Comprehensive whole-genome sequence analyses provide insights into the genomic architecture of cerebral palsy.

We performed whole-genome sequencing (WGS) in 327 children with cerebral palsy (CP) and their biological parents. We classified 37 of 327 (11.3%) children as having pathogenic/likely pathogenic (P/LP) variants and 58 of 327 (17.7%) as having variants of uncertain significance. Multiple classes of P/LP variants included single-nucleotide variants (SNVs)/indels (6.7%), copy number variations (3.4%) and mitochondrial mutations (1.5%). The COL4A1 gene had the most P/LP SNVs. We also analyzed two pediatric control cohorts (n = 203 trios and n = 89 sib-pair families) to provide a baseline for de novo mutation rates and genetic burden analyses, the latter of which demonstrated associations between de novo deleterious variants and genes related to the nervous system. An enrichment analysis revealed previously undescribed plausible candidate CP genes (SMOC1, KDM5B, BCL11A and CYP51A1). A multifactorial CP risk profile and substantial presence of P/LP variants combine to support WGS in the diagnostic work-up across all CP and related phenotypes.

Exploring demographic, medical, and developmental determinants of adaptive behaviour in children with hemiplegic cerebral palsy.

Hemiplegic cerebral palsy (CP), the most common subtype, is characterized by high levels of mobility. Despite this, children with hemiplegic CP can face challenges functioning in and adapting to situations of everyday life. The purpose of this cross-sectional study (Hemi-NET database) was to identify factors associated with adaptive behaviour in 59 children with hemiplegic CP (ages 4-18; GMFCS I-IV). Using multivariate regression analyses, the relationship between demographic, medical, and developmental factors and adaptive behaviour (measured by the Adaptive Skills Composite score of the BASC-2) was explored. Results indicate that 34% of children had impaired adaptive skills. An autism diagnosis and lower communication functioning were significantly associated with poorer adaptive skills (R2 = 0.42, F(4, 43) = 7.87, p < 0.001), while factors such as IQ scores and GMFCS level were not. The results contribute to the growing literature that suggests that clinicians and researchers need to look beyond motor functioning when working with individuals with CP.

Somatosensory Plasticity in Hemiplegic Cerebral Palsy Following Constraint Induced Movement Therapy.

BACKGROUND: Children with hemiplegic cerebral palsy (HCP) experience upper limb somatosensory and motor deficits. Although constraint-induced movement therapy (CIMT) improves motor function, its impact on somatosensory function remains underinvestigated. OBJECTIVE: The objective of this study was to evaluate somatosensory perception and related brain responses in children with HCP, before and after a somatosensory enhanced CIMT protocol, as measured using clinical sensory and motor assessments and magnetoencephalography. METHODS: Children with HCP attended a somatosensory enhanced CIMT camp. Clinical somatosensory (tactile registration, 2-point discrimination, stereognosis, proprioception, kinesthesia) and motor outcomes (Quality of Upper Extremity Skills [QUEST] Total/Grasp, Jebsen-Taylor Hand Function Test, grip strength, Assisting Hand Assessment), as well as latency and amplitude of magnetoencephalography somatosensory evoked fields (SEF), were assessed before and after the CIMT camp with paired sample t-tests or Wilcoxon signed-rank tests. RESULTS: Twelve children with HCP (mean age: 7.5 years, standard deviation: 2.4) participated. Significant improvements in tactile registration for the affected (hemiplegic) hand (Z = 2.39, P = 0.02) were observed in addition to statistically and clinically significant improvements in QUEST total (t = 3.24, P = 0.007), QUEST grasp (t = 3.24, P = 0.007), Assisting Hand Assessment (Z = 2.25, P = 0.03), and Jebsen-Taylor Hand Function Test (t = -2.62, P = 0.03). A significant increase in the SEF peak amplitude was also found in the affected hand 100 ms after stimulus onset (t = -2.22, P = 0.04). CONCLUSIONS: Improvements in somatosensory clinical function and neural processing in the affected primary somatosensory cortex in children with HCP were observed after a somatosensory enhanced CIMT program. Further investigation is warranted to continue to evaluate the effectiveness of a sensory enhanced CIMT program in larger samples and controlled study designs.

