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Purpose A challenging clinical scenario is distinguishing between normal tension glaucoma (NTG) and non-glaucomatous optic neuropathies (NGON). The key to the assessment remains identifying the presence of optic nerve head cupping. Recent optical coherence tomography (OCT) measurements now allow objective assessment of cupping by minimum rim width at Bruch's membrane opening (MRW-BMO). This study assessed the hypothesis that the MRW-BMO measurement quantifies cupping and therefore can differentiate between NTG and NGON. Design Diagnostic evaluation with area under the curve. Methods Setting: multicenter tertiary hospitals and outpatient clinics. Patient population: 81 eyes of 81 patients were enrolled, 27 with NTG and 54 with NGON, including ischemic optic neuropathy, previous optic neuritis, and compressive and inherited optic neuropathies. All NGON patients with intraocular pressure >21 mm Hg, narrow drainage angles, or a family history of glaucoma were excluded. Observational procedure: optic disc OCT images were obtained of both the retinal nerve fiber layer thickness and the MRW-BMO. Main outcome measurements: the utility of the MRW-BMO in differentiating GON from NGON was assessed using the area under the curve (AUC) estimated from a logistic regression model. Results The 5-fold cross-validated AUC for glaucoma versus nonglaucoma from logistic regression models using MRW-BMO values from all sectors was 0.95 (95% confidence interval: 0.86-1.00). Conclusions The measurement of MRW-BMO effectively differentiates between NTG and NGON with a high level of sensitivity and specificity. Incorporating this measurement into routine glaucoma assessment may provide a robust method of assisting clinicians to improve diagnosis and therefore treatment of optic nerve diseases.

To compare macular vasculature in patients with primary open-angle glaucoma (POAG) and atrophic nonarteritic anterior ischemic optic neuropathy (NAION).Prospective, cross-sectional study.Thirty-seven eyes with moderate and advanced POAG, 19 eyes with atrophic NAION, and 40 eyes of normal subjects were imaged using optical coherence tomography angiography (OCT-A). Macular ganglion cell complex (GCC) and peripapillary retinal nerve fiber layer (RNFL) thicknesses were measured in addition to macular superficial and deep vasculature after projection removal using custom software.Linear models showed that while averaged peripapillary RNFL and macular GCC were not different between NAION and POAG eyes, both were significantly thinner than control eyes. Whole image macular superficial vessel density was significantly lower in NAION and glaucoma eyes (P = .003 and.001, respectively) than in normal eyes, with lower vessel density in glaucoma than in NAION eyes (P = .01). Whole image and parafoveal deep macular vessels in glaucoma eyes (21.0%±8.7%, 24.4%±9.6%) were significantly lower than in control eyes (27.4%±8.6%, 31.9%±10.6%) (P = .01 and P = .01, respectively). No significant differences in deep vessels were observed between NAION and control eyes. Glaucomatous eyes had lower temporal and inferior parafoveal deep vasculature values than NAION eyes (P = .007 and .03, respectively).In NAION and POAG with similar RNFL and macular damage, macular OCT-A shows less involvement of superficial and deep vascular plexus in NAION in contrast to POAG, which might show a primary vascular insult in addition to secondary vascular damage due to ganglion cell damage.

PURPOSE: To report anterior ischemic optic neuropathy associated with systemic hypotension in a patient undergoing continuous ambulatory peritoneal dialysis. METHODS: Case report. A 58-year-old man undergoing continuous ambulatory peritoneal dialysis developed painless blurred vision in both eyes and bilateral optic disk swelling with an altitudinal field defect in the left eye. Twenty-four-hour ambulatory blood pressure monitoring was requested in addition to other routine investigations. RESULTS: Routine blood pressure measurement in the clinic was 130/86 mm Hg, but ambulatory blood pressure monitoring demonstrated pronounced early morning hypotension with individual readings as low as 91/41 mm Hg. CONCLUSIONS: Renal dialysis can render patients hypotensive, and this may be associated with anterior ischemic optic neuropathy. The overnight drop in blood pressure may not be appreciated with routine blood pressure measurement. Therefore, 24-hour ambulatory blood pressure monitoring should be considered when investigating patients with suspected anterior ischemic optic neuropathy who are undergoing renal replacement.

