Search

A 36-year-old woman had intermittent downbeat nystagmus. Between episodes of downbeat nystagmus, eye movements were normal, except for stationary positional nystagmus and decreased vestibulo-ocular responses (VOR) to rotation. Smooth pursuit, optokinetic nystagmus, and visual-VOR interactions, which are usually impaired in patients with downbeat nystagmus and cerebellar dysfunction, were normal. Computed tomography and findings during a suboccipital craniotomy demonstrated a mild Chiari malformation, type 1. Episodic downbeat nystagmus that is not associated with other ocular motor abnormalities due to cerebellar dysfunction can be an initial sign of a Chiari malformation.

On the assumption that myelin intrusion into the papilla and peripapillary region might occur with traumatic lesions of the optic nerve, a study was made of the clinical and histopathologic changes that might be expected. Nine monkey eyes were subjected to hemostat compression of the nerve close to the globe and studied over variable periods up to 28 days. Myelin was demonstrable ophthalmoscopically, followed by variable and increasing amounts of hemorrhage. The myelin was demonstrable histopathologically only during the first 2 weeks after the manipulation and was then masked by the associated hemorrhage and gliosis. The optic nerve showed expected myelinolytic reactions.

Eighteen patients with congenital nystagmus were studied with the techniques of electronystagmography and computer analysis. We found several complex wave forms of congenital nystagmus in the primary position of gaze above those of jerk and pendular nystagmus as defined clinically. An etiologic classification of motor fixation defect and sensory fixation defect nystagmus based on waveforms is not justified. Ocular tracking studies demonstrate that the smooth pursuit system is operational in congenital nystagmus and substantiate the belief that the fast component of jerk nystagmus is a corrective movement generated by the saccadic system. Patients with congenital nystagmus are able to produce voluntary saccades with normal velocity-amplitude relationships.

A comparison was made between the phase of vestibulo-ocular reflex (VOR) responses to sinusoidal rotatory tests and the phase of the subjective sensation (SS) to rotation. A parallelism was found between the results of both sets of data such that they behaved in accordance with the prediction of the pendulum model of vestibular function. The time constant of the VOR (17.5 seconds) was longer, however, than that of the SS (13 seconds). Measurements in patients with unilateral labyrinthine pathology also showed comparable changes in both responses. One group of patients with complete unilateral labyrinthine paralysis showed an advance of the phase of the VOR and of the SS. However, phase measurements in the group of patients with only partial unilateral labyrinthine paralysis were not significantly different statistically from those of normal subjects.

In monkey extraocular muscles (EOM), a battery of histochemical reactions delineates three muscle fiber types, coarse, fine, and granular. Normal EOM are compared with EOM denervated by intracranial oculomotor nerve section. The experimentally denervated EOM fibers did not show the constellation of histologic responses typical of denervated limb muscle, making a diagnosis of a denervation process in EOM muscle very difficult. Although the denervated fine and granular fibers (but not the coarse fibers) develop diffuse extrajunctional acetylcholine receptors (AChR) following experimental denervation, this is not a reliable criterion of denervation because not all of those fibers developed it and they did not show it beyond a 12-week period following nerve section; moreover, myopathic mechanisms have previously been shown capable of provoking diffuse extrajunctional AChR in limb-muscle fibers.

Quantitative analysis of electro-oculographic recordings of eye movement in response to precise visual and vestibular stimuli makes possible the differentiation of three categories of vestibular syndromes due to pathological changes in three different parts of the visual vestibulo-ocular reflex arc: (1) decreased vestibulo-ocular reflex gain (e.g., decrease in slow component velocity), but normal fast components and visual-vestibular interaction (labyrinth and eighth nerve); (2) normal slow component velocity but abnormal fast components to all stimuli (pontine or medullar reticular formation); and (3) normal slow component velocity to vestibulo-ocular stimulaton but abnormal visual-vestibular interaction as well as normal fast components (visual-motor pathways or cerebellum).

Craniopharyngiomas are common tumors located in the suprasellar region. Contrast enhancement, cyst formation, and calcification are the three characteristic features of craniopharyngiomas on computed tomographic scan. More than 90% of suprasellar craniopharyngiomas exhibit at least two of these three features, thus providing easy radiologic detection. We treated a 41-year-old man in whom a large suprasellar craniopharyngioma producing severe visual loss was not detected by computed tomography but was easily identified with magnetic resonance imaging. Thus, despite high-resolution computed tomographic scan, large suprasellar craniopharyngiomas can be missed. Magnetic resonance imaging may be superior to computed tomography in detecting these tumors.

