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To report the findings in 3 cases of bilateral negative electroretinograms (ERGs) with acute onset of photophobia. Retrospective case series. The medical charts of the 3 patients were reviewed. A 43-year-old woman, a 68-year-old woman, and a 41-year-old woman were referred to Nagoya University Hospital. Their main symptom was bilateral acute photophobia. None of the patients had any systemic diseases or specific medical history. The decimal best-corrected visual acuity (> 0.8) and Humphrey visual fields (mean deviation > -3 dB) were relatively well preserved in all 3 patients. The optical coherence tomography (OCT) and fundus autofluorescence findings were essentially normal. Fluorescein angiography showed mild leakage in 1 patient but no abnormality in the other 2 patients. However, the ERGs of the 3 patients had the features of abnormal ERGs found in patients with incomplete congenital stationary night blindness (CSNB). Exome analyses found no pathogenic variants related to known CSNB-related genes. The symptoms and ERGs of the 3 patients have not progressed or recovered after a relatively long follow-up period. The ERG characteristics of 3 patients with bilateral photophobia were similar to those of incomplete CSNB, suggesting post-phototransductional abnormalities. The symptoms and genetic analyses indicated the possibility of an acquired condition rather than a hereditary retinal disease.

Background Evidence-based criteria for the treatment of autoimmune retinopathy (AIR) have not been established. The pathology and clinical features of each antibody causing AIR, and its long-term course are still undetermined. We report our findings in a case of non-paraneoplastic AIR (npAIR) that developed in the fellow eye 10 years after the onset in the first eye. Case presentation Our patient had photophobia in both eyes and a rapidly progressing visual field defect in his right eye at the initial examination. He was diagnosed with non-paraneoplastic AIR based on the clinical findings and immunoblot analyses for anti-retinal antibodies, and he was treated with steroids. Ten years later, a visual field defect developed in the fellow eye, and a diagnosis of npAIR was made. Immunoblot analyses were positive for anti-α-enolase antibodies. He was treated with steroids, immunosuppressants, and plasma exchange. However, the response to the treatment was poor and both eyes eventually became blind. Conclusions As best we know, this is the first case report of npAIR that developed in the fellow eye over 10 years after the development in the first eye. Long-term follow-up and a search for tumor lesions are necessary in cases of npAIR. Further understanding of the long-term course of AIR can contribute to an understanding of the pathology and treatment of npAIR.

Purpose: To describe a Japanese girl with unilateral optic neuritis who was seropositive for the anti-myelin-oligodendrocyte glycoprotein (MOG). Serial recordings of the pattern visual evoked potentials (pVEPs) were made to follow the dynamic changes of the disease activity. Observations: A 5-year-old girl developed a sudden reduction of vision and deep ocular pain in her right eye. On examination at our university hospital, the best-corrected visual acuity (BCVA) was light perception, and a swelling of the optic disc and tortuous vessels at the posterior pole of the right eye were observed. MRI demonstrated that her right optic nerve was hyperintense on short TI inversion recovery (STIR) sequence. A diagnosis of right papillitis was made, and she was treated with steroid pulse therapy followed by a gradual tapering of oral prednisolone. The visual acuity decreased to no light perception and plasmapheresis combined with high-dose intravenous immunoglobulin therapy was performed. The decimal visual acuity rapidly improved and recovered to 1.2, and no recurrence was observed for at least 1 year. On day 19, she was found to be anti-MOG antibody positive and anti-Aquaporin 4 antibody negative. pVEPs were recorded during the course of the disease process which showed the dynamic changes of the physiology of the visual pathways. The implicit times of the N75 and P100 components were prolonged in the right eye in the acute phase. The right visual acuity remained at 1.2 for at least 1 year, but the implicit times of the N75 and P100 components of the pVEPs of the right eye were still prolonged compared to left eye. Conclusion: Our findings indicate a positive relationship between the anti-MOG antibodies-positivity and the prolonged pVEPs. Further analyses of the pVEPs and other clinical findings of the optic neuritis are needed to establish the clinical significance of the anti-MOG antibodies positivity and optic neuritis for the diagnosis, treatment, and prognosis for this disease.