PURPOSE: To report clinical features of amiodarone-induced optic neuropathy and outline the differentiation of amiodarone optic neuropathy from nonarteritic anterior ischemic optic neuropathy. METHOD: We reviewed data from 73 patients reported to have developed an optic neuropathy while taking amiodarone. RESULTS: Amiodarone optic neuropathy is characterized by an insidious onset, slow progression, bilateral visual loss, and protracted disk swelling that tends to stabilize within several months of discontinuing the medication. Nonarteritic ischemic optic neuropathy is characterized by acute, unilateral visual loss that is usually complete at onset, with resolution of disk edema over several weeks. CONCLUSION: Unique clinical features of amiodarone-induced optic neuropathy may help clinicians diagnose and distinguish between amiodarone-induced optic neuropathy and nonarteritic anterior ischemic optic neuropathy.

PURPOSE: To report a case of sequential anterior ischemic optic neuropathy temporally related to the sequential use of a decongestant nasal spray. METHODS: A 43-year-old woman was evaluated for sequential bilateral vision loss following the use of oxymetazoline nasal spray. RESULTS: Thorough investigation of possible etiologies causing the bilateral anterior ischemic optic neuropathy was negative. Our patient had several predisposing risk factors for ischemic optic neuropathy, including diabetes mellitus, hypertension, and crowded optic nerve heads. Before each acute optic neuropathy, the patient used two to three puffs of oxymetazoline nasal spray. CONCLUSION: Decongestants have been associated with ischemic and hemorrhagic vascular events. Vasoconstriction secondary to oxymetazoline use may precipitate anterior ischemic optic neuropathy.

Purpose To determine the feasibility of a case-control study of phosphodiesterase (PDE)-5 inhibitors and nonarteritic ischemic optic neuropathy (NION). Design Retrospective cohort study using the National Veterans Health Administration's pharmacy and clinical databases. Methods setting: National database. patient population: Male veterans 50 years of age and older with newly diagnosed NION or "possible" NION. interventional procedure: Dispensed prescription for a PDE-5 inhibitor. outcome measure: Incidence of NION and "possible" NION based on exposure to PDE-5 inhibitors. Results The relative risk of NION among men prescribed a PDE-5 inhibitor was 1.02 (95% confidence interval [CI]: 0.92 to 1.12). The relative risk of possible NION among men prescribed a PDE-5 inhibitor was 1.34 (95% CI: 1.17 to 1.55). Conclusions The collective risk of NION and possible NION was marginally significant (relative risk [RR] 1.10 95% CI: 1.02 to 1.20). A case-control study, in which the dependent variable is NION and the effect size of the PDE-5 inhibitor is assumed small, is feasible.

Purpose To report four cases of optic nerve neuropathy in three children treated with periocular carboplatin injections for unilateral or bilateral intraocular retinoblastoma. Design Retrospective, observational case series. Methods setting : University-based Ophthalmology Practice. study population : Four eyes of three children with retinoblastoma enucleated after nonsuccessful multimodality treatment including periocular carboplatin injections. observation procedures : The enucleated eyes were routinely processed and evaluated by light microscopy. A retrospective chart review of all four cases was performed. Results Three enucleated eyes (Reese-Ellsworth groups III and VB) were obtained from two children with bilateral multifocal retinoblastoma, and one eye (Reese-Ellsworth group IIB) was harvested from a child with unilateral retinoblastoma. All affected eyes underwent three to seven periocular carboplatin injections before enucleation. Additional treatment modalities included systemic chemotherapy, transpupillary thermotherapy, transscleral cryotherapy, and external beam radiotherapy. Histopathologic evaluation of the enucleated eyes revealed focal areas of ischemic necrosis or atrophy of the optic nerve along with dystrophic calcification and mild inflammation in the surrounding fibrovascular adipose tissue. Conclusions Periocular injections of carboplatin may be a useful treatment approach in the management of patients with advanced intraocular retinoblastoma and may minimize systemic side-effects. However, ophthalmologists and pediatric oncologists should be aware of potential marked local complications with periocular carboplatin delivery, including ischemic optic neuropathy. Modifying the injection site/location (for example, subtenon space) or adding other delivery routes adjuncts (for example, fibrin sealant) deserves further study.

