Selective dorsal rhizotomy in non-ambulant children with cerebral palsy: a multi-center prospective study.

PURPOSE: Assess the effects of selective dorsal rhizotomy (SDR) on motor function and quality of life in children with a Gross Motor Function Classification System (GMFCS) level of IV or V (non-ambulatory). METHODS: This is a prospective, observational study in three tertiary neurosurgery units in England, UK, performing SDR on children aged 3-18 with spastic diplegic cerebral palsy, and a GMFCS level of IV or V, between 2012 and 2019. The primary outcome measure was the change in the 66-item Gross Motor Function Measure (GMFM-66) from baseline to 24 months after SDR, using a linear mixed effects model. Secondary outcomes included spasticity, bladder function, quality of life, and pain scores. RESULTS: Between 2012 and 2019, 144 children who satisfied these inclusion criteria underwent SDR. The mean age was 8.2 years. Fifty-two percent were female. Mean GMFM-66 score was available in 77 patients (53.5%) and in 39 patients (27.1%) at 24 months after SDR. The mean increase between baseline and 24 months post-SDR was 2.4 units (95% CI 1.7-3.1, p < 0.001, annual change 1.2 units). Of the 67 patients with a GMFM-66 measurement available, a documented increase in gross motor function was seen in 77.6% (n = 52). Of 101 patients with spasticity data available, mean Ashworth scale decreased after surgery (2.74 to 0.30). Of patients' pain scores, 60.7% (n = 34) improved, and 96.4% (n = 56) of patients' pain scores remained the same or improved. Bladder function improved in 30.9% of patients. CONCLUSIONS: SDR improved gross motor function and reduced pain in most patients at 24 months after surgery, although the improvement is less pronounced than in children with GMFCS levels II and III. SDR should be considered in non-ambulant patients.

Selective dorsal rhizotomy for the treatment of severe spastic cerebral palsy: efficacy and therapeutic durability in GMFCS grade IV and V children.

BACKGROUND: Selective dorsal rhizotomy (SDR) has been established as an effective surgical treatment for spastic diplegia. The applicability of SDR to the full spectrum of spastic cerebral palsy and the durability of its therapeutic effects remain under investigation. There are currently limited data in the literature regarding efficacy and outcomes following SDR in Gross Motor Function Classification System (GMFCS) IV and V patients. Intrathecal baclofen has traditionally been the surgical treatment of choice for these patients. When utilised primarily as a treatment for the relief of spasticity, it is proposed that SDR represents a rational and effective treatment option for this patient group. We report our outcomes of SDR performed on children with severe cerebral palsy (GMFCS grade IV and V). The commensurate improvement in upper as well as lower limb spasticity is highlighted. Apparent benefit to urological function following SDR in this patient group is also discussed. METHOD: A retrospective review of prospectively collected data for 54 paediatric patients with severe cerebral palsy (GMFCS IV-V) who received SDR plus specialised physiotherapy. Mean age was 10.2 years (range, 3.0-19.5). SDR guided by electrophysiological monitoring was performed by a single experienced neurosurgeon. All subjects received equivalent physiotherapy. The primary outcome measure was change to the degree of spasticity following SDR. Spasticity of upper and lower limb muscle groups were quantified and standardised using the Ashworth score. Measures were collected at baseline and at 2-, 8- and 14-month postoperative intervals. In addition, baseline and 6-month postoperative urological function was also evaluated as a secondary outcome measure. RESULTS: The mean lower limb Ashworth score at baseline was 3.2 (range, 0-4). Following SDR, significant reduction in lower limb spasticity scores was observed at 2 months and maintained at 8 and 14 months postoperatively (Wilcoxon rank, p < 0.001). The mean reduction at 2, 8 and 14 months was 3.0, 3.2 and 3.2 points respectively (range, 1-4), confirming a sustained improvement of spasticity over a 1-year period of follow-up. Significant reduction in upper limb spasticity scores following SDR was also observed (mean, 2.9; Wilcoxon rank, p < 0.001). Overall, the improvement to upper and lower limb tone following SDR-generally to post-treatment Ashworth scores of 0-was clinically and statistically significant in GMFCS IV and V patients. Urological assessment identified pre-existing bladder dysfunction in 70% and 90% of GMFCS IV and V patients respectively. Following SDR, improvement in urinary continence was observed in 71% of affected GMFCS IV and 42.8% of GMFCS V patients. No serious postoperative complications were identified. CONCLUSIONS: We conclude that SDR is safe and-in combination with physiotherapy-effectively reduces spasticity in GMFCS grade IV and V patients. Our series suggests that spastic quadriplegia is effectively managed with significant improvements in upper limb spasticity that are commensurate with those observed in lower limb muscle groups. These gains are furthermore sustained more than a year postoperatively. In light of these findings, we propose that SDR constitutes an effective treatment option for GMFCS IV and V patients and a rational alternative to intrathecal baclofen.

