Foreword 2

Free Papers

Session A: Cerebral Palsy Outcome Tools 3

Session B: Baclofen 6

Session C: Botulinum Toxin 9

Session D: Cerebral Palsy – Orthopaedics 12

Session E: Spina Bifida 15

Session F: Therapy 18

Session G: Cerebral Palsy – General 21

Session H: Development/Transition 25

Session I: Health and Well-Being 28

Scientific Posters 32

Audio-Visual Program 50

Index 51

The two important articles on melatonin in this issue make a very interesting contrast. In the first we requested and got a personal view based on years of experience and the available evidence, laced with clinical nuances and subtleties, to guide those of us who are less familiar with the field. The other, by a team who are also very experienced in the use of melatonin, has followed an evidence based approach and shows how few hard data are available when rigid criteria are applied. Surprisingly, a proposed multicentre trial in the UK has not so far been funded, despite scientific validity and ethical approval (Appleton RE, personal communication). It does not seem quite attractive enough to the unelected judges on the various charities' governing bodies. It is a very apposite example of the difficulties in proving that what we do is effective, even when most of us believe it to be so.

Diagnostic labels describing specific diagnoses can be valuable in analyzing a child's problems and may be critical in securing medical and educational services, ‘but sometimes we forget that there is actually but one child with one dysfunctional brain’ (Rapin I, unpublished). Co-occurring developmental difficulties require an integrated approach in both terminology and care. The term ‘neurodevelopmental disorder’, as well as encompassing major impairments such as the cerebral palsies, embraces overlapping problems with speech and language, motor-perceptive function, behaviour, and learning.

‘Good food’, I remarked argumentatively ‘should be the occasion and not the topic of good conversation’. I was rounded on by my friends who berated me for such a naïve remark. From antiquity to the present day the experience of good food, the mealtime, has been a major feature of civilization and the cultural importance unimpeachable. Just so. But perhaps my view was biased by having spent time with families with children who have been hard to feed. Preparing the right food for them (good food) and helping the child consume that food over many hours can cause endless distress. When we started our first Feeding Clinic at our Child Development Centre I naïvely imagined that most of the children who came to see us would be there with social and behavioural reasons for the feeding difficulty (and there are such of course). But we soon saw children with all sorts of disabilities and it was distressing to find, as a clinician, that children referred for ‘feeding difficulties’ proved to have a neurological problem and we were telling parents that was the case at the same time as trying to help them with the problems that had brought them to the clinic. It is in these children that alternative feeding methods have to be considered. Already for many of them nasogastric feeding had been a feature of their early lives. Sampson-Fang and her colleagues have reviewed more than a decade of not very robust evidence of the value of gastrostomy feeding in children with cerebral palsy (CP; DMCN 2003, 45: 415–426). But it is important to remember that CP is not the only diagnostic category where we see feeding problems (e.g. cardiac, genetic conditions).

Foreword 2

Oral Presentations

Session 1 3

Session 4 6

Poster Presentations

Session 2 10

Session 3 21

Video Presentations

Session 5 31

Index 33

Victimization by peers affects 10 to 20% of school children under the age of 12 years. Physical, verbal, and psychological victimization (being pushed, hit, called names, teased, being the target of rumours, theft, extortion) is associated with short- and long-term adjustment problems, such as peer rejection, social withdrawal, low self-esteem, anxiety, loneliness, and depression, as well as academic problems and school drop-out. Research on populations of school children (primary and secondary) has associated victimization with personal risk factors (the victim's characteristics and behaviour) and interpersonal risk factors (social relationships between peers). Studies on the social adjustment of preterm children at school age show that, even in the absence of a major motor or cognitive disability, this population has several personal risk factors associated with victimization. The objective of this study was to compare the level of victimization experienced by a group of 96 seven-year-old children born extremely preterm (EP, <29 weeks of gestation; 49 females) against that experienced by a group of 63 term children (34 females) matched for age and sex, maternal level of education, and family socioeconomic status. The children born EP had a mean gestational age of 27.3 weeks (SD 1.2) and a mean birthweight of 1001.1g (SD 223) and normal birthweight children had a mean gestational age of 39.5 weeks (SD 1.5) and a mean birthweight of 3468.7g (SD 431). Physical and verbal victimization were assessed in a school setting by peers with individual sociometric interviews (Modified Peer Nomination Inventory). After controlling for physical growth (height and weight) at the age of 7 years, the data indicate two independent effects: males were more victimized than females, and children born preterm experienced more verbal victimization by their peers than their term classmates, even when participants with a visible motor, intellectual, or sensory disability were excluded. Several hypotheses are presented to account for the higher incidence of verbal victimization of preterm children.

