Degree of ventriculomegaly predicts school-aged functional outcomes in preterm infants with intraventricular hemorrhage.

BACKGROUND: Greater ventriculomegaly in preterm infants with intraventricular hemorrhage (IVH) has been associated with worse neurodevelopmental outcomes in infancy. We aim to explore the relationship between ventriculomegaly and school-age functional outcome. METHODS: Retrospective review of preterm infants with Grade III/IV IVH from 2006 to 2020. Frontal-occipital horn ratio (FOHR) was measured on imaging throughout hospitalization and last available follow-up scan. Pediatric Cerebral Performance Category (PCPC) scale was used to assess functional outcome at ≥4 years. Ordinal logistic regression was used to determine the relationship between functional outcome and FOHR at the time of Neurosurgery consult, neurosurgical intervention, and last follow-up scan while adjusting for confounders. RESULTS: One hundred and thirty-four infants had Grade III/IV IVH. FOHR at consult was 0.62 ± 0.12 and 0.75 ± 0.13 at first intervention (p < 0.001). On univariable analysis, maximum FOHR, FOHR at the last follow-up scan, and at Neurosurgery consult predicted worse functional outcome (p < 0.01). PVL, longer hospital admission, and gastrotomy/tracheostomy tube also predicted worse outcome (p < 0.05). PVL, maximum FOHR, and FOHR at consult remained significant on multivariable analysis (p < 0.05). Maximum FOHR of 0.61 is a fair predictor for moderate-severe impairment (AUC 75%, 95% CI: 62-87%). CONCLUSIONS: Greater ventricular dilatation and PVL were independently associated with worse functional outcome in Grade III/IV IVH regardless of neurosurgical intervention. IMPACT: Ventriculomegaly measured by frontal-occipital horn ratio (FOHR) and periventricular leukomalacia are independent correlates of school-age functional outcomes in preterm infants with intraventricular hemorrhage regardless of need for neurosurgical intervention. These findings extend the known association between ventriculomegaly and neurodevelopmental outcomes in infancy to functional outcomes at school age. FOHR is a fair predictor of school-age functional outcome, but there are likely other factors that influence functional status, which highlights the need for prospective studies to incorporate other clinical and demographic variables in predictive models.

Measuring the inch stones for progress: Gross motor function in the developmental and epileptic encephalopathies.

OBJECTIVE: Developmental and epileptic encephalopathies (DEE) entail moderate to profound impairments in gross motor skills and mobility, which are poorly quantified with clinical outcomes assessments (COA) used in neuro-typical populations. We studied the motor domain of the Adaptive Behavior Assessment System-3 for ages 0-5 years (ABAS) used outside of its intended age range with a focus on raw scores. METHODS: In a cross-sectional survey, 117 parents of children with a variety of DEEs (ages 1-35 years, median = 9) completed the motor domain section of the ABAS. Floor and ceiling effects and associations with epilepsy-related factors were assessed with appropriate parametric and nonparametric statistical techniques. The sensitivity of the ABAS and additional measures of mobility borrowed from the cerebral palsy literature (Functional Activities Questionnaire (FAQ-22) walking level (FAQ-WL)) to different levels of the Functional Mobility Scale was determined. RESULTS: ABAS motor scores corresponded to a median age equivalent of 20.5 months (Inter-Quartile Range (IQR) 8-34). Most raw scores corresponded to standardized scores > 2 standard deviations below the ABAS standardization sample mean. ABAS raw scores demonstrated minimal floor and ceiling effects (<5%). In linear regression models, scores increased with age under 6 years (p < 0.0001) but flattened out thereafter. Scores varied substantially by DEE group (p < 0.001) and decreased with higher convulsive seizure frequency (<0.0001) and number of seizure medications (p < 0.001). ABAS and other motor scores were sensitive to important differences in mobility as represented by the FMS at 5 yards. Further, they correlated with declines in mobility function from 5 to 500 yards. SIGNIFICANCE: An out-of-range COA with raw scores may provide a measure of motor ability and mobility sensitive within the range of moderate to profound impairment seen in patients with DEE. This approach could shorten the time to appropriate COA development and ensure timely clinical trial readiness for novel therapies for rare DEEs.

Supportive mobility device use across the life span by individuals with cerebral palsy: A qualitative study.

AIM: To understand the mobility experiences, supportive mobility device (SMD) use, and desired participation outcomes of individuals with cerebral palsy (CP) across the life span, and describe how perspectives of rehabilitation care and professional resources may influence mobility decision-making processes and outcomes. METHOD: In the second phase of an overarching study, focus groups were conducted with 164 participants (68 individuals with CP; 32 females, 36 males; mean age 17y 8mo, SD 11y 11mo, range 3-68y), 74 caregivers (50 females, 24 males), and 22 healthcare providers (14 females, eight males) across four US cities. Sessions were audio-recorded, transcribed, and analysed using constant comparison. RESULTS: Six themes emerged. Five presented across all stakeholder groups: (1) the system is broken; (2) equipment is simultaneously liberating and restricting; (3) adaptation across the life span; (4) designed for transport, not for living; and (5) sharing our stories and sharing resources. One theme (theme 6) was specific to healthcare providers: caught in the middle. INTERPRETATION: This qualitative study underscores the simultaneous value and frustration associated with SMDs as described by the community with CP, and recognition among all stakeholders of the need to improve connections and resource networks within the community with CP to improve SMD design and provision processes across device types and across the life span for individuals with CP. WHAT THIS PAPER ADDS: Supportive mobility devices (SMDs) were most often equated with freedom, participation, and independence. Frustration with SMDs across the life span persisted with regard to design, function, cost, and maintenance. Stakeholders in the community with cerebral palsy are seeking greater networking and resource sharing to enhance SMD provision processes. Access to appropriate SMDs across the life span and the need for system improvement are critical.

