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At least 901 records · Page 50Linked to original sources
PubMed1998

Oculomotor phenotypes in autosomal dominant ataxias.

OBJECTIVE: To quantify the oculomotor features of the common spinocerebellar ataxia (SCA) syndromes. SETTING: University ataxia clinic. PATIENTS: Twenty probands with documented SCA mutations. METHODS: Electro-oculographic recordings of saccadic, smooth pursuit, optokinetic, vestibular, and visual-vestibular eye movements. RESULTS: Distinct phenotype and genotype patterns were identified with modest overlap between patterns. Slowing of saccade peak velocities occurred only in SCA1 and SCA2, being present in 100% of patients with SCA2. Impaired vestibulo-ocular reflex gain occurred with SCA3 only. Patients with SCA6 had prominent deficits in smooth tracking but normal saccade velocities and vestibuloocular reflex gain. CONCLUSIONS: The oculomotor findings are consistent with pure cerebellar involvement in SCA6, pontine involvement in SCA1 and SCA2, and vestibular nerve or nuclei involvement in SCA3. These phenotypes can be useful for clinical diagnosis and for investigating the mechanism of system specificity with the SCA syndromes.

Adult↗
PubMed1988

The Hirschberg test: a double-masked clinical evaluation.

Though the Hirschberg test has been used for over a 100 years, several different formulas are still being recommended and used clinically to determine the amount of a given ocular deviation. The purpose of this study was to obtain a double-masked clinical evaluation of the Hirschberg test. Several strabismic patients were evaluated by one investigator using the alternate cover test and by another investigator using a photographic Hirschberg procedure in a double-masked procedure. It was determined that the Hirschberg test can be used for strabismic patients of all ages and that the most appropriate formula to use is 1 mm = 22 delta.

Clinical Trials as Topic↗
PubMed1995

Dynamic properties of the human vestibulo-ocular reflex during head rotations in roll.

We investigated the dynamic properties of the human vestibulo-ocular reflex (VOR) during roll head rotations in three human subjects using the magnetic search coil technique. In the first of two experiments, we quantify the behavior of the ocular motor plant in the torsional plane. The subject's eye was mechanically displaced into intorsion, extorsion or abduction, and the dynamic course of return of the eye to its resting position was measured. The mean predominant time constants of return were 210 msec from intorsion, 83 msec from extorsion, and 217 msec from abduction, although there was considerable variability of results from different trials and subjects. In the second experiment, we quantify the efficacy of velocity-to-position integration of the vestibular signal. Position-step stimuli were used to test the torsional or horizontal VOR, being applied with subjects heads erect or supine. After a torsional position-step, the eye drifted back to its resting position, but after a horizontal position-step the eye held its new horizontal position. To interpret these responses we used a simple model of the VOR with parameters of the ocular motor plant set to values determined during Exp 1. The time constant of the velocity-to-position neural integrator was smaller (typically 2 sec) in the torsional plane than in the horizontal plane (> 20 sec). No disconjugacy of torsional eye movements was observed. Thus, the dynamic properties of the VOR in roll differ significantly from those of the VOR in yaw, reflecting different visual demands placed on this reflex in these two planes.

Adult↗
PubMed1998

Human head-free gaze saccades to targets flashed before gaze-pursuit are spatially accurate.

Previous studies have shown that accurate saccades can be generated, in the dark, that compensate for movements of the visual axis that result from movements of either the eyes alone or the head alone that intervene between target presentation and saccade onset. We have carried out experiments with human subjects to test whether gaze saccades (gaze = eye-in-space = eye-in-head + head-in-space) can be generated that compensate for smooth pursuit movements of gaze that intervene between target onset and gaze-saccade onset. In both head-unrestrained (head-free) and -restrained (head-fixed) conditions, subjects were asked to make gaze shifts, in the dark, to the remembered location of a briefly flashed target. On most trials, during the memory period, the subjects carried out intervening head-free gaze pursuit or head-fixed ocular pursuit along the horizontal meridian. On the remaining (control) trials, subjects did not carry out intervening pursuit movements during the memory period; this was the classical memory-guided saccade task. We found that the subjects accurately compensated for intervening movements of the visual axis in both the head-free and head-fixed conditions. We conclude that the human gaze-motor system is able to monitor on-line changes in gaze position and add them to initial retinal error, to program spatially accurate gaze saccades.

Adult↗
PubMed1981

Visual-vestibular interaction in multiple sclerosis.

