SURVEY OF OPHTHALMOLOGY

VOLUME 36

l

NUMBER 1 . JULY-AUGUST 1991

CLINICAL CHALLENGES,

RONALD M. BURDE, EDITOR

Optic Atrophy MICHAEL

WALL,

M.D.

Department of Ophthalmology, Tulane University Medical Center, New Orleans, Louisiana

Comments by Steven Newman, M.D., Michael Slavin, M.D., and Lyn A. Sedwick, M.D. (In keeping with CPC format the abstract and key words appear at the end of the article.)

Comments

Case Report. A thirty-seven year old woman was well until one month prior to her evaluation when, while driving her car, she noticed she could not see well with her left eye. She also related left frontal headaches. She denied any eye pain or diplopia. Her past medical history was unremarkable except for a history of a skin disorder noted eight years previously. The patient was examined by a general ophthalmologist, found to have optic atrophy, and referred for further evaluation. On examination, the patient had a 1.5 cm hypopigmented macule on her left malar area. The best corrected visual acuity was 20/15 in the right eye and 20/40 in the left. Confrontation fields showed the patient could count fingers in all four quadrants, but on face comparison the examiners faces appeared “foggy” with the left eye. Goldmann perimetry of the right eye was normal. The results of Goldmann perimetry of the left eye are shown below (Fig. 1). She correctly identified 12 of 14 color plates in the right eye and 10 of 14 in the left. There was a 100% red desaturation in the left eye. Pupils were 4 mm, round, and reactive to light with a 1.2 log unit left relative afferent pupillary defect. Motility examination was normal and funduscopic examination showed a pale left optic disc. Computerized tomography showed extensive nodular enhancement of both optic nerves, cavernous sinuses and contiguous meninges (Figs. 2 and 3). A procedure was performed.

Comments by Steven Newman, M.D., Department of Ophthalmology, University of Virginia Health Sciences Center, Charlottesville, Virginia This case raises the familiar problem of a patient presenting with “sudden visual loss.” Because of our binocularity, it is not uncommon for unilateral visual loss to exist unrecognized for months to years. One major clue that visual loss in this patient has not been acute is the presence of optic atrophy. In experimental studies, Quigleyz8 and Anderson’ demonstrated that atrophy of the disc usually takes at least six weeks to develop following experimental optic nerve lesions. The presence of an optic neuropathy is confirmed in this patient by decreased visual acuity, a central scotoma, depressed color vision, and a relative left afferent pupillary defect. Retinal lesions also may be associated with decreased central visual function and even an afferent pupillary defect, but these would usually be seen on funduscopy. With those lesions that tend to affect the small central optic nerve fibers (compression, inflammatory, nutritional, hereditary, and toxic), color vision is relatively damaged. It should be pointed out, however, that not all optic neuropathies reduce central function. In glaucoma, the optic neuropathy of increased intracranial pressure, and in some forms of ischemic optic neuropathy (particularly the rare retrobulbar form), central vi51

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Fig. 2. Computerized tomography scan of the head, with contrast enhancement, shows diffuse optic nerve enlargement. Note also the diffuse nodular enhancement in the area of the cavernous sinuses, and contiguous meninges. Fig. 1. Goldmann perimetry of the left eye showing constriction of the visual field with a central scotoma and associated inferior altitudinal nerve fiber bundle defect.

sual function including color vision and acuity may be relatively well-preserved. In the patient under discussion, the optic neuropathy has been present for some time. The time course, when known, is often very helpful in suggesting an etiology. Inflammatory, ischemic, and toxic optic neuropathies tend to occur acutely. Slowly progressive visual dysfunction is much more compatible with a compressive etiology. Nutritional, toxic, and hereditary optic neuropathies are usually bilateral, although the onset may be asymmetric. The relative preservation of visual acuity here (20/40 vision being quite good in the setting of a 1.2 log unit left afferent pupillary defect) suggests several possibilities: (1) those optic neuropathies that tend to spare central function such as ischemic optic neuropathy; (2) partially recovered optic neuritis where central function tends to recover better than paracentral; (3) a slowly progressive, compressive or infiltrative process (with relative sparing of the central fibers).g Associated symptoms and signs sometimes can help with the differential diagnosis. The presence of pain with ocular motility suggests optic neuritis.“5 Local orbital signs indicate a compressive lesion. A small, anatomically crowded disc may predispose the patient to anterior ischemic optic neuropathy. Optic disc cupping suggests glaucoma, although it can occur with other optic neuropathies, such as giant cell arteritis.34 Optic atrophy alone tends to be

