bluuebunny/crossref_metadata_2025 huggingface dataset:
Fetal growth and subsequent mental health problems in children aged 4 to 13 years
Authors: Zubrick Stephen R , Kurinczuk Jennifer J , McDermott Brett M C , McKelvey Robert S , Silburn Sven R , Davies Lisa C
Published: July 2010

To test the hypothesis that children with suboptimal fetal growth have significantly poorer mental health outcomes than those with optimal growth, a population random sample survey of children aged 4 to 16 years in Western Australia in 1993 was conducted. The Child Behavior Checklist (Achenbach 1991a) and the Teacher Report Form (Achenbach 1991b) were used to define mental health morbidity. Survey data for 1775 children aged 4 to 13 years were available for linkage with original birth information. The percentage of expected birth weight (PEBW) was used as the measure of fetal growth. Children below the 2nd centile of PEBW who had achieved only 57% to 72% of their expected birthweight given their gestation at delivery were at significant risk of a mental health morbidity (OR 2.9, 95% CI 1.18, 7.12). In addition, they were more likely to be rated as academically impaired (OR 6.0, 95% CI 2.25, 16.06) and to have poor general health (OR 5.1, 95% CI 1.69, 15.52).
Severe bronchopulmonary dysplasia increases risk for later neurological and motor sequelae in preterm survivors
Authors: Majnemer Annette , Riley Patricia , Shevell Michael , Birnbaum Rena , Greenstone Harriet , Coates Allan L
Published: July 2010

Preterm children who develop severe chronic lung disease may be developmentally compromised by exposure to hypoxic episodes. This study aims to determine if children with severe bronchopulmonary dysplasia (BPD) who required home oxygen therapy were at greater risk for neurological and motor deficits at school age than preterm peers without BPD. This study evaluated 27 subjects with BPD and 27 preterm control infants matched for gestational age, birth weight, sex, and year of birth at a mean age of 9.9 years (2.0 SD) using standardized neuromotor outcome measures. Pair‐matched comparisons and regression analyses were used to determine if subjects with BPD were at increased risk for neuromotor sequelae. Neurological abnormalities, including subtle neurological signs, cerebral palsy, microcephaly, and behavioral difficulties were highly prevalent in the BPD group (71% compared with 19% in control group, P<0.005). Over half the BPD cohort had difficulties in gross and/or fine motor skills. There were significant differences in postural stability between groups. Duration of hospitalization and home oxygen treatment, and decreased lung function at school age, markers of severity of illness, correlated with motor outcomes. The findings underline the importance of preventing the cardiorespiratory complications associated with chronic lung disease to minimize disability in preterm children. For children with severe BPD, better recognition and subsequent remediation of neuromotor impairments that manifest at school age may help maximize their functional potential.
Therapeutic effects of functional electrical stimulation of the upper limb of eight children with cerebral palsy
Authors: Wright P A , Granat M H
Published: July 2010

Functional electrical stimulation (FES) of the upper limb has been used for patients with a variety of neurological conditions, although few studies have been conducted on its use on the upper limb of children with cerebral palsy (CP). The aim of this study was to investigate the effect of cyclic FES on the wrist extensor muscles of a group of eight children (five boys, three girls) with hemiplegic CP (mean age 10 years). The study design involved a baseline (3 weeks), treatment (6 weeks), and follow‐up (6 weeks). FES was applied for 30 minutes daily during the treatment period of the study. Improvements in hand function (p≤0.039) and active wrist extension (p=0.031) were observed at the end of the treatment period. These improvements were largely maintained until the end of the follow‐up period. No significant change was observed in the measurements of wrist extension moment during the treatment period (p=0.274). Hand function in this group of children improved after they were exposed to FES of wrist extensor muscles. This suggests that FES could become a useful adjunct therapy to complement existing management strategies available for this patient population.
Macrocephaly in autism and other pervasive developmental disorders
Authors: Fidler Deborah J , Bailey Julia N , Smalley Susan L
Published: July 2010

To assess the prevalence of macrocephaly (head circumference ≥1.88 standard deviations above normative data for age and sex or >97th centile) in autism and other pervasive developmental disorders, 41 children with autism, and a comparison group of 21 children with tuberous sclerosis complex (TSC) or an unspecified seizure disorder were studied. Familiality of head circumference was also assessed from measurements of 133 first‐degree relatives. Significantly higher rates of macrocephaly were found in probands with autism (12.2%) and their first‐degree relatives (15.5%) when compared against a published normative sample. The incidence of macrocephaly in the comparison group of probands with TSC and seizure disorder (9.5%) and their first‐degree relatives (8.3%) was higher than normative data as well, although the relation between macrocephaly and autism was more pronounced. Head circumference and extreme scores reflecting macrocephaly were moderately heritable in the present sample (H2= 0.47). The increased prevalence of macrocephaly in relatives of children with autism compared with control children suggests that this characteristic may be a familial risk factor in the pathogenesis of autism.
Patterns of neuropsychological deficits in children with medulloblastoma according to craniospatial irradiation doses
Authors: Kieffer‐Renaux Virginie , Bulteau Christine , Grill Jacques , Kalifa Chantal , Viguier Delphine , Jambaque Isabelle
Published: July 2010

