The inter-relationships between cerebral visual impairment, autism and intellectual disability.

From birth, vision guides our movement, facilitates social interaction and accords recognition and understanding of the environment. In children, vision underpins development of these skills, and is crucial for typical development. Deficits in visual processing may lead to impairment of cognitive, motor, and social development, placing children at risk of developing features of autism. Severe early onset visual dysfunction accords the greatest risk. Cerebral Visual Impairment (CVI) can lead to disorders of cognitive and social development that resemble Autism Spectrum Disorder (ASD). Similarly, children who appear primarily affected by cognitive and social developmental disorders, can manifest a range of visual and perceptual deficits that may be contributory to their disorder. This dual perspective highlights the need for links between impaired vision and neurodevelopmental disorders to be identified and acted upon by means of applying appropriate social and educational strategies. There is good evidence to show that targeted systematic screening for visual and perceptual impairments, and implementation of long-term management approaches, is now required for all at risk children.

[Children with high potential and difficulties: Contributions of clinical research]. / Les enfants à haut potentiel en difficulté : apports de la recherche clinique.

We have been sensitized to children with high intellectual potential (HIP) having difficulties given the number of children consulting in our outpatient medico-psychological centres for scholastic problems (possibly leading to school failure), anxiety disorders or behavioral disorders such as attention deficit/hyperactivity disorder (ADHD), and in which a high intellectual potential was discovered during psychological assessments. It is the contrast, and more precisely the paradox, between the high intellectual potential of these children and their scholastic difficulties (including school failure), and the psychic suffering expressed by some of them, which led us to question, challenge and propose therapeutic and educational care adapted to these children. It is in this context that we created in December 2005 the CNAHP (National Center for Assistance to High Potential children and adolescents) which is a public centre integrated into the hospital-university department of child and adolescent psychiatry at Rennes. It is noteworthy that not all children with HIP have difficulties, and children with school failure or behavioral problems are not always children with HIP. However, it is necessary not to minimize the problem raised by children with HIP with difficulties by ignoring its frequency or by considering that these children are "intelligent" enough to manage by themselves and do not need to be helped, whereas some of them can show school failure and even be de-scholarized. Indeed, based on the definition of the World Health Organization (WHO) of an intellectual Quotient (IQ) above 130 (level corresponding to a statistical threshold), the frequency of children with HIP represents 2.3% of the population of schoolchildren aged 6 to 16. The frequency is therefore not so rare. However, it remains to be determined by French epidemiological studies what is the actual frequency of children with difficulties within a population of children with HIP. The analysis of the CNAHP research data from a clinical population (children with HIP consulting for difficulties) highlights that children with HIP can show major school problems (including school failure, defined here as having or foreseeing repetition of a grade), which corresponds to 7.5% of 611 children with HIP consulting at the CNAHP) and socioemotional problems (emotional regulation disorders) in relation to their high intellectual potential. In particular, anxiety disorders were the most frequent psychiatric disorders observed in this population (40.5%) and were significantly associated with high verbal potential. This significant association requires further studies to avoid establishing a simplistic unidirectional and reductive linear cause-effect relationships. Indeed, a high verbal potential can elicit and/or reinforce anxiety-producing representations, but anxiety disorders may also lead to a defensive over investment of verbal language. The results are discussed in this article and suggest that scholastic and/or psychological difficulties encountered by some children with HIP can be related to their high intellectual potential. It is necessary to develop therapeutic and educational care adapted to these children from a better understanding, based on research results, of their possible difficulties but also cognitive abilities. Even when children with HIP have scholastic and/or psychological difficulties, some of their cognitive skills can be preserved contrary to appearances, with for example, as seen in the CNAHP results, excellent attentional capacities shown by cognitive tests contrasting with behavioral attention deficit reported by parents. These skills are important to identify as they are resources which support the therapeutic and educational project. It is probably through an articulation among professionals from national education, health and research, in alliance with the family (parents, child, and siblings), that advances will be made. In the same way that professionals have been interested in children with intellectual disabilities, it is important to be concerned by children with HIP and difficulties located at the other end of the continuum. It is a question of ethics which concerns both caregivers and teachers. It is also a societal issue that concerns all of us given that the expression of high intellectual and creative potential in children may be essential to the societal development of innovative strategies and each nation's future. Finally, the discussion can be extended to all children, independent of their potential. What we learn from children with HIP and difficulties can be applied to each child: it is important at family, school and societal levels to facilitate the expression of the potential of children, to value their skills, and to help them to remove possible inhibitions of their potential based on individualized projects. The acceptance of singularity and differences in children can contribute to tolerance and the development of creativity, in the interest of the subject and of society.

