Cases reported "Retinal Diseases"

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1/29. 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.
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2/29. melanoma-associated retinopathy versus abnormal retinal function due to interferon-alpha/isotretinoin therapy in cutaneous malignant melanoma.

    PURPOSE: To analyze whether an abnormal retinal function in patients with a cutaneous malignant melanoma was due to paraneoplastic retinopathy or due to isotretinoin or interferon-alpha. methods: We studied 15 patients with malignant melanoma in stage IIa and IIb who are all participants in a randomized, multicentered, double-blind placebo-controlled clinical trial comparing interferon-alpha/isotretinoin versus interferon-alpha/placebo performed by the Department of dermatology, University of Graz. Our assessment included a full ophthalmic history and examination, electrophysiological testing (ERG, EOG), dark adaption, color vision and visual field testing. RESULTS: The most prevalent ocular symptom patients complained about was ocular dryness (8 patients). Electrophysiological as well as psychophysical testings showed no abnormalities in 12 patients. In 1 patient the therapy was stopped because of electrophysiological and psychophysiological pathology. This patient suffered from severe reduction of night vision and visual disturbances. Another patient had had night blindness since childhood which remained stable. CONCLUSIONS: We postulate that in 1 of 15 patients, visual complaints are caused with a high probability by melanoma-associated retinopathy although, in the literature, isotretinoin is described to show similar effects on retinal function.
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3/29. Bacterial subretinal abscess: a case report and review of the literature.

    PURPOSE: To report a case of klebsiella subretinal abscess with a successful visual outcome with treatment and to review the literature pertaining to focal intraocular infection in bacterial endophthalmitis. methods: Clinical data including medical history, findings on physical examination, blood cultures, and an abdominal computed tomographic scan were collected in a 32-year-old man with klebsiella sepsis, liver abscesses, and a focal subretinal abscess. Ocular data including visual acuity, fundus photographs, fluorescein angiography, and ultrasound were evaluated, as were results of culture and histopathologic studies. RESULTS: Despite immediate intervention, including vitreous tap and intravitreal antibiotics, the eye deteriorated, with enlargement of the abscess. A pars plana vitrectomy was performed in which the subretinal abscess material was removed after an extensive retinectomy of the involved area in association with an endophotocoagulative barrier and intravitreal amikacin without gas or oil tamponade. culture confirmed klebsiella subretinal infection. A retinal detachment occurred 1 month postoperatively and was successfully repaired. visual acuity was 20/30 and has remained stable for 14 months. CONCLUSION: klebsiella endophthalmitis with subretinal abscess formation is a rare but devastating ocular condition. In the present case, prompt intervention with extensive retinectomy, complete abscess excision, and intravitreal antibiotic therapy resulted in unprecedented visual recovery.
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4/29. Unilateral cone dysfunction with bull's eye maculopathy.

    OBJECTIVE: To report a Japanese subject who presented with an acute-onset, unilateral cone dysfunction with bull's eye maculopathy and to describe the functional changes determined by psychophysical and electrophysiologic tests. DESIGN: A single observational case report. methods: In addition to a complete ophthalmic examination, the subject underwent some electrophysiologic and psychophysical tests. MAIN OUTCOME MEASURES: Kinetic visual field test, cone and rod perimetry (two-color perimetry), full-field electroretinograms (ERGs), focal macular ERGs, and multifocal ERGs. RESULTS: The full-field ERGs and two-color perimetry showed a predominant loss of cone function in the right eye, whereas the left eye was normal. Cone perimetry and multifocal ERGs revealed that there were small regions functioning normally and other retinal areas that were severely altered in the right eye. CONCLUSIONS: The topographical function analysis suggested that the disorder affected the retina unevenly. The cause of this rare case of unilateral cone dysfunction with bull's eye maculopathy still remains unknown.
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5/29. Idiopathic central serous chorioretinopathy--a physical complication of stress?

    The adverse psychological sequelae of stress are well recognized by occupational health specialists. Potential adverse physical effects, such as ischaemic heart disease, are more contentious but are biologically plausible. This report outlines a case of idiopathic central serous chorioretinopathy (ICSC), an uncommon but potentially sight-threatening condition, which is widely accepted amongst ophthalmologists to be stress related. The condition is not referred to in standard occupational health texts or databases. The report includes a brief review of the ophthalmological literature on which the connection between ICSC and stress has been made, and a need for further research promoted.
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6/29. Presumed sertraline maculopathy.

