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 Neuro-Ophthalmology

The Evaluation of Isolated Unexplained Optic Atrophy

Andrew G. Lee, MD

Introduction

The evaluation and management of isolated and unexplained optic atrophy is a relatively common, but difficult, clinical problem for a comprehensive ophthalmologist. Although, in most cases, the history and examination are sufficient to determine the etiology, some patients are left without a diagnosis at the end of the initial ophthalmology encounter. Multiple factors contribute to the clinical problem including: patients may present with physiologic pallor of the disc without other evidence for a true optic neuropathy; patients may be asymptomatic despite an optic neuropathy clinically; patients may report an acute onset of symptoms that is due to acute awareness of their visual loss; and the fundus signs of a previous event (i.e., a "footprint" of the etiology) may be subtle or nonspecific, especially when the patient is seen months or years after the acute episode.

The differential diagnosis of optic atrophy is large and testing for all possible etiologies (i.e., "shotgun" approach) is not appropriate, not feasible, not cost effective, and not necessary in most patients. This tutorial reviews a stepwise approach to the evaluation of isolated optic atrophy (Table 1).

Table 1: Ten Steps for Evaluating Optic Atrophy
(Modified from Lee A, et al. The evaluation of isolated optic atrophy. An update and review. Comprehensive Ophthalmology. 2004;5:297-304.)
  1. Ensure the optic atrophy is not physiologic pallor by establishing the clinical evidence for an optic neuropathy
  2. Exclude common etiologies first (i.e., optic neuritis and ischemic optic neuropathy)
  3. Obtain a complete history to establish an active or previous etiology for optic atrophy (e.g., past medical history of optic neuritis or multiple sclerosis, trauma history, vasculopathic risk factors and documentation of prior disc edema, family history of dominant optic atrophy, toxic-nutritional risk factors, inflammatory disorders such as sarcoidosis)
  4. Perform a complete ocular examination (including formal visual field testing) for findings that might suggest an etiology for the optic atrophy (e.g., uveitis, cecocentral scotomas with temporal pallor OU, hemianopic visual field loss).
  5. Review the records for neuroimaging suggesting a compressive etiology.
  6. If, following the complete history and examination, the patient has unexplained, isolated optic atrophy, proceed with cranial and orbital neuroimaging with contrast (i.e., magnetic resonance imaging [MRI] of head and orbit with fat suppression and gadolinium or a computed tomography [CT] scan if MRI is contraindicated).
  7. Consider further serologic testing as directed by history and examination. Screening evaluations are not recommended unless the pretest likelihood of disease is sufficient to warrant testing (e.g., syphilis, Lyme disease, sarcoid are endemic in the study population).
  8. Perform serial evaluations to verify the stability of the visual loss. If progressive optic neuropathy develops, then consideration for neuro-ophthalmic consultation, repeat neuroimaging, and additional testing are reasonable.
  9. Strongly consider personally reviewing the neuroimaging with a neuroradiologist in cases of unexplained optic atrophy even with a "normal" imaging report.
  10. Consider consultation with a neuro-ophthalmologist, especially in neurologically nonisolated or progressive cases.

Step 1: Ensure that the optic atrophy is real vs. physiologic

Physiologic pallor of the optic disc can occur in patients with media opacity or after lens extraction (aphakia or pseudophakia). This can be especially difficult in cases where one eye is pseudophakic and the other eye is phakic with a cataract because comparison between the two eyes can accentuate the apparent pallor of the disc. In addition, patients with significant peripapillary atrophy, optic nerve hypoplasia, an anomalous disc configuration, optic disc drusen, or a myopic fundus configuration may have difficulty judging true pallor of the disc. Patients with suspected optic atrophy should therefore undergo a complete ocular examination (including formal perimetry) to document the presence or absence of clinical evidence for a true optic neuropathy (e.g., loss of visual acuity or visual field, dyschromatopsia, and a relative afferent pupillary defect). The absence of a relative afferent pupillary defect (RAPD) in a strictly unilateral case is strong evidence against a true optic atrophy, but patients with recovered optic neuropathy (e.g., optic neuritis) or a bilateral and symmetric optic neuropathy (e.g. toxic-nutritional) may have a subtle or no RAPD. Measurement of the retinal nerve fiber layer with optical imaging (e.g., optical coherence tomography) may also prove reassuring when the nerve fiber layer is normal or may confirm the clinical suspicion of true optic atrophy when the nerve fiber layer is abnormal. Optic disc photos may be useful in establishing stability of the disc appearance over time.

