Search

Cerebral palsy (CP) neurologic care and research efforts typically focus on children. However, most people with CP are adults. Adults with CP are at increased risk of new neurologic conditions, such as stroke and myelopathy, that require ongoing neurologic surveillance to distinguish them from baseline motor impairments. Neurologic factors could also contribute to the motor function decline, chronic pain, and chronic fatigue that are commonly experienced by adults with CP. Based on a systematic literature review, we suggest (1) guidelines for neurologic surveillance and neurologist referral and (2) clinical research questions regarding the evolving neurologic risks for adults with CP. ANN NEUROL 2021;89:860-871. [ABSTRACT FROM AUTHOR]

Aim: To compare short‐term outcomes between conus medullaris (conus) and cauda equina (cauda) selective dorsal rhizotomy (SDR) techniques in children with spastic cerebral palsy. Method: This was a retrospective review of SDR at a single center from 2013 to 2017. Gait and functional outcome measures were assessed at no more than 18 months pre‐SDR (baseline) and 8 to 36 months post‐SDR (follow‐up). Transient complications during inpatient stay were quantified. Results: In total, 21 and 59 children underwent conus and cauda SDR respectively. Ashworth Scale scores were nearly normalized at follow‐up. Most physical examination and functional measures exhibited similar baseline to follow‐up responses for both groups. From baseline to follow‐up, sagittal plane knee kinematics for both groups significantly improved (p<0.01) by 11° at initial contact, 9° to 10° in stance phase, and 4° in swing phase. Sagittal plane ankle kinematics improved more for the cauda group than the conus group in both stance phase (10° vs 2°, p<0.01) and swing phase (13° vs 3°, p<0.01). Post‐surgical complications were similar between groups. Interpretation: Conus and cauda SDR techniques resulted in similar short‐term outcomes except in ankle kinematics at follow‐up. The cauda group exhibited a large improvement towards dorsiflexion, while there was residual equinus in the conus group despite Ashworth Scale scores normalizing equally in both groups. What this paper adds: Conus and cauda selective dorsal rhizotomy (SDR) resulted in mostly similar short‐term gait and functional outcomes.Conus SDR resulted in residual equinus dynamically, despite normalized spasticity measures.Post‐surgical complications were mostly similar between SDR techniques. What this paper adds: Conus and cauda selective dorsal rhizotomy (SDR) resulted in mostly similar short‐term gait and functional outcomes.Conus SDR resulted in residual equinus dynamically, despite normalized spasticity measures.Post‐surgical complications were mostly similar between SDR techniques. [ABSTRACT FROM AUTHOR]

