bluuebunny/crossref_metadata_2025 huggingface dataset:
Immune mediated chorea encephalopathy syndrome in childhood
Authors: Hartley LM , Ng SY , Dale RC , Church AJ , Martinez A , De Sousa C
Published: July 2010

We report four previously healthy female children, aged between 3 and 8 years, who presented with encephalopathy and an extrapyramidal movement disorder (chorea n=4, rigidity n=2, oculogyric crisis n=2). In addition, an acute behavioural disturbance occurred in two patients and mutism in two others. Seizures heralded the onset of the illness in three patients. Acute MRI was either normal or initially normal with later generalized cerebral atrophy. All infective (including streptococcus), biochemical, and metabolic investigations were normal, although all four patients had oligoclonal bands in the (CSF) but not the serum, indicating intrathecal immunoglobulin synthesis. All four children made an apparently full recovery within four months of the onset. We suggest that these patients represent an immune‐mediated movement disorder and encephalopathy syndrome.
Increased gyrification in Williams syndrome: evidence using 3D MRI methods
Authors: Schmitt J Eric , Watts Katie , Eliez Stephan , Bellugi Ursula , Galaburda Albert M , Reiss Allan L
Published: July 2010

Understanding patterns of gyrification in neurogenetic disorders helps to uncover the neurodevelopmental etiology underlying behavioral phenotypes. This is particularly true in Williams syndrome (WS), a condition caused by de novo deletion of approximately 1 to 2Mb in the 7q11.23 region. Individuals with WS characteristically possess an unusual dissociation between deficits in visual‐spatial ability and relative preservations in language, music, and social drive. A preliminary postmortem study reported anomalous gyri and sulci in individuals with WS. The present study examined gyrification patterns in 17 participants with WS (10 females, 7 males; mean age 28 years 11 months, SD 8 years 6 months) and 17 age‐ and sex‐matched typically developing control participants (mean age 29 years 1 month, SD 8 years 1 month) using new automated techniques in MRI. Significantly increased cortical gyrification was found globally with abnormalities being more marked in the right parietal (p=0.0227), right occipital (p=0.0249), and left frontal (p=0.0086) regions. These results suggest that one or more genes in the 7q11.23 region are involved during the critical period when cortical folding occurs, and may be related to the hypothesized dorsal/ventral dissociation in this condition.
Neuroimpairments, activity limitations, and participation restrictions in children with cerebral palsy
Authors: Beckung Eva , Hagberg Gudrun
Published: July 2010

In a representative series of 176 children with cerebral palsy (CP), aged 5 to 8 years, associations were studied between additional neuroimpairments, activity limitations, and participation restrictions in the domains of mobility, education, and social relations as proposed in the International Classification of Functioning Disability and Health (ICF). Learning disability occurred in 40%, epilepsy in 35%, visual impairment in 20%, and infantile hydrocephalus in 9% of the children. Additional neuroimpairments were most frequently seen in children with tetraplegia and dystonic CP and in those with antecedents of brain malformations or severe perinatal compromise. Activity limitations were studied with the Gross Motor Function Classification System (GMFCS) and a system for grading bimanual fine motor function (BFMF) was developed. There was a strong correlation of 0.74 between the GMFCS and BFMF (p<0.001). Learning disability, activity limitations, and participation restrictions were all clinically strongly associated with each other (p<0.001). Restriction in mobility was best predicted by the GMFCS, learning disability, and the BFMF; in education by learning disability and the GFMCS; and in social relations by learning disability, the GMFCS, and BFMF. Motor function and learning disability were important predictors for participation restrictions in children with CP. The ICF has the capacity to be a model to help plan interventions for specific functional goals and to ascertain the child's participation in society.
Disability and quality of life in spina bifida and hydrocephalus
Authors: Pit‐ten Cate Ineke M , Kennedy Colin , Stevenson Jim
Published: July 2010

This study examined the impact of severity and type of condition and family resources on quality of life in children with spina bifida and hydrocephalus. A national UK sample of children aged between 6 and 13 years with spina bifida (n=62), hydrocephalus (n=354), and spina bifida plus hydrocephalus (n=128) were identified via the register of the Association for Spina Bifida and Hydrocephalus (ASBAH). Parents completed standardized measures of Child Health Related Quality Of Life (CQOL), family needs survey (FNS), and caregiving self‐efficacy scale (CSES) as well as questions on children's health and physical ability. Results showed there were no significant differences in the overall quality of life for the three disability conditions. The overall CQOL was over 1 SD lower for those with spina bifida and hydrocephalus than for children with other physical conditions. Sex and age were not related to overall CQOL. Specific aspects of CQOL differentiated the three groups. Children with spina bifida had poorer CQOL scores on self‐care, continence, and mobility/activities whilst those with hydrocephalus had poorer scores on school activities, worries, sight, and communication. Severity of condition and family resources, i.e. CSES and FNS, predicted 32% of the variance in CQOL. Associations were also found between overall CQOL and problems discernible at birth as well as epilepsy. Other factors, including those related to shunts, were not significantly related to CQOL. It was concluded that hydrocephalus is just as great a threat to CQOL as spina bifida. Beyond the general effect of condition severity on CQOL, family resources (as measured by the CSES and FNS) represent an additional influence on CQOL.
Pattern‐reversal visual evoked potentials in infants: gender differences during early visual maturation
Authors: Malcolm CA , McCulloch DL , Shepherd AJ
Published: July 2010

