Combined translational and rotational perturbations of standing balance reveal contributions of reduced reciprocal inhibition to balance impairments in children with cerebral palsy.

Balance impairments are common in cerebral palsy. When balance is perturbed by backward support surface translations, children with cerebral palsy have increased co-activation of the plantar flexors and tibialis anterior muscle as compared to typically developing children. However, it is unclear whether increased muscle co-activation is a compensation strategy to improve balance control or is a consequence of reduced reciprocal inhibition. During translational perturbations, increased joint stiffness due to co-activation might aid balance control by resisting movement of the body with respect to the feet. In contrast, during rotational perturbations, increased joint stiffness will hinder balance control as it couples body to platform rotation. Therefore, we expect increased muscle co-activation in response to rotational perturbations if co-activation is caused by reduced reciprocal inhibition but not if it is merely a compensation strategy. We perturbed standing balance by combined backward translational and toe-up rotational perturbations in 20 children with cerebral palsy and 20 typically developing children. Perturbations induced forward followed by backward movement of the center of mass. We evaluated reactive muscle activity and the relation between center of mass movement and reactive muscle activity using a linear feedback model based on center of mass kinematics. In typically developing children, perturbations induced plantar flexor balance correcting muscle activity followed by tibialis anterior balance correcting muscle activity, which was driven by center of mass movement. In children with cerebral palsy, the switch from plantar flexor to tibialis anterior activity was less pronounced than in typically developing children due to increased muscle co-activation of the plantar flexors and tibialis anterior throughout the response. Our results thus suggest that a reduction in reciprocal inhibition causes muscle co-activation in reactive standing balance in children with cerebral palsy.

Histological analysis of the gastrocnemius muscle in preschool and school age children with cerebral palsy compared with age-matched typically developing children.

Inconsistent alterations in skeletal muscle histology have been reported in adolescents with cerebral palsy (CP) and whether alterations are present in young children and differ from older children is not yet known. This study aimed to define histological alterations in the medial gastrocnemius (MG) of ambulant CP (gross-motor classification system, GMFCS I-III) stratified in two age groups (preschool children, PS: 2-5 and school age children, SA: 6-9-yr old) compared with age-matched typically developing (TD) children. We hypothesized that alterations in muscle microscopic properties are already present in PS-CP and are GMFCS level specific. Ultrasound guided percutaneous microbiopsies were collected in 46 CP (24-PS) and 45 TD (13-PS) children. Sections were stained to determine fiber cross-sectional area (fCSA) and proportion, capillary, and satellite cell amount. Average absolute and normalized fCSA were similar in CP and TD, but a greater percentage of smaller fibers was found in CP. Coefficient of variation (CV) was significantly larger in PS-CP-GMFCS I-II and for type I fiber. In SA-CP, all fiber types contributed to the higher CV. Type IIx proportion was higher and type I was lower in PS-CP-GMFCS-III and for all SA-CP. Reduced capillary-to-fiber ratio was present in PS-CP-GMFCS II-III and in all SA-CP. Capillary fiber density was lower in SA-CP. Capillary domain was enhanced in all CP, but capillary spatial distribution was maintained as was satellite cell content. We concluded that MG histological alterations are already present in very young CP but are only partly specific for GMFCS level and age.NEW & NOTEWORTHY Inconsistent histological alterations have been reported in children with cerebral palsy (CP) but whether they are present in very young and ambulant CP children and differ from those reported in old CP children is not known. This study highlighted for the first time that enhanced muscle fiber size variability and loss of capillaries are already present in very young CP children, even in the most ambulant ones, and these alterations seem to extend with age.

Muscle morphology and architecture of the medial gastrocnemius between typically developing children with different ancestral background.

Muscle ultrasonography is frequently used to improve the understanding of musculoskeletal impairments in children with spastic cerebral palsy (SCP). So far, most studies on muscle morphology and architecture have included typically developing children and children with SCP with similar ancestry, being mainly Caucasian. Less is known about differences in muscle morphology between children with different ancestral backgrounds. Therefore, the aim of this study was to compare muscle morphology and architecture of the medial gastrocnemius in typically developing children with African, South Asian and Southeast Asian descent from Suriname. This explorative cohort study identified children as Maroon (Ghana, African descent), Hindustani (India, South Asian) or Javanese (Indonesia, Southeast Asian), aged 5-10 years. Using 3D freehand ultrasound with the subject prone, the following medial gastrocnemius parameters were defined: muscle tendon unit (MTU) length, muscle belly length, tendon length, muscle volume, muscle thickness, anatomical cross-sectional area (ACSA), fascicle length, pennation angle, and physiological cross-sectional area (PCSA). In addition, differences between ancestral groups were assessed for the length of the MTU, muscle, tendon and fascicles in two passive stretch conditions corresponding to an externally applied joint torque of 1Nm and 4Nm. One-way ANOVA with post hoc t-tests were used to investigate differences between the ancestral groups. In total, 100 Hindustani (n = 34), Javanese (n = 34) and Maroon (n = 32) children were included. For statistical analyses, we matched the children by age, which resulted in groups of 25 children per ancestral group (n = 75). There were no differences found in MTU length, muscle belly length, ACSA, PCSA and muscle volume. Tendon length, fascicle length and pennation angle were different between ancestral groups. Compared to Javanese children, tendon length was longer (p = 0.001) and pennation angle (p = 0.001) was larger in Maroon children and fascicle length was shorter in both Maroon and Hindustani children (p < 0.001). While there was a difference found in MTU length at different conditions of passive stretch between ancestries, no differences were found in the muscle, tendon and fascicles. This is the first study that investigated macroscopic morphological and architectural parameters for the medial gastrocnemius in one extended cohort of typically developing children, stratified in three ancestral subgroups. The current results imply that ancestry-specific reference data for children are needed, especially for tendon length, fascicle length and pennation angle when investigating altered muscle morphology in neurological or neuromuscular pathologies, such as SCP. Future studies should report the ancestral background when describing muscle morphology and architecture of children and ancestral specifications should be included in normative databases.

