bluuebunny/crossref_metadata_2025 huggingface dataset:
Chickenpox in Mid‐Trimester Pregnancy: Always Innocent?
Authors: Wheatley Robert , Morton Richard E , Nicholson J.
Published: July 2010

A case of congenital varicella infection is presented, in which despite profound cerebral damage, the cardinal features of cicatricial skin lesions and limb shortening were absent. The case is compared with previously described cases, and with published series, in which this combination is uncommon. The risks of maternal varicella infection to the developing fetus are discussed in the light of recent prospective studies which demonstrate a rather lower risk than had previously been thought to be the case.

La varicelle durant le deuxième trhnestre de la grossesse: lotijottrs sans effets?

L'article rapporte un cas de varicelle congénitale, dans Iequel, en dépit de dommages cérébraux profonds, les signes caractéristiques de lésions cicatriciclles cutanécs et le raccourcissement des membres étaient absents. Le cas est comparéà des cas préalablement décrits, et avec des séries publiécs, dans lesquels cette combinaison est inhabituelle. Les risques d'une infection varicelleuse maternelle sur le foetus en développement sont discutés à la lumière d'études prospectives récentes qui démontrent un risque plutôt plus faible qu'il n'avait été cstimé auparavant.

Windpocken im millleren Trimester der Schwangerschaft ‐ immer hannlos?

Es wird ein Fall mit kongenitaler Varicellen‐Infektion vorgestellt, bei dem trotz eines schweren Cerebralschadens die Kardinalsymptome ‐ narbige Hautläsionen und Extremitätenverkürzungen fehlten. Der Fall wird mit früher beschriebenen Fällen und mit publizierten Untersuchungsreihen verglichen, bei denen diese Kombination ungewöhnilch ist. Die Risiken einer mütterlichen Varicelleninfektion für den Fetus werden anhand kürzlich erschienener prospektiver Studien diskutiert, die ein relativ niedrigeres Risiko zeigen als bisher angenommen.

Varwela en el segundo trimestre de embarazo. Es siempre inocente?

Se presenta un caso de infección varicelosa congénita, en la cual a pesar de una lesión cerebral grave, las características cardinales de lesiones cutáneas cicatriciales y acortamiento de miembros estaban ausentes. El caso se compara con otros descritos previamente y casos publicados, en los cuales esta combinación no es corriente. El riesgo de que una varicela materna pucda afectar al feto en desarrollo, se discute a la luz de estudios recientes prospectivos que demuestran un riego más bajo de lo que en principio se creia.
SURVIVAL AT 5 YEARS OF A COHORT OF NEWBORN INFANTS WITH MYELOMENINGOCELE
Authors: Worley Gordon , Schuster James M , Oakes W Jerry
Published: July 2010

Over a nine‐year period, 63 viable newborns with myelomeningocele were consecutively treated, of whom 11 (17%) developed brainstem symptoms assessed to be potentially life‐threatening. All 11 underwent brainstem decompression by cervical laminectomy with stent placement between the fourth ventricle and the spinal subarachnoid space, at a median âge of 8 months. 86% survived to 60 months of age. Those with brainstem dysfunction had a significantly greater mortality than those without, despite aggressive neurosurgical management by brainstem decompression.

Survie a 5 ans ď'un groupe de nouveaux‐nes avec myéloméningocèle

Sur une periode de neuf ans, 63 nouveaux‐ns viables avec myelomeningocele ont été traits consecutivement, parmi lesquels 11 (17%) presenterent des signes d'atteinte du tronc cerebral considerees comme potentiellement capables de mettre en jeu le pronostic vital. Une decompression du tronc cerebral par laminectomie cervicale avec placement d'une derivation entre le quatrieme ventricule et l'espace medullaire sousarachnoi'dien, fut entreprise chez ces onze patients, a un âge moyen de 8 mois. Quatre‐vingt six survivaient a l'âge de 60 mois. Les patients ayant presente des troubles du tronc cerebral avaient un taux signiftcativement plus eleve de mortalite que les autres, en depit de l'intervention neurochirurgicale pour decompression du tronc cerebral.

Das iiberleben von enter Grttppe von Neugeborenen bis ‐urn 5 Lebensjahr

Uber einen Zeitraum von neun Jahren wurden 63 lebensfahige Neugeborene mit Myelomeningocele behandelt, von denen 11(17%) potentiell lebensbedrohliche Hirnstammsymptome entwickelten. Bei alien wurde im mittleren Alter von 8 Monaten eine Hirnstammdekompression durch cervicale Laminektomie mit einer Drainâge zwischen dem vierten Ventrikel und dem Subarachnoidalraum durchgefiihrt. 86% hat ubergclebt bis zum 60 Monaten. Die Kinder mit Himstammdysfunktion hatten eine signifikant hohere Mortalitat als die ohne Himstammdysfunktion, trotz des agressiven neurochirurgischen Eingriffs zur Hirnstammdekompression.

