Parental reports of the oral health-related quality of life of children with cerebral palsy

Background: The severity of physical and mental impairments and oral problems, as well as socioeconomic factors, may have an impact on quality of life of children with cerebral palsy (CP). The aim of this research was to assess the impact of impairments and oral health conditions, adjusted by socioeconomic factors, on the Oral Health-Related Quality of Life (OHRQoL) of children with CP using their parents as proxies. Methods: Sixty children, between 6-14 years of age were selected. Their parents answered a children's OHRQoL instrument (5 domains) which combines the Parental-Caregivers Perception Questionnaire (P-CPQ) and Family Impact Scale (FIS). The severity of dental caries, type of CP, communication ability, gross motor function, seizures and socioeconomic conditions were assessed. Results: Considering the total score of the OHRQoL instrument, only the reduction of communication ability and dental caries severity had a negative impact on the OHRQoL (p < 0.05). Considering each domain of the instrument, the severity of the type of CP and its reduction of communication ability showed a negative impact on oral symptoms and functional limitations domains (p < 0.05). Seizures have a negative impact on oral symptoms domain (p = 0.006). The multivariate fitted model showed that the severity of dental caries, communication ability and low family income were negatively associated with the impact on OHRQoL (p = 0.001). Conclusions: The severity of dental caries, communication ability, and family income are conditions strongly associated with a negative impact on OHRQoL of children with CP.

Visual impairment in children with spastic cerebral palsy measured by psychophysical and electrophysiological grating acuity tests

Background: This study measured grating visual acuity in 173 children between 6-48 months of age who had different types of spastic cerebral palsy (CP). Method: Behavioural acuity was measured with the Teller Acuity Cards (TAC) using a staircase psychophysical procedure. Electrophysiological visual acuity was estimated using the sweep VEP (sVEP). Results: The percentage of children outside the superior tolerance limits was 44 of 63 (69%) and 50 of 55 (91%) of tetraplegic, 36 of 56 (64%) and 42 of 53 (79%) of diplegic, 10 of 48 (21%) and 12 of 40 (30%) of hemiplegic for sVEP and TAC, respectively. For the sVEP, the greater visual acuity deficit found in the tetraplegic group was significantly different from that of the hemiplegic group (p < 0.001). In the TAC procedure the mean visual acuity deficits of the tetraplegic and diplegic groups were significantly different from that of hemiplegic group (p < 0.001). The differences between sVEP and TAC means of visual acuity difference were statistically significant for the tetraplegic (p < 0.001), diplegic (p < 0.001), and hemiplegic group (p = 0.004). Discussion: Better visual acuities were obtained in both procedures for hemiplegic children compared to diplegic or tetraplegic. Tetraplegic and diplegic children showed greater discrepancies between the TAC and sVEP results. Inter-ocular acuity difference was more frequent in sVEP measurements. Conclusions: Electrophysiologically measured visual acuity is better than behavioural visual acuity in children with CP.

Revisão sistemática das escalas utilizadas para avaliação funcional na doença de Pompe

OBJETIVO: Identificar as escalas utilizadas para avaliação funcional na doença de Pompe (DP) e descrever seu nível de evidência e recomendação. FONTES DE DADOS: Revisão sistemática sobre as escalas de avaliação funcional na DP. Pesquisa realizada nos bancos de dados Medline, Lilacs, Registro Cochrane de Ensaios Controlados Central (CCTR) e SciELO com artigos (exceto artigos de revisão) publicados entre 2000 e 2010. As palavras-chave utilizadas nos idiomas português e inglês foram: doença de depósito de glicogênio tipo II, atividades cotidianas, avaliação. Os artigos foram classificados em nível de evidência e recomendação. SÍNTESE DOS DADOS: Foram incluídos 14 estudos que avaliaram desde recém-nascidos a adultos (amostra total=449). Foram encontradas as seguintes escalas na literatura: Pediatric Evaluation of Disability Inventory (PEDI) e sua forma adaptada para DP (Pompe-PEDI), Alberta Infant Motor Scale (AIMS), Rotterdam Handiscap Scale (RHS), Functional Independence Measure (FIM), Gross Motor Function Measure (GMFM) e Peabody Developmental Motor Scales (PDMS-II). A maioria dos estudos apresentou nível de evidência III, por serem não randomizados. Grau de recomendação das escalas: C para AIMS e Pompe-PEDI; D para GMFM e PDMS-II; E para RHS e FIM. CONCLUSÕES: A maioria das escalas utilizadas para avaliação funcional na DP apresenta baixo nível de evidência e recomendação. As que apresentam melhor grau de recomendação (C) são as escalas AIMS e Pompe-PEDI aplicadas em Pediatria.

