Detection using the multifocal electroretinogram of mosaic retinal dysfunction in carriers of X-linked retinitis pigmentosa

PURPOSE: To examine whether a mosaic pattern of retinal dysfunction in obligate carriers of X-linked retinitis pigmentosa (XLRP) could be observed in local electroretinographic responses obtained with the multifocal electroretinogram (mfERG). DESIGN: Prospective observational case series. PARTICIPANTS: Five obligate carriers of XLRP (mean age, 53.2 years) were recruited into the study. METHODS: Examination of each subject included a complete ocular examination, Humphrey visual field, standard full-field electroretinogram (ERG), and mfERG testing. For the mfERG, we used a 103-scaled hexagonal stimulus array that subtended a retinal area of approximately 40 in diameter. The amplitudes and implicit times in each location for the mfERG was compared with the corresponding value determined for a group of normally sighted, age-corrected control subjects. MAIN OUTCOME MEASURES: Mapping of 103 local electroretinographic response amplitudes and implicit times within the central 40 with the multifocal electroretinogram. RESULTS: Localized regions of reduced mfERG amplitudes and/or delayed implicit times were found in four of five carriers. In one of these four carriers, a mosaic pattern of mfERG dysfunction was present even in the absence of any clinically apparent retinal changes, retinal sensitivity losses on Humphrey field testing, or abnormal full-field cone ERG responses. However, one carrier with a typical tapetal-like reflex demonstrated no deficit on any functional tests. CONCLUSIONS: The mfERG demonstrated patchy areas of retinal dysfunction in some carriers of XLRP. This mosaic pattern of dysfunction may be observed in some patients with a normal-appearing fundus, normal psychophysical thresholds, and normal amplitude and implicit time full-field ERG cone responses.     

Use of the multifocal electroretinogram in the evaluation of a patient with central areolar choroidal dystrophy

PURPOSE: To assess the multifocal electroretinogram and full-field electroretinogram findings in a patient with advanced central areolar choroidal dystrophy.DESIGN: Observational case report. METHODS: A 53-year-old man with central areolar choroidal dystrophy underwent multifocal electroretinogram and full-field electroretinogram testing. RESULTS: The multifocal electroretinogram demonstrated relative preservation of foveal function compared with the severely depressed retinal function of the perifoveal macula corresponding to the location of the retinal and retinal pigment epithelium atrophy. The full-field electroretinogram was normal for both photopic and scotopic responses. CONCLUSION: Despite a normal full-field electroretinogram, the multifocal electroretinogram demonstrated significant macular dysfunction with relative preservation of foveal function in a patient with central areolar choroidal dystrophy.     

Acute Visual Field Defect following Vitrectomy Determined to Originate from Optic Nerve by Electrophysiological Tests

Purpose

To present our findings on the cause of an acute visual field defect (VFD) that developed in a patient on the day after vitrectomy for proliferative diabetic retinopathy.

Case

A 50-year-old man complained of a blind area in the superior visual field that developed one day after vitrectomy. The patient had undergone uncomplicated vitrectomy for a long-duration vitreous hemorrhage associated with proliferative diabetic retinopathy. Residual vitreous hemorrhage hampered a clear view of the fundus. Goldmann perimetry showed a horizontal VFD in the superior field. The area corresponding to the VFD was examined by multifocal electroretinograms (mfERGs) and multifocal visual evoked potentials (mfVEPs). The amplitudes of the mfVEPs were reduced with prolonged implicit times especially when the superior hemifield was stimulated, while the amplitudes and implicit times were within the normal range when other parts of the visual field were stimulated. In addition, the full-field photopic ERGs and photopic negative responses were attenuated in the right eye. These findings suggested that the VFD did not originate from alterations in the retinal inner and middle layer but in the ganglion cells. The visual acuity improved to 1.2 but his optic disc became pale and the VFD remained unchanged more than 12 years after the surgery.

