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PubMedDate not supplied

Psychosis in a patient with Usher syndrome: a case report.

The present report is the case of a 26-year-old man, born with Usher syndrome. The patient had had a significant hearing impairment since birth and had developed retinitis pigmentosa. He had originally been diagnosed with a depressive disorder and treated with antidepressants, with no subsequent improvement in his mental state. Following a deterioration in his mental state he was admitted for reassessment at the Queen Elizabeth Psychiatric Hospital, Birmingham, and antidepressants were stopped. It subsequently became apparent from observations, interviews with the patient and information from the patient's carers and relatives that he had a psychotic illness. Treatment was started with the antipsychotic drug risperidone, after which he showed significant improvement. The association between Usher syndrome and psychosis is discussed.

Adult↗
PubMed1992

Differential cell adhesion and expression of N-cadherin among retinoblastoma cell lines.

Retinoblastoma (RB) is the most common intraocular childhood cancer, but little is known regarding its cellular biology and the factors that might regulate its progression and metastatic potential. Although alterations in cell-adhesion interactions could contribute to the metastatic behavior of RB, no specific cell-adhesion molecules have been identified in RB cells. The current study examined the adhesive properties of three RB cell lines (Weri-Rb1, Weri-Rb27, and Y79) and determined whether N-cadherin, a specific cell-adhesion protein expressed during normal retinal development, was expressed by these cell lines. This study revealed marked differences in the cell-adhesive properties of the three cell lines with respect to several parameters assayed, including cell morphology, calcium dependence of the cell adhesion, and the presence of N-cadherin. N-cadherin was expressed by human RB cells, but the level of expression, determined both functionally and biochemically, varied among different RB cell lines.

Cadherins↗
PubMed2005

[Clinical analysis of early secondary glaucoma associated with ocular trauma].

OBJECTIVE: To determine the classification and management of early secondary glaucoma associated with ocular trauma. METHODS: The records of 139 patients (139 eyes) were reviewed and analyzed. RESULTS: Five different types of early secondary glaucoma associated with trauma were determined by clinical findings: hemorrhage type, chamber angle injury type, lens related type, synechia and proliferation type, and inflammation type. Each type of secondary glaucoma received different therapeutic methods. Thirty-seven cases (26.62%) received medical treatment; 32 cases (23.02%) received medical treatment and anterior chamber paracentesis or washing; 70 cases (50.36%) received other surgical procedures, including vitrectomy, filtration surgery and combine operation. After follow-up for 3 - 6 months, the intraocular pressure (IOP) was successfully controlled without anti-glaucoma medical treatment in 121 eyes (87.05%) (6 mm Hg < or = IOP < or = 21 mm Hg); IOP > 21 mm Hg was found in 13 eyes (9.35%) and IOP < 6 mm Hg in 5 eyes (3.60%). The postoperative visual acuity was decreased or lost in 7 eyes, remained the same or improved in 132 eyes. Eight eyes had hemorrhage during or after the operation. Two eyes developed retinal detachment. CONCLUSIONS: Clinical findings of early secondary glaucoma associated with trauma are complex. Each subtype of patients should take relevant different treatments.

Adolescent↗
PubMed2005

[Intraoperative choroidal hemorrhage in patient with Osler-Rendu syndrome].

UNLABELLED: The authors describe a 79-year-old man with Osler-Rendu-Weber syndrome, who developed intraoperative choroidal hemorrhage in left eye. During phacoemulsification under local anesthesia, shallowing of the anterior chamber was observed. Acetazolamide was administered and peribulbar block performed. Phacoemulsification was continued and intraocular lens implanted. Five months postoperatively resolution of the choroidal hemorrhage was observed. Best corrected visual acuity was 0.5. Patient developed retinal embolism in his right eye 16 years earlier. CONCLUSIONS: (1) Choroidal hemorrhage may occur more commonly in patients with Osler-Rendu-Weber syndrome. Recognition of this possible association and institution of appropriate intraoperative precautions may facilitate a good visual outcome. (2) Paradoxical embolization (stroke, retinal embolism) is the most serious consequence of hereditary hemorrhagic telangiectasia.

Aged↗
PubMed1992

Antimalarial agents and the eye.

Antimalarial compounds have been demonstrated to be both safe and efficacious in the treatment of lupus erythematosus as well as several other dermatologic conditions. It is imperative, however, that physicians familiarize themselves with the potential complications associated with the use of these agents. Furthermore, it is essential that a successful collaborative relationship be developed between the dermatologist and the ophthalmologist who are to treat and monitor the patient to avoid any untoward complications that might afflict the patient. It is important to stress that patients on antimalarial drug therapy be evaluated by an ophthalmologist prior to starting treatment and every 4 to 6 months thereafter. The use of Amsler's grids in compliant patients is recommended. If the patient develops retinal changes consistent with the diagnosis of premaculopathy, then careful consideration should be given to the availability and use of alternative agents.

