Can the positions of the spastic upper limb in stroke survivors help muscle choice for botulinum toxin injections? / A posição do membro superior de indivíduos com doença cerebrovascular pode auxiliar na escolha de músculos para a injeção de toxina botulínica?

ABSTRACT Motor impairments in stroke survivors are prevalent and contribute to dependence in daily activities, pain and overall disability, which can further upper-limb disability. Treatment with botulinum toxin A (BoNT-A) is indicated for focal spasticity and requires knowledge of biomechanics and anatomy to best select muscles to be injected in the limb. Objective: We aimed to describe the frequency of posture patterns in a Brazilian sample of stroke survivors and correlate them with recommendations of muscle selection for treatment with BoNT-A. Methods: Fifty stroke patients with spastic upper limbs scheduled for neuromuscular block were photographed and physically examined, to be classified by three independent evaluators according to Hefter's classification. Muscles that were injected with BoNT-A by their routine doctors were retrieved from medical charts. Results: Pattern III and IV were the most common (64.7%, 21.6%). We further subclassified pattern III according to the rotation of the shoulder, which effectively interfered in muscle choice. The muscles most frequently treated were shoulder adductors and internal rotators, elbow flexors and extensors, in forearm, the pronator teres and finger and wrist flexors, and, in the hand the adductor pollicis. Conclusion: Frequencies of upper-limb postures differed from previous reports. Other clinical features, besides spasticity, interfered with muscle choice for BoNT-A injection, which only partially followed the recommendations in the literature.

RESUMO As deficiências motoras que ocorrem nos indivíduos com doença cerebrovascular (DCV) são prevalentes e contribuem para dependência, dor e incapacidade, o que pode atrasar a reabilitação do membro superior e sua funcionalidade. O tratamento com toxina botulínica do tipo A (BoNT-A) é indicado para a espasticidade focal e requer conhecimento da biomecánica e anatomia para melhor selecionar os músculos a serem injetados. Objetivo: Descrever a frequência de padrões posturais numa amostra de brasileiros com sequelas de DCV e correlacioná-los com as recomendações de seleção de músculos. Métodos: Cinquenta pacientes com comprometimento do membro superior devido a DCV do ambulatório de bloqueios neuromusculares foram fotografados e examinados para categorização de acordo com a Classificação de Hefter. Os músculos tratados pelos seus médicos de rotina foram obtidos a partir dos prontuários. Resultados: Os padrões III e IV de Hefter foram mais comuns (64,7%; 21,6%). Nós propusemos a subclassificação do padrão III de acordo com a rotação do ombro, pois isso interferiu na escolha dos músculos tratados. Os músculos tratados com maior frequência foram os adutores e rotadores internos do ombro; flexores e extensores do cotovelo; no antebraço, o pronador redondo, flexores dos dedos e do carpo e na mão, o adutor do polegar. Conclusão: As frequências das posições do membro superior diferiram de relatos prévios. Além da espasticidade, outros fatores interferiram na escolha dos músculos tratados, que seguiram parcialmente as recomendações da literatura.

Botulinum toxin for hereditary spastic paraplegia: effects on motor and non-motor manifestations / Toxina botulínica nas paraplegias espásticas hereditárias: efeitos nas manifestações motoras e não-motoras

ABSTRACT Motor and non-motor manifestations are common and disabling features of hereditary spastic paraplegia (HSP). Botulinum toxin type A (Btx-A) is considered effective for spasticity and may improve gait in these patients. Little is known about the effects of Btx-A on non-motor symptoms in HSP patients. Objective To assess the efficacy of Btx-A on motor and non-motor manifestations in HSP patients. Methods Thirty-three adult patients with a clinical and molecular diagnosis of HSP were evaluated before and after Btx-A injections. Results Mean age was 41.7 ± 13.6 years and there were 18 women. Most patients had a pure phenotype and SPG4 was the most frequent genotype. The Btx-A injections resulted in a decrease in spasticity at the adductor muscles, and no other motor measure was significantly modified. In contrast, fatigue scores were significantly reduced after Btx-A injections. Conclusion Btx-A injections resulted in no significant functional motor improvement for HSP, but fatigue improved after treatment.

