Foreword 2

Free Papers

Session A: Orthopaedics 3

Session B: Eclectic 6

Session C: Botulinum Toxin A 9

Session D: Orthopaedics 13

Session E: Early Development 16

Session F: Gait 19

Session G: Functional Scales 23

Session H: Imaging 25

Session I: Quality of Life 29

Scientific Posters 32

Audio-Visual Program 53

Index 55

A detailed study was made of the pathology of specimens removed by hemispherectomy for the treatment of intractable epilepsy in children with unilateral middle cerebral artery stroke. Neuropathological criteria were used to differentiate strokes that occurred in early intrauterine development (before 28 weeks gestational age) from those occurring in the last trimester, at birth, or after birth: 19 children had early strokes and 21 late. There was no difference in seizure history or occurrence of febrile convulsions in these two groups. Hippocampal tissue was available in 20 patients; pathology in the hippocampus, remote from the infarcted area, showed a marked difference between early-onset and late-onset groups. Hippocampal sclerosis was uncommon in children with early-onset strokes but developed in most of the children whose strokes were of later origin. However, hippocampal sclerosis was more closely related to a clinical history of a late initial precipitating insult irrespective of infarct timing. These findings demonstrate the changing vulnerability of the developing brain and show that hippocampal pathology is more closely related to the timing of an insult than seizure history or the occurrence of febrile convulsions.

The aim of this study was to evaluate whether EEG (i.e. positive Rolandic sharp waves) can be used to predict neurodevelopment in newborn infants with periventricular leukomalacia and compare the predictive value with that of MRI. A sequential cohort of neonates (n=45; 33 males, 12 females; mean gestational age 31.2 weeks, SD 2.7, range 27 to 37.8 weeks; mean birthweight 1592g, SD 601g) with periventricular hyperechogenicities on cranial ultrasound was recruited for this study. EEGs were analyzed for positive Rolandic sharp waves. Neurodevelopment was evaluated at the ages of 12 and 18 months. In the whole group the probability of a poor outcome was 24% and the probability of any impairment was 33%. If the number of positive Rolandic sharp waves was no more than 0.1 per minute, the probability of a poor outcome was reduced to 9% (95% confidence interval [95%CI] 2 to 27%) and the probability of any impairment was reduced to 13% (95%CI 4 to 32%). In all infants with more than 0.1 positive Rolandic sharp waves per minute the probability of a poor outcome was 41% (95%CI 23 to 61%) and of any impairment was 55% (95%CI 34 to 73%). In these infants MRI identified infants with a poor outcome with a sensitivity of 1.00 (95%CI 0.70 to 1.00) and a specificity of 0.92 (95%CI 0.67 to 0.99), and infants with any impairment with a sensitivity of 0.83 (95%CI 0.55 to 0.95) and a specificity of 1.00 (95%CI 0.72 to 1.00). Results suggest that if an EEG of an infant with periventricular leukomalacia contains no more than 0.1 positive Rolandic sharp waves per minute the probability of a normal or mildly delayed development is high (0.91, 95%CI 0.73 to 0.98). MRI enhances the accuracy of the outcome prediction slightly; however, owing to a wide confidence interval, this advantage is negligible. However, if the frequency of the positive Rolandic sharp waves exceeds 0.1 per minute, MRI can significantly enhance the precision of the prediction of outcome.

This study examined the effect of massed practice in balance recovery of stability in six children (four males, two females; mean age 9 years 2 months, SD 2 years, range 7 years 5 months to 12 years 11 months) with cerebral palsy (CP). Four children were diagnosed with spastic diplegia (Gross Motor Function Classification System [GMFCS] level II) and two with spastic hemiplegia (GMFCS level I). A single-subject, multiple-baseline experimental design involving three pairs of children matched for diagnosis was used. A moveable forceplate system was used to test and train reactive balance control. Area per second (i.e. area covered by the center of pressure over a one second period) and time to stabilization from center of pressure measures were calculated following perturbations. The intervention phase consisted of massed practice on the moving platform (100 perturbations/day for 5 days). Analysis included hierarchical linear modeling and a repeated measures ANOVA. All children demonstrated a significant improvement in their ability to recover stability as demonstrated by reduced center of pressure area and time to stabilization following training. These improvements were still present 30 days following completion of training. Results suggest that postural control mechanisms in school-age children (7 to 13 years) with CP are modifiable.

