Search

Background: Deep brain stimulation (DBS) has been increasingly used in the management of dyskinetic cerebral palsy (DCP). Data on long-term effects and the safety profile are rare., Objectives: We assessed the efficacy and safety of pallidal DBS in pediatric patients with DCP., Methods: The STIM-CP trial was a prospective, single-arm, multicenter study in which patients from the parental trial agreed to be followed-up for up to 36 months. Assessments included motor and non-motor domains., Results: Of the 16 patients included initially, 14 (mean inclusion age 14 years) were assessed. There was a significant change in the (blinded) ratings of the total Dyskinesia Impairment Scale at 36 months. Twelve serious adverse events (possibly) related to treatment were documented., Conclusion: DBS significantly improved dyskinesia, but other outcome parameters did not change significantly. Investigations of larger homogeneous cohorts are needed to further ascertain the impact of DBS and guide treatment decisions in DCP. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society., (© 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

Aim: To create a shortened, more user-friendly Second Edition of the Dyskinesia Impairment Scale (DIS-II) to assess dystonia and choreoathetosis, and evaluate its construct validity and reliability., Method: Scale development included an online expert meeting (n = 21) and iterative discussions within the research group (n = 6). A Rasch measurement model analysis on DIS scores from individuals with dyskinetic cerebral palsy or inherited/idiopathic dystonia (n = 123, 74 males, mean age 14 years, SD 5 years) was performed to evaluate the construct validity and reliability of the DIS-II., Results: The DIS-II evaluates dystonia and choreoathetosis in action and rest in 11 body regions, with action items scored from 0 to 3 and rest items 0 to 2. The number of videos to record are reduced from 26 to 14 and the items to score are reduced from 144 to 88. Rating scale functioning, goodness-of-fit evaluation, principal component analysis, and targeting met the predefined quality criteria of the study and construct validity was therefore considered good. Furthermore, person reliability indicated that the DIS-II can separate individuals into eight distinct ability levels., Interpretation: The DIS-II provides valid and reliable measures for dystonia and choreoathetosis, and reduces the administration and scoring time compared with the DIS. The DIS-II logit scores (interval level data) enhance comparison over time and between individuals in clinical practice and research., What This Paper Adds: Compared with the Dyskinesia Impairment Scale (DIS), the shortened edition (DIS-II) requires half of the number of videos to be scored. The DIS-II has a simplified rating scale, requiring scoring of 88 instead of 144 items. The DIS-II has shown excellent reliability and good construct validity. The interval properties of the DIS-II are superior to the ordinal level outcome measures of the DIS., (© 2022 Mac Keith Press.)

Background -Movement patterns in dyskinetic cerebral palsy (DCP) are characterized by abnormal postures and involuntary movements. Current evaluation tools in DCP are subjective and time-consuming. Sensors could yield objective information on pathological patterns in DCP, but their reliability has not yet been evaluated. The objectives of this study were to evaluate (i) reliability and (ii) discriminative ability of sensor parameters. Methods -Inertial measurement units were placed on the arm, forearm, and hand of individuals with and without DCP while performing reach-forward, reach-and-grasp-vertical, and reach-sideways tasks. Intra-class correlation coefficients (ICC) were calculated for reliability, and Mann-Whitney U-tests for between-group differences. Results -Twenty-two extremities of individuals with DCP (mean age 16.7 y) and twenty individuals without DCP (mean age 17.2 y) were evaluated. ICC values for all sensor parameters except jerk and sample entropy ranged from 0.50 to 0.98 during reach forwards/sideways and from 0.40 to 0.95 during reach-and-grasp vertical. Jerk and maximal acceleration/angular velocity were significantly higher for the DCP group in comparison with peers. Conclusions -This study was the first to assess the reliability of sensor parameters in individuals with DCP, reporting high between- and within-session reliability for the majority of the sensor parameters. These findings suggest that pathological movements of individuals with DCP can be reliably captured using a selection of sensor parameters.

