Best Practices for Intrathecal Baclofen Therapy: Dosing and Long-Term Management.

INTRODUCTION: Intrathecal baclofen (ITB) therapy aims to reduce spasticity and provide functional control. METHOD: An expert panel consulted on best practices. RESULTS: Pump fill and drug delivery can be started intraoperatively, with monitoring for at least eight hours. Initiate with the 500 mcg/mL concentration. The starting daily dose should be twice the effective bolus screening dose, or the screening dose if the patient had a prolonged response (greater than eight hours) or negative reactions. Oral antispasmodics can be weaned, one drug at a time beginning with oral baclofen after ITB begins. Assessment should occur within 24 hours of a dose change. For adults, daily dose increases may be 5% to 15% once every 24 hours for cerebral-origin spasticity and 10% to 30% once every 24 hours for spinal-origin spasticity. Daily dose increases can be 5% to 15% once every 24 hours for children. Inpatients should be assessed at least every 24 hours and receive rehabilitation. Step dosing can be used for outpatients who cannot return daily. Dosing options include simple continuous dosing, variable 24-hour flex dosing, or regularly scheduled boluses. Patients/caregivers should understand the care plan, responsibilities, and possible side-effects. Low-reservoir alarm dates and refill schedules should be written down, along with emergency contact information. A higher concentration at refill can extend refill intervals, and a bridge bolus must be programmed. Time changes may affect flex dosing. Pump replacement should be scheduled at least three months in advance. CONCLUSIONS: ITB dosing is multistep and individualized.

Prolonged, severe intrathecal baclofen withdrawal syndrome: a case report.

Intrathecal baclofen (ITB) withdrawal is a well-recognized complication when drug delivery is disrupted for any reason. ITB withdrawal varies widely in its severity and poses the very real possibility of death if not promptly managed. Cases of withdrawal lasting greater than 1 or 2 weeks, however, are sparse. We report the case of an 11-year-old girl with spastic quadriplegic cerebral palsy who developed an infected pump and subsequent meningitis, prompting the removal of her pump and catheter. She subsequently developed a severe, prolonged baclofen withdrawal syndrome marked by increased spasticity, agitation, hypertension, and tachycardia that lasted nearly 2 months, requiring intensive care and continuous intravenous sedation with benzodiazepines and opiates. Her pump was eventually replaced on hospital day 56 and within 24 hours her symptoms dramatically improved. She was eventually weaned off sedating medications and returned to baseline functional status. Typical management of baclofen withdrawal is reviewed. To date, the literature has not discussed the potential role for opiates in managing baclofen withdrawal, yet a growing body of literature is examining the interplay between opiates and gamma-aminobutyric acid B pathways. A potential role for opiates in managing severe baclofen withdrawal is proposed.

Orthopedic surgery in children with intrathecal baclofen pumps.

AIMS: Children with cerebral palsy often have severe spasticity leading to deformity that requires multiple orthopedic surgeries. Intrathecal baclofen pump implantation effectively decreases severe spasticity. The objective of this study was to determine whether children who have a baclofen pump implanted at a young age have fewer orthopedic surgeries than those who have a baclofen pump implanted at later ages. METHODS: In this retrospective study of 310 children, we compared occurrence of surgery in relation to having or not having the baclofen pump, by using survival analysis with surgery as the outcome, presence of baclofen pump as the exposure of interest, modeled as a time-dependent variable, and age as the time scale. RESULTS: There was no significant effect of pump placement on overall surgery frequency. Analyses by type of surgery showed that those without a pump in place had a 64% lower hazard of scoliosis surgery. CONCLUSIONS: No evidence was found to indicate that children who have a baclofen pump implanted at a young age have fewer orthopedic surgeries than those who have a baclofen pump implanted at later ages.

Stereotactic endoscopic placement of third ventricle catheter for long-term infusion of baclofen in patients with secondary generalized dystonia.

Continuous infusion of baclofen is a treatment option for severe generalized dystonia. Catheter insertion within the third ventricle has been described as an alternative to standard intrathecal placement to maximize intracranial concentrations of baclofen. The authors describe their experience with a novel technique for stereotactic endoscopic insertion of baclofen infusion catheters in the third ventricle in 3 patients with severe secondary generalized dystonia. Insertion was successful in all 3 patients, and all of them experienced significant improvement in dystonia scores on the Barry-Albright Dystonia Scale. Follow-up ranged from 5.5 to 7 months (mean 6 months), and no mechanical complications or CSF leaks were observed. The stereotactic endoscopic insertion of a baclofen infusion catheter into the third ventricle appears to be a safe method for continuous intraventricular baclofen infusion in patients with generalized secondary dystonia.

Selective dorsal rhizotomy in children with spastic hemiparesis.

