Nystagmus and ataxia associated with antiganglioside antibodies.

PubMed

Jeong, Seong-Hae; Nam, Jungmoo; Kwon, Min Jeong; Kim, Jong Kuk; Kim, Ji Soo

2011-12-01

Antiganglioside antibodies are found in various neurological disorders that constitute a continuum from peripheral neuropathy to encephalitis. However, nystagmus has rarely been described in patients with ataxia associated with antiganglioside antibodies. From January 2008 to July 2009, we identified 3 patients with acute ataxia and nystagmus in 2 University Hospitals of Korea, who were found to have anti-GD1b, anti-GM1, or anti-GQ1b antibodies. In addition to acute ataxia, all 3 patients showed various combinations of nystagmus, which included central positional nystagmus (n = 3), vertical nystagmus (n = 1), and periodic alternating nystagmus (n = 1). The spontaneous and positional nystagmus were mostly detectable only with the elimination of fixation and magnification of the eyes using video goggles. Two patients also exhibited gaze-evoked nystagmus that was noticeable without the aid of video goggles. Patients had serum IgG antibodies to GD1b, GM1, or GQ1b. Cerebrospinal fluid examination, nerve conduction studies, and brain MRI were normal. In all patients, the symptoms and signs resolved over 3-12 months. Various forms of nystagmus with acute ataxia may be a sole or predominant manifestation of disorders related to antiganglioside antibodies. The nystagmus indicates a central pathology involving the cerebellum or brainstem in this antibody-associated disorder. Antiganglioside antibodies should be measured in patients with nystagmus and acute ataxia of undetermined etiology.

Transition from upbeat to downbeat nystagmus observed in a patient with Wernicke's encephalopathy.

PubMed

Suzuki, Yasuo; Matsuda, Taisuke; Washio, Noriaki; Ohtsuka, Kenji

2005-01-01

We report an unusual case of Wernicke's encephalopathy presenting with transient upbeat nystagmus that changed to a persistent downbeat nystagmus. A 27-year-old man presented with upbeat nystagmus. Three months earlier, he had been diagnosed with Wernicke's encephalopathy after fasting for a month. This diagnosis was supported by his symptoms (ataxia, a confused state). Clinical recovery followed thiamine therapy. His upbeat nystagmus had linear slow phases with average amplitude and frequency (+/-SD) during fixation straight ahead of 2.8 +/- 0.7 degrees and 4.6 +/- 2.2 Hz, respectively. Two months later, the primary position upbeat nystagmus had diminished and downbeat nystagmus (0.9 +/- 0.5 degrees and 3.2 +/- 0.7 Hz on average) for a 20 degrees downward gaze had developed. Then, 8 months later, he showed only downbeat nystagmus, which obeyed Alexander's law. His primary position downbeat nystagmus was completely suppressed by clonazepam, a gamma-aminobutyric acid (GABA) agonist. Owing to an underlying central vestibular imbalance, even after the recovery of acute neurological symptoms, Wernicke's encephalopathy can be complicated by persistent downbeat nystagmus, which can be treated by a GABA agonist.

The effect of mental alerting on peripheral vestibular nystagmus during spontaneous, gaze (30 degrees left, 30 degrees right) and body positional (left & right lateral lying) testing using electronystagmography (ENG).

PubMed

McGovern, Tracey N; Fitzgerald, John E

2008-10-01

The performance of mental alerting during caloric testing has always been considered important, however its use/benefit during electronystagmography (ENG)/videonystagmography (VNG) testing has been questioned. The aim of this study was to investigate the effect of mental alerting tasks on peripheral type vestibular nystagmus recorded during ENG. Thirty patients with significant spontaneous/gaze or positional nystagmus (slow phase velocity >or= 6 degrees /s) were recruited from consecutive referrals for vestibular assessment. Nystagmus was recorded by ENG both in the presence and absence of mental alerting for each patient. Investigation of nystagmus by analysis of variance (ANOVA) revealed significantly larger nystagmus (higher value SPV) with mental alerting than with no alerting (p<0.001), and for some patients nystagmus traces were reduced to a flat line (no nystagmus) with no alerting. The study demonstrates the importance of mental alerting in helping overcome central suppression of nystagmus and highlights its importance to help identify peripheral type nystagmus during ENG.

Medical treatment of acquired nystagmus.

PubMed

Ehrhardt, David; Eggenberger, Eric

2012-11-01

This article synthesises recent findings and addresses relevant anatomy, pathophysiologic considerations, and current treatment options for common forms of acquired nystagmus including vestibular and gaze holding dysfunction. Some forms of nystagmus have relatively specific treatments, such as baclofen for periodic alternating nystagmus, and repositioning for benign paroxysmal positional vertigo. Recent studies have brought changes to many of the treatments of nystagmus variants. Additionally, other recent advances in nystagmus treatment, like the usage of 4-aminopyridine, have added potent medications to the physician's armamentarium. Nystagmus is a commonly encountered entity in clinical practice. However, evidence supported treatments are scarce. Medical treatment of nystagmus is difficult, with often limited and variable response to pharmacologic therapies. This mandates a continued re-evaluation of patients and creation of an individualized approach to this common clinical problem.

Acquired pendular nystagmus.

PubMed

Kang, Sarah; Shaikh, Aasef G

2017-04-15

Acquired pendular nystagmus is comprised of quasi-sinusoidal oscillations of the eyes significantly affecting gaze holding and clarity of vision. The most common causes of acquired pendular nystagmus include demyelinating disorders such as multiple sclerosis and the syndrome of ocular palatal tremor. However, several other deficits, such as pharmacological intoxication, metabolic and genetic disorders, and granulomatous disorders can lead to syndromes mimicking acquired pendular nystagmus. Study of the kinematic features of acquired pendular nystagmus has suggested a putative pathophysiology of an otherwise mysterious neurological disorder. Here we review clinical features of neurological deficits that co-occur with acquired pendular nystagmus. Subsequent discussion of the pathophysiology of individual forms of pendular nystagmus speculates on mechanisms of the underlying disease while providing insights into pharmacotherapy of nystagmus. Copyright © 2017 Elsevier B.V. All rights reserved.

Change in head posture and character of nystagmus in a patient with neurological upbeat nystagmus.

PubMed

Gonzalez, Caleb; Seth, Rajeev K; Ramos-Esteban, Jerome C

2007-01-01

To report a case of a patient with chin-up head posture and presumed congenital toxoplasmosis chorioretinal scars, who had a change in the character of the nystagmus and therefore the head posture following treatment for a neurological upbeat nystagmus. A 5 month old female presented with a chin up head posture and upbeat nystagmus. Magnetic resonance imaging of the brain revealed an arachnoid cyst in the area of the pineal gland. Nine months after cyst-peritoneal shunt surgery, the upbeat nystagmus was dampened but change in character to a rotary nystagmus worse on the left gaze. The patient had assumed a left face turn, shifting the null point from the vertical to the horizontal plane. The left face turn was successfully corrected at age eight years with a Kestenbaum procedure. This case emphasizes the possibility of having two distinct types of nystagmus associated with two etiologies. In this case, an acquired upbeat nystagmus secondary to an arachnoid cyst, and a congenital left rotary nystagmus from the chorioretinal scars. Furthermore, there can be a change in head position and character of nystagmus after treating the cause of the central motility disorder, thereby affecting the choice and timing of surgical intervention to correct the head positioning.

Therapy for nystagmus.

PubMed

Thurtell, Matthew J; Leigh, R John

2010-12-01

Pathological forms of nystagmus and their visual consequences can be treated using pharmacological, optical, and surgical approaches. Acquired periodic alternating nystagmus improves following treatment with baclofen, and downbeat nystagmus may improve following treatment with aminopyridines. Gabapentin and memantine are helpful in reducing acquired pendular nystagmus due to multiple sclerosis. Ocular oscillations in oculopalatal tremor may also improve following treatment with memantine or gabapentin. The infantile nystagmus syndrome (INS) may have only a minor impact on vision if "foveation periods" are well developed, but symptomatic patients may benefit from treatment with gabapentin, memantine, or base-out prisms to induce convergence. Several surgical therapies are also reported to improve INS, but selection of the optimal treatment depends on careful evaluation of visual acuity and nystagmus intensity in various gaze positions. Electro-optical devices are a promising and novel approach for treating the visual consequences of acquired forms of nystagmus.

Nystagmus Assessments Documented by Emergency Physicians in Acute Dizziness Presentations: A Target for Decision Support?

PubMed Central

Kerber, Kevin A.; Morgenstern, Lewis B.; Meurer, William J.; McLaughlin, Thomas; Hall, Pamela A.; Forman, Jane; Fendrick, A. Mark; Newman-Toker, David E.

2011-01-01

Objectives Dizziness is a common presenting complaint to the emergency department (ED), and emergency physicians (EPs) consider these presentations a priority for decision support. Assessing for nystagmus and defining its features are important steps for any acute dizziness decision algorithm. The authors sought to describe nystagmus documentation in routine ED care to determine if nystagmus assessments might be an important target in decision support efforts. Methods Medical records from ED visits for dizziness were captured as part of a surveillance study embedded within an ongoing population-based cohort study. Visits with documentation of a nystagmus assessment were reviewed and coded for presence or absence of nystagmus, ability to draw a meaningful inference from the description, and coherence with the final EP diagnosis when a peripheral vestibular diagnosis was made. Results Of 1,091 visits for dizziness, 887 (81.3%) documented a nystagmus assessment. Nystagmus was present in 185 out of 887 (20.9%) visits. When nystagmus was present, no further characteristics were recorded in 48 of the 185 visits (26%). The documentation of nystagmus (including all descriptors recorded) enabled a meaningful inference about the localization or cause in only 10 of the 185 (5.4%) visits. The nystagmus description conflicted with the EP diagnosis in 113 (80.7%) of the 140 visits that received a peripheral vestibular diagnosis. Conclusions Nystagmus assessments are frequently documented in acute dizziness presentations, but details do not generally enable a meaningful inference. Recorded descriptions usually conflict with the diagnosis when a peripheral vestibular diagnosis is rendered. Nystagmus assessments might be an important target in developing decision support for dizziness presentations. PMID:21676060

The Development of a Nystagmus-Specific Quality-of-Life Questionnaire.

PubMed

McLean, Rebecca J; Maconachie, Gail D E; Gottlob, Irene; Maltby, John

2016-09-01

To develop a nystagmus-specific quality-of-life (QOL) questionnaire derived from patient concerns based on eudaimonic aspects of well-being. Cross-sectional study. A total of 206 participants with nystagmus for factor analysis phase and an additional 42 participants with nystagmus for construct validity phase. Questionnaire items were written on the basis of the 6 domains of everyday living affected by nystagmus that were elicited by previous semistructured interviews conducted with 21 people with nystagmus. After consultation with 8 nystagmus experts, 37 items were administered to 206 people with nystagmus. Factor analysis was used to identify latent factors among the items and identify items to propose new nystagmus QOL scales. Cronbach's alpha was used to assess the internal reliability of the new scales. To assess for discriminate and concurrent validity between the new nystagmus scales and an existing vision-related QOL tool, the Visual Function Questionnaire-25 (VFQ-25) was administered to 42 additional participants. Questionnaire response scores on nystagmus-specific QOL items. The factor analysis revealed the retention of 29 items to form a measure comprising 2 distinct subscales reflecting "personal and social" and "physical and environmental" functioning as relating to nystagmus-specific QOL. The Cronbach's alpha coefficients for the "personal and social" functioning scale and "physical and environmental" functioning were 0.95 and 0.93, respectively. Tests for validity of the measure, consistent with a priori predictions, when compared with the VFQ-25, revealed the "physical and environmental" subscale showed concurrent validity (0.88), whereas the "personal and social" subscale was demonstrated to have discriminative validity (0.81). We have developed a 29-item, nystagmus-specific QOL questionnaire (NYS-29) based on eudaimonic aspects of well-being with subscales that address not only physical functioning but also psycho-social issues. The NYS-29 is grounded in the perspectives and concerns of those who have nystagmus and can be used to determine the impact of nystagmus on daily living in terms of both physical and psychosocial aspects. Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

Combining recessions (nystagmus and strabismus) with tenotomy improved visual function and decreased oscillopsia and diplopia in acquired downbeat nystagmus and in horizontal infantile nystagmus syndrome.

PubMed

Wang, Zhong I; Dell'Osso, Louis F; Tomsak, Robert L; Jacobs, Jonathan B

2007-04-01

To investigate the effects of combined tenotomy and recession procedures on both acquired downbeat nystagmus and horizontal infantile nystagmus. Patient 1 had downbeat nystagmus with a chin-down (upgaze) position, oscillopsia, strabismus, and diplopia. Asymmetric superior rectus recessions and inferior rectus tenotomies reduced right hypertropia and rotated both eyes downward. Patient 2 had horizontal infantile nystagmus, a 20 degrees left-eye exotropia, and alternating (abducting-eye) fixation. Lateral rectus recessions and medial rectus tenotomies were performed. Horizontal and vertical eye movements were recorded pre- and postsurgically using high-speed digital video. The eXpanded Nystagmus Acuity Function (NAFX) and nystagmus amplitudes and frequencies were measured. Patient 1: The NAFX peak moved from 10 degrees up to primary position where NAFX values improved 17% and visual acuity increased 25%. Vertical NAFX increased across the -10 degrees to +5 degrees vertical range. Primary-position right hypertropia decreased approximately 50%; foveation time per cycle increased 102%; vertical amplitude, oscillopsia, and diplopia were reduced, and frequency was unchanged. Patient 2: Two lateral, narrow high-NAFX regions (due to alternating fixation) became one broad region with a 43% increase in primary position (acuity increased approximately 92.3%). Diplopia amplitude decreased; convergence and gaze holding were improved. Primary-position right exotropia was reduced; foveation time per cycle increased 257%; horizontal-component amplitude decreased 45.7%, and frequency remained unchanged. Combining tenotomy with nystagmus or strabismus recession procedures increased NAFX and visual acuities and reduced diplopia and oscillopsia in downbeat nystagmus and infantile nystagmus.

Arnold-Chiari malformation and nystagmus of skew

PubMed Central

Pieh, C.; Gottlob, I.

2000-01-01

The Arnold-Chiari malfomation is typically associated with downbeat nystagmus. Eye movement recordings in two patients with Arnold-Chiari malfomation type 1 showed, in addition to downbeat and gaze evoked nystagmus, intermittent nystagmus of skew. To date this finding has not been reported in association with Arnold-Chiari malfomation. Nystagmus of skew should raise the suspicion of Arnold-Chiari malfomation and prompt sagittal head MRI examination.

 PMID:10864619

Allelic variation of the FRMD7 gene in congenital idiopathic nystagmus.

PubMed

Self, James E; Shawkat, Fatima; Malpas, Crispin T; Thomas, N Simon; Harris, Christopher M; Hodgkins, Peter R; Chen, Xiaoli; Trump, Dorothy; Lotery, Andrew J

2007-09-01

To perform a genotype-phenotype correlation study in an X-linked congenital idiopathic nystagmus pedigree (pedigree 1) and to assess the allelic variance of the FRMD7 gene in congenital idiopathic nystagmus. Subjects from pedigree 1 underwent detailed clinical examination including nystagmology. Screening of FRMD7 was undertaken in pedigree 1 and in 37 other congenital idiopathic nystagmus probands and controls. Direct sequencing confirmed sequence changes. X-inactivation studies were performed in pedigree 1. The nystagmus phenotype was extremely variable in pedigree 1. We identified 2 FRMD7 mutations. However, 80% of X-linked families and 96% of simplex cases showed no mutations. X-inactivation studies demonstrated no clear causal link between skewing and variable penetrance. We confirm profound phenotypic variation in X-linked congenital idiopathic nystagmus pedigrees. We demonstrate that other congenital nystagmus genes exist besides FRMD7. We show that the role of X inactivation in variable penetrance is unclear in congenital idiopathic nystagmus. Clinical Relevance We demonstrate that phenotypic variation of nystagmus occurs in families with FRMD7 mutations. While FRMD7 mutations may be found in some cases of X-linked congenital idiopathic nystagmus, the diagnostic yield is low. X-inactivation assays are unhelpful as a test for carrier status for this disease.

Diversity of head shaking nystagmus in peripheral vestibular disease.

PubMed

Kim, Min-Beom; Huh, Se Hyung; Ban, Jae Ho

2012-06-01

To evaluate the characteristics of head shaking nystagmus in various peripheral vestibular diseases. Retrospective case series. Tertiary referral center. Data of 235 patients with peripheral vestibular diseases including vestibular neuritis, Ménière's disease, and benign paroxysmal positional vertigo, were retrospectively analyzed. All subjects presented between August 2009 and July 2010. Patients were tested for vestibular function including head shaking nystagmus and caloric information. Regarding vestibular neuritis, all tests were again performed during the 1-month follow-up. Head shaking nystagmus was classified as monophasic or biphasic and, according to the affected ear, was divided as ipsilesional or contralesional. Of the 235 patients, 87 patients revealed positive head shaking nystagmus. According to each disease, positive rates of head shaking nystagmus were as follows: 35 (100%) of 35 cases of vestibular neuritis, 11 (68.8%) of 16 cases of Ménière's disease, and 41 (22.2%) of 184 cases of benign paroxysmal positional vertigo. All cases of vestibular neuritis initially presented as a monophasic, contralesional beating, head shaking nystagmus. However, 1 month after first visit, the direction of nystagmus was changed to biphasic (contralesional first then ipsilesional beating) in 25 cases (72.5%) but not in 10 cases (27.5%). There was a significant correlation between the degree of initial caloric weakness and the biphasic conversion of head shaking nystagmus (p = 0.02). In 72.5% of vestibular neuritis cases, head shaking nystagmus was converted to biphasic during the subacute period. The larger the initial canal paresis was present, the more frequent the biphasic conversion of head shaking nystagmus occurred. However, Ménière's disease and benign paroxysmal positional vertigo did not have specific patterns of head shaking nystagmus.

Upbeat nystagmus changes to downbeat nystagmus with upward gaze in a patient with Wernicke's encephalopathy.

PubMed

Shin, Byoung-Soo; Oh, Sun-Young; Kim, Ji Soo; Lee, Hyung; Kim, Eui-Jung; Hwang, Seung-Bae

2010-11-15

We describe a patient with Wernicke's encephalopathy who showed spontaneous upbeat nystagmus with decelerating slow phases that changed to downbeat nystagmus during upward gaze and increased during downward gaze. He also showed horizontal gaze-evoked nystagmus and impaired upward smooth pursuit. Magnetic resonance imaging demonstrated symmetric lesions involving the bilateral medial thalami, periaqueductal gray matters and inferior cerebellar peduncles. In this patient, the decelerating slow phases and disobedience to Alexander's law of upbeat nystagmus suggest both deficient (leaky) and unstable neural integrators subserving vertical eye motion. Dysfunction of the interstitial nucleus of Cajal or its descending pathway to the vestibulocerebellum via the paramedian tract cell groups may be responsible for the upbeat nystagmus and its modulation by gazes in our patient with Wernicke's encephalopathy. Copyright © 2010 Elsevier B.V. All rights reserved.

Periodic alternating nystagmus during caloric stimulation.

PubMed

Taki, Masakatsu; Hasegawa, Tatsuhisa; Adachi, Naoko; Fujita, Tomoki; Sakaguchi, Hirofumi; Hisa, Yasuo

2014-04-01

Periodic alternating nystagmus (PAN) is a form of horizontal jerk nystagmus characterized by periodic reversals in direction. We report a case who exhibited transient PAN induced by caloric stimulation. The patient was a 75-year-old male. He had experienced floating sensation in January 2010. Eight months later, he was referred to our university hospital. Gaze nystagmus and positional tests revealed no nystagmus. Only weak right-beating horizontal nystagmus was observed during left Dix-Hallpike maneuver. Electronystagmography showed normal saccadic and smooth pursuit eye movements. The optokinetic nystagmus pattern test was also bilaterally normal. However, during the caloric stimulation to the right ear, at 166 s from the start of irrigation, the direction of nystagmus alternated from leftward to rightward, and thereafter this reversal of direction repeated 15 times. Magnetic resonance imaging showed no significant lesion except for chronic ischemia in the brain. The patient probably had some kind of latent lesion of impaired velocity storage and exhibited transient PAN induced by caloric stimulation. Caloric stimulation is useful and simple examination to disclose latent eye movement disorders of which velocity storage mechanism is impaired. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Horizontal nystagmus and multiple sclerosis using 3-Tesla magnetic resonance imaging.

PubMed

Iyer, P M; Fagan, A J; Meaney, J F; Colgan, N C; Meredith, S D; Driscoll, D O; Curran, K M; Bradley, D; Redmond, J

2016-11-01

Nystagmus in patients with multiple sclerosis (MS) is generally attributed to brainstem disease. Lesions in other regions may result in nystagmus. The identification of these other sites is enhanced by using 3-Tesla magnetic resonance imaging (3TMRI) due to increased signal-to-noise ratio. We sought to evaluate the distribution of structural lesions and disruption of tracts in patients with horizontal nystagmus secondary to MS using 3TMRI. Twenty-four patients (20 women, 4 men; age range 26-55 years) with horizontal nystagmus secondary to MS underwent 3TMRI brain scans; and 18 patients had diffusion tensor imaging (DTI) for tractography. Nystagmus was bidirectional in 11, right-sided in 6 and left-sided in 7. We identified 194 lesions in 20 regions within the neural integrator circuit in 24 patients; 140 were within the cortex and 54 were within the brainstem. Only two patients had no lesions in the cortex, and 9 had no lesions in the brainstem. There was no relationship between side of lesion and direction of nystagmus. Thirteen of 18 (72 %) had tract disruption with fractional anisotropy (FA) values below 0.2. FA was significantly lower in bidirectional compared to unidirectional nystagmus (p = 0.006). In MS patients with horizontal nystagmus, lesions in all cortical eye fields and their descending connections were evident. Technical improvements in tractography may help identify the specific site(s) resulting in nystagmus in MS.

Nystagmus as an early ocular alteration in Machado-Joseph disease (MJD/SCA3)

PubMed Central

2014-01-01

Background Machado-Joseph disease (MJD), also named spinocerebellar ataxia type 3 (SCA3) is the most common autosomal dominant ataxia worldwide. Although nystagmus is one of the most frequently reported ocular alterations in MJD patients its behaviour during the course of the disease, namely in its early stages, has only recently started to be investigated. The main goal of this work was to characterize the frequency of nystagmus in symptomatic and presymptomatic carriers of the MJD mutation, and investigate its usefulness as an early indicator of the disease. Methods We conducted an observational study of Azorean MJD family members, comprising a total of 158 subjects which underwent neurological evaluation. Sixty eight were clinically and molecularly diagnosed with MJD, 48 were confirmed asymptomatic carriers and 42 were confirmed non-carriers of the MJD mutation. The frequency of nystagmus was calculated for the 3 groups. Results Nystagmus was present in 88% of the MJD patients. Seventeen percent of the at-risk subjects with a carrier result in the molecular test and none of the 42 individuals who received a non-carrier test result displayed nystagmus (p < 0.006). Although not reaching statistical significance, symptomatic subjects showing nystagmus had a tendency for a higher length of the CAG tract in the expanded allele, when compared to individuals who did not have nystagmus. Conclusions The frequency of nystagmus in asymptomatic carriers and its absence in non-carriers of the mutation, suggests that nystagmus may appear before gait disturbance and can thus be considered an early sign of MJD. PMID:24450306

Effect of Pitch Tilt on Vertical Optokinetic Nystagmus,

DTIC Science & Technology

1996-09-23

Naval Aerospace Medical Research Laboratory NAMRL-1394 EFFECT OF PITCH TILT ON VERTICAL OPTOKINETIC NYSTAGMUS M. J. Correia, O. I. Kolev, A...MEDICAL RESEARCH LABORATORY 51 HOVEY ROAD, PENSACOLA, FL 32508-1046 NAMRL-1394 EFFECT OF PITCH TILT ON VERTICAL OPTOKINETIC NYSTAGMUS M. J...Florida Pensacola, Florida Approved for public release; distribution unlimited. ABSTRACT Vertical optokinetic nystagmus (VOKN) and VOKN after

Clinical Correlation between Perverted Nystagmus and Brain MRI Abnormal Findings

PubMed Central

Han, Won-Gue; Yoon, Hee-Chul; Kim, Tae-Min; Rah, Yoon Chan

2016-01-01

Background and Objectives To analyze the clinical correlation between perverted nystagmus and brain magnetic resonance imaging (MRI) abnormal findings and to evaluate whether perverted nystagmus is clinically significant results of brain abnormal lesions or not. Subjects and Methods We performed medical charts review from January 2008 to July 2014, retrospectively. Patients who were suspected central originated vertigo at Frenzel goggles test were included among patients who visited our hospital. To investigate the correlation with nystagmus suspected central originated vertigo and brain MRI abnormal findings, we confirmed whether performing brain MRI or not. Then we exclude that patients not performed brain MRI. Results The number of patients with perverted nystagmus was 15, upbeating was 1 and down-beating was 14. Among these patients, 5 patients have brain MRI abnormal findings. However, 2 patients with MRI abnormal findings were not associated correctly with perverted nystagmus and only 3 patients with perverted nystagmus were considered central originated vertigo and further evaluation and treatment was performed by the department of neurology. Conclusions Perverted nystagmus was considered to the abnormalities at brain lesions, especially cerebellum, but neurologic symptoms and further evaluation were needed for exact diagnosis of central originated vertigo. PMID:27626081

Sudden sensorineural hearing loss with positional vertigo: Initial findings of positional nystagmus and hearing outcomes.

PubMed

Kim, Chang-Hee; Shin, Jung Eun; Yang, Young Soo; Im, Donghyuk

2016-10-01

To investigate the initial findings of positional nystagmus in patients with sudden sensorineural hearing loss (SSNHL) and positional vertigo, and to compare hearing improvement among patients with different types of positional nystagmus. The characteristics of positional nystagmus upon initial examination were analysed, and the initial mean pure-tone audiometry (PTA) threshold was compared with that at three months after treatment. Forty-four SSNHL patients with concomitant positional vertigo were included. Positional nystagmus was classified into five subgroups; persistent geotropic direction-changing positional nystagmus (DCPN) in head-roll test (HRT) and negative Dix-Hallpike test (DHT), persistent apogeotropic DCPN in HRT and negative DHT, positive DHT and negative HRT, persistent geotropic DCPN in HRT and positive DHT, and persistent apogeotropic DCPN in HRT and positive DHT. PTA threshold improvement was significantly greater in SSNHL patients with negative DHT than with positive DHT (p = 0.027). When geotropic DCPN was elicited by HRT, the nystagmus was persistent, which suggests that alteration of specific gravity of the endolymph, rather than the lateral canal canalolithiasis, may be a cause of this characteristic positional nystagmus. Positive DTH may be a prognostic factor for worse hearing recovery among patients with SSNHL and positional vertigo.

A new light on caloric test--what was disclosed by three dimensional analysis of caloric nystagmus?

NASA Technical Reports Server (NTRS)

Arai, Y.

2001-01-01

For better understanding of caloric nystagmus, this phenomenon will be reviewed historically in three stages. 1) The first light on caloric nystagmus was thrown by Barany 1906. Through direct observation of eye movements, Barany established the caloric test as an important tool to determine the side of lesion for vertigo. 2) The second light is shed by electrooculogram (EOG) from the late 1950th. EOG enabled qualitative analysis of caloric nystagmus, and proved Barany's convection theory, but resulted in neglect of vertical and roll eye movements. 3) The third light is gained by 3D recording of eye movements started from the late 1980th. 3D recordings of eye movements enabled us to analyze the spatial orientation of caloric nystagmus, and disclose the close correlation of the nystagmus components in the head vertical and the space vertical planes, suggesting a contribution of the velocity storage integrator. The 3D property of caloric nystagmus will be explained in detail.

The clinical and molecular genetic features of idiopathic infantile periodic alternating nystagmus

PubMed Central

Thomas, Mervyn G.; Crosier, Moira; Lindsay, Susan; Kumar, Anil; Thomas, Shery; Araki, Masasuke; Talbot, Chris J.; McLean, Rebecca J.; Surendran, Mylvaganam; Taylor, Katie; Leroy, Bart P.; Moore, Anthony T.; Hunter, David G.; Hertle, Richard W.; Tarpey, Patrick; Langmann, Andrea; Lindner, Susanne; Brandner, Martina

2011-01-01

Periodic alternating nystagmus consists of involuntary oscillations of the eyes with cyclical changes of nystagmus direction. It can occur during infancy (e.g. idiopathic infantile periodic alternating nystagmus) or later in life. Acquired forms are often associated with cerebellar dysfunction arising due to instability of the optokinetic-vestibular systems. Idiopathic infantile periodic alternating nystagmus can be familial or occur in isolation; however, very little is known about the clinical characteristics, genetic aetiology and neural substrates involved. Five loci (NYS1-5) have been identified for idiopathic infantile nystagmus; three are autosomal (NYS2, NYS3 and NYS4) and two are X-chromosomal (NYS1 and NYS5). We previously identified the FRMD7 gene on chromosome Xq26 (NYS1 locus); mutations of FRMD7 are causative of idiopathic infantile nystagmus influencing neuronal outgrowth and development. It is unclear whether the periodic alternating nystagmus phenotype is linked to NYS1, NYS5 (Xp11.4-p11.3) or a separate locus. From a cohort of 31 X-linked families and 14 singletons (70 patients) with idiopathic infantile nystagmus we identified 10 families and one singleton (21 patients) with periodic alternating nystagmus of which we describe clinical phenotype, genetic aetiology and neural substrates involved. Periodic alternating nystagmus was not detected clinically but only on eye movement recordings. The cycle duration varied from 90 to 280 s. Optokinetic reflex was not detectable horizontally. Mutations of the FRMD7 gene were found in all 10 families and the singleton (including three novel mutations). Periodic alternating nystagmus was predominantly associated with missense mutations within the FERM domain. There was significant sibship clustering of the phenotype although in some families not all affected members had periodic alternating nystagmus. In situ hybridization studies during mid-late human embryonic stages in normal tissue showed restricted FRMD7 expression in neuronal tissue with strong hybridization signals within the afferent arms of the vestibulo-ocular reflex consisting of the otic vesicle, cranial nerve VIII and vestibular ganglia. Similarly within the afferent arm of the optokinetic reflex we showed expression in the developing neural retina and ventricular zone of the optic stalk. Strong FRMD7 expression was seen in rhombomeres 1 to 4, which give rise to the cerebellum and the common integrator site for both these reflexes (vestibular nuclei). Based on the expression and phenotypic data, we hypothesize that periodic alternating nystagmus arises from instability of the optokinetic-vestibular systems. This study shows for the first time that mutations in FRMD7 can cause idiopathic infantile periodic alternating nystagmus and may affect neuronal circuits that have been implicated in acquired forms. PMID:21303855

Hyperventilation-induced nystagmus in a large series of vestibular patients.

PubMed

Califano, L; Melillo, M G; Vassallo, A; Mazzone, S

2011-02-01

The Hyperventilation Test is widely used in the "bed-side examination" of vestibular patients. It can either activate a latent nystagmus in central or peripheral vestibular diseases or it can interact with a spontaneous nystagmus, by reducing it or increasing it. Aims of this study were to determine the incidence, patterns and temporal characteristics of Hyperventilation-induced nystagmus in patients suffering from vestibular diseases, as well as its contribution to the differential diagnosis between vestibular neuritis and neuroma of the 8(th) cranial nerve, and its behaviour in some central vestibular diseases. The present study includes 1202 patients featuring, at vestibular examination, at least one sign of vestibular system disorders or patients diagnosed with a "Migraine-related vertigo" or "Chronic subjective dizziness". The overall incidence of Hyperventilation-induced nystagmus was 21.9%. It was detected more frequently in retrocochlear vestibular diseases rather than in end-organ vestibular diseases: 5.3% in Paroxysmal Positional Vertigo, 37.1% in Menière's disease, 37.6% in compensated vestibular neuritis, 77.2% in acute vestibular neuritis and 91.7% in neuroma of the 8(th) cranial nerve. In acute vestibular neuritis, three HVIN patterns were observed: Paretic pattern: temporary enhancement of the spontaneous nystagmus; Excitatory pattern: temporary inhibition of the spontaneous nystagmus; Strong excitatory pattern: temporary inversion of the spontaneous nystagmus. Excitatory patterns proved to be time-dependent in that they disappeared and were replaced by the paretic pattern over a period of maximum 18 days since the beginning of the disorder. In acoustic neuroma, Hyperventilation-induced nystagmus was frequently observed (91.7%), either in the form of an excitatory pattern (fast phases towards the affected site) or in the form of a paretic pattern (fast phases towards the healthy side). The direction of the nystagmus is only partially related to tumour size, whereas other mechanisms, such as demyelination or a break in nerve fibres, might have an important role in triggering the situation. Hyperventilation-induced nystagmus has frequently been detected in cases of demyelinating diseases and in cerebellar diseases: in multiple sclerosis, hyperventilation inhibits a central type of spontaneous nystagmus or evokes nystagmus in 75% of patients; in cerebellar diseases, hyperventilation evokes or enhances a central spontaneous nystagmus in 72.7% of patients. In conclusion the Hyperventilation Test can provide patterns of oculomotor responses that indicate a diagnostic investigation through cerebral magnetic resonance imaging enhanced by gadolinium, upon suspicion of neuroma of the 8(th) cranial nerve or of a central disease. In our opinion, however, Hyperventilation-induced nystagmus always needs to be viewed within the more general context of a complete examination of the vestibular and acoustic system.

Variants of windmill nystagmus.

PubMed

Choi, Kwang-Dong; Shin, Hae Kyung; Kim, Ji-Soo; Kim, Sung-Hee; Choi, Jae-Hwan; Kim, Hyo-Jung; Zee, David S

2016-07-01

Windmill nystagmus is characterized by a clock-like rotation of the beating direction of a jerk nystagmus suggesting separate horizontal and vertical oscillators, usually 90° out of phase. We report oculographic characteristics in three patients with variants of windmill nystagmus in whom the common denominator was profound visual loss due to retinal diseases. Two patients showed a clock-like pattern, while in the third, the nystagmus was largely diagonal (in phase or 180° out of phase) but also periodically changed direction by 180°. We hypothesize that windmill nystagmus is a unique manifestation of "eye movements of the blind." It emerges when the central structures, including the cerebellum, that normally keep eye movements calibrated and gaze steady can no longer perform their task, because they are deprived of the retinal image motion that signals a need for adaptive recalibration.

Extraocular Muscles in Patients With Infantile Nystagmus

PubMed Central

Berg, Kathleen T.; Hunter, David G.; Bothun, Erick D.; Antunes-Foschini, Rosalia; McLoon, Linda K.

2013-01-01

Objective To test the hypothesis that the extraocular muscles (EOMs) of patients with infantile nystagmus have muscular and innervational adaptations that may have a role in the involuntary oscillations of the eyes. Methods Specimens of EOMs from 10 patients with infantile nystagmus and postmortem specimens from 10 control subjects were prepared for histologic examination. The following variables were quantified: mean myofiber cross-sectional area, myofiber central nucleation, myelinated nerve density, nerve fiber density, and neuromuscular junction density. Results In contrast to control EOMs, infantile nystagmus EOMs had significantly more centrally nucleated myofibers, consistent with cycles of degeneration and regeneration. The EOMs of patients with nystagmus also had a greater degree of heterogeneity in myofiber size than did those of controls, with no difference in mean myofiber cross-sectional area. Mean myelinated nerve density, nerve fiber density, and neuromuscular junction density were also significantly decreased in infantile nystagmus EOMs. Conclusions The EOMs of patients with infantile nystagmus displayed a distinct hypoinnervated phenotype. This represents the first quantification of changes in central nucleation and myofiber size heterogeneity, as well as decreased myelinated nerve, nerve fiber, and neuromuscular junction density. These results suggest that deficits in motor innervation are a potential basis for the primary loss of motor control. Clinical Relevance Improved understanding of the etiology of nystagmus may direct future diagnostic and treatment strategies. PMID:22411664

[Case of acute ophthalmoparesis with gaze nystagmus].

PubMed

Ikuta, Naomi; Tada, Yukiko; Koga, Michiaki

2012-01-01

A 61-year-old man developed double vision subsequent to diarrheal illness. Mixed horizontal-vertical gaze palsy in both eyes, diminution of tendon reflexes, and gaze nystagmus were noted. His horizontal gaze palsy was accompanied by gaze nystagmus in the abducent direction, indicative of the disturbance in central nervous system. Neither limb weakness nor ataxia was noted. Serum anti-GQ1b antibody was detected. Brain magnetic resonance imaging (MRI) findings were normal. The patient was diagnosed as having acute ophthalmoparesis. The ophthalmoparesis and nystagmus gradually disappeared in 3 months. The accompanying nystagmus suggests that central nervous system disturbance may also be present with acute ophthalmoparesis.

Eye and Head Response to Peripheral Targets

DTIC Science & Technology

1989-08-01

nystagmus movements of the eyes. These move- ments tend to be oscillatory or unstable in nature and can be elicited in three ways: stimuli 2 in the...Hall and Cusack, 1972). Nystagmus can best be described through example. As mentioned previously, the com- pensatory eye movements serve to stabilize...movements are what are referred to as nystagmus . The direction of the nystagmus is identified by the movement of the fast phase, that is, the direction

Effectiveness of Botulinum Toxin Administered to Abolish Acquired Nystagmus

NASA Technical Reports Server (NTRS)

Leigh, R. John; Tomsak, Robert L.; Grant, Michael P.; Remler, Bernd F.; Yaniglos, Stacy S.; Lystad, Lisa; Dell'Osso, Louis F.

1992-01-01

We injected botulinum toxin into the horizontal rectus muscles of the right eyes of two patients who had acquired pendular nystagmus with horizontal, vertical, and torsional components. This treatment successfully abolished the horizontal component of the nystagmus in the injected eye in both patients for approximately 2 months. Both patients showed a small but measurable improvement of vision in the injected eye that may have been limited by coexistent disease of the visual pathways. The vertical and torsional components of the nystagmus persisted in both patients. In one patient, the horizontal component of nystagmus in the noninjected eye increased; we ascribe this finding to plastic-adaptive changes in response to paresis caused by the botulinum toxin. Such plastic-adaptive changes and direct side effects of the injections - such as diplopia and ptosis - may limit the effectiveness of botulinum toxin in the treatment of acquired nystagmus. Neither patient elected to repeat the botulinum treatment.

PubMed Central

CALIFANO, L.; MELILLO, M.G.; VASSALLO, A.; MAZZONE, S.

2011-01-01

SUMMARY The Hyperventilation Test is widely used in the "bed-side examination" of vestibular patients. It can either activate a latent nystagmus in central or peripheral vestibular diseases or it can interact with a spontaneous nystagmus, by reducing it or increasing it. Aims of this study were to determine the incidence, patterns and temporal characteristics of Hyperventilation-induced nystagmus in patients suffering from vestibular diseases, as well as its contribution to the differential diagnosis between vestibular neuritis and neuroma of the 8th cranial nerve, and its behaviour in some central vestibular diseases. The present study includes 1202 patients featuring, at vestibular examination, at least one sign of vestibular system disorders or patients diagnosed with a "Migraine-related vertigo" or "Chronic subjective dizziness". The overall incidence of Hyperventilation-induced nystagmus was 21.9%. It was detected more frequently in retrocochlear vestibular diseases rather than in end-organ vestibular diseases: 5.3% in Paroxysmal Positional Vertigo, 37.1% in Menière's disease, 37.6% in compensated vestibular neuritis, 77.2% in acute vestibular neuritis and 91.7% in neuroma of the 8th cranial nerve. In acute vestibular neuritis, three HVIN patterns were observed: Paretic pattern: temporary enhancement of the spontaneous nystagmus; Excitatory pattern: temporary inhibition of the spontaneous nystagmus; Strong excitatory pattern: temporary inversion of the spontaneous nystagmus. Excitatory patterns proved to be time-dependent in that they disappeared and were replaced by the paretic pattern over a period of maximum 18 days since the beginning of the disorder. In acoustic neuroma, Hyperventilation-induced nystagmus was frequently observed (91.7%), either in the form of an excitatory pattern (fast phases towards the affected site) or in the form of a paretic pattern (fast phases towards the healthy side). The direction of the nystagmus is only partially related to tumour size, whereas other mechanisms, such as demyelination or a break in nerve fibres, might have an important role in triggering the situation. Hyperventilation-induced nystagmus has frequently been detected in cases of demyelinating diseases and in cerebellar diseases: in multiple sclerosis, hyperventilation inhibits a central type of spontaneous nystagmus or evokes nystagmus in 75% of patients; in cerebellar diseases, hyperventilation evokes or enhances a central spontaneous nystagmus in 72.7% of patients. In conclusion the Hyperventilation Test can provide patterns of oculomotor responses that indicate a diagnostic investigation through cerebral magnetic resonance imaging enhanced by gadolinium, upon suspicion of neuroma of the 8th cranial nerve or of a central disease. In our opinion, however, Hyperventilation-induced nystagmus always needs to be viewed within the more general context of a complete examination of the vestibular and acoustic system. PMID:21808459

Exercise-induced downbeat nystagmus in a Korean family with a nonsense mutation in CACNA1A.

PubMed

Choi, Jae-Hwan; Seo, Jae-Deuk; Choi, Yu Ri; Kim, Min-Ji; Shin, Jin-Hong; Kim, Ji Soo; Choi, Kwang-Dong

2015-08-01

Episodic ataxia type 2 (EA2) is characterized by recurrent attacks of vertigo and ataxia lasting hours triggered by emotional stress or exercise. Although interictal horizontal gaze-evoked nystagmus and rebound nystagmus are commonly observed in patients with EA2, the nystagmus has been rarely reported during the vertigo attack. To better describe exercise-induced nystagmus in EA2, four affected members from three generations of a Korean family with EA2 received full neurological and neuro-otological evaluations. Vertigo was provoked in the proband with running for 10 min to record eye movements during the vertigo attack. We performed a polymerase chain reaction-based direct sequence analysis of all coding regions of CACNA1A in all participants. The four affected members had a history of exertional vertigo, imbalance, childhood epilepsy, headache, and paresthesia. The provocation induced severe vertigo and imbalance lasting several hours, and oculography documented pure downbeat nystagmus during the attack. Genetic analyses identified a nonsense mutation in exon 23 which has been registered in dbSNP as a pathogenic allele (c.3832C>T, p.R1278X) in all the affected members. Ictal downbeat nystagmus in the studied family indicates cerebellar dysfunction during the vertigo attack in EA2. In patients with episodic vertigo and ataxia, the observation of exercise-induced nystagmus would provide a clue for EA2.

Role of irregular otolith afferents in the steady-state nystagmus during off-vertical axis rotation

NASA Technical Reports Server (NTRS)

Angelaki, D. E.; Perachio, A. A.; Mustari, M. J.; Strunk, C. L.

1992-01-01

1. During constant velocity off-vertical axis rotations (OVAR) in the dark a compensatory ocular nystagmus is present throughout rotation despite the lack of a maintained signal from the semicircular canals. Lesion experiments and canal plugging have attributed the steady-state ocular nystagmus during OVAR to inputs from the otolith organs and have demonstrated that it depends on an intact velocity storage mechanism. 2. To test whether irregularly discharging otolith afferents play a crucial role in the generation of the steady-state eye nystagmus during OVAR, we have used anodal (inhibitory) currents bilaterally to selectively and reversibly block irregular vestibular afferent discharge. During delivery of DC anodal currents (100 microA) bilaterally to both ears, the slow phase eye velocity of the steady-state nystagmus during OVAR was reduced or completely abolished. The disruption of the steady-state nystagmus was transient and lasted only during the period of galvanic stimulation. 3. To distinguish a possible effect of ablation of the background discharge rates of irregular vestibular afferents on the velocity storage mechanism from specific contributions of the dynamic responses from irregular otolith afferents to the circuit responsible for the generation of the steady-state nystagmus, bilateral DC anodal galvanic stimulation was applied during optokinetic nystagmus (OKN) and optokinetic afternystagmus (OKAN). No change in OKN and OKAN was observed.(ABSTRACT TRUNCATED AT 250 WORDS).

Vertigo and nystagmus in orthostatic hypotension.

PubMed

Choi, J-H; Seo, J-D; Kim, M-J; Choi, B-Y; Choi, Y R; Cho, B M; Kim, J S; Choi, K-D

2015-04-01

Generalized cerebral ischaemia from cardiovascular dysfunction usually leads to presyncopal dizziness, but several studies reported a higher frequency of rotatory vertigo in cardiovascular patients. Whether generalized cerebral ischaemia due to cardiovascular disorders may produce objective vestibular dysfunction was investigated. Thirty-three patients with orthostatic dizziness/vertigo due to profound orthostatic hypotension and 30 controls were recruited. All participants underwent recording of eye movements during two orthostatic challenging tests: the Schellong and the squatting-standing tests. Most patients had neuroimaging, and patients with abnormal eye movements were subjected to follow-up evaluations. Symptoms associated with orthostatic dizziness/vertigo included blurred vision, fainting and tinnitus. Ten (30%) of 33 patients developed rotatory vertigo and nystagmus during the Schellong (n = 5) or squatting-standing test (n = 5). Four of them showed pure downbeat nystagmus whilst five had downbeat and horizontal nystagmus with or without torsional component. Patients with orthostatic nystagmus had shorter duration of orthostatic intolerance than those without nystagmus (1.0 ± 1.6 vs. 11.0 ± 9.7 months, P < 0.001). In two patients, orthostatic nystagmus disappeared during follow-up despite the persistence of profound orthostatic hypotension. Generalized cerebral ischaemia caused by orthostatic hypotension induces rotatory vertigo due to objective vestibular dysfunction. The presence of orthostatic vertigo and nystagmus has an association with the duration of orthostatic intolerance. © 2014 EAN.

Nystagmus using video-oculography in psychiatric patients.

PubMed

Kiyomizu, Kensuke; Matsuda, Keiji; Torihara, Koji; Nakayama, Meiho; Komaki, Shogo; Tono, Tetsuya; Ishida, Yasushi; Yoshida, Kensei; Kimitsuki, Takashi

2009-08-01

To evaluate whether nystagmus has clinical significance in psychiatric patients who have functional and/or organic brain dysfunction. We performed gaze, positional and positioning nystagmus tests on 227 patients with psychiatric diseases (144 men, 83 women, with an average age +/- SD of 62.5 +/- 14.0 years) in order to evaluate the frequency and characteristics of nystagmus. Patients were classified according to the underlying disease. Normal control subjects were 107 subjects (26 men, 81 women, with an average age +/- SD of 35.6 +/- 10.0 years). Nystagmus was observed in 56 (24.7%) of 227 cases. Nystagmus was seen in 16 (59.3%) of 27 cases of alcoholism, 14 (22.2%) of 63 cases of organic psychiatric disorders, 25 (20.2%) of 124 cases of schizophrenia, 1 (20.0%) of 5 cases of excited mental retardation, 0 (0.0%) of 7 cases of mood disorders, 0 (0.0%) of 1 case of anxiety disorders and 1 (0.9%) of 107 subjects of normal control. There was a significant difference between psychiatric diseases and normal control. These results indicate that nystagmus may also be a very important clinical finding not only in patients with neurological and neuro-otological diseases, but also in patients with psychiatric diseases.

Immediate postoperative nystagmus and vestibular symptoms after stapes surgery.

PubMed

Hirvonen, Timo P; Aalto, Heikki

2013-08-01

Vestibular disturbance is frequent, but mild even immediately after stapes surgery. Vestibular symptoms improved or disappeared quickly, and they did not correlate with nystagmus. Outpatient stapes surgery performed under local anaesthesia is a feasible approach. Vestibular symptoms are common and may prevent outpatient surgery. The time course of vestibular disturbance is unclear, and we aimed to evaluate it immediately after the operation in the recovery room. Twenty patients with otosclerosis undergoing stapedotomy were prospectively included in the study. Postoperative symptoms were collected and nystagmus was recorded with video-oculography (VOG) on average 29 min after the surgery. None of the patients had spontaneous nystagmus with gaze fixation. Nine patients (45%) had slow spontaneous horizontal nystagmus (mean slow phase velocity of 1.1°/s) in the primary position without gaze fixation. In seven of these, the nystagmus obeyed Alexander's law. Nine patients (45%) had vestibular symptoms at the end of the surgery, and four patients at the time of VOG recording. Vertigo was experienced immediately after the operation in five, floating sensation in two, and unspecific dizziness in two patients. Vestibular symptoms were mild or moderate in most patients. The occurrence of nystagmus did not correlate with vestibular symptoms (p > 0.05).

Effect of 3,4-diaminopyridine on the postural control in patients with downbeat nystagmus.

PubMed

Sprenger, Andreas; Zils, Elisabeth; Rambold, Holger; Sander, Thurid; Helmchen, Christoph

2005-04-01

Downbeat nystagmus (DBN) is a common, usually persistent ocular motor sign in vestibulocerebellar midline lesions. Postural imbalance in DBN may increase on lateral gaze when downbeat nystagmus increases. 3,4-Diaminopyridine (3,4-DAP) has been shown to suppress the slow-phase velocity component of downbeat nystagmus and its gravity-dependent component with concomitant improvement of oscillopsia. Because the pharmacological effect is thought to be caused by improvement of the vestibulocerebellar Purkinje cell activity, the effect of 3,4-DAP on the postural control of patients with downbeat nystagmus syndrome was examined. Eye movements were recorded with the video-based Eyelink II system. Postural sway and pathway were assessed by posturography in lateral gaze in the light and on eye closure. Two out of four patients showed an improvement of the area of postural sway by 57% of control (baseline) on eye closure. In contrast, downbeat nystagmus in gaze straight ahead and on lateral gaze did not benefit in these two patients, implying a specific influence of 3,4-DAP on the vestibulocerebellar control of posture. It was concluded that 3,4-DAP may particularly influence the postural performance in patients with downbeat nystagmus.

Gravity-dependent nystagmus and inner-ear dysfunction suggest anterior and posterior inferior cerebellar artery infarct.

PubMed

Shaikh, Aasef G; Miller, Benjamin R; Sundararajan, Sophia; Katirji, Bashar

2014-04-01

Cerebellar lesions may present with gravity-dependent nystagmus, where the direction and velocity of the drifts change with alterations in head position. Two patients had acute onset of hearing loss, vertigo, oscillopsia, nausea, and vomiting. Examination revealed gravity-dependent nystagmus, unilateral hypoactive vestibulo-ocular reflex (VOR), and hearing loss ipsilateral to the VOR hypofunction. Traditionally, the hypoactive VOR and hearing loss suggest inner-ear dysfunction. Vertigo, nausea, vomiting, and nystagmus may suggest peripheral or central vestibulopathy. The gravity-dependent modulation of nystagmus, however, localizes to the posterior cerebellar vermis. Magnetic resonance imaging in our patients revealed acute cerebellar infarct affecting posterior cerebellar vermis, in the vascular distribution of the posterior inferior cerebellar artery (PICA). This lesion explains the gravity-dependent nystagmus, nausea, and vomiting. Acute onset of unilateral hearing loss and VOR hypofunction could be the manifestation of inner-ear ischemic injury secondary to the anterior inferior cerebellar artery (AICA) compromise. In cases of combined AICA and PICA infarction, the symptoms of peripheral vestibulopathy might masquerade the central vestibular syndrome and harbor a cerebellar stroke. However, the gravity-dependent nystagmus allows prompt identification of acute cerebellar infarct. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

Suppression of spontaneous nystagmus during different visual fixation conditions.

PubMed

Hirvonen, Timo P; Juhola, Martti; Aalto, Heikki

2012-07-01

Analysis of spontaneous nystagmus is important in the evaluation of dizzy patients. The aim was to measure how different visual conditions affect the properties of nystagmus using three-dimensional video-oculography (VOG). We compared prevalence, frequency and slow phase velocity (SPV) of the spontaneous nystagmus with gaze fixation allowed, with Frenzel's glasses, and in total darkness. Twenty-five patients (35 measurements) with the peripheral vestibular pathologies were included. The prevalence of nystagmus with the gaze fixation was 40%, and it increased significantly to 66% with Frenzel's glasses and regular room lights on (p < 0.01). The prevalence increased significantly to 83% when the regular room lights were switched off (p = 0.014), and further to 100% in total darkness (p = 0.025). The mean SPV of nystagmus with visual fixation allowed was 1.0°/s. It increased to 2.4°/s with Frenzel's glasses and room lights on, and additionally to 3.1°/s, when the regular room lights were switched off. The mean SPV in total darkness was 6.9°/s. The difference was highly significant between all test conditions (p < 0.01). The frequency of nystagmus was 0.7 beats/s with gaze fixation, 0.8 beats/s in both the test conditions with Frenzel's glasses on, and 1.2 beats/s in total darkness. The frequency in total darkness was significantly higher (p < 0.05) than with Frenzel's glasses, and more so than with visual fixation (p = 0.003). The VOG in total darkness is superior in detecting nystagmus, since Frenzel's glasses allow visual suppression to happen, and this effect is reinforced with gaze fixation allowed. Strict control of visual surroundings is essential in interpreting peripheral nystagmus.

Fusion maldevelopment (latent/manifest latent) nystagmus syndrome: effects of four-muscle tenotomy and reattachment.

PubMed

Dell'Osso, Louis F; Orge, Faruk H; Jacobs, Jonathan B; Wang, Zhong I

2014-01-01

To examine the waveform and clinical effects of the four-muscle tenotomy and reattachment procedure in fusion maldevelopment nystagmus syndrome (FMNS) and to compare them to those documented in infantile nystagmus syndrome (INS) and acquired nystagmus. Both infrared reflection and high-speed digital video systems were used to record the eye movements in a patient with FMNS (before and after tenotomy and reattachment). Data were analyzed using the eXpanded Nystagmus Acuity Function (NAFX) that is part of the OMtools software. Model simulations and predictions were performed using the authors' behavioral ocular motor system model in MATLAB Simulink (The MathWorks, Inc., Natick, MA). The model predicted, and the patient's data confirmed, that the tenotomy and reattachment procedure produces improvements in FMN waveforms across a broader field of gaze and decreases the Alexander's law variation. The patient's tenotomy and reattachment plots of NAFX after surgery versus gaze angle were higher and had lower slope than before surgery. Clinically, despite moderate improvements in both peak measured acuity and stereoacuity, dramatic improvements in the patient's abilities and lifestyle resulted. The four-muscle tenotomy and reattachment nystagmus surgery produced beneficial therapeutic effects on FMN waveforms that are similar to those demonstrated in INS and acquired nystagmus. These results support the authors' prior recommendation that tenotomy and reattachment nystagmus should be added to required strabismus procedures in patients who also have FMNS (ie, perform tenotomy and reattachment on all unoperated muscles in the plane of the nystagmus). Furthermore, when strabismus surgery is not required, four-muscle tenotomy and reattachment may be used to improve FMN waveforms and visual function. Copyright 2014, SLACK Incorporated.

Abnormal Head Position in Infantile Nystagmus Syndrome

PubMed Central

Noval, Susana; González-Manrique, Mar; Rodríguez-Del Valle, José María; Rodríguez-Sánchez, José María

2011-01-01

Infantile nystagmus is an involuntary, bilateral, conjugate, and rhythmic oscillation of the eyes which is present at birth or develops within the first 6 months of life. It may be pendular or jerk-like and, its intensity usually increases in lateral gaze, decreasing with convergence. Up to 64% of all patients with nystagmus also present strabismus, and even more patients have an abnormal head position. The abnormal head positions are more often horizontal, but they may also be vertical or take the form of a tilt, even though the nystagmus itself is horizontal. The aim of this article is to review available information about the origin and treatment of the abnormal head position associated to nystagmus, and to describe our treatment strategies. PMID:24533187

Toxicology observation: nystagmus after marijuana use.

PubMed

Kibby, Thomas; Halcomb, S Eliza

2013-05-01

Traditional teaching has held that horizontal-gaze nystagmus is a sign of intoxication by sedatives such as alcohol but not marijuana. This is a case report of an adult male who presents with 3 days of visual disturbance and dizziness following marijuana use. The exam was notable for gaze-evoked nystagmus and ataxia. Lab testing was normal except that urine drug screening was positive for marijuana only. Imaging included computed tomography (CT) and magnetic resonance imaging (MRI) scans of the head. Prior studies showing a negative association of nystagmus with marijuana are reviewed. This case is presented as a possible exception to the generalisation that marijuana is not associated with nystagmus. Copyright © 2012 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

Lithium-induced downbeat nystagmus.

PubMed

Schein, Flora; Manoli, Pierre; Cathébras, Pascal

2017-09-01

We report the case of a 76-year old lady under lithium carbonate for a bipolar disorder who presented with a suspected optic neuritis. A typical lithium-induced downbeat nystagmus was observed. Discontinuation of lithium therapy resulted in frank improvement in visual acuity and disappearance of the nystagmus.

Spontaneous nystagmus in benign paroxysmal positional vertigo: is it a new sign?

PubMed

Hajiabolhassan, Fahimeh; Tavanai, Elham

2013-01-01

Benign Paroxysmal Positional Vertigo (BPPV) is a condition that indicates a benign inner ear disorder. It is generally believed that BPPV is due to the dislodged otoconial particles from otolith organs and unusual collection of them within any of semicircular canals or even in all three semicircular canals. Although the typical features of nystagmus in BPPV have been well-studied, very few studies (just four articles) have highlighted the presence of spontaneous nystagmus in BPPV recently. During the past 10 years, 2850 patients have been examined at the audiology unit of our department, and 254 patients have received diagnoses of BPPV but recently 2 patients presented with BPPV and spontaneous nystagmus, a new symptom that has been never observed in our clinical records. We herein describe this rare symptom in 2 case of BPPV. A 50-year-old woman with BPPV who showed an 18 degree spontaneous nystagmus treated with Epley maneuver and a 53-year-old man with 3 degree spontaneous nystagmus.

Spontaneous nystagmus in dorsolateral medullary infarction indicates vestibular semicircular canal imbalance.

PubMed

Rambold, H; Helmchen, C

2005-01-01

Spontaneous nystagmus caused by dorsolateral medullary infarction may be of vestibular origin. To test if imbalance of the central pathways of the semicircular canals contributes to spontaneous nystagmus in dorsolateral medullary syndrome. We examined four patients with dorsolateral medullary syndrome and recorded spontaneous nystagmus binocularly at gaze straight ahead with the three-dimensional search coil technique. The median slow phase velocity of the nystagmus was analysed in the light and in the dark, and the normalised velocity axes were compared with the rotation axes as predicted from anatomical data of the semicircular canal. The slow phase rotation axes of all patients aligned best with the rotation axes resulting from stimulation of the contralesional posterior and horizontal semicircular canals. This alignment cannot be explained by pure otolith imbalance. We propose that vestibular imbalance caused by an ipsilesional lesion of the central semicircular canal pathways of the horizontal and anterior semicircular canals largely accounts for spontaneous nystagmus in dorsolateral medullary syndrome.

Nystagmus in SCA territory cerebellar infarction: pattern and a possible mechanism.

PubMed

Lee, Hyung; Kim, Hyun-Ah

2013-04-01

To investigate the frequency and pattern of nystagmus associated with isolated cerebellar infarction in the territory of the superior cerebellar artery (SCA) and to discuss its possible mechanism. We identified 41 consecutive patients with isolated SCA territory cerebellar infarction diagnosed by MRI. Each patient completed a standardised dizziness questionnaire and underwent neurotological evaluations. Eye movements were recorded using 3-dimensional video-oculography during the acute period. Approximately half (19/41) of the patients experienced true vertigo early in the course of the SCA distribution infarct. 11 (27%) of the 41 patients showed spontaneous nystagmus (SN) or direction changed bidirectional gaze-evoked nystagmus (GEN). SN was observed in 10 patients (24%) and the horizontal component of SN was predominant in most case (80%, 8/10) and always beat towards the lesion side. Direction changed bidirectional GEN was observed in five patients and was mostly (4/5) accompanied by SN. Lesion subtraction analyses revealed that damage to the rostral anterior cerebellum including the ala of the central lobule and part of the quadrangular lobule was more frequent in patients with nystagmus compared to patients without nystagmus (9/11, 82% vs 11/30, 37%) (p=0.015). In most (82%, 9/11) patients with SN or GEN, the nystagmus subsided within 1 week after hospitalisation. Vertigo and nystagmus in SCA territory cerebellar infarction are more common than previously thought. Ipsilesional SN may result from damage to the anterior lobe of the cerebellum, which transmits the vestibular output to the fastigial nucleus.

21 CFR 886.1905 - Nystagmus tape.

Code of Federal Regulations, 2010 CFR

2010-04-01

... Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES OPHTHALMIC DEVICES Diagnostic Devices § 886.1905 Nystagmus tape. (a) Identification. Nystagmus tape is a device that is a long, narrow strip of fabric or other flexible material on which a series of objects are...

Emotional reaction evaluation provoked by the vestibular caloric test through physiological variables monitoring.

PubMed

Barona-de-Guzmán, Rafael; Krstulovic-Roa, Claudio; Donderis-Malea, Elena; Barona-Lleó, Luz

2018-03-08

The emotional evaluation of the causes of vertigo is made using the clinical records and several subjective questionnaires. The aim of the present study is to evaluate the emotional response objectively, in normal subjects, during an induced vertigo crisis. A caloric vestibular test with cold water was performed on 30 healthy subjects. The following physiological parameters were monitored during the 60seconds prior to and the 60seconds after the stimulation: Skin Conductivity, Peripheral Pulse Volume, Body Temperature, Muscle Contraction, Heart Rate, and Respiratory Rate. The maximum angular speed of the nystagmus slow phase at each stimulation was assessed. Skin conductance presented a statistically significant increase during the vertigo crisis in relation to the prior period while the peripheral pulse volume presented a statistically significant decrease. There was no relationship between the slow phase of the provoked nystagmus angular speed and skin conductance and peripheral pulse volume changes. The decrease in peripheral pulse volume was significantly higher in the second vertigo crisis. Skin conductance and peripheral pulse volume changed significantly during a vertigo crisis. There was no relation between the provoked vertiginous crisis intensity and the changes produced in those variables. The stress generated by the caloric stimulation is higher in the second crisis, when the subject has experience of the vertigo caused by the stimulation. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

Uncoupling VOR and vestibuloautonomic retention to Coriolis acceleration training in student pilots and control subjects.

PubMed

Wang, Linjie; Cao, Yi; Tan, Cheng; Zhao, Qi; He, Siyang; Niu, Dongbin; Tang, Guohua; Zou, Peng; Xing, Lei

2017-01-01

Explore the different vestibular physiologic response retention patterns after Coriolis acceleration training in student pilots and extend the results for use with Chinese astronauts in the future. Twelve healthy control male subjects were screened from males familiar with vestibular training and who physically resembled the astronauts. Fourteen student pilots were selected from 23 participants by rotational vestibular function tests. All subjects were exposed to five-day continuous or intermittent Coriolis acceleration training. Subjective motion sickness (MS) symptom scores, electrocardiography, electrogastrography (EGG), post-rotatory nystagmus and renin-angiotensin system responses were measured before, during and after rotational vestibular function tests at different times after vestibular training. Subjects could tolerate 10 min or 15 min of vestibular with mild MS symptoms. Retention of vestibular autonomic responses (retention of MS symptom scores, heart rate variability, power density of EGG, variations in levels of arginine vasopressin) were approximately 1 week for control subjects and approximately 5 weeks for student pilots. Decreases in slow-phase velocity of post-rotatory nystagmus were maintained for 14 weeks for control subjects and 9 weeks for student pilots. Retention of the vestibulo-autonomic reaction after vestibular training was different for control subjects and student pilots. All parameters related to autonomic responses could be maintained at low levels after vestibular training for approximately 1 week for control subjects and approximately 5 weeks for student pilots. Uncoupling patterns between post-rotatory nystagmus and the vestibulo-autonomic reaction may be helpful in the design of clinical rehabilitation plans for balance-disorder patients and for exploration of artificial gravity in future space missions.

Visual-Ocular Control of Normal and Learning-Disabled Children.

ERIC Educational Resources Information Center

Polatajko, H. J.

1987-01-01

Differences in visual-ocular function, particularly optokinetic nystagmus (OKN), were compared with 40 learning disabled and 40 normal children (8-12 years-old). No significant differences were found between groups on the variables tested (refixation saccades, smooth ocular pursuit, spontaneous nystagmus, gaze nystagmus, and OKN). (Author/DB)

Continuing Support of the ASA Standards Program

DTIC Science & Technology

1993-05-20

ba-Ic vestibular function test battery consisting of six separate tests: spontaneous nystagmus , gaze -evoked nystagmus , saccade test, pursuit testing...positional nystagmus and caloric testing. S3 LIAISON WORKING GROUPS a) S3/L-1 S3 TAG Liaison to IEC/TC 87 Ultrasonics - W.Nybg To provide liaison on

Gaze-evoked nystagmus induced by alcohol intoxication.

PubMed

Romano, Fausto; Tarnutzer, Alexander A; Straumann, Dominik; Ramat, Stefano; Bertolini, Giovanni

2017-03-15

The cerebellum is the core structure controlling gaze stability. Chronic cerebellar diseases and acute alcohol intoxication affect cerebellar function, inducing, among others, gaze instability as gaze-evoked nystagmus. Gaze-evoked nystagmus is characterized by increased centripetal eye-drift. It is used as an important diagnostic sign for patients with cerebellar degeneration and to assess the 'driving while intoxicated' condition. We quantified the effect of alcohol on gaze-holding using an approach allowing, for the first time, the comparison of deficits induced by alcohol intoxication and cerebellar degeneration. Our results showed that alcohol intoxication induces a two-fold increase of centripetal eye-drift. We establish analysis techniques for using controlled alcohol intake as a model to support the study of cerebellar deficits. The observed similarity between the effect of alcohol and the clinical signs observed in cerebellar patients suggests a possible pathomechanism for gaze-holding deficits. Gaze-evoked nystagmus (GEN) is an ocular-motor finding commonly observed in cerebellar disease, characterized by increased centripetal eye-drift with centrifugal correcting saccades at eccentric gaze. With cerebellar degeneration being a rare and clinically heterogeneous disease, data from patients are limited. We hypothesized that a transient inhibition of cerebellar function by defined amounts of alcohol may provide a suitable model to study gaze-holding deficits in cerebellar disease. We recorded gaze-holding at varying horizontal eye positions in 15 healthy participants before and 30 min after alcohol intake required to reach 0.6‰ blood alcohol content (BAC). Changes in ocular-motor behaviour were quantified measuring eye-drift velocity as a continuous function of gaze eccentricity over a large range (±40 deg) of horizontal gaze angles and characterized using a two-parameter tangent model. The effect of alcohol on gaze stability was assessed analysing: (1) overall effects on the gaze-holding system, (2) specific effects on each eye and (3) differences between gaze angles in the temporal and nasal hemifields. For all subjects, alcohol consumption induced gaze instability, causing a two-fold increase [2.21 (0.55), median (median absolute deviation); P = 0.002] of eye-drift velocity at all eccentricities. Results were confirmed analysing each eye and hemifield independently. The alcohol-induced transient global deficit in gaze-holding matched the pattern previously described in patients with late-onset cerebellar degeneration. Controlled intake of alcohol seems a suitable disease model to study cerebellar GEN. With alcohol resulting in global cerebellar hypofunction, we hypothesize that patients matching the gaze-holding behaviour observed here suffered from diffuse deficits in the gaze-holding system as well. © 2016 The Authors. The Journal of Physiology © 2016 The Physiological Society.

The Effect of Gaze Angle on Visual Acuity in Infantile Nystagmus.

PubMed

Dunn, Matt J; Wiggins, Debbie; Woodhouse, J Margaret; Margrain, Tom H; Harris, Christopher M; Erichsen, Jonathan T

2017-01-01

Most individuals with infantile nystagmus (IN) have an idiosyncratic gaze angle at which their nystagmus intensity is minimized. Some adopt an abnormal head posture to use this "null zone," and it has therefore long been assumed that this provides people with nystagmus with improved visual acuity (VA). However, recent studies suggest that improving the nystagmus waveform could have little, if any, influence on VA; that is, VA is fundamentally limited in IN. Here, we examined the impact of the null zone on VA. Visual acuity was measured in eight adults with IN using a psychophysical staircase procedure with reversals at three horizontal gaze angles, including the null zone. As expected, changes in gaze angle affected nystagmus amplitude, frequency, foveation duration, and variability of intercycle foveation position. Across participants, each parameter (except frequency) was significantly correlated with VA. Within any given individual, there was a small but significant improvement in VA (0.08 logMAR) at the null zone as compared with the other gaze angles tested. Despite this, no change in any of the nystagmus waveform parameters was significantly associated with changes in VA within individuals. A strong relationship between VA and nystagmus characteristics exists between individuals with IN. Although significant, the improvement in VA observed within individuals at the null zone is much smaller than might be expected from the occasionally large variations in intensity and foveation dynamics (and anecdotal patient reports of improved vision), suggesting that improvement of other aspects of visual performance may also encourage use of the null zone.

Horizontal gaze nystagmus: a review of vision science and application issues.

PubMed

Rubenzer, Steven J; Stevenson, Scott B

2010-03-01

The Horizontal Gaze Nystagmus (HGN) test is one component of the Standardized Field Sobriety Test battery. This article reviews the literature on smooth pursuit eye movement and gaze nystagmus with a focus on normative responses, the influence of alcohol on these behaviors, and stimulus conditions similar to those used in the HGN sobriety test. Factors such as age, stimulus and background conditions, medical conditions, prescription medications, and psychiatric disorder were found to affect the smooth pursuit phase of HGN. Much less literature is available for gaze nystagmus, but onset of nystagmus may occur in some sober subjects at 45 degrees or less. We conclude that HGN is limited by large variability in the underlying normative behavior, from methods and testing environments that are often poorly controlled, and from a lack of rigorous validation in laboratory settings.

A Sparse Matrix Approach for Simultaneous Quantification of Nystagmus and Saccade

NASA Technical Reports Server (NTRS)

Kukreja, Sunil L.; Stone, Lee; Boyle, Richard D.

2012-01-01

The vestibulo-ocular reflex (VOR) consists of two intermingled non-linear subsystems; namely, nystagmus and saccade. Typically, nystagmus is analysed using a single sufficiently long signal or a concatenation of them. Saccade information is not analysed and discarded due to insufficient data length to provide consistent and minimum variance estimates. This paper presents a novel sparse matrix approach to system identification of the VOR. It allows for the simultaneous estimation of both nystagmus and saccade signals. We show via simulation of the VOR that our technique provides consistent and unbiased estimates in the presence of output additive noise.

Vestibular and Oculomotor Physiology: International Meeting of the Barany Society. Volume 374. Annals of the New York Academy of Sciences

DTIC Science & Technology

1981-11-06

A. L. 1968. The Brain Stem of the Cat. University of Wisconsin Press. Madison . Wis. 8. KICVER, 1-. & E. BARRERA. 1953. A method for the combined...Inhibition of central vestibular neurons from the W~ UW % labyrinth and its mediating pathway. 1. NeurophysioL 29: 467-492. S1 MI 1111 t BAME & A. GLWTYN. 1980...revealed lethargy, slow speech, nystagmus, dysmetria, and ataxia. A lumbar puncture at that time showed a spinal fluid protein of 110 mg%, glucose of 15

Effect of gravity on vertical eye position.

PubMed

Pierrot-Deseilligny, C

2009-05-01

There is growing evidence that gravity markedly influences vertical eye position and movements. A new model for the organization of brainstem upgaze pathways is presented in this review. The crossing ventral tegmental tract (CVTT) could be the efferent tract of an "antigravitational" pathway terminating at the elevator muscle motoneurons in the third nerve nuclei and comprising, upstream, the superior vestibular nucleus and y-group, the flocculus, and the otoliths. This pathway functions in parallel to the medial longitudinal fasciculus pathways, which control vertical eye movements made to compensate for all vertical head movements and may also comprise the "gravitational" vestibular pathways, involved in the central reflection of the gravity effect. The CVTT could provide the upgaze system with the supplement of tonic activity required to counteract the gravity effect expressed in the gravitational pathway, being permanently modulated according to the static positions of the head (i.e., the instantaneous gravity vector) between a maximal activity in the upright position and a minimal activity in horizontal positions. Different types of arguments support this new model. The permanent influence of gravity on vertical eye position is strongly suggested by the vertical slow phases and nystagmus observed after rapid changes in hypo- or hypergravity. The chin-beating nystagmus, existing in normal subjects with their head in the upside-down position, suggests that gravity is not compensated for in the downgaze system. Upbeat nystagmus due to brainstem lesions, most likely affecting the CVTT circuitry, is improved when the head is in the horizontal position, suggesting that this circuitry is involved in the counteraction of gravity between the upright and horizontal positions of the head. In downbeat nystagmus due to floccular damage, in which a permanent hyperexcitation of the CVTT could exist, a marked influence of static positions of the head is also observed. Finally, the strongest argument supporting a marked role of gravity in vertical eye position is that the eye movement alterations observed in the main, typical physiological and pathological conditions are precisely those that would be expected from a direct effect of gravity on the eyeballs, with, moreover, no single alternative interpretation existing so far that could account for all these different types of findings.

Phenotypical Characteristics of Idiopathic Infantile Nystagmus with and without Mutations in "FRMD7"

ERIC Educational Resources Information Center

Thomas, Shery; Proudlock, Frank A.; Sarvananthan, Nagini; Roberts, Eryl O.; Awan, Musarat; McLean, Rebecca; Surendran, Mylvaganam; Kumar, A. S. Anil; Farooq, Shegufta J.; Degg, Chris; Gale, Richard P.; Reinecke, Robert D.; Woodruff, Geoffrey; Langmann, Andrea; Lindner, Susanne; Jain, Sunila; Tarpey, Patrick; Raymond, F. Lucy; Gottlob, Irene

2008-01-01

Idiopathic infantile nystagmus (IIN) consists of involuntary oscillations of the eyes. The familial form is most commonly X-linked. We recently found mutations in a novel gene "FRMD7" (Xq26.2), which provided an opportunity to investigate a genetically defined and homogeneous group of patients with nystagmus. We compared clinical features and eye…

Infantile nystagmus adapts to visual demand.

PubMed

Wiggins, Debbie; Woodhouse, J Margaret; Margrain, Tom H; Harris, Christopher M; Erichsen, Jonathan T

2007-05-01

To determine the effect of visual demand on the nystagmus waveform. Individuals with infantile nystagmus syndrome (INS) commonly report that making an effort to see can intensify their nystagmus and adversely affect vision. However, such an effect has never been confirmed experimentally. The eye movement behavior of 11 subjects with INS were recorded at different gaze angles while the subjects viewed visual targets under two conditions: above and then at resolution threshold. Eye movements were recorded by infrared oculography and visual acuity (VA) was measured using Landolt C targets and a two-alternative, forced-choice (2AFC) staircase procedure. Eye movement data were analyzed at the null zone for changes in amplitude, frequency, intensity, and foveation characteristics. Waveform type was also noted under the two conditions. Data from 11 subjects revealed a significant reduction in nystagmus amplitude (P < 0.05), frequency (P < 0.05), and intensity (P < 0.01) when target size was at visual threshold. The percentage of time the eye spent within the low-velocity window (i.e., foveation) significantly increased when target size was at visual threshold (P < 0.05). Furthermore, a change in waveform type with increased visual demand was exhibited by two subjects. The results indicate that increased visual demand modifies the nystagmus waveform favorably (and possibly adaptively), producing a significant reduction in nystagmus intensity and prolonged foveation. These findings contradict previous anecdotal reports that visual effort intensifies the nystagmus eye movement at the cost of visual performance. This discrepancy may be attributable to the lack of psychological stress involved in the visual task reported here. This is consistent with the suggestion that it is the visual importance of the task to the individual rather than visual demand per se which exacerbates INS. Further studies are needed to investigate quantitatively the effects of stress and psychological factors on INS waveforms.

