Factors Related to Quality of Life in Children With Cerebral Palsy.

BACKGROUND: We investigated the influence of relevant demographic, clinical, neuropsychological, and psychosocial variables on the proxy-reported quality of life (QOL) of children with cerebral palsy (CP). METHODS: The proxy-reported Cerebral Palsy Quality of Life-Child questionnaire (CP QOL-Child) was completed by 58 children with CP (mean age 10.22 years, SD 1.67). Relationships between QOL scores and demographic, clinical, neuropsychological, and psychosocial variables were assessed. CP QOL scores and other variables that correlated significantly were introduced into a multiple linear regression model. RESULTS: Executive functioning and motor functional status were explanatory variables for the CP QOL total score. Executive functions explained three specific QOL domains: Social Wellbeing and Acceptance, Feelings about Functioning, and Emotional Wellbeing and Self-esteem. Parental stress also explained Social Wellbeing and Acceptance. Motor functional status and visual perception were explanatory variables for the Access to Services domain. Finally, autism spectrum disorder (ASD) traits were an explanatory variable for the Participation and Physical Health domain. CONCLUSION: Executive functioning and motor functional status importantly influence QOL of children with CP. Visual perception, ASD symptoms, and parental stress variables are related with specific QOL domains. These findings demonstrate that interventions targeting cognitive functions in children with CP may positively influence QOL.

Study protocol of a randomized controlled trial of home-based computerized executive function training for children with cerebral palsy.

BACKGROUND: Cerebral palsy (CP) is frequently associated with specific cognitive impairments, such as executive dysfunction which are related to participation and quality of life (QOL). The proposed study will examine whether a computerized executive function (EF) training programme could provide superior benefits for executive functioning, participation, QOL and brain plasticity, as compared to usual care. METHODS: A single-blind randomized controlled trial (RCT) design will be performed. Thirty children with CP aged 8 to 12 years will participate in a home-based computerized multi-modal executive training programme (12 weeks, 5 days a week, 30 min a day training, total dose = 30 h). Thirty children with CP matched by age, sex, motor and intelligence quotient (IQ) will compose the waitlist group. Cognitive, behavioural, emotional, participation and QOL measures will be obtained at three time points: before, immediately after and 9 months after completing the training. Additionally, structural and functional (resting state) magnetic resonance images (MRI) will be obtained in a subsample of 15 children from each group. Outcomes between groups will be compared following standard principles for RCTs. DISCUSSION: The study will test whether the cognitive training programme exerts a positive effect not only on neuropsychological and daily functioning of children with CP but also on other measures such as participation and QOL. We will also use brain MRI to test brain functional and structural changes after the intervention. If this on-line and home-based training programme proves effective, it could be a cost-effective intervention with short- and long-term effects on EF, participation or QOL in CP. TRIAL REGISTRATION: ClinicalTrials.gov: NCT04025749. Registered 19 July 2019. Retrospectively registered.

Executive function and general intellectual functioning in dyskinetic cerebral palsy: Comparison with spastic cerebral palsy and typically developing controls.

AIM: To comprehensively describe intellectual and executive functioning (EF) in people with dyskinetic cerebral palsy (DCP), by comparing their performance with that of: 1) age- and sex-matched typically developing controls (TDC); and 2) participants with spastic cerebral palsy (SCP) matched for age, sex, term/preterm and gross motor function classification system (GMFCS). METHOD: This cross-sectional study was conducted by the University of Barcelona in collaboration with five institutions. Participants were people with DCP (n = 52; 24 females, median age 20.5 y: 5mo, interquartile range [IQR] = 13.75 y: 7mo; GMFCS I-V). As comparison groups, participants with SCP (n = 20; 10 females, median age = 20.5 y: 5.5mo, IQR = 13.75 y 9mo; GMFCS I-V) and TDC (n = 52; 24 females, median age = 20 y: 4mo, IQR = 12 y 7mo) were included. Intelligence and EF were assessed using common tests in all participants. RESULTS: Both CP groups had lower intelligence than TDC and performed poorer in almost all EF tasks. Intelligence was higher in DCP than SCP (z = -2.51, p = 0.01). Participants with DCP also performed significantly better in goal-setting tasks (z = 2.27, p = 0.03) and information processing (z = -2.54, p = 0.01) than those with SCP. CONCLUSION: People with DCP present lower general intellectual functioning and poorer EF across multiple domains than typically developing controls. People with DCP have higher general intellectual functioning and better EF than people with SCP when levels of motor severity are similar.

Cognitive functioning in dyskinetic cerebral palsy: Its relation to motor function, communication and epilepsy.