The aim of this study was to evaluate the effects of transcorneal electrical stimulation in subjects with primary open-angle glaucoma. Five eyes of four male subjects with primary open-angle glaucoma (average age: 52.25 ± 14.68 years) were enrolled. The subjects underwent transcorneal electrical stimulation every 3 months according to the following procedure. A Dawson-Trick-Litzkow electrode was placed on the cornea, and biphasic electric current pulses (10 ms, 20 Hz) were delivered using a stimulator (BPG-1,BAK Electronics) and a stimulus isolation unit (BSI-2). A current that evoked a phosphene that the subject perceived in the whole visual area was delivered continuously for 30 min. Humphrey visual field testing was performed after every third transcorneal electrical stimulation treatment. Changes in mean deviation (MD) values were evaluated with a linear regression model. Transcorneal electrical stimulation was performed 18.2 ± 9.4 times over a period of 49.8 ± 23.0 months. The average pretranscorneal electrical stimulation intraocular pressure, best corrected visual acuity, and MD values were 11.8 ± 1.79 mmHg, 0.14 ± 0.19 (logMAR) and -17.28 ± 6.24 dB, respectively. No significant differences were observed in intraocular pressure before and after transcorneal electrical stimulation. However, there was a significant positive linear relationship between changes in MD values and the number of transcorneal electrical stimulation treatments (R2 = 0.176, P = 0.005, Spearman correlation R =0.294, P = 0.008). Transcorneal electrical stimulation treatment may improve glaucomatous visual field defects in subjects with primary open-angle glaucoma. Large-scale studies are necessary to confirm these preliminary findings.

To report the outcome of transcorneal electrical stimulation (TES) of the visual system on long-standing retinal artery occlusion (RAO).Open labeled, case series.Two patients with central RAO (15 and 33 months respectively) and one with branch RAO (26 months) underwent TES therapy. Subjective and objective ophthalmological evaluations were performed before and after the TES. The ages of the patients were 38, 49, and 63 years. The TES (20 Hz biphasic pulses, 30 minutes, up to 1100 uA) was delivered by a bipolar contact lens electrode once a month for 3 months. Perimetric and/or electrophysiological examinations were performed as outcome measures.The visual acuity improved by more than 0.2 logMAR units in two cases, and the visual fields were improved in all three cases. The multifocal ERGs which had been reduced in the loci corresponding to the ischemic retinal area were improved after the treatment in two cases. Neither ocular nor systemic adverse effects were observed except for transient superficial keratitis.TES of the retina can improve retinal function in eyes with long-standing RAOs.

Purpose To establish whether the visual evoked fields (VEFs) in patients of unilateral spatial neglect (USN) caused by cerebrovascular accident (CVA) are different from ones in subjects without USN. Method In three patients of CVA with left USN and three controls (a healthy subject, a patient of CVA without USN and a patient of hemianopsia), the VEFs following pattern reversal stimulation on the left hemifield were measured. Magnetic recordings were conducted using a 160-channel helmet-type axial superconducting quantum interference device (SQUID) gradiometer system. The behavioural inattention test (BIT) was also scored in patients of USN. Results Magnetic source imaging revealed two components (P100m and N145m) on the VEFs in the healthy subject and patient of CVA without USN, and no component in the patient of hemianopsia. The pattern of two components in patients with USN was various. Only P100m for patient 1, N145m for patient 2 and both components for patient 3 were detected. Concerning the BIT scores, line bisection and cancellation task for patient 1, coping, drawing and cancellation task for patient 2, and only cancellation task for patient 3 were impaired. Conclusion Our results demonstrate that pattern of two components (P100m and N145m) on the VEFs does not reflect only hemianopsia, but also USN caused by deficits of the cortical process of attention.

To establish an objective visual field analysis by visual evoked magnetic fields. Forty-eight focal areas of the visual field were stimulated by the visual evoked response imaging system (VERIS). The multifocal visual evoked magnetic fields (mfVEFs) of 11 healthy subjects were recorded. The output signals were recorded with VERIS, and the second-order kernel was calculated. The equivalent current dipoles (ECDs) were estimated, and the relative positioning of ECDs was determined by a magnetoencephalography (MEG) system. The mfVEFs consisted of either two- or three-peak waves. Large amplitude mfVEFs were elicited when the stimulus was confined to 6° of the central visual field, but a strong response could not always be obtained between 6° and 12°. All ECDs were estimated to originate in the occipital striate cortex. The ECDs for the upper (lower) field stimulations were estimated to be on the lower (upper) cortex, while those for right (left) field stimulations were on the left (right) cortex. The locations of mfVEF peak ECDs were correlated with the stimulated visual field and generally matched the cruciform model. In combination with the multifocal technique, MEG can be used for objective visual field analysis. Jpn J Ophthalmol 2004;48:115–122 © Japanese Ophthalmological Society 2004

