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Aim: To determine if pain is associated with 12-month incidence of mood affective disorders (MAD) in adults with cerebral palsy (CP).Method: Data from Optum Clinformatics® Data Mart (2013-2016) were used for this retrospective cohort study. Diagnostic codes were used to identify adults (≥18y) with CP, incident cases of MAD, and covariates (other neurodevelopmental conditions, sleep disorders, arthritis). Pain (any type, location) was identified between 1st October 2014 and 30th September 2015. The pain group was divided into new or consistent pain if they had a history of pain (i.e. consistent) in the 12 months before their first pain claim date. Crude incidence rates of MAD (expressed per 100 person-years) were estimated. Cox regression was used to estimate hazard ratio (95% confidence interval [CI]) of MAD after adjusting for covariates.Results: Adults that had new pain (n=859; incidence rate=15.5) or consistent pain (n=1303; incidence rate=17.9) had greater crude incidence rate of MAD compared to adults without pain (n=3726; incidence rate=5.9). The elevated rate of MAD remained after adjusting for covariates, for new pain (hazard ratio=2.4; 95% CI=1.9-3.0) and consistent pain (hazard ratio=2.1; 95% CI=1.7-2.7).Interpretation: Pain is associated with greater incidence of MAD in adults with CP. This association remained after accounting for potential confounding factors.What This Paper Adds: What this paper adds Pain was associated with higher 12-month incidence of mood affective disorders (MAD). The 12-month MAD incidence was similar between new and consistent pain groups. The MAD incidence remained higher adjusting for neurodevelopmental comorbidities, sleep disorders, and arthritis. [ABSTRACT FROM AUTHOR]

Aim: To validate the Whitney Comorbidity Index (WCI), which was recently developed to monitor disease status for adults with cerebral palsy (CP), and to identify WCI scores associated with an increased mortality risk using a representative sample of adults with CP.Method: Data from 2016 to 2018 were used from a random 20% sample from the fee-for-service Medicare database for this retrospective cohort study. The WCI was examined as unweighted (WCIunw ) and weighted (WCIw ) among adults at least 18 years old with CP. Cox regression models were developed with mortality as the outcome after adjusting for demographics. A concordance statistic (C-statistic) of at least 0.70 was considered as showing sufficient validity. The hazard ratio of mortality for each WCI score was estimated. Secondary analyses were performed for subgroups with co-occurring epilepsy and/or intellectual disabilities.Results: For the entire group (n=16 728) and subgroups, the WCI showed sufficient validity (C-statistic 0.73-0.81). For the entire group, the mortality rate was elevated for a score of 1 compared with 0 from the WCIunw (hazard ratio 3.06; 95% confidence interval [CI] 1.52-6.17) and WCIw (hazard ratio 4.08; 95% CI 1.69-9.85), and became larger with each WCI score. Results were similar for the subgroups.Interpretation: The WCI is a valid marker for health/disease status for adults with CP. Several WCI score thresholds were identified to assist in clinical decision making for preventive medicine and intervention implementation. What this paper adds The Whitney Comorbidity Index (WCI) is valid among 16 728 adults with CP. The WCI is valid for those with co-occurring epilepsy and/or intellectual disabilities. Thresholds of the WCI score were identified to assist clinical decision making. [ABSTRACT FROM AUTHOR]

Aim: To determine if walking ability and presence of intellectual disability and epilepsy change from childhood to 50 years of age in individuals with cerebral palsy (CP), and if such changes are related to age, sex, or CP subtype.Method: This was a population-based follow-up study of 142 adults born from 1959 to 1978 (82 males, 60 females; mean age 48y 4mo, range 37-58y; 44% unilateral, 35% bilateral, 17% dyskinetic, and 4% ataxic CP) listed in the CP register of western Sweden. We compared childhood data with a follow-up assessment in 2016.Results: At follow-up, walking ability had changed significantly (p<0.001). The proportion of participants walking without aids had decreased from 71% to 62%, and wheelchair ambulation increased from 18% to 25%. Walking ability was related to subtype (p=0.001), but not to age, sex, pain, fatigue, or body mass index. The proportion classified as having intellectual disability had increased from 16% to 22% (p=0.039) and the proportion with epilepsy from 9% to 18% (p=0.015). Of those with childhood epilepsy, 46% were seizure-free without medication.Interpretation: Walking ability and the presence of intellectual disability and epilepsy had changed significantly since childhood. Life-long access to specialized health care is warranted for re-evaluation of impairments, treatment, and assistance. What this paper adds Changes in impairments in individuals with cerebral palsy (CP) over time are related to CP subtype. After 50 years, walking ability in CP may have deteriorated or improved. Intellectual disability in CP may not always be detected in early childhood assessments. Epilepsy in CP may develop after childhood or may be outgrown. [ABSTRACT FROM AUTHOR]

Aim: To describe the trajectory of clinical signs in children who developed human immunodeficiency virus encephalopathy (HIVE) after starting early antiretroviral therapy (ART).Method: This was a retrospective case-cohort description of HIVE among Cape Town participants from the Children with HIV Early AntiRetroviral treatment (CHER) trial. Criteria for HIVE diagnosis were at least two of: (1) acquired central motor deficit, (2) impaired brain growth, and (3) failure to attain or loss of developmental milestones in the absence of an alternative aetiology.Results: Of 133 surviving participants who initiated ART at a median age of 9 weeks and who were followed until a median age of 6 years, 20 (12%) developed HIVE at a median age 31 months (interquartile range 19-37). In these, the first neurological deterioration was noticed at a median age of 19 months, when 16 were on ART and nine had undetectable HIV viral load for a median of 12 months. Signs of upper motor neurons were present in 18, of whom 12 resolved and four had persistent spastic diplegia; 19 had motor delay, of whom 14 resolved; 12 had language delay, of whom 11 resolved; and 16 had impaired brain growth, of whom only five recovered. For the 16 participants already on ART at HIVE diagnosis, regimens were not altered in response to diagnosis.Interpretation: HIVE may occur despite early ART initiation and virological suppression and then resolve on unchanged ART, most likely as intrathecal inflammation subsides.What This Paper Adds: Despite suppressive antiretroviral therapy, children can develop human immunodeficiency virus encephalopathy, The most common manifestations are motor deficits and impaired brain growth. Most experience improvement, with many resolving without additional intervention. [ABSTRACT FROM AUTHOR]