BACKGROUND: Cerebral palsy (CP) is a disorder of motor function often accompanied by cognitive impairment. There is a paucity of research focused on cognition in dyskinetic CP and on the potential effect of related factors. AIM: To describe the cognitive profile in dyskinetic CP and to assess its relationship with motor function and associated impairments. METHOD: Fifty-two subjects with dyskinetic CP (28 males, mean age 24 y 10 mo, SD 13 y) and 52 typically-developing controls (age- and gender-matched) completed a comprehensive neuropsychological assessment. Gross Motor Function Classification System (GMFCS), Communication Function Classification System (CFCS) and epilepsy were recorded. Cognitive performance was compared between control and CP groups, also according different levels of GMFCS. The relationship between cognition, CFCS and epilepsy was examined through partial correlation coefficients, controlling for GMFCS. RESULTS: Dyskinetic CP participants performed worse than controls on all cognitive functions except for verbal memory. Milder cases (GMFCS I) only showed impairment in attention, visuoperception and visual memory. Participants with GMFCS II-III also showed impairment in language-related functions. Severe cases (GMFCS IV-V) showed impairment in intelligence and all specific cognitive functions but verbal memory. CFCS was associated with performance in receptive language functions. Epilepsy was related to performance in intelligence, visuospatial abilities, visual memory, grammar comprehension and learning. CONCLUSION: Cognitive performance in dyskinetic CP varies with the different levels of motor impairment, with more cognitive functions impaired as motor severity increases. This study also demonstrates the relationship between communication and epilepsy and cognitive functioning, even controlling for the effect of motor severity.

Measuring intellectual ability in cerebral palsy: The comparison of three tests and their neuroimaging correlates.

Standard intelligence scales require both verbal and manipulative responses, making it difficult to use in cerebral palsy and leading to underestimate their actual performance. This study aims to compare three intelligence tests suitable for the heterogeneity of cerebral palsy in order to identify which one(s) could be more appropriate to use. Forty-four subjects with bilateral dyskinetic cerebral palsy (26 male, mean age 23 years) conducted the Raven's Coloured Progressive Matrices (RCPM), the Peabody Picture Vocabulary Test-3rd (PPVT-III) and the Wechsler Nonverbal Scale of Ability (WNV). Furthermore, a comprehensive neuropsychological battery and magnetic resonance imaging were assessed. The results show that PPVT-III gives limited information on cognitive performance and brain correlates, getting lower intelligence quotient scores. The WNV provides similar outcomes as RCPM, but cases with severe motor impairment were unable to perform it. Finally, the RCPM gives more comprehensive information on cognitive performance, comprising not only visual but also verbal functions. It is also sensitive to the structural state of the brain, being related to basal ganglia, thalamus and white matter areas such as superior longitudinal fasciculus. So, the RCPM may be considered a standardized easy-to-administer tool with great potential in both clinical and research fields of bilateral cerebral palsy.

Disgenesias del tronco encefálico: pronóstico funcional y tratamiento rehabilitador. Serie de nueve casos / Brainstem dysgenesis: functional prognosis and rehabilitative treatment. A series of nine cases

Introducción. La disgenesia del tronco encefálico es una entidad clínica heterogénea, de baja incidencia y alta variabilidad clínica, que afecta a estructuras del tronco del encéfalo. La combinación de sintomatología, neuroimagen y estudios neurofisiológicos es la base diagnóstica. Objetivo. Conocer las características clínicas comunes, pronóstico funcional y necesidades de tratamiento rehabilitador en un grupo de niños con disgenesia del tronco encefálico. Pacientes y métodos. Estudio retrospectivo observacional de nueve pacientes diagnosticados de disgenesia del tronco encefálico controlados en consultas externas. Resultados. La edad media de los pacientes era de 5,5 años. Cinco presentaron alteraciones en la neuroimagen y, en los cinco con estudio neurofisiológico, éste estaba alterado. Seis presentaron hipotonía muscular; ocho, amimia/hipomimia;seis, hipoacusia central, y cinco, gastrostomía. Un tercio presentó un episodio de parada cardiorrespiratoria. En todos se detectó retraso psicomotor. Actualmente cinco realizan marcha autónoma por interiores y cuatro de ellos por exteriores. Un porcentaje elevado (77,7%) comprende órdenes simples y es capaz de comunicarse (66,6%). Conclusiones. Las alteraciones de los pares craneales, del tono muscular y la disfagia son las manifestaciones más comunesen nuestra población. El riesgo de broncoaspiración y parada cardiorrespiratoria supone una amenaza vital para estos pacientes. Todos los niños presentan retraso psicomotor y la mitad de ellos alcanza marcha autónoma. Dada la diversidadde discapacidad que presentan estos pacientes, consideramos necesario un tratamiento de rehabilitación integral eindividualizado para conseguir un nivel funcional óptimo. Necesitamos estudios con muestras más amplias para obtener grupos homogéneos y establecer el pronóstico funcional y las necesidades de tratamiento rehabilitador (AU)

