Nystagmus: Diagnosis, Topographic Anatomical Localization and Therapy

2021 ◽  
Vol 238 (11) ◽  
pp. 1186-1195
Author(s):  
Michael Leo Strupp ◽  
Dominik Straumann ◽  
Christoph Helmchen

AbstractNystagmus is defined as rhythmic, most often involuntary eye movements. It normally consists of a slow (pathological) drift of the eyes, followed by a fast central compensatory movement back to the primary position (refixation saccade). The direction, however, is reported according to the fast phase. The cardinal symptoms are, on the one hand, blurred vision, jumping images (oscillopsia), reduced visual acuity and, sometimes, double vision; many of these symptoms depend on the eye position. On the other hand, depending on the etiology, patients may suffer from the following symptoms: 1. permanent dizziness, postural imbalance, and gait disorder (typical of downbeat and upbeat nystagmus); 2. if the onset of symptoms is acute, the patient may experience spinning vertigo with a tendency to fall to one side (due to ischemia in the area of the brainstem or cerebellum with central fixation nystagmus or as acute unilateral vestibulopathy with spontaneous peripheral vestibular nystagmus); or 3. positional vertigo. There are two major categories: the first is spontaneous nystagmus, i.e., nystagmus which occurs in the primary position as upbeat or downbeat nystagmus; and the second includes various types of nystagmus which are induced or modified by certain factors. Examples are gaze-evoked nystagmus, head-shaking nystagmus, positional nystagmus, and hyperventilation-induced nystagmus. In addition, there are disorders similar to nystagmus, such as ocular flutter or opsoclonus. The most common central types of spontaneous nystagmus are downbeat and upbeat, infantile, pure torsional, pendular fixation, periodic alternating, and seesaw nystagmus. Many types of nystagmus allow a precise neuroanatomical localization: for instance, downbeat nystagmus, which is most often caused by a bilateral floccular lesion or dysfunction, or upbeat nystagmus, which is caused by a lesion in the midbrain or medulla. Examples of drug treatment are the use of 4-aminopyridine for downbeat and upbeat nystagmus, memantine or gabapentin for pendular fixation nystagmus, or baclofen for periodic alternating nystagmus. In this article we are focusing on nystagmus. In a second article we will focus on central ocular motor disorders, such as saccade or gaze palsy, internuclear ophthalmoplegia, and gaze-holding deficits. Therefore, these types of eye movements will not be described here in detail.

2021 ◽  
Vol 238 (11) ◽  
pp. 1197-1211
Author(s):  
Michael Leo Strupp ◽  
Dominik Straumann ◽  
Christoph Helmchen

AbstractThe key to the diagnosis of ocular motor disorders is a systematic clinical examination of the different types of eye movements, including eye position, spontaneous nystagmus, range of eye movements, smooth pursuit, saccades, gaze-holding function, vergence, optokinetic nystagmus, as well as testing of the function of the vestibulo-ocular reflex (VOR) and visual fixation suppression of the VOR. This is like a window which allows you to look into the brain stem and cerebellum even if imaging is normal. Relevant anatomical structures are the midbrain, pons, medulla, cerebellum and rarely the cortex. There is a simple clinical rule: vertical and torsional eye movements are generated in the midbrain, horizontal eye movements in the pons. For example, isolated dysfunction of vertical eye movements is due to a midbrain lesion affecting the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), with impaired vertical saccades only or vertical gaze-evoked nystagmus due to dysfunction of the Interstitial nucleus of Cajal (INC). Lesions of the lateral medulla oblongata (Wallenberg syndrome) lead to typical findings: ocular tilt reaction, central fixation nystagmus and dysmetric saccades. The cerebellum is relevant for almost all types of eye movements; typical pathological findings are saccadic smooth pursuit, gaze-evoked nystagmus or dysmetric saccades. The time course of the development of symptoms and signs is important for the diagnosis of underlying diseases: acute: most likely stroke; subacute: inflammatory diseases, metabolic diseases like thiamine deficiencies; chronic progressive: inherited diseases like Niemann-Pick type C with typically initially vertical and then horizontal saccade palsy or degenerative diseases like progressive supranuclear palsy. Treatment depends on the underlying disease. In this article, we deal with central ocular motor disorders. In a second article, we focus on clinically relevant types of nystagmus such as downbeat, upbeat, fixation pendular, gaze-evoked, infantile or periodic alternating nystagmus. Therefore, these types of nystagmus will not be described here in detail.


