The effect of botulinum toxin type A on the functional ability of the child with spastic hemiplegia a randomized controlled trial.

It has been demonstrated that botulinum toxin type A (BTX-A) injections reduce spasticity and improve muscle growth in children with spasticity. It has been postulated that BTX-A allows the learning of more normal movement patterns. The aim of this study was to measure the effect of this treatment on functional ability, as measured by the Gross Motor Function Measure (GMFM), in children with spastic hemiplegic cerebral palsy. Children of 3--13 years and meeting the selection criteria were randomly allocated to the control or injection group using a matched pair design. A match constituted a child within 6 months of age with the same Modified Ashworth Score (MAS) for the gastroc-soleus and within 10% of the same goal scores on the Gross Motor Function Measure. Twelve matched pairs were enrolled. Outcomes were measured on enrolment and at 1, 3 and 6 months post injection. The time course of the response to BTX-A was assessed with measurements of the MAS, dynamic range of motion (R1) and static muscle length (R2). Motor function was assessed using the 88-item GMFM and parental satisfaction with a 10-point visual analogue scale. Within pair comparisons of the GMFM using the Wilcoxon signed rank test indicated that the treatment group made significantly greater gains than controls at 3 months (P=0.02) with even greater differences seen at 6 months (P=0.004). Using parametric statistics, the intrapair difference in proportional change of GMFM increased from 35% (4 to 65) at 3 months to 52% (17--87) at 6 months. Response to injection was confirmed by a decrease in MAS in the treatment group and very little change in controls. This difference was significant (P=0.002) at 3 months and was attenuated but still significant (P=0.016) at 6 months; the difference in proportional change decreased from 44% at 3 months to 22% at 6 months. Changes in R1 reflected those of MAS in the treatment group and deteriorated significantly over the study period in controls. Parents of children in the treatment group were more satisfied than controls, but satisfaction scores did not correlate with changes in function or technical outcomes suggesting that this may be a placebo effect. The changes in GMFM correlated with changes in technical outcomes at 3 months, suggesting a causal relationship. The intrapair differences in GMFM continued to increase even after the local response to injection had started to wane.

Latex allergy in an Australian population of children and adolescents with spinal dysfunction.

OBJECTIVES: To determine the prevalence of latex allergy in an Australian population of children and adolescents with spinal cord dysfunction and a comparison population of their siblings without spinal dysfunction. DESIGN AND SETTING: Cross-sectional study of all patients with spinal cord dysfunction attending the single tertiary spinal dysfunction clinic in Western Australia, and of their siblings closest in age. SUBJECTS: 104 patients with spinal dysfunction born 1978-1996 inclusive, and 50 siblings. MAIN OUTCOME MEASURES: Prevalence estimates and adjusted odds ratio estimates of the risk of having a history of latex allergy and of testing latex-specific IgE positive. RESULTS: Of the patients, 15.4% (95% CI, 9.1%-23.8%) had a history of latex allergy compared with none (95% CI, 0-5.8%) of the siblings. Of the 84 patients tested, 36.9% (95% CI, 26.6%-48.1%) were latex-specific IgE positive compared with 15.4% (95% CI, 4.4%-35.9%) of the 26 siblings tested. For patients, every operation after the first increased the risk of a positive IgE result cumulatively by 41% (odds ratio, 1.41; 95% CI, 1.18-1.68). CONCLUSIONS: The prevalence of latex sensitivity and clinical allergy in children and adolescents with spinal dysfunction in Australia is as high as that seen in the United States, and is related to latex exposure during surgery. The management policy regarding latex exposure for patients with spinal dysfunction requires urgent consideration.

Intravesical therapy for the treatment of neurogenic bladder in children.

BACKGROUND: Oral pharmacotherapy has been commonly used as an adjunct to clean intermittent catheterization (CIC) in the treatment of neurogenic bladder in order to achieve continence, but may be associated with unacceptable side effects. The authors' experience with sterile intravesical preparations of oxybutynin hydrochloride and ephedrine in children is reported here. METHODS: Patients requiring CIC for neurogenic bladder but with incontinence that was unresponsive to standard oral therapy or that was associated with severe systemic side effects were studied over a 1-year period. Clinical, radiological and urodynamic assessments were made prior to commencing treatment with intravesical oxybutynin hydrochloride. Patients who remained incontinent with poor internal sphincter muscle tone had intravesical ephedrine added. RESULTS: Seven patients were involved in the study over a 1-year period. Two patients became continent and one patient had an improvement in upper tract dilatation. One patient had a limited improvement with oxybutynin alone but became continent with the addition of ephedrine. Three patients had no response to treatment. There were few side effects. CONCLUSION: Intravesical agents have a role in the management of paediatric neurogenic bladder for those children with significant adverse sequelae from oral pharmacotherapy who would otherwise require surgical intervention. Intravesical therapy is a safe technique in children with sterile preparations. Further investigation of this modality should be pursued.

Trisomy-18 mosaicism with features of Russel-Silver syndrome.

A child is described who presented at 16 months with developmental delay and clinical features of the Russell-Silver syndrome. Chromosome analysis revealed trisomy-18 mosaicism. Only one other similar case has been reported in the literature. It is recommended that chromosomal studies continue to be included in the clinical investigation of children with Russell-Silver syndrome.

Infantile autism. I: A prospective study of the diagnosis.

A prospective study was made of 200 consecutive children to evaluate the usefulness in the diagnosis of infantile autism of the behavioral scale reported by Clancy and coworkers in 1969. On this scale seven or more of 14 behavioral manifestations must be present before a diagnosis of autism can be made. Using this scale alone, 48 of the 200 children studied were "scale positive", i.e. could be considered autistic. However, further study of this group showed that only one child fulfilled the classical criteria of Kanner (1943) for a diagnosis of early infantile autism. Scale "positivity" was found to correlate with mental retardation and to be associated with other developmental defects, especially learning disorders and hearing loss.