Introducing the 24-2C Visual Field Test in Neuro-Ophthalmology.

PURPOSE: The Humphrey 24-2C visual field test is a modified 24-2 visual field test that incorporates 10 additional test points in the central 10° of vision. This study compares the new 24-2C test to the standard Humphrey 10-2 visual field test in patients presenting for neuro-ophthalmology evaluation to evaluate its ability to detect central visual field defects. METHODS: Twenty-five neuro-ophthalmology patients (42 eyes) underwent both 24-2C and 10-2 visual field testing using the Humphrey perimeter. The number of flagged total deviation (TD) and pattern deviation (PD) points of the 10 added test points of the 24-2C were compared with the corresponding 10-2 fields at the P < 5%, P < 2%, and P < 1% significance levels. The total number of flagged TD points were further analyzed by diagnosis. An experienced neuro-ophthalmologist evaluated all visual fields, commenting on the added value for clinical practice. RESULTS: There was no significant difference between the number of flagged TD and PD points of the 10 extra 24-2C points and corresponding 10-2 points at all significance levels. When analyzed by diagnosis, there was no significant difference in the number of flagged TD points in patients with optic neuritis, ischemic optic neuropathy, optic atrophy, and no neuro-ophthalmic disease. The added 24-2C points aided in identifying visual field defects and areas of spared central vision and had similar diagnostic value as the 10-2. CONCLUSIONS: The 24-2C is able to detect visual field loss in the central 10° that corroborates with loss detected in the 10-2 pattern. The 24-2C exhibits potential to be used as a hybrid between the 24-2 and 10-2 to better evaluate visual field defects.

Telemedicine Evaluations in Neuro-Ophthalmology During the COVID-19 Pandemic: Patient and Physician Surveys.

BACKGROUND: The novel coronavirus 2019 (COVID-19) pandemic has transformed health care. With the need to limit COVID-19 exposures, telemedicine has become an increasingly important format for clinical care. Compared with other fields, neuro-ophthalmology faces unique challenges, given its dependence on physical examination signs that are difficult to elicit outside the office setting. As such, it is imperative to understand both patient and provider experiences to continue to adapt the technology and tailor its application. The purpose of this study is to analyze both neuro-ophthalmology physician and patient satisfaction with virtual health visits during the time of the COVID-19 pandemic. METHODS: Across three institutions (NYU Langone Health, Indiana University Health, and Columbia University Medical Center), telemedicine surveys were administered to 159 patients. Neuro-ophthalmologists completed 157 surveys; each of these were linked to a single patient visit. Patient surveys consisted of 5 questions regarding visit preparation, satisfaction, challenges, and comfort. The physician survey included 4 questions that focused on ability to gather specific clinical information by history and examination. RESULTS: Among 159 patients, 104 (65.4%) reported that they were satisfied with the visit, and 149 (93.7%) indicated that they were comfortable asking questions. Sixty-eight (73.9%) patients found the instructions provided before the visit easy to understand. Potential areas for improvement noted by patients included more detailed preparation instructions and better technology (phone positioning, Internet connection, and software). More than 87% (137/157) of neuro-ophthalmologists surveyed reported having performed an examination that provided enough information for medical decision-making. Some areas of the neuro-ophthalmologic examination were reported to be easy to conduct (range of eye movements, visual acuity, Amsler grids, Ishihara color plates, and pupillary examination). Other components were more difficult (saccades, red desaturation, visual fields, convergence, oscillations, ocular alignment, and smooth pursuit); some were especially challenging (vestibulo-ocular reflex [VOR], VOR suppression, and optokinetic nystagmus). Clinicians noted that virtual health visits were limited by patient preparation, inability to perform certain parts of the examination (funduscopy and pupils), and technological issues. CONCLUSIONS: Among virtual neuro-ophthalmology visits evaluated, most offer patients with appointments that satisfy their needs. Most physicians in this cohort obtained adequate clinical information for decision-making. Even better technology and instructions may help improve aspects of virtual health visits.

Optic pathway glioma of childhood.