Preoperative MRI findings and functional outcome after selective dorsal rhizotomy in children with bilateral spasticity

PURPOSE: To identify MRI characteristics that may predict the functional effect of selective dorsal rhizotomy (SDR) in children with bilateral spastic paresis. METHODS: We performed SDR in a group of 36 patients. The gross motor functioning measure-66 (GMFM-66) was applied before and after SDR. Available cerebral MRIs were retrospectively classified into three diagnostic groups: periventricular leucomalacia (PVL; n = 10), hydrocephalus (n = 2), and normal (n = 6). In patients with PVL, we scored the severity of the MR abnormalities. We compared the changes in the GMFM-66 after SDR in the diagnostic groups. In patients with PVL, we correlated the severity of the MR abnormalities with the changes in the GMFM-66. RESULTS: The mean follow-up period was 5 years and 4 months (range, 1 year and 1 month to 9 years). The best improvement in gross motor function was observed in patients with normal MRI, and the slightest improvement was observed in patients with hydrocephalus. The severity of the PVL did correlate with the GMFM-66 score before SDR but not with the functional effect of SDR. CONCLUSION: We conclude that with respect to gross motor skills, the improvements after SDR are good in patients with no MRI abnormalities. In the patients with hydrocephalus, the improvements after SDR were insignificant. In patients with PVL, the improvements were intermediate and did not correlate with the degree of PVL.

The updated European Consensus 2009 on the use of Botulinum toxin for children with cerebral palsy

An interdisciplinary European group of clinical experts in the field of movement disorders and experienced Botulinum toxin users has updated the consensus for the use of Botulinum toxin in the treatment of children with cerebral palsy (CP). A problem-orientated approach was used focussing on both published and practice-based evidence. In part I of the consensus the authors have tabulated the supporting evidence to produce a concise but comprehensive information base, pooling data and experience from 36 institutions in 9 European countries which involves more than 10,000 patients and over 45,000 treatment sessions during a period of more than 280 treatment years. In part II of the consensus the Gross Motor Function Measure (GMFM) and Gross Motor Function Classification System (GMFCS) based Motor Development Curves have been expanded to provide a graphical framework on how to treat the motor disorders in children with CP. This graph is named "CP(Graph) Treatment Modalities - Gross Motor Function" and is intended to facilitate communication between parents, therapists and medical doctors concerning (1) achievable motor function, (2) realistic goal-setting and (3) treatment perspectives for children with CP. The updated European consensus 2009 summarises the current understanding regarding an integrated, multidisciplinary treatment approach using Botulinum toxin for the treatment of children with CP.

Outcome at two years of age in a Swiss national cohort of extremely preterm infants born between 2000 and 2008

Background While survival rates of extremely preterm infants have improved over the last decades, the incidence of neurodevelopmental disability (ND) in survivors remains high. Representative current data on the severity of disability and of risk factors associated with poor outcome in this growing population are necessary for clinical guidance and parent counselling. Methods Prospective longitudinal multicentre cohort study of preterm infants born in Switzerland between 240/7 and 276/7 weeks gestational age during 2000–2008. Mortality, adverse outcome (death or severe ND) at two years, and predictors for poor outcome were analysed using multilevel multivariate logistic regression. Neurodevelopment was assessed using Bayley Scales of Infant Development II. Cerebral palsy was graded after the Gross Motor Function Classification System. Results Of 1266 live born infants, 422 (33%) died. Follow-up information was available for 684 (81%) survivors: 440 (64%) showed favourable outcome, 166 (24%) moderate ND, and 78 (11%) severe ND. At birth, lower gestational age, intrauterine growth restriction and absence of antenatal corticosteroids were associated with mortality and adverse outcome (p < 0.001). At 360/7 weeks postmenstrual age, bronchopulmonary dysplasia, major brain injury and retinopathy of prematurity were the main predictors for adverse outcome (p < 0.05). Survival without moderate or severe ND increased from 27% to 39% during the observation period (p = 0.02). Conclusions In this recent Swiss national cohort study of extremely preterm infants, neonatal mortality was determined by gestational age, birth weight, and antenatal corticosteroids while neurodevelopmental outcome was determined by the major neonatal morbidities. We observed an increase of survival without moderate or severe disability.

