Optic Nerve/Neuro-ophthalmic Pathways Flashcards

1
Q

In general, at which location in the eye do axons of the nerve fiber layer become myelinated?

Anterior to the lamina cribrosa
At the equator
Posterior to the lamina cribrosa
Juxtafoveally

A

Posterior to the lamina cribrosa

Explanation
Axons of the nerve fiber layer generally become myelinated just after they pass posteriorly through the lamina cribrosa. Occasionally, oligodendrites will enter the eye during ebryogenesis causing myelination of the nerve fiber layer. This will appear as a white nerve fiber layer with feathery margins usually extending from the optic disc. In general, axons within the retina should not be myelinated. Myelination of the nerve fiber layer can be associated with amblyopia, strabismus, nystagmus, optic neuritis, neovascularization of the retina and myopia. However, in most cases, individuals that display this condition are asymptomatic.

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2
Q

Which of the following are the MOST common causes of a pupil sparing and pupil involving third nerve palsy, respectively?

Demyelinating disease, compressive lesions
Compressive lesions, demyelinating disease
Microvascular disease, demyelinating disease
Compressive lesions, microvascular disease
Microvascular disease, compressive lesions
Demyelinating disease, microvascular disease

A

Microvascular disease, compressive lesions

Explanation
The parasympathetic pupillary fibers, along with the surface of the oculomotor nerve, derive their blood supply from the surrounding blood vessels of the pia mater. Conversely, the main trunk of the third nerve is supplied by the vasa nervorum, which courses internally in the nerve. Due to the location of pupillary fibers and the difference in blood supply to the superficial and internal fibers of the oculomotor nerve, certain features that present in cases of third nerve palsies can help differentiate a “surgical” from a “medical” lesion.

Surgical lesions (aneurysms, trauma, uncal herniation) characteristically involve the pupil due to compression of the superficially coursing pupillary fibers and pial blood vessels. These cases are known as “pupil involving third nerve palsies.” Medical lesions (hypertension, diabetes) commonly spare the pupil because these conditions typically involve the vasa nervorum, leading to ischemia of the internal fibers of the oculomotor nerve while sparing the superficial pupillary fibers. This condition is also known as a “pupil-sparing third nerve palsy.”

It is important to note that these principles are not infallible. There are some cases in which pupil involvement is associated with diabetes, and compressive lesions may not initially cause pupil involvement.

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3
Q

The optic nerve is vital for transmission of visual information to the brain. Where does the optic nerve terminate?

At the optic chiasm
At the optic disc
At the visual cortex
At the lateral geniculate nucleus

A

At the optic chiasm

Explanation
The optic nerve is approximately 50 mm in length and originates at the optic disc which is visible upon examination of the eye with an ophthalmoscope. The optic nerve extends from the globe and terminates at the optic chiasm. At the optic chiasm the nasal fibers cross over contralaterally whereas the temporal fibers remain ipsilateral. The chiasm then gives way to the optic tract which courses to the visual cortex.

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4
Q

The trigeminal nerve has three main divisions. Which of the following corresponds to division 1 of the trigeminal nerve (V1)?

Ophthalmic division
Long ciliary
Supraorbital
Posterior ethmoid
Infratrochlear
A

Ophthalmic division

Explanation
Cranial nerves and their respective divisions can be very confusing and are best understood and remembered with diagrams and mnemonics.

The trigeminal nerve has three main divisions: ophthalmic (V1), maxillary (V2), mandibular (V3).

The ophthalmic division (V1) has three sub-divisions (think NFL) which are the nasociliary, frontal, and lacrimal.

The frontal division of the V1 branch possesses two sub-divisions: the supraorbital and the supratrochlear. The frontal division is the most commonly affected in herpes zoster ophthalmicus.

The nasociliary division has four sub-divisions (think LINE): long ciliary, infratrochlear, nasal, and the posterior ethmoid.

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5
Q

The ophthalmic portion (V1) of cranial nerve V (the trigeminal nerve) possesses three main sub-divisions. Which of the following is a subdivision of V1?

Supratrochlear division
Mandibular division
Maxillary division
Frontal division

A

Frontal division

Explanation
Cranial nerves and their respective divisions can be very confusing and are best understood and remembered with diagrams and mnemonics.

The trigeminal nerve has three main divisions: ophthalmic (V1), maxillary (V2), mandibular (V3).

The ophthalmic division (V1) has three sub-divisions (think NFL) which are the nasociliary, frontal and lacrimal.

The frontal division of the V1 branch possesses two sub-divisions: the supraorbital and the supratrochlear divisions. The frontal division is the most commonly affected in herpes zoster ophthalmicus.

The nasociliary division has four sub-divisions (think LINE): long ciliary, infratrochlear, nasal, and the posterior ethmoid.

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6
Q

While performing confrontation fields, your patient reports seeing all of your fingers when presented on the left and right side of her visual field exclusively; however when your fingers are presented on both sides simultaneously, the patient only reports seeing the fingers on your left hand (her right visual field). What type of lesion is consistent with these findings?

