Signs of perceptual disorder during movement were reliably assessed in children with cerebral palsy in Sweden.

AIM: The aim of this Swedish study was to evaluate the assessment of clinical signs of perceptual disorder in children with cerebral palsy (CP). METHODS: Three experienced raters assessed 56 videos of 19 children from 1 to 18 years of age with bilateral spastic CP, which were recorded by colleagues at an Italian hospital. Six signs were evaluated for inter-rater reliability and criterion validity. Clinical applicability was evaluated by assessing inter-rater reliability between 47 Swedish clinicians, who examined 15 of the videos during face-to-face and online education seminars. There were 41 physiotherapists, two occupational therapists and four doctors, with 1-37 years of clinical experience and a median of 10 years. RESULTS: The experienced raters demonstrated moderate to almost perfect inter-rater reliability (kappa 0.54-0.81) and criterion validity (0.54-0.87) for startle reaction, upper limbs in startle position, averted eye gaze and eye blinking. The clinicians recognised these signs with at least moderate reliability (0.56-0.88). Grimacing and posture freezing were less reliable (0.22-0.35) and valid (0.09-0.50). CONCLUSION: Four of the six signs of perceptual disorder were reliably recognised by experienced raters and by clinicians after education seminars. Extended education and larger study samples are needed to recognise all the signs.

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.

RaceRunning training improves stamina and promotes skeletal muscle hypertrophy in young individuals with cerebral palsy.

BACKGROUND: Individuals with cerebral palsy (CP) are less physically active, spend more time sedentary and have lower cardiorespiratory endurance as compared to typically developed individuals. RaceRunning enables high-intensity exercise in individuals with CP with limited or no walking ability, using a three-wheeled running bike with a saddle and a chest plate for support, but no pedals. Training adaptations using this type of exercise are unknown. METHODS: Fifteen adolescents/young adults (mean age 16, range 9-29, 7 females/8 males) with CP completed 12 weeks, two sessions/week, of RaceRunning training. Measurements of cardiorespiratory endurance (6-min RaceRunning test (6-MRT), average and maximum heart rate, rate of perceived exertion using the Borg scale (Borg-RPE)), skeletal muscle thickness (ultrasound) of the thigh (vastus lateralis and intermedius muscles) and lower leg (medial gastrocnemius muscle) and passive range of motion (pROM) of hip, knee and ankle were collected before and after the training period. RESULTS: Cardiorespiratory endurance increased on average 34% (6-MRT distance; pre 576 ± 320 m vs. post 723 ± 368 m, p < 0.001). Average and maximum heart rate and Borg-RPE during the 6-MRT did not differ pre vs. post training. Thickness of the medial gastrocnemius muscle increased 9% in response to training (p < 0.05) on the more-affected side. Passive hip flexion increased (p < 0.05) on the less-affected side and ankle dorsiflexion decreased (p < 0.05) on the more affected side after 12 weeks of RaceRunning training. CONCLUSIONS: These results support the efficacy of RaceRunning as a powerful and effective training modality in individuals with CP, promoting both cardiorespiratory and peripheral adaptations.

The role of visual stimuli on standing posture in children with bilateral cerebral palsy.

BACKGROUND: In children with bilateral cerebral palsy (CP) maintaining a standing position can be difficult. The fundamental motor task of standing independently is achieved by an interaction between the visual, somatosensory, and vestibular systems. In CP, the motor disorders are commonly accompanied by sensory and perceptual disturbances. Our aims were to examine the influence of visual stimuli on standing posture in relation to standing ability. METHODS: Three dimensional motion analysis with surface electromyography was recorded to describe body position, body movement, and muscle activity during three standing tasks: in a self-selected position, while blindfolded, and during an attention-demanding task. Participants were twenty-seven typically-developing (TD) children and 36 children with bilateral CP, of which 17 required support for standing (CP-SwS) and 19 stood without support (CP-SwoS). RESULTS: All children with CP stood with a more flexed body position than the TD children, even more pronounced in the children in CP-SwS. While blindfolded, the CP-SwS group further flexed their hips and knees, and increased muscle activity in knee extensors. In contrast, the children in CP-SwoS maintained the same body position but increased calf muscle activity. During the attention-demanding task, the children in CP-SwoS stood with more still head and knee positions and with less muscle activity. CONCLUSIONS: Visual input was important for children with CP to maintain a standing position. Without visual input the children who required support dropped into a further crouched position. The somatosensory and vestibular systems alone could not provide enough information about the body position in space without visual cues as a reference frame. In the children who stood without support, an intensified visual stimulus enhanced the ability to maintain a quiet standing position. It may be that impairments in the sensory systems are major contributors to the difficulties to stand erect in children with CP.

