SD 1y 8mo Research Articles

Outcomes in influenza and RANBP2 mutation‐associated acute necrotizing encephalopathy of childhood

To evaluate clinical and imaging features in patients with acute necrotizing encephalopathy of childhood (ANEC) to identify predictors of RANBP2 mutations, influenza association, and long-term outcomes. A retrospective chart review in patients with ANEC (2012-2020) seen at a tertiary pediatric center was performed. Children were included if they had acute inflammatory lesions in the basal ganglia and pons. Variables included presenting features, imaging characteristics, RANBP2 gene testing, nasopharyngeal swab findings, therapies, and long-term outcomes. Twenty patients were included (average age at presentation 3y 6mo, interquartile range3y 7mo, SD2y 8mo; 14 females, six males). Three of the 20 experienced recurrences; one of the 20 died. Ten patients were influenza positive. Seven patients were RANBP2 mutation positive. A higher likelihood of hemorrhage was observed in patients who were influenza positive compared to influenza negative (p=0.048). Patients with influenza had a higher degree of thalamic hemorrhage (2, p=0.035) and greater extent of diffusion restriction (3, p=0.035) in semiquantitive analysis. Children with ANEC who are positive for influenza are more likely to have hemorrhage and greater thalamic swelling. RANBP2 status was predictive of relapse but not predictive of overall outcome.

Program for the Education and Enrichment of Relational Skills for adolescents with an acquired brain injury: A randomized controlled trial

To test the efficacy of a group social skills intervention on social functioning in adolescents with a brain injury. Thirty-six adolescents (mean age 14y, SD 1y 8mo, age range 12y 1mo-16y 3mo; 17 females) with acquired brain injury (ABI; ≥12mo postintervention; n=19) or cerebral palsy (n=17) were randomly allocated to the Program for the Education in Enrichment of Relational Skills (PEERS) or usual care. The primary outcome was the Social Skills Improvement System-Rating Scales (SSIS-RS). Secondary outcomes were scores derived from the Test of Adolescent Social Skills Knowledge-Revised (TASSK-R), Social Responsiveness Scale, Second Edition, and Quality of Socialization Questionnaire. Between-group differences postintervention and at the 26-week retention time point were compared using linear mixed modelling for continuous outcomes and Poisson regression for count data. There were no between-group differences on the primary outcome (SSIS-RS). Regarding the secondary outcomes, the PEERS-exposed group achieved significantly greater improvements on the TASSK-R (mean difference [MD]=6.8, 95% confidence interval [CI]=4.8-8.8, p<0.001), which were maintained at the 26-week retention time point (MD=8.1, 95% CI=6.0-10.2, p<0.001). PEERS was also associated with a significant increase in parent-reported invited get-togethers at 26 weeks (incidence rate ratio=4.0, 95% CI=1.0-16.0, p=0.05). Adolescents with brain injury who completed the PEERS learned and retained social knowledge and increased social participation.

External validity of the Both Hands Assessment for evaluating bimanual performance in children with bilateral cerebral palsy.

To investigate the external validity of the Both Hands Assessment (BoHA), a new test evaluating bimanual performance in children with bilateral cerebral palsy (CP), by analysing its relationship to established measurements of hand function and self-care skills. In this cross-sectional study, we recruited children with bilateral CP and manual ability corresponding to Manual Ability Classification System (MACS) levels I to III attending three habilitation units in Norway. All participants completed the BoHA. Unimanual capacity was assessed using the Bimanual Fine Motor Function (BFMF) classification, the Boxand Block Test (BBT), and the Melbourne Assessment 2 (MA2). Self-care skills were assessed with the Pediatric Evaluation of Disability Inventory (PEDI). Thirty-nine children (19 males, 20 females; mean age 8y 2mo, SD 2y 8mo; age range 2y 8mo-12y 6mo) were included. Spearman's correlation coefficient (ρ) suggested high correlation between the BoHA and MACS (p=0.89; 95% confidence interval [CI] 0.79-0.94), BFMF classification (p=0.84; 95% CI 0.71-0.91), and BBT for the non-dominant (p=0.85; 95% CI 0.68-0.95) and dominant hand (p=0.72; 95% CI 0.53-0.85). The Spearman's ρ between the BoHA and the MA2 subscales varied between 0.48 and 0.83, while the PEDI's ρ was 0.51 (95% CI 0.33-0.67). The BoHA provides valid measures of hand use as suggested by its high correlation with other activity-based measures of hand function.