Everyday functioning is described in 95 children with cerebral palsy (CP; 55 males and 40 females; mean age 58 months, SD 18 months, range 25 to 87 months) using the three scales of the Pediatric Evaluation of Disability Inventory (PEDI): Functional Skills, Caregiver Assistance, and Modifications of the Environment. Types of CP in the children were hemiplegia, (n=19), spastic/ataxic diplegia, (n=44), spastic quadriplegia, (n=16), dyskinetic, (n=9), and mixed (n=7). Symptoms were grouped by severity according to the Gross Motor Function Classification System (GMFCS): 23% were classified at level I, 21% at level II, 10% at level III, 23% at level IV, and 23% level V. A large variability in functioning in mobility, self-care, and social function was seen because of the heterogeneity of children with CP. Limitations in achievement of activities, need for assistance, and use of assistive devices increased progressively with GMFCS level. Furthermore, these children differed to a great extent from the normative sample of the PEDI. Stepwise regression analysis showed that the GMFCS was a good predictor of everyday functioning with age and learning problems as significantly contributing factors, particularly in self-care and social function. In conclusion, the three scales of the PEDI represent different but strongly related aspects of everyday functioning in young children with CP.

Prevalence of drooling in children with cerebral palsy (CP) and its severity across dental age (i.e. children with primary, mixed, and permanent dentitions) was investigated. One hundred and sixty children and adolescents (91 males, 69 females; mean age 10 years 10 months, SD 4 years 2 months; range 4 to 18 years) with CP who attended special schools were involved in this study. Sixty eight per cent of participants had spastic CP with the majority (45%) diagnosed as having quadriplegia. Presence or absence of drooling was determined by direct observation of the participants. Other data, such as age, sex, learning disability, and type of CP were collected using questionnaires. Results showed 93 of 160 children (58%) with CP had a drooling condition and of these 53 (33%) had severe drooling. It was found that the degree of drooling decreased as the child's dental age increased. Prevalence of drooling in children with primary dentition was found to be 75% as compared with 43% of those with permanent dentition, although this difference was not statistically significant. It was concluded that any invasive treatment to reduce drooling should, perhaps, be postponed until the child's oromusculature has matured.

This study describes the physical disability of 30 children and adolescents with Pompe disease (23 males, 7 females; mean age 7 years 7 months, SD 5 years 6 months; range 6 months to 22 years 1 month) using a disease-specific functional instrument. Data were collected by telephone interview with parents using a modified version of the Pediatric Evaluation of Disability Inventory. The sample included mostly males of Caucasian origin, recruited from several countries. Disability profiles in mobility and self-care skills were heterogeneous because functional status was not related to chronological age. Most children had severe functional deficits: nearly two-thirds of the sample was non-ambulatory and could not perform age-expected self-care skills. Three-quarters of the children used a ventilator. Two children were able to participate in age-appropriate sports and peer activities. Although the mean chronological age of the sample was 7 years 7 months, the mean age-performance for self-care skills was under 2 years 6 months and under 1 year 6 months for mobility. Implications of physical disability findings for individuals with Pompe disease are discussed.

Surface electromyography and accelerometry provide essential information on the neurophysiological characteristics of essential tremor. There are many reports on neurophysiological features in adult-onset essential tremor, but to our knowledge there have been no similar investigations of essential tremor in children. We conducted a neurophysiological study of nine children, six males and three females, with definite essential tremor. They were subdivided into two groups according to age: a ‘children's group’, consisting of four patients aged from 7 to 12 years, and an ‘adolescent group’, consisting of five patients aged from 14 to 16 years. Finger tremor as opposed to hand tremor was studied. In children the mean tremor frequency was 5.3Hz (SD 0.5) with arms extended, which increased to 8.2Hz (SD 1.5) when we added a mass of 300g. In adolescents the mean tremor frequency was 9.0Hz (SD 1.4) with arms extended, and 7.2Hz (SD 1.8) with added mass. We discuss several hypotheses to find an explanation for these results.