Purpose To report a patient with Takayasu disease with severe proliferative retinopathy progressing to bilateral blindness. A 57-year-old Japanese woman suffering from Takayasu disease for 16 years manifested severe proliferative retinopathy in both eyes, leading to blindness due to optic atrophy. Design Interventional case report. Methods A 57-year-old Japanese woman suffering from Takayasu disease for 16 years underwent a comprehensive opthalmologic examination. Panretinal photocoagulation and vitrectomy were performed on both eyes. Settings Institutional practice. Results At initial examination, the patient was found to have severe proliferative retinopathy with tractional retinal detachment in both eyes. The retinal detachments were repaired by the vitrectomy, however, the patient developed bilateral blindness due to optic atrophy. Conclusions Patients with a long history of Takayasu disease can demonstrate severe proliferative retinopathy that may be resistant to photocoagulation and/or vitreous surgery. Ocular ischemia can lead to blindness from ischemic optic neuropathy.

PURPOSE: To document clinical features of uveitis in patients with familial juvenile systemic granulomatosis. DESIGN: Retrospective chart review. METHODS: Ophthalmologic examination, medical history, and clinical course in 16 patients from eight families examined at six academic medical centers. RESULTS: Of the 16 patients, 15 had evidence of panuveitis with multifocal choroiditis. One patient had only an anterior uveitis. Ischemic optic neuropathy, presumably due to a small vessel vasculopathy, and retinal vasculopathy each occurred in one patient. Ocular complications were common, including cataracts in 11, glaucoma in six, band keratopathy in six, cystoid macular edema in six, and optic disk edema in six. All 16 patients had polyarthritis, and at least nine had skin rash. Often patients were misdiagnosed initially as having either juvenile rheumatoid arthritis or sarcoidosis. CONCLUSIONS: Familial juvenile systemic granulomatosis is an uncommon genetic disease characterized by polyarthritis and uveitis. Panuveitis and multifocal choroiditis often may be present. Patients with a diagnosis of juvenile rheumatoid arthritis but having a family history of the disease and multifocal choroiditis should be suspected of having familial juvenile systemic granulomatosis.

PURPOSE: To examine the prevalence and incidence of second eye nonarteritic anterior ischemic optic neuropathy (NAION) and associated patient characteristics in patients enrolled in the Ischemic Optic Neuropathy Decompression Trial (IONDT) Follow-up Study. DESIGN: Randomized clinical trial with observational cohort. METHODS: Patients randomized to optic nerve sheath decompression surgery or careful follow-up had a diagnosis of acute unilateral NAION, visual acuity between 20/64 and light perception, and were aged 50 years or older. Eligible patients who declined randomization or whose visual acuity was better than 20/64 were not randomized but followed as part of an observational cohort. Follow-up examinations took place at 3, 6, 12, 18, and 24 months and annually thereafter. RESULTS: Four hundred eighteen patients were enrolled; 258 randomized and 160 observed. Previous NAION or other optic neuropathy was present in the fellow eye of 21.1% (88/418) of patients at baseline. Four patients developed optic neuropathy in the fellow eye at follow up that could not be conclusively diagnosed as NAION. New NAION in the fellow eye occurred in 14.7% (48/326) of patients at risk during a median follow up of 5.1 years. Randomized patients experienced a higher incidence (35/201; 17.4%) than nonrandomized patients (13/125; 10.4%). A history of diabetes and baseline visual acuity of 20/200 or worse in the study eye, but not age, sex, aspirin use, or smoking were significantly associated with new NAION in the fellow eye. Final fellow eye visual acuity was significantly worse in those patients with new fellow eye NAION whose baseline study eye visual acuity was 20/200 or worse. CONCLUSIONS: Follow-up data from the IONDT cohort provide evidence that the incidence of fellow eye NAION is lower than expected: new NAION was diagnosed in 14.7% of IONDT patients over approximately 5 years. Increased incidence is associated with poor baseline visual acuity in the study eye and diabetes, but not age, sex, smoking history, or aspirin use.

