bluuebunny/crossref_metadata_2025 huggingface dataset:
Fine and gross motor ability in males with ADHD
Authors: Pitcher Thelma M , Piek Jan P , Hay David A
Published: July 2010

In the this study, both fine and gross motor ability of males with attention‐deficit‐hyperactivity disorder (ADHD) were compared with a group of control children. Three groups of males with the following ADHD subtypes: predominantly inattentive (ADHD‐PI; n=50), hyperactive/impulsive (ADHD‐HI; n=16), or combined (ADHD‐C; n=38) were compared with 39 control males. Mean ages for the control group were 10 years 4 months (SD1 year 4 months, range 7 years 8 months to 12 years 11 months); for the ADHD‐PI group, 10 years (SD 1 year 2 months, range 7 years 10 months to 13 years); for the ADHD‐HI group, 9 years 11 months (SD 1 year 2 months), range 7 years 11 months to 12 years 6 months); and for the ADHD‐C group 10 years 2 months (SD 1 year 4 months, range 8 to 13 years). The Australian Disruptive Behaviours Scale and Connors’Parent Rating Scale‐Revised were used to assess ADHD symptomatology. Verbal IQ was estimated using two verbal subtests of the Wechsler Intelligence Scale for Children, and movement ability was assessed using the Movement Assessment Battery for Children (MABC) and the Purdue Pegboard test. Findings demonstrated that the children with ADHD had significantly poorer movement ability than control children. A high percentage of these children displayed movement difficulties consistent with developmental coordination disorder (DCD). In addition, the current study found that the type and degree of movement difficulty differed between subtypes. The Total Impairment score, as derived from the MABC, was less severe for the ADHD‐HI group than the other two ADHD groups, but more severe than for the control group. Males with ADHD‐PI and ADHD‐C had significantly poorer fine motor ability (p<0.001) than control males, whereas the ADHD‐HI group did not differ significantly from any of the other groups. As children with ADHD only and the control group did not differ significantly on fine motor ability but were significantly better than children categorized with both ADHD and DCD, it was argued that poorer fine motor ability found in children with ADHD could not be attributed to deficits in attention and concentration, but rather to factors relating to their motor ability.
Development of visual perception and attention, assessed by backward masking and application in children with epilepsy
Authors: Macchi Marta , Rossi Livia Nicoletta , Cortinovis Ivan , Menegazzo Lucia , Carmen Claudia , Romeo Antonino , Vassella Franco
Published: July 2010

Visual masking assesses visual perception and attention; it occurs when a visual stimulus (mask) interferes with the perception of a stimulus that the participant is trying to identify (target). A backward masking study (target presented before mask) was perfor Med on 662 children without disabilities (338 females), aged between 6 and 17 years, in order to evaluate if performance varies with age. In the masking procedure 10 letters were presented through a tachistoscope as target stimuli. Fragments of letters oriented at random (‘noise’) represented the mask. A slight improvement of visual performance from the beginning of school age to 9–12 years of age was found. This paper gives normative data for the most important parameters which can be used as a standardized reference for the procedure employed. We also studied 113 children with epilepsy (56 females), aged between 5 and 19 years, who attended a mainstream school and had been seizure free for at least 2 years. Children were tested just before starting antiepileptic drug withdrawal and re‐tested 1 year later; they were drug free for 3 months before the second test. These children showed, during and after treatment, only slightly worse results when compared with healthy children of the same age; after therapy withdrawal, their visual performance slightly improved but this was not statistically significant.
Combination of caudal myxopapillary ependymoma and dermal sinus: a single shared embryologic lesion?
Authors: Wolf Nicole I , Harting Inga , Aschoff Alfred , Sommer Clemens , Ebinger Friedrich
Published: July 2010

A female child presenting with acute flaccid paraparesis at 18 months was found to have a dermal sinus in combination with a dermoid cyst and a myxopapillary ependymoma of the cauda equina and conus Medullaris. A possible embryologic relation between these lesions is discussed.
Effect of balance training on recovery of stability in children with cerebral palsy
Authors: Shumway‐Cook Anne , Hutchinson Susan , Kartin Deborah , MSME Robert Price , Woollacott Marjorie
Published: July 2010

This study examined the effect of massed practice in balance recovery of stability in six children (four males, two females; mean age 9 years 2 months, SD 2 years, range 7 years 5 months to 12 years 11 months) with cerebral palsy (CP). Four children were diagnosed with spastic diplegia (Gross Motor Function Classification System [GMFCS] level II) and two with spastic hemiplegia (GMFCS level I). A single‐subject, multiple‐baseline experimental design involving three pairs of children matched for diagnosis was used. A moveable forceplate system was used to test and train reactive balance control. Area per second (i.e. area covered by the center of pressure over a one second period) and time to stabilization from center of pressure measures were calculated following perturbations. The intervention phase consisted of massed practice on the moving platform (100 perturbations/day for 5 days). Analysis included hierarchical linear modeling and a repeated measures ANOVA. All children demonstrated a significant improvement in their ability to recover stability as demonstrated by reduced center of pressure area and time to stabilization following training. These improvements were still present 30 days following completion of training. Results suggest that postural control mechanisms in school‐age children (7 to 13 years) with CP are modifiable.
Pompe disease and physical disability
Authors: Haley Stephen M , Fragala Maria A , Skrinar Alison M
Published: July 2010