RESUMO Manifestações motoras e não motoras são comuns e incapacitantes nas paraparesias espásticas hereditárias (PEH). Toxina botulínica do tipo A (TB-A) é considerada eficaz no tratamento da espasticidade e pode melhorar a marcha nesses pacientes. Pouco se sabe sobre os efeitos da TB-A sobre sintomas não-motores. Objetivo avaliar a eficácia da TB-A sobre manifestações motoras e não-motoras nas PEH. Método trinta e três pacientes adultos com PEH foram avaliados antes e depois das aplicações de TB-A. Resultados A média de idade foi 41,7 ± 13,6 anos e havia 18 mulheres. A maioria dos pacientes portava a forma pura e o genótipo mais comum foi SPG4. Houve diminuição da espasticidade dos músculos adutores da coxa sem melhora da marcha. A pontuação da fadiga reduziu após as injeções. Conclusão As aplicações de TB-A não melhoraram a marcha nos pacientes mas a redução da fadiga foi significativa após o tratamento.

Uso prático da AbobotulinumtoxinA no tratamento de espasticidade em crianças com paralisia cerebral / Practical use of AbobotulinumtoxinA for the treatment of spasticity in children with cerebral palsy

AbobotulinumtoxinA (ABO) tem sido utilizada para o tratamento da espasticidade em crianças com paralisia cerebral (PC). Seu uso requer uma administração cuidadosa, quanto à dosagem, seleção de locais de aplicação, intervalo entre aplicações, eficácia e segurança. Este foi o primeiro painel de especialistas no tratamento da espasticidade que desenvolveu um guia sobre questões gerais relacionadas a terapêutica de médicos que utilizam ABO, incluindo a indicação da dosagem a ser aplicada por músculo. O tratamento deve ser iniciado o mais rápido, idealmente entre dois e seis anos de idade. Uma avaliação clínica deve identificar os músculos espásticos e determinar o objetivo: melhora funcional, analgesia, facilidade de cuidados e posicionamento, prevenção da luxação dos quadris, melhora da marcha e postura, facilitação do processo de educação, maior participação social e/ou melhora estética. Os pré-requisitos para alcançar bons resultados são a seleção muscular adequada, a dosagem de ABO e a técnica de injeção. Muitos padrões patológicos comuns podem ser tratados se vários músculos forem simultaneamente injetados em uma única sessão de tratamento; O planejamento da dose de ABO por músculo deve levar em consideração a dosagem máxima em unidades por músculo e a dose de ABO máxima total por sessão (30 U / kg de peso corporal do paciente, não superior a 1000 U). Após a aplicação, as crianças devem ser submetidas a programas de fisioterapia e terapia ocupacional, focadas em orientações domiciliares e em orientações para a família, aumentando as chances de ganho terapêutico. O tratamento com ABO é multidisciplinar e requer abordagens integradas

AbobotulinumtoxinA (ABO) has been used for the treatment of spasticity in children with cerebral palsy (CP). Its use requires careful administration, regarding dosing, selection of local of application, interval between applications and efficacy and safety monitoring. This was the first panel of experts on the treatment of spasticity, which developed a guide to provide an overview on important issues related to therapeutic strategies adopted by physicians using ABO, including its dosage to be applied per muscle.Treatment should be initiated as soon as possible, ideally between two and six years old. A clinical evaluation should identify muscles presenting spastic activity, and determine desired outcome: improvement of function, esthetics/aspect, pain treatment, easing care and positioning, preventing hips dislocation, improvement of walking and posture, and to provide conditions for education and social participation. Pre-requisites to achieve good results are adequate muscle selection, adequate ABO dosage and exact injection technique. Many common pathological patterns can be adequately treated if several muscles are simultaneously injected in a single treatment session; planning ABO dosage per muscle should take into consideration the maximum dosage in units per muscle and the total maximum ABO dosage per session (30 U/kg patient´s body weight, not exceeding 1000 U).After application children should be submitted to physical therapy and occupational therapy, focused on home therapy, and family involvement, increasing chances of therapeutic gain. Treatment with ABO is multidisciplinary and requires integrated approaches

