Jules Becher

To examine associations over longitudinal measurements between neuromusculoskeletal function and gross motor capacity in children and youth with cerebral palsy (CP). A prospective cohort study. Rehabilitation departments of university medical centers and rehabilitations centers. A sample (N=327) consisting of 148 children (aged 5-9y) and 179 youth (aged 11-20y) with CP, Gross Motor Function Classification System level I (n=180), level II (n=44), level III (n=36), level IV (n=34), and level V (n=33). Not applicable. Gross motor capacity was assessed with the Gross Motor Function Measure-66 over a period of 2 to 4 years in different age cohorts. Neuromusculoskeletal function included selective motor control (SMC), muscle strength, spasticity, and range of motion (ROM) of the lower extremities. Multilevel analyses showed that SMC was significantly associated with gross motor capacity in children and youth with CP, showing higher values and a more favorable course of gross motor capacity in those with better SMC. Strength was only associated with gross motor capacity in youth. Reduced ROM of hip (children) and knee extension (youth) and spasticity of the hip adductors (youth) were additionally-but more weakly-associated with lower values and a less favorable course of gross motor capacity. Results indicate that children and youth with more severely impaired SMC and youth with reduced muscle strength have a less favorable course of gross motor capacity, while spasticity and reduced ROM are less determinative.

Cerebral palsy is the most common cause of childhood-onset, lifelong physical disability in most countries, affecting about 1 in 500 neonates with an estimated prevalence of 17 million people worldwide. Cerebral palsy is not a disease entity in the traditional sense but a clinical description of children who share features of a non-progressive brain injury or lesion acquired during the antenatal, perinatal or early postnatal period. The clinical manifestations of cerebral palsy vary greatly in the type of movement disorder, the degree of functional ability and limitation and the affected parts of the body. There is currently no cure, but progress is being made in both the prevention and the amelioration of the brain injury. For example, administration of magnesium sulfate during premature labour and cooling of high-risk infants can reduce the rate and severity of cerebral palsy. Although the disorder affects individuals throughout their lifetime, most cerebral palsy research efforts...

Selective dorsal rhizotomy (SDR) surgery is a well-established treatment for ambulatory children with bilateral spastic paresis and is performed to eliminate spasticity and improve walking. The objective of this case report is to describe sudden falls as a persistent complication of SDR. The authors report on 3 patients with bilateral spastic paresis, aged 12, 6, and 7 years at the time of surgery. The percentage of transected dorsal rootlets was around 40% at the L2-S1 levels. Sudden falls were reported with a frequency of several a day, continuing for years after SDR. The falls were often triggered by performing dual tasks as well as occurring in the transition from sitting to standing, during running, after strenuous exercise, or following a fright. Patients also had residual hyperesthesia and dysesthesia of the foot sole. The authors hypothesize that the sudden falls are caused by a muscle inhibition reflex of the muscles in the legs, as an abnormal reaction to a sensory stimulu...

Dynamometry has been used extensively to measure knee extensor strength in individuals with cerebral palsy (CP). However, increased coactivation can lead to underestimation of knee extensor strength and, therefore, reduce validity of strength measurements. It is yet unknown to what extent coactivation occurs during dynamometry testing and whether coactivation is influenced by severity of CP, load levels, and muscle fatigue. The aims of this study were: (1) to investigate coactivation in adolescents with and without CP during dynamometer tests and (2) to assess the effect of Gross Motor Function Classification System (GMFCS) level, load level, and muscle fatigue on coactivation. A cross-sectional observational design was used. Sixteen adolescents with CP (GMFCS levels I and II: n=10/6; age range=13-19 years) and 15 adolescents without CP (n=15; age range=12-19 years) performed maximal isometric contractions (maximal voluntary torque [MVT]) and a series of submaximal dynamic contracti...

Intrathecal baclofen (ITB) treatment is applied in patients with spastic cerebral palsy (SCP), dystonic cerebral palsy (DCP) and progressive neurological disease (PND). Our aim was to investigate whether ITB treatment has a different effect on activities of daily life (ADL) in these groups. A retrospective and cross-sectional survey was conducted using a questionnaire to assess the qualitative effect of ITB (Likert scale) on different domains of functioning (mobility, personal care, communication, comfort) and satisfaction with the results. Groups were compared using non-parametric statistics. Questionnaires were completed for 68 patients (39 SCP, 13 DCP, 16 PND). Satisfaction scores were relatively high in all groups (7-8) and the positive effect on personal care and communication was similar in all groups. The PND group had the shortest follow-up and scored significantly less favourably for the effect on mobility and comfort. This is the first study to show that ITB treatment has similar effects on personal care and communication in stable and progressive neurological disease. The decrease in mobility in the PND group is likely due to the progressive nature of the disease. The different effect on comfort between groups is mainly due to the smaller effect on startles in the PND group.