OBJETIVO: Compreender as experiências de mobilidade, o uso de dispositivos de mobilidade de apoio e os resultados de participação desejados de pessoas com paralisia cerebral (PC) ao longo da vida e descrever como as perspectivas de cuidados de reabilitação e recursos profissionais podem influenciar os processos de tomada de decisão de mobilidade e resultados. MÉTODO: Na segunda fase de um estudo abrangente, foram realizados grupos focais com 164 participantes (68 pessoas com PC; 32 mulheres, 36 homens; idade média de 17 anos e 8 meses, DP 11 anos e 11 meses, faixa de 3 a 68 anos), 74 cuidadores (50 mulheres, 24 homens) e 22 profissionais de saúde (14 mulheres, oito homens) em quatro cidades dos EUA. As sessões foram gravadas em áudio, transcritas e analisadas por meio de comparação constante. RESULTADOS: Emergiram seis temas. Cinco apresentados em todos os grupos de partes interessadas: (1) o sistema está quebrado; (2) o equipamento está simultaneamente liberando e restringindo; (3) adaptação ao longo da vida; (4) projetado para transporte, não para a vida; e (5) compartilhar nossas histórias e recursos. Um tema (tema 6) era específico para os profissionais de saúde: "pego no meio". INTERPRETAÇÃO: Este estudo qualitativo ressalta o valor e a frustração simultâneos associados aos dispositivos de mobilidade de apoio, conforme descrito pela comunidade com CP, e o reconhecimento entre todas as partes interessadas da necessidade de melhorar as conexões e redes de recursos dentro da comunidade com CP para melhorar os processos de projeto e fornecimento de SMD em todos os dispositivos tipos e ao longo da vida para pessoas com PC.

Association Between Daily Physical Activity and Clinical Anthropomorphic Measures in Adults With Cerebral Palsy.

OBJECTIVE: To describe the relationship between activity level and cardiovascular risk measures as well as describe general activity patterns of adults with cerebral palsy. DESIGN: Cross-sectional. SETTING: Academic outpatient rehabilitation clinic. PARTICIPANTS: Adults with cerebral palsy (N=47). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Gross Motor Functional Classification System (GMFCS) level was determined by validated self-report questionnaire. Activity (daily step count, walk time, sitting time, standing time, and transitional movements) over 6 days recorded using an activPAL. Weight, body mass index (BMI), and waist-to-hip ratio were measured. Bivariate relationships between anthropomorphic and activity measures were assessed. RESULTS: Thirty-eight participants completed all measurements. Nine were excluded because of incomplete activPAL data. The median age was 28.50 years (interquartile range [IQR]=24.25-47.00), range 18-77 years. Participants' GMFCS levels were I: 13%; II: 16%; III: 21%; IV: 34%; and V: 16%. Median steps/day for GMFCS I/II participants were 5258.3 (IQR=3606.8-6634.7), and median steps/day were 1681.3 (IQR=657.2-2751.8) and 30.0 (IQR=6.8-54.2) for GMFCS level III and IV/V participants, respectively. Significantly greater steps/day were found for GMFCS I/II or III participants compared to those GMFCS IV/V (P<.001 and P=.0074, respectively). In addition, 60.5% of the subjects had a BMI in the normal range, 10.5% were obese, 23.6% were overweight, and 5.3% were underweight. For subjects with GMFCS I/II, the Spearman's rank correlation coefficient for time standing and waist circumference was -0.73 (0.01). GMFCS III and GMFCS IV/V participants had respective correlations of -0.16 (0.71) and -0.01 (0.98). For subjects with GMFCS I/II, the Spearman's rank correlation coefficient for standing time and BMI was -0.55 (P=.08). For the GMFCS III and GMFCS IV/V groups the respective correlations were -0.19 (0.67) and 0.00 (1.00). CONCLUSIONS: Subjects with GMFCS level I or II who engaged in more activity tended to have more favorable anthropometric profiles. Subjects with GMFCS level III, IV, or V did not have a similar trend. Our findings suggest factors beyond activity patterns affect anthropometrics to a greater degree in those with higher GMFCS levels.

An autosomal dominant neurological disorder caused by de novo variants in FAR1 resulting in uncontrolled synthesis of ether lipids.

