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Competing Interests: The authors report no conflicts of interest.

An initial misdiagnosis of papilloedema in a patient with optic nerve head swelling can be anxiety-provoking and may result in unnecessary, invasive, and costly tests. Cirrus high definition, spectral domain-optical coherence tomography (Cirrus HD-OCT) may provide a rapid and non-invasive test. We sought to determine an upper limit of average retinal nerve fibre layer (RNFL) thickness in patients with pseudopapilloedema without visible drusen using Cirrus HD-OCT that could be utilised in conjunction with the clinical presentation and physical examination when managing patients with optic nerve head swelling. Inclusion criteria consisted of at least two neuro-ophthalmological visits and repeated imaging of the optic nerve head with Cirrus HD-OCT at least 6 months apart. Exclusion criteria included clinically visible drusen along with previous or concomitant diagnosis of retinal or other optic nerve pathology. Thirty-eight eyes from 19 patients with pseudopapilloedema were included in this study. The upper limit of average RNFL thickness was defined as two standard deviations above the mean of the average RNFL thickness and was calculated to be 158.65 µm for scans obtained with Cirrus HD-OCT devices. A patient with suspected optic nerve head swelling, an average RNFL thickness less than 158.65 µm, and no other evidence of papilloedema or neurological signs or symptoms can be managed with serial follow-ups with OCT imaging for at least 6 months. If the patient continues to have no clinical symptoms suggesting increased intracranial pressure and the average RNFL thickness is stable, the likelihood of papilloedema is minimal., Competing Interests: No potential conflict of interest was reported by the authors., (© 2022 Taylor & Francis Group, LLC.)

Purpose: The authors describe a case of new bilateral epiretinal membranes and vitreous cells after intravitreal and sub-Tenon's injections of bone marrow-derived stem cells., Methods: A case report of a 43-year-old man with OPA-1 autosomal dominant optic atrophy who self-enrolled in a stem cell therapy clinical trial and received simultaneous bilateral intravitreal and sub-Tenon's injections of bone marrow-derived stem cells., Results: Within one month of receiving the injections, the patient developed epiretinal membranes and vitreous cells in both eyes, seen with optical coherence tomography., Conclusion: Stem cell therapy has been gaining popularity as a potential intervention for progressive retinal and optic nerve diseases; however, the mechanism of action of bone marrow-derived stem cells is still not well understood and may result in unintended cell differentiation.

Background: Although nonarteritic anterior ischemic optic neuropathy (NAION) is considered a disorder that primarily affects the optic nerve head, optical coherence tomography (OCT) shows peripapillary and foveal subretinal fluid associated with optic disc swelling from NAION. We sought to further evaluate retinal and vitreous changes in patients with NAION., Methods: Patients diagnosed with NAION at the New England Eye Center between 2013 and 2017 were evaluated using OCT. The presence and distribution of subretinal fluid was analyzed. Evidence of other vitreoretinal changes, including vitreopapillary traction (VPT) and the presence of hyperreflective dots (HRD), were also determined., Results: Twenty-five eyes from 20 patients who presented within 4 weeks of symptom onset were assessed. Peripapillary subretinal fluid was seen in 16 eyes (64%). Of those eyes, the subretinal fluid extended into the macula in 4 eyes (16%). Visual acuity improved in 2 of 4 eyes after subfoveal fluid resolution. Intraretinal cysts located in the peripapillary region were seen in 8 eyes (32%), HRD were noted in 11 (44.0%). There was no evidence of VPT., Conclusions: A substantial number of patients with NAION have subretinal fluid on OCT, consistent with prior reports. Resolution of subfoveal fluid may result in some recovery of visual acuity. Other retinal changes, such as intraretinal cysts and HRD, are present but have unclear implications. We did not find evidence of a primary role of VPT in the pathophysiology of NAION., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Background: Although giant cell arteritis (GCA) is a well-known cause of transient and permanent vision loss, diplopia as a presenting symptom of this condition is uncommon. We compared symptoms and signs of patients presenting with diplopia from GCA to those from other causes., Methods: This was a multicenter, retrospective study comparing the clinical characteristics of patients presenting with diplopia from GCA with age-matched controls. Demographic information, review of symptoms, ophthalmic examination, and laboratory data of biopsy-proven patients with GCA were compared with those of age-matched controls presenting with diplopia., Results: A total of 27 patients presented with diplopia from GCA, 19 with constant diplopia, and 8 with transient diplopia. All patients with constant diplopia from GCA were matched with 67 control subjects who had diplopia from other etiologies. Patients with GCA were more likely to describe other accompanying visual symptoms (58% vs 25%, P = 0.008), a greater number of systemic GCA symptoms (3.5, GCA vs 0.6, controls, P < 0.001) such as headache (94% [17/18] vs 39% [23/67]; P < 0.001), jaw claudication (80% [12/15] vs 0% [0/36]; P < 0.001), and scalp tenderness (44% [7/16] vs 7% [3/43]; P < 0.001). Ocular ischemic lesions (26% vs 1%, P < 0.001) were also common in patients with diplopia from GCA. Inflammatory markers were elevated significantly in patients with GCA vs controls (erythrocyte sedimentation rate: 91% [10/11] vs 12% [3/25], P < 0.001; C-reactive protein: 89% [8/9] vs 11% [2/19], P < 0.001)., Conclusions: GCA is a rare but serious cause of diplopia among older adults and must be differentiated from other more common benign etiologies. Our study suggests that most patients with diplopia from GCA have concerning systemic symptoms and/or elevated inflammatory markers that should trigger further work-up. Moreover, careful ophthalmoscopic examination should be performed to look for presence of ocular ischemic lesions in older patients presenting with acute diplopia.