Objectives : To map the presence, severity, and distribution of spasticity and passive range of motion (pROM) deviations in dyskinetic cerebral palsy (DCP), and to explore their relation with dystonia, choreoathetosis, and functional abilities. Methods : This cross-sectional study included 53 participants with DCP. Spasticity was assessed with the Modified Ashworth Scale, limited- and increased pROM (hypermobility) with a goniometer, dystonia and choreoathetosis with the Dyskinesia Impairment Scale, gross motor and manual abilities with corresponding functional classification systems. Results : Spasticity and limited pROM were correlated with dystonia of the upper limbs (0.41< r s <0.47, <0.001 <  p < .002) and lower limbs (0.31< r s <0.41, 0.002 <  p < .025), and both functional systems of gross motor (0.32< r s <0.51, <0.001 <  p < .018) and fine manual abilities (0.34< r s <0.44, 0.001 <  p < .014). Hypermobility is correlated only with choreoathetosis of the lower limbs (0.44, p = .001). Conclusions : Coexisting spasticity and pROM deviations in DCP are functionally limiting and should be addressed accordingly. Hypermobility may lead to an increased luxation risk.

Objectives: To investigate the operational competences screen navigation and dwell function underlying eye gaze performance, and the relation of dystonia and choreoathetosis with eye gaze performance in children with dyskinetic cerebral palsy (DCP)., Methods: During a 5-week intervention, ten participants with DCP played eye gaze video games daily for 30 minutes. Six games were used to assess task performance, fixation count, and eye movement accuracy during four measurements. Dystonia and choreoathetosis were evaluated using the Dyskinesia Impairment Scale., Results: Eye gaze performance improved over time ( p = .013). Moderate to strong within-subject correlations were found between eye movement accuracy and task performance, and between eye movement accuracy and fixation count. No significant correlations were found with the movement disorders., Conclusions: Eye gaze technology shows great potential to be a successful computer interface for children with severe DCP, thereby potentially improving their communication skills, participation levels, and quality of life.

Background: This cross-sectional study aims to investigate the presence and severity of overflow movements of dystonia and choreoathetosis in dyskinetic cerebral palsy (CP) and to assess the relationship of overflow movements with functional classification scales. Methods: Fifty-two subjects with dyskinetic CP were included. Presence and severity of dystonia and choreoathetosis overflow movements were assessed with the Dyskinesia Impairment Scale. Functional abilities were classified with the Gross Motor Function Classification System, Manual Ability Classification System, Viking Speech Scale, Communication Function Classification System, and Eating and Drinking Ability Classification System. Results: Dystonia and choreoathetosis overflow movements were simultaneously present. Median scores of dystonia overflow movements were significantly higher than choreoathetosis overflow movements. Dystonia and choreoathetosis overflow movements were significantly higher in extremities than in the central body. Correlations between dystonia and choreoathetosis overflow movements were fair. Moderate to good correlations were found between dystonia overflow score and Gross Motor Function Classification System, Manual Ability Classification System, and Eating and Drinking Ability Classification System. Conclusions: This is the first study to assess overflow movements in dyskinetic CP. All participants presented with dystonia and choreoathetosis overflow movements, with higher values for dystonia overflow movements. Dystonia overflow movements seem to have a larger impact on functional abilities.Implications for rehabilitationDystonia and choreoathetosis overflow movements are both present in children with dyskinetic cerebral palsy, with dystonia overflow movements being more severe than choreoathetosis overflow movements.Overflow movements impact heavily on daily functional ability and the execution of voluntary activities.Dystonia overflow movements show good correlations with functional classification scales.The measurable characteristics of overflow movements can be used as a guideline for targeted treatment with, e.g., botulinum-toxin-A injections.