Práticas de bloqueios neuromusculares para o tratamento da espasticidade no Brasil / Neuromuscular block practice in the treatment of spasticity in Brazil

A toxina botulínica do tipo A (TBA) é um dos tratamentos mais efetivos e seguros para espasticidade. Para a avaliação algumas escalas podem ser utilizadas como a Escala Modificada de Ashworth (MAS). O sucesso terapêutico na aplicação depende da correta identificação do problema biomecânico e aplicação no músculo acometido, o que pode ser feito por técnicas de palpação de superfície ou métodos auxiliares como eletromiografia. Objetivo: Avaliar os métodos de aplicações da TBA e outros tipos de bloqueios como fenol para tratamento da espasticidade realizados na prática de médicos de reabilitação infantil e de adultos. Métodos: Estudo exploratório, transversal, com amostra dimensionada pela conveniência em eventos científicos nas cidades Rio de Janeiro, São Paulo, Goiânia, Belo Horizonte, Curitiba e Ribeirão Preto com questionários a respeito do tratamento da espasticidade que continham questões de múltipla escolha sobre grupos de pacientes e músculos tratados. As respostas foram analisadas quanto à frequência para cada questão. Não foi foram realizados testes de associação de variáveis ou de hipótese. Resultados: Foram analisados 49 questionários. 47% aplicam TBA há menos de 5 anos. A técnica mais utilizada para localização de pontos de aplicação foi a palpação muscular (80%). Para quantificação dos ganhos funcionais 78% utilizam a escala MAS. 57% faz aplicação em adultos e crianças. A faixa etária de tratamento mais comum entre as crianças foi 5 a 10 anos (83%) com o grupo muscular mais aplicado o tríceps sural (73,8%). Em relação ao uso do fenol, 16 utilizam com uma frequência de 1 a 5 pacientes por mês. 45% dos aplicadores sempre utilizam fenol em conjunto com TBA. Conclusão: A TBA é largamente utilizada no tratamento de espasticidade, porém não existe uma padronização na forma de aplicação, método de avaliação ou sobre necessidade de outro agente combinado

Botulinum toxin type A (TBA) is one of the most effective and safe treatments for spasticity. To evaluate some scales can be used, as the Modified Ashworth Scale (MAS). Therapeutic success in the application depends on the correct identification of the problem and biomechanical application of the affected muscle, which can be done by surface palpation techniques or auxiliary methods such as electromyography. Objective: To evaluate the methods of TBA applications for the treatment of spasticity performed in the practice of medical rehabilitation for children and adults. Methods: exploratory, cross-sectional study with a convenience sample size recruited in scientific events in Rio de Janeiro, São Paulo, Goiânia, Belo Horizonte, Curitiba and Ribeirão Preto (Brazil). Questionnaires regarding the treatment of spasticity containing multiple choice questions about groups of patients and treated muscles were applied. Responses were analyzed for frequency for each answer. No association test of variables or hypothesis were used. Results: 49 questionnaires were analyzed. 47% apply TBA for less than five years. The most used technique for locating points of application was muscular palpation (80%). To quantify the functional gains, 78% reported the use the MAS scale. 57% applies in adults and children. The most common children age group treated was 5-10 years (83%) and the most treated muscle group was the triceps surae (73.8%). Regarding the use of phenol, 16 used with a frequency of 1 to 5 patients per month. 45% of applicators used phenol associeted with TBA. Conclusion: The TBA is widely used in the treatment of spasticity, however there is no standardization in the form of application, method of analyzing the treatment success or the necessity of a combined agent

Botulinum Toxin Type A Injection for Spastic Equinovarus Foot in Children with Spastic Cerebral Palsy: Effects on Gait and Foot Pressure Distribution

