The relationship between manual ability, dystonia and choreoathetosis severity and upper limb movement patterns during reaching and grasping in children and young adults with dyskinetic cerebral palsy.

INTRODUCTION: Impaired upper limb movements are a key feature in dyskinetic cerebral palsy (CP). However, information on how specific movement patterns relate to manual ability, performance and underlying movement disorders is lacking. Insight in these associations may contribute to targeted upper limb management in dyskinetic CP. This study aimed to explore associations between deviant upper limb movement patterns and (1) manual ability, (2) severity of dystonia/choreoathetosis, and (3) movement time/trajectory deviation during reaching and grasping. PARTICIPANTS/METHODS: Participants underwent three-dimensional upper limb analysis during reaching forwards (RF), reaching sideways (RS) and reach-and-grasp vertical (RGV) as well as clinical assessment. Canonical correlation and regression analysis with statistical parametric mapping were used to explore associations between clinical/performance parameters and movement patterns (mean and variability). RESULTS: Thirty individuals with dyskinetic CP participated (mean age 16±5 y; 20 girls). Lower manual ability was related to higher variability in wrist flexion/extension during RF and RS early in the reaching cycle (p < 0.05). Higher dystonia severity was associated with higher mean wrist flexion (40-82 % of the reaching cycle; p = 0.004) and higher variability in wrist flexion/extension (31-75 %; p < 0.001) and deviation (2-14 %; p = 0.007/60-73 %; p = 0.006) during RF. Choreoathetosis severity was associated with higher elbow pro/supination variability (12-19 %; p = 0.009) during RGV. Trajectory deviation was associated with wrist and elbow movement variability (p < 0.05). CONCLUSION: Current novel analysis of upper limb movement patterns and respective timings allows to detect joint angles and periods in the movement cycle wherein associations with clinical parameters occur. These associations are not present at each joint level, nor during the full movement cycle. This knowledge should be considered for individualized treatment strategies.

Altered upper limb kinematics in individuals with dyskinetic cerebral palsy in comparison with typically developing peers - A statistical parametric mapping study.

BACKGROUND: Dyskinetic cerebral palsy (DCP) is clinically characterized by involuntary movements and abnormal postures, which can aggravate with activity. While upper limb movement variability is often detected in the clinical picture, it remains unknown how movement patterns of individuals with DCP differ from typically developing (TD) peers. RESEARCH QUESTION: Do individuals with DCP show i) higher time-dependent standard deviations of upper limb joint angles and ii) altered upper limb kinematics in time and/or amplitude during functional upper limb tasks in comparison with TD individuals? METHODS: Three-dimensional upper limb movement patterns were cross-sectionally compared in 50 individuals with and without DCP during three functional tasks: reach forward (RF), reach and grasp vertical (RGV) and reach sideways (RS). Mean and point-wise standard deviations of angular waveform of the upper limb joint angles were compared between groups to evaluate differences in time and/or amplitude using traditional and non-linear registration statistical parametric mapping. RESULTS: Thirty-five extremities from 30 individuals (mean age 17y4m, range 5-25 y; MACS level I(n = 2); II(n = 15); III(n = 16); IV(n = 2)) with DCP and twenty TD individuals (mean age 16y8m, range 8-25 y) were evaluated. The DCP compared to TD group showed higher point-wise standard deviations at the level of all joints, which was time-dependent and varied between tasks. Mean wrist and elbow flexion was higher for the DCP group during RF (0-83 % wrist; 57-100 % elbow), RGV (0-82 % wrist; 12-100 % elbow) and RS (0-43 % wrist; 70-100 % elbow). SIGNIFICANCE: This is the first study exploring the movement patterns of individuals with DCP during reaching using quantitative measures. Analyzing these individual movement patterns by statistical parametric mapping (SPM) allows us to focus on both specific joint or on specific timing during the movement cycle. The individual information that this method yields can guide individual therapy aiming to improve reaching function in different parts of the movement cycle or evaluate intervention effects on upper extremity treatment.

Reliability and Discriminative Validity of Wearable Sensors for the Quantification of Upper Limb Movement Disorders in Individuals with Dyskinetic Cerebral Palsy.

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.

Quantitative assessment of trunk movements in functional reaching in children and adolescents with dyskinetic cerebral palsy.

