The translation regulator Zar1l controls timing of meiosis in Xenopus oocytes.

Oocyte maturation and early embryo development occur in vertebrates in the near absence of transcription. Thus, sexual reproduction of vertebrates critically depends on the timely translation of mRNAs already stockpiled in the oocyte. Yet how translational activation of specific mRNAs is temporally coordinated is still incompletely understood. Here, we elucidate the function of Zar1l, a yet uncharacterized member of the Zar RNA-binding protein family, in Xenopus oocytes. Employing TRIM-Away, we demonstrate that loss of Zar1l accelerates hormone-induced meiotic resumption of Xenopus oocytes due to premature accumulation of the M-phase-promoting kinase cMos. We show that Zar1l is a constituent of a large ribonucleoparticle containing the translation repressor 4E-T and the central polyadenylation regulator CPEB1, and that it binds directly to the cMos mRNA. Partial, hormone-induced degradation of Zar1l liberates 4E-T from CPEB1, which weakens translational repression of mRNAs encoding cMos and likely additional M-phase-promoting factors. Thus, our study provides fundamental insights into the mechanisms that ensure temporally regulated translation of key cell cycle regulators during oocyte maturation, which is essential for sexual reproductivity.

Further recommendations of the International Paediatric Brain Injury Society (IPBIS) for the post-acute rehabilitation of children with acquired brain injury.

BACKGROUND: Paediatric acquired brain injury is a life-long condition which impacts on all facets of the individual's lived experience. The existing evidence base continues to expand and new fields of enquiry are established as clinicians and researchers uncover the extent of these impacts. PRIMARY OBJECTIVE: To add to recommendations described in the International Paediatric Brain Injury Society's 2016 paper on post-acute care for children with acquired brain injury and highlight new areas of enquiry. REVIEW OF INFORMATION: Recommendations were made based on the opinions of a group of experienced international clinicians and researchers who are current or past members of the board of directors of the International Paediatric Brain Injury Society. The importance of each recommendation was agreed upon by means of group consensus. OUTCOMES: This update gives new consideration to areas of study including injuries which occur in pre-school children, young people in the military, medical referral, young offenders and the use of technology in rehabilitation.

Clinical utility of a pediatric hand exoskeleton: identifying users, practicability, and acceptance, and recommendations for design improvement.

BACKGROUND: Children and adolescents with upper limb impairments can experience limited bimanual performance reducing daily-life independence. We have developed a fully wearable pediatric hand exoskeleton (PEXO) to train or compensate for impaired hand function. In this study, we investigated its appropriateness, practicability, and acceptability. METHODS: Children and adolescents aged 6-18 years with functional limitations in at least one hand due to a neurological cause were selected for this cross-sectional evaluation. We characterized participants by various clinical tests and quantified bimanual performance with the Assisting Hand Assessment (AHA). We identified children whose AHA scaled score increased by ≥ 7 points when using the hand exoskeleton and determined clinical predictors to investigate appropriateness. The time needed to don each component and the number of technical issues were recorded to evaluate practicability. For acceptability, the experiences of the patients and the therapist with PEXO were evaluated. We further noted any adverse events. RESULTS: Eleven children (median age 11.4 years) agreed to participate, but data was available for nine participants. The median AHA scaled score was higher with PEXO (68; IQR: 59.5-83) than without (55; IQR: 37.5-80.5; p = 0.035). The Box and Block test, the Selective Control of the Upper Extremity Scale, and finger extensor muscle strength could differentiate well between those participants who improved in AHA scaled scores by ≥ 7 points and those who did not (sensitivity and specificity varied between 0.75 and 1.00). The median times needed to don the back module, the glove, and the hand module were 62, 150, and 160 s, respectively, but all participants needed assistance. The most critical failures were the robustness of the transmission system, the electronics, and the attachment system. Acceptance was generally high, particularly in participants who improved bimanual performance with PEXO. Five participants experienced some pressure points. No adverse events occurred. CONCLUSIONS: PEXO is a safe exoskeleton that can improve bimanual hand performance in young patients with minimal hand function. PEXO receives high acceptance. We formulated recommendations to improve technical issues and the donning before such exoskeletons can be used under daily-life conditions for therapy or as an assistive device. Trial registration Not appropriate.