Rigid Ankle-Foot Orthoses (AFOs) are commonly prescribed to counteract excessive knee flexion during the stance phase of gait in children with cerebral palsy (CP). While rigid AFOs may normalize knee kinematics and kinetics effectively, it has the disadvantage of impeding push-off power. A spring-like AFO may enhance push-off power, which may come at the cost of reducing the knee flexion less effectively. Optimizing this trade-off between enhancing push-off power and normalizing knee flexion in stance is expected to maximize gait efficiency. This study investigated the effects of varying AFO stiffness on gait biomechanics and efficiency in children with CP who walk with excessive knee flexion in stance. Fifteen children with spastic CP (11 boys, 10±2 years) were prescribed with a ventral shell spring-hinged AFO (vAFO). The hinge was set into a rigid, or spring-like setting, using both a stiff and flexible performance. At baseline (i.e. shoes-only) and for each vAFO, a 3D-gait analysis and 6-minute walk test with breath-gas analysis were performed at comfortable speed. Lower limb joint kinematics and kinetics were calculated. From the 6-minute walk test, walking speed and the net energy cost were determined. A generalized estimation equation (p<0.05) was used to analyze the effects of different conditions. Compared to shoes-only, all vAFOs improved the knee angle and net moment similarly. Ankle power generation and work were preserved only by the spring-like vAFOs. All vAFOs decreased the net energy cost compared to shoes-only, but no differences were found between vAFOs, showing that the effects of spring-like vAFOs to promote push-off power did not lead to greater reductions in walking energy cost. These findings suggest that, in this specific group of children with spastic CP, the vAFO stiffness that maximizes gait efficiency is primarily determined by its effect on knee kinematics and kinetics rather than by its effect on push-off power. Dutch Trial Register NTR3418.

ABSTRACT Doel van dit onderzoek is een analyse van de complicaties bij kinderen met intrathecale baclofentherapie. Meer specifiek werd gekeken naar aard, frequentie, impact (d.m.v. verzorgertevredenheid) en mogelijke determinanten van het optreden van complicaties. Hiertoe werd retrospectief statusonderzoek uitgevoerd en een cross-sectionele tevredenheidsvragenlijst afgenomen. Alle kinderen (t/m 17 jaar) behandeld met intrathecale baclofentherapie in het VU medisch centrum zijn geanalyseerd. De minimale follow-up was zes maanden. Het betreft baclofenpompimplantaties tussen maart 2001 en mei 2007. Een complicatiefrequentie van 0,28 (0,19-0,42) complicaties per patiënt per jaar werd gevonden. In de literatuur bedraagt de complicatiefrequentie tussen de 0,09 en 0,58 complicaties per patiënt per jaar. Kathetergerelateerde complicaties kwamen het meest frequent voor. Drie gevonden determinanten voor het optreden van complicaties zijn: kathetertiphoogte, pulmonale comorbiditeit en corticosteroïdengebruik. Het tevredenheidsonderzoek wees uit dat ouders/verzorgers van kinderen met één of meer complicaties niet minder tevreden waren dan ouders van kinderen zonder complicaties. Van de ondervraagden zou 88% de keuze voor intrathecale baclofentherapie nog steeds maken. De resultaten van het tevredenheidsonderzoek bevestigen de hypothese dat ouders/verzorgers in hun oordeel de functionele waarde van de behandeling zwaarder laten wegen dan de eventuele complicaties. Aim of this research is an analysis of the complications in children with intrathecal baclofen therapy. More specific by analysing nature, frequency, impact (by means of caregiver satisfaction) and possible determinants of complications. For that purpose retrospective data research was conducted and a cross-sectional satisfaction questionnaire was administered. All children treated with intrathecal baclofen therapy in the VU medical centre, Amsterdam, are included in the study. Minimum follow-up was six months. The baclofen pumps were implanted from March 2001 to May 2007.We found 0.28 (0.19-0.42) complications per patient per year. In other studies this value varies between 0.09 and 0.58 complications per patient per year. Catheter-related complications were the most common. Three determinants proved to be associated with the rate of complications: the level of the catheter tip, pulmonary co-morbidity and the use of corticosteroids. The results of the satisfaction questionnaire showed that there is no less satisfaction in parents/guardians of children with one or more complications as compared to parents of children without complications. Of parents questioned, 88% would again choose for intrathecal baclofen therapy. The results of the satisfaction questionnaire support the hypothesis that in their judgement parents/guardians focus more on functional gain of the treatment than on possible complications.