Effects of prenatal stress on fetal and child development: a critical literature review.

Many studies have examined effects of prenatal stress on pregnancy and fetal development, especially on prematurity and birthweight, and more recently long-term effects on child behavioral and emotional development. These studies are reviewed and their limitations are discussed with regard to definitions (including the concepts of stress and anxiety), stress measurements, samples, and control for confounds such as depression. It appears necessary to assess individual stress reactivity prospectively and separately at each trimester of pregnancy, to discriminate chronic from acute stress, and to take into consideration moderator variables such as past life events, sociocultural factors, predictability, social support and coping strategies. Furthermore, it might be useful to examine simultaneously, during but also after pregnancy, stress, anxiety and depression in order to understand better their relationships and to evaluate their specific effects on pregnancy and child development. Finally, further research could benefit from an integrated psychological and biological approach studying together subjective perceived stress and objective physiological stress responses in pregnant women, and their effects on fetal and child development as well as on mother-infant interactions.

[Cortical blindness]. / La cécité corticale.

Cortical blindness refers to a visual loss induced by a bilateral occipital lesion. The very strong cooperation between psychophysics, cognitive psychology, neurophysiology and neuropsychology these latter twenty years as well as recent progress in cerebral imagery have led to a better understanding of neurovisual deficits, such as cortical blindness. It thus becomes possible now to propose an earlier diagnosis of cortical blindness as well as new perspectives for rehabilitation in children as well as in adults. On the other hand, studying complex neurovisual deficits, such as cortical blindness is a way to infer normal functioning of the visual system.

Sustained motion perception deficit following optic neuritis: Behavioral and cortical evidence.

OBJECTIVE: To assess the recovery process in patients after an acute optic neuritis (ON) attack, comparing static and dynamic visual functions. METHODS: In this prospective controlled study, 21 patients with unilateral, first-ever ON were followed over the course of 1 year. Standard visual tests, visual evoked potentials, and optical coherence tomography were assessed repeatedly. In addition, we developed a novel set of motion perceptual tasks to test dynamic visual deficits. fMRI examinations were performed to study the neuronal correlates for the behavioral findings. RESULTS: Four months after the acute phase, the affected eyes had returned to normal performance levels in the routine visual testing. However, motion perception remained impaired throughout the 12-month period. In agreement with the clinical findings, fMRI studies showed recovery in cortical activation during static object recognition, as opposed to sustained deficit in tasks that require motion perception. CONCLUSIONS: Sustained motion perception deficit following ON may explain the continued visual complaints of patients long after recovery of visual acuity. Cortical activation patterns suggest that if plastic processes in higher visual regions contribute to the recovery of vision, this may be limited to static visual functions. Alternatively, cortical activation may reflect the visual percept (intact for visual acuity and impaired for motion perception), rather than demonstrating plastic processes. We suggest that motion perception should be included in the routine ophthalmologic tests following ON.

[Presentation of an assessment battery for visual mental imagery and visual perception]. / Normalisation d'une batterie d'évaluation de l'imagerie mentale visuelle et de la perception visuelle.

