Neuro‐ophthalmology


Chapter 3
Neuro‐ophthalmology


The eye is part of the brain. The earliest beginnings of the brain began 550 million years ago in single‐celled organisms. “Eye spots” on the cells’ surface contained photoreceptor proteins that sensed light. For a description of imaging, Chapter 5. Six muscles (Table 2) move each eye around three axes. They are innervated by the III, IV, and VI cranial nerves (Table 3, Figs 7882).


Eye movements

Schematic illustration of lateral orbital view: adduction and abduction are around the superior–inferior axis.

Fig 78 Lateral orbital view: adduction and abduction are around the superior–inferior axis (SI).

Schematic illustration of the eye rotates around three different axes coordinated by the action of six extraocular muscles.

Fig 79 The eye rotates around three different axes coordinated by the action of six extraocular muscles.

Schematic illustration of superior orbital view. Elevation and depression are on the horizontal axis passing from the nasal to temporal side of the eye. Torsion is on the anterior–posterior axis.

Fig 80 Superior orbital view. Elevation and depression are on the horizontal axis (NT, nasal–temporal) passing from the nasal to temporal side of the eye. Torsion is on the anterior–posterior axis (AP).

Schematic illustration of blood being supplied to the brain by two vertebral arteries and two carotid arteries. The circle of Willis is a cerebral arterial circle connecting the carotid to posterior cerebral arteries. It allows collateral flow if one vessel narrows. Eighty percent of ischemic strokes originate from the carotid and 20 percentage from the vertebral or basilar circulation. The two most common cerebral aneurysms occur in this circle. The one connecting the two anterior cerebral arteries may press on the optic chiasm, sometimes causing a bitemporal hemianopsia. The other, at the junction of the carotid and posterior communicating arteries, may press on CN III, causing a dilated pupil. If the aneurysms rupture, they may cause a severe headache, a stiff painful neck, blurred or double vision, and photophobia.

Fig 81 Blood is supplied to the brain by two vertebral arteries and two carotid arteries. The circle of Willis (green) is a cerebral arterial circle connecting the carotid to posterior cerebral arteries. It allows collateral flow if one vessel narrows. Eighty percent of ischemic strokes originate from the carotid and 20% from the vertebral or basilar circulation. The two most common cerebral aneurysms occur in this circle. The one connecting the two anterior cerebral arteries may press on the optic chiasm, sometimes causing a bitemporal hemianopsia. The other, at the junction of the carotid and posterior communicating arteries, may press on CN III, causing a dilated pupil. If the aneurysms rupture, they may cause a severe headache, a stiff painful neck, blurred or double vision, and photophobia.


Table 2 Extraocular muscles.


CN, cranial nerve.












































Muscle Actions Neural control
Medial rectus Adducts Oculomotor nerve (CN III)
Inferior rectus Mainly depresses, also extorts adducts Oculomotor nerve (CN III)
Superior rectus Mainly elevates, also intorts, adducts Oculomotor nerve (CN III)
Inferior oblique Mainly extorts, also elevates, abducts Oculomotor nerve (CN III)
Superior oblique Mainly intorts, also depresses, abducts Trochlear nerve (CN IV)
Lateral rectus Abducts Abducens nerve (CN VI)
Levator palpebrae Elevates upper lid Oculomotor nerve (CN III)
Müller’s muscle Elevates upper lid Sympathetic nerve
Orbicularis oculi Closes lids Facial nerve (CN VII)

Table 3 Nerves to ocular structures.


CN, cranial nerve.
































































Optic nerve, cranial nerve (CN) II The axon of the retinal ganglion cell which transmits visual impulse from the eye to the brain
Oculomotor nerve (CN III) Innervates Action
Motor (1–5) 1 Medial rectus muscle Adducts

2 Inferior rectus muscle Mainly depresses, also extorts, adducts

3 Superior rectus muscle Mainly elevates, also intorts, adducts

4 Inferior oblique muscle Mainly extorts, also elevates, abducts

5 Levator palpebrae muscle Elevates upper lid
Parasympathetic (6 and 7) 6 Pupil constrictor muscle Responds to light and near focus

7 Ciliary muscle Focuses lens for near
Trochlear nerve (CN IV) Superior oblique muscle Mainly intorts, also depresses, abducts
Trigeminal nerve (Fig. 108) CN V (Fig. 108); CN V branch 1: upper lid, orbit, and nose Sensory

CN V branch 2: lower lid
Abducens nerve (CN VI) Lateral rectus muscle Abducts
Facial nerve (CN VII, Fig. 110) Orbicularis muscle Closes upper and lower lids
Sympathetic nerve (Fig. 126) 1 Müller’s muscle 1 Elevates upper lid

2 Pupil dilator muscle 2 Opens pupil in response to stress, “fight or flight,” and adrenergic drugs

3 Skin of lid 3 Sweat glands
Schematic illustration of types of strabismus.

Fig 82 Types of strabismus.