    PURPOSE: To report a unique case of a woman who developed simultaneous bilateral maculopathy presumed to result from intake of sertraline hydrochloride, a serotonin reuptake inhibitor. methods: Full clinical ocular examination, color vision testing, automated static perimetry, electroretinography, electrooculography and fundus fluorescein angiography were performed. Living members of her family were also examined. RESULTS: The patient had normal electroretinography and electrooculography results. Automated static perimetry showed generalized reduction of sensitivity and central scotomas. Macular lesions resolved 6 months after discontinuation of sertraline, however, during twenty months of follow-up her visual acuity and abnormalities in other psychophysical tests did not improve. CONCLUSION: patients started on sertraline should be informed of the potential risk of developing maculopathy, and they should be examined regularly to detect possible early alterations.
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7/29. gyrate atrophy of the choroid and retina: further experience with long-term reduction of ornithine levels in children.

    OBJECTIVE: To determine whether the long-term reduction of plasma ornithine levels by way of an arginine-restricted diet in patients with gyrate atrophy will slow the progression of this chorioretinal degeneration. DESIGN: natural history study of 2 pairs of siblings with gyrate atrophy treated with an arginine-restricted diet. MAIN OUTCOME MEASURES: Fundus photography and electrophysical and psychophysical retinal function tests. RESULTS: After 16 to 17 years of receiving an arginine-restricted diet, the younger sibling in each pair, who was prescribed the diet at an earlier age than the older sibling, demonstrated a slower progression of lesions compared with the older sibling. CONCLUSIONS: If started at an early age, long-term substantial reduction of plasma ornithine levels may appreciably slow the progression of the chorioretinal lesions and, to a lesser extent, the progressive loss of retinal function in patients with gyrate atrophy.
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8/29. Intrauterine west nile virus: ocular and systemic findings.

    PURPOSE: To report the first documented case of intrauterine transmission of west nile virus (WNV) with resulting congenital chorioretinal scarring and central nervous system malformation in a newborn. DESIGN: Case report. methods: Ophthalmic findings and laboratory data in an otherwise presumed healthy 2-day-old female are presented. The infant's mother developed paraplegia due to WNV during the second trimester of her pregnancy. The newborn's external and general physical examination were unremarkable. RESULTS: Ophthalmic examination disclosed marked chorioretinal changes, and magnetic resonance imaging of the brain demonstrated severe abnormalities. serology for WNV was positive. Other causes of congenital chorioretinal changes were ruled out with the appropriate serology. CONCLUSIONS: Intrauterine transmission of WNV may result in significant ocular and neurologic morbidity. Titers for this important and emerging viral pathogen should be obtained when standard serologies are negative in an infant with congenital chorioretinal scarring.
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9/29. Atypical Vogt-Koyanagi-Harada disease or new uveomeningitic syndrome?

    PURPOSE: To report on a patient affected by bilateral intermediate uveitis (IU) as the initial sign of an uveomeningitic syndrome. methods: Thorough history, physical examination and ancillary laboratory and radiological testing were performed in this observational case study. RESULTS: A 23-year-old Caucasian man developed bilateral IU, primarily diagnosed as "idiopathic" since a detailed etiologic work-up was not indicative of underlying disease. Seven months later, he presented with poliosis and vitiligo. Lumbar puncture revealed cerebrospinal fluid pleocytosis. Optical coherence tomography showed bilateral subclinical macular edema (ME). The visual acuity was still 20/20 in both eyes. Clinical, laboratory and radiological results did not fit into any known syndrome. CONCLUSIONS: According to all the tests performed, the disease in our patient is a uveomeningitic disease with IU and ME which could be interpreted as an atypical form of Vogt-Koyanagi-Harada disease or a new uveomeningitic syndrome because there is no evidence for any other known disease.
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10/29. Callosal agenesis, chorioretinal lacunae, absence of infantile spasms, and normal development: aicardi syndrome without epilepsy?

    aicardi syndrome is defined by the clinical triad of infantile spasms, agenesis of the corpus callosum, and pathognomonic chorioretinal lacunae. Almost all patients are females with severe cognitive and physical disabilities. All of the cases reported in the literature have had early-onset seizures. Most cases of aicardi syndrome exhibit very slow development, even when seizures are eventually controlled, and the cases with a relatively favourable outcome are associated with low intelligence quotient levels. A relationship between chorioretinal changes or severity of the agenesis of the corpus callosum and prognosis of aicardi syndrome has been claimed, but few data are available about the clinical features that can predict clinical outcome. We describe a case of aicardi syndrome in a female aged 24 months. magnetic resonance imaging showed complete agenesis of the corpus callosum and ophthalmoscopy revealed chorioretinal lacunae in the left eye. She had never had seizures and her psychomotor and language development were normal for age.
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