INTRODUCTION: The relationship between visual perception and visual mental imagery are at the center of a lively theoretical debate between those postulating common neurocognitive processes between perception and imagery and those who emphasize the differences between these two entities. Neuropsychology can make an important contribution to this debate, by assessing associations and dissociations between perceptual and imaginal deficits in patients with brain damage. However, currently there is no standardized test battery available for such assessments. MATERIAL AND METHODS: Here we present a battery of paper-and-pencil tests assessing different domains of visual mental imagery and visual perception abilities: object form and color, animals, orthographic material, numbers, faces, and space. We also explored the effects of age, educational level and gender on performance on a group of 103 participants free of neurological damage. RESULTS: The battery includes two parts: one composed of 14 tests assessing mental imagery and the second part composed of eight tests assessing the abilities of visual perception. We calculated the correlations between the tests, and found that, with the exception of orthographic material, there were generally poor correlations between imagery and perceptual tests. CONCLUSION: This result seems inconsistent with hypotheses postulating a strict correspondence between perceptual and imagery abilities.

[Unilateral spatial neglect: 30 years of research, discoveries, hope, and (especially) questions]. / La négligence spatiale unilatérale : trente ans de recherches, de découvertes, d'espoirs et (surtout) de questions.

Unilateral spatial neglect (USN) was defined 30 years ago as "a failure to report, respond, or orient to stimuli that are presented contralateral to a brain lesion, provided that this failure is not due to elementary sensory or motor disorders" by Heilman and Valenstein (1979). Even though this definition still holds, the last 30 years have been characterized by a profusion of clinical descriptions, neuroanatomical investigations and theoretical models of neglect. This article summarizes the wealth of neuroanatomical, clinical and experimental data concerning USN, by focusing on attentional and spatial deficits. Finally, some perspectives on neglect research are outlined.

Brain networks of spatial awareness: evidence from diffusion tensor imaging tractography.

Left unilateral neglect, a dramatic condition which impairs awareness of left-sided events, has been classically reported after right hemisphere cortical lesions involving the inferior parietal region. More recently, the involvement of long range white matter tracts has been highlighted, consistent with the idea that awareness of events occurring in space depends on the coordinated activity of anatomically distributed brain regions. Damage to the superior longitudinal fasciculus (SLF), linking parietal to frontal cortical regions, or to the inferior longitudinal fasciculus (ILF), connecting occipital and temporal lobes, has been described in neglect patients. In this study, four right-handed patients with right hemisphere strokes underwent a high definition anatomical MRI with diffusion tensor imaging (DTI) sequences and a pencil and paper neglect battery of tests. We used DTI tractography to visualise the SLF, ILF and the inferior fronto-occipital fasciculus (IFOF), a pathway running the depth of the temporal lobe, not hitherto associated with neglect. Two patients with cortical involvement of the inferior parietal and superior temporal regions, but intact and symmetrical fasciculi, showed no signs of neglect. The other two patients with signs of left neglect had superficial damage to the inferior parietal cortex and white matter damage involving the IFOF. These findings suggest that superficial damage to the inferior parietal cortex per se may not be sufficient to produce visual neglect. In some cases, a lesion to the direct connections between ventral occipital and frontal regions (ie, IFOF) may contribute to the manifestation of neglect by impairing the top down modulation of visual areas from the frontal cortex.

Social and psycho-intellectual outcome following radical removal of craniopharyngiomas in childhood. A prospective series.

BACKGROUND: A prospective study on childhood craniopharyngiomas (CPs) was conducted from 1994 to 1998 to appreciate the pre- and postoperative clinical, endocrine, mental, and intellectual status of the patients and to determine the incidence and severity of the postoperative hypothalamic syndrome. METHODS: The series included 14 consecutive CPs. Twelve were retrochiasmatic and intraventricular, and two were partly prechiasmatic and extraventricular. All were treated with the aim of "total" removal. The removal was "total" in nine cases but incomplete in the other five. Immediate postoperative follow-up was uncomplicated in all cases. CONCLUSION: At 2-year follow-up, the two children with an extraventricular CP and a "total" tumor removal were intellectually normal, had no hypothalamic syndrome, and attended normal school with good results. The 12 others, although still intellectually normal, were more or less severely affected by a hypothalamic syndrome which altered their social insertion and caused academic failure. The authors conclude from this study that radical surgery should be reserved to extraventricular CPs only.

Right spatial neglect after left hemisphere stroke: qualitative and quantitative study.