Dysphagia limit in children with cerebral palsy aged 4 to 12 years.

AimTo assess the dysphagia limit in children with cerebral palsy (CP) according to Eating and Drinking Ability Classification System (EDACS) level, sex, and age compared to typically developing children.MethodSeventy‐seven children with CP (54 males, 23 females; mean age 7y 6mo, SD 2y 2mo, age range 4–12y) were assessed with the Maximum Volume Water Swallow Test. Median dysphagia limit in the CP group was compared with data of typically developing children.ResultsThe dysphagia limit of children with CP differed significantly (p<0.001) from typically developing children. The latter showed a threefold higher median dysphagia limit (22mL) compared to children with CP in EDACS level I (7mL). The higher the EDACS level, the lower the dysphagia limit in children with CP. EDACS level explained 55% of the variance in the dysphagia limit of the CP group.InterpretationWhere children with CP in EDACS levels IV and V showed that their capacity met the level of their performance, children in EDACS level I had the ability to perform a maximum capacity task, but still had a threefold lower median dysphagia limit than typically developing children. Establishment of the dysphagia limit should be part of general swallowing assessment in children with CP.

Sit-to-stand training for self-care and mobility in children with cerebral palsy: a randomized controlled trial.

To investigate if a sit-to-stand exercise programme for children with cerebral palsy (CP) would improve self-care and mobility. Thirty-eight children with CP (19 males, 19 females; mean age 8y 0mo, SD 2y 4mo, age range 4y 0mo-12y 4mo) classified in Gross Motor Function Classification System (GMFCS) levels III and IV and their caregivers were randomly allocated to sit-to-stand training plus routine physiotherapy (balance and gait training) or routine physiotherapy only (controls). Task-specific sit-to-stand training was completed five times a week for 6 weeks under physiotherapist (twice weekly) and caregiver (three times weekly) supervision. Blinded outcome assessments at week 7 were the self-care and mobility domains of the Functional Independence Measure for Children, Five Times Sit-to-Stand Test (FTSST), and Modified Caregiver Strain Index (MCSI). The sit-to-stand group self-care increased by 2.2 units (95% confidence interval [CI] 1.3-3.1) and mobility increased by 2.2 units (95% CI 1.4-3.0) compared to the control group. In the sit-to-stand group, the FTSST was reduced by 4.0 seconds (95% CI -4.7 to -3.2) and the MCSI was reduced by 0.8 units (95% CI -1.2 to -0.4) compared to the control group. A sit-to-stand exercise programme for children with CP classified in GMFCS levels III and IV improved sit-to-stand performance and resulted in small improvements in self-care and mobility, while reducing caregiver strain. What this paper adds Sit-to-stand training improved independence in self-care and mobility for children with cerebral palsy (CP). Home-based sit-to-stand training programmes for children with CP can reduce the burden on supervising caregivers.

Repeated onabotulinum neurotoxin A injections for drooling in children with neurodisability.

AimTo evaluate the effect of repeated onabotulinum neurotoxin A injections for the treatment of drooling in children with neurodisabilities.MethodThis was a retrospective cohort study, in which the first, second, and third onabotulinum neurotoxin A injection were compared within children treated between 2000 and 2020. Primary outcomes included drooling quotient, visual analogue scale (VAS), and treatment success defined as ≥50% reduction in drooling quotient and/or VAS 8 weeks after treatment. Each outcome was obtained at baseline and 8 weeks posttreatment.ResultsSeventy‐seven children were included (mean age at first injection: 8y 3mo, SD 3y 7mo, range 3–17y; 44 males, 33 females; 51.9% with cerebral palsy, 45.5% wheelchair‐bound). The objective (drooling quotient) and subjective (VAS) effect after the second injection was lower compared to the first injection. The third injection showed less objective and significantly less subjective effect compared to the first injection. An overall success rate of 74.0%, 41.6%, and 45.8% were found for the first, second, and third injection respectively.InterpretationAlthough onabotulinum neurotoxin A remained effective throughout the entire treatment course, there is less effect of subsequent onabotulinum neurotoxin A injections compared to the first. Although there might be a loss of effect after repeated injections, there is continued improvement for most children. What this paper adds Repeated injections show a diminished treatment effect after the second injection.A continued improvement is seen in most patients.