Conclusion

We suggest that vitrectomy can cause ischemic optic neuropathy by interfering with the circulation associated with diabetes mellitus. Evaluations by mfERGs, mfVEPs, and full-field photopic ERGs were helpful in making the diagnosis.Key words: Ischemic optic neuropathy, Proliferative diabetic retinopathy, Multifocal electroretinogram, Multifocal visual evoked potentials, Photopic negative response     

Morphology and functional characteristics in adult vitelliform macular dystrophy

PURPOSE: Detailed morphologic and functional evaluation of adult vitelliform macular dystrophy (AVMD). METHODS: The records of 61 consecutive AVMD patients (inclusion criterion: vitelliform lesion smaller than one disk diameter at least in one eye) were evaluated retrospectively regarding visual acuity, color vision, perimetry, retinal pigment epithelium (RPE) autofluorescence, fluorescein angiography, electro-oculography, full-field and multifocal electroretinography, and molecular genetic evaluation of the VMD2 and RDS/peripherin genes. RESULTS: The mean age of subjects was 54.6 years. Visual loss was variable (median, 0.6; range, 1.25-0.05). Color vision and visual field were normal in about half of the patients but presented defects with high variability in the remaining patients. Autofluorescence findings showed increased fluorescence within the foveal yellow lesion in 76%. In the majority of eyes, the amplitude of the 30 Hz flicker response of the full-field electroretinogram (72%) and the central P1 amplitude of the multifocal electroretinogram (63%) were reduced. Mutational analyses revealed a potentially disease-associated mutation in the RDS/peripherin gene in one patient. CONCLUSION: AVMD is characterized by late onset, slow progression, good prognosis, and high variability of morphologic and functional abnormalities resulting frequently in misdiagnosis. Autofluorescence findings indicate lipofuscin accumulation in the yellow lesion. Electroretinography revealed a generalized cone system dysfunction with increasing severity toward the fovea.     

Early changes of retinal function in diabetic patients detected by multifocal electroretinogram

To investigate the early changes of retinal function in diabetic patients detected by multifocal electroretinogram (mfERG). ·METHODS: The first-order kernel responses of mfERG were recorded fromeyes of 33 normal control subjects, 63 diabetic patients without retinopathy and 43 diabetic patients with background retinopathy. The response densities and implicit times of N₁ and P₁ were compared among the control, diabetic patients without retinopathy and diabetic patients with retinopathy. ·RESULTS: The response densities of N1 and P1 in central 3 rings were reduced significantly in diabetic eyes with and without retinopathy. And the implicit times of N₁ and P₁ were delayed significantly only in diabetic eyes with retinopathy. ·CONCLUSION: mfERG can detect the early changes of retinal function quantitatively in diabetic patients. Analysis of response densities and implicit times of N₁ and P₁ can reflect the progress of local retinal dysfunction in diabetes     

PFCL and ILM peeling in macular hole with retinal detachment

To investigate the early changes of retinal function in diabetic patients detected by multifocal electroretinogram (mfERG). ·METHODS: The first-order kernel responses of mfERG were recorded fromeyes of 33 normal control subjects, 63 diabetic patients without retinopathy and 43 diabetic patients with background retinopathy. The response densities and implicit times of N₁ and P₁ were compared among the control, diabetic patients without retinopathy and diabetic patients with retinopathy. ·RESULTS: The response densities of N1 and P1 in central 3 rings were reduced significantly in diabetic eyes with and without retinopathy. And the implicit times of N₁ and P₁ were delayed significantly only in diabetic eyes with retinopathy. ·CONCLUSION: mfERG can detect the early changes of retinal function quantitatively in diabetic patients. Analysis of response densities and implicit times of N₁ and P₁ can reflect the progress of local retinal dysfunction in diabetes     

Tamoxifen-Retinopathie: Eine Fallserie von klinischen und funktionelle Daten

BACKGROUND: Tamoxifen is used in the treatment of selected patients with breast carcinoma. Rarely, it has been shown to cause ocular toxic effects including crystalline retinopathy. METHODS: Retrospective analysis of clinical and functional (visual acuity, visual field, colour vision) data of a case series of eight female patients under tamoxifen therapy with electrophysiological examination. RESULTS: Seven of eight patients complained of visual disturbances. In one case, examination showed crystalline deposits in the cornea and macular area. Three patients revealed changes in full-field and multifocal electroretinogram, and two had a pathological multifocal electroretinogram only. In six cases we applied a desaturated panel D-15 colour vision test; five of these showed some disorders. CONCLUSIONS: Most tamoxifen patients who complained of visual disturbances showed electrophysiological changes, particularly in the multifocal electroretinogram and often without a certain morphological correlate. We recommend electrophysiological examination for patients with unclear visual deterioration who are receiving tamoxifen therapy.     

