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[Clinical and polysomnographic features of rapid eye movement sleep behavior disorder].

OBJECTIVE: To evaluate the clinical and polysomnographic features of rapid eye movement sleep behavior disorder (RBD) in Chinese patients. METHODS: Six parasomnic patients, 4 males and 2 females, with the mean onset age of 58 years (range 50 approximately 66 years) and 6 age and sex matched controls, were video-monitored for successive two nights, to record the electroencephalography, electrooculography, electromyography, electrocardiography and nasal airflow. RESULTS: Polysomnographic recordings disclosed an augmented muscle tone, which appeared intermittently or continuously in REM sleep, accompanied by complex behaviors correlated to dream contents observed by video-monitoring, and without seizure activity. All the 6 patients presented with a long history of parasomnia. The average total-sleep time of the patients was 353 +/- 42 minutes, the stage II sleep time was 139 +/- 76 minutes, and sleep efficiency was 74.3% +/- 12.3% lower compared with that of the controls. Pakinsonism occurred in 1 patient 9 years after parasomnia and dementia in 2 patients 8 and 18 years respectively after symptom of RBD. Clonazepam was administered for parasomnia in 3 cases with a favorable response. CONCLUSION: REM sleep without atonia is demonstrated in association with violent movements by video-monitored polysomnography in 6 Chinese patients.

Adult↗

[Macular dystrophies].

Macular dystrophies are a group of hereditary disorders of the macula occurring in children or young adults. The most frequent in France will be presented in detail: Best disease, Stargardt macular dystrophy, cone dystrophy, X-linked retinoschisis, pattern dystrophy, and malattia leventinese. Molecular biology studies have now mapped and identified the genes involved in these macular dystrophies. Analysis of the features of fundus examination will lead to further examinations such as fluorescein angiography, indocyanine green angiography, optical coherent tomography, electroretinography, or electrooculography, in order to confirm the diagnosis. We will also present the differential diagnosis of each of these macular dystrophies.

Adult↗

Effect of aging on saccadic eye movements to visual and auditory targets.

Elderly patients with diseases of the central nervous system often show saccadic disorders. Before these disorders can be called pathological, they must be distinguished from the physiological effects of aging. The purpose of this study was to determine the effect of aging on visual and auditory saccadic eye movements. Ninety healthy volunteers were divided into three groups (younger than 30 years; 30-50 years; older than 50 years), with 30 volunteers in each group. Visual and auditory predictive 15-degree saccades were evoked and recorded with electrooculography. Recorded parameters were peak velocity, duration, and latency. Both stimuli showed increasing latencies with increasing age and a higher peak velocity in the middle group as compared to the oldest group. The latter result was significant only for saccades to visual targets. Duration was almost identical for both patterns and all age groups. Between the age groups, latencies were significantly shorter for the saccades to auditory targets, and no differences in peak velocity occurred. The results stress the importance of an age-related assessment for saccadic parameters. The increasing latency and decreasing peak velocity in elderly people probably result from age-related degenerative changes in central nervous system parts that are involved in the generation of saccadic eye movements. We found no indications of a different effect of aging through either the visual or the auditory pathway for saccadic parameters.

Acoustic Stimulation↗

[Quality of life in patients suffering from choroideremia].

PURPOSE: To direct attention on every-day problems in choroideremia, which are not a barrier in patients occupation. MATERIAL AND METHODS: Two cases of patients with choroideremia which was diagnosed when they were 30 and 54 are described. In spite of widespread visual field changes patients with good visual acuity did not have to change their lifestyles or their jobs (miner and mechanic). Visual acuity, visual field, mesoptometry, adaptometry, electroretinography, electrooculography were done. The mother of the younger patient was also examined. Her fundus of the eye showed typical changes for carriers. In the older patient's family among 41 family members 7 men were affected in 3 generations. Follow-up was 6 years. RESULTS: In ERG rod activity was very low or abolished and cone activity was reduced, more in older patient. In the younger patient visual acuity and mesoptometry were normal, rod adaptation was very low. The patient was able to work as a miner due to good mesopic vision. In follow-up no significant progression of the disease was observed. The older patient had previously been treated for retinitis pigmentosa for a few years. Diagnosis of choroideremia was made when visual acuity of the patient declined. It was impossible to perform mesoptometry and adaptometry because of advanced night blindness. In the dark room the patient found it very difficult to move. In follow-up lowering of the visual acuity, progression of visual field as well as electroretinographic changes occurred. CONCLUSIONS: Patients with choroideremia may be professionally engaged despite of every-day problems connected with widespread scotomas in visual field and nyctalopia. They should not become pensioners too early. The disease progression is faster in older than in younger patients.

Adult↗

[Polish contribution to the electrophysiological studies in ophthalmology. I. Technology and animal studies].

The methods of investigations by means of electroretinography, electrooculography, electronystagmography and electromyography and the use of visual evoked potentials are presented. Described are experimental investigations and the obtained results in animals (frogs, rabbits and vietnamese pigs) by several Polish research workers and contained in ca. 120 works published in the period 1960-1986.

