Mean Age 10y Research Articles

Relationship between cognitive dysfunction, gait, and motor impairment in children and adolescents with neurofibromatosis type 1

Motor skill impairment and cognitive dysfunction are commonly reported features of neurofibromatosis type 1 (NF1). We characterized and determined the relationship between motor impairment, gait variables, and cognitive function in children and adolescents with NF1. Motor function, gait, and neurocognitive abilities were assessed in 46 children and adolescents with NF1 (26 males, 20 females; age range 7-17 y; mean age 11 y 1 mo, SD 3 y 2 mo). Tests to establish correlations between neurocognitive, motor, and gait variables were performed. Compared with normative data, 28/39 of our NF1 cohort demonstrated impaired performance for balance and upper limb coordination and 16/38 for running speed and agility. Gait data revealed a strategy to preserve balance at the expense of velocity, with the unexpected exception of a tendency for reduced base of support. Neurocognitive testing confirmed mean IQ in the low average range (86.0) and deficits in spatial working memory and strategy generation. Significant correlations between a number of neurocognitive measures and motor abilities and gait were identified. The largest associations were between gait width and spatial working memory (r=0.594) and running speed and agility with strategy generation (r=0.549). We have identified a relationship between balance, running speed and agility, gait, and cognition in children with NF1. Findings suggest a shared abnormal neurodevelopmental process underlying some cognitive and motor abilities in NF1. Results are discussed within the context of evidence highlighting abnormal dopamine-mediated corticostriatal circuitry in NF1.

The inadequacy of the Y-axis of growth (SNGn) for the vertical pattern assessment in patients with sagittal discrepancies.

The aim of this cephalometric study was to evaluate the influence of the sagittal skeletal pattern on the 'Y-axis of growth' measurement in patients with different malocclusions. Lateral head films from 59 patients (mean age 16y 7m, ranging from 11 to 25 years) were selected after a subjective analysis of 1630 cases. Sample was grouped as follows: Group 1 - class I facial pattern; group 2 - class II facial pattern; and Group 3 - class III facial pattern. Two angular measurements, SNGoGn and SNGn, were taken in order to determine skeletal vertical facial pattern. A logistic regression with errors distributed according to a binomial distribution was used to test the influence of the sagittal relationship (Class I, II, III facial patterns) on vertical diagnostic measurement congruence (SNGoGn and SNGn). RESULTS show that the probability of congruence between the patterns SNGn and SNGoGn was relatively high (70%) for group 1, but for groups II (46%) and III (37%) this congruence was relatively low. The use of SNGn appears to be inappropriate to determine the vertical facial skeletal pattern of patients, due to Gn point shifting throughout sagittal discrepancies. Clinical Significance: Facial pattern determined by SNGn must be considered carefully, especially when severe sagittal discrepancies are present.

Changes in saccadic eye movement and memory function after mild closed head injury in children

The aim of this study was to determine whether volitional saccadic impairments are present in children with mild closed head injury (mCHI) and whether these deficits are predictive of ongoing cognitive impairment. We analysed a sample of 26 children with mCHI (20 males, 6 females; mean age 13y 1mo, SD 2y) and 29 age-matched comparison children (20 males, 9 females; mean age 12y 2mo, SD 2y). Participants completed a battery of saccadic eye movement tasks and a set of computer-based cognitive tasks at three time points: within 2 weeks of mCHI, and at 3 months and 6 months. The group with mCHI made fewer errors on the antisaccade task at the first time point and showed increased latencies on prosaccades, correct antisaccades, and corrected antisaccade errors at the third time point (6mo). The group with mCHI also showed poorer performance on the cognitive tasks assessing memory. Even very mild, uncomplicated mCHI in children may persistently affect aspects of executive control and visual processing.