Copyright of Developmental Medicine & Child Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Abrupt discontinuation of baclofen can result in a potentially severe withdrawal syndrome. The current treatment for baclofen withdrawal is inadequate, resulting in a critical need to develop an alternative method to prevent or treat this withdrawal syndrome.Objective: To evaluate the safety profile and pharmacokinetics of oral (PO) and investigational intravenous (IV) baclofen formulations at clinically relevant doses.Design: Randomized, open-label, dose-escalation, crossover study.Setting: Contract Research Organization (CRO).Methods: Three cohorts of 12 healthy adults received single doses of PO baclofen (10 mg, 15 mg or 20 mg) and 10-minute infusions of IV baclofen (7.5 mg, 11.5 mg, or 15 mg) with a minimum 48-hour wash-out period. The third cohort also received a 60-minute infusion of 15 mg IV baclofen after an additional 48-hour wash-out period.Main Outcome Measures: Subjects were observed in a CRO for 24 hours after each dose of baclofen, and were assessed for nystagmus, ataxia, and sedation. Blood samples were collected from 0 to 24 hours and analyzed for baclofen concentration using high-performance liquid chromatography-mass spectroscopy. Noncompartmental pharmacokinetic analyses were performed. Dose linearity and proportionality was assessed using 2-way repeated-measures analysis of variance and a power model analysis.Results: None of the PO or IV doses resulted in significant sedation compared to baseline. All subjects could perform tandem gait after each baclofen dose. The most common side effect, transient mild nystagmus, was noted in 4 of 36 and in 13 of 36 subjects after PO and IV administration, respectively. This was likely related to increased maximum concentrations (Cmax). After the 20 mg PO and 15 mg IV doses, mean Cmax levels were 255 and 722 ng/mL and half-lives were 5.24 and 5.79 hours for PO and IV baclofen, respectively. The mean oral bioavailability for the 20-mg PO dose was approximately 80%.Conclusions: All PO and IV doses of baclofen were well tolerated clinically. The 80% bioavailability suggests that a 20% reduction in IV dose will produce comparable total drug exposures to that of the PO dose. When PO therapy is interrupted, bridging with IV baclofen may be feasible.Level Of Evidence: II. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to determine the feasibility and efficacy of five treatments of 6 Hz primed, low-frequency, repetitive transcranial magnetic stimulation (r TMS) combined with constraint-induced movement therapy ( CIMT) to promote recovery of the paretic hand in children with congenital hemiparesis. Method Nineteen children with congenital hemiparesis aged between 8 and 17 years (10 males, nine females; mean age 10 years 10 months, SD 2 years 10 months; Manual Ability Classification Scale levels I- III) underwent five sessions of either real r TMS ( n=10) or sham r TMS ( n=9) alternated daily with CIMT. CIMT consisted of 13 days of continuous long-arm casting with five skin-check sessions. Each child received a total of 10 hours of one-to-one therapy. The primary outcome measure was the Assisting Hand Assessment ( AHA) and the secondary outcome variables were the Canadian Occupational Performance Measure ( COPM) and stereognosis. A Wilcoxon signed-rank sum test was used to analyze differences between pre- and post-test scores within the groups. Analysis of covariance was used to compute mean differences between groups adjusting for baseline. Fisher's exact test was used to compare individual change in AHA raw scores with the smallest detectable difference ( SDD) of 4 points. Results All participants receiving treatment finished the study. Improvement in AHA differed significantly between groups ( p=0.007). No significant differences in the secondary outcome measures were found. Eight out of 10 participants in the r TMS/ CIMT group showed improvement greater than the SDD, but only two out of nine in the sham r TMS/ CIMT group showed such improvement ( p=0.023). No serious adverse events occurred. Interpretation Primed, low-frequency r TMS combined with CIMT appears to be safe, feasible, and efficacious in pediatric hemiparesis. Larger clinical trials are now indicated. [ABSTRACT FROM AUTHOR]

Aim To determine whether intrathecal baclofen (ITB) changes mortality risk in persons with cerebral palsy (CP). Method Records were reviewed for all persons with CP who were managed with ITB for hypertonicity at a specialty hospital in Minnesota between May 1993 and August 2007. A comparison cohort was randomly selected from clients of the California Department of Developmental Services who were initially evaluated between 1987 and 1990 and were matched to those with ITB for age, sex, Gross Motor Function Classification System (GMFCS) level, presence or absence of epilepsy, and feeding-tube use. Survival probabilities were estimated using the Kaplan–Meier method, and differences were tested via log-rank. Results Three hundred and fifty-nine persons with CP (202 males, 157 females) receiving ITB for hypertonicity (mean age 12y 8mo, SD 7y 9mo, range 3y 1mo to 39y 9mo) were matched to 349 persons without ITB pumps (195 males, 154 females; mean age 12y 7mo, SD 8y 4mo, range 2y 7mo to 40y). The proportion of patients at different GMFCS levels in the ITB and in the non-ITB cohorts, respectively, was as follows: level II 3% and 3%, level III 16% and 16%, level IV 38% and 37%, and level V 43% and 44%. Survival at 8 years of follow-up was 92% (SD 1.9%) in the ITB cohort and 82% (SD 2.4%) in the non-ITB cohort ( p<0.001). After adjustment to account for recent trends in improved survival in CP, 8-year survival in the non-ITB cohort was 88%, which was not significantly different from the ITB cohort ( p=0.073). Interpretation ITB therapy does not increase mortality in individuals with CP and may suggest an increase in life expectancy. [ABSTRACT FROM AUTHOR]