Antimalarials↗
PubMed1990

[Diabetic retinopathy and pregnancy].

The problem concerning the influence of pregnancy on diabetic retinopathy is still a matter for discussion. The questions are: can the pregnancy itself influence the onset of a retinopathy? and can a pregnancy worsen the prognosis of a retinopathy and if it is the case is there any difference regarding the type of the retinopathy: background or proliferative? Despite controversies in the literature, it seems that: If no retinopathy is present at the onset of the pregnancy, the risk for developing retinal complications is very low. A background retinopathy can worsens and may become proliferative. But improvement is often observed in post-partum. A proliferative retinopathy worsen very frequently and the proper management of patients with this type of retinopathy is of extreme importance regarding to the risk of complications and loss of vision. Before the advent of photocoagulation, the alternative to therapeutic abortion was pituitary ablation. Now, the photocoagulation became the treatment of choice for proliferative retinopathy with the same results in pregnant or non pregnant woman. But the proliferative retinopathy is associated with a high incidence of foetal morbidity. Some studies provide evidence that changes in background retinopathy may be related to the degree of diabetic control achieved during pregnancy. The rapidity of improvement in diabetic control may be critical. Diabetic women who are wishing pregnancies may undergo ocular examination with angiography. During pregnancy strict ocular examination is necessary through dilated pupils. Angiography does not seem to induce any side effect on pregnant women.

Diabetic Retinopathy↗
PubMed1990

Intraocular foreign bodies: results of 27 cases.

Twenty-seven consecutive cases of various kinds of perforating injuries with foreign bodies, referred to the Ophthalmology Department of Hacettepe Medical School between January 1984 and April 1988, were evaluated retrospectively. Whenever technically possible magnetic extraction was the preferred method of removing the foreign body. Thirteen (48%) eyes developed endophthalmitis, 18 (66.6%) eyes developed traumatic cataract, and eight (29.6%) eyes developed retinal detachment after the trauma. Twelve (44.5%) eyes had a visual acuity of 1/10 or better after the appropriate medical and surgical treatment.

Adolescent↗
PubMed1985

Spontaneous posterior bulbar perforation of congenital scleral coloboma and its surgical treatment: a case report.

A 35-year-old male presented with abrupt hypotonic oculopathy caused by a spontaneous bulbar perforation, due to a scleral defect in the equatorial area of the nasal side of the left eye. The scleral defect was surgically repaired using lyophilized homologous dura mater encephali and fibrin glue (Tisseel). Highly satisfactory results were obtained. The pathogenesis of this scleral defect was suspected to involve complex factors related to abnormal retinal development and abnormal ocular muscles. Such a case has not been reported previously.

Adult↗
PubMed1988

Retinoid-binding proteins in retinoblastoma tumors.

A combination of Western blot, Northern blot, and radiolabeled ligand-binding techniques was used to investigate retinoid-binding proteins in retinoblastoma (RB) cells from fresh tumors and from 19 RB tumor lines cultured in vitro. Using rabbit anti-bovine cellular retinal-binding protein (CRA1BP) antibodies, no CRA1BP could be detected. As determined by [3H]retinol binding, cellular retinol-binding protein was sometimes not detectable but averaged 2.3 +/- 2.7 means +/- SD, n = 7) pmol [3H]retinol bound/mg protein, similar to adult retina cytosol. Using [3H]retinoic acid as ligand, cellular retinoic acid-binding protein was not detectable in some lines and averaged 1.0 +/- 1.2 (means +/- SD, n = 7) pmol [3H]retinoic acid bound/mg protein, well below the adult retina cytosol level of 94.4 +/- 16.3 (means +/- SD, n = 4) pmol [3H]retinoic acid bound/mg. Using rabbit antibovine interstitial retinol-binding protein (IRBP) antibodies, IRBP of the same molecular mass as human IRBP (135,000) was detected in the medium from all cultured RB cells and averaged 75.9 +/- 19.2 pmol/10(8) cells (bovine IRBP immunochemical equivalents). Cytosol levels were less than 1% of the medium. Using a human IRBP complementary DNA probe, levels of IRBP RNA transcripts in 19 RB cell lines were comparable to adult retina. The Y-79 RB cell line was different from the others; the amount of IRBP in the medium was only about 1% of the RB cell lines. Levels of cellular retinol-binding protein were comparable with the other lines, but cellular retinoic acid-binding protein was 9 times more abundant. IRBP RNA transcripts in Y-79 cells were below the limits of detectability but appeared at low levels after induction of differentiation of Y-79 by 10(-6) M retinoic acid. Although this cell line has been in culture longer than the others, it may also have been initiated at an earlier stage of retinal development.