quite nonspecific, although arterial attenuation suggests an ischemic pathophysiology.33 The patient’s age is often helpful in the differential diagnosis of an optic neuropathy. A compressive lesion may occur at any time, but is more frequent in the older or middle-aged patient. (Regardless of age, compressive lesions must always be considered as part of the differential diagnosis.) Optic neuropathy in a young patient is most likely to be inflammatory. Within the first two decades, etiology is usually post-viral. I6 After the teens, the risk of demyelinating disease increases.‘3*36 Ischemic optic neuropathy is much less common in young patients, although it may occur in association with vasospasmz2 (migraine) or with disorders of coagulation. In middle-aged or elderly patients, ischemic optic neuropathy is more frequent,2~“*23 although not as common as glaucoma. In the elderly, we are always concerned about the possibility of giant cell arteritis,5 as the incidence rises dramatically in patients over the age of eighty.4 A particularly interesting aspect of this case is the possible association between the patient’s optic neuropathy and a dermatologic condition. While many pathophysiological mechanisms affect both the ocular surface and the facial or eyelid skin, the embryologic connection between the epithelium and the rest of the visual system is less obvious. The phakomatoses are a group of syndromes encompassing involvement of the skin, eye, and central nervous system. The embryologic connection here is not with the epithelium per se, but rather with the underlying neural-crest-derived cells that make up

OPTIC

53

ATROPHY

Fig. 3. Computerized tomography head scan with contrast shows diffuse nodular enhancement in the area of the cavernous sinuses, and anterior and middle fossa extending into the left frontotemporal convexity.

both the visual pathways and the other nerves of the central and peripheral nervous system. In neurofibromatosis (von Recklinghausen’s disease), the first described35 and the most common of the phakomatoses, skin manifestations include cafeau-lait spots, axillary freckles, multiple skin fibromas, and plexiform neurohbromas. This later lesion, often described as a “bag of worms,” may affect the eyelids and orbit and is often associated with underlying bony overgrowth and facial asymmetry. Additional ophthalmic manifestations include significant increased incidence of congenital glaucoma, iris melanocytic (Lisch) nodules, thickening of the cornea1 nerves, and occasional astrocytic hamartomas in the fundus.lg The central nervous system manifestations” include an increased incidence of neurofibromas, schwannomas, and meningiomas.” Meningiomas in young patients are almost always associated with neurofibromatosis.15 Finally, gliomas of the visual system have a higher incidence in patients with neuroftbromatosis.‘* Ophthalmic involvement occurs in greater than 50% of patients with tuberous sclerosis.“g Optic nerve head drusen, occasionally associated with decreased visual field and acuity, and so-called “mulberry” drusen often involve the disc (astrocytic hamartomas). Additional astrocytic hamartomas may involve the retina more peripherally and, of course, papilledema may result from ventricular obstruction and hydrocephalus. Mental retardation and seizure activity are related to calcified glial nodules often around the ventricles (subependymal astrocytic hamartomas), basal ganglia, or cortex (cor-