Neuropsychological impairments possibly underlying academic and/or behavioural limitations were studied in 149 school-age survivors of bacterial meningitis, 68 with and 81 without academic and/or behavioural limitations. Academic limitations affected mathematics, reading, and writing. Behavioural limitations were inferred from scores in the clinical range on the Child Behaviour Checklist. These children had been selected from a cohort of 674 children (57% males) who had recovered from non-Haemophilus influenzae type B bacterial meningitis and who had a mean age at infection of 2 years 4 months (range 1mo to 9y 5mo). They had neither ‘complex onset’ meningitis, prior cognitive or behavioural problems, nor severe disease sequelae. They were assessed with standardized assessment methods a mean of 7.8 years (range 4 to 10.4) after meningitis. Children with limitations (32% of the cohort) performed generically poorly on measures of cognitive functioning, speed, and motor steadiness, rather than having impairments in specific neuropsychological domains. The presence of two or more minor neurological signs was more frequent in the group with than in the group without limitations (30% versus 9%); this may explain the relatively poor speed and motor steadiness of the group with limitations.

The aim of this prospective cohort study was to evaluate the impact of gastrostomy tube feeding on the quality of life of carers of children with cerebral palsy (CP). Short-Form 36 version II was used to measure quality of life in carers of 57 Caucasian children with CP (28 females, 29 males; median age 4y 4mo, range 5mo to 17y 3mo) six and 12 months after insertion of a gastrostomy tube. Responses were calibrated against a normative dataset (Oxford Healthy Life Survey III). Six months after gastrostomy feeding was started, a substantial rise in mean domain scores for mental health, role limitations due to emotional problems, physical functioning, social functioning, and energy/vitality were observed. At 12 months after gastrostomy placement, carers reported significant improvements in social functioning, mental health, energy/vitality (mean increase >9.8 points; p<0.03), and in general health perception (mean increase 6.35 points; p=0.045) compared with results at baseline. Moreover, the values obtained for these domains at 12 months were not significantly different from the normal reference standard. Carers reported a significant reduction in feeding times, increased ease of drug administration, and reduced concern about their child's nutritional status. This study has demonstrated a significant, measurable improvement in the quality of life of carers after insertion of a gastrostomy feeding tube.

Childhood craniopharyngiomas are histologically benign tumours arising from remnants of Rathke's pouch in the hypothalamic–pituitary region. The two common treatment approaches are primary total resection or limited resection followed by radiotherapy. To study the outcome after a primary surgical approach, we followed 25 consecutive patients (10 females, 15 males) under 16 years of age who were treated in a single institution with a management policy of radical tumour excision (mean age at diagnosis 9 years 2 months, SD 4 years 3 months; range 2 years 9 months to 15 years 11 months). Mean follow-up after primary surgery was 11 years 3 months (SD 7 years 7 months). Tumour control, and neurological, endocrine, and hypothalamic complications and their impact on health-related quality of life were assessed (medical follow-up, semi-structured interview, and questionnaires). Results of tumour control were generally good, however, local failure was observed in 6 of 25 patients, and severe late-treatment complications decreased quality of life for many long-time survivors. Endocrine deficiency occurred in 24/25, visual complications in 16/24, neurological complications in 8/24, obesity in 14/23, increased daytime sleepiness in 6/21, and significant school problems in 10/20. Patients with craniopharyngioma rated their health-related quality of life as considerably lower than healthy controls; the domains of social and emotional functioning were particularly affected. Parents' ratings were considerably lower than those of the patients. Poor functional outcome was associated with large tumours infiltrating or displacing the hypothalamus, the occurrence of hydrocephalus, and young age at diagnosis, but also with multiple operations due to tumour recurrence. Alternative treatment strategies should be considered, especially in very young patients with large tumours.

