The September bulletin of the British Paediatric Surveillance Unit (BPSU) stated that the Sir Peter Tizard research bursary has been awarded to a proposed epidemiological survey of paediatric idiopathic intracranial hypertension (IIH), also known as benign intracranial hypertension or pseudotumour celebri. IIH can lead to visual failure but is treatable. Its diagnosis, natural history, and medical and surgical management in childhood remain controversial.

Badawi and colleagues (p 85) report on a positive association between newborn encephalopathy (NE) in term neonates and the risk of autism spectrum disorder (ASD). The study they conducted has several strengths worth mentioning that include the population-based nature of their sample, the systematic ascertainment of cases of NE in a well-defined population, the selection of a proper control series, and a comprehensive independent assessment of case status at follow-up.

Between June 1993 and December 1996, 276 term newborn infants with encephalopathy and 564 randomly selected term controls were enrolled in a population-based study of moderate and severe term newborn encephalopathy (NE) in Western Australia. During comprehensive neurobehavioural and cognitive follow-up of all patients and controls at 3 years and again at 5 years of age we found an unexpected but strong association between NE and autism spectrum disorders (ASDs). A diagnosis of ASD by age 5 years was reached using criteria according of the Diagnostic Statistical Manual, 4th edition. Linking records to the Western Australian Disability Services Commission Register ensured that no child in the study with ASD was missed. By age 5 years, 37 (13.4%) infants with NE and one (0.2%) control had died. Among the 239 survivors of NE, 12 (5%) were diagnosed with an ASD. Of these, 10 (4.2%) met the full criteria for autism, one had pervasive developmental disorder–not otherwise specified, and one had Asperger syndrome. Among the 563 surviving controls, five (0.8%) were diagnosed with an ASD: three with autism, one with autism/possible Asperger syndrome, and one with Asperger syndrome. Compared with the controls, the children who had experienced NE were 5.9 times (95% confidence interval 2.0–16.9) more likely to have been diagnosed with an ASD.

The aim of this study was to investigate long-term survival and examine causes of death in adult patients with cerebral palsy (CP). A 1940–1950 birth cohort based on paediatric case referral allows for long-term survival follow-up. Survival is analyzed by birth characteristics and severity of disability from age 20 years (and age 2y for a subset of the data). Survival outcome compared with that expected in the general population based on English life tables. The main cohort consisted of 341 individuals, with 193 males and 148 females. Conditional on surviving to age 20 years, almost 85% of the cohort survived to age 50 years (a comparable estimate for the general population is 96%). Very few deaths were attributed to CP for those people dying over 20 years of age. Females survived better than males. However, females faced a greater increase in risk relative to the general population than did males. We conclude that survival outlook is good though lower than in the general population. The relative risk of death compared with the UK population decreases with age, although it shows some indication of rising again after age 50 years. Many more deaths were caused by diseases of the respiratory system among those dying in their 20s and 30s than would be expected in the general population. Many fewer deaths than expected in this age group are caused by injuries and accidents. For those people who die in their 40s and 50s, an increase in deaths due to diseases of the circulatory system and neoplasms is observed. More deaths than expected in this age group are due to diseases of the nervous system.

We report on seven children (five males, two females) who presented with marked, often asymmetrical, toe-walking from onset of independent walking, associated with abnormal foot postures and increased tone at the ankles with characteristics of dystonia. Most of the children had presented with unusual pre-walking locomotion and a mild delay in independent walking. They did not fit into the usual categories of ‘habitual’ toe-walking or congenital short tendo calcaneus but nor did they have the clinical signs of spastic diplegia or of a peripheral neuromuscular disease. Normalization occurred progressively in the second to fourth years of life. The children were re-examined several years later (1 to 11y) and were normal. We believe that their persistent toe-walking corresponded to a variant of ‘transient focal dystonia of infancy’. Knowledge of its existence may justify a period of observation without special investigations, surgery, or casting.

The purpose of this study was to investigate the effect of salivary flow reduction on daily life and provision of care in children with cerebral palsy (CP). Parents of children with CP were asked to fill in a questionnaire on the impact of drooling on the daily life of their children and their families and the data were then analyzed. Forty-five children with severe drooling (28 males, 17 females; mean age 9y 5mo [SD 3y 7mo]; range 3 to 16y) were monitored before and after receiving medication (scopolamine and botulinum toxin) to reduce salivary flow. Type of CP included hypotonia (n=1), spastic paresis (n=27), and mixed motor disorders with spastic and dyskinetic paresis (n=17). Eight children were independently ambulant and 37 children were wheelchair users. Thirty-four children had learning disability with a developmental age of below 6 years. Six participants dropped out of the study; data on 39 children were analyzed. Results showed that anticholinergic agents effectively reduced salivary flow. Drooling diminished substantially and this was accompanied by a significant reduction in care needs, making daily care less demanding. The amount of reported damage to communication devices and computers decreased. In addition to the evaluation of primary variables, such as the salivary flow rate, investigation of impact of drooling on daily life provides useful information about the outcome of treatment for reduction in drooling.

