Cognitive outcome and its neural correlates after cardiorespiratory arrest in childhood.

Hypoxia-ischaemia (HI) can result in structural brain abnormalities, which in turn can lead to behavioural deficits in various cognitive and motor domains, in both adult and paediatric populations. Cardiorespiratory arrest (CA) is a major cause of hypoxia-ischaemia in adults, but it is relatively rare in infants and children. While the effects of adult CA on brain and cognition have been widely studied, to date, there are no studies examining the neurodevelopmental outcome of children who suffered CA early in life. Here, we studied the long-term outcome of 28 children who suffered early CA (i.e., before age 16). They were compared to a group of control participants (n = 28) matched for age, sex and socio-economic status. The patient group had impairments in the domains of memory, language and academic attainment (measured using standardised tests). Individual scores within the impaired range were most commonly found within the memory domain (79%), followed by academic attainment (50%), and language (36%). The patient group also had reduced whole brain grey matter volume, and reduced volume and fractional anisotropy of the white matter. In addition, lower performance on memory tests was correlated with bilaterally reduced volume of the hippocampi, thalami, and striatum, while lower attainment scores were correlated with bilateral reduction of fractional anisotropy in the superior cerebellar peduncle, the main output tract of the cerebellum. We conclude that patients who suffered early CA are at risk of developing specific cognitive deficits associated with structural brain abnormalities. RESEARCH HIGHLIGHTS: Our data shed light on the long-term outcome and associated neural mechanisms after paediatric hypoxia-ischaemia as a result of cardiorespiratory arrest. Patients had impaired scores on memory, language and academic attainment. Memory impairments were associated with smaller hippocampi, thalami, and striatum. Lower academic attainment correlated with reduced fractional anisotropy of the superior cerebellar peduncle.

Longitudinal fNIRS and EEG metrics of habituation and novelty detection are correlated in 1-18-month-old infants.

INTRODUCTION: Habituation and novelty detection are two fundamental and widely studied neurocognitive processes. Whilst neural responses to repetitive and novel sensory input have been well-documented across a range of neuroimaging modalities, it is not yet fully understood how well these different modalities are able to describe consistent neural response patterns. This is particularly true for infants and young children, as different assessment modalities might show differential sensitivity to underlying neural processes across age. Thus far, many neurodevelopmental studies are limited in either sample size, longitudinal scope or breadth of measures employed, impeding investigations of how well common developmental trends can be captured via different methods. METHOD: This study assessed habituation and novelty detection in N = 204 infants using EEG and fNIRS measured in two separate paradigms, but within the same study visit, at 1, 5 and 18 months of age in an infant cohort in rural Gambia. EEG was acquired during an auditory oddball paradigm during which infants were presented with Frequent, Infrequent and Trial Unique sounds. In the fNIRS paradigm, infants were familiarised to a sentence of infant-directed speech, novelty detection was assessed via a change in speaker. Indices for habituation and novelty detection were extracted for both EEG and NIRS RESULTS: We found evidence for weak to medium positive correlations between responses on the fNIRS and the EEG paradigms for indices of both habituation and novelty detection at most age points. Habituation indices correlated across modalities at 1 month and 5 months but not 18 months of age, and novelty responses were significantly correlated at 5 months and 18 months, but not at 1 month. Infants who showed robust habituation responses also showed robust novelty responses across both assessment modalities. DISCUSSION: This study is the first to examine concurrent correlations across two neuroimaging modalities across several longitudinal age points. Examining habituation and novelty detection, we show that despite the use of two different testing modalities, stimuli and timescale, it is possible to extract common neural metrics across a wide age range in infants. We suggest that these positive correlations might be strongest at times of greatest developmental change.

Neuroimaging and Cognitive Function in Sickle Cell Disease: A Systematic Review.