Introduction. Brainstem dysgenesis is a heterogeneous clinical entity, with low incidence and high clinical variability, which affects structures in the brainstem. Diagnosis is based on the combination of symptoms, neuroimaging and neurophysiological studies. Aim. To determine the common clinical features, functional prognosis and rehabilitative treatment needs in a group of children with brainstem dysgenesis Patients and methods. Observation-based retrospective study of nine patients diagnosed with brainstem dysgenesis monitored in an outpatients department. Results. The mean age of the patients was 5.5 years. Five presented alterations in the neuroimaging scan and, in the five with a neurophysiological study, the results showed it to be altered. Six presented muscular hypotonia, eight had amimia/ hypomimia, six had central hypoacusis and five had gastrostomy. A third of them presented an episode of cardiorespiratory arrest. Delayed psychomotor skills were detected in all cases. Currently five of them are capable of walking autonomously indoors and four of them outdoors. A high percentage of them (77.7%) understand simple orders and are capable of communicating (66.6%). Conclusions. Alterations affecting the cranial nerves and muscle tone together with dysphagia are the most common manifestations in our population. The risk of bronchial aspiration and cardiorespiratory arrest is a threat to the lives of these patients. All the children present psychomotor retardation and half of them manage to accomplish autonomous walking. Given the diversity of the disability displayed by these patients, we believe a personalised, integral rehabilitative treatment is needed to obtain an optimal level of functioning. Further studies with wider samples are required to be able to obtain homogeneous groups and to establish the functional prognosis and needs in terms of rehabilitative treatment (AU)

[Brainstem dysgenesis: functional prognosis and rehabilitative treatment. A series of nine cases]. / Disgenesias del tronco encefalico: pronostico funcional y tratamiento rehabilitador. Serie de nueve casos.

INTRODUCTION: Brainstem dysgenesis is a heterogeneous clinical entity, with low incidence and high clinical variability, which affects structures in the brainstem. Diagnosis is based on the combination of symptoms, neuroimaging and neurophysiological studies. AIM: To determine the common clinical features, functional prognosis and rehabilitative treatment needs in a group of children with brainstem dysgenesis. PATIENTS AND METHODS: Observation-based retrospective study of nine patients diagnosed with brainstem dysgenesis monitored in an outpatients department. RESULTS: The mean age of the patients was 5.5 years. Five presented alterations in the neuroimaging scan and, in the five with a neurophysiological study, the results showed it to be altered. Six presented muscular hypotonia, eight had amimia/hypomimia, six had central hypoacusis and five had gastrostomy. A third of them presented an episode of cardiorespiratory arrest. Delayed psychomotor skills were detected in all cases. Currently five of them are capable of walking autonomously indoors and four of them outdoors. A high percentage of them (77.7%) understand simple orders and are capable of communicating (66.6%). CONCLUSIONS: Alterations affecting the cranial nerves and muscle tone together with dysphagia are the most common manifestations in our population. The risk of bronchial aspiration and cardiorespiratory arrest is a threat to the lives of these patients. All the children present psychomotor retardation and half of them manage to accomplish autonomous walking. Given the diversity of the disability displayed by these patients, we believe a personalised, integral rehabilitative treatment is needed to obtain an optimal level of functioning. Further studies with wider samples are required to be able to obtain homogeneous groups and to establish the functional prognosis and needs in terms of rehabilitative treatment.

TITLE: Disgenesias del tronco encefalico: pronostico funcional y tratamiento rehabilitador. Serie de nueve casos.Introduccion. La disgenesia del tronco encefalico es una entidad clinica heterogenea, de baja incidencia y alta variabilidad clinica, que afecta a estructuras del tronco del encefalo. La combinacion de sintomatologia, neuroimagen y estudios neurofisiologicos es la base diagnostica. Objetivo. Conocer las caracteristicas clinicas comunes, pronostico funcional y necesidades de tratamiento rehabilitador en un grupo de niños con disgenesia del tronco encefalico. Pacientes y metodos. Estudio retrospectivo observacional de nueve pacientes diagnosticados de disgenesia del tronco encefalico controlados en consultas externas. Resultados. La edad media de los pacientes era de 5,5 años. Cinco presentaron alteraciones en la neuroimagen y, en los cinco con estudio neurofisiologico, este estaba alterado. Seis presentaron hipotonia muscular; ocho, amimia/hipomimia; seis, hipoacusia central, y cinco, gastrostomia. Un tercio presento un episodio de parada cardiorrespiratoria. En todos se detecto retraso psicomotor. Actualmente cinco realizan marcha autonoma por interiores y cuatro de ellos por exteriores. Un porcentaje elevado (77,7%) comprende ordenes simples y es capaz de comunicarse (66,6%). Conclusiones. Las alteraciones de los pares craneales, del tono muscular y la disfagia son las manifestaciones mas comunes en nuestra poblacion. El riesgo de broncoaspiracion y parada cardiorrespiratoria supone una amenaza vital para estos pacientes. Todos los niños presentan retraso psicomotor y la mitad de ellos alcanza marcha autonoma. Dada la diversidad de discapacidad que presentan estos pacientes, consideramos necesario un tratamiento de rehabilitacion integral e individualizado para conseguir un nivel funcional optimo. Necesitamos estudios con muestras mas amplias para obtener grupos homogeneos y establecer el pronostico funcional y las necesidades de tratamiento rehabilitador.