Animals↗

[Electrophysiological recording of nocturnal sleep and the multiple sleep latency test in obstructive sleep apnea syndrome].

Polysomnographic studies were performed in 6 patients with obstructive sleep apnoea syndrome (OSA). The sleep study consisted of: electroencephalography, electromyography, electrooculography, electrocardiography, pulse oximetry and observation of respiration. During day multiple sleep latency tests were performed. In all patients fragmentation of sleep with prevalent stages 1. and 2. of NREM and occasionally deep sleep and REM phase were observed. Concomitantly with the appearance of electrophysiologic sleep stages the muscle tone decreased and episodes of obstructive apnoea occurred. The periods of sleep and apnoea alternated with wakefulness and breathing. In MSLF the mean latency was 3 +/- 2 min. In OSA syndrome episodes of obstructive sleep apnoea cause sleep fragmentation and prevalence of light sleep stages. Excessive daytime somnolence observed in this syndrome is caused by sleep disturbances. MSLT demonstrated pathologic hypersomnolence in OSA syndrome.

Adult↗

[Diagnosis and treatment of ocular myasthenia (author's transl)].

After discussion of the modern concepts of pathophysiology of ocular myasthenia the ocular symptoms such as ptosis and eye muscle palsies are discussed. As important diagnostic sign the Simpson lid fatigue test before and after application of Tensilon is described. For diagnosis of myasthenic eye muscle palsies electrooculography has a special significance especially in connection with the application of Edrophonium, which normalizes myasthenic hypometric saccades and transforms them even in hypermetric saccades. In doubtful cases of eye muscle palsies the electromyogram of the affected muscle in connection with the Edrophonium-test is extremely valuable. With regard to modern treatment apart from cholinesterase inhibitors (Pyridostigmine, Neostigmine) thymectomy, the application of corticosteroids, ACTH and especially also immune suppressive drugs (Imurel etc.) is discussed. Of great significance in ocular myasthenia is the local application of cholinesterase inhibitors like Eserine, Prostigmin or Phospholine Iodide.

Adrenocorticotropic Hormone↗

[Posturography in unilateral peripheral vestibular deficiency].

A thorough study of vestibular function cannot be limited to the evaluation of VOR alone; it must also include the study of VSR which serves to control erect stance under both static and dynamic conditions. In unilateral peripheral vestibular lesions (UVL) computerized stabilometry makes it possible to perform a more accurate evaluation of the VSR complex through the quantification of "postural sway". Furthermore, the diagnostic potential of stabilometry can be further fine-tuned by introducing a sensitization test, in particular the head retroflexion (OCR) and head shaking (OC-HST) tests. In the present study 61 patients with UVL of various etiology (viral, toxic, vascular, idiopathic) were studied. Besides electrooculography, stabilometry was performed under base conditions (eyes open and closed) and with sensitizing tests (OCR, OC-HST). L and S were used as statokinesigram parameters while frequency and oscillation amplitude on the sagittal and transverse planes were used as stabilogram parameters. Of the 61 UVL patients, 47.5% showed VOR compensation while static postural alterations were found in 31.1%; this raised to 50.8% when the sensitization test was introduced. Apparently the compensation phenomena became manifest more quickly for VOR than for VSR. The introduction of the sensitization test, however, markedly reduced this variance. The two sensitization, maneuvers used provided quantitatively equivalent results although in the absence of reciprocal agreement. Agreement was not even recorded between stabilometric HST and the head shaking test. This confirms the presence of two different routes for VOR and VSR. Nonetheless, the introduction of the sensitization test made it possible to identify those "false negative" VSR cases which were really pathological, thus making it possible to properly identify the patients' condition and arrange for adequate treatment.

Adolescent↗

Voluntary modification of the rotatory induced vestibulo-ocular reflex by fixating imaginary targets.

Voluntary enhancement and suppression of the vestibulo-ocular reflex (VOR) was investigated in 12 normal subjects aged 23-74. Eye movements were recorded using DC electrooculography in response to two patterns of horizontal rotation: single-frequency sinusoids in the 0.05-0.8 Hz frequency range; and pseudorandom waveforms consisting of a sum of sinusoids in the 0.05-0.35 Hz range. Subjects were able to significantly alter the gain and phase of their VOR responses by fixating imaginary targets in both patterns of stimulation. The amount of VOR modification decreased as the frequency of stimulation increased but was not correlated with age. In the clinical setting, potential voluntary alteration of the VOR must be considered in judging the normality of a subject's responses.

Adult↗

[Vigilance and beta blockade. The influence of the beta blocker atenolol and other substances on the reaction time of the visual system].

Visual reaction times as a measure of vigilance and of the psycho-physiological condition of subjects was determined after combined physical and mental stress to examine beta-blocker influence. Using the technique of electrooculography 40 subjects aged 25,7 +/- 6 with a mean blood pressure to 126/79 mmHg were measured in a double-blind cross-over design after application of placebo or 50 mg of atenolol (Tenormin) for 3 days. Visual reaction time was defined as the time between display of a peripheral light signal and the start of the eye movement that shifts the direction of gaze from the reference point to the stimulus. The results of the study show, that under these experimental conditions there is a positive effect of beta-blocker medication on vigilance. Findings of other authors are discussed. To prove the sensitivity of the test method in a preliminary study, the effects of the well-described drugs fenetyllinhydrochloride, diazepam, oxazepam and alcohol on visual reaction time were investigated.

