Understanding factors that influence goal setting in rehabilitation for paediatric acquired brain injury: a qualitative study using the Theoretical Domains Framework.

Background While goal setting with children and their families is considered best practice during rehabilitation following acquired brain injury, its successful implementation in an interdisciplinary team is not straightforward. This paper describes the application of a theoretical framework to understand factors influencing goal setting with children and their families in a large interdisciplinary rehabilitation team. Methods A semi-structured focus group was conducted with rehabilitation clinicians and those with lived experience of paediatric acquired brain injury (ABI). The 90-min focus group was audio-recorded and transcribed verbatim. Data were thematically coded and mapped against the Theoretical Domains Framework (TDF) to understand influencing factors, which were then linked to the Capability, Opportunity, Motivation - Behaviour (COM-B) model. Results A total of 11 participants (nine paediatric rehabilitation clinicians, one parent and one young person with lived experience of paediatric ABI) participated in the focus group. Factors influencing collaborative goal setting mapped to the COM-B and six domains of the TDF: Capabilities (Skills, Knowledge, Beliefs about capabilities, and Behavioural regulation), Opportunities (Environmental context and resources), and Motivation (Social/professional role and identity). Results suggest that a multifaceted intervention is needed to enhance rehabilitation clinicians' and families' skills and knowledge of goal setting, restructure the goal communication processes, and clarify the roles clinicians play in goal setting within the interdisciplinary team. Conclusion The use of the TDF and COM-B enabled a systematic approach to understanding the factors influencing goal setting for children with acquired brain injury in a large interdisciplinary rehabilitation team, and develop a targeted, multifaceted intervention for clinical use. These represent important considerations for the improvement of collaborative goal setting in paediatric rehabilitation services to ensure that best practice approaches to goal setting are implemented effectively in clinical practice.

Functional correlates of clinical phenotype and severity in recurrent SCN2A variants.

In SCN2A-related disorders, there is an urgent demand to establish efficient methods for determining the gain- (GoF) or loss-of-function (LoF) character of variants, to identify suitable candidates for precision therapies. Here we classify clinical phenotypes of 179 individuals with 38 recurrent SCN2A variants as early-infantile or later-onset epilepsy, or intellectual disability/autism spectrum disorder (ID/ASD) and assess the functional impact of 13 variants using dynamic action potential clamp (DAPC) and voltage clamp. Results show that 36/38 variants are associated with only one phenotypic group (30 early-infantile, 5 later-onset, 1 ID/ASD). Unexpectedly, we revealed major differences in outcome severity between individuals with the same variant for 40% of early-infantile variants studied. DAPC was superior to voltage clamp in predicting the impact of mutations on neuronal excitability and confirmed GoF produces early-infantile phenotypes and LoF later-onset phenotypes. For one early-infantile variant, the co-expression of the α1 and ß2 subunits of the Nav1.2 channel was needed to unveil functional impact, confirming the prediction of 3D molecular modeling. Neither DAPC nor voltage clamp reliably predicted phenotypic severity of early-infantile variants. Genotype, phenotypic group and DAPC are accurate predictors of the biophysical impact of SCN2A variants, but other approaches are needed to predict severity.

Epidemiology of paediatric chronic fatigue syndrome in Australia.

