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Aim: To evaluate presence and severity of social impairments in alternating hemiplegia of childhood (AHC) and determine factors that are associated with social impairments.Method: This was a retrospective analysis of 34 consecutive patients with AHC (19 females, 15 males; mean age: 9y 7mo, SD 8y 2mo, range 2y 7mo-40y), evaluated with the Social Responsiveness Scale, Second Edition (SRS-2).Results: SRS-2 scores, indicating level of social impairment, were higher than population means (75, SD 14 vs 50, SD 10, p<0.001). Of these, 27 out of 34 had high scores: 23 severe (>76), four moderate (66-76). All subscale domains, including social cognition, social communication, social awareness, social motivation, restricted interests, and repetitive behavior, had abnormal scores compared to population means (p<0.001). High SRS-2 scores were associated with the presence of autism spectrum disorder (ASD) and epilepsy (p=0.01, p=0.04), but not with other scales of AHC disease symptomatology. All nine patients who received formal evaluations for ASD, because they had high SRS-2 scores, were diagnosed with ASD.Interpretation: Most patients with AHC have impaired social skills involving multiple domains. ASD is not uncommon. High SRS-2 scores in patients with AHC support referral to ASD evaluation. Our findings are consistent with current understandings of the pathophysiology of AHC and ASD, both thought to involve GABAergic dysfunction.What This Paper Adds: Most patients with alternating hemiplegia of childhood (AHC) have impaired social skills involving multiple domains. These impairments are significant compared to population means. Most patients with AHC have high Social Responsiveness Scale, Second Edition (SRS-2) scores. Patients with AHC with high SRS-2 scores are likely to have autism spectrum disorder. [ABSTRACT FROM AUTHOR]

Aim: To characterize fatigue in children with hemiparesis with perinatal stroke and explore associations with measures of motor performance and corticospinal excitability.Method: Forty-five children (16 females, 29 males), aged 6 to 18 years (mean [SD] 12y [4]), with magnetic resonance imaging-confirmed perinatal stroke participated. Associations between fatigue (Pediatric Quality of Life Inventory Version 3.0 cerebral palsy module fatigue subscale), motor performance (Assisting Hand Assessment [AHA], Box and Blocks Test, grip strength), and excitability of corticospinal projections to both hands were examined using ranked tests of correlation, robust regression, and the Mann-Whitney U test.Results: Nearly half of the participants (n=21) reported experiencing fatigue in the previous month. Function in the less affected hand (Box and Blocks Test, grip strength) was correlated with fatigue scores. Participants with preserved ipsilateral projections to the more affected hand had less fatigue, and scores correlated with the excitability of these projections. Fatigue scores were not associated with age, sex, or AHA score.Interpretation: Fatigue is common in children with hemiparesis with perinatal stroke and is associated with motor performance and the presence and excitability of ipsilateral corticospinal projections from the contralesional hemisphere to the more affected hand.What This Paper Adds: Fatigue is common in children with hemiparesis with perinatal stroke. Fatigue was associated with motor performance and strength in the less affected, but not the more affected, hand. Fatigue was associated with the presence and excitability of ipsilateral corticospinal projections from the contralesional hemisphere. [ABSTRACT FROM AUTHOR]

Aim: To describe the development of the parent-rated Hand-Use-at-Home questionnaire (HUH) assessing the amount of spontaneous use of the affected hand in children with unilateral paresis, and to test its internal structure, unidimensionality, and validity.Method: Parents of children with unilateral cerebral palsy (CP) and professionals participated in the development of the HUH. To examine internal validity, data of 322 children (158 males, 164 females; mean age 6y 7mo, standard deviation [SD] 2y 1mo) with unilateral CP (n=131) or neonatal brachial plexus palsy (NBPP) (n=191) were collected. Rasch analysis was used to examine discriminative capacity of the 5-category rating scale as well as unidimensionality and hierarchy of the item set. Additionally, data of 55 children with typical development (24 males, 31 females; 6y 9mo, SD 2y 5mo) were used to examine construct validity.Results: The 5-category rating scale was disordered in all items and was collapsed to obtain the best discriminating sum score. Ten misfitting or redundant items were removed. Eighteen hierarchically ordered bimanual items fitted the unidimensional model within acceptable range. The HUH significantly discriminated between the three groups (children with typical development, NBPP, unilateral CP; H(2) =118.985, p<0.001), supporting its construct validity.Interpretation: The HUH is a valid instrument to assess the amount of spontaneous use of the affected hand in children with unilateral upper-limb paresis. [ABSTRACT FROM AUTHOR]

Aim: To characterize motor function profiles in alternating hemiplegia of childhood, and to investigate interrelationships between these domains and with age.Method: We studied a cohort of 23 patients (9 males, 14 females; mean age 9y 4mo, range 4mo-43y) who underwent standardized tests to assess gross motor, upper extremity motor control, motor speech, and dysphagia functions.Results: Gross Motor Function Classification System (GMFCS), Gross Motor Function Measure-88 (GMFM-88), Manual Ability Classification System (MACS), and Revised Melbourne Assessment (MA2) scales manifested predominantly mild impairments; motor speech, moderate to severe; Modified Dysphagia Outcome and Severity Scale (M-DOSS), mild-to moderate deficits. GMFCS correlated with GMFM-88 scores (Pearson's correlation, p=0.002), MACS (p=0.038), and MA2 fluency (p=0.005) and accuracy (p=0.038) scores. GMFCS did not correlate with motor speech (p=0.399), MA2 dexterity (p=0.247), range of motion (p=0.063), or M-DOSS (p=0.856). Motor speech was more severely impaired than the GMFCS (p<0.013). There was no correlation between any of the assessment tools and age (p=0.210-0.798).Interpretation: Our data establish a detailed profile of motor function in alternating hemiplegia of childhood, argue against the presence of worse motor function in older patients, identify tools helpful in evaluating this population, and identify oropharyngeal function as the more severely affected domain, suggesting that brain areas controlling this function are more affected than others. [ABSTRACT FROM AUTHOR]

Aim: To evaluate 'remind-to-move' (RTM) treatment by comparing it with constraint-induced movement therapy (CIMT) and conventional rehabilitation of the upper extremity in children with hemiplegic cerebral palsy (CP).Method: Seventy-three children (44 males, 29 females; mean age 11y 8mo, standard deviation [SD] 3y 1mo) - with 20, 38, and 15 in Manual Ability Classification System levels I, II, and III respectively - were recruited from three special schools and randomly selected for an RTM (n=25) or CIMT (n=24) programme (for 75h over 3wks) or for conventional rehabilitation (n=24). The Jebsen-Taylor Hand Function Test, the Bruininks-Oseretsky Test of Motor Proficiency (Subtest 3), the Caregiver Functional Use Survey, and arm movement duration captured by accelerometers were used at the baseline, post-test, and 1-month and 3-month follow-ups.Results: Both the RTM and CIMT treatments achieved significant gains in manual capacities and spontaneous hand use immediately after the intervention compared with conventional rehabilitation, but there were no significant differences between the two interventions.Interpretation: The RTM treatment demonstrated similar therapeutic effects with CIMT in manual dexterity and functional hand use, but both interventions were superior to conventional rehabilitation. RTM is recommended as an alternative treatment for the hemiplegic upper extremity in children with CP. [ABSTRACT FROM AUTHOR]