Selection criteria for selective dorsal rhizotomy in children with spastic cerebral palsy: a systematic review of the literature

AIM Information regarding the selection procedure for selective dorsal rhizotomy (SDR) in children with spastic cerebral palsy (CP) is scarce. Therefore, the aim of this study was to summarize the selection criteria for SDR in children with spastic CP. METHOD A systematic review was carried out using the following databases: MEDLINE, CINAHL, EMBASE, PEDro, and the Cochrane Library. Additional studies were identified in the reference lists. Search terms included 'selective dorsal rhizotomy', 'functional posterior rhizotomy', 'selective posterior rhizotomy', and 'cerebral palsy'. Studies were selected if they studied mainly children (<18y of age) with spastic CP, if they had an intervention of SDR, if they had a detailed description of the selection criteria, and if they were in English. The levels of evidence, conduct of studies, and selection criteria for SDR were scored. RESULTS Fifty-two studies were included. Selection criteria were reported in 16 International Classification of Functioning, Disability and Health model domains including 'body structure and function' (details concerning spasticity [94%], other movement abnormalities [62%], and strength [54%]), 'activity' (gross motor function [27%]), and 'personal and environmental factors' (age [44%], diagnosis [50%], motivation [31%], previous surgery [21%], and follow-up therapy [31%]). Most selection criteria were not based on standardized measurements. INTERPRETATION Selection criteria for SDR vary considerably. Future studies should describe clearly the selection procedure. International meetings of experts should develop more uniform consensus guidelines, which could form the basis for selecting candidates for SDR.

Validación de un instrumento para evaluar la carga del cuidador en parálisis cerebral

Esta investigación pretende desarrollar y validar una escala para evaluar la carga del cuidador del niño con parálisis cerebral (PC) de niveles funcionales GMFCS (Gross Motor Function Classification System) IV y V.

Parental reports of the oral health-related quality of life of children with cerebral palsy

BACKGROUND The severity of physical and mental impairments and oral problems, as well as socioeconomic factors, may have an impact on quality of life of children with cerebral palsy (CP). The aim of this research was to assess the impact of impairments and oral health conditions, adjusted by socioeconomic factors, on the Oral Health-Related Quality of Life (OHRQoL) of children with CP using their parents as proxies. METHODS Sixty children, between 6-14 years of age were selected. Their parents answered a children's OHRQoL instrument (5 domains) which combines the Parental-Caregivers Perception Questionnaire (P-CPQ) and Family Impact Scale (FIS). The severity of dental caries, type of CP, communication ability, gross motor function, seizures and socioeconomic conditions were assessed. RESULTS Considering the total score of the OHRQoL instrument, only the reduction of communication ability and dental caries severity had a negative impact on the OHRQoL (p < 0.05). Considering each domain of the instrument, the severity of the type of CP and its reduction of communication ability showed a negative impact on oral symptoms and functional limitations domains (p < 0.05). Seizures have a negative impact on oral symptoms domain (p = 0.006). The multivariate fitted model showed that the severity of dental caries, communication ability and low family income were negatively associated with the impact on OHRQoL (p = 0.001). CONCLUSIONS The severity of dental caries, communication ability, and family income are conditions strongly associated with a negative impact on OHRQoL of children with CP.

L'effet de l'hippothérapie sur le contrôle postural et la motricité d'enfants ayant une déficience motrice cérébrale légère