This study aimed to analyse the relationship between supratentorial irradiation dose and the intellectual outcome in 36 children (aged between 5 and 15 years) treated for medulloblastoma. The supratentorial radiation dose was reduced to 25 Gy in 23 children and given at the standard dose, 35 Gy, in 13 other children. Neuropsychological evaluation was performed at a mean of 4.3 years (SD 4.7 years) after radiotherapy. The supratentorial radiation dose was the principal risk factor associated with impaired intellectual outcome. Verbal fluency, immediate word list recall, block design, and fine motricity of the dominant hand were significantly lower in children irradiated at the standard doses than in those irradiated at reduced doses. These findings suggest that the dose of radiotherapy applied to the brain strongly influences later verbal and non‐verbal skills in children with medulloblastoma. This should be taken into account in treatment planning and in rehabilitation programs.
Can sodium valproate improve learning in children with epileptiform bursts but without clinical seizures?
Authors: Ronen Gabriel M , Richards Joanne E , Cunningham Charles , Secord Margaret , Rosenbloom David
Published: July 2010

This study aimed to determine whether sodium valproate (VPA) improves cognitive performance and behaviour in children with learning and behavioural problems associated with electrographic epileptiform discharges but without clinical seizures. A randomized, double‐blind, single‐crossover trial was carried out with VPA or placebo on eight participants with different learning and behaviour problems. Participants also underwent neuropsychological testing under video EEG and the parent and teacher Behaviour Check List (CBCL; Achenbach 1991a, b) during each treatment phase. Clinically none of the children improved on VPA. On formal testing children were more distractable, had increased delay in response time, and showed lower memory scores while on VPA. In addition, parents reported higher internalizing scores on the CBCL while children were on VPA. Our data do not support the use of VPA in similar patients.
Association of epilepsy with different groups of microcephaly
Authors: Abdel‐Salam Ghada M H , Halász Adrienne A , Czeizel Andrew E
Published: July 2010

Sixty‐six participants (33 males, 33 females) with microcephaly (MC), age range from 2 to 19 years old, were evaluated. MC was classified pathogenetically into isolated MC (IMC) and multiple MC (MMC) and classified etiologically into primary MC (PMC) and secondary MC (SMC). Both IMC and MMC were further classified. Overall prevalence of epilepsy was 40.9%. Furthermore, there was a significantly higher prevalence of epilepsy in males. Main seizure type was generalized tonic‐clonic seizures. Generally, learning disability (LD) was diagnosed in 93.9% and profound LD was evident in 43.9% of participants. There was an inverse correlation between severity of epilepsy and IQ but a positive correlation between severity of epilepsy and degree of LD. Differences in the success rate between monotherapy and polytherapy or response to antiepileptic drugs were not observed. Results suggest that epilepsy may be associated with the lower cognitive ability of the participants with microcephaly. The pathogenetic classification proposed is of value in delineating the prevalence of epilepsy and LD in the different varieties of MC as compared with the etiological classification.
Psychomotor development and minor anomalies in children exposed to antiepileptic drugs in utero: a prospective population‐based study
Authors: Wide Katarina , Winbladh Birger , Tomson Torbjörn , Sars‐Zimmer Kerstin , Berggren Eva
Published: July 2010

The aim of this study was to assess psychomotor development, using Griffiths’test, and the incidence of minor anomalies at birth in children who had been exposed to antiepileptic drugs (AEDs) in utero. The study sample comprised 100 children of mothers who were treated with AEDs during pregnancy and 100 matched control children. Women with epilepsy were recruited from a pregnant urban population (450 000 inhabitants). The lowest possible dose of the fewest AEDs to maintain seizure control was used. Drug levels were controlled on a monthly basis. The children were assessed at 9 months of age. The study children had a significant increase in the number of minor anomalies (31 compared with 18 control children; odds ratio 2.4, CI 1.15 to 5.02, P=0.02 McNemars test), and an increased number of facial anomalies after carbamazepine exposure (11 compared with six control children). Drug exposure did not influence the Griffiths’score at 9 months of age. Even a meticulous AED treatment strategy during pregnancy increases the number of minor anomalies. However, treatment with AEDs does not necessarily influence short‐term psychomotor development.
Children with neurological disorders do not always need fundoplication concomitant with percutaneous endoscopic gastrostomy
Authors: Puntis John W L , Thwaites Rosemary , Abel Gill , Stringer Mark D
Published: July 2010

