bluuebunny/crossref_metadata_2025 huggingface dataset:
Development of cortical and subcortical brain structures in childhood and adolescence: a structural MRI study
Authors: Sowell Elizabeth R , Trauner Doris A , Gamst Anthony , Jernigan Terry L
Published: July 2010

The purpose of the present study was to describe in greater anatomical detail the changes in brain structure that occur during maturation between childhood and adolescence. High‐resolution MRI, tissue classification, and anatomical segmentation of cortical and subcortical regions were used in a sample of 35 normally developing children and adolescents between 7 and 16 years of age (mean age 11 years; 20 males, 15 females). Each cortical and subcortical measure was examined for age and sex effects on raw volumes and on the measures as proportions of total supratentorial cranial volume. Results indicate age‐related increases in total supratentorial cranial volume and raw and proportional increases in total cerebral white matter. Gray‐matter volume reductions were only observed once variance in total brain size was proportionally controlled. The change in total cerebral white‐matter proportion was significantly greater than the change in total cerebral gray‐matter proportion over this age range, suggesting that the relative gray‐matter reduction is probably due to significant increases in white matter. Total raw cerebral CSF volume increases were also observed. Within the cerebrum, regional patterns varied depending on the tissue (or CSF) assessed. Only frontal and parietal cortices showed changes in gray matter, white matter, and CSF measures. Once the approximately 7% larger brain volume in males was controlled, only mesial temporal cortex, caudate, thalamus, and basomesial diencephalic structures showed sex effects with the females having greater relative volumes in these regions than the males. Overall, these results are consistent with earlier reports and describe in greater detail the regional pattern of age‐related differences in gray and white matter in normally developing children and adolescents.
Wartenberg pendulum test: objective quantification of muscle tone in children with spastic diplegia undergoing selective dorsal rhizotomy
Authors: Nordmark Eva , Andersson Gert
Published: July 2010

The aim of this study was to investigate the reliability and sensitivity of the Wartenberg pendulum test for quantification of muscle tone in young children with spastic diplegia undergoing selective dorsal rhizotomy (SDR). Fourteen non‐disabled children (mean age of 5.5 years, age range 2.3 to 8.8 years, one female and one male in each year) were tested twice. Twenty children with spastic diplegia (12 males, eight females; mean age of 4.3 years, age range 2.5 to 6.3 years) consecutively selected for SDR, were assessed before and 6 months after SDR. Parameters of the pendulum test: R2, R1, maximal velocity, and swing time were correlated with clinical assessments for spasticity (modified Ash worth scale, quadriceps reflex) and measurements of gross motor function: the Gross Motor Function Classification System and the Gross Motor Function Measure. The Wartenberg pendulum test was found to be an objective and sensitive method for quantifying spasticity in knee extensor muscles in children as young as 2.5 years old. The method was responsive to changes after SDR. The only correlation with clinical measurements of spasticity was between the R2 ratio and the quadriceps reflex. Swing time was the most reliable and sensitive variable; it showed a weak correlation with measurements for gross motor function.
Cerebellar abnormalities in infants and toddlers with Williams syndrome
Authors: Jones Wendy , Hesselink John , PhD Eric Courchesne , Duncan Tim , Matsuda Kevin , Bellugi Ursula
Published: July 2010

One commonly observed neuroanatomical abnormality in adults with Williams syndrome is an enlarged cerebellum relative to a small cerebrum. Our study is the first to examine neuroanatomy in young children with Williams syndrome. Clinical brain MRI was examined in nine young children with Williams syndrome (mean age 21 months, range 7 to 43 months) relative to nine age‐ and sex‐matched normally developing control children (mean age 29 months, range 20 to 42 months), and two children with undiagnosed developmental disorders (6 and 41 months). Two neuroradiologists who were blinded to participant classification, hypotheses, and regions of interest for the study, sorted the brain scans into two groups on the basis of six neuroanatomical criteria. The raters placed more of the MR scans from children with Williams syndrome into a separate group when they analyzed features of the cerebellum, but not when they analyzed other brain regions. Based on their written comments, the raters focused on the large size of the cerebellum in the children with Williams syndrome. The results lead us to suggest that abnormal cerebellar enlargement is evident in those with Williams syndrome at an early age. Our results are discussed relative to the cognitive delays observed in Williams syndrome versus other disorders such as autism, leading us to suggest that the cerebellum may play a role in cognition.
Hearing loss in early infancy affects maturation of the auditory pathway
Authors: Tibussek Daniel , Meister Hartmut , Walger Martin , Foerst Astrid , Von Wedel Hasso
Published: July 2010

The influence of early cochlear hearing loss on maturation of the auditory pathway was studied by measuring auditory brainstem responses (ABR). In a retrospective study, 85 children with normal hearing (46 males, 39 females; age range 2 months to 14 years) and 165 children with binaural cochlear hearing impairment (89 males, 76 females; age range 1 month to 16 years) were examined. A significant positive correlation (p<0.001) between the degree of hearing loss and interpeak latencies I‐V (IPLI‐V) of the ABR was observed. No significant correlation (p=0.85) was found between hearing loss and interpeak latencies I–III (IPLI–III). These findings can be interpreted as indicating a marked delay in maturation of higher brainstem structures due to reduced auditory input during infancy. The correlation differs notably from results of comparable studies of adults published in recent literature. This leads to the assumption that the developing human brain is particularly sensitive to auditory deprivation. Thus, our results indicate the importance of a normal acoustic environment during sensitive periods in early childhood to ensure normal hearing and speech development.
Somatosensory testing in idiopathic scoliosis
Authors: Ólafsson Yngvi , Odergren Thomas , Persson Hans E , Saraste Helena
Published: July 2010