Aim: To determine the efficacy of mirror therapy in children with hemiparesis.Method: The design was an observer-blinded parallel-group randomized controlled trial (International Standard Randomised Controlled Trial Number 48748291). Randomization was computer-generated, 1:1 allocation to mirror therapy or comparison groups. The settings were home-based intervention and tertiary centre assessments. Participants were 90 children with hemiparesis aged 7 to 17 years. Intervention was 15 minutes per day of simultaneous arm training, 5 days a week, for 5 weeks. The mirror therapy group used a mirror; those in the comparison group looked at their paretic limb. Assessments comprised measures of upper limb strength, function (Melbourne Assessment 2), daily performance (ABILHAND-Kids), and sensory function at weeks 0 (T0 ), 5 (T1 ), and 10 (T2 ).Results: There were no significant differences in outcomes and their progression over time between the mirror therapy and comparison groups. Post-hoc intention-to-treat analyses showed significant improvements in both groups for grasp strength (T0 -T1 +12.6%), pinch strength (T0 -T2 +9.1%), upper limb function in terms of accuracy (T0 -T2 +2.7%) and fluency (T0 -T2 +5.0%), as well as daily performance (T0 -T2 +16.6%). Per protocol analyses showed additional improvements in dexterity (T0 -T2 +4.0%).Interpretation: The use of the mirror illusion during therapy had no significant effect on treatment outcomes. However, 5 weeks of daily simultaneous arm training significantly improved paretic upper limb strength, function, and daily use. [ABSTRACT FROM AUTHOR]

Aim: The ability to determine the relationship between selective motor control and upper extremity function in children with unilateral cerebral palsy (CP), and to measure the functional outcome and efficacy of interventions designed to improve selective motor control, has been limited by the lack of an objective, validated tool. The primary objective of this study is to describe the development of a clinical tool entitled Selective Control of the Upper Extremity Scale (SCUES), and present evidence of its validity and reliability.Method: Content validity was established through an expert panel (eight clinicians, mean and median of 17y of clinical experience, range 2-30y). Intra- and interrater reliability was determined by six occupational therapists who scored 10 participant studies. Construct validity of the SCUES was established by comparison to the spontaneous functional analysis section of the Shriners Hospitals Upper Extremity Evaluation, the Manual Ability Classification System, and the Box and Block test for 25 children with unilateral CP.Results: The content validity ratio values were greater than 0 (indicating >50% agreement) for 33 of the 34 items (97%), and equal or greater than 0.5 (indicating ≥75% agreement) for 26 of the 34 items (76%). Intrarater reliability was excellent (intraclass correlation coefficient [ICC] >0.75) for all segments and joints of the affected extremity. Interrater reliability was excellent for all segments and joints of the affected extremity except the shoulder (ICC=0.72). The SCUES was strongly correlated with the SHUEE (Spearman's rho=0.69, p=0.003). The SCUES was not correlated with the Manual Ability Classification System (rho=-0.24, p=0.369) or the Box and Block test (rho=0.47, p=0.066).Interpretation: Psychometric analysis of the SCUES revealed comparable validity to other accepted video-based clinical assessment tools for the upper extremity in children with CP. [ABSTRACT FROM AUTHOR]

Aim To determine the extent to which children's mastery motivation predicts occupational performance outcomes following upper limb intervention ( ULI). Method In this cohort study, participants received 45 hours of ULI, either in an intensive group-based or distributed individualized model. The Dimensions of Mastery Questionnaire ( DMQ) measured mastery motivation at baseline. Occupational performance outcomes were assessed at baseline and 13 weeks' post-intervention using the Canadian Occupational Performance Measure ( COPM). Multivariable models determined the contribution of mastery motivation to COPM outcome irrespective of group membership. Results Forty-two children with congenital hemiplegia (29 males, 13 females; mean age 7y 8mo [ SD 2y 2mo]; range 5y 1mo-12y 8mo; Manual Ability Classification System [ MACS] I=20 and II=22; predominant motor type unilateral spastic n=41) participated in the study. Significant gains were seen in COPM performance and satisfaction scores ( p<0.001) post-intervention with no between group differences. Children who had greater persistence with object-oriented tasks ( p=0.02) and better manual ability ( p=0.03) achieved higher COPM performance scores at 13 weeks. Children's persistence on object-oriented tasks was the strongest predictor of COPM satisfaction ( p=0.01). Interpretation Children's persistence with object-oriented tasks as well as manual abilities needs to be considered when undertaking ULI. Predetermining children's motivational predispositions can assist clinicians to tailor therapy sessions individually based on children's strengths, contributing to effective engagement in ULI. [ABSTRACT FROM AUTHOR]

Aim Hemiconvulsion-hemiplegia syndrome ( HHS) is a rare severe epilepsy of infancy consisting of unilateral convulsive status epilepticus immediately followed by transient or lasting ipsilateral hemiplegia. HHS may occur either in patients with previous brain pathology or without any identified cause, so-called 'idiopathic HHS'. Method We retrospectively analysed clinical and MRI longitudinal findings of a series of 10 patients (six females, four males) presenting with HHS. Age at the study inclusion ranged from 2 years 6 months to 15 years (mean of 5y 10mo, median 4y 2mo). After defining magnetic resonance imaging ( MRI) features as 'typical', i.e. strictly unilateral involvement, and 'atypical', i.e. bilateral, we compared clinical data from both groups. Cognitive level was assessed using Brunet-Lézine or Wechsler scales. Results HHS occurred at a mean age of 20.5 months (range 8-48mo). In all cases, status epilepticus lasted for more than 1 hour and was characterised by unilateral clonic seizures followed by ipsilateral hemiplegia (persistent in five patients). Two patients in this series died: the first from multi-organ failure 2 weeks after the status epilepticus and the other from a second episode of ipsilateral intractable febrile status epilepticus 3 years after the first episode. Early MRI (days 1-7 from status epilepticus) showed hemispheric cytotoxic oedema in all, extending to the contralateral side for one. T2 hyperintensity in the basal ganglia was disclosed in 70% of patients and in the hippocampus in 60%. After 1 month (in intermediate and chronic phases), all surviving patients but one showed hemispheric cortical atrophy corresponding to the regions involved during the early stage. Comparing clinical features of patients presenting with 'typical' features, to those with 'atypical' findings, the second group presented psychomotor delay before status epilepticus. Interpretation This series underlines the major value of early MRI for the prompt diagnosis of HHS, and shows that involvement of subcortical structures has been underestimated. Hippocampal involvement is not constant. [ABSTRACT FROM AUTHOR]

The article presents abstracts of various scientific posters on developmental medicine and neurology which include research on the reduction in thalamic volume in congenital hemiplegia, a study on using a sequential mixed method design to evaluate the independent living factors and perceived quality of life of adults with cerebral palsy and research on the possible contribution of stretched sarcomeres to contracture in cerebral palsy.

