Intrathecal baclofen in hereditary spastic paraparesis: benefits and limitations]

BACKGROUND: Chronic intrathecal delivery of baclofen has been introduced for treatment of severe spinal spasticity. Very little is known about this treatment in hereditary spastic paraparesis. Here we review the benefits and limitations of pump implantation for baclofen delivery in this population. METHODS: Consecutive patients presenting with hereditary spastic paraparesis were assessed for spasticity (Ashworth and Penn scores), muscular strength and walking (speed, comfort and perimeter length). The effect of intrathecal delivery of baclofen was judged after progressive bolus injections or chronic administration by electrical syringe. The pump implantation was proposed when spasticity scores decreased by 2 or more points, with muscular strength preserved and walking area increased. RESULTS: We investigated 6 patients (3 males; mean age 48 years) with hereditary spastic paraparesis. The mean follow-up was 19 years; for 4 patients who received pump implantation, the mean follow-up was 6.2 years. The mean baclofen daily dose was 75 mug. Satisfaction was high for patients who received implantation early instead of waiting for the natural course of the disease. DISCUSSION: Some patients with hereditary spastic paraparesis have good functional improvement with chronic intrathecal delivery of baclofen if walking is still possible. Despite the natural history of the disease, functional results are stable during the first 5 years of treatment. The data indicate a possible compromise between decreased spasticity and muscular strengthening with the treatment.     

Intrathecal baclofen therapy improves functional intelligibility of speech in cerebral palsy

OBJECTIVE: To assess the effect of intrathecal baclofen on spastic dysarthia in cerebral palsy. DESIGN: Single case study. METHODS: Functional outcome measures, including the Assessment of Intelligibility of Dysarthric Speech, were performed before and after a trial of intrathecal baclofen in an adult patient with spastic dysarthria due to cerebral palsy. The patient proceeded to intrathecal baclofen pump implantation and was reassessed after six months of continuous intrathecal baclofen therapy. RESULTS: Improvement in function including speech intelligibility was seen following the intrathecal baclofen trial. The improvement was sustained at six months post pump implantation. CONCLUSIONS: Intrathecal baclofen improved functional intelligibility of speech in a carefully selected subject. The Assessment of Intelligibility of Dysarthric Speech was found to be a useful quantitative tool to assess the effect of intrathecal baclofen on spastic dysarthria.     

Management of increase in spasticity in patients with intrathecal baclofen pumps]

INTRODUCTION: The incidence of complications after baclofen pump implantation is relatively high. Diagnosis of these complications can be difficult. A diagnostic tree would be a useful tool in cases of suspected malfunctioning of the intrathecal bacolfen infusion system and would standardise the diagnostic procedure. METHOD: From results in the literature and the experience of our department, we designed a diagnostic tree to aid in finding the cause of a recrudescence of spasticity in patients with implanted baclofen pumps. RESULTS: The potential causes of recrudescence of spasticity are described and a diagnostic pathway is proposed. DISCUSSION: The aim of a standardised hierarchical method of diagnosis of the cause of increased spasticity in patients with intrathecal baclofen pumps is to gain time in the diagnosis and treatment. Such diagnosis should improve patient care by permitting rapid restoration of an adequate level of baclofen infusion as well as decreasing the length of hospital stay and, as a consequence, the cost relating to malfunctioning pumps.     

Intrathecal baclofen infusion. Effect on bladder management programs in patients with myelopathy

Intrathecal administration of Baclofen, a GABA agonist, through an implantable drug delivery pump has been demonstrated to be effective in the treatment of limb spasticity in patients with myelopathy. Three patients, followed before and after pump placement, experienced satisfactory spasticity relief and improvement in areas of self-care and mobility. Improvement in the bladder management programs of each patient was noted. These changes coincided with improvement on urodynamic studies, defined as either an increase in bladder capacity or a decrease in sphincter dyssynergia. Changes in bladder function were associated with the initiation of intrathecal therapy and with changes in pump-delivered dosages. In selected patients, intrathecal baclofen infusion can have a beneficial effect on bladder management programs.     

