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Progressive and Recurrent Nonarteritic Anterior Ischemic Optic Neuropathy
Progressive and Recurrent Nonarteritic Anterior Ischemie Optic Neuropathy Mark Borchert, M . D . , a n d S i m m o n s Lessell, M . D .
We examined ten patients with nonarteritic anterior ischemie optic neuropathy in whom the visual deficit progressed over a number of weeks or who suffered a recurrence in the same eye. The visual deficits progressed over a month or more in five patients, four patients suffered a recurrence in the same eye after a long interval, and one patient had bilateral progression and a recurrence. NONARTERITIC ANTERIOR ischemie optic neu ropathy characteristically causes precipitous visual loss. The visual deficit is either maximal when first noticed or evolves over hours to days. Progression rarely occurs over weeks. 1 After an interval, the fellow eye is commonly involved but recurrences in the same eye are said to be uncommon. 1 We examined ten pa tients with nonarteritic anterior ischemie optic neuropathy w h o suffered recurrences in the same eye or progression over intervals ranging from weeks to months.
Case Reports Case 1 A 68-year-old man was well except for a maculopathy of his right eye that had not af fected his vision. On Jan. 12, 1985, he suddenly lost part of the lower visual field of his left eye. There was no associated pain or other symp toms. When examined 48 hours later, his visual acuity was 20/15 in each eye and he correctly identified all Ishihara color test plates. The left eye had a relative afferent pupillary defect, segmentai disk edema, and an inferior altitudiAccepted for publication June 3, 1988. From the Department of Ophthalmology, Massachu setts Eye & Ear Infirmary, Boston. Reprint requests to Simmons Lessell, M.D., Depart ment of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles St., Boston, MA 02114.
nal visual field defect. Over the next five days, the visual field defect gradually enlarged. Re sults of a neurologic examination, computed tomographic scan of the head with contrast injection, and an erythrocyte sedimentation rate were all normal. Three weeks of oral highdose prednisone treatment did not appear to influence his course, and the visual field defect slowly progressed over the next three months. The defect extended into the upper half of the field and involved fixation (Fig. 1). By April 30, 1985, visual acuity in the left eye was 20/200 and there was a profound dyschromatopsia. Visual function in his left eye has remained unchanged since that time. On Sept. 29, 1985, the patient experienced a ten-minute episode of pulse-synchronous flashing lights, with obscuration of vision on right gaze. Findings on examination the follow ing day were unchanged from five months earlier. Results of oculoplethysmography were normal. On Nov. 29, 1985, the vision in his right eye suddenly became impaired. Visual acuity was correctable to 20/20 and color vision was unim paired, but there was an inferior altitudinal visual field defect and sectoral disk edema. He was hospitalized and treated with nifedipine, high-dose prednisone, acetazolamide, topical timolol, and oxygen inhalation. Despite this regimen, his visual loss progressed over the next month. His disorder then stabilized until July 22, 1986, when he suddenly lost more of his right visual field (Fig. 2). At that time, results of a temporal artery biopsy, cerebrospinal fluid examination, serologie test for syphi lis, serum protein and lipoprotein electrophoresis, chest x-ray, serum angiotensin converting enzyme level, tuberculin skin test, and complete blood cell count were normal. Results of repeat head and orbit computed tomography were unchanged. During the next 18 months he lost two more areas of his field, but his visual acuity remained 20/20.
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Fig. 1 (Borchert and Lessell). Case 1, left eye. Left, Automated visual field at the initial examination. Numbers indicate the depth of the defect in decibels. Right, Visual field 3V2 months later.
Case 2 A 50-year-old man with insulin-dependent diabetes mellitus for two years had injured his right eye in childhood, which reduced visual acuity in that eye to counting fingers. Five days after a routine ophthalmologic examination, in which visual acuity in his left eye was 20/20, he suddenly and painlessly lost the inferior visual
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field of that eye. Reexamination showed that visual acuity was 20/50 and the upper half of the left disk was edema tous. Visual field exami nation demonstrated an inferior altitudinal de fect (Fig. 3). Results of a complete blood cell count, erythrocyte sedimentation rate, serolog ie test for syphilis, antinuclear antibody titer, and serum angiotensin converting enzyme
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Fig. 2 (Borchert and Lessell). Case 1, right eye. Left, Automated visual field on Nov. 30, 1985. Right, Visual field 15 months later.
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Fig. 3 (Borchert and Lessell). Case 2, left eye. Left, Goldmann visual field at time of initial visual loss. Right, Goldmann visual field after progression of visual loss one month later.
