Foreword 2

Free Papers

Session A: Orthopaedic Surgery and Gait 3

Session B: Early Development: ADHD and Sleep 7

Session C: Orthopaedics and Spasticity 11

Session D: Epidemiology, Drug Therapy, and Bone Density 15

Session E: Measurements and Assessments 19

Session F: Quality of Life, Function, and Adult Outcome 23

Scientific Posters 27

Audio-Visual Program 52

Index 53

‘Cerebral palsy’ is an emotive term, which to parents often has the same impact as cancer or other dreaded diagnoses. For a child it is a label which can open doors to extra provision in schools and in the community, but which in itself may mean segregation, causing difficulties in participation. For a clinician it can be a diagnostic trap due to the failure to consider other conditions that impair motor function. For both clinicians and researchers it can be difficult to specify. Concerned about limitations of the current concept, a group of selected experts met last year to discuss the definition and classification of cerebral palsy. The preliminary results of their work are printed in this issue (p 571–576), with accompanying invited personal commentaries. We hope that it will provoke vigorous discussion.

‘Publish and be damned’, the Duke of Wellington's reported retort to attempted blackmail in a kiss-and-tell saga two hundred years ago, has acquired a Don't in front of it in the modern academic world. However, how research should be published has become a controversial topic. In the US the National Institutes of Health (NIH) have decided that any work they support should be made freely available after six months. The subtext is eventual immediate availability, which can already happen if authors publish in some electronic fora. If so, subscription journals could disappear. It is suggested that, instead of the existing system, authors should pay to publish their work, at a generally quoted cost of US$1500 per article. Future NIH grants would include publication costs. Other large grant giving bodies in the UK and elsewhere may follow the NIH example.

Cerebral Palsy (CP) has been discussed as a disease entity for decades. Today it is considered a group of disorders with different aetiologies, which constitutes a useful socio-medical framework for certain children with motor disabilities with special needs. Many countries run registers, which are important for service planning and for monitoring trends in CP rates.

Several articles in this issue reinforce Aicardi's dictum that in epilepsy, seizures are only the tip of the iceberg. They remind us that epilepsy is a symptom of a brain disorder and that children with epilepsy can have extra needs that may or may not be due to the epilepsy itself. Rightly, there has been increasing emphasis that proper medical management encompasses more than ‘just’ seizure control. Quality of life measures in childhood epilepsy are still not perfect, but they suggest only a moderate association with seizure control. Social inclusion, lifestyle restrictions, effects on family life, associated physical impairments, learning and/or behaviour difficulties, and unwanted effects from treatment are all recognized as major factors, as well as seizure frequency and severity. More recently we are finding how prevalent attentional difficulties, including attention-deficit–hyperactivity disorder, are in children with epilepsy and the importance of sleep problems.

Constraint-induced movement therapy (CIMT) is becoming increasingly recognized as a potentially useful means of treating children with hemiplegia but meanwhile attracts vigorous criticism and controversy. It has been suggested that constraint therapy is physically and developmentally intrusive in a number of ways: preventing use of the normal hand leaves a child with a poorly functioning hand which could cause distress and frustration; restraining the uninvolved limb at a critically sensitive stage of development may theoretically compromise function; there is a possible danger from lack of protection in case of falls; and the activities a child may be asked to perform may be inappropriate for his capability, resulting in resentment and lack of motivation.

Peer-review assessment of manuscripts is generally accepted to be an appropriate way to help the editorial staff of scientific journals to reach informed decisions about which papers to accept for publication. It is also a means by which reviewers can contribute to the effective translation of new ideas into print. Implicit in this statement, however, are two assumptions: first, that editors can recruit skilled reviewers to guide the journals' decision-making efforts; and second, that reviewers will take the time to do a credible job of this unpaid, usually unsung work.

Physical therapy is considered to be an important part of the management of cerebral palsy (CP); but what type of therapy, at what intensity, and for how long? These are questions which are not easily answered. Much valid criticism is directed towards the so-called ‘named’ therapy approaches because of the lack of a sound scientific basis and proof of efficacy. There is certainly little or no evidence to support the effectiveness of any particular approach, nor is there evidence to demonstrate superiority of one approach over another. Increasingly, therapists are being urged to adopt evidence-based intervention, but is there adequate, robust evidence available to enable them to do this? And how can available evidence be translated into effective practice? Certainly there are pockets of evidence to support the use of various forgotten and emerging modalities, such as muscle strengthening, constraint-induced movement therapy, and task-specific learning. This is encouraging; but such evidence cannot be generalized to all children with CP, particularly those who are classified as level IV and V on the Gross Motor Function Classification System, or to those who reside in disadvantaged environments. How can therapy for children with a multifaceted and complex disorder such as CP reach the stage where it is based on sound evidence?

