Ординатура / Офтальмология / Английские материалы / Neuro-Ophthalmology_Kidd, Newman, Biousse_2008
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1 Neuro-Ophthalmologic Anatomy and Examination Techniques |
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Figure 1–12 Diagram of the pupillary light reflex pathway. (From Kardon RH: Anatomy and physiology of the autonomic nervous system. In Miller NR, Newman NJ (eds): Walsh and Hoyt’s Clinical Neuro-Ophthalmology, 6th ed. Philadelphia, Lippincott, Williams & Wilkins, 2005, p 664.)
neuron descends caudally from the hypothalamus to the first synapse, which is located in the cervical spinal cord (levels C8-T2—ciliospinal center of Budge). The second-order neuron travels from the sympathetic trunk, through the brachial plexus, over the lung apex, and ascends to the superior cervical ganglion. This ganglion is located near the angle of the mandible and the bifurcation of the common carotid artery. Distal to the superior cervical ganglion (second synapse in pathway), the third-order neuron then ascends within the adventitia of the internal carotid artery and through the cavernous sinus. The oculosympathetic neuron then joins the ophthalmic (V1) division of the fifth cranial nerve (trigeminal nerve). In the eye and orbit, the oculosympathetic fibers innervate the iris dilator muscle as well as Mu¨ller’s muscle, a small smooth muscle in the eyelid responsible for a minor portion of upper eyelid elevation and lower lid depression. Note (Fig. 1–13) that those sympathetic fibers responsible for facial sweating and vasodilation branch off at the superior cervical ganglion from the remainder of the oculosympathetic pathway, explaining why patients with
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Figure 1–13 The oculosympathetic pathways. Hypothalamic sympathetic fibers descend to the ciliospinal center of Budge (first-order neuron). The second-order neuron takes a circuitous course through the posterosuperior aspect of the chest and ascends in the neck in relationship with the carotid system. Third-order neurons originate in the superior cervical ganglion and are distributed to the face with branches of the external carotid artery and to the orbit via the ophthalmic artery and ophthalmic (V1) division of the fifth (trigeminal) nerve. (From Liu GT: Disorders of the eyes and eyelids: Disorders of the pupil. In Samuels MA, Feske S (eds): The Office Practice of Neurology. New York, Churchill Livingstone, 1996, p 62.)
third-order neuron Horner’s syndrome (oculosympathetic paresis) may not have anhidrosis. The signs of Horner’s syndrome, including mild ptosis, pupillary miosis, and, in some cases, anhidrosis, always occur ipsilateral to the side of the lesion, as there is no decussation or cross-over of the oculosympathetic pathway.
1 Neuro-Ophthalmologic Anatomy and Examination Techniques |
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The Neuro-Ophthalmologic Examination
In this section, we review the components of the neuro-ophthalmologic examination and the techniques necessary to assess the patient’s vision, pupillary function, ocular motility, and funduscopic findings.
VISUAL ACUITY
The neuro-ophthalmologic examination should begin with an assessment of visual acuity. The standard method of testing visual acuity at distance uses the Snellen chart placed at 20 feet (6 meters).23 The patient is asked to cover one eye either by using a tissue or an occluding device. The patient should be wearing glasses or contact lenses because the examiner is most interested in the best corrected acuity. It is not uncommon for meaningless, subnormal acuities to be recorded while the patient’s glasses lie in a pocket or in a drawer. If the patient’s glasses are unavailable or subnormal acuity is obtained despite glasses, a pinhole test should be performed (Fig. 1–14). A pinhole device focuses a small point of light on the retina permitting a window of near optimal refraction. Most Snellen lines end with the numbers to let the examiner know what line is being tested. For example, the 20/30 line ends with a 3 and the 20/25 line ends with numbers 2–5. Acuity is recorded for the right eye first. The numerator represents the testing distance, which is 20 feet. The denominator denotes the size of the letter seen or denotes the distance at which an eye with normal vision sees the same letter. The 20/40 line contains letters twice the size of the 20/20 line. However, the ability to read the 20/40 line correlates with approximately 85% of normal visual function. If the patient cannot read the 20/400 E, then a 200 size E can be slowly brought toward the patient (Fig. 1–15). For instance, if the patient sees the E at 5 feet from their eye, vision is recorded as 5/200 E. More severe acuity loss may be recorded as count fingers, hand motion, light perception, or no light perception. Abnormal visual acuity may be the result of the following:
1.Refractive error (cornea, lens, vitreous)
2.Optic nerve or chiasmal disease and rarely from a bilateral cortical lesion
3.Macular dysfunction
4.Amblyopia
5.Functional visual loss
Figure 1–14 A pinhole device will focus a point of light on the retina correcting most refractive errors.
