Any good paediatrician will point out to the parents of a child with cerebral palsy (CP) that their child's brain damage is fixed and cannot be altered. This is not being over pessimistic but simply honest. The paediatrician then suggests a variety of treatments/therapies aimed at maximizing the child's potential. However, it is understandable if parents still go on to seek treatments which they hope can alter their child's condition. Such treatments include conductive education, cranial osteopathy, and hyperbaric oxygen (HBO) – to name a few. This article seeks to review available information on the use of HBO in the treatment of children with CP.

The ketogenic diet has been in use since the early 1920s as a treatment for epilepsy. It is a high fat, low protein, and low carbohydrate diet which was developed to mimic the effects of fasting, which has been found to decrease seizure activity. The diet produces ketosis, as does fasting, but the role of ketosis in seizure control is not known. These days, the diet is generally used to treat seizures which do not respond to drug therapy.

A 21-year-old male presented with occipital lobes that were extensively damaged by bilateral infarcts present at birth. The absence of the striate cortex was confirmed with anatomic and functional MRI and high-resolution EEG. His cortical visual impairment was severe, but he retained a remarkable ability to see fast-moving stimuli. Horizontal optokinetic nystagmus could be elicited from either eye. Resolution acuity was close to normal providing the patient was allowed to move his head and eyes. The direction of motion in random-dot patterns could be discriminated with perfect accuracy at speeds above 2 deg/s, and the patient reported that he could ‘see’ the motion at fast but not at slow speeds. This conscious residual vision for motion is known as Riddoch's phenomenon, but it has never been reported in the complete absence of the striate cortex. Functional neuroimaging revealed activation that was outside the motion-responsive regions of the extrastriate cortex. This case demonstrates remarkable plasticity in the human visual system and may have implications for understanding the functional organization of the motion pathways.

Diagnostic labels describing specific diagnoses can be valuable in analyzing a child's problems and may be critical in securing medical and educational services, ‘but sometimes we forget that there is actually but one child with one dysfunctional brain’ (Rapin I, unpublished). Co-occurring developmental difficulties require an integrated approach in both terminology and care. The term ‘neurodevelopmental disorder’, as well as encompassing major impairments such as the cerebral palsies, embraces overlapping problems with speech and language, motor-perceptive function, behaviour, and learning.

Virtual Reality (VR) Therapy is one of the emerging and most effective applications of VR technology, where patients are exposed to stimuli in fully controllable environments.

Salla disease, a free sialic acid storage disorder, is one of the 36 currently known disorders in Finland that form the Finnish disease heritage. Salla disease leads to learning disability with a wide clinical variation. Two main categories of the disease have been classified: a conventional subtype and a severe subtype with more severe defects. We present detailed neurocognitive profiles of 41 Finnish patients with Salla disease (19 females, 22 males; age range 11mo to 63y, median 19y). The neurocognitive development of patients with Salla disease was assessed by psychological and neuropsychological testing. All patients were also examined by a paediatric neurologist and a speech therapist. The characteristic cognitive profile consisted of a lower non-verbal performance (mean developmental age 13mo) compared with linguistic skills (mean developmental age 17mo). In particular, spatial and visual-constructive impairments were typical of these patients. Tactile and visual discrimination of forms was poor. Tasks demanding hand–eye coordination, maintenance of visual attention, and those requiring short-term visual memory and executive skills were performed better. Receptive language skills were notably better compared with expressive speech. The patients' interactive and non-verbal communication skills were quite strong. Another typical pattern with Salla disease was severe motor disability. After the second decade of life, the decline in these skills was more pronounced than patients' cognitive deterioration. Our results indicate that even though there is a considerable variation in the clinical findings of patients with Salla disease, the characteristic neurocognitive profile of the disease can be outlined.