level were normal. His field loss was stable for one week, but then slowly worsened over the next month, leaving him with a small island of central vision in his left eye (Fig. 3). Visual acuity remained 20/50. His left disk was pale superiorly and edematous inferiorly. Results of cytologie cerebrospinal fluid stud ies, chest x-ray, computed tomographic scan of the head and orbits with contrast injection, and tuberculin skin test were all normal. Case 3 A 57-year-old healthy man consulted his ophthalmologist because he had seen flashing light for four days and had blurred vision for two days in his left eye. Visual acuity was 20/20 in each eye and results of color vision testing were normal. However, there was a left superonasal visual field defect and the inferior portion of the left disk was edematous. One month later, his vision again suddenly blurred. Visual acuity decreased to 20/50 and there was a central visual field defect. There was dyschromatopsia and a left afferent pupillary defect. The left disk was diffusely swollen. Results of an erythrocyte sedimentation rate were normal and a temporal artery biopsy showed normal tissue. Three months later, the patient realized that he had lost more of his visual field. Results of perimetry confirmed his impression. The left disk was still diffusely edematous. Results of a computed tomographic scan of the head and orbits with contrast injection, erythrocyte sedi mentation rate, antinuclear antibody titer, ser
ologie test for syphilis, and serum protein electrophoresis were normal. The patient refused a lumbar puncture. His findings in the left eye have remained unchanged. Ten months after the condition of his left eye stabilized, he suddenly lost the lower half of the field of his right eye. Examination showed that visual acuity was 20/20 and results of color vision testing were normal, but he had an inferior altitudinal visual field defect and edema of the superonasal segment of the disk. One week later, visual acuity in the right eye decreased to counting fingers and the disk swelling had become more extensive. Vision never recovered. Case 4 A 74-year-old man with treated hypertension and atrial fibrillation suffered sudden, painless loss of vision in his left eye in June 1985. His ophthalmologist diagnosed nonarteritic ante rior ischemie optic neuropathy. Visual acuity was 20/30, and he had an inferior altitudinal visual field defect and optic atrophy. Findings in the right eye were unremarkable. He was then well until Sept. 22, 1986, when sectoral edema of the right optic disk was noted on a routine ophthalmologic examination. Vis ual acuity in the right eye was 20/20 and the visual field was full. He then described a slowly progressive "break-up" of the upper half of the visual field of his right eye. When his condition stabilized three months later, visual acuity was 20/40, there was a dense, extensive upper alti tudinal visual field defect, and his disk was
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atrophie. Results of a temporal artery biopsy, erythrocyte sedimentation rate, and computed tomographic scan of the head and orbits with contrast injection were normal. There have been no subsequent changes. Case5 A 65-year-old woman with treated endometrial carcinoma suddenly lost the superonasal quadrant of her left visual field in September 1975. Visual acuity was R.E.: 20/25 and L.E.: 20/30. Perimetry demonstrated a superonasal step in her left eye. The left disk was swollen inferiorly and there was a peripapillary hemor rhage. Results of an erythrocyte sedimentation rate were norma}. The patient, a physician, checked her central visual field every other day with an Amsler grid. No change was noted until April 1976, at which time she found that her field loss had increased (Fig. 4). On reexamination, visual acuity was still 20/20 but there was edema of the left disk, especially superiorly. Results of a repeat erythrocyte sedimentation rate were normal. Her vision has remained unchanged and her general health has been good over the ensuing 12 years. Case 6 A healthy 66-year-old man lost vision in his left eye over a one-week period. When vision started to fail in his right eye 5Vi weeks later, he
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consulted an ophthalmologist. He had a life long exotropia and a visual acuity of R.E.: 20/25 and L.E.: 20/20 had previously been recorded. On examination, visual acuity was R.E.: 20/40 and L.E.: light perception. A scotoma was noted superotemporal to fixation when the pa tient viewed an Amsler grid, but no defect could be detected on a tangent screen in the right eye. There was a left afferent pupillary defect. The right disk was edematous and the left was atrophie. His erythrocyte sedimenta tion rate was 11 mm/hour and temporal artery biopsy yielded normal tissue. Over the next two weeks there was progres sive loss of the superior portion of the visual field of the right eye. Reexamination showed no change in visual acuity, but there was a dense, extensive upper altitudinal field defect in the right eye. No change has occurred in the subse quent three years. Case 7 A 55-year-old healthy man was examined by his ophthalmologist on April 9, 1977, because the inferior field of his right eye had been blurred for two weeks. Visual acuity was R.E.: 20/20-2 and L.E.: 20/20. There was a partial inferior altitudinal field defect of variable den sity in the right eye. The right disk was swol len, with peripapillary hemorrhages. His erythrocyte sedimentation rate was 4 mm/hour and a computed tomographic scan of the head