Sickle cell disease (SCD) is the most common inherited single-gene disease. Complications include chronic anaemia, reduced oxygen-carrying capability, and cerebral vasculopathy, resulting in silent cerebral infarction, stroke, and cognitive dysfunction with impairments in measures of executive function, attention, reasoning, language, memory, and IQ. This systematic review aims to investigate the association between neuroimaging findings and cognition in children with SCD. Searches of PubMed and Embase were conducted in March 2022. Studies were included if participants were <18 years, if original data were published in English between 1960 and 2022, if any genotype of SCD was included, and if the relationship between cognition and neuroimaging was examined. Exclusion criteria included case studies, editorials, and reviews. Quality was assessed using the Critical Appraisal Skills Programme Case Control Checklist. A total of 303 articles were retrieved; 33 met the eligibility criteria. The presence of overt or silent strokes, elevated blood flow velocities, abnormal functional connectivity, and decreased fMRI activation were associated with neuropsychological deficits in children with SCD when compared to controls. There is a critical need to address the disease manifestations of SCD early, as damage appears to begin at a young age. Most studies were cross-sectional, restricting the interpretation of the directionality of relationships. Future research employing longitudinal neuroimaging and neuropsychological assessments could improve our understanding of the cumulative consequences of SCD on the developing brain.

Validation of a touchscreen assessment tool to screen for cognitive delay at 24 months.

AIM: To validate a touchscreen assessment as a screening tool for mild cognitive delay in typically developing children aged 24 months. METHOD: Secondary analysis of data was completed from an observational birth cohort study (The Cork Nutrition & Microbiome Maternal-Infant Cohort Study [COMBINE]), with children born between 2015 and 2017. Outcome data were collected at 24 months of age, at the INFANT Research Centre, Ireland. Outcomes were the Bayley Scales of Infant and Toddler Development, Third Edition cognitive composite score and a language-free, touchscreen-based cognitive measure (Babyscreen). RESULTS: A total of 101 children (47 females, 54 males) aged 24 months (mean = 24.25, SD = 0.22) were included. Cognitive composite scores correlated with the total number of Babyscreen tasks completed, with moderate concurrent validity (r = 0.358, p < 0.001). Children with cognitive composite scores lower than 90 (1 SD below the mean, defined as mild cognitive delay) had lower mean Babyscreen scores than those with cognitive scores equal to or greater than 90 (8.50 [SD = 4.89] vs 12.61 [SD = 3.68], p = 0.001). The area under the receiver operating characteristic curve for the prediction of a cognitive composite score less than 90 was 0.75 (95% confidence interval = 0.59-0.91; p = 0.006). Babyscreen scores less than 7 were equivalent to less than the 10th centile and identified children with mild cognitive delay with 50% sensitivity and 93% specificity. INTERPRETATION: Our 15-minute, language-free touchscreen tool could reasonably identify mild cognitive delay among typically developing children.

Neural Marker of Habituation at 5 Months of Age Associated with Deferred Imitation Performance at 12 Months: A Longitudinal Study in the UK and The Gambia.

Across cultures, imitation provides a crucial route to learning during infancy. However, neural predictors which would enable early identification of infants at risk of suboptimal developmental outcomes are still rare. In this paper, we examine associations between ERP markers of habituation and novelty detection measured at 1 and 5 months of infant age in the UK (n = 61) and rural Gambia (n = 214) and infants' responses on a deferred imitation task at 8 and 12 months. In both cohorts, habituation responses at 5 months significantly predicted deferred imitation responses at 12 months of age in both cohorts. Furthermore, ERP habituation responses explained a unique proportion of variance in deferred imitation scores which could not be accounted for by a neurobehavioural measure (Mullen Scales of Early Learning) conducted at 5 months of age. Our findings highlight the potential for ERP markers of habituation and novelty detection measured before 6 months of age to provide insight into later imitation abilities and memory development across diverse settings.

Late- but not early-onset blindness impairs the development of audio-haptic multisensory integration.

Integrating different senses to reduce sensory uncertainty and increase perceptual precision can have an important compensatory function for individuals with visual impairment and blindness. However, how visual impairment and blindness impact the development of optimal multisensory integration in the remaining senses is currently unknown. Here we first examined how audio-haptic integration develops and changes across the life span in 92 sighted (blindfolded) individuals between 7 and 70 years of age. We used a child-friendly task in which participants had to discriminate different object sizes by touching them and/or listening to them. We assessed whether audio-haptic performance resulted in a reduction of perceptual uncertainty compared to auditory-only and haptic-only performance as predicted by maximum-likelihood estimation model. We then compared how this ability develops in 28 children and adults with different levels of visual experience, focussing on low-vision individuals and blind individuals that lost their sight at different ages during development. Our results show that in sighted individuals, adult-like audio-haptic integration develops around 13-15 years of age, and remains stable until late adulthood. While early-blind individuals, even at the youngest ages, integrate audio-haptic information in an optimal fashion, late-blind individuals do not. Optimal integration in low-vision individuals follows a similar developmental trajectory as that of sighted individuals. These findings demonstrate that visual experience is not necessary for optimal audio-haptic integration to emerge, but that consistency of sensory information across development is key for the functional outcome of optimal multisensory integration.