Longitudinal trajectory of medial gastrocnemius muscle growth in the first years of life.

AIM: To define the longitudinal trajectory of gastrocnemius muscle growth in 6- to 36-month-old children with and without spastic cerebral palsy (SCP) and to compare trajectories by levels of gross motor function (Gross Motor Function Classification System, GMFCS) and presumed brain-lesion timing. METHOD: Twenty typically developing children and 24 children with SCP (GMFCS levels I-II/III-IV = 15/9), were included (28/16 females/males; mean age at first scan 15.4 months [standard deviation 4.93, range 6.24-23.8]). Three-dimensional freehand ultrasound was used to repeatedly assess muscle volume, length, and cross-sectional area (CSA), resulting in 138 assessments (mean interval 7.9 months). Brain lesion timing was evaluated with magnetic resonance imaging classification. Linear mixed-effects models defined growth rates, adjusted for GMFCS levels and presumed brain-lesion timing. RESULTS: At age 12 months, children with SCP showed smaller morphological muscle size than typically developing children (5.8 mL vs 9.8 mL, p < 0.001), while subsequently no differences in muscle growth were found between children with and without SCP (muscle volume: 0.65 mL/month vs 0.74 mL/month). However, muscle volume and CSA growth rates were lower in children classified in GMFCS levels III and IV than typically developing children and those classified in GMFCS levels I and II, with differences ranging from -56% to -70% (p < 0.001). INTERPRETATION: Muscle growth is already hampered during infancy in SCP. Muscle size growth further reduces with decreasing functional levels, independently from the brain lesion. Early monitoring of muscle growth combined with early intervention is needed.

A comprehensive normative reference database of muscle morphology in typically developing children aged 3-18 years-a cross-sectional ultrasound study.

During childhood, muscle growth is stimulated by a gradual increase in bone length and body mass, as well as by other factors, such as physical activity, nutrition, metabolic, hormonal, and genetic factors. Muscle characteristics, such as muscle volume, anatomical cross-sectional area, and muscle belly length, need to continuously adapt to meet the daily functional demands. Pediatric neurological and neuromuscular disorders, like cerebral palsy and Duchenne muscular dystrophy, are characterized by impaired muscle growth, which requires treatment and close follow-up. Nowadays ultrasonography is a commonly used technique to evaluate muscle morphology in both pediatric pathologies and typically developing children, as it is a quick, easy applicable, and painless method. However, large normative datasets including different muscles and a large age range are lacking, making it challenging to monitor muscle over time and estimate the level of pathology. Moreover, in order to compare individuals with different body sizes as a result of age differences or pathology, muscle morphology is often normalized to body size. Yet, the usefulness and practicality of different normalization techniques are still unknown, and clear recommendations for normalization are lacking. In this cross-sectional cohort study, muscle morphology of four lower limb muscles (medial gastrocnemius, tibialis anterior, the distal compartment of the semitendinosus, rectus femoris) was assessed by 3D-freehand ultrasound in 118 typically developing children (mean age 10.35 ± 4.49 years) between 3 and 18 years of age. The development of muscle morphology was studied over the full age range, as well as separately for the pre-pubertal (3-10 years) and pubertal (11-18 years) cohorts. The assumptions of a simple linear regression were checked. If these assumptions were fulfilled, the cross-sectional growth curves were described by a simple linear regression equation. Additional ANCOVA analyses were performed to evaluate muscle- or gender-specific differences in muscle development. Furthermore, different scaling methods, to normalize muscle morphology parameters, were explored. The most appropriate scaling method was selected based on the smallest slope of the morphology parameter with respect to age, with a non-significant correlation coefficient. Additionally, correlation coefficients were compared by a Steiger's Z-test to identify the most efficient scaling technique. The current results revealed that it is valid to describe muscle volume (with exception of the rectus femoris muscle) and muscle belly length alterations over age by a simple linear regression equation till the age of 11 years. Normalizing muscle morphology data by allometric scaling was found to be most useful for comparing muscle volumes of different pediatric populations. For muscle lengths, normalization can be achieved by either allometric and ratio scaling. This study provides a unique normative database of four lower limb muscles in typically developing children between the age of 3 and 18 years. These data can be used as a reference database for pediatric populations and may also serve as a reference frame to better understand both physiological and pathological muscle development.

Reliability of 3D freehand ultrasound to assess lower limb muscles in children with spastic cerebral palsy and typical development.