PURPOSE: In this study we investigate the disease etiology in 12 patients with de novo variants in FAR1 all resulting in an amino acid change at position 480 (p.Arg480Cys/His/Leu). METHODS: Following next-generation sequencing and clinical phenotyping, functional characterization was performed in patients' fibroblasts using FAR1 enzyme analysis, FAR1 immunoblotting/immunofluorescence, and lipidomics. RESULTS: All patients had spastic paraparesis and bilateral congenital/juvenile cataracts, in most combined with speech and gross motor developmental delay and truncal hypotonia. FAR1 deficiency caused by biallelic variants results in defective ether lipid synthesis and plasmalogen deficiency. In contrast, patients' fibroblasts with the de novo FAR1 variants showed elevated plasmalogen levels. Further functional studies in fibroblasts showed that these variants cause a disruption of the plasmalogen-dependent feedback regulation of FAR1 protein levels leading to uncontrolled ether lipid production. CONCLUSION: Heterozygous de novo variants affecting the Arg480 residue of FAR1 lead to an autosomal dominant disorder with a different disease mechanism than that of recessive FAR1 deficiency and a diametrically opposed biochemical phenotype. Our findings show that for patients with spastic paraparesis and bilateral cataracts, FAR1 should be considered as a candidate gene and added to gene panels for hereditary spastic paraplegia, cerebral palsy, and juvenile cataracts.

Dysautonomia and functional impairment in rare developmental and epileptic encephalopathies: the other nervous system.

AIM: To determine whether functional impairments and autonomic symptoms are correlated in young people with developmental and epileptic encephalopathies (DEEs). METHOD: Cross-sectional, online surveys (2018-2020) of parents recruited from family groups obtained information on several aspects of children's conditions including functional abilities (mobility, hand use, eating, and communication), 18 autonomic symptoms in six groups (cardiac, respiratory, sweating, temperature, gastrointestinal, and other), and parental stress. Bivariate and multivariable logistic regression analyses examined associations of dysautonomias with functional impairment, adjusted for type of DEE and age. RESULTS: Of 313 participants with full information on function and dysautonomias, 156 (50%) were females. The median age was 8 years (interquartile range 4-12y); 255 (81%) participants had symptoms in at least one autonomic symptom group; 283 (90%) had impairment in at least one functional domain. The number of functional impairment domains and of autonomic symptom groups varied significantly across DEE groups (both p<0.001). The number of functional impairment domains and of autonomic symptom groups were correlated (Spearman's r=0.35, p<0.001) on bivariate and multivariable analysis adjusted for DEE group and age. Parental stress was also independently correlated with dysautonomias (p<0.001). INTERPRETATION: Parent-reported dysautonomias are common in children with DEEs. They correlate with extent of functional impairment and may contribute to caregiver stress. What this paper adds Dysautonomic symptoms are common in young people with developmental and epileptic encephalopathies (DEEs). Burden of dysautonomias is strongly correlated with burden of functional impairments. Aspects of dysautonomic function may provide biomarkers of DEE disease severity.

Diet quality in adults with cerebral palsy: a modifiable risk factor for cardiovascular disease prevention.

AIM: To assess diet quality and its relationship with cardiovascular health measures for adults with cerebral palsy (CP). METHOD: A convenience sample of 45 adults with CP (26 females, 19 males; mean age 35y 10mo [SD 14y 9mo]). were recruited for this cross-sectional study. Demographic, medical, and Gross Motor Function Classification System (GMFCS) information were obtained through in-person visits. Participants completed two 24-hour dietary recalls using the Automated Self-Administered 24-hour Dietary Assessment Tool. Specific macronutrient intake was compared to 2015 to 2020 US Department of Agriculture (USDA) guidelines. Other data included body mass index (BMI), waist-to-hip ratio (WHR), blood pressure, and hemoglobin A1c (HgA1c;n=43). RESULTS: Adults across GMFCS levels I to V were enrolled, 20 participants were in GMFCS levels IV or V. Mean calorie intake was 1777.91/day (SD 610.54), while sodium intake was 3261.75mg/day (SD 1484.92). Five participants met USDA vegetable and seven fruit guidelines. None met whole grain targets. Sixteen were overweight/obese by BMI. Sixteen participants without hypertension diagnoses had elevated blood pressure and nine had abnormal HgA1c without prediabetes/diabetes history. Percent calories from saturated fat was inversely associated with WHR in unadjusted and adjusted models (p=0.002 and p=0.003 respectively); all other dietary recommendations assessed (total calories, sodium, and sugar) were non-significant. Post hoc analyses were unchanged using 2020 to 2025 USDA guidelines. INTERPRETATION: Assessment of nutrient intake and diet quality is feasible and warrants further study in adults with CP, as USDA guidelines are largely unmet. What this paper adds Adults with cerebral palsy (CP) do not meet US Department of Agriculture dietary quality recommendations. Prediabetes and hypertension may be common, but unrecognized, in adults with CP. Screening for nutrient intake and diet quality should be performed to facilitate nutritional counseling.

Nonseizure consequences of Dravet syndrome, KCNQ2-DEE, KCNB1-DEE, Lennox-Gastaut syndrome, ESES: A functional framework.