Mother-infant interactions with infants with congenital visual impairment and associations with longitudinal outcomes in cognition and language.

BACKGROUND: This study investigated mother-infant interactions, including maternal maintaining of infant attentional focus and sensitivity, with infants with congenital severe and profound visual impairment (VI) and the association with developmental trajectories from one to three years. METHOD: Fifty-five infants and mothers were video-recorded playing together with a standard set of toys at Time 1 (T1) mean age 12.95 months (8.13-17.05 months). Maintain was categorized as the mother following and maintaining the child's focus, and Sensitivity, the mother's responsiveness and contingency to infant behaviour. Vision level was measured using the Near Detection Scale. Cognition and language were measured at T1, 12 months later (T2) and 24 months later (T3) using the Reynell-Zinkin Scales. RESULTS: Cross-sectional analyses showed that mothers of infants with severe VI (basic form vision) produced higher rates of Maintain compared to those with children with profound VI (light perception at best). Linear mixed-effects models examining developmental progression from T1 to T3 (controlling for vision level) showed an average increase of 5 DQ points (CI 95%: 1.03-9.08) in verbal comprehension for higher Sensitivity. No significant findings were found for Maintain. CONCLUSIONS: The findings suggest that mother-infant interactions (maternal Maintain) are associated with level of vision at infancy, but only maternal Sensitivity has a long-term positive association with advances in verbal comprehension from infancy to about three years. They highlight the need for incorporating strategies related to parent-infant interactions, including increased sensitivity, into early intervention for children with visual impairment.

Concurrent validity of a touchscreen application to detect early cognitive delay.

OBJECTIVE: To explore the ability of an interactive screening tool to identify cognitive delay in children aged 18 to 24 months. DESIGN: Children were assessed using the Bayley Scale of Infant and Toddler Development-third edition (BSID-III) and a touchscreen measure of problem-solving (Babyscreen V.1.5). We examined the internal consistency and concurrent validity between the two measures. A BSID-III cognitive composite score (BSID-IIIcc) ≤1 SD below population mean was used to indicate a low average cognitive ability. RESULTS: 87 children with a mean (SD) age of 20.4 (1.3) months who experienced complications at delivery (n=53) and healthy age-matched controls (n=34) were included in the study. A moderate positive correlation between the BSID-IIIcc and the total number of tasks completed on the Babyscreen suggested reasonable concurrent validity (r=0.414, p<0.001). Children with a BSID-IIIcc ≤90 had lower median (IQR) Babyscreen score (7 (6, 8.5) vs 11 (8.5, 13); p=0.003) and a lower median (IQR) age-adjusted z-score (BST z-score) for number of items completed compared with those >90 (-1.08 (-1.5 to -0.46) vs 0.31 (-0.46 to 0.76); p=0.001). The area under the receiver operating characteristic curve for the prediction of a low normal BSID-IIIcc was 0.787 (CI 0.64 to 0.93). A BST z-score of <-0.44 yielded 82.4% sensitivity and 71.4% specificity in identifying children with cognitive delay. CONCLUSIONS: A touchscreen-based application has concurrent validity with the BSID-IIIcc and could be used to screen for cognitive delay at 18-24 months of age.

Developmental trajectories of infants born at less than 30 weeks' gestation on the Bayley-III Scales.

