and a slight ptosis (1-2 mm) may result from interruption of the oculosym- pathetic pathway anywhere along the three-neuron chain: from the hypothalamus to the ciliospinal center of Budge-Waller (C8–T1), across the lung apex to the superior cervical ganglion, or from the superior cervical ganglion by means of the carotid plexus sympathetic nerves to the pupillary dilator. Postganglionic interruption (at or distal to the superior cervical ganglion) is commonly benign or idiopathic, but preganglionic or central oculosympathetic pareses are associated with malignancy in about half the cases.
Pharmacologic evaluation in Horner’s syndrome
(1) Apraclonidine0.5%isadirectalpharecep- tor agonist. It does not cause pupil dilata- tion in the presence of an intact sympathetic innervation, but will dilate any pupil with sympathetic denervation. In Horner’s it will thus reverse the aniso- coria and elevate the ptotic lid.
(2) Cocaine 10% dilates the normal (non- Horner’s) pupil by blocking the reuptake of norepinephrine into sympathetic nerve endings producing a prolonged mydriasis over 45 min. Due to the lack of norepi- nephrine in sympathetic denervated pupil (Horner’s) blocking reuptake does not produce a mydriasis. Persistence/wors- ening of anisocoria establishes the diagnosis.
(3) Hydroxyamphetamine actively releases norepinephrine from adrenergic nerve endings to dilate a normal pupil. In Hor- ner’s syndrome due to first- or second- order neuron damage, intact third-order neurons will respond to hydroxyampheta- mine as those in a normal pupil and dilate. However, with Horner’s due to a third- order (postganglionic) lesion, the damaged nerve endings cannot release norepineph- rine and the pupil will not dilate.

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6
Q

A 22-year-old female complains of reduced
vision and pain in the left eye starting 3 days
ago. On examination vision is normal in the
right eye but 6/12 (20/40) in the left eye, Left
relative afferent pupillary defect, pain when
looks to right orleft, no redness or photophobia.
a. Arteritic anteriorischemic optic neuropathy
b. Central retinal artery occlusion
c. Iritis
d. Optic neuritis
e. Orbital cellulitis

A

d. Optic neuritis

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7
Q

A 34-year-old man present with intermittent diplopia, worse when looking to the left. Exa- mination findings are shown below. Which one of the following is most likely?
a. Left abducens nerve palsy
b. Left conjugate gaze paresis
c. Left internuclear ophthalmoplegia
d. Left hypotropia
e. Right trochlear palsy

A

a. Left abducens nerve palsy

Trauma is a common cause of abducens palsy in all age groups. Acute, painful abducens paresis in a patient 45 years or older who has hypertension or diabetes suggests an ischemic cranial mono- neuropathy. Recovery over a period of 2-6 months is the rule. In young patients, abducens palsies may occur as a postviral syndrome, but the clinician must remain alert for the possibility of tumor. Elevation of intracranial pressure can affect the function of one or both VI nerves.

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8
Q

On examination, this 15-year-old boy was found to have upgaze paresis, ocular tilt reaction (right superior rectus skew devia- tion and head tilt), papilledema, and aniso- coria. The pupils were moderate in size and poorly reactive to light, but reactive to near stimuli.
a. Adie’s tonic pupil
b. Argyll Robertson pupil
c. Horner syndrome
d. Parinaud syndrome
e. Wallenberg syndrome

A

d. Parinaud syndrome

Dorsal midbrain syndrome (Parinaud’s syndrome) consists of upgaze palsy, convergence spasm or paresis, bilateral mid-dilated pupils with light- near dissociation (poor reaction to light and good reaction to near), and convergence-retraction nystagmus. The nystagmus is unique to this syn- drome and consists of rapid convergence with retraction of the globes on attempted upgaze. Common causes include pinealomas, aneurysms of the vein of Galen, and noncommunicating hydrocephalus with distention of the third ventri- cle and the anterior aqueduct of Sylvius. This syn- drome may also occur with stroke as one of the “top of the basilar” syndromes.