This paper investigates gender differences in the peak latency and amplitude of the P1 component of the pattern‐reversal visual evoked potential (pattern‐reversal VEP) recorded in healthy term infants. Pattern‐reversal VEPs in response to a series of high contrast black and white checks (check widths 120′, 60′, 30′, 24′, 12′, 6′) were recorded in 50 infants (20 males, 30 females) at 50 weeks post‐conceptional age (PCA) and in 49 infants (22 males, 27 females) at 66 weeks PCA. Peak latency of the major component, P1, was considerably shorter in female compared with male infants. Differences in head circumference do not entirely account for the gender differences in peak latency reported here. A gender difference in P1 amplitude was not detected. These findings stress the importance of considering gender norms as well as age‐matched norms when utilizing the pattern‐reversal VEP in clinical investigations. Studies including a wider range of ages are clearly necessary in order to establish whether the earlier peak latencies in female infants represents a difference in the onset or rate of visual maturation.
Threshold electrical stimulation (TES) in ambulant children with CP: a randomized double‐blind placebo‐controlled clinical trial
Authors: Dali Christineí , Hansen Flemming Juul , Pedersen Søren Anker , Skov Liselotte , Hilden Jørgen , Bjørnskov Inge , Strandberg Charlotte , Christensen Jette , Haugsted Ulla , Herbst Grethe , Lyskjær Ulla
Published: July 2010

A randomized double‐blind placebo‐controlled clinical trial was carried out to determine whether a group of stable children with cerebral palsy (36 males, 21 females; mean age 10 years 11 months, range 5 to 18 years) would improve their motor skills after 12 months of threshold electrical stimulation (TES). Two thirds received active and one third received inactive stimulators. For the primary outcome we constructed a set of plausible motor function tests and studied the change in summary indices of the performance measurements. Tests were videotaped and assessed blindly to record qualitative changes that might not be reflected in performance measurements. We also judged range of motion, degree of spasticity, and muscle growth measured by CT. Fifty seven of 82 outpatients who were able to walk at least with a walker, completed all 12 months of treatment (hemiplegia n=25, diplegia n=32). There was no significant difference between active and placebo treatment in any of the tested groups, nor combined. Visual and subjective assessments favoured TES (ns), whereas objective indices showed the opposite trend. We conclude that TES in these patients did not have any significant clinical effect during the test period.
Infant sucking ability, non‐organic failure to thrive, maternal characteristics, and feeding practices: a prospective cohort study
Authors: Ramsay Maria , Gisel Erika G , McCusker Jane , PhD François Bellavance , PhD Robert Platt
Published: July 2010

This prospective study examined the relation of neonatal sucking to later feeding, postnatal growth, maternal postpartum depression, and feeding practices. Healthy infants of at least 37 weeks gestational age were recruited. At 1 week of age, a strain‐gage device was attached to the infant's cheeks during sucking to identify sucking efficiency. Two‐hundred and two infants (100 males, 102 females; mean age 39.6 weeks, SD 1.1 weeks) with efficient sucking and 207 (101 males, 106 females; mean gestational age 39.4 weeks, SD 1.2 weeks) with inefficient sucking were identified. Growth was measured at 2, 6, 10, and 14 months. Mothers completed a feeding questionnaire and the Edinburgh Postnatal Depression Scale at the same testing points. While 18 infants (5%) showed a downward shift in growth, their clinical picture did not present as non‐organic failure to thrive (NFTT). Inefficient neonatal sucking did not predict postnatal growth, later feeding difficulties, nor maternal feeding practices, but concurrent inefficient feeding did. Maternal depression did not affect feeding practices, infant feeding abilities, nor growth, suggesting that the importance of maternal postpartum depression in association with feeding may be less than previously assumed. The term NFTT, therefore, merits reexamination and a more focused definition.
Qualitative analysis of therapeutic motor intervention programmes for children with cerebral palsy: an update
Authors: Siebes Renate C , Wijnroks Lex , Vermeer Adri
Published: July 2010

This study presents a comprehensive evaluation of therapeutic motor interventions for children with or at risk for cerebral palsy (CP), examining 50 studies covering the period from 1990 to 2001. The purpose was to review noticeable changes in the scientific quality of the studies and to highlight trends and changes in the nature of the evaluated programmes. Our results were compared with those of Vermeer and Bakx (1990) who reviewed the period from 1980 to 1989. It was concluded that fundamental research with adequate methodology was applied more often in the study period than in the period reviewed by Vermeer and Bakx. However, these developments did not lead to a substantial improvement in the scientific foundation of the interventions under study. Single case studies, combined with efforts to develop measures specifically for children with CP and with high sensitivity, might make more valuable contributions to the scientific justification of therapeutic interventions.
Physical capacity in non‐ambulatory people with Duchenne muscular dystrophy or spinal muscular atrophy: a longitudinal study
Authors: Steffensen Birgit F , Lyager Søren , Werge Birgit , Rahbek Jes , Mattsson Eva
Published: July 2010