OBJECTIVE: To describe the cognitive, language and motor developmental trajectories of children born very preterm and to identify perinatal factors that predict the trajectories. DESIGN: Data from a cohort of 1142 infants born at <30 weeks' gestation who were prospectively assessed on the Bayley Scales of Infant and Toddler Development, third edition (Bayley-III) at 3, 6, 12 and 24 months corrected age, were analysed using the Super Imposition by Translation and Rotation (SITAR) growth curve analysis model. MAIN OUTCOME MEASURES: Developmental trajectory SITAR models for Bayley-III cognitive, language (receptive and expressive communication subscales) and motor (fine and gross motor subscales) scores. RESULTS: The successfully fitted SITAR models explained 62% of variance in cognitive development, 68% in receptive communication, 53% in fine motor and 68% in the gross motor development. There was too much variation in the expressive communication subscale to fit a SITAR model. The rate of development (gradient of the curve) best explains the variation in trajectories of development in all domains. Lower gestational age, lower birth weight and male sex significantly predicted a slower rate of development. CONCLUSION: The rate of development, rather than single time point developmental assessment, best predicts the very preterm infant's developmental trajectory and should be the focus for monitoring and early intervention.

ERP markers are associated with neurodevelopmental outcomes in 1-5 month old infants in rural Africa and the UK.

INTRODUCTION: Infants and children in low- and middle-income countries are frequently exposed to a range of poverty-related risk factors, increasing their likelihood of poor neurodevelopmental outcomes. There is a need for culturally objective markers, which can be used to study infants from birth, thereby enabling early identification and ultimately intervention during a critical time of neurodevelopment. METHOD: In this paper, we investigate developmental changes in auditory event related potentials (ERP) associated with habituation and novelty detection in infants between 1 and 5 months living in the United Kingdom and The Gambia, West Africa. Previous research reports that whereas newborns' ERP responses are increased when presented with stimuli of higher intensity, this sensory driven response decreases over the first few months of life, giving rise to a cognitively driven, novelty-based response. Anthropometric measures were obtained concurrently with the ERP measures at 1 and 5 months of age. Neurodevelopmental outcome was measured using the Mullen Scales of Early Learning (MSEL) at 5 months of age. RESULTS: The described developmental change was observed in the UK cohort, who exhibited an intensity-based response at 1 month and a novelty-based response at 5 months of age. This change was accompanied by greater habituation to stimulus intensity at 5 compared to 1 month. In the Gambian cohort we did not see a change from an intensity-to a novelty-based response, and no change in habituation to stimulus intensity across the two age points. The degree of change from an intensity towards a novelty-based response was further found to be associated with MSEL scores at 5 months of infant age, whereas infants' growth between 1 and 5 months was not. DISCUSSION: Our study highlights the utility of ERP-based markers to study young infants in rural Africa. By implementing a well-established paradigm in a previously understudied population we have demonstrated its use as a culturally objective tool to better understand early learning in diverse settings world-wide. Results offer insight into the neurodevelopmental processes underpinning early neurocognitive development, which may in the future contribute to early identification of infants at heightened risk of adverse neurodevelopmental outcome.

Brain structure in children with congenital visual disorders and visual impairment.

AIM: To examine if congenital visual impairment is associated with differences in brain anatomy in children. METHOD: Ten children (8-12y) with congenital disorders of the peripheral visual system with severe visual impairment (SVI; >0.8 logMAR) or mild-to-moderate visual impairment (MVI; 0.6-0.8 logMAR) were compared to 21 typically sighted comparison (TSC) children. Thalamus volume, grey matter density, white matter microstructure, and integrity of visual tracts were investigated in SVI, MVI, and TSC groups with anatomical and diffusion-weighted magnetic resonance imaging. RESULTS: Compared to the TSC group, the SVI group had lower white matter integrity in tracts of the visual system (optic radiations: SVI 0.35±0.015, TSC 0.39±0.007 [p=0.022]; posterior corpus callosum: SVI 0.37±0.019; TSC 0.42±0.009 [p=0.033]) and lower left thalamus volume (SVI 4.37±0.087; TSC 4.99±0.339 [p=0.015]). Neuroanatomical differences were greater in the SVI group, while no consistent differences between the MVI and TSC group were observed. INTERPRETATION: Posterior tracts of the visual system are compromised in children with congenital visual impairment versus those who are typically sighted. The severity of visual input appears to have affected neuroanatomical development as significant reductions were only found in the SVI group. WHAT THIS PAPER ADDS: Severe visual impairment in mid-childhood is associated with reduced integrity of visual pathways and reduced thalamus volume.

Temperament in preschool children with sickle cell anaemia.