OBJECTIVES: Comparatively little research has been conducted on right neglect after left brain damage. The authors sought to assess contralateral neglect in subacute left hemisphere stroke patients using a comprehensive test battery validated in a large control group after right hemisphere stroke. METHODS: Seventy-eight left hemisphere stroke patients were assessed. The test battery included a preliminary assessment of anosognosia and visual extinction, a clinical assessment of gaze orientation and personal neglect, and paper-and-pencil tests of spatial neglect in the peripersonal space. Only nonverbal tests were used. RESULTS: Drawing and cancellation tasks revealed neglect in 10 to 13% of patients. The combined battery was more sensitive than any single test alone. A total of 43.5% of patients showed some degree of neglect on at least one measure. Anatomic analyses showed that neglect was more common and severe when the posterior association cortex was damaged. CONCLUSIONS: The frequency of occurrence of right neglect was, as expected, much lower than that reported in a study using the same assessment battery in right brain damage stroke patients. Nevertheless, neglect was found in a substantial proportion of patients at a subacute stage, suggesting that it should be considered in the rehabilitation planning of left brain damage stroke patients.

Sensitivity of clinical and behavioural tests of spatial neglect after right hemisphere stroke.

OBJECTIVES: The lack of agreement regarding assessment methods is responsible for the variability in the reported rate of occurrence of spatial neglect after stroke. The aim of this study was to assess the sensitivity of different tests of neglect after right hemisphere stroke. METHODS: Two hundred and six subacute right hemisphere stroke patients were given a test battery including a preliminary assessment of anosognosia and of visual extinction, a clinical assessment of gaze orientation and of personal neglect, and paper and pencil tests of spatial neglect in the peripersonal space. Patients were compared with a previously reported control group. A subgroup of patients (n=69) received a behavioural assessment of neglect in daily life situations. RESULTS: The most sensitive paper and pencil measure was the starting point in the cancellation task. The whole battery was more sensitive than any single test alone. About 85% of patients presented some degree of neglect on at least one measure. An important finding was that behavioural assessment of neglect in daily life was more sensitive than any other single measure of neglect. Behavioural neglect was considered as moderate to severe in 36% of cases. A factorial analysis revealed that paper and pencil tests were related to two underlying factors. Dissociations were found between extrapersonal neglect, personal neglect, anosognosia, and extinction. Anatomical analyses showed that neglect was more common and severe when the posterior association cortex was damaged. CONCLUSIONS: The automatic rightward orientation bias is the most sensitive clinical measure of neglect. Behavioural assessment is more sensitive than any single paper and pencil test. The results also support the assumption that neglect is a heterogeneous disorder.

Is the optimal viewing position in reading influenced by familiarity of the letter string?

An optimal viewing position (OVP) for word recognition has been proposed by several authors. The location of this position would be located at the center of the word or just left of it. Several hypotheses ranging from perceptual to hemispheric factors have been proposed to explain this phenomenon. In the experiment presented here, the effect of the nature of the stimulus was tested: word, pseudo-word, or nonword on the existence and location of this position. Little research has investigated this issue. Five-letter words, pseudo-words, and non-words were presented, with the subject fixating initially on one of the five possible letter positions. The number of letters correctly identified and reading performance were recorded for each stimulus. Results show that an initial fixation on the third letter entails better letter identification for all kinds of stimuli. However, in terms of reading, only word reading benefit from a fixation on the third letter. These results are discussed in relation to the different hypotheses of OVP in reading. These are (1) hemispheric specialization, (2) reading habits, and (3) lexical constraints.

Unilateral neglect: the effect of competing stimuli on estimated line length.

Normal subjects and patients with right hemisphere lesions with or without signs of left unilateral neglect judged the length of a horizontal line presented on the left or on the right side of space. In half of the trials, the line was presented with a centrally located square or diamond, and subjects had to identify the central stimulus before performing the judgment of length. The presence of the central stimulus improved accuracy of performance in controls and in patients without neglect; neglect patients, however, produced more overestimations of left-sided lines when these was presented with a central stimulus than when the lines occurred in isolation. This finding underlines the importance of attentional factors in length estimates performed by neglect patients in their neglected space.