Sixty‐seven consecutive patients (59 females, eight males; mean age 13.6 years, age range 8.1 to 18 years) with adolescent idiopathic scoliosis were evaluated for segmental dystonia, thermal and vibratory thresholds, and cortical somatosensory evoked potentials (cSEPs). Clinically, there were no signs of segmental dystonia. No significant side difference was found in cSEPs and thermal thresholds. Vibration thresholds were increased on the right side with a significant side difference at all sites. However, these changes did not correlate to curve size, rotation, curve type, or convexity. It was concluded that, at the present level of somatosensory testing, no segmental neurophysiological disturbance can be related to the appearance of idiopathic scoliosis. The importance of asymmetric vibratory thresholds measurements cannot be deduced from this study.
Post‐streptococcal autoimmune dystonia with isolated bilateral striatal necrosis
Authors: Dale Russell C , Church Andrew J , Benton Sarah , Surtees Robert A , Lees Andrew , Thompson Edward J , Giovannoni Gavin , Neville Brian G
Published: July 2010

Infantile bilateral striatal necrosis (IBSN) is characterized by a dystonic movement disorder and basal ganglia imaging abnormalities. Acute IBSN often occurs after upper respiratory tract infections although no specific micro‐organism which may cause IBSN has been identified. We present 2 children (1 year 2 months and 4 years) with acute IBSN after clinical pharyngitis. Both IBSN patients had serological evidence of recent beta‐haemolytic streptococcal infection. Due to the association of post‐streptococcal disorders with anti‐basal ganglia antibodies (ABGA), we examined both patients for anti‐neuronal antibodies. For comparison, 20 children with dystonia (9 females, 11 males; mean age 4 years 1 month), and 20 children with uncomplicated streptococcal infection (12 females, 8 males; mean age 5 years 9 months) were examined. Both IBSN patients had antibodies reactive against basal ganglia constituents of molecular weight 40 kDa. Immunohistochemistry showed antibody reactivity against large striatal neurons only. Other anti‐neuronal antibodies were negative, supporting striatal specificity. All controls were negative for ABGA. Acute IBSN is part of the post‐streptococcal autoimmune neuropsychiatric spectrum. An autoimmune aetiology should be considered in this phenotype, as immunomodulatory therapies may reduce morbidity and mortality.
Correlation between physical functioning and gait measures in children with cerebral palsy
Authors: Tervo Raymond C , Azuma Scott , Stout Jean , Novacheck Tom
Published: July 2010

The primary aim of this investigation was to assess the correlation between the POSNA Musculoskeletal Functional Health Questionnaire (POSNA) and gait analysis in children with cerebral palsy (CP). POSNA and computerized gait analysis were used to evaluate individuals with CP. Correlations were investigated between POSNA scales, gait parameters, and the Gillette Functional Assessment Questionnaire (FAQ) in 63 children (31 males, 32 females; mean age 9.17 years [SD 3.06], age range 3.75 to 16.44 years) with spastic CP. Twelve participants had hemiplegia, 29 diplegia, 12 quadriplegia, and 10 triplegia. The result of backwards stepwise multiple regression analysis indicated that the Log normalcy index (NI) was a significant predictor of the POSNA Global Function and Comfort scale. Energy expenditure (EE) did not add significantly to the prediction. The POSNA scales differentiated between the different topographical types of CP. The POSNA scale is a valid and useful clinical measure. Used in conjunction with the NI, EE, and FAQ, the POSNA scale provides a more complete appraisal of change in functioning.
Clinically prescribed orthoses demonstrate an increase in velocity of gait in children with cerebral palsy:a retrospective study
Authors: White Hank , Jenkins Jennifer , Neace William P , Tylkowski Chester , Walker Janet
Published: July 2010

The purpose of this study was to determine the effect clinically prescribed ankle‐foot orthoses (AFOs) have on the temporal‐spatial parameters of gait, as compared with barefoot walking in children with cerebral palsy. A retrospective chart review of data collected between 1995 and 1999 in our motion analysis laboratory was performed. A retrospective chart review of 700 patients revealed 115 patients (mean age 9 years) who had a primary diagnosis of CP (diplegia n=97, hemiplegia n=18). All were wearing clinically prescribed hinged or solid AFOs at the time of undergoing a three dimensional gait analysis. In line with our standard clinical practice, data for both conditions (braced and barefoot walking) were collected the same day by the same examiner. Statistical analyses indicated the temporal and spatial gait parameters of velocity, stride length, step length, and single limb stance were significantly increased (p<0.001) with the use of AFOs versus barefoot walking. Cadence was the only parameter found to not be statistically different.
Head circumference in autism, Asperger syndrome, and ADHD: a comparative study
Authors: Gillberg Christopher , De Souza Linda
Published: July 2010