The purpose of this study was to describe functional ability, muscle strength, forced vital capacity, and clinical events in participants with Duchenne muscular dystrophy (DMD) or spinal muscular atrophy (SMA) in the non‐ambulatory stages of the diseases. Nineteen non‐ambulatory participants with DMD (all males; 13 to 24 years) and 13 with SMA (six males, seven females; 11 to 57 years) were assessed once a year over 5 years. The assessments comprised functional ability measured with the EK scale and upper extremity grade, muscle strength measured with the manual muscle test, and forced vital capacity defined as a percentage of normal values (FVC%). In the DMD group all variables measured deteriorated and there was a direct correlation between them. In the SMA group only muscle strength and FVC% deteriorated and there was no close relation between the variables measured. In the DMD group, 16 participants had cardiorespiratory clinical events leading to death in five cases. In the SMA group only four participants had respiratory clinical events, none leading to death. Although the participants with SMA had been extremely weak and non‐ambulatory since early childhood they were older and less exposed to life‐threatening events than the participants with DMD.
Intracranial hypertension in neuroborreliosis
Authors: Härtel Christoph , Schilling Stefan , Neppert Birte , Tiemer Bettina , Sperner Jürgen
Published: July 2010

Neuroborreliosis is an infection of the nervous system caused by the spirochete Borrelia burgdorferi, from which patients most commonly develop lymphocytic meningitis, radiculoneuritis, or cranial neuropathy. In this report a 9‐year‐old male with an unusual neurological complication of neuroborreliosis ‐ benign intracranial hypertension (BIH) ‐is described. Clinical symptoms of BIH, which consist of increased CSF pressure in the absence of an intracranial mass or obstruction to the circulation of CSF, resolved completely after antibiotic therapy with ceftriaxone.
Adults with cerebral palsy: walking ability after progressive strength training
Authors: Andersson C , Grooten W , Hellsten M , Kaping K , Mattsson E
Published: July 2010

The purpose of this study was to evaluate effects of a progressive strength training programme on walking ability in adults with cerebral palsy. Ten individuals with spastic diplegia (seven males, three females; mean age 31, range 23–44 years) participated twice a week over 10 weeks. Seven individuals with spastic diplegia (four males, three females; mean age 33, range 25–47 years) who did not receive strength training served as controls. All individuals were ambulatory but motor ability ranged from functional walkers to individuals who always required walking aids and used a wheelchair regularly. Significant improvements were seen in isometric strength (hip extensorsp=0.006, hip abductorsp=0.01), and in isokinetic concentric work at 30/s (knee extensorsp=0.02) but not in eccentric work. Results also showed significant improvements in Gross Motor Function Measure (GMFM) dimensions D and E (p=0.005), walking velocity (p=0.005), and Timed Up and Go (p=0.01). There was no increase in spasticity for those who underwent strength training. Individuals in the control group did not show any significant improvement in any measured variable. The groups were small, however, and there was no significant difference between the groups in any measured variable. These findings suggest that a 10‐week progressive strength training programme improves muscle strength and walking ability without increasing spasticity.
Ischaemic stroke subtypes in children and adults
Authors: Wraige E , Hajat C , Jan W , Pohl K R E , Wolfe C D A , Ganesan V
Published: July 2010

Ischaemic stroke subtypes in children and adults were compared to determine the similarity in aetiologies. Thirty‐six children (22 females, 14 males; median age 5 years 7 months, range 6 weeks to 15 years 10 months) and 50 adults (35 males, 15 females; median age 44 years, range 17 years 2 months to 49 years 11 months) who had presented with ischaemic stroke between 1995 and 2000, were categorized using a modified version of the Trial of Org 10172 in Acute Stroke Therapy (TOAST) classification. Proportions of patients in the subtypes of the TOAST classification system were significantly different in the two groups (χ2 test, p<0.01). The first three subtypes (large artery atherosclerosis, cardioembolic, and small vessel disease) accounted for the majority of adult strokes (27 of 50). In contrast, only three of 36 children were accounted for within these three subtypes. The majority of children (29 of 36) were classified within the‘other determined aetiology’subtype. Aetiology was undetermined in 12 of 50 adults compared with three of 36 children. Causes of ischaemic stroke in children and adults are distinct. A classification system for ischaemic stroke in children would be useful for collaborative studies.
Grip force parameters in precision grip of individuals with myelomeningocele
Authors: Gölge M , Schütz C , Dreesmann M , Kuhtz‐Buschbeck J P , Hoppe B , Wenzelburger R , Stephani U , Illert M
Published: July 2010

The aim of this study was to characterize impairments of hand function in individuals with myelomeningocele (MMC) using a quantitative method. A grip‐lift task was studied in 29 individuals with MMC (17 females, 12 males; mean age 12 years 4 months, SD 5 years 6 months; range 4 to 28 years) and 29 age‐ and sex‐matched control participants. A small object (weight 200g) was grasped and lifted with a precision grip of the dominant hand and grip forces (GF) and time intervals were measured. Sensibility was examined with a two‐point discrimination test. In those with MMC, the latency between GF onset of the thumb and index finger and ensuing preload duration were both significantly prolonged (p<0.01). Fingertip forces were excessively high and variable. Several parameters differed significantly between those with MMC and control individuals: GF peak, GF at start of loading, mean GF, and the SDs of the GF during static holding. Although the two‐point discrimination task indicated deficits of sensibility, these did not correlate with grip force parameters. Results demonstrate that fine motor skills in patients with MMC are impeded by slowness and inadequate adjustments of manipulative forces.
Neurocognitive functioning in children with type‐1 diabetes with and without episodes of severe hypoglycaemia
Authors: Hannonen Riitta , Tupola Sarimari , Ahonen Timo , Riikonen Raili
Published: July 2010