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and orbits with contrast injection showed no abnormalities. Thereafter, he suffered further progressive loss of vision in his right eye. By April 14, 1977, visual acuity had decreased to 20/100. Two weeks later, it was counting fingers at 2 feet. He was left with optic atrophy and a central scotoma, although visual acuity improved to 13/200 after several weeks. Case 8 On Feb. 11, 1986, a healthy 65-year-old woman suddenly noticed a "film" over the lower part of the visual field of her right eye. There were no symptoms of giant cell arteritis. Examination two days later disclosed a visual acuity of 20/20 in each eye and no pupillary abnormality. The upper half of the right disk was edematous. Thereafter, the patient noticed further decline in vision. On March 3, 1986, her visual acuity had decreased to R.E.: 20/400 and L.E.: 20/50. There was marked edema of the right disk, but the left disk appeared normal. Her erythrocyte sedimentation rate was 39 mm/ hour, results of a complete blood cell count were normal, and a temporal artery biopsy showed only arteriosclerosis. She was treated with 60 mg of prednisone daily for one month, but vision continued to decline in the left eye. On March 11, 1986, visual acuity in the left eye was 20/70 and both disks were edematous. Results of spinal fluid examination and com puted tomographic scan of the head and orbits with contrast injection were normal. On April 2, 1986, visual acuity in the left eye was 20/100, and bilateral dyschromatopsia and inferior altitudinal visual field defects were noted. The right disk was atrophie and the left disk was slightly swollen except for a zone of atrophy superiorly. Case 9 On March 12, 1981, a 61-year-old man whose medical history had been notable only for a right acoustic neuroma noticed a "film" over the lower half of the field of his left eye. He immediately consulted an ophthalmologist who found that his visual acuity was R.E.: 20/30 and L.E.: 20/40. The left disk was swollen. Fluorescein angiography showed only the disk swelling. Results of orbital ultrasound were normal. He was then well until April 19, 1981, when he lost more of his visual field over a period of several hours. Reexamination two days later showed that his visual acuity was R.E.: 20/30
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and L.E.: 20/200. The left eye had dyschroma topsia, an afferent pupillary defect, and an inferior altitudinal visual field defect. His left disk was more swollen than it was five weeks before and peripapillary hemorrhages were present. His erythrocyte sedimentation rate was 27 mm/hour. A computed tomographic scan of the head and orbits with contrast injection showed only changes related to his resected acoustic neuroma. The patient's visual function remained unchanged for the next six months, but optic atrophy supervened in the left eye. Case 10 In 1977, a 70-year-old man with treated hy pertension sustained sudden, painless, perma nent loss of vision in his left eye. Other details of that illness are unavailable. He was other wise well until June 7, 1987, when vision in his right eye suddenly blurred. His ophthalmolo gist recorded a visual acuity of R.E.: 8/200 and L.E.: light perception. There was an inferior altitudinal visual field defect involving fixation. The right disk was edematous without hemor rhages and the retinal vessels were attenuated. The left disk was atrophie and there was a hole in the macula. He had no symptoms of giant cell arteritis, but his erythrocyte sedimentation rate was 42 mm/hour. Biopsy of his temporal artery showed normal tissue. Results of a com plete blood cell count, serologie test for syphi lis, serum protein electrophoresis, computed tomographic scan of his head and orbits with contrast injection, and noninvasive carotid tests were normal. After a two-week regimen of high-dose oral prednisone, his vision improved slightly, stabilizing at 20/100 in the right eye. However, on Feb. 7, 1988, he suddenly suffered further loss of vision in his right eye. At that time visual acuity was barely counting fingers in the superotemporal portion of his field and there was new disk edema inferiorly. His erythrocyte sedimentation rate was 25 mm/ hour and he had no systemic symptoms. The patient declined treatment or additional exami nation.
Discussion Nine patients had either a recurrence in the same eye after a long interval (four patients) or progression over a month or longer in one eye (five patients). The tenth patient, a man with
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bilateral progression, also suffered a recurrence in one eye. While the recurrence or progression was often limited to further field loss, five eyes suffered reduction in Snellen acuity. In each instance, the diagnosis of anterior ischemie optic neuropathy appears to have been appropriate. Although there is no means of proving the diagnosis, the setting (a middle aged or elderly patient), mode of onset (sudden loss of visual acuity or field), presence of disk edema, failure to recover, and lack of evidence of an alternative diagnosis on laboratory tests or follow-up make any other diagnosis unlike ly. None of the patients had symptoms of giant cell arteritis. Temporal artery biopsies showed no evidence of arteritis in six of the patients. The other four patients who did not undergo biopsy had normal erythrocyte sedimentation rates. While a normal erythrocyte sedimenta tion rate does not rule out giant cell arteritis, it makes the diagnosis unlikely. Of the four pa tients, one was only 50 years old and he re tained a visual acuity of 20/50 without corticosteroid treatment. The other three patients, who were also untreated, did not develop ante rior ischemie optic neuropathy in the second eye. This provides further evidence against an arteritic origin.
was available. Most of these cases were said to have occurred within nine days. Knox and Duke 7 described the pathologic findings in a patient with slow progression of visual loss but did not provide complete clinical information. Since progressive anterior ischemie optic neu ropathy appears to be rare, the diagnosis should be reserved for those patients in whom other causes of progressive optic neuropathy have been ruled out. The pathogenesis of arteritic anterior ische mie optic neuropathy is generally accepted as the result of occlusion of the posterior ciliary arteries. The pathogenesis of the nonarteritic variety has not been as firmly established, but a posterior ciliary vaso-occlusive process is pos tulated as the initial event. 8 This is supported by the finding that nonarteritic anterior ische mie optic neuropathy tends to occur in the age group most susceptible to the manifestations of atherosclerotic vascular disease, and fluorescein angiography has shown delayed perfusion of the peripapillary choroid in this disease. 8,9 Several studies have implicated the structure of the disk in the pathogenesis, establishing the association of a small optic cup with non arteritic anterior ischemie optic neuropa thy. 1013
