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Comparison of oculomotor findings in the progressive ataxia syndromes.

In this study we compare the results of quantitative oculomotor function testing in patients with Friedreich's ataxia (FA), olivopontocerebellar atrophy (OPCA) and cerebello-olivary atrophy (CA). Common features in all three syndromes included gaze-evoked nystagmus, saccade dysmetria and prolonged saccade reaction times. Patients with FA showed a characteristic combination of frequent saccadic intrusions, especially ocular flutter, relatively preserved optokinetic nystagmus (OKN) and smooth pursuit, and impaired vestibulo-ocular reflex (VOR) responses. In patients with CA saccadic intrusions were infrequent, OKN and smooth pursuit were severely impaired and VOR gain was normal or increased. Results in OPCA were more variable. When present, slowing of saccades or the combined loss of pursuit and vestibular function were characteristic for OPCA. The ability to suppress the VOR with a head fixed target was relatively preserved in FA, normal to moderately impaired in OPCA and always severely impaired in CA. We conclude that oculomotor testing is useful in the differential diagnosis of the progressive ataxia syndromes.

Adult↗

Control of eye orientation: where does the brain's role end and the muscle's begin?

Our understanding of how the brain controls eye movements has benefited enormously from the comparison of neuronal activity with eye movements and the quantification of these relationships with mathematical models. Although these early studies focused on horizontal and vertical eye movements, recent behavioural and modelling studies have illustrated the importance, but also the complexity, of extending previous conclusions to the problems of controlling eye and head orientation in three dimensions (3-D). An important facet in understanding 3-D eye orientation and movement has been the discovery of mobile, soft-tissue sheaths or 'pulleys' in the orbit which might influence the pulling direction of extraocular muscles. Appropriately placed pulleys could generate the eye-position-dependent tilt of the ocular rotation axes which are characteristic for eye movements which follow Listing's law. Based on such pulley models of the oculomotor plant it has recently been proposed that a simple two-dimensional (2-D) neural controller would be sufficient to generate correct 3-D eye orientation and movement. In contrast to this apparent simplification in oculomotor control, multiple behavioural observations suggest that the visuo-motor transformations, as well as the premotor circuitry for saccades, pursuit eye movements and the vestibulo-ocular reflexes, must include a neural controller which operates in 3-D, even when considering an eye plant with pulleys. This review summarizes the most recent work and ideas on this controversy. In addition, by proposing directly testable hypotheses, we point out that, in analogy to the previously successful steps towards elucidating the neural control of horizontal eye movements, we need a quantitative characterization first of motoneuron and next of premotor neuron properties in 3-D before we can succeed in gaining further insight into the neural control of 3-D motor behaviours.

Animals↗

A mathematical model of the optokinetic reflex.

The role of the optokinetic reflex (OKR) is that of cooperating with the vestibulo-ocular reflex (VOR) in the task of image stabilization on the retina during head rotations in a stationary visual surround. Since the dynamics of VOR was already well established, it has been possible to make a broad estimation of what the dynamics of OKR should be in order to obtain the performances observed in normal subjects. A mathematical model of OKR has been presented, and the experimental results obtained by Raphan et al. (1977) in the monkey and by Collins et al. (1970) in man were used to validate the model and to obtain a precise estimation of its parameters.

Animals↗

Shortage of binocular cells in area 17 of visual cortex in cats with congenital strabismus.

Twenty-nine pigmented offspring of an innately esotropic female cat exhibited varying deficits in the number of binocular cells recorded in area 17 of the visual cortex as compared to 12 normal cats. Misalignment of the two eyes in these cats was found in the awake as well as in the paralysed state. Pupillography combined with measurements of visual disparity yielded abnormal esotropia of up to 8.4 degrees under paralysis, which corresponds to an abnormal convergence of the freely moving eyes of up to 14 degrees (average 7.4 degrees). In the majority of animals cortical binocularity was found reduced by the two eyes controlling independent sets of separate units (U-shaped ocular dominance distribution) whereas in 7 cats the reduction was due to a partial loss of one eye's influence. The proportion of monocular units correlated with the degree of crossover of the visual axes (r = 0.73). Anatomical investigation of the retinofugal projections revealed normal appearance in three previously recorded cats in which more than 50% of cortical units had been monocularly driven. The small angles of esotropia and the "normal" appearance of eye position judged by the pupillary positions in the orbit of these cats, might suggest that we found an animal model for microstrabismus.