Previous studies have shown that recurrent severe hypoglycaemia can cause long‐term cognitive impairment in children with type‐1 diabetes, but the results are controversial, possibly due to the heterogeneity of samples and lack of comprehensive neuropsychological assessments of children. The aim of this study was to assess the effects of diabetes and severe hypoglycaemia on the neurocognitive functioning of children with a standardized, wide age‐range neuropsychological test battery designed for the assessment of children. Eleven children with diabetes and a history of severe hypoglycaemia, 10 children with diabetes without a history of severe hypoglycaemia, and 10 healthy control children (a total of 31 children: 14 males and 17 females, age range 5 years 6 months to 11 years 11 months, mean 9 years 4 months, SD 1 year 11 months) were studied using the Wechsler Intelligence Scale for Children‐Revised (WISC‐R) and the NEPSY, a Developmental Neuropsychological Assessment. The NEPSY assessed development in attention and executive functions, language, sensorimotor functions, visuospatial processing, and learning and memory. Children with a history of severe hypoglycaemia had more neuropsychological impairments, more learning difficulties (as reported by parents), and needed more part‐time special education than those in the other groups. Significant differences were found in verbal short‐term memory and phonological processing. Results suggest that severe hypoglycaemia is a risk factor for learning due to deficits in auditory‐verbal functioning.
Childhood medulloblastoma in northwest England 1954 to 1997: incidence and survival
Authors: Alston R. D. , Newton R , Kelsey A , Newbould M. J. , Birch J M , Lawson B , McNally RJQ
Published: July 2010

The incidence, survival patterns, and presenting symptoms of children with medulloblastoma were studied. Data were ascertained from the Manchester Tumour Registry which is population‐based and has collected data on all childhood malignancies in northwest England since 1954. Incidence rates standardized to the European standard population were calculated and Poisson regression models were used to examine temporal changes in the incidence rates during the period 1954 to 1997. Kaplan‐Meier survival curves were derived and used to study changes in survival patterns. World‐standardized incidence rates were 5.5 per million child years in males and 3.4 per million child years in females. Incidence rates increased from the 1950s to the 1980s but have declined recently. The 5‐year survival rate has improved from 29 to 58% with similar rates for males and females. The 1‐year survival rate has also improved, but females had worse survival at this point (58%) than males (75%). The type of symptom or sign at presentation is strongly affected by age, with 10 of the 22 recorded symptoms or signs showing significant age differences. The older the child is, the more likely is the presentation to show pressure features of headache, vomiting, and ophthalmic signs. Younger children present with non‐specific features such as lethargy, behavioural disturbance, or increasing head size. Ataxia is seen in about 75% of children across the age range.
Integration of suck and swallow rhythms during feeding in preterm infants with and without bronchopulmonary dysplasia
Authors: Gewolb Ira H , Bosnia James F , Reynolds Eric W , Vice Frank L
Published: July 2010

The aim of this study was to define quantitative measures for assessing the integration and maturation of suck and swallow rhythms in preterm infants as they relate to each other. Fourteen preterm infants (eight males, six females; gestational age range 26 to 32 weeks) with bronchopulmonary dysplasia (BPD) and an age‐matched cohort of 20 infants (10 males, 10 females; gestational age range 26 to 33 weeks) without BPD were studied weekly from time of initiation of oral feeding using simultaneous recordings of nipple and pharyngeal pressure. The integration of suck and swallow rhythms was quantified by using the coefficient of variation (COV) of the suck—swallow dyad interval. Infants without BPD had a significant correlation between increasing postmenstrual age (PMA) and decreasing COV of the dyadic interval (increasing stabilization; r=0.45; P=0.008). In the non‐BPD cohort 35 weeks or less PMA, the mean dyadic COV was 0.42 (SD 0.12) versus 0.34 (SD 0.09) in those more than 35 weeks PMA (P=0.039). In contrast, dyadic stability in infants with BPD was not correlated with PMA. Infants with BPD of more than 35 weeks PMA had less dyadic stability (0.45, SD 0.10) than did age‐matched controls (P < 0.001). Dyadic stability was also correlated with feeding efficiency in the non‐BPD group (r=0.46; P=0.007) but not in the BPD cohort. Therefore, ontogeny of rhythmic suckle feeding can be described quantitatively in preterm infants, allowing comparison with at‐risk populations. Infants with BPD do not follow predicted maturational patterns of suck‐swallow rhythmic integration.
Electrical stimulation of gluteus maximus in children with cerebral palsy: effects on gait characteristics and muscle strength
Authors: Van Der Linden ML , Hazlewood ME , Aitchison AM , Hillman S J , Robb JE
Published: July 2010