A lesion in the left parietal lobe
A lesion in the right frontal lobe
A lesion in the right parietal lobe
A lesion in the right occipital lobe
A lesion in the left frontal lobe
A

A lesion in the right parietal lobe

Explanation
This type of finding is consistent with a person who suffers from a right parietal lobe lesion. Remember, the right side of the brain processes info from the left side of the body and visual field. A person who has this type of lesion may also demonstrate visual neglect, meaning that they will ignore the half of their body on the opposite side of the lesion. For example a person with a right parietal lesion will apply make-up to the right side of her face but not the left side. The neglect is much more pronounced if a person is presented with stimuli to both sides of their visual field simultaneously. For this reason it is important to test confrontational fields on both sides of the visual field at the same time in order to manifest a phenomenon called visual/parietal extinction. In this phenomenon, the object presented contralateral to the lesion will not be seen by the patient because it is extinguished by the object presented on the unaffected side. This allows for easier detection of a potential lesion, and it just takes a few more seconds of chair time for the patient.

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7
Q

Which area of the retina naturally corresponds with a sensitivity of 0 decibels on visual field testing?

The macula
The optic nerve head
The papillomacular bundle
The superior arcade
The inferior arcade
A

The optic nerve head

Explanation
The optic nerve head, also known as the blind spot, will demonstrate a sensitivity of 0 decibels, because this area of the eye does not contain any visual receptors, and therefore does not possess any viable vision. The area of the retina with the highest decibel level (hence the highest sensitivity) as seen with visual field testing is the fovea. The sensitivity to stimuli decreases with increasing distance from the fovea.

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8
Q

Which of the following is LEAST likely to result in the observation of an afferent pupillary defect?

Severe, bilateral, asymmetrical macular degeneration
Asymmetric, bilateral, glaucomatous damage with 20/20 acuity in both eyes
A dense unilateral cataract
A patient with a dense corneal scar in one eye and glaucomatous damage in the contralateral eye

A

A dense unilateral cataract

Explanation
The afferent pathway (the pathway from the eye to the brain) is evaluated by having the patient focus on a distant target under moderate lighting conditions. A bright controlled light source (transilluminator) is then directed from below the patient’s line of sight into the pupil. A normal pupil will quickly constrict and then slowly increase in size until it is mid-dilated, followed by hippus. This procedure is then repeated on the other eye. The light source is then alternated quickly back and forth between the pupils to evaluate the consensual response. Damage to the afferent pathway will cause an afferent pupil defect or light-near dissociation. Light-near dissociation occurs when the pupil response to a near stimulus is greater than the pupil reaction to light.

Afferent pupillary defects are observed if there is retinal or optic nerve damage. However, the damage between the eyes must be asymmetrical in order for an APD to be observed. Even a small amount of nerve damage may cause an APD, whereas a much larger degree of retinal damage is required to produce an APD. As long as the light source used is adequate, a dense cataract or a corneal scar should (in theory) not cause an APD.

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9
Q

A patient with bilateral tilted discs whose crescents are inferior and nasally displaced will typically display a defect located along which portion of the visual field test?

Inferior nasal
Superior temporal
Inferior temporal
Superior nasal

A

Superior temporal

Explanation
A patient who has tilted discs will have an optic nerve that exits the eye superiorly, and may be accompanied by situs inversus, fundus ectasia, myopia, and superior temporal visual field defects that do not generally respect the midline. Sometimes the visual field defects can appear similar to those of a person who suffers from a pituitary tumor. It is important to distinguish between the two and this situation requires further evaluation.

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10
Q

The FIRST synapse in the visual pathway occurs in which of the following layers of the retina?

Photoreceptor layer
Inner plexiform layer
Outer plexiform layer
Ganglion cell layer
Inner nuclear layer
Outer nuclear layer
A

Outer plexiform layer

Explanation
The first synapse of the visual system occurs in the outer plexiform layer (OPL) of the retina. It is here that the cone pedicles and rod spherules are synaptic upon the dendrites of various bipolar cells. Additionally, projections from horizontal cells also make contact with rods, cones, and bipolar cells in the outer plexiform layers.

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11
Q

Visual acuity can be used to assess the function of which cranial nerve?

II
VI
VI
IV
III
A

II

Explanation
Visual acuity, along with confrontation fields, pupil reflex evaluation, and the red cap desaturation test, can be utilized to assess the integrity of cranial nerve II (optic). Cranial nerves III (oculomotor), IV (trochlear), and VI (abducens) can be evaluated via extraocular motility (versions). Cranial nerve V (trigeminal) is measured via facial stimulation (forehead, nose, and jawline to assess all three divisions), corneal sensitivity, and palpation of the masseter muscles while the patient clenches their teeth.

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12
Q

Damage to the left trochlear nucleus will affect which of the following muscles?