Intrathecal baclofen pumps do not accelerate progression of scoliosis in quadriplegic spastic cerebral palsy.

PURPOSE: To compare scoliosis progression in quadriplegic spastic cerebral palsy with and without intrathecal baclofen (ITB) pumps. METHODS: A retrospective matched cohort study was conducted. Patients with quadriplegic spastic cerebral palsy, GMFCS level 5, treated with ITB pumps with follow-up >1 year were matched to comparable cases by age and baseline Cobb angle without ITB pumps. Annual and peak coronal curve progression, pelvic obliquity progression and need for spinal fusion were compared. RESULTS: ITB group: 25 patients (9 female), mean age at pump insertion 9.4 and Risser 0.9. Initial Cobb angle 25.6° and pelvic tilt 3.2°. Follow-up 4.3 (1.0-7.8) years. Cobb angle at follow-up 76.1° and pelvic tilt 18.9°. Non-ITB group: 25 patients (14 female), mean age at baseline 9.2 and Risser 1.0. Initial Cobb angle 29.7° and pelvic tilt 7.1°. Follow-up 3.5 (1.0-7.5) years. Cobb angle at follow-up 69.1° and pelvic tilt 21.0°. The two groups were statistically similar for baseline age, Cobb angle and Risser grade. Mean curve progression was 13.6°/year for the ITB group vs 12.6°/year for the non-ITB group (p = 0.39). Peak curve progression was similar between the groups. Pelvic tilt progression was comparable; ITB group 4.5°/year vs non-ITB 4.6°/year (p = 0.97). During follow-up 5 patients in the ITB group and 9 in the non-ITB group required spinal fusion surgery for curve progression (p = 0.35). CONCLUSIONS: Patients with quadriplegic spastic cerebral palsy with and without ITB pumps showed significant curve progression over time. ITB pumps do not appear to alter the natural history of curve progression in this population.

Selective dorsal rhizotomy as an alternative to intrathecal baclofen pump replacement in GMFCS grades 4 and 5 children.

BACKGROUND: Conventionally, selective dorsal rhizotomy (SDR) has been reserved for ambulant children and implantation of intrathecal baclofen (ITB) pump for non-ambulant children with cerebral palsy. Rather than replacing the ITB pump in selected Gross Motor Function Classification System (GMFCS) grades 4 and 5 children, we elected to undertake SDR instead. We discuss the rationale and outcomes. OBJECTIVES: To assess if children with severe spasticity treated with long-term ITB pump would benefit from SDR as alternative procedure to replacement of ITB pump. METHOD: This study is a prospective review of ten children with severe spasticity. Indications for ITB pump replacement in 3/10 children were previous ITB pump infection and the remaining seven were nearing depletion of drug delivery system. Pre- and post-SDR mean modified Ashworth scores, assessment of urological function and survey of parent/carer satisfaction were undertaken. RESULT: Mean Ashworth score reductions post-SDR in the lower limbs and upper limbs were 2.4 and 1.70, respectively. An improvement in urological function was also noticed in 27% of patients. Overall, 90% of parents/carers felt that functional outcome with SDR was improved compared with that of ITB. CONCLUSION: SDR in comparison to ITB in this subgroup is cheaper, less intrusive by avoiding refills/replacement and found to be more effective than ITB in reducing spasticity and providing ease for nursing care. We therefore suggest that consideration should be given to SDR as an alternative in patients previously implanted with ITB systems complicated by infection or nearing end of battery life.