Foreword 2

Session 1: Wednesday, 1.30–3.30pm 3

Oral presentations

Session 2: Thursday, 12.00pm–1.00pm 6

Posters with oral presentations (Oral presentations for the first 10 posters only)

Session 3: Friday, 10.30am–12.00pm 24

Oral presentations

Session 4: Friday, 1.30pm–3.00pm 26

Oral presentations

Index 29

Status epilepticus is a common neurological emergency in children admitted to hospitals in resource-poor countries where it has a high mortality and is associated with a significant morbidity. In the last 5 years, some pharmaceutical companies have announced their intention to stop manufacturing parenteral preparations of phenobarbitone and chlormethaziole, and the supply of paraldehyde has been erratic. Paraldehyde and phenobarbitone are recommended for the management of status epilepticus in the recently published Advanced Paediatric Life Support guidelines. Their withdrawal would have serious repercussions for the management of status epilepticus in resource-poor countries as these drugs are relatively cheap and easy to administer in these settings.

Foreword 2

Oral Presentations and Posters 3

Index 63

Methodological concerns are a major reason behind having to turn down papers offered to Developmental Medicine and Child Neurology. One important cause is inadequate validation of measures used in the work. The validity of a test is one of its most important attributes, whether it is a clinical assessment, laboratory assay, electrophysiological or neuroimaging investigation, or a measurement of function, such as motor or IQ scales. With such a plethora of tests used in both everyday clinical work and research, and with the difficulties involved, it is not surprising that some are incompletely validated.

The timing, mechanism and force, and the likelihood or otherwise of an innocent explanation of inflicted head injury in early childhood was thrown into doubt by three papers by Geddes et al. published in 2001 and 2003. They challenged the generally accepted position that the forces involved in the generation of subdural and retinal haemorrhages in infants are always considerable, and proposed in its place the hypothesis that an infant might suffer a craniocervical injury that caused respiratory disturbance leading to global cerebral hypoxia. The resultant hypoxia, brain swelling, and rise in central venous pressure could cause subdural leakage of blood from intracranial vessels and retinal haemorrhages.

Developmental Coordination Disorder (DCD) is a condition affecting explicitly the motoric, physical, psycho-social, and academic spheres of approximately 8% of children and adolescents. Considerable research has been conducted in order to understand the latent pathophysiology generating the various negative outcomes of DCD. Unfortunately, no convincing evidence exists as yet to support one particular theory.

The original study by Badawi et al. investigated risk factors for neonatal encephalopathy (NE) and/or neonatal seizures in term infants. They used a broad definition appropriate for examining etiology and capable of asking such questions as: what proportion of NE arises after birth asphyxia? The answer was: some, but not much. That study was, and still is, the only large population-based investigation of multiple potential risk factors for NE, and it identified novel risk factors such as maternal thyroid disease, family history of seizures, and placental abnormalities. Several of these factors have been found to be risk factors for NE, neonatal seizures, or cerebral palsy (CP) in other studies.

deux choses sont simples: raconter le passé et prédire l'avenir. y voir clair au jour le jour est une autre entreprise.

the tradition of the european academy of childhood disability (eacd) suggests that i write an editorial for this issue about trends, prospects, and priorities. the quote above, from the delicious armand salacrou, makes me humble in this task.

Children with cerebral palsy (CP) frequently grow poorly, compared with their typically-developing peers. Moreover, as children with CP age, their growth diverges further and further from that of typical children. The reasons for poor growth are multi-factorial and include nutritional, hormonal, physical, and neurological causes. Nevertheless, it appears that much of the poor growth is related to acute and chronic malnutrition at various points across the lifespan. The malnutrition is largely due to inefficient and dysfunctional feeding. While remediable to some extent, this dysfunctional feeding often cannot be overcome. Consequently, in children with (usually severe) CP, malnutrition is often treated by bypassing the dysfunctional oral feeding through the use of a gastrostomy.

Skeletal health in children is made up of a number of factors including the overall pattern of the skeleton, the appropriate size and shape of individual bones, bone mass and internal architecture of both tubular and plate-like bones, and bone metabolism and turnover. Where among this group of attributes does skeletal maturity fit in?

Jose Prats Viñas and coworkers' (p 419) report in this issue is of great interest, first because it raises the problem of the definition and limits of Aicardi syndrome but also, accepting as I do that it belongs to the syndrome, it suggests a much wider spectrum of expression.

A diagnosis of 22q.11 deletion syndrome (del22q.11) in a child brings with it a plethora of uncertainty in terms of developmental and neuropsychiatric prognosis. This relatively common genetic disorder has a highly variable phenotype with a multitude of potential physical features including palatal anomalies, congenital heart defects, facial dysmorphism, immunodeficiency, and hypoparathyroidism. As a result, patients may present in paediatric, cleft lip/palate, psychiatric, or antenatal clinics. It is necessary, therefore, that the multidisciplinary team are aware of this diagnosis. The overall clinical phenotype ranges from subtle isolated findings to severe multisystem involvement, even in the same family. The neurobehavioural phenotype is also highly variable. The spectrum of developmental delay, learning difficulties, behavioural problems, speech and language deficits, and psychiatric disorders seen in del22q.11 is well recognized. The implications for parents, medical professionals, educational staff and, indeed, the patients are significant. Thus, better understanding of the cognitive and behavioural problems could lead to the development of effective, targeted interventions and improved management.