In disabling orthopaedic and neurological disorders, in addition to clinical and imaging examination, observation of the motion is an important method of examination. It serves as a starting point for the motion analysis using digital video. In this way, muscle functions can be measured with the aid of a kinesiological EMG. Load on the joints of the lower extremities can be deduced from a projection of the foot reaction vector on the body. Combination of a video film of the motion (the gait) with measurement of the kinesiological EMG provides a motion analysis based on multimedia techniques. With modern personal computers, interactive examination of these digital data is simple. Three examples of this functionality can be viewed at the Internet site of this journal (http:@appendix.niwi.knaw.nl): the normal walking cycle, the active stabilization of the knee after an injury of the anterior cruciate ligament and the gait of a woman with cerebral palsy before and after orthopaedic surgery. Motion analysis using multimedia techniques makes it possible to analyse relationships between muscular and articular functions on the one hand and impaired motion on the other in a way that is meaningful to the clinician.

The purpose of this study was to describe the physical activity level of adolescents with cerebral palsy (CP) and to investigate the associated factors. The physical activity level was measured by means of a questionnaire filled in by the parents of 72 adolescents with CP (12-16 years of age) and expressed in METs. Older age, female gender, and hip dysplasia were significantly associated with a lower level of physical activity. Eighty-nine percent were not physically active enough according to the Dutch norm. Physical activity needs to be promoted, especially among older adolescents with CP (age 14-16 years), girls, and adolescents with hip dysplasia.

To assess spoken language comprehension in non-speaking children with severe cerebral palsy (CP) and to explore possible associations with motor type and disability. Eighty-seven non-speaking children (44 males, 43 females, mean age 6y 8mo, SD 2y 1mo) with spastic (54%) or dyskinetic (46%) CP (Gross Motor Function Classification System [GMFCS] levels IV [39%] and V [61%]) underwent spoken language comprehension assessment with the computer-based instrument for low motor language testing (C-BiLLT), a new and validated diagnostic instrument. A multiple linear regression model was used to investigate which variables explained the variation in C-BiLLT scores. Associations between spoken language comprehension abilities (expressed in z-score or age-equivalent score) and motor type of CP, GMFCS and Manual Ability Classification System (MACS) levels, gestational age, and epilepsy were analysed with Fisher's exact test. A p-value <0.05 was considered statistically significant. Chrono...

The primary aim of this prospective cohort study was to evaluate the short-term (1 year) and long-term (mean 6 years) effects of selective dorsal rhizotomy (SDR) on gross motor function and spasticity in ambulatory children with spastic diplegia. Secondary aims were to investigate side effects, additional treatment during follow-up (botulinum toxin type A injections or orthopedic surgery), and parental satisfaction. Thirty-three children who had undergone SDR at a mean age of 6 years and 7 months (± 2 years) were included. There were 7 children at Gross Motor Function Classification System (GMFCS) Level I, 7 at Level II, and 19 at Level III. Gross motor function was assessed with the Gross Motor Function Measure-66 (GMFM-66). Spasticity was measured according to a modified Tardieu scale. Side effects, additional treatment, and parental satisfaction were recorded using a parental questionnaire and medical records. At 1-year follow-up, mean GMFM-66 scores improved significantly by 4.3...

... Heleen Beckerman, Jules G Becher and Gustaaf J Lankhorst, Department of Rehabilitation Medicine, VU UniversityMedical Center, Amsterdam, The Netherlands 10.1 191/ 0269215505cr872xx Page 2. 346 Letter to the editor References ... 3 Altman DG, Bland JM. ...

The aim of this study is to show the relationship between test-retest reproducibility and responsiveness and to introduce the smallest real difference (SRD) approach, using the sickness impact profile (SIP) in chronic stroke patients as an example. Forty chronic stroke patients were interviewed twice by the same examiner, with a 1-week interval. All patients were interviewed during the qualification period preceding a randomized clinical trial. Test-retest reproducibility has been quantified by the intraclass correlation coefficient (ICC). the standard error of measurement (SEM) and the related smallest real difference (SRD). Responsiveness was defined as the ratio of the clinically relevant change to the SD of the within-stable-subject test-retest differences. The ICC for the total SIP was 0.92, whereas the ICCs for the specified SIP categories varied from 0.63 for the category 'recreation and pastime' to 0.88 for the category 'work'. However, both the SEM and the S...

Based on the results of several electrodiagnostic and biomechanical studies, the following classification of muscle dysfunction in spastic hemiplegia is proposed: changes in muscle activation (excess symptoms, e.g., spasticity, and deficit symptoms, e.g., paresis); changes in muscle stiffness; and changes in muscle length. The clinical significance of this classification is that different types of muscle dysfunction might require specific treatment. The authors have developed techniques to measure quantitatively each type of muscle dysfunction: free frequency repetitive movement (FFRM) and torque angle diagram (TAD). Surface EMGs of tibialis anterior, gastrocnemius, and soleus muscle are recorded during active (FFRM) and passive (TAD) ankle movements. EMG data are converted to parameters for abnormal muscle activation (excess and deficit symptoms). Parameters for muscle stiffness and muscle length are derived from the hysteresis curve of the TAD. This article describes the measureme...