Whether antireflux surgery should be routinely performed at the time of gastrostomy in children with neurological disorders is debatable because of the risk of gastroesophageal reflux. Some argue that these children should be screened for occult gastroesophageal reflux as this will determine the need for fundoplication. This study retrospectively examines outcome in 29 children with neurological disorders who underwent percutaneous endoscopic gastrostomy (PEG) without concomitant fundoplication. Children were included if they had no clinical evidence of severe gastroesophageal reflux before PEG insertion. The median age of children at PEG insertion was 5.6 years (range 1.1 to 18.0). The children were followed for a median of 2.6 years (range 0.4 to 4.9). Insertion of PEG was technically impossible in two children; and an asymptomatic gastrocolic fistula in another child led to subsequent tube removal. Fourteen of the 26 remaining children developed symptomatic gastroesophageal reflux after PEG; five of these showed no reflux on pH monitoring prePEG. Control of symptoms was achieved by medical intervention in 12, but two required fundoplication. Our findings indicate that in the child with neurological disabilities without symptoms indicating severe gastroesophageal reflux, fundoplication is unlikely to be necessary as a consequence of PEG insertion. We conclude that routine investigation for gastroesophageal reflux in the child without severe vomiting can be avoided and the number of antireflux procedures reduced.
Visual function in school‐aged children born before 29 weeks of gestation: a population‐based study
Authors: Hård Anna‐Lena , Niklasson Aimon , Svensson Elisabeth , Hellström Ann
Published: July 2010

The aim of this study was to assess visual function, including visual perception, in a geographically‐based population of school‐aged children, with a median age of 7.2 years (range 5.1 to 9.3 years), born before 29 weeks of gestation to mothers living in Göteborg, Sweden. Fifty‐one preterm children participated in the study, six of whom had known brain lesions. Visual acuity, visual fields, stereoacuity, and visual perception were tested. The Test of Visual Perceptual Skills —Revised (TVPS‐R, Gardner 1996) was used to measure visual perception, and the results were compared with those of 50 term (control) subjects. Six percent of the preterm children were visually impaired, with a visual acuity of less than 0.3 (6/18), while 42% of all the preterm children and 34% of those without known brain lesions had a total score below the 5th centile of the reference material for the test, compared with 14% of the control subjects. In conclusion, visual‐perceptual problems seem to be common among very preterm children and should be screened for and assessed before the children start school.
Foix‐Chavany‐Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster‐Drought syndrome
Authors: Christen; H‐J , Hanefeld F , Kruse E , Imhäuser S , Ernst J P , Finkenstaedt M
Published: July 2010

Foix‐Chavany‐Marie syndrome (FCMS) is a distinct clinical picture of suprabulbar (pseudobulbar) palsy due to bilateral anterior opercular lesions. Symptoms include anarthria/severe dysarthria and loss of voluntary muscular functions of the face and tongue, and problems with mastication and swallowing with preservation of reflex and autonomic functions. FCMS may be congenital or acquired as well as persistent or intermittent. The aetiology is heterogeneous; vascular events in adulthood, nearly exclusively affecting adults who experience multiple subsequent strokes; CNS infections; bilateral dysgenesis of the perisylvian region; and epileptic disorders. Of the six cases reported here, three children had FCMS as the result of meningoencephalitis, two children had FCMS due to a congenital bilateral perisylvian syndrome, and one child had intermittent FCMS due to an atypical benign partial epilepsy with partial status epilepticus. The congenital dysgenetic type of FCMS and its functional epileptogenic variant share clinical and EEG features suggesting a common pathogenesis. Consequently, an increased vulnerability of the perisylvian region to adverse events in utero is discussed. In honour of Worster‐Drought, who described the clinical entity in children 40 years ago, the term Worster‐Drought syndrome is proposed for this unique disorder in children.
A randomized controlled trial of early dietary supply of long‐chain polyunsaturated fatty acids and mental development in term infants
Authors: Birch Eileen E , Garfield Sharon , Hoffman Dennis R , Uauy Ricardo , Birch David G
Published: July 2010

The effects of dietary docosahexaenoic acid (DHA) supply during infancy on later cognitive development of healthy term infants were evaluated in a randomized clinical trial of infant formula milk supplemented with 0.35% DHA or with 0.36% DHA and 0.72% arachidonic acid (AA), or control formula which provided no DHA or AA. Fifty‐six 18‐month‐old children (26 male, 30 female) who were enrolled in the trial within the first 5 days of life and fed the assigned diet to 17 weeks of age were tested using the Bayley Scales of Infant Development, 2nd edition (BSID‐II) (Bayley 1993) at the Retina Foundation of the Southwest, Dallas, TX. These children had also been assessed at 4 months and 12 months of age for blood fatty‐acid composition, sweep visual evoked potential (VEP) acuity, and forced‐choice preferential looking (FPL) acuity (Birch et al. 1998). Supplementation of infant formula with DHA+AA was associated with a mean increase of 7 points on the Mental Development Index (MDI) of the BSID‐II. Both the cognitive and motor subscales of the MDI showed a significant developmental age advantage for DHA‐ and DHA+AA‐supplemented groups over the control group. While a similar trend was found for the language subscale, it did not reach statistical significance. Neither the Psychomotor Development Index nor the Behavior Rating Scale of the BSID‐II showed significant differences among diet groups, consistent with a specific advantage of DHA supplementation on mental development. Significant correlations between plasma and RBC‐DHA at 4 months of age but not at 12 months of age and MDI at 18 months of age suggest that early dietary supply of DHA was a major dietary determinant of improved performance on the MDI.
Neurodevelopmental profile of a new dysmorphic syndrome associated with submicroscopic partial deletion of 1p36.3
Authors: Knight‐Jones Eve , Knight Samantha , Heussler Helen , Regan Regina , Flint Jonathan , Martin Katherine
Published: July 2010