RATIONALE: Developmental epilepsies and encephalopathies (DEEs) are characterized by many severe developmental impairments, which are not well-described. A functional framework could facilitate understanding of their nature and severity and guide the selection instruments to measure improvements in therapeutic trials. METHODS: An online survey administered through several parent-organized foundations utilized accepted functional classifications and questionnaires derived from common instruments to determine levels of mobility, fine motor, communication, and feeding functions. Statistical analyses focused on overall levels of function and across-group comparisons adjusted for age. RESULTS: From 6/2018 to 2/2020, 252 parents provided information for one or more functional domains. Median age was 7.2 years (interquartile range (IQR): 3.9 to 11.8), and 128 (51%) were females. DEE groups were Dravet syndrome (N = 72), KCNQ2-DEE (N = 80), KCNB1-DEE, (N = 33), Lennox-Gastaut syndrome (LGS; N = 26), electrographic status epilepticus in sleep (ESES; N = 15), and others (N = 26). Overall, functional hand grasp was absent in 48 (20%). Of children ≥2 years old, 60/214 (28%) could not walk independently, 85 (40%) were dependent on someone else for feeding, and 153 (73%) did not effectively communicate with unfamiliar people. Impairments entailing absence or near absence of independent function (profound impairment) were observed in 0, 1, 2, 3, and 4 domains for 58 (25%), 78 (34%), 40 (17%), 33 (14%), and 22 (10%) children, respectively. After adjustment for age, impairment levels varied substantially across DEE group for mobility (p < 0.0001), feeding (p < 0.0001), communication (p < 0.0001), hand grasp (p < 0.0001), and number of profoundly impaired domains (p < 0.0001). Three or four profoundly affected domains were reported in 44% of KCNQ2-DEE participants, followed by LGS (29%), KCNB1-DEE (27%), ESES (7%), and Dravet syndrome (6%). CONCLUSIONS: Many children with DEEs experience severe functional impairments, and few children have typical function. As precision therapies will emphasize nonseizures consequences of DEEs, understanding the nature of abilities and impairments will be critical to selecting appropriate outcome measures in therapeutic trials.

Spasticity and pain in adults with cerebral palsy.

AIM: To evaluate whether spasticity measures are related to pain in adults with cerebral palsy (CP). METHOD: This cross-sectional study recruited individuals aged 16 to 89 years with a diagnosis of CP. Participants completed the Penn Spasm Frequency Scale (PSFS), Brief Pain Inventory (BPI), and PROMIS Pain Interference measures. The Modified Ashworth Scale (MAS) and Tardieu spasticity angles of six joints were rated and summed to composite MAS and Tardieu scores for each participant. Associations between spasticity and pain measures were evaluated. RESULTS: Forty-seven participants (27 females, 20 males) with a mean age of 35 years 7 months (range 18-77y) spanning all Gross Motor Function Classification System (GMFCS) levels were included. Twenty-six participants reported their average pain level on BPI as greater than 0 over the past week (median pain level 4.0). Median PSFS was 1.0 (range 0.0-1.0) and this correlated with average BPI and Pain Interference T scores (median 40.7; ρ=0.33 and ρ=0.31 respectively [both p=0.01]). When adjusted for pain medication use and age, MAS correlated with BPI (ρ=0.30; p=0.04). Other pain and spasticity measures, or GMFCS level, were not significantly related with pain interference or BPI rating. Age was weakly associated with BPI (slope=0.10; p<0.01). INTERPRETATION: PROMIS Pain Interference was lower than population-based norms. Patient-rated spasm frequency demonstrated better association with pain levels and interference than physician-rated MAS and Tardieu. WHAT THIS PAPER ADDS: Pain was not associated with Gross Motor Function Classification System level. Pain increased with age, as anticipated. Self-reported spasm scores were associated with increased pain in contrast to clinical examination scales. Adjusted, summed spasticity on the Modified Ashworth Scale was associated with pain scores on the Brief Pain Inventory. Although pain is experienced by adults with cerebral palsy, pain did not interfere with activities.

Neural and non-neural contributions to ankle spasticity in children with cerebral palsy.

AIM: To assess the neural and non-neural contributions to spasticity in the impaired ankle of children with cerebral palsy (CP). METHOD: Instrumented tapping of the Achilles tendon was done isometrically to minimize non-neural contributions and elicit neural contributions. Robot-controlled ankle stretching was done at various velocities, including slow stretching, with minimized neural contributions. Spasticity was assessed as having neural (phasic and tonic stretch reflex torque, tendon reflex gain, contraction rate, and half relaxation rate) and non-neural origin (elastic stiffness and viscous damping) in 17 children with CP (six females and 11 males; mean age [SD] 10y 8mo [3y 11mo], range 4y-18y) and 17 typically developing children (six females and 11 males; mean age [SD] 12y 7mo [2y 9mo], range 7y-18y). All torques were normalized to weight×height. RESULTS: Children with CP showed increased phasic and tonic stretch reflex torque (p=0.004 and p=0.001 respectively), tendon reflex gain (p=0.02), contraction rate (p=0.038), half relaxation rate (p=0.02), elastic stiffness (p=0.01), and viscous damping (p=0.01) compared to typically developing children. INTERPRETATION: Controlled stretching and instrumented tendon tapping allow the systematic quantification of various neural and non-neural changes in CP, which can be used to guide impairment-specific treatment. WHAT THIS PAPER ADDS: Ankle spasticity is associated with increased phasic and tonic stretch reflexes, tendon reflex gain, and contraction and half relaxation rates. Ankle spasticity is also associated with increased elastic stiffness and viscous damping.