The many concerns of parents of children with hydrocephalus have not previously been assessed using a reliable, quantitative measurement. The development and preliminary use of a questionnaire to measure the concerns of these parents is reported. A general health status measure for hydrocephalus was developed concurrently. Items were generated primarily from parent focus groups resulting in a comprehensive list of 22 unique parental concern items. The most important of these items, as selected by another sample of parents of 60 children (27 females, 33 males; mean age 8y 4mo, [SD 4y 6mo]) were retained for the final questionnaire. This 9-item parental concerns questionnaire was tested for reliability and construct validity (by assessing correlations with various independent measures of health status, including Health Utilities Index–2 and Impact-on-Family Scale) using a cohort consisting of the parents of 80 children (39 females, 41 males; mean age 10y, [SD 3y 6mo]). An exploratory analysis was performed to determine which patient variables were associated with greater parental concern. The parental concerns questionnaire demonstrated excellent test–retest reliability (0.86, 95% CI 0.77–0.92) and internal consistency (Cronbach's alpha=0.89). As expected, interrater reliability was somewhat lower (0.67, 95% CI 0.49–0.79). Construct validity was demonstrated by consistently moderate Pearson's correlations with several independent measures of the child's health. The presence of epilepsy and younger patient age appeared to be significantly associated with greater parental concern. This new questionnaire has demonstrated very good psychometric properties. It can be used to broaden the scope of future clinical research in pediatric hydrocephalus to include the impact on parents.

Knowledge about the level of functional independence that can be expected in adulthood might support decisions on the treatment of newborn infants with spina bifida. This study determined functional independence among young adults with spina bifida and its relationships with pathological characteristics known from birth (hydrocephalus and level of lesion). Data were collected from medical records and by physical examination. Functional independence was assessed on six domains (self-care, sphincter control, transfers, locomotion, communication, and social cognition) using the Functional Independence Measure (FIM). Participants were 165 patients with spina bifida (69 males, 96 females; age range 16 to 25y, mean 20y 9mo [SD 2.9]; 117 with hydrocephalus). Patients without hydrocephalus were independent for all FIM domains except sphincter control, as were patients with hydrocephalus with a lesion level below L2. Most patients with hydrocephalus and a lesion at L2 or above were dependent as regards sphincter control (98%), locomotion (79%), and self-care (54%), and quite a few needed support in transfers (38%), social cognition (29%), and communication (15%).

The purpose of the study was to provide normative data for the Teddy Bear Cancellation Test (TBCT) and to evaluate prospectively the frequency of unilateral spatial neglect (USN) in children with acquired brain injury (ABI). In the control group (n=419; 218 males, 201 females; mean age 5y 1mo [SD 1y 4mo]; range 3 to 8y) omissions were rare and decreased with age. A left displacement of the first three teddy bears cancelled was observed with increasing age. This preferential left-to-right cancelling strategy was interpreted as learned under the influence of reading habits. The same test was used prospectively in 41 children with ABI (24 males, 17 females; mean age 5y 5mo [SD 2y]; range 3 to 8y) admitted to a paediatric rehabilitation department specializing in acquired brain lesions. In patients and controls, children under 6 years of age omitted more items than older children. The localization of omissions was skewed significantly to the left in children with right-sided lesions compared with children with left-sided lesions. USN was observed in seven patients with ABI. Left USN was found in three of the 10 patients with right-sided ABI. Right USN was present in two of the patients with 15 left-sided ABI and two of the 16 patients with non-lateralized ABI. Left USN is frequent in children after right-sided brain injury. The relatively high incidence of right spatial neglect in children is discussed in relation to the development of hemispheric specialization.

This study aimed at investigating the possible dissociation between visual–object and visual–spatial working memory (WM) in individuals with Williams syndrome (WS) and Down syndrome (DS). Four study groups were included: WS group (10 males, 5 females) with a mean chronological age (CA) of 19 years 8 months (SD 6y 1mo) and a mean mental age (MA)of 6 years 11 months (SD 1y 5mo); WS comparison group (7 males, 8 females) comprised of typically developing children with a mean CA of 6 years 10 months (SD 10mo) and a mean MA of 6 years 11 months (SD 8mo)matched as a group with the participants with WS on the basis of mental age; DS group (11 males, 7 females) with a mean CA of 15 years 10 months (SD 5y 8mo) and a mean MA of 5 years 2 months (SD 8mo); and DS comparison group (10 males, 8 females) with a mean CA of 5 years and 1 month (SD 7mo)and a mean MA of 5 years 2 months (SD 8mo) selected to match the DS group on the basis of mental age. They were all administered tests that explored visual perception (Visual Perception Test – Subtest 4 and Line Orientation tests), visual imagery (imaging the colour of objects and the tail length of well-known animals), spatial imagery (mental rotation of visually presented or verbally evoked objects), and WM for visual–object and visual–spatial information. Individuals with WS exhibited specific difficulties in the visual–spatial, but not the visual–object, WM task. Instead, people with DS showed reduced performance in both tests. However, whereas the observed deficit in individuals with DS persisted when perceptual abilities were taken into account, the deficit in individuals with DS was compensated when their scores were adjusted for performance on perceptual tasks. These results support the hypothesis of a dissociation within the sketch-pad slave system in the WM model and reinforce the view of intellectual disability as a non-unitary condition.