tical tubers). It should be noted, however, that the classic triad’ of epilepsy (myoclonic spasms in infancy replaced with grand ma1 seizures as the patients age), mental retardation, and adenoma sebaceum occurs in only the minority of cases.” While adenoma sebaceum (really angiofibromas) is the most characteristic cutaneous manifestation in tuberous sclerosis, hypopigmented macules, “mountain ash leaf spots,” are a common finding.‘* Tumors may involve other organs, especially the kidneys (angiomyolipoma). Other phakomatoses including von Hippel-Landau, Sturge-Weber, Wyburn-Mason, and ataxia-telangiectasia, are not routinely associated with optic nerve pathology. In this patient, the history of a “skin disorder” plus the 1.5 cm hypopigmented macule on her left malar region is interesting. It is certainly possible that this represents a manifestation of one of the phakomatoses. While the skin description would be more compatible with tuberous sclerosis, the unilateral optic nerve pathology without evidence of increased intracranial pressure is far more suggestive of neurofibromatosis. The presence of an optic nerve glioma could cause moderately decreased vision, a fairly prominent afferent pupillary defect, and optic atrophy. A possible mass lesion should be suspected, and a neuroimaging study is clearly indicated. Since introduction, CT scanning has become the gold standard of orbital and anterior visual pathway assessment. In particular, an enhanced direct coronal CT will visualize the optic nerve through the orbit, canal, and intracranial subarachnoid cistern to the chiasm. This is highly sensitive to a mass lesion within the orbit or canal. Because of the surrounding CSF, CT is relatively less sensitive to the presence of intracranial optic nerve pathology. On the other hand, magnetic resonance images easily distinguish the intracranial optic nerve and chiasm. MRI is particularly sensitive to blood (both old and new), inflammatory lesions, and low-grade gliomas.” MRI has been somewhat weak in studying the orbit, mainly because of the high signal (on Tl weighted images) from orbital fat, the long data acquisition time (leading

to artifacts of movement),

of bone signal (this may actually assessing

and the lack

be an advantage

in

lesions in the orbital apex). MRI has been

particularly disappointing in the study of meningiomas, the most common intracranial tumor to affect the orbit. The introduction of paramagnetic enhancement agents (gadolinium) has markedly improved the ability to image meningiomas. Gadolinium is quite useful in separating tumor from surrounding edema and visualizing dural pathology, although it is non-specific, i.e., it will enhance any area of blood-brain barrier breakdown.

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Angiography, while still a primary diagnostic procedure for arteriovenous malformations and aneurysms, is now used in a more adjunctive fashion following MRI and CT. Pneumoencephalography and venography have essentially been replaced by computerized imaging. SPECT and PET scanning can provide additional information about blood flow and metabolism. Because of the equipment requirements, however, they are not generally clinically available. I would suggest an initial work-up in this woman consisting of an MRI scan, possibly with gadolinium enhancement; images should be obtained in the coronal plane. If MRI is not available, an enhanced direct coronal CT scan would be the study of choice. I would also suggest obtaining automated quantitative static perimetry as a baseline in this patient. Especially in this setting with relatively retained central acuity, static perimetry may be very sensitive to subtle changes in the patient’s optic neuropathy. Static perimetry, obviously combined with repeat measurement of Snellen acuity, contrast sensitivity, and color vision, may well be more sensitive to subtle changes in the patient’s optic neuropathy. Static perimetry, obviously combined with repeat measurement of Snellen acuity, contrast sensitivity, and color vision, may well be more sensitive to progression than repeat neuroimaging studies. If the neuroimaging studies are negative, I might consider a spinal fluid analysis with cytospin and oligoclonal band testing and serum screening studies for an infectious or autoimmune disorder (VDRL, sedimentation rate, ANA). If these studies are unremarkable I would document the disc appearance with fundus photography and follow the patient. I routinely see patients with optic neuropathies for follow-up in six weeks, although I see them sooner if I am concerned about a possible evolving pathology. The patients are told to call immediately if they experience any perceived change in ocular symptoms.

Comments by Michael L. Slavin, M.D., Department of Ophthalmology, Long Island Jewish Medical Center, New Hyde Park, New York A 37-year-old woman first “noticed” left visual dysfunction one month earlier. Her past medical history revealed associated left frontal headaches and a “skin disorder,” which had presented eight years previously. On neuro-ophthalmologic examination, classical features of a left optic neuropathy were detected: afferent pupil defect, dyschromatopsia, central scotomas with inferior visual field extension and optic atrophy. The findings of frank

