Clinical course of pain intensity in individuals with cerebral palsy: A prognostic systematic review.

AIM: To describe the clinical course of pain intensity in individuals with cerebral palsy (CP) resulting from usual care or specific interventions. METHOD: We conducted an exploratory prognostic systematic review searching electronic databases from inception to 31st December 2021. Evidence from low and moderate risk-of-bias studies was synthesized. RESULTS: We retrieved 2275 citations; 18 studies met the inclusion criteria and 10 were synthesized. The course of pain intensity in children with CP receiving usual care was stable over 15 weeks (χ2 [2]  = 1.8, p = 0.5). Children who received continuous intrathecal baclofen (CITB) reported significant pain intensity reduction (visual analogue scale [VAS] = -4.2 out of 10, 95% confidence interval [CI] = -6.3 to -2.1]) 6 months postinsertion but similar children receiving usual care had no significant change over 6 months (VAS = 1.3 out of 10, 95% CI = -1.3 to 3.6). Children receiving botulinum neurotoxin A (BoNT-A) injections had significant decreases in pain after 1 month (numeric rating scale = -6.5, 95% CI = -8.0 to -5.0). Adults with chronic pain receiving usual care reported stable pain intensity over time; pain intensity improved in ambulatory adults exercising and those treated surgically for cervical myelopathy. INTERPRETATION: The course of pain intensity in individuals with CP is unclear. Evidence suggests that children and adults receiving usual care had stable pain intensity over the short or long term. Interventions (CITB and BoNT-A in children and exercise and surgical treatment for cervical myelopathy in adults) had pain intensity reduction. Larger study samples are needed to confirm these results. WHAT THIS PAPER ADDS: Pain intensity was stable in children with cerebral palsy (CP) receiving usual care. Adults with CP and chronic pain receiving usual care had stable, persistent pain intensity. Children receiving continuous intrathecal baclofen via pump and botulinum neurotoxin A reported significantly lower pain intensities. Adults with chronic pain and dyskinetic CP and cervical myelopathy reported significantly lower pain intensity with exercise or cervical decompression. Limited high-quality evidence exists describing non-procedural pain changes in individuals with CP.

Gross motor change after inpatient rehabilitation for children with acquired brain injury: A 10-year retrospective review.

AIM: To estimate gross motor change in inpatient school-aged children with subacute acquired brain injury (ABI), identify factors associated with gross motor change, and describe inpatient physiotherapy focus. METHOD: This retrospective chart review involved inpatient children (5-18 years) with subacute ABI who had either two Gross Motor Function Measure (GMFM-88) assessments or one GMFM-88 with another pre/post gross motor outcome measure. Outcome change scores and Goal Attainment Scaling (GAS) T scores were calculated. Regression analyses examined factors predicting gross motor change. GAS goal areas were analysed to determine physiotherapy focus. RESULTS: Of the 546 charts screened, 266 (118 female) met study criteria. The GMFM-88 was generally administered first, followed by other measures. GMFM-88 (n = 202), Community Balance and Mobility Scale (n = 89), and Six-Minute Walk Test (6MWT) (n = 98) mean change scores were 18.03% (SD 19.34), 17.85% (SD 10.77), and 142.3 m (SD 101.8) respectively. The mean GAS T score was 55.06 (SD 11.50). Lower baseline scores and increased time between assessments were most predictive of greater GMFM-88 change (r ≥ 0.40). Twenty-five percent of GAS goals were ambulation-based. INTERPRETATION: Appropriate outcome measure selection is integral to detecting gross motor change in pediatric inpatient ABI rehabilitation. Mean change score estimates can be used to compare standard inpatient rehabilitation with new treatment approaches.

Integrating Transcranial Direct Current Stimulation into an Existing Inpatient Physiotherapy Program to Enhance Motor Learning in an Adolescent with Traumatic Brain Injury: A Case Report.