Influence of External Visual Focus on Gait in Children With Bilateral Cerebral Palsy.

PURPOSE: To explore whether focusing a target influenced gait in children with cerebral palsy (CP) and typical development (TD). METHODS: Thirty children with bilateral CP (Gross Motor Function Classification System [GMFCS] I-III) and 22 with TD looked at a light at walkway end (Gaze Target) while walking and returned (No Target). RESULTS: During Gaze versus No Target, children with TD reduced temporal-spatial parameters and movements in the sagittal (SPM) and transverse planes. In comparison, during Gaze Target, children in CP1 (GMFCS I) had larger trunk SPM, children in CP2 (GMFCS II) larger neck (SPM), and children in CP3 (GMFCS III) greater head and neck frontal plane movements, and reduced cadence and single support. CONCLUSIONS: Focusing a target altered gait in children with CP. Children in CP1 reduced movements similar to children with TD, children in CP2 behaved nearly unchanged, whereas children in CP3 reduced movements and temporal-spatial parameters, potentially as a consequence of lack of sensory information from lower limbs.

Muscle strength does not explain standing ability in children with bilateral spastic cerebral palsy: a cross sectional descriptive study.

BACKGROUND: In bilateral cerebral palsy (CP) muscle strength is considered important for development of gross motor functions, but its influence on standing ability has not been explored. Our aims were to examine muscle strength with respect to the ability to stand with (SwS) or without (SwoS) hand support, asymmetrical weight bearing (WB), and whether the ability to produce strength was influenced by different seated conditions. METHODS: In this cross sectional descriptive study standing posture was recorded with 3D motion analysis, and muscle strength was measured with a hand-held dynamometer, in 25 children with bilateral CP, GMFCS levels II-III, SwS (n = 14, median age 11.4 years), or SwoS, (n = 11, median age 11.4 years). Strength measurements were taken in the hip flexors, knee extensors, dorsiflexors and plantarflexors, in two seated conditions; a chair with arm- and backrests, and a stool. RESULTS: Compared to SwoS, children SwS stood with a more flexed posture, but presented with equal strength in the hip flexors, dorsiflexors and plantarflexors, and with somewhat more strength in the knee extensors. Despite asymmetric WB during standing, both limbs were equally strong in the two groups. No differences in strength were measured between the two seated conditions. CONCLUSIONS: Despite challenges measuring muscle strength in CP, the lower limb muscle strength cannot be considered an explanatory factor for variations in standing in this group of children with bilateral CP. The findings rather strengthen our hypothesis that deficits in the sensory systems could be as determinant for standing as muscle weakness in children with bilateral spastic CP.

Postural orientation during standing in children with bilateral cerebral palsy.

PURPOSE: To investigate postural orientation and maintenance of joint position during standing in children with bilateral spastic cerebral palsy (BSCP). METHODS: Standing was examined with 3-D motion analysis in 26 children with BSCP, and 19 children typically developing (TD). Two groups of children with cerebral palsy (CP) were analyzed: 15 who were able to maintain standing without support and 11 who needed support. RESULTS: Children with CP stood with more flexion than children TD. In the CP groups, children standing without support stood more asymmetrically with less hip and knee flexion and less movement than those who required support. CONCLUSION: Children with CP had varying abilities to stand and maintain standing posture with or without support. Both CP groups stood with more flexion than their potential passive joint angle, more obvious in children requiring support. Investigations on how muscle strength and spatial perception influence posture remains to be explored.

