bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hidden Intelligence of a Multiply Handicapped Child with Joubert Syndrome
Authors: Ziegler Anne‐Lise , Deonna Thierry , Calame André 
Published: September 2010

SUMMARYA child with Joubert syndrome (episodic hyperpnoea, abnormal eye‐movements and ataxia) was followed from birth to eight years of age. Although severe mental retardation was suspected until approximately five years of age, unexpected and exceptional mental capacities were evident at later follow‐up. Adequate testing and appropriate stimulation of children with such a severe early motor disorder are difficult, so they may give a false impression of mental retardation. The history of this child challenges the notion that mental retardation is part of Joubert syndrome.RÉSUMÉIntelligence masquée chez un enfant à incapacités multiplesUn enfant présentant un syndrome de Joubert (hyperpneé épisodique, mouvements oculaires anormaux et ataxie) fut suivi de la naissance jusqu'à l'âge de huit ans. Bien qu'une arriération mentale sévère ait été suspectée jusqu'à l'âge de cinq ans, des capacités mentales inattendues et exceptionnelles devinrent évidentes au suivi ultérieur. Une évaluation adéquate et une stimulation appropriée des enfants avec un tel déficit moteur précoce sont difficiles et peuvent donner lieu à une impression erronée de retard mental. L'histoire de cet enfant met en cause le fait qu'un retard mental soit un signe régulier du syndrome de Joubert.ZUSAMMENFASSUNGVerborgene Intelligenz bei einem mehrfach behinderten KindEin Kind mit Joubert Syndrom (episodische Hyperventilation, abnorme Augenbewegungen und Ataxie) wurde von der Geburt bis zum achten Lebensjahr kontrolliert. Obwohl man in den ersten fùnf Jahren eine schwere geistige Retardierung angenommen hatte, fanden sich bei späteren Kontrolluntersuchnungen unerwartete und außergewohnliche geistige Fähigkeiten. Eine genaue Testung und Stimulation der Kinder mit solch einer schweren, fruh auftretenden motorischen Erkrankungen ist schwer, sodaß der falsche Eindruck einer geistigen Retardierung entstehen kann. Die Anamnese dieses Kindes stellt die Auffassung in Frage, daß die geistige Retardierung zum Joubert Syndrom gehort.RESUMENInteligencia escondida en un niño con múltiples incapacidadesSe siguió un niño con síndrome de Joubert (hiperpnea episódica, movimientos oculares anormales y ataxia) desde el nacimiento a los ocho años de edad. Aunque se sospechó in grave retraso mental hacia los cinco años de edad, más adelante se revelaron unas capacidades mentales exceptionales. Unos tests adecuados y una estimulación apropiada para este tipo de niños son dificiles de aplicar, por lo que pueden dar la falsa impresión de un retraso mental. La historia de este niño reta la noción de que el retraso mental forma parte de síndrome de Joubert.
EARLY DEVELOPMENT OF BOYS WITH DUCHENNE MUSCULAR DYSTROPHY
Authors: Smith R. A. , Sibert J. R. , Harper P. S. 
Published: September 2010