Long-term outcomes of children with drug-resistant epilepsy across multiple cognitive domains.

To simultaneously evaluate long-term outcomes of children with drug-resistant epilepsy (DRE) across multiple cognitive domains and compare the characteristics of participants sharing a similar cognitive profile. Participants were adolescents and young adults (AYAs) diagnosed with DRE in childhood, who completed a comprehensive neuropsychological battery evaluating intelligence, memory, academic, and language skills at the time of surgical candidacy evaluation and at long-term follow-up (4-11y later). Hierarchical k-means clustering identified subgroups of AYAs showing a unique pattern of cognitive functioning in the long-term. Participants (n=93; mean age 20y 1mo [standard deviation {SD} 4y 6mo]; 36% male) were followed for 7 years (SD 2y 4mo), of whom 65% had undergone resective epilepsy surgery. Two subgroups with unique patterns of cognitive functioning were identified, which could be broadly categorized as 'impaired cognition' (45% of the sample) and 'average cognition' (55% of the sample); the mean z-score across cognitive measures at follow-up was -1.86 (SD 0.62) and -0.23 (SD 0.54) respectively. Surgical and non-surgical patients were similar with respect to seizure control and their long-term cognitive profile. AYAs in the average cognition cluster were more likely to have better cognition at baseline, an older age at epilepsy onset, and better seizure control at follow-up. The underlying abnormal neural substrate and seizure control were largely associated with long-term outcomes across cognitive domains.

Analysis of dynamic elbow flexion deformity in children with hemiplegic cerebral palsy.

BackgroundCerebral palsy affects 1 per 1.000 children, and in 83% of the cases upper extremity is involved. Dynamic elbow flexion deformity is a movement disorder observed in individuals with hemiparesis secondary to cerebral palsy. We sought to determine whether children with hemiplegic cerebral palsy exhibit dynamic elbow flexion deformity during daily activities and its influence to reaching function. MethodsSixteen children with upper limb hemiparesis and cerebral palsy (age 11y 7mo (SD 3y 2mo); 11 boys, 5 girls; Gross Motor Function Classification System level I or II) were included in this observational descriptive study. Manual Ability Classification System, Children's Hand-use Experience Questionnaire and Shriners Hospital for Children Upper Extremity Evaluation were used to evaluate affected upper extremity function. Spasticity was assessed with Modified Ashworth scale. Involuntary elbow flexion was recorded in eight daily activities. Elbow motion during reach function was measured. FindingsFifteen out of 16 individuals showed dynamic elbow flexion deformity. There was a significative increase of involved median elbow flexion in all the activities studied, except for “high speed stairs climbing” evaluation. Children's Hand-use Experience Questionnaire showed that children were independent in most of the daily activities (21 out of 29). Correlation between dynamic elbow flexion deformity and Shriners Hospital for Children Upper Extremity Evaluation was observed only in sitting-standing activity (Spearman's ρ 0.549, P = 0.028). InterpretationDynamic elbow flexion deformity is very common in hemiplegic cerebral palsy and occurs proportionally to the degree of the effort demanded by the activities. Despite of presenting this movement disorder, it does not affect in children participation in daily activities.

Locomotor and robotic assistive gait training for children with cerebral palsy.

