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Neuroradiological and eye movement correlates in children with intermittent saccade failure: "ocular motor apraxia".

Ocular motor apraxia (OMA) is a clinical sign involving the intermittent inability to initiate saccades, and a failure of quick phases during optokinetic nystagmus (OKN) and vestibular nystagmus (VN). Some patients have no other associated abnormalities (idiopathic), whereas others have a variety of neurological conditions. We quantified the severity of the saccade failure and correlated it with neuro-radiological and other oculomotor findings in 62 children (aged 17 days - 14 years). Saccades, smooth pursuit, OKN and VN were recorded using electrooculography and the extent of "locking up" (absent quick phases during OKN and VN) was measured. Saccades were usually hypometric. Pursuit and OKN gains were normal in the majority of the idiopathic cases but were low in those with other neurological conditions. Twenty-four patients had essentially normal scans, whereas 38 had abnormal scans: Delayed myelination, cerebellar abnormalities (particularly involving the vermis), and agenesis of the corpus callosum were the most common findings. A significant positive correlation was present between increasing neuro-radiological deficits and severity of "locking up" during OKN. Principal component analysis showed that brainstem and cerebellar vermis abnormalities were the main factors involved. A pathophysiological basis of OMA is discussed in the light of animal and clinical studies.

Adolescent↗

[Course of early vestibular compensation after acute labyrinthine lesions. A clinical-experimental longitudinal study].

This study discusses early vestibular compensation in man. Fifty-five patients suffering from acute vestibular lesions were clinically followed up over six months. More than 450 examinations were carried out, using several subtests such as observation of spontaneous, position, and positional nystagmus with Frenzel's glasses, and registrations of pendular rotation test and bilateral bithermal calories using electrooculography. The results were calculated and recorded on graphs to permit estimation of the influence of vestibular compensation on the features examined. In 71%, i.e., in 39 of the 55 patients, the side of hypoexcitability, the direction of spontaneous nystagmus, direction of preponderance, and deviation in Unterberger's stepping test changed from left to right and vice versa during compensation. It was not possible to discover a homogeneous time-course of compensation. The results have to be considered with respect to the evaluation of vestibular findings, especially in preoperative examinations to locate vestibular disturbances. The article proposes a graduated time schedule for follow-up examinations.

Acute Disease↗

Portable and programmable clinical EOG diagnostic system.

Monitoring eye movements is clinically important in diagnosis of diseases of the central nervous system. Electrooculography (EOG) is one method of obtaining such records which uses skin electrodes, and utilizes the anterior posterior polarization of the eye. A new EOG diagnostic system has been developed that utilizes two off-the-shelf portable notebook computers, one projector and simple electronic hardware. It can be operated under Windows 95, 98, NT, and has significant advantages over any other similar equipment, including programmability, portability, improved safety and low cost. Especially, portability of the instrument is extremely important for acutely ill or handicapped patients. The purpose of this paper is to introduce the techniques of computer animation, data acquisition, real time analysis of measured data, and database management to implement a portable, programmable and inexpensive contacting EOG instrument. It is very convenient to replace the present expensive, inflexible and large-sized commercially available EOG instruments. A lot of interesting stimulation patterns for clinical application can be created easily in different shape, time sequence, and colour by programming in Delphi language. With the help of Winstar (a software package that is used to control I/O and interrupt functions of the computer under Windows 95, 98, NT), the I/O communication between two notebook computers and A/D interface module can be effectively programmed. In addition, the new EOG diagnostic system is battery operated and it has the advantages of low noise as well as isolation from electricity. Two kinds of EOG tests, pursuit and saccade, were performed on 20 normal subjects with this new portable and programmable instrument. Based on the test result, the performance of the new instrument is superior to the other commercially available instruments. In conclusion, we hope that it will be more convenient for doctors and researchers to do the clinical EOG diagnosis and basic medical science research by using this new creation.

Adult↗

Childhood blindness at a school for the blind in Riyadh, Saudi Arabia.