Aim The aim of this study was to determine if gait analysis improves correction of excessive hip internal rotation in ambulatory children with spastic cerebral palsy ( CP). Method Children undergoing orthopedic surgery were randomized to receive or not receive a preoperative gait analysis report. This secondary analysis included all participants whose gait report recommended external femoral derotation osteotomy ( FDRO). One-year postoperative, and pre- to postoperative change in femoral anteversion, mean hip rotation in stance, and mean foot progression in stance were compared between groups and in subgroups based on whether the recommendation for FDRO was followed. Results Outcomes did not differ between the group which received a gait report ( n=39; 19 males, 20 females; mean age 10y 4mo [ SD 3y]; hemiplegia, 3; di/triplegia, 28; quadriplegia, 8; Gross Motor Function Classification System [ GMFCS]: level I, 5; level II, 12; level III 19; level IV, 3) and the control group ( n=26; 14 males, 12 females; mean age 9y 5mo [ SD 2y 10mo]; hemiplegia, 1; di/triplegia, 21; quadriplegia, 4; GMFCS: level I, 4; level II, 1; level III, 9; level IV, 2; all p values >0.29), but improved more in the gait report subgroup in which the FDRO recommendation was followed (seven limbs; change in anteversion −32.9°, hip rotation −25.5°, foot progression −36.2°) than in the control group (anteversion −12.2°, hip rotation −7.6°, foot progression −12.4°; all p values ≤0.02) and the gait report subgroup in which FDRO was not performed (32 limbs; anteversion −1.0°, hip rotation 0.5°, foot progression −8.0°; all p values ≤0.003). Postoperative measures became normal only in the gait report subgroup in which the recommended FDRO was performed. Interpretation Gait analysis can improve outcomes when its recommendations are incorporated in the treatment plan. [ABSTRACT FROM AUTHOR]

Aim 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. Method 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. Results 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; η2=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; η2=0.1). Interpretation 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. [ABSTRACT FROM AUTHOR]

Aim Clinical determination of spasticity is confounded by the difficulty in distinguishing reflex from passive contributions to muscle stiffness. There is, therefore, a risk that children with cerebral palsy ( CP) receive antispasticity treatment unnecessarily. To investigate this, we aimed to determine the contribution of reflex mechanisms to changes in the passive elastic properties of muscles and tendons in children with CP. Method Biomechanical and electrophysiological measures were used to determine the relative contribution of reflex and passive mechanisms to ankle muscle stiffness in 35 children with spastic CP (21 males, 14 females; mean age 9y, SD 3y 4mo; range 3-15y) and 28 control children without CP (19 males, nine females; mean age 8y 11mo, SD 2y 10mo; range 3-15y). Twenty-seven children were diagnosed as having spastic hemiplegia, six with spastic diplegia, and two with spastic tetraplegia. According to the Gross Motor Function Classification System, 31 children were classified in level I, two in level II, and two in level III. Results Only seven children with spastic CP showed reflex stiffness outside the range of the control children. In contrast, 20 children with spastic CP showed abnormal passive muscle stiffness ( p<0.001). No correlation between increased reflex or increased passive muscle stiffness and age was observed within the age range studied. Interpretation These data suggest that increased reflex-mediated muscle stiffness is difficult to distinguish clinically from changes in passive muscle stiffness and that signs of changes in muscle properties are already present from the age of 3 years in children with CP. This emphasizes the importance of accurately distinguishing different contributions to muscle stiffness to avoid unnecessary antispasticity treatment. [ABSTRACT FROM AUTHOR]

Aim 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. Method 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 3 months after intervention. Two groups underwent additional assessments 2 weeks before the camp. Results 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. Interpretation 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. [ABSTRACT FROM AUTHOR]

Aim The purpose of this study was to evaluate a population-based radiographic hip surveillance programme for children with cerebral palsy (CP) and to assess the natural history of hip displacement. Method The study comprised 335 children (188 males, 147 females), born during 2002 to 2006 in the 10 south-eastern counties in Norway. Their mean age at the first radiograph was 3 years (range 6mo-7y 11mo) and the mean age at the most recent follow-up was 5 years 5 months. Distribution according to CP type was spastic hemiplegia in 38%, diplegia in 27%, quadriplegia in 21%, dyskinesia in 10%, and ataxia in 3%; Gross Motor Function Classification System (GMFCS) levels I to V were, 44%, 14%, 8%, 11%, and 23% respectively. Migration percentage (MP), acetabular index, and pelvic obliquity were measured on the radiographs. Results Hip displacement (MP>33%) occurred in 26% of all children (subluxation in 22% and dislocation in 4%) and in 63% of those in GMFCS levels IV or V. Dislocation occurred in 14 children at a mean age of 4 years 5 months (range 1y 10mo-9y 7mo). The mean migration percentage was 20.4% at the initial radiographs and 34.0% at the last follow-up. Mean progression in migration percentage increased markedly with decreasing functional level, from 0.2% per year at GMFCS level I to 9.5% at level V. Interpretation There is a pronounced trend towards hip displacement in nonambulant children. Close surveillance from age 1 to 2 years is needed to find the appropriate time for preventive surgery. Since 12% of the nonambulant children developed dislocation, our routines for hip surveillance need improvement. [ABSTRACT FROM AUTHOR]

Aim To determine the prevalence of symptomatic neurogenic bladder (SNB) and social and functional variables in a large sample of people with cerebral palsy (CP). Method The medical records of 214 individuals (96 females, 118 males) with CP between the years 1990 and 2000 were retrospectively reviewed. Individuals with frequency, urgency, or incontinence were assigned Gross Motor Function Classification System levels and underwent cystometrogram/electromyelogram studies. Neurogenic bladders were classified according to the nomenclature of the International Continence Society. Results Fifty-two patients had hemiplegia, 42 diplegia, 117 quadriplegia, and three dyskinesia. Educational levels ranged from full special education to those with graduate degrees. Thirty-five individuals in our group aged 5 to 66 years had SNB with a prevalence of 16.4%. The median age for the entire population was 9 years 7 months and for those with SNB 12 years 4 months (range 5-57y). Over 80% of individuals who underwent investigation were found to have spastic hyper-reflexic type bladders. Ninety-one percent obtained total continence or major improvement with conservative care. SNB was documented across the lifespan, educational spectrum, and functional level. Upper urinary tract pathology was infrequent. Interpretation SNB is a common finding in individuals with CP. In most patients it is readily diagnosed and treated with conservative interventions. [ABSTRACT FROM AUTHOR]

