Safety of botulinum toxin injections in children less than one year old: A retrospective chart review.

PURPOSE: Infants can have muscle hypertonia due to cerebral palsy, muscle strength imbalances due to brachial plexus palsy, refractory clubfoot, and torticollis. These muscle problems can cause significant development impairments. A child with severe sialorrhea and dysphagia from leukodystrophy can aspirate, causing respiratory problems. Botulinum toxin (BoNT) injections can improve these conditions but may lead to adverse effects from the toxin spreading to non-targeted muscles, potentially impacting breathing, swallowing, and overall strength. This is particularly concerning in infants. This study assessed the safety of BoNT injections in children less than one year of age. METHODS: This was a retrospective cohort study. RESULTS: Forty-seven patients (22 male, 25 female) received BoNT injections before one year of age (three to 12 months). Thirty-seven received one round of injections and 10 were injected on multiple occasions. Forty-five received onabotulinumtoxinA (15-100 units [U], 1.9-15.2 U/kg), one received abobotulinumtoxinA (70 U, 9.0 U/kg), and one received incobotulinumtoxinA (25 U, 3.5 U/kg). Lower extremities were treated in 15 patients, upper extremities in 38, the sternocleidomastoid in two, and the salivary glands in one. Forty-five patients had no reported complications. One experienced transient fever, vomiting, and diarrhea. The parent of another reported subjective weakness in one muscle. CONCLUSION: BoNT injections in children less than one year of age appear to be safe.

Acquired amputations in patients with spina bifida.

PURPOSE: The purpose of this study was to assess if the incidence of lower extremity (LE) pressure injuries resulting in amputations was more frequent in ambulatory versus non-ambulatory patients with spina bifida. METHODS: The medical records of all patients at a large pediatric rehabilitation hospital who had spina bifida and a history of a pressure injury between 1997-2018 were retrospectively reviewed. RESULTS: In this study, 112 patients with spina bifida developed LE pressure injuries, 53 patients walked with braces and/or an assistive device, and 59 patients used a wheelchair for mobility. Of the 11 patients who had amputations, 10 walked and 1 did not walk prior to their amputation. Ambulatory patients with LE pressure injuries had a 19% chance (10 out of 53 patients) of requiring an amputation. Non-ambulatory patients with a LE pressure injury had only a 2% chance (1 out of 59 patients) of requiring an amputation. Ambulatory patients with LE pressure injuries were at a much higher risk of requiring an amputation than patients who did not walk (p-value of < 0.005). CONCLUSION: Patients with spina bifida who are ambulatory and develop a pressure injury have a high risk of ultimately needing an amputation and should be monitored and treated aggressively.

Management of hypertonia in cerebral palsy.

PURPOSE OF REVIEW: The review provides an update on the treatment of hypertonia in cerebral palsy, including physical management, pharmacotherapy, neurosurgical, and orthopedic procedures. RECENT FINDINGS: Serial casting potentiates the effect of Botulinum neurotoxin A injections for spasticity. Deep brain stimulation, intraventricular baclofen, and ventral and dorsal rhizotomy are emerging tools for the treatment of dystonia and/or mixed tone. The long-term results of selective dorsal rhizotomy and the timing of orthopedic surgery represent recent advances in the surgical management of hypertonia. SUMMARY: Management of hypertonia in cerebral palsy targets the functional goals of the patient and caregiver. Treatment options are conceptualized as surgical or nonsurgical, focal or generalized, and reversible or irreversible. The role of pharmacologic therapies is to improve function and mitigate adverse effects. Further investigation, including clinical trials, is required to determine the role of deep brain stimulation, intraventricular baclofen, orthopedic procedures for dystonia, and rhizotomy.

Use of rimabotulinum toxin for focal hypertonicity management in children with cerebral palsy with nonresponse to onabotulinum toxin.