The right superior rectus
The right superior oblique
The left superior oblique
The left medial rectus
The left superior rectus
A

The right superior oblique

Explanation
Fibers of the trochlear nerve decussate, and therefore innervate, the CONTRALATERAL superior oblique muscle. The superior oblique is responsible for incyclotorsion, depression, and abduction. Cranial nerve (CN) IV is unique in that it travels the longest intracranial course when compared to all of the other cranial nerves. The trochlear nerve is also the smallest cranial nerve, and because this nerve is so thin it is prone to damage. A CN IV palsy will cause a hypertropia of the eye on the affected side and the patient will report vertical diplopia that is worse in downgaze and at near.

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13
Q

Which of the following ocular conditions will NEVER produce a relative afferent pupillary defect in the ipsilateral eye?

Optic nerve glioma
Central retinal artery occlusion
Optic neuritis
Mature cataract
Total retinal detachment
A

Mature cataract

Explanation
A relative afferent pupillary defect (RAPD) occurs in patients who have a history of an incomplete optic nerve lesion (a complete lesion would result in an absolute afferent pupillary defect), or the presence of severe retinal disease. The clinical features of a relative afferent pupillary defect include a pupil that responds weakly to light stimulation in the diseased eye and briskly to light stimulation in the normal eye. The results are exaggerated during the swinging flashlight test, in which both eyes will constrict when the normal eye is presented with a light stimulus, and both eyes will slowly dilate when the diseased eye is presented with the same amount of light. This anomalous result of dilation of the pupils in response to light occurs because the normal dilation that occurs when the light is withdrawn from the normal eye is more pronounced than the constriction that is produced by stimulating the abnormal eye.

There are several conditions that may cause an afferent pupillary defect. These include severe retinal diseases, or macular scarring, severe asymmetric glaucoma, an optic nerve glioma or meningioma, central retinal artery occlusion or ischemic central retinal vein occlusion, optic neuritis, or any lesion that occurs along any segment of the optic nerve, usually prior to the chiasm. A dense cataract will never cause a relative afferent pupillary defect in the same eye, and should, therefore, never be used to explain the presence of an RAPD.

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14
Q

Which space in the meninges of the optic nerve is continuous with the space between the brain meninges such that an increase in cerebrospinal fluid pressure may cause the appearance of papilledema?

The subdural space
Subarachnoid space
Dural sinuses
The subpial space

A

Subarachnoid space

Explanation
The subarachnoid space of the optic nerve is continuous with that of the brain. The subarachnoid space exists between the arachnoid and the pia mater and ends at the lamina cribrosa where it folds back on itself and terminates in a cul-de-sac. This space is filled with cerebrospinal fluid (CSF) and therefore should anything cause an increase in CSF pressure, this can translate down to the nerve and manifest as papilledema.

The dural sinuses are venous channels that carry blood from the brain to the heart, not cerebrospinal fluid.

The dura is generally firmly attached to the skull or bones and the arachnoid is connected to the dura. In the event of injury, trauma or sickness the dura and arachnoid may become separated resulting in a space called the subdural space.

In a healthy individual there should not exist a space underneath the pia mater. The pia mater is an extremely thin membrane that adheres very closely to the brain, spinal cord and optic nerve such that it follows the sulci and gyri of the brain’s surface.

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15
Q

Which of the following BEST describes the rotation of the eyeball during Bell’s phenomenon?

Upward and outward
Downward and outward
Upward and inward
Downward and inward

A

Upward and outward

Explanation
Bell’s phenomenon is a reflex coordinated between the facial nerve and the oculomotor nuclei in which the eyeball is rotated upward and outward upon closure of the eyelids. The reflexive movement occurs as a protective mechanism that repositions the cornea up under the eyelid and, therefore, away from potential danger. The neurological pathway of this phenomenon is not completely understood. It is also not present in about 10% of otherwise healthy individuals; thus, its absence cannot be relied upon as a cause or cited as a sign of disease.

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16
Q

Ocular perfusion pressure (OPP) is defined as which of the following?

Systemic blood pressure minus episcleral venous pressure
Systemic blood pressure minus intraocular pressure
Systolic blood pressure minus diastolic blood pressure
Episcleral venous pressure minus intraocular pressure

A

Systemic blood pressure minus intraocular pressure

Explanation
Ocular perfusion pressure (OPP) is defined as systemic blood pressure minus intraocular pressure (IOP). OPP is important in that many researchers believe that it potentially plays a role in the pathophysiology of glaucoma; although this remains controversial. Some believe that low OPP results in decreased perfusion of ocular tissues, which may contribute to hypoxia or ischemia of optic nerve tissue.

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17
Q

Which of the following pupillary fibers is damaged in patients presenting with an Adie’s tonic pupil?

