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Neuro-Ophthalmic History

  • Author: Edsel Ing, MD, FRCSC; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Mar 16, 2016
 

Overview

Ophthalmology is a specialty in which diagnoses are most often made based on observational skills and visual cues. In contrast, neuro-ophthalmic diagnosis usually requires an intensive, detailed history correlated with the physical examination. Findings on physical examination often direct further questions. While conversing with the patient, perform a gestalt examination and note the patient's gait, visage, eyes, ocular adnexa, hands, clothing, and mannerisms. After completing the history and physical examination, the neuro-ophthalmic diagnosis is often apparent.

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Technique

Identifying data and chief complaint

Identifying data

Document the patient's age, sex, race, and occupation in the first sentence of the chief complaint.

Although office staff may have already documented the patient's age on the chart, the authors routinely ask older patients, "How young are you?" This polite question quickly ascertains the patient's facility with English, memory, and ability to abstract, which can help predict the reliability of the history.

In neurologic histories, the patient's hand dominance may be included in the first sentence of the identifying data. Hand dominance may be important in some patients with stroke affecting the visual pathway.

Chief complaint

When possible, document the chief complaint in the patient's own words. Patients may misuse medical jargon and should be encouraged to use simple terminology at the start of the interview. It is not uncommon that the patient's conception of the neuro-ophthalmic problem differs markedly from the referring physician's diagnosis.

Note the duration of the patient's symptoms and the lateralization or binocularity of visual deficits.

In patients who present with multiple seemingly unrelated complaints, it may be necessary to redirect the interview by asking the following questions: (1) If I could solve one problem for you today, what would it be? (2) Tell me about your problem in one or two sentences.

If a third-party payer audits the notes from the patient consultation, it may be useful to preface the chief complaint with statements such as, "Patient referred for evaluation and opinion of symptoms of _____."

History of present illness

Document the onset, quality, severity, lateralization, temporal characteristics, and associated features of the patient's visual symptoms. If the patient is unreliable, family members should be solicited. The medication list, prior neuroimaging reports, and laboratory results help guide inquiry into the history of the present illness. In patients who seem cognitively intact but do not or cannot provide details of their visual problem, suspect functional visual loss. When questioned, patients with functional visual loss often repeatedly state, "I don't know."

Unexplained visual loss is one of the most common reasons for neuro-ophthalmic consultation.

Vital diagnostic elements

Lateralization, location, duration, tempo, and presence or absence of pain

Determine if the visual loss is monocular or binocular by asking the patient what happens when either eye is occluded. Patients frequently mistake homonymous visual loss as a deficit confined to the eye that is ipsilateral to the field loss.

If the visual loss respects the horizontal midline, a monocular visual loss is more likely. If the visual loss respects the vertical midline, exclude a homonymous or heteronymous defect.

Document if the perceived vision loss is at distance fixation, near fixation, or both. Patients often present to the neuro-ophthalmologist's office with complaints of presbyopia.

Degree of recovery

Following vision loss, the extent and timing of visual recovery can provide valuable diagnostic clues. Transient visual obscurations that last for seconds may result from increased intracranial pressure. "Dry eyes" can also cause transient visual phenomena that improves with blinking.

Painless unilateral visual loss that lasts several minutes may be embolic (amaurosis fugax), especially if it respects the horizontal midline. Gaze-evoked amaurosis may result from a mass lesion at the orbital apex.

Transient visual loss with scintillations, fortification spectra, and movement that lasts 20-30 minutes and then completely resolves suggests migraine. Migraine is a diagnosis of exclusion, but it is the most common cause of transient visual loss in young patients. Transient visual obscurations last only seconds. Amaurosis fugax usually lasts seconds to minutes at the most.

Sudden-onset visual loss with little recovery suggests an ischemic event. Subacute visual loss with almost complete recovery of acuity after several weeks or months is characteristic of optic neuritis. Slowly progressive visual loss may be due to a compressive optic neuropathy. Painful visual loss may indicate optic neuritis. Visual loss with headache, scalp tenderness, or jaw claudication in an elderly patient may be due to giant cell arteritis.

Consider mucormycosis in patients with new-onset visual loss and a history of diabetic ketoacidosis (without vitreous hemorrhage) or immunocompromise.