Purpose: To describe the rapid time course of visual and electroretinographic recovery from vitamin A deficiency in a patient with a history of multiple resected abdominal tumors, including ileal carcinoid and pancreatic adenocarcinoma., Methods: A 61-year-old white man with a history of resected malignant ileal carcinoid and Stage III pancreatic adenocarcinoma referred with complaints of 6 weeks of difficulty with night vision., Results: Initial testing showed significantly reduced scotopic rod responses in both eyes and decreased vitamin A levels and a normal cancer-associated retinopathy laboratory panel. He had complete recovery of both his symptoms and full-field electroretinography within 5 days of starting intramuscular vitamin A., Conclusion: Vitamin A deficiency-related retinopathy after abdominal surgery may be an underreported complication. This case provides a unique clinical perspective in our patient with a history of ileal carcinoid and Stage III pancreatic adenocarcinoma and confirms that rapid symptomatic and electroretinographic recovery is possible with appropriate treatment.

Background: Optical coherence tomography angiography (OCTA) has demonstrated good utility in qualitative analysis of retinal and choroidal vasculature and therefore may be relevant in the diagnostic and treatment efforts surrounding nonarteritic anterior ischemic optic neuropathy (NAION)., Methods: Retrospective, cross-sectional study of 10 eyes of 9 patients with a previous or new diagnosis of NAION that received imaging with OCTA between November 2015 and February 2016. Two independent readers qualitatively analyzed the retinal peripapillary capillaries (RPC) and peripapillary choriocapillaris (PCC) for flow impairment. Findings were compared with automated visual field and structural optical coherence tomography (OCT) studies., Results: Flow impairment seen on OCTA in the RPC corresponded to structural OCT deficits of the retinal nerve fiber layer (RNFL) and ganglion cell layer complex (GCC) in 80% and 100% of eyes, respectively, and to automated visual field deficits in 90% of eyes. Flow impairment seen on OCTA in the PCC corresponded to structural OCT deficits of the RNFL and GCC in 70% and 80% of eyes, respectively, and to visual field deficits in 60%-80% of eyes., Conclusions: OCTA can noninvasively visualize microvascular flow impairment in patients with NAION.

Purpose of Review: Neuroophthalmologists and neurosurgeons are often asked by their patients what their visual prognosis will be after decompression of an optic chiasm lesion. Previous methods have been studied but have not provided consistent guidance. However, a recent algorithm which allows for retinal ganglion cell analysis from optical coherence tomography (OCT) may be more helpful., Recent Findings: Recent studies have shown that ganglion cell layer complex (GCC) measurements from OCT strongly correlate with the visual field loss associated with compressive optic neuropathies. For example, GCC measurements show corresponding binasal thinning with bitemporal hemianopia. Some investigators have also shown that more preserved GCC thickness is preoperatively associated with better postsurgical outcome. Interestingly, some patients experience almost complete recovery of visual fields despite considerable GCC thinning, and others may have GCC loss before they develop demonstrable visual field defects., Summary: GCC measurements on OCT strongly correlate with visual field defects from optic chiasm compressive lesions and may help with regard to prognosis following treatment. However, considerable visual recovery is possible despite persistent GCC loss.

Clinical data suggest that optic neuropathy and retinal ganglion cell loss are the main cause of visual decline in patients with familial dysautonomia, but this has not previously been confirmed by pathological analyses. We studied retinas and optic nerves in 6 eyes from 3 affected patients obtained at autopsy. Analyses included routine neurohistology and immunohistochemistry for neurofilaments, cytochrome c oxidase (COX), and melanopsin-containing ganglion cells. We observed profound axon loss in the temporal portions of optic nerves with relative preservation in the nasal portions; this correlated with clinical and optical coherence tomography findings in 1 patient. Retinal ganglion cell layers were markedly reduced in the central retina, whereas melanopsin-containing ganglion cells were relatively spared. COX staining was reduced in the temporal portions of the optic nerve indicating reduced mitochondrial density. Axonal swelling with degenerating lysosomes and mitochondria were observed by electron microscopy. These findings support the concept that there is a specific optic neuropathy and retinopathy in patients with familial dysautonomia similar to that seen in other optic neuropathies with mitochondrial dysfunction. This raises the possibility that defective expression of the IkB kinase complex-associated protein (IKAP) resulting from mutations in IKBKAP affects mitochondrial function in the metabolism-dependent retinal parvocellular ganglion cells in this condition., (© 2017 American Association of Neuropathologists, Inc. All rights reserved.)