Combined selective dorsal rhizotomy and scoliosis correction procedure in patients with cerebral palsy.

UNLABELLED: Intrathecal baclofen (ITB) therapy for spasticity has been suggested to accelerate the development of scoliosis. We present the case of a 17-year-old female patient with cerebral palsy who had ITB therapy from the age of 11 years. During this period, she developed a severe scoliosis measuring 86° from T11 to L4, with pain due to costo-pelvic impingement. Her baclofen pump had reached its end of life and required replacement if ITB therapy was to continue. This coincided with plans for scoliosis corrective surgery. METHODS: We performed scoliosis correction along with removal of baclofen pump and selective dorsal rhizotomy (SDR), as a single combined procedure. SDR was performed instead of ITB pump replacement for management of spasticity. RESULTS: Following surgery, scoliosis improved to 24°. At 6 month follow-up, there was significant improvement in spasticity and quality of life. CONCLUSIONS: This report illustrates the feasibility of a combined procedure to correct scoliosis and manage spasticity with SDR. We present the case details, our management and review of the published literature regarding the factors influencing treatment of scoliosis and spasticity.

Cervical catheter placement for intrathecal baclofen test dose: is it safe?

INTRODUCTION: Intrathecal baclofen testing is usually performed via a catheter inserted at the lumbar spine. However, in patients with scoliosis, the distorted anatomy and bone fusion from corrective spinal surgery obviates access at the lumbar spine. PATIENTS AND METHODS: We report a method for inserting the intrathecal catheter via a posterior cervical approach and discuss our outcome. We describe our experience in 20 patients in whom intrathecal baclofen test catheter was inserted at the lower cervical spine. Data was collected prospectively. DISCUSSION: The procedure was successful in all 20 patients. There were no complications as a result of surgery or from baclofen test dose administration via a cervical catheter. Of the 20 patients, 15 went on to have baclofen pump implantation via a cervical catheter. In one patient, subsequent pump implantation procedure was unsuccessful due to presence of extradural scar tissue. In the remaining four patients pump implantation was declined due impending corrective spinal surgery or social reasons. CONCLUSION: Our experience shows that low cervical catheter insertion for administration of a test dose of intrathecal baclofen and feasible and safe to perform via the method described.

Controlled study of the effects of continuous intrathecal baclofen infusion in non-ambulant children with cerebral palsy.

AIM: To measure changes in children with severe spastic cerebral palsy (CP) after continuous intrathecal baclofen (ITB) infusion over 18 months and to compare the results with those of a comparison group awaiting treatment. METHOD: Thirty-eight children with severe spastic CP considered suitable for ITB were assessed when first seen, just before insertion of an intrathecal pump, and 9 months and 18 months later. Eighteen children waited around 9 months for a pump (group 1: nine males, nine females; mean age 9y 11mo [SD 3y 7mo], nine in Gross Motor Function Classification System [GMFCS] level IV, nine in level V). This baseline period was used as a control for comparison with the first and second 9-month periods after the pump for the remaining 20 children (group 2: 11 males, nine females; mean age 10y 2mo [SD 3y 1mo], nine in GMFCS level IV, 11 in level V). The main outcome measure was the Pediatric Evaluation of Disability Inventory (PEDI); other assessments were of function, ease of care, quality of life, and costs of new equipment. RESULTS: No significant change was found in the PEDI between group 1 while awaiting treatment and group 2 in the two periods afterwards, nor in the Lifestyle Assessment Questionnaire or the cost of new equipment. Significant changes were found in group 2 in the first 9 months according to the modified Ashworth score (difference between mean values for groups -1.7, standard error 0.58; p=0.008), Penn Spasm score (-1.3, 0.37; p=0.001), mean joint range of movement (8.3°, 2.8; p=0.005), and Caregiver Questionnaire (-19.7, 5.1; p=0.01), and in the second 9 months for the Modified Ashworth Scale score (-0.62, 0.12; p=0.001). INTERPRETATION: ITB in children with severe spastic CP over the first 18 months improves their quality of life in terms of comfort and ease of care. It has less effect on function, participation in society, or the overall cost of new equipment.