PURPOSE: To report nonarteric anterior ischemic optic neuropathy (NAION) as an ocular manifestation in a woman with combined primary antiphospholipid syndrome and Factor V Leiden (FVL) mutation. METHODS: Case report of a middle-aged woman with hematological investigations confirming the diagnosis of both primary antiphospholipid syndrome and Factor V Leiden mutation, who presented with visual disturbance in her left eye. RESULTS: NAION was noted in her left eye. The patient was promptly treated with low molecular weight heparin, followed by warfarin, which resulted in the reversal of the ischemic optic neuropathy. CONCLUSIONS: Primary antiphospholipid syndrome and coexisting Factor V Leiden mutation should be considered in the differential diagnosis of NAION. Prompt treatment with anticoagulants can result in the reversal of the ischemic process.

PURPOSE: To report the ophthalmologic symptoms and signs associated with extracranial internal carotid artery dissection. METHODS: One hundred forty-six consecutive patients with extracranial internal carotid artery dissection were evaluted; 29 were studied retrospectively from 1972 to 1984 and 117 prospectively from 1985 to 1997. RESULTS: Sixty-two percent of patients (91/146) with extracranial internal carotid artery dissection had ophthalmologic symptoms or signs that were the presenting symptoms or signs of dissection in 52% (76/146). Forty-four percent (65/146) had painful Horner syndrome, which remained isolated in half the cases (32/65). Twenty-eight percent (41/146) had transient monocular visual loss, which was painful in 31 cases, associated with Horner syndrome in 13 cases, and described as “scintillations” or “flashing lights”—often related to postural changes or exposure to bright lights—suggesting acute choroidal hypoperfusion in 23 cases. Four patients had ischemic optic neuropathy; one had diplopia. Among the 76 patients with ophthalmologic symptoms or signs as the presenting features of carotid dissection, a nonreversible ocular or hemispheric stroke later occurred in 27, within a mean of 6.2 days (range, 1 hour to 31 days). Eighteen patients had a stroke within the first week after the onset of neuro-ophthalmic symptoms and signs, and 24 had a stroke within the first 2 weeks. CONCLUSION: Ophthalmologic symptoms or signs are frequently associated with and are often the presenting features in internal carotid artery dissection. Painful Horner syndrome or transient monocular visual loss should prompt investigations to diagnose carotid artery dissection and begin early treatment to prevent a devastating ocular or hemispheric stroke.

To investigate through the use of cerebral Doppler technology whether emboli are a more common cause of nonarteritic anterior ischemic optic neuropathy (NAION) than previously recognized.Eleven patients with a recent (121 days) history of nonarteritic anterior ischemic optic neuropathy and 10 age-matched controls (event121 days) were examined using a Nicolet Pioneer 2020 transcranial Doppler (TCD) unit with a 2-MHz bilateral continuous monitoring capability. The right and left middle cerebral arteries were evaluated simultaneously for 30 minutes at a depth of 50 to 55 mm, and the number of emboli, blood flow velocities, and pulsatility indices were recorded. Data were stored by computer, and hard-copy color recordings were made.None of 11 patients with a recent history of NAION demonstrated microemboli by TCD examination. One patient in the control group who had a remote history of NAION had a microembolic event rate of 12 per hour (six over 30 minutes). This patient had a history of prosthetic cardiac valve replacement and was taking anticoagulation medication at the time of the examination.Our study did not reveal an increased incidence of embolic events in patients with NAION when they were examined in a transcranial Doppler study of the middle cerebral arteries. This study does not support embolism as a frequent cause of NAION.