Many ophthalmologists reassure their pa tients with idiopathic anterior ischemie optic neuropathy that there is little or no risk of progression in the same eye. Indeed, there are remarkably few reports of recurrence. Beck and associates 2 described four cases of recurrent anterior ischemie optic neuropathy in the same eye, three of which recurred within three weeks. The other recurred after 45 months. Three of 83 patients with idiopathic anterior ischemie optic neuropathy followed up for a mean of five years by Repka and colleagues 3 demonstrated evidence of recurrence in the same eye manifested by further visual field loss. Ellenberger, Keltner, and Bürde 4 noted recurrence in both eyes of one patient, but the intervals between the initial attacks and recur rences were not reported. Two other cases have been described with recurrent attacks of ante rior ischemie optic neuropathy after intervals of two and three years. 5,6 Based on the number of previous reports, prolonged progression seems rarer than recur rence of anterior ischemie optic neuropathy. Boghen and Glaser 5 found evidence of progres sion for up to four weeks in 11 of 39 patients with nonarteritic anterior ischemie optic neu ropathy in whom information on time course
It has been suggested that crowding of the nerve fibers in the scierai canal predisposes certain optic disks to anterior ischemie optic neuropathy by allowing swollen axons to fur ther compromise blood flow in the capillaries of the nerve head. 13 Thus, what would have been a subclinical event of minimal axonal swelling from occlusion of one or more posterior ciliary artery branches becomes instead a symptomat ic episode of massive ischemia of the disk. This event is apoplectic in nature because the ische mia from progressive axonal swelling must reach a critical level to become clinically detect able, or even noticeable, by the patient. That disk edema is sometimes noted before the loss of vision, as in Case 4, argues for this theory. 8 Although it has been assumed that the ische mia and functional loss stop abruptly, our cases indicate that they may not. An astute patient, or a monocular patient, may notice progression of field loss over days to months. Six of our patients were essentially monocular when they developed progressive or recurrent anterior ischemie optic neuropathy. Physicians may overlook this progression as attention is turned to the better eye in what is widely viewed as a completed ischemie event. Beck and associates 2 suggested that recur-
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rences of anterior ischemie optic neuropathy in the same eye may be uncommon because the blood supply from the infarcted area of the disk may be shunted to the remainder of the disk to provide protection. Quantitation of vessels and blood flow in experimental optic atrophy does not support this theory. 14 · 15 Perhaps relief of the crowding in the prelaminar or intralaminar por tion of the optic nerve from atrophy of the infarcted axons may account for this phenome non. If this were the case, patients with relative sparing of axons from the initial event would be the most susceptible to recurrent attacks. Slow progression or recurrences of anterior ischemie optic neuropathy could continue until suffi cient axons were lost to relieve the crowding. Our cases support this theory in that all pa tients had relatively spared visual acuity and fields at the time of their progression or recur rence. In the series reported by Beck and asso ciates, 2 the worst visual acuity was 20/40 before the recurrent episode. The visual loss before recurrence in other reports cannot be deter mined. We suspect that the incidence of recurrence and progression of anterior ischemie optic neu ropathy in the same eye may be higher than is generally appreciated. Prospective evaluation of patients with this problem, including serial visual acuity and visual field measurements, may prove this to be the case and lead to a better understanding of the pathogenesis of this disease.
References 1. Miller, N. R.: Clinical Neuro-Ophthalmology, ed. 4. Baltimore, Williams & Wilkins, 1982, pp. 213-216. 2. Beck, R. W., Savino, P. J., Repka, M. X., Schatz, N. J., and Sergott, R. C : Optic disc structure in
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anterior ischemie optic neuropathy. Ophthalmology 91:1334, 1984. 3. Repka, M. X., Savino, P. J., Schatz, N. J., and Sergott, R. C. : Clinical profile and long-term implica tions of anterior ischemie optic neuropathy. Am. J. Ophthalmol. 96:478, 1983. 4. Ellenberger, C , Keltner, J. L., and Bürde, R. M.: Acute optic neuropathy in older patients. Arch. Neurol. 28:182, 1973. 5. Boghen, D. R., and Glaser, J. S.: Ischaemic optic neuropathy. The clinical profile and natural history. Brain 98:689, 1975. 6. Smith, J. L., and Goldhammer, Y.: Hypertensive optic neuropathy. Trans. Am. Acad. Ophthalmol. Otolaryngol. 79:520, 1975. 7. Knox, D. L., and Duke, J. R.: Slowly progressive ischemie optic neuropathy. A clinicopathologic case report. Trans. Am. Acad. Ophthalmol. Otolaryngol. 75:1065, 1971. 8. Hayreh, S. S.: Anterior Ischemie Optic Neuropa thy. New York, Springer-Verlag, 1975, pp. 12-22. 9. : Anterior ischaemic optic neuropathy. II. Fundus on ophthalmoscopy and fluorescein angiography. Br. J. Ophthalmol. 58:964, 1974. 10. Beck, R. W., Savino, P. J., Schatz, N. J., Smith, C. H., and Sergott, R.: Anterior ischaemic optic neuropathy. Recurrent episodes in the same eye. Br. J. Ophthalmol. 67:705, 1983. 11. Feit, R. H., Tomsak, R. L., and Ellenberger, C : Structural factors in the pathogenesis of ischemie optic neuropathy. Am. J. Ophthalmol. 98:105, 1984. 12. Doro, S., and Lesseil, S.: Cup-disc ratio and ischemie optic neuropathy. Arch. Ophthalmol. 103:1143, 1985. 13. Beck, R. W., Servais, G. E., and Hayreh, S. S.: Anterior ischemie optic neuropathy. IX. Cup-to-disc ratio and its role in pathogenesis. Ophthalmology 94:1503, 1987. 14. Quigley, H. A., Hohman, B. A., and Addicks, E. M.: Quantitative study of optic nerve head capil laries in experimental optic disk pallor. Am. J. Oph thalmol. 93:689, 1982. 15. Sebag, J., Feke, G. T., Delori, F. C , and Weiter, J. J.: Anterior optic nerve blood flow in experimental optic atrophy. Invest. Ophthalmol. Vis. Sci. 26:1415, 1985.