Accuracy of skinfold and bioelectrical impedance assessments of body fat percentage in ambulatory individuals with cerebral palsy

This study assessed the accuracy of measurements of body fat percentage in ambulatory individuals with cerebral palsy (CP) from bioelectrical impedance analysis (BIA) and skinfold equations. One hundred and twenty-eight individuals (65 males, 63 females; mean age 12y, SD 3, range 6-18y) with CP (Gross Motor Function Classification System [GMFCS] levels I (n=6), II (n=46), and III (n=19) participated. Body fat percentage was estimated from (1) BIA using standing height and estimated heights (knee height and tibial length) and (2) triceps and subscapular skinfolds using standard and CP-specific equations. All estimates of body fat percentage were compared with body fat percentage from dual-energy X-ray absorptiometry (DXA) scans. Differences between DXA, BIA, and skinfold body fat percentage were analyzed by comparing mean differences. Agreement was assessed by Bland-Altman plots and concordance correlation coefficients (CCC). BMI was moderately correlated with DXA (Pearson's r=0.53). BIA body fat percentage was significantly different from DXA when using estimated heights (95% confidence intervals [CIs] do not contain 0) but not standing height (95% CI -1.9 to 0.4). CCCs for all BIA comparisons indicated good to excellent agreement (0.75-0.82) with DXA. Body fat percentage from skinfold measurements and CP-specific equations was not significantly different from DXA (mean 0.8%; SD 5.3%; 95% CI -0.2 to 1.7) and demonstrated strong agreement with DXA (CCC 0.86). Accurate measures of body fat percentage can be obtained using BIA and two skinfold measurements (CP-specific equations) in ambulatory individuals with CP. These findings should encourage assessments of body fat in clinical and research practices.

Mastery motivation in children with congenital hemiplegia: individual and environmental associations

The aim of this study was to examine the relationship between mastery motivation and individual and environmental characteristics in school-aged children with congenital hemiplegia. Forty-eight child-caregiver dyads (children's mean age 7y 11mo, SD 2y 4mo; 33 males, 15 females; Manual Ability Classification System [MACS] level I, n=25, and level II, n=23; predominant motor type spastic hemiplegia, n=47) were recruited to this cross-sectional study. Children were assessed using the Melbourne Assessment of Unilateral Upper Limb Function (MUUL) and the Assisting Hand Assessment. Caregivers completed the Dimensions of Mastery Questionnaire, the Parenting Scale, and a demographic questionnaire. Consistent and positive parental disciplinary practices were associated with higher total motivation (p=0.006) and instrumental aspect scores (p=0.009). Children with siblings and from single-parent families experienced greater negative reactions to failure (p=0.006). Children from two-parent families (p=0.018) and with better bimanual performance (p=0.015) demonstrated greater object-oriented persistence. Age, sex, limitations in manual ability (MACS), and movement and body function of the impaired limb (MUUL) did not contribute significantly to mastery motivation. Inconsistent, excessively lax, and verbose parenting practices may discourage children from persevering with challenging tasks. Functional parenting styles, positive discipline practices, and autonomy-supportive strategies for task engagement should be encouraged when intervening with children with cerebral palsy. Parents should be supported to engage in these practices in all aspects of daily activities.

Postural control and isometric strength of leg extensor muscle in moderately physically active young and elderly men

not described. The aim of this study was to describe standing posture in relation to joint restrictions in children with bilateral CP. Patients/materials and methods: Standing posture in 26 children with CP, mean age 11y (SD 3y), and 19 typically developing children, was recorded for 30 s with 3D motion analysis. Fifteen children with ability to stand without support were designated CP-Group A (GMFCS I-III), and 11 who needed support for standing CP-Group B (GMFCS III-IV). All children were tested with their habitual footwear. Passive joint range of motion (ROM) was measured with a goniometer. Lower limb contractures were defined as passive ROM less than neutral joint position. Results: Unior bilateral joint contractures were equally presented among the children in both CP groups A and B; in the hip (4 vs 7), the knee (6 vs 9), and in the ankle (2 vs 3). CP-Group B presentedwith significantly less passive joint ROM than CPGroup A in hip extension (−4◦(SD 5◦) vs 1◦(SD 5◦), p=0.022), knee extension (−12◦(SD 10◦) vs 0◦(SD 6◦), p≤0.001), and ankle dorsiflexion (2◦(SD 13◦) vs 7◦(SD 7◦), p=0.009) (Fig. 1). Children in bothCP groupsA andB stoodwith significantlymore flexion than their passive jointROMin thehip (p≤0.001vsp=0.004 respectively), in the knee (p=0.003 vs p=0.003), and in the ankle in CP Group A (p=0.012 vs p=0.182) (Fig. 1). Discussion and conclusions: The flexed joint position during standing with respect to the passive joint extension ROM in both CP groups indicates that the children did not utilize their possible joint extensionROMduring upright standing. Kneeflexor spasticity was observed in both groups and might have contributed to difficulties to erect their lower limbs. The large difference between the joint position in the unloaded and the upright positions, in particular in the children who needed support (CPGroup B), might also be interpreted as lower limbmuscle weakness. Which factors contribute to the difficulties to align the body segments and maintain a stable body position with respect to gravity during standing in children with bilateral CP requires further exploration.