PURPOSE OF REVIEW: Optic pathway gliomas (OPG) are the most common tumor of the anterior visual pathway and can involve the optic nerve, chiasm, tract, and optic radiations. They are typically benign lesions, often pilocytic astrocytomas, which are diagnosed in childhood. We review the epidemiology, clinical presentation, diagnosis, and management of these lesions in patients with and without neurofibromatosis type 1 (NF-1). RECENT FINDINGS: Most commonly, patients diagnosed with OPG have NF-1 especially if the lesions are bilateral. Such lesions tend to have a relatively indolent course and at least 50% of patients have no evidence of visual loss. Rarely, children without NF-1 may sporadically develop OPG with such lesions often having a more aggressive nature and greater propensity for visual dysfunction. The gold standard for diagnosis and follow-up are thorough neuro-ophthalmic examinations with specific attention to visual acuity. Management must be individualized and may comprise conservative follow-up, chemotherapy, radiation and/or surgical intervention. SUMMARY: OPG may range in their behavior based upon the nature of the tumor (NF-1 or sporadic). Current guidelines recommend following patients with regular clinical examinations. Management of these lesions is highly individualized based upon the nature and extent of the lesion, visual function and side-effect profile of the treatment. Clinicians should be aware of the available options to determine which may be best suited for their patient.

Cobalt-Chromium Metallosis With Normal Electroretinogram.

BACKGROUND: Ocular cobalt toxicity is a rare phenomenon reported with increased frequency due to the rise of cobalt-chromium metal hip implants. We report the case of a 66-year-old previously healthy man who developed decreased vision due to cobalt-chromium toxicity from a metal-on-metal hip arthroplasty. Our objective was to determine whether the origin of his visual loss was due to toxicity of the optic nerve, of the retina, or of both. METHODS: Ocular examination, 10-2 SITA-Standard Humphrey Visual Field (VF), standard full-field electroretinogram (ERG) as indicated by the International Society for Clinical Electrophysiology of Vision (ISCEV), multifocal electroretinogram (mfERG), multifocal visual evoked potentials (mfVEP), and optical coherence tomography (OCT) were conducted. RESULTS: Ocular examination revealed decreased visual acuity, poor color vision, normal funduscopy, and cecocentral scotomas on VF testing. Because his right eye was amblyopic since childhood, test results from only the left eye are shown. Electrophysiology studies revealed an ISCEV standard full-field ERG with photopic and scotopic responses within normal limits, mfERG with amplitudes and latencies within normal limits, and mfVEP with latencies within normal limits, but with decreased central amplitudes. Peripapillary and macular OCT showed retinal nerve fiber layer and retinal ganglion cell-inner plexiform layer thickness within normal limits. CONCLUSION: Because decreased color vision and cecocentral scotoma on 10-2 VF are most consistent with toxic optic neuropathy, and decreased central amplitudes on mfVEP are suggestive of neural dysfunction, we hypothesize that our patient presented with an early stage of optic nerve toxicity that was not yet apparent as a structural abnormality on OCT.

Downbeat nystagmus secondary to familial hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis is a rare autosomal recessive disorder characterized by severe inflammation induced by defective natural killer cell function, which triggers a state of highly stimulated but ineffective immune response. This disorder can affect multiple organ systems, and neurologic manifestations include irritability, seizures, impaired consciousness, meningismus, and cranial nerve palsies. We describe a unique case of hemophagocytic lymphohistiocytosis in which downbeat nystagmus developed due to cerebellar swelling with compression of the cervicomedullary junction.

The Peripapillary Retina - A Common Juncture in Stargardt Disease and Idiopathic Intracranial Hypertension.

