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AIM: To synthesize existing evidence on the effectiveness of speech-language teleinterventions delivered via videoconferencing to users of augmentative and alternative communication (AAC) devices. METHOD: A systematic literature search was conducted in 10 electronic databases, from inception until August 2021. Included were speech-language teleinterventions delivered by researchers and/or clinicians via videoconferencing to users of AAC devices, without restrictions on chronological age and clinical diagnosis. The quality of the studies included in the review was appraised using the Downs and Black checklist and the Single-Case Experimental Design Scale; risk of bias was assessed using the Risk Of Bias In Non-Randomized Studies - of Interventions and the single-case design risk of bias tools. RESULTS: Six teleinterventions including 25 participants with a variety of conditions, such as Down syndrome, autism, Rett syndrome, and amyotrophic lateral sclerosis met the inclusion criteria. Five studies used a single-case experimental design and one was a cohort study. Teleinterventions included active consultation (n = 2), functional communication training (n = 2), brain-computer interface (n = 1), and both teleintervention and in-person intervention (n = 1). All teleinterventions reported an increase in participants' independent use of AAC devices during the training sessions compared to baseline, as well as an overall high satisfaction and treatment acceptability. INTERPRETATION: Speech-language teleinterventions for users of AAC devices show great potential for a successful method of service delivery. Future telehealth studies with larger sample sizes and more robust methodology are strongly encouraged to allow the generalization of results across different populations. WHAT THIS PAPER ADDS: Individuals can learn to use augmentative and alternative communication (AAC) devices independently during tele-AAC interventions. Service providers and recipients reported an overall high satisfaction and acceptability for AAC services delivered via teleinterventions. Speech-language teleinterventions may be an effective method of providing AAC intervention services.

Previous studies showed reduced activity of the anterior cingulate cortex (ACC) and supplementary motor area during inhibition in children with attention-deficit/hyperactivity disorder (ADHD). This study aimed to investigate deep brain generators underlying alterations of evoked potential components triggered by visual GO/NoGO tasks in children with ADHD compared with typically developing children (TDC). Standardized weighted low-resolution electromagnetic tomography (swLORETA) source analysis showed that lower GO-P3 component in children with ADHD was explained not only by a reduced contribution of the frontal areas but also by a stronger contribution of the anterior part of the caudate nucleus in these children compared with TDC. While the reduction of the NoGO-P3 component in children with ADHD was essentially explained by a reduced contribution of the dorsal ACC, the higher NoGO-P2 amplitude in these children was concomitant to the reduced contribution of the dorsolateral prefrontal cortex, the insula, and the cerebellum. These data corroborate previous findings showed by fMRI studies and offered insight relative to the precise time-related contribution of the caudate nucleus and the cerebellum during the automatic feature of inhibition processes in children with ADHD. These results were discussed regarding the involvement of the fronto-basal ganglia and fronto-cerebellum networks in inhibition and attention alterations in ADHD., SCOPUS: ar.j, info:eu-repo/semantics/published

BACKGROUND AND AIMS: Malnutrition is common in neurologically impaired (NI) children. It is, however, ill-defined and under-diagnosed. If not recognized and treated, it increases the burden of comorbidities and affects the quality of life of these children. The aim of this study was to characterize the nutritional status of a cohort of children followed up at a reference center for cerebral palsy (CP) in Brussels, Belgium, and to investigate possible links with the occurrence of comorbidities. MATERIAL AND METHODS: We conducted a single-center retrospective study including all the children followed up at the Inter-university Reference Center for Cerebral Palsy ULB-VUB-ULg. The data were obtained by reviewing medical files. Anthropometric measurements as well as the etiology of neurological impairment, comorbidities, feeding patterns, and laboratory test results were collected. The children were assigned a nutritional diagnosis according to the World Health Organization and Waterlow definitions. RESULTS: A total of 260 children with cerebral palsy were included, 148 males and 112 females. Their mean age was 10.9±4.3 years. The gross motor function classification system (GMFCS) level was I for 79 children, II for 63 children, III for 35 children, IV for 33 children, and V for 50 children. Of the children, 54% had a normal nutritional status, 34% showed malnutrition, and 8% were obese; 38% had oropharyngeal dysphagia. The sensitivity of mean upper arm circumference of