OBJECTIVE: The aim of this study was to review the effect of rimabotulinum toxin (BoNT-B) for focal hypertonicity management in children with cerebral palsy and secondary nonresponse to onabotulinum toxin treated at the authors' tertiary care academic medical center. DESIGN: A retrospective review of the medical treatment of children was conducted at the authors' institution (March 16, 2001, to August 2, 2002) using the key words botulinum toxin B and Myobloc (Solstice Neurosciences Inc, South San Francisco, CA). Demographic information was analyzed using descriptive statistics (number [percentage] and mean [range]). The Pearson χ test was used to evaluate differences in incidence of adverse events. RESULTS: Eighty-two children had BoNT-B injections (116 treatments). Overall, 26.8% (19/71) of the children or their parents/guardians reported no or minimal response to the injections, with 89.5% (17/19) of these children having secondary nonresponse to onabotulinum toxin. Adverse events were frequent but did not require hospitalization of any patient. No significant differences were found in incidence of adverse events related to BoNT-B dosing, medical fragility, or Gross Motor Function Classification System level. CONCLUSIONS: More than one-fourth of the children receiving BoNT-B injections had nonresponse, with most having previous nonresponse to onabotulinum toxin. Adverse events related to BoNT-B injections were frequent and unpredictable but not severe.

Severity of disability in patients with cerebral palsy secondary to symptomatic congenital cytomegalovirus encephalopathy.

PURPOSE: Cytomegalovirus (CMV) is a leading cause of congenital encephalopathy and cerebral palsy (CP). In this study we report the severity of disability in individuals who developed CP secondary to symptomatic congenital CMV encephalopathy. METHODS: The medical records of patients with CP secondary to symptomatic congenital CMV encephalopathy diagnosed from 1995 to 2011 were retrospectively reviewed. Gross Motor Functional Classification Scale (GMFCS) level, language function, and swallowing function were collected. RESULTS: Twenty-three patients were found. Of those 23 patients, 83% (19/23) were at a GMFCS level IV or V, 9% (2/23) each GMFCS level II or III and none (0%) at GMFCS I. Eighteen patients were non-verbal, 3 had minimal to moderate verbal skills and 2 had no verbal impairment. Eighteen patients also had severe dysphagia requiring gastrostomy tube (GT) feedings, and 5 ate orally. There was a strong correlation between the severity of GMFCS and having a gastrostomy tube (p< 0.0006) and GMFCS and verbal skills (p< 0.0023). CONCLUSION: This study shows that patients with CP secondary to symptomatic congenital cytomegalovirus encephalopathy have a very high risk of having severe physical and cognitive disabilities. This information can help healthcare providers and caregivers plan for the potential long-term medical, rehabilitation, and financial needs of this group of patients.

Definition and classification of hyperkinetic movements in childhood.

Hyperkinetic movements are unwanted or excess movements that are frequently seen in children with neurologic disorders. They are an important clinical finding with significant implications for diagnosis and treatment. However, the lack of agreement on standard terminology and definitions interferes with clinical treatment and research. We describe definitions of dystonia, chorea, athetosis, myoclonus, tremor, tics, and stereotypies that arose from a consensus meeting in June 2008 of specialists from different clinical and basic science fields. Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. Chorea is an ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments. Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture. Myoclonus is a sequence of repeated, often nonrhythmic, brief shock-like jerks due to sudden involuntary contraction or relaxation of one or more muscles. Tremor is a rhythmic back-and-forth or oscillating involuntary movement about a joint axis. Tics are repeated, individually recognizable, intermittent movements or movement fragments that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movement. Stereotypies are repetitive, simple movements that can be voluntarily suppressed. We provide recommended techniques for clinical examination and suggestions for differentiating between the different types of hyperkinetic movements, noting that there may be overlap between conditions. These definitions and the diagnostic recommendations are intended to be reliable and useful for clinical practice, communication between clinicians and researchers, and for the design of quantitative tests that will guide and assess the outcome of future clinical trials.