bluuebunny/crossref_metadata_2025 huggingface dataset:
Delayed visual maturation: pupillary responses implicate subcortical and cortical visual systems
Authors: Cocker Kenneth D , Moseley Merrick J , Stirling Heather F , Fielder Alistair R
Published: July 2010

Vision in very early infancy is probably subserved by subcortical pathways, with many cortical processes only fully emerging by 3 months of age. The improvement of vision in delayed visual maturation (DVM) occurs around this time, and this has given rise to the Suggestion that the condition may have a subcortical basis that resolves with the appearance of cortical function. To explore further the role of cortical and subcortical visual systems in DVM we studied the visual development in identical twins, one of whom had type lb DVM. Two non‐invasive methods of investigating visual pathway function were employed: the acuity card procedure (a behavioural response) and luminance and grating pupillometry. While the former reflects both subcortical and cortical function and can be detected at birth, pupil responses to gratings reflect cortical activity alone and normally become measurable at 1 month of age. Development of both behavioural and pupillary responses was delayed in DVM, indicating that although the underlying defect is primarily subcortical, secondarily it delays the emergence of cortically mediated responses. The observed rapidity of improvement‐over a very few days and within a narrow age range ‐ suggests a discrete rather than a widespread structural abnormality, the improvement of which is closely linked to postmenstrual age.
The impact of diagnostic labelling in population‐based research into cerebral palsy
Authors: Williams Katrina , Alberman Eva
Published: July 2010

This study explores and quantifies the impact on the estimation of prevalence rates and aetiological hypotheses of inclusion and exclusion of different diagnostic labels and types of cerebral palsy (CP). The study was based on data from a CP register which had been established in the English North East Thames Regional Health Authority (NETRHA). As a deliberate policy, no definition of CP was given to notifiers and no inclusion or exclusion criteria were specified. Clinical information, including known malformations, genetic disorders, and features that made the diagnosis doubtful, was requested. Rates and relative risks for different inclusion and exclusion criteria were calculated. The crude rate of CP as defined above was 1.6/1000 (95%CI 1.5,1.7). “Exclusion of all cases with a known or potentially causal” association reduced this rate to 1.2/1000 (95%CI 1.0, 1.3). Comparison with an intensively investigated sample from Germany and Sweden showed that more of the same causal associations (or diagnostic labels) were found, particularly where MRI studies had been carried out. Future comparative studies in CP will need to be very precise in specifying inclusions and exclusions and in estimating the effects they will have on monitoring trends over time and aetiological hypotheses.
Acquired spinal cord lesion associated with os odontoideum causing deterioration in dystonic cerebral palsy: case report and review of the literature
Authors: Amess P , Chong W K , Kirkham F J
Published: July 2010

A boy with a dystonic quadriparesis presented with acquired paralysis, spasticity, and a feeding disorder. Spinal MRI revealed a cervical cord lesion and os odontoideum. Excessive movement of the neck, leading to failure of ossification of the dens and then to cervical cord trauma was the likely mechanism. The poor outcome of this subject is described, emphasising the need to pay careful attention to neurological changes in children with extrapyramidal cerebral palsy, who may be at particular risk of cord pathology. The management issues are discussed.
Academic achievement in children with epilepsy or asthma
Authors: Austin Joan K , Huberty Thomas J , Huster Gertrude A , Dunn David W
Published: July 2010

The purposes of the study were to compare academic achievement between children with epilepsy and those with asthma and to identify child perception, school adaptive functioning, and condition severity factors related to academic achievement. Subjects were 225 children (117 with epilepsy and 108 with asthma) aged between 8 and 12 years. Academic achievement was measured using school‐administered group tests. Self‐report questionnaires were used to measure child attitudes and school self‐concept. Teachers rated school adaptive functioning. Data were analyzed using ANCOVA and multiple regression. Children with epilepsy had significantly lower achievement scores than children with asthma. Boys with severe epilepsy were most at risk for underachievement. Factors related to poor academic achievement in both samples were: high condition severity, negative attitudes, and lower school adaptive functioning scores. Less variance was accounted for in the model for epilepsy (R2=0.25) than for asthma (R2=0.36). Boys with high seizure severity were most at risk for achievement‐related problems. Future research in epilepsy should consider additional factors in the model predicting academic achievement.
Epidermoid tumours associated with lumbar punctures performed in early neonatal life
Authors: Potgieter Steph , Dimin Supion , Lagae Lieven , Van Calenbergh Frank , Plets Christiaan , Demaerel Philippe , Casaer Paul
Published: July 2010