To determine if robotic assisted gait training (RAGT) using surface muscle electrical stimulation and locomotor training enhances mobility outcomes when compared to locomotor training alone in children with cerebral palsy (CP). Forty children (18 females, 22 males; mean age 8y 1mo, SD 2y 1mo; range 5y 1mo-12y 11mo) with CP in Gross Motor Function Classification System levels (GMFCS) III, IV, and V were randomly assigned to the RAGT and locomotor training (RAGT+LT) group or locomotor training only group (dosage for both: three 1-hour sessions a week for 6weeks). Outcomes were assessed at baseline T1 (week 0), post-treatment T2 (week 6), and retention T3 (week 26). The primary outcome measure was the Goal Attainment Scale. Secondary outcome measures included the 10-metre walk test, children's functional independence measure mobility and self-care domain, the Canadian Occupational Performance Measure, and the Gross Motor Function Measure. There were no significant differences between the groups for both the primary and secondary outcome measures. All participants completed the intervention in their original group allocation. There were no reported adverse events. The addition of RAGT to locomotor training does not significantly improve motor outcomes in children with CP in GMFCS levels III, IV, and V. Future studies could investigate health and well-being outcomes after locomotor training. Marginally ambulant and non-ambulant children with cerebral palsy can participate in locomotor training. Robotic assisted gait training when added to locomotor training does not appear to be any more effective than locomotor training alone.

Cytokine dysregulation in children with cerebral palsy

To examine pro- and anti-inflammatory cytokines in children with cerebral palsy (CP) at baseline and in response to endotoxin (lipopolysaccharide), and correlate outcomes compared with age-matched comparisons, to evaluate their ability to mount an immune response. Serum cytokines were assessed in 12 children (eight males, four females; mean age 10y 1mo [SD 1y 8mo], 6-16y) with CP against 12 age-matched comparisons (eight males, four females; mean age 9y 1mo [SD 1y 1mo]). Pro- and anti-inflammatory cytokines (interleukin-1β, interleukin-2, interleukin-6, interleukin-8, interleukin-10, interleukin-18, tumour necrosis factor [TNF]-α, TNF-β, interferon-γ, granulocyte-macrophage colony-stimulating factor [GM-CSF], vascular endothelial growth factor [VEGF], erythropoietin, and interleukin-1 receptor antagonist) were measured at baseline and in response to in vitro simulation with lipopolysaccharide by multiplex enzyme-linked immunosorbent assay. Significantly higher erythropoietin was found at baseline in children with CP compared with the comparison group. There was a strong response to lipopolysaccharide for interleukin-8, VEGF, TNF-α, and GM-CSF in both children with CP and the comparison group; however, there was significant lipopolysaccharide hyporesponsiveness in children with CP compared with the comparison group for interleukin-1α, interleukin-1β, interleukin-2, and interleukin-6. Altered cytokine responses in children with CP compared with the comparison group demonstrate an altered inflammatory state that may contribute to ongoing sequelae and could be a target for therapy. Altered inflammatory responses persist in children with cerebral palsy (CP). Erythropoietin is elevated in children with CP compared with the comparison group. Children with CP have reduced interleukin-1α, interleukin-1β, interleukin-2, and interleukin-6 inflammatory responses to lipopolysaccharide.

Motor imagery in children with unilateral cerebral palsy: a case-control study.

To evaluate whether children with cerebral palsy (CP) are able to engage in a motor imagery task. Possible associations between motor imagery and functional performance, working memory, age, and intelligence were also investigated. This is a case-control study that assessed 57 children (25 females, 32 males) with unilateral CP, aged 6 to 14 years (mean age: 10y 4mo; SD 2y 8mo) and 175 typically developing (control) children, aged 6 to 13 years (87 females, 88 males; mean age: 9y 4mo; SD 1y 11mo). The hand laterality judgment task was used to measure motor imagery ability. Reaction time, accuracy, and the effect of the biomechanical constraints were assessed in this task. Performance in both groups followed the biomechanical constraints of the task, that is, longer reaction times to recognize stimuli rotated laterally when compared to medial stimuli. Reaction time means did not differ significantly between groups (p>0.05). Significant differences between the unilateral CP and control groups were observed for accuracy (p<0.05). Functional performance and working memory were correlates of motor imagery tasks. Results suggest that children with unilateral CP can engage in motor imagery; however, they commit more errors than typically developing controls. In addition, their performance in tasks of motor imagery is influenced by functional performance and working memory.

