Search

Objectives: To compare acute nystagmus characteristics of posterior circulation stroke (PCS) and acute vestibular neuritis (AVN) in the emergency room (ER) within 24 h of presentation., Methods: ER-based video-nystagmography (VNG) was conducted, recording ictal nystagmus in 101 patients with PCS (on imaging) and 104 patients with AVN, diagnosed on accepted clinical and vestibular test criteria., Results: Patients with stroke in the brainstem (38/101, affecting midbrain (n = 7), pons (n = 19), and medulla (n = 12)), cerebellum (31/101), both (15/101) or other locations (17/101) were recruited. Common PCS territories included posterior-inferior-cerebellar-artery (41/101), pontine perforators (18/101), multiple-territories (17/101) and anterior-inferior-cerebellar-artery (7/101). In PCS, 44/101 patients had no spontaneous nystagmus. Remaining PCS patients had primary position horizontal (44/101), vertical (8/101) and torsional (5/101) nystagmus. Horizontal nystagmus was 50% ipsiversive and 50% contraversive in lateralised PCS. Most PCS patients with horizontal nystagmus (28/44) had unidirectional "peripheral-appearing" nystagmus. 32/101 of PCS patients had gaze-evoked nystagmus. AVN affected the superior, inferior or both divisions of the vestibular nerve in 55/104, 4/104 and 45/104. Most (102/104) had primary position horizontal nystagmus; none had gaze-evoked nystagmus. Two inferior VN patients had contraversive torsional-downbeat nystagmus. Horizontal nystagmus with SPV ≥ 5.8 °/s separated AVN from PCS with sensitivity and specificity of 91.2% and 83.0%. Absent nystagmus, gaze-evoked nystagmus, and vertical-torsional nystagmus were highly specific for PCS (100%, 100% and 98.1%)., Conclusion: Nystagmus is often absent in PCS and always present in AVN. Unidirectional 'peripheral-appearing' horizontal nystagmus can be seen in PCS. ER-based VNG nystagmus assessment could provide useful diagnostic information when separating PCS from AVN., (© 2022. Crown.)

A patient with a postoperative fistula of the left posterior semicircular canal is presented. Negative pressure in the external ear canal produced upbeat-torsional nystagmus, which was recorded in three dimensions using binocular scleral search coils. The nystagmus was conjugate, without skew deviation, and its trajectory corresponded to the anatomic axis of the left posterior canal. The current study helps validate Ewald's first law in humans: the axis of nystagmus should match the anatomic axis of the semicircular canal that generated it. This law is clinically useful in diagnosing pathology of the vestibular end-organ, such as benign paroxysmal positional vertigo or the superior semicircular canal dehiscence syndrome.

Objective: Complete unilateral loss of vestibular function results in a phase advance (reduced time constant) of the horizontal slow-phase nystagmus response to yaw-axis rotation. The objective of this study was to determine whether partial losses of lateral semicircular canal function would result in proportional reductions in the time constant. SETTING AND STUDY DESIGN: This was a retrospective study of consecutive patients' records at two tertiary referral centers for vestibular disorders., Patients: Four hundred fifty-four patients who presented for evaluation of vertigo or imbalance or both and who were found to have partial or complete unilateral canal paresis on caloric testing., Main Outcome Measures: In 372 patients, the gain and time constant of the horizontal nystagmus response was measured using a 5-second velocity ramp of constant yaw-axis acceleration. Caloric responses to standard bithermal irrigations at 30 degrees and 44 degrees were obtained using an open-loop irrigation system. In a second group of 82 patients, the gain and time constant of the horizontal vestibulo-ocular reflex were measured using a sum-of-sines (pseudorandom) yaw-axis acceleration. The caloric response was measured using a closed-loop system., Results: In both groups, the peak gain of the nystagmus response was independent of the level of the canal paresis. However, the time constant of the response both toward and away from the lesioned side decreased proportionally with increasing canal paresis., Conclusion: This result supports the hypothesis that bilateral symmetrical peripheral vestibular input is a necessary condition for the mechanisms or processes underlying normal horizontal slow-phase velocity storage.