Purpose: It is known that even after visual loss, younger patients with Leber's hereditary optic neuropathy (LHON) can recover vision. The purpose of this study was to determine the mean age at onset for LHON patients with and without visual recovery who carried the 11778 mutation, and to determine the pattern of central vision recovery. Methods: Thirty-five LHON patients with the 11778 mutation of mitochondrial DNA who had visited the Keio University Hospital between 1980 and 1999 and were followed for 2 to 20 years, were the subjects of this retrospective study. The patients who had recovered vision were tested by Goldmann perimetry, Humphrey perimetry, and landmark-driven fundus microperimetry with a scanning laser ophthalmoscope (SLO). The fixation status was assessed by SLO microperimetry. Results: Nine of the 35 patients (14 of 70 eyes) demonstrated a recovery of visual acuity to better than 0.3 in at least one eye. The mean age of disease onset was 15.9 ± 4.6 years in patients with visual recovery and 25.5 ± 8.9 years in patients without visual recovery. This difference in the mean age at onset was significant ( P = .0001; Welch t -test). These 9 patients (14 eyes) showed fenestrated central scotomas in testing by Humphrey 10-2 threshold and SLO microperimetry. The nasal side of the central visual fields had a higher sensitivity than the temporal side in 7 of the 9 patients in Humphrey 10-2 threshold testing. Areas insensitive to 0 dB were detected on the nasal side of the central retina in these patients by SLO microperimetry, and fixation stability was related to the degree of clinical visual acuity. Conclusion: The LHON patients with the 11778 mutation and a younger age of onset were more likely to show visual recovery. The findings made by perimetry suggest that the nerve fiber bundles in the nasal field (retina temporal to the fovea) may have a greater potential to recover function in LHON patients.

Purpose: To present two clinical cases diagnosed with predominant cone dystrophy and demonstrating early disturbance in the on-centre bipolar cells (ONpathway). Methods: Electrophysiological findings are presented in two siblings with predominant cone dystrophy. The subjects showed no remarkable ophthalmoscopic or fluorescein angiographic retinal changes, but demonstrated progressive visual disturbance during their 20s. Results: The electroretinograms (ERGs) showed reduced dark-adapted responses but the positive component of the photopic ERG was absent. Response to 30 Hz flicker was severely reduced. Electroretinograms elicited by long-duration stimuli showed a loss of the b-wave, and the off-response was slightly reduced. In both patients, multifocal ERGs (m-ERGs) were more reduced within the central 10ae, where the ON-pathway is normally a major contributor. Conclusion: We conclude that these patients may be affected by an abnormality of the synapses of the cone receptors and that their decrease in vision might, at least initially, be due to selective ON-pathway dysfunction.

To examine the physiologic condition of the middle retinal layer of patients with X-linked juvenile retinoschisis (xlRS) by studying the on- and off-responses of the photopic electroretinograms (ERGs).Eleven unrelated Japanese men (mean age; 24.9 +/- 7.6 years) who were clinically diagnosed with xlRS and molecularly confirmed as having XLRS1 mutations were investigated. For the photopic ERGs, the a-, b- and d-wave amplitudes elicited by long duration stimuli were recorded, and the responses from the xlRS patients were compared to those recorded from normal subjects (n = 14, mean age, 27.5 +/- 4.5 years). We also examined the relationship between the photopic ERG responses and the genotype.No significant difference was found between the a- and d-wave amplitudes in the xlRS patients (34.2 +/- 8.7 microV, 52.5 +/- 10.4 microV, respectively), and those in normal subjects (40.4 +/- 10.3 microV, 44.7 +/- 6.3 microV, respectively). The mean b-wave amplitude in the xlRS patients was significantly smaller (10.5 +/- 7.7 microV) than the mean of normal subjects (46.4 +/- 10.2 microV) (P0.0001). No significant correlation was found between the ERG responses and the locus of the mutation.The photopic ERG demonstrated considerable impairment of the on-pathway arising from an abnormality of the on-bipolar cells or possibly secondary to Müller cell abnormality in xlRS.