One-Minute Walk and modified Timed Up and Go tests in children with cerebral palsy: performance and minimum clinically important differences.

AIM This prospective multicenter study assessed performance and changes over time, with and without surgical intervention, in the modified Timed Up and Go (mTUG) and One-Minute Walk tests (1MWT) in children with bilateral cerebral palsy (CP). Minimum clinically important differences (MCIDs) were established for these tools. METHOD Two hundred and nineteen participants with bilateral spastic CP (Gross Motor Function Classification System [GMFCS] levels I–III) were evaluated at baseline and 12 months follow-up. The non-surgical group (n=168; 54 females, 114 males; mean age 12y 11mo, [SD 2y 7mo], range 8y 1mo–19y) had no surgical interventions during the study. The surgical group (n=51; 19 females, 32 males; mean age 12y 10mo [SD 2y 8mo] range 8y 2mo–17y 5mo) underwent soft-tissue and/or bony procedures within 12 months from baseline. The mTUG and 1MWT were collected and MCIDs were established from the change scores of the non-surgical group. RESULTS Dependent walkers (GMFCS level III) required more time to complete the mTUG (p≤0.01) than independent walkers (GMFCS levels I and II). For the 1MWT, distance walked decreased with increasing impairment (p≤0.01). 1MWT and mTUG change scores were not significantly different at any GMFCS level for either the surgical or non-surgical groups (p≤0.01). INTERPRETATION Children with varying levels of function (GMFCS level) perform differently on the 1MWT and mTUG. The data and MCID values can assist clinicians in interpreting changes over time and in assessing interventions.

The role of serotonin receptor alleles and environmental stressors in the development of post‐concussive symptoms after pediatric mild traumatic brain injury

To determine whether post-injury depressive symptoms, and pre-injury major life stressors and genetic factors (HTR1A C(-1019)G alleles; rs6295) are more common in children with mild traumatic brain injury (mTBI) who develop postconcussion syndrome (PCS) symptoms compared with children with asymptomatic mTBI. This was a cross-sectional study of 47 symptomatic children (32 males, 15 females; mean age 14y [SD 3y 3mo]) who experienced post-concussive symptoms for 7 or more days and 42 asymptomatic children (26 males 16 females; mean age 13y 6mo [SD 3y 1mo]) after mTBI. Outcome measures were the Postconcussion Symptoms Inventory (PCSI), the Children's Depression Inventory (CDI), standard questionnaire of previous life events, and buccal DNA analysis to determine genotype and allele frequencies for the HTR1A C(-1019)G polymorphism. Depressive symptoms were uncommon. CDI scores did not differ between groups. Allelic and genotypic frequencies for HTR1A C(-1019)G were similar in both groups. Symptomatic children continued to have elevated PCS scores compared with asymptomatic children 1.72 (SD 0.69) years later and had experienced significantly more life stressors (Wald (1)=8.51, p=0.004). HTR1A polymorphisms do not differ in children with PCS. Children who have experienced more significant life stresses are more likely to develop PCS symptoms after mTBI.

Classification of intellectual disability using the Wechsler Intelligence Scale for Children: Full Scale IQ or General Abilities Index?