Visual modulation of the vestibuloocular reflex (VOR) was analyzed in 20 patients with multiple sclerosis who had no vestibular or ocular motor symptoms. Visual suppression of the VOR was impaired in 75% of patients. VOR gains in darkness were elevated in 35% of patients. Elevated VOR gain in darkness is attributed to vestibular adaptation to defective smooth pursuit. This vestibular plasticity achieved retinal image stability by matching eye velocity to head velocity when stationary targets were viewed. The high incidence of impaired visual suppression of the VOR rivals the sensitivity of other physiologic tests used to identify multiple sclerosis.

Adult↗
PubMed1992

[Balint's syndrome--visual disorientation].

The three major components of the Balint's syndrome are described: 1) visual disorientation or simultanagnosia, i.e. the inability to perceive the visual field as a whole, 2) ocular apraxia, a deficit of visual scanning and 3) optic ataxia, an impairment of pointing and reaching under visual guidance. Our description of the syndrome is illustrated by three case histories. The complete syndrome is rare, and partial syndromes have been reported more frequently. Sudden and severe hypotension resulting in bilateral borderzone infarction in the occipito-parietal region is reported to be the most frequent cause of complete Balint's syndrome. Lack of awareness of the syndrome may lead to misdiagnosis such as blindness, psychosis, or dementia.

Adult↗
PubMed2004

Impaired modulation of the vestibulo-ocular reflex in Huntington's disease.

The vestibulo-ocular reflex (VOR) stabilizes gaze during movement, in conjunction with other afferent information: visual, proprioceptive, and somaesthetic. The reflex can either be augmented or suppressed, depending on visual requirements, and undergoes long-term adaptation to compensate for physical changes in the subject. Importantly, over relatively short periods of time, the VOR should function consistently under the same circumstances. This study examines VOR function in patients with Huntington's disease (HD), with a view to investigating cortical influences on the reflex. Horizontal eye movements were recorded in 9 patients with HD and 7 normal subjects, using the scleral search coil technique, in response to high frequency, unpredictable head rotations imposed manually. To establish base VOR function, recordings were made in darkness, without instruction, before and after wearing x2 magnifying lenses for a period of 2 hours to adapt the reflex. Recordings were also made before adaptation, while fixating a stationary visual target (VOR augmentation), and while fixating a target moving with the head (VOR suppression). Although results suggest that the VOR is preserved in HD, with relatively normal gain values and appropriate augmentation and suppression of the reflex with visual input, patients were unable to adapt the VOR to altered visual conditions. This represents a novel finding in HD and suggests that cortical structures compromised in HD exert influences on the long-term adaptation of the VOR.

Adult↗
PubMed1995

The human ocular torsion position response during yaw angular acceleration.

Recent results by Wearne [(1993) Ph.D. thesis] using the scleral search-coil method of measuring eye position indicate that changes in ocular torsion position (OTP) occur during yaw angular acceleration about an earth vertical axis. The present set of experiments, using an image processing method of eye movement measurement free from the possible confound of search coil slippage, demonstrates the generality and repeatability of this phenomenon and examines its possible causes. The change in torsion position is not a linear vestibulo-ocular reflex (LVOR) response to interaural linear acceleration stimulation of the otoliths, but rather the effect is dependent on the characteristics of the angular acceleration stimulus, commencing at the onset and decaying at the offset of the angular acceleration. In the experiments reported here, the magnitude of the angular acceleration stimulus was varied and the torsion position response showed corresponding variations. We consider that the change in torsion position observed during angular acceleration is most likely to be due to activity of the semicircular canals.

Acceleration↗
PubMed1995

[Posterior cortical atrophy with progressive visual agnosia].

Interest in progressive focal cerebral syndromes associated with classical degenerative diseases has increased in recent years. Descriptions of posterior cortical atrophy with progressive visual agnosia are relatively rare. We present 5 patients (2 women) ranging in age between 57 and 72 years old. In all cases symptoms began and progressed with no known etiology. All cases were sporadic. The main clinical signs are difficulty in recognizing objects, colors, persons or places; topographical disorientation and visual memory alterations; alexia, simultagnosia, loss of ocular fixing and optic ataxia. Some patients presented other disturbances of praxis or memory and 2 progressed to global dementia. Language function was preserved and behavioral disturbances did not develop. The amplitude of the P100 visual evoked potential was low but latency was normal in 4 patients and prolonged in 1. Brain images showed atrophy and hypoperfusion in the parieto-occipital area. The neuropathology status of these patients is unknown.