We report a case of acute zonal occult outer retinopathy (AZOOR) with a central scotoma and normal fundus. A 17-year-old man presented with reduced vision in his right eye.A relative afferent pupillary defect was not present, and his fundi were normal. He had a central scotoma in the right eye, but the left eye was normal. Central flicker fusion and color vision were normal in both eyes. Fluorescein angiography showed a somewhat mottled retina in the midperiphery of the right eye. The visually evoked potentials were reduced bilaterally. The a- and b-waves of the photopic ERGs and the amplitude of the flicker ERGs were reduced. In addition, the multifocal electroretinograms (mERGs) were reduced. A diagnosis of AZOOR was made. Electrophysiological studies were helpful in making a diagnosis of AZOOR in this case.

To determine whether visual field defects can be detected by the multifocal VEP technique.Multifocal VEPs were elicited by a pseudorandom binary m-sequence stimulus (VERIS II). The stimulus was a dartboard-like pattern of 61 sectors, and the luminance of each sector alternated between white and black. The stimulus area subtended approximately 25 degrees. Each recording was divided into 8 equal segments, and the total recording time was about 4 min. Multifocal VEPs were recorded from 25 normal subjects and six patients with visual field loss. The responses summed within 4 quadrants were used in the analysis and were compared with the visual fields obtained by perimetry.In six perimetrically-documented visual field defects, the responses summed over each quadrant of the field were reduced in the corresponding affected quadrants. In addition, recovery of the visual field loss following treatment was accompanied by a recovery of the responses.Multifocal VEPs summed within four quadrants can be used for an objective evaluation of the visual fields. The testing can be obtained in 4 min with no pain or discomfort to the patient.

Interferon (IFN), which is an established maintaining therapy for multiple sclerosis (MS), has been reported various adverse effects. This paper describes a case of IFN-associated retinopathy, representing an overlooked adverse effect in MS. A 46-year-old woman with diabetes mellitus was diagnosed with MS and treated with IFNbeta-1b for three years. She displayed sudden defect of the visual field and ocular fundi showed retinal cotton wool spots indicating IFN-retinopathy, which resolved rapidly after IFN was discontinued. To evaluate the prevalence of retinopathy in MS, we performed fundoscopic examination on twenty MS patients treated with IFNbeta-1b in our hospital. However, none of the patients displayed retinopathy. Only four other cases of IFN- retinopathy in MS have been reported in the literature. Therefore, IFN-retinopathy may be uncommon in MS, but neurologist should be mindful of this adverse effect and regularly check the fundus, particularly in patients with possible risk factor, diabetes mellitus.

Receptive-field properties of retinal ganglion cells (RGCs) that had regenerated their axons were studied by recording single-unit activity from strands teased from peripheral nerve (PN) grafts apposed to the cut optic nerve in adult cats. Of the 286 visually responsive units recorded from PN grafts in 20 cats, 49.7% were classified, according to their receptive-field properties, as Y-cells, 39.5% as X-cells, 6.6% as W-cells, and 4.2% were unclassified. The predominant representation of Y-cells is consistent with a corresponding morphological study (Watanabe et al. 1993a), which identified alpha-cells as the RGC type with the largest proportion of regenerating axons. Among the X-cells, we only found ON-center types, whereas both ON-center and OFF-center Y-cells were found. As in intact retinas, the receptive-field center sizes of Y-cells and W-cells were larger than those of X-cells at corresponding displacements from the area centralis. Within the 10 degrees surrounding the area centralis, the receptive fields of X-cells with regenerated axons were larger than those in intact retinas, suggesting that some rearrangement of retinal circuitry occurred as a consequence of degeneration and regeneration. Receptive-field center responses of Y-, X-, and W-type units with regenerated axons were similar to those found in intact retinas, but the level of spontaneous activity of Y- and X-type units was, in general, less than that of intact RGCs. Receptive-field surrounds were weak or not detected in more than half of the visually responsive RGCs with regenerated axons.

We describe a patient with Leber's hereditary optic neuropathy (LHON) who had a unilateral involvement and a gradual recovery of vision. A 50-year-old woman was referred to our clinic in December 2004 for the treatment of left optic neuritis. The visual acuity was 0.01 in her left eye and 1.5 in her right eye. The left eye had a central scotoma and a relative afferent pupillary defect. Ophthalmoscopy revealed a hyperaemic optic disc with indistinct margins in the left eye. Fluorescein angiography showed circumpapillary microangiopathy in both eyes and staining of the left optic disc. An nt 11778 mutation was identified and she was diagnosed with LHON. The central scotoma gradually improved, and the visual acuity had recovered to 0.3 in August 2007. LHON should still be considered even in older female patients presenting with unilateral acute visual loss when microangiopathy is seen. In such cases, molecular testing is effective in confirming a diagnosis of LHON.