Pre-ganglionic sympathetic
Post-ganglionic sympathetic
Pre-ganglionic parasympathetic
Post-ganglionic parasympathetic

A

Post-ganglionic parasympathetic

Explanation
An Adie’s tonic pupil is usually an idiopathic, benign form of internal ophthalmoplegia that occurs as a result of damage and subsequent denervation of the postganglionic parasympathetic pupillomotor fibers. These fibers originate from the ciliary ganglion (which is located within the muscle cone, just behind the globe), and travel forward to eventually supply the pupil sphincter.

Around 90% of patients presenting with an Adie’s pupil are women, typically between the ages of 20 and 40. The condition is unilateral in 80% of cases; however, involvement of the fellow eye may develop later. Initially, anisocoria is present, in which the abnormal eye is dilated and reacts poorly to light and accommodates poorly. Slit-lamp evaluation will commonly show an iris that exhibits segmental contraction in response to light (vermiform movement). Later, the affected pupil will become miotic (“little old Adie’s”).

18
Q

A patient presenting with neurosyphilis is MOST likely to exhibit which of the following pupil conditions?

Unilateral Adie's tonic pupil
Bilateral Argyll Robertson pupil
Absolute afferent pupillary defect
Relative afferent pupillary defect
Bilateral Adie's tonic pupil
Unilateral Argyll Robertson pupil
A

Bilateral Argyll Robertson pupil

Explanation
Bilateral Argyll Robertson pupils are a highly specific sign found in patients with neurosyphilis. In these cases, patients typically present with small pupils that do not respond well to light but will exhibit significant constriction when fixation on a near object occurs. This is usually bilateral but may be asymmetrical. This particular finding is also known as “light-near dissociation.” The exact pathophysiology of this condition is not completely known; however, most investigators believe that syphilis damages the intercalated neurons that make the connection between the pretectal nucleus and each Edinger-Westphal nuclei.

There are other conditions that may cause bilateral light-near dissociation such as diabetes, myotonic dystrophy, Parinaud’s dorsal midbrain syndrome, familial amyloidosis, encephalitis, and chronic alcoholism. Disorders that may result in unilateral light-near dissociation include Adie’s tonic pupil, herpes zoster ophthalmicus, and aberrant regeneration of the 3rd nerve.

19
Q

A college student presents with bilateral internuclear ophthalmoplegia (INO). This finding is pathognomonic for which one of the following?

Bell’s palsy
Pseudotumor cerebri
Lyme disease
Multiple sclerosis

A

Multiple sclerosis

Explanation
An internuclear ophthalmoplegia is caused by an interruption or dysfunction in the medial longitudinal fasciculus. This is a heavily myelinated tract, and the clinical disturbance is a failure of conjugate lateral gaze. When an attempt to look to the right is made, the affected eye (in this case, the left eye) cannot adduct (move in to maintain conjugate gaze) while the unaffected eye (in this case, the right eye) will usually manifest a nystagmus due to overcompensation during abduction. In young patients, either a one-sided or bilateral INO is highly suggestive of multiple sclerosis. In older patients, it may represent a vascular defect or stroke.

Lyme disease does not produce INOs but can present with facial palsy; it is said that bilateral facial palsies are pathognomonic for Lyme disease. Bell’s palsy is a paralysis of the seventh cranial nerve (CN VII). Pseudotumor cerebri is a defect in the reabsorption of cerebrospinal fluid leading to elevated intracranial pressure and the finding of papilledema. It is most commonly seen in young overweight females.

20
Q

Which of the following nerves does NOT travel within the lateral wall of the cavernous sinus but rather passes through the middle of the sinus alongside the internal carotid artery?

Oculomotor nerve
Maxillary branch of trigeminal nerve
Trochlear nerve
Ophthalmic branch of trigeminal nerve
Abducens nerve
A

Abducens nerve

Explanation
Unlike the oculomotor, trochlear, and ophthalmic and maxillary branches of the trigeminal nerve, the abducens nerve (CN VI) does not run within the lateral wall of the cavernous sinus. Rather, it courses through the body of the sinus alongside the internal carotid artery.

21
Q

The anterior knee of von Willebrand is composed of optic nerve fibers carrying information from which of the following quadrants of the retina?

Inferior-nasal
Superior-temporal
Superior-nasal
Inferior-temporal

A

Inferior-nasal

Explanation
The optic chiasm is formed by the union of the right and left optic nerves. It is in this area that the fibers originating from the nasal retina (temporal visual field) decussate to join the uncrossed temporal fibers (nasal visual field) and course posteriorly as the optic tracts.

The inferior nasal fibers remain low as they traverse the optic chiasm and decussate more anteriorly. Because of their location, these fibers are more vulnerable to damage from expanding pituitary lesions, leading to involvement of the superior temporal visual fields early in the disease. It is also important to note that these inferior-nasal fibers actually loop forward into the contralateral optic nerve before coursing posteriorly in the optic tract. This region is known as the anterior knee of von Willebrand.

