Foreword 2

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Session A: Motor Performance 3

Session B: Botulinum Toxin A 6

Session C: Treatment of Cerebral Palsy 10

Session D: Outcome Scales and Baclofen 12

Session E: Orthopaedic Surgery 15

Session F: Spina Bifida 18

Session G: Selective Dorsal Rhizotomy 22

Session H: Developmental Pediatric Issues 25

Session I: Eclectic 27

Scientific Posters 31

Audio-Visual Program 50

Index 52

In a population-based follow-up study (the Stockholm Neonatal Project), 182 children with a birthweight of 1500g or less (very-low birthweight: VLBW) and a control group of 125 children born healthy at term were examined with the Wechsler Preschool and Primary Scale of Intelligence–Revised (WPPSI–R) and a neuropsychological test battery (Nepsy) at 5½ years of age. The WPPSI–R results of the VLBW children fell well within the normal range: WPPSI–R full-scale IQ 95.7, verbal subscale IQ 99.9, and performance subscale IQ 91.6. Nevertheless, the control group had significantly better results than the VLBW group which could be attributed to the greater variability of the VLBW group, with a larger proportion falling in the lower area of the IQ distribution, especially on the performance subscale. Likewise, the control group displayed better executive function (Nepsy). Paternal education was equal in the two groups and was the single most important predictor of IQ, possibly acting as a protective factor. The need for glasses or lenses was inversely associated with all IQ measures and severe retinopathy of prematurity (ROP) had the most negative impact on full-scale and performance IQ. These two IQ measures were also negatively associated with intrauterine growth retardation late in pregnancy. We conclude that VLBW children, in the absence of these identified risk factors, have normal cognitive development.

The aim of the study was to examine whether school performance was reduced in children with very-low birthweight beyond what could be explained by the deficit in intelligence-score at 4 years of age. Participants (n=333) examined at 4 years of age included three subgroups on the basis of birthweight: very-low birthweight (VLBW <1501g; n=102), low birthweight (LBW 1500–2300g; n=139), and normal birthweight (NBW [ges ]2500g; n=92). The general cognitive index index (CGI) from the McCarthy Scales of Children's Abilities was used as an intelligence measurement at this assessment. At 18 to 20 years of age a telephone interview concerning school performance was conducted. Two hundred and fifty-seven participants (53% male, 47% female) entered the analysis. A model with School Performance score after the last year of compulsory school as the outcome variable, and McCarthy GCI and birthweight group as predicting variables, was used in linear regression analyses. Twenty-four per cent of the variance was explained. Birthweight had no influence in this model. In conclusion, the study did not find that birthweight influenced school performance beyond that represented by the McCarthy GCI found at age 4 years.

The purpose of the present study was to determine the long-term outcome of 76 children (40 females and 36 males) diagnosed and treated with modern antituberculosis drugs. The median age of the children on admission was 29.5 months and on follow-up 9 years. Antituberculosis therapy consisted of daily isoniazid (20mg/kg), rifampicin (20mg/kg), ethionamide (20mg/kg), and pyrazinamide (40mg/kg) for 6 months. Twenty-three children received daily prednisone (2–4mg/kg) for the first month of treatment. Raised intracranial pressure was actively monitored and treated. Patients with non-communicating hydrocephalus received ventriculo-peritoneal shunts shortly after admission while communicating hydrocephalus was treated with oral acetazolamide (100mg/kg/day) and furosemide (1mg/kg/day) in 3–4 divided doses. Communicating hydrocephalus that did not respond to this regimen within the first month of treatment also underwent ventriculo-peritoneal shunting. Only 20% of children were functionally completely normal at follow-up. Main areas of functional deficit were cognitive impairment (80%), poor scholastic progress (43%), and emotional disturbance (40%). Twenty-five per cent of children had evidence of motor impairment, but all could walk and only 5 of 76 children (6% of total) were unable to run. One child was blind but no child had sensori-neural deafness. It was concluded that these disabilities in children from mainly deprived socioeconomic backgrounds have serious implications for their future social, academic, and career prospects. A high index of suspicion of TBM in high tuberculosis incidence communities will help prevent the morbidity documented in this study.

