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Neuro-Ophthalmic Exam from Ophthalmology/Neurologic Disorders
Author Information | Introduction & Initial Exam | Sensory Testing, Acuity Testing & Color Vision | Amsler Grid And Visual Fields | Pupils, Anterior Segment And Fundi | Ocular Motility And Head Posture | Eyelids & Orbit | Cranial Nerves V, Vii & Viii And Non-ocular Exam | Increasing Exam Efficiency, The Bedside Exam & Functional Visual Loss | Pictures | Bibliography

AUTHOR INFORMATION Section 1 of 11    Click here to go to the top of this page Click here to go to the next section in this topic

Authored by Edsel Ing, MD, FRCS, Consulting Staff, Department of Oculoplastics and Neuro-Ophthalmology, MD Eye Clinic

Edited by Andrew W Lawton, MD, Medical Director, Baptist Eye Center, Baptist Health Medical Center; Donald S Fong, MD, MPH, Assistant Clinical Professor of Ophthalmology, UCLA School of Medicine, Department of Ophthalmology, Southern California Permamente Medical Group; Brian R Younge, MD, Military Consultant to the Surgeon General in Neuro-Ophthalmology, Associate Professor, Department of Ophthalmology, Mayo Clinic; Lance L Brown, OD, MD, Consulting Staff, Department of Ophthalmology, University of Kansas Medical Center; and Hampton Roy, Sr, MD, Clinical Associate Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Topic Last Updated: May 18, 2000
Author's Email: Edsel Ing, MD, FRCSClick here to view conflict-of-interest information on the author of this topic
Editor's Email:Andrew W Lawton, MD

INTRODUCTION & INITIAL EXAM Section 2 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Introduction:

Initial Exam:

  • Although the "Neuro-ophthalmic history" and "Neuro-ophthalmic exam" are separate chapters in eMedicine text, in actual practice, the two processes occur somewhat concurrently. Findings on the physical exam guide further questioning.

  • Much information can be garnered if the physician has time to escort the patient to the examining lane.

    • The patient's behavior in the waiting room, interaction with family or caregivers, the patient's gait and navigational ability can be ascertained.

    • The examining lane should be brightly illuminated to allow perusal of the patient’s face, and eyes. Anomalous head posture such as head tilt, which may suggest contralateral fourth nerve palsy, should be noted.

    • The patient's dress and cleanliness are often a clue to visual function. When shaking hands, the patient's fingers, skin and grip should be noted. Mental status, speech and affect can be assessed during the history taking.
SENSORY TESTING, ACUITY TESTING & COLOR VISION Section 3 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Sensory Testing:

  • Stereo vision testing and Worth 4 dot testing are not routine parts of the neuro-ophthalmic exam but are useful in the situations delineated below.

    • Sensory testing is best performed prior to disrupting fusion (i.e., occlusion of one eye). The author's preferred stereo vision test is the Titmus stereofly.

      • Stereo vision testing is commonly used in pediatric ophthalmology exams, and useful in patients with suspected functional visual loss.

      • A normal stereo vision test suggests 20/20 acuity in both eyes and is not compatible with poor visual acuity.

    • Worth 4 dot testing is useful if patients have difficulty expressing their diplopia.

      • In patients with strabismus and good acuity in both eyes, central suppression suggests an element of amblyopia rather than an acquired motility deficit.

      • The green filter should be placed before the better-seeing eye. The red-green glasses can also be used for duochrome tests of patients suspected of malingering.

Refraction:

  • The best possible refraction should be obtained. Although measuring pinhole acuity is useful, it should be remembered that the pinhole only corrects about 3 diopters of refractive error.

    • Some patients may have a significant refractive error that may not improve with pinhole due to macular disease or hand tremor.

  • Some patients with poor mentation or poor head control are unable to use the phoropter. In such patients refraction should be done with a trial frame.

  • It is not uncommon for patients with keratoconus to present to the neuro-ophthalmologist with diplopia or blurry vision.

  • Scissoring of the retinoscopic reflex, or irregular keratometry mires may be helpful to record, if corneal topography is not readily available.

