Surgical treatment of spasticity in children: comparison of selective dorsal rhizotomy and intrathecal baclofen pump implantation

Introduction Neurosurgical treatments for spasticity in children include the traditional selective dorsal rhizotomy (SDR) and intrathecal baclofen pumps (ITBPs), which have been widely used in the past decade as an attractive alternative. The purpose of the study was to examine and compare the outcomes of these two procedures in the treatment of children with severe spasticity. Materials and methods A consecutive series of 71 children who underwent SDR for treatment of spasticity was compared with a group of 71 children matched by age and preoperative score on the Gross Motor Function Classification System (GMFCS) who underwent ITBP placement. Change in GMFCS score, lower-extremity tone (based on the Modified Ashworth–Bohannon Scale), and lower-extremity passive range of movement (PROM) at 1 year as well as the need for subsequent orthopedic procedures and parents’ satisfaction were selected as outcome measures. Results At 1 year, both SDR and ITBP decreased tone, increased PROM, and improved function. Both procedures resulted in a high degree of patient satisfaction. Compared with ITBP, SDR provided a larger magnitude of improvement in tone (−2.52 vs −1.23, p < 0.0001), PROM (−0.77 vs −0.39, p = 0.0138), and gross motor function (−0.66 vs −0.08, p < 0.0001). In addition, fewer patients in the SDR group required subsequent orthopedic procedures (19.1 vs 40.8%, p = 0.0106). Conclusions For children with moderate to severe spasticity, SDR and ITBP are both effective surgical treatments. Our results indicate SDR is more effective in reducing the degree of spasticity and improving function than ITBP is in this group of patients.     

Selective dorsal rhizotomy: efficacy and safety in an investigator-masked randomized clinical trial

The objective of this single-center investigator-masked randomized clinical trial was to investigate the efficacy and safety of selective dorsal rhizotomy (SDR) in children with spastic diplegia. Forty-three children with spastic diplegia were randomly assigned on an intention-to-treat basis to receive SDR plus physical therapy (PT), or PT alone. Thirty-eight children completed follow-up through 24 months. Twenty-one children received SDR (SDR+PT group) and 17 received PT (PT Only group). SDR was guided with electrophysiological monitoring and performed by one experienced neurosurgeon. All subjects received equivalent PT. Spasticity was quantified with an electromechanical torque measurement device (spasticity measurement system [SMS]). The Gross Motor Function Measure (GMFM) was used to document changes in functional mobility. Primary outcome measures were collected at baseline, 6, 12, and 24 months by evaluators masked to treatment.
At 24 months, the SDR+PT group exceeded the PT Only group in mean reduction of spasticity by SMS measurement (-8.2 versus +5.1 newton meters/radian, P=0.02). The SDR+PT group and the PT Only group demonstrated similar improvements in independent mobility on the GMFM (7.0 versus 7.2 total percent score, P=0.94). Outcomes on secondary variables were consistent with primary outcomes. There were no serious adverse events. We conclude that SDR is safe and reduces spasticity-in children with spastic diplegia. SDR plus PT and equivalent PT without SDR result in equal improvements in independent mobility at 24 months. SDR may not be an efficacious treatment for children with mild spastic diplegia.     

Epilepsy and intrathecal baclofen therapy in children with cerebral palsy

The objective of this study was to analyze the relationship between epilepsy and intrathecal baclofen by investigating a consecutive sample of 150 children with cerebral palsy or spasticity of cerebral origin who underwent intrathecal baclofen. The medical charts of the 150 children were retrospectively reviewed. A series of 100 children with cerebral palsy, operated on other procedures, was reviewed as a control group. Forty percent of the 150 children had epilepsy before intrathecal baclofen pump implantation; 13.3% had a decrease in seizure frequency after intrathecal baclofen, while two children worsened and one child had seizures ex novo. We conclude that in children with spasticity of cerebral origin, intrathecal baclofen does not seem to aggravate or induce seizure activity.     

Indication, efficiency and complications of intrathecal pump supported baclofen treatment in spinal spasticity.

In 19 patients, who suffered from severe spinal spasticity of different etiologies and did not respond sufficiently to oral antispastic therapy, intrathecal Baclofen test boli were administered. In 11 patients a DAD (Drug Administration Device) [SynchroMedR Model 8611 H, Medtronic Inc. Minneapolis, USA] was implanted. Catheter dislocation or torsion was the most common complication to be observed in these 11 patients. Long term intrathecal Baclofen application was effective in all patients, as reducing spasticity, flexor spasms and spasm induced pain. In some cases the motor performance ameliorated.     