Carrier Proteins↗
PubMed1988

Retinotopic organization of the developing retinotectal projection in the zebrafish embryo.

Developing retinal axons in the zebrafish embryo were stained with HRP or with the fluorescent dyes dil and diO to study the formation of the retinotectal projection. Retinal axons leave the eye at 34-36 hr postfertilization (PF), invade the tectum at 46-48 hr PF, and innervate the tectal neuropil at 70-72 hr PF. Dorsal and ventral axons occupy separate aspects of the optic nerve and tract and pass into their retinotopically appropriate ventral and dorsal hemitectum, respectively. Nasal and temporal axons are segregated in the nerve, mixed in the tract, and are coextensive over the rostral half of tectum until 56 hr PF. They then segregate again, due to the progression of nasal axons into the open caudal tectum. Thus, at 70-72 hr PF, dorsal and ventral as well as temporal and nasal axons occupy their retinotopically appropriate tectal quadrants. After ablation of the temporal retina prior to the time of axonal outgrowth, the nasal axons bypass the vacant rostral tectum to terminate in the caudal tectal half. Temporal axons in the absence of nasal axons remain restricted to their appropriate rostral tectal half, suggesting that nasal and temporal axons possess a preference for their retinotopically appropriate tectal domains. Measurements of individual terminal arbors and the tectal areas in embryos and in adult zebrafish showed that individual arbors are large with respect to the embryonic tectum but are about 14-15 times smaller than in the adult. However, the proportion of tectum covered by embryonic arbors is about 7 times larger than in the adult, suggesting that a higher precision of the adult projection is achieved as a result of a greater enlargement of the tectum than of the arbors.

Animals↗
PubMed1988

Retinopathy of prematurity: what causes it?

Retinopathy of prematurity continues to be a major cause of morbidity in surviving prematures despite attempts to regulate oxygen. Developing retinal vessels apparently can be disrupted by any of a considerable number of influences. By far the preeminent risk factor is extreme prematurity itself.

Humans↗
PubMed1985

Quinolinate. A selective neurotoxin in embryonic and posthatching chicken retinas.

Quinolinate (QUIN), an endogenous dicarboxylic amino acid, structurally related to the putative retinal neurotransmitter aspartate, acts as a specific neurotoxin in the chick neural retina. Qualitative analysis of QUIN's neurotoxic effects reveals that sensitivity to the amino acid is first detected in the 9-day-old embryonic chick retina. Nuclei and cytoplasm of some cells in the inner region of the inner nuclear layer and in the ganglion cell layer appear hypochromatic or electron lucent when examined by light or electron microscopy, respectively. Between day 10 and 12, the sensitivity of the embryonic retina to QUIN increases and remains around the day 12 level throughout the remaining embryonic and initial posthatching period. Cells in the inner half of the inner nuclear layer continue to be the most severely affected throughout retinal development, ganglion cells less so. Photoreceptor and most cells in the outer region of the inner nuclear layer remain undamaged. QUIN effects are partially reversible: retinas exposed to QUIN briefly in vitro and then transferred to fresh QUIN-free medium are not as severely affected as those allowed no recovery time. In day 1 posthatching chick retinas, similar patterns of QUIN-toxicity were observed in vitro (0.5-5 mM QUIN; 5-30 min) and in vivo (200-600 micrograms QUIN/eye; 0.5-24 hr following intravitreal injection).

Animals↗
PubMed1988

Vitamin E deficiency in goldfish fed a shellfish derived diet.

Six month old, laboratory-raised goldfish (Carassius auratus) used in morphologic studies of retinal development developed progressive fraying and deterioration of the tail and fins that reached epizootic proportions and culminated in fungal infection and death. Diagnostic histopathology showed marked skeletal muscle degeneration in the muscles of the tail and caudal body wall compatible with a vitamin E/selenium deficiency. Ration analysis demonstrated inadequate vitamin E in the commercial shrimp-based diets fed to the goldfish fry. Replacement of shrimp-derived diets by a commercial trout diet containing adequate levels of vitamin E was curative.

Animal Feed↗
PubMed1985

Malignant melanoma of the iris: recurrence after 19 years.

Late recurrences of malignant melanoma of the iris are rarely reported. The case of a patient who had apparently had a spindle and nevoid cell melanoma totally excised from his iris is presented. Two and a half years later he sustained blunt trauma and developed retinal detachment in the same eye. The eye became phthisic after two unsuccessful attempts to repair the detachment. Seventeen years later he was seen with discomfort in the eye and was found to have a ruptured globe with prolapse of dark jelly-like material. Pathologic study revealed this to be recurrent spindle-B melanoma. This case, along with two similar cases from the literature, emphasizes that iris melanomas can recur many years after the original excision and that long-term follow-up is indicated.