This study describes the physical disability of 30 children and adolescents with Pompe disease (23 males, 7 females; mean age 7 years 7 months, SD 5 years 6 months; range 6 months to 22 years 1 month) using a disease‐specific functional instrument. Data were collected by telephone interview with parents using a modified version of the Pediatric Evaluation of Disability Inventory. The sample included mostly males of Caucasian origin, recruited from several countries. Disability profiles in mobility and self‐care skills were heterogeneous because functional status was not related to chronological age. Most children had severe functional deficits: nearly two‐thirds of the sample was non‐ambulatory and could not perform age‐expected self‐care skills. Three‐quarters of the children used a ventilator. Two children were able to participate in age‐appropriate sports and peer activities. Although the mean chronological age of the sample was 7 years 7 months, the mean age‐performance for self‐care skills was under 2 years 6 months and under 1 year 6 months for mobility. Implications of physical disability findings for individuals with Pompe disease are discussed.
Reliability of classification of cerebral palsy in low‐birthweight children in four countries
Authors: Paneth Nigel , Qiu Hong , Saigal Saroj , Bishai Sharif , BS James Jetton , Den Lya , Broyles Sue , Tyson Jon , Kugler Karl
Published: July 2010

The reliability of classification of cerebral palsy (CP) in low‐birth weight children was assessed by using clinical and research study records sampled from population‐based cohort studies in the USA, the Netherlands, Canada, and Germany. Records of neurological examination findings and functional motor assessments were submitted to up to five pediatricians with expertize in CP diagnosis, who grouped children into categories referred to as‘disabling’CP,‘non‐disabling’CP, and no CP. Each study provided between 31 and 51 records of children assessed between 2 and 8 years of age, approximately equally divided among the three groupings. The discrimination between‘any CP’and‘no CP’was only fair (mean Kappa coefficients 0.37 to 0.69). However, when more detailed information describing motor function was used, children with‘disabling’CP could be distinguished, on the basis of records, from those without CP or with‘non‐disabling’CP with good to excellent reliability (mean Kappa coefficients 0.69 to 0.88). Because of the substantially higher agreement observed when these functional distinctions are made, we recommend that reports or comparisons of rates of CP should include levels of motor function of children with CP, and not simply total CP, among the outcomes of interest.
Acute bilateral thalamic necrosis in a child with Mycoplasma pneumoniae
Authors: Ashtekar CS , FRCP T Jaspan , FRCPCH D Thomas , MRCP V Weston , Gayatri N A , Whitehouse WP
Published: July 2010

A previously neurodevelopmentally intact 5–year‐old male was admitted to hospital with a right lower lobe pneumonia with pleural effusion, subsequently confirmed to be a Mycoplasma pneumoniae infection. On the seventh day of the illness he had a prolonged generalized tonic or tonic‐clonic convulsion, requiring intubation and ventilation. He was slow to regain consciousness (Child's Glasgow Coma Score 7–10 over 6 days) and brain imaging with CT and then MRI demonstrated bilateral thalamic lesions with oedema and central haemorrhage suggestive of acute bilateral thalamic necrosis, without striatal or white‐matter involvement. He was treated with a 2–week course of erythromycin, and as an autoimmune process was considered possible, 5 days of intravenous methylprednisolone (20mg/kg/day) followed by a 4–week oral prednisolone taper. He made a slow recovery over the next few weeks with almost complete neurological recovery by 2 months but with significant dysarthria, drooling, and a mild left hemiparesis. At 9 months, significant dystonia continued to affect his speech and, together with tremor, his upper‐limb fine motor function bilaterally. His gait, personality, and higher cognitive functions appeared to have recovered fully. Although acute striatal necrosis, acute disseminated encephalomyelitis, and encephalitis have been reported with Mycoplasma pneumoniae and a similar picture of acute bilateral thalamic necrosis with influenza‐A (‘acute necrotizing encephalopathy’), this is the first reported case of Mycoplasma pneumoniae‐associated isolated acute bilateral thalamic necrosis.
Disturbances in programming goal‐directed arm movements in children with ADHD
Authors: Eliasson Ann‐Christin , Rösblad Birgit , Forssberg Hans
Published: July 2010

We investigated in children with attention‐deficit‐hyperactivity disorder (ADHD) the ability to programme and execute goal‐directed arm movements. The sample consisted of 25 males with ADHD (mean age 11 years 6 months, SD1 year 11 months, range 8 to 15 years) and 25 age‐matched typically developing males. The children moved a cursor on a screen by moving a hand‐held indicator on a horizontal digitizing tablet. Start and target positions on the screen were always visible during the movement. The screen cursor, however, could either be visible throughout the movement (visual feedback) or blanked at movement initiation (without visual feedback). Analysis showed that movement control was impaired in children with ADHD and that their problems were especially pronounced during the without‐visual‐feedback condition. In this condition, the children with ADHD exhibited large end‐point errors and prolonged movement durations. As there can be no visual corrections of the movement during this condition, results indicate poorer motor programming in children with ADHD. Moreover, children with ADHD performed jerky movements and showed a reduced capacity to select a movement speed that met with the accuracy demands of the movement.
Control of reaching movements in children and young adults with myelomeningocele
Authors: Norrlin Simone , Dahl Margareta , Rösblad Birgit
Published: July 2010