Intrathecal baclofen for spasticity management in Rett syndrome

Intrathecal baclofen infusions have proven to be effective for management of spasticity during the last two decades. Efficacy of intrathecal baclofen for spasticity of spinal origin has been well established and has shown promise in treatment of spasticity that is not spinal in origin. Rett syndrome is a neurodevelopmental disorder primarily affecting girls and women. Manifested in the advanced stages of this syndrome is increased spasticity leading to functional decline. Presented is a case report of a 32-yr-old white woman with Rett syndrome, diagnosed before the age of 2 yr, and significant spasticity that was successfully managed with intrathecal baclofen. After placement of an intrathecal baclofen pump, the dose was increased slowly during 1 yr to 800 microg/day with good clinical response. There was observed a significant decrease in upper and lower limb Ashworth scores, from an average of 3-4 to 2-3, during this 1-yr period. The decrease in spasticity in this patient eventually led to improved range of motion, positioning, skin care, hygiene, and quality of life. Intrathecal baclofen is an effective option in managing severe spasticity from Rett syndrome.     

Improvement in walking speed in poststroke spastic hemiplegia after intrathecal baclofen therapy: a preliminary study

OBJECTIVE: To explore whether intrathecal baclofen (ITB) therapy improves ambulation in stroke survivors. DESIGN: Case series. SETTING: Tertiary care center. PARTICIPANTS: Ten adults with poststroke hemiparesis who were ambulatory at the time of pump implantation. INTERVENTIONS: Implantation of ITB pump after inadequate control of spasticity with other interventions. Time from stroke onset to implantation averaged 28.6 months (range, 9-55mo). MAIN OUTCOME MEASURES: Customary walking speed was measured from the time required to walk 50ft (15m) at a self-selected pace. Evaluators rated spastic hypertonia and functional mobility. RESULTS: Statistically significant improvements occurred in walking speed, functional mobility ratings, and spasticity (P<.05) at a follow-up interval that averaged 8.9 months. Mean walking speed over 50ft improved from 36.6 to 52cm/s. Mean Modified Ashworth Scale scores in the muscles of the affected lower limb improved from 2.0 to 0.4. Normal muscle strength (5/5) was preserved in the unaffected limbs. CONCLUSIONS: This preliminary study suggests that ITB therapy, in combination with physical therapy, may improve walking speed and functional mobility in ambulatory individuals with poststroke spastic hemiplegia.     

Mechanical measurements of the effects of intrathecal baclofen dosage adjustments in cerebral palsy: a pilot study

OBJECTIVE: The purpose of this pilot study was to determine the feasibility of using mechanical measures of stretch reflexes to monitor spasticity after intrathecal baclofen dosage changes. DESIGN: Mechanical measures were made in studying six participants with cerebral palsy who were undergoing treatment for spasticity using intrathecal baclofen therapy. Mechanical measurements of stretch reflexes were made before and after pump implant in four of the six participants and after dosage changes in all participants, for a period of up to 2 yrs. The measurements comprised electromyograms and resistive torque responses to movement of the ankle, imposed with an isokinetic dynamometer. For each test session, random movements were applied to the ankle at each of four to seven speeds, repeated three or five times. RESULTS: Stretch reflex excitability decreased in three of four participants after initiation of intrathecal baclofen therapy and decreased with increasing dosages in three of the six participants. Broken catheters coincided with heightened mechanical measures in two cases. CONCLUSION: Mechanical measures of stretch reflexes change after intrathecal baclofen dosage adjustments, reflecting the clinical course of intrathecal baclofen therapy.     

