Resumo Introdução: A síndrome de Cornelia de Lange (SCL) é uma síndrome genética rara. Crianças com SCL geralmente necessitam de fisioterapia, porém a eficácia da intervenção fisioterapêutica nessa população é escassa na literatura científica. Objetivo: O objetivo deste estudo foi relatar o efeito da Terapia Neuromotora Intensiva (TNMI) sobre a função motora grossa e participação de uma criança com SCL usando o modelo de Classificação Internacional de Funcionalidades, Incapacidades e Saúde (CIF). Método: Uma criança brasileira com SCL foi acompanhada durante sete meses, enquanto submetida a três módulos de TNMI. Resultados: A criança demonstrou uma evolução da função motora grossa com ganhos de 11,28% no primeiro módulo, 9,22% no segundo módulo e 10,29% no terceiro módulo do TNMI. Conclusão: a TNMI resultou em melhorias na função motora grossa e participação durante as atividades diárias em uma criança com SCL. São necessários mais estudos de coortes para investigar a eficácia do TNMI em crianças com SCL.

Abstract Introduction: The Cornelia de Lange Syndrome (CdLS) is a rare genetic syndrome. Children with CdLS usually require physical therapy, however the efficacy of physical therapy intervention in this population is lacking in the research literature. Objective: The aim of this study was to report the effect of Intensive Neuromotor Therapy (INMT) on gross motor function and participation of a child with CdLS using the International Classification of Functioning, Disabilities and Health (ICF) model. Method: A Brazilian child with CdLS was followed for over seven months while undergoing three modules of INMT. Results: The child demonstrated an evolution of gross motor function with gains of 11.28% in the first module, 9.22% in the second module, and 10.29% in the third module of INMT. Conclusion: INMT resulted in improvements in gross motor function and participation during daily activities in a child with CDLS. Further studies of larger cohorts are needed to investigate the efficacy of INMT in children with CdLS.

Resumen Introducción: El síndrome de Cornelia de Lange (SCL) es un síndrome genético raro. Los niños con SCL por lo general requieren fisioterapia, sin embargo, es escasa en la literatura científica la eficacia de esta intervención en este público. Objetivo: Informar el efecto de la terapia neuromotora intensiva (TNMI) en la función motora gruesa y la participación de un niño con SCL mediante el modelo de Clasificación Internacional de Funcionamiento, Discapacidades y Salud (CIF). Método: Se puso en seguimiento a un niño brasileño con SCL durante siete meses mientras se sometía a tres módulos de TNMI. Resultados: El niño demostró una evolución de la función motora gruesa con ganancias de un 11,28% en el primer módulo, de un 9,22% en el segundo y de un 10,29% en el tercer módulo de TNMI. Conclusión: La TNMI presentó mejoras en la función motora gruesa y en la participación durante las actividades diarias en un niño con SCL. Se necesitan futuras investigaciones adicionales de cohortes para investigar la eficacia de TNMI en niños con SCL.

Cerebral palsy (CP) is the most common disability in children caused by central nervous system lesion. The aim of the present study was to verify the intensive neuromotor therapy effects in children with CP, in a reference Brazilian centre. In this study, three years of medical records from a Brazilian reference Centre of Intensive Neuromotor Therapy (INMT) which use the INMT protocol were analysed. The motor evaluation for each child was done by the Gross Motor Function Classification System (GMFCS) and GMFM-88 by an experienced professional, before and after each INMT module. A total of 53 children between the ages of 1 and 15 years (age at treatment initiation, initial evaluation), with a mean age of 5.94±3.38 years, participated in the study. Participants performed between 1 and 10 INMT modules. There was no strong correlation between age and overall performance on the GMFM scale, but it was observed a strong negative correlation between the percentage of GMFM gains and the number of modules (r=-0.709; R² = 0.50; p = 0.022, CI95%[0.014 - 0.026]), suggesting that patients tend to present higher percentage gains in the first modules. Through an intra-module comparison, it was observed statistical difference in the total score in each of the modules.

Abstract Introduction The aim of this study was to develop a specific mathematical model to estimate the body fat percentage (BF%) of children with cerebral palsy, based on a Brazilian population of patients with this condition. Method This is a descriptive cross-sectional study. The study included 63 Caucasian children with cerebral palsy, both males and females, aged between three and ten-years-old. Participants were assessed for functional motor impairment using the Gross Motor Function Classification System (GMFCS), dual energy x-ray absorptiometry (DXA) and skinfold thickness. Total body mass (TBM) and skinfolds thickness from: triceps (Tr), biceps (Bi), Suprailiac (Si), medium thigh (Th), abdominal (Ab), medial calf (Ca) and subscapular (Se) were collected. Fat mass (FM) was estimated by dual energy x-ray absorptiometry (gold standard). Results The model was built from multivariate linear regression; FM was set as a dependent variable and other anthropometric variables, age and sex, were set as independent variables. The final model was established as F%=((0.433xTBM + 0.063xTh + 0.167xSi - 6.768) ÷ TBM) × 100, the R2 value was 0.950, R2adjusted=0.948 and the standard error of estimate was 1.039 kg. Conclusion This method was shown to be valid to estimate body fat percentage of children with cerebral palsy. Also, the measurement of skinfolds on both sides of the body showed good results in this modelling.

INTRODUÇÃO: O Protocolo PediaSuitTM é uma terapia intensiva com uma abordagem holística para o tratamento de indivíduos com distúrbios neurológicos, como paralisia cerebral (PC), atrasos de desenvolvimento, lesões cerebrais traumáticas, autismo e outras condições que afetam as funções motoras e/ou cognitivas de uma criança. OBJETIVO: O objetivo do presente trabalho foi descrever o Protocolo PediaSuitTM. MÉTODOS: A equipe de autores permaneceu dois meses observando os cuidados prestados em uma clínica com fisioterapeutas treinados pela equipe PediaSuit ProtocolTM (EUA). RESULTADOS: O PediaSuitTM é um protocolo terapêutico que utiliza um traje especial combinado com a terapia física intensiva, com duração de até quatro horas por dia, cinco dias por semana, durante três ou quatro semanas. O Protocolo PediaSuitTM é personalizado para atender às necessidades de cada criança, com objetivos específicos, e, geralmente, envolve um programa intensivo de reabilitação. Ele combina os melhores elementos de várias técnicas e métodos e tem bons fundamentos com base na fisiologia do exercício. CONCLUSÃO: Esse protocolo antecipa resultados obtidos somente com longos períodos de fisioterapia tradicional.

INTRODUCTION: PediaSuit ProtocolTM is an intensive therapy with a holistic approach to the treatment of individuals with neurological disorders like cerebral palsy (CP), developmental delays, traumatic brain injuries, autism and other conditions which affect a child's motor and/or cognitive functions. OBJECTIVE: The aim of the present work is to describe the PediaSuit ProtocolTM. METHODS: The authors team remained two months observing the care provided in a clinic with physical therapists trained by the PediaSuit ProtocolTM team (USA). RESULTS: The PediaSuitTM is a therapeutic protocol which uses a suit combined with intensive physical therapy and consists of up to four hours of therapy a day, five days a week, during three or four weeks. The PediaSuit ProtocolTM is customized to fit the needs of each child, with specific functional goals, and usually involves an intensive rehabilitation program. It combines the best elements of various techniques and methods, and has a sound rationale based on exercise physiology. CONCLUSION: This protocol anticipates results obtained only with long periods of conventional physical therapy.