Supervivencia a los cinco anos de una cohorte de recien nacidos con mielomeningocele En un periodo de nueve anos, se trataron 63 recien nacidos con mielomeningocele consecutivamente. De ellos 11 (el 17%) desarrollaron si'ntomas de tronco cerebral, evaluados como potencialmente.mortales. Todos los 11 casos fueron tratados con una laminectomia cervical descopmpresora con colocacion de una fijacion entre el cuarto ventrfculo y el espacio subaracnoideo espinal, a una edad promedio de meses. El 86% sobrevivio hasta los 60 meses de edad. Los que tenfan una disfuncion del tronco cerebral tenfan una mortalidad significativamente mayor que los que no la tenian, a pesar de un manejo neuro‐quirurgico agresivo con descomprension del tronco cerebral.
Helicobacter pylori in an institution for disabled children in Hong Kong
Authors: Lewindon P J. , D Lau MBBS, MRCP(UK)  , Chan A.
Published: July 2010

Anti‐Helicobacterpylori antibodies were determined in 157 institutionalised Cantonese children, mean age 9.5 ± 3.9 (SD) years, with profound neurodevelopmental disabilities. Eighty‐seven (55.4%) wereH.pylari seropositive compared with four of 50 (8%, P>0.0002) of an age‐matched control group, mean age 7.2 ±4.3 (SD) years. Eight of 15 seropositive children with a recent history of upper gastrointestinal bleeding underwent endoscopy and in all cases gastric infection with H.pylcri was confirmed. Anthropometric data from institutionalised children revealed marked malnutrition but showed no significant difference between seropositive and seronegative children. Disabled children receiving long‐term residential care in Hong Kong are confirmed to be at increased risk of H.pybri infection.
Visual agnosia with bilateral temporo‐occipital brain lesions in a child with autistic disorder: a case study
Authors: Mottron Laurent , Mineau Susanne , Décarie Jean‐Claude , Jambaqué Isabelle
Published: July 2010

A 2‐year‐old boy meeting the criteria for autistic disorder was diagnosed 2 years later with a visual agnosia characterised by a combination of certain aspects of associative and apperceptive agnosia. MRI then revealed a severe encephalomalacia of the right temporal lobe and bilateral temporo‐occipital areas. This association is discussed in terms of a clinical and aetiological relation between autistic disorder and visual agnosia.
Families of children with 5p‐ (cri du chat) syndrome: familial stress and sibling reactions
Authors: AWijma Cindy , Masino Linda L. , Hodapp Robert M.
Published: July 2010

This research examined family stress and sibling reactions in families of children with 5p‐ (cri du chat) syndrome aged 1 to 18 years who were living at home. In Study 1,99 parents reported on themselves and their child with 5p‐, as well as on family demographics, social supports, and stress. The best predictor of familial stress was the child's amount of maladaptive behavior, accounting for 12 to 38% of the variance across different stress measures. In Study 2, sibling concerns were examined in 44 unaffected siblings. The major finding was that parents and siblings disagreed on the extent of the siblings' interpersonal concerns. Parents reported that siblings felt ignored and misunderstood, whereas siblings themselves rated these concerns at much lower levels.
Assessment of general movements: towards a better understanding of a sensitive method to evaluate brain function in young infants
Authors: Hadders‐Algra Mijna , Van den Nieuwendijk Annekt WJ Klip‐ , Maitijn Albert , van Eykern Leo A.
Published: July 2010

The consistent presence of an abnormal quality of general movements (GMs) during the first postnatal months points to a high risk for the development of a neurological disability. To elucidate the significance of abnormal GMs during a single assessment, a classification system for abnormal GMs was developed. To this end six term and ten preterm infants with abnormal GMs were studied longitudinally with video and electromyographic (EMG) recordings till 59 weeks postmenstrual age (PMA). Two basic categories of abnormal GMs were distinguished: (1) mildly abnormal GMs (two types), which lacked fluency while conserving pattern complexity, and (2) definitely abnormal GMs (four types), which lacked fluency and complexity altogether. GM type before 39 weeks PMA correlated with findings on neonatal ultrasound brain scans. GM quality after 47 weeks PMA was strongly related to neurodevelopmental outcome at IK years of age, suggesting that the absence of the age‐specific ‘fidgety’ character of GMs could be a herald of disability.
The action of the rectus femoris muscle following distal tendon transfer: does it generate knee flexion moment?
Authors: Riewald Scott A. , Delp Scott L.
Published: July 2010

Rectus femoris transfer surgery involves detaching the rectus femoris from the patella and reattaching it posterior to the knee. While this procedure is thought to convert the rectus femoris from a knee extensor to a knee flexor, the moments generated by this muscle after transfer have never been measured. We used intramuscular electrodes to stimulate the rectus femoris in four subjects, two after transfer to the semitendinosus and two after transfer to the iliotibial band, while measuring the resultant knee moment.