Background: Patterns of ganglion cell complex (GCC) loss detected by optical coherence tomography provide an objective measure of optic nerve injury. These patterns aid in early diagnosis and localization of chiasmal lesions., Methods: Twenty-three patients with chiasmal compression seen between 2010 and 2015 were imaged with the Cirrus high-definition optical coherence tomography macular cube 512 × 128, retinal nerve fiber layer (RNFL) scan protocols and automated (30-2 Humphrey) visual fields (VFs). Age-matched controls were included for comparison. Generalized estimating equations were performed comparing RNFL and GCC thicknesses between patients and their controls. Effect size (d) was calculated to assess the magnitude of difference between patients and controls. The average GCC and RNFL thicknesses also were correlated with VF mean deviation (MD). Pre operative average GCC thickness was correlated to post operative VF MD., Results: Patterns of GCC thinning corresponded to VF defects. The average GCC thickness was 67 ± 9 μm in patients and 86 ± 5 μm in controls (P < 0.001). The effect size was the greatest for GCC thickness (d = 2.72). The mean deviation was better correlated with GCC thickness (r =0.25) than RNFL thicknesses (r =0.15). Postoperatively, VF MD improved in 7 of 8 patients with persistent nasal GCC thinning. Six patients had no VF defect and showed statistically significant loss of GCC compared with controls (P = 0.001)., Conclusions: Distinct patterns of GCC loss were identified in patients with chiasmal compression. Binasal GCC loss was typical and could be seen with minimal or no detectable VF loss. Thinning of the GCC may be detected before loss of the RNFL in some patients. After decompression, the majority of patients showed improvement in VF despite persistent GCC loss. Patients with less GCC loss before decompression had better postoperative VFs. Therefore, GCC analysis may be an objective method to diagnose and follow patients with chiasmal lesions.

Purpose: To determine whether a pattern of altitudinal ganglion cell loss, as detected and measured by optical coherence tomography (OCT), can be used to distinguish non-arteritic ischaemic optic neuropathy (NAION) from optic neuritis (ON) during the acute phase, and whether the rate or severity of ganglion cell loss differs between the two diseases., Methods: We performed a retrospective, case-control study of 44 patients (50 eyes) with ON or NAION and 44 age-matched controls. Non-arteritic ischaemic optic neuropathy and ON patients had OCT at presentation and four consecutive follow-up visits. Controls had OCT at one point in time. The ganglion cell complex (GCC) was evaluated in the macula, and the retinal nerve fibre layer (RNFL) was evaluated in the peripapillary region. Ganglion cell complex thickness, RNFL thickness and GCC mean superior and inferior hemispheric difference were compared between NAION and ON patients at each time-point using unpaired t-tests and between disease and control subjects at first measurement using paired t-tests., Results: Mean time from onset of symptoms to initial presentation was 10.7 ± 6.6 days in NAION and 11.7 ± 8.6 days in ON (p = 0.67). There was a significantly greater vertical hemispheric difference in GCC thickness in NAION patients than ON patients at all time-points (5.5-10.7 μm versus 3.1-3.6 μm, p = 0.01-0.049). Mean GCC thickness was significantly decreased at less than 2 weeks after onset in NAION compared to age-matched controls (72.1 μm versus 82.1 μm, p < 0.001), as well as in ON compared to age-matched controls (74.3 μm versus 84.5 μm, p < 0.001). Progression and severity of GCC and RNFL loss did not differ significantly between NAION and ON., Conclusion: A quantitative comparison of mean superior and inferior hemispheric GCC thickness with OCT may be used to distinguish NAION from ON., (© 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Background: Objective measures of disease progression that can be used as endpoints in clinical trials of MSA are necessary. We studied retinal thickness in patients with MSA and assessed changes over time to determine its usefulness as an imaging biomarker of disease progression., Methods: This was a cross-sectional study including 24 patients with MSA, 20 with PD, and 35 controls, followed by a longitudinal study of 13 MSA patients. Patients were evaluated with high-definition optical coherence tomography and the Unified Multiple System Atrophy Rating Scale. Evaluations were performed at baseline and at consecutive follow-up visits for up to 26 months., Results: MSA subjects had normal visual acuity and color discrimination. Compared to controls, retinal nerve fiber layer (P = 0.008 and P = 0.001) and ganglion cell complex (P = 0.013 and P = 0.001) thicknesses were reduced in MSA and PD. No significant differences between MSA and PD were found. Over time, in patients with MSA, there was a significant reduction of the retinal nerve fiber layer and ganglion cell complex thicknesses, with estimated annual average losses of 3.7 and 1.8 μm, respectively., Conclusions: Visually asymptomatic MSA patients exhibit progressive reductions in the thickness of the retinal nerve fiber layer and, to a lesser extent, in the macular ganglion cell complex, which can be quantified by high-definition optical coherence tomography. Specific patterns of retinal nerve fiber damage could be a useful imaging biomarker of disease progression in future clinical trials., (© 2015 International Parkinson and Movement Disorder Society.)

