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Biomedical subjects

G B Arden

Publications and source records attributed to G B Arden.

At least 55 records · Page 3Linked to original sources
PubMed1994

Acute zonal occult outer retinopathy (AZOOR) associated with multifocal choroidopathy.

Acute zonal occult outer retinopathy (AZOOR) may be precipitated by various retinal disorders and is characterised by rapid loss of visual field which cannot be explained by the ophthalmoscopic changes consequent upon the initiating disease. The electroretinogram is abnormal, indicating that the field loss is due to retinal dysfunction. The phenomenon was first recognised in the multiple evanescent white dot syndrome (MEWDS) as the enlarged blind spot syndrome. It was subsequently described with multifocal inner choroidopathy and acute macular neuropathy (AMN). We have identified 7 patients who presented with widespread visual loss associated with multifocal inner choroidopathy in whom functional loss was documented with electroretinography and automated visual field testing. All patients were young, myopic, and otherwise healthy women. Initial photopsia was noted by 4 patients. Fundus findings included scattered small partially pigmented yellowish lesions resembling those in multifocal inner choroidopathy or pseudo presumed ocular histoplasmosis syndrome, disc swelling, vitritis, and secondary choroidal neovascularisation. Two patients had bilateral involvement. All patients had an enlargement of the blind spot, and widespread visual field loss which was not explained by fundus changes. All had an abnormal electroretinogram suggesting widespread retinal disease. In AZOOR retinal dysfunction occurs without corresponding visible retinal lesions. This disorder appears to be precipitated by several conditions, although the causal relationship between the initiating event and the widespread functional loss is unknown.

Acute Disease↗
PubMed1994

Ocular findings associated with a 3 base pair deletion in the peripherin-RDS gene in autosomal dominant retinitis pigmentosa.

Affected members of a family with autosomal dominant retinitis pigmentosa were found to have a 3 base pair deletion at codon 118 or 119 of the retinal degeneration slow gene. This mutation causes the loss of a highly conserved cysteine residue in the predicted third transmembrane domain of peripherin-rds, a photo-receptor specific structural glycoprotein localised to both rod and cone outer segment disc membranes. Four of these individuals underwent detailed clinical, psychophysical, and electroretinographic testing in order to characterise their photoreceptor dysfunction. Nyctalopia was reported early in the second decade by all patients. Global rod and cone dysfunction was recorded by the third decade with severe reduction of both photopic and scotopic function by age 30 years. This retinal degeneration slow gene mutation may lead to the primary loss of both rod and cone photo-receptor function.

Adult↗
PubMed1994

Ocular manifestations in autosomal dominant retinitis pigmentosa with a Lys-296-Glu rhodopsin mutation at the retinal binding site.

A lysine to glutamic acid substitution at codon 296 in the rhodopsin gene has been reported in a family with autosomal dominant retinitis pigmentosa. This mutation is of particular functional interest as this lysine molecule is the binding site of 11-cis-retinal. The clinical features of a family with this mutation have not been reported previously. We examined 14 patients with autosomal dominant retinitis pigmentosa and a lysine-296-glutamic acid rhodopsin mutation. Four had detailed psychophysical and electrophysiological testing. Most affected subjects had severe disease with poor night vision from early life, and marked reduction of visual acuity and visual field by their early forties. Psychophysical testing showed no demonstrable rod function and severely reduced cone function in all patients tested.

Adolescent↗
PubMed1993

Dominant retinitis pigmentosa associated with two rhodopsin gene mutations. Leu-40-Arg and an insertion disrupting the 5'-splice junction of exon 5.

OBJECTIVE: To determine the phenotypes of two families in which retinitis pigmentosa cosegregates with a rhodopsin (RHO) gene mutation: a leucine-to-arginine change at codon 40 (Leu-40-Arg) in one family, and a 150-base pair insertion that disrupts the RHO 5'-splice junction of exon 5 in another. PATIENTS: Three affected members of each family. RESULTS: The Leu-40-Arg mutation was associated with the onset of night blindness in the first decade of life. By the fourth decade, severe retinal functional loss was evident on dark-adapted static threshold perimetry, and electroretinographic responses were absent or barely detectable. In contrast, the RHO 150-base pair insertion was associated with the later onset of mild night vision difficulties; in two individuals, mild night vision difficulties were first noticed in the second decade while a third, a 25-year-old woman, was asymptomatic. Dark-adapted static threshold perimetry of this latter individual revealed a "regional" or class 2 pattern of retinal functional loss associated with equal loss of rod and cone electroretinographic responses. CONCLUSION: The RHO Leu-40-Arg mutation causes symptomatic retinal dysfunction by the end of the first decade while the insertion disrupting the 5'-splice junction of RHO exon 5 causes later onset "regional" or class 2 retinal dysfunction.