Electromyographic activity was monitored in the quadriceps, hamstrings, and gastrocnemius muscles to verify that the rectus femoris was the only muscle activated by the stimulus. We found that the rectus femoris generated a knee extension moment in all of the subjects tested. This finding suggests that transfer surgery does not convert the rectus femoris to a knee flexor, and that a mechanism exists which may transmit the force generated by the rectus femoris anterior to the knee joint center after distal tendon transfer.
The relation between standing balance and walking function in children with spastic diplegic cerebral palsy
Authors: Liao Hua‐Fang , Jeny Suh‐Fang , Lai Jin‐Shin , Cheng Cheng Kung , Hu Ming‐Hsia
Published: July 2010

To establish and compare the relationship between standing balance and walking performance, eight children with spastic diplegic cerebral palsy (CF) and 16 non‐disabled, age‐ and sex‐matched children were studied. The results showed that the children with CP had worse static balance stability in various sensory environments and dynamic balance (rhythmic shifting ability) than the non‐disabled children. Moreover, the children with CP walked at a slower speed but at a greater physiological cost than the non‐disabled children. In the children with CP, dynamic balance significantly correlated with walking function. It is suggested that rhythmic weight‐shift training should be encouraged to improve the walking performance of children with CP.
Salla disease ‐ rare or underdiagnosed?
Authors: Robinson Richard O. , Fensom Anthony H. , Lake Brian D.
Published: July 2010

Salla disease is described in two English children. Eighty‐seven of the 89 cases so far reported come from Finland. It may be genuinely rare outside Finland or possibly underdiagnosed. Although a lysosomal disorder, it lacks many of their more characteristic features. Deterioration, for example, in the paediatric age range is rare. The clinical features are, however, consistent and specific. Definitive diagnosis is achieved by demonstrating increased amounts of free sialic acid in cultured skin fibroblasts. If the colorimetric method in widespread use is employed for this, a false negative result may be obtained. High‐pressure liquid chromatography is sufficiently sensitive. It is possible therefore that Salla disease is under‐reported, both from lack of clinical awareness and from lack of appropriate laboratory confirmation.
Intraoperative anaphylaxis due to allergy to latex in children who have cerebral palsy: a report of six cases
Authors: Delfico A J. , Dormans J P. , Crayhorne C B. , Templeton J J.
Published: July 2010

Healthcare professionals caring for children with cerebral palsy should lie aware of an association between it and latex anaphylaxis. Between 1989 and 1992 we had 22 patients with intraoperative anaphylactic reactions to latex at our institutions. Of these, six had cerebral palsy. The case histories of these six children are reported here. Most had had multiple operations and ventriculoperitoneal shunts. Preoperative recognition is key to prevention. If suspected from a patient's history, latex antibody testing is available. If needed, anesthesia for these individuals should be given using a latex‐free environment with or without medical prophylaxis.
Development and reliability of a system to classify gross motor function in children with cerebral palsy
Authors: Palisano Robert , Rosenbaum Peter , Walter Stephen , Russell Dianne , Wood Ellen , Galuppi Barbara
Published: July 2010

To address the need for a standardized system to classify the gross motor function of children with cerebral palsy, the authors developed a five‐level classification system analogous to the staging and grading systems used in medicine. Nominal group process and Delphi survey consensus methods were used to examine content validity and revise the classification system until consensus among 48 experts (physical therapists, occupational therapists, and developmental pediatricians with expertise in cerebral palsy) was achieved. Interrater reliability (k) was 0.55 for children less than 2 years of age and 0.75 for children 2 to 12 years of age. The classification system has application for clinical practice, research, teaching, and administration.
The outcome of walking in stable neuromuscular deficiencies
Authors: Commare MC , Miranda A. , Touzeau C. , Kassis B. , Dural‐Beaupère G.
Published: July 2010

In some patients with stable or very gradually worsening neuromuscular disorders, walking performance nevertheless decreases with increasing hip flexion and spinal deformity as the patient grows. The relations between muscular deficiency, pelvic and spinal deformity, head stability, gravit; parameters and walking performance were studied in 43 patients aged 18 months to 38 years with a view to finding out how these parameters are related, whether progressive spinal deformity and loss of walking can be avoided or delayed, and whether specific therapy for each of these parameters can help. Early combating of hip flexion deformity by physiotherapy, accompanied by limbering‐up exercises of the spine to counteract lumbar and thoracic lordosis are useful, as are orthoses to correct pelvic tilt anteversion, spinal lordosis and head instability. If physiotherapy is ineffectual or too late, tenoctomy of the rectus femoris may help.
Longitudinal study of neuropsychological outcome in blind extremely‐low‐birthweight children
Authors: Gosch Angela , Brambring Michael , Gennat Heike , Rohlmann Annette
Published: July 2010

This study evaluated neurological and psychological development in 10 blind children over a 4‐year period. Five of the children were born preterm with an extremely low birthweight (ELBW) and a diagnosis of retinopathy, whereas the other five were term. All children received their first neurological examination at a mean age of 10 months and then annual follow ups. In addition, the Bielefeld Developmental Test for Blind Infants and Preschoolers (BDTB) was administered every 6 months (from the ages of 18 to 48 months) to assess developmental outcome in different domains (e.g. cognition, language, gross motor abilities). Results showed a higher number of peri‐ and neonatal complications in blind ELBW children as well as a significantly higher number of neurological symptoms over the 4‐year period. At the mean age of 4 years 10 months, blind ELBW children had a significantly lower body weight, body height, head circumference, and body‐mass index compared with the term children. Findings on psychological development revealed that blind ELBW children also had significantly lower scores on all domains covered by the BDTB. Finally, the overall score on the BDTB correlated significantly with gestational age, birthweight, duration of mechanical respiration, and days spent in hospital after delivery.
The progression of untreated lumbar kyphosis and the compensatory thoracic lordosis in myelomeningocele
Authors: MD. Thomas Doers , MD Janet L Walker , MD Keith D van den Brink , MD. David B Stevens
Published: July 2010