Retinal dysfunction and anterior segment deposits in a patient treated with rifabutin

PURPOSE: To describe the clinical and electrophysiological findings in a young boy with decreased vision possibly due to retinal damage by rifabutin. METHODS: An 8-year-old boy with osteomyelitis was referred due to visual disturbance. During a period of 4 years, the boy was examined six times with electroretinography. Ophthalmological examination included testing of visual acuity, slit-lamp inspection, fundus inspection, fundus photography and kinetic perimetry. Two electrophysiological methods were performed for objective evaluation of retinal function, namely full-field electroretinography and multifocal electroretinography. RESULTS: We found a slightly reduced visual acuity, a slowly increasing amount of yellow-white deposits on the posterior surface of the cornea and on the anterior part of the lens, a normal fundus appearance, and normal visual fields. However, the electroretinogram was abnormal on several occasions during therapy with rifabutin, but returned to normal 3 months after withdrawal of the medication. The multifocal electroretinogram returned to normal after the full-field electroretinogram had done so. The anterior chamber deposits still remain. CONCLUSION: Long-term treatment with rifabutin may have a reversible and previously undescribed side-effect on retinal function. The drug may also accumulate irreversibly on the posterior surface of the cornea and on the anterior surface of the lens. We suggest that objective evaluation of retinal function with electrophysiological methods should be performed in patients with visual disturbance during treatment with rifabutin.     

Retinal disorders in northern Brazilian patients treated with chloroquine assessed by multifocal ERG

The effects of chloroquine intake on the retinal function in a Brazilian population of patients were assessed by multifocal electroretinography. Twenty-four randomly chosen eyes of patients treated with chloroquine for rheumatoid arthritis and systemic lupus erythematosus were examined using multifocal electroretinography (mfERG). Control measurements were acquired from 21 randomly chosen eyes of age-matched healthy subjects. None of the study participants had an inherited retinal disease or a Snellen visual acuity reduced to less than 20/40. In patients and control subjects, cumulative chloroquine dose, total daily dose, duration of treatment, retinal examination, visual field defects, visual acuity, and the mfERG were assessed. The average amplitudes and implicit times of the N1, P1, and P2 components of the mfERGs were measured in the central hexagon (R1) and in five rings (R2–R6). The values measured in patients and normal subjects were compared. The P1 amplitudes in R2 were significantly decreased in the patients. In addition, the amplitudes of N1 and N2 in R1 were significantly smaller in the patients. The implicit times of none of the components were significantly different between patients and controls. The response amplitude was not significantly correlated with cumulative dose and duration of intake. There was no correlation with retinal appearance, visual field, and visual acuity. In agreement with earlier data, the central mfERG amplitudes were decreased in chloroquine patients indicating functional alterations in the retina. These changes are also present in a Brazilian population suggesting that the effects of chloroquine are general and that genetic background and life circumstances probably have, if at all, only little effect.     

Multifocal electroretinogram delays reveal local retinal dysfunction in early diabetic retinopathy.

PURPOSE: To identify local retinal abnormalities in diabetic patients with and without retinopathy, by using the multifocal electroretinogram (M-ERG). METHODS: Electroretinograms were recorded at 103 discrete retinal locations in each eye of eight persons with nonproliferative diabetic retinopathy (NPDR) and eight diabetic persons without retinopathy, using VERIS (EDI, San Mateo, CA). The amplitude and implicit time of each local (first-order) retinal response were derived and compared with normal values obtained from 16 age-matched, nondiabetic subjects. Maps of local response amplitude and implicit time were compared with fundus photographs taken at the time of testing. RESULTS: In eyes with NPDR, the implicit times of responses from retinal sites manifesting clinical pathologic fundus lesions (e.g., microaneurysms and focal edema), were markedly delayed (e.g., up to 7 msec from normal). Responses from adjacent retinal sites that were more normal in clinical appearance were also delayed, but to a lesser extent (e.g., 2-5 msec). Smaller, yet significant local response delays were also found in eyes without retinopathy. By contrast, local response amplitudes bore no consistent relationship to fundus abnormalities in eyes with retinopathy, and amplitudes were typically normal in eyes without retinopathy. CONCLUSIONS: The M-ERG reveals local retinal dysfunction in diabetic eyes even before retinopathy. The magnitude of delay of local ERG implicit time reflects the degree of local clinical abnormality in eyes with retinopathy. Local response delays found in some eyes without retinopathy suggest that the M-ERG detects subclinical local retinal dysfunction in diabetes. Analysis of M-ERG implicit time, independent of amplitude, improves the sensitivity of detection of local retinal dysfunction in diabetes.     