The purpose of this study was to determine whether electrical stimulation of the gluteus maximus would improve hip extensor strength, decrease excessive passive and dynamic internal hip rotation, and improve gross motor function in children with cerebral palsy (CP). Twenty‐two ambulant children (15 females, 7 males, mean age 8 years 6 months, SD 2 years 9 months, aged 5 to 14 years) with diplegic (n=14), hemiplegic (n=7), and quadriplegic (n=1) CP participated in this study. All were randomly assigned to either the stimulation or control group. The stimulation group (n=11) received electrical stimulation of the gluteus maximus of the most affected legs for 1 hour a day, 6 days a week for a period of 8 weeks. Electrodes were applied proximally and distally over the gluteus maximus, with the active electrode initially positioned over the motor points. The control group (n=11) did not receive any extra treatment. Measurements of hip extensor strength, gait analysis, passive limits of hip rotation, and section E of the Gross Motor Function Measure were made before and after treatment for both groups. Subjectively, 7 of the 11 parents thought that the treatment made a difference to their child. However, no statistically or clinically significant improvement was found in the stimulation group when compared with the control group.
Reliability and validity of the Observational Gait Scale in children with spastic diplegia
Authors: Mackey Anna H , Lobb Glenis L , Walt Sharon E , Stott N Susan
Published: July 2010

The aim of this study was to establish the reliability and validity of visual gait assessment in children with spastic diplegia, who were community or household ambulators, using a modified version of the Physicians Rating Scale, known as the Observational Gait Scale (OGS). Two clinicians viewed edited split‐screen video recordings of 20 children/adolescents (11 males, 9 females; mean age 12 years, range 6 to 21 years) made at the time of three‐dimensional gait analysis (3‐DGA). Walking ability in each child was scored at initial assessment and reassessed from the same videos three months later using the first seven sections of the OGS. Validity of the OGS score was determined by comparison with 3‐DGA. The OGS was found to have acceptable interrater and intrarater reliability for knee and foot position in mid‐stance, initial foot contact, and heel rise with weighted kappas (wk) ranging from 0.53 to 0.91 (intrarater) and 0.43 to 0.86 (interrater). Comparison with 3‐DGA suggests that these sections might also have high validity(wk range 0.38–0.94). Base of support and hind foot position had lower interrater and intrarater reliabilities (wk 0.29 to 0.71 and wk 0.30 to 0.78 respectively) and were not easily validated by 3‐DGA.
Speech, language, and cognitive development in children with isolated sagittal synostosis
Authors: Shipster Caroleen , Stackhouse Joy , Wade Angie
Published: July 2010

This study investigated the occurrence, nature, and severity of speech, language, and cognitive impairment in 76 children (61 males, 15 females) with isolated sagittal synostosis (ISS) aged 9 months to 15 years 7 months. There was no increased prevalence of global cognitive impairment in the group but there was a high prevalence rate of speech and/or language impairment with 28 (37%) displaying impairment of whom 20 (71%) had moderate or severe impairments that fulfilled the criteria for specific impairments. Prevalence rates were only increased for children over two years of age. Expressive language impairment occurred most frequently. Raised intracranial pressure, perineonatal risk factors, otitis media, or being operated were not associated with impairment. Surgery at a later age and a family history of speech and language impairment were both associated with impairments but numbers were small. The findings suggest that children with ISS are at an increased risk of developing speech and language impairment.
Behavioural problems in children and adolescents with spinal muscular atrophy and their siblings
Authors: Laufersweiler‐Plass C , Rudnik‐Schöneborn S , Zerres K , Backes M , Lehmkuhl G , von Gontard A
Published: July 2010

Spinal muscular atrophy (SMA) is a chronic illness characterized by loss of motor function. The aim of the study was to investigate behavioural adjustment in 96 children and adolescents with SMA (47 males, 49 females; mean age 11 years 2 months, range 6 to 18 years). Forty‐five non‐affected siblings (26 males, 19 females; mean age 11 years 6 months, range 6 to 18 years) and 59 normally developing children (33 males, 26 females; mean age 10 years 8 months, range 6 to 18 years) were recruited as control participants. Behavioural symptoms were measured with the Child Behaviour Checklist (CBCL) and disorders were assessed with a structured psychiatric interview (Kinder‐DIPS). Of the patients with SMA, 12.5% fulfilled the criteria for an ICD‐10 or DSM‐IV diagnosis, with separation anxiety disorder being the most common diagnosis. The CBCL total score was in the clinical range for 11.5% of patients, 20% of the siblings, and 11.7% of the control children; the externalizing score rates were 2.1%, 22.2%, and 11.9% respectively; the internalizing score 18.9%, 24.4%, and 13.6% respectively. Comorbid psychopathology was not influenced by sex, IQ, nor severity of SMA, and only externalizing behaviour was correlated to age. In conclusion, children and adolescents with SMA are characterized by a low psychiatric comorbidity not different from control individuals. The group with the highest rate of behavioural problems and with the greatest need for intervention were the non‐affected siblings who had a two‐ to threefold higher rate of behavioural problems than the normative population.
A randomized clinical trial of strength training in young people with cerebral palsy
Authors: Dodd Karen J , Taylor Nicholas F , Graham H Kerr
Published: July 2010