Radiographs of 37 patients with untreated lumbar kyphosis without congenital vertebral anomalies associated with myelomeningocele were analyzed. With an average interval between radiographs of 6.2 years, the kyphosis was noted to increase at a mean rate of 4.3o per year without correlation to its initial magnitude. The compensatory lordosis was more variable and progressed at a mean of 2.5o per year. Children under the age of 2 years were more likely to increase the Cobb angle and the height of their kyphosis. There was an inverse relationship between the height of the kyphus and the lumbar spine height and the resultant growth of each. A modified kyphotic index less than 4. correlated with an increase in the curve and height of the kyphosis and the subsequent desire for surgery. Wide variablity in radiographic parameters make predictions for an individual patient difficult.
MRI findings in patients with spastic cerebral palsy. II: correlation with type of cerebral palsy
Authors: MD Akihisa Okumura , MD Toru Kato , MD Kuniyoshi Kuno , MD Fumio Hayakawa , MD Kazuyoshi Watanabe
Published: July 2010

The authors studied MR images of the brain in 152 patients, aged 1 to 19 years (mean 3.3), who had spastic cerebral palsy (CP) and were attending two hospitals in Japan in 1993 and 1994. Eighty‐one patients had diplegia, 45 had quadriplegia, and 26 had hemiplegia. Of patients with diplegia, 72 had periventricular leukomalacia (PVL) and very few had other types of lesions. In patients with quadriplegia, three main types of brain lesions were observed: PVL in 12 patients, term‐type brain injury in 22, and brain anomaly in 10. In the 26 patients with hemiplegia, 17 had a unilateral lesion (rare in patients with diplegia and quadriplegia), and bilateral lesions were seen in seven others.
How do children who are clumsy remember modelled movements?
Authors: BSc. Vickie Skorji , PhD. Beryl McKenzie
Published: July 2010

The ability of children who are clumsy, and of control children, to reproduce short sequences of simple movements immediately, and after a delay of 15 seconds, was examined. Four kinds of interference were introduced during the delay. These were visual or kinaesthetic each with either a high or a low spatial component. The reproductions of the movements by clumsy children were inferior to those by control children only after visual interference with a high spatial involvement It was concluded that the memory of clumsy children for modelled movements is more dependent on visuospatial rehearsal than is the memory of normal children.
Tactile recognition in infantile nephropathy cystinosis
Authors: Colah Serena , MD. Doris A Trauner
Published: July 2010

Previous studies have demonstrated that individuals with cystinosis, an inherited metabolic disorder, have difficulty processing visual information, and may be selectively impaired in the ability to mentally rotate figures, despite having normal IQs and normal primary sensory function. In our novel task ‐ the ‘Black Box’ ‐ subjects identified objects solely by feeling the contours. Twenty‐three subjects with cystinosis, aged 4 to 34 years, were individually matched with controls on age, sex, handedness, and test form. Subjects with cystinosis performed significantly worse in identifying objects than did controls. In addition, when only subjects over 7 years of age were included, those with cystinosis took significantly longer to correctly identify objects than did controls. Our findings suggest that individuals with cystinosis have difficulty with tactile recognition of common objects. These results support the hypothesis that a genetic disorder may have specific behavioral correlates.
Severity of brain injury following neonatal extracorporeal membrane oxygenation and outcome at age 5 years
Authors: Glass Penny , I Bulas Dorothy , E Wagner Ann , R Rajasingham Shusila , A Civitello Lucy , HPapero Patricia , E Coffman Cara , L Short Billie
Published: July 2010

Neurodevelopmental evaluation in childhood provides an opportunity to study complex neurological compensation following documented neonatal brain injury, and furnishes important clinical information which may have an impact on patient care. We studied 152 term children treated with extracorporeal membrane oxygenation (ECMO) as neonates and who received routine neonatal neuroimaging and comprehensive neurodevelopmental evaluation at age 5 years. The cohort was divided into four groups based on an independent neuroimaging score: No lesion, N=88; Mild lesion, N=38; Moderate lesion, N= 12; and Severe lesion, N=14. Standardized testing at age 5 included complete neuropsychological assessment, neurological evaluation, and assessment of motor function. All testing was conducted without knowledge of the neuroimaging score. The occurrence of disability by severity of neuroimaging was: No lesion=10%; Mild=13%; Moderate=33%; Severe=57%. The relative risk within the ECMO population for disability at age 5 after moderate or severe neonatal lesion was 4.3 (CI=1.0 to 17.5) and 11.7 (CI=3.3 to 41.3), respectively. The remaining non‐disabled children who had moderate to severe lesions functioned within normal limits. Severity of neonatal neuroimaging was inversely associated with IQ scores, pre‐academic skills, and neuromotor function. The effect size was small but the rank order was predictable. Our data identify in 5‐year‐old children an impact of brain lesion severity demonstrated on routine neonatal neuroimaging. The results indicate potential compensation following moderate and severe lesions, and suggest a subtle but consistent influence of even mild neonatal brain injury.
The importance of prenatal factors in childhood blindness in India
Authors: Rahi J S. , Sripathi S. , Gilbert C E. , Foster A.
Published: July 2010