Source: Aksanaku/Shutterstock.com.


Strabismus


Strabismus (Table 4, p. 32) refers to the nonalignment of the eyes such that an object in space is not visualized simultaneously by the fovea of each eye (see Table 4).


If one eye is occluded while both eyes are fusing, the occluded eye may turn in (esophoria, noted with the letter E) or out (exophoria, X). Small phorias are usually asymptomatic. A phoria may degenerate into a tropia. A tropia is an eye‐turn that occurs spontaneously. A tropia is more likely to occur as the amount of the phoria increases and as the patient’s ability to compensate decreases. This occurs with tiredness later in the day and from any stimulus that dissociates the eyes, such as poor vision in one eye. Absence of a phoria (perfectly straight eyes) is termed orthophoria.


Table 4 Types of eye‐turn.





















Esotropia (ET) Deviation of eye nasally
Exotropia (XT) Deviation of eye outward (temporally)
Hypertropia (HT) Deviation of eye upward
Intermittent tropia A phoria that spontaneously breaks to a tropia; indicate with parentheses. Example: R (ET) = right intermittent esotropia.
Constant monocular tropia Present at all times in one eye. Example: RXT, constant right exotropia. Often associated with loss of vision, if onset is in childhood.
Alternating tropia Either eye can deviate. Vision is usually equal in both eyes.

Complications of strabismus


Amblyopia


Also called lazy eye, amblyopia is decreased vision due to improper use of an eye in childhood. The two common causes are an eye‐turn (strabismic amblyopia) or a refractive error (refractive amblyopia), uncorrected before age 8. In strabismus, children unconsciously suppress the deviated eye to avoid diplopia.

Photo depicts patching for amblyopia.

Fig 83 Patching for amblyopia.


Strabismic amblyopia is treated by patching the good eye (Fig. 83), thereby forcing the child to use the amblyopic eye. The better eye is patched full time: 1 week for each year of age. It is repeated until there is no improvement or if the vision drops off again on cessation of patching.


Refractive amblyopia is treated by correcting the refractive error with glasses and patching the better eye. Both types must be treated in early childhood because after age 5 it is difficult to improve vision. After age 8, improvement is almost impossible, but should be tried.


Poor cosmetic appearance


Tropias that cannot be corrected with spectacles may be cosmetically unacceptable and the patient may desire surgery.


Loss of fusion (binocular vision)


Fusion occurs when the images from both eyes are perceived as one object, with resulting stereopsis (three‐dimensional vision). Many patients with tropias never gain the ability to fuse. Finer grades of fusion are assessed by using the Wirt stereopsis test (see Fig. 84).

Photo depicts Wirt stereopsis.

Fig 84 Wirt stereopsis.


While wearing polarized glasses, the patient views a test card. The degree of fusion is determined by the number of pictures correctly described in three dimensions.


Near point of convergence (NPC) (Fig. 85)

Photo depicts near point of convergence.

Fig 85 Near point of convergence.


The NPC is the closest point at which the eyes can cross to view a near object. It is measured by having the patient make a maximal effort to fixate on a small object as it is moved toward his or her eyes. The distance at which the eyes stop converging and one turns out is recorded as the NPC. Convergence insufficiency must be considered if the NPC is greater than 8 cm. These patients may complain of diplopia or other difficulties while reading and is common in patients with Parkinson’s disease. Exercises or prism glasses may help.


Accommodative esotropia (Figs 86 and 87)


When the lens of a normal eye focuses, it simultaneously causes the eyes to converge. Patients with hyperopia who are not wearing glasses must focus the lens of their eye (accommodation) to see clearly near and far. This focusing stimulates the accommodative reflex, causing convergence of the eyes. When the ratio of convergence to accommodation is abnormally high, an esotropia results, which corrects with lenses.