L'enfant ayant une déficience motrice cérébrale (DMC) légère présente des déficits posturaux qui limitent son intégration sociale. L'hippothérapie est une forme d'intervention utilisant le mouvement du cheval pour stimuler des réajustements posturaux. L’objectif de cette recherche était de quantifier l’effet de 10 semaines d’hippothérapie sur le contrôle postural et la motricité d'enfants ayant une DMC légère. Un devis pré-expérimental à mesures répétées où chaque enfant (n=13) est son propre contrôle a été utilisé. Les critères d’inclusion étaient: être âgés entre 4 et 16 ans et avoir une DMC légère. L’intervention d’une durée de 10 semaines consistait en une séance par semaine d’hippothérapie de 30 minutes, où l'enfant était placé dans différentes positions sur le cheval. La motricité globale, particulièrement la capacité à se tenir debout, marcher, courir et sauter a été mesurée par les dimensions D et E du Gross Motor Function Measure (GMFM-88) et la coordination, la vitesse, la force et l’équilibre par le Bruininks-Oseretski Test of Motor Proficiency-Short Form (BOT2-SF). La motricité fine a été évaluée par différentes tâches de précision, d’intégration et de dextérité manuelle (BOT2-SF). Les variables biomécaniques évaluant la stabilité posturale en position assise et debout ont été quantifiées par le déplacement du centre de pression (CdeP). Le déplacement des membres supérieurs a été enregistré lors de tâches unilatérales à l'aide d’un système d’analyse tridimensionnelle du mouvement (VICON). Treize sujets (âgés de 7,3 ± 2,7 ans) ont été évalués avant (mesure de base), après l’intervention et à 10 semaines post-intervention (mesure de suivi). La stabilité posturale de l’enfant sur le cheval a été évaluée à l’aide de modules inertiels multiaxiaux fixés sur le cheval et sur l’enfant (tête, tronc) à deux temps pendant l’intervention. À la suite de l’intervention, la motricité globale s’est améliorée significativement (GMFM-88 dimensions; p=0,005 et BOT2-SF total; p=0,006), et spécifiquement au niveau de la force des abdominaux et des membres supérieurs (p=0,012), de l’équilibre (p=0,025) et des activités de précision de la main (p=0,013). Les analyses du contrôle postural sur le cheval montrent une augmentation de la fréquence cumulée dans la direction médiolatérale (M/L) (p=0,033), et une diminution de l’amplitude de la fréquence cumulée en vertical (p=0,007). Ces résultats peuvent s’interpréter comme étant une augmentation de la rapidité d'adaptation dans la direction M/L doublée d'un amortissement du tronc diminué dans l’axe vertical. Le contrôle postural debout statique s'est amélioré (p=0,013) dans l'axe M/L attribuable aux enfants diplégiques de l'étude. Ces résultats se sont maintenus après la fin de l’intervention. Aucune amélioration du déplacement des membres supérieurs n'a été notée. Nos résultats permettent de suggérer l’utilisation de l'hippothérapie, par les professionnels de la réadaptation, comme complément à l'intervention traditionnelle.

As versões portuguesas da GMFM-66 B&C e da GMFM-66 IS

A Gross Motor Function Measure (GMFM) é a medida de ouro para avaliar alterações na função motora ao longo do tempo ou em resposta a uma intervenção em crianças com Paralisia Cerebral (PC) (Russell D. , Rosenbaum, Avery, & Lane, 2002). Uma das barreiras à utilização mais frequente da GMFM é o seu tempo de administração, que dura entre 40 a 60 minutos. Para responder à necessidade de versões mais reduzidas da GMFM mas sem perder o seu carácter discriminativo e altamente sensível à mudança, foram publicadas as versões Gross Motor Function Measure- Item Sets (GMFM-66 IS) e a Gross Motor Function Measure Basal and Ceiling (GMFM-66-B&C), tornando a avaliação da função motora menos morosa, e assim melhorando a sua aplicabilidade. A GMFM-66 IS baseia-se num algoritmo para determinar quais os itens a serem avaliados e a GMFM-66 B&C tem como abordagem os efeitos de chão e teto de acordo com as idades e níveis do Sistema de Classificação da Função Motora Grosseira (SCFMG) (Brutton & Bartlett, 2011). O objetivo deste estudo foi criar as versões portuguesas da GMFM-IS e GMFM-B&C. Tratou-se de um estudo de natureza metodológica, descritivo, longitudinal em crianças com PC, dividido em duas fases: 1. Tradução e adaptação cultural e linguística da GMFM-66 IS e da GMFM-66 B&C; 2. Estudo de validação com análise da fiabilidade (coerência interna, reprodutibilidade e fiabilidade inter-observador), validade e poder de resposta. A amostra em estudo foi constituída por 100 crianças com PC com idades compreendidas entre os 2 e os 12 anos, representativa de todos os 5 níveis do Sistema de Classificação da Função Motora Global. As versões portuguesas da GMFM-66-IS e da GMFM-66-B&C apresentam equivalência conceptual e semântica com as versões originais revelando fácil aplicabilidade. Demonstrou-se que as versões reduzidas portuguesas da GMFM apresentam muito boa consistência interna, com valores globais do Alfa de Cronbach de 0,998, muito boa concordância entre os avaliadores (ICC de 0,998 para a GMFM-66-B&C e de 0,999 para a GMFM-66-IS), e com valores de fiabilidade intra-observador excelentes (ICC de 0,999 para a GMFM-66-B&C e de 1,000 para a GMFM-66-IS). Quanto ao poder de resposta os resultados não foram tão expressivos, provavelmente comprometidos por uma amostra demasiado pequena. As versões portuguesas da GMFM-66-IS e da GMFM-66-B&C revelaram ter características psicométricas adequadas à sua aplicação em PC, necessitando, no entanto, de mais investigação relativamente à sua capacidade de detetar mudança como resultado de intervenções.

