島田 佳明

OBJECTIVE: To report a case of bilateral reversible optic neuropathy as the first sign of Waldenström macroglobulinemia (WM). METHODS: Observational case report. RESULTS: A 52-year-old man had a sudden loss of vision in the left eye. Examinations revealed the presence of a serum monoclonal immunoglobulin (IgM kappa) in the serum. Even after a session of steroid pulse therapy, optic neuropathy became bilateral and then resolved almost completely after 4 months. The condition progressed to WM with multiorgan lesions years later. There was no evidence of optic neuropathy recurrence. The literature revealed two cases of monoclonal gammopathy (MG): a 64-year-old man with multiple myeloma (MM) with IgA lambda and a 51-year-old man with MM with IgG kappa. These cases have similar conditions: 1) visual reduction as an initial symptom of MG, 2) bilateral involvement, 3) no sign of central nervous system (CNS) infiltration shown by normal brain magnetic resonance images, and 4) recovery to a visual acuity of ≥1.0 bilaterally with no reoccurrence. The excessive Igs or B-cell hyperactivity may activate an autoimmune mechanism that reversibly interferes with the bilateral optic nerves. CONCLUSION: Bilateral optic neuropathy was the initial symptom of WM. There was no evidence of CNS infiltration; it recovered and then did not reoccur. The pathogenesis remained unknown, but two cases of MG were reported in the literature with remarkably similar conditions.

PURPOSE: This study reports a case of unilateral retinopathy with extinguished full-field ERGs (ffERGs), wherein the visual acuity was 16/16 and the visual field was spared. METHODS: Observational case report. RESULTS: A 39-year-old female had developed nyctalopia in her left eye. Two years later, she visited an ophthalmologist who noted a bilaterally reduced pigmentation of the fundus. Her best-corrected visual acuity was 16/16 in both the eyes. Goldmann perimetry demonstrated that her visual field was bilaterally fully spared. ffERGs measurement was performed in accordance with the ISCEV standard protocol and indicated that her right eye was normal. However, all ERG responses were severely attenuated in her left eye. Multifocal ERG responses were found to be normal in the right eye and extinguished in the left eye except for residual responses that were exclusively located at the center. During the 7 years of the follow-up period, the visual field in the left eye, which was once normal, became shaded, and the development of a ring scotoma was identified. The visual field in the right eye is still full. CONCLUSIONS: The pathogenesis of this patient's condition still remains unknown, while unilateral retinitis pigmentosa, unilateral pigmentary retinopathy, acute zonal occult outer retinopathy, and autoimmune retinopathy can all be considered as possible explanations. The uniqueness of this case study is that the extinguished ERG responses are predictive of the functional alteration in the affected eye, when the initial visual acuity and the visual field were normal.

Background: The acute physiological changes induced by focal retinal photocoagulation (PC) have been largely unexplored. Methods: This was a case-series study. We recorded multifocal electroretinograms (mfERGs) just before PC, and mfERGs were also recorded 5′, 15′, one hour, 24 h, and one week after the PCs. Transient changes of mfERGs were analyzed in eyes which underwent PCs to treat diabetic macular edema. The mfERGs recorded from the predominantly irradiated area and that from non-irradiated areas were analyzed separately. Results: Fifteen eyes of 15 patients were included in this study. The mfERGs elicited from non-irradiated areas did not change after PC, but the mfERGs elicited from the irradiated area changed with time; the amplitude was larger at 60′ than that before (p < 0.05) and at 5′ after PC (p < 0.01) and significantly smaller at 24 h and 1 week than that before and at 60′ after the PC (p < 0.01). The implicit time was significantly prolonged after PC. mfERG on irradiated area with the severe diabetic change was less altered after PCs. Conclusions: The transient increase in the amplitude at 60′ likely resulted from a biological amplification of partially damaged cells adjacent to the PC spots. The mfERGs manifested the dynamic alterations of the retinal function following PCs.