Unlabelled: OBJECT.: The benefits of treating intracranial aneurysms in the region of the anterior visual pathways are well understood. However, the adverse effects of endovascular stenting across the ophthalmic artery have received little attention. The authors reviewed their experience with patients who had stents deployed across the ophthalmic artery origin., Methods: Patients' medical charts and imaging studies were reviewed to identify all patients with a non-flow diverting stent deployed over the ophthalmic artery origin for the treatment of intracranial aneurysms. All patients with neuro-ophthalmic complaints were referred for formal ophthalmological evaluation., Results: A total of 104 consecutive patients with 106 aneurysms were identified to meet criteria for inclusion in the study cohort. Preoperatively, 30 patients (29%) described headache symptoms and 32 patients (31%) reported visual complaints. Of the patients with preoperative headaches, 15 (54%) of 28 patients for whom follow-up was available experienced improvement in their symptoms. Of the patients with preoperative visual complaints, improvement was noted in 11 (41%) of the 27 patients for whom follow-up was available, 9 (33%) of 27 patients reported no change in visual symptoms, and 7 (26%) of 27 patients reported progression of symptoms. Visual field defects developing posttreatment were noted to occur in 8 (7.7%) of 104 patients: 3 with immediate postoperative retinal infarcts, 1 with perioperative hemianopia that resolved by the time of discharge, 1 with a subjective visual field defect, 1 with subjective migratory visual field defects, and 2 with nonspecific visual symptoms. Compressive symptoms from aneurysm mass effect were noted in 6 patients preoperatively, with 4 of those patients experiencing persistent worsening, resolution in 1 case, and no change in 1 case. One patient developed a novel cranial nerve palsy from mass effect in the immediate postoperative period., Conclusions: Deployment of stents across the ophthalmic artery origin for the treatment of intracranial aneurysms appears to be relatively safe with regard to visual outcomes. Neuro-ophthalmic complaint resolution rates were comparable to endovascular procedures that do not employ stents, with headache resolution rates comparable to coil-only aneurysm obliteration and low rates of retinal ischemic events. For patients presenting with mass effect, stent-assisted coiling appears to be less effective than microsurgery with decompression for relief of compressive symptoms.

Optical coherence tomography (OCT) of the macula in patients with primary optic neuropathy has revealed the presence of structural changes in the neurosensory retina in addition to the nerve fibre layer. Subretinal fluid has been documented in papilloedema and non-arteritic ischaemic optic neuropathy, and may account for decreased visual acuity in affected patients. Subretinal fluid has also been described from other causes of optic nerve head swelling including diabetic papillopathy and papillitis. Drugs used in the treatment of multiple sclerosis, such as corticosteroids and fingolimod can cause decreased vision due to central serous and cystoid macular oedema sometimes confused with recurrent optic neuritis. A subset of patients with various types of optic atrophy show microcystic changes in the inner nuclear layer on spectral domain OCT imaging. The pathophysiology and visual significance of these retinal changes remain unclear, but may affect the diagnosis and management of optic nerve disorders., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Purpose: The purpose of this study was to report a case series of patients with acute macular neuroretinopathy in which patients concomitantly used oral norepinephrine reuptake inhibitors., Methods: Retrospective case review., Patients: Three patients with retinal findings consistent with acute macular neuroretinopathy are presented., Conclusion: The use of oral neuromodulators should be considered in cases of acute macular neuroretinopathy.

Background: To define the clinical neuro-ophthalmic abnormalities of patients with familial dysautonomia (FD)., Methods: Sixteen patients (32 eyes) with the clinical and molecular diagnoses of FD underwent thorough neuro-ophthalmic clinical evaluation., Results: Visual acuity ranged from 0.05 to 1.0 decimal units and was reduced in 15 of 16 patients. Mild to moderate corneal opacities were found in most patients but were visually significant in only 2 eyes. Red-green color vision was impaired in almost all cases. Depression of the central visual fields was present on automated visual fields in all patients, even in those with normal visual acuity. Temporal optic nerve pallor was present in all cases and was associated with retinal nerve fiber layer loss in the papillomacular region. Various ocular motility abnormalities also were observed., Conclusion: Patients with FD have a specific type of optic neuropathy with predominant loss of papillomacular nerve fibers, a pattern similar to other hereditary optic neuropathies caused by mutations either in nuclear or in mitochondrial DNA, affecting mitochondrial protein function. Defects of eye movements, particularly saccades, also appear to be a feature of patients with FD.

