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Botulinum toxin A (BTX-A) is widely used in the management of muscle spasticity in children. However, at present the dose of BTX-A for a given patient is selected empirically. The aim of this study is to provide dosage guidelines that are based on risk/benefit assessment. This was a multicentre retrospective study of the safety profile and efficacy of BTX-A in children with chronic muscle spasticity. Data in 758 patients who received a total of 1594 treatments were analysed (mean age 7.2 years; 429 males, 329 females). Spastic cerebral palsy (CP) was the most common diagnosis (94% of the study sample). Of all treatments 7% resulted in adverse events; incidence was related to the total dose rather than the dose calculated on the basis of body weight. The highest incidence of adverse events was observed in patients who received >1000 IU of BTX-A per treatment session. The odds of an adverse event was 5.1 times greater for this group of patients than for those who had 250 IU or less (p<0.001). A good overall response to treatment was reported in 82% and treatment goals were fully or partially achieved in 3% and 94% of participants respectively. More patients in the highest dose group reported functional deterioration. Interestingly, multilevel treatments resulted in a better response than single-level treatments (odds ratio 1.7, 95% CI 1.3 to 2.2,p=0.001).

Background: Cerebral palsy is the commonest cause of severe physical disability in childhood. For many years treatment has centred on the use of physiotherapy and orthotics to overcome the problems of leg spasticity, which interferes with walking and can lead to limb deformity. Intramuscular botulinum toxin (BT-A) offers a targeted form of therapy to reduce spasticity in specific muscle groups., Aims: To determine whether intramuscular BT-A can improve walking in children with cerebral palsy., Design: Randomised, double blind, placebo controlled trial., Methods: Forty patients with spastic diplegia or hemiplegia were enrolled. Twenty two received botulinum toxin and 18 received placebo. The primary outcome measure was video gait analysis and secondary outcome measures were gross motor function measure (GMFM), physiological cost index (PCI), and passive ankle dorsiflexion., Results: Video gait analysis showed clinically and statistically significant improvement in initial foot contact following BT-A at six weeks and 12 weeks compared to placebo. Forty eight per cent of BT-A treated children showed clinical improvement in VGA compared to 17% of placebo treated children. The GMFM (walking dimension) showed a statistically significant improvement in favour of the botulinum toxin treated group. Changes in PCI and passive ankle dorsiflexion were not statistically significant., Conclusion: The study gives further support to the use of intramuscular botulinum toxin type A as an adjunct to conventional physiotherapy and orthoses to reduce spasticity and improve functional mobility in children with spastic diplegic or hemiplegic cerebral palsy.

The effectiveness of ankle-foot orthoses (AFOs) on walking pattern was studied in 12 children with cerebral palsy between the ages of 3 and 7 years. Over a 2-year period two trials of fortnightly periods without AFOs were carried out. The range of ankle dorsiflexion, video analysis looking specifically at footfall, and rank scoring of the mediolateral shear force obtained using an oscilloscope printout from a Kistler force platform were compared with measurements obtained during periods when the child was wearing AFOs. The results using different outcomes were consistent, and indicated that the range of movement and gait deteriorated during the two trial periods during which the splints were not worn compared with periods during which the splints were worn. This finding needs to be confirmed in a large randomised controlled trial.

A case of progressive spinocerebellar syndrome due to isolated vitamin E deficiency is reported. Measurement of the vitamin E concentration in serum should be included when investigating all children with unexplained, progressive ataxia, even in the absence of malabsorption. Replacement treatment in patients with a vitamin E deficiency can arrest or improve the associated neurological disorder.

All surviving infants with birthweight < or = 1500 g born in 1982 and 1983 at St. James's University Hospital, Leeds, were followed up for 5 years. There were 88 survivors (including 5 in utero transfers) from the original cohort of 126 infants. In their fifth year the following assessments were made: neurological, audiological, intellectual, behavioural, growth and general health. A comparison group of full-term male infants was also studied with respect to intellectual status, social and emotional behaviour and general health. Principal neurological impairments found were: cerebral palsy 9 (10.2%), hydrocephalus 1 (1.1%), epilepsy 2 (2.3%) and sensorineural deafness 2 (2.3%). One third of the VLBW children required the services of the child development centre. Seventy-nine of the 88 VLBW children were tested with the WPPSI. Seven (8.8%) scored below 70. The VLBW boys had mean IQ scores of 90.6 while the mean for the girls was 100.2. The very low birthweight boys were significantly intellectually impaired compared with their peers. Socially and emotionally they were largely comparable with their full-term peers. The findings suggest that there has been no increase in severe disability following a policy of active neonatal intensive care. However, the quality of survival of VLBW children born in the 1980s, despite improvements in perinatal care, remains a major concern.

