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BACKGROUND: Accurate visual information is needed to guide and perform efficient movements in daily life. AIMS: To investigate the relation between visual functions, functional vision, and bimanual function in children with unilateral cerebral palsy (uCP). METHODS AND PROCEDURES: In 49 children with uCP (7-15 y), we investigated the relation between stereoacuity (Titmus Stereo Fly test), visual perception (Test of Visual Perceptual Skills), visuomotor integration (Beery Buktenica Test of Visual-Motor Integration) and functional vision (Flemish cerebral visual impairment questionnaire) with bimanual dexterity (Tyneside Pegboard Test), bimanual coordination (Kinarm exoskeleton robot, Box opening task), and functional hand use (Children's Hand-use Experience Questionnaire; Assisting Hand Assessment) using correlations (rs) and elastic-net regularized regressions (d). OUTCOMES AND RESULTS: Visual perception correlated with bimanual coordination (rs=0.407-0.436) and functional hand use (rs=0.380-0.533). Stereoacuity (rs=-0.404), visual perception (rs=-0.391 to -0.620), and visuomotor integration (rs=-0.377) correlated with bimanual dexterity. Functional vision correlated with functional hand use (rs=-0.441 to -0.458). Visual perception predicted bimanual dexterity (d=0.001-0.315), bimanual coordination (d=0.004-0.176), and functional hand use (d=0.001-0.345), whereas functional vision mainly predicted functional hand use (d=0.001-0.201). CONCLUSIONS AND IMPLICATIONS: Visual functions and functional vision are related to bimanual function in children with uCP highlighting the importance of performing extensive visual assessment to better understand children's difficulties in performing bimanual tasks. WHAT THIS PAPER ADDS: Previous findings showed that up to 62 % of children with unilateral cerebral palsy (uCP) present with visual impairment, which can further compromise their motor performance. However, the relation between visual and motor function has hardly been investigated in this population. This study makes a significant contribution to the literature by comprehensively investigating the multi-level relation between the heterogenous spectrum of visual abilities and bimanual function in children with uCP. We found that mainly decreased visual perception was related to decreased bimanual dexterity, bimanual coordination, and functional hand use while impairments in functional vision were only related to decreased functional hand use. Additionally, elastic-net regression models showed that visual assessments can predict bimanual function in children with uCP, however, effect sizes were only tiny to small. With our study, we demonstrated a relation between visual functions and bimanual function in children with uCP. These findings suggest the relevance of thoroughly examining visual functions in children with uCP to identify the presence of visual impairments that may further compromise their bimanual function.
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Parálisis Cerebral , Destreza Motora , Desempeño Psicomotor , Agudeza Visual , Percepción Visual , Humanos , Parálisis Cerebral/fisiopatología , Niño , Femenino , Masculino , Adolescente , Destreza Motora/fisiología , Desempeño Psicomotor/fisiología , Percepción Visual/fisiología , Agudeza Visual/fisiología , Mano/fisiopatología , Percepción de Profundidad/fisiologíaRESUMEN
AIM: To explore the effect of a 4-week structured power wheelchair skills training programme (WSTP) intervention on mobility skills and participation in children and young people (CYP) with cerebral palsy (CP). METHOD: This was a one-group, repeated-measures study; baseline, intervention, and retention phases, each lasting 4 weeks, were used. Twelve participants (three females, nine males) with a mean age of 15 years 11 months (SD = 3 years 6 months) classified in Gross Motor Function Classification System levels IV and V participated in the study. To be included in the study, individuals had to be aged 6 to 21 years and currently using a power wheelchair. Participants received 12 WSTP training sessions of 45 minutes, 3 times per week. Power mobility skills were assessed using the Wheelchair Skills Test (WST) before baseline, before the intervention, after the intervention, and at the follow-up; mobility-related participation was assessed with the Canadian Occupational Performance Measure (COPM). Generalized mixed models with Bonferroni correction were used to assess the differences between the assessment points (p < 0.05). RESULTS: Statistical analysis showed a 10.4% (12.5) increase in WST total scores (p < 0.001) after the intervention compared to before the intervention, and a 1-point (0.9) increase in the COPM performance subdomain (p = 0.002). INTERPRETATION: Power mobility skills and mobility-related participation improved after a 4-week WSTP intervention in CYP with CP. Thus, task-based power mobility skills training based on the WSTP, and in line with individualized needs and capabilities, taking place in a natural environment, should be recommended. Power mobility skills training needs to be structured and individualized; the training interventions must consider the individual, the task, and the environment.