Dysarthria syndromes in children with cerebral palsy

To investigate whether dysarthria syndromes acquired in adulthood can also be observed in children with cerebral palsy (CP) and, if so, whether they align with children's CP subtypes. Twenty-six children with CP participated (mean age 7y 8mo [SD 1y 2mo], 5y 1mo-9y 10mo; 16 males and 10 females). Speech samples were elicited in a computer-based game and were analysed using the auditory perceptual criteria of the Bogenhausen Dysarthria Scales (BoDyS). For statistical classification, three comparison groups of adults with standard dysarthria syndromes (i.e. spastic, hyperkinetic, and ataxic) were used. Their BoDyS data were entered into a mixture discriminant analysis, with data from the comparison groups as the training sample and those from the children with CP as the test sample. Results were related to findings in a group of adults with CP. Among the children with CP, most had spastic (n=14), while fewer had ataxic (n=9) or hyperkinetic (n=3), dysarthria. However, syndrome allocations were significantly more ambiguous than in adults with CP. For 11 children, their dysarthria syndromes did not align with their CP subtype. Dysarthria syndromes are less clear cut in children than in adults with CP because of a number of developmental factors. Children with cerebral palsy (CP) show diverse patterns of dysarthric symptoms. Dysarthria syndromes do not seem to manifest fully during childhood. Dysarthria syndrome and CP subtype may not align in children with CP.

Sleep quantity and its relation with physical activity in children with cerebral palsy; insights using actigraphy.

AimTo objectively assess the sleep quantity, and explore the relationships between sleep quantity and quality, and physical activity and sedentary behaviour in children and adolescents with cerebral palsy (CP).MethodsAn observational cross‐sectional study was conducted. In total, 36 children with spastic CP (mean age 15y 4mo, SD 2y 6mo; classified as Gross Motor Function Classification System levels I (25), II (9), III (1) and IV (1)) were included. Active time, sedentary time and sleep quantity were measured using an activity monitor for 7 consecutive days.ResultsTotal sleep duration of children with CP ranged between 7.2 and 11.2 h. No significant correlations were found between active time and sleep quantity for total week, weekdays, and weekend days. Moderate negative correlations were found between sedentary time and sleep quantity during total week (r = −0.456, P = 0.005), weekdays (r = −0.453, P = 0.006) and weekend days (r = −0.48, P = 0.003).ConclusionsOur findings suggest that children with CP are getting the recommended sleep duration, and that sedentary behaviour is correlated with sleep quantity in children with CP and may be more applicable to children with better motor functions. Future studies using more elaborate, objective sleep quantity and quality measures are recommended.

Documenting change with the Canadian Occupational Performance Measure for children with cerebral palsy.

To assess the Canadian Occupational Performance Measure's (COPM) ability to document change over 3years in children with cerebral palsy (CP). This was a prospective study with ambulatory children with CP, aged 2 to 6years. Caregivers set one to three COPM goals which were rescored annually over 3years. A ceiling effect for performance goals was operationalized as a score of 8. A Wald χ2 generalized estimating equations model adjusted for age, sex, and Gross Motor Function Classification System (GMFCS) level, evaluated change over time. In total, 124 children (47 [37.9%] females, 77 [62.1%] males; mean age 3y 11mo [SD 1y 1mo]; GMFCS level I [n=78, 62.9%], II [n=21, 16.9%], and III [n=25, 20.2%]) were set 345 COPM goals at baseline. By Year 3, 106 participants (85.5%) rescored 287 of the goals (83.2%). Performance scores increased between baseline mean (SD) 2.93 (0.56), Year 1 5.98 (0.58) with 34.8% at ceiling; Year 2 6.74 (0.60) 48.3% at ceiling; and Year 3 7.37 (0.60) 59.6% at ceiling (Wald χ2 [3]=607.18, p<0.001). Satisfaction scores increased between baseline 4.42 (0.59), Year 1 6.82 (0.60) with 48% at ceiling; Year 2 7.53 (0.60) with 62.2% at ceiling (Wald χ2 [3]=208.48, p<0.001); with no significant increase by Year 3 7.82 (0.62) with 66.9% at ceiling. COPM performance scores increased steadily over 3years. By Year 2, a ceiling effect was seen in about half of the goals. The COPM may have utility to measure change over 3years; periodic resetting of the descriptors of goal success are required to minimize ceiling.