The aim of the present study was to analyze the ability to programme and execute reaching movements in individuals with myelomeningocele (MMC) and in a control group. Thirty‐one participants (18 males, 13 females; mean age 12 years 11 months, SD 2 years 7 months, range 9 to 19 years) with MMC and 31 participants (matched for age and sex) without disabilities were investigated. Reaching was performed with and without visual feedback toward three targets displayed on a computer screen and data were collected using a digitizing tablet linked to a computer. The kinematics of reaching were analyzed and analysis of variance was used for statistical analysis. Results showed that both groups were able to programme reaching movements under both visual conditions. Although the execution of reaching was poor in the MMC group compared with the control individuals, as indicated by larger end‐point errors (p=0.002), less straight movements (p=0.018), and shorter deceleration phases (p=0.004), movement time was not prolonged in the MMC group. Those with shunt treatment (n=21) had more difficulties when visual feedback was provided. Those with symptoms of early brainstem dysfunction (n=5) had shorter deceleration phases under both visual conditions.
Energy cost of walking in children with cerebral palsy: relation to the Gross Motor Function Classification System
Authors: Johnston Therese E , Moore Stephanie E , Quinn Lance T , Smith Brian T
Published: July 2010

This study compared the energy cost of walking in children with cerebral palsy (CP) classified at different levels of the Gross Motor Function Classification System (GMFCS) with that in children with typical development. Sixteen female and 14 male children with CP (mean age 9 years 6 months, SD 2 years 4 months, range 6 years 4 months to 13 years 4 months) and 14 male and 13 female typically developing children (mean age 10 years, SD 1 year 6 months, range 7 years 1 month to 12 years 11 months) participated. Children with CP were classified at GMFCS level I, n=5; level II, n=10; level III, n=9; and level IV, n=6. Energy cost was assessed by the gas dilution method as each child walked around an oval track wearing a dilution mask. Significant differences were found across GMFCS levels (p < 0. 0001) and between adjacent levels (p < 0.013). Children with CP displayed a higher energy cost of walking than the typically developing children (p < 0.0001). A strong correlation (0.87) was found between the energy cost of walking and GMFCS level (p < 0.01) when children with typical development were assigned a GMFCS level of zero to allow statistical analysis. This indicates increasing energy cost of walking with increasing severity of functional involvement. These differences in energy cost across GMFCS levels provide another distinguishing factor between GMFCS levels and further emphasize the importance of considering metabolic demand in determining treatment options.
Congenital trigeminal anaesthesia
Authors: Hashmi S J , Chow G , Bittner S B , Rittey C D , Williams LHP
Published: July 2010

Two patients with congenital trigeminal nerve anaesthesia are described. The first (male, aged 14 years) had an isolated unilateral loss of sensation in all three divisions of the trigeminal nerve with no other abnormalities. The second patient (male, aged 3 years 6 months) had bilateral loss of sensation in all three divisions of the trigeminal nerve, associated with other neurological abnormalities. No explanation for their abnormalities was found. The Rosenberg classification of congenital trigeminal anaesthesia is discussed with reference to these patients and also with reference to the developmental biology of the trigeminal nerve.
Impact of gastrostomy tube feeding on the quality of life of carers of children with cerebral palsy
Authors: Sullivan Peter B , Juszczak Edmund , Bachlet Allison ME , Thomas Adrian G , Lambert Bridget , Vernon‐Roberts Angharad , Grant Hugh W , Eltumi Muftah , Alder Nicola , Jenkinson Crispin
Published: July 2010

The aim of this prospective cohort study was to evaluate the impact of gastrostomy tube feeding on the quality of life of carers of children with cerebral palsy (CP). Short‐Form 36 version II was used to measure quality of life in carers of 57 Caucasian children with CP (28 females, 29 males; median age 4y 4mo, range 5mo to 17y 3mo) six and 12 months after insertion of a gastrostomy tube. Responses were calibrated against a normative dataset (Oxford Healthy Life Survey III). Six months after gastrostomy feeding was started, a substantial rise in mean domain scores for mental health, role limitations due to emotional problems, physical functioning, social functioning, and energy/vitality were observed. At 12 months after gastrostomy placement, carers reported significant improvements in social functioning, mental health, energy/vitality (mean increase >9.8 points;p<0.03), and in general health perception (mean increase 6.35 points;p=0.045) compared with results at baseline. Moreover, the values obtained for these domains at 12 months were not significantly different from the normal reference standard. Carers reported a significant reduction in feeding times, increased ease of drug administration, and reduced concern about their child's nutritional status. This study has demonstrated a significant, measurable improvement in the quality of life of carers after insertion of a gastrostomy feeding tube.
Is improved survival of very‐low‐birth weight infants in the 1980s and 1990s associated with increasing intellectual deficit in surviving children?
Authors: Hansen Bo Mølholm , Greisen Gorm
Published: July 2010