Changes of ampulla pressure in the semicircular canal of pigeons by caloric stimulation

NASA Astrophysics Data System (ADS)

Wada, Yoshiro; Suzuki, Hiroyuki; Watanabe, Satoru

Still now several hypotheses about the mechanisms of the caloric nystagmus have been in conclusive. In this study we confirmed the convection effect and the volume change effect of the endolymph in horizontal semicircular canal following the caloric stimulation using pigeons ( Columba livia). Although the direction of the caloric nystagmus depended on the head position and the stimulus site of calorization, the caloric nystagmus disappeared after plugging of horizontal semicircular canal. On the other hand, the ampulla pressure increased by cold calorization and decreased by hot calorization and these pressure changes had no relation to the head position. These results show that the main role of the mechanisms of the caloric nystagmus under 1G is the convection effect but the volume change effect may act on the caloric nystagmus not only under 1G but also under microgravity.

Clinical course of persistent geotropic direction-changing positional nystagmus with neutral position-Light cupula.

PubMed

Seo, Toru; Shiraishi, Ko; Kobayashi, Takaaki; Mutsukazu, Kitano; Doi, Katsumi

2016-01-01

A short clinical course and frequent recurrence are common features of persistent geotropic direction-changing positional nystagmus with neutral position (positional nystagmus of light cupula: PNLC) and cupulolithiasis of the lateral semicircular canal. It is suggested that PNLC is caused by light debris attached to the cupula of the lateral semicircular canal. PNLC is a sub-type of direction-changing positional nystagmus. It is thought to be caused by anti-gravitational deviation of the cupula of the lateral semicircular canal (light cupula); however, the exact mechanism is yet to be elucidated. To this end, the clinical features of PNLC were studied. Clinical charts of 27 patients (13 men and 14 women) with PNLC were reviewed. The nystagmus had resolved within a week in 70% and within 30 days in 89% of the patients. The recurrence rate was 33%. The subjects did not have a history of alcohol intake, head trauma, or vestibular neuritis.

Influence of gravity on the spatial orientation of eye nystagmus induced by unilateral lesion of horizontal semicircular canal.

PubMed

Pettorossi, V E; Ermanno, M; Pierangelo, E; Silvarosa, G

2000-03-01

The influence of gravity in the orientation and slow phase eye velocity of the ocular nystagmus following unilateral damage of the cupula in the ampulla of the horizontal semicircular canal (UHCD) was investigated. The nystagmus was analysed at different sagittal head positions using the x-y infrared eye monitor technique. The nystagmus was almost horizontal at 0 degrees head pitch angle and remained partially fixed in space when the head was pitched upward or downward. The reorientation gain of the slow and quick phases was high (about 0.75) within +/- 45 degrees of head pitch angle, but beyond this range, it decreased greatly. The gain value depended on the lesion extension to otolithic receptors. The absolute value of the slow phase eye velocity of UHCD nystagmus was also modified systematically by the head pitch, showing a reduction in the upward and an increase in the downward.

Pharmacological tests of hypotheses for acquired pendular nystagmus.

PubMed

Shaikh, Aasef G; Thurtell, Matthew J; Optican, Lance M; Leigh, R John

2011-09-01

Acquired pendular nystagmus (APN) occurs with multiple sclerosis (MS) and oculopalatal tremor (OPT); distinct features of the nystagmus have led to the development of separate models for their pathogenesis. APN in MS has been attributed to instability in the neural integrator, which normally ensures steady gaze. APN in OPT may result from electrotonic coupling between neurons in the hypertrophied inferior olivary nucleus, which induces maladaptive learning in cerebellar cortex. We tested these two hypotheses by analyzing the effects of gabapentin, memantine, and baclofen on both forms of nystagmus. No drug changed the dominant frequency of either form of APN, but the variability of frequency was affected with gabapentin and memantine in patients with OPT. The amplitude of APN in both MS and OPT was reduced with gabapentin and memantine, but not baclofen. Analyzing the effects of drug therapies on ocular oscillations provides a novel approach to test models of nystagmus. © 2011 New York Academy of Sciences.

Pharmacological tests of hypotheses for acquired pendular nystagmus

PubMed Central

Shaikh, Aasef G.; Thurtell, Matthew J.; Optican, Lance M.; Leigh, R. John

2011-01-01

Acquired pendular nystagmus (APN) occurs with multiple sclerosis (MS) and oculopalatal tremor (OPT); distinct features of the nystagmus have led to the development of separate models for the pathogenesis. APN in MS has been attributed to instability in the neural integrator, which normally ensures steady gaze. APN in OPT may result from electrotonic coupling between neurons in the hypertrophied inferior olivary nucleus, which induces maladaptive learning in cerebellar cortex. We tested these two hypotheses by analyzing the effects of gabapentin, memantine, and baclofen on both forms of nystagmus. No drug changed the dominant frequency of either form of APN, but the variability of frequency was affected with gabapentin and memantine in patients with OPT. The amplitude of APN in both MS and OPT was reduced with gabapentin and memantine, but not baclofen. Analyzing the effects of drug therapies on ocular oscillations provides a novel approach to test models of nystagmus. PMID:21951011

An eye for an I: a 35-year-old woman with fluctuating oculomotor deficits and dissociative identity disorder.

PubMed

Bhuvaneswar, Chaya; Spiegel, David

2013-01-01

Physiologic changes, including neurological or pseudo-neurological symptoms, occur across identity states in dissociative identity disorder DID) and can be objectively measured. The idea that dissociative phenomena might be associated with changes in brain function is consistent with research on the brain effects of hypnosis. The authors report a case of psycho-physiologic differences among 4 alter personalities manifested by a 35-year-old woman with DID. Differences in visual acuity, frequency of pendular nystagmus, and handedness were observed in this patient both when the alter personalities appeared spontaneously and when elicited under hypnosis. The authors consider several diagnostic possibilities for these findings and discuss whether prevailing treatment recommendations for DID patients could possibly be modified to ameliorate such visual and neurologic symptoms.

[Dissociated nystagmus in side gaze. Major symptoms in the diagnosis of an internuclear ophthalmoplegia].

PubMed

Neugebauer, P; Neugebauer, A; Fricke, J; Michel, O

2004-07-01

A prerequisite for a qualified analysis of nystagmus is the recognition of uncommon forms of this condition. In internuclear ophthalmoplegia (INO), a dissociated nystagmus in side gaze is typical. This is accompanied by limited medial excursion of the adducted eye together with a dissociated nystagmus, which is stronger in the abducting fellow eye. This motility disturbance stems from a lesion in the medial longitudinal fasciculus running in the brain stem between the sixth and the third nerve nuclei. The lesion is often due to multiple sclerosis, but can also be ischemic, traumatic, neoplastic or inflammatory (e.g. HIV infection).

A probable cavernoma in the medulla oblongata presenting only as upbeat nystagmus.

PubMed

Choi, Hojin; Kim, Chang-Hun; Lee, Kyu-Yong; Lee, Young Joo; Koh, Seong-Ho

2011-11-01

A cavernoma is a vascular malformation in the central nervous system. Brainstem cavernoma are relatively common and induce variable neurological symptoms. A 19-year-old woman visited our hospital with complaints of continuous dizziness. On a neurological examination, continuous conjugated upbeat nystagmus was observed in the primary position of gaze. A brain CT scan and MRI showed focal hemorrhagic signals in the central caudal medulla caused by a cavernoma. The spontaneous upbeat nystagmus disappeared gradually. To our knowledge, this is the first report of a probable cavernoma in the medulla oblongata presenting with upbeat nystagmus only. Copyright © 2011 Elsevier Ltd. All rights reserved.

Visual suppression of the vestibulo-ocular reflex during space flight

NASA Technical Reports Server (NTRS)

Uri, John J.; Thornton, William E.; Moore, Thomas P.; Pool, Sam L.

1989-01-01

Visual suppression of the vestibulo-ocular reflex was studied in 16 subjects on 4 Space Shuttle missions. Eye movements were recorded by electro-oculography while subjects fixated a head mounted target during active sinusoidal head oscillation at 0.3 Hz. Adequacy of suppression was evaluated by the number of nystagmus beats, the mean amplitude of each beat, and the cumulative amplitude of nystagmus during two head oscillation cycles. Vestibulo-ocular reflex suppression was unaffected by space flight. Subjects with space motion sickness during flight had significantly more nystagmus beats than unaffected individuals. These susceptible subjects also tended to have more nystagmus beats before flight.

Downbeat nystagmus due to a paramedian medullary lesion.

PubMed

Nakamagoe, Kiyotaka; Shimizu, Kotone; Koganezawa, Tadachika; Tamaoka, Akira

2012-11-01

Cell groups of the paramedian tract, which are located in the paramedian region of the lower brainstem, are eye-movement-related neurons that project to the cerebellar flocculus. Their inactivation produces downbeat nystagmus, which resembles eye movement disorders resulting from lesions of the cerebellar flocculus in animal experiments. Therefore, paramedian tract cells are assumed to fulfill an important function in ocular movement control, such as gaze-holding and maintaining vestibular balance. This paper presents a 50-year-old female who manifested downbeat nystagmus due to damage to the paramedian tract cells caused by a localized ischemic lesion in the medulla oblongata. We found that a paramedian medullary lesion-induced nystagmus, similar to that observed following floccular lesions, clearly indicates that a subgroup of paramedian tract cells projecting to the flocculus was impaired. This finding has important implications in considering a brainstem-cerebellar feedback loop involved in vestibulo-oculomotor controls, such as vestibular balance. Although there have been a few reports of downbeat nystagmus caused by lesions in the midline region of the lower brainstem, to our knowledge none report the occurrence of nystagmus due to a strictly localized medullar lesion, such as the one described here. Copyright © 2012 Elsevier Ltd. All rights reserved.

Eye movement abnormalities in hermansky-pudlak syndrome.

PubMed

Gradstein, Libe; FitzGibbon, Edmond J; Tsilou, Ekaterini T; Rubin, Benjamin I; Huizing, Marjan; Gahl, William A

2005-08-01

Hermansky-Pudlak Syndrome (HPS) is a type of oculocutaneous albinism associated with a bleeding diathesis and pulmonary fibrosis. Although it is known that patients with HPS exhibit nystagmus, the nature of these abnormal eye movements has not been studied. Twenty-seven patients with HPS, diagnosed by platelet morphology and genetic analysis, underwent a systemic evaluation and complete eye examination. Twenty-five had eye movement recordings using magnetic search coil, infrared, or video oculography. All patients had iris transillumination, foveal hypoplasia, and variable hypopigmentation in skin and eyes. All had bleeding tendencies, and 2 reported excessive bleeding during strabismus surgery. Nine patients had pulmonary fibrosis. Visual acuities ranged from 20/20- to 20/320. Twenty patients had strabismus despite 6 having strabismus surgery previously. Ocular oscillations consistent with congenital nystagmus (CN) were clinically evident in 24 of 27 patients, and half showed periodic alternating nystagmus. In 3 patients without CN, eye movement recordings revealed minimal end-gaze nystagmus, square-wave jerks, drift during fixation and saccades, and low-gain pursuit. These patients had melanin in the posterior pole and better visual acuities than the others (P = 0.002). Most patients with HPS have CN, and many have periodic alternating nystagmus. Some have subtle eye movement abnormalities without clinically evident nystagmus, which can obscure the diagnosis, especially if hypopigmentation is mild. Absence of clinical nystagmus in a child with HPS suggests good vision. Patients with albinism, especially before surgery, should be evaluated for HPS to prevent life-threatening complications.

Infantile Nystagmus and Abnormalities of Conjugate Eye Movements in Down Syndrome.

PubMed

Weiss, Avery H; Kelly, John P; Phillips, James O

2016-03-01

Subjects with Down syndrome (DS) have an anatomical defect within the cerebellum that may impact downstream oculomotor areas. This study characterized gaze holding and gains for smooth pursuit, saccades, and optokinetic nystagmus (OKN) in DS children with infantile nystagmus (IN). Clinical data of 18 DS children with IN were reviewed retrospectively. Subjects with constant strabismus were excluded to remove any contribution of latent nystagmus. Gaze-holding, horizontal and vertical saccades to target steps, horizontal smooth pursuit of drifting targets, OKN in response to vertically or horizontally-oriented square wave gratings drifted at 15°/s, 30°/s, and 45°/s were recorded using binocular video-oculography. Seven subjects had additional optical coherence tomography imaging. Infantile nystagmus was associated with one or more gaze-holding instabilities (GHI) in each subject. The majority of subjects had a combination of conjugate horizontal jerk with constant or exponential slow-phase velocity, asymmetric or symmetric, and either monocular or binocular pendular nystagmus. Six of seven subjects had mild (Grade 0-1) persistence of retinal layers overlying the fovea, similar to that reported in DS children without nystagmus. All subjects had abnormal gains across one or more stimulus conditions (horizontal smooth pursuit, saccades, or OKN). Saccade velocities followed the main sequence. Down syndrome subjects with IN show a wide range of GHI and abnormalities of conjugate eye movements. We propose that these ocular motor abnormalities result from functional abnormalities of the cerebellum and/or downstream oculomotor circuits, perhaps due to extensive miswiring.

Effect of Gabapentin/Memantine on the Infantile Nystagmus Syndrome in the Zebrafish Model: Implications for the Therapy of Ocular Motor Diseases.

PubMed

Bögli, Stefan Yu; Afthinos, Maresa; Huang, Melody Ying-Yu

2017-06-01

Infantile nystagmus syndrome (INS) is a disorder characterized by typical horizontal eye oscillations. Due to the uncertain etiology of INS, developing specific treatments remains difficult. Single reports demonstrated, on limited measures, alleviating effects of gabapentin and memantine. In the current study, we employed the zebrafish INS model belladonna (bel) to conduct an in-depth study of how gabapentin and memantine interventions alleviate INS signs, which may further restore visual conditions in affected subjects. Moreover, we described the influence of both medications on ocular motor functions in healthy zebrafish, evaluating possible iatrogenic effects. Ocular motor function and INS characteristics were assessed by eliciting optokinetic response, spontaneous nystagmus, and spontaneous saccades in light and in dark, in 5- to 6-day postfertilization bel larvae and heterozygous siblings. Single larvae were recorded before and after a 1-hour drug treatment (200 mM gabapentin/0.2 mM memantine). Both interventions significantly reduced nystagmus intensity (gabapentin: 59.98%, memantine: 39.59%). However, while the application of gabapentin affected all tested ocular motor functions, memantine specifically reduced nystagmus amplitude and intensity, and thus left controls completely unaffected. Finally, both drug treatments resulted in specific changes in nystagmus waveform and velocity. Our study provides deeper insight into gabapentin and memantine treatment effect in the zebrafish INS model. Moreover, this study should establish zebrafish as a pharmacologic animal model for treating nystagmus and ocular motor disease, serving as a basis for future large-scale drug screenings.

Oculomotor Deficits in Aryl Hydrocarbon Receptor Null Mouse

PubMed Central

Chevallier, Aline; Mialot, Antoine; Petit, Jean-Maurice; Fernandez-Salguero, Pedro; Barouki, Robert

2013-01-01

The Aryl hydrocarbon Receptor or AhR, a ligand-activated transcription factor, is known to mediate the toxic and carcinogenic effects of various environmental pollutants such as 2,3,7,8-Tetrachlorodibenzo-p-dioxin (TCDD). Recent studies in Caenorhabditis elegans and Drosophila melanogaster show that the orthologs of the AhR are expressed exclusively in certain types of neurons and are implicated in the development and the homeostasis of the central nervous system. While physiological roles of the AhR were demonstrated in the mammalian heart, liver and gametogenesis, its ontogenic expression and putative neural functions remain elusive. Here, we report that the constitutive absence of the AhR in adult mice (AhR−/−) leads to abnormal eye movements in the form of a spontaneous pendular horizontal nystagmus. To determine if the nystagmus is of vestibular, visual, or cerebellar origin, gaze stabilizing reflexes, namely vestibulo-ocular and optokinetic reflexes (VOR and OKR), were investigated. The OKR is less effective in the AhR−/− mice suggesting a deficit in the visuo-motor circuitry, while the VOR is mildly affected. Furthermore, the AhR is expressedin the retinal ganglion cells during the development, however electroretinograms revealed no impairment of retinal cell function. The structure of the cerebellum of the AhR−/− mice is normal which is compatible with the preserved VOR adaptation, a plastic process dependent on cerebellar integrity. Finally, intoxication with TCDD of control adults did not lead to any abnormality of the oculomotor control. These results demonstrate that the absence of the AhR leads to acquired central nervous system deficits in the adults. Given the many common features between both AhR mouse and human infantile nystagmus syndromes, the AhR−/− mice might give insights into the developmental mechanisms which lead to congenital eye disorders. PMID:23301081

Abnormal Vestibulo-Ocular Reflexes in Autism: A Potential Endophenotype

DTIC Science & Technology

2013-06-01

Annual Report for 15 May 2012 – 14 May 2013 8 Table 5. Summary of Gaze Evoked Nystagmus Tests (no differences between groups) Target...abnormalities of vestibulo-ocular reflexes (VOR) in Autism Spectrum Disorder (ASD). Specific Aim 1: Characterize horizontal VOR post-rotary nystagmus ...without optokinetic feedback using a velocity step test. We hypothesize that in ASD vertical eye movement intrusions during horizontal nystagmus will

Rebound nystagmus: EOG analysis of a case with a floccular tumour.

PubMed Central

Yamazaki, A; Zee, D S

1979-01-01

Eye movements were recorded and quantitatively analysed in a patient with a tumour initially involving the cerebellar flocculus. Ocular motor abnormalities included (1) impaired smooth pursuit, (2) impaired cancellation of the vestibulo-ocular reflex when fixating an object rotating with the head, and (3) gaze paretic and rebound nystagmus. Comparable findings have been reported in monkeys with experimental floccular lesions. The rebound nystagmus (but not the other ocular motor abnormalities) disappeared when the tumour appeared to invade the brain stem in the region near the vestibular nuclei. This finding suggests that the floccular lesion unmasked a bias which created rebound nystagmus and that the bias probably arose in the vestibular nuclei. PMID:508695

Finger extension weakness and downbeat nystagmus motor neuron disease syndrome: A novel motor neuron disorder?

PubMed

Delva, Aline; Thakore, Nimish; Pioro, Erik P; Poesen, Koen; Saunders-Pullman, Rachel; Meijer, Inge A; Rucker, Janet C; Kissel, John T; Van Damme, Philip

2017-12-01

Disturbances of eye movements are infrequently encountered in motor neuron diseases (MNDs) or motor neuropathies, and there is no known syndrome that combines progressive muscle weakness with downbeat nystagmus. To describe the core clinical features of a syndrome of MND associated with downbeat nystagmus, clinical features were collected from 6 patients. All patients had slowly progressive muscle weakness and wasting in combination with downbeat nystagmus, which was clinically most obvious in downward and lateral gaze. Onset was in the second to fourth decade with finger extension weakness, progressing to other distal and sometimes more proximal muscles. Visual complaints were not always present. Electrodiagnostic testing showed signs of regional motor axonal loss in all patients. The etiology of this syndrome remains elusive. Because finger extension weakness and downbeat nystagmus are the discriminating clinical features of this MND, we propose the name FEWDON-MND syndrome. Muscle Nerve 56: 1164-1168, 2017. © 2017 The Authors Muscle & Nerve Published by Wiley Periodicals, Inc.

Finger extension weakness and downbeat nystagmus motor neuron disease syndrome: A novel motor neuron disorder?

PubMed Central

Delva, Aline; Thakore, Nimish; Pioro, Erik P.; Poesen, Koen; Saunders‐Pullman, Rachel; Meijer, Inge A.; Rucker, Janet C.; Kissel, John T.

2017-01-01

ABSTACT Introduction: Disturbances of eye movements are infrequently encountered in motor neuron diseases (MNDs) or motor neuropathies, and there is no known syndrome that combines progressive muscle weakness with downbeat nystagmus. Methods: To describe the core clinical features of a syndrome of MND associated with downbeat nystagmus, clinical features were collected from 6 patients. Results: All patients had slowly progressive muscle weakness and wasting in combination with downbeat nystagmus, which was clinically most obvious in downward and lateral gaze. Onset was in the second to fourth decade with finger extension weakness, progressing to other distal and sometimes more proximal muscles. Visual complaints were not always present. Electrodiagnostic testing showed signs of regional motor axonal loss in all patients. Discussion: The etiology of this syndrome remains elusive. Because finger extension weakness and downbeat nystagmus are the discriminating clinical features of this MND, we propose the name FEWDON‐MND syndrome. Muscle Nerve 56: 1164–1168, 2017 PMID:28440863

A Review of Motion Sickness with Special Reference to Simulator Sickness

DTIC Science & Technology

1985-04-15

Harris & Graybiel’, 1964). Notable exceptions are the loss in visual acuity and tracking problems associated with vestibular nystagmus when the visual...Dilated pupils during qmesis. Small pupils. Nystagmus . Adapted from Nicogossian & Parker. 1982. $ 𔃽.3.> , NAVTRAEQUIPCEN 81-C-0105-16 probably will...E., Crampton. W. E., & Posner, J. B. Effects of mental activity on vestibular nystagmus and the electro- encephalogram. Nature, 1961, 190, 194-195

Analysis of Spatial Disorientation Mishaps in the US Navy

DTIC Science & Technology

2003-02-01

optokinetic after- nystagmus (OKAN) and vestibular nystagmus . In: Baker R, Berthoz A, eds. Control of gaze by grain stem neurons, Amsterdam: Elsevier...of explaining by modeling. In: Baker R, Berthoz A, eds. Control of gaze by grain stem neurons, developments in neuroscience, Vol. 1. Amsterdam...Elsevier/North-Holland Biomedical Press, 49-58. Raphan T, Matsuo V, Cohen B. (1977) A velocity storage mechanism responsible for optokinetic nystagmus (OKN

See-saw nystagmus and brainstem infarction: MRI findings

NASA Technical Reports Server (NTRS)

Kanter, D. S.; Ruff, R. L.; Leigh, R. J.; Modic, M.

1987-01-01

A patient with see-saw nystagmus had a lesion localized by Magnetic Resonance Imaging (MRI) to the paramedian ventral midbrain with involvement of the right interstitial nucleus of Cajal. This the first MRI study of see-saw nystagmus associated with a presumed brainstem vascular event. Our findings support animal and human studies suggesting that dysfunction of the interstitial nucleus of Cajal or its connections is central in this disorder.

A case report of HTLV-I associated myelopathy presenting with cerebellar ataxia and nystagmus.

PubMed

Taki, Masakatsu; Nin, Fumiaki; Hasegawa, Tatsuhisa; Sakaguchi, Hirofumi; Suzuki, Toshihiro; Hisa, Yasuo; Azuma, Yumiko; Nakagawa, Masanori

2011-06-01

HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is characterized by spastic paraparesis in the lower extremities, and urinary disturbance. HAM/TSP has also been less frequently associated with cerebellar syndromes and nystagmus. We report a case of HAM/TSP presenting with cerebellar ataxia and nystagmus. The patient was a 73-year-old woman who was born in southern Japan. At age 41, she developed pain and spasticity in the bilateral lower limbs and gradually progressive gait disturbance. At age 57, she was diagnosed with HAM/TSP based on spastic paraparesis in the lower limbs, urinary disturbance and positive anti HTLV-I antibody in serum and cerebrospinal fluid. In June 2008, she was referred to our university and hospitalized for rehabilitation. Twenty days later, she experienced rotatory vertigo sensation. Magnetic resonance imaging revealed pontocerebellar atrophy. The patient presented with cerebellar signs in the upper limbs, gaze-evoked nystagmus in the sitting position and right-beating horizontal nystagmus in the supine and head-hanging positions. Electronystagmography (ENG) showed horizontal saccadic overshoot dysmetria and horizontal saccadic pursuit. Nystagmus is rare among the literature on HAM/TSP. ENG is helpful to evaluate and confirm the cerebellar syndromes of HAM/TSP. Copyright © 2010. Published by Elsevier Ireland Ltd.

Effects of tenotomy on patients with infantile nystagmus syndrome: foveation improvement over a broadened visual field.

PubMed

Wang, Zhong; Dell'Osso, Louis F; Jacobs, Jonathan B; Burnstine, Robert A; Tomsak, Robert L

2006-12-01

To investigate the effects of four-muscle tenotomy on visual function and gaze angle in patients with infantile nystagmus syndrome (INS). Eye movements of nine patients with infantile nystagmus were recorded using infrared reflection or high-speed digital video techniques. Experimental protocols were designed to record the patients' eye-movement waveforms, pre- and post-tenotomy, at different gaze angles. We used the eXpanded Nystagmus Acuity Function (NAFX) to measure tenotomy-induced changes in the nystagmus at primary position and various gaze angles. The longest foveation domains (LFD) were measured from fitted curves. Peak-to-peak nystagmus amplitudes and foveation-period durations were also measured. All measurements were made unmasked. All seven patients with narrow, high-NAFX, gaze-angle regions showed broadening of these regions of higher visual function. Three patients showed moderate NAFX improvement (13.9-32.6%) at primary position, five showed large improvement (39.9-162.4%), and one showed no NAFX change (due to his high pretenotomy NAFX). Primary position measured acuities improved in six patients. All patients had reductions in nystagmus amplitudes ranging from 14.6 to 37%. The duration of the foveation period increased in all nine patients (11.2-200%). The percentage improvements in both the NAFX and the LFD decreased with higher pretenotomy values. In addition to elevating primary position NAFX, tenotomy also broadens the high-NAFX regions. This broadening effect is more prominent in patients who had sharp pretenotomy NAFX peaks. Four-muscle tenotomy produces higher primary position NAFX increases in infantile nystagmus patients whose pretenotomy values are relatively low, with the improvement decreasing at higher pretenotomy values. The tenotomy procedure improves visual function beyond primary position acuity. This extends the utility of surgical therapy to several different classes of patients with INS for whom other procedures are contraindicated. The pretenotomy NAFX can now be used to predict both primary position acuity improvements and broadening of a patient's high-NAFX range of gaze angles.

Correlation between vestibulo-ocular reflex and optokinetic afternystagmus in normal subjects and in patients with vestibular system disorders

PubMed Central

Dellepiane, M; Medicina, MC; Barettini, L; Mura, AC

2006-01-01

Summary Optokinetic afternystagmus follows optokinetic nystagmus as an expression of the central velocity storage integrator discharge and its fast phase is beating in the same direction as the previous optokinetic nystagmus. We investigated the correlation between vestibulo-ocular reflex and optokinetic afternystagmus in normal subjects and in patients with bilateral vestibular disorders. The aim of this study was to determine the possible role of optokinetic afternystagmus as a diagnostic test for identifying functional vestibular disorders. The subjects were examined by electronystagmography and vestibulo-ocular reflex, optokinetic nystagmus stare type as well as optokinetic afternystagmus were recorded. They were restrained in a rotatory drum chair, both the chair and the drum could be rotated, independently or coupled. For vestibulo-ocular reflex analysis, we studied post-rotatory-nystagmus from a velocity of 90°s. Optokinetic nystagmus was recorded at a drum velocity of 30°s and the registration continued in total darkness, after the illumination was switched off, to study optokinetic afternystagmus. We considered vestibulo-ocular reflex and optokinetic nystagmus gain, vestibulo-ocular reflex and optokinetic afternystagmus constant of time (tc) defined as the time necessary for the slow phase eye velocity to be reduced to 37% of its initial value. Results demonstrated that vestibulo-ocular reflex gain and ct showed a significant difference only in patients with reduced vestibular reflexia, while optokinetic nystagmus gain was greater only in patients with increased reflexia; optokinetic afternystagmus ct was different from the control group only in patients with hyporeflexia. In conclusion, our results suggest that vestibulo-ocular reflex and optokinetic afternystagmus ct are clinically more useful than the gain alone in testing vestibular disorders with hyporeflexia. On the other hand, we propose a new mathematical and statistical approach to study the temporal evolution of more parameters of the nystagmus. PMID:18383753

Oculomotor Reflexes as a Test of Visual Dysfunctions in Cognitively Impaired Observers

DTIC Science & Technology

2013-09-01

right. Gaze horizontal position is plotted along the y-axis. The red bar indicates a visual nystagmus event detected by the filter. (d) A mild curse word...experimental conditions were chosen to simulate testing cognitively impaired observers. Reflex Stimulus Functions Visual Nystagmus luminance grating low-level...developed a new stimulus for visual nystagmus to 8 test visual motion processing in the presence of incoherent motion noise. The drifting equiluminant

Oculomotor Reflexes as a Test of Visual Dysfunctions in Cognitively Impaired Observers

DTIC Science & Technology

2012-10-01

visual nystagmus much more robust. Because the absolute gaze is not measured in our paradigm (this would require a gaze calibration, involving...the dots were also drifting to the right. Gaze horizontal position is plotted along the y-axis. The red bar indicates a visual nystagmus event...for automated 5 Reflex Stimulus Functions Visual Nystagmus luminance grating low-level motion equiluminant grating color vision contrast gratings at 3

An Experimental Evaluation of a Field Sobriety Test Battery in the Marine Environment

DTIC Science & Technology

1990-06-01

Turn, Horizontal Gaze Nystagmus , Finger to Nose, Finger Count, and Tracing. Of these six tests, Walk and Turn, One-Leg Stand, and Horizontal Gaze ...served as the lead officer, administering the tests while the other two officers observed. All officers administered the Horizontal Gaze Nystagmus ...administered the Horizontal Gaze Nystagmus (HGN) individually. After giving a tes’ or pair of tests (as designated) each officer on the team gave a

Upbeat nystagmus in anti-Ma2 encephalitis.

PubMed

Garcia-Reitboeck, Pablo; Thompson, Graham; Johns, Paul; Al Wahab, Yasir; Omer, Salah; Griffin, Colette

2014-02-01

Anti-Ma2 encephalitis is a paraneoplastic disorder characterised by brainstem and/or limbic involvement. Eye movement abnormalities can occur in this condition, often with confusion or somnolence. We describe a patient with progressive oscillopsia (with upbeat nystagmus) and unsteadiness, followed by acute pancreatitis. She did not respond to immunomodulatory treatment and subsequently died of complications related to pancreatitis and sepsis. There was no tumour identified at autopsy, but the anti-Ma2 antibodies in her serum and the discovery of a brainstem-predominant inflammatory infiltrate at autopsy strongly suggest a paraneoplastic disorder. Our case illustrates that upbeat nystagmus can be a predominant feature in anti-Ma2 encephalitis; clinicians should consider testing for anti-Ma2 antibodies in patients with upbeat nystagmus of unknown cause.

Dark as a dungeon - The rise and fall of coal miners' nystagmus

DOE Office of Scientific and Technical Information (OSTI.GOV)

Fishman, R.S.

2006-11-15

Coal miners' nystagmus was one of the first occupational illnesses ever recognized as being due to a hazardous working environment. It aroused great concern and much controversy in Great Britain in the first half of the 20th century but was not seen in the United States. Miners' nystagmus became a significant financial problem for the British workmen's compensation program, and the British medical literature became a forum for speculation as to the nature of the condition. Although new cases of miners' nystagmus were rare after World War II, the condition continued to be discussed in textbooks through the 1970s, aftermore » which it abruptly disappeared without any authoritative summing-up, and thereby hangs a tale.« less

Cross-Over Trial of Gabapentin and Memantine as Treatment for Acquired Nystagmus

PubMed Central

Thurtell, Matthew J.; Joshi, Anand C.; Leone, Alice C.; Tomsak, Robert L.; Kosmorsky, Gregory S.; Stahl, John S.; Leigh, R. John

2010-01-01

We conducted a masked, cross-over, therapeutic trial of gabapentin (1200mg/day) versus memantine (40mg/day) for acquired nystagmus in 10 patients (28–61 years; 7 female; MS: 3, post-stroke: 6, post-traumatic: 1). Nystagmus was pendular in 6 patients (oculopalatal tremor: 4, MS: 2) and jerk upbeat, hemi-seesaw, torsional, or upbeat-diagonal in each of the others. Both drugs reduced median eye speed (p<0.001), gabapentin by 32.8% and memantine by 27.8%, and improved visual acuity (p<0.05). Each patient improved with one or both drugs. Side-effects included unsteadiness with gabapentin and lethargy with memantine. Both drugs should be considered as treatment for acquired forms of nystagmus. PMID:20437565

[Reduced vision in Wernicke's syndrome with symptoms of nystagmus].

PubMed

Lindholt, Michael E

2006-04-03

A 42-year-old man presented in the Department of Ophthalmology of Holstebro Central Hospital with a history of alcohol abuse, vomiting and weight loss. The initial symptoms of beriberi were stiffness of the legs and a picture of Wernicke's syndrome. He had bilateral loss of horizontal eccentric gaze holding and upbeat nystagmus, and visual acuity was reduced to counting fingers. Visual acuity was tested in the down-gaze position and increased to 0.3 because of reduced nystagmus. The patient's oculomotor function improved dramatically after treatment with thiamine. Wernicke's encephalopathy and beriberi are discussed, highlighting that nystagmus may be the single ocular symptom. It should be considered in the differential diagnosis of bilateral ophthalmoplegia even in the absence of altered mental status.

Comparative assessment of prognosis of the stop stimulus and trapezoidal rotation programs

NASA Technical Reports Server (NTRS)

Grigorova, V. K.; Popov, V. K.; Todorova, V. S.

1980-01-01

For prognosis of the diagnostic possibilities of the stop stimulus and trapezoidal rotation programs with respect to the nystagmus response, 24 healthy young persons with normal auditory and vestibular analysers were studied experimentally. The trapezoidal program more accurately reflects the function and tone balance of the vestibular system than the stop stimulus program and causes the subject no unpleasant sensations during the study. Some optimum couples, acceleration and armchair rotation rate, necessary for effective deviation of the cupuloendolymphatic system were determined. The maximum angular velocity of the slow nystagmus component was more informative than nystagmus duration. The trapezoidal program is recommended for otoneurological practice and the maximum angular velocity of the slow nystagmus component as the basic index.

A gene for nystagmus-associated episodic ataxia maps to chromosome 19p

DOE Office of Scientific and Technical Information (OSTI.GOV)

Kramer, P.L.; Root, D.; Gancher, S.

1994-09-01

Episodic ataxia (EA) is a rare, autosomal dominant disorder, characterized by attacks of generalized ataxia and relatively normal neurological function between attacks. Onset occurs in childhood or adolescence and persists through adulthood. Penetrance is nearly complete. EA is clinically heterogeneous, including at least two distinct entities: (1) episodes of ataxia and dysarthria lasting hours to days, generally with interictal nystagmus (MIM 108500); (2) episodes of ataxia and dysarthria lasting only minutes, with interictal myokymia (MMM 160120). The EA/nystagmus patients sometimes develop persistent ataxia and cerebellar atrophy. Previously we reported linkage in four EA/myokymia families to a K{sup +} channel genemore » on chromosome 12p. We excluded this region in a large family with EA/nystagmus. We now report evidence for linkage to chromosome 19p in this and in one other EA/nystagmus family, based on eight microsatellite markers which span approximately 30 cM. The region is flanked distally by D19S209 and proximally by D19S226. All six markers within this region gave positive evidence for linkage; the highest total two-point lod scores occurred wtih D19S221 (3.98 at theta = 0.10) and D19S413 (3.37 at theta = 0.05). Interestingly, Joutel et al. (1993) mapped a gene for familial hemiplegic migraine (FHM) to the region around D19S221. Some individuals in these families have ataxia, cerebellar atrophy and interictal nystagmus, but no episodic ataxia. These results demonstrate that the clinical heterogeneity in EA reflects underlying genetic hetreogeneity. In addition, they suggest that EA/nystagmus and some FHM may represent different mutations in the same gene locus on chromosome 19p.« less

Ocular motor characteristics of different subtypes of spinocerebellar ataxia: distinguishing features.

PubMed

Kim, Ji Sun; Kim, Ji Soo; Youn, Jinyoung; Seo, Dae-Won; Jeong, Yuri; Kang, Ji-Hoon; Park, Jeong Ho; Cho, Jin Whan

2013-08-01

Because of frequent involvement of the cerebellum and brainstem, ocular motor abnormalities are key features of spinocerebellar ataxias and may aid in differential diagnosis. Our objective for this study was to distinguish the subtypes by ophthalmologic features after head-shaking and positional maneuvers, which are not yet recognized as differential diagnostic tools in most common forms of spinocerebellar ataxias. Of the 302 patients with a diagnosis of cerebellar ataxia in 3 Korean University Hospitals from June 2011 to June 2012, 48 patients with spinocerebellar ataxia types 1, 2, 3, 6, 7, or 8 or with undetermined spinocerebellar ataxias were enrolled. All patients underwent a video-oculographic recording of fixation abnormalities, gaze-evoked nystagmus, positional and head-shaking nystagmus, and dysmetric saccades. Logistic regression analysis controlling for disease duration revealed that spontaneous and positional downbeat nystagmus and perverted head-shaking nystagmus were strong predictors for spinocerebellar ataxia 6, whereas saccadic intrusions and oscillations were identified as positive indicators of spinocerebellar ataxia 3. In contrast, the presence of gaze-evoked nystagmus and dysmetric saccades was a negative predictor of spinocerebellar ataxia 2. Positional maneuvers and horizontal head shaking occasionally induced or augmented saccadic intrusions/oscillations in patients with spinocerebellar ataxia types 1, 2, and 3 and undetermined spinocerebellar ataxia. The results indicated that perverted head-shaking nystagmus may be the most sensitive parameter for SCA6, whereas saccadic intrusions/oscillations are the most sensitive for spinocerebellar ataxia 3. In contrast, a paucity of gaze-evoked nystagmus and dysmetric saccades is more indicative of spinocerebellar ataxia 2. Head-shaking and positional maneuvers aid in defining ocular motor characteristics in spinocerebellar ataxias. © 2013 Movement Disorder Society. Copyright © 2013 Movement Disorder Society.

Autosomal-dominant nystagmus, foveal hypoplasia and presenile cataract associated with a novel PAX6 mutation.

PubMed

Thomas, Shery; Thomas, Mervyn G; Andrews, Caroline; Chan, Wai-Man; Proudlock, Frank A; McLean, Rebecca J; Pradeep, Archana; Engle, Elizabeth C; Gottlob, Irene

2014-03-01

Autosomal-dominant idiopathic infantile nystagmus has been linked to 6p12 (OMIM 164100), 7p11.2 (OMIM 608345) and 13q31-q33 (OMIM 193003). PAX6 (11p13, OMIM 607108) mutations can also cause autosomal-dominant nystagmus, typically in association with aniridia or iris hypoplasia. We studied a large multigenerational white British family with autosomal-dominant nystagmus, normal irides and presenile cataracts. An SNP-based genome-wide analysis revealed a linkage to a 13.4-MB region on chromosome 11p13 with a maximum lod score of 2.93. A mutation analysis of the entire coding region and splice junctions of the PAX6 gene revealed a novel heterozygous missense mutation (c.227C>G) that segregated with the phenotype and is predicted to result in the amino-acid substitution of proline by arginine at codon 76 p.(P76R). The amino-acid variation p.(P76R) within the paired box domain is likely to destabilise the protein due to steric hindrance as a result of the introduction of a polar and larger amino acid. Eye movement recordings showed a significant intrafamilial variability of horizontal, vertical and torsional nystagmus. High-resolution in vivo imaging of the retina using optical coherence tomography (OCT) revealed features of foveal hypoplasia, including rudimentary foveal pit, incursion of inner retinal layers, short photoreceptor outer segments and optic nerve hypoplasia. Thus, this study presents a family that segregates a PAX6 mutation with nystagmus and foveal hypoplasia in the absence of iris abnormalities. Moreover, it is the first study showing detailed characteristics using eye movement recordings of autosomal-dominant nystagmus in a multigenerational family with a novel PAX6 mutation.

Tonic Investigation Concept of Cervico-vestibular Muscle Afferents

PubMed Central

Dorn, Linda Josephine; Lappat, Annabelle; Neuhuber, Winfried; Scherer, Hans; Olze, Heidi; Hölzl, Matthias

2016-01-01

Introduction Interdisciplinary research has contributed greatly to an improved understanding of the vestibular system. To date, however, very little research has focused on the vestibular system's somatosensory afferents. To ensure the diagnostic quality of vestibular somatosensory afferent data, especially the extra cranial afferents, stimulation of the vestibular balance system has to be precluded. Objective Sophisticated movements require intra- and extra cranial vestibular receptors. The study's objective is to evaluate an investigation concept for cervico-vestibular afferents with respect to clinical feasibility. Methods A dedicated chair was constructed, permitting three-dimensional trunk excursions, during which the volunteer's head remains fixed. Whether or not a cervicotonic provocation nystagmus (c-PN) can be induced with static trunk excursion is to be evaluated and if this can be influenced by cervical monophasic transcutaneous electrical nerve stimulation (c-TENS) with a randomized test group. 3D-video-oculography (VOG) was used to record any change in cervico-ocular examination parameters. The occurring nystagmuses were evaluated visually due to the small caliber of nystagmus amplitudes in healthy volunteers. Results The results demonstrate: no influence of placebo-controlled c-TENS on the spontaneous nystagmus; a significant increase of the vertical nystagmus on the 3D-trunk-excursion chair in static trunk flexion with cervical provocation in all young healthy volunteers (n = 49); and a significant difference between vertical and horizontal nystagmuses during static trunk excursion after placebo-controlled c-TENS, except for the horizontal nystagmus during trunk torsion. Conclusion We hope this cervicotonic investigation concept on the 3D trunk-excursion chair will contribute to new diagnostic and therapeutic perspectives on cervical pathologies in vestibular head-to-trunk alignment. PMID:28050208

Audiovestibular impairments associated with intracranial hypotension.

PubMed

Choi, Jae-Hwan; Cho, Kee-Yong; Cha, Seung-Yi; Seo, Jae-Deuk; Kim, Min-Ji; Choi, Yu Ri; Kim, Sung-Hee; Kim, Ji-Soo; Choi, Kwang-Dong

2015-10-15

To investigate the patterns and mechanisms of audiovestibular impairments associated with intracranial hypotension. We had consecutively recruited 16 patients with intracranial hypotension at the Neurology Center of Pusan National University Hospital for two years. Spontaneous, gaze-evoked, and positional nystagmus were recorded using 3D video-oculography in all patients, and the majority of them also had pure tone audiometry and bithermal caloric tests. Of the 16 patients, five (31.3%) reported neuro-otological symptoms along with the orthostatic headache while laboratory evaluation demonstrated audiovestibular impairments in ten (62.5%). Oculographic analyses documented spontaneous and/or positional nystagmus in six patients (37.5%) including weak spontaneous vertical nystagmus with positional modulation (n=4) and pure positional nystagmus (n=2). One patient presented with recurrent spontaneous vertigo and tinnitus mimicking Meniere's disease, and showed unidirectional horizontal and torsional nystagmus with normal head impulse tests during the attacks. Bithermal caloric tests were normal in all nine patients tested. Audiometry showed unilateral (n=6) or bilateral (n=1) sensorineural hearing loss in seven (53.8%) of the 13 patients tested. Intracranial hypotension frequently induces audiovestibular impairments. In addition to endolymphatic hydrops and irritation of the vestibulocochlear nerve, compression or traction of the brainstem or cerebellum due to loss of CSF buoyancy may be considered as a mechanism of frequent spontaneous or positional vertical nystagmus in patients with intracranial hypotension. Copyright © 2015 Elsevier B.V. All rights reserved.

Nystagmus-based approach to vertebrobasilar stroke presenting as vertigo without initial neurologic signs.

PubMed

Kim, Min-Beom; Boo, Sung Hyun; Ban, Jae Ho

2013-01-01

We aimed to investigate the clinical courses and common nystagmus of isolated vertigo patients with vertebrobasilar stroke. The patients who presented with isolated acute spontaneous vertigo with spontaneous nystagmus (acute vestibular syndrome) at the Emergency Department were retrospectively analyzed. They were referred to the Otolaryngology Department due to the absence of neurologic signs or even of imaging abnormalities after the initial examination at the Emergency Department. Various clinical features, including presenting symptoms, delayed neurologic signs, the site of infarction, and videonystagmographic (VNG) findings were analyzed. Of the 468 cases of acute vestibular syndrome, 23 (4.9%) cases of radiologically proven vertebrobasilar stroke were identified. Of the 23 patients, 17 (74%) showed aggravation of vertigo or delayed neurologic signs during the admission. In the analysis of VNG, 11 (48%) cases of direction-changing gaze-evoked nystagmus, 7 (30%) cases of fixation failure in the caloric test, 6 (27%) cases of periodic alternating nystagmus, and 4 (17%) cases of atypical head-shaking nystagmus were presented. Stroke occurred in the cerebellum (n=18, 78%), medulla (n=4, 17%), and pons (n=1, 4%). In the early stage of vertebrobasilar stroke, an accurate diagnosis was difficult in the Emergency Department even though a radiologic study was performed, but various VNG abnormalities and delayed neurologic signs could help to diagnose whether the origin is central or not. Copyright © 2013 S. Karger AG, Basel.

Positional and positioning down-beating nystagmus without central nervous system findings.

PubMed

Ogawa, Yasuo; Suzuki, Mamoru; Otsuka, Koji; Shimizu, Shigetaka; Inagaki, Taro; Hayashi, Mami; Hagiwara, Akira; Kitajima, Naoharu

2009-12-01

We report the clinical features of 4 cases with positional or positioning down-beating nystagmus in a head-hanging or supine position without any obvious central nervous system disorder. The 4 cases had some findings in common. There were no abnormal findings on neurological tests or brain MRI. They did not have gaze nystagmus. Their nystagmus was observed only in a supine or head-hanging position and it was never observed upon returning to a sitting position and never reversed. The nystagmus had no or little torsional component, had latency and tended to decrease with time. The positional DBN (p-DBN) is known to be indicative of a central nervous system disorder. Recently there were some reports that canalithiasis of the anterior semicircular canal (ASC) causes p-DBN and that patients who have p-DBN without obvious CNS dysfunction are dealt with anterior semicircular canal (ASC) benign paroxysmal positional vertigo (BPPV). There are some doubts as to the validity of making a diagnosis of ASC-BPPV in a case of p-DBN without CNS findings. It is hard to determine the cause of p-DBN in these cases.

Congenital Head Nodding and Nystagmus with Cerebrocerebellar Degeneration

ERIC Educational Resources Information Center

Kalyanaraman, K.; And Others

1973-01-01

Reported are three case histories of children with congenital head nodding and nystagmus (rhytmic oscillation of the eyeballs) associated with brain degeneration and motor and mental retardation. (DB)

Downbeating nystagmus and muscle spasms in a patient with glutamic-acid decarboxylase antibodies.

PubMed

Ances, Beau M; Dalmau, Josep O; Tsai, Jean; Hasbani, M Josh; Galetta, Steven L

2005-07-01

To report the ophthalmic findings and response to treatment in a patient with glutamic-acid decarboxylase antibodies. Case report. A 55-year-old woman developed progressive, painful, low back muscle spasms, vertical diplopia, downbeating nystagmus, and asymmetric appendicular ataxia. Downbeating nystagmus was present in primary gaze with an alternating skew deviation in lateral gaze. Serum and cerebrospinal fluid GAD antibodies were detected. Treatment with diazepam led to resolution of spasticity, whereas repeated courses of intravenous immunoglobulin improved cerebellar function, including appendicular ataxia and downbeating nystagmus. Patients with GAD antibodies may have elements of both Stiff-person syndrome (muscle rigidity and spasms) and prominent cerebellar dysfunction. Treatment with diazepam rapidly improved Stiff-person symptoms, whereas IVIg was partially effective at the early stage of cerebellar dysfunction.

Instruments and attachments for electronystagmography

NASA Technical Reports Server (NTRS)

Mironenko, Y. T.; Vilenskiy, A. A.

1980-01-01

A portable set of instruments and devices was developed which makes it possible to record spontaneous nystagmus with open and closed eyes. Rotational, caloric, position, and pressure nystagmus under any conditions may also be recorded.

Oscillopsia in "inverse latent" infantile nystagmus syndrome.

PubMed

Abel, Larry A; Malesic, Linda A

2007-11-01

A possibly unique individual with infantile nystagmus syndrome presented with incessant oscillopsia but good stereopsis when viewing binocularly; her nystagmus was greatly reduced with left eye occlusion. We have attempted to explain this and to identify an intervention that preserves binocular vision while maximizing perceptual stability. Eye movements were recorded and analyzed for duration of foveation (% time when the target was on or near the fovea and the eye was moving at < or = 4 degrees /sec) under different viewing conditions. Changes in foveation were compared with the patient's reports of her perceptual stability. In her right gaze null with her right eye fixating, foveation was 52.9%. This fell to 32.3% for the same eye in primary position and to 0.8% when viewing binocularly in primary position. When viewing binocularly oscillopsia was incessant; when viewing with her right eye vision was stable except in left gaze. Prism correction of her phoria greatly reduced her oscillopsia when viewing binocularly while preserving stereopsis; foveation went up to 33.7%. The patient's ability to maintain good foveation only when viewing with her right eye forces her to choose between stereopsis and stable vision. This may result from the rare combination of (1) requiring good foveation for oscillopsia suppression and (2) nystagmus deteriorating under the stress of maintaining binocularity. There may be many other infantile nystagmus syndrome patients who do not develop oscillopsia but may suffer sufficient asthenopia from a phoria to exacerbate their nystagmus.

Reading strategies in infantile nystagmus syndrome.

PubMed

Thomas, Mervyn G; Gottlob, Irene; McLean, Rebecca J; Maconachie, Gail; Kumar, Anil; Proudlock, Frank A

2011-10-17

The adaptive strategies adopted by individuals with infantile nystagmus syndrome (INS) during reading are not clearly understood. Eye movement recordings were used to identify ocular motor strategies used by patients with INS during reading. Eye movements were recorded at 500 Hz in 25 volunteers with INS and 7 controls when reading paragraphs of text centered at horizontal gaze angles of -20°, -10°, 0°, 10°, and 20°. At each location, reading speeds were measured, along with logMAR visual acuity and nystagmus during gaze-holding. Adaptive strategies were identified from slow and quick-phase patterns in the nystagmus waveform. Median reading speeds were 204.3 words per minute in individuals with INS and 273.6 words per minute in controls. Adaptive strategies included (1) suppression of corrective quick phases allowing involuntary slow phases to achieve the desired goal, (2) voluntarily changing the character of the involuntary slow phases using quick phases, and (3) correction of involuntary slow phases using quick phases. Several individuals with INS read more rapidly than healthy control volunteers. These findings demonstrate that volunteers with INS learn to manipulate their nystagmus using a range of strategies to acquire visual information from the text. These strategies include taking advantage of the stereotypical and periodic nature of involuntary eye movements to allow the involuntary eye movements to achieve the desired goal. The versatility of these adaptations yields reading speeds in those with nystagmus that are often much better than might be expected, given the degree of foveal and ocular motor deficits.

Motor neuronopathy with dropped hands and downbeat nystagmus: a distinctive disorder? A case report.

PubMed

Thakore, Nimish J; Pioro, Erik P; Rucker, Janet C; Leigh, R John

2006-01-12

Eye movements are clinically normal in most patients with motor neuron disorders until late in the disease course. Rare patients are reported to show slow vertical saccades, impaired smooth pursuit, and gaze-evoked nystagmus. We report clinical and oculomotor findings in three patients with motor neuronopathy and downbeat nystagmus, a classic sign of vestibulocerebellar disease. All patients had clinical and electrodiagnostic features of anterior horn cell disease. Involvement of finger and wrist extensors predominated, causing finger and wrist drop. Bulbar or respiratory dysfunction did not occur. All three had clinically evident downbeat nystagmus worse on lateral and downgaze, confirmed on eye movement recordings using the magnetic search coil technique in two patients. Additional oculomotor findings included alternating skew deviation and intermittent horizontal saccadic oscillations, in one patient each. One patient had mild cerebellar atrophy, while the other two had no cerebellar or brainstem abnormality on neuroimaging. The disorder is slowly progressive, with survival up to 30 years from the time of onset. The combination of motor neuronopathy, characterized by early and prominent wrist and finger extensor weakness, and downbeat nystagmus with or without other cerebellar eye movement abnormalities may represent a novel motor neuron syndrome.

Motor neuronopathy with dropped hands and downbeat nystagmus: A distinctive disorder? A case report

PubMed Central

Thakore, Nimish J; Pioro, Erik P; Rucker, Janet C; Leigh, R John

2006-01-01

Background Eye movements are clinically normal in most patients with motor neuron disorders until late in the disease course. Rare patients are reported to show slow vertical saccades, impaired smooth pursuit, and gaze-evoked nystagmus. We report clinical and oculomotor findings in three patients with motor neuronopathy and downbeat nystagmus, a classic sign of vestibulocerebellar disease. Case presentation All patients had clinical and electrodiagnostic features of anterior horn cell disease. Involvement of finger and wrist extensors predominated, causing finger and wrist drop. Bulbar or respiratory dysfunction did not occur. All three had clinically evident downbeat nystagmus worse on lateral and downgaze, confirmed on eye movement recordings using the magnetic search coil technique in two patients. Additional oculomotor findings included alternating skew deviation and intermittent horizontal saccadic oscillations, in one patient each. One patient had mild cerebellar atrophy, while the other two had no cerebellar or brainstem abnormality on neuroimaging. The disorder is slowly progressive, with survival up to 30 years from the time of onset. Conclusion The combination of motor neuronopathy, characterized by early and prominent wrist and finger extensor weakness, and downbeat nystagmus with or without other cerebellar eye movement abnormalities may represent a novel motor neuron syndrome. PMID:16409626

Phenotypical characteristics of idiopathic infantile nystagmus with and without mutations in FRMD7.

PubMed

Thomas, Shery; Proudlock, Frank A; Sarvananthan, Nagini; Roberts, Eryl O; Awan, Musarat; McLean, Rebecca; Surendran, Mylvaganam; Kumar, A S Anil; Farooq, Shegufta J; Degg, Chris; Gale, Richard P; Reinecke, Robert D; Woodruff, Geoffrey; Langmann, Andrea; Lindner, Susanne; Jain, Sunila; Tarpey, Patrick; Raymond, F Lucy; Gottlob, Irene

2008-05-01

Idiopathic infantile nystagmus (IIN) consists of involuntary oscillations of the eyes. The familial form is most commonly X-linked. We recently found mutations in a novel gene FRMD7 (Xq26.2), which provided an opportunity to investigate a genetically defined and homogeneous group of patients with nystagmus. We compared clinical features and eye movement recordings of 90 subjects with mutation in the gene (FRMD7 group) to 48 subjects without mutations but with clinical IIN (non-FRMD7 group). Fifty-eight female obligate carriers of the mutation were also investigated. The median visual acuity (VA) was 0.2 logMAR (Snellen equivalent 6/9) in both groups and most patients had good stereopsis. The prevalence of strabismus was also similar (FRMD7: 7.8%, non-FRMD7: 10%). The presence of anomalous head posture (AHP) was significantly higher in the non-FRMD7 group (P < 0.0001). The amplitude of nystagmus was more strongly dependent on the direction of gaze in the FRMD7 group being lower at primary position (P < 0.0001), compared to non-FRMD7 group (P = 0.83). Pendular nystagmus waveforms were also more frequent in the FRMD7 group (P = 0.003). Fifty-three percent of the obligate female carriers of an FRMD7 mutation were clinically affected. The VA's in affected females were slightly better compared to affected males (P = 0.014). Subnormal optokinetic responses were found in a subgroup of obligate unaffected carriers, which may be interpreted as a sub-clinical manifestation. FRMD7 is a major cause of X-linked IIN. Most clinical and eye movement characteristics were similar in the FRMD7 group and non-FRMD7 group with most patients having good VA and stereopsis and low incidence of strabismus. Fewer patients in the FRMD7 group had AHPs, their amplitude of nystagmus being lower in primary position. Our findings are helpful in the clinical identification of IIN and genetic counselling of nystagmus patients.

Gaze-evoked and rebound nystagmus in a case of migrainous vertigo.

PubMed

Oh, Sun-Young; Seo, Man-Wook; Kim, Young-Hyun; Choi, Kwang-Dong; Kim, Dae-Seong; Shin, Byoung-Soo

2009-03-01

A patient with migrainous vertigo showed pronounced gaze-evoked and rebound nystagmus during an attack. These findings, which have not been previously documented, suggest that migrainous vertigo is based on central vestibular dysfunction.

The robustness of the horizontal gaze nystagmus test

DOT National Transportation Integrated Search

2007-09-01

Police officers follow procedures set forth in the NHTSA/IACP curriculum when they administer the Standardized Field Sobriety Tests (SFSTs) to suspected alcohol-impaired drivers. The SFSTs include Horizontal Gaze Nystagmus (HGN) test, Walk-and-Turn (...

[Neurotological long-term follow-up in Minamata disease in Niigata, Japan].

PubMed

Mizukoshi, Kanemasa; Watanabe, Yukio; Shojaku, Hideo; Aso, Shin; Asai, Masatsugu; Inukai, Kenya; Takahashi, Sugata

2002-03-01

To determine the long-term influence of organic mercurial intoxication on audiological and equilibrium findings, we followed up 36 patients neurotologically during 1980-1987 and 1991-2000 at Kido Hospital in Niigata. Typical findings were as follows: 1. In pure-tone audiometry, 24 of 72 ears (33%) showed slight hearing deterioration and 3 (4%) showed improvement. 2. Spontaneous nystagmus had disappeared in 5 patients (14%), but appeared in new 13 patients (36%). Positional nystagmus did not improve in any patient, and deteriorated in 11 (31%). 3. In optokinetic nystagmus (OKN) tests, especially in vertical OKN test showing significant deterioration (44%). 4. The caloric nystagmus test showed marked deterioration (47%). Body-equilibrium testing showed slight deterioration in 11 patients (31%) and improvement in 5 (14%). Neurotological findings thus varied widely among patients and we were concluded that these differences were caused both by duration of methyl mercury contamination and by aging factors in patients.

Tonic cervical influences on eye nystagmus following hemilabyrinthectomy: immediate and plastic effects.

PubMed

Pettorossi, V E; Petrosini, L

1984-12-17

In intact guinea pigs a passive horizontal rotation of the body about the fixed head induces compensatory ocular movements (cervico-ocular reflex). When the static neck deviation is maintained, a significant ocular displacement is observed. In acutely hemilabyrinthectomized animals, static body deviation towards the lesion side tonically alters eye nystagmus. It affects slow phase eye velocity and quick phase amplitude and frequency causing the eye to reach a less eccentric orbital position. Apart from such immediate influences, a plastic effect on eye nystagmus abatement is induced. In the animals restrained with no body-on-head deviation, abatement of nystagmus is delayed with respect to the animals restrained with 35 degrees body deviation towards the lesion side. Thus the head position signal is not only a contributing factor for the correction of postural deficits but also influences the time course of the ocular balancing process following unilateral vestibular damage.

A Case Report of Nystagmus with Acute Comitant Esotropia Secondary to Heroin Withdrawal: A Novel Presentation

PubMed Central

Rabin, Richard L.

2015-01-01

Background Acute comitant esotropia secondary to heroin withdrawal is a rarely reported phenomenon that has never been described with nystagmus. Adverse effects of heroin on eye alignment were first reported in soldiers returning from Vietnam, yet no theory is generally accepted as the cause of these abnormalities. Method We present a case of a 22-year-old female who developed 40 prism diopters of alternating comitant esotropia with nystagmus 8 days after abrupt heroin cessation, review the existing literature, and propose a novel hypothesis for this phenomenon. Results After 76 days, her esotropia resolved, and she was left with 7 prism diopters of esophoria. Conclusion This case demonstrates that acquired nystagmus can present in addition to acute-onset esotropia after abrupt heroin cessation. We compare and contrast the theories of this mechanism and review the literature. PMID:26483678

Polyamines in the lateral vestibular nuclei of the squirrel monkey and their potential role in vestibular compensation

NASA Technical Reports Server (NTRS)

Henley, C.; Igarashi, M.

1993-01-01

Polyamine synthesis increases in response to injurious stimuli including axotomy and denervation. Reduced eye nystagmus and head-deviation have been observed in unilateral labyrinthectomized (UL) guinea pigs treated with an inhibitor of polyamine synthesis, alpha-difluoromethylornithine (DFMO). We quantified polyamines in the lateral vestibular nuclei (LVN) of control and UL squirrel monkeys during the phase of vestibular compensation (VC) and performed an experiment to determine if DFMO reduces nystagmus previously observed in the guinea pig. Polyamines were detected in the LVN of control and UL squirrel monkeys. Putrescine and spermidine increased in the ipsilateral LVN 3 days after UL with no change in the contralateral LVN. No left-right differences were noted in the 5-day post-UL monkey. DFMO reduced nystagmus in a UL squirrel monkey. These findings suggest that polyamines are important in vestibular function and may contribute to nystagmus observed in VC.

[Analysis of 180 patients with sensory defect nystagmus (SDN) and congenital idiopathic nystagmus (CIN)].

PubMed

Lorenz, B; Gampe, E

2001-01-01

Analysis of the diseases underlying congenital nystagmus in a series of patients registered during 6 years as a prerequisite for adequate counselling of the families. Retrospective study of all patients that presented between 1992 and 1998 with congenital nystagmus not related to visual deprivation or acquired pathologies of the visual pathways. The patients were examined clinically and in dependence on the findings also by electrophysiological (Ganzfeld ERG and VEP, Albino-flash-VEP), psychophysical (colour vision, dark adaptation, spectral sensitivity), and molecular genetic methods. When estimated necessary, family members affected by history and unaffected family members were also examined. In cases of complex neuroophthalmological diseases a neuropaediatric examination including neuroimaging was initiated. In total, 180 patients could be analysed. A sensory defect nystagmus (SDN) was present in 142 patients (79%). The diagnoses were as follows: albinism (any form) in 56 patients (30%), progressive photoreceptor dystrophy in 20 patients (11%), stationary cone dysfunction in 18 patients (10%), bilateral optic nerve hypoplasia in 15 patients (8%), chorioretinal or optic nerve colobomata in 10 patients (6%), aniridia and its variants in 10 patients (6%), familial isolated nystagmus in 8 patients (5%), and congenital stationary night blindness in 5 patients (3%). 38 patients (21%) could not (yet) be classified. The prevalence of SDN as the manifesting symptom of a variety of well defined diseases in the present series of at least 79% is similar to that of 90% reported earlier. The precise diagnosis is a prerequisite for counselling the families as to functional prognosis and recurrence risk. Unnecessary neurological examinations including neuroimaging can be avoided.

Importance of spontaneous nystagmus detection in the differential diagnosis of acute vertigo.

PubMed

Pavlin-Premrl, Davor; Waterston, John; McGuigan, Sean; Infeld, Bernard; Sultana, Ron; O'Sullivan, Richard; Gerraty, Richard P

2015-03-01

Vertigo is a common cause of emergency department attendance. Detection of spontaneous nystagmus may be a useful sign in distinguishing vestibular neuritis from other vestibular diagnoses. We aimed to assess the contribution of spontaneous nystagmus in the diagnosis of acute vertigo. We enrolled consecutive consenting patients arriving at a single emergency department with acute vertigo. There was no declared protocol for the emergency department staff. A standardized history and examination was conducted by the investigators. Observation for spontaneous nystagmus, its response to visual fixation, and testing the vestibulo-ocular reflex with the horizontal head impulse test were the chief examination components. MRI was obtained within 24 hours. Clinical criteria and MRI were used to reach the final diagnosis. The investigators' physical findings and final neurological diagnosis were compared with the initial emergency department examination findings and the referral diagnosis. There were 28 patients, 15 with vestibular neuritis, six with benign paroxysmal positional vertigo, one with stroke, suspected clinically, and three with migraine. In three the diagnosis remained uncertain. Spontaneous nystagmus was seen in all 15 patients with vestibular neuritis, fixation-suppressed in eight of 11 tested for this. The head impulse test was positive in 12 of 15 with vestibular neuritis. The emergency department referral diagnosis was correct in six of 23 patients. The ability to detect spontaneous nystagmus is useful in vestibular diagnosis, both in support of a diagnosis of vestibular neuritis and in avoiding false positive diagnoses of benign paroxysmal positional vertigo. Copyright © 2014 Elsevier Ltd. All rights reserved.

Effects of bilateral caloric habituation of nystagmus responses of the cat.

DOT National Transportation Integrated Search

1964-09-01

It has been suggested that the decline (habituation) of the nystagmus response to repeated vestibular stimulation is a form of learning in that it possesses the necessary features of learned behavior - acquisition, retention, and transfer.

Upward gaze-evoked nystagmus with organoarsenic poisoning.

PubMed

Nakamagoe, Kiyotaka; Ishii, Kazuhiro; Tamaoka, Akira; Shoji, Shin'ichi

2006-01-10

The authors report assessment of abnormal ocular movements in three patients after organoarsenic poisoning from diphenylarsinic acid. The characteristic and principal sign is upward gaze-evoked nystagmus. Moreover, vertical gaze holding impairment was shown by electronystagmography on direct current recording.

Some characteristics of optokinetic eye-movement patterns : a comparative study.

DOT National Transportation Integrated Search

1970-07-01

Long-associated with transportation ('railroad nystagmus'), optokinetic (OPK) nystagmus is an eye-movement reaction which occurs when a series of moving objects crosses the visual field or when an observer moves past a series of objects. Similar cont...

Alcohol and disorientation-related responses. II, Nystagmus and "vertigo" during angular acceleration.

DOT National Transportation Integrated Search

1971-04-01

The integrity of the visual and vestibular systems is important in the maintenance of orientation during flight. Although alcohol is known to affect the vestibular system through the development of a positional alcohol nystagmus, information concerni...

Damping of monocular pendular nystagmus with vibration in a patient with multiple sclerosis.

PubMed

Beh, Shin C; Tehrani, Ali Saber; Kheradmand, Amir; Zee, David S

2014-04-15

Acquired pendular nystagmus (PN) occurs commonly in multiple sclerosis (MS) and results in a highly disabling oscillopsia that impairs vision. It usually consists of pseudo-sinusoidal oscillations at a single frequency (3-5 Hz) that often briefly stop for a few hundred milliseconds after saccades and blinks. The oscillations are thought to arise from instability in the gaze-holding networks ("neural integrator") in the brainstem and cerebellum.(1,2) Here we describe a patient with monocular PN in whom vibration on the skull from a handheld muscle massager strikingly diminished or stopped her nystagmus.

Novel Eye Movement Disorders in Whipple's Disease-Staircase Horizontal Saccades, Gaze-Evoked Nystagmus, and Esotropia.

PubMed

Shaikh, Aasef G; Ghasia, Fatema F

2017-01-01

Whipple's disease, a rare systemic infectious disorder, is complicated by the involvement of the central nervous system in about 5% of cases. Oscillations of the eyes and the jaw, called oculo-masticatory myorhythmia, are pathognomonic of the central nervous system involvement but are often absent. Typical manifestations of the central nervous system Whipple's disease are cognitive impairment, parkinsonism mimicking progressive supranuclear palsy with vertical saccade slowing, and up-gaze range limitation. We describe a unique patient with the central nervous system Whipple's disease who had typical features, including parkinsonism, cognitive impairment, and up-gaze limitation; but also had diplopia, esotropia with mild horizontal (abduction more than adduction) limitation, and vertigo. The patient also had gaze-evoked nystagmus and staircase horizontal saccades. Latter were thought to be due to mal-programmed small saccades followed by a series of corrective saccades. The saccades were disconjugate due to the concurrent strabismus. Also, we noted disconjugacy in the slow phase of gaze-evoked nystagmus. The disconjugacy of the slow phase of gaze-evoked nystagmus was larger during monocular viewing condition. We propose that interaction of the strabismic drifts of the covered eyes and the nystagmus drift, putatively at the final common pathway might lead to such disconjugacy.

Viewing condition dependence of the gaze-evoked nystagmus in Arnold Chiari type 1 malformation.

PubMed

Ghasia, Fatema F; Gulati, Deepak; Westbrook, Edward L; Shaikh, Aasef G

2014-04-15

Saccadic eye movements rapidly shift gaze to the target of interest. Once the eyes reach a given target, the brainstem ocular motor integrator utilizes feedback from various sources to assure steady gaze. One of such sources is cerebellum whose lesion can impair neural integration leading to gaze-evoked nystagmus. The gaze evoked nystagmus is characterized by drifts moving the eyes away from the target and a null position where the drifts are absent. The extent of impairment in the neural integration for two opposite eccentricities might determine the location of the null position. Eye in the orbit position might also determine the location of the null. We report this phenomenon in a patient with Arnold Chiari type 1 malformation who had intermittent esotropia and horizontal gaze-evoked nystagmus with a shift in the null position. During binocular viewing, the null was shifted to the right. During monocular viewing, when the eye under cover drifted nasally (secondary to the esotropia), the null of the gaze-evoked nystagmus reorganized toward the center. We speculate that the output of the neural integrator is altered from the bilateral conflicting eye in the orbit position secondary to the strabismus. This could possibly explain the reorganization of the location of the null position. Copyright © 2014 Elsevier B.V. All rights reserved.

Image-based computer-assisted diagnosis system for benign paroxysmal positional vertigo

NASA Astrophysics Data System (ADS)

Kohigashi, Satoru; Nakamae, Koji; Fujioka, Hiromu

2005-04-01

We develop the image based computer assisted diagnosis system for benign paroxysmal positional vertigo (BPPV) that consists of the balance control system simulator, the 3D eye movement simulator, and the extraction method of nystagmus response directly from an eye movement image sequence. In the system, the causes and conditions of BPPV are estimated by searching the database for record matching with the nystagmus response for the observed eye image sequence of the patient with BPPV. The database includes the nystagmus responses for simulated eye movement sequences. The eye movement velocity is obtained by using the balance control system simulator that allows us to simulate BPPV under various conditions such as canalithiasis, cupulolithiasis, number of otoconia, otoconium size, and so on. Then the eye movement image sequence is displayed on the CRT by the 3D eye movement simulator. The nystagmus responses are extracted from the image sequence by the proposed method and are stored in the database. In order to enhance the diagnosis accuracy, the nystagmus response for a newly simulated sequence is matched with that for the observed sequence. From the matched simulation conditions, the causes and conditions of BPPV are estimated. We apply our image based computer assisted diagnosis system to two real eye movement image sequences for patients with BPPV to show its validity.

The clinical evaluation of infantile nystagmus: What to do first and why

PubMed Central

Bertsch, Morgan; Floyd, Michael; Kehoe, Taylor; Pfeifer, Wanda; Drack, Arlene V.

2017-01-01

Introduction Infantile nystagmus has many causes, some life threatening. We determined the most common diagnoses in order to develop a testing algorithm. Methods Retrospective chart review. Exclusion criteria were no nystagmus, acquired after 6 months, or lack of examination. Data collected: pediatric eye examination findings, ancillary testing, order of testing, referral, and final diagnoses. Final diagnosis was defined as meeting published clinical criteria and/or confirmed by diagnostic testing. Patients with a diagnosis not meeting the definition were “unknown.” Patients with incomplete testing were “incomplete.” Patients with multiple plausible etiologies were “multifactorial.” Patients with negative complete workup were “motor.” Results 284 charts were identified; 202 met inclusion criteria. The 3 most common causes were Albinism(19%), Leber Congenital Amaurosis(LCA)(14%) and Non-LCA retinal dystrophy (13%). Anatomic retinal disorders comprised 10%, motor another 10%. The most common first test was MRI (74/202) with a diagnostic yield of 16%. For 28 MRI-first patients, nystagmus alone was the indication; for 46 MRI-first patients other neurologic signs were present. 0/28 nystagmus-only patients had a diagnostic MRI while 14/46 (30%) with neurologic signs did. Yield of ERG as first test was 56%, OCT 55%, and molecular genetic testing 47%. 90% of patients had an etiology identified. Conclusion The most common causes of infantile nystagmus were retinal disorders (56%), however the most common first test was brain MRI. For patients without other neurologic stigmata complete pediatric eye examination, ERG, OCT and molecular genetic testing had a higher yield than MRI scan. If MRI is not diagnostic, a complete ophthalmologic workup should be pursued. PMID:28177849

Topical brinzolamide (Azopt) versus placebo in the treatment of infantile nystagmus syndrome (INS).

PubMed

Hertle, Richard W; Yang, Dongsheng; Adkinson, Tonia; Reed, Michael

2015-04-01

To test the hypothesis that the topical carbonic anhydrase inhibitor brinzolamide (Azopt) has beneficial effects versus placebo on measures of nystagmus and visual acuity in adult subjects with infantile nystagmus syndrome (INS). Prospective, cross-over, double masked clinical trial. Single centre. Five subjects ≥18 years old with typical INS and best-binocular visual acuity in their primary position null zone ETDRS 55 letters to 85 letters (20/200 to 20/50) and had no previous treatment for nystagmus. In a randomised order, each subject received one drop of Azopt or placebo in both eyes three times a day separated by a washout period of at least a week followed by Azopt or placebo in both eyes three times a day; thus each subject got the drug and placebo, each acting as his or her own control. The nystagmus acuity function and INS waveforms obtained from eye movement recordings, binocular optotype visual acuity, using the ETDRS protocol analysed individually and as a group before and after Azopt and placebo. Versus placebo and baseline measures, topical Azopt significantly improved; INS waveform characteristics in the primary position null zone, group mean values of the nystagmus acuity function across gaze (p<0.01) and group mean ETDRS binocular letter visual acuity (p<0.05). There was a predictable decrease in intraocular pressure (IOP) without any systemic or ocular adverse events. Although a prospective large-scale clinical trial is needed to prove effectiveness, an eye-drop-based therapy for INS may emerge as a viable addition to optical, surgical, behavioural and systemic drug therapies for INS. NCT01312402. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Effects of topical brinzolamide on infantile nystagmus syndrome waveforms: eyedrops for nystagmus.

PubMed

Dell'osso, Louis F; Hertle, Richard W; Leigh, R John; Jacobs, Jonathan B; King, Susan; Yaniglos, Stacia

2011-09-01

Recent advances in infantile nystagmus syndrome (INS) surgery have uncovered the therapeutic importance of proprioception. In this report, we test the hypothesis that the topical carbonic anhydrase inhibitor (CAI) brinzolamide (Azopt) has beneficial effects on measures of nystagmus foveation quality in a subject with INS. Eye movement data were taken, using a high-speed digital video recording system, before and after 3 days of the application of topical brinzolamide 3 times daily in each eye. Nystagmus waveforms were analyzed by applying the eXpanded Nystagmus Acuity Function (NAFX) at different gaze angles and determining the longest foveation domain (LFD) and compared to previously published data from the same subject after the use of a systemic CAI, contact lenses, and convergence and to other subjects before and after eye muscle surgery for INS. Topical brinzolamide improved foveation by both a 51.9% increase in the peak value of the NAFX function (from 0.395 to 0.600) and a 50% broadening of the NAFX vs Gaze Angle curve (the LFD increased from 20° to 30°). The improvements in NAFX after topical brinzolamide were equivalent to systemic acetazolamide or eye muscle surgery and were intermediate between those of soft contact lenses or convergence. Topical brinzolamide and contact lenses had equivalent LFD improvements and were less effective than convergence. In this subject with INS, topical brinzolamide resulted in improved-foveation INS waveforms over a broadened range of gaze angles. Its therapeutic effects were equivalent to systemic CAI. Although a prospective clinical trial is needed to prove efficacy or effectiveness in other subjects, an eyedrops-based therapy for INS may emerge as a viable addition to optical, surgical, behavioral, and systemic drug therapies.

Oblique muscle surgery for treatment of nystagmus with head tilt.