PURPOSE: Describe how transcranial direct current stimulation (tDCS) was incorporated into an inpatient physiotherapy program for an adolescent with severe traumatic brain injury (TBI), detail the motor learning focus of the physiotherapy sessions, and summarize gross motor progress. METHOD: This case report describes an adolescent who received 20 minutes of anodal tDCS immediately prior to 16 physiotherapy sessions over four weeks. Potential side effects were tracked pre/post tDCS. Gross motor outcomes were measured pre-intervention, post-intervention, and three months post-intervention. Physiotherapy session content was analyzed using therapist documentation and the Motor Learning Strategies Rating Instrument. RESULTS: The youth tolerated tDCS well. The primary side effect was itchiness under the electrodes during tDCS sessions. His mobility progressed from wheelchair use pre- 'tDCS + physiotherapy' to ambulation with a walker post-intervention. His Gross Motor Function Measure score increased 33.1% points pre/post intervention. Session tasks often had several foci (e.g., skill acquisition, strength, and balance) with task focus changing as the youth progressed. Various motor learning strategies were layered within tasks to support performance and learning. CONCLUSIONS: tDCS was successfully integrated into an existing inpatient physiotherapy program for an adolescent with TBI. This protocol provides a structure for implementing, monitoring, and measuring tDCS + physiotherapy in pediatric rehabilitation.

Pain trajectories and well-being in children and young people with cerebral palsy: A cohort study.

AIM: To identify 5-week pain intensity trajectories and their association with physical and psychological well-being in children/young people with cerebral palsy (CP). METHOD: A cohort study was conducted with 101 Canadian children/young people with CP, of whom 49 were female, with an overall mean age of 12 years 11 months (SD 3 years 1 month), range of 8 to 18 years, and classified in any Gross Motor Function Classification System level. Self-reported pain intensity (Faces Pain Scale - Revised) was collected weekly for 5 weeks and physical and psychological well-being (KIDSCREEN-27) at baseline and 5 weeks. Statistical analyses included latent class growth and general linear models. RESULTS: All Gross Motor Function Classification System levels were represented (I = 40.6%; II = 15.8%; III = 20.8%; IV = 13.9%; V = 8.9%). Five pain intensity trajectories were identified. Three trajectories had very low (35.4%), low (32.4%), or high (4.9%) mean stable pain. Two trajectories had moderate changing pain (16.8%) and high pain decreasing to moderate levels (10.5%) respectively. Trajectory participants with stable high pain had the lowest physical well-being (adjusted ß = -10.01; 95% confidence interval [CI] = -19.37 to -0.66). Those in the three trajectories with the highest mean baseline pain intensity (>3 out of 10) had the lowest psychological well-being (adjusted ß = -8.27, 95% CI = -14.84 to -1.70; ß = -6.74, 95% CI = -12.43 to -1.05; ß = -5.82, 95% CI = -15.34 to 3.71). INTERPRETATION: Almost one-third of participants had moderate-to-high pain intensity trajectories. Membership in the higher pain intensity trajectories was associated with lower physical and psychological well-being. WHAT THIS PAPER ADDS: Five distinct 5-week pain intensity trajectories were identified in children/young people with cerebral palsy. Thirty-two per cent of participants had moderate-to-high pain intensity trajectories. Participants in the trajectories with higher pain intensity reported lower physical and psychological well-being.