This randomized clinical trial evaluated the effects of a home‐based, six‐week strength‐training programme on lower limb strength and physical activity of 21 young people (11 females, 10 males; mean age 13 years 1 month, SD 3 years 1 month; range 8 to 18 years) with spastic diplegic cerebral palsy (CP) with independent ambulation, with or without gait aids; (Gross Motor Function Classification System levels I to III). Compared with the 10 controls, the 11 participants in the strength‐training programme increased their lower limb strength (combined ankle plantarflexor and knee extensor strength as measured by a hand‐held dynamometer) at 6 weeks (F(1,19)=4.58, p=0.046) and at a follow‐up 12 weeks later (F(1,18)=6.25, p=0.041). At 6 weeks, trends were also evident for improved scores in Gross Motor Function Measure dimensions D and E for standing, running and jumping, and faster stair climbing. A relatively short clinically feasible home‐based training programme can lead to lasting changes in the strength of key lower‐limb muscles that may impact on the daily function of young people with CP.
Clinical utility of the Duncan‐Ely test for rectus femoris dysfunction during the swing phase of gait
Authors: Marks M C , BS J Alexander , Chambers HG
Published: July 2010

The Ely Test (or Duncan‐Ely test) has been accepted as a clinical tool to assess rectus femoris spasticity by passively flexing the knee rapidly while the patient lies prone in a relaxed state. In this retrospective review, patients’dynamic knee range of motion (ROM) during gait and an electromyogram (EMG) were compared with the results of the Ely test. Data for 70 patients (44 males, 26 females; 104 limbs) were included. Mean age of patients was 13 years, SD 9 years, range 4 years 5 months to 54 years. All patients were diagnosed with cerebral palsy (spastic diplegia, n=42; spastic quadriplegia, n=15, and hemiplegia, n=13). All patients were ambulatory (50 independent, 20 with assistive devices). A standard matrix was used to calculate sensitivity and specificity of the Ely test as well as its positive and negative predictive value. For the gait variables examined (decreased dynamic knee ROM, timing of peak knee flexion, and abnormal EMG in swing) the sensitivity of the Ely test ranged from 56 to 59% and the specificity ranged from 64 to 85%. For the same variables the positive predictive value ranged from 91 to 98% and the negative predictive value ranged from 4 to 19%. The Ely test was shown to have a good positive predictive value (i.e. the certainty about the presence of rectus spasticity in patients with a positive Ely test result) for rectus femoris dysfunction during gait.
Pyridoxine‐dependent seizures and cognition in adulthood
Authors: Baynes Kathleen , Tomaszewski Sarah , Gospe Sidney M
Published: July 2010

A case report of neonatal onset pyridoxine‐dependent seizures in a male patient with early diagnosis and treatment is presented. The patient's epilepsy was recognized and treated with pyridoxine (vitamin B6) within 8 hours of birth. Treatment has been nearly continuous since that time. This paper reports the results of a full neuropsychological evaluation at age 37 years and MRI completed at age 31 years. Consistent with other case reports in the literature, there was a significant Performance IQ (PIQ) advantage with decreased Verbal IQ (VIQ) and expressive language skills (Full‐Scale IQ 71, VIQ 64, PIQ 85). MRI demonstrated characteristic thinning of the posterior corpus callosum. This report provides an example of early treatment that nonetheless results in a mild mental retardation. The similarity of the structural changes on MRI and the cognitive profile of this patient to those of others reported in the literature suggest that the underlying mechanism for both may be the same.
Melbourne Assessment of Unilateral Upper Limb Function: construct validity and correlation with the Pediatric Evaluation of Disability Inventory
Authors: Bourke‐Taylor Helen
Published: July 2010

The Melbourne Assessment of Unilateral Upper Limb Function (Melbourne Assessment) is an evaluation tool that objectively measures upper‐extremity function in children with cerebral palsy (CP). This study investigates how well performance on the Melbourne Assessment relates to the child's actual performance in functional tasks. Eighteen children with CP (5 to 14 years of age; nine males, nine females) were assessed using the Melbourne Assessment and the Pediatric Evaluation of Disability Inventory (PEDI). Five children had spastic quadriplegia, eight had spastic diplegia, two had spastic hemiplegia and diplegia, two had athetosis, and one had hypotonic quadriplegia with mobile ventilator dependence. Children's performances were statistically correlated using Spearman's rho to establish the relation between these tools. Very high correlation coefficients were calculated between the Melbourne Assessment and self‐care (0.939) and mobility domains (0.783) of the PEDI and the overall functional skills section of the PEDI (0.718). The Melbourne Assessment demonstrates excellent construct validity for upper limb functioning.
Perinatal morbidity, mild motor delay, and later school outcomes
Authors: Sullivan Mary C , McGrath Margaret M
Published: July 2010

Four dimensions of motor competence were evaluated in 4‐year‐old term and preterm children and were related to academic achievement and use of school services at age 8 years. The objective of the study was to evaluate a graduated ‘stair‐step’ effect between perinatal morbidity, mild motor delay, and later school outcomes in 168 children (88 females, 80 males) stratified into four study groups: 134 healthy term infants ± 37 weeks’gestation, birth weight ± 2500g); 134 preterm infants ±37 weeks’gestation divided into healthy preterm (n=41), clinically ill preterms (n=59), and preterm infants with neurological illness (n=34). Significant differences were found in total, fine and gross motor performance, and visual‐motor integration at age 4 years. Scores for the preterm groups decreased with increasing morbidity. At age 4 years, mild motor delay was found in all preterm groups. Children with mild motor delay had lower academic achievement scores (Wide Range Achievement Test‐3) and higher rates of school service use at age 8 years. Perinatal morbidity and compromised motor performance are important precursors of educational underachievement.
Effect of environmental setting on mobility methods of children with cerebral palsy
Authors: Palisano Robert J , Tieman Beth L , Walter Stephen D , Bartlett Doreen J , Rosenbaum Peter L , MSc Diane Russell , Hanna Steven E
Published: July 2010