WALL optic atrophy just one month after the onset of symptoms makes an acute process unlikely. Although it is possible that the patient first “noticed” monocular visual loss that had actually occurred years earlier (e.g., an old optic neuritis, ischemic optic neuropathy, para-infectious or traumatic optic neuropathy), a slowly progressive optic neuropathy or acute decompensation of a chronic process (acute-on-chronic) are more likely. In real time, my initial approach in this case would be to get a complete history regarding the associated frontal headaches. Are the headaches long-standing and intermittent (of the migrainous type), or are they more suggestive of an intracranial space occupying lesion? The dermatologist or internist should be contacted in reference to the present and past cutaneous lesion(s), in case the visual loss is related to the skin disorder. A family history regarding neoplasia and dermatologic conditions should be sought. For the purpose of this “Clinical Challenge,” however, Dr. Wall has elected to withhold specific information regarding the skin disorder. In the discussion that follows, I will address the more general issue of the evaluation of optic atrophy. I will then integrate where possible the association of optic atrophy with cutaneous disorders and specifically “hypopigmented” skin macules. An acquired optic neuropathy may be caused by specific clinical entities which fall into the following categories: vascular, inflammatory/infectious, compressive/neoplastic, demyelinating, traumatic, toxic, metabolic/endocrine, heredofamilial, and degenerative. Demyelinating (optic neuritis) and vascular (anterior and posterior ischemic optic neuropathy) disease, for all intents and purposes, cause acute visual loss, which is not in keeping with the temporal profile in this case (vide supra). Anterior ischemic optic neuropathy (e.g., caused by vasculitis, migraine, etc., in the young) would present with optic disc edema during the acute phase and optic atrophy with retinal arteriolar narrowing later on. A traumatic etiology is excluded by history and the fact that visual loss with this entity is usually stable. Toxic processes (e.g., tobacco/alcohol, ethambutol, chloramphenicol, etc.) are invariably bilateral (although perhaps asymmetrical). Inherited optic neuropathies are also bilateral, associated with ceco-central scotomas, a positive family history, and in Leber’s hereditary optic neuropathy,32 typical fundus findings (circumpapillary telangiectasia). Graves’ ophthalmopathy may be associated with an optic neuropathy, but other clinical signs (ophthalmoplegia, proptosis, etc.) are invariably present. Metabolic and degenerative processes rarely cause isolated optic neuropathy (notwithstanding skin disease).

55 A compressive/neoplastic optic neuropathy (a likely etiology in a patient with visual loss and headache) should be ruled out by high resolution neuroimaging (CT, MRI). An orbital lesion (e.g., cavernous hemangioma, or an intrinsic lesion such as optic glioma or optic nerve sheath meningioma) compressing the optic nerve would commonly be associated with proptosis and sometimes ophthalmoplegia, which were not evident in this case. A careful perimetric evaluation of the fellow eye to exclude subtle temporal or supero-temporal defects is mandatory. Such evidence of chiasmal involvement almost always leads to the finding of a compressive etiology. Compressive lesions of the intracranial portion of the optic nerve may be caused by pituitary adenomas, craniopharyngiomas, and suprasellar meningiomas, although signs of chiasmal involvement or bilateral optic nerve dysfunction are more likely with those entities. A meningioma in the vicinity of an anterior clinoid process may result in slowly progressing monocular visual loss. Acute-onchronic visual loss may be seen with expansion of the cystic component of a craniopharyngioma or hemorrhage into a pituitary adenoma (pituitary apoplexy). Again, bilateral visual loss with evidence of chiasmal involvement is usual. Aneurysmal compression of the anterior visual pathwaysz5 may result in a chiasmal syndrome which is indistinguishable from other causes, but commonly may cause insidious unilateral visual loss. Sudden aneurysmal expansion may give rise to an acute-on-chronic process and headache. Infiltrative optic neuropathies (e.g., leukemia, lymphoma) will almost always be associated with systemic manifestations. In what conditions may a compressive optic neuropathy be associated with cutaneous signs? Neurofibromatosis is a hereditary disorder in which cutaneous signs (cafe ou lait spots, plexiform neuromas and axillary freckles) may be associated with peripheral nerve schwannomas and intracranial tumors (optic or chiasmatic glioma, meningioma, acoustic neuroma). The skin lesions, however, are apparent within the first several years of life, as opposed to the late onset dermatologic dysfunction noted in Dr. Wall’s patient. In tuberous sclerosis, astrocytic hamartomas of the optic nerve and retina may be detected, while similar lesions may be found intracranially. Skin lesions include adenoma sebaceum and hypopigmented macules on the trunk and limbs (ash leaf sign), The associated mental retardation and seizure disorder in many such cases, as well as the early onset of disease, make the diagnosis straightforward. Inflammatory/infectious causes of optic neuropathy are legion, and may present as acute, subacute, chronic progressive or acute-on-chronic visual loss.