OBJETIVO: Identificar trajetórias de intensidade de dor de 5 semanas e sua associação com o bem-estar físico e psicológico em crianças/jovens com paralisia cerebral (PC). MÉTODO: Foi realizado um estudo de coorte com 101 crianças/jovens canadenses com PC, sendo 49 do sexo feminino, com média de idade geral de 12 anos e 11 meses (DP 3 anos 1 mês), faixa de 8 a 18 anos, e classificados em qualquer nível do Sistema de Classificação da Função Motora Grossa. A intensidade da dor autorreferida (Faces Pain Scale - Revised) foi coletada semanalmente por 5 semanas e o bem-estar físico e psicológico (KIDSCREEN-27) no início e 5 semanas. As análises estatísticas incluíram crescimento de classe latente e modelos lineares gerais. RESULTADOS: Todos os níveis do Sistema de Classificação da Função Motora Grossa (GMFCS) foram representados (I = 40,6%; II = 15,8%; III = 20,8%; IV = 13,9%; V = 8,9%). Cinco trajetórias de intensidade de dor foram identificadas. Três trajetórias tiveram muito baixa (35,4%), baixa (32,4%) ou alta (4,9%) média de dor estável. Duas trajetórias apresentaram dor moderada em mudança (16,8%) e dor alta diminuindo para níveis moderados (10,5%), respectivamente. Os participantes com trajetória com dor alta estável tiveram o menor bem-estar físico (ß ajustado = -10,01; intervalo de confiança de 95% [IC] = -19,37 a -0,66). Aqueles nas três trajetórias com a maior intensidade média de dor na linha de base (> 3 em 10) tiveram o menor bem-estar psicológico (ß ajustado = -8,27, IC 95% = -14,84 a -1,70; ß = -6,74, 95% IC = -12,43 a -1,05; ß = -5,82, IC 95% = -15,34 a 3,71). INTERPRETAÇÃO: Quase um terço dos participantes tiveram trajetórias de intensidade de dor moderada a alta. A participação nas trajetórias de maior intensidade de dor foi associada a menor bem-estar físico e psicológico.

Identifying pain trajectories in children and youth with cerebral palsy: a pilot study.

BACKGROUND: Although chronic pain is common in children with cerebral palsy (CP), little is known about short-term pain fluctuations and their impact on children's well-being. High-quality cohort studies are needed to understand the clinical course of pain in this population. We aimed to determine the feasibility of conducting a multicentre cohort study. In this pilot study we assessed: 1) study processes, 2) resource and 3) management indicators including recruitment and follow-up rates, data completeness, participant characteristics, and successes and barriers in the study conduct. METHODS: A multi-centre pilot cohort study was conducted with 10 Canadian children/youth with CP attending one of two children's rehabilitation centers. We collected self-reported pain intensity (Faces Pain Scale-Revised [FPS-R], Numeric Rating Scale [NRS]); pain interference (PROMIS PI); pain location (pain diagram); physical and psychological well-being (KIDSCREEN-27), sleep characteristics, preceding months' interventions, and some clinical characteristics at baseline. Average pain intensity was reported weekly for five weeks. Well-being, sleep and interventions were measured at baseline and again at five weeks. We used feasibility indicators to evaluate:1) study processes (e.g. recruitment, attrition rates); 2) resources (e.g. data completion, budgetary challenges); and 3) management (e.g. data optimization, variability of participants and pain scores). RESULTS: Between March and May 2019, 24 children and their parents/guardians were contacted and 20 met eligibility criteria. Of those, 10 agreed to in-person screening (50%) and were subsequently enrolled. The follow-up rate was 90% and self-reported missing data was minimal. Ninety percent of participants chose e-questionnaire follow-ups versus mailed paper questionnaires. Sixty percent required reminders to complete e-follow-ups. Participants were aged 8-17 years, five were female, GMFCS levels I-IV (none with level V), 90% had spastic CP and 80% reported having pain in the preceding week. Pain intensity (FPS-R) between participants ranged from 0-8/10 at baseline and 0-6/10 across all four weekly follow-ups. CONCLUSIONS: This pilot study demonstrates the feasibility of conducting a multicentre cohort study to identify short-term pain trajectories and measure their association with well-being in children and youth with CP. Additional strategies to improve recruitment and accessibility for those with GMFCS levels V should be implemented in future studies.

Deep brain stimulation for pediatric dystonia: a meta-analysis with individual participant data.