Music-supported training is more efficient than functional motor training for recovery of fine motor skills in stroke patients

MOTOR IMPAIRMENTS ARE COMMON AFTER STROKE but efficacious therapies for these dysfunctions are scarce. Extending an earlier study on the effects of music-supported training (MST), behavioral indices of motor function were obtained before and after a series of training sessions to assess whether this new treatment leads to improved motor functions. Furthermore, music-supported training was contrasted to functional motor training according to the principles of constraint-induced therapy (CIT). In addition to conventional physiotherapy, 32 stroke patients with moderately impaired motor function and no previous musical experience received 15 sessions of MST over a period of three weeks, using a manualized, step-bystep approach. A control group consisting of 15 patients received 15 sessions of CIT in addition to conventional physiotherapy. A third group of 30 patients received exclusively conventional physiotherapy and served as a control group for the other three groups. Fine as well as gross motor skills were trained by using either a MIDI-piano or electronic drum pads programmed to emit piano tones. Motor functions were assessed by an extensive test battery. MST yielded significant improvement in fine as well as gross motor skills with respect to speed, precision, and smoothness of movements. These improvements were greater than after CIT or conventional physiotherapy. In conclusion, with equal treatment intensity, MST leads to more pronounced improvements of motor functions after stroke than CIT.

Restoring function after neuromuscular trauma: novel paradigms for enhancing volitional control of neural activity in untethered environments and vHAB, a gamified therapy platform

Thesis (Ph.D.)--University of Washington, 2016-08

Methods for objective and interpretative evaluation of function and motor control: gait in perturbed condition and in the aquatic therapy

Gait analysis allows to characterize motor function, highlighting deviations from normal motor behavior related to an underlying pathology. The widespread use of wearable inertial sensors has opened the way to the evaluation of ecological gait, and a variety of methodological approaches and algorithms have been proposed for the characterization of gait from inertial measures (e.g. for temporal parameters, motor stability and variability, specific pathological alterations). However, no comparative analysis of their performance (i.e. accuracy, repeatability) was available yet, in particular, analysing how this performance is affected by extrinsic (i.e. sensor location, computational approach, analysed variable, testing environmental constraints) and intrinsic (i.e. functional alterations resulting from pathology) factors. The aim of the present project was to comparatively analyze the influence of intrinsic and extrinsic factors on the performance of the numerous algorithms proposed in the literature for the quantification of specific characteristics (i.e. timing, variability/stability) and alterations (i.e. freezing) of gait. Considering extrinsic factors, the influence of sensor location, analyzed variable, and computational approach on the performance of a selection of gait segmentation algorithms from a literature review was analysed in different environmental conditions (e.g. solid ground, sand, in water). Moreover, the influence of altered environmental conditions (i.e. in water) was analyzed as referred to the minimum number of stride necessary to obtain reliable estimates of gait variability and stability metrics, integrating what already available in the literature for over ground gait in healthy subjects. Considering intrinsic factors, the influence of specific pathological conditions (i.e. Parkinson’s Disease) was analyzed as affecting the performance of segmentation algorithms, with and without freezing. Finally, the analysis of the performance of algorithms for the detection of gait freezing showed how results depend on the domain of implementation and IMU position.

Twin-twin Transfusion Syndrome: Neurodevelopmental Screening Test.

Objective To assess the neurodevelopmental functions (cognition, language and motor function) of survivors of twin-twin transfusion syndrome (TTTS). Method Observational cross-sectional study of a total of 67 monochorionic diamniotic twins who underwent fetoscopic laser coagulation (FLC) for treatment of TTTS. The study was conducted at the Center for Investigation in Pediatrics (CIPED), Universidade Estadual de Campinas. Ages ranged from one month and four days to two years four months. Bayley Scales of Infant and Toddler Development Screening Test-III, were used for evaluation. Results Most children reached the competent category and were classified as having appropriate performance. The preterm children scored worse than term infants for gross motor subtest (p = 0.036). Conclusion The majority of children reached the expected development according to their age. Despite the good neurodevelopment, children classified at risk should be monitored for development throughout childhood.