We examined ten patients with nonarteritic anterior ischemie optic neuropathy in whom the visual deficit progressed over a number of weeks or who suffered a recurrence in the same eye. The visual deficits progressed over a month or more in five patients, four patients suffered a recurrence in the same eye after a long interval, and one patient had bilateral progression and a recurrence. NONARTERITIC ANTERIOR ischemie optic neu ropathy characteristically causes precipitous visual loss. The visual deficit is either maximal when first noticed or evolves over hours to days. Progression rarely occurs over weeks. 1 After an interval, the fellow eye is commonly involved but recurrences in the same eye are said to be uncommon. 1 We examined ten pa tients with nonarteritic anterior ischemie optic neuropathy w h o suffered recurrences in the same eye or progression over intervals ranging from weeks to months.
Case Reports Case 1 A 68-year-old man was well except for a maculopathy of his right eye that had not af fected his vision. On Jan. 12, 1985, he suddenly lost part of the lower visual field of his left eye. There was no associated pain or other symp toms. When examined 48 hours later, his visual acuity was 20/15 in each eye and he correctly identified all Ishihara color test plates. The left eye had a relative afferent pupillary defect, segmentai disk edema, and an inferior altitudiAccepted for publication June 3, 1988. From the Department of Ophthalmology, Massachu setts Eye & Ear Infirmary, Boston. Reprint requests to Simmons Lessell, M.D., Depart ment of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles St., Boston, MA 02114.
nal visual field defect. Over the next five days, the visual field defect gradually enlarged. Re sults of a neurologic examination, computed tomographic scan of the head with contrast injection, and an erythrocyte sedimentation rate were all normal. Three weeks of oral highdose prednisone treatment did not appear to influence his course, and the visual field defect slowly progressed over the next three months. The defect extended into the upper half of the field and involved fixation (Fig. 1). By April 30, 1985, visual acuity in the left eye was 20/200 and there was a profound dyschromatopsia. Visual function in his left eye has remained unchanged since that time. On Sept. 29, 1985, the patient experienced a ten-minute episode of pulse-synchronous flashing lights, with obscuration of vision on right gaze. Findings on examination the follow ing day were unchanged from five months earlier. Results of oculoplethysmography were normal. On Nov. 29, 1985, the vision in his right eye suddenly became impaired. Visual acuity was correctable to 20/20 and color vision was unim paired, but there was an inferior altitudinal visual field defect and sectoral disk edema. He was hospitalized and treated with nifedipine, high-dose prednisone, acetazolamide, topical timolol, and oxygen inhalation. Despite this regimen, his visual loss progressed over the next month. His disorder then stabilized until July 22, 1986, when he suddenly lost more of his right visual field (Fig. 2). At that time, results of a temporal artery biopsy, cerebrospinal fluid examination, serologie test for syphi lis, serum protein and lipoprotein electrophoresis, chest x-ray, serum angiotensin converting enzyme level, tuberculin skin test, and complete blood cell count were normal. Results of repeat head and orbit computed tomography were unchanged. During the next 18 months he lost two more areas of his field, but his visual acuity remained 20/20.
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Case 2 A 50-year-old man with insulin-dependent diabetes mellitus for two years had injured his right eye in childhood, which reduced visual acuity in that eye to counting fingers. Five days after a routine ophthalmologic examination, in which visual acuity in his left eye was 20/20, he suddenly and painlessly lost the inferior visual
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field of that eye. Reexamination showed that visual acuity was 20/50 and the upper half of the left disk was edema tous. Visual field exami nation demonstrated an inferior altitudinal de fect (Fig. 3). Results of a complete blood cell count, erythrocyte sedimentation rate, serolog ie test for syphilis, antinuclear antibody titer, and serum angiotensin converting enzyme
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Fig. 3 (Borchert and Lessell). Case 2, left eye. Left, Goldmann visual field at time of initial visual loss. Right, Goldmann visual field after progression of visual loss one month later.