We examined the implications of using the Full Scale IQ (FSIQ) versus the General Abilities Index (GAI) for determination of intellectual disability using the Wechsler Intelligence Scales for Children, fourth edition (WISC-IV). Children referred for neuropsychological assessment (543 males, 290 females; mean age 10y 5mo, SD 2y 9mo, range 6-16y) were administered the WISC-IV and the Adaptive Behavior Assessment System, second edition (ABAS-II). GAI and FSIQ were highly correlated; however, fewer children were identified as having intellectual disability using GAI (n=159) than when using FSIQ (n=196). Although the 44 children classified as having intellectual disability based upon FSIQ (but not GAI) had significantly higher adaptive functioning scores than those meeting intellectual disability criteria based upon both FSIQ and GAI, mean adaptive scores still fell within the impaired range. FSIQ and GAI were comparable in predicting impairments in adaptive functioning. Using GAI rather than FSIQ in intellectual disability diagnostic decision-making resulted in fewer individuals being diagnosed with intellectual disability; however, the mean GAI of the disqualified individuals was at the upper end of criteria for intellectual impairment (standard score 75), and these individuals remained adaptively impaired. As GAI and FSIQ were similarly predictive of overall adaptive functioning, the use of GAI for intellectual disability diagnostic decision-making may be of limited value.

Executive function in children and adolescents with unilateral cerebral palsy

The aim of this study was to compare executive function in children with left- and right-sided unilateral cerebral palsy (CP) and typically developing children. There was a cross-sectional cohort of 46 children with unilateral CP (24 right-side, 22 left-side; 25 males, 21 females; mean age 11y 1mo, SD 2y 5mo) and 20 typically developing children (nine males, 11 females; mean age 10y 10mo, SD 2y 4mo). Four cognitive domains of executive function were assessed: attentional control, cognitive flexibility, goal setting, and information processing. Subtests from the Delis-Kaplan Executive Function System, the Test of Everyday Attention for Children, the Rey-Osterrieth Complex figure, and the Wechsler Intelligence Scale for Children - Fourth Edition were utilized. Between-group differences (right unilateral CP, left unilateral CP, and typically developing children) were examined using analyses of covariance. Children with CP performed significantly more poorly than typically developing children on all executive function measures (aggregate executive function: F(1,63)=31.16; p<0.001; η(2) =0.33). There were no significant differences between children with left and right unilateral CP, except in the case of inhibition/switching total errors, with children with left unilateral CP making fewer errors than children with right unilateral CP (F(1,39)=4.14; p=0.049; η(2) =0.1). Children and adolescents with unilateral CP experience difficulties across multiple executive function domains compared with typically developing children, irrespective of the side of hemiplegia. This finding supports an early vulnerability model of early brain injury and has implications for intervention for children with CP.

Muscle volume alterations in spastic muscles immediately following botulinum toxin type‐A treatment in children with cerebral palsy

With evidence for an atrophic effect of botulinum toxin type A (BoNT-A) documented in typically developing muscles, this study investigated the immediate morphological alterations of muscles in children with cerebral palsy (CP) after BoNT-A treatment. Fifteen children (10 males, five females; age range 5-11y, mean age 8y 5mo, SD 1y 10mo) with spastic diplegic CP [Gross Motor Function Classification System Levels I (n=9) and II (n=6)] receiving BoNT-A injections for spasticity management were included. None of the children was a first-time receiver of BoNT-A. Magnetic resonance imaging and Mimics software assessed muscle volume, timed 2 weeks before and 5 weeks after injection. All participants received BoNT-A bilaterally to the gastrocnemius muscle, and five participants also received BoNT-A bilaterally to the medial hamstring muscles. Functional assessment measures used were the 6-Minute Walk Test (6-MWT), the Timed Up and Go (TUG) test, and hand-held dynamometry. Whilst total muscle group volume of the injected muscle group remained unchanged, a 4.47% decrease in the injected gastrocnemius muscle volume (p=0.01) and a 3.96% increase in soleus muscle volume (p=0.02) was evident following BoNT-A. There were no statistically significant changes in function after BoNT-A as assessed by the TUG. There was also no statistically significant change in distance covered in the 6-MWT. Muscle strength, as assessed using hand-held dynamometry was also not statistically different after BoNT-A treatment. Muscle volume decreases were observed in the injected muscle (gastrocnemius), with synergistic muscle hypertrophy that appeared to compensate for this decrement. The 4% to 5% decrease in the volume of BoNT-A injected muscles are not dramatic in comparison to reports in recent animal studies, and are a positive indication for BoNT-A, particularly as it also did not negatively alter function.