Selective dorsal rhizotomy in cerebral palsy to improve functional abilities: evaluation of criteria for selection

Objectives The aim of this study is to evaluate the effect of selective dorsal rhizotomy (SDR) on functional abilities in a well-defined group of ambulatory children with spastic diplegia.Methods Nine children were selected for SDR (mean age 65 months, range 43–82 months). Gross motor function was measured with the Gross Motor Function Measure (GMFM-88). Self-care was assessed with the Pediatric Evaluation of Disability Inventory (PEDI) and gait pattern was measured with the Edinburgh Visual Gait Score (EGS). There were nine single-case research designs with a 12-month follow-up after surgery.Results After 12 months the mean improvement in the total GMFM-88 scores was 8.8%. On an individual level, all patients improved significantly in comparison with baseline. Functional skills and care-giver assistance measured with the PEDI showed significant improvement. Improvement in gait was also found; in particular, better initial contact and heel-lift resulted in an increased EGS.Conclusion In this well-defined group of ambulatory children SDR had a small but significant positive effect on gross motor function, self-care and gait pattern.     

Experience with external pump trial prior to implantation for intrathecal baclofen in ambulatory patients with spastic cerebral palsy.

OBJECTIVES: To evaluate effectiveness and safety of intrathecal baclofen administration (ITB) testing with continuous infusion via an external pump before the implantation of an internal one in ambulatory spastic patients with cerebral palsy (CP). PATIENTS AND METHODS: Seven CP patients (3 diplegic, 4 quadriplegic - 18.4+/-7.0 years) with a progressive decrease in walking ability were included. Assessments included: Ashworth's scale, Observational Gait Scale (OGS), and GMFM-66. RESULTS: During the ITB test (45-150 microg/24h), spasticity decreased by more than two points on Ashworth's scale (p<0.001) and walking ability improved (median OGS increased from 7 to 9, p相似文献   

Effect of intrathecal baclofen delivered by an implanted programmable pump on health related quality of life in patients with severe spasticity

OBJECTIVES—To compare clinical effectiveness andhealth related quality of life in patients with severe spasticity whoreceived intrathecal baclofen or a placebo.
METHODS—In a double blind, randomised,multicentre trial 22 patients were followed up during 13 weeks andsubsequently included in a 52 week observational longitudinal study.Patients were those with chronic, disabling spasticity who did notrespond to maximum doses of oral baclofen, dantrolene, and tizanidine.After implantation of a programmable pump patients were randomlyassigned to placebo or baclofen infusion for 13 weeks. After 13 weeksall patients received baclofen. Clinical efficacy was assessed by theAshworth scale, spasm score, and self reported pain, and health related quality of life by the sickness impact profile (SIP) and the Hopkins symptom checklist (HSCL).
RESULTS—At three months the scores of the placeboand baclofen group differed slightly for the spasm score (effectsize=0.20) and substantially for the Ashworth scale (effect size=1.40)and pain score (effect size=0.94); health related quality of lifeshowed no significant differences. Three months after implantation the baclofen group showed a significant, substantial improvement on the SIP"physical health", "mental health", "mobility", and"sleep and rest" subscales and on the HSCL mental health scale;patients receiving placebo showed no change. After one year of baclofen treatment significant (P<0.05) improvement was found on the SIP dimensions "mobility" and "body care and movement" withmoderate effect sizes. Improvement on the SIP subscale "physicalhealth" (P<0.05; effect size 0.86), the SIP overall score (without"ambulation"), and the "physical health" and overall scale ofthe HSCL was also significant, with effect sizes >0.80. Changes inhealth related behaviour were noted for "sleep and rest" and"recreation and pastimes" (P<0.01, P<0.05; effect size 0.95 and0.63, respectively). Psychosocial behaviour showed no improvement.
CONCLUSIONS—Intrathecal baclofen delivered by animplanted, programmable pump resulted in improved self reported qualityof life as assessed by the SIP, and HSCL physical health dimensionsalso suggest improvement.

     

Preoperative MRI findings and functional outcome after selective dorsal rhizotomy in children with bilateral spasticity

Purpose  

To identify MRI characteristics that may predict the functional effect of selective dorsal rhizotomy (SDR) in children with bilateral spastic paresis.     

Changes in gait following continuous intrathecal baclofen infusion in ambulant children and young adults with cerebral palsy

Objective: To assess the effect of continuous intrathecal baclofen infusion (ITB) on gait parameters of ambulant children with cerebral palsy (CP).

Methods: The assessment before and 16 months after ITB on seven children with CP (mean age 15 years, SD 5.4) included: Ashworth scale score, range of motion (hip, knee, ankle), Gillette functional assessment questionnaire (FAQ), joint kinematics, spatiotemporal parameters and Gillette Gait Index (GGI).

Results: Gillette FAQ significantly improved from 6.1 to 7.1. Mean GGI improved from 554.50 to 489.25, which was not significant although the improvement was large for three children. Parameters in the sagittal plane were significantly modified with a significant increase in step length (0.65 m to 0.74 m) and a significant increase in hip extension during stance phase (32.25° to 21.6°). These results were correlated to clinical assessments.

