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Foreword 2

Session A: Botox - Non-Surgical 3

Session B: Upper Extremity 6

Session C: Adult Cerebral Palsy 9

Session D: Orthopaedic Aspects of Myelomeningocele 12

Session E: Intrathecal Baclofen Therapy 16

Session F: Surgery 19

Session G: General Pediatrics 22

Session H: General Pediatrics and Scales 25

Session I: Selective Dorsal Rhizotomy 28

Scientific Posters 32

Audio-Visual Program 44

Index 48

There is now substantial evidence to suggest that the presence of inflammatory molecules within the fetal circulation and brain is associated with damage to vulnerable oligodendrocyte precursor cells before 32 weeks' gestation and long-term white matter damage (WMD); however, the nature of the inflammatory stimulus remains unclear. The common association between ascending bacterial infection from the vagina, haematogenous spread of periodontal organisms, chorioamnionitis, preterm delivery, and neonatal brain injury provides indirect clinical evidence supporting an important role for bacteria. Animal experiments in which live bacteria, or endotoxins, lead to the classical focal necrotic lesions of periventricular leukomalacia add further support to this concept. The isolation of bacteria-specific DNA from only a small proportion of cases where there is evidence of histological chorioamnionitis, suggests that there may be non-bacterial causes of fetal inflammation. Viruses make attractive alternative candidates; they are a common cause of maternal pyrexia, can cross the placenta (e.g. cytomegalovirus and rubella), are known to cause WMD and microcephaly, and are difficult to isolate. A recent study of viral DNA in stored neonatal blood spot specimens suggested a possible link between viral exposure and later cerebral palsy, particularly in preterm babies. Dammann and Leviton have been even more specific, identifying maternal pestivirus (PV) infection as a hypothetical cause of WMD in preterm neonates.

Systematic studies of cognitive development in individuals with congenital giant interhemispheric cysts (GIC) without neurosurgical intervention are rare. However, data from such studies are of great importance in deciding whether or not to perform cerebral shunting in otherwise asymptomatic patients. In this study, psychomotor function was examined in 13 children (three females, 10 males; mean age at follow-up: females 1 year 6 months, males 3 years 2 months; age range: females 13 months to 4.2 years, males 10 months to 10 years) all diagnosed with GIC during the neonatal period after indicative foetal ultrasound. Eight patients received neurosurgical treatment due to increased intracerebral pressure during the first year of life. Psychomotor development was evaluated using Griffith's Psychomotor Developmental Scales, the Sniders–Oomen Non-verbal Intelligence Scale for Young Children and the Kaufmann Assessment Battery for Children. The majority of patients showed normal intelligence, irrespective of the presence or absence of neurosurgical intervention. Evidence was found that the cognitive outcome might be related to an association of GIC with agenesis of the corpus callosum or additional congenital cerebral malformations, such as gyration anomalies. Main postoperative complications were chronic subdural effusions with spastic cerebral (hemi)-paresis; were present in three patients. In view of the risk of severe postoperative neurological complications and a comparable psychomotor development in patients without neurosurgical treatment, recommendations for early neurosurgical intervention in otherwise asymptomatic patients with a foetal or neonatal diagnosis of GIC should be viewed with caution.

Eliasson et al. are to be warmly congratulated on the publication of their excellent paper ‘The Manual Ability Classification System (MACS) for children with cerebral palsy: scale development and evidence of validity and reliability’ in this issue of Developmental Medicine and Child Neurology.

Transient neurological deficits experienced by patients with Sturge–Weber syndrome can be caused by epilepsy, or may result from temporary ischaemia of the cortex underlying the vascular malformation. To show the difficulty in distinguishing seizures from ischaemic symptoms, two male children with episodes of acute unilateral weakness are presented here as well as a review of the literature. The first child presented at 2 years of age with a sudden increase in his pre-existing right hemiparesis accompanied by screaming. Ictal epileptiform activity was recorded at the moment of the attack, and subsequent seizures were controlled by adjustment of antiepileptic drug treatment. The second child presented at 4 years of age with attacks of vomiting and a coinciding increase in the pre-existing paresis of the left leg. Electroencephalogram (EEG) recording did not show ictal epileptiform activity. The origin was presumed to be vascular. Treatment with aspirin led to control of these transient ischaemic attacks. Ictal EEG is needed to differentiate between an epileptic and an ischaemic origin of transient focal deficit. Treatment with aspirin should be considered if an ischaemic origin cannot be excluded.

The purpose of this study was to determine the effectiveness of muscle-release surgery for children with cerebral palsy (CP) using longitudinal and stratified analysis. Twenty-five children with CP (15 females, 10 males; age range 4 to 16 years; mean age 8 years 2 months, SD 3 years 2 months) were selected from five treatment centres in Japan. Twenty-two children had spastic diplegia, two had spastic quadriplegia, and one had athetospastic quadriplegia. Motor function for each child was assessed using the Gross Motor Function Measure (GMFM). Assessment was conducted on eight separate occasions: 1 month and 1 week before surgery, and 1, 2, 4, 6, 9, and 12 months after surgery. Participants' motor function before surgery was classified using the Gross Motor Function Classification System (GMFCS). Six children were classified at level I, three at level II, six at level III, and 10 at level IV. A significant difference was found after surgery in the GMFCS levels III and IV groups (p<0.05). Improvement in GMFM scores between 1 week before surgery and 12 months after surgery were 1, 5, 8.5, and 8.5 for GMFCS levels I to IV respectively. Results indicate that this treatment is advantageous for improving motor function in children within GMFCS levels III and IV.

Giant axonal neuropathy (GAN) is a chronic progressive disease of the peripheral nervous system, but lesions can also occur in the central nervous system (CNS), especially in the brainstem and cerebellum. Its mode of inheritance is autosomal recessive, with parents of affected children often being consanguineous, but sporadic cases have been reported.

Botulinum toxin has rapidly gained wide acceptance as a treatment for focal spasticity, and is becoming more readily available. Early studies established its effectiveness, but many issues are still open for discussion. After a brief review of the historical and scientific development of botulinum toxin as a clinical tool and an outline of previous work in the field, some important questions are discussed in the light of more recent trials.

The Battery for Rapid Evaluation of Cognitive Functions (Batterie Rapide d'Evaluation des Fonctions Cognitives: BREV) is a quick test to screen children with higher-functioning disorders and to define the patterns of their disorders. After standardization tests in 500 normally developing children aged 4 to 8 years, validation consisted of comparative evaluation of the specificity and sensitivity of the BREV with a wide reference battery in 202 children with epilepsy (108 males, 94 females; mean age 6 years 6 months, SD 1 year 8 months). Children were divided into 10 age groups from 4 to 8 years of age and represented eight epileptic syndromes. The reference battery included verbal and non-verbal intelligence assessment using the Wechsler scale, oral language assessment with a French battery for oral language study, drawing with the Rey figure, verbal and visuo-spatial memory with the McCarthy scale subtest and the Rey figure recall, and educational achievement with the Kaufman subtests. Every function evaluated with the BREV was significantly correlated with the reference battery testing a similar function (p=0.01 to 0.001). Specificity and sensitivity of the BREV verbal and non-verbal scores were correlated with those of the Wechsler scale in more than 75% of children. The BREV, therefore, appears to be a reliable test which has been carefully standardized and validated and is valuable in screening for cognitive impairment in children.