‘Publish and be damned’, the Duke of Wellington's reported retort to attempted blackmail in a kiss-and-tell saga two hundred years ago, has acquired a Don't in front of it in the modern academic world. However, how research should be published has become a controversial topic. In the US the National Institutes of Health (NIH) have decided that any work they support should be made freely available after six months. The subtext is eventual immediate availability, which can already happen if authors publish in some electronic fora. If so, subscription journals could disappear. It is suggested that, instead of the existing system, authors should pay to publish their work, at a generally quoted cost of US$1500 per article. Future NIH grants would include publication costs. Other large grant giving bodies in the UK and elsewhere may follow the NIH example.

Children with cerebral palsy (CP) frequently grow poorly, compared with their typically-developing peers. Moreover, as children with CP age, their growth diverges further and further from that of typical children. The reasons for poor growth are multi-factorial and include nutritional, hormonal, physical, and neurological causes. Nevertheless, it appears that much of the poor growth is related to acute and chronic malnutrition at various points across the lifespan. The malnutrition is largely due to inefficient and dysfunctional feeding. While remediable to some extent, this dysfunctional feeding often cannot be overcome. Consequently, in children with (usually severe) CP, malnutrition is often treated by bypassing the dysfunctional oral feeding through the use of a gastrostomy.

We report a longitudinal, prospective, multicentre cohort study designed to measure the outcomes of gastrostomy tube feeding in children with cerebral palsy (CP). Fifty-seven children with CP (28 females, 29 males; median age 4y 4mo, range 5mo to 17y 3mo) were assessed before gastrostomy placement, and at 6 and 12 months afterwards. Three-quarters of the children enrolled (43 of 57) had spastic quadriplegia; other diagnoses included mixed CP (6 of 57), hemiplegia (3 of 57), undiagnosed severe neurological impairment (3 of 57), ataxia (1 of 57), and extrapyramidal disorder (1 of 57). Only 7 of 57 (12%) could sit independently, and only 3 of 57 (5%) could walk unaided. Outcome measures included growth/anthropometry, nutritional intake, general health, and complications of gastrostomy feeding. At baseline, half of the children were more than 3SD below the average weight for their age and sex when compared with the standards for typically-developing children. Weight increased substantially over the study period; the median weight z score increased from –3 before gastrostomy placement to –2.2 at 6 months and –1.6 at 12 months. Almost all parents reported a significant improvement in their child's health after this intervention and a significant reduction in time spent feeding. Statistically significant and clinically important increases in weight gain and subcutaneous fat deposition were noted. Serious complications were rare, with no evidence of an increase in respiratory complications.

To study the agreement between three tests for aspiration, barium videofluoroscopy, salivagram, and milk scan we studied 63 children with severe non-ambulant spastic quadriplegic cerebral palsy (CP) aged 14 months to 16 years (32 males, 31 females). The salivagram was most frequently positive (56%, 95% confidence interval 43 to 68%); the next most frequently positive was barium videofluoroscopy when aspiration was defined as the presence of either laryngeal penetration of material or frank aspiration (39%, 95% confidence interval 26 to 53%). The milk scan was rarely positive (6%, 95% confidence interval 2 to 16%). Agreement between the tests of aspiration was poor. The maximum agreement (kappa=0.20) was between aspiration as diagnosed with the salivagram and by barium videofluoroscopy. Positive tests for aspiration are frequent in children with severe CP. Frequency varies widely depending on the investigation used. There is poor agreement between tests used for the diagnosis of aspiration. This information is of importance in assessing the significance of test results.

There are few well-controlled, published evaluations of sleep interventions for children with developmental disabilities. This paper evaluates a parent training programme using behavioural principles to reduce sleep problems in children with autism or fragile X syndrome (FXS). Training included bedtime routines, reinforcement, effective instructions, partner support, and extinction (removing reinforcement to reduce a behaviour). Programme efficacy was demonstrated by using a multiple baseline across-participant design. Social validity was also assessed. Five children with autism and one with Asperger syndrome (four males, two females; mean age 5y 6mo; age range 3y 5mo to 7y 4mo) and their parents; and seven children with FXS (six males, one female; mean age 4y 9mo; age range 1y 11mo to 9y 1mo) and their parents participated. Ten families completed intervention within the multiple baseline design. Most parents' goals were achieved and visual analysis of the sleep data indicated improvement. Settling problems, night waking, and co-sleeping were effectively reduced. The programme had high social validity and outcomes were clinically significant and maintained at follow-up.

In this pilot study we investigated the feasibility of The Infant Behavioral Assessment and Intervention Program (IBAIP) in a group of preterm infants. At the age of 6 months, the neurobehavioural organization and self-regulatory competence of an intervention group was compared with a control group who had received the standard follow-up care. The intervention group consisted of 13 males and seven females (mean gestational age [GA] 29.2 weeks, SD1.3wks; mean birthweight 1232g, SD320g). The control group consisted of 11 males and nine females (mean GA 29wks, SD1.6wks; mean birthweight 1198g, SD397g). Inclusion criteria were: a GA of 32 weeks and family residence in the district of Amsterdam. Exclusion criteria were: severe congenital abnormalities, intraventricular haemorrhage grade III or IV, periventricular leukomalacia grade III or IV, and infants whose mothers had a history of illicit drug use. The intervention infants received 6 to 8 IBAIP interventions at home, from discharge until 6 months of age. The Neonatal Behavioral Assessment Scale was administered at term; the Infant Behavioral Assessment (IBA) at term, 3, and 6 months of age; and the Bayley Scales of Infant Development–II at 3 and 6 months (corrected age). At 6 months, intervention infants showed less stress and more approach behaviours on the IBA compared with control infants. These promising results warrant further evaluation in a randomized controlled trial.