PURPOSE: To investigate the effect of intramuscular Botulinum toxin type A (BoNT-A) injection on gait and dynamic foot pressure distribution in children with spastic cerebral palsy (CP) with dynamic equinovarus foot. MATERIALS AND METHODS: Twenty-five legs of 25 children with CP were investigated in this study. BoNT-A was injected into the gastrocnemius (GCM) and tibialis posterior (TP) muscles under the guidance of ultrasonography. The effects of the toxin were clinically assessed using the modified Ashworth scale (MAS) and modified Tardieu scale (MTS), and a computerized gait analysis and dynamic foot pressure measurements using the F-scan system were also performed before injection and at 1 and 4 months after injection. RESULTS: Spasticity of the ankle plantar-flexor in both the MAS and MTS was significantly reduced at both 1 and 4 months after injection. On dynamic foot pressure measurements, the center of pressure index and coronal index, which represent the asymmetrical weight-bearing of the medial and lateral columns of the foot, significantly improved at both 1 and 4 months after injection. The dynamic foot pressure index, total contact area, contact length and hind foot contact width all increased at 1 month after injection, suggesting better heel contact. Ankle kinematic data were significantly improved at both 1 and 4 months after injection, and ankle power generation was significantly increased at 4 months after injection compared to baseline data. CONCLUSION: Using a computerized gait analysis and foot scan, this study revealed significant benefits of BoNT-A injection into the GCM and TP muscles for dynamic equinovarus foot in children with spastic CP.

Effects of botulinum toxin type A for spastic foot in post-stroke patients enrolled in a rehabilitation program / Efeitos da toxina botulinica tipo A no pe espastico em pacientes pos-acidente vascular encefalico inseridos em programa de reabilitacao

The objective of this study was to evaluate the effects of botulinum toxin type A (BTX-A) on spastic foot in stroke patients in a rehabilitation program. Method: Hemiparetic stroke patients (n=21) enrolled in a rehabilitation program were divided into two groups. The first group (n=11) received a total of 300UI BTX-A, and the second group (n=10) received 100 UI BTX-A. All patients were assessed at baseline and 2, 4, 8 and 12 weeks after injection for Modified Ashworth Score, time walking 10 meters, and the Functional Independence Measure (mFIM) motor score. Results: The higher-dose group exhibited a significant improvement in spasticity, and both groups showed an improvement in time walking 10 meters and mFIM, with no significant differences between them. Conclusions: Our findings suggest that gains in gait velocity and functional independence were not correlated to BTX-A dose. .

O objetivo deste estudo foi avaliar os efeitos da toxina botulínica tipo A (TXB-A) sobre a espasticidade de membro inferior em pacientes pós-AVE em reabilitação. Método: 21 pacientes hemiparéticos foram divididos em dois grupos que receberam doses de TXB-A de 300UI (Grupo 1) e 100UI (Grupo 2) e foram avaliados antes da injeção e 2, 4, 8 e 12 semanas após, quanto à escala de Ashworth modificada, tempo para andar 10 metros e escore motor da Medida de Independência Funcional (MIFm). Resultados: O grupo que utilizou dose mais alta teve melhora significativa da espasticidade. Ambos os grupos tiveram melhora do tempo para andar 10 metros e da MIFm sem diferença significativa entre eles. Conclusões: A melhora da velocidade de marcha e da independência funcional não foram correlacionadas com a dose de TXB-A na amostra analisada. .