Intrathecal baclofen pumps in the management of hypertonia in childhood: a UK and Ireland wide survey.

BACKGROUND: Intrathecal baclofen (ITB) is a useful treatment for hypertonia where non-invasive treatments have been ineffective or poorly tolerated. There is an absence of national guidance on selection criteria and a lack of literature regarding patient characteristics and treatment details for children and young people (CYP) receiving ITB therapy in the UK and Ireland. We aimed to gather patient and treatment characteristics for CYP receiving ITB in the UK and Ireland. METHODS: An electronic survey was sent to all paediatric ITB centres in the UK and Ireland. Anonymised data were returned between December 2019 and April 2020. CYP >16 years and those awaiting ITB pump removal were excluded from the dataset. RESULTS: 176 CYP were identified as receiving ITB therapy across the UK and Ireland. The majority of CYP with ITB pumps were non-ambulant (93%) with a diagnosis of cerebral palsy (79%). Median age of ITB insertion was 9 years; median current age was 14 years. 79% of CYP had significant spasticity, 55% had significant dystonia. The most commonly used ITB dosing modes were continuous (73%) and flexible (23%). CONCLUSIONS: ITB pumps were most frequently used for non-ambulant CYP with cerebral palsy and existence of spasticity and/or dystonia in the UK and Ireland. Most CYP were receiving a continuous dose of ITB. There is significant variation in the number of paediatric ITB pumps across UK and Ireland. There is a need for development of nationally accepted paediatric referral criteria and clinical standards for ITB use.

Intrathecal baclofen therapy for spasticity of cerebral origin--does the position of the intrathecal catheter matter?

OBJECTIVE: To assess the correlation between the position of the intrathecal baclofen therapy catheter with the clinical outcome and response to the spasticity. METHODS AND RESULTS: A prospective cohort study was done by reviewing the paediatric group of patients with spasticity of cerebral origin who had insertion of a programmable baclofen pump for intrathecal administration in the last 10 years (August 1998 to September 2007). A total of 190 procedures were carried out in 166 patients, under a single paediatric neurosurgeon, with an age range of 18 months-16 years (mean 8.75 years) with follow up of 1-10 years (mean 5 years). The routine post-operative X-rays, which were done as per protocol, were reviewed, and the position of the intrathecal catheter tip was documented in relation to the vertebral bodies. The maintenance intrathecal baclofen dose was 25 microgms to 1,000 microgms (mean 255.8 microgms) based on the clinical response to spasticity. Statistical analysis was carried out to assess the correlation of the position of the tip of the intrathecal catheter with the outcome in spasticity based on GMFCS (Gross Motor Function Classification System) and Ashworth score. CONCLUSION: In our series, there was no statistically significant correlation between the position of the intrathecal catheter and the clinical response to the spasticity.

Selective dorsal rhizotomy in ambulant children with cerebral palsy: an observational cohort study.

BACKGROUND: Selective dorsal rhizotomy (SDR) is an irreversible surgical procedure involving the division of selected sensory nerve roots, followed by intensive physiotherapy. The aim is to improve function and quality of life in children with cerebral palsy and a Gross Motor Function Classification System (GMFCS) level of II or III (walks with or without assistive devices, respectively). We assessed gross motor function before and after SDR and postoperative quality of life in a study commissioned by NHS England. METHODS: We did a prospective observational study in five hospitals in England who were commissioned to perform SDR on children aged 3-9 years with spastic diplegic cerebral palsy. The primary outcome was score changes in the 66-item Gross Motor Function Measure (GMFM-66) and seven domains of the Cerebral Palsy Quality of Life Questionnaire ([CP-QoL] social wellbeing and acceptance, feelings about functioning, participation and physical health, emotional wellbeing and self-esteem, access to services, family health, and pain and impact of disability) from before to 24 months after SDR. FINDINGS: From Sept 4, 2014, to March 21, 2016, 137 children underwent SDR. The mean age was 6·0 years (SD 1·8). The mean GMFM-66 score increased after SDR with an annual change of 3·2 units (95% CI 2·9 to 3·5, n=137). Of the seven CP-QoL domains, five showed significant improvements over time: feelings about functioning mean annual change 3·0 units (95% CI 2·0 to 4·0, n=133), participation and physical health 3·9 units (2·5 to 5·3, n=133), emotional wellbeing and self-esteem 1·3 units (0·2 to 2·3, n=133), family health 2·0 units (0·7 to 3·3, n=132), and pain and impact of disability -2·5 units (-3·9 to -1·2, n=133). 17 adverse events were reported in 15 children, of which none were severe and 15 (88%) resolved. INTERPRETATION: SDR improved function and quality of life in the 24 months after surgery in children with cerebral palsy classified as GMFCS levels II and III. On the basis of these findings, an interim national policy decision was made that SDR would be funded for eligible children in England from 2018. FUNDING: National Institute for Health and Care Excellence, National Institute for Health Research Biomedical Research Centre, NHS England.