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9
Q

Which one of the following is the most likely
cause of this patient’s Horner’s syndrome?
a. Urinary Bence-Jones protein
b. Urinary catecholamines
c. Urinary ketones
d. Urinary casts
e. Urinary protein/creatinine ratio

A

b. Urinary catecholamines

Horner’s syndrome in children may be caused by
neuroblastomas, and 50% are adrenal in origin.

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10
Q

An 82-year-old man had sudden, profound vision loss in his right eye. This was preceded by 2 days of brief episodes of transient vision loss in the affected eye lasting seconds. He also complained of “tender cords” on his scalp, jaw claudication, and weight loss. The erythrocyte sedimentation rate (ESR) was normal and fundoscopy showed a dif- fusely swollen right optic disc.
a. Anterior ischemic optic neuropathy
b. Iritis
c. Central retinal artery occlusion
d. Central retinal vein occlusion
e. Optic neuritis

A

a. Anterior ischemic optic neuropathy

This is the commonest cause of acute visual loss in older patients, and thought to be due to ischemia in posterior circulation of the gobe—predomi- nantly vessels supplying the optic nerve at its exit from the eye. It is classified as non-arteritic or arteri- tic, the former being much more common. In all cases of AION, it is vital to determine whether there is any evidence of an arteritic cause (e.g., giant cell arteritis). Untreated giant cell arteritis can cause rapid, sequential, or simultaneous blindness in both eyes. If giant cell arteritis is suspected because of an elevated ESR or CRP, or both, or because of symptoms such as headache, scalp and temple tenderness, myalgias, arthralgias, low-grade fever, anemia, malaise, weight loss, anorexia, or jaw clau- dication, oral or intravenous steroid treatment should be instituted immediately. In acute cases, the patient may benefit from high-dose intravenous steroids. Biopsy of the temporal artery should follow within days of steroid therapy. In arteritic AION, the visual loss is usually profound, and the optic nerve is often diffusely swollen and pale.

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11
Q

Which one of the following is the most likely cause of this patient’s Horner’s syndrome?
a. Carotid dissection
b. Congenital
c. Neuroblastoma
d. Hypothalamic tumor
e. Cervical cord ependymoma

A

b. Congenital

Iris heterochromia in idiopathic congenital
Horner’s syndrome.

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12
Q

A54-year-old male presented after being told he had unequal pupils by a colleague. Examination findings are shown to light, accom- modation and 30 min after administration of 0.1% pilocarpine to both eyes. Which one of the following is most likely?
a. Left Argyll Robertson pupil
b. Left Horner’s syndrome
c. Right Adie’s tonic pupil
d. Right oculomotor palsy
e. Right relative afferent pupillary defect

A

c—Right Adie’s tonic pupil

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13
Q

A 69-year-old presented with recurrent falls and unsteadiness. Visual acuity is normal. Examination findings are shown to at rest, to light and to accommodation. Which one of the following is demonstrated?
a. Adie’s tonic pupil
b. Argyll Robertson pupils
c. Parinaud syndrome
d. Marcus-Gunn pupil
e. Marcus-Gunn phenomenon

A

b—Argyll Robertson pupils

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14
Q

A 5-year-old child presents with diplopia and numbness over right forehead. Examination findings shown in primary gaze, down gaze, left gaze and right gaze. Which one of the following are most likely?
a. Gradenigo’s syndrome
b. One-and-a-half syndrome
c. Orbital apex syndrome
d. Parinaud’s syndrome
e. Third nerve palsy

A

c—Orbital apex syndrome

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15
Q

A 17-year-old presents after a head injury with diplopia, particularly worse when walk- ing down stairs and two images are oblique to each other. Examination findings are shown
in right gaze, primary gaze, left gaze, right head tilt, left head tilt and at rest. Which one of the following is most likely?
a. Left abducens palsy
b. Left fourth nerve palsy
c. Left hypotropia
d. Right exotropia
e. Right fourth nerve palsy

A

b—Left fourth nerve palsy

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16
Q

Which one of the following is most likely in the images below?
a. Cavernous sinus syndrome
b. Internuclear ophthalmoplegia
c. Left oculomotor nerve palsy
d. Left trochlear nerve palsy
e. Right Horner’s syndrome