Measurements were made of the lesion-induced changes in vestibulo-ocular reflexes to rotatory stimuli in a group of patients with total unilateral labyrinthine paralysis and in a group with partial unilateral labyrinthine paralysis. Significant differences were found in the amplitude and phase of the response to low-frequency rotation and in the time constant of the post-rotatory response to impulsive rotation between normal subjects and patients with total unilateral labyrinthine paralysis. The results were evaluated by the use of a simplified model of vestibular function that allows the parametric evaluation of the characteristics of vestibular responses. It was found that changes in vestibulo-ocular reflex characteristics take place in parallel in both the time constant and the sensitivity of the system parameters used to describe the responses. The physiologic significance of the changes and their implication for diagnostic purposes are discussed.

We present the clinical findings in affected members of a large kindred with Gerstmann-Sträussler-Scheinker disease. Sixty-four patients exhibited progressive ataxia, dementia, and parkinsonian features. Inheritance appears to be autosomal dominant. Impaired smooth-pursuit eye movements, defective short-term memory, clumsiness of the hands, and ataxia of gait develop in the late 30s to early 60s. Eye movement abnormalities are characteristic of cerebellar dysfunction. Dementia progresses gradually over several years. Later, rigidity and bradykinesia appear and, at this stage, there is often psychosis or severe depression with rapid weight loss. Death occurs in 6 months to 2 years after onset of rigidity. Magnetic resonance imaging in 2 affected individuals showed cerebellar atrophy. There is decreased T2 signal in the basal ganglia, consistent with iron deposition.

Smooth pursuit and other eye movements were quantitatively studied in patients with chronic schizophrenia to characterize the pattern and severity of eye movement abnormalities in this disorder. Twenty-one patients with schizophrenia, 13 patients with other psychiatric disorders (manic-depressive psychosis, schizoaffective disorder, depression and obsessive-compulsive disorder), and 19 normal subjects were studied. Horizontal eye movements were recorded with DC electro-oculography and analyzed by an online computer system. Eye velocity and closed-loop gain of pursuit, optokinetic nystagmus (OKN), vestibulo-ocular response (VOR) and visual-vestibular interactions were calculated. The latency, accuracy and peak velocity-amplitude relationships of voluntary saccades were measured. The group mean gains of pursuit and OKN of the schizophrenic patients were significantly lower than those of the normal subjects. Suppression of the VOR by fixation was impaired, and the latency of saccades was prolonged. However, the differences in group mean values were small in magnitude, and the frequencies of outliers among the schizophrenic patients were low. The patients with other psychiatric disorders had a similar pattern and severity of eye movement abnormalities. The frequency and severity of eye movement abnormalities in schizophrenic patients are lower than those indicated by previous studies that used different techniques of analysis.

Voluntary saccades were studied by electro-oculography in ten patients with myasthenia gravis (MG) and in eight patients with other types of ophthalmoplegia. Despite limited range of eye movements, maximum velocities of 20 degree and 40 degree saccades in patients with MG were not significantly different from those in normal individuals, whereas maximum velocities in patients with other types of ophthalmoplegia were significantly decreased. In some myasthenic patients, small amplitude saccades were hypermetric and had high velocities, appearing clinically as "quiver" movements characteristic of MG. In MG the preservation of saccades with high initial velocities, even in the presence of severe ophthalmoplegia, suggests that muscle fibers generating rapid movements during saccades (twitch fibers) can be relatively spared when muscle fibers responsible for maintenance of excentric gaze (tonic fibers) are severely affected.

We report detailed oculomotor studies in 3 patients with central nervous system lesions and markedly decreased time constants (less than 2 seconds) of the vestibuloocular reflex (VOR). In 1 patient with Chiari type I malformation, serial measurements over 3 years documented a progressive decrease in the duration of postrotatory nystagmus (100 deg/sec steps, acceleration 140 deg/sec2) until finally there was no sustained nystagmus. At this time, the patient had no response to caloric stimulation or to sinusoidal rotation below 0.2 Hz but normal gain (peak slow-phase eye velocity/peak chair velocity) above 0.4 Hz (phase lead increased). Gaze holding, saccades, smooth pursuit, and optokinetic nystagmus were normal, but optokinetic-after-nystagmus disappeared. The other 2 patients (combined brainstem-cerebellar atrophy) had impaired gaze holding, abnormal smooth pursuit and optokinetic nystagmus, and absent optokinetic-after-nystagmus. VOR gain to step and high-frequency sinusoidal stimuli was increased. The neural mechanism that normally prolongs the VOR time constant may have reduced it in our patients.