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Automated quantification of brain tissues on MR images has greatly contributed to the diagnosis and follow-up of neurological pathologies across various life stages. However, existing solutions are specifically designed for certain age ranges, limiting their applicability in monitoring brain development from infancy to late adulthood. This retrospective study aims to develop and validate a brain segmentation model across pediatric and adult populations. First, we trained a deep learning model to segment tissues and brain structures using T1-weighted MR images from 390 patients (age range: 2-81 years) across four different datasets. Subsequently, the model was validated on a cohort of 280 patients from six distinct test datasets (age range: 4-90 years). In the initial experiment, the proposed deep learning-based pipeline, icobrain-dl, demonstrated segmentation accuracy comparable to both pediatric and adult-specific models across diverse age groups. Subsequently, we evaluated intra- and inter-scanner variability in measurements of various tissues and structures in both pediatric and adult populations computed by icobrain-dl. Results demonstrated significantly higher reproducibility compared to similar brain quantification tools, including childmetrix, FastSurfer, and the medical device icobrain v5.9 (p-value< 0.01). Finally, we explored the potential clinical applications of icobrain-dl measurements in diagnosing pediatric patients with Cerebral Visual Impairment and adult patients with Alzheimer's Disease.
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Encéfalo , Aprendizaje Profundo , Imagen por Resonancia Magnética , Humanos , Adulto , Encéfalo/diagnóstico por imagen , Anciano , Niño , Adolescente , Preescolar , Anciano de 80 o más Años , Persona de Mediana Edad , Adulto Joven , Femenino , Masculino , Imagen por Resonancia Magnética/métodos , Estudios Retrospectivos , Procesamiento de Imagen Asistido por Computador/métodos , Reproducibilidad de los ResultadosRESUMEN
Most people with intellectual disability (ID) do not receive a molecular diagnosis following genetic testing. To identify new etiologies of ID, we performed a genetic association analysis comparing the burden of rare variants in 41,132 noncoding genes between 5,529 unrelated cases and 46,401 unrelated controls. RNU4-2, which encodes U4 small nuclear RNA, a critical component of the spliceosome, was the most strongly associated gene. We implicated de novo variants among 47 cases in two regions of RNU4-2 in the etiology of a syndrome characterized by ID, microcephaly, short stature, hypotonia, seizures and motor delay. We replicated this finding in three collections, bringing the number of unrelated cases to 73. Analysis of national genomic diagnostic data showed RNU4-2 to be a more common etiological gene for neurodevelopmental abnormality than any previously reported autosomal gene. Our findings add to the growing evidence of spliceosome dysfunction in the etiologies of neurological disorders.
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Discapacidad Intelectual , Mutación , Trastornos del Neurodesarrollo , ARN Nuclear Pequeño , Humanos , ARN Nuclear Pequeño/genética , Trastornos del Neurodesarrollo/genética , Discapacidad Intelectual/genética , Mutación/genética , Femenino , Masculino , Empalmosomas/genética , Microcefalia/genética , Microcefalia/epidemiología , Estudios de Asociación Genética , NiñoRESUMEN
AIM: To describe the aetiological risk factors, clinical characteristics, access to rehabilitation, and educational status of children with cerebral palsy (CP) in Suriname. METHOD: Hospital-based surveillance of children with CP aged younger than 18 years was conducted at the Academic Hospital Paramaribo, Suriname (known as the Suriname CP Register [SUR-CPR]). Data were collected on sociodemographic characteristics, aetiological risk factors, clinical characteristics, rehabilitation, and educational status. Registry data on aetiological risk factors were compared with available national prevalence rates in Suriname. Descriptive statistics were reported. RESULTS: Between August 2018 and March 2020, 82 children with CP (mean [SD] age 5 years 10 months [3 years 10 months]) attending the Academic Hospital Paramaribo were registered in the SUR-CPR. The mean (SD) age at diagnosis was 5 years 5 months (4 years 1 month). Spastic CP was predominant in 90.8% of children and 58.8% were classified in Gross Motor Function Classification System levels III to V. Overall, 43.9% had preterm birth compared with 13.9% reported nationally (p < 0.001) and 61.6% had birth-related complications compared with 15% reported nationally (p < 0.001). Additionally, 39.1% had birth asphyxia and 23.2% had early feeding difficulties. Sixty-two percent were admitted to the neonatal intensive care unit, 54.0% of whom required ventilation. Most children (82.5%) had CP acquired pre- or perinatally and 17.5% had CP acquired postneonatally. Seventeen percent had never received any rehabilitation services, and 31.9% of the school-aged children were not enrolled in any education system. INTERPRETATION: The high burden of known aetiological risk factors, delayed diagnosis, and severe functional impairment among children with CP registered at the Academic Hospital Paramaribo is concerning. Public health interventions targeting early diagnosis and early intervention could improve the functional outcome of children with CP in Suriname.
