Foreword 2

Oral Presentations 3

Posters 11

Video Presentations 40

Index 44

Ultrasound images were obtained of the medial gastrocnemius at different ankle joint positions with the knee extended. Fascicle length and deep fascicle angle were measured in five normally developing adults (mean age 33 years, age range 24 to 36 years) and in five normally developing children (mean age 7.8 years, age range 7 to 11 years), and in seven children with spastic diplegia (mean age 10 years, age range 6 to 13 years). These architectural variables were similar in the groups of normally developing adults and children. Importantly, no statistical difference could be found between the normally developing children and those with diplegia for fascicle length. Deep fascicle angles were reduced significantly in the clinical group at a particular ankle joint angle but not at the resting angles. The difference in deep fascicle angles is explained as a function of resting muscle length and is not attributed any clinical importance. Our results do not explain the structural origin of muscle contracture explicitly. However, they do indicate that most of the fixed shortness in the medial gastrocnemii of ambulant children with spastic diplegia is not due to reduced muscle fascicle length. We suggest that muscle contracture may be better explained in terms of shortness of the aponeuroses of pennate muscles, such as the medial gastrocnemius, through reduced muscle fascicle diameter.

Results of surgery to correct fixed flexion contracture of the knee and improve voluntary knee extension in 39 knees in 20 children (11 females, 9 males; mean age 12 years 8 months, age range 5 to 20 years) with cerebral palsy were analyzed. All patients had neuromotor disease and 18 children had spastic diplegia or quadriplegia. All patients could initiate voluntary knee extension but lacked full passive extension. Five patients (10 knees) were free walkers with a mean motor severity index of 19 and mean fixed knee contracture of 20 degrees. Fifteen patients (29 knees) were not free walkers and 13 were wheelchair ambulators. They had a mean motor severity index of 8 and mean fixed knee contractures of 30 degrees. Surgical procedures included various combinations of hamstring lengthening and/or posterior capsulotomy to allow free passive knee extension, with or without quadriceps mechanism shortening, to enhance voluntary extension. The best results were in patients who had hamstring lengthening, posterior capsulotomy, and quadriceps mechanism shortening.

In orthopaedic clinical practice hip pain is the main complaint of adults with cerebral palsy (CP). The aim of this descriptive study was to specify prevalence of hip pain and to propose methods of care other than surgery. The study was a cross sectional multicentre one based in the Rhône-Alpes region. Patients over 15 years of age, with CP who were non-ambulatory were included. Two hundred and thirty-four patients were questioned. Mean age of the patients was 27 years 10 months, median 26 years 1 month, with 59.3% males and 40.7% females. Patients were questioned about pain with precise information about the circumstances of pain, tolerance, and treatment. Pain was judged to be intolerable if it prevented usual activities. Prevalence of hip pain was 47.2%. Pain was judged to be tolerable in 35.6% of the 234 patients, i.e. in 75.5% of patients with hip pain. There were three types of pain: provoked pain, pain linked to position, and spontaneous pain. Medical treatment was given to only 13.6% of patients with hip pain. The first treatment for hip pain is to avoid circumstances where pain occurs; medical treatment is reserved for when daily life cannot be adapted sufficiently to prevent pain. Medical treatment must be appropriate with doses of adequate strength before proposing surgery.

The Children's Communication Checklist (CCC) was developed to provide an objective assessment of pragmatic aspects of children's communication difficulties. We aimed to (1) see whether the checklist provided valid and reliable information when completed by parents, and (2) consider its usefulness in a clinical context. Checklists were completed by parents and a professional who knew the child well for all 5 to 17-year-old referrals to a tertiary developmental paediatrics centre over a 31-month period. Children who were not yet speaking in sentences were excluded. From a sample of 151 children (81% male; mean age 8.7 years) with pervasive or specific developmental disorders, valid checklists were completed by 119 parents and 93 professionals. Reliability, as measured by internal consistency, was 0.7 or higher for most scales. Correlations between ratings for parents and professionals were in the range of 0.30 to 0.58 for individual pragmatic scales, with a correlation of 0.46 (n=82) for the pragmatic composite. For both parents and professionals, the pragmatic composite was lowest for children with a diagnosis of autism; intermediate for those with a diagnosis of Asperger syndrome, pervasive developmental disorder not otherwise specified or attention-deficit–hyperactivity disorder (ADHD); and highest for those with a diagnosis of specific learning disability. The strongest relation between the pragmatic composite and diagnosis was seen when ratings from parents and professionals were combined. Differences between diagnostic groups were not explicable in terms of age or verbal IQ.

