Dystonic Cerebral Palsy Research Articles

Multi-center improvement in screening for dystonia in young people with cerebral palsy.

Dystonia is a common, debilitating, and often treatment refractory motor symptom of cerebral palsy (CP), affecting 70-80% of this population based on research assessments. However, routine clinical evaluation for dystonia in CP has failed to match these expected numbers. Addressing this diagnostic gap is a medical imperative because the presence of dystonia rules in or out certain treatments for motor symptoms in CP. Therefore, our objective was to optimize rates of clinical dystonia screening to improve rates of clinical dystonia diagnosis. Using the quality improvement (QI) infrastructure of the Cerebral Palsy Research Network (CPRN), we developed and implemented interventions to increase the documentation percentage of five features of dystonia in young people with CP, aged 3-21 years old. This QI initiative was implemented by seven physiatry and pediatric movement disorders physicians at four tertiary-care pediatric hospitals between 10/10/21 and 7/1/23. We collected visit data cross-sectionally across all participating sites every 2 weeks and tracked our progress using control charts. We assessed 847 unique visits, mostly for established patients (719/847, 85%) who were 9.2 years old on average (95% CI 8.8-9.5). By 4/10/22, the mean percentage of dystonia screening elements documented across all sites rose from 39% to 90% and the mean percentage of visits explicitly documenting the presence or absence of dystonia rose from 65% to 94%. By 10/23/22, the percentage of visits diagnosing dystonia rose from 57% to 74%. These increases were all sustained through the end of the study period in 7/1/23. Using a rigorous QI-driven process across four member sites of a North American learning health network (CPRN), we demonstrated that we could increase screening for dystonia and that this was associated with increased clinical dystonia diagnosis, matching expected research-based rates. We propose that similar screening should take place across all sites caring for people with CP.

Clinical Phenotypes and Etiological Risk Factors in Children with Cerebral Palsy: A Retrospective Study

Background: Cerebral palsy is a motor impairment syndrome that arises from a lesion in the developing brain. The condition varies based on the timing of the lesion, as well as differences in clinical presentation, location, and severity. Objectives: The aim of this study is to determine the clinical features of patients with cerebral palsy, identify the associated risk factors, and analyze cranial MRI findings in relation to the different types of cerebral palsy. Methods: This study retrospectively evaluated the medical records of children diagnosed with cerebral palsy. Data collected included demographic information, details of potential relevant abnormalities during pregnancy, birth, and the neonatal period, comorbidities, types of cerebral palsy, cranial MRI findings, and classifications of motor impairments. Results: The mean age of the 202 patients with cerebral palsy in the study was 8.02 years, with a male predominance (60.4%). The most common type of cerebral palsy observed was spastic cerebral palsy, accounting for 81.6% of the cases. The most frequent cranial MRI finding was white matter damage of immaturity, present in 36.1% of the patients, while 8.4% of the cases had normal cranial MRI results. Epilepsy was the most common comorbidity, affecting 50.5% of the patients. White matter damage of immaturity was most commonly associated with spastic diparesis, and the identified risk factors for these patients included prematurity, low birth weight, oligohydramnios, multiple pregnancy, and neonatal sepsis/meningitis. Spastic tetraparesis was most frequently linked to miscellaneous MRI findings, with risk factors such as term birth, normal birth weight, polyhydramnios, asphyxia/resuscitation, and a history of neonatal convulsions. Focal infarction/hemorrhage was most often associated with spastic hemiparesis, while basal ganglia-thalamus damage was frequently seen in patients with choreoathetotic and dystonic cerebral palsy. In patients with dystonic cerebral palsy, vaginal delivery and a history of hyperbilirubinemia were identified as common risk factors. Cerebellar damage and malformations were most frequently associated with ataxic cerebral palsy. Conclusions: In this study, it was determined that MRI findings can serve as a valuable guide for identifying different types of cerebral palsy. Additionally, the risk factors, disease severity, and accompanying comorbidities were found to vary according to the specific type of cerebral palsy.