Anesthesia, General↗

Lens-sparing external beam radiotherapy of intraocular metastases: our experiences with twenty four eyes.

Occurrence of uveal metastases is higher, than the number of clinically diagnosed cases, furthermore all cases are not amenable to therapy. Treatment of primary cancer is permanently improving, as a result life prospective is better, with an increasing number of late distant metastases in an unusual location, as e.g. intraocular metastasis. As surgical approach is not suitable, and chemo/hormonal therapy often has a limited effect on intraocular dissemination, other treatment modalities are needed for the maintenance of visual acuity, and prevention of further deterioration of the quality of life. The study was made to evaluate the efficacy of external beam radiotherapy (EBRT) with lens-sparing techniques in the management of patients developing intraocular metastases (IOM). Between March 1994 and March 2002, 24 eyes of 17 patients with tumors metastatic to the eye were treated by EBRT. The female:male ratio was 8.5:1, age ranged between 37 and 74 years (mean: 56 years). The site of the primary tumor was: breast (11), lung (4) and others (2). The visual acuity at the beginning of irradiation was between 0.1-0.7 (mean 0.5) and a mean KPS of 60% was detected. The mean time elapsed from the diagnosis of primary tumor and recognition of metastasis was 38.9 months in case of breast, and 6.7 months in lung cancer. Eyes were treated by 6 MV photon beams, using a modified technique of Schipper's lens-sparing retinoblastoma treatment method. The following parameters were studied: visual acuity changes, local response rate, survival times from irradiation and ocular complications. Mean follow-up time was 24 months. Mean visual acuity improved two lines on the Snellen chart. The mean survival time after treatment of IOM was 21 months in breast and 4.9 months in lung cancer patients. Local response rate was 78%. No radiation cataract was observed. Only one patient developed radiation retinopathy 32 months after the treatment. External beam radiotherapy is recommended for the treatment of intraocular metastases to improve quality of life. In selected cases--especially breast cancer patients--lens-sparing technique is the treatment of choice.

Adult↗

Ocular-motor function and information processing: implications for the reading process.

This paper discusses the dichotomy between continually moving eyes and the lack of blurred visual experience. A discontinuous model of visual perception is proposed, with the discontinuities being phase and temporally related to saccadic eye movements. It is further proposed that deviant duration and angular velocity characteristics of saccades in patients with hypertonic motor impairment relate to information processing defects. Stabilized retinal image procedures, which control for the effects of eye movements, significantly increase the ability of these patients actively to recall information presented for periods of less than three sec. A model of the reading process is presented based on these findings that addresses itself to the specific components of an interactions between eye movement, information transmission and information processing.

Alpha Rhythm↗

The contribution of midbrain circuits in the control of gaze.

The midbrain contains several structures important for the generation of torsional and vertical eye movements including the rostral interstitial nucleus of the MLF (riMLF) and the interstitial nucleus of Cajal (iC). While the riMLF is the immediate premotor structure for the generation of torsional and vertical saccades, the iC is considered a major part of the neural integrator for torsional and vertical eye movements. Experiments in monkeys show that a unilateral inactivation of the riMLF with muscimol leads to spontaneous contralesional torsional nystagmus, whereas an iC inactivation causes ipsilesional torsional nystagmus. In addition, inactivation of either structure leads to a tonic ocular torsion to the contralesional side. While the deficits after a riMLF lesion are thought to result from an imbalance of the saccade generator, a vestibular imbalance probably causes the deficits after an iC lesion. Contralesional and ipsilesional torsional nystagmus is also found in patients with unilateral mesencephalic lesions. A detailed analysis of the lesions from MRI scans shows a preferential involvement of the riMLF for patients with contralesional torsional nystagmus, and a major involvement of iC in cases with ipsilesional torsional nystagmus. Thus, the direction of torsional nystagmus appears to be a valuable topodiagnostic sign for patients with midbrain lesions.

