Use of botulinum toxin type A in the management of neonatal brachial plexus palsy.

OBJECTIVE: To evaluate functional outcomes and the impact on surgical interventions after the use of botulinum neurotoxin type A (BoNT-A) for muscle imbalance, cocontractions, or contractures with neonatal brachial plexus palsy. DESIGN: A retrospective cohort study. SETTING: A brachial plexus center in a tertiary children's hospital. PARTICIPANTS: Fifty-nine patients with neonatal brachial plexus palsy (75 injection procedures, 91 muscles and/or muscle groups) received BoNT-A injections (mean age at injection, 36.2 months; range, 6-123 months; 31 boys; 30 right-sided injuries, 28 left-sided injuries, 1 bilateral injury). METHODS: Data collected retrospectively from medical records, from procedure notes and clinic visits before BoNT-A use, at ≤6 months follow-up (BoNT-A active [BA]) and at ≥7 months follow-up (BoNT-A not active [BNA]) included demographics, injection indication, side, and site(s), previous surgical history, occupational therapy and/or physical therapy plan, and outcome measurements. MAIN OUTCOME MEASUREMENTS: Outcomes assessed before and after injections included active and passive range of motion, Mallet and Toronto scores, parent comments about arm function, preinjection surgical considerations, and postinjection surgical history. RESULTS: Injection procedures included 51 to shoulder internal rotators, 15 triceps, 15 pronator teres, 9 biceps, and 1 flexor carpi ulnaris. Active and passive shoulder external rotation (SER) range of motion improved after shoulder internal rotator injections (P = .0003 and P = .002, respectively), as did Mallet scores with BA; the latter were sustained with BNA. Surgical intervention was averted, modified, or deferred after BoNT-A in 45% (n = 20) under surgical consideration before BoNT-A. Active elbow flexion improved in 67% (P = .005), sustained BNA (P = .004) after triceps injections; 2 of 7 patients averted surgery. Active supination improved with BA (P = .002), with gains sustained BNA (P = .016). Passive elbow extension improved after biceps injections by an average 17° (P = .004) BA, although not sustained BNA. CONCLUSIONS: BoNT-A is an effective adjunct to therapy and surgery in managing muscle imbalance, cocontractions, and contractures in neonatal brachial plexus palsy. Use of BoNT-A can result in averting, modifying, or deferring surgical interventions in a number of affected children.

Clinical predictors of outcome following inflicted traumatic brain injury in children.

BACKGROUND: The study aimed to determine which acute injury variables were predictors of long-term functional outcome following inflicted traumatic brain injury (iTBI). METHODS: A retrospective case review of 35 children with iTBI was performed. After controlling for age at injury and time since injury, the generalized estimation equations method was used to identify acute injury variables that were significantly related to the Glasgow Outcome Scale scores at the initial follow-up assessments. When available, functional sequelae at these and longer-term follow-ups were also examined. RESULTS: In bivariate generalized estimation equations analyses, a low Glasgow Coma Scale (GCS) eye component score, a low GCS motor component score, a low GCS verbal component score, need for neurosurgical intervention, seizures in the first week after injury, need for mechanical ventilation for more than 10 days, length of intensive care unit stay of more than 10 days, initial hyperglycemia, and neuroimaging findings of cerebral edema or loss of gray-white matter differentiation were significantly (p ≤ 0.05) related to having a poor outcome, as defined by their Glasgow Outcome Scale score at the initial follow-up. In multivariable analyses, considering the significant predictors while controlling for age at injury and time since injury, the presence of cerebral edema on neuroimaging (odds ratio, 27.21; 95% confidence interval, 4.40-168.22), and length of intensive care unit stay of more than 10 days (odds ratio, 21.57; 95% confidence interval, 3.09-150.48) were significantly related to having a poor outcome. CONCLUSION: Early clinical data following iTBI help predict long-term functional outcome. Further research to support these findings may help delineate acutely after injury which children with iTBI are at risk for a poor prognosis and should be more closely followed up over time. LEVEL OF EVIDENCE: Prognostic study, level IV.