We report two cases of anterior ischaemic optic neuropathy in whom tissue blood flow at the disc rim was correlated with the visual field defect. Tissue blood flow of each eye was evaluated with Heidelberg retina flowmeter. Both cases experienced acute visual loss and an altitudinal hemianopsia associated with optic disc oedema in the affected eye. In each case, the tissue blood flow at the affected (upper or lower half) disc rim corresponding to visual field deficit was reduced compared with that at the opposite-sided half disc rim in the affected eye and with the corresponding area in the fellow eye. The reduction of blood flow in the affected half disc rim associated with the visual field defect demonstrated that retina flowmetry can detect differences in tissue blood flow between superior and inferior sectional disc rim areas as well as between eyes non-invasively.

To report an improvement of the visual acuity after transcorneal electrical stimulation (TES) in a case of Best vitelliform macular dystrophy (BVMD).A 26-year-old woman diagnosed with BVMD presented with reduced vision. Her best corrected visual acuity (BCVA) was reduced to 20/200 in the right eye, and TES was performed once a month for two sessions. The current of the biphasic pulses (anodic first; duration, 10 msec; frequency, 20 Hz) was delivered using a DTL-electrode, and the duration of the TES was 30 min.The BCVA in the right eye slowly improved after the TES, and 6 months later the BCVA was 20/25. The results of Humphrey visual field tests (VF) and multifocal ERGs (mfERGs) were only slightly changed. Two years later, the BCVA decreased, and it was improved again after another session of TES with the same parameters of the electrical pulses.The improvement of the visual acuity in our case of BVMD indicates that TES should be tried in other cases of retinal dystrophy. Further clinical and laboratory studies on TES are needed.

To report the clinical characteristics of occult macular dystrophy (OMD) in members of one family with a mutation of the RP1L1 gene.Fourteen members with a p.Arg45Trp mutation in the RP1L1 gene were examined. The visual acuity, visual fields, fundus photographs, fluorescein angiograms, full-field electroretinograms, multifocal electroretinograms, and optical coherence tomographic images were examined. The clinical symptoms and signs and course of the disease were documented.All the members with the RP1L1 mutation except one woman had ocular symptoms and signs of OMD. The fundus was normal in all the patients during the entire follow-up period except in one patient with diabetic retinopathy. Optical coherence tomography detected the early morphologic abnormalities both in the photoreceptor inner/outer segment line and cone outer segment tip line. However, the multifocal electroretinograms were more reliable in detecting minimal macular dysfunction at an early stage of OMD.The abnormalities in the multifocal electroretinograms and optical coherence tomography observed in the OMD patients of different durations strongly support the contribution of RP1L1 mutation to the presence of this disease.

Occult macular dystrophy (OMD) is an inherited macular dystrophy characterized by progressive loss of macular function but normal ophthalmoscopic appearance. Typical OMD is characterized by a central cone dysfunction leading to a loss of vision despite normal ophthalmoscopic appearance, normal fluorescein angiography, and normal full-field electroretinogram (ERGs), but the amplitudes of the focal macular ERGs and multifocal ERGs are significantly reduced at the central retina. Linkage analysis of two OMD families was performed by the SNP High Throughput Linkage analysis system (SNP HiTLink), localizing the disease locus to chromosome 8p22-p23. Among the 128 genes in the linkage region, 22 genes were expressed in the retina, and four candidate genes were selected. No mutations were found in the first three candidate genes, methionine sulfoxide reductase A (MSRA), GATA binding 4 (GATA4), and pericentriolar material 1 (PCM1). However, amino acid substitution of p.Arg45Trp in retinitis pigmentosa 1-like 1 (RP1L1) was found in three OMD families and p.Trp960Arg in a remaining OMD family. These two mutations were detected in all affected individuals but in none of the 876 controls. Immunohistochemistry of RP1L1 in the retina section of cynomolgus monkey revealed expression in the rod and cone photoreceptor, supporting a role of RP1L1 in the photoreceptors that, when disrupted by mutation, leads to OMD. Identification of RP1L1 mutations as causative for OMD has potentially broader implications for understanding the differential cone photoreceptor functions in the fovea and the peripheral retina.