Reading-related visual loss in older patients

When older patients complain of visual loss when reading, presbyopia is not always the cause. Determine if the patient has difficulty seeing whole words, finding the next line (possible left homonymous field loss), or reading the next word (possible right homonymous field loss).

Diplopia

Diplopia is a common neuro-ophthalmic complaint. Document the following features: monocular or binocular nature, relative orientation of the separated images, frequency or variability of the diplopia, activities that enhance the diplopia, and associated ptosis or lid retraction.

Double vision that persists despite closing either eye but improves with pinhole is characteristic of monocular diplopia caused by conditions such as "dry eye," uncorrected refractive error, optical aberrations of the ocular media, or on occasion, retinal disorders that distort the fovea.

Monocular diplopia that does not resolve despite pinhole may be due to palinopsia (perseverance of visual images) from an evolving cerebral lesion, often in the nondominant hemisphere.

Binocular diplopia with vertical or diagonal separation of objects, worse when the patient is reading or climbing stairs and better with head tilt to one side, suggests trochlear nerve palsy (cranial nerve IV).

Binocular diplopia with horizontal separation that is worse in the distance suggests cranial nerve (CN) VI palsy.

Patients who develop complete CN III palsy may initially complain of diplopia. If the ptosis becomes severe enough to block the visual axis, the diplopia may resolve.

Variability or worsening of the diplopia as the day progresses with associated ptosis suggests myasthenia gravis.

Table 1 at the end of this section describes relatively uncommon visual complaints that are of neuro-ophthalmic interest.

Periocular pain and headache

When patients present to the neuro-ophthalmologist, they frequently complain of periocular pain or headache.

Always consider giant cell arteritis in older patients who present with headache, scalp tenderness, or occipital tenderness.

Burning discomfort in a unilateral segment of the trigeminal nerve distribution occasionally precedes the appearance of herpetic vesicles; at times, early herpes zoster can be mistaken for temporal arteritis.

Migraine headaches, which often consist of several hours of throbbing hemicranial and retro-orbital discomfort, are common. Migraine may be accompanied by visual scintillations and fortification spectra, which resolve over 15-30 minutes. A family history of migraine is common, and trigger factors, such as certain foods, may be noted.

Cluster headache is characterized by early morning awakening with unilateral periocular pain, accompanied by lacrimation and ipsilateral Horner syndrome.

Patients with an increased probability of having abnormal findings on neuroimaging studies may indicate a history of having the worst headaches of their life with meningismus, awakening with headaches, numbness or tingling, dizziness or lack of coordination, or a rapid increase in the frequency of headaches.

Eye pain has several differential diagnoses. The number of patients with keratitis sicca who are referred for neuro-ophthalmic opinion is surprising. Dry eye is the likely diagnosis in patients who complain of sandy eyes or sharp eye pain that is worse in the evening than at other times. Corneal abrasions, iritis, and angle-closure glaucoma are best differentiated on slit-lamp examination, but clues from the patient's history may also help.

Patients with corneal abrasion usually report trauma, contact lens wear, or prior recurrent corneal erosions. Patients with iritis usually complain of intense photophobia, and they may have an underlying autoimmune disease. In rare cases, patients with persistent photophobia unexplained by ocular abnormalities may have a chiasmal tumor.[1] Angle-closure glaucoma may be misdiagnosed as an intracranial process (eg, aneurysm) because of the prominent headache, nausea and vomiting, and middilated pupil.

Medications and allergies

Medications

Systemic or topical steroids may predispose patients to glaucomatous cupping of the optic nerve.

Tetracycline, vitamin A, and other medications have been linked to "idiopathic" intracranial hypertension or pseudotumor cerebri. The antituberculosis agents ethambutol, rifampin, and isoniazid can cause a toxic optic neuropathy. Amiodarone-induced optic neuropathy has been reported. Chronic hydroxychloroquine use may result in maculopathy. Procainamide, penicillamine, and other drugs have been implicated in drug-induced myasthenia gravis. Digoxin, even at therapeutic levels, may cause visual disturbances, typically xanthopsia.

Phosphodiesterase type 5 medications for erectile dysfunction (ie, sildenafil, tadalafil, vardenafil) may cause blue-tinged vision, and these medications have been implicated in cases of nonarteritic ischemic optic neuropathy. At least one report exists of oculomotor nerve palsy 36 hours following ingestion.[2]

Agents implicated in exacerbation of drug-induced myasthenia gravis include d-penicillamine, the -mycin antibiotics (especially telithromycin), botulinum toxin, vecuronium, interferon, systemic fluoroquinolones and tetracyclines, procainamide, quinidine, statins (rare), and beta-blocker drops for glaucoma.

