Extraction of three-dimensional shapes in glaucoma patients in response to monocular depth cues.

PURPOSE: To assess the impact of glaucoma on perceiving three-dimensional (3D) shapes based on monocular depth cues. STUDY DESIGN: Clinical observational study. METHODS: Twenty glaucoma patients, subjected to binocular visual-field sensitivity (binocular-VFS) tests using a Humphrey Visual Field Analyzer, and 20 age-matched healthy volunteers, underwent two tasks: identifying the nearest vertex of a 3D shape using monocular shading (3D-SfS), texture (3D-SfT), or motion (3D-SfM) cues, and distinguishing elementary one-dimensional (1D) features of these cues. The association of the visual-field index (VFI) of binocular-VFS with 3D shape perception in glaucoma patients was also examined. RESULTS: Glaucoma patients demonstrated reduced accuracy in distinguishing 1D luminance brightness and a larger "error-in-depth" between the perceived and actual depths for 3D-SfM and 3D-SfS compared to healthy volunteers. Six glaucoma patients with a 100% VFI for binocular-VFS exhibited a similar error-in-depth to the other fourteen glaucoma patients; they had a larger error-in-depth for 3D-SfM compared to healthy volunteers. No correlation between the error-in-depth values and the VFI values of binocular-VFS was observed. CONCLUSIONS: The 3D shape perception in glaucoma patients varies based on the depth cue's characteristics. Impaired 1D discrimination and larger thresholds for 3D-SfM in glaucoma patients with a 100% VFI for binocular-VFS indicate more pronounced perceptual deficits of lower-level elementary features for 3D-SfS and higher-level visual processing of 3D shapes for 3D-SfM. The effects of the location and degree of binocular visual-field defects on 3D shape perception remain to be elucidated. Our research provides insights into the 3D shape extraction mechanism in glaucoma.

Clinically Diagnosed Occult Macular Dystrophy Habouring an m.14502T>C Mitochondrial DNA Mutation Associated with Leber's Hereditary Optic Neuropathy: Case Report and Literature Review.

A 29-year-old female with no family history presented with bilateral progressive blurred vision. Her symptoms appeared at 12-years-old and her visual acuity had since deteriorated from 0.6 to 0.2 bilaterally with decreased critical flicker frequency and bilateral central scotomas. She did not have a relative afferent pupillary defect. Fundoscopy revealed no distinct disc hyperaemia, atrophy, or peripapillary telangiectatic vessels. The retinal nerve fibre layer appeared normal on optical coherence tomography in each eye; however, loss of the interdigitation zone and the disruption of the ellipsoid zone at the fovea were observed in both eyes. Multifocal electroretinography revealed decreased amplitudes at both macula regions. Mitochondrial deoxyribonucleic acid analysis identified an m.14502T>C mutation, one of the primary mutations causing Leber's hereditary optic neuropathy (LHON). Despite the presence of a marked LHON mutation, however, she was clinically diagnosed as having an occult macular dystrophy. There have only been five previous case reports, all of which were sporadic, which detail the clinical characteristics of the m.14502T>C mutation. The m.14502T>C phenotype is somewhat consistent with that of the other major mutations, including young onset, bilateral progressive visual impairment, and a typical LHON fundus. Nevertheless, m.14502T>C alone has an extremely low penetrance and its phenotype may be minimal or subclinical, as seen in our case. Since little is known about the clinical course of the m.14502T>C mutation it may be possible that the LHON phenotype may appear in later stages of life. Moreover, m.14502T>C may function as a modifier gene, which alters the phenotype of other coexisting major LHON mutations, including penetrance and the severity of the disease, through synergistic effects.

Agreement in the detection of chiasmal and postchiasmal visual field defects between imo binocular random single-eye test and Humphrey monocular test.

PURPOSE: To compare the ability of imo binocular random single-eye test (BRSET) to detect visual field (VF) defects due to chiasmal and postchiasmal lesions (C/PCLs) with a Humphrey Field Analyzer (HFA) monocular test. STUDY DESIGN: Prospective multicenter study METHODS: This study enrolled 40 patients with C/PCLs and measured their VFs using both imo BRSET and HFA monocular test. The VFs were classified into three groups using the cluster criterion: 1) bitemporal group, 2) homonymous group, and 3) others. The agreement and correlation of VF results between imo and HFA were analyzed using the Bland-Altman plot and Spearman correlation coefficient. RESULTS: The VFs of 34 patients were analyzed and classified. There were 13 patients in the bitemporal, 6 in the homonymous, and 15 in the others group. BRSET showed a significantly shorter test duration than HFA. The imo systematically yielded a lower sensitivity than HFA. The average sensitivity at each test location correlated well between the perimeters in all groups, with the correlation coefficients ranging from 0.89 to 0.98. Bland-Altman plots showed wider limits of agreement in the affected quadrants compared to the unaffected quadrants in the bitemporal and homonymous groups. The fixation loss rate did not differ between the perimeters, but there were significant differences in the false positive and false negative rates between perimeters. CONCLUSION: BRSET detected VF defects due to C/PCLs as accurately as the HFA monocular test with a shorter test duration.

