1/11. Genetic factors in human sleep disorders with special reference to Norrie disease, prader-willi syndrome and Moebius syndrome.Sleep-wake problems are common in specific inborn errors of metabolism and structure of the central nervous system. Psychological factors, behavioural difficulties, metabolic disturbances, and widespread rather than focal damage to the nervous system are present in many of these diseases and all influence the sleep-wake cycle. However, a number of conditions cause relatively focal damage to the neuroanatomical substrate of sleeping and waking. These include fatal familial insomnia, with involvement of the prion protein gene on chromosome 20, Norrie disease, the prader-willi syndrome and the Moebius syndrome. The last three important conditions, although rare, are considered in detail in this review. They result in sensory deprivation, hypothalamic and mid-brain damage, and involve the X-chromosome, chromosome 15, and chromosome 13, respectively. These conditions cause a wide variety of sleep disturbance, including parasomnias, daytime sleepiness, and a condition like cataplexy. The place of the relevant gene products in normal sleep regulation needs further exploration.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/11. diagnosis and treatment of a severe psychotic illness in a man with dual severe sensory impairments caused by the presence of Usher syndrome.The present paper reports the case history of a 50-year-old man born with Usher syndrome, who developed a psychotic illness later in life, to illustrate the specific diagnostic problems, and the value of direct observation and a detailed assessment of communication. The subject had had a significant hearing impairment since birth, problems with balance and developed retinitis pigmentosa, leaving him with progressively limited vision in adult life. A pattern of bizarre and aggressive behaviour, and a disintegration in his ability to communicate using signs developed over 3 months. An initial diagnosis of depression was made, but it later became clearer that the subject had developed a psychotic illness. This condition responded well to a combination of antidepressant and antipsychotic medication. The possible association between Usher syndrome and psychotic illness is also discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/11. Can a motion-blind patient reach for moving objects?It has been claimed that the visual brain is organized in two separate processing streams for spatial vision: one for perception and one for action. To determine whether motion vision is also divided into vision for action and for perception we examined the interceptive behaviour of the motion-blind patient LM. The task for LM and three age-matched control subjects was to reach-and-grasp for an object that moved away. Three experiments were conducted to examine the effects on perfomance of target speed (Expt 1), observation time (Expt 2) and visual feedback (Expt 3). As LM is only able to reach for objects which move at 0.5 m/s or less, her performance is inferior to that of controls who can reach for objects moving at 1.0 m/s, but it is better than would be expected from her performance in psychophysical experiments on her motion vision. Kinematic analysis of LM's reaching movements showed that she adapted the speed of her moving hand to the speed of the target but only when full vision was available. In contrast to normal subjects, LM required long observation times and vision of her moving hand to produce successful reaching responses. Thus, the impairment of both perception and action in LM suggests that the motion area MT/V5 is located at an early stage of the extrastriate hierarchy and provides input to both the perception and the action processing streams.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/11. Secondary diurnal enuresis: evaluation of cueing and reinforcement interventions with a sensory-impaired youth.Secondary enuresis consists of sporadic urinary accidents in persons who, otherwise, possess toileting skills. The present study evaluated the behavioural treatment of secondary enuresis displayed by a 19-year-old, developmentally disabled female with multiple sensory impairments. Treatment consisted of external and self-cueing procedures combined with reinforcement for on-toilet urination. As evaluated in a multiple baseline design, intervention resulted in a decrease in wetting incidents and an increase in self-initiated toileting. Treatment effects were maintained through a 7-month follow-up assessment.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/11. Sensory integration in the rehabilitation of blind adults.learning difficulties displayed by the blind adult prompted an investigation of a link with sensory integrative dysfunction. A review of the literature provided evidence that tactile and vestibular deficiencies were prevalent within this population. Through observational assessment, sensory integrative dysfunction was identified in a number of clients and individual sensory integrative treatment programs were initiated. In the three case studies presented, improvements were noted in mobility, activities of daily living, handwriting, and behaviour after six months of treatment. A discussion is provided on those deficit areas responding to sensory integrative treatment.