Long-term use of the antiepileptic vigabatrin has been associated with peripheral field loss.[3] Topiramate is used in patients with seizures and in some patients with headaches. Topiramate can cause acute myopia and bilateral angle-closure glaucoma.[4] Desferrioxamine, an iron-chelating agent, increases the risk of mucormycosis.

Fingolimod (Gilenya) has been used for demyelinating disease and has been associated with a low incidence of macular edema.[17]

Maternal use of Dilantin or alcohol during pregnancy may result in a predisposition to optic nerve hypoplasia.

Patients often do not mention that they are taking aspirin. Over-the-counter medications, such as vitamin E, ginseng, and ginkgo biloba, may promote bleeding. Consider this point if surgery of the optic nerve or orbit or for strabismus is planned.[5, 6]

Allergies

Allergy to sulfonamide antibiotic may not preclude the use of the nonantibiotic acetazolamide (Diamox) in patients with idiopathic intracranial hypertension (pseudotumor cerebri).[13, 14]

Contrary to popular belief, shellfish allergy is not a contraindication to iodinated contrast CT scan.

Medical history

For patients who cannot remember their medical history, the following items may help jog their memory: past surgery, hospital admissions, previous medications, reasons for seeing a physician, results of past neuroimaging studies or blood tests, and trauma (eg, motor vehicle accidents). Whenever feasible, personally review the patient's CT scans or MRIs.

A history of cancer must be carefully excluded in all patients. The author of this article has encountered women with new-onset orbital disease who denied their past history of breast cancer. Patients may not mention a history of lymphoma because they do not believe that lymphoma is related to cancer. Patients with a remote history of cutaneous melanoma may overlook this fact. When neoplasia is suspected, refresh the patient's memory by specifically asking about past biopsy procedures and courses of chemotherapy or radiation therapy.

Functional inquiry

Amaurosis fugax with contralateral extremity weakness may indicate ipsilateral internal carotid artery disease.

Ambulatory difficulties, bowel or bladder dysfunction, paresthesias, and weakness may accompany optic neuritis in patients with multiple sclerosis.[7, 8]

Headache may be associated with neuro-ophthalmic problems, such as increased intracranial pressure, giant cell arteritis, migraine, and other neuro-ophthalmic entities. Headaches associated with increased intracranial-pressure are often associated with nausea and vomiting and usually worse in the morning, in the supine position, or during a Valsalva maneuver than in other conditions.

Ophthalmologists must maintain a high index of suspicion for giant cell arteritis in all elderly patients. In addition to headache, scalp tenderness, vision loss, or diplopia, inquire about symptoms of polymyalgia rheumatica, jaw claudication (pain with mastication that resolves with rest but returns after a period of chewing), chills or fevers, weight loss, and malaise. Tongue claudication is a variant of jaw claudication. Erythrocyte sedimentation rate (ESR) results may be normal in about 10% of patients. Determine if the ESR was determined by means of a Wintrobe test or a Westergren test because results are lower with the former than with the latter.

Diabetes mellitus, hypertension, and hypercholesterolemia are common diseases in patients with neuro-ophthalmic problems. Be aware that many patients do not consider that they have diabetes if their condition is controlled with diet or oral hypoglycemics. Likewise, some patients with hypertension consider themselves cured, denying that they have high blood pressure because they take antihypertensives.

Dysthyroidism is a common disorder that can cause neuro-ophthalmic problems, such as proptosis, diplopia, or vision loss. Subtle cases of thyroid-related ophthalmopathy and confirmatory symptoms of systemic dysthyroidism may be overlooked unless patients are carefully examined for lid retraction.

Atrial fibrillation increases the risk of embolic disease. Edrophonium (Tensilon) is not available in all countries, but careful cardiopulmonary inquiry should be performed before considering edrophonium testing.[15] Pacemakers preclude the use of MRI.

In all patients with ocular myasthenia, inquire about the following symptoms that may have systemic generalization: dyspnea, dysphagia, and difficulty getting out of chairs or climbing stairs (proximal weakness).