Humans↗
PubMed1985

Cerebral and ocular toxicity induced by desferrioxamine.

Seven patients with rheumatoid disease were given the iron-chelating drug desferrioxamine (DFX) to evaluate its possible anti-inflammatory effects. Two of these patients, who also received the anti-emetic prochlorperazine, lost consciousness for 48-72 h and then fully recovered. Electroencephalography showed abnormalities of the type associated with metabolic disturbance. One of these patients showed pyramidal features and subsequently developed an optic neuropathy and pigmentary retinopathy. Analysis of his cerebrospinal fluid showed a decrease in loosely-bound (catalytic) iron and increase in loosely-bound (catalytic) copper, total iron and products of lipid peroxidation, with values approaching normal as the symptoms resolved. Subsequent in vivo/vitro studies clearly demonstrated that the neurological effects were due to a synergistic action of desferrioxamine and prochlorperazine, probably resulting in exceptional fluxes of intra/extra cellular iron/copper disturbing noradrenergic and serotonergic systems. Two other patients who did not receive prochlorperazine, developed retinal problems which later improved, one after only 15 g of desferrioxamine. Our observations suggest a new model for metabolic encephalopathy studies and provide insight into the mechanisms of pigmentary retinopathy.

Adult↗
PubMed1983

Ocular functions and incidence of acute mountain sickness in women at altitude.

The effects of altitude on a series of ocular functions were studied on seven expedition members, all women aged 23-53 years, during the first ascent of the 6798-m peak Brigupanth in the Indian Himalayas. The only consistent change was a decrease in convergence amplitude. The amplitude of accommodation remained stable among the younger climbers, but decreased markedly among the older ones as higher altitudes were reached. There also appeared to be a lessened vascular reactivity to the hypoxia of altitude in the older members. Stereoscopic vision was unimpaired at all altitudes tested and extra-ocular muscle balance remained unaffected in all but two members who had an increase in their baseline phorias. Two of the summit climbers developed retinal hemorrhages. There was an average weight loss of 5.4 kg during the climb, but general health was good. Symptoms of acute mountain sickness were noted infrequently, and there were only moderate changes in the menstrual cycle.

Adult↗
PubMed1982

Monoclonal antibodies selective for the inner portion of the chick retina.

Five monoclonal antibodies were selected by immunizing mice with embryonic chick optic nerve and screening the resultant hybridoma antibodies by immunofluorescence on sections of the retina. The optic nerve was chosen as the immunizing tissue since it is presumably enriched in ganglion cell-specific antigens. These antibodies bind to antigens which are largely localized to the inner part of the retina. Two of the antibodies, RET1 and RET2, show highly selective binding to the optic fiber layer. Another, RET3, stains the optic fiber layer, ganglion cell layer, and inner plexiform layer. Antibody RET4 stains the optic fiber layer, ganglion cell layer, inner plexiform layer, and the inner one-half of the inner nuclear layer; this antibody binds to an antigen on or associated with the plasma membrane. Antibody RET5 stains the retinal ganglion cell layer faintly and the inner and outer plexiform layers strongly. The potential utility of these antibodies for future studies on retinal development is discussed.

Animals↗
PubMed1995

Evidence of active cytomegalovirus infection in clinically stable HIV-infected individuals with CD4+ lymphocyte counts below 100/microliters of blood: features and relation to risk of subsequent CMV retinitis.

To determine the frequency and significance of cytomegalovirus (CMV) viremia and viruria in HIV-positive subjects with low CD4+ lymphocyte counts but with no clinical indications for culture, we studied 100 consecutive clinically stable subjects with CD4+ cells < or = 100/microliters of blood who agreed to culture of blood and urine. Serum was tested for CMV antibody, p24 antigen, neopterin, and liver enzyme concentrations, and patients were offered funduscopic examination. Subjects' records were reviewed an average of 9.1 months after enrollment for evidence of subsequent CMV retinitis. Three of the original cohort proved ineligible because of CD4+ count > 100/microliters; CMV antibody was present in 96% of the remainder. Isolation of CMV from blood was uncommon (2 of 93 seropositive subjects) whereas viruria occurred in 51.6%; likelihood of having a positive urine culture was significantly related to the subject's absolute CD4+ lymphocyte count: 60% for those with CD4+ < or = 50/microliters, vs. 26.1% for those with CD4+ 51-100/microliters. Neither serum p24 antigen nor neopterin was predictive of CMV in urine or blood. No subjects submitting to ophthalmologic exam had unsuspected CMV retinitis. Subsequent development of retinitis correlated with CMV viruria on entry: 13.5% if urine-positive, 1.9% if negative (p = 0.029; Fisher exact test).

Adult↗