Adult↗
PubMed1993

Eye movements in a familial vestibulocerebellar disorder.

Eye movement abnormalities consisting of poor or absent smooth pursuit and vestibulo-ocular reflex suppression, gaze-paretic and rebound nystagmus, slow build-up of optokinetic nystagmus, mildly hyperactive vestibulo-ocular reflex, and a high incidence of strabismus were inherited in an autosomal dominant fashion in 10 members of a non-consanguineous English caucasian family. The onset was in early childhood, but was not congenital. In 7 cases there was no tremor, dizziness, consistent ataxia, or other cerebellar signs that are often associated with these ocular motor deficits, and apart from strabismus, patients were asymptomatic. Magnetic resonance imaging of the propositus was normal. After childhood there appears to be no progression, with the oldest affected member being 40 years. Two members had been prone to falling in childhood, and one admitted to dizziness when tired. This condition, which is probably benign, has not been previously described and may represent a very mild variant of episodic ataxia or a new vestibulocerebellar syndrome.

Adolescent↗
PubMed1984

Ocular motility in craniofacial reconstruction.

Of 140 children undergoing major craniofacial reconstruction at The Children's Hospital of Philadelphia and examined preoperatively and postoperatively by the Ophthalmology Division, only 10 had surgically induced alteration in primary position horizontal alignment, 4 greater than 10 prism diopters. Only 2 patients had a new strabismus in primary position created by craniofacial surgery, both cranial nerve palsies. We believe early strabismus surgery is advantageous for attainment of binocularity and ease of tissue manipulation. Of 44 patients with craniofacial dysostosis (Crouzon), 20 had preoperative strabismus, and 12 required extraocular muscle surgery. Five of 12 had anomalies of extraocular muscle number and structure, usually involving the superior rectus muscle.

Adolescent↗
PubMed2005

Non-invasive phakometric measurement of corneal and crystalline lens alignment in human eyes.

We describe a non-invasive phakometric method for determining corneal axis rotation relative to the visual axis (beta) together with crystalline lens axis tilt (alpha) and decentration (d) relative to the corneal axis. This does not require corneal contact A-scan ultrasonography for the measurement of intraocular surface separations. Theoretical inherent errors of the method, evaluated by ray tracing through schematic eyes incorporating the full range of human ocular component variations, were found to be larger than the measurement errors (beta < 0.67 degrees , alpha < 0.72 degrees and d < 0.08 mm) observed in nine human eyes with known ocular component dimensions. Intersubject variations (mean +/- S.D.: beta = 6.2 +/- 3.4 degrees temporal, alpha = 0.2 +/- 1.8 degrees temporal and d = 0.1 +/- 0.1 mm temporal) and repeatability (1.96 x S.D. of difference between repeat readings: beta +/- 2.0 degrees , alpha +/- 1.8 degrees and d +/- 0.2 mm) were studied by measuring the left eyes of 45 subjects (aged 18-42 years, 29 females and 16 males, 15 Caucasians, 29 Indian Asians, one African, refractive error range -7.25 to +1.25 D mean spherical equivalent) on two occasions.

Adolescent↗
PubMed1989

[The effect of alcohol on the control of the vestibulo-ocular reflex and gaze].

The effects of ethyl alcohol on vestibulo-ocular reflex (VOR) and gaze functions were investigated in normal adults. Twelve normal adults were administered whisky at the rate of 1.0ml/kg (ethyl alcohol 0.43g/kg), so that the mean peak concentration of breath alcohol amounted to 0.15mg/l. Before and after alcohol administration, the subjects underwent sinusoidal rotation tests with an amplitude of 40 degrees and frequencies of 0.2-0.85Hz and eye tracking tests of unidirectional motion at 30 degrees-70 degrees/sec. For the rotation test, 7 subjects were examined at the dose of 0.65g/kg ethyl alcohol. The following results were obtained. 1. The mean value of VOR gain under the condition of performing mental arithmetic in the dark was not significantly altered by alcohol. However, the magnitude of change in VOR gain showed a highly significant correlation with the gain before drinking; whereas small values were enlarged and large values were suppressed by alcohol. 2. The accuracy of spacial gaze fixation was scarcely affected by alcohol, even at the dose of 0.65g/kg. 3. Visual fixation on a head-fixed target (fixation-induced suppression of VOR) and smooth pursuit were significantly impaired by alcohol. 4. The present study indicated that VOR is regulated even in the dark by both gaze stabilizing functions, i.e., spacial gaze fixation and smooth pursuit.

