bluuebunny/crossref_metadata_2025 huggingface dataset:
Non‐progressive congenital ataxia with or without cerebellar hypoplasia: a review of 34 subjects
Authors: Steinlin Maja , Barbara Zangger MD  , Eugen Boltshauser MD
Published: July 2010

Information on the long‐term development of larger series of children with non‐progressive congenital ataxia (NPCA) is scarce. We have updated a personal cohort of subjects previously diagnosed as having NPCA. Children with brain malformations, acquired neurological illness, or defined syndromes were excluded. From 58 subjects, 34 were available for review (including three pairs of siblings). All our subjects had delayed motor and speech development. Truncal ataxia persisted but became less significant. Two subjects developed spasticity and three a focal dystonia. Epilepsy was a feature in 10 of the subjects. Cognitive impairment was present in 22 of 34 subjects. MRI was normal in 15 of 27. There were no obvious correlations between degree of motor delay, severity of ataxia, cognitive impairment, and neuroimaging. Although genetically and clinically not a homogeneous entity, NPCA is a helpful diagnostic label. Major problems arise in the majority of subjects related to cognitive impairment and less to neurological symptoms. Early individual prognosis is not possible from early developmental milestones', neurological signs, or neuroimaging.
Sleep dysfunction in Rett syndrome: a trial of exogenous melatonin treatment
Authors: McArthur Angela J , Budden Sarojini S
Published: July 2010

Nine girls with Rett syndrome (mean age, 10.1 years) were monitored 24 hours a day over a period of 10 weeks using wrist actigraphy. Baseline sleep‐wake patterns were assessed for 1 week. Subsequently, patients underwent a 4‐week melatonin treatment period in a double‐blind, placebo‐controlled, crossover protocol that employed a 1‐week washout between treatment trials. Melatonin doses ranged from 2.5 to 7.5 mg, based upon individual body weight. Baseline sleep quality was poor compared with healthy children. At baseline the group demonstrated a low sleep efficiency (mean [±SE], 68.0±3.9%), long sleep‐onset latency (42.1±12.0 minutes), and a short and fragmented total sleep time (7.5±0.3 hours; 15±2 awakenings per night). Melatonin significantly decreased sleep‐onset latency to (mean ± SE) 19.1±5.3 minutes (P<0.05) during the first 3 weeks of treatment. While the variability of individual responsiveness was high, melatonin appeared to improve total sleep time and sleep efficiency in the patients with the worse baseline sleep quality. Finally, a 4‐week administration of melatonin appears to be a safe treatment as no adverse side effects were detected, yet long‐term effects of chronic melatonin use in pediatric patients are unknown at this time.
Bladder control a consequence of maturation: evidence after renal transplantation
Authors: Neuhaus Thomas J , Ritter Susanne , Largo Remo H
Published: July 2010

This report contains case studies on three children with early end‐stage renal failure due to renal malformation or nephrotic syndrome, but without bladder involvement. All patients became anuric in the second year of life, before having obtained bladder control. They underwent successful cadaveric renal transplantation, having been anuric for almost 2 to 4 years. When the bladder catheter was removed 5 days after transplantation, all three children asked for the urine potty without ever having been prompted. Three weeks after transplantation, all three children achieved complete bladder control during the day, and two of them also at night. These observations add further evidence to the notion that the development of bladder control is a consequence of maturation and not of training.
Prognostic value of flash visual evoked potentials in preterm infants
Authors: Shepherd A J , Saunders K J , McCulloch D L , Dutton G N
Published: July 2010

This paper investigates the prognostic value of flash visual evoked potentials (VEPs) recorded in preterm infants at birth and at term age with respect to severe neurological outcome. Flash VEPs were recorded in 81 preterm infants at birth (i.e. <35 weeks’ gestation) and repeated in 56 of these infants at term age. The preterm infants were assigned to either a healthy or at‐risk subgroup based on clinical birth factors. Normal ranges of flash‐VEP latencies, amplitudes, and number of components present were obtained from the subgroup of healthy preterm infants and from 68 term infants tested postnatally. The flash‐VEP results of the entire preterm group were compared with the normal ranges and any abnormalities noted. Seven preterm infants in the at‐risk group died, six of whom had abnormal flash VEPs before term age. Of the five infants from the at‐risk group diagnosed with cerebral palsy (CP), three had abnormal flash VEPs before term age. Thus the sensitivity and specificity of the flash VEP with respect to survival was 86% and 89% respectively, and with respect to the development of CP was 60% and 92%. The abnormal features of the flash VEP associated with adverse outcomes comprised a delayed N3 component before term age and the absence of a positive component (P2) at term age.
Characterization and comparison of autistic subgroups: 10 years' experience with autistic children
Authors: Fattal‐Valevski Aviva , Kramer Uri , Leitner Yael , Nevo Yoram , Greenstein Yoram , Harel Shaul
Published: July 2010

