Foreword 2

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Session A: Orthopaedic Surgery and Gait 3

Session B: Early Development: ADHD and Sleep 7

Session C: Orthopaedics and Spasticity 11

Session D: Epidemiology, Drug Therapy, and Bone Density 15

Session E: Measurements and Assessments 19

Session F: Quality of Life, Function, and Adult Outcome 23

Scientific Posters 27

Audio-Visual Program 52

Index 53

As First Vice-President of the American Academy of Cerebral Palsy and Developmental Medicine (AACPDM), I have the privilege of writing the editorial for the September issue of Developmental Medicine & Child Neurology (DMCN) which coincides with the annual meeting of the Academy. My interest in spinal muscular atrophy (SMA) and children with disabilities was spurred by one patient, whom I met early in my career at Newington Children's Hospital, Connecticut, USA. Although DMCN has not published many articles on the subject of SMA in the past 10 years (8 of 1389 articles), I would like to take this opportunity to discuss my experience of this condition. This autosomal recessive disease, of which our knowledge has changed greatly over the past 30 years, is the second most common recessive disease in infancy and childhood.

Virtual Reality (VR) Therapy is one of the emerging and most effective applications of VR technology, where patients are exposed to stimuli in fully controllable environments.

This study examined the association between infant developmental milestones and educational level at 31 years of age in the Northern Finland 1966 Birth Cohort (n=12 058). Developmental data (age at standing, walking, speaking, and measures of bowel and bladder control) were gathered from children's welfare centres. Information on type of schooling at 14 years of age was reported by children and parents. School achievement at 16 years of age and educational level at 31 years were obtained from national registers. Those who reached infant developmental milestones sooner in their first year of life had significantly better (p<0.05) mean scores in teacher ratings at 16 years, and at 31 years they were more likely to have achieved a better educational level than slower developers. The adjusted odds ratios for individuals who developed more slowly to remain at a basic educational level (7 to 16y) ranged significantly from 1.1 to 1.3. The possibility of advancing from secondary to tertiary level was 1.4 times greater in faster developers than in slow developers. In conclusion, those who develop faster during their first year of life tend to attain higher levels of education in adolescence and adulthood.

The aim of this study was to investigate the difference in rates of cerebral palsy (CP) between singletons and twins by considering factors that may be predictive of CP. Data were taken from the Scottish Register of Children with a Motor Deficit of Central Origin and the Scottish Morbidity Record series. All children born in Scotland between 1984 and 1990 inclusive comprised the cohort. There were 646 children with CP (370 males, 276 females) of whom 57 were from twin pregnancies. Prevalence of CP was higher in twins than in singletons. Also, for singleton and twin births, the prevalence of CP was higher for infants who had low birthweight for gestational age (GA), were preterm, and who were male. Prevalence of CP by GA followed a different pattern for twins than for singletons, being lower for twins in the middle range of GAs than for singletons. After allowing for GA and birthweight, twins appeared to be at increased risk for CP compared with singletons. The type of CP in singletons and twins also differed with 64.9% of twins having spastic bilateral CP compared with 48.5% for singletons. The aetiology of CP in twins and singletons may differ.

The aim of the study was to determine whether pediatric patients with cerebral palsy (CP; 24.3% hemiplegic, 30.4% diplegic, 38.5% quadriplegic) can self-report their health-related quality of life (HRQOL) with the Pediatric Quality of Life Inventory Version 4.0 (PedsQL 4.0) Generic Core Scales. The PedsQL was administered to 148 families (84 males, 64 females; mean 10y, SD 3y 9mo, range 5 to 18y) recruited from a children's hospital clinic and state medical therapy clinics. Sixty-nine children (47%) were able to self-report. Children with CP self-reported a significantly lower HRQOL (mean 65.9) than healthy children (mean 83.8). Children with CP self-reported a similar HRQOL to pediatric cancer patients receiving treatment (mean 68.9). Children diagnosed with quadriplegia (mean 49.4) self-reported more impaired HRQOL than children with diplegia (mean 69.1) and hemiplegia (mean 72.4). Parent–child concordance was lowest for emotional functioning. Providers and parents should obtain the children's HRQOL perceptions whenever possible.