Three children (ages 5,7, and 12 years) with epidermoid tumours in the spinal canal, all of whom had a lumbar puncture during the early neonatal period, are reported. A considerable delay occurred from the first symptoms until the diagnosis was made. MRI of the lumbar spine was the method of choice in the diagnostic work‐up. All three cases were successfully surgically treated. The link between lumbar punctures and epidermoid tumours and the possible risk factors involved are explored. Because of the variable clinical presentation, the possibility of the existence of these tumours should be considered in the differential diagnosis.
Peer problems of 9‐ to 11‐year‐old children with hemiplegia in mainstream schools. Can these be predicted?
Authors: Yud Carole , Goodman Robert
Published: July 2010

Is it possible to predict relatively early in the life of children with disabilities those who are likely to develop peer problems if they get no extra help? This question is examined in our prospective study of a representative sample of children with hemiplegia attending mainstream schools. Hemiplegia is a particularly suitable model for studying integration as it is a relatively homogeneous condition that does not usually preclude mainstream placement. Our aim was to investigate whether the excess of peer problems could be predicted from information obtained some 4 years earlier. A representative sample of 55 children with hemiplegia in mainstream education was followed prospectively from a mean age of 7.1 years (Time 1) to a mean age of 10.7 years (Time 2). Using standardized measures of peer rejection, lack of friends, and victimization, two‐thirds of the sample had at least one of these problems at Time 2. A greater number of peer problems was primarily predicted by two Time‐1 variables: lower IQ and more teacher‐reported externalizing problems (disruptiveness and hyperactivity). A risk index based on these two variables identified a high‐risk subgroup that might particularly have benefited from early intervention to reduce behavioural problems, and nurture social skills. As more children with special needs are integrated into mainstream schools, it is increasingly important to remember that supporting these children requires appropriate provision to foster their social as well as their academic and physical development.
Placental pathology and neurodevelopment of the infant with intrauterine growth restriction
Authors: Gray Peter H , O'Callaghan Michael J , Harvey Jacqueline M , Burke Christopher J , Payton Diane J
Published: July 2010

The placentas of 68 infants with intrauterine growth restriction (IUGR) were examined for evidence of impaired uteroplacental circulation and compared with those of 65 appropriately grown infants. Infarcts and/or accelerated villous maturation were present in the placentas in 27 (40%) of the infants with IUGR compared with seven (11%) of the infants without IUGR (P<0.001). The infants were followed‐up at 4 and 12 months of age and growth parameters recorded. Medical and developmental assessments and neuromotor developmental examinations were also performed. The 23 infants in the IUGR group with placentas with evidence of impaired uteroplacental circulation were compared with the 31 infants with IUGR with normal placentas. There was no difference between the groups in growth, cognitive development, or neuromotor abnormality. It was concluded that IUGR is strongly associated with placental markers of impaired uteroplacental blood flow while it would appear that there is no association between placental pathology and growth or neurodevelopment in the first year.
Prevalence of cerebral palsy in north‐east Italy from 1965 to 1989
Authors: Bottos Michele , Granato Tatiana , Allibrio Giuseppa , Gioachin Caterina , Puato Maria Luisa
Published: July 2010

This study examines the prevalence of cerebral palsy (CP) in two north‐east Italian provinces, Padua and Rovigo (overall population 1 030 000). Six‐hundred and ten children with a diagnosis of CP (330 male, 280 female), born between 1965 and 1989, were studied. The prevalence of CP progressively increased from the 1960s to the mid‐1980s, and then decreased in the 5‐year period, 1985 to 1989. These quantitative changes were associated with qualitative ones. For example, the number of low‐birthweight (LBW) infants progressively increased, similar to results of epidemiological studies from other European countries. The clinical features of the types of CP in this study (hemiplegia, diplegia, ataxic diplegia, quadriplegia, pure ataxia, dyskinesia) generally correspond with those described in other studies. However, the prevalence of quadriplegia in this study is higher. The prevalence of types of CP related to preterm birth, such as diplegia, increased over the years, while those associated with term babies, such as dyskinesia, decreased. This study suggests that prenatal factors are associated with some types of CP, while in others, such as diplegia, quadriplegia, and dyskinesia, the perinatal factors are notable. Perinatal factors were associated with LBW children, while prenatal factors were greater for normal‐birthweight infants. The variation in the prevalence of CP over the years, with two peaks corresponding with the introduction of neonatal intensive care units in Padua and Rovigo, suggests that changes in neonatal care could influence the levels of CP, independent of the original presence of predisposing prenatal factors.
Hypotonia, congenital nystagmus, ataxia, and abnormal auditory brainstem responses: a report on the first white patient
Authors: Polizzi Agata , Mauceri Laura , Ruggieri Martino
Published: July 2010