2020 ◽  
Vol 267 (10) ◽  
pp. 2865-2870
Author(s):  
Torstein R. Meling ◽  
Aria Nouri ◽  
Adrien May ◽  
Nils Guinand ◽  
Maria Isabel Vargas ◽  
...  

Abstract Introduction CNS cavernomas are a type of raspberry-shaped vascular malformations that are typically asymptomatic, but can result in haemorrhage, neurological injury, and seizures. Here, we present a rare case of a brainstem cavernoma that was surgically resected whereafter an upbeat nystagmus presented postoperatively. Case report A 42-year old man presented with sudden-onset nausea, vomiting, vertigo, blurred vision, marked imbalance and difficulty swallowing. Neurological evaluation showed bilateral ataxia, generalized hyperreflexia with left-sided predominance, predominantly horizontal gaze evoked nystagmus on right and left gaze, slight left labial asymmetry, uvula deviation to the right, and tongue deviation to the left. MRI demonstrated a 13-mm cavernoma with haemorrhage and oedema in the medulla oblongata. Surgery was performed via a minimal-invasive, midline approach. Complete excision was confirmed on postoperative MRI. The patient recovered well and became almost neurologically intact. However, he complained of mainly vertical oscillopsia. The videonystagmography revealed a new-onset spontaneous upbeat nystagmus in all gaze directions, not suppressed by fixation. An injury of the rarely described intercalatus nucleus/nucleus of Roller is thought to be the cause. Conclusion Upbeat nystagmus can be related to several lesions of the brainstem, including the medial longitudinal fasciculus, the pons, and the dorsal medulla. To our knowledge, this is the first case of an iatrogenic lesion of the nucleus intercalatus/nucleus of Roller resulting in an upbeat vertical nystagmus. For neurologists, it is important to be aware of the function of this nucleus for assessment of clinical manifestations due to lesions within this region.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Talora L. Martin ◽  
Jordan Murray ◽  
Kiran Garg ◽  
Charles Gallagher ◽  
Aasef G. Shaikh ◽  
...  

AbstractWe evaluated the effects of strabismus repair on fixational eye movements (FEMs) and stereopsis recovery in patients with fusion maldevelopment nystagmus (FMN) and patients without nystagmus. Twenty-one patients with strabismus, twelve with FMN and nine without nystagmus, were tested before and after strabismus repair. Eye-movements were recorded during a gaze-holding task under monocular viewing conditions. Fast (fixational saccades and quick phases of nystagmus) and slow (inter-saccadic drifts and slow phases of nystagmus) FEMs and bivariate contour ellipse area (BCEA) were analyzed in the viewing and non-viewing eye. Strabismus repair improved the angle of strabismus in subjects with and without FMN, however patients without nystagmus were more likely to have improvement in stereoacuity. The fixational saccade amplitudes and intersaccadic drift velocities in both eyes decreased after strabismus repair in subjects without nystagmus. The slow phase velocities were higher in patients with FMN compared to inter-saccadic drifts in patients without nystagmus. There was no change in the BCEA after surgery in either group. In patients without nystagmus, the improvement of the binocular function (stereopsis), as well as decreased fixational saccade amplitude and intersaccadic drift velocity, could be due, at least partially, to central adaptive mechanisms rendered possible by surgical realignment of the eyes. The absence of improvement in patients with FMN post strabismus repair likely suggests the lack of such adaptive mechanisms in patients with early onset infantile strabismus. Assessment of fixation eye movement characteristics can be a useful tool to predict functional improvement post strabismus repair.


1991 ◽  
Vol 1991 (Supplement48) ◽  
pp. 128-132
Author(s):  
Atsushi Sakuma ◽  
Isao Kato ◽  
Sadao Ogino ◽  
Tomoyuki Okada ◽  
Isamu Takeyama

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Akihide Ichimura ◽  
Shigeto Itani

Here, we report a patient with persistent positional upbeat nystagmus in a straight supine position with no evident abnormal central nervous system findings. A 43-year-old woman with rotatory positional vertigo and nausea visited our clinic 7 days after the onset. Initially, we observed persistent upbeat nystagmus in straight supine position with a latency of 2 s during the supine head roll test. However, an upbeat nystagmus disappeared on turning from straight to the left ear-down supine position, and while turning from the left to right ear-down position, an induced slight torsional nystagmus towards the right for >22 s was observed. In the Dix–Hallpike test, the left head-hanging position provoked torsional nystagmus towards the right for 50 s. In prone seated position, downbeat nystagmus with torsional component towards the left was observed for 45 s. Neurological examination and brain computed tomography revealed no abnormal findings. We speculated that persistent positional upbeat nystagmus in this patient was the result of canalolithiasis of benign paroxysmal positional vertigo of bilateral posterior semicircular canals.