To investigate whether the decrease in mean birth weight of the smallest infants surviving with a very low birth weight (VLBW) in the past two decades is associated with an increase in intellectual deficit rates, we compared the results obtained in two different follow‐up studies: one included a cohort of children of VLBW (<1500g; n=102,52% male) born in the early 1980s; the other included a cohort of children of extremely low birthweight (ELBW; <1000g; n=183,46% male) born in the mid‐1990s. Intellectual development was evaluated in both cohorts. McCarthy Scales of Children's Abilities were used in the cohort from the early 1980s; Wechsler's Preschool and Primary Scale of Intelligence ‐ Revised was used in the cohort from the mid‐1990s. The children were assessed at age 4–5 years, and a control group was included for each of the two studies. Mean scores in the measures of intelligence found at the assessment were recalculated to standard deviation scores from the two control groups. We found no difference in the standard deviation scores between the two cohorts despite the mean birthweight being 353g (p<0.001) lower in the cohort from the mid‐1990s, and the overall survival rate of infants of VLBW having increased. Our results suggest that the improved survival of the smallest babies in the 1980s and 1990s has been achieved without an increase in the intellectual deficit in the surviving children.
Neurological recovery in obstetric brachial plexus injuries: an historical cohort study
Authors: Hoeksma Agnes F , Ter Steeg Anne Marie , Nelissen Rob G H H , Van Ouwerkerk Willem J R , Lankhorst Gustaaf J , De Jong Bareld A
Published: July 2010

An historical cohort study was conducted to investigate the rate and extent of neurological recovery in obstetric brachial plexus injury (OBPI) and to identify possible prognostic factors in a cohort of children with OBPI from birth to 7 years. All children (n=56; 31 females, 25 males) with OBPI were evaluated at fixed time intervals by one examiner. They underwent a final neurological examination at a mean age of 3 years 10 months (range 1 to 7 years). Neurological outcome was not as favourable as is often reported: complete neurological recovery occurred in 37 out of 56 children (66%). In half of these there was delayed recovery, in which case complete neurological recovery was assessed from 1.5 to 16 months of age (median age 6.5 months, SD 4.2 months). External rotation and supination were the last to recover and recovered the least. Although biceps function at three months was considered to be the best indicator for operative treatment, external rotation and supination were found to be better in predicting eventual complete recovery. Initial symptoms directly post partum were not found to be prognostic. Functional outcome was mainly reported to be good.
Electrostimulation at sensory level improves function of the upper extremities in children with cerebral palsy: a pilot study
Authors: Mäenpää Helena , Jaakkola Riitta , Sandström Marita , Von Wendt Lennart
Published: July 2010

The aim of this study was to evaluate the effect of electrical stimulation (ES) on the function of the upper extremities in children with cerebral palsy (CP). The participants were 12 children (seven females and five males) with spastic hemiplegia (mean age 5 years 7 months, SD 3 years 9 months). Indications were weak wrist dorsiflexion and elbow extension. The ES was given at sensory level (20–40 minutes) on the infraspinatus muscle and on the wrist dorsiflexors during 12 regularly scheduled physical and occupational therapy sessions (during 4–5 weeks). The Goal Attainment Scale, the Zancolli classification of hand function, muscle testing according to Daniels and Worthington, and King hypertonicity scale were used for evaluation. Assessments were made twice before (between 4 weeks) and twice after (between 12 weeks) the stimulation period except the King hypertonicity scale, which was used once before and 3 months after the stimulation period. Active elbow extension, wrist dorsiflexion, and forearm supination with the elbow flexed and extended improved when the results of assessments before ES were compared with those made immediately before (p < 0.001) and three months after (p < 0.01) this treatment. Results of this pilot uncontrolled study suggest that ES at sensory level can be used as an adjunct to physiotherapy and/or occupational therapy in children with spastic hemiplegia. These results will be used as basis for further research.
International Classification of Functioning, Disability and Health in a cohort of children with cognitive, motor, and complex disabilities
Authors: Battaglia Mariamalia , Russo Emanuela , Bolla Alessandra , Chiusso Alessio , Bertelli Stefano , Pellegri Alda , Borri Gabriella , Martinuzzi Andrea
Published: July 2010

The recently endorsed World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) is proposed as a new tool to describe human functioning and health in a multiprofessional approach for individuals of all ages. Its application to paediatric neurorehabilitation may be of great help, especially in children with complex disabilities. However, experience with its application in this field is still limited. We tested the application of ICF in 40 children (26 males, 14 females; age range 3 to 18 years; mean age 11 years 1 month, SD 5 years 3 months) with various types and degrees of disability. We tested the applicability and reliability of the classification, and studied its correlation with well‐established measures of functioning (Verbal IQ; gross motor function measure; functional independence measure). The ICF proved to be applicable and reliable, and strongly correlated with established scales. However, several of the Activity and Participation components do not fully capture the developmental nature of many abilities of children. Our study, although acknowledging the universal application of the ICF, and the ICF's value as a clinical tool, calls for its specific adaptation to accommodate better the peculiarities of child functioning and disability.
Effect of age at thyroid stimulating hormone normalization on postural control in children with congenital hypothyroidism
Authors: Gauchard Gérome C , Control Balance , Deviterne Dominique , Leheup Bruno , Perrin Philippe P
Published: July 2010

