bluuebunny/crossref_metadata_2025 huggingface dataset:
Adolescents with cerebral palsy: stability in measurement of quality of life and health‐related quality of life over 1 year
Authors: Livingston Michael H , Rosenbaum Peter L
Published: June 2009

This study assessed stability of measurement of quality of life (QOL) and health‐related quality of life (HRQOL) over the course of 1 year among 185 adolescents (mean age 16y, SD 1y 9mo) with cerebral palsy (CP). Participants were classified on the Gross Motor Function Classification System as level I (n=55), II (n=30), III (n=27), IV (n=46), or V (n=27). QOL was assessed by self‐ (n=125) or proxy‐report (n=60) with the Short Version of the Quality of Life Instrument for People with Developmental Disabilities (QOL Instrument), which describes domains of Being, Belonging, and Becoming. HRQOL was captured through parent proxy‐reports with the Health Utilities Index Mark 3 (HUI3). Generalizability coefficients (G) for domain and Overall QOL scores on the QOL Instrument ranged from 0.50 to 0.73, indicating that between 50 and 73% of the variance was stable over 1 year. Stability on the HUI3 was excellent (G>0.90) for ambulation and overall utility scores; moderate (G=0.70–0.90) for speech, vision, dexterity, cognition, and hearing; and low for pain (G=0.48) and emotion (G=0.24). Correlations between scores on the two instruments were moderate even when adjustments were made for the lack of perfect stability over 1 year. This supports the notion that QOL and HRQOL are different aspects of life experience among adolescents with CP.
Genetic mechanisms in idiopathic epilepsies
Authors: Weber Yvonne G , Lerche Holger
Published: June 2009

Idiopathic epilepsies are considered to be genetically determined. The inheritance can be monogenic and the detected mutation considered sufficient to cause the phenotype. In contrast, when the inheritance is complex, the epileptic phenotype is determined by several minor genetic defects that are much more difficult to discover. In recent years, an increasing number of mutations, mainly associated with rare monogenic idiopathic epilepsy syndromes, have been identified in genes encoding subunits of voltage‐ or ligand‐gated ion channels. A few mutations have also been found in the frequent classical forms of idiopathic generalized epilepsies which are thought to follow a complex genetic trait, for example, in absence or juvenile myoclonic epilepsies. Functional studies characterizing the molecular defects of the mutant channels point to an important role of GABAergic synaptic inhibition in the pathophysiology of idiopathic epilepsies. As a result of genetic and functional investigations, not only will the pathophysiology of epilepsy be better understood, but newly discovered genes and pathophysiological pathways may also determine novel targets for pharmacotherapy, as has been shown for the anticonvulsant drug retigabine, which enhances the activity of neuronal KCNQ potassium channels.
Gross motor functional abilities in preterm‐born children with cerebral palsy due to periventricular leukomalacia
Authors: Van Haastert I C , De Vries L S , Eijsermans M J C , Jongmans M J , Helders P J M , Gorter J W
Published: June 2009

To describe the impact of periventricular leukomalacia (PVL) on gross motor function, data on 59 children (37 males, 22 females) with a gestational age (GA) of 34 weeks or less with cerebral palsy (CP) due to PVL grade I (n=20), II (n=13), III (n=25), and IV (n=1) were studied; (mean GA 29wk 4d [SD 4wk 6d]; mean birthweight 1318g [SD 342]). Two independent raters used the Gross Motor Function Classification System (GMFCS) at four time points: T1, mean corrected age (CA) 9 months 15 days (SD 2mo 6d); T2, mean CA 16 months (SD 1mo 27d); T3, mean CA 24 months 27 days (SD 2mo 3d); and T4, median age 7 years 6 months (range 2y 2mo–16y 8mo). Interrater reliability and stability across time with respect to the total cohort were κ≥0.86 and ρ≥0.74 respectively. The association between PVL and gross motor outcome at T4 was strong (positive and negative predictive values 0.92 and 0.85 respectively). The proportion of children who remained in the same GMFCS level increased from 27% (T1–T4) to 53% (T2–T4) and 72% (T3–T4). PVL grade I to II, as diagnosed in the neonatal period, has a better functional mobility prognosis than PVL grade III–IV. These findings have implications for habilitation counselling and intervention strategies.
Participation and enjoyment of leisure activities in school‐aged children with cerebral palsy
Authors: Majnemer Annette , Shevell Michael , Law Mary , Birnbaum Rena , Chilingaryan Gevorg , Rosenbaum Peter , Poulin Chantal
Published: June 2009