Position of the egocentric reference and performance in line bisection and subjective vertical estimation tasks.

Egocentric body coordinates such as the sagittal midline have been proposed to act as a reference for ballistic movements in extracorporeal space. Symmetrical functioning of the multiple neural structures processing sensory information would account for the normal sagittal position of the egocentric reference. According to this hypothesis a unilateral parietal lesion would induce a ipsilesional deviation which would prevent neglect patients from responding to stimuli that occur on that side. In this line of reasoning, an experimental manipulation of the position of the egocentric reference by way of a postural perturbation should have an effect on visuospatial tasks performance in normal subjects. Seventeen right-handed subjects were submitted in the dark to two visuomotor tasks: the adjustment of a luminescent rod to the vertical and the bisection of a luminescent line, either with the trunk and the head aligned at 0 degrees or with a 30 degrees trunk rotation to the right or to the left. Results revealed no significant effect of trunk rotation on the performance in both tasks. We discuss these findings and their implication for the understanding of the neglect syndrome.

Laterally directed arm movements and right unilateral neglect after left hemisphere damage.

Signs of unilateral neglect for events occurring in one hemispace most often result from right hemisphere lesions. Right unilateral neglect after left hemisphere damage is much rarer, and has received less attention. The present study explores the relationships between right unilateral neglect and asymmetries in producing laterally directed arm movements in the horizontal plane in left brain-damaged (LBD) patients. Participants produced right- or left-directed arm movements with their left arm in response to centrally located visual stimuli. Results showed that LBD patients with signs of right unilateral neglect were consistently slowed when producing arm movements toward the right (neglected) side, as compared to left-directed movements. Taking into account patients with and without signs of neglect, this directional asymmetry positively correlated with a reaction-time measure of perceptual spatial bias. These findings stand in contrast with previous results obtained with the same experimental paradigm in right brain-damaged patients, in whom a consistent slowing of leftward-directed movements was rare and apparently unrelated to the presence and severity of left neglect. These conflicting results are discussed with respect to the hypothesis that different mechanisms may underlie left and right unilateral neglect.

Modulating the attentional bias in unilateral neglect: the effects of the strategic set.

Left unilateral neglect is a neurological condition characterized by an impairment in orienting and responding to events occurring on the left side. To gain insight into the brain mechanisms of space processing and to provide theoretical foundations for patient rehabilitation, it is important to explore the attentional bias shown by neglect patients in the light of existing models of normal attentional orienting. Three experiments tested the hypothesis that attentional bias in neglect involves primarily exogenous, or stimulus-based, orienting of attention, with relatively preserved endogenous, or voluntary, orienting. Six patients with right hemisphere damage and left unilateral neglect and 18 age-matched participants without brain damage performed a cued reaction time (RT) task to targets which could appear in one of two lateral boxes. Cues consisted of a brief brightening of the contour of one of the boxes. The target followed the cue at 150, 550, or 1000 ms stimulus-onset asynchrony (SOA). In experiment 1, the cues were not informative about the future location of the target, and thus elicited a purely exogenous orienting of attention. Controls showed slowed RTs to the cued locations at SOAs > 150 ms, consistent with the notion of inhibition of return (IOR). Neglect patients had no evidence of IOR for right targets; they showed a disproportionate cost for left targets preceded by right (invalid) cues; this cost was maximal at the shortest SOA, consistent with the idea of a biased exogenous orienting in neglect. In experiment 2, 80% of the cues were valid (i.e., they correctly predicted the location of the impending target), thus inducing an initially exogenous, and later endogenous, attentional shift toward the cued box. Neglect patients showed again a cost for left invalidly cued targets, which this time persisted at SOAs > 150 ms, as if patients' attention had been cued to the right side not only exogenously, but also endogenously, thus rendering more difficult an endogenous reorienting toward the left. In experiment 3, only 20% of the cues were valid, so that the best response strategy was to endogenously orient attention toward the box opposite to the cued one. Controls were able to take advantage of invalid cues to rapidly respond to targets. In this condition, neglect patients were able to nullify their spatial bias; they achieved their fastest RTs to left targets, which were in the range of their RTs to right targets. However, for neglect patients fast responses to left targets occurred only at 1000 ms SOA, while controls were able to redirect their attention to the uncued box already at 550 ms SOA. Altogether, these results suggest that endogenous orienting is relatively spared, if slowed, in unilateral neglect.