PubMed

Lueder, Gregg T; Galli, Marlo

2012-08-01

Patients with nystagmus may adopt an abnormal head posture if they have a null zone in eccentric gaze. These patients uncommonly present with torticollis due to a null zone when the head is tilted. We describe the results of surgery on the oblique muscles to improve the abnormal head posture in this condition. This was a retrospective review of patients who had head tilts due to null zones of nystagmus. Surgery consisted of an anterior 50% tenectomy of the superior oblique tendon on one side and recession of the inferior oblique muscle to a position 6 mm posterior to the insertion of the inferior rectus muscle on the contralateral side. The patients' clinical histories and outcomes were reviewed. Six patients underwent the procedure. Of these, four had infantile nystagmus syndrome and two were born prematurely and had histories of intraventricular hemorrhages. Five of the patients had previous Kestenbaum surgery that corrected the horizontal component of their abnormal head postures. Age at time of surgery for the head tilt ranged from 3 to 13 years. Postoperative follow-up ranged from 1.5 to 3 years. The preoperative head tilts ranged from 25° to 45° (mean, 39°). The postoperative improvement ranged from 20° to 40° (mean, 28°). One of the patients with a history of intraventricular hemorrhage required additional surgery for strabismus unrelated to nystagmus. Anterior tenectomy of the superior oblique tendon combined with contralateral recession of the inferior oblique muscle improved head tilts related to a null zone of nystagmus. Copyright © 2012 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.

Observations on the elicitation of secondary and inverted primary nystagmus from the cat by unilateral caloric irrigation.

DOT National Transportation Integrated Search

1963-02-01

Vestibular stimulation by repeated unilateral caloric irrigation of cats occasioned the appearance of secondary, tertiary, and inverted primary nystagmus in some animals. These inverse responses were recorded with stimulus temperatures of 5, 23.5, an...

General purpose algorithms for characterization of slow and fast phase nystagmus

NASA Technical Reports Server (NTRS)

Lessard, Charles S.

1987-01-01

In the overall aim for a better understanding of the vestibular and optokinetic systems and their roles in space motion sickness, the eye movement responses to various dynamic stimuli are measured. The vestibulo-ocular reflex (VOR) and the optokinetic response, as the eye movement responses are known, consist of slow phase and fast phase nystagmus. The specific objective is to develop software programs necessary to characterize the vestibulo-ocular and optokinetic responses by distinguishing between the two phases of nystagmus. The overall program is to handle large volumes of highly variable data with minimum operator interaction. The programs include digital filters, differentiation, identification of fast phases, and reconstruction of the slow phase with a least squares fit such that sinusoidal or psuedorandom data may be processed with accurate results. The resultant waveform, slow phase velocity eye movements, serves as input data to the spectral analysis programs previously developed for NASA to analyze nystagmus responses to pseudorandom angular velocity inputs.

A gene for autosomal dominant congenital nystagmus localizes to 6p12

DOE Office of Scientific and Technical Information (OSTI.GOV)

Kerrison, J.B.; Arnould, V.J.; Koenekoop, R.K.

1996-05-01

Congenital nystagmus is an idiopathic disorder characterized by bilateral ocular oscillations usually manifest during infancy. Vision is typically decreased due to slippage of images across the fovea. As such, visual acuity correlates with nystagmus intensity, which is the amplitude and frequency of eye movements at a given position of gaze. X-linked, autosomal dominant, and autosomal recessive pedigrees have been described, but no mapping studies have been published. We recently described a large pedigree with autosomal dominant congenital nystagmus. A genome-wide search resulted in six markers on 6p linked by two-point analysis at {theta} = 0 (D6S459, D6S452, D6S465, FTHP1, D6S257,more » D6S430). Haplotype analysis localizes the gene for autosomal dominant congenital motor mystagmus to an 18-cM region between D6S271 and D6S455. 16 refs., 1 fig., 1 tab.« less

Magnetic Oculomotor Prosthetics for Acquired Nystagmus.

PubMed

Nachev, Parashkev; Rose, Geoff E; Verity, David H; Manohar, Sanjay G; MacKenzie, Kelly; Adams, Gill; Theodorou, Maria; Pankhurst, Quentin A; Kennard, Christopher

2017-10-01

Acquired nystagmus, a highly symptomatic consequence of damage to the substrates of oculomotor control, often is resistant to pharmacotherapy. Although heterogeneous in its neural cause, its expression is unified at the effector-the eye muscles themselves-where physical damping of the oscillation offers an alternative approach. Because direct surgical fixation would immobilize the globe, action at a distance is required to damp the oscillation at the point of fixation, allowing unhindered gaze shifts at other times. Implementing this idea magnetically, herein we describe the successful implantation of a novel magnetic oculomotor prosthesis in a patient. Case report of a pilot, experimental intervention. A 49-year-old man with longstanding, medication-resistant, upbeat nystagmus resulting from a paraneoplastic syndrome caused by stage 2A, grade I, nodular sclerosing Hodgkin's lymphoma. We designed a 2-part, titanium-encased, rare-earth magnet oculomotor prosthesis, powered to damp nystagmus without interfering with the larger forces involved in saccades. Its damping effects were confirmed when applied externally. We proceeded to implant the device in the patient, comparing visual functions and high-resolution oculography before and after implantation and monitoring the patient for more than 4 years after surgery. We recorded Snellen visual acuity before and after intervention, as well as the amplitude, drift velocity, frequency, and intensity of the nystagmus in each eye. The patient reported a clinically significant improvement of 1 line of Snellen acuity (from 6/9 bilaterally to 6/6 on the left and 6/5-2 on the right), reflecting an objectively measured reduction in the amplitude, drift velocity, frequency, and intensity of the nystagmus. These improvements were maintained throughout a follow-up of 4 years and enabled him to return to paid employment. This work opens a new field of implantable therapeutic devices-oculomotor prosthetics-designed to modify eye movements dynamically by physical means in cases where a purely neural approach is ineffective. Applied to acquired nystagmus refractory to all other interventions, it is shown successfully to damp pathologic eye oscillations while allowing normal saccadic shifts of gaze. Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

On the characteristics of caloric nystagmus in healthy persons. [in response to caloric stimuli

NASA Technical Reports Server (NTRS)

Bodo, D.; Baranova, V. P.; Matsnev, E. I.; Yakovleva, M. Y.

1974-01-01

The asymmetry of reflex activity of labyrinths and directional preponderance of the reaction were studied on healthy persons subjected to caloric tests. Calorization with hot water was accompanied by less pronounced reactions in all parameters of nystagmus than analogous indices at cold water stimulation. The symmetry of labyrinth function shifted to the right in individuals with greater activity of the left central vestibular formations, analogous to right handedness behavior. It is concluded that asymmetry of reflex nystagmus in healthy persons can be due to a certain preponderance of functional activity in structures of the left hemisphere of the brain.

Nystagmus as a Sign of Labyrinthine Disorders-Three-Dimensional Analysis of Nystagmus-

PubMed Central

2008-01-01

In order to diagnose the pathological condition of vertiginous patients, a detailed observation of nystagmus in addition to examination of body equilibrium and other neurotological tests are essential. How to precisely record the eye movements is one of the goals of the researchers and clinicians who are interested in the analysis of eye movements for a long time. For considering that, one has to think about the optimal method for recording eye movements. In this review, the author introduced a new method, that is, an analysis of vestibular induced eye movements in three-dimensions and discussed the advantages and limitations of this method. PMID:19434275

Chiari I malformation presenting as downbeat nystagmus: clinical presentation, diagnosis, and management.

PubMed

Goodwin, Denise; Halvorson, Ami R

2012-02-15

Chiari I malformation is a congenital, neurological condition that is characterized by defects of the skull base resulting in herniation of the cerebellum through the foramen magnum into the cervical spinal canal. Because the condition can result in visual symptoms, patients will often search for answers from their eye care providers; A 28-year-old Hispanic diabetic male with a 10-year history of nystagmus was referred to the neuro-ophthalmic disease clinic following the initiation of oscillopsia 1 year previous. Downbeat nystagmus, which worsened in right and down gaze, was evident. Cranial nerve testing was unremarkable, but the patient did report trouble with choking on food and drink. Neuroimaging revealed Chiari I malformation. The patient underwent a suboccipital craniectomy which resulted in lessened nystagmus and improved symptoms; The majority of patients with Chiari I malformation have an onset of symptoms in the second or third decade of life. Most commonly, a suboccipital headache that worsens with Valsalva maneuver is present. Visual symptoms include retro-orbital pain, flashing lights or floaters, blurred vision, photophobia, diplopia, transient vision loss, and peripheral vision loss. Objective evidence is often lacking in these patients; however, horizontal or vertical nystagmus is present in up to 45% of those with Chiari I malformation. Surgery has proven to be an effective and safe method to treat symptomatic Chiari I malformation. American Optometric Association.

Cerebellopontine angle epidermoid tumour presenting with bilateral gaze nystagmus.

PubMed

Han, I B; Huh, R; Chung, S S; Kim, O J

2008-06-01

Vestibular symptoms have been rarely described in cerebellopontine angle epidermoid tumours. We report a case of CPA epidermoid tumour presenting with subacute onset of vestibular symptoms such as vertigo, gait ataxia, and nystagmus masquerading as acute vestibular neuritis or central vertigo. The vestibular symptoms disappeared after excision of the tumour.

Main principles and technique of electronystagmography (a brief survey of the literature)

NASA Technical Reports Server (NTRS)

Tanchev, K. S.

1980-01-01

Electronystagmography (ENG) is one of the modern methods for objective recording of nystagmus, its quantitative and qualitative assessment. It is used more and more often in clinical practice. A brief review of the history of recording of nystagmus and a survey of the relevant literature is presented.

Nystagmus responses of the cat to rotation and to directionally equivalent and non-equivalent stimuli after unitaleral caloric habituation.

DOT National Transportation Integrated Search

1963-08-01

Six cats were exposed to mild angular acceleration before and after an intervening serried of 15 caloric irrigations. All trials were in total darkness. Slowphase displacement of the eyes, beat frequency, and duration of nystagmus were scored. All...

Anophthalmia, cleft lip/palate, absent vomer bone, nystagmus, and mental-motor retardation: a new syndrome or Fryns "anophthalmia-plus" syndrome?

PubMed

Ozçelik, Derya; Sağlam, Ibrahim; SIlan, Fatma; Sezen, Gülbin; Unveren, Toygar

2008-05-01

We report that a 4-year-old boy presented with right unilateral complete cleft lip and palate, right anophthalmos, left congenital nystagmus, absence of the vomer bone, mental-motor retardation, and normal lymphocyte karyotype (46, XY). For reconstruction of the deformities, we performed cleft lip repair by Millard's rotation-advancement technique and planned cleft palate repair. This combination of cleft lip and palate, anophthalmos, congenital nystagmus, absent vomer bone, and mental-motor retardation has not, to our knowledge, previously been described. We suggest that this represents either another case of the rare Fryns "anophthalmia-plus" syndrome or a new syndrome.

Can upbeat nystagmus increase in downward, but not upward, gaze?

PubMed

Kim, Hyun-Ah; Yi, Hyon-Ah; Lee, Hyung

2012-04-01

Upbeat nystagmus (UBN) is typically increased with upward gaze and decreased with downward gaze. We describe a patient with acute multiple sclerosis who developed primary position UBN with a linear slow phase waveform, in which the velocity of nystagmus was intensified in downward gaze and decreased during upward gaze. Brain MRI showed high signal lesions in the paramedian dorsal area of the caudal medulla encompassing the most caudal part of the perihypoglossal nuclei. Clinicians should be aware of possibility of a caudal medullary lesion in a patient with UBN, especially when the velocity of the UBN is increased in downward gaze. Copyright © 2011 Elsevier Ltd. All rights reserved.

Gaze failure, drifting eye movements, and centripetal nystagmus in cerebellar disease.

PubMed Central

Leech, J; Gresty, M; Hess, K; Rudge, P

1977-01-01

Three abnormalities of eye movement in man are described which are indicative of cerebellar system disorder, namely, centripetally beating nystagmus, failure to maintain lateral gaze either in darkness or with eye closure, and slow drifting movements of the eyes in the absence of fixation. Similar eye movement signs follow cerebellectomy in the primate and the cat. These abnormalities of eye movement, together with other signs of cerebellar disease, such as rebound alternating, and gaze paretic nystagmus, are explained by the hypothesis that the cerebellum helps to maintain lateral gaze and that brain stem mechanisms which monitor gaze position generate compensatory biases in the absence of normal cerebellar function. PMID:603785

Caloric Analysis of Patients with Benign Paroxysmal Positional Vertigo.

PubMed

Yetişer, Sertaç; İnce, Dilay

2017-12-01

The aim of this study is to compare nystagmus characteristics after caloric irrigation in patients with lateral canal (LC) and posterior canal (PC) benign paroxysmal positional vertigo (BPPV) and to analyze the role of symptom duration. A prospective study was conducted in 65 patients with BPPV (20 LC and 45 PC) who were subjected to caloric testing. Average slow-phase velocity and nystagmus duration were analyzed. Caloric hypo-excitability was 20.4%. It was more evident in patients with apogeotropic-type LC-BPPV. The comparison of average slow-phase velocity of the nystagmus and nystagmus duration between selected types of BPPV for pathologic, non-pathologic, and the control ears after warm and cold stimulation was not statistically significant (p>0.05). No correlation was found between caloric results and symptom duration (p>0.05). Some patients presented caloric hypo-excitability. Reliability of caloric testing to differentiate the ear with normal and abnormal vestibular function in different types of BPPV was low. No difference was found in the analysis of the impact of symptom duration. Caloric testing is not an ideal tool to study BPPV.

Incidence and anatomy of gaze-evoked nystagmus in patients with cerebellar lesions.

PubMed

Baier, Bernhard; Dieterich, Marianne

2011-01-25

Disorders of gaze-holding--organized by a neural network located in the brainstem or the cerebellum--may lead to nystagmus. Based on previous animal studies it was concluded that one key player of the cerebellar part of this gaze-holding neural network is the flocculus. Up to now, in humans there are no systematic studies in patients with cerebellar lesions examining one of the most common forms of nystagmus: gaze-evoked nystagmus (GEN). The aim of our present study was to clarify which cerebellar structures are involved in the generation of GEN. Twenty-one patients with acute unilateral cerebellar stroke were analyzed by means of modern MRI-based voxel-wise lesion-behavior mapping. Our data indicate that cerebellar structures such as the vermal pyramid, the uvula, and the tonsil, but also parts of the biventer lobule and the inferior semilunar lobule, were affected in horizontal GEN. It seems that these structures are part of a gaze-holding neural integrator control system. Furthermore, GEN might present a diagnostic sign pointing toward ipsilesionally located lesions of midline and lower cerebellar structures.

Surgical management of anomalous head posture because of horizontal gaze palsy or acquired vertical nystagmus.

PubMed

Campos, E C; Schiavi, C; Bellusci, C

2003-07-01

To confirm the effectiveness of the Kestenbaum-Anderson principle in the surgical management of compensatory head posture because of horizontal gaze palsy and acquired vertical nystagmus. Nine patients with anomalous head posture because of horizontal gaze palsy, and four patients with acquired vertical nystagmus and oscillopsia and compensatory torticollis underwent surgery according to the Kestenbaum-Anderson principle. As in the treatment of congenital nystagmus, the eyes have to be shifted in the orbits, in the direction of anomalous head posture. Homonymously based prisms were used preoperatively to assess the potential benefit of surgery. At the time of surgery, the clinical conditions of the patients had been stable for at least 1 year. After surgery, compensatory head posture and visual performances improved in all cases and the results remained stable for at least 2 years. Contrary to what is generally believed, the ocular condition of the patients with compensatory head posture secondary to neurological causes can be often improved with surgery. The aim of surgery is obviously not to modify ocular motility, but rather to improve the head position.

Quick Phases of Infantile Nystagmus Show the Saccadic Inhibition Effect

PubMed Central

Harrison, James J.; Sumner, Petroc; Dunn, Matt J.; Erichsen, Jonathan T.; Freeman, Tom C. A.

2015-01-01

Purpose. Infantile nystagmus (IN) is a pathological, involuntary oscillation of the eyes consisting of slow, drifting eye movements interspersed with rapid reorienting quick phases. The extent to which quick phases of IN are programmed similarly to saccadic eye movements remains unknown. We investigated whether IN quick phases exhibit ‘saccadic inhibition,' a phenomenon typically related to normal targeting saccades, in which the initiation of the eye movement is systematically delayed by task-irrelevant visual distractors. Methods. We recorded eye position from 10 observers with early-onset idiopathic nystagmus while task-irrelevant distractor stimuli were flashed along the top and bottom of a large screen at ±10° eccentricity. The latency distributions of quick phases were measured with respect to these distractor flashes. Two additional participants, one with possible albinism and one with fusion maldevelopment nystagmus syndrome, were also tested. Results. All observers showed that a distractor flash delayed the execution of quick phases that would otherwise have occurred approximately 100 ms later, exactly as in the standard saccadic inhibition effect. The delay did not appear to differ between the two main nystagmus types under investigation (idiopathic IN with unidirectional and bidirectional jerk). Conclusions. The presence of the saccadic inhibition effect in IN quick phases is consistent with the idea that quick phases and saccades share a common programming pathway. This could allow quick phases to take on flexible, goal-directed behavior, at odds with the view that IN quick phases are stereotyped, involuntary eye movements. PMID:25670485

Refractory Positional Vertigo With Apogeotropic Horizontal Nystagmus After Labyrinthitis: Surgical Treatment and Identification of Dysmorphic Ampullae.

PubMed

Ahmed, Sameer; Heidenreich, Katherine D; McHugh, Jonathan B; Altschuler, Richard A; Carender, Wendy J; Telian, Steven A

2015-09-01

To describe the rationale, intraoperative details, and histopathologic findings discovered when treating an unusual case of apogeotropic horizontal canal positional vertigo with a transmastoid labyrinthectomy. A single case report. Therapeutic. Resolution of apogeotropic nystagmus and improvement of positional vertigo. The apogeotropic variant of horizontal canal positional vertigo can be a difficult entity to treat. This report describes a patient who developed profound sensorineural hearing loss and vertigo after an acute left labyrinthitis. Ten months later, she developed vertigo with apogeotropic positional nystagmus involving the left horizontal semicircular canal. Particle repositioning maneuvers and vestibular physical therapy were unsuccessful. In addition, she developed intermittent positional vertigo affecting the ipsilateral vertical semicircular canals. Given the persistence of her vertigo, multiple canal involvement, and patient preference for definitive treatment, a transmastoid labyrinthectomy was performed. Intraoperatively, the ampulla of the horizontal canal as well as that of the other canals was grossly abnormal as later confirmed on histology. After surgery, her apogeotropic nystagmus and vertigo resolved, and her balance ability gradually improved to a highly functional level. This case illustrates a unique form of positional vertigo that developed and persisted after acute labyrinthitis. Conservative measures were unsuccessful and a transmastoid labyrinthectomy documented dense inflammatory tissue involving all three ampullae. We postulate that the post-labyrinthitic inflammatory changes resulted in mass loading of the membranous ampullae, causing abnormal nystagmus patterns and positional vertigo, which resolved after the labyrinthectomy.

Beat-to-beat control of human optokinetic nystagmus slow phase durations

PubMed Central

Furman, Joseph M.

2016-01-01

This study provides the first clear evidence that the generation of optokinetic nystagmus fast phases (FPs) is a decision process that is influenced by performance of a concurrent disjunctive reaction time task (DRT). Ten subjects performed an auditory DRT during constant velocity optokinetic stimulation. Eye movements were measured in three dimensions with a magnetic search coil. Slow phase (SP) durations were defined as the interval between FPs. There were three main findings. Firstly, human optokinetic nystagmus SP durations are consistent with a model of a Gaussian basic interval generator (a type of biological clock), such that FPs can be triggered randomly at the end of a clock cycle (mean duration: 200–250 ms). Kolmogorov-Smirnov tests could not reject the modeled cumulative distribution for any data trials. Secondly, the FP need not be triggered at the end of a clock cycle, so that individual SP durations represent single or multiple clock cycles. Thirdly, the probability of generating a FP at the end of each interval generator cycle decreases significantly during performance of a DRT. These findings indicate that the alternation between SPs and FPs of optokinetic nystagmus is not purely reflexive. Rather, the triggering of the next FP is postponed more frequently if a recently presented DRT trial is pending action when the timing cycle expires. Hence, optokinetic nystagmus FPs show dual-task interference in a manner usually attributed to voluntary movements, including saccades. NEW & NOTEWORTHY This study provides the first clear evidence that the generation of optokinetic nystagmus (OKN) fast phases is a decision process that is influenced by performance of a concurrent disjunctive reaction time task (DRT). The slow phase (SP) durations are consistent with a Gaussian basic interval generator and multiple interval SP durations occur more frequently in the presence of the DRT. Hence, OKN shows dual-task interference in a manner observed in voluntary movements, such as saccades. PMID:27760815

[Efficacy of surgery on congenital nystagmus with convergence damping].

PubMed

Wang, Yuan; Wu, Qian; Bai, Dayong; Cao, Wenhong; Cui, Yanhui; Fan, Yunwei; Hu, Shoulong; Yu, Gang

2015-11-01

To evaluate the efficacy of surgery in the treatment of congenital nystagmus with convergence damping. Retrospective and comparative case series. Eight patients diagnosed as congenital nystagmus with convergence damping at Beijing Children's Hospital between September 2010 and September 2012 were enrolled in this study. The ages were 9.5 (12, 6) years old, and follow-up was 9 (24, 6) months. All patients received prism induced convergence and the same surgery of bimedial rectus recession and bilateral rectus tenotomy. The best corrected visual acuity, the range of fusion and the nystagmus waveforms were analyzed before and after surgery. The range of fusion was -3.75±1.83° to +19.38±3.16° before surgery and -3.88±1.55° to +19.00±3.02° after surgery; there was no significant difference (t=0.24, P=0.82). The binocular visual acuity increased from 0.21±0.15 without convergence to 0.28±0.18 using convergence; there was significant difference (t=-4.43, P=0.00). The visual acuity was 0.32±0.20 after surgery, significantly different from that before surgery without convergence (t=-5.29, P=0.00), but not significantly different from that before surgery using convergence (t=-2.12, P=0.07). Patients had significant improvements in the frequency (t=3.28, 3.02, P<0.05) and intensity of the nystagmus waveforms when using convergence and postoperatively (t=3.27, 3.48; P<0.05), but there was no significant improvement in the amplitude of the waveforms (t=1.31, 1.57, 0.31, P>0.05). Surgery for congenital nystagmus with convergence damping can provide expectations for ocular motor and visual results. The range of fusion should be wide enough, and the effect of convergence on the frequency is greater than that on the amplitude.

Gaze-stabilizing deficits and latent nystagmus in monkeys with brief, early-onset visual deprivation: eye movement recordings.

PubMed

Tusa, R J; Mustari, M J; Burrows, A F; Fuchs, A F

2001-08-01

The normal development and the capacity to calibrate gaze-stabilizing systems may depend on normal vision during infancy. At the end of 1 yr of dark rearing, cats have gaze-stabilizing deficits similar to that of the newborn human infant including decreased monocular optokinetic nystagmus (OKN) in the nasal to temporal (N-T) direction and decreased velocity storage in the vestibuloocular reflex (VOR). The purpose of this study is to determine to what extent restricted vision during the first 2 mo of life in monkeys affects the development of gaze-stabilizing systems. The eyelids of both eyes were sutured closed in three rhesus monkeys (Macaca mulatta) at birth. Eyelids were opened at 25 days in one monkey and 40 and 55 days in the other two animals. Eye movements were recorded from each eye using scleral search coils. The VOR, OKN, and fixation were examined at 6 and 12 mo of age. We also examined ocular alignment, refraction, and visual acuity in these animals. At 1 yr of age, visual acuity ranged from 0.3 to 0.6 LogMAR (20/40-20/80). All animals showed a defect in monocular OKN in the N-T direction. The velocity-storage component of OKN (i.e., OKAN) was the most impaired. All animals had a mild reduction in VOR gain but had a normal time constant. The animals deprived for 40 and 55 days had a persistent strabismus. All animals showed a nystagmus similar to latent nystagmus (LN) in human subjects. The amount of LN and OKN defect correlated positively with the duration of deprivation. In addition, the animal deprived for 55 days demonstrated a pattern of nystagmus similar to congenital nystagmus in human subjects. We found that restricted visual input during the first 2 mo of life impairs certain gaze-stabilizing systems and causes LN in primates.

Intravenous sub-anesthetic ketamine for perioperative analgesia

PubMed Central

Gorlin, Andrew W; Rosenfeld, David M; Ramakrishna, Harish

2016-01-01

Ketamine, an N-methyl-d-aspartate antagonist, blunts central pain sensitization at sub-anesthetic doses (0.3 mg/kg or less) and has been studied extensively as an adjunct for perioperative analgesia. At sub-anesthetic doses, ketamine has a minimal physiologic impact though it is associated with a low incidence of mild psychomimetic symptoms as well as nystagmus and double vision. Contraindications to its use do exist and due to ketamine's metabolism, caution should be exercised in patients with renal or hepatic dysfunction. Sub-anesthetic ketamine improves pain scores and reduces perioperative opioid consumption in a broad range of surgical procedures. In addition, there is evidence that ketamine may be useful in patients with opioid tolerance and for preventing chronic postsurgical pain. PMID:27275042

Influence of gravitoinertial force level on vestibular and visual velocity storage in yaw and pitch.

PubMed

Dizio, P; Lackner, J R

1992-01-01

Velocity storage is an important aspect of sensory-motor control of body orientation. The effective decay rate and three-dimensional organization of velocity storage are dependent upon body orientation relative to gravity and also are influenced by gravitoinertial force (G) level. Several of the inputs to velocity storage including otolithic, somatosensory, proprioceptive, and possibly motor are highly dependent on G level. To see whether the G dependency of velocity storage is related to changes in the effective coupling of individual sensory inputs to the velocity storage mechanism or to alterations in the time constant of velocity storage per se, we have studied horizontal vestibular nystagmus, horizontal optokinetic after nystagmus (OKAN) and vertical vestibular nystagmus as a function of force level. Horizontal OKAN and vestibular nystagmus both showed no effect of G level on their initial or peak slow phase velocities but their decay rates were quicker in 0G and 1.8G than in 1G. Vertical vestibular nystagmus also showed no effect of G level on peak velocity but decayed quicker in 0G relative to 1G. These-findings indicate that the intrinsic decay rate of a common velocity storage mechanism is affected by the magnitude of G. A negligible amount of slow phase eye velocity was observed in planes outside the planes of stimulation, thus short-term changes in G across multiple body axes can change velocity storage, but the change is restricted to the axis common to the rotary stimulus and the G vector.

Two hypothetical nystagmus procedures: augmented tenotomy and reattachment and augmented tendon suture (Sans tenotomy).

PubMed

Dell'Osso, Louis F; Tomsak, Robert L; Thurtell, Matthew J

2009-01-01

To review the hypothetical mechanism and therapeutic benefits of the four-muscle tenotomy and reattachment (T&R) procedure using knowledge accrued over the 10 years since its proposal; to describe an augmented tendon suture (ATS) technique to improve the procedure based on one of the originally suggested alternative methods (mechanical); and to hypothesize a new ATS procedure to achieve the same therapeutic benefits without extraocular muscle tenotomy or reattachment to the globe. Standard surgical methods were used. The T&R procedure damps and improves infantile nystagmus syndrome (INS) waveforms, improves eXtended Nystagmus Acuity Function (NAFX) values, broadens the NAFX peak versus gaze angle, and damps slow eye movements but not saccades. The T&R procedure also damps acquired pendular and downbeat nystagmus, decreasing the patients' oscillopsia, and lowers the target acquisition time in INS. The T&R procedure directly affects only the enthesis of the tendon; there is idiosyncratic variation in the distribution of afferent fibers in the tendons. The ATS technique consists of placing several additional sutures in the tendon proximal to the tenotomy. Based on the hypothetical proprioceptive mechanism for the beneficial effects of the T&R procedure, the authors hypothesize that the ATS technique will maximize the therapeutic benefits and that an ATS procedure, using only tendon sutures without tenotomy, will duplicate the therapeutic effects of T&R. Eliminating the tenotomy component results in a simpler procedure more suitable for single-session, multi-muscle surgery that may be required for improving the waveforms of multiplanar nystagmus and less prone to cause complications. Copyright 2009, SLACK Incorporated.

The minimal ice water caloric test compared with established vestibular caloric test procedures.

PubMed

Schmäl, Frank; Lübben, Björn; Weiberg, Kerstin; Stoll, Wolfgang

2005-01-01

Caloric testing of the vestibular labyrinth is usually performed by classical caloric test procedures (CCTP) using water warmed to 30 degrees C and 44 degrees C. Ice water irrigation (4 degrees C) is usually not performed, although it might be useful as a bedside test. To verify the validity of the Minimal Ice Water Caloric Test (MIWCT), comparative video-oculographic investigations were performed in 22 healthy subjects using ice water (0.5 ml, 1.0 ml, 2 ml), CCTP, and cold air (27 degrees C). Frequency, amplitude, slow phase velocity (SPV), the onset, and the duration of nystagmus were documented. After addition of three ice cubes, the temperature of conventional tap water (16 degrees C) fell within 13 min to 4 degrees C. In pessimum position the subjects demonstrated no nystagmus response. Compared to CCTP, MIWCT was associated with a significantly later onset of nystagmus and a significant prolongation of the nystagmus reaction. In contrast to air stimulation (27 degrees C), a significant Spearman's correlation was noted between MIWCT (1 and 2 ml) and established CCTP in respect of essential nystagmus parameters (frequency, amplitude and SPV). Furthermore, MIWCT (0.5 and 1 ml) showed a higher sensitivity and specificity with regard to the detection of canal paresis based on Jongkees' formula compared to stimulation with air 27 degrees C. Thus, MIWCT appears to be a suitable procedure for bedside investigation of vestibular function outside the vestibular laboratory, e.g. in a hospital ward, where bedridden patients with vertigo occasionally require vestibular testing.

[MRI in congenital nystagmus].

PubMed

Denis, D; Girard, N; Toesca, E; Zanin, E; Gambarelli, N; Lebranchu, P; Mancini, J

2010-03-01

Congenital nystagmus (CN) that is present by the age of 3 months is the most common form of nystagmus in childhood. We present a prospective study (2001-2008) in which we report imaging findings in 48 children with CN. Twenty-six boys and 22 girls with CN underwent a complete ophthalmologic assessment and a cerebral MRI (mean age of examination under general anesthesia: 11 months). Three CN groups were formed: neurologic (n=27), sensory visual disturbance (n=14), and isolated (n=7). Cerebral MRI was interpreted by the same pediatric neuroradiologist (NG). Of the children studied, 98 % were born at term. The MRI abnormalities were classified as morphologic abnormalities (malformative or nonmalformative) and as signal abnormalities. The location of brain abnormalities was within the posterior fossa, (brain stem, cerebellum, dental nuclei, cisterna magna) and the cerebral hemisphere (white matter, perivascular spaces, midline commissures, basal ganglia). Pendular nystagmus was prevalent in sensory and neurologic nystagmus. On fundus examination, optic disc abnormalities were present in 70 % (19) of neurologic CN and associated with white matter abnormalities of the optic radiations in 40 % of cases. On MRI, malformative morphologic abnormalities were present in 27 cases, nonmalformative abnormalities were found in 67, and signal abnormalities in 68. Within the brain stem, signal abnormalities were found as a cockade appearance of the posterior pons in the reticular regions (neurologic n=14, sensory n=6, isolated n=3). Other bright (most frequent) signal abnormalities were found within the dentate nuclei of the posterior fossa (neurologic n=10, sensory n=3, isolated n=3) and the cerebral white matter (neurologic n=17, sensory n=7, isolated n=5) of which 24 (neurologic n=15, sensory n=5, isolated n=4) involved the optic radiations. Most of these abnormalities were related and were seen most frequently in neurologic nystagmus. The most frequent association was signal abnormalities of the white matter, ventricular dilatation, and dilatation of the perivascular spaces (60.4 %) (neurologic n=13, sensory n=6). This study showed the fundamental contribution of the cerebral MRI in CN. Cerebral abnormalities were found at the pathways for ocular motility, particularly at the saccadic pathways.

The nucleus of the optic tract. Its function in gaze stabilization and control of visual-vestibular interaction

NASA Technical Reports Server (NTRS)

Cohen, B.; Reisine, H.; Yokota, J. I.; Raphan, T.

1992-01-01

1. Electrical stimulation of the nucleus of the optic tract (NOT) induced nystagmus and after-nystagmus with ipsilateral slow phases. The velocity characteristics of the nystagmus were similar to those of the slow component of optokinetic nystagmus (OKN) and to optokinetic after-nystagmus (OKAN), both of which are produced by velocity storage in the vestibular system. When NOT was destroyed, these components disappeared. This indicates that velocity storage is activated from the visual system through NOT. 2. Velocity storage produces compensatory eye-in-head and head-on-body movements through the vestibular system. The association of NOT with velocity storage implies that NOT helps stabilize gaze in space during both passive motion and active locomotion in light with an angular component. It has been suggested that "vestibular-only" neurons in the vestibular nuclei play an important role in generation of velocity storage. Similarities between the rise and fall times of eye velocity during OKN and OKAN to firing rates of vestibular-only neurons suggest that these cells may receive their visual input through NOT. 3. One NOT was injected with muscimol, a GABAA agonist. Ipsilateral OKN and OKAN were lost, suggesting that GABA, which is an inhibitory transmitter in NOT, acts on projection pathways to the brain stem. A striking finding was that visual suppression and habituation of contralateral slow phases of vestibular nystagmus were also abolished after muscimol injection. The latter implies that NOT plays an important role in producing visual suppression of the VOR and habituating its time constant. 4. Habituation is lost after nodulus and uvula lesions and visual suppression after lesions of the flocculus and paraflocculus. We postulate that the disappearance of vestibular habituation and of visual suppression of vestibular responses after muscimol injections was due to dysfacilitation of the prominent NOT-inferior olive pathway, inactivating climbing fibers from the dorsal cap to nodulouvular and flocculoparafloccular Purkinje cells. The prompt loss of habituation when NOT was inactivated, and its return when the GABAergic inhibition dissipated, suggests that although VOR habituation can be relatively permanent, it must be maintained continuously by activity of the vestibulocerebellum.

The Role of Superior Oblique Posterior Tenectomy Along With Inferior Rectus Recessions for the Treatment of Chin-up Head Positioning in Patients With Nystagmus.

PubMed

Escuder, Anna G; Ranka, Milan P; Lee, Kathy; Nam, Julie N; Steele, Mark A

2018-05-29

To evaluate the clinical outcomes of bilateral superior oblique posterior 7/8th tenectomy with inferior rectus recession on improving chin-up head positioning in patients with horizontal nystagmus. Medical records were reviewed from 2007 to 2017 for patients with nystagmus and chin-up positioning of 15° or more who underwent combined bilateral superior oblique posterior 7/8th tenectomy with an inferior rectus recession of at least 5 mm. Thirteen patients (9 males and 4 females) were included, with an average age of 7.3 years (range: 1.8 to 15 years). Chin-up positioning ranged from 15° to 45° degrees (average: 30°). Three patients had prior horizontal muscle surgeries, 1 for esotropia and 2 for horizontal null zones causing anomalous face turns. Ten patients underwent other concomitant eye muscle surgery: 3 had esotropia, 1 had exotropia, and 2 had biplanar nystagmus null point requiring a horizontal Anderson procedure. Four patients underwent simultaneous bilateral medial rectus tenotomy and reattachment. All patients had improved chin-up positioning. Eight patients had complete resolution, whereas 5 had minimal residual chin-up positioning. Three patients developed an eccentric horizontal gaze null point with compensatory anomalous face turn with onset 2 weeks, 2 years, and 3 years postoperatively. Average follow-up was 42.7 months. No postoperative pattern deviations, cyclodeviations, or inferior oblique overaction were seen. No surgical complications were noted. Bilateral superior oblique posterior 7/8th tenectomy in conjunction with bilateral inferior rectus recession is a safe and effective procedure for improving chin-up head positioning in patients with horizontal nystagmus with a down gaze null point. [J Pediatr Ophthalmol Strabismus. 201X;XX(X):XX-XX.]. Copyright 2018, SLACK Incorporated.

Vestibulo-Cervico-Ocular Responses and Tracking Eye Movements after Prolonged Exposure to Microgravity

NASA Technical Reports Server (NTRS)

Kornilova, L. N.; Naumov, I. A.; Azarov, K. A.; Sagalovitch, S. V.; Reschke, Millard F.; Kozlovskaya, I. B.

2007-01-01

The vestibular function and tracking eye movements were investigated in 12 Russian crew members of ISS missions on days 1(2), 4(5-6), and 8(9-10) after prolonged exposure to microgravity (126 to 195 days). The spontaneous oculomotor activity, static torsional otolith-cervico-ocular reflex, dynamic vestibulo-cervico-ocular responses, vestibular reactivity, tracking eye movements, and gaze-holding were studied using videooculography (VOG) and electrooculography (EOG) for parallel eye movement recording. On post-flight days 1-2 (R+1-2) some cosmonauts demonstrated: - an increased spontaneous oculomotor activity (floating eye movements, spontaneous nystagmus of the typical and atypical form, square wave jerks, gaze nystagmus) with the head held in the vertical position; - suppressed otolith function (absent or reduced by one half amplitude of torsional compensatory eye counter-rolling) with the head inclined statically right- or leftward by 300; - increased vestibular reactivity (lowered threshold and increased intensity of the vestibular nystagmus) during head turns around the longitudinal body axis at 0.125 Hz; - a significant change in the accuracy, velocity, and temporal characteristics of the eye tracking. The pattern, depth, dynamics, and velocity of the vestibular function and tracking eye movements recovery varied with individual participants in the investigation. However, there were also regular responses during readaptation to the normal gravity: - suppression of the otolith function was typically accompanied by an exaggerated vestibular reactivity; - the structure of visual tracking (the accuracy of fixational eye rotations, smooth tracking, and gaze-holding) was disturbed (the appearance of correcting saccades, the transition of smooth tracking to saccadic tracking) only in those cosmonauts who, in parallel to an increased reactivity of the vestibular input, also had central changes in the oculomotor system (spontaneous nystagmus, gaze nystagmus).

Muscimol inactivation caudal to the interstitial nucleus of Cajal induces hemi-seesaw nystagmus.

PubMed

Das, Vallabh E; Leigh, R John; Swann, Michelle; Thurtell, Matthew J

2010-09-01

Hemi-seesaw nystagmus (hemi-SSN) is a jerk-waveform nystagmus with conjugate torsional and disjunctive vertical components. Halmagyi et al. in Brain 117(Pt 4):789-803 (1994), reported hemi-SSN in patients with unilateral lesions in the vicinity of the Interstitial Nucleus of Cajal (INC) and suggested that an imbalance in projections from the vestibular nuclei to the INC was the source of the nystagmus. However, this hypothesis was called into question by Helmchen et al. in Exp Brain Res 119(4):436-452 (1998), who inactivated INC in monkeys with muscimol (a GABA(A) agonist) and induced failure of vertical gaze-holding (neural integrator) function but not hemi-SSN. We injected 0.1-0.2 microl of 2% muscimol into the supraoculomotor area, 1-2 mm dorso-lateral to the right oculomotor nucleus and caudal to the right INC. A total of seven injections in two juvenile rhesus monkeys were performed. Hemi-SSN was noted within 5-10 min after injection for six of the injections. Around the time the hemi-SSN began, a small skew deviation also developed. However, there was no limitation of horizontal or vertical eye movements, suggesting that the nearby oculomotor nucleus was not initially compromised. Limitations in eye movement range developed about (1/2)-1 h following the injections. Clinical signs that were observed after the animal was released to his cage included a moderate to marked head tilt toward the left (contralesional) side, consistent with an ocular tilt reaction. We conclude that hemi-SSN can be caused by lesions just caudal to the INC, whereas lesions of the INC itself cause down-beat nystagmus and vertical gaze-holding failure, as demonstrated by Helmchen et al. Combined deficits may be encountered with lesions that involve several midbrain structures.

Postural Ataxia in Cerebellar Downbeat Nystagmus: Its Relation to Visual, Proprioceptive and Vestibular Signals and Cerebellar Atrophy.

PubMed

Helmchen, Christoph; Kirchhoff, Jan-Birger; Göttlich, Martin; Sprenger, Andreas

2017-01-01

The cerebellum integrates proprioceptive, vestibular and visual signals for postural control. Cerebellar patients with downbeat nystagmus (DBN) complain of unsteadiness of stance and gait as well as blurred vision and oscillopsia. The aim of this study was to elucidate the differential role of visual input, gaze eccentricity, vestibular and proprioceptive input on the postural stability in a large cohort of cerebellar patients with DBN, in comparison to healthy age-matched control subjects. Oculomotor (nystagmus, smooth pursuit eye movements) and postural (postural sway speed) parameters were recorded and related to each other and volumetric changes of the cerebellum (voxel-based morphometry, SPM). Twenty-seven patients showed larger postural instability in all experimental conditions. Postural sway increased with nystagmus in the eyes closed condition but not with the eyes open. Romberg's ratio remained stable and was not different from healthy controls. Postural sway did not change with gaze position or graviceptive input. It increased with attenuated proprioceptive input and on tandem stance in both groups but Romberg's ratio also did not differ. Cerebellar atrophy (vermal lobule VI, VIII) correlated with the severity of impaired smooth pursuit eye movements of DBN patients. Postural ataxia of cerebellar patients with DBN cannot be explained by impaired visual feedback. Despite oscillopsia visual feedback control on cerebellar postural control seems to be preserved as postural sway was strongest on visual deprivation. The increase in postural ataxia is neither related to modulations of single components characterizing nystagmus nor to deprivation of single sensory (visual, proprioceptive) inputs usually stabilizing stance. Re-weighting of multisensory signals and/or inappropriate cerebellar motor commands might account for this postural ataxia.

Postural Ataxia in Cerebellar Downbeat Nystagmus: Its Relation to Visual, Proprioceptive and Vestibular Signals and Cerebellar Atrophy

PubMed Central

Helmchen, Christoph; Kirchhoff, Jan-Birger; Göttlich, Martin; Sprenger, Andreas

2017-01-01

Background The cerebellum integrates proprioceptive, vestibular and visual signals for postural control. Cerebellar patients with downbeat nystagmus (DBN) complain of unsteadiness of stance and gait as well as blurred vision and oscillopsia. Objectives The aim of this study was to elucidate the differential role of visual input, gaze eccentricity, vestibular and proprioceptive input on the postural stability in a large cohort of cerebellar patients with DBN, in comparison to healthy age-matched control subjects. Methods Oculomotor (nystagmus, smooth pursuit eye movements) and postural (postural sway speed) parameters were recorded and related to each other and volumetric changes of the cerebellum (voxel-based morphometry, SPM). Results Twenty-seven patients showed larger postural instability in all experimental conditions. Postural sway increased with nystagmus in the eyes closed condition but not with the eyes open. Romberg’s ratio remained stable and was not different from healthy controls. Postural sway did not change with gaze position or graviceptive input. It increased with attenuated proprioceptive input and on tandem stance in both groups but Romberg’s ratio also did not differ. Cerebellar atrophy (vermal lobule VI, VIII) correlated with the severity of impaired smooth pursuit eye movements of DBN patients. Conclusions Postural ataxia of cerebellar patients with DBN cannot be explained by impaired visual feedback. Despite oscillopsia visual feedback control on cerebellar postural control seems to be preserved as postural sway was strongest on visual deprivation. The increase in postural ataxia is neither related to modulations of single components characterizing nystagmus nor to deprivation of single sensory (visual, proprioceptive) inputs usually stabilizing stance. Re-weighting of multisensory signals and/or inappropriate cerebellar motor commands might account for this postural ataxia. PMID:28056109

Maximal subtotal extirpation of the horizontal rectus extraocular muscles for the treatment of nystagmus with no null point. A report of four successful human cases.

PubMed

Sinskey, Robert M; Eshete, Almaz

2002-01-01

To evaluate the visual and restoration of normal appearance results of maximal excision of the horizontal rectus muscles in nystagmus patients. Menelik II Hospital, Addis Ababa, Ethiopia and the Sinskey Eye Institute, Santa Monica, California. The medial and lateral rectus muscles were extirpated as far back as possible with an enucleation snare in four patients with horizontal nystagmus. A complete eye examination was performed pre- and postoperatively. Using a camcorder, ocular movements were recorded before surgery, and at postop; days 1 and 40, and months 1, 3 and 10. All four patients had a marked reduction in both abnormal and normal horizontal eye movement, and improvement in objective visual acuity. Postoperative residual intermittent fine horizontal movement was recorded in the left eye in a 6 year old and in both eyes of a 41 year old patient. A residual rotary component was recorded in a 15 year-old patient. The 6 and 9 year-old patients each developed a moderate exotropia. The 15 and 41 year-old patients maintained binocular fusion with some residual ability to converge. Vision increased subjectively in all cases. Subtotal myectomy of the horizontal muscles in horizontal nystagmus with no null point was very effective in improving and/or eliminating horizontal eye movement. Restoration of normal or near normal appearance and improvement in visual acuity occurred in all cases. None of the patients complained of their loss of horizontal gaze and eye movement. More complete myectomy of the muscles should produce total elimination of both normal and abnormal horizontal eye movement including nystagmus.

Perceptual Learning in Children With Infantile Nystagmus: Effects on 2D Oculomotor Behavior.

PubMed

Huurneman, Bianca; Boonstra, F Nienke; Goossens, Jeroen

2016-08-01

To determine changes in oculomotor behavior after 10 sessions of perceptual learning on a letter discrimination task in children with infantile nystagmus (IN). Children with IN (18 children with idiopathic IN and 18 with oculocutaneous albinism accompanied by IN) aged 6 to 11 years were divided into two training groups matched on diagnosis: an uncrowded training group (n = 18) and a crowded training group (n = 18). Target letters always appeared briefly (500 ms) at an eccentric location, forcing subjects to quickly redirect their gaze. Training occurred twice per week for 5 consecutive weeks (3500 trials total). Norm data and test-retest values were collected from children with normal vision (n = 11). Outcome measures were: nystagmus characteristics (amplitude, frequency, intensity, and the expanded nystagmus acuity function); fixation stability (the bivariate contour ellipse area and foveation time); and saccadic eye movements (latencies and accuracy) made during a simple saccade task and a crowded letter-identification task. After training, saccadic responses of children with IN improved on the saccade task (latencies decreased by 14 ± 4 ms and gains increased by 0.03 ± 0.01), but not on the crowded letter task. There were also no training-induced changes in nystagmus characteristics and fixation stability. Although children with normal vision had shorter latencies in the saccade task (47 ± 14 ms at baseline), test-retest changes in their saccade gains and latencies were almost equal to the training effects observed in children with IN. Our results suggest that the improvement in visual performance after perceptual learning in children with IN is primarily due to improved sensory processing rather than improved two-dimensional oculomotor behavior.

[A sporadic case of episodic ataxia with nystagmus (EA-2)].

PubMed

Namekawa, M; Takiyama, Y; Ueno, N; Nishizawa, M

1998-05-01

A 39-year-old man with episodic ataxia with nystagmus (EA-2) was reported. He showed intermittent cerebellar dysfunction, i.e., ataxia, nystagmus, dysarthria and vertigo, since he was 10 years old. Although this attack lasted for several hours, he was normal with exception of interictal nystagmus. His parents and sister showed no episodic ataxia. We ruled out the diseases, which may cause episodic ataxia, such as multiple sclerosis, vascular disorders, metabolic disorders and congenital anomalies. He was released from the attack by treatment with acetazolamide. EA-2 has been associated with mutations in the alpha 1A-voltage dependent calcium channel gene (CACNL1A4), which is also affected in familial hemiplegic migraine (FMH) and spinocerebellar ataxia type 6 (SCA6). In EA-2, frame-shift mutation leading to premature stop and splice-site mutation leading to truncated, non-functional channel protein have been reported. However, our patient did not have the mutations in the CACNL1A4 gene that were previously reported. In addition, our patient did not have an expanded CAG allele in the CACNL1A4 gene which is responsible for SCA6. Further examination is required to address whether a new mutation exists in the CACNL1A4 gene in our patient.

Evaluation of Vestibular Functions in Patients with Vogt-Koyanagi-Harada Disease.

PubMed

Fujiwara, Keishi; Morita, Shinya; Hoshino, Kimiko; Fukuda, Atsushi; Nakamaru, Yuji; Homma, Akihiro

2017-01-01

Vogt-Koyanagi-Harada (VKH) disease is an idiopathic, multisystem autoimmune disorder characterized by bilateral, diffuse granulomatous uveitis associated with neurological, audiovestibular, and dermatological manifestations. The purpose of this study is to investigate vestibular functions in patients with VKH disease. A total of 43 patients with VKH disease in Hokkaido University Hospital were enrolled in this study. Subjective symptoms such as dizziness or vertigo and the results of various vestibular examinations including nystagmus testing, caloric testing, and vestibular-evoked myogenic potential (VEMP) testing were investigated. Eight of 42 patients (19.0%) complained of subjective vestibular symptoms. On the other hand, 12 of 28 patients (42.9%) showed nystagmus, and 7 of 15 patients (46.7%) showed unilateral or bilateral weakness in the caloric test. VEMP testing was performed for 16 patients. Seven (43.8%) and 8 (50.0%) patients were evaluated as abnormal in cervical VEMP and ocular VEMP testing, respectively. The rate of detection of nystagmus was significantly higher than that of subjective symptoms. As vestibular dysfunction in patients with VKH disease cannot be detected through history taking alone, nystagmus testing, caloric testing, and VEMP testing should be performed to evaluate vestibular functions associated with VKH disease. It is considered that abnormal VEMP findings are associated with otolith organ dysfunction. © 2017 S. Karger AG, Basel.

Head-Shaking Nystagmus Depends on Gravity

PubMed Central

Marti, Sarah; Straumann, Dominik

2005-01-01

In acute unilateral peripheral vestibular deficit, horizontal spontaneous nystagmus (SN) increases when patients lie on their affected ear. This phenomenon indicates an ipsilesional reduction of otolith function that normally suppresses asymmetric semicircular canal signals. We asked whether head-shaking nystagmus (HSN) in patients with chronic unilateral vestibular deficit following vestibular neuritis is influenced by gravity in the same way as SN in acute patients. Using a three-dimensional (3-D) turntable, patients (N = 7) were placed in different whole-body positions along the roll plane and oscillated (1 Hz, ±10°) about their head-fixed vertical axis. Eye movements were recorded with 3-D magnetic search coils. HSN was modulated by gravity: When patients lay on their affected ear, slow-phase eye velocity significantly increased upon head shaking and consisted of a horizontal drift toward the affected ear (average: 1.2°/s ±0.5 SD), which was added to the gravity-independent and directionally nonspecific SN. In conclusion, HSN in patients with chronic unilateral peripheral vestibular deficit is best elicited when they are lying on their affected ear. This suggests a gravity-dependent mechanism similar to the one observed for SN in acute patients, i.e., an asymmetric suppression of vestibular nystagmus by the unilaterally impaired otolith organs. PMID:15735939

Motion sickness is linked to nystagmus-related trigeminal brain stem input: a new hypothesis.

PubMed

Gupta, Vinod Kumar

2005-01-01

Motion sickness is a common and distressing but poorly understood syndrome associated with nausea/vomiting and autonomic nervous system accompaniments that develops in the air or space as well as on sea or land. A bidirectional aetiologic link prevails between migraine and motion-sickness. Motion sickness provokes jerk nystagmus induced by both optokinetic and vestibular stimulation. Fixation of gaze or closure of eyes generally prevents motion sickness while vestibular otolithic function is eliminated in microgravity of space, indicating a predominant pathogenetic role for visuo-sensory input. Scopolamine, dimenhydrinate, and promethazine reduce motion-related nystagmus. Contraction of extraocular muscles generates proprioceptive neural traffic and can provoke an ocular hypertensive response. It is proposed that repetitive contractions of the extraocular muscles during motion-related jerk nystagmus rapidly augment brain stem afferent input by increasing proprioceptive neural traffic through connections of the oculomotor nerves with the ophthalmic nerve in the lateral wall of the cavernous sinus as well as by raising the intraocular pressure thereby stimulating anterior segment ocular trigeminal nerve fibers. This verifiable hypothesis defines the pathophysiological basis of individual susceptibility to motion sickness, elucidates the preventive mechanism of gaze fixation or ocular closure, advances the aetiologic link between MS and migraine, rationalizes the mechanism of known preventive drugs, and explores new therapeutic possibilities.

Eye-head coordination during optokinetic stimulation in squirrel monkeys

NASA Technical Reports Server (NTRS)

Kubo, T.; Igarashi, M.; Jensen, D. W.; Homick, J. L.

1981-01-01

Head and eye movements in the yaw plane were recorded during and after optokinetic stimulation in squirrel monkeys. 1) Phasic or tonic head deviations to the side of the ocular quick phase occurred in 94% of total recordings (n = 50) during the perstimulus period, and in 75% of recordings (n = 49) during the poststimulus period. Magnitude of mean head deviation was significantly different between perstimulus and poststimulus periods. 2) Head nystagmus associated with eye nystagmus was consistently observed in seven of nine squirrel monkeys during optokinetic stimulation. Squirrel monkeys are thereby less prone to display head nystagmus than either guinea pigs, pigeons or chickens. 3) Slow phase speeds of coupled head and eye nystagmus were subjected to statistical analysis. A highly significant negative correlation was found between slow phase head and eye speeds. The correlation coefficient was - 0.81 at 60 degrees / sec stimulus (n = 119) and -0.72 at 100 degrees / sec stimulus (n = 131). The gaze speed, calculated by summing the head and eye speeds, was 59.1 plus or minus 6.8 / sec at 60 degrees / sec and 92.2 plus or minus 11.4 at 100 degrees / sec stimulus. There was no significant difference between the gaze speed in a free head condition and the eye speed when the head was fixed.

Annotated Bibliography of Reports: Supplement No. 7, 1 July 1974 - 30 June 1975,

DTIC Science & Technology

1975-06-30

studies have shown that alcohol interferes with visual control of vestibular nystagmus . The present study was designed to assess three partially inde...suppression of vestibular nystagmus ; a second involved smooth oculomotor tracking of a moving target; and a third required repetitive rapid voluntary shifts in... gaze . Oculomotor control was degraded on the first two tasks with recovery toward the initial performance level 4 hours after drinking. Performance on

Problems of space biology. Volume 50: Nystagmometry for evaulation of the status of the vestibular function

NASA Technical Reports Server (NTRS)

Levashov, M. M.; Kislyakov, V. A. (Editor)

1985-01-01

Various aspects of nystagmometry are studied, primarily those in which the study of hystagmus serves as a means to learn about the vestibular apparatus. Along with exhaustive published material, the monograph presents data from many years of research on the physioloigical mechanisms of nystagmus, the features of nystagmus when vestibular stimulation is combined with optokinetic, the pole of vertibular afferentation asymmetry in the asymmetry of reactions to optokinetic stimulus, a nystagmometric approach to studying the hydrodynamic interaction among semicircular canals, as well as several other questions. A great deal of attention is given to methods of recording nystagmus, calibrating nystagmograms, quantitative evaluation of nystagmographic material, new nystagmometric characteristics and diagnostic techniques. A diagnostic model is proposed which makes it possible to obtain important information on the condition of the vestibular system from results of vestibular testing.

Nucleus prepositus hypoglossi lesions produce a unique ocular motor syndrome

PubMed Central

Kim, Sung-Hee; Zee, David S.; du Lac, Sascha; Kim, Hyo Jung

2016-01-01

Objective: To describe the ocular motor abnormalities in 9 patients with a lesion involving the nucleus prepositus hypoglossi (NPH), a key constituent of a vestibular-cerebellar-brainstem neural network that ensures that the eyes are held steady in all positions of gaze. Methods: We recorded eye movements, including the vestibulo-ocular reflex during head impulses, in patients with vertigo and a lesion involving the NPH. Results: Our patients showed an ipsilesional-beating spontaneous nystagmus, horizontal gaze-evoked nystagmus more intense on looking toward the ipsilesional side, impaired pursuit more to the ipsilesional side, central patterns of head-shaking nystagmus, contralateral eye deviation, and decreased vestibulo-ocular reflex gain during contralesionally directed head impulses. Conclusions: We attribute these findings to an imbalance in the NPH–inferior olive–flocculus–vestibular nucleus loop, and the ocular motor abnormalities provide a new brainstem localization for patients with acute vertigo. PMID:27733568

Paraneoplastic disorders of eye movements

PubMed Central

Wray, Shirley H.; Dalmau, Josep; Chen, Athena; King, Susan; Leigh, R. John

2011-01-01

Paraneoplastic syndromes affecting the brainstem and cerebellum are reported to cause a variety of abnormalities of eye movements. Recent studies have begun to account for the mechanisms underlying several syndromes, characterized by opsoclonus, slow, or dysmetric saccades, as well as downbeat nystagmus. We provide evidence that upbeat nystagmus in a patient with pancreatic cancer reflected a cerebellar-induced imbalance of otolithic pathways: she showed marked retropulsion, and her nystagmus was dependent on head position, being absent when supine, and suppressed with convergence. In addition to anti-Hu antibodies, we demonstrated antibodies to a novel neuronal cell surface antigen. Taken with other recent studies, our findings suggest that paraneoplastic syndromes arise due to antibodies against surface neuronal antigens, including receptors and channels. Abnormal eye movements in paraneoplastic syndromes offer insights into the pathogenesis of these disorders and the opportunity to test potential therapies, such as new drugs with effects on neuronal channels. PMID:21951005

ANOMALOUS HEAD POSTURES IN STRABISMUS AND NYSTAGMUS - DIAGNOSIS AND MANAGEMENT -

PubMed Central

Teodorescu, Luminita

2015-01-01

Abnormal head positions are adopted in order to improve visual acuity, to avoid diplopia or to obtain a more comfortable binocular vision. The head can be turned or tilted toward right or left, with the chin rotated up or downwards or combination of these positions. The ophthalmologic examination including the assessment of versions leads to the diagnosis. When versions are free, the cause may be congenital nystagmus or strabismus with large angle. When versions are limited we suspect paralytic or restrictive strabismus. The head tilted to one shoulder suggests cyclotropia (IV Nerve Palsy) or congenital nystagmus. We present few of the above cases. An adequate surgical treatment can improve or correct the ocular deviation, diplopia and the abnormal head posture. Conclusions: The abnormal head posture must be assessed and treated early in order to correct the ocular position and head posture. All patient presenting abnormal head position HAD TO BE investigated by an ophthalmologist. PMID:26978880

[Convergence nystagmus and vertical gaze palsy of vascular origin].

PubMed

Jouvent, E; Benisty, S; Fenelon, G; Créange, A; Pierrot-Deseilligny, C

2005-05-01

A case of convergence-retraction nystagmus with upward vertical gaze paralysis and skew deviation (right hypotropia), without any other neurological signs, is reported. The probably vascular lesion was located at the mesodiencephalic junction, lying between the right border of the posterior commissure, the right interstitial nucleus of Cajal and the periaqueductal grey matter, accounting for the three ocular motor signs. The particular interest of this case is due to the relative smallness of the lesion.

Evaluation of vestibular functions in children with vertigo attacks

PubMed Central

Uneri, A; Turkdogan, D

2003-01-01

Aim: To examine vestibular system functions in children with episodic vertigo attacks. Methods: Thirty four children (20 males) aged 4–18 years with paroxysmal dizziness and/or vertigo attacks were evaluated. A medical history for vestibular symptoms and migraine was taken. Vestibular and auditory functions were assessed. Results: Chronic headache attacks consistent with migraine were reported in 12 children and motion sickness was reported in 30. Family history in first degree relatives was positive for migraine in 29 children and for episodic vertigo in 22. Electronystagmography and videonystagmography showed two types of nystagmus: spontaneous vestibular nystagmus (41%) and benign paroxysmal positional nystagmus (BPPN) (59%). The first type of nystagmus was assessed as a sign of vestibulopathy and the patients with BPPN were diagnosed as having benign paroxysmal positional vertigo (BPPV). Audiometric examination in four cases revealed bilateral sensory neural hearing loss in low frequencies. Pure tone averages in 30 cases were within normal ranges; however low frequencies in 28 of them were approximately 10 dB lower than high frequencies. Unilateral caloric responses diminished in eight children. Conclusions: Peripheral vestibular problems in childhood present in a wide spectrum, which varies from a short episode of dizziness to a typical vestibular attack with fluctuating sensory neural hearing loss or episodes of BPPV. A considerable number of these vestibular problems might be related to the migraine syndrome. PMID:12765917

Ocular stability and set-point adaptation

PubMed Central

Jareonsettasin, P.; Leigh, R. J.

2017-01-01

A fundamental challenge to the brain is how to prevent intrusive movements when quiet is needed. Unwanted limb movements such as tremor impair fine motor control and unwanted eye drifts such as nystagmus impair vision. A stable platform is also necessary to launch accurate movements. Accordingly, nature has designed control systems with agonist (excitation) and antagonist (inhibition) muscle pairs functioning in push–pull, around a steady level of balanced tonic activity, the set-point. Sensory information can be organized similarly, as in the vestibulo-ocular reflex, which generates eye movements that compensate for head movements. The semicircular canals, working in coplanar pairs, one in each labyrinth, are reciprocally excited and inhibited as they transduce head rotations. The relative change in activity is relayed to the vestibular nuclei, which operate around a set-point of stable balanced activity. When a pathological imbalance occurs, producing unwanted nystagmus without head movement, an adaptive mechanism restores the proper set-point and eliminates the nystagmus. Here we used 90 min of continuous 7 T magnetic field labyrinthine stimulation (MVS) in normal humans to produce sustained nystagmus simulating vestibular imbalance. We identified multiple time-scale processes towards a new zero set-point showing that MVS is an excellent paradigm to investigate the neurobiology of set-point adaptation. This article is part of the themed issue ‘Movement suppression: brain mechanisms for stopping and stillness’. PMID:28242733

On the Vertigo Due to Static Magnetic Fields

PubMed Central

Mian, Omar S.; Li, Yan; Antunes, Andre; Glover, Paul M.; Day, Brian L.

2013-01-01

Vertigo is sometimes experienced in and around MRI scanners. Mechanisms involving stimulation of the vestibular system by movement in magnetic fields or magnetic field spatial gradients have been proposed. However, it was recently shown that vestibular-dependent ocular nystagmus is evoked when stationary in homogenous static magnetic fields. The proposed mechanism involves Lorentz forces acting on endolymph to deflect semicircular canal (SCC) cupulae. To investigate whether vertigo arises from a similar mechanism we recorded qualitative and quantitative aspects of vertigo and 2D eye movements from supine healthy adults (n = 25) deprived of vision while pushed into the 7T static field of an MRI scanner. Exposures were variable and included up to 135s stationary at 7T. Nystagmus was mainly horizontal, persisted during long-exposures with partial decline, and reversed upon withdrawal. The dominant vertiginous perception with the head facing up was rotation in the horizontal plane (85% incidence) with a consistent direction across participants. With the head turned 90 degrees in yaw the perception did not transform into equivalent vertical plane rotation, indicating a context-dependency of the perception. During long exposures, illusory rotation lasted on average 50 s, including 42 s whilst stationary at 7T. Upon withdrawal, perception re-emerged and reversed, lasting on average 30 s. Onset fields for nystagmus and perception were significantly correlated (p<.05). Although perception did not persist as long as nystagmus, this is a known feature of continuous SSC stimulation. These observations, and others in the paper, are compatible with magnetic-field evoked-vertigo and nystagmus sharing a common mechanism. With this interpretation, response decay and reversal upon withdrawal from the field, are due to adaptation to continuous vestibular input. Although the study does not entirely exclude the possibility of mechanisms involving transient vestibular stimulation during movement in and out of the bore, we argue these are less likely. PMID:24205304

On the vertigo due to static magnetic fields.

PubMed

Mian, Omar S; Li, Yan; Antunes, Andre; Glover, Paul M; Day, Brian L

2013-01-01

Vertigo is sometimes experienced in and around MRI scanners. Mechanisms involving stimulation of the vestibular system by movement in magnetic fields or magnetic field spatial gradients have been proposed. However, it was recently shown that vestibular-dependent ocular nystagmus is evoked when stationary in homogenous static magnetic fields. The proposed mechanism involves Lorentz forces acting on endolymph to deflect semicircular canal (SCC) cupulae. To investigate whether vertigo arises from a similar mechanism we recorded qualitative and quantitative aspects of vertigo and 2D eye movements from supine healthy adults (n = 25) deprived of vision while pushed into the 7T static field of an MRI scanner. Exposures were variable and included up to 135s stationary at 7T. Nystagmus was mainly horizontal, persisted during long-exposures with partial decline, and reversed upon withdrawal. The dominant vertiginous perception with the head facing up was rotation in the horizontal plane (85% incidence) with a consistent direction across participants. With the head turned 90 degrees in yaw the perception did not transform into equivalent vertical plane rotation, indicating a context-dependency of the perception. During long exposures, illusory rotation lasted on average 50 s, including 42 s whilst stationary at 7T. Upon withdrawal, perception re-emerged and reversed, lasting on average 30 s. Onset fields for nystagmus and perception were significantly correlated (p<.05). Although perception did not persist as long as nystagmus, this is a known feature of continuous SSC stimulation. These observations, and others in the paper, are compatible with magnetic-field evoked-vertigo and nystagmus sharing a common mechanism. With this interpretation, response decay and reversal upon withdrawal from the field, are due to adaptation to continuous vestibular input. Although the study does not entirely exclude the possibility of mechanisms involving transient vestibular stimulation during movement in and out of the bore, we argue these are less likely.

Muscimol inactivation caudal to the interstitial nucleus of Cajal induces hemi-seesaw nystagmus

PubMed Central

Leigh, R. John; Swann, Michelle; Thurtell, Matthew J.

2010-01-01

Hemi-seesaw nystagmus (hemi-SSN) is a jerk-waveform nystagmus with conjugate torsional and disjunctive vertical components. Halmagyi et al. in Brain 117(Pt 4):789–803 (1994), reported hemi-SSN in patients with unilateral lesions in the vicinity of the Interstitial Nucleus of Cajal (INC) and suggested that an imbalance in projections from the vestibular nuclei to the INC was the source of the nystagmus. However, this hypothesis was called into question by Helmchen et al. in Exp Brain Res 119(4):436–452 (1998), who inactivated INC in monkeys with muscimol (a GABAA agonist) and induced failure of vertical gaze-holding (neural integrator) function but not hemi-SSN. We injected 0.1–0.2 μl of 2% muscimol into the supraoculomotor area, 1–2 mm dorso-lateral to the right oculomotor nucleus and caudal to the right INC. A total of seven injections in two juvenile rhesus monkeys were performed. Hemi-SSN was noted within 5–10 min after injection for six of the injections. Around the time the hemi-SSN began, a small skew deviation also developed. However, there was no limitation of horizontal or vertical eye movements, suggesting that the nearby oculomotor nucleus was not initially compromised. Limitations in eye movement range developed about ½–1 h following the injections. Clinical signs that were observed after the animal was released to his cage included a moderate to marked head tilt toward the left (contralesional) side, consistent with an ocular tilt reaction. We conclude that hemi-SSN can be caused by lesions just caudal to the INC, whereas lesions of the INC itself cause down-beat nystagmus and vertical gaze-holding failure, as demonstrated by Helmchen et al. Combined deficits may be encountered with lesions that involve several midbrain structures. PMID:20686890

A FRMD7 variant in a Japanese family causes congenital nystagmus.

PubMed

Kohmoto, Tomohiro; Okamoto, Nana; Satomura, Shigeko; Naruto, Takuya; Komori, Takahide; Hashimoto, Toshiaki; Imoto, Issei

2015-01-01

Idiopathic congenital nystagmus (ICN) is a genetically heterogeneous eye movement disorder that causes a large proportion of childhood visual impairment. Here we describe a missense variant (p.L292P) within a mutation-rich region of FRMD7 detected in three affected male siblings in a Japanese family with X-linked ICN. Combining sequence analysis and results from structural and functional predictions, we report p.L292P as a variant potentially disrupting FRMD7 function associated with X-linked ICN.

A FRMD7 variant in a Japanese family causes congenital nystagmus

PubMed Central

Kohmoto, Tomohiro; Okamoto, Nana; Satomura, Shigeko; Naruto, Takuya; Komori, Takahide; Hashimoto, Toshiaki; Imoto, Issei

2015-01-01

Idiopathic congenital nystagmus (ICN) is a genetically heterogeneous eye movement disorder that causes a large proportion of childhood visual impairment. Here we describe a missense variant (p.L292P) within a mutation-rich region of FRMD7 detected in three affected male siblings in a Japanese family with X-linked ICN. Combining sequence analysis and results from structural and functional predictions, we report p.L292P as a variant potentially disrupting FRMD7 function associated with X-linked ICN. PMID:27081518

The origin of downbeat nystagmus: an asymmetry in the distribution of on-directions of vertical gaze-velocity Purkinje cells.

PubMed

Marti, Sarah; Straumann, Dominik; Glasauer, Stefan

2005-04-01

Various hypotheses on the origin of cerebellar downbeat nystagmus (DBN) have been presented; the exact pathomechanism, however, is still not known. Based on previous anatomical and electrophysiological studies, we propose that an asymmetry in the distribution of on-directions of vertical gaze-velocity Purkinje cells leads to spontaneous upward ocular drift in cerebellar disease, and therefore, to DBN. Our hypothesis is supported by a computational model for vertical eye movements.

[Characteristics of the rat nystagmic reaction after a flight on the Kosmos-1129 biosatellite].

PubMed

Shipov, A A; Tabakova, L A

1982-01-01

The vestibular nystagmus of rats flown for 18.5 days on Cosmos-1129 was examined with reference to the latent period, number of beats, duration and the average velocity. The nystagmus was elicited by increasing angular acceleration of 10, 20, 30 degrees/sec2. As compared to the controls, the flown animals showed a significant inhibition of the nystagmic reaction (P less than less than 0.001). The inhibition can be attributed to the desynchronosis which developed inflight.

An Indirect System Identification Technique for Stable Estimation of Continuous-Time Parameters of the Vestibulo-Ocular Reflex (VOR)

NASA Technical Reports Server (NTRS)

Kukreja, Sunil L.; Wallin, Ragnar; Boyle, Richard D.

2013-01-01

The vestibulo-ocular reflex (VOR) is a well-known dual mode bifurcating system that consists of slow and fast modes associated with nystagmus and saccade, respectively. Estimation of continuous-time parameters of nystagmus and saccade models are known to be sensitive to estimation methodology, noise and sampling rate. The stable and accurate estimation of these parameters are critical for accurate disease modelling, clinical diagnosis, robotic control strategies, mission planning for space exploration and pilot safety, etc. This paper presents a novel indirect system identification method for the estimation of continuous-time parameters of VOR employing standardised least-squares with dual sampling rates in a sparse structure. This approach permits the stable and simultaneous estimation of both nystagmus and saccade data. The efficacy of this approach is demonstrated via simulation of a continuous-time model of VOR with typical parameters found in clinical studies and in the presence of output additive noise.

Single-plane compensatory phase shift of head and eye oscillations in infantile nystagmus syndrome.

PubMed

Anagnostou, Evangelos; Spengos, Konstantinos; Anastasopoulos, Dimitri

2011-09-15

A 43-year-old man with infantile nystagmus syndrome complained of "head tremor" that would occur during attempted reading. Three-dimensional, combined eye and head recordings were performed with the magnetic search coil technique in two conditions: 1) looking straight-ahead under photopic conditions without a particular attentional focus and 2) reading a simple text held one meter away. A mainly vertical-horizontal spontaneous nystagmus was evident in both conditions, whereas head nodding emerged in the second condition. The head oscillated only in the vertical plane and concomitant analysis of eye and head displacement revealed a counterphase, compensatory pattern of the first harmonic of the INS waveform. This was verified by the significant negative peak of the crosscorrelogram at zero lag. Eye-in-space (gaze) displacement during nystagmic oscillations was thereby reduced suggesting a central adaptive behavior that may have evolved to partly compensate for the abnormal eye movements during reading. Copyright © 2011 Elsevier B.V. All rights reserved.

Rare frequency of downbeat positioning nystagmus in spinocerebellar ataxia type 31.

PubMed

Yabe, Ichiro; Matsushima, Masaaki; Yoshida, Kunihiro; Ishikawa, Kinya; Shirai, Shinichi; Takahashi, Ikuko; Sasaki, Hidenao

2015-03-15

Spinocerebellar ataxia type 31 (SCA31) and spinocerebellar ataxia type 6 (SCA6) are the most frequent types of spinocerebellar degeneration in Japan. Previous reports described that it was difficult to distinguish SCA6 and SCA31 in clinical situations. There is not much difference except that the onset age of SCA31 is slightly higher than that of SCA6. Therefore we surveyed our medical records retrospectively, and then compared clinical symptoms of SCA6 and SCA31. As previously stated, the onset age of SCA31 is higher than that of SCA6. Gaze-evoked nystagmus is more frequent in SCA6 than in SCA31. The percentage in downbeat positioning nystagmus (DPN) is as high as 63% in SCA6. In contrast, DPN in SCA31 is rare and subtle. Our study suggests that the presence of DPN is an important sign that can differentiate SCA6 from SCA31 clinically. Copyright © 2015 Elsevier B.V. All rights reserved.

Three dimensional eye movements of squirrel monkeys following postrotatory tilt

NASA Technical Reports Server (NTRS)

Merfeld, D. M.; Young, L. R.; Paige, G. D.; Tomko, D. L.

1993-01-01

Three-dimensional squirrel monkey eye movements were recorded during and immediately following rotation around an earth-vertical yaw axis (160 degrees/s steady state, 100 degrees/s2 acceleration and deceleration). To study interactions between the horizontal angular vestibulo-ocular reflex (VOR) and head orientation, postrotatory VOR alignment was changed relative to gravity by tilting the head out of the horizontal plane (pitch or roll tilt between 15 degrees and 90 degrees) immediately after cessation of motion. Results showed that in addition to post rotatory horizontal nystagmus, vertical nystagmus followed tilts to the left or right (roll), and torsional nystagmus followed forward or backward (pitch) tilts. When the time course and spatial orientation of eye velocity were considered in three dimensions, the axis of eye rotation always shifted toward alignment with gravity, and the postrotatory horizontal VOR decay was accelerated by the tilts. These phenomena may reflect a neural process that resolves the sensory conflict induced by this postrotatory tilt paradigm.

[Diagnosis and treatment options in vertigo syndromes].

PubMed

Strupp, M; Dieterich, M; Zwergal, A; Brandt, T

2015-10-01

The key to diagnosing vertigo and balance disorders is systematic analysis of case history with clinical examination of the vestibular, oculomotor, and cerebral systems in particular. Important criteria for differentiating between the various vertigo syndromes are 1) the time course of symptoms, 2) the type of symptoms, 3) modulating factors, and 4) associated symptoms. For clinical examination of the vestibular system, six important tests are available: assessment of spontaneous nystagmus, head impulse test, dynamic visual acuity, subjective visual verticality, positioning manoeuvre, and the Romberg test/gait analysis with eyes open and closed. On the basis of five clinical signs (vertical divergence, central fixation nystagmus, gaze-evoked nystagmus, saccades, normal head impulse test), the clinical examination is able to differentiate between acute central and peripheral vestibular syndromes with a sensitivity and specificity of over 90%. The most relevant laboratory examinations are caloric irrigation and the video head-impulse test for canal function and the vestibular evoked myogenic potentials for otolith function. Finally, treatment is based upon four therapeutic principles: physiotherapy, pharmacotherapy, psychotherapy, and in rare cases, surgery.