PURPOSE: To present the multimodal imaging and functional exam findings in a case of combined Stargardt disease and idiopathic intracranial hypertension. METHODS: The patient was evaluated with multimodal imaging including color fundus photography, short wavelength autofluorescence, spectral domain optical coherence tomography as well as functional testing such as Humphrey visual fields and full-field electroretinogram. RESULTS: A 35-year-old woman was referred for evaluation of bilateral transient visual obscurations over the course of 2 months. Optic disc edema was observed in both eyes as well as a bulls-eye maculopathy with pisciform flecks. Magnetic resonance imaging and subsequent lumbar puncture confirmed a diagnosis of idiopathic intracranial hypertension. Fundus autofluorescence demonstrated hyperautofluorescent flecks surrounding both the macula and the disc. Genetic testing and full-field electroretinogram confirmed a diagnosis of Stargardt disease. Notably, the peripapillary retina was not spared as is frequently seen in Stargardt disease, possibly due to the impact of disc edema in the area. The patient was treated with increasing doses of acetazolamide and familial testing for Stargardt disease was recommended. CONCLUSION: Both Stargardt disease and idiopathic intracranial hypertension are separately rare diseases with common anatomic intersection at the peripapillary retina. Stargardt disease typically spares the peripapillary retina; however, in the present case shows some evidence of peripapillary involvement. This finding suggests some relationship between disc edema due to idiopathic intracranial hypertension and the natural history of Stargardt disease.

Osteopathia striata with cranial sclerosis causing a compressive optic neuropathy.

BACKGROUND: Osteopathia striata combined with cranial sclerosis (OS-CS) is an inherited skeletal dysplasia that manifests with macrocephaly, orofacial abnormalities, thickened craniofacial bones, and vertically oriented radiodensities of the long bones. CASE REPORT: Here, we present a severe case of OS-CS in a 4-year-old girl causing optic neuropathy as shown by radiographic evidence, ophthalmic findings, and histopathology. Previous genetic testing in this patient revealed a de novo heterozygous mutation in AMER1 (c.1057C>T, p.Arg353Ter). Although the patient had a pre-existing, appropriately functioning, ventriculoperitoneal (VP) shunt, a subsequent MRI of the brain and orbits showed narrowing of the bilateral optic nerve canals secondary to osseous thickening causing bilateral optic nerve atrophy, worse on the left. The patient underwent staged bilateral orbital osteotomies, optic canal decompression, and bilateral frontal craniotomy, and at 11 months postoperatively, her vision remained stable. Conclusions: While up to 50% of the patients with OS-CS may experience hearing loss due to cranial nerve compression, we present a case of severe visual loss secondary to OS-CS-associated optic nerve compression.

Lyme-associated orbital inflammation presenting as painless subacute unilateral ptosis.

A 90-year-old woman presented with subacute painless left ptosis. Examination of the left eye revealed ptosis with loss of the superior eyelid sulcus, 2 mm of proptosis, mild tenderness with retropulsion, and optic disc edema. Levator function and extraocular movements were normal, and there was no relative afferent pupillary defect. MRI demonstrated thickening of the extraocular muscles in the left orbit with lacrimal gland enlargement and mild enhancement of the optic nerve sheath. Serology revealed a positive enzyme-linked immunosorbent assay for Lyme antibodies and a positive Western blot of Lyme IgG titer. The patient recalled a tick bite 6 months earlier, at which time Lyme serologies were negative. After 3 weeks of intravenous ceftriaxone, she had a significant improvement and a full recovery by 3 months. Lyme disease should be included in the differential diagnosis of orbital inflammation, especially in Lyme-endemic areas.

Arterial sheathing in Leber hereditary optic neuropathy.

PURPOSE: Presentation of a case of Leber hereditary optic neuropathy (LHON) with arterial sheathing eleven years after initial loss of vision. OBSERVATIONS: A 46-year-old female was referred for re-evaluation of Leber hereditary optic neuropathy. She first noticed rapid painless loss of vision eleven years prior. Fundus imaging performed at that time did not demonstrate arterial sheathing. Genetic testing revealed the presence of the LHON 11778 G-A mitochondrial mutation. Laboratory values were within normal limits save for angiotensin-converting enzyme elevated to 69 U/L. Eleven years later, visual acuity was count fingers at 12 feet with complete loss of color vision. Funduscopic examination of the optic nerve revealed bilateral pallor, sheathing of the retinal arteries, diffuse vessel narrowing, and tortuous retinal vessels. CONCLUSIONS AND IMPORTANCE: We present a case of LHON that demonstrates retinal arterial sheathing and possibly broadens the spectrum of LHON fundus findings.