Photo depicts accommodative esotropia.

Fig 86 Accommodative esotropia.

Photo depicts accommodative esotropia corrected with hyperopic lenses.

Fig 87 Accommodative esotropia corrected with hyperopic lenses.

Schematic illustration of recession to weaken muscle.

Fig 88 Recession to weaken muscle.

Schematic illustration of resection to strengthen muscle.

Fig 89 Resection to strengthen muscle.


Nonaccommodative esotropia (Figs 89 and 90)

Photo depicts strabismus surgery: after incising the conjunctiva, the medial rectus muscle is exposed and isolated with two muscle hooks.

Fig 90 Strabismus surgery: after incising the conjunctiva (C), the medial rectus muscle is exposed and isolated with two muscle hooks.


Source: Courtesy of Elliot Davidoff, MD.


This is due to a defect in the brain not related to the accommodative reflex. It is corrected by surgically weakening the medial rectus muscle by recessing its insertion posteriorly on the sclera or by tightening the lateral rectus muscle by resecting part of it (Figs 88 and 89). Less often, botulinum toxin is injected to weaken eye muscles. Adjustable sutures with slip knots could enable the tension on the muscle to be altered during the early postoperative period.


An epicanthal skin fold connects the nasal upper and lower lids (Fig. 91) and is common in infants and Asians. It gives the false impression of a cross‐eye, called pseudostrabismus.

Photo depicts epicanthal folds causing a false impression of cross-eye.

Fig 91 Epicanthal folds causing a false impression of cross‐eye (pseudostrabismus).


Measurement of the amount of eye‐turn with prisms


Ocular deviations are measured in prism diopters. When light passes through a prism, it is bent toward the base of the prism. One prism diopter (1 Δ) displaces the image 1 cm at a distance of 1 m from the prism. Do not confuse prism diopters (Δ) with lens diopters (D).


In a right esotropia, the right fovea is turned temporally. To focus the light on the right fovea, a prism (apex‐in) is placed in front of the right eye (Fig. 92). For an exotropia, use apex‐out. Rule: point the prism apex in the direction of the tropia.

Schematic illustration of right esotropia neutralized with prism.

Fig 92 Right esotropia neutralized with prism (apex‐in).


Prism cover test for measurement of eye‐turn (Fig. 93)

Photo depicts prism cover test.

Fig 93 Prism cover test.


The patient fixates on an object at 20 ft (6 m). When the fixating eye is occluded, the deviated eye must move to look at the target. Increasing amounts of prism are placed in front of the deviated eye until no movement is noted when the cover is moved back and forth over each eye.


Hirschberg’s test


When the cover test is difficult to perform on infants, the angle of strabismus can be estimated by using Hirschberg’s test (Figs 9496). As the child fixates on a point source of light, the position of the corneal light reflex is noted. Each 1 mm of deviation from the center of the cornea is equivalent to approximately 14 Δ of deviation. A reflex 2 mm temporal to the center of the cornea indicates an esotropia of approximately 28 Δ.

Photo depicts Hirschberg: esotropia.

Fig 94 Hirschberg: esotropia.

Photo depicts Hirschberg: exotropia.

Fig 95 Hirschberg: exotropia.

Photo depicts Hirschberg: left hypotropia.

Fig 96 Hirschberg: left hypotropia.


Causes of strabismus



  1. Paralytic strabismus is due to cranial nerve (III, IV, or VI) disease or eye‐muscle weakness from thyroid disease, traumatic contusions, myasthenia gravis, or orbital floor fractures.
  2. Nonparalytic strabismus is due to a malfunction of a center in the brain. It is often inherited and begins in childhood. Exotropias occur in over 1% of American children and may be treated for cosmetic reasons, to restore binocularity, or for headache and eye fatigue resulting from efforts to keep the eyes straight. Treatment may include eye exercises, prism glasses, or eye muscle surgery.

Demonstration of paralytic strabismus (Table 5)


In paralytic strabismus, the amount of deviation is greatest when gaze is directed in the field of action of the weakened muscle. To demonstrate underaction of any of the 12 external ocular muscles, the patient fixates on an object moved into each of the six cardinal fields of gaze (Fig. 97). Each position tests one muscle of each eye (e.g., position 3 tests the right inferior rectus and the left superior oblique muscles). In addition to observing for underaction or overaction of the muscles, ask the patient where diplopia is greatest. For exact measurements, use the prism cover test.


