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Biomedical subjects

L F Dell'Osso

Publications and source records attributed to L F Dell'Osso.

At least 19 recordsLinked to original sources
PubMed2006

The sub-clinical see-saw nystagmus embedded in infantile nystagmus.

A transient, decompensated vertical phoria in an individual with infantile nystagmus syndrome (INS) resulted in two images that oscillated vertically-a diplopic oscillopsia. Ocular motor studies during the vertical oscillopsia recreated by vertical prisms, led to the identification of a sub-clinical see-saw nystagmus (SSN), present under the prism-induced diplopic condition. Retrospective analysis of ocular motor recordings made prior to the above episode of vertical diplopia revealed the presence of that same sub-clinical SSN. The SSN had not been detected previously despite extensive observations and recordings of this subject's pendular IN over a period of forty years. Three- dimensional search-coil data from fourteen additional INS subjects (with pendular and jerk waveforms) confirmed the existence of sub-clinical SSN embedded within the clinically detectable horizontal-torsional IN in seven of the fifteen and a sub-clinical, conjugate, vertical component in the remaining eight. Unlike the clinically visible SSN found in achiasma, the cause of this sub-clinical SSN is hypothesized to be due to a failure of the forces of the oblique muscles (responsible for the torsional component of the IN) to balance out the associated forces of the vertical recti; the net result is a small, sub-clinical SSN. Thus, so-called "horizontal" IN is actually a horizontal-torsional oscillation with a secondary, sub-clinical SSN or conjugate vertical component. The suppression of oscillopsia by efference copy in INS appears to be accomplished for each eye individually, even in a binocular individual. However, failure to fuse the two images results in oscillopsia of one of them.

Diplopia↗
PubMed2006

Tenotomy does not affect saccadic velocities: support for the "small-signal" gain hypothesis.

We investigated the effects of four-muscle tenotomy on saccadic characteristics in infantile nystagmus syndrome (INS) and acquired pendular nystagmus (APN). Eye movements of 10 subjects with INS and one with APN were recorded using infrared reflection, magnetic search coil, or high-speed digital video. The expanded nystagmus acuity function (NAFX) quantified tenotomy-induced foveation changes in the INS. Saccadic characteristics and peak-to-peak nystagmus amplitudes were measured. Novel statistical tests were performed on the saccadic data. Six out of the 10 INS subjects showed no changes in saccadic duration, peak velocity, acceleration, or trajectory. In the other four, the differences were less than in peak-to-peak amplitudes (from 14.6% to 39.5%) and NAFX (from 22.2% to 162.4%). The APN subject also showed no changes despite a 50% decrease in peak-to-peak amplitude and a 34% increase in NAFX. The "small-signal" changes (peak-to-peak nystagmus amplitude and NAFX) were found to far exceed any "large-signal" changes (saccadic). Tenotomy successfully reduced INS and APN, enabling higher visual acuity without adversely affecting saccadic characteristics. These findings support the peripheral, small-signal gain reduction (via proprioceptive tension control) hypothesis. Current linear plant models, limited to normal steady-state muscle tension levels, cannot explain the effects of the tenotomy.

Acceleration↗
PubMed2004

Time-varying, slow-phase component interaction in congenital nystagmus.

We investigated the nystagmus of a 12-year-old boy with suspected X-linked congenital nystagmus (CN) and exophoria to determine the underlying mechanisms and component signals in the 'dual-velocity' and other slow phases of his Asymmetric (a)Periodic Alternating Nystagmus (APAN). Fast Fourier transforms (FFT) were performed on the waveforms and residual data after subtracting a sawtooth waveform whose amplitude and frequency matched those of the jerk nystagmus. The FFT analyses identified two frequency components (jerk--4 Hz and pendular--4 and 8 Hz, variable) that varied differently in intensity and frequency/phase over the time-course of the APAN. We synthesized each of the patient's slow phases using summation of sawtooth and sinusoidal waveforms. The resulting waveforms included jerk (with different slow-phase appearances), dual jerk, and pendular. We demonstrated that the pendular nystagmus seen during the neutral phase of APAN and the appearance of either decelerating (mimicking latent nystagmus), dual-velocity, or dual-jerk slow phases can be explained and produced by the summation of linear and pendular components of variable amplitudes and frequencies/phases. Thus, one mechanism may be responsible for all the variation seen in this patient's slow phases, rather than the less parsimonious hypothesis of a switched-tonic-imbalance mechanism that we had originally suggested to simulate the dual-velocity waveform.