A white Italian boy, aged 5 years and 8 months, is reported with failure to thrive, hypotonia, truncal ataxia, psychomotor retardation, and congenital horizontal pendular nystagmus with only waves I and II on auditory brainstem responses. Our patient's clinical picture resembles that previously reported in 10 male Oriental patients. He did not manifest spastic diplegia by the age of 2 years, as did the subjects reported in the literature, but knee‐jerk hyperreflexia was evident at the most recent clinical reevaluation. Serial brain MRI studies revealed a cystic brain lesion and peritrigonal hyperintensities with no brainstem abnormalities. To date, no other child with a similar syndrome has been described either in Europe or in America. The clinical features of this condition are consistent and characteristic. A definitive diagnosis is achieved by demonstrating the absence of all waves following wave I or wave II on auditory brainstem responses as early as 3 months of age. Due to the predominance of males, the occurrence in siblings, the early age at onset, the non‐progressive course, and the characteristic auditory brainstem response findings, the syndrome may have a genetic origin and be attributable to a dysgenetic brainstem lesion.
Cognitive deficits after cryptogenic infantile spasms with benign seizure evolution
Authors: Gaily Eija , Appelqvist Kati , Kantola‐Sorsa Elisa , Liukkonen Elina , Kyyrönen Paula , Sarpola Mia , Huttunen Heli , Valanne Leena , Granström Marja‐Liisa
Published: July 2010

Between 1989 and 1994, 18 children with cryptogenic infantile spasms ‐ defined by normal development before onset of spasms, symmetrical hypsarrhythmia or multifocal spikes, and typical spasms on presentation, and no abnormal findings on aetiological studies including neuroradiology ‐were diagnosed and treated. To assess the risk of cognitive impairment later in life, 15 of these 18 children whose spasms completely resolved within the first year of life were studied. Age at onset of spasms varied between 4.4 and 9.8 months (mean 6.5 months). Children were effectively treated with adrenocorticotrophic hormone (10 children), pyridoxine (three), vigabatrin (one), or sodium valproate (one). Spasms lasted between 11 and 138 days (mean 50 days) and stopped between the age of 6.3 and 10.2 months(mean 8.1 months). EEGs normalized between the age of 7.1 and 13.2 months (mean 9.4 months). Early development was assessed on presentation and within a few months after spasms had stopped. A detailed neuropsychological assessment was performed between the age of 4.0 and 5.9 years. Twelve children had normal intelligence; specific cognitive deficits were found in five. Three children had mild learning disability. Abnormal developmental status at age 8 to 15 months after complete resolution of spasms and EEG abnormalities was associated with cognitive deficits at age 4 to 6 years.
High‐ or low‐technology measurements of energy expenditure in clinical gait analysis?
Authors: Boyd Roslyn , Fatone Stefania , Rodda Jill , Olesch Christine , Starr Roland , Cullis Elise , Gallagher Donnacha , Carlin John B , Nattrass Gary R , Graham Kerr
Published: July 2010

The repeatability of energy‐expenditure measurements were studied in five children and four adults without disabilities using the Cosmed K4 (high technology). The ability to detect change in measurements was compared between this instrument and the Physiological Cost Index (PCI; low technology). The results of repeatability (95% range) for oxygen cost were 13.1% in children and 13% in adults. In contrast, the SD of PCI was 6 to 72% of the mean in adults and wider in children (91%; 95% range). The validity of PCI as an outcome measure was questioned. In addition, 177 children with motor disability were prospectively studied using the Cosmed K4. Previous experience with the Cosmed K2 (intermediate technology) helped to develop a practical and repeatable protocol for testing children with disability using the Cosmed K4. The protocol commenced with 5 minutes of rest to achieve baseline values of heart rate and oxygen consumption, followed by 10 minutes of continuous walking at a self‐selected speed on a 10–metre level oval walking track. The test concluded with 5 minutes of rest to monitor the return to baseline values. Ninety‐one percent of the children with disability quickly reached a steady‐state of oxygen consumption and carbon‐dioxide production. The carbon‐dioxide sensor in the Cosmed K4 has enabled a new group of severely involved children with cerebral palsy (9%) to be defined. These children have been termed ‘physiologically marginal ambulators’.
Medical aspects of school‐aged children with Down syndrome
Authors: Leonard Seonaid , Bower Carol , Petterson Beverly , Leonard Helen
Published: July 2010