OCT is probably one of the most revolutionary technologies that has appeared in ophthalmology in recent years. We are still trying to understand and clarify the use of OCT in neuro-ophthalmology. In this article we share our experience using various OCT devices over the last ten years, as well as the experience of others as reported in the literature.

An 85-year-old male presented with unilateral third, fourth, and sixth cranial nerve palsies. Neuroimaging revealed a mass involving the medial wall of the left cavernous sinus, without orbital involvement. Biopsy of the mass provided a pathologic diagnosis of esthesioneuroblastoma.

Bernard Schwartz, MD, PhD (1927-2007) was first and foremost a scholar. He was a teacher, serving as Professor of ophthalmology for many years at Tufts University; an administrator, chairing the department of ophthalmology for over 20 years; a clinician, practicing primarily in the field of glaucoma; a scientist with a broad interest in ophthalmic research; an historian, especially of medical history; and an editor, primarily of Survey of Ophthalmology. Throughout all of his activities, the central theme was always scholarship.

Objective: To demonstrate the development of subfoveal fluid associated with optic disc swelling from nonarteritic anterior ischemic optic neuropathy., Methods: Optical coherence tomographic studies obtained during a 3-year period (October 1, 2003, to December 30, 2006) from 76 patients who developed ischemic optic neuropathy from 2 institutions were evaluated. The presence or absence, and the distribution, of subretinal fluid was determined., Results: Seventy-six patients underwent macular optical coherence tomography within 4 weeks of developing sudden loss of vision in one eye, decreased visual acuity, a visual field defect, a relative afferent pupillary defect, and optic disc swelling with peripapillary hemorrhages. Eight patients had apparent subretinal fluid extending into the subfoveal space. Visual acuity improved in 5 of the 8 patients as the subfoveal fluid resolved., Conclusions: Subretinal fluid develops in some patients with nonarteritic anterior ischemic optic neuropathy and may contribute to some of the visual loss associated with this condition. Furthermore, resolution of the subretinal fluid could account for some of the visual improvement that can follow anterior ischemic optic neuropathy.

A 42-year-old man with Hunter syndrome developed bilateral visual field loss. Visual field testing demon-strated bilateral ring scotomata that corresponded to areas of thinning seen on standard resolution optical coherence tomography. High-speed, ultrahigh resolution optical coherence tomography, capable of 3.5-micron axial resolution, showed a loss of photoreceptors outside the fovea and cystoid spaces within the inner nuclear, ganglion cell, and outer nuclear layers. These results were consistent with histopathologic features that have been reported previously in patients with Hunter syndrome. Optical coherence tomography could be used as a diagnostic modality to monitor patients with Hunter syndrome and to detect subclinical forms of disease.

Objectives: To compare structural changes in the retina seen on high-speed ultra-high-resolution optical coherence tomography (hsUHR-OCT) with multifocal electroretinography (mfERG) and automated visual fields in patients receiving hydroxychloroquine., Methods: Fifteen patients receiving hydroxychloroquine were evaluated clinically with hsUHR-OCT, mfERG, and automated visual fields. Six age-matched subjects were imaged with hsUHR-OCT and served as controls., Results: Distinctive discontinuity of the perifoveal photoreceptor inner segment/outer segment junction and thinning of the outer nuclear layer were seen with hsUHR-OCT in patients with mild retinal toxic effects. Progression to complete loss of the inner segment/outer segment junction and hyperscattering at the outer segment level were seen in more advanced cases. The mfERG abnormalities correlated with the hsUHR-OCT findings. Asymptomatic patients had normal hsUHR-OCT and mfERG results., Conclusion: Distinctive abnormalities in the perifoveal photoreceptor inner segment/outer segment junction were seen on hsUHR-OCT in patients receiving hydroxychloroquine who also were symptomatic and had abnormalities on automated visual fields and mfERG.

A 17-year-old woman developed a sixth cranial nerve palsy from a malignant peripheral nerve sheath tumor of the trigeminal nerve. This case is unusual in that the principal symptom was diplopia stemming from a sixth cranial nerve palsy. Pain was mild, and trigeminal function was preserved. Imaging evidence of rapid growth of the cavernous sinus mass gave rise to an initial impression that the cause might be inflammatory. Treatment with gamma knife stereotactic radiosurgery produced some improvement in sixth cranial nerve function and reduction in tumor size over a follow-up period of 9 months.