The causes of visual loss in 1411 children attending schools for the blind in different geographical areas in India are. described. Ninety‐three percent (1318) of the children were severely visually impaired (SVI) or blind (i.e. corrected acuity in the better eye of <20/200 [<6/60]). In 60% of SVI/blind children vision loss was attributable to factors operating in the prenatal period, in 47% the prenatal factors were known and definite, and in 13% prenatal factors were the most probable causes. Hereditary retinal dystrophies and albinism were seen in 19% of SVI/blind children and 23% had congenital ocular anomalies. There were variations in the relative importance of different causes by state. The observed pattern of causes of visual loss is intermediate between those seen in industrialised countries and in the poorest developing countries. This suggests that strategies to combat childhood blindness in India need to address concurrently both preventable and treatable causes. The need for aetiological studies, particularly on anophthalmos and microphthalmos, is highlighted.
EEG and evoked potentials in infantile neuronal ceroid‐lipofuscinosis
Authors: Vanhanen Sanna‐Leena , Sainio Kimmo , Lappi Marjatta , Santavuori Pirkko
Published: July 2010

Sixteen children with infantile neuronal ceroid‐lipofuscinosis (INCL), age range 0.5 to 5.4 years, were studied using EEG, electroretinograms (ERG), visual evoked potentials (VEP) and somatosensory evoked potentials (SEP).

Electroencephalography was the first of these examinations to reveal abnormalities, however the EEG may be normal at the preclinical stage. The first abnormality to appear was an attenuated reaction to passive eye opening and closing which was followed by disturbances in background activity and diminution in amplitude, and by disappearance of sleep spindles. The gradual disappearance of posterior rhythm reactivity and of sleep spindles suggests that thalamic dysfunction progresses with time. EEG inactivity appeared by the age of 3 years. Evoked potentials were normal in the early stages of the disease. SEP showed abnormalities at Stage 2 (1.7 years), while ERG and VEP abnormalities appeared at Stage 3 (by the age of 2.5 years). All neurophysiologies! reactions examined were abolished by the age of 4 years. Follow‐up EEG gives important hints as to the early diagnosis of INCL. Progression of the disease can be followed by evoked potentials which may also be helpful in the differential diagnostics.
Soleus muscle length, stretch reflex excitability, and the contractile properties of muscle in children and adults: a study of the functional joint angle
Authors: Lin Jean‐Pierre , Brown J Keith , Walsh E Geoffrey
Published: July 2010

The influence of the joint angle on stretch reflex excitability of the soleus muscle at the ankle has been studied in 22 children aged 3.9 to 13.6 years and 9 adults aged 19 to 70 years. For all subjects, reflex EMG and mechanical twitch torque gain were trivial at resting plantar flexion. The reflex EMG gain reached a maximum between‐15o and‐10o of plantar flexion beyond the neutral angle, 0o, denned as the foot at right angle to the tibia, diminishing steeply with further dorsiflexion. The reflex mechanical gain rose to a peak between 0o and +10o of dorsiflexion beyond neutral, declining steeply thereafter. By contrast, axonally stimulated muscle twitch torque increased serially up to +30o dorsiflexion beyond neutral. For the soleus muscle, the optimal reflex neuromechanical angle lies approximately midway between the angle for optimal reflex EMG gain (in mild plantar flexion, at which the largest and strongest motor units can be activated) and the optimal muscle mechanical angle (at the extreme of soleus muscle dorsiflexion). These studies confirm that the excitability of the spinal alpha motor neuron pool in vivo is strongly influenced by muscle length and explain the variability in reflex excitability within and between subjects, if the joint angle is not controlled. They also indicate how posture influences movement, agreeing with the known function of the soleus muscle in the stance phase of gait and the modulation of motor unit recruitment during voluntary alternating movements at the ankle. Soleus muscle twitch characteristics show a fivefold to eightfold increase in peak force associated with a tenfold reduction in compliance in the first two decades of life and an apparent speeding up of twitch time in the first decade.
The epidemiology and clinical manifestations of hamstring muscle and plantar foot flexor shortening
Authors: Jóźwiak Marek , Pietrzak Szymon , Tobjasz Franciszek
Published: July 2010

A population of 920 healthy children was studied with the aim of assessing the incidence of hamstring muscle and plantar foot flexor tightness, and to correlate such symptoms with gait, posture, and low back discomfort or pain. Special attention was paid to the popliteal angle and dorsal foot flexion. The borderline values for the popliteal angle in the following age groups were, boys: 3 to 5 years, 40o; 6 to 15 years, 50o; and 16 to 19 years, 40o; girls: 3 to 5 years, 30o; 6 to 14 years, 45o; 15 to 19 years, 30oo. The borderline values for dorsal foot flexion in the following age groups were 3 to 4 years, 7o; 5 to 13 years, 10o; and 14 to 19 years, 5o. The results obtained indicate a natural increase in hamstring tightness, particularly shortly before the pubertal growth spurt. This seems to be linked with the natural evolution of lumbar lordosis and pelvic tilt. When hamstring tightness surpassed borderline values, dorsiflexion and lumbar lordosis decreased leading to postural deformities, bending‐forward deficit, discomfort when sitting, and a shambling gait.
Intrapartum fetal asphyxia and the occurrence of minor deficits in 4‐to 8‐year‐old children
Authors: Handley‐Derry Mark , A Low James , Burke Sharon O. , Waurick Meg , Killen Helen
Published: July 2010