It is well known from behavioural experiments that children with specific language impairment (SLI) have difficulties discriminating consonant–vowel (CV) syllables such as /ba/, /da/, and /ga/. Mismatch negativity (MMN) is an auditory event-related potential component that represents the outcome of an automatic comparison process. It could, therefore, be a promising tool for assessing central auditory processing deficits for speech and non-speech stimuli in children with SLI. MMN is typically evoked by occasionally occurring ‘deviant’ stimuli in a sequence of identical ‘standard’ sounds. In this study MMN was elicited using simple tone stimuli, which differed in frequency (1000 versus 1200Hz) and duration (175 versus 100ms) and to digitized CV syllables which differed in place of articulation (/ba/, /da/, and /ga/) in children with expressive and receptive SLI and healthy control children (n=21 in each group, 46 males and 17 females; age range 5 to 10 years). Mean MMN amplitudes between groups were compared. Additionally, the behavioural discrimination performance was assessed. Children with SLI had attenuated MMN amplitudes to speech stimuli, but there was no significant difference between the two diagnostic subgroups. MMN to tone stimuli did not differ between the groups. Children with SLI made more errors in the discrimination task, but discrimination scores did not correlate with MMN amplitudes. The present data suggest that children with SLI show a specific deficit in automatic discrimination of CV syllables differing in place of articulation, whereas the processing of simple tone differences seems to be unimpaired.

To investigate the relationship between the presence of language disorder, type of epilepsy, and epileptic seizures in childhood, language levels, types of language impairment, and aetiologies were examined in 109 children, aged between 5 and 17 years, attending a national children's epilepsy assessment unit over a 4-year period. There were 70 males and 39 females. Median age was 11 years 4 months (range 5 to 18 years 9 months). In addition to neurological assessment, simultaneous video and EEG monitoring and prolonged ambulatory EEG, each child underwent a comprehensive series of multidisciplinary tests, including intelligence, language, and communication assessments. Classification of seizures and epilepsy syndromes was agreed in conference by a physician specializing in childhood epilepsies, a paediatric neurologist, and a neuropsychiatrist. Other test procedures were administered by a speech and language pathologist with assistance from a neuropsychologist when relevant. Level of language disability in these children was associated with a range of aetiological factors. Evidence was found of a significant number of associations between focal epilepsies, certain seizure types, and language disorder. Of the 46 (42.2%) children with language disorders in the research sample, 30 had localization-related epilepsies and a further three had epilepsies which were undetermined as to whether focal or generalized. Children with focal epilepsies were 30% more likely to have language disorder than other language disability subtypes. The research demonstrated a clear though often subtle association between focal (localization-related) epilepsy and language disorder, indicating an increased risk in this patient group. Children with simple or complex partial seizures were more likely to have language disorder than other language disability subtypes; they also tended to have the lowest number of seizure types per case. This is of importance to clinicians managing children with epilepsy and has implications for their educational and social welfare.

The objective of this study was to examine the descriptive epidemiology of vision impairment among 6- to 10-year-old children in metropolitan Atlanta, Georgia, USA. Children with vision impairment (n=310; 42% black, 56% white; 57% male, 43% female), defined as a best corrected visual acuity in the better eye of 20/70 or worse, were identified through the Metropolitan Atlanta Developmental Disabilities Surveillance Program. The overall prevalence was 10.7 per 10000 children. Fifty-nine percent had low vision; nearly two-thirds had coexisting disabilities. Educational program varied by vision impairment severity and presence of coexisting disabilities. The common presence of coexisting disabilities emphasizes the importance of multidisciplinary services. The inclusion of case ascertainment sources other than vision impairment classes is critical to ensure an accurate prevalence rate and unbiased description of children with vision impairment.