Autism and attention-deficit/hyperactivity disorders and symptoms in children with neurofibromatosis type 1.

To evaluate if autism symptoms and diagnoses are more common in children with neurofibromatosis type 1 (NF1) than in typically developing children, to which levels, and to determine if co-occurring attention-deficit/hyperactivity disorder (ADHD) symptomatology accounts for this increase. We searched hospital electronic medical records (EMR) for International Classification of Diseases, 10th Revision NF1 and co-occurring diagnoses codes. We recruited a subsample of 45 children (mean age 9y 2mo; SD 2y 7mo; range 5-12y; 22 males, 23 females) and collected parental reports of autism symptomatology, adaptive behavior, and behavioral problems that were compared to those of 360 age- and sex-matched controls from the Simons Simplex Collection (SSC) with autism spectrum disorder (ASD; SSC-ASD) or typically developing (SSC-TD). The EMR search identified 968 children with NF1; 8.8% had ADHD and 2.1% had ASD co-occurring diagnoses. In the subsample, the mean autism scale score for participants with NF1 was below cut-off for significant autism symptoms. Participants with NF1 had significantly more autism and behavioral symptoms than SSC-TD participants, and significantly less than SSC-ASD participants, with one exception: ADHD symptom levels were similar to those of SSC-ASD participants. In analyses that controlled for internalizing, ADHD, and communication scores, the difference in autism symptom levels between participants with NF1 and typically developing controls disappeared almost entirely. Our results do not support an association between NF1 and autism, both at the symptom and disorder levels. Diagnoses of attention-deficit/hyperactivity disorder (ADHD) were more common in children with neurofibromatosis type 1 (NF1) than in the general child population. Diagnoses of autism spectrum disorder were no more common in children with NF1 than in the general child population. Increases in autism symptoms did not reach clinically significant thresholds. Co-occurring ADHD symptoms accounted for increased autism questionnaire scores. Adaptive behavior in participants with NF1 showed normal socialization but lower communication proficiency.

Submandibular duct ligation after botulinum neurotoxin A treatment of drooling in children with cerebral palsy

AimTo assess: (1) the effect on drooling of bilateral submandibular duct ligation as surgical therapy after the administration of submandibular botulinum neurotoxin A (BoNT‐A) for excessive drooling and (2) the predictive value of treatment success with BoNT‐A on treatment success after bilateral submandibular duct ligation.MethodThis was a within‐participant retrospective observational study in which 29 children with severe drooling (15 males, 14 females) received BoNT‐A treatment at a mean age of 9 years 6 months (SD 2y 5mo), followed by bilateral submandibular duct ligation at a mean age of 10 years 11 months (SD 2y 4mo). Fifteen children were diagnosed with cerebral palsy (CP), with 12 children classified in Gross Motor Function Classification System levels IV and V. The 14 children without CP had non‐progressive developmental disorders. The primary drooling severity outcomes were the Visual Analogue Scale (VAS; subjective assessment) and drooling quotient (objective assessment). Measurements were taken before each intervention and again at 8 and 32 weeks.ResultsThe VAS was significantly lower after bilateral submandibular duct ligation at follow‐up compared to BoNT‐A treatment (mean difference −33, p≤0.001; 95% confidence interval [CI]=−43.3 to −22.9). The mean drooling quotient did not significantly differ between BoNT‐A treatment and bilateral submandibular duct ligation at follow‐up (3.3, p=0.457; 95% CI=−4.35 to 9.62) or between 8 and 32 weeks (4.7, p=0.188; 95% CI=−2.31 to 11.65).InterpretationBoNT‐A treatment and bilateral submandibular duct ligation are both effective treatment modalities for drooling. At 32‐week follow‐up, subjective drooling severity after bilateral submandibular duct ligation was significantly lower compared to previous BoNT‐A injections in participants. However, treatment success with BoNT‐A is no precursor to achieving success with bilateral submandibular duct ligation.What this paper adds Bilateral submandibular duct ligation is an effective therapy for drooling after treatment with botulinum neurotoxin A (BoNT‐A).Treatment success with BoNT‐A is not a predictor of successful therapy with bilateral submandibular duct ligation.