To summarize our 10‐year experience with autistic children at the Tel Aviv Child Development Center, the files of all 55 children with autism treated at our center over a 10‐year period were retrospectively reviewed. Particular attention was addressed to the value of the medical work‐up in detecting the etiology of autism and to factors differentiating infantile autism (IA) from autistic‐like behavior (ALB). Twenty‐four subjects (44%) had IA and 31 (56%) had ALB. These subgroups were compared for demographic, perinatal, familial, neurological, and psychological findings, and outcome at discharge. Associated medical conditions and the yield of metabolic work‐up and neuroimaging and electroencephalography studies are discussed. The two subgroups differed only in severity of autistic symptoms and cognitive function (P<0.05), but not in demographic or neurobiological findings. It was concluded that IA and ALB are similar conditions, and autism has a wide continuum of clinical expressions.
Brain perfusion SPECT and MRI in foetal alcohol syndrome
Authors: Riikonen R , Salonen I , Partanen K , Verho S
Published: July 2010

Six boys and five girls with a mean age of 8.6 (range 3 to 13) years with foetal alcohol syndrome (FAS) were studied by MRI and single photon emission computed tomography (SPECT) to find specific areas of vulnerability. Morphological anomalies shown in six of 11 patients by MRI were situated both cortically and subcortically: cortical atrophy (N=2), dilated ventricle (N=1), corpus callosum hypoplasia (N=1), cerebellar atrophy (N=2), one of the latter with Arnold‐Chiari malformation (N=1). Delayed myelination of the white matter was seen in two patients. Volumetric studies of the hippocampus showed morphological left‐right asymmetry in five of eight patients. However, SPECT showed mild hypoperfusion of the left hemisphere in all 10 subjects. The negative left‐right index was located especially in the left parietooccipital region, i.e. in the brain areas implicated in arithmetical and logical‐grammatical functions, which are known to be affected in FAS. Normal left‐right dominance was also lacking in the frontal area, i.e. the brain area affected in attention‐deficit‐hyperactivity disorder (ADHD). Detection of these abnormalities, although they are not unique to FAS, may be helpful in the diagnosis and any attempts at rehabilitation. Diverse morphological and functional abnormalities are more frequent than has usually been believed even in less impaired children with FAS.
Cutaneous flexion reflex in human neonates: a quantitative study of threshold and stimulus‐response characteristics after single and repeated stimuli
Authors: Andrews Katharine , Fitzgerald Maria
Published: July 2010

The cutaneous flexion reflex has been used to study spinal sensory processing in 68 infants (37 female, 31 male) aged between 28 and 42 weeks postconceptional age (PCA). Mechanical and electrical stimuli were singularly and repeatedly applied to the foot, and single‐surface EMG responses were recorded from the biceps femoris muscle. A clear correlation was demonstrated between the mechanical stimulus intensity and latency and the amplitude of the reflex. Mechanical threshold normally increased with age, but the flexion‐reflex threshold was lowered by local limb‐tissue damage in the contralateral limb. The incidence of response to repeated mechanical stimulation at 2.8 x threshold decreased significantly with increasing age. Repeated mechanical stimulation at 2.8 threshold caused a build‐up in the size of the response followed by a diminution. The flexion reflex can, therefore, be used to investigate sensory processing in the neonate, and the effects of tissue damage. The importance of using natural rather than electrical stimulation is highlighted.
Neuropsychological and behavioral status of children with complex partial seizures
Authors: Schoenfeld Jen , Seidenberg Michael , Woodard Austin , Hecox Kurt , Inglese Chris , Mack Ken , Hermann Bruce
Published: July 2010

Neuropsychological and behavioral status were examined in 57 children aged 7 to 16 years with complex partial seizures (CPS) and compared with 27 sibling control children of the same age. Epilepsy had a significant effect on both cognitive and behavioral adjustment measures. Children with CPS had significant impairment across all seven cognitive domains assessed, reflective of a profile of relatively diffuse and generalized cognitive dysfunction. Age at onset of recurrent seizures was the strongest and most consistent predictor of adequacy of cognitive functioning; earlier age at onset was associated with poorer cognitive status. Children with CPS also had more problems compared with sibling control children on measures of social and school competence and internalizing behavior problems, but not externalizing behaviors. Further, frequency of seizure activity in the past year, rather than age at seizure onset, emerged as the strongest predictor of these behavioral difficulties. These findings are discussed in the context of understanding the impact of CPS on cognition and behavioral adjustment, and identifying the contribution of various aspects of the neurodevelopmental course of CPS to these issues.
Brain activity and language assessment using event‐related potentials: development of a clinical protocol
Authors: Byrne Joseph M , Connolly John F , MacLean Shannon E , Dooley Joseph M , Gordon Kevin E , Beattie Tricia L
Published: July 2010

To test the validity of a new computerized task to assess children's receptive vocabulary, event‐related potentials (ERPs) were recorded from 56 typically developing children ranging in age from 5 to 12 years. This ERP‐computerized vocabulary task does not require a child to give a verbal or motor (i.e. pointing) response. Single pictures, from an existing standardized test of receptive vocabulary, were presented on a computer screen and simultaneously named either correctly (congruent) or incorrectly (incongruent) via a computer. As predicted, the N400 amplitude was found to be significantly higher to the incongruent picture‐word pair (i.e. the child knew it was an incorrect pairing) than to the congruent picture‐word pair (i.e. the child knew it was a correct pairing). This effect was found for each of the four age groups (5 to 6 years, 7 to 8 years, 9 to 10 years, 11 to 12 years). This task accurately estimated current receptive vocabulary in typically developing children. Although still in the development stage, it may eventually serve as an adjunct to a thorough neurological and neurodevelopmental assessment of some children presenting with moderate to severe cerebral palsy.
The diagnosis of autism and Asperger syndrome: findings from a survey of 770 families
Authors: Howlin Patricia , Asgharian Anna
Published: July 2010