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BACKGROUND: Besides motor impairments, up to 90% of the children and adolescents with unilateral cerebral palsy (uCP) present with somatosensory impairments in the upper limb. As somatosensory information is of utmost importance for coordinated movements and motor learning, somatosensory impairments can further compromise the effective use of the impaired upper limb in daily life activities. Yet, intervention approaches specifically designated to target these somatosensory impairments are insufficiently investigated in children and adolescents with uCP. Therefore, the aim of this randomized controlled trial (RCT) is to compare the effectiveness of somatosensory discrimination therapy and dose-matched motor therapy to improve sensorimotor upper limb function in children and adolescents with uCP, who experience somatosensory impairments in the upper limb. We will further explore potential behavioral and neurological predictors of therapy response. METHODS: A parallel group, evaluator-blinded, phase-II, single-center RCT will be conducted for which 50 children and adolescents with uCP, aged 7 to 15 years, will be recruited. Participants will be randomized to receive 3 weekly sessions of 45 minutes of either somatosensory discrimination therapy or upper limb motor therapy for a period of 8 weeks. Stratification will be performed based on age, manual ability, and severity of tactile impairment at baseline. Sensorimotor upper limb function will be evaluated at baseline, immediately after the intervention and after 6 months follow-up. The primary outcome measure will be bimanual performance as measured with the Assisting Hand Assessment. Secondary outcomes include a comprehensive test battery to objectify somatosensory function and measures of bimanual coordination, unimanual motor function, and goal attainment. Brain imaging will be performed at baseline to investigate structural brain lesion characteristics and structural connectivity of the white matter tracts. DISCUSSION: This protocol describes the design of an RCT comparing the effectiveness of somatosensory discrimination therapy and dose-matched motor therapy to improve sensorimotor upper limb function in children and adolescents with uCP. The results of this study may aid in the selection of the most effective upper limb therapy, specifically for children and adolescents with tactile impairments. TRIAL REGISTRATION: ClinicalTrials.gov (NCT06006065). Registered on August 8, 2023.
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Parálisis Cerebral , Niño , Humanos , Adolescente , Parálisis Cerebral/diagnóstico , Parálisis Cerebral/terapia , Extremidad Superior , Mano , Encéfalo , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
Children born very preterm (VPT, < 32 weeks of gestation) have an increased risk of developing socio-emotional difficulties. Possible neural substrates for these socio-emotional difficulties are alterations in the structural connectivity of the social brain due to premature birth. The objective of the current study was to study microstructural white matter integrity in VPT versus full-term (FT) born school-aged children along twelve white matter tracts involved in socio-emotional processing. Diffusion MRI scans were obtained from a sample of 35 VPT and 38 FT 8-to-12-year-old children. Tractography was performed using TractSeg, a state-of-the-art neural network-based approach, which offers investigation of detailed tract profiles of fractional anisotropy (FA). Group differences in FA along the tracts were investigated using both a traditional and complementary functional data analysis approach. Exploratory correlations were performed between the Social Responsiveness Scale (SRS-2), a parent-report questionnaire assessing difficulties in social functioning, and FA along the tract. Both analyses showed significant reductions in FA for the VPT group along the middle portion of the right SLF I and an anterior portion of the left SLF II. These group differences possibly indicate altered white matter maturation due to premature birth and may contribute to altered functional connectivity in the Theory of Mind network which has been documented in earlier work with VPT samples. Apart from reduced social motivation in the VPT group, there were no significant group differences in reported social functioning, as assessed by SRS-2. We found that in the VPT group higher FA values in segments of the left SLF I and right SLF II were associated with better social functioning. Surprisingly, the opposite was found for segments in the right IFO, where higher FA values were associated with worse reported social functioning. Since no significant correlations were found for the FT group, this relationship may be specific for VPT children. The current study overcomes methodological limitations of previous studies by more accurately segmenting white matter tracts using constrained spherical deconvolution based tractography, by applying complementary tractometry analysis approaches to estimate changes in FA more accurately, and by investigating the FA profile along the three components of the SLF.
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Nacimiento Prematuro , Sustancia Blanca , Niño , Femenino , Humanos , Recién Nacido , Sustancia Blanca/diagnóstico por imagen , Imagen de Difusión Tensora , Encéfalo/diagnóstico por imagen , Imagen de Difusión por Resonancia MagnéticaRESUMEN
AIM: To investigate visual (perceptual) function and functional vision in children with unilateral cerebral palsy (CP) and children with neurotypical development (NTD). METHOD: Fifty children with unilateral CP (mean age 11 years 11 months, SD 2 years 10 months, range 7-15 years; 27 males; 26 left-sided unilateral CP; Manual Ability Classification System [MACS] levels: I, 27; II, 16; III, 7) and 50 age- and sex-matched children with NTD participated in a cross-sectional study. Visual acuity, stereoacuity, and visual-perceptual functions were measured with standardized clinical tests. Functional vision was assessed in children with unilateral CP with the Flemish cerebral visual impairment questionnaire (FCVIQ). Group differences were investigated with Mann-Whitney U tests, Kruskal-Wallis tests, and the relative effect sizes r, η2 respectively. Correlations between visual assessments and the FCVIQ were investigated with Spearman's rank correlations. RESULTS: The total group of children with unilateral CP showed reduced visual acuity compared with children with NTD (p = 0.02, r = 0.23). Only children with left-sided unilateral CP scored lower than those with NTD on stereoacuity (p < 0.01, r = 0.36). Children with right/left-sided unilateral CP scored significantly lower than those with NTD on visual-perceptual functions (p = 0.001-0.02), with large effect sizes on visuomotor integration and visual closure (both r = 0.57). Children with unilateral CP classified in MACS level III showed significantly lower scores on visual-perceptual assessments than children classified in MACS level I. Stereoacuity and visual-perceptual functions negatively correlated with the FCVIQ, with the highest association with visual (dis)interest and anxiety-related behaviours. INTERPRETATION: Multi-level visual profiling is warranted in the clinical intake of children with unilateral CP to detect visual impairments that further compromise their level of functioning.