The effect of continuous intrathecal baclofen on sitting in children with severe cerebral palsy.

AIM: To investigate the effect of intrathecal baclofen (ITB) on sitting in children with cerebral palsy with severe spasticity; and identify potential sub-groups of patients at particular risk of deterioration. METHOD: Twenty three children with cerebral palsy, mean age 10 yrs 10 mo were assessed before and after ITB treatment using the Sitting dimension of the Gross Motor Function Measure. Sitting prior to treatment was compared to sitting following ITB treatment in the same children. Exploration of sub groups was also attempted to investigate affects of ITB on sitting according to age and severity of motor impairment. RESULTS: No significant difference was found in sitting before ITB treatment compared to sitting following insertion of an ITB pump (p = 0.09). No specific age group or classification of motor impairment demonstrated significant deterioration in sitting following ITB treatment. CONCLUSION: Sitting does not improve or deteriorate in children following treatment with ITB, independent of age or severity of motor impairment.

Intrathecal baclofen therapy--how we do it.

Intrathecal baclofen has become an invaluable tool to treat spasticity of various etiologies. Children who benefit from intrathecal baclofen are often significantly underweight due to underlying disease pathology, and they lack adequate soft-tissue mass to effectively provide cover to the pump. Thus, in this population, subfascial implantation is favored over the subcutaneous technique in view of the high frequency of wound dehiscence and subsequent explantation of the pump associated with the latter method. The authors describe and review their unit's adapted subfascial implantation technique that has been performed over a period of 10 years in 182 children. This technique provides better tissue coverage for the pump and has resulted in lowering the risk of complications as compared with the subcutaneous technique.

Cervical implantation of intrathecal baclofen pump catheter in children with severe scoliosis.

OBJECT: Intrathecal baclofen (ITB) pump catheter placement is traditionally performed through entry into the spinal sac at the lumbar spine. A minority of children with cerebral palsy have severe concomitant neuromuscular scoliosis. In these children, whether surgically treated or not, access to the intradural space via the lumbar spine may prove technically challenging. The authors report on a series of children in whom, for various reasons, an ITB catheter was implanted using a posterior cervical spine approach. METHODS: The records of 20 children in whom a baclofen catheter had been placed were retrospectively reviewed to assess the demographic details, indications, and outcome of this procedure. RESULTS: This approach was successful in all but one of the children in whom the procedure was abandoned given the presence of significant extradural scar tissue. Of the 20 children, 7 had previously undergone lumbar ITB catheter implantation, although the catheter was subsequently, iatrogenically transected during scoliosis surgery. Nine children had had corrective scoliosis surgery, and the fusion mass obviated access to the lumbar spinal sac. Four children had untreated scoliosis and corrective surgery was being contemplated. Complications included infection requiring explantation (2 patients), catheter migration (1 patient), intolerance to ITB (1 patient), and failure of implantation (1 patient). All patients who tolerated the ITB experienced improvement in spasticity. No complications were associated with the spinal level of catheter insertion. CONCLUSIONS: Implantation of an ITB catheter via a cervical approach is safe and feasible and should be considered in children with severe corrected or uncorrected scoliosis, and thus avoiding the lumbar spinal sac.

Goals and outcomes for non ambulant children receiving continuous infusion of intrathecal baclofen.

