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Biomedical subjects

R E Carr

Publications and source records attributed to R E Carr.

At least 55 records · Page 3Linked to original sources

Retinal degeneration with nanophthalmos, cystic macular degeneration, and angle closure glaucoma. A new recessive syndrome.

Seven related patients had a progressive pigmentary retinal degeneration, characterized by nyctalopia, visual field restriction, and cystic macular degeneration in younger patients and a macula of nonspecific atrophic appearance in older patients. In addition, each patient had high hyperopia (+9.50 to +16.00) and nanophthalmos (axial lengths, less than 20 mm), with diffuse choroidal thickening on ultrasound. Younger patients had slitlike anterior chamber angles; older patients developed progressive synechial angle closure and eventual glaucoma. Chromosomes were normal. On electroretinographic testing, younger patients had absent rod signals, with normal cone wave form and near-normal b-wave amplitudes but markedly delayed cone b-wave implicit times; older patients had severely diminished or extinguished electroretinograms. This family appears to represent a newly recognized autosomal-recessive syndrome.

Adult↗

Cone function in congenital nyctalopia.

A patient with congenital stationary night blindness (CSNB) (Schubert-Bornschein type) transmitted as an autosomal recessive trait was studied with several tests of electrical function as well as a variety of psychophysical procedures. Comparison of the patient's present findings with those obtained 23 years earlier showed that while rod thresholds have remained the same, cone sensitivity has decreased. Subjective flicker thresholds obtained following a bleach were unchanged during the course of dark adaptation. The absence of rod-cone interaction, together with an absent scotopic b-wave, implies that the defect is in the mid-retinal layers. Further, the absence of oscillatory potentials in the photopic electroretinogram (ERG) suggests that the interplexiform cell may be implicated in some manner. The focal ERG of the CSNB patient showed normal amplitude and normal phase delays, supporting the idea that the focal ERG samples primarily cone photoreceptor activity.

Adult↗

Progressive cone dystrophy.

Psychophysical, reflectometric, and electrophysiologic studies were done on four members of a dominant pedigree with progressive cone dystrophy. The two youngest individuals were asymptomatic at the initial examination, and none of the subjects complained of problems associated with night vision. Nevertheless, absent or grossly reduced cone-mediated electroretinographic (ERG) responses showed the widespread loss of cone function, and moderate elevations (less than 1 log unit) in absolute threshold together with reductions in rhodopsin levels in the mid-peripheral retina provided evidence of impairment of the rod system. The progressive nature of the disease was apparent from the case histories and the changes in visual performance that occurred on re-test after a 5-year interval. Moreover, the results of increment threshold measurements at several retinal loci suggested that peripheral cones may be affected earlier and more severely than those in the central retina.

Adolescent↗

Progressive cone dystrophy.

Psychophysical, reflectometric, and electrophysiological studies were performed on four members of a dominant pedigree with progressive cone dystrophy. The two youngest individuals were asymptomatic at the initial examination, and none of the subjects complained of problems associated with night vision. Absent or grossly reduced cone-mediated ERG responses revealed the widespread loss of cone function. Moderate elevations (1 log unit) in absolute threshold together with reductions in rhodopsin levels in the midperipheral retina provided evidence of a mild impairment of the rod system also, although not to the degree seen in a cone-rod dystrophy. The progressive nature of the disease was apparent from the case histories and the changes in visual performance that occurred on re-test after a 5-year interval. Likewise, the results of incremental threshold measurements at several retinal loci suggested that peripheral cones may be affected earlier and more severely than those in the central retina.

Adolescent↗

Testicular swelling associated with desipramine.

Two cases of testicular swelling associated with desipramine therapy are described. In one case, the swelling resolved with discontinuation of desipramine; in the other, the swelling subsided despite continuation and an increase in dose. These cases may represent a local variant of angioneurotic edema; however, further study is required to determine the exact etiologic mechanism.

Adult↗

Objective assessment of temporal modulation transfer functions using the focal ERG.

Temporal modulation transfer functions (MTF's) were recorded from the macula of nine normal subjects using focal electroretinography (FERG). An array of light emitting diodes (LED's) was used to experimentally manipulate stimulus temporal frequency, modulation depth, and mean luminance values. Two techniques were used to derive FERG modulation thresholds at several temporal frequencies: conventional averaging with extrapolation to a criterion amplitude, and a swept stimulus lock-in retrieval method. These two methods produced comparable results. The electrophysiologically derived MTF's were similar in shape to those obtained psychophysically. Six patients with retinal disease were also examined; all patients showed sensitivity losses which were most marked at the higher frequencies. Such losses tended to be greater in patients with poorer visual acuity.

Adult↗

Evaluating macular function using the focal ERG.

A stimulus consisting of 96 red LEDs mounted in the rear of a ganzfeld bowl was used to elicit focal electroretinograms (FERG) from the central 9 degrees of the retina in human subjects. The luminance of the stimulus was driven sinusoidally at frequencies from 10-60 Hz. The temporal responsiveness and response phase lags of normal subjects and patients with retinal disease were measured. Normal subjects produced maximum amplitude FERG responses to stimuli between 30-40 Hz. Patients with retinitis pigmentosa showed a low-pass pattern of amplitude loss, with an additional frequency independent loss in sensitivity in those with poorer visual acuity. Patients with macular degeneration showed general amplitude loss associated with a relative sparing of the mid-temporal frequencies. The response phase lags in both patient groups were not significantly different from the normals. These findings point to a loss in temporal responsiveness accompanied by a secondary loss of sensitivity in these heredoretinal degenerations.