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Figure 1–15 When the patient sees less than 20/200, but better than hand motions, a 200 sized E can be brought toward the patient. If the patient is able to see the E at 4 feet from the eye, visual acuity is recorded as “4/200.”
Once again, the importance of the pinhole examination cannot be overemphasized in this differential diagnosis because it will correct most refractive errors. If acuity cannot be corrected by a pinhole device, then optic nerve disease, macular dysfunction, or amblyopia may be present. These entities are further distinguished by visual field, pupillary, and funduscopic examination. Amblyopic patients usually have a long-standing history of visual loss in one eye resulting from strabismus, asymmetric refractive error, or a media opacity.
NEAR VISION
Despite the accuracy of a Snellen acuity, it is often necessary to assess acuity at near. Near vision may be tested using the Rosenbaum hand held card (Fig. 1–16). Because the test card has been designed to be held at 14 inches (3 5 cm) from the patient, any deviation of the testing distance profoundly affects the acuity equivalent seen. It is for this reason that most ophthalmologists prefer to use the Jaeger numbers. Thus, a near acuity of 20/25 may be written as J1 at 14 inches. Reading cards are also available and they provide an added benefit by testing for alexia. Once again, it must be emphasized that an uncorrected near acuity is virtually useless.
Figure 1–16 The Rosenbaum near card. Note near acuity is often recorded using the Jaeger numbers. For instance, “J1” is equivalent to a 20/25 near acuity.
1 Neuro-Ophthalmologic Anatomy and Examination Techniques |
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COLOR VISION
Color vision may be assessed using the Ishihara or the Hardy-Rand-Rittler (HRR) color plates series (Fig. 1–17). We prefer the latter because it is designed to test both acquired and congenital color blindness (includes yellow-blue plates). The Ishihara series only tests for congenital color blindness (red-green plates only). The major utility of color plate testing is to detect the difference in color perception between the two eyes. Most color plates contain numbers or geometric shapes created by a series of colored dots. The examiner should record the number of plates correctly identified by each eye. Nearly 9% of males and 1% of females are congenitally color blind.21 When color plates are unavailable, difference in color perception between the two eyes may be identified using a color pen or bottle top (Fig. 1–18). Even with normal color plates testing, the patient may readily recognize a color difference in a red bottle top alternately presented to each eye. The patient may tell the examiner that one eye sees the bottle top as pink or orange. The patient may also be able to give the examiner
Figure 1–17 Pseudo-isochromatic color plates. A, In the Ishihara color series, the patient is asked to identify the number among the colored dots. B, In the Hardy Rand Rittler series, the patient is asked to identify the geometric shapes.
Figure 1–18 When color plates are unavailable, the examiner may use a color bottle top to detect a color difference between the two eyes.
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a percentage of this desaturation. On occasion, green or blue bottle tops may detect a color difference not appreciated when the red tops were used. More extensive color vision testing can be carried out using the color sorting tests known as the Farnsworth-Munsell 100 hue test or the Farnsworth D-15 panel test.24
Acquired dyschromatopsia usually results from retinal or optic nerve lesions. In general, dyschromatopsia is more common with optic nerve lesions even if the visual acuity loss is mild. Dyschromatopsia secondary to retinal disease is typically associated with funduscopic abnormalities such as large macular scarring or diffuse pigmentary disturbances.