Pharmacological and neurosurgical interventions for individuals with cerebral palsy and dystonia: a systematic review update and meta-analysis.

AIM: To update a systematic review of evidence published up to December 2015 for pharmacological/neurosurgical interventions among individuals with cerebral palsy (CP) and dystonia. METHOD: Searches were updated (January 2016 to May 2020) for oral baclofen, trihexyphenidyl, benzodiazepines, clonidine, gabapentin, levodopa, botulinum neurotoxin (BoNT), intrathecal baclofen (ITB), and deep brain stimulation (DBS), and from database inception for medical cannabis. Eligible studies included at least five individuals with CP and dystonia and reported on dystonia, goal achievement, motor function, pain/comfort, ease of caregiving, quality of life (QoL), or adverse events. Evidence certainty was evaluated using GRADE. RESULTS: Nineteen new studies met inclusion criteria (two trihexyphenidyl, one clonidine, two BoNT, nine ITB, six DBS), giving a total of 46 studies (four randomized, 42 non-randomized) comprising 915 participants when combined with those from the original systematic review. Very low certainty evidence supported improved dystonia (clonidine, ITB, DBS) and goal achievement (clonidine, BoNT, ITB, DBS). Low to very low certainty evidence supported improved motor function (DBS), pain/comfort (clonidine, BoNT, ITB, DBS), ease of caregiving (clonidine, BoNT, ITB), and QoL (ITB, DBS). Trihexyphenidyl, clonidine, BoNT, ITB, and DBS may increase adverse events. No studies were identified for benzodiazepines, gabapentin, oral baclofen, and medical cannabis. INTERPRETATION: Evidence evaluating the use of pharmacological and neurosurgical management options for individuals with CP and dystonia is limited to between low and very low certainty. What this paper adds Meta-analysis suggests that intrathecal baclofen (ITB) and deep brain stimulation (DBS) may improve dystonia and pain. Meta-analysis suggests that DBS may improve motor function. Clonidine, botulinum neurotoxin, ITB, and DBS may improve achievement of individualized goals. ITB and DBS may improve quality of life. No direct evidence is available for oral baclofen, benzodiazepines, gabapentin, or medical cannabis.

Inpatient Exergames for Children with Cerebral Palsy following Lower Extremity Orthopedic Surgery: A Feasibility Study.

Objective: Evaluate the feasibility of implementing cycling-based exergames for children with cerebral palsy (CP) following lower extremity orthopedic surgery and explore its impact on pain and well-being.Methods: Ten children with CP were recruited; the first five received physiotherapy (comparison) and next five received fifteen exergame sessions over 3 weeks and physiotherapy (case) (NCT0376907). Feasibility indicators evaluated recruitment, questionnaire and exergame completion. Faces Pain Scale-Revised (FPS-R), PROMIS Pediatric Pain Interference Scale (PPIS), and KIDSCREEN-27 were administered. Wilcoxon signed-rank and effect size (r) tests evaluated within-group differences and between-group differences were assessed using Mann-Whitney U tests.Results: All feasibility indicators were met. Large effects for improved case group pain were identified (FPS-R r = 0.60, PPIS r = 0.58), as well as significant improvement in KIDSCREEN-27 total (U = 0.50, p = .05) and psychological well-being (U = 3.00, p = .01) scores, favoring the case group.Conclusions: Incorporating pediatric exergames is feasible and demonstrates potential for improving pain and well-being.

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

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

Pharmacological and neurosurgical interventions for managing dystonia in cerebral palsy: a systematic review.