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Parálisis Cerebral , Agudeza Visual , Percepción Visual , Humanos , Niño , Parálisis Cerebral/fisiopatología , Parálisis Cerebral/complicaciones , Femenino , Masculino , Adolescente , Estudios Transversales , Agudeza Visual/fisiología , Percepción Visual/fisiología , Trastornos de la Visión/fisiopatología , Trastornos de la Visión/etiologíaRESUMEN
OBJECTIVE: Our objective was to investigate the executive function and its relationship with gestational age, sex, maternal education, and neurodevelopmental outcome at 2 years corrected age in children born preterm. METHOD: Executive function was assessed by means of the Multisearch Multilocation Task (MSML), Reversed Categorization Task (RevCat), and Snack Delay Task (SDT). Infant and maternal characteristics were gathered from the child's record. The developmental outcome was measured by the Bayley Scales and a multidisciplinary risk evaluation for autism. RESULTS: The executive function battery was completed by 97 children. The majority were able to successfully complete the MSML and SDT but failed RevCat. The lower the gestational age and the maternal education, the lower the executive function scores. Better cognition and motor function, as well as low autism risk, were associated with better executive function scores. Executive function was not related to sex. INTERPRETATION: This cohort study provides evidence that it is feasible to assess executive function in 2-year-olds born preterm. Executive function is related to gestational age and maternal education and is positively correlated with behavioral outcome. Therefore, executive functions can be a valuable target for early intervention, resulting in improvements in neurodevelopmental outcomes in children born preterm.
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Cognición , Función Ejecutiva , Recién Nacido , Lactante , Niño , Humanos , Preescolar , Estudios de Cohortes , Edad GestacionalRESUMEN
AIM: To define the longitudinal trajectory of gastrocnemius muscle growth in 6- to 36-month-old children with and without spastic cerebral palsy (SCP) and to compare trajectories by levels of gross motor function (Gross Motor Function Classification System, GMFCS) and presumed brain-lesion timing. METHOD: Twenty typically developing children and 24 children with SCP (GMFCS levels I-II/III-IV = 15/9), were included (28/16 females/males; mean age at first scan 15.4 months [standard deviation 4.93, range 6.24-23.8]). Three-dimensional freehand ultrasound was used to repeatedly assess muscle volume, length, and cross-sectional area (CSA), resulting in 138 assessments (mean interval 7.9 months). Brain lesion timing was evaluated with magnetic resonance imaging classification. Linear mixed-effects models defined growth rates, adjusted for GMFCS levels and presumed brain-lesion timing. RESULTS: At age 12 months, children with SCP showed smaller morphological muscle size than typically developing children (5.8 mL vs 9.8 mL, p < 0.001), while subsequently no differences in muscle growth were found between children with and without SCP (muscle volume: 0.65 mL/month vs 0.74 mL/month). However, muscle volume and CSA growth rates were lower in children classified in GMFCS levels III and IV than typically developing children and those classified in GMFCS levels I and II, with differences ranging from -56% to -70% (p < 0.001). INTERPRETATION: Muscle growth is already hampered during infancy in SCP. Muscle size growth further reduces with decreasing functional levels, independently from the brain lesion. Early monitoring of muscle growth combined with early intervention is needed.