  • Cycloplegic refraction should be considered in patients with comitant esotropia, unexplained headache, or unexplained visual complaints.

Visual Acuity:

  • Documentation of the best-corrected visual acuity is essential. The patient should be carefully observed during acuity testing to ensure that the chart is being read monocularly. In children the author routinely tapes a tissue over the eye not being tested.

  • Patients with homonymous hemianopsia may "cut" i.e., ignore relevant portions of the eye chart, or adopt anomalous head postures.

  • Correlation of the distance vision with the near acuity is invaluable. If near vision is better than distance vision or vice versa, ensure that the best refraction was in place and that the distance at which the near card was held was appropriate.

    • Some illiterate patients may read the numbers on the standard near card quite readily, yet be unable to read the letters on conventional distance charts.

    • In young patients, illiterate patients, or patients with decreased mentation, it is useful to have a HOTV chart, Sheridan Gardner chart, illiterate E charts, or Landolt C charts. Optokinetic response suggests the patient has at least 20/400 vision.

  • In patients with decreased Snellen acuity, who have no visual complaints, exclude latent nystagmus.

    • In patients with latent nystagmus, document binocular visual acuity, and check the vision monocularly while fogging the other eye with an overplussed refraction.

Color Vision:

  • The Ishihara color plates are a commonly used, effective means of screening color vision. Patients with vision as poor as 20/200 may still be able to see the control plate of the Ishihara.

  • In patients who are congenitally red-green defective, the Hardy-Rand-Rittler plates may be useful. Asymmetry in the speed of recognition of the color plates may be significant.

  • The Farnsworth Panel D-15, and Farnsworth-Munsell 100-hue test are more involved color vision tests that allow determination of the axis of dyschromatopsia. The latter test also indicates the severity of the dyschromatopsia.

Other Tests:

  • The author does not routinely perform spatial contrast sensitivity testing, or photostress recovery. Patients with normal Snellen acuity may have elevated contrast thresholds, but unfortunately there is not always a clear-cut delineation between normal and abnormal patients.

  • Visually-evoked cortical potentials (VEP) may be useful in non-verbal or malingering patients.

  • Electroretinograms (ERG) may be useful when retinal dystrophy cannot be distinguished from optic neuropathy.

  • The author employs VEP and ERG in only a select minority of neuro-ophthalmic patients.
AMSLER GRID AND VISUAL FIELDS Section 4 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Amsler Grid:

  • The Amsler grid is a valuable part of the neuro-ophthalmic exam, and its use should not be restricted to patients with macular degneration.

    • Amsler testing is performed monocularly with the near correction in place, if applicable. The Amsler grid may detect small central or paracentral scotomas missed between the 6 degree spacing of standard threshold automated perimetry.

    • The small homonymous paracentral scotomas of occipital stroke are more efficiently screened with the Amsler grid versus central 10-degree automated perimetry. Confrontation fields at 4 meters followed by tangent screen examination may reveal a small homonymous scotoma.

  • Some patients with unexplained visual blurring may be referred for neuro-ophthalmic exam only to be discovered to have metamorphopsia i.e., the straight lines of the Amsler grid are distorted.

  • Some neurologists may mistake central serous retinopathy for optic neuritis. The former will often show metamorphopsia.

Confrontation Fields:

  • Field defects respecting the vertical midline usually indicate chiasmal or retrochiasmal pathology.

  • Field defects respecting the horizontal midline usually indicate ocular disease from nerve fiber layer involvement or branch retinal vessel occlusion. A rare exception to this rule is occipital stroke above or below the calcarine fissure.

  • Confrontation fields are an essential part of the eye exam. It is not uncommon for patients to mistake homonymous visual loss for ipsilateral monocular visual loss. Furthermore cognitively-impaired patients may be incapable of performing formal perimetry.

    • They should be performed monocularly with the patient fixating on the examiner's nose. By convention confrontation field defects should be recorded from the perspective of the patient rather than the examiner.

    • Some examiners test confrontation fields by moving a finger, pen, or red-headed pin inwards form the periphery while the patient is fixating centrally.