PURPOSE: To determine the major causes of eye diseases leading to visual loss and blindness among children attending a school for the blind in Riyadh, Saudi Arabia. METHODS: A total of 217 school children with visual disabilities attending a school for the blind in Riyadh were included. All children were brought to The Eye Center, Riyadh, and had complete ophthalmologic examinations including visual acuity testing, biomicroscopy, ophthalmoscopy, tonometry and laboratory investigations. In addition, some patients were subjected to electroretinography (ERG), electrooculography (EOG), measurement of visual evoked potentials (VEP), and laboratory work-up for congenital disorders. RESULTS: There were 117 male students with an age range of 6-19 years and a mean age of 16 years. In addition, there were 100 females with an age range of 6-18 years and a mean age of 12 years. Of the 217 children, 194 (89%) were blind from genetically determined diseases or congenital disorders and 23 (11%) were blind from acquired diseases. The major causes of bilateral blindness in children were retinal degeneration, congenital glaucoma, and optic atrophy. The most common acquired causes of childhood blindness were infections and trauma. CONCLUSION: The etiological pattern of childhood blindness in Saudi Arabia has changed from microbial keratitis to genetically determined diseases of the retina and optic nerve. Currently, the most common causes of childhood blindness are genetically determined causes. Consanguineous marriages may account for the autosomal recessive disorders. Public education programs should include information for the prevention of trauma and genetic counseling. Eye examinations for preschool and school children are mandatory for the prevention and cure of blinding disorders.

Adolescent↗

Retinal circadian rhythms in humans.

Circadian rhythms in the retina may reflect intrinsic rhythms in the eye. Previous reports on circadian variability in electrophysiological human retinal measures have been scanty, and the results have been somewhat inconsistent. We studied the circadian variation of the electrooculography (EOG), electroretinography (ERG), and visual threshold (VTH) in subjects undergoing a 36h testing period. We used an ultrashort sleep-wake cycle to balance effects of sleep and light-dark across circadian cycles. Twelve healthy volunteers (10 males, 2 females; mean age 26.3 years, standard deviation [SD] 8.0 years, range 19-40 years) participated in the study. The retinal functions and oral temperature were measured every 90 min. The EOG was measured in the light, whereas the ERG and the VTH were measured in the dark. Sleep was inferred from activity detected by an Actillume monitor. The EOG peak-to-peak responses followed a circadian rhythm, with the peak occurring late in the morning (acrophase 12:22). The ERG b-wave implicit time peaked in the early morning (acrophase 06:46). No statistically significant circadian rhythms could be demonstrated in the ERG a-wave implicit time or peak-to-peak amplitude. The VTH rhythm peaked in the early morning (acrophases 07:59 for blue and 07:32 for red stimuli). All retinal rhythms showed less-consistent acrophases than the temperature and sleep rhythms. This study demonstrated several different circadian rhythms in retinal electrophysiological and psychophysical measures of healthy subjects. As the retinal rhythms had much poorer signal-to-noise ratios than the temperature rhythm, these measures cannot be recommended as circadian markers.

Adult↗

The effect of increasing age on the latency for saccadic eye movements.

The latency for saccadic eye movements to a visual stimulus was studied in 59 adults whose ages ranged from 20.7 to 79.5 years. All were free of neurologic disease and drug use. Horizontal eye movements were recorded by electrooculography and the latency from the onset of a peripheral visual stimulus to the onset of a saccadic refixation eye movement was determined. A linear regression analysis revealed a correlation between increasing age and increasing latency for saccadic refixation eye movements. The direct relationship between increasing age and increasing latency for saccadic eye movements is a factor that should be taken into account in eye-movement studies as well as other methodologies such as tachistoscopic studies in which saccadic eye movements play a role in study design.

Adult↗

Differential effects of sleep deprivation on saccadic eye movements.

STUDY OBJECTIVES: This study was designed to show the influence of sleep deprivation on different types of saccadic eye movements. DESIGN: Performance of saccadic eye movements was compared after normal sleep and sleep deprivation in a randomized, within-subjects paradigm. Parameters of voluntary and reflexive saccades were measured before and after experimental nights and after a night of recovery sleep. Additionally, subjects spent 1 adaptation night in the laboratory before the experiments. SETTING: Experiments took place under controlled laboratory conditions. PARTICIPANTS: Fifteen healthy male volunteers (aged 19-30 years). INTERVENTIONS: Each subject participated in 1 night of sleep deprivation followed by a night of recovery sleep and, on another occasion, in 2 successive nights of undisturbed sleep. MEASUREMENTS AND RESULTS: Horizontal prosaccades, antisaccades, and memory-guided saccades were recorded by means of electrooculography. They were analysed semiautomatically with respect to accuracy, peak velocity, and latency. Peak velocity was significantly reduced in all saccade tasks after 1 night of sleep deprivation but recovered after another night of sleep. Latency was prolonged after sleep deprivation only for memory-guided saccades; accuracy showed a decrease after 1 night without sleep only for prosaccades. CONCLUSIONS: Sleep deprivation has a general impairing effect on the peak velocity of saccades, reflecting possible dysfunction at the level of the brainstem reticular formation. Deficits of accuracy and latency point to dysfunction of specific brain sites such as the supplementary eye field and cerebellum, whereas the cardinal functions of the frontal and parietal eye fields were not affected. These results suggest the possibility of measuring fatigue by means of saccadic parameters, especially saccadic peak velocity.