Calcineurin promotes APC/C activation at meiotic exit by acting on both XErp1 and Cdc20.

Vertebrate oocytes await fertilization arrested at metaphase of the second meiotic division. Fertilization triggers a transient calcium wave, which induces the activation of the anaphase-promoting complex/cyclosome (APC/C) and its co-activator Cdc20 resulting in the destruction of cyclin B and hence meiotic exit. Two calcium-dependent enzymes are implicated in fertilization-induced APC/CCdc20 activation: calcium-/calmodulin-dependent kinase type II (CaMKII) and calcineurin (CaN). While the role of CaMKII in targeting the APC/C inhibitor XErp1/Emi2 for destruction is well-established, it remained elusive how CaN affects APC/CCdc20 activation. Here, we discover that CaN contributes to APC/CCdc20 activation in Xenopus laevis oocytes by two independent but interrelated mechanisms. First, it facilitates the degradation of XErp1 by dephosphorylating it at a site that is part of a phosphorylation-dependent recruiting motif for PP2A-B'56, which antagonizes inhibitory phosphorylation of XErp1. Second, it dephosphorylates Cdc20 at an inhibitory site, thereby supporting its APC/C-activating function. Thus, our comprehensive analysis reveals that CaN contributes to timely APC/C activation at fertilization by both negatively regulating the APC/C inhibitory activity of XErp1 and positively regulating the APC/C-activating function of Cdc20.

Dual-task training of children with neuromotor disorders during robot-assisted gait therapy: prerequisites of patients and influence on leg muscle activity.

BACKGROUND: Walking in daily life is complex entailing various prerequisites such as leg strength, trunk stability or cognitive and motor dual task (DT) activities. Conventional physiotherapy can be complemented with robot-assisted gait therapy (RAGT) and exergames to enhance the number of step repetitions, feedback, motivation, and additional simultaneously performed tasks besides walking (e.g., dual-task (DT) activities). Although DT gait training leads to improvements in daily ambulation in adult patient groups, no study has evaluated RAGT with a DT exergame in children with neurological gait disorders. Therefore, we investigated children's functional and cognitive prerequisites to walk physiologically during RAGT with a DT exergame and analysed the influence of DT on leg muscle activity. METHODS: Children and adolescents (6-18 years) with neurological gait disorders completed RAGT with and without a DT exergame in this quasi-experimental study. We assessed several measures on the body function and activity domains (according to the International Classification of Functioning, Disability, and Health (ICF)) and determined whether these measures could distinguish well between children who walked physiologically during the DT RAGT or not. We measured leg muscle activity with surface electrodes to identify changes in EMG-amplitudes and -patterns. RESULTS: Twenty-one children participated (7 females, 6.5-17.3 years, Gross Motor Function Classification System (GMFCS) levels I-IV). Most activity measures distinguished significantly between participants performing the DT exergame physiologically or not with moderate to good sensitivity (0.8 ≤ sensitivity≤1.0) and specificity (0.5 ≤ specificity≤0.9). Body function measures differentiated less well. Despite that the EMG-amplitudes of key stance muscles were significantly lower during DT versus no DT exergaming, the mean activation patterns of all muscles correlated high (ρ > 0.75) between the conditions. CONCLUSION: This study is the first that investigated effects of a DT exergame during RAGT in children with neurological gait disorders. Several performance measures could differentiate well between patients who walked with physiological versus compensatory movements while performing the DT exergame. While the DT exergame affected the leg muscle activity amplitudes, it did not largely affect the activity patterns of the muscles.

Advanced Robotic Therapy Integrated Centers (ARTIC): an international collaboration facilitating the application of rehabilitation technologies.