SUMMARYThirty‐three boys with Duchenne muscular dystrophy (DMD) and a mean age of 3–4 years were assessed with the Griffiths Developmental Scales, the Reynell Language Scales and the British Picture Vocabulary Scales at six‐monthly intervals over a one‐year period. The boys showed developmental delay, which was most severe in the locomotor and language areas. The locomotor quotients deteriorated over time, but the other quotients did not. Maternal intelligence, home environment and social class had little effect on the aetiology of the developmental delay, and the behavioural problems seen in young boys with DMD may be secondary to it.RÉSUMÉDéveloppement précoce d'une myopathie de DuchenneTrente‐trois garçons avec myopathie de Duchenne (DMD) et un âge moyen de troisans quatre mois ont été examinés aux Echelles de Développement de Griffiths, aux Echelles de Langage de Reynell et à la British Picture Vocabulary Scales à six mois d'intervalle sur une période d'un an. Les garçons présentaient un retard de développement particulièrement marqué pour la locomotion et le langage. Les quotients de locomotion se détérioraient en fontion du temps mais pas les autres quotients. L'intelligence maternelle, l'environnement à la maison et la classe sociale avaient peu d'effets sur l'étiologie du retard de développement et les problèmes comportementaux observés chez les jeunes gaçons avec DMD peuvent être secondaires à ce retard.ZUSAMMENFASSUNGFrühentwicklung bei Kindern mit Duchenne'scher Muskeldystrophie33 Jungen mit Duchenne'scher Muskeldystrophie (DMD) wurden im mittleren Alter von 3·4 Jahren mit den Griffith Developmental Scales, den Reynell Language Scales und British Picture Vocabulary Scales über einen Zeitraum von einem Jahr, in Intervallen von sechs Monaten untersucht. Die Jungen zeigten eine Entwicklungsverzögerung, die in den lokomotorischen und sprachlichen Bereichen am ausgeprägtesten war. Mütterliche Intelligenz, häusliches Umfeld und Sozialschicht hatten einen geringen Einfluß auf die Ätiologie der Entwicklungsverzögerung, und die Verhaltensprobleme, die bei den Jungen mit DMD auffielen, sind möglicherweise davon abhängig.RESUMENDesarrollo precoz en la distrofia muscular de DuchenneTreinta y tres niños con distrofia muscular de Duchenne (DMD) de una edad promedio de 3·4 años fueron evaluados con las Griffiths Developmental Scales, las Reynell Language Scales y las British Picture Vocabulary Scales a intervalos de seis meses durante un periodo de un año. Los niños mostraron un retraso de desarrollo que era más grave en las àreas locomotora y del lenguaje. Los cocientes locomotores se deterioraron con tiempo pero los otros cocientes no. La inteligencia materna, el ambiente del hogar y la clase social tenian poco efecto en la etiología del retraso del desarrollo y los problemas conductuales vistos en muchachos jóvenes con DMD pueden ser secundarios a este retraso.
Limits of colour vision in dim light
Authors: Kelber Almut , Lind Olle 
Published: September 2010

AbstractHumans and most vertebrates have duplex retinae with multiple cone types for colour vision in bright light, and one single rod type for achromatic vision in dim light. Instead of comparing signals from multiple spectral types of photoreceptors, such species use one highly sensitive receptor type thus improving the signal‐to‐noise ratio at night. However, the nocturnal hawkmoth Deilephila elpenor, the nocturnal bee Xylocopa tranquebarica and the nocturnal gecko Tarentola chazaliae can discriminate colours at extremely dim light intensities. To be able to do so, they sacrifice spatial and temporal resolution in favour of colour vision. We review what is known about colour vision in dim light, and compare colour vision thresholds with the optical sensitivity of the photoreceptors in selected animal species with lens and compound eyes.
Naming versus matching and the stability of unique hues
Authors: Panorgias A. , Kulikowski J. J. , Parry N. R. A. , McKeefry D. J. , Murray I. J. 
Published: September 2010

AbstractIt is known that there is a distortion of hue and saturation in the peripheral visual field. In a previous study, when an asymmetric matching paradigm was used, four hues in the blue, red, yellow and green regions of colour space were unchanged and these were referred to as peripherally invariant (Parry et al., J Opt Soc Am A, 23, 2006, 1586). Three of these invariant hues were similar to unique blue, red and yellow. However, for most observers there was a marked difference between unique and invariant green. To investigate this apparent paradox, we have measured unique hues using a range of eccentricities and colourimetric purities. An asymmetric matching and a 4‐AFC paradigm were used to establish peripherally invariant and unique hues, respectively. In the asymmetric matching task the observer matched a peripheral spot with a para‐foveal spot, for 24 different hues at 18° eccentricity. In the 4‐AFC paradigm, 41 hues were presented 20 times at three purities (0.5, 0.75 and 1.0) and three eccentricities (18°, 10° and 1°). The observer had to name the hues as red, blue, green or yellow. Unique hues were found to be constant with eccentricity and purity. The unique green, established with 4‐AFC, was found to differ from the invariant green, determined using the matching task. However, red, blue and yellow invariant hues correspond well with unique hues. The data suggest that different mechanisms mediate the matching of green compared with the identification of unique hues. This is similar to the difference between detection and discrimination of spectral stimuli: the detection process is dominated by the cone opponent mechanisms and is most sensitive, whereas more central processes, serving unique hues, influence discrimination.
Natural designs for manipulating the appearance of surfaces
Authors: Vukusic Pete 
Published: September 2010