Aim The aim of this randomized controlled trial was to assess whether action observation treatment may improve upper limb motor functions in children with cerebral palsy (CP). Method All children with CP admitted to our unit for rehabilitation from May 2009 to May 2010 were eligible. Inclusion criteria were age between 6 years and 11 years, an IQ of at least 70, and no major visual and/or auditory deficits. Fifteen children were enrolled and randomly assigned to either a case group ( n=8; four males, four females; median age 7y 6mo) or control group ( n=7; five males, two females; median age 8y). Six participants had left-sided hemiplegia, six right-sided hemiplegia, and three had tetraplegia; 10 were independent walkers. Those in the case group were asked to observe video clips showing daily age-appropriate actions, and afterwards to imitate them. Participants in the control group were asked to observe video clips with no motor content and afterwards to execute the same actions as cases. The primary outcome measure was the Melbourne Assessment Scale. Children were scored twice at baseline (2wks apart), and at the end of treatment, by a physician blind to group assignment. Results At baseline groups did not differ on functional evaluation. After treatment, the functional score gain (Δ) was significantly different in the case and control groups ( p=0.026). Interpretation The present results support the notion that action observation treatment can be an effective part of the rehabilitation programme in children with CP. [ABSTRACT FROM AUTHOR]

Aim Structural connectivity analysis using diffusion magnetic resonance imaging (dMRI) and tractography has become the method of choice for studying white matter pathology and reorganization in children with congenital hemiplegia. To evaluate its role in the research domain, we systematically reviewed the literature about children with cerebral palsy (CP) to document common findings and identify strengths and possible limitations of this neuroimaging technology. Method A literature search was performed for peer-reviewed studies pertaining to dMRI and CP. Results Twenty-two studies met the inclusion criteria. The corticospinal tract was studied in greatest detail (18/22). The most common finding was decreased fractional anisotropy and/or increased mean diffusivity, indicating significant loss in the integrity of these corticomotor pathways. Fewer studies assessed ascending sensorimotor pathways including the posterior and superior thalamic radiations, which also showed decreased fractional anisotropy. Anisotropy indices (fractional anisotropy, mean diffusivity) obtained for both corticomotor and sensorimotor tracts were repeatedly shown to correlate with clinical measures. Other tracts studied included commissural and association fibres, which showed conflicting results. Interpretation There is sound evidence that dMRI-based connectivity techniques are useful for improving our understanding of the structure-function relationships of corticomotor and sensorimotor neural networks in CP. [ABSTRACT FROM AUTHOR]

Aim To describe the clinical and radiological features of four new families with a childhood presentation of COL4A1 mutation. Method We retrospectively reviewed the clinical presentation. Investigations included radiological findings and COL4A1 mutation analysis of the four cases. Affected family members were identified. COL4A1 mutation analysis was performed in all index cases and, where possible, in affected family members. Results The three male and one female index cases presented with recurrent childhood-onset stroke, infantile hemiplegia/spastic quadriplegia, and infantile spasms. Additional features such as congenital cataracts and anterior segment dysgenesis were present. Microcephaly and developmental delay/learning difficulties were present in three cases. Three cases had one or more family member affected in multiple generations, with a total of 11 such individuals identified. The clinical features showed a wide intrafamilial variation. Magnetic resonance imaging (MRI) showed bilateral white matter change in all cases, except in one mutation-positive family member. Unilateral or bilateral porencephaly was present in cases with infantile hemiplegia, and a diagnosis of clinical stroke was supported by the presence of intracerebral haemorrhage. The age at diagnosis was between 1 year and 6 years for the children with presentation in infancy and 12 months after stroke in a 14-year-old male. Three new pathogenic mutations were identified in the COL4A1 gene. Interpretation COL4A1 mutations can present in children with infantile hemiplegia/quadriplegia, stroke or epilepsy, and a motor disorder. The presence of eye features and white matter change on MRI in childhood can help point towards the diagnosis. Once the diagnosis is made, a careful search can identify affected family members. [ABSTRACT FROM AUTHOR]

A letter about prehensile force control while grasping and lifting an object in children with hemiplegic cerebral palsy (CP) is presented. Particular focus is given to a study which examined the hypothesis that anticipatory force coupling in the involved hand while walking with a hand-held object is impaired. Findings showed nearly identical average gait velocity and peak-to-peak inertial force (IF) difference while walking with hand-held object. The study also revealed impairments in the grip-inertial force coupling in the involved hand.

To determine if constraint-induced movement therapy (CIMT) is more effective than bimanual training (BIM) in improving upper limb activity outcomes for children with congenital hemiplegia in a matched-pairs randomized trial. Sixty-three children (mean age 10.2, SD 2.7, range 5-16y; 33 males, 30 females), 16 in Manual Ability Classification System level I, 46 level II, and 1 level III and 16 in Gross Motor Function Classification level I, 47 level II) were randomly allocated to either CIMT or BIM group day camps (60 hours over 10 days). The Melbourne Assessment of Unilateral Upper Limb Function assessed unimanual capacity of the impaired limb and Assisting Hand Assessment evaluated bimanual coordination at baseline, 3 and 26 weeks, scored by blinded raters. After concealed random allocation, there was no baseline difference between groups. CIMT had superior outcomes compared with BIM for unimanual capacity at 26 weeks (estimated mean difference [EMD] 4.4, 95% confidence interval [CI] 2.2-6.7; p<0.001). There was no other significant difference between groups post-intervention. Both groups demonstrated significant improvements in bimanual performance at 3 weeks, with gains maintained by BIM at 26 weeks (EMD 2.3; 95% CI 0.6-4.0; p=0.008). Overall, there were only small differences between the two training approaches. CIMT yielded greater changes in unimanual capacity of the impaired upper limb compared with BIM. Results generally reflect specificity of practice, with CIMT improving unimanual capacity and BIM improving bimanual performance. Considerable inter-individual variation in response to either intervention was evident. Future research should consider serial sequencing unimanual then BIM approaches to optimize upper limb outcomes for children with congenital hemiplegia. [ABSTRACT FROM AUTHOR]

The aim of this study was to describe and identify factors associated with motivation in children with cerebral palsy (CP). Children with CP were recruited for this cross-sectional study. Children were assessed using the Leiter Intelligence Test, the Gross Motor Function Measure, and the Vineland Adaptive Behavior Scale. Parents completed the Dimensions of Mastery Questionnaire (DMQ) and questionnaires on demographics, child behaviour, and family functioning. The parents of 74 children (46 males, 28 females; mean age 9y 2mo, SD 2y 1mo, range 5y 10mo-12y 11mo) completed the DMQ. Just over half of the children (39/74) were classified at Gross Motor Function Classification System (GMFCS) level I, with 13 classified at GMFCS level II, one at level III, six at level IV, and 14 at level V; one child was not classified. The most common diagnoses were spastic hemiplegia and quadriplegia (23 each), followed by diplegia (14). The highest motivation scores were obtained for the dimensions of mastery pleasure and social persistence and the lowest for persistence with motor or cognitive tasks. Age and sex were not predictive of scores on the DMQ. Higher IQ ( r=0.41), better motor ability ( r=0.43), and fewer limitations in self-care, communication, and socialization ( r=0.44-0.53) were positively associated with motivation total score. A negative impact of the child's disability on the family was associated with lower motivation ( r=−0.44). Positive social behaviours were positively correlated with motivation ( r=0.38-0.66), whereas hyperactivity and peer problems were negatively associated. High motivation was associated with fewer activity limitations and behavioural problems and reduced family burden. Low motivation may adversely influence a child's functional potential and the effectiveness of interventions. Strategies focusing on the child, peers, adults, or activities are proposed to enhance the children's motivation to engage in more challenging activities. [ABSTRACT FROM AUTHOR]