Infantile aperiodic alternating nystagmus.

PubMed

Hertle, Richard W; Reznick, Leah; Yang, Dongsheng

2009-01-01

This study identifies the clinical and ocular motility characteristics of the periodic and aperiodic forms of infantile alternating nystagmus (IAPAN) and establishes the range of electrophysiological and clinical characteristics while providing clues to its presence and pathophysiology. Seventy-eight patients with ocular oscillations consistent with IAPAN were reported. Outcome variables were: age, follow-up in months, vision, strabismus, other eye and systemic abnormalities, head position, periodicity, cycle and null period duration, foveation time, waveforms, and cycle symmetry. Age range was 1 to 67 years, 50% had pure periodic and aperiodic forms, 46% had albinism, 26% had binocular acuity of 20/40 or greater, 72% had strabismus, 35% had amblyopia, 31% had other eye disease, 14% had systemic disease, 87% had an anomalous head posture, and 65% had binocular directional asymmetry. The periodic cycle averaged 224 seconds and the aperiodic cycle ranged from 2 to more than 300 seconds. One in three patients with strabismus and nystagmus periodicity had a static head posture. Fifteen percent of the infantile nystagmus syndrome population had either the periodic or aperiodic form. A changing null period is often clinically missed because of long or irregular cycles, decreased acuity, associated strabismus, and either a nonexistent or inconsistent head posture. The changing null period is easier to recognize using eye movement recordings or if the non-preferred eye is occluded and the preferred eye is examined with the head straight and gaze in primary position for at least 5 to 7 minutes. The recognition of this variant has profound treatment implications.

Can imaginary head tilt shorten postrotatory nystagmus?

PubMed

Gianna-Poulin, C C; Voelker, C C; Erickson, B; Black, F O

2001-08-01

In healthy subjects, head tilt upon cessation of a constant-velocity yaw head rotation shortens the duration of postrotatory nystagmus. The presumed mechanism for this effect is that the velocity storage of horizontal semicircular canal inputs is being discharged by otolith organ inputs which signal a constant yaw head position when the head longitudinal axis is no longer earth-vertical. In the present study, normal subjects were rotated head upright in the dark on a vertical-axis rotational chair at 60 degrees/s for 75 s and were required to perform a specific task as soon as the chair stopped. Horizontal position of the right eye was recorded with an infra-red video camera. The average eye velocity (AEV) was measured over a 30-s interval following chair acceleration/deceleration. The ratios (postrotatory AEV/perrotatory AEV) were 1.1 (SD 0.112) when subjects (N=10) kept their head erect, 0.414 (SD 0.083) when subjects tilted their head forward, 1.003 (SD 0.108) when subjects imagined watching a TV show, 1.012 (SD 0.074) when subjects imagined looking at a painting on a wall, and 0.995 (SD 0.074) when subjects imagined floating in a prone position on a lake. Thus, while actual head tilt reduced postrotatory nystagmus, the imagination tasks did not have a statistically significant effect on postrotatory nystagmus. Therefore, velocity storage does not appear to be under the influence of cortical neural signals when subjects imagine that they are floating in a prone orientation.

Spatial orientation of caloric nystagmus in semicircular canal-plugged monkeys.

PubMed

Arai, Yasuko; Yakushin, Sergei B; Cohen, Bernard; Suzuki, Jun-Ichi; Raphan, Theodore

2002-08-01

We studied caloric nystagmus before and after plugging all six semicircular canals to determine whether velocity storage contributed to the spatial orientation of caloric nystagmus. Monkeys were stimulated unilaterally with cold ( approximately 20 degrees C) water while upright, supine, prone, right-side down, and left-side down. The decline in the slow phase velocity vector was determined over the last 37% of the nystagmus, at a time when the response was largely due to activation of velocity storage. Before plugging, yaw components varied with the convective flow of endolymph in the lateral canals in all head orientations. Plugging blocked endolymph flow, eliminating convection currents. Despite this, caloric nystagmus was readily elicited, but the horizontal component was always toward the stimulated (ipsilateral) side, regardless of head position relative to gravity. When upright, the slow phase velocity vector was close to the yaw and spatial vertical axes. Roll components became stronger in supine and prone positions, and vertical components were enhanced in side down positions. In each case, this brought the velocity vectors toward alignment with the spatial vertical. Consistent with principles governing the orientation of velocity storage, when the yaw component of the velocity vector was positive, the cross-coupled pitch or roll components brought the vector upward in space. Conversely, when yaw eye velocity vector was downward in the head coordinate frame, i.e., negative, pitch and roll were downward in space. The data could not be modeled simply by a reduction in activity in the ipsilateral vestibular nerve, which would direct the velocity vector along the roll direction. Since there is no cross coupling from roll to yaw, velocity storage alone could not rotate the vector to fit the data. We postulated, therefore, that cooling had caused contraction of the endolymph in the plugged canals. This contraction would deflect the cupula toward the plug, simulating ampullofugal flow of endolymph. Inhibition and excitation induced by such cupula deflection fit the data well in the upright position but not in lateral or prone/supine conditions. Data fits in these positions required the addition of a spatially orientated, velocity storage component. We conclude, therefore, that three factors produce cold caloric nystagmus after canal plugging: inhibition of activity in ampullary nerves, contraction of endolymph in the stimulated canals, and orientation of eye velocity to gravity through velocity storage. Although the response to convection currents dominates the normal response to caloric stimulation, velocity storage probably also contributes to the orientation of eye velocity.

Tenotomy procedure alleviates the "slow to see" phenomenon in infantile nystagmus syndrome: model prediction and patient data.

PubMed

Wang, Z I; Dell'Osso, L F

2008-06-01

Our purpose was to perform a systematic study of the post-four-muscle-tenotomy procedure changes in target acquisition time by comparing predictions from the behavioral ocular motor system (OMS) model and data from infantile nystagmus syndrome (INS) patients. We studied five INS patients who underwent only tenotomy at the enthesis and reattachment at the original insertion of each (previously unoperated) horizontal rectus muscle for their INS treatment. We measured their pre- and post-tenotomy target acquisition changes using data from infrared reflection and high-speed digital video. Three key aspects were calculated and analyzed: the saccadic latency (Ls), the time to target acquisition after the target jump (Lt) and the normalized stimulus time within the cycle. Analyses were performed in MATLAB environment (The MathWorks, Natick, MA) using OMLAB software (OMtools, available from http://www.omlab.org). Model simulations were performed in MATLAB Simulink environment. The model simulation suggested an Lt reduction due to an overall foveation-quality improvement. Consistent with that prediction, improvement in Lt, ranging from approximately 200 ms to approximately 500 ms (average approximately 280 ms), was documented in all five patients post-tenotomy. The Lt improvement was not a result of a reduced Ls. INS patients acquired step-target stimuli faster post-tenotomy. This target acquisition improvement may be due to the elevated foveation quality resulting in less inherent variation in the input to the OMS. A refined behavioral OMS model, with "fast" and "slow" motor neuron pathways and a more physiological plant, successfully predicted this improved visual behavior and again demonstrated its utility in guiding ocular motor research.

Heterozygous KIDINS220/ARMS nonsense variants cause spastic paraplegia, intellectual disability, nystagmus, and obesity.

PubMed

Josifova, Dragana J; Monroe, Glen R; Tessadori, Federico; de Graaff, Esther; van der Zwaag, Bert; Mehta, Sarju G; Harakalova, Magdalena; Duran, Karen J; Savelberg, Sanne M C; Nijman, Isaäc J; Jungbluth, Heinz; Hoogenraad, Casper C; Bakkers, Jeroen; Knoers, Nine V; Firth, Helen V; Beales, Philip L; van Haaften, Gijs; van Haelst, Mieke M

2016-06-01

We identified de novo nonsense variants in KIDINS220/ARMS in three unrelated patients with spastic paraplegia, intellectual disability, nystagmus, and obesity (SINO). KIDINS220 is an essential scaffold protein coordinating neurotrophin signal pathways in neurites and is spatially and temporally regulated in the brain. Molecular analysis of patients' variants confirmed expression and translation of truncated transcripts similar to recently characterized alternative terminal exon splice isoforms of KIDINS220 KIDINS220 undergoes extensive alternative splicing in specific neuronal populations and developmental time points, reflecting its complex role in neuronal maturation. In mice and humans, KIDINS220 is alternative spliced in the middle region as well as in the last exon. These full-length and KIDINS220 splice variants occur at precise moments in cortical, hippocampal, and motor neuron development, with splice variants similar to the variants seen in our patients and lacking the last exon of KIDINS220 occurring in adult rather than in embryonic brain. We conducted tissue-specific expression studies in zebrafish that resulted in spasms, confirming a functional link with disruption of the KIDINS220 levels in developing neurites. This work reveals a crucial physiological role of KIDINS220 in development and provides insight into how perturbation of the complex interplay of KIDINS220 isoforms and their relative expression can affect neuron control and human metabolism. Altogether, we here show that de novo protein-truncating KIDINS220 variants cause a new syndrome, SINO. This is the first report of KIDINS220 variants causing a human disease. © The Author 2016. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Nystagmus responses in a group of normal humans during earth-horizontal axis rotation

NASA Technical Reports Server (NTRS)

Wall, Conrad, III; Furman, Joseph M. R.

1989-01-01

Horizontal eye movement responses to earth-horizontal yaw axis rotation were evaluated in 50 normal human subjects who were uniformly distributed in age (20-69 years) and each age group was then divided by gender. Subjects were rotated with eyes open in the dark, using clockwise and counter-clockwise 60 deg velocity trapezoids. The nystagmus slow component velocity is analyzed. It is shown that, despite large intersubject variability, parameters which describe earth-horizontal yaw axis responses are loosely interrelated, and some of them vary significantly with gender and age.

Objective appraisal of tolerance to ventriculography with various radiocontrast media (according to electronystagmography data)

NASA Technical Reports Server (NTRS)

Blagoveshchenskaya, N. S.; Puchkov, V. L.

1980-01-01

The vestibulo oculomotor reflex (nystagmus) was recorded by means of electronystagmography in 33 neurosurgical patients in dynamics prior to and after ventriculography. For contrasting the ventricular system a water soluble medium (Conrey, dimer X or Amipak) was used in 18 patients and contrast mixtures of water soluble agents in combination with Myodil emulsion in 15. It was established that after ventriculography with water soluble media the trunk vestibular reactions in all types of nystagmus grew frequently and sharply and the vestibulovegetative reactions increased markedly.

The horizontal optokinetic reflex of the opossum (Didelphis marsupialis aurita): physiological and anatomical studies in normal and early monoenucleated specimens.

PubMed

Nasi, J P; Volchan, E; Tecles, M T; Bernardes, R F; Rocha-Miranda, C E

1997-05-01

In the opossum the symmetrical binocular horizontal optokinetic nystagmus gives way to an asymmetrical monocular reflex: the nasotemporal (NT) stimulation yielding lower gain than the temporonasal (TN). In adults, monocularly enucleated at postnatal days 21-25 (pnd21-25), the gain of NT responses is markedly increased, approaching that of TN. Severe cell loss was detected in the nucleus of the optic tract (NOT) on the deafferented side in early monoenucleated specimens. In normal animals retinal afferents to the NOT are all crossed, while in animals enucleated at pnd21-25 sparse uncrossed retinal elements were observed. Although this abnormal projection might influence the increased NT response in this subgroup, it is argued that the increased symmetry in monoenucleated opossums may be the result of changes mediated by the commissural connection between both NOTs.

Interventions for eye movement disorders due to acquired brain injury.

PubMed

Rowe, Fiona J; Hanna, Kerry; Evans, Jennifer R; Noonan, Carmel P; Garcia-Finana, Marta; Dodridge, Caroline S; Howard, Claire; Jarvis, Kathryn A; MacDiarmid, Sonia L; Maan, Tallat; North, Lorraine; Rodgers, Helen

2018-03-05

Acquired brain injury can cause eye movement disorders which may include: strabismus, gaze deficits and nystagmus, causing visual symptoms of double, blurred or 'juddery' vision and reading difficulties. A wide range of interventions exist that have potential to alleviate or ameliorate these symptoms. There is a need to evaluate the effectiveness of these interventions and the timing of their implementation. We aimed to assess the effectiveness of any intervention and determine the effect of timing of intervention in the treatment of strabismus, gaze deficits and nystagmus due to acquired brain injury. We considered restitutive, substitutive, compensatory or pharmacological interventions separately and compared them to control, placebo, alternative treatment or no treatment for improving ocular alignment or motility (or both). We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (containing the Cochrane Eyes and Vision Trials Register) (2017, Issue 5), MEDLINE Ovid, Embase Ovid, CINAHL EBSCO, AMED Ovid, PsycINFO Ovid, Dissertations & Theses (PQDT) database, PsycBITE (Psychological Database for Brain Impairment Treatment Efficacy), ISRCTN registry, ClinicalTrials.gov, Health Services Research Projects in Progress (HSRProj), National Eye Institute Clinical Studies Database and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). The databases were last searched on 26 June 2017. No date or language restrictions were used in the electronic searches for trials. We manually searched the Australian Orthoptic Journal, British and Irish Orthoptic Journal, and ESA, ISA and IOA conference proceedings. We contacted researchers active in this field for information about further published or unpublished studies. We included randomised controlled trials (RCTs) of any intervention for ocular alignment or motility deficits (or both) due to acquired brain injury. Two review authors independently selected studies and extracted data. We used standard methods expected by Cochrane. We employed the GRADE approach to interpret findings and assess the quality of the evidence. We found five RCTs (116 participants) that were eligible for inclusion. These trials included conditions of acquired nystagmus, sixth cranial nerve palsy and traumatic brain injury-induced ocular motility defects. We did not identify any relevant studies of restitutive interventions.We identified one UK-based trial of a substitutive intervention, in which botulinum toxin was compared with observation in 47 people with acute sixth nerve palsy. At four months after entry into the trial, people given botulinum toxin were more likely to make a full recovery (reduction in angle of deviation within 10 prism dioptres), compared with observation (risk ratio 1.19, 95% CI 0.96 to 1.48; low-certainty evidence). These same participants also achieved binocular single vision. In the injection group only, there were 2 cases of transient ptosis out of 22 participants (9%), and 4 participants out of 22 (18%) with transient vertical deviation; a total complication rate of 24% per injection and 27% per participant. All adverse events recovered. We judged the certainty of evidence as low, downgrading for risk of bias and imprecision. It was not possible to mask investigators or participants to allocation, and the follow-up between groups varied.We identified one USA-based cross-over trial of a compensatory intervention. Oculomotor rehabilitation was compared with sham training in 12 people with mild traumatic brain injury, at least one year after the injury. We judged the evidence from this study to be very low-certainty. The study was small, data for the sham training group were not fully reported, and it was unclear if a cross-over study design was appropriate as this is an intervention with potential to have a permanent effect.We identified three cross-over studies of pharmacological interventions for acquired nystagmus, which took place in Germany and the USA. These studies investigated two classes of pharmacological interventions: GABAergic drugs (gabapentin, baclofen) and aminopyridines (4-aminopyridines (AP), 3,4-diaminopyridine (DAP)). We judged the evidence from all three studies as very low-certainty because of small numbers of participants (which led to imprecision) and risk of bias (they were cross-over studies which did not report data in a way that permitted estimation of effect size).One study compared gabapentin (up to 900 mg/day) with baclofen (up to 30 mg/day) in 21 people with pendular and jerk nystagmus. The follow-up period was two weeks. This study provides very low-certainty evidence that gabapentin may work better than baclofen in improving ocular motility and reducing participant-reported symptoms (oscillopsia). These effects may be different in pendular and jerk nystagmus, but without formal subgroup analysis it is unclear if the difference between the two types of nystagmus was chance finding. Quality of life was not reported. Ten participants with pendular nystagmus chose to continue treatment with gabapentin, and one with baclofen. Two participants with jerk nystagmus chose to continue treatment with gabapentin, and one with baclofen. Drug intolerance was reported in one person receiving gabapentin and in four participants receiving baclofen. Increased ataxia was reported in three participants receiving gabapentin and two participants receiving baclofen.One study compared a single dose of 3,4-DAP (20 mg) with placebo in 17 people with downbeat nystagmus. Assessments were made 30 minutes after taking the drug. This study provides very low-certainty evidence that 3,4-DAP may reduce the mean peak slow-phase velocity, with less oscillopsia, in people with downbeat nystagmus. Three participants reported transient side effects of minor perioral/distal paraesthesia.One study compared a single dose of 4-AP with a single dose of 3,4-DAP (both 10 mg doses) in eight people with downbeat nystagmus. Assessments were made 45 and 90 minutes after drug administration. This study provides very low-certainty evidence that both 3,4-DAP and 4-AP may reduce the mean slow-phase velocity in people with downbeat nystagmus. This effect may be stronger with 4-AP. The included studies provide insufficient evidence to inform decisions about treatments specifically for eye movement disorders that occur following acquired brain injury. No information was obtained on the cost of treatment or measures of participant satisfaction relating to treatment options and effectiveness. It was possible to describe the outcome of treatment in each trial and ascertain the occurrence of adverse events.

[Reference values for the vestibulo-ocular reflex response to the head shaking and the bithermal caloric tests].

PubMed

Molina, M I; Zapata, C; Palma, M J; López-Escámez, J A

2006-01-01

To obtain reference values for the vestibulo-ocular reflex response to the head-shaking nystagmus and the bithermal caloric test in the spanish population. A descriptive study. SET UP: General hospital. One hundred and seven healthy sex and age stratified voluntiers were included. The final sample included 97 individuals over 18 years of age. Spontaneous nystagmus (SN), head-shaking nystagmus (HSN) and caloric induced nystagmus were explored. The eyes movement was recorded by a video-oculographic system. The SN or HSN was considered significant when at least 6 consecutive beats with a slow phase velocity of 2 degrees/sec were detected. The caloric test was performed with water flow at 250mL/20 s at 30 degrees C and 44 degrees C with an interval of 10 minutes between irrigations. The percentage of canal paresis (CP) and directional preponderance (DP) was calculated using maximum slow phase velocity and the Jongkees index formula. Presence of SN, horizontal and vertical SHN, percentage of CP and DP. SN was found in 10,3% individuals (6 women and 4 men). Horizontal HSN was also observed in 9 women and 10 men (19,5%), and vertical HSN in 8 women and 10 men (18,6%). The mean and 95 percentil for CP were 13,10 and 28,60 in women and 11,02 and 27,30 in men. For DP, the mean and 95 percentil were 11,76 and 35,80 in women and 11,58 and 28,65 in men. The 5 percentils for slow phase velocity of nystagmus after each irrigation were 6,30; 5,14; 5,96; 4,02 degrees/s (left ear 44 degrees, right ear 44 degrees, left ear 30 degrees, right ear 30 degrees, respectively) in the group of women; and 5,82; 6,99; 5,67; 4,55 degrees/s in men (with the same irrigation sequence). Results presented should be considered as a population-based norms and may be useful as references for water bithermal test for VOG studies. The SN or HSN could be observed in subjects without vestibular pathology.

Effects of 4-aminopyridine on nystagmus and vestibulo-ocular reflex in ataxia-telangiectasia.

PubMed

Shaikh, Aasef G; Marti, Sarah; Tarnutzer, Alexander A; Palla, Antonella; Crawford, Thomas O; Zee, David S; Straumann, Dominik

2013-11-01

Ataxia-telangiectasia (A-T) is a progressive neurodegenerative disorder with prominent eye movement deficits localizing to the cerebellum. We sought to determine if 4-aminopyridine (4-AP), which putatively enhances the precision of Purkinje neurons, could improve the disorders of eye movements and vestibular function in A-T. The influence of 4-AP on disorders of eye movements and vestibular function was studied in four A-T patients. The effects on the cerebellar control of vestibulo-ocular reflex (VOR) was quantitatively assessed by the decay time constant of per- and post-rotational nystagmus during constant velocity en bloc rotations. The length of the VOR time constant determines the fidelity of the vestibular velocity storage, a neural mechanism that increases the bandwidth of VOR under cerebellar control. The VOR time constant was not increased in A-T patients. The latter is explained by the extent of cerebellar lesion as previously described in A-T and other cerebellar disorders. Nevertheless, 4-AP shortened the VOR time constant during horizontal rotations. Severe disinhibition of velocity storage in subjects with putatively profound cerebellar degeneration manifest periodic alternating nystagmus (PAN). Among two A-T subjects who manifested PAN, 4-AP reduced the peak slow phase velocity of the more severely affected individual and abrogated the PAN in the other. Two A-T subjects manifested horizontal and vertical spontaneous nystagmus (SN) in primary gaze, 4-AP reduced its slow phase velocity. We conclude that in subjects with A-T 4-AP has a prominent effect on the ocular motor and vestibular deficits that are ascribed to the loss of cerebellar Purkinje neurons.

A prospective clinical evaluation of augmented Anderson procedure for idiopathic infantile nystagmus.

PubMed

Gupta, Ritesh; Sharma, Pradeep; Menon, Vimala

2006-08-01

To evaluate the efficacy of the augmented Anderson procedure in idiopathic infantile nystagmus (IIN). Twelve consecutive patients older than 5 years having IIN with eccentric null position and anomalous head position were enrolled in an institution-based study. Best-corrected visual acuity (uniocular and binocular and in both null position and primary position), slit-lamp examination, fundus examination, ocular motility examination, and stereopsis using TNO were recorded. In all cases the augmented Anderson procedure, that is, recession of the yoke muscles (9-mm medial rectus and 12-mm lateral rectus), was performed. Eye movements were recorded before and 3 months after surgery in the primary position, right gaze 15 and 30 degrees, and left gaze 15 and 30 degrees. All patients had improvement in their anomalous head posture after surgery (p = 0.0001). The nystagmus intensity in the primary position decreased from 22.0 +/- 15.9 degrees cycles/s preoperatively to 10.6 +/- 10.2 degrees cycles/s at 3 months postoperatively. The change was statistically significant (p = 0.006). After surgery, binocular visual acuity using the Early Treatment Diabetic Retinopathy Study chart improved in primary position (p = 0.007). No patient developed more than mild limitation of horizontal movements after surgery. The augmented Anderson procedure is successful in correcting face turn in patients having IIN with eccentric null position, resulting in an increase in visual acuity and a decrease in nystagmus intensity in primary position. Further studies with a longer follow-up are required to assess the long-term efficacy of this procedure.

Effects of acetazolamide on infantile nystagmus syndrome waveforms: comparisons to contact lenses and convergence in a well-studied subject.

PubMed

Thurtell, M J; Dell'osso, L F; Leigh, R J; Matta, M; Jacobs, J B; Tomsak, R L

2010-07-29

To determine if acetazolamide, an effective treatment for certain inherited channelopathies, has therapeutic effects on infantile nystagmus syndrome (INS) in a well-studied subject, compare them to other therapies in the same subject and to tenotomy and reattachment (T&R) in other subjects. Eye-movement data were taken using a high-speed digital video recording system. Nystagmus waveforms were analyzed by applying an eXpanded Nystagmus Acuity Function (NAFX) at different gaze angles and determining the Longest Foveation Domain (LFD). Acetazolamide improved foveation by both a 59.7% increase in the peak value of the NAFX function (from 0.395 to 0.580) and a 70% broadening of the NAFX vs Gaze Angle curve (the LFD increased from 20° to 34°). The resulting U-shaped improvement in the percent NAFX vs Gaze Angle curve, varied from ~60% near the NAFX peak to over 1000% laterally. The therapeutic improvements in NAFX from acetazolamide (similar to T&R) were intermediate between those of soft contact lenses and convergence, the latter was best; for LFD improvements, acetazolamide and contact lenses were equivalent and less effective than convergence. Computer simulations suggested that damping the central oscillation driving INS was insufficient to produce the foveation improvements and increased NAFX values. Acetazolamide resulted in improved-foveation INS waveforms over a broadened range of gaze angles, probably acting at more than one site. This raises the question of whether hereditary INS involves an inherited channelopathy, and whether other agents with known effects on ion channels should be investigated as therapy for this condition.

Downbeat nystagmus caused by thiamine deficiency: an unusual presentation of CNS localization of large cell anaplastic CD 30-positive non-Hodgkin's lymphoma.

PubMed

Mulder, A H; Raemaekers, J M; Boerman, R H; Mattijssen, V

1999-02-01

A 24-year-old woman with a large cell anaplastic CD 30-positive T-cell non-Hodgkin's lymphoma (NHL) developed downbeat nystagmus, anisocoria, and oscillopsia. Prior to overt cerebral invasion by NHL, she had a thiamine deficiency with very low thiamine concentrations in the CSF, probably caused by protracted vomiting and increased vitamin B1 consumption by intrathecal tumor cells. We believe that her neurologic symptoms were caused -- at least partly -- by thiamine deficiency, as she reacted well to thiamine supplementation at the beginning of treatment.

Acute Cerebellar Ataxia Induced by Nivolumab

PubMed Central

Kawamura, Reina; Nagata, Eiichiro; Mukai, Masako; Ohnuki, Yoichi; Matsuzaki, Tomohiko; Ohiwa, Kana; Nakagawa, Tomoki; Kohno, Mitsutomo; Masuda, Ryota; Iwazaki, Masayuki; Takizawa, Shunya

2017-01-01

A 54-year-old woman with adenocarcinoma of the lung and lymph node metastasis experienced nystagmus and cerebellar ataxia 2 weeks after initiating nivolumab therapy. An evaluation for several autoimmune-related antibodies and paraneoplastic syndrome yielded negative results. We eventually diagnosed the patient with nivolumab-induced acute cerebellar ataxia, after excluding other potential conditions. Her ataxic gait and nystagmus resolved shortly after intravenous steroid pulse therapy followed by the administration of decreasing doses of oral steroids. Nivolumab, an immune checkpoint inhibitor, is known to induce various neurological adverse events. However, this is the first report of acute cerebellar ataxia associated with nivolumab treatment. PMID:29249765

Vestibular Responses and Motion Sickness during Pitch, Roll, and Yaw Sinusoidal Whole-Body Oscillation.

DTIC Science & Technology

1990-03-01

vestibulo-ocular reflex) pitch, ’- 11’, 1 3c\\ " ’ I...[ 16. PRC C nystagmus 1 ’>"( k 1.,’ roll V-) , __r_ ,_t_ _ _ 17. SECURITY CLASSIFICATION 1S SECURITY...of amplification of EOG was 3.0 s. Because of the position of subjEcts’ heads relative to the axis of rotation, vertical nystagmus comprised the VOR...response in Groups I and III. To achieve measurement of the VOR in Groups IV and V, subjects were instructed to gaze downward, 30 deg in Group IV, and

Stabilization of gaze during circular locomotion in darkness. II. Contribution of velocity storage to compensatory eye and head nystagmus in the running monkey

NASA Technical Reports Server (NTRS)

Solomon, D.; Cohen, B.

1992-01-01

1. Yaw eye in head (Eh) and head on body velocities (Hb) were measured in two monkeys that ran around the perimeter of a circular platform in darkness. The platform was stationary or could be counterrotated to reduce body velocity in space (Bs) while increasing gait velocity on the platform (Bp). The animals were also rotated while seated in a primate chair at eccentric locations to provide linear and angular accelerations similar to those experienced while running. 2. Both animals had head and eye nystagmus while running in darkness during which slow phase gaze velocity on the body (Gb) partially compensated for body velocity in space (Bs). The eyes, driven by the vestibuloocular reflex (VOR), supplied high-frequency characteristics, bringing Gb up to compensatory levels at the beginning and end of the slow phases. The head provided substantial gaze compensation during the slow phases, probably through the vestibulocollic reflex (VCR). Synchronous eye and head quick phases moved gaze in the direction of running. Head movements occurred consistently only when animals were running. This indicates that active body and limb motion may be essential for inducing the head-eye gaze synergy. 3. Gaze compensation was good when running in both directions in one animal and in one direction in the other animal. The animals had long VOR time constants in these directions. The VOR time constant was short to one side in one animal, and it had poor gaze compensation in this direction. Postlocomotory nystagmus was weaker after running in directions with a long VOR time constant than when the animals were passively rotated in darkness. We infer that velocity storage in the vestibular system had been activated to produce continuous Eh and Hb during running and to counteract postrotatory afterresponses. 4. Continuous compensatory gaze nystagmus was not produced by passive eccentric rotation with the head stabilized or free. This indicates that an aspect of active locomotion, most likely somatosensory feedback, was responsible for activating velocity storage. 5. Nystagmus was compared when an animal ran in darkness and in light. the beat frequency of eye and head nystagmus was lower, and the quick phases were larger in darkness. The duration of head and eye quick phases covaried. Eye quick phases were larger when animals ran in darkness than when they were passively rotated. The maximum velocity and duration of eye quick phases were the same in both conditions. 6. The platform was counterrotated under one monkey in darkness while it ran in the direction of its long vestibular time constant.(ABSTRACT TRUNCATED AT 400 WORDS).

Modelling drug modulation of nystagmus.

PubMed

Glasauer, Stefan; Rössert, Christian

2008-01-01

A better understanding of the neural and functional mechanisms underlying drug-induced changes in pathological nystagmus is likely to improve medical treatment. A treatment option for downbeat nystagmus (DBN), a common form of acquired fixation nystagmus that often occurs with cerebellar degeneration, is low doses of the potassium channel blocker 4-aminopyridine (4-AP). The upward ocular drift in DBN has a spontaneous and a vertical gaze-evoked component. Detailed analysis of the effect of 4-AP in patients showed that the drug consistently improved the gaze-evoked component, but had less effect in reducing the spontaneous drift. We show by a combination of computational modelling at the systems level and at the neuronal level how this differential effect can be investigated. We have previously postulated that DBN is caused by damage to the floccular lobe (FL). 4-AP, which has been shown to increase the excitability of Purkinje cells (PCs) in slice experiments, may thus suppress DBN by partly restoring floccular function. We simulated the effect of low concentrations of 4-AP on the cellular level using a multicompartment model of a PC, in which we changed ion channel properties to simulate damage. The transition from the cellular level to the systems level was achieved by constructing a population response. Systems level modelling predicted that the effect of 4-AP on the PCs should reduce DBN, but the predicted effect on the gaze-dependent component was less than is observed in patients. Our results suggest that the beneficial effect of 4-AP on DBN cannot be solely explained by its effect at the neuronal level of PCs, and suggests added effects at the level of the population of neurons.

HINTS to diagnose stroke in the acute vestibular syndrome: three-step bedside oculomotor examination more sensitive than early MRI diffusion-weighted imaging.

PubMed

Kattah, Jorge C; Talkad, Arun V; Wang, David Z; Hsieh, Yu-Hsiang; Newman-Toker, David E

2009-11-01

Acute vestibular syndrome (AVS) is often due to vestibular neuritis but can result from vertebrobasilar strokes. Misdiagnosis of posterior fossa infarcts in emergency care settings is frequent. Bedside oculomotor findings may reliably identify stroke in AVS, but prospective studies have been lacking. The authors conducted a prospective, cross-sectional study at an academic hospital. Consecutive patients with AVS (vertigo, nystagmus, nausea/vomiting, head-motion intolerance, unsteady gait) with >or=1 stroke risk factor underwent structured examination, including horizontal head impulse test of vestibulo-ocular reflex function, observation of nystagmus in different gaze positions, and prism cross-cover test of ocular alignment. All underwent neuroimaging and admission (generally <72 hours after symptom onset). Strokes were diagnosed by MRI or CT. Peripheral lesions were diagnosed by normal MRI and clinical follow-up. One hundred one high-risk patients with AVS included 25 peripheral and 76 central lesions (69 ischemic strokes, 4 hemorrhages, 3 other). The presence of normal horizontal head impulse test, direction-changing nystagmus in eccentric gaze, or skew deviation (vertical ocular misalignment) was 100% sensitive and 96% specific for stroke. Skew was present in 17% and associated with brainstem lesions (4% peripheral, 4% pure cerebellar, 30% brainstem involvement; chi(2), P=0.003). Skew correctly predicted lateral pontine stroke in 2 of 3 cases in which an abnormal horizontal head impulse test erroneously suggested peripheral localization. Initial MRI diffusion-weighted imaging was falsely negative in 12% (all <48 hours after symptom onset). Skew predicts brainstem involvement in AVS and can identify stroke when an abnormal horizontal head impulse test falsely suggests a peripheral lesion. A 3-step bedside oculomotor examination (HINTS: Head-Impulse-Nystagmus-Test-of-Skew) appears more sensitive for stroke than early MRI in AVS.

Can emergency physicians accurately and reliably assess acute vertigo in the emergency department?

PubMed

Vanni, Simone; Nazerian, Peiman; Casati, Carlotta; Moroni, Federico; Risso, Michele; Ottaviani, Maddalena; Pecci, Rudi; Pepe, Giuseppe; Vannucchi, Paolo; Grifoni, Stefano

2015-04-01

To validate a clinical diagnostic tool, used by emergency physicians (EPs), to diagnose the central cause of patients presenting with vertigo, and to determine interrater reliability of this tool. A convenience sample of adult patients presenting to a single academic ED with isolated vertigo (i.e. vertigo without other neurological deficits) was prospectively evaluated with STANDING (SponTAneousNystagmus, Direction, head Impulse test, standiNG) by five trained EPs. The first step focused on the presence of spontaneous nystagmus, the second on the direction of nystagmus, the third on head impulse test and the fourth on gait. The local standard practice, senior audiologist evaluation corroborated by neuroimaging when deemed appropriate, was considered the reference standard. Sensitivity and specificity of STANDING were calculated. On the first 30 patients, inter-observer agreement among EPs was also assessed. Five EPs with limited experience in nystagmus assessment volunteered to participate in the present study enrolling 98 patients. Their average evaluation time was 9.9 ± 2.8 min (range 6-17). Central acute vertigo was suspected in 16 (16.3%) patients. There were 13 true positives, three false positives, 81 true negatives and one false negative, with a high sensitivity (92.9%, 95% CI 70-100%) and specificity (96.4%, 95% CI 93-38%) for central acute vertigo according to senior audiologist evaluation. The Cohen's kappas of the first, second, third and fourth steps of the STANDING were 0.86, 0.93, 0.73 and 0.78, respectively. The whole test showed a good inter-observer agreement (k = 0.76, 95% CI 0.45-1). In the hands of EPs, STANDING showed a good inter-observer agreement and accuracy validated against the local standard of care. © 2015 Australasian College for Emergency Medicine and Australasian Society for Emergency Medicine.

Paraneoplastic downbeat nystagmus associated with cerebellar hypermetabolism especially in the nodulus.

PubMed

Choi, Seo Young; Park, Seong-Ho; Kim, Hyo-Jung; Kim, Ji-Soo

2014-08-15

A 52-year-old man with vertigo and imbalance for two weeks showed spontaneous downbeat (DBN), horizontal gaze-evoked, and positional apogeotropic nystagmus along with severe limb and truncal ataxia. Gadolinium-enhanced brain MRI was normal, but whole body and brain 2-deoxy-2-[F18]fluoro-d-glucose-positron emission tomography revealed hypermetabolism in the right lower lobe of the lung and the cerebellum, especially in the nodulus. The lesion in the lung was confirmed as mixed cell carcinoma. Paraneoplastic DBN may be associated with cerebellar hypermetabolism, especially in the nodulus. Copyright © 2014 Elsevier B.V. All rights reserved.

Automated nystagmus analysis. [on-line computer technique for eye data processing

NASA Technical Reports Server (NTRS)

Oman, C. M.; Allum, J. H. J.; Tole, J. R.; Young, L. R.

1973-01-01

Several methods have recently been used for on-line analysis of nystagmus: A digital computer program has been developed to accept sampled records of eye position, detect fast phase components, and output cumulative slow phase position, continuous slow phase velocity, instantaneous fast phase frequency, and other parameters. The slow phase velocity is obtained by differentiation of the calculated cumulative position rather than the original eye movement record. Also, a prototype analog device has been devised which calculates the velocity of the slow phase component during caloric testing. Examples of clinical and research eye movement records analyzed with these devices are shown.

The influence of gravitoinertial force level on oculomotor and perceptual responses to sudden stop stimulation

NASA Technical Reports Server (NTRS)

Dizio, Paul; Lackner, James R.; Evanoff, John N.

1987-01-01

The goal of this investigation was to determine whether the vestibular response to vertical, z-axis body rotation in the dark is influenced by the magnitude of gravitoinertial force. The parameters measured were the nystagmus and the duration of illusory self-motion elicited in blindfolded subjects by cessation of such rotation during the free-fall, high, and terrestrial force phases of parabolic flight maneuvers. The pattern of findings is consistent with the responses that were observed earlier to constant levels of Coriolis cross-coupled stimulation during parabolic flight maneuvers both in terms of the mode of nystagmus suppression and the effect of G-level.

Gaze-evoked nystagmus: a case report and literature review.

PubMed

Rett, Doug

2007-09-01

A sustained gaze-evoked nystagmus (GEN) is an important ocular finding that may indicate serious neurologic pathology. It is also a finding that can be missed easily during routine extraocular muscle (EOM) testing. This report presents a case that should familiarize the reader with GEN and presents a novel approach to testing EOM function. The mother of an otherwise healthy 4-year-old girl noted that her daughter's eyes crossed occasionally, the right lid drooped on one occasion, and she had been having strange headaches. An asymmetric, sustained, gaze-evoked nystagmus was detected using a different approach to EOM testing. Magnetic resonance imaging found a large, brainstem astrocytoma in the cerebellar-pontine angle. EOM function often is overlooked or underperformed but is an important part of the battery of clinical tests to rule out neurologic problems. Most forms of EOM testing will check for muscle palsies but little else. If the time is taken to extend the patient's gaze to the extreme ends, to attempt to hold the gaze in all 9 positions, and to maintain an accurate speed, the clinician can stand to gain much more information regarding the neurologic system.

Kestenbaum procedure on the vertical rectus muscles with simultaneous compensation of the induced cyclodeviation for nystagmus patients with chin-up or chin-down head posture.

PubMed

Schild, A M; Fricke, J; Rüssmann, W; Neugebauer, A

2009-10-01

Kestenbaum surgery is performed for nystagmus-related abnormal head posture, and symmetrically changes the position of both eyes to shift the null point to the primary position. Most patients with infantile nystagmus have their null point zone in a lateral gaze position. Less frequently, surgery can be performed to reduce chin-up or chin-down head posture. We report indications for, and the results of eight consecutive interventions performed according to the Kestenbaum principle for the reduction of a chin-up or chin-down head posture. In a retrospective study, the clinical findings for eight patients who consecutively underwent treatment in the University Eye Hospital of Cologne between 2001 and 2007 were investigated. The patients were aged 6 to 16 years; median age was 6.5 years. For all patients, surgery was to correct a chin-up or chin-down head posture due to infantile nystagmus. Preoperatively, five patients showed a chin-down, three a chin-up head posture. All vertical rectus muscles were recessed or tucked between 6 and 7 mm; the resulting cyclodeviation was reduced by an intervention on the superior oblique muscles (6 to 8 mm tucking, in the case of chin-down, or recession in the case of chin-up head posture). Surgery was successful in seven of the eight patients, with a reduction of the vertical head posture to less than 10 degrees. In the cases of chin-down posture, head posture was reduced to between 0 degrees and a maximum of 20 degrees in one case postoperatively (before the operation 20 degrees to 35 degrees ); in the cases of chin-up posture, to less than 8 degrees (before the operation 25 degrees to 35 degrees). One case showed no postoperative improvement in chin-down posture but a head turn to the left of up to 20 degrees; another case had a remaining chin-up posture of 8 degrees with a right turn of 15 degrees . Binocular vision was better or the same in all cases after surgery. For nystagmus patients with chin-up or chin-down head posture, surgery for bilateral parallel shifting of the eyes can considerably improve the head posture. It is possible to compensate the induced cyclodeviation at the same time by bilateral surgery on the superior oblique muscles.

[Serial change of perilymphatic potassium ion concentration in the scala tympani after introducing KCl-solution into the guinea pigs' tympanic cavity].

PubMed

Ikeno, K

1990-09-01

Characteristic nystagmus similar to the Meniere's attack could be observed after introducing KCl solution into the tympanic cavity of guinea pigs. To confirm the fact that this nystagmus was provoked by the high perilymphatic potassium ion concentration, the K+ activity of perilymph was recorded serially through the K+ specific microelectrode inserted into the scala tympani. The rapid increment of K+ activity reached maximum at 120 minutes after introducing KCl solution, and then it decreased gradually to a half of the maximum activity. However, such change of perilymphatic potassium ion concentration was not observed by introducing sucrose solution as control.

The Newcastle Papers in Industrial Medicine over the Last 21 Years

PubMed Central

Browne, R. C.

1968-01-01

The Chair of Industrial Health in Newcastle upon Tyne was first filled in March, 1946. For about the first 12 years diseases of coalminers posed the main clinical and research problems. They stimulated surveys of pneumoconiosis in the Durham and Northumberland coalfields and led to studies of ϰ-ray viewing techniques and of the relationship of radiological appearance to symptoms in dust disease of the chest. A section of medical statistics, now headed by a professor, was one of the earliest elements of the department to be inaugurated, and this was followed by a section of pulmonary physiology. The emphasis on industrial pulmonary disease, on which 14 papers have been published, has gradually widened and become transferred from coal to beryllium, asbestos, and antimony. Coalminers with nystagmus were shown to be socially similar to miners without the disease but psychologically less stable. They also suffered from a breakdown of their binocular vision. A darkness-induced nystagmus was experimentally produced in kittens, with increasing difficulty as they became older. Decompression sickness has become an important interest. In the build-up of the department demands for occupational hygiene soon led to the formation of a section which started work on the decalcification of teeth by organic acids in a fruit salt factory. Lead poisoning in shipbreakers and smelters, and vanadium poisoning in fitters and gasmakers were studied jointly with clinicians. The thermal decomposition of protective coatings and welding rods has also attracted research, as also have the noise levels in a glassblowing school and in several power stations. All medical, engineering, and public health undergraduates are taught by the department, and also the honours chemists. All medical undergraduates and honours engineers are examined in industrial health. The departmental budget (including the field service) is about £54,000, and the department inhabits 12,660 sq. ft. of space. PMID:5663422

Task-induced Changes in Idiopathic Infantile Nystagmus Vary with Gaze.

PubMed

Salehi Fadardi, Marzieh; Bathke, Arne C; Harrar, Solomon W; Abel, Larry Allen

2017-05-01

Investigations of infantile nystagmus syndrome (INS) at center or at the null position have reported that INS worsens when visual demand is combined with internal states, e.g. stress. Visual function and INS parameters such as foveation time, frequency, amplitude, and intensity can also be influenced by gaze position. We hypothesized that increases from baseline in visual demand and mental load would affect INS parameters at the null position differently than at other gaze positions. Eleven participants with idiopathic INS were asked to determine the direction of Tumbling-E targets, whose visual demand was varied through changes in size and contrast, using a staircase procedure. Targets appeared between ±25° in 5° steps. The task was repeated with both mental arithmetic and time restriction to impose higher mental load, confirmed through subjective ratings and concurrent physiological measurements. Within-subject comparisons were limited to the null and 15° away from it. No significant main effects of task on any INS parameters were found. At both locations, high mental load worsened task performance metrics, i.e. lowest contrast (P = .001) and smallest optotype size reached (P = .012). There was a significant interaction between mental load and gaze position for foveation time (P = .02) and for the smallest optotype reached (P = .028). The increase in threshold optotype size from the low to high mental load was greater at the null than away from it. During high visual demand, foveation time significantly decreased from baseline at the null as compared to away from it (mean difference ± SE: 14.19 ± 0.7 msec; P = .010). Under high visual demand, the effects of increased mental load on foveation time and visual task performance differed at the null as compared to 15° away from it. Assessment of these effects could be valuable when evaluating INS clinically and when considering its impact on patients' daily activities.

Development of vestibular function: biochemical, morphological and electronystagmographical assessment in the rat.

PubMed

Meza, G; Acuña, D; Gutiérrez, A; Merchan, J M; Rueda, J

1996-07-01

Glutamate decarboxylase and choline acetyltransferase were measured in homogenated ampullar cristae of rats during development from postnatal day 13 to 60 to determine changes in levels of these enzymes during early postnatal development. Afferent and efferent innervation of the hair cells of the developing cristae were studied using electron microscopy. In parallel, groups of rats, postrotatory nystagmus were used to assess the development of semicircular canal function during the same time interval. The level of glutamate decarboxylase was high on postnatal day 15 and did not change notably over the remaining days to day 60. Activity of choline acetyltransferase was nearly absent at day 15, but reached levels seen in mature animals by day 17, and remained almost unchanged thereafter. In contrast, as revealed by electronmicroscopy, afferent and efferent innervation appeared to be mature by day 8. Postrotatory nystagmus presented the adult-like features from day 19 onward. According to these results, a role for glutamate decarboxylase in afferent transmission is suggested by the parallel development of levels of glutamate decarboxylase and afferent innervation of the ampullary cristae. The finding of a similar time course of development of choline acetyltransferase levels and postrotatory nystagmus suggests that a cholinergic efferent innervation is involved in the onset of vestibular-ocular function.

Motion sickness susceptibility in parabolic flight and velocity storage activity

NASA Technical Reports Server (NTRS)

Dizio, Paul; Lackner, James R.

1991-01-01

In parabolic flight experiments, postrotary nystagmus is as found to be differentially suppressed in free fall (G) and in a high gravitoinertial force (1.8 G) background relative to 1 G. In addition, the influence of postrotary head movements on nystagmus suppression was found to be contingent on G-dependency of the velocity storage and dumping mechanisms. Here, susceptibility to motion sickness during head movements in 0 G and 1.8 G was rank-correlated with the following: (1) the decay time constant of the slow phase velocity of postrotary nystagmus under 1 G, no head movement, baseline conditions, (2) the extent of time constant reduction elicited in 0 G and 1.8 G; (3) the extent of time constant reduction elicited by head tilts in 1 G; and (4) changes in the extent of time constants reduction in 0 G and 1.8 G over repeated tests. Susceptibility was significantly correlated with the extent to which a head movement reduced the time constant in 1 G, was weakly correlated with the baseline time constant, but was not correlated with the extent of reduction in 0 G or 1.8 G. This pattern suggests a link between mechanisms evoking symptoms of space motion sickness and the mechanisms of velocity storage and dumping. Experimental means of evaluating this link are described.

Cortical modulation of the nucleus of the optic tract in the rabbit.

PubMed

Pettorossi, V E; Troiani, D

1983-09-01

We analyzed in rabbits the relationships between the temporooccipital nystagmogenic cortex (NGC)--the region sited at the border between cortical areas 17, 21, and 22--and the nucleus of the optic tract (NOT). Two experimental approaches were used: (a) eye movement analysis before and after electrolytic lesion of the NOT region provided an indication of the importance of the NOT for the interaction between the ocular nystagmus elicited by natural optokinetic stimulation (OKN) and the nystagmus evoked by electrical stimulation of the nystagmogenic area; (b) NOT direction-selective and velocity-sensitive units were tested with single shock or repetitive electrical stimulation of the nystagmogenic region. Single-shock stimulation evoked single or multiple spikes in 50% of NOT units analyzed and repetitive stimuli induced prolonged facilitation and inhibitory rebounds in 70% of the units tested. Comparison of orthodromic activation latencies of the NOT cells (3.2 and 6.1 ms) after cortical stimulation and of antidromic activation latencies of cortical nystagmogenic units (2.6 ms) after NOT shocks, suggested monosynaptic as well as polysynaptic connections between the temporooccipital cortex and the NOT. The existence of such cortical-NOT linkage indicates that the NOT is intercalated between the cortex and the oculomotor centers and represents the most probable site of interaction of the cortical nystagmus pathway with the optokinetic reflex arc.

A field study of the accuracy and reliability of a biometric iris recognition system.

PubMed

Latman, Neal S; Herb, Emily

2013-06-01

The iris of the eye appears to satisfy the criteria for a good anatomical characteristic for use in a biometric system. The purpose of this study was to evaluate a biometric iris recognition system: Mobile-Eyes™. The enrollment, verification, and identification applications were evaluated in a field study for accuracy and reliability using both irises of 277 subjects. Independent variables included a wide range of subject demographics, ambient light, and ambient temperature. A sub-set of 35 subjects had alcohol-induced nystagmus. There were 2710 identification and verification attempts, which resulted in 1,501,340 and 5540 iris comparisons respectively. In this study, the system successfully enrolled all subjects on the first attempt. All 277 subjects were successfully verified and identified on the first day of enrollment. None of the current or prior eye conditions prevented enrollment, verification, or identification. All 35 subjects with alcohol-induced nystagmus were successfully verified and identified. There were no false verifications or false identifications. Two conditions were identified that potentially could circumvent the use of iris recognitions systems in general. The Mobile-Eyes™ iris recognition system exhibited accurate and reliable enrollment, verification, and identification applications in this study. It may have special applications in subjects with nystagmus. Copyright © 2012 Forensic Science Society. Published by Elsevier Ireland Ltd. All rights reserved.

Real-time computer-based visual feedback improves visual acuity in downbeat nystagmus - a pilot study.

PubMed

Teufel, Julian; Bardins, S; Spiegel, Rainer; Kremmyda, O; Schneider, E; Strupp, M; Kalla, R

2016-01-04

Patients with downbeat nystagmus syndrome suffer from oscillopsia, which leads to an unstable visual perception and therefore impaired visual acuity. The aim of this study was to use real-time computer-based visual feedback to compensate for the destabilizing slow phase eye movements. The patients were sitting in front of a computer screen with the head fixed on a chin rest. The eye movements were recorded by an eye tracking system (EyeSeeCam®). We tested the visual acuity with a fixed Landolt C (static) and during real-time feedback driven condition (dynamic) in gaze straight ahead and (20°) sideward gaze. In the dynamic condition, the Landolt C moved according to the slow phase eye velocity of the downbeat nystagmus. The Shapiro-Wilk test was used to test for normal distribution and one-way ANOVA for comparison. Ten patients with downbeat nystagmus were included in the study. Median age was 76 years and the median duration of symptoms was 6.3 years (SD +/- 3.1y). The mean slow phase velocity was moderate during gaze straight ahead (1.44°/s, SD +/- 1.18°/s) and increased significantly in sideward gaze (mean left 3.36°/s; right 3.58°/s). In gaze straight ahead, we found no difference between the static and feedback driven condition. In sideward gaze, visual acuity improved in five out of ten subjects during the feedback-driven condition (p = 0.043). This study provides proof of concept that non-invasive real-time computer-based visual feedback compensates for the SPV in DBN. Therefore, real-time visual feedback may be a promising aid for patients suffering from oscillopsia and impaired text reading on screen. Recent technological advances in the area of virtual reality displays might soon render this approach feasible in fully mobile settings.

Emergency department documentation templates: variability in template selection and association with physical examination and test ordering in dizziness presentations.

PubMed

Kerber, Kevin A; Hofer, Timothy P; Meurer, William J; Fendrick, A Mark; Morgenstern, Lewis B

2011-03-24

Clinical documentation systems, such as templates, have been associated with process utilization. The T-System emergency department (ED) templates are widely used but lacking are analyses of the templates association with processes. This system is also unique because of the many different template options available, and thus the selection of the template may also be important. We aimed to describe the selection of templates in ED dizziness presentations and to investigate the association between items on templates and process utilization. Dizziness visits were captured from a population-based study of EDs that use documentation templates. Two relevant process outcomes were assessed: head computerized tomography (CT) scan and nystagmus examination. Multivariable logistic regression was used to estimate the probability of each outcome for patients who did or did not receive a relevant-item template. Propensity scores were also used to adjust for selection effects. The final cohort was 1,485 visits. Thirty-one different templates were used. Use of a template with a head CT item was associated with an increase in the adjusted probability of head CT utilization from 12.2% (95% CI, 8.9%-16.6%) to 29.3% (95% CI, 26.0%-32.9%). The adjusted probability of documentation of a nystagmus assessment increased from 12.0% (95%CI, 8.8%-16.2%) when a nystagmus-item template was not used to 95.0% (95% CI, 92.8%-96.6%) when a nystagmus-item template was used. The associations remained significant after propensity score adjustments. Providers use many different templates in dizziness presentations. Important differences exist in the various templates and the template that is used likely impacts process utilization, even though selection may be arbitrary. The optimal design and selection of templates may offer a feasible and effective opportunity to improve care delivery.

Topographic analysis of the skull vibration-induced nystagmus test with piezoelectric accelerometers and force sensors.

PubMed

Dumas, Georges; Lion, Alexis; Perrin, Philippe; Ouedraogo, Evariste; Schmerber, Sébastien

2016-03-23

Vibration-induced nystagmus is elicited by skull or posterior cervical muscle stimulations in patients with vestibular diseases. Skull vibrations delivered by the skull vibration-induced nystagmus test are known to stimulate the inner ear structures directly. This study aimed to measure the vibration transfer at different cranium locations and posterior cervical regions to contribute toward stimulus topographic optimization (experiment 1) and to determine the force applied on the skull with a hand-held vibrator to study the test reproducibility and provide recommendations for good clinical practices (experiment 2). In experiment 1, a 100 Hz hand-held vibrator was applied on the skull (vertex, mastoids) and posterior cervical muscles in 11 healthy participants. Vibration transfer was measured by piezoelectric sensors. In experiment 2, the vibrator was applied 30 times by two experimenters with dominant and nondominant hands on a mannequin equipped to measure the force. Experiment 1 showed that after unilateral mastoid vibratory stimulation, the signal transfer was higher when recorded on the contralateral mastoid than on the vertex or posterior cervical muscles (P<0.001). No difference was observed between the different vibratory locations when vibration transfer was measured on vertex and posterior cervical muscles. Experiment 2 showed that the force applied to the mannequin varied according to the experimenters and the handedness, higher forces being observed with the most experienced experimenter and with the dominant hand (10.3 ± 1.0 and 7.8 ± 2.9 N, respectively). The variation ranged from 9.8 to 29.4% within the same experimenter. Bone transcranial vibration transfer is more efficient from one mastoid to the other mastoid than other anatomical sites. The mastoid is therefore the optimal site for skull vibration-induced nystagmus test in patients with unilateral vestibular lesions and enables a stronger stimulation of the healthy side. In clinical practice, the vibrator should be placed on the mastoid and should be held by the clinician's dominant hand.

Effect of head pitch and roll orientations on magnetically induced vertigo.

PubMed

Mian, Omar S; Li, Yan; Antunes, Andre; Glover, Paul M; Day, Brian L

2016-02-15

Lying supine in a strong magnetic field, such as in magnetic resonance imaging scanners, can induce a perception of whole-body rotation. The leading hypothesis to explain this invokes a Lorentz force mechanism acting on vestibular endolymph that acts to stimulate semicircular canals. The hypothesis predicts that the perception of whole-body rotation will depend on head orientation in the field. Results showed that the direction and magnitude of apparent whole-body rotation while stationary in a 7 T magnetic field is influenced by head orientation. The data are compatible with the Lorentz force hypothesis of magnetic vestibular stimulation and furthermore demonstrate the operation of a spatial transformation process from head-referenced vestibular signals to Earth-referenced body motion. High strength static magnetic fields are known to induce vertigo, believed to be via stimulation of the vestibular system. The leading hypothesis (Lorentz forces) predicts that the induced vertigo should depend on the orientation of the magnetic field relative to the head. In this study we examined the effect of static head pitch (-80 to +40 deg; 12 participants) and roll (-40 to +40 deg; 11 participants) on qualitative and quantitative aspects of vertigo experienced in the dark by healthy humans when exposed to the static uniform magnetic field inside a 7 T MRI scanner. Three participants were additionally examined at 180 deg pitch and roll orientations. The effect of roll orientation on horizontal and vertical nystagmus was also measured and was found to affect only the vertical component. Vertigo was most discomforting when head pitch was around 60 deg extension and was mildest when it was around 20 deg flexion. Quantitative analysis of vertigo focused on the induced perception of horizontal-plane rotation reported online with the aid of hand-held switches. Head orientation had effects on both the magnitude and the direction of this perceived rotation. The data suggest sinusoidal relationships between head orientation and perception with spatial periods of 180 deg for pitch and 360 deg for roll, which we explain is consistent with the Lorentz force hypothesis. The effects of head pitch on vertigo and previously reported nystagmus are consistent with both effects being driven by a common vestibular signal. To explain all the observed effects, this common signal requires contributions from multiple semicircular canals. © 2015 The Authors. The Journal of Physiology published by John Wiley & Sons Ltd on behalf of The Physiological Society.

4-aminopyridine restores vertical and horizontal neural integrator function in downbeat nystagmus.

PubMed

Kalla, Roger; Glasauer, Stefan; Büttner, Ulrich; Brandt, Thomas; Strupp, Michael

2007-09-01

Downbeat nystagmus (DBN), the most common form of acquired fixation nystagmus, is often caused by cerebellar degeneration, especially if the vestibulo-cerebellum is involved. The upward ocular drift in DBN has a spontaneous and a vertical gaze-evoked component. Since cerebellar involvement is suspected to be the underlying pathomechanism of DBN, we tested in 15 patients with DBN whether the application of the potassium-channel blocker 4-aminopyridine (4-AP), which increases the excitability of cerebellar Purkinje cells as shown in animal experiments, reduces the vertical ocular drift leading to nystagmus. Fifteen age-matched healthy subjects served as the control group. 4-AP may affect spontaneous drift or gaze-evoked drift by either enhancing visual fixation ability or restoring vision-independent gaze holding. We therefore recorded 3D slow-phase eye movements using search coils during attempted fixation in nine different eye positions and with or without a continuously visible target before and 45 min after ingestion of 10mg 4-AP. Since the effect of 4-AP may depend on the associated etiology, we divided our patients into three groups (cerebellar atrophy, n = 4; idiopathic DBN, n = 5; other etiology, n = 6). 4-AP decreased DBN during gaze straight ahead in 12 of 15 patients. Statistical analysis showed that improvement occurred predominantly in patients with cerebellar atrophy, in whom the drift was reduced from -4.99 +/- 1.07 deg/s (mean +/- SE) before treatment to -0.60 +/- 0.82 deg/s afterwards. Regression analysis of slow-phase velocity (SPV) in different eye positions revealed that vertical and horizontal gaze-evoked drift was significantly reduced independently of the patient group and caused perfect gaze holding on the average. Since the observed improvements were independent of target visibility, 4-AP improved fixation by restoring gaze-holding ability. All in all, the present study demonstrates that 4-AP has a differential effect on DBN: drift with gaze straight ahead was predominantly reduced in patients with cerebellar atrophy, but less so in the remaining patients; 4-AP on the average improved neural integrator function, i.e. gaze-evoked drift, regardless of etiology. Our results thus show that 4-AP was a successful treatment option in the majority of DBN patients, possibly by increasing Purkinje cell excitability in the cerebellar flocculi. It may work best when DBN is associated with cerebellar atrophy. Furthermore, 4-AP may be a promising treatment option for patients with a dominant gaze-evoked component of nystagmus, regardless of its etiology.

Crowding in central vision in normally sighted and visually impaired [corrected] children aged 4 to 8 years: the influence of age and test design.

PubMed

Huurneman, Bianca; Boonstra, F Nienke; Cillessen, Antonius H N; van Rens, Ger; Cox, Ralf F A

2012-06-01

To investigate crowding ratios in children with a visual impairment due to ocular disease (n = 58) and normally sighted children (n = 75) aged 4 to 8 years using several variants of two clinically available tests with different optotype spacing (fixed or proportional to the optotype size). Crowding ratios, calculated by dividing the single acuity by the linear acuity, were measured binocularly with the C-test and the LH line chart. Ratios >1.00 indicate crowding. The charts with fixed spacing revealed significantly higher crowding ratios for visually impaired children than normally sighted children (both for measurements at 40 cm and 5 m). The age-related reduction of the crowding ratios seen in normally sighted children when tested with near-vision charts with fixed spacing was not present in the visually impaired group. Visually impaired children with nystagmus showed higher crowding ratios than visually impaired children without nystagmus. The chart with proportional intersymbol spacing (ISS) did not reveal differences between the normally sighted and visually impaired children; nor did it show group, age, or nystagmus effects. Visually impaired children showed higher crowding ratios than normally sighted children when measured with charts with fixed ISS. This study illustrates that test design and target/flanker interference as a manifestation of crowding are critical issues to bear in mind when assessing crowding ratios in children.

Characterization of slow and fast phase nystagmus

NASA Technical Reports Server (NTRS)

Lessard, Charles S.; Rodriguez-Garcia, Carlos A.; Wong, Wing Chan; Im, Jae J.; Schmidt, Glenn F.

1991-01-01

A current literature review of the analog and digital process of vestibular and optical kinetic nystagmus reveals little agreement in the methods used by various labs. The strategies for detection of saccade (fast phase velocity component of nystagmus) vary between labs, and most of the process have not been evaluated and validated with a standard database. A survey was made of major vestibular labs in the U.S. that perform computer analyses of vestibular and optokinetic reflexes to stimuli, and a baseline was established from which to standardize data acquisition and analysis programs. The concept of an Error Index was employed as the criterium for evaluating the performance of the vestibular analysis software programs. The performance criterium is based on the detection of saccades and is the average of the percentages of missed detections and false detections. Evaluation of the programs produced results for lateral gaze with saccadic amplitude of one, two, three, five, and ten degrees with various signal-to-noise ratios. In addition, results were obtained for sinusoidal pursuit of 0.05, 0.10, and 0.50 Hz with saccades from one to ten degrees at various signal-to-noise ratios. Selection of the best program was made from the performance in the lateral gaze with three degrees of saccadic amplitude and in the 0.10 Hz sinusoid with three degrees of saccadic amplitude.

A dynamic model of the eye nystagmus response to high magnetic fields.

PubMed

Glover, Paul M; Li, Yan; Antunes, Andre; Mian, Omar S; Day, Brian L

2014-02-07

It was recently shown that high magnetic fields evoke nystagmus in human subjects with functioning vestibular systems. The proposed mechanism involves interaction between ionic currents in the endolymph of the vestibular labyrinth and the static magnetic field. This results in a Lorentz force that causes endolymph flow to deflect the cupulae of the semi-circular canals to evoke a vestibular-ocular reflex (VOR). This should be analogous to stimulation by angular acceleration or caloric irrigation. We made measurements of nystagmus slow-phase velocities in healthy adults experiencing variable magnetic field profiles of up to 7 T while supine on a bed that could be moved smoothly into the bore of an MRI machine. The horizontal slow-phase velocity data were reliably modelled by a linear transfer function incorporating a low-pass term and a high-pass adaptation term. The adaptation time constant was estimated at 39.3 s from long exposure trials. When constrained to this value, the low-pass time constant was estimated at 13.6 ± 3.6 s (to 95% confidence) from both short and long exposure trials. This confidence interval overlaps with values obtained previously using angular acceleration and caloric stimulation. Hence it is compatible with endolymph flow causing a cupular deflection and therefore supports the hypothesis that the Lorentz force is a likely transduction mechanism of the magnetic field-evoked VOR.

A novel mutation in FRMD7 causing X-linked idiopathic congenital nystagmus in a large family

PubMed Central

He, Xiang; Gu, Feng; Wang, Yujing; Yan, Jinting; Zhang, Meng; Huang, Shangzhi

2008-01-01

Purpose To identify the gene responsible for causing an X-linked idiopathic congenital nystagmus (XLICN) in a six-generation Chinese family. Methods Forty-nine members of an XLICN family were recruited and examined after obtaining informed consent. Affected male individuals were genotyped with microsatellite markers around the FRMD7 locus. Mutations were comprehensively screened by direct sequencing using gene specific primers. An X-inactivation pattern was investigated by X chromosome methylation analysis. Results The patients showed phenotypes consistent with XLICN. Genotype analysis showed that male affected individuals in the family shared a common haplotype with the selected markers. Sequencing FRMD7 revealed a G>T transversion (c.812G>T) in exon 9, which caused a conservative substitution of Cys to Phe at codon 271 (p.C271F). This mutation co-segregated with all affected individuals and was present in the obligate, non-penetrant female carriers. However, the mutation was not observed in unaffected familial males or 400 control males. Females with the mutant gene could be affected or carrier and they shared the same inactivated X chromosome harboring the mutation in blood cells, which showed there is no clear causal link between X-inactivation pattern and phenotype. Conclusions We identified a novel mutation in FRMD7 and confirmed the role of this mutation in the pathogenesis of X-linked congenital nystagmus. PMID:18246032

Preoperative vestibular assessment protocol of cochlear implant surgery: an analytical descriptive study.

PubMed

Bittar, Roseli Saraiva Moreira; Sato, Eduardo Setsuo; Ribeiro, Douglas Jósimo Silva; Tsuji, Robinson Koji

Cochlear implants are undeniably an effective method for the recovery of hearing function in patients with hearing loss. To describe the preoperative vestibular assessment protocol in subjects who will be submitted to cochlear implants. Our institutional protocol provides the vestibular diagnosis through six simple tests: Romberg and Fukuda tests, assessment for spontaneous nystagmus, Head Impulse Test, evaluation for Head Shaking Nystagmus and caloric test. 21 patients were evaluated with a mean age of 42.75±14.38 years. Only 28% of the sample had all normal test results. The presence of asymmetric vestibular information was documented through the caloric test in 32% of the sample and spontaneous nystagmus was an important clue for the diagnosis. Bilateral vestibular areflexia was present in four subjects, unilateral arreflexia in three and bilateral hyporeflexia in two. The Head Impulse Test was a significant indicator for the diagnosis of areflexia in the tested ear (p=0.0001). The sensitized Romberg test using a foam pad was able to diagnose severe vestibular function impairment (p=0.003). The six clinical tests were able to identify the presence or absence of vestibular function and function asymmetry between the ears of the same individual. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Nystagmus

MedlinePlus

... Plastic Surgery Center Laser Surgery Education Center Redmond Ethics Center Global Ophthalmology Guide Academy Publications EyeNet Ophthalmology ... Plastic Surgery Center Laser Surgery Education Center Redmond Ethics Center Global Ophthalmology Guide Find an Ophthalmologist Advanced ...

Abnormal Eye Movements in Creutzfeldt-Jakob Disease

NASA Technical Reports Server (NTRS)

Grant, Michael P.; Cohen, Mark; Petersen, Robert B.; Halmagyi, G. Michael; McDougall, Alan; Tusa, Ronald J.; Leigh, R. John

1993-01-01

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 one 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.

DIZZINESS AND HEAD INJURY.

PubMed

BARBER, H O

1965-05-01

Dizziness, whether vague or specifically rotational, is a common sequel to head injury, and is often postural. One hundred and sixty-five patients with this symptom were examined. The simple posture tests employed to detect positional nystagmus are described. This physical finding was present in one-quarter of the entire group, and in nearly one-half of cases of longitudinal fracture of temporal bone. In such cases, it is an objective finding that corresponds precisely to the patient's complaint of vertigo.Transverse fracture of temporal bone destroys the inner ear in both cochlear and vestibular parts. Longitudinal fracture is commoner and causes bleeding from the ear; inner-ear damage is usually minor.In the rare cases where persisting postural vertigo and positional nystagmus are disabling, relief of the symptom may be achieved by vestibular denervation of the affected side.

Spasmus nutans

MedlinePlus

... infants and young children. It involves rapid, uncontrolled eye movements, head bobbing, and, sometimes, holding the neck in ... spasmus nutans include: Small, quick, side-to-side eye movements called nystagmus (both eyes are involved, but each ...

Wernicke-Korsakoff syndrome

MedlinePlus

... cause leg tremor Vision changes such as abnormal eye movements (back and forth movements called nystagmus), double vision , ... may show damage to many nerve systems: Abnormal eye movement Decreased or abnormal reflexes Fast pulse (heart rate) ...

Aviation medicine translations : annotated bibliography of recently translated material, III.

DOT National Transportation Integrated Search

1965-04-01

An annotated bibliography of translations of foreignlanguage research articles is presented. The 26 listed entries are concerned with studies of aviation medicine, periodicity, optokinetic nystagmus, vision, vestibular function, and physical science....

Astronaut John Glenn tests balance mechanism performance

NASA Image and Video Library

1962-02-01

S64-14849 (1962) --- Astronaut John H. Glenn Jr.'s balance mechanism (semi-circular-canals) is tested by running cool water into his ear and measuring effect on eye motions (nystagmus). Photo credit: NASA

Low Vision Training in Sweden.

ERIC Educational Resources Information Center

Inde, Krister

1978-01-01

The article describes the team work approach used in Sweden to provide services to the four main categories of visual impairment: central scotoma, nystagmus, loss of peripheral vision while retaining central vision, and amblyopia. (Author/PHR)

Dizziness and Head Injury

PubMed Central

Barber, H. O.

1965-01-01

Dizziness, whether vague or specifically rotational, is a common sequel to head injury, and is often postural. One hundred and sixty-five patients with this symptom were examined. The simple posture tests employed to detect positional nystagmus are described. This physical finding was present in one-quarter of the entire group, and in nearly one-half of cases of longitudinal fracture of temporal bone. In such cases, it is an objective finding that corresponds precisely to the patient's complaint of vertigo. Transverse fracture of temporal bone destroys the inner ear in both cochlear and vestibular parts. Longitudinal fracture is commoner and causes bleeding from the ear; inner-ear damage is usually minor. In the rare cases where persisting postural vertigo and positional nystagmus are disabling, relief of the symptom may be achieved by vestibular denervation of the affected side. PMID:14285289

Convection, buoyancy or endolymph expansion: what is the actual mechanism responsible for the caloric response of semicircular canals?

PubMed

Valli, Paulo; Buizza, Angelo; Botta, Laura; Zucca, Giampiero; Ghezzi, Luciano; Valli, Stefano

The mechanisms underlying caloric nystagmus are still matter of debate. The original theory proposed by Barany and more recently by Pau and Limberg suggested that convective endolymphatic currents were involved. In contrast Gentine et al. suggested that the main mechanism responsible for caloric nystagmus is buoyancy due to calorization of the endolymph, without the need of continuous convective currents. Finally, other authors (Scherer and Clarke, Arai et al.) proposed that thermal expansion or contraction of the endolymph were involved. In the present study experimental conditions have been considered able to discriminate between these different models. The experiments, were carried out on isolated labyrinth preparations of the frog. Only the predictions of the model based on buoyancy were fully consistent with the experimental results whereas those provided by the other models were not.

Downbeat nystagmus due to ranitidine in a pediatric patient.

PubMed

Butragueño Laiseca, Laura; Toledo Del Castillo, Blanca; Rodríguez Jimenez, Cristina; Manrique-Rodríguez, Silvia; Pérez Moreno, Jimena

2017-07-01

Ranitidine has not been considered as a potential cause of ocular movement conditions. However, it is known that the vestibular nucleus complex, that has a key role in gaze control and vestibule-ocular reflexes, receives hypothalamic histaminergic innervations. Some studies reported the effect of ranitidine blocking the excitatory responses of vestibular nuclei neurons to histamine. We report the first case of a downbeat nystagmus secondary to ranitidine in an infant. A 3-month-old female developed a downbeat gaze after starting treatment with ranitidine for a pediatric gastroesophageal reflux disease. Microbiological test were negative and neuroblastoma evaluation was normal. As ranitidine is widely prescribed in the pediatric population, clinicians should be aware of its potential to cause ocular movements disorders. Copyright © 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Preventable visual impairment in children 
with nonprofound intellectual disability.

PubMed

Aslan, Lokman; Aslankurt, Murat; Aksoy, Adnan; Altun, Hatice

2013-01-01

To assess the preventable visual impairment in children with nonprofound intellectual disability (ID). 
 A total of 215 children with IDs (90 Down syndrome [DS], 125 nonprofound ID) and 116 age- and sex-matched healthy subjects were enrolled in this study. All participants underwent ophthalmologic examinations including cycloplegic refraction measurements, ocular movement evaluation, screening for strabismus (Hirschberg, Krimsky, or prism cover test), slit-lamp biomicroscopy, funduscopy, and intraocular pressure measurements. All data were recorded for statistical analysis.
 Ocular findings in decreasing prevalence were as follows: refractive errors 55 (61.1%), strabismus 30 (33.2%), cataract 7 (7.8%), and nystagmus 7 (7.8%) in children with DS; refractive errors 57 (45.6%), strabismus 19 (15.2%), cataract 7 (6.4%), nystagmus 5 (4%), and glaucoma 1 (0.8%) in children with other ID; and refractive errors 13 (11.2%) and strabismus 4 (3.5%) in controls. Cataracts, glaucoma, and nystagmus were not observed in the control group. The most common ophthalmic findings in children with DS compared with other ID and controls were with hyperopia (p<0.03 and 
p<0.001, respectively) and esotropia (p<0.01 and p<0.01, respectively).
 The pediatric population with ID has a high prevalence of preventable visual impairments, refractive errors, strabismus, and cataracts. The prevalence of strabismus and refractive errors was more frequent in children with DS. The importance of further health screenings including ophthalmic examinations should be utilized to implement appropriate care management and improve quality of life.

The neural mechanism for Latent (fusion maldevelopment) nystagmus.

PubMed

Tychsen, Lawrence; Richards, Michael; Wong, Agnes; Foeller, Paul; Bradley, Dolores; Burkhalter, Andreas

2010-09-01

Latent nystagmus (LN) is the by-product of fusion maldevelopment in infancy. Because fusion maldevelopment--in the form of strabismus and amblyopia--is common, LN is a prevalent form of pathologic nystagmus encountered in clinical practice. It originates as an afferent visual pathway disorder. To unravel the mechanism for LN, we studied patients and nonhuman primates with maldeveloped fusion. These experiments have revealed that loss of binocular connections within striate cortex (area V1) in the first months of life is the necessary and sufficient cause of LN. The severity of LN increases systematically with longer durations of binocular decorrelation and greater losses of V1 connections. Decorrelation durations that exceed the equivalent of 2-3 months in human development result in an LN prevalence of 100%. No manipulation of brain stem motor pathways is required. The binocular maldevelopment originating in area V1 is passed on to downstream extrastriate regions of cerebral cortex that drive conjugate gaze, notably MSTd. Conjugate gaze is stable when MSTd neurons of the right and left cerebral hemispheres have balanced binocular activity. Fusion maldevelopment in infancy causes unbalanced monocular activity. If input from one eye dominates and the other is suppressed, MSTd in one hemisphere becomes more active. Acting through downstream projections to the ipsilateral nucleus of the optic tract, the eyes are driven conjugately to that side. The unbalanced MSTd drive is evident as the nasalward gaze-holding bias of LN when viewing with either eye.

Refractive errors and ocular findings in children with intellectual disability: A controlled study

PubMed Central

Akinci, Arsen; Oner, Ozgur; Bozkurt, Ozlem Hekim; Guven, Alev; Degerliyurt, Aydan; Munir, Kerim

2015-01-01

PURPOSE To evaluate the ocular findings and refractive errors in children with intellectual disability and in controls of average intellectual development of similar socioeconomic backgrounds. METHODS The study was conducted at Diskapi Children’s Hospital in Ankara, Turkey: 724 subjects with intellectual disability and 151 control subjects were evaluated. The subjects with intellectual disability were subdivided into mild (IQ 50–69, n = 490), moderate (IQ 35–49, n = 164), and severe (IQ <34, n = 70) groups, and syndromic (n = 138) versus nonsyndromic (n = 586) disability. All children underwent cycloplegic autorefraction or retinoscopy, slit-lamp biomicroscopy, and dilated fundus examination. Ocular alignment was assessed by Hirschberg, Krimsky, or prism cover test. The main outcome measure was the prevalence of refractive errors and ocular findings. RESULTS Seventy-seven percent of subjects with intellectual disability, and 42.4% of controls, had ocular findings. The children with intellectual disability had significantly more nystagmus, strabismus, astigmatism, and hypermetropia than controls. Children with syndromic intellectual disability had significantly more nystagmus, strabismus, astigmatism, and hypermetropia than subjects with nonsyndromic intellectual disability. Increasing severity of intellectual disability was related to higher prevalence of nystagmus, strabismus, astigmatism, hypermetropia, and anisometropia. CONCLUSIONS From a public health perspective, evaluation and treatment of ocular and refractive findings in children with moderate, severe, and syndromic intellectual disability categories is urgently needed and likely to be highly effective in alleviating future health and social care costs, as well as improving the productive lives of individuals with intellectual disability. PMID:18595752

Aviation medicine translations : annotated bibliography of recently translated material, VI.