Contribuciones neuronales y no neuronales a la espasticidad del tobillo en niños con parálisis cerebral OBJETIVO: Evaluar las contribuciones neurales y no neurales a la espasticidad en el tobillo comprometido de niños con parálisis cerebral (PC). MÉTODO: La percusión instrumentada en el tendón de Aquiles se realizó de forma isométrica para minimizar las contribuciones no neurales y un tirón del tendón exagerado, para obtener contribuciones neurales. El estiramiento del tobillo controlado por robot se realizó a varias velocidades, incluido el estiramiento lento, con contribuciones neurales minimizadas. Se evaluó la espasticidad como neural (torque reflejo de estiramiento fásico y tónico, ganancia del reflejo tendinoso, tasa de contracción y media tasa de relajación) y origen no neural (rigidez elástica y amortiguación viscosa) en 17 niños con PC (seis mujeres y 11 varones; edad media [DE] 10a 8m [3a 11m], rango 4a-18a) y 17 niños con desarrollo típico (seis mujeres y 11 hombres; edad media [SD] 12a 7m [2a 9m], rango 7a-18a). Todos los pares de torsion se normalizaron al peso × altura. RESULTADOS: Los niños con PC mostraron un aumento del torque reflejo de estiramiento fásico y tónico (p = 0,004 y p = 0,001 respectivamente), ganancia refleja del tendón (p = 0,02), tasa de contracción (p = 0,038), tasa de relajación media (p = 0,02), rigidez elastica (p = 0,01) y amortiguación viscosa (p = 0,01) en comparación con los niños con desarrollo normal. INTERPRETACIÓN: El estiramiento controlado y la percusión instrumentada del tendón, permiten la cuantificación sistemática de varios cambios neuronales y no neuronales en la PC, que pueden usarse para guiar el tratamiento específico de la discapacidad.

Contribuições neurais e não neurais para a espasticidade do tornozelo em crianças com paralisia cerebral OBJETIVO: Avaliar as contribuições neurais e não-neurais para a espasticidade no tornozelo comprometido de crianças com paralisia cerebral (PC). MÉTODO: O golpeamento instrumentalizado do tendão de Aquiles foi realizado isometricamente para minimizar as contribuições não-neurais e um desvio exagerado do tendão, e assim eliciar as contribuições neurais. O alongamento do tornozelo controlado por um robô foi realizado em várias velocidades, incluindo alongamento lento, com contribuições neurais limitadas. A espasticidade foi avaliada como tendo origem neural (torque do reflexo fásico e tônico, ganho do reflexo tendinoso, taxa de contração, e taxa de meio relaxamento) e não-neural (rigidez elástica e amortecimento viscoso) em 17 crianças com PC (seis do sexo feminino e 11 do sexo masculino; média de idade [DP] 10a 8m [3 11m], variação 4a-18a) e 17 crianças com desenvolvimento típico (seis do sexo feminino e 11 do sexo masculino; média de idade [DP] 12a 7m [2a 9m], variação 7a-18a). Todos os torques foram normalizados para peso x altura. RESULTADOS: Crianças com PC mostraram aumento do torque do reflexo tônico e fásico e (p=0,004 e p=0,001 respectivamente), ganho do reflexo tendinoso (p=0,02), taxa de contração (p=0,038), taxa de meio relaxamento (p=0,02), rigidez elástica (p=0,01), e amortecimento viscoso (p=0,01) em comparação com as crianças com desenvolvimento típico. INTERPRETAÇÃO: O alongamento controlado e o golpeamento instrumentalizado do tendão permitem quantificação sistemática de várias mudanças neurais e não-neurais em PC, as quais podem ser usadas para guiar tratamento específico para a deficiência observada.

Understanding children with cerebral palsy and bullying: A mixed methods approach.

AIM: The study aims to explore the views of children with cerebral palsy (CP) regarding their experiences being bullied and their perceptions of their primary bully. METHOD: Forty-three children aged 10-18 with CP were given the California Bullying Victimization Scale. Fourteen of the children shared a bullying experience orally or written after the survey. Using a mixed methods analysis, the survey answers were tabulated with descriptive statistics and analysed by Gross Motor Function Classification System (GMFCS) level. The comments were coded to determine qualitative responses. RESULTS: Most bullied children viewed the bully as less than or at least equal to them regarding popularity (73%), performance in schoolwork (85%), and physical strength (56%). Bullied children preferentially told an adult at home. Code words and common categories of bullying were determined from their experiences. INTERPRETATION: Children with CP experience bullying but do not view themselves as inferior to their bully in popularity, intelligence, or physical strength. Most bullied participants confided in an adult at home. Children with GMFCS Levels 1, 2, and 3 seem to be at greater risk for bullying than children with GMFCS Levels 4 and 5. Children with CP demonstrate individualized strategies for resilience with reliance on adults for resources.

