Sixty-nine very-low-birthweight infants out of a population of 923 had cerebral palsy (CP) at an 18-month follow-up. Thirty-nine of these had cranial ultrasound abnormalities in the neonatal period and 30 had normal cranial ultrasounds. The distribution of subtypes of CP differed markedly between the two groups, with hemiplegia predominating in those with abnormal cranial ultrasounds and diplegia in those with normal cranial ultrasounds. Regardless of ultrasound appearance, the relative risk of CP increased approximately fourfold with a neonatal history of sepsis.

A neuropsychological investigation of the association between specific language impairment (SLI) and hyperactivity was conducted on four groups of 6-year-old children. The groups, each consisting of five boys and five girls, were: children with SLI alone, hyperactivity alone, both SLI and hyperactivity, and neither disorder (controls). Cognitive functioning was examined using selected non-verbal tests from the Cambridge Neuropsychological Test Automated Battery. SLI was not associated with significantly reduced performance on any of the neuropsychological measures. However, hyperactivity was associated with deficits on a test of attentional set shifting previously demonstrated to be sensitive to frontostriatal dysfunction. Further, hyperactive children showed significantly reduced spatial spans on a test of spatial working memory thought to measure parietal lobe functioning. There were no substantial differences between the groups on tests of frontal lobe function except for reduced performance on a task of attention in the hyperactive group. There were no significant interactions between hyperactivity and SLI on any of the neuropsychological measures. The pattern of neuropsychological deficits shown by children with attention-deficit–hyperactivity disorder (ADHD) suggests relatively specific problems in inhibitory control of attentional selection. This cognitive deficit implicates brain areas including basal ganglia and ventro-lateral prefrontal cortical areas in ADHD, thus providing evidence for disruption of frontostriatal functional loops. SLI and hyperactivity can occur as comorbid syndromes or symptom complexes and this should be noted when interpreting results of tests measuring cognitive performance. The findings suggest that these two groups of disorders have different cognitive correlates.

Visual–perceptual abilities were assessed in 5-year-old children with the following neonatal neurological conditions: born preterm with normal ultrasound scan (NL, n=17); born preterm with ultrasound diagnosis of intraventricular haemorrhage (IVH, n=17); born preterm with ultrasound diagnosis of periventricular leukomalacia (PVL, n=12); born term with hypoxic–ischaemic encephalopathy (HIE, n=11). Visual–perceptual ability was evaluated with the L94: eight visual–perceptual tasks designed to evaluate different aspects of visual perception at the preschool level in children with multiple disabilities. Impairment was established in comparison to the performance age obtained on non-verbal intelligence subtests, instead of chronological age. Frequency of L94 impairment was highest in children with PVL, while children with IVH did not differ from the NL control group. Impairment rates were increased also in children with transient periventricular echodensities, and in children with HIE. Impairments were only moderately related to the delay of visual acuity maturation in infancy.

A newly established tertiary epilepsy clinic at Guy's Hospital, London was evaluated using a structured questionnaire. Fifty parents of children with epilepsy completed this questionnaire, with some contribution from the children. While a high degree of satisfaction was obtained by the parents and where appropriate the children, specific questions about additional resources implied a large number of unmet needs. This raises methodological issues about the value of open-ended questions in audit questionnaires. In addition, concerns were expressed about many areas, some of which had direct medical implications that were not always raised with the doctors. It is concluded that the service has benefited from both clinical and neuropsychological support. The audit has prompted a number of improvements, including establishing a discussion group for adolescents.

Set-shifting is an important aspect of attention and regulation of behaviour. The ability of young children to shift set is debated. Set-shifting is usually examined using sorting tasks that are designed for adults and hence are inadequate for children. In this study, an experimental Sorting Task for Children (STC) is administered to 54 healthy children (aged 4 to 10 years) and compared with a widely used adult sorting task, the Modified Card Sorting Test. This experiment shows that preschool children are capable of set-shifting. An extensive error analysis of the STC reveals that perseverations are not predominant. Hence, set-shifting in young children may be characterised by ‘trial and error’ rather than by being stuck in a mental set, which is suggested to be typical of adults with frontal lobe damage. A pilot study of 10 children with idiopathic or cryptogenic epilepsy shows unstable set-shifting rather than inability to shift set, which is concordant with known problems in regulation of behaviour.

This study evaluates the reliability of isokinetic testing of the knee flexors and extensors in children with cerebral palsy (CP). Twelve children (seven girls, five boys), aged 9 to 15 years, participated in this study. The children's strength was measured bilaterally, using a dynamometer. Each participant was tested twice at an interval of 1 week. During each session, the participant performed five consecutive cycles of knee extension and flexion. Testing velocity was set at 90°/s, and the range of motion was 80°, starting with the knee flexed at 80° and ending in full extension. The measured variable was the peak torque. Reliabilities were determined using intraclass coefficient (ICC) with two-way ANOVA model. The ICCs for individual sessions range from 0.90 to 0.99. ICCs for the eight repetitions over the two sessions range from 0.95 to 0.98. The findings indicate that measuring isokinetic strength in the tested population is highly reliable and should be considered in rehabilitation protocols.