We describe four children with dysmorphic syndrome with severe learning disability (SLD). Their chromosomes had been normal on conventional cytogenetic examination. However, screening using a multiprobe fluorescence in situ hybridisation (FISH) technique for subtelomeric abnormalities revealed a deletion of the p arm of chromosome 1. The physical features include body asymmetry, microcephaly, distinctive facies with deep‐set eyes, sharply defined eye sockets, and mid‐face hypoplasia; the neurodevelopmental profile was characterised by SLD, motor delay with hypotonia, markedly delayed visual maturation, and postural asymmetry together with epilepsy. This phenotype is consistent with that described for partial monosomy for 1p36.3.
Dysfunction of the auditory cortex persists in infants with certain cleft types
Authors: Čeponiene R , Hukki J , Cheour M , Haapanen M‐L , Koskinen M , Alho K , Näätänen R
Published: July 2010

Language and learning disabilities occur in almost half of individuals with oral clefts. The characteristics of these cognitive dysfunctions vary according to the cleft type, and the mechanisms underlying the relation between cleft type, cognitive dysfunction, and cleft‐caused middle‐ear disease are unknown. This study investigates preattentive auditory discrimination, which plays a significant role in language acquisition and usage, in infants with different cleft types. A mismatch negativity (MMN) component of brain evoked potentials, which indexes preconscious sound discrimination, and brain responses to rare sine‐wave tones were recorded in 12 healthy infants and 32 infants with oral clefts at the ages of 0 and 6 months. Infants with clefts were subdivided into two categories: those with cleft lip and palate (CLP) (n=11 at birth, n=6 at the age of 6 months) and those with cleft palate only (CPO) (n=17 at birth, n=8 at the age of 6 months). At both ages, brain responses to rare sounds tended to be smaller in both cleft subgroups than in healthy peers. However, in the latency range of 300 to 500 ms, the MMN was significantly smaller in infants with CPO. In infants with CLP, the MMN was comparable to that of healthy infants. Differences in auditory discrimination between infants with CLP and CPO, as reflected by MMN, were detectable at birth and persisted into later infancy. This pattern parallels known behavioural differences between children with these cleft types. Brain responses to rare sounds, in contrast, had no differentiative power with respect to the cleft type.
Air swallowing in Rett syndrome
Authors: Morton R E , Pinnington L , Ellis R E
Published: July 2010

The possible causes of excessive swallowing of air leading to bloating, which is common in Rett syndrome (RS), were investigated during feeding and at rest. Seven individuals with RS aged between 4 and 33 years (three with air bloat) underwent feeding videoflouroscopy and concurrent respiration monitoring. The results were compared with a randomly selected group of 11 individuals, aged between 2 and 16 years, with quadriplegic cerebral palsy and feeding problems, some of whom had mild air bloat. All individuals from both groups had isolated pharyngeal swallows and several mouth breathed; this may account for some air swallowing but not the severe air bloat characteristic of RS. Thirty‐three individuals with RS aged between 3 and 44 years were monitored for nasal respiration, chest movements, swallowing, and vocal cord position at rest (between feeding). Twenty had air bloat, 17 of whom swallowed air during breath‐holding in the same way, and three gulped air during hyperventilation. Of the 13 without air bloat, eight did not have recurrent breath‐holding and five did, but without concurrent air swallowing. Several methods for reducing air swallowing in apnoea were investigated. The most successful was a dummy with an air leak, but this was poorly tolerated and could only be used for short periods of time. Apnoeas and air bloat are often worse when individuals are distressed and may in some individuals be reduced by anxiolytic medications.
Body composition in nutritionally adequate ambulatory and non‐ambulatory children with cerebral palsy and a healthy reference group
Authors: Chad K E , McKay H A , Zello G A , Bailey D A , Faulkner R A , Snyder R E
Published: July 2010