AIM: We performed a meta-analysis with individual participant data of deep brain stimulation (DBS) for dystonia in children and young people. METHOD: Three databases (PubMed, Embase, and Web of Science) were queried from January 1999 to August 2017 with no language restrictions to identify case studies and cohort studies reporting on pediatric patients (age ≤21y) with dystonia. The primary outcomes were changes in Burke-Fahn-Marsden (BFM) or Barry-Albright Dystonia Scale scores. A mixed-effects regression was used to identify associations between clinical covariates and outcomes. RESULTS: Of 2509 citations reviewed, 72 articles (321 children) were eligible. At last follow-up (median 12mo, 25th centile=9.0; 75th centile=32.2), 277 (86.3%) patients showed improvement in dystonia, while 66.1 percent showed clinically significant (>20%) BFM Dystonia Rating Scale-motor improvement. On multivariable hierarchical regression, older age at dystonia onset, inherited dystonia without nervous system pathology and idiopathic dystonia (vs inherited with nervous system pathology or acquired dystonia), and truncal involvement indicated a better outcome (p<0.05). INTERPRETATION: The data suggest that DBS is effective and should be considered in selected children with inherited or idiopathic dystonia. WHAT THIS PAPER ADDS: Deep brain stimulation is effective in selected children with inherited or idiopathic dystonia.

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.

A good night's sleep: pain trajectories and sleep disturbance in children with cerebral palsy.

STUDY OBJECTIVES: Sleep quality is important during childhood and adolescence. Given the high prevalence of pain in children/youth with cerebral palsy, we aimed to measure the association between short-term pain trajectories and sleep disturbance in these individuals. METHODS: We accrued the cohort between November 2019 and October 2020 and recruited children/youth who (1) were 8-18 years old; (2) had cerebral palsy with any Gross Motor Function Classification System level; and (3) could self-report pain and sleep disturbance. We collected self-reported baseline and weekly follow-up data using electronic questionnaires completed every week for 5 weeks. Sleep disturbance at 5 weeks was the primary outcome (pediatric Patient-Reported Outcomes Measurement Information System short form, v1.0-4a). We used general linear regression to assess the association between pain intensity trajectory group and sleep disturbance controlling for confounders. RESULTS: A total of 190 individuals were eligible; 102 were enrolled and 89 were included in our final analysis. Pain trajectory groups had estimated crude mean sleep disturbance scores at 5 weeks ranging from 56.0 (95% confidence interval, 51.8, 60.8) to 61.8 (55.7, 67.9). Compared to those with stable, no/very mild pain, those in the stable, high-pain group had the greatest sleep disturbance (adjusted ß = 5.7; 95% confidence interval, 1.2, 10.2). CONCLUSIONS: Irrespective of pain trajectory, children and youth with cerebral palsy reported sleep disturbances. Those with a stable, high pain intensity in the previous 5 weeks reported the greatest sleep disturbance. The results highlight the importance of considering pain trajectories and their impact on sleep in children with cerebral palsy. CITATION: Shearer HM, Côté P, Hogg-Johnson S, Fehlings DL. A good night's sleep: pain trajectories and sleep disturbance in children with cerebral palsy. J Clin Sleep Med. 2024;20(5):719-726.

Association of Gestational Age at Birth and Changes on MRI With Prevalence and Spectrum of Comorbidities in Children With Cerebral Palsy.