Step 2: Exclude common etiologies first

Optic neuritis is the most common unilateral acquired optic neuropathy in young patients (< 40 years old) and anterior ischemic optic neuropathy is the most common acute optic neuropathy in older patients (> 40 years old). Patients should be specifically questioned regarding the temporal onset and course of their optic neuropathy to establish optic neuritis or anterior ischemic optic neuropathy as an etiology for optic atrophy before embarking on aggressive or extensive evaluations.

Step 3 and Step 4: Ensure that optic atrophy is neurologically isolated and look for clues to the etiology by performing a complete history and by performing a complete eye exam

An isolated and "unexplained optic atrophy" for the purposes of this tutorial includes patients with no proven etiology following the initial neuro-ophthalmic evaluation. Patients should be specifically questioned and examined for possible etiologies for optic atrophy (Table 2) including known prior optic neuropathy (e.g., ischemic, demyelinating, traumatic, congenital, hereditary, toxic-nutritional); known ocular or systemic disorder producing the optic atrophy (e.g., retinal detachment, glaucoma, compressive lesion or other prior etiology for optic neuropathy); other localizing neuro-ophthalmic symptoms or signs (e.g. proptosis, other cranial neuropathy, neurologic deficit); or neuro-ophthalmic history suggestive of an etiology for optic atrophy (e.g., prior intracranial tumor, syphilis, giant cell arteritis, toxin exposure, nutritional deficiency, family history of optic neuropathy).

Table 2: Important Historical Features in Patients with Optic Atrophy
  • Age of the patient (e.g., older than 50 years or younger than 40 years of age)
  • Pertinent family history (e.g., dominant optic atrophy, Leber's hereditary optic neuropathy, neurofibromatosis)
  • Past ocular and medical history (e.g., prior malignancy, known demyelinating, infectious or inflammatory disease, vasculopathic risk factors, giant cell arteritis, trauma, toxin or medicine exposure)
  • Social history (e.g., alcohol and tobacco use, occupation)
  • Temporal onset of the visual loss (e.g., acute, subacute, chronic onset).

The optic nerve has an intraocular, intraorbital, intracanalicular, and intracranial course. A complete eye examination should be performed on patients with optic atrophy (Table 3). Evidence for active or prior intraocular disease (e.g., uveitis, choroidal or retinal disease, retinovascular disorders, and surgery or trauma) may exist. Proptosis, lid abnormalities (e.g., lid retraction, ptosis), pupil findings (e.g., RAPD, anisocoria), or ophthalmoplegia may suggest an intraorbital (e.g., thyroid compressive optic neuropathy) or intracranial (e.g., superior orbital fissure, suprasellar) etiology. Optic atrophy in the setting of bitemporal (chiasm) or homonymous hemianopsia (optic tract) is strongly suggestive of anterior visual pathway (pre-geniculate) disease and is localizing. A complete neuro-ophthalmic history and examination should therefore be performed to exclude other topographically localizing findings in all patients with optic atrophy.

Step 5: The chart should be reviewed for previous testing and neuroimaging that might suggest an etiology for optic atrophy

The medical record ("chart biopsy") should be reviewed if possible for previous history (e.g., ethambutol use), laboratory testing, or neuroimaging (e.g., computed tomography or magnetic resonance imaging [MRI]) that may suggest an etiology for the optic atrophy (e.g., pituitary adenoma, meningioma). A patient may not recall or report systemic or intracranial disorders associated with optic atrophy to the ophthalmologist because the patient mistakenly believes that it is unrelated.

Step 6: Consider neuroimaging when optic atrophy remains unexplained after complete history and examination

Despite a detailed medical history, a thorough chart review, and complete ocular examination, a patient may remain with unexplained optic atrophy. Even the experts, however, can have difficultly in making the diagnosis in this setting. Trobe and colleagues gave five ophthalmologists 163 fundus stereophotographs of nine "unknowns" to review. Although glaucoma (i.e., cupping), central retinal artery occlusion, and ischemic optic neuropathy (ION) were diagnosed by at least one observer with accuracy greater than 80%, the remaining optic neuropathies (i.e., optic neuritis, compressive, traumatic, and hereditary optic neuropathies) were identified in fewer than 50% of cases. A sufficient fundus "footprint" to suggest prior disc swelling was present in only 11% of the cases that had documented papillitis or ischemic optic neuropathy.1