Efficacy of Oral Trihexyphenidyl Plus Clonazepam Versus Trihexyphenidyl for the Treatment of Dystonia in Children With Dystonic Cerebral Palsy: An Open-Label Randomized Controlled Trial

BackgroundTrihexyphenidyl and clonazepam are commonly used to treat dystonia in children with cerebral palsy (CP). However, there is a notable gap in the literature when it comes to studies that combine these first-line agents for the management of dystonia. MethodsThis open-label, randomized controlled trial aimed to compare the efficacy of adding oral clonazepam to trihexyphenidyl (THP + CLZ) versus using trihexyphenidyl alone (THP) in reducing the severity of dystonia, as measured by the Barry-Albright Dystonia (BAD) score. The study was conducted over a 12-week therapy period in children with dystonic CP aged two to 14 years. ResultsEach group enrolled 51 participants. The THP + CLZ group showed significantly better improvement in dystonia severity at 12 weeks compared with the THP group alone (−4.5 ± 2.9 vs −3.4 ± 1.7, P = 0.02). Furthermore, the THP + CLZ group exhibited superior improvement in the severity of choreoathetosis, upper limb function, pain perception by the child, and quality of life, with P values of 0.02, 0.009, 0.01, and 0.01, respectively. The number of participants experiencing treatment-emergent adverse events was comparable in both groups (P = 0.67). Importantly, none of the participants in any of the groups reported any serious adverse events. ConclusionA combination of oral THP + CLZ proves to be more efficacious than using THP alone for the treatment of dystonic CP in children aged two to 14 years in terms of reducing the severity of dystonia.

Physician Approaches to the Pharmacologic Treatment of Dystonia in Cerebral Palsy.

Physician Approaches to the Pharmacologic Treatment of Dystonia in Cerebral Palsy.

Dystonic Cerebral Palsy-Like Presentation Caused by a Novel TCF20 Variant.

Dystonic Cerebral Palsy-Like Presentation Caused by a Novel TCF20 Variant.

Consensus guidelines for the diagnosis and management of isolated sulfite oxidase deficiency and molybdenum cofactor deficiencies.

Sulfite intoxication is the hallmark of four ultrarare disorders that are caused by impaired sulfite oxidase activity due to genetic defects in the synthesis of the molybdenum cofactor or of the apoenzyme sulfite oxidase. Delays on the diagnosis of these disorders are common and have been caused by their unspecific presentation of acute neonatal encephalopathy with high early mortality, followed by the evolution of dystonic cerebral palsy and also by the lack of easily available and reliable diagnostic tests. There is significant variation in survival and in the quality of symptomatic management of affected children. One of the four disorders, molybdenum cofactor deficiency type A (MoCD-A) has recently become amenable to causal treatment with synthetic cPMP (fosdenopterin). The evidence base for the rational use of cPMP is very limited. This prompted the formulation of these clinical guidelines to facilitate diagnosis and support the management of patients. The guidelines were developed by experts in diagnosis and treatment of sulfite intoxication disorders. It reflects expert consensus opinion and evidence from a systematic literature search.

Ablation of Iron Regulatory Protein 2 produces a neurological disorder characterized by motor, somatosensory, and executive dysfunction in mice

Iron is an important cofactor for many proteins and is used to create Fe-S clusters and heme prosthetic groups that enzymes use to catalyze enzymatic reactions. Proteins involved in the import, export, and sequestration of iron are regulated by Iron Regulatory Proteins (IRPs). Recently, a patient with bi-allelic loss of function mutations in IREB2 leading to the absence of IRP2 protein was discovered. The patient failed to achieve developmental milestones and was diagnosed with dystonic cerebral palsy, epilepsy, microcytic hypochromic anemia, and frontal lobe atrophy. Several more IREB2 deficient patients subsequently identified manifested similar neurological problems. To better understand the manifestations of this novel neurological disease, we subjected an Irp2-null mouse model to extensive behavioral testing. Irp2-null mice had a significant motor deficit demonstrated by reduced performance on rotarod and hanging wire tests. Somatosensory function was also compromised in hot and cold plate assays. Their spatial search strategy was impaired in the Barnes maze and they exhibited a difficulty in flexibly adapting their response in the operant touchscreen reversal learning task. The latter is a cognitive behavior known to require an intact prefrontal cortex. These results suggest that loss of Irp2 in mice causes motor and behavioral deficits that faithfully reflect the IREB2 patient's neurodegenerative disorder.