Eye research in infants who experience significant perinatal complications has been restricted to evaluation of structural ocular disease and spatial vision, and results show that these aspects of vision are at increased risk for abnormal or delayed development. To expand upon previous work, a battery of 17 vision tests was employed to assess, comprehensively, long-term outcome of functional vision. Seventy-six children (38 males, 38 females), between the corrected ages of 2 years 11 months and 10 years 2 months (mean 6y 6mo) with various significant complications (e.g. very preterm birth, bronchopulmonary dysplasia, intraventricular hemorrhage) were compared to normally developing, age-matched control children (n=61; mean age 7y 1mo) on measures of visual acuity, contrast sensitivity, stereoacuity, peripheral vision, color vision, astigmatism, and binocular alignment. Results showed that at-risk children had more test results that fell within the suspect or abnormal range. At-risk children also had a slightly higher incidence of ocular disorders (e.g. strabismus) and refractive error. These data imply that children who experienced significant perinatal risk factors are at risk for long-term deficits of functional vision. However, most of these deficits appear to be relatively mild.

Twenty children (11 females; age range 8y 2mo to 14y 7mo, mean 10y 6mo, SD 1y 8mo) with electroclinically typical rolandic epilepsy (RE), were investigated to demonstrate possible occurrence of abnormal oromotor functions and oral sensibility, linguistic problems, and impaired auditory discrimination. Twelve children were treated with an antiepileptic drug (carbamazepine, valproate, or sulthiame). They were compared with an age- and sex-matched control group of 24 children (14 females; age range 8y 2mo to 14y 5mo, mean 11y, SD 1y 8mo). The test battery included tests for facial expressions, lip and tongue movements, repeated syllables, and articulation using nonsense and tongue-twisting words. Sensibility on tongue and lips was tested with two-point discrimination. As language tests, Rapid Confrontation Naming, orthographic, and phonologic decoding tests were used. A dichotic listening test was performed in 13 children with RE and in 14 controls. Compared to control children, those with RE had significantly greater problems concerning tongue movements (p<0.05) and articulation (nonsense words and tongue-twisting words; p<0.01), and worse performance on dichotic listening (p<0.05). Oro-lingual sensibility and results of language tests did not differ between those with RE and control children. The results indicate that children with RE have distinct but mild problems with oromotor performance and auditory discrimination.

The purpose of this study was to identify factors associated with health-related quality of life (HRQOL) in individuals with myelomeningocele. Eighteen female and 16 male patients with myelomeningocele (mean age 13y, SD 6y; range 4 to 27y) were prospectively enrolled. Twenty-four of the patients had lesions at or above the L4 level, and 11 were wheelchair dependent. Twenty-five patients had shunted hydrocephalus; mean IQ of the cohort on testing was 85 (SD 18; range 36 to 111). Functional independence was measured in three areas (self-care, mobility, and social cognition) using the Functional Independence Measure for Children (WeeFIM). The Support Function Scale (social supports available to the family), the Amount of Assistance Questionnaire (assistance required for activities of daily living), and the Health Utilities Index II (HUI-II; perceived health status measure) were completed by participants or their parents. Each patient's HRQOL was quantified using a ‘feeling thermometer’, which is a plastic ‘thermometer’ scaled from 0 (least desirable health state) to 100 (perfect health). Results of multiple regression analysis using HRQOL as the dependent variable revealed that the Amount of Assistance Questionnaire, WeeFIM self-care, and HUI-II were significantly correlated with quality of life ratings (p<0.025). Maximizing functional independence should be a priority in improving HRQOL in individuals with myelomeningocele.

The aim of this pilot study was to evaluate functional training for children with cerebral palsy (CP). The outcomes examined were goal-achievement, gross motor function, performance of everyday activities, caregiver assistance, parents' perception of family-centredness, and preschool assistants' feeling of competence in the care of the children. The outcome measures used were goal attainment scaling, Gross Motor Function Measure, Pediatric Evaluation of Disability Inventory (functional skills and caregiver assistance scales), Measure of Processes of Care, and a questionnaire. The intervention was carried out in the context of daily life settings, i.e. in an ecological framework. Fourteen children (three females, 11 males; age range 1y 6mo to 6y) with diplegia and tetraplegia, classified at Gross Motor Function Classification System levels II–V, participated in the five-month intervention. Frequency of training towards a specific goal varied between twice a day and 23 times a day, depending on the type of goal. Seventy-seven per cent of goals were fully attained. Gross motor function and performance of daily activities, including social function, improved significantly. A decrease in caregiver assistance was noted in relation to the children's mobility. Parents' perception of family-centredness improved in all domains. Furthermore, the children's preschool assistants felt more competent in their care of the children.