Thus, for the patients with unexplained optic atrophy, neuroimaging (preferably contrast head and orbit MRI with fat suppression views) should be considered. An alternative to neuroimaging may be considered in individual cases (e.g., financial reasons, patient's wishes, contraindication to imaging). If a specific etiology (e.g., ischemic optic neuropathy in a vasculopathic patient with static visual loss and optic atrophy of 6 months duration) is clinically suspected, then a presumptive clinical diagnosis can be made and the patient can be followed for progression. If no progressive visual loss and no new neurologic symptoms develop over time, then continued serial follow-up is an option. The author recommends that the patient be informed of the risks and benefits for this choice and that the rationale for not proceeding with neuroimaging be documented in the chart. However, clinical evidence must support the decision not to perform imaging. In one study of neuroimaging for optic atrophy, 20% of patients had an etiology for the optic atrophy on neuroimaging. Of the 18 patients with abnormal imaging studies (e.g., meningioma, pituitary adenoma, craniopharyngioma), 11 cases of optic atrophy were bilateral and seven were unilateral. Five of the 18 patients had progressive visual loss, three had temporal or homonymous hemianopic visual field loss, and 12 were younger than age 50.2

Step 7: Consider a directed evaluation

In a patient with a history or documentation of prior etiology (e.g., optic neuritis, ischemic optic neuropathy, or papilledema) and a static clinical course, no further evaluation is generally necessary. The author typically performs the evaluation in stages and, unless there is clear and compelling evidence for a specific etiology, recommends starting with the neuroimaging study (generally, MRI of the head/orbit with gadolinium and fat suppression). If the neuroimaging study is negative, then a directed rather than a shotgun evaluation is recommended for patients who have unexplained optic atrophy.3

In a previous review of the literature (n=447 abstracts) on optic atrophy (English language Medline, 1966-2004)4 the available evidence was graded: class 1 (randomized, prospective, clinical trial), class 2 (prospective or retrospective, large case-control studies), class 3 (cases series or case reports), and class 4 (expert opinion).1-50 Table 3 and Table 4 summarize the historical and examination findings that can direct the evaluation for optic atrophy based upon a literature review and the author's clinical experience.2,4 (No class 1 or 2 data exist.)

To perform this directed (rather than shotgun) laboratory evaluation, a clinician should consider two issues: the pretest likelihood of disease based upon the clinical history and examination and the prevalence of the suspected disorder in the test population. In the previously described study of the diagnostic evaluation for optic atrophy,2 laboratory testing was performed in 51 of 91 patients (44%) and included serologic testing, lumbar puncture, carotid Doppler studies, or heavy metal screening. None of these patients had abnormal laboratory findings that could be causally linked to optic atrophy. Thus, the yield for a nondirected laboratory evaluation is low.

On the other hand, it is known that infectious and inflammatory conditions can produce optic neuropathy. Sarcoidosis can produce optic atrophy from multiple mechanisms including direct granuloma of the optic nerve, inflammatory or infiltrative optic neuritis, intracranial or meningeal involvement, or post-papilledema optic atrophy.21-24 Although an increased incidence of sarcoidosis exists in young African Americans, the disease can occur in any age, gender, or race. Clinical findings such as associated uveitis may increase the likelihood for disease. Also, although Jabs and colleagues in a review of optic neuropathy in systemic lupus erythematosus (SLE) found two of 31 patients who presented with optic atrophy, SLE has specific diagnostic criteria. The author does not typically order testing for SLE (e.g., antinuclear antibody) unless at least two organ systems are involved based on the American College of Rheumatology SLE criteria.36 Lyme disease can produce an optic neuropathy, but testing in non-endemic areas should be reserved for patients with the rash of erythema chronica migrans or a history of tick exposure or travel to an endemic region. The yield for Lyme titer for isolated optic atrophy has not been studied but may still be useful in endemic areas. Criteria for establishing ocular Lyme disease include "the lack of evidence of other disease, clinical findings consistent with Lyme disease, occurrence in patients living in an endemic area, positive (Lyme) serology, and, in most cases, response to treatment."13-14 Optic atrophy in tuberculosis can occur after intraocular (e.g., uveitis), intraorbital, or intracranial tuberculosis (e.g., granuloma or meningitis), or may be the result of toxicity from the treatment (e.g., ethambutol, isoniazid). The yield of tuberculosis screening in isolated optic atrophy has not been studied but is unlikely to occur in isolation.34,35 Tuberculosis screening should be performed in patients from endemic areas (e.g., Asian immigrants), and patients with risk factors for tuberculosis (e.g., immunosuppression or exposure history) or other signs or symptoms of tuberculosis. Leber's hereditary optic neuropathy testing should also be considered in patients with bilateral sequential or simultaneous optic neuropathy (especially presumed toxic-nutritional optic neuropathy) who have the typical history of a young male with bilateral central or cecocentral scotomas with subsequent optic atrophy. Although Leber's hereditary optic neuropathy is typically a disorder of young males, it can occur at any age and in either gender.