OBJECTIVE: To estimate the paediatrician-diagnosed incidence of chronic fatigue syndrome (CFS) in Australia, and describe demographic and clinical features, as well as approaches to diagnosis and management. METHODS: The Australian Paediatric Surveillance Unit facilitates monthly national surveillance of uncommon conditions seen by paediatricians. Data from young people aged <18 years diagnosed with CFS were collected. Incidence was estimated based on new cases reported from April 2015 to April 2016. RESULTS: A total of 164 cases of newly diagnosed CFS in young people aged 4-17 years were identified for inclusion. The estimated national incidence for children aged 4-9 years was 0.25 per 100 000 per annum. In children aged 10-17 years, the estimated incidence of paediatrician-diagnosed cases for Victoria (17.48 per 100 000) was substantially greater than other Australian states (range 1.31-5.51 per 100 000). Most cases were female and Caucasian, most commonly presenting after an infectious illness with symptoms gradual in onset. The majority were diagnosed at least 13 months after symptom onset. Symptoms, associations, investigations and management strategies were highly variable. CONCLUSIONS: Current findings suggest that, consistent with other countries, the Australian incidence of CFS in children aged <10 years is very low. In contrast, the national incidence of CFS in older children and adolescents (aged 10-17 years) is more unclear, with marked variability between geographical regions apparent. This may be due to variation in service accessibility and clinician understanding of CFS. Accordingly, national initiatives to improve equity of care for children with CFS may be required.

The Gait Outcomes Assessment List (GOAL): validation of a new assessment of gait function for children with cerebral palsy.

AIM: We investigated the validity of the Gait Outcomes Assessment List (GOAL), as an assessment of gait function in children with cerebral palsy (CP). METHOD: We studied a prospective cohort of 105 children with CP (Gross Motor Function Classification System [GMFCS] levels I-III; 65 males, 40 females; mean [SD] age 11y 11mo [3y 5mo], range 6-20y), who attended gait assessment over a 10-month period. Parents completed the GOAL, Functional Mobility Scale (FMS), and Functional Assessment Questionnaire (FAQ) during their child's gait evaluation. Ninety children completed instrumented gait analysis (IGA). Total GOAL and domain scores, Gait Profile Score (GPS), and Gait Variable Scores were calculated. RESULTS: The total GOAL discriminated between GMFCS levels (mean [SD] GMFCS level I, 72.5 [12.7]; GMFCS level II, 61.4 [13.0]; GMFCS level III, 38.8 [10.6]; [F2,97 =42.4, p<0.001]). Moderate correlations were found between total GOAL and FMS (5m and 50m r=0.59; 500m r=0.66) and FAQ walking (r=0.77) and activities list (r=0.75, p<0.01). There was a moderate negative correlation between total GOAL and GPS (r=-0.59) and gait appearance domain and GPS (r=-0.52, p<0.01). INTERPRETATION: The GOAL is a valid assessment of gait function in ambulant children with CP. It has the potential to improve understanding of the child's and parents' priorities and thus, in conjunction with IGA, provide a more balanced assessment across the domains of the World Health Organization's International Classification of Functioning, Disability and Health. WHAT THIS PAPER ADDS: The Gait Outcomes Assessment List (GOAL) can discriminate between Gross Motor Function Classification System levels. The GOAL correlates with standard functional assessments and gait analysis. Used with gait analysis, the GOAL provides comprehensive assessment across all International Classification of Functioning, Disability and Health domains.

Sagittal gait patterns in cerebral palsy: the plantarflexor-knee extension couple index.

The identification of gait patterns in cerebral palsy offers a common language for clinicians and contributes to management algorithms. We describe a quantitative classification of sagittal gait patterns based on the plantarflexor-knee extension couple index. This consists of a scatter plot based on ankle and knee scores, and allows objective identification of the sagittal gait pattern. Sagittal kinematic data from 200 limbs of 100 patients with bilateral spastic cerebral palsy were utilized to validate the algorithm against the assessment of a clinician with expertise in gait pattern identification. A dataset of 776 cerebral palsy patients, 1552 limbs, was used to compare the sagittal gait patterns against k-means statistical clustering. The classification was further explored with respect to the knee kinetics during the middle of stance and physical examination measurements of the gastrocnemius-soleus complex. Two supplementary materials (Appendices 2 and 3) provide in-depth discussion about statistical properties of the plantarflexor-knee extension couple index as well as its relationship with statistical clustering. The plantarflexor-knee extension index achieved 98% accuracy and may be suitable for the computational classification of large patient cohorts and multicentre studies. The sagittal gait patterns were strongly related to k-means statistical clustering and physical examination of the gastrocnemius-soleus complex. Patients in crouch gait had normal soleus and gastrocnemius lengths but spasticity in the gastrocnemius. Patients in jump gait exhibited a short gastrocnemius and soleus and gastrocnemius spasticity. Patients in true equinus presented with a moderately contracted soleus and gastrocnemius and gastrocnemius spasticity. Patients in apparent equinus did not show abnormal physical examination measurements for the gastrocnemius-soleus complex.