Assessing Responses of the Macula in Patients with Macular Holes using a New System Measuring Localized Visual Acuity and the mfERG

Purpose: To evaluate acuity and multifocal electroretinogram (mfERG) responses from the macula in affected and unaffected fellow eyes of patients with macular holes. Methods: We tested 10 eyes with macular hole and 10 fellow eyes from 11 patients. We measured local visual acuity thresholds at 27 discrete locations within 21° diameter using the Functional Fundus Imaging System (FFIS), a psychophysical system that measures visual acuity as a function of visual field location, and local ERG responses within 45° diameter using the mfERG. Results: In the affected eyes, the mean FFIS visual acuity thresholds were significantly elevated within the central 21° diameter area, compared to a group of control eyes. No significant differences were found between the acuities of the fellow eyes compared to those of the control group. The amplitudes of the first positive peak of the mfERG were reduced in the central 7.8° in affected eyes. In the central 2°, 4 out of 10 affected eyes showed non-measurable ERG signals. The remaining six eyes showed significantly reduced mean amplitudes, but not delayed implicit times, when compared to the control group. For the fellow eyes, the mean amplitudes of the mfERG and implicit times did not differ from the means of the control eyes. Conclusions: Both local psychophysical and electrophysiological testing demonstrated retinal dysfunction extending beyond the site of the macular holes in some patients (three of the patients had central mfERG amplitudes falling within the normal range).     

Acute zonal occult outer retinopathy in a patient with graft-versus-host disease

PURPOSE: To investigate the mechanism of bilateral central vision loss in a patient with graft-versus-host disease. DESIGN: Observational case report. METHODS: A 43-year-old man with graft-versus-host disease developed acute painless progressive central vision loss, first in the left eye and then in the right. The patient underwent slit-lamp biomicroscopy, indirect ophthalmoscopy, fluorescein angiography, visual field testing, full-field electroretinography, multifocal electroretinography, and testing for paraneoplastic antibodies. RESULTS: Fundus examination and fluorescein angiography were unremarkable. Goldmann perimetry revealed enlarged blind spots with central scotomas bilaterally. An electroretinogram testing showed asymmetric retinal dysfunction, consistent with acute zonal occult outer retinopathy. No paraneoplastic autoantibodies were detected. The patient continued to have asymmetric progressive vision loss that stabilized over the next 6 months. CONCLUSIONS: Graft-versus-host disease should be included in the autoimmune conditions associated with AZOOR.     

A Korean family with an early-onset autosomal dominant macular dystrophy resembling North Carolina macular dystrophy

PURPOSE: To characterize and report the phenotype of a Korean family with an early-onset autosomal dominant macular dystrophy resembling North Carolina macular dystrophy (NCMD). METHODS: Five members of a Korean family were examined clinically and underwent fundus photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography, full field electroretinogram (ERG), multifocal ERG, electro-oculography (EOG), a color vision test, and a visual field test. RESULTS: Visual acuity ranged from 20/200 to 20/20. Fundus findings demonstrated varying degrees of involvement ranging from drusen only to chorioretinal involvement. Central scotoma corresponded to retinal lesions in two patients. Full field ERG was normal but multifocal ERG showed decreased amplitude and delayed implicit time in the macular area. EOG was normal except in one patient. Color vision tests were also normal. CONCLUSIONS: The phenotype of this Korean family is consistent with NCMD. Linkage analysis is required to confirm the diagnosis.     

Acute effects of sildenafil on the electroretinogram and multifocal electroretinogram

PURPOSE: To evaluate the acute effects of sildenafil (Viagra; Pfizer, Inc, New York, New York) on the electroretinogram and multifocal electroretinogram. METHODS: Eighteen healthy individuals (ages 21-49 years) were studied; 14 were given 200 mg sildenafil orally and four were given only water. All subjects were tested before sildenafil and 1 hour after sildenafil (or water) with a desaturated Panel D-15 color test, a full-field standard electroretinogram, and a multifocal electroretinogram using the VERIS system; five subjects were also tested 5 hours after sildenafil. RESULTS: Responses from the subjects who received sildenafil were compared with those from the control subjects. At 1 hour after sildenafil, photopic single-flash waveforms were attenuated by 9% and scotopic maximal response amplitudes were increased slightly. Photopic and 30-Hz flicker electroretinogram responses were delayed; multifocal electroretinogram waveforms were delayed (5%-9%) and attenuated (14%-22%) across the posterior pole. These changes did not resolve by 5 hours. Nine of the subjects who had received sildenafil (64%) reported visual or systemic symptoms, including one who reported bluish vision. Ten of those subjects (71%) showed a slight increase in color test errors 1 hour after sildenafil. CONCLUSIONS: For at least 5 hours after taking 200 mg of sildenafil, cone function was slightly depressed in the macula and periphery, as measured by full-field electroretinogram and multifocal electroretinogram recordings. However, the affected electroretinogram and multifocal electroretinogram parameters still remained within normal limits.     