Aim: To assess the responsiveness, concurrent validity, and feasibility of the Dyskinesia Impairment Scale (DIS) in non-ambulatory patients with dyskinetic cerebral palsy (CP)., Method: The study is a secondary analysis of data collected in the IDYS trial, a randomized controlled trial on the effects of intrathecal baclofen (ITB). The DIS and Barry-Albright Dystonia Scale (BADS) were conducted at baseline and after 3 months of ITB or placebo treatment. Responsiveness was assessed by comparing the effect sizes and correlation of change after treatment between the DIS and BADS. Concurrent validity was evaluated by assessing the correlations between scales. Feasibility was evaluated for each DIS item by the number of participants who successfully accomplished the item., Results: Thirty-three non-ambulatory patients (9 females, 24 males) with dyskinetic CP (ITB-treated: n=17, mean [SD] age: 14y 1mo [4y 1mo]; placebo-treated: n=16, mean [SD] age: 14y 7mo [4y]) were included in the study. The effect sizes for BADS and DIS were similar in The ITB-treated group (-0.29 and -0.22 respectively). Changes after treatment on the DIS dystonia subscale correlated with changes on the BADS (r=0.64; p<0.001). The DIS dystonia subscale and BADS correlated at baseline and follow-up (r=0.78; p<0.001 and r=0.79; p<0.001). Not all DIS activity items could be performed in this sample of patients., Interpretation: For non-ambulatory patients with dyskinetic CP, the responsiveness of the DIS equalled the responsiveness of BADS. Concurrent validity was adequate. Feasibility for activity items was restricted in patients with severe dyskinetic CP., What This Paper Adds: The Dyskinesia Impairment Scale (DIS) and Barry-Albright Dystonia Scale showed similar responsiveness in non-ambulatory patients with dyskinetic cerebral palsy (CP). No floor or ceiling effect was observed for DIS in non-ambulatory participants. The concurrent validity of DIS was adequate in non-ambulatory participants. Patients with dyskinetic CP in Gross Motor Function Classification System levels IV and V could not perform all DIS activity items., (© 2019 The Authors. Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.)

Aim: To assess test-retest reliability of the Dyskinesia Impairment Scale (DIS) in children and young adults with dyskinetic cerebral palsy (CP)., Method: Dystonia and choreoathetosis were assessed in 15 participants with dyskinetic CP (13 males, 2 females; age range 5-22y, mean 14y, SD 4y) using the DIS in two separate sessions over 7 days. Exclusion criteria were changes in muscle relaxant medication within the previous 3 months, orthopaedic or neurosurgical interventions within the previous year, and spinal fusion. Intraclass correlation coefficient, confidence intervals (CI), standard error of measurement, and the minimal detectable difference (MDD) were determined for test-retest reliability., Result: Intraclass correlation coefficients of the DIS, the dystonia subscale of the DIS, and the choreoathetosis subscale of the DIS were 0.98 (95% CI 0.94-0.99), 0.97 (95% CI 0.92-0.99), and 0.96 (95% CI 0.90-0.99). The standard error of measurement and MDD were 2.6% and 7.2%., Interpretation: The DIS is a reliable tool to assess dystonia and choreoathetosis; it remains stable over time in children and young adults with dyskinetic CP. These results add to the current evidence for good clinimetric properties of the DIS., What This Paper Adds: The Dyskinesia Impairment Scale (DIS) shows stability in scoring dystonia and choreoathetosis. The total DIS score and dystonia and choreoathetosis subscales are clinically useful., (© 2019 Mac Keith Press.)

