horizontal pendular nystagmus
1 min readThere is always associated strabismus and ocular motor recordings show four types of slow phases with jerk in direction of fixing eye. Anesthesia affects the ERG varying with type and depth of anesthesia. Clearly, falling is neither the direct cause nor the specific cause of bone fracture; the direct cause in all of these cases and in others (eg, being struck by a hard object) is deformation of the bone beyond its limit of elasticity. It presents at birth or early infancy and is clinically characterized by involuntary oscillations of the eyes. Cranial nerve VI contains somatic motor fibers only that supply the lateral rectus muscle of the eye. 4, solid), low (Fig. Most external retinal layers, specifically the external limiting membrane, photoreceptor inner segment layer, and photoreceptor outer segment layer, appear normal, in agreement with histopathological studies and less detailed TD-OCT reported findings of either absent or rudimentary foveal pits in oculocutaneous albinism. 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Individuals with NBS use a convergence-like movement to damptheir INS while viewing a distant target. Abadi RV. Congenital, latent and manifest latent nystagmus - similarities, differences and relation to strabismus. Contrast sensitivity, color vision, time-dependent acuity, and visual field testing may also be helpful. Pre-therapy NAFX curves of equal LFD values and post-therapy VA curves for conditions b) and c) are shown in grey. Torsional direction is most unambiguously described by the ear toward which the top or upper pole of the eye (the 12 o'clock position) rotates (eg, top pole of the eye beating toward the right ear). Surgically shifting the point of maximum foveation quality, CEMAS_Working_Group. The cerebellum plays an important role in eye movements. One in every 5,000 to 10,000 people suffers from Nystagmus, a relatively common clinical condition. Nystagmus basics. 1967;30(5):383392. Thus, the NBS encompasses two different types of infantile nystagmus and the ability to willfully change the amount of esotropia present to improve the nystagmus waveform and thereby, visual acuity. In the NBS, it is possible that the bimedial recession procedure could be therapeutically beneficial becausethe purposive esotropia is different from ordinary strabismus; the latter is not under conscious control. The eyes may shake more when looking in certain directions. Being "slow to see" is a dynamic visual function consequence of infantile nystagmus syndrome: Model predictions and patient data identify stimulus timing as its cause. Table 1. Measured VA vs. Gaze Angle plots for patients with mid-range VApk NAFXpk in or near primary position, high HAgar = LFD, including: VAf = VAn, strabismus or VAf < VAn, no strabismus. Also, based on research data, it is possible to use the curves shown in Figure 8 to estimate preoperatively the postoperative improvements in both NAFXpk (and VApk) and the LFD (and HAgar). Attempts to do either could result in broken fusion, diplopia, and loss of stereopsis. When considering possible treatments for INS, it is important to keep several things in mind: 1) INS in isolation is not a very debilitating condition (it does not prevent stereopsis nor greatly diminish peak acuity); 2) all of the major ocular motor functions that are necessary for good dynamic visual function are intact and functioning normally (gaze-holding range, saccades to locate targets, pursuit to track moving targets, vergence to fuse near targets, and the VOR and OKR to stabilize gaze in the presence of body or environmental motion); 3) the cosmetic effects of INS are usually minimal, often go unnoticed, and should not be the major factor in determining therapy (despite possibly being the primary concern of a parent; the child with INS is the patient, not the parent). There are also several methods of stimulating the eye. The disease is caused by damage to more than one component of the vestibular system. They include: several types of vestibular nystagmus; gaze-holding nystagmus; visual loss nystagmus (chiasmal and pre- and post-chiasmal); pendular nystagmus associated with central myelin disease (tremor, myoclonus, and pendular vergence nystagmus); convergence and convergence-evoked nystagmus; upbeat and downbeat nystagmus; torsional and see-saw nystagmus; and lid nystagmus. That is, there is no nystagmus when both eyes are viewing, but when one eye is occluded, jerk nystagmus develops in both eyes, with the fast phases toward the uncovered eye. The nystagmus is generally bilateral (but can differ in each eye and may even be strictly monocular), and it oscillates in horizontal, torsional, or vertical directions. The results of pattern-reversal stimuli are less variable in waveform and timing than the results elicited by other stimuli. It and the contents of most early medical texts on infantile nystagmus were based solely on those