Bone‐mineral content (BMC; g) and density (BMD; g/cm2) were measured by dual energy X‐ray absorptiometry in the proximal femur, femoral neck, and total body of nutritionally adequate children (n=17; 11 girls, six boys; aged 7.6 to 13.8 years) with spastic cerebral palsy (CP). Bone‐mineral‐free lean tissue (BMFL; g) and fat mass (FM; g) were obtained from total body scans. Chronological and developmental age‐based z scores for the children with CP were derived from a pediatric database (n=894). Children with CP had BMC z scores from ‐1.8 (total body) to ‐3.2 (femoral neck) SDs below the normative sample. Non‐independent ambulators had lower z scores for total body BMD, femoral neck BMD, and BMC than independent ambulators. The BMFL z score of individuals with CP was 2 SDs below that of the reference group and higher in the independent ambulators than in the non‐independent ambulators, whereas FM deviated little. These findings suggest that non‐nutritional factors, such as ambulation, account for the low BMC, BMD, and BMFL tissue observed in this population.
Recurrent artery of Heubner infarction in infancy
Authors: Miller Steven P , O'Gorman Augustin M , Shevell Michael I
Published: July 2010

Classically, acquired occlusion of the recurrent artery of Heubner (RAH) results in hemiparesis with faciobrachial predominance. Infarction in the territory of the RAH represents a specific stroke syndrome not yet described in infancy with a range of motor and functional manifestations. An infant is described with apparent congenital infarction of the recurrent artery of Heubner. The child had prominent involvement of the contralateral upper extremity with athetosis. Neuroimaging changes were evident in the vascular territory classically attributed to the RAH. The clinician should suspect congenital RAH infarction in those infants with congenital upper‐extremity athetosis.
Effect of vitamin D and calcium on bone mineral density in children with CP and epilepsy in full‐time care
Authors: Jekovec‐Vrhovsěk Maja , Kocijancic Andreja , Preželj Janez
Published: July 2010

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.
Neonatal cerebral ischaemia with elevated maternal and infant anticardiolipin antibodies
Authors: Chow Gabriel , Mellor David
Published: July 2010

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.
Cytochrome oxidase deficiency presenting as birth asphyxia
Authors: Willis Tracey A , Davidson Judith , Gray R George F , Poulton Kelvin , Ramani Pramila , Whitehouse William
Published: July 2010

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.
Human herpesvirus‐6 associated encephalitis with subsequent infantile spasms and cerebellar astrocytoma
Authors: Rantala Heikki , Mannonen Laura , Ahtiluoto Satu , Linnavuori Kimmo , Herva Riitta , Vaheri Antti , Koskiniemi Marjaleena
Published: July 2010

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 2 cm 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.
Neurological and MRI profiles of children with developmental language impairment
Authors: Trauner Doris , Wulfeck Beverly , Tallal Paula , Hesselink John
Published: July 2010

Children with developmental language impairment (LI) are defined partly by the absence of other identifiable neurological diagnoses. Such children are generally considered to be neurologically normal, but no systematic studies of neurological function have been reported. We obtained detailed medical histories and conducted neurological examinations for 72 children aged 5 to 14 years with LI and 82 typically developing age‐matched control children. All the children took a standardized test of language, and those who were at least 8 years old and were willing to have brain MRI scans (35 children with LI and 27 control children) had scans. Analysis of developmental milestones from the medical histories revealed that children with LI were not only significantly later in speaking, but also mildly but significantly delayed in motor milestones, particularly walking. On neurological examination, abnormalities were found in 70% of the children with LI and only 22% of the control children. The most common abnormalities in the LI group included obligatory synkinesis, fine motor impairments, and hyperreflexia. The children with LI with the most abnormal neurological findings had the lowest language scores. Finally, 12 of 35 children with LI had abnormalities on their MRI scan, while none of the 27 control children had abnormal scans. Abnormal findings included ventricular enlargement (in five), central volume loss (in three), and white matter abnormalities (in four). These findings suggest that developmental LI is not an isolated finding but is indicative of more widespread nervous system dysfunction. Children with LI may need more comprehensive intervention programs than language therapy alone, depending on their other areas of dysfunction. Early identification of such problems may allow for more successful remediation.
Auditory attention processing in 5‐year‐old children born preterm: evidence from event‐related potentials
Authors: Dupin Remy , Laurent Jean‐Paul , Stauder Johannes E A , Saliba Elie
Published: July 2010

The assumption that children born preterm have difficulties in maintaining active attention was tested in passive and active tasks. Twenty 5‐year‐old children born preterm at 26 to 32 weeks gestational age were compared with 20 children born at term, matched for age and IQ, using an auditory paradigm. In the passive task participants had to watch a videotape of a cartoon and ignore auditory stimuli. In the active task they had to detect a rare tone (the ‘target’ tone; 10% of the tones presented) among frequent tones (the ‘standard’ tone; 90%). Accuracy and reaction time were analysed, and event‐related potentials (ERPs) were recorded at the scalp sites Fz, Cz, T3, T4, Pz, Oz, and two electrodes for the left mastoid (passive task); and Fz, F7, F8, Cz, T3, T4, Pz, and Oz (for the active task). Behavioural and electrophysiological data were analysed with repeated‐measure ANOVAs. The results showed a significant group effect only on the active task. The preterm group scored fewer correct hits (correct detection of target tone) and were less efficient in their attentional strategy as assessed by ERP components.
Abnormal myelin formation in rhizomelic chondrodysplasia punctata type 2 (DHAPAT‐deficiency)
Authors: Sztriha László , Al‐Gazali Lihadh I , Wanders Ronald J A , Ofman Rob , Nork Michael , Lestringant Gilles G
Published: July 2010