This study investigated the reliability of 3-dimensional freehand ultrasound (3DfUS) to quantify the size (muscle volume [MV] and anatomical cross-sectional area [aCSA]), length (muscle length [ML], tendon length [TL], and muscle tendon unit length [MTUL]), and echo-intensity (EI, whole muscle and 50% aCSA), of lower limb muscles in children with spastic cerebral palsy (SCP) and typical development (TD). In total, 13 children with SCP (median age 14.3 (7.3) years) and 13 TD children (median age 11.1 (1.7) years) participated. 3DfUS scans of rectus femoris, semitendinosus, medial gastrocnemius, and tibialis anterior were performed by two raters in two sessions. The intra- and inter-rater and intra- and inter-session reliability were defined with relative and absolute reliability measures, that is, intra-class correlation coefficients (ICCs) and absolute and relative standard error of measurement (SEM and SEM%), respectively. Over all conditions, ICCs for muscle size measures ranged from 0.818 to 0.999 with SEM%s of 12.6%-1.6%. For EI measures, ICCs varied from 0.233 to 0.967 with SEM%s of 15.6%-1.7%. Length measure ICCs ranged from 0.642 to 0.999 with SEM%s of 16.0%-0.5%. In general, reliability did not differ between the TD and SCP cohort but the influence of different muscles, raters, and sessions was not constant for all 3DfUS parameters. Muscle length and muscle tendon unit length were the most reliable length parameters in all conditions. MV and aCSA showed comparable SEM%s over all muscles, where tibialis anterior MV was most reliable. EI had low-relative reliability, but absolute reliability was better, with better reliability for the distal muscles in comparison to the proximal muscles. Combining these results with earlier studies describing muscle morphology assessed in children with SCP, 3DfUS seems sufficiently reliable to determine differences between cohorts and functional levels. The applicability on an individual level, for longitudinal follow-up and after interventions is dependent on the investigated muscle and parameter. Moreover, the semitendinosus, the acquisition, and processing of multiple sweeps, and the definition of EI and TL require further investigation. In general, it is recommended, especially for longitudinal follow-up studies, to keep the rater the same, while standardizing acquisition settings and positioning of the subject.

Muscle synergy structure and gait patterns in children with spastic cerebral palsy.

AIM: To determine if muscle synergy structure (activations and weights) differs between gait patterns in children with spastic cerebral palsy (CP). METHOD: In this cross-sectional study, we classified 188 children with unilateral (n=82) or bilateral (n=106) spastic CP (mean age: 9y 5mo, SD: 4y 3mo, range: 3y 9mo-17y 7mo; 75 females; Gross Motor Function Classification System [GMFCS] level I: 106, GMFCS level II: 55, GMFCS level III: 27) into a minor deviations (n=34), drop foot (n=16), genu recurvatum (n=26), apparent equinus (n=53), crouch (n=39), and jump gait pattern (n=20). Surface electromyography recordings from eight lower limb muscles of the most affected side were used to calculate synergies with weighted non-negative matrix factorization. We compared synergy activations and weights between the patterns. RESULTS: Synergy structure was similar between gait patterns, although weights differed in the more impaired children (crouch and jump gait) when compared to the other patterns. Variability in synergy structure between participants was high. INTERPRETATION: The similarity in synergy structure between gait patterns suggests a generic motor control strategy to compensate for the brain lesion. However, the differences in weights and high variability between participants indicate that this generic motor control strategy might be individualized and dependent on impairment level.

Interventions and lower-limb macroscopic muscle morphology in children with spastic cerebral palsy: a scoping review.

AIM: To identify and map studies that have assessed the effect of interventions on lower-limb macroscopic muscle-tendon morphology in children with spastic cerebral palsy (CP). METHOD: We conducted a literature search of studies that included pre- and post-treatment measurements of lower-limb macroscopic muscle-tendon morphology in children with spastic CP. Study quality was evaluated and significant intervention effects and effect sizes were extracted. RESULTS: Twenty-eight articles were identified. They covered seven different interventions including stretching, botulinum neurotoxin A (BoNT-A), strengthening, electrical stimulation, whole-body vibration, balance training, and orthopaedic surgery. Study quality ranged from poor (14 out of 28 studies) to good (2 out of 28). Study samples were small (n=4-32) and studies were variable regarding which muscles and macroscopic morphological parameters were assessed. Inconsistent effects after intervention (thickness and cross-sectional area for strengthening, volume for BoNT-A), no effect (belly length for stretching), and small effect sizes were reported. INTERPRETATION: Intervention studies reporting macroscopic muscle-tendon remodelling after interventions are limited and heterogeneous, making it difficult to generalize results. Studies that include control groups and standardized assessment protocols are needed to improve study quality and data synthesis. Lack or inconclusive effects at the macroscopic level could indicate that the effects of interventions should also be evaluated at the microscopic level. WHAT THIS PAPER ADDS: Muscle-targeted interventions to remodel muscle morphology are not well understood. Studies reporting macroscopic muscle remodelling following interventions are limited and heterogeneous. Passive stretching may preserve but does not increase muscle length. The effects of isolated botulinum neurotoxin A injections on muscle volume are inconsistent. Isolated strengthening shows no consistent increase in muscle volume or thickness.

Proximal femoral derotation osteotomy in children with CP : long term outcome and the role of age at time of surgery.