Current and comprehensive information about the medical issues affecting children with Down syndrome (DS) is of value in counselling parents who are considering prenatal diagnosis and in planning services for people with DS as they age, especially given the continued improvements in their survival. Parents of school‐aged children (mean age 11.37 years, 57.3% male, 42.7% female) with DS were identified by linking registers from the Disability Services Commission and the Birth Defects Registry. Less than half the children had cardiac and bowel conditions. More than half had ear conditions and more than three quarters had eye conditions. Ear, nose, and throat professionals were the specialists seen most often and the rate of tympanostomy tube insertion was nearly 17 times that of the general childhood population. Children with DS were over five times more likely to wear glasses than other children. These findings suggest that chronic, non life‐threatening conditions impose a burden on families but do not threaten quality of life.
Painful seizures with allodynia in an 11–year‐old boy
Authors: Kocher Laurence , Rambaud Loïc , Rousselle Christophe , Mottolese Carmine , Ryvlin Philippe , Gonnaud Pierre‐Marie
Published: July 2010

An 11–year‐old boy's epileptic seizures started with a feeling of impending crisis, dizziness, headache, and a bad taste in the mouth. This was followed by swallowing and a burning sensation in the left hand. At the same time, other parts of the body experienced allodynia. MRI and CT scans showed a right anteromesial temporal lesion which proved at neuropathology to be a ganglioglioma. Lesionectomy resulted in complete cessation of seizures. Seizures were absent at an 18–month follow‐up. Allodynia is discussed in relation to the locality of the lesion.
Behavioral and neuropsychological correlates of hyperactivity and inattention in Brazilian school children
Authors: Brito Gilberto N O , Pereira Claudia C S , Santos‐Morales Tanna R
Published: July 2010

Attempts at subtyping attention‐deficit‐hyperactivity disorder (ADHD) along the hyperactivity dimension are considered controversial. This study addresses this issue by dividing a non‐clinical sample of Brazilian children (mean age, 9.4 years; SD, 2.9), who were attending a mainstream school in the Greater Rio de Janeiro area, into four behavioral domain groups (normal [NO, N=324], hyperactive/impulsive [HI, N=17], inattentive [IA, N=48], and combined [C,N=13]) on the basis of teacher ratings on an ADHD scale. The groups did not differ in intellectual level as determined by the Human Figure Drawing test. Comparisons were made between groups along the factorial dimensions extracted from the Composite Teacher Rating Scale, academic performance and neuropsychological measures were then performed. Our data showed that IA and C children are less independent and more prone to socialization problems than NO children, and that HI and C children are less anxious and fearful than IA children. Furthermore, the groups differed in academic and neuropsychological performance. The results could be considered consistent with the hypothesis that ADD with hyperactivity (ADD/+) and ADD without hyperactivity (ADD/‐) represent singular nosological entities.
Stance balance control with orthoses in a group of children with spastic cerebral palsy
Authors: Burtner Patricia A , Woollacott Marjorie H , Qualls Clifford
Published: July 2010

Although ankle‐foot orthoses (AFOs) are frequently prescribed to correct skeletal malalignment in children with spastic diplegia, their effect on standing balance abilities has not been documented. This study investigated balance differences related to the presence of pathology and orthotic conditions during conditions of unexpected stance perturbation by comparing four children aged between 3 and 15 years with spastic cerebral palsy and four control children matched for years of independent walking experience. Electromyographic and kinematic data were collected and compared between groups and in three orthotic conditions (no AFOs, solid AFOs, dynamic AFOs). Results revealed that balance responses of children with spasticity were characterized by: increased coactivation of muscles as opposed to distal to proximal recruitment, decreased presence of upright posture in stance, increased use of‘on‐toes’strategies, and different sway characteristics compared with the typically developing children. In both groups of children, the use of solid AFOs during perturbed stance resulted in: decreased activation of gastrocnemius muscles, disorganized muscle‐response patterns, decreased use of ankle strategies, and increased joint angular velocities at the knee compared with conditions without AFOs or with dynamic AFOs. These preliminary results support the use of dynamic AFOs to correct skeletal malalignment in children with spastic diplegia.
The relation between intracranial pressure and outcome in non‐accidental head injury
Authors: Barlow Karen M , Minns Robert A
Published: July 2010