AIM: To systematically review evidence for pharmacological/neurosurgical interventions for managing dystonia in individuals with cerebral palsy (CP) to inform a care pathway. METHOD: Searches included studies with a minimum of five participants with dystonia in CP receiving oral baclofen, benzodiazepines (clonazepam, diazepam, lorazepam), clonidine, gabapentin, levodopa, trihexyphenidyl, botulinum toxin, intrathecal baclofen (ITB), or deep brain stimulation (DBS). Evidence was classified according to American Academy of Neurology guidelines. RESULTS: Twenty-eight articles underwent data extraction: one levodopa, five trihexyphenidyl, three botulinum toxin, six ITB, and 13 DBS studies. No articles for oral baclofen, benzodiazepines, clonidine, or gabapentin met the inclusion criteria. Evidence for reducing dystonia was level C (possibly effective) for ITB and DBS; level C (possibly ineffective) for trihexyphenidyl; and level U (inadequate data) for botulinum toxin. INTERPRETATION: For dystonia reduction, ITB and DBS are possibly effective, whereas trihexyphenidyl was possibly ineffective. There is insufficient evidence to support oral medications or botulinum toxin to reduce dystonia. There is insufficient evidence for pharmacological and neurosurgical interventions to improve motor function, decrease pain, and ease caregiving. The majority of the pharmacological and neurosurgical management of dystonia in CP is based on clinical expert opinion. WHAT THIS PAPER ADDS: Intrathecal baclofen and deep brain stimulation are possibly effective in reducing dystonia. Current evidence does not support effectiveness of oral medications or botulinum toxin to reduce dystonia. Evidence is inadequate for pharmacological/neurosurgical interventions impact on improving motor function, pain/comfort, and easing caregiving. The majority of the care pathway rests on expert opinion.

The use of functional electrical stimulation to improve upper limb function in children with hemiplegic cerebral palsy: A feasibility study.

BACKGROUND: Grasping and manipulating objects are common problems for children with hemiplegic cerebral palsy. Multichannel-functional electrical stimulation may help facilitate upper limb movements and improve function. OBJECTIVE: To evaluate the feasibility of multichannel-functional electrical stimulation to improve grasp and upper limb function in children with hemiplegic cerebral palsy to inform the development of a clinical trial. METHODS: A prospective pre-/post-test/follow-up (six months) design with three children, aged 6-13 years, was used. Multichannel-functional electrical stimulation (mFES) was applied to the hemiplegic upper limb for up to 48 sessions over 16 weeks. Feasibility indicators included recruitment of participants and adherence rates, safety, and discomfort/pain. Effectiveness was assessed using the grasp domain of the Quality of Upper Extremity Skills Test, and other secondary clinical outcome measures with "success" criteria set a priori. RESULTS: Participant recruitment target was not met but adherence was high, and multichannel-functional electrical stimulation was found to be safe and comfortable. Of the three participants, two improved in grasp at post-test, whereas one child's ability deteriorated. Only one child met success criteria on most outcomes at post-test. CONCLUSIONS: Feasibility indicators met success criteria, except for participant recruitment. Treatment effectiveness was mixed. A future case comparison investigation with a larger but more selected sample is suggested.

De novo and rare inherited copy-number variations in the hemiplegic form of cerebral palsy.

PurposeHemiplegia is a subtype of cerebral palsy (CP) in which one side of the body is affected. Our earlier study of unselected children with CP demonstrated de novo and clinically relevant rare inherited genomic copy-number variations (CNVs) in 9.6% of participants. Here, we examined the prevalence and types of CNVs specifically in hemiplegic CP.MethodsWe genotyped 97 unrelated probands with hemiplegic CP and their parents. We compared their CNVs to those of 10,851 population controls, in order to identify rare CNVs (<0.1% frequency) that might be relevant to CP. We also sequenced exomes of "CNV-positive" trios.ResultsWe detected de novo CNVs and/or sex chromosome abnormalities in 7/97 (7.2%) of probands, impacting important developmental genes such as GRIK2, LAMA1, DMD, PTPRM, and DIP2C. In 18/97 individuals (18.6%), rare inherited CNVs were found, affecting loci associated with known genomic disorders (17p12, 22q11.21) or involving genes linked to neurodevelopmental disorders.ConclusionWe found an increased rate of de novo CNVs in the hemiplegic CP subtype (7.2%) compared to controls (1%). This result is similar to that for an unselected CP group. Combined with rare inherited CNVs, the genomic data impacts the understanding of the potential etiology of hemiplegic CP in 23/97 (23.7%) of participants.