The femoral derotation osteotomy (FDO) is seen as the golden standard treatment in children with cerebral palsy and internal rotated gait. This study provides quantitative evidence in support of the beneficial effect of FDO after long term follow up. Retrospective clinical and kinematic evaluation of 31 CP patients (55 operated limbs) pre-, 1 and 3 years postoperatively after proximal FDO was conducted for a minimal follow-up of 3 years. This group con- sisted of 20 men and 11 women, aged 10.68±3.31 years at the time of surgery. Minimum follow up was 3 years (3.16±0.53 years), with 22 patients (38 operated limbs) having an additional follow up at 5 years (5.02±0.49 years). Age at FU3 and FU5 was 14.06±3.52 years and 15.39±3.08 years respectively. A set of clinical and kinematic parameters were ana- lyzed and showed a significant correction of mean hip rotation and femoral anteversion after FDO. Further plotting of individual data comparing 3 or 5 year postoperative values to 1 year postoperative values showed no further significant changes, indicating sustained correction of internally rotted gait until end of our follow up. Plotting mean hip rotation in stance as well as kinematic knee parameters according to age grouped cohorts could not show age at time of surgery to be a significant factor in recurrence of internally rotated gait or preoperative disturbances of knee motion in the sagittal plane. This study provides quantitative evidence on the beneficial effect of FDO, a surgical technique to improve internally rotated gait in cerebral palsy patients with spastic diplegia. Pre- and postoperative clinical and kinematic para- meters are compared and results are discussed. Minimum follow up was 3 years with a mean follow up of 4.65±0.83 years. The effect of age at time of surgery on recurrence and kinematic parameters were studied.

Tone Reduction and Physical Therapy: Strengthening Partners in Treatment of Children with Spastic Cerebral Palsy.

The aim of this paper is to provide a clinically applicable overview of different tone reducing modalities and how these can interact with or augment concurrent physical therapy (PT). Botulinum toxin (BoNT), oral tone-regulating medication, intrathecal baclofen (ITB), and selective dorsal rhizotomy are discussed within a physiotherapeutic context and in view of current scientific evidence. We propose clinical reasoning strategies to identify treatment goals as well as the appropriate and corresponding treatment interventions. Instrumented measurement of spasticity, standardized clinical assessment, and 3D clinical motion analysis are scientifically sound tools to help select the appropriate treatment and, when needed, to selectively target or spare individual muscles. In addition, particular attention is given to strength training as a necessary tool to tackle muscle weakness associated with specific modalities of tone reduction. More research is needed to methodologically assess the long-term effectiveness of such individualized tone treatment, optimize parameters such as medication dosage, and gain more insight into the kind of PT techniques that are essential in conjunction with tone reduction.

Role of femoral derotation on gait after selective dorsal rhizotomy in children with spastic cerebral palsy.

AIM: To evaluate the long-term outcome of selective dorsal rhizotomy (SDR) on gait and the influence of previous femoral derotation osteotomy (FDO). METHOD: In a retrospective cohort study of 29 children (16 females, 13 males) with spastic diplegic cerebral palsy, 14 children received FDO before SDR, whereas 15 children with moderate or near-normal internal femoral rotation during gait received only SDR. Three-dimensional gait data were obtained pre-FDO, pre-SDR, 1 year post-SDR, and 3 to 5 years post-SDR, to study the Gait Profile Score (GPS), pelvic tilt, and knee and hip kinematics. A mixed analysis of variance with the repeated measure 'time' was performed between different time points for each group. RESULTS: Children who first underwent FDO and then SDR started with a more complex gait pathology but showed fewer gait deviations 3 to 5 years post-SDR, compared to children who only underwent SDR. This was reflected by a lower GPS and pelvic tilt, as well as less knee flexion in stance. INTERPRETATION: The effect of SDR on gait is only significant in the mid- to long-term if the bony lever arms are also corrected. Thus, the clinical outcome after SDR is dependent on good proximal alignment. WHAT THIS PAPER ADDS: Pelvic tilt remains stable after femoral derotation osteotomy (FDO)+selective dorsal rhizotomy (SDR). But pelvic tilt deteriorates after SDR only. Hip and knee extension is better after FDO+SDR than after SDR only. Spasticity reduction (by SDR) combined with bony lever arm correction (by FDO) improves gait.

PAPEL DE LA DESROTACION FEMORAL EN LA MARCHA DESPUES DE UNA RIZOTOMIA SELECTIVE POSTERIOR EN NIÑOS CON PARÁLISIS CEREBRAL: OBJETIVO: Evaluar el resultado a largo plazo de la rizotomía selective posterior (RSP) sobre la marcha y la influencia de la osteotomía femoral desrotadora (OFD) previa. METODO: Estudio de cohorte retrospectivo de 29 niños (13 mujeres, 16 varones) con parálisis cerebral subtipo diplejia espástica, 14 niños recibieron OFD antes de la RSP, mientras que 15 niños con rotación femoral interna moderada o casi normal durante la marcha recibieron solo RSP. Se obtuvieron datos tridimensionales de la marcha pre-OFD, pre-RSP, 1 año post-RSP y 3 a 5 años post-RSP, para estudiar la puntuación del perfil de la marcha (Gait Profile Score. GPS, sigla en inglés), inclinación pélvica y cinemática de rodilla y cadera. Se realizó un análisis mixto de varianza con la medida repetida "tiempo" entre diferentes puntos de tiempo para cada grupo. RESULTADOS: Los niños que primero se sometieron a OFD y luego RSP comenzaron con una patología de la marcha más compleja, pero mostraron menos desviaciones de la marcha de 3 a 5 años después de la RSP, en comparación con los niños que solo se sometieron a RSP. Esto se reflejó por una menor GPS e inclinación pélvica, así como una menor flexión de la rodilla durante el apoyo. INTERPRETACIÓN: El efecto de la RSP en la marcha solo es significativo a medio o largo plazo si los brazos de palanca óseos también se corrigen. Por lo tanto, el resultado clínico después de la RSP depende de una buena alineación proximal.