The case of a Yemeni girl with isolated peroxisomal acyl‐CoA:dihydroxyacetonephosphateacyltransferase (DHAPAT) deficiency is reported. She had rhizomelic chondrodysplasia punctata, microcephaly, failure to thrive, delayed motor and mental development, and spastic quadriplegia. Deficient de novo plasmalogen synthesis in her fibroblasts as a result of low DHAPAT activity was found, while her very‐long‐chain fatty acid profile, phytanic acid concentration, alkyl‐dihydroxyacetonephosphate synthase (alkyl‐DHAP synthase) activity, and peroxisomal 3‐ketoacyl‐CoA thiolase protein were normal. A mutation in her DHAPAT complementary DNA resulted in the substitution of an arginine residue in the protein at position 211 by a histidine (R211H). Magnetic resonance imaging showed abnormal white matter signal in the centrum semiovale involving the arcuate fibers, while the corpus callosum was normal. DHAPAT and alkyl‐DHAP synthase initiate the synthesis of plasmalogens, which are major constituents of myelin phospholipids. The reported girl's abnormal formation of myelin is probably related to the inadequacy of plasmalogen biosynthesis, which is likely to be due to deficient DHAPAT activity.
Benign partial epilepsy of childhood: a longitudinal neuropsychological and EEG study of cognitive function
Authors: Deonna Thierry , Zesiger Pascal , Davidoff Veronique , Maeder Malin , Mayor Claire , Roulet Eliane
Published: July 2010

The study combined prospective neuropsychological and EEG results of 22 children presenting with typical benign partial epilepsy with rolandic spikes (n=19) and occipital spikes (n=3). The aims were to assess the types of cognitive problems which may be encountered in this population, to evaluate the course of cognitive and learning capacities during the active phase of epilepsy, and to see if there was a correlation with paroxysmal activity on the EEG. Average age at entry in the study was 8.4 years and each child was seen two to four times over a period of 1 to 3 years. EEGs showed persistent spike foci in most cases that worsened in three cases, but there were no continuous spike‐waves during sleep. No child had persistent stagnation, marked fluctuations, or a regression in cognitive abilities. Of 22 children, 21 had average IQ (>80). Eight children had school difficulties requiring special adjustment. No single cognitive profile was identified. Four children had delayed language development and eight children had transient weak scores in one isolated domain (verbal, visuospatial, memory) which improved or normalized during the course of the study with concomitant EEG improvement or normalization. In two of the three children with aggravation of the paroxysmal EEG activity, clinical changes were documented. A proportion of children with typical benign partial epilepsy with rolandic spikes showed mild, varied, and transient cognitive difficulties during the course of their epilepsy, and in most cases this probably had a direct relation with the paroxysmal EEG activity.
Non‐invasive technique for assessment and management planning of oral‐pharyngeal dysphagia in children with cerebral palsy
Authors: Selley Wilfred G , Parrott Lynsey C , Lethbridge Penny C , Flack Frederick C , Ellis Richard E , Johnston Kerry J , Tripp John H
Published: July 2010

The non‐invasive Exeter Dysphagia Assessment Technique (EDAT) was evaluated as a method of assessing the aetiology of dysphagia in children with cerebral palsy (CP). Data were collected from a group of 20 typically developing children (nine girls, 11 boys; age range 7 to 14 years) for comparison with 125 dysphagic children with CP (81 boys, 44 girls; age range 1 to 18 years). The swallowing mechanism has been separated into physiological phases: anticipatory, delivery, oral transit, and oral‐pharyngeal. Normal or abnormal function in each phase was recorded and the common causes of any impaired phase were considered, starting with generalized possibilities before focusing on specific parts of swallowing physiology. Data from 125 dysphagic children with CP show marked differences from the data for the typically developing children. Interpreting individual results was valuable in assisting the assessment team to formulate management strategies; two examples are presented. The technique appears to provide a cost‐effective, non‐invasive, and valuable clinical tool.
Reduced accommodative function in dyskinetic cerebral palsy: a novel management strategy
Authors: Ross Linda M , Heron Gordon , Mackie Roisin , McWilliam Robert , Dutton Gordon N
Published: July 2010

A9‐year‐old boy with dyskinetic cerebral palsy secondary to neonatal encephalopathy is described. He presented with blurring of near vision which had begun to impact on his school work. Objective assessment of accommodation showed that very little was present, although convergence was almost normal. The near‐vision symptoms were completely removed and reading dramatically improved with the provision of varifocal spectacles. Varifocal lenses provide an optimal correction for far, intermediate (i.e. for computer screens), and near distances (i.e. for reading). Managing this type of patient with varifocal spectacles has not been previously reported. It is clearly very important to prescribe an optimal spectacle correction to provide clear vision to optimize learning.
Birth defects in children with newborn encephalopathy
Authors: Felix Janine F , Badawi Nadia , Kurinczuk Jennifer J , Bower Carol , Keogh John M , Pemberton Patrick J
Published: July 2010