BACKGROUND: The application of rehabilitation robots has grown during the last decade. While meta-analyses have shown beneficial effects of robotic interventions for some patient groups, the evidence is less in others. We established the Advanced Robotic Therapy Integrated Centers (ARTIC) network with the goal of advancing the science and clinical practice of rehabilitation robotics. The investigators hope to exploit variations in practice to learn about current clinical application and outcomes. The aim of this paper is to introduce the ARTIC network to the clinical and research community, present the initial data set and its characteristics and compare the outcome data collected so far with data from prior studies. METHODS: ARTIC is a pragmatic observational study of clinical care. The database includes patients with various neurological and gait deficits who used the driven gait orthosis Lokomat® as part of their treatment. Patient characteristics, diagnosis-specific information, and indicators of impairment severity are collected. Core clinical assessments include the 10-Meter Walk Test and the Goal Attainment Scaling. Data from each Lokomat® training session are automatically collected. RESULTS: At time of analysis, the database contained data collected from 595 patients (cerebral palsy: n = 208; stroke: n = 129; spinal cord injury: n = 93; traumatic brain injury: n = 39; and various other diagnoses: n = 126). At onset, average walking speeds were slow. The training intensity increased from the first to the final therapy session and most patients achieved their goals. CONCLUSIONS: The characteristics of the patients matched epidemiological data for the target populations. When patient characteristics differed from epidemiological data, this was mainly due to the selection criteria used to assess eligibility for Lokomat® training. While patients included in randomized controlled interventional trials have to fulfill many inclusion and exclusion criteria, the only selection criteria applying to patients in the ARTIC database are those required for use of the Lokomat®. We suggest that the ARTIC network offers an opportunity to investigate the clinical application and effectiveness of rehabilitation technologies for various diagnoses. Due to the standardization of assessments and the use of a common technology, this network could serve as a basis for researchers interested in specific interventional studies expanding beyond the Lokomat®.

Protein phosphatase 1 is essential for Greatwall inactivation at mitotic exit.

Entry into mitosis is mediated by the phosphorylation of key cell cycle regulators by cyclin-dependent kinase 1 (Cdk1). In Xenopus embryos, the M-phase-promoting activity of Cdk1 is antagonized by protein phosphatase PP2A-B55. Hence, to ensure robust cell cycle transitions, Cdk1 and PP2A-B55 must be regulated so that their activities are mutually exclusive. The mechanism underlying PP2A-B55 inactivation at mitotic entry is well understood: Cdk1-activated Greatwall (Gwl) kinase phosphorylates Ensa/Arpp19, thereby enabling them to bind to and inhibit PP2A-B55. However, the re-activation of PP2A-B55 during mitotic exit, which is essential for cell cycle progression, is less well understood. Here, we identify protein phosphatase PP1 as an essential component of the PP2A-B55 re-activation pathway in Xenopus embryo extracts. PP1 initiates the re-activation of PP2A-B55 by dephosphorylating Gwl. We provide evidence that PP1 targets the auto-phosphorylation site of Gwl, resulting in efficient Gwl inactivation. This step is necessary to facilitate subsequent complete dephosphorylation of Gwl by PP2A-B55. Thus, by identifying PP1 as the phosphatase initiating Gwl inactivation, our study provides the molecular explanation for how Cdk1 inactivation is coupled to PP2A-B55 re-activation at mitotic exit.

Regulation of Cell Division.

The challenging task of mitotic cell divisions is to generate two genetically identical daughter cells from a single precursor cell. To accomplish this task, a complex regulatory network evolved, which ensures that all events critical for the duplication of cellular contents and their subsequent segregation occur in the correct order, at specific intervals and with the highest possible fidelity. Transitions between cell cycle stages are triggered by changes in the phosphorylation state and levels of components of the cell cycle machinery. Entry into S-phase and M-phase are mediated by cyclin-dependent kinases (Cdks), serine-threonine kinases that require a regulatory cyclin subunit for their activity. Resetting the system to the interphase state is mediated by protein phosphatases (PPs) that counteract Cdks by dephosphorylating their substrates. To avoid futile cycles of phosphorylation and dephosphorylation, Cdks and PPs must be regulated in a manner such that their activities are mutually exclusive.

Insulin-like growth factor 2 mRNA-binding protein 1 (IGF2BP1) is an important protumorigenic factor in hepatocellular carcinoma.