The neonatal predictors of microcephaly, defined as a head circumference <5th centile in children born preterm, has not been systematically assessed. Children were drawn from the Developmental Epidemiology Network (DEN) cohort of very low-birthweight children (VLBW: 500–1500g) born from 1991 to 1993 at three sites in the USA. Neurological assessments were carried out among 198 singleton children (mean age 6 years 8 months, SD 0.5 years). Ninety-six children (48.5%) were male. Microcephaly was observed in 30 children (15%) and, using multivariate analysis, it was found to be associated with gestational age <26 weeks and bronchopulmonary dysplasia (BPD). Sonography-defined white-matter damage (WMD, i.e. echolucency or echodensities) was not associated with increased odds of microcephaly, while occurrence of intraventricular hemorrhage (IVH) was in univariate but not multivariate analysis. In analyses that excluded children with IVH/WMD, odds of microcephaly increased in dose-related fashion according to number of days on ventilator: >5 days, OR=4.5; 95%CI=1.4 to 15; >10 days, OR=5.7; 95%CI=1.7 to 19; >15 days OR=8.3; 95% CI=2.3 to 29.2. Among children without BPD, microcephaly was not associated with differences in IQ, while IQ scores among children with BPD or any ventilation were disproportionately lower among those with microcephaly. In multivariate analyses predicting IQ at age 7 years, microcephaly was found to modify the association between neonatal lung disease and IQ.

All mainstream primary schools in Cardiff were invited in July 1998 to participate in a prevalence survey of autistic spectrum disorder. Teachers of each class filled in a questionnaire based on ICD-10 criteria for autistic disorders. The Autism Spectrum Screening Questionnaire (ASSQ) was completed on children identified with problems identified by the questionnaire. A total of 11692 children born between 1 September 1986 and 31 August 1990 were screened: 234 (2%) children were identified as requiring an ASSQ; 151 of 234 (65%) ASSQs were returned. Of the 151, 60 children (52 male, 8 female; 40%) scored 22 or more. Their notes and the involved professionals were consulted. Thirty-five children, unknown to specialist services or with complex features, required additional assessment. Seventeen children (all male) were found to be on the autistic spectrum. When the overall rubric was disentangled we found a diverse population of affected children including a handful who did not fit easily into ICD-10 classification. Correcting for incomplete ascertainment we found a minimum prevalence of 20.2 out of every 10000 (SE=4.5) for autistic spectrum disorder in this population.

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.

The Battery for Rapid Evaluation of Cognitive Functions (Batterie Rapide d'Evaluation des Fonctions Cognitives: BREV) was designed to provide health professionals with a quick clinical tool for screening acquired and developmental cognitive deficits in children aged 4 to 8 years. The BREV explores oral language in both its expressive and receptive forms, non-verbal functions, attention, verbal and visuo-spatial memory, and main learning acquisition. Results of the first phase of validation are presented in this report consisting of internal validity measurements gained by testing 500 normally developing school children (257 females, 243 males; mean age 6 years 7 months, SD 1 year 6 months. The validation provides appropriate values for each of the 17 subtests assessing cognitive functions (oral language, non-verbal abilities, attention and memory, educational achievement) in 10 age groups, from 4 to 8 years of age. All subtests with the same content for any age revealed values which increased significantly with age. Interreliability was tested in a retest for 70 children and scores obtained on retesting correlated significantly with initial values. The BREV is a reliable test with carefully established normative values, appropriate for preschool and school-age children.

To investigate whether the decrease in mean birthweight of the smallest infants surviving with a very low birthweight (VLBW) in the past two decades is associated with an increase in intellectual deficit rates, we compared the results obtained in two different follow-up studies: one included a cohort of children of VLBW (<1500g; n=102, 52% male) born in the early 1980s; the other included a cohort of children of extremely low birthweight (ELBW; <1000g; n=183, 46% male) born in the mid-1990s. Intellectual development was evaluated in both cohorts. McCarthy Scales of Children's Abilities were used in the cohort from the early 1980s; Wechsler's Preschool and Primary Scale of Intelligence – Revised was used in the cohort from the mid-1990s. The children were assessed at age 4–5 years, and a control group was included for each of the two studies. Mean scores in the measures of intelligence found at the assessment were recalculated to standard deviation scores from the two control groups. We found no difference in the standard deviation scores between the two cohorts despite the mean birthweight being 353g (p<0.001) lower in the cohort from the mid-1990s, and the overall survival rate of infants of VLBW having increased. Our results suggest that the improved survival of the smallest babies in the 1980s and 1990s has been achieved without an increase in the intellectual deficit in the surviving children.