Dance and rehabilitation in cerebral palsy: a systematic search and review.

AIM: To conduct a review of research literature on the use of dance and movement with music (rhythmic auditory stimulation [RAS]) in the neurorehabilitation of children and adults with cerebral palsy (CP). METHOD: We conducted a systematic search and quality appraisal of the research literature on dance and RAS in CP. Additionally, we linked the research outcomes to the International Classification of Functioning, Disability and Health (ICF) framework. RESULTS: Studies showed preliminary evidence of the benefits of dance and RAS on body functions, particularly balance, gait, walking, and cardiorespiratory fitness for individuals with CP. Research gaps are evident across all domains of the ICF, particularly in the participation and environment domains. INTERPRETATION: To facilitate translation of quantitative research outcomes to the clinical classification of the ICF, a table was constructed that links traditional areas of quantitative rehabilitation research with the ICF categories highlighting areas of research strengths and areas where increased rigor is desirable. The potential for dance and RAS to have positive impacts on body functions, emotional expression, social participation, and attitudinal change are indicated areas for consideration in future research. WHAT THIS PAPER ADDS: The potential for dance and movement to music help balance, gait, and walking in children and adults with cerebral palsy. Research gaps are evident across International Classification of Functioning, Disability and Health domains, particularly participation and environment domains.

LA DANZA Y LA REHABILITACIÓN EN LA PARÁLISIS CEREBRAL: UNA BÚSQUEDA SISTEMÁTICA Y REVISIÓN: OBJETIVO: Realizar una revisión de la literatura de investigación sobre el uso de la danza y el movimiento con la música (estimulación auditiva rítmica [RAS]) en la neurorrehabilitación de niños y adultos con parálisis cerebral (PC). MÉTODO: Llevamos a cabo una búsqueda sistemática y una evaluación de calidad de la literatura de investigación sobre danza y RAS en PC. Además, vinculamos los resultados de la investigación al marco teórico de la Clasificación Internacional del Funcionamiento, de la Discapacidad y de la Salud (CIF), RESULTADOS: Los estudios mostraron evidencia preliminar de los beneficios de la danza y el RAS en las funciones corporales, particularmente el equilibrio, la marcha, la marcha y la capacidad cardiorrespiratoria para las personas con PC. Las brechas de investigación son evidentes en todos los dominios de la CIF, particularmente en los ámbitos de participación y medio ambiente. INTERPRETACIÓN: Para facilitar la traducción de los resultados de la investigación cuantitativa a la clasificación clínica de la CIF, se construyó una tabla que vincula las áreas tradicionales de investigación de rehabilitación cuantitativa con las categorías CIF. destacando áreas de fortalezas de investigación y áreas donde es deseable un mayor rigor. El potencial de la danza y el RAS para tener un impacto positivo en las funciones corporales, la expresión emocional, la participación social y el cambio de actitud son áreas identificadas para tener en consideración en investigaciones futuras.

DANÇA E REABILITAÇÃO EM PARALISIA CEREBRAL: UMA PESQUISA E REVISÃO SISTEMÁTICA: OBJETIVO: Conduzir uma revisão da literaturs sobre o uso da dança e movimento com música (estimulação auditiva rítmica [EAR]) na neuroreabilitação de crianças e adolescentes com paralisia cerebral (PC). MÉTODO: Conduzimos uma busca sistemática e avaliação de qualidade da literatura sobre dança e EAR em PC. Adicionalmente, relacionamos os resultados da pesquisa com a estrutura da Classificação Internacional de Funcionalidade, Incapacidade e Saúde (CIF). RESULTADOS: Os estudos mostraram evidência preliminar dos benefícios e EAR nas funções do corpo, particularmente equilíbrio, marcha, caminhar e aptidão cárdio-respiratória em indivíduos com PC. Lacunas nas pesquisas são evidentes em todos os domínios da Classificação Internacional de Funcionalidade, Incapacidade e Saúde , particularmente nos domínios de participação e ambiente. INTERPRETAÇÃO: Para facilitar a transição de resultados de pesquisas quantitativas para a classificação clínica da CIF, uma tabela foi elaborada para relacionar áreas tradicionais de pesquisas quantitativas em reabilitação com as categorias da CIF. Foram realçadas áreas de em que pesquisas estão fortes e áreas onde maior rigor é necessário. O potencial da dança e EAR para proporcionar impactos positivos nas funções do corpo, expressão emocional, participação social, e mudança atitudinal são indicados como áreas para consideração em futuras pesquisas.

The validity and reliability of the Test of Arm Selective Control for children with cerebral palsy: a prospective cross-sectional study.