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Parálisis Cerebral , Músculo Esquelético , Niño , Masculino , Femenino , Humanos , Lactante , Preescolar , Músculo Esquelético/patología , Imagen por Resonancia MagnéticaRESUMEN
OBJECTIVES: Guanine nucleotide-binding protein alpha-activating activity polypeptide O (GNAO1) syndrome, a rare congenital monogenetic disorder, is characterized by a neurodevelopmental syndrome and the presence of dystonia. Dystonia can be very pronounced and even lead to a life-threatening status dystonicus. In a small number of pharmaco-refractory cases, deep brain stimulation (DBS) has been attempted to reduce dystonia. In this study, we summarize the current literature on outcome, safety, and outcome predictors of DBS for GNAO1-associated dystonia. MATERIALS AND METHODS: We conducted a systematic review and meta-analysis on individual patient data. We included 18 studies describing 28 unique patients. RESULTS: The mean age of onset of symptoms was 2.4 years (SD 3.8); 16 of 28 patients were male, and dystonia was nearly always generalized (20/22 patients). Symptoms were present before DBS for a median duration of 19.5 months, although highly variable, occurring between 3 and 168 months. The exact phenotype, genotype, and radiologic abnormalities varied and seemed to be of little importance in terms of DBS outcome. All studies described an improvement in dystonia. Our meta-analysis focused on pallidal DBS and found an absolute and relative improvement in Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) of 32.5 points (37.9%; motor part; p = 0.001) and 5.8 points (21.5%; disability part; p = 0.043) at last follow-up compared with preoperative state; 80% of patients were considered responders (BFMDRS-M reduction by ≥25%). Although worsening over time does occur, an improvement was still observed in patients after >10 years. All reported cases of status dystonicus resolved after DBS surgery. Skin erosion and infection were observed in 18% of patients. CONCLUSION: Pallidal DBS can be efficacious and safe in GNAO1-associated dystonia.
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Estimulación Encefálica Profunda , Distonía , Trastornos Distónicos , Trastornos Heredodegenerativos del Sistema Nervioso , Preescolar , Femenino , Humanos , Masculino , Distonía/genética , Distonía/terapia , Trastornos Distónicos/genética , Trastornos Distónicos/terapia , Globo Pálido/fisiología , Subunidades alfa de la Proteína de Unión al GTP Gi-Go , Resultado del Tratamiento , Recién Nacido , Lactante , NiñoRESUMEN
OBJECTIVE: The structure-function relation between magnetic resonance imaging (MRI) and visual impairment (VI) in children with cerebral palsy (CP) has not been fully unravelled. The present systematic review aims to summarize the relation between brain lesions on MRI and VI in children and adolescents with CP. METHODS: PubMed, Embase, Web of Science Core Collection, and Cochrane Database were systematically searched according to the PRISMA checklist. A total of 45 articles met the inclusion criteria. RESULTS: White matter lesions were most frequently associated with VI. Only 25 studies described lesions within specific structures, mainly in the optic radiations. Only four studies reported on the thalamus. 8.4% of children with CP showed no brain abnormalities on MRI. Diffusion-weighted MRI studies showed that decreased structural connectivity in the optic radiations, superior longitudinal fasciculus, posterior limb of the internal capsule, and occipital lobe is associated with more severe VI. CONCLUSIONS: All types of brain lesions lead to visual dysfunctions, arguing for a comprehensive visual assessment in all children with CP. Whereas white matter damage is a well-known contributor, the exact contribution of specific visual structures requires further investigation, to enable early prediction, detection, and intervention.
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Parálisis Cerebral , Niño , Humanos , Adolescente , Parálisis Cerebral/complicaciones , Parálisis Cerebral/diagnóstico por imagen , Imagen de Difusión por Resonancia Magnética , Imagen por Resonancia Magnética , Neuroimagen , Trastornos de la Visión/diagnóstico por imagen , Trastornos de la Visión/etiología , EncéfaloRESUMEN
BACKGROUND: Robots have been proposed as tools to measure bimanual coordination in children with unilateral cerebral palsy (uCP). However, previous research only examined one task and clinical interpretation remains challenging due to the large amount of generated data. This cross-sectional study aims to examine bimanual coordination by using multiple bimanual robotics tasks in children with uCP, and their relation to task execution and unimanual performance. METHODS: The Kinarm exoskeleton robot was used in 50 children with uCP (mean age: 11 years 11 months ± 2 years 10 months, Manual Ability Classification system (MACS-levels: l = 27, ll = 16, lll = 7)) and 50 individually matched typically developing children (TDC). All participants performed three tasks: object-hit (hit falling balls), ball-on-bar (balance a ball on a bar while moving to a target) and circuit task (move a cursor along a circuit by making horizontal and vertical motions with their right and left hand, respectively). Bimanual parameters provided information about bimanual coupling and interlimb differences. Differences between groups and MACS-levels were investigated using ANCOVA with age as covariate (α < 0.05, [Formula: see text]). Correlation analysis (r) linked bimanual coordination to task execution and unimanual parameters. RESULTS: Children with uCP exhibited worse bimanual coordination compared to TDC in all tasks (p ≤ 0.05, [Formula: see text] = 0.05-0.34). The ball-on-bar task displayed high effect size differences between groups in both bimanual coupling and interlimb differences (p < 0.001, [Formula: see text] = 0.18-0.36), while the object-hit task exhibited variations in interlimb differences (p < 0.001, [Formula: see text] = 0.22-0.34) and the circuit task in bimanual coupling (p < 0.001, [Formula: see text] = 0.31). Mainly the performance of the ball-on-bar task (p < 0.05, [Formula: see text] = 0.18-0.51) was modulated by MACS-levels, showing that children with MACS-level lll had worse bimanual coordination compared to children with MACS-level l and/or II. Ball-on-bar outcomes were highly related to task execution (r = - 0.75-0.70), whereas more interlimb differences of the object-hit task were moderately associated with a worse performance of the non-dominant hand (r = - 0.69-(- 0.53)). CONCLUSION: This study gained first insight in important robotic tasks and outcome measures to quantify bimanual coordination deficits in children with uCP. The ball-on-bar task showed the most discriminative ability for both bimanual coupling and interlimb differences, while the object-hit and circuit tasks are unique to interlimb differences and bimanual coupling, respectively.