The article presents abstracts of studies to be presented at the 64th Annual Meeting of the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM), including "Comparison of dosage of Constraint Induced Movement Therapy versus Bimanual Training for children with congenital hemiplegia--Is half the dose enough?" by R. N. Boyd et al, "A multicenter clinical trial of pediatric Constraint-Induced therapy in children with CP: What is the dose?" by S. C. DeLuca, and "Both intensive bimanual and unimanual training improve unimanual reach-grasp-eat movement control in children with hemiplegia," by Y. Hung et al.

Aim This systematic review and critical evaluation of the literature was conducted to determine how gross muscle morphology and structure are altered in individuals with spastic cerebral palsy (CP). Method Electronic databases were searched for articles describing studies of muscle morphological and structural properties in individuals with spastic CP. Data describing muscle fascicle length, belly length, fascicle angle, cross-sectional area, volume, and thickness were extracted and effect sizes were computed for comparisons between individuals with spastic CP and typically developed individuals, between the paretic and non-paretic side in individuals with hemiplegia for all muscles examined, and across the full spectrum of gross motor function in individuals with spastic CP. Results The final yield consisted of 15 articles that met the inclusion criteria. The main finding of the review was the consistent evidence for reduced muscle belly length, muscle volume, cross-sectional area, and muscle thickness in the comparisons between paretic and typically developed muscle and the paretic and non-paretic muscle across a range of muscles. Interpretation Given the importance of muscle morphology and structure for generating muscle force, it is likely that the observed alterations that occur secondary to the neural lesion in individuals with spastic CP contribute to muscle weakness and the attendant loss of motor function in spastic CP. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to explore the predictive value of quantitative assessment of hand movements in 3-month-old infants after neonatal stroke. Method Thirteen infants born at term (five females, eight males; mean gestational age 39.4wks, SD 1.19, range 37–41wks; mean birthweight 3240g, SD 203, range 2900–3570g) with neonatal arterial ischaemic cerebral infarction, and 13 healthy infants (mean gestational age 39.1wks, range 37–41wks, SD 1.26; mean birthweight 3190g, SD 259, range 2680–3490g) were enrolled in the study. The absolute frequency and the asymmetry of global hand opening and closing, wrist segmental movements, and independent digit movements were assessed from videotapes recorded at around 12 weeks. Neurological outcome was assessed when the infants were at least 18 months old using Touwen’s neurological examination. Results Five of the 13 infants with neonatal stroke had normal neurological development, and eight had hemiplegia. Asymmetry of wrist segmental movements and the absolute frequency of independent digit movements were significantly different between infants with and without hemiplegia ( p=0.006 and p=0.008, respectively). No differences were found in global hand movements. Interpretation We propose that the observed abnormalities of hand movements are the result of two different mechanisms: direct disruption of the corticospinal projection to the spinal cord, and altered modulation of the central pattern generators of general movements. [ABSTRACT FROM AUTHOR]

Aim  The aim of this study was to describe how the usefulness of the hemiplegic hand develops in children with unilateral cerebral palsy (CP) aged between 18 months and 8 years. Method  A prospective longitudinal study of 43 children (22 males, 21 females) with unilateral CP was conducted. Inclusion age was 18 months to 5 years 4 months (mean 2y 8mo [SD 1y 1mo]). Children were assessed with the Assisting Hand Assessment (AHA) 3 to 11 times per child over a mean period of 4 years 6 months. Two models were used for grouping children: by AHA score at 18 months and by Manual Ability Classification System (MACS) levels. Estimated average motor development curves were fitted with a nonlinear mixed-effects model. Results  Children with a high AHA score (high ability level) at 18 months reached a significantly higher ability level and at a higher progression rate than children with a low 18-month AHA score. Limits of development differed between the three MACS levels. Interpretation  Results indicate that the AHA score at 18 months can be used to discuss future development of affected hand use in bimanual tasks in children with unilateral CP. [ABSTRACT FROM AUTHOR]

Aim This systematic review aimed to compare the validity, reliability, evaluative validity, and clinical utility of upper limb activity measures for children aged 5 to 16 years with congenital hemiplegia. Method Electronic databases were searched to identify assessments that measure upper limb activity available for use and for which published validity and reliability data for the population are obtainable. Assessment items were coded according to the International Classification of Functioning, Disability and Health (ICF) categories to determine if at least 35% of the assessment items fell within the activity component of the ICF. Assessments that met these criteria were included in the review. Results Thirty-eight measures were identified, and five met the inclusion criteria. The best measure of unimanual capacity was the Melbourne Assessment of Unilateral Upper Limb Function (MUUL); however, the Shriners Hospital Upper Extremity Evaluation (SHUEE) and the Quality of Upper Extremity Skills Test (QUEST) could also be considered, depending on the type of information required. The performance-based measure of bimanual upper limb activity in children with hemiplegia with the best psychometric properties was the Assisting Hand Assessment (AHA). The ABILHAND-Kids is a parent-report, performance-based questionnaire with excellent clinical utility and psychometric properties. Interpretation Clinicians may choose to use more than one of these measures to detect changes in unimanual or bimanual upper limb activity. [ABSTRACT FROM AUTHOR]

Aims Our aim was to examine intellectual development in children with congenital hemiplegia from early childhood to adolescence. Method Full-scale IQ (FIQ), Verbal IQ (VIQ), and Performance IQ (PIQ) scores were measured in 32 participants (19 males, 13 females) with congenital hemiplegia at mean ages of 4 years 6 months (SD 7mo; 31 participants), 7 years (SD 6mo; 23 participants), and 14 years (SD 1y 5mo; 26 participants). Results The FIQ and VIQ scores did not change with age, but the PIQ declined significantly (0.7 points per year; p=0.004). The estimated mean (95% confidence intervals) scores in males born at term with right-sided lesions without epilepsy were FIQ 106.5 (95.29–117.74), VIQ 105.9 (95.57–116.24), and PIQ 103.7 (93.19–114.31). Those means were negatively associated with preterm birth. PIQ was negatively associated with epilepsy. VIQ increased more quickly in males and in children with right-sided lesions. Interpretation The results confirm previous findings of FIQ stability, PIQ decline, the impact of epilepsy, and the status of females with left-sided lesions, and also reveal the effect of gestational age at birth. They underline the importance of management focused on nonverbal functions and further the debate about the early lateralization of language, the ‘crowding effect’, and the difference in brain plasticity between males and females. [ABSTRACT FROM AUTHOR]