AIM: This study examined the reliability and validity of the Test of Arm Selective Control (TASC) to examine upper extremity selective voluntary motor control in children and adolescents with all types of spastic cerebral palsy (CP). METHOD: Fifty-six participants with CP, ranging in age from 5 years 9 months to 18 years 11 months (average 11y 7mo, SD 3y 9mo; 25 males, 31 females), participated in this prospective cross-sectional study. They were evaluated using the TASC and several clinical measures. RESULTS: TASC and Manual Ability Classification System (r=-0.529, p<0.001), TASC and ABILHAND-Kids (r=0.596, p<0.001), and TASC and affected extremities (r=-0.486, p=0.001) were moderately correlated. There was a weak correlation between the TASC and Gross Motor Function Classification System (r=-0.363, p=0.006) and no correlation between the TASC and age (p=0.366) or rater (p=0.713). Interrater reliability for upper extremity total score (intraclass correlation coefficient [ICC]=0.92-0.94) and upper extremity limb scores (ICC=0.92-0.96) was high for two independent rater groups (p≤0.001). Average time to administer was 16 minutes, 18 seconds. INTERPRETATION: The TASC is a reliable and valid tool for objective assessment of selective voluntary motor control. Clinically this measure may guide the selection of medical, surgical, or therapy interventions and may improve outcome prognosis. WHAT THIS PAPER ADDS: The Test of Arm Selective Control (TASC) demonstrates a high degree of reliability and multiple aspects of validity when assessing upper extremity selective control in those with cerebral palsy. The TASC is an upper limb companion to the Selective Control Assessment of the Lower Extremity.

Advanced Robotic Therapy Integrated Centers (ARTIC): an international collaboration facilitating the application of rehabilitation technologies.

BACKGROUND: The application of rehabilitation robots has grown during the last decade. While meta-analyses have shown beneficial effects of robotic interventions for some patient groups, the evidence is less in others. We established the Advanced Robotic Therapy Integrated Centers (ARTIC) network with the goal of advancing the science and clinical practice of rehabilitation robotics. The investigators hope to exploit variations in practice to learn about current clinical application and outcomes. The aim of this paper is to introduce the ARTIC network to the clinical and research community, present the initial data set and its characteristics and compare the outcome data collected so far with data from prior studies. METHODS: ARTIC is a pragmatic observational study of clinical care. The database includes patients with various neurological and gait deficits who used the driven gait orthosis Lokomat® as part of their treatment. Patient characteristics, diagnosis-specific information, and indicators of impairment severity are collected. Core clinical assessments include the 10-Meter Walk Test and the Goal Attainment Scaling. Data from each Lokomat® training session are automatically collected. RESULTS: At time of analysis, the database contained data collected from 595 patients (cerebral palsy: n = 208; stroke: n = 129; spinal cord injury: n = 93; traumatic brain injury: n = 39; and various other diagnoses: n = 126). At onset, average walking speeds were slow. The training intensity increased from the first to the final therapy session and most patients achieved their goals. CONCLUSIONS: The characteristics of the patients matched epidemiological data for the target populations. When patient characteristics differed from epidemiological data, this was mainly due to the selection criteria used to assess eligibility for Lokomat® training. While patients included in randomized controlled interventional trials have to fulfill many inclusion and exclusion criteria, the only selection criteria applying to patients in the ARTIC database are those required for use of the Lokomat®. We suggest that the ARTIC network offers an opportunity to investigate the clinical application and effectiveness of rehabilitation technologies for various diagnoses. Due to the standardization of assessments and the use of a common technology, this network could serve as a basis for researchers interested in specific interventional studies expanding beyond the Lokomat®.

The development of Bayesian integration in sensorimotor estimation.

Examining development is important in addressing questions about whether Bayesian principles are hard coded in the brain. If the brain is inherently Bayesian, then behavior should show the signatures of Bayesian computation from an early stage in life. Children should integrate probabilistic information from prior and likelihood distributions to reach decisions and should be as statistically efficient as adults, when individual reliabilities are taken into account. To test this idea, we examined the integration of prior and likelihood information in a simple position-estimation task comparing children ages 6-11 years and adults. Some combination of prior and likelihood was present in the youngest sample tested (6-8 years old), and in most participants a Bayesian model fit the data better than simple baseline models. However, younger subjects tended to have parameters further from the optimal values, and all groups showed considerable biases. Our findings support some level of Bayesian integration in all age groups, with evidence that children use probabilistic quantities less efficiently than adults do during sensorimotor estimation.

Robotic Resistance Treadmill Training Improves Locomotor Function in Children With Cerebral Palsy: A Randomized Controlled Pilot Study.

OBJECTIVE: To determine whether applying controlled resistance forces to the legs during the swing phase of gait may improve the efficacy of treadmill training as compared with applying controlled assistance forces in children with cerebral palsy (CP). DESIGN: Randomized controlled study. SETTING: Research unit of a rehabilitation hospital. PARTICIPANTS: Children with spastic CP (N=23; mean age, 10.6y; range, 6-14y; Gross Motor Function Classification System levels, I-IV). INTERVENTIONS: Participants were randomly assigned to receive controlled assistance (n=11) or resistance (n=12) loads applied to the legs at the ankle. Participants underwent robotic treadmill training 3 times a week for 6 weeks (18 sessions). A controlled swing assistance/resistance load was applied to both legs starting from the toe-off to mid-swing phase of gait during training. MAIN OUTCOME MEASURES: Outcome measures consisted of overground walking speed, 6-minute walk distance, and Gross Motor Function Measure scores and were assessed pre and post 6 weeks of training and 8 weeks after the end of training. RESULTS: After 6 weeks of treadmill training in participants from the resistance training group, fast walking speed and 6-minute walk distance significantly improved (18% and 30% increases, respectively), and 6-minute walk distance was still significantly greater than that at baseline (35% increase) 8 weeks after the end of training. In contrast, overground gait speed and 6-minute walk distance had no significant changes after robotic assistance training. CONCLUSIONS: The results of the present study indicated that robotic resistance treadmill training is more effective than assistance training in improving locomotor function in children with CP.