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/11. Persistent behavioural blindness after early visual deprivation and active visual rehabilitation: a case report.Early long lasting binocular deprivation results in behavioural blindness in both man and experimental animals. However, few reported cases show that visual rehabilitation may improve visual behaviour. A 34-year-old man who had experienced 30 years of binocular deprivation due to bilateral cataracts received visual rehabilitation for one year. The rehabilitation included training in eye-hand co-ordination, recognition of objects, evaluation of distance and size, and mobility training. Despite signs of recovery of visual functions the patient never started to use vision in his normal life. The negative outcome of the rehabilitation is partly attributed to the patient's motivational problems and to the relatively short rehabilitation time. Visual rehabilitation may be successful when started immediately after the corrective operation on the eyes when the level of motivation is also high.- - - - - - - - - - ranking = 6keywords = behaviour (Clic here for more details about this article) |
7/11. Conjugate lateral eye movements and cognitive mode: blindness as a control for visually-induced oculomotor effects.The relationship between lateral eye movement directionality and type of cognitive task was investigated in a single blind subject with septo-optic dysplasia. Results from an 80-item test showed that rightward eye movements were significantly associated with verbal tasks and leftward eye movements with spatial tasks. As visual influences on oculomotor behaviour were eliminated, the findings suggested that lateralized eye movements might have arisen as a consequence of asymmetrical activation of cognitive origin triggering the frontal eye field orienting mechanism.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/11. evoked potentials during recovery from blindness recorded serially from an infant and his normally sighted twin.Visual evoked potentials (VEPs) elicited by diffuse field flashes wee recorded from a behaviourally blind infant with his twin as control. The patient was tested at ages 4, 5, 6, 8, 10 and 15 months. In spite of his behavioural blindness, clear VEPs were recorded from the patient at age 4 months, although the wave form was monophasic as contrasted with the multiphasic wave form recorded from his twin at the same age. Latency to first deflection and to first peak were considerably longer for the patient. The patient's VEP wave forM grew progressively more complex with age, paralleling recovery of useful vision. However, the VEP development anticipated behavioural recovery.- - - - - - - - - - ranking = 3keywords = behaviour (Clic here for more details about this article) |
9/11. Tourette's syndrome and anophthalmia in a girl: complex differential diagnosis.This is a report of a 15 year old girl with anophthalmia who met the DSM-III criteria for Tourette's Syndrome (TS). The case presented a complex differential diagnosis with previous diagnoses of behaviour disorder and schizophrenia, complicated by the issues of blindness, pharmacological, and environmental factors. Once the diagnosis was made, and due to intricate biopsychosocial interactions, a comprehensive treatment approach was adopted with good results. The authors comment on the non-existence of studies about the incidence of TS in blind children and recommend the discrimination between the motor behaviour of the tic disorder versus mannerisms associated with blindness. Thus a reasonable degree of suspicion is warranted in the treatment of blind children with severe behavioural disturbances.- - - - - - - - - - ranking = 3keywords = behaviour (Clic here for more details about this article) |
10/11. Visual rehabilitation after long lasting early blindness.A 23-year-old woman, blinded at an early age due to retinopathy of prematurity and cataract was successfully operated. After the operation the patient was behaviourally blind. Visual rehabilitation was started 9 months later and lasted 1 1/2 years. During this time the visual behaviour of the patient improved. Quantitative tests were designed to depict the progress. The functional improvement of the patient is in agreement with the findings of animal studies; binocular deprivation during the critical period of development causes behavioural blindness, which is partially recoverable. The result of this study indicates that the visual rehabilitation facilitates the process of recovery. The progress in visual behaviour is likely to reflect an improvement of the function of the associative systems of the brain.- - - - - - - - - - ranking = 4keywords = behaviour (Clic here for more details about this article) |
| | Next -> |