Ability-Based Balancing Using the Gross Motor Function Measure in Exergaming for Youth with Cerebral Palsy.

OBJECTIVE: To test if the gross motor function measure (GMFM) could be used to improve game balancing allowing youth with cerebral palsy (CP) with different physical abilities to play a cycling-based exercise videogame together. Our secondary objective determined if exergaming with the GMFM Ability-Based algorithm was enjoyable. MATERIALS AND METHODS: Eight youth with CP, 8-14 years of age, GMFM scores between 25.2% and 87.4% (evenly distributed between Gross Motor Function Classification System levels II and III), competed against each other in head-to-head races, totaling 28 unique race dyads. Dyads raced three times, each with a different method of minimizing the distance between participants (three balancing algorithms). This was a prospective repeated measures intervention trial with randomized and blinded algorithm assignment. The GMFM Ability-Based algorithm was developed using a least squares linear regression between the players' GMFM score and cycling cadence. Our primary outcome was dyad spread or average distance between players. The GMFM Ability-based algorithm was compared with a control algorithm (No-Balancing), and an idealized algorithm (one-speed-for-all [OSFA]). After each race, participants were asked "Was that game fun?" and "Was that game fair?" using a five-point Likert scale. RESULTS: Participants pedaled quickly enough to elevate their heart rate to an average of 120 ± 8 beats per minute while playing. Dyad spread was lower when using GMFM Ability-Based balancing (4.6 ± 4.2) compared with No-Balancing (11.9 ± 6.8) (P < 0.001). When using OSFA balancing, dyad spread was (1.6 ± 0.9), lower than both GMFM Ability-Based (P = 0.006) and No-Balancing (P < 0.001). Cycling cadence positively correlated to GMFM, equal to 0.58 (GMFM) +33.29 (R2adj= 0.662, P = 0.004). Participants rated the games a median score 4/5 for both questions: "was that game fun?" and "was that game fair?." CONCLUSION: The GMFM Ability-Based balancing decreased dyad spread while requiring participants to pedal quickly, facilitating interaction and physical activity.

Balancing for Gross Motor Ability in Exergaming Between Youth with Cerebral Palsy at Gross Motor Function Classification System Levels II and III.

OBJECTIVE: To test how three custom-built balancing algorithms minimize differences in game success, time above 40% heart rate reserve (HRR), and enjoyment between youth with cerebral palsy (CP) who have different gross motor function capabilities. Youth at Gross Motor Function Classification System (GMFCS) level II (unassisted walking) and level III (mobility aids needed for walking) competed in a cycling-based exercise video game that tested three balancing algorithms. MATERIALS AND METHODS: Three algorithms: a control (generic-balancing [GB]), a constant non-person specific (One-Speed-For-All [OSFA]), and a person-specific (Target-Cadence [TC]) algorithms were built. In this prospective repeated measures intervention trial with randomized and blinded algorithm assignment, 10 youth with CP aged 10-16 years (X ± standard deviation = 12.4 ± 1.8 years; GMFCS level II n = 4, III n = 6) played six exergaming sessions using each of the three algorithms. Outcomes included game success as measured by a normalized game score, time above 40% HRR, and enjoyment. RESULTS: The TC algorithm balanced game success between GMFCS levels similarly to GB (P = 0.11) and OSFA (P = 0.41). TC showed poorer balancing in time above 40% HRR compared to GB (P = 0.02) and OSFA (P = 0.02). Enjoyment ratings were high (6.4 ± 0.7/7) and consistent between all algorithms (TC vs. GB: P = 0.80 and TC vs. OSFA: P = 0.19). CONCLUSION: TC shows promise in balancing game success and enjoyment but improvements are needed to balance between GMFCS levels for cardiovascular exercise.