CONTRAST SENSITIVITY AND LOW-CONTRAST LETTER ACUITY
Eye charts have been designed for maximal contrast (i.e., dark black letters on a white background). Contrast sensitivity testing uses shades of gray to better assess visual resolution problems. In some contrast sensitivity tests, the distance between the bars (thickness) can be altered to give various spatial frequencies; other types of contrast sensitivity testing capture minimum level of contrast perception using large sized letters (Pelli-Robson chart, as used in the Optic Neuritis Treatment Trial, has letters that correspond to 20/680 Snellen equivalent). However, patients with neurologic disorders may have selective losses of contrast vision at small letter sizes ( 20/25 or 20/20 Snellen equivalent); low-contrast letter acuity charts (Sloan charts, Fig. 1–19) may have even greater sensitivity for capturing visual dysfunction in neuro-ophthalmologic patients. In addition to optic neuropathies, macular disease and media opacities such as cataracts may be associated with impaired contrast sensitivity and low-contrast acuity. Contrast sensitivity is only one measure of visual function and should not be substituted for all other measures.25
Figure 1–19 Low-contrast letter acuity charts (Sloan charts) measure patients’ capacity to perceive letters of progressively smaller size on a white background.
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1 Neuro-Ophthalmologic Anatomy and Examination Techniques |
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Figure 1–20 The Amsler grid resembles graph paper and may be used to assess macula function or the central ten degrees of the visual field.
AMSLER GRID TESTING
The Amsler grid, which resembles graph paper, is an extremely useful test to detect macular abnormalities as a cause for central acuity loss (Fig. 1–20). Each eye is tested separately. The patient should fixate on the central dot and determine whether all the lines on the paper are straight and whether all lines are present. The patient with a maculopathy will often see the straight lines as curved (metamorphopsia). Patients may also detect abnormal areas of the Amsler grid that correspond with their visual field loss. For documentation, the patient may draw the perceived field defect on the Amsler grid paper.
VISUAL FIELD TESTING
The field of each eye is tested individually. The first step is to have the patient examine the examiner’s face. One should ask them to fixate on the examiner’s nose. If the nose is not clear to the patient, this implies a central scotoma. One should have the patient compare the examiner’s eyes and upper and lower face for any difference. Altitudinal or hemianopic defects may be readily detected by this method. The next step is to have the patient count fingers in the four quadrants (Fig. 1–21). Simultaneous presentations of fingers in two separate quadrants increase the yield of finding a field defect. The examiner should hold up both hands on each side of the vertical or horizontal meridian and have the patient compare them for clarity (Fig. 1–22). If a field defect is found, the hand is moved from the defective field to the normal one to establish the boundaries of the defect. Red bottle tops can also be used in a similar fashion. For example, when testing for a central scotoma, a red bottle top is placed in front of the examiner’s nose and another cap is held slightly off the midline. If the patient sees the cap held in the periphery as a better red, a central scotoma is suggested. Visual fields are recorded from the patient’s perspective (Fig. 1–23).
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Figure 1–21 Confrontation visual field testing. Each eye is tested separately. The patient is asked to count the number of fingers that are flashed in crossed quadrants of the field.
Figure 1–22 When checking for a subtler hemianopia, the patient is asked to compare the clarity of two hands placed (A) on each side of the vertical meridian; (B) Two red bottle tops can be used in a similar fashion.
Patient’s view: Left homonymous inferior quadrantanopsia
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Figure 1–23 Visual fields are recorded in the chart from the patient’s perspective. CF, counting fingers. In this diagram, the patient has a left inferior quadrantanopsia.
1 Neuro-Ophthalmologic Anatomy and Examination Techniques |
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Visual field defects may be associated with disorders of higher cortical function. Patients with neglect are usually able to perceive a single stimulus such as a finger. However, as soon as the examiner uses double simultaneous stimulation across the vertical meridian of the field, one of the stimuli will be neglected. Neglect is often seen with parietal lesions.
Patients with visual agnosia have altered visual perception. For instance, patients with prosopagnosia are unable to recognize familiar faces. This might be tested by having the patient examine recognizable faces published in a newspaper or magazine. This is also a useful technique to examine for simultagnosia (the inability to interpret a complex visual scene). Central achromatopsia typically results from bilateral occipitotemporal lesions. Such patients are unable to sort isoluminent colored threads or discs. Cortical visual disorders are covered in Chapter 14.