AIMS: Few studies have investigated the potential impact of sickle cell anaemia (SCA) on temperament. The aim of the current study was to investigate temperament in preschool children with SCA and to establish the reliability of the Children's Behaviour Questionnaire (CBQ) in this population. METHODS: The CBQ, a parent-report measure of temperament, was completed by parents of 21 preschool children with SCA and a control group of parents of typically developing children, matched for age, ethnicity and socioeconomic status. RESULTS: A significant difference between groups was identified for the dimension of negative affectivity only, with specific differences observed in the discomfort subdomain. Patients with a greater number of hospital admissions in the previous year were reported to have higher levels of discomfort. CONCLUSIONS: Preschool children with SCA are reported to have higher rates of negative affectivity, particularly discomfort. Future research is required to investigate the potential influence of dysregulated negative emotions and discomfort on disease management and quality of life throughout childhood.

Implementing neuroimaging and eye tracking methods to assess neurocognitive development of young infants in low- and middle-income countries.

Infants and children in low- and middle-income countries (LMICs) are frequently exposed to a range of environmental risk factors which may negatively affect their neurocognitive development. The mechanisms by which factors such as undernutrition and poverty impact development and cognitive outcomes in early childhood are poorly understood. This lack of knowledge is due in part to a paucity of objective assessment tools which can be implemented across different cultural settings and in very young infants. Over the last decade, technological advances, particularly in neuroimaging, have opened new avenues for research into the developing human brain, allowing us to investigate novel biological associations. This paper presents functional near-infrared spectroscopy (fNIRS), electroencephalography (EEG) and eye tracking (ET) as objective, cross-cultural methods for studying infant neurocognitive development in LMICs, and specifically their implementation in rural Gambia, West Africa. These measures are currently included, as part of a broader battery of assessments, in the Brain Imaging for Global Health (BRIGHT) project, which is developing brain function for age curves in Gambian and UK infants from birth to 24 months of age. The BRIGHT project combines fNIRS, EEG and ET with behavioural, growth, health and sociodemographic measures. The implementation of these measures in rural Gambia are discussed, including methodological and technical challenges that needed to be addressed to ensure successful data acquisition. The aim is to provide guidance to other groups seeking to implement similar methods in their research in other LMICs to better understand associations between environmental risk and early neurocognitive development.

Understanding the development of face and emotion processing under a predictive processing framework.

In the present work, we explore the development of processing of emotional facial configurations under a predictive processing (or predictive coding) framework. Predictive processing provides a new approach to brain function that has been used to explain a wide range of processes, from perception to socioemotional processing. The explanatory power of this framework for adult brain function is widely recognized, but it has yet to be systematically applied to understanding the developing brain. Studying the findings of developmental research under this framework may allow a deeper understanding of the predictive mechanisms and their ontogenetic course, and adds to knowledge on brain functions and developmental processes. Therefore, the goal of this work was to explore the potential complementarity of predictive processing and development. Specifically, we focus on how the development of facial and emotion processing may be understood under a predictive processing framework. The processing of facial expressions was selected because of the developmental relevance of these stimuli, their impact on general emotional development, as well as the large body of literature on this topic (comprised of both well-established but also incongruent findings, which a novel approach may clarify). Considering the main findings of developmental research on the processing of emotion-related facial configurations under this framework, we argue that predictive processing is consistent with developmental evidence and provides a promising avenue for developmental research, as it reveals new questions in the fields of development and emotion processing. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

Active touch facilitates object size perception in children but not adults: A multisensory event related potential study.

In order to increase perceptual precision the adult brain dynamically combines redundant information from different senses depending on their reliability. During object size estimation, for example, visual, auditory and haptic information can be integrated to increase the precision of the final size estimate. Young children, however, do not integrate sensory information optimally and instead rely on active touch. Whether this early haptic dominance is reflected in age-related differences in neural mechanisms and whether it is driven by changes in bottom-up perceptual or top-down attentional processes has not yet been investigated. Here, we recorded event-related-potentials from a group of adults and children aged 5-7 years during an object size perception task using auditory, visual and haptic information. Multisensory information was presented either congruently (conveying the same information) or incongruently (conflicting information). No behavioral responses were required from participants. When haptic size information was available via actively tapping the objects, response amplitudes in the mid-parietal area were significantly reduced by information congruency in children but not in adults between 190 ms-250 ms and 310 ms-370 ms. These findings indicate that during object size perception only children's brain activity is modulated by active touch supporting a neural maturational shift from sensory dominance in early childhood to optimal multisensory benefit in adulthood.

