Linda E. Krach

Intrathecal Baclofen for Management of Spastic Cerebral Palsy: Multicenter Trial

Intrathecal baclofen infusion has demonstrated effectiveness in decreasing spasticity of spinal origin. Oral antispasticity medication is minimally effective or not well tolerated in cerebral palsy. This study assessed the effectiveness of intrathecal baclofen in reducing spasticity in cerebral palsy. Candidates were screened by randomized, double-blind, intrathecal injections of baclofen and placebo. Responders were defined as those who experienced an average reduction of 1.0 in the lower extremities on the Ashworth Scale for spasticity. Responders received intrathecal baclofen via the SynchroMed System and were followed for up to 43 months. Fifty-one patients completed screening and 44 entered open-label trials. Lower-extremity spasticity decreased from an average baseline score of 3.64 to 1.90 at 39 months. A decrease in upper-extremity spasticity was evidenced over the same study period. Forty-two patients reported adverse events. Most common reports were hypotonia, seizures (no new onset), somnolence, and nausea or vomiting. Fifty-nine percent of the patients experienced procedural or system-related events. Spasticity in patients with cerebral palsy can be treated effectively by continuous intrathecal baclofen. Adverse events, although common, were manageable. (J Child Neurol 2000;15:71-77).

Pharmacotherapy of Spasticity: Oral Medications and Intrathecal Baclofen

Spasticity is a common problem in children with neurologic impairment, particularly in those with cerebral palsy. Clinicians commonly make use of oral medications to attempt to reduce spasticity and increase function. Little has been published in the literature concerning the use of these medications in children and their effects on both muscle tone and function. This article is a review of the sites of action, side effects, and efficacy of benzodiazepines, baclofen, dantrolene sodium, alpha2-adrenergic agonists, and gabapentin. A brief review of intrathecal baclofen is included as well. (J Child Neurol 2001;16:31-36).

Primed low-frequency repetitive transcranial magnetic stimulation and constraint-induced movement therapy in pediatric hemiparesis: a randomized controlled trial.

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 (rTMS) combined with constraint‐induced movement therapy (CIMT) to promote recovery of the paretic hand in children with congenital hemiparesis.

Comprehensive short-term outcome assessment of selective dorsal rhizotomy.

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.

GMFM 1 year after continuous intrathecal baclofen infusion

The purpose of this study was to assess whether there is an improvement in motor function in persons with cerebral palsy (CP) who have had a reduction of muscle tone by continuous intrathecal baclofen infusion. This was a prospective, open label, non-blinded case series without a control group, conducted at multiple centres. There were 31 subjects, aged 4–29 years. All had a pre-treatment mean lower extremity Ashworth scores of  ≥ 3 and a significant reduction in tone after a bolus injection of intrathecal baclofen (ITB) and received an implanted pump for continuous delivery of ITB. Motor function was assessed by the Gross Motor Function Measure (GMFM) prior to and 1 year following pump implantation. Significant improvement (p < 0.05) in mean GMFM scores was seen in subjects < 8 years (mean change 4.1) and in those from 8–18 years (mean change 3.7) and in subjects with CP Classes 2 and 5 (mean changes 6.2 and 2.9). There was a statistically significant decrease (p < 0.05) in Ashworth scores in CP classes 2–5. Subjects or their caregivers that completed a survey about perceived changes stated that motor control, positioning and endurance improved.

Late improvements in mobility after acquired brain injuries in children.

The purpose of this investigation was to describe the late improvements in functional mobility in children who have sustained severe acquired anoxic or traumatic brain injuries. Ninety-eight children from a consecutive series of 199 with acquired brain injuries met inclusion criteria. As expected, children with traumatic injuries had better mobility at time of discharge from rehabilitation than did children with anoxic injury. In addition, children with traumatic injuries improved more in mobility status during the first 2 years after injury than did children with anoxic injury. The children who continued to improve in mobility after discharge were unconscious for a shorter time. Children who became community ambulators during the first year after discharge had higher mobility ratings at discharge. Although children who eventually achieved more functional mobility were admitted to rehabilitation service sooner after injury, discriminant analysis revealed that the duration of unconsciousness, and therefore severity of injury, was more closely correlated with the final mobility status.