The superior nasal fibers remain high as they traverse the optic chiasm and decussate more posteriorly. Therefore, these fibers are typically first involved in cases of lesions growing from above the optic chiasm (craniopharyngiomas), affecting the inferior temporal visual fields early in the disease progression.

22
Q

Which 2 of the following muscles are innervated by the superior division of the third cranial nerve? (Select 2)

Superior oblique
Medial rectus
Mueller's muscle
Superior rectus
Sphincter pupillae
Levator palpebrae superioris
A

Superior rectus
Levator palpebrae superioris

Explanation
The superior division of the third cranial nerve innervates the levator palpebrae superioris and the superior rectus muscles.

The inferior division innervates the medial rectus, inferior rectus, and inferior oblique muscles. The branch that leads to the inferior oblique also contains preganglionic parasympathetic pupillary fibers from the Edinger-Westphal subnucleus, which innervate the pupillary sphincter and ciliary muscle.

23
Q

Which of the following medications has been identified as a risk factor for the development of non-arteritic anterior ischemic optic neuropathy (NAION)?

Omeprazole
Simvastatin
Hydrochlorothiazide
Celecoxib
Atenolol
Sildenafil
A

Sildenafil

Explanation
Non-arteritic anterior ischemic optic neuropathy is an ocular condition that typically presents as a sudden, painless loss of vision that is thought to be triggered by inadequate perfusion of the optic nerve head. This eventually leads to infarction, causing swelling and compression of the tissue, ischemia, and further infarction. Unlike an arteritic anterior ischemic optic neuropathy, there typically aren’t any warning signs of the impending condition, but there have been associated risk factors identified of which clinicians should be aware. These predisposing factors include a small optic disc with a small or crowded cup (known as a “disc at risk”). In these cases, if the disc begins to swell, the fibers have no place to go except to further compress on nearby axons, which leads to further swelling. Nocturnal (or nighttime) hypotension is also thought to be a risk factor due to the fact that many cases of NAION occur upon wakening or shortly thereafter. Several studies have also found a connection between sleep apnea and cases of NAION.

When it comes to medication correlations, there have been several reported cases of NAION occurring in men after taking sildenafil citrate (Viagra®). The exact mechanism is unclear; however, it is believed that because the drug is involved in modulating blood vessel diameter, it is also then likely to have an effect on perfusion to the optic nerve tissue.

24
Q

Visual acuity can be used to assess the function of which cranial nerve?

II
IV
VI
VI
III
A

CN II

Explanation
Visual acuity, along with confrontation fields, pupil reflex evaluation, and the red cap desaturation test, can be utilized to assess the integrity of cranial nerve II (optic). Cranial nerves III (oculomotor), IV (trochlear), and VI (abducens) can be evaluated via extraocular motility (versions). Cranial nerve V (trigeminal) is measured via facial stimulation (forehead, nose, and jawline to assess all three divisions), corneal sensitivity, and palpation of the masseter muscles while the patient clenches their teeth.

25
Q

Which of the following cranial nerves is affected in a patient diagnosed with Bell’s palsy?

Eighth cranial nerve
Fifth cranial nerve
Third cranial nerve
Eleventh cranial nerve
Seventh cranial nerve
Ninth cranial nerve
A

Seventh cranial nerve

Explanation
Bell’s palsy is an idiopathic isolated peripheral palsy of the ipsilateral seventh cranial nerve (facial nerve). It is thought that inflammation of the nerve results in compression of the fibers where it exits the skull within its bony canal, thereby blocking the transmission of neural signals.

26
Q

Double-ring sign is an observable feature associated with which of the following congenital optic nerve anomalies?

Megalopapilla
Optic nerve hypoplasia
Optic disc dysplasia
Morning glory syndrome
Buried optic disc drusen
Optic disc coloboma
A

Optic nerve hypoplasia

Explanation
Patients with optic nerve hypoplasia present with several characteristic funduscopic features that comprise its diagnosis. Classically, the optic disc is small, has a greyish appearance, and is surrounded by a mottled, yellow, hypo-pigmented peripapillary border that encircles the disc, forming a halo. This halo is also bordered by a darker pigmented ring, which leads to the name “double ring sign.” The outer ring represents what would have been the margin of the disc had it been of normal size.

27
Q

Which of the following branches of the trigeminal nerve is MOST commonly affected in herpes zoster infections?

Mandibular division
Frontal division
Maxillary division
Ophthalmic division

A

Ophthalmic division

Explanation
The most frequently affected branch of the trigeminal nerve in herpes zoster infections is the ophthalmic division (V1) with its supraorbital, lacrimal and nasociliary distributions. The ophthalmic nerve carries sensory information from the scalp and forehead, the upper eyelid, the conjunctiva and cornea, the nose (including the tip of the nose), the nasal mucosa, the frontal sinuses, and part of the meninges.