AbstractThe surfaces of animals and plants are frequently adorned with a wealth of pattern, colour, and texture. Some insects for example, exhibit uniformly‐coloured unpatterned white surfaces; some display bright iridescent blue or green hues; others may present varying shades of black, brown or selected combinations of spectral colours. Additionally, unseen by human observers, certain species’ wing or body surfaces may produce strong ultra‐violet or polarisation signatures. This review will introduce the characteristics of structural colour effects and describe examples of them in the natural world. It will summarise some of the recent work on a range of insect species, focussing predominantly on Lepidoptera as an exemplar order.
How does the color influence figure and shape formation, grouping, numerousness and reading? The role of chromatic wholeness and fragmentation
Authors: Pinna Baingio , Uccula Arcangelo , Tanca Maria 
Published: September 2010

AbstractIn this work it is suggested that color induces phenomenal wholeness, part‐whole organization and fragmentation. The phenomenal wholeness subsumes the set of its main attributes: homogeneity, continuity, univocality, belongingness, and oneness. If color induces wholeness, it can also induce fragmentation. Therefore, in order to understand the role played by color within the process of part‐whole organization, color is used both as a wholeness and as a fragmentation tool, thus operating synergistically or antagonistically with other wholeness processes. Therefore, color is expected to influence figure‐ground segregation, grouping, shape formation and other visual processes that are related to the phenomenal wholeness. The purpose of this study is to rate the influence of color in inducing whole and part‐whole organization and, consequently, in determining the perception of figure‐ground segregation, grouping, shape formation, numerousness evaluation and time reading. We manipulated experimental conditions by using equiluminant colors to favor or break (parcel‐out) the wholeness of objects like geometrical composite figures and words. The results demonstrated that color is aimed, among other psychological and biological purposes, at: (1) relating each chromatic component of an object, thus favoring the emergence of the whole object; (2) eliciting a part‐whole organization, whose components are interdependent; (3) eliciting fragments and then breaking up the whole and favoring the emergence of single components. Wholeness, part‐whole organization and fragmentation can be considered as three further purposes of color.
Colour‐opponent mechanisms are not affected by age‐related chromatic sensitivity changes
Authors: Wuerger Sophie , Xiao Kaida , Fu Chenyang , Karatzas Dimosthenis 
Published: September 2010

AbstractThe purpose of this study was to assess whether age‐related chromatic sensitivity changes are associated with corresponding changes in hue perception in a large sample of colour‐normal observers over a wide age range (n = 185; age range: 18–75 years). In these observers we determined both the sensitivity along the protan, deutan and tritan line; and settings for the four unique hues, from which the characteristics of the higher‐order colour mechanisms can be derived. We found a significant decrease in chromatic sensitivity due to ageing, in particular along the tritan line. From the unique hue settings we derived the cone weightings associated with the colour mechanisms that are at equilibrium for the four unique hues. We found that the relative cone weightings (wL/wMandwL/wS) associated with the unique hues were independent of age. Our results are consistent with previous findings that the unique hues are rather constant with age while chromatic sensitivity declines. They also provide evidence in favour of the hypothesis that higher‐order colour mechanisms are equipped with flexible cone weightings, as opposed to fixed weights. The mechanism underlying this compensation is still poorly understood.
Cone contrast influence on components of the pattern onset/offset VECP
Authors: Gomes B. D. , Souza G. S. , Saito C. A. , Da Silva Filho M. , Rodrigues A. R. , Ventura D. F. , Silveira L. C. L. 
Published: September 2010