Recommendations for the use of common outcome measures in pediatric traumatic brain injury research.

This article addresses the need for age-relevant outcome measures for traumatic brain injury (TBI) research and summarizes the recommendations by the inter-agency Pediatric TBI Outcomes Workgroup. The Pediatric Workgroup's recommendations address primary clinical research objectives including characterizing course of recovery from TBI, prediction of later outcome, measurement of treatment effects, and comparison of outcomes across studies. Consistent with other Common Data Elements (CDE) Workgroups, the Pediatric TBI Outcomes Workgroup adopted the standard three-tier system in its selection of measures. In the first tier, core measures included valid, robust, and widely applicable outcome measures with proven utility in pediatric TBI from each identified domain including academics, adaptive and daily living skills, family and environment, global outcome, health-related quality of life, infant and toddler measures, language and communication, neuropsychological impairment, physical functioning, psychiatric and psychological functioning, recovery of consciousness, social role participation and social competence, social cognition, and TBI-related symptoms. In the second tier, supplemental measures were recommended for consideration in TBI research focusing on specific topics or populations. In the third tier, emerging measures included important instruments currently under development, in the process of validation, or nearing the point of published findings that have significant potential to be superior to measures in the core and supplemental lists and may eventually replace them as evidence for their utility emerges.

Arthroscopically assisted Sever-L'Episcopo procedure improves clinical and radiographic outcomes in neonatal brachial plexus palsy patients.

BACKGROUND: Muscle pathology resulting in internal rotation contractures in children with neonatal brachial plexus palsy places abnormal stresses on the glenohumeral joint and limits global shoulder function. The objective of this study was to assess the clinical and radiographic outcomes in children treated with an arthroscopic release with or without tendon transfer, the so-called arthroscopically assisted Sever-L'Episcopo procedure. METHODS: Fifty children with an average age of 5.1 years who underwent an arthroscopic release with or without tendon transfer were retrospectively reviewed. Clinical outcomes were assessed using Mallet classification scores, whereas glenoid retroversion and posterior humeral head subluxation were measured on magnetic resonance images to quantify radiographic outcomes. Mean clinical follow-up was 30 months (range: 24 to 65 mo) and mean radiographic follow-up was 24 months (range: 11 to 42 mo). RESULTS: Aggregate Mallet score improved significantly from 12.6 to 16.3 (P<0.0001), with shoulder abduction from 3.4 to 3.8 (P=0.0007), shoulder external rotation from 2.2 to 3.3 (P<0.0001), hand-to-neck from 2.3 to 3.2 (P<0.0001), and hand-to-mouth from 2.3 to 3.3 (P<0.0001). Hand-to-spine Mallet score did not significantly change from preoperative (2.4) to postoperative (2.6) (P=0.1348), although 4 patients experienced a loss in internal rotation function. Forty-eight percent of children improved by at least 4 points on the total Mallet score. Glenoid retroversion improved from 25 to 14.1 degrees (P<0.0001) and percent humeral head anterior to the central axis of the scapula increased from 30.5% to 38.8% (P=0.0001). Sixty-seven percent of patients demonstrated glenohumeral joint remodeling on magnetic resonance imaging. No child exhibited a worsening of glenohumeral anatomy. CONCLUSIONS: An arthroscopic release with or without tendon transfer is effective in reducing internal rotation contractures and increasing global shoulder function. Both clinical and radiographic outcomes were significantly improved at 2-year follow-up. Furthermore, in the majority of children, aggregate, abduction, and external rotation Mallet scores all increased without sacrificing internal rotation. LEVEL OF EVIDENCE: Therapeutic Level IV.

EMG and MRI are independently related to shoulder external rotation function in neonatal brachial plexus palsy.