Aim: To evaluate pain prevalence and characteristics in children and adolescents with predominant dyskinetic and mixed (dyskinetic/spastic) cerebral palsy (CP) motor types.Method: Seventy-five participants with a diagnosis of CP and confirmed dyskinetic or mixed (dyskinetic/spastic) motor type took part in a multisite cross-sectional study. The primary outcome was carer-reported pain prevalence (preceding 2wks) measured using the Health Utilities Index-3. Secondary outcomes were chronicity, intensity, body locations, quality of life, and activity impact.Results: Mean participant age was 10 years 11 months (SD 4y 2mo, range 5-18y). There were 44 males and 31 females and 37 (49%) had predominant dyskinetic CP. Pain was prevalent in 85% and it was chronic in 77% of participants. Fifty-two per cent experienced moderate-to-high carer-reported pain intensity, which was significantly associated with predominant dyskinetic motor types (p=0.008). Pain occurred at multiple body locations (5 out of 21), with significantly increased numbers of locations at higher Gross Motor Function Classification System levels (p=0.02). Face, jaw, and temple pain was significantly associated with predominant dyskinetic motor types (p=0.005). Poorer carer proxy-reported quality of life was detected in those with chronic pain compared to those without (p=0.03); however, chronic pain did not affect quality of life for self-reporting participants.Interpretation: Pain was highly prevalent in children and adolescents with predominant dyskinetic and mixed (dyskinetic/spastic) motor types, highlighting a population in need of lifespan pain management.What This Paper Adds: Chronic pain prevalence in children and adolescents with predominant dyskinetic and mixed (dyskinetic/spastic) motor types is high. Pain occurs across multiple body locations in predominant dyskinetic and mixed (dyskinetic/spastic) motor types. Less recognized locations of pain include the face, jaw, and temple for predominant dyskinetic motor types. [ABSTRACT FROM AUTHOR]

Aim: To evaluate the effect of progressive resistance training of the ankle plantarflexors on gait efficiency, activity, and participation in adolescents with cerebral palsy (CP).Method: Sixty-four adolescents (10-19y; 27 females, 37 males; Gross Motor Function Classification System [GMFCS] levels I-III) were randomized to 30 sessions of resistance training (10 supervised and 20 unsupervised home sessions) over 10 weeks or usual care. The primary outcome was gait efficiency indicated by net nondimensional oxygen cost (NNcost). Secondary outcomes included physical activity, gross motor function, participation, muscle strength, muscle and tendon size, and muscle and tendon stiffness. Analysis was intention-to-treat.Results: Median attendance at the 10 supervised sessions was 80% (range 40-100%). There was no between-group difference in NNcost at 10 (mean difference: 0.02, 95% confidence interval [CI] -0.07 to 0.11, p=0.696) or 22 weeks (mean difference: -0.08, 95% CI -0.18 to 0.03, p=0.158). There was also no evidence of between-group differences in secondary outcomes at 10 or 22 weeks. There were 123 adverse events reported by 27 participants in the resistance training group.Interpretation: We found that 10 supervised sessions and 20 home sessions of progressive resistance training of the ankle plantarflexors did not improve gait efficiency, muscle strength, activity, participation, or any biomechanical outcome among adolescents with CP.What This Paper Adds: Thirty sessions of progressive resistance training of the ankle plantarflexors over 10 weeks did not improve gait efficiency among ambulatory adolescents with cerebral palsy. Resistance training did not improve muscle strength, activity, or participation. Ninety percent of participants experienced an adverse event. Most adverse events were expected and no serious adverse events were reported. [ABSTRACT FROM AUTHOR]

Aim: To assess the efficacy, safety, and tolerability of oromucosal nabiximols cannabinoid medicine as adjunct therapy for children with spasticity due to cerebral palsy/traumatic central nervous system injury with inadequate response to existing treatment.Method: Overall, 72 patients (mean [SD] age 12y 4mo [3y 1mo], range 8-18y) were randomized at a ratio of 2:1 to receive nabiximols (n=47; 29 males, 18 females) or placebo (n=25; 15 males, 10 females) for 12 weeks (12 sprays/day max. based on clinical response/tolerability). The primary outcome was change from baseline in level of spasticity on a 0 to 10 Numerical Rating Scale (NRS), assessed by the primary caregiver at 12 weeks. Secondary outcomes included additional measures for spasticity, sleep quality, pain, health-related quality of life, comfort, depression, and safety.Results: There was no significant difference in the spasticity 0 to 10 NRS between nabiximols versus placebo groups after 12 weeks. No statistically significant differences were observed for any secondary endpoint. Adverse events were predominantly mild or moderate in severity; however, three cases of hallucinations were reported.Interpretation: Nabiximols was generally well tolerated; however, neuropsychiatric adverse events were observed. No significant reduction in spasticity with nabiximols treatment versus placebo was observed.What This Paper Adds: Oromucosal nabiximols is generally well tolerated by paediatric patients. However, three cases of hallucinations were observed, one of which involved auditory hallucinations and a suicide attempt. Oromucosal nabiximols versus placebo did not reduce cerebral palsy/central nervous system injury-related spasticity. [ABSTRACT FROM AUTHOR]