DOT National Transportation Integrated Search

1971-01-01

An annotated bibliography of translations of foreign-language articles is presented. The 22 entries are concerned with studies in aviation medicine, vestibular function, body temperature, color vision, cholinesterase, nystagmus, alcohol, vestibulo-oc...

Nystagmus

MedlinePlus

... ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 919. Review Date 2/23/2017 Updated by: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony ... NY. Review provided by VeriMed Healthcare Network. Also reviewed by ...

Genetics Home Reference: CASK-related intellectual disability

MedlinePlus

... XL-ID with or without nystagmus (rapid, involuntary eye movements) is a milder form of CASK -related intellectual ... to promote development of the nerves that control eye movement (the oculomotor neural network). Mutations in the CASK ...

Aviation medicine translations : annotated bibliography of recently translated material, IV.

DOT National Transportation Integrated Search

1966-01-01

An annotated bibliography of translations of foreign-language articles is presented. The 25 listed entries are concerned with studies in aviation medicine, vertigo and nystagmus, physical science, hypoxia, vision, altitude, drugs, binaural hearing, t...

Aviation medicine translations : annotated bibliography of recently translated material, VII.

DOT National Transportation Integrated Search

1972-05-01

An annotated bibliography of translations of foreign-language articles is presented. The 25 listed entries are concerned with studies in aviation medicine, vertigo and nystagmus, physical science, hypoxia, vision, altitude, drugs, binaural hearing, t...

Genetics Home Reference: X-linked infantile nystagmus

MedlinePlus

... Free article on PubMed Central Thomas MG, Thomas S, Kumar A, Proudlock FA, Gottlob I. FRMD7-Related Infantile ... Roberts EO, Awan M, McLean R, Surendran M, Kumar AS, Farooq SJ, Degg C, Gale RP, Reinecke ...

Congenital Nystagmus Gene FRMD7 Is Necessary for Establishing a Neuronal Circuit Asymmetry for Direction Selectivity

PubMed Central

Yonehara, Keisuke; Fiscella, Michele; Drinnenberg, Antonia; Esposti, Federico; Trenholm, Stuart; Krol, Jacek; Franke, Felix; Scherf, Brigitte Gross; Kusnyerik, Akos; Müller, Jan; Szabo, Arnold; Jüttner, Josephine; Cordoba, Francisco; Reddy, Ashrithpal Police; Németh, János; Nagy, Zoltán Zsolt; Munier, Francis; Hierlemann, Andreas; Roska, Botond

2016-01-01

Summary Neuronal circuit asymmetries are important components of brain circuits, but the molecular pathways leading to their establishment remain unknown. Here we found that the mutation of FRMD7, a gene that is defective in human congenital nystagmus, leads to the selective loss of the horizontal optokinetic reflex in mice, as it does in humans. This is accompanied by the selective loss of horizontal direction selectivity in retinal ganglion cells and the transition from asymmetric to symmetric inhibitory input to horizontal direction-selective ganglion cells. In wild-type retinas, we found FRMD7 specifically expressed in starburst amacrine cells, the interneuron type that provides asymmetric inhibition to direction-selective retinal ganglion cells. This work identifies FRMD7 as a key regulator in establishing a neuronal circuit asymmetry, and it suggests the involvement of a specific inhibitory neuron type in the pathophysiology of a neurological disease. Video Abstract PMID:26711119

Signs of neurobehavioral dysfunction in a sample of learning disabled children: stability and concurrent validity.

PubMed

Morrison, D C; Hinshaw, S P; Carte, E T

1985-12-01

Of 270 learning disabled children with average intelligence and significant delays in reading comprehension a sample of 37 were evaluated for signs of neurobehavioral dysfunction. All such signs--primitive reflexes, equilibrium reactions, and postrotary nystagmus--were reliably assessed. A subsample of 19 children was compared with developmentally normal and mentally retarded samples for the occurrence of tonic neck reflexes and equilibrium reactions. The learning disabled children consistently showed deviancies like those of the retarded children; both of these groups differed from the normal children on most measures. These deviant responses persisted over a 9-mo. period for the learning disabled group. Compared with norms, the total learning disabled sample displayed hyponystagmus, and this depressed nystagmus persisted for 11 mo. Results are discussed in relation to the lack of correlation among the various signs of neurobehavioral dysfunction in the learning disabled children.

A locus for the nystagmus-associated form of episodic ataxia maps to an 11-cM region on chromosome 19p

DOE Office of Scientific and Technical Information (OSTI.GOV)

Kramer, P.L.; Gancher, S.T.; Nutt, J.G.

1995-07-01

Episodic ataxia (EA) is a rare neurological disorder characterized by attacks of generalized ataxia and near-normal neurological function between attacks. Most inherited cases are the result of an autosomal dominant condition with unknown neuropathology. It is heterogeneous and includes at least two distinct forms. In EA-1, attacks last minutes and interictal myokymia may be present. In EA-2, attacks may last hours and interictal nystagmus may occur. We reported linkage in four EA-1 families to chromosome 12p13 and identified mutations in these families in a potassium channel gene, KCNA1. Recently, we reported linkage in two EA-2 families to a 30-cM regionmore » on chromosome 19p. This report is based on members of the same two families and one additional kindred. 18 refs., 1 fig., 1 tab.« less

Variations in gravitoinertial force level affect the gain of the vestibulo-ocular reflex - Implications of the etiology of space motion sickness

NASA Technical Reports Server (NTRS)

Lackner, J. R.; Graybiel, A.

1981-01-01

Recordings of horizontal nystagmus were obtained on 16 male subjects exposed to repeated patterns of horizontal angular acceleration, constant velocity rotation, and sudden-stop deceleration in the laboratory and in the free-fall and high-force periods of parabolic flight. Nystagmus intensity was a clear function of gravitoinertial force level: slow phase velocity and beat frequency increased during exposure to high force levels and decreased in free-fall compared to values obtained at 1 G. These findings indicate that the gain of the vestibulo-ocular reflex decreases in free-fall. This fact likely accounts for the disorientation and dizziness sometimes experienced by astronauts when moving their heads in the early phases of orbital flight and again after splashdown. The implications of the present findings, both for the etiology and for the treatment of space motion sickness, are discussed.

Hyperventilation-induced nystagmus in vestibular schwannoma and unilateral sensorineural hearing loss.

PubMed

Mandalà, Marco; Giannuzzi, Annalisa; Astore, Serena; Trabalzini, Franco; Nuti, Daniele

2013-07-01

We evaluated the incidence and characteristics of hyperventilation-induced nystagmus (HVN) in 49 patients with gadolinium-enhanced magnetic resonance imaging evidence of vestibular schwannoma and 53 patients with idiopathic unilateral sensorineural hearing loss and normal radiological findings. The sensitivity and specificity of the hyperventilation test were compared with other audio-vestibular diagnostic tests (bedside examination of eye movements, caloric test, auditory brainstem responses) in the two groups of patients. The hyperventilation test scored the highest diagnostic efficiency (sensitivity 65.3 %; specificity 98.1 %) of the four tests in the differential diagnosis of vestibular schwannoma and idiopathic unilateral sensorineural hearing loss. Small tumors with a normal caloric response or caloric paresis were associated with ipsilateral HVN and larger tumors and severe caloric deficits with contralateral HVN. These results confirm that the hyperventilation test is a useful diagnostic test for predicting vestibular schwannoma in patients with unilateral sensorineural hearing loss.

Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough.

PubMed

Oh, Angela Jinsook; Lanzman, Bryan Alexander; Liao, Yaping Joyce

2018-06-01

Midline structural defects in the neural axis can give rise to neuro-ophthalmic symptoms. We report a rare case of keyhole aqueduct syndrome presenting after two years of severe cough due to gastroesophageal reflux disease. A 58-year-old woman with a 2-year history of daily, severe cough presented to the neuro-ophthalmology clinic with progressive diplopia and oscillopsia. Examination revealed a 1-2 Hz down-beating nystagmus in primary gaze that worsened with left, right, and down gazes. Gaze evoked nystagmus and mild paresis were also seen with up gaze. There was an incomitant left hypertropia due to skew deviation that worsened with right and up gazes and improved with down gaze. She also had a right-sided ptosis and a 3 mm anisocoria not due to cranial nerve 3 paresis or Horner's syndrome. Brain magnetic resonance imaging showed a 1.5 mm × 11.7 mm × 6 mm midline cleft in the ventral midbrain communicating with the cerebral aqueduct, consistent with keyhole aqueduct syndrome. Her nystagmus and diplopia improved with oral acetazolamide treatment, at high doses of 2500-3000 mg per day. We report the first case of midbrain keyhole aqueduct syndrome with ocular motor and other neuro-ophthalmic manifestations associated with severe cough. Although her cough was effectively treated and intracranial pressure measurement was normal, her ophthalmic symptoms continued to progress, which is common in previous cases reported. Treatment with acetazolamide led to significant improvement, supporting the use of acetazolamide in this rare condition.

Optokinetic and vestibular stimulation determines the spatial orientation of negative optokinetic afternystagmus in the rabbit.

PubMed

Pettorossi, V E; Errico, P; Ferraresi, A; Barmack, N H

1999-02-15

Prolonged binocular optokinetic stimulation (OKS) in the rabbit induces a high-velocity negative optokinetic afternystagmus (OKAN II) that persists for several hours. We have taken advantage of this uniform nystagmus to study how changes in static head orientation in the pitch plane might influence the orientation of the nystagmus. After horizontal OKS, the rotation axis of the OKAN II remained almost constant in space as it was kept aligned with the gravity vector when the head was pitched by as much as 80 degrees up and 35 degrees down. Moreover, during reorientation, slow-phase eye velocity decreased according to the head pitch angle. Thereafter, we analyzed the space orientation of OKAN II after optokinetic stimulation during which the head and/or the OKS were pitched upward and downward. The rotation axis of OKAN II did not remain aligned with an earth vertical axis nor a head vertical axis, but it tended to be aligned with that of the OKS respace. The slow-phase eye velocity of OKAN II was also affected by the head pitch angle during OKS, because maximal OKAN II velocity occurred at the same head pitch angle as that during optokinetic stimulation. We suggest that OKAN II is coded in gravity-centered rather than in head-centered coordinates, but that this coordinate system may be influenced by optokinetic and vestibular stimulation. Moreover, the velocity attenuation of OKAN II seems to depend on the mismatch between the space-centered nystagmus rotation axis orientation and that of the "remembered" head-centered optokinetic pathway activated by OKS.

Molecular genetic analysis of patients with sporadic and X-linked infantile nystagmus

PubMed Central

Zhao, Hui; Huang, Xiu-Feng; Zheng, Zhi-Li; Deng, Wen-Li; Lei, Xin-Lan; Xing, Dong-Jun; Ye, Liang; Xu, Su-Zhong; Chen, Jie; Zhang, Fang; Yu, Xin-Ping; Jin, Zi-Bing

2016-01-01

Objectives Infantile nystagmus (IN) is a genetically heterogeneous condition characterised by involuntary rhythmic oscillations of the eyes accompanied by different degrees of vision impairment. Two genes have been identified as mainly causing IN: FRMD7 and GPR143. The aim of our study was to identify the genetic basis of both sporadic IN and X-linked IN. Design Prospective analysis. Patients Twenty Chinese patients, including 15 sporadic IN cases and 5 from X-linked IN families, were recruited and underwent molecular genetic analysis. We first performed PCR-based DNA sequencing of the entire coding region and the splice junctions of the FRMD7 and GPR143 genes in participants. Mutational analysis and co-segregation confirmation were then performed. Setting All clinical examinations and genetic experiments were performed in the Eye Hospital of Wenzhou Medical University. Results Two mutations in the FRMD7 gene, including one novel nonsense mutation (c.1090C>T, p.Q364X) and one reported missense mutation (c.781C>G, p.R261G), were identified in two of the five (40%) X-linked IN families. However, none of putative mutations were identified in FRMD7 or GPR143 in any of the sporadic cases. Conclusions The results suggest that mutations in FRMD7 appeared to be the major genetic cause of X-linked IN, but not of sporadic IN. Our findings provide further insights into FRMD7 mutations, which could be helpful for future genetic diagnosis and genetic counselling of Chinese patients with nystagmus. PMID:27036142

The Gait Disorder in Downbeat Nystagmus Syndrome

PubMed Central

Schniepp, Roman; Wuehr, Max; Huth, Sabrina; Pradhan, Cauchy; Schlick, Cornelia; Brandt, Thomas; Jahn, Klaus

2014-01-01

Background Downbeat nystagmus (DBN) is a common form of acquired fixation nystagmus with key symptoms of oscillopsia and gait disturbance. Gait disturbance could be a result of impaired visual feedback due to the involuntary ocular oscillations. Alternatively, a malfunction of cerebellar locomotor control might be involved, since DBN is considered a vestibulocerebellar disorder. Methods Investigation of walking in 50 DBN patients (age 72±11 years, 23 females) and 50 healthy controls (HS) (age 70±11 years, 23 females) using a pressure sensitive carpet (GAITRite). The patient cohort comprised subjects with only ocular motor signs (DBN) and subjects with an additional limb ataxia (DBNCA). Gait investigation comprised different walking speeds and walking with eyes closed. Results In DBN, gait velocity was reduced (p<0.001) with a reduced stride length (p<0.001), increased base of support (p<0.050), and increased double support (p<0.001). Walking with eyes closed led to significant gait changes in both HS and DBN. These changes were more pronounced in DBN patients (p<0.001). Speed-dependency of gait variability revealed significant differences between the subgroups of DBN and DBNCA (p<0.050). Conclusions (I) Impaired visual control caused by involuntary ocular oscillations cannot sufficiently explain the gait disorder. (II) The gait of patients with DBN is impaired in a speed dependent manner. (III) Analysis of gait variability allows distinguishing DBN from DBNCA: Patients with pure DBN show a speed dependency of gait variability similar to that of patients with afferent vestibular deficits. In DBNCA, gait variability resembles the pattern found in cerebellar ataxia. PMID:25140517

On the predictive control of foveal eye tracking and slow phases of optokinetic and vestibular nystagmus.

PubMed Central

Yasui, S; Young, L R

1984-01-01

Smooth pursuit and saccadic components of foveal visual tracking as well as more involuntary ocular movements of optokinetic (o.k.n.) and vestibular nystagmus slow phase components were investigated in man, with particular attention given to their possible input-adaptive or predictive behaviour. Each component in question was isolated from the eye movement records through a computer-aided procedure. The frequency response method was used with sinusoidal (predictable) and pseudo-random (unpredictable) stimuli. When the target motion was pseudo-random, the frequency response of pursuit eye movements revealed a large phase lead (up to about 90 degrees) at low stimulus frequencies. It is possible to interpret this result as a predictive effect, even though the stimulation was pseudo-random and thus 'unpredictable'. The pseudo-random-input frequency response intrinsic to the saccadic system was estimated in an indirect way from the pursuit and composite (pursuit + saccade) frequency response data. The result was fitted well by a servo-mechanism model, which has a simple anticipatory mechanism to compensate for the inherent neuromuscular saccadic delay by utilizing the retinal slip velocity signal. The o.k.n. slow phase also exhibited a predictive effect with sinusoidal inputs; however, pseudo-random stimuli did not produce such phase lead as found in the pursuit case. The vestibular nystagmus slow phase showed no noticeable sign of prediction in the frequency range examined (0 approximately 0.7 Hz), in contrast to the results of the visually driven eye movements (i.e. saccade, pursuit and o.k.n. slow phase) at comparable stimulus frequencies. PMID:6707954

The gait disorder in downbeat nystagmus syndrome.

PubMed

Schniepp, Roman; Wuehr, Max; Huth, Sabrina; Pradhan, Cauchy; Schlick, Cornelia; Brandt, Thomas; Jahn, Klaus

2014-01-01

Downbeat nystagmus (DBN) is a common form of acquired fixation nystagmus with key symptoms of oscillopsia and gait disturbance. Gait disturbance could be a result of impaired visual feedback due to the involuntary ocular oscillations. Alternatively, a malfunction of cerebellar locomotor control might be involved, since DBN is considered a vestibulocerebellar disorder. Investigation of walking in 50 DBN patients (age 72 ± 11 years, 23 females) and 50 healthy controls (HS) (age 70 ± 11 years, 23 females) using a pressure sensitive carpet (GAITRite). The patient cohort comprised subjects with only ocular motor signs (DBN) and subjects with an additional limb ataxia (DBNCA). Gait investigation comprised different walking speeds and walking with eyes closed. In DBN, gait velocity was reduced (p<0.001) with a reduced stride length (p<0.001), increased base of support (p<0.050), and increased double support (p<0.001). Walking with eyes closed led to significant gait changes in both HS and DBN. These changes were more pronounced in DBN patients (p<0.001). Speed-dependency of gait variability revealed significant differences between the subgroups of DBN and DBNCA (p<0.050). (I) Impaired visual control caused by involuntary ocular oscillations cannot sufficiently explain the gait disorder. (II) The gait of patients with DBN is impaired in a speed dependent manner. (III) Analysis of gait variability allows distinguishing DBN from DBNCA: Patients with pure DBN show a speed dependency of gait variability similar to that of patients with afferent vestibular deficits. In DBNCA, gait variability resembles the pattern found in cerebellar ataxia.

Manipulation of arousal and its effects on human vestibular nystagmus induced by caloric irrigation and angular accelerations.

DOT National Transportation Integrated Search

1962-10-01

Alterations in the nystagmic reaction of human subjects exposed to vestibular stimulation have been frequently noted in studies of drug effects, habituation, and general features of the labyrinthine response.

Development and field test of psychophysical tests for DWI arrest

DOT National Transportation Integrated Search

1981-03-01

Administration and scoring procedures were standardized for a sobriety test battery consisting of the walk-and-turn test, the one leg stand test, and horizontal gaze nystagmus. The effectiveness of the standardized battery was then evaluated in the l...

Adaptation to vestibular disorientation. IX, Influence of head position on the habituation of vertical nystagmus.

DOT National Transportation Integrated Search

1968-03-01

Interactions of linear and angular accelerations are frequently experienced by pilots during aircraft maneuvers. Several recent studies have indicated that the otoliths (detectors of linear acceleration) may influence responses of the semicircular ca...

Adaptation to vestibular disorientation. II, Nystagmus and vertigo following high-velocity angular accelerations.

DOT National Transportation Integrated Search

1965-09-01

Professional figure skaters who, as part of their daily routine, subject themselves to high levels of disorientation-and vertigo-producing stimuli, were given a series of laboratory tests consisting primarily of caloric irrigations and mild angular a...

Adaptation to vestibular disorientation. I, Vertigo and nystagmus following repeated clinical stimulation.

DOT National Transportation Integrated Search

1965-05-01

Forty unilateral caloric irrigations were administered in a habituation series to each of two groups of subjects. One group was tested in total darkness. Subjects in the second group were stimulated in illumination and actively attempted to control a...

[Clinical characteristics and surgical treatment of idiopathic congenital nystagmus in 224 patients].

PubMed

Ding, J; Zhao, K X; Li, Y P; Ma, H Z; Chen, X; Guo, X; Zhu, L N; Li, N D; Zhang, W

2016-08-01

To study clinical characteristics and surgical treatment of idiopathic congenital nystagmus (ICN). A retrospective study was conducted in 224 patients with ICN in Tianjin eye hospital from July 2007 to February 2013. There were 224 patients, 158 (70.54%) males and 66 (29.5%) females, mean age was (11.6±8.4) years and (11.4±6.4) years separately. Horizontal nystgamus happened in 215 cases, 3 cases were vertical type and 6 cases were mixed. 214 cases were with no history of operation and 10 patients had ever underwent surgeries before. Furthermore, 151 patients combined with strabismus and refractive error, anterior segment or retinal disorders, which accounting for 67.4% of all the patients. 48 patients were associated myopia, 30 patients with hyperopia, 43 patients with strabismus. Among them, 153 cases of compensatory head position direction were horizontal with face turn, 43 cases (43/153, 28.1%) showed face turning to the left, 110 cases (110/153, 71.9%) showed face turning to the right. Surgeries were designed according to the compensatory head position and head retroversion angle. For 15 patients with double intermediate zones, the position which was often used with good visual function was chosen for operation design. As for the patients with nystagmus and strabismus, the transfer null zone to primary position for the dominant eye and strabismus surgery for the other eye was chosen. And for complicated patients with compensative head position, the dominant head posture were designed for surgery. ICN is dominated by male with variable clinical manifestations. Surgical choice for ICN depends on the direction of head position and if there is strabismus accompanying it.The aim of ocular muscle surgery is to transfer null zone to primary position. (Chin J Ophthalmol, 2016, 52: 574-578).

Clinical characteristics of children with severe visual impairment but favorable retinal structural outcomes from the Early Treatment for Retinopathy of Prematurity (ETROP) study.

PubMed

Siatkowski, R Michael; Good, William V; Summers, C Gail; Quinn, Graham E; Tung, Betty

2013-04-01

To describe visual function and associated characteristics at the 6-year examination in children enrolled in the Early Treatment for Retinopathy of Prematurity Study who had unfavorable visual outcomes despite favorable structural outcomes in one or both eyes. The clinical examination records of children completing the 6-year follow-up examination were retrospectively reviewed. Eligible subjects were those with visual acuity of ≤20/200 in each eye (where recordable) and a normal fundus or straightening of the temporal retinal vessels with or without macular ectopia in at least one eye. Data regarding visual function, retinal structure, presence of nystagmus, optic atrophy, optic disk cupping, seizures/shunts, and Functional Independence Measure for Children (ie, WeeFIM: pediatric functional independence measure) developmental test scores were reviewed. Of 342 participants who completed the 6-year examination, 39 (11%) met inclusion criteria. Of these, 29 (74%) had normal retinal structure, 18 (46%) had optic atrophy, and 3 (8%) had increased cupping of the optic disk in at least one eye. Latent and/or manifest nystagmus occurred in 30 children (77%). The presence of nystagmus was not related to the presence of optic atrophy. Of the 39 children, 28 (72%) had a below-normal WeeFIM score. In 25 participants (7%) completing the 6-year examination, cortical visual impairment was considered the primary cause of visual loss. The remainder likely had components of both anterior and posterior visual pathway disease. Clinical synthesis of ocular anatomy and visual and neurologic function is required to determine the etiology of poor vision in these children. Copyright © 2013 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.

Failure of Fixation Suppression of Spontaneous Nystagmus in Cerebellar Infarction: Frequency, Pattern, and a Possible Structure.

PubMed

Kim, Hyun-Ah; Yi, Hyon-Ah; Lee, Hyung

2016-04-01

To investigate the frequency and pattern of failure of the fixation suppression (FFS) of spontaneous nystagmus (SN) in unilateral cerebellar infarction, and to identify the structure responsible for FFS, 29 patients with acute, mainly unilateral, isolated cerebellar infarction who had SN with a predominantly horizontal component were enrolled in this study. The ocular fixation index (OFI) was defined as the mean slow phase velocity (SPV) of the horizontal component of SN with fixation divided by the mean SPV of the horizontal component of SN without fixation. The OFI from age- and sex-matched patients with vestibular neuritis was calculated and used as the control data. The FFS of SN was only found in less than half (41 %, 12/29) of the patients. Approximately 65 % (n = 7) of the patients with isolated anterior inferior cerebellar artery territory cerebellar infarction showed FFS, whereas only a quarter (n = 3) of the patients with isolated posterior inferior cerebellar artery (PICA) territory cerebellar infarction showed FFS. The proportion of gaze-evoked nystagmus (6/12 [50 %] vs. 2/17 [12 %], p = 0.04) and deficient gain of ipsilesional pursuit (10/12 [83 %] vs. 6/17 [35 %], p = 0.05) was more frequent in the FFS group than in the group without FFS. Lesion subtraction analysis in isolated PICA territory cerebellar infarction revealed that the nodulus was commonly damaged in patients with FFS, compared to that of patients without FFS. Our study shows that FFS of SN due to acute cerebellar infarction is less common than previously thought and the nodulus may be an important structure for the suppression of SN in humans.

The Vestibular Effects of Repeated Low-Level Blasts.

PubMed

Littlefield, Philip D; Pinto, Robin L; Burrows, Holly L; Brungart, Douglas S

2016-01-01

The objective of this study was to use a prospective cohort of United States Marine Corps (USMC) instructors to identify any acute or long-term vestibular dysfunction following repeated blast exposures during explosive breaching training. They were assessed in clinic and on location during training at the USMC Methods of Entry School, Quantico, VA. Subjects received comprehensive baseline vestibular assessments and these were repeated in order to identify longitudinal changes. They also received shorter assessments immediately following blast exposure in order to identify acute findings. The main outcome measures were the Neurobehavioral Symptom Inventory, vestibular Visual Analog Scale (VAS) of subjective vestibular function, videonystagmography (VNG), vestibular evoked myogenic potentials (VEMP), rotary chair (including the unilateral centrifugation test), computerized dynamic posturography, and computerized dynamic visual acuity. A total of 11 breachers and 4 engineers were followed for up to 17 months. No acute effects or longitudinal deteriorations were identified, but there were some interesting baseline group differences. Upbeat positional nystagmus was common, and correlated (p<0.005) with a history of mild traumatic brain injury (mTBI). Several instructors had abnormally short low-frequency phase leads on rotary chair testing. This study evaluated breaching instructors over a longer test period than any other study, and the results suggest that this population appears to be safe from a vestibular standpoint at the current exposure levels. Upbeat positional nystagmus correlated with a history of mTBI in this population, and this has not been described elsewhere. The data trends also suggest that this nystagmus could be an acute blast effect. However, the reasons for the abnormally short phase leads seen in rotary chair testing are unclear at this time. Further investigation seems warranted.

[A tracking function of human eye in microgravity and during readaptation to earth's gravity].

PubMed

Kornilova, L N

2001-01-01

The paper summarizes results of electro-oculography of all ways of visual tracking: fixative eye movements (saccades), smooth pursuit of linearly, pendulum-like and circularly moving point stimuli, pursuit of vertically moving foveoretinal optokinetic stimuli, and presents values of thresholds and amplification coefficients of the optokinetic nystagmus during tracking of linear movement of foveoretinal optokinetic stimuli. Investigations were performed aboard the Salyut and Mir space stations with participation of 31 cosmonauts of whom 27 made long-term (76 up to 438 day) and 4 made short-term (7 to 9 day) missions. It was shown that in space flight the saccadic structure within the tracking reaction does not change; yet, corrective movements (additional microsaccades to achieve tracking) appeared in 47% of observations at the onset and in 76% of observations on months 3 to 6 of space flight. After landing, the structure of vertical saccades was found altered in half the cosmonauts. No matter in or after flight, reverse nystagmus was present along with the gaze nystagmus during static saccades in 22% (7 cosmonauts) of the observations. Amplitude of tracking vertically, diagonally or circularly moving stimuli was significantly reduced as period on mission increased. Early in flight (40% of the cosmonauts) and shortly afterwards (21% of the cosmonauts) the structure of smooth tracking reaction was totally broken up, that is eye followed stimulus with micro- or macrosaccades. The structure of smooth eye tracking recovered on flight days 6-8 and on postflight days 3-4. However, in 46% of the cosmonauts on long-term missions the structure of smooth eye tracking was noted to be disturbed periodically, i.e. smooth tracking was replaced by saccadic.

[The significance of directional preponderance in the evaluation of vestibular function in patients with vertigo].

PubMed

Wang, J; Zhou, Y J; Yu, J; Gu, J

2017-03-07

Objective: To analyze the relationship between directional preponderance (DP), spontaneous nystagmus(SN) and vestibular disorders, and to investigate the significance of DP in directing peripheral vestibular function in patients with vertigo. Methods: This was a retrospective analysis of 394 cases diagnosed with peripheral vestibular disease accompanied by vertigo from March 2012 to June 2014 in the Outpatient Department of the Eye & ENT Hospital of Fudan University. Results of static and dynamic posture equilibrium tests, SN, unilateral weakness(UW), and DP in videonystagmography(VNG) were analyzed and compared. Results: The mean interval time between the last vertigo attack and examination in patients with SN or DP in caloric test were 4.4 d and 7.3 d respectively, and those without SN or DP were 18.3 d and 17.5 d respectively. The patients were divided into two groups according to DP results of caloric test. DP-normal group had 203 cases and DP-abnormal group had 191 cases. Spontaneous nystagmus was presented in 44 cases in the DP-normal group (21.67%) and four in the DP-abnormal group (2.09%). A significant difference was found between the two groups (χ 2 =35.27, P =0.000). Deficiency of vestibular function was noted in 165 cases in the DP-normal group (81.28%) and 123 (64.40%) in the DP-abnormal group in static and dynamic posture equilibrium tests. The difference between the two groups was statistically significant (χ 2 =14.26, P =0.000). Conclusion: Compared with DP-normal patients, DP-abnormal patients are more likely to have spontaneous nystagmus and balance disorders due to vestibular dysfunction.

Genetics Home Reference: UNC80 deficiency

MedlinePlus

... absent, although in some cases individuals have limited communication using body language, gestures, and signs. Seizures, involuntary side-to-side movements of the eyes (nystagmus), eyes that do not point in the same direction (strabismus), and a high-pitched cry can also occur in this disorder. ...

Alcohol and disorientation-related responses. I, Nystagmus and "vertigo" during caloric and optokinetic stimulation.

DOT National Transportation Integrated Search

1971-02-01

Although the sensory systems (vestibular and visual) most involved in disorientation and 'pilot's vertigo' would appear to be affected by the ingestion of alcohol, the locus and nature of the effect are not established. For example, there are apparen...

Alcohol and disorientation-related responses. IV, Effects of different alcohol dosages and display illumination tracking performance during vestibular stimulation.

DOT National Transportation Integrated Search

1971-07-01

A previous CAMI laboratory investigation showed that alcohol impairs the ability of men to suppress vestibular nystagmus while visually fixating on a cockpit instrument, thus degrading visual tracking performance (eye-hand coordination) during angula...

Astronaut Walter Schirra gets modified calonic test

NASA Image and Video Library

1962-01-01

S62-06157 (1962) --- Astronaut Walter Schirra Jr. gets modified calonic test. His balance mechanism (semicircular canals) are tested by running cool water into ear and measuring effect on eye motions (nystagmus) after his six-orbit flight in the Sigma 7 spacecraft. Photo credit: NASA

Adaptation to vestibular disorientation. X, Modification of vestibular nystagmus and "vertigo" by means of visual stimulation.

DOT National Transportation Integrated Search

1968-10-01

A conflict among sensory signals frequently underlies problems of disorientation, vertigo, and motion sickness. In this study, visual information in conflict with vestibular signals was presented to groups of subjects by illuminating the test room fo...

Task-control of arousal and the effects of repeated unidirectional angular acceleration on human vestibular responses.

DOT National Transportation Integrated Search

1963-11-01

Subjects were exposed to a 10-day habituation series of 200 CW accelerations in total darkness while performing attention-demanding tasks. Decelerations were sub-threshold. Preliminary and post-tests indicated that slow-phase nystagmus and duration o...

Identifcation of a Novel Mutation p.I240T in the FRMD7 gene in a Family with Congenital Nystagmus

NASA Astrophysics Data System (ADS)

Zhu, Yihua; Zhuang, Jianfu; Ge, Xianglian; Zhang, Xiao; Wang, Zheng; Sun, Ji; Yang, Juhua; Gu, Feng

2013-10-01

Congenital Nystagmus (CN) is a genetically heterogeneous ocular disease, which causes a significant proportion of childhood visual impairment. To identify the underlying genetic defect of a CN family, twenty-two members were recruited. Genotype analysis showed that affected individuals shared a common haplotype with markers flanking FRMD7 locus. Sequencing FRMD7 revealed a T > C transition in exon 8, causing a conservative substitution of Isoleucine to Tyrosine at codon 240. By protein structural modeling, we found the mutation may disrupt the hydrophobic core and destabilize the protein structure. We reviewed the literature and found that exons 2, 8, and 9 (11.4% of the sequence of FRMD7 mRNA) represent the majority (55.3%) of the reported FRMD7 mutations. In summary, we identified a novel mutation in FRMD7, showed its molecular consequence, and revealed the mutation-rich exons of the FRMD7 gene. Collectively, this provides molecular insights for future CN clinical genetic diagnosis and treatment.

Otolithic influences on extraocular and intraocular muscles

NASA Technical Reports Server (NTRS)

Gernandt, B. E.

1973-01-01

Selective stimulation of utricular gravireceptors leads to gross activation of the bulbar reticular formation where a strong interaction with evoked spino-bulbo-spinal reflex activity occurs. The utricular neurons encountered by microelectrodes in the lateral vestibular nuclei show four types of elicited activity; two of these display an increased firing rate, and two exhibit pronounced inhibitory effects. Application of a stimulus of long duration and constant intensity to the utricle has shown that rapid adaptation of the peripheral receptors is a prominent feature. The effects of selective utricular stimulation upon eye movements, as recorded by the corneoretinal potential method, have been studied in experiments on cats and monkeys and it can be firmly stated that prolonged stimulation of the utricle can evoke strong primary nystagmus, followed by a secondary nystagmus at the cessation of stimulation. The action of utricular stimulation on ocular reflexes has been examined further, with particular attention to evoked pupillary reactions in both cats and monkeys: constriction during the fast phase of the brisk conjugate eye movement, and dilatation during the flow phase.

The Horizontal Gaze Nystagmus test: fraudulent science in the American courts.

PubMed

Booker, J L

2004-01-01

The Horizontal Gaze Nystagmus (HGN) test was conceived, developed and promulgated as a simple procedure for the determination of the blood alcohol concentration of drivers suspected of driving while intoxicated (DWI). Bypassing the usual scientific review process and touted through the good offices of the federal agency responsible for traffic safety, it was rushed into use as a law enforcement procedure, and was soon adopted and protected from scientific criticism by courts throughout the United States. In fact, research findings, training manuals and other relevant documents were often held as secrets by the state. Still, the protective certification of its practitioners and the immunity afforded by judicial notice failed to silence all the critics of this deeply flawed procedure. Responding to criticism, the sponsors of the test traveled the path documented in this paper that led from mere (if that word can ever truly apply to a matter of such gravity) carelessness in research through self-serving puffery and finally into deliberate fraud--always at the expense of the citizen accused.

Homozygous variegate porphyria presenting with developmental and language delay in childhood.

PubMed

Pinder, V A E; Holden, S T; Deshpande, C; Siddiqui, A; Mellerio, J E; Wraige, E; Powell, A M

2013-10-01

Variegate porphyria is an autosomal dominant disorder that usually presents with photosensitivity and acute neurological crises in adulthood. It is caused by heterozygous mutations in the protoporphyrinogen oxidase gene (PPOX). A rarer variant, homozygous variegate porphyria (HVP), presents in childhood with recurrent skin blisters and scarring. More variable features of HVP are short stature, brachydactyly, nystagmus, epilepsy, developmental delay and mental retardation. We describe a child who presented with nystagmus, developmental delay and ataxia, combined with a photosensitive eruption. Analysis of porphyrins in plasma, urine and stool supported a clinical diagnosis of HVP. DNA from the patient showed that he is compound heterozygous for two novel missense mutations in the PPOX coding region: c.169G>C (p.Gly57Arg) and c.1259C>G (Pro420Arg). Interestingly, cranial magnetic resonance imaging showed an absence of myelin, a feature not previously reported in HVP, which expands the differential diagnosis of childhood hypomyelinating leucoencephalopathies. © 2013 British Association of Dermatologists.

Bariatric Surgery and the Neuro-Ophthalmologist

PubMed Central

Moss, Heather E.

2016-01-01

Background As the prevalence of obesity increases, so are the prevalences of weight related diseases and the incidence of surgical procedures to promote weight loss. It is important for neuro-ophthalmologists to be familiar with these procedures and possible downstream effects on afferent and efferent visual function. Evidence acquisition Review of ophthalmology, neurology, general surgery, obesity, endocrinology, nutrition, psychiatry and neurosurgery literature. Results Bariatric surgery is a safe and effective treatment for weight loss in obese individuals. There is level IV evidence that it is associated with improvement in idiopathic intracranial hypertension(IIH). Laboratory nutrient deficiencies are common following some types of bariatric procedures. Symptomatic deficiencies are less common but can be devastating. Thiamine deficiency can cause nystagmus and other symptoms in weeks to months following surgery, B12 or copper deficiency can cause optic neuropathy in the years to decades following bariatric surgery. Conclusions Bariatric surgery may be a treatment for IIH. Postoperative vitamin deficiencies may present with nystagmus, optic neuropathy, nyctalopia and/or ophthalmoparesis weeks to years after surgery. PMID:26764529

Bariatric Surgery and the Neuro-Ophthalmologist.

PubMed

Moss, Heather E

2016-03-01

As the prevalence of obesity increases, so, too, do the prevalences of weight-related diseases and surgical procedures to promote weight loss. It is important for neuro-ophthalmologists to be familiar with these procedures and possible downstream effects on afferent and efferent visual function. Review of ophthalmology, neurology, general surgery, obesity, endocrinology, nutrition, psychiatry, and neurosurgery literature. Bariatric surgery is a safe and effective treatment for weight loss in obese individuals. There is Level IV evidence that it is associated with improvement in idiopathic intracranial hypertension (IIH). Laboratory nutrient deficiencies are common following some types of bariatric procedures. Symptomatic deficiencies are less common but can be devastating. Thiamine deficiency can cause nystagmus and other symptoms in weeks to months after surgery, whereas B12 or copper deficiency can cause optic neuropathy in years to decades following bariatric surgery. Bariatric surgery is a potential treatment for IIH. Postoperative vitamin deficiencies may cause nystagmus, optic neuropathy, nyctalopia, and/or ophthalmoparesis weeks to years after surgery.

Proprioceptive influence on the optokinetic nystagmus.

PubMed

Botti, F; Anastasopoulos, D; Kostadima, V; Bambagioni, D; Pettorossi, V E

2001-01-01

The influence of neck and leg proprioceptive inputs on optokinetic-induced quick phases was studied in humans. Ten subjects received unidirectional horizontal optokinetic stimulation (10-20%/s) during sinusoidal neck, leg and combined neck + leg proprioceptive stimulation. The optokinetic reflex was measured by electro-oculography. Neck stimulation induced a shift in the nystagmus beating field in the opposite direction to body movement (gain 0.3 0.4, phase 140-180 degrees). The beating field shift resulted totally from the amplitude and frequency modulation of optokinetic quick phases, as slow phases were not affected. Leg proprioceptive stimulation induced a similar effect, but the phase of the response lagged by approximately 90 degrees compared with that of neck response. With combined neck + leg stimulation, the amplitude of the effect was a sum of the separate effects, but the phase coincided with that of the leg response. This suggests that neck and leg proprioceptive signals do not add linearly and that the leg signal determines the time of the response.

Sensorimotor aspects of high-speed artificial gravity: I. Sensory conflict in vestibular adaptation

NASA Technical Reports Server (NTRS)

Brown, Erika L.; Hecht, Heiko; Young, Laurence R.

2002-01-01

Short-radius centrifugation offers a promising and affordable countermeasure to the adverse effects of prolonged weightlessness. However, head movements made in a fast rotating environment elicit Coriolis effects, which seriously compromise sensory and motor processes. We found that participants can adapt to these Coriolis effects when exposed intermittently to high rotation rates and, at the same time, can maintain their perceptual-motor coordination in stationary environments. In this paper, we explore the role of inter-sensory conflict in this adaptation process. Different measures (vertical nystagmus, illusory body tilt, motion sickness) react differently to visual-vestibular conflict and adapt differently. In particular, proprioceptive-vestibular conflict sufficed to adapt subjective parameters and the time constant of nystagmus decay, while retinal slip was required for VOR gain adaptation. A simple correlation between the strength of intersensory conflict and the efficacy of adaptation fails to explain the data. Implications of these findings, which differ from existing data for low rotation rates, are discussed.

Preservation of vestibular function after scala vestibuli cochlear implantation.

PubMed

Suzuki, Mitsuya; Goto, Takio; Kashio, Akinori; Yasui, Takuya; Sakamoto, Takashi; Ito, Ken; Yamasoba, Tatsuya

2011-10-01

A 58-year-old man, in whom the cochlear implant (CI) had been inserted into the left ear, had right middle-ear cancer. The CI was removed immediately before receiving subtotal removal of right temporal bone. Four months later, the CI was again inserted in his left cochlea. Because of obliterated scala tympani, the 22 active electrodes of the CI were placed into the scala vestibuli. After the surgery, the patient complained that he experienced rotary vertigo and "jumbling of vertical direction" of objects on walking. Using rotation test, we evaluated vestibular function of remaining left ear. Numerous horizontal nystagmus beats were induced during earth-vertical axis rotation, whereas vertical downbeat nystagmus was scarcely induced during off-vertical axis rotation. The horizontal vestibulo-ocular reflex (VOR) was almost normally induced by sinusoidal stimulation at 0.8Hz. These data suggest that the scala vestibuli insertion of CI would be not so invasive against the lateral semicircular canal. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Identifcation of a novel mutation p.I240T in the FRMD7 gene in a family with congenital nystagmus.

PubMed

Zhu, Yihua; Zhuang, Jianfu; Ge, Xianglian; Zhang, Xiao; Wang, Zheng; Sun, Ji; Yang, Juhua; Gu, Feng

2013-10-30

Congenital Nystagmus (CN) is a genetically heterogeneous ocular disease, which causes a significant proportion of childhood visual impairment. To identify the underlying genetic defect of a CN family, twenty-two members were recruited. Genotype analysis showed that affected individuals shared a common haplotype with markers flanking FRMD7 locus. Sequencing FRMD7 revealed a T > C transition in exon 8, causing a conservative substitution of Isoleucine to Tyrosine at codon 240. By protein structural modeling, we found the mutation may disrupt the hydrophobic core and destabilize the protein structure. We reviewed the literature and found that exons 2, 8, and 9 (11.4% of the sequence of FRMD7 mRNA) represent the majority (55.3%) of the reported FRMD7 mutations. In summary, we identified a novel mutation in FRMD7, showed its molecular consequence, and revealed the mutation-rich exons of the FRMD7 gene. Collectively, this provides molecular insights for future CN clinical genetic diagnosis and treatment.

Identifcation of a Novel Mutation p.I240T in the FRMD7 gene in a Family with Congenital Nystagmus

PubMed Central

Zhu, Yihua; Zhuang, Jianfu; Ge, Xianglian; Zhang, Xiao; Wang, Zheng; Sun, Ji; Yang, Juhua; Gu, Feng

2013-01-01

Congenital Nystagmus (CN) is a genetically heterogeneous ocular disease, which causes a significant proportion of childhood visual impairment. To identify the underlying genetic defect of a CN family, twenty-two members were recruited. Genotype analysis showed that affected individuals shared a common haplotype with markers flanking FRMD7 locus. Sequencing FRMD7 revealed a T > C transition in exon 8, causing a conservative substitution of Isoleucine to Tyrosine at codon 240. By protein structural modeling, we found the mutation may disrupt the hydrophobic core and destabilize the protein structure. We reviewed the literature and found that exons 2, 8, and 9 (11.4% of the sequence of FRMD7 mRNA) represent the majority (55.3%) of the reported FRMD7 mutations. In summary, we identified a novel mutation in FRMD7, showed its molecular consequence, and revealed the mutation-rich exons of the FRMD7 gene. Collectively, this provides molecular insights for future CN clinical genetic diagnosis and treatment. PMID:24169426

Artificial gravity: head movements during short-radius centrifugation

NASA Technical Reports Server (NTRS)

Young, L. R.; Hecht, H.; Lyne, L. E.; Sienko, K. H.; Cheung, C. C.; Kavelaars, J.

2001-01-01

Short-radius centrifugation is a potential countermeasure to long-term weightlessness. Unfortunately, head movements in a rotating environment induce serious discomfort, non-compensatory vestibulo-ocular reflexes, and subjective illusions of body tilt. In two experiments we investigated the effects of pitch and yaw head movements in participants placed supine on a rotating bed with their head at the center of rotation, feet at the rim. The vast majority of participants experienced motion sickness, inappropriate vertical nystagmus and illusory tilt and roll as predicted by a semicircular canal model. However, a small but significant number of the 28 participants experienced tilt in the predicted plane but in the opposite direction. Heart rate was elevated following one-second duration head turns. Significant adaptation occurred following a series of head turns in the light. Vertical nystagmus, motion sickness and illusory tilt all decreased with adaptation. Consequences for artificial gravity produced by short-radius centrifuges as a countermeasure are discussed. Grant numbers: NCC 9-58. c 2001. Elsevier Science Ltd. All rights reserved.

Four cases of vertebrobasilar insufficiency.

PubMed

Inui, H; Yoneyama, K; Kitaoku, Y; Nakane, M; Ohue, S; Yamanaka, T; Ueda, T; Fujita, N; Miyahara, H; Matsunaga, T

1998-01-01

Four cases of vertebrobasilar insufficiency are reported. Case 1 was a 38-year-old man who felt a sudden onset of dizziness when he turned his head to the back. An abnormal positional nystagmus was observed when he rotated his head to the left. A magnetic resonance angiogram (MRA) demonstrated total occlusion of the left vertebral artery (VA). Case 2 was a 31-year-old woman who had a total occlusion of her left VA as observed in the MRA. Case 3 was a 68-year-old man who noted dizziness. The systolic blood pressure change on his Schellong test was 28 mmHg. On his MRA, severe displacement of the basilar and the vertebral arteries was visible. Case 4 was a 76-year-old woman who noted a blackout-like sensation. Optokinetic nystagmus was noted with a hyponystagmus pattern, and an eye tracking test showed a saccadic pattern. On her MRA, the vertebrobasilar system was narrowed. The arterial architecture and any stenosis of the blood vessels could be detected non-invasively by MRA.

[Built-in emergency brake in the balance system. Animal experiment research shows that a hierarchy of mechanisms compensate after acute peripheral vestibular decline].

PubMed

Magnusson, Anna K; Tham, Richard

A sudden unilateral loss of peripheral vestibular input results in the onset of acute dizziness and imbalance associated with spontaneous nystagmus, postural instability and nausea. Fortunately, these symptoms ameliorate rapidly, even without treatment, due to central nervous plastic changes which are collectively termed "vestibular compensation". This concept has become a widely accepted research model for studying lesion-induced plasticity. Recent research has dealt in particular with the plasticity of the medial vestibular nuclei that mediate the horizontal vestibulo-ocular reflex. Studies range from a cellular level in vitro to a functional level in vivo. Taken together, results from such studies have contributed greatly to what is known of vestibular compensation today. This article summarises evidence for several plasticity mechanisms that drive the recovery of spontaneous nystagmus, one of which is dependent on an endocrine stress-response. In the long run, such knowledge might influence the management and treatment of patients with balance disorders.

Stabilization of gaze during circular locomotion in light. I. Compensatory head and eye nystagmus in the running monkey

NASA Technical Reports Server (NTRS)

Solomon, D.; Cohen, B.

1992-01-01

1. A rhesus and cynomolgus monkey were trained to run around the perimeter of a circular platform in light. We call this "circular locomotion" because forward motion had an angular component. Head and body velocity in space were recorded with angular rate sensors and eye movements with electrooculography (EOG). From these measurements we derived signals related to the angular velocity of the eyes in the head (Eh), of the head on the body (Hb), of gaze on the body (Gb), of the body in space (Bs), of gaze in space (Gs), and of the gain of gaze (Gb/Bs). 2. The monkeys had continuous compensatory nystagmus of the head and eyes while running, which stabilized Gs during the slow phases. The eyes established and maintained compensatory gaze velocities at the beginning and end of the slow phases. The head contributed to gaze velocity during the middle of the slow phases. Slow phase Gb was as high as 250 degrees/s, and targets were fixed for gaze angles as large as 90-140 degrees. 3. Properties of the visual surround affected both the gain and strategy of gaze compensation in the one monkey tested. Gains of Eh ranged from 0.3 to 1.1 during compensatory gaze nystagmus. Gains of Hb varied around 0.3 (0.2-0.7), building to a maximum as Eh dropped while running past sectors of interest. Consistent with predictions, gaze gains varied from below to above unity, when translational and angular body movements with regard to the target were in opposite or the same directions, respectively. 4. Gaze moved in saccadic shifts in the direction of running during quick phases. Most head quick phases were small, and at times the head only paused during an eye quick phase. Eye quick phases were larger, ranging up to 60 degrees. This is larger than quick phases during passive rotation or saccades made with the head fixed. 5. These data indicate that head and eye nystagmus are natural phenomena that support gaze compensation during locomotion. Despite differential utilization of the head and eyes in various conditions, Gb compensated for Bs. There are various frames of reference in which an estimate of angular velocity that drives the head and eyes could be based. We infer that body in space velocity (Bs) is likely to be represented centrally to provide this signal.

Gaze‐evoked nystagmus induced by alcohol intoxication

PubMed Central

Tarnutzer, Alexander A.; Straumann, Dominik; Ramat, Stefano; Bertolini, Giovanni

2017-01-01

Key points The cerebellum is the core structure controlling gaze stability. Chronic cerebellar diseases and acute alcohol intoxication affect cerebellar function, inducing, among others, gaze instability as gaze‐evoked nystagmus.Gaze‐evoked nystagmus is characterized by increased centripetal eye‐drift. It is used as an important diagnostic sign for patients with cerebellar degeneration and to assess the ‘driving while intoxicated’ condition.We quantified the effect of alcohol on gaze‐holding using an approach allowing, for the first time, the comparison of deficits induced by alcohol intoxication and cerebellar degeneration.Our results showed that alcohol intoxication induces a two‐fold increase of centripetal eye‐drift.We establish analysis techniques for using controlled alcohol intake as a model to support the study of cerebellar deficits.The observed similarity between the effect of alcohol and the clinical signs observed in cerebellar patients suggests a possible pathomechanism for gaze‐holding deficits. Abstract Gaze‐evoked nystagmus (GEN) is an ocular‐motor finding commonly observed in cerebellar disease, characterized by increased centripetal eye‐drift with centrifugal correcting saccades at eccentric gaze. With cerebellar degeneration being a rare and clinically heterogeneous disease, data from patients are limited. We hypothesized that a transient inhibition of cerebellar function by defined amounts of alcohol may provide a suitable model to study gaze‐holding deficits in cerebellar disease. We recorded gaze‐holding at varying horizontal eye positions in 15 healthy participants before and 30 min after alcohol intake required to reach 0.6‰ blood alcohol content (BAC). Changes in ocular‐motor behaviour were quantified measuring eye‐drift velocity as a continuous function of gaze eccentricity over a large range (±40 deg) of horizontal gaze angles and characterized using a two‐parameter tangent model. The effect of alcohol on gaze stability was assessed analysing: (1) overall effects on the gaze‐holding system, (2) specific effects on each eye and (3) differences between gaze angles in the temporal and nasal hemifields. For all subjects, alcohol consumption induced gaze instability, causing a two‐fold increase [2.21 (0.55), median (median absolute deviation); P = 0.002] of eye‐drift velocity at all eccentricities. Results were confirmed analysing each eye and hemifield independently. The alcohol‐induced transient global deficit in gaze‐holding matched the pattern previously described in patients with late‐onset cerebellar degeneration. Controlled intake of alcohol seems a suitable disease model to study cerebellar GEN. With alcohol resulting in global cerebellar hypofunction, we hypothesize that patients matching the gaze‐holding behaviour observed here suffered from diffuse deficits in the gaze‐holding system as well. PMID:27981586

The Standardized Field Sobriety Tests (SFST) and measures of cognitive functioning.

PubMed

Downey, Luke A; Hayley, Amie C; Porath-Waller, Amy J; Boorman, Martin; Stough, Con

2016-01-01

The Standardized Field Sobriety Tests (SFST) are utilised widely to assess fitness to drive when law enforcement suspects a driver's ability to drive is impaired, whether by drugs or alcohol. The SFST ostensibly achieve this through assessment of the level of drivers' cognitive and psychomotor impairment, although no studies have explicitly assessed the relatedness of cognitive ability and performance on the SFST. The current study aimed to assess the relationship between the three components of the SFST with a well validated computerised cognitive battery. A sub-set of 61 placebo condition participants comprised the sample, with 33 females and 28 males (mean age 25.45 years). Correlations between the individual SFST subscales 'Horizontal Gaze Nystagmus' (HGN), the 'One Leg Stand' (OLS) and the 'Walk and Turn' test (WAT) and Cognitive Drug Research (CDR) sub-scales of 'Quality of Working Memory', 'Power of Attention' and 'Continuity of Attention' were analysed using point-biserial correlation. Sixty participants were included for analyses. A weak-moderate positive (five subscales) and a moderate-strong negative (two subscales) association was noted between seven of the nine individual CDR subscales and the SFST subscale of the WAT test (all p<0.05). Individually, a moderate positive association was noted between the sub-scale 'Nystagmus lack of smooth pursuit' and 'digit vigilance reaction time' and 'choice reaction time; reaction time' (both p<0.05) and 'Nystagmus head move and/or jerk' and 'simple reaction time' (p<0.001). When assessed as a partially composite factor, a comparable association was also noted between the composite score of the SFST subscale 'Nystagmus head move and/or jerk' and both (a) simple and (b) digit vigilance reaction time (both p<0.05). No association was noted between any of the individual cognitive variables and the SFST subscale 'OLS', or between composite cognitive scores 'Quality of Working Memory', 'Power of Attention' and 'Continuity of Attention' and total SFST scores. Variation in some aspects of cognitive performance was found to be moderately and positively correlated with some individual aspects of the SFST; particularly among tasks which assess reaction time. Impairment of these cognitive processes can also contribute to the completion of complex tasks such as driving or the SFST. Complex behavioural tasks such as driving are often severely impaired due to intoxication, and thus in a practical sense, the SFST can still be considered a useful screening tool to identify drug or alcohol impaired drivers. Copyright © 2015 Elsevier Ltd. All rights reserved.

Visual Behaviors and Adaptations Associated with Cortical and Ocular Impairment in Children.

ERIC Educational Resources Information Center

Jan, J. E.; Groenveld, M.

1993-01-01

This article shows the usefulness of understanding visual behaviors in the diagnosis of various types of visual impairments that are due to ocular and cortical disorders. Behaviors discussed include nystagmus, ocular motor dyspraxia, head position, close viewing, field loss adaptations, mannerisms, photophobia, and abnormal color perception. (JDD)

Is the Cerebellum Involved in Motor and Perceptual Timing: A Case Study.

DTIC Science & Technology

1985-05-15

return to regular employment as a typist. *The visual fields and cranial nerves were intact. The extraocular movements wererfull and there was no nystagmus ...Her saccades were hypermetric, especially on gaze to the left. Examination of motor function in the upper extremities revealed normal strength

Motion and Spatial Disorientation Systems: Special Research Capabilities.

DTIC Science & Technology

1993-04-01

stimulation of’ the with an efficient slip-ring transmission system horizontal semicircular canals. The RS-3 has for transmitting nystagmus signals. The chair...houses driven power source, air conditioning system, equipment related to a k1erformance-based and water service, is designed to operate test of gaze

Adaptation to vestibular disorientation. VII, Special effects of brief periods of visual fixation on nystagmus and sensations of turning.

DOT National Transportation Integrated Search

1967-05-01

The influences of (a) disorientation experience and (b) brief periods of visual still-fixation were examined in exposing figure skaters and ordinary subjects to various rates of angular acceleration. Both nystagmic eye movements and sensations of tur...

Relation of Infant Vision to Early Cognitive and Language Status.

ERIC Educational Resources Information Center

Duckman, Robert; Tulloch, Deborah

Relationships between infant visual skills and the development of object permanence and expressive language skills were examined with 31 infants in three groups: visually typical, visually atypical, and Down Syndrome. Measures used to evaluate visual status were: forced preferential looking, optokinetic nystagmus, and behavioral. Object permanence…

Hypomyelination Associated with Bovine Viral Diarrhea Virus Type 2 Infection in a Longhorn Calf

USDA-ARS?s Scientific Manuscript database

A newborn Longhorn heifer calf presented to the Veterinary Medical Teaching Hospital at Texas A&M University with generalized tremors, muscle fasciculations, ataxia, and nystagmus. At necropsy, gross central nervous system lesions were not observed. Histopathologic evaluation of the brain and spin...

Vestibular Dysfunction in Preschool Children with a History of Otitis Media.

ERIC Educational Resources Information Center

Denning, Jennifer; Mayberry, Wanda

1987-01-01

Thirty-five preschoolers, assigned to otitis media (OM) or no OM groups, were administered the Southern California Postrotary Nystagmus Test and the Miller Assessment for Preschoolers (MAP). Children with a history of OM had significantly decreased scores on the Stepping and Vertical Writing MAP tests, indicating vestibulospinal dysfunction. (SK)

Application of the System Identification Technique to Goal-Directed Saccades.

DTIC Science & Technology

1985-07-01

Saccadic eye movements are among the fastest voluntary muscle movements the human body is capable of producing and are characterized by a rapid shift of gaze ...moving the target the same distance the eyeball moves. Collewijn and Van der Mark (9), in their study of the slow phase of optokinetic nystagmus , used

Adaptation to vestibular disorientation : V : Eye-Movement and subjective turning responses to two durations of angular acceleration.

DOT National Transportation Integrated Search

1967-05-01

Recordings of ocular nystagmus were obtained from a group of cats and a groups of human subjects to 4/sec2 angular accelerations of 8.4 sec and of 36 sec duration. Laternal canals and vertical canals were stimulated on seperate trails. Results showed...

Children of Methadone-Maintained Mothers: Three-Year Follow-Up.

ERIC Educational Resources Information Center

Johnson, Helen L.; And Others

The physical and neurobehavioral findings at 3 years of age for 39 children born to mothers on methadone- maintenance and 23 children born to drug-free comparison mothers are reported. The methadone children had a higher incidence of head circumferences less than the third percentile, nystagmus/strabismus, and otitis media. No differences were…

AhR-deficiency as a cause of demyelinating disease and inflammation.

PubMed

Juricek, Ludmila; Carcaud, Julie; Pelhaitre, Alice; Riday, Thorfinn T; Chevallier, Aline; Lanzini, Justine; Auzeil, Nicolas; Laprévote, Olivier; Dumont, Florent; Jacques, Sebastien; Letourneur, Frank; Massaad, Charbel; Agulhon, Cendra; Barouki, Robert; Beraneck, Mathieu; Coumoul, Xavier

2017-08-29

The Aryl hydrocarbon Receptor(AhR) is among the most important receptors which bind pollutants; however it also regulates signaling pathways independently of such exposure. We previously demonstrated that AhR is expressed during development of the central nervous system(CNS) and that its deletion leads to the occurrence of a congenital nystagmus. Objectives of the present study are to decipher the origin of these deficits, and to identify the role of the AhR in the development of the CNS. We show that the AhR-knockout phenotype develops during early infancy together with deficits in visual-information-processing which are associated with an altered optic nerve myelin sheath, which exhibits modifications in its lipid composition and in the expression of myelin-associated-glycoprotein(MAG), a cell adhesion molecule involved in myelin-maintenance and glia-axon interaction. In addition, we show that the expression of pro-inflammatory cytokines is increased in the impaired optic nerve and confirm that inflammation is causally related with an AhR-dependent decreased expression of MAG. Overall, our findings demonstrate the role of the AhR as a physiological regulator of myelination and inflammatory processes in the developing CNS. It identifies a mechanism by which environmental pollutants might influence CNS myelination and suggest AhR as a relevant drug target for demyelinating diseases.

Early Cerebellar Network Shifting in Spinocerebellar Ataxia Type 6

PubMed Central

Falcon, M.I.; Gomez, C.M.; Chen, E.E.; Shereen, A.; Solodkin, A.

2016-01-01

Spinocerebellar ataxia 6 (SCA6), an autosomal dominant degenerative disease, is characterized by diplopia, gait ataxia, and incoordination due to severe progressive degeneration of Purkinje cells in the vestibulo- and spinocerebellum. Ocular motor deficits are common, including difficulty fixating on moving objects, nystagmus and disruption of smooth pursuit movements. In presymptomatic SCA6, there are alterations in saccades and smooth-pursuit movements. We sought to assess functional and structural changes in cerebellar connectivity associated with a visual task, hypothesizing that gradual changes would parallel disease progression. We acquired functional magnetic resonance imaging and diffusion tensor imaging data during a passive smooth-pursuit task in 14 SCA6 patients, representing a range of disease duration and severity, and performed a cross-sectional comparison of cerebellar networks compared with healthy controls. We identified a shift in activation from vermis in presymptomatic individuals to lateral cerebellum in moderate-to-severe cases. Concomitantly, effective connectivity between regions of cerebral cortex and cerebellum was at its highest in moderate cases, and disappeared in severe cases. Finally, we noted structural differences in the cerebral and cerebellar peduncles. These unique results, spanning both functional and structural domains, highlight widespread changes in SCA6 and compensatory mechanisms associated with cerebellar physiology that could be utilized in developing new therapies. PMID:26209844

Horizontal angular VOR, nystagmus dumping, and sensation duration in spacelab SLS-1 crewmembers

NASA Technical Reports Server (NTRS)

Oman, C. M.; Balkwill, M. D.; Young, L. R. (Principal Investigator)

1993-01-01

In 1G, the apparent time constant (Td) of postrotatory SPV decay with the head tilted face down is 55% of that with head erect (Te). This phenomenon is called "nystagmus dumping" and has been attributed to G effects on VOR velocity storage. Similarly, postrotatory sensation duration with head tilted (Dd) is 32% of that when head erect (De). In parabolic flight, Te and De are 70% of 1-G values, but a pitch back dumping movement produces no further change. Te, Td, and Dd have not previously been measured in orbital flight. VOR and sensation duration was tested in 4 crewmembers in 4 preflight, 1 inflight (days 4 or 5) and 4 post flight sessions. Bitemporal EOG was recorded with eyes open in darkness. Instructions were to "gaze straight ahead," and indicate when "rotation sensation disappears or becomes ambiguous". Subjects were rotated CW and CCW head erect for 1 min at 120 degrees/s, stopped, and EOG was recorded for another 1 min. This procedure was then used to study dumping, except that immediately after chair stop, subjects pitched their head forward 90 degrees. SPV was calculated using order statistic filtering, and dropouts removed using an iterative model fitting method. Te and Td were determined by logarithmic linear regression of mean SPV for each subject. In orbit, 90 degrees pitch movement produced rapid subjective dumping, but not nystagmus dumping. Dd was noticeably shorter ("almost instantaneous") compared to preflight Dd. Te and Td in orbit were similar to preflight Te for 3/4 subjects (rather than to preflight Td as expected). No consistent VOR gain changes were seen in orbit. Although Te is known to decrease acutely in parabolic flight, a longer time constant was measured in 3/4 subjects after 4-5 days adaptation to weightlessness, suggesting a return of angular velocity storage.

Audio-vestibular signs and symptoms in Chiari malformation type i. Case series and literature review.

PubMed

Guerra Jiménez, Gloria; Mazón Gutiérrez, Ángel; Marco de Lucas, Enrique; Valle San Román, Natalia; Martín Laez, Rubén; Morales Angulo, Carmelo

2015-01-01

Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I). We performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease. There were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; one patient showed a sudden hearing loss; and one case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms. The appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Transtympanic gentamicin and fibrin tissue adhesive for treatment of unilateral Menière's disease: effects on vestibular function.

PubMed

Casani, Augusto; Nuti, Daniele; Franceschini, Stefano Sellari; Gaudini, Elisa; Dallan, Iacopo

2005-12-01

To determine the effects of transtympanic injections, with a mixture composed of gentamicin and fibrin tissue adhesive (FTA), on vestibular function of patients with intractable unilateral Menière's disease. This was an open, prospective study. The study was performed at 2 tertiary referral centers. Twenty-six patients affected by "definite" unilateral Menière's disease, unresponsive to medical therapy for at least 6 months, were enrolled. A buffered gentamicin solution mixed with FTA was injected in the middle ear until the development of bedside vestibular hypofunction signs and/or caloric weakness in the treated ear. Vestibular function was evaluated by 3 bedside vestibular tests (observation of spontaneous nystagmus, head shaking test, and head thrust test) and by a caloric test. Tests were performed on days 10 and 30 after completion of treatment. Tests were also performed 3, 6, and 12 months from completion of the gentamicin-FTA protocol. The effects of treatment were also assessed in terms of hearing levels, control of vertigo, and disability status. In 22 of the 26 patients, only 1 gentamicin-FTA injection was necessary to obtain 1 or more signs indicating a reduction of the vestibular function in the treated ear. Four patients needed another treatment because of the persistence of their incapacitating symptoms during the follow-up. Four patients needed more than 1 injection to obtain a vestibular hypofunction. None of the patients who received 1 or 2 injections presented hearing loss in direct temporal relationship to the treatment. A mixture of gentamicin and fibrin glue makes it possible to considerably reduce the number of administrations in patients with intractable unilateral Menière's disease. Spontaneous nystagmus, post head shaking nystagmus, and a head thrust sign are the clinical signs that indicate onset or progression of unilateral vestibular hypofunction. These signs were obtained with only 1 injection in 81% of patients.

Investigating the relationship between foveal morphology and refractive error in a population with infantile nystagmus syndrome.

PubMed

Healey, Natasha; McLoone, Eibhlin; Mahon, Gerald; Jackson, A Jonathan; Saunders, Kathryn J; McClelland, Julie F

2013-04-26

We explored associations between refractive error and foveal hypoplasia in infantile nystagmus syndrome (INS). We recruited 50 participants with INS (albinism n = 33, nonalbinism infantile nystagmus [NAIN] n = 17) aged 4 to 48 years. Cycloplegic refractive error and logMAR acuity were obtained. Spherical equivalent (SER), most ametropic meridian (MAM) refractive error, and better eye acuity (VA) were used for analyses. High resolution spectral-domain optical coherence tomography (SD-OCT) was used to obtain foveal scans, which were graded using the Foveal Hypoplasia Grading Scale. Associations between grades of severity of foveal hypoplasia, and refractive error and VA were explored. Participants with more severe foveal hypoplasia had significantly higher MAMs and SERs (Kruskal-Wallis H test P = 0.005 and P = 0.008, respectively). There were no statistically significant associations between foveal hypoplasia and cylindrical refractive error (Kruskal-Wallis H test P = 0.144). Analyses demonstrated significant differences between participants with albinism or NAIN in terms of SER and MAM (Mann-Whitney U test P = 0.001). There were no statistically significant differences between astigmatic errors between participants with albinism and NAIN. Controlling for the effects of albinism, results demonstrated no significant associations between SER, and MAM and foveal hypoplasia (partial correlation P > 0.05). Poorer visual acuity was associated statistically significantly with more severe foveal hypoplasia (Kruskal-Wallis H test P = 0.001) and with a diagnosis of albinism (Mann-Whitney U test P = 0.001). Increasing severity of foveal hypoplasia is associated with poorer VA, reflecting reduced cone density in INS. Individuals with INS also demonstrate a significant association between more severe foveal hypoplasia and increasing hyperopia. However, in the absence of albinism, there is no significant relation between refractive outcome and degree of foveal hypoplasia, suggesting that foveal maldevelopment in isolation does not impair significantly the emmetropization process. It likely is that impaired emmetropization evidenced in the albinism group may be attributed to the whole eye effect of albinism.

Control of spatial orientation of the angular vestibuloocular reflex by the nodulus and uvula.

PubMed

Wearne, S; Raphan, T; Cohen, B

1998-05-01

Spatial orientation of the angular vestibuloocular reflex (aVOR) was studied in rhesus monkeys after complete and partial ablation of the nodulus and ventral uvula. Horizontal, vertical, and torsional components of slow phases of nystagmus were analyzed to determine the axes of eye rotation, the time constants (Tcs) of velocity storage, and its orientation vectors. The gravito-inertial acceleration vector (GIA) was tilted relative to the head during optokinetic afternystagmus (OKAN), centrifugation, and reorientation of the head during postrotatory nystagmus. When the GIA was tilted relative to the head in normal animals, horizontal Tcs decreased, vertical and/or roll time constants (Tc(vert/roll)) lengthened according to the orientation of the GIA, and vertical and/or roll eye velocity components appeared (cross-coupling). This shifted the axis of eye rotation toward alignment with the tilted GIA. Horizontal and vertical/roll Tcs varied inversely, with T(chor) being longest and T(cvert/roll) shortest when monkeys were upright, and the reverse when stimuli were around the vertical or roll axes. Vertical or roll Tcs were longest when the axes of eye rotation were aligned with the spatial vertical, respectively. After complete nodulo-uvulectomy, T(chor) became longer, and periodic alternating nystagmus (PAN) developed in darkness. T(chor) could not be shortened in any of paradigms tested. In addition, yaw-to-vertical/roll cross-coupling was lost, and the axes of eye rotation remained fixed during nystagmus, regardless of the tilt of the GIA with respect to the head. After central portions of the nodulus and uvula were ablated, leaving lateral portions of the nodulus intact, yaw-to-vertical/roll cross-coupling and control of Tc(vert/roll) was lost or greatly reduced. However, control of Tchor was maintained, and T(chor) continued to vary as a function of the tilted GIA. Despite this, the eye velocity vector remained aligned with the head during yaw axis stimulation after partial nodulo-uvulectomy, regardless of GIA orientation to the head. The data were related to a three-dimensional model of the aVOR, which simulated the experimental results. The model provides a basis for understanding how the nodulus and uvula control processing within the vestibular nuclei responsible for spatial orientation of the aVOR. We conclude that the three-dimensional dynamics of the velocity storage system are determined in the nodulus and ventral uvula. We propose that the horizontal and vertical/roll Tcs are separately controlled in the nodulus and uvula with the dynamic characteristics of vertical/roll components modulated in central portions and the horizontal components laterally, presumably in a semicircular canal-based coordinate frame.

Orientation of human optokinetic nystagmus to gravity: a model-based approach

NASA Technical Reports Server (NTRS)

Gizzi, M.; Raphan, T.; Rudolph, S.; Cohen, B.

1994-01-01

Optokinetic nystagmus (OKN) was induced by having subjects watch a moving display in a binocular, head-fixed apparatus. The display was composed of 3.3 degrees stripes moving at 35 degrees/s for 45 s. It subtended 88 degrees horizontally by 72 degrees vertically of the central visual field and could be oriented to rotate about axes that were upright or tilted 45 degrees or 90 degrees. The head was held upright or was tilted 45 degrees left or right on the body during stimulation. Head-horizontal (yaw axis) and head-vertical (pitch axis) components of OKN were recorded with electro-oculography (EOG). Slow phase velocity vectors were determined and compared with the axis of stimulation and the spatial vertical (gravity axis). With the head upright, the axis of eye rotation during yaw axis OKN was coincident with the stimulus axis and the spatial vertical. With the head tilted, a significant vertical component of eye velocity appeared during yaw axis stimulation. As a result the axis of eye rotation shifted from the stimulus axis toward the spatial vertical. Vertical components developed within 1-2 s of stimulus onset and persisted until the end of stimulation. In the six subjects there was a mean shift of the axis of eye rotation during yaw axis stimulation of approximately 18 degrees with the head tilted 45 degrees on the body. Oblique optokinetic stimulation with the head upright was associated with a mean shift of the axis of eye rotation toward the spatial vertical of 9.2 degrees. When the head was tilted and the same oblique stimulation was given, the axis of eye rotation rotated to the other side of the spatial vertical by 5.4 degrees. This counterrotation of the axis of eye rotation is similar to the "Muller (E) effect," in which the perception of the upright is counterrotated to the opposite side of the spatial vertical when subjects are tilted in darkness. The data were simulated by a model of OKN with a "direct" and "indirect" pathway. It was assumed that the direct visual pathway is oriented in a body, not a spatial frame of reference. Despite the short optokinetic after-nystagmus time constants, strong horizontal to vertical cross-coupling could be produced if the horizontal and vertical time constants were in proper ratio and there were no suppression of nystagmus in directions orthogonal to the stimulus direction. The model demonstrates that the spatial orientation of OKN can be achieved by restructuring the system matrix of velocity storage. We conclude that an important function of velocity storage is to orient slow-phase velocity toward the spatial vertical during movement in a terrestrial environment.

The Efficacy of Dextroamphetamine as a Motion Sickness Countermeasure for the Use in Military Operational Environments

DTIC Science & Technology

2008-07-09

movement and to ensure head-centered movement during rotation. The subject’s gaze was directed to a black visual field inside the device to provide a...vertical nystagmus . (NAMI-1079 NASA Order No. R-93). Pensacola, FL: Naval Aerospace Medical Institute. Homick, J. L., Kohl, R. L., Reschke, M. F

Auditory, Vestibular and Cognitive Effects due to Repeated Blast Exposure on the Warfighter

DTIC Science & Technology

2012-07-01

for testing): • Spontaneous nystagmus • Smooth Harmonic Acceleration (.01, .08, .32, .64, 1. 75) • Saccades Horizontal and Vertical • Smooth...Pursuit Horizontal (0.1, 0.2, 0.4, . 71) • Smooth Pursuit Vertical (0 .1, 0.2, 0.4, .71) • Gaze Horizontal • Gaze Vertical • OKN Trapezoidal (20, 40, 60

Evaluation of a Simple Device for Scoring Nystagmus.

DTIC Science & Technology

1979-05-15

thle NC 0- 1 00. s-oi ril, begoan at tilie end of tile iirl igat ion Pm~ iod and e.nded after the resporrw hodi~ cbd its mfi xisiruril value anld was...arid other similar artifacts would then permit selec- tion of the appropriate interval for handscoring, or by thc NCO-100 if the record were on tape

[These vestibular problems in the absence of gravity...].

PubMed

Timsit, C

1986-01-01

For a few years, more and more astronauts complain to endure space motion sickness during the two or three first days of their mission. This is due to the repetition of shifting and sudden head movements, which becomes possible by the increasing of volume of the new space stations. To avoid that payload specialists onboard be obliged to renounce to conduct planned experiments, it has been necessary to find effective solutions to detect by ground based tests the candidates sensitive to space motion sickness and perfect therapeutic means able to avoid unexpected arrival of these symptoms, and even to treat them. The best results are undeniably obtained by the "Biofeedback" and the "tolerance" training, but we base wide hopes on ginger roots and on tolerance with sensorial deprivation lockers. However, we must not disregard the trigger action of emotional factors and anxiety in space motion sickness. The European mission SPACELAB-1 has been marked by the display of a caloric nystagmus during the vestibular experiments in weightlessness. If no explanation is given to this phenomenon, it will be necessary to call in question the role of the thermal convection described by Barany in the appearance of the caloric nystagmus.

A low-cost video-oculography system for vestibular function testing.

PubMed

Jihwan Park; Youngsun Kong; Yunyoung Nam

2017-07-01

In order to remain in focus during head movements, vestibular-ocular reflex causes eyes to move in the opposite direction to head movement. Disorders of vestibular system decrease vision, causing abnormal nystagmus and dizziness. To diagnose abnormal nystagmus, various studies have been reported including the use of rotating chair tests and videonystagmography. However, these tests are unsuitable for home use due to their high costs. Thus, a low-cost video-oculography system is necessary to obtain clinical features at home. In this paper, we present a low-cost video-oculography system using an infrared camera and Raspberry Pi board for tracking the pupils and evaluating a vestibular system. Horizontal eye movement is derived from video data obtained from an infrared camera and infrared light-emitting diodes, and the velocity of head rotation is obtained from a gyroscope sensor. Each pupil was extracted using a morphology operation and a contour detection method. Rotatory chair tests were conducted with our developed device. To evaluate our system, gain, asymmetry, and phase were measured and compared with System 2000. The average IQR errors of gain, phase and asymmetry were 0.81, 2.74 and 17.35, respectively. We showed that our system is able to measure clinical features.