Multilevel surgery for equinus gait in children with spastic diplegic cerebral palsy: medium-term follow-up with gait analysis.

BACKGROUND: In children with spastic diplegia, surgery for ankle equinus contracture is associated with a high prevalence of both overcorrection, which may result in a calcaneal deformity and crouch gait, and recurrent equinus contracture, which may require revision surgery. We sought to determine if conservative surgery for equinus gait, in the context of multilevel surgery, could result in the avoidance of overcorrection and crouch gait as well as an acceptable rate of recurrent equinus contracture at the time of medium-term follow-up. METHODS: This was a retrospective, consecutive cohort study of children with spastic diplegia who had had surgery for equinus gait between 1996 and 2006. All children had distal gastrocnemius recession or differential gastrocnemius-soleus complex lengthening, on one or both sides, as part of single-event multilevel surgery. The primary outcome measures were the Gait Variable Scores (GVS) and Gait Profile Score (GPS) at two time points after surgery. RESULTS: Forty children with spastic diplegia, Gross Motor Function Classification System (GMFCS) level II or III, were included in this study. There were twenty-five boys and fifteen girls. The mean age was ten years at the time of surgery and seventeen years at the time of final follow-up. The mean postoperative follow-up period was 7.5 years. The mean ankle GVS improved from 18.5° before surgery to 8.7° at the time of short-term follow-up (p < 0.005) and 7.8° at the time of medium-term follow-up. The equinus gait was successfully corrected in the majority of children, with a low rate of overcorrection (2.5%) and a high rate of recurrent equinus (35%), as determined by sagittal ankle kinematics. Mild recurrent equinus was usually well tolerated and conferred some advantages, including contributing to strong coupling at the knee and independence from using an ankle-foot orthosis. CONCLUSIONS: Surgical treatment for equinus gait in children with spastic diplegia was successful, at a mean of seven years, in the majority of cases when combined with multilevel surgery, orthoses, and rehabilitation. No patient developed crouch gait, and the rate of revision surgery for recurrent equinus was 12.5%.

Management of children with ambulatory cerebral palsy: an evidence-based review. Commentary by Hugh Williamson Gait Laboratory staff.

The evaluation of complex interventions, such as Single Event Multilevel Surgery (SEMLS) requires more than randomized controlled trials. Rehabilitation following SEMLS is prolonged and the outcomes of interest may not be apparent for 5 years or more after the surgery. We suggest long term, prospective cohort studies with objective outcome measures be recognized as of equal importance to randomized controlled trials. The evidence in support of instrumented gait analysis (IGA) is also reviewed. We suggest that clinical levels of evidence are not an appropriate method to evaluate a measurement tool. Specifically, IGA should be evaluated in terms of validity, reliability and cost effectiveness. We demonstrate that the use of IGA has improved medium and long term outcomes in ambulant children with cerebral palsy in a center where IGA has been used routinely both for planning SEMLS and for monitoring outcomes.

Progressive gait deterioration in adolescents with Dravet syndrome.