Psychosocial and functional outcomes in young adults with childhood-onset epilepsy: a 10-year follow-up.

To compare long-term psychosocial and functional outcomes of young adults with uncomplicated childhood-onset epilepsy (COE) to population norm controls utilizing a controlled prospective cohort study. Psychosocial and functional outcomes were assessed at 10-year follow-up. Fifty-three young adults (27 males, 26 females) with COE (n=21 remission; 18y 1mo-30y 9mo; mean age 23y 4mo [SD 3y 4mo]; mean age of epilepsy onset 12y [SD 3y 2mo]) were compared to 55 (23 males, 32 females) first-degree cousin controls (18y 5mo-29y 8mo; mean age 23y 6mo [SD 3y]). Seizure remission status and baseline comorbidities (attention-deficit/hyperactivity disorder [ADHD], depressive disorders, anxiety disorders, and academic problems) were examined as possible risk factors for significant differences in functional outcomes. Poorer functional outcomes, indicated by patient rated cognition and overall disability, were evident among young adults with epilepsy compared to controls (all p<0.05). These difficulties were due to baseline comorbid ADHD and academic problems. Remission status was not related to measured cognition and overall disability. Psychosocial outcomes of young adults with COE were similar to controls. In contrast, functional outcomes were worse in epilepsy across cognition and overall disability. Baseline comorbid ADHD and academic problems were identified as risk factors at 10-year follow-up suggesting that these early recognized comorbidities at or near diagnosis have long-term impacts. Young adults with childhood-onset epilepsy (COE) and controls have similar psychosocial outcomes 10 years after diagnosis. Young adults with COE report greater limitations in cognition and overall disability than controls. Baseline presence of attention-deficit/hyperactivity disorder and academic problems significantly affect cognitive and overall disability scores.

What predicts the proxy-reported health-related quality of life of adolescents with cerebral palsy in Bangladesh?

BackgroundThe health-related quality of life (HRQoL) of adolescents with CP in low and middle-income countries is often poor, as is the case in Bangladesh. This exploratory study examined what factors predict the proxy-reported HRQoL of adolescents with CP in rural Bangladesh, a typical low- and middle-income country (LMIC).MethodsAdolescents with CP (10 to 18y) were identified using the Bangladesh Cerebral Palsy Register. HRQoL was assessed using the Cerebral Palsy Quality of Life-Teens proxy-report questionnaire (CPQoL-Teens), adolescent mental health using the Strengths and Difficulty Questionnaire (SDQ) and caregiver mental health using the Depression, Anxiety and Stress Scale (DASS-21). Theoretical and statistical interests (i.e. bivariate analysis, p < 0.05) identified potential predictors which were entered into hierarchical multiple linear regression (HMLR) models in order of clinical significance; HMLR related adolescent clinical characteristics, adolescent and caregiver mental health and proxies of socioeconomic status to CPQoL-Teens dimensions.ResultsOne hundred fifty-four adolescents with CP (mean age 15y 1mo, SD 1y 8mo, female 31.2%) participated in this study.Twenty-four factors were identified to explore for relationship to adolescent proxy-reported HRQoL. Fifteen of the factors correlated to one or more CPQoL-Teens dimension; strongest correlation was between ‘feelings about functioning’ and motor impairment (r = 0.545). Nine were predictive of CPQoL-Teens dimensions; adolescent sex, school attendance, severity of motor impairment, hearing and speech impairment, mother’s education, primary caregiver depression and stress, and having a sanitary latrine at home resulting in score changes of between 0.79 (95% CI 0.24 to 1.35) to 35.1 (95% CI 6.03 to 64.22).ConclusionsMany of the factors predicting the proxy-reported HRQoL of adolescents with CP are amenable to intervention, and have the potential to improve adolescent wellbeing. Several determinants are priorities of the sustainable development goals (SDGs); these findings should inform resource prioritization to improve the wellbeing of adolescents with CP in Bangladesh and other LMICs.