Change in pain status in children with cerebral palsy.

AIM: To identify factors associated with a change in pain over time in children with cerebral palsy (CP). METHOD: Pain was assessed at two time-points by physicians and caregiver-rated Health Utilities Index 3 (HUI3) pain scores. RESULTS: One hundred and forty-eight children out of 179 approached from outpatient clinics (83% response; 104 males, 44 females mean age 8y 8mo, range 3y-16y) across all Gross Motor Function Classification System (GMFCS) levels were included. Fifty-five percent had changes in caregiver-reported HUI3 pain. A backward stepwise multiple linear regression retained HUI3 pain score at visit 1 and GMFCS level (F[2,144] =23.40, R2 =0.35; p<0.001) as variables associated with a change in pain status (HUI3 pain at visit 1: ß=0.61, p<0.001; GMFCS level: ß=-0.17, p<0.015). The association between HUI3 pain at visit 1 and GMFCS level was significant (ß=-0.15, p<0.036). There was an association between pain etiology and pain trajectory (F[3,144] =5.39, p=0.002). Post-hoc testing revealed musculoskeletal pain had the greatest improvements compared with the no pain group (p=0.006). INTERPRETATION: Children with CP with more severe initial pain and higher gross motor function have lower pain at follow-up indicating an improvement in pain status over time.

Informing evidence-based clinical practice guidelines for children with cerebral palsy at risk of osteoporosis: an update.

AIM: To investigate the impact of new evidence for weight-bearing, bisphosphonates, and vitamin D and calcium interventions, towards updating the systematic review and clinical practice guidelines for osteoporosis in children with cerebral palsy (CP) published in 2011. METHOD: Computer-assisted literature searches were conducted for articles published from 2010 to 2016. Searches focused on children with CP functioning at Gross Motor Function Classification System levels III to V and limited to weight-bearing activities, bisphosphonates, and vitamin D and/or calcium supplementation. Articles were classified according to the American Academy of Neurology guidelines to update the grading of the evidence for improving bone mineral density (BMD) and decreasing fragility fractures. RESULTS: Six new articles underwent full-text review and data abstraction. These included one weight-bearing, three bisphosphonate, and two mixed intervention studies (bisphosphonate and vitamin D/calcium supplementation). Overall, there continues to be 'probable' evidence for bisphosphonates, 'possible' evidence for vitamin D/calcium, and 'insufficient' evidence for weight-bearing activities as effective interventions to improve low BMD in children with CP. There is 'possible' evidence for bisphosphonates in reducing fragility fractures. INTERPRETATION: The grading of evidence to support the use of weight-bearing activities, bisphosphonates, and vitamin D and calcium supplementation in pediatric CP osteoporosis clinical practice guidelines remained the same.

An innovative cycling exergame to promote cardiovascular fitness in youth with cerebral palsy.

OBJECTIVE: To evaluate the effects of an internet-platform exergame cycling programme on cardiovascular fitness of youth with cerebral palsy (CP). METHODS: In this pilot prospective case series, eight youth with bilateral spastic CP, Gross Motor Functional Classification System (GMFCS) level III, completed a six-week exergame programme. Outcomes were obtained at baseline and post-intervention. The primary outcome measure was the GMFCS III-specific shuttle run test (SRT-III). Secondary outcomes included health-related quality of life (HQL) as measured by the KIDSCREEN-52 questionnaire, six-minute walk test, Wingate arm cranking test and anthropomorphic measurements. RESULTS: There were significant improvements in the SRT-III (t = -2.5, p = 0.04, d = 0.88) post-intervention. There were no significant changes in secondary outcomes. CONCLUSION: An exergame cycling programme may lead to improvement in cardiovascular fitness in youth with CP. This study was limited by small sample size and lack of a comparison group. Future research is warranted.