Objective: To report a case of unintentional retina artery amputation during macular peeling. Methods: Observational case report Results: A 73-year-old female underwent surgery to treat epiretinal membrane (ERM) associated with severe staphyloma in her left eye. While the ERM/posterior hyaloid membrane visually enhanced with triamcinolone (TA) was peeled, a movement of the forceps unintentionally involved the inferior temporal branch artery near the inner edge of the staphyloma. The artery was avulsed and amputated. Oozing from the retinal cleft that had once enfolded the artery and microscopic bleeding from the amputation stump were observed. The vitreous hemorrhage obscured a view of the fundus more than 4 weeks after the surgery. After 8 weeks, postoperative visual acuity was improved; however, the superior nasal visual field was lost, and the patient was aware of the broken vessel as a floater in her vision. Conclusions: Macular peeling is technically challenging, so meticulous attention must be paid to avoid any damage on vessels. The retina tissue was stretched in a staphyloma and vessels were bulged into the vitreous space especially at the inner edge of the staphyloma. High levels of TA dye here buried the texture of the retina. Excessive TA should be removed prior to macular peeling.

The use of a conjunctival retractor that expands a small incision in the conjunctiva/Tenon's capsule facilitated the performance of strabismus surgery through a fornix incision. A wide field of exposure revealed the entire area of the muscle insertion. While there is a risk of conjunctival tearing in elderly patients, the use of the retractor is valuable.

PURPOSE: To determine adherence to face-down positioning (FDP) among patients who underwent vitrectomy and gas tamponade for macular hole (MH) repair. METHOD: A total of 69 patients (37 females and 32 males) who underwent primary vitrectomy for MH repair were studied. Nurses recorded whether the patient complied with FDP each time they examined the patient. FDP score was obtained from the nursing records; patients were given a score of 1 if they complied with the FDP and 0 if they did not. The score was recorded four times per day for the first 3 postoperative days. A perfect FDP score was 12. RESULTS: The mean ± standard deviation of the FDP scores was 10.6±1.8 (range: 4-12). Overall, 32 (46.4%) patients scored a perfect 12, and 7 (10.1%) patients scored <8. Failure of the MH closure was observed in only one patient (1.4%), who showed the poorest adherence to FDP (score =4). Consequently, the closure rate in patients with FDP score <7 (2/3, 66.7%) was significantly lower than in patients with an FDP score ≥7 (66/66, 100%) (P<0.05, Fisher's exact probability test). CONCLUSION: While adherence to FDP after MH surgery was better than that observed after vitrectomy for rhegmatogenous retinal detachments in our previous study, the percentage of patients with FDP scores <8 did not differ. Statistically, the poor adherence to FDP can negatively impact the effectiveness of the surgery for MH repair.

Leber hereditary optic neuropathy (LHON) causes visual loss, predominantly in healthy young men. We recently examined a patient who previously had bilateral macular holes and subsequently developed LHON at 74 years of age. Although his central scotomas were initially attributed to the macular holes, his visual acuity declined following an initial improvement after operative closure of the macular holes; thus, other diagnoses, including LHON, were considered. Furthermore, macular optical coherence tomography (OCT) images remained unchanged in this time. A mitochondrial genetic analysis identified a 11778G→A mutation. From this case, we propose that LHON remains in the differential diagnosis even in older patients, as has previously been reported.

We report a case of a 38-year-old man who presented with a recently self-detected lump under his left eyebrow. Previous ophthalmological history was unremarkable except for unilateral high myopia (left eye) since childhood. The appearance of the left eye was seemingly normal; however, with the top lid pulled up on downward gaze, a dark brown bulge emerged. The bulge was 10 × 7 mm and approximately 4 mm in height, and was covered by the extended superior rectus muscle. The diagnosis of equatorial staphyloma was made after coronal T1-weighted magnetic resonance imaging of the orbit revealed the dilatation of the vitreous cavity. Ocular movements were fully maintained and visual acuity was largely spared: 20/15 in the right eye without correction and 20/25 in the left eye with -10.00 spheres and -4.00 × 80 degrees cylinders. His past and family histories were unremarkable; however, small neurofibromas and café au lait spots all over his body led to the diagnosis of neurofibromatosis type 1 (NF1). From this case, similar to previous reports, we suggest that manifestations of NF1 are extremely variable and unpredictable.