BACKGROUND AND OBJECTIVES: For individuals with cerebral palsy (CP) and caregivers, comorbidities may be a greater challenge than neuromotor impairment. Clinicians may make assumptions regarding risk of comorbidities based simply on term vs preterm birth, but this has not been well examined. To better understand factors affecting comorbidity pattern, we investigated the relationship between gestational age (GA) and imaging pattern on the presence of specific comorbidities. METHODS: This is a cross-sectional study of data extracted from the Canadian Cerebral Palsy Registry of children with CP. Multivariable analysis was used to evaluate the relationship between brain injury, GA, and comorbidities. Comorbidities included in the analysis were communication, cognitive, visual, and auditory impairment, seizures in the past year, and gavage feeding. Each comorbidity was assessed as a separate nonexclusive outcome, with GA, MRI pattern, birth weight, postneonatal insult, 5-minute Apgar score, and male sex considered as potential modifiers. RESULTS: The only comorbidity affected by GA on multivariable analysis was seizures within the past year that were more prevalent in term children (odds ratio [OR] 1.1 95% CI 1.0-1.2) and was also affected by Apgar score (OR 0.9 95% CI 0.85-0.94), but not MRI pattern. MRI pattern appeared important for communication impairment (deep gray OR 4.2 95% CI 1.8-10.0; total brain injury OR 8.5, 95% CI 3.2-22.6; malformation OR 2.7, 95% CI 1.3-5.7) and cognitive impairment (deep gray OR 5.6, 95% CI 2.4-13.2; total brain injury OR 10.1, 95% CI 4.0-25.3; malformation OR 3.3, 95% CI 1.6-6.8; watershed OR 3.6, 95% CI 1.4-8.9). Focal injury compared with normal MRI was associated with reduced odds of visual impairment (OR 0.24, 95% CI 0.12-0.48), auditory impairment (OR 0.2195% CI 0.10-0.46) and communication impairment (OR 0.46, 95% CI 0.26-0.82), and overall number of comorbidities (coefficient -0.73, 95% CI -1.2 to -0.31). The number of comorbidities was increased by total brain injury pattern (coefficient 0.65, 95% CI 0.15-1.13) and reduced by focal brain injury (coefficient -0.73, 95% CI -1.2 to -0.31) and increasing 5-minute Apgar score (coefficient -0.11, 95% CI -0.16 to -0.07). DISCUSSION: In those with brain injuries sufficient to cause CP, development of additional comorbidities is less affected by GA at birth and more related to the underlying cause of CP as reflected by MRI patterns.

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.

Uncertainties regarding cerebral palsy diagnosis: opportunities to operationalize the consensus definition.

Background and Objectives: Cerebral palsy (CP), the most common motor disability of childhood, is variably diagnosed. We hypothesized that child neurologists and neurodevelopmentalists, often on the frontlines of CP diagnosis in North America, harbor uncertainties regarding the practical application of the most recent CP consensus definition from 2006. Methods: We conducted a cross-sectional survey of child neurologists and neurodevelopmentalists at the 2022 Child Neurology Society Annual Meeting. Attendees were provided the 2006 CP consensus definition and asked whether they had any uncertainties about the practical application of the definition across four hypothetical clinical vignettes. Results: Of 230 attendees, 164 responded to the closing survey questions (71%). 145/164 (88%) expressed at least one uncertainty regarding the clinical application of the 2006 definition. Overwhelmingly, these areas of uncertainty focused on: 1) Age, both with regards to the minimum age of diagnosis and the maximum age of brain disturbance or motor symptom onset, (67/164, 41%), and 2) Interpretation of the term "non-progressive" (48/164, 29%). The vast majority of respondents (157/164, 96%) answered 'Yes' to the question: Do you think we should revise the 2006 consensus definition of CP? Discussion: We propose that the uncertainties we identified could be addressed by operationalizing the 2006 consensus definition to support a more uniform CP diagnosis. To address the most common CP diagnostic uncertainties we identified, we propose 3 points of clarification based on the available literature: 1) Motor symptoms/signs should be present by 2 years old; 2) CP can and should be diagnosed as early as possible, even if activity limitation is not yet present, if motor symptoms/signs can be reasonably predicted to yield activity limitation (e.g. by using standardized examination instruments, Brain MRI, and a suggestive clinical history); and 3) The clinical motor disability phenotype should be non-progressive through 5 years old. We anticipate that operationalizing the 2006 definition of CP in this manner could clarify the uncertainties we identified among child neurologists and neurodevelopmentalists and reduce the diagnostic variability that currently exists.

Motor Evoked Potential Amplitude in Motor Behavior-based Transcranial Direct Current Stimulation Studies: A Systematic Review.