ABSTRACT Cerebrale parese is een verzamelnaam van een groep stationaire neurologische beelden, die zich onder andere uiten in storingen van tonus en beweging. In dit artikel wordt een overzicht gegeven van de diagnostiek en van de hedendaagse conservatieve en operatieve behandelmethoden. Cerebral palsy can be described as a group of stationary neurological abnormalities causing, among other symptoms, abnormalities of muscle tone and movement. This review deals with aspects of diagnosis and current, conservative and operative, treatment options.

The aim of this study was to examine determinants of the course and level of social functioning and communication in school-aged children with cerebral palsy (CP) over a 2-year period. A clinic-based sample of 5 and 7 years old children with CP (n=108; 72 males; mean age 6 y 3 mo, SD 12 mo; Gross Motor Function Classification System (GFMCS) level I-V) was followed longitudinally in three yearly assessments. Social functioning and communication were measured with the Vineland Adaptive Behavior Scales (VABS). Data were analyzed with generalized estimated equations. The results showed that social function followed a course of progressive restrictions over time in non-ambulatory children with CP aged 5-9 compared to children who could walk with or without walking aids. Overall lower levels of social functioning were found in children with GMFCS V, epilepsy, speech problems, lower intellectual capacity and older age at baseline. For communication more restrictions over time were found in children with lower intellectual capacity. Children with GMFCS V, speech problems and older age at baseline had overall greater restrictions in communication. It was concluded that motor functioning and intellectual ability can be used to identify children at risk for progressive restrictions in social functioning and communication. For children with CP and social and communicative restrictions, multidisciplinary assessment and treatment may be indicated to counteract an unfavorable development.

Selective dorsal rhizotomy is an effective treatment for spasticity in children with cerebral palsy who have a spastic motor disorder. It is hypothesized that muscle shortening is related to spasticity; the lack of stretch of a muscle is thought to be the cause of muscle shortening. If this is true, the treatment for spasticity should prevent the occurrence of muscle shortening during growth. We present the case of 1 child with cerebral palsy and spastic diplegia, for whom the treatment with selective dorsal rhizotomy was successful in improving the walking abilities. She did, however, develop muscle shortening during growth. In conclusion, the development of muscle shortening during growth in children with cerebral palsy and spastic paresis cannot be prevented by treatment for the spasticity alone.

Intractable spasticity can be treated effectively with continuous infusion of intrathecal baclofen. Because evidence for its use in the treatment of children with spastic cerebral palsy is lacking, we conducted a randomised controlled trial. To test whether continuous infusion of intrathecal baclofen is effective in comparison with standard treatment only. Seventeen children, aged 13.2 (SD 2.8) years, with intractable spastic cerebral palsy were randomised to receive a Synchromed pump for continuous infusion of intrathecal baclofen after either 1 month (CITB group) or 6 months (Control group). Primary outcomes were the 6-month-change scores on the 0-10 visual analogue scale for individually formulated problems and the caregiver assistance scale of the Pediatric Evaluation of Disability Inventory self-care domain. One of the secondary outcome measures was health related quality of life as measured with the Child Health Questionnaire-PF50. Nine children were randomly assigned to the CITB group and eight to the Control group. The visual analogue scale for individual problems improved with 4.0 (SD 1.7) in the CITB group and changed with -0.2 (SD 1.3) in the Control group (p=0.001). Pediatric Evaluation of Disability Inventory scores did not change significantly. The Child Health Questionnaire-PF50 6-month-change score significantly differed in favour of the CITB group for the domains of bodily pain/discomfort (p=0.014), mental health (p=0.045), psychosocial status (p=0.027) and parents' personal time limitation (p=0.043). The results of this randomised controlled trial establish continuous infusion of intrathecal baclofen to be effective in carefully selected children with problems caused by intractable spastic cerebral palsy.

To chart the 3-year course of health-related quality of life (HRQoL) of 9--13-year-old children with cerebral palsy (CP), and to determine its relationship with gross motor abilities and mental health. Children (n = 91; 58 boys, mean age 11 years, age ranging from 8 years and 6 months to 13 years and 8 months) and parents were assessed annually with the TNO-AZL questionnaires for children's health-related quality of life as a dependent variable, and the gross motor function measure for children with CP and the child behaviour check list as independent variables. The children reported lower HRQoL compared with children in the general population, but reported a higher HRQoL than their parents. The HRQoL remained fairly stable over the 3 years, except for an increase in the autonomy domain. The HRQOL was moderately associated with gross motor abilities, and negatively associated with internalising mental health problems. Externalising problems were only negatively associated with parent-reported HRQoL. Children with CP are more resilient and positive about their HRQoL than their parents think they are. In general, mental health in children with CP appeared to be important in understanding their perceived QoL, in addition to the severity of the CP itself.