The aim of this retrospective study was to ascertain whether physiological derangement and potential secondary brain insult from raised intracranial pressure (ICP) or reduced cerebral perfusion pressure (CPP) in non‐accidental head injury (NAHI) influences outcome. Any child who had a diagnosis of NAHI and had ICP monitoring or measurements during the acute illness was entered in the study. Seventeen children with an average age 5.1 months (range 1 to 20 months) were identified. Details of the acute encephalopathy, lowest mean arterial blood pressure (MAP), mean of maximum ICP measured, lowest CPP, and neurodevelopmental outcome at follow up were obtained from the hospital case notes. Seshia's (1994) outcome classification scale was used. The lowest CPP was very significantly related to outcome (P=0.0047, τ=–0.544). Mean of maximum ICP did not correlate with outcome. The lowest MAP was significantly related to outcome (P=0.039). It was concluded that the degree of secondary brain insult from reduced CPP influences outcome. Developmental Medicine & Child Neurology 1999, 41: 220–225
The effect of botulinum toxin A on gastrocnemius length: magnitude and duration of response
Authors: Eames Niall WA , Baker Richard , Hill Nan , Graham Kerr , Taylor Trevor , Cosgrove Aidan
Published: July 2010

Thirty‐nine ambulant children (22 with hemiplegia, 17 with diplegia) with spastic cerebral palsy receiving isolated gastrocnemius muscle injection with botulinum toxin A were studied prospectively. The children had a mean age of 6 years (range 3 to 13 years). Measurement of gastrocnemius muscle length was used to estimate the dynamic component of each child's spasticity and to quantify the response. There was a strong correlation between the dynamic component of spasticity before injection and the corresponding magnitude of the response after injection. Children undergoing repeated injections showed similar correlations. A strong correlation was found between the duration of response and the dynamic component. Children with hemiplegia showed twice the duration for a given dynamic component compared with those with diplegia when injected with the same total dose per unit body weight. Long‐term lengthening did not occur for the cohort, although some patients showed a response at a 12‐month follow‐up. By delaying shortening, the injections may have a role in delaying the need for surgery. Injections were well tolerated with few side effects.
Congenital paraplegia following maternal hypotension
Authors: Suri S , Salfield S , Baxter P
Published: July 2010

Congenital paraplegia, with partial improvement over the first months of life, occurred in a female neonate born at 35 weeks’gestation, whose mother had suffered hypotension and hypoxaemia due to anaphylaxis 12 weeks earlier. Our patient subsequently had an acute encephalopathy during a respiratory illness with later developmental delay. We speculate that the baby's paraplegia resulted from spinal‐cord ischaemia in utero. The aim of this report is to describe the spinal presentation.
Executive function of children with extremely low birthweight: a case control study
Authors: Harvey Jacqueline M , O'Callaghan Michael J , Mohay Heather
Published: July 2010

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.
Survivors of neonatal extracorporeal membrane oxygenation at school age: unusual findings on intelligence testing
Authors: Iklé Linda , Iklé David N , Moreland Susan G , Fashaw Lucy M , Rosenberg Adam R
Published: July 2010

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.
Spinal cord insults in the prenatal, perinatal, and neonatal periods
Authors: Ruggieri Martino , Pike Michael
Published: July 2010

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.
Ictal hemiparesis
Authors: Dale Russell C , Cross J Helen
Published: July 2010

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.
Consciousness in congenitally decorticate children: developmental vegetative state as self‐fulfilling prophecy
Authors: Shewmon D Alan , Holmes Gregory L , Byrne Paul A
Published: July 2010

According to traditional neurophysiological theory, consciousness requires neocortical functioning, and children born without cerebral hemispheres necessarily remain indefinitely in a developmental vegetative state. Four children between 5 and 17 years old are reported with congenital brain malformations involving total or near‐total absence of cerebral cortex but who, nevertheless, possessed discriminative awareness: for example, distinguishing familiar from unfamiliar people and environments, social interaction, functional vision, orienting, musical preferences, appropriate affective responses, and associative learning. These abilities may reflect‘vertical’plasticity of brainstem and diencephalic structures. The relative rarity of manifest consciousness in congenitally decorticate children could be due largely to an inherent tendency of the label‘developmental vegetative state’to become a self‐fulfilling prophecy.
Behaviour of Finnish 3‐year‐old children. I: Effects of sociodemographic factors, mother's health, and pregnancy outcome
Authors: Uljas Heikki , Rautava Päivi , Helenius Hans , Sillanpää Matti
Published: July 2010