To determine whether exposure of the cornea and retina of rats to flashes from a commercial photographic flash lamp is phototoxic.Sprague-Dawley rats were exposed to 10, 100, or 1,000 flashes of the OPTICAM 16M photographic flash lamp (Fujikoeki, Japan) placed 0.1, 1, or 3 m from the eyes. Corneal damage was assessed by a fluorescein staining score, and the retinal damage by eletroretinography (ERG) and histology before and 24 h after exposure.Exposure of the eyes to 1,000 flashes at 0.1 m increased the fluorescein staining score significantly (P = 0.009, the Mann-Whitney test). Scanning electron microscopy (SEM) of the cornea showed a detachment of the epithelial cells from the surface after this exposure. The amplitude of the a-wave was decreased significantly by 23.0% (P = 0.026) of the amplitude before the exposure, and the b-wave by 19.7% (P = 0.0478) following 1,000 flashes at 0.1 m but not by the other exposures. TUNEL-positive cells were present in the outer nuclear layer only after the extreme exposure, but no significant decrease in retinal thickness was seen under any condition. The fluorescein staining score and ERGs recovered to control levels within 1 week.Light exposure to a photographic flash lamp does not induce damage to the cornea and retina except when they are exposed to 1,000 flashes at 0.1 m.

Purpose To investigate the concordance between subjectively and objectively acquired visual fields in patients with subjectively determined hemianopsia. Design Retrospective observational study. Methods Ten patients, six men and four women, ranging in age from 28 to 68 years, were studied. Goldmann or Humphrey perimeters were used to obtain the subjectively determined visual fields for up to 25 degrees of eccentricity, and the VERIS Scientific System (Electro-Diagnostic Imaging, San Francisco, California, USA) was used to record multifocal visual evoked potential [VEPs] (mfVEPs) to obtain the objective visual fields. Each of the 60 black-and-white segments of the checkerboard stimulus was alternated according to a binary m sequence. The first slices of the second-order kernels were extracted and analyzed. Results In five cases, the visual field loci where the mfVEPs were within normal limits corresponded to the scotomatous areas obtained by conventional perimetry. In these discordant cases, the lesions (e.g., arteriovenous malformation) were located in the occipital lobe. Two of these cases had a complete recovery of the subjective visual field. The lesions of the concordant cases were located outside the occipital lobe (e.g., pituitary adenoma). In these cases, no visual field improvement was seen. The temporal crescent syndrome was ruled out in patients with posterior lesions by computed tomography (CT) or magnetic resonance imaging (MRI) findings. Conclusions In some patients with occipital lesions, the subjective and objective visual field results are discordant, and some of them will show a recovery of the visual field deficits.

To present the clinical features of two brothers with molecularly confirmed X-linked juvenile retinoschisis (xlRS) but with non-characteristic electrophysiological findings.Comprehensive ophthalmological examinations were performed. The electroretinograms (ERGs) were recorded under ISCEV standards, and ERGs elicited by long-duration stimuli were also evaluated. Standard genetic analysis of peripheral blood leukocytes was performed.Molecular testing revealed a novel 473-bp deletion including exon 4 in the XLRS1 gene in both siblings. This resulted in a frameshift mutation and a premature termination at codon 78. The scotopic and photopic ERGs were reduced, but the "negative-type" ERG, characteristic of xlRS, was not observed. Flicker ERGs were also highly reduced. Long-duration stimuli elicited ERGs with a complete loss of the b-wave and a preservation of the off-response, i.e., negative-type ERG. The phenotype/genotype relationship was not determined.The consistency of the ERGs elicited by long-duration stimuli in xlRS patients suggests that this type of stimuli provides responses that are a better indicator for the progression or stage of the disease.

Abnormal optic disc excavations are reportedly seen in patients with Leber's hereditary optic neuropathy (LHON), a mitochondrial dysfunction disease. We examined the disc morphology in the eyes of patients with LHON at the atrophic stage and compared it to that in eyes with normal-tension glaucoma (NTG).We studied 15 LHON patients with the 11778 mutation, 15 patients with NTG, and 25 normal subjects. The optic disc morphology was analyzed by Heidelberg retinal tomography (HRT). Ten parameters of the optic disc obtained by HRT were evaluated, including the diagnostic classification of glaucoma.Six of the nine morphological HRT parameters of the LHON patients, the exceptions being disc area, mean cup depth, and maximum cup depth, differed significantly from those of the normals. NTG patients had a significantly greater mean and maximum cup depth than LHON patients. The HRT glaucoma diagnostic software classified 22 (73%) of the 30 optic discs in LHON patients as glaucomatous.The optic discs at the atrophic stage of LHON eyes have glaucoma-like morphological changes. However, the cups were significantly deeper in NTG than LHON. The similarity in the optic disc findings in LHON and NTG suggests that alterations in mitochondrial function may be related to optic disc excavations.