level were normal. His field loss was stable for one week, but then slowly worsened over the next month, leaving him with a small island of central vision in his left eye (Fig. 3). Visual acuity remained 20/50. His left disk was pale superiorly and edematous inferiorly. Results of cytologie cerebrospinal fluid stud ies, chest x-ray, computed tomographic scan of the head and orbits with contrast injection, and tuberculin skin test were all normal. Case 3 A 57-year-old healthy man consulted his ophthalmologist because he had seen flashing light for four days and had blurred vision for two days in his left eye. Visual acuity was 20/20 in each eye and results of color vision testing were normal. However, there was a left superonasal visual field defect and the inferior portion of the left disk was edematous. One month later, his vision again suddenly blurred. Visual acuity decreased to 20/50 and there was a central visual field defect. There was dyschromatopsia and a left afferent pupillary defect. The left disk was diffusely swollen. Results of an erythrocyte sedimentation rate were normal and a temporal artery biopsy showed normal tissue. Three months later, the patient realized that he had lost more of his visual field. Results of perimetry confirmed his impression. The left disk was still diffusely edematous. Results of a computed tomographic scan of the head and orbits with contrast injection, erythrocyte sedi mentation rate, antinuclear antibody titer, ser
ologie test for syphilis, and serum protein electrophoresis were normal. The patient refused a lumbar puncture. His findings in the left eye have remained unchanged. Ten months after the condition of his left eye stabilized, he suddenly lost the lower half of the field of his right eye. Examination showed that visual acuity was 20/20 and results of color vision testing were normal, but he had an inferior altitudinal visual field defect and edema of the superonasal segment of the disk. One week later, visual acuity in the right eye decreased to counting fingers and the disk swelling had become more extensive. Vision never recovered. Case 4 A 74-year-old man with treated hypertension and atrial fibrillation suffered sudden, painless loss of vision in his left eye in June 1985. His ophthalmologist diagnosed nonarteritic ante rior ischemie optic neuropathy. Visual acuity was 20/30, and he had an inferior altitudinal visual field defect and optic atrophy. Findings in the right eye were unremarkable. He was then well until Sept. 22, 1986, when sectoral edema of the right optic disk was noted on a routine ophthalmologic examination. Vis ual acuity in the right eye was 20/20 and the visual field was full. He then described a slowly progressive "break-up" of the upper half of the visual field of his right eye. When his condition stabilized three months later, visual acuity was 20/40, there was a dense, extensive upper alti tudinal visual field defect, and his disk was
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atrophie. Results of a temporal artery biopsy, erythrocyte sedimentation rate, and computed tomographic scan of the head and orbits with contrast injection were normal. There have been no subsequent changes. Case5 A 65-year-old woman with treated endometrial carcinoma suddenly lost the superonasal quadrant of her left visual field in September 1975. Visual acuity was R.E.: 20/25 and L.E.: 20/30. Perimetry demonstrated a superonasal step in her left eye. The left disk was swollen inferiorly and there was a peripapillary hemor rhage. Results of an erythrocyte sedimentation rate were norma}. The patient, a physician, checked her central visual field every other day with an Amsler grid. No change was noted until April 1976, at which time she found that her field loss had increased (Fig. 4). On reexamination, visual acuity was still 20/20 but there was edema of the left disk, especially superiorly. Results of a repeat erythrocyte sedimentation rate were normal. Her vision has remained unchanged and her general health has been good over the ensuing 12 years. Case 6 A healthy 66-year-old man lost vision in his left eye over a one-week period. When vision started to fail in his right eye 5Vi weeks later, he
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October, 1988
consulted an ophthalmologist. He had a life long exotropia and a visual acuity of R.E.: 20/25 and L.E.: 20/20 had previously been recorded. On examination, visual acuity was R.E.: 20/40 and L.E.: light perception. A scotoma was noted superotemporal to fixation when the pa tient viewed an Amsler grid, but no defect could be detected on a tangent screen in the right eye. There was a left afferent pupillary defect. The right disk was edematous and the left was atrophie. His erythrocyte sedimenta tion rate was 11 mm/hour and temporal artery biopsy yielded normal tissue. Over the next two weeks there was progres sive loss of the superior portion of the visual field of the right eye. Reexamination showed no change in visual acuity, but there was a dense, extensive upper altitudinal field defect in the right eye. No change has occurred in the subse quent three years. Case 7 A 55-year-old healthy man was examined by his ophthalmologist on April 9, 1977, because the inferior field of his right eye had been blurred for two weeks. Visual acuity was R.E.: 20/20-2 and L.E.: 20/20. There was a partial inferior altitudinal field defect of variable den sity in the right eye. The right disk was swol len, with peripapillary hemorrhages. His erythrocyte sedimentation rate was 4 mm/hour and a computed tomographic scan of the head
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Fig. 4 (Borchert and Lessell). Case 5, left eye. Left, initial examination. Right, Amsler grid pattern se grayness.
>ler grid pattern defining area of "grayness" at time of months later demonstrating progression of area of