Medial vestibulospinal tract lesions impair sacculo-collic reflexes.

PubMed

Kim, Seonhye; Lee, Hak-Seung; Kim, Ji Soo

2010-05-01

The medial vestibulospinal tract (VST) is known to mediate the vestibular-evoked myogenic potential (VEMP) in the contracting sternocleidomastoid muscle (SCM). To determine whether disruption of the medial VST in the medulla impairs formation of VEMP, we measured VEMP in 14 patients with medial medullary infarction (MMI). VEMP was induced by a short tone burst and was recorded in contracting SCM while patients turned their heads forcefully to the contralateral side against resistance. Normative data were obtained from 47 healthy volunteers. Seven patients (50%) had abnormal VEMP in the side of the MMI lesion, absent in two, decreased in four, and delayed in two. One patient showed both decreased and delayed response. Of the seven patients with abnormal VEMP, five had the lesions that extended to the dorsal tegmentum while five of the seven patients with normal VEMP showed restricted anteromedial lesions mainly involving the pyramids. Spontaneous nystagmus (4/7, 57%), gaze-evoked nystagmus (6/7, 86%), and ocular tilt reaction/tilt of the subjective visual vertical (4/7, 57%) were frequently observed in the patients with abnormal VEMP. The abnormal VEMP in patients with infarctions involving the medullary tegmentum supports that VEMP is mediated by the medial VST descending within the medial longitudinal fasciculus.

Divided visual attention: A comparison of patients with multiple sclerosis and controls, assessed with an optokinetic nystagmus suppression task.

PubMed

Williams, Isla M; Schofield, Peter; Khade, Neha; Abel, Larry A

2016-12-01

Multiple sclerosis (MS) frequently causes impairment of cognitive function. We compared patients with MS with controls on divided visual attention tasks. The MS patients' and controls' stare optokinetic nystagmus (OKN) was recorded in response to a 24°/s full field stimulus. Suppression of the OKN response, judged by the gain, was measured during tasks dividing visual attention between the fixation target and a second stimulus, central or peripheral, static or dynamic. All participants completed the Audio Recorded Cognitive Screen. MS patients had lower gain on the baseline stare OKN. OKN suppression in divided attention tasks was the same in MS patients as in controls but in both groups was better maintained in static than in dynamic tasks. In only dynamic tasks, older age was associated with less effective OKN suppression. MS patients had lower scores on a timed attention task and on memory. There was no significant correlation between attention or memory and eye movement parameters. Attention, a complex multifaceted construct, has different neural combinations for each task. Despite impairments on some measures of attention, MS patients completed the divided visual attention tasks normally. Copyright © 2016 Elsevier Ltd. All rights reserved.

Crowded visual search in children with normal vision and children with visual impairment.

PubMed

Huurneman, Bianca; Cox, Ralf F A; Vlaskamp, Björn N S; Boonstra, F Nienke

2014-03-01

This study investigates the influence of oculomotor control, crowding, and attentional factors on visual search in children with normal vision ([NV], n=11), children with visual impairment without nystagmus ([VI-nys], n=11), and children with VI with accompanying nystagmus ([VI+nys], n=26). Exclusion criteria for children with VI were: multiple impairments and visual acuity poorer than 20/400 or better than 20/50. Three search conditions were presented: a row with homogeneous distractors, a matrix with homogeneous distractors, and a matrix with heterogeneous distractors. Element spacing was manipulated in 5 steps from 2 to 32 minutes of arc. Symbols were sized 2 times the threshold acuity to guarantee visibility for the VI groups. During simple row and matrix search with homogeneous distractors children in the VI+nys group were less accurate than children with NV at smaller spacings. Group differences were even more pronounced during matrix search with heterogeneous distractors. Search times were longer in children with VI compared to children with NV. The more extended impairments during serial search reveal greater dependence on oculomotor control during serial compared to parallel search. Copyright © 2014 Elsevier B.V. All rights reserved.

Effect of 4-aminopyridine on gravity dependence and neural integrator function in patients with idiopathic downbeat nystagmus.

PubMed

Sander, T; Sprenger, A; Marti, S; Naumann, T; Straumann, D; Helmchen, C

2011-04-01

Downbeat nystagmus (DBN) is a frequent sign in patients with cerebellar degeneration. It consists of an upward drift of the eye that does not depend on vertical head position (spontaneous drift, SD), a gravity-dependent component (GD), and a gaze-evoked drift reflecting gaze-holding impairment (deficient neural integrator function). The potassium-channel blocker 4-aminopyridine (4-AP) is reported to reduce DBN in patients with cerebellar atrophy but with little or no effect in patients with idiopathic DBN. We prospectively studied the effect of 4-AP on all three components in a large (n = 24) group of the clinically frequent idiopathic DBN. DBN was reduced by 22-31% when the head was off the head erect position. In contrast, there was no effect on vertical gaze-evoked drift. This indicates the therapeutic efficacy of 4-AP not only in patients with cerebellar atrophy but also in idiopathic DBN patients. This beneficial effect, which might be missed when gravity-dependent head positions are not tested, was not related to an improvement of gaze-holding deficit. We suggest it may be related to the restored inhibition of the overacting otolith-ocular reflex.

Vestibuloocular reflex of rhesus monkeys after spaceflight

NASA Technical Reports Server (NTRS)

Cohen, Bernard; Kozlovskaia, Inessa; Raphan, Theodore; Solomon, David; Helwig, Denice; Cohen, Nathaniel; Sirota, Mikhail; Iakushin, Sergei

1992-01-01

The vestibuloocular reflex (VOR) of two rhesus monkeys was recorded before and after 14 days of spaceflight. The gain (eye velocity/head velocity) of the horizontal VOR, tested 15 and 18 h after landing, was approximately equal to preflight values. The dominant time constant of the animal tested 15 h after landing was equivalent to that before flight. During nystagmus induced by off-vertical axis rotation (OVAR), the latency, rising time constant, steady-state eye velocity, and phase of modulation in eye velocity and eye position with respect to head position were similar in both monkeys before and after flight. There were changes in the amplitude of modulation of horizontal eye velocity during steady-state OVAR and in the ability to discharge stored activity rapidly by tilting during postrotatory nystagmus (tilt dumping) after flight: OVAR modulations were larger, and tilt dumping was lost in the one animal tested on the day of landing and for several days thereafter. If the gain and time constant of the horizontal VOR exchange in microgravity, they must revert to normal soon after landing. The changes that were observed suggest that adaptation to microgravity had caused alterations in way that the central nervous system processes otolith input.

Leber congenital amaurosis associated with Chiari I malformation: Two cases and a review of the literature

PubMed Central

Petraglia, Anthony L.; Chengazi, Harris U.; Chung, Mina M.; Silberstein, Howard J.

2012-01-01

Objective: Leber congenital amaurosis (LCA) is a rare, clinically and genetically heterogeneous disorder characterized by severe loss of vision in the first year of life, affecting approximately 3000 people in the United States. Some LCA patients manifest developmental abnormalities of the central nervous system (CNS) and neuroradiological studies have revealed a variety of cerebral anomalies in association with LCA; however, Chiari I malformations (CMI) have never been described. Case Description: We report two sisters who were referred to the pediatric neurosurgery clinic for evaluation of CMI. The elder sister presented with convergence nystagmus from 3 months of age and magnetic resonance imaging (MRI) demonstrated evidence of significant CMI. Her younger sister began developing nystagmus at 4 months of age. Both had symptomatic progression and underwent suboccipital decompression. Both were subsequently diagnosed with LCA. Case specifics and imaging findings are presented. Conclusions: CMI have been found in association with several genetic syndromes, but not with LCA. These patients represent the first reported cases of CMI with LCA and suggest an additional potential CNS anomaly. The unique occurrence in siblings and the association with another inherited disorder are suggestive of a genetic basis for CMI. PMID:22347674

Electro-oculography of smooth pursuit and optokinetic nystagmus eye movements in type I Duane's retraction syndrome.

PubMed

Melek, Nélida B; Blanco, Susana; Garcia, Horacio

2006-01-01

These two eye movements have not been previously studied in this condition by this method. Five cases were studied. Both visual acuity and eye examination of anterior and posterior segments were normal. A Nicolet Nystar Plus system with chloride silver electrodes was used to record the EOG. Of the two systems under study, the smooth pursuit system showed the most relevant anomalies, both in the Duane's eye and in the apparently healthy eye. No correlation was found between the pursuit and optokinetic nystagmus disorders. In some cases, more significant abnormalities were observed in the clinically normal eye. The results clearly demonstrated a significant impairment of the pursuit system. This suggests that this motor disorder is not exclusively caused by hypoplasia or aplasia of the nucleus of the abducens nerve (VIth cranial nerve). These abnormalities might be related to a poor development of the rhombencephalon since both supramotor nuclei as well as the pathways of this system arise from this region of the embryonic brain. In the particular case of OKN, the supramotor nuclei have a different origin. Therefore, these systems might be affected differently.

Surgical treatment of vertigo in cochlear implantees by electrode resealing.

PubMed

Karimi, Dania; Mittmann, Philipp; Ernst, Arneborg; Todt, Ingo

2017-10-01

Our present findings demonstrate that resealing to cover the electrode is an effective method to treat vertigo after CI. An insufficient cochleostomy sealing can be regarded as a cause of postoperatively newly occuring vertigo after CI. A transtympanic revision is a promising treatment option in cases of post-operative dizziness. Intoduction: A well-known and frequently reported complication after cochlear implantation is the appearance of postoperative vertigo symptoms. The aim of the present study was to observe if the postoperatively new occurrence of vertigo can be treated by resealing of the round window patch after cochlear implantation. A retrospective analysis revealed that 10 patients underwent revision surgery transtympanally. Vertigo was assessed preoperatively and directly postoperatively and after 6 month after revision surgery by using the Dizziness Handycap Inventory (DHI). The most common symptom was rotating vertigo. A spontanous nystagmus was seen in four cases. No nystagmus was found after the revision surgery. In three cases, the onset of dizziness was associated with an event (sneezing, otitis media, climbing a mountain). A preoperative CT showed insuspectible results in seven patients but revealed pathologies two patients. Vertigo was improved significantly in six patients, and three of them were symptom-free.

Impaired eye movements in presymptomatic spinocerebellar ataxia type 6.

PubMed

Christova, Peka; Anderson, John H; Gomez, Christopher M

2008-04-01

Early detection of impaired neurological function in neurodegenerative diseases may aid in understanding disease pathogenesis and timing of therapeutic trials. To identify early abnormalities of ocular motor function in individuals who have the spinocerebellar ataxia type 6 (SCA6) gene (CACNA1A) but no clinical symptoms. Physiological techniques were used to record and analyze eye movements and postural sway. Four presymptomatic and 5 ataxic patients with SCA6, genetically identified, and 10 healthy controls. Presymptomatic individuals had normal postural sway but definite ocular motor abnormalities. Two had a low-amplitude horizontal gaze-evoked nystagmus, 1 of whom had a significantly decreased eye velocity for upward saccades and an abnormal frequency of square-wave jerks. Another had abnormal square-wave jerks and a fourth had a reduced gain for pursuit tracking. Not all of the presymptomatic patients had the same findings, but a multivariate analysis discriminated the presymptomatic patients, as a group, from healthy controls and the ataxic patients. Among the earliest functional deficits in SCA6 are eye movement abnormalities, including impaired saccade velocity, saccade metrics, and pursuit gain. This suggests that early functional impairments are caused by cellular dysfunction and/or loss in the posterior cerebellar vermis and flocculus. These findings might help to determine the timing of a treatment and to define variables that could be used as outcome measures for the efficacy of therapeutic trials.

Plasticity of the human otolith-ocular reflex

NASA Technical Reports Server (NTRS)

Wall, C. 3rd; Smith, T. R.; Furman, J. M.

1992-01-01

The eye movement response to earth vertical axis rotation in the dark, a semicircular canal stimulus, can be altered by prior exposure to combined visual-vestibular stimuli. Such plasticity of the vestibulo-ocular reflex has not been described for earth horizontal axis rotation, a dynamic otolith stimulus. Twenty normal human subjects underwent one of two types of adaptation paradigms designed either to attenuate or enhance the gain of the semicircular canal-ocular reflex prior to undergoing otolith-ocular reflex testing with horizontal axis rotation. The adaptation paradigm paired a 0.2 Hz sinusoidal rotation about a vertical axis with a 0.2 Hz optokinetic stripe pattern that was deliberately mismatched in peak velocity. Pre- and post-adaptation horizontal axis rotations were at 60 degrees/s in the dark and produced a modulation in the slow component velocity of nystagmus having a frequency of 0.17 Hz due to putative stimulation of the otolith organs. Results showed that the magnitude of this modulation component response was altered in a manner similar to the alteration in semicircular canal-ocular responses. These results suggest that physiologic alteration of the vestibulo-ocular reflex using deliberately mismatched visual and semicircular canal stimuli induces changes in both canal-ocular and otolith-ocular responses. We postulate, therefore, that central nervous system pathways responsible for controlling the gains of canal-ocular and otolith-ocular reflexes are shared.

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.

PubMed

Wang, Z I; Dell'Osso, L F

2007-05-01

The objective of this study was to investigate the dynamic properties of infantile nystagmus syndrome (INS) that affect visual function; i.e., which factors influence latency of the initial reflexive saccade (Ls) and latency to target acquisition (Lt). We used our behavioral ocular motor system (OMS) model to simulate saccadic responses (in the presence of INS) to target jumps at different times within a single INS cycle and at random times during multiple cycles. We then studied the responses of 4 INS subjects with different waveforms to test the model's predictions. Infrared reflection was used for 1 INS subject, high-speed digital video for 3. We recorded and analyzed human responses to large and small target-step stimuli. We evaluated the following factors: stimulus time within the cycle (Tc), normalized Tc (Tc%), initial orbital position (Po), saccade amplitude, initial retinal error (e(i)), and final retinal error (e(f)). The ocular motor simulations were performed in MATLAB Simulink environment and the analysis was performed in MATLAB environment using OMLAB software. Both the OMS model and OMtools software are available from http://http:www.omlab.org. Our data analysis showed that for each subject, Ls was a fixed value that is typically higher than the normal saccadic latency. Although saccadic latency appears somewhat lengthened in INS, the amount is insufficient to cause the "slow-to-see" impression. For Lt, Tc% was the most influential factor for each waveform type. The main refixation strategies employed by INS subjects made use of slow and fast phases and catch-up saccades, or combinations of them. These strategies helped the subjects to foveate effectively after target movement, sometimes at the cost of increased target acquisition time. Foveating or braking saccades intrinsic to the nystagmus waveforms seemed to disrupt the OMS' ability to accurately calculate reflexive saccades' amplitude and refoveate. Our OMS model simulations demonstrated this emergent behavior and predicted the lengthy target acquisition times found in the patient data.

Parinaud syndrome: a 25-year (1991-2016) review of 40 consecutive adult cases.

PubMed

Shields, Melissa; Sinkar, Swati; Chan, WengOnn; Crompton, John

2017-12-01

To characterize the clinical features, aetiology and management of ophthalmic symptoms in adult patients with Parinaud syndrome. This is a retrospective, non-comparative observational case series. We reviewed 40 consecutive charts of adult patients with the clinical diagnosis of Parinaud syndrome at the Royal Adelaide Hospital Department of Ophthalmology in Adelaide, South Australia, between 1991 and 2016. Charts were reviewed for the following: (1) demographic information, (2) clinical presentation, (3) neuro-ophthalmology signs, (4) aetiology of Parinaud syndrome, and (5) management. Examination findings were collected at initial evaluation, throughout the course of follow-up, and at last follow-up. All the cases were assessed by one of the authors (JLC). The commonest presenting symptoms were diplopia (67.5%) and blurred vision (25%) followed by visual field defect (12.5%), ataxia (7.5%) and manifest squint (7.5%). The commonest presenting signs were vertical gaze palsy (100%), convergence-retraction nystagmus (87.5%) and light-near dissociation (65.0%). Only 65.0% patients had the classical triad of vertical gaze palsy, convergence-retraction nystagmus and light-near dissociation. Midbrain pathologies including haemorrhage (30.0%), infarction (20.0%) and tumour (15.0%) were the commonest aetiology. Pineal region tumours accounted for 30.0% of presentations. Symptoms were managed conservatively in 45% of cases with temporary occlusion, prisms or refractive correction, and observation in 42.5% of cases. Surgical intervention for refractory diplopia was required in 12.5% of cases, of which 80% reported symptom resolution following surgery. Our series highlights the variable clinical presentation of Parinaud syndrome. The classic triad of conjugate upgaze paralysis, convergence-retraction nystagmus and light-near dissociation was only present in 65% of cases. Pineal neoplasms remain an important aetiological consideration; however, primary midbrain pathology including infarction and haemorrhage constituted the majority of our cases and should be considered in all patients. Conservative management approaches for ocular symptoms are sufficient in most cases although surgical treatment of upgaze palsy can be a useful option in refractory cases. © 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

A young woman with seizures and psychosis

PubMed Central

Naha, Sowjanya; Naha, Kushal; Hande, H Manjunath; Vivek, Ganapathiraman

2014-01-01

We present a case of a 24-year-old woman with abnormal behaviour of recent onset. She had been diagnosed previously with epilepsy and had been started on antiepileptic medication. Clinical examination confirmed features of psychosis including paranoid delusions and auditory hallucination. Neurological examination showed nystagmus and dysmetria. Further evaluation revealed the underlying cause for her symptoms. She responded promptly to appropriate therapy with complete resolution of psychosis. PMID:25008334

Dual Purkinje-Image Eyetracker

DTIC Science & Technology

1996-01-01

Abnormal nystagmus can also be detected through the use of an eyetracker [4]. Through tracking points of eye gaze within a scene, it is possible to...moving, even when gazing . Correcting for these unpredictable micro eye movements would allow corrective procedures in eye surgery to become more accurate...victim with a screen of letters on a monitor. A calibrated eyetracker then provides a processor with information about the location of eye gaze . The

Risk of Central Nervous System Decompression Sickness in Air Diving to No-Stop Limits

DTIC Science & Technology

2009-01-01

190 9 1 10 1. Weak, faint, vertigo 2. Heavy legs, abnormal gait 3. Vertigo and cardiorespiratory symptoms 4. Bilateral numbness and paresthesia ...original report," were included. 6. Pain in both feet, paresthesia 7. Hearing deficit, anisocoria, nystagmus, confusion, emotionally labile, abnormal...tandem gait 8. Scintillating scotoma, abnormal left foot dorsiflexion 9. Weakness, general left side paresthesia , numbness, abnormal gait 10. See

Analysis of residual symptoms after treatment in benign paroxysmal positional vertigo using questionnaire.

PubMed

Lee, No Hee; Kwon, Hee Jun; Ban, Jae Ho

2009-08-01

Canalith repositioning procedure (CRP) provides rapid and long-lasting relief of symptoms in most patients with benign paroxysmal positional vertigo. However, some patients express nonspecific symptoms such as anxiety or discomfort after treatment, even after the disappearance of nystagmus and vertigo. The purpose of this study was to assess the residual symptoms after CRP in patients with benign paroxysmal positional vertigo using the Dizziness Handicap Inventory (DHI) in a questionnaire format. Controlled, prospective study. CRP was performed in 135 patients until nystagmus and vertigo disappeared. Patients were asked to complete the questionnaire before and 5 to 7 days after treatment. A control group of 135 normal volunteers was selected and cross-matched according to the age and sex of the patient group. The data were compared for the pre-CRP, post-CRP, and control groups. There was a significant improvement in the DHI scores when comparing the pre- and post-CRP groups (P=0.000), although six items showed incomplete improvement. Subsequent comparison of DHI scores between the control group and the post-CRP group still showed a difference in some items so that the improvement was incomplete. Even after successful CRPs, Dizziness Handicap Inventory scores indicated that residual subjective symptoms may remain. Thus, additional follow-up and management are important for these patients.

Vestibulo-ocular reflex function in children with high-functioning autism spectrum disorders.

PubMed

Carson, Tana B; Wilkes, Bradley J; Patel, Kunal; Pineda, Jill L; Ko, Ji H; Newell, Karl M; Bodfish, James W; Schubert, Michael C; Radonovich, Krestin; White, Keith D; Lewis, Mark H

2017-02-01

Sensorimotor processing alterations are a growing focus in the assessment and treatment of Autism Spectrum Disorders (ASD). The rotational vestibulo-ocular reflex (rVOR), which functions to maintain stable vision during head movements, is a sensorimotor system that may be useful in understanding such alterations and their underlying neurobiology. In this study, we assessed post-rotary nystagmus elicited by continuous whole body rotation among children with high-functioning ASD and typically developing children. Children with ASD exhibited increased rVOR gain, the ratio of eye velocity to head velocity, indicating a possible lack of cerebellar inhibitory input to brainstem vestibular nuclei in this population. The ASD group also showed less regular or periodic horizontal eye movements as indexed by greater variance accounted for by multiple higher frequency bandwidths as well as greater entropy scores compared to typically developing children. The decreased regularity or dysrhythmia in the temporal structure of nystagmus beats in children with ASD may be due to alterations in cerebellum and brainstem circuitry. These findings could potentially serve as a model to better understand the functional effects of differences in these brain structures in ASD. Autism Res 2017, 10: 251-266. © 2016 International Society for Autism Research, Wiley Periodicals, Inc. © 2016 International Society for Autism Research, Wiley Periodicals, Inc.

Oculomotor Neurocircuitry, a Structural Connectivity Study of Infantile Nystagmus Syndrome

PubMed Central

Kashou, Nasser H.; Zampini, Angelica R.

2015-01-01

Infantile Nystagmus Syndrome (INS) is one of the leading causes of significant vision loss in children and affects about 1 in 1000 to 6000 births. In the present study, we are the first to investigate the structural pathways of patients and controls using diffusion tensor imaging (DTI). Specifically, three female INS patients from the same family were scanned, two sisters and a mother. Six regions of interest (ROIs) were created manually to analyze the number of tracks. Additionally, three ROI masks were analyzed using TBSS (Tract-Based Spatial Statistics). The number of fiber tracks was reduced in INS subjects, compared to normal subjects, by 15.9%, 13.9%, 9.2%, 18.6%, 5.3%, and 2.5% for the pons, cerebellum (right and left), brainstem, cerebrum, and thalamus. Furthermore, TBSS results indicated that the fractional anisotropy (FA) values for the patients were lower in the superior ventral aspects of the pons of the brainstem than in those of the controls. We have identified some brain regions that may be actively involved in INS. These novel findings would be beneficial to the neuroimaging clinical and research community as they will give them new direction in further pursuing neurological studies related to oculomotor function and provide a rational approach to studying INS. PMID:25860806

Creutzfeldt-Jakob Disease Presenting With Dizziness and Gaze-Evoked Nystagmus: A Case Report.

PubMed

Choi, Yun-Ju; Kang, Kyung-Wook; Lee, Sae-Young; Kang, Seung-Ho; Lee, Seung-Han; Kim, Byeong C

2016-02-01

Sporadic Creutzfeldt-Jakob disease (CJD) is clinically characterized by rapidly progressive dementia combined with other cardinal symptoms, such as myoclonus, visual or cerebellar disturbances, extrapyramidal or pyramidal disturbance, and akinetic mutism. However, as an initial manifestation, focal neurologic deficits other than the aforementioned or nonspecific generalized symptoms may lead to a misdiagnosis or a delayed diagnosis. The authors report a case of 66-year-old male patient with sporadic CJD who had dizziness, gaze-evoked nystagmus (GEN), and other central eye signs (impaired smooth pursuit, saccadic dysmetria) as an initial manifestation without dementia. The central eye signs led us to perform brain magnetic resonance images, which showed abnormal cortical high-signal intensity in both the cerebral and cerebellar hemispheres including the vestibulocerebellum. We reached a presumptive diagnosis of CJD, but the findings did not meet diagnostic criteria for probable CJD at that time. Three weeks after the initial work-ups, the patient presented with typical neurological findings of CJD: rapidly progressive dementia, akinetic mutism, and myoclonus of the left arm. Cerebrospinal fluid was positive for 14-3-3 protein, and electroencephalography showed periodic sharp wave complexes. In this patient, GEN and other central eye signs provided diagnostic clues for CJD. These unusual neurological manifestations may help physicians have a thorough knowledge of early deficits of CJD.

The ataxic mouse as a model for studying downbeat nystagmus.

PubMed

Stahl, John S; Thumser, Zachary C; Oommen, Brian S

2012-01-01

Downbeat nystagmus (DBN) is a common eye movement complication of cerebellar disease. Use of mice to study pathophysiology of vestibulocerebellar disease is increasing, but it is unclear if mice can be used to study DBN; it has not been reported in this species. We determined whether DBN occurs in the ataxic mutant tottering, which carries a mutation in the Cacna1a gene for P/Q calcium channels. Spontaneous DBN occurred only rarely, and its magnitude did not exhibit the relationship to head tilt seen in human patients. DBN during yaw rotation was more common and shares some properties with the tilt-independent, gaze-independent component of human DBN, but differs in its dependence on vision. Hyperactivity of otolith circuits responding to pitch tilts is hypothesized to contribute to the gaze-independent component of human DBN. Mutants exhibited hyperactivity of the tilt maculo-ocular reflex (tiltMOR) in pitch. The hyperactivity may serve as a surrogate for DBN in mouse studies. TiltMOR hyperactivity correlates with hyperdeviation of the eyes and upward deviation of the head during ambulation; these may be alternative surrogates. Muscimol inactivation of the cerebellar flocculus suggests a floccular role in the tiltMOR hyperactivity and provides insight into the rarity of frank DBN in ataxic mice.

The ataxic mouse as a model for studying downbeat nystagmus

PubMed Central

Stahl, John S.; Thumser, Zachary C.; Oommen, Brian S.

2016-01-01

Downbeat nystagmus (DBN) is a common eye movement complication of cerebellar disease. Use of mice to study pathophysiology of vestibulocerebellar disease is increasing, but it is unclear if mice can be used to study DBN; it has not been reported in this species. We determined whether DBN occurs in the ataxic mutant tottering, which carries a mutation in the Cacna1a gene for P/Q calcium channels. Spontaneous DBN occurred only rarely, and its magnitude did not exhibit the relationship to head tilt seen in human patients. DBN during yaw rotation was more common and shares some properties with the tilt-independent, gaze-independent component of human DBN, but differs in its dependence on vision. Hyperactivity of otolith circuits responding to pitch tilts is hypothesized to contribute to the gaze-independent component of human DBN. Mutants exhibited hyperactivity of the tilt maculo-ocular reflex (tiltMOR) in pitch. The hyperactivity may serve as a surrogate for DBN in mouse studies. TiltMOR hyperactivity correlates with hyperdeviation of the eyes and upward deviation of the head during ambulation; these may be alternative surrogates. Muscimol inactivation of the cerebellar flocculus suggests a floccular role in the tiltMOR hyperactivity and provides insight into the rarity of frank DBN in ataxic mice. PMID:23302704

The use of handheld spectral domain optical coherence tomography in pediatric ophthalmology practice: Our experience of 975 infants and children.

PubMed

Mallipatna, Ashwin; Vinekar, Anand; Jayadev, Chaitra; Dabir, Supriya; Sivakumar, Munsusamy; Krishnan, Narasimha; Mehta, Pooja; Berendschot, Tos; Yadav, Naresh Kumar

2015-07-01

Optical coherence tomography (OCT) is an important imaging tool assessing retinal architecture. In this article, we report a single centers experience of using handheld spectral domain (SD)-OCT in a pediatric population using the Envisu 2300 (Bioptigen Inc., Research Triangle Park, NC, USA). We studied SD-OCT images from 975 patients imaged from January 2011 to December 2014. The variety of cases that underwent an SD-OCT was analyzed. Cases examples from different case scenarios were selected to showcase unique examples of many diseases. Three hundred and sixty-eight infants (37.7%) were imaged for retinopathy of prematurity, 362 children (37.1%) underwent the test for evaluation of suboptimal vision or an unexplained vision loss, 126 children (12.9%) for evaluation of nystagmus or night blindness, 54 children (5.5%) for an intraocular tumor or a mass lesion such as retinoblastoma, and 65 children (6.7%) for other diseases of the pediatric retina. The unique findings in the retinal morphology seen with some of these diseases are discussed. The handheld SD-OCT is useful in the evaluation of the pediatric retinal diseases. The test is useful in the assessment of vision development in premature children, evaluation of unexplained vision loss and amblyopia, nystagmus and night blindness, and intraocular tumors (including retinoblastoma).

Motion perception without Nystagmus--a novel manifestation of cerebellar stroke.

PubMed

Shaikh, Aasef G

2014-01-01

The motion perception and the vestibulo-ocular reflex (VOR) each serve distinct functions. The VOR keeps the gaze steady on the target of interest, whereas vestibular perception serves a number of tasks, including awareness of self-motion and orientation in space. VOR and motion perception might abide the same neurophysiological principles, but their distinct anatomical correlates were proposed. In patients with cerebellar stroke in distribution of medial division of posterior inferior cerebellar artery, we asked whether specific location of the focal lesion in vestibulocerebellum could cause impaired perception of motion but normal eye movements. Thirteen patients were studied, 5 consistently perceived spinning of surrounding environment (vertigo), but the eye movements were normal. This group was called "disease model." Remaining 8 patients were also symptomatic for vertigo, but they had spontaneous nystagmus. The latter group was called "disease control." Magnetic resonance imaging in both groups consistently revealed focal cerebellar infarct affecting posterior cerebellar vermis (lobule IX). In the "disease model" group, only part of lobule IX was affected. In the disease control group, however, complete lobule IX was involved. This study discovered a novel presentation of cerebellar stroke where only motion perception was affected, but there was an absence of objective neurologic signs. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

Rotational vertebral artery occlusion: mechanisms and long-term outcome.

PubMed

Choi, Kwang-Dong; Choi, Jae-Hwan; Kim, Ji-Soo; Kim, Hyo Jung; Kim, Min-Ji; Lee, Tae-Hong; Lee, Hyung; Moon, In Soo; Oh, Hui Jong; Kim, Jae-Il

2013-07-01

To elucidate the mechanisms and prognosis of rotational vertebral artery occlusion (RVAO). We analyzed clinical and radiological characteristics, patterns of induced nystagmus, and outcome in 21 patients (13 men, aged 29-77 years) with RVAO documented by dynamic cerebral angiography during an 8-year period at 3 University Hospitals in Korea. The follow-up periods ranged from 5 to 91 months (median, 37.5 months). Most patients (n=19; 90.5%) received conservative treatments. All the patients developed vertigo accompanied by tinnitus (38%), fainting (24%), or blurred vision (19%). Only 12 (57.1%) patients showed the typical pattern of RVAO during dynamic cerebral angiography, a compression of the dominant vertebral artery at the C1-2 level during contralateral head rotation. The induced nystagmus was mostly downbeat with horizontal and torsional components beating toward the compressed vertebral artery side. None of the patients with conservative treatments developed posterior circulation stroke, and 4 of them (21.1%) showed resolution of symptoms during the follow-ups. RVAO has various patterns of vertebral artery compression, and favorable long-term outcome with conservative treatments. In most patients with RVAO, the symptoms may be ascribed to asymmetrical excitation of the bilateral labyrinth induced by transient ischemia or by disinhibition from inferior cerebellar hypoperfusion. Conservative management might be considered as the first-line treatment of RVAO.

Improvement of fine motor skills in children with visual impairment: an explorative study.

PubMed

Reimer, A M; Cox, R F A; Nijhuis-Van der Sanden, M W G; Boonstra, F N

2011-01-01

In this study we analysed the potential spin-off of magnifier training on the fine-motor skills of visually impaired children. The fine-motor skills of 4- and 5-year-old visually impaired children were assessed using the manual skills test for children (6-12 years) with a visual impairment (ManuVis) and movement assessment for children (Movement ABC), before and after receiving a 12-sessions training within a 6-weeks period. The training was designed to practice the use of a stand magnifier, as part of a larger research project on low-vision aids. In this study, fifteen children trained with a magnifier; seven without. Sixteen children had nystagmus. In this group head orientation (ocular torticollis) was monitored. Results showed an age-related progress in children's fine-motor skills after the training, irrespective of magnifier condition: performance speed of the ManuVis items went from 333.4s to 273.6s on average. Accuracy in the writing tasks also increased. Finally, for the children with nystagmus, an increase of ocular torticollis was found. These results suggest a careful reconsideration of which intervention is most effective for enhancing perceptuomotor performance in visually impaired children: specific 'fine-motor' training or 'non-specific' visual-attention training with a magnifier. Copyright © 2011 Elsevier Ltd. All rights reserved.

Integrating a Motion Base into a CAVE Automatic Virtual Environment: Phase 1

DTIC Science & Technology

2001-07-01

this, a CAVE system must perform well in the following motion-related areas: visual gaze stability, simulator sickness, realism (or face validity...and performance validity. Visual Gaze Stability Visual gaze stability, the ability to maintain eye fixation on a particular target, depends upon human...reflexes such as the vestibulo-ocular reflex (VOR) and the optokinetic nystagmus (OKN). VOR is a reflex that counter-rotates the eye relative to the

[Neuritis vestibularis can be a cause of vertigo among children].

PubMed

Warner, Tine Caroc; Login, Elke; Petersen, Anita

2014-10-27

Neuritis vestibularis (NV) as a cause of vertigo is common among adults but very rare among children and is often underrecognized and underdiagnosed. Viral infection is suspected to be the most common cause and symptoms are sudden onset of vertigo, nausea, vomiting, impaired balance and horizontal nystagmus. This case report describes a three-year-old boy diagnosed with NV. To our knowledge it is the first case diagnosed in Denmark.

Bilateral optic neuritis--the only ocular finding in a case of subacute sclerosing panencephalitis.

PubMed

Ozer, Pinar Altiaylik; Ozkan, Mehpare; Sekeroglu, Hande Taylan; Kadayifcilar, Sibel; Yuksel, Deniz; Aksoy, Ayse

2014-02-01

Subacute sclerosing panencephalitis is a rare disease of central nervous system caused by defective measles virus. Chorioretinitis with macular involvement is the mostly observed ocular finding in the disease. Other reported ocular findings in the disease are cortical blindness, hemianopsia, nystagmus, extraocular muscle paresis and optic atrophy. We present a rare case of subacute sclerosing panencephalitis with isolated bilateral optic neuritis as the only ocular finding without macular involvement.

Threat effects on human oculo-motor function.

PubMed

Naranjo, E N; Cleworth, T W; Allum, J H J; Inglis, J T; Lea, J; Westerberg, B D; Carpenter, M G

2017-09-17

Neuro-anatomical evidence supports the potential for threat-related factors, such as fear, anxiety and vigilance, to influence brainstem motor nuclei controlling eye movements, as well as the vestibular nuclei. However, little is known about how threat influences human ocular responses, such as eye saccades (ES), smooth pursuit eye tracking (SP), and optokinetic nystagmus (OKN), and whether these responses can be facilitated above normal baseline levels with a natural source of threat. This study was designed to examine the effects of height-induced postural threat on the gain of ES, SP and OKN responses in humans. Twenty participants stood at two different surface heights while performing ES (ranging from 8° to 45° from center), SP (15, 20, 30°/s) and OKN (15, 30, 60°/s) responses in the horizontal plane. Height did not significantly increase the slope of the relationship between ES peak velocity and initial amplitude, or the gain of ES amplitude. In contrast height significantly increased SP and OKN gain. Significant correlations were found between changes in physiological arousal and OKN gain. Observations of changes with height in OKN and SP support neuro-anatomical evidence of threat-related mechanisms influencing both oculo-motor nuclei and vestibular reflex pathways. Although further study is warranted, the findings suggest that potential influences of fear, anxiety and arousal/alertness should be accounted for, or controlled, during clinical vestibular and oculo-motor testing. Copyright © 2017 IBRO. Published by Elsevier Ltd. All rights reserved.

Somnolence and Stuttering as the Primary Manifestations of a Midbrain Stroke

PubMed Central

Karakis, Ioannis; Ellenstein, Aviva; Roselló, Gemma R; Romero, José R

2008-01-01

Background: Stroke can occasionally manifest with non-lateralizing findings such as somnolence and stuttering. We describe a case and discuss the anatomical and physiological implications of this rare combination of symptoms. Case report: A 51-year-old woman presented with 3 days of “feeling drunk”. She could further specify her symptoms as blurry vision, slurred speech, and gait instability. She had a history of hypertension and hyperlipidemia. Her examination at presentation was remarkable only for marked somnolence. Over the next several hours she developed mild upgaze limitation and vertical nystagmus. Non-enhanced computed tomography of the brain was normal. Brain magnetic resonance imaging (MRI) revealed a 5 mm acute infarct in the caudal midbrain. The first week the patient remained somnolent and manifested marked stuttering. The patient improved gradually with speech therapy. Conclusion: Strokes affecting the diencephalic-mesencephalic junction can manifest with stuttering. Defective projections of the reticular formation to the supplementary motor area, damaged extrapyramidal circuits, and/or aberrant propioceptive feedback due to involvement of the mesencephalic nucleus of the trigeminal nerve are the proposed pathophysiological mechanisms. Somnolence can also be part of the presentation and is likely due to disruptions of sleep pathways subserved by the reticular activating system. The accurate diagnosis of these cases depends on careful clinical assessment and high index of suspicion for stroke, especially in lieu of preexisting vascular risk factors and lack of an alternative explanation such as toxic-metabolic encephalopathy. PMID:22518226

N-Acetyl-L-Leucine Accelerates Vestibular Compensation after Unilateral Labyrinthectomy by Action in the Cerebellum and Thalamus

PubMed Central

Xiong, Guoming; Potschka, Heidrun; Jahn, Klaus; Bartenstein, Peter; Brandt, Thomas; Dutia, Mayank; Dieterich, Marianne; Strupp, Michael; la Fougère, Christian; Zwergal, Andreas

2015-01-01

An acute unilateral vestibular lesion leads to a vestibular tone imbalance with nystagmus, head roll tilt and postural imbalance. These deficits gradually decrease over days to weeks due to central vestibular compensation (VC). This study investigated the effects of i.v. N-acetyl-DL-leucine, N-acetyl-L-leucine and N-acetyl-D-leucine on VC using behavioural testing and serial [18F]-Fluoro-desoxyglucose ([18F]-FDG)-μPET in a rat model of unilateral chemical labyrinthectomy (UL). Vestibular behavioural testing included measurements of nystagmus, head roll tilt and postural imbalance as well as sequential whole-brain [18F]-FDG-μPET was done before and on days 1,3,7 and 15 after UL. A significant reduction of postural imbalance scores was identified on day 7 in the N-acetyl-DL-leucine (p < 0.03) and the N-acetyl-L-leucine groups (p < 0.01), compared to the sham treatment group, but not in the N-acetyl-D-leucine group (comparison for applied dose of 24 mg i.v. per rat, equivalent to 60 mg/kg body weight, in each group). The course of postural compensation in the DL- and L-group was accelerated by about 6 days relative to controls. The effect of N-acetyl-L-leucine on postural compensation depended on the dose: in contrast to 60 mg/kg, doses of 15 mg/kg and 3.75 mg/kg had no significant effect. N-acetyl-L-leucine did not change the compensation of nystagmus or head roll tilt at any dose. Measurements of the regional cerebral glucose metabolism (rCGM) by means of μPET revealed that only N-acetyl-L-leucine but not N-acetyl-D-leucine caused a significant increase of rCGM in the vestibulocerebellum and a decrease in the posterolateral thalamus and subthalamic region on days 3 and 7. A similar pattern was found when comparing the effect of N-acetyl-L-leucine on rCGM in an UL-group and a sham UL-group without vestibular damage. In conclusion, N-acetyl-L-leucine improves compensation of postural symptoms after UL in a dose-dependent and specific manner, most likely by activating the vestibulocerebellum and deactivating the posterolateral thalamus. PMID:25803613

Experiment K-7-30: Effects of Spaceflight in the Cosmos Biosatellite 2044 on the Vestibular-Ocular Reflex (VOR) of Rhesus Monkeys

NASA Technical Reports Server (NTRS)

Cohen, B.; Cohen, N.; Helwig, D.; Solomon, D.; Kozlovskaya, I.; Sirota, M.; Yakushin, S.; Raphan, T.

1994-01-01

This technical paper discusses the following: (1) The VOR of two rhesus monkeys was studied before and after 14 days of spaceflight to determine effects of microgravity on the VOR. Horizontal, vertical and roll eye movements were recorded in these and six other monkeys implanted with scleral search coils. Animals were rotated about a vertical axis to determine the gain of the horizontal, vertical and roll VOR. They were rotated about axes tilted from the vertical (off-vertical axis rotation, OVAR) to determine steady state gains and effects of gravity on modulations in eye position and eye velocity. They were also tested for tilt dumping of post-rotatory nystagmus. (2) The gain of the horizontal VOR was close to unity when animals were tested 15 and 18 hours after flight. VOR gain values were similar to those registered before flight. If the gain of the horizontal VOR changes in microgravity, it must revert to normal soon after landing. (3) Steady state velocities of nystagmus induced by off-vertical axis rotation (OVAR) were unchanged by adaptation to microgravity, and the phase of the modulations was similar before and after flight. However, modulations in horizontal eye velocity had more variation after landing and were on mean about 50% larger for angles of tilt of the axis of rotation between 50 and 90?/s after flight. This difference was similar in both animals and was significant. (4) A striking finding was that tilt dumping was lost in the one animal tested for this function. This loss persisted for several days after return. This is reminiscent of the loss of response to pitch while rotating in the M-131 experiments of Skylab, and must be studied in detail in future spaceflights. (5) Thus, two major findings emerged from these studies: after spaceflight the modulation of horizontal eye velocity was larger during OVAR, and one animal lost its ability to tilt-dump its nystagmus. Both findings are consistent with the postulate that adaptation to microgravity causes alterations in the way that otolith information is processed in the central nervous system. The experiments lay the groundwork for studying the vertical and roll VOR before and after future space flights, as well as for studying modulations in vertical and roll eye position during OVAR and tilt dumping.

N-acetyl-L-leucine accelerates vestibular compensation after unilateral labyrinthectomy by action in the cerebellum and thalamus.

PubMed

Günther, Lisa; Beck, Roswitha; Xiong, Guoming; Potschka, Heidrun; Jahn, Klaus; Bartenstein, Peter; Brandt, Thomas; Dutia, Mayank; Dieterich, Marianne; Strupp, Michael; la Fougère, Christian; Zwergal, Andreas

2015-01-01

An acute unilateral vestibular lesion leads to a vestibular tone imbalance with nystagmus, head roll tilt and postural imbalance. These deficits gradually decrease over days to weeks due to central vestibular compensation (VC). This study investigated the effects of i.v. N-acetyl-DL-leucine, N-acetyl-L-leucine and N-acetyl-D-leucine on VC using behavioural testing and serial [18F]-Fluoro-desoxyglucose ([18F]-FDG)-μPET in a rat model of unilateral chemical labyrinthectomy (UL). Vestibular behavioural testing included measurements of nystagmus, head roll tilt and postural imbalance as well as sequential whole-brain [18F]-FDG-μPET was done before and on days 1,3,7 and 15 after UL. A significant reduction of postural imbalance scores was identified on day 7 in the N-acetyl-DL-leucine (p < 0.03) and the N-acetyl-L-leucine groups (p < 0.01), compared to the sham treatment group, but not in the N-acetyl-D-leucine group (comparison for applied dose of 24 mg i.v. per rat, equivalent to 60 mg/kg body weight, in each group). The course of postural compensation in the DL- and L-group was accelerated by about 6 days relative to controls. The effect of N-acetyl-L-leucine on postural compensation depended on the dose: in contrast to 60 mg/kg, doses of 15 mg/kg and 3.75 mg/kg had no significant effect. N-acetyl-L-leucine did not change the compensation of nystagmus or head roll tilt at any dose. Measurements of the regional cerebral glucose metabolism (rCGM) by means of μPET revealed that only N-acetyl-L-leucine but not N-acetyl-D-leucine caused a significant increase of rCGM in the vestibulocerebellum and a decrease in the posterolateral thalamus and subthalamic region on days 3 and 7. A similar pattern was found when comparing the effect of N-acetyl-L-leucine on rCGM in an UL-group and a sham UL-group without vestibular damage. In conclusion, N-acetyl-L-leucine improves compensation of postural symptoms after UL in a dose-dependent and specific manner, most likely by activating the vestibulocerebellum and deactivating the posterolateral thalamus.

The Role of Attention in Binocular Rivalry as Revealed through Optokinetic Nystagmus.

DTIC Science & Technology

1995-11-01

break down selectively when parts of the stri- ate and prestriate cortex is damaged. Speci cally, a group of patients su ering fromApperceptive Agnosia ...1981). Visual performance in cases of visual agnosia . In M. van- Ho , & G. Hohn (Eds.), Functional recovery from brain damage (pp. 275{286...macaques. Nature, 373, 609{611. Breese, B. (1899). On inhibition. Psychol.Rev., 3, 1{65. Campion, J., & Latto, R. (1985). Apperceptive agnosia due to

Spinocerebellar ataxia type 6 with positional vertigo and acetazolamide responsive episodic ataxia.

PubMed

Jen, J C; Yue, Q; Karrim, J; Nelson, S F; Baloh, R W

1998-10-01

The SCA6 mutation, a small expansion of a CAG repeat in a calcium channel gene CACNA1A, was identified in three pedigrees. Point mutations in other parts of the gene CACNA1A were excluded and new clinical features of SCA6 reported--namely, central positional nystagmus and episodic ataxia responsive to acetazolamide. The three allelic disorders, episodic ataxia type 2, familial hemiplegic migraine, and SCA6, have overlapping clinical features.

X-linked ocular albinism in Blacks. Ocular albinism cum pigmento.

PubMed

O'Donnell, F E; Green, W R; Fleischman, J A; Hambrick, G W

1978-07-01

X-linked ocular albinism can be an unsuspected cause of congenital nystagmus in blacks. In this study, eight of ten black ocular albinos from two kindreds had nonalbinotic, moderately pigmented fundi and no transillumination of the iris. We refer to this paradoxical condition as "ocular albinism cum pigmento." The only constant ophthalmoscopic feature was a foveal hypoplasia. Biopsy of clinically normal skin to demonstrate giant pigment granules is the most accurate means of diagnosis.

Update on strabismus and amblyopia.

PubMed

Campos, E C

1995-01-01

This review analyzes progress in the field of infantile strabismus and amblyopia. Only works which are of clinical interest have been reviewed. A brief survey is provided also of subjects matter of international debate, as surgery for accommodative esotropia, the prism adaptation test and the use of sectors for amblyopia treatment. Mention is made of new developments in the field which are not yet applicable to clinical practice. Finally, progress in the approach towards ocular nystagmus is considered as well.

Caffeine increases the velocity of rapid eye movements in unfatigued humans.

PubMed

Connell, Charlotte J W; Thompson, Benjamin; Turuwhenua, Jason; Hess, Robert F; Gant, Nicholas

2017-08-01

Caffeine is a widely used dietary stimulant that can reverse the effects of fatigue on cognitive, motor and oculomotor function. However, few studies have examined the effect of caffeine on the oculomotor system when homeostasis has not been disrupted by physical fatigue. This study examined the influence of a moderate dose of caffeine on oculomotor control and visual perception in participants who were not fatigued. Within a placebo-controlled crossover design, 13 healthy adults ingested caffeine (5 mg·kg -1 body mass) and were tested over 3 h. Eye movements, including saccades, smooth pursuit and optokinetic nystagmus, were measured using infrared oculography. Caffeine was associated with higher peak saccade velocities (472 ± 60° s -1 ) compared to placebo (455 ± 62° s -1 ). Quick phases of optokinetic nystagmus were also significantly faster with caffeine, whereas pursuit eye movements were unchanged. Non-oculomotor perceptual tasks (global motion and global orientation processing) were unaffected by caffeine. These results show that oculomotor control is modulated by a moderate dose of caffeine in unfatigued humans. These effects are detectable in the kinematics of rapid eye movements, whereas pursuit eye movements and visual perception are unaffected. Oculomotor functions may be sensitive to changes in central catecholamines mediated via caffeine's action as an adenosine antagonist, even when participants are not fatigued.

Differences in gaze anticipation for locomotion with and without vision

PubMed Central

Authié, Colas N.; Hilt, Pauline M.; N'Guyen, Steve; Berthoz, Alain; Bennequin, Daniel

2015-01-01

Previous experimental studies have shown a spontaneous anticipation of locomotor trajectory by the head and gaze direction during human locomotion. This anticipatory behavior could serve several functions: an optimal selection of visual information, for instance through landmarks and optic flow, as well as trajectory planning and motor control. This would imply that anticipation remains in darkness but with different characteristics. We asked 10 participants to walk along two predefined complex trajectories (limaçon and figure eight) without any cue on the trajectory to follow. Two visual conditions were used: (i) in light and (ii) in complete darkness with eyes open. The whole body kinematics were recorded by motion capture, along with the participant's right eye movements. We showed that in darkness and in light, horizontal gaze anticipates the orientation of the head which itself anticipates the trajectory direction. However, the horizontal angular anticipation decreases by a half in darkness for both gaze and head. In both visual conditions we observed an eye nystagmus with similar properties (frequency and amplitude). The main difference comes from the fact that in light, there is a shift of the orientations of the eye nystagmus and the head in the direction of the trajectory. These results suggest that a fundamental function of gaze is to represent self motion, stabilize the perception of space during locomotion, and to simulate the future trajectory, regardless of the vision condition. PMID:26106313

Training shortens search times in children with visual impairment accompanied by nystagmus.

PubMed

Huurneman, Bianca; Boonstra, F Nienke

2014-01-01

Perceptual learning (PL) can improve near visual acuity (NVA) in 4-9 year old children with visual impairment (VI). However, the mechanisms underlying improved NVA are unknown. The present study compares feature search and oculomotor measures in 4-9 year old children with VI accompanied by nystagmus (VI+nys [n = 33]) and children with normal vision (NV [n = 29]). Children in the VI+nys group were divided into three training groups: an experimental PL group, a control PL group, and a magnifier group. They were seen before (baseline) and after 6 weeks of training. Children with NV were only seen at baseline. The feature search task entailed finding a target E among distractor E's (pointing right) with element spacing varied in four steps: 0.04°, 0.5°, 1°, and 2°. At baseline, children with VI+nys showed longer search times, shorter fixation durations, and larger saccade amplitudes than children with NV. After training, all training groups showed shorter search times. Only the experimental PL group showed prolonged fixation duration after training at 0.5° and 2° spacing, p's respectively 0.033 and 0.021. Prolonged fixation duration was associated with reduced crowding and improved crowded NVA. One of the mechanisms underlying improved crowded NVA after PL in children with VI+nys seems to be prolonged fixation duration.

[Comparative assessment of results in cervical vertigo pharmacotherapy vs physiotherapy treatment].

PubMed

Olszewski, Jurek; Repetowski, Marcin; Kuśmierczyk, Krzysztof

2007-01-01

The aim of the study was to assess the effectiveness of pharmacotherapy and physiotherapy in cervical vertigo treatment. 80 patients with cervical vertigo (41 women and 39 men), aged 20 to 85, were treated by pharmacotherapy or physiotherapy. There were two groups: group I was treated by pharmacotherapy and group II was treated by physiotherapy and kinesitherapy. The effects of vertigo treatment were assessed by positional nystagmus testing according to Cawthorne and Rosen; cervical nystagmus testing in neck rotation test; by everyday task self-control cards and vertigo staging system according to Silvoniemi (0-4 points). The effects of treatment were assessed before therapy, 6 and 12 weeks after therapy. Patients from group I were treated by Nootropil and Betaserc; group II received magnetic Fidel, laserotherapy, massage and kinesitherapy exercises. The obtained results showed that the most useful methods of cervical vertigo diagnosis were neck rotation test and Rose method. The average number of points (according to Silvoniemi scale) on the basis of ten everyday activities, was lower after 6 weeks of treatment than before the treatment and the lowest after 12 weeks of treatment. The difference was higher in group II than in group I. Patients treated by physiotherapy (group II) performed better in everyday activities than the patients treated by pharmacotherapy (group I). Cervical vertigo treatment by physiotherapy is cheap and underestimated method.

Visual defects in Nepalese children with Down syndrome.

PubMed

Paudel, Nabin; Leat, Susan J; Adhikari, Prakash; Woodhouse, J Margaret; Shrestha, Jyoti Baba

2010-03-01

Down syndrome (DS) is a common chromosomal anomaly. People with this syndrome have recognisable physical characteristics and limited intellectual abilities. The aim of this study was to determine visual defects, especially refractive error and binocular anomalies, in a sample of Nepalese children with DS. Thirty-six children with DS (19 boys and 17 girls) from the Kathmandu valley, aged from four months to 18 years, underwent detailed optometric examination. Cycloplegic refraction was performed on all subjects. Vision on presentation of all the children was assessed with preferential looking cards, the Kay picture cards, the Bailey-Lovie logMAR chart or the Snellen chart. Binocular function was assessed with cover test, Hirschberg or Bruckner test. Cycloplegic refraction of the children revealed that 80 per cent of the children had significant refractive error. Most of them had hyperopia (55 per cent), followed by astigmatism (44 per cent), myopia (25 per cent) and anisometropia (19 per cent). Only two (5.6 per cent) children were strabismic and both of them were alternating esotropes. Nystagmus was present in 10 (28 per cent). Other ocular findings were upward slanting palpebral fissures, blepharitis, congenital nasolacrimal duct obstruction, blepharoconjunctivitis, chalazion and lenticular opacities. Nepalese children with DS have a high prevalence of refractive error and nystagmus. Regular eye examinations are indicated for these children to enable early diagnosis and appropriate management of ocular disorders to improve their vision and quality of life.

Infantile nystagmus syndrome is associated with inefficiency of goal-directed hand movements.

PubMed

Liebrand-Schurink, Joyce; Cox, Ralf F A; van Rens, Ger H M B; Cillessen, Antonius H N; Meulenbroek, Ruud G J; Boonstra, F Nienke

2014-12-23

The effect of infantile nystagmus syndrome (INS) on the efficiency of goal-directed hand movements was examined. We recruited 37 children with INS and 65 control subjects with normal vision, aged 4 to 8 years. Participants performed horizontally-oriented, goal-directed cylinder displacements as if they displaced a low-vision aid. The first 10 movements of 20 back-and-forth displacements in a trial were performed between two visually presented target areas, and the second 10 between remembered target locations (not visible). Motor performance was examined in terms of movement time, endpoint accuracy, and a harmonicity index reflecting energetic efficiency. Compared to the control group, the children with INS performed the cylinder displacements more slowly (using more time), less accurately (specifically in small-amplitude movements), and with less harmonic acceleration profiles. Their poor visual acuity proved to correlate with slower and less accurate movements, but did not correlate with harmonicity. When moving between remembered target locations, the performance of children with INS was less accurate than that of the children with normal vision. In both groups, movement speed and harmonicity increased with age to a similar extent. Collectively, the findings suggest that, in addition to the visuospatial homing-in problems associated with the syndrome, INS is associated with inefficiency of goal-directed hand movements. ( http://www.trialregister.nl number, NTR2380.). Copyright 2015 The Association for Research in Vision and Ophthalmology, Inc.

Inner retinal dystrophy in a patient with biallelic sequence variants in BRAT1.

PubMed

Oatts, Julius T; Duncan, Jacque L; Hoyt, Creig S; Slavotinek, Anne M; Moore, Anthony T

2017-12-01

Mutations in the BRCA1-associated protein required for the ataxia telangiectasia mutated (ATM) activation-1 (BRAT1) gene cause lethal neonatal rigidity and multifocal seizure syndrome characterized by rigidity and intractable seizures and a milder phenotype with intellectual disability, seizures, nonprogressive cerebellar ataxia or dyspraxia, and cerebellar atrophy. To date, nystagmus, cortical visual impairment, impairment of central vision, optic nerve hypoplasia, and optic atrophy have been described in this condition. This article describes the retinal findings in a patient with biallelic deleterious sequence variants in BRAT1. Case report of a child with biallelic sequence variants in the BRAT1 gene. This patient had developmental delay, microcephaly, nystagmus, and esotropia, and full-field electroretinography (ERG) revealed an inner retinal dystrophy. She was found on exome sequencing to have compound heterozygous sequence variants in the BRAT1 gene: one maternally inherited frameshift variant (c.294dupA, predicting p.Leu99Thrfs*92), which has previously been reported, and one paternally inherited novel missense variant (c.803G>A, p.Arg268His), which is likely to affect protein function. Biallelic sequence variants in BRAT1 have been reported to cause a variety of ocular and systemic manifestations, but to our knowledge, this is the first report of inner retinal dysfunction manifest as selective loss of full-field ERG scotopic and photopic b-wave amplitudes.

Ocular and perceptual responses to linear acceleration in microgravity: alterations in otolith function on the COSMOS and Neurolab flights.

PubMed

Moore, Steven T; Clément, Gilles; Dai, Mingjai; Raphan, Theodore; Solomon, David; Cohen, Bernard

2003-01-01

In this paper we review space flight experiments performed by our laboratory. Rhesus monkeys were tested before and after 12 days in orbit on COSMOS flights 2044 (1989) and 2229 (1992-1993). There was a long-lasting decrease in post-flight ocular counter-rolling (70%) and vergence (50%) during off-vertical axis rotation. In one animal, the orientation of optokinetic after-nystagmus shifted by 28 degrees from the spatial vertical towards the body vertical early post-flight. Otolith-ocular and perceptual responses were also studied in four astronauts on the 17-day Neurolab shuttle mission (STS-90) in 1998. Ocular counter-rolling was unchanged in response to 1-g and 0.5-g Gy centrifugation during and after flight and to post-flight static roll tilts relative to pre-flight values. Orientation of the optokinetic nystagmus eye velocity axis to gravito-inertial acceleration (GIA) during centrifugation was also unaltered by exposure to microgravity. Perceptual orientation to the GIA was maintained in-flight, and subjects did not report sensation of translation during constant velocity centrifugation. These studies suggest that percepts and ocular responses to tilt are determined by sensing the body vertical relative to the GIA. The findings also raise the possibility that 'artificial gravity' during the Neurolab flight counteracted adaptation of these otolith-ocular responses.

Space Biology and Aerospace Medicine, Number 5, 1977.

DTIC Science & Technology

1977-11-10

Severity of Caloric Nystagmus (V. N. Krut’ko, et al.)..,»,..,.. 94 A Study of the Pulsed Method of Laundering (B. A, Adamovich, et al,)’ .... 100...diminished motor activity. As we know, hypo - kinesia is very important as an etiological factor in onset of a number of diseases, mainly referable to...artificial gravitation; they were 8x8x28 cm in size and we put 5 mice in each. The animals were on a mixed diet , and they were fed both during

Effects of Low and High Oxygen Tensions and Related Respiratory Conditions on Visual Performance: A Literature Review

DTIC Science & Technology

1988-07-01

and of the same subject after a diet that made him deficient in Vitamin A. They used only one healthy well-trained observer. Measurementv of...background intensity. The authors do not discuss hypoxia changes in some other visual process, but this improvement above 15,000 feet may reflect hypo ...these studies, Gellhorn and Spiesman (1935) induced caloric nystagmus while subjects breathed mixtures high in CO2 and low in oxygen, and also after

Measure and Analysis of a Gaze Position Using Infrared Light Technique

DTIC Science & Technology

2001-10-25

MEASURE AND ANALYSIS OF A GAZE POSITION USING INFRARED LIGHT TECHNIQUE Z. Ramdane-Cherif1,2, A. Naït-Ali2, J F. Motsch2, M. O. Krebs1 1INSERM E 01-17...also proposes a method to correct head movements. Keywords: eye movement, gaze tracking, visual scan path, spatial mapping. INTRODUCTION The eye gaze ...tracking has been used for clinical purposes to detect illnesses, such as nystagmus , unusual eye movements and many others [1][2][3]. It is also used

Abnormalities of gaze in cerebrovascular disease.

PubMed

Pedersen, R A; Troost, B T

1981-01-01

Disorders of ocular motility may occur after injury at several levels of the neuraxis. Unilateral supranuclear disorders of gaze tend to be transient; bilateral disorders more enduring. Nuclear disorders of gaze also tend to be enduring and are frequently present in association with long tract signs and cranial nerve palsies on opposite sides of the body. Nystagmus is a reliable sign of posterior fossa or peripheral eight nerve pathology. Familiarity with these concepts may help the clinician answer questions regarding localization and prognosis.

Systems analysis of the vestibulo-ocular system. [mathematical model of vestibularly driven head and eye movements

NASA Technical Reports Server (NTRS)

Schmid, R. M.

1973-01-01

The vestibulo-ocular system is examined from the standpoint of system theory. The evolution of a mathematical model of the vestibulo-ocular system in an attempt to match more and more experimental data is followed step by step. The final model explains many characteristics of the eye movement in vestibularly induced nystagmus. The analysis of the dynamic behavior of the model at the different stages of its development is illustrated in time domain, mainly in a qualitative way.

A versatile stereoscopic visual display system for vestibular and oculomotor research.

PubMed

Kramer, P D; Roberts, D C; Shelhamer, M; Zee, D S

1998-01-01

Testing of the vestibular system requires a vestibular stimulus (motion) and/or a visual stimulus. We have developed a versatile, low cost, stereoscopic visual display system, using "virtual reality" (VR) technology. The display system can produce images for each eye that correspond to targets at any virtual distance relative to the subject, and so require the appropriate ocular vergence. We elicited smooth pursuit, "stare" optokinetic nystagmus (OKN) and after-nystagmus (OKAN), vergence for targets at various distances, and short-term adaptation of the vestibulo-ocular reflex (VOR), using both conventional methods and the stereoscopic display. Pursuit, OKN, and OKAN were comparable with both methods. When used with a vestibular stimulus, VR induced appropriate adaptive changes of the phase and gain of the angular VOR. In addition, using the VR display system and a human linear acceleration sled, we adapted the phase of the linear VOR. The VR-based stimulus system not only offers an alternative to more cumbersome means of stimulating the visual system in vestibular experiments, it also can produce visual stimuli that would otherwise be impractical or impossible. Our techniques provide images without the latencies encountered in most VR systems. Its inherent versatility allows it to be useful in several different types of experiments, and because it is software driven it can be quickly adapted to provide a new stimulus. These two factors allow VR to provide considerable savings in time and money, as well as flexibility in developing experimental paradigms.

Clinical and ocular motor analysis of the infantile nystagmus syndrome in the first 6 months of life.

PubMed

Hertle, R W; Maldanado, V K; Maybodi, M; Yang, D

2002-06-01

The infantile nystagmus syndrome (INS) usually begins in infancy and may or may not be associated with visual sensory system abnormalities. Little is known about its specific waveforms in the first 6 months of life or their relation to the developing visual system. This study identifies the clinical and ocular motility characteristics of the INS and establishes the range of waveforms present in the first 6 months of life. 27 infants with involuntary ocular oscillations typical of INS are included in this analysis. They were evaluated both clinically and with motility recordings. Eye movement analysis was performed off line from computer analysis of digitised data. Variables analysed included age, sex, vision, ocular abnormalities, head position, and null zone, neutral zone characteristics, symmetry, conjugacy, waveforms, frequencies, and foveation times. Ages ranged from 3 to 6.5 months (average 4.9 months). 15 patients (56%) had abnormal vision for age, nine (33%) had strabismus, five (19%) had an anomalous head posture, 13 (48%) had oculographic null and neutral positions, nine (33%) had binocular asymmetry, and only two showed consistent dysconjugacy. Average binocular frequency was 3.3 Hz, monocular frequency 6.6 Hz. Average foveation periods were longer and more "jerk" wave forms were observed in those patients with normal vision. Common clinical characteristics and eye movement waveforms of INS begin in the first few months of infancy and waveform analysis at this time may help with both diagnosis and visual status.

Vestibulo-cortical Hemispheric Dominance: the link between Anxiety and the Vestibular System?

PubMed

Bednarczuk, Nadja F; Casanovas Ortega, Marta; Fluri, Anne-Sophie; Arshad, Qadeer

2018-05-16

Vestibular processing and anxiety networks are functionally intertwined, as demonstrated by reports of reciprocal influences upon each other. Yet whether there is an underlying link between these two systems remains unknown Previous findings have highlighted the involvement of hemispheric lateralisation in processing of both anxiety and vestibular signals. Accordingly, we explored the interaction between vestibular cortical processing and anxiety by assessing the relationship between anxiety levels and the degree of hemispheric lateralisation of vestibulo-cortical processing in 64 right-handed, healthy individuals. Vestibulo-cortical hemispheric lateralisation was determined by gaging the degree of caloric-induced nystagmus suppression following modulation of cortical excitability using trans-cranial direct current stimulation targeted over the posterior parietal cortex, an area implicated in the processing of vestibular signals. The degree of nystagmus suppression yields an objective biomarker, allowing the quantification of the degree of right vestibulo-cortical hemisphere dominance. Anxiety levels were quantified using the Trait component of the Spielberger State-Trait Anxiety Questionnaire. Our findings demonstrate that the degree of an individual's vestibulo-cortical hemispheric dominance correlates with their anxiety levels. That is, those individuals with greater right hemispheric vestibulo-cortical dominance exhibited lower levels of anxiety. By extension, our results support the notion that hemispheric lateralisation determines an individual's emotional processing, thereby linking cortical circuits involved in processing anxiety and vestibular signals respectively. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

A Reevaluation of the Vestibulo-Ocular Reflex: New Ideas of its Purpose, Properties, Neural Substrate, and Disorders

NASA Technical Reports Server (NTRS)

Leigh, R. John; Brandt, Thomas

1992-01-01

Conventional views of the Vestibulo-Ocular Reflex (VOR) have emphasized testing with caloric stimuli and by passively rotating patients at low frequencies in a chair. The properties of the VOR tested under these conditions differ from the performance of this reflex during the natural function for which it evolved-locomotion. Only the VOR (and not visually mediated eye movements) can cope with the high-frequency angular and linear perturbations of the head that occur during locomotion; this is achieved by generating eye movements at short latency (less than 16 msec). Interpretation of vestibular testing is enhanced by the realization that, although the di- and trisynaptic components of the VOR are essential for this short-latency response, the overall accuracy and plasticity of the VOR depend upon a distributed, parallel network of neurons involving the vestibular nuclei. Neurons in this network variously encode inputs from the labyrinthine semicircular canals and otoliths, as well as from the visual and somatosensory systems. The central vestibular pathways branch to contact vestibular cortex (for perception) and the spinal cord (for control of posture). Thus, the vestibular nuclei basically coordinate the stabilization of gaze and posture, and contribute to the perception of verticality and self-motion. Consequently, brainstem disorders that disrupt the VOR cause not just only nystagmus, but also instability of posture (eg, increased fore-aft sway in patients with downbeat nystagmus) and disturbance of spatial orientation (eg, tilt of the subjective visual vertical in Wallenberg's syndrome).

Effect of microgravity on spatial orientation and posture regulation during Coriolis stimulation.

PubMed

Takahashi, Masahiro; Sekine, Motoki; Ikeda, Takuo; Watanuki, Koichi; Hakuta, Shuzo; Takeoka, Hajime

2004-05-01

To elucidate spatial orientation and posture regulation under conditions of microgravity. Coriolis stimulation was done with five normal subjects on the ground (1 g) and onboard an aircraft (under conditions of microgravity during parabolic flight). Subjects were asked to tilt their heads forward during rotation at speeds of 0, 50, 100 and 150 degrees/s on the ground and 100 degrees/s during flight. Body sway was recorded using a 3D linear accelerometer and eye movements using an infrared charge-coupled device video camera. Flight experiments were performed on 5 consecutive days, and 11-16 parabolic maneuvers were done during each flight. Two subjects boarded each flight and were examined alternately at least five times. Coriolis stimulation at 1 g caused body sway, nystagmus and a movement sensation in accordance with inertial inputs at 1 g. Neither body sway, excepting a minute sway due to the Coriolis force, nor a movement sensation occurred in microgravity, but nystagmus was recorded. Posture, eye movement and sensation at 1 g are controlled with reference to spatial coordinates that represent the external world in the brain. Normal spatial coordinates are not relevant in microgravity because there is no Z-axis, and the posture regulation and sensation that depend on them collapse. The discrepancy in responses between posture and eye movement under conditions of microgravity may be caused by a different constitution of the effectors which adjust posture and gaze.

Investigations of the pathogenesis of acquired pendular nystagmus

NASA Technical Reports Server (NTRS)

Averbuch-Heller, L.; Zivotofsky, A. Z.; Das, V. E.; DiScenna, A. O.; Leigh, R. J.

1995-01-01

We investigated the pathogenesis of acquired pendular nystagmus (APN) in six patients, three of whom had multiple sclerosis. First, we tested the hypothesis that the oscillations of APN are due to a delay in visual feedback secondary, for example, to demyelination of the optic nerves. We manipulated the latency to onset of visually guided eye movements using an electronic technique that induces sinusoidal oscillations in normal subjects. This manipulation did not change the characteristics of the APN, but did superimpose lower-frequency oscillations similar to those induced in normal subjects. These results are consistent with current models for smooth (non-saccadic) eye movements, which predict that prolongation of visual feedback could not account for the high-frequency oscillations that often characterize APN. Secondly, we attempted to determine whether an increase in the gain of the visually-enhanced vestibulo-ocular reflex (VOR), produced by viewing a near target, was accompanied by a commensurate increase in the amplitude of APN. Increases in horizontal or vertical VOR gain during near viewing occurred in four patients, but only two of them showed a parallel increase in APN amplitude. On the other hand, APN amplitude decreased during viewing of the near target in the two patients who showed no change in VOR gain. Taken together, these data suggest that neither delayed visual feedback nor a disorder of central vestibular mechanisms is primarily responsible for APN. More likely, these ocular oscillations are produced by abnormalities of internal feedback circuits, such as the reciprocal connections between brainstem nuclei and cerebellum.

A novel truncation mutation in CRYBB1 associated with autosomal dominant congenital cataract with nystagmus.

PubMed

Rao, Yan; Dong, Sufang; Li, Zuhua; Yang, Guohua; Peng, Chunyan; Yan, Ming; Zheng, Fang

2017-01-01

To identify the potential candidate genes for a large Chinese family with autosomal dominant congenital cataract (ADCC) and nystagmus, and investigate the possible molecular mechanism underlying the role of the candidate genes in cataractogenesis. We combined the linkage analysis and direct sequencing for the candidate genes in the linkage regions to identify the causative mutation. The molecular and bio-functional properties of the proteins encoded by the candidate genes was further explored with biophysical and biochemical studies of the recombinant wild-type and mutant proteins. We identified a c. C749T (p.Q227X) transversion in exon 6 of CRYBB1 , a cataract-causative gene. This nonsense mutation changes a phylogenetically conserved glutamine to a stop codon and is predicted to truncate the C-terminus of the wild-type protein by 26 amino acids. Comparison of the biophysical and biochemical properties of the recombinant full-length and truncated βB1-crystallins revealed that the mutation led to the insolubility and the phase separation phenomenon of the truncated protein with a changed conformation. Meanwhile, the thermal stability of the truncated βB1-crystallin was significantly decreased, and the mutation diminished the chaperoning ability of αA-crystallin with the mutant under heating stress. Our findings highlight the importance of the C-terminus in βB1-crystallin in maintaining the crystalline function and stability, and provide a novel insight into the molecular mechanism underlying the pathogenesis of human autosomal dominant congenital cataract.

Effects of static orientation upon human optokinetic afternystagmus

NASA Technical Reports Server (NTRS)

Wall, C. 3rd; Merfeld, D. M.; Zupan, L.

1999-01-01

"Normal" human subjects were placed in a series of 5 static orientations with respect to gravity and were asked to view an optokinetic display moving at a constant angular velocity. The axis of rotation coincided with the subject's rostro-caudal axis and produced horizontal optokinetic nystagmus and afternystagmus. Wall (1) previously reported that these optokinetic afternystagmus responses were not well characterized by parametric fits to slow component velocity. The response for nose-up, however, was larger than for nose-down. This suggested that the horizontal eye movements measured during optokinetic stimulation might include an induced linear VOR component as presented in the body of this paper. To investigate this hypothesis, another analysis of these data has been made using cumulative slow component eye position. Some subjects' responses had reversals in afternystagmus direction. These reversals were "filled in" by a zero slow component velocity. This method of analysis gives a much more consistent result across subjects and shows that, on average, responses from the nose-down horizontal (prone) orientation are greatly reduced (p < 0.05) compared to other horizontal and vertical orientations. Average responses are compared to responses predicted by a model previously used to predict successfully the responses to post-rotatory nystagmus after earth horizontal axis rotation. Ten of 11 subjects had larger responses in their supine than their prone orientation. Application of horizontal axis optokinetic afternystagmus for clinical otolith function testing, and implications for altered gravity experiments are discussed.

[Vertigo and dizziness in the emergency room].

PubMed

Zwergal, A; Möhwald, K; Dieterich, M

2017-06-01

Vertigo and dizziness are among the most common chief complaints in the emergency department. Etiologies can be categorized into three subgroups: neurootological (vestibular), medical (especially cardiovascular, metabolic), and psychiatric disorders. The diagnostic approach in the emergency department is based on a systematic analysis of case history (type, time course of symptoms, modulating factors, associated symptoms), clinical examination of the vestibular, ocular motor, and cerebellar systems (head impulse test, nystagmus, skew deviation, positioning maneuver, test of gait and stance), as well as a basal monitoring (vital signs, 12-lead ECG, blood tests). For differentiation of peripheral and central etiologies in acute vestibular syndrome, the HINTS exam (head impulse test, nystagmus, test of skew) and examination of smooth pursuit and saccades should be applied. Nonselective use of neuroimaging is not indicated due to a low diagnostic yield. Cranial imaging should be done in the following constellations: (1) detection of focal neurological or central ocular motor and vestibular signs on clinical exam, (2) acute abasia with only minor ocular motor signs, (3) presence of various cardiovascular risk factors, (4) headache of unknown quality as an accompanying symptom. Besides the symptomatic therapy of vertigo and dizziness with antiemetics or analgesics, further diagnostic differentiation is urgent to guide proper treatment. Examples are the acute therapy in cerebral ischemia, the execution of positioning maneuvers in benign paroxysmal positional vertigo, the use of corticosteroids in acute unilateral vestibulopathy, as well as the readjustment of metabolic homeostasis in medical disorders.