Purpose Nonarteritic anterior ischemic optic neuropathy is the most common acute optic nerve disease of adults over age 50 years. This study determined the incidence of acute nonarteritic anterior ischemic optic neuropathy in the circumscribed population of Olmsted County, Minnesota. Methods This was a retrospective study of the incidence of acute nonarteritic anterior ischemic optic neuropathy between 1981 and 1990. The Rochester Epidemiology Project medical records linkage system facilitates identification of the medical records of virtually all Olmsted County residents with a given diagnosis. All cases of acute nonarteritic anterior ischemic optic neuropathy that fulfilled certain inclusion and exclusion criteria were identified. Results Twenty-two cases in 21 patients (11 men and 10 women) were recorded. The crude annual incidence rate was 10.3 per 100,000 individuals (95% confidence interval [CI] = 5.1 to 18.4). When adjusted to the age and sex distribution of the 1990 United States white population, the incidence rate was 10.2 per 100,000 (95% CI=6.5 to 15.6). At diagnosis, the median age was 72 years, mean visual acuity was 20/200 in the affected eye, and the most common visual field defect was an altitudinal deficit (10 cases). Conclusions Although results of this small study should be interpreted cautiously, extrapolation of our findings to the United States white population indicates that nearly 5,700 new cases of acute nonarteritic anterior ischemic optic neuropathy may be expected to occur each year in this group.

Purpose To describe the known ocular side effects of agents used to treat erectile dysfunction. Design Retrospective observational case series and literature review. Methods We reviewed 892 case reports from the National Registry of Drug-Induced Ocular Side Effects (Casey Eye Institute, Portland, Oregon), the Food and Drug Administration (FDA), and the World Health Organization (WHO) and conducted a comprehensive review of the literature. Results Ocular side effects of these drugs appear fully reversible and transitory. Conclusions To date, evidence does not exist to discourage use of erectile dysfunction agents attributable to harmful ocular side effects. Ischemic optic neuropathy (ION) is "possible" by WHO classification, however, conclusive evidence linking ION to erectile dysfunction medications is still lacking.

No effective treatment has been established for nonarteritic anterior ischemic optic neuropathy. Although most cases occur spontaneously, acute hypotension plays a clear role in a subset of patients. We examined three patients with severe visual loss from ischemic optic neuropathy induced by hypotension. The first patient developed anterior ischemic optic neuropathy after excessively rapid correction of malignant hypertension. In the second patient, anterior ischemic optic neuropathy occurred after an episode of orthostatic hypotension from systemic hypovolemia. The third patient developed anterior ischemic optic neuropathy after becoming hypotensive during a routine hemodialysis session. Measures were undertaken immediately to reverse the hypotension in all three patients. This intervention resulted in partial recovery of vision in each patient.

Purpose To determine whether an association exists between sleep apnea and open-angle glaucoma, normal-tension glaucoma, nonarteritic ischemic optic neuropathy (NAION), papilledema, or idiopathic intracranial hypertension (IIH) and whether treatment with continuous positive airway pressure affects the development of these conditions. Design Retrospective, longitudinal cohort study. Methods Billing records for beneficiaries 40 years of age and older enrolled in a large United States managed care network from 2001 through 2007 were reviewed. Incidence of open-angle glaucoma, normal-tension glaucoma, NAION, papilledema, and IIH were determined for the beneficiaries and were stratified by sleep apnea status. Cox regression analyses determined the hazard of each of these conditions developing among individuals with and without sleep apnea, with adjustment for sociodemographic, ocular, and medical conditions. Results Among the 2 259 061 individuals in the study, 156 336 (6.9%) had 1 or more sleep apnea diagnoses. The hazard of open-angle glaucoma was no different among persons with sleep apnea either treated (adjusted hazard ratio [HR], 0.99; 95% confidence interval [CI], 0.82 to 1.18) or untreated with continuous positive airway pressure (HR, 1.01; 95% CI, 0.98 to 1.05) and individuals without sleep apnea. Similar findings were observed when assessing the hazard of normal-tension glaucoma developing ( P > .05 for both comparisons). A significantly increased hazard of NAION developing (HR, 1.16; 95% CI, 1.01 to 1.33) and IIH (HR, 2.03; 95% CI, 1.65 to 2.49) was observed among individuals with sleep apnea who were not receiving continuous positive airway pressure therapy as compared with individuals without sleep apnea, although similar increased risks could not be demonstrated among continuous positive airway pressure-treated sleep apnea patients for these conditions ( P > .05 for both comparisons). Conclusions Patients with untreated sleep apnea are at increased risk for IIH and NAION. Clinicians should consider appropriate screening for these conditions in sleep apnea patients.