    • The patient is asked to indicate if and when they see the moving test object. There are some potential drawbacks to this method of kinetic testing.

      • The speed at which the test object is moved may cause considerable variation in the results. Moving objects are seen more readily than static ones (statokinetic dissociation) rendering the kinetic test potentially less sensitive.

      • Finally the "yes", "no" responses are somewhat nebulous compared to the exact quantitative response required by finger counting.

    • The author prefers to test confrontation fields by simultaneous finger presentations across the vertical midline in the upper and then the lower quadrants.

    • If the patient is unable to count fingers, then hand motions, and light perception can be tested in each quadrants.

    • If a subtle chiasmal lesion is suspected, desaturation of the palms of the hands, or a red bottle top across the vertical midline may be useful to document prior to formal perimetry.

    • In some cognitively-impaired patients, confrontation fields may provide a more accurate indication of the patient's problem than automated perimetry.

    • If a patient does not fixate well for confrontation field-testing, it is unlikely that accurate tangent screen, Goldmann or automated perimetry can be performed.

      • Central 30-degree threshold automated perimetry will detect visual field defects of neuro-ophthalmic importance with the exception of the temporal crescent.

      • In neuro-ophthalmic patients, carefully performed Goldmann perimetry may yield results comparable to automated perimetry. The tangent screen is a useful screening device but results are difficult to reproduce.

      • In most ophthalmic practices, the tangent screen is reserved for patients with suspected malingering and tunnel vision.

        • The tangent screen is occasionally of use in patients with small central scotomas since the 1-meter test distance of the tangent screen yields scotomas 3 times larger than on standard perimetry based on a 33-cm test distance.
PUPILS, ANTERIOR SEGMENT AND FUNDI Section 5 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Pupils:

  • Pupils of any patient with unexplained visual loss, ptosis or dysmotility should not be dilated until reviewed by the senior examiner for the following reasons:

    • Pupil examination is one of the few objective indicators of potential visual function. The pupils are an important clue to patients with suspected functional visual loss.

    • Horners syndrome is easily missed, unless the combination of anisocoria worse in the dark, pupil dilation lag and ptosis can be correlated.

    • The presence or absence of pupil involvement markedly changes the management of new-onset third nerve palsy.

    • The pupils are clinically normal in myasthenia gravis.

  • When recording pupil size, a pupil gauge or millimeter rule should be used with the patient fixating in the distance, in both a dark and illuminated environment.

    • If there is a difference in the size of the pupils (anisocoria) determine if the anisocoria is greater in the dark or in the presence of light.

      • If the anisocoria is less than 1 mm, and remains the same in the light and dark and if the pupils are round and reactive, the patient may have physiologic anisocoria.

      • Anisocoria greater in the dark suggests sympathetic disease such as Horners syndrome.

      • Anisocoria greater in the light suggests parasympathetic disease such as Adies tonic pupil.

    • It should be noted that even if one eye is completely blind, in the absence of iris trauma, the pupil sizes should be equal.

  • The briskness of the pupil response should be noted. If the pupils do not react briskly, vermiform constriction, iris notches, and the response to near stimulus should be recorded.

    • The near reaction is best tested with an accommodative target such as a reading card. When testing the near response, do not use an illuminated light source as the accommodative target since the pupillary light response will be added to the near response.

      • If the pupil reacts better to near than with light, light-near dissociation is present. The most common cause of light-near dissociation is probably optic neuropathy.

      • Well known eponymous causes of light-near dissociation include Adies tonic pupil, Parinauds syndrome (dorsal midbrain syndrome), and Argyll Robertson pupil (tertiary syphilis).

  • Testing for a relative afferent pupillary defect (RAPD or Marcus Gunn pupil) is an essential part of any eye exam. The designation "relative" afferent pupillary defect is important.

    • Bilateral afferent pupillary defects are common (e.g., optic neuritis patients), but a bilateral relative afferent pupillary defect cannot exist.

    • In a dimly illuminated room, the patient fixates in the distance and a bright light is alternately flashed for 2-3 seconds in each eye.