OBJECTIVE: To characterize changes in gait by age in patients with Dravet syndrome. DESIGN: Prospective, cross-sectional study. SETTING: Tertiary children's hospital. PATIENTS: Twenty-six subjects with Dravet syndrome, aged 2 to 34 years. Twenty-three patients had mutations of the sodium channel α1 subunit gene, SCN1A. INTERVENTIONS: Assessment via video gait analysis, physical examination of the lower limbs, use of the Functional Mobility Scale, and radiographs of the pelvis and feet. MAIN OUTCOME MEASURES: Classification of the sagittal gait pattern and foot posture, assessment of muscle extensibility and joint range, and rating of functional mobility. RESULTS: Children aged 0 to 5 years had a normal or near-normal gait, whereas 5 of 10 children aged 6 to 12 years and 8 of 9 children aged 13 years or older had crouch gait. Physical examination showed that with increasing age, passive knee extension (P = .008) and hip extension (P = .003) decreased, external tibial torsion (P = .007) and pes planovalgus (P = .05) increased, and increased hip internal rotation did not show age-related change (P = .27). The Functional Mobility Scale showed universal independent walking over 5 and 50 m; however, adolescents showed wide variation in their ratings over 500 m, indicating mobility ranging from wheelchair use to independent walking (P = .02). CONCLUSIONS: Children with Dravet syndrome show progressive gait deterioration in the second decade of life, with crouch gait and skeletal malalignment comprising increased femoral neck anteversion, external tibial torsion, and pes valgus. These age-related changes have a significant impact on mobility and independence in the community setting.

The use of videoconferencing to enhance interprofessional clinical education for allied health students.

The Health-e-Learning project investigated the use of videoconferencing to deliver interprofessional clinical education to allied health students. Via a broadband link, students observed DVD footage of a clinical session then participated in discussion with the clinicians at the Royal Children's Hospital (RCH), Melbourne. The videoconference sessions were evaluated with respect to session content, the effectiveness of videoconferencing in providing interprofessional education (IPE) and the satisfaction with this as a supplement to facility-based placements. During the two semesters of the project, 28 videoconference sessions were conducted and 724 participants were surveyed. Student responses were positive with 84% indicating that these videoconferences increased their understanding of interprofessional practice (IPP) and 95% agreeing that the sessions were an effective learning tool. The results of this study support the use of videoconferencing to provide interprofessional clinical education.

The minimal clinically important difference for the Gait Profile Score.

The minimally clinically important difference (MCID) is an important concept for interpreting the results of clinical research. This paper proposes a rationale for defining an MCID for the Gait Profile Score (GPS) based on an analysis of the difference in median GPS for children classified at different levels of the Functional Assessment Questionnaire. A strong linear correlation between median score and FAQ level was found. An MCID of 1.6° is therefore suggested, reflecting the mean difference between adjacent FAQ levels. Comparison of this value with (i) the standard deviation of GPS from typically developing children (1.4°) and (ii) the percentage of the difference between the median GPS for each FAQ level and that for typically developing children offers further support to suggest that 1.6° is an appropriate figure.

Management of the knee in spastic diplegia: what is the dose?

This article discusses the sagittal gait patterns in children with spastic diplegia, with an emphasis on the knee, as well as the concept of the "dose" of surgery that is required to correct different gait pathologies. The authors list the various interventions in the order of their increasing dose. The concept of dose is useful in the consideration of the management of knee dysfunction.

The static examination of children and young adults with cerebral palsy in the gait analysis laboratory: technique and observer agreement.

The assessment of ambulant children with spastic cerebral palsy frequently includes an evaluation in the motion analysis laboratory, consisting of a standardized physical examination and instrumented gait analysis. We therefore designed a repeatability study to evaluate observer agreement of five joint range of motion parameters, in 20 patients with cerebral palsy aged 5-25 years. These five parameters are some of the most important measurements made during the static examination in the gait laboratory. Intra-observer agreement was high (concordance correlation coefficient range, 0.67 -0.96), and using Bland and Altman analysis it was clinically acceptable with 95% limits of agreement of all parameters close to within +/- 10degrees. The level of inter-observer agreement was less satisfactory, particularly in hip flexion deformity.