Child↗
PubMed2002

An expanded nystagmus acuity function: intra- and intersubject prediction of best-corrected visual acuity.

The Nystagmus Acuity Function (NAF) provides an objective measurement of the foveation characteristics of nystagmus waveforms and an assessment of potential visual acuity for subjects with congenital (CN) or latent/manifest latent (LMLN) nystagmus. It is based on the subjects' ability to maintain fixation within a physiologically based 'foveation window' of +/- 0.5 degrees and +/- 4.0 degrees/s. However, some subjects are incapable of controlling fixation well enough to remain within this window with duration sufficient for good foveation. To obtain a measure of the CN waveforms of these individuals, we are proposing an eXpanded Nystagmus Acuity Function (NAFX) that relaxes either the position limit, the velocity limit, or both. Data used in this study comes from 11 human subjects with CN (10 idiopathic and 1 with achiasma) and a Belgian sheepdog with achiasma. Visual acuity was tested with a standard Snellen chart and eye movements recorded with infrared oculography or scleral search coil. For the NAFX to be useful, it must not only be applicable for subjects who cannot maintain fixation within the standard limits of the NAF, but also must yield results equivalent to those obtained with the NAF when testing subjects who are capable of maintaining good fixation control. For the latter subjects, the amount of time when position and velocity fell within the expanded limits was measured, the standard deviations of the position and velocity during these times were calculated, and a tau-surface for the exponential function was generated to guarantee the equivalence between the NAF and the NAFX. We developed an automated NAFX equivalent to the original NAF. We demonstrated that equivalence in 10 subjects and the use of the NAFX on two additional (1 human and 1 canine) subjects who were incapable of maintaining fixation within the standard position and velocity limits. We demonstrated the effects of surgery and related the results to visual acuity. We found the results to be comparable to those seen when applying the NAF to subjects who had good fixation control. The NAFX can be determined for CN and LMLN subjects with poor control of fixation by extending the standard NAF position and/or velocity limits for foveation. The resulting function can be used along with the longest foveation domain (derived from the NAFX to measure breadth of a high-NAFX region) to identify the gaze or convergence angles with the best waveform and to predict the best-possible visual acuity that could be achieved with the reduction of their nystagmus.

Algorithms↗
PubMed2002

The torsional component of "horizontal" congenital nystagmus.

OBJECTIVES: To study the relationship between the major horizontal and minor torsional components of congenital nystagmus to elucidate the diagnostic importance, effects on vision, and pathogenetic implications of the torsional components. METHODS: We recorded the eye movements of 13 subjects with congenital nystagmus using a three-dimensional magnetic search coil technique over a 15-year period. The subjects fixated on stationary targets straight ahead and along the horizontal and vertical meridians. Six of the 10 subjects with horizontal congenital nystagmus were asymptomatic; the remaining 4 (plus two with a vertical component to their congenital nystagmus) had adult-onset symptoms. An additional subject without symptoms had a vertical congenital nystagmus component plus seesaw nystagmus; one of the symptomatic subjects also had seesaw nystagmus. RESULTS: In all 13 subjects, the horizontal and torsional cycles were phase-locked, and positive horizontal (rightward), vertical (upward, if any), and torsional (clockwise) motion coincided in 10 subjects. That is, rightward horizontal eye rotation coincided with clockwise curvilinear motion (rightward and downward) of the upper pole of each eye. During the horizontal foveation periods, torsional motion was also of low velocity. In 2 of 13 subjects, the torsional waveforms differed from those in the horizontal plane; in others, the direction or the variation with gaze angle differed from that predicted by Listing. In each of the 13 subjects, the torsional components ranged from 8.16% to 94.42% (median, 32.94%) of the peak-to-peak magnitudes of the congenital nystagmus. In most cases, the measured torsion was far greater than that predicted by Listing's law for a worst-case analysis (range, 0.69-11.83%; median, 4.91%). The torsional components of the two subjects with seesaw nystagmus were 60.48% and 264.02%. CONCLUSIONS: The manner in which the horizontal and torsional components of "horizontal" congenital nystagmus were phase-locked made clinical detection of the torsional component difficult. Most "horizontal" congenital nystagmus is actually horizontal-torsional congenital nystagmus. Visual acuity during horizontal foveation periods is not significantly diminished by torsional motion. In only one subject did the torsional component of the congenital nystagmus have an amplitude equivalent to Listing torsion; in the other 12 subjects, torsion exceeded our estimate of what Listing's law would predict. The torsional components of the seesaw nystagmus in two subjects also greatly exceeded the torsion predicted by Listing torsion. The most parsimonious explanation for our data is that the cyclic torsion in congenital nystagmus was generated centrally and not a result of Listing torsion, mechanical crosstalk, or normal or abnormal extraocular-muscle (plant) dynamics. Further measurements are needed to confirm this hypothesis.