This study was undertaken to test the hypothesis that children with autistic spectrum disorders often have macrocephalus, and that those without comorbid learning disability* are most frequently affected. Fifty consecutive children with Asperger syndrome (45 males, five females; mean age 9 years, range 1 year 6 months to 16 years) without indications of underlying medical disorders were matched for birth year and sex with 50 children (45 males, five females; mean age 6 years 4 months, range 1 year 4 months to 13 years 11 months) who met criteria for autistic disorder (a lower‐functioning disorder within the autism spectrum) and with 50 children (45 males, five females; mean age 8 years 4 months, range 1 year 6 months to 15 years 5 months) who met criteria for attention‐deficit‐hyperactivity disorder. Birth and neuropsychiatric follow‐up records were examined and data relating to occipitofrontal circumference, weight, and height were detailed. The group with Asperger syndrome included a subset of individuals with macrocephalus recorded both at birth and at follow‐up after the first year of life. Another subgroup developed macrocephalus during early childhood. Autistic spectrum disorders include a subgroup with macrocephalus characterized by a relatively high level of functioning and a clinical presentation most often consistent with a diagnosis of Asperger syndrome.
Horseback riding in children with cerebral palsy: effect on gross motor function
Authors: Sterba John A , Rogers Brian T , France Amy P , Vokes Deborah A
Published: July 2010

The effects of recreational horseback riding therapy (HBRT) on gross motor function in children with cerebral palsy (CP: spastic diplegia, spastic quadriplegia, and spastic hemiplegia) were determined in a blinded study using the Gross Motor Function Measure (GMFM). Seventeen participants (nine females, eight males; mean age 9 years 10 months, SE 10 months) served as their own control. Their mean Gross Motor Function Classification System score was 2.7 (SD 0.4; range 1 to 5). HBRT was 1 hour per week for three riding sessions of 6 weeks per session (18 weeks). GMFM was determined every 6 weeks: pre‐riding control period, onset of HBRT, every 6 weeks during HBRT for 18 weeks, and 6 weeks following HBRT. GMFM did not change during pre‐riding control period. GMFM Total Score (Dimensions A‐E) increased 7.6% (p<0.04) after 18 weeks, returning to control level 6 weeks following HBRT. GMFM Dimension E (Walking, Running, and Jumping) increased 8.7% after 12 weeks (p<0.02), 8.5% after 18 weeks (p<0.03), and remained elevated at 1.8% 6 weeks following HBRT (p<0.03). This suggests that HBRT may improve gross motor function in children with CP, which may reduce the degree of motor disability. Larger studies are needed to investigate this further, especially in children, with more severe disabilities. Horseback riding should be considered for sports therapy in children with CP.
Minor neurological signs and developmental performance in high risk children at preschool age
Authors: Gosselin Julie , Amiel‐Tison Claudine , Infante‐Rivard Claire , Fouron Catherine , Fouron Jean‐Claude
Published: July 2010

The aim of this study was to establish correlations between minor neurological findings and developmental performance. A cohort of 72 preschool children was studied (38 females, 34 males; mean age 3 years 8 months, SD 1 year 2 months, range 2 to 5 years) who were considered to be at high risk due to placental insufficiency. The cohort was divided into four categories of neurological status: (1) minimal cerebral palsy (MCP) with independent walking before age 2 years; (2) Amiel‐Tison triad (ATT) including imbalance of passive axial tone, phasic stretch reflex in triceps surae, and cranial signs, particularly on the squamous suture; (3) intermediate with one or two of the three ATT signs; and (4) absence of neurological findings. Six subscales of the Griffiths Mental Developmental Scales assessing locomotion, eye‐hand coordination, interpersonal skills, language, performance, and practical reasoning were administered. Significant differences were found according to neurological status in three specific domains of development: coordination (F=2.84,p=0.04), language (F=3.65,p=0.02), and practical reasoning (F=3.62, p=0.02). In addition, significant differences were also found in language (L) and practical reasoning (R) performances according to the side of the abnormal stretch reflex: bilateral stretches (L=87.8; R=75.3) or an isolated right stretch (L=95.3; R=83.6) are more strongly associated with impaired developmental performances than an isolated left stretch (L=101.3; R=88.2) with F=2.94;p=0.04 for language and F=3.00,p=0.04 for practical reasoning. We concluded that a short neurological examination, easily performed by pediatricians and family practitioners, can identify permanent markers of minor brain damage occurring before, during, or soon after birth and so anticipate consequences.
Melatonin treatment for sleep disorders in children with neurodevelopmental disorders: an observational study
Authors: Ross Caroline , Davies Paul , Whitehouse William
Published: July 2010

The study aim was to quantify melatonin‐associated improvement in sleep by means of a parent‐completed sleep diary during routine outpatient activity. An investigation into sleep disturbance was made at neurology outpatient appointments. Those parents who identified a problem were asked to complete a sleep diary, after which treatment was initiated. The first week of the diary was completed before treatment, the second when established on the maximum dose of melatonin required. Forty‐nine patients (26 males, 23 females) aged from one to 13 years, were treated between 1997 and 1998: 28 of these returned interpretable diaries. In a further 18 patients, an assessment could be made of the usefulness of the treatment. Patients were fairly typical of those attending a tertiary centre, the most common primary diagnosis being epilepsy (n=26). Only seven patients were visually impaired. Of the 46 patients who were assessed, 34 showed an improvement. No adverse effects were attributed to the treatment.
The BREV neuropsychological test: Part I. Results from 500 normally developing children
Authors: Billard C , Vol S , Livet M O , Vallée L , Gillet P
Published: July 2010

