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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.)

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.

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.

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: 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.

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.

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: To investigate the potential role of mitochondrial DNA (mtDNA) mutations in the recent outbreak in Cuba of optic neuropathy and peripheral neuropathy (COPN)., Design and Methods: Historical features were reviewed and neuro-ophthalmologic examinations were performed on a sample of COPN patients (n = 9) and Cuban patients with other forms of optic neuropathy (n = 2). Molecular genetic methods were then used to test for the presence of 9 mtDNA mutations that were previously associated with Leber's hereditary optic neuropathy (LHON)., Results: Two (22%) of 9 COPN patients harbored an LHON-associated mtDNA mutation at nucleotide position 9438 and a novel mutation at nucleotide position 9738 in the cytochrome c oxidase subunit III gene. None of the Cuban patients harbored any of the 8 other LHON-associated mtDNA mutations. Detailed sequence analysis revealed that the Cuban patients could be divided into 7 distinct mtDNA haplotypes and that the 2 COPN patients with mtDNA mutations in the cytochrome c oxidase subunit III gene were not members of the same maternal lineage., Conclusions: The pathogenesis of epidemic COPN is likely complex and multifactorial. Our preliminary results in a small sample of Cuban patients suggest that mtDNA mutations may play a role in some cases. mtDNA mutations may render an individual genetically susceptible to a variety of factors that impair oxidative phosphorylation, including nutritional deficiency, tobacco, alcohol, and other toxins.

Extracranial internal carotid artery occlusive disease usually produces stroke in the middle cerebral artery territory or the border zone between the middle and anterior cerebral arteries. It is unusual for occipital infarction in the posterior cerebral artery territory to be caused by internal carotid artery disease despite the fact that the posterior cerebral artery may arise directly from the internal carotid artery as an anatomic variation. We describe a patient with a fetal posterior cerebral artery originating from the internal carotid artery, and the initial manifestation of his extracranial internal carotid artery occlusive disease was hemianopsia from occipital infarction.

Optic nerve dysfunction occurred several weeks after traumatic carotid-cavernous fistula developed in a 21-year-old man. Vision was completely restored after the fistula was closed with an intra-arterial detachable balloon. By carefully monitoring visual function in patients with traumatic carotid-cavernous fistulas, delayed optic neuropathy can be recognized and treated successfully.

Ophthalmic complications from moyamoya disease, or multiple progressive intracranial arterial occlusion syndrome, have rarely been reported. This case report describes a patient with this syndrome who developed acute retinal artery occlusion.

Sixty-three patients with third cranial nerve palsies (CNPs), either isolated (31) or in association with other neurologic deficits (32), underwent neuroophthalmologic and neuroradiologic evaluation. Discrepancies between the clinical and radiologic evaluations were analyzed and useful clinical presenting symptoms were identified. Microvascular infarction secondary to diabetes mellitus and/or hypertension was the most common cause in patients with isolated third CNP, and extensive neuroradiologic evaluation is not indicated in this subgroup. The overall diagnostic yield of high-resolution CT for isolated third CNPs was low (30%), but improved to 60% if diabetes and hypertension were excluded. However, CT was highly sensitive (90%) in those patients with third CNPs associated with additional neurologic deficits. The status of the pupil in and of itself cannot be the sole determinant as to whether angiography is indicated to exclude an aneurysm. Careful ophthalmologic observation and relating the severity of pupillomotor dysfunction to extraocular ophthalmoplegia is mandatory to determine the logical sequence of radiologic evaluation. Retroorbital pain taken in isolation is a nonspecific presenting symptom and has differential diagnostic value only if it is correlated temporally with the onset of third CNP and the presence or absence of additional cranial nerve deficits.