Objective. We aimed to analyze whether complexity of brain electrical activity (EEG) measured by multiscale entropy (MSE) increases with brain maturation during the first two years of life. We also aimed to investigate whether this complexity shows regional differences across the brain, and whether changes in complexity are influenced by extrauterine life experience duration. Approach. We measured MSE of EEG signals recorded longitudinally using a high-density setup (64 or 128 electrodes) in 84 typically developing infants born preterm (Main results. We found an increase of strong inter-channel correlation of MSE (R > 0.8) with increasing age. Regional analysis showed significantly increased MSE between 3 and 24 months of corrected age in the posterior and middle regions with respect to the anterior region. We found a weak relationship (adjusted R2 = 0.135) between MSE and extrauterine life duration. Significance. These findings suggest that brain functional connectivity increases with maturation during the first two years of life. EEG complexity shows regional differences with earlier maturation of the visual cortex and brain regions involved in joint attention than of regions involved in cognitive analysis, abstract thought, and social behavior regulation. Finally, our MSE analysis suggested only a weak influence of early extrauterine life experiences (prior to term age) on EEG complexity.

Polymicrogyria (PMG) is a heterogeneous brain malformation that may result from prenatal vascular disruption or infection, or from numerous genetic causes that still remain difficult to identify. We identified three unrelated patients with polymicrogyria and duplications of chromosome 2p, defined the smallest region of overlap, and performed gene pathway analysis using Cytoscape. The smallest region of overlap in all three children involved 2p16.1-p16.3. All three children have bilateral perisylvian polymicrogyria (BPP), intrauterine and postnatal growth deficiency, similar dysmorphic features, and poor feeding. Two of the three children had documented intellectual disability. Gene pathway analysis suggested a number of developmentally relevant genes and gene clusters that were over-represented in the critical region. We narrowed a rare locus for polymicrogyria to a region of 2p16.1-p16.3 that contains 33-34 genes, 23 of which are expressed in cerebral cortex during human fetal development. Using pathway analysis, we showed that several of the duplicated genes contribute to neurodevelopmental pathways including morphogen, cytokine, hormonal and growth factor signaling, regulation of cell cycle progression, cell morphogenesis, axonal guidance, and neuronal migration. These findings strengthen the evidence for a novel locus associated with polymicrogyria on 2p16.1-p16.3, and comprise the first step in defining the underlying genetic etiology.

OBJECTIVES: To investigate the operational competences screen navigation and dwell function underlying eye gaze performance, and the relation of dystonia and choreoathetosis with eye gaze performance in children with dyskinetic cerebral palsy (DCP). METHODS: During a 5-week intervention, ten participants with DCP played eye gaze video games daily for 30 minutes. Six games were used to assess task performance, fixation count, and eye movement accuracy during four measurements. Dystonia and choreoathetosis were evaluated using the Dyskinesia Impairment Scale. RESULTS: Eye gaze performance improved over time (p = .013). Moderate to strong within-subject correlations were found between eye movement accuracy and task performance, and between eye movement accuracy and fixation count. No significant correlations were found with the movement disorders. CONCLUSIONS: Eye gaze technology shows great potential to be a successful computer interface for children with severe DCP, thereby potentially improving their communication skills, participation levels, and quality of life. ispartof: Developmental Neurorehabilitation vol:23 issue:8 pages:548-556 ispartof: location:England status: Published online