The purpose of this study was to compare the cumulative efficacy (three treatment sessions) of botulinum toxin A (BTX-A) alone, casting alone, and the combination of BTX-A and casting in the management of dynamic equinus in ambulatory children with spastic cerebral palsy (CP). Thirty-nine children with spastic CP (mean age 5y 10mo, range 3 to 9y) were enrolled in the study. A multicenter, randomized, double blind, placebo-controlled prospective study was used. Children were randomly assigned to one of three treatment groups: BTX-A only (B), placebo injection plus casting (C), or BTX-A plus casting (B+C). The dosage for the BTX-A injections was 4U/kg per extremity. Assessments were performed at baseline, 3, 6, 7.5, and 12 months with a total of three treatments administered after the evaluations at baseline, 3, and 6 months. Primary outcome measures were ankle kinematics, velocity, and stride length. Secondary outcome measures were ankle spasticity, strength, range of motion, and ankle kinetics. Group B made no significant change in any variable at any time. Groups C and B+C demonstrated significant improvements in ankle kinematics, spasticity, passive range of motion, and dorsiflexor strength. Results of this 1-year study indicate that BTX-A alone provided no improvement in the parameters measured in this study, while casting and BTX-A/casting were effective in the short- and long-term management of dynamic equinus in children with spastic CP.

Virtual reality (VR) therapy is a new, neurorehabilitation intervention aimed at enhancing motor performance in children with hemiparetic cerebral palsy (CP). This case report investigated the effects of VR therapy on cortical reorganization and associated motor function in an 8-year-old male with hemiparetic CP. Cortical activation and associated motor development were measured before and after VR therapy using functional magnetic resonance imaging (fMRI) and standardized motor tests. Before VR therapy, the bilateral primary sensorimotor cortices (SMCs) and ipsilateral supplementary motor area (SMA) were predominantly activated during affected elbow movement. After VR therapy, the altered activations disappeared and the contralateral SMC was activated. This neuroplastic change was associated with enhanced functional motor skills including reaching, self-feeding, and dressing. These functions were not possible before the intervention. To our knowledge, this is the first fMRI study in the literature that provides evidence for neuroplasticity after VR therapy in a child with hemiparetic CP.

Lesch–Nyhan syndrome (LNS) is an X-linked recessive disorder resulting from a deficiency of the metabolic enzyme hypozanthine–guanine phosphoribosyltransferase (HPRT). This syndrome presents with abnormal metabolic and neurological manifestations including hyperuricemia, mental retardation, spastic cerebral palsy (CP), dystonia, and self-mutilation. The mechanism behind the severe self-mutilating behavior exhibited by patients with LNS is unknown and remains one of the greatest obstacles in providing care to these patients. This report describes a 10-year-old male child with confirmed LNS who was treated for self-mutilation of his hands, tongue, and lips with repeated botulinum toxin A (BTX-A) injections into the bilateral masseters. Our findings suggest that treatment with BTX-A affects both the central and peripheral nervous systems, resulting in reduced self-abusive behavior in this patient.

This review presents a discussion of the sexual development of children and adolescents with disabilities, described in the framework of body structure and function, individual activities, and societal perspectives presented in the World Health Organization's International Classification of Functioning, Disability and Health. Issues of sexual development, gynecological care and contraception, sexual functioning, societal barriers, sexual victimization, and sexuality education are presented. Overall, adolescents with disabilities seem to be participating in sexual relationships without adequate knowledge and skills to keep them healthy, safe, and satisfied. Although their sexual development may be hindered both by functional limitations and by intentional or unintentional societal barriers, the formal and informal opportunities for teenagers with disabilities to develop into sexually expressive and fulfilled persons do exist. Health care providers are urged to increase their awareness of this unmet need and to implement strategies that promote the physical, emotional, social, and psychosexual independence of children, teenagers, and young adults with disabilities.

Despite access to a wide source of information via the internet, there is little that can beat a meeting of clinicians with common interests. To this end, this year's BPNA meeting in the extremely congenial atmosphere of the Institute of Child Health (ICH) did not disappoint.