Effect of intrathecal baclofen bolus injection on temporospatial gait characteristics in patients with acquired brain injury

OBJECTIVE: To evaluate changes in temporospatial gait parameters after intrathecal baclofen (ITB) bolus administration in patients with spasticity resulting from acquired brain injury by using computerized gait analysis. DESIGN: Case series; before-after intervention. Walking performance and spasticity in lower-extremity muscles were assessed before and at 2, 4, and 6 hours after ITB bolus injection. SETTING: Tertiary care free-standing rehabilitation hospital. PARTICIPANTS: Consecutive sample of 28 adults with acquired brain injury due to stroke, trauma, or anoxia. INTERVENTION: A 50-microg ITB bolus injection (2 subjects received 75 microg and 100 microg, respectively). MAIN OUTCOME MEASURES: Ashworth Scale scores, self-selected gait velocity, stride length, cadence, step length symmetry, step width, and percentage of single support on the more involved side. RESULTS: Ashworth score decreased from 2.0+/-0.5 at baseline to 1.3+/-0.3 at peak response ( P <.001), whereas gait velocity increased from 41+/-26 to 47+/-31 cm/s ( P <.001). Significant improvements also occurred in stride length ( P <.05) and step width ( P <.001). Gait velocity was the most sensitive temporospatial outcome measure for differentiating functional response to ITB bolus injection. Sixteen patients increased velocity by an average of 12 cm/s, representing a mean gain of 33% over their baseline walking speed, while 5 decreased (mean loss, -6 cm/s [52% of baseline]) and 7 were unchanged. There was a significant correlation between baseline velocity and peak change in velocity after ITB bolus ( r =.39, P <.05). Baseline Ashworth score correlated inversely with velocity, stride length, cadence, and percentage single support (r range, -.46 to -.57). No significant correlations were found between change in Ashworth score and change in any temporospatial outcome measure. CONCLUSIONS: ITB bolus injection consistently reduces spasticity in ambulatory patients with acquired brain injury but may result in a range of changes in walking performance that can be reliably detected using computerized motion analysis. Velocity appears to be the most sensitive parameter with which to classify individual patient response. The relation between baseline gait velocity and peak change in velocity after bolus administration may have application in predicting the outcome of screening trials for pump implantation; it warrants further investigation.     

Pilot study assessing the impact of intrathecal baclofen administration mode on sleep-related respiratory parameters

Bensmail D, Marquer A, Roche N, Godard A-L, Lofaso F, Quera-Salva M-A. Pilot study assessing the impact of intrathecal baclofen administration mode on sleep-related respiratory parameters.ObjectiveTo assess the impact of intrathecal baclofen (ITB) mode of administration on sleep and sleep-related breathing events in severely disabled patients with severe spasticity.DesignOpen prospective trial.SettingPhysical medicine and rehabilitation department.ParticipantsPatients (N=11) treated with ITB pump for severe spasticity.InterventionAssessment of patients' sleep before and after ITB pump implantation, and comparison of polysomnography results after continuous or bolus mode of administration of ITB.Main Outcome MeasuresPolysomnography and sleep-related breathing events.ResultsITB reduced periodic limb movements and increased the respiratory disturbance index (RDI) and central apneas in our population of patients. This study showed that ITB mode of administration may affect sleep-disordered breathing. Indeed, we observed a significant increase of respiratory events in the bolus condition (RDI and central apneas). In contrast, continuous infusion did not induce a significant modification of sleep-disordered breathing. When a sleep apnea syndrome was preexisting, it was generally severely worsened by the bolus mode of administration.ConclusionsThese results indicate that sleep function and sleep-related respiratory events should be assessed before ITB pump implantation. It is probably better to use a continuous mode of infusion if patients have preexisting sleep-disordered breathing.     