Home-Based Versus Laboratory-Based Robotic Ankle Training for Children With Cerebral Palsy: A Pilot Randomized Comparative Trial.

OBJECTIVE: To examine the outcomes of home-based robot-guided therapy and compare it to laboratory-based robot-guided therapy for the treatment of impaired ankles in children with cerebral palsy. DESIGN: A randomized comparative trial design comparing a home-based training group and a laboratory-based training group. SETTING: Home versus laboratory within a research hospital. PARTICIPANTS: Children (N=41) with cerebral palsy who were at Gross Motor Function Classification System level I, II, or III were randomly assigned to 2 groups. Children in home-based and laboratory-based groups were 8.7±2.8 (n=23) and 10.7±6.0 (n=18) years old, respectively. INTERVENTIONS: Six-week combined passive stretching and active movement intervention of impaired ankle in a laboratory or home environment using a portable rehabilitation robot. MAIN OUTCOME MEASURES: Active dorsiflexion range of motion (as the primary outcome), mobility (6-minute walk test and timed Up and Go test), balance (Pediatric Balance Scale), Selective Motor Control Assessment of the Lower Extremity, Modified Ashworth Scale (MAS) for spasticity, passive range of motion (PROM), strength, and joint stiffness. RESULTS: Significant improvements were found for the home-based group in all biomechanical outcome measures except for PROM and all clinical outcome measures except the MAS. The laboratory-based group also showed significant improvements in all the biomechanical outcome measures and all clinical outcome measures except the MAS. There were no significant differences in the outcome measures between the 2 groups. CONCLUSIONS: These findings suggest that the translation of repetitive, goal-directed, biofeedback training through motivating games from the laboratory to the home environment is feasible. The benefits of home-based robot-guided therapy were similar to those of laboratory-based robot-guided therapy.

Kinematic and EMG Responses to Pelvis and Leg Assistance Force during Treadmill Walking in Children with Cerebral Palsy.

Treadmill training has been used for improving locomotor function in children with cerebral palsy (CP), but the functional gains are relatively small, suggesting a need to improve current paradigms. The understanding of the kinematic and EMG responses to forces applied to the body of subjects during treadmill walking is crucial for improving current paradigms. The objective of this study was to determine the kinematics and EMG responses to the pelvis and/or leg assistance force. Ten children with spastic CP were recruited to participate in this study. A controlled assistance force was applied to the pelvis and/or legs during stance and swing phase of gait through a custom designed robotic system during walking. Muscle activities and spatial-temporal gait parameters were measured at different loading conditions during walking. In addition, the spatial-temporal gait parameters during overground walking before and after treadmill training were also collected. Applying pelvis assistance improved step height and applying leg assistance improved step length during walking, but applying leg assistance also reduced muscle activation of ankle flexor during the swing phase of gait. In addition, step length and self-selected walking speed significantly improved after one session of treadmill training with combined pelvis and leg assistance.

Cross-Sectional Study of Bowel Symptoms in Adults With Cerebral Palsy: Prevalence and Impact on Quality of Life.

OBJECTIVES: To determine the prevalence and type of bowel symptoms, and their impact on health-related quality of life (HRQOL) in adults with cerebral palsy (CP). DESIGN: Prospective cross-sectional study. SETTING: Urban, outpatient rehabilitation facility. PARTICIPANTS: Adults with CP (N=91; 46 men, 45 women; mean age, 36y; age range, 18-79y). INTERVENTION: Not applicable. MAIN OUTCOME MEASURES: Participants were interviewed using standardized instruments to assess the frequency and types of bowel dysfunction. The International Consultation of Incontinence Questionnaire-Bowel was used to assess bowel incontinence and impact on quality of life, and constipation presence was determined using the Rome III criteria for constipation. Constipation symptoms were rated by the Patient Assessment of Constipation-Symptom Scale. Participants' mobility status was classified using the Gross Motor Function Classification System (GMFCS). Interactions between mobility measures, anthropometric measures, and bowel symptoms were assessed. RESULTS: Of the 91 participants enrolled, 62.6% were GMFCS IV or V. Twenty-eight participants (30.8%) reported severe difficulty with control of liquid stool (rating never or rarely); these participants were more likely to have a greater GMFCS level (P=.0004). Twenty-six participants (28.6%) reported that bowel function caused embarrassment some/most/all of the time. Fifty-nine participants (64.8%) met criteria for chronic constipation, which did not differ by GMFCS levels. Overall, 57.1% of participants reported that bowel symptoms interfered with life; 40.7% reported moderate to severe interference. CONCLUSIONS: Bowel symptoms were frequent, a source of embarrassment, and impacted HRQOL in these adults with CP. Addressing bowel-related symptoms has the potential to improve HRQOL in these adults.