As part of a wider survey of parents of children with autistic spectrum disorders in the UK, the diagnostic experiences of 614 parents of children with autism and 156 with Asperger syndrome were compared. Although the ages of the children in the two groups were very similar at the time of the survey, parents of children given a diagnosis of Asperger syndrome had experienced significantly longer delays and greater frustration in obtaining a diagnosis than those with a child with autism. In the‘autism group’the average age when diagnosis was confirmed was around 5.5 years; in the‘Asperger group’it was 11 years. Parents of children with a diagnosis of autism were generally aware of problems in their child's development by 18 months of age; in the Asperger group concerns emerged later, at around 30 months of age. Initial worries in both groups centred around abnormal social development but parents of children with Asperger syndrome were less likely to have noted communication problems. Stereotyped or repetitive behaviours were not prominent in the early years in either group. Despite the problems inherent in data collected by postal survey, many of the findings of this study are supported by other research. The practical implications of delayed diagnosis, especially in the case of more able children with Asperger syndrome are discussed.
Evaluation of the effect of thyroxine supplementation on behavioural outcome in very preterm infants
Authors: Briët Judy M , Van Wassenaer Aleid G , Van Baar Anneloes , Dekker Friedo W , Kok Joke H
Published: July 2010

Two‐hundred infants of <30 weeks gestational age were included in a randomized double‐blind controlled trial to study the effect of thyroxine administration on neurodevelopmental outcome in very preterm children. The infants were given either a fixed dose of thyroxine (8 γ/kg birthweight/day) or placebo for the first 6 weeks of life. This paper evaluates the effect of thyroxine administration on behavioural outcome at the age of 2 years. More externalizing, especially destructive, behaviours were found in the group given thyroxine than in the placebo group. This difference was more pronounced in boys and in children born after 27 weeks’gestation. The thyroxine‐treated children with behavioural problems had lower plasma‐free thyroxine levels than the thyroxine‐treated children without behavioural problems. This finding suggests that the presence of more behavioural problems in the group given thyroxine was not an immediate consequence of the treatment.
Oral‐motor dysfunction in children who fail to thrive: organic or non‐organic?
Authors: Reilly S M , Skuse D H , Wolke D , Stevenson J
Published: July 2010

Forty‐seven children with non‐organic failure to thrive (NOFT) were identified from a whole‐population survey of children's growth and development. A significant proportion (N=17) of these 47 children were found to have oral‐motor dysfunction (OMD) identified using a previously validated assessment tool. NOFT children with OMD and those with normal oral‐motor function (N=30) were compared in order to ascertain whether there were any neurodevelopmental differences which might explain this finding. We hypothesized that children with OMD might have a subtle neurodevelopmental disorder. Few psychosocial variables discriminated the two groups. However, cognitive stimulation within the home and cognitive‐growth fostering during mealtimes was much poorer for children with OMD. Some evidence has suggested that NOFT children with OMD may be‘biologically’more vulnerable from birth. We suggest that the continued use of the term‘non‐organic’to describe failure to thrive in such children is questionable and requires redefining.
Cognitive functioning in Lesch–Nyhan syndrome: a 4‐year follow‐up study
Authors: Matthews Wendy S , Solan Anita , Barabas Gabor , Robey Kenneth
Published: July 2010

Lesch–Nyhan syndrome (LNS) is a rare disorder of metabolism caused by a defective gene on the X chromosome. It is typically characterized by choreoathetosis, hypertonia, hyperreflexia, and self‐mutilation. The present study is a 4‐year follow‐up investigation of the cognitive status of six subjects with a mean age of 17 years 10 months (range 14 years 9 months to 23 years). The Stanford–Binet Intelligence Scale: IV was used. Each of the four domains assessed by this battery (verbal reasoning, abstract/visual reasoning, quantitative, and short‐term memory) was compared with previous findings of the same subjects at their initial test and 2‐year follow up; the aim being to gain further insight into the clinical course of LNS over time. The results suggest that while the subjects generally continued to acquire new information and skills over time, their standardized scores declined, indicating that a plateau was reached in their skill levels relative to their peers, as also seen in other developmental disabilities. Deficits were noted in working memory, particularly on tasks that involve considering multiple features simultaneously.
Epilepsies of neonatal onset: seizure type and evolution
Authors: Watanabe Kazuyoshi , Miura Kiyokuni , Natsume Jun , Hayakawa Fumio , Furune Sunao , Okumura Akihisa
Published: July 2010

