Foreword 2

Invited Speakers 3

Oral Presentations 18

Posters 31

Audio-Visual 65

Index 67

This randomized clinical trial evaluated the effects of a home-based, six-week strength-training programme on lower limb strength and physical activity of 21 young people (11 females, 10 males; mean age 13 years 1 month, SD 3 years 1 month; range 8 to 18 years) with spastic diplegic cerebral palsy (CP) with independent ambulation, with or without gait aids; (Gross Motor Function Classification System levels I to III). Compared with the 10 controls, the 11 participants in the strength-training programme increased their lower limb strength (combined ankle plantarflexor and knee extensor strength as measured by a hand-held dynamometer) at 6 weeks (F(1,19)=4.58, p=0.046) and at a follow-up 12 weeks later (F(1,18)=6.25, p=0.041). At 6 weeks, trends were also evident for improved scores in Gross Motor Function Measure dimensions D and E for standing, running and jumping, and faster stair climbing. A relatively short clinically feasible home-based training programme can lead to lasting changes in the strength of key lower-limb muscles that may impact on the daily function of young people with CP.

This qualitative study investigated the positive and negative outcomes of a home-based strength-training programme for young people with cerebral palsy (CP). Eleven young people with spastic diplegic CP (seven females, four males; mean age 12 years 9 months, SD 2 years 10 months; range 8 to 18 years) and their parents were interviewed. Gross Motor Function Classification System scores ranged from I (walks without limitations) to III (walks with assistive device), with a mode of III. The strength-training programme, which was conducted in the participants' homes three times per week for 6 weeks (total of 18 prescribed sessions), comprised three exercises targeting the major support muscles of the lower limbs. Exercises were bilateral half squats, heel raises, and step-ups. The training load was increased by adding free weights to a backpack so that 8 to 10 repetitions of each exercise could be performed. Using thematic coding, three categories of outcome emerged: body function and structure, activity, and participation, which were influenced by environmental and personal contextual factors. The programme generated overwhelmingly positive outcomes with only minor negative responses about some equipment and the need for parental involvement. Benefits included perceptions that strength, flexibility, posture, walking, and the ability to negotiate steps had improved. In addition, participants reported psychological benefits such as a feeling of increased well-being and improved participation in school and leisure activities. The contextual factors highlighted the fact that sufficient clinician resources must be allocated to solve individual exercise and equipment problems. As well as providing further evidence that strength training can be beneficial, this study provides useful indicators to guide future quantitative studies of outcomes that are meaningful for people with CP.

The aim of this study was to determine the reliability and magnitude of error of three lower-limb clinical measures for children with cerebral palsy (CP): the Modified Ashworth Scale of Spasticity (MAS), passive range of movement (PROM) and the modified Tardieu scale (MTS). Six physiotherapists measured 11 females and seven males (mean age 6 years 4 months, SD 2 years 4 months; age range 2 years 4 months to 10 years) on two occasions using a repeated measures design, collecting all data over 6 days. The severity of CP spanned all five levels of the Gross Motor Function Classification System and all children demonstrated varying degrees of spasticity. Exclusion criteria included botulinum toxin injections, inhibitory plasters, and orthopaedic surgery within the 6 months before study entry. For PROM and the MTS interrater reliability was acceptable with an intraclass correlation coefficient of 0.7, but results for MAS were lower. Standard error of measurement for repeated measures of PROM and MTS was about five degrees, but 95% confidence interval ranges were considerably higher. Test–retest results varied widely, particularly for the MAS. These measurement tools should be used with caution when evaluating changes in young children with CP.

A consecutive series of 106 children and adolescents (mean age 10 years, 6 months; SD 4 years, 8 months) with recent traumatic brain injury admitted to a regional hospital-based rehabilitation program was assessed to determine the rate of walking recovery, and characteristics that distinguish between independent walkers, non-walkers, and device-assisted walkers at hospital discharge. Data were collected through a retrospective medical record review of patients admitted between 1994 and 2001. Mean hospital stays were 66.7 days (SD 88.5, range 7 to 140 days). All children (72 male, 34 female) had recent injuries (from 1 to 8 weeks after onset of traumatic brain injury) and were independent walkers before injury. Sixty-four children (60.4%) were discharged as independent walkers, 13 (12.3%) walked with the assistance of a device, and 29 (27.3%) were non-walkers. Non-walkers had a higher proportion of prolonged loss of consciousness, lower-extremity injury, impaired responsiveness, and lower-extremity spasticity than independent walkers. In addition, non-walkers had poorer discharge mobility and social function scores, longer average hospital stays, and a greater proportion of non-community discharges. Device-only walkers were older, more likely to be male, and had a higher proportion of lower-extremity injuries than independent walkers. Results highlight several demographic, clinical, and outcome variables that distinguish independent walkers from device-assisted walkers and non-walkers. These variables might help to determine the prognosis for ambulation, resource needs, and discharge plans for children and adolescents with traumatic brain injury after episodes of inpatient rehabilitation.