We performed optic nerve sheath decompression on four patients (five eyes) with visual loss secondary to nonarteritic anterior ischemic optic neuropathy. Four of the five eyes had marked improvement in visual function after the operation. Optic nerve sheath decompression is an effective treatment for patients with nonarteritic ischemic optic neuropathy and progressive visual loss.

Support for the diagnosis as the primary phospholipid antibody syndrome includes the insidious onset of pallor and visual loss, which would not be expected with temporal arteritis, ischemic optic neuropathy, or collagen vascular disease, and the lack of any other positive tests supporting alternative diagnoses. The aortic valvular disease also is an interesting finding, because a significant number of patients with this syndrome have been reported to have associated valve lesions.' We believe this case most likely represents a primary antiphospholipid antibody syndrome as the cause of this patient's optic atrophy. This syndrome is important to recognize, because treatment with corticosteroids and immunosuppressive agents has been shown to lower antibody titer with some resolution of disease, although clinical results are unpredictable.' This syndrome should be included in the differential diagnosis of optic atrophy.

Some patients with nonarteritic ischemic optic neuropathy have no signs or symptoms of systemic vascular disease; therefore, nonvascular factors may play a role in the pathogenesis of this syndrome. We observed that the average cup-disk ratio of the unaffected optic disks in a group of patients with ischemic optic neuropathy was significantly (P = .005) smaller than the average ratio in a group of age-matched controls (0.36 +/- 0.19 vs 0.21 +/- 0.16). Fourteen of 30 patients but only two of 29 controls had cup-disk ratios equal to or less than 0.1. Mechanical factors, such as constriction of axons as they traverse the scleral canal, may contribute to the pathogenesis of ischemic optic neuropathy.

Eighteen patients with idiopathic optic neuropathy lacked symptoms and signs of cardiovascular and cerebrovascular disease, especially when compared to three groups of patients with sudden visual loss caused by retinal infarction, transient ischemia, and cerebral infarction. Many patients in the latter groups had hypertension, carotid bruits, heart disease, transient ischemic attack, and stroke. But among the patients with ischemic optic neuropathy, hypertension was the only evidence of cardiovascular disease, affecting 44% of the patients. We argue that, in many cases, ischemic optic neuropathy represents a direct and early complication of hypertension arterial disease affecting small arterioles supplying the anterior part of the optic nerve. The pathologic process may thus be similar or identical to lacunar infarction of the brain.

Summary A 32-year-old woman developed bilateral blindness along with severe preeclampsia of pregnancy. Ophthalmoscopic examination disclosed chalky-white optic disks with only minimal changes in the retinal vasculature. After caesarean section, the preeclampsia improved, and complete return of visual acuity occurred during a three-day period. Blindness has rarely been reported as a complication of preeclampsia. In almost all cases it has been associated with severe compromise of the retinal vasculature. Conversely, the ophthalmoscopic findings in our case strongly support acute ischemic optic neuropathy as the cause of blindness.