Abnormal multifocal ERG findings in patients with normal-appearing retinal anatomy.

To evaluate eyes with abnormal visual fields and multifocal electroretinograms (mfERGs) but normal-appearing frequency-domain optical coherence tomography (fdOCT) scans, the thicknesses of the outer retinal layers were measured. A total of 25 eyes from 17 patients, including 15 eyes previously tested (Dale et al. in Doc Ophthalmol 120(2):175-186, 2009) were examined. All patients were evaluated with standard automated perimetry (SAP) using the 24-2 and/or 10-2 program (Zeiss Meditec), mfERG with 103 hexagons (Veris, EDI), and fdOCT imaging (3DOCT-2000, Topcon) with scans of the macula. All patients had reliable visual fields showing macular defects and good quality mfERG and fdOCT results. The mfERG results were classified as abnormal based on decreased amplitudes and/or increased latencies corresponding to the abnormal visual field. Based on visual inspection, three experienced observers classified the fdOCT scans as normal or inconclusive, as opposed to clearly abnormal. Retinal layers of the fdOCT scans were manually segmented with the aid of a computer program and compared to mean thicknesses from 20 controls. The thicknesses of the outer segment plus retinal pigment epithelium, total receptor, and inner nuclear layers were measured. Quantitative analysis of fdOCT scans demonstrated thinning of the outer retina in some scans that was not readily apparent on visual inspection. One or more of the outer retinal layers was significantly thinner in 15 of the 25 eyes. The absence of significant thinning in the other 10 eyes represents instances in which functional loss measured by visual fields and mfERGs can precede clear structural changes on fdOCT.

Occult temporal arteritis in a 54-year-old man.

A 54-year-old white man with a remote history of pars planitis reported transient monocular visual loss (TMVL) in the left eye on standing. The following week he experienced multiple similar episodes. He denied associated systemic symptoms. Initial examination showed old peripheral retinal vascular sheathing and delayed retinal arterial filling time. Complete blood count, erythrocyte sedimentation rate, and MRI studies of the head and neck were normal. One week later, there were multiple cotton wool spots in the posterior pole, a relative afferent pupillary defect, and subtle visual field loss in the left eye. Evaluation for infectious, inflammatory, or embolic etiologies was nonrevealing. Biopsy of the prominent but nontender temporal arteries showed granulomatous inflammation, fragmentation, and duplication of the internal elastic lamina consistent with the temporal arteritis (TA). Radiography and MRI of the chest revealed dilation of the ascending aorta. The patient began treatment with high-dose oral steroids with resolution of his TMVL and retinal cotton wool spots and decrease in the size of the temporal arteries. Our case demonstrates the importance of considering TA in the setting of TMVL, visual loss, cotton wool spots, or dilated nontender temporal arteries in an otherwise asymptomatic patient even with normal inflammatory markers. Long-term follow-up is essential in unusual cases such as this one, given the high risk of ocular and systemic morbidity with TA.

A comparison of multifocal ERG and frequency domain OCT changes in patients with abnormalities of the retina.

To compare the ability of the multifocal electroretinogram (mfERG) and frequency domain optical coherence tomography (fdOCT) to detect retinal abnormalities. A total of 198 eyes (100 patients) were referred by neuro-ophthalmologists to rule out a retinal etiology of visual impairment. All patients were evaluated with static automated perimetry (SAP) (Humphrey Visual Field Analyzer; Zeiss Meditec), mfERG (Veris, EDI) and fdOCT (3D-OCT 1000, Topcon). The mfERG was performed with 103 scaled hexagons and procedures conforming to ISCEV standards (Hood DC et al. (2008) Doc Ophthalmol 116(1):1-11). The fdOCT imaging included horizontal and vertical line scans through the fovea. Local mfERG and fdOCT abnormalities were compared to local regions of visual field sensitivity loss measured with SAP and categorized as normal/inconclusive or abnormal. 146 eyes were categorized as normal retina on both fdOCT and mfERG. The retina of 52 eyes (36 patients) was categorized as abnormal based upon mfERG and/or fdOCT. Of this group, 25 eyes (20 patients) were abnormal on both tests. However, 20 eyes (13 patients) were abnormal on mfERG, while the fdOCT was normal/inconclusive; and 7 eyes (7 patients) had normal or inconclusive mfERG, but abnormal fdOCT. Considerable disagreement exists between these two methods for detection of retinal abnormalities. The mfERG tends to miss small local abnormalities that are detectable on the fdOCT. On the other hand, the fdOCT can appear normal in the face of clearly abnormal mfERG and SAP results. While improved imaging and analysis may show fdOCT abnormalities in some cases, in others early damage may not appear on structural tests.