We examined a patient with an orbital stab injury with fatal intracranial extension in whom the initial emergency examination did not reveal the extent of damage. Ophthalmologic consultation showed an afferent pupillary defect (Marcus-Gunn pupil), which prompted radiologic studies. Orbital tomograms and computerized tomography demonstrated an optic canal fracture and penetration to the contralateral right ventricle. Diabetes insipidus and thermal irregularity developed, and death occurred seven days later.

Ophthalmoplegia, ataxia, and areflexia are present in a distinctive syndrome called Fisher's syndrome. Eye-movement recordings were used to analyze ophthalmoplegia and other eye-movement abnormalities in three patients. Disorders of supranuclear ocular motor pathways (palsy of upward gaze and internuclear ophthalmoplegia) and peripheral cranial nerve palsies could account for the ophthalmoplegia. In one patient, computed tomography demonstrated a lucency in the midbrain that could be correlated with palsy of upward gaze and bilateral internuclear ophthalmoplegia. Eye-movement abnormalities characteristic of lesions of the midline structures of the cerebellum were documented in two patients. A review of reported cases of Fisher's syndrome and brain-stem encephalitis suggested that these syndromes comprise a spectrum of a reversible, parainfectious process affecting the peripheral cranial nerves and CNS.

Eye movements were studied quantitatively using electro-oculography in seven patients with rod monochromacy. Attention was given to eye movement abnormalities that could help to differentiate rod monochromacy from other forms of congenital nystagmus. Horizontal nystagmus in center gaze had pendular and jerk waveforms with much lower amplitude than that in patients with other forms of congenital nystagmus and visual acuity of 20/200-20/400. Slow build-up of slow component velocity over many seconds and directional asymmetry of optokinetic nystagmus (OKN) were observed during monocular optokinetic stimulation. The directional asymmetry of OKN was characterized by a higher OKN gain (eye velocity/OKN drum velocity) during rotation of the OKN drum in the temporal-to-nasal direction in the visual field, than that during drum rotation in the nasal-to temporal direction. Similar directional asymmetry and slow build-up are found in afoveate animals, such as the rabbit, during monocular optokinetic stimulation, but are not found in normal human subjects or in patients with other forms of congenital nystagmus.

Quantitative electro-oculographic recording techniques were used to analyze four characteristic eye movement abnormalities in 21 patients with internuclear ophthalmoplegia (INO). The frequency of each of the abnormalities was determined to suggest a pattern that is the most sensitive in detecting the syndrome of INO. Slowing of the adducting saccade was the most frequently found abnormality, being present in all patients. The other characteristic eye movement disorders were found less frequently: dissociated nystagmus at 30 degrees of eccentric gaze, dysmetria of the abducting eye, and limitation of adduction. The most sensitive pattern for detecting an INO seems to be slowing of the adducting saccade combined with either dissociated nystagmus or dysmetria of the abducting eye. Limitation of adduction was seen much less frequently.

We report the oculographic findings in a family whose members have a dominantly inherited, early onset, non-progressive syndrome which includes spontaneous upbeating nystagmus and mild cerebellar ataxia associated with cerebellar vermian atrophy seen on magnetic resonance scanning. Eye movements recorded with electro-oculography and a magnetic scleral search coil revealed severely impaired horizontal and vertical smooth pursuit, optokinetic nystagmus and visual-vestibular interaction, symmetrical horizontal but asymmetrical vertical vestibulo-ocular reflex, and normal saccades. The midline cerebellum appears to be essential for both horizontal and vertical visual tracking and visual modification of the vestibulo-ocular reflex in man.

