Five papers in this issue report on outcome studies, all of which follow up children who presented with illnesses in the neonatal period. The studies by Ruggieri et al. and Watanabe et al. are principally concerned with the outcome of a specific condition. The former examines spinal-cord injury and the latter, convulsions. Both are chiefly concerned with how the seizures progress and how the specific signs relate to neurological findings. Both mention general functional outcomes – Watanabe discusses psychomotor delay, while Ruggieri concentrates on learning and behaviour disorders – but neither explains how they were measured.

This study examines the executive function (EF) skills of extremely-low-birthweight (ELBW) children at school compared with their peers. Thirty children with ELBW and 50 control children (both with a mean age of 62±4 months) were administered tests of EF including the Tower of Hanoi task, Finger Sequencing task, and Tapping Test. Children with ELBW, including those who scored more than 1 SD below the mean on the Peabody Picture Vocabulary Test – Revised, scored significantly lower than their peers on all executive tasks. There was limited correlation between EF and previous general quotient index scores obtained at routine assessment using the McCarthy Scales of General Ability at 4 years of age for the children with ELBW. Results suggest that children with ELBW are at risk for deficits in ‘executive’ behaviours including planning, sequencing, and inhibition which may have implications for later learning.

Damage to the occipital cortex in children can result in many complex disorders of cognitive visual function. A series of clinical questions, developed from the specific problems of a cohort of children with cortical visual impairment, was asked of the parents of 200 children with no history of cerebral pathology, aged 5 to 12 years. One hundred and ninety-two parents gave reliable consistent responses. The results show a progressive improvement in performance with age, culminating in few 11- and 12-year olds having frequent problems, apart from 8% having frequent difficulty with orientation in new surroundings and 2% having problems with simultaneous perception tasks. The parents of 52 children (aged 5 to 17 years) with shunted hydrocephalus were then asked the same set of questions. Evidence of cognitive visual problems was identified in 27 of these children of whom 16 manifested multiple difficulties. The disabilities identified by our study comprised problems with: shape recognition, simultaneous perception, perception of movement, colour perception, orientation, object recognition, and face recognition. The range, nature, and combinations of these disorders are presented in this paper.

Data are presented on 17 children who received extracorporeal membrane oxygenation (ECMO) in the neonatal period for persistent pulmonary hypertension (PPHN). These children are being followed as part of a larger program of follow-up research on children who have been treated for PPHN with several treatment methods. On intelligence testing at ages 5 to 8 years, these 17 children had unusual patterns of results. A higher-than-predicted percentage of the ECMO survivors had discrepancies between their Verbal and Performance IQ and a much-higher-than-predicted percentage had areas of unusual strength or weakness on their IQ subtest scores. Also, there was a significant correlation between the amount of time a child received ECMO and the child's Performance IQ: the longer the child received ECMO, the higher the Performance IQ. While findings of unusual weaknesses or deficits on intelligence testing at school age in children who have been severely ill in the neonatal period are not unusual, findings of high scores and areas of strength are not easily explained, particularly when these findings seem to relate to an invasive treatment like ECMO. Similar findings have been reported in two other small studies, which suggest that the impact of ECMO on the developing infant brain may not be purely detrimental.

We investigated the features of children with spinal cord insults (SCI) occurring in the pre-, peri-, and neonatal periods by sending 340 questionnaires to all paediatric neurologists, paediatric urologists, and neonatologists in the UK and Ireland. We requested information about timing, nature, and level of SCI in their patients; family and maternal history; pregnancy, delivery, and neonatal period; clinical presentation, imaging, laboratory studies, and outcome. Two-hundred and sixty-one questionnaires were returned with data on 58 patients with SCI. Seven out of the 58 children with SCI had pure dysraphic cord syndromes and were excluded. Fifty-one patients (33 males, 17 females, one unknown), born between 1972 and 1996, remained. Clinical presentations included severe respiratory failure ( N=20; five of whom died neonatally) and hypotonia or weakness recognized either during the neonatal period (N=12) or after 28 days (N=10). Data on clinical presentation were not given in nine cases. Lesions were cervical (N=22) and thoraco-lumbar (N=29). SCI was ascribed to ischaemia (N=12), trauma (N=4), and other associated underlying conditions (N=11), whilst the aetiology was unknown in 24 cases. Mean gestational age (36.2 weeks) and birthweight (2.6 kg) were lower than previously reported with the lowest figures associated with thoraco-lumbar and ischaemic lesions. More males were affected by lesions than females and the incidence of preterm delivery, multiple pregnancy, breech presentation, forceps delivery, and caesarean delivery were higher than average. Forceps delivery was associated with cervical lesions. Outcome data were given in 47 children, nine of whom died either neonatally or within the first 20 months of life. Motor disability ranged from a complete recovery in one out of 40 to paraparesis in 26 out of 40, and tetraparesis in 13 out of 40 patients: 17 out of 39 were ambulant. Sphincter dysfunction was present in 22 out of 38 patients and scoliosis in 16 out of 37. Learning difficulties were present in 10 out of 39, behavioural problems in five out of 39 and seizures in four out of 39 patients. SCI in the pre-, peri-, and neonatal periods are rare but probably under-diagnosed and are heterogeneous in aetiology, presentation, and outcome. Boys appear to be more susceptible than girls.