Term newborn infants, 48 of whom had mild intrapartum fetal asphyxia, and 43 controls were assessed between 4 and 8 years of age for evidence of selective brain damage and minor deficits. Measures of motor and cognitive development were obtained at 4 and at 6 to 8 years of age. Memory tests, including the Alternation Test and determinations of Extended Digit Span, Spatial Memory, and Paired Associate Learning, were given annually between 4 and 8 years of age. Assessments of the family, child health, and child behaviour were obtained. There were no differences between the asphyxia and control groups for motor or cognitive development or the serial tests of memory. The two groups did not differ in regard to family characteristics, child health, or child behaviour. These findings indicate that term newborn infants exposed to mild intrapartum fetal asphyxia do not have minor motor and cognitive deficits later in childhood.
Personality characteristics and behaviour problems in individuals of different ages with Williams syndrome
Authors: Gosch Angela , Pankau Rainer
Published: July 2010

To study personality characteristics and behaviour problems in different age groups more precisely, the parents of 105 children with Williams syndrome (WS) were asked to complete a questionnaire with a list of 25 personality adjectives and 18 behaviour disturbances. Three age groups – children under 10 years, adolescents between 10 and 20 years, and adults over 20 years of age – were compared. Adults with WS were described as being less lively, determined, active, restless, tearful, quarrelsome, impertinent, and over‐friendly in comparison with children with WS. Additionally, adolescents and adults were assessed as being better balanced and more withdrawn than children with WS. Females were found to be less cheerful and happy as well as more tearful and quarrelsome than males, but these results showed only a statistical tendency. A discriminant analysis was performed to prove whether the three age groups could be discriminated on the basis of personality aspects. The results showed correct classification to one of the three age groups in 86% of the individuals with WS. The most discriminating adjectives were active, lively, well balanced, withdrawn, being over‐friendly, and vigorous. No differences regarding age or sex were found after calculating a composite score of behaviour problems reported in each individual. However, a comparison of single behaviour problems showed a decrease in external aggressive behaviours and greater depressive symptoms with increasing age.
Pontocerebellar hypoplasia with microcephaly and dyskinesia: report of two cases
Authors: Barbot Clara , Carneiro Gisela
Published: July 2010

We present two clinically diagnosed cases of pontocerebellar hypoplasia with microcephaly and dyskinesia (pontocerebellar hypoplasia type 2) from two different Portuguese families. Both children presented neurological involvement from birth, progressive microcephaly, exuberant chorea and dystonia, myoclonic jerks, pontocerebellar hypoplasia, and progressive cerebral cortical atrophy. One child had consanguineous parents.
Megalencephalic leukoencephalopathy: a further case of a new neurodegenerative white matter disease
Authors: Mejascar;ki‐Bošnjak Vlatka , Bešenski Nada
Published: July 2010

The case is presented of a 4.5‐year‐old boy with cystic megalencephalic leukoencephalopathy who met the diagnostic criteria of a recently described neurodegenerative white matter disorder, i.e. leukoencephalopathy with swelling and a discrepancy mild clinical course (van der Knaap 1995). He demonstrated an extremely mild and slowly progressive clinical course with near normal psychomotor development, particularly of mental functions, which contrasted with somewhat disturbed gross and fine motor skills. Repeated CT and MRI scanning showed extensive hemispheral cerebral white matter changes and demonstrated predominant frontal involvement of the periventricular and subcortical white matter. MRI was more sensitive in the detection of preserved structures, i.e. occipital subcortical and central white matter, as well as in the visualization of swelling and cystic lesions in the tip of the temporal lobes, which represent the hallmark of this entity. Thus, MRI is an essential diagnostic tool for this entity.
The influence of forearm crutches on pelvic and hip kinematics in children with myelomeningocele: don't throw away the crutches
Authors: Vankoski Stephen , Moore Carolyn , Statler Kimberly D. , Sarwark John P. , Dias Luciano
Published: July 2010

Gait analysis was performed on 16 children with high‐sacral‐level myelomeningocele who walked with and without crutches to evaluate the influence of crutches on their unique walking pattern. All of the patients used solid ankle‐foot orthoses (AFOs). Deviations in coronal and transverse planes improved with assisted walking. The timing of stance phase pelvic depression and the magnitude of stance phase hip abduction improved with crutch walking. Pelvic rotation, which was seven times the normal range of motion during no‐crutch walking, decreased to four times normal with crutches. Walking velocity was not significantly different between conditions. The results demonstrated that deviations in pelvic and hip kinematics are related to muscle weakness and improve with cratch use. Crutches enable the patient to transfer some weight bearing to their upper extremities which decreases the demand on weak lower‐extremity musculature. This allows them to maintain functional ambulation with a closer to normal gait pattern.
Relations between family environment and adjustment outcomes in young adults with spina bifida
Authors: Loomis James W. , Javornisky J Gregory , Monahan James J. , Burke Georgine , Lindsay Anne
Published: July 2010