AIM: To evaluate the success of goals and compare these to actual outcomes in severely disabled children receiving continuous intrathecal baclofen [ITB]. METHOD: 37 non ambulant children with severe spasticity were assessed just before implantation of a pump for ITB, and 9 and 18 months afterwards. Three key goals were chosen for treatment by the family and therapist. These were reviewed at the assessments, together with caregivers' views of the outcome of treatment in 14 different aspects. At the first and last assessment, the degree of deformity of the hips and spine were reviewed, and Orthopaedic Surgeons were also asked to predict what surgery would be needed in the next 2 years. RESULTS: The most common successful outcomes were ease of nursing care, better sitting, spasm reduction, more relaxed/better mood, and improved sleep. This was reflected in the goals selected which were therefore realistic for this treatment. All 3 pre-set goals were achieved by 80% of children. Deformities of the hip and spine continued to occur. The predicted number of orthopaedic operations before and after ITB remained unchanged. INTERPRETATION: ITB is a major treatment for children with severe disability and should be undertaken with understanding of what can and cannot be achieved, therefore allowing realistic goals to be set.

Dynamic facial prosthetics for sufferers of facial paralysis.

BACKGROUND: This paper discusses the various methods and the materials for the fabrication of active artificial facial muscles. The primary use for these will be the reanimation of paralysed or atrophied muscles in sufferers of non-recoverable unilateral facial paralysis. METHOD: The prosthetic solution described in this paper is based on sensing muscle motion of the contralateral healthy muscles and replicating that motion across a patient's paralysed side of the face, via solid state and thin film actuators. The development of this facial prosthetic device focused on recreating a varying intensity smile, with emphasis on timing, displacement and the appearance of the wrinkles and folds that commonly appear around the nose and eyes during the expression. An animatronic face was constructed with actuations being made to a silicone representation musculature, using multiple shape-memory alloy cascades. Alongside the artificial muscle physical prototype, a facial expression recognition software system was constructed. This forms the basis of an automated calibration and reconfiguration system for the artificial muscles following implantation, so as to suit the implantee's unique physiognomy. RESULTS: An animatronic model face with silicone musculature was designed and built to evaluate the performance of Shape Memory Alloy artificial muscles, their power control circuitry and software control systems. A dual facial motion sensing system was designed to allow real time control over model - a piezoresistive flex sensor to measure physical motion, and a computer vision system to evaluate real to artificial muscle performance. Analysis of various facial expressions in real subjects was made, which give useful data upon which to base the systems parameter limits. CONCLUSION: The system performed well, and the various strengths and shortcomings of the materials and methods are reviewed and considered for the next research phase, when new polymer based artificial muscles are constructed and evaluated.

Status dystonicus resembling the intrathecal baclofen withdrawal syndrome: a case report and review of the literature.

INTRODUCTION: Status dystonicus is a rare but life-threatening disorder characterized by increasingly frequent and severe episodes of generalized dystonia that may occur in patients with primary or secondary dystonia. Painful and repetitive spasms interfere with respiration and may cause metabolic disturbances such as hyperpyrexia, dehydration, respiratory insufficiency, and acute renal failure secondary to rhabdomyolysis. Intrathecally administered baclofen, delivered by an implantable pump system, is widely used for the treatment of refractory spasticity. Abrupt cessation of intrathecal baclofen infusion has been associated with a severe withdrawal syndrome comprised of dystonia, autonomic dysfunction, hyperthermia, end-organ failure and sometimes death. The aetiology of this syndrome is not well understood. Status dystonicus describes the episodes of acute and life-threatening generalized dystonia, which occasionally manifest themselves in patients with dystonic syndromes. CASE PRESENTATION: We present the case of a nine-year-old Caucasian boy who experienced a severe episode of status dystonicus with no known cause and clinical features resembling those described in intrathecal baclofen withdrawal. Our patient subsequently underwent the placement of an intrathecal baclofen pump without incident. CONCLUSION: The similarity between the clinical features of the case we present and those reported in connection to abrupt withdrawal of intrathecal baclofen is emphasized. Several drugs, although not intrathecal baclofen withdrawal, have previously been associated with status dystonicus. The similarity between the life-threatening dystonic episode experienced by our patient, and those reported in intrathecal baclofen withdrawal, highlights the possibility that, rather than representing a true physiological withdrawal syndrome, abrupt withdrawal of intrathecal baclofen may simply precipitate an episode of status dystonicus in susceptible individuals. The clinical similarities between the intrathecal baclofen withdrawal syndrome and status dystonicus have not previously been highlighted.