Spasticity in children with cerebral palsy: a retrospective review of the effects of intrathecal baclofen

The administration of baclofen by intrathecal pump is a new technique used to reduce spasticity for individuals with upper motor neuron system injuries. Children with cerebral palsy often have difficulty in mobility because of this form of spasticity. The purpose of this study was to assess the functional outcomes of intrathecal baclofen pump therapy with spasticity in children with cerebral palsy. A retrospective review of medical records for pediatric cerebral palsy patients receiving intrathecal baclofen for intractable spasticity was performed. Of 23 sequential medical records meeting requirements for inclusion in the study, 17 subjects had sufficient recorded data to be included in the study. Data from the medical records included Ashworth scores, therapy complications, and changes in mobility and independence. Although no significant changes in the upper extremities with intrathecal baclofen occurred at one and three months, the trial bolus showed statistically significant changes in mean Ashworth scores. The pre- and posttrial bolus Ashworth scores for the lower extremities showed statistically significant decreases in the posttrial scores and at one and three months when compared with the pretrial scores (p < .001). Complications were resolved with conservative management without long-term sequelae. No infections, respiratory depressions, or deaths occurred as a result of intrathecal baclofen therapy in this study. Although intrathecal baclofen had a significant effect in reducing lower extremity spasticity in children with cerebral palsy, further prospective studies are needed to determine the effects of intrathecal baclofen on such indicators as activities of daily living.     

Continuous intrathecal baclofen in spinal cord spasticity. A prospective study.

Continuous intrathecal infusion of the well known antispastic medication baclofen was evaluated in ten consecutive patients. One year after pump implantation the average Ashworth scale for muscle tone decreased, compared with before treatment, 2.32 points (P < 0.0001), reflexes decreased 2.22 points (P < 0.0001) and the spasm score decreased 1.65 points (P < 0.0001). The average dose increased from 92.22 to 290.95 micrograms (P < 0.0001) between the 1st month of treatment and 1 yr of treatment. The dosage for all patients more than doubled (P < 0.0022) between 3 months and 1 yr postimplantation. There was no significant difference for muscle tone, reflexes or spasms at 3 months v 1 yr. Complications were not unusual and included temporary atelectasis, orthostatic hypotension with escalation of baclofen dose, loss of penile erections, postsurgical pseudo-meningoceles, catheter disruptions and exhausted pump reservoirs. One patient suffered a seizure apparently related to a rapid withdrawal from intrathecal baclofen as a result of catheter sequestration. All patients required a period of intensive inpatient rehabilitation to benefit functionally from the decreased motor tone and/or increased voluntary motor control. The procedure is expensive and close follow-up is necessary for assessing efficacy and refilling the pump. Intrathecal baclofen infusion by subcutaneous pump is useful in treating the effects of spinal spasticity resistant to oral medications. However, there appears to be accommodation to intrathecal baclofen necessitating escalating doses to maintain clinical effects.     

Motor function improvement following intrathecal baclofen pump placement in a patient with locked-in syndrome

We describe a patient with locked-in syndrome who had minimal volitional motor function and severe spasticity in all four extremities. The patient showed a significant improvement in volitional motor function following intrathecal baclofen pump therapy to control spasticity. This case study suggests that intrathecal baclofen pump therapy might improve motor function in select patients with locked-in syndrome.     

电针联合巴氯芬对控制脑卒中痉挛状态的作用

目的:探讨电针刺激与巴氯芬联合治疗脑卒中高痉挛状态的临床效果。方法:脑卒中单侧偏瘫伴痉挛患者58例,随机分为对照组和电针组各29例,均常规给予口服巴氯芬片,中频电刺激患肢痉挛拮抗肌,抗痉挛体位练习及功能训练。电针组同时加用电针刺激,上肢取劣势侧伸肌,下肢取劣势侧屈肌。结果:治疗2个月后,电针组与治疗前及对照组比较,上下肢痉挛评分均明显降低;Barthel评分明显提高(均P<0.01)。结论:电针联合巴氯芬能有效控制脑卒中所至高痉挛状态,有利于患者运动训练的进行,使ADL能力得到提高。     

Instrumental evaluation of gait modifications before and during intrathecal baclofen therapy: a 2-year follow-up case study