Aim: To assess the neural and non-neural contributions to spasticity in the impaired ankle of children with cerebral palsy (CP).Method: Instrumented tapping of the Achilles tendon was done isometrically to minimize non-neural contributions and elicit neural contributions. Robot-controlled ankle stretching was done at various velocities, including slow stretching, with minimized neural contributions. Spasticity was assessed as having neural (phasic and tonic stretch reflex torque, tendon reflex gain, contraction rate, and half relaxation rate) and non-neural origin (elastic stiffness and viscous damping) in 17 children with CP (six females and 11 males; mean age [SD] 10y 8mo [3y 11mo], range 4y-18y) and 17 typically developing children (six females and 11 males; mean age [SD] 12y 7mo [2y 9mo], range 7y-18y). All torques were normalized to weight×height.Results: Children with CP showed increased phasic and tonic stretch reflex torque (p=0.004 and p=0.001 respectively), tendon reflex gain (p=0.02), contraction rate (p=0.038), half relaxation rate (p=0.02), elastic stiffness (p=0.01), and viscous damping (p=0.01) compared to typically developing children.Interpretation: Controlled stretching and instrumented tendon tapping allow the systematic quantification of various neural and non-neural changes in CP, which can be used to guide impairment-specific treatment.What This Paper Adds: Ankle spasticity is associated with increased phasic and tonic stretch reflexes, tendon reflex gain, and contraction and half relaxation rates. Ankle spasticity is also associated with increased elastic stiffness and viscous damping. [ABSTRACT FROM AUTHOR]

Aim: To investigate the underlying factor structure of the 46-item Flemish cerebral visual impairment (CVI) questionnaire, differentiate the factor scores of children with and without CVI, and examine the impact of comorbidities on factor scores.Method: The records of 630 children (386 males, 244 females; median age 77mo; interquartile range 63-98mo) who visited the CVI clinic and the Centre for Developmental Disabilities at the University Hospitals of Leuven from 2001 to 2018 were reviewed systematically. Inclusion criteria included an up-to-date questionnaire, a definitive diagnosis, and clinical assessment.Results: Three hundred and forty-five children (179 with CVI [108 males, 71 females; median age 74mo; interquartile range 61-93mo] and 166 without CVI [110 males, 56 females; median age 88mo; interquartile range 70-107mo]) were included. An exploratory factor analysis resulted in a 5-factor (object and face processing impairments; visual (dis)interest; clutter and distance viewing impairments; moving in space impairments; and anxiety-related behaviours) biologically and clinically plausible model, which retained 35 items and explained 56% of the total variance. Mann-Whitney U tests indicated that factors 1 to 4 were significantly higher in children with CVI compared to children without CVI (p-values ranged from p<0.001 to p<0.05; effect sizes ranged from 0.11 to 0.33); factor 5 showed no differences. Autism, developmental coordination disorder, epilepsy, and cerebral palsy impacted factor scores.Interpretation: A 5-factor structure of the Flemish CVI questionnaire differentiates children with and without CVI. Comorbidities should be accounted for when researching CVI.What This Paper Adds: Cerebral visual impairment (CVI) is characterized by impaired object and face processing and impaired visual interest. CVI is also characterized by impaired clutter and distance viewing, and impaired moving in space. All children (with or without CVI) demonstrated anxiety-related behaviours. Autism affected object/face processing, whereas developmental coordination disorder, epilepsy, and cerebral palsy affected visual interest. [ABSTRACT FROM AUTHOR]

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

Aim: To explore how patients with cerebral palsy (CP) perceive their gait and evaluate associations between subjective gait perception and: objective gait parameters, endurance, pain, and fatigue.Method: Sixty-two patients (21 females and 41 males; mean [SD] age 20y [5y 1mo], range 15-29y) performed a clinical gait analysis. Self-selected walking speed, Gait Profile Score, and Gait Variable Score were calculated. Subjective gait perception was assessed with a visual analogue scale using the question: 'On a scale from 0 (worst) to 10 (optimal), how would you describe your walking today?'. A 6-minute walk test (6MWT) measured endurance; the 36-Item Short Form Health Survey (SF-36) evaluated quality of life. T-tests, Pearson correlations, and univariate and multiple linear regression models were used to compare and find associations between the data.Results: Overall mean (SD) subjective gait perception was 7.5 (1.8) and was significantly higher for patients in Gross Motor Function Classification System (GMFCS) level I (7.9 [1.5]) than for patients in GMFCS levels II and III (5.9 [2.0]). Positive correlations were found between subjective gait perception and gait scores, walking speed, 6MWT distance, and SF-36 score. Only walking speed was a significant predictor of subjective gait perception.Interpretation: Subjective gait perception was influenced by GMFCS level and linked partially with the walking speed. The gait quality did not explain subjective gait perception. It is important to combine subjective and objective gait scores to develop personalized therapeutic goals.What This Paper Adds: Subjective gait perception is influenced by the physical impairment levels of patients with cerebral palsy. Subjective gait perception and objective gait scores are associated. Walking speed is the only predictor of gait perception. [ABSTRACT FROM AUTHOR]