Adult↗

Autosomal dominant vitreoretinochoroidopathy.

BACKGROUND: Autosomal dominant vitreoretinochoroidopathy recently has been described as a condition characterized by peripheral chorioretinal atrophy and areas of hypopigmentation and hyperpigmentation between the equator and the ora serrata circumferentially in the ocular fundus. We describe the clinical features of a family, some members of which have this disorder. This is the first such report of a family outside the United States. METHODS: We examined a family of 15 individuals, seven of whom were affected. RESULTS: The main clinical findings were peripheral pigmentary changes for 360 degrees, with a discrete boundary near the equator. In one patient, a partial vitreous detachment was found that was creating increasing traction to the macula and to the peripheral retina. Vitreous surgery successfully relieved the traction, and vision recovered from 20/100 to 20/25. One patient lost visual acuity at the age of 10 years when complete rhegmatogenous detachment occurred. In two women, a horizontal nystagmus was present showing typical signs of a congenital nystagmus. Results of electrooculography demonstrated a marked reduction of light rise and a clear reduced Arden ratio in one patient. CONCLUSIONS: Autosomal dominant vitreoretinochoroidopathy appears clinically as mainly a peripheral tapetoretinal disease; patients with this disease have been reported in and outside the United States. In addition to the typical peripheral features, significant vitreous traction maculopathy and congenital nystagmus associated with the disease were found.

Adult↗

Gyrate atrophy-like phenotype with normal plasma ornithine.

PURPOSE: To describe the clinical characteristics of a chorioretinal disease with a gyrate atrophy-like phenotype and normal plasma ornithine. METHODS: One family with three men who had progressive chorioretinal disease and three additional patients with simplex cases were examined clinically and with standard electroretinography, electrooculography, and dark adaptometry. RESULTS: In the family, a 70-year-old man and his two sons (39 and 41 years of age) were affected. On ophthalmoscopy, sharply demarcated peripheral patches of retinal pigment epithelium and choroidal atrophy were seen to progress to the posterior pole in the father's eye. In three unrelated men (62, 70, and 80 years of age), chorioretinal atrophy was present in the mid- and far periphery. Visual acuity was normal in the two youngest of all six patients; however, electroretinogram and electrooculogram waves were reduced. Advanced visual field defects and visual acuity loss occurred in the four older patients. Electroretinogram and electrooculogram were reduced, and the dark adaptation thresholds were elevated. In all patients, serum ornithine levels were normal. Ornithine-delta-aminotransferase activity in cultured skin fibroblasts and the apparent Michaelis constant (Km) for ornithine and alpha-ketoglutarate were within the normal range in all patients. CONCLUSIONS: A gyrate atrophy-like phenotype can result from causes other than deficient ornithine-delta-aminotransferase. Its occurrence in three male members in two generations in one family suggests an autosomal dominant inheritance in at least some such patients.

Adult↗

Anterior canal benign paroxysmal positional vertigo: an underappreciated entity.