PAPEL DA DERROTAÇÃO FEMORAL NA MARCHA APÓS RIZOTOMIA DORSAL SELETIVA EM CRIANÇAS COM PARALISIA CEREBRAL ESPÁSTICA: OBJETIVO: Avaliar o resultado em longo prazo da rizotomia dorsal seletiva (RDS) na marcha e a influência da osteotomia derrotativa femoral (ODF). MÉTODO: Em um estudo de coorte retrospectivo com 29 crianças (13 do sexo feminino, 16 do sexo masculino) com paralisia cerebral diplégica espástica, 14 crianças receberam ODF antes da RDS, enquanto 15 crianças com rotação femoral interna moderada ou quase normal durante a marcha receberam apenas RDS. Dados tridimensionais de marcha foram obtidos pré ODF, pré RDS, 1 ano pós RDS, e 3 a 5 anos após RDS, para estudar o Escore do Perfil da Marcha (EPM), inclinação pélvica, e cinemática de joelho e quadril. Uma análise mista de variância com a medida repetida 'tempo' foi realizada entre os diferentes pontos para cada grupo. RESULTADOS: Crianças que primeiro passaram por ODF e depois pela RDS começaram com patologia de marcha mais complexa, mas tiveram menos desvios na marcha 3 a 5 anos após RDS, comparadas com crianças que receberam apenas RDS. Isso foi refletido por menor escore EMG e inclinação pélvica, assim como menos flexão do joelho na fase de apoio. INTERPRETAÇÃO: O efeito da RDS na marcha apenas é significativo no médio a longo prazo se os braços de alavanca ósseos também são corrigidos. Assim, o resultado clínico após RDS depende de bom alinhamento proximal.

Medial gastrocnemius volume and echo-intensity after botulinum neurotoxin A interventions in children with spastic cerebral palsy.

AIM: This cross-sectional investigation evaluated whether recurrent botulinum neurotoxin A (BoNT-A) interventions to the medial gastrocnemius have an influence on muscle morphology, beyond Gross Motor Function Classification System (GMFCS) level. METHOD: A cohort of typically developing children (n=67; 43 males, 24 females; median age 9y 11mo [range 7y 10mo-11y 6mo]), a cohort of children with spastic cerebral palsy (CP) naive to BoNT-A interventions (No-BoNT-A; n=19; 10 males, nine females; median age 9y 3mo [range 8y 5mo-10y 10mo]) and a cohort of children with spastic CP with a minimum of three recurrent BoNT-A interventions to the medial gastrocnemius (BoNT-A; n=19; 13 males, six females; median age 9y 8mo [range 7y 3mo-10y 7mo]) were recruited. Three-dimensional freehand ultrasound was used to estimate medial gastrocnemius volume normalized to body mass and echo-intensity. RESULTS: Normalized medial gastrocnemius volume and echo-intensity significantly differed between the two spastic CP cohorts (p≤0.05), with the BoNT-A cohort having larger alterations. Associations between normalized medial gastrocnemius volume and echo-intensity were highest in the No-BoNT-A cohort, followed by the BoNT-A cohort. Multiple regression analyses revealed that both GMFCS level and BoNT-A intervention history were significantly associated with smaller normalized medial gastrocnemius volume and higher echo-intensity. INTERPRETATION: Recurrent BoNT-A interventions may induce alterations to medial gastrocnemius volume and echo-intensity beyond the natural history of the spastic CP pathology. WHAT THIS PAPER ADDS: In spastic cerebral palsy, medial gastrocnemius volumes are smaller and echo-intensities higher compared with typical development. Alterations after botulinum neurotoxin A intervention (BoNT-A) are larger than in no BoNT-A intervention. Gross Motor Function Classification System level and BoNT-A history significantly associate with medial gastrocnemius and echo-intensity alterations.