Determinants of Frame Running Capacity in Athletes With Cerebral Palsy to Improve Training Routines and Classification Strategies: A Cross-sectional Observational Study.

OBJECTIVES: The aim of the study were to (1) investigate what physical and physiological parameters are most important for Frame Running capacity, a parasport for individuals with ambulatory difficulties, and (2) determine whether Frame Running capacity can be predicted in athletes with cerebral palsy. DESIGN: Athletes with cerebral palsy ( N = 62, Gross Motor Classification System I-V; 2/26/11/21/2) completed a 6-min Frame Running test. Before the 6-min Frame Running test, muscle thickness, passive range of motion (hip, knee, ankle), selective motor control, and spasticity (hip, knee, ankle) were measured in both legs. In total, 54 variables per individual were included. Data were analyzed using correlations, principal component analysis, orthogonal partial least square regression, and variable importance in projection analysis. RESULTS: The mean 6-min Frame Running test distance was 789 ± 335 m and decreased with motor function severity. The orthogonal partial least square analysis revealed a modest degree of covariance in the variables analyzed and that the variance in the 6-min Frame Running test distance could be predicted with 75% accuracy based on all the variables measured. Variable importance in projection analysis indicated hip and knee extensor spasticity (negative effect), and muscle thickness (positive effect) arose as the most important factors contributing to Frame Running capacity. CONCLUSIONS: These results are an important resource to enable optimization of training regimes to improve Frame Running capacity and contribute to evidence-based and fair classification for this parasport.

Knee Flexion While Walking Exceeds Knee Flexion Contracture in Children with Spastic Cerebral Palsy.

Flexed knee gait is commonly related to contractures in children with cerebral palsy (CP). Therefore, knee position while walking was compared with passive knee extension and explored with respect to functional mobility. Gait was assessed with 3D motion analysis in 30 children with bilateral spastic CP, Gross Motor Function Classification System (GMFCS) levels I-III, and in 22 typically developing (TD) children. Knee angle at initial contact (KneeAngleIC) was greater than knee flexion in stance (MinKneeFlexSt) in all groups. MinKneeFlexSt exceeded knee contractures at GMFCS levels II and III. Both KneeAngleIC and MinKneeFlexSt were greater at GMFCS II and III than at GMFCS I and the TD group. The excessive knee flexion while walking at GMFCS II and III could not be explained by knee joint contractures. Functional mobility measured with the timed-up-and-go test took longer in children at GMFCS level III compared to the other groups, assumed to be explained by the energy-requiring flexed knee gait and spatial insecurity. Discriminating between passive knee extension at the physical assessment and maximum knee extension while weight bearing may contribute to further understanding of flexed knee gait and its causes in ambulating children with spastic bilateral CP.

Responses to Sensory Events in Daily Life in Children with Cerebral Palsy from a Parent Reported Perspective and in a Swedish Context.

The motor disorders of cerebral palsy (CP) are often accompanied by sensory disturbances, but knowledge of their relationship to motor functioning is sparse. This study explored responses to sensory events in relation to spastic subtype and motor functioning in children with CP. Parents of 60 children with CP (unilateral: 18, bilateral: 42) with GMFCS levels I:29, II:13, III:15 and IV:3 of mean age 12.3 years (3.7 SD) participated. The parents (n = 55) rated their children´s responses with the norm-referenced questionnaire Child Sensory Profile-2© (CSP-2©), Swedish version, incorporating nine sections and four sensory processing patterns/quadrants, and replied (n = 57) to two additional questions. On the CSP-2©, thirty (55%) of the children were reported to have responses "much more than others" (>2 SD) in one or more of the sections and/or quadrants and 22 (40%) in the section of Body Position, overrepresented by the children with bilateral CP. The additional questions revealed that a greater proportion of children at GMFCS levels III-IV compared to level I frequently were requested to sit/stand up straight (14/17 versus 6/26, p < 0.001) and were sound sensitive at a younger age (14/17 versus 10/26, p = 0.005). The findings of this study highlight the sensory aspects of motor functioning in children with spastic CP.