clinical impressions, unwarranted presumptions, and contradictions to the laws of physics. Quantitative characteristics of the. 5) and high curve for NAFX (Fig. Nystagmus is characterized by numerous phenomenological attributes, many of which are linked to specific underlying pathologic mechanisms. Lemos J, Eggenberger E. Saccadic intrusions: review and update. Similar to INS, there are both sensory and motor components. OCT depends on optical ranging; in other words, shining a beam of light onto the object, then recording the echo time delay of light measure distances. On either side of the null, the amplitude grows and, more importantly, foveation worsens. All patients with FMNS have strabismus. Prior to T&R therapy, the identification of individuals to both sides is very difficult. In Type I, the extended foveation periods will increase the NAFX over that measured during binocular fixation. These estimations can then form data-driven foundations for therapeutic decisions to be made by the physician and the patient. It disappears during sleep but may persist when the child is lying down. your express consent. Other clinical characteristics of INS, with variable association, include: INS remains horizontal in up gaze (in contrast to acquired and/or vestibular nystagmus, which changes direction in vertical gaze); INS increases intensity with fixation attempt or stress and decreases with sleep or inattention; INS had variable intensity in different positions of gaze (usually about a null position); INS changes direction in different positions of gaze (about a neutral position); INS has decreased intensity (damps) with convergence; INS exhibits anomalous head posturing; INS is associated with strabismus; and INS is associated with an increased incidence of significant refractive errors. What are the characteristics of physiologic nystagmus? For clarity in the more complex cases of Figures 5 and 6, individual VA measurements were deemphasized in favor of the 2nd-order trend lines for some of the possible conditions. Included in the definition of strabismus, is latent strabismus(ie, the phoria resulting when you cover an eye). Congenital nystagmus onset is typically between 6 weeks and several months of age. Target foveation depends on which type of waveform (INS or FMNS) is present. FitzGibbon EJ, Calvert PC, Dieterich M, Brandt T, Zee DS. This consists of the frontal eye fields, superior colliculus, brainstem nuclei, vestibular nuclei, and cerebellum. Conversely, rare patients with infantile esotropia display horizontal or vertical head oscillations that resolve following surgical realignment of the eyes. Dell'Osso LF. Jacobs JB, Dell'Osso LF, Wang ZI, Acland GM, Bennett J. Acquired pendular nystagmus (APN) and pathological gaze-evoked nystagmus usually also have a brainstem or cerebellar localization . 44. Thus, the NBS remains both a poorly understood and an over-diagnosed phenomenon related to INS. Figure 1 illustrates a clinical algorithm that could lead to a putative diagnosis of the type of nystagmus present in a patient. Thus, proprioceptive control of static muscle tension could affect the small-signal (linear, push-pull motor signals) gain of the EOM, and reducing that gain could damp an ongoing nystagmusthat is precisely the hypothetical action by which EOM surgery damps INS. Kim JS, Moon SY, Choi KD, Kim JH, Sharpe JA. This Special Interest article represents a summary of that work, with the goal of providing physical therapists with the necessary information to appropriately examine and classify nystagmus to guide subsequent evaluation and management. A muscle pulley system maintains the stability of the muscle paths. It is inconstant and irregular and can be horizontal or vertical, or both. In Type II, because of the Alexanders law variation of FMN, a large head turn is common. If an adult falls on a concrete sidewalk and fractures his arm, it is equally irresistible and false to attribute the cause of either bone fracture in general or, in that specific case, to the fall. It is significant that these associated deficits are unrelated to each other, occur in different planes (or in no plane), and are mechanistically unrelated to horizontal nystagmus. Applying this software to the extreme case of eye motion, nystagmus, and incorporating it into a technology that is already available to clinicians would be an exciting and powerful application of the SD-OCT. Because the nystagmus population can also fall victim to common ocular diseases, such as diabetes, glaucoma, and macular degeneration, the use of SD-OCT, especially with software that may reduce eye motion and help recover 3-dimensional spatial integrity, would be an important diagnostic and management tool. Kalyanaraman K, Jagannathan K, Ramanujam RA, Ramamurthi B. Congenital head nodding and nystagmus with cerebrocerebellar degeneration. Patients with NBS also apply these same therapeutic maneuvers. Neurophysiologic and