Late-onset Ocular Toxicity Presenting as Uveitis Caused by Crizotinib.

A 64-year-old female suffering from lung cancer was treated with crizotinib. Two years later, whitish massive optic disc oedema was observed in the right eye. The fluorescein angiography results were suggestive of uveitis but also revealed leakage from the optic disc, retinal veins, and capillaries in the posterior retina and the periphery. These findings remained for over a year without deterioration of vision and disappeared immediately after crizotinib was replaced with alectinib. Late-onset ocular toxicity by crizotinib was strongly suspected, given the clinical course. This is the first report precisely documenting crizotinib-induced morphological changes in the optic disc and retina.

Investigation of intraocular pressure of the anterior chamber and vitreous cavity of porcine eyes via a novel method.

To evaluate a new method of measuring the intraocular pressure (IOP) in the vitreous cavity. IOPs in the anterior chamber and vitreous cavities of 24 porcine eyes (12 eyes with lenses and 12 eyes without lenses) were measured directly, continuously, and simultaneously. We used a needle as a part of the pressure sensor to measure the anterior chamber IOP and a disk-shaped sensor to measure the vitreous cavity IOP. A significant group-by-condition interaction on the vitreous cavity IOP between the two groups (phakia and aphakia) and four conditions of anterior chamber IOP were observed (F[3,258] = 5.8564, p < 0.001). A positive correlation was observed between the vitreous cavity IOP and anterior chamber IOP in both the phakia group (R = 0.96, p < 0.001) and the aphakia group (R = 0.97, p < 0.001). No significant correlation was observed between the ΔIOPv-a (vitreous cavity IOP - anterior chamber IOP) and anterior chamber IOP in either group (phakia group: R = - 0.18, p = 0.034; aphakia group: R = - 0.029, p = 0.73). The vitreous cavity IOP measured with the new sensor was well-correlated with the anterior chamber IOP in the physiological range tested.

Clinical Characteristics of Neuronal Intranuclear Inclusion Disease-Related Retinopathy With CGG Repeat Expansions in the NOTCH2NLC Gene.

Purpose: To report the ocular characteristics of neuronal intranuclear inclusion disease (NIID)-related retinopathy with expansion of the CGG repeats in the NOTCH2NLC gene. Methods: Seven patients from six families (aged 66-81 years) diagnosed with adult-onset NIID were studied. Ophthalmologic examinations, including the best-corrected visual acuity (BCVA), Goldmann perimetry, fundus photography, fundus autofluorescence (FAF) imaging, optical coherence tomography (OCT), and full-field electroretinography (ERGs), were performed. The expansion of the CGG repeats in the NOTCH2NLC gene was determined. Results: All patients had an expansion of the CGG repeats (length approximately from 330-520 bp) in the NOTCH2NLC gene. The most common symptoms of the five symptomatic cases were reduced BCVA and night blindness. The other two cases did not have any ocular symptoms. The decimal BCVA varied from 0.15 to 1.2. Goldmann perimetry was constricted in all four cases tested; physiological blind spot was enlarged in two of the cases. The FAF images showed an absence of autofluorescence (AF) around the optic disc in all cases and also showed mild hypo-AF or extinguished AF in the midperiphery. In all cases, the OCT images showed an absence of the ellipsoid zone of the photoreceptors in the peripapillary region, and hyperreflective dots were also present between the retinal ganglion cell layer and outer nuclear layer. The macular region was involved in the late stage of the retinopathy. The full-field ERGs showed rod-cone dysfunction. Conclusions: Patients with adult-onset NIID with CGG repeats expansions in the NOTCH2NLC gene had similar ophthalmologic features, including rod-cone dysfunction with progressive retinal degeneration in the peripapillary and midperipheral regions. The primary site is most likely the photoreceptors. Because the ocular symptoms are often overlooked due to dementia and occasionally precede the onset of dementia, detailed ophthalmological examinations are important for the early diagnosis of NIID-related retinopathy.