Adolescent↗

Spatiotemporal conditions which elicit or abolish the oblique effect in man: direct measurement with swept evoked potential.

Reversing sine wave gratings were electronically swept in spatial frequency and contrast. The acuity limits and contrast thresholds of 4 observers were inferred from evoked potential stimulus-response functions elicited by these stimuli and retrieved with a quadrature lock-in amplifier. The evoked potential functions, linearized in the case of contrast by increasing contrast logarithmically with time, were extrapolated to the point of zero response. This point provides an electrophysiologically defined threshold value for acuity and for contrast. An oblique effect (superior sensitivity for HV-oriented gratings) could reliably be demonstrated in both acuity and contrast threshold performance. This oblique effect could readily be abolished under low spatial/high temporal frequency conditions. The findings are discussed in terms of shifting relative strengths of X and Y contributions to the steady-state evoked potential.

Adult↗

A rapid evoked potential index of cortical adaptation.

Contrast thresholds and acuity limits were measured in 4 observers with the swept visual evoked potential (VEP) technique. In this technique, grating contrast or grating spatial frequency is electronically varied while the subject's evoked response is retrieved in real time (without averaging). Contrast or spatial frequency variation make the stimulus vary in intensity; zero VEP response amplitude indicates the threshold intensity. Large shifts occur in the indicated threshold when stimulus sweep direction is reversed. Thresholds are always relatively elevated when the run begins with the strongest stimulus value. These shifts do not have a technical origin in the delay of the instrument (Nelson et al. 1984b). Here, it is shown that the shifts are due to orientation and spatial frequency selective adaptation, probably of cortical origin. Measureable adaptation is produced by momentary exposure to contrasts as low as 1.25%; nearly maximum adaptation (0.6 log units) is reached with 20% contrast. These findings support the concept of a contrast gain control mechanism in visual cortex, and pose practical problems for visual assessment with the evoked potential.

Adaptation, Physiological↗

Functional abnormalities in vincristine-induced night blindness.

Various noninvasive test procedures were used to evaluate retinal function in a patient who had become night blind following vincristine chemotherapy. The results obtained were strikingly similar to those reported previously in subjects with recessively inherited stationary night blindness; the dark-adaptation curve was monophasic (ie, no evidence of a scotopic branch), rhodopsin kinetics were entirely normal, and spectral threshold data revealed the presence of residual rod-mediated vision. Also like the heritable condition, the b-wave of the ERG was depressed grossly despite normal a-wave potentials. These findings, and the fact that vincristine is known to disrupt the structural integrity of neuronal microtubules, suggest that the drug-induced defect involves the process of synaptic transmission between the photoreceptors and their second-order neurons.

Adaptation, Ocular↗

Lock-in techniques for the swept stimulus evoked potential.

We discuss the use of synchronous-demodulation (lock-in) techniques for evoked potential retrieval. Application to electronically swept visual displays is emphasized. These techniques permit a visual threshold to be specified in 20 s, but their application to visual assessment requires careful consideration of several problems, notably alleged delay in the instrumentation, specification of the baseline response level, and the nature of EEG interference. In addition, since stimulus waveform information is lost in all lock-in methods, questions concerning what activity is contributing to the measured response must be answered. A technique addressing these issues and combining phase-sensitive detection and vector computation is presented.

Computers↗

The assessment of evoked potential contrast thresholds using real-time retrieval.

Electrophysiologic contrast sensitivity functions (CSF) have been estimated using lock-in amplifier signal retrieval of the visually evoked response (VER). These CSFs were compared with CSFs obtained psychophysically using the same stimulus conditions. The two measures of contrast sensitivity behave similarly in response to variations of temporal and spatial frequency. The major advantage of using real-time retrieval is speed. Threshold for a single spatiotemporal condition can be estimated in as little as 20 sec, making the application of electrophysiologic contrast sensitivity testing feasible for clinical populations.

Adult↗

Contrast sensitivity loss in multiple sclerosis. Selectivity by eye, orientation, and spatial frequency measured with the evoked potential.

Multiple sclerosis can produce highly selective losses in visual function. Psychophysical studies have demonstrated contrast sensitivity deficits for spatial frequencies or for stimulus orientations. Using real-time lock-in retrieval of the visual evoked potential, the authors measured contrast sensitivity in 15 cases with probable or definite multiple sclerosis and acuities of 20/40 or better. Sine-wave grating contrast threshold determinations for three spatial frequencies (1, 4, and 8 cycles/deg) and four orientations (0, 45, 90, and 135 deg) revealed contrast deficits in at least one spatial frequency and orientation in every case. In most cases the visual losses were spotty or multifocal, and not the same in both eyes. Some cases with highly selective patterns of orientation or spatial frequency losses were observed and are discussed in terms of involvement of cortical functional architecture in the disease.

Adult↗