The aim of this prospective follow‐up study was to assess the effects of pre‐ and perinatal factors on children's behaviour at the age of 3 years. Nulliparous women who attended a maternity health care clinic were invited to participate: 1443 women were included. Data were obtained from the mother, father, and clinic nurse. A Finnish translation of Achenbach's Child Behavior Checklist for Ages 2–3 was completed by 1086 families at the child's 3‐year visit to the well‐baby clinic. The results were analysed using Pearson's c 2, multivariate regression analysis, and coefficient of determination (100’r2). A good basic education (i.e. >9 years) of the mother and age over 25 years were associated with low syndrome‐scale scores, indicating fewer behavioural problems. Poor health of the mother, a high number of pregnancy‐related symptoms, and low Apgar scores for the newborn infant were associated with high syndrome‐scale scores. In this population sample, the mother's health during pregnancy together with level of education, and marital status are more important in explaining the variation in a child's behaviour than biological risks during pregnancy.
Early brain proton magnetic resonance spectroscopy and neonatal neurology related to neurodevelopmental outcome at 1 year in term infants after presumed hypoxic–ischaemic brain injury
Authors: Amess P N , Penrice Juliet , Wylezinska Marzena , Lorek Ann , Townsend Janice , Wyatt J S , Amiel‐Tison Claudine , Cady E B , Stewart Ann
Published: July 2010

This study investigated the accuracy of prediction of neurodevelopmental outcome at 1 year using cerebral proton magnetic resonance spectroscopy (MRS) and structured neonatal neurological assessment in term infants after presumed hypoxic–ischaemic brain injury. Eighteen control infants and 28 infants with presumed hypoxic–ischaemic brain injury underwent proton MRS investigation. Studies were carried out as soon as possible after the cerebral insult, most within 48 hours. Infants had an early structured neurological assessment at a median of 19 hours (range 0 hours to 9 days) from the presumed hypoxic–ischaemic insult and a late assessment at a median of 7 days (range 3 to 25 days) during recovery. The maximum cerebral peak–area ratio lactate:N‐acetylaspartate measured by proton MRS accurately predicted adverse outcome at 1 year with a specificity of 93% and positive predictive value of 92%. Neurological assessment had a tendency for false‐positive predictions. However, both early and late neurological examination can be used as a reliable indicator for a favourable outcome at 1 year having negative predictive values of 100% and 91% respectively.
Spinal cord ultrasonography in children with myelomeningocele
Authors: Brezner Amichai , Kay Barrie
Published: July 2010

Secondary tethering of the spinal cord in people with myelomeningocele (MMC) is almost universal but there is a relatively low incidence of the secondary tethered cord syndrome (S‐TCS). In view of this, we wish to explore the notion that cord pulsation, as demonstrated by ultrasound of the spinal cord, can become an independent measure for increased tension of the spinal cord in patients with MMC. One‐hundred and six patients with MMC underwent ultrasonographic examination of their back, a thorough physical examination, and a careful review of their clinical history. Of these patients, 53 had MRI of their back. Cord pulsation was graded and the statistical relation between pulsation and various clinical and MRI findings was analysed. Age and arm span were not correlated with grades of cord pulsation. The absence of cord pulsation correlated with the presence of symptoms but this relation may be explained by MRI findings of certain structural cord malformations and not by tethering per se. Assuming that cord pulsation is a reliable marker for the tension within the spinal cord, the results of our study suggest that overstretching of the cord is not the main cause for the development of the S‐TCS.
Does academic achievement in children with epilepsy change over time?
Authors: Austin Joan K , Huberty Thomas J , Huster Gertrude A , Dunn David W
Published: July 2010

A 4‐year follow‐up study of academic achievement in children aged between 11 and 17 years with epilepsy or asthma was carried out to identify differences between the two samples and to identify change in achievement over time. Differences based on sex and seizure severity also were explored. There were 98 subjects in the group with epilepsy and 96 subjects in the group with asthma. Academic achievement in five areas (Composite, Reading, Mathematics, Language, and Vocabulary) was measured using school‐administered group test scores. To explore change over time in condition severity, each child was categorized as having a low or high condition severity at baseline (time I) and again 4 years later, resulting in four groups: low–low, low–high, high–low, and high–high. There were too few cases in the low–high group to be included in the analyses. Data were processed using analysis of covariance (ANCOVA), intraclass correlation coefficients, and paired t tests. At follow‐up the children with epilepsy continued to perform significantly worse in all five achievement areas than the children with asthma. Children with either inactive or low‐severity epilepsy had mean scores comparable to national norms; those with high seizure severity had mean scores ranging from 3 to 5 points below national norms. No changes were found in academic achievement over time for either sample, even among those whose conditions improved. Although boys with high‐severity epilepsy continued to have the lowest achievement scores, there was no trend for them to decline in achievement over time.
Neurobehavioral evidence for working‐memory deficits in school‐aged children with histories of prematurity
Authors: Luciana Monica , Lindeke Linda , Georgieff Michael , Mills Marla , Nelson Charles A
Published: July 2010