To present two clinical cases diagnosed with predominant cone dystrophy and demonstrating early disturbance in the on-centre bipolar cells (ON-pathway).Electrophysiological findings are presented in two siblings with predominant cone dystrophy. The subjects showed no remarkable ophthalmoscopic or fluorescein angiographic retinal changes, but demonstrated progressive visual disturbance during their 20s.The electroretinograms (ERGs) showed reduced dark-adapted responses but the positive component of the photopic ERG was absent. Response to 30 Hz flicker was severely reduced. Electroretinograms elicited by long-duration stimuli showed a loss of the b-wave, and the off-response was slightly reduced. In both patients, multifocal ERGs (m-ERGs) were more reduced within the central 10 degrees, where the ON-pathway is normally a major contributor.We conclude that these patients may be affected by an abnormality of the synapses of the cone receptors and that their decrease in vision might, at least initially, be due to selective ON-pathway dysfunction.

Purpose: To investigate whether the visual evoked potentials (VEPs) obtained by a newly designed stereogram were generated by some higher function like depth perception, we examined the effect of attention on the VEPs. Subjects and Method: Eight subjects participated in the experiments. The VEPs were recorded according to the international 10/20 system. The stimulus consisted of circles with or without disparity presented on 4 locations around the fixation point, and they appeared in one of the 3 patterns in random order when viewed through red/green spectacles (1) at the same distance as the fixation point, (2) in front of it, or (3) at the back of it. The near pattern was defined as the target. In Experiment 1, the subjects just fixated on the fixation point. In Experiments 2 and 3, they were required to respond to the target by pressing a button and counting the number (target discrimination task). Results: A scalp negative potential with a latency of 170–280 msec was elicited in the lateral occipital area. For each of the 3 patterns the amplitude was greater in Experiments 2 and 3 than in Experiment 1. In Experiments 2 and 3, stimuli with disparity resulted in larger amplitude than without disparity. The target stimulus also evoked larger potentials than non-target stimuli did, although no such effect was apparent in Experiment 1. Conclusion: Attention, as well as disparity, increased the VEPs amplitude, which indicates that the potential could be generated by neural activity higher than V 1 including depth perception. Moreover, the potential showed characteristics similar to attention-related potentials modulated by visuo-spatial attention.

Objective To characterize fundus autofluorescence (FAF) images of eyes with autosomal dominant occult macular dystrophy (OMD). Methods All patients received a comprehensive ophthalmologic examination for diagnosis of OMD. We evaluated the FAF images in 13 eyes of 7 patients with autosomal dominant OMD by confocal scanning laser ophthalmoscopy with excitation at 488 nm and emission more than 500 nm. Results The FAF images showed unspecific weak foveal hyperfluorescence in 4 eyes of 2 patients; one showed a thin hyperfluorescence in the temporal fovea bilaterally and the other showed weak hyperfluorescence in the fovea bilaterally. The optical coherence tomographic images showed abnormalities of the photoreceptor inner segment–outer segment line and cone outer segment tip line in all patients. However, 5 patients had normal FAF images regardless of morphological abnormalities of the photoreceptor. Conclusions Fundus autofluorescence is a useful method to acquire additional information of photoreceptor/retinal pigment epithelium function in eyes with OMD. Fundus autofluorescence will be also helpful for the differential diagnosis of eyes with OMD vs eyes with other dystrophies that have a distinctive FAF pattern.