Adult↗
PubMed1993

Survival of cone responses in postmortem human retina.

A cone-mediated electroretinogram was recordable from human retinas 17-18 hours postmortem, after regeneration of visual pigments by application of 11-cis and 9-cis retinal. The cone was separated from the rod component by stimulation with flickering red light. In addition to these neuronal responses, a P2 and a slow P3 were present.

Adolescent↗
PubMed1993

Autosomal dominant retinitis pigmentosa with apparent incomplete penetrance: a clinical, electrophysiological, psychophysical, and molecular genetic study.

Twenty five symptomatic individuals and six asymptomatic obligate gene carriers from four families with autosomal dominant retinitis pigmentosa (adRP) showing apparent incomplete penetrance have been studied. Symptomatic individuals from three families showed early onset of night blindness, non-recordable rod electroretinograms, and marked elevation of both rod and cone thresholds in all subjects tested. In the fourth family, there was more variation in the age of onset of night blindness and some symptomatic individuals showed well preserved rod and cone function in some retinal areas. All asymptomatic individuals tested had evidence of mild abnormalities of rod and cone function, indicating that these families show marked variation in expressivity rather than true non-penetrance of the adRP gene. No mutations of the rhodopsin or RDS genes were found in these families and the precise genetic mutation(s) remain to be identified.

Adolescent↗
PubMed1993

Comparison of new psychophysics and perimetry with electrophysiological techniques in the diagnosis of glaucoma.

Many accounts show that the pattern electroretinogram is a sensitive index of glaucomatous visual damage, and can demonstrate losses before field defects are apparent. If the pattern electroretinogram is not specific, and although it is likely to have a role in patient management, it is not a screening technique. Many psychophysical tests, particularly of titan color vision, appear to be promising in this regard.

Color Perception↗
PubMed1993

[Color vision defect. A early sign of Sorsby retinal dystrophy?].

In 34 asymptomatic patients from three families at 50% risk of developing Sorsby's fundus dystrophy, colour contrast sensitivity was measured. In 16 the thresholds--mainly of the tritan axis--were raised above the normal values. It is concluded that testing of colour vision is useful in detecting the abnormal genotype. Raised colour contrast sensitivity was not observed before the first fundus or fluorescein angiographic changes in two of the families, while the colour defect occurred in the absence of ophthalmoscopic abnormality in the third family. Therefore, our results support the clinical suggestion that Sorsby's fundus dystrophy is more than one disease.

Adolescent↗
PubMed1993

Evidence from human electroretinogram A and off responses that color processing occurs in the cones.

PURPOSE: To investigate two apparent anomalies of the human electroretinogram: the "on" and "off" components of the cone based PIII are unequally sized, and transitions from red to green, which are electroretinographically silent, yield reverse transitions (green to red) in which a-waves develop. METHODS: Ganzfeld electroretinograms were obtained with intense 100 msec flickering flashes from red and green light-emitting diode. Such stimuli light-adapt the retina, and the responses are caused by the excitation of long and medium wavelength cones. RESULTS: In the 10-20 msec after the beginning of a flash (black to green or black to red) the beginning of rapid receptor-generated a-wave is seen. Ten to twenty milliseconds after the end of the flash, the beginning of a rapid positive-going off response, also derived from receptors can be seen. If the retina is stimulated by the abrupt change from one wavelength of light to another (eg, from "green" to "red"), at times > 20 msec after the change there are always slow changes in potential (presumably caused by postsynaptic activity) regardless of the relative intensities of red and green. However, if the two light intensities are adjusted appropriately, 10-20 msec after the transition from green to red no electroretinographic a-wave (or off response) develops--the transition is "silent." When the transition reverses (changes back from red to green), an a-wave occurs. In the same way if a red-to-green transition is made silent by altering the relative light intensities, the green-to-red reversal evokes an a-wave. This occurs for numerous pairs of red and green intensities. Rod intrusion or minor electroretinogram components do not explain this result. The relative red:green intensity in two color-anomalous subjects is different to that in three normal subjects. The rule for a silent transition is that the decrease in excitation in one cone type should be twice the increase in excitation in the second cone type. CONCLUSIONS: The most likely cause is a reduction in the amplitude of cone receptor potentials 20-50 msec after the onset of the stimulus, caused by a sign-reversing feedback mechanism such as that described in amphibians. This implies that the chromatic signals for color vision required by theorists are partly generated in the cones.