The aim of this study was to document the rate of survival among 288 severely affected pediatric patients (154 females, 134 males) with spasticity and neuromuscular scoliosis who underwent spinal fusion (mean age at surgery 13 years 11 months, SD 3 years 4 months), and to identify exposure variables that could significantly predict survival times. Kaplan–Meier survival analysis was performed demonstrating a mean predicted survival of 11 years 2 months after spinal surgery for this group of globally involved children with cerebral palsy (CP). Cox's proportional hazards model was used to evaluate predictive efficacy of exposure variables, such as sex, age at surgery, level of ambulation, cognitive ability, degree of coronal and sagittal plane spinal deformity, intraoperative blood loss, surgical time, days in hospital, and days in the intensive care unit. Number of days in intensive care unit after surgery and the presence of severe preoperative thoracic hyperkyphosis were the only factors affecting survival rates. This demonstrated statistically significant predictability for decreased life expectancy after spinal fusion in children with CP.

The purpose of this cross-sectional descriptive study was to describe the particular types of behavioral problems, self-concept, and symptoms of depression experienced by children with both low IQ and epilepsy. Three groups of children (83 males, 81 females; mean age 11 years 10 months, SD 1 year 10 months; age range 9 to 14 years) with epilepsy were compared: (Group 1) Low IQ (<85), n=48, 25 males, 23 females; (Group 2) Middle IQ (85 to 100), n=58, 24 males, 34 females; and (Group 3) High IQ (>100), n=58, 34 males, 24 females. The Child Behavior Checklist, Piers–Harris Self-Concept Scale, and Children's Depression Inventory were used to measure behavior, self-concept, and depression respectively. Results indicated that children in the Low IQ group had the most behavioral and mental health problems. Additionally, there were IQ group-by-sex interactions, with females in the Low IQ group being at the highest risk for poor self-concept. Findings suggest that children with both epilepsy and low IQ should be carefully assessed for mental health problems in the clinical setting.

Attention-deficit–hyperactivity disorder (ADHD) is a common neurodevelopmental disorder with a high degree of associated behavioural problems. In order to study characteristics of ADHD with and without oppositional defiant disorder (ODD) in a representative group of young children with clinical impairment in Sweden, 131 children (101 males, 30 females) with ADHD (mean age 5 years, SD 1 year 5 months; range 3 to 7 years) were clinically examined, and their parents interviewed. Independent questionnaire data (Child Behavior Checklist, ADHD Rating Scale-IV, ODD Rating Scale-IV, Conners Hyperactivity Index) were collected. For comparison 131 children without ADHD were matched for sex, age, marital status, and socioeconomic status (115 males, 16 females; mean age 4 years 10 months, SD 1 year 5 months; range 3 to 7 years). Sixty percent of those with ADHD met full DSM-IV criteria for ODD. Only 10 of the 131 children with ADHD had no symptoms of ODD at all. The rate of children meeting full diagnostic criteria for ODD was similar across all age cohorts. Males were overrepresented in ODD, as were children of divorced parents and of mothers with low socioeconomic status. ADHD combined subtype was a stable independent factor influencing the diagnosis of ODD, regardless of psychosocial factors. Those with ADHD with ODD consistently showed higher rates of ADHD symptoms than did those with ADHD without ODD. The prevalent comorbidity of ADHD with ODD indicates that all children presenting with ADHD or ODD symptoms need to be assessed with a view to exploring both types of problem behaviours. The link between ODD and some psychosocial variables indicates the need to address these, possibly by measures such as parent training and network support.

