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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

This report describes trends, predictors, and causes of mortality in persons with cerebral palsy (CP) using individuals identified by the Western Australian Cerebral Palsy Register and born between 1958 and 1994. Two thousand and fourteen people were identified (1154 males, 860 females), of whom 225 had died by 1 June 1997. Using date-of-death data, crude and standardized mortality rates were estimated and predictors of mortality sought using survival analysis stratified by decade of birth, description of impairments, and demographic and perinatal variables. For those born after 1967, the cause of death profile was examined over time. Mortality exceeded 1% per annum in the first 5 years and declined to age 15 years after which it remained steady at about 0.35% for the next 20 years. The strongest single predictor was intellectual disability, but all forms of disability contributed to decreased life expectancy. Half of those with IQ/DQ score <20 survived to adulthood, increasing to 76% with IQ/DQ score 20-34, and exceeding 92% for higher scores. Severe motor impairment primarily increased the risk of early mortality. Despite there being 72 persons aged from 25 to 41 years with severe motor impairment in our data set, none had died after the age of 25 years. Infants born after more than 32 weeks' gestation were at significantly higher risk of mortality than very preterm infants, accounted for by their higher rates of intellectual disability. No improvements in survival of persons with CP were seen over the study period despite advances in medical care, improved community awareness, and the increasing proportion of very preterm births among people with CP. This may be the result of improved neonatal care enabling the survival of infants with increasingly severe disabilities.

This study examined the evolution of individuals with cerebral palsy (CP) from childhood to adulthood. Seventy-two adults with a diagnosis of CP born between 1934 and 1980 were studied. Individuals were recruited and data comprehensively collected using case notes and through direct assessments of the majority of participants from three rehabilitation units in Bologna, Padua, and Rovigo in Italy. The main findings can be summarized as follows: contact with health and rehabilitation services was radically reduced once individuals reached adulthood; more individuals who were integrated into mainstream schools achieved and maintained literacy than those who had attended special schools; in a high number of participants, motor performance deteriorated once into adulthood. Independent walking or other forms of supported locomotion were lost in many on reaching adulthood. Of those who continued to walk, walking deteriorated in terms of distance. It was concluded that even though CP has been considered as predominantly a childhood pathological condition, the evolution of the effects of CP do not stop at 16 or 18 years of age. For this reason, the traditional child- (or infant-) oriented approach concentrating mainly or exclusively on the achievement of independent walking, may not be an ideal approach to children with CP. Instead a more independence-oriented therapeutic approach would be appropriate.

To assess time costs of caring for children with severe disabilities in the community compared to caring for children without disabilities, a diary- and questionnaire-based study was carried out. Sixteen complete data sets were obtained from families with children who have disabilities (mean age 8.7 years) and 31 complete data sets from families with normally developing children (mean age 4.9 years). Diagnoses in the study group included cerebral palsy, autism, Sanfillipo syndrome, lissencephaly, and osteogenesis imperfecta. Items of personal care per waking hour were significantly greater in children with disabilities than non-disabled children (p<0.001). In the study group, there was no correlation (r=–0.12) between age and frequency of care whereas a significant correlation was observed between degree of disability as measured by the Functional Independence Measure for children (WeeFIM) and frequency of care items (r=0.89). Twelve of the 16 mothers in the study group were not in paid employment. Twelve had little or no extended family support. Benefits awarded did not correlate with the degree of disability as measured by the WeeFIM (r=–0.11). Care needs of children with severe disabilities are significantly greater than those of non-disabled children and do not decrease with advancing age. Mothers of children with disabilities are unable to work outside the home because of these care needs. This brings the family income, even when benefits are included, to a level that is less than peer families with non-disabled children. A Functional Disability Score may help to achieve more appropriate allocation of state resources.

The present study addressed the question whether the deviant postural adjustments in children with spastic diplegia can be attributed to their crouched sitting position or primarily to their neural deficit. Postural adjustments during sitting in an erect and in a crouched position on a movable platform were assessed in 10 children, aged 3 to 7 years 6 months, with mild-to-severe forms of spastic diplegia and 10 age- and sex-matched control children. Multiple surface EMGs of neck, trunk, and leg muscles and kinematics of head, body sway, and pelvis were recorded during forward and backward translations. The children with normal motor development showed a distinct adaptation of postural adjustments to sitting position. The children with cerebral palsy (CP) had a deficient adaptational capacity which was more pronounced in the erect than in the crouched position. Thus, the crouched sitting position did not induce postural deficiency but seemed to offer a solution to the sensory–motor problem of the instability experienced. Children with severe diplegia exhibited a lack of direction specificity in the leg muscles during backward body sway, which points to a basic deficit in postural control. In addition, these children showed marked dysfunctions in the precise tuning of the postural adjustments to task-specific conditions. In the children with mild-to-moderate forms of CP the basic level of control was intact.