The Battery for Rapid Evaluation of Cognitive Functions (Batterie Rapide d'Evaluation des Fonctions Cognitives: BREV) was designed to provide health professionals with a quick clinical tool for screening acquired and developmental cognitive deficits in children aged 4 to 8 years. The BREV explores oral language in both its expressive and receptive forms, non‐verbal functions, attention, verbal and visuo‐spatial memory, and main learning acquisition. Results of the first phase of validation are presented in this report consisting of internal validity measurements gained by testing 500 normally developing school children (257 females, 243 males; mean age 6 years 7 months, SD 1 year 6 months. The validation provides appropriate values for each of the 17 subtests assessing cognitive functions (oral language, nonverbal abilities, attention and memory, educational achievement) in 10 age groups, from 4 to 8 years of age. All subtests with the same content for any age revealed values which increased significantly with age. Interreliability was tested in a retest for 70 children and scores obtained on retesting correlated significantly with initial values. The BREV is a reliable test with carefully established normative values, appropriate for preschool and school‐age children.
Familial dyslexia: neurocognitive and genetic correlation in a large Finnish family
Authors: Nopola‐Hemmi Jaana , Myllyluoma Birgitta , Voutilainen Arja , Leinonen Seija , Kere Juha , Ahonen Timo
Published: July 2010

Neuropsychological findings of individuals with dyslexia (n=24) from a large, three‐generation Finnish family are presented. We have previously performed whole genome linkage scanning in this family and found that dyslexia in this kindred segregates with a single locus in the pericentromeric area of chromosome 3. Those included in the analyses were carefully evaluated for general cognitive ability, reading and spelling skills, and reading‐related neurocognitive skills. The neurocognitive type of dyslexia segregating in this family consisted of deficits in phonological awareness, verbal short‐term memory, and rapid naming. Severe dyslexia also seemed to be connected with a general language difficulty and was most common in the eldest generation.
Cervical accelerometry in preterm infants
Authors: Reynolds Eric W , Vice Frank L , Bosma James F , Gewolb Ira H
Published: July 2010

The objective of this study was to develop a method to use digital signal processing (DSP) technology to describe quantitatively and statistically swallow‐associated sounds in preterm infants and to use this method to analyze changes as infants mature. Twelve recordings of accelerometric and physiological data on bottle‐feeding preterm infants between 32 and 39 weeks’postmenstrual age (PMA) were analyzed. Cervical auscultation was performed using an accelerometer attached over the larynx. Acoustic data were recorded and graphically displayed using DSP software. Initial discrete sounds (IDSs) were identified and used to construct an average waveform from which a‘variance index’(VI) was calculated for each infant. The shape of the IDS waveforms became progressively more uniform with advancing PMA, as indicated by a significant inverse correlation between VI and PMA (r=0.739;p=0.006). DSP technology facilitated the development of a new method to quantitatively analyze feeding in preterm infants. This method provides an elegant tool to track maturation of infant feeding and assessing feeding readiness. This technique makes the interpretation of cervical auscultation data less subjective by replacing the verbal description of the sounds of feeding with quantitative numeric values. It is anticipated that this method can be automated to facilitate further the analysis of cervical accelerometry data.
Plasma long‐chain polyunsaturated fatty acids and neurodevelopment through the first 12 months of life in phenylketonuria
Authors: Agostoni Carlo , Verduci Elvira , Massetto Nicoletta , Radaelli Giovanni , Riva Enrica , Giovannini Marcello
Published: July 2010

The aims of the study were to examine the relationship between long‐chain polyunsaturated fatty acid (LCPUFA) status at diagnosis of phenylketonuria (PKU)and neurodevelopment through the first 12 months of life, and to assess whether any difference exists between infants breastfed and bottlefed in the first days of life on the basis of LCPUFA status. Twenty infants with PKU were prospectively examined through the first year of life. Plasma fatty acids were measured in infants at diagnosis. Plasma phenylalanine levels were determined monthly. Main outcome measures were the Bayley Mental Developmental Index (MDI) and Psychomotor Developmental Index (PDI) at 5 and 12 months of age, and the visual function at 12 months, evaluated by P100 wave latencies with visual evoked potentials. A higher PDI score was associated with higher plasma arachidonic acid at diagnosis (adjusted correlation coefficient of PDI at 5 months, r=0.38, p=0.05). P100 wave latency at 15 minutes of arc (15′) was associated with the early plasma arachidonic acid (adjusted r=‐0.56, p=0.02) and phenylalanine levels at 12 months (adjusted r=0.22, p=0.05). No association was found between MDI score and any essential fatty acids. Breastfed infants exhibited higher plasma arachidonic acid (mean difference, Δ,=3.4%; 95% confidence interval [CI]=1.2–5.6%) and shorter P100 wave latency at 15’(Δ=‐21ms, 95%CI=‐30 to ‐12) than bottlefed infants. Within the population of this study, a weak positive association has been found between plasma LCPUFAs at diagnosis (higher in breastfed infants) and neurodevelopmental indices through the first year of life.
Visual and spatial working memory dissociation: evidence from Williams syndrome
Authors: Vicari Stefano , Bellucci Samantha , Carlesimo Giovanni Augusto
Published: July 2010