UNLABELLED: Hepatocarcinogenesis is a stepwise process. It involves several genetic and epigenetic alterations, e.g., loss of tumor suppressor gene expression (TP53, PTEN, RB) as well as activation of oncogenes (c-MYC, MET, BRAF, RAS). However, the role of RNA-binding proteins (RBPs), which regulate tumor suppressor and oncogene expression at the posttranscriptional level, are not well understood in hepatocellular carcinoma (HCC). Here we analyzed RBPs induced in human liver cancer, revealing 116 RBPs with a significant and more than 2-fold higher expression in HCC compared to normal liver tissue. We focused our subsequent analyses on the Insulin-like growth factor 2 messenger RNA (mRNA)-binding protein 1 (IGF2BP1) representing the most strongly up-regulated RBP in HCC in our cohort. Depletion of IGF2BP1 from multiple liver cancer cell lines inhibits proliferation and induces apoptosis in vitro. Accordingly, murine xenograft assays after stable depletion of IGF2BP1 reveal that tumor growth, but not tumor initiation, strongly depends on IGF2BP1 in vivo. At the molecular level, IGF2BP1 binds to and stabilizes the c-MYC and MKI67 mRNAs and increases c-Myc and Ki-67 protein expression, two potent regulators of cell proliferation and apoptosis. These substrates likely mediate the impact of IGF2BP1 in human liver cancer, but certainly additional target genes contribute to its function. CONCLUSION: The RNA-binding protein IGF2BP1 is an important protumorigenic factor in liver carcinogenesis. Hence, therapeutic targeting of IGF2BP1 may offer options for intervention in human HCC.

Practical Recommendations for Robot-Assisted Treadmill Therapy (Lokomat) in Children with Cerebral Palsy: Indications, Goal Setting, and Clinical Implementation within the WHO-ICF Framework.

Active participation and the highest level of independence during daily living are primary goals in neurorehabilitation. Therefore, standing and walking are key factors in many rehabilitation programs. Despite inconclusive evidence considering the best application and efficacy of robotic tools in the field of pediatric neurorehabilitation, robotic technologies have been implemented to complement conventional therapies in recent years. A group of experienced therapists and physicians joined in an "expert panel." They compared their clinical application protocols, discussed recurring open questions, and developed experience-based recommendations for robot-assisted treadmill therapy (exemplified by the Lokomat, Hocoma, Volketswil, Switzerland) with a focus on children with cerebral palsy. Specific indications and therapeutic goals were defined considering the severity of motor impairments and the International Classification of Functioning, Disability and Health framework (ICF). After five meetings, consensus was found and recommendations for the implementation of robot-assisted treadmill therapy including postsurgery rehabilitation were proposed. This article aims to provide a comprehensive overview on therapeutical applications in a fast developing field of medicine, where scientific evidence is still scarce. These recommendations can help physicians and therapists to plan the child's individual therapy protocol of robot-assisted treadmill therapy.

Intrathecal baclofen therapy in children with acquired brain injuries after drowning: A case series.

UNLABELLED: Objective: To investigate clinical efficacy as well as the incidence and extent of complications regarding intrathecal baclofen (ITB) therapy in children. METHODS: This is a retrospective medical chart review of three paediatric patients with acquired brain injuries (ABI) resulting from drowning who underwent ITB pump implantation for treatment of severe spasticity. RESULTS: Compared to the pre-operative state, ITB therapy reduced spasticity with a corresponding decrease of modified Ashworth scale in upper (3.2 ± 1.4 to 1.3 ± 0.6) and lower extremities (3.5 ± 0.9 to 2.0 ± 1.0). Overall, six complications, five device-related and one accidental, were found in two out of three patients. CONCLUSION: Intrathecal baclofen is an effective therapy option for paediatric patients with ABI after drowning to significantly reduce spasticity of upper and lower extremities. A word of caution must be addressed to the incidence and extent of complications related to ITB therapy.

Clinicoradiological correlation of scoliosis in children with Jarcho-Levin and Escobar syndromes: associated "flat bone or wing-like" imaging findings.