Cognitive performance in 7‐ to 9‐year‐old preterm neonatal intensive‐care survivors was compared with that in age‐matched control children. Non‐verbal memory span, spatial working‐memory abilities, planning, set‐shifting, and recognition memory for both spatial and patterned stimuli were assessed using the Cambridge Neuropsychological Testing Automated Battery. Relative to children in the control group, neonatal intensive‐care unit (NICU) survivors demonstrated 25% more memory errors on the spatial working‐memory task. Their use of strategy on this task was similar to a control group of 5‐year‐olds. Planning times on‘Tower of London’problems were long relative to those of term controls. NICU survivors demonstrated poorer pattern recognition as well as a shorter spatial memory span. The groups did not differ in visual‐discrimination learning or in spatial‐recognition memory. No specific neonatal risk factor accounted for the observed differences, although scores on the Neurobiological Risk Score (NBRS), a composite measure of neonatal risk, did predict several aspects of later task performance. Whether these data reflect a developmental delay in brain maturation in NICU survivors or the presence of a permanent information‐processing deficit due to adverse neonatal events must be assessed through continued follow‐up.
Health‐related quality of life in childhood epilepsy: the results of children's participation in identifying the components
Authors: Ronen Gabriel M , Rosenbaum Peter , Law Mary , Streiner David L
Published: July 2010

Separate groups of children with epilepsy, recruited from a regional pediatric epilepsy database, and their parents were established to discuss their life with epilepsy. Twenty‐nine children (aged between 6 years and 10 years 4 months) and 42 of their parents were placed into nine and 17 groups respectively. The participants provided information about their own perceptions of life with epilepsy. Discussions were taped and textual analysis followed to extract, understand, explain, and categorize the health‐related quality of life (HRQL) components. The process enabled us to identify the burdens and concerns of children with epilepsy, and to identify five emerging dimensions: (1) the experience of epilepsy, (2) life fulfillment and time use, (3) social issues, (4) impact of epilepsy, and (5) attribution. Identifying and understanding the components of HRQL is crucial for developing an HRQL scale in childhood epilepsy. In addition, this list of elements can help health professionals improve their services by considering and addressing aspects of the epilepsy experience beyond the traditional issues for children with epilepsy and their families.
Relation between clinical measures and fine manipulative control in children with hemiplegic cerebral palsy
Authors: Gordon Andrew M , Duff Susan V
Published: July 2010

The present investigation examines the relation between various clinical measures and the performance of a functional precision grip‐lift task. Fifteen children with hemiplegic cerebral palsy (CP), aged 8 to 14 years, and 15 age‐matched control children grasped and lifted an object whose surface texture was varied while their fingertip forces were recorded. The force coordination was compared with tactile sensibility, grip strength, manual dexterity, and spasticity using correlational and regression analyses. The findings highlight the importance of tactile sensibility in this task. However, the manner in which sensibility was related varied for the sensory adaptation of fingertip forces, the anticipatory scaling of the force increase, and the smooth transitions between the temporal phases comprising the grip‐lift task. The findings also indicate that spasticity affects some measures of the task, but not others, suggesting that the relation between spasticity and motor performance may not be absolute. The results further suggest that the impairments in grasping in children with hemiplegic CP are largely but not exclusively due to disturbed sensory mechanisms which may have direct implications for therapeutic intervention.
Development of an instrument to measure manual praxis
Authors: O'Hare Anne , Gorzkowska Jill , Elton Rob
Published: July 2010

The purpose of this study was to develop an instrument to measure manual praxis by assessing areas of function considered important in learning and planning movement; use of tools, imitation of gesture, and motor sequencing. Participants included 362 healthy children aged between 3 and 12 years. Their parents completed a questionnaire on hand skills. Performance on all tasks improved with age. However, transitive gestures reached an early ceiling effect at around 5 years of age. Assessment of manual praxis from the parental questionnaire correlated with the child's directly measured competency during a test of object use (P<0.01) but not with the ability to imitate transitive gestures or enact a motor sequence with a‘novel’task. Children from schools where a higher number were eligible for free school meals because of low family income had more difficulty with imitation of gesture and motor sequencing to verbal and picture commands (P<0.001, P<0.05, P<0.05). They were neither disadvantaged in tool use nor in appreciation of extracorporeal space. The pattern of errors made in tool use and motor sequencing differed from those described in adults with acquired dyspraxia. These results suggest that assessment of manual praxis in clinical populations should include tool use, imitation of gesture, and motor sequencing and that they should not necessarily be regarded as measuring a unitary function.
Sensory‐modulation disruption, electrodermal responses, and functional behaviors
Authors: McIntosh Daniel N , Miller Lucy Jane , Shyu Vivian , Hagerman Randi J
Published: July 2010