Validity and reliability of the Dynamic Gait Index in children with hemiplegic cerebral palsy

BackgroundDynamic Gait Index (DGI) is a performance-based tool can be applied in a short time and evaluates dynamic balance and gait ability. Research questionIs the DGI valid and reliable for assessing gait and balance disorders in children with hemiplegic cerebral palsy (CP)? MethodsSixteen children with hemiplegic CP (5 females, 11 males; mean age 10y 3mo, SD 2y 7mo; range 6–14y; Gross Motor Function Classification System (GMFCS) levels I [n = 9], II [n = 7]) and 16 age-matched typically developing (TD) (8 females, 8 males; mean age 9y 9mo, SD 2y 6mo; range 6–14y) participated. The relationship between the DGI, Four-Square Step Test (FSST), Timed Up and Go Test (TUG) and Pediatric Berg Balance Scale (PBS) was analyzed. To determine the test-retest reliability, the DGI was performed twice and; for the inter-rater reliability, only DGI was reapplied by a different rater on the same day. Internal consistency was obtained by Cronbach-α value. Validity was tested by Spearman correlation coefficient and reliability was calculated by Intraclass correlation coefficient (ICC). ResultsThere was a significant difference between hemiplegic CP and TD and between the children with GMFCS level I and II in the comparison of results of the DGI and other tests. All items on the DGI had appropriate internal consistency (Cronbach-α = 0.969). The test-retest (ICC = 0.970 CI(0.915- 0.990)) and inter-rater (ICC = 0.983 CI(0.882- 0.998)) reliabilities were found to be excellent. A negative, moderate correlation between FSST and DGI (rs = −0.673, p = 0.004); a positive, high correlation between PBS (rs = 0.724, p = 0.002) and DGI and a negative, high correlation between TUG and DGI (rs = −0.828, p < 0.001) was detected. SignificanceDGI with features such as its feasibility in a short time, being simple but distinctive and not requiring heavy equipment is a valid and reliable method in children with hemiplegic CP.

Functional parameter measurements in children with ataxia telangiectasia.

To collect preliminary functional data on ataxia telangiectasia and create a disease specific scale: the Ataxia Telangiectasia Functional Scale (ATFS). Retrospective information on patients with ataxia telangiectasia referred to the Assistive Technology Unit was included. Functional mobility scales (the Gross Motor Function Classification System [GMFCS] and the Functional Mobility Scale [FMS]-5, FMS-50, FMS-500) and activities of daily living [ADL] parameters were recorded. We created a 51-point ATFS, that consisted of three ambulation items adapted for ataxia telangiectasia in the frame of FMS (home, school, outdoors), five ADL items, and one schooling item. Twenty-seven participants (17 males, 10 females; mean age 10y 8mo [SD 5y 1mo], range 1y 9mo-25y 6mo), were enrolled; 168 measurements were recorded. Patients walked at a mean age of 1year 4months (SD 5y 4mo) and lost ambulatory capacity at 8years 8months (SD 2y 1mo). GMFCS level and FMS-5, FMS-50, FMS-500 assessments correlated with age (Spearman's correlations r=0.555, -0.617, -0.639, -0.662 respectively, p<0.01 for all), but plateaued after 12years of age. ATFS mean score was 37.46 (SD 7.88) and increased with age (Spearman's correlation r=0.585, p<0.01). The scale showed threestages of disease progression. In this pilot study we show longitudinal functional data of ambulation and ADL skills in ataxia telangiectasia, and created a framework for a functional scale. This functional scale closely approximated disease course, but further validation is required. The Gross Motor Function Classification System and the Functional Mobility Scale are ill-suited for ataxia telangiectasia assessments. Three functional mobility scales (home, school, outdoors) suited to ataxia telangiectasiawere created. The Ataxia Telangiectasia Functional Scale (ATFS) combines mobility and items of activities of daily living. The ATFS closely approximates the three-stage progression of the disorder.