This study aimed at investigating the possible dissociation between visual and spatial working memory (WM) by means of two different experiments. In the first experiment, a WM test for visual material and for spatial information was given to a group of 202 normally developing children (chronological age 7 years 5 months, SD 1.6 years; 109 males, 93 females). Results document a different developmental trend in the two span tests. In the second experiment, we presented the same visual and spatial WM tests to a group of 13 individuals with Williams syndrome (WS), seven males and six females (chronological age mean 18 years 3 months, SD 5.1; mental age mean 7 years 2 months, SD 1.5 years), and to a control group of 26 typically developing children 14 males and 12 females matched for mental age (mean 7 years 2 months, SD 1.4). The results of this second experiment show that, on average, the spatial span obtained by individuals with WS was significantly lower than control participants, but visual span was comparable in the two groups. Our data support the hypothesis of a dissociation within the visuo‐spatial sketch pad slave system in the WM model.
Modulation of spatial attention in a child with developmental unilateral neglect
Authors: Dobler Veronika B , Manly Tom , Verity Chris , Woolrych Jonathan , Robertson Ian H
Published: July 2010

Attentional neglect of left space is one of the most striking acquired neurological disorders of adulthood. Recent evidence indicates a link between left spatial neglect and general right‐hemisphere impairments in sustained attention and alertness. Poor sustained attention and alertness is also a central feature of other disorders, particularly childhood attention‐deficit‐hyperactivity disorder (ADHD). Here we present the case of a 7‐year‐old male showing that frank neglect can be present in children with sustained attention problems without a clear aetiological event, or obvious structural brain abnormalities as indicated by a normal MRI. Experimental amelioration of the neglect through left‐hand movement and externally alerting stimulation by uninformative sounds further suggest close similarities to the adult disorder. We suggest that such distortions of spatial attention may be more common in childhood than previously thought.
Prevalence of autistic spectrum disorder in children attending mainstream schools in a Welsh education authority
Authors: Webb E , Morey J , Thompsen W , Butler C , Barber M , Fraser WI
Published: July 2010

All mainstream primary schools in Cardiff were invited in July 1998 to participate in a prevalence survey of autistic spectrum disorder. Teachers of each class filled in a questionnaire based on ICD‐10 criteria for autistic disorders. The Autism Spectrum Screening Questionnaire (ASSQ) was completed on children identified with problems identified by the questionnaire. A total of 11692 children born between 1 September 1986 and 31 August 1990 were screened: 234 (2%) children were identified as requiring an ASSQ; 151 of 234 (65%) ASSQs were returned. Of the 151, 60 children (52 male, 8 female; 40%) scored 22 or more. Their notes and the involved professionals were consulted. Thirty‐five children, unknown to specialist services or with complex features, required additional assessment. Seventeen children (all male) were found to be on the autistic spectrum. When the overall rubric was disentangled we found a diverse population of affected children including a handful who did not fit easily into ICD‐10 classification. Correcting for incomplete ascertainment we found a minimum prevalence of 20.2 out of every 10000 (SE=4.5) for autistic spectrum disorder in this population.
Intrarater reliability of lower limb sagittal range‐of‐motion measures in children with spastic diplegia
Authors: Kilgour Gaela , McNair Peter , Stott N Susan
Published: July 2010

In this study, 10 sagittal lower limb range‐of‐motion measures were conducted in a blinded fashion in 25 children with spastic diplegic cerebral palsy and in 25 age‐ and sex‐matched controls. The participants comprised 22 males, mean age 10 years 8 months and 28 females, with mean age 9 years 8 months; age range 6 to 17 years. One paediatric physical therapist performed duplicate goniometric measures at zero time and 7 days later using the same sequence of measures, location, and time of day. Mean absolute differences for measures within one session ranged from 0.7 to 2.9° in controls and from 1 to 4.2° in children with spastic diplegia. Most intra‐class correlation coefficients (ICCs) for intra‐sessional measures were more than 0.90 in both groups. Measures between sessions were less reliable. Mean absolute differences between sessions were up to 7.1° for children with spastic diplegia and 8.6° for controls, with most ICCs being less than 0.80. Inter‐sessional variation in measures was similar in both groups, suggesting that measurement variability is not influenced by the presence of spasticity. Averaging of two measures did not improve inter‐sessional reliability compared with the use of a single measure. Dynamic measures (R1) were as reliable as passive measures (R2), but there were inter‐sessional differences in calculations using R1 and R2 measures of up to 30°.
Individual differences in responsivity to a neurobehavioural examination predict crying patterns of 1‐week‐old infants at home
Authors: James‐Roberts Ian St , Bargn Jenny Goodwin , Peter Bernice , Adams Diana , Hunt Steve
Published: July 2010