UNLABELLED: Congenital or early onset scoliosis may be the lead clinical feature in several rare syndromes. In this paper, we present the imaging findings in two children with early onset scoliosis related to the Jarcho-Levin and Escobar syndromes and an osseous plate or wing-like bar extending along the posterior elements of the spine on computed tomography. The clinical phenotypes in these syndromes are variable. A thorough clinical evaluation with imaging correlation is essential. The recognition of underlying spinal anomalies is essential in planning treatment and estimating prognosis. In young children with progressive scoliosis, cross-sectional imaging plays a major role in the diagnostic work-up. CONCLUSION: Congenital scoliosis requires a comprehensive clinical evaluation and imaging work-up. The presence of an osseous plate or wing-like fusion of posterior elements of the spine may suggest the diagnosis of Jarcho-Levin and Escobar syndromes.

Perspectives of Swiss Paediatric Health Care Professionals on Factors Influencing Physical Activity Participation in Children with Disabilities.

AIM: Investigation of the perspectives of paediatric health care professionals (PHCPs) in Switzerland regarding factors that influence participation in physical activity programs for children and adolescents with disabilities or chronic conditions (CADCCs). Evaluation of self-reported exercise counselling behavior of those professionals. METHOD: A cross-sectional survey was used to collect the opinions of 171 PHCPs working with CADCCs using a structured questionnaire. The information obtained was evaluated by performing a combined quantitative and qualitative statistical analysis. RESULTS: PHCPs in Switzerland think that CADCC do not get enough physical activity and see the underlying reasons in lacking information/knowledge and organizational factors. We found that the level of knowledge about disability sports opportunities among PHCPs has a positive influence on their exercise counselling behaviour. CONCLUSIONS: We propose three approaches to increase the level of physical activity in CADCC: Establishing personalized exercise counseling, intensifying information about disability sports programmes towards PHCPs, and improving inclusion and integration in PE lessons or regular sports clubs.

The Variable Influence of Orthotic Management on Hip and Pelvic Rotation in Children with Unilateral Neurogenic Equinus Deformity.

BACKGROUND: Equinus deformity with or without concomitant drop foot is a common finding in children with unilateral spastic cerebral palsy and spastic hemiplegia of other causes. Hypothetically, these deformities may lead to pelvic retraction and hip internal rotation during gait. Orthoses are used to reduce pes equinus during gait and to restore hindfoot first contact. OBJECTIVE: We aimed to investigate whether the use of orthotic equinus correction reduces rotational hip and pelvic asymmetries. METHODS: In a retrospective study, 34 children with unilateral spastic cerebral palsy or spastic hemiplegia of other causes underwent standardized instrumented 3D gait analysis with and without orthotic equinus management. We analyzed the differences in the torsional profile during barefoot walking and while wearing orthoses, as well as investigated the influence of ankle dorsiflexion and femoral anteversion on pelvic and hip kinematics and hip kinetics. RESULTS: Wearing orthoses corrected pes equinus and pelvic internal rotation at the end of the stance phase and in the swing phase compared to barefoot walking. Hip rotation and the rotational moment did not significantly change with orthoses. Orthotic management or femoral anteversion did not correlate to pelvic and hip asymmetry. CONCLUSION: The findings indicate that the correction of the equinus by using orthoses had a variable effect on the asymmetry of the hip and pelvis and internal rotation; both appear to have a multifactorial cause that is not primarily driven by the equinus component.

A Tailorable Robotic Hand Orthosis to Support Children with Neurological Hand Impairments: a Case Study in a Child's Home.