AIM: To compare physiological age-relatedness between dyskinesia (dystonia/choreoathetosis), dystonia and ataxia rating scale scores in healthy children.METHOD: Three movement disorders specialists quantified dyskinetic-like features in healthy children (n = 52; 4-16 years) using the Dyskinesia Impairment Scale (DIS = DIS-choreoathetosis (DIS-C) + DIS-dystonia (DIS-D)). We compared the age-related regression coefficients of the DIS with data processed from previous studies on dystonia and ataxia rating scales (Burke-Fahn-Marsden Movement and Disability Scales (BFMMS and BFMDS) and Scale for Assessment and Rating of Ataxia (SARA), International Cooperative Ataxia Rating Scale (ICARS) and Brief Ataxia Rating Scale (BARS)).RESULTS: Dyskinetic scores were obtained in 79% (DIS); 65% (DIS-D) and 17% (DIS-C) versus dystonic and ataxic scores in 98% (BFMMS) and 89% (SARA/ICARS/BARS) of the children. Age-related DIS and DIS-D scores (B = -0.90 and 0.77; p < 0.001) were correlated with age-related BFMMS scores (B = -0.49; p < 0.001; r = 0.87; p < 0.001), whereas DIS-C scores were age-independent. Ataxic scores revealed stronger age-related regression coefficients than dyskinetic and dystonic scores (4-8 years; p < 0.05).INTERPRETATION: In healthy children, comparison between physiological dyskinesia, dystonia and ataxia rating scale scores revealed: 1. inverse age-relatedness for dystonic and ataxic scores, but not for choreoathetotic scores, 2. interrelated dystonic DIS-D and BFMMS scores, 3. the strongest age-related expression by ataxic scores. In healthy children, these physiological movement disorder-like features are interpreted as an expression of the developing underlying motor centres.

Cerebral palsy is the most frequent cause of severe physical disability in childhood. Dyskinetic cerebral palsy (DCP) is the second most common type of cerebral palsy after spastic forms. DCP is typically caused by non-progressive lesions to the basal ganglia or thalamus, or both, and is characterised by abnormal postures or movements associated with impaired tone regulation or movement coordination. In DCP, two major movement disorders, dystonia and choreoathetosis, are present together most of the time. Dystonia is often more pronounced and severe than choreoathetosis, with a major effect on daily activity, quality of life, and societal participation. The pathophysiology of both movement disorders is largely unknown. Some emerging hypotheses are an imbalance between indirect and direct basal ganglia pathways, disturbed sensory processing, and impaired plasticity in the basal ganglia. Rehabilitation strategies are typically multidisciplinary. Use of oral drugs to provide symptomatic relief of the movement disorders is limited by adverse effects and the scarcity of evidence that the drugs are effective. Neuromodulation interventions, such as intrathecal baclofen and deep brain stimulation, are promising options.

Executive functions include a group of cognitive processes that contribute to the regulation of purposeful reasoning and behaviour. They thus play a major role in an extremely wide range of our activities. Core components, which depend on one another to some extent, are inhibition, updating the content of working memory during the course of a task and cognitive flexibility in shifting between mental sets or reallocating attention to different tasks. The current construct of executive functioning is still largely based on empirical findings in adults. However, a growing body of data on executive function in typical development, early brain injury and a number of neurodevelopmental conditions (eg, attention-deficit hyperactivity disorder, autism spectrum disorder) provide insights into maturational factors as well as the role of experience. Assessment remains challenging and in this context Twomey and colleagues propose to use touch screen technology to evaluate early cognitive functioning with instruments that do not rely on verbal language.1 This has potentially high relevance for very young children with limited language development, like the participants in this study. It may also prove relevant to disabled children or adults whose language impairment would interfere with such testing. The applications that Twomey et al studied focused on selective attention, working memory, object permanence and learning, that is, skills that are pertinent to executive functioning and therefore to cognitive and social functioning. Twomey et al ’s approach is made complex by findings suggesting that experience with the touch screen media itself influences young children’s executive functioning abilities.2 Moreover, training of non-executive skills associated with such experience may also bias the results when measuring executive function in research or clinical …