TANGENT SCREEN FIELD TESTING
The tangent screen is a black spider-web-like mural that hangs in many offices. This testing instrument can provide invaluable information about the central 30 degrees of the visual field. The patient is placed at 1 meter from the screen. In the traditional format, small white discs are presented kinetically and statically (usually at least a 3- and 6-mm white disc are used). Alternatively, a projection light or laser pointer can be used from behind the patient (Fig. 1–24). The major advantage of the laser pointer technique is the patient will be unaware of the target origin. This technique is invaluable for the hospitalized patient who is unable to leave the inpatient floor. The wall of the patient’s room can also serve as a makeshift tangent screen.
Tangent screen examination is also useful in evaluating functional visual disorders. The basic principle that the field should expand as the patient is moved away from the tangent screen is rarely appreciated by the functional patient. The patient is first tested at 1 meter using a 3-mm white target. The field is plotted and then the patient is retested at 2 m using a 6-mm white target (doubling the testing distance requires doubling the test target size to keep the testing conditions the same). Nonexpansion of the visual field at increasing testing distance is a nonphysiologic finding. In fact, it is not uncommon for the functional patient to further constrict the field at increasing distance (a reverse tunnel). The examiner may also do a similar but less accurate assessment of field expansion by using one finger at 1 foot and the entire hand at 5 feet from the patient.
Figure 1–24 Tangent screen testing with the patient placed at one meter from the screen. One eye is covered and the examiner is behind the patient using a laser pointer to map the visual field.
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FORMAL PERIMETRY
Automated (computer) perimetry has become the primary technique used to assess the visual field formally. Computerized perimetry is particularly useful to serially follow patients with glaucoma or papilledema. The introduction of the SITA fast programs has resulted in a 50% reduction in testing time, thereby permitting a larger cohort of patients who are able to tolerate the duration of this test. However, some patients are unable to concentrate well enough to perform computerized perimetry. In this case, Goldmann perimetry remains an excellent means to test for visual field defects in neuro-ophthalmic patients. It provides information about the central and peripheral field. The light used in the Goldmann perimetry may be varied in size (I ¼ smallest target, V ¼ largest target) or illumination (2E is not as bright as 4E). Some helpful rules in localizing visual field defects are presented in Table 1–2.
PUPILLARY EXAMINATION
Pupil size should be recorded in light and dark with the patient fixating at distance. The direct response to light is assessed in one eye and then in the other. The light is brought from below to avoid triggering a near reaction. Optimally, the light reaction of the pupil should be tested with the halogen transilluminator or indirect ophthalmoscope. A pupil gauge is conveniently located on the bottom of most near acuity cards. The direct response can be graded from 0 to 3þ:
0 ¼ no reaction
1þ ¼ sluggish
2þ ¼ slightly sluggish
3þ ¼ normal
The near response is tested by having the patient look at his or her thumb as the examiner draws it closer. Again, the pupillary response is recorded on a scale of 0 to 3. One should remember the near response requires voluntary effort from the patient so one must encourage the patient vigorously. Light-near dissociation of the pupil is tested with a light directed at one pupil. The patient is then asked to look at his or her thumb, which is held at 14 inches. A much better pupillary response to near suggests light-near dissociation of the pupils.
The afferent pupillary defect represents a sign of asymmetric optic nerve or severe retinal dysfunction (Fig. 1–25). To perform this test, one should have the patient fixate at distance and one should quickly shine the light in each eye to
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TABLE 1–2 |
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Visual Field Gems: Keys to Localization of Defects |
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Localization |
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Vertical and horizontal meridians not respected |
Retina and choroid |
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Horizontal meridian respected |
Optic nerve |
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Vertical meridian respected |
Chiasm, retrochiasmal |
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Vertical and horizontal respected in both eyes |
Occipital cortex |
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Central scotoma and normal blind spot |
Macula, optic nerve |
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Central scotoma and enlarged blind spot |
Optic nerve |
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