Adrenergic beta-Antagonists↗

Respiration during sleep in children.

In 22 children (11 boys and 11 girls), aged 9 to 13 years, respiration was monitored during one night of sleep. No child had a significant history of breathing problems during sleep. Sleep was recorded using standard techniques (electroencephalography, electrooculography, electromyography), and respiration was measured with nasal thermistors and abdominal or thoracic strain gauges. Respiratory pauses (five seconds or longer) were determined for all sleep stages. Respiratory rate was scored only in the first and last sleep cycles and during ten waking minutes before sleep onset. Respiratory rate was significantly affected by wakefulness or stage of sleep: highest in wakefulness and stage 1, lowest in stage 2 of the last sleep cycle. Regularity of respiratory rate showed a similar effect. Variance of respiratory rate was significantly lower in girls than boys. Respiratory pauses during sleep were seen in every child, ranging from 3 to 40 pauses per night (average, 17.2 for boys and 18.0 for girls). Significantly greater numbers of pauses per minute were seen in stage 1 and rapid eye movement (REM) sleep than in stages 2, 3 and 4. The longest respiratory pause was 25 seconds. The conclusion is made that a small number of respiratory pauses during sleep are normal in children of this age.

Adolescent↗

The 1994 Clinical Research Award. A prospective clinical study of the polysomnographic stages of sleep after burn injury.

Although subjective evidence suggests that patients with burns are deprived of sleep, previous clinical studies have been limited to observational data and have not to date included electroencephalographic or polysomnographic recordings. The purpose of this study was to characterize the sleep pattern of patients suffering from thermal injury. Biweekly 24-hour polysomnographic measurements (electromyography, electrooculography, and electroencephalography) were performed with 12 leads. This measuring permitted continuous recording of intrinsic electrical activity in skeletal muscles via chin electrodes, eye movement via outer canthal electrodes, and brain wave activity with the other bipolar electrodes. Determinations were obtained on 11 patients with thermal injuries for a total of 43 24-hour periods. The patients had a mean age of 8.31 +/- 1.5 years (range 1.4 to 16 years), a mean total body surface area burn of 55.1% +/- 16.5% (range 17.5% to 90.5%), and a mean full-thickness burn of 48.5% +/- 8.1% (range 10.5% to 90.5%). Although mean total sleep time was seemingly adequate (625.1 +/- 31.6 min/patient/24 hrs), large aberrations in sleep stage distribution were noted. Significant decreases in stage 3 + 4 and in rapid eye movement (deep sleep) and increases in stages 1 and 2 (light sleep) were noted, suggesting a cycling back to stages 1 or 2 after disruption of sleep. Overall, in 43 runs 40% of the subjects were completely lacking stage 3 + 4, and 19% were missing rapid eye movement during an entire 24-hour run.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[A case of myotonic dystrophy with impairment of smooth pursuit eye movement].

We reported a 36-year-old woman of myotonic dystrophy with impairment of smooth pursuit eye movement. The patient, whose mother was also affected, had psychomotor retardation from childhood. DNA analysis by Southern hybridization technique showed large DNA fragment (14/8.6 kb). Oculomotor limitation and gaze nystagmus were not observed, and saccadic eye movements were normal. In electrooculography, smooth pursuit eye movements were impaired toward both directions, and interrupted by saccades. The caloric test elicited a satisfactory response, but the visual suppression test produced no suppression. CT scan displayed paraventricular low density area, and MRI showed the multiple foci of high signal intensity in the white matter on T2-weighted images. No changes were detected on CT or MRI in the brainstem and cerebellum. The lesions in the brain are considered to be correlated with smooth pursuit impairment.

Adult↗

[The characteristics of the structure of night sleep in patients who have had a stroke].

The aim of the study was investigation of the structure of the night sleep in relation to the disease stage and location of the destructive focus. 18 patients with ischemic stroke (in the right hemisphere-8 cases, in the left one-6 persons, with brain stem location-4 patients) were observed as well as 5 practically healthy individuals. The diagnosis was verified by computer tomography in all cases. Clinical neurological investigation and polygraphy of night sleep (electroencephalography, electrooculography, electromyography) were performed. Analysis of sleep parameters was carried out according to program elaborated by the Center of Sleep Research of Sechenov Moscow Medical Academy. This program includes the analysis of segmental sleep structure in addition to standard parameters. The sleep quality was estimated by Sleep Index (Slind) according to the degree of intactness or destruction of its structure. Rude disorganization of night sleep structure was revealed in the patients, including disturbances of mechanisms of the whole sleep organisation, as well as generation and maintenance of its separate stages. The most rude disorders of sleep in patients with ischemic stroke were connected with location of ischemic focus in right hemisphere and in medial, deep structures.

Brain↗