Treponemal infections, such as syphilis3’ and Lyme disease,” may be associated with a present or past history of a cutaneous lesion (vide in+). Visual loss with syphilis may occur at the time of the rash of secondary disease or within years of the onset of infection (syphilitic meningitis, tabes dorsalis). Acute or chronic progressive optic neuropathy may be the sole sign in some cases. Sarcoidosis,’ a granulomatous inflammatory disease of young adults, may rarely present with isolated involvement of the optic nerves or chiasm. In most reported cases, the diagnosis may be made by the finding of an abnormal chest x-ray or elevated angiotensin converting enzyme (ACE). Neuro-imaging may show a diffuse mass lesion confluent with anterior visual pathways. Infectious causes of optic neuropathy, such as tuberculosis and crytococcosis, among others, will often present as a meningitic process with symptoms including headache and visual loss. A lumbar puncture would be diagnostic. Lastly in this category, optic neuropathy associated with collagen vascular diseaseI should be addressed. A patient with diagnosed systemic lupus erythematosis (SLE) may suffer visual loss due to retinovascular disease or, less commonly, to optic neuropathy. On examination, a picture of anterior ischemic optic neuropathy may be noted, or findings compatible with acute retrobulbar optic neuropathy, or insidious visual loss and optic atrophy. In almost all cases, however, there are associated systemic manifestations, including transverse myelitis, seizures, serositis, pericarditis, psychosis, nephritis, and cutaneous vasculitis. In a review of the literature by Jabs and associates,‘4 only 2 of 31 cases (including their 7 cases) presented with optic neuropathy as the initial sign of SLE. Although visual outcome is poor in most cases, it is prudent to use systemic corticosteroids in hopes of recovery of the affected eye, to ward off visual involvement of the fellow eye, and to prevent vasculitic spinal cord involvement. Severe recurrent or progressive optic neuropathy in patients with serological evidence of autoimmune disease but without accompanying systemic features of collagen vascular disease comprise the category of autoimmune optic neuropathy.‘j*” Visual loss is often forestalled or reversed with high dose corticosteroids, but cytotoxic agents are sometimes necessary to preserve vision. Many of the inflammatory processes that cause optic neuropathy above are associated with dermatopathology. Patients with SLE, for instance, may display a macular butterfly eruption, which is usually erythematous. Chronicity of the process (postinflammatory) may result in atrophic skin patches with hypopigmented areas. The rash of SLE, however, is typically accompanied by fever, malaise, and

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sometimes arthalgia. Chronic discoid lupus is an indolent skin disease often affecting the face. The lesions may consist of erythematous papules and plaques, which later become atrophic and hypopigmented. A very small percentage of such patients will develop SLE. Sarcoidosis may affect the skin with multiple maculopapular small yellowishbrown lesions or with the erythema nodosum syndrome. Erythema chronicum migrans will usually be the earliest sign of Lyme disease, and a diffuse fleeting maculopapular rash (including the palms and soles) is usually evident in the secondary stage of syphilis. Hypopigmented anesthetic macules may be seen with leprosy, but, to my knowledge, this disease has not been associated with optic neuropathy. Lastly, rare vasculitic mucocutaneous ocular syndromes such as Behset’s disease and Dego’s syndrome (malignant atrophic papulosis) may cause retinal vascular occlusion and secondary optic atrophy, in addition to devastating systemic findings. In summary, a 37-year-old with insidious visual loss and optic atrophy has frontal headaches, and a skin disorder which presented eight years earlier. A CT scan (MRI) should be obtained to rule out a compressive or inflammatory process. The hypopigmented skin lesion may reflect a post-inflammatory process. If neuro-imaging is nondiagnostic, a chest x-ray and ACE should be done to rule out sarcoidosis, FTA-ABS and Lyme titer for treponema1 disease, and ANA and LE prep for collagen vascular disease. A team effort including an internist and dermatologist would be indicated in the work-up of this challenging case.