Thirty‐two young adults with spina bifida completed a questionnaire (Family Environment Scale) assessing their perceptions of family social environment while growing up. Additionally, subjects responded to a structured interview addressing their current employment status, residential situation, level of community mobility, and extent of social activity. Multiple logistic regression analyses were used to assess the relation between family environment and adjustment as a young adult. With this limited sample, results indicated that perceived family environment explained variance in employment, community mobility, and social activity as an adult, even beyond that explained by lesion level and intelligence. Regression coefficients showed positive relations between perceived family encouragement of independence and achievement and young adult outcomes. In contrast, perceived moral/religious emphasis of the family and degree of family involvement with intellectual/cultural activities evidenced negative relations with the measures of young adult adjustment.
Inter‐ and intrasensory modality matching in children with hand‐eye coordination problems: exploring the developmental lag hypothesis
Authors: Sigmundsson H. , Ingvaldsen R P. , Whiting HT A.
Published: July 2010

This study set out to explore the suggestion that the problems experienced by 8‐year‐old children diagnosed as clumsy in the area of hand‐eye coordination (HECP) might be attributed to a developmental lag. The performances of this group of HECP children were compared with those of groups of 5‐year‐old and 8‐year‐old controls without such deficits, when required to carry out a task involving pointing, without vision, to targets located, visually, visually/proprioceptively, or proprioceptively, the dependent variable being the distance error score from the centre of the target. The performances of the HECP children, when vision or vision/proprioception was used to locate the targets, were shown to be inferior to those of the two control groups of children thereby supporting a visual deficit hypothesis. When the targets had to be located proprioceptively, the performance of the HECP children was shown to be similar to that of the 5‐year‐olds, while both groups were inferior to the 8‐year‐olds, thereby supporting a developmental lag hypothesis in proprioceptive terms. However, when the scores for the preferred and non‐preferred hands were analysed separately a marked deterioration in the performances of both the 5‐year‐old controls and the HECP children was observed while the 8Jyear‐old controls were unaffected. While this finding supports a developmental lag explanation of the inferior performances of the HECP children, it was necessary to qualify such an explanation when the within‐group performances using the preferred and non‐preferred hands were compared. Only the HECP children, under the visual/proprioceptive or proprioceptive conditions, showed significant performance differences, in. favour of the preferred hand. This finding was taken as a suggestion that the developmental lag exhibited by the HECP children might have pathological overtones possibly related to the development of the corpus callosum.
Changes in prevalence, aetiology, age at detection, and associated disabilities in preschool children with hearing impairment born in Göteborg
Authors: Darin Niklas , Hanner Per
Published: July 2010

The prevalence of hearing impairment (HI) in preschool children born in Göteborg in 1980 to 1984 was 2.0 per 1000. This was significantly lower than the 3.8 per 1000 found in a previous study for 1970 to 1974. The decrease took place in the moderate to severe range of HI, while deafness and mild HI remained unchanged during the period. The cause was considered to be prenatal in 58% with heredity in 33% as the main causative factor. Following the introduction of the MPR (Morbilli‐Parotitis‐Rubella) vaccination programme in Sweden, no cases of rubella‐ or mumps‐induced HI could be found. The number of HI of perinatal origin decreased by half, in spite of the fact that the figures for preterm baby survival almost doubled during the period. Associated disabilities were diagnosed in 62% of the children with HI; speech retardation in 33%, visual abnormalities in 30%, mental retardation in 12% and neuropsychiatric disorders in 9% of the cases. The importance of collaborative efforts between the otolaryngologist and the neurologically and neuropsychiatrically interested paediatrician in the complete evaluation of additional difficulties in the HI child is emphasized.
Children's attitudes toward peers with disabilities: the Israeli perspective
Authors: Tirosh Emanuel , Schanin Michael , Reiter Shunit
Published: July 2010

Attitudes toward peers with disabilities were compared in two groups of elementary school children, Israeli (2845 children) and Canadian (1831 children), using the Chedoke‐McMaster Attitudes Toward Children with Handicaps (CATCH) scale. Israeli children expressed significantly more positive general attitudes (P=0.0001). The more favourable attitudes were seen in both areas evaluated by the CATCH, the cognitive and the affective‐behavioural. In both groups, children who had had previous experience with a disabled person expressed more positive attitudes (P=0.001). An effect of gender was seen in the Canadian but not in the Israeli children. Cultural factors appear to play a role in modelling children's attitudes toward their disabled peers and therefore should be considered before educational programmes are implemented.
Biotinidase deficiency: two cases of very early presentation
Authors: Andersen Jesper Brandt , Blichfeldt Susanne , Christensen Mogens Fjord , Haagerup Annette
Published: July 2010

Two infants with early presentation of biotinidase deficiency (age 3 weeks and 2 weeks) are described. On admission, both children had severe neurological symptoms. In the first patient, magnetic resonance imaging (MRI) of the brain showed frontal and temporal atrophy, and in the second patient, CT of the brain showed diffuse periventricular hypodensities, particularly in the frontal region. Oral treatment with biotin (15mg and 10mg per day respectively) made all symptoms disappear within a few weeks. On follow‐up 13 and 16 months later, both children were still asymptomatic on this treatment. Their psychomotor development was normal. MRI and CT of the brain had normalized. Later, a moderate hearing loss was detected in the first patient.