Based upon the prior literature review and clinical experience, the yield for laboratory testing is likely to be low in patients without history or examination findings suggestive of an etiology on history or examination.2 Limitations to this "directed" approach to optic atrophy include: the available literature is limited and strong evidence based guidelines can not be made; and the incidence and prevalence of different etiologies for optic atrophy are different for different populations at risk based upon geographic distribution and demographics for the disease (e.g., syphilis, Lyme disease, sarcoidosis, or tuberculosis).

Table 3: Historical Features Suggesting the Etiology of Optic Atrophy (From Lee A, et al. The diagnostic yield of the evaluation for isolated unexplained optic atrophy. Ophthalmology. 2005;112:757-759.)
Historical featureCommentPossible etiology
Painless progressive unilateral or bilateral optic neuropathy.Neuroimaging (usually magnetic resonance imaging with contrast) is recommended.Rule out compressive optic neuropathy.
Painless progressive bilateral simultaneous loss of visual acuity and central visual field (e.g., central or ceco-central scotomas).Consider diagnosis in patients with poor nutrition (e.g., alcoholics, strict vegetarians, gastric surgery), or toxin or drug exposure (e.g., methanol, ethambutol, heavy metals, amiodarone, thallium).Rule out toxic-nutritional amblyopia.
Bilateral rapidly, sequential optic neuropathy with visual acuity and central visual field loss (especially in a young male)Consider mitochondrial testingRule out Leber's hereditary optic neuropathy.
Patients with underlying infectious risk factors (e.g., tick bite, cat scratch, sexually transmitted disease, tuberculosis exposure).Consider chest radiography, purified protein derivative (ppd), Lyme testing, cat scratch antibody testing, syphilis serology.Rule out Lyme disease, cat scratch disease, syphilis, or tuberculosis.
History of inflammatory disorder (e.g., systemic lupus erythematosus, Behcet's disease, Wegener's granulomatosis) or inflammatory symptoms (e.g., rash, sinus disease, renal, pulmonary, cardiac, or gastrointestinal dysfunction) or evidence for inflammatory ocular signs (e.g., anterior or posterior uveitis, sheathing of vessels).Consider antinuclear antibody (ANA), anti-neutrophilic cytoplasmic antibody (ANCA), chest radiograph, renal function testing, urinalysis, medicine consultation.Rule out inflammatory optic neuropathy.
History of prior acute visual loss, pain with eye movement, and visual recovery suggestive of optic neuritis or prior neurologic symptoms suggesting demyelinating disease.Consider neuroimaging and evaluation for demyelinating disease.Rule out multiple sclerosis or other demyelinating optic neuropathy.
History of prior acute and static, painless, visual loss in a patient with vasculopathic risk factors (e.g., hypertension, diabetes, elevated cholesterol, smoking). Arteritic ischemic optic neuropathy typically has associated signs and symptoms of giant cell arteritis.Consider obtaining old records to try and document presence of optic disc edema supporting the diagnosis of anterior ischemic optic neuropathy.Consider old non-arteritic anterior ischemic optic neuropathy. Rule out giant cell arteritis in elderly patients.
Steroid responsive optic neuropathy.Consider inflammatory or autoimmune optic neuropathy.Consider evaluation for sarcoidosis (chest radiograph, angiotensin converting enzyme), lumbar puncture, or other evaluation for underlying inflammatory disease.
History of trauma.Optic nerve is typically normal at onset and develops optic atrophy over time.Consider traumatic optic neuropathy.
Family history of optic neuropathy.Typically bilateral, central and symmetric with childhood or young adulthood age of onset.Consider hereditary optic neuropathy
Table 4: Topographical Localization of a Nonisolated Optic Neuropathy (From Lee A, et al. The diagnostic yield of the evaluation for isolated unexplained optic atrophy. Ophthalmology. 2005;112:757-759.)
Clinical findings in addition to optic neuropathyLocalizationComment
Intraocular inflammation (e.g., anterior or posterior uveitis).Sarcoid optic neuropathy, Lupus optic neuropathy, neuroretinitis, syphilis.Rule out neurosarcoidosis with optic nerve involvement.
Bilateral central or cecocentral scotoma.Bilateral optic nerves.Leber's hereditary optic neuropathy, toxic-nutritional amblyopia (e.g., vitamin B12, folate, CBC), bilateral compressive optic neuropathy less likely (MRI head/orbit).
Proptosis and optic neuropathy (e.g., orbital mass or Grave's ophthalmopathy).Ipsilateral orbit/orbital apex.MRI orbit and head with fat suppression and gadoliniumOrbital CT may be useful for bone and sinus disease.
Any combination of ocular motor cranial neuropathy, Horner syndrome, and optic neuropathy.Superior orbital fissure/cavernous sinus.MRI orbit and head with fat suppression and gadolinium.
Bilateral optic neuropathy, junctional visual field loss, or bitemporal field loss.Bilateral optic nerves, junction of optic nerve and chiasm, or chiasmal lesion.MRI head/orbit with fat suppression and gadolinium.
Homonymous hemianopsia with optic neuropathy (may have band optic atrophy or relative afferent pupillary defect).Optic tract.MR head (sella sequence) with gadolinium.