Although premonitory sensory phenomena (PSP) and suppressibility of tics (SPT) are important in Tourette syndrome not only when behavioural therapeutic approaches in children are considered, there is a lack of developmental information on these phenomena. Therefore, a cross-sectional survey of these factors in children and adolescents was carried out. Rates of PSP and SPT were gathered using a questionnaire for the assessment of Tourette syndrome. The 254 outpatients (212 males, 42 females) with Tourette syndrome investigated had an age range of 8 to 19 years, normal intelligence, and diagnosis according to DSM-IV-TR/ICD-10. To test for developmental effects, the total group was stratified into three age groups (8 to 10, 11 to 14, and 15 to 19 years). Data were statistically evaluated using χ2 tests. Of the 254 participants, 37% reported PSP, while 64% were able to suppress their tics. Only a subgroup of 119 patients gave unequivocal answers to both questions and only 60% of these experienced both PSP and SPT. Statistically significant stepwise increases were found at two different age levels. One was around 10 years (PSP ‘Yes’ or ‘No’ and SPT), the other around age 14 (PSP ‘Yes’). There was no influence of tic duration and age at tic onset on PSP/SPT. The reported data suggest that PSP is experienced rarely in younger children with Tourette syndrome and is not a necessary prerequisite for SPT. Increasing PSP with age merely seems to reflect cognitive development rather than intrinsic aspects of Tourette syndrome. In children under 10 years of age, SPT might require more awareness of tics than in older age groups. Developmental aspects of PSP and SPT should be taken into consideration when studies of cognitive behavioural treatment for children and adolescents with Tourette syndrome are planned.

The mitochondrial transfer ribonucleic acid for leucine is encoded by nucleotides 3230-3304. A-to-G transition at nucleotide 3243 can cause maternally transmitted diabetes mellitus–deafness syndrome, and MELAS syndrome. MELAS syndrome is a rare disorder of mitochondrial energy production, and is an acronym for myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. Cortical malformations are heterogeneous and result from abnormal cell proliferation/apoptosis, migration, and/or differentiation of neuroepithelial cells. They are an important and relatively common cause of intractable epilepsy and neurodevelopmental disorders. The association between these A3243G mutations and cortical malformation has never before been reported. Here a 14-year-old female with A3243G mutation and polymicrogyria is described and possible aetiologies of this association are discussed.

Fibromuscular dysplasia (FMD) is a systemic arteriopathy which tends to affect renal arteries followed by cervicocranial vessels. It can lead to cerebral infarction if cephalic arteries are involved. FMD is an unusual cause of stroke in childhood that generally affects the carotid area. Only four cases of vertebral FMD and subsequent stroke have been reported previously and we present the youngest patient of all. A healthy 3-year-old female was admitted to Hospital Doce de Octubre in Madrid, Spain with cerebellar infarction. Angiography disclosed basilar artery thrombosis and typical signs of FMD in both vertebral arteries. No other angiographic alteration was noted in the other vessels studied. Her phenotype and other investigations were unremarkable. The patient was treated with anti-aggregation therapy (aspirin) and the outcome was excellent. Investigation of the occurrence in childhood of this kind of arteriopathy may lead to clarification of its natural history and speculation about its unclear pathogenesis.

The conditions subsumed under the term ‘autistic spectrum disorder’ (ASD) vary in severity at the time of initial presentation; they also vary in expression over time. Unfortunately, the most widely adopted reference standard for the diagnosis of ASD, the Diagnostic and Statistical Manual of Mental Disorders, 4th edition (DSM-IV), does not quantify severity of expression of ASD at the time of initial diagnosis, nor can it be used to document longitudinal changes in symptom expression, other than to state in a ‘yes/no’ fashion that an individual might no longer meet criteria for diagnosis. In clinical practice, measures intended to quantify the severity of ASD, such as the Childhood Autism Rating Scale, the Gilliam Autism Rating Scale, or the Autism Diagnostic Observation Schedule (ADOS), are typically given once, rather than serially (unlike IQ or academic achievement testing, which are commonly given every 2 or 3 years, if not annually). Finally, the diagnosis of ASD is generally made without reference to the affected individual's level of general intelligence. Most scales for assessing ASD do not take the participants' chronological age or level of general development into account, although there are exceptions. These clinical practices constrain our thinking about ASD. Here I present a schema for thinking about ASD along three dimensions: (1) severity of atypical symptoms, (2) level of general cognitive ability or comorbid mental retardation, and (3) age. This schema is not meant as a diagnostic instrument in and of itself. Rather, it is intended as a frame of reference within which to locate the scores of existing instruments, and as a way of conceptualizing ASD. I suggest that this frame of reference will lead to improved clinical care for individuals of all ages with ASD, and might point the way toward further researchable questions in areas pertaining to the etiology, educational management, and epidemiology of autism and related disorders.