Gait is an essential human activity which organizes many functional and cognitive behaviors. The biomechanical constraints of bipedalism implicating a permanent control of balance during gait are taken into account by a complex dialog between the cortical, subcortical and spinal networks. This networking is largely based on oscillatory coding, including changes in spectral power and phase-locking of ongoing neural activity in theta, alpha, beta and gamma frequency bands. This coding is specifically modulated in actual gait execution and representation, as well as in contexts of gait observation or imagination. A main challenge in integrative neuroscience oscillatory activity analysis is to disentangle the brain oscillations devoted to gait control. In addition to neuroimaging approaches, which have highlighted the structural components of an extended network, dynamic high-density EEG gives non-invasive access to functioning of this network. Here we revisit the neurophysiological foundations of behavior-related EEG in the light of current neuropsychological theoretic frameworks. We review different EEG rhythms emerging in the most informative paradigms relating to human gait and implications for rehabilitation strategies.

Introduction Preterm and very low birthweight infants are at increased risk for neurodevelopmental disorders, including cerebral palsy, sensory impairment and intellectual disability. Several early intervention approaches have been designed in the hope of optimising neurological development in this context. It seems important that the intervention takes into account parental mental health, focuses on parent-child interactions and lasts sufficiently long. This study aims to evaluate the effects of a stimulation programme administered by parents until 6 months post-term on motor and neurophysiological development of infants born preterm. Methods and analysis Participants will be infants born, SCOPUS: ar.j, info:eu-repo/semantics/published

SCOPUS: no.j, info:eu-repo/semantics/published

Efforts to document early changes in the developing brain have resulted in the construction of increasingly accurate structural images based on magnetic resonance imaging (MRI) in newborn infants. Tractography diagrams obtained through diffusion tensor imaging have focused on white matter microstructure, with particular emphasis on neuronal connectivity at the level of fibre tract systems. Electroencephalography (EEG) provides a complementary approach with more direct access to brain electrical activity. Its temporal resolution is excellent, and its spatial resolution can be enhanced to physiologically relevant levels, through the combination of high-density recordings (e.g. by using 64 channels in newborn infants) and mathematical models (e.g. inverse modelling computation), to identify generators of different oscillation bands and synchrony patterns. The integration of functional and structural topography of the neonatal brain provides insights into typical brain organization, and the deviations seen in particular contexts, for example the effect of hypoxic-ischaemic insult in terms of damage, eventual reorganization, and functional changes. Endophenotypes can then be used for pathophysiological reasoning, management planning, and outcome measurements, and allow a longitudinal approach to individual developmental trajectories.

In contrast to childhood ADHD that is characterized by inattention, impulsivity and hyperactivity, most adults with ADHD predominantly exhibit inattention. We used a new oddball paradigm using implicit navigational images and analyzed EEG dynamics with swLORETA inverse modeling of the evoked potential generators to study cortical processing in adults with ADHD and age-matched controls. In passive observation, we demonstrated that P350 amplitude, alpha-beta oscillation event-related synchronization (ERS) anticipation, and beta event-related desynchronization (ERD) were significantly smaller in ADHD. In the active condition, P100 duration was reduced and N140 amplitude increased for both deviant and frequent conditions in the ADHD. Alpha ERS and delta-theta ERS were reduced in the ADHD in the deviant condition. The left somatosensory area (BA2) and the right parietal lobe (BA31, BA40) contributed more to the P100 generators in the control than in the ADHD group, while the left frontal lobe (BA10) contributed more to the P100 generators in the ADHD. The left inferior parietal lobe (BA40) contributed more to the N140 generators in the control than the ADHD group while the right posterior cingulate (BA30) contributed more to the N140 generators in the ADHD. These findings reinforce the notion that earlier cortical stages of visual processing are compromised in adult ADHD by inducing the emergence of different even-related potential generators and EEG dynamics in ADHD. Considering that classical approaches for ADHD diagnosis are based on qualitative clinical investigation possibly biased by subjectivity, EEG analysis is another objective tool that might contribute to diagnosis, future neurofeedback or brain stimulation therapies.