Intrathecal baclofen therapy has been used for several years, despite the fact that long-term gait modifications in ambulatory patients have not been thoroughly investigated. A 31-yr-old male patient affected by hereditary spastic paraparesis was evaluated clinically and by gait analysis. Evaluations were made before and at 6, 12, 16, and 24 mos after implantation. The patient showed a clear improvement in self-selected speed, step and stride length, knee and ankle kinematics, and ankle kinetics. Moreover, the response observed on self-selected speed is consistent with the intrathecal baclofen dose administered. To our knowledge, this is the first report of a long-term instrumental gait analysis assessment of a patient receiving intrathecal baclofen. Gait analysis could be a reliable and feasible assessment tool to evaluate ambulatory patients receiving intrathecal baclofen therapy over time and to help clinicians in determining exact dose requirements.     

Symptoms of recurrent intrathecal baclofen withdrawal resulting from drug delivery failure: a case report.

A 24-yr-old, completely (T8) paraplegic male patient presenting with severe spasticity had a drug administration device implanted in April 1991 for continuous intrathecal administration of baclofen. After a period of remarkable improvement in both the spasticity level and his quality of life, the patient experienced several short-lasting episodes of increased spasticity, with severe spasms. Among the possible causes of these deleterious episodes were microcrystalluria, obstipation, a decubitus ulcer, a foreign body in the buttocks, drug tolerance to baclofen, electromagnetic interference, and erroneous filling and programing of the pump. The catheter was the most common source of intrathecal baclofen withdrawal symptoms and had to be changed four times in 5 yr. Intrathecal baclofen administered through an implantable drug administration device is a highly effective but complex and expensive procedure that requires careful patient selection and close monitoring by highly qualified and well-trained health professional. Withdrawal symptoms may be related to noncompliance on the part of the patient, erroneous filling or programing of the pump, depletion of the battery, random component failure, concomitant illness, drug tolerance, or advancement of the disease itself. When failure of the device is suspected, substitution with oral baclofen is recommended until a full work-up is performed to determine the defect.     

Functional outcome after delivery of intrathecal baclofen

Patients with multiple sclerosis or spinal cord injury often have severe, disabling spasticity. This is frequently treated with oral medications or with destructive neurosurgical procedures. We report on a group of patients with spasticity not relieved by these methods. These patients were subsequently treated with intrathecal baclofen delivered by an implanted programmable drug pump. Twenty-one patients have received this form of treatment, and the functional status of eight has been tracked by the Patient Evaluation Conference System (PECS) for at least six months. In most cases, spasticity, performance of bowel and bladder programs, and performance of ADL improved after delivery of intrathecal baclofen. The improvements appear to be due to the decrease in hypertonicity and the increased ease of movement (passive or active) in affected extremities. Intrathecal baclofen should be considered as a treatment method in patients with severe spasticity of spinal origin.     

Intrathecal baclofen in subjects with spastic hemiplegia: assessment of the antispastic effect during gait

OBJECTIVE: To determine whether leg muscle stiffness is measurably reduced after intrathecal baclofen (ITB) in subjects with spastic hemiplegia. DESIGN: Nonrandomized trial. SETTING: Inpatient multidisciplinary rehabilitation unit in France. PARTICIPANTS: Seven consecutive subjects with spastic hemiplegia having Ashworth Scale scores for their quadriceps and triceps greater than 2. INTERVENTION: Subjects were given ITB by lumbar puncture after a dose-selecting test period. MAIN OUTCOME MEASURES: Triceps and quadriceps Ashworth scores, gait analysis at preferred and maximal speed measured by a motion analysis system with 2 forceplates, and electromyographic recording of leg muscles before and 4 hours after ITB. The slopes of the moment-angle curves were measured on the hemiplegic side at the onset of ankle and knee flexion to assess muscle stiffness during walking. Pre- and post-ITB spatiotemporal, kinetic, and kinematic data were compared by using a nonparametric test (Wilcoxon signed-rank test). RESULTS: Ashworth scores of the quadriceps and triceps of all subjects decreased significantly after ITB. Maximal walking speed increased significantly, with a significant increase in stride length, but the preferred walking speed was unchanged. Minimal knee extension and maximal ankle flexion were the only kinematic data significantly different (increased) after ITB. The slope of the ankle moment-angle curve decreased significantly after ITB at preferred gait speed; it also decreased at maximal gait speed in all but 1 subject. Of the 4 available moment-angle curves, 3 showed decreased knee extensor muscle stiffness. The duration of the bursts of spastic muscles decreased after ITB. CONCLUSION: Acute ITB improved walking and reduced muscle stiffness at both the ankles and knees on the spastic hemiplegic side of our subjects. Electromyographic findings suggest that some of the post-ITB reduction in muscle stiffness might be attributed to decreased spasticity.     