    • If the light is directed towards one eye and the ipsilateral pupil appears to dilate paradoxically, an RAPD is present.

    • If the light is flashed on one eye and the pupil does not appear to constrict, or there is increased pupillary escape from constriction, an RAPD may also be present.

    • The RAPD can still be tested in patients where one pupil is dilated or unreactive. The examiner examines the pupillary reaction of the undilated pupil while the light is flashed back and forth between the two eyes.

    • In patients with strabismus, direct the light along the pupillary axis of each eye, so as not to induce an RAPD in the deviated eye.

    • Although an RAPD may correspond with ipsilateral poor vision, it is not always the case. For example, macular hole will result in poor acuity, but not result in an RAPD.

    • In patients who have marked unilateral field loss, or unilateral dyschromatopsia, there is usually an ipsilateral RAPD.

    • A lesion confined to the optic tract may cause an incongruous homonymous field defect and a contralateral RAPD.

    • In patients with lesions of the brachium of the superior colliculus, there may be a contralateral RAPD without field loss (midbrain RAPD).

  • The differential diagnosis of heterochromia iridum (more commonly known as heterochromia iridis) is vast. Patients with heterochromia iridum may present to the neuro-ophthalmologist with congenital Horners syndrome, Sturge-Weber syndrome, or unilateral glaucoma treated with latanoprost.

  • Slit lamp examination is of great utility when the pupil is suspected to be abnormal.

    • Slight irregularities in the shape of the pupil are best seen with the slit lamp. Irregularities in pupil shape may be congenital as with iris coloboma, or acquired as with synechiae (iritis), iris notches (trauma), and iridocorneal endothelial syndrome (glaucoma).

    • In patients with type I neurofibromatosis, Lisch nodules closely resembling iris nevi may be seen on the slit lamp.

    • Purse stringing of the pupil may be seen with Adies tonic pupil; it is best elicited by quickly turning on and off the slit-lamp beam while examining the pupil.

  • Pupil-gaze synkinesis is a sign of aberrant regeneration of the third cranial nerve. Most commonly the pupil constricts on adduction. While the patient is fixating in the distance, the size of the pupils in primary gaze to the pupil size on adduction is compared.

Anterior Segment:

  • In addition to the slit-lamp findings of abnormal pupillary function, several other anterior segment findings may be of neuro-ophthalmic import.

    • Cells and flare, keratic precipitates, and posterior synechiae are typical manifestations of iritis.

      • Iritis is seen with various neuro-ophthalmic conditions including sarcoidosis, Wegener's granulomatosis, and orbital inflammatory syndrome.

      • A low-grade iritis is seen in some cases of ocular ischemic syndrome caused by carotid occlusive disease.

    • The phakomatoses have particular anterior segment findings.

      • Type 1 neurofibromatosis patients may show Lisch nodules, as mentioned in the pupil section.

      • Type 2 neurofibromatosis may show posterior subcapsular cataracts.

      • Conjunctival telangiectasias are seen in patients with Louis-Bar syndrome (ataxia telangiectasia).

    • Corkscrew, arterialized conjunctival vessels and ipsilateral glaucoma suggests a dural arteriovenous malformation or carotid-cavernous fistula.

    • Acute angle-closure glaucoma is sometimes mistaken for cerebral aneurysm due to the dilated pupil, cephalgia and vomiting.

      • However the misty cornea, narrow angle in the ipsilateral and usually contralateral eye, and elevated intraocular pressure will differentiate the two entities.

    • Examination of the fornices in a patient with proptosis occasionally reveals the salmon patch lesions of orbital lymphoma.

Fundus:

  • Dilated fundus exam with stereo slit-lamp illumination and indirect ophthalmoscopy affords visualization of fundus details not possible by our non-ophthalmic colleagues. Notwithstanding, the utility of the direct ophthalmoscope should not be overlooked.

    • The direct ophthalmoscope provides very high magnification of fundus details.

    • Venous sheathing is most readily seen with the direct ophthalmoscope. Use of the red-free light may show nerve fiber layer defects.

  • There are numerous optic disc anomalies of neuro-ophthalmic significance, but only a select few will be reviewed.