Comments by Lyn A. Sedwick, M.D., Department of Neuro-Ophthalmology, Florida Hospital, Orlando, Florida This patient noted visual loss in her left eye, and on examination had decreased vision, visual field loss in a nerve fiber bundle pattern, decreased color vision and left optic atrophy. In taking the history, it is important to try to discover whether there is a difference between the likely time of onset of the visual problem versus her awareness of it. For example, did she notice the visual defect only because a bug flew into her right eye and blocked its vision momentarily, or did she feel that her vision was blurred and then cover her eyes independently to find that the left eye was the source of the blur? Although the presence of optic atrophy indicates that at least six weeks have passed since the nerve was significantly damaged, the history might suggest a much longer timeframe. Continuing the history-taking, one might ask whether the patient had ever had an injury to the left eye or brow. Traumat-

WALL ic optic neuropathy can result from relatively mild head trauma, and may not be fully appreciated at the time of the injury, in my experience. After obtaining a complete ocular history, I would want to know more about her history of a skin disorder. The most dramatic skin-and-eye diseases are the phakomatoses - Sturge-Weber, von Hippel-Lindau, neurofibromatosis and tuberous sclerosis - which are generally recognized in childhood and have characteristic features. Her only skin abnormality mentioned on examination is a “hypopigmented macula” on her face. It seems unlikely that this represents an “ash leaf’ spot of tuberous sclerosis, since there are no other stigmata of the disease. More likely would be a postinflammatory hypomelanosis, as might be seen in a variety of conditions. Candidates include sarcoidosis, systemic lupus erythematosis, syphilis, and Lyme disease. To complete the initial evaluation of this patient, one would need a few pertinent negatives from her ocular examination. Her intraocular pressures are not given, nor is there any comment about whether orbital asymmetry, proptosis or decreased retropulsion is present. An orbital or sphenoid bone lesion, such as meningioma or optic nerve sheath tumor, would probably cause proptosis at this stage of optic atrophy, but this might be a subtle finding. Asymmetric glaucoma does occur and the atrophy associated with it may be difficult to distinguish from nonglaucomatous optic atrophy.33 Also, early loss of visual acuity can occur in glaucoma.27 What is likely to be the matter with this patient, given her history and examination? A practical differential diagnosis list includes previous incompletely healed optic neuritis and compressive optic neuropathy, with honorable mention to asymmetric glaucoma and traumatic optic neuropathy. Although she has no marked proptosis or other evidence indicative of an orbital, sphenoid, or optic nerve tumor, her discovery of visual loss after the development of optic atrophy is most consistent with slow compression of the optic nerve and subsequent decline in visual function. She has a history of left frontal headaches, and it is tempting to guess that she will harbor a subfrontal meningioma as the cause of both her headaches and optic atrophy. Any lesion selectively pressing on the left optic nerve is possible in this case, including a pituitary adenoma, for example. The slow-growing nature of meningioma puts this lesion at the top of my list for compressive optic neuropathy in this case. Because the possibility of a tumor is so high, my first test in this patient would be either a computerized tomographic scan with and without contrast dye or a gadolinium-enhanced magnetic resonance scan, either done with special attention to the anterior visu-

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ATROPHY

Fig. 4. Skin biopsy showing non-caseating typical of sarcoidosis.

al pathways

(orbits

and sella).

If a good

quality

computerized

granulomata

Follow-up A biopsy of the facial lesion revealed non-caseating granulomas (Fig. 4). The patient was treated with prednisone, 80 mg/day with marked improvement in her visual function. Final diagnosis: sarcoidosis.

tomographic

or

magnetic resonance scan were negative, then optic neuritis with incomplete healing would need to be considered.