Rashes may accompany numerous conditions, such as syphilis, Lyme disease, sarcoidosis, and collagen-vascular disease. Results of previous skin biopsy may suggest basal cell carcinoma, squamous cell carcinoma, and melanoma. If phakomatosis is suspected in a child, querying the mother about skin lesions is often helpful.[9, 10]

Determining a history of kidney disease is important. Hypertensive nephropathy is not uncommon, and hypertensive or diabetic nephropathy may be a relative contraindication for contrast CT scanning or MRI with gadolinium (nephrogenic systemic fibrosis).

Collagen-vascular diseases may be associated with nephritis or hematuria.

Pseudotumor cerebri and kidney stones are relative contraindications to acetazolamide.

Obstructive sleep apnea is an increasingly recognized condition. Elevated body mass index, daytime somnolence, and loud snoring are possible clues in addition to patient physiognomy.

Rheumatologic and collagen-vascular disease, which may be associated with joint disease, can cause vasculitis that affects the visual pathway. Lupus has far-ranging systemic manifestations.

Shoulder pain that is worse in the morning than at other times, especially when the patient reaches for top cupboards or puts on pullover clothing, may suggest polymyalgia rheumatica. Polymyalgia rheumatica is associated with giant cell arteritis.

Chiropractic manipulation has been associated with vertebral artery dissections or carotid artery dissections.

Acquired hearing loss may be important in various neuro-ophthalmic conditions (eg, acoustic schwannoma, Harada disease, retinocochlear cerebral vasculitis).

A history of psychiatric problems may increase the index of suspicion for functional visual loss. However, the clinician must be wary to exclude an accompanying component of organic disease in patients with such findings. Claustrophobic patients may require sedation or benefit from open-field MRI.

Family history and social history

Family history

The patients' family history helps delineate optic neuropathies.

In patients with optic disc hypoplasia, a history of maternal diabetes mellitus or maternal drug use (eg, phenytoin [Dilantin]) may be helpful.

A family history of glaucoma is helpful in patients with cupping of the optic nerve that is not disproportionate to pallor. Likewise, a family history of multiple sclerosis is a risk factor for optic neuritis.

Patients with dominant optic atrophy may have a supportive family history. Blindness in a patient's brother or maternal uncles may indicate Leber hereditary optic neuropathy. Asymptomatic inferior field defects and the appearance of superior segmental dysplasia is associated with maternal diabetes.

Many of the phakomatoses (with the exception of Sturge-Weber syndrome and ataxia telangiectasia) are autosomal dominant.

Social history

The social history is important, as it reveals the patient’s social situation, as well as details of the disease.

The patient's vocation may indicate his or her understanding of medical language and expectations. The patient's occupation may determine his or her work restrictions and ability to safely continue work, as well as the need for vocational rehabilitation. Visual dysfunction may have grave implications for patients who have jobs that involve commercial driving, working at heights, or discharging firearms.

The confabulation of alcoholism may thwart the validity of the history. A history of alcoholism raises the possibilities of nutritional amblyopia and Wernicke alcoholic ophthalmoplegia.

Patients with a military history may have increased risk for Treponema infection. Because military recruits usually undergo screening of their color vision, the current review is often a good opportunity to determine the chronicity or progression of dyschromatopsia.

Previous transfusions, the patient's sexual habits and sexual orientation, and the use of intravenous recreational drugs may raise the possibility of HIV-related disease.

Table 1. Relatively Uncommon Visual Complaints of Neuro-Ophthalmic Interest (Open Table in a new window)

Complaint Description
Pulfrich phenomenon Objects moving in a straight-line path take on a curved trajectory because of asymmetric conduction of the optic nerve. This is most often described with optic neuritis.
Constant photopsias In the absence of retinal tears, exclude paraneoplastic retinopathy.*
Episodic tilting Exclude superior oblique myokymia.
90-180° illusory tilt Wallenberg syndrome
Palinopsia Patient may complain of diplopia due to visual perseveration. Palinopsia does not resolve with pinhole. Multiple etiologies have been identified and include medication use. Evolving parietal, occipital, or temporal lobe lesions should be excluded.
Prosopagnosia Inability to recognize faces due to bilateral inferior occipital infarct, often accompanied by cerebral dyschromatopsia.
Hemifield slide (hemiretinal slip) Patient may complain of double vision. The visual field is split with horizontal or vertical separation. The patient has a complete bitemporal hemianopsia, usually due to a chiasmal lesion and an underlying phoria.
Postfixational blindness When the patient fixates on an object, everything behind it disappears. It usually is due to chiasmal tumor with complete bitemporal hemianopsia.
*Many neoplasms have been associated with paraneoplastic retinopathy. Carcinoma of the lung is a common cause of cancer-associated retinopathy. The retinal arterioles are typically narrowed, the field is constricted, and the electroretinogram is depressed. The cancer-associated retinopathy autoantibody test is commercially available (from Athena Diagnostics, phone 1-800-394-4493).
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Pearls

