1/196. 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) |
2/196. Treatment of paraneoplastic visual loss with intravenous immunoglobulin: report of 3 cases.BACKGROUND: Paraneoplastic visual loss is an autoimmune disorder believed to be caused by the remote effects of cancer on the retina (cancer-associated retinopathy [CAR]) or optic nerve. Both disorders may result in rapid and complete blindness. Spontaneous recovery of vision has not been reported. The serum of patients with CAR contains autoantibodies against recoverin, enolase, or unidentified retinal proteins. autopsy examination results of eyes of blind patients with CAR show complete absence of the retinal neurons involved in phototransduction. Corticosteroids and plasmapheresis are the only treatment options previously described. OBJECTIVE: To treat paraneoplastic visual loss. DESIGN AND methods: Three patients with metastatic cancer developed rapidly progressive loss of vision. The first patient had visual acuity of hand movements in each eye before intravenous immunoglobulin treatment. The second patient had visual acuity of light perception in both eyes. The third patient's visual acuity was 20/400 OD and 20/20 OS. Diagnostic tests included magnetic resonance imaging of the head and cytologic examination of the cerebrospinal fluid to exclude metastasis as the cause of visual loss and then an electroretinogram and serum tests for autoantibodies against retinal antigens to confirm the clinical diagnosis of CAR. patients 1 and 2 were treated with intravenous immunoglobulin (400 mg/kg per day) for 5 days; however, patient 3 received only a single dose due to adverse effects consisting of shortness of breath and itching. RESULTS: Within 24 hours of taking the first dose of intravenous immunoglobulin, the visual acuity of patient 1 improved from hand movements only in both eyes to 20/50 OD and 20/200 OS. After the third day of treatment, visual acuity in the left eye further improved to 20/40. Even with the improved acuity, Goldmann visual field perimetry results showed poor responses in both eyes. However, 2 weeks later there was marked visual field improvement, and visual acuity was maintained at 20/50 OD and 20/40 OS. Patient 2 had no improvements and continued to have light perception in both eyes. Patient 3 had improvements in visual field defects but remained 20/400 OD and 20/20 OS. CONCLUSION: Intravenous immunoglobulin may be another treatment option offered to patients with paraneoplastic visual loss in addition to corticosteroids or plasmapheresis because a review of the medical literature has shown no spontaneous improvements of visual function without treatment.- - - - - - - - - - ranking = 40.399331647799keywords = retinopathy (Clic here for more details about this article) |
3/196. Ocular findings in ichthyosis follicularis, atrichia, and photophobia syndrome.ichthyosis follicularis, atrichia, and photophobia (IFAP) are typical features of a rare neuroichthyosis termed IFAP syndrome. We demonstrate the ultrastructural findings of the eyes from a 33-year-old patient with IFAP syndrome. Clinically, eyebrows and eyelashes were absent from birth, and photophobia was noted at the age of 1 year. The globes measured 28 and 29 mm, respectively, and both eyes showed a posterior staphyloma. Histopathologically, bilateral centrally located subepithelial avascular corneal scarring with secondary corneal amyloid deposition was found. In addition to already described ocular abnormalities in IFAP syndrome we demonstrate ultrastructural anomalies of desmosomes and tonofilaments in corneal epithelium; defects of basement membrane, Bowman layer, and anchoring fibrils; secondary corneal amyloid deposition; and keratocyte degeneration. A defective tear film, recurrent atopic keratoconjunctival inflammations, or a primary anomaly of corneal epithelial adhesion are potential causes for the corneal defects. photophobia is most likely due to corneal abnormalities.- - - - - - - - - - ranking = 1keywords = degeneration (Clic here for more details about this article) |
4/196. Macular fibrosis associated with talc retinopathy.PURPOSE: To investigate a patient with talc retinopathy who developed macular fibrosis with resultant visual loss. methods: A 64-year-old intravenous drug abuser was evaluated for bilateral peripheral retinal neovascularization. He admitted to abusing oral methylphenidate intravenously. Funduscopy showed numerous intravascular talc particles in the macular area of both eyes. Over a period of next 4 years, visually significant macular fibrosis gradually developed in both eyes, the left eye more than the right eye. RESULTS: fluorescein angiography confirmed the presence of bilateral peripheral retinal neovascularization with adjacent areas of ischemic retina. The patient was treated with peripheral laser treatment to the ischemic retina with resultant regression of peripheral retinal neovascularization. Bilateral macular fibrosis accounted for the reduced vision in our patient. CONCLUSION: talc retinopathy can be associated with macular fibrosis with resultant visual loss.- - - - - - - - - - ranking = 242.39598988679keywords = retinopathy (Clic here for more details about this article) |
5/196. 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 = 42.877716412589keywords = retinopathy, dystrophy, degeneration (Clic here for more details about this article) |
6/196. Ocular toxicity of systemic medications: a case series.BACKGROUND: There are many visually threatening conditions that may result from long-term use of systemic medications. Many of these adverse side effects can be greatly reduced or prevented with close monitoring of patients. In view of current knowledge, updated clinical guidelines for appropriate monitoring of ocular toxicity from systemic medications need to be developed for the eye care practitioner. CASE review: There have been many reports of ocular toxicity from isoniazid, thioridazine, steroids, and amiodarone therapy. Clinical cases illustrating possible adverse ocular side effects are presented, which include INH-induced optic neuropathy, phenothiazine-induced retinopathy, steroid-induced glaucoma, and vortex epitheliopathy secondary to amiodarone. CONCLUSION: Optometrists should be aware of the potential for ocular side effects from systemic medications. Eye care guidelines for monitoring ocular side effects from thioridazine, INH, steroids, and amiodarone use are suggested.- - - - - - - - - - ranking = 40.399331647799keywords = retinopathy (Clic here for more details about this article) |