The term "papilledema" indicates swelling of the optic discs secondary to increased intracranial pressure. Papilledema can be caused by an intracranial mass lesion or by other factors. Visual symptoms frequently accompany papilledema, which can lead to permanent visual loss if left untreated. Starting with an illustrative case, the authors review the pathophysiology of the visual signs and symptoms of papilledema. They also briefly review potential treatment options, focusing on the role of the neurosurgeon in the treatment of patients with papilledema.

Objective: To evaluate the use of multifocal visual evoked potentials in the diagnosis of nonorganic visual field loss., Methods: Five patients with unexplained visual field loss underwent full neuro-ophthalmic examination, Humphrey visual field testing, and multifocal visual evoked potential testing using the Accumap objective perimeter (ObjectiVision Pty Ltd, Sydney, Australia)., Results: In all 5 cases, the results of the ophthalmic examination did not correlate with the degree of visual field loss seen on Humphrey visual field testing. Multifocal visual evoked potentials testing showed essentially normal tracings., Conclusions: Multifocal visual evoked potentials may be useful in cases of difficult to prove functional visual field loss or in cases in which objective documentation of normal function is needed.

Multifocal VEP and mfERG techniques show promise for routine clinical use in neuro-ophthalmology. OCT is now used in many subspecialty areas of ophthalmology, especially in studies of the retina and glaucoma. Although neuro-ophthalmologists must still rely on time-consuming history taking and careful clinical examination, the three modalities described herein can provide objective proof regarding clinical diagnoses in selected cases, saving time and avoiding costly neurologic investigations.

Objective: To determine if patients with nonarteritic ischemic optic neuropathy (NAION) have sleep apnea syndrome (SAS), an entity characterized by repetitive upper airway obstructions during sleep, inducing hypoxia and sleep disruption., Methods: We recruited 17 patients with NAION and 17 age- and sex-matched controls from patients referred for treatment because of suspected restless legs syndrome. We performed overnight polysomnography and determined the respiratory disturbance index during night sleep, a value used to diagnose and grade SAS. We compared the proportions of patients with SAS among patients with NAION and matched controls using the chi(2) test. Additionally, we compared the proportions of patients with SAS among patients with NAION and a large SAS prevalence study using the binomial test., Results: Twelve (71%) of 17 patients with NAION had SAS. According to the respiratory disturbance index, 4 patients (24%) had mild, 4 patients (24%) had moderate, and 4 patients (24%) had severe SAS. Only 3 (18%) of 17 controls had SAS (P =.005). In the 45- to 64-year age group, 4 (50%) of 8 patients with NAION had SAS; 51 (11.9%) of 430 of the random sample in the prevalence study had SAS (P =.005). In the group older than 64 years, 8 (89%) of 9 patients with NAION had SAS; 18 (24%) of 75 of the random sample in the prevalence study had SAS (P<.001)., Conclusions: We found a high prevalence of SAS in patients with NAION, which supports previous case reports suggesting that such an association exists. This association may explain why approximately 75% of all patients with NAION discover visual loss on first awakening or when they first use vision critically after sleeping. Our findings indicate that SAS may play an important role in the pathogenesis of NAION.

Objective: To evaluate macular changes in eyes with papilledema from increased intracranial pressure using optical coherence tomography (OCT)., Methods: Fifty-five patients with papilledema seen during 1998 and 1999 were studied with OCT of the optic nerve and retinal nerve fiber layer. Nineteen of these also had OCT of the macula during periods of acute, subacute, or recurrent papilledema and were evaluated in detail for this report., Results: Seven patients had OCT evidence of subretinal fluid involving the macula. All had some reduction in visual acuity. The subretinal fluid appeared to arise from the peripapillary region, and all showed some improvement in central vision as the fluid resolved., Conclusions: Subretinal fluid accumulations can cause decreased visual acuity in patients with papilledema. Optical coherence tomography can demonstrate subretinal fluid and can be used to follow the course of this important visual complication of papilledema.

Involvement of the optic nerve, either because of inflammation or increased intracranial pressure, is a rare manifestation of Lyme disease. Of the 4 children reported here with optic nerve abnormalities, 2 had decreased vision months after disease onset attributable to optic neuritis, and 1 had headache and diplopia early in the infection because of increased intracranial pressure associated with Lyme meningitis. In these 3 children, optic nerve involvement responded well to intravenous ceftriaxone therapy. The fourth child had headache and visual loss attributable to increased intracranial pressure and perhaps also to optic neuritis. Despite treatment with ceftriaxone and steroids, he had persistent increased intracranial pressure leading to permanent bilateral blindness. Clinicians should be aware that neuro-ophthalmologic involvement of Lyme disease may have significant consequences. If increased intracranial pressure persists despite antibiotic therapy, measures must be taken quickly to reduce the pressure.