Variability of response times as a marker of diverted attention.

Anderson et al. (Variability not ability: another basis for performance decrements in neglect. Neuropsychologia 2000;38:785-796) have recently reported that variability of response times (RTs) progressively increases from the right to the left side in left neglect patients. Anderson et al. propose that this lack of consistency is an important determinant of patients' behaviour, and may result from a deficit independent of other mechanisms causing neglect. Here we suggest that an increase of variability, and not only of RTs, is to be expected when attention is exogenously biased away from the probed location. Consequently, space-based variability can be interpreted in the framework of existing models of unilateral neglect. According to one such model, a basic impairment in left neglect is a bias toward rightward exogenous orienting of attention. As a result, left targets often fail to rapidly capture patients' attention, thus yielding slow RTs. However, since the probability for a left target attracting attention is low but not null, relatively fast RTs can occur on those rare occasions in which a left target does capture patients' attention. The coexistence of these relatively fast with slow RTs could be at the basis of space-based variability in neglect. Empirical support for our hypothesis comes from the results of a re-analysis for variability of cued RTs obtained in 18 normal individuals and six left neglect patients. Cues were peripheral and non-informative, thus eliciting an exogenous attentional shift. For normal individuals, invalid trials yielded less consistent response times than valid trials at short (150 ms) cue-target interval; for neglect patients, a similar phenomenon occurred for left invalidly-cued targets, thus paralleling the disproportionate cost in RTs typically evoked by this condition in unilateral neglect. We conclude by discussing some possible determinants of gradient-shaped effects and by outlining the implications of space-based variability for current models of unilateral neglect.

[Presenting a battery for assessing spatial neglect. Norms and effects of age, educational level, sex, hand and laterality]. / Présentation d'une batterie de dépistage de la négligence spatiale. Normes et effets de l'âge, du niveau d'éducation, du sexe, de la main et de la latéralité.

The aim of this study was to build up a battery for assessing spatial neglect, then to analyse the norms and potential effects of age, education level, sex, hand used, and laterality. It was also to contribute evaluating the pseudoneglect phenomenon described by Heilman, which consists in a tendency of normal subjects to neglect the right peripersonal space. Tasks selected were presented to important groups of normal subjects, most often larger than 450. The battery comprised of a bell cancellation test, scene copy, clock drawing, two line bisection tasks, identification of overlapping figures, text reading, writing task, and the representational task of the France map. For each of them, different variables were selected, especially investigating the difference between performance in the right and the left hemispaces. This study allowed defining the threshold values (percentiles 5 and 95) for deciding of the pathological character of a patient performance. It also showed that the pseudoneglect phenomenon is more obvious in some tasks such as line bisection, and probably also in the representational task of the France map and writing. His importance and at times his side were influenced by the factors we studied, with between tasks differences, but also by the nature of the task to be performed, and especially his verbal component.

Visual target detection paradigm for the study of selective attention.

The current protocol can be used to examine selective attention. It has been used to acquire behavioral performance data in neurologically healthy normal control subjects and schizophrenic patients. A modified version, also described here, has been used to acquire functional neuroimaging data in normal subjects using positron emission tomography. Subject response accuracy and reaction times are recorded while subjects detect visual stimuli in either hemifield (left vs. right of a fixation point) or along the vertical meridian (above or below fixation). The lateralized presentation of stimuli permits the study of hemispheric specialization for selective attentional processes. Attentional load is manipulated by presenting larger-sized target stimuli alone (i.e., the letter 'O') or smaller-sized target stimuli surrounded by flanking letters. This protocol report includes a description of subject exclusion criteria, procedural details, relevant experimental conditions and variables, suggestions for data analysis, expected results, and a discussion of the protocol's significance for attentional research along with suggestions for future research.