Motor evoked potential amplitude (MEPamp) is frequently measured in transcranial direct current stimulation (tDCS) studies that target the primary motor cortex (M1), and a subset of these studies involve motor behavior. This systematic review explored the role of MEPamp as an indicator of neural change in M1-targeted tDCS studies involving motor behavior (i.e., motor practice and/or evaluation of motor performance) in healthy individuals, and examined the association between changes in motor performance and MEPamp. We executed our search strategy across four bibliographic databases. Twenty-two manuscripts met eligibility criteria. While anodal tDCS combined with motor practice frequently increased MEPamp, MEPamp outcomes did not necessarily align with changes in motor performance. Thus, MEPamp may not be the most appropriate indicator of neural change in tDCS studies that aim to improve motor performance.

Top 10 Research Themes for Dystonia in Cerebral Palsy: A Community-Driven Research Agenda.

Dystonia in cerebral palsy (DCP) is a common, debilitating, but understudied condition. The CP community (people with CP and caregivers) is uniquely equipped to help determine the research questions that best address their needs. We developed a community-driven DCP research agenda using the well-established James Lind Alliance methodology. CP community members, researchers, and clinicians were recruited through multiple advocacy, research, and professional organizations. To ensure shared baseline knowledge, participants watched webinars outlining our current knowledge on DCP prepared by a Steering Group of field experts (cprn.org/research-cp-dystonia-edition). Participants next submitted their remaining uncertainties about DCP. These were vetted by the Steering Group and consolidated to eliminate redundancy to generate a list of unique uncertainties, which were then prioritized by the participants. The top-prioritized uncertainties were aggregated into themes through iterative consensus-building discussions within the Steering Group. 166 webinar viewers generated 67 unique uncertainties. 29 uncertainties (17 generated by community members) were prioritized higher than their randomly matched pairs. These were coalesced into the following top 10 DCP research themes: (1) develop new treatments; (2) assess rehabilitation, psychological, and environmental management approaches; (3) compare effectiveness of current treatments; (4) improve diagnosis and severity assessments; (5) assess the effect of mixed tone (spasticity and dystonia) in outcomes and approaches; (6) assess predictors of treatment responsiveness; (7) identify pathophysiologic mechanisms; (8) characterize the natural history; (9) determine the best treatments for pain; and (10) increase family awareness. This community-driven research agenda reflects the concerns most important to the community, both in perception and in practice. We therefore encourage future DCP research to center around these themes. Furthermore, noting that community members (not clinicians or researchers) generated the majority of top-prioritized uncertainties, our results highlight the important contributions community members can make to research agendas, even beyond DCP.

Definition and classification of hyperkinetic movements in childhood.

Hyperkinetic movements are unwanted or excess movements that are frequently seen in children with neurologic disorders. They are an important clinical finding with significant implications for diagnosis and treatment. However, the lack of agreement on standard terminology and definitions interferes with clinical treatment and research. We describe definitions of dystonia, chorea, athetosis, myoclonus, tremor, tics, and stereotypies that arose from a consensus meeting in June 2008 of specialists from different clinical and basic science fields. Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. Chorea is an ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments. Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture. Myoclonus is a sequence of repeated, often nonrhythmic, brief shock-like jerks due to sudden involuntary contraction or relaxation of one or more muscles. Tremor is a rhythmic back-and-forth or oscillating involuntary movement about a joint axis. Tics are repeated, individually recognizable, intermittent movements or movement fragments that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movement. Stereotypies are repetitive, simple movements that can be voluntarily suppressed. We provide recommended techniques for clinical examination and suggestions for differentiating between the different types of hyperkinetic movements, noting that there may be overlap between conditions. These definitions and the diagnostic recommendations are intended to be reliable and useful for clinical practice, communication between clinicians and researchers, and for the design of quantitative tests that will guide and assess the outcome of future clinical trials.

Youth with cerebral palsy with differing upper limb abilities: how do they access computers?