Computations underlying the visuomotor transformation for smooth pursuit eye movements

PubMed Central

Murdison, T. Scott; Leclercq, Guillaume; Lefèvre, Philippe

2014-01-01

Smooth pursuit eye movements are driven by retinal motion and enable us to view moving targets with high acuity. Complicating the generation of these movements is the fact that different eye and head rotations can produce different retinal stimuli but giving rise to identical smooth pursuit trajectories. However, because our eyes accurately pursue targets regardless of eye and head orientation (Blohm G, Lefèvre P. J Neurophysiol 104: 2103–2115, 2010), the brain must somehow take these signals into account. To learn about the neural mechanisms potentially underlying this visual-to-motor transformation, we trained a physiologically inspired neural network model to combine two-dimensional (2D) retinal motion signals with three-dimensional (3D) eye and head orientation and velocity signals to generate a spatially correct 3D pursuit command. We then simulated conditions of 1) head roll-induced ocular counterroll, 2) oblique gaze-induced retinal rotations, 3) eccentric gazes (invoking the half-angle rule), and 4) optokinetic nystagmus to investigate how units in the intermediate layers of the network accounted for different 3D constraints. Simultaneously, we simulated electrophysiological recordings (visual and motor tunings) and microstimulation experiments to quantify the reference frames of signals at each processing stage. We found a gradual retinal-to-intermediate-to-spatial feedforward transformation through the hidden layers. Our model is the first to describe the general 3D transformation for smooth pursuit mediated by eye- and head-dependent gain modulation. Based on several testable experimental predictions, our model provides a mechanism by which the brain could perform the 3D visuomotor transformation for smooth pursuit. PMID:25475344

Horizontal vestibuloocular reflex evoked by high-acceleration rotations in the squirrel monkey. III. Responses after labyrinthectomy

NASA Technical Reports Server (NTRS)

Lasker, D. M.; Hullar, T. E.; Minor, L. B.; Shelhamer, M. J. (Principal Investigator)

2000-01-01

The horizontal angular vestibuloocular reflex (VOR) evoked by high-frequency, high-acceleration rotations was studied in four squirrel monkeys after unilateral labyrinthectomy. Spontaneous nystagmus was measured at the beginning and end of each testing session. During the period that animals were kept in darkness (4 days), the nystagmus at each of these times measured approximately 20 degrees /s. Within 18-24 h after return to the light, the nystagmus (measured in darkness) decreased to 2.8 +/- 1.5 degrees /s (mean +/- SD) when recorded at the beginning but was 20.3 +/- 3.9 degrees /s at the end of the testing session. The latency of the VOR measured from responses to steps of acceleration (3,000 degrees /s(2) reaching a velocity of 150 degrees /s) was 8.4 +/- 0.3 ms for responses to ipsilesional rotations and 7.7 +/- 0.4 ms for contralesional rotations. During the period that animals were kept in darkness after the labyrinthectomy, the gain of the VOR measured during the steps of acceleration was 0.67 +/- 0.12 for contralesional rotations and 0.39 +/- 0.04 for ipsilesional rotations. Within 18-24 h after return to light, the VOR gain for contralesional rotations increased to 0.87 +/- 0.08, whereas there was only a slight increase for ipsilesional rotations to 0.41 +/- 0. 06. A symmetrical increase in the gain measured at the plateau of head velocity was noted after the animals were returned to light. The VOR evoked by sinusoidal rotations of 2-15 Hz, +/-20 degrees /s, showed a better recovery of gain at lower (2-4 Hz) than at higher (6-15 Hz) frequencies. At 0.5 Hz, gain decreased symmetrically when the peak amplitude was increased from 20 to 100 degrees /s. At 10 Hz, gain was decreased for ipsilesional half-cycles and increased for contralesional half-cycles when velocity was raised from 20 to 50 degrees /s. A model incorporating linear and nonlinear pathways was used to simulate the data. Selective increases in the gain for the linear pathway accounted for the recovery in VOR gain for responses at the velocity plateau of the steps of acceleration and for the sinusoidal rotations at lower peak velocities. The increase in gain for contralesional responses to steps of acceleration and sinusoidal rotations at higher frequencies and velocities was due to an increase in the contribution of the nonlinear pathway. This pathway was driven into cutoff and therefore did not affect responses for rotations toward the lesioned side.

Neural basis for eye velocity generation in the vestibular nuclei of alert monkeys during off-vertical axis rotation

NASA Technical Reports Server (NTRS)

Reisine, H.; Raphan, T.; Cohen, B. (Principal Investigator)

1992-01-01

Activity of "vestibular only" (VO) and "vestibular plus saccade" (VPS) units was recorded in the rostral part of the medial vestibular nucleus and caudal part of the superior vestibular nucleus of alert rhesus monkeys. By estimating the "null axes" of recorded units (n = 79), the optimal plane of activation was approximately the mean plane of reciprocal semicircular canals, i.e., lateral canals, left anterior-right posterior (LARP) canals or right anterior-left posterior (RALP) canals. All units were excited by rotation in a direction that excited a corresponding ipsilateral semicircular canal. Thus, they all displayed a "type I" response. With the animal upright, there were rapid changes in firing rates of both VO and VPS units in response to steps of angular velocity about a vertical axis. The units were bidirectionally activated during vestibular nystagmus (VN), horizontal optokinetic nystagmus (OKN), optokinetic after-nystagmus (OKAN) and off-vertical axis rotation (OVAR). The rising and falling time constants of the responses to rotation indicated that they were closely linked to velocity storage. There were differences between VPS and VO neurons in that activity of VO units followed the expected time course in response to a stimulus even during periods of drowsiness, when eye velocity was reduced. Firing rates of VPS units, on the other hand, were significantly reduced in the drowsy state. Lateral canal-related units had average firing rates that were linearly related to the bias or steady state level of horizontal eye velocity during OVAR over a range of +/- 60 deg/s. These units could be further divided into two classes according to whether they were modulated during OVAR. Non-modulated units (n = 5) were VO types and all modulated units (n = 5) were VPS types. There was no significant difference between the bias level sensitivities relative to eye velocity of the units with and without modulation (P > 0.05). The modulated units had no sustained change in firing rate in response to static head tilts and their phases relative to head position varied from unit to unit. The phase did not appear to be linked to the modulation of horizontal eye velocity during OVAR. The sensitivities of unit activity to eye velocity were similar during all stimulus modalities despite the different gains of eye velocity vs stimulus velocity during VN, OKN and OVAR. Therefore, VO and VPS units are likely to carry an eye velocity signal related to velocity storage.(ABSTRACT TRUNCATED AT 400 WORDS).

Influence of mobility restriction on habituation of the vestibular apparatus

NASA Technical Reports Server (NTRS)

Gorgiladze, G. I.; Kazanskaya, G. S.

1980-01-01

Test results presented indicate that 30-day hypokinesia did not affect the intensity of nystagmus: velocity of slow phase, total number of jerks, and duration of the reaction in animals were the same as before mobility restriction and did not differ from those of the control group. However, hypokinesia resulted in the disappearance of habituation of the vestibulary system to repeated angular accelerations. The known hypokinetic changes in the endocrine system were studied. It was concluded that reduction in adrenergic function may be the cause of disappearance of vestibular apparatus habituation during hypokinesia.

Leopard spot retinal pigmentation in infancy indicating a peroxisomal disorder.

PubMed

Lyons, C J; Castano, G; McCormick, A Q; Applegarth, D

2004-02-01

Neonatal adrenoleucodystrophy (NALD) is a rare disorder resulting from abnormal peroxisomal biogenesis. Affected patients present in infancy with developmental delay, hypotonia, and seizures. Blindness and nystagmus are prominent features. The authors suggest a characteristic leopard spot pigmentary pattern in the peripheral retina to be diagnostic. Three patients are reported with this presentation; the characteristic retinal appearance resulted in early diagnosis for one of these. Leopard spot retinopathy in an infant with hypotonia, seizures, developmental delay, with or without dysmorphic features and hearing impairment, is a clue to the diagnosis of NALD.

Engineering Applications of Bird Flight

DTIC Science & Technology

2009-04-27

Onboard
 video
 sequence
 of
 head
 nystagmus 
 during
 a
 banked
 turn.
 Note
 the
 fast
 saccadic
head
movement
in
the
first
two
frames,
which
is
followed
by
a
period
of
 gaze 
fixation
in
 the
remaining
frames.

 
 ...bird
such
as
the
Steppe
Eagle,
however,
the
eyes
have
relatively
little
freedom
of
 movement
and
large
 gaze 
shifts
are
therefore
effected
by
movements
of...voluntary
turns.
This
 differs
 from
 the
 pattern
 observed
 during
 gaze 
 stabilization
 in
 response
 to
 imposed
involuntary
turns,
in
which
the

Albinism: Particular Attention to the Ocular Motor System

PubMed Central

Hertle, Richard W.

2013-01-01

The purpose of this report is to summarize an understanding of the ocular motor system in patients with albinism. Other than the association of vertical eccentric gaze null positions and asymmetric, (a) periodic alternating nystagmus in a large percentage of patients, the ocular motor system in human albinism does not contain unique pathology, rather has “typical” types of infantile ocular oscillations and binocular disorders. Both the ocular motor and afferent visual system are affected to varying degrees in patients with albinism, thus, combined treatment of both systems will maximize visual function. PMID:24014991

Neuro-ophthalmology of type 1 Chiari malformation

PubMed Central

Shaikh, Aasef G.; Ghasia, Fatema F.

2016-01-01

Chiari malformation is a congenital deformity leading to herniation of cerebellar tonsils. Headache is a typical symptom of this condition, but patients with Chiari malformation often present with double vision and vertigo. Examination of eye movements in such patients often reveals nystagmus and strabismus. Eye movement deficits in the context of typical symptomatic presentation are critical clinical markers for the diagnosis of Chiari malformation. We will review eye movement deficits that seen in patients with type 1 Chiari malformation. We will then discuss the underlying pathophysiology and therapeutic options for such deficits. PMID:26843890

Glucocorticoids improve acute dizziness symptoms following acute unilateral vestibulopathy.

PubMed

Batuecas-Caletrío, Angel; Yañez-Gonzalez, Raquel; Sanchez-Blanco, Carmen; Pérez, Pedro Blanco; González-Sanchez, Enrique; Sanchez, Luis Alberto Guardado; Kaski, Diego

2015-11-01

Acute unilateral vestibulopathy (AUV) is characterized by acute vertigo, nausea, and imbalance without neurological deficits or auditory symptomatology. Here, we explore the effect of glucocorticoid treatment on the degree of canal paresis in patients with AUV, and critically, establish its relationship with dizziness symptom recovery. We recruited consecutive patients who were retrospectively assigned to one of the two groups according to whether they received glucocorticoid treatment (n = 32) or not (n = 44). All patients underwent pure-tone audiometry, bithermal caloric testing, MRI brain imaging, and were asked to complete a dizziness handicap inventory on admission to hospital and just prior to hospital discharge. In the treatment group, the canal paresis at discharge was significantly lower than in the control group (mean ± SD % 38.04 ± 21.57 versus 82.79 ± 21.51, p < 0.001). We also observed a significant reduction in the intensity of nystagmus in patients receiving glucocorticoid treatment compared to the non-treatment group (p = 0.03). DHI test score was significantly lower at discharge in the treatment group (mean ± SD % 23.15 ± 12.40 versus 64.07 ± 12.87, p < 0.001), as was the length of hospital stay (2.18 ± 1.5 days versus 3.6 ± 1.7 days, p = 0.002). Glucocorticoid treatment leads to acute symptomatic improvement, with a reduced hospital stay and reduction in the intensity of acute nystagmus. Our findings suggest that glucocorticoids may accelerate vestibular compensation via a restoration of peripheral vestibular function, and therefore has important clinical implications for the treatment of AUV.

Directional abnormalities of vestibular and optokinetic responses in cerebellar disease

NASA Technical Reports Server (NTRS)

Walker, M. F.; Zee, D. S.; Shelhamer, M. J. (Principal Investigator)

1999-01-01

Directional abnormalities of vestibular and optokinetic responses in patients with cerebellar degeneration are reported. Three-axis magnetic search-coil recordings of the eye and head were performed in eight cerebellar patients. Among these patients, examples of directional cross-coupling were found during (1) high-frequency, high-acceleration head thrusts; (2) constant-velocity chair rotations with the head fixed; (3) constant-velocity optokinetic stimulation; and (4) following repetitive head shaking. Cross-coupling during horizontal head thrusts consisted of an inappropriate upward eye-velocity component. In some patients, sustained constant-velocity yaw-axis chair rotations produced a mixed horizontal-torsional nystagmus and/or an increase in the baseline vertical slow-phase velocity. Following horizontal head shaking, some patients showed an increase in the slow-phase velocity of their downbeat nystagmus. These various forms of cross-coupling did not necessarily occur to the same degree in a given patient; this suggests that different mechanisms may be responsible. It is suggested that cross-coupling during head thrusts may reflect a loss of calibration of brainstem connections involved in the direct vestibular pathways, perhaps due to dysfunction of the flocculus. Cross-coupling during constant-velocity rotations and following head shaking may result from a misorientation of the angular eye-velocity vector in the velocity-storage system. Finally, responses to horizontal optokinetic stimulation included an inappropriate torsional component in some patients. This suggests that the underlying organization of horizontal optokinetic tracking is in labyrinthine coordinates. The findings are also consistent with prior animal-lesion studies that have shown a role for the vestibulocerebellum in the control of the direction of the VOR.

A reevaluation of the vestibulo-ocular reflex: new ideas of its purpose, properties, neural substrate, and disorders

NASA Technical Reports Server (NTRS)

Leigh, R. J.; Brandt, T.

1993-01-01

Conventional views of the vestibulo-ocular reflex (VOR) have emphasized testing with caloric stimuli and by passively rotating patients at low frequencies in a chair. The properties of the VOR tested under these conditions differ from the performance of this reflex during the natural function for which it evolved--locomotion. Only the VOR (and not visually mediated eye movements) can cope with the high-frequency angular and linear perturbations of the head that occur during locomotion; this is achieved by generating eye movements at short latency (< 16 msec). Interpretation of vestibular testing is enhanced by the realization that, although the di- and trisynaptic components of the VOR are essential for this short-latency response, the overall accuracy and plasticity of the VOR depend upon a distributed, parallel network of neurons involving the vestibular nuclei. Neurons in this network variously upon a distributed, parallel network of neurons involving the vestibular nuclei. Neurons in this network variously encode inputs from the labyrinthine semicircular canals and otoliths, as well as from the visual and somatosensory systems. The central vestibular pathways branch to contact vestibular cortex (for perception) and the spinal cord (for control of posture). Thus, the vestibular nuclei basically coordinate the stabilization of gaze and posture, and contribute to the perception of verticality and self-motion. Consequently, brainstem disorders that disrupt the VOR cause not just only nystagmus, but also instability of posture (eg, increased fore-aft sway in patients with downbeat nystagmus) and disturbance of spatial orientation (eg, tilt of the subjective visual vertical in Wallenberg's syndrome).

Correlation between bithermal caloric test results and vestibular evoked myogenic potentials (VEMPs) in normal subjects.

PubMed

Andrade, Isabel Vaamonde Sanchez; Santos-Perez, Sofia; Diz, Pilar Gayoso; Caballero, Torcuato Labella; Soto-Varela, Andrés

2013-05-01

Bithermal caloric testing and vestibular evoked myogenic potentials (VEMPs) are both diagnostic tools for the study of the vestibular system. The first tests the horizontal semicircular canal and the second evaluates the saccule and lower vestibular nerve. The results of these two tests can therefore be expected to be correlated. The aim of this study was to compare bithermal caloric test results with VEMP records in normal subjects to verify whether they are correlated. A prospective study was conducted in 60 healthy subjects (30 men and 30 women) who underwent otoscopy, pure tone audiometry, bithermal caloric testing and VEMPs. From the caloric test, we assessed the presence of possible vestibular hypofunction, whether there was directional preponderance and reflectivity of each ear (all based on both slow phase velocity and nystagmus frequency). The analysed VEMPs variables were: p1 and n1 latency, corrected amplitude, interaural p1 latency difference and p1 interaural amplitude asymmetry. We compared the reflectivity, hypofunction and directional preponderance of the caloric tests with the corrected amplitudes and amplitude asymmetries of the VEMPs. No correlations were found in the different comparisons between bithermal caloric testing results and VEMPs except for a weak correlation (p = 0.039) when comparing preponderance based on the number of nystagmus in the caloric test and amplitude asymmetry with 99 dB tone burst in the VEMPs test. The results indicate that the two diagnostic tests are not comparable, so one of them cannot replace the other, but the use of both increases diagnostic success in some conditions.

[Septo-optic dysplasia].

PubMed

Martínez-Sánchez, L; Arce, A; Caritg-Bosch, J; Campistol, J; Pavía, C; Gean-Molins, E

Septo optic syndrome, described by De Morsier in 1956, consists in the hypoplasia of one or both optic nerves, mid line brain malformations and hypothalamohypophysial dysfunction, which is inconstant. It is an infrequent, but treatable, cause of hepatic and neurological damage, and it is important to obtain an early diagnosis and to begin hormone replacement therapy. We report the clinical case of a female baby who was diagnosed early on as suffering from septo?optic dysplasia, after discovery of the existence of cholestatic jaundice. In our case the three components of the syndrome were present: hypothalamohypophysial dysfunction, bilateral hypoplasia of the optic nerves and brain malformations with dysplasia of the transparent septum. All this gives rise to complex clinical features and the predominance of hypernatraemic dehydration secondary to insipid diabetes, nystagmus and serious psychomotor retardation. Our patient died, as in other cases reported in the literature, from an episode of sudden death. Despite the importance of an early diagnosis of this disorder, it is usually late. Most children who present hypopituitarism traits in the neonatal period are not diagnosed at that time, with the subsequent risk of death or brain damage. Some clinical findings, which appear early on and can provide clues which aid us to reach a diagnosis, are the appearance of episodes of hypoglycaemia in the neonatal period, the existence of micropenis and cryptorchidism with hypoplasic testes, jaundice or the appearance of clinical manifestations of insipid diabetes. Later on nystagmus and neurological symptoms may appear. The final diagnosis is performed through the use of neuroimaging techniques (CT or MRI) and hormonal studies.

Apogeotropic variant of lateral semicircular canal benign paroxysmal positional vertigo: is there a correlation between clinical findings, underlying pathophysiologic mechanisms and the effectiveness of repositioning maneuvers?

PubMed

Riga, Maria; Korres, Stavros; Korres, George; Danielides, Vasilios

2013-08-01

The apogeotropic variant of horizontal semicircular canal (h-SCC) benign paroxysmal positional vertigo (BPPV) is attributed to canalithiasis of the anterior arm or cupulolithiasis. This study is an attempt to distinguish the most effective maneuvers for each case, by investigating any correlation, between the clinical findings or the treatment options and the possible location of the displaced debris. A review of the literature (1990-2012) was conducted via the PubMed database with the search terms "apogeotropic nystagmus and benign paroxysmal positional vertigo." Articles on central nervous system lesions were excluded. The studies included in the analysis provided detailed diagnostic and therapeutic protocols, supported by the resolution of the signs and symptoms through repositioning maneuvers. Descriptive statistics were used to summarize the findings. Intergroup and intragroup comparisons were performed through Pearson's χ or Fischer's exact test. Protocols vary considerably among studies. Nystagmus from seated to supine position is the best studied secondary clinical sign and possibly a clinical indication of cupulolithiasis. In patients with symmetrical responses in the head yaw test, no significant differences can be detected in the occurrence of secondary signs of lateralization compared to patients with asymmetrical responses. The Gufoni maneuver seems to be effective in all pathophysiologic types of apogeotropic h-SCC BPPV. The Barbeque and Vannucchi-Asprella maneuvers mainly target at lithiasis of the anterior ampullary arm. The results of this analysis may imply that different clinical subgroups of h-SCC BPPV may regard to different pathophysiologic and therapeutical mechanisms.

Vibration-induced nystagmus in patients with vestibular schwannoma: Characteristics and clinical implications.

PubMed

Lee, Jeon Mi; Kim, Mi Joo; Kim, Jin Won; Shim, Dae Bo; Kim, Jinna; Kim, Sung Huhn

2017-07-01

To investigate the clinical significance of vibration-induced nystagmus (VIN) in unilateral vestibular asymmetry and vestibular schwannoma. Thirteen patients with vestibular schwannoma underwent the VIN test, in which stimulation was applied to the mastoid processes and sternocleidomastoid (SCM) muscles on the ipsilateral and contralateral sides of lesions. Preoperative VIN was measured, and changes in VIN were followed up for 6months after tumor removal. Significance of VIN was determined by evaluation of its sensitivity, correlation with vestibular function tests and tumor volume, and postoperative changes. The overall pre and postoperative sensitivities of VIN were 92.3% and 100%, respectively, considering stimulation at all four sites. Maximum slow-phase velocity (MSPV) of VIN was linearly correlated with caloric weakness and tumor volume, especially when stimulation was applied to the SCM muscle. Postoperative MSPV of VIN exhibited stronger linear correlation with postoperative changes in canal paresis value and inverse correlation with tumor size upon stimulation of the ipsilateral SCM muscle than upon stimulation of other sites. During the 6-month follow-up period, persistence of VIN without changes in MSPV was observed even after vestibular compensation. Evoking VIN by stimulation of the mastoid processes and SCM muscles is effective for detecting vestibular asymmetry. It could also help determine the degree of vestibular asymmetry and volume of vestibular schwannoma if stimulation is applied to the SCM muscle. The results of this study could provide clues for the basic application of VIN in patients with vestibular loss and vestibular schwannoma. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

Eye hyperdeviation in mouse cerebellar mutants is comparable to the gravity-dependent component of human downbeat nystagmus.

PubMed

Stahl, John S; Oommen, Brian S

2008-01-01

Humans with cerebellar degeneration commonly exhibit downbeat nystagmus (DBN). DBN has gravity-independent and -dependent components, and the latter has been proposed to reflect hyperactive tilt maculo-ocular reflexes (tilt-MOR). Mice with genetically determined cerebellar ataxia do not exhibit DBN, but they do exhibit tonic hyperdeviation of the eyes, which we have proposed to be the DBN equivalent. As such, the tilt-MOR might be predicted to be hyperactive in these mutant mice. We measured the tilt-MOR in 10 normal C57BL/6 mice and in 6 tottering, a mutant exhibiting ataxia and ocular motor abnormalities due to mutation of the P/Q calcium channel. Awake mice were placed in body orientations spanning 360 degrees about the pitch axis. The absolute, equilibrium vertical angular deviations of one eye were measured using infrared videooculography. In both strains, eye elevation varied quasi-sinusoidally with tilt angle in the range of 90 degrees nose-up to 90 degrees nose-down. Beyond this range the eye returned to a neutral position. Deviation over +/-30 degrees of tilt was an approximately linear function of the projection of the gravity vector into the animal's horizontal plane, and can thus be summarized by its slope (sensitivity). Sensitivity measured 14.9 degrees/g for C57BL/6 and 20.3 degrees/g for tottering, a statistically significant difference. Thus the pitch otolithic reflex of the ataxic mutants is hyperactive relative to controls and could explain tonic hyperdeviation of the eyes, consistent with the idea that the tonic hyperdeviation is analogous to DBN.

Head position modulates optokinetic nystagmus

PubMed Central

Ferraresi, A.; Botti, F. M.; Panichi, R.; Barmack, N. H.

2011-01-01

Orientation and movement relies on both visual and vestibular information mapped in separate coordinate systems. Here, we examine how coordinate systems interact to guide eye movements of rabbits. We exposed rabbits to continuous horizontal optokinetic stimulation (HOKS) at 5°/s to evoke horizontal eye movements, while they were statically or dynamically roll-tilted about the longitudinal axis. During monocular or binocular HOKS, when the rabbit was roll-tilted 30° onto the side of the eye stimulated in the posterior → anterior (P → A) direction, slow phase eye velocity (SPEV) increased by 3.5–5°/s. When the rabbit was roll-tilted 30° onto the side of the eye stimulated in the A → P direction, SPEV decreased to ~2.5°/s. We also tested the effect of roll-tilt after prolonged optokinetic stimulation had induced a negative optokinetic afternystagmus (OKAN II). In this condition, the SPEV occurred in the dark, “open loop.” Modulation of SPEV of OKAN II depended on the direction of the nystagmus and was consistent with that observed during “closed loop” HOKS. Dynamic roll-tilt influenced SPEV evoked by HOKS in a similar way. The amplitude and the phase of SPEV depended on the frequency of vestibular oscillation and on HOKS velocity. We conclude that the change in the linear acceleration of the gravity vector with respect to the head during roll-tilt modulates the gain of SPEV depending on its direction. This modulation improves gaze stability at different image retinal slip velocities caused by head roll-tilt during centric or eccentric head movement. PMID:21735244

Head position modulates optokinetic nystagmus.

PubMed

Pettorossi, V E; Ferraresi, A; Botti, F M; Panichi, R; Barmack, N H

2011-08-01

Orientation and movement relies on both visual and vestibular information mapped in separate coordinate systems. Here, we examine how coordinate systems interact to guide eye movements of rabbits. We exposed rabbits to continuous horizontal optokinetic stimulation (HOKS) at 5°/s to evoke horizontal eye movements, while they were statically or dynamically roll-tilted about the longitudinal axis. During monocular or binocular HOKS, when the rabbit was roll-tilted 30° onto the side of the eye stimulated in the posterior → anterior (P → A) direction, slow phase eye velocity (SPEV) increased by 3.5-5°/s. When the rabbit was roll-tilted 30° onto the side of the eye stimulated in the A → P direction, SPEV decreased to ~2.5°/s. We also tested the effect of roll-tilt after prolonged optokinetic stimulation had induced a negative optokinetic afternystagmus (OKAN II). In this condition, the SPEV occurred in the dark, "open loop." Modulation of SPEV of OKAN II depended on the direction of the nystagmus and was consistent with that observed during "closed loop" HOKS. Dynamic roll-tilt influenced SPEV evoked by HOKS in a similar way. The amplitude and the phase of SPEV depended on the frequency of vestibular oscillation and on HOKS velocity. We conclude that the change in the linear acceleration of the gravity vector with respect to the head during roll-tilt modulates the gain of SPEV depending on its direction. This modulation improves gaze stability at different image retinal slip velocities caused by head roll-tilt during centric or eccentric head movement.

Barriers and facilitators to ED physician use of the test and treatment for BPPV

PubMed Central

Forman, Jane; Damschroder, Laura; Telian, Steven A.; Fagerlin, Angela; Johnson, Patricia; Brown, Devin L.; An, Lawrence C.; Morgenstern, Lewis B.; Meurer, William J.

2017-01-01

Abstract Background: The test and treatment for benign paroxysmal positional vertigo (BPPV) are evidence-based practices supported by clinical guideline statements. Yet these practices are underutilized in the emergency department (ED) and interventions to promote their use are needed. To inform the development of an intervention, we interviewed ED physicians to explore barriers and facilitators to the current use of the Dix-Hallpike test (DHT) and the canalith repositioning maneuver (CRM). Methods: We conducted semi-structured in-person interviews with ED physicians who were recruited at annual ED society meetings in the United States. We analyzed data thematically using qualitative content analysis methods. Results: Based on 50 interviews with ED physicians, barriers that contributed to infrequent use of DHT/CRM that emerged were (1) prior negative experiences or forgetting how to perform them and (2) reliance on the history of present illness to identify BPPV, or using the DHT but misattributing patterns of nystagmus. Based on participants' responses, the principal facilitator of DHT/CRM use was prior positive experiences using these, even if infrequent. When asked which clinical supports would facilitate more frequent use of DHT/CRM, participants agreed supports needed to be brief, readily accessible, and easy to use, and to include well-annotated video examples. Conclusions: Interventions to promote the use of the DHT/CRM in the ED need to overcome prior negative experiences with the DHT/CRM, overreliance on the history of present illness, and the underuse and misattribution of patterns of nystagmus. Future resources need to be sensitive to provider preferences for succinct information and video examples. PMID:28680765

Causes of visual impairment in children with low vision.

PubMed

Shah, Mufarriq; Khan, Mirzaman; Khan, Muhammad Tariq; Khan, Mohammad Younas; Saeed, Nasir

2011-02-01

To determine the main causes of visual impairment in children with low vision. To assess the need of spectacles and low vision devices (LVDs) in children and to evaluate visual outcome after using their LVDs for far and near distance. Observational study. Khyber Institute of Ophthalmic Medical Sciences, Peshawar, Pakistan, from June 2006 to December 2007. The clinical record of 270 children with low vision age 4-16 years attending the Low Vision Clinic were included. All those children, aged 4-16 years, who had corrected visual acuity (VA) less than 6/18 in the better eye after medical or surgical treatment, were included in the study. WHO low vision criteria were used to classify into visually impaired, severe visually impaired and blind. Results were described as percentage frequencies. One hundred and eighty nine (70%) were males and 81 (30%) were females. The male to female ratio was 2.3:1. The main causes of visual impairment included nystagmus (15%), Stargardt's disease (14%), maculopathies (13%), myopic macular degeneration (11%) and oculocutaneous albinism (7%). The percentages of visually impaired, severe visually impaired and blind were 33.8%, 27.2% and 39.0% respectively. Spectacles were prescribed to 146 patients and telescopes were prescribed to 75 patients. Spectacles and telescope both were prescribed to 179 patients while Ocutech telescope was prescribed to 4 patients. Retinal diseases nystagmus and macular conditions were mainly responsible for low vision in children. Visually impaired children especially with hereditary/congenital ocular anomalies benefit from refraction and low vision services which facilitate vision enhancement and inclusive education.

Magnetic vestibular stimulation modulates default mode network fluctuations.

PubMed

Boegle, Rainer; Stephan, Thomas; Ertl, Matthias; Glasauer, Stefan; Dieterich, Marianne

2016-02-15

Strong magnetic fields (>1 Tesla) can cause dizziness and it was recently shown that healthy subjects (resting in total darkness) developed a persistent nystagmus even when remaining completely motionless within a MR tomograph. Consequently, it was speculated that this magnetic vestibular stimulation (MVS) might influence fMRI results, as nystagmus is indicative of an imbalance in the vestibular system, potentially influencing other systems via multisensory vestibular interactions. The objective of our study was to investigate whether MVS does indeed modulate BOLD signal fluctuations. We recorded eye movements, as well as, resting-state fMRI of 30 volunteers in darkness at 1.5 T and 3.0 T to answer the question whether MVS modulated parts of the default mode resting-state network (DMN) in accordance with the Lorentz-force model for MVS, while distinguishing this from the known signal increase due to field strength related imaging effects. Our results showed that modulation of the default mode network occurred mainly in areas associated with vestibular and ocular motor function, and was in accordance with the Lorentz-force model, i.e., double than the expected signal scaling due to field strength alone. We discuss the implications of our findings for the interpretation of studies using resting-state fMRI, especially those concerning vestibular research. We conclude that MVS needs to be considered in vestibular research to avoid biased results, but it might also offer the possibility of manipulating network dynamics and may thus help in studying the brain as a dynamical system. Copyright © 2015 Elsevier Inc. All rights reserved.

Objective and automated measurement of dynamic vision functions

NASA Technical Reports Server (NTRS)

Flom, M. C.; Adams, A. J.

1976-01-01

A phoria stimulus array and electro-oculographic (EOG) arrangements for measuring motor and sensory responses of subjects subjected to stress or drug conditions are described, along with experimental procedures. Heterophoria (as oculomotor function) and glare recovery time (time required for photochemical and neural recovery after exposure to a flash stimulus) are measured, in research aimed at developing automated objective measurement of dynamic vision functions. Onset of involuntary optokinetic nystagmus in subjects attempting to track moving stripes (while viewing through head-mounted binocular eyepieces) after exposure to glare serves as an objective measure of glare recovery time.

Bilateral aniridia lenticular coloboma and snowflake retinal degeneration.

PubMed

Doganay, Selim; Emre, Sinan; Firat, Penpegül

2009-01-01

A 6-year-old boy presented with bilateral aniridia associated with lens coloboma and snowflake retinal degeneration. Ophthalmologic examination revealed bilateral corneal peripheral epithelial thickening and aniridia. Additionally, the patient had lenticular coloboma and snowflake retinal degeneration in both eyes. Intraocular pressure was 22 mm Hg bilaterally. The patient also had pendular nystagmus. Uncorrected visual acuity was counting fingers at 2 meters for both eyes, but improved to 0.2 and 0.05, respectively, with correction. Congenital aniridia has been reported with various ophthalmic pathologies, but this is the first case to display bilateral lenticular coloboma and snowflake retinal degeneration associated with aniridia.

Experiment K-7-31: Studies of Vestibular Primary Afferents and Eye Movements in Normal, Hypergravity and Hypogravity - Axon Cosmos Flight 2044

NASA Technical Reports Server (NTRS)

Correia, M. J.; Perachio, A. A.; Dickman, J. D.; Kozlovskaya, I.; Sirota, M.; Yakushin, S.; Beloozerova, I. N.

1994-01-01

Fourteen days of active head movements in microgravity appear to modify the gain and neural adaptation properties of the horizontal semicircular canals in the rhesus monkey. This is the first demonstration of adaptive plasticity in the sensory receptor. Reversing prisms, for example, do not modify the gain of the primary afferent response. Pulse yaw rotation, sinusoidal rotation, and sum of sinusoidal rotation testing during the first day following recovery revealed that the gain of a sample of afferents was significantly greater than the gain derived from afferent responses obtained during pre-flight and control monkey testing. There was no strong evidence of tilt sensitivity in the sample of afferents that we tested either during the pre-flight or control tests or during the first day post-flight. Two irregular afferents tested on postflight day 2 showed changes with tilt but the responses were not systematic. The spontaneous discharge did not change following flight. Mean firing rate and coefficient of variation remained constant during the post flight tests and was near the value measured during pre flight tests. The change in gain of horizontal canal afferents might be adaptive. The animals were required to look at a target for food. This required active head and eye movements. Active head movements have been shown to be hypometric and eye movements have been shown to be hypermetric during the first few days of past Cosmos flights (see introduction). It might be that the increased gain in the horizontal semicircular canals permit accurate target acquisition during hypometric head movements by driving the eyes to greater angles for smaller angles of head movement. The mechanism by which the semicircular canals recalibrate (increase their gain) is unknown. The efferent vestibular system is a logical candidate. Horizontal nystagmus during rotation about an earth vertical axis with the horizontal semicircular canals in the plane of rotation produced the same response during postflight day 1 and post-flight day 9. But when the head was pitched down 45? the nystagmus slow phase velocity was greater and the duration was about twice during post-flight day 1. Apparently, this response involving the interaction of the horizontal and vertical semicircular canals and the otoliths did not recalibrate during post-flight day 1. The 'DC' bias of the slow phase velocity of the horizontal nystagmus during constant velocity horizontal axis rotation was roughly 4 times for one flight monkey and roughly 2 times for the other on post-flight day 1 compared to post-flight day 9. These results suggest that the otolith mediated response during constant velocity rotation also did not recalibrate on post-flight day 1.

Contrasting effects of strabismic amblyopia on metabolic activity in superficial and deep layers of striate cortex.

PubMed

Adams, Daniel L; Economides, John R; Horton, Jonathan C

2015-05-01

To probe the mechanism of visual suppression, we have raised macaques with strabismus by disinserting the medial rectus muscle in each eye at 1 mo of age. Typically, this operation produces a comitant, alternating exotropia with normal acuity in each eye. Here we describe an unusual occurrence: the development of severe amblyopia in one eye of a monkey after induction of exotropia. Shortly after surgery, the animal demonstrated a strong fixation preference for the left eye, with apparent suppression of the right eye. Later, behavioral testing showed inability to track or to saccade to targets with the right eye. With the left eye occluded, the animal demonstrated no visually guided behavior. Optokinetic nystagmus was absent in the right eye. Metabolic activity in striate cortex was assessed by processing the tissue for cytochrome oxidase (CO). Amblyopia caused loss of CO in one eye's rows of patches, presumably those serving the blind eye. Layers 4A and 4B showed columns of reduced CO, in register with pale rows of patches in layer 2/3. Layers 4C, 5, and 6 also showed columns of CO activity, but remarkably, comparison with more superficial layers showed a reversal in contrast. In other words, pale CO staining in layers 2/3, 4A, and 4B was aligned with dark CO staining in layers 4C, 5, and 6. No experimental intervention or deprivation paradigm has been reported previously to produce opposite effects on metabolic activity in layers 2/3, 4A, and 4B vs. layers 4C, 5, and 6 within a given eye's columns. Copyright © 2015 the American Physiological Society.

Diagnosing Stroke in Acute Vertigo: The HINTS Family of Eye Movement Tests and the Future of the "Eye ECG".

PubMed

Newman-Toker, David E; Curthoys, Ian S; Halmagyi, G Michael

2015-10-01

Patients who present to the emergency department with symptoms of acute vertigo or dizziness are frequently misdiagnosed. Missed opportunities to promptly treat dangerous strokes can result in poor clinical outcomes. Inappropriate testing and incorrect treatments for those with benign peripheral vestibular disorders leads to patient harm and unnecessary costs. Over the past decade, novel bedside approaches to diagnose patients with the acute vestibular syndrome have been developed and refined. A battery of three bedside tests of ocular motor physiology known as "HINTS" (head impulse, nystagmus, test of skew) has been shown to identify acute strokes more accurately than even magnetic resonance imaging with diffusion-weighted imaging (MRI-DWI) when applied in the early acute period by eye-movement specialists. Recent advances in lightweight, high-speed video-oculography (VOG) technology have made possible a future in which HINTS might be applied by nonspecialists in frontline care settings using portable VOG. Use of technology to measure eye movements (VOG-HINTS) to diagnose stroke in the acute vestibular syndrome is analogous to the use of electrocardiography (ECG) to diagnose myocardial infarction in acute chest pain. This "eye ECG" approach could transform care for patients with acute vertigo and dizziness around the world. In the United States alone, successful implementation would likely result in improved quality of emergency care for hundreds of thousands of peripheral vestibular patients and tens of thousands of stroke patients, as well as an estimated national health care savings of roughly $1 billion per year. In this article, the authors review the origins of the HINTS approach, empiric evidence and pathophysiologic principles supporting its use, and possible uses for the eye ECG in teleconsultation, teaching, and triage. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

[Epidural anaesthesia: Simulated intravascular test dose with S(+) ketamine, lidocaine and adrenaline. A prospective, randomized, double blind and placebo controlled study].

PubMed

Higueras, R; Errando, C L; Soriano-Bru, J L

2015-02-01

The use of a test dose in epidural anaesthesia is a safety recommendation. However specificity and sensitivity of the drugs used with this indication have been not conclusive. The main objective of this study was to compare the effectiveness and the adverse effects of a simulated intravascular test dose of adrenaline, lidocaine and S(+)-ketamine. A prospective, randomized, double blinded, placebo controlled study was designed. ASA I patients scheduled for elective surgery were included. These were randomized to the following study groups: S(+)-ketamine 0.5 mg.kg-1 (S+K group), 5% lidocaine 1.5 mg.kg-1 (L5% group), adrenaline 15μg (ADR group), and physiological saline 3 ml (SF group; control group). An evaluation was made during the first 15 minutes after the study drug was administered. Variables including heart rate (HR) systolic and diastolic blood pressure (sBP and dBP), mean arterial pressure (MAP), and SpO2 were recorded at 0 min (baseline) and at 2, 5, 8, 10 and 15 minutes after drug injection. An increase of at least 20 beats per minute (bpm) in relation to the baseline measurement was considered a positive result, as was an increase sBP >15 mmHg. The clinical effects described as related to iv injection of the study drugs recorded were: sedation-hypnosis, dizziness, nystagmus, metallic taste perception, perioral or facial paresthesias, tinnitus, as well as any other effect the patients mentioned. Sensitivity and specificity were calculated as was the percent increase in the parameters in order to see if these were clinically useful. A total of 80 patients, 20 per group, were included. The sBP, dBP, and MAP were significantly raised at the 2, 5, 8 and 10 minutes measurements in the S(+)K group compared to the rest of the groups (P<.05), as well as HR in the 2, 5, 8, 10 and 15 minute measurements in the S(+)K compared to the rest of the groups (P<.05). Sensitivity and specificity were high, and significant in the S(+)K group from minute 2 to minute 8 compared with the placebo group, as well as percentage points differences in the same interval. There were significant differences in the incidence of sedation-hypnosis, nystagmus and dizziness, which were more frequent in the S(+)K group. There were no differences in the incidence of metallic taste, perioral and facial paresthesias or tinnitus. The effects in the S(+)K group always occurred before minute 5 after drug injection. Both lidocaine an adrenaline at the scheduled doses showed low sensitivity and specificity as a simulated iv epidural test dose. S(+)-ketamine could be a feasible marker after accidental iv injection during epidural anaesthesia or analgesia. Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

Eye Movement Abnormalities in Joubert Syndrome

PubMed Central

Weiss, Avery H.; Doherty, Dan; Parisi, Melissa; Shaw, Dennis; Glass, Ian; Phillips, James O.

2011-01-01

Purpose Joubert syndrome is a genetic disorder characterized by hypoplasia of the midline cerebellum and deficiency of crossed connections between neural structures in the brain stem that control eye movements. The goal of the study was to quantify the eye movement abnormalities that occur in Joubert syndrome. Methods Eye movements were recorded in response to stationary stimuli and stimuli designed to elicit smooth pursuit, saccades, optokinetic nystagmus (OKN), vestibulo-ocular reflex (VOR), and vergence using video-oculography or Skalar search coils in 8 patients with Joubert syndrome. All patients underwent high-resolution magnetic resonance imaging (MRI). Results All patients had the highly characteristic molar tooth sign on brain MRI. Six patients had conjugate pendular (n = 4) or see-saw nystagmus (n = 2); gaze holding was stable in four patients. Smooth-pursuit gains were 0.28 to 1.19, 0.11 to 0.68, and 0.33 to 0.73 at peak stimulus velocities of 10, 20, and 30 deg/s in six patients; smooth pursuit could not be elicited in four patients. Saccade gains in five patients ranged from 0.35 to 0.91 and velocities ranged from 60.9 to 259.5 deg/s. Targeted saccades could not be elicited in five patients. Horizontal OKN gain was uniformly reduced across gratings drifted at velocities of 15, 30, and 45 deg/s. VOR gain was 0.8 or higher and phase appropriate in three of seven subjects; VOR gain was 0.3 or less and phase was indeterminate in four subjects. Conclusions The abnormalities in gaze-holding and eye movements are consistent with the distributed abnormalities of midline cerebellum and brain stem regions associated with Joubert syndrome. PMID:19443711

The Clinical Evaluation of Alcohol Intoxication Is Inaccurate in Trauma Patients.

PubMed

Kumar, Ashwini; Holloway, Travis; Cohn, Stephen M; Goodwiler, Gregory; Admire, John R

2018-02-14

Discharging patients from emergency centers based on the clinical features of intoxication alone may be dangerous, as these may poorly correlate with ethanol measurements. We determined the feasibility of utilizing a hand-held breath alcohol analyzer to aid in the disposition of intoxicated trauma patients by comparing serial breathalyzer (Intoximeter, Alco-Sensor FST, St. Louis, Missouri, USA] data with clinical assessments in determining the readiness of trauma patients for discharge. A total of 20 legally intoxicated (LI) patients (blood alcohol concentration (BAC) >80 mg/dL) brought to our trauma center were prospectively investigated. Serial breath samples were obtained using a breathalyzer as a surrogate measure of repeated BAC. A clinical exam (nystagmus, one-leg balance, heel-toe walk) was performed prior to each breath sampling. The enrollees were 85% male, age 30±10 (range 19-51), with a body mass index (BMI) of 29±7. The average initial body alcohol level (BAL) was 245±61 (range 162-370) mg/dL. Based on breath samples, the alcohol elimination rates varied from 21.5 mg/dL/hr to 45.7 mg/dL/hr (mean 28.5 mg/dL/hr). There were no significant differences in alcohol elimination rates by gender, age, or BMI. The clinical exam also varied widely among patients; only seven of 16 (44%) LI patients demonstrated horizontal nystagmus (suggesting sobriety when actually LI) and the majority of the LI patients (66%) were able to complete the balance tasks (suggesting sobriety). Intoxicated trauma patients have an unreliable clinical sobriety exam and a wide range of alcohol elimination rates. The portable alcohol breath analyzer represents a potential option to easily and inexpensively establish legal sobriety in this population.

Comparative analysis of pharmacological treatments with N-acetyl-DL-leucine (Tanganil) and its two isomers (N-acetyl-L-leucine and N-acetyl-D-leucine) on vestibular compensation: Behavioral investigation in the cat.

PubMed

Tighilet, Brahim; Leonard, Jacques; Bernard-Demanze, Laurence; Lacour, Michel

2015-12-15

Head roll tilt, postural imbalance and spontaneous nystagmus are the main static vestibular deficits observed after an acute unilateral vestibular loss (UVL). In the UVL cat model, these deficits are fully compensated over 6 weeks as the result of central vestibular compensation. N-Acetyl-dl-leucine is a drug prescribed in clinical practice for the symptomatic treatment of acute UVL patients. The present study investigated the effects of N-acetyl-dl-leucine on the behavioral recovery after unilateral vestibular neurectomy (UVN) in the cat, and compared the effects of each of its two isomers N-acetyl-L-leucine and N-acetyl-D-leucine. Efficacy of these three drug treatments has been evaluated with respect to a placebo group (UVN+saline water) on the global sensorimotor activity (observation grids), the posture control (support surface measurement), the locomotor balance (maximum performance at the rotating beam test), and the spontaneous vestibular nystagmus (recorded in the light). Whatever the parameters tested, the behavioral recovery was strongly and significantly accelerated under pharmacological treatments with N-acetyl-dl-leucine and N-acetyl-L-leucine. In contrast, the N-acetyl-D-leucine isomer had no effect at all on the behavioral recovery, and animals of this group showed the same recovery profile as those receiving a placebo. It is concluded that the N-acetyl-L-leucine isomer is the active part of the racemate component since it induces a significant acceleration of the vestibular compensation process similar (and even better) to that observed under treatment with the racemate component only. Copyright © 2015 Elsevier B.V. All rights reserved.

Head-shaking nystagmus predicts greater disability in unilateral peripheral vestibulopathy.

PubMed

Angeli, Simon I; Velandia, Sandra; Snapp, Hillary

2011-01-01

The objective of this study was to determine the association of the bedside test of head-shaking nystagmus (HSN) with patients' self-perceived dizziness handicap as well as this test's sensitivity and specificity in unilateral peripheral vestibular hypofunction. A retrospective case-control study was performed. The study was held at an academic, tertiary referral center. Fifty-three adult patients with unilateral peripheral hypofunction defined by the caloric test of the videonystagmography with documented bedside HSN and who had completed questionnaires of self-perceived dizziness handicap were included. The sensitivity and specificity of the bedside HSN in patients and 10 healthy controls in diagnosing unilateral vestibular hypofunction defined by videonystagmographic caloric testing and by abnormal gain and symmetry of the vestibular-ocular reflex by rotary chair testing were determined. Scores of the screening test of the Dizziness Handicap Index and Functional Level Scale questionnaires were taken. When using the caloric irrigation test as the reference standard for unilateral vestibular hypofunction, the sensitivity, specificity, and positive predictive value of the bedside HSN were 31%, 96%, and 97%, respectively. When comparing with results of rotational chair testing (vestibular-ocular reflex gain and symmetry), the sensitivity of the HSN test increases to 71%. Patients with positive bedside HSN had higher scores (greater self-perceived dizziness handicap) of the Dizziness Handicap Index (P = .049) and higher (worse) scores of the Functional Level Scale (P = .0377) than those with negative bedside HSN (Wilcoxon rank test). Greater perceived handicap was correlated with a positive bedside HSN in patients with unilateral peripheral vestibulopathy. The HSN has sufficient sensitivity to be used as screening test of uncompensated vestibulopathy in this series. However, a negative HSN alone does not rule out the diagnosis of peripheral vestibular dysfunction. Copyright © 2011 Elsevier Inc. All rights reserved.

Vestibulo-oculomotor behaviour in rats following a transient unilateral vestibular loss induced by lidocaine.

PubMed

Magnusson, A K; Tham, R

2003-01-01

The effects of a transient vestibular nerve blockade, achieved by intra-tympanic instillation of lidocaine, were studied in rats by recording horizontal eye movements in darkness. Evaluation of the dose-response relationship showed that a maximal effect was attained with a concentration of 4% lidocaine. Within 15 min of lidocaine instillation, a vigorous spontaneous nystagmus was observed which reached maximal frequency and velocity of the slow phase after about 20 min. Subsequently, the nystagmus failed for approximately half an hour before it reappeared. This could be avoided by providing visual feedback in between the recordings in darkness or by a contralateral instillation of 2.5% lidocaine. It is suggested that the failure reflects an overload of the vestibulo-oculomotor circuits. After recovery from the nerve blockade, when the gaze was stable, dynamic vestibular tests were performed. They revealed that a decrease of the slow phase velocity gain and the dominant time constant during, respectively, sinusoidal- and step stimulation toward the unanaesthetised side, had developed with the nerve blockade. These modulations were impaired by a nodulo-uvulectomy but not by bilateral flocculectomy, which is consistent with the concept of vestibular habituation. A GABA(B) receptor antagonist, CGP 56433A, given systemically during the nerve blockade, aggravated the vestibular asymmetry. The same effect has previously been demonstrated in both short- (days) and long-term (months) compensated rats, by antagonising the GABA(B) receptor. In summary, this study provides the first observations of vestibulo-oculomotor disturbances during the first hour after a rapid and transient unilateral vestibular loss in the rat. By using this method, it is possible to study immediate behavioural consequences and possible neural changes that might outlast the nerve blockade.

Contributing factors to VEP grating acuity deficit and inter-ocular acuity difference in children with cerebral visual impairment.

PubMed

Cavascan, Nívea Nunes; Salomão, Solange Rios; Sacai, Paula Yuri; Pereira, Josenilson Martins; Rocha, Daniel Martins; Berezovsky, Adriana

2014-04-01

To investigate contributing factors to visual evoked potential (VEP) grating acuity deficit (GAD) and inter-ocular acuity difference (IAD) measured by sweep-VEPs in children with cerebral visual impairment (CVI). VEP GAD was calculated for the better acuity eye by subtracting acuity thresholds from mean normal VEP grating acuity according to norms from our own laboratory. Deficits were categorized as mild (0.17 ≤ deficit < 0.40 log units), moderate (0.40 ≤ deficit < 0.70 log units) or severe (deficit ≥0.70 log units). Maximum acceptable IAD was 0.10 log units. A group of 115 children (66 males-57 %) with ages ranging from 1.2 to 166.5 months (median = 17.7) was examined. VEP GAD ranged from 0.17 to 1.28 log units (mean = 0.68 ± 0.27; median = 0.71), and it was mild in 23 (20 %) children, moderate in 32 (28 %) and severe in 60 (52 %). Severe deficit was significantly associated with older age and anti-seizure drug therapy. IAD ranged from 0 to 0.49 log units (mean = 0.06 ± 0.08; median = 0.04) and was acceptable in 96 (83 %) children. Children with strabismus and nystagmus had IAD significantly larger compared to children with orthoposition. In a large cohort of children with CVI, variable severity of VEP GAD was found, with more than half of the children with severe deficits. Older children and those under anti-seizure therapy were at higher risk for larger deficits. Strabismus and nystagmus provided larger IADs. These results should be taken into account on the clinical management of children with this leading cause of bilateral visual impairment.

Playing checkers: detection and eye hand coordination in simulated prosthetic vision

NASA Astrophysics Data System (ADS)

Dagnelie, Gislin; Walter, Matthias; Yang, Liancheng

2006-09-01

In order to assess the potential for visual inspection and eye hand coordination without tactile feedback under conditions that may be available to future retinal prosthesis wearers, we studied the ability of sighted individuals to act upon pixelized visual information at very low resolution, equivalent to 20/2400 visual acuity. Live images from a head-mounted camera were low-pass filtered and presented in a raster of 6 × 10 circular Gaussian dots. Subjects could either freely move their gaze across the raster (free-viewing condition) or the raster position was locked to the subject's gaze by means of video-based pupil tracking (gaze-locked condition). Four normally sighted and one severely visually impaired subject with moderate nystagmus participated in a series of four experiments. Subjects' task was to count 1 to 16 white fields randomly distributed across an otherwise black checkerboard (counting task) or to place a black checker on each of the white fields (placing task). We found that all subjects were capable of learning both tasks after varying amounts of practice, both in the free-viewing and in the gaze-locked conditions. Normally sighted subjects all reached very similar performance levels independent of the condition. The practiced performance level of the visually impaired subject in the free-viewing condition was indistinguishable from that of the normally sighted subjects, but required approximately twice the amount of time to place checkers in the gaze-locked condition; this difference is most likely attributable to this subject's nystagmus. Thus, if early retinal prosthesis wearers can achieve crude form vision, then on the basis of these results they too should be able to perform simple eye hand coordination tasks without tactile feedback.

Reliability and Validity of Gaze-Dependent Functional Vision Space: A Novel Metric Quantifying Visual Function in Infantile Nystagmus Syndrome.

PubMed

Roberts, Tawna L; Kester, Kristi N; Hertle, Richard W

2018-04-01

This study presents test-retest reliability of optotype visual acuity (OVA) across 60° of horizontal gaze position in patients with infantile nystagmus syndrome (INS). Also, the validity of the metric gaze-dependent functional vision space (GDFVS) is shown in patients with INS. In experiment 1, OVA was measured twice in seven horizontal gaze positions from 30° left to right in 10° steps in 20 subjects with INS and 14 without INS. Test-retest reliability was assessed using intraclass correlation coefficient (ICC) in each gaze. OVA area under the curve (AUC) was calculated with horizontal eye position on the x-axis, and logMAR visual acuity on the y-axis and then converted to GDFVS. In experiment 2, validity of GDFVS was determined over 40° horizontal gaze by applying the 95% limits of agreement from experiment 1 to pre- and post-treatment GDFVS values from 85 patients with INS. In experiment 1, test-retest reliability for OVA was high (ICC ≥ 0.88) as the difference in test-retest was on average less than 0.1 logMAR in each gaze position. In experiment 2, as a group, INS subjects had a significant increase (P < 0.001) in the size of their GDFVS that exceeded the 95% limits of agreement found during test-retest. OVA is a reliable measure in INS patients across 60° of horizontal gaze position. GDFVS is a valid clinical method to be used to quantify OVA as a function of eye position in INS patients. This method captures the dynamic nature of OVA in INS patients and may be a valuable measure to quantify visual function patients with INS, particularly in quantifying change as part of clinical studies.

Vestibular adaptation to space in monkeys.

PubMed

Dai, M; Raphan, T; Kozlovskaya, I; Cohen, B

1998-07-01

Otolith-induced eye movements of rhesus monkeys were studied before and after the 1989 COSMOS 2044 and the 1992 to 1993 COSMOS 2229 flights. Two animals flew in each mission for approximately 2 weeks. After flight, spatial orientation of the angular vestibulo-ocular reflex was altered. In one animal the time constant of postrotatory nystagmus, which had been shortened by head tilts with regard to gravity before flight, was unaffected by the same head tilts after flight. In another animal, eye velocity, which tended to align with a gravitational axis before flight, moved toward a body axis after flight. This shift of orientation disappeared by 7 days after landing. After flight, the magnitude of compensatory ocular counter-rolling was reduced by about 70% in both dynamic and static tilts. Modulation in vergence in response to naso-occipital linear acceleration during off-vertical axis rotation was reduced by more than 50%. These changes persisted for 11 days after recovery. An up and down asymmetry of vertical nystagmus was diminished for 7 days. Gains of the semicircular canal-induced horizontal and vertical angular vestibulo-ocular reflexes were unaffected in both flights, but the gain of the roll angular vestibulo-ocular reflex was decreased. These data indicate that there are short- and long-term changes in otolith-induced eye movements after adaptation to microgravity. These experiments also demonstrate the unique value of the monkey as a model for studying effects of vestibular adaptation in space. Eye movements can be measured in three dimensions in response to controlled vestibular and visual stimulation, and the results are directly applicable to human beings. Studies in monkeys to determine how otolith afferent input and central processing is altered by adaptation to microgravity should be an essential component of future space-related research.

Small strokes causing severe vertigo: frequency of false-negative MRIs and nonlacunar mechanisms.

PubMed

Saber Tehrani, Ali S; Kattah, Jorge C; Mantokoudis, Georgios; Pula, John H; Nair, Deepak; Blitz, Ari; Ying, Sarah; Hanley, Daniel F; Zee, David S; Newman-Toker, David E

2014-07-08

Describe characteristics of small strokes causing acute vestibular syndrome (AVS). Ambispective cross-sectional study of patients with AVS (acute vertigo or dizziness, nystagmus, nausea/vomiting, head-motion intolerance, unsteady gait) with at least one stroke risk factor from 1999 to 2011 at a single stroke referral center. Patients underwent nonquantitative HINTS "plus" examination (head impulse, nystagmus, test-of-skew plus hearing), neuroimaging to confirm diagnoses (97% by MRI), and repeat MRI in those with initially normal imaging but clinical signs of a central lesion. We identified patients with diffusion-weighted imaging (DWI) strokes ≤10 mm in axial diameter. Of 190 high-risk AVS presentations (105 strokes), we found small strokes in 15 patients (median age 64 years, range 41-85). The most common vestibular structure infarcted was the inferior cerebellar peduncle (73%); the most common stroke location was the lateral medulla (60%). Focal neurologic signs were present in only 27%. The HINTS "plus" battery identified small strokes with greater sensitivity than early MRI-DWI (100% vs 47%, p < 0.001). False-negative initial MRIs (6-48 hours) were more common with small strokes than large strokes (53% [n = 8/15] vs 7.8% [n = 7/90], p < 0.001). Nonlacunar stroke mechanisms were responsible in 47%, including 6 vertebral artery occlusions or dissections. Small strokes affecting central vestibular projections can present with isolated AVS. The HINTS "plus" hearing battery identifies these patients with greater accuracy than early MRI-DWI, which is falsely negative in half, up to 48 hours after onset. We found nonlacunar mechanisms in half, suggesting greater risk than might otherwise be assumed for patients with such small infarctions. © 2014 American Academy of Neurology.

Predictors of Sensitivity to Perceptual Learning in Children With Infantile Nystagmus.

PubMed

Huurneman, Bianca; Boonstra, F Nienke; Goossens, Jeroen

2017-08-01

To identify predictors of sensitivity to perceptual learning on a computerized, near-threshold letter discrimination task in children with infantile nystagmus (idiopathic IN: n = 18; oculocutaneous albinism accompanied by IN: n = 18). Children were divided into two age-, acuity-, and diagnosis-matched training groups: a crowded (n = 18) and an uncrowded training group (n = 18). Training consisted of 10 sessions spread out over 5 weeks (grand total of 3500 trials). Baseline performance, age, diagnosis, training condition, and perceived pleasantness of training (training joy) were entered as linear regression predictors of training-induced changes on a single- and a crowded-letter task. An impressive 57% of the variability in improvements of single-letter visual acuity was explained by age, training condition, and training joy. Being older and training with uncrowded letters were associated with larger single-letter visual acuity improvements. More training joy was associated with a larger gain from the uncrowded training and a smaller gain from the crowded training. Fifty-six percent of the variability in crowded-letter task improvements was explained by baseline performance, age, diagnosis, and training condition. After regressing out the variability induced by training condition, baseline performance, and age, perceptual learning proved more effective for children with idiopathic IN than for children with albinism accompanied by IN. Training gains increased with poorer baseline performance in idiopaths, but not in children with albinism accompanied by IN. Age and baseline performance, but not training joy, are important prognostic factors for the effect of perceptual learning in children with IN. However, their predictive value for achieving improvements in single-letter acuity and crowded letter acuity, respectively, differs between diagnostic subgroups and training condition. These findings may help with personalized treatment of individuals likely to benefit from perceptual learning.

Perceptual Learning in Children With Infantile Nystagmus: Effects on Visual Performance.

PubMed

Huurneman, Bianca; Boonstra, F Nienke; Goossens, Jeroen

2016-08-01

To evaluate whether computerized training with a crowded or uncrowded letter-discrimination task reduces visual impairment (VI) in 6- to 11-year-old children with infantile nystagmus (IN) who suffer from increased foveal crowding, reduced visual acuity, and reduced stereopsis. Thirty-six children with IN were included. Eighteen had idiopathic IN and 18 had oculocutaneous albinism. These children were divided in two training groups matched on age and diagnosis: a crowded training group (n = 18) and an uncrowded training group (n = 18). Training occurred two times per week during 5 weeks (3500 trials per training). Eleven age-matched children with normal vision were included to assess baseline differences in task performance and test-retest learning. Main outcome measures were task-specific performance, distance and near visual acuity (DVA and NVA), intensity and extent of (foveal) crowding at 5 m and 40 cm, and stereopsis. Training resulted in task-specific improvements. Both training groups also showed uncrowded and crowded DVA improvements (0.10 ± 0.02 and 0.11 ± 0.02 logMAR) and improved stereopsis (670 ± 249″). Crowded NVA improved only in the crowded training group (0.15 ± 0.02 logMAR), which was also the only group showing a reduction in near crowding intensity (0.08 ± 0.03 logMAR). Effects were not due to test-retest learning. Perceptual learning with or without distractors reduces the extent of crowding and improves visual acuity in children with IN. Training with distractors improves near vision more than training with single optotypes. Perceptual learning also transfers to DVA and NVA under uncrowded and crowded conditions and even stereopsis. Learning curves indicated that improvements may be larger after longer training.

Vestibular adaptation to space in monkeys

NASA Technical Reports Server (NTRS)

Dai, M.; Raphan, T.; Kozlovskaya, I.; Cohen, B.

1998-01-01

Otolith-induced eye movements of rhesus monkeys were studied before and after the 1989 COSMOS 2044 and the 1992 to 1993 COSMOS 2229 flights. Two animals flew in each mission for approximately 2 weeks. After flight, spatial orientation of the angular vestibulo-ocular reflex was altered. In one animal the time constant of postrotatory nystagmus, which had been shortened by head tilts with regard to gravity before flight, was unaffected by the same head tilts after flight. In another animal, eye velocity, which tended to align with a gravitational axis before flight, moved toward a body axis after flight. This shift of orientation disappeared by 7 days after landing. After flight, the magnitude of compensatory ocular counter-rolling was reduced by about 70% in both dynamic and static tilts. Modulation in vergence in response to naso-occipital linear acceleration during off-vertical axis rotation was reduced by more than 50%. These changes persisted for 11 days after recovery. An up and down asymmetry of vertical nystagmus was diminished for 7 days. Gains of the semicircular canal-induced horizontal and vertical angular vestibulo-ocular reflexes were unaffected in both flights, but the gain of the roll angular vestibulo-ocular reflex was decreased. These data indicate that there are short- and long-term changes in otolith-induced eye movements after adaptation to microgravity. These experiments also demonstrate the unique value of the monkey as a model for studying effects of vestibular adaptation in space. Eye movements can be measured in three dimensions in response to controlled vestibular and visual stimulation, and the results are directly applicable to human beings. Studies in monkeys to determine how otolith afferent input and central processing is altered by adaptation to microgravity should be an essential component of future space-related research.

Vestibular evoked myogenic potential (VEMP) in patients with acoustic neuromas.

PubMed

Takeichi, N; Sakamoto, T; Fukuda, S; Inuyama, Y

2001-05-01

To study the utility of VEMP (vestibular-evoked myogenic potential) in the diagnosis of acoustic neuromas. Eighteen patients with unilateral acoustic neuromas were subjected to this study. Myogenic potential responding to loud click stimuli was recorded at ipsilateral sternocleidomastoid muscle. A normal range of VEMP was obtained from 20 controls. VEMP responses were compared with both, clinical symptoms and results of caloric tests. Thirteen out of 18 patients showed decreased responses of VEMP at the affected side. VEMP responses seemed to have little relation with dysequilibrium, spontaneous nystagmus, canal paresis and pure-tone hearing. VEMP is useful for detecting dysfunction of inferior vestibular nerve in patients with acoustic neuromas.

The influence of gravitoinertial force level on oculomotor and perceptual responses to Coriolis, cross-coupling stimulation

NASA Technical Reports Server (NTRS)

Dizio, Paul; Lackner, James R.; Evanoff, John N.

1987-01-01

The goal of the present experiment was to determine whether gravitoinertial force magnitude influences oculomotor and perceptual responses to Coriolis cross-coupling stimulation. Blindfolded subjects who were rotating at constant velocity were asked to make standardized head movements during the free-fall and high-force phases of parabolic flight, and the characteristics of their horizontal nystagmus and the magnitude of their experienced self-motion were measured. Both responses were less intense in the free-fall periods than in the high-force periods. These findings suggest that the response to semicircular canal stimulation depends on the background level of gravitoinertial force.

[A case of fibromyalgia treated with medical and autogenic training].

PubMed

Goto, Fumiyuki; Asama, Youji; Nakai, Kimiko

2005-12-01

Fibromyalgia, which is relatively rare, may include symptoms of dizziness, vertigo and tinnitus. Subject was 38 years old woman reporting vertigo and whole body pain. Cochleovestibular function was normal. Pain was gradually intensified during her outpatient clinic and she was admitted. Treatments including intramusclular injection of botulinus toxin and intravenous injection of steroid were applied. Psychological counseling and autogenic training were effective in relieving her pain and vertigo. During her admission, several spells of vertigo occurred but no nystagmus was found. The abnormality in proprioception and neural disintegration may be related to vertigo. Treatment should start as early as possible together with psychological therapy.

Modifications of spontaneous oculomotor activity in microgravitational conditions

NASA Astrophysics Data System (ADS)

Kornilova, L. N.; Goncharenko, A. M.; Polyakov, V. V.; Grigorova, V.; Manev, A.

Investigations on spontaneous oculomotor activity were carried out prior to and after (five cosmonauts) and during space flight (two cosmonauts) on the 3rd, 5th and 164th days of the space flight. Recording of oculomotor activity was carried out by electrooculography on automated data acquisition and processing system "Zora" based on personal computers. During the space flight and after it all the cosmonauts with the eyes closed or open and dark-goggled showed an essential increase of the movements' amplitude when removing the eyes into the extreme positions especially in a vertical direction, occurrence of correcting saccadic movements (or nystagmus), an increase in time of fixing reactions.

Changes of endolymphatic pressure in the semicircular canal of pigeon by caloric stimulation

NASA Astrophysics Data System (ADS)

Wada, Y.; Suzuki, H.; Watanabe, S.

1994-08-01

It gets into difficult to explain the mechanism of caloric nystagmus only by convection theory from results of microgravity experiments. One of the other theories is an occurrence of a relative volume change due to a temperature change. Since the volume change must lead to a pressure change after caloric stimulation, we tried to measure the ampulla pressure of the horizontal semicircular canal in pigeons (Columba livia) using an improved servo micropipette system. The main result was that the ampulla pressure increased by cooling and decreased by heating. The changes of the ampulla pressure depended on the temperature change but were not influenced by the pigeon's head position.

Reversible cerebellar dysfunction associated with ciguatera fish poisoning.

PubMed

Oh, Sun-Young; Kim, Do-Hyung; Seo, Man-Wook; Shin, Byoung-Soo

2012-10-01

Ciguatera-fish poisoning (or ciguatera) is a common but underdiagnosed food-borne illness related to fish consumption that is characterized by nausea, vomiting and neurologic symptoms such as tingling in the fingers or toes. We describe the case of a young man who suffered from diarrhea and abdominal pain after eating raw fish and who also developed severe ataxia with spontaneous downbeat and perverted head-shaking nystagmus. The patient experienced visual fixation suppression failure during the bithermal caloric test and bilateral smooth-pursuit impairment. Oculomotor findings suggested dysfunction of the vestibulocerebellum, especially the flocculus. These findings suggest that both the peripheral and the central nervous systems can be involved in ciguatera. Copyright © 2012 Elsevier Inc. All rights reserved.

MIT-KSC space life sciences telescience testbed

NASA Technical Reports Server (NTRS)

1989-01-01

A Telescience Life Sciences Testbed is being developed. The first phase of this effort consisted of defining the experiments to be performed, investigating the various possible means of communication between KSC and MIT, and developing software and hardware support. The experiments chosen were two vestibular sled experiments: a study of ocular torsion produced by Y axis linear acceleration, based on the Spacelab D-1 072 Vestibular Experiment performed pre- and post-flight at KSC; and an optokinetic nystagmus (OKN)/linear acceleration interaction experiment. These two experiments were meant to simulate actual experiments that might be performed on the Space Station and to be representative of space life sciences experiments in general in their use of crew time and communications resources.

Keratoconus presenting with bilateral simultaneous acute corneal hydrops.

PubMed

Bilgin, Burak; Unal, Betül; Unal, Mustafa; Doğan, Erkan; Cetinkaya, Aslı; Akyol, Mahmut; Yücel, Iclal; Akar, Yusuf; Apaydın, Cemil; Ilhan, Deniz

2013-04-01

To report a case of unknown keratoconus presenting with bilateral simultaneous acute corneal hydrops. Case report. A case of a 12-year-old male patient with Leber congenital amaurosis (LCA) presented with sudden whitening and lacrimation for 2 days in both eyes simultaneously. At the initial examination, there were bilateral acute corneal hydrops, enophthalmic eyes and roving nystagmus. Ultrasonography revealed clear crystalline lenses and attached retina. Initial management consisted of topical hypertonic solutions, steroids and artificial tears. Bilateral simultaneous acute corneal hydrops has not been reported before in the literature. It may be the presenting sign of keratoconus. Copyright © 2012 British Contact Lens Association. Published by Elsevier Ltd. All rights reserved.

Neurologic syndrome associated with homozygous mutation at MAG sialic acid binding site.

PubMed

Roda, Ricardo H; FitzGibbon, Edmond J; Boucekkine, Houda; Schindler, Alice B; Blackstone, Craig

2016-08-01

The MAG gene encodes myelin-associated glycoprotein (MAG), an abundant protein involved in axon-glial interactions and myelination during nerve regeneration. Several members of a consanguineous family with a clinical syndrome reminiscent of Pelizaeus-Merzbacher disease and demyelinating leukodystrophy on brain MRI were recently found to harbor a homozygous missense p.Ser133Arg MAG mutation. Here, we report two brothers from a nonconsanguineous family afflicted with progressive cognitive impairment, neuropathy, ataxia, nystagmus, and gait disorder. Exome sequencing revealed the homozygous missense mutation p.Arg118His in MAG. This Arg118 residue in immunoglobulin domain 1 is critical for sialic acid binding, providing a compelling mechanistic basis for disease pathogenesis.

Unilateral glaucoma in Sotos syndrome (cerebral gigantism).

PubMed

Yen, M T; Gedde, S J; Flynn, J T

2000-12-01

To report a patient with unilateral glaucoma associated with Sotos syndrome. Sotos syndrome (cerebral gigantism) is a disorder of growth and development with characteristic facial changes and normal endocrine function. Ocular manifestations may also include megalocornea, iris hypoplasia, cataracts, megalophthalmos, strabismus, nystagmus, and retinal dystrophy. Case report. A 50 year-old man with the clinical features of Sotos syndrome presented with complaints of decreased vision in the left eye. Ophthalmologic examination revealed bilateral megalocornea, megalophthalmos, iris hypoplasia and transillumination defects, cataracts, and unilateral glaucoma. Intraocular pressure was lowered, and visual field loss was stabilized with topical medications. Sotos syndrome patients should be examined routinely to allow for early detection and treatment of potential ocular problems, including glaucoma.

Gaze holding deficits discriminate early from late onset cerebellar degeneration.

PubMed

Tarnutzer, Alexander A; Weber, K P; Schuknecht, B; Straumann, D; Marti, S; Bertolini, G

2015-08-01

The vestibulo-cerebellum calibrates the output of the inherently leaky brainstem neural velocity-to-position integrator to provide stable gaze holding. In healthy humans small-amplitude centrifugal nystagmus is present at extreme gaze-angles, with a non-linear relationship between eye-drift velocity and eye eccentricity. In cerebellar degeneration this calibration is impaired, resulting in pathological gaze-evoked nystagmus (GEN). For cerebellar dysfunction, increased eye drift may be present at any gaze angle (reflecting pure scaling of eye drift found in controls) or restricted to far-lateral gaze (reflecting changes in shape of the non-linear relationship) and resulting eyed-drift patterns could be related to specific disorders. We recorded horizontal eye positions in 21 patients with cerebellar neurodegeneration (gaze-angle = ±40°) and clinically confirmed GEN. Eye-drift velocity, linearity and symmetry of drift were determined. MR-images were assessed for cerebellar atrophy. In our patients, the relation between eye-drift velocity and gaze eccentricity was non-linear, yielding (compared to controls) significant GEN at gaze-eccentricities ≥20°. Pure scaling was most frequently observed (n = 10/18), followed by pure shape-changing (n = 4/18) and a mixed pattern (n = 4/18). Pure shape-changing patients were significantly (p = 0.001) younger at disease-onset compared to pure scaling patients. Atrophy centered around the superior/dorsal vermis, flocculus/paraflocculus and dentate nucleus and did not correlate with the specific drift behaviors observed. Eye drift in cerebellar degeneration varies in magnitude; however, it retains its non-linear properties. With different drift patterns being linked to age at disease-onset, we propose that the gaze-holding pattern (scaling vs. shape-changing) may discriminate early- from late-onset cerebellar degeneration. Whether this allows a distinction among specific cerebellar disorders remains to be determined.

Long-term evaluation of the effect of middle ear effusion on the vestibular system in children.

PubMed

Pazdro-Zastawny, Katarzyna; Pośpiech, Lucyna; Zatoński, Tomasz

2018-06-01

Otitis media with effusion (OME) is one of the most common clinical conditions in childhood. Fluid accumulation in the middle ear may impact inner ear. The purpose of this random sample cohort study was to investigate whether the past history of middle ear effusion has a long-term negative impact on the vestibular system in children. The study was carried out on 22 children aged 7-15 years who had undergone drainage of the middle ear 5 years before evaluation. The control group consisted of 29 healthy children aged 4-17 years. Vestibular function was examined using sway posturography and electronystagmography (ENG). The stabilogram parameters of the study group and the control group were compared. The field of developed area (FDA) and the average body sway velocity (ASV) were analyzed. Elevated stabilogram parameters of FDA and ASV, both with eyes open and eyes closed, were found in the study group. Statistically significant values (p < 0.05) were present for ASV with eyes open and with eyes closed. The ENG recordings were analyzed in both groups. In the study group, spontaneous nystagmus was observed in 40.9% of the children and positional nystagmus occurred in 63.6% of the children. According to tests, eye tracking test was impaired in 27.3% of cases. Rotatory chair testing revealed asymmetry in 18.2% of the children. The presence of effusion in the middle ear in the past has a negative impact on the vestibular part of the inner ear. Clinicians should be aware of the possible negative impact of middle ear effusion on the vestibular function in children with a history of otitis media with effusion. With seeimingly asymptomatic children clinicians should inquire parents about symptoms of dysequlibrium and imbalance. Copyright © 2018 Elsevier B.V. All rights reserved.

Neurological signs in 23 dogs with suspected rostral cerebellar ischaemic stroke.

PubMed

Thomsen, Barbara; Garosi, Laurent; Skerritt, Geoff; Rusbridge, Clare; Sparrow, Tim; Berendt, Mette; Gredal, Hanne

2016-06-07

In dogs with ischaemic stroke, a very common site of infarction is the cerebellum. The aim of this study was to characterise neurological signs in relation to infarct topography in dogs with suspected cerebellar ischaemic stroke and to report short-term outcome confined to the hospitalisation period. A retrospective multicentre study of dogs with suspected cerebellar ischaemic stroke examined from 2010-2015 at five veterinary referral hospitals was performed. Findings from clinical, neurological, and paraclinical investigations including magnetic resonance imaging were assessed. Twenty-three dogs, 13 females and 10 males with a median age of 8 years and 8 months, were included in the study. The Cavalier King Charles Spaniel (n = 9) was a commonly represented breed. All ischaemic strokes were located to the vascular territory of the rostral cerebellar artery including four extensive and 19 limited occlusions. The most prominent neurological deficits were gait abnormalities (ataxia with hypermetria n = 11, ataxia without hypermetria n = 4, non-ambulatory n = 6), head tilt (n = 13), nystagmus (n = 8), decreased menace response (n = 7), postural reaction deficits (n = 7), and proprioceptive deficits (n = 5). Neurological signs appeared irrespective of the infarct being classified as extensive or limited. All dogs survived and were discharged within 1-10 days of hospitalisation. Dogs affected by rostral cerebellar ischaemic stroke typically present with a collection of neurological deficits characterised by ataxia, head tilt, and nystagmus irrespective of the specific cerebellar infarct topography. In dogs with peracute to acute onset of these neurological deficits, cerebellar ischaemic stroke should be considered an important differential diagnosis, and neuroimaging investigations are indicated. Although dogs are often severely compromised at presentation, short-term prognosis is excellent and rapid clinical improvement may be observed within the first week following the ischaemic stroke.