Syphilitic

optic neuritis

coidosis is a serious diagnostic contender based on previous impressions of her skin rash or laboratory testing, lumbar puncture again is in order, as involvement of the optic nerve may be evidence of central nervous system involvement with sarcoidosis. To recap, this 37-year-old woman with unilateral optic atrophy deserves first either good computerized tomographic or magnetic resonance scanning to rule out a compressive lesion. If this is unrevealing, she should have a chest radiograph and be sent to the laboratory for complete blood count, sedimentation rate, angiotension converting enzyme, VDRL, FTA-ABS, antinuclear antibody testing and Lyme disease antibody testing. Depending on the above results, lumbar puncture may be necessary. If all this is negative, careful serial checks of intraocular pressure should be performed and the patient followed regularly.

and perineuri-

tis with subsequent optic atrophy is possible, as is optic neuritis with Lyme disease.’ Either disease can

cause skin rash and, thus, this patient’s history of a previous skin disorder becomes interesting. Hypopigmented macula can be seen as a postinflammatory lesion in secondary syphilis.24 For either disease, serologic testing is important - VDRL and FTA-ABS or their equivalents for syphilis and Lyme antibody titers. If any of these are positive, lumbar puncture is indicated to look for evidence of current activity in the cerebrospinal fluid and to guide therapy. Even if serologic testing is positive for either disease, without other historical or characteristic clinical features, diagnosis of either syphilis or Lyme disease as the cause of the optic atrophy should probably be made only after exclusion of all other possibilities. Two other types of optic neuritis deserve consideration in this case - that associated with systemic lupus erythematosis and sarcoidosis. Either of these diseases can cause a skin disorder and can produce hypopigmented areas as the lesions hea1.24 Testing should include chest radiograph, angiotension converting enzyme level, complete blood count, antinuclear antibody, and sedimentation rate. If sar-

Editor’s Comments Dr. Wall has chosen to emphasize the difference between sudden visual loss and the sudden discovery of visual loss. The discussants were not privy to the results of the neuroimaging studies, which made their assignment tougher. Altogether, an exercise well worth our effort.

References 1. Anderson DR: Ascending and descending optic atrophy produced experimentally in squirrel monkeys.Am J Ophthdmol 76:693-7 11, 1973 2. Boghen DR, Glaser JS: lschemic optic neuropathy. Bruin

98:689-708,

1975

3. Bourneville DM: Contribution a l’ttude de l’idiote: S&rose tuberose des circonvolutions c&+brale: Idiote et epilepsie hemiplegique. Arch New01 1:89-91, 1880 4. Cullen JF: Occult temporal arteritis. A common cause of blindness in old age. Br J Ophthalmol 51:513-525, 1967 5. Cullen JF, Coleiro JA: Ophthalmic complications of giant cell arteritis. Sun, Ophthalmol 20:247-260, 1976 6. Dutton JJ, Burde RM, Klingele TG: Autoimmune retrobulbar neuritis. Am J Ophthalmol 94: 11-l 7, 1982 7. Farris BK, Webb RM: Lyme disease and optic neuritis. J Clin Neuro-Ophthalmology 8:73-78, 1988 8. Gelwan MJ, Kellen RI, Burde RM, Kupersmith MJ: Sarcoidosis of the anterior visual pathway: successes and failures. J Neural Neurosurg Psychialr 51:1473-1480, 1988 9. Goodman ST, Rosenbaum AL, Hasso A, Itabashi H: Large optic nerve- glioma with normal vision. Arch Ophthal&l 93:991-995, 1975 10. Haik BG, Saint Louis L, Bierly J, et al: Magnetic resonance imaging in the evaluation of optic nerve gliomas. Ophthalmology 94:709-717, 1987