A parietal region processing numerosity of observed actions: An FMRI study.

When observing others' behavior, it is important to perceive not only the identity of the observed actions (OAs), but also the number of times they were performed. Given the mounting evidence implicating posterior parietal cortex in action observation, and in particular that of manipulative actions, the aim of this study was to identify the parietal region, if any, that contributes to the processing of observed manipulative action (OMA) numerosity, using the functional magnetic resonance imaging technique. Twenty-one right-handed healthy volunteers performed two discrimination tasks while in the scanner, responding to video stimuli in which an actor performed manipulative actions on colored target balls that appeared four times consecutively. The subjects discriminated between two small numerosities of either OMAs ("Action" condition) or colors of balls ("Ball" condition). A significant difference between the "Action" and "Ball" conditions was observed in occipito-temporal cortex and the putative human anterior intraparietal sulcus (phAIP) area as well as the third topographic map of numerosity-selective neurons at the post-central sulcus (NPC3) of the left parietal cortex. A further region of interest analysis of the group-average data showed that at the single voxel level the latter area, more than any other parietal or occipito-temporal numerosity map, favored numerosity of OAs. These results suggest that phAIP processes the identity of OMAs, while neighboring NPC3 likely processes the numerosity of the identified OAs.

Cancer-associated Retinopathy Developing After 10 Years of Complete Breast Cancer Remission.

A 73-year-old female with a past medical history of breast cancer, who 10 years earlier experienced complete remission, complained of bilateral visual field disturbances and photopsia, 2 months prior. Tumour recurrence and metastatic lesions were not found during the medical examination, but antibodies against recoverin were detected in her serum. Despite immunosuppressive treatment with prednisolone and plasmapheresis, rapid and diffuse degeneration of the patient's photoreceptors and deterioration of her visual field were observed. This is a rare case of cancer-associated retinopathy with a long interval (10 years) between the diagnosis of the malignancy and visual loss.

Binocular stereo acuity affects monocular three-dimensional shape perception in patients with strabismus.

BACKGROUND/AIMS: To evaluate the perception of three-dimensional (3D) shape in patients with strabismus and the contributions of stereopsis and monocular cues to this perception. METHODS: Twenty-one patients with strabismus with and 20 without stereo acuity as well as 25 age-matched normal volunteers performed two tasks: (1) identifying the closest vertices of 3D shapes from monocular shading (3D-SfS), texture (3D-SfT) or motion cues (3D-SfM) and from binocular disparity (3D-SfD), (2) discriminating 1D elementary features of these cues. RESULTS: Discrimination of the elementary features of luminance, texture and motion did not differ across groups. When the distances between reported and actual closest vertices were resolved into sagittal and frontoparallel plane components, sagittal components in 3D-SfS and frontoparallel components in 3D-SfT indicated larger errors in patients with strabismus without stereo acuity than in normal subjects. These patients could not discriminate one-dimensional elementary features of binocular disparity. Patients with strabismus with stereo acuity performed worse for both components of 3D-SfD and frontoparallel components of 3D-SfT compared with normal subjects. No differences were observed in the perception of 3D-SfM across groups. A comparison between normal subjects and patients with strabismus with normal stereopsis revealed no deficit in 3D shape perception from any cue. CONCLUSIONS: Binocular stereopsis is essential for fine perception of 3D shape, even when 3D shape is defined by monocular static cues. Interaction between these cues may occur in ventral occipitotemporal regions, where 3D-SfS, 3D-SfT and 3D-SfD are processed in the same or neighbouring cortical regions. Our findings demonstrate the perceptual benefit of binocular stereopsis in patients with strabismus.

Anti-Collapsing Response-Mediating Protein-5 Antibody-Positive Paraneoplastic Perioptic Neuritis without Typical Neurological Symptoms.

A 68-year-old male presented with blurred vision in both eyes. Ophthalmoscopy revealed bilateral prominent disc swelling and vitritis. No systematic neurological symptoms were observed. Magnetic resonance imaging revealed bilateral meningeal enhancement of the optic nerve. Small cell carcinoma was found, and antibodies against collapsing response-mediating protein-5 (CRMP-5) were detected in the serum. Ophthalmological manifestations disappeared during a decrease in tumour size with treatment for the malignancy. This case report describes this rare case of anti-CRMP-5 antibody-positive paraneoplastic perioptic neuritis without neurological symptoms, showing that prompt diagnosis and timely treatment of the underlying tumour are crucial to prevent increased levels of autoantibodies and irreversible damage to the nervous system.