VOLUMEN DEL MÚSCULO GASTROCNEMIUS MEDIAL E INTENSIDAD DE LA ECOGRAFÍA DESPUÉS DE LA COLOCACIÓN DE NEUROTOXINA BOTULÍNICA (TIPO A), EN NIÑOS CON PARÁLISIS CEREBRAL ESPÁSTICA (PC): OBJETIVO: Esta investigación de corte transversal evaluó si la colocación recurrente de neurotoxina botulínica tipo A (BoNT-A) en el músculo gastrocnemius medial tienen una influencia en la morfología muscular, más allá del nivel del Sistema de Clasificación de la Función Motora Gruesa (GMFCS, siglas en ingles). MÉTODO: Una cohorte de niños con desarrollo tipico (n = 67; 43 varones, 24 mujeres; mediana de edad 9 años 11 meses [rango 7 años 10 meses -11 años 6 meses]), otra cohorte de niños con parálisis cerebral espástica (PC) que no habían recibido intervenciones de BoNT-A ( n = 19; 10 hombres, nueve mujeres; mediana de edad 9 años 3 meses [rango 8 años 5 meses -10 años 10 meses]) y una cohorte de niños con PC espástica con un mínimo de tres administraciones recurrentes de BoNT-A en el músculo gastrocnemius medial (BoNT-A; n = 19; 13 varones, seis mujeres; mediana de edad 9 años 8 meses [rango 7 años 3 meses - 10 años 7 meses]) fueron reclutados. Se usó ultrasonido tridimensional a mano alzada para estimar el volumen del músculo gastrocnemius medial normalizado a la masa corporal y la intensidad del eco. RESULTADOS: El volumen del músculo gastrocnemius medial normalizado y la intensidad del eco difirieron significativamente entre las dos cohortes de PC espástica (p≤0,05), la cohorte BoNT-A tuvo alteraciones mayores. Las asociaciones entre el volumen músculo gastrocnemius medial normalizado y la intensidad del eco fueron más altas en la cohorte No-BoNT-A, seguidas de la cohorte BoNT-A. Los análisis de regresión múltiple revelaron que tanto el nivel de GMFCS como el antecedente de intervención de BoNT-A se asociaron significativamente con un volumen del músculo gastrocnemius medial normalizado más pequeño y una mayor intensidad de eco. INTERPRETACIÓN: La colocación recurrentes de BoNT-A pueden inducir alteraciones en el volumen del músculo gastrocnemius medial y en la intensidad del eco más allá de la historia natural de la patología por PC espástica.

VOLUME E ECO-INTENSIDADE DO MÚSCULO GASTROCNÊMIO MEDIAL APÓS INTERVENÇÕES COM NEUROTOXINA BOTULÍNICA A EM CRIANÇAS COM PARALISIA CEREBRAL ESPÁSTICA: OBJETIVO: Esta investigação transversal avaliou se intervenções recorrentes com neurotoxina botulínica A (NTBo-A) no músculo gastrocnêmio medial têm influência na morfologia muscular, além do nível segundo o Sistema de Classificação da Função Motora Grossa (GMFCS). MÉTODO: Uma coorte de crianças com desenvolvimento típico (n=67; 43 do sexo masculino, 24 do sexo feminino; idade mediana 9a 11m [variação 7a 10m-11a 6m]), uma coorte de crianças com paralisia cerebral espástica (PC) que nunca recebeu intervenções com NTBo-A (No-NTBo-A; n=19; 10 do sexo masculino, nove do sexo feminino; idade mediana 9a 3m [variação 8a 5m-10a 10m]) e uma coorte de crianças com PC espástica com no mínimo três intervenções de NTBo-A no músculo gastrocnêmio medial (NTBo-A; n=19; 13 do sexo mascuino, seis do sexo feminino; idade mediana 9a 8m [variação 7a 3m-10a 7m]) foram recrutadas. Ultrassom tridimensional foi usado para estimar o volume do gastrocnêmio normalizado para a massa corporal e eco-intensidade. RESULTADOS: O volume normalizado e eco-intensidade do músculo gastrocnêmio medial diferiu significantemente entre as duas coortes de PC espástica (p≤0,05), com a coorte NTBo-A tendo maiores alteracões. Associações entre o volume normalizado do gastrocnêmio e eco-intensidade foram maiores na coorte No-NTBo-A, seguida pela coorte NTBo-A. Análises de regressão múltipla revelaram que tanto o nível GMFCS quanto a história de intervenção com NTBo-A foram significativamente associadas com menor volume normalizado do músculo gastrocnêmio medial e maior eco-intensidade. INTERPRETAÇÃO: Intervenções recorrentes com NTBo-A podem induzir alterações no volume e eco-intensidade do músculo gastrocnêmio medial, além da história natural da patologia da PC espástica.

The migration percentage measured on EOS® standing full-leg radiographs: equivalent and advantageous in ambulant children with cerebral palsy.

BACKGROUND: During ambulatory follow-up of patients with cerebral palsy (CP) systematic radiographic screening is required firstly to evaluate hip migration and development in the prevention of hip dislocation and secondly to analyse lower limb alignment and leg length. The Migration Percentage (MP) is a radiographic measurement used to describe the extent of femoral head lateralisation on conventional supine pelvic radiographs. Our goal was to assess the comparability of the MP measured on low radiation dose EOS® standing full-leg radiographs with that of conventional supine pelvic radiographs. METHODS: Patients presenting with CP were prospectively selected from our outpatient follow-up consultation at our institutions CP reference centre and underwent conventional supine pelvic and EOS® standing full-leg radiographs the same day for diagnostic and screening reasons. RESULTS: Out of 28 prospectively selected patients we included 21 (42 hips), of which 10 were female, with a mean age of 9.25 years and GMFCS levels of I, II and III. Seven out of 28 patients were excluded due to insufficient quality of radiographic images. The absolute differences in MP measured on both conventional supine pelvic and EOS® standing full-leg radiographs ranged between - 8 and 6% with an absolute mean difference of 0% (SD ±3.5) and were not statistically significant (p = 0.99). A Bland-Altman plot showed acceptable agreement between both measurements without proportional bias. CONCLUSION: There is no statistical significant difference between the Migration Percentage measured on conventional supine pelvic radiographs and EOS® standing full-leg radiographs in ambulant patients. These images use lower radiation doses and contain more radiographic information. TRIAL REGISTRATION: Approved by the Medical Research Ethics committee of the University Hospitals Leuven ( MP001492 ).