Clinical examinations and eye movement recordings of 91 consecutive patients with DBN were analyzed to describe the characteristics of DBN and to localize the lesions producing this abnormality. Horizontal and vertical eye movement recordings were made with EOG and/or magnetic search coil. The most frequent causes were infarction, cerebellar and spinocerebellar degeneration syndromes, MS and developmental anomalies affecting the pons and cerebellum. Toxicity from anticonvulsant drugs probably caused nystagmus in a few patients. Clinical examinations, excluding electronic eye movement recordings, were used to localize lesions. Localizations included the cerebellum in 88% of the patients. However, localizations to structures outside of the cerebellum were made in several patients. The effects of DBN of gaze position, convergence, blockage of fixation, and positioning of the head and body were observed. Almost all patients had DBN in some position of gaze while sitting and fixating a distant target. A few patients demonstrated DBN only with convergence, in the dark, or with positioning of the head and body. Horizontal gaze increased DBN in most patients. The nystagmus slow components usually had constant-velocity or increasing-velocity waveforms. The effects of vertical gaze on DBN were variable. In general, statistically significant differences in the frequencies of these effects among the various causes and localizations of lesions were not found. Horizontal eye movements were electronically recorded in DBN patients, in a group of normal subjects, and in a group of patients with isolated cerebellar atrophy who did not have DBN. The pattern of abnormal horizontal eye movements characteristic of damage to the midline structures of the cerebellum (impaired pursuit, impaired OKN, and inability to suppress VOR) was found in almost all DBN patients (99%), including patients with lesions localized to structures outside the cerebellum by clinical examination. DBN is usually produced by lesions in the cerebellum that also damage pathways that control horizontal tracking and visual-vestibulo-ocular interactions.

The results of experiments are evaluated in terms of a simple model for the interaction of eye movement responses to simultaneous optokinetic and vestibular stimuli. The model predictions agree with the results of these experiments and explain many clinical observations concerning the effect of vision on nystagmus. The model accounts for the dominance of the visual system's response over the vestibular system's response at low frequencies. It also accounts for the inability of patients with decreased smooth pursuit system response to suppress the vestibulo-ocular reflex during simultaneous optokinetic and vestibular stimulations. The model provides useful information for the design of combined optokinetic and vestibular stimuli for test vestibulo-ocular reflexes.

We found a consistent pattern of response to low and high frequency sinusoidal rotational testing in patients with chronic compensated unilateral and bilateral peripheral vestibular lesions. Gain (peak slow phase eye velocity/peak chair velocity) was decreased and phase lead increased at low frequencies but both measurements approached normal at high frequencies. Asymmetries in gain, when present, were approximately the same in the low and high frequency range. These changes can be explained by a simple first order linear model of the vestibulo-ocular reflex if one assumes that the goal of the compensation process is to maintain gain in the high frequency range.

1. Horizontal and vertical eye movements were induced in normal human subjects by sinusoidal linear acceleration on a parallel swing. The swing frequency was 0.3 Hz and the peak horizontal and vertical acceleration ranged from 0.17 to 0.48 and 0.03 to 0.34 g, respectively. Eye movements were recorded with the scleral search coil technique. 2. With the subjects seated in the dark to stimulate the otolith-ocular reflex, swing displacement along the interaural axis induced horizontal eye movements with a mean sensitivity to translation (ST) (peak eye velocity/peak swing velocity) of 3.8 to 4.7 degrees/m and a mean phase shift (eye velocity re swing velocity) of -152 to -160 degrees. Vertical eye movements had ST and phase values comparable to those of the horizontal eye movements. When the subjects sat facing forward so that the horizontal linear accelerations occurred in the occipitonasal axis, almost identical vertical but no consistent horizontal eye movements were induced. In each case the horizontal and vertical eye movements were proportional to the horizontal and vertical displacement of the swing. 3. With the subject seated in the light looking to an earth-fixed target (synergistic visual-vestibular interaction), the gain (peak eye velocity/peak target velocity) of induced eye movements was near 1, and the phase was compensatory (i.e., approximately -180 degrees) for all stimuli (even at target velocities at which the pursuit gain was less than 1). Subjects were able to suppress the otolith-ocular responses by fixating on a target attached to the swing. The ST decreased by an order of magnitude compared with measurements in the dark without a fixation target. 4. Subjects were able to augment the ST (horizontal and vertical) by imagining an earth-fixed target. Halving the distance of the imagined target approximately doubled the ST. 5. In two of three subjects tested, the ST measured with mental alerting in the dark adaptively increased (approximately doubled) after 20 min of continuous synergistic visual-vestibular interaction. The subject who did not show an adaptive increase in ST began with the highest value of the 10 normal subjects. 6. We conclude that during linear accelerations of the head the otolith signal is correctly interpreted as head movement and not rotation of the gravity vector. The otolith-ocular reflex interacts with the visual pursuit system to improve ocular stability during translational head movements.