Color Perception↗
PubMed1992

The pattern electroretinogram in glaucoma and ocular hypertension.

Thirty one eyes with established glaucoma, 61 high risk ocular hypertensive (OHTs) eyes, 66 medium risk OHT eyes, 58 low risk OHT eyes, and 47 control eyes have been followed for up to 2 years by clinical examination and pattern electroretinography (PERG). The study was 'masked' so electrophysiological and clinical data were kept separate. Criteria have been devised which enable PERG measurements to distinguish all established glaucomatous eyes from all normal controls; these criteria demonstrate abnormalities in some OHT eyes, particularly those at high risk. The PERG abnormality is greatest in eyes with established glaucoma in which the intraocular pressure has been lowered by treatment. The PERG becomes smaller as the degree of clinical abnormality increases. Test-retest variability of the PERG is sufficiently low to ensure that most of those first described as abnormal continue to be so.

Adult↗
PubMed1992

Abnormal dark adaptation kinetics in autosomal dominant sector retinitis pigmentosa due to rod opsin mutation.

The time course of dark adaptation was measured in 10 subjects from three families with autosomal dominant sector retinitis pigmentosa (RP) due to mutations in the first exon of the rod opsin gene. In each subject cone adaptation and the early part of the recovery of rod sensitivity followed the normal time course, but the later phase of rod adaptation was markedly prolonged. The recovery of rod sensitivity is much slower than that reported in any other outer retinal dystrophy. Using a model based upon primate data of rod outer segment length and turnover, we have calculated that the delayed phase of the recovery of rod sensitivity in the RP patients tested following strong light adaptation could be due in part to formation of new disc membrane with its normal concentration of rhodopsin rather than in situ regeneration of photopigment.

Adolescent↗
PubMed1992

Electrode comparison in pattern electroretinography.

In recent years, there has been great interest in recording the pattern electroretinogram (PERG) in glaucomatous and diabetic populations. The Dawson, Trick, and Litzkow thread electrode (DTLTE) and the gold foil electrode (GFE), commonly used for recording PERGs, were compared for variations in amplitude of response, test-retest variability, and patient comfort. Two study centers collected data on a total of 32 normal subjects. The subjects from the London center showed a slight (but not significant) preference for the DTLTE, and the Houston subjects also found the DTLTE to be significantly more comfortable (chi-square = 39, P less than 0.001). In both study groups, the GFE was found to produce a statistically larger amplitude of response than that obtained with the DTLTE. Significant differences were found regardless of the slow (transient, 3.1 Hz; F = 6.24; P = 0.0192) or fast (steady state, 8.3 Hz; F = 18.38; P = 0.0001) stimulus-presentation rate. Larger differences between the two electrodes occurred under steady-state conditions. Although there is no consensus as to the optimum recording conditions to obtain the subtle PERG, it appears the the GFE records larger responses than the DTLTE. However, test-retest data confirmed that the GFE records twice the amplitude of the DTLTE, and it also produced twice the variability (average percent difference over time for GFE, 15%; for DTLTE, 8%).

Adolescent↗
PubMed1991

Nyctalopia with normal rod function: a suppression of cones by rods.

Twenty-nine patients with exaggerated rod-cone interaction are described. All were referred because they appeared to suffer from night blindness. ERG and EOGs were performed but were normal. However, investigation with a modified dark-adaptometry technique showed that in these patients cone flicker thresholds rise considerably more during dark adaptation than is normal, and this is sufficient to explain the symptoms. In one case, the condition appears familial. Many patients report their symptoms begin in early adult life and slowly get worse, but we have no objective evidence of progression.