Estimating the Binocular Visual Field of Glaucoma Patients With an Adjustment for Ocular Dominance.

PURPOSE: To investigate whether it is possible to improve estimation of the binocular visual field (VF) using monocular sensitivities on a linear scale adjusted for ocular dominance. METHODS: Monocular and binocular VF measurements were evaluated using the Humphrey Field Analyzer (HFA; 24-2 Swedish Interactive Threshold Algorithm standard program) in 60 eyes of 30 patients with open angle glaucoma. Ocular dominance was measured twice in each patient and the average value was used. Measured binocular sensitivity was then predicted based on monocular measurements using the "better sensitivity" integrated visual field (IVF) method, monocular sensitivity summation methods on the dB scale, linear scale (1/Lambert), and finally monocular sensitivity summation methods on the linear scale adjusted for the ocular dominance. RESULTS: The absolute prediction error with the linear scale summation method (mean ± SD: 3.11 ± 4.00) was significantly smaller than the IVF method (3.15 ± 4.09; P = 0.014). Further, the absolute prediction error for the ocular dominance adjusted method (3.10 ± 3.99) was significantly smaller than the nonadjusted linear scale summation method (P = 0.014). The absolute prediction error associated with the dB scale summation method was significantly larger than any other method (8.15 ± 5.06; P < 0.0001). CONCLUSIONS: The most accurate estimation of binocular sensitivity was achieved using the linear monocular sensitivity summation model adjusted for ocular dominance.

Amblyopia Associated with Congenital Facial Nerve Paralysis.

The association between congenital facial paralysis and visual development has not been thoroughly studied. Of 27 pediatric cases of congenital facial paralysis, we identified 3 patients who developed amblyopia, a visual acuity decrease caused by abnormal visual development, as comorbidity. These 3 patients had facial paralysis in the periocular region and developed amblyopia on the paralyzed side. They started treatment by wearing an eye patch immediately after diagnosis and before the critical visual developmental period; all patients responded to the treatment. Our findings suggest that the incidence of amblyopia in the cases of congenital facial paralysis, particularly the paralysis in the periocular region, is higher than that in the general pediatric population. Interestingly, 2 of the 3 patients developed anisometropic amblyopia due to the hyperopia of the affected eye, implying that the periocular facial paralysis may have affected the refraction of the eye through yet unspecified mechanisms. Therefore, the physicians who manage facial paralysis should keep this pathology in mind, and when they see pediatric patients with congenital facial paralysis involving the periocular region, they should consult an ophthalmologist as soon as possible.

Anti-NMDA receptor encephalitis associated with transient cerebral dyschromatopsia, prosopagnosia, and lack of stereopsis.

A 20-year-old woman suffered from anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and was treated with removal of an ovarian teratoma and retroperitoneal ganglioneuroma in addition to immunotherapy. She was incapable of face recognition, had difficulty with object recognition, and lacked color sensation and stereo perception during recovery. These symptoms were transient and completely resolved over 4 months. Our report documents additional aspects of visual impairment associated with anti-NMDAR encephalitis and suggests that the disease can lead to diffuse cerebral dysfunction including the cortical visual system.

Juvenile onset of ocular hypertension associated with de novo nail-patellar syndrome.

PURPOSE: To report a rare case of juvenile-onset ocular hypertension (OHT) associated with nail-patellar syndrome (NPS). METHOD: Clinical data of the patient were reviewed retrospectively. RESULTS: A 29-year-old woman was referred due to uncontrollable OHT. The OHT was found to be associated with NPS, a disorder characterized by anomalies of nails and skeletal bones. The patient had a history of high recorded intraocular pressure (IOP), beginning when she was 13 years old. Trabeculotomy was performed in both eyes due to uncontrollable high IOP. Postoperatively, the IOP has been well controlled without deterioration of optic disc appearance or visual field defects. CONCLUSION: When evaluating patients presenting with juvenile-onset OHT, clinicians should be aware of NPS as a differential diagnosis.

Recurrent Optic Neuropathy Caused by a Mucocele of the Anterior Clinoid Process after a 5-Year Remission: A Case Report and Literature Review.