A 67-year-old woman, a retired writer, was ad­ mitted to the hospital on March IS, 1968. She stated that she had been in excellent health until the previous summer, when she noted an unusual lack of energy. In December, 1967, she experienced the abrupt onset of bitemporal aching pains which waxed and waned in severity and which radiated to her jaw. Examination revealed that both tem­ poral arteries were stony hard and very tender. An erythrocyte sedimentation rate (ESR) was 8.0/ mm/hour. In view of the history and physical ex­ amination, temporal arteritis was diagnosed. Be­ cause of personal fears, the patient refused to take corticosteroids, and aspirin, 10 gr every four hours, was prescribed. One week later she awakened in the morning and noted severe visual impairment in her left eye, pro­ gressing within a day to monocular blindness. Eye examination the following week revealed a visual

We measured the horizontal diameter, vertical diameter, and area of the optic disk in fellow eyes of patients with unilateral nonarteritic ischemic optic neuropathy and in control eyes without ocular disease. The fellow eyes of patients with anterior ischemic optic neuropathy had a smaller horizontal than vertical disk diameter (P less than .05), a difference not present in controls. Both the horizontal disk diameter and the disk area were significantly smaller in fellow eyes of patients with anterior ischemic optic neuropathy than in controls (P less than .05). There was no statistical difference in the vertical disk diameters between the two groups. Our findings suggest that a small disk area along with a horizontal shortening of the scleral canal can lead to crowding of optic nerve fibers, predisposing to a circulatory compromise of the optic nerve head in non-arteritic anterior ischemic optic neuropathy.

We performed magnetic resonance imaging on nine patients with nonarteritic ischemic optic neuropathy and 11 control patients with sensorineural hearing loss and suspected acoustic neuroma. We then counted the number of subcortical lesions in each patient, which may represent an index of generalized cerebral vascular disease. Although patients in the ischemic optic neuropathy group had an average of 3.2 subcortical lesions and those in the control group had an average of 0.9 lesions, this difference was not significant. Based on the presence of subcortical lesions, patients with nonarteritic ischemic optic neuropathy do not appear to have an increased incidence of generalized cerebral vascular disease.

Ischemic optic neuropathy occurred in three patients (a 55-year-old man and two women, 64 and 68 years old). Visual loss followed coronary bypass surgery in two patients who also had diffuse atherosclerotic disease. Retinal emboli were present in both eyes of each. The third patient developed ischemic optic neuropathy with evidence of ipsilateral retinal emboli shortly after cardiac catheterization. Her only other risk factor for ischemic optic neuropathy was systemic hypertension. Although highly unusual, ischemic optic neuropathy may be associated with and possibly caused by a shower of emboli to the eye.

On examining the postmortem enucleated eyes of a 68-year-old man, we found the left eye had focal infarction 3 mm behind the lamina cribrosa caused by thromboembolic compromise of three discrete pial and pial-derived arterioles. The optic nerve had been destroyed focally, whereas the retinal and choroidal tissue supplied by a similarly occluded vessel had been preserved. We diagnosed nonarteritis ischemic optic neuropathy.

Six patients with nonarteritic ischemic optic neuropathy experienced worsening of visual acuity and field loss during the six-week period after onset (range, three to six weeks), without apparent ophthalmoscopic changes. Thereafter, visual function remained unchanged. Various medications were used, but none prevented deterioration of visual function. Although not a widely recognized phenomenon, progression of visual deficit occurred in these patients in the early weeks after onset of ischemic optic neuropathy.

We used an infrared videopupillometer combined with an electronic circuit that regulated the retinal light level as a function of pupil area to assess the regularity of pupil cycling in normal subjects and in patients with known abnormalities in the pupil light reflex pathways. The light stimulus was turned on whenever pupil area exceeded a preset value. Two types of abnormalities were observed for patients with demyelinative optic neuropathy: a failure of the pupil to cycle despite a preserved pupillary response to a single light pulse; and, for those patients in whom cycling was possible, a characteristic intermittent irregularity in the amplitude of pupil cycling. These abnormalities were not seen in normal subjects or in patients with ischemic optic neuropathy, surgical lesions involving the optic chiasm, Adie's syndrome, or Horner's syndrome.