The Effect of Obturator Nerve Block on Hip Lateralization in Low Functioning Children with Spastic Cerebral Palsy

PURPOSE: Hip adductor spasticity has a great impact on developing hip displacement in children with cerebral palsy (CP). Obturator nerve (ON) block is less invasive intervention rather than soft tissue surgery for reduction of hip adductor spasticity. The aim of this study is to investigate the effect of ON block on hip lateralization in low functioning children with spastic CP. MATERIALS AND METHODS: The study was performed by retrospective investigation of the clinical and radiographic follow-up data of low functioning children [gross motor function classification system (GMFCS) level III to V] with spastic cerebral palsy whose hip was subluxated. Migration percentage (MP) was measured on hip radiographs and its annual change was calculated. In intervention group, ON block was done with 50% ethyl alcohol under the guidance of electrical stimulation. RESULTS: The data of 49 legs of 25 children for intervention group and the data of 41 legs of 23 children for nonintervention group were collected. In intervention group, the MP were significantly reduced at 1st follow-up and the MPs at 2nd and last follow-up did not show significant differences from initial MP. Whereas in nonintervention group, the MPs at 1st, 2nd and last follow-up were all significantly increased compared to initial MPs. CONCLUSION: ON block with ethyl alcohol is useful as an early effective procedure against progressive hip displacement in these children with spastic CP.

Effects of early spasticity treatment on children with hemiplegic cerebral palsy: a preliminary study / Efeitos do tratamento precoce da espasticidade em criancas com paralisia cerebral hemiplegica: um estudo preliminar

Objective To compare motor and functional performance of two groups of children with hemiplegic cerebral palsy (HCP). Only the study group (SG) received early treatment of spasticity with botulinum neurotoxin type A (BXT-A). Methods Gross Motor Function Measure (GMFM), functional performance (Pediatric Evaluation of Disability Inventory - PEDI), range of movement, gait pattern (Physician Rating Scale - PRS) and the speed of hand movements were considered. Results The SG, composed of 11 HCP (45.64±6.3 months), was assessed in relation to the comparison group, composed of 13 HCP (45.92±6.4 months). SG showed higher scores in four of the five GMFM dimensions, which included scores that were statistically significant for dimension B, and higher scores in five of the six areas evaluated in the PEDI. Active wrist extension, the speed of hand movements and PRS score were higher in the SG. Conclusion Children who received early BXT-A treatment for spasticity showed higher scores in motor and functional performance. .

Objetivo Comparar a performance motora e funcional de dois grupos de crianças com paralisia cerebral hemiplégica (PCH). Apenas o grupo de estudo (GE) recebeu tratamento precoce da espasticidade com toxina botulínica do tipo A (BXT-A). Métodos Foram considerados a Função Motora Grossa (Gross Motor Function Measure - GMFM), performance funcional (Pediatric Evaluation of Disability Inventory - PEDI), amplitude de movimento, padrão da marcha (Physician Rating Scale - PRS) e a velocidade de movimento das mãos. Resultados O GE, composto de 11 PCH (45,64±6,3 meses), foi analisado em relação ao grupo de comparação, composto por 13 PCH (45,92±6,4 meses). O GE mostrou maiores escores em quatro das cinco dimensões da GMFM, sendo a diferença estatisticamente significativa na dimensão B, e melhores escores em cinco das seis áreas avaliadas na PEDI. A extensão ativa do punho, a velocidade de movimento das mãos e o escore na PRS foram maiores no GE. Conclusão As crianças que receberam tratamento precoce da espasticidade com BXT-A mostraram melhores escores motores e funcionais. .

Avaliação de um programa de aplicação de toxina botulínica tipo A em crianças do Vale do Jequitinhonha com paralisia cerebral / Evaluation of a program of application of the botulinum toxin type A in children with cerebral palsy in Vale do Jequitinhonha

O objetivo do estudo foi avaliar os efeitos de um programa público de aplicação de toxina botulínica tipo A (TBA) na espasticidade muscular, amplitude de movimento, qualidade da marcha, independência funcional e qualidade de vida de crianças e adolescentes com paralisia cerebral (PC). Foi realizado um estudo quase experimental que avaliou os efeitos do emprego da TBA, aplicada três vezes, com intervalos de três meses, em 14 crianças com PC. Estas foram avaliadas através da Escala Modificada de Ashworth, Goniometria Manual, Physician Rating Scale, Inventário de Avaliação Pediátrica de Incapacidade (PEDI) e Questionário do Cuidador da Criança (QCC). Foi observada redução da espasticidade, aumento da amplitude de movimento, melhora das habilidades funcionais de autocuidado e mobilidade do PEDI e das áreas de cuidado pessoal, conforto e interação/comunicação do QCC. O programa de aplicação de TBA em crianças e adolescentes com PC, realizado em um núcleo de reabilitação público do Vale do Jequitinhonha, foi efetivo para a população beneficiada.