The aim of the study was to describe ocular motility in a sample of 56 patients affected by cerebral visual impairment (CVI) of hypoxic–ischemic origin. The sample consisted of 56 participants (37 males and 19 females), ranging in age from 2 to 16 years. In all cases CVI was associated with MRI-verified damage of the cerebral visual system. A complete ophthalmologic and neurological assessment was performed. Behaviour of gaze was studied in four conditions: during scanning of the surrounding environment, during fixation, execution of saccades, and pursuing. In addition, strabismus, nystagmus, and paroxysmal ocular deviations were evaluated. Ocular motility was studied by video recording the patients' eye motility during orthoptic examination. Each pattern of ocular motility studied revealed profound alterations in all the individuals examined. Typical features of ocular motility in CVI were: paroxysmal ocular deviations (present in 78%); the presence of variable angle strabismus (86%); and defective coordination of saccades (93%). Exploration of the environment and fixation were also impaired (88% and 84%, respectively). Disorders of initiation and performing saccades, absence of smooth pursuit, vergence abnormalities, nystagmus beats, instability of fixation, and difficulty in the systematic exploration of the environment were observed. These abnormalities characterize lack of gaze coordination found in children with brain damage. An early and detailed evaluation of ocular motility in individuals with CVI is important, especially when rehabilitation intervention is intended.

Despite several trials showing reductions in tone and improvements in gait, the relation between botulinum toxin A (BTX-A) dose and response has rarely been investigated. A double-blind randomized comparison of two doses of BTX-A in children with spastic hemiplegic cerebral palsy (n=48, mean age 7 years 6 months, range 3 to 15 years) was undertaken. The two doses selected were representative of the lower and the higher doses used in clinical practice (24 units/kg body weight and 8 units/kg body weight). Using gait analysis we evaluated hip, knee, and ankle joint kinetics and sagittal kinematics throughout the gait cycle. Gastrocnemius muscle length was calculated at each visit using the method described by Eames and used as our primary outcome measure. Our secondary outcome variable was maximum ankle angle measured during stance and swing phases. In summary, we found that there were indications that 24 units/kg body weight was more effective and lasted longer than 8 units/kg. Analysis in terms of absolute dose suggested that the dose–response correlation was non-linear, and that the optimal range lay between 200 and 500 units BTX-A (Dysport).

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.

In the past 40 years, children with minor developmental motor disorders have been studied by professionals from various fields: paediatricians, neurologists, psychiatrists, psychologists, and physical and occupational therapists. The differences in background of these professionals led to a plethora of terms used to refer to these conditions: e.g. minimal cerebral palsy, minimal cerebral dysfunction, developmental apraxia or dyspraxia, minimal brain dysfunction, sensory integrative dysfunction, and developmental coordination disorder. It was generally acknowledged that such a multitude of names was confusing and so, in 1994, an international consensus meeting of professionals from various fields agreed to use the term ‘developmental coordination disorder’ (DCD). DCD in general refers to children with normal intelligence who have poor motor coordination without clear evidence of a neurological pathology, such as cerebral palsy (CP) or muscular dystrophy. In other words, DCD is a ‘final common output’ term, without aetiological or pathophysiological foundation. The advantage of such an umbrella term is that it includes all children with motor problems that interfere with their daily life, i.e. children who deserve clinical attention. However, the disadvantage of the term DCD is its aspecificity. Indeed, it is becoming increasingly clear that children with DCD constitute a heterogeneous population with various types of motor dysfunction.

A 6-year-old female presented with right hemichorea, initially thought to be post-streptococcal, which subsequently progressed to a right dystonic hemiplegia. At 7½ years she developed right focal and secondary generalized tonic–clonic seizures. These became intractable. A brain biopsy was consistent with Rasmussen's encephalitis (RE). At 9½ years the child underwent a left hemispherectomy making a good recovery with resolution of seizures. At 12 years she presented with recurrent episodes of abdominal pain, fever, and malar rash with serological evidence of systemic lupus erythematosus (SLE). A possible link between RE and SLE is discussed.