Neurological disorders such as traumatic brain injuries (TBI) can lead to hand impairments in children, negatively impacting their quality of life. Fully wearable robotic hand orthoses (RHO) have been proposed to actively support children and promote the use of the impaired limb in daily life. Here we report a case study on the feasibility of using the pediatric RHO PEXO for assistance at home in a 13- year-old child with hand impairment after TBI. The size and functionalities of the RHO were first fully tailored to the child's needs. We trained the child and their parent on independently using the RHO before taking it home for a period of two weeks. The use of the RHO improved hand ability. Additionally, the tailoring and training benefited the unimanual capacity (Box and Block Test score +2 after tailoring) and bimanual performance (Assisting Hand Assessment score +4) of the child with PEXO. Further, it increased device acceptance by the child and the parent. The child used PEXO at home for 76 minutes distributed over three days during eating and drinking tasks. Personal and environmental factors caused the moderate use. No adverse events or safety-related issues occurred. This study highlights the value of tailoring an assistive RHO and, for the first time, demonstrates the feasibility of home use of a pediatric RHO by children with neurological hand impairments.

Assessment of compression forces in a digitally modified short leg cast for pressure injury risk monitoring in healthy children.

Introduction: Casting is an essential treatment for neuro-orthopedic conditions in children with cognitive, sensory, and communicational disabilities. However, a main side-effect is the development of pressure injuries resulting in additional (wound) therapies and prolongation of the hospital stay. The primary aim of our study was to investigate the potential of objective pressure measurements in casts to assess the risk for pressure injury development. Methods: Five pediatric healthy participants were included in this study. We measured the global and the local compression force at body sites prone to pressure injury development for different body positions and the transfer in-between in a cast equipped with pressure sensors. These conditions resulted in partial or full body weight loading. Results and discussion: The global maximum compression force was affected significantly by body postures with partial and full loading of the cast and during transfer. The local compression force significantly correlated with the global compression force at the heel and instep area. In conclusion, the integration of sensing technologies into casts bears a high potential for early recognition of critical conditions inside the cast and inducing preventive measures in the at-risk population.

Cyclin B3 implements timely vertebrate oocyte arrest for fertilization.

To ensure successful offspring ploidy, vertebrate oocytes must halt the cell cycle in meiosis II until sperm entry. Emi2 is essential to keep oocytes arrested until fertilization. However, how this arrest is implemented exclusively in meiosis II and not prematurely in meiosis I has until now remained enigmatic. Using mouse and frog oocytes, we show here that cyclin B3, an understudied B-type cyclin, is essential to keep Emi2 levels low in meiosis I. Direct phosphorylation of Emi2 at an evolutionarily highly conserved site by Cdk1/cyclin B3 targets Emi2 for degradation. In contrast, Cdk1/cyclin B1 is inefficient in Emi2 phosphorylation, and this provides a molecular explanation for the requirement of different B-type cyclins for oocyte maturation. Cyclin B3 degradation at exit from meiosis I enables Emi2 accumulation and thus timely arrest in meiosis II. Our findings illuminate the evolutionarily conserved mechanisms that control oocyte arrest for fertilization at the correct cell-cycle stage, which is essential for embryo viability.

Design of a compliant, stabilizing wrist mechanism for a pediatric hand exoskeleton.

Children affected by hand impairment due to cerebral palsy or stroke experience serious difficulties when performing activities of daily life (ADL), which reduces their quality of life and development. Wearable robots such as hand exoskeletons have been proposed to support people with hand impairment in therapy as well as daily tasks. While numerous actuated wearable robots have been developed, few designs support both fingers and wrist function, despite being mutually relevant for reach-to-grasp tasks. A recent feasibility study investigating the use of PEXO, a lightweight and fully wearable pediatric hand exoskeleton, showed that a wrist fixed in a slightly extended position may limit the user's ability to reach and grasp during ADL and restrict the user group. These insights and further interactions with clinicians inspired a novel design of PEXO that features an additional degree of freedom in the wrist. In this paper, we present a compliant wrist mechanism extending the existing leaf spring finger mechanism of the device. The novel design provides both wrist motion capability of 60° in flexion and extension and wrist stabilization at the same time while actively supporting finger motion. Preliminary results suggest that the adjustability in the wrist enables a larger variety of grasping gestures. The implemented wrist support has the potential to allow for a more versatile use of PEXO and increase the potential target user group.

Cohort profile: the Swiss Cerebral Palsy Registry (Swiss-CP-Reg) cohort study.

