1/297. Human psychophysical analysis of receptive field-like properties: V. Adaptation of stationary and moving windmill target characteristics to clinical populations.This paper describes the application of several key parameters of a windmill-shaped target (used in determinations of the psychophysical transient-like function) to clinical populations as a diagnostic tool for static perimetry. A technique for independently analyzing sustained- (Westheimer function) and transient-like retinally-based psychophysical responses is outlined, and stimulus characteristics, reliability and diagnostic potential of the tests are examined. Several particularly interesting clinical cases (one closure of a branch retinal arteriole) and two senile macular degeneration patients) are presented for illustrative purposes. In addition, a tentative 'working hypothesis' is presented as a basis for extensive future analysis of various clinical populations.- - - - - - - - - - ranking = 1keywords = degeneration (Clic here for more details about this article) |
2/297. Successful treatment of acute subretinal hemorrhage in age-related macular degeneration by combined intravitreal injection of recombinant tissue plasminogen activator and gas.Subretinal hemorrhage secondary to age-related macular degeneration (AMD) has a poor visual prognosis. Surgical drainage of the blood improves visual acuity only in selected patients. We report on two elderly patients with spontaneous subretinal hemorrhage from AMD. In one eye, recombinant tissue plasminogen activator (rTPA), combined with a long-acting gas (SF6), was injected into the vitreous cavity. The other eye was treated first by gas instillation followed 3 days later by rTPA injection. Both treatments led to nearly complete displacement of the subretinal hemorrhage from the macular region. In both eyes, an inferior exudative retinal detachment reabsorbed spontaneously within 2 weeks. Bilateral vitreous opacities after rTPA injection resolved without further treatment. Postoperative visual acuity increased to 0.3 and 0.4. The combined treatment is a valuable method for management of acute subretinal hemorrhage. Rapid displacement of this abnormality can minimize clot-induced damage of the highly sensitive macula and increase visual acuity.- - - - - - - - - - ranking = 5keywords = degeneration (Clic here for more details about this article) |
3/297. A novel ABCR nonsense mutation responsible for late-onset fundus flavimaculatus.PURPOSE: To report the ophthalmologic features of a novel truncating mutation in the ABCR gene in a patient affected with late-onset fundus flavimaculatus (FFM). methods: A complete ophthalmologic examination was performed in a 70-year-old patient, including best-corrected visual acuity measurement, slit lamp and fundus examination, fundus photographs, frequent fluorescein and indocyanine green angiographies, visual field testing, color vision analysis, electroretinogram, and electro-oculogram. The 50 exons of the ABCR gene were analyzed using direct sequencing. RESULTS: Fluorescein and indocyanine green angiographies confirmed the diagnosis of FFM. A heterozygous base change was found, resulting in the substitution of an arginine to a stop at codon 152 of the ABCR gene. CONCLUSIONS: A heterozygous nonsense ABCR gene mutation was found in a patient affected with FFM. No other mutation has been identified in the entire coding sequence and the promoter region, suggesting that a heterozygous severe ABCR mutant may be responsible for a mild and delayed FFM phenotype, different from that of age-related macular degeneration.- - - - - - - - - - ranking = 1keywords = degeneration (Clic here for more details about this article) |
4/297. Macular translocation with retinotomy and retinal rotation for exudative age-related macular degeneration.PURPOSE: To determine the effectiveness of macular translocation with retinotomy and retinal rotation in exudative age-related macular degeneration. methods: After written informed consent was obtained, 20 patients underwent macular translocation. We created a 180-degree retinotomy superior, inferior, and temporal to the macula near the equator. The hinged retinal flap was rotated superiorly or inferiorly to place the center of the fovea over an area of healthy retinal pigment epithelium. The retina was flattened under silicone oil and laser photocoagulation was placed. RESULTS: The fovea was moved 425 to 1,700 microm (965 /-262 microm) superiorly or inferiorly. Follow-up time was 2 to 12 months (median 8 months). Complications included macular pucker (3 eyes), subfoveal hemorrhage (2 eyes), macular hole (1 eye), and progression of cataract in phakic eyes (3 eyes). Thirteen of 20 eyes showed various degrees of proliferative vitreoretinopathy with epiretinal membrane formation over the inferior peripheral retina with the inferior retinal detachment stabilized by the silicone oil. One eye progressed to phthisis bulbi. Initial visual acuity ranged from 20/80 to 20/800 (median 20/150) and final visual acuity ranged from light perception to 20/200 (median 20/1000). CONCLUSION: The fovea can be moved up to 1,700 microm with retinotomy and retinal rotation; however, there is a high rate of complications. Proliferative vitreoretinopathy is the major complication of this technique and is probably related to the extensive retinotomy and subretinal irrigation inherent in the technique. Other techniques such as scleral shortening may have fewer complications.