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Parálisis Cerebral , Dispositivo Exoesqueleto , Robótica , Humanos , Niño , Parálisis Cerebral/complicaciones , Estudios Transversales , ManoRESUMEN
OBJECTIVE: Lumbosacral selective dorsal rhizotomy is a neurosurgical treatment option to reduce spasticity in the lower extremities in children with cerebral palsy. Surprisingly, concomitant improvement of spasticity in the upper extremities and functionality of the hands has been sporadically reported postoperatively. In this systematic review, the authors aimed to quantify the postoperative improvement in upper-extremity spasticity and functionality, identify predictors, and discuss underlying mechanisms. METHODS: The authors searched the MEDLINE and Embase databases for studies reporting upper-extremity outcomes in cerebral palsy patients after selective dorsal rhizotomy that reported one or more of the following clinical scales: the Ashworth Scale (AS), the Modified AS (MAS), the fine motor skills domain of the Peabody Developmental Motor Scales (PDMS), the Quality of Upper Extremity Skills Test (QUEST), the self-care domain of the Functional Independence Measure for Children (WeeFIM), or the self-care domain of the Pediatric Evaluation of Disability Inventory (PEDI). The authors arbitrarily divided postoperative follow-up into short-term (< 6 months), medium-term (6-24 months), and long-term (> 24 months) follow-up. A 1-point change in MAS score has been reported as clinically significant. To assess bias, the Cochrane Collaboration's tool and ROBINS-I tool were used. RESULTS: The authors included 24 articles describing 752 patients. Spasticity reduction of the upper extremities ranged from 0.30 to 0.55 (AS) and between 0 and 2.9 (MAS) at medium-term follow-up. This large variability may partially be attributed to a floor effect since patients with normal upper-extremity function would not be expected to have further improvement. QUEST improvement ranged from 2.7% to 4.5% at medium-term follow-up. The mean improvements in functional skills of the self-care domain of the PEDI were 4.3 at short-term and 7 at medium-term follow-ups and ranged from 10.8 to 34.7 at long-term follow-up. There are insufficient data to draw meaningful conclusions regarding the PDMS fine motor skills and the WeeFIM self-care domains. CONCLUSIONS: The literature suggests that a pronounced postoperative spasticity reduction in the lower extremities and a moderately severe preoperative upper-extremity spasticity may positively predict postoperative reduction in upper-extremity spasticity. There are at least 5 hypotheses that may explain the postoperative reduction in upper-extremity spasticity and functionality: 1) a somatosensory cortex reorganization favoring the hand region over the leg region, 2) a decrease in abnormal electrical transmission throughout the spinal cord, 3) an indirect result of improved posture due to improved truncal and leg stability, 4) an indirect consequence of occupational/physical therapy intensification, and 5) a maturation effect. However, all remain unproven to date.
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Parálisis Cerebral , Rizotomía , Niño , Humanos , Rizotomía/efectos adversos , Parálisis Cerebral/complicaciones , Parálisis Cerebral/cirugía , Espasticidad Muscular/cirugía , Espasticidad Muscular/complicaciones , Destreza Motora , Mano , Resultado del TratamientoRESUMEN
BACKGROUND AND OBJECTIVES: To report on prevalence, associated impairments, severity, and neuroimaging findings in children with ataxic cerebral palsy (CP). METHODS: In children coded as having ataxic CP in the Central database of Joint Research Center-Surveillance of Cerebral Palsy in Europe (JRC-SCPE) and born during 1980-2010, birth characteristics, severity profiles including associated impairments, neuroimaging patterns, and the presence of syndromes were analyzed. Definitions were according to validated SCPE guidelines. Prevalence over time was estimated using Poisson regression. RESULTS: In total, 679 children with ataxic CP were identified in 20 European CP registers. The proportion with ataxic CP was 3.8% and varied from 0% to 12.9%. Prevalence over time showed no significant trend. Approximately 70% of children with ataxic CP were able to walk, and 40% had severe intellectual impairment and a high impairment index. Children with ataxic CP were mostly born at term (79%) and with normal birth weight (77%). Neuroimaging patterns revealed normal findings in 29%, brain maldevelopments in 28.5%, miscellaneous findings in 23.5%, and brain injuries in 19%, according to the SCPE classification. Genetic syndromes were described in 9%. DISCUSSION: This register-based multicenter study on children with ataxic CP provides a large sample size for the analysis of prevalence, severity, and origin of this rare CP subtype. Even with strict inclusion and classification criteria, there is variation between registers on how to deal with this subtype, and diagnosis of ataxic CP remains a challenge. Ataxic cerebral palsy differs from other CP subtypes: children with ataxic CP have a disability profile that is more pronounced in terms of cognitive than gross motor dysfunction. They are mostly term born and the origin rarely suggests acquired injuries. In addition to neuroimaging, a comprehensive genetic workup is particularly recommended for children with this CP type.