Unexplained crying in infants aged 1 to 3 months is a common concern for Western parents and health services. This study examined the hypothesis that the crying is due to high infant responsivity, and provides evidence about the types of stimulation that trigger crying in infants who present with high responsivity. The sample included 93 eight‐day‐old infants from a community sample (47 females, 46 males; mean birth weight 3457g; mean gestation 39.4 weeks; mean Apgar scores 8.28 at 1 minute and 9.59 at 5 minutes). Infants were tested for their response to two standard, mildly challenging, procedures: a neurobehavioural test involving undressing, putting down, and handling, and the Guthrie test, involving a painful heel prick to obtain a blood sample. The infants’ crying over 24 hours was recorded in parental diaries. Newborn infants who exhibited high responsivity during the neurobehavioural assessment cried, rather than fussed, the most at home. High responsivity during the neurobehavioural assessment also predicted those infants who cried a lot and met a definition of ‘colic’ at home. The findings support the responsivity hypothesis and show that infants with high responsivity are upset by undressing, putting down, and sustained handling. Explanations for this and implications for the management of infant crying and colic are discussed.
Prevalence of reduced bone mass in children and adults with spastic quadriplegia
Authors: King Wilson , Levin Ronald , Schmidt Rosemary , Oestreich Alan , Heubi James E
Published: July 2010

This study was designed to test the hypothesis that non‐ambulatory patients with spastic quadriplegia will have reduced bone mass which worsens with increasing age. Forty‐eight patients (age 5 to 48 years, median age 15 years; 19 females and 29 males) were studied. Anticonvulsants were used in 29 patients (60.4%). Lumbar spine bone mineral density (LS‐BMD) was markedly reduced compared with age‐and sex‐matched control individuals with a z score of ‐2.37±0.21. Twenty‐eight (58%) had z scores of less than ‐2. A history of documented previous fracture was present in 19 patients (39%). Patients with a history of fracture had significantly lower (p=0.05) LS‐BMD z scores (‐2.81±0.29) compared with those without a history of fracture (‐2.11±0.26). Mean serum 25‐OH vitamin D was 29.6±1.9ng/mL (normal 9 to 37.6ng/mL) with three patients having serum 25‐OH vitamin D levels less than 15ng/mL. These findings indicate that BMD is markedly reduced in non‐ambulatory children and adults with neuromuscular disease. Reductions in bone mass put them at greater risk for non‐traumatic fractures.
Prosopagnosia in a preschool child with Asperger syndrome
Authors: Pietz Joachim , Ebinger Friedrich , Rating Dietz
Published: July 2010

We investigated a male, aged 4 years 11 months, who fulfilled the criteria of Asperger syndrome). In addition to the typical pattern of autistic symptoms, psychological testing revealed prosopagnosia in tasks for face recognition and matching. Prosopagnosia was also present when he tried to identify the faces of his parents and himself in photographs whenever these were presented with photographs of other persons. Although impairment in reciprocal social interaction in individuals with Asperger syndrome is closely correlated to their impaired perceptional abilities in non‐verbal communication, especially facial expression, overt prosopagnosia seems to be a rare neuropsychological symptom in persons with autistic disorders.
Measures of muscle and joint performance in the lower limb of children with cerebral palsy
Authors: Fosang Adrienne L , Galea Mary P , McCoy Anne T , Reddihough Dinah S , Story Ian
Published: July 2010

The aim of this study was to determine the reliability and magnitude of error of three lower‐limb clinical measures for children with cerebral palsy (CP): the Modified Ashworth Scale of Spasticity (MAS), passive range of movement (PROM) and the modified Tardieu scale (MTS). Six physiotherapists measured 11 females and seven males (mean age 6 years 4 months, SD 2 years 4 months; age range 2 years 4 months to 10 years) on two occasions using a repeated measures design, collecting all data over 6 days. The severity of CP spanned all five levels of the Gross Motor Function Classification System and all children demonstrated varying degrees of spasticity. Exclusion criteria included botulinum toxin injections, inhibitory plasters, and orthopaedic surgery within the 6 months before study entry. For PROM and the MTS interrater reliability was acceptable with an intraclass correlation coefficient of 0.7, but results for MAS were lower. Standard error of measurement for repeated measures of PROM and MTS was about five degrees, but 95% confidence interval ranges were considerably higher. Test‐retest results varied widely, particularly for the MAS. These measurement tools should be used with caution when evaluating changes in young children with CP.
Autism spectrum disorders in children with active epilepsy and learning disability: comorbidity, pre‐ and perinatal background, and seizure characteristics
Authors: Steffenburg Suzanne , Gillberg Christopher
Published: July 2010

The aim of this study was to examine the comorbidity pattern, seizure characteristics, and aetiology in a representative group of children with a combination of autism spectrum disorder (ASD), active epilepsy, and learning disability. Ninety children (47 males, 43 females; mean age 11 years 2 months, range 8 to 16 years at the time of psychiatric examination) with active epilepsy and learning disability, identified in a population‐based study in Göteborg, Sweden, were subdivided into those with and those without ASD and compared with respect to aetiology, additional neuroimpairments, and seizure characteristics. In addition, the cohorts were examined for trends of prevalence over a period of time. Results indicated that established aetiology was much more often present in the prenatal period than in the peri‐ or postnatal periods in the ASD group. Cerebral palsy and visual impairment were under‐represented in the ASD group. Partial seizures tended to be more common and generalized seizures less common in the ASD group compared with the non‐ASD group. Seizure onset was later in the ASD group. Many of the significant differences were accounted for by a large group of psychiatrically unclassifiable participants in the non‐ASD group. There was no trend towards an increase of affected children over the 12‐year period. There was no increase in the prevalence of active epilepsy and learning disability nor in the rate of autism with active epilepsy and learning disability in children born between 1981 and 1986 compared with those born from 1976 to 1980, indicating no statistical association with the general measles‐mumps‐rubella vaccination introduced in the early 1980s.
Deficient coordination of associated postural adjustments during a lifting task in children with neurodevelopmental disorders
Authors: Jucaite Aurelija , Fernell Elisabeth , Forssberg Hans
Published: July 2010