Safety and Feasibility of Transcranial Direct Current Stimulation in Pediatric Hemiparesis: Randomized Controlled Preliminary Study

Background Transcranial direct current stimulation (tDCS) is a form of noninvasive brain stimulation that has shown improved adult stroke outcomes. Applying tDCS in children with congenital hemiparesis has not yet been explored. Objective The primary objective of this study was to explore the safety and feasibility of single-session tDCS through an adverse events profile and symptom assessment within a double-blind, randomized placebo-controlled preliminary study in children with congenital hemiparesis. A secondary objective was to assess the stability of hand and cognitive function. Design A double-blind, randomized placebo-controlled pretest/posttest/follow-up study was conducted. Setting The study was conducted in a university pediatric research laboratory. Participants Thirteen children, ages 7 to 18 years, with congenital hemiparesis participated. Measurements Adverse events/safety assessment and hand function were measured. Intervention Participants were randomly assigned to either an intervention group or a control group, with safety and functional assessments at pretest, at posttest on the same day, and at a 1-week follow-up session. An intervention of 10 minutes of 0.7 mA tDCS was applied to bilateral primary motor cortices. The tDCS intervention was considered safe if there was no individual decline of 25% or group decline of 2 standard deviations for motor evoked potentials (MEPs) and behavioral data and no report of adverse events. Results No major adverse events were found, including no seizures. Two participants did not complete the study due to lack of MEP and discomfort. For the 11 participants who completed the study, group differences in MEPs and behavioral data did not exceed 2 standard deviations in those who received the tDCS (n=5) and those in the control group (n=6). The study was completed without the need for stopping per medical monitor and biostatisticial analysis. Limitations A limitation of the study was the small sample size, with data available for 11 participants. Conclusions Based on the results of this study, tDCS appears to be safe, feasible, and well tolerated in most children with hemiparesis. Future investigations of serial sessions of tDCS in conjunction with rehabilitation in pediatric hemiparesis are indicated to explore the benefit of a synergistic approach to improving hand function.

Outcome of severe anoxic/ischemic brain injury in children

The outcome of 25 children who had anoxic or ischemic brain injuries at 2 months to 14 years of age is reported. Follow-up was from 1 to 14 years after injury; causes were near-drowning, 11; suffocation, 7; cardiac arrest, 3; electrocution with cardiac arrest, 2; strangulation, 1; aborted sudden infant death syndrome, 1. All patients were unconscious for at least 24 hours. Of 11 remaining in vegetative states, 5 died. Seven children regained some language skills and are in special education or self-contained classrooms. Seven are profoundly impaired and show only a social smile. Cognitive and motor outcomes were correlated with the severity of injury as indicated by the duration of unconsciousness. All children who regained language skills or the ability to walk were unconscious less than 60 days. Dystonic rigidity was observed in all children who were nonambulatory. Outcome was also correlated with the cause of injury; mortality, cognitive outcome, feeding outcome, and duration of unconsciousness were all worse in children with near-drowning.

Kluver-Bucy syndrome following heat stroke in a 12-year-old girl

A 12-year-old girl developed Kluver-Bucy syndrome (KBS) following heat stroke. She demonstrated the features typical of human KBS, including visual agnosia, hypermetamorphosis, hypersexuality, language disorder with aphasia, hyperorality, placidity, flat affect, and memory dysfunction. Magnetic resonance imaging 11 1/2 months after onset disclosed mild, diffuse atrophy. Fourteen months after onset, she did not consistently respond to language or communicate verbally, and was dependent on others. A comparison is made with previously reported KBS cases in children.

Gram‐negative meningitis and infections in individuals treated with intrathecal baclofen for spasticity: a retrospective study

The aim of this retrospective study was to describe signs, symptoms, and clinical outcomes of individuals undergoing intrathecal baclofen (ITB) therapy who experienced pump-related Gram-negative infections including meningitis. Participants included 12 individuals (nine males, three females) aged 10 to 32 years (mean 17y 9mo), nine of whom had quadriplegic CP. A total of 571 baclofen pump surgeries were performed with 45 total infections. Of the 45 infections, 12 were by Gram-negative organisms, two resulting in meningitis. Ten of 12 Gram-negative infections (21 site encounters) occurred within 60 days of surgery. Eleven of 12 pumps were explanted. By site encounters, Pseudomonas aeruginosa accounted for eight Gram-negative infections, Escherichia coli for five, Proteus for three, Enterobacter cloacae for two, and Klebsiella, Enterobacter aerogenes, and Enterobacter vulnaris for one each. Two individuals with Gram-negative meningitis were admitted 72 to 96 hours after hospital discharge following pump replacement. Both patients had rapid deterioration requiring transfer to the pediatric intensive care unit, and developed coagulopathy and decrease in responsiveness. Both have improved and have elected not to replace the ITB pump. In Gram-negative infections in ITB therapy, the progression of signs and symptoms can be swift and devastating. Identification of the infectious agent in such cases is imperative; these infections can quickly become life threatening.