When booking appointments, the scheduler should remind patients to bring their medication list, medical history, CT scans and/or MRIs, and old photographs, if applicable. When appropriate, organize the charts 1 day before the patient arrives; this ensures that the referring physician has faxed or sent the notes, fields, and results of past neuroimaging studies.[11]

Have patients complete a screening inventory of questions online or in the waiting room. A standardized history form may save time and help organize dictations. Some physicians may find an inquiry checklist helpful in the workup of common neuro-ophthalmic problems; for an example, see the Sample Neuro-Ophthalmologic Functional Inquiry Checklist in the next section. To save writing, this checklist organized items so that positive findings can be circled and negative findings crossed out. A question inventory may help the chart documentation comply with the appropriate coding levels for Medicare audits.

In some practices, the patient's history is obtained by residents or other healthcare professionals. Unfortunately, ophthalmic technicians and novice trainees may miss the significance of certain comments from the patient. Although standardized history forms may be useful in a neuro-ophthalmology practice, the senior physician should personally review the neuro-ophthalmic history with the patient. If the physician does not specifically hear the chief complaint, a patient might leave the office without the primary concern being addressed. The patient's compliant or history often changes when questioned a second time.

Patients often seek second or third opinions from neuro-ophthalmologists. In such cases, telephone consultation with the patient's previous caregiver may be invaluable. Although knowledge of the previous clinician's working diagnoses may bias clinical judgment, it may prevent unnecessary repetition of tests. The second clinician can also avoid repeating the conditions of the initial examiner that led to the patient's dissatisfaction. It may be helpful to review the patient's prior imaging with another neuroradiologist.

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Neuro-Ophthalmologic Functional Inquiry Checklist

Table 2. Sample Neuro-Ophthalmologic Functional Inquiry Checklist (Open Table in a new window)

Area of Inquiry Findings and Answer Choices Circle if positive, cross out if negative
Acuity and field loss Eye(s): OD, OS, or OU



Did not occlude 1 eye: Yes or no



Number of previous episodes: _______



Recovery: complete, partial, or none



Date when glasses were changed: _____; near or distance



Features: sudden or gradual, painless or painful, central or peripheral, stable or progressive



Respect: horizontal midline or vertical midline



Other findings: positive scotoma, fortification spectra



Diplopia Lateralization: monocular or binocular



Number of previous episodes: _____



Features: constant or intermittent; near, reading, or distance



Gaze: right or left, up or down



Separation: vertical, horizontal, diagonal, tilting, with twitch or without twitch



Numbness: CN V1 or cornea



Other findings: variability, ptosis, dyspnea, dysphagia, proximal weakness



Strabismus Eye(s): OD, OS, or alternating



Age of onset: ________



Nature: eso, exo, hyper, or hypo



Duration: constant or intermittent



Family history: siblings, children, or parents



Change: increasing in frequency, increasing in duration



Abnormal head posture: yes or no



History: previous glasses, patching, strabismus, amblyopia



Eye pain Eye(s): OD, OS, or OU



Number of previous episodes: _____



Posttraumatic: yes or no



Onset: sudden or gradual



Duration: constant or intermittent



Time of day: AM or PM



Nature: itchy, sandy, sharp, dull, pressure



Exacerbating factors: reading, driving, wind



Other findings: trichiasis, Bell palsy



Red eye Eye(s): OD, OS, or OU



Duration: _____



Discharge: clear, pus



Contact lens wear: yes or no



History: metal on metal, eye trauma, corneal erosion



Other findings: sty, glaucoma, bruit Other factors: seasonal nature, allergy, exposure to pets, sexually transmitted disease (STD)