Neonatal screening programmes allow early treatment to limit the consequences of congenital hypothyroidism on maturation of the central nervous system, and on psychomotor and educational outcome. Consequences of age at thyroid stimulating hormone (TSH) normalization on postural control were evaluated in 17 children with congenital hypothyroidism (14 females, three males; median age 12 years 1 month, range 7 to 14 years) and in 11 control individuals (eight females, three males: median age 10 years 6 months, range 8 to 14 years). Children with congenital hypothyroidism were split into two groups according to time of TSH normalization: before (group 1) or after (group 2) 3 months of age. Posturography (static and dynamic tests), educational (school performance), and psychomotor (WISC and revised Bruininks Oseretsky tests) evaluations were carried out. Group 2 results showed significant abnormalities in postural, educational, and psychomotor aptitudes in comparison with group 1 and control children (stability, Bruininks‐Oseretsky, school performance:p≦0.05 or p≦0.01). Early TSH normalization is necessary to allow for normal development of the neurosensorial afferent pathways (vestibular, proprioceptive) and of central integration (cerebellum, vestibular nuclei).
Neuropsychological functioning in children with early‐treated phenylketonuria: impact of white matter abnormalities
Authors: Anderson Peter J , Wood Stephen J , Francis Dorothy E , Coleman Lee , Warwick Linda , Casanelia Sue , Anderson Vicki A , MD Avihu Boneh
Published: July 2010

Impact of white matter abnormalities (WMAs) on neuropsychological functioning in children with early‐treated phenylketonuria (ETPKU) was examined. Children with ETPKU (20 males, 12 females, mean age 11 years 2 months, SD 3 years 6 months) and controls (20 males, 14 females, mean age 10 years 4 months, SD 3 years 1 month) aged 7 to 18 years were assessed using tests of attention, processing speed, memory and learning, executive function, and academic achievement. Those with ETPKU, exhibiting WMAs extending into subcortical/frontal regions (n=14), displayed significant impairments in a number of domains. Children with ETPKU but no WMAs (n=6), or pathology restricted to the posterior periventricular region (n=12), displayed only mild deficits. Concurrent phenylalanine levels correlated weakly with cognitive parameters, whereas lifetime phenylalanine levels were associated with deficits in several cognitive domains. Impairments in children with extensive WMAs are consistent with compromised neural transmission, which is characterized by dysmyelination. However, children with no detectable, or mild WMAs, also displayed cognitive problems, indicating that neuropsychological functioning in children with ETPKU is determined by a complex interaction of biological and environmental factors.
Postural control during reaching in preterm children with cerebral palsy
Authors: Van Der Heide Jolanda C , Begeer Co , Fock Johanna M , Otten Bert , Stremmelaar Elisabeth , Van Eykern Leo A , Hadders‐Algra Mijna
Published: July 2010

Postural control during reaching with the dominant arm was assessed in 58 preterm children with cerebral palsy (CP) aged 2 to 11 years, comprising 34 with spastic hemiplegia (17 males, 17 females) and 24 with bilateral spastic CP (bilateral CP; 15 male, 9 females). Assessments were made by multiple surface electromyogram (EMG) and kinematic recording. Mean gestational age at birth for the children with spastic hemiplegia and those with bilateral CP was 28.6 weeks (SEM 0.33) and 28.2 weeks (SEM 0.34) respectively; their mean birth weights were 1158g (SEM 58) and 1190g (SEM 59) respectively. All but one of the children with spastic hemiplegia could walk without restriction, the exception being a child who had self‐mobility with limitations. In the group of children with bilateral CP, nine walked without assistive devices, 10 could walk with assistive devices, and five children needed a wheelchair for self‐mobility. Comparison data of 29 typically developing children (10 males, 19 females) born at term with appropriate birth weight were available. Results indicated that in most children with CP the basic level of postural control (‘direction‐specificity’, ie. muscle activation on the side opposite to direction of body sway) was intact. However, the children with CP showed dysfunctions in: (1) recruitment order of the postural muscles, i.e. they exhibited a stereotyped top‐down recruitment; and (2) the ability to modulate muscle contraction (that registers on EMG) to task‐specific conditions. The latter dysfunction was more pronounced in children with bilateral CP than in those with spastic hemiplegia. Postural dysfunctions were correlated to some extent with the degree of disability in everyday activities as assessed by the Pediatric Evaluation of Disability Inventory.
The Leg Drop Pendulum Test performed under general anesthesia in spastic cerebral palsy
Authors: Fee James W , Miller Freeman
Published: July 2010

The general aim of this study was to examine the stiffness and damping characteristics (rate at which oscillations diminish) of limbs of a small population of patients with spastic cerebral palsy (CP) during the Leg Drop Pendulum Test while under general anesthesia and to compare them with patients without CP, both awake and under similar anesthetized conditions. To facilitate a quantitative comparison the authors defined a set of parameters that reflect the stiffness and damping of limbs with spasticity under general anesthesia. To give these parameters more meaning a similar set was constructed for children without disabilities under conditions of normal wakefulness and under anesthesia. The test population of 10 patients with spasticity ranged in age from 4 to 19 years and had a mean age of 10 years 9 months (SD 5 years 10 months); there were four females and six males. The control population consisted of eight patients without disabilities who ranged in age from 7 to 19 years with mean age of 13 years 9 months (SD 3 years 3 months); there were six females and two males. To even out the populations, two limbs from the non‐affected sides of two patients with hemiplegic spasticity (both female) were used in the control group. The Leg Drop Pendulum Test was administered to 10 limbs with spastic CP and 10 control limbs under general anesthesia. The two test populations were chosen from patients who were previously scheduled to undergo surgery. Two measures compared limbs of those under anesthetic with those under alert conditions: the damped natural frequency and the damping ratio. The natural frequency is the frequency at which a pendulum oscillates as it comes to rest; the damping ratio is a measure of how quickly the oscillations diminish. In spasticity, active muscle tone disappeared under anesthesia. In both groups the underlying biomechanics of the limb varied considerably. In controls the variation seemed to be growth related.
Gross Motor Function Classification System and outcome tools for assessing ambulatory cerebral palsy: a multicenter study
Authors: Oeffinger D J , Tylkowski C M , Rayens M K , Davis R F , Gorton G E , D'Astous J , Nicholson D E , Damiano D L , Abel M F , Bagley A M , Luan J
Published: July 2010