Conclusion: ITB seems to improve sagittal gait parameters of children and young adults with CP.     

Selective auditory attention in 3- to 5-year-old children: an event-related potential study

Behavioral and electrophysiological evidence suggests that the development of selective attention extends over the first two decades of life. However, much of this research may underestimate the attention abilities of young children. By providing strong, redundant attention cues, we show that sustained endogenous selective attention has similar effects on ERP indices of auditory processing in adults and children as young as 3 years old. All participants were cued to selectively attend to one of two simultaneously presented stories that differed in location (left/right), voice (male/female), and content. The morphology of the ERP waveforms elicited by probes embedded in the stories was very different for adults, who showed a typical positive-negative-positive pattern in the 300 ms after probe onset, and children, who showed a single broad positivity during this epoch. However, for 3- to 5-year-olds, 6- to 8-year-olds, and adults, probes in the attended story elicited larger amplitude ERPs beginning around 100 ms after probe onset. This attentional modulation of exogenously driven components was longer in duration for the youngest children. In addition, attended linguistic probes elicited a larger negativity 300-500 ms for all groups, indicative of additional attentional processing. These data show that with adequate cues, even children as young as 3 years old can selectively attend to one auditory stream while ignoring another and that doing so alters auditory sensory processing at an early stage. Furthermore, they suggest that the neural mechanisms by which selective attention affects auditory processing are remarkably adult-like by this age.     

Long-term outcome and adverse effects of selective dorsal rhizotomy in children with cerebral palsy: a systematic review

Aim To assess the long‐term outcome and adverse events of selective dorsal rhizotomy (SDR) in children with spastic cerebral palsy (CP). Method Studies were selected based on the following inclusion criteria: children with CP that underwent SDR with a follow‐up period of at least 5 years. The following databases were searched: MEDLINE, Web of Science, Embase, PEDro, and the Cochrane library. Studies meeting the inclusion criteria were scored by two reviewers, who graded the level of evidence and the quality/conduct of the studies. Outcomes were classified according to the International Classification of Functioning, Disability and Health (ICF). Results Only three of the 21 studies who met the inclusion criteria allowed a tentative conclusion on outcome. There is moderate evidence that SDR has a positive long‐term influence on the ICF body structure and body function domains but there is no evidence that SDR has an influence on the ICF activity and participation domains. Spinal abnormalities seem to be common, but no conclusion can be drawn about their relation to SDR. Interpretation There is lack of evidence concerning the long‐term outcomes after SDR. Future studies need to clarify the long‐term influence of SDR – especially in the ICF domains of activity and participation.     

Placebo controlled utility and feasibility study of the H-reflex and flexor reflex in spastic children treated with intrathecal baclofen.

OBJECTIVE: To evaluate feasibility and utility of the soleus H-reflex and tibialis anterior flexor reflex (FR) in identifying spinal cord neuronal response to intrathecal baclofen (ITB) in children with severe spastic cerebral palsy. METHODS: During a randomized, double-blind, placebo-controlled dose-escalation test treatment, maximum H amplitude/maximum M amplitude (H/M ratio) and FR parameters were bilaterally recorded at baseline and 2-3 h after intrathecal bolus administration of placebo and increasing doses of baclofen until both an improvement in the individual treatment goal(s) and a one-point reduction on the Ashworth scale were observed. RESULTS: Electrophysiological data of 14 children were studied. The H-reflex was feasible in 13 children, the FR threshold area in 9 and the FR, elicited with supramaximal stimulation, in only one child. After ITB, the H/M ratio significantly decreased (left: 0.67+/-0.47 to 0.15+/-0.18, P=0.005; right: 0.55+/-0.32 to 0.14+/-0.19, P=0.002) without placebo effect. FR threshold area after ITB, only decreased significantly in children not taking oral baclofen (left: 146+/-53 to 41+/-54 mV ms, P=0.000; right: 156+/-80 to 66+/-48 mV ms, P=0.002). CONCLUSIONS: This is the first randomized, double-blind, placebo-controlled dose-escalation study in spastic children demonstrating the soleus H-reflex to be a feasible and objective measure to quantify the decreasing motoneuron excitability in response to ITB bolus administration. Only in children not taking oral baclofen, FR threshold area can also be used as an objective outcome measure, yet feasibility is limited. SIGNIFICANCE: We suggest introducing the H-reflex as the electrophysiological gold standard for the evaluation of the effect of ITB in spastic children.     