Purpose: The aim was to evaluate the value of multifocal electroretinogram (mfERG) and optical coherence tomography (OCT) in predicting post-operative visual acuity (post-VA) after removal of an idiopathic epiretinal membrane (ERM). Methods: Fifty-one eyes of 51 patients with an idiopathic macular ERM were examined by OCT and mfERG before three-port pars plana vitrectomy with removal of the ERM. The parameters of the OCT images and mfERGs, pre-operative visual acuity (pre-VA) and the duration of symptoms (DS) were compared with the post-VA. Results: The pre-VA (r = 0.570, p &lt; 0.001) and the duration of the symptoms (r = 0.389, p &lt; 0.035; n = 35) were significantly correlated with the post-VA. The central retinal thickness (CRT) was significantly correlated with the pre-VA (r = 0.379, p &lt; 0.01) and the post-VA (r = 0.318, p &lt; 0.03). The amplitudes of macular electroretinogram (amERGs) were not significantly correlated with the pre-VA or post-VA. The CRT to amERG ratio (CRT/amERG) was correlated with the post-VA (r = 0.429, p &lt; 0.002) but not with the pre-VA (p &gt; 0.1). Comparative analyses among groups of cases based on CRT/amERG showed significant differences in the post-VA but not in the pre-VA and the duration of symptoms. Conclusion: As with the large CRT/amERG, the pre-operative morphologically thickened and electroretinographically reduced retinas have a greater likelihood of being affected by the irreversible photoreceptor damages that could have been masked or modified by other factors before the removal of the ERM. Because the CRT/amERG values are obtained objectively, they should be valuable in predicting the post-operative visual function along with the pre-VA and the duration of symptoms.

PURPOSE. To investigate the functional characteristics of the detached retina on a serous retinal detachment (SRD) in eyes with central serous chorioretinopathy (CSC) with spared visual acuity. METHODS. Multifocal electroretinograms (mfERGs) were recorded with a long recording time of 14 minutes, 34 seconds, to obtain accurate measurement of the second-order kernel (K2.1), an index of functional adaptability of the retina, from seven eyes with CSC (visual acuity, &gt;= 1.0). The first-order kernel (K1) and the K2.1, elicited by stimulating the area of the SRD, were compared with those from the corresponding areas in eyes of 15 age-matched volunteers (controls) and in 6 eyes of patients with diabetic retinopathy (DR) that have been reported to have a K2.1 attenuation. RESULTS. K2.1 was essentially flat in the SRD eye. The K2.1 amplitude and log-scaled amplitude ratio of K2.1 to K1 (K2.1/K1) were severely reduced (to &lt;95% confidence interval [CI] of control levels) in all eyes. The value of K2.1/K1 of the SRD was less than that in any of the control and DR eyes. K1 was moderately reduced but was not smaller than the 95% CI of control eyes. The mfERGs from the area without the SRD and those from the fellow eyes did not differ significantly from those in control eyes. CONCLUSIONS. A possible cause of the flat K2.1 observed on the SRD is the separation of the sensory retina. A substantial disparity between the recovery of cones and rods could contribute to the loss of retinal adaptability, resulting in the flat K2.1 as well as the unique visual impairments in CSC eyes. (Invest Ophthalmol Vis Sci. 2010; 51: 3210-3215) DOI:10.1167/iovs.09-4637

A 51-year-old man had reduced vision and bilateral optic disc swelling as the initial clinical manifestation of multiple myeloma. Brain imaging failed to disclose any abnormalities. Before any therapy was begun, visual function began to improve substantially. Three months after chemotherapy was started, visual function and optic disc appearance returned to near normal. There were no features to suggest polyneuropathy-organomegaly-endocrinopathy-M protein-skin changes (POEMS) syndrome. Visual loss in myeloma is usually caused by compression or infiltration of the optic nerves by tumor. The mechanism of the optic neuropathy in this case remains unknown. © 2006 Lippincott Williams &amp Wilkins, Inc.