This study was designed to investigate birth defects found in association with newborn encephalopathy. All possible birth defects were ascertained in a population‐based study of 276 term infants with moderate or severe encephalopathy and 564 unmatched term control infants. A strong association between birth defects and newborn encephalopathy was found with defects affecting 27.5% of children with encephalopathy and 4.3% of control children (odds ratio 8.55; 95% confidence interval 5.25 to 13.91; p<0.001). In 11.8% of infants with a birth defect the defect was not diagnosed until after the newborn period, illustrating one of the difficulties in attempting to exclude infants with birth defects from studies of newborn encephalopathy. The majority of defects (89%) were not specific anomalies of the CNS. In 36.8% of children with encephalopthy who had a birth defect, the defect was considered to be the probable cause of the encephalopathy. Infants with birth defects who had encephalopathy had a poorer prognosis than those without: they were twice as likely to die by the age of 2 years and three times more likely to have cerebral palsy. This study catalogues the spectrum of birth defects associated with newborn encephalopathy and illustrates the importance of their inclusion when investigating both the aetiology and outcome of this condition.
Comparative study of inter‐ and intrahemispheric somatosensory functions in children with partial and complete agenesis of the corpus callosum
Authors: Friefeld Sharon , MacGregor Daune , Chuang Sylvester , Saint‐Cyr Jean
Published: July 2010

Inter‐ and intrahemispheric somatosensory functions were evaluated in blinded tests of 11 children with partial and complete agenesis of the corpus callosum (age range 2 years 8 months to 11 years 9 months) and compared with those of age‐ and sex‐matched control individuals both normally developing and with neurological impairment. Tests included uni‐ and bimanual measures of stereognosis, haptic visual discrimination, kinaesthesis, and texture matching. Children with callosal absence displayed significant difficulties with many somatosensory functions, but most deficits were no greater than those of the children who had neurological dysfunction and an intact corpus callosum. Children with callosal absence, however, had significantly greater difficulty with bimanual texture matching, seemingly because this task required refined spatial interpretation of somatosensory input. Response times for the individuals with callosal absence were significantly greater than those for control participants with neurological impairment on several tasks in which overall accuracy of performance among groups did not significantly differ. These findings suggest that children with agenesis of the corpus callosum may have less efficient and slower processing capacity for certain types of somatosensory information. Possible compensatory mechanisms are discussed.
Prenatal alcohol exposure and signs of minor neurological dysfunction at preschool age
Authors: Larroque Béatrice , Kaminski Monique , Dehaene Phillipe , Subtil Damien , Querleu Denis
Published: July 2010

High levels of alcohol consumption during pregnancy affect the development of the baby's central nervous system. Pregnant women were interviewed about their alcohol consumption during pregnancy, when they first visited the maternity hospital in Roubaix, France. Of the 698 women interviewed, 156 of their children were investigated at the age of 41/2 years with a standardised examination to assess the effects of prenatal alcohol exposure on neurological status. Two scores were calculated: a posture score, which measured items while the child was standing; and a minor neurological signs score. The posture score was not related to prenatal alcohol exposure. Consumption of 21 drinks/week (3 drinks/ day) or more during pregnancy was significantly associated with a higher number of minor neurological signs, after controlling for relevant covariables. Although prenatal alcohol exposure was related to a lower General Cognitive Index (GCI), minor neurological signs were associated with prenatal alcohol exposure after controlling for GCI.
Five cases of brain injury following amniocentesis in mid‐term pregnancy
Authors: Squier M , Chamberlain P , Zaiwalla Z , Anslow P , Oxbury J , Gould S , McShane MA
Published: July 2010

This paper describes the neuroimaging and neuropathological findings in five cases of severe brain damage after traumatic mid‐trimester amniocentesis, all performed between 1986 and 1994. Although fetal injury after amniocentesis has been reported, reports of brain injury are infrequent. Continuous ultrasound monitoring may reduce the risk of fetal injury but follow‐up ultrasound scans can be falsely reassuring. Withdrawal of blood‐stained fluid, particularly if it contains tissue fragments, should alert the operator to the possibility of fetal damage. Histological examination of such tissue fragments may confirm the nature of the fetal damage. The consequences of fetal brain injury are severe, all five of our cases showed evidence of disruption of brain development compatible with mid‐term injury. Obstetricians and their patients should be aware of the small but significant risk of brain damage after mid‐term amniocentesis.
Intrathecal baclofen for generalized dystonia
Authors: Leland Albright A , Barry Margaret J , Shafron David H , Ferson Susan S
Published: July 2010