The scope of this study was to evaluate the effects of a public program of application of botulinum toxin type A (BoNT-A) in terms of muscle spasticity, range of motion, quality of gait, functional independence and quality of life of children and adolescents with cerebral palsy (CP). A quasi-experimental study was conducted with 14 children with CP to evaluate the effects of three applications of BoNT-A, at intervals of three months. Children were assessed using the Modified Ashworth Rating Scale, the Manual Goniometer, the Physician Rating Scale, the Pediatric Evaluation of Disability Inventory (PEDI) and the Caregiver Questionnaire (CQ). There was a reduction of spasticity, an increase in the range of motion, an improvement of the functional abilities of self-care and mobility of PEDI and the areas of personal care, comfort and interaction/communication with the CQ. The BoNT-A application program for children and adolescents with CP, conducted in a public rehabilitation service in the Jequitinhonha Valley, was effective for the targeted population.

Consenso chileno sobre Abobotulinumtoxina A (Dysport®): indicaciones, técnicas / Chilean Consensus for on abobotulinumtoxin A (Dysport®): indications, techniques

Botulinum neurotoxin is widely used to treat a variety of movement disorders, especially dystonia and spasticity. There botúlica different preparations of botulinum type A, that because biologics are difficult to compare. AbobotulinumtoxinA (Dysport ®) has recently been introduced in Chile for treating dystonia and spasticity. The aim of this paper is to provide a review by experts on the existing evidence and propose a practical guide to the use of the drug. Methods and Results: A group of Chilean experts met on 6 and 7 July 2012 to review AbobotulinumtoxinA doses along with injection protocols for dystonia, spasticity and other movement disorders. We worked in two groups, one composed of neurologists who reviewed the on dystonias and other by physiatrists who reviewed the indications for spasticity. Conclusions: Finally, there is a proposal in terms of dose, injection points and recommendations for dystonia and spasticity...

La neurotoxina botulínica es ampliamente usada para tratar una variedad de trastornos del movimiento, especialmente la distonía y la espasticidad. Existen diferentes preparados de toxina botúlica tipo A, que por ser productos biológicos resultan difícilmente comparables. La abobotulinumtoxinA (Dysport®) ha sido introducida en Chile recientemente para el tratamiento de la distonía y la espasticidad. El objetivo de este trabajo es entregar una revisión hecha por expertos sobre la evidencia existente y plantear una guía práctica para el uso del medicamento. Métodos y Resultados: Un grupo de expertos chilenos se reunieron el 6 y 7 julio de 2012 para revisar las dosis abobotulinumtoxin A, junto con los protocolos de inyección para la distonía, la espasticidad y otros trastornos del movimiento. Se trabajó en dos grupos, uno compuesto por neurólogos que revisaron las indicaciones en distonías y otro por fisiatras que revisaron las indicaciones para espasticidad. Conclusiones: Finalmente, se realiza una propuesta en cuanto a dosis, puntos a inyectar y recomendaciones para la distonia y espasticidad...

literature review on the efficacy and safety of botulinum toxin: an injection in post-stroke spasticity

A variety of techniques for the management of spasticity have been suggested, including positioning, cryotherapy, splinting and casting, biofeedback, electrical stimulation, and medical management by pharmacological agents, Botulinum toxin A [BTA] is now the pharmacological treatment of choice in focal spasticity. BTA by blocking acetylcholine release at neuromuscular junctions accounts for its therapeutic action to relieve spasticity. A computerized search of Pub Med was carried out to find the latest result about efficacy of BTA in management of post stroke spasticity. Among 84 articles were found, frothy of them included in this review and divided to lower and upper extremity. BTA is a treatment choice in reducing tone and managing post stroke spasticity