Rectus femoris transfer improves stiff knee gait in hemiplegic adults following stroke or traumatic brain injury.

The aim of this study was to provide quantitative evidence of the effect of rectus femoris (RF) transfer surgery on improving gait in adults suffering from stiff knee gait (SKG) following stroke or traumatic brain injury (TBI). Retrospective cohort study University hospital, department of orthopaedic surgery Hemiplegic patients with decreased peak knee flexion in swing, reduced total knee range of motion and spasticity of the RF demonstrated by a positive Duncan Ely test and a pathologic dynamic electromyography of the RF. Ten right hemiplegic patients had a distal RF transfer. Pre- and postoperative kinematic, kinetic, and spatiotemporal parameters derived from 3D gait analysis and parameters from clinical examinations were retrospectively compared. All patients (average age 40 ± 29 years) had an improvement of their gait. Statistically significant improvements were observed in walking velocity and peak knee flexion in swing (19.93° ±11.80°), knee flexion velocity at toe-off (110.26° ± 65.74°) and total knee range of motion (20.78° ± 0.66°). RF transfer improves knee flexion in swing in adult patients suffering from SKG following stroke or TBI and is thus a reliable treatment option.

Evolution of self-care and functional mobility after single-event multilevel surgery in children and adolescents with spastic diplegic cerebral palsy.

AIM: To explore the evolution of self-care and functional mobility after multilevel surgery in children and adolescents with spastic diplegic cerebral palsy and to identify factors affecting these outcomes. METHOD: Thirty-four participants (22 males, 12 females) were evaluated before surgery, and at 2 months, 6 months, 1 year, 18 months, and 2 years after surgery. Self-care was assessed with the Pediatric Evaluation of Disability Inventory Dutch edition. The Mobility Questionnaire47 (MobQues47) and Functional Mobility Scale (FMS) were used to measure functional mobility. RESULTS: All outcomes revealed a significant decrease 2 months after single-event multilevel surgery (SEMLS) (p-value between <0.001 and 0.02) followed by a significant increase at 6 months (p<0.001 and p=0.045). Between 6 months and 1 year, a significant increase was also revealed for Mobques47 (p<0.001), FMS (p≤0.008), and the Pediatric Evaluation of Disability Inventory Functional Skills Scale (PEDI-FSS) (p=0.001). Improvement continued until 18 months for the PEDI-FSS. Initial score, initial muscle strength, Gross Motor Function Classification System level, age, and number of surgical interventions significantly influenced time trends for self-care and/or functional mobility. INTERPRETATION: Most preoperative scores are regained at 6 months after SEMLS. Further improvement is seen until 18 months. Participants with a higher functional level before surgery will temporarily lose more than participants with lower initial functional ability, but they also fast regain their function. WHAT THIS PAPER ADDS: Self-care and functional mobility decrease significantly in the first months after single-event multilevel surgery (SEMLS). Six months after SEMLS most preoperative scores are regained. Impact of SEMLS is more pronounced for functional mobility than for self-care. Muscle strength and functionality at baseline are important influencing factors on the evolution after SEMLS.

Identification of joint patterns during gait in children with cerebral palsy: a Delphi consensus study.

AIM: This study aims to achieve an international expert consensus on joint patterns during gait for children with cerebral palsy (CP) by means of Delphi surveys. METHOD: In Stage 1, seven local experts drafted a preliminary proposal of kinematic patterns for each lower limb joint in the sagittal, coronal, and transverse plane. In Stage 2, 13 experts from eight gait laboratories (four in the USA and four in Europe), participated in a Delphi consensus study. Consensus was defined by a pre-set cut-off point of 75% agreement among participants. RESULTS: After the first stage, 44 joint patterns were presented in a first survey and 29 patterns reached consensus. Consensus improved to 47 out of 48 patterns in the third survey. Only one pattern, 'abnormal knee pattern during loading response', did not reach consensus. The expert panel agreed to define six patterns for the knee during swing, most of them representing characteristics of a stiff knee pattern. INTERPRETATION: The defined joint patterns can support clinical reasoning for children with CP as joint patterns during gait might be linked to different treatment approaches. Automating the classification process and incorporating additional trunk, foot, and electromyography features should be prioritized for the near future.

Motor endplate-targeted botulinum toxin injections of the gracilis muscle in children with cerebral palsy.