Most neonatal seizures are occasional seizures and not true epilepsy. This study investigates seizure types of true neonatal epilepsies and their evolution with development. Seventy-five children with epilepsies of onset within 1 month of life, who were examined between 1970 and 1995, and whose seizure types could be confirmed with ictal EEG recordings, were studied. The patients were followed up for a minimum of 3 years and the evolution of epileptic syndromes was investigated. Sixty-three (84%) of 75 patients had partial seizures, while nine had generalized seizures, and only three had both generalized and partial seizures. Twenty-three of 24 neonates with benign familial or non-familial neonatal convulsions presented with partial seizures; these syndromes should not necessarily be categorized into generalized epilepsy as they are in the present International Classification. Age-dependent changes were a common feature of symptomatic neonatal epilepsies. Eighteen (41%) of 44 patients with symptomatic epilepsies of neonatal onset developed West syndrome in infancy. Fifteen (83%) of these 18 patients presented with symptomatic localization-related epilepsy in the neonatal period. In seven of these 15 patients, West syndrome was followed by localization-related epilepsy. Symptomatic localization-related epilepsy with transient West syndrome in infancy is another type of age-dependent epileptic syndrome.

This study compares bone mass in a national sample of girls with Rett syndrome (RS) with a sample of control children. The Australian RS Database was the source of cases for this population-based study. Hand radiographs were available from 101 of 137 subjects (74% of the known Australian population of girls with RS aged [les ]20 years). Control radiographs matched for age, sex, and laterality were obtained from hospital radiology departments. A measure of cortical thickness was made from the difference between the outer diameter and the medullary space in the second metacarpal bone. A mean z-score value for cortical thickness and percentage cortical area for each individual was calculated. The mean cortical thickness (z score) for girls with RS was −1.94 compared with −0.38 for control children (P<0.001). In girls with RS, the mean cortical thickness decreased with age (P<0.001). In girls who were taking epilepsy medication it was −2.21 compared with −1.23 in those not taking epilepsy medication (P<0.001). There was no evidence of a beneficial effect of increased calcium intake on cortical thickness. A similar pattern was obtained when percentage cortical area was estimated. In multivariate analysis, increasing age and use of anticonvulsant medication were associated with decreased cortical thickness and only use of anticonvulsant medication with decreased percentage cortical area. Fractures had occurred in one-third of cases and it was estimated that just over 40% of girls would sustain a fracture by the age of 15 years. Girls with RS may be at increased risk of fractures and their bone quality compromised as determined by cortical thickness and percentage cortical area measurements from the second metacarpal.

Lower respiratory tract infections in children with severe neurodisability are usually caused by aspiration of stomach contents from gastroesophageal reflux (GOR) or direct aspiration (DA) of food due to oral and pharyngeal motor problems. To determine the contributions and interactions of GOR and DA, oesophageal 24-hour pH monitoring and feeding videofluoroscopy were performed in 34 children (age range 7 months to 16 years, mean 7 years) who had severe physical and learning disabilities and who were slow feeders. Subjects were divided into three groups according to the frequency of their respiratory tract infections. Subjects in group 1 had no respiratory tract infections (N=10); five had GOR and none had DA. Subjects in group 2 had minor respiratory tract infections but had not received more than one course of antibiotics for this in the previous year (N=8); two had GOR alone, four had DA alone, and two had neither. All subjects in group 3 had recurrent respiratory tract infections (N=16); one had GOR alone, seven had DA alone, and eight had both GOR and DA. This study suggests that oral and pharyngeal motor problems are the major cause of respiratory tract infection in children with severe neurodisability. These problems lead to DA and, if GOR is present, to the aspiration of stomach contents. Those children with both DA and GOR are more likely to have severe respiratory tract infections which may lead to gastrostomy feeding (together with fundoplication). GOR without sufficient oral and pharyngeal motor problems to cause DA is less likely to cause respiratory tract infection in children with severe neurodisability.

