Foreword 2

Oral Presentations

Session 1: Neuromuscular/Encephalopathy 3

Session 2: Epilepsy 5

Session 3: Stroke/Neurovascular 9

Session 4: Videos 11

Session 5: Neurodisability/Miscellaneous 13

Session 6: Breaking News 16

Posters

Session 1: Neuromuscular/Encephalopathy 17

Session 2: Epilepsy 19

Session 3: Stroke/Neurovascular 24

Session 4: Neurodisability/Miscellaneous 26

Index 33

The aim of this study was to establish the reliability and validity of visual gait assessment in children with spastic diplegia, who were community or household ambulators, using a modified version of the Physicians Rating Scale, known as the Observational Gait Scale (OGS). Two clinicians viewed edited split-screen video recordings of 20 children/adolescents (11 males, 9 females; mean age 12 years, range 6 to 21 years) made at the time of three-dimensional gait analysis (3-DGA). Walking ability in each child was scored at initial assessment and reassessed from the same videos three months later using the first seven sections of the OGS. Validity of the OGS score was determined by comparison with 3-DGA. The OGS was found to have acceptable interrater and intrarater reliability for knee and foot position in mid-stance, initial foot contact, and heel rise with weighted kappas (wk) ranging from 0.53 to 0.91 (intrarater) and 0.43 to 0.86 (interrater). Comparison with 3-DGA suggests that these sections might also have high validity(wk range 0.38–0.94). Base of support and hind foot position had lower interrater and intrarater reliabilities (wk 0.29 to 0.71 and wk 0.30 to 0.78 respectively) and were not easily validated by 3-DGA.

This study was designed to test the hypothesis that non-ambulatory patients with spastic quadriplegia will have reduced bone mass which worsens with increasing age. Forty-eight patients (age 5 to 48 years, median age 15 years; 19 females and 29 males) were studied. Anticonvulsants were used in 29 patients (60.4%). Lumbar spine bone mineral density (LS-BMD) was markedly reduced compared with age- and sex-matched control individuals with a z score of –2.37±0.21. Twenty-eight (58%) had z scores of less than –2. A history of documented previous fracture was present in 19 patients (39%). Patients with a history of fracture had significantly lower (p=0.05) LS-BMD z scores (–2.81±0.29) compared with those without a history of fracture (–2.11±0.26). Mean serum 25-OH vitamin D was 29.6±1.9ng/mL (normal 9 to 37.6ng/mL) with three patients having serum 25-OH vitamin D levels less than 15ng/mL. These findings indicate that BMD is markedly reduced in non-ambulatory children and adults with neuromuscular disease. Reductions in bone mass put them at greater risk for non-traumatic fractures.

In order to test the practicability and safety of whole-body cooling in term neonates with moderate-to-severe hypoxic–ischaemic encephalopathy (HIE) and to report outcomes, a prospective pilot study was carried out in 25 term infants (median postmenstrual age 38 weeks, range 36 to 41 weeks; 20 males, five females). Whole-body cooling, to a target core temperature of 33 to 34°C, started within 6 hours of birth and was maintained for 72 hours. Of the 25 newborn infants (19 Sarnat II and six Sarnat III, 18 outborn), 18 survived, including 13 (72%) with normal cerebral signal by MRI. Temperature instability occurred during cooling in 15 infants, but neither severe haemodynamic instability nor renal failure was seen. Thrombocytopenia developed in 12 infants, including seven with biological disseminated intravascular coagulation. One patient had hypoxaemia with right-to-left shunting through the ductus arteriosus, and seven had limited meningeal or subdural bleeding. Whole-body cooling is feasible in term neonates, with no life-threatening adverse events. Improvements are needed to obtain stable hypothermia for 72 hours.