Precision grip and concomitant anticipatory postural adjustments were investigated in 11 children (three females, eight males; mean age 9 years 1 month, SD 11 months) with attention‐deficit‐hyperactivity disorder (ADHD); 12 children (three females, nine males; mean age 9 years, SD 7 months) with developmental coordination disorder (DCD), and 13 children (two females, 11 males; mean age 9 years 9 months, SD 11 months) with a combination of ADHD and DCD (ADHD+). There were two comparison groups: an age‐matched group (four females, 11 males; mean age 9 years 1 month, SD 14 months) and a younger age group (five females, six males; mean age 6 years 5 months, SD 8 months). Adaptation to different weights was evaluated by lifting a specialized grip instrument monitoring grip force, load force, and centre of foot pressure displacements. Children with ADHD+ showed: (1) excessive grip forces, (2) decreased amplitude and prolonged onset of postural adjustments, and (3) reduced ability to adapt the motor output. Children with ADHD and DCD did not scale manual and postural forces in amplitude and time domains. Children with DCD also differed in delayed timing of postural adjustments. Results indicate that children with ADHD and DCD show a spectrum of neural dysfunctions underlying poor motor coordination, which are not specific to the clinical disorder.
Alternating hemiplegia of childhood: a syndrome inherited with an autosomal dominant trait
Authors: Kanavakis Emmanuel , Xaidara Athina , Papathanasiou‐Klontza Dimitra , Velentza Stavroula , Youroukos Sotiris
Published: July 2010

Alternating hemiplegia of childhood is a rare disorder characterized by recurrent attacks of hemiplegia affecting either side of the body, oculomotor and autonomic disturbances, movement disorders, and progressive cognitive impairment. We report on one family with autosomal dominant alternating hemiplegia. The disorder was first recognized in a 9‐year‐old child, the third son of the family, who presented with learning disability,* tonic‐clonic seizures, dystonic attacks, and episodes of alternating hemiplegia starting at the age of 21/2 years. His mother and three brothers had similar symptoms. The maternal uncle, who has learning disability, had experienced multiple dystonic attacks. Tests performed on the family, including computerized tomography, magnetic resonance imaging, and magnetic resonance angiography of the brain as well as metabolic evaluation, were normal. Cytogenetic analysis was normal and mitochondrial DNA analysis revealed no deletions or mutations in the four affected family members and the grandmother. An autosomal dominant mode of inheritance is suggested by the fact that both sexes are affected in two generations.
Epilepsy and perisylvian polymicrogyria in a patient with Kabuki syndrome
Authors: R H W , E P , Sisodiya S M
Published: July 2010

Kabuki syndrome is a dysmorphogenic syndrome which has been reported in over 300 patients since it was first described in Japan in 1981. In addition to its cardinal features (typical facies, mild‐to‐moderate learning disability, short stature, skeletal anomalies, and dermatoglyphic abnormalities with persistent foetal fingerpads), neurological anomalies are frequently reported, including epilepsy in 8% of those with the syndrome. We present here a 22‐year‐old white female patient with refractory partial epilepsy, Kabuki syndrome, and bilateral perisylvian polymicrogyria on MRI: the first reported case of this association. The aetiology of the syndrome, including the diverse genetic changes recognized, is then discussed.
Early neurodevelopmental markers predictive of mortality in infants infected with HIV‐1
Authors: Llorente Antolin , Brouwers Pim , Charurat Manhattan , Malee Kathleen , Mellins Claude , Ware Janice , Hittleman Joan , Mofenson Lynne , Velez‐Borras Jesus , Adeniyi‐Jones Samuel
Published: July 2010

One‐hundred and fifty‐seven vertically infected HIV‐1 positive infants (85 males, 72 females) underwent longitudinal assessment to determine whether early neurodevelopmental markers are useful predictors of mortality in those infants who survive to at least 4 months of age. Survival analysis methods were used to estimate time to death for quartiles of 4‐month scores (baseline) on the Bayley Scales of Infant Development (BSID). Cox proportional hazards progression was used to estimate relative hazard (RH, 95% CI) of death for BSID scores and potential confounders. Thirty infants with BSID scores at 4 months of age died during follow‐up. Survival analysis revealed greater mortality rates in infants with BSID (Mental Developmental Index and Psychomotor Developmental Index) scores in the lower quartile(p=0.004,p=0.036). Unadjusted univariate analyses revealed increased mortality associated with baseline CD4+ 29%, gestational age <37 weeks, smaller head circumference, advanced HIV and higher plasma viral load. BSID scores independently predicted mortality after adjusting for treatment, clinical category, gestational age, plasma viral load and CD4+ percentage.
Assessing the impact of pediatric epilepsy and concomitant behavioral, cognitive, and physical/neurologic disability: Impact of Childhood Neurologic Disability Scale
Authors: Camfield Carol , Breau Lynn , Camfield Peter
Published: July 2010