Cerebral palsy is the most frequent cause of severe physical disability in childhood. Dyskinetic cerebral palsy (DCP) is the second most common type of cerebral palsy after spastic forms. DCP is typically caused by non-progressive lesions to the basal ganglia or thalamus, or both, and is characterised by abnormal postures or movements associated with impaired tone regulation or movement coordination. In DCP, two major movement disorders, dystonia and choreoathetosis, are present together most of the time. Dystonia is often more pronounced and severe than choreoathetosis, with a major effect on daily activity, quality of life, and societal participation. The pathophysiology of both movement disorders is largely unknown. Some emerging hypotheses are an imbalance between indirect and direct basal ganglia pathways, disturbed sensory processing, and impaired plasticity in the basal ganglia. Rehabilitation strategies are typically multidisciplinary. Use of oral drugs to provide symptomatic relief of the movement disorders is limited by adverse effects and the scarcity of evidence that the drugs are effective. Neuromodulation interventions, such as intrathecal baclofen and deep brain stimulation, are promising options., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Aim: This cross-sectional study aimed to map the functional profile of individuals with dyskinetic cerebral palsy (CP), to determine interrelationships between the functional classification systems, and to investigate the relationship of functional abilities with dystonia and choreoathetosis severity., Methods: Fifty-five children (<15y) and young people (15-22y) (30 males, 25 females; mean age 14y 6mo, standard deviation 4y 1mo) with dyskinetic CP were assessed using the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), Communication Function Classification System (CFCS), Eating and Drinking Ability Classification System (EDACS), and Viking Speech Scale (VSS), as well as the Dyskinesia Impairment Scale., Results: Over 50 per cent of the participants exhibited the highest limitation levels in GMFCS, MACS, and VSS. Better functional abilities were seen in EDACS and CFCS. Moderate to excellent interrelationship was found among the classification scales. All scales had significant correlation (r s =0.65 - 0.81) with dystonia severity except for CFCS in the young people group. Finally, only MACS (r s =0.40) and EDACS (r s =0.55) in the young people group demonstrated significant correlation with choreoathetosis severity., Interpretation: The need for inclusion of speech, eating, and drinking in the functional assessment of dyskinetic CP is highlighted. The study further supports the strategy of managing dystonia in particular at a younger age followed by choreoathetosis in a later stage., (© 2017 Mac Keith Press.)

Background: The Dyskinesia Impairment Scale (DIS) is a new scale for measuring dystonia and choreoathetosis in dyskinetic Cerebral Palsy (CP). Previously, reliability of this scale has only been assessed for raters highly experienced in discriminating between dystonia and choreoathetosis., Aims: The aims of this study are to examine the reliability of the DIS used by inexperienced raters, new to discriminating between dystonia and choreoathetosis and to determine the effect of clinical expertise on reliability., Methods: Twenty-five patients (17 males; 8 females; age range 5-22 years; mean age = 13 years 6 months; SD = 5 years 4 months) with dyskinetic CP were filmed with the DIS standard video protocol. Two junior physiotherapists (PTs) and three senior PTs, all of whom were new to discriminating between dystonia and choreoathetosis, were trained in scoring the DIS. Afterward, they independently scored all patients from the video recordings using the DIS. Reliability was assessed by (1) Intraclass Correlation Coefficient (ICC), (2) Standard Error of Measurement (SEM) and Minimal Detectable Difference (MDD) and (3) Cronbach's alpha for internal consistency., Results: Interrater reliability for the total DIS, and for the dystonia and choreoathetosis subscales was good for the junior PTs and moderately high to excellent for the senior PTs. SEM and MDD values for the total DIS were 6% and 15% respectively for the junior PTs and 4% and 12% respectively for the senior PTs. Cronbach's alpha ranged between 0.87 and 0.95 for the junior PTs and between 0.76 and 0.93 for the senior PTs., Conclusions: Reliability of the DIS scores for the inexperienced junior and senior PTs was sufficient in comparison with scores from the experienced raters in the previous study, indicating that the DIS can be used by inexperienced PTs new to discriminating between dystonia and choreoathetosis, and also that its reliability is not dependent on clinical expertise. However, based on the measurement errors and questionnaire data, familiarity with operational definitions of dystonia and choreoathetosis is crucial to improve scoring reliability., (Copyright © 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)