Selective breeding of two rat lines differing in sensitivity to GHB and baclofen

Two Wistar-derived rat lines, one sensitive (GHB-S) and the other resistant (GHB-R) to the anesthetic effect of gamma-hydroxybutyric acid (GHB), have been selectively bred. GHB-S and GHB-R rats were also sensitive and resistant, respectively, to the anesthetic effect of baclofen, the prototype GABA(B) receptor agonist, suggesting that they may be useful to elucidate not only the role of endogenous GHB but also that of GABA(B) receptors in sleep and anesthesia.     

Use of intrathecal baclofen therapy in ambulant children and adolescents with spasticity and dystonia of cerebral origin: a systematic review

Aim Studies on the use of intrathecal baclofen (ITB) for ambulant adults with spasticity and/or dystonia of cerebral origin are scarce, and are even more limited for children and adolescents. This systematic review investigates the use of ITB to improve walking, transfer ability, and gross motor activities in ambulant children and adolescents with spasticity and/or dystonia of cerebral origin. Method Electronic databases (MEDLINE, CINAHL, PsycINFO, EMBASE, full Cochrane Library, and PEDro) were searched from the earliest date available until March 2011 using combined subject headings and free text if supported by the databases. Studies were included if they had examined individuals who: (1) received ITB therapy by any method (bolus injection, an external delivery system, or an implanted pump); (2) had spasticity and/or dystonia of cerebral origin; (3) were able to ambulate with or without a walking device, i.e. individuals with cerebral palsy (CP) who were in levels I to III of the Gross Motor Function Classification System or individuals with similar functional mobility if they did not have CP; and (4) were aged 18 years or under. Publications in English in peer‐reviewed journals reporting any type of research design, except reviews and expert opinions, were included. Studies were excluded if participants had spasticity and/or dystonia of spinal origin and if baclofen was administered only orally. Studies that compared ITB with other interventions such as botulinum toxin were also excluded. Results Two independent reviewers scored 16 studies against the guidelines for developing systematic reviews from the American Academy of Cerebral Palsy and Developmental Medicine (AACPDM). Interpretation Fifteen studies were of levels IV or V evidence and only one of level II according to the evidence levels of the AACPDM guidelines, but all were of low quality. No study was found on the use of ITB in ambulant children or adolescents with dystonia of cerebral origin. Not all studies used objective outcome measures to assess the ambulation, transfer ability, and gross motor activities of the participants. A proportion of participants showed improvement in all these areas but adverse events were common. A proportion of participants compromised their ambulatory and transfer abilities after ITB. There was no evidence to support the clinical use of ITB in ambulant individuals with hypertonicity without further rigorous longitudinal studies.     

Near-infrared spectroscopy as an alternative to the Wada test for language mapping in children, adults and special populations.

The intracarotid amobarbital test (IAT) is the most widely used procedure for pre-surgical evaluation of language lateralization in epileptic patients. However, apart from being invasive, this technique is not applicable in young children or patients who present mental retardation and/or language deficits. Functional magnetic resonance imaging (fMRI) is increasingly employed as a non-invasive alternative. Again, this method is more difficult to use with young children, especially hyperactive ones, since they have to remain motionless during data acquisition. The aim of this study was to determine whether near-infrared spectroscopy (NIRS) can be used as an alternative technique to investigate language lateralization in children and special populations. Unlike Wada test, NIRS is non-invasive, and it is more tolerant to movement artefacts than fMRI. In the present study, NIRS data were acquired in four epileptic children, a 12-year-old boy with pervasive developmental disorder and a 3-year-old, healthy child, as well as three healthy and two epileptic adults, while they performed a verbal fluency task and a control task. When applicable, the results were compared to the subjects' fMRI and/or IAT findings. Clear laterality of speech was obtained in all participants, including the two non-epileptic children, and NIRS results matched fMRI and IAT findings. These results, if replicable in larger samples, are encouraging and suggest that NIRS has the potential to become a viable, non-invasive alternative to IAT and fMRI in the determination of speech lateralization in children and clinical populations that cannot be submitted to more invasive techniques.     

Comparison of the Gross Motor Function Measure and Paediatric Evaluation of Disability Inventory in assessing motor function in children undergoing selective dorsal rhizotomy

This study was designed to compare assessment with the functional outcome measures Gross Motor Function Measure (GMFM) and Pediatric Evaluation of Disability Inventory (PEDI) over time, in children with cerebral palsy (CP) undergoing selective dorsal rhizotomy combined with individualised physiotherapeutic interventions. Using the Gross Motor Function Classification System (GMFCS), 18 children with spastic diplegia were divided into two groups according to age-related severity of motor function impairment. Data were collected preoperatively, and at 6 and 12 months postoperatively. Both instruments were sensitive to changes in function over time in the series as a whole and in the group with milder impairment, although the PEDI detected significant changes earlier. In the group with more severe impairment, changes in function were detected only with the PEDI, not with the GMFM. Thus, the instruments are to be considered complementary tests, because they measure different aspects of function.