The reliability and validity of a research‐grade pedometer for children and adolescents with cerebral palsy

The aim of this study was to determine the reliability, validity, and optimal placement of pedometers in children with cerebral palsy (CP) who ambulate without aids. Seventeen participants aged 7 to 17 years with CP (eight males, nine females; mean age 12y 4mo; SD 3y 2mo), who could ambulate without aids, wore four New Lifestyles pedometers (NL-1000) on an elasticized waist belt. Fourteen participants had hemiplegia, two diplegia, and one triplegia; all were classified in Gross Motor Function Classification System (GMFCS) level I (n=8) or II (n=9). Participants completed 3-minute walking and running trials around an indoor course and were videotaped to verify the actual number of steps taken during each trial. Inter-pedometer reliability was determined by comparing pedometer readings using intraclass correlation coefficients (ICCs). Validity was determined by comparing pedometer step counts with video step counts using ICC, t-tests, and Bland-Altman plots. Optimal pedometer placement was determined using Wilcoxon signed-rank tests to compare the percentage error for pedometers positioned on the dominant and non-dominant hips. Excellent reliability (ICC 0.88-0.99) and validity (ICC 0.78-0.95) were demonstrated with no significant difference between the video step counts and pedometer step counts. There was no significant difference between the step counts recorded by pedometers on the dominant and non-dominant hips. This study showed that NL-1000 pedometers have a high degree of reliability and validity in ambulant children with CP in controlled conditions.

Epilepsy in Prader–Willi syndrome: experience of a national referral centre

The aim of the study was to characterize epilepsy, febrile seizures, electrographic features, and brain abnormalities in a large, national cohort of individuals with Prader-Willi syndrome (PWS). This was an observational cohort study. Clinic charts of 126 individuals (63 males, 63 females) with genetically confirmed PWS (due to a deletion in 72 cases, to uniparental disomy [UPD] in 51 cases, and to an imprinting centre defect in two cases), aged from 1 month to 48 years (mean age 13y), were reviewed and 119 interviews conducted. Information regarding seizures, medication, imaging studies, and family history of seizures was collected. Ninety-five individuals (aged 1mo-48y) underwent electroencephalography (EEG). Five individuals had epilepsy (4.0%), three of whom had major cerebral findings on imaging, and eight others had febrile seizures (6.4%). Of the three genetic abnormalities, deletion was associated with seizures. Focal epileptiform abnormalities were found in 12 out of 94 individuals, and five out of these 12 had a frank electrographic seizure pattern. Epileptogenic EEG abnormalities were associated with young age. The risk of epilepsy and febrile seizures in PWS is significantly lower than in Angelman syndrome and is associated with brain abnormalities. Electrographic seizures and focal epileptiform activity were present in 5% of individuals and were associated with young age. The underpinnings of epileptiform abnormalities in PWS and how they differ from those of the Angelman syndrome should be studied further.

G187(P) Causes of Severe Anaemia (Hb

Severe childhood anaemia risks significant morbidity and mortality though may have different benign or malignant aetiologies. We set out to define the characteristics of this group of patients at our...

Ventilatory function in children with severe motor disorders using night‐time postural equipment

Night-time postural equipment (NTPE) can prevent hip subluxation in children with severe motor disorders (SMDs). However, it is unclear how it affects ventilatory function. The aims of the study were to determine how NTPE use affects ventilatory function and to compare night-to-night variability of ventilatory function in children with SMDs and typically developing healthy children. Fifteen NTPE users (six males, nine females), aged 1 to 19years (mean age 8y 7mo) alternated sleep condition between NTPE and sleeping unsupported for 14 nights. In all but two participants, gross motor function was classified as Gross Motor Function Classification System (GMFCS) level V; in the other two it was level IV. Oxyhaemoglobin saturation (SpO2 ) was monitored each night and transcutaneous CO2 (PtcCO2 ) for one night in each sleep condition. In 17 healthy children of similar age, home SpO2 only was monitored for seven nights. In 13 of 15 NTPE users and 12 of the 17 typically developing children, SpO2 monitoring was satisfactorily completed. Of the children with SMDs, two had mean SpO2 levels below the treatment threshold for supplemental oxygen, which was uniquely associated with use of NTPE in only one participant, and three had nocturnal hypoventilation, which was uniquely associated with NTPE use in only one case. Night-to-night SpO2 variability was higher in children with SMDs than in typically developing children. NTPE may impair or enhance ventilatory function in a minority of children. Owing to night-to-night variability in SpO2 , at least three nights of monitoring are recommended to determine optimal positioning for effective ventilation before and after NTPE introduction.