Medulloblastoma in childhood: revisiting intrathecal therapy in infants and children.

INTRODUCTION: Intrathecal chemotherapy is being explored in medulloblastoma in pre-school children as part of brain-sparing strategies and as an alternative to unacceptably neurotoxic cranio-spinal radiotherapy. The range of drugs suitable for this route of administration is restricted by the lack of research evidence of pharmacological suitability and efficacy of other drugs in medulloblastoma. METHODS: Ideal clinical, biological, physicochemical and pharmaceutical properties for intrathecal administration were defined through literature review of pharmaceutical texts, Medline, Embase and consulting the manufacturers. A total of 126 chemotherapy agents were assessed against these criteria by searching the academic domain of pharmaceutical texts, computer databases and consultation with manufacturers. RESULTS: Of the 126 candidate drugs, 99 were rejected because of documentation of their irritant nature, neurotoxicity and requirement for hepatic activation in standard pharmaceutical texts. Fifty were rejected for a single identifiable reason including, neurotoxicity (n = 24), irritant (n = 15), needs enzyme activation (n = 5), clinical evidence of intrathecal neurotoxicity (n = 4) and no evidence of tumour-specific efficacy (n = 2). Where two reasons were cited the justifications were: neurotoxic and irritant (n = 3) and needs activation and systemic administration results in equivalent concentration (n = 1). Twenty-seven drugs remained of which 12 were selected as eligible for further clinical investigation, and 15 were selected for further pre-clinical investigation. CONCLUSIONS: The pre-determined criteria were not applicable, in their entirety, in the majority of drugs, due to lack of information in the academic domain, emphasising the importance of a more open approach for sharing basic drug information. The prioritised list of 12 candidate drugs for clinical trial and 15 for pre-clinical investigation justify that a concerted research effort in this area of practice is made.

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.

Intrathecal baclofen in the management of spasticity due to cerebral palsy.

Intrathecal baclofen is a cornerstone in the treatment of spasticity in children. However, further research is required to set criteria for treatment. This paper offers insight into the national act on baclofen and the experience at the Nottingham University Hospital.

A clinical study of intrathecal baclofen using a programmable pump for intractable spasticity.

OBJECTIVE: To determine the impact of intrathecal baclofen (ITB) therapy on outcomes of functional independence, pain, subjective improvement, performance, and standard measures of spasticity. DESIGN: A noncomparative, multicenter, prospective cohort trial of patients with implanted pumps followed up over a 12-month period for the assessment of spasticity, pain, and function. SETTING: Twenty-four European centers of neurology or rehabilitation familiar with implantable pump technique participated. PARTICIPANTS: Patients with intractable spasticity (N=138) who responded positively to a trial dose of baclofen (n=133) and who began ITB therapy (n=129) were enrolled. INTERVENTION: Implantation of a Medtronic SynchroMed Infusion System with the administration of ITB therapy. MAIN OUTCOME MEASURES: Ashworth Scale assessment, Penn Spasm Frequency Scale scores, pain assessment, FIM instrument scores or WeeFIM scores for children, Canadian Occupational Performance Measure (COPM), and subjective ratings of overall relief were the tools administered. RESULTS: Muscle tone, spasm scores, and pain intensity reductions were observed. Overall FIM scores increased significantly in cognitive and motor function. COPM scores for both performance and satisfaction also improved significantly. Patients reported increased relief from pain and spasticity, supported by physician reports. Forty-three percent of patients reported adverse events, mostly related to patients' underlying conditions (20%), the device implant surgery (10%), or complications with the catheter (9%). CONCLUSIONS: ITB therapy using a programmable pump is clinically effective and well tolerated, despite a seemingly high level of adverse events, in patients with intractable spasticity of spinal or cerebral origin and may offer improvements in pain relief and function.