Late onset cone dystrophy

Cone dystrophies are a hereditary, progressive and heterogeneous group of retinal diseases with cone system degeneration. They lead to reduced visual acuity, colour vision impairment and photophobia. Full-field electroretinogram (ERG) reveals severe cone function impairment, with normal rod responses or slightly depressed in advanced stages in some cases. The purpose of the study was to present a case of late onset cone dystrophy in 47-year-old male and the proper diagnostic procedure. A 47-year-old patient presented with progressive visual loss for several years and mild photophobia, which he observed recently. The patient underwent fundus photography, fluorescein angiography, colour vision testing, Goldmann visual field testing, full-field electroretinogram (ERG) and multifocal electroretinogram (mfERG). Symptoms and signs of late onset cone dystrophy may be unclear and establishing the proper diagnosis may be difficult in these cases. Patients may be misdiagnosed as having other diseases, especially in case of absence or subtle changes in the macula. The electrophysiological testing is essential in these cases, and ERG is the most useful clinical test in early and differential diagnosis of retinal dystrophies.     

Macular function and morphology in acute retinal pigment epithelitis

A 20-year-old man applied with vision loss in the left eye. Right eye examination was unremarkable. Best-corrected visual acuity (BCVA) in the left eye was 20/200. Fundus examination revealed a few yellow spots within a round-shaped macular lesion. Autofluorescence imaging showed hyperautofluorescence in the lesion. Central amplitudes in multifocal electroretinogram (mfERG) were depressed. The patient reported a rhinopharyngitis 7–10 days before the visual loss. The patient was diagnosed as acute retinal pigment epithelitis. BCVA improved gradually up to 20/20 in 4 weeks. mfERG amplitudes returned to normal. A slight pigmentary distortion was the only residual fundus finding.     

Multifocal electroretinogram in diabetic subjects

PurposeTo identify local retinal abnormalities and evaluate the nature and extent of retinal dysfunction in diabetics using full field electroretinogram (ERG) and multifocal ERG (MF-ERG) and to determine the correlation between features of optical coherence tomography (OCT) and MF-ERG.MethodsTwenty-eight normal subjects (Control Group; 56 eyes) and 37 patients (72 eyes) with diabetes mellitus (DM Group) were evaluated. In the DM Group, 17 eyes had no retinopathy (grade 1), 18 eyes had early non proliferative diabetic retinopathy (NPDR) (grade 3), 16 eyes had late NPDR (grade 4), 21 eyes had proliferative diabetic retinopathy (PDR) (grade 5). Full field ERG and MF-ERG, were used to assess the effects of diabetic retinopathy on retinal function. OCT and fluorescein angiography were used to assess and compare morphological changes with functional changes in diabetes mellitus.ResultsIn diabetic patients without retinopathy (17 eyes), the amplitudes of the second order component of MF-ERG were reduced and implicit times were delayed, while only implicit times of first order component of MF-ERG were delayed but the amplitudes of first order component were normal. In diabetic patients with retinopathy (55 eyes), the overall amplitudes were reduced and peak implicit time increased in the first order component and second order component.OCT of the DM Group showed the fovea of eyes with edema were thicker than the Normal Group. The fovea of eyes with cystoid macular edema (CME) were significantly thicker than the fovea of eyes with diffuse swelling. The implicit times of MF-ERG were directly correlated with foveal thickness.ConclusionMF-ERG reveals local retinal dysfunction in diabetic patients. MF-ERG offers the advantage of topographic mapping of retinal dysfunction. The magnitude of delay of MF-ERG implicit time reflects the degree of local clinical abnormalities in eyes with retinopathy. Local response delays found in eyes without retinopathy detects subclinical local retinal dysfunction in diabetics. The combination of OCT and MF-ERG may provide objective criteria for evaluation and assessment of diabetic retinopathy.     