- - - - - - - - - - ranking = 5keywords = degeneration (Clic here for more details about this article) |
5/297. Allogenic fetal retinal pigment epithelial cell transplant in a patient with geographic atrophy.PURPOSE: To test the hypothesis that healthy fetal retinal pigment epithelium (RPE) can rescue the remaining viable RPE and choriocapillaries and thereby the photoreceptors in non-neovascular age-related macular degeneration (ARMD) (geographic atrophy [GA]). methods: A 65-year-old legally blind woman with non-neovascular ARMD underwent fetal RPE transplantation. Best-corrected visual acuity testing, detailed fundus examination, fundus photography, fluorescein angiography, scanning laser ophthalmoscope macular perimetry, and humoral and cellular immune response testing were performed. A suspension of RPE was infused into the subretinal space through a retinotomy along the superotemporal arcade at the edge of the area of GA. The patient did not take systemic immunosuppressants. RESULTS: The patient's vision remained unchanged for 5 months after the surgery. fluorescein angiography after transplantation showed leakage and staining at the level of the outer retina. There was progressive subretinal fibrosis in the area of the transplant. Immune response studies showed a weakly positive mixed lymphocyte response against phosducin and rhodopsin. CONCLUSION: Although it is surgically feasible to transplant fetal RPE to the subretinal space of patients with GA, such an allogenic RPE transplant without immunosuppression leads to leakage on fluorescein angiography and eventual fibrosis. A very weak immune response against proteins associated with photoreceptors is also of concern.- - - - - - - - - - ranking = 1keywords = degeneration (Clic here for more details about this article) |
6/297. Progressive cone dystrophies.patients with progressive generalized cone dystrophy often present nystagmus (or strabism) and complain of photophobia, decrease in visual acuity or disturbances in colour perception. The most classic fundus abnormality is the bull's eye maculopathy or a pallor of the optic disc. Minimal macular changes are sometimes seen, which may progress to a bull's eye type of macular degeneration. The photopic ERG is always very affected, whereas at first the scotopic ERG seems normal. Progressive deterioration of the visual functions is accompanied by increasing fundus lesions and rod involvement, as suggested by the modifications of the dark adaptation curve and the scotopic ERG. However, the progression of typical generalized cone dysfunction is very slow. On the contrary, in some cases of so-called Stargardt's disease with peripheral participation, a very rapid progression has been observed. In such cases a normal ERG does not necessarily mean that the disease will remain localized to the macular area. No definite prognosis can be made on one single ERG. In 3 cases with sector pigmentary retinopathy the photopic ERG was more affected than the scotopic ERG. However, these cases are probably primary cone-rod dystrophies. Although there is no electrophysiological control, our clinical impression is that the evolution, if possible, is very slow.- - - - - - - - - - ranking = 1keywords = degeneration (Clic here for more details about this article) |
7/297. Sorsby's fundus dystrophy in two Japanese families with unusual clinical features.PURPOSE: To describe two Japanese families with Sorsby's fundus dystrophy (SFD) with unusual clinical features. methods: Two families from Kagoshima Prefecture with senile-onset macular dystrophy were examined. Three affected individuals through three successive generations of one family and three affected siblings in another family were examined and followed. RESULTS: The initial symptom of these patients was a rapid or slow central visual loss that occurred at an average age of 67.4 years. The major ophthalmoscopic changes consisted of soft drusen and hemorrhagic or atrophic lesions in the macula, which were progressive and ultimately led to disciform scarring. They had no difficulty with night vision. All the patients had normal peripheral retina with intact peripheral fields. They maintained good ambulatory vision and could walk unguided until late in life. These patients had a novel mutation in the tissue inhibitor of the metalloproteinases-3 (TIMP3) gene. CONCLUSIONS: This is the first report of SFD from the East. Its clinical features differ from those of SFD patients of the West, appearing closer to features of age-related macular degeneration. These two unrelated Japanese families with an identical mutation in the TIMP3 gene might be descendants of a common ancestor who carried the mutant gene.- - - - - - - - - - ranking = 1keywords = degeneration (Clic here for more details about this article) |