The aim of this study was to evaluate the effects of intrathecal bacolfen (ITB) on patients with severe generalized dystonia. Eighty‐six participants ranging in age from 3 to 42 years (median age 13 years) with generalized dystonia refractory to oral medications were offered treatment with ITB. Dystonia was associated with cerebral palsy in 71% of participants. Response to ITB was tested by continuous infusions in 72%, and by bolus injections in 17% of participants who had both dystonia and spasticity. Ninety‐one percent of participants responded to the screening infusion and 93% to the bolus injections. Pumps were implanted in 77 participants. Dystonia scores at 3, 6, 12, and 24 months were significantly decreased (p<0.005) compared with baseline scores. Dystonia scores were significantly lower in those with intrathecal catheters positioned at T4, or higher than in those with catheters at T6 or lower (p=0.005). Ninety‐two percent of participants implanted with a pump retained their responses to ITB during a median follow‐up of 29 months. Patient questionnaires indicated that quality of life and ease of care improved in 86% and speech improved in 33%. Side effects of ITB occurred in 26% of participants. Surgical complications occurred in 38% and included CSF leaks, infections, and catheter problems. ITB is probably the treatment of choice for generalized dystonia if oral medications are ineffective.
Infantile spasms: diagnosis and assessment of treatment response by video‐EEG
Authors: Gaily Eija , Liukkonen Elina , Paetau Ritva , Rekola Merja , Granström Marja‐Liisa
Published: July 2010

The purpose of this study was to investigate early electroclinical manifestations and evaluate treatment responses by video‐EEG in infants with newly diagnosed spasms. Spasms were recorded in 44 infants (27 males, 17 females) before adequate treatment. Mean ages at onset of spasms and at first video‐EEG were 5.3 months (range 0.9 to 9 months) and 5.9 months (range 2.4 to 11.5 months) respectively. Thirteen infants had cryptogenic and 31 had symptomatic aetiology. First treatment was vigabatrin in 36 infants. All infants were followed until 12 months of age or death. Treatment response in the first months of therapy was assessed by repeated video‐EEG studies in 23 infants. On the first video‐EEG, 34 infants had typical symmetric motor spasms, three infants showed asymmetric or asynchronous behaviour, and seven infants had only subtle spasms. Interictal EEG showed hypsarrhythmia in 27 infants and multifocal spikes with normal or nearly normal background in 17 infants. Subtle spasms, asymmetric or asynchronous spasms, and asymmetric ictal or interictal EEG abnormalities were associated with symptomatic aetiology and poor cognitive and seizure outcome at 12 months. Serial video‐EEG recordings showed a transition from motor to subtle spasms during the first 2 weeks of vigabatrin therapy in four infants and only subtle spasms in two therapy‐resistant infants at 12 months. Cessation of spasms usually preceded disappearance of hypsarrhythmia or multifocal spikes, but persistence of multifocal spikes over several weeks was always associated with existing spasms. Transition of hypsarrhythmia into multifocal spikes was observed during vigabatrin therapy even in infants with intractable spasms. Initial diagnosis of infantile spasms requires video‐EEG studies especially in infants with symptomatic aetiology who may show only subtle spasms. Video‐EEG is the only reliable method for assessing treatment response as spasms and interictal EEG abnormalities are modified by treatment and may become subtle.
Lateralized cognitive deficits in children following cerebellar lesions
Authors: Scott Richard B , Stoodley Catherine J , Anslow Philip , Paul Caroline , Stein John F , Sugden Elaine M , Mitchell Christopher D
Published: July 2010

The aim of this preliminary study was to examine the developing cognitive profiles of children with cerebellar tumours in a consecutive series of clinical patients. MRI and longitudinal intellectual profiles were obtained on seven children (two females, five males; mean age 3 years at diagnosis; mean age 7 years at first assessment). Tumours in three of the children were astrocytomas; of the remaining tumours, two were medulloblastomas, one low‐grade glioma, and one ependymoma. In right‐handed children, we observed an association between greater damage to right cerebellar structures and a plateauing in verbal and/or literacy skills. In contrast, greater damage to left cerebellar structures was associated with delayed or impaired non‐verbal/spatial skills. Long‐term cognitive development of the children studied tentatively supports a role for the cerebellum in learning/development. These findings suggest that lateralized cerebellar damage may selectively impair the development of cognitive functions subserved by the contralateral cerebral hemisphere and, in addition, that all children with cerebellar lesions in early childhood should routinely undergo long‐term monitoring of their intellectual development.
Idiopathic central pontine myelinolysis in childhood
Authors: Menakaya J O , Wassmer E , Bradshaw K , Seri S , Whitehouse W P
Published: July 2010

Central pontine myelinolysis (CPM) is rare in childhood with only a few cases reported in world literature. We report a 7‐year‐old male who presented with acute ataxia, swallowing difficulties, dysarthria, and radiological features consistent with the disorder. He improved remarkably with oral prednisolone therapy and was almost back to normal by 2 weeks. A review of the literature is also included.