The relationships between different levels of severity of ambulatory cerebral palsy, defined by the Gross Motor Function Classification System (GMFCS), and several pediatric outcome instruments were examined. Data from the Gross Motor Function Measure (GMFM), Pediatric Orthopaedic Data Collection Instrument (PODCI), temporal‐spatial gait parameters, and oxygen cost were collected from six sites. The sample size for each assessment tool ranged from 226 to 1047 participants. There were significant differences among GMFCS levels I, II, and III for many of the outcome tools assessed in this study. Strong correlations were seen between GMFCS level and each of the GMFM sections D and E scores, the PODCI measures of Transfer and Mobility, and Sports and Physical Function, Gait Velocity, and Oxygen Cost. Correlations among tools demonstrated that the GMFM sections D and E scores correlated with the largest number of other tools. Logistic regression showed GMFM section E score to be a significant predictor of GMFCS level. GMFM section E score can be used to predict GMFCS level relatively accurately (76.6%). Study data indicate that the assessed outcome tools can distinguish between children with different GMFCS levels. This study establishes justification for using the GMFCS as a classification system in clinical studies.
Comparison of direct and indirect measures of walking energy expenditure in children with hemiplegic cerebral palsy
Authors: Keefer Daniel J , Tseh Wayland , Caputo Jennifer L , Apperson Kathy , McGreal Sheri , Morgan Don W
Published: July 2010

The purpose of this study was to investigate the use of the heart‐rate (HR) version of the energy expenditure index (EEIHR) as a proxy for measurement of walking oxygen consumption (VO2) in children with cerebral palsy (CP). Thirteen children (eight males, five females; mean age 11 years 2 months [SD 3 years], age range 6 to 15 years) with hemiplegic CP, participated in this study. The study was conducted over three sessions. During session 1, participants were familiarized with testing procedures and given 5 minutes of treadmill walking practice. In session 2, participants completed three 5‐minute walking bouts on the treadmill at 0.67m˙s‐1 to familiarize themselves with treadmill locomotion. During the final session participants walked at 0.67, 0.89, and 1.12m˙s‐1 for 5 minutes while gross oxygen consumption (gross VO2; walking VO2/speed), net VO2 ([walking VO2‐resting VO2]/speed), and EEIHR ([walking HR‐resting HR]/speed) were measured during the last 2 minutes of each bout. Correlational analyses indicated no relationship (p>0.05) between measures of gross VO2 and EEIHR at each speed. Although no association was evident between net VO2 and EEIHR at 0.67 and 0.89m˙s‐1, a moderate relationship (r=0.64;p < 0.05) was present between these variables at 1.12m˙s‐1. Examination of individual data revealed that most participants displayed an unmatched pattern of response between net VO2 and EEIHR. Our results suggest that caution should be applied when using EEIHR to estimate walking energy expenditure in children with CP.
Cognitive development in Silver‐Russell syndrome: a sibling‐controlled study
Authors: Noeker Meinolf , Wollmann Hartmut A
Published: July 2010

This study examined cognitive development in Silver‐Russell syndrome (SRS), a condition with intrauterine growth retardation, persisting short stature, and specific stigmata. Neuropsychological function and cognitive abilities were assessed in a sample of 36 children with SRS (21 males, 15 females; mean age 7 years 6 months, SD 2 years 8 months; age range 3 to 12 years) and 25 normally developing siblings (mean age 8 years 6 months, SD 2 years 7 months; age range 3 to 12 years) using the Kaufman Assessment Battery for Children. Special measures were taken to control for confounding factors and sample bias. Mean overall IQ score in the total SRS sample (95.7, SD 10.63), as measured by the Mental Processing Composite Scale, was significantly reduced (p=0.021) compared with test norms (IQ 100, SD 15), indicating a moderate cognitive impairment. Subscale analysis revealed some specific deficiencies. However, these cannot be attributed to an established category of specific learning disorder. The mean score in the Achievement Scale (91.25, SD 14.92), which is more sensitive to educational influences, showed stronger deficits (p=0.001). The sibling control group achieved a slightly better mean IQ score (104.20, SD 12.32) than test norms (p=0.10). Direct analysis of paired differences between the subsample of children with SRS and a sibling among the control group (n=25) revealed a significant mean difference of 8.08 IQ points (p=0.011). Risk factor analysis revealed that cognitive development is not associated with birth length (p=0.404), birthweight (p=0.820), growth hormone therapy (p=0.810), phenotypic severity (p=0.828), or sex (p=0.880). Two children with maternal uniparental disomy for the entire chromosome 7 had markedly lower IQ scores (81 and 84 respectively). In contrast to the few previous findings, children with SRS show only moderate, but significant, impairments in cognitive outcome, which are more striking in our sample when compared with siblings than with test norms.
Height prediction from ulna length
Authors: Gauld Leanne M , Kappers Johanna , Carlin John B , Robertson Colin F
Published: July 2010