OBJECTIVE: Evaluate the frequency and characteristics of benign paroxysmal positional vertigo (BPPV) arising from involvement of the anterior semicircular canal (AC) as compared with the posterior canal (PC) and horizontal canal (HC). STUDY DESIGN: Prospective review of patients with BPPV. SETTING: Tertiary referral center. PATIENTS: A total of 260 patients who were evaluated for vertigo were identified as experiencing BPPV. INTERVENTIONS: Standard vestibular assessment including the use of electrooculography (EOG) or video-oculography (VOG) was completed on all patients. Based on EOG/VOG findings, the BPPV origin was attributed to AC, PC, or HC involvement secondary to canalithiasis versus cupulolithiasis. Treatment was performed with canalith repositioning maneuvers (CRMs) appropriate for type of canal involvement. RESULTS: For the 260 patients, the positionally induced nystagmus patterns suggested the canal of origin to be AC in 21.2%, PC in 66.9%, and HC in 11.9%. Cupulolithiasis was observed in 27.3% of the AC, 6.3% of the PC, and 41.9% of the HC patients. Head trauma was confirmed in the history preceding the onset of vertigo in 36.4% of the AC, versus 9.2% of the PC and 9.7% of the HC patients (p < 0.001). The number of CRMs completed to treat the BPPV did not differ between canals involved (1.32 for AC, 1.49 for PC, and 1.34 for HC). CONCLUSION: The direction of subtle vertical-beating nystagmus underlying the torsional component is critical in differentiating AC versus PC origin; EOG/VOG aids in accurate assessment of the vertical component for the diagnosis of canal involvement. AC involvement may be more prevalent than previously appreciated, particularly if the examiner does not appreciate the vertical component of the nystagmus or the diagnosis is made without the assistance of EOG/VOG. Head trauma history is significantly more frequent in AC versus other forms of BPPV, and patients with a history of head trauma should be examined closely for AC involvement. CRM is as successful for treatment of AC BPPV as for other types of BPPV.

Adult↗

Saccadic strategies in children with hemianopia.

Multiple hypometric (undershooting) saccades are generally reported as a compensatory strategy in adults with homonymous hemianopia. However, hypermetric (overshooting) saccades have been reported to develop spontaneously as a beneficial strategy in response to predictable targets. We examined the saccades of 10 children (aged 5 to 16 years) with homonymous hemianopia to determine the type of compensatory eye-movement strategies employed 6 months to 16 years after hemianopia onset. Homonymous hemianopia was identified using perimetry and/or pattern visual evoked potentials and supported with results of neuroimaging. Eye movements were recorded using bitemporal electrooculography. Saccades were elicited to a red light source in a semipredictable paradigm. We found that hypermetria was not a consistent compensatory strategy in our patients. In spite of the predictability of our paradigm and the long follow-up period, multiple hypometric saccades into the blind field appeared to be the preferred strategy.

Adolescent↗

Relative sparing of extraocular muscles in myotonic dystrophy: an electrooculographic study.

We studied 40 patients with myotonic dystrophy (MD) to investigate whether saccadic eye movement (SEM) abnormalities have a central or peripheral origin. SEMs were recorded by electrooculography and analyzed by a computer system. Six patients were followed up to 2-7 years. Slow SEMs were present in 70% of patients, while saccadic latency and accuracy were within normal ranges. Peak saccadic velocity (PSV) did not correlate with disease duration and muscular disability, and showed a significant reduction only in 1 patient during the follow-up. Muscular disability correlated significantly with age and disease duration and worsened in 4 patients over time. The doll's head maneuver elicited vestibular compensatory eye movements with high velocities. These findings suggest that the extraocular muscles are at least partially spared in MD and that supranuclear structures, most likely the burst cells in brainstem reticular formation, may contribute to the slowing of SEMs.

Adolescent↗

Saccade fatigue and response to edrophonium for the diagnosis of myasthenia gravis.

Maximum velocity and amplitude of repetitive ( 1-per-second) 30 degrees saccadic eye movements were quantitatively assessed for 4 minutes before and after intravenous edrophonium chloride as a diagnostic test for myasthenia gravis. Atropine was given initially to suppress muscarinic side effects. Eye movements were recorded by electrooculography and a digital computer identified saccadic eye movements and plotted amplitude-velocity relationships. When compared with control subjects, eleven of twelve patients with proven MG had a significant increase in saccade amplitude and/or maximum velocity after edrophonium chloride. Only three of twelve proven MG patients had clinically apparent extraocular muscle weakness. The initial period of fatigue improved the sensitivity of the test in those patients who began with normal saccade amplitude and maximum velocity. Two of the patients with positive saccade fatigue tests had no change in optokinetic nystagmus amplitude before and after edrophonium chloride. It is concluded that, quantitative assessment of repetitive large angle saccades before and after edrophonium chloride is a sensitive test for extraocular muscle involvement in MG.

Adolescent↗

Combined vitamin A and E therapy prevents retinal electrophysiological deterioration in abetalipoproteinaemia.

Eight patients with abetalipoproteinaemia had the typical ocular, systemic, and laboratory findings of this disease. Combined therapy with vitamins A and E was administered, starting as early as the first day of life and as late as 26 years of age. The patients were followed up for 2-6 years. Electroretinography was undertaken in all cases and electrooculography in some. After initiation of vitamin A and E therapy no progression of disturbed visual function could be detected in any patient. These objective tests of retinal function demonstrated that the combined vitamin A and E therapy may be useful in arresting retinal deterioration in abetalipoproteinaemia.