Adult↗
PubMed2001

A normal ocular motor system model that simulates the dual-mode fast phases of latent/manifest latent nystagmus.

The fast phases of latent/manifest latent nystagmus (LMLN) may either cause the target image to fall within (foveating) or outside (defoveating) the foveal area. We previously verified that both types are generated by the same mechanism as voluntary saccades and propose a hypothetical, dual-mode mechanism (computer model) for LMLN that utilizes normal ocular-motor control functions. Fixation data recorded during the past 30 years from 97 subjects with LMLN using both infrared and magnetic search coil oculography were used as a basis for our simulations. The MATLAB/Simulink software was used to construct a robust, modular, ocular motor system model, capable of simulating LMLN. Fast-phase amplitude versus both peak velocity and duration of simulated saccades were equivalent to those of saccades in normal subjects. Based on our LMLN studies, we constructed a hypothetical model in which the slow-phase velocity acted to trigger the change between foveating and defoveating LMLN fast phases. Foveating fast phases were generated during lower slow-phase velocities whereas defoveating fast phases occurred during higher slow-phase velocities. The bidirectional model simulated Alexander's law behavior under all viewing and fixation conditions. Our ocular-motor model accurately simulates LMLN patient ocular motility data and provides a hypothetical explanation for the conditions that result in both foveating and defoveating fast phases. As is the case for normal physiological saccades, the position error determined the saccadic amplitudes for foveating fast phases. However, the final slow-phase velocity determined the amplitudes of defoveating fast phases. In addition, we suggest that individuals with LMLN use their fixation subsystem to further decrease the slow-phase velocity as the target image approaches the foveal center.

Adolescent↗
PubMed2000

Saccades to sounds: effects of tracking illusory visual stimuli.

In 10 normal human subjects, we studied the accuracy of memory-guided saccades made to the remembered locations of visual targets and sounds. During the time of stimulus presentation, subjects were smoothly tracking a projected laser spot that was moving horizontally across a tangent screen, sinusoidally +/-15 degrees at 0.25 Hz. In one set of experiments, the laser spot moved across a 40 degrees x 28 degrees random dot display that moved synchronously in the vertical plane; this induced a strong illusion that the trajectory of the laser spot was diagonal (variant of Duncker illusion). In control experiments, the laser spot moved across the same display, which was stationary. The visual targets and speakers were at six locations (range +/-15 degrees ) in the horizontal plane. Saccades made to the remembered locations of targets presented during background motion (illusion) were significantly (P < 0.05) more inaccurate than with the background stationary (control) in 9 of 10 subjects for lights and in 6 of 10 subjects for sounds. As a group, the median change in errors due to the Duncker illusion was approximately 2.5 times greater for visual compared with acoustic targets (P < 0.001). These findings are consistent with electrophysiological studies which have shown that neurons in the primate lateral intraparietal area (LIP) may respond to both visual and auditory targets and these neurons are also influenced by the Duncker illusion during programming of memory-guided saccades.

Acoustic Stimulation↗
PubMed1999

A new surgery for congenital nystagmus: effects of tenotomy on an achiasmatic canine and the role of extraocular proprioception.