Animals↗

Postural disturbance in patients with benign paroxysmal positional nystagmus.

Abnormal vestibular function disrupts postural and ocular muscle control system references to gravity (earth) vertical. Vestibular disorders also prevent satisfactory resolution of normally redundant, but often conflicting, visual and somatosensory spatial references required for normal postural control during active and passive body motion. Using a moving platform and visual surrounds posturography technique to systematically interact visual, somatosensory, and vestibular inputs, it was clearly demonstrated that patients with the benign paroxysmal positional nystagmus type of distorted vestibular function employ an unstable, visually dependent postural sway distinct from the postural instability associated with unilateral or bilateral vestibular functional deficits. These findings have important clinical implications for diagnosis and management of patients with vestibular disorders.

Adult↗

Congenital ocular motor apraxia and brainstem tumor.

Congenital ocular motor apraxia is considered a benign disorder of horizontal saccadic eye movements. A case is presented of an 8-month-old boy with this clinical picture in whom, four months later, signs of increased intracranial pressure from a large, cystic neoplasm of the rostral part of the brainstem developed, the second such case reported to our knowledge. Occasional abnormalities of the nervous system in patients with this disorder are reviewed. Careful neurologic examination and continuing reevaluation of the conditions of these patients are encouraged, with consideration of computerized axial tomography of the brain if neurologic findings or deterioration dictates.

Apraxias↗

Effect of refractive surgery on binocular vision and ocular alignment in patients with manifest or intermittent strabismus.

OBJECTIVE: To evaluate the effect of refractive surgery on binocular vision and ocular alignment in patients with manifest or intermittent strabismus, with or without vertical component. SETTING: University Hospital Antwerp, Edegem, Belgium. PATIENTS AND METHODS: 13 patients (22 eyes) with strabismus underwent refractive surgery. Five of these patients presented with an esotropia and four of them with a small vertical deviation. Five patients had a manifest exotropia, of whom two presented with a small vertical deviation. Two patients had an intermittent exotropia with binocular vision, of whom one patient had a vertical deviation. One patient had a hypertropia with a dissociated vertical deviation. RESULTS: Ocular alignment and binocular function remained unchanged postoperatively in all except two patients with high anisometropia who experienced an improvement in binocular function. In these patients, the preoperative manifest deviation became intermittent or latent after surgery, allowing fusion and stereopsis. Vertical deviation was found preoperatively in 8 of the 13 patients. This vertical deviation remained unchanged postoperatively, but improved in one patient with anisometropia. CONCLUSION: Preoperative intermittent or manifest strabismus is not a contraindication for refractive surgery provided some specific recommendations are taken into account, such as an adequate preoperative orthoptic examination and aiming at emmetropia for both eyes.

Adult↗

Comparison of predictable smooth ocular and combined eye-head tracking behaviour in patients with lesions affecting the brainstem and cerebellum.

We compared the ability of eight normal subjects and 15 patients with brainstem or cerebellar disease to follow a moving visual stimulus smoothly with either the eyes alone or with combined eye-head tracking. The visual stimulus was either a laser spot (horizontal and vertical planes) or a large rotating disc (torsional plane), which moved at one sinusoidal frequency for each subject. The visually enhanced vestibulo-ocular reflex (VOR) was also measured in each plane. In the horizontal and vertical planes, we found that if tracking gain (gaze velocity/target velocity) for smooth pursuit was close to 1, the gain of combined eye-hand tracking was similar. If the tracking gain during smooth pursuit was less than about 0.7, combined eye-head tracking was usually superior. Most patients, irrespective of diagnosis, showed combined eye-head tracking that was superior to smooth pursuit; only two patients showed the converse. In the torsional plane, in which optokinetic responses were weak, combined eye-head tracking was much superior, and this was the case in both subjects and patients. We found that a linear model, in which an internal ocular tracking signal cancelled the VOR, could account for our findings in most normal subjects in the horizontal and vertical planes, but not in the torsional plane. The model failed to account for tracking behaviour in most patients in any plane, and suggested that the brain may use additional mechanisms to reduce the internal gain of the VOR during combined eye-head tracking. Our results confirm that certain patients who show impairment of smooth-pursuit eye movements preserve their ability to smoothly track a moving target with combined eye-head tracking.