Neuroplastic Sensorimotor Resting State Network Reorganization in Children With Hemiplegic Cerebral Palsy Treated With Constraint-Induced Movement Therapy.

Using resting state functional magnetic resonance imaging (MRI), we aim to understand the neurologic basis of improved function in children with hemiplegic cerebral palsy treated with constraint-induced movement therapy. Eleven children including 4 untreated comparison subjects diagnosed with hemiplegic cerebral palsy were recruited from 3 clinical centers. MRI and clinical data were gathered at baseline and 1 month for both groups, and 6 months later for the case group only. After constraint therapy, the sensorimotor resting state network became more bilateral, with balanced contributions from each hemisphere, which was sustained 6 months later. Sensorimotor resting state network reorganization after therapy was correlated with a change in the Quality of Upper Extremity Skills Test score at 1 month (r = 0.79, P = .06), and Canadian Occupational Performance Measure scores at 6 months (r = 0.82, P = .05). This clinically correlated resting state network reorganization provides further evidence of the neuroplastic mechanisms underlying constraint-induced movement therapy.

Investigating the impact of pain, age, Gross Motor Function Classification System, and sex on health-related quality of life in children with cerebral palsy.

AIM: To explore whether health-related quality of life (HRQOL) can be predicted by pain, age, Gross Motor Function Classification System (GMFCS) level, and sex in children with cerebral palsy (CP) and whether different pain etiologies have varying effects on HRQOL. METHODS: Children with CP aged 3 to 19 years and their caregivers were consecutively recruited. Caregivers reported their child's pain (Health Utilities Index 3 [HUI3] pain subset) and HRQOL (DISABKIDS questionnaires). Physicians identified pain etiologies. A multiple linear regression model determined whether pain, GMFCS level, sex, and age predicted HRQOL. An ANOVA evaluated the effects of pain etiologies on HRQOL. RESULTS: Three hundred and forty-four participants were approached and 87% (n=300) participated. Sufficient data were available on 248 (72% of total sample). Sixty-six participants (27%) formed the pain group with HUI3 pain scores of at least 3. The presence of pain and increasing age significantly negatively predicted HRQOL (p<0.001, R(2) =0.141), while GMFCS and sex did not. Musculoskeletal deformity (24%) and hypertonia (18%) were the most frequent pain causes. HRQOL statistically differed depending on the pain etiology (p=0.028) with musculoskeletal deformity showing the lowest mean HRQOL. INTERPRETATION: The presence of pain and increasing age negatively predict HRQOL in CP. musculoskeletal deformity has the greatest negative impact on HRQOL.

Resting State and Diffusion Neuroimaging Predictors of Clinical Improvements Following Constraint-Induced Movement Therapy in Children With Hemiplegic Cerebral Palsy.

The aim was to identify neuroimaging predictors of clinical improvements following constraint-induced movement therapy. Resting state functional magnetic resonance and diffusion tensor imaging data was acquired in 7 children with hemiplegic cerebral palsy. Clinical and magnetic resonance imaging (MRI) data were acquired at baseline and 1 month later following a 3-week constraint therapy regimen. A more negative baseline laterality index characterizing an atypical unilateral sensorimotor resting state network significantly correlated with an improvement in the Canadian Occupational Performance Measure score (r = -0.81, P = .03). A more unilateral network with decreased activity in the affected hemisphere was associated with greater improvements in clinical scores. Higher mean diffusivity in the posterior limb of the internal capsule of the affect tract correlated significantly with improvements in the Jebsen-Taylor score (r = -0.83, P = .02). Children with more compromised networks and tracts improved the most following constraint therapy.