A 32-year-old male presented with acute left vision loss during a second recurrence of optic neuropathy. Steroid pulse therapy had been effective in both the first episode 9 years previously and the first recurrence 5 years previously. Magnetic resonance imaging demonstrated an anterior clinoid process mucocele compressing the optic nerve. Although surgical treatment was performed, improvement was limited. This report indicates that steroid pulse therapy could be an alternative treatment to obtain temporary remission, but surgical treatment should be considered to prevent irreversible neurological deficits. This paper also presents a review of the literature on anterior clinoid process mucoceles.

Multisynaptic inputs from the medial temporal lobe to V4 in macaques.

Retrograde transsynaptic transport of rabies virus was employed to undertake the top-down projections from the medial temporal lobe (MTL) to visual area V4 of the occipitotemporal visual pathway in Japanese monkeys (Macaca fuscata). On day 3 after rabies injections into V4, neuronal labeling was observed prominently in the temporal lobe areas that have direct connections with V4, including area TF of the parahippocampal cortex. Furthermore, conspicuous neuron labeling appeared disynaptically in area TH of the parahippocampal cortex, and areas 35 and 36 of the perirhinal cortex. The labeled neurons were located predominantly in deep layers. On day 4 after the rabies injections, labeled neurons were found in the hippocampal formation, along with massive labeling in the parahippocampal and perirhinal cortices. In the hippocampal formation, the densest neuron labeling was seen in layer 5 of the entorhinal cortex, and a small but certain number of neurons were labeled in other regions, such as the subicular complex and CA1 and CA3 of the hippocampus proper. The present results indicate that V4 receives major input from the hippocampus proper via the entorhinal cortex, as well as "short-cut" pathways that bypass the entorhinal cortex. These multisynaptic pathways may define an anatomical basis for hippocampal-cortical interactions involving lower visual areas. The multisynaptic input from the MTL to V4 is likely to provide mnemonic information about object recognition that is accomplished through the occipitotemporal pathway.

Segregated pathways carrying frontally derived top-down signals to visual areas MT and V4 in macaques.

The bottom-up processing of visual information is strongly influenced by top-down signals, at least part of which is thought to be conveyed from the frontal cortex through the frontal eye field (FEF) and the lateral intraparietal area (LIP). Here we investigated the architecture of multisynaptic pathways from the frontal cortex to the middle temporal area (MT) of the dorsal visual stream and visual area 4 (V4) of the ventral visual stream in macaques. In the first series of experiments, the retrograde trans-synaptic tracer, rabies virus, was injected into MT or V4. Three days after rabies injections, the second-order (disynaptically connected) neuron labeling appeared in the ventral part of area 46 (area 46v), along with the first-order (monosynaptically connected) neuron labeling in FEF and LIP. In the MT-injection case, second-order neurons were also observed in the supplementary eye field (SEF). In the next series of experiments, double injections of two fluorescent dyes, fast blue and diamidino yellow, were made into MT and V4 to examine whether the frontal inputs are mediated by distinct or common neuronal populations. Virtually no double-labeled neurons were observed in FEF or LIP, indicating that separate neuronal populations mediate the frontal inputs to MT and V4. The present results define that the multisynaptic frontal input to V4 arises primarily from area 46v, whereas the input to MT arises from not only area 46v but also SEF, through distinct FEF and LIP neurons. Segregated pathways from the frontal cortex possibly carry the functionally diverse top-down signals to each visual stream.

Clinical features and haplotype analysis of newly identified Japanese patients with gelsolin-related familial amyloidosis of Finnish type.

Familial amyloidosis of the Finnish type (FAF) is an autosomal dominant form of systematic amyloidosis characterized by lattice corneal dystrophy, cranial neuropathy, and cutis laxa. Although FAF has been frequently found in the Finnish population, FAF is a considerably rare disorder in other regions. In this study, we examined the clinical characteristics as well as the haplotypes of six Japanese patients with FAF from five families. They showed the typical clinical presentations of FAF, but we found a broad range of ages at onset of neurological symptoms. All members had the c.654G>A mutation in GSN. To evaluate the disease haplotypes, high-density single-nucleotide polymorphism (SNP) arrays were used and disease-relevant haplotypes were reconstructed. Haplotype analysis in the four apparently unrelated families suggested a common founder haplotype. In a sporadic FAF patient, however, the haplotype was dissimilar to the founder haplotype. The present study demonstrated that a founder mutation in most of the Japanese families with FAF, except for a sporadic patient in whom a de novo mutation event was suggested as the origin of the mutation.