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and orbits with contrast injection showed no abnormalities. Thereafter, he suffered further progressive loss of vision in his right eye. By April 14, 1977, visual acuity had decreased to 20/100. Two weeks later, it was counting fingers at 2 feet. He was left with optic atrophy and a central scotoma, although visual acuity improved to 13/200 after several weeks. Case 8 On Feb. 11, 1986, a healthy 65-year-old woman suddenly noticed a "film" over the lower part of the visual field of her right eye. There were no symptoms of giant cell arteritis. Examination two days later disclosed a visual acuity of 20/20 in each eye and no pupillary abnormality. The upper half of the right disk was edematous. Thereafter, the patient noticed further decline in vision. On March 3, 1986, her visual acuity had decreased to R.E.: 20/400 and L.E.: 20/50. There was marked edema of the right disk, but the left disk appeared normal. Her erythrocyte sedimentation rate was 39 mm/ hour, results of a complete blood cell count were normal, and a temporal artery biopsy showed only arteriosclerosis. She was treated with 60 mg of prednisone daily for one month, but vision continued to decline in the left eye. On March 11, 1986, visual acuity in the left eye was 20/70 and both disks were edematous. Results of spinal fluid examination and com puted tomographic scan of the head and orbits with contrast injection were normal. On April 2, 1986, visual acuity in the left eye was 20/100, and bilateral dyschromatopsia and inferior altitudinal visual field defects were noted. The right disk was atrophie and the left disk was slightly swollen except for a zone of atrophy superiorly. Case 9 On March 12, 1981, a 61-year-old man whose medical history had been notable only for a right acoustic neuroma noticed a "film" over the lower half of the field of his left eye. He immediately consulted an ophthalmologist who found that his visual acuity was R.E.: 20/30 and L.E.: 20/40. The left disk was swollen. Fluorescein angiography showed only the disk swelling. Results of orbital ultrasound were normal. He was then well until April 19, 1981, when he lost more of his visual field over a period of several hours. Reexamination two days later showed that his visual acuity was R.E.: 20/30
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and L.E.: 20/200. The left eye had dyschroma topsia, an afferent pupillary defect, and an inferior altitudinal visual field defect. His left disk was more swollen than it was five weeks before and peripapillary hemorrhages were present. His erythrocyte sedimentation rate was 27 mm/hour. A computed tomographic scan of the head and orbits with contrast injection showed only changes related to his resected acoustic neuroma. The patient's visual function remained unchanged for the next six months, but optic atrophy supervened in the left eye. Case 10 In 1977, a 70-year-old man with treated hy pertension sustained sudden, painless, perma nent loss of vision in his left eye. Other details of that illness are unavailable. He was other wise well until June 7, 1987, when vision in his right eye suddenly blurred. His ophthalmolo gist recorded a visual acuity of R.E.: 8/200 and L.E.: light perception. There was an inferior altitudinal visual field defect involving fixation. The right disk was edematous without hemor rhages and the retinal vessels were attenuated. The left disk was atrophie and there was a hole in the macula. He had no symptoms of giant cell arteritis, but his erythrocyte sedimentation rate was 42 mm/hour. Biopsy of his temporal artery showed normal tissue. Results of a com plete blood cell count, serologie test for syphi lis, serum protein electrophoresis, computed tomographic scan of his head and orbits with contrast injection, and noninvasive carotid tests were normal. After a two-week regimen of high-dose oral prednisone, his vision improved slightly, stabilizing at 20/100 in the right eye. However, on Feb. 7, 1988, he suddenly suffered further loss of vision in his right eye. At that time visual acuity was barely counting fingers in the superotemporal portion of his field and there was new disk edema inferiorly. His erythrocyte sedimentation rate was 25 mm/ hour and he had no systemic symptoms. The patient declined treatment or additional exami nation.
Discussion Nine patients had either a recurrence in the same eye after a long interval (four patients) or progression over a month or longer in one eye (five patients). The tenth patient, a man with
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bilateral progression, also suffered a recurrence in one eye. While the recurrence or progression was often limited to further field loss, five eyes suffered reduction in Snellen acuity. In each instance, the diagnosis of anterior ischemie optic neuropathy appears to have been appropriate. Although there is no means of proving the diagnosis, the setting (a middle aged or elderly patient), mode of onset (sudden loss of visual acuity or field), presence of disk edema, failure to recover, and lack of evidence of an alternative diagnosis on laboratory tests or follow-up make any other diagnosis unlike ly. None of the patients had symptoms of giant cell arteritis. Temporal artery biopsies showed no evidence of arteritis in six of the patients. The other four patients who did not undergo biopsy had normal erythrocyte sedimentation rates. While a normal erythrocyte sedimenta tion rate does not rule out giant cell arteritis, it makes the diagnosis unlikely. Of the four pa tients, one was only 50 years old and he re tained a visual acuity of 20/50 without corticosteroid treatment. The other three patients, who were also untreated, did not develop ante rior ischemie optic neuropathy in the second eye. This provides further evidence against an arteritic origin.
was available. Most of these cases were said to have occurred within nine days. Knox and Duke 7 described the pathologic findings in a patient with slow progression of visual loss but did not provide complete clinical information. Since progressive anterior ischemie optic neu ropathy appears to be rare, the diagnosis should be reserved for those patients in whom other causes of progressive optic neuropathy have been ruled out. The pathogenesis of arteritic anterior ische mie optic neuropathy is generally accepted as the result of occlusion of the posterior ciliary arteries. The pathogenesis of the nonarteritic variety has not been as firmly established, but a posterior ciliary vaso-occlusive process is pos tulated as the initial event. 8 This is supported by the finding that nonarteritic anterior ische mie optic neuropathy tends to occur in the age group most susceptible to the manifestations of atherosclerotic vascular disease, and fluorescein angiography has shown delayed perfusion of the peripapillary choroid in this disease. 8,9 Several studies have implicated the structure of the disk in the pathogenesis, establishing the association of a small optic cup with non arteritic anterior ischemie optic neuropa thy. 1013