The reliability of classification of cerebral palsy (CP) in low-birthweight children was assessed by using clinical and research study records sampled from population-based cohort studies in the USA, the Netherlands, Canada, and Germany. Records of neurological examination findings and functional motor assessments were submitted to up to five pediatricians with expertize in CP diagnosis, who grouped children into categories referred to as ‘disabling’ CP, ‘non-disabling’ CP, and no CP. Each study provided between 31 and 51 records of children assessed between 2 and 8 years of age, approximately equally divided among the three groupings. The discrimination between ‘any CP’ and ‘no CP’ was only fair (mean Kappa coefficients 0.37 to 0.69). However, when more detailed information describing motor function was used, children with ‘disabling’ CP could be distinguished, on the basis of records, from those without CP or with ‘non-disabling’ CP with good to excellent reliability (mean Kappa coefficients 0.69 to 0.88). Because of the substantially higher agreement observed when these functional distinctions are made, we recommend that reports or comparisons of rates of CP should include levels of motor function of children with CP, and not simply total CP, among the outcomes of interest.

A previously neurodevelopmentally intact 5-year-old male was admitted to hospital with a right lower lobe pneumonia with pleural effusion, subsequently confirmed to be a Mycoplasma pneumoniae infection. On the seventh day of the illness he had a prolonged generalized tonic or tonic–clonic convulsion, requiring intubation and ventilation. He was slow to regain consciousness (Child's Glasgow Coma Score 7–10 over 6 days) and brain imaging with CT and then MRI demonstrated bilateral thalamic lesions with oedema and central haemorrhage suggestive of acute bilateral thalamic necrosis, without striatal or white-matter involvement. He was treated with a 2-week course of erythromycin, and as an autoimmune process was considered possible, 5 days of intravenous methylprednisolone (20mg/kg/day) followed by a 4-week oral prednisolone taper. He made a slow recovery over the next few weeks with almost complete neurological recovery by 2 months but with significant dysarthria, drooling, and a mild left hemiparesis. At 9 months, significant dystonia continued to affect his speech and, together with tremor, his upper-limb fine motor function bilaterally. His gait, personality, and higher cognitive functions appeared to have recovered fully. Although acute striatal necrosis, acute disseminated encephalomyelitis, and encephalitis have been reported with Mycoplasma pneumoniae and a similar picture of acute bilateral thalamic necrosis with influenza-A (‘acute necrotizing encephalopathy’), this is the first reported case of Mycoplasma pneumoniae-associated isolated acute bilateral thalamic necrosis.

Tonsillectomy is a frequently performed surgical procedure. Surgical complications can be serious. We report the case of a 7-year-old female who experienced an arterial ischaemic cerebral infarction following elective adenotonsillectomy.

In paediatric outpatient departments it is not uncommon for a child to complain of muscle pain and stiffness. These may be labelled as ‘growing pains’, a diagnosis of exclusion when no obvious cause is found. Pain and stiffness occur most frequently at night and are located usually in the leg muscles, and not in the joints. They are intermittent, and tend to occur after the child has had an especially active day, so that a detailed history which notes pain after almost any amount of exercise (as opposed to stress at the site of the pain) will reveal the differences from McArdle's disease. If examination results are entirely normal investigations are to be avoided unless the symptoms persist. If they do, a number of causes may be found, from orthopaedic abnormalities to metabolic disorders. This paper is concerned with one of the latter: glycogenosis type V or McArdle's disease, a rare autosomal recessive condition resulting from muscle phosphorylase deficiency, an enzyme which initiates the breakdown of glycogen in skeletal muscle. Cardiac muscle is not significantly affected. The condition usually presents in the first two decades of life, but can occur in infancy and adulthood.