8/297. Estrogen and visual hallucinations in a patient with Charles Bonnet syndrome.PURPOSE: To describe the occurrence of visual hallucinations in a patient with Charles Bonnet syndrome associated with estrogen intake. METHOD: Case report. RESULTS: An 84-year-old woman with poor visual acuity secondary to bilateral, nonexudative, age-related macular degeneration had nonthreatening visual hallucinations 2 weeks after starting oral estrogen for osteoporosis. The estrogen was stopped, and the hallucinations subsided. The patient was given estrogen twice more and each time the hallucinations recurred. CONCLUSION: We report a case of Charles Bonnet syndrome associated with estrogen intake in an 84-year-old woman. Estrogen may have promoted release phenomena and triggered the hallucinatory episodes in our patient.- - - - - - - - - - ranking = 1keywords = degeneration (Clic here for more details about this article) |
9/297. Improvement in macular function after retinal translocation surgery in a patient with age-related macular degeneration.PURPOSE: To assess focal electroretinographic findings before and after retinal translocation surgery in a patient with age-related macular degeneration. METHOD: Case report. A 79-year-old man with a well-defined subfoveal choroidal neovascular membrane from age-related macular degeneration underwent preoperative and postoperative focal electroretinography. RESULTS: After retinal translocation surgery, best-corrected Snellen visual acuity improved from 9/200 to 20/60. A significant increase in mean foveal amplitude, from 0.08 microV to 0.16 microV (P = 0.008) was recorded. CONCLUSION: Age-related macular degenerative changes in visual acuity and foveal electroretinogram amplitude may be reversible after retinal translocation surgery.- - - - - - - - - - ranking = 6keywords = degeneration (Clic here for more details about this article) |
10/297. ICGA-guided laser photocoagulation of feeder vessels of choroidal neovascular membranes in age-related macular degeneration. indocyanine green angiography.PURPOSE: To report the ability of indocyanine green angiography (ICGA) with a confocal scanning laser ophthalmoscope (SLO) to identify feeder vessels of choroidal neovascular membranes (CNVM) secondary to age-related macular degeneration (ARMD) and to show the feasibility of inducing complete closure of the CNVM by photocoagulation targeted exclusively to the feeder vessels. methods: Five consecutive patients with exudative ARMD in whom ICGA with the confocal SLO showed extrafoveal feeder vessels supplying choroidal neovascular nets had laser photocoagulation done only to the feeder vessels. In two patients, two separate membranes were seen. RESULTS: Laser photocoagulation resulted in closure of the feeder vessels and the CNVM in four patients. Complete closure was achieved with one treatment in one patient and with two treatments in three patients. In one patient, two treatments failed to close the feeder vessel and the CNVM, but a third, more intense laser treatment resulted in temporary closure of the feeder vessel and CNVM, which recanalized 2 to 4 weeks later with development of a large rip in the retinal pigment epithelium. In one patient, two separate CNVMs grew from the edge of the laser scars, but they were not directly related to the original CNVM and its feeder vessel, and were treated successfully. The same eye later developed subfoveal occult CNVM with gradual deterioration of visual acuity. In the other four patients, visual acuity improved in two and was unchanged in two. CONCLUSIONS: indocyanine green angiography with the confocal SLO can identify choroidal feeder vessels supplying CNVM secondary to ARMD. Laser treatment to such extrafoveal feeder vessels, particularly in membranes that are large or subfoveal, may be effective in closing the feeder vessel and CNVM with preservation of the fovea and central vision. More than one treatment may be required, however, and failures and complications may be expected with this treatment modality.- - - - - - - - - - ranking = 5keywords = degeneration (Clic here for more details about this article) |
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