Abetalipoproteinemia↗

Scanning laser densitometry in visual acuity loss of unknown origin.

AIM: To assess foveal cone photoreceptor function in patients with unexplained loss of central visual acuity. METHODS: Testing of foveal cone photoreceptor function was performed using scanning laser densitometry, colour matching (Rayleigh equation), and pattern electroretinography (ERG). Standard tests included full field ERG, electrooculography, visual evoked potentials, static perimetry, and fluorescein angiography. RESULTS: Decreased foveal cone photopigment density and abnormal pattern ERG were found in three patients. Results of colour matching were not unequivocal. CONCLUSION: Testing of foveal cone photoreceptor function using scanning laser densitometry may determine the location of pathological changes in certain patients with unexplained visual loss.

Adolescent↗

Primary biliary cirrhosis, dark adaptometry, electro-oculography, and vitamin A state.

Twenty five patients with primary biliary cirrhosis were studied for vitamin A state. In nine patients found to have low circulating vitamin A concentrations no abnormality was found on electro-oculography or in dark adaptation. A positive correlation was found between retinol binding protein and vitamin A values (r = +0.88; p less than 0.001) and between serum albumin and vitamin A values (r = +0.75; p less than 0.001). A weaker and negative correlation was found between serum bilirubin (r = -0.47; p less than 0.05) and vitamin A values. Patients with primary biliary cirrhosis should not receive regular parenteral or even oral vitamin A supplementation unless dark adaptometry or electrooculography yields an abnormal result.

Dark Adaptation↗

Suppression of congenital nystagmus.

Suppression of congenital nystagmus by eyelid closure was studied by electrooculography in four cases under various conditions in order to elucidate whether the suppression is caused by blocking of fixation or not. The nystagmus persisted in the light as well as in the dark provided that the eyes were kept open. Frenzel's glasses did not suppress the nystagmus except for one case in whom jerky nystagmus was attenuated. In the other cases, only voluntary lid closure suppressed the nystagmus. Passive lid closure while the patient attempted to keep eyes open did not suppress the nystagmus except in one case. Reflex eyelid closure by glabellar tap did not suppress the nystagmus in spite of the occurrence of Bell's phenomenon. Passive eyelid opening while the patient attempted to keep eyes closed, on the other hand, did not reproduce the nystagmus. The suppression of congenital nystagmus by lid closure, at least in some cases, seems to be related to the voluntary effort to keep eyes closed but not specifically to blocking of fixation.

Adolescent↗

Optokinetic nystagmus in cats with congenital strabismus.

1. Eye movements were recorded in seven innately esotropic cats during monocular and binocular horizontal optokinetic stimulation, using the search coil technique in five cats and electrooculography in two cats. 2. During closed loop measurements in these strabismic cats, slow phases of optokinetic nystagmus (OKN) were characterized by an overall reduced gain when compared with normal controls. In addition, response gain to monocular nasotemporal stimulation was even more reduced than that to temporonasal stimulation, resulting in an increased asymmetry of closed loop gain. 3. During open loop measurements, eye velocity in strabismic cats was very low at all velocities tested. 4. Differential analysis of the symmetry of OKN revealed that all our strabismic cats had a "good" or more symmetric and a "poor" or more asymmetric eye. In addition, when analyzed separately at individual velocities, the symmetry index of the good eye was fairly constant over the velocity range tested. By contrast, the symmetry index of the poor eye dropped dramatically at higher stimulus velocities. 5. To analyze the relationship of OKN symmetry and cortical physiology, we calculated the ratio between the percentage of neurons driven by one eye in the ipsilateral and the contralateral cortical hemisphere. We found a weak correlation between OKN symmetry and this cortical symmetry index (P < 0.05, analysis of variance). 6. In conclusion, slow eye movements in cats with congenital esotropia are characterized by extremely low gain, especially at higher stimulus velocities. In addition, OKN symmetry during monocular stimulation is decreased. Our data suggest that OKN symmetry is weakly correlated with the proportion of binocular neurons in the visual cortex ipsilateral to the stimulated eye. However, OKN characteristics seem to reflect to a higher degree the response properties of neurons in the pretectal nucleus of the optic tract and the dorsal terminal nucleus of the accessory optic system than properties of neurons in the visual cortex.

Animals↗