Most neonatal seizures are occasional seizures and not true epilepsy. This study investigates seizure types of true neonatal epilepsies and their evolution with development. Seventy‐five children with epilepsies of onset within 1 month of life, who were examined between 1970 and 1995, and whose seizure types could be confirmed with ictal EEG recordings, were studied. The patients were followed up for a minimum of 3 years and the evolution of epileptic syndromes was investigated. Sixty‐three (84%) of 75 patients had partial seizures, while nine had generalized seizures, and only three had both generalized and partial seizures. Twenty‐three of 24 neonates with benign familial or non‐familial neonatal convulsions presented with partial seizures; these syndromes should not necessarily be categorized into generalized epilepsy as they are in the present International Classification. Age‐dependent changes were a common feature of symptomatic neonatal epilepsies. Eighteen (41%) of 44 patients with symptomatic epilepsies of neonatal onset developed West syndrome in infancy. Fifteen (83%) of these 18 patients presented with symptomatic localization‐related epilepsy in the neonatal period. In seven of these 15 patients, West syndrome was followed by localization‐related epilepsy. Symptomatic localization‐related epilepsy with transient West syndrome in infancy is another type of age‐dependent epileptic syndrome.
Respiratory depression in children receiving diazepam for acute seizures: a prospective study
Authors: Norris Elizabeth , Marzouk Omnia , Nunn Anthony , McIntyre John , Choonara Imti
Published: July 2010

The aim of this study was to determine the incidence of respiratory depression following the use of diazepam in children presenting with seizures. All children presenting with seizures to a children's A & E department over a period of 9 months were studied prospectively. Respiratory depression was defined as a fall in respiratory rate or oxygen saturation, or apnoea resulting in ventilation or resuscitation with bag‐and‐mask oxygen. There were 130 patient episodes involving 97 children who received treatment for their seizures before admission and/or in the A & E department. Administration of diazepam resulted in 122 patient episodes. The route of administration was rectal in 91 episodes, intravenous in 12 episodes, and both rectal and intravenous in 19 episodes. Eleven children had respiratory depression in relation to diazepam administration. Eight of these children required ventilation. The overall incidence of respiratory depression following the use of diazepam was 9%. The incidence of respiratory depression following diazepam given intravenously or rectally is high. The use of diazepam as first‐line therapy for children with acute seizures needs to be reviewed.
Status epilepticus‐induced brain damage and opercular syndrome in childhood
Authors: Pascual‐Castroviejo Ignacio , Pascual‐Pascual Samuel I , Peña Walter , Talavera Miguel
Published: July 2010

This study reports on a girl with a permanent cerebral lesion and opercular syndrome after status epilepticus (SE). She had previously been healthy and had her first focal motor seizure at 5 years of age, which was controlled with intravenous phenytoin and rectal diazepam. Twenty‐four hours later, she developed partial SE consisting of right facial twitching and right‐hand clonic movements. These uncontrollable seizures lasted for 5 days, after which the partial SE changed to generalized SE, and the seizures continued for another 5 days. CT performed the day before onset of SE revealed no brain abnormality. Another CT performed a year later disclosed bilateral brain lesions, more severe in the left hemisphere. Follow up at 16 years of age revealed moderate motor sequelae of the right‐hand side of the body, anarthria, difficulty chewing, dysphagia, bilateral facial weakness, and drooling, all of which clinically characterize opercular syndrome. An MRI study performed at 14 years of age showed a cerebral parenchymatous lesion which extended between the parietal cortices of both hemispheres, more severe on the left side, and which crossed the corpus callosum, destroying the posterior‐middle zone. Evidence from the CT indicates that the lesion was not present before onset of SE. It seems likely that the focal SE caused the focal brain damage, but the possibility that the subsequent generalized SE played a role cannot be excluded.
Does abnormal branching of inputs to motor neurones explain abnormal muscle cocontraction in cerebral palsy?
Authors: Gibbs John , Harrison Linda M , Stephens John A , Evans Andrew L
Published: July 2010

The common synaptic drive shared between two groups of motor neurones synchronizes the timing of discharges between the motor‐neurone groups. Recordings were made of motor‐unit discharges during cocontraction of ipsilateral pairs of thumb muscles in eight subjects with cerebral palsy (CP) aged 4 to 13 years and eight neurologically healthy subjects aged 4 to 12 years, and in pairs of lower‐limb muscles in 21 subjects with CP and 21 control subjects, both aged 3 to 15 years. Common synaptic drive, likely to be derived at least partly from activity in branched corticospinal‐tract neurones, produced motor‐unit synchronization between pairs of thumb muscles in control subjects but was absent in all subjects with CP. Motor unit synchronization was not found between lower‐limb antagonist muscles that cocontract abnormally in CP, nor was synchronization present in more widely separated muscle pairs. Therefore, abnormal patterns of muscle activation and more widespread muscle reflex responses do not result from an abnormal distribution of common synaptic drive in CP.
Outcome of bilateral periventricular nodular heterotopia in monozygotic twins with megalencephaly
Authors: Jan Mohammed M S
Published: July 2010

Bilateral periventricular nodular heterotopia (BPNH) is a neuronal migration disorder, characterized by grey‐matter cellular rests in the periventricular regions. Patients frequently present with seizures during childhood or later in life. Monozygotic twin sisters were found to have enlarged head circumferences (>95th centile) during a routine medical visit at 6 months of age. A brain CT scan revealed grey‐matter cellular rests in the subependymal regions extending to the subcortical white matter. In some areas these cell aggregates had nodular margins, consistent with the diagnosis of BPNH. At 6 years of age they are cognitively and neurologically normal. They have not had any seizures and their heads continue to grow along the 95th centile.
Hemispheric specialization using SPECT and stimulation tasks in children with dysphasia and dystrophia
Authors: Chiron C , Pinton F , Masure M C , Duvelleroy‐Hommet C , Leon F , Billard C
Published: July 2010