Epilepsy has a significant impact on a child's life, the extent to which is based on four factors: epilepsy, cognition, behavioral, and physical/neurologic function. This study evaluates the ability of the 44‐item Impact of Childhood Neurologic Disability Scale (ICND) to assess each of these four realms. Parents of children (aged 2 to 18 years) with epilepsy rated their child's overall quality of life and completed the ICND. External validation compared the ICND with (1) neurologists’reports of children's behavior, cognitive abilities, physical/neurologic disability, and epilepsy; and (2) parents, teachers, and children's ratings on six‘criterion standard’questionnaires. Families of 68 children with epilepsy only and 29 children with‘epilepsy‐plus’(additional cognitive, behavioral, or physical/neurologic disability; 39 males, 58 females; mean age at testing 10 years 3 months [SD 4.5] age range 2 to 17 years) participated. Internal consistency was excellent (Cronbach's α=0.92) as was test‐retest reliability (intraclass correlation=0.89). Caregivers distinguished the impact of each of the four realms. Scores were negatively related to quality of life (Pearson's r=‐0.59). Children with high ICND scores had more difficulties at home and school. Their parents saw them as less rewarding and adaptable and the children saw themselves as less intelligent and less popular with more emotional problems. In addition, children with‘epilepsy‐plus’had significantly higher total ICND scores as well as markedly elevated scores within each of the four realms when compared with the epilepsy‐only group. It is concluded that the ICND is an accurate, quick measurement tool reflecting the impact of behavior, cognitive learning ability, physical/neurologic disability, and epilepsy on children and their families.
Hemispatial neglect in young children with early unilateral brain damage
Authors: Trauner Doris A
Published: July 2010

Hemispatial neglect is commonly observed in adults following right‐hemisphere brain lesions. Given the potential for reorganization in the developing brain, spatial neglect may not be apparent following early unilateral damage. This study aimed to determine whether infants who experienced pre‐ or perinatal focal brain lesions showed evidence of spatial neglect. Study participants were 33 infants/toddlers (22 males, 11 females; age range 6 to 48 months); 27 preschool children (14 males, 13 females; age range 28 to 75 months) with either left hemisphere (LH) or right hemisphere (RH) damage of pre‐ or perinatal onset (total 60), and 36 control individuals (15 females, 21 males; age range 28 to 75 months). Participants were assessed using two object‐removal preference tasks. Control children showed no lateralized preference for object removal. Those with LH or RH damage preferentially removed objects from the side of the board ipsilateral to the lesion first and the contralateral side last. These results suggest that spatial neglect may be found in young children even after very early unilateral brain damage. Further, in contrast to the adult pattern, contralateral neglect is present for up to 6 years after either LH or RH damage. This pattern suggests that there may be a different distribution of attention to space in the developing brain as compared with the mature brain. The persistence of spatial neglect suggests that there are some limitations on plasticity in the developing human brain.
Behavioural and emotional characteristics in children with Sotos syndrome and learning disabilities
Authors: Sarimski Klaus , Psychologist Clinical
Published: July 2010

In contrast to physical characteristics, the developmental and behavioural characteristics of children with Sotos syndrome are not well documented. Data from a survey of 27 children (17 males and 10 females; mean age 10 years 7 months; range 6 to 15 years) with mild and moderate learning disabilities were obtained concerning school placement, social networks, adaptive competence, and behavioural problems and compared with a control group matched for cognitive level and age. Ten children attended a mainstream school (with remedial assistance), eight were placed in a school for children with learning disabilities, nine children attended other special schools. Social contact problems and anxious behaviour were reported as the most prominent characteristics of children with Sotos syndrome. Some implications for psychological counselling and educational support are discussed.
Comorbid Asperger and Tourette syndromes with localized mesencephalic, infrathalamic, thalamic, and striatal damage
Authors: Berthier Marcelo L , Kulisevsky Jaime , MD Jesús Aparicio
Published: July 2010

We describe the coexistence of Asperger and Tourette syndromes (AS and TS) caused by discrete hypoxic‐ischaemic necrosis of the midbrain, infrathalamic and thalamic nuclei, and striatum in an adolescent male with positive family history for tics and obsessive‐compulsive disorder. Behavioural ratings, cognitive tests, and volumetric measurements of the basal ganglia were performed in the patient and five other individuals with AS‐TS unassociated with MRI lesions. Cognitive deficits in attentional, executive, and visual‐spatial domains were found both in the patient and control AS‐TS group, though deficits were more severe in the former. MRI showed reduction of the left basal ganglia volume compared with the right in the patient, whereas the control group showed reduction of right basal ganglia volume compared with the left. It is suggested that individuals with a genetic predisposition to TS may develop AS and TS after involvement of midbrain and related components of basal ganglia‐thalamocortical circuits normally implicated in the integration of emotional, cognitive, and motor functions.