Purpose: To describe unusual as well as typical ocular findings in eight patients with Takayasu's disease., Methods: The ophthalmic manifestations and clinical courses of eight patients with Takayasu's disease were evaluated., Results: All patients had clinical and angiographic evidence of pulseless disease. Typical findings included retinal venous congestion, fluorescein staining, capillary drop-out, microaneurysms and arteriovenous shunting. Unusual findings included cotton wool spots, anterior ischemic neuropathy and retinal emboli. In one case microaneurysms disappeared after carotid bypass surgery. Several patients had profound, transient, visual loss with changes in head posture., Conclusion: Takayasu's disease can cause a variety of retinal manifestations. Less common findings include cotton wool spots, anterior ischemic optic neuropathy and retinal emboli.

Retinal ischemia is often caused by emboli arising from the cardiac chambers or the common carotid artery bifurcation; the latter are often composed of cholesterol. However, in many patients no lesions are identified after evaluation of these sources of emboli. Two patients were observed who had retinal ischemia and emboli originating from aortic atheromatous plaques that were visualized by transesophageal echocardiography. Cardiac, carotid, and intracranial sources of emboli were excluded. The embolic nature of retinal ischemia was further corroborated by the presence of microembolic signals during transcranial Doppler insonation of the middle cerebral artery on the side ipsilateral to the symptomatic retina. In patients with Hollenhorst plaques the aortic arch can be a potential source of emboli. Transesophageal echocardiography should be considered in these patients when the initial evaluation does not identify a cardiac or carotid lesion.

Objective: The purpose of the study was to evaluate the effect of optic nerve head drusen (ONHD) on nerve fiber layer (NFL) thickness by visual field testing, red-free photography of NFL, and optical coherence tomography (OCT)., Design: The study design was a prospective clinical study., Participants: Twenty-three eyes of 15 consecutive patients with ONHD and 27 eyes of 27 age-matched control subjects participated., Intervention: Ophthalmologic examination, color and red-free photography, automated Humphrey visual field testing, and OCT were performed. Each of the drusen study eyes were graded on a scale of 0 to III based on the amount of visible ONHD. Grade 0 represented the absence of clinically visible ONHD, and grade III represented an optic nerve head with abundant drusen., Main Outcome Measures: Findings from clinical evaluation and color optic nerve head photographs and NFL evaluation by red-free photography, visual fields, and OCT were measured., Results: The number of study eyes with visual field defects increased with the higher grade drusen discs, corresponding both with progressively thinner NFL measurements by OCT and NFL loss shown by NFL photography. The NFL evaluation showed NFL thinning by red-free photography in 12 (71%) of 17 eyes with visible drusen (grades I-III discs) and visual field defects in 9 (53%) of 17 eyes in this group. By OCT measurements, the superior and inferior NFLs were significantly thinner in the eyes with visible ONHD compared with those of control eyes in the superior quadrant (P < 0.001) and inferior quadrant (P = 0.004). Compared with grade 0 discs, grades I through III discs showed statistically significant thinning of the NFL superiorly (P < 0.001). No statistical significant thinning of the NFL was seen in grade 0 discs compared with those of control subjects., Conclusions: Optical coherence tomography is able to detect NFL thinning in eyes with ONHD and appears to be a sensitive and early indicator of NFL thinning. Increased numbers of clinically visible ONHD correlated with NFL thinning shown by OCT measurements and both visual field defects and NFL loss seen by red-free photography.

Purpose: A healthy 13-year-old girl, previously diagnosed with amblyopia in her right eye, was seen in consultation after her vision continued to decrease., Methods: A complete ophthalmologic examination including visual field testing and optic nerve photography was performed in the neuro-ophthalmologic clinic. Magnetic resonance imaging study was also obtained., Results: Visual sensory deficits and pale optic nerves were noted on clinical examination. Visual field testing showed a chiasmatic junctional defect. Magnetic resonance imaging verified a large chiasmatic mass, histologically proven to be a juvenile pilocytic astrocytoma., Conclusion: Early recognition of signs and symptoms of chiasmatic lesions is essential for preventing visual loss.