Developmental dysphasia, a severe childhood learning disorder, is thought to result from problems in hemispheric specialization involving both left and right cerebral hemispheres. Regional cerebral blood flow (rCBF) was measured at rest and during stimulation of both hemispheres independently: dichotic listening for the left, dichaptic palpation for the right. Eight right‐handed boys with expressive dysphasia, aged 8 to 12 years, were investigated using SPECT and compared with eight right‐handed age‐matched boys with Duchenne muscular dystrophy with reading disorders but normal speech. rCBF values at rest were also compared with those of five right‐handed age‐matched normal boys. In the dichotic task, children with dysphasia differed from children with dystrophia by failure to increase rCBF in the left hemisphere, in Broca's area, but rCBF increased in the right hemisphere, in the region homologous to Broca's area. In the dichaptic task, rCBF increased bilaterally for children with dysphasia whereas in children with dystrophia rCBF increased only in the right hemisphere. At rest the physiological asymmetry was reversed in favor of the right hemisphere in all areas except Broca's area. Surprisingly, the same applied at rest and for all areas in children with dystrophia. These results confirm that functional specialization of both hemispheres is impaired in developmental dysphasia. Moreover, they suggest that learning disabilities associated with Duchenne muscular dystrophy could also be related to abnormal hemispheric specialization.
Effects of age on distally and proximally generated drawing movements: a kinematic analysis of school children and adults
Authors: Blank R , Miller V , Von Voß H , Von Kries R
Published: July 2010

The effects of age on basic fine motor functions were assessed using a kinematic analysis of simple repetitive drawing movements in a sample of 53 right‐handed children aged 7 to 14 years 11 months and 16 adults aged 27 to 54 years with no detectable abnormality with neurological examination. Lines, circles, and half‐circles were drawn at a self‐chosen and maximum velocity with a pressure‐sensitive pen on a computer graphics tablet. Patterns generated by finger, wrist, and arm movements, and those generated by combined finger‐wrist movements with and without a change in the drawing direction were analysed. The frequencies of all movement patterns increased until adulthood: 0.1 Hz per year for half‐circles, 0.15 to 0.2 Hz per year for circles and lines generated by arm and wrist movements. The greatest increase (0.35 Hz per year) was observed for lines produced by finger movements. Differences in movement frequencies between finger versus wrist or arm movements when drawing lines decreased from about 3 Hz in 7– to 8‐year‐olds to 0.5 to 1 Hz in adolescents. In adults the difference between maximum finger, wrist, and arm movement frequencies decreased to almost zero. In contrast, the differences in movement patterns with and without change in direction remained stable until adulthood. Based on these results it is hypothesized that the differential effects of finger and more proximally generated movements on age reflect maturation of distinct neuronal sites of the cortico‐striatal‐cerebellar circuit rather than acquisition of motor skills or myelinization of corticospinal pathways.
Parental and professional agreement in developmental assessment of very‐low‐birthweight and term infants
Authors: Heiser Andrea , Curcin Oliver , Luhr Catharina , Grimmer Ingrid , Metze Boris , Obladen Michael
Published: July 2010

The aim of this prospective follow‐up study was to evaluate the accuracy of a parent‐completed questionnaire compared with professionally detected developmental delay. Parents of 108 very‐low‐birthweight (VLBW) infants and parents of 279 term control infants completed the German version of the Revised Prescreening Developmental Questionnaire (R‐PDQ) at the corrected age of 12 months. Simultaneously, infants underwent developmental examination using the Griffiths Developmental Scale. Sixty‐nine VLBW infants were classified as not delayed, 16 as delayed by both methods (conegativity 76% and copositivity 94%), as compared to 240 and six term control infants (conegativity 88%, copositivity 94%). The questionnaire suggested delay in 22 VLBW infants and 32 control infants, which was not substantiated by professional examination (P=0.006). In contrast, examination‐diagnosed delay was missed by the questionnaire in one infant in each group. The R‐PDQ is a reliable monitoring instrument for both VLBW and term infants at the age of 12 months. Parents of VLBW infants tend to underestimate their infants’development.
Biomechanical transformation of the gastroc‐soleus muscle with botulinum toxin A in children with cerebral palsy
Authors: Boyd Roslyn N , Pliatsios Victoria , Starr Roland , Wolfe Rory , Graham H Kerr
Published: July 2010

Objective measures (kinematics and kinetics) were used to study prospectively the effects of botulinum toxin A (BTX/A) on the gastroc‐soleus muscle in ambulant children with cerebral palsy. In this prospective before and after trial, 15 children with diplegia and 10 children with hemiplegia were studied (mean age 5 years 7 months, range 4 years to 9 years). A range of standardized clinical measures was undertaken but the emphasis for this report is on the three‐dimensional gait analysis (3DGA) results. All children showed improvements in sagittal ankle kinematics, as has been previously reported. Two new measures of ankle kinetics were devised: ankle moment quotient (AMQ), and ankle power quotient (APQ). Before intervention, ankle moments were characterized by a‘double bump’ankle moment. A typical abnormal baseline ankle‐power curve was triphasic with an initial trough of absorption followed by abnormal mid‐stance power generation, instead of the usual A1 pattern, and reduced terminal stance power generation (A2). Three weeks after treatment with BTX/A alone there was a statistically significant improvement of AMQ and APQ; some patients required potentiation of BTX/A with a short period of serial casts. Both groups (BTX/A alone and BTX/A plus casting) continued to show improvement in ankle kinetics from baseline after 12 and 24 weeks. This is the first study to demonstrate improvements in the typical abnormal ankle kinetics which we believe provides evidence of the ‘biomechanical transformation of muscle’.
Ankle spasticity and strength in children with spastic diplegic cerebral palsy
Authors: Engsberg Jack R , Ross Sandy A , Olree Kenneth S , Park Tae Sung
Published: July 2010

