Upper-extremity spasticity and functionality after selective dorsal rhizotomy for cerebral palsy: a systematic review.

OBJECTIVE: Lumbosacral selective dorsal rhizotomy is a neurosurgical treatment option to reduce spasticity in the lower extremities in children with cerebral palsy. Surprisingly, concomitant improvement of spasticity in the upper extremities and functionality of the hands has been sporadically reported postoperatively. In this systematic review, the authors aimed to quantify the postoperative improvement in upper-extremity spasticity and functionality, identify predictors, and discuss underlying mechanisms. METHODS: The authors searched the MEDLINE and Embase databases for studies reporting upper-extremity outcomes in cerebral palsy patients after selective dorsal rhizotomy that reported one or more of the following clinical scales: the Ashworth Scale (AS), the Modified AS (MAS), the fine motor skills domain of the Peabody Developmental Motor Scales (PDMS), the Quality of Upper Extremity Skills Test (QUEST), the self-care domain of the Functional Independence Measure for Children (WeeFIM), or the self-care domain of the Pediatric Evaluation of Disability Inventory (PEDI). The authors arbitrarily divided postoperative follow-up into short-term (< 6 months), medium-term (6-24 months), and long-term (> 24 months) follow-up. A 1-point change in MAS score has been reported as clinically significant. To assess bias, the Cochrane Collaboration's tool and ROBINS-I tool were used. RESULTS: The authors included 24 articles describing 752 patients. Spasticity reduction of the upper extremities ranged from 0.30 to 0.55 (AS) and between 0 and 2.9 (MAS) at medium-term follow-up. This large variability may partially be attributed to a floor effect since patients with normal upper-extremity function would not be expected to have further improvement. QUEST improvement ranged from 2.7% to 4.5% at medium-term follow-up. The mean improvements in functional skills of the self-care domain of the PEDI were 4.3 at short-term and 7 at medium-term follow-ups and ranged from 10.8 to 34.7 at long-term follow-up. There are insufficient data to draw meaningful conclusions regarding the PDMS fine motor skills and the WeeFIM self-care domains. CONCLUSIONS: The literature suggests that a pronounced postoperative spasticity reduction in the lower extremities and a moderately severe preoperative upper-extremity spasticity may positively predict postoperative reduction in upper-extremity spasticity. There are at least 5 hypotheses that may explain the postoperative reduction in upper-extremity spasticity and functionality: 1) a somatosensory cortex reorganization favoring the hand region over the leg region, 2) a decrease in abnormal electrical transmission throughout the spinal cord, 3) an indirect result of improved posture due to improved truncal and leg stability, 4) an indirect consequence of occupational/physical therapy intensification, and 5) a maturation effect. However, all remain unproven to date.

Tone Reduction and Physical Therapy: Strengthening Partners in Treatment of Children with Spastic Cerebral Palsy.

The aim of this paper is to provide a clinically applicable overview of different tone reducing modalities and how these can interact with or augment concurrent physical therapy (PT). Botulinum toxin (BoNT), oral tone-regulating medication, intrathecal baclofen (ITB), and selective dorsal rhizotomy are discussed within a physiotherapeutic context and in view of current scientific evidence. We propose clinical reasoning strategies to identify treatment goals as well as the appropriate and corresponding treatment interventions. Instrumented measurement of spasticity, standardized clinical assessment, and 3D clinical motion analysis are scientifically sound tools to help select the appropriate treatment and, when needed, to selectively target or spare individual muscles. In addition, particular attention is given to strength training as a necessary tool to tackle muscle weakness associated with specific modalities of tone reduction. More research is needed to methodologically assess the long-term effectiveness of such individualized tone treatment, optimize parameters such as medication dosage, and gain more insight into the kind of PT techniques that are essential in conjunction with tone reduction.

Botulinum toxin type A treatment in children with cerebral palsy: evaluation of treatment success or failure by means of goal attainment scaling.