28
Q

Which 4 of the following nerves are strictly MOTOR in function? (Select 4)

Cranial nerve III
Cranial nerve IV
Cranial nerve XI
Cranial nerve I
Cranial nerve II
Cranial nerve VI
A

Cranial nerve III
Cranial nerve IV
Cranial nerve XI
Cranial nerve VI

Explanation
Cranial nerves I (olfactory), II (optic), and VIII (vestibulocochlear) are sensory in function. Cranial nerves III (oculomotor), IV (trochlear), VI (abducens), XI (spinal-accessory) and XII (hypoglossal) have exclusively motor functions. Cranial nerves V (trigeminal), VII (facial), IX (glossopharyngeal) and X (vagus) possess both motor and sensory functions. A good mnemonic is: Some Say Marry Money But My Brother Says Big Brains Matter More (The words correspond to the cranial nerves in proper numerical order, with all ‘S’ words representing sensory function: ‘M’ words depict exclusive motor function, and ‘B’ words signify nerves with both motor and sensory function).

29
Q

Which of the following optic nerve conditions results from an increase in intracranial pressure?

Ischemic optic neuropathy
Papilledema
Papillitis
Optic neuritis

A

Papilledema

Explanation
Papilledema is defined as passive swelling of the optic disc caused by increased intracranial pressure, usually as a result of intracranial tumors or malignant hypertension. The condition is almost always bilateral, and vision is usually normal unless there is associated edema or exudates in the macular area.

Optic neuritis is inflammation of the optic nerve that can occur at any location as it courses from the eye. It is typically caused by swelling and destruction of the myelin sheath that surrounds the nerve.

Papillitis is a specific type of optic neuritis in which there is an inflammation of the optic nerve head. This condition is almost always unilateral, and because the optic nerve is inflamed, it is commonly associated with vision loss.

Ischemic optic neuropathy is caused by obstruction of blood flow to the optic nerve, resulting in the loss of structure or function of a portion of the nerve. It is classified as either arteritic or non-arteritic, according to the location of the nerve that is affected. Patients with this condition will often present with acute loss of visual acuity and an altitudinal visual field defect.

30
Q

Which of the following vessels provides the majority of the blood supply to the optic nerve head?

Central retinal artery
Short posterior ciliary arteries
Long posterior ciliary arteries
Anterior ciliary arteries

A

Short posterior ciliary arteries

Explanation
The ophthalmic artery provides the majority of the supply of blood to both the inner retina and the optic nerve.
- The central retinal artery (CRA) branch of the ophthalmic artery enters the optic nerve approximately 12mm behind the globe
- In the retina, the retinal ganglion cell bodies and the nerve fiber layer are primarily supplied by capillary branches of the central retinal artery that emerge from the optic nerve head
- As the CRA courses over the optic disc, it provides partial perfusion to some of the superficial optic disc; however, it provides minimal perfusion to the optic nerve itself, through which it courses
- Branches of the medial and lateral short posterior ciliary arteries (SPCAs) also originate from the ophthalmic artery
- These provide the majority of the blood supply to the optic nerve head as well as the choroid
- Most notably, anastomoses of the SPCAs create the Circle of Zinn-Haller, which provides significant perfusion to the optic nerve head

31
Q

Which of the following is the correct order of structures through which the pupillary fiber pathway passes?

Optic nerve-> optic chiasm -> Lateral geniculate nucleus in the thalamus-> Edinger-Westphal nucleus

Optic nerve -> optic chiasm -> optic tract -> pretectal region of the midbrain -> Lateral geniculate nucleus in the thalamus

Optic nerve -> optic chiasm -> brachium of the superior colliculus -> pretectal region of the midbrain -> Edinger-Westphal nucleus

Optic nerve -> optic chiasm -> optic tract -> Lateral geniculate nucleus in the thalamus

A

Optic nerve -> optic chiasm -> brachium of the superior colliculus -> pretectal region of the midbrain -> Edinger-Westphal nucleus

Explanation
The pupillary fibers exit the eye through the optic nerve and pass through the optic chiasm, where they then exit the optic tract and enter into the brachium of the superior colliculus and synapse onto cells in the pretectal area of the midbrain. The pathway then continues and stimulates intercalated neurons, which in turn stimulate cells in the Edinger-Westphal nucleus. The axons of the pupillary pathway never enter the lateral geniculate nucleus.