Author(s):  
Koji Harada ◽  
Isao Kato ◽  
Yoshio Koike ◽  
Yo Kimura ◽  
Tadashi Nakamura ◽  
...  

eLife ◽  
2016 ◽  
Vol 5 ◽  
Author(s):  
Tao Yao ◽  
Madhura Ketkar ◽  
Stefan Treue ◽  
B Suresh Krishna

Maintaining attention at a task-relevant spatial location while making eye-movements necessitates a rapid, saccade-synchronized shift of attentional modulation from the neuronal population representing the task-relevant location before the saccade to the one representing it after the saccade. Currently, the precise time at which spatial attention becomes fully allocated to the task-relevant location after the saccade remains unclear. Using a fine-grained temporal analysis of human peri-saccadic detection performance in an attention task, we show that spatial attention is fully available at the task-relevant location within 30 milliseconds after the saccade. Subjects tracked the attentional target veridically throughout our task: i.e. they almost never responded to non-target stimuli. Spatial attention and saccadic processing therefore co-ordinate well to ensure that relevant locations are attentionally enhanced soon after the beginning of each eye fixation.


Perception ◽  
2019 ◽  
Vol 48 (9) ◽  
pp. 850-861 ◽  
Author(s):  
Takashi Mitsuda ◽  
Jiawei Luo ◽  
Qiyan Wang

When people choose between two items, they usually look at them alternately before deciding. The frequency and duration of contact are usually determined unconsciously. However, in a previous study, looking at one item for longer than the other increased participants’ preference for the former, but only when they had to move their eyes to look at each item. This result implies that eye movements not only gather information but are also closely related to decision-making. By analogy, this study examines the relation between hand movements and haptic preference. When participants touched two handkerchiefs in a pre-determined order before choosing the one they preferred, the likelihood of choosing the more frequently touched handkerchief was greater than chance. Bias in the choice was greater with increased difference in the frequency of touching between the two handkerchiefs. It was also greater when participants moved their arm to touch the handkerchiefs, compared with when a machine carried the handkerchiefs to their hand. These results indicate that both the reaching movement for touching and the frequency of touching affect the preference judgment using haptics.


1986 ◽  
Vol 55 (5) ◽  
pp. 1044-1056 ◽  
Author(s):  
H. P. Goldstein ◽  
D. A. Robinson

Two trained monkeys made saccadic eye movements to a small visual target. The activity of 39 isolated abducens units, presumed to be motoneurons or abducens internuclear neurons, was recorded in relation to these eye movements. After a calibration trial, a test trial repeatedly elicited 20 degrees horizontal saccades to primary position from either the left or right. On average, the steady-state firing rate at primary position depended on the direction of the saccade. For saccades where the neuron showed a burst in activity during the saccade (on-saccades) the steady-state firing rates were usually higher than for those saccades that showed a pause in activity during the saccade (off-saccades). For the population of units this hysteresis measured 5.4 spikes/s, which may be compared with an average primary-position rate of 97 spikes/s. The average hysteresis for individual units ranged from -2.1 to 18.5 spikes/s. The steady-state firing rate after equal saccades in the same direction and ending at the same position (primary) varied slowly over time. Across all units the variability (standard deviation) ranged from 0.5 to 11.8 spikes/s with a mean of 4.7 spikes/s. Furthermore, for any one unit the variations following on-saccades generally correlated with the variations following the off-saccades. Hysteresis, doubted by many, does exist. Fortunately, it is small enough, 5.5% of typical primary-position rate, that it can be neglected for many purposes. Nevertheless, it poses the interesting theoretical question of how the oculomotor system compensates for hysteresis. The simplest explanation of slow variations in background rate is cocontractive noise: a slow fluctuation in all abducens neurons so that these variations do not result in fluctuations of eye position.


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