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This study investigated the reliability of 3-dimensional freehand ultrasound (3DfUS) to quantify the size (muscle volume [MV] and anatomical cross-sectional area [aCSA]), length (muscle length [ML], tendon length [TL], and muscle tendon unit length [MTUL]), and echo-intensity (EI, whole muscle and 50% aCSA), of lower limb muscles in children with spastic cerebral palsy (SCP) and typical development (TD). In total, 13 children with SCP (median age 14.3 (7.3) years) and 13 TD children (median age 11.1 (1.7) years) participated. 3DfUS scans of rectus femoris, semitendinosus, medial gastrocnemius, and tibialis anterior were performed by two raters in two sessions. The intra- and inter-rater and intra- and inter-session reliability were defined with relative and absolute reliability measures, that is, intra-class correlation coefficients (ICCs) and absolute and relative standard error of measurement (SEM and SEM%), respectively. Over all conditions, ICCs for muscle size measures ranged from 0.818 to 0.999 with SEM%s of 12.6%–1.6%. For EI measures, ICCs varied from 0.233 to 0.967 with SEM%s of 15.6%–1.7%. Length measure ICCs ranged from 0.642 to 0.999 with SEM%s of 16.0%–0.5%. In general, reliability did not differ between the TD and SCP cohort but the influence of different muscles, raters, and sessions was not constant for all 3DfUS parameters. Muscle length and muscle tendon unit length were the most reliable length parameters in all conditions. MV and aCSA showed comparable SEM%s over all muscles, where tibialis anterior MV was most reliable. EI had low-relative reliability, but absolute reliability was better, with better reliability for the distal muscles in comparison to the proximal muscles. Combining these results with earlier studies describing muscle morphology assessed in children with SCP, 3DfUS seems sufficiently reliable to determine differences between cohorts and functional levels. The applicability on an individual level, for longitudinal follow-up and after interventions is dependent on the investigated muscle and parameter. Moreover, the semitendinosus, the acquisition, and processing of multiple sweeps, and the definition of EI and TL require further investigation. In general, it is recommended, especially for longitudinal follow-up studies, to keep the rater the same, while standardizing acquisition settings and positioning of the subject.

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.

Introduction: Due to the heterogeneous clinical presentation of spastic cerebral palsy (SCP), which makes spasticity treatment challenging, more insight into the complex interaction between spasticity and altered muscle morphology is warranted. Aims: We studied associations between spasticity and muscle morphology and compared muscle morphology between commonly observed spasticity patterns (i.e. different muscle activation patterns during passive stretches). Methods: Spasticity and muscle morphology of the medial gastrocnemius (MG) and semitendinosus (ST) were defined in 74 children with SCP (median age 8 years 2 months, GMFCS I/II/III: 31/25/18, bilateral/unilateral: 46/27). Using an instrumented assessment, spasticity was quantified as the difference in muscle activation recorded during passive stretches at low and high velocities and was classified in mixed length-/velocity-dependent or pure velocity-dependent activation patterns. Three-dimensional freehand ultrasound was used to assess muscle morphology (volume and length) and echogenicity intensity (as a proxy for muscle quality). Spearman correlations and Mann-Whitney-U tests defined associations and group differences, respectively. Results: A moderate negative association (r = −0.624, p < 0.001) was found between spasticity and MG muscle volume, while other significant associations between spasticity and muscle morphology parameters were weak. Smaller normalized muscle volume (MG p = 0.004, ST p=