Height is fundamental to assessing growth and nutrition, calculating body surface area, and predicting pulmonary function in childhood. Its measurement is hindered by muscle weakness, joint, or spinal deformity. Arm span has been used as a substitute, but is inaccurate. The objective of the study was to identify a limb measurement that precisely and reproducibly predicts height in childhood. Males (n=1144) and females (n=1199), aged 5 years 4 months to 19 years 7 months, without disability were recruited from Melbourne schools. Height, arm span, ulna, forearm, tibia, and lower leg lengths were measured with a Harpenden stadiometer and anthropometer. Prediction equations for height based on ulna length (U) and age in years (A) were developed using linear regression. Ulna centile charts were developed by the LMS method. For males, height (cm)=4.605U+1.308A+28.003 (R2=0.96); for females, height (cm)=4.459U+1.315A+31.485 (R2=0.94). Intra‐ and inter‐observer variability was 0.41% and 0.61% relative to the mean, respectively. Height prediction equations from tibia, forearm, and lower leg length were calculated. We show that ulna measurement is reproducible and precisely predicts height in school‐age children. It appears to be superior to arm span measurement when neuromuscular weakness, joint, or spinal deformity exists. Ulna growth charts should facilitate growth assessment.
Changes to medial gastrocnemius architecture after surgical intervention in spastic diplegia
Authors: Shortland Adam P , Fry Nicola R , Eve Linda C , Gough Martin
Published: July 2010

We assessed the architecture of the medial gastrocnemius in nine children (five males, four females; age range 6 to 15 years; mean 10 years 10 months, SD 3 years 6 months) with spastic diplegia by ultrasound imaging before and after a gastrocnemius recession. The children were ambulant (seven independent, one with a posterior walker, one using crutches) before and after surgical intervention. We compared values for fascicle lengths and deep fascicular‐aponeurosis angles with those from a group of normally developing children (five males, five females; age range 6 to 11 years; mean 8 years 4 months, SD 1 year 4 months). Despite a variable interval between assessments (from 56 to 610 days), fascicles were shorter (p=0.00226) and the deep fascicular‐aponeurosis angle increased (p=0.0152) after intervention. Fascicle lengths of patients were similar to those in the group of normally developing children before surgery. After surgery, fascicles in the group of children with spastic diplegia were shorter than in their normally developing peers (p=0.00109). The gastrocnemius recession procedure alters muscle architecture, though the degree of fascicular shortening varied, with four of the participants in our study losing less than 10% of their original fascicular length at maximum dorsiflexion. Increases in ankle‐joint power in walking, observed after surgical intervention in children with spastic diplegia, may be due to a more normal ankle position rather than to improvements in the active mechanical performance of the gastrocnemius.
Influence of gait pattern on the body's centre of mass displacement in children with cerebral palsy
Authors: Massaad Firas , Dierick Frédéric , Van Den Hecke Adélaïde , Detrembleur Christine
Published: July 2010

We assessed the influence of digitigrade gait pattern, topographical types, severity of motor involvement, and locomotor experience on the body's centre of mass (COM) displacement during gait in children with spastic cerebral palsy (CP). Three‐dimensional COM displacements were computed from ground reaction forces in 51 independent digitigrade walkers (29 males, 22 females; mean age 10 years 6 months, SD 2 years 7 months, range 7 to 15 years). Results obtained from 10 participants without disabilities (five males, five females), in the same age range as the patients with CP, were used as a reference plantigrade group. Vertical and forward COM displacements were significantly different between the digitigrade and the plantigrade walkers. Neither the topographical type (quadriplegia, n=5; diplegia, n=20; right hemiplegia, n=13; left hemiplegia, n=13), nor the severity of motor involvement, nor the locomotor experience influenced COM displacements. We conclude that the COM displacement during gait in patients with CP was mainly influenced by the digitigrade gait pattern encountered in this neurological disorder rather than the different topographical types and motor involvements.
Joubert syndrome: long‐term follow‐up
Authors: Hodgkins Peter R , Harris Christopher M , Shawkat Fatima S , Thompson Dorothy A , Chong Kling , Timms Christine , Russell‐Eggitt Isabelle , Taylor David S , Kriss †Anthony
Published: July 2010