Two mechanisms by which patients compensate for congenital nystagmus are assumption of a head position that places the eyes in a "null zone" of least nystagmus, known as nystagmus compensation syndrome (NCS), and convergence to dampen nystagmus, known as nystagmus blockage syndrome (NBS). We followed five patients in two groups who combine these mechanisms. The first group presented with spontaneous alternation between nystagmus and a head turn with orthotropia (NCS), and a large-angle esotropia with a variable head position that persisted even when patched (NBS). The second group of patients presented with a constant large-angle esotropia that lessened their nystagmus and variable head posture (NBS). Following recession of both medial rectus muscles with or without placement of posterior fixation sutures, they developed an abnormal head posture with orthotropia (NCS). The presence of mild amblyopia may contribute to the alternation between the two compensatory mechanisms.

Saccadic eye movements were measured in ten patients with unilateral limitation of function of an extraocular muscle. With the paretic eye fixating, there were a series of saccades when refixating in the direction opposite to the paralysis. Between each saccade, the eye drifted back toward the starting position; the drift rate decreased as the eye moved into the field of action of the strong agonist muscle. In one patient, the normal (covered) eye was immobilized during attempted refixations. In this case, the abnormal pattern of saccades made by the paretic eye persisted. Thus, the central nervous system uses innervation or efference to calculate the position of the eye in the orbit for saccadic eye movements.

Saccades (horizontal and vertical) and dissociated nystagmus were quantitatively assessed in four patients with internuclear ophthalmoplegia. Two patients had bilateral medial longitudinal fasciculus (MLF) lesions associated with multiple sclerosis and two had unilateral lesions associated with brain stem vascular disease. Adducting saccades made on the side of an MLF lesion were slowed in each patient (P less than .01). At the same time, abducting saccades in the contralateral eye had normal velocity, but consistently overshot the target. After the overshoot, the eye returned to the target with an exponentially decaying course. With large angular deviations (usually more than 15 degrees), the abducting eye developed nystagmus characterized by slow components that moved toward the midposition with an initially high velocity followed by a segment of slower velocity. The adducting eye had either no nystagmus or a low-amplitude nystagmus characterized by a rounded junction between the fast and slow component. The saccade abnormalities and waveform of the dissociated nystagmus can be explained by a pulse-step mismatch at the agonist motoneurons.

Rapid eye movements, having high velocity and low amplitude, are described in 11 patients with myasthenia gravis. These movements occur with various degrees of ophthalmoplegia. To distinguish them from the somewhat similar lid-twitch phenomenon, they are called quiver movements. We believed that their presence is pathognomonic of myasthenia and results from a differential involvement of the two myoneural mechanisms that are peculiar to the extraocular muscles.

Experiments were conducted to quantify the effect of a vestibular stimulation of known magnitude on a constant optokinetic nystagmus (OKN). Ten normal human subjects were tested with varying magnitudes of vestibular stimuli that were superimposed on a constant 30 degrees optokinetic stimulus. The gain of the vestibular system in the dark was 0.42 +/- 0.11, and the gain in the light during superimposition testing was 0.12 +/- 0.02. From these results, predictions were made that the degree of vestibular imbalance necessary to produce an asymmetric OKN would generate a spontaneous nystagmus in the dark, which would be equivalent to 20 to 30 degrees. Data from a large group of patients were used for corroboration of the results.

A severe defect of optokinetic nystagmus (OKN) was found in 46 patients with congenital nystagmus. Abnormal patterns of OKN, such as superimposition of pendular oscillations on the optokinetic slow component and inversion of OKN, were observed. Optokinetic gain (eye movement velocity/drum velocity) was decreased compared to that in normal subjects, and optokinetic after-nystagmus, or transient persistence of OKN after cessation of visual stimulation, was absent. These findings suggest that there is a defect in the subcortical optokinetic system in congenital nystagmus. Optokinetic responses did not clearly differentiate patients who had ocular lesions that impair vision, such as ocular albinism and opacities of the ocular media, from patients who did not have such lesions. Similar abnormal patterns of OKN were found in both groups of patients, although optokinetic gain tended to be lower in patients with ocular lesions than in those without lesions.