Classic ketogenic diet versus further antiseizure medicine in infants with drug-resistant epilepsy (KIWE): a UK, multicentre, open-label, randomised clinical trial

Many infancy-onset epilepsies have poor prognosis for seizure control and neurodevelopmental outcome. Ketogenic diets can improve seizures in children older than 2 years and adults who are unresponsive to antiseizure medicines. We aimed to establish the efficacy of a classic ketogenic diet at reducing seizure frequency compared with further antiseizure medicine in infants with drug-resistant epilepsy. In this phase 4, open-label, multicentre, randomised clinical trial, infants aged 1-24 months with drug-resistant epilepsy (defined as four or more seizures per week and two or more previous antiseizure medications) were recruited from 19 hospitals in the UK. Following a 1-week or 2-week observation period, participants were randomly assigned using a computer-generated schedule, without stratification, to either a classic ketogenic diet or a further antiseizure medication for 8 weeks. Treatment allocation was masked from research nurses involved in patient care, but not from participants. The primary outcome was the median number of seizures per day, recorded during weeks 6-8. All analyses were by modified intention to treat, which included all participants with available data. Participants were followed for up to 12 months. All serious adverse events were recorded. The trial is registered with the European Union Drug Regulating Authorities Clinical Trials Database (2013-002195-40). The trial was terminated early before all participants had reached 12 months of follow-up because of slow recruitment and end of funding. Between Jan 1, 2015, and Sept 30, 2021, 155 infants were assessed for eligibility, of whom 136 met inclusion criteria and were randomly assigned; 75 (55%) were male and 61 (45%) were female. 78 infants were assigned to a ketogenic diet and 58 to antiseizure medication, of whom 61 and 47, respectively, had available data and were included in the modifified intention-to-treat analysis at week 8. The median number of seizures per day during weeks 6-8, accounting for baseline rate and randomised group, was similar between the ketogenic diet group (5 [IQR 1-16]) and antiseizure medication group (3 [IQR 2-11]; IRR 1·33, 95% CI 0·84-2·11). A similar number of infants with at least one serious adverse event was reported in both groups (40 [51%] of 78 participants in the ketogenic diet group and 26 [45%] of 58 participants in the antiseizure medication group). The most common serious adverse events were seizures in both groups. Three infants died during the trial, all of whom were randomly assigned a ketogenic diet: one child (who also had dystonic cerebral palsy) was found not breathing at home; one child died suddenly and unexpectedly at home; and one child went into cardiac arrest during routine surgery under anaesthetic. The deaths were judged unrelated to treatment by local principal investigators and confirmed by the data safety monitoring committee. In this phase 4 trial, a ketogenic diet did not differ in efficacy and tolerability to a further antiseizure medication, and it appears to be safe to use in infants with drug-resistant epilepsy. A ketogenic diet could be a treatment option in infants whose seizures continue despite previously trying two antiseizure medications. National Institute for Health and Care Research.

Power Mobility, Supported Standing and Stepping Device Use in the First Two Years of Life: A Case Report of Twins Functioning at GMFCS V

Mobility experience has a positive impact on activity, participation, socialisation, language and cognition, but children with cerebral palsy (CP), Gross Motor Function Classification System (GMFCS) level V require assistive devices or assistance in all environments. Supported standing devices afford upright, weight-bearing positions to promote muscle, bone, joint and overall health. Supported stepping devices afford stepping and upright independent mobility, positively impacting self-esteem and participation, while power mobility is the only possibility for effective, independent community mobility. These devices and opportunities should be introduced at the age when children who are typically developing are pulling to stand, moving and exploring their environment. A detailed case description including lived experience and device use data is presented for female twins with dystonic tetraplegic CP born at 25 weeks gestational age and functioning at GMFCS level V. The feasibility of using power mobility, standing and stepping devices in home and community settings within the first two years is illustrated. The twins transitioned from spending 24 h in lying positions or being held in arms to spending more than 2 h daily in upright positions and having opportunities to move independently. Positioning and mobility devices can help to address all the F-words for child development: functioning, family, fitness, fun, friends and future.

Two Cases of Degenerative Cervical Spondylotic Myelopathy in Adults with Athetoid and Dystonic Cerebral Palsy.