The Role of Family Functioning in the Development of Executive Functions in Preschool Children with Sickle Cell Anemia.

Executive functions are compromised in children with sickle cell anemia. There is limited research on the development of executive functions in preschool children with sickle cell anemia and the factors that contribute to executive dysfunction. We looked at the relation between biomedical and environmental factors, including family functioning and socioeconomic status, and executive functions in 22 preschool children with sickle cell anemia. We found that family functioning was the strongest predictor of executive outcomes in young children with sickle cell anemia with no evidence for an influence of disease severity at this early stage.

Intelligence and memory outcomes within 10 years of childhood convulsive status epilepticus.

Long-term intelligence and memory outcomes of children post convulsive status epilepticus (CSE) have not been systematically investigated despite evidence of short-term impairments in CSE. The present study aimed to describe intelligence and memory outcomes in children within 10 years of CSE and identify potential risk factors for adverse outcomes. In this cohort study, children originally identified by the population-based North London Convulsive Status Epilepticus in Childhood Surveillance Study (NLSTEPSS) were prospectively recruited between July 2009 and February 2013 and invited for neuropsychological assessments and magnetic resonance imaging (MRI) scans. Full-scale intelligence quotients (FSIQs) were measured using the Wechsler Abbreviated Scales of Intelligence (WASI), and global memory scores (GMS) was assessed using the Children's Memory Scale (CMS). The cohort was analyzed as a whole and stratified into a prolonged febrile seizures (PFS) and non-PFS group. Their performance was compared with population norms and controls. Regression models were fitted to identify predictors of outcomes. With a mean of 8.9 years post-CSE, 28.5% of eligible participants were unable to undertake testing because of their severe neurodevelopmental deficits. Children with CSE who undertook formal testing (N = 94) were shown to have significantly lower FSIQ (p = 0.001) and GMS (p = 0.025) from controls; the PFS group (N = 34) had lower FSIQs (p = 0.022) but similar memory quotients (p = 0.88) with controls. Intracranial volume (ICV), developmental delay at baseline, and active epilepsy at follow-up were predictive of long-term outcomes in the non-PFS group. The relationship between ICV and outcomes was absent in the PFS group despite its presence in the control and non-PFS groups. Post-CSE, survivors reveal significant intelligence and memory impairments, but prognosis differs by CSE type; memory scores are uncompromised in the PFS group despite evidence of their lower FSIQ whereas both are compromised in the non-PFS group. Correlations between brain volumes and outcomes differ in the PFS, non-PFS, and control groups and require further investigation.

Executive performance on the preschool executive task assessment in children with sickle cell anemia and matched controls.

Executive deficits are commonly reported in children with sickle cell anemia. Earlier identification of executive deficits would give more scope for intervention, but this cognitive domain has not been routinely investigated due to a lack of age-appropriate tasks normed for preschool children. In particular, information relating to patient performance on an executive task that reflects an everyday activity in the classroom could provide important insight and practical recommendations for the classroom teacher at this key developmental juncture as they enter the academic domain. The performance of 22 children with sickle cell anemia was compared to 24 matched control children on the Preschool Executive Task Assessment. Findings reveal that children with sickle cell anemia are performing poorer than their matched peers on this multi-step assessment. In particular, children with sickle cell anemia required more structured support to shift focus after a completed step, as reflected by poorer scores in the quantitative Sequencing and Completion domains. They also required more support to stay on task, as seen by poorer ratings in the qualitative Distractibility domain. Abbreviations:PETA: Preschool Executive Task Assessment; SCA: Sickle Cell Anemia; EF: Executive Functioning.

Home-based early intervention in infants and young children with visual impairment using the Developmental Journal: longitudinal cohort study.