Ankle spasticity and strength in 27 children with spastic diplegic cerebral palsy (CP) (mean age 9 years, range 3 to 18 years) and a group of 12 children without CP (comparison group) (mean age 9 years, range 5 to 18 years) were observed. To measure spasticity, a KinCom dynamometer dorsiflexed the passive ankle at five different speeds and recorded the resistive plantarflexion torques. Work values for the torque‐angle data were calculated at each speed. Using this data, linear regression was used to measure spasticity. To measure strength, the dynamometer rotated the ankle from maximum dorsiflexion to maximum plantarflexion at a speed of 10°/s while the child performed a maximum plantarflexion concentric contraction. The movement was reversed to record maximum dorsiflexion. Maximum torques and work by the plantarflexors and dorsiflexors were calculated. The group with CP had significantly more spasticity in the plantarflexors and significantly less strength in the plantarflexors and dorsiflexors than the group without CP. Results provide objective information quantifying ankle spasticity and strength in children with CP.
A study of EEG, electroretinogram, visual evoked potential, and eye movements in classical lissencephaly
Authors: Hodgkins P R , Kriss A , Boyd S , Chong K , Thompson D , Taylor D S I , Russell‐Eggitt I , Harris C M
Published: July 2010

EEG, flash electroretinogram (ERG), and visual evoked potential (VEP) findings are described in eight children with classical lissencephaly (six girls, two boys), with a mean age of 17.6 months (range 2 to 60 months). The EEG shows typically high‐voltage activity. Eye movements were formally recorded in two patients, and both showed features associated with oculomotor apraxia. The ERG and VEP to flash stimulation were normal in all cases. Two subjects had pattern reversal stimulation, and their pattern VEPs were within normal limits. Some patients with lissencephaly may appear to have delayed visual maturation on first presentation, and EEG and eye movement studies are valuable in indicating neurological deficiency at an early stage in these subjects.
Perceptions of mainstreaming: a systems approach
Authors: Llewellyn Ann
Published: July 2010

This paper examines the perceptions of mainstreaming among six young people (mean age, 15.5 years; range 13 to 18 years) with physical disabilities, their parents, and seven teaching staff of one‘designated’school in the United Kingdom. The study used semistructured interviews to allow the participants to talk about their experiences of mainstreaming in their own words. The results were then analysed and formed the basis for a multiperspective case study. The analysis revealed that the processes involved that exclude the child with a physical disability from mainstream school life are highlighted. They appear to be subtle and also specific to individual children and families.
Functional outcome at 5 years in children with obstetrical brachial plexus palsy with and without microsurgical reconstruction
Authors: Strömbeck Christina , Krumlinde‐Sundholm Lena , Forssberg Hans
Published: July 2010

Between 1987 and 1998, 470 patients visited the national clinic for obstetrical brachial plexus palsies at the Karolinska Hospital, Stockholm, Sweden. This study reports on 247 children examined at 5 years of age using a special protocol for testing the sensory and motor function of the hand. The children were distributed in various groups depending on the number of injured nerves, whether they had some muscle activity in their biceps or deltoid muscles at 3 months of age, and whether any operations with nerve reconstruction had been performed. The shoulder range of movement in C5–6 palsies was significantly better in the group which had been operated on (operated group), but otherwise there were no differences between children who had been operated on (non‐operated group) and those who had not. A decrease in grip strength and bimanual function in level C5–6 palsies was found, though these roots should not innervate the distal hand. Outcome was not influenced by the number of avulsions in upper‐plexus palsies or whether the operation took place before or after the age of 6 months. The group with extensive lesions (C5‐Th1) had the most root avulsions, showing a correlation between increased avulsions and decreased hand function. This study does not support operating on children with no activity of the biceps and deltoid muscles at 3 months of age, as other authors have concluded. Rather, it favours waiting for a late recovery.
Respiratory sinus arrhythmia during feeding: a measure of vagal regulation of metabolism, ingestion, and digestion in preterm infants
Authors: Suess Patricia E , Alpan Gad , Dulkerian Susan J , Doussard‐Roosevelt Jane , Porges Stephen W , Gewolb Ira H
Published: July 2010