    • Optic nerve hypoplasia is sometimes overlooked if the examiner does not appreciate the true nerve diameter in patients with the "double ring" sign.

      • CNS midline defects (e.g., abnormal hypothalamic-pituitary axis) should be excluded, especially if optic nerve hypoplasia is associated with increased venous tortuosity.

    • The differentiation between papilledema and pseudo-papilledema is discussed in a separate chapter. Findings suggestive of papilledema include blurring of the fine peripapillary vessels (due to nerve fiber layer edema), peripapillary hemorrhages, venous congestion, and disc leakage on fluorescein angiography.

      • The presence of spontaneous venous pulsations, disc drusen, or myelinated nerve fibers suggests pseudopapilledema.

    • Chalky white, pale, swollen discs are classically described in elderly patients presenting with giant cell arteritis.

    • Painless visual loss in a patient with sectoral disc edema, altitudinal field loss, and small cup disc ratios in a patient older than 50 years suggests non-arteritic ischemic optic neuropathy.

      • After about a month the disc edema resolves, and sectoral disc pallor will be seen. The author finds the red-free light most useful in detecting sectoral disc pallor in patients with suspected, past, non-arteritic ischemic optic neuropathy.

    • A swollen optic nerve with peripapillary telangiectasias, in the setting of painless visual loss, especially in a young male patient suggests Leber's hereditary optic neuropathy.

    • Optic atrophy with optociliary shunt vessels (optochoroidal collaterals) is classically described with optic nerve sheath meningioma, but is also seen in other conditions including central retinal vein occlusion, optic nerve glioma, and chronic papilledema.

    • Thinning of the neural rim, especially vertical notching may be seen with glaucoma. Peripapillary splinter hemorrhages (Drance hemorrhages) have been described with normal tension glaucoma. Disc pallor disproportionate to cupping suggests against glaucoma.

    • Many retinal findings are of potential neuro-ophthalmic import.

      • Central retinal artery occlusion may be seen with embolic disease as well as giant cell arteritis.

      • Multiple branch retinal artery occlusions in a patient with deafness and cerebrovascular accident may suggest Susacs syndrome.

      • Retinochoroidal striae may indicate an orbital mass lesion.

    • Some of the phakomatoses have classic fundus findings.

      • Astrocytic hamartomas (mulberry-like tumors) may be seen with tuberous sclerosis.

      • Retinal angiomatosis may be seen in patients with von Hippel's disease.

      • Retinal arteriovenous malformation may be seen with Wyburn-Mason syndrome.

      • Diffuse choroidal hemangiomas of Sturge-Weber syndrome (tomato catsup fundus) are best noted by comparing the two fundi during indirect ophthalmoscopy. Increased cupping may be seen in the involved eye of patients with Sturge-Weber syndrome.
OCULAR MOTILITY AND HEAD POSTURE Section 6 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Ocular Motility:

Anomalous Head Positions:

  • Anomalous head position may be due to ophthalmic or non-ophthalmic causes.

    • Non-ocular causes for abnormal head position include cervical torticollis, unilateral deafness and habit posture.

    • Ophthalmic causes for a head posture may be due to nystagmus, or non-nystagmus-related problems.

  • Patients with nystagmus may adopt a head posture to place the eyes in the null point.

  • Cranial nerve palsies may result in anomalous head posture.

    • Patients with a fourth nerve palsy usually tilt their head to the side opposite the palsy.

    • Patients with sixth nerve palsy will usually turn their head towards the side ipsilateral to the palsy to avoid diplopia.

  • Chin up positions may be seen with ptosis, A-pattern esodeviations or V-pattern exodeviations.

  • Abnormal head postures may be a clue to field loss.
EYELIDS & ORBIT Section 7 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Eyelids:

Orbit:

  • Pseudoproptosis may be seen with lid retraction, unilateral high myopia, facial asymmetry and contralateral enophthalmos.

  • Proptosis can be screened for by gazing from above the patient's forehead, and then quantified with an exophthalmometer.