The effect of asphyxia on iron metabolism and lipid peroxidation in newborn infants with hypoxic–ischemic encephalopathy (HIE) was investigated. Non-protein-bound iron (NPBI) and lipid peroxidation (thiobarbituric-acid-reactive species; TBARS) in plasma and hematological iron indices were measured in 15 healthy newborn infants (mean gestational age 39 04weeks, SD 1); 15 asphyxiated infants without neurological abnormalities (AS–HIE; mean gestational age 38.8 weeks, SD 0.9); and 15 asphyxiated infants with neurological abnormalities (AS+HIE; mean gestational age 39.75 weeks, SD 1.4). Follow-up was performed at the age of 5 months. It was found that the detectable rates of NPBI in 10 of 15 of the AS–HIE group and 13 of 15 of the AS+HIE group were significantly higher than that of the control group (5 of 15; both p<0.01). Plasma levels of TBARS in the control (9.20µmol/L, SD 1.9) and AS–HIE infants (10.13µmol/L, SD 2.7) were significantly lower than those of the AS+HIE group (13.42µmol/L, SD 2.8). Serum iron, total iron binding capacity, and transferrin saturation in the AS+HIE group was higher than the corresponding values of the control and AS–HIE groups, although no statistical difference was found among them. At 5 months of age, all control and AS–HIE infants were neurologically normal, whether or not their NPBI was detectable. Of the 12 AS+HIE infants, four (all of whom had detectable NPBI) were neurologically impaired. The average Gross Development Quotient of AS+HIE infants was significantly lower than that of the control or AS–HIE groups (p<0.01). Results showed that asphyxia could affect iron metabolism and lead to a significant increase in NPBI and lipid peroxidation in newborn infants with HIE, indicating that iron delocalization induced by asphyxia plays a role in the brain injury of asphyxiated infants.

To determine the long-term risks and benefits of a separate CSF reservoir in the management of 52 children (23 males, 29 females) with shunted hydrocephalus, a retrospective study was performed comparing the use and complications after separate reservoir insertion, with a prereservoir control period. Median age at first shunt insertion was 1 month and median age at reservoir insertion was 2 years 6 months. Median follow-up for shunt with the additional reservoir was 19 years 1 month. There was no mortality due to shunt failure nor CNS infection, and there were significantly fewer episodes of ventriculitis (p<0.01) and shunt blockage (p<0.0001) compared with the prereservoir period. There was no hemiplegia, epilepsy, visual, nor cognitive loss from the additional reservoir. The reservoir was used for access in 344 attendances (mean 6.62 attendances per patient) for diagnosis or treatment of raised pressure or CNS infection. It was concluded that a separate CSF reservoir is useful in the long-term management of patients with shunted hydrocephalus and is without mortality or significant increased morbidity.

This study investigated the occurrence, nature, and severity of speech, language, and cognitive impairment in 76 children (61 males, 15 females) with isolated sagittal synostosis (ISS) aged 9 months to 15 years 7 months. There was no increased prevalence of global cognitive impairment in the group but there was a high prevalence rate of speech and/or language impairment with 28 (37%) displaying impairment of whom 20 (71%) had moderate or severe impairments that fulfilled the criteria for specific impairments. Prevalence rates were only increased for children over two years of age. Expressive language impairment occurred most frequently. Raised intracranial pressure, peri-neonatal risk factors, otitis media, or being operated were not associated with impairment. Surgery at a later age and a family history of speech and language impairment were both associated with impairments but numbers were small. The findings suggest that children with ISS are at an increased risk of developing speech and language impairment.