7/196. Childhood blindness and visual loss: an assessment at two institutions including a "new" cause.PURPOSE: This study was initiated to investigate the causes of childhood blindness and visual impairment in the united states. We also sought a particular etiology--congenital lymphocytic choriomeningitis virus (LCMV)--which has been considered exceedingly rare, in a fixed target population of children, the severely mentally retarded. methods: We undertook a library-based study of the world literature to shed light on the causes of childhood blindness internationally and to put our data in context. We prospectively examined all consented children (159) at 2 institutions in the united states to determine their ocular status and the etiology of any visual loss present. One of the institutions is a school for the visually impaired (hereafter referred to as Location V), in which most of the students have normal mentation. The other is a home for severely mentally retarded, nonambulatory children (hereafter referred to as Location M). This institution was selected specifically to provide a sample of visual loss associated with severe retardation because the handful of cases of LCMV in the literature have been associated with severe central nervous system insults. Histories were obtained from records on site, and all children received a complete cyclopleged ophthalmic examination at their institution performed by the author. patients at Location M with chorioretinal scars consistent with intrauterine infection (a possible sign of LCMV) had separate consents for blood drawing. Sera was obtained and sent for standard TORCHS titers, toxoplasmosis titers (Jack S. Remington, MD, Palo Alto, Calif), and ELISA testing for LCMV (Centers for Disease Control and Prevention, Atlanta, Ga). RESULTS: The diagnoses at Location V were varied and included retinopathy of prematurity (19.4%), optic atrophy (19.4%), retinitis pigmentosa (14.5%), optic nerve hypoplasia (12.9%), cataracts (8.1%), foveal hypoplasia (8.1%), persistent hyperplastic primary vitreous (4.8%), and microphthalmos (3.2%). The most common diagnosis at Location M was bilateral optic atrophy, which was found in 65% of the patients examined who had visual loss. Of these, the insults were most often congenital (42.6%), with birth trauma, prematurity, and genetics each responsible for about 15% of the optic atrophy. The second most common diagnosis was cortical visual impairment (24%), followed by chorioretinal scars (5%), which are strongly suggestive of intrauterine infection. Of 95 patients examined at Location M, 4 had chorioretinal scars. Two of these had dramatically elevated titers for LCMV, as did one of their mothers. One of the other 2 children died before serum could be drawn, and the fourth had negative titers for both TORCHS and LCMV. CONCLUSIONS: At both locations studied, visual loss was most often due to congenital insults, whether genetic or simply prenatal. The visual loss at Location V was twice as likely as that at Location M to be caused by a genetic disorder. The genetic disorders at Location V were more often isolated eye diseases, while those among the severely retarded at Location M were more generalized genetic disorders. Our study identified optic atrophy as a common diagnosis among the severely mentally retarded with vision loss, a finding that is supported by previous studies in other countries. In our population of severely retarded children, the target etiology of lymphocytic choriomeningitis virus was responsible for half the visual loss secondary to chorioretinitis from intrauterine infection. This is more common than would be predicted by the few cases previously described in the literature, and strongly suggests that LCMV may be a more common cause of visual loss than previously appreciated. We believe that serology for LCMV should be part of the workup for congenital chorioretinitis, especially if the TORCHS titers are negative, and that perhaps the mnemonic should be revised to "TORCHS L." Childhood blindness and visual impairment are tragic and co- - - - - - - - - - ranking = 68.185126623757keywords = retinopathy, retinitis pigmentosa, retinitis, pigmentosa (Clic here for more details about this article) |
8/196. 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) |
9/196. Enlarged gamma band response of neuromagnetic auditory evoked fields in a visually impaired subject.Under acoustic stimulation a phase-locked response in the gamma band (near 40 Hz) in the latency range between 20 and 130 ms is evoked. We report on a considerably visually impaired woman with Gronblad-Strandberg syndrome which involves degeneration at the level of retina, but has no overt central nervous component to the degeneration. The subject exhibited an extraordinarily high power in the phase-locked gamma band response (GBR) which was found to be more than three, and sometimes more than four, standard deviations above the average of a group of 25 subjects with normal vision. Furthermore, the dipoles of her mismatch reaction and M200 were found to be located posteriorly to the dipoles of the M100. overall, both enlarged GBR and changed cortical representation could be results of cortical plasticity related to visual impairment.- - - - - - - - - - ranking = 2keywords = degeneration (Clic here for more details about this article) |
10/196. Corticobasal ganglionic degeneration with Balint's syndrome.Corticobasal ganglionic degeneration (CBGD) is a neurodegenerative dementia characterized by asymmetric parkinsonism, ideomotor apraxia, myoclonus, dystonia, and the alien hand syndrome. This report describes a patient with CBGD who developed Balint's syndrome with simultanagnosia, oculomotor apraxia, and optic ataxia.- - - - - - - - - - ranking = 5keywords = degeneration (Clic here for more details about this article) |
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