    • When performing exophthalmometry record the base measurement. Orbital retropulsion is decreased with orbital mass-occupying lesions, and is useful to document in all patients with proptosis. If orbital compliance is markedly decreased, have an increased index of suspicion for compressive optic neuropathy.

  • The differential diagnosis for proptosis is vast.

    • In adults with proptosis look closely for lid lag and lid retraction, as thyroid-associated ophthalmopathy is the most common culprit in this age group.

    • In children with proptosis exclude orbital cellulitis and rhabdomyosarcoma.

  • Acquired enophthalmos may be seen following orbital fractures, neurosurgical decompression of the orbital walls, and with scirrhous breast carcinoma.

  • Pulsatile proptosis may occur with carotid-cavernous fistula, sinus mucoceles, neurofibromatosis with sphenoid wing dysplasia, and following removal of the bony orbital rim.

    • If spontaneous orbital pulsations are not readily visible, they can be examined for by placing long-handled cotton tip applicators over the closed eyelids and forehead, and watching for oscillations of the cotton tip applicators.

  • Orbital masses usually displace the globe in the direction opposite the tumor. For example frontal sinus mucocele may cause inferior globe displacement; intraconal tumors will cause axial proptosis.
CRANIAL NERVES V, VII & VIII AND NON-OCULAR EXAM Section 8 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Cranial Nerves V, VII, and VIII:

  • Numbness of the ophthalmic (V1), maxillary (V2) or mandibular (V3) divisions of the trigeminal nerve in a patient with a motility deficit may indicate a cavernous sinus process.

    • For screening purposes, the author simultaneously touches both sides of the forehead (V1), upper cheek (V2), and chin (V3) to see if there is asymmetry in sensation.

    • Traditionally corneal sensation (V1) is tested with dental floss or kleenex in four different quadrants of the cornea. Occasionally the author will place artificial tears in the eyes instead and ask the patient to compare corneal sensation.

  • Bell's palsy (idiopathic lower motor neuron seventh nerve palsy) is discussed in a separate chapter and will only be briefly mentioned here.

    • In acute lower motor neuron facial nerve palsies, both the forehead, eyelid and lower face will droop.

    • Orbicularis oculi function should be tested by asking the patient to gently close the eyelid as if they are sleeping. The amount of lagophthalmos can be measured with a millimeter rule.

    • The presence of absence of Bell's phenomenon should be documented as it indicates how well the cornea is protected when the patient is asleep. Corneal epithelial defects and corneal sensation should be noted.

    • Patients with seventh nerve palsy and corneal anesthesia may require early tarsorraphy. Loss of spontaneous blink may occur in patients with apparently normal voluntary lid closure, and is a helpful clue to suggest prior seventh nerve palsy.

    • Exam for otic vesicles (possible Ramsay-Hunt syndrome), palpation of the partoid gland with a gloved finger (e.g., epithelial tumors or sarcoidosis), and exam of the tongue for furrows (Melkerson Rosenthal syndrome) should be performed in patients with seventh nerve palsy.

  • It is important to test hearing in patients with nystagmus, hemifacial spasm, facial nerve weakness and Cogan's syndrome.

    • To test hearing, the author whispers a number in one ear while scratching a prescription pad over the patient's other ear (masking).

Non-ocular Exam:

  • Vital signs

    • Although vital signs are usually not recorded in ophthalmology patients, they may be important to record.

      • Blood pressure should be measured in patients with papilledema and fundus hemorrhages.

      • In patients with retinal emboli, checking the pulse may confirm suspicion of atrial fibrillation.

  • Auscultation

    • Intracranial bruits are often better appreciated by the patient than the examiner. The bell of the stethoscope can be applied to the affected orbit or temple to detect the bruit.

    • Cervical bruits may be auscultated in patients with retinal emboli or stroke. However, patients with a high grade or complete occlusion may not manifest a bruit.

  • Temple

    • A prominent temple may be seen ipsilateral to sphenoid wing meningioma.

    • Patients with giant cell arteritis may have ropey temporal arteries. There may be asymmetry in the pulse of the superficial temporal artery.

      • Point tenderness of the artery may help guide temporal artery biopsy.