Multifocal electroretinogram in multiple evanescent white dot syndrome

Electrophysiologic findings including multifocal electroretinogram and visual evoked cortical potentials were studied in a patient with multiple evanescent white dot syndrome. A 19-year-old woman was diagnosed as having multiple evanescent white dot syndrome because of decreased visual acuity, white dots on fundus examination and hyperfluorescence of the white dots in the right eye. The amplitude of the flicker electroretinogramwas reduced, but the single-flash electroretinogram was within the normal range. The P100 latency of the pattern visual evoked cortical potentials was slightly prolonged. The amplitudes of multifocal electroretinogram were, markedly reduced in the area corresponding to the scotoma and moderately reduced in other regions of the central field in the affected eye. The results suggest that the retinal dysfunction area is wider than that detected by subjective methods or conventional electrophysiological examinations. The delayed recovery of the visual evoked cortical potential latency suggests the involvement of the optic nerve in multiple evanescent white dot syndrome.     

Chloroquine/hydroxychloroquine: variability of retinotoxic cumulative doses

OBJECTIVE: The critical dose of chloroquine/hydroxychloroquine leading to a maculopathy or generalised retinopathy remains undetermined. In the literature, 100 g is considered the dose at which regular vision checks should be performed. Generally, chloroquine is said to be more toxic than hydroxychloroquine. A young patient presenting with toxic maculopathy after 57 g of hydroxychloroquine and a daily dosage of 2 mg/kg body weight prompted us to retrospectively look at our patients examined in this respect over about 1 year. METHODS: The data of patients who were examined because of chloroquine/hydroxychloroquine intake or a respective maculopathy/retinopathy were retrospectively analysed. The time period was January 2005 until March 2006. Retinal damage was defined by fundus changes and alteration of the multifocal electroretinogram (ERG). RESULTS: Twenty-one patients--18 women and three men--were examined. The mean age was 51 years (range 6-71). Five of the nine chloroquine-treated patients developed a maculopathy, and one of them developed an additional generalised retinopathy. Of the patients treated by hydroxychloroquine, three of 12 suffered from a maculopathy and one from an additional generalised retinopathy. The cumulative doses leading to retinal damage ranged from 170 g to 1650 g for chloroquine and from 57 g to 1190 g for hydroxychloroquine. The highest cumulative doses without leading to signs of retinopathy were 790 g for chloroquine and 1200 g for hydroxychloroquine. CONCLUSIONS: There is a high variability of cumulative doses of chloroquine/hydroxychloroquine that lead to a toxic retinopathy. Therefore, early and regular ophthalmologic examinations are recommended. Electrophysiological testing should be performed once a year, corresponding to about 60 g of base with one tablet a day. For electrophysiology, the multifocal ERG has turned out to be the most important test in this regard. However, visual acuity and funduscopy should be performed more frequently.     

Melanoma-associated retinopathy with unknown primary site in a Japanese woman

BACKGROUND: We report the clinical features of the first case of a Japanese person with melanoma-associated retinopathy. CASE: A 44-year-old woman complained of photopsia and blurred vision in her right eye, and was treated with steroids for uveitis by an ophthalmologist. She was referred to our hospital for further examination. After one month of treatment, she still complained of photopsia in her right eye. The best corrected visual acuity in the right eye was 0.8 and these was sensitivity loss in the central visual field test. Ophthalmoscopy and fluorescein angiography showed some retinal vasculitis in the right eye. A full-field electroretinogram demonstrated a negative-type electroretinogram (ERG) waveform with attenuation of the b-wave amplitude in the right eye. A dark adaptation test revealed sensitivity loss of the rods. The lymph nodes on the right side of her neck were examined and the diagnosis was made of metastic cutaneous melanoma with unknown primary site; her visual dysfunction was diagnosed as melanoma-associated retinopathy. The retinal inflammation improved after steroid treatment, but her visual dysfunction remained. Chemotherapy and an immunotherapy regimen was begun, but 36 months later she died of metastatic melanoma in the lungs. CONCLUSIONS: A woman treated for uveitis without any prior systemic and ocular diseases was diagnosed with melanoma-associated retinopathy and metastatic melanoma in the cervical lymph nodes of unknown primary origin. The first ocular symptoms were photopsia and blurred vision, not night blindness. ERG was useful for diagnosing this rare ocular condition in an early stage.