To determine retinal function after transpupillary thermotherapy (TTT) for subfoveal choroidal neovascularization using multifocal electroretinograms (mfERGs).Multifocal electroretinograms were recorded before and after TTT (wavelength, 810 nm; diameter, 3 mm; duration, 60 seconds; power, 350 mW) in 9 eyes in 9 patients with subfoveal choroidal neovascularizations. The stimulus consisted of 7 hexagons; the central hexagon covered the laser-irradiated area and the surrounding 6 hexagons covered the nonirradiated area. Each recording was completed within 1 minute, and mfERGs were recorded periodically during the first 60 minutes after TTT and also at 24 hours and 1 week after TTT.The amplitude of mfERGs from irradiated areas was significantly reduced at 1 minute after TTT (P.01) and then recovered soon. The peak time was prolonged at 15 minutes after TTT (P.01), recovered to pre-TTT levels at 60 minutes, and then was prolonged again at 24 hours (P.05) and 1 week (P.05) after TTT. The mfERGs in nonirradiated areas were unchanged during the observational period.We found amplitude reduction in central focal ERGs at 1 minute after TTT, transient peak-time delay at 15 minutes, and a delay at 24 hours. Early reduction is probably directly caused by an increase in temperature during TTT as previously reported in focal flicker ERGs. Peak-time delays at 15 minutes and 24 hours may be caused by other factors, such as increased intracellular calcium (Ca(2+)), the release of nitric oxide or heat shock proteins, vasodilation, or change in choroidal neovascularization. Our findings indicate that recording mfERGs may be a useful tool for evaluating TTT procedures.

The multifocal VEPs (mfVEPs) obtained from the right and left eyes are practically identical in shape and amplitude, however small differences in the implicit times (ITs) have been reported. We have analyzed the ITs in detail to show that they are dependent on the radial location of the stimulus in the visual field. The differences are large, up to 8.3 ms, near the horizontal meridian, and are probably due to differences in the distances of the nasal and temporal ganglion cell axons from the optic disc. The intraretinal conduction velocity of ganglion cell axons, determined by dividing the axonal length to the optic disc by the ITs, was 0.29 m/s at an eccentricity of 0.5-1.4degrees and 1.39 m/s at 8-12degrees. (C) 2004 Elsevier Ltd. All rights reserved.

Purpose: To investigate the retinal responses generated by flash interactions in a recently introduced "global (full-screen) flash" stimulus paradigm to record the multifocal electroretinogram (mfERG). Methods: Five normal individuals were studied with stimulation combining multifocal (pseudorandom) flashes with interleaved global (periodic, full-screen) flashes. The intensities of the two flashes were independently varied. Two distinct first-order response components were obtained: the mean response to the focal flashes (referred to as the direct response, DR) and the effect of the focal flash on the responses evoked by the global flash (the induced component, IC). Results: Increasing the global flash luminance reduced DR amplitude and shortened DR implicit time. IC amplitude peaked with a moderate global flash (1.33-2.67 cds/m2). With a global flash of the right intensity, a weak focal flash could evoke a considerable IC even when the DR was barely detectable. Moderate global flashes maximized the IC, and its intersubject variability was reduced at lower focal flash luminances. IC topography had a large naso-temporal asymmetry. Conclusions: While the DR is the average response generated by the local flashes regardless of the context of preceding and following flashes, the IC represents the difference in the global flash response in the presence and absence of the preceding focal flash. As the focal flashes were always preceded by the periodically occurring global flashes, the DR reflects the resulting adapted or desensitized state of the retinal patch. The pure, nonlinear IC is thought to reflect predominantly inner retinal function.

A healthy 5-year-old girl presented with bilateral hyphema as an initial symptom of uveitis. The ocular findings and the patient's age suggested that the uveitis was due to juvenile rheumatic arthritis or chronic iridocyclitis.