Evaluation of Knee Position Sense in Children with Motor Disabilities and Children with Typical Development: A Cross-Sectional Study.

BACKGROUND: In children with motor disabilities, knee position during walking is often of concern in rehabilitation. This study aimed to investigate knee joint position sense. Thirty-seven children with Cerebral Palsy (CP), 21 with Myelomeningocele (MMC), 19 with Arthrogryposis (AMC), and 42 TD children participated in the study. Knee joint position sense, i.e., the difference between the criterion angle and the reproduced angle (JPS-error), was assessed in sitting while 3D motion capture was recorded at flexed knee 70 (Knee70), 45 (Knee45), and 20 (Knee20) degrees, and after three seconds at maintained criterion angle (CAM) and maintained reproduced angle (RAM). No differences were found between the groups in JPS-error, CAM, and RAM. At Knee70, CAM differed between the right and left legs in the TD group (p = 0.014) and RAM in the MMC group (p = 0.021). In the CP group, CAM was greater than RAM at Knee70 in the left leg (p = 0.002), at Knee45 in both legs (p = 0.004, p = 0.025), and at Knee20 in the right leg (p = 0.038). Difficulties in maintaining the knee position at CAM in the CP group sheds light on the need for complementary judgments of limb proprioception in space to explore the potential influence on knee position during walking.

Motor Development in Children with Cerebral Palsy in Sweden-A Population-Based Longitudinal Register Study.

The aim was to explore longitudinal motor development in children with cerebral palsy (CP) in Sweden with respect to the Gross Motor Function Classification System (GMFCS). In this national CP registry-based study, 2138 children aged 0.5-19 years participated (42% girls). The distribution with respect to GMFCS was I: 49%, II: 16%, III: 10%, IV: 14%, and V: 11%. In total, 5538 assessments (mean 2.7, min-max: 1-9) with the Gross Motor Function Measure-66 were included. Data were analysed using non-linear mixed-effects regression models, and the Stable Limit Model was selected to fit data. Five distinct curves of predicted gross motor development with respect to GMFCS levels were obtained. The achieved motor development was maintained over time. The estimated average GMFM-66 limit and the average age when 90% of the expected limits were reached were at GMFCS I: 88 at age 4.5; GMFCS II: 71 at age 4.2; GMFCS III: 54 at age 3.1; GMFCS IV: 38 at age 2.6, and at GMFCS V: 18 at age 0.9. In conclusion, this is the first national population-based study following motor development in CP. Five distinct curves reported in previous controlled research studies were confirmed. Our study adds knowledge about motor development captured in children's everyday context.

Dystonia during hand activity in children with spastic unilateral cerebral palsy, an observational study.

BACKGROUND: Spasticity and dyskinesia are motor signs that co-exist in cerebral palsy (CP). It is well accepted that, in spastic bilateral CP, dystonia can be present in addition to spasticity, and equally that spasticity is often present in individuals with dyskinetic CP. In unilateral spastic CP, dystonia of the upper extremity is only rarely identified or addressed. The aim of this study was to investigate if dystonia was present in the hand of children with unilateral spastic CP, and, if present, to what extent, and when it was first noticeable. METHOD: Ninety-seven children with unilateral spastic CP, born 1999-2014, with standardized digital films of hand function from Assisting Hand Assessments (AHA), were included. Films were reviewed, and presence or absence, of dystonia and choreoathetosis were scored by three experienced raters. RESULTS: Dystonia in the hand was present during activities in 70% (68/97) of the children at a mean age of 12 years (SD 4,4). In 74% (50/68) of these children, dystonia was present more than 50% of the evaluated time. For 63% (43/68) more than one digital recording at younger ages were available. Dystonia could first clearly be observed at a mean age of 3,8 years. Choreoathetosis was observed in 7% (5/68) of the children with dystonia. Children without dystonia had significantly higher (corresponding to better function) AHA units (median: 75, 25th - 75th: 45-82) in comparison to children with dystonia (median: 57, 25th - 75th: 52-63) (p = 0.01). CONCLUSION: Dystonia in the hand is common in unilateral CP and correlates to lower hand functioning.