OBJECT: Neurological conditions including cerebral palsy, brain injury, and stroke often result in severe spasticity, which can lead to significant deformity and interfere with function. Treatments for spasticity include oral medications, intramuscular botulinum toxin type A injections, orthopedic surgeries, intrathecal baclofen pump implantation, and selective dorsal rhizotomy (SDR). Selective dorsal rhizotomy, which has been well studied in children with spastic diplegia, results in significant reduction in spasticity and improved function in children. To the authors' knowledge, there are no published outcome data for SDR in patients with spastic hemiparesis. The object of this study was to examine the effects of SDR on spastic hemiparesis. METHODS: A 2-year study was undertaken including all children with spastic hemiparesis who underwent SDR at the authors' institution. The degree of spasticity, as measured by the Modified Ashworth Scale or quality of gait rated using the visual gait assessment scale, the gait parameters, and velocity were compared in patients before and after undergoing SDR. RESULTS: Thirteen children (mean age 6 years 7 months) with spastic hemiparesis underwent SDR performed by the same surgeon during a 2-year period. All of the patients had a decrease in tone in the affected lower extremity after the procedure. The mean reduction in tone in 4 muscle groups (hip adductors, knee flexors, knee extensors, and ankle plantar flexors) according to the modified Ashworth scale score was 2.6 ± 1.26 (p < 0.0001). The quality of gait was assessed in 7 patients by using the visual gait assessment scale. This score improved in 6 patients and remained the same in 1. Stride length and gait velocity were measured in 4 children. Velocity increased in 3 patients and decreased in a 3-year-old child. Parents and clinicians reported an improvement in quality of gait after the procedure. Stride length increased bilaterally in 3 patients and increased on one side and decreased on the other in the other patient. CONCLUSIONS: Selective dorsal rhizotomy showed efficacy in the treatment of spastic hemiparesis in children. All of the patients had decreased tone after SDR as measured by the modified Ashworth scale. The majority of patients had qualitative and quantitative improvements in gait.

Combining botulinum toxin and phenol to manage spasticity in children.

OBJECTIVE: To describe the specific techniques and adverse reactions of using concurrent, multiple injections of both botulinum toxin and phenol to manage spasticity in children with cerebral palsy (CP) and other neurologic conditions. DESIGN: A retrospective case series. SETTING: A tertiary care children's hospital. PARTICIPANTS: Consecutive patients (N=68) with spasticity related to CP or other neurologic conditions. INTERVENTION: Ninety injection sessions combining botulinum toxin and phenol to manage spasticity. MAIN OUTCOME MEASURE: Documentation of adverse reactions. RESULTS: The mean phenol dosage was 9.5mL at a mean of 0.6mL/kg per injection dose. The mean botulinum toxin type A (Botox) dose injected was 193U (12U/kg), and the mean of botulinum toxin type B (Myobloc) dose injected was 7750U (530U/kg). The mean number of muscles injected was 14. Adverse reactions are described but were infrequent. Dysesthetic hand pain occurred in 2 patients. One patient developed a systemic reaction to Myobloc. CONCLUSIONS: Using botulinum toxin and phenol injections allowed many muscles to be injected to manage spasticity in children with CP and other neurologic conditions. Using this combination allowed an increased number of injections at the maximal recommended dose.

Care provider assessment of intrathecal baclofen in children.

Intrathecal baclofen is used increasingly to manage severe spasticity in children. Before implanting the baclofen pump, care providers typically ask how it will benefit their child. The purpose of this study was to describe the perceptions of care providers about intrathecal baclofen for management of spasticity in 80 individuals (52 males, 28 females). The individuals were younger than 22 years at the time of implantation (mean age 11 years, SD 5 years; range 3 to 21 years). Participants had been implanted with the pump for a minimum of one year at the time of evaluation. The most common diagnoses were quadriplegic and diplegic cerebral palsy and traumatic brain injury. Most participants were at level IV and V on the Gross Motor Function Classification System. After pump implantation most participants had tone reduction on the Ashworth scale of 1 to 1.9 in the lower extremities and 0 to 0.9 in the upper extremities. Lower extremity range of motion was maintained in 43 of 51 individuals (84%) and lost in 8 participants (16%). Complications requiring surgery occurred in 63 of a larger group of 152 patients (incidence per patient-year of follow-up was 0.19). Thirty-one of the 80 children had orthopedic procedures after pump placement. Only one of these was unexpected and none had rapid progression of scoliosis. Most treatment goals were achieved. Goals most commonly chosen (decreased pain, prevention of worsening of deformity, and improved ease of care) were improved in 91%, 91%, and 88% of participants respectively. Ninety-five per cent of care providers agreed that they would have this procedure performed again (81% strongly agreed, 14% slightly agreed). All care providers reported improvement in scores on the Caregiver Questionnaire. This information has been helpful to families considering intrathecal baclofen therapy.

Complications of intrathecal baclofen pumps in children.

Intrathecal baclofen is increasingly being used to manage severe spasticity in children. Although substantial tone reduction with this treatment has been documented, complications also occur. In this study, we describe the device- and major non-device-related complications in a group of 100 consecutive children and young adults who received 117 intrathecal baclofen pumps for the management of severe spasticity. Twenty-four patients (24%) experienced a total of 48 complications. The most common complication was disconnection of the catheter at its connection to the pump, occurring in 9% of pumps implanted. This complication occurred more frequently in pumps with catheter access ports (16%) than in those without ports (2%). Catheter dislodgement from the intrathecal space was the next most common complication, occurring in 8% of pumps implanted (13% of pumps with ports, 4% of pumps without ports). To decrease the occurrence of the most common complications of intrathecal pumps, we now typically implant pumps without catheter access ports, and we use 2-piece catheters. Although the lack of an access port may be a disadvantage for troubleshooting, most complications can be detected in pumps without a port. Patient and family education is critical in preventing serious consequences of baclofen withdrawal resulting from catheter-related complications.