32
Q

Which 2 of the following signs and symptoms can aid in differentiating between a microvascular and compressive third cranial nerve palsy? (Select 2)

The presence of pain is more common in aneurysmal third nerve palsies

Microvascular third nerve palsies do not typically involve the pupil

Aberrant regeneration typically result from microvascular third nerve palsies

Third nerve palsies due to compressive lesions will always have an affect on pupil size

Third nerve palsies associated with microvascular disease commonly spontaneously resolve within 3 months

A

Microvascular third nerve palsies do not typically involve the pupil

Third nerve palsies associated with microvascular disease commonly spontaneously resolve within 3 months

Explanation
There are several signs and symptoms that clinicians utilize in order to aid in differentiating between microvascular and compressive third nerve palsies. Some of these are summarized below:
- Third nerve palsies associated with microvascular disease commonly spare the pupil due to the superficial location of pupillary fibers as they travel in the nerve
- Third nerve palsies associated with compressive lesions commonly involve the pupil due to compression of the superficial pupil fibers
- It is important to note that this is only a general principle; there are several cases in which compressive lesions do not affect the pupil initially but may eventually if the lesion continues to grow (also, some diabetic palsies have been shown to cause pupil dilation)
- Third nerve palsies due to microvascular lesions will typically resolve within a period of three months (if a suspected microvascular lesion does not resolve within this time, imaging must be completed)
- Aberrant regeneration will only occur following acute traumatic or compressive third nerve palsies (but never after a microvascular palsy) due to the fact that the endoneural nerve sheaths remain intact in vascular pathology
- The presence of pain is common in both diabetic and aneurysmal third nerve palsies; therefore, it cannot be used to differentiate the two

33
Q

Which of the following conditions has been associated with an intact pupillary light response and absent near response?

Neurosyphilis
There is no clinical condition in which this exists
Myotonic dystrophy
Diabetes
Aberrant regeneration of the third nerve
Adie's tonic pupil
A

There is no clinical condition in which this exists

Explanation
The near reflex is activated when a person changes gaze from a distant to a near object. It is comprised of a triad of accommodation, convergence, and pupil miosis. Vision is not required for this response, and there is no clinical condition in which the pupillary light reflex is intact and the near response is absent. However, there are several conditions that are associated with dissociation of light and near responses in which the light reflex is either absent or sluggish and the near response is normal. Although the final pathways for both responses are identical, the center for the near reflex is controlled by a supranuclear influence.

34
Q

Which of the following sequences describes the correct order of the oculosympathetic pathway as the neurons descend from the brain?

Hypothalamus–> superior cervical ganglion–> ciliospinal center of Budge–> ophthalmic division of trigeminal nerve

Pretectal nucleus–> superior cervical ganglion–> ciliospinal center of Budge–> ophthalmic division of trigeminal nerve

Pretectal nucleus–> ciliospinal center of Budge–> superior cervical ganglion–> ophthalmic division of trigeminal nerve

Hypothalamus–> ciliospinal center of Budge–> superior cervical ganglion–> ophthalmic division of trigeminal nerve

A

Hypothalamus–> ciliospinal center of Budge–> superior cervical ganglion–> ophthalmic division of trigeminal nerve

Explanation
The oculosympathetic pathway contains three neurons as it courses from the brain to structures of the eye. The first neuron (central neuron) in this pathway begins at the posterior region of the hypothalamus. It descends down the brainstem to synapse at the ipsilateral ciliospinal center of Budge, which lies between C8 and T2 of the spinal cord. The second neuron (pre-ganglionic neuron) traverses a long course from the ciliospinal center to the superior cervical ganglion in the neck region. During its path, it is closely related to the apical area of the lung where it can be damaged by a Pancoast tumor, or in some cases, during surgery on the neck. The final neuron in the oculosympathetic pathway (post-ganglionic neuron) ascends with the internal carotid artery where it eventually enters the cavernous sinus and joins with the ophthalmic division of the trigeminal nerve. From here, the sympathetic fibers reach the pupil dilator and ciliary body by means of the long ciliary nerves and nasociliary nerves.

35
Q

A healthy retinal nerve fiber layer is thickest at which portion of the optic nerve head?

Inferiorly
Temporally
Superiorly
Nasally

A

Inferiorly

Explanation
The nerve fiber layer is thickest at the inferior and superior regions of the nerve, respectively. The inferior and superior arcades are composed of large diameter axons with little overlap of the receptive fields, thus explaining why a field defect occurs in these regions first for early cases of glaucoma. Inferior or superior notching of the nerve is highly suspect for glaucomatous damage, and must undergo further testing in order to rule out glaucoma. The next thickest area of nerve fiber layer tissue is nasally, which is comprised of the nasal radial fibers. These axons are affected in the later stages of glaucoma, thus explaining why a temporal island of the visual field is often left remaining in advanced cases of glaucoma. Lastly, the temporal rim area is the thinnest. Temporal rim tissue is comprised of the papillomacular bundle. The fibers in this area are very small and compact, with a high degree of receptive field overlap, therefore because of the receptive field redundancy, a visual field defect correlating to this region will occur only after significant fiber loss has occurred. Due to the fact that these fibers are so small in diameter, even though they are numerous, the fibers do not occupy a lot of space in the optic nerve. The thickness of the nerve fiber layer rim tissue is best remembered as ISNT, with inferior being the thickest and temporal rim tissue being the thinnest.

36
Q

Which layer of the retina is present at the optic disc?