BACKGROUND Although there is increasing recognition of the role of somatic mutations in genetic disorders, the prevalence of somatic mutations in neurodevelopmental disease and the optimal techniques to detect somatic mosaicism have not been systematically evaluated. METHODS Using a customized panel of known and candidate genes associated with brain malformations, we applied targeted high-coverage sequencing (depth, = 200x) to leukocyte-derived DNA samples from 158 persons with brain malformations, including the double-cortex syndrome (subcortical band heterotopia, 30 persons), polymicrogyria with megalencephaly (20), periventricular nodular heterotopia (61), and pachygyria (47). We validated candidate mutations with the use of Sanger sequencing and, for variants present at unequal read depths, subcloning followed by colony sequencing. RESULTS Validated, causal mutations were found in 27 persons (17%; range, 10 to 30% for each phenotype). Mutations were somatic in 8 of the 27 (30%), predominantly in persons with the double-cortex syndrome (in whom we found mutations in DCX and LIS1), persons with periventricular nodular heterotopia (FLNA), and persons with pachygyria (TUBB2B). Of the somatic mutations we detected, 5 (63%) were undetectable with the use of traditional Sanger sequencing but were validated through subcloning and subsequent sequencing of the subcloned DNA. We found potentially causal mutations in the candidate genes DYNC1H1, KIF5C, and other kinesin genes in persons with pachygyria. CONCLUSIONS Targeted sequencing was found to be useful for detecting somatic mutations in patients with brain malformations. High-coverage sequencing panels provide an important complement to whole-exome and whole-genome sequencing in the evaluation of somatic mutations in neuropsychiatric disease. (Funded by the National Institute of Neurological Disorders and Stroke and others.)

Background Identification and diagnosis of autism spectrum disorders is essentially based on behavioral presentation and developmental history. The current average age at diagnosis is older than 3 years. Methods Over the past 15 years, there has been increasing documentation of the early signs of autism spectrum disorders through both individual retrospective parental reports and screening studies. Recent longitudinal studies have focused on early medical and behavioral features of children regarded at risk, namely younger siblings of children with autism spectrum disorders or children who required neonatal intensive care, with a later diagnosis of autism spectrum disorders. Results Potentially useful early neurological signs and developmental predictors for autism spectrum disorders could be identified, with a typical profile that evolved with age. Conclusions Assessment of early social attention and communication skills with adapted scales in children before the age of 18 months in very large community-based settings may lead to high positive predictive values.

Most clinical tools for measuring spasticity, such as the Modified Ashworth Scale (MAS) and the Modified Tardieu Scale (MTS), are not sufficiently accurate or reliable. This study investigated the clinimetric properties of an instrumented spasticity assessment. Twenty-eight children with spastic cerebral palsy (CP) and 10 typically developing (TD) children were included. Six of the children with CP were retested to evaluate reliability. To quantify spasticity in the gastrocnemius (GAS) and medial hamstrings (MEH), three synchronized signals were collected and integrated: surface electromyography (sEMG); joint-angle characteristics; and torque. Muscles were manually stretched at low velocity (LV) and high velocity (HV). Spasticity parameters were extracted from the change in sEMG and in torque between LV and HV. Reliability was determined with intraclass-correlation coefficients and the standard error of measurement; validity by assessing group differences and correlating spasticity parameters with the MAS and MTS. Reliability was moderately high for both muscles. Spasticity parameters in both muscles were higher in children with CP than in TD children, showed moderate correlation with the MAS for both muscles and good correlation to the MTS for the MEH. Spasticity assessment based on multidimensional signals therefore provides reliable and clinically relevant measures of spasticity. Moreover, the moderate correlations of the MAS and MTS with the objective parameters further stress the added value of the instrumented measurements to detect and investigate spasticity, especially for the GAS. ispartof: Gait & Posture vol:38 issue:1 pages:141-147 ispartof: location:England status: published