[ effect of foot serial casting along with botulinum toxin type-A injection on spasticity in children with cerebral palsy]

The goal of this study was to determine the effect of foot serial casting along with botulinum toxin type-A injection on spasticity in children with cerebral palsy. This study was a randomized clinical trial performed as a pre-post, double blind study. It was performed on 25 children with hemiplegia and diplegia [2-8 years] in Tehran city, who were referred to valiasr rehabilitation foundation. Participants were chosen by simple randomized sampling and were matched for age, Gross Motor Function Classification System [GMFCS] and type. They were randomly divided into two groups. The first group [n=13] underwent BTX-A injection alone and the second group [n=12] had BTX-A injection and foot serial casting after the injection. Clinical assessments were done using the GMFCS and Modified Ashworth Scale before and at 1, 3, 6 and 12 months after the interventions. Data were analyzed by Wilcoxon signed rank test and mann-whitney U. Comparison of two groups in regard to the right and left knee spasticity at, 1, 3, 6 and 12 months after injection showed no significant difference in comparison to those before interventions. Furthermore, comparison of right and left ankle spasticity before injection with that at 1, and 3 months follow ups did not show statistically significant difference, but significant differences were found when compared with 6 and 12 month follow-ups [P<0.05] It seems, one of the proper approaches to reduce spasticity in children with cerebral palsy is foot serial casting along with botulinum toxin type-A injection and it can decrease the muscle tone when applied more than six months

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS): typical clinical and neuroimaging features in a Brazilian family / Ataxia espástica autossômica recessiva de Charlevoix-Saguenay (ARSACS): aspectos clínicos e de neuroimagem típicos em uma família brasileira

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurodegenerative disorder characterized by late-infantile onset spastic ataxia and other neurological features. ARSACS has a high prevalence in northeastern Quebec, Canada. Several ARSACS cases have been reported outside Canada in recent decades. This is the first report of typical clinical and neuroimaging features in a Brazilian family with probable diagnosis of ARSACS.

A ataxia espástica autossômica recessiva de Charlevoix-Saguenay (ARSACS) é doença degenerativa do sistema nervoso, caracterizada por ataxia associada a espasticidade, entre outras manifestações neurológicas, de início na infância. A doença tem alta prevalência na região de Quebec, no Canadá. Muitos relatos de ARSACS têm sido descritos fora do Canadá nas últimas décadas. Nesse artigo, relatamos a primeira descrição dos aspectos clínicos e de neuroimagem típicos em uma família brasileira com provável diagnóstico de ARSACS.

Tratamento farmacológico da espasticidade na paralisia cerebral / Pharmacological spasticity treatment on cerebral palsy

A espasticidade é uma desordem motora caracterizada por aumento dos reflexos de estiramento tônicos (tônus muscular), velocidade dependente, resultado da hiper excitabilidade deste reflexo, como um componente da Síndrome do Neurônio Superior, extremamente comum a várias condições neuropatológicas. Neste artigo faremos uma revisão da fisiopatologia da espasticidade e dos principais fármacos utilizados no seu tratamento.

Spasticity is a movement disorder characterized by a velocity dependant tonic stretch reflex (muscle tone) increase as a result of the reflex?s hyper-excitability and as a component of the Upper Neuron Syndrome, extremely common to in many neuropathological conditions. In this article we will review the physiopathology of espasticidade and the main drugs used in its treatment.

Bloqueios químicos para o tratamento da espasticidade na paralisia cerebral / Chemical blockage for cerebral palsy spasticity treatment

Os bloqueios químicos são considerados armas importantes no moderno tratamento da espasticidade tanto em adultos como em crianças. Eles podem ser realizados com fenol, com toxina botulínica do tipo A ou ainda com ambos, os chamados bloqueios mistos. Neste artigo discutiremos em detalhes os diferentes tipos de bloqueios químicos utilizados para o tratamento da espasticidade.