It was hypothesized that children clinically identified with sensory‐modulation disruptions (SMD) would have atypical physiological responses to sensation, and that such responses would predict parent‐reported behavioral responses to sensation. Nineteen children with clinically identified disruptions, aged 3 to 9 years, mean 6.0 years, and 19 age‐and sex‐matched healthy (control) children, aged 3 to 9 years, mean 6.6 years, were examined. The subjects were presented with five stimuli. Ten trials were conducted for each stimulus and the electrodermal activity of the child was recorded. Four children with SMD did not show electrodermal responses (EDR) to stimulation; all control children responded. Excluding non‐responders, children with SMD showed more and larger EDR than control children. Participants with disruptions habituated more slowly to repeated stimulation, as measured by the number of responses to stimuli and proportion of stimuli that evoked responses. Children with atypical EDR had more parent‐reported abnormal behavioral responses to sensation. Children with clinically identified SMD respond physiologically differently to sensory stimuli than typically developing children; these differences have ramifications for functional behavior.
Modulations in breathing patterns during intermittent feeding in term infants and preterm infants with bronchopulmonary dysplasia
Authors: Craig C M , Lee D N , Freer Y N , Laing I A
Published: July 2010

As infants with bronchopulmonary dysplasia (BPD) have difficulty maintaining adequate levels of oxygenation during rest, it was decided to investigate how the additional respiratory demands associated with nutritive feeding disrupt their breathing rates. The sucking and breathing patterns of six (three male, three female) preterm infants (between 23 and 29 weeks gestational age at birth), classified as having BPD were individually compared with the patterns observed in 12 (six male, six female) healthy term (control) infants (< 38 weeks gestational age at birth) with no known respiratory ailments. All infants were recruited from the neonatal unit at Simpson's Maternity Pavilion, Edinburgh, Scotland. In general, the breathing patterns recorded for the infants with BPD during the pause periods of intermittent feeding lacked the striking regularity observed in the term infants. It was found that the severity of the BPD affected breathing rates by significantly reducing the duration and the regularity of a breath (P<0.05) while sucking during the intermittent phase of feeding.
Visual evoked potential evidence of albino‐like chiasmal misrouting in a patient with Angelman syndrome with no ocular features of albinism
Authors: Thompson D A , Kriss A , Cottrell S , Taylor D
Published: July 2010

An 8‐month‐old boy with global developmental delay, including visual and hearing inattention, was examined in the ophthalmic clinic. Monocular flash visual evoked potentials demonstrated a crossed asymmetry in scalp distribution, a feature considered to be pathognomic of albinism.

Remarkably a foveal reflex was noted in each eye and this patient did not have nystagmus, iris transillumination, nor conspicuously pale fundi. The optic discs appeared normal. He was noted to have very fair skin and hair, with a small head and flat occiput. Cytogenetic studies demonstrated a microdeletion of the maternal chromosome 15q11–q13, and he was diagnosed with Angelman syndrome.
Persisting motor control problems in 11‐ to 12‐year‐old boys previously diagnosed with deficits in attention, motor control and perception (DAMP)
Authors: Christiansen Annette Sandahl
Published: July 2010

The aim of this study was to examine whether boys who had been previously diagnosed between the ages of 5 and 8 years with deficits in attention, motor control and perception (DAMP) still have problems with motor control, which influence their spare‐time and everyday activities, at 11 to 12 years. The study comprised a well defined cohort of 10 boys with DAMP and a control group of 20 boys without DAMP matched for age, height, and weight. The Movement Assessment Battery for Children was used to assess motor control in ability to perform everyday activities, and the spare‐time activities in which the boys participated were recorded. Individually, the boys previously diagnosed with DAMP had a markedly higher total score (poor performance) than the boys without DAMP (P<0.001). The everyday activities of boys with DAMP were significantly affected, and they chose to participate in different sports from the control boys, i.e. none participated in team sports. The present study does not support the concept of improvements in motor control with age in children with DAMP.