Respiratory sinus arrhythmia (RSA), a non‐invasive indicator of vagal regulation of the heart, and heart period (HP) were monitored before, during, and after oral or gastric‐tube bolus feedings in 32 preterm infants. Group 1 infants (n=15) were ≤30 weeks gestational age (GA) at birth (mean 28.3 weeks) and group 2 infants (n=17) were ≥31 weeks GA at birth (mean=33.2 weeks). Mean postmenstrual ages at the time of study were 33.5 ± 2.3 (SD) weeks in group 1 and 33.9 ± 1.6 (SD) weeks in group 2. RSA and HP decreased in both groups during feeding. However, postfeeding RSA and HP increased toward prefeed levels only for group 2 infants. In addition, RSA and HP changes during feeding were correlated only for group 2 infants. The results suggest that the preterm infant may experience a maturational lag in vagal function and in the influence of vagal activity on metabolic mechanisms (i.e. heart rate) related to ingestive needs. This maturational lag may contribute to continued feeding difficulties and may be a measurable marker of subtle neurodevelopmental problems.
Development of the corticospinal system and hand motor function: central conduction times and motor performance tests
Authors: Fietzek U M , Heinen F , Berweck S , Maute S , Hufschmidt A , Schulte‐Mönting J , Lücking C H , Korinthenberg R
Published: July 2010

Maturation of the corticospinal (CS) tract and hand motor function provide paradigms for central nervous system development. In this study, involving 112 participants (aged from 0.2 to 30 years), we evaluated central motor conduction times (CMCT) obtained with transcranial magnetic stimulation (TMS) during preinnervation conditions of facilitation and relaxation. Auditory reaction time, velocity of a ballistic movement of the arm, finger tapping, diadochokinesis, and fine motor visuomanual tracking were also examined. The maturation profiles for every parameter were calculated. CMCTs for the different preinnervation conditions reached adult values at different times and this could be explained by maturation of excitability at the cortical and spinal level. A stable phase for CMCTs and reaction time was reached during childhood. Parameters which measured motor speed and skill indicated that the development of these continued into adulthood. The maturation of the fast CS tract seems to be completed before the acquisition of the related motor performance has been accomplished. In conclusion, we could demonstrate that data from several neurophysiological methods can be combined and used to study the maturation of the function of the nervous system. This approach could allow appraisal of pathological conditions that show parallels with omissions or lack of developmental progress.
Identification of neonates at risk of developing feeding problems in infancy
Authors: Hawdon J M , Beauregard N , Slattery J , Kennedy G
Published: July 2010

The increased survival of sick and preterm neonates may be associated with long‐term problems which must be recognised and managed if outcome is to be optimised. In a prospective study of 35 neonates (median gestational age at birth 34 weeks) admitted to a neonatal intensive care unit over a 3‐month period, we have documented a high incidence (14 of 35) of immature or abnormal feeding patterns when infants were assessed at 36 to 40 weeks postmenstrual age. Neonates with prolonged respiratory support and delayed enteral and oral feeding were most affected. Compared with neonates who have normal initial feeding assessments, neonates with disorganised or dysfunctional feeding were six times more likely to vomit and three times more likely to cough when offered solid food at 6 months of age. At 12 months of age significant differences were also found in tolerating lumpy food and enjoying mealtimes. We hypothesise that these feeding problems contribute to failure to thrive and psychosocial distress after discharge from the neonatal unit and propose potential neonatal measures to reduce their incidence.
Colpocephaly in two siblings: further evidence of a genetic transmission
Authors: Cerullo A , Marini C , Cevoli S , Carelli V , Montagna P , Tinuper P
Published: July 2010

Colpocephaly is an abnormal congenital enlargement of the occipital horns of the lateral ventricles, which has typically been associated with learning disability, seizures, and motor and visual abnormalities. This study describes familial colpocephaly in two siblings with different fathers in a Dominican family. There were no other neurological problems in their relatives. Child 1 is a 7‐year‐old girl, who has had partial epileptic seizures since 4 years of age. She attends a mainstream school. She has coloboma and chorioretinic atrophy in her right eye and a right microphthalmia. MRI revealed colpocephaly and hypogenesis of the corpus callosum. Her brother, aged 2 years 8 months showed a mild delay in speech acquisition. He has had three febrile convulsions and two non‐febrile tonic‐clonic seizures since 6 months of age. Neurological and ophthalmological examinations were normal. MRI documented colpocephaly with right occipital horn prevalence. There are only two literature reports of genetically transmitted colpocephaly hypothesised in two identical twins and in two brothers. In the children in this study, a genetic basis for colpocephaly is confirmed. We hypothesised a maternal transmission with X‐linked or autosomal dominance with an incomplete penetrance model of inheritance.
Prognosis of ischemic stroke in childhood: a long‐term follow‐up study
Authors: De Schryver Els L L M , Kappelle L Jaap , Jennekens‐Schinkel Aag , Peters A C Boudewyn
Published: July 2010

Little is known about long‐term physical sequelae, cognitive functioning, and quality of life in children who have experienced ischemic stroke. Thirty‐seven patients under 16 years of age were studied; the median interval after stroke was 7 years. CT‐scans were reassessed to determine the type of infarction at baseline. Occurrences of death, of new cardiovascular events, and of seizures during follow‐up were recorded. Surviving patients were invited for a follow‐up examination, including physical check‐up, global screening of cognition, and an inventory of subjective health perception. Only two patients were lost to follow‐up. During follow‐up four died, nine developed seizures, eight had transient ischemic attacks, and two experienced a recurrent ischemic stroke. None of the patients had cardiac complications during follow‐up. In 11 of 27, no functional impairment was found, in 15 there was a hemiparesis of varying severity, and in one a paraplegia. There was a significant shift in cognitive functioning towards lower levels, especially in children with epilepsy. Remedial teaching was frequently needed. Many of the parents’perceived their child's behavior to be very changeable. Three‐quarters of the children considered themselves as healthy as other children, and almost all of them as happy. The physical and functional prognosis after ischemic stroke in childhood is relatively good, particularly in children with no serious causative illness, but special education is often needed and social changes occur.
Relation between visual perceptual impairment and neonatal ultrasound diagnosis of haemorrhagic‐ischaemic brain lesions in 5‐year‐old children
Authors: van den Hout B M , Stiers P , Haers M , van der Schouw Y T , Eken P , Vandenbussche E , van Nieuwenhuizen O , de Vries L S
Published: July 2010