Adult↗

Smooth-pursuit initiation in the presence of a textured background in monkey.

Smooth pursuit eye movements in the monkey were studied by measuring the average eye acceleration in the first 100 msec of the ocular response to suddenly moving constant velocity discrete visual targets. The tracking target's motion was initiated on or eccentric to the fovea against a very dimly lit homogeneous background or an illuminated, highly textured, large-field background. The effect of the textured background was always to reduce the initial eye acceleration as compared to that present in the homogeneous background case. Movement of the background in the opposite direction to target spot movement also reduced the initial eye acceleration. In contrast, steady-state tracking measured several hundred msec after the onset of pursuit under the same conditions was only marginally reduced by the presence of the textured background.

Animals↗

Effects of vestibular and cerebellar deficits on gaze and torso stability during ambulation.

We measured gaze, head, and torso stability during ambulation to determine how vestibulo-ocular reflex dysfunction caused by unilateral vestibulopathy, bilateral vestibulopathy, and cerebellar dysfunction might affect image stabilization on the retina. Subjects were tested during standing, walking, and running on a treadmill. Gaze velocity, vestibulo-ocular reflex gain, and head velocities were calculated from angular positions of the eye and head, as well as linear positions of the head and trunk. Mean gaze velocity with a visible, distant target was below 4 degrees /second for all measurement conditions in control and vestibulopathic subjects. The performance of unilaterally vestibulopathic subjects was indistinguishable from that of control subjects except that the former had less vertical translation during walking. Bilaterally vestibulopathic subjects demonstrated less head translation than control subjects but had higher gaze velocity. In subjects with cerebellar dysfunction, gaze velocity was elevated by pathologic nystagmus, but head movements were similar to those of control subjects.

Adult↗

A method for simulation of foveal vision during wear of corrective lenses.

PURPOSE: The aim was to simulate the visual appearance of images viewed through corrective lenses having known, arbitrary types and amounts of monochromatic aberration, so that the visual effect of changing the design parameters of the lens could be explored. METHODS: We first calculate the optical response of the eye and any corrective lens using a numerical model eye. We then use this response as a filter, which we convolve with a selected original (unaberrated) image, to obtain an initial simulated retinal image. This image is then deconvolved by a second filter, which is calculated as the optical response of the eye of the observer who views the final image displayed on a video monitor. The originality of our approach to visual simulation is to take the aberrational characteristics of the observer's eye into account in the calculation. We validated our simulation by comparing images degraded by simulated dioptric blur with real defocused images seen through corresponding optical lenses. RESULTS: When using a small (2.5 mm) pupil size and a "typical" observer wavefront aberration model, there was a close resemblance between optical and simulated blurs. Although it was not necessary to consider the measured aberrations of the subject when simulating vision with a small pupil size, this requirement could not be ignored when vision through a larger pupil was simulated. With a 5.7-mm pupil diameter, use of Shack-Hartmann measurements of the ocular aberrations of the individual observers rather than "typical" levels of aberrations for the entire population gave excellent agreement between the effects of simulated and real defocus blur in monochromatic and polychromatic light. A Bland-Altman analysis of the differences between matching simulated and real blurs for a 5.7-mm pupil in polychromatic light with the model including allowance for individual measured aberrations gave mean differences close to zero and 95% confidence limits of about +/-0.25 D over a defocus range of -2.00 to +2.00 D. CONCLUSION: The simulation technique can be expected to be a useful tool to evaluate the potential performance of an eye that wears various designs of corrective lens.