Sudden unexpected death in epilepsy (SUDEP) has been defined as occurring in an individual suffering from epilepsy (characterized by recurrent seizures), who dies unexpectedly while in a reasonable state of health, and with death happening suddenly (in minutes), and during normal activities and benign circumstances. Also there should be no obvious medical cause, and death should not be the result of a seizure or status epilepticus, such as inhalation of vomit.

This report describes the case history of two male siblings with sensorineural hearing loss and an enlarged vestibular aqueduct (EVA). Sibling 1 presented with a history of intermittent self-limiting ataxia and hearing loss at the age of 25 months and sibling 2 presented with a similar history at the age of 18 months. MRI showed an enlarged endolymphatic duct and sac bilaterally in both children. Perchlorate discharge tests were positive in both infants leading to a diagnosis of Pendred syndrome. A number of conditions associated with EVA are discussed with a view to devising management strategies.

Three planes are discerned during ultrasonographic screening of the insula in parasagittal view: opercular, insular, and fissural. Six newborn infants with normal brain anatomy, including two each of 28, 34, and 40 weeks' gestation, were selected for a description of the evolution of these parasagittal planes. Opercularization of the insula begins to be detected on a sonogram at about the 24th gestational week and progresses cranially. On coronal section the insular space forms a shallow groove at 24 weeks, becoming a slit at 28 weeks that grows longer and develops branches after 32 weeks. At 28 weeks the ascending anterior branch of the circular groove and the lateral fissure at the bottom of the insula are clearly seen in parasagittal section. Subsequent change consists of undulation and bifurcation of the lateral fissure, together with elongation of the anterior margin. Secondary gyri become visible in the insular dome between 28 and 34 weeks, forming short anterior and long posterior insular gyri. Five term newborn infants with perisylvian polymicrogyria were observed with both neonatal ultrasound and magnetic resonance imaging: two of unknown cause, and one each due to cytomegalovirus, bifunctional peroxisomal protein deficiency, and monozygous twinning. With polymicrogyria, on parasagittal sonographic examination at fissural level the anterior margin of the circular groove is a short rudiment. In insular view, only rudimentary sulci ascend from the lateral fissure; short and long gyri are not seen. In opercular view, no secondary branches are seen from the lateral fissure, an image akin to that seen at 28 weeks. A complete insular triangle is not recognized in any of these sections. A deep abnormal sulcus may prolong the lateral fissure when schizencephaly is associated with polymicrogyria. The insula in glutaric aciduria type II has no secondary gyri at term and is reduced to a simple and small triangle. Not all instances of polymicrogyria lead to macroscopically recognizable alteration of insular gyri.

Intramuscular botulinum toxin A injections are beneficial for the treatment of functional shortening of the iliopsoas muscle, but it is difficult to achieve precise needle positioning and injection. As a solution to this we present an ultrasound-guided injection technique for the iliopsoas muscle using an anterior approach from the groin. The procedure was performed 26 times in 13 patients (seven males, six females; mean age 11 years, SD 9 years 8 months; age range 4 to 31 years), 10 times bilaterally. Indications were functional iliopsoas shortening due to cerebral palsy (17 hips), hereditary spastic paraplegia (four hips), and Perthes disease (five hips). In all cases the iliopsoas muscle was identified easily by ultrasound; the placement of the injection needle and injection into the site of interest were observed during real time. No complications were encountered.