Adult↗
PubMed1992

A quantitative study of eye and head movements during smooth pursuit in patients with cerebellar disease.

Eye and head movements were analysed during smooth pursuit in 16 patients with various forms of cerebellar disease. Smooth pursuit gain was reduced across all frequencies and velocities of target motion for the patient group as a whole, during both sinusoidal and pseudo-random target motion. The graded breakdown in the pursuit response, as pseudo-random target motion became less predictable, was of a similar magnitude in patients and controls, implying that the predictive pursuit mechanisms were intact in these patients. During head-free pursuit, when vestibulo-ocular reflex (VOR) suppression was necessary, performance was not significantly different from that observed during head-fixed pursuit in the patient group. This finding is similar to that noted in control subjects, and is consistent with the observation that the VOR gains associated with head movements in darkness were similar in the patient and control groups. The deficits in pursuit and VOR suppression in patients with cerebellar disease therefore represent a decrease in gain in the closed-loop visual feedback pathways with apparent sparing of the predictive pathways.

Adult↗
PubMed1994

Ocular motor behaviour of monozygotic twins with tyrosinase negative oculocutaneous albinism.

The involuntary nystagmus movements of 16-year-old monozygotic twins with tyrosinase negative oculocutaneous albinism were examined. On primary gaze both girls exhibited bilateral conjugate horizontal nystagmus, a jerk with extended foveation waveform, and similar frequencies (2.0 Hz:1.9 Hz), although the fast phases were in opposite directions. The mean amplitudes differed markedly (6.8 degrees:3.7 degrees), as did the position of the null zones (+20 degrees to +30 degrees:-25 degrees to -35 degrees) and the widths of the neutral zones (-25 degrees to +20 degrees:-25 degrees to -35 degrees). Since the twins have identical genotypes these differences must have arisen from other sources.

Adolescent↗
PubMed1999

Apparent dissociation between saccadic eye movements and the firing patterns of premotor neurons and motoneurons.

Saccadic eye movements result from high-frequency bursts of activity in ocular motoneurons. This phasic activity originates in premotor burst neurons. When the head is restrained, the number of action potentials in the bursts of burst neurons and motoneurons increases linearly with eye movement amplitude. However, when the head is unrestrained, the number of action potentials now increase as a function of the change in the direction of the line of sight during eye movements of relatively similar amplitudes. These data suggest an apparent uncoupling of premotor neuron and motoneuron activity from the resultant eye movement.

Abducens Nerve↗
PubMed1979

Congenital ocular motor apraxia. A possible disconnection syndrome.

In 1952, Cogan introduced the term "congenital ocular motor apraxia" (COA) to describe an abnormality of eye movements characterized by absent or defective voluntary horizontal gaze. Since his original description, there have been few subsequent reports of this disorder. A ten-year review of clinical records from the University of Wisconsin Hospitals disclosed eight patients with COA. In two patients subjected to neuroradiologic testing, agenesis of the corpus callosum was detected. Voluntary horizontal gaze appears to be a learned phenomenon, and defective interhemispheric transfer of visual information may be important in the pathogenesis of COA.

Agenesis of Corpus Callosum↗
PubMed1990

Evaluation of retinal orientation and gaze direction in the perception of the vertical.

The orientation of the median plane of the eye with respect to the head varies with gaze direction according to Listing's Law. The subjective vertical (SV), however, is known to be only partially affected by these involuntary variations of eye orientation. In order to learn more about the compensatory process underlying this finding, six normal-sighted young subjects were tested monocularly and binocularly in eight directions of gaze. The results show that: (1) the SVs, determined monocularly for both eyes, fall on corresponding retinal meridians, the binocular SV-settings generally lie between the monocular ones; (2) the tilt of the SV is not linearly related to the tilt angle of the median plane of the eye, as hitherto assumed. On theoretical considerations, the dependence of ocular tilt on gaze direction may be decomposed into three components, which are all treated differently in the compensation process. We interpret these results in the following way: the SV is determined from the sensorially fused image of both eyes and the tilts of the eyes are accounted for by an extra-retinal signal which is common to both eyes. The characteristics of the compensation mechanism may be explained by an extra-retinal signal which relies on information about gaze direction and Listing's Law. Such a signal might be derived from an efference copy of gaze direction commands.

Eye Movements↗