The chemical blockades are considered important weapons in the modern treatment of the espasticidade even in adults as in children. They can be carried through with phenol, botulinum toxin of the type A or both (called mixing blockades). In this article we will review in details the different types and uses of chemical blockades for the treatment of the spasticity.

Multilevel botulinum toxin type a as a treatment for spasticity in children with cerebral palsy: a retrospective study

INTRODUCTION: Cerebral palsy is the most common cause of physical disability in children. Spasticity is a disabling clinical symptom that is prevalent among patients suffering from cerebral palsy. The treatment of spasticity with botulinum toxin type A (BTX-A) is a well-established option in the interdisciplinary management of spasticity, providing focal reductions in muscle tone in cerebral palsy patients. OBJECTIVE: The aim of this retrospective study was to describe the effect of multilevel BTX-A injections in the lower extremities, focusing mainly on gross motor function and functional status in cerebral palsy patients. METHODS: Data from 71 cerebral palsy patients (64 percent male, 36 percent female, mean age 6.7 ±3.2 years) were analyzed retrospectively. We used the Ashworth and Tardieu scales to evaluate the degree of spasticity. Motor function was measured by the Gross Motor Function Measure (GMFM-88), and functional status was classified by the Gross Motor Function Classification System (GMFCS I-V). Multilevel BTX-A injections were applied after sedation and with electrostimulation guidance. The evaluations were repeated every three months, and the patients were followed for six months. RESULTS: We found that the Ashworth and Tardieu scores decreased significantly at the three-month evaluation (p<0.05) but not at the six-month evaluation (p>0.05). Although the improvement in spasticity was not maintained at the six-month evaluation, GMFM-88 scores increased significantly at the three- and six-month assessments. GMFSC levels showed no change in the three- and six-month assessments. CONCLUSION: We believe that a single multilevel BTX-A injection reduces spasticity and improves motor function in children with cerebral palsy.

Botulinum toxin type A in the treatment of lower-limb spasticity in children with cerebral palsy / Toxina botulínica tipo A como tratamento para espasticidade de membros inferiores em crianças com paralisia cerebral

We evaluated the safety and effectiveness of botulinum toxin A (BoNT/A) in the treatment of spasticity in 20 children with spastic diplegic cerebral palsy (CP). All the patients received injections in the gastrocnemius and soleus, and 15 received injections in the adductors. The total dose varied from 70 to 140 U (99.75±16.26 U), or 7.45±2.06 U/kg per patient. The treatment improved the patients' walking and gait pattern significantly. There was also a significant alteration in the heel-ground distance and increased motion of the ankle joint. These structural changes in the feet were sustained until the end of the follow-up, although the same was not observed for the functional parameters. Three patients complained of weakness in the lower limbs. In conclusion, BoNT/A is safe and effective when used in a single session of injections and produces a sustained structural modification of the lower limbs. However, functional changes are temporary and are only observed during the peak effect of the drug.

Para avaliação da segurança e eficácia do tratamento com toxina botulínica A (TB-A) na espasticidade na paralisia cerebral (PC), foram selecionadas 20 crianças com a forma diplegia espástica. Todos os pacientes receberam injeções nos gastrocnêmios e sóleos, 15 receberam doses nos adutores da coxa. A dose total variou de 70 a 140 Us (99,75±16,26 U), 7,45±2,06 U/Kg por paciente. O tratamento com a TB-A melhorou significativamente a deambulação e o padrão de marcha. Houve também significativa alteração da distância tornozelo-solo e aumento da amplitude de movimento da articulação do tornozelo. Essas mudanças estruturais dos pés se mantiveram até o final do acompanhamento. O mesmo não foi observado com parâmetros funcionais. Três pacientes apresentaram fraqueza em membros inferiores. Conclui-se que a TB-A, em uma única aplicação, é segura e eficaz. Há modificação sustentada da estrutura motora dos membros inferiores, porém mudanças funcionais são temporárias, durante o pico de ação do medicamento.