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

Prolonged convulsive seizures are a common neurological emergency and a potential cause of neuronal damage and functional sequelae. We explored the role of seizure duration and various background factors for neurological sequelae in children with prolonged convulsive seizures. The population-base of this study was all children (age <16 years) who had been admitted to the Tampere University Hospital, Finland between 1993 and 1999 with convulsive seizures lasting more than 5 minutes. Patients were followed up individually (mean length of follow-up 2 years 1 month, range 0 to 7 years 8 months). All available data on the prolonged seizure episodes and clinical follow-up were analyzed retrospectively by a detailed review of all medical charts and records. In 186 children (94 males, 92 females; mean age 4 years 5 months, SD 3 years 10 months, range 1 month to 15 years 4 months) there were 279 separate convulsive seizure episodes lasting over 5 minutes, yielding an annual incidence of 47.5 out of every 100000 episodes. Seizure aetiology was idiopathic in 26.2% of episodes, febrile in 41.9%, remote symptomatic in 28%, and acute symptomatic in 3.9% of episodes. Mean duration of all seizure episodes was 42.5 minutes (SD 46.1 minutes) and was significantly correlated with the aetiology: shortest in the febrile group (mean 35.4 minutes) and longest in the acute symptomatic group (mean 88.6 minutes; p<0.001). There was no mortality related directly to these acute seizure episodes. The most common sequela was an onset of epilepsy in 40 children (22%). Permanent neurological sequelae were noted in only four patients (2.2%; mean seizure duration 16 minutes) and non-permanent sequelae in six patients (3.2%; mean seizure duration 38 minutes). Neurological sequelae of prolonged convulsive seizures in children are rare and are related to aetiological factors rather than the duration of a single seizure. The role of acute seizures in the evolution of epilepsy in children remains obscure.

The Paediatric Pain Profile (PPP) is a 20-item behaviour rating scale designed to assess pain in children with severe neurological disability. We assessed the validity and reliability of the scale in 140 children (76 females, mean age 9 years 11 months, SD 4 years 7 months; range 1 to 18 years), unable to communicate through speech or augmentative communication. Parents used the PPP to rate retrospectively their child's behaviour when ‘at their best’ and when in pain. To assess interrater reliability, two raters concurrently observed and individually rated each child's behaviour. To assess construct validity and responsiveness of the scale, behaviour of 41 children was rated before and for four hours after administration of an ‘as required’ analgesic. Behaviour of 30 children was rated before surgery and for five days after. Children had significantly higher scores when reported to have pain than ‘at their best’ and scores increased in line with global evaluations of pain. Internal consistency ranged from 0.75 to 0.89 (Cronbach's alpha) and interrater reliability from 0.74 to 0.89 (intraclass correlation). Sensitivity (1.00) and specificity (0.91) were optimized at a cut-off of 14/60. PPP score was significantly greater before administration of the analgesic than after (paired-sample t-tests, p<0.001). Though there was no significant difference in mean pre- and postoperative scores, highest PPP score occurred in the first 24 hours after surgery in 14 (47%) children. Results suggest that the PPP is reliable and valid and has potential for use both clinically and in intervention research.

We investigated in children with attention-deficit–hyperactivity disorder (ADHD) the ability to programme and execute goal-directed arm movements. The sample consisted of 25 males with ADHD (mean age 11 years 6 months, SD 1 year 11 months, range 8 to 15 years) and 25 age-matched typically developing males. The children moved a cursor on a screen by moving a hand-held indicator on a horizontal digitizing tablet. Start and target positions on the screen were always visible during the movement. The screen cursor, however, could either be visible throughout the movement (visual feedback) or blanked at movement initiation (without visual feedback). Analysis showed that movement control was impaired in children with ADHD and that their problems were especially pronounced during the without-visual-feedback condition. In this condition, the children with ADHD exhibited large end-point errors and prolonged movement durations. As there can be no visual corrections of the movement during this condition, results indicate poorer motor programming in children with ADHD. Moreover, children with ADHD performed jerky movements and showed a reduced capacity to select a movement speed that met with the accuracy demands of the movement.

The aim of the present study was to analyze the ability to programme and execute reaching movements in individuals with myelomeningocele (MMC) and in a control group. Thirty-one participants (18 males, 13 females; mean age 12 years 11 months, SD 2 years 7 months, range 9 to 19 years) with MMC and 31 participants (matched for age and sex) without disabilities were investigated. Reaching was performed with and without visual feedback toward three targets displayed on a computer screen and data were collected using a digitizing tablet linked to a computer. The kinematics of reaching were analyzed and analysis of variance was used for statistical analysis. Results showed that both groups were able to programme reaching movements under both visual conditions. Although the execution of reaching was poor in the MMC group compared with the control individuals, as indicated by larger end-point errors (p=0.002), less straight movements (p=0.018), and shorter deceleration phases (p=0.004), movement time was not prolonged in the MMC group. Those with shunt treatment (n=21) had more difficulties when visual feedback was provided. Those with symptoms of early brainstem dysfunction (n=5) had shorter deceleration phases under both visual conditions.