A

c—Left oculomotor nerve palsy

17
Q

A 45-year-old man presents with bilateral adduction deficit during attempted gaze (with nystagmus of contralateral abducting eye). Adduction is intact during converging (and accommodating) during viewing of a near target. Which one of the following is most likely?
a. Bilateral internuclear ophthalmoplegia
b. Superior orbital fissure syndrome
c. Light-near dissociation
d. Oculomotor neuropathy
e. Myasthenia gravis

A

a—Bilateral internuclear ophthalmoplegia
(INO)

18
Q

Which one of the following is the most likely cause?
a. Bilateral internuclear ophthalmoplegia
b. Left internuclear ophthalmoplegia
c. Light-near dissociation
d. Left one-and-a-half syndrome
e. Tabes dorsalis

A

d—Left one-and-a-half syndrome

19
Q

An 18-year-old presents with progressive bilateral symmetrical ptosis and ophthal- moplegia, pigmentary retinopathy and car- diac arrhythmia.
Ophthalmoplegia:
a. Abducens palsy
b. Bilateral internuclear ophthalmoplegia/ WEBINO
c. Cavernous sinus thrombosis
d. Oculomotor palsy
e. Kearns-Sayre syndrome
f. Miller Fisher syndrome
g. Myasthenia gravis
h. One-and-a-half syndrome
i. Orbital apex syndrome
j. Parinaud’s syndrome
k. Steele-Richardson-Olszewski syndrome
l. Superior orbital fissure syndrome
m. Thyroid ophthalmopathy
n. Trochlear palsy
o. Unilateral internuclear ophthalmoplegia
p. Wallenberg syndrome

A

1—e, Kearns-Sayre syndrome is a form of
chronic progressive external ophthalmoplegia (CPEO; mitochondrial myopathies) with
onset before the age of 20 years. In addition
to ophthalmoplegia, pigmentary retinopathy
and complete heart block they may also have
cerebellar ataxia, dementia, deafness, short
stature and endocrine disturbance.

20
Q

A 32-year-old presents with ophthalmople- gia, ataxia and areflexia. There is a recent history of diarrheal illness.
Ophthalmoplegia:
a. Abducens palsy
b. Bilateral internuclear ophthalmoplegia/ WEBINO
c. Cavernous sinus thrombosis
d. Oculomotor palsy
e. Kearns-Sayre syndrome
f. Miller Fisher syndrome
g. Myasthenia gravis
h. One-and-a-half syndrome
i. Orbital apex syndrome
j. Parinaud’s syndrome
k. Steele-Richardson-Olszewski syndrome
l. Superior orbital fissure syndrome
m. Thyroid ophthalmopathy
n. Trochlear palsy
o. Unilateral internuclear ophthalmoplegia
p. Wallenberg syndrome

A

2—f, Miller Fisher syndrome (variant of GuillainBarré syndrome),

21
Q

A 72-year-old admitted with aspiration pneumonia is noted to have impaired downgaze, nuchal rigidity, slurred speech and loss of Bell’s phenomenon.
Ophthalmoplegia:
a. Abducens palsy
b. Bilateral internuclear ophthalmoplegia/ WEBINO
c. Cavernous sinus thrombosis
d. Oculomotor palsy
e. Kearns-Sayre syndrome
f. Miller Fisher syndrome
g. Myasthenia gravis
h. One-and-a-half syndrome
i. Orbital apex syndrome
j. Parinaud’s syndrome
k. Steele-Richardson-Olszewski syndrome
l. Superior orbital fissure syndrome
m. Thyroid ophthalmopathy
n. Trochlear palsy
o. Unilateral internuclear ophthalmoplegia
p. Wallenberg syndrome

A

3—k, Steele-Richardson-Olszewski syndrome
(Progressive supranuclear palsy) is an neurodegenerative disorder and included as one of the
Parkinson plus syndromes

22
Q

A 43-year-old presenting with progressive headache and vomiting. Examination reveals light-near dissociation associated with upgaze paresis.
Pupillary abnormalities:
a. Aberrant regeneration of oculomotor nerve
b. Adie’s tonic pupil
c. Argyll Robertson pupil
d. Holmes-Adie’s syndrome
e. Horner’s syndrome
f. Marcus-Gunn pupil
g. Pretectal pupil (Parinaud’s syndrome)
h. Physiologic anisocoria
i. Pupil involving third nerve palsy