Motor function at increasing postural demands in children with bilateral cerebral palsy.

BACKGROUND: Among children with cerebral palsy (CP) some choose to be mobile by crawling or walking on their knees despite some bipedal walking ability. This motor behavior raises questions; so, we wanted to enhance understanding of the child's choice of mobility. AIM: To explore gross motor abilities in positions with various postural demands focusing on floor mobility among children with CP. DESIGN: A cross-sectional observational study. SETTING: The study was performed at the Neuropediatric Outpatient Department at Karolinska University Hospital. POPULATION: Thirty-six children with bilateral CP, median age 11.2 years, functioning at Gross Motor Function Classification System (GMFCS) level I-IV. METHODS: Motor skills using the Gross Motor Function Measure (GMFM-88), and lower leg muscles strength in hip flexors, knee extensors, ankle dorsiflexors and plantarflexors with a hand-held dynamometer were assessed. A Kruskal-Wallis Test with post-hoc Bonferroni corrections were used to compare GMFM percentage (%) scores and muscle strength between the GMFCS levels. RESULTS: GMFM-88 (%) scores in walking (E) and standing (D) dimensions were significantly higher at GMFCS levels I and II, compared to levels III and IV. In crawling and kneeling (C) level I achieved higher score than levels III and IV, and in sitting (B) than level IV. Muscle strength values in the plantarflexors were significantly higher at GMFCS level I compared to level II. CONCLUSIONS: As expected the children at GMFCS III performed less than those at GMFCS II at high postural demands in GMFM dimensions including standing and walking. Identical GMFM-scores in dimension C confirm similar motor function in items including kneeling and knee walking. Since lower limb muscle strength was similar, the difference in postural behavior between the groups at high level motor activities may be associated with sensorimotor disturbances along with the children's motor disorder. CLINICAL REHABILITATION IMPACT: The GMFM-88, in particular dimension C, including kneeling and walking items can be used as an identification of preference of floor mobility in children with CP. Awareness and understanding of how postural positions affect movement is of importance for prognosis, and physiotherapy.

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.

Head and Trunk Movements During Turning Gait in Children with Cerebral Palsy.

Thirty children with cerebral palsy (CP) and 22 typical developing (TD) were tested with 3D-gait analysis. At turning, trunk rotation was larger in CP2 (GMFCS II) than in TD and CP1 (GMFCS I), and head flexion was larger in CP3 (GMFCS III) than TD. Maximum head and trunk flexion values during the entire trial were larger in CP3 than in the other groups, and trunk flexion was larger in CP2 than in TD. Trial time increased with GMFCS-level. Less trunk rotation than TD and CP1 reflects spatial insecurity in CP2, which in CP3 is compensated by the walker. The flexed head and trunk in CP3 and trunk in CP2 may reflect deficits in proprioception and sensation requiring visual control of the lower limbs.

Influence of heel lifts during standing in children with motor disorders.

Heel wedges may influence standing posture but how and to what extent are unknown. Thirty-two children with motor disorders - 16 with arthrogryposis multiplex congenita (AMC) and 16 with cerebral palsy (CP) - and 19 control children underwent a three-dimensional motion analysis. Unassisted standing during 20s with shoes only and with heel lifts of 10, 20 and 30mm heights was recorded in a randomized order. The more weight-bearing limb or the right limb was chosen for analysis. In both the AMC and CP groups, significant changes were seen between various heel lifts in ankle, knee and pelvis, and in the control group in the ankle only. Between orthosis and non-orthosis users significant differences were seen between different heel lift conditions in ankle, knee and trunk in the AMC group and in the ankle in the CP group. Pelvis position changed toward less anterior tilt with increasing heel height, but led to increasing knee flexion in most of the children, except for the AMC Non-Ort group. Children with AMC and CP represent different motor disorders, but the heel wedges had a similar influence on pelvis, hip and knee positions in all children with CP and in the AMC orthosis users. A challenge is to apply heel heights adequate to each individual's orthopaedic and neurologic conditions to improve biomechanical alignment with respect to all body segments.