Aim: To describe health-related quality of life (HRQoL), pain, fatigue, and other health variables in young adults with cerebral palsy (CP), and to explore associations with the Gross Motor Function Classification System - Expanded and Revised (GMFCS-ER) and physical activity.Method: This was a cross-sectional study of 61 young adults at a mean age of 21 years 2 months (standard deviation 8mo, range 20-22y) with CP, from a geographically defined area. Data collection included: Short Form 36 version 2 for HRQoL, Brief Pain Inventory - Short Form, Fatigue Severity Scale, level of physical activity, medical history, and physical examination.Results: Overall HRQoL equalled that of population norms; however self-reported physical health was lower in GMFCS-ER levels III to V compared to GMFCS-ER levels I to II. Self-reported mental health was, inversely, lower in GMFCS-ER levels I to II compared to GMFCS-ER levels III to V. Pain prevalence was 49%, and pain was present across all GMFCS-ER levels. Fatigue, as well as sleep problems, had 41% prevalence, with fatigue severity decreasing with increasing level of physical activity.Interpretation: General HRQoL in young adults with CP was comparable to population norms. Pain and fatigue are important to address in high motor-functioning individuals also. Physical activity could be a possible protective factor against fatigue.What This Paper Adds: Health-related quality of life in young adults with cerebral palsy (CP) was comparable to population norms. Pain, fatigue, and sleep problems occurred at all Gross Motor Function Classification System levels. There is a possible protective effect of physical activity on fatigue. [ABSTRACT FROM AUTHOR]

Aim: To evaluate whether spasticity measures are related to pain in adults with cerebral palsy (CP).Method: This cross-sectional study recruited individuals aged 16 to 89 years with a diagnosis of CP. Participants completed the Penn Spasm Frequency Scale (PSFS), Brief Pain Inventory (BPI), and PROMIS Pain Interference measures. The Modified Ashworth Scale (MAS) and Tardieu spasticity angles of six joints were rated and summed to composite MAS and Tardieu scores for each participant. Associations between spasticity and pain measures were evaluated.Results: Forty-seven participants (27 females, 20 males) with a mean age of 35 years 7 months (range 18-77y) spanning all Gross Motor Function Classification System (GMFCS) levels were included. Twenty-six participants reported their average pain level on BPI as greater than 0 over the past week (median pain level 4.0). Median PSFS was 1.0 (range 0.0-1.0) and this correlated with average BPI and Pain Interference T scores (median 40.7; ρ=0.33 and ρ=0.31 respectively [both p=0.01]). When adjusted for pain medication use and age, MAS correlated with BPI (ρ=0.30; p=0.04). Other pain and spasticity measures, or GMFCS level, were not significantly related with pain interference or BPI rating. Age was weakly associated with BPI (slope=0.10; p<0.01).Interpretation: PROMIS Pain Interference was lower than population-based norms. Patient-rated spasm frequency demonstrated better association with pain levels and interference than physician-rated MAS and Tardieu.What This Paper Adds: Pain was not associated with Gross Motor Function Classification System level. Pain increased with age, as anticipated. Self-reported spasm scores were associated with increased pain in contrast to clinical examination scales. Adjusted, summed spasticity on the Modified Ashworth Scale was associated with pain scores on the Brief Pain Inventory. Although pain is experienced by adults with cerebral palsy, pain did not interfere with activities. [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 prevalence of cerebral palsy (CP), subtype distribution, motor and intellectual impairment, and epilepsy in adults with CP compared with children with CP.Method: CP subtype and impairment data from the population-based CP register of western Sweden and population data from Statistics Sweden were used to compare surviving adults (n=581; 244 females, 337 males) born between 1959 and 1978, with the same cohort as children (n=723; 307 females, 416 males), and with the most recent cohort, born from 2007 to 2010 (n=205; 84 females, 121 males).Results: Prevalence of CP in adults born between 1959 and 1978 was 1.14 per 1000. The occurrence of impairments differed between CP subtypes. Motor and intellectual impairment were closely related, regardless of subtype. Subtype distribution among survivors differed significantly from the original cohorts (p=0.002), and the most recent cohort (p<0.01), tetraplegia and dyskinetic CP being less common in survivors. Severe motor impairment, intellectual disability, and epilepsy were less common among survivors than in the original cohorts (p=0.004, p=0.002, p=0.037) and the most recent cohort (p=0.004, p=0.008, p<0.01).Interpretation: Data on prevalence, subtype distribution, and impairments in children with CP are not applicable to adults with CP. Population-based studies of adults with CP are needed.What This Paper Adds: Cerebral palsy (CP) subtypes are differently distributed in adults compared to children. The prevalence of impairments in adults with CP is related to CP subtype. Spastic tetraplegia and dyskinetic CP are less common in adults than children. Severe motor impairment, intellectual disability, and epilepsy are less common in adults. [ABSTRACT FROM AUTHOR]

Aim: This cross-sectional investigation evaluated whether recurrent botulinum neurotoxin A (BoNT-A) interventions to the medial gastrocnemius have an influence on muscle morphology, beyond Gross Motor Function Classification System (GMFCS) level.Method: A cohort of typically developing children (n=67; 43 males, 24 females; median age 9y 11mo [range 7y 10mo-11y 6mo]), a cohort of children with spastic cerebral palsy (CP) naive to BoNT-A interventions (No-BoNT-A; n=19; 10 males, nine females; median age 9y 3mo [range 8y 5mo-10y 10mo]) and a cohort of children with spastic CP with a minimum of three recurrent BoNT-A interventions to the medial gastrocnemius (BoNT-A; n=19; 13 males, six females; median age 9y 8mo [range 7y 3mo-10y 7mo]) were recruited. Three-dimensional freehand ultrasound was used to estimate medial gastrocnemius volume normalized to body mass and echo-intensity.Results: Normalized medial gastrocnemius volume and echo-intensity significantly differed between the two spastic CP cohorts (p≤0.05), with the BoNT-A cohort having larger alterations. Associations between normalized medial gastrocnemius volume and echo-intensity were highest in the No-BoNT-A cohort, followed by the BoNT-A cohort. Multiple regression analyses revealed that both GMFCS level and BoNT-A intervention history were significantly associated with smaller normalized medial gastrocnemius volume and higher echo-intensity.Interpretation: Recurrent BoNT-A interventions may induce alterations to medial gastrocnemius volume and echo-intensity beyond the natural history of the spastic CP pathology.What This Paper Adds: In spastic cerebral palsy, medial gastrocnemius volumes are smaller and echo-intensities higher compared with typical development. Alterations after botulinum neurotoxin A intervention (BoNT-A) are larger than in no BoNT-A intervention. Gross Motor Function Classification System level and BoNT-A history significantly associate with medial gastrocnemius and echo-intensity alterations. [ABSTRACT FROM AUTHOR]