Headache Duration: _____



Awakens patient in the morning: yes or no



Symptoms: nausea and vomiting, seizure; worsens with Valsalva maneuver; migraine or equivalents



Location: frontal, cervical; right, left, or bilateral



Nature: sharp, dull, throbbing, pressure Radiation: _____



Giant cell arteritis Duration: _____



Symptoms: scalp tenderness; sore shoulders and/or hips, morning pain, jaw claudication, weight loss, fever



ESR determined by Westergren method: yes or no; date: _____



C-reactive protein (CRP) test: yes or no; date: _____



Idiopathic intracranial hypertension (pseudotumor cerebri) Height: _____



Weight _____; gain, loss, or same



Transient visual obscurations: yes or no, with or without Valsalva maneuver, pulsatile tinnitus



Lumbar puncture (LP): pending or not pending; opening pressure _____ mm water; _____ cells



Medications: vitamin A, tetracycline, lithium, danazol



Other findings: liver, sleep apnea, lupus, otitis media



Optic neuritis and/or multiple sclerosis Eye(s): OD, OS, or OU Duration: _____



Symptoms: pain on eye movement, color desaturation



White matter lesions on MRI: yes or no



Mediations: intravenous or oral steroids, interferon



Preceding viral infection: yes or no



Family history: blind maternal uncles or brothers



Other findings: Uhthoff, Lhermitte, gait, motor, sensory, bowel, bladder



Anisocoria Eye: OD or OS



Abnormality: large or small



Duration: _____; constant or intermittent



Old photos: yes or no



Symptoms: ptosis, diplopia, facial pain, heterochromia



Other factors: eye drops, motion sickness, drug abuse, paramedical, exposure to pets or their flea collars, migraine, trauma, metal foreign body, iritis, previous intraocular surgery



Ptosis Eye(s): OD, OS, or OU



Duration: _____



Old photos: yes or no



Symptoms or findings: progression, pupils, facial pain, diplopia, variability



History: dysthyroidism, trauma, previous surgery, contact lens wear



Proptosis Eye(s): OD, OS, or OU



Duration: _____ Thyroid: hyperthyroid, euthyroid, hypothyroid, unknown



History: cancer, lymphoma, rhabdomyosarcoma, sinusitis



Puffy lids: AM or all day; OD, OS, or OU



Stare (lid retraction): OD, OS, or OU



Other factors: smoking, exposure to iodine-131 past or future, steroid use, external beam irradiation



Note.—OD = oculus dexter (right eye), OS = oculus sinister (left eye), OU = oculus uterque (both eyes).
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Contributor Information and Disclosures
Author

Edsel Ing, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Hospital for Sick Children and Sunnybrook Hospital

Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Royal College of Physicians and Surgeons of Canada, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, Canadian Society of Oculoplastic Surgery, European Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Medical Association, Ontario Medical Association, Statistical Society of Canada, Chinese Canadian Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Sabrina Ing, MD Consulting Staff, Department of Family Practice, Private Practice

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Andrew W Lawton, MD Neuro-Ophthalmology, Ochsner Health Services

Andrew W Lawton, MD is a member of the following medical societies: American Academy of Ophthalmology, Arkansas Medical Society, Southern Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

References
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  2. Donahue SP, Taylor RJ. Pupil-sparing third nerve palsy associated with sildenafil citrate (Viagra). Am J Ophthalmol. 1998 Sep. 126(3):476-7. [Medline].

  3. Krauss GL, Johnson MA, Miller NR. Vigabatrin-associated retinal cone system dysfunction: electroretinogram and ophthalmologic findings. Neurology. 1998 Mar. 50(3):614-8. [Medline].

  4. Sankar PS, Pasquale LR, Grosskreutz CL. Uveal effusion and secondary angle-closure glaucoma associated with topiramate use. Arch Ophthalmol. 2001 Aug. 119(8):1210-1. [Medline].

  5. Shen CC, Silver AL, O'Donnell TJ, Fleming JC, Karcioglu ZA. Optic neuropathy caused by naso-orbital mass in chronic intranasal cocaine abuse. J Neuroophthalmol. 2009 Mar. 29(1):50-3. [Medline].

  6. Donati RJ, Maino DM, Bartell H, Kieffer M. Polypharmacy and the lack of oculo-visual complaints from those with mental illness and dual diagnosis. Optometry. 2009 May. 80(5):249-54. [Medline].