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Parálisis Cerebral , Niño , Humanos , Parálisis Cerebral/diagnóstico por imagen , Parálisis Cerebral/epidemiología , Parálisis Cerebral/genética , Prevalencia , Europa (Continente)/epidemiología , Neuroimagen , Sistema de RegistrosRESUMEN
OBJECTIVE: To survey the incidence of intraventricular hemorrhage (IVH) and periventricular leukomalacia (PVL) by gestational age and to report the impact on mortality and neurodevelopmental outcome in very preterm/very low birthweight infants. STUDY DESIGN: This was a population-based cohort study of 1927 very preterm/very low birthweight infants born in 2014-2016 and admitted to Flemish neonatal intensive care units. Infants underwent standard follow-up assessment until 2 years corrected age with the Bayley Scales of Infant and Toddler Development and neurological assessments. RESULTS: No brain lesion was present in 31% of infants born at <26 weeks of gestation and 75.8% in infants born at 29-32 weeks of gestation. The prevalence of low-grade IVH/PVL (grades I and II) was 16.8% and 12.7%, respectively. Low-grade IVH/PVL was not related significantly to an increased likelihood of mortality, motor delay, or cognitive delay, except for PVL grade II, which was associated with a 4-fold increase in developing cerebral palsy (OR, 4.1; 95% CI, 1.2-14.6). High-grade lesions (III-IV) were present in 22.0% of the infants born at <26 weeks of gestational and 3.1% at 29-32 weeks of gestation, and the odds of death were ≥14.0 (IVH: OR, 14.0; 95% CI, 9.0-21.9; PVL: OR, 14.1; 95% CI, 6.6-29.9). PVL grades III-IV showed an increased odds of 17.2 for motor delay and 12.3 for cerebral palsy, but were not found to be associated significantly with cognitive delay (OR, 2.9; 95% CI, 0.5-17.5; P = .24). CONCLUSIONS: Both the prevalence and severity of IVH/PVL decreased significantly with advancing gestational age. More than 75% of all infants with low grades of IVH/PVL showed normal motor and cognitive outcome at 2 years corrected age. High-grade PVL/IVH has become less common and is associated with adverse outcomes.
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Parálisis Cerebral , Enfermedades del Prematuro , Leucomalacia Periventricular , Recién Nacido , Lactante , Humanos , Niño , Leucomalacia Periventricular/epidemiología , Recien Nacido Extremadamente Prematuro , Parálisis Cerebral/etiología , Estudios de Cohortes , Estudios Prospectivos , Recién Nacido de muy Bajo Peso , Hemorragia Cerebral/epidemiología , Hemorragia Cerebral/complicaciones , Enfermedades del Prematuro/epidemiologíaRESUMEN
AIM: To report on the prevalence, neuroimaging patterns, and function of children with cerebral palsy (CP) in Belgium for birth years 2007-2012, and identify distinctive risk indicators and differences in outcome between CP subtypes. METHODS: Antenatal and perinatal/neonatal factors, motor and speech function, associated impairments, and neuroimaging patterns were extracted from the Belgian Cerebral Palsy Register. Prevalence was estimated per 1000 (overall, ante/perinatal, spastic, dyskinetic CP) or 10,000 (post-neonatal, ataxic CP) live births. Multinomial logistic regression analyses were performed to ascertain the effects of antenatal/perinatal/neonatal factors and neuroimaging patterns on the likelihood of dyskinetic or ataxic CP relative to spastic CP, and test the likelihood of the occurrence of impaired motor and speech function and associated impairments in dyskinetic or ataxic CP relative to spastic CP. RESULTS: In total, 1127 children with CP were identified in Belgium. The birth prevalence of overall CP was 1.48 per 1000 live births. The likelihood of dyskinetic CP increases if the child was born to a mother aged ≥35 years, mechanically ventilated, and had predominant grey matter injury, while an increased likelihood of ataxic CP is associated with ≥2 previous deliveries. Children with dyskinetic and ataxic CP are more likely to function with impairments in motor, speech, and intellectual abilities. CONCLUSION: Distinctive risk indicators and differences in outcome between CP subtypes were identified. These factors can be incorporated into clinical practice to facilitate early, accurate, and reliable classification of CP subtype, and may lead to individually tailored neonatal care and other (early) intervention options.