BACKGROUND Patients with athetoid and dystonic cerebral palsy (ADCP) may develop degenerative changes in the cervical spine that can aggravate their neurological symptoms in adulthood. This report is of 2 cases of ADCP associated with degenerative cervical spondylotic myelopathy in a 39-year-old woman and a 52-year-old man, requiring different surgical treatments. CASE REPORT Case 1. The patient was a 39-year-old woman who had fallen down 7 years before surgery and had since been walking with a cane. Her gait disturbance had worsened in the 2 years prior to surgery, and numbness in her upper limbs appeared. In the year before surgery, spasticity and numbness in the lower limbs worsened, and fine motor impairment also appeared. Because of mild involuntary movements of the neck, cervical laminoplasty from C3 to C6 was performed, and her symptoms remained stable until the last follow-up 4.5 years after surgery. Case 2. The patient was a 52-year-old man who had fallen down 7 years before surgery, resulting in transient limb weakness. In the year before surgery, he had developed fine motor impairment. He subsequently developed gait disturbance and requested cervical surgery. Because of involuntary movements involving the neck and trunk, he underwent cervical posterior fusion from C2 to T1. Six months after surgery, the gait disturbance had improved. CONCLUSIONS This report describes 2 adults with a history of ADCP since birth and highlights that degenerative changes of the cervical spine can occur at a relatively early age in adulthood, requiring an individualized approach to management.

Upper Extremity Dystonia Features in People With Spastic Cerebral Palsy.

Dystonia in cerebral palsy (CP) is debilitating and common, but underdiagnosed, especially when coexistent with spasticity. With dedicated research-based assessment, dystonia is found in most people with spastic CP but is only clinically diagnosed in the minority. To begin addressing the high rates of dystonia underdiagnosis in this population, we determined the key feature experts use to assess upper extremity dystonia in people with spastic CP. In this prospective cohort study, 3 pediatric movement disorder specialists assessed upper extremity dystonia in neurologic examination videos of people with spastic CP and isolated periventricular leukomalacia (PVL) on brain MRI (i.e., those with a brain injury pattern typical for spastic CP). Dystonia severity was rated using the 10-point Global Dystonia Severity Rating Scale, first by each expert independently and then again after consensus-building discussion. Conventional content analysis of these discussions revealed salient features ("codes") that experts used to assess upper extremity dystonia. Code frequency distributions were compared between dystonia severity categories using χ2 tests. We identified 96 people with spastic CP with isolated PVL on brain MRI seen in the St. Louis Children's Hospital CP Center between 2005 and 2018. Of them, 26 people were able and willing to be recorded while doing a standardized set of upper extremity examination maneuvers (age 4-25 years; 28% nonambulatory, 77% White). When assessing their videos, experts cited the "hand" less often and "shoulder" more often with increasing dystonia severity (p < 0.005, χ2 test). "Mirror movements" and the "hand open/close" examination maneuver were cited significantly more frequently in videos when experts were attempting to distinguish between no dystonia and mild dystonia (p < 0.005). Expert clinicians use distinct movement features to assess upper extremity dystonia in people with spastic CP and PVL. Attention to involuntary shoulder (vs hand) movements can help gauge dystonia severity. Differentiation between mirror movements and dystonia, particularly during the hand open/close examination maneuver, may help identify mild dystonia. These results can help guide upper extremity dystonia assessment in people with spastic CP, thus potentially helping mitigate dystonia underdiagnosis.

Identifying Effective Treatments for Dystonia in Patients With Cerebral Palsy: A Precision Therapeutics Approach.