BACKGROUND: Trunk control and upper limb function are often disturbed in people with dyskinetic cerebral palsy. While trunk control is fundamental in upper limb activities, insights in trunk control in dyskinetic cerebral palsy are missing. This study aimed to determine trunk movement characteristics in individuals with dyskinetic cerebral palsy during reaching. METHODS: Twenty individuals with dyskinetic cerebral palsy (MACS level I-III (16y6m)) and 20 typical developing peers (17y2m) were included. Participants performed three tasks: reach forward, reach sideways, and reach and grasp vertically, using a cross-sectional study design. Movements were analyzed using 3D motion capture and a sensor on the trunk. Trunk range of motion, joint angle at point of task achievement, peak and range of angular velocity and linear acceleration were compared between groups using Mann-Whitney U and independent t-tests. FINDINGS: Participants with dyskinetic cerebral palsy showed higher trunk range of motion in all planes during reach forward and reach and grasp vertically, and in rotation and lateral flexion during reach sideways. During reach and grasp vertically, the joint angle at point of task achievement differed in the transversal plane. Ranges of angular velocity and linear acceleration were higher for all tasks and planes for participants with dyskinetic cerebral palsy, and for peak values in nearly all planes. INTERPRETATION: Current results provide insights in trunk control at population level. This is a first step towards a better and individualized evaluation and treatment for trunk control, being an important factor in improving functional activities for individuals with dyskinetic cerebral palsy.

The Dyskinesia Impairment Scale, Second Edition: Development, construct validity, and reliability.

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.

Psychometric properties of upper limb kinematics during functional tasks in children and adolescents with dyskinetic cerebral palsy.

Dyskinetic cerebral palsy (DCP) is characterised by involuntary movements, and the movement patterns of children with DCP have not been extensively studied during upper limb tasks. The aim of this study is to evaluate psychometric properties of upper limb kinematics in participants with DCP and typically developing (TD) participants. In current repeatability and validity study, forty individuals with typical development (n = 20) and DCP (n = 20) performed a reach forward/sideways and a reach and grasp task during motion analysis on two occasions. Joint angles at point of task achievement (PTA) and spatio-temporal parameters were evaluated within-and between-sessions using intra-class correlation coefficients (ICC) and standard error of measurement (SEM). Independent t-tests/Mann-Whitney-U tests were used to compare parameters between groups. Within-session ICC values ranged from 0.45 to 1.0 for all parameters for both groups. Within-session SEM values ranged from 1.1° to 11.7° for TD participants and from 1.9° to 13.0° for participants with DCP. Eight within-session repetitions resulted in the smallest change in ICC and SEM values for both groups. Within-session variability was higher for participants with DCP in comparison with the TD group for the majority of the joint angles and spatio-temporal parameters. Intrinsic variability over time was small for all angles and spatio-temporal parameters, whereas extrinsic variability was higher for elbow and scapula angles. Between-group differences revealed lower shoulder adduction and higher elbow flexion, pronation and wrist flexion, as well as higher trajectory deviation and a lower maximal velocity for participants with DCP. This is the first study to assess the psychometric properties of upper limb kinematics in children and adolescents with DCP, showing that children with DCP show higher variability during task execution, requiring a minimum of eight repetitions. However, their variable movement pattern can be reliably captured within-and between-sessions, confirming the potential of three-dimensional motion analysis for assessment of rehabilitation interventions in DCP.

Assessment of movement disorders using wearable sensors during upper limb tasks: A scoping review.

Background: Studies aiming to objectively quantify movement disorders during upper limb tasks using wearable sensors have recently increased, but there is a wide variety in described measurement and analyzing methods, hampering standardization of methods in research and clinics. Therefore, the primary objective of this review was to provide an overview of sensor set-up and type, included tasks, sensor features and methods used to quantify movement disorders during upper limb tasks in multiple pathological populations. The secondary objective was to identify the most sensitive sensor features for the detection and quantification of movement disorders on the one hand and to describe the clinical application of the proposed methods on the other hand. Methods: A literature search using Scopus, Web of Science, and PubMed was performed. Articles needed to meet following criteria: 1) participants were adults/children with a neurological disease, 2) (at least) one sensor was placed on the upper limb for evaluation of movement disorders during upper limb tasks, 3) comparisons between: groups with/without movement disorders, sensor features before/after intervention, or sensor features with a clinical scale for assessment of the movement disorder. 4) Outcome measures included sensor features from acceleration/angular velocity signals. Results: A total of 101 articles were included, of which 56 researched Parkinson's Disease. Wrist(s), hand(s) and index finger(s) were the most popular sensor locations. Most frequent tasks were: finger tapping, wrist pro/supination, keeping the arms extended in front of the body and finger-to-nose. Most frequently calculated sensor features were mean, standard deviation, root-mean-square, ranges, skewness, kurtosis/entropy of acceleration and/or angular velocity, in combination with dominant frequencies/power of acceleration signals. Examples of clinical applications were automatization of a clinical scale or discrimination between a patient/control group or different patient groups. Conclusion: Current overview can support clinicians and researchers in selecting the most sensitive pathology-dependent sensor features and methodologies for detection and quantification of upper limb movement disorders and objective evaluations of treatment effects. Insights from Parkinson's Disease studies can accelerate the development of wearable sensors protocols in the remaining pathologies, provided that there is sufficient attention for the standardisation of protocols, tasks, feasibility and data analysis methods.