AIM: Intramuscular botulinum toxin-A (BoNT-A) injections reduce spasticity by blocking neurotransmission at the motor endplate (MEP). The goal of this study was to assess the reduction in spasticity achieved by injecting BoNT-A at different sites of the gracilis muscle. METHOD: Thirty-four gracilis muscles, in 27 children (10 females and 17 males, mean age of 8.6y [SD 2.5y]) with spastic cerebral palsy (unilateral and bilateral, Gross Motor Function Classification System [GMFCS] levels I-IV), were randomly assigned to one of two groups. In one group BoNT-A was injected proximally (at a site 25% of the distance from the pubic tubercle and the medial epicondyle) and in the other it was injected at the MEP zones (half of the dose was administered at 30% of this distance and half at 60%). Spasticity was assessed before and after BoNT-A injection using simultaneous measurements of surface electromyography (sEMG) and angular velocity during passive muscle stretch applied at different velocities. The primary outcome measure included the velocity-dependent change in average root mean square electromyography (RMS-EMG). Secondary outcome was assessed with the Modified Ashworth Scale (MAS) and Modified Tardieu Scale (MTS). RESULTS: Spasticity decreased more in MEP-targeted muscles than in proximally injected muscles, as demonstrated by a larger reduction in average RMS-EMG values (p=0.04), though this difference was not found with the MAS or MTS. INTERPRETATION: The results suggest that BoNT-A injection of the gracilis at sites with a high concentration of MEPs is effective at reducing spasticity. These preliminary findings should be confirmed by larger studies. In the case of long muscles, such as the gracilis, the injection site is important.

Role of motor end plate-targeted Botulinum toxin type A injections in children with cerebral palsyitle.

Botulinum toxin type A (BTX) injections are frequently used in children with cerebral palsy (CP) to control spasticity. Injection variables still lead to variable outcomes of this treatment. Using instrumented spasticity assessment and muscle volume assessment the most effective location of the injection was demonstrated for gracilis and psoas muscles in children with CP. It was found that this treatment is most effective when injected in the motor endplate zones of the selected muscles. This review article presents all available research on the role of motor endplate-targeting of BTX injections in children with CP.

Is an instrumented spasticity assessment an improvement over clinical spasticity scales in assessing and predicting the response to integrated botulinum toxin type a treatment in children with cerebral palsy?

OBJECTIVE: To compare responsiveness and predictive ability of clinical and instrumented spasticity assessments after botulinum toxin type A (BTX) treatment combined with casting in the medial hamstrings (MEHs) in children with spastic cerebral palsy (CP). DESIGN: Prospective cohort study. SETTING: Hospital. PARTICIPANTS: Consecutive sample of children (N=31; 40 MEH muscles) with CP requiring BTX injections. INTERVENTION: Clinical and instrumented spasticity assessments before and on average ± SD 53±14 days after BTX. MAIN OUTCOME MEASURES: Clinical spasticity scales included the Modified Ashworth Scale and the Modified Tardieu Scale. The instrumented spasticity assessment integrated biomechanical (position and torque) and electrophysiological (surface electromyography) signals during manually performed low- and high-velocity passive stretches of the MEHs. Signals were compared between both stretch velocities and were examined pre- and post-BTX. Responsiveness of clinical and instrumented assessments was compared by percentage exact agreement. Prediction ability was assessed with a logistic regression and the area under the receiver operating characteristic (ROC) curves of the baseline parameters of responders versus nonresponders. RESULTS: Both clinical and instrumented parameters improved post-BTX (P≤.005); however, they showed a low percentage exact agreement. The baseline Modified Tardieu Scale was the only clinical scale predictive for response (area under the ROC curve=0.7). For the instrumented assessment, baseline values of root mean square (RMS) electromyography and torque were better predictors for a positive response (area under the ROC curve=.82). Baseline RMS electromyography remained an important predictor in the logistic regression. CONCLUSIONS: The instrumented spasticity assessment showed higher responsiveness than the clinical scales. The amount of RMS electromyography is considered a promising parameter to predict treatment response.

Identification of Neural and Non-Neural Origins of Joint Hyper-Resistance Based on a Novel Neuromechanical Model.

Joint hyper-resistance is a common symptom in neurological disorders. It has both neural and non-neural origins, but it has been challenging to distinguish different origins based on clinical tests alone. Combining instrumented tests with parameter identification based on a neuromechanical model may allow us to dissociate the different origins of joint hyper-resistance in individual patients. However, this requires that the model captures the underlying mechanisms. Here, we propose a neuromechanical model that, in contrast to previously proposed models, accounts for muscle short-range stiffness (SRS) and its interaction with muscle tone and reflex activity. We collected knee angle trajectories during the pendulum test in 15 children with cerebral palsy (CP) and 5 typically developing children. We did the test in two conditions - hold and pre-movement - that have been shown to alter knee movement. We modeled the lower leg as an inverted pendulum actuated by two antagonistic Hill-type muscles extended with SRS. Reflex activity was modeled as delayed, linear feedback from muscle force. We estimated neural and non-neural parameters by optimizing the fit between simulated and measured knee angle trajectories during the hold condition. The model could fit a wide range of knee angle trajectories in the hold condition. The model with personalized parameters predicted the effect of pre-movement demonstrating that the model captured the underlying mechanism and subject-specific deficits. Our model may help with the identification of neural and non-neural origins of joint hyper-resistance and thereby opens perspectives for improved diagnosis and treatment selection in children with spastic CP, but such applications require further studies to establish the method's reliability.