In biotinidase deficiency, early diagnosis and treatment with oral biotin are essential in order to prevent irreversible damage to the central nervous system and early death from metabolic acidosis. Neonatal screening for biotinidase deficiency would fulfil this goal.
Life expectancy of adults with cerebral palsy
Authors: FASA David Strauss PhD , PhD Robert Shavelle
Published: July 2010

To determine the predictors of mortality and find the life expectancies of adults with cerebral palsy (CP), data on 24768 individuals aged 15 years and over who received services in California between January‐1980 and December 1995 were analyzed. Multivariate statistical methods to identify mortality predictors, and actuarial methods to determine corresponding life expectancies were used. The key predictors were lack of basic functional skills: mobility and feeding. Adults lacking these skills had much reduced life expectancies, as short as 11 years for the worst functioning groups. By contrast, survival of high‐functioning adults was close to that of the general population. The influence on survival of cognitive skills, type of CP, and other factors appears to be expressed largely through their effect on basic functioning. Life expectancies of adults of a given age can differ by 40 years or more, according to their functional level.
Survival in cerebral palsy: the role of severity and diagnostic labels
Authors: FRACP Katrina Williams , FFPHM Eva Alberman FRCP , Emeritus Professor
Published: July 2010

The aim of this study was to review survival and certified causes of death of 584 children on a population‐based register of cerebral palsy (CP), and to assess the impact on these of an additional diagnostic label. The register, established in 1985, comprised children with CP born from 1980 to about 1987 who were resident at the time of notification in the south‐east of England in a region with between 3 and 4 million population, defined by the boundaries of the regional health authority (North East Thames Regional Health Authority). The current report concerns deaths of residents born between 1980 and 1986, who had been registered but had later died, as well as of eligible children who had not been registered while alive but whose cause of death was CP. These were identified at the Office of Population Censuses and Surveys who also supplied copies of death certificates for this study. For children notified while alive, information about motor severity and other diagnostic labels was sought at entry to the register and again between 3.5 and 4 years and between 7 and 8 years. For this study, children known to have a postneonatal onset, a progressive or non‐cerebral cause of motor signs, or minimal motor involvement were excluded. Thirty‐nine of 584 children included in this study had died by the end of 1995. No deaths had occurred in children known to have less than four‐limb involvement. Survival of the group known to have an additional diagnostic label was significantly lower (86.2%) than that of the group with no known label (96.3%; P=0.01), and remained lower, although not significantly, if only those with severe four‐limb involvement were compared (75.3% versus 92.4%; P=0.2). The greater severity of limb involvement in those with an additional diagnosis may not account for this difference. Of the 37 children with death certificates available, CP was mentioned as a cause in only 24.
Speech and language development of children born at 32 weeks' gestation: a 5‐year prospective follow‐up study
Authors: Luoma Laila , MD Eila Herrgård , MD Anneli Martikainen , PhD Timo Ahonen
Published: July 2010

Speech and language comprehension and production were assessed at the age of 5 years in a cohort of children born preterm at 32 weeks' gestational age (N=55) in comparison with children born at term and of similar age, sex, and social backgrounds. Data both including and excluding major neurological disabilities are presented. Mean performance for the entire group of preterm children was significantly lower than for the controls on most of the measures including the composite IQ scores. When the nine children who had major neurological disabilities were excluded from the preterm “group, statistically significant differences were found on four of the total 12 speech and language measures. Intellectually normal preterm children without major neurological disability were slower than the controls on rapid word retrieval. In addition, difficulties in comprehending relative concepts were typical for the preterm children. The results suggest ‘subtle dysnomia’, which is indicative of later reading problems. On global verbal measures and on the basic speech and language aspects the study groups did not differ. Specific language impairment, defined as a discrepancy of ID between Performance IQ and Verbal IQ scores, showed a tendency to be more common in the control group. Within both the study groups, the boys showed a tendency for a greater discrepancy between their Performance and Verbal IQ scores.
The predictive value of developmental testing of extremely jaundiced African infants
Authors: PhD M J Wolf , MD B Wolf , PhD C Bijleveld , PhD G Beunen , PhD P Casaer MD
Published: July 2010

The predictive value of the Neonatal Neurological Examination (NNE) adapted from Prechtl (1977) and the Infant Motor Screen (IMS) from Nickel (1989) at 4 months was studied in severely jaundiced infants in Zimbabwe. Fifty infants were examined with the NNE, 41 with the IMS and 43 with the Bayley Scales of Infant Development (BSID) (Bayley 1969). Five infants had choreoathetosis and six had a motor delay at age 1 year. The NNE and IMS proved to be sensitive instruments particularly when two infants who became malnourished after the neonatal period were excluded. Logistic regression was used to investigate the relation between the BSID and five selected predictors from the NNE. This resulted in a correct classification of 93%. By using only the predictors acoustic blink and traction response, 80% of the infants were correctly classified but the number of false negatives was reduced from three to one.
Turkish children's performance on Denver II: effect of sex and mother's education
Authors: Duimazlar Nezih , Ozturk Çagatay , Ural Birgül , Karaagaoglu Ergun , MD Banu Anlar
Published: July 2010

One thousand and ninety‐one healthy Turkish children were tested with the Denver II developmental screening test and the data were analysed for sex and sociocultural differences. Few and inconsistent differences were observed between boys and girls. On the other hand, there were marked differences between sociocultural groups, particularly in terms of fine motor and language areas and in older preschool children. The effect of maternal education on the child's development is more important in countries where preschool education is not commonly available. Whether this effect diminishes after 1 or 2 years of schooling is to be investigated.