The aim of this study was to: (1) describe the usual mobility methods of children with cerebral palsy (CP) at home, school, and outdoors or in the community and (2) examine whether children with CP are more dependent on adult assistance for mobility in certain settings. The participants were a stratified random sample of 636 children with CP (355 males and 281 females; 2 to 12 years of age, mean 6.8 years SD 2.7), receiving rehabilitation services in Ontario, Canada. Children were grouped by age and Gross Motor Function Classification System (GMFCS) level. Among the five levels of the GMFCS, there were 185 children classified at level I, 81 children at level II, 113 children at level III, 132 children at level IV, and 125 children at level V. Information on children's usual mobility was obtained by parent report. The results of logistic regression indicated that compared with the school setting, children were more dependent on adult assistance for mobility when outdoors/in the community and less dependent at home. The majority of children aged from 4 to 12 years at levels III to V used wheelchair mobility at school and outdoors or in the community, however, only a small percentage self‐propelled their wheelchair or used powered mobility. Of the children aged 4 to 12 years at level V, 39% were carried at home. The findings suggest that environmental setting is an important consideration for assessment and intervention to improve mobility of children with CP. For children who do not walk, attention should be given to the needs of caregivers and factors that are important for successful powered mobility.
Maternal fever at birth and non‐verbal intelligence at age 9 years in preterm infants
Authors: Dammann Olaf , Drescher Johannes , Veelken Norbert
Published: July 2010

To test the hypothesis that characteristics of perinatal infection are associated with long‐term cognitive limitations among preterm infants, we analyzed data from 294 infants (142 females, 152 males) ≤1500g birthweight and <37 completed weeks of gestation who were examined at age 9 years. We identified 47 children (20 females, 27 males) who had a non‐verbal Kaufman Assessment Battery for Children (K‐ABC) scale standard value below 70, i.e. more than 2 SDs below the age‐adjusted mean. The 247 children (122 females, 125 males) with a score ≥70 served as control participants. Maternal nationality and education, and low gestational age were significantly associated with a K‐ABC non‐verbal standard value <70. Both neonatal brain damage (intraventricular hemorrhage) and long‐term sequelae (cerebral palsy [CP], diagnosed at age 6 years) were significantly associated with a below‐normal non‐verbal K‐ABC score. Maternal fever at birth was present in five cases (11%) and eight controls (3%; odds ratio 3.6, 95% confidence interval 1.1 to 11.4). Clinical chorioamnionitis and preterm labor and/or premature rupture of membranes (as opposed to toxemia and other initiators of preterm delivery) were also more common among cases than control participants. When adjusting for potential confounders such as gestational age, maternal education and nationality, and CP, the risk estimate for maternal fever remained unchanged (3.8, 0.97 to 14.6). We conclude that perinatal infection might indeed contribute to an increased risk for long‐term cognitive deficits in preterm infants.
Health status of Australian children with mild to severe cerebral palsy: cross‐sectional survey using the Child Health Questionnaire
Authors: Wake M , BA L Salmon , Reddihough Dinah
Published: July 2010

The aim of this study was to describe child health and well‐being across the spectrum of cerebral palsy (CP) and to report on psychometric properties of the Child Health Questionnaire (CHQ) in this population. Parents of 80 individuals (45 males, 35 females; mean age 11 years 4 months, SD 3 years 6 months; range 5 to 18 years) attending a CP clinic in Victoria, Australia were surveyed. Measures included the CHQ PF‐50, a 50‐item parent‐reported heath status measure; severity of CP, based on the Gross Motor Function Classification System; and cognitive status and presence of epilepsy. CHQ data were compared with normative data collected in Victoria two years earlier. The CHQ demonstrated good psychometric properties for children with CP. Children with CP had markedly poorer health on every CHQ scale than those in the normative sample. Health status did not vary by cognitive status or epilepsy. Children with severe CP had the poorest physical health, but psychosocial health and emotional impact on parents were similar for mild and severe CP showing that these should not be assumed to be less prevalent when CP is mild.
Cervical accelerometry in preterm infants with and without bronchopulmonary dysplasia
Authors: Reynolds Eric W , Vice Frank L , Gewolb Ira H
Published: July 2010