Human brain adaptation in weightlessness follows the necessity to reshape the dynamic integration of the neural information acquired in the new environment. This basic aspect was here studied by the electroencephalogram (EEG) dynamics where oscillatory modulations were measured during a visuo-attentional state preceding a visuo-motor docking task. Astronauts in microgravity conducted the experiment in free-floating aboard the International Space Station, before the space flight and afterwards. We observed stronger power decrease (∼ERD: event related desynchronization) of the ∼10 Hz oscillation from the occipital-parietal (alpha ERD) to the central areas (mu ERD). Inverse source modelling of the stronger alpha ERD revealed a shift from the posterior cingulate cortex (BA31, from the default mode network) on Earth to the precentral cortex (BA4, primary motor cortex) in weightlessness. We also observed significant contribution of the vestibular network (BA40, BA32, and BA39) and cerebellum (lobule V, VI). We suggest that due to the high demands for the continuous readjustment of an appropriate body posture in free-floating, this visuo-attentional state required more contribution from the motor cortex. The cerebellum and the vestibular network involvement in weightlessness might support the correction signals processing necessary for postural stabilization, and the increased demand to integrate incongruent vestibular information., SCOPUS: ar.j, info:eu-repo/semantics/published

BACKGROUND: Children with cerebral palsy show dysfunctional postural control which interferes with their functional performance and daily-life activities. OBJECTIVE: The aim of the study was to identify the effect of a 3D supporting garment on trunk postural control and interjoint coordination during gait in children with bilateral cerebral palsy. METHODS: We analyzed tridimensional trunk motion, trunk-thigh and interjoint coordination in 15 4-10 year-old children with bilateral spastic cerebral palsy (GMFCS I or II) and 16 4-10 year-old typically developing children while walking with or without a supporting garment. RESULTS: We found significantly changes in the coordination between trunk and lower limbs in children with cerebral palsy. Step velocity and cadence both increased significantly in children with cerebral palsy but in controls, the cadence remained unaltered. Interjoint coordination between hip-knee and knee-ankle was altered during the stance phase only in the subgroup of children with cerebral palsy without any limitations in ankle joint passive range of motion. CONCLUSION: 3D supporting garments improve trunk-thigh and lower limb interjoint coordination in walking in children with bilateral cerebral palsy.

Aim To relate dystonia and choreoathetosis with activity, participation and quality of life (QOL) in children and youth with dyskinetic Cerebral Palsy (CP). Methods Fifty-four participants with dyskinetic CP (mean age 14y6m, SD 4y2m, range 6–22y) were included. The Dyskinesia Impairment Scale (DIS) was used to evaluate dystonia and choreoathetosis. Activity, participation and quality of life (QOL) were assessed with the Gross Motor Function Measure (GMFM), the Functional Mobility Scale (FMS), the Jebsen-Taylor Hand Function Test (JTT), the ABILHAND-Kids Questionnaire (ABIL-K), the Life Habits Kids (LIFE-H) and the Quality of Life Questionnaire for children with CP (CP-QOL). Spearman's rank correlation coefficient ( r s ) was used to assess the relationship between the movement disorders and activity, participation and QOL measures. Results Significant negative correlations were found between dystonia and the activity scales with Spearman's rank correlation coefficient ( r s ) varying between −0.65 (95% CI = −0.78 to −0.46) and −0.71 (95% CI = −0,82 to −0.55). Correlations were also found with the LIFE-H ( r s = −0.43; 95%CI = −0.64 to −0.17) and the CP-QOL ( r s = −0.32; 95%CI = −0.56 to −0.03). As far as choreoathetosis is concerned, no or only weak relationships were found with the activity, participation and quality of life scales. Interpretation This cross-sectional study is the first to examine the relationship of dystonia and choreoathetosis in dyskinetic CP with the level of activity, participation and QOL. The results revealed dystonia has a higher impact on activity, participation and quality of life than choreoathetosis. These findings seem to suggest it is necessary to first focus on dystonia reducing intervention strategies and secondly on choreoathetosis.