[Cerebellar infarction in vascular teritorry of arteria cerebelli superior].

PubMed

Savić, Dejan; Savić, Ljiljana

2010-01-01

Cerebellar vascular diseases are focal cerebrovascular diseases in posterior circulation--vertebrobasilar system. The cerebellum is supplied by three main arteries arising from the vertebrobasilar system: arteria cerebelli inferior posterior, arteria cerebelli inferior anterior and arteria cerebelli superior. Cerebelar infarctions are rare but unpredictable disorders. The aim of this study was determination of main risk factors, clinical presentation and prognosis of the cerebellar infarctions in distal vascular teritorry of the arteria cerebelli superior. We evaluated 60 patients hospitalized after acute cerebellar infarction among other hospitalized patients in five year period. In 18 patients computerized tomography demonstrated infarction in distal vascular teritorry of the arteria cerebelli superior. All patients underwent clinical and other diagnostic investigations (computerized tomography, electrocardyography and standard blood tests) and were questioned by phone after finishing hospital treatment. Cerebellar infarcts in distal vascular teritorry of arteria cerebell superior was 30% of all cerebellar infarcts. The most frequent risk factor was hypertension (66.7%). Symptomatology and clinical signs were heterogenous but the most frequent were instability (77.8%), vertigo (72.2%) and vomiting (55.6%) followed by ataxia of the limbs (77.8%) and the body (61.1%), nystagmus (55.6%) and disarthria (33.3%) in clinical presentation. All patients had good recovery in hospital and one year afterwards. Infarctions in distribution of arteria cerebelli superior are rare and have multiple risk factors and various clinical features in majority of other studies as in this one. Mass effects are present in several studies but none in this one which reflects contraversions present in other published investigations. Cerebellar infarctions in vascular teritorry of arteria cerebelli superior have multiple risk factors, mostly heterogenous clinical presentations with predominance of instability, vertigo and vomiting with ataxia of the limbs and the body as well as nystagmus and disarthria in clinical presentation. The outcome and prognosis of disease is good despite the large amount of arteria cerebelli superior vascular teritorry.

Hyperventilation-induced nystagmus in patients with vestibular schwannoma.

PubMed

Califano, Luigi; Iorio, Giuseppina; Salafia, Francesca; Mazzone, Salvatore; Califano, Maria

2015-02-01

To determine the utility of the hyperventilation test (HVT) in the diagnosis of vestibular schwannoma (VS). A retrospective analysis of hyperventilation-induced nystagmus (HVIN) in 45 patients with unilateral VS. A tertiary referral center. Forty-five patients with VS; 30 patients with chronic vestibular neuritis; 20 healthy subjects with normal hearing and without symptoms or a history of vertigo, migraine, or neurological diseases (control group). Audiological and vestibular examination; "side-stream" measurement of end-tidal CO2 pressure (P(EtCO2)) to standardize the procedure; magnetic resonance imaging (MRI) centered on the cerebellopontine angle. An analysis of HVIN, its patterns, and its appearance threshold via the measurement of P(EtCO2) correlations with the tumor size. HVIN was observed in 40 of 45 cases (88.9%) in the schwannoma group and in 12 of 30 cases (40%) in the chronic vestibular neuritis group; HVIN was not observed in the control group (0/20 cases) (p < 0.001). In the schwannoma group, HVIN was evoked at a mean P(EtCO2) value of 16.5 ± 1.15 mm Hg. The hypofunctional labyrinth was identified with high sensibility and specificity through caloric test, head shaking test, and head thrust test. The excitatory pattern, which included HVIN with slow phases that beat toward the hypofunctional side, and the paretic pattern, which included HVIN with slow phases that beat toward the hypofunctional side, were not significantly associated with VS size (19.04 ± 10.56 mm for the excitatory pattern and 19.06 ± 11.01 mm for the paretic pattern). The difference in the VS size in HVIN+ (19.05 ± 10.60 mm) and HVIN- (8.40 ± 2.19 mm) cases was significant (p = 0.009). A 60-second hyperventilation event causes metabolic changes in the vestibular system and reveals a latent vestibular asymmetry. The presence of an excitatory pattern is the major criterion that suggests VS in patients with signs of unilateral vestibular deficit.

Perceptual Learning in Children With Infantile Nystagmus: Effects on Reading Performance.

PubMed

Huurneman, Bianca; Boonstra, F Nienke; Goossens, Jeroen

2016-08-01

Perceptual learning improves visual acuity and reduces crowding in children with infantile nystagmus (IN). Here, we compare reading performance of 6- to 11-year-old children with IN with normal controls, and evaluate whether perceptual learning improves their reading. Children with IN were divided in two training groups: a crowded training group (n = 18; albinism: n = 8; idiopathic IN: n = 10) and an uncrowded training group (n = 17; albinism: n = 9; idiopathic IN: n = 8). Also 11 children with normal vision participated. Outcome measures were: reading acuity (the smallest readable font size), maximum reading speed, critical print size (font size below which reading is suboptimal), and acuity reserve (difference between reading acuity and critical print size). We used multiple regression analyses to test if these reading parameters were related to the children's uncrowded distance acuity and/or crowding scores. Reading acuity and critical print size were 0.65 ± 0.04 and 0.69 ± 0.08 log units larger for children with IN than for children with normal vision. Maximum reading speed and acuity reserve did not differ between these groups. After training, reading acuity improved by 0.12 ± 0.02 logMAR and critical print size improved by 0.11 ± 0.04 logMAR in both IN training groups. The changes in reading acuity, critical print size, and acuity reserve of children with IN were tightly related to changes in their uncrowded distance acuity and the changes in magnitude and extent of crowding. Our findings are the first to show that visual acuity is not the only factor that restricts reading in children with IN, but that crowding also limits their reading performance. By targeting both of these spatial bottlenecks in children with IN, our perceptual learning paradigms significantly improved their reading acuity and critical print size. This shows that perceptual learning can effectively transfer to reading.

[A Retrospective Series of 77 Pediatric Patients with Vertigo at a National Center for Child Health and Development].

PubMed

Goto, Fumiyuki; Suzuki, Noriomi; Hara, Mariko; Tsuchihashi, Nana; Morimoto, Noriko

2015-07-01

The evaluation and management of vertigo in children varies among institutional and medical specialties. The aim of this study was to describe the characteristics of vertigo in children presenting at a national pediatric center. Patients < 16 years old presenting with vertigo to the department of otolaryngology at a national center for child health and development from April 2004 to October 2009 were included (N = 77; 42 males and 35 females; average age, 8.7 ± 3.4 years) in this study. The most common diagnoses were vestibular migraine (VM; N = 21), benign paroxysmal vertigo (BPV; N =16), unilateral vestibulopathy (N = 12), and psychogenic vertigo (N = 8). Significant differences were observed in the frequency of the diagnoses between children aged older and younger than 7 years: BPV was most common in children < 7 years of age (p < 0.01) and VM was most common in ≥ 27 years of age (p < 0.05). Because obtaining adequate information from children for making a correct diagnosis is sometimes difficult, acquiring sufficient information from the parents is important. In addition, getting the parents to record the nystagmus during a vertigo attack with a digital camera or cellular phone can be useful because observing the nystagmus recorded on the video is helpful for making a diagnosis. Furthermore, the parents are participating in their child's care by attempting to record the attack, strengthening the relationship between the parents and the child. The incidence of psychogenic vertigo is low (less than 10%). Therefore, although physicians have recently tended to define the disorder as psychogenic when no objective abnormality is found in a patient, making a diagnosis of psychogenic vertigo is not recommended. Because vertigo can sometimes make a child anxious, delivering the correct diagnosis and treatment at the early stage is important for preventing anxiety in affected children.

Nitric oxide facilitates GABAergic neurotransmission in the cat oculomotor system: a physiological mechanism in eye movement control

PubMed Central

Moreno-López, Bernardo; Escudero, Miguel; Estrada, Carmen

2002-01-01

Nitric oxide (NO) synthesis by prepositus hypoglossi (PH) neurons is necessary for the normal performance of horizontal eye movements. We have previously shown that unilateral injections of NO synthase (NOS) inhibitors into the PH nucleus of alert cats produce velocity imbalance without alteration of the eye position control, both during spontaneous eye movements and the vestibulo-ocular reflex (VOR). This NO effect is exerted on the dorsal PH neuropil, whose fibres increase their cGMP content when stimulated by NO. In an attempt to determine whether NO acts by modulation of a specific neurotransmission system, we have now compared the oculomotor effects of NOS inhibition with those produced by local blockade of glutamatergic, GABAergic or glycinergic receptors in the PH nucleus of alert cats. Both glutamatergic antagonists used, 2-amino-5-phosphonovaleric acid (APV) and 2,3-dihydro-6-nitro-7-sulphamoyl-benzo quinoxaline (NBQX), induced a nystagmus contralateral to that observed upon NOS inhibition, and caused exponential eye position drift. In contrast, bicuculline and strychnine induced eye velocity alterations similar to those produced by NOS inhibitors, suggesting that NO oculomotor effects were due to facilitation of some inhibitory input to the PH nucleus. To investigate the anatomical location of the putative NO target neurons, the retrograde tracer Fast Blue was injected in one PH nucleus, and the brainstem sections containing Fast Blue-positive neurons were stained with double immunohistochemistry for NO-sensitive cGMP and glutamic acid decarboxylase. GABAergic neurons projecting to the PH nucleus and containing NO-sensitive cGMP were found almost exclusively in the ipsilateral medial vestibular nucleus and marginal zone. The results suggest that the nitrergic PH neurons control their own firing rate by a NO-mediated facilitation of GABAergic afferents from the ipsilateral medial vestibular nucleus. This self-control mechanism could play an important role in the maintenance of the vestibular balance necessary to generate a stable and adequate eye position signal. PMID:11927688

Asymmetric vestibular stimulation reveals persistent disruption of motion perception in unilateral vestibular lesions.

PubMed

Panichi, R; Faralli, M; Bruni, R; Kiriakarely, A; Occhigrossi, C; Ferraresi, A; Bronstein, A M; Pettorossi, V E

2017-11-01

Self-motion perception was studied in patients with unilateral vestibular lesions (UVL) due to acute vestibular neuritis at 1 wk and 4, 8, and 12 mo after the acute episode. We assessed vestibularly mediated self-motion perception by measuring the error in reproducing the position of a remembered visual target at the end of four cycles of asymmetric whole-body rotation. The oscillatory stimulus consists of a slow (0.09 Hz) and a fast (0.38 Hz) half cycle. A large error was present in UVL patients when the slow half cycle was delivered toward the lesion side, but minimal toward the healthy side. This asymmetry diminished over time, but it remained abnormally large at 12 mo. In contrast, vestibulo-ocular reflex responses showed a large direction-dependent error only initially, then they normalized. Normalization also occurred for conventional reflex vestibular measures (caloric tests, subjective visual vertical, and head shaking nystagmus) and for perceptual function during symmetric rotation. Vestibular-related handicap, measured with the Dizziness Handicap Inventory (DHI) at 12 mo correlated with self-motion perception asymmetry but not with abnormalities in vestibulo-ocular function. We conclude that 1 ) a persistent self-motion perceptual bias is revealed by asymmetric rotation in UVLs despite vestibulo-ocular function becoming symmetric over time, 2 ) this dissociation is caused by differential perceptual-reflex adaptation to high- and low-frequency rotations when these are combined as with our asymmetric stimulus, 3 ) the findings imply differential central compensation for vestibuloperceptual and vestibulo-ocular reflex functions, and 4 ) self-motion perception disruption may mediate long-term vestibular-related handicap in UVL patients. NEW & NOTEWORTHY A novel vestibular stimulus, combining asymmetric slow and fast sinusoidal half cycles, revealed persistent vestibuloperceptual dysfunction in unilateral vestibular lesion (UVL) patients. The compensation of motion perception after UVL was slower than that of vestibulo-ocular reflex. Perceptual but not vestibulo-ocular reflex deficits correlated with dizziness-related handicap. Copyright © 2017 the American Physiological Society.

Anesthetic Management in Pediatric Patient for Percutaneous Endoscopic Gastrostomy with Mitochondrial Myopathy: Leigh Syndrome

PubMed Central

Kiliç, Ebru Tarikçi; Gerenli, Nelgin; Akdemir, Mehmet Salim; Tastan, Necmi Onur; Atag, Egemen

2018-01-01

Leigh syndrome (LS) is a rare disease mainly affecting the central nervous system due to the abnormalities of mitochondrial energy generation and seen in early childhood with progressive loss of movement, mental abilities, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respiratory failure. Anesthesia and surgery exacerbate the risks of aspiration, wheezing, and breathing difficulties. Tracheal irritability can be stimulated with the efforts of intubation. We report the anesthetic management of a rare case of an 11-year-old boy with a severe form of LS for percutaneous endoscopic gastrostomy insertion. The patient was closely monitored during the procedure and the postoperative period. Carefully chosen anesthetic agents, good pain control, and close monitoring are essential. PMID:29628597

Effect of the stringency of conditions on caloric test results in healthy subjects.

PubMed

Krstulovic, Claudio; Tulsidas Mahtani, Bharti; Atrache Al Attrache, Nabil; Pérez-Garrigues, Herminio

The caloric test is widely used to assess vestibular function, but the conditions in which it is performed can vary. Caloric nystagmus obtained in 57 healthy subjects were compared: 24 subjects studied in ideal conditions and 33 subjects in non-ideal conditions. A statistically significant decrease in the slow phase velocity of the 4 irrigations performed on the subjects in non-ideal conditions was observed. This must be considered, especially in subjects with suspected bilateral involvement. Stringent conditions reduce the risk of misdiagnosis with bilateral deficit. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

Diagnosis of latent forms of labyrinthine affections

NASA Technical Reports Server (NTRS)

Vaslilyeva, V. P.

1980-01-01

Features and significance of individual vestibular symptoms for the diagnosis of latent labyrinthitis and limited forms of labyrinthine affections offering considerable difficulties are discussed. Vestibular symptoms are indistinct. In case of the negative fistular symptom the greatest significance is acquired by the study of posture nystagmus according to the results of electronystagmograms, changes of tonic reactions and statics, as well as data of experimental vestibular tests. The necessity of evaluation of all the vestibular symptoms from the point of view of their vector characteristics and in a complex of evidence obtained by otoneurological examination of the patient is emphasized. Delicate topic and differential diagnosis of vestibular disturbances is of great importance and significance in the choice of the conservative or surgical method of treatment.

Wernicke-Korsakoff syndrome associated with hyperemesis gravidarum.

PubMed

Yoon, Chang-Kyoon; Chang, Moo-Hwan; Lee, Dong-Cho

2005-09-01

The authors hereby describe a case of Wernicke-Korsakoff syndrome with accompanying ocular findings that is caused by hyperernesis gravidarum. We experienced a 27-year-old female at 12 weeks of pregnancy, who visited our clinic because of weight loss, gait disturbance, decreased mentality and dizziness after prolonged vomiting for 2 months. Neurological examination demonstrated ataxia of gait and loss of orientation. Ophthalmologic examination showed decreased visual acuity, upbeat nystagmus, diplopia and retinal hemorrhage. We report a relatively rare case of Wernicke-Korsakoff syndrome with ophthalmic symptoms induced by hyperemesis gravidarum. If a pregnant women has symptoms of severe vomiting along with other ocular findings such as retinal hemorrhage or restricted extraocular movement, one must suspect the diagnosis of Wernicke-Korsakoff syndrome, and should start appropriate treatment immediately.

[How to manage vertigo in adult?

PubMed

Vuong Chaney, Hella; Rohmer, Dominique; Charpiot, Anne

2017-11-01

The interrogation and the clinical examination are critical in the research of vertigo etiology. In the context of vertigo, the vital emergencies are of neurological and vascular origins. It is therefore necessary to be able to identify them quickly and simply. Vertigo is a symptom to relieve and the cause is to be diagnosed. Careful questioning combined with neurological examination, nystagmus study, Halmagyi test and skew deviation search are more reliable than imaging in the first 48hours to detect a stroke. There are 12% false negatives in cerebral MRI and 74% false negative in cerebral CT-scan in the first 48hours of an ischemic stroke. Labyrinth emergencies are infectious labyrinthitis, perilymphatic fistula and aeroembolism of the vestibule. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

On the biological plausibility of Wind Turbine Syndrome.

PubMed

Harrison, Robert V

2015-01-01

An emerging environmental health issue relates to potential ill-effects of wind turbine noise. There have been numerous suggestions that the low-frequency acoustic components in wind turbine signals can cause symptoms associated with vestibular system disorders, namely vertigo, nausea, and nystagmus. This constellation of symptoms has been labeled as Wind Turbine Syndrome, and has been identified in case studies of individuals living close to wind farms. This review discusses whether it is biologically plausible for the turbine noise to stimulate the vestibular parts of the inner ear and, by extension, cause Wind Turbine Syndrome. We consider the sound levels that can activate the semicircular canals or otolith end organs in normal subjects, as well as in those with preexisting conditions known to lower vestibular threshold to sound stimulation.

Unilateral facial paralysis caused by Ramsay Hunt syndrome.

PubMed

Pereira, Flávia P; Guskuma, Marcos H; Luvizuto, Eloá R; Faco, Eduardo F S; Magro-Filho, Osvaldo; Hochuli-Vieira, Eduardo

2011-09-01

The Ramsay Hunt syndrome is a rare disease caused by an infection of the geniculate ganglion by the varicella-zoster virus. The main clinical features of the syndrome are as follows: Bell palsy unilateral or bilateral, vesicular eruptions on the ears, ear pain, dizziness, preauricular swelling, tingling, tearing, loss of taste sensation, and nystagmus. We describe a 23-year-old white woman, who presented with facial paralysis on the left side of the face, pain, fever, ear pain, and swelling in the neck and auricular region on the left side. She received appropriate treatment with acyclovir, vitamin B complex, and CMP nucleus. After 30 days after presentation, the patient did not show any signs or symptoms of the syndrome. At follow-up at 1 year, she showed no relapse of the syndrome.

Effects of optokinetic stimulation induced by virtual reality on locomotion: a preliminary study.

PubMed

Ohyama, Seizo; Nishiike, Suetaka; Watanabe, Hiroshi; Matsuoka, Katsunori; Takeda, Noriaki

2008-11-01

Exposure to a virtual environment for 20 min was sufficient to cause adaptive changes in locomotion in healthy subjects, suggesting that virtual environments might improve locomotor deviation in patients with unilateral labyrinthine defects. Postural and locomotor control in patients with unilateral labyrinthine defects deviates towards the lesion side. The aim of this study was to examine whether active locomotion within a virtual environment can increase the functionality of rehabilitation. We examined the effects of optokinetic stimulation produced by a virtual reality environment on ocular movement and locomotor tracks in 10 healthy subjects. During the 20 min experiment, the mean locomotor deviation and the mean frequency and mean amplitude of optokinetic nystagmus during the last period of the experiment were significantly higher than those during the initial period.

Mental disorders induced by carbamazepine.

PubMed

Mizukami, K; Naito, Y; Yoshida, M; Nakanishi, T; Koizumi, J

1990-03-01

We present here a case with various physical and neuropsychiatric symptoms caused by the administration of carbamazepine. The patient suffering from right ophthalmic neuralgia showed fever, eczema, erythema, lymphoadenopathy, eosinophilia, vomiting, headache, dizziness, nystagmus, and various mental disorders which consisted of emotional instability, personality change, delusions of reference and persecution, depressive state, and hyperventilation syndrome during the administration of carbamazepine. The physical symptoms in the present case were conformable to the side effect of carbamazepine. The mental disorders appeared in a few days from the start of carbamazepine administration and disappeared after the discontinuation of the administration of this drug without antipsychotic therapy and have never relapsed until now. The mental disorders and the physical symptoms were in parallel with their clinical course. This kind of mental disorders induced by carbamazepine has not yet been reported.

Anti-Yo Mediated Paraneoplastic Cerebellar Degeneration Associated with Pseudobulbar Affect in a Patient with Breast Cancer.

PubMed

Martin, Allison N; Dillon, Patrick M; Jones, David E; Brenin, David R; Lapides, David A

2017-01-01

Paraneoplastic cerebellar degeneration (PCD) is a rare anti-Yo mediated paraneoplastic syndromes rarely that is infrequently associated with breast cancer. We present a case of a 52-year-old female presenting with diplopia, gait instability, dysarthria, dysphagia, nystagmus, and, most notably, new onset paroxysmal episodes of uncontrollable crying concerning for pseudobulbar affect (PBA). Serologic testing showed anti-Yo antibodies. The patient was found to have stage IIIA breast cancer as the inciting cause of the paraneoplastic syndrome. The patient was treated with neoadjuvant chemotherapy, modified radical mastectomy, adjuvant Herceptin, and pertuzumab. She was given IVIG for paraneoplastic syndrome, antidepressants, and dextromethorphan-quinidine (Nuedexta), the first FDA-approved therapy for PBA. With multimodality therapy, she demonstrated significant improvement in neurologic and mood symptoms associated with PCD and PBA.

Research on biophysical evaluation of the human vestibular system

NASA Technical Reports Server (NTRS)

Young, L. R.

1974-01-01

The human vestibular function was studied by the combined approach of advanced measurement and mathematical modelling. Fundamental measurements of some physical properties of endolymph and perilymph, combined with nystagmus measurements and fluid mechanical analysis of semicircular canal function furthered the theory of canal mechanical response to angular acceleration, caloric stimulation and relating linear acceleration. The effects of adaptation seen at low frequency angular stimulation were studied and modelled to remove some shortcomings of the torsion pendulum models. Otolith function was also studied experimentally and analytically, leading to a new set of models for subjective orientation. Applications to special problems of space, including the case of rotating spacecraft were investigated and the interaction of visual and vestibular cues and their relation to proprioceptive information was explored relative to postural control.

Neuro-ophthalmologic aspects of multiple sclerosis: Using eye movements as a clinical and experimental tool

PubMed Central

Niestroy, Annette; Rucker, Janet C; Leigh, R John

2007-01-01

Ocular motor disorders are a well recognized feature of multiple sclerosis (MS). Clinical abnormalities of eye movements, early in the disease course, are associated with generalized disability, probably because the burden of disease in affected patients falls on the brainstem and cerebellar pathways, which are important for gait and balance. Measurement of eye movements, especially when used to detect internuclear ophthalmoplegia (INO), may aid diagnosis of MS. Measurement of the ocular following response to moving sinusoidal gratings of specified spatial frequency and contrast can be used as an experimental tool to better understand persistent visual complaints in patients who have suffered optic neuritis. Patients with MS who develop acquired pendular nystagmus often benefit from treatment with gabapentin or memantine. PMID:19668480

Studies of the Ability to Hold the Eye in Eccentric Gaze: Measurements in Normal Subjects with the Head Erect

NASA Technical Reports Server (NTRS)

Reschke, Millard F.; Somers, Jeffrey T.; Feiveson, Alan H.; Leigh, R. John; Wood, Scott J.; Paloski, William H.; Kornilova, Ludmila

2006-01-01

We studied the ability to hold the eyes in eccentric horizontal or vertical gaze angles in 68 normal humans, age range 19-56. Subjects attempted to sustain visual fixation of a briefly flashed target located 30 in the horizontal plane and 15 in the vertical plane in a dark environment. Conventionally, the ability to hold eccentric gaze is estimated by fitting centripetal eye drifts by exponential curves and calculating the time constant (t(sub c)) of these slow phases of gazeevoked nystagmus. Although the distribution of time-constant measurements (t(sub c)) in our normal subjects was extremely skewed due to occasional test runs that exhibited near-perfect stability (large t(sub c) values), we found that log10(tc) was approximately normally distributed within classes of target direction. Therefore, statistical estimation and inference on the effect of target direction was performed on values of z identical with log10t(sub c). Subjects showed considerable variation in their eyedrift performance over repeated trials; nonetheless, statistically significant differences emerged: values of tc were significantly higher for gaze elicited to targets in the horizontal plane than for the vertical plane (P less than 10(exp -5), suggesting eccentric gazeholding is more stable in the horizontal than in the vertical plane. Furthermore, centrifugal eye drifts were observed in 13.3, 16.0 and 55.6% of cases for horizontal, upgaze and downgaze tests, respectively. Fifth percentile values of the time constant were estimated to be 10.2 sec, 3.3 sec and 3.8 sec for horizontal, upward and downward gaze, respectively. The difference between horizontal and vertical gazeholding may be ascribed to separate components of the velocity position neural integrator for eye movements, and to differences in orbital mechanics. Our statistical method for representing the range of normal eccentric gaze stability can be readily applied in a clinical setting to patients who were exposed to environments that may have modified their central integrators and thus require monitoring. Patients with gaze-evoked nystagmus can be flagged by comparing to the above established normative criteria.

Health-related quality of life in patients over sixty years old with benign paroxysmal positional vertigo.

PubMed

Gámiz, Maria J; Lopez-Escamez, Jose A

2004-01-01

Benign paroxysmal positional vertigo (BPPV) is a common cause of dizziness within the geriatric population causing disability. The diagnosis is established by the position-induced rotatory nystagmus, related to the involved ear, demonstrated by the Dix-Hallpike test (DHT). Although the Epley's modified manoeuvre, or particle respositioning manoeuvre (PRM), is an effective treatment for this disorder, its impact on health outcome in patients over 60 years old is unknown. To assess BPPV health-related quality of life in elderly individuals using the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36) and the Dizziness Handicap Inventory Short Form (DHI-S). A prospective study including new cases of BPPV in patients older than 60 years was carried out. The diagnosis was based on the history of recurrent sudden crises of vertigo and a typical positional-induced nystagmus during the DHT. All patients were treated by a single PRM and relapses were investigated a the 30th post-treatment day. DHT was found negative in 82% (23/28) individuals at 30 days. The eight scales of the SF-36 have a good internal consistency reliability in patients with BPPV (Cronbach's alpha > 0.7). The average standardized score for each SF-36 scale was compared with the reference population normative data, showing differences with norms for role physical, body pain, social function, role emotional and mental health. After PRM, patients restored scores to norms, showing a significant increase in role physical (p < 0.05), body pain (p < 0.04), vitality (p < 0.02), social function (p < 0.003) and mental health scores (p < 0.005). DHI-S total score significantly decreased from 17.19 +/- 9.06 (mean +/- SD) at the first day to 9.70 +/- 10.13 at 30 days (p < 0.001). BPPV has a significant impact on health-related quality of life in elderly patients on their emotional and physical states compared to those unaffected. The PRM can restore health-related quality of life in elderly patients with BPPV. Copyright 2004 S. Karger AG, Basel

Effect of gravity on the caloric stimulation of the inner ear

NASA Technical Reports Server (NTRS)

Kassemi, Mohammad; Deserranno, Dimitri; Oas, John G.

2004-01-01

Robert Barany won the 1914 Nobel Prize in medicine for his convection hypothesis for caloric stimulation. Microgravity caloric tests aboard the 1983 SpaceLab 1 mission produced nystagmus results that contradicted the basic premise of Barany's convection theory. In this paper, we present a fluid structural analysis of the caloric stimulation of the lateral semicircular canal. Direct numerical simulations indicate that on earth, natural convection is the dominant mechanism for endolymphatic flow. However, in the microgravity environment of orbiting spacecraft, where buoyancy effects are mitigated, an expansive convection becomes the sole mechanism for producing endolymph motion and cupular displacement. Transient 1 g and microgravity case studies are presented to delineate the different dynamic behaviors of the 1 g and microgravity endolymphatic flows. The associated fluid-structural interactions are also analyzed based on the time evolution of cupular displacements.

Eye instability induced by vestibular stimulation in rabbits.

PubMed

Ferraresi, A; Azzena, G B; Troiani, D

2001-07-03

The slow compensatory phases of the vestibulo-ocular reflex (VOR) in the rabbit tend to drift and the drift reverses the direction. This periodic alternating drift (PAD) has two peculiar characteristics: (1) it is induced by sinusoidal vestibular stimulation in naive animals, being evoked immediately after stimulus onset and persisting after the end of stimulation; (2) the peak velocity and period of the drift are dependent on stimulus amplitude. PAD of the rabbit has strong similarities with PAN, a periodic alternating nystagmus observed in humans with cerbellar disorders and in monkeys after nodulo-uvulectomy, although its peak velocity is smaller. It is hypothesized that PAD is due to a slight instability, caused by vestibular stimulation in darkness, of the cerebellar adaptive loop, which exerts a variable gain control on the time constant of the velocity storage integrator.

Orthodontic Protocol Using Mini-Implant for Class II Treatment in Patient with Special Needs

PubMed Central

Carvalho Ferreira, Fernando Pedrin; de Paula, Eliana de Cássia Molina; Ferreira Conti, Ana Claudia de Castro; Valarelli, Danilo Pinelli; de Almeida-Pedrin, Renata Rodrigues

2016-01-01

Improving facial and dental appearance and social interaction are the main factors for special needs (SN) patients to seek orthodontic treatment. The cooperation of SN patients and their parents is crucial for treatment success. Objective. To show through a case report the satisfactory results, both functional and esthetic, in patients with intellectual disability, congenital nystagmus, and severe scoliosis. Materials Used. Pendulum device with mini-implants as anchorage unit. Results. Improvement of facial and dental esthetics, correction of Class II malocclusion, and no root resorption shown in the radiographic follow-up. Conclusion. Knowing the limitations of SN patients, having a trained team, motivating and counting on the cooperation of parents and patients, and employing quick and low-cost orthodontic therapy have been shown to be the essential factors for treatment success. PMID:27847652

Anti-Yo Mediated Paraneoplastic Cerebellar Degeneration Associated with Pseudobulbar Affect in a Patient with Breast Cancer

PubMed Central

Martin, Allison N.; Jones, David E.; Brenin, David R.; Lapides, David A.

2017-01-01

Paraneoplastic cerebellar degeneration (PCD) is a rare anti-Yo mediated paraneoplastic syndromes rarely that is infrequently associated with breast cancer. We present a case of a 52-year-old female presenting with diplopia, gait instability, dysarthria, dysphagia, nystagmus, and, most notably, new onset paroxysmal episodes of uncontrollable crying concerning for pseudobulbar affect (PBA). Serologic testing showed anti-Yo antibodies. The patient was found to have stage IIIA breast cancer as the inciting cause of the paraneoplastic syndrome. The patient was treated with neoadjuvant chemotherapy, modified radical mastectomy, adjuvant Herceptin, and pertuzumab. She was given IVIG for paraneoplastic syndrome, antidepressants, and dextromethorphan-quinidine (Nuedexta), the first FDA-approved therapy for PBA. With multimodality therapy, she demonstrated significant improvement in neurologic and mood symptoms associated with PCD and PBA. PMID:28377827

Unilateral Vision Loss after a Dental Visit

PubMed Central

Khattab, Mohammed H.; Wiegand, Annette; Storch, Marcus; Hoerauf, Hans; Feltgen, Nicolas

2018-01-01

Intraoral local anesthetics are widely used for performing painless dental treatments; however, in some cases, they may cause ocular complications such as meiosis, diplopia, nystagmus, ophthalmoplegia, ptosis, and amaurosis. Mostly, the symptoms disappear after several hours; rarely, they have a prolonged character. We describe the case of a 38-year-old young man who had reduced vision in the left eye 5 days after having received intraoral local anesthesia. A diagnosis of cilioretinal artery occlusion with optic disc swelling was made. Ten weeks later, the patient's visual acuity had increased to 20/20, and the swelling of the optic disc had subsided. Although various possible mechanisms for ocular complications after intraoral local anesthetic administration were suggested in the literature, the exact etiology remains unclear. In this case, inadvertent intravascular injection is believed to be the cause. PMID:29681838

The vestibular system of the owl

NASA Technical Reports Server (NTRS)

Money, K. E.; Correia, M. J.

1973-01-01

Five owls were given vestibular examinations, and two of them were sacrificed to provide serial histological sections of the temporal bones. The owls exhibited a curious variability in the postrotatory head nystagmus following abrupt deceleration; sometimes a brisk nystagnus with direction opposite to that appropriate to the stimulus would occur promptly after deceleration. It was found also that owls can exhibit a remarkable head stability during angular movement of the body about any axis passing through the skull. The vestibular apparatus in the owl is larger than in man, and a prominent crista neglecta is present. The tectorial membrane, the cupula, and the otolithic membranes of the utricle, saccule, and lagena are all attached to surfaces in addition to the surfaces hearing hair cells. These attachments are very substantial in the utricular otolithic membrane and in the cupula.

Hypomyelination associated with bovine viral diarrhea virus type 2 infection in a longhorn calf.

PubMed

Porter, B F; Ridpath, J F; Calise, D V; Payne, H R; Janke, J J; Baxter, D G; Edwards, J F

2010-07-01

A newborn Longhorn heifer calf presented with generalized tremors, muscle fasciculations, ataxia, and nystagmus. At necropsy, no gross central nervous system lesions were observed. Histologically, the brain and spinal cord had mild to moderate diffuse microgliosis and astrocytosis, minimal nonsuppurative encephalitis, and decreased myelin staining. Ultrastructural examination revealed thinning and absence of myelin sheaths. Various cell types were immunohistochemically positive for bovine viral diarrhea virus (BVDV). Noncytopathogenic BVDV was isolated from the brain and identified as BVDV type 2 by phylogenetic analysis. BVDV-induced hypomyelination is rare and analogous to lesions in neonates infected with border disease and classical swine fever viruses. This is the first documented case of hypomyelination in a calf specifically attributed to BVDV type 2 and the first description of the ultrastructural appearance of BVDV-induced hypomyelination.

Nicergoline facilitates vestibular compensation in aged male rats with unilateral labyrinthectomy.

PubMed

Rampello, L; Drago, F

1999-05-28

The ergoline derivatives, nicergoline (NIC) or dihydroergocristine (DHE) were administered at various doses (0.1, 0.5 and 1 mg/kg) to aged male rats subjected to labyrinth unilateral lesion (LBX). The nystagmus rate appeared to be lower in animals treated with DHE or NIC 1mg/kg than in saline-injected rats, when observed on day 1 and 2 after operation. The number of falls in the rotorod test of LBX animals was decreased by NIC 0.5 or 1 mg/kg at all observation times. This parameter was affected by DHE only at the higher dose. These results suggest that NIC facilitates vestibular compensation of LBX rats. DHE appeared to be less potent in this respect. Since both drugs act on central dopaminergic neurotransmission, it is possible that this neurotransmission may be involved in their mechanism of action.

Differential auditory-oculomotor interactions in patients with right vs. left sided subjective tinnitus: a saccade study

PubMed Central

Lang, Alexandre; Vernet, Marine; Yang, Qing; Orssaud, Christophe; Londero, Alain; Kapoula, Zoï

2013-01-01

Subjective tinnitus (ST) is a frequent but poorly understood medical condition. Recent studies demonstrated abnormalities in several types of eye movements (smooth pursuit, optokinetic nystagmus, fixation, and vergence) in ST patients. The present study investigates horizontal and vertical saccades in patients with tinnitus lateralized predominantly to the left or to the right side. Compared to left sided ST, tinnitus perceived on the right side impaired almost all the parameters of saccades (latency, amplitude, velocity, etc.) and noticeably the upward saccades. Relative to controls, saccades from both groups were more dysmetric and were characterized by increased saccade disconjugacy (i.e., poor binocular coordination). Although the precise mechanisms linking ST and saccadic control remain unexplained, these data suggest that ST can lead to detrimental auditory, visuomotor, and perhaps vestibular interactions. PMID:23550269

[Squirrel monkey--an ideal primate (correction of prmate) model of space physiology].

PubMed

Matsunami, K

1997-06-01

Investigation of the vestibulo-ocular system of the squirrel monkey was reviewed in consideration of space motion sickness (SMS), or which is recently more often termed as space adaptation syndrome (SAS). Since the first launching of the space satellite, Sputnik [correction of Sputonik] in October 1957, many experiments were carried out in biological and medical fields. A various kind of creatures were used as experimental models from protozoa to human beings. Rats and monkeys are most favorite animals, particularly the non-human primate seems to be the one, because of its phylogenetic relatives akin to the human beings. Chimpanzees, rhesus monkeys, pig tailed-monkeys, red-faced monkeys and squirrel monkeys have been used mostly in American space experiments. Russian used rhesus monkeys. Among these, however, the squirrel monkey has an advantage of the small size of the body, ranging from 600- l000g in adult. This small size as a primate is very advantageous in experiments conducted in a narrow room of the space satellite or shuttle because of its space-saving. The squirrel monkey has another advantage to rear easily as is demonstrated to keep it as a pet. Accordingly, this petit animal provides us a good animal model in biological and medical experiments in space craft. The size of the brain of the squirrel monkey is extraordinary large relative to the body size, which is even superior to that of the human beings. This is partly owed to enlargement of the occipito-temporal cortices, which are forced to well develop for processing a huge amount of audio-visual information indispensable to the arboreal habitant to survive in tropical forest. The vestibular system of the squirrel monkey seems to be the most superior as well, when judged from it relative size of the vestibular nuclear complex. Balancing on swinging twigs or jumping from tree to tree developed the capability of this equilibrium system. Fernandez, Goldberg and his collaborators used the squirrel monkey to elucidate functions of the peripheral vestibular system. A transfer function was proposed to explain the behaviors of regular and irregular unit activity of vestibular nerve fibers. The physiologic characteristics of the second order vestibular neuron was investigated in combination of electrophysiological and micro-morphological way, with using WGA-HRP methods, in relation to somato-motor and eye movements. Interconnections between vestibular neurons and cerebellum, interstitial nucleus of Cajal, oculomotor nuclear complex, superior colliculus and cervical spinal cord were elucidated. In physiological field of the vestibular system, the vestibulo-ocular reflex is well studied and results obtained from the squirrel monkey experiments were reviewed. The squirrel monkey, particularly the Bolivian, is a unique animal in that it is vulnerable to motion sickness induced by visual-motion stimulation with phase mismatch of the two stimuli. Experimental results of labyrinthectomy or bilateral ablation of the maculae staticae led to the conclusion that both semicircular and otolith organs are involved in the genesis of space motion sickness. On the other hand, destruction of the area postrema, acknowledged as the vomiting center to chemical stimulants, produced controversial results. However, it must be pointed out that the a human subject underwent to resection of the area postrema, became insensitive to administration of apomorphine, a well known chemical stimulant of vomiting. Finally the experiments in space revealed the presence of at least two origins of caloric nystagmus, that is, attributable to convection and non-convection current of the endolymphatic fluid.

Strabismus and the Oculomotor System: Insights from Macaque Models

PubMed Central

Das, Vallabh E.

2017-01-01

Disrupting binocular vision in infancy leads to strabismus and oftentimes to a variety of associated visual sensory deficits and oculomotor abnormalities. Investigation of this disorder has been aided by the development of various animal models, each of which has advantages and disadvantages. In comparison to studies of binocular visual responses in cortical structures, investigations of neural oculomotor structures that mediate the misalignment and abnormalities of eye movements have been more recent, and these studies have shown that different brain areas are intimately involved in driving several aspects of the strabismic condition, including horizontal misalignment, dissociated deviations, A and V patterns of strabismus, disconjugate eye movements, nystagmus, and fixation switch. The responses of cells in visual and oculomotor areas that potentially drive the sensory deficits and also eye alignment and eye movement abnormalities follow a general theme of disrupted calibration, lower sensitivity, and poorer specificity compared with the normally developed visual oculomotor system. PMID:28532347

A headache not to be sneezed at.

PubMed

Garry, D; Forrest-Hay, A

2009-05-01

A 32-year-old male patient presented to the emergency department (ED) complaining of a headache and vertigo precipitated by sneezing. He had a recent history of neck trauma. Examination revealed horizontal nystagmus and a gait that veered to the left, exacerbated by heel to toe walking. A diagnosis of vertebral artery dissection (VAD) was suspected. A bleed was ruled out in the ED by computerised tomography, after which the patient was loaded with aspirin. The diagnosis was confirmed by magnetic resonance imaging and magnetic resonance angiography. Although optimal treatment for VAD is unknown, the Cervical Artery Dissection in Stroke Study (CADISS) is an ongoing randomised multicentre prospective study comparing antiplatelet therapy with anticoagulation for patients with both carotid artery dissection and VAD. Headache is a very common presentation to the ED and a full neurological examination is essential if rarer causes are not to be missed.

Cerebellar ataxia and epilepsy with anti-GAD antibodies: treatment with IVIG and plasmapheresis

PubMed Central

Georgieva, Zoya; Parton, Matthew

2014-01-01

Glutamic acid decarboxylase autoantibody (GAD-65) catalyses glutamate conversion into γ-aminobutyric acid (GABA) in the central nervous system and in the pancreatic β cells. Antibodies targeting GAD-65 are of uncertain pathogenic significance and occur in stiff person syndrome, cerebellar ataxia, epilepsy, limbic encephalitis and combinations thereof and diabetes mellitus. A 45-year-old man with a cerebellar gait ataxia, dysmetria, nystagmus and mild cerebellar dysarthria was diagnosed with insulin-dependent diabetes mellitus a year after the onset of neurological symptoms. He also developed complex and tonic-clonic seizures, resistant to anticonvulsant medication and deteriorated cognitively. Blood and cerebrospinal fluid serology, and imaging supported the diagnosis of GAD-65 cerebellar ataxia and epilepsy. He was treated with intravenous immunoglobulin and subsequently plasmapheresis. We report the outcome of 3 years of treatment, which resulted in the improvement of cerebellar signs (particularly gait), with some ultimate decline of efficacy. PMID:24419643

Ophthalmologic Findings in Patients with Neuro-metabolic Disorders.

PubMed

Jafari, Narjes; Golnik, Karl; Shahriari, Mansoor; Karimzadeh, Parvaneh; Jabbehdari, Sayena

2018-01-01

We aimed to present the ophthalmic manifestations of neuro-metabolic disorders. Patients who were diagnosed with neuro-metabolic disorders in the Neurology Department of Mofid Pediatric Hospital in Tehran, Iran, between 2004 and 2014 were included in this study. Disorders were confirmed using clinical findings, neuroimaging, laboratory data, and genomic analyses. All enrolled patients were assessed for ophthalmological abnormalities. A total of 213 patients with 34 different neuro-metabolic disorders were included. Ophthalmological abnormalities were observed in 33.5% of patients. Abnormal findings in the anterior segment included Kayser-Fleischer rings, congenital or secondary cataracts, and lens dislocation into the anterior chamber. Posterior segment (i.e., retina, vitreous body, and optic nerve) evaluation revealed retinitis pigmentosa, cherry-red spots, and optic atrophy. In addition, strabismus, nystagmus, and lack of fixation were noted during external examination. Ophthalmological examination and assessment is essential in patients that may exhibit neuro-metabolic disorders.

Myristicin and phenytoin toxicity in an infant

PubMed Central

Sivathanu, Shobhana; Sampath, Sowmya; David, Henry Suresh; Rajavelu, Kulandai Kasthuri

2014-01-01

A developmentally normal infant presented with repeated episodes of afebrile status epilepticus following nutmeg ingestion. He had developed two episodes of afebrile status epilepticus and had received different treatments earlier, but the details of treatment were not available. On admission, he redeveloped convulsions and loading doses of phenytoin, phenobarbitone and midazolam were administered. However, seizures persisted and extrapyramidal movements, nystagmus and visual dysfunction were noted. Iatrogenic phenytoin toxicity was considered and confirmed by drug levels. His symptoms completely disappeared after discontinuation of phenytoin therapy. The initial seizures were attributed to myristicin, an active component of nutmeg, because of the temporal association. However, the subsequent seizures were due to phenytoin toxicity caused by administration of multiple loading doses. This case highlights that nutmeg, a spice, can cause serious toxic effects like status epilepticus. Furthermore, treatment of status epilepticus with phenytoin can cause iatrogenic seizures due to its narrow therapeutic range. PMID:24903724

Neurological abnormalities in the `cri-du-chat' syndrome 1

PubMed Central

Colover, Jack; Lucas, Mary; Comley, J. A.; Roe, A. M.

1972-01-01

An unusual case of the cri-du-chat syndrome is described in a 6½ year old boy, who, as well as attacks of stridor and choking, showed disorders of spatial perception and cerebellar signs in the form of nystagmus, clumsiness of the hands, and ataxia. Pyramidal signs were also present. He was only mildly retarded mentally. Psychological testing showed that he had a severe deficit for number processing, and also constructional apraxia. Surprisingly, his vocabulary was quite good, as was his reading capacity. Chromosome analysis showed a very small deletion of the short arm of the group B chromosome. In infancy this diagnosis may be suspected because of the high-pitched cry and attacks of stridor and choking. In late childhood, when the signs may be only of a neurological disorder, its recognition may be difficult without confirmation from chromosome studies. The neurological features of this disease are reviewed. Images PMID:5084140

Acute necrosis after Gamma Knife surgery in vestibular schwannoma leading to multiple cranial nerve palsies.

PubMed

Kapitza, Sandra; Pangalu, Athina; Horstmann, Gerhard A; van Eck, Albert T; Regli, Luca; Tarnutzer, Alexander A

2016-08-01

We discuss a rare acute complication after Gamma Knife therapy (Elekta AB, Stockholm, Sweden) in a single patient. A 52-year-old woman presented with vertigo, facial weakness and hearing loss emerging 48hours following Gamma Knife radiosurgery for a right-sided vestibular schwannoma. Neurological examination 6days after symptom onset showed right-sided facial palsy, spontaneous left-beating nystagmus and pathologic head-impulse testing to the right. Pure-tone audiogram revealed right-sided sensorineural hearing loss. A diagnosis of acute vestibulocochlear and facial neuropathy was made. Brain MRI demonstrated focal contrast sparing within the schwannoma, likely related to acute radiation necrosis. Acute multiple cranial neuropathies of the cerebellopontine angle after Gamma Knife treatment should raise suspicion of acute tissue damage within the schwannoma and should result in urgent MRI. Treatment with steroids may be considered based on accompanying swelling and edema. Copyright © 2016 Elsevier Ltd. All rights reserved.

[A case of bilateral medial medullary infarction caused by unilateral vertebral artery dissection].

PubMed

Akimoto, Takayoshi; Hara, Makoto; Saito, Mari; Takahashi, Keiko; Kamei, Satoshi

2015-01-01

A 34-year-old man developed right neck pain. Several hours later, he felt numbness of his extremities and presented at our hospital. He developed right hemiparesis and hypoesthesia of the right extremities. A few hours later, upbeat nystagmus and dysarthria appeared along with a sensory disturbance that spread to all extremities, and right hemiparesis progressed to tetraplegia. Brain MR diffusion-weighted images revealed a high-intensity lesion in the bilateral medial medulla oblongata and we diagnosed this bilateral medial medullary infarction. Three dimentional CT angiography revealed dissection of the right VA. We administered intravenous argatroban, edaravone, glycerin and oral clopidogrel. He was assessed as having modified Rankin scale 4 and was transferred to another hospital for rehabilitation on day 30. When the medial medulla oblongata is supplied by the unilateral VA, a unilateral VA dissection can cause bilateral medial medullary infarction.

Acute vestibular syndrome: clinical head impulse test versus video head impulse test.

PubMed

Celebisoy, Nese

2018-03-05

HINTS battery involving head impulse test (HIT), nystagmus, and test of skew is the critical bedside examination to differentiate acute unilateral peripheral vestibulopathy from posterior circulation stroke (PCS) in acute vestibular syndrome (AVS). The highest sensitivity component of the battery has been reported to be the horizontal HIT, whereas skew deviation is defined as the most specific but non-sensitive sign for PCS. Video-oculography-based HIT (vHIT) may have an additional power in making the differentiation. If vHIT is undertaken, then both gain and gain asymmetry should be taken into account as anterior inferior cerebellar artery (AICA) strokes are at risk of being misclassified based on VOR gain alone. Further refinement in video technology, increased operator proficiency and incorporation with saccade analysis will increase the sensitivity of vHIT for PCS diagnosis. For the time being, clinical examination seems adequate in frontline diagnostic evaluation of AVS.

Cognitive deterioration from long-term abuse of dextromethorphan: a case report.

PubMed Central

Hinsberger, A; Sharma, V; Mazmanian, D

1994-01-01

Dextromethorphan (DM), the dextrorotatory isomer of 3-hydroxy-N-methylmorphinan, is the main ingredient in a number of widely available, over-the-counter antitussives. Initial studies (Bornstein 1968) showed that it possessed no respiratory suppressant effects and no addiction liability. Subsequently, however, several articles reporting abuse of this drug have appeared in the literature. The drug is known to cause a variety of acute toxic effects, ranging from nausea, restlessness, insomnia, ataxia, slurred speech and nystagmus to mood changes, perceptual alterations, inattention, disorientation and aggressive behavior (Rammer et al 1988; Katona and Watson 1986; Isbell and Fraser 1953; Devlin et al 1985; McCarthy 1971; Dodds and Revai 1967; Degkwitz 1964; Hildebrand et al 1989). There have also been two reported fatalities from DM overdoses (Fleming 1986). However, there are no reports describing the effects of chronic abuse. This report describes a case of cognitive deterioration resulting from prolonged use of DM. PMID:7803371

[Wolfram syndrome: from definition to molecular bases].

PubMed

Ribeiro, Maria Regina F; Crispim, Felipe; Vendramini, Márcio F; Moisés, Regina S

2006-10-01

Wolfram syndrome (WS) is an autosomal recessive progressive neurodegenerative disorder characterized by diabetes mellitus and optic atrophy. Diabetes insipidus and sensorineural deafness are also noted frequently, explaining the acronym DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy and deafness) by which the syndrome is also referred. Additional manifestations such as atonic bladder, ataxia, nystagmus and predisposition for psychiatric illness may be present. The Wolfram syndrome gene, WFS1, was mapped to chromosome 4p16.1 by positional cloning. It encodes an 890-amino-acid polypeptide named wolframin. Although the wolframin function is still not completely known, its localization to the endoplasmic reticulum suggests it can play a role in calcium homeostasis, membrane trafficking and protein processing. Knowing the cellular function of wolframin is necessary for understanding the pathophysiology of Wolfram syndrome. This knowledge may lead to development of therapies to prevent or reduce the outcomes of WS.

Ataxia and Encephalitis in a Young Adult with EBV Mononucleosis: A Case Report.

PubMed

Hussain, Rashid S; Hussain, Naaz A

2013-01-01

Neurological manifestations of mononucleosis are extremely rare, occurring in about 1% of all cases. However, when they occur, appropriate treatment must be undertaken to ensure appropriate symptomatic management and reduce morbidity. We present the case of a 25-year-old graduate student with weeklong complaints of fever, sore throat, fatigue, nausea, and "dizziness." She later developed increased sleep requirements, ataxia, vertigo, and nystagmus with a positive EBV IgM titer confirming acute infectious mononucleosis. The patient was clinically diagnosed with EBV-associated cerebellitis and encephalitis, displaying neurological and psychiatric impairment commonly seen in postconcussion syndrome. MRI showed no acute changes. She was started on valacyclovir and a prednisone taper, recovering by the end of twelve weeks. Though corticosteroids and acyclovir are not recommended therapy in patients presenting with EBV-associated ataxia, clinicians may want to keep a low threshold to start these medications in case more serious neurological sequelae develop.

Neuropathologic findings in an aged albino gorilla.

PubMed

Márquez, M; Serafin, A; Fernández-Bellon, H; Serrat, S; Ferrer-Admetlla, A; Bertranpetit, J; Ferrer, I; Pumarola, M

2008-07-01

Pallido-nigral spheroids associated with iron deposition have been observed in some aged clinically normal nonhuman primates. In humans, similar findings are observed in neurodegeneration with brain iron accumulation diseases, which, in some cases, show associated mutations in pantothenate kinase 2 gene (PANK2). Here we present an aged gorilla, 40 years old, suffering during the last 2 years of life from progressive tetraparesis, nystagmus, and dyskinesia of the arms, hands, and neck, with accompanying abnormal behavior. The postmortem neuropathologic examination revealed, in addition to aging-associated changes in the brain, numerous corpora amylacea in some brain areas, especially the substantia nigra, and large numbers of axonal spheroids associated with iron accumulation in the internal globus pallidus. Sequencing of the gorilla PANK2 gene failed to detect any mutation. The clinical, neuropathologic, and genetic findings in this gorilla point to an age-related pallido-nigral degeneration that presented PKAN-like neurologic deficits.

Introduction to genetics in ophthalmology, value of family studies

PubMed

Ohba

2000-05-01

This paper reviews the author's personal experience with genetic eye diseases and discusses the significance of family studies in providing key information for the advancement of molecular research. Choroideremia: This disease has long been known as an X-linked progressive tapetoretinal degeneration, but it was first described in Japan in 1974 after finding asymptomatic fundus changes in heterozygous female carriers that are compatible with X chromosomal inactivation. Mutations in the disease-causing gene (REP-1) provide a clue to the diagnosis and pathophysiology of the disease.Leber's Hereditary Optic Neuropathy: The clinical expression is so variable among affected individuals and families that mild optic nerve disease of insidious onset should be differentiated from autosomal dominant optic atrophy. Molecular assessment of mitochondrial DNA leads to a definite diagnosis of the disease, but mitochondrial DNA mutations do not fully account for the clinical manifestation and phenotypic variability of the disease.Norrie Disease: This rare X-linked vitreoretinal dysplasia, characterized by congenital bilateral blindness, was documented in Japan some twenty years ago and the disease has been identified in four unrelated Japanese families. The disease, once diagnosed on the basis of elaborate clinical and familial studies, can now be defined by molecular assessment of the Norrie disease gene.Congenital Nystagmus: A four-generation family was described which presented with autosomal dominantly inherited congenital nystagmus, peripheral corneal opacity, and foveal hypoplasia without any iris tissue malformation. The diagnosis of this family was established by detection of a missense mutation in the paired domain of the PAX 6 gene, hence conforming to a forme fruste of congenital aniridia.Sorsby's Fundus Dystrophy: Two Japanese families with Sorsby's fundus dystrophy showed late-onset retinal dystrophy characterized by submacular hemorrhage and atrophy. Our patients presented with visual loss as late as 50 years of age or older due to macula-confined degenerative changes that were similar in all respects to exudative age-related macular degeneration and showed a novel mutation in the tissue inhibitor of the metalloproteinases-3 gene.Age-Related Macular Degeneration (ARMD): We have studied whether there is any association of candidate polymorphic genes involving xenobiotic or antioxidant metabolism with susceptibility to ARMD. Preliminary results suggest that the genetic polymorphism of microsomal epoxide hydrolase is related to potential risk of ARMD.

[Introduction to genetics in ophthalmology. Value of family studies].

PubMed

Ohba, N

1999-12-01

This paper reviews the author's personal experience with genetic eye diseases and discusses the significance of family studies in providing key information for the advancement of molecular research. CHOROIDEREMIA: This disease has long been known as an X-linked progressive tapetoretinal degeneration, but it was first described in Japan in 1974 after finding asymptomatic fundus changes in heterozygous female carriers that are compatible with X chromosomal inactivation. Mutations in the disease-causing gene (REP-1) provide a clue to the diagnosis and pathophysiology of the disease. LEBER'S HEREDITARY OPTIC NEUROPATHY: The clinical expression is so variable among affected individuals and families that mild optic nerve disease of insidious onset should be differentiated from autosomal dominant optic atrophy. Molecular assessment of mitochondrial DNA leads to a definite diagnosis of the disease, but mitochondrial DNA mutations do not fully account for the clinical manifestation and phenotypic variability of the disease. NORRIE DISEASE: This rare X-linked vitreoretinal dysplasia, characterized by congenital bilateral blindness, was documented in Japan some twenty years ago and the disease has been identified in four unrelated Japanese families. The disease, once diagnosed on the basis of elaborate clinical and familial studies, can now be defined by molecular assessment of the Norrie disease gene. CONGENITAL NYSTAGMUS: A four-generation family was described which presented with autosomal dominantly inherited congenital nystagmus, peripheral corneal opacity, and foveal hypoplasia without any iris tissue malformation. The diagnosis of this family was established by detection of a missense mutation in the paired domain of the PAX 6 gene, hence conforming to a forme fruste of congenital aniridia. SORSBY'S FUNDUS DYSTROPHY: Two Japanese families with Sorsby's fundus dystrophy showed late-onset retinal dystrophy characterized by submacular hemorrhage and atrophy. Our patients presented with visual loss as late as 50 years of age or older due to macula-confined degenerative changes that were similar in all respects to exudative age-related macular degeneration and showed a novel mutation in the tissue inhibitor of the metalloproteinases-3 gene. AGE-RELATED MACULAR DEGENERATION (ARMD): We have studied whether there is any association of candidate polymorphic genes involving xenobiotic or antioxidant metabolism with susceptibility to ARMD. Preliminary results suggest that the genetic polymorphism of microsomal epoxide hydrolase is related to potential risk of ARMD.

Functions of the nucleus of the optic tract (NOT)

PubMed Central

Yakushin, Sergei B.; Gizzi, Martin; Reisine, Harvey; Raphan, Theodore; Büttner-Ennever, Jean; Cohen, Bernard

2007-01-01

Ocular pursuit in monkeys, elicited by sinusoidal and triangular (constant velocity) stimuli, was studied before and after lesions of the nucleus of the optic tract (NOT). Before NOT lesions, pursuit gains (eye velocity/target velocity) were close to unity for sinusoidal and constant-velocity stimuli at frequencies up to 1 Hz. In this range, retinal slip was less than 2°. Electrode tracks made to identify the location of NOT caused deficits in ipsilateral pursuit, which later recovered. Small electrolytic lesions of NOT reduced ipsilateral pursuit gains to below 0.5 in all tested conditions. Pursuit was better, however, when the eyes moved from the contra-lateral side toward the center (centripetal pursuit) than from the center ipsilaterally (centrifugal pursuit), although the eyes remained in close proximity to the target with saccadic tracking. Effects of lesions on ipsilateral pursuit were not permanent, and pursuit gains had generally recovered to 60–80% of baseline after about 2 weeks. One animal had bilateral NOT lesions and lost pursuit for 4 days. Thereafter, it had a centrifugal pursuit deficit that lasted for more than 2 months. Vertical pursuit and visually guided saccades were not affected by the bilateral NOT lesions in this animal. We also compared effects of these and similar NOT lesions on opto-kinetic nystagmus (OKN) and optokinetic after-nystagmus (OKAN). Correlation of functional deficits with NOT lesions from this and previous studies showed that rostral lesions of NOT in and around the pretectal oli-vary nucleus, which interrupted cortical input through the brachium of the superior colliculus (BSC), affected both smooth pursuit and OKN. In two animals in which it was tested, NOT lesions that caused a deficit in pursuit also decreased the rapid and slow components of OKN slow-phase velocity and affected OKAN. It was previously shown that slightly more caudal NOT lesions were more effective in altering gain adaptation of the angular vestibulo-ocular relfex (aVOR). The present findings suggest that cortical pathways through rostral NOT play an important role in maintenance of ipsilateral ocular pursuit. Since lesions that affected ocular pursuit had similar effects on ipsilateral OKN, processing for these two functions is probably closely linked in NOT, as it is elsewhere. PMID:10803412

Three new PAX6 mutations including one causing an unusual ophthalmic phenotype associated with neurodevelopmental abnormalities.

PubMed

Dansault, Anouk; David, Gabriel; Schwartz, Claire; Jaliffa, Carolina; Vieira, Véronique; de la Houssaye, Guillaume; Bigot, Karine; Catin, Françise; Tattu, Laurent; Chopin, Catherine; Halimi, Philippe; Roche, Olivier; Van Regemorter, Nicole; Munier, Francis; Schorderet, Daniel; Dufier, Jean-Louis; Marsac, Cécile; Ricquier, Daniel; Menasche, Maurice; Penfornis, Alfred; Abitbol, Marc

2007-04-02

The PAX6 gene was first described as a candidate for human aniridia. However, PAX6 expression is not restricted to the eye and it appears to be crucial for brain development. We studied PAX6 mutations in a large spectrum of patients who presented with aniridia phenotypes, Peters' anomaly, and anterior segment malformations associated or not with neurological anomalies. Patients and related families were ophthalmologically phenotyped, and in some cases neurologically and endocrinologically examined. We screened the PAX6 gene by direct sequencing in three groups of patients: those affected by aniridia; those with diverse ocular manifestations; and those with Peters' anomaly. Two mutations were investigated by generating crystallographic representations of the amino acid changes. Three novel heterozygous mutations affecting three unrelated families were identified: the g.572T>C nucleotide change, located in exon 5, and corresponding to the Leucine 46 Proline amino-acid mutation (L46P); the g.655A>G nucleotide change, located in exon 6, and corresponding to the Serine 74 Glycine amino-acid mutation (S74G); and the nucleotide deletion 579delG del, located in exon 6, which induces a frameshift mutation leading to a stop codon (V48fsX53). The L46P mutation was identified in affected patients presenting bilateral microphthalmia, cataracts, and nystagmus. The S74G mutation was found in a large family that had congenital ocular abnormalities, diverse neurological manifestations, and variable cognitive impairments. The 579delG deletion (V48fsX53) caused in the affected members of the same family bilateral aniridia associated with congenital cataract, foveal hypolasia, and nystagmus. We also detected a novel intronic nucleotide change, IVS2+9G>A (very likely a mutation) in an apparently isolated patient affected by a complex ocular phenotype, characterized primarily by a bilateral microphthalmia. Whether this nucleotide change is indeed pathogenic remains to be demonstrated. Two previously known heterozygous mutations of the PAX6 gene sequence were also detected in patients affected by aniridia: a de novo previously known nucleotide change, g.972C>T (Q179X), in exon 8, leading to a stop codon and a heterozygous g.555C>A (C40X) recurrent nonsense mutation in exon 5. No mutations were found in patients with Peters' anomaly. We identified three mutations associated with aniridia phenotypes (Q179X, C40X, and V48fsX53). The three other mutations reported here cause non-aniridia ocular phenotypes associated in some cases with neurological anomalies. The IVS2+9G>A nucleotide change was detected in a patient with a microphthalmia phenotype. The L46P mutation was detected in a family with microphthalmia, cataract, and nystagmus. This mutation is located in the DNA-binding paired-domain and the crystallographic representations of this mutation show that this mutation may affect the helix-turn-helix motif, and as a consequence the DNA-binding properties of the resulting mutated protein. Ser74 is located in the PAX6 PD linker region, essential for DNA recognition and DNA binding, and the side chain of the Ser74 contributes to DNA recognition by the linker domain through direct contacts. Crystallographic representations show that the S74G mutation results in no side chain and therefore perturbs the DNA-binding properties of PAX6. This study highlights the severity and diversity of the consequences of PAX6 mutations that appeared to result from the complexity of the PAX6 gene structure, and the numerous possibilities for DNA binding. This study emphasizes the fact that neurodevelopmental abnormalities may be caused by PAX6 mutations. The neuro-developmental abnormalities caused by PAX6 mutations are probably still overlooked in the current clinical examinations performed throughout the world in patients affected by PAX6 mutations.

Three new PAX6 mutations including one causing an unusual ophthalmic phenotype associated with neurodevelopmental abnormalities

PubMed Central

Dansault, Anouk; David, Gabriel; Schwartz, Claire; Jaliffa, Carolina; Vieira, Véronique; de la Houssaye, Guillaume; Bigot, Karine; Catin, Françise; Tattu, Laurent; Chopin, Catherine; Halimi, Philippe; Roche, Olivier; Van Regemorter, Nicole; Munier, Francis; Schorderet, Daniel; Dufier, Jean-Louis; Marsac, Cécile; Ricquier, Daniel; Menasche, Maurice; Penfornis, Alfred

2007-01-01

Purpose The PAX6 gene was first described as a candidate for human aniridia. However, PAX6 expression is not restricted to the eye and it appears to be crucial for brain development. We studied PAX6 mutations in a large spectrum of patients who presented with aniridia phenotypes, Peters' anomaly, and anterior segment malformations associated or not with neurological anomalies. Methods Patients and related families were ophthalmologically phenotyped, and in some cases neurologically and endocrinologically examined. We screened the PAX6 gene by direct sequencing in three groups of patients: those affected by aniridia; those with diverse ocular manifestations; and those with Peters' anomaly. Two mutations were investigated by generating crystallographic representations of the amino acid changes. Results Three novel heterozygous mutations affecting three unrelated families were identified: the g.572T>C nucleotide change, located in exon 5, and corresponding to the Leucine 46 Proline amino-acid mutation (L46P); the g.655A>G nucleotide change, located in exon 6, and corresponding to the Serine 74 Glycine amino-acid mutation (S74G); and the nucleotide deletion 579delG del, located in exon 6, which induces a frameshift mutation leading to a stop codon (V48fsX53). The L46P mutation was identified in affected patients presenting bilateral microphthalmia, cataracts, and nystagmus. The S74G mutation was found in a large family that had congenital ocular abnormalities, diverse neurological manifestations, and variable cognitive impairments. The 579delG deletion (V48fsX53) caused in the affected members of the same family bilateral aniridia associated with congenital cataract, foveal hypolasia, and nystagmus. We also detected a novel intronic nucleotide change, IVS2+9G>A (very likely a mutation) in an apparently isolated patient affected by a complex ocular phenotype, characterized primarily by a bilateral microphthalmia. Whether this nucleotide change is indeed pathogenic remains to be demonstrated. Two previously known heterozygous mutations of the PAX6 gene sequence were also detected in patients affected by aniridia: a de novo previously known nucleotide change, g.972C>T (Q179X), in exon 8, leading to a stop codon and a heterozygous g.555C>A (C40X) recurrent nonsense mutation in exon 5. No mutations were found in patients with Peters' anomaly. Conclusions We identified three mutations associated with aniridia phenotypes (Q179X, C40X, and V48fsX53). The three other mutations reported here cause non-aniridia ocular phenotypes associated in some cases with neurological anomalies. The IVS2+9G>A nucleotide change was detected in a patient with a microphthalmia phenotype. The L46P mutation was detected in a family with microphthalmia, cataract, and nystagmus. This mutation is located in the DNA-binding paired-domain and the crystallographic representations of this mutation show that this mutation may affect the helix-turn-helix motif, and as a consequence the DNA-binding properties of the resulting mutated protein. Ser74 is located in the PAX6 PD linker region, essential for DNA recognition and DNA binding, and the side chain of the Ser74 contributes to DNA recognition by the linker domain through direct contacts. Crystallographic representations show that the S74G mutation results in no side chain and therefore perturbs the DNA-binding properties of PAX6. This study highlights the severity and diversity of the consequences of PAX6 mutations that appeared to result from the complexity of the PAX6 gene structure, and the numerous possibilities for DNA binding. This study emphasizes the fact that neurodevelopmental abnormalities may be caused by PAX6 mutations. The neuro-developmental abnormalities caused by PAX6 mutations are probably still overlooked in the current clinical examinations performed throughout the world in patients affected by PAX6 mutations. PMID:17417613

Keratopathy in congenital aniridia.

PubMed

Mayer, Kristine L; Nordlund, Michael L; Schwartz, Gary S; Holland, Edward J

2003-04-01

Although the most apparent clinical finding in aniridia is the absence of iris tissue, additional ocular structures are often affected. Mutations of the Pax 6 gene, which is important for eye development, have been identified in families with members affected by aniridia. Poor vision in aniridic eyes may be the result of macular hypoplasia, nystagmus, amblyopia, cataracts, glaucoma, and corneal disease, termed aniridic keratopathy. Advances in surgical techniques have improved management of some of the visually disabling manifestations of aniridia, but aniridic keratopathy remains a significant source of visual loss. We have conducted a large, retrospective study of patients with aniridia to gain information about the natural course of aniridic keratopathy. In this paper, we report the results of our study, as well as findings reported in the literature. Penetrating keratoplasty alone has not been a successful treatment for severe stromal scarring, as it does not treat the underlying epithelial causes of corneal disease. However, it has been successful in corneas that have achieved stable epithelium following limbal stem cell transplantation.

Mutations in the unfolded protein response regulator ATF6 cause the cone dysfunction disorder achromatopsia

PubMed Central

Kohl, Susanne; Zobor, Ditta; Chiang, Wei-Chieh; Weisschuh, Nicole; Staller, Jennifer; Menendez, Irene Gonzalez; Chang, Stanley; Beck, Susanne C; Garrido, Marina Garcia; Sothilingam, Vithiyanjali; Seeliger, Mathias W; Stanzial, Franco; Benedicenti, Francesco; Inzana, Francesca; Héon, Elise; Vincent, Ajoy; Beis, Jill; Strom, Tim M; Rudolph, Günther; Roosing, Susanne; den Hollander, Anneke I; Cremers, Frans P M; Lopez, Irma; Ren, Huanan; Moore, Anthony T; Webster, Andrew R; Michaelides, Michel; Koenekoop, Robert K; Zrenner, Eberhart; Kaufman, Randal J; Tsang, Stephen H; Wissinger, Bernd; Lin, Jonathan H

2015-01-01

Achromatopsia (ACHM) is an autosomal recessive disorder characterized by color blindness, photophobia, nystagmus and severely reduced visual acuity. Using homozygosity mapping and whole-exome and candidate gene sequencing, we identified ten families carrying six homozygous and two compound-heterozygous mutations in the ATF6 gene (encoding activating transcription factor 6A), a key regulator of the unfolded protein response (UPR) and cellular endoplasmic reticulum (ER) homeostasis. Patients had evidence of foveal hypoplasia and disruption of the cone photoreceptor layer. The ACHM-associated ATF6 mutations attenuate ATF6 transcriptional activity in response to ER stress. Atf6−/− mice have normal retinal morphology and function at a young age but develop rod and cone dysfunction with increasing age. This new ACHM-related gene suggests a crucial and unexpected role for ATF6A in human foveal development and cone function and adds to the list of genes that, despite ubiquitous expression, when mutated can result in an isolated retinal photoreceptor phenotype. PMID:26029869

First report on the diagnosis and treatment of encephalic and urinary paracoccidioidomycosis in a cat.

PubMed

Gonzalez, Juan F; Montiel, Nestor A; Maass, Rodrigo L

2010-08-01

A male Persian cat was presented with persistent fever, anorexia, weakness, hypopyon, nystagmus, and intention tremors. The hemogram showed severe neutropenia and laboratory analysis on cerebrospinal fluid (CSF) smears revealed abundant yeast cells compatible with Paracoccidioides brasiliensis. Urinalysis demonstrated persistent funguria and an increased urine protein-to-creatinine ratio (UPC) in addition to mild azotemia. Long-term therapy with oral fluconazole was effective in controlling the nervous system signs. Funguria was resolved with subcutaneous administration of diluted amphotericin B in a large volume of saline solution for a period of 12 weeks during the second year after initial diagnosis. Throughout 5 years of treatment, no adverse effects were observed and tolerance to the drugs was normal. Due to development of progressive uremic syndrome the animal was euthanased. To the best of our knowledge, this report is the first clinical case described of a nervous and urinary system infection caused by the P brasiliensis in a cat. Copyright 2010 ISFM and AAFP. Published by Elsevier Ltd. All rights reserved.

Galvanic vestibular stimulation speeds visual memory recall.

PubMed

Wilkinson, David; Nicholls, Sophie; Pattenden, Charlotte; Kilduff, Patrick; Milberg, William

2008-08-01

The experiments of Alessandro Volta were amongst the first to indicate that visuo-spatial function can be altered by stimulating the vestibular nerves with galvanic current. Until recently, the beneficial effects of the procedure were masked by the high levels of electrical current applied, which induced nystagmus-related gaze deviation and spatial disorientation. However, several neuropsychological studies have shown that much weaker, imperceptible currents that do not elicit unpleasant side-effects can help overcome visual loss after stroke. Here, we show that visual processing in neurologically healthy individuals can also benefit from galvanic vestibular stimulation. Participants first learnt the names of eight unfamiliar faces and then after a short delay, answered questions from memory about how pairs of these faces differed. Mean correct reaction times were significantly shorter when sub-sensory, noise-enhanced anodal stimulation was administered to the left mastoid, compared to when no stimulation was administered at all. This advantage occurred with no loss in response accuracy, and raises the possibility that the procedure may constitute a more general form of cognitive enhancement.

Michelangelo's eye disease.

PubMed

Gallenga, P E; Neri, Giampiero; D'Anastasio, Ruggero; Pettorrossi, Vito Enrico; Alfieri, Emilio; Capasso, Luigi

2012-06-01

Charged by the Pope Julius II for painting the Cappella Sistina in Rome (between 1508 and 1512), Michelangelo worked in an elevated scaffolding, in an anomalous position with dyes (including poisoning lead salts) and solvents (such as toxic turpentine) dripping on his face and continuously inhaling, in a dim environment illuminated only with oil lamps and candles, as he described himself and sketched in a sonet addressed to Giovanni da Pistoia. In 1510 he began suffering from eye disease: the main symptom was the necessity to elevate the document he was reading up to the level of his eyes. This defect disappeared few months after he finished painting his masterpiece. We hypothesize that the Michelangelo's eyes disease was a form of acquired and transitory nystagmus induced by the many hours he spent in up gaze, with a skew deviation, a form of ocular tilt reaction resulting from the impairment of spatial sensitivity (inversion illusion) due to the persistence of the artist's head in a horizontal position, looking upward. Copyright © 2012 Elsevier Ltd. All rights reserved.

Severe anemia caused by babesiosis in a maned wolf (Chrysocyon brachyurus).

PubMed

Phair, Kristen A; Carpenter, James W; Smee, Nicole; Myers, Carl B; Pohlman, Lisa M

2012-03-01

An 8-yr-old, captive, spayed, female maned wolf (Chrysocyon brachyurus) developed progressive lethargy and weakness over a 24-hr period. Clinical signs included vomiting, recumbency, horizontal nystagmus, possible blindness, pale icteric mucus membranes, and port-wine colored urine. A complete blood cell count revealed severe anemia (packed cell volume [PCV], 6%) and intraerythrocytic piroplasms consistent with a Babesia species. Polymerase chain reaction testing later confirmed babesiosis. The wolf was treated with imidocarb dipropionate, antibiotics, and fluid therapy. A whole-blood transfusion from a sibling maned wolf also was performed. Despite aggressive treatment, the wolf failed to improve and was euthanized. To the authors' knowledge, this is the first documented case of babesiosis in a captive maned wolf in North America. Surveillance of infectious diseases in captive and wild maned wolf populations should be expanded to include screening for Babesia species. Tick control also should be implemented to prevent and decrease transmission of the disease to this endangered species.

[Epileptic encephalopathy associated with forced normalization after administration of levetiracetam].

PubMed

Kikuchi, Takahiro; Kato, Mitsuhiro; Takahashi, Nobuya; Nakamura, Kazuyuki; Hayasaka, Kiyoshi

2013-09-01

Here we report a case of a 10-year-old female with unclassified epileptic encephalopathy who showed forced normalization after administration of levetiracetam (LEV). She initially presented with intractable tonic and myoclonic seizures that were observed about 10 times a day along with frequent multifocal sharp and slow wave complexes on electroencephalography (EEG). We were forced to decrease the topiramate dose because of the appearance of nystagmus, and her myoclonic seizures became worse. We added LEV (250 mg/day) and her tonic and myoclonic seizures disappeared one day after initiation of LEV administration. However, she showed hyporesponsiveness and akinesia. The disappearance of paroxysmal discharges on EEG confirmed the diagnosis of forced normalization. Despite continuous administration of LEV, tonic and myoclonic seizures relapsed within a month but her psychotic symptoms resolved simultaneously. To the best of our knowledge, this is the first reported case of forced normalization after LEV administration. It should be noted that LEV may cause forced normalization although it can be started at an adequate dosage.