Cervical accelerometry with digital signal processing (DSP) can identify signals that are consistently associated with swallowing during feeding of infants. It is shown that these signals, called initial discrete sounds (IDSs), become more uniform with advancing postmenstrual age (PMA) in healthy preterm infants. The objectives of this study were to determine if cervical accelerometry and DSP at a higher sample rate than previously used (22kHz versus 16kHz), in conjunction with a DSP software package that allows manipulation of the sound files, would improve the accuracy of the previously developed variance index (VI) method of analyzing accelerometric information. The modified VI method was then used to measure developmental differences in the IDS morphology of infants with and without bronchopulmonary dysplasia (BPD). VIs were calculated for 24 feeding studies of infants of between 32 and 39 weeks PMA: 12 studies on healthy preterm infants (n=10: three males, seven females; mean gestational age [GA] 28.6 weeks, SD 0.4; mean birth weight [BW] 1080g, SD 82; PMA mean 35.2 weeks, SD 0.6) and 12 studies on infants with BPD (n=7: five males, two females; GA 27.1 weeks, SD 0.4; BW 911g, SD 71; PMA 36.2 weeks, SD 0.6). There was a significant inverse correlation between VI and PMA for the healthy preterm group (r=0.66, m=‐2.31/week, p=0.02). There was no significant correlation between VI and PMA for the BPD cohort. The VI of infants with BPD was significantly different from that of infants without BPD (p< 0.007, multiple regression analysis, interaction PMA×Group). Additionally, using our modified VI technique, 100% of swallows were found to have the expected IDS signals.
Profile of childhood epilepsy in Bangladesh
Authors: Banu Selina H , Khan Naila Z , Hossain Mahmuda , Jahan Anisa , MSc Monwara Parveen , MSc Narsis Rahman , Boyd Stewart H , FRCP Brian Neville
Published: July 2010

Very little is known about childhood epilepsies in Bangladesh. This study was conducted within a national children's hospital in Dhaka city to provide baseline information on diagnosis and clinical outcomes of 151 children (98 males, 53 females, age range between 2 months to 15 years, median age of 3 years). Participants who presented with recurrent unprovoked seizures were followed up in an epilepsy clinic for at least 1 year. Of presenting families, 68.3% were from middle‐income and lower‐income groups. A history of perinatal asphyxia and neonatal seizures was present in 46.4% and 41.1% of participants respectively. Generalized, partial, and unclassifiable epilepsy were found in 63.6%, 25.2%, and 11.2% respectively. Severe outcome (malignant) epilepsy syndromes were diagnosed in 14.6%. Symptomatic epilepsy was found in 61%. Poor cognitive development was present in 72.8% and poor adaptive behaviour in 57%. Poor seizure remission occurred in 50.3%. Factors most predictive of poor seizure remission were: multiple types of seizures, poor cognition at presentation, high rates of seizures, associated motor disability, and EEG abnormalities. The study suggests that most children presenting at tertiary hospitals for seizure disorders come late and with associated neurodevelopmental morbidities. Specialized services are needed closer to their homes. The process for establishing early referral and comprehensive management of childhood epilepsies in Bangladesh requires further study.
Clinical characteristics of language regression in children
Authors: Wilson Sy , Djukic Aleksandra , Shinnar Shlomo , Dharmani Charles , Rapin Isabelle , Korey Saul R
Published: July 2010

The spectrum of language regression in childhood is incompletely understood. To describe the features of this disorder more fully, we reviewed the records of 196 consecutive children (143 males and 53 females) with language regression or perceived plateau evaluated between 1988 and 1994 by a child neurologist. Mean age at regression was 21.2 months and the mean interval to referral was 34.8 months. A trigger for the regression was identified in 74 of the children (38%) and was associated with a more rapid regression. Mean age at follow‐up was 64 months (SD 55). Seventy per cent of the children became nonverbal, and 75% were cognitively impaired. Language regression was associated with a more global autistic regression in 93% of children. There was a history of seizures in 15% of the children. Some recovery occurred in 61% but only one child recovered fully. Improvement was more likely in the 49% who were entirely developmentally normal before the regression. We conclude that language regression in childhood is a serious disorder with significant long‐term morbidity.
Autism, primary pragmatic difficulties, and specific language impairment: can we distinguish them using psycholinguistic markers?
Authors: Botting Nicola , Conti‐Ramsden Gina
Published: July 2010

Three groups of children with communication disorders were examined using a series of psycholinguistic markers to explore whether the tasks could identify children with impairments other than specific language impairment (SLI), and to examine whether the different groups within this clinical population could be distinguished reliably from one another. The groups comprised children with autistic spectrum disorders (ASD; n=13, all males; mean age 10 years 10 months, range 10 years 2 months to 12 years 6 months); children with primary pragmatic language impairment (PLI) but who did not have definite ASD diagnoses (n=25, 22 males, three females; mean age 11 years 3 months, range 10 years 2 months to 12 years 5 months); and children with specific language impairment (SLI) without marked pragmatic language difficulties (n=29, 25 males, 4 females; mean age 10 years 10 months, range 10 years 2 months to 11 years 9 months). Clinical markers examined were: the Children's Non‐Word Repetition (CNRep), the Past Tense Task (PTT), and the Clinical Evaluation of Language Fundamentals, Recalling Sentences. First, it was found that the a priori groupings were not sufficiently defined and that four groups were actually present. The PLI group was in fact two separate samples: those with PLI pure and those with some autistic‐like behaviours (referred to here as PLI plus, following Bishop 1998). Second, group comparisons indicated that CNRep was significantly lower for children with SLI than all other groups (although this measure was not such a good discriminator using a specificity analysis). Third, the markers were able to discriminate between all types of communication impairment in normal control participants (n=100; 51 females, 49 males; mean age 11 years, range 10 years 5 months to 11 years 6 months) with sensitivity levels of at least 75% and specificity of 80%. Recalling Sentences was the most efficient marker for all groups. Finally, analysis showed that children with PLI plus could be accurately distinguished from all others, scoring most favourably overall on communication markers and on performance IQ scores.