AIM: To investigate the effects of home-based early intervention in children with severe visual impairment (SVI) using the Developmental Journal for babies and young children with visual impairment (DJVI). METHOD: A longitudinal observational study was undertaken with a national cohort (OPTIMUM) of infants with congenital disorders of the peripheral visual system (CDPVS) and profound-SVI; and followed up after 12 months and 24 months. Intervention was categorized according to the practitioner diary records of their usual practice over 12 months from baseline comparing those receiving the DJVI and those receiving 'Other Support'. Outcome measures of cognition and language, behaviour difficulties, parenting stress, and satisfaction with parent-practitioner partnership were collected. RESULTS: In the 54 children (26 males, 28 females, baseline mean age 13.5mo, SD 2.3mo, range 8-17mo) with 'total' CDPVS (including 16 'complex' and 38 'simple' with or without known brain disorder respectively), linear mixed effects pointed towards acceleration in sensorimotor understanding and expressive language especially in the 'simple' subsample (11.72 developmental quotient, 95% confidence interval -1.17 to 24.61, p>0.05) in those receiving the DJVI. Vision level also predicted outcomes (p<0.05). The DJVI group showed improvements in behavioural withdrawal (η2 =0.20, p=0.02, 'simple') and parenting stress (d=0.78, d=0.92, p=0.02 total and 'simple' respectively) and perceived practitioner-parent relationship (η2 =0.16, p=0.01). INTERPRETATION: Infants and young children with visual impairment receiving home-based early intervention using the DJVI with a structured developmental approach had better outcomes than those receiving 'other' home-based early interventions. Moderate to large effect improvements were found in child cognition and language, behaviour and parenting stress and the perceived practitioner-parent relationship, although cognition did not reach 5% significance level. WHAT THIS PAPER ADDS: Early intervention using the Developmental Journal for babies and young children with visual impairment was associated with enhanced developmental outcomes compared to other approaches. Improvements were also found in child behaviour, parenting stress, and perceived parent practitioner outcomes. Type and complexity of visual impairment also influenced outcomes.

INTERVENCIÓN TEMPRANA DOMICILIARIA EN LACTANTES Y NIÑOS PEQUEÑOS CON DISCAPACIDAD VISUAL USANDO EL DIARIO DE DESARROLLO: ESTUDIO LONGITUDINAL DE COHORTE: OBJETIVO: Investigar los efectos de la intervención temprana en el hogar en niños con discapacidad visual grave utilizando el Diario de Desarrollo para bebés y niños pequeños con discapacidad visual (DJVI). MÉTODO: Se realizó un estudio observacional longitudinal con una cohorte nacional (OPTIMUM) de bebés con trastornos congénitos del sistema visual periférico (CDPVS) y discapacidad visual profunda-severa; y seguimiento después de 12 meses y 24 meses. La intervención se categorizó de acuerdo con los registros del diario de desarrollo del médico en su práctica habitual más de 12 meses desde el inicio, comparando los que recibieron el DJVI y los que recibieron "otro apoyo". Se recopilaron las medidas de resultado de la cognición y el lenguaje, las dificultades de comportamiento, el estrés de los padres y la satisfacción con la asociación entre padres y profesionales. RESULTADOS: En los 54 niños (26 varones, 28 mujeres, edad media de referencia 13,5 meses, DS 2,3 meses, rango 8-17 meses) con CDPVS 'total' (incluidos 16 'complejo' y 38 'simple' con o sin trastorno cerebral conocido respectivamente), los efectos mixtos lineales apuntan hacia la aceleración en la comprensión sensoriomotora y el lenguaje expresivo, especialmente en la submuestra "simple" (cociente de desarrollo 11,72, intervalo de confianza del 95% -1,17 a 24,61, p>0,05) en los que recibieron el DJVI. El nivel de visión también predijo resultados (p <0,05). El grupo DJVI mostró mejoras en la abstinencia conductual (η2 =0,20, p=0,02, 'simple') y el estrés parental (d=0,78 − d=0,92, p=0,02 total y 'simple' respectivamente) y la relación percibida entre el médico y el padre (η2 =0,16, p=0,01). INTERPRETACIÓN: Los bebés y niños pequeños con discapacidad visual que recibieron una intervención temprana en el hogar utilizando el DJVI, con un enfoque de desarrollo estructurado, tuvieron mejores resultados que los que recibieron "otras" intervenciones tempranas en el hogar. Se encontraron mejoras de efecto moderado a grande en la cognición infantil y el lenguaje, el comportamiento y el estrés parental y la relación percibida entre el médico y el padre, aunque la cognición no alcanzó el nivel de significación del 5%.

