WHAT'S KNOWN ON THIS SUBJECT: Pain in children with cerebral palsy is underrecognized and undertreated and negatively affects quality of life. Communication challenges and multiple pain etiologies complicate management. There is a wide range of pain prevalence reported in the literature (14% to 73%). WHAT THIS STUDY ADDS:The impact of pain on activities in children with cerebral palsy across a wide age range and motor abilities is investigated. Physician-identified causes of pain are systematically assessed and reported. Concordance of physician and caregiver identification of pain is evaluated. abstract OBJECTIVES: Pain in children with cerebral palsy (CP) is underrecognized, undertreated, and negatively affects quality of life. Communication challenges and multiple pain etiologies complicate diagnosis and treatment. The primary objectives of this study were to determine the impact of pain on activities and to identify the common physicianidentified causes of pain in children and youth ages 3 to 19 years across all levels of severity of CP. METHODS:The study design was cross-sectional, whereby children/ youth aged 3 to 19 years and their families were consecutively recruited. The primary caregivers were asked to complete a onetime questionnaire, including the Health Utilities Index 3 pain subset, about the presence and characteristics of pain. The treating physician was asked to identify the presence of pain and provide a clinical diagnosis for the pain, if applicable. RESULTS:The response rate was 92%. Of 252 participants, 54.8% reported some pain on the Health Utilities Index 3, with 24.4% of the caregivers reporting that their child experienced pain that affected some level of activities in the preceding 2 weeks. Physicians reported pain in 38.7% and identified hip dislocation/subluxation, dystonia, and constipation as the most frequent causes of pain. CONCLUSIONS:One-quarter of our sample experienced pain that limited activities and participation. Clinicians should be aware that hip subluxation/dislocation and dystonia were the most common causes of pain in children/youth with CP in this study. Potential causes of pain should be identified and addressed early to mitigate the negative impact of pain on quality of life. Pediatrics 2013;132: e407-e413 AUTHORS:

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.

RESULTS Three hundred and forty-four participants were approached and 87% (n=300) participated. Sufficient data were available on 248 (72% of total sample). Sixty-six participants (27%) formed the pain group with HUI3 pain scores of at least 3. The presence of pain and increasing age significantly negatively predicted HRQOL (p<0.001, R 2 =0.141), while GMFCS and sex did not. Musculoskeletal deformity (24%) and hypertonia (18%) were the most frequent pain causes. HRQOL statistically differed depending on the pain etiology (p=0.028) with musculoskeletal deformity showing the lowest mean HRQOL. INTERPRETATIONThe presence of pain and increasing age negatively predict HRQOL in CP. musculoskeletal deformity has the greatest negative impact on HRQOL.Quality of life refers to an 'individuals´ perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns'.1 Health-related quality of life (HRQOL) takes a holistic view of well-being, encompassing physical, social, and psychological wellbeing. It is a multidimensional construct based on an individual's subjective evaluation of his/her function and therefore differs from quality of life where individuals can evaluate their life regardless of impairments and function. While individuals with severe disabilities can maintain high HRQOL, 3 several studies have shown that children and adolescents with cerebral palsy (CP) have lower HRQOL scores compared to typically developing peers. 4,5 Studies have sought to determine the factors that influence HRQOL for children with CP. The presence of pain has consistently been identified as an important factor negatively affecting HRQOL. 6,7 The Study of Participation of Children with Cerebral Palsy Living in Europe (SPAR-CLE) identified that pain was associated with lower scores across all HRQOL domains in children with CP aged 8 to 12 years. 7 The impact of age and sex on HRQOL in children and adolescents with CP is more variable. Age did not impact HRQOL for individuals with CP across the ages of 6 to 30 years 8 and 11 to 17 years 4 in two independent studies. In contrast, older adolescents (8-16y) with chronic conditions including CP reported lower emotional well-being scores compared with younger children. 9 In the same study, females were found to have lower emotional well-being than males. 9 This sex difference in HRQOL was not seen in a different study involving adolescents with CP.4 Gross motor function categorized by the Gross Motor Function Classification System (GMFCS) 10 has been associated with lower HRQOL in several studies. 4,5,7 Studies have investigated the relationship of age, GMFCS and/or sex with pain in children with CP. The SPARCLE study identified that older children reported more pain than younger children.11 Likewise, another study found that older age (>14y) predicted the presence of musculoskeletal pain.12 A positive association between severity of motor impairment (GMFCS level) and parentreported pain ...

Children with cerebral palsy (CP) want to play fast-paced action-oriented videogames similar to those played by their peers without motor disabilities. This is particularly true of exergames, whose physically-active gameplay matches the fast pace of action games. But disabilities resulting from CP can make it difficult to play action games. Guidelines for developing games for people with motor disabilities steer away from high-paced action, including recommendations to avoid the need for time-sensitive actions and to keep game pace slow. Through a year-long participatory design process with children with CP, we have discovered that it is in fact possible to develop action-oriented exergames for children with CP at level III on the Gross Motor Function Classification Scale. We followed up the design process with an eight-week home trial, in which we found the games to be playable and enjoyable. In this paper, we discuss the design of these games, and present a set of design recommendations for how to achieve both actionorientation and playability.

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.