A

1—g, Parinaud syndrome;

23
Q

A 54-year-old with chronic cough and weight loss presents with a left ptosis and miosis. Hydroxyamphetamine testing dilates the miotic pupil.
Horner’s syndrome:
a. Brachial plexus injury
b. Carotid dissection
c. Carotid thrombosis
d. Cavernous sinus lesion
e. Cluster headache
f. Head and neck surgery g. Hypothalamic injury
h. Intraoral trauma
i. Lateral medullary stroke
j. Midbrain injury
k. Neuroblastoma
l. Pancoast tumor
m. Pontine injury
n. Small vessel ischemia
o. Spinal cord lesion

A

1—l, Pancoast tumor;

24
Q

A 22-year-old with recurrent sinusitis pre- sents with pyrexia, headache and complex bilateral ophthalmoplegia and loss of cor- neal reflex on the left.
Horner’s syndrome:
a. Brachial plexus injury
b. Carotid dissection
c. Carotid thrombosis
d. Cavernous sinus lesion
e. Cluster headache
f. Head and neck surgery g. Hypothalamic injury
h. Intraoral trauma
i. Lateral medullary stroke
j. Midbrain injury
k. Neuroblastoma
l. Pancoast tumor
m. Pontine injury
n. Small vessel ischemia
o. Spinal cord lesion

A

2—d, Cavernous sinus lesion;

25
Q

A 3-year-old child presents with a right Horner’s syndrome. There is no iris hetero- chromia, no evidence or history of trauma. Urinary catecholamines are increased
Horner’s syndrome:
a. Brachial plexus injury
b. Carotid dissection
c. Carotid thrombosis
d. Cavernous sinus lesion
e. Cluster headache
f. Head and neck surgery g. Hypothalamic injury
h. Intraoral trauma
i. Lateral medullary stroke
j. Midbrain injury
k. Neuroblastoma
l. Pancoast tumor
m. Pontine injury
n. Small vessel ischemia
o. Spinal cord lesion

A

3—k, Neuroblastoma

26
Q

A 21-year-old motorcyclist involved in a road traffic collision where he was thrown 20 ft. He has an obvious right sided scalp laceration and GCS was E2V2M4 at scene. He was intubated and in the ambulance his right pupil dilates to size 6 and is unreactive to light.

Third nerve palsy: a. Atherosclerosis
b. Basilar tip aneurysm
c. Carotid cavernous fistula
d. Cavernous sinus syndrome
e. Chordoma
f. Chronic progressive external ophthal- moplegia
g. Clival meningioma
h. Giant cell arteritis
i. Intraorbital lesion
j. Peripheral neuropathy
k. Posterior communicating artery aneurysm
l. Trauma
m. Uncal herniation

A

1—m, Uncal herniation;

27
Q

A 47-year-old type 1 diabetic develops diplopia. On examination he has a left ptosis and weakness of superior rectus. There is no anisocoria or abnormal pupil- lary reflex.

Third nerve palsy: a. Atherosclerosis
b. Basilar tip aneurysm
c. Carotid cavernous fistula
d. Cavernous sinus syndrome
e. Chordoma
f. Chronic progressive external ophthal- moplegia
g. Clival meningioma
h. Giant cell arteritis
i. Intraorbital lesion
j. Peripheral neuropathy
k. Posterior communicating artery aneurysm
l. Trauma
m. Uncal herniation

A

2—j, Peripheral neuropathy

28
Q

A 22-year-old female with right optic neu- ritis. Swinging flashlight test appears to cause dilatation of the right pupil when light is swung from the left eye to the right eye.
Pupillary abnormalities:
a. Aberrant regeneration of oculomotor nerve
b. Adie’s tonic pupil
c. Argyll Robertson pupil
d. Holmes-Adie’s syndrome
e. Horner’s syndrome
f. Marcus-Gunn pupil
g. Pretectal pupil (Parinaud’s syndrome)
h. Physiologic anisocoria
i. Pupil involving third nerve palsy

A

2—f, MarcusGunn pupil