INTERVENÇÃO PRECOCE DOMICILIAR EM LACTENTES E CRIANÇAS JOVENS COM DEFICIÊNCIA VISUAL USANDO O DEVELOPMENTAL JOURNAL: ESTUDO DE COORTE LONGITUDINAL: OBJETIVO: Investigar os efeitos da intervenção precoce domiciliar em crianças com deficiência visual severa usando o Developmental Journal para lactentes e crianças jovens com deficiência visual (DJDV). METODO: Um estudo observacional longitudinal foi realizado com uma coorte nacional (OPTIMUM) de crianças com distúrbios congênitos do sistema visual periférico (DCSVP) e deficiência visual grave-profunda, estes foram acompanhados após 12 meses e 24 meses. A intervenção foi categorizada de acordo com os registros diários do profissional de sua prática habitual ao longo de 12 meses, a partir de uma linha de base, comparando aqueles que receberam a DJDV e os que receberam "outro suporte". Resultados dos testes de cognição e linguagem, dificuldades de comportamento, estresse parental e satisfação com a parceria entre pais e profissionais, foram coletados. RESULTADOS: Nas 54 crianças (26 do sexo masculino e 28 do feminino, média de idade na linha de base de 13,5 meses; DP 2,3 meses; variação de 8 a 17 meses) com DCSVP total (incluindo 16 'complexos' e 38 'simples' com ou sem distúrbio cerebral conhecido, respectivamente), efeitos mistos lineares apontaram para um avanço na compreensão sensório-motora e de linguagem expressiva, especialmente, no subgrupo 'simples' (11,72 quociente de desenvolvimento, IC 95% -1,17 a 24,61; p>0,05) naqueles que receberam o DJDV. Nível visual também foi preditivo dos desfechos (p<0,05). O grupo DJDV apresentou melhora no comportamento de retraimento social (η2 =0,20; p=0,02; 'simples'), no estresse parental (d=0,78 − d=0,92; p=0,02 total e 'simples', respectivamente) e na percepção do relacionamento profissional-pais (η2 =0,16; p=0,01). INTERPRETAÇÃO: Lactentes e crianças jovens com deficiência visual que recebem intervenção domiciliar precoce usando a DJVI com uma abordagem de desenvolvimento estruturado tiveram resultados melhores do que aqueles que receberam "outras" intervenções precoces em casa. Melhorias com efeito de moderado a grande foram encontradas na cognição e linguagem, no comportamento infantil e estresse parental, e no relacionamento percebido entre pais e profissionais, embora a cognição não tenha alcançado nível de significância de 5%.

Adaptive behaviour and quality of life in school-age children with congenital visual disorders and different levels of visual impairment.

BACKGROUND: Adaptive behaviours are vital skills that allow individuals to function independently and are potentially amenable to behavioural interventions. Previous research indicated that adaptive behaviours are reduced in children and adolescents with severe to profound VI, but it was unclear if this was also the case for children with mild to moderate VI. AIM: The aim of the study was to assess differences in adaptive behaviour in children with congenital visual disorders and different levels of visual impairment and their influence on quality of life and everyday strengths and difficulties. METHODS AND PROCEDURES: Questionnaires about adaptive behaviour, strengths and difficulties, and quality of life were completed by parents of school-age children with severe-to-profound VI (S/PVI, n = 9, 0.9 logMAR - light perception only), mild-to-moderate VI (MVI, n = 9, 0.1-0.7 logMAR), or typical sight (control, n = 18, -0.3 to 0.1 logMAR). Differences in questionnaire domains by the severity of VI and relationships between adaptive behaviour and quality of life were analysed in general linear models. OUTCOMES AND RESULTS: The questionnaire ratings indicated reduced adaptive behaviour, more difficulties, and reduced quality of life in children with S/PVI compared to typically-sighted peers. Effects were smaller for children with MVI, but indicated a significant reduction in quality of life compared to typically-sighted children. The effect of visual impairment on quality of life in school was partially mediated by adaptive behaviour. CONCLUSION AND IMPLICATION: Severe congenital visual impairment affects adaptive behaviour in children with verbal abilities in the typical range. This effect is less pronounced in children with mild-to-moderate VI, but still impacts on quality of life, particularly in school.