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11. Hayreh SS: Anterior ischemic optic neuropathy: I. Terminology and pathogenesis. Br J Ophthalmol58:955-963, 1974 12. Hurwitz S, Braverman IM: White spots in tuberous sclerosis.] Pediatr 77.587-594, 1970 13. Hutchinson WM: Acute optic neuritis and the prognosis for multiple sclerosis. J Neuro Neurosurg Psychiatr 39:283-289, 1976 14. Jabs DA, Miller NR, Newman SA, et al: Optic neuropathy in ;Tz6mic lupus erythematosis. Arch Ophthulmol104:564-568, 15. Karp LA, Zimmerman LE, Borit A, Spencer W: Primary intraorbital meningiopas. Arch Ophthalmol 91:2428, 1974 16. Kennedy C, Carol1 FD: Optic neuritis in children. Arch Ophlhulmol63:747-755, 1960 17. Kupersmith MJ, Burde RM, Warren FA, et al: Autoimmune optic neuropathy: evaluation and treatment. J Neural Neurosurg Psych&r 51:1381-1386, 1988 18. Lewis RA, Gerson LP, Axelson KA, et al: von Recklinghausen neurofibromatosis. II. Incidence of optic gliomata. Ophi%almology91:929-935, 1984 19. Martyn LJ, Knox DL: Glial hamartoma of the retina in generalized neurofibromatosis, von Recklinghausen’s disease. BrJ Ophthalmol56:487-+9l, 1972 20. Mashiyama S, Mori T, Seki H, Suzuki J: Multiple brain tumours with von Recklinghausen’s disease. Actu Neurochir 84:29-35, 1987 21. MacDonald AB: Lyme disease. A neuro-ophthalmologic view. J Clin Neuro-ophthalmol 7:185-190, 1987 22. McDonald WI, Sanders MD: Migraine complicated by ischemic papillopathy. Lancet 2.521-523, 1971 23. Miller CR, Smith JL: Ischemic optic neuropathy. Am J Ophthalmol62:103-115, 1966 24. Mosher DB, Fitzpatrick TB, Ortonne JP, Hori Y: Disorders of pigmentation, in Fitzpatrick TB, Eisen AZ, Wolff K, et al ~gd~;I;nnafolo~ in General Medicine, Vol I, 1987, ed 3, pp 25. Norwood

EC,

Kline

LB,

Chandra-Sekar

B, Harsh

CR:

26. 27.

28.

29.

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31. 32. 33.

34.

35.

36.

Aneurysmal compression of the anterior visual pathways. Neurology 36:1035-1041, 1986 Perkin GD, Rose FC: Optic Neuritis and Its Differenlial Diagnosis. Oxford, Oxford Medical Publications, 1979, pp 33-37 Pickett JE, Terry SA, O’Connor PS, O’Hara M: Early loss of central visual acuity in glaucoma. Ophthulmology 92:891-896, 1985 Quigley HA, Davis EB, Anderson DR: Descending optic nerve degeneration in primates. Invest Ophthalmol Vis Sci 16:841-849, 1977 Robertson DM: Ophthalmologic findings, in Gomez MR (ed): Tuberous Sclerosis. New York, Raven Press, 1979, pp 121-142 Rubenstein AE, Mytilineoau C, Yahr MD, Revoltella RP: Neurological aspects of neurofibromatosis. Adv Neural 29: 11-21, 1981 Simon RP: Neurosyphilis. Arch Neural 42:606-613, 1985 Smith JL, Hoyt WF, Susan JO: Ocular fundus in acute Leber optic neuropathy. Arch Ophthalmol90:344354, 1973 Trobe JD, Glaser JS, Cassady JC: Optic atrophy. Differential diagnosis by fundus observation alone. Arch Ophthulmol 98:1040-1045, 1980 Trobe JD, Glaser JS, Cassady J, et al: Non-glaucomatous excavation of the optic disc. Arch Ophthnlmol98:lO4t%lO5O, 1980 von Recklinghausen FD: Uber die multiplen Fibrome der Haut: Ihre Beziehung zu den mulliplen Neuromen. Berlin, A Hirschwald, 1882 Wikstrom J, Poser S, Ritter G: Optic neuritis as in initial symptom in multiple sclerosis. Acta Neural Scandinav 61: 178-185, 1980

Supported in part by an unrestricted Prevent Blindness, Inc., NY, NY. Reprints are not available.

grant

from Research

Abstract. A young woman became suddenly aware of visual loss in her left eye. She was found to have optic atrophy giving chronicity to the disease process. A hypopigmented macule on her face along with neuroimaging studies suggested an inflammatory process. A biopsy of the skin lesion was compatible with sarcoidosis. The patient responded to corticosteroid therapy. Surv Ophtbalmol35:51-58, 1991)

Key words. skin lesion

l

inflammatory visual loss

disease

l

optic neuropathy

l

saracoidosis

l

to