Little is known about chronic posttraumatic headache (PTH) in children and adolescents. In this study we report on symptoms, clinical findings, and management of all children seen in our headache clinic who presented with recurrent headache following head injuries. A total of 23 children were identified over a period of 4 years. Twenty-one children (13 male, eight female; mean age 11.2 years, age range 3.3 to 14.9 years, median 12.1 years) fulfilled the International Headache Society's clinical criteria for the diagnosis of chronic PTH. Mean duration of headache was 13.3 months (range 2 to 60 months, median 7 months). Children were followed up for a period of 5 to 29 months (mean 12.5 months, median 9 months). Head injuries were classified as serious in four patients and minor in 17. Clinical variables were compared between children with PTH and those without a history of head injury. The headaches were migraine in five patients, tension type in 13, and mixed in three patients. Tension-type headache was significantly more common in children with chronic PTH than in those with no history of head injury, but the clinical course was comparable in the two groups.

This study reports on cognitive abilities and attainment in 29 children and 21 adults with Smith-Magenis syndrome. There were 13 boys and 16 girls aged 6 to 16 years, and nine men and 12 women aged 16 to 52 years. All had mild to severe learning disabilities with no differences overall between verbal and performance skills, but with a particular profile of cognitive strengths and weaknesses. Levels of attainment and of adaptive behaviour were strikingly low, and the group of adults emerged as much more dependent on carers than might have been expected from their general level of intellectual functioning. Reasons for this discrepancy are explored in terms of the severe behavioural difficulties characteristic of the syndrome.

Chronic constipation is a common problem in children; it is responsible for 5% of referrals to general paediatricians, 20% of referrals to paediatric gastroenterologists, and 10000 admissions to hospitals in the United Kingdom every year. Constipation is particularly common in children with disabilities; in a recent epidemiological survey of nutritional and gastrointestinal problems in children with cerebral palsy, constipation (defined as opening of the bowels less frequently than once in every 3 days) was reported in 98 of 377 (26%) of children. There is frequently a delay in the recognition and appropriate treatment of constipation in children and symptoms are often present for months or years before appropriate treatment is provided. Such delay is a particular problem in children with disabilities either because it is accepted as an inevitable consequence of the neurological impairment or because a higher priority is given to other aspects of medical management such as treatment of convulsions or postural deformity. Moreover, communication difficulties compound delay and failure to recognize the problem as the child with disabilities is often unable to express the discomfort caused by constipation. Abdominal pain is a frequently encountered symptom of chronic constipation in those children who are able to give an account of their experience and contributes considerably to the adverse effect that chronic constipation has on their quality of life. Similarly, adequate treatment of constipation provides relief for the child and can be observed to lead to an improvement in appetite or behaviour. For instance, the improvement in behaviour of children with autism following evacuation of retained faeces after constipation is frequently encountered in clinical practice.

The new antiepileptic drugs introduced over the last decade provide a welcome increase in the therapeutic options available for the 25% of children with epilepsy who remain refractory to traditional therapy. In the mid 1980s, vigabatrin (VGB) was the first of these to be made available in clinical practice. In 1997, the visual field deficits reported in association with VGB prompted a review of its place in the management of epilepsy. We provide an overview of the uses of VGB and a discussion of the difficulties which currently affect our clinical practice.

During the past 40 years, research in developmental neurology has provided a number of significant new paradigms on the functional development of the human nervous system. It is important to understand the historical background to the previous paradigms and the reasons why they became outdated and how they have been replaced on empirical grounds.

The case is presented of a female infant with a distal deletion of 8p (8p23.1→pter) whose development was monitored over a 5-year period from 12 months of age. Although previous literature has suggested that 8p deletion is associated with mild to moderate intellectual disability, the child reported here has normal intelligence. Despite initial delays in gross motor and language skills, cognitive development (assessed with the Bayley Scales of Infant Development) and intellectual ability (measured on the Stanford-Binet Intelligence Scale) were within average range. It is argued that the small number of previous case reports may have created a misleading impression of intellectual development in individuals with distal deletions of 8p.