  7. Plant GT. Optic neuritis and multiple sclerosis. Curr Opin Neurol. 2008 Feb. 21(1):16-21. [Medline].

  8. Mowry EM, Loguidice MJ, Daniels AB, et al. Vision related quality of life in multiple sclerosis: correlation with new measures of low and high contrast letter acuity. J Neurol Neurosurg Psychiatry. 2009 Jul. 80(7):767-72. [Medline].

  9. Bernardini FP, Croxatto JO, Orcioni GF, Bianchi S. Visual loss secondary to orbital apex invasion as the first manifestation of recurrent nasopharyngeal carcinoma. Ophthal Plast Reconstr Surg. 2009 May-Jun. 25(3):248-50. [Medline].

  10. Bezo C, Majzoub S, Nochez Y, Leruez S, Charlin JF, Milea D, et al. [Ocular and neuro-ophthalmic manifestations of sarcoidosis: retrospective study of 30 cases]. J Fr Ophtalmol. 2013 Jun. 36(6):473-80. [Medline].

  11. McClelland C, Van Stavern GP, Shepherd JB, Gordon M, Huecker J. Neuroimaging in patients referred to a neuro-ophthalmology service: the rates of appropriateness and concordance in interpretation. Ophthalmology. 2012 Aug. 119(8):1701-4. [Medline]. [Full Text].

  12. Ing EB, Ing TGE. Neuro-ophthalmology: pitfalls and pearls. Ophthalmic Practice. 1999. 17:146-50.

  13. Platt D, Griggs RC. Use of acetazolamide in sulfonamide-allergic patients with neurologic channelopathies. Arch Neurol. 2012 Apr. 69 (4):527-9. [Medline].

  14. Lee AG, Anderson R, Kardon RH, Wall M. Presumed "sulfa allergy" in patients with intracranial hypertension treated with acetazolamide or furosemide: cross-reactivity, myth or reality?. Am J Ophthalmol. 2004 Jul. 138 (1):114-8. [Medline].

  15. Ing EB, Ing SY, Ing T, Ramocki JA. The complication rate of edrophonium testing for suspected myasthenia gravis. Can J Ophthalmol. 2000 Apr. 35 (3):141-4; discussion 145. [Medline].

  16. Zarbin MA, Jampol LM, Jager RD, Reder AT, Francis G, Collins W, et al. Ophthalmic evaluations in clinical studies of fingolimod (FTY720) in multiple sclerosis. Ophthalmology. 2013 Jul. 120 (7):1432-9. [Medline].

 
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Table 1. Relatively Uncommon Visual Complaints of Neuro-Ophthalmic Interest
Complaint Description
Pulfrich phenomenon Objects moving in a straight-line path take on a curved trajectory because of asymmetric conduction of the optic nerve. This is most often described with optic neuritis.
Constant photopsias In the absence of retinal tears, exclude paraneoplastic retinopathy.*
Episodic tilting Exclude superior oblique myokymia.
90-180° illusory tilt Wallenberg syndrome
Palinopsia Patient may complain of diplopia due to visual perseveration. Palinopsia does not resolve with pinhole. Multiple etiologies have been identified and include medication use. Evolving parietal, occipital, or temporal lobe lesions should be excluded.
Prosopagnosia Inability to recognize faces due to bilateral inferior occipital infarct, often accompanied by cerebral dyschromatopsia.
Hemifield slide (hemiretinal slip) Patient may complain of double vision. The visual field is split with horizontal or vertical separation. The patient has a complete bitemporal hemianopsia, usually due to a chiasmal lesion and an underlying phoria.
Postfixational blindness When the patient fixates on an object, everything behind it disappears. It usually is due to chiasmal tumor with complete bitemporal hemianopsia.
*Many neoplasms have been associated with paraneoplastic retinopathy. Carcinoma of the lung is a common cause of cancer-associated retinopathy. The retinal arterioles are typically narrowed, the field is constricted, and the electroretinogram is depressed. The cancer-associated retinopathy autoantibody test is commercially available (from Athena Diagnostics, phone 1-800-394-4493).
Table 2. Sample Neuro-Ophthalmologic Functional Inquiry Checklist
Area of Inquiry Findings and Answer Choices Circle if positive, cross out if negative
Acuity and field loss Eye(s): OD, OS, or OU