Dual search coils were used to record horizontal, vertical and torsional eye movement components of one eye during nystagmus caused by off-center yaw rotation (yaw centrifugation). Both normal healthy human subjects (n=7) and patients with only one functioning labyrinth (n=12) were studied in order to clarify how the concomitant linear acceleration affected the nystagmus response. Each subject was seated with head erect on the arm of a fixed-chair human centrifuge, 1 m away from the center of the rotation, and positioned to be facing along a radius; either towards (facing-in) or away from (facing-out) the center of rotation. Both yaw right and yaw left angular accelerations of 10 degrees s(-2) from 0 to 200 degrees/s were studied. During rotation a centripetal linear acceleration (increasing from 0 to 1.24xg units) was directed along the subject's naso-occipital axis resulting in a shift of the resultant angle of the gravitoinertial acceleration (GIA) of 51 degrees in the subject's pitch plane and an increase in the total GIA magnitude from 1.0 to 1.59xg. In normal subjects during the angular acceleration off-center there were, in addition to the horizontal eye velocity components, torsional and vertical eye velocities present. The magnitude of these additional components, although small, was larger than observed during similar experiments with on-center angular acceleration (Haslwanter et al. 1996), and the change in these components is attributed to the additional effect of the linear acceleration stimulation. In the pitch plane the average size of the shift of the axis of eye velocity (AEV) during the acceleration was about 8 degrees for a 51 degrees shift of the GIA (around 16% of the GIA shift) so that the AEV-GIA alignment was inadequate. There was a very marked difference in the size of the AEV shift depending on whether the person was facing-in [AEV shift forward (i.e. non-compensatory) of about 4 degrees] or facing-out [AEV shift forward (i.e. compensatory) of around 12 degrees]. The linear acceleration decreased the time constant of decay of the horizontal component of the post-rotatory nystagmus: from an average of 24.8 degrees/s facing-in to an average of 11.3 degrees/s facing-out. The linear acceleration dumps torsional eye velocity in an manner analogous to, but independent of, the dumping of horizontal eye velocity. Patients with UVD had dramatically reduced torsional eye velocities for both facing-in and facing-out headings, and there was little if any shift of the AEV in UVD patients. The relatively small effects of linear acceleration on human canal-induced nystagmus found here confirms other recent studies in humans (Fetter et al. 1996) in contrast to evidence from monkeys and emphasizes the large and important differences between humans and monkeys in otolith-canal interaction. Our results confirm the vestibular control of the axis of eye velocity of humans is essentially head-referenced whereas in monkeys that control is essentially space-referenced.

See-saw nystagmus is an uncommon but highly characteristic eye movement disorder comprising intorsion and elevation of one eye, with synchronous extorsion and depression of the other. It generally has a pendular waveform and is due to a midline, extrinsic, suprasellar mass lesion compressing or invading the brainstem bilaterally at the meso-diencephalic junction. This report deals with the clinical and MRI findings in three patients (and binocular three-dimensional quantitative oculographic findings in one patient) with a jerk waveform see-saw nystagmus due in each case to a unilateral meso-diencephalic lesion. In each patient the torsional component of the nystagmus fast phases rotated the upper poles of the eyes toward the side of the lesion. Jerk see-saw nystagmus can be clinically indistinguishable from pendular see-saw nystagmus and from the torsional-vertical nystagmus which occurs with medullary lesions. We propose that jerk see-saw nystagmus is due to unilateral inactivation of the torsional eye-velocity integrator, thought to be in the interstitial nucleus of Cajal, with sparing of the torsional fast-phase generator, thought to be in the adjacent rostral interstitial nucleus of the medial longitudinal fasciculus.

We report 3 patients with autopsy-proven Creutzfeldt-Jakob disease who, early in their course, developed abnormal eye movements that included periodic alternating nystagmus and slow vertical saccades. These findings suggested involvement of the cerebellar nodulus and uvula, and the brainstem reticular formation, respectively. Cerebellar ataxia was also an early manifestation and, in 1 patient, a frontal lobe brain biopsy was normal at a time when ocular motor and cerebellar signs were conspicuous. As the disease progressed, all saccades and quick phases of nystagmus were lost, but periodic alternating gaze deviation persisted. At autopsy, 2 of the 3 patients had pronounced involvement of the cerebellum, especially of the midline structures. Creutzfeldt-Jakob disease should be considered in patients with subacute progressive neurological disease when cognitive changes are overshadowed by ocular motor findings or ataxia.