Clinical Presentation of Spasticity and Passive Range of Motion Deviations in Dyskinetic Cerebral Palsy in Relation to Dystonia, Choreoathetosis, and Functional Classification Systems.

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< rs<0.47, <0.001 < p < .002) and lower limbs (0.31< rs<0.41, 0.002 < p < .025), and both functional systems of gross motor (0.32< rs<0.51, <0.001 < p < .018) and fine manual abilities (0.34< rs<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.

Reliability and Validity of the Dyskinesia Impairment Scale in Children and Young Adults with Inherited or Idiopathic Dystonia.

BACKGROUND: The Dyskinesia Impairment Scale (DIS) is a new assessment scale for dystonia and choreoathetosis in children and youth with dyskinetic cerebral palsy. Today, the Burke-Fahn-Marsden Dystonia Rating Scale (BFM) is mostly used to assess dystonia in children with inherited dystonia. The aim of this study was to assess reliability and validity of the DIS in children and youth with inherited or idiopathic dystonia. METHODS: Reliability was measured by (1) the intraclass correlation coefficients (ICCs) for inter-rater and test-retest reliability, as well as (2) standard error of measurement (SEM) and minimal detectable difference (MDD). For concurrent validity of the DIS-dystonia subscale, the BFM was administered. RESULTS: In total, 11 males and 9 females (median age 16 years and 7 months, range 6 to 24 years) were included. For inter-rater reliability, the ICCs for the DIS total score and the dystonia and choreoathetosis subscale scores were 0.83, 0.87, and 0.71, respectively. For test-retest reliability, the ICCs for the DIS total score and the dystonia and choreoathetosis subscale scores were 0.95, 0.88, and 0.93, respectively. The SEM and MDD for the total DIS were 3.98% and 11.04%, respectively. The Spearman correlation coefficient between the dystonia subscale and the BFM was 0.88 (p < 0.01). CONCLUSIONS: Good to excellent inter-rater, test-retest reliability, and validity were found for the total DIS and the dystonia subscale. The choreoathetosis subscale showed moderate inter-rater reliability and excellent test-retest reliability. The DIS may be a promising tool to assess dystonia and choreoathetosis in children and young adults with inherited or idiopathic dystonia.

Eye Gaze Gaming Intervention in Children with Dyskinetic Cerebral Palsy: A Pilot Study of Task Performance and Its Relation with Dystonia and Choreoathetosis.

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.

Presence and severity of dystonia and choreoathetosis overflow movements in participants with dyskinetic cerebral palsy and their relation with functional classification scales.

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.

Test-retest reliability of the Dyskinesia Impairment Scale: measuring dystonia and choreoathetosis in dyskinetic cerebral palsy.

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.

Lower limb muscle volume estimation from maximum cross-sectional area and muscle length in cerebral palsy and typically developing individuals.

BACKGROUND: Deficits in muscle volume may be a significant contributor to physical disability in young people with cerebral palsy. However, 3D measurements of muscle volume using MRI or 3D ultrasound may be difficult to make routinely in the clinic. We wished to establish whether accurate estimates of muscle volume could be made from a combination of anatomical cross-sectional area and length measurements in samples of typically developing young people and young people with bilateral cerebral palsy. METHODS: Lower limb MRI scans were obtained from the lower limbs of 21 individuals with cerebral palsy (14.7±3years, 17 male) and 23 typically developing individuals (16.8±3.3years, 16 male). The volume, length and anatomical cross-sectional area were estimated from six muscles of the left lower limb. FINDINGS: Analysis of Covariance demonstrated that the relationship between the length*cross-sectional area and volume was not significantly different depending on the subject group. Linear regression analysis demonstrated that the product of anatomical cross-sectional area and length bore a strong and significant relationship to the measured muscle volume (R2 values between 0.955 and 0.988) with low standard error of the estimates of 4.8 to 8.9%. INTERPRETATION: This study demonstrates that muscle volume may be estimated accurately in typically developing individuals and individuals with cerebral palsy by a combination of anatomical cross-sectional area and muscle length. 2D ultrasound may be a convenient method of making these measurements routinely in the clinic.