Recent focus on improving the recognition of dystonia in cerebral palsy (DCP) has highlighted the need for more effective treatments. Evidence supports improved functional outcomes with early interventions for patients with cerebral palsy, but it is not known which interventions are most effective for DCP. Current pharmacologic recommendations for DCP are based largely on anecdotal evidence, with medications demonstrating minimal to moderate improvements in dystonia and variable efficacy between patients. Patients, families, and clinicians have identified the need for new and improved treatments in DCP, naming this as the top research theme in a recent Neurology® publication. Precision therapeutics focuses on providing early effective interventions that are individualized to every patient and can guide research priorities to improve treatments for DCP. This commentary outlines current obstacles to improving treatment of DCP and addresses how precision therapeutics can address each of these obstacles through 4 key components: (1) identification of predictive biomarkers to select patients likely to develop DCP in the future and for whom early intervention may be appropriate to delay or prevent full manifestation of dystonia, (2) stratification of patients with DCP into subgroups according to shared features (clinical, functional, biochemical, etc) to provide a targeted intervention based on those shared features, (3) administration of an individualized dose of an effective intervention to ensure adequate concentrations of the therapeutic entity at the site of action, and (4) monitoring of objective biomarkers of response to intervention. With implementation of each of these components of precision therapeutics, new and more effective treatments for every person with DCP can be realized.

N°61 – Abnormal event-related modulation of sensorimotor cortex alpha/mu activity indicates impaired sensorimotor processing in young people with dystonia and dystonic cerebral palsy

N°61 – Abnormal event-related modulation of sensorimotor cortex alpha/mu activity indicates impaired sensorimotor processing in young people with dystonia and dystonic cerebral palsy

Identifying Upper Extremity Features of Dystonia in People with Cerebral Palsy (P5-9.012)

<h3>Objective:</h3> To identify expert clinician-cited features of upper extremity dystonia in people with cerebral palsy (CP). <h3>Background:</h3> Dystonia in CP is debilitating yet underdiagnosed, particularly when co-existent with spasticity. Subjective expert clinician consensus remains the diagnostic gold standard, but the specific features leading experts to make a dystonia diagnosis remain unclear. <h3>Design/Methods:</h3> To determine expert clinician-cited features of dystonia, we performed a conventional content analysis of consensus-building discussions between three pediatric movement disorder specialists as they evaluated upper extremity dystonia severity in 26 neurologic exam videos of seated subjects with CP and spasticity. <h3>Results:</h3> 45.8% of discussion codes related to body region, actions, movement features, laterality, or examination features (with the remainder on severity score deliberation and dystonia diagnostic difficulty). Experts cited involvement of the “shoulder” significantly more frequently as dystonia severity increased (<i>p</i>&lt;0.005, chi-squared test) as opposed to the “hand” which was cited less frequently (<i>p</i>&lt;0.0005, chi-squared test). “Mirror movements” were cited significantly more frequently in videos with no or mild dystonia (p&lt;0.005, chi-squared test). Though “unilateral” was the top cited laterality code (72%) and “variability over time” was the top cited movement feature code (34%), neither were significantly associated with dystonia severity (p=0.09, p=0.052 respectively, chi-squared test), in contrast to our previous lower extremity dystonia analysis findings. In videos where diagnostic consensus was reached only after consensus-building discussion (4/26 videos), the repetitive hand “open/close” exam maneuver was the top cited exam maneuver (28%) and was referred to significantly more frequently than for videos where consensus was reached prior to any discussion (p&lt;0.005). <h3>Conclusions:</h3> Experts use distinct movement features to diagnose upper extremity dystonia in people with CP and spasticity. Efforts like this can be used to target examinations and codify the defining features of dystonia in people with CP, thus helping to facilitate dystonia diagnosis. <b>Disclosure:</b> Dr. Gilbert has nothing to disclose. Ms. Gandham has nothing to disclose. Dr. Pearson has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Teva Pharmaceuticals. The institution of Dr. Pearson has received research support from NIH. Dr. Ueda has nothing to disclose. Dr. Aravamuthan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurocrine Biosciences. An immediate family member of Dr. Aravamuthan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for SK Life Science, Inc.. Dr. Aravamuthan has received research support from National Institute of Neurological Disorders and Stroke.

Trihexyphenidyl in young children with dystonic cerebral palsy: A single arm study.