AbstractTransient visual evoked cortical potentials (VECP) were recorded from the scalp of healthy normal trichromats (n = 12). VECPs were elicited by onset/offset presentation of patterned stimuli of two kinds: isochromatic luminance‐modulated, and equiluminant red‐green modulated, sine wave gratings. The amplitude and latency of the major onset components of the onset/offset VECP were measured and plotted as a function of the logarithm of pooled cone contrast. The early onset components, achromatic C1 and chromatic N1, increase linearly with log contrast, but N1 has a higher contrast gain than C1. The late onset components, achromatic C2 and chromatic N2, have similar contrast gain, and similar response as a function of contrast level: both increase in the low‐to‐medium range of contrasts and saturate at high contrast levels. In the range of pooled cone contrast tested, C1 and N1 show similar latencies, whilst C2 shows shorter latencies than N2. We suggest that C1 and N1 are generated by the same visual mechanism with high red‐green contrast gain and low luminance contrast gain, whilst C2 and N2 are generated by different visual mechanisms.
Basic colour names for 2D samples: effects of presentation media and illuminants
Authors: Hedrich Monika , Bloj Marina 
Published: September 2010

AbstractWe have previously shown (Bloj et al., 2008; Abstracts Materials & Sensations 2008) that under particular conditions colour memory is independent of presentation media, and of the illuminants under which colours are viewed. In the present study we investigate whether colour naming is also unaffected by these two factors.Forty‐eight colour samples from the Natural Colour System (NCS) collection were presented as real paper samples or as accurate computer simulations displayed on a calibrated monitor. The colour swatches could be presented under a daylight illuminant – two intensities, 85 (‘D1’) or 60 cd m−2 (‘D2’) – or a purple illuminant, 45 cd m−2 (‘Lily’). The colour samples were shown in arrays of 16 (4 × 4 layout) and the observer’s task was to assign one of the eleven basic colour terms to each of the samples. Six observers repeated this colour naming task five times for each presentation medium and illuminant.On average, in 73% of the cases the same colour term was assigned to surface and display colours. This level of agreement was highest for colour samples under daylight (D1‐82%, D2‐73%) and poor for Lily (65%). Although colour memory is unaffected by the nature of the colour stimulus, here we show that there are limitations to cross‐media agreement in colour naming.
L‐ and M‐cone input to 12Hz and 30Hz flicker ERGs across the human retina
Authors: Challa N. K. , McKeefry D. , Parry N. R. A. , Kremers J. , Murray I. J. , Panorgias A. 
Published: September 2010

AbstractWe recorded L‐ and M‐cone isolating ERGs from human subjects using a silent substitution technique at temporal rates of 12 and 30 Hz. These frequencies isolate the activity of cone‐opponent and non‐opponent post‐receptoral mechanisms, respectively. ERGs were obtained using a sequence of stimuli with different spatial configurations comprising; (1) circular stimuli of different sizes which increased in 10° steps up to 70°diameter, or (2) annular stimuli with a 70° outer diameter but with different sized central ablations from 10° up to 60°. L‐ and M‐cone isolating ERGs were obtained from five colour normal subjects using a DTL fibre electrode. Fourier analysis of the ERGs was performed and we measured the amplitude of the first harmonic of the response. For 12 Hz ERGs the L:M cone response amplitude ratio (L:MERG) was close to unity and remained stable irrespective of the spatial configuration of the stimulus. The maintenance of this balanced ratio points to the existence of cone selective input across the human retina for the L‐M cone opponent mechanism. For 30 Hz the L:MERG ratio was greater than unity but varied depending upon which region of the retina was being stimulated. This variation we consider to be a consequence of the global response properties of M‐cone ERGs rather than representing a real variation in L:M cone ratios across the retina.
Simulations of adaptation and color appearance in observers with varying spectral sensitivity
Authors: Webster Michael A. , Juricevic Igor , McDermott Kyle C. 
Published: September 2010

