Neuro-Ophthalmic History

Ophthalmology is a specialty in which diagnoses most often are 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 often is apparent. ^{[1, 2, 3]}  If neuroimaging is performed, it should be corroborate the patient's history.

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 already may have recorded 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. ^[2]

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. ^{[1, 3]} 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, monocular vision 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. "Dry eyes" can cause transient visual phenomena that improves with blinking. Transient visual obscurations that last for seconds may result from increased intracranial pressure.

Painless unilateral visual loss that lasts several minutes may be embolic (amaurosis fugax), especially if it respects the horizontal midline. Gaze-evoked visual loss 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.

In patients who have undergone cataract surgery, dysphotopsia is not uncommon, but retinal pathology should be excluded with dilated fundus examination. Negative dysphotopsia usually manifests as shadows in the periphery. Positive dysphotopsia may appear as haloes or streaks of light.

Sudden-onset visual loss with little recovery suggests an ischemic event. Subacute painful 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. In elderly patients with newly onset vision loss or diplopia, especially with accompanying headache, scalp tenderness, or jaw/tongue claudication, giant cell arteritis should be excluded.

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, worsening with certain direction of gaze, 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 (CN IV) nerve palsy.

Binocular diplopia with horizontal separation that is worse in the distance may suggest abducens (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 go unnoticed.

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

Uncommonly, diplopia may be the presenting symptom of giant cell arteritis in older adults and must be differentiated from other, more common, benign etiologies. ^[4]

In patients with persistent complaints of binocular diplopia, not improved by prism, exclude an epiretinal membrane or other macular pathology. Compression or stretching of the macular photoreceptors may cause macropsia or micropsia. Patients with a "dragged fovea" may have worsening of their diplopia in the light compared to the dark.

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 and may be associated with nausea, vomiting, photophobia, or phonophobia. 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 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 compressive lesion of the optic chiasm that may resolve following treatment. ^[5]  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.

Certain medications (eg, oral contraceptives, steroids, vitamin A, isotretinoin, lithium, growth hormone (GH) replacement, nitrofurantoin, phenytoin, sulfa drugs, minocycline, tamoxifen, nalidixic acid, tetracycline, some chemotherapeutic drugs) have been linked to idiopathic intracranial hypertension or pseudotumor cerebri. Although many of these agents have been associated with Idiopathic intracranial hypertension (IIH), some of the associations may not be causal, and others have different mechanisms for producing increased intracranial pressure (eg, oral contraceptives and venous sinus thrombosis or steroid withdrawal versus steroid use). 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. ^[6]

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 was thought to cause peripheral field loss, but baseline field defects should be carefully measured. ^{[7, 8]} Topiramate is used in patients with seizures and in some patients with headaches. Topiramate can cause acute myopia and bilateral angle-closure glaucoma. ^[9] Desferrioxamine, an iron-chelating agent, increases the risk for mucormycosis.

Fingolimod (Gilenya) has been used for demyelinating disease and has been associated with a low incidence of macular edema. ^[10]

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. ^{[11, 12]}

Allergies

Most so-called allergic reactions to medications are not true IgE-mediated allergic reactions. Thus, an “allergy” to sulfonamide antibiotic may not preclude the use of the nonantibiotic sulfa moiety–containing agents such as acetazolamide (Diamox) in patients with idiopathic intracranial hypertension (pseudotumor cerebri). ^{[13, 14]}

In addition, contrary to popular belief, shellfish “allergy” is not a contraindication to iodinated contrast CT scan.

Past 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, weakness, heat intolerance, and back or appendicular "electric shocks" with neck flexion may accompany optic neuritis in patients with multiple sclerosis. ^{[15, 16, 17]}

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 often are 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. The erythrocyte sedimentation rate, C-reactive protein level, and platelet values may be normal in up to 8% of patients. ^[18]

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 for embolic disease. Edrophonium (Tensilon) is not available in all countries, but careful cardiopulmonary inquiry should be performed before considering edrophonium testing. ^[19] Some 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).

In patients with optic neuritis andntractable hiccoughs or vomiting, or narcolepsy, neuromyelitis optica spectrum disorder should be excluded.

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. ^{[20, 21]}

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.

In patients with idiopathic intracranial hypertension and kidney stones, the use of acetazolamide is a relative contraindication.

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. Eating disorders may contribute to Vitamin B1 or  Vitamin A deficiency. 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 (asymptomatic inferior visual field defects), 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.

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

Myotonic dystrophy and oculopharyngeal dystrophy are two examples of autosomal-dominant conditions that may present to the ophthalmologist as ptosis and ophthalmoplegia.

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)

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. ^[22]

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 complaint 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 also can 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.

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