BACKGROUND: Few studies exist with regard to the ability of electromyography (EMG) and volumetric magnetic resonance imaging (MRI) of the infraspinatus muscle to complement the physical assessment of active global shoulder external rotation (GER) in the neonatal brachial plexus palsy (NBPP) population. Therefore, the purpose of this study was to evaluate the relationships of EMG and MRI with active GER based on analysis of the infraspinatus muscle. METHODS: Seventy-four NBPP patients (mean age, 5 y 1 m; range, 1 y 1 m to 13 y 3 m) who had undergone physical examination of the shoulder, EMG evaluation of the infraspinatus muscle, and shoulder MRI were included in this study. The outcome variable active GER was dichotomized into <0 degree active GER (poor) and ≥0 degree active GER (good). The interference pattern on EMG of the infraspinatus muscle was graded on a 6-point scale and dichotomized into ≤4 and ≥5. On shoulder MRI, infraspinatus muscle volume was measured. The infraspinatus muscle interference pattern and volume were compared with active GER. RESULTS: Interference pattern on EMG of the infraspinatus muscle was significantly related to the Mallet Score (P=0.0022), with a poor interference pattern associated with an approximately 7 times higher likelihood [odds ratio=7.391; 95% confidence interval (2.054, 26.588)] of poor active GER. Infraspinatus muscle volume decrease on MRI was also significantly related to active GER (P=0.0413), with each percent volume decrease corresponding to an increase of 0.094 in the odds of having a poor Mallet Score for active GER [odds ratio=1.094; 95% confidence interval (1.004, 1.193)]. CONCLUSIONS: The interference pattern of the infraspinatus muscle on EMG and the infraspinatus muscle volume on MRI are strongly related to active GER as assessed by the Mallet Score. Integrating clinical assessment with electrophysiological and imaging findings may improve the accuracy in evaluating shoulder dysfunction in NBPP and provide improved guidance in selecting interventions specific to the patient's pattern of deficits. LEVEL OF EVIDENCE: Diagnostic study, level II.

Pharmacokinetics of amantadine in children with impaired consciousness due to acquired brain injury: preliminary findings using a sparse-sampling technique.

OBJECTIVE: To evaluate the pharmacokinetics of amantadine in children with impaired consciousness from acquired brain injury. DESIGN: Randomized, double-blind, placebo-controlled, crossover study with sparse sampling for pharmacokinetics. SETTING: Tertiary care pediatric hospital. PARTICIPANTS: Children, ages 6-18 years, with impaired consciousness 5-10 weeks after acquired brain injury. METHODS: Subjects received amantadine for 3 weeks. Subjects were randomized to placebo or amantadine 4 mg/kg/day for 7 days followed by 6 mg/kg/day for 14 days. Crossover was after a 7-day washout period. MAIN OUTCOME MEASURES: The Coma/Near-Coma Scale and Coma Recovery Scale-Revised were done 3 times per week to evaluate arousal and consciousness. Plasma concentrations of amantadine were determined for pharmacokinetic parameter estimation and evaluation of the exposure-response relationship. Adverse events were monitored. RESULTS: Nine subjects met the final inclusion and exclusion criteria, 7 of whom agreed to participate. Five subjects completed both arms of the study. Amantadine total body clearance was 0.17 L/h/kg with a half-life of 13.9 hours. Higher exposure of amantadine (average concentration of amantadine during 6 mg/kg/day > 1.5 mg/L) may be associated with better recovery of consciousness. CONCLUSIONS: Amantadine was well-tolerated in children with acquired brain injury and demonstrates pharmacokinetics similar to those reported for healthy young adults. Based on the preliminary data, higher dosing may be considered in the setting of brain injury.

Effects of amantadine in children with impaired consciousness caused by acquired brain injury: a pilot study.