The external limiting membrane
The outer nuclear layer
The outer plexiform layer
The nerve fiber layer

A

The nerve fiber layer

Explanation
The nerve fiber layer is the only layer of the retina that is present at the optic disc. The disc serves as the convergent point for axons of the nerve fiber layer which then form the optic nerve to convey visual information to the brain for further processing. The actual optic disc does not contain photoreceptors and thus will not generate nervous signals. This explains why this area is also known as the ‘blind spot’.

37
Q

A 9-year old patient presents with a unilateral ptosis of the left eyelid that improves in position when he widely opens his jaw. This condition, known as Marcus-Gunn jaw winking, is the result of aberrant connections between which 2 of the following cranial nerves? (Select 2)

Cranial nerve VII
Cranial nerve II
Cranial nerve V
Cranial nerve IV
Cranial nerve VI
Cranial nerve III
A

Cranial nerve V

Cranial nerve III

Explanation
Marcus-Gunn jaw winking is a phenomenon that is thought to occur as a result of an abnormal connection between cranial nerve V and cranial nerve III. Branches from the motor root of the trigeminal nerve that are meant for the ipsilateral pterygoid muscle aberrantly connect with the ipsilateral levator muscle instead. This allows for an elevation effect of the upper eyelid when jaw movements are initiated. This type of aberrant innervation may occur in up to 5% of patients presenting with a congenital ptosis; it tends to lessen in severity with age. This condition should not be confused with aberrant regeneration of cranial nerve VII; it is an isolated neurologic finding and does not require further work-up. Furthermore, Marcus-Gunn jaw winking is typically monitored indefinitely unless there is cosmetic concern and/or surgical intervention is desired.

38
Q

Which of the following statements is TRUE in regards to pharmacologic testing in patients with a suspected Horner’s syndrome?

Cocaine and hydroxyamphetamine can be used interchangeably to differentiate between pre and post-ganglionic lesions

Cocaine and hydroxyamphetamine can be used interchangeably to confirm a diagnosis of Horner’s

Cocaine confirms a diagnosis of Horner’s; hydroxyamphetamine will differentiate between pre and post-ganglionic lesions

Hydroxyamphetamine confirms a diagnosis of Horner’s; cocaine will differentiate between pre and post-ganglionic lesions

A

Cocaine confirms a diagnosis of Horner’s; hydroxyamphetamine will differentiate between pre and post-ganglionic lesions

Explanation
In patients with a suspected Horner’s syndrome, cocaine will confirm the diagnosis, while hydroxyamphetamine will differentiate a pre-ganglionic from a post-ganglionic lesion. A summary of pharmacologic testing in Horner’s is summarized below.

10% Cocaine

  • The normal pupil will dilate, but the abnormal pupil will not
  • The rationale for this is that in a normal pupil, norepinephrine is released from the post-ganglionic sympathetic nerve endings, which is eventually re-absorbed, terminating the nerve impulse
  • Cocaine inhibits the re-uptake of norepinephrine, allowing it to accumulate in the synapse, and will cause dilation of the pupil
  • In Horner’s syndrome, the abnormal eye will not produce any norepinephrine in the first place, so cocaine has no effect on the pupil size

1% Hydroxyamphetamine

  • In a pre-ganglionic lesion, both pupils dilate
  • In a post-ganglionic lesion, the abnormal pupil will not dilate
  • The rationale for this is that hydroxyamphetamine will stimulate the release of norepinephrine from the post-ganglionic neurons
  • If there is damage to a pre-ganglionic neuron (leaving the post-ganglionic intact), norepinephrine will be released, and dilation will occur
  • If there is damage to the post-ganglionic neuron, hydroxyamphetamine will have no effect on this neuron, no norepinephrine will be released, and the pupil will not dilate
39
Q

What is the correct order of the optic nerve meninges progressing from most external to most internal?

Pia mater, arachnoid, dura mater
Arachnoid, pia mater, dura mater
Dura mater, arachnoid, pia mater
Dura mater, pia mater, arachnoid
Arachnoid, dura mater, pia mater
A

Dura mater, arachnoid, pia mater

Explanation
The correct order of the optic nerve meninges is the same as those found on the brain which makes sense since they are extensions of each other. The dura mater is the most external layer and is composed of very dense connective tissue. Next is the arachnoid follows next whose connective tissue is less ordered than the dura mater. The most internal of the meninges is the pia mater which is adhered to the nerve.

40
Q

Which 3 of the following extraocular muscles are supplied by the MEDIAL muscular branch of the ophthalmic artery? (Select 3)

Inferior rectus
Superior rectus
Inferior oblique
Medial rectus
Lateral rectus
Superior oblique
A

Inferior rectus
Inferior oblique
Medial rectus

Explanation
The medial muscular branch of the ophthalmic artery supplies the medial rectus, inferior rectus, and the inferior oblique extra-ocular muscles, while the lateral muscular branch supplies the superior rectus, superior oblique, lateral rectus, and levator palpebrae superioris.