Visual‐perceptual abilities were assessed in 5‐year‐old children with the following neonatal neurological conditions: born preterm with normal ultrasound scan (NL,n=17); born preterm with ultrasound diagnosis of intraventricular haemorrhage (IVH,n=17); born preterm with ultrasound diagnosis of periventricular leukomalacia (PVL,n=12); born term with hypoxic‐ischaemic encephalopathy (HIE,n=11). Visual‐perceptual ability was evaluated with the L94: eight visual‐perceptual tasks designed to evaluate different aspects of visual perception at the preschool level in children with multiple disabilities. Impairment was established in comparison to the performance age obtained on non‐verbal intelligence subtests, instead of chronological age. Frequency of L94 impairment was highest in children with PVL, while children with IVH did not differ from the NL control group. Impairment rates were increased also in children with transient periventricular echodensities, and in children with HIE. Impairments were only moderately related to the delay of visual acuity maturation in infancy.
Reliability of isokinetic strength measurements of the knee in children with cerebral palsy
Authors: Ayalon Moshe , Ben‐Sira David , Hutzler Yeshayahu , Gilad Talila
Published: July 2010

This study evaluates the reliability of isokinetic testing of the knee flexors and extensors in children with cerebral palsy (CP). Twelve children (seven girls, five boys), aged 9 to 15 years, participated in this study. The children's strength was measured bilaterally, using a dynamometer. Each participant was tested twice at an interval of 1 week. During each session, the participant performed five consecutive cycles of knee extension and flexion. Testing velocity was set at 90°/s, and the range of motion was 80°, starting with the knee flexed at 80° and ending in full extension. The measured variable was the peak torque. Reliabilities were determined using intraclass coefficient (ICC) with two‐way ANOVA model. The ICCs for individual sessions range from 0.90 to 0.99. ICCs for the eight repetitions over the two sessions range from 0.95 to 0.98. The findings indicate that measuring isokinetic strength in the tested population is highly reliable and should be considered in rehabilitation protocols.
Cerebral herniation in pyogenic meningitis: prevalence and related dilemmas in emergency room populations in developing countries
Authors: Akpedez George O , Ambe Jose P
Published: July 2010

This study aimed to determine the frequency and outcome of possible cerebral herniation in relation to lumbar puncture (LP) in postneonatal infants and children with pyogenic meningitis in the tropics. Children with meningitis aged between 6 weeks and 15 years (mean age 4.07 years; n=123) were recruited consecutively over 31/2 years at the University of Maiduguri Teaching Hospital, Nigeria. The frequency of possible herniation was determined by clinical evaluation in relation to the severity of illness (high versus low risk) on presentation and performance of LP. Previously described scoring schemes were used. Eighteen (15%) patients had evidence of herniation on presentation. The relative risk (RR) of herniation in high‐ versus low‐risk patients was 66.6 (9.3 to 477.1, 95% CI), p<0.0001 while the RR of death or neurological sequelae in high‐ versus low‐risk patients was 2.6 (1.8 to 3.7, 95% CI), p<0.0001. In 99 patients with known outcomes who had LP on presentation, 21 of 81 without herniation pre‐ or post‐LP, four of four with herniation pre‐and post‐LP, seven of eight with herniation post‐LP only, and five of six with herniation pre‐LP only died or recovered with sequelae (χ2= 25.24, df= 3, p<0.0001). It is concluded that outcome depends on the severity of illness and the presence and timing of herniation. A policy of selective rather routine LPs may improve the outcome in meningitis in developing countries.
Lasting auditory attention impairment after persistent middle ear infections: a dichotic listening study
Authors: Asbjørnsen Arve , Holmefjord Anders , Reisæter Sigvor , Møller Per , Klausen Olav , Prytz Bente , Boliek Carol , Obrzut John E
Published: July 2010

Dichotic listening performance was studied in children who at an early age had undergone a myringotomy with insertion of ventilating tubes for persistent middle ear infections (otitis media with effusion; OME) and compared with age‐equivalent children who had no history of otitis media or hearing problems. The OME group consisted of 19 children with a median age of 9 years; 15 of whom were right‐handed, and 14 were boys. The comparison sample comprised 18 children with a median age was 9 years 4 months. Hand preference and sex were matched with the OME group. Both groups were tested with dichotic listening to consonant‐vowel syllables and additional forced‐attention tasks. The comparison sample showed a weak right‐ear advantage, and age‐adequate attentional modulations. The children in the OME group showed a strong right‐ear advantage, but were not able to modulate the ear advantage during directed‐attention tasks. Models for interpreting the result are discussed.