The aim of this population study was to examine the severity and prevalence of co-occurring developmental delays in 4-year-old children, the rate of overlapping problems, and sex differences. A sample of 434 children (196 males, 238 females; mean age 4 years 3 months, SD 1 month) were administered the ‘Lene’ test: a comprehensive neurodevelopmental screening test. Results suggest that co-occurrence of attention-behavioural, motor-perceptual, and language delays occurring in school-aged children could already be detected at the age of 4 years. Isolated delays were usually mild, but co-occurring difficulties were mostly moderate or severe. Overlap between developmental delays depended on the severity of the problems. It emerged that males had more severe and more often co-occurring problems than females. Co-occurrence of developmental delays as a risk factor at the early stage of development is discussed.

The incidence of spina bifida has dropped significantly in many countries over the last several years. This change has occurred secondary to the use of folic acid, and prenatal diagnosis with termination of pregnancy. The overall care of children with spina bifida has also changed substantially in the past 30 years. These changes have occurred in all specialties: neurosurgery, urology, orthopaedics, rehabilitation, and orthotics.

White matter alterations in chromosomal disorders have been reported mainly in 18q–syndrome. Our aim was to evaluate white matter alterations in patients with chromosomal abnormalities detected through conventional cytogenetic techniques. Forty-four patients with chromosomal abnormalities, excluding trisomy 21, were diagnosed in our hospital between May 1999 and December 2002 (24 males, 20 females; mean age 6 years 4 months [SD 3 years 2 months], range 0 to 18 years). Of the 44 patients, 14 had brain magnetic resonance imaging (12 males, 2 females; mean age 4 years 2 months [SD 4 years 4 months]; five with sex chromosomal disorders [SCD] and nine with autosomal chromosomal disorders [ACD]). Of these 14 patients, eight (four with SCD and four with ACD) had abnormal white matter findings of similar patterns. These patients had pseudonodular, subcortical, and periventricular white matter high signal intensity images in T2, and fluid-attenuated inversion recovery sequences that were isolated or confluent. The images did not correlate with the neurological clinical state. Given that eight of the 14 patients showed these lesions, their prevalence in different chromosomal abnormalities appears to be high, even though they have not been well reported in the literature. To our knowledge, these alterations have never been described in SCD. We concluded that unknown factors related to the myelination processes may be localized in different chromosomes.

The study aimed to determine whether degree of participation of children with cerebral palsy (CP) is influenced by where they live, as predicted by the social model of disability. Ninety-two per cent children with CP resident in Northern England and born 1991–1996 were entered into the study. Participation was measured by the Lifestyle Assessment Score and its six component domain scores. Regression analysis was used to investigate variations in participation. There were 443 children (265 male, 178 female; mean age 4 years 8 months [SD 1 year 1 month] at time of assessment) in the study. In the regression analysis the following factors remained significant with regard to level of participation: type of CP (167 with hemiplegia, and of those remaining 240 with bilateral spasticity); intellectual impairment (105 with IQ<50, 113 with IQ 50 to 70, and 225 with IQ>70); presence of seizures (115 with active epilepsy); walking disability (114 unable to walk, 81 restricted and needing aids, 186 restricted but unaided, 62 unrestricted); communication problems (61 no formal communication, 51 use alternative formal methods, 126 some delay or difficulty, 205 no communication problems). After adjustment for these factors, there were significant variations with regard to level of participation in the Lifestyle Assessment Score by district of residence. The magnitude of these variations in Lifestyle Assessment Score between districts is similar to that accounted for by severe intellectual impairment. Similar models were obtained for four of the six domain scores. For one of these four, restriction of social interaction, the significant variation between districts was minimally influenced by the underlying type of CP, walking ability, or presence of seizures. Higher levels of participation among children with CP are associated with residence in certain districts. This is not attributable to variations in case-mix or functional capacity of the children. Participation of children with disability is partly a product of their environment.