The measurement of motor deficits in individuals with cerebral palsy (CP) has been largely based on clinical criteria. Yet functional imaging and non-invasive stimulation methods provide a means to measure directly abnormalities of the motor system. The size and location of muscles and movement representations can be determined with transcranial magnetic stimulation (TMS) and functional magnetics resonance imaging. Thus the homunculus can be individually mapped in children with CP. Because size of representation within the homunculus relates to quality of motor control, measurement of the distance between body parts provides a metric that may be useful in classifying deficits. Bilateral motor control in one hemisphere, while normal in neonates, persists variably in CP, providing another physiological metric. In this study, we used TMS to measure hand and ankle representations in a convenience sample of children with spastic CP. Overlapping thumb and ankle maps were found in children with both hemiplegia and diplegia, and these maps may be from either side of the body. While more participants are required to make conclusions about disability and compression/bilaterality of the homunculus, it appears as if TMS-derived metrics relate to motor abnormalities. These abnormal motor maps also are a therapeutic target, as stimulation methods are being developed as adjuncts to physical means of rehabilitation. [ABSTRACT FROM AUTHOR]

The article presents abstracts on topics related to cerebral palsy which include improvement in arm and hand function in adults with hemiplegic cerebral palsy using internet-based home training, body mass index and body fats in adults with cerebral palsy, and health issues in adults with cerebral palsy.

The specific aims of this study were to (1) examine the psychometric properties (unidimensionality, differential item functioning, scale coverage) of an item bank of upper-extremity skills for children and adolescents with cerebral palsy (CP); (2) evaluate a simulated computer-adaptive test (CAT) using this item bank; (3) examine the concurrent validity of the CAT with the Pediatric Outcomes Data Collection Instrument (PODCI) upper-extremity core scale; and (4) determine the discriminant validity of the simulated CAT with Manual Ability Classification System (MACS) levels and CP type (i.e. diplegia, hemiplegia, or quadriplegia). Parents ( n=180) of children and adolescents with CP (spastic diplegia 49%, hemiplegia 22%, or quadriplegia 28%) consisting of 102 males and 78 females with a mean age of 10 years 6 months (SD 4y 1mo, range 2–21y), and MACS levels I through V participated in calibration of an item pool and completed the PODCI. Confirmatory factor analyses supported a unidimensional model using 49 of the 53 upper-extremity items. Simulated CATs of 5, 10, and 15 items demonstrated excellent accuracy (intraclass correlation coefficient [ICCs] >0.93) with the full item bank, had high correlations with the PODCI upper-extremity core scale score (ICC 0.79), and discriminated among MACS levels. The simulated CATs demonstrated excellent overall content coverage over a wide age span and severity of upper-extremity involvement. The future development and refinement of CATs for parent report of physical function in children and adolescents with CP is supported by our work. [ABSTRACT FROM AUTHOR]

In this article we describe visual perceptual abilities of a clinical population, referred for visual problems to our multidisciplinary team and assessed with the five computer tasks from the L94 visual perceptual battery. Clinical and neuroimaging findings were correlated with the findings on this task battery. Seventy children (35 males, 35 females) constituted our cohort. Age ranged from 4 to 20 years (mean 7y [SD 3y]). Forty children were born before 37 weeks gestational age. Thirty-six children had cerebral palsy (CP), of whom 24 had spastic diplegia, five had spastic hemiplegia, and four had spastic quadriplegia. Three children had ataxic CP. Perceptual visual impairment (PVI) was established in comparison to the performance age obtained on non-verbal intelligence subtests, instead of chronological age. Our results suggest that children with a history of preterm birth and a clinical CP picture are most at risk for a specific PVI. Correlations among other clinical variables did not define a clinical subgroup more at risk. Children with periventricular leucomalacia were almost equally represented in both PVI and non-PVI groups. Normal magnetic resonance imaging did not exclude the presence of PVI. In these children, however, we found another impairment profile, more in favour of dorsal stream impairment. [ABSTRACT FROM AUTHOR]

We report the pharmacological treatment of a case of alternating hemiplegia of childhood (AHC) in a 14-year-old female with an established diagnosis. Although the patient’s symptoms are consistent with those of the condition, she did not respond to treatment with haloperidol, flunarizine, or propranolol. Treatment with aripiprazole resulted in a reduction in the frequency, duration, and severity of episodes of alternating hemiplegia, along with other therapeutic benefits. After treatment with aripiprazole was started, the patient was inadvertently given an inactive drug, resulting in a worsening of her hemiplegic episodes, which improved again on rechallenge. A comparison of the pharmacological actions of successful and unsuccessful treatments for AHC is made. Modulation of both dopamine and histamine systems together appears to be important in the treatment of AHC and further investigation of such pharmacotherapies is suggested. [ABSTRACT FROM AUTHOR]

This study investigated interrater reliability and measurement error of the Melbourne Assessment of Unilateral Upper Limb Function (Melbourne Assessment) and the Quality of Upper Extremity Skills Test (QUEST), and assessed the relationship between both scales in 21 children (15 females, six males; mean age 6y 4mo [SD 1y 3mo], range 5–8y) with hemiplegic CP. Two raters scored the videotapes of the assessments independently in a randomized order. According to the House Classification, three participants were classified as level 1, one participant as level 3, eight as level 4, three as level 5, one participant as level 6, and five as level 7. The Melbourne Assessment and the QUEST showed high interrater reliability (intraclass correlation 0.97 for Melbourne Assessment; 0.96 for QUEST total score; 0.96 for QUEST hemiplegic side). The standard error of measurement and the smallest detectable difference was 3.2% and 8.9% for the Melbourne Assessment and 5.0% and 13.8% for the QUEST score on the hemiplegic side. Correlation analysis indicated that different dimensions of upper limb function are addressed in both scales. [ABSTRACT FROM AUTHOR]

This prospective longitudinal multicenter study of ambulatory children with cerebral palsy (CP) examined changes in outcome tool score over time, tool responsiveness, and used a systematic method for defining minimum clinically important differences (MCIDs). Three hundred and eighty-one participants with CP (Gross Motor Function Classification System [GMFCS] Levels I–III; age range 4–18y, mean age 11y [SD 4y 4mo]; 265 diplegia, 116 hemiplegia; 230 males, 151 females). At baseline and follow-up at least 1 year later, Functional Assessment Questionnaire, Gross Motor Function Measure, Pediatric Quality of Life Inventory, Pediatric Outcomes Data Collection Instrument, Pediatric Functional Independence Measure, temporal–spatial gait parameters, and oxygen cost were collected. Adjusted standardized response means determined tool responsiveness for nonsurgical ( n=292) and surgical ( n=87) groups at GMFCS Levels I to III. Most scores reaching medium or large effect sizes were for GMFCS Level III. Nonsurgical group change scores were used to calculate MCID thresholds for ambulatory children with CP. These values were verified by examining participants who changed GMFCS levels. Tools measuring function were responsive when a change large enough to cause a change in GMFCS level occurred. MCID thresholds assess change in study populations over time, and serve as the basis for designing prospective intervention studies. [ABSTRACT FROM AUTHOR]