Rett syndrome: an eye‐tracking study of attention and recognition memory

The aim of this study was to examine attention and recognition memory for faces and patterns in Rett syndrome, a severely disabling neurodevelopmental disorder caused by mutations in the X-linked MECP2 gene. Because Rett syndrome impairs speech and hand use, precluding most neuropsychological testing, the visual paired-comparison paradigm (VPC) was used, together with eye tracking. In the VPC, two identical stimuli are presented for familiarization. On test, the familiar stimulus and a new one are paired, and recognition inferred from preferential looking to the novel target. Attention is measured by looking time, gaze dispersion, and number/length of fixations. Twenty-seven female patients with Rett syndrome (mean age 10y 6mo; SD 6y 8mo, age range 2-22y) from the Rett clinic at a children's hospital were assessed in this study, along with 30 age- and sex-matched typically developing participants (outpatients from the same hospital). Although patients with Rett syndrome showed recognition of both faces and patterns, with novelty scores greater than chance (50%), their performance was significantly poorer than that of the typically developing comparison group. Their attention to both was less mature and marked by a more narrowly focused gaze, with fewer and longer fixations. When inspecting faces, attention to the eyes was similar in both groups; however, patients with Rett syndrome tended to ignore the nose and mouth. This is one of the first studies to characterize attention and memory in individuals with Rett syndrome. Visually based techniques, such as the VPC, open a new avenue for quantifying the cognitive phenotype associated with this syndrome.

The impact of methylphenidate on seizure frequency and severity in children with attention‐deficit–hyperactivity disorder and difficult‐to‐treat epilepsies

Difficult-to-treat epilepsies and attention-deficit-hyperactivity disorder (ADHD) often co-occur. Because of concerns about the use of stimulants in children with this comorbidity, the impact of ADHD treatment on seizure frequency and severity is not known. This pilot study evaluated the safety and efficacy of methylphenidate in this population. After a 3 month period in which antiepileptic drugs were adjusted, 22 patients recruited from a specialist outpatient clinic for severe epilepsy (16 males, six females; mean age 11 y 2 mo, SD 3 y 2 mo) received methylphenidate for 3 months in an open label, non-controlled trial; four with generalized or multifocal (symptomatic/cryptogenic) epilepsy, one with generalized (idiopathic) epilepsy, 17 with partial (symptomatic/cryptogenic) epilepsy; five with partial seizures only, 17 with primarily or secondarily generalized seizures). Epilepsy, ADHD symptoms, and side effects were assessed using the Swanson, Nolan, and Pelham Questionnaire, the Child Behavior Checklist, the Hague Seizure Severity Scale, and the Side Effects Rating Scale. Methylphenidate significantly improved ADHD. After 3 months of treatment, 73% of patients no longer had clinically significant symptoms. Methylphenidate also reduced seizure severity (9-point median decrease on the Hague Seizure Severity Scale). Seizure frequency increased in four out of 22 patients, but only one patient withdrew from the study for this reason. Most patients experienced no major side effects. These data are among the first showing that low doses of methylphenidate are safe and effective to treat ADHD symptoms in patients with difficult-to-treat epilepsies. Randomized controlled trials are needed to replicate the findings.