Step 8: Perform serial evaluations to verify the stability of the visual loss

If a patient with isolated optic atrophy has undergone a negative neuroimaging study and directed laboratory evaluation, then serial follow-up is necessary to ensure stability over time. Progressive symptoms of an optic neuropathy should prompt consideration for a neuro-ophthalmic consultation, repeat neuroimaging, or additional second- or third-line testing (Table 5).

Table 5: Potential Evaluation of Unexplained Optic Atrophy (Modified with permission from Lee AG, Brazis PW. Clinical Pathways in Neuro-ophthalmology: An Evidence-Based Approach. 2nd ed. New York, NY: Thieme; 2003.)
1. First-line testing for nonprogressive, isolated unexplained unilateral or bilateral optic atrophy
  • Consider prior anterior ischemic optic neuropathy
  • Consider prior optic neuritis
  • Magnetic resonance imaging (MRI) of optic nerve(s) and brain with contrast
  • Syphilis serology: Treponemal antibody testing (e.g., fluorescent treponemal antibody [FTA-Abs]) and non-treponemal antibody testing (e.g., rapid plasma reagin [RPR] or Venereal Disease Research Laboratory [VDRL])
  • Radiography or computed tomography (CT) of the chest (if sarcoid or tuberculosis suspected)
  • Serum angiotensin converting enzyme (ACE) if sarcoidosis suspected
2. Second-line testing for progressive optic neuropathy or suggestive clinical history*
  • Erythrocyte sedimentation rate (ESR) or C-reactive protein in elderly patient
  • Lumbar puncture (especially if meningeal signs)
  • Gallium scan or chest CT scan if sarcoidosis suspected
  • Purified protein derivative (PPD) skin testing if tuberculosis suspected
  • Antinuclear antibody (ANA), anti-double-stranded DNA, complement levels, if systemic lupus erythematosus or other collagen vascular disease suspected
  • Lyme titer if in endemic area, history of exposure, or suggestive examination findings
3. Bilateral or rapidly sequential optic atrophy testing*
  • Neuroimaging (i.e., MRI head and orbit with gadolinium and fat suppression)
  • Leber's hereditary optic neuropathy mutation blood test (if appropriate clinical history and examination)
  • Heavy metal screen (if exposure history)
  • Serum vitamin B12 and folate levels (if bilateral central or cecocentral scotoma)
  • Lyme titer (if suggestive history, exposure to endemic area or tick bite)
  • Paraneoplastic antibody profile (if history of underlying malignancy)
  • Lumbar puncture (if indicated)
*Pretest likelihood of disease and prevalence of disease in the study population should be considered prior to ordering these studies.

Step 9: Strongly consider reviewing the neuroimaging with a neuroradiologist in cases of unexplained optic atrophy even with a "normal" imaging report

There are multiple reasons why a neuroimaging study may be reported as "normal" in a patient with a structural cause for optic atrophy including: inadequate scan quality; incorrect area imaged (e.g., head instead of orbit); incomplete study (e.g., lack of contrast material, lack of fat suppression); incorrect study (e.g., CT instead of MRI); artifact obscuring area of interest; or inaccurate neuroradiologic interpretation of study. If a patient has a progressive optic neuropathy, then a repeat neuroimaging study or repeat evaluation should be considered. Personal review of the study with the neuroradiologist and detailed communication of the clinical findings might allow more specific attention to be paid to the optic nerve.