Aim: To examine the longitudinal trends of cardiometabolic diseases in a large sample of adults with cerebral palsy (CP).Method: The Optum Clinformatics Data Mart is a de-identified nationwide claims database of beneficiaries from a single private payer. Beneficiaries were included if they had an International Classification of Diseases, Ninth Revision, Clinical Modification code for a diagnosis of CP. Adults with at least 3 years of continuous enrollment on a single plan between 2002 and 2009 were included in the final analyses (n=2659). We examined the longitudinal trends of incident diabetes mellitus, hypercholesterolemia, hypertension, cardiac dysrhythmias, and atherosclerosis, stratified by age categories: 18 to 39 years, 40 to 59 years, and 60 years and over. Kaplan-Meier product-limit survival curves were compared across age categories for each of the cardiometabolic outcomes, and a Cox proportional hazards regression was run to determine adjusted hazard ratios.Results: The cumulative incidence of each of the cardiometabolic diseases ranged from 6.0% for atherosclerosis to 34.4% for hypercholesterolemia at 3 years and over. Risk-adjusted Cox proportional hazard models revealed that age was a robust predictor of survival for each outcome, with higher hazard ratio ranges in middle age (hazard ratio 1.41-2.72) and older adults (hazard ratio 2.20-5.93) compared with young adults.Interpretation: Adults with CP have high rates of cardiometabolic diseases; and disease-free survival shortens significantly with higher ages.What This Paper Adds: Adults with cerebral palsy have high rates of cardiometabolic diseases. Disease-free survival of all cardiometabolic diseases shortens significantly with higher ages. The highest rates were for hypercholesterolemia and hypertension. [ABSTRACT FROM AUTHOR]

Aim: To investigate trends in birth prevalence of cerebral palsy (CP) overall and by gestational age, and examine the distribution of motor type, spastic topography, and severity using Australian CP Register data from 1995 to 2009.Method: Prenatal and perinatal CP data were collated from state/territory CP registers. Birth prevalence estimates per 1000 live births and per 1000 neonatal survivors (NNS) were calculated in five epochs. Data from three state registers with population-level ascertainment were used to investigate birth prevalence trends by gestational age using Poisson regression. Distribution of motor type, spastic topography, and moderate to severe disability (IQ≤50 and/or Gross Motor Function Classification System levels III-V) were evaluated within birthweight categories.Results: Birth prevalence of CP varied across population-level states but within each state declined significantly over time (p<0.05). Birth prevalence per 1000 neonatal survivors declined amongst children born before 28 weeks (South Australia, Victoria p<0.001) and those born at or after 37 weeks (Victoria p<0.001, Western Australia p<0.002). Across Australia the percentage of children with bilateral spastic CP declined amongst those born less than 1000g. The percentage of children with moderate to severe disability decreased (48%-34%, p<0.001).Interpretation: Birth prevalence of CP declined. Encouragingly, the percentage of children with CP whose disability was moderate to severe also decreased.What This Paper Adds: Birth prevalence of cerebral palsy (CP) differed but declined across Australian states (1995-2009). Australian CP birth prevalence declined significantly amongst children born before 28 weeks and those born at or after 37 weeks. The percentage of children with moderate to severe disability decreased. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to investigate the longitudinal development of passive range of motion (ROM) in the upper limbs in a population-based sample of children with cerebral palsy (CP), and to investigate which children are more likely to develop contractures related to functional level, CP subtype, and age.Method: Registry data of annual passive ROM measurements of the upper limbs from 771 children with CP (417 males, 354 females; mean age 11y 8mo, [SD 5mo] range 1-18y) were analysed. Mixed models were used to investigate at what age decreased passive ROM occurs. Odds ratios were calculated to compare risks and logistic regression analysis was used to predict contracture development.Results: Thirty-four per cent of the children had developed contractures. Among these children, decreased passive ROM was significant at a mean age of 4 years for wrist extension and 7 years for shoulder flexion, elbow extension, and supination. Children at Manual Ability Classification System (MACS) level V had a 17-times greater risk of contractures than children at MACS level I.Interpretation: One-third of the children in the total population developed upper-limb contractures while passive ROM decreased with age. MACS level was the strongest predictor of contracture development.What This Paper Adds: In a population-based sample of 771 children with cerebral palsy, 34% developed an upper-limb contracture. Contracture development started at preschool age. The first affected movements were wrist extension and supination. Passive range of motion decreased with age. High Manual Ability Classification System level was the most important predictor of contractures. [ABSTRACT FROM AUTHOR]