clinical correlations of epileptic, 31. Therefore, 2 types of information, motion vs. color and form, are kept in separate layers in the LGN. Benign positional. Distinguishing the FMNS waveform and the tropia of the non-fixating eyerequires DC-coupled, high-bandwidth recordings of both eyes simultaneously. In the case of complete nystagmus blockage, the accuracy is essentially the same as in unaffected individuals. The latter arewaveforms where a low-amplitude, high-frequency pendular nystagmus is superimposed on either a decreasing-velocityslow phase jerk waveform (dual jerk) or a slow pendular waveform (dual pendular). Cranial nerve IV contains somatic motor fibers only. Effects of extraocular muscle surgery on 15 patients with oculo-cutaneous albinism (OCA) and infantile nystagmus syndrome (INS). Thus, although the percent improvement in VApk is directly related to the pre-therapy NAFXpk, the actual magnitude of post-therapy VApk will be limited by the magnitude of the sensory deficit. Here and in Figure 6, h = high NAFXpk (>0.6), m = mid-range NAFXpk (0.25 NAFXpk 0.6), and l = low NAFXpk (<0.25). Kim JS, Ahn KW, Moon SY, Choi KD, Park SH, Koo JW. Three major stimuli elicit vergences: (1) retinal disparity that leads to fusional vergence; (2) retinal blur that evokes accommodative vergence; and (3) motion that induces both disparity and accommodative vergence. Various theories have been advanced to explain FMNS. The failure in calibration of this damping has been hypothesized as the primary (direct) cause of all major, complex INS waveforms (the other being an imbalance in the visuo-vestibular system that produces linear slow-phase waveforms). Pendular nystagmus has only slow phases. modify the keyword list to augment your search. MRI brains of such patients demonstrate very striking features that can easily lead to the diagnosis in combination with a thorough clinical history and physical examination. 2010;6(9):519523. 43. 70. Unfortunately, they cannot be identified by clinical observation; eye-movement data are required. Pathologic nystagmus may be spontaneous, gaze-evoked, or triggered by provocative maneuvers. The slow vergence system is elicited by small disparity errors and/or disparity velocities of less than 3/sec. A unifying model-based hypothesis for the diverse waveforms of infantile nystagmus syndrome. Neurology. There is now general agreement that head nodding in SNS is compensatory. 2011;77(21):1929. The vestibular apparatus drives reflex eye movements, which keep images of the world steady on the retinas during head or body motion. Based on our research, the NBS can now be more accurately defined as a syndrome in which a patient with INS plus a variable esotropia willfully deviates the fixating eye into adduction to accomplish either a damping of the IN or a switch from IN to a low-amplitude FMN.1, The first characteristic revealed by eye-movement data was that there were two mechanisms by which blockage of the ongoing nystagmus can be accomplished; that resulted in two different types of patients with the NBS.39; 40 Patients with both types had IN when their eyes were aligned. Pattern contrast thresholds in latent nystagmus. Gaze-angle variations usually result in a single, static null position where the waveforms yield maximal foveation quality. to maximize the individual patients overall visual function. Neurology. Some error has occurred while processing your request. involuntary, rapid, rhythmic movement (horizontal, vertical, rotatory, or mixed, i.e., of two types) of the eyeball. Neurology. Dell'Osso LF. When evaluating an infant or child with INS for the first time, historical points suggest afferent visual pathway dysfunction. Disconjugate may be dissociated if the velocity or amplitude of the movements is different in the 2 eyes or disjunctive if the 2 eyes simultaneously rotate in different directions. Since they may vary independently, both are necessary factors in a function that is related to potential visual acuity and functions that do not contain both will not accurately reflect acuity in all patients. For other combinations, the probability of correctly diagnosing and assessing therapeutic success in INS is low. The effects of increased visual task demand on foveation in congenital nystagmus. Gradstein L, Goldstein HP, Wizov SS, Hayashi T, Reinecke RD. [from HPO] Term Hierarchy GTR MeSH CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar CROGVHorizontal pendular nystagmus Am J Otol. The Ganzfeld allows the best control of background illumination and stimulus flash intensity. Patterns of spontaneous and head-shaking, 55. Dell'Osso LF. Note that when the peak is high and the range of high-visual acuity (Hi VA) gaze angles is broad, their values cannot be significantly increased and, therefore, no waveform foveation improvements are possible; only under these simultaneous conditions is nystagmus therapy precluded. Leech J, Gresty M, Hess K, Rudge P. Gaze