Step 10: Consider consultation with a neuro-ophthalmologist especially in neurologically nonisolated or progressive cases

If the comprehensive ophthalmologist remains uncertain about the evaluation even after considering the steps above, then formal neuro-ophthalmologic consultation may be warranted. This is especially true for cases that are not neurologically isolated or are progressive, or patients who have undergone negative first-line testing including neuroimaging.

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Summary

Optic atrophy remains a difficult and sometimes complex problem for the comprehensive ophthalmologist. A complete history should focus on establishing an etiology for the optic atrophy including the most common causes (i.e., optic neuritis and ischemic optic neuropathy). A complete ocular examination (with formal perimetry) may establish a prior inflammatory or infectious etiology or assist with topographical localization in nonisolated cases.

Neuroimaging should be strongly considered in patients with hemianopic (e.g., junctional, bitemporal, or homonymous) visual field loss, band optic atrophy, progressive symptoms, or nonisolated optic atrophy. Additional laboratory testing should be directed by the risk factors identified in the history or examination rather than a "shotgun" approach. A negative initial imaging study should prompt serial examinations to verify the stability of the visual loss over time. A clinician should consider reviewing the neuroimaging study with a neuroradiologist even in the setting of a normal report and repeat or continued evaluation may be necessary for patients with progressive symptoms or signs. Neuro-ophthalmology consultation and a repeated directed neuroimaging study may be appropriate in selected cases.

Slide 1

Case Study 1

A 30-year-old white woman with multiple sclerosis (MS) for the past 10 years is seen for a routine eye examination. She just recovered from a recent exacerbation of her MS characterized by right lower extremity numbness and tingling. An MRI scan of the head showed multifocal white matter lesions consistent with MS (Slide 1). Her neurologist treated her with intravenous steroids for 5 days followed by an oral taper, and her neurologic symptoms resolved. She is asymptomatic and wants "new glasses." The visual acuity is 20/20 OU. There is a small 0.3-log-unit relative afferent pupillary defect (RAPD) OD. The right visual field shows a small paracentral depression, but the left visual field was normal. The remainder of the eye examination was normal except for mild temporal pallor OD. The patient was presumed to have had prior optic neuritis OD, and a repeat exam 3 months later showed no change in her visual field.

Slide 2


Case Study 2

A 65-year-old man with hypertension, history of smoking, coronary artery disease, obstructive sleep apnea, hyperlipidemia, and diabetes mellitus presents with a history of loss of vision to 20/200 OD that occurred 2 years ago. The ocular examination OS was normal. There was a right relative afferent pupillary defect. Goldmann perimetry showed an inferior altitudinal visual field defect OD and was normal OS. Fundus examination shows optic atrophy OD and a normal optic nerve OS with a cup:disc ratio of 0.1 OS. Review of the records from 1 year ago indicated that the patient had optic disc edema OD (Slide 2) that resolved to sector optic atrophy and the vision remained unchanged since that time. Serial follow-up over the next year showed no change. A presumptive diagnosis of non-arteritic anterior ischemic optic neuropathy (AION) was made.

Case Study 3

A 40-year-old white woman presented with painless progressive loss of vision (20/400) and visual field (diffuse depression) OS and normal acuity (20/20) and visual field OD. She had no hypertension or diabetes. An examination showed a right relative afferent pupillary defect and a diffusely pale optic nerve OD (Slide 3A) with a normal optic nerve OS. Axial T1-weighted contrast-enhanced MRI with fat suppression showed enhancement of the left optic nerve sheath consistent with optic nerve sheath meningioma (Slide 3B).

Slide 3A
 Slide 3B

Case Study 4

A 55-year-old white man with a long history of chronic alcohol abuse (two 12 packs of beer per day for 20 years) and a 120 pack/year smoking history presents with a 2-month history of painless, progressive bilateral visual acuity loss to the 20/80 OD and counting fingers level OS and cecocentral scotomas OU (Slide 4A and Slide 4B). Ophthalmoscopy showed bilateral temporal pallor. Leber's hereditary optic neuropathy testing was negative. An MRI scan of the head was negative but the serum B12 and folate were both low. He was treated with replacement vitamin B12 and folate and multivitamins and discontinued alcohol and tobacco use. One year later, his vision was improved to 20/50 OU.

Slide 4A
 Slide 4B

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