Aim: This study aimed to explore eye movements and stress during eye-tracking gaming performance in children with dyskinetic cerebral palsy (CP) compared with typically developing children, and associations between eye-tracking performance, eye movements, stress, and participants' characteristics. Method: This cohort study included 12 children with dyskinetic CP aged 5 to 12 years (mean age 8 years 7 months, standard deviation [SD] 2 years 3 months) and 23 typically developing children aged 5 to 13 years (mean age 9 years 0 months, SD 2 years 7 months). Participants played 10 eye-tracking games. Tobii X3-120 and Tobii Pro Lab were used to record and analyse eye movements. Stress was assessed through heart rate variability (HRV), recorded during rest, and eye-tracking performance using the Bittium Faros360° ECG Holter device. Eye-tracking performance was measured using gaming completion time. Fixation and saccade variables were used to quantify eye movements, and time- and frequency-domain variables to quantify HRV. Non-parametric statistics were used. Results: Gaming completion time was significantly different (p < 0.001) between groups, and it was negatively correlated with experience (r s = −0.63, p = 0.029). No significant differences were found between groups in fixation and saccade variables. HRV significantly changed from rest to eye-tracking performance only in typically developing children and not in children with dyskinetic CP. Interpretation: Children with dyskinetic CP took longer to perform the 10 games, especially the inexperienced users, indicating the importance of the early provision of eye-tracking training opportunities. It seems that eye-tracking tasks are not a source of increased stress and effort in children with dyskinetic CP. What this paper adds: Participants with dyskinetic cerebral palsy (CP) took twice as long to perform 10 eye-tracking games than typically developing peers. Participants with dyskinetic CP with previous eye-tracking experience performed the games faster. Fixation and saccade variables were not significantly different between children with and without dyskinetic CP. Heart rate variability showed no differences between rest and performance in participants with dyskinetic CP. Gross Motor Function Classification System, Manual Ability Classification System, and Viking Speech Scale levels were not correlated to the eye movements or stress variables.

Cerebral palsy is a lifelong neurodevelopmental condition arising from non-progressive disorders occurring in the fetal or infant brain. Cerebral palsy has long been categorised into discrete motor types based on the predominance of spasticity, dyskinesia, or ataxia. However, these motor disorders, muscle weakness, hypotonia, and impaired selective movements should also be discriminated across the range of presentations and along the lifespan. Although cerebral palsy is permanent, function changes across the lifespan, indicating the importance of interventions to improve outcomes in motor disorders associated with the condition. Mounting evidence exists for the inclusion of several interventions, including active surveillance, adapted physical activity, and nutrition, to prevent secondary and tertiary complications. Avenues for future research include the development of evidence-based recommendations, low-cost and high-quality alternatives to existing therapies to ensure universal access, standardised cerebral palsy registers to harmonise epidemiological and clinical information, improved adult screening and check-up programmes to facilitate positive lived experiences, and phase 3 trials for new interventions.

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.

We report a 50-year-old man with celiac disease who presented with occipital epilepsy. Brain MRI showed right occipital subcortical white matter hyperintensities, consistent with the posterior epileptic focus suggested by the clinical features of the seizures and documented on EEG. Shortly after the introduction of a gluten-free diet, the white matter abnormalities resolved. The patient went on to develop simultagnosia. Follow-up MRI showed right occipital lobe atrophy. This report emphasizes the importance of recognizing gluten-associated neurologic manifestations and usefulness of thegluten-free diet.

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.

The recent advances in the application of artificial neural networks in the biological field have been inspired by the functional organization of real biological structures (Draye et al.,1997a; Anastasio & Gad, 2007). The fascination exerted by the oculomotor system upon both engineers and neuroscientists have played an important role in this issue. In particular, since the definitive evidence of the existence of a neural integrator in the brainstem (Cheron et al., 1986a; Cannon & Robinson, 1987; Robinson, 1989 for a review) performing mathematical integration of the eye velocity into eye position signals, numerous artificial networks have been developed allowing a better understanding of the fundamental question of how the brain control movement. Such bio-mimetic strategy has recently permitted to elaborate different dynamic recurrent neural networks (DRNN) specifically dedicated to the command of humanoid robot (Tani et al., 2008). Hierarchical neuralinspired modules have also been proposed forming cascades of forward dynamics models (Jordan & Rumelhart, 1992; Kawato et al., 1987; Tani, 2003) in which top-down and bottomup influences allowed generating behavioural primitives. This Chapter describes the main steps performed in the development of our DRNN from the neural integrator models to those applied in the field of human movement control.

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.