Twenty‐nine patients (16 males, 13 females) with Joubert syndrome were identified from ophthalmology, neurology, and genetic databases covering a 15‐year period at Great Ormond Street Hospital, London. Criteria for diagnosis included absent or markedly hypoplastic cerebellar vermis, abnormal eye movements, and developmental delay. Five patients had died. Scans and notes were available for 22 patients, and 18 cases were clinically reviewed. The median age was 10 years 10 months (range 3mo to 19y) and the median follow‐up was 8 years 5 months (range 3mo to 19y, with one new patient seen at 3mo of age). Cerebellar vermis hypoplasia/aplasia with‘molar tooth sign’in the axial plane was present in 22 of 22 patients, coloboma in 6 of 22, and polydactyly in 6 of 22. In the 18 clinically reviewed, apnoea occurred in 13 patients. Five had renal problems with cysts and 4 of 5 had abnormal electroretinograms (ERGs). Visual electrophysiology was abnormal in 14 of 18 patients, and in 6 there was evidence of deterioration in the ERG. Blood investigations of organic acids, phytanic acid, very‐long‐chain fatty acid, and transferrin were normal in 12 patients tested. Developmental assessment showed that 6 of 15 patients aged more than 5 years were at mainstream school, and 12 of 18 had started walking between 22 months and 10 years. Speech difficulties and behavioural problems were prominent.
Rolling over in infants: age, ethnicity, and cultural differences
Authors: Nelson E A S , Yu LM , Wong D , Wong HYE , Yim L
Published: July 2010

A preliminary cross‐sectional study of 72 Hong Kong Chinese infants suggested that these infants learn to roll from supine‐to‐prone before rolling from prone‐to‐supine i.e. the opposite to teaching in most western texts. Three‐hundred and sixty mothers were recruited postpartum and asked to record, on a chart, information related to the developmental milestone of rolling over for their infants (49% male) during the next 9 months. Telephone reminders were given at 4 and 8 months and, by the end of the study, information had been obtained from 240 mothers (67% of original sample, 51% male infants). Mean ages of rolling over were 5.1 months (SD1.5) for supine‐to‐prone and 5.7 months (SD1.3) for prone‐to‐supine. Age of rolling over from supine‐to‐prone was not influenced by usual sleep position, infant's sex, mother's intention to breastfeed infant, number of siblings, marital status, main daytime caregiver, or feeding method over 9 months. Hong Kong Chinese infants roll from supine‐to‐prone before they roll from prone‐to‐supine.
Pyruvate dehydrogenase deficiency presenting as dystonia in childhood
Authors: Head R A , De Goede C G E L , Newton R W N , Walter J H , McShane M A , Brown R M , Brown G K
Published: July 2010

Two individuals with pyruvate dehydrogenase (PDH) deficiency due to missense mutations in the gene for the E1α subunit (PDHA1) presented during childhood with dystonia. The first patient, a male, presented at age 4 years with dystonia affecting the lower limbs, which responded to treatment with combined carbidopa and levodopa. The second patient, a female, was first investigated at age 6 years because of a dystonic gait disorder. In both patients, the main clue to the biochemical diagnosis was a raised concentration of lactate in the cerebrospinal fluid. PDH activity was significantly reduced in cultured fibroblasts in both cases. Dystonia is a previously unrecognized major manifestation of PDH deficiency and is of particular interest as the mutations in the PDHA1 gene in these patients have both been identified previously in individuals with typical presentations of the condition.
Effect of intensive neurodevelopmental treatment in gross motor function of children with cerebral palsy
Authors: Tsorlakis Nikos , Evaggelinou Christina , Grouios George , Tsorbatzoudis Charalambos
Published: July 2010

This study examined the effect of neurodevelopmental treatment (NDT) and differences in its intensity on gross motor function of children with cerebral palsy (CP). Participants were 34 children (12 females, 22 males; mean age 7y 3mo [SD 3y 6mo], age range 3 to 14y) with mild to moderate spasticity and hemiplegia (n=10), diplegia (n=12), and tetraplegia (n=12). Gross Motor Function Classification System levels were: I (n=10), II (n=10), and III (n=14). The paired sample, which was obtained by ratio stratification and matching by sex, age, and distribution of impairment from a total of 114 children with CP, was assigned randomly to two groups: group A underwent NDT twice a week and group B five times a week for 16 weeks. The outcome measure used was the Gross Motor Function Measure, which assessed the performance of the children before and after intervention. The paired‐sample t‐test revealed that gross motor function of children from both groups improved significantly after intervention (p<0.05). Children in group B performed better and showed significantly greater improvement than those in group A (p<0.05). Results support the effectiveness of NDT and underline the need for intensive application of the treatment.
Internal representation of movement in children with developmental coordination disorder: a mental rotation task
Authors: Wilson PH , Maruff P , Butson M , Williams J , Lum J , Thomas PR
Published: July 2010

Recent studies show that children with developmental coordination disorder (DCD) have difficulties in generating an accurate visuospatial representation of an intended action, which are shown by deficits in motor imagery. This study sought to test this hypothesis further using a mental rotation paradigm. It was predicted that children with DCD would not conform to the typical pattern of responding when required to imagine movement of their limbs. Participants included 16 children with DCD and 18 control children; mean age for the DCD group was 10 years 4 months, and for controls 10 years. The task required children to judge the handedness of single‐hand images that were presented at angles between 0° and 180° at 45° intervals in either direction. Results were broadly consistent with the hypothesis above. Responses of the control children conformed to the typical pattern of mental rotation: a moderate trade‐off between response time and angle of rotation. The response pattern for the DCD group was less typical, with a small tradeoff function. Response accuracy did not differ between groups. It was suggested that children with DCD, unlike controls, do not automatically enlist motor imagery when performing mental rotation, but rely on an alternative object‐based strategy that preserves speed and accuracy. This occurs because these children manifest a reduced ability to make imagined transformations from an egocentric or first‐person perspective.