PURPOSE. To evaluate the characteristics of stray light-induced response in multifocal ERG (mfERG) elicited by the stimulus falling on the disc. METHODS. A patient with an enlarged optic disc (4 X 4 disc diameters of disc of normal fellow eye) and four normal volunteers served as subjects. The mfERGs elicited by different stimulus intensities (0.67- 4.67 cd-sec/m(2)) were recorded from the patient, and the mfERGs obtained with stimuli on the enlarged optic disc. For comparison, full-field pseudorandom ERGS (ffprERGs) were also recorded in all subjects. The first-order kernels (K1) and the second-order kernels (K2.1) were analyzed. RESULTS. A small and delayed K1 was recorded on the enlarged disc, but K2.1 was flat on the disc at all intensities. The implicit time of K1 at lower intensities was longer than at higher intensities. ffprERGs at very low intensities in the patient and normal subjects were similar to the mfERG on the disc (delayed K1 associated with flat K2. 1). CONCLUSIONS. The responses elicited by stimulating the disc were delayed in K1 and flat in K2.1. Because similar ffprERGs were observed at very low intensities, it is likely that an optic disc with high reflectance scattered the stimulus light to create a weak full-field stimulus. Thus, care must be taken when focal lesions are investigated with mfERGs.

A transparent irrigation cannula was developed to facilitate bimanual removal of subincisional lens cortex after phacoemulsification. The cannula allows visualization of the proximal cortex and provides ample inflow to maintain an inflated anterior chamber even at a high aspiration pressure. © 2002 ASCRS and ESCRS.

Aims - To assess early functional retinal changes in diabetics without retinopathy, a new multifocal stimulus paradigm was used that emphasises fast adaptive response contributions. Methods - 25 normal control subjects (25 eyes) and 11 diabetics without retinopathy (22 eyes) served as subjects. Stimulation and analysis were performed with Veris Science 4.0. A stimulation protocol was used that combines regular multifocal flicker stimulation with a periodic "global" flash inserted between the multifocal stimuli. The multifocal stimuli were presented four video frames apart. The global flash covered the entire screen in the third frame of the four frame interval. The remaining two frames were dark. The periodic global flashes could only contribute to the focal responses if they were affected by the multifocal stimulation. A non-linear component induced by the interaction of the focal and global flashes was observed. The differences between control subjects and diabetics were assessed in both the multifocal responses and their induced effect on the following global flashes. Results - The responses to focal flashes were reduced significantly in diabetics matched in age to the control subjects. The induced components showed large intersubject variability in controls and patients, and did not differ significantly between the two groups. Conclusion - The periodic global flashes produce a greater multifocal response reduction in diabetics than in normals, indicating impairment in the rate or magnitude of recovery from the bright preceding stimulus. The new stimulation protocol reveals early changes in retinal function of diabetics.

<jats:p>To investigate the macular contribution to the amplitude and latency of pattern visual evoked cortical potentials (PVECPs), we recorded PVECPs in patients with unilateral macular disease (retinal diseases involving the macular region) and optic neuritis. We selected patients with visual acuities better than 0.3, which is thought to be the minimum acuity to provide clearly discernible responses. The visual stimulus was a checkerboard. We varied the check size in 4 steps as 7, 14, 28, and 56 min of arc, and the contrast was 20% or 80%. The amplitude of the sinusoidal wave of steady‐state VECPs (12 Hz) and the P100 component of a transient VECP (3 Hz) were measured. Significant attenuation and delay of PVECPs in the affected eye were found in the macular disease group. Macular disease may cause an increase of VECP latency. However, VECP seemed to be a more sensitive test of optic nerve disease than of macular disease, when patients had similar visual acuities.</jats:p>

We developed a new visual stimulating system for recording visual evoked cortical potentials and electroretinograms. The stimulus was a color checkerboard, in which each check kept its chromaticity but changed its luminance with its corresponding check. Color-coded pattern stimuli using red and green checks did not produce visual evoked cortical potentials, while yellow checks produced clear responses in a normal subject. Moreover, five color stairs from red and green to yellow showed only that the more colors are different, the smaller the visual evoked cortical potentials become. In addition electroretinogram recordings indicated that color-coded patterns behave in the same way as in visual evoked cortical potentials. The mechanism that causes the small color visual evoked cortical potentials may already be present in the retina. Color perception may be able to induce a suppression of responses for luminance contrast that appears to be formed already in the retina. Retinal responses were affected whether the stimulus field was color coded or not. Pattern electroretinograms appear to be more than the sum of local on and off responses.