The aim of this study was to illustrate the difficulties in establishing a diagnosis of mitochondrial respiratory chain (MRC) disorders based on clinical grounds in combination with intermediate activities of the MRC enzyme complexes. We reviewed retrospectively all medical and laboratory records of patients initially considered likely to have MRC disorders on clinical grounds, and subsequently diagnosed with other disorders (n=20; 11 males, 9 females). Data were retrieved from hospital records, referral letters, and results of enzymatic analysis at a reference laboratory. Clinical symptoms included developmental delay, epilepsy, hypotonia, movement disorder, spastic quadriplegia, tetany, microcephaly, visual problems, carpopedal spasms, dysmorphism, hearing loss, muscle weakness and rhabdomyolysis, and fulminant hepatitis. Blood and cerebrospinal fluid lactate levels were elevated in 13/20 and 9/20 respectively. One or more MRC complex activities (expressed as ratios relative to citrate synthase and/or complex II activity) were less than 50% of control mean activity in 11/20 patients (including patients with deficiencies of pyruvate dehydrogenase complex, pantothenate kinase, holocarboxylase synthetase, long-chain hydroxy acyl-CoA dehydrogenase, molybdenum co-factor, and neonatal haemochromatosis). One patient had a pattern suggestive of mitochondrial proliferation. We conclude that intermediate results of MRC enzymes should be interpreted with caution and clinicians should be actively looking for other underlying diagnoses.

Transdermal scopolamine may be used to reduce drooling in children with disabilities. Side-effects include dilated pupils and a reduction in the near point of accommodation (the closest point at which clear vision is possible). Two male children with epilepsy, one with spinal dysraphism (aged 7y 6mo) and one with cerebral palsy (aged 5y 8mo), who have undergone treatment for drooling with transdermal scopolamine are described. Near visual acuity was reduced, and both children showed dilated pupils with reduced or no response to light. These responses became normal on cessation of the scopolamine patch. As the effect of this drug may be cumulative, and many patients are unable to communicate difficulties, clinicians need to be aware of these possible side-effects.

We report a case of a 7-month-old male with primary intracranial haemorrhage 2 months after infection with varicella zoster virus (VZV). His initial clinical course was complicated by seizures and right hemiparesis; when last seen at 22 months the only positive finding was of left hand preference. Although the literature has recently established the association of arterial ischaemic stroke and VZV infection, primary intracranial haemorrhage has been reported only in one case. The child reported here had anterior interhemispheric haemorrhage due to a focal arteritis of the left anterior cerebral artery. The vascular abnormality was transient and had radiological features compatible with either a focal arteritis or vasospasm as a direct result of blood surrounding the vessels. We postulate that direct invasion of VZV caused extensive inflammation of the vessel wall and aggressive tissue penetration resulting in necrotizing angiitis and intracranial haemorrhage. We suggest that VZV infection should be considered a potential risk factor for intracranial haemorrhage in children.

Children with developmental disabilities often show a variety of associated impairments that lead to a lifelong need for additional care. Careful assessment of these impairments is required not only for diagnostic purposes but also to inform the parents about the expected additional care needs in the future. We present a systematic review of the literature to identify instruments that classify the type and amount of this care for the individual child. A literature search was performed in the Medline database (January 1966 – June 2005) on instruments that classify the type and amount of expected additional care needs in the future. Seven standardized measurement instruments describing current additional care needs were identified, but none of these instruments was developed to provide information about the expected need for additional care in the future. For parents of young children with non-progressive developmental disorders it is essential to be informed on the expectations of required additional care in the future. However, comprehensive instruments providing such information are currently lacking and, thus, need to be developed.

Young people with cerebral palsy (CP) are often assumed to have low self-concept, in other words, they do not feel good about themselves. We systematically searched the literature to determine whether this assumption was supported by empirical research. Relevant trials were identified by searching electronic databases, and this was supplemented by citation tracking. Of 1355 papers initially identified, six met the criteria for review. Results showed that adolescent females with CP have a lower self-concept than females without disability in the domains of physical appearance (d=–1.16; 95% confidence interval [CI] –2.06 to –0.26); social acceptance (d=–1.24; 95% CI –2.15 to –0.33); athletic competence (d=–0.93; 95% CI –1.79 to –0.07); and scholastic competence (d=–0.86; 95% CI, –1.71 to –0.01). Adolescent females with CP may be an at-risk group owing to their vulnerable self-concept. Clinicians may need to monitor and implement appropriate intervention strategies with this group. There was insufficient evidence to conclude that children with CP, in general, have a lower global self-concept compared with those without disability.