Objective: To define orbital circulation abnormalities identified by color Doppler imaging in patients with severe carotid occlusive disease., Patients: Twenty-four patients referred to a hospital-based neuro-ophthalmology service with hemodynamically significant carotid occlusive disease (> 75% stenosis) were prospectively studied. Eight had signs of ocular ischemic syndrome; 12 of the 24 patients underwent endarterectomy., Main Outcome Measures: Peak systolic velocity of the central retinal, posterior ciliary, and ophthalmic artery and pulsatility indexes as determined by color Doppler imaging., Methods: Color Doppler imaging was performed using a 7.5-MHz probe. Both eyes were studied in all patients and carotid duplex imaging was obtained., Results: All patients with hemodynamically significant carotid occlusive disease had lower mean peak systolic velocities in the central retinal, posterior ciliary, and ophthalmic arteries and higher pulsatility indexes compared with normal control patients. Endarterectomy improved peak systolic velocities. Reversal of ophthalmic flow direction as a separate variable was unassociated with altered mean central retinal or posterior ciliary artery flow velocities. Patients with ocular ischemic syndrome may have similar orbital color Doppler imaging findings compared with patients with severe carotid occlusive disease without overt manifestations of chronic ocular ischemia., Conclusion: Orbital circulation is highly adaptable even when faced with severe compromise in proximal blood flow.

During 1992 and 1993 an epidemic of optic and peripheral neuropathy affected over 50,000 Cubans. This occurred in the unique setting of a communist country which had a widespread health care network and wherein sudden changes in the economy affected most of the population. Although nutritional factors appeared to play a key role in the pathogenesis of the epidemic neuropathy, viral, toxic, and genetic factors were investigated by Cuban and North American scientists. The authors, representing different disciplines and different groups that visited Cuba during the epidemic, review and reflect on the clinical and laboratory findings which became available through their own experience and through reviewing the literature. The recent Cuban epidemic is compared to similar outbreaks of optic and peripheral neuropathy which have occurred in the past.

Retinal nerve fiber layer (RNFL) photographs from 26 patients with Alzheimer's disease and 23 normal, age-matched, control subjects were reviewed for quality and abnormalities by two observers. A higher proportion of Alzheimer's patients showed RNFL abnormalities when compared to control subjects. There was some disagreement between the two observers regarding quality and frequency of abnormalities, reflecting suboptimal quality of the photographs obtained in patients with advanced Alzheimer's disease. Although these findings add to the clinical and histopathological evidence that ganglion cell degeneration occurs in Alzheimer's disease, the difficulty in obtaining and evaluating retinal nerve fiber layer photographs, especially in advanced cases, may limit the clinical usefulness of retinal nerve fiber layer analysis in such patients.

The temporal variables of extinction in two patients with right hemispheric stroke and two normal controls were studied using a scanning laser ophthalmoscope (SLO). This instrument enabled eye movements to be controlled for by projecting time controlled stimuli directly on to the retina. Both patients had recovered from their stroke (four months and five years) and seemed clinically normal. At 50 ms, patient 1 extinguished one of two stimuli on seven of 40 double simultaneous stimulation (DSS) trials (five left, two right) and patient 2 extinguished the left stimulus on every DSS trial. At 100 ms, patient 1 performed almost perfectly, whereas patient 2 extinguished on 30% of the DSS trials (10 left, two right). At 200 ms, patient 2 performed perfectly. Neither of the controls showed extinction to DSS at any presentation time. Thus detecting DSS was time dependent in the patients but not the normal subjects. These data support the theory that recovery from neglect after right hemispheric damage may be mediated by the left hemisphere.

1. The use of color doppler imaging (CDI) in ophthalmology is a new and exciting area in vascular imaging. The physiologic information obtained and the anatomy evaluated has been inaccessible by any other noninvasive or invasive technique. 2. Although CDI for ophthalmic use does not, as of this time, have FDA approval, clinical trials have begun. 3. It is our expectation that CDI will be an accurate, reproducible, noninvasive method to assist ophthalmologists in the diagnosis and treatment of patients with orbital and ocular vascular disease.

Purpose: To demonstrate dynamic, reversible abnormalities in ophthalmic artery blood flow velocity identified with color Doppler imaging (CDI) in patients with clinical findings of ocular ischemia with and without carotid artery stenosis., Methods: One patient with ocular ischemia and normal carotid arteries had abnormal ophthalmic artery velocities demonstrated by CDI. Two other patients with reversed ophthalmic artery flow and critical internal carotid artery stenosis were studied before and after carotid endarterectomy. Peak systolic and diastolic velocities as well as pulsatility indices of ophthalmic, posterior ciliary, and central retinal arteries were calculated., Results: The patient who had ocular ischemic syndrome without carotid artery stenosis showed increased ophthalmic artery velocities initially, and reversal of flow within the ophthalmic artery subsequently developed. Clinical findings and symptoms improved gradually as ophthalmic artery, posterior ciliary, and central retinal artery velocities increased. The patients with critical internal carotid artery stenosis had reversed ophthalmic artery blood flow initially which reverted to normal after carotid endarterectomy., Conclusion: Ocular ischemic syndrome may occur due to abnormal blood flow in the ophthalmic artery in the absence as well as in the presence of carotid artery stenosis. Flow dynamics in the ophthalmic artery and its branches can be shown by CDI to revert toward normal as the clinical findings improve spontaneously or after opening an occluded carotid artery.