Many ophthalmologists reassure their pa tients with idiopathic anterior ischemie optic neuropathy that there is little or no risk of progression in the same eye. Indeed, there are remarkably few reports of recurrence. Beck and associates 2 described four cases of recurrent anterior ischemie optic neuropathy in the same eye, three of which recurred within three weeks. The other recurred after 45 months. Three of 83 patients with idiopathic anterior ischemie optic neuropathy followed up for a mean of five years by Repka and colleagues 3 demonstrated evidence of recurrence in the same eye manifested by further visual field loss. Ellenberger, Keltner, and Bürde 4 noted recurrence in both eyes of one patient, but the intervals between the initial attacks and recur rences were not reported. Two other cases have been described with recurrent attacks of ante rior ischemie optic neuropathy after intervals of two and three years. 5,6 Based on the number of previous reports, prolonged progression seems rarer than recur rence of anterior ischemie optic neuropathy. Boghen and Glaser 5 found evidence of progres sion for up to four weeks in 11 of 39 patients with nonarteritic anterior ischemie optic neu ropathy in whom information on time course
It has been suggested that crowding of the nerve fibers in the scierai canal predisposes certain optic disks to anterior ischemie optic neuropathy by allowing swollen axons to fur ther compromise blood flow in the capillaries of the nerve head. 13 Thus, what would have been a subclinical event of minimal axonal swelling from occlusion of one or more posterior ciliary artery branches becomes instead a symptomat ic episode of massive ischemia of the disk. This event is apoplectic in nature because the ische mia from progressive axonal swelling must reach a critical level to become clinically detect able, or even noticeable, by the patient. That disk edema is sometimes noted before the loss of vision, as in Case 4, argues for this theory. 8 Although it has been assumed that the ische mia and functional loss stop abruptly, our cases indicate that they may not. An astute patient, or a monocular patient, may notice progression of field loss over days to months. Six of our patients were essentially monocular when they developed progressive or recurrent anterior ischemie optic neuropathy. Physicians may overlook this progression as attention is turned to the better eye in what is widely viewed as a completed ischemie event. Beck and associates 2 suggested that recur-
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rences of anterior ischemie optic neuropathy in the same eye may be uncommon because the blood supply from the infarcted area of the disk may be shunted to the remainder of the disk to provide protection. Quantitation of vessels and blood flow in experimental optic atrophy does not support this theory. 14 · 15 Perhaps relief of the crowding in the prelaminar or intralaminar por tion of the optic nerve from atrophy of the infarcted axons may account for this phenome non. If this were the case, patients with relative sparing of axons from the initial event would be the most susceptible to recurrent attacks. Slow progression or recurrences of anterior ischemie optic neuropathy could continue until suffi cient axons were lost to relieve the crowding. Our cases support this theory in that all pa tients had relatively spared visual acuity and fields at the time of their progression or recur rence. In the series reported by Beck and asso ciates, 2 the worst visual acuity was 20/40 before the recurrent episode. The visual loss before recurrence in other reports cannot be deter mined. We suspect that the incidence of recurrence and progression of anterior ischemie optic neu ropathy in the same eye may be higher than is generally appreciated. Prospective evaluation of patients with this problem, including serial visual acuity and visual field measurements, may prove this to be the case and lead to a better understanding of the pathogenesis of this disease.
References 1. Miller, N. R.: Clinical Neuro-Ophthalmology, ed. 4. Baltimore, Williams & Wilkins, 1982, pp. 213-216. 2. Beck, R. W., Savino, P. J., Repka, M. X., Schatz, N. J., and Sergott, R. C : Optic disc structure in
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anterior ischemie optic neuropathy. Ophthalmology 91:1334, 1984. 3. Repka, M. X., Savino, P. J., Schatz, N. J., and Sergott, R. C. : Clinical profile and long-term implica tions of anterior ischemie optic neuropathy. Am. J. Ophthalmol. 96:478, 1983. 4. Ellenberger, C , Keltner, J. L., and Bürde, R. M.: Acute optic neuropathy in older patients. Arch. Neurol. 28:182, 1973. 5. Boghen, D. R., and Glaser, J. S.: Ischaemic optic neuropathy. The clinical profile and natural history. Brain 98:689, 1975. 6. Smith, J. L., and Goldhammer, Y.: Hypertensive optic neuropathy. Trans. Am. Acad. Ophthalmol. Otolaryngol. 79:520, 1975. 7. Knox, D. L., and Duke, J. R.: Slowly progressive ischemie optic neuropathy. A clinicopathologic case report. Trans. Am. Acad. Ophthalmol. Otolaryngol. 75:1065, 1971. 8. Hayreh, S. S.: Anterior Ischemie Optic Neuropa thy. New York, Springer-Verlag, 1975, pp. 12-22. 9. : Anterior ischaemic optic neuropathy. II. Fundus on ophthalmoscopy and fluorescein angiography. Br. J. Ophthalmol. 58:964, 1974. 10. Beck, R. W., Savino, P. J., Schatz, N. J., Smith, C. H., and Sergott, R.: Anterior ischaemic optic neuropathy. Recurrent episodes in the same eye. Br. J. Ophthalmol. 67:705, 1983. 11. Feit, R. H., Tomsak, R. L., and Ellenberger, C : Structural factors in the pathogenesis of ischemie optic neuropathy. Am. J. Ophthalmol. 98:105, 1984. 12. Doro, S., and Lesseil, S.: Cup-disc ratio and ischemie optic neuropathy. Arch. Ophthalmol. 103:1143, 1985. 13. Beck, R. W., Servais, G. E., and Hayreh, S. S.: Anterior ischemie optic neuropathy. IX. Cup-to-disc ratio and its role in pathogenesis. Ophthalmology 94:1503, 1987. 14. Quigley, H. A., Hohman, B. A., and Addicks, E. M.: Quantitative study of optic nerve head capil laries in experimental optic disk pallor. Am. J. Oph thalmol. 93:689, 1982. 15. Sebag, J., Feke, G. T., Delori, F. C , and Weiter, J. J.: Anterior optic nerve blood flow in experimental optic atrophy. Invest. Ophthalmol. Vis. Sci. 26:1415, 1985.