PURPOSE: Human eye-movement recordings have documented that surgical treatment of congenital nystagmus (CN) also produces a broadening of the null zone and changes in foveation that allow increased acuity. We used the achiasmatic Belgian sheepdog, a spontaneously occurring animal model of human CN and see-saw nystagmus (SSN), to test the hypothesis that changes induced by surgical interruption of the extraocular muscle afference without a change in muscle-length tension could damp both oscillations. METHODS: An achiasmatic dog with CN and SSN underwent videotaping and infrared oculography in a sling apparatus and head restraints before and after all extraocular muscles (stage 1: 4 horizontal rectus muscles and stage 2 [4 months later]: 4 vertical rectus muscles and 4 oblique muscles) were surgically tenotomized and immediately reattached at their original insertions. RESULTS: The dog had immediate and persistent visible, behavioral, and oculographic changes after each stage of this new procedure. These included damped CN and SSN, increased ability to maintain fixation, and increased periods of maintaining the target image on the area centralis over a broad range of gaze angles. CONCLUSIONS: Severing and reattaching the tendons of the extraocular muscles affect some as-yet-unknown combination of central nervous system processes producing the above results. This new procedure may prove effective in patients with CN with either no null, a null at primary position, or a time-varying null (due to asymmetric, (a)periodic, alternating nystagmus). We infer from our results in an achiasmatic dog that tenotomy is the probable cause of the damping documented in human CN after Anderson-Kestenbaum procedures and should also damp CN and SSN in achiasma in humans. It may also prove useful in acquired nystagmus to reduce oscillopsia. The success of tenotomy in damping nystagmus in this animal suggests that the proprioceptive feedback loop has a more important role in ocular-motor control than has been appreciated. Finally, we propose a modified bimedial recession procedure, on the basis of the damping effects of tenotomy.

Animals↗
PubMed1999

Clinical and ocular motor analysis of congenital nystagmus in infancy.

PURPOSE: The purpose of this study was to identify the clinical and ocular motility characteristics of congenital nystagmus and to establish the range of waveforms present in infancy. BACKGROUND: The clinical condition of congenital nystagmus usually begins in infancy and may or may not be associated with visual sensory system abnormalities. Little is known about its specific waveforms in infancy or their relationship to the developing visual system. METHODS: Forty-three infants with involuntary ocular oscillations typical of congenital nystagmus were included in this analysis. They were evaluated both clinically and with motility recordings. Eye movement analysis was performed off line from both chart recordings and computer analysis of digitized data. Variables analyzed included age, sex, vision, ocular abnormalities, head position, null-zone or neutral-zone characteristics, symmetry, conjugacy, waveforms, frequencies, foveation times, and responses to convergence and to monocular cover. RESULTS: Patient ages ranged from 3 to 18 months (average, 9.2 months). Seventeen patients (40%) had abnormal vision, 3 had a positive family history of nystagmus, 11 had strabismus, 16 (37%) had a head posture, 26 (60%) had null and neutral positions, 14 (33%) had binocular asymmetry, and all were horizontally conjugate. Average binocular frequency was 2.8 Hz, and average monocular frequency was 4.6 Hz. The waveforms were both jerk and pendular; average foveation periods in patients with normal vision were more than twice as long as those in patients with abnormal vision. CONCLUSIONS: Common clinical characteristics and eye-movement waveforms of congenital nystagmus begin in infancy, and waveform analysis at this time helps with both diagnosis and visual status.

Electrooculography↗
PubMed1999

Enhancement of the vestibulo-ocular reflex by prior eye movements.

We investigated the effect of visually mediated eye movements made before velocity-step horizontal head rotations in eleven normal human subjects. When subjects viewed a stationary target before and during head rotation, gaze velocity was initially perturbed by approximately 20% of head velocity; gaze velocity subsequently declined to zero within approximately 300 ms of the stimulus onset. We used a curve-fitting procedure to estimate the dynamic course of the gain throughout the compensatory response to head rotation. This analysis indicated that the median initial gain of compensatory eye movements (mainly because of the vestibulo-ocular reflex, VOR) was 0. 8 and subsequently increased to 1.0 after a median interval of 320 ms. When subjects attempted to fixate the remembered location of the target in darkness, the initial perturbation of gaze was similar to during fixation of a visible target (median initial VOR gain 0.8); however, the period during which the gain increased toward 1.0 was >10 times longer than that during visual fixation. When subjects performed horizontal smooth-pursuit eye movements that ended (i.e., 0 gaze velocity) just before the head rotation, the gaze velocity perturbation at the onset of head rotation was absent or small. The initial gain of the VOR had been significantly increased by the prior pursuit movements for all subjects (P < 0.05; mean increase of 11%). In four subjects, we determined that horizontal saccades and smooth tracking of a head-fixed target (VOR cancellation with eye stationary in the orbit) also increased the initial VOR gain (by a mean of 13%) during subsequent head rotations. However, after vertical saccades or smooth pursuit, the initial gaze perturbation caused by a horizontal head rotation was similar to that which occurred after fixation of a stationary target. We conclude that the initial gain of the VOR during a sudden horizontal head rotation is increased by prior horizontal, but not vertical, visually mediated gaze shifts. We postulate that this "priming" effect of a prior gaze shift on the gain of the VOR occurs at the level of the velocity inputs to the neural integrator subserving horizontal eye movements, where gaze-shifting commands and vestibular signals converge.