The purpose of this article is to demonstrate: (1) the accuracy and (2) the reduction in amount of time and effort in assessing physical functioning (self-care and mobility domains) of children and adolescents using computer-adaptive testing (CAT). A CAT algorithm selects questions directly tailored to the child's ability level, based on previous responses. Using a CAT algorithm, a simulation study was used to determine the number of items necessary to approximate the score of a full-length assessment. We built simulated CAT (5-, 10-, 15-, and 20-item versions) for self-care and mobility domains and tested their accuracy in a normative sample (n=373; 190 males, 183 females; mean age 6y 11mo [SD 4y 2m], range 4mo to 14y 11mo) and a sample of children and adolescents with Pompe disease (n=26; 21 males, 5 females; mean age 6y 1mo [SD 3y 10mo], range 5mo to 14y 10mo). Results indicated that comparable score estimates (based on computer simulations) to the full-length tests can be achieved in a 20-item CAT version for all age ranges and for normative and clinical samples. No more than 13 to 16% of the items in the full-length tests were needed for any one administration. These results support further consideration of using CAT programs for accurate and efficient clinical assessments of physical functioning.

To determine the natural history of developmental dyscalculia (DC) and factors impacting on its prognosis, we performed a prospective six-year longitudinal study. One hundred and forty children of normal intelligence diagnosed with DC in the fifth grade of elementary school were re-examined for dyscalculia three and six years later, in eighth (n=123) and eleventh (n=104; 41 males, 63 females) grades respectively. Mean age of the children in fifth grade was 11 years 1 month (SD 4 months), in eighth grade 14 years 2 months (SD 1 month), and in eleventh grade 17 years 2 months (SD 5 months). The assessment included standardized arithmetic, reading and writing tests, behavioural rating scales, information on socioeconomic status, educational interventions, and familial learning problems. Participants in eleventh grade were recategorized as having DC if their score on the arithmetic test was not more than the fifth centile for grade. At the six-year follow-up, 99/104 (95%) children diagnosed with dyscalculia in fifth grade were still performing poorly in arithmetic, scoring within the lowest quartile for their grade, and 42/104 (40%) were recategorized with DC. Chronicity of DC was associated with severity of the dyscalculia in fifth grade (p<0.05), lower IQ (p<0.01), inattention (p<0.01), and writing problems (p<0.01). Thus, DC is an enduring specific learning difficulty, persisting into late adolescence in almost half of affected individuals.

This study evaluated the cognitive profiles of children with idiopathic generalized epilepsy (IGE), uniformly treated with valproic acid with well-controlled seizures. Twenty-four were neuropsychologically evaluated. They comprised: 14 females, 10 males: 12 with generalized tonic–clonic seizures (GTCS), mean age 14y 4mo, SD 1y 7mo, range 12y to 16y 4 mo; 12 with absence seizures (AS) mean age 14y 5mo, SD 1y 10mo, range 11y to 16y 4mo, with intellectual abilities within the normal range and age-appropriate scholastic skills, and 20 healthy controls (12 females, 8 males; mean age 14y 5mo, SD 1y 10mo, range 10y 7mo to 16y 7mo). As a group, children with IGE performed significantly poorer in all tests (non-verbal and verbal attention, verbal learning and memory, word fluency, and controlled sequential fine motor responses) excluding non-verbal memory. Analysis according to type of seizure revealed that both patient groups (AS and GTCS) had an attention deficit, whereas only children with AS showed deficits in verbal learning and memory, word fluency, and controlled fine motor responses. These results suggest a long-term risk of learning impairment for children with IGE, even if they have normal intelligence and their seizures are well controlled.

In 1997, Miura et al. isolated a novel homeobox gene in zebrafish and mice with striking homology to the Drosophila gene aristaless. They called this gene Arx (aristaless-related homeobox gene) and showed that it was highly conserved between zebrafish and mice and expressed in the developing central nervous system. The human orthologue of this gene, ARX, is located on the short arm of the X chromosome (Xp22.13). It consists of five exons and encodes a 562 amino acid protein that may be a nuclear transcriptional regulator. It is expressed in the fetal and adult brain, with much higher expression in the fetal brain. In the fetal brain the ARX gene is primarily expressed in neuronal precursors in the germinal matrix and ventricular zone at all stages of development but also in the subventricular zone, cortical plate, caudate nucleus, putamen, substantia nigra, cingulate, and hippocampus. It is also expressed in fetal testes and adult heart, skeletal muscle, and liver.

‘Auntie, speak to me! I'm frightened because it's dark’‘What good would that do? You can't see me’‘That doesn't matter. If anyone speaks it gets light’

Problems are painful, not only for what they are, manifest for all to see in the world beyond, but from how they are perceived within ourselves. A change in our perception of them can alter them in ways which may seem magical.