AbstractA model of adaptation and visual coding was used to simulate how color appearance might vary among individuals that differ only in their sensitivity to wavelength. Color responses to images were calculated for cone receptors with spectral sensitivities specific to the individual, and in postreceptoral mechanisms tuned to different combinations of the cones. Adaptation was assumed to normalize sensitivity within each cone and postreceptoral channel so that the average response to an ensemble of scenes equaled the mean response in channels defined for the reference observer. Image colors were then rendered from the adapted channels’ outputs. The transformed images provide an illustration of the variations in color appearance that could be attributed to differences in spectral sensitivity in otherwise identical observers adapted to identical worlds, and examples of these predictions are shown for both normal variation (e.g. in lens and macular pigment) and color deficiencies (anomalous trichromacy). The simulations highlight the role that known processes of adaptation may play in compensating color appearance for variations in sensitivity both within and across observers, and provide a novel tool for visualizing the perceptual consequences of any variation in visual sensitivity including changes associated with development or disease.
Spatially coherent colour image reconstruction from a trichromatic mosaic with random arrangement of chromatic samples
Authors: Alleysson David 
Published: September 2010

AbstractRecent high resolution imaging of the human retina confirms that the trichromatic cone mosaic follows a random arrangement. Moreover, both the cones’ arrangements and proportion widely differ from individual to individual. These findings provide new insights to our understanding of colour vision as most of the previous vision models ignored the mosaic sampling. Here, we propose a cone mosaic sampling simulation applied to colour images. From the simulation, we can infer the processing needs for retrieving spatial and chromatic information from the mosaic without spatial ambiguity. In particular, the focus is on the ability of the visual system to reconstruct coherent spatial information from a plurality of local neighbourhoods. We show that normalized linear processing allows the recovery of achromatic and chromatic information from a mosaic of trichromatic samples arranged randomly. Also, low frequency components of achromatic information can serve to coarsely estimate orientation, which in turn improves the interpolation of chromatic information. An implication for the visual system is the possibility that, in the cortex, the low frequency achromatic spatial information of the magnocellular pathway helps separate chromatic information from the mixed achromatic/chromatic information carried by the parvocellular pathway.
A behavioural investigation of human visual short term memory for colour
Authors: Nemes V. A. , Parry N. R. A. , McKeefry D. J. 
Published: September 2010

AbstractWe examined visual short term memory (VSTM) for colour using a delayed‐match‐to‐sample paradigm. In these experiments we measured the effects of increasing inter‐stimulus interval (ISI), varying between 0 and 10 s, on the ability of five colour normal human observers to make colour matches between a reference and subsequently presented test stimuli. The coloured stimuli used were defined by different chromatic axes on the isoluminant plane of DKL colour space. In preliminary experiments we used a hue scaling procedure to identify a total of 12 colour stimuli which served as reference hues in the colour memory experiments: four stimuli were exemplars of red, green, blue and yellow colour appearance categories, four were located between these categories and a further four were located on the cardinal axes that isolated the activity of the cone‐opponent mechanisms. Our results demonstrate that there is a reduction in the ability of observers to make accurate colour matches with increasing ISIs and that this reduced performance was similar for all colour stimuli. However, the shifts in hue that were measured between the reference and matched test stimuli were significantly greater for the cardinal stimuli compared to those measured for the stimuli defined by the hue scaling procedure. This deterioration in the retention of hue in VSTM for stimuli that isolate cone‐opponent mechanisms may be a reflection of the reorganisation of colour processing that occurs in the cortex where colour appearance mechanisms become more prominent.
Colour detection thresholds as a function of chromatic adaptation and light level
Authors: Jennings B. J. , Barbur J. L. 
Published: September 2010

AbstractColour threshold discrimination ellipses were measured for a number of states of chromatic adaptation and a range of luminance levels using the Colour Assessment and Diagnosis (CAD) test. An analysis of these results was carried out by examining the cone excitation signals along the cardinal axes that correspond to detection thresholds in the +L‐M (reddish), −L+M (greenish), +S (bluish) and −S (yellowish) colour directions. The results reveal a strong linear relationship between the excitations induced by the adapting background field in each cone class and the corresponding changes needed for threshold detection. These findings suggest that the cone excitation change for threshold detection of colour signals is always the same for a given background excitation level (in any cone class), independent of the excitations generated in the other cone classes. These observations have been used to develop a model to predict colour detection thresholds for any specified background luminance and chromaticity within the range of values investigated in this study (e.g., luminances in the range 0.3 to 31 cd.m−2 and chromaticities within the gamut of typical CRT displays). Predicted colour thresholds were found to be in close agreement with measured values with errors that do not, in general, exceed the measured within‐subject variability.