BACKGROUND: Cerebral Palsy (CP) is a group of permanent disorders of movement and posture that follow injuries to the developing brain. It results in motor dysfunction and a wide variety of comorbidities like epilepsy; pain; speech, hearing and vision disorders; cognitive dysfunction; and eating and digestive difficulties. Central data collection is essential to the study of the epidemiology, clinical presentations, care, and quality of life of patients affected by CP. CP specialists founded the Swiss Cerebral Palsy Registry (Swiss-CP-Reg) in 2017. This paper describes the design, structure, aims and achievements of Swiss-CP-Reg and presents its first results. METHODS: Swiss-CP-Reg records patients of any age diagnosed with CP who are born, are treated, or live in Switzerland. It collects data from medical records and reports, from questionnaires answered by patients and their families, and from data linkage with routine statistics and other registries. The registry contains information on diagnosis, clinical presentation, comorbidities, therapies, personal information, family history, and quality of life. RESULTS: From August 2017 to August 2021, 546 participants (55% male, mean age at registration 8 years [interquartile range IQR: 5-12]), were enrolled in Swiss-CP-Reg. Most had been born at term (56%), were less than two years old at diagnosis (73%, median 18 months, IQR: 9-25), and were diagnosed with spastic CP (76%). Most (59%) live with a mild motor impairment (Gross Motor Function Classification System [GMFCS] level I or II), 12% with a moderate motor impairment (GMFCS level III), and 29% with a severe motor impairment (GMFCS level IV or V). In a subset of 170 participants, we measured intelligence quotient (IQ) and saw lower IQs with increasing GMFCS level. Swiss-CP-Reg has a strong interest in research, with four nested projects running currently, and many more planned. CONCLUSIONS: Swiss-CP-Reg collects and exchanges national data on people living with CP to answer clinically relevant questions. Its structure enables retrospective and prospective data collection and knowledge exchange between experts to optimise and standardise treatment and to improve the health and quality of life of those diagnosed with CP in Switzerland.

Changes in health-related quality of life after spinal fusion and scoliosis correction in patients with cerebral palsy.

BACKGROUND: The literature is scarce on the impact of spinal fusion for scoliosis in patients with cerebral palsy (CP) regarding the health-related quality of life (HRQL). The purpose of this study was to evaluate the outcome of surgical scoliosis correction measured by the subjective change in the HRQL and the objective radiologic changes. Factors that could influence the subjective outcome were examined to investigate their correlation to the results of HRQL. METHODS: A retrospective review of 50 consecutive patients with CP, who had spinal fusion for scoliosis with minimal 2-year follow-up was carried out. Radiographic data were obtained from preoperative, postoperative, and last follow-up examinations. The assessment of the HRQL was done through a modified version of the "Caregiver Priorities and Child Health Index of Life with Disabilities" questionnaire, assessed by the caregivers of the patients. RESULTS: There was a significant improvement (P = 0.001) of HRQL after the operation. The satisfaction rate of the patients with the outcome of the operation was 91.7%. There was an average of 64.3% scoliosis correction, 57.7% pelvic tilt correction, 53% improvement of apical vertebral rotation, and 67.2% improvement of apical vertebral translation. At the last follow-up, the average scoliosis angle was 32.0 degrees and pelvic tilt was 8.8 degrees. Weak but not significant correlation between the amount of scoliosis correction and the subjective change in the HRQL could be established (R = 0.321, P = 0.078). No correlation between the occurrence of complications and changes in the HRQL (P = 0.122) or the satisfaction rate with the outcome of the operation (P = 0.764) was found. Extension of spinal fusion to sacropelvis had no influence on the occurrence of complications (P = 0.42) or on the changes in HRQL (P = 0.71). CONCLUSIONS: Life quality improved after surgical scoliosis correction in patients with CP. There is a high satisfaction rate of patients and their caregivers. Subjective changes in HRQL after the operation do not correlate with objective radiographic changes brought about by the operation, which indicates that the present operation indications and achieved correction are adequate to achieve an improvement of the subjective HRQL in this patient group. LEVEL OF EVIDENCE: Therapeutic level IV, retrospective study.