      • Prior to temporal artery biopsy, the proximal superficial temporal artery should be compressed for one minute to exclude the remote possibility of a critical collateral to cerebral circulation provided by the external carotid.

  • Saddle nose

    • The differential for saddle nose include congenital syphilis, Wegner's granulomatosis, relapsing polychondritis, lethal midline granuloma, and intranasal cocaine abuse.

  • Mouth

    • An oral examination may be important in the neuro-ophthalmic exam.

      • A palatal eschar should be excluded in any patient with suspected mucormycosis.

      • Patients with vertical pendular nystagmus or in some cases torsional nystagmus, may show palatal undulations indicative of oculopalatal myoclonus.

      • Oral ulcers or tongue ulcers are occasionally seen in patient with giant cell arteritis and jaw claudication.

      • Tongue atrophy may be seen with Mobius syndrome. The relevance of the oral exam in patients with seventh nerve palsy is discussed above.

  • Neurological exam

    • Although a full neurological exam is useful in neuro-ophthalmic workup, it is usually not feasible in the ophthalmologist's office, and better deferred to our neurology colleagues.

    • Notwithstanding, the patient's gait, speech and mental status can be ascertained during the interview.

      • In patients with homonymous hemianopsia, or retinal emboli, the presence or absence of hemiparesis should be noted.

      • Neurological exam may help localize the various fascicular syndromes of third and sixth cranial nerve palsies. For example third nerve palsy with a contralateral tremor may indicate involvement of the red nucleus and Benedikt's syndrome.
INCREASING EXAM EFFICIENCY, THE BEDSIDE EXAM & FUNCTIONAL VISUAL LOSS Section 9 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Increasing Efficiency in the Neuro-Ophthalmic Exam:

  • It is important to note the best-corrected visual acuity. Having a paddle occluder with attached pinhole may save much time. Autorefractors are usually quite accurate and will help the clinician to quickly obtain the patient's optimal refraction.

  • Time can be saved if the distance acuity, near acuity, color vision, Amsler grid and confrontation field are checked for one eye before switching to the other eye.

    • The author pastes a near card and Amsler grid into an abridged version of the Ishihara color plates. Near vision checks are quicker if a "Post-It" note is used to direct the patient to the lower portions of the near card.

  • Following the above, alternate cover testing at distance and near can be performed if ocular misalignment is suspected.

    • Versions are documented, Hirschberg testing, and pupil testing are then performed. The pupils are tested at this time so that the bright lights will not obscure the visual function needed for proper performance of the prior tests.

  • Much time, effort and money may be saved if old photographs are reviewed to determine the chronicity of neuro-ophthalmic signs such as abnormal head posture, anisocoria, strabismus and lid retraction.

    • At the time the appointment is made, it may be useful for the receptionist to ask the patient to bring in old photos. If these are not available, identification cards such as a driver's license may be of help.

    • Small photos can be better viewed with the indirect condensing lens, the direct ophthalmoscope or the slit lamp.

  • A well-trained ophthalmic assistant will do much to increase office efficiency. However, it is the author's bias that technicians working in the neuro-ophthalmology clinic not administer any dilating drops until the physician has reviewed the visual acuity, and examined the pupils, lids and motility. Even dilation with phenylephrine may obscure small amounts of ptosis.

Bedside Exam:

  • Eye exams at the bedside are often more challenging than office visits for a variety of reasons.

    • Hospitalized patients tend to be sicker, less reliable historians, and may be uncooperative or even unconscious during the eye exam.

    • Lighting conditions may hamper examination of the pupils.

    • Customary examining equipment may not be available. If neurological vital signs are being followed, pharmacological dilation of the pupils, and thorough fundus exam may not be possible.

  • When checking the near acuity make the patient sit up whenever possible, so that they can take advantage of the near segment of their bifocal and overhead lighting.

    • A pair of reading glasses should be kept in the examining kit for presbyopic patients. In conscious patients who are intubated, vision can be checked by asking the patient to imitate fingers at increasing distances.