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AIM: To systematically review the effectiveness of wheeled mobility interventions in children and young people with cerebral palsy (CP). METHOD: A systematic literature search was performed in MEDLINE, Embase, Cochrane Central Register of Controlled Trials, EBSCO, PEDro, and Web of Science using database-specific concepts such as 'child' and 'wheelchair'. Studies focusing on wheeled mobility skill interventions with participants aged 6 to 21 years with CP were included. RESULTS: Twenty studies with 203 participants were included. The impact of wheeled mobility skill interventions was assessed on mobility skills (n = 18), activity and participation (n = 10), and quality of life (n = 3). No studies reported effects on stress, fatigue, and motivational aspects. Interventions included power wheelchair skill training (n = 12), computer-based training (n = 5), smart wheelchair training (n = 2), and manual wheelchair training (n = 1), showing positive wheeled mobility intervention effects. Study quality based on the Methodological Index for Non-Randomized Studies scale was 9 out of 16 and 14 out of 24 for non-comparative and comparative studies respectively. Risk of bias was serious-to-critical on the Risk of Bias in Non-Randomized Studies of Interventions. INTERPRETATION: Wheeled mobility interventions showed promising beneficial effects on wheeled mobility, activity and participation, and quality of life for children and young people with CP. Future studies with structured and standardized training programmes and assessment tools are warranted to further accelerate the wheeled mobility skill acquisition process in this population. WHAT THIS PAPER ADDS: Wheeled mobility interventions improve wheeled mobility skills in individuals with cerebral palsy. The mobility skill acquisition process benefits from structured and standardized training. Knowledge of the intervention effects on participation and quality of life is limited. Most studies had moderate quality of evidence because of methodological limitations.
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Parálisis Cerebral , Calidad de Vida , Humanos , Niño , AdolescenteRESUMEN
OBJECTIVE: The purpose of this study was to investigate differences in static and dynamic motor fatigability during grip and pinch tasks between children with unilateral spastic cerebral palsy (USCP) and children with typical development (TD) and between preferred and nonpreferred hands. METHODS: Fifty-three children with USCP and 53 age-matched children with TD (mean = 11 years 1 month; SD = 3 years 8 months) participated in 30-second maximum exertion sustained and repeated grip and pinch tasks. For sustained tasks, the Static Fatigue Index and the ratio of mean force between the first and last thirds of the curve were calculated. For repeated tasks, the ratio of mean force and the ratio of numbers of peaks between the first and last thirds of the curve were calculated. RESULTS: Higher Static Fatigue Index scores for grip and pinch were found with USCP in both hands and between hands in both groups. Dynamic motor fatigability showed inconsistent results, with higher levels of fatigability in children with TD than in children with USCP for grip in the ratio of mean force between the first and last thirds of the curve in nonpreferred hands and in the ratio of number of peaks between the first and last thirds of the curve in preferred hands. CONCLUSION: Higher motor fatigability in children with USCP than in children with TD was found for static but not dynamic grip and pinch. Underlying mechanisms may play different roles in static and dynamic motor fatigability. IMPACT: These results highlight that static motor fatigability in grip and pinch tasks should be part of a comprehensive upper limb assessment and that this could be the target of individualized interventions.
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Parálisis Cerebral , Humanos , Niño , Fuerza de la Mano , Extremidad Superior , Mano , FatigaRESUMEN
Only cross-sectional studies have demonstrated muscle deficits in children with spastic cerebral palsy (SCP). The impact of gross motor functional limitations on altered muscle growth remains unclear. This prospective longitudinal study modelled morphological muscle growth in 87 children with SCP (age range 6 months to 11 years, Gross Motor Function Classification System [GMFCS] level I/II/III = 47/22/18). Ultrasound assessments were performed during 2-year follow-up and repeated for a minimal interval of 6 months. Three-dimensional freehand ultrasound was applied to assess medial gastrocnemius muscle volume (MV), mid-belly cross-sectional area (CSA) and muscle belly length (ML). Non-linear mixed models compared trajectories of (normalized) muscle growth between GMFCS-I and GMFCS-II&III. MV and CSA growth trajectories showed a piecewise model with two breakpoints, with the highest growth before 2 years and negative growth rates after 6-9 years. Before 2 years, children with GMFCS-II&III already showed lower growth rates compared to GMFCS-I. From 2 to 9 years, the growth rates did not differ between GMFCS levels. After 9 years, a more pronounced reduction in normalized CSA was observed in GMFCS-II&III. Different trajectories in ML growth were shown between the GMFCS level subgroups. These longitudinal trajectories highlight monitoring of SCP muscle pathology from early ages and related to motor mobility. Treatment planning and goals should stimulate muscle growth.