Gen Hanazono1,2, Hisao Ohde2,3, Kei Shinoda1,4, Kazushige Tsunoda1,2, Kazuo Tsubota2, Yozo Miyake1,51Department of Ophthalmology, National Institute of Sensory Organs, Tokyo, Japan; 2Department of Ophthalmology, Keio University School of Medicine, Tokyo, Japan; 3Kamoshita Eye Clinic, Tokyo, Japan; 4Department of Ophthalmology, Teikyo University School of Medicine, Tokyo, Japan; 5Aichi Medical School, Aichi, JapanPurpose: Occult macular dystrophy (OMD) is a hereditary retinal disease characterized by a normal fundus, normal full-field electroretinograms (ERGs), progressive decrease of visual acuity, and abnormal focal macular ERGs. The purpose of this study was to report pattern-reversal visual-evoked potential (pVEPs) findings in OMD patients.Patients and method: The pVEPs recorded from four patients with OMD (aged 42–61 years; 2 men and 2 women) were reviewed. The visual acuities ranged from 20/200 to 20/30. The amplitudes of the N-75 and P-100 (P2 amplitude) and the latency of the N-75 components (N1 latency) were analyzed.Results: The mean (±SD) P2 amplitude was 2.7 ± 1.9 µV for the 5´, 4.8 ± 2.9 µV for the 10´, 3.2 ± 2.1 µV for the 20´, and 4.4 ± 3.5 µV for the 40´ checkerboard stimuli. The N1 latency was 122.2 ± 6.4 ms for the 5´, 105.0 ± 11.5 ms for the 10´, 97.7 ± 10.0 ms for the 20´, and 91.0 ± 13.7 ms for the 40´ checkerboard stimuli. The mean P2 amplitude was reduced and the N1 latency was delayed in comparison with the laboratory standard for the Keio University Hospital.Conclusions: The delayed latency and reduced amplitude suggest a major contribution of the central cone pathway to the pVEPs.Keywords: occult macular dystrophy, visual-evoked potential, electroretinogram

PURPOSE To investigate retinal circulation and function in eyes with branch retinal artery occlusion(BRAO) using a scanning laser Doppler flow-meter (SLDF) and multifocal electroretinogram (mERG). METHODS In juxtapapillary and paramacular areas close to the occluded artery of the affected eye and the corresponding area of the fellow eye, the mean flow measured by SLDF, indicating microcirculation, and the p-wave amplitude in the response of mERG, indicating retinal function, were determined in three patients with BRAO at the period of reperfusion. The ratios of the affected eye to fellow eye(a/f ratio) were calculated, and the ratios of the mean flow (Fa/f) were compared with those of the p-wave amplitude(Pa/f). RESULTS In the juxtapapillary area, both Fa/f and Pa/f decreased consistently, but in the paramacular area, Pa/f were lower than Fa/f in all cases. CONCLUSIONS Functional recovery is likely to be later in the paramacular area than in the juxtapapillary area in the course of recovery of retinal circulation.

We measured the extracted central nucleus (diameter and thickness) of 88 eyes (average patient age, 72 years) undergoing extracapsular cataract extraction (ECCE). The average diameter was 6.51 ± 0.75 mm; the average thickness, 2.96 ± 0.33 mm. Since the average lens diameter is approximately 10 mm, a corneoscleral incision of 7 to 8 mm in chord length should be sufficient for its removal in most ECCE cases. Accumulation of these data and further analysis correlating them with age and degree and type of cataract in larger series will be useful in determining the smallest possible incision size required for individual patients.

In humans with albinism, a large percentage of the ganglion cell axons from the temporal retina decussate abnormally in the chiasm and synapse in the contralateral LGN. The aim of this study was to determine whether the misrouting of the optic fibers can be detected by magnetoencephalography (MEG). Visually evoked magnetic fields (VEFs) were recorded from three patients with albinism. After monocular stimulation, the isofield contour maps of the VEFs showed a single current dipole pattern over the contralateral hemisphere in patients with albinism. These results clearly illustrated the reduced uncrossed retinofugal pathway of patients with albinism.

BACKGROUND/AIM—EAT/mcl-1 (EAT), an immediate early gene, functions in a similar way to bcl-2 in neutralising Bax mediated cytotoxicity, suggesting that EAT is a blocker of cell death. The aim of this study was to determine the effect of overexpression of the human EAT gene on light induced retinal cell apoptosis. METHODS—EAT transgenic mice incorporating the EF-1α promoter were utilised, and expression of human EAT was detected by RT-PCR. Light damage was induced by raising mice under constant illumination. Two groups of animals, EAT transgenic mice (n=14) and littermates (n=13), were examined by ERG testing and histopathology at regular time points up to 20 weeks of constant light stimulation. Electrophysiological and histopathological findings were evaluated by established systems of arbitrary scoring as scores 0-2 and scores 0-3, respectively. RESULTS—The mean score (SD) of ERG response was significantly lower in EAT transgenic mice (0.79 (0.89)) than in littermates (1.69 (0.48)) (p