Long-term intrathecal (i.t.) infusion of bupivacaine relieved intractable pain and spasticity in a patient with multiple sclerosis

There is no reliable method to relieve both ‘refractory'; pain and spasticity in patients with multiple sclerosis (MS). This paper reports on the long-term use of continuous intrathecal bupivacaine infusion in such a patient. The patient under study was a 56-year-old woman affected for 18 years by MS, unsuccessfully treated with analgesics, baclofen, opioids, peripheral neurolysis (obturator nerves, lumbar plexus) and six intrathecal neurolyses of the L4-S3 nerve roots, each time with 1.5 ml of 50% phenol in glycerol. Intrathecal baclofen was not considered (MS with bulbar location and neurogenic pains). An intrathecal catheter was inserted via the L3-L4 interspace and its tip was placed at the height of the T12-L1 intervertebral disc. An intrathecal infusion of 0.5% bupivacaine at a rate of 3 ml (=15 mg)/day was started. The infusion rate was gradually increased from 20 mg on the first day to 95 mg/day after 68 days. The pain intensity decreased from a mean visual analogue score (VAS/_mean) of 7 before treatment to 1 (on a 0–10 scale) during the intrathecal treatment. The patient became free from pain and spasticity. No side-effects or complications were recorded. The treatment was given for 712 days, at which point the patient died (unrelated to the treatment). Intrathecal infusion of bupivacaine relieved ‘refractory'; spasticity and pain in a MS patient in whom administration of intrathecal baclofen was contraindicated and neurodestructive procedures had been inefficient.     

Intrathecal baclofen for spasticity caused by thrombotic stroke

This study was undertaken to determine the efficacy of intrathecal baclofen therapy in patients who suffer spasticity after a stroke. This case involves a 64-yr-old woman whose thrombotic strokes resulted in severe left-upper and lower-limb spasticity. The patient achieved substantial functional gains as well as improved ease of care, quality of life, and cessation of narcotic use.     

Use of intrathecal baclofen in a pediatric cerebral palsy patient with refractory hemiplegia to maintain orthopaedic surgery gains

Spasticity, a common symptom accompanying cerebral palsy (CP), can severely affect patients' function and cause disability in childhood. Intrathecal baclofen (ITB) therapy is a widely used treatment to reduce spasticity in quadriparetic CP patients. Likewise, adults sustaining strokes and subsequent spastic hemiplegic have proven excellent candidates as well from ITB therapy. However, data on ITB treatment in pediatric patients with hemiplegic CP are lacking. This is the first report to present such a case. A nine-year old girl with spastic hemiplegic CP failed chemical denervation and serial casting of lower extremity spasticity and an associated equinovarus contracture. An ITB bolus test revealed an improvement in her Ashworth score from a mean of 2.8 to 1.2 on the involved side, whereas the unaffected side stayed constant from a mean of 1.3 to 1.2. The patient was subsequently treated with continuous ITB with improvement of Ashworth scores from a mean of 2.8 at baseline to 1.1 at 17 months after implantation of the ITB pump. Subsequent surgery was performed to correct the residual ankle deformity resulting in improvements in passive range of motion, gait function and brace tolerance. Hemiplegic CP pediatric patients can be successfully treated with ITB to reduce spasticity, improve function, and retain postoperative surgical correction without affecting the normal side.