BACKGROUND: There is considerable variability in the amount of response to BTX-A treatment between and within patients with cerebral palsy (CP). AIMS: The purpose of this retrospective cohort study was to evaluate the clinical responsiveness of Botulinum toxin type A (BTX-A) treatment in children with CP and specifically delineate features of treatment success and failure. METHODS: Four hundred and thirty-eight children (251 boys, 187 girls; mean age 8 years 2 months, SD 4 years) were included into the study. Goal Attainment Scaling (GAS) was used to classify and evaluate treatment efficacy. Two study groups were defined: one group with an excellent response (GAS≥60.0) and one group with a lack of response (GAS≤40.0) to BTX-A. RESULTS: Seventy-five patients (17.1%) had an excellent response and treatment was found to be unsuccessful for 31 patients (7.1%). Children with a lack of response to BTX-A were significantly older compared to children with a high responsiveness (p=0.0013). In the latter group, more children received multi-level injections and fewer children had injections in proximal parts of the lower limb compared to the low responsiveness group (p=0.0024). Moreover, there was a significant difference in the use of different types of casts between both study groups (p=0.0263). CONCLUSION: Age, level of treatment and casting seem to be crucial features of BTX-A treatment success or failure in children with CP.

Consensus on the appropriate use of intrathecal baclofen (ITB) therapy in paediatric spasticity.

Among features of motor disorders in children, spasticity is associated with considerable morbidity and problems in care, particularly in severely affected patients. Intrathecal baclofen (ITB) has been increasingly used as a relatively specific treatment modality for spasticity. To date, most of the evidence for its use in paediatric patients has come from retrospective and uncontrolled studies, although randomised, controlled trials of screening ITB and ITB therapy itself have recently been published. This consensus statement on the use of ITB in paediatric patients with spasticity was developed on the basis of currently available evidence, with the aim of providing information for clinicians, promoting an expert opinion and a consistent approach to the management of these patients and emphasising the need for further prospective, large-scale studies.

Long-term use of botulinum toxin type A in children with cerebral palsy: treatment consistency.

At the University Hospital of Pellenberg (Belgium), more than 1000 patients have been treated with Botulinum toxin type A (BTX-A) over the last decade. Ten percent of these patients (n=106) received multiple (at least four times), multi-level, high-dosage treatments. The aim of this study was to evaluate the stability of dosage and treatment intervals in long-term, multi-level, high-dosage treated children with cerebral palsy and to evaluate the evidence for a safe and stable response to this treatment. Data on disease, age, dosage and target muscles were extracted for each treatment session of 106 patients who received multiple BTX-A treatment sessions. Patients had a follow-up of 4y 6mo (range 1y 8mo-8y 9mo) on average and received 4 to 12 BTX-A treatments within the period of January 1996 and December 2005. Patients received a mean dosage of 23.5+/-5.2U/kgbw at first treatment with stable subsequent values. Mean dosages for children with diplegia, hemiplegia and quadriplegia were 24.5+/-4.7U/kgbw, 15.9+/-3.7U/kgbw and 22.0+/-4.8U/kgbw, respectively. Mean age at first treatment was 4y 6mo (range 1y 11mo-18y 10mo) with a majority of patients (76.4%) first treated within 2 and 4y of age. Treatment intervals of approximately 1y remained stable within four, five and six subsequent treatments. Long-term, high-dosage, multi-level BTX-A applications can be considered as a safe and stable treatment option for children with cerebral palsy and the formation of antibodies, responsible for secondary non-response, can be indirectly precluded.

Motor function following multilevel botulinum toxin type A treatment in children with cerebral palsy.

This study evaluated the effects of multilevel botulinum toxin type A (BTX-A) treatments on the gait pattern of children with spastic cerebral palsy (Gross Motor Function Classification System Levels I-III). In this nested case-control design, 30 children (mean age 6y 11mo [SD 1y 5mo]; 21 males, nine females; 19 with hemiplegia, 11 with diplegia) were treated according to best practice guidelines in paediatric orthopaedics, including BTX-A injections. A matched control group of 30 children (mean age 7y 8mo [SD 1y 10mo]; 13 males, 17 females; 19 with hemiplegia, 11 with diplegia) were treated identically, but without BTX-A. Motor development status at 5 to 10 years of age was assessed by means of three-dimensional gait analysis at a mean time of 1 year 10 months (SD 10mo) after the last BTX-A treatment. The control group showed a significantly more pronounced pathological gait pattern than the BTX-A group. Major differences were found for pelvic anterior tilt, maximum hip and knee extension, and internal hip rotation. These results provide evidence for a prolonged effect of BTX-A and suggest that BTX-A injections, in combination with common conservative treatment options, result in a gait pattern that is less defined by secondary problems (e.g. bony deformities) at 5 to 10 years of age, minimizing the need for complex surgery at a later age and enhancing quality of life.