Hemimegalencephaly (HME) is a developmental brain lesion consisting of a unilateral enlarged, dysplastic, and often highly epileptogenic cerebral hemisphere. Most patients exhibit early onset intractable seizures, status epilepticus, hemiplegia, hemianopsia, and developmental delay. Major surgical procedures are advocated for limiting the devastating consequences of epilepsy. We studied a female with HME, early onset intractable seizures and recurrent status epilepticus, in whom progressive hemiatrophy of the enlarged hemisphere and normal growth of the contralateral hemisphere, exceeding the size of the dysplastic hemisphere, was demonstrated by magnetic resonance imaging. Histopathology, following functional hemispherectomy at the age of 7 years, demonstrated severe neuronal loss with an elevated number of cells exhibiting the morphological and biochemical features of apoptosis. Eighteen months after surgery the patient was seizure-free (Engel class I) and exhibited improved motor and language skills, alertness and social behaviour. We hypothesize that nearly continuous seizure activity might sustain seizure-induced brain injury in the dysplastic hemisphere but causal heterogeneity and associated anatomical factors may influence differently the individual predisposition to atrophic changes. [ABSTRACT FROM AUTHOR]

The article presents abstracts of papers on developmental medicine and child neurology which include "Botulinum Toxin A in the Upper Limb in Children With Cerebral Palsy: State of the Evidence," by Brian Hoare, Margaret Wallen and Barry Rawicki, "Intranasal Fentanyl As an Analgesic Agent in the Administration of Botulinum Toxin A," by Katherine Langdon, Jane Valentine and Anna Gubbay and "Relationship Between Unimanual Capacity and Bimanual Performance in Children With Hemiplegia," by Roslyn Boyd, R. Gilmore and K. Corn.

The effect of spastic cerebral palsy on in vivo gastrocnemius muscle fascicle length is not clear. Similarity of fascicle lengths in children with diplegia and typically developing children, but shortening of fascicle lengths in the paretic legs of children with hemiplegia compared with the non-paretic legs, are both reported. In the former case, comparisons were made between fascicle lengths normalized to leg length, whereas in the latter case, absolute fascicle lengths were compared. The inherent assumptions when normalizing fascicle length (measured via ultrasonography) were not validated, raising the possibility that inappropriate normalization contributed to the controversy. We used statistical methods to control the potential confounding effect of leg length on fascicle length, and tested the feasibility of the normalization method for a group of 18 children with diplegia (nine males, nine females; mean age 8y 7mo [SD 3y 11mo], range 2-15y; Gross Motor Function Classification System levels II and III) and 50 typically developing children (20 males, 30 females; mean age 9y 1mo [SD 2y 4mo], range 4-14y). Children with diplegia, as a group, had shorter absolute and normalized fascicle lengths ( p<0.05) but we could not refute the appropriateness of the normalization method. Other methodological issues (such as sample characteristics) might have contributed to the apparent controversy between the stdies. [ABSTRACT FROM AUTHOR]

Several positive influences of orthoses on gait in children with cerebral palsy have been documented, as well as some detrimental effects. Most importantly, push-off is decreased in orthoses, compromising a physiological third ankle rocker. The aim of this study was to evaluate the effect of three types of orthosis on gait in a homogeneous group of children. All orthoses aimed at improving push-off and normalizing the pathological plantarflexion–knee extension couple. Thirty-seven children (22 females, 15 males) with hemiplegia, aged 4 to 10 years (30 Gross Motor Function Classification System [GMFCS] Level I, six GMFCS Level II), walked barefoot and with orthoses being either Orteams® (orthoses with the dorsal part containing 11 sleeves), posterior leafsprings (PLS), or Dual Carbon Fibre Spring ankle foot orthosis (AFOs®; CFO: carbon fibre at the dorsal part of the orthosis). All orthoses were expected to prevent plantarflexion and allow dorsiflexion, thus improving first, second, and third rocker. The orthoses were compared through objective gait analysis, including 3D kinematics and kinetics. All orthoses successfully improved the gait pattern and only small differences were noted between the configurations of the different orthoses. The CFO®, however, allowed a more physiological third ankle rocker compared with the Orteam®/PLS. Although the PLS ensured the highest correction at the ankle around initial contact, the CFO® created a significantly higher maximal hip flexion moment in stance. In general, the results of this study indicated a substantial functional flexibility of the CFO®. [ABSTRACT FROM AUTHOR]

The aim of this study was to explore motor development in children with cerebral palsy (CP) using developmental curves for CP, subtypes, and the five severity levels of the Gross Motor Function Classification System (GMFCS). The Gross Motor Function Measure (GMFM) and the GMFCS were applied to 317 children (145 females, 172 males) with CP, aged between 1 and 15 years. The CP type distribution was spastic diplegia in 157 (49%), spastic hemiplegia in 101 (33%), spastic tetraplegia in 11 (3%), dyskinesia in 38 (12%), and ataxia in 10 (3%). Forty-five physiotherapists were trained in the GMFM and intra- and interrater reliability was tested. The GMFM was measured prospectively every 6 months up to the age of 4 years and once a year thereafter. Developmental curves were constructed for 258 children with spastic CP. About three-quarters of the children at GMFCS Level I reached 90% of the maximum GMFM score at 5 years of age. The performance peaked at 7 years of age. Children at GMFCS Level II reached 90% at a median age of 5 years, which was also the upper limit, reached by about three-quarters at 7 years of age. The majority of children at GMFCS Level III reached 80% of the GMFM by 7 years of age and most of the children at GMFCS Level IV reached 30% at 5 years and remained there. The median score for children at GMFCS Level V was 20%. The intra- and interrater reliability for the GMFM 88 among physiotherapists were Spearman's rank correlation coefficient 0.91 and 0.99 respectively. There were 931 measurements with a median of 2 (1–11) per child. The gross motor development was demonstrated for the five GMFCS levels in children with spastic CP. These kind of curves may be useful for monitoring and predicting motor development, for planning treatment, and for evaluating outcome after interventions. [ABSTRACT FROM AUTHOR]

A recent therapeutic intervention, constraint-induced movement therapy (CIMT), has been shown to improve movement efficiency and quality of movement in the involved hand of children with hemiplegic cerebral palsy (CP). In the present study, we investigate the long-term effects of CIMT and the effect of a second course on involved limb function using an ABABA design. Eight children with mild to moderate hemiplegic CP (six males, two females; mean age 8y 7mo [SD 2y 6mo]; range 5–11y), who had received a CI therapy intervention 12 months before this study, participated in a second intervention. In both interventions, the children wore a sling on their non-involved upper extremity for 6 hours per day during 10 out of 12 consecutive days and were engaged in play and functional activities that provided structured practice using the involved upper extremity. The results indicated initial improvements in movement efficiency, as measured by the Jebsen–Taylor Test of Hand Function, the Speed and Dexterity subtest (no. 8) of the Bruininks–Oseretsky Test of Motor Proficiency, and caregivers' perceptions of amount of use and quality of movement of the involved limb, were retained 12 months after the first intervention. The second intervention resulted in further improvement on these measures. Results indicate that intensive practice associated with CIMT may be retained long term, and that continued improvements may occur after a second intervention dose. [ABSTRACT FROM AUTHOR]