Outcome was studied of all children and adolescents with pseudoseizures without epilepsy, who were referred and treated as in-patients and/or day patients in the tertiary psychiatric ward at Birmingham Children's Hospital, UK between 1988 and 1994. Information was derived from case-note data. Freedom from seizures for six months was defined as ‘cure’, as no recurrences after this period were noted. Kaplan–Meier survival analysis was used. Seventeen patients were identified: 15 females and two males; mean age at presentation to the tertiary psychiatric service was 12 years 9 months (SD 26 months; range 8 years 3 months to 15 years 9 months). Fourteen participants recovered and resumed regular school attendance. Three were lost to follow-up due to being referred on to other agencies before recovery because they became too old for the service. Recovery followed an exponential distribution, with a mean symptom survival time following treatment of 1.5 years. These results are consistent with a treatment effect. Younger age at presentation, female sex, having more types of seizures, and not receiving both in-patient and outpatient treatment were associated with better outcome. The deteriorating prognosis with age at treatment suggests prompt identification may be important in ensuring a good outcome.

Pediatric hemifacial spasm can be a sign of an intracranial tumor. We examined two females, ages 2 and 6 years, who presented with hemifacial spasm as an early sign of accommodative esotropia. Initial ophthalmologic examination disclosed high hyperopia without detectable esotropia. Both children developed an intermittent esotropia over the following month. In the first child, the hemifacial spasm resolved concurrent with the onset of amblyopia. In the second child, spectacle correction produced immediate cessation of the spasms. In both children, squinting to avoid diplopia produced an overflow facial movement that manifested as hemifacial spasm. Accommodative esotropia should be considered in the differential diagnosis of pediatric hemifacial spasm.

Haemophagocytic lymphohistiocytosis (HLH) is characterized anatomically by an infiltration of multiple tissues with lymphocytes and haemophagocytic histiocytes. First symptoms are usually hepatosplenomegaly, pancytopenia, and intractable fever. Up to 73% of those with HLH develop CNS involvement during the disease course. The peculiarity of the two patients presented here, a 20-month-old Italian female and a 4-year-old Moroccan female, is that the initial presenting neurological symptoms mimicked an encephalitis, anticipating the typical systemic symptoms by 1 and 4 months. They developed progressive encephalopathy accompanied by status epilepticus, one child developed a secondary hydrocephalus. In both children it was not possible to detect an underlying infection or malignant disease and there were no other cases in the family that suggested a familial form of HLH. Diagnosis and initiation of treatment was delayed because of the initial encephalopathic clinical picture and the late onset of the typical systemic features. As early diagnosis allows better therapeutical approaches, haemophagocytic lymphohistiocytosis should be considered in children with persistent or progressive findings of encephalopathy, especially in the absence of identification of a plausible pathogen.

In this report, an unusual intracranial haemorrhage in a term male infant born to a mother with diabetes is explained on the basis of occlusion of both basal veins of Rosenthal. This diagnosis relies on anatomical location and iconographic aspect of the clots. Evidence that this vessel is occluded cannot be ascertained from ultrasound or MR angiographic techniques in the neonatal period. The basal vein has not been implicated in previous reports of neonatal brain haemorrhage.

On 27th to 28th February 2000, a multidisciplinary workshop was convened in the USA to explore the current state of knowledge, best clinical practice, and research needs for the management of equinus gait associated with cerebral palsy (CP). The workshop began with a brief review of CP epidemiology, and an inquiry into the current understanding of the natural history of equinus. Attention then shifted to indications for and goals of intervention; a summary of interventional approaches; the criteria by which successful intervention is evaluated; and, finally, research opportunities. What follows is an overview of the workshop.

Why is it important to detect and define learning disability in a child, and how can one do so? The answers to the first question are, largely, not in dispute. Not only does the recognition and definition of learning disability contribute to our understanding of the basis of problems in a child's educational, developmental, and social progress, it also has significant long-term consequences, especially where the disability is more severe. The diagnosis or medical aetiology of the child's learning disability is a significant element of the story and carries major phenotypic implications, in terms of dysmorphology, organogenesis, development, and behaviour. It is one thing to diagnose, say, Down syndrome, and quite another to reach an accurate appraisal of the individual child's intellect, developmental attainments, and capacities. In turn, the definition of learning disability is not always straightforward and is sometimes quite controversial. This is partly because, traditionally, at least three different approaches are used to define learning disability in children, and each has its inherent attractions and pitfalls. These approaches are: definition by IQ alone; definition by IQ combined with social functioning and age at onset; and definition by service contact, the so-called ‘administrative definition’.