Most often the cause for cranial nerve (CN) III, IV, and VI paralysis cannot be confirmed, since it is due to ischemia from small‐vessel closure. In adults, ischemia from diabetes is the most common cause and often resolves within 10 weeks. Testing is done to rule out causes such as multiple sclerosis, aneurysms, neoplasms, and other rarer conditions, especially in younger individuals where vessel closure is not likely.


Table 5 Comparison of paralytic and nonparalytic strabismus.




























Paralytic Nonparalytic
Age of onset Usually in older persons Usually starts before 6 years of age
Complaint since Diplopia Cosmetic eye‐turn; less diplopia: child suppresses deviated eye
Eye‐turn Largest deviation in field of action of affected muscle No one muscle is underactive; deviation similar in all directions
Vision Not affected Deviated eye may have loss of vision (amblyopia)
Plan Neurologic workup Ophthalmic workup
Schematic illustration of the six cardinal fields of gaze.

Fig 97 The six cardinal fields of gaze.

Photo depicts Right CN III paralysis. In straight gaze, eye turns down and out with dilated pupil and ptosis.

Fig 98 Right CN III paralysis. In straight gaze, eye turns down and out with dilated pupil and ptosis.


Cranial nerves III–VIII


Oculomotor nerve (CN III)


CN III paralysis (Figs 98100) results in underaction of the inferior oblique and medial, inferior, and superior rectus muscles, resulting in an eye turned down and out. Since this nerve also innervates the levator palpebral muscle, which elevates the lid and the pupillary constrictor muscle, the lid is drooped and the pupil is dilated. CN III paralysis due to diabetes often spares the pupil.

Photo depicts inability of right eye to look to the left due to medial rectus paralysis.

Fig 99 Inability of right eye to look to the left due to medial rectus paralysis.


Always examine for a dilated pupil after head trauma. CN III parallels the posterior communicating artery (see Fig. 81) so that ruptured aneurysms in the circle of Willis are a common cause of paralysis with a dilated pupil and an explosive headache (Figs 101 and 102). Also, CN III passes under the tentorial ridge in the brain and is highly susceptible to uncal herniation of the brain. Herniation may follow increased intracranial pressure from cerebral edema, hematoma, tumor, abscess, or cerebral spinal fluid obstruction. Although a dilated pupil is a more common ominous sign after head injury, small or unequal pupils could indicate serious insults to other parts of the brain.

Photo depicts inability of the right eye to look up to right due to superior rectus paralysis.

Fig 100 Inability of the right eye to look up to right due to superior rectus paralysis.


Source: Courtesy of David Taylor.

Photo depicts cerebral angiogram of right carotid artery showing a 3 mm × 4 mm posterior communicating arterial aneurysm. This occurred in a 50-year-old man with a subarachnoid hemorrhage and the worst headache of his life. Fifteen percent of patients with subarachnoid hemorrhages die before reaching the hospital. Aneurysms may be surgically clipped or obliterated with endovascular coiling.

Fig 101 Cerebral angiogram of right carotid artery showing a 3 mm × 4 mm posterior communicating arterial aneurysm (↑) (Figs 81 and 144). This occurred in a 50‐year‐old man with a subarachnoid hemorrhage and the worst headache of his life. Fifteen percent of patients with subarachnoid hemorrhages die before reaching the hospital. Aneurysms may be surgically clipped or obliterated with endovascular coiling.

Photo depicts stent-assisted platinum coiling embolization of aneurysm. A small electric charge is sent to the linear platinum tip when it enters the aneurysm. This charge detaches it, causes folding, and promotes thrombosis.

Fig 102 Stent‐assisted platinum coiling embolization of aneurysm. A small electric charge is sent to the linear platinum tip when it enters the aneurysm. This charge detaches it, causes folding, and promotes thrombosis.


Source: Courtesy of Stavropoula I. Tjoumakaris, MD, and Robert Rosenwasser, MD, Thomas Jefferson University Hospital Endovascular Neurological Surgery Department.

Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Nov 20, 2022 | Posted by in OPHTHALMOLOGY | Comments Off on Neuro‐ophthalmology

Full access? Get Clinical Tree

Get Clinical Tree app for offline access