Aim: To determine the long-term course of difficulty in participation of individuals with cerebral palsy (CP) without intellectual disability between 16 years and 34 years of age.Method: One hundred and fifty-one individuals with CP aged 16 to 20 years were included (63% male, 37% female; Gross Motor Function Classification System [GMFCS] levels I-IV; without intellectual disability). The Assessment of Life Habits questionnaire 3.0 general short form was used up to three times biennially and at 13-year follow-up (13-year follow-up: n=98). Scores (range 0-10) reflect difficulty and assistance in participation in housing, education and employment, interpersonal relationships, recreation, community life, and responsibilities. Multilevel models were used to determine the course of difficulty in participation by GMFCS level.Results: Despite high average participation levels, 41% to 95% of adolescents and young adults with CP experienced difficulty. Difficulty in participation in housing and interpersonal relationships increased from age 16 years onwards and in most other life areas in the mid- and late 20s. In adolescents in GMFCS levels III and IV, participation in recreation and community life improved up to age 23 years.Interpretation: Individuals with CP experience increasing difficulties in participation in their mid- and late 20s. Clinicians should systematically check for participation difficulties in young adults with CP and offer timely personalized treatment.What This Paper Adds: Many individuals with cerebral palsy (CP) aged 16 to 34 years experience difficulty in participation. Difficulty in participation increases in the mid- and late 20s for individuals with CP. Participation in recreation/community life improves before age 23 years for those in Gross Motor Function Classification System levels III and IV. [ABSTRACT FROM AUTHOR]

Aim: People with cerebral palsy (CP) are often unable to express pain owing to cognitive or speech impairments. Reports that rely on observation can be inaccurate, because behaviours such as grimacing, common in people with spastic CP, resemble pain expressions. We examined preliminary validity and reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale in people with spastic CP.Method: Forty-eight young people and adults (35 females, 13 males; mean [SD] age 29y 2mo [13y]) were video-recorded during a standard examination, rating their pain (0-10) afterwards. Two raters completed the r-FLACC using the video recordings. Interrater reliability was assessed with an unconditional cross-classified random-effects model and item response theory approach; Pearson correlations measured agreement between raters and participants.Results: Mean (SD) participant (n=48) pain scores were 2.48 (2.5) and mean (SD) r-FLACC scores were 1.46 (1.68). There was moderate agreement between raters (intraclass coefficient 0.41 and 0.57 respectively) but low agreement between participants and raters (r=0.26). There were no significant effects for raters (lay observers, nurses, physicians, and inexperienced raters).Interpretation: Results provide mixed support for the interrater reliability of the r-FLACC in people with spastic CP.What This Paper Adds: The revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale can be reliably used by experts and lay raters for people with spastic cerebral palsy (CP). Support is mixed for interrater reliability of the r-FLACC scale used with people with spastic CP. [ABSTRACT FROM AUTHOR]

Aim: This study aimed to track alterations in muscle volume for 6 months in children with cerebral palsy (CP) after the first exposure to botulinum neurotoxin A (BoNT-A), a commonly used focal spasticity treatment.Method: Eleven ambulant children (eight males, three females) with spastic CP, mean age 8 years 10 months (SD 3y 1mo) participated. Participants received injections to the affected gastrocnemius. The muscle volume of the gastrocnemius, soleus, tibialis anterior, and hamstrings was measured using magnetic resonance imaging. Muscle volume was normalized to bone length, and changes analysed relative to baseline. Assessments were conducted 1 week before, and 4 weeks, 13 weeks, and 25 weeks after BoNT-A treatment.Results: All children demonstrated positive clinical and functional gains. Muscle volume of the injected gastrocnemius was found to be significantly reduced at 4 weeks (-5.9%), 13 weeks (-9.4%), and 25 weeks (-6.8%). Significant increases in normalized soleus muscle volume were identified at each follow-up, while hamstrings showed significant increase at 4 weeks only.Interpretation: Absolute and normalized muscle volume of the injected muscle reduces after first BoNT-A exposure, and does not return to baseline volume by 25 weeks. Hypertrophy is seen in the soleus up to 25 weeks; the volume of the plantar flexor compartment is stable.What This Paper Adds: Muscle atrophy after first botulinum neurotoxin A (BoNT-A) exposure in children with cerebral palsy is noted. Mild BoNT-A-induced muscle atrophy is still apparent 6 months after BoNT-A exposure. Hypertrophy is evident in soleus after gastrocnemius BoNT-A exposure. Total plantarflexor volume is unchanged. [ABSTRACT FROM AUTHOR]

Aim: Femoral derotation osteotomy (FDO) can be conducted either proximally or distally to correct internal rotation gait (IRG) and increased anteversion in children with cerebral palsy (CP). Previous studies with limited numbers of participants have presented comparable short-term static and kinematic outcomes for both techniques. The objective of this retrospective multicentre study was to verify this thesis with a larger number of patients.Method: In total, 119 children with CP and IRG were included after matching the groups on preoperative mean stance hip rotation: 67 intertrochanteric (proximal group) FDO (average age at surgery 9y [SD 3y]); 52 supracondylar (distal group) FDO (average age at surgery 12y [SD 3y]). One random limb of each child was analyzed. Both transverse plane kinematic gait data and torsional parameters of clinical examination were assessed preoperatively and postoperatively.Results: On average, both groups' mean hip rotation and midpoint of hip rotation improved postoperatively, and groups did not differ preoperatively or postoperatively for these variables (p>0.05).Interpretation: The osteotomy location does not influence short-term gait kinematics nor static measurements. Therefore, the choice of performing proximal or distal FDO in children with CP for treatment of IRG and increased anteversion should be motivated by considerations other than these outcome parameters.What This Paper Adds: Femoral derotation osteotomy (FDO) location does not influence mean stance hip rotation in cerebral palsy (CP). FDO location does not influence mid-point hip rotation in CP. FDO location should be motivated by concomitant procedures performed. [ABSTRACT FROM AUTHOR]