See-saw nystagmus is a unique torsional-vertical eye movement disorder with a characteristic appearance. It is a pendular nystagmus with two distinct components: a conjugate torsional component and a disjunctive vertical component. In those cases of see-saw nystagmus in which a focal lesion has been identified, the lesion is usually a bilateral, symmetric lesion located at the mesodiencephalic junction. We report an unusual case of see-saw nystagmus which was due to a strictly unilateral mesodiencephalic lesion. Furthermore, the see-saw nystagmus had, in this case, a jerk wave-form rather than the usual pendular wave-form, with the torsional component of the jerk see-saw nystagmus beating toward the side of the lesion. These observations have an impact upon the localizing and lateralizing significance of torsional nystagmus in general and of see-saw nystagmus in particular.

We reviewed the clinical and oculomotor findings in 62 patients with downbeating nystagmus (DBN). Only those patients whose DBN was enhanced in lateral gaze were included. Apart from gait ataxia, few patients had additional neurologic signs. The two most common causes of DBN were cerebellar ectopia (25%) and cerebellar degeneration (25%) with another 10% having a variety of conditions. In about 40% the cause remained undiagnosed. In some patients with idiopathic DBN and in others with DBN due to cerebellar ectopia, the disease progressed slowly, if at all. In DBN the slow-phase velocity is dependent on vertical head position and head velocity in pitch; vertical pursuit, particularly downward pursuit, is defective and vertical vestibulo-ocular reflexes are intact. We concluded that at least some cases of DBN were due to an imbalance in otolithocular reflexes. The lesion causing DBN appears to be in the vestibulocerebellum, perhaps the nodulus, a structure that normally inhibits otolith-ocular reflexes.

We studied reversed optokinetic nystagmus (OKN) in 31 patients and found that it is actually the patient's own gaze-modulated spontaneous nystagmus shifted to the primary position of gaze by optokinetic stimulation. Two of our 31 patients were known to have congenital nystagmus. Most of the 29 others were adults who presented with neurological symptoms and nystagmus and were therefore suspected of having posterior fossa lesions. However, their nystagmographic findings and benign natural history suggest that they also had congenital nystagmus, which was unrecognized until they developed incidental neurological symptoms. These cases support the view that reversed OKN is pathognomonic of a benign, probably congenital, nystagmus.

We examined six patients who developed blurring or oscillopsia as a result of downbeat nystagmus while being treated with lithium carbonate. Of these six plus six previously described similar patients, all but two developed downbeat nystagmus insidiously as an isolated disorder in the setting of otherwise satisfactory therapeutic control, without clinical or biochemical evidence of acute lithium intoxication. Only six of these 12 patients were able either to reduce or to stop taking lithium, and in only two of these six did the downbeat nystagmus improve or remit.

Head shaking and congenital nystagmus were recorded in a patient presented with visual tasks. When she was at rest the nystagmus took a 6 cycles per second saw-tooth wave-form. When she was attentive the nystagmus beat at a 2 to 2.6 cycles per second with a saddle-shaped deformation which permitted foveation. The head shaking occurred occasionally when the patient was attentive and was phase-locked to the nystagmus with resemblances in wave form and direction. Deceleration of the head shaking to zero velocity and peak displacement (to the left) coincided with the onset of the saddle of the nystagmus and hence assisted foveation; all other parts of the head-shaking cycle were detrimental to vision. It is proposed that the head shaking has a common pathological origin with the nystagmus and that, just as an isolated congenital nystagmus wave form becomes altered with attention to permit periods of foveal fixation, the pattern of combined head and eye nodding in this patient provided similar peroids of fixation.

Two patients with longstanding acquired periodic alternating nystagmus (PAN) were treated with baclofen, 30 mg/day. Baclofen abolished the PAN and relieved oscillopsia in both patients but was ineffective in another patient with congenital PAN.