The objective of this study was to characterize participation in leisure activities in children with cerebral palsy (CP) and identify determinants of greater involvement. Ninety‐five children of school age (9y 7mo [SD 2y 1mo]) with CP were recruited, and participation was evaluated with the Children’s Assessment of Participation and Enjoyment in a subset (67/95; 42 males, 25 females) who could actively participate in completion of the assessment. Most had mild motor dysfunction (Gross Motor Function Classification System: 59% level I, 23% level II, 18% levels III–V) and had a spastic subtype of CP (23 hemiplegia, 17 diplegia, 16 quadriplegia, 11 other). Biomedical, child, family and environmental predictor variables were considered in the analysis. Results demonstrated that these children were actively involved in a wide range of leisure activities and experienced a high level of enjoyment. However, involvement was lower in skill‐based and active physical activities as well as community‐based activities. Mastery motivation and involvement in rehabilitation services enhanced involvement (intensity and diversity) in particular leisure activities, whereas cognitive and behavioral difficulties, activity limitations, and parental stress were obstacles to participation.
Effectiveness of sedation using nitrous oxide compared with enteral midazolam for botulinum toxin A injections in children
Authors: Zier Judith L , Rivard Patrick F , Krach Linda E , Wendorf Heather R
Published: June 2009

This randomized, double‐blind, placebo‐controlled study compared the efficacy of inhaled nitrous oxide (N2O) with enteral midazolam for sedation of children with cerebral palsy (CP) undergoing botulinum toxin A (BoNT‐A) injections. Fifty children (29 males, 21 females; mean age 8y 2mo [SD 4y 5mo]; range 1–16y) were randomized to sedation with N2O (n=25) or midazolam (n=25). Groups were similar in type of CP (diplegia, 11; triplegia, three; quadriplegia, 16; hemiplegia, 16; other, three) and Gross Motor Function Classification System level (Level I, 4; II, 24; III, 4; IV, 13; V, 5). Both groups were equally sedated at time of injection (p=0.661), but those in the midazolam group were more sedated at time of discharge (p<0.001). N2O was more effective in reducing pain compared with midazolam as measured using the Face, Legs, Activity, Cry, Consolability (FLACC) scale (p=0.010), parental estimate of pain (p=0.009), and nursing estimate of pain (p=0.007). Parents in the N2O group rated it better than prior sedation with midazolam for BoNT‐A injections (p=0.031). Physicians and nurses reported no difference in ease of procedure between the groups. One child in the midazolam group and eight in the N2O group had adverse effects, all of which resolved promptly. N2O appears to be an effective means of sedation for children undergoing outpatient BoNT‐A injections.
Rapid eye movement sleep behaviour disorder in children and adolescents
Authors: Stores Gregory
Published: June 2009

Rapid eye movement (REM) sleep behaviour disorder (RBD) is a recently described parasomnia with important implications for diagnosis, treatment, and prognosis. Once thought to occur only in elderly males, it has now been reported in other groups including children and adolescents. The cardinal feature of RBD is the loss of REM atonia, which allows dreams to be acted out. Depending on the dream content, the accompanying behaviour can be dramatic, sometimes causing self‐injury or harm to others. In adults, increasing numbers of associated conditions have been reported, notably neurodegenerative disorders of which RBD can be a prodrome. Other associations include narcolepsy, epilepsy, and antidepressant medications. Awareness that RBD can occur in young patients (admittedly rarely, it seems) is important. It is essential to distinguish between RBD and other dramatic parasomnias because each has different implications and treatment requirements. Children and adolescents displaying both clinical and polysomnographic features of RBD, and others with only polysomnographic evidence of loss of REM atonia, have been reported in association with various neurological and neurodevelopmental disorders. Reports of these cases are reviewed, together with suggestions for further reporting and research.
Group aquatic aerobic exercise for children with disabilities
Authors: Fragala‐Pinkham Maria , Haley Stephen M , O’Neil Margaret E
Published: June 2009

The effectiveness and safety of a group aquatic aerobic exercise program on cardiorespiratory endurance for children with disabilities was examined using an A–B study design. Sixteen children (11 males, five females) age range 6 to 11 years (mean age 9y 7mo [SD 1y 4mo]) participated in this twice‐per‐week program lasting 14 weeks. The children’s diagnoses included autism spectrum disorder, myelomeningocele, cerebral palsy, or other developmental disability. More than half of the children ambulated independently without aids. Children swam laps and participated in relay races and games with a focus of maintaining a defined target heart rate zone. The strengthening component consisted of exercises using bar bells, aquatic noodles, and water resistance. The following outcomes were measured: half‐mile walk/run, isometric muscle strength, timed floor to stand 3‐meter test, and motor skills