  • Examination of the anterior segment may be difficult if a portable slit lamp is not available. Shining a penlight through the 20 D or 28 D indirect ophthalmoscopy condensing lens is a useful maneuver. Media opacities can be screened for by examing the red reflex through the direct ophthalmoscope.

  • Motility may change dramatically in patients who are not fully awake or sedated with medications. The 3-step test does not work well in patients who are supine.

  • In all unconscious patients exclude corneal exposure.

  • It is useful to carry an optokinetic tape in the examination kit. If one is not available, removing alternate strips from a long row of brightly colored sticker labels (e.g., allergy stickers) will work well.

Functional Visual Loss:

  • The neuro-ophthalmic exam of patients with suspected functional visual loss merits a chapter in itself. There are numerous techniques for detecting suspected functional visual loss, and each clinician should have a repertoire of these. Interested readers are referred to the excellent chapter by Miller and Keane in Walsh and Hoyts Clinical Neuro-ophthalmology (5th edition, Volume 1, Chapter 37, page 1765).

  • In general, functional patients claiming monocular visual loss are easier to diagnose than those claiming binocular visual loss. Likewise, functional patients claiming profound visual loss are easier to identify than patients indicating moderate visual loss.

    • In patients claiming bilateral visual loss, observation of navigational skills, pupil reflexes, and prism-diplopia responses may be helpful.

    • In patients claiming profound visual loss in one or both eyes, mirror testing and optokinetic testing can be performed. The presence of optokinetic nystagmus suggests there is at least 20/400 vision.

  • The absence of a relative afferent pupillary defect in patients with monocular visual loss and a normal fundus exam should be viewed with great suspicion.

    • A diplopia response to 4 prism diopter base out testing over the affected eye may provide further objective evidence of functional visual loss.

    • When performing the A-O vectograph, cylinder fogging tests, duochrome tests, stereo acuity tests, and binocular visual field testing for suspected functional visual loss, ensure that the patient is not closing the affected eye.

  • In patients with functional field loss, confrontation fields, tangent screen testing and Goldmann perimetry correlated with Amsler grid findings are often superior to automated perimetry.

    • In patients with visual field constriction, tangent screen testing at increasing distances (with proportionate increase in target size) is classically performed.

    • If a tangent screen in unavailable, performing confrontation testing at increasing distances will also provide useful information if the patient is "tunnelling".

  • Patients with suspected functional visual loss often benefit from a repeat exam. On the second exam observe the patient's behaviour as they enter and leave the office, and in the waiting room. The physician should recheck acuity by "walking" the patient up from the 20/10 line.

    • Correlation of the distance and near acuities should be repeated. When testing near vision ensure that the proper refraction and card-test distances are employed.

    • Re-examine the pupillary responses carefully, and on completion of this consider redilating the patient with possible cycloplegic refraction.

  • It is not uncommon for organic pathology to accompany functional disease, and the clinician should be wary of this association.

    • If patients with functional visual loss do not improve, the author usually performs neuroimaging and in select cases initiates ERG testing.

    • When possible review the CTs or MRIs with careful attention to the orbital apex and occipital lobe.
PICTURES Section 10 of 11   Click here to go to the next section in this topic Click here to go to the top of this page Click here to go to the next section in this topic

Caption: Picture 1. A paddle occluder with attached pinhole facilitates determination of the visual acuity.
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Caption: Picture 2. Confrontation visual fields are tested monocularly with the patient fixating on the examiner's eye or nose. The patient can be asked to add up the number of fingers simultaneously presented on either side of the midline.
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Caption: Picture 3. Time can be saved if the near card and Amsler grid are pasted in to the color vision test book. A "Post-It®" note can be used to direct the patient to the appropriate section of the near card.
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BIBLIOGRAPHY Section 11 of 11   Click here to go to the next section in this topic Click here to go to the top of this page

NOTE:
Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors, editors, and publisher of this textbook have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of this text do not warrant the information in this text is accurate or complete, nor are they responsible for omissions or errors in the text or for the results of using this information. The reader should confirm the information in this text from other sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert. FULL DISCLAIMER
Topic last updated May 18, 2000.

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