Intrathecal baclofen (ITB) is an effective treatment for both spasticity and dystonia in people with cerebral palsy (CP). Its use is becoming increasingly common. ITB is typically associated with fewer side effects than the oral form of the product, but there are risks related to the hardware needed for intrathecal delivery. Much of what has been reported in the literature about ITB is based on experience with children or groups of children and adults; few reports exclusively address its use in adults with CP. These reports indicate that muscle tone is consistently reduced, but there is some variability in functional outcomes. Few well-controlled studies have been done. Controversies remain concerning ITB, including whether a trial is needed before pump implantation, proper catheter tip placement, and programming options, as well as whether it contributes to the development or progression of scoliosis. These and other unanswered questions should be addressed in a systematic way. [ABSTRACT FROM AUTHOR]

This randomized, double-blind, placebo-controlled study compared the efficacy of inhaled nitrous oxide (N2O) with enteral midazolam for sedation of children with cerebral palsy (CP) undergoing botulinum toxin A (BoNT-A) injections. Fifty children (29 males, 21 females; mean age 8y 2mo [SD 4y 5mo]; range 1–16y) were randomized to sedation with N2O ( n=25) or midazolam ( n=25). Groups were similar in type of CP (diplegia, 11; triplegia, three; quadriplegia, 16; hemiplegia, 16; other, three) and Gross Motor Function Classification System level (Level I, 4; II, 24; III, 4; IV, 13; V, 5). Both groups were equally sedated at time of injection ( p=0.661), but those in the midazolam group were more sedated at time of discharge ( p<0.001). N2O was more effective in reducing pain compared with midazolam as measured using the Face, Legs, Activity, Cry, Consolability (FLACC) scale ( p=0.010), parental estimate of pain ( p=0.009), and nursing estimate of pain ( p=0.007). Parents in the N2O group rated it better than prior sedation with midazolam for BoNT-A injections ( p=0.031). Physicians and nurses reported no difference in ease of procedure between the groups. One child in the midazolam group and eight in the N2O group had adverse effects, all of which resolved promptly. N2O appears to be an effective means of sedation for children undergoing outpatient BoNT-A injections. [ABSTRACT FROM AUTHOR]

This study retrospectively evaluated the safety and efficacy of selective dorsal rhizotomy (SDR) in participants who underwent a rigorous selection process, uniform surgical procedure, and a standardized postoperative rehabilitation process. Outcome measures assessed were the Ashworth scale for spasticity, the Gillette Gait Index (GGI) for overall gait pathology, oxygen cost for gait efficiency, and the Gillette Functional Assessment Questionnaire (functional walking ability scale; [FAQ]) for functional mobility. Outcomes were evaluated for 136 children (81 males, 55 females; mean age 7y 3mo [SD 2y 1mo], range 3y 5mo–18y 9mo) for an average of 18.3 months (SD 4.4mo) postoperatively. All participants had a diagnosis of cerebral palsy (CP): 10 quadriplegia, 19 triplegia, and 107 diplegia. Preoperative Gross Motor Function Classification System levels were: Level I n=6; Level II n=64; Level III n=59, and Level IV n=7. All outcome measures improved for the group as a whole. Spasticity improved with 66 to 92% of possible gain in Ashworth scores; GGI was 7.5 times more likely to have a good as opposed to a poor outcome; energy efficiency improved in over half of the participants, and the FAQ demonstrated a statistically significant improvement of 0.9 levels ( p<0.001). The rate of complications was low, with peri- and postoperative complications resolved by time of discharge. [ABSTRACT FROM AUTHOR]