Did not occlude 1 eye: Yes or no



Number of previous episodes: _______



Recovery: complete, partial, or none



Date when glasses were changed: _____; near or distance



Features: sudden or gradual, painless or painful, central or peripheral, stable or progressive



Respect: horizontal midline or vertical midline



Other findings: positive scotoma, fortification spectra



Diplopia Lateralization: monocular or binocular



Number of previous episodes: _____



Features: constant or intermittent; near, reading, or distance



Gaze: right or left, up or down



Separation: vertical, horizontal, diagonal, tilting, with twitch or without twitch



Numbness: CN V1 or cornea



Other findings: variability, ptosis, dyspnea, dysphagia, proximal weakness



Strabismus Eye(s): OD, OS, or alternating



Age of onset: ________



Nature: eso, exo, hyper, or hypo



Duration: constant or intermittent



Family history: siblings, children, or parents



Change: increasing in frequency, increasing in duration



Abnormal head posture: yes or no



History: previous glasses, patching, strabismus, amblyopia



Eye pain Eye(s): OD, OS, or OU



Number of previous episodes: _____



Posttraumatic: yes or no



Onset: sudden or gradual



Duration: constant or intermittent



Time of day: AM or PM



Nature: itchy, sandy, sharp, dull, pressure



Exacerbating factors: reading, driving, wind



Other findings: trichiasis, Bell palsy



Red eye Eye(s): OD, OS, or OU



Duration: _____



Discharge: clear, pus



Contact lens wear: yes or no



History: metal on metal, eye trauma, corneal erosion



Other findings: sty, glaucoma, bruit Other factors: seasonal nature, allergy, exposure to pets, sexually transmitted disease (STD)



Headache Duration: _____



Awakens patient in the morning: yes or no



Symptoms: nausea and vomiting, seizure; worsens with Valsalva maneuver; migraine or equivalents



Location: frontal, cervical; right, left, or bilateral



Nature: sharp, dull, throbbing, pressure Radiation: _____



Giant cell arteritis Duration: _____



Symptoms: scalp tenderness; sore shoulders and/or hips, morning pain, jaw claudication, weight loss, fever



ESR determined by Westergren method: yes or no; date: _____



C-reactive protein (CRP) test: yes or no; date: _____



Idiopathic intracranial hypertension (pseudotumor cerebri) Height: _____



Weight _____; gain, loss, or same



Transient visual obscurations: yes or no, with or without Valsalva maneuver, pulsatile tinnitus



Lumbar puncture (LP): pending or not pending; opening pressure _____ mm water; _____ cells



Medications: vitamin A, tetracycline, lithium, danazol



Other findings: liver, sleep apnea, lupus, otitis media



Optic neuritis and/or multiple sclerosis Eye(s): OD, OS, or OU Duration: _____



Symptoms: pain on eye movement, color desaturation



White matter lesions on MRI: yes or no



Mediations: intravenous or oral steroids, interferon



Preceding viral infection: yes or no



Family history: blind maternal uncles or brothers



Other findings: Uhthoff, Lhermitte, gait, motor, sensory, bowel, bladder



Anisocoria Eye: OD or OS



Abnormality: large or small



Duration: _____; constant or intermittent



Old photos: yes or no



Symptoms: ptosis, diplopia, facial pain, heterochromia



Other factors: eye drops, motion sickness, drug abuse, paramedical, exposure to pets or their flea collars, migraine, trauma, metal foreign body, iritis, previous intraocular surgery



Ptosis Eye(s): OD, OS, or OU



Duration: _____



Old photos: yes or no



Symptoms or findings: progression, pupils, facial pain, diplopia, variability



History: dysthyroidism, trauma, previous surgery, contact lens wear



Proptosis Eye(s): OD, OS, or OU



Duration: _____ Thyroid: hyperthyroid, euthyroid, hypothyroid, unknown



History: cancer, lymphoma, rhabdomyosarcoma, sinusitis



Puffy lids: AM or all day; OD, OS, or OU



Stare (lid retraction): OD, OS, or OU



Other factors: smoking, exposure to iodine-131 past or future, steroid use, external beam irradiation



Note.—OD = oculus dexter (right eye), OS = oculus sinister (left eye), OU = oculus uterque (both eyes).
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