Atraumatic fractures are often seen in children and adolescents with cerebral palsy (CP) and epilepsy in full-time care. Increased bone fragility was postulated to be due to osteopenia resulting from a combination of factors including immobilization and antiepileptic treatment. The aim of this study was to determine the effect of vitamin D and calcium substitution on bone mineral density (BMD) in a group of children with CP in full-time care. Twenty children with the most severe form of CP (spastic quadriplegia) who had been treated with antiepileptic drugs for a relatively long period of time were included in the study. Physical examination and laboratory analyses excluded other possible causes of osteopenia. BMD was measured by dual X-ray absorptiometry. Thirteen patients were treated for 9 months with 1,25-dihydroxy-cholecalciferol vitamin D (0.25 mcg daily) and with calcium (500 mg daily). Seven control children were used for observation only. BMD greatly increased in the treated group, while children with CP in full-time care who did not receive vitamin D and calcium substitution continued to lose their bone mass. It can be concluded that the addition of vitamin D and calcium increases BMD in children with the most severe form of CP, who are receiving antiepileptic drugs.

Eighty-two women who were consuming alcohol while pregnant attended a special clinic at the University Central Hospital, Helsinki with the aim of reducing heavy drinking during pregnancy. The children born to these women were followed up regularly. During their preschool years the children were assessed to have fetal alcohol syndrome, fetal alcohol effects, alcohol-related neurodevelopmental disorder, pre- and/or postnatal growth retardation, or they were assessed to have normal cognitive and somatic growth. Of the original children, 70 of 82 could be traced at the age of 12 years. Through semistructured interview and contact with the health and social care authorities, information was gathered about schooling, family structure, whether help had been sought for behavioural difficulties and major adverse events in the family. The longer the intrauterine alcohol exposure and the more severe the diagnosis related to prenatal alcohol exposure, the more often the children required special education, were temporarily or permanently taken into care, and had behavioural problems. There is a considerable need for prolonged multidisciplinary follow-up and support of all children whose mothers have not been able to reduce drinking in early pregnancy, whether or not cognitive disturbances are evident in early childhood.

A baby girl born by elective lower segment caesarean section was found to have left-sided focal seizures at 48 hours after birth. Her mother had previously had a neonatal death at 26 weeks' gestation and another child born at 32 weeks' gestation had a congenital right hemiplegia with a left middle cerebral artery infarct on CT scan. The mother had raised anticardiolipin IgG antibodies at the time of delivery of her second child, with no thrombotic symptoms. Therefore, during this pregnancy, she had been treated with low molecular weight heparin and aspirin. The baby's mother had raised IgG and IgM anticardiolipin antibodies and the baby had IgG anticardiolipin antibodies at the upper range of normal 4 days after delivery. The seizures were controlled with phenobarbitone and phenytoin. CT and MRI scans showed evidence of cerebral ischaemia. A repeat MRI scan at 4 months of age was normal, anticonvulsants were discontinued, and her latest neurological examination at 5 months was normal.

Hypoxic–ischaemic encephalopathy (HIE) was diagnosed in an infant with acidosis. At 7 weeks of age further investigations revealed abnormal neuroimaging (CT and MRI scans) and a raised plasma and CSF lactate. A skeletal–muscle biopsy at 2 months of age confirmed the diagnosis of cytochrome oxidase deficiency. The course of the patient's disorder has taken that of a static encephalopathy (cerebral palsy). Inborn disorders of the respiratory chain should be considered in the differential diagnosis of HIE.

A 14-month-old girl presented after 3 days of fever, floppiness, and diffuse urticarial exanthem. She developed encephalitis and carditis and 1 week later, intractable seizures. Initial CT and MRI showed no changes in the brain parenchyma. On days 14 and 34 after the onset of symptoms, a human herpesvirus-6 (HHV-6) genome in cerebrospinal fluid was identified by polymerase chain reaction (PCR). Convulsions became more frequent and 11 weeks from the onset, they changed to typical infantile spasms with hypsarrhythmic electroencephalogram. She gradually lost her social contact and ability to walk and sit. Eleven months after the primary infection, a repeated MRI of the brain revealed a cystic tumour of 2cm in diameter near the vermis. The tumour was surgically removed, and shown to be a pilocytic astrocytoma on histopathological examination. HHV-6 DNA was detected by PCR in new tumour tissue. This is the first reported case of HHV-6 encephalitis associated with carditis, infantile spasms, and a subsequent brain tumour containing the HHV-6 genome.

Recent molecular genetics research has established that most cases of velocardiofacial syndrome (VCF) and of DiGeorge syndrome result from a submicroscopic deletion at chromosome 22q11.2. The medical features of this disorder include hypocalcemia, immunodeficiency, cleft palate, subtle facial dysmorphism, ‘conotruncal’ cardiac malformations (e.g. truncus arteriosus, tetralogy of Fallot, interrupted aortic arch, and certain types of ventricular septal defects), and other congenital malformations. While none of these complications is found in all patients with the 22q11.2 microdeletion, each is sufficiently common to be considered part of the disorder's medical phenotype.