Further evaluation of the scoring, reliability, and validity of the Hypertonia Assessment Tool (HAT).

We assessed the impact of videotape analysis on scoring of the Hypertonia Assessment Tool (HAT) that discriminates between hypertonia subtypes. The HAT was administered to 28 children with cerebral palsy (mean age 9 years, range 4-17 years, 61% male). HAT examinations were videotaped; scores were assigned before and after videotape review. Neurological examination provided the gold standard diagnosis. Interrater reliability, criterion validity and individual item validation were assessed using prevalence and bias-adjusted kappa (PABAK). Videotape review did not significantly change the HAT item scores or diagnoses. Item validation eliminated 1 dystonia item. Interrater reliability was moderate for dystonia (PABAK = 0.43) and excellent for spasticity and rigidity (PABAK = 0.86-1.0). Criterion validity was substantial for spasticity (PABAK = 0.71), moderate for dystonia (PABAK = 0.43-0.57) and excellent for the absence of rigidity (PABAK = 1.0). The HAT can be administered without videotape review. Dystonia item 1 did not change the HAT hypertonia diagnosis and will be removed from the HAT.

Interactive computer play as "motor therapy" for individuals with cerebral palsy.

The aim of the study was to evaluate the quality of evidence for interactive computer play (ICP) to improve motor performance (including motor control, strength, or cardiovascular [CVS] fitness) in individuals with cerebral palsy. A computer-assisted literature search was completed, focusing on ICP as a therapeutic modality to improve motor outcomes in individuals of all ages with cerebral palsy with a specific focus on upper and lower extremity motor outcomes and promotion of CVS fitness. Articles were classified according to American Academy of Neurology guidelines and recommendation classifications were given based on the levels of evidence. Seventeen articles underwent full-text review including 6 on upper extremity motor function, 5 on lower extremity motor function, 1 on CVS fitness, and 5 on studies with a combination of upper or lower extremity or CVS fitness focus or both. Overall, there was level B (probable) evidence for ICP interventions to improve lower extremity motor control or function. However, there was inadequate evidence (level U) for ICP interventions improving upper limb motor control or function or CVS fitness. Although promising trends are apparent, the strongest level of evidence exists for the use of ICP to improve gross motor outcomes. Additional evidence is warranted especially when evaluating the effect of ICP on upper limb motor outcomes and CVS fitness.

Characteristics of pain in children and youth with cerebral palsy.

OBJECTIVES: Pain in children with cerebral palsy (CP) is underrecognized, undertreated, and negatively affects quality of life. Communication challenges and multiple pain etiologies complicate diagnosis and treatment. The primary objectives of this study were to determine the impact of pain on activities and to identify the common physician-identified causes of pain in children and youth ages 3 to 19 years across all levels of severity of CP. METHODS: The study design was cross-sectional, whereby children/youth aged 3 to 19 years and their families were consecutively recruited. The primary caregivers were asked to complete a one-time questionnaire, including the Health Utilities Index 3 pain subset, about the presence and characteristics of pain. The treating physician was asked to identify the presence of pain and provide a clinical diagnosis for the pain, if applicable. RESULTS: The response rate was 92%. Of 252 participants, 54.8% reported some pain on the Health Utilities Index 3, with 24.4% of the caregivers reporting that their child experienced pain that affected some level of activities in the preceding 2 weeks. Physicians reported pain in 38.7% and identified hip dislocation/subluxation, dystonia, and constipation as the most frequent causes of pain. CONCLUSIONS: One-quarter of our sample experienced pain that limited activities and participation. Clinicians should be aware that hip subluxation/dislocation and dystonia were the most common causes of pain in children/youth with CP in this study. Potential causes of pain should be identified and addressed early to mitigate the negative impact of pain on quality of life.