A multi‐site study of functional outcomes following a themed approach to hand–arm bimanual intensive therapy for children with hemiplegia

This study investigated the effects of a theme-based ('magic') variation of the hand-arm bimanual intensive therapy programme, in two different countries, in improving activity performance for children with hemiplegia, including those with severe movement restrictions. Twenty-three children with spastic hemiplegia (13 males, 10 females; mean age 10y 7mo, range 7-15y; Manual Ability Classification System level I, two; level II, 13; level III, eight), participated in one of three, 2-week, summer camps. A within-participant experimental design was used with the Assisting Hand Assessment and Children's Hand Experience Questionnaire as primary outcome measures. Evaluations occurred immediately before the first day, on the last day, and 3months after intervention. Two groups underwent additional assessments 2weeks before the camp. Significant intervention effects were seen on the Assisting Hand Assessment (p=0.002) and on the Children's Hand Experience Questionnaire (p<0.001), the latter maintained at follow-up. The affected hand was reported to be used in 25% of bimanual activities before the camp, progressing to 93% after camp, and decreasing to 86% at follow-up. Severity of impairment did not influence progress. This themed approach to intensive intervention showed positive results in bimanual use, with improvements in independence sustained at follow-up. Although children across camps and motor severity made progress, some questions remain about intensity and duration of intervention to optimize longer-term outcomes.

Criterion validity of the GMFM‐66 item set and the GMFM‐66 basal and ceiling approaches for estimating GMFM‐66 scores

The aim of this study was to compare the accuracy of two abbreviated approaches for estimating Gross Motor Function Measure 66 (GMFM-66) scores against the full GMFM-66 and to explore their strengths and limitations. An existing dataset (n=224) comprising children aged 1 to 13years (mean age 6y 11mo, SD 4y 6mo; 132 males, 92 females) with cerebral palsy (CP) of all Gross Motor Function Classification System (GMFCS) levels was used to compare the validity of the item set version (GMFM-66-IS) and the basal and ceiling version (GMFM-66-B&C) with the full GMFM-66 scores. Follow-up assessment at 1year (n=109) allowed evaluation of change scores and accuracy at a single point in time. The cross-sectional agreement was excellent for both abbreviated measures (all intraclass correlation coefficients [ICCs] >0.98). When measuring change over time, both the GMFM-66-IS and the GMFM-66-B&C showed good agreement for children with bilateral CP (ICCs >0.9). However, the GMFM-66-IS assessed change over 1year more accurately than the GMFM-66-B&C in children with unilateral CP (ICC=0.89 vs ICC=0.58; 95% confidence intervals do not overlap). Both approaches for estimating GMFM-66 scores are accurate at a single point in time. If the primary goal of assessment is to measure change, the full GMFM-66 should still be regarded as the criterion standard. The GMFM-66-IS should be the preferred shortened measure for children with unilateral CP.

Intensive dysarthria therapy for younger children with cerebral palsy

The aim of this study was to investigate if intervention targeting breath support, phonation, and speech rate increases speech intelligibility and participation in the conversational interactions of younger children with dysarthria and cerebral palsy (CP). Fifteen children with dysarthria and CP (nine males, six females; age range 5-11 y, mean age 8 y, SD 2 y; CP type: eight spastic, four dyskinetic, one ataxia, two Worster Drought syndrome; Gross Motor Function Classification System levels II-IV, median level II) participated in this study. Children received three sessions of individual therapy per week for 6 weeks. Intelligibility of single words and connected speech was compared across five points: 1 and 6 weeks before therapy and 1, 6, and 12 weeks after therapy. Three familiar listeners and three unfamiliar listeners scored each recording. Participation in communicative interactions was measured using the Focus on the Outcomes of Communication Under Six (FOCUS) tool. Analyses of variance and paired t-tests were used to investigate change. Mean speech intelligibility increased after therapy to familiar listeners (single words 10.8%, 95% confidence interval [CI] 7.2-14.4; connected speech 9.4%, 95% CI 4.8-14.1) and unfamiliar listeners (single words 9.3%, 95% CI 6.8-11.8; connected speech 10.5%, 95% CI 7.3-13.8). FOCUS scores increased following therapy for parents (mean increase 30.3, 95% CI 10.2-50.4) and for teachers (28.25, 95% CI 14.4-42.1), but changes did not correlate with intelligibility. A wide variation was seen in individual responses to therapy. Brief intensive therapy is associated with gains in intelligibility and communicative interactions for some younger children with dysarthria.