OBJECTIVE: To conduct a pilot study of amantadine in children with impaired consciousness caused by acquired brain injury, to establish design feasibility, and to assess the effect on level of arousal and consciousness. DESIGN: Randomized, double-blind, placebo-controlled crossover trial. Seven subjects (mean age, 12.7 yrs) with an acquired brain injury (mean duration, 6 wks) were randomized to receive either 3 wks of placebo or amantadine, followed by a 1-wk washout period and then 3 wks of the other agent. Main outcome measures were the Coma/Near-Coma Scale and Coma Recovery Scale-Revised, each done three times per week. Subjective evaluations of change in arousal and consciousness by the parent and physician were done weekly. RESULTS: Five subjects completed the study. There was no significant difference in the slopes of recovery during either arm for the Coma/Near-Coma Scale (P = 0.24) or the Coma Recovery Scale-Revised (P = 0.28), although improvements in consciousness were noted by the physician during weeks when amantadine was given (P = 0.02). CONCLUSIONS: This study suggests that amantadine facilitates recovery of consciousness in pediatric acquired brain injury and provides important information necessary to design future more definitive studies.

Late proton magnetic resonance spectroscopy following traumatic brain injury during early childhood: relationship with neurobehavioral outcomes.

We sought to extend previous research that demonstrates reduced neurometabolite concentrations during the chronic phase of pediatric traumatic brain injury (TBI) in children injured during early childhood. We hypothesized that young children with TBI in the chronic phase post-injury would have lower N-acetyl aspartate (NAA) metabolite concentrations in gray and white matter in comparison to controls. We also hypothesized that metabolite levels would be correlated with acute TBI severity and neurobehavioral skills. Ten children with a history of TBI between the ages of 3 and 6 years were compared to an age, gender, and race-matched group of 10 children with a history of an orthopedic injury (OI). Children completed neurobehavioral testing at 12 months post-injury. Proton magnetic resonance (MR) spectroscopy was completed at least 12 months post-injury when the children were 6-9 years old. Groups were compared on metabolite concentrations in the medial frontal gray matter and left frontal white matter. Metabolite levels were correlated with Glasgow Coma Scale (GCS) scores and neurobehavioral functioning. There was a trend for lower NAA concentrations in the medial frontal gray matter for the TBI group. Late NAA and Cr levels in the medial frontal gray matter and NAA levels in the left frontal white matter were strongly positively correlated with initial GCS score. Metabolite levels were correlated with some neurobehavioral measures differentially for children with TBI or OI. Some neurometabolite levels differed between the TBI and OI groups more than 1 year post-injury and were related to injury severity, as well as some neurobehavioral outcomes following TBI during early childhood.

Neural substrate differences in language networks and associated language-related behavioral impairments in children with TBI: a preliminary fMRI investigation.

The present study examined whether functional MRI (fMRI) can identify changes in the neural substrates of language in young children following traumatic brain injury (TBI). Eight children with TBI (F/M=3/5, age (Mean +/- SD)=7.98 +/- 1 years, range = 6-9 years) and a comparison group of nine children with orthopedic injuries (OI) (F/M=4/5, age (Mean +/- SD)=7.4 +/- 1 years, range=6-9 years) participated in an fMRI study of covert verb generation (VG). Results revealed significantly different BOLD signal activation in perisylvian language areas between the groups, after accounting for potential confounders such as verbal fluency and executive function. We also found significant associations between the BOLD signal activation and performance on language-specific neuropsychological tests (NEPSY verbal fluency score, Verbal IQ) and Glasgow Coma Scale (GCS) score. This study suggests that children with TBI have significantly different brain activation patterns in language circuitry compared to children with orthopedic injuries. Although we found clear differences in brain activation between the two groups, conventional MR images showed no evidence of structural abnormalities in five of eight children with TBI. Our study demonstrates the feasibility and potential utility of fMRI as a means of quantifying changes associated with language deficits in future pediatric TBI studies.

Prescribing therapy services for children with motor disabilities.

Pediatricians often are called on to prescribe physical, occupational, and speech-language therapy services for children with motor disabilities. This report defines the context in which rehabilitation therapies should be prescribed, emphasizing the evaluation and enhancement of the child's function and abilities and participation in age-appropriate life roles. The report encourages pediatricians to work with teams including the parents, child, teachers, therapists, and other physicians to ensure that their patients receive appropriate therapy services.