In order to test the hypothesis suggested in a recent report that the birthweight ratio might be a useful predictor of several important clinical outcome measures in babies of less than 31 weeks' gestation, we examined the association between the birthweight ratio and aspects of both short and long term outcome in 436 Leeds babies of less than 31 weeks' gestation. Unlike the report, and contrary to what we had expected, we were unable to find any significant association between birthweight ratio and length of time on the ventilator, mortality, neurological outcome, or intellectual outcome.

Hemiplegic cerebral palsy is appearing as the commonest cerebral palsy syndrome found at the Yorkshire Regional Child Development Centre, Leeds, and in the majority of cases does not follow a definable perinatal injury. As a practical consequence of this changing pattern of cerebral palsy a large proportion of these children will now be detected at 'well baby' clinics rather than at hospital neonatal 'at risk' clinics. As lack of awareness of the signs of cerebral palsy in the young infant lead to delay in detection and management, the early signs of congenital hemiplegia are discussed.

Monozygotic twins with idiopathic external hydrocephalus are reported. Characteristic computerised tomographic features were associated with early gross motor delay and rapid improvement. A genetic basis for the syndrome is supported by its occurrence in identical twins.

Children attending primary schools in Leeds were tested soon after school entry with the Standardized Motor Test Battery (SMTB), specifically designed for this study and comprising: finger tapping, peg moving, bead threading, standing on one leg, hopping and walking. Norms of motor performance are provided for each test. Seventeen children failed the SMTB and were designated to the clumsy group and then matched for age and sex with a control group of children from the survey. Observations by parents and teachers rated the clumsy children inferior to their controls in writing, sporting ability and clumsiness. The clumsy children and their controls were then followed up for a minimum of 2 years, when educational performance was assessed and motor performance reassessed. The clumsy children were found to have impaired educational attainment, particularly in writing, compared to their controls, and their motor performance although much improved was still very inferior to controls. The findings of this study suggest that the SMTB is a sensitive test for the early identification of clumsy children.

Two families are described with Rubinstein-Taybi syndrome. In one family the syndrome is seen in its full form in the index case and his uncle; three cousins of the index case also show varying degrees of expression of the disorder. In the other family a brother and sister are affected. This case report illustrates the varying expression of the disorder and the change in facial appearance with age. A polygenic basis of inheritance is supported and it is suggested that the typical facial appearance in infancy is the best means of identifying the disorder, as broad thumbs and toes may not be apparent or may be only a borderline abnormality.

Over a 4-year period a significant fall occurred in the mean birthweight and mean gestational age of prematurely born children who subsequently developed spastic diplegia. This coincided with a considerable improvement in perinatal mortality in the unit in which they were delivered. However, although the number of premature deliveries remained stable, the disorder did not become less common. No specific aetiological factors were identified, but the 24 affected children frequently had a history of adverse prenatal or perinatal factors. We conclude that high standards of obstetric and neonatal care prevent the subsequent development of spastic diplegia in low birthweight infants. However, these factors have also improved the survival of very low birthweight infants who have a much higher risk for the disorder and this has prevented a significant reduction in its frequency.

Charing Cross Hospital provides facilities for the study of handicapped children in a normal nursery which forms part of its Child Development Centre. The period of assessment varies from one day to three weeks; the advantages of prolonged assessment are discussed. Parental involvement and support, and the teaching of medical students are emphasized. A study of the Centre's first 385 patients is presented.

Current procedures for cognitive assessment of severely motor impaired infants are of limited value because of their reliance on motor responsivity. Methodologies which make use of infant attentional responsiveness offer the possibility of assessing cognitive function relatively independently of motor ability. We report the results of a study in 15 motor impaired infants of attentional changes to visual and auditory stimuli using heart rate change and observed behaviour as indices. Using these methods it was possible to show differences in infant cognitive functioning in otherwise 'hard to test' infants.