Adult↗

Human surge linear vestibulo-ocular reflex during tertiary gaze viewing.

The otolith-mediated linear vestibulo-ocular reflex (IVOR) was studied in 9 normal humans undergoing transient whole-body surges at 0.5 g peak acceleration while viewing targets eccentrically placed in tertiary positions that combined horizontal and vertical eccentricities at distance of 15, 25, or 50 cm both in darkness and light. Mean velocity gain (+/-SEM) for the horizontal component was 0.61 +/- 0.04 in darkness and increased to 0.72 +/- 0.03 for visible targets (P < 0.05), and for the vertical component was 0.54 +/- 0.02 in darkness, not significantly different from horizontal component gain. For visible targets, vertical component gain significantly increased to 0.63 +/- 0.04 (P < 0.05) with visible targets, but remained significantly less than horizontal component gain.

Eye Movements↗

The Hirschberg test: correlation with corneal radius and axial length.

The results of this study indicate that the axial length is not strongly associated with the Hirschberg formula, and therefore the axial length does not have to be considered when measuring an ocular deviation with the Hirschberg test. This study does demonstrate an association between the radius of curvature of the cornea and the Hirschberg formula. For patients with keratometer readings below 46 D, the use of the standard Hirschberg formula, 1 mm = 22 delta, is appropriate. For patients with keratometer readings greater than 46 D, and with deviations greater than about 30 delta, the use of the Hirschberg formula, 1 mm = 27 delta, is more appropriate. When performing the Hirschberg test on infants up to about 2 months of age, the use of the higher Hirschberg formula would probably be more appropriate because this age group often has higher keratometer readings.

Cornea↗

Alterations of ocular motility in cerebellar pathology. An electro-oculographic study.

Saccadic as well as smooth pursuit movements were studied by means of electro-oculograms in a group of 14 patients affected by cerebellar diseases. Ten patients had cerebellar atrophies, and four had undergone surgery for cerebellar tumors. Loss of gain of the pursuit system and metric alteration of saccades were the most striking abnormalities observed. Dysmetria was shown to be related to the amplitude of the movement and to the sector of the perimeter within which the movement occurs (movements occurring in the more eccentric sector were more disturbed). A tendency to produce saccades slower than normal was noted in patients affected by olivopontocerebellar atrophy. The clinical and pathophysiological significance of this finding is discussed with particular reference to Wadia-Swaami hereditary ataxia.

Adult↗

[The one and a half vertical syndrome as the only evidence of bilateral thalamo-subthalamic infarction].

INTRODUCTION: The one and a half vertical syndrome consists of paralysis of upward vertical conjugated gaze and monocular paralysis of downward gaze or vice-versa. It occurs as a consequence of a mesencephalodiencephalic lesion, either unilaterally or bilaterally, due to effects on structures such as the interstitial nucleus of Cajal, posterior commissure and rostral interstitial nucleus of the medial longitudinal bundle. The arterial supply of the structures involved in the supranuclear control of vertical gaze depends on the paramedian thalamic arteries (or posterior thalamo-subthalamic arteries) and the paramedian peduncular arteries. CLINICAL CASE: We present the case of a 58 year old woman with non-insulin dependent diabetes mellitus who presented with sudden onset of diplopia of vertical gaze. On examination there was paralysis of superior vertical conjugated gaze and monocular paralysis of ocular infraversion (one and a half syndrome) with no other pathological findings. Cerebral angioresonance and Doppler studies of the territory supplied by the posterior cerebral artery were normal. CONCLUSIONS: Both unilateral and bilateral mesencephalodiencephalic infarcts usually present clinically as disorders of consciousness and of conduct (the almost universal form of presentation), involvement of the pyramidal tract, the third cranial nerves and a supranuclear disorder of vertical gaze. The unusual feature of the case we present is a bilateral thalamo-subthalamic infarct which presented solely as a one and a half vertical syndrome with no clinically apparent effect on anatomically adjacent structures. We have not found any similar cases in the literature.

Cerebral Infarction↗