Adult↗
PubMed1991

Colour contrast sensitivity changes caused by peripheral retinal laser photocoagulation.

Macular phototoxicity is known to occur with laser use, and there is evidence that the wavelength of the light used influences this effect. In this study, a computer based colour contrast sensitivity test was used to assess the immediate macular effects of photocoagulation of peripheral flat retinal holes in otherwise normal retinas, using blue-green (488 and 514 nm), yellow (577 nm), orange (595 nm) or red (647 nm) laser light. The laser aiming beam was not allowed to traverse the macula at any stage during treatment. No protan or deutan axis threshold changes were noted in the 17 patients tested irrespective of the laser wavelength used. Tritan axis sensitivity was significantly reduced one hour after treatment with the blue-green laser, but no tritan axis change was found after treatment with longer wavelength lasers. The effect was no longer present the day after treatment in the subjects tested. The results show that even peripheral retinal treatment with blue-green laser can cause acute macular phototoxicity.

Color Perception↗
PubMed1991

Macular colour contrast sensitivity in ocular hypertension and glaucoma: evidence for two types of defect.

Colour contrast sensitivity (CCS) of a large cohort of glaucomatous patients, ocular hypertensive patients (OH), and normal persons was measured at six-month intervals during a two-year period. The OHs were graded into high, medium, and low risk groups. 69% of glaucomatous patients and 32% of all OHs had CCS thresholds greater than the mean plus 2 SDs of the controls. Satisfactory specificity and sensitivity could not be obtained by adjusting the criterion of threshold. In abnormal eyes, progressive small increases of threshold occurred during the study, but glaucomatous eyes with normal thresholds on the first visit retained normal thresholds in the subsequent visits. Although our system is very sensitive and precise, the proportion of abnormalities detected is no greater than with other techniques. In some glaucomatous patients there is a true preservation of colour vision which does not merely reflect the limitations of the test employed.

Color Perception↗
PubMed1991

Peripheral color contrast. A new screening test for preglaucomatous visual loss.

A new test of peripheral color contrast is described. A high-definition color monitor driven by a personal computer with a graphics interface card displays an annulus subtending 25 degrees at the eye. The color contrast between the annulus and the background can be varied. Forty-five degrees of the annulus is randomly removed in one of four quadrants. Patients are asked to identify the position of the gap while fixating a central spot. The minimum color contrast between annulus and background at which the identification is possible is between 13-16% for the protan, deuteran, and tritan axis in normal subjects. This threshold value changes little with age, refractive error, or pupillary aperture, and test-retest variability is low. Testing one eye takes only 1-2 min. The test was applied to ocular-hypertensive and glaucomatous patients. All patients with glaucoma had thresholds greater than two standard deviations (SD) above the normal mean. In addition, 97% of glaucoma patients had thresholds greater than four SDs, and 95% had thresholds more than five SDs above the normal mean. Most patients with ocular hypertension and clinical signs indicating a low or medium risk of conversion to glaucoma had thresholds under the upper limit of normal. High-risk patients with ocular hypertension fell into two groups. One approximated to normal; the other had elevated thresholds, which in many cases were more than four SDs above the normal mean. The epidemiologic consequences of this test are discussed.

Adult↗
PubMed1991

Temporal and spatial properties of suppressive rod-cone interaction.

Recordings were obtained from rods and horizontal cells of Xenopus, using an eyecup preparation. The enhancement of cone signals produced by rod backgrounds was measured using flickering red spots of varying intensity and diameter, and the experiments were repeated with cone stimuli consisting of alteration of wavelengths of 660 and 605 nm, adjusted for equal effects on rods or cones ("silent substitution"). Rods responded to red flicker with discrete wavelets up to 5 Hz. The characteristics of suppressive rod-cone interaction (SRCI) depend on the precise stimulus parameters. In particular, the reported low-pass attenuation of SRCI is absent with silent substitution. An analysis of the responses to backgrounds in horizontal cells, and the effects of the red light flashes in rods, led to the conclusion that the characteristics of SRCI are determined partially by the fact that "cone" stimuli excite rods and vice versa. This result simplifies the mechanism of SRCI and permits a comparison between the studies of SRCI using electroretinograms and horizontal cells.

Animals↗