OBJECTIVE: To identify the current level of awareness of different computer access technologies and the choices made regarding mode of access by youth with cerebral palsy (CP) and their families. DESIGN: Survey. SETTING: Two tertiary-level rehabilitation centers in New Zealand and Canada. PARTICIPANTS: Youth (N=60) with CP, Manual Ability Classification Scale (MACS) levels I to V, age 13 to 25 years. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: Questionnaire. RESULTS: Fifty (83%) of the 60 youth were aware of at least 1 available assistive technology (AT), such as touch screens and joysticks. However, only 34 youth (57%) were familiar with the accessibility options currently available in the most common operating systems. Thirty-three (94%) of 35 youth who were MACS I and II used a standard mouse and keyboard, while few chose to use assistive technology or accessibility options. In contrast, 10 (40%) of 25 youth who were MACS III to V used a variety of assistive technologies such as touch screens, joysticks, trackballs, and scanning technologies. This group also had the highest use of accessibility options, although only 15 (60%) of the 25 were aware of them. CONCLUSION: Most youth with CP were aware of, and used, assistive technologies to enhance their computer access but were less knowledgeable about accessibility options. Accessibility options allow users to modify their own computer interface and can thus enhance computer access for youth with CP. Clinicians should be knowledgeable enough to give informed advice in this area of computer access, thus ensuring that all youth with CP can benefit from both AT and accessibility options, as required.

Cortical reorganization after modified constraint-induced movement therapy in pediatric hemiplegic cerebral palsy.

Constraint-induced movement therapy improves motor function in the affected hand of children with hemiplegic cerebral palsy and results in cortical changes in adults with stroke. This study measured clinical improvement and cortical reorganization in a child with hemiplegia who underwent modified constraint-induced movement therapy for 3 weeks. Clinical, functional magnetic resonance imaging and magnetoencephalography measurements were done at baseline, after therapy, and 6 months after therapy. Modified constraint-induced movement therapy resulted in clinical improvement as measured by the Pediatric Motor Activity Log. Functional magnetic resonance imaging showed bilateral sensorimotor activation before and after therapy and a shift in the laterality index from ipsilateral to contralateral hemisphere after therapy. Magnetoencephalography showed increased cortical activation in the ipsilateral motor field and contralateral movement evoked field after therapy. Cortical reorganization was maintained at the 6-month follow-up. This is the first study to demonstrate cortical reorganization after any version of constraint-induced movement therapy in a child with hemiplegia.

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.

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.

Efficacy and safety of deflazacort vs prednisone and placebo for Duchenne muscular dystrophy.

OBJECTIVE: To assess safety and efficacy of deflazacort (DFZ) and prednisone (PRED) vs placebo in Duchenne muscular dystrophy (DMD). METHODS: This phase III, double-blind, randomized, placebo-controlled, multicenter study evaluated muscle strength among 196 boys aged 5-15 years with DMD during a 52-week period. In phase 1, participants were randomly assigned to receive treatment with DFZ 0.9 mg/kg/d, DFZ 1.2 mg/kg/d, PRED 0.75 mg/kg/d, or placebo for 12 weeks. In phase 2, placebo participants were randomly assigned to 1 of the 3 active treatment groups. Participants originally assigned to an active treatment continued that treatment for an additional 40 weeks. The primary efficacy endpoint was average change in muscle strength from baseline to week 12 compared with placebo. The study was completed in 1995. RESULTS: All treatment groups (DFZ 0.9 mg/kg/d, DFZ 1.2 mg/kg/d, and PRED 0.75 mg/kg/d) demonstrated significant improvement in muscle strength compared with placebo at 12 weeks. Participants taking PRED had significantly more weight gain than placebo or both doses of DFZ at 12 weeks; at 52 weeks, participants taking PRED had significantly more weight gain than both DFZ doses. The most frequent adverse events in all 3 active treatment arms were Cushingoid appearance, erythema, hirsutism, increased weight, headache, and nasopharyngitis. CONCLUSIONS: After 12 weeks of treatment, PRED and both doses of DFZ improved muscle strength compared with placebo. Deflazacort was associated with less weight gain than PRED. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that for boys with DMD, daily use of either DFZ and PRED is effective in preserving muscle strength over a 12-week period.