Spinal muscular atrophy (SMA) is a chronic illness characterized by loss of motor function. The aim of the study was to investigate behavioural adjustment in 96 children and adolescents with SMA (47 males, 49 females; mean age 11 years 2 months, range 6 to 18 years). Forty-five non-affected siblings (26 males, 19 females; mean age 11 years 6 months, range 6 to 18 years) and 59 normally developing children (33 males, 26 females; mean age 10 years 8 months, range 6 to 18 years) were recruited as control participants. Behavioural symptoms were measured with the Child Behaviour Checklist (CBCL) and disorders were assessed with a structured psychiatric interview (Kinder-DIPS). Of the patients with SMA, 12.5% fulfilled the criteria for an ICD-10 or DSM-IV diagnosis, with separation anxiety disorder being the most common diagnosis. The CBCL total score was in the clinical range for 11.5% of patients, 20% of the siblings, and 11.7% of the control children; the externalizing score rates were 2.1%, 22.2%, and 11.9% respectively; the internalizing score 18.9%, 24.4%, and 13.6% respectively. Comorbid psychopathology was not influenced by sex, IQ, nor severity of SMA, and only externalizing behaviour was correlated to age. In conclusion, children and adolescents with SMA are characterized by a low psychiatric comorbidity not different from control individuals. The group with the highest rate of behavioural problems and with the greatest need for intervention were the non-affected siblings who had a two- to threefold higher rate of behavioural problems than the normative population.

Attention-deficit–hyperactivity disorder (ADHD) has been associated with childhood epilepsy; prevalence figures have ranged from 8 to 77%, depending on the sample studied and the criteria used for diagnosis. In the general population the prevalence of ADHD is approximately 5%, with the majority of affected children having ADHD combined type. As part of a larger study of behavioral problems in children with epilepsy, we assessed 175 children (90 males, 85 females; age range 9 to 14 years, mean age was 11 years 10 months, SD 1 year 8 months) for evidence of ADHD. The children had at least a 6-month history of epilepsy. The primary caregiver completed both the Child Behavior Checklist (CBCL) and the Child Symptom Inventory-4 (CSI) or Adolescent Symptom Inventory-4 (ASI). On the CBCL, the mean attention problem T score was 64.6 (SD 10.5) for adolescents and 67.9 (SD 11.6) for children. On the CSI or ASI, 20 of 175 children met DSM-IV criteria for ADHD combined type; 42 of 175 had ADHD predominantly inattentive type; and 4 of 175 met criteria for ADHD predominantly hyperactive–impulsive type. There were significant correlations between the CBCL attention score and inattention (r=0.68) and hyperactivity–impulsivity (r=0.59). Sex, seizure type, and focus of seizure discharge were not predictors of symptoms of ADHD. Children with epilepsy are at risk for symptoms of ADHD. They differ from other samples of children with ADHD by the higher proportion of children with ADHD predominantly inattentive type and by an equal male:female ratio.

We investigated a male, aged 4 years 11 months, who fulfilled the criteria of Asperger syndrome). In addition to the typical pattern of autistic symptoms, psychological testing revealed prosopagnosia in tasks for face recognition and matching. Prosopagnosia was also present when he tried to identify the faces of his parents and himself in photographs whenever these were presented with photographs of other persons. Although impairment in reciprocal social interaction in individuals with Asperger syndrome is closely correlated to their impaired perceptional abilities in non-verbal communication, especially facial expression, overt prosopagnosia seems to be a rare neuropsychological symptom in persons with autistic disorders.

In the current practice of physical therapy for children with chronic physical disabilities, more and more attention is paid to parental participation. In the past, physical therapy was mainly impairment oriented, with supposed effects on the daily functioning of the child. It was the exclusive domain of therapists and physicians. Parents were usually not involved in the therapy for their children. It was not until recently that physical therapists became more aware of the possible role of parents. In the early 1980s an important development in this area started. The role of parents changed from a passive one into an active participating one. Parents became a sort of co-therapist, meaning that while therapists decided on the treatment goals and methods, parents were expected to perform the treatment activities at home in cooperation with the treatment activities of the therapist in the clinic. Since then, the discussion about the optimal role of parents in the physical therapy of their children has continued. This discussion has resulted in the current belief that parents should be actively involved, and should be working with therapists as equal partners in deciding the goals and content of the physical therapy for their children.