Aim: To disentangle the respective impacts of manual dexterity and cerebral palsy (CP) in cognitive functioning after neonatal arterial ischaemic stroke.Method: The population included 60 children (21 females, 39 males) with neonatal arterial ischaemic stroke but not epilepsy. The presence of CP was assessed clinically at the age of 7 years and 2 months (range 6y 11mo-7y 8mo) using the definition of the Surveillance of CP in Europe network. Standardized tests (Nine-Hole Peg Test and Box and Blocks Test) were used to quantify manual (finger and hand respectively) dexterity. General cognitive functioning was evaluated with the Wechsler Intelligence Scale for Children, Fourth Edition. Simple and multiple linear regression models were performed while controlling for socio-economic status, lesion side, and sex.Results: Fifteen children were diagnosed with CP. In simple regression models, both manual dexterity and CP were associated with cognitive functioning (β=0.41 [p=0.002] and β=0.31 [p=0.019] respectively). However, in multiple regression models, manual dexterity was the only associated variable of cognitive functioning, whether or not a child had CP (β=0.35; p=0.007). This result was reproduced in models with other covariables (β=0.31; p=0.017).Interpretation: As observed in typically developing children, manual dexterity is related to cognitive functioning in children having suffered a focal brain insult during the neonatal period.What This Paper Adds: Manual dexterity predicts cognitive functioning after neonatal arterial ischaemic stroke. Correlations between manual dexterity and cognitive functioning occur irrespective of sex, lesion side, presence of cerebral palsy, and socio-economic status. Residual motor ability may support cognitive functioning. [ABSTRACT FROM AUTHOR]

Aim: To assess feeding difficulties and nutritional status among children with cerebral palsy (CP) in Ghana, and whether severity of feeding difficulties and malnutrition are independently associated with caregiver quality of life (QoL).Method: This cross-sectional survey included 76 children with CP (18mo-12y) from four regions of Ghana. Severity of CP was classified using the Gross Motor Function Classification System and anthropometric measures were taken. Caregivers rated their QoL (using the Pediatric Quality of Life Inventory Family Impact Module) and difficulties with eight aspects of child feeding. Logistic regression analysis explored factors (socio-economic characteristics, severity of CP, and feeding difficulties) associated with being underweight. Linear regression was undertaken to assess the relationship between caregiver QoL and child malnutrition and feeding difficulties.Results: Poor nutritional status was common: 65% of children aged under 5 years were categorized as underweight, 54% as stunted, and 58% as wasted. Reported difficulties with child's feeding were common and were associated with the child being underweight (odds ratio 10.7, 95% confidence interval 2.3-49.6) and poorer caregiver QoL (p<0.001). No association between caregiver QoL and nutritional status was evident.Interpretation: Among rural, low resource populations in Ghana, there is a need for appropriate, accessible caregiver training and support around feeding practices of children with CP, to improve child nutritional status and caregiver well-being.What This Paper Adds: Malnutrition is very common among children with cerebral palsy in this rural population in Ghana. Feeding difficulties in this population were strongly associated with being underweight. Feeding difficulties were associated with poorer caregiver quality of life (QoL). Child nutritional status was not associated with caregiver QoL. [ABSTRACT FROM AUTHOR]

Aim: To investigate whether docosahexaenoic acid (DHA), choline, and uridine-5-monophosphate (UMP) supplementation improves neurodevelopmental outcome in infants with suspected cerebral palsy (CP) versus a comparison group of children.Method: Infants aged 1 to 18 months with suspected CP were recruited from UK child development centres. Participants received daily treatment or control supplementation for 2 years (double-blind randomized control design). Stratification was by age, sex, predominant pattern of motor involvement (four limbs or other), and visual impairment (or not). The primary outcome was the cognitive composite score of the Bayley Scales of Infant and Toddler Development, Third Edition (CCS-Bayley-III). Secondary outcomes included language composite and motor composite scores of the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III).Results: Forty infants were recruited; 35 began supplementation, 29 completed 1 to 2 years' supplementation. The treatment group CCS-Bayley-III was non-significantly higher than the comparison group (mean 77.7 [SD 19.2] and 72.2 [SD 19.8] respectively, mean modelled difference 4.4 [-2.8, 11.6]). The treatment group language scores, but not motor scores, were non-significantly higher than for the comparison group.Interpretation: Most families found supplementation feasible. No statistically significant differences in neurodevelopmental outcome between the treatment and comparison groups were identified. Further investigation of neurodevelopmental outcome after supplementation with DHA, choline, and UMP of infants with suspected CP is warranted.What This Paper Adds: This was the first trial of phosphatidylcholine precursor supplementation in infants with suspected cerebral palsy (CP). Families of infants with suspected CP found 2-year nutritional supplementation feasible. There was no statistically significant neurodevelopmental advantage for the treatment group versus the comparison group. However, treatment group cognitive and language advantage were of clinically meaningful magnitude. [ABSTRACT FROM AUTHOR]