Alternating hemiplegia of childhood has many factors that make it difficult to manage. These include its rarity of about one case per million, the variability of the manifestations, with seven characteristic features, and the potential for disabilities and acute, often severe, episodes in a disease that is of uncertain cause and for which treatment evidence is sparse. An integrated multidisciplinary team and emergency availability are key medical requirements, as well as an educational setting that understands the variations in performance that occur. The mainstays of treatment have been flunarizine, antiepilepsy drugs for the 50% of patients with epilepsy, attempts to avoid trigger situations, and the rapid encouragement of sleep when attacks begin. The diagnostic and management predicament of child, parent, and paediatrician in complex rare disorders are well illustrated by this condition. [ABSTRACT FROM AUTHOR]

Constraint-induced (CI) movement therapy is a physical intervention that has been receiving increasing attention in pediatric rehabilitation. So far, the evidence suggests that practice associated with CI therapy may improve impaired unimanual hand function in some children with hemiplegic cerebral palsy (CP). However, CI therapy has several important limitations. Most importantly, children with hemiplegia have impairments in bimanual coordination beyond their unilateral impairments. Thus, an intervention approach to increase functional independence during activities of daily living by using both hands in cooperation is needed. Here we briefly review the etiology of hemiplegic CP, describe studies of pediatric CI therapy efficacy in relation to the etiology, discuss the conceptual and practical limitations of CI therapy for this population, and describe bimanual coordination impairments in children with hemiplegia. Finally, we introduce a new intervention for children with hemiplegia, hand–arm bimanual intensive training (HABIT), to address the limitations of CI therapy and to improve bimanual coordination. HABIT retains the two major elements of pediatric CI therapy (intensive structured practice and child-friendliness). The proposed methodology demonstrates that extensive targeted practice can be provided in a child-friendly manner without using a physical restraint, although the efficacy of such an approach remains to be determined. [ABSTRACT FROM AUTHOR]

An ongoing population-based register of cerebral palsy (CP) in the West of Ireland was established in 2002 to calculate the prevalence of CP and to monitor CP epidemiological trends in the area. Children were only included if they were at least 5 years of age; children with postneonatal CP were also included. Eighty-five children were identified, giving an overall prevalence for the period 1990 to 1999 of 1.88 per 1000 neonatal survivors (95% confidence interval 1.5–2.4). Males accounted for 68% (n=51) and females for 32% (n=24) of all cases. Among infants weighing less than 1500g at birth, the rate of CP was 39/1000 neonatal survivors compared with 1.3/1000 for infants weighing more than 2500g. The most common CP subtype was bilateral spastic CP (51%), followed by hemiplegia (32%), dyskinesia (9%), and ataxia (7%). Eighteen per cent of all children were unable to walk, 21% had a sensory impairment, and 56% had an intellectual impairment. [ABSTRACT FROM AUTHOR]

We assessed the influence of equinus gait treatments on the vertical displacement of the body's centre of mass (COM) in 21 patients with cerebral palsy (14 males, 7 females; mean age 8y 9mo [SD 2y]; range 3y 7mo–17y) presenting different topographical types (quadriplegia, n=1; diplegia, n=6; right hemiplegia, n=6; and left hemiplegia, n=8). Vertical COM displacement was computed from ground reaction forces, and lower limb kinematics was recorded simultaneously. Equinus gait was treated with non-operative treatments (i.e. botulinum toxin injections and stretching casts) in 14 patients, and with operative treatments in seven patients. After non-operative treatments, the entire ankle displacement shifted towards dorsiflexion throughout the gait cycle, but the amplitude of the third foot rocker (TR) and vertical COM displacement remained unchanged. However, after operative treatments, the amplitude of TR increased and vertical COM displacement decreased. A negative linear correlation was found between the former variables in all the patients where 53% of the changes in their vertical COM displacement, after equinus gait treatments, were explained by the changes in TR amplitude. In fact, TR remains a main gait determinant, reducing the vertical COM displacement after equinus gait treatment and influencing the general gait pattern. [ABSTRACT FROM AUTHOR]

The goal was to compare children with hemiplegia with those with diplegia within Gross Motor Functional Classification System (GMFCS) levels using multiple validated outcome tools. Specifically, we proposed that children with hemiplegia would have better gait and gross motor function within levels while upper extremity function would be poorer. Data were collected on 422 ambulatory children with cerebral palsy: 261 with diplegia and 161 with hemiplegia, across seven centers. Those with hemiplegia in each level performed significantly and consistently better on gait or lower extremity function and poorer on upper extremity and school function than those with diplegia. In GMFCS Level II, the group with hemiplegia walked faster (p=0.017), scored 6.6 points higher on Dimension E of the Gross Motor Function Measure (p=0.017), 6.7 points lower on Upper Extremity subscale of the Pediatric Outcomes Data Collection Instrument, and 9.1 points lower on WeeFIM self-care (p=0.002). Basing motor prognosis on GMFCS level alone may underestimate lower extremity skills of children with hemiplegia, and overestimate those of children with diplegia. [ABSTRACT FROM AUTHOR]

Social integration and independence is the ultimate goal of habilitation and social support for patients with cerebral palsy (CP). Having a partner and having children provide support for social integration of adults with or without a disability. We studied 416 participants with CP born between 1965 and 1970 (243 males, 173 females; mean age 32y 2mo [SD 2y]; age range 29–35y) and compared them with 2247 age-matched comparison individuals. Diagnostic subtypes of the 416 participants were: 31% hemiplegia, 49% diplegia, 11% tetraplegia, and 9% other types. The level of motor impairment, estimated in childhood, with regard to walking ability was 65% able to walk without assistance, 22% with assistance, and 12% not able to walk (for 1% of the participants their walking ability was not known). We found no sign of increased social integration over the past two or three decades in Denmark. Sixty-eight per cent lived independently, 13% lived with their parents, and 16% lived at an accommodation facility arranged by the county (institution). Twenty-eight per cent of the participants were cohabiting and 19% had children. The presence of epilepsy and the severity of physical or cognitive impairment as assessed in childhood predicted independent living and physical and cognitive impairment predicted cohabitation, but parents' socioeconomic position and region of living did not. Fifty-five percent of the participants, compared with 4% of the comparison group, had no competitive employment, cohabiting partner, or biological children. The remaining participants had at least one of these types of social contact, but this more optimally socially integrated half of the participants only combined all three types of social contact half as often as the comparison group. This could be due to cognitive difficulties or premature ageing. [ABSTRACT FROM AUTHOR]