failure, drifting. It is particularly important to obtain replicate responses from children to assure that the response measured is a reliable signal and not an artifact. The earliest detectable response has a peak latency of approximately 30 ms post-stimulus and components are recordable with peak latencies of up to 300 ms. Such radical therapies sacrifice both visual and ocular motor function (eg, accurate saccades to targets, binocular alignment for stereopsis, and both the optokinetic and vestibulo-ocular reflexes necessary to maintain stable vision in the presence of either environmental or body motion). These involuntary eye movements can occur from side to side, up and down, or in a circular pattern. Figure 2. Robinson DA, Zee DS, Hain TC, Holmes A, Rosenberg LF. In fact, once monocular refraction is determined and monocular acuity measured, the prisms and -1.00S should be added. Brain Cogn. Several attributes are used to describe nystagmus: binocularity, conjugacy, velocity, waveform, frequency, amplitude, intensity, temporal profile, and age at first appearance. Bertholon P, Tringali S, Faye MB, Antoine JC, Martin C. Prospective study of positional. End-point, 9. As for adults, additional channels of recording may be important for diagnosis of chiasmal and post-chiasmal dysfunction. Indeed, it can even present as spasmus nutans (see Section 4).28. Each pair operates in a push-pull manner to rotate the eye in their plane of action. Nystagmus is a rhythmic regular oscillation of the eyes. It may be different in the two eyes, sometimes even monocular. Horizontal Vertical Torsional Elliptical (i.e. Congenital Nystagmus "CN" is an oscillation of the eyes across the line of regard (target); There are 2 types of "CN," "sensory" and "motor;" "Sensory CN" is caused by a visual sensory deficit and has a pendular waveform; "Motor (aka "idiopathic") CN" has no known cause and a jerk waveform; There are no treatments for "CN;" and Although the nystagmus may clinically resemble that recorded in SNS, until a proper study comparing the actual waveforms of SNS with those recorded in children with known neurological disease, they should not be presumed to be identical. Bertholon P, Bronstein AM, Davies RA, Rudge P, Thilo KV. The combination of attributes allows differentiation between the many peripheral and central forms. In contradistinction to INS, visual acuity is minimally affected in SNS. Recordings are required to document the decelerating or linear slow-phase waveforms characteristic of FMNS from the accelerating slow phases predominant in the INS. Solid = pre-therapy curve, and dashed and dot-dashed = post-therapy T&R and BMR/BOPr curves respectively, Because Figures 5 and 6 include different possible combinations of INS characteristics, they are more complex than the simple plot from an individual patient, which, like Figure 4, would only contain that patients personal pre- and post-therapy acuities. The medical and surgical therapies applied to IN and FMN utilize the respective characteristics of the nystagmus. SNS appears as a high-frequency, asymmetric, disjugate ocular oscillation. The ERG should be recorded using single, scotopic white flashes. Jerk-waveform see-saw, 29. Characteristics of braking saccades in congenital nystagmus. The, more common, manifest form of FMN is present with both eyes open and mimics the latent form exactly if it isbidirectional. The same is not true for nystagmus types associated with neurological disease. Individuals with FMNS often fixate stationary targets with their adducting eye; this strategy is also used during smooth pursuit. SNS may appear at or after birth and usually, but notnecessarily, ceases clinically (diminishes) by the age of three. Eye-muscle proprioceptive signals provide information used in normal sensorimotor functions; these include various aspects of perception, and of the control of eye movement. Pre-therapy NAFX curves of equal LFD values and post-therapy VA curves for conditions b) and c) are shown in grey. 33. Gresty MA, Bronstein AM, Page NG, Rudge P. Congenital-type nystagmus emerging in later life. Gottlob I, Wjizov SS, Reinecke RD. Results in 10 adults. The VEP can provide important diagnostic information regarding the functional integrity of the visual system. For the flash VEP, the most robust components are the N2 and P2 peaks. Therefore, different therapies and adaptations by the patient can act in distinct mechanistic ways to damp the FMN and, in some cases, restore fusion. The cerebellum in eye movement control: 5. The fact that INS "disobeys" Alexander's law under binocular conditions (which states that, in peripheral vestibular nystagmus, the direction of the nystagmus increases in the direction of the fast phase and decreases but never reverses in the direction of the slow phase) is often useful in distinguishing it from horizontal peripheral vestibular nystagmus.
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