This study compared the energy cost of walking in children with cerebral palsy (CP) classified at different levels of the Gross Motor Function Classification System (GMFCS) with that in children with typical development. Sixteen female and 14 male children with CP (mean age 9 years 6 months, SD 2 years 4 months, range 6 years 4 months to 13 years 4 months) and 14 male and 13 female typically developing children (mean age 10 years, SD 1 year 6 months, range 7 years 1 month to 12 years 11 months) participated. Children with CP were classified at GMFCS level I, n=5; level II, n=10; level III, n=9; and level IV, n=6. Energy cost was assessed by the gas dilution method as each child walked around an oval track wearing a dilution mask. Significant differences were found across GMFCS levels (p<0.0001) and between adjacent levels (p[les ]0.013). Children with CP displayed a higher energy cost of walking than the typically developing children (p<0.0001). A strong correlation (0.87) was found between the energy cost of walking and GMFCS level (p<0.01) when children with typical development were assigned a GMFCS level of zero to allow statistical analysis. This indicates increasing energy cost of walking with increasing severity of functional involvement. These differences in energy cost across GMFCS levels provide another distinguishing factor between GMFCS levels and further emphasize the importance of considering metabolic demand in determining treatment options.

The aim of this study was to determine the effectiveness of an intraoral appliance, the Innsbruck Sensorimotor Activator and Regulator (ISMAR), in improving drooling and eating skills in a group of children with cerebral palsy, and to determine which factors might indicate good candidates for this type of treatment. Eighteen children (13 males, five females; mean age 7 years 10 months, range 4 to 13 years) were selected. Measures of drooling and feeding skills were taken at baseline, at the completion of a 6-month control phase, and at two more 6-monthly time points after the ISMAR was fitted. Children varied greatly in both the length of time taken to tolerate wearing the ISMAR and duration for which the appliance was worn. Only six children (four females, two males) completed the full study. Their motor disabilities were athetosis (n=3), spastic quadriplegia (n=2), and hypotonia (n=1); four of the six children used a wheelchair for locomotion. None had epilepsy and none had greater than mild cognitive impairment. For these children drooling severity scores and eating and drinking skills improved significantly over the treatment period in comparison with the control phase. We conclude that the ISMAR remains a valid option in improving drooling in children and merits further study.

Very-low-birthweight (VLBW) individuals are at high risk of brain injury in the perinatal period. We wished to determine how such early brain lesions affect brain structure in adulthood. Thirty-two VLBW adults (20 female, 12 male) and 18 term, normal birthweight sibling control individuals (nine female, nine male) underwent structural MRI at a mean age of 23 years 4 months (range 17 to 33 years; SD 3.4). Images were analyzed using an automated tissue segmentation algorithm in order to estimate whole brain tissue class volumes in native space. Images were then warped to a template image in standard space. There was no significant between-group difference in whole brain, grey matter, white matter, or total cerebral spinal fluid (CSF) volumes. However, lateral ventricular volume was significantly increased by 41% in those with VLBW. The ratio of grey to white matter was also significantly increased (by 10%) in those with VLBW. Group comparison maps showed widespread changes in the distribution of grey and white matter, and relative excess of ventricular CSF, in the brains of VLBW individuals. Increased ventricular volume predicted decreased grey matter in subcortical nuclei and limbic cortical structures, and decreased periventricular white matter. We conclude that these diffuse abnormalities of grey and white matter are a consequence of the interaction of perinatal brain injury and ongoing neurodevelopmental processes.

Two patients with congenital trigeminal nerve anaesthesia are described. The first (male, aged 14 years) had an isolated unilateral loss of sensation in all three divisions of the trigeminal nerve with no other abnormalities. The second patient (male, aged 3 years 6 months) had bilateral loss of sensation in all three divisions of the trigeminal nerve, associated with other neurological abnormalities. No explanation for their abnormalities was found. The Rosenberg classification of congenital trigeminal anaesthesia is discussed with reference to these patients and also with reference to the developmental biology of the trigeminal nerve.

Partial trisomy of the long arm of chromosome 9 represents a very rare and heterogeneous group of chromosomal aberrations. Associated clinical features include learning disability and pyloric stenosis. We present the first patient to be reported with a duplication of the chromosome region 9q22.1→q33. The patient (female, age 17 years) presented with growth retardation, microcephaly, facial dysmorphia, oesophageal atresia, aortic stenosis, ventricular septal defect, atrial septal defect II, hypothyroidism, and learning disability, but no pyloric stenosis. A review of all cases of partial trisomy 9q reported in the literature demonstrates that learning disability is a characteristic feature of this group of chromosomal aberrations. However, there are cases of duplications of the same chromosome 9 material, with and without pyloric stenosis. This study provides new information for future genetic counselling, especially in cases of prenatal diagnosis of partial trisomy 9q.

The Gross Motor Function Classification System (GMFCS) was developed in response to the need to have a standardized system to measure the ‘severity of movement disability’ in children with cerebral palsy (CP). The Surveillance of Cerebral Palsy in Europe classification ably describes the primary neurological impairment, subtypes, and topography of body involvement but currently includes non-standardized estimations of the degree of functional limitation. The reliability of other classification systems that measure functional ability such as ‘mild’, ‘moderate’ and ‘severe’, or ‘restricted walking’, ‘walking with aids’ or ‘non-walking’, or ‘household and community ambulator’ have not, to our knowledge, been evaluated. Palisano and colleagues used the underlying construct of self-initiated functional abilities in sitting and walking and the need for assistive devices, such as walkers or wheelchairs, to develop a new system, and set out to test its validity and reliability. The GMFCS now provides a method of describing the functional ability of children with CP in one of five levels. Children in Level I can perform all the activities of their age-matched peers, albeit with some difficulty with speed, balance, and coordination; children in Level V have difficulty controlling their head and trunk posture in most positions and in achieving any voluntary control of movement. Over the past few years the developers of the GMFCS have provided further evidence of the measurement properties of the system and it is being widely used. Here we systematically review publications that have cited the GMFCS, to describe the state of our knowledge about its properties and to describe its impact in the fields of research, clinical practice, and education to enhance our understanding of children with CP.