The incidence of dystonic cerebral palsy causing significant morbidity is on the rise. There is a paucity of evidence for the management of dystonia in children. Forty-one children aged 6 months-5 years with predominantly dystonic cerebral palsy were started on a predetermined protocol of trihexyphenidyl (0.25-0.52 mg/kg) and followed up at 3, 6 and 12 weeks. Dystonia severity, motor function and developmental age at baseline and 12 weeks were compared using the Global Dystonia Scale (GDS), the Gross Motor Function Measure (GMFM), and Fine Motor/Perceptual Subscale of the Early Developmental Profile-2. Thirty-four children completed the entire 12 weeks of intervention. The mean age of participants was 25±11 months. A significant decrease in median total dystonia scores on the GDS was observed post-intervention (74.5 to 59, p < 0.0001), and 64% of participants gained motor milestones. GMFM scores increased significantly from a median of 19.8% pre-intervention to 26.5% post-intervention (p < 0.0001). There was improvement in the fine motor domain as compared to the baseline (p < 0.0001). The number of children classified at Gross Motor Function Classification System levels 1 and 2 increased to 47.05% from 5.88% in the pre-intervention group. Trihexyphenidyl significantly improved dystonia, motor function and development in children with dystonic cerebral palsy in this study. Additional studies are needed to clarify its role in larger numbers of children with this condition.

Predictors of whole exome sequencing in dystonic cerebral palsy and cerebral palsy-like disorders

IntroductionCerebral palsy (CP) is a group of permanent disorders attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. Cerebral palsy-like (CP-like) disorders may clinically resemble CP but do not fulfill CP criteria and have often a progressive course and/or neurodevelopmental regression. To assess which patients with dystonic CP and dystonic CP-like disorder should undergo Whole Exome Sequencing (WES), we compared the rate of likely causative variants in individuals regarding their clinical picture, co-morbidities, and environmental risk factors. MethodIndividuals with early onset neurodevelopmental disorder (ND) manifesting with dystonia as a core feature were divided into CP or CP-like cohorts based on their clinical picture and disease course. Detailed clinical picture, co-morbidities, and environmental risk factors including prematurity, asphyxia, SIRS, IRDS, and cerebral bleeding were evaluated. ResultsA total of 122 patients were included and divided into the CP group with 70 subjects (30 males; mean age 18y5m±16y6m, mean GMFCS score 3.3 ± 1.4), and the CP-like group with 52 subjects (29 males; mean age 17y7m±1y,6 m, mean GMFCS score 2,6 ± 1,5). The WES-based diagnosis was present in 19 (27.1%) CP patients and 30 CP-like patients (57.7%) with genetic conditions overlap in both groups. We found significant differences in diagnostic rate in CP individuals with vs. without risk factors (13.9% vs. 43.3%); Fisher's exact p = 0.0065. We did not observe the same tendency in CP-like (45.5% vs 58.5%); Fisher's exact p = 0.5. ConclusionWES is a useful diagnostic method for patients with dystonic ND, regardless of their presentation as a CP or CP-like phenotype.

Future directions for the assessment of gait dystonia in cerebral palsy.

This commentary is on the original article by Aravamuthan et al. on pages 968–977 of this issue.

Home-based measurements of dystonia in cerebral palsy using smartphone-coupled inertial sensor technology and machine learning: A proof-of-concept study

Home-based measurements of dystonia in cerebral palsy using smartphone-coupled inertial sensor technology and machine learning: A proof-of-concept study

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.

Functional characterization of patients with cerebral palsy living in the Magallanes region and the Chilean Antarctic

To functionally characterize patients with Cerebral Palsy (CP) living in the Magallanes Re gion and the Chilean Antarctic. Descriptive-retrospective observational study of patients with cerebral palsy, registered in the Outpatient Rehabilitation Program of the Corporación de Rehabilitación Club de Leones Cruz del Sur de Punta Arenas between 1986 and 2018. Patients with CP were clinically categorized and then functionally characterized according to gross motor skills (GMFCS), manual ability (MACS), feeding ability (EDACS), and communication function (CFCS). 106 patients were included. Regarding the clinical classification, the most common type of CP was bilateral spastic paralysis, with the highest percentage of functional involvement in each of the evaluated areas, followed by unilateral spastic paralysis, while cases of dystonic CP and other non-classifiable types presented were less frequent. According to the clinical subclassification, spastic diplegia was more frequent, especially affecting manual and communication skills level I compared with hemiplegia, while cases of mixed and unclassifiable quadriplegia were less frequent with greater overall involvement of level I feeding skills. The observed results of CP in the Magalla nes Region and the Chilean Antarctic are similar to studies available in the literature. The complete evaluation and classification of patients with CP enable a better understanding of the pathology for future studies.