This study examined phonological working memory and speech discrimination among children with attention-deficit–hyperactivity disorder (ADHD) with and without motor problems. Forty-one children were assigned to three groups; children with ADHD (N=9), children with ADHD plus developmental coordination disorder (ADHD+, N=13), and age-matched control children (N=19). The subjects' ability to classify stimulus pairs was examined in two experiments. The first experiment required subjects to discriminate pairs of monosyllabic stimuli with contrasting consonants to test speech discrimination without using a working-memory load. In the second protocol, subjects were exposed to two- to five-syllabic non-word pairs with contrasting vowels in order to test speech discrimination with a working-memory load. The subjects classified the pairs as being either the same or different in both experiments. No significant differences were found between the subject groups in the discrimination task with monosyllables. When exposed to the two- to five-syllabic stimuli, the ADHD+ group scored significantly lower than both other groups. This was attributed to a higher sensitivity to working-memory load. Some possible explanations of this effect are discussed.

The aim of this study was to determine the incidence of respiratory depression following the use of diazepam in children presenting with seizures. All children presenting with seizures to a children's A & E department over a period of 9 months were studied prospectively. Respiratory depression was defined as a fall in respiratory rate or oxygen saturation, or apnoea resulting in ventilation or resuscitation with bag-and-mask oxygen. There were 130 patient episodes involving 97 children who received treatment for their seizures before admission and/or in the A & E department. Administration of diazepam resulted in 122 patient episodes. The route of administration was rectal in 91 episodes, intravenous in 12 episodes, and both rectal and intravenous in 19 episodes. Eleven children had respiratory depression in relation to diazepam administration. Eight of these children required ventilation. The overall incidence of respiratory depression following the use of diazepam was 9%. The incidence of respiratory depression following diazepam given intravenously or rectally is high. The use of diazepam as first-line therapy for children with acute seizures needs to be reviewed.

Two subjects with ictal hemiparesis are described. Both children presented with evolving paresis associated with seizure activity. Structural neuroimaging remained consistently normal, although EEG demonstrated slow-wave activity, and SPECT scanning in one child showed perfusion asymmetry. Both children had resolution of the hemiparesis when seizure activity was adequately controlled. The historically proposed pathophysiology of ictal hemiparesis is that of inhibition of the somatosensory and motor areas of the cortex. The presence of an evolving hemiparesis and seizure activity associated with normal neuroimaging should prompt consideration of ictal hemiparesis. Confirmation of this rare diagnosis can only be made when seizure control leads to resolution of the paresis.

Sleep problems are common in childhood. They are reported in about a quarter of young children, usually in the form of settling difficulties or night waking. The types and frequency of sleep disturbance tend to vary with age and, although some remit spontaneously, a substantial proportion of them persist. Particularly high rates of sleep disturbance occur in children who have learning disabilities, are physically ill, or psychologically disturbed. The immediate effects of sleep problems are important but so, also, are the longer-term effects which may influence the child's physical, psychological, and social development. Persistent sleep disturbance is associated with behavioural disturbance in children and may affect performance in school. Raised levels of maternal stress and impairment of parenting skills have also been reported as a consequence.

Ataxia is the cardinal sign of cerebellar dysfunction and when it occurs, it indicates the possibility of cerebellar lesions. However, this may not always be the correct explanation. Appenzeller and Hanson reviewed studies, published between 1916 and 1953, of patients with cerebellar signs attributed to parietal lesions. Extensive lesions within the parietal lobes and sensory changes were common in these patients. They also reported on an adult who presented with pain and weakness of the left arm, lasting for 2 hours. When examined 2 weeks later, the limb was severely ataxic, but with no detectable weakness or sensory deficit. In addition, the patient had a dressing apraxia. At autopsy a small infarct in the arm area of the right postcentral gyrus was observed. The cerebellum and brainstem were normal.