Retinal nerve fiber structure versus visual field function in patients with ischemic optic neuropathy. A test of a linear model.

PURPOSE: To test a linear model relating the regional loss in retinal nerve fiber (RNFL) thickness to the corresponding regional loss in sensitivity with data from patients with previous anterior ischemic optic neuropathy (AION). DESIGN: Case-control study. PARTICIPANTS: Twenty-four individuals with AION and 20 with normal vision were tested. The time since the AION attack ranged from 5.2 months to more than 20.3 years (median, 2.95 years). METHODS: Eyes were tested with standard automated perimetry (SAP) and with optical coherence tomography (OCT), both RNFL thickness scans. The average RNFL thickness of the inferior and superior disc sectors was plotted against the average total deviations (linear units) of the corresponding superior and inferior arcuate field regions, and a linear model was fitted. According to the model, the RNFL thickness R=s(o)T+b, (1), where T is the relative SAP sensitivity loss (on a linear scale; e.g., for -3 dB, T = 0.5), s(o) is the RNFL thickness attributable to axons in the healthy or normal state (T = 1.0), and b is the residual RNFL measured when all sensitivity and axons are lost. MAIN OUTCOME MEASURES: Optical coherence tomography RNFL thickness and SAP sensitivity. RESULTS: The data from the AION patients resembled the data from glaucoma patients previously tested and were described by the linear model. For patients with SAP losses of more than -10 dB in the arcuate region, the RNFL thickness provided an estimate of residual RNFL thickness, b. The median value of b (45.5 microm) was similar to the value for patients with glaucoma. It varied among individuals (range, 30.4-63.3 microm), showing a very weak correlation with patient's age (r = 0.30) and the time since the AION episode (r = 0.26), but an excellent correlation (r(2) = 0.94; P<0.01) with the value of s(o), estimated from the unaffected eyes. CONCLUSIONS: The relationship between a structure (OCT RNFL thickness) and function (SAP sensitivity loss) is the same for patients with AION and glaucoma and can be approximated by a simple linear model. The model may provide a framework for identifying those patients with ganglion cell axons that are malfunctioning but are alive.

Colored floaters as a manifestation of digoxin toxicity.

PURPOSE: Since its report in one patient more than 70 years ago, digitalis-induced colored muscae volitantes have not surfaced again in the literature. We report here a case of digoxin induced colored floaters. OBSERVATIONS: An 89-year-old man on 0.25 mg digoxin daily developed visual hallucinations and colored floaters. He had floaters in the past but now they were in various colors including yellow, green, blue and red, though predominantly in yellow. These "weirdly" shaped little particles wiggled around as if in a viscous solution and casted shadows in his vision. He also saw geometric shapes, spirals, and cross hatch patterns of various colors that moved and undulated, especially on wallpaper. Ophthalmic examination revealed reduced visual acuity, poor color vision especially in his left eye, along with central depression on Amsler grid and Humphrey visual field in his left eye. Discontinuation of digoxin resulted in complete resolution of his visual symptoms. On subsequent ophthalmic examination, the patient's visual acuity, field testing and color vision improved and he had normal Amsler grid test results. CONCLUSIONS AND IMPORTANCE: Colored floaters may occur in patients taking cardiac glycosides but this association has not been explored. Unlike optical illusions and visual hallucinations, floaters are entoptic phenomena casting a physical shadow upon the retina and their coloring likely arise from retinal dysfunction. Colored floaters may be a more common visual phenomenon than realized.