Adult↗
PubMed1999

Latent and congenital nystagmus in Down syndrome.

OBJECTIVES: Although nystagmus has been reported in Down syndrome (DS), it has been poorly characterized, because most investigators have relied on clinical observations rather than on eye movement recordings. This study was conducted to investigate nystagmus in DS, using quantitative measurements of eye movements. METHODS: Ocular motility and visual functions were examined in 26 unselected adults with DS and compared with those in an age-matched group of 35 subjects with other causes of mental retardation. The eye movements of those with clinically evident nystagmus were recorded with the infrared technique. We also recorded the eye movements of a child with DS and nystagmus. RESULTS: Nystagmus was identified in six (23%) adults with DS and in none in the control group. All six patients showed latent/manifest latent nystagmus (LMLN), prominent with the covering of one eye, and esodeviations of 10 to 30 prism diopters. Eye movement recordings confirmed LMLN with its exponentially decaying waveform. Frequencies ranged from 2 to 5 Hz and amplitudes from 5 degrees to 20 degrees. While attempting to fixate straight ahead in the absence of visual cues, three subjects exhibited shifts in the mean eye position. In contrast with the findings in adults, the only child with DS examined had both congenital nystagmus and LMLN waveforms. CONCLUSIONS: The predominant type of nystagmus in the study subjects with DS is LMLN. The high prevalence of LMLN may reflect abnormal integration of visuospatial information that is typical of DS. The concurrent presence of congenital nystagmus in a child but only LMLN in the adults with DS raises the possibility of age-related waveform changes or could reflect sample variation.

Adult↗
PubMed1998

Pendular nystagmus in patients with peroxisomal assembly disorder.

BACKGROUND: Pendular nystagmus commonly occurs in congenital and acquired disorders of myelin. OBJECTIVE: To characterize the nystagmus in 3 siblings with an infantile form of an autosomal recessive peroxisomal assembly disorder causing leukodystrophy. DESIGN: We examined visual function and measured eye movements using infrared oculography. We noted changes in eye speed and frequency before and after the administration of gabapentin to 1 patient. RESULTS: All 3 siblings showed optic atrophy and pendular nystagmus that was predominantly horizontal, at a frequency of 3 to 6 Hz, with phase shifts of 45 degrees to 80 degrees between the oscillations of each eye. Gabapentin administered to 1 child caused a modest improvement of vision and the reduction of the velocity and frequency of oscillations in the eye with worse nystagmus. CONCLUSION: The pendular nystagmus in these patients was due to their leukodystrophy and may have a similar pathogenesis to the oscillations seen in other disorders affecting central myelin.

Adolescent↗
PubMed1998

The congenital and see-saw nystagmus in the prototypical achiasma of canines: comparison to the human achiasmatic prototype.

We applied new methods for canine eye-movement recording to the study of achiasmatic mutant Belgian Sheepdogs, documenting their nystagmus waveforms and comparing them to humans with either congenital nystagmus (CN) alone or in conjunction with achiasma. A sling apparatus with head restraints and infrared reflection with either earth- or head-mounted sensors were used. Data were digitized for later evaluation. The horizontal nystagmus (1-6 Hz) was similar to that of human CN. Uniocular and disconjugate nystagmus and saccades were recorded. See-saw nystagmus (SSN), not normally seen with human CN, was present in all mutants (0.5-6 Hz) and in the one human achiasmat studied thus far. This pedigree is an animal model of CN and the SSN caused by achiasma or uniocular decussation. Given the finding of SSN in all mutant dogs and in a human, achiasma may be sufficient for the development of congenital SSN and, in human infants, SSN should alert the clinician to the possibility of either achiasma or uniocular decussation. Finally, the interplay of conjugacy and disconjugacy suggests independent ocular motor control of each eye with variable yoking in the dog.

Animals↗
PubMed1998

Two additional scenarios for see-saw nystagmus: achiasma and hemichiasma.

The discovery of canine achiasma and hemichiasma has been followed by the identification of human achiasma (four individuals, to date). See-saw nystagmus was present in all cases of achiasma (canine and human) and in one of two cases of canine hemichiasma studied. Human infants with see-saw nystagmus should be imaged for possible structural abnormalities of the optic chiasm.

Animals↗