Aim: To evaluate the relationship between the movement abnormalities of the impaired upper limb in children with unilateral cerebral palsy (CP) and bimanual performance.Method: Twenty-three children with unilateral CP (mean age 11y 10mo [SD 2y 8mo]) underwent evaluation of bimanual performance (Assisting Hand Assessment [AHA]) and a three-dimensional movement analysis to measure deviations in the movement of their affected upper limb, and compared with 23 typically developing children (TDC) (mean age 11y 11mo [SD 2y 5mo]). Kinematic indices, such as the Global Arm Profile Score (APS), which summarizes the global movement deviation of the upper limb from the norm, and the Global Arm Variable Score (AVS), which represent movement deviations for a given joint, were calculated and correlated to AHA.Results: Values of kinematic indices were significantly higher in children with unilateral CP than in TDC. A strong correlation between Global-APS and AHA score (r=-0.75) was found. Other significant correlations were found with Global-AVS, especially in distal joints.Interpretation: Children with unilateral CP had more movement deviations than TDC. The global movement deviation of the impaired upper limb was strongly correlated with bimanual performance. The influence of distal abnormalities confirms the importance of considering these limitations in therapeutics.What This Paper Adds: Children with unilateral cerebral palsy had more movement deviations than typically developing children in unimanual tasks. A strong relationship was found between movement deviations of the impaired upper limb and bimanual performance. [ABSTRACT FROM AUTHOR]

Aim: If increased muscle stiffness and contractures in children with cerebral palsy (CP) are related to impaired muscle growth, reduced muscle growth should precede or coincide with increased muscle stiffness during development. Here, we compared the volume of the medial gastrocnemius muscle and the passive (non-neural) stiffness of the triceps surae musculotendinous unit in typically developing children and children with CP from birth until 4 years of age.Method: Forty-one children with CP and 45 typically developing children were included. Freehand three-dimensional ultrasound was used to evaluate the volume of the medial gastrocnemius muscle. Biomechanical and electrophysiological measures were used to determine passive and reflex mediated stiffness of the triceps surae musculotendinous unit.Results: Medial gastrocnemius muscle volume increased with the same rate in typically developing and children with CP until 12 months of age, when a significant smaller rate of growth was observed in children with CP. Passive stiffness of the triceps surae musculotendinous unit showed a linear increase with age in typically developing children. Children with CP older than 27 months showed a significant increase in passive stiffness. Reflex mediated stiffness was only pathologically increased in four children with CP.Interpretation: The deviation of medial gastrocnemius muscle volume, earlier than musculotendinous unit stiffness, is consistent with the hypothesis. The data also point out that muscle atrophy and muscle stiffness already develops within the first 1 to 2 years. This emphasizes the necessity of early interventions to promote lower limb muscle growth in this population.What This Paper Adds: Medial gastrocnemius muscle growth is reduced in children with cerebral palsy (CP) around 12 months after birth. Triceps surae musculotendinous unit stiffness is increased in children with CP around 27 months after birth. Reflex excitability is rarely increased in children with CP. Reduced muscle growth may be involved in the pathophysiology of contractures. [ABSTRACT FROM AUTHOR]

Aim: As there is little evidence for concurrent validity of the Eating and Drinking Ability Classification System (EDACS), this study aimed to determine its concurrent validity and reliability in children and adolescents with cerebral palsy (CP).Method: After an extensive translation procedure, we applied the German language version to 52 participants with CP (30 males, 22 females, mean age 9y 7mo [SD 4y 2mo]). We correlated (Kendall's tau or Kτ ) the EDACS levels with the Bogenhausener Dysphagiescore (BODS), and the EDACS level of assistance with the Manual Ability Classification System (MACS) and the item 'eating' of the Functional Independence Measure for Children (WeeFIM). We further quantified the interrater reliability between speech and language therapists (SaLTs) and between SaLTs and parents with Kappa (κ).Results: The EDACS levels correlated highly with the BODS (Kτ =0.79), and the EDACS level of assistance correlated highly with the MACS (Kτ =0.73) and WeeFIM eating item (Kτ =-0.80). Interrater reliability proved almost perfect between SaLTs (EDACS: κ=0.94; EDACS level of assistance: κ=0.89) and SaLTs and parents (EDACS: κ=0.82; EDACS level of assistance: κ=0.89).Interpretation: The EDACS levels and level of assistance seem valid and showed almost perfect interrater reliability when classifying eating and drinking problems in children and adolescents with CP.What This Paper Adds: The Eating and Drinking Ability Classification System (EDACS) correlates well with a dysphagia score. The EDACS level of assistance proves valid. The German version of EDACS is highly reliable. EDACS correlates moderately to highly with other classification systems. [ABSTRACT FROM AUTHOR]

Aim: To describe the development of bimanual performance among young children with unilateral or bilateral cerebral palsy (CP).Method: A population-based sample of 102 children (53 males, 49 females), median age 28.5 months (interquartile range [IQR] 16mo) at first assessment and 47 months (IQR 18mo) at last assessment, was assessed half-yearly with the Assisting Hand Assessment (AHA) or the Both Hands Assessment (BoHA) for a total of 329 assessments. Developmental limits and rates were estimated by nonlinear mixed-effects models. Developmental trajectories were compared between levels of manual ability (Mini-Manual Ability Classification System [Mini-MACS] and MACS) and AHA or BoHA performance at 18 months of age (AHA-18/BoHA-18) for both CP subgroups, and additionally between children with bilateral CP with symmetric or asymmetric hand use.Results: For both CP subgroups, children classified in Mini-MACS/MACS level I, and those with high AHA-18 or BoHA-18 reached the highest limits of performance. For children with bilateral CP the developmental change was small, and children with symmetric hand use reached the highest limits.Interpretation: Mini-MACS/MACS levels and AHA-18 or BoHA-18 distinguished between various developmental trajectories both for children with unilateral and bilateral CP. Children with bilateral CP changed their performance to a smaller extent than children with unilateral CP.What This Paper Adds: Manual Ability Classification System levels and Assisting Hand Assessment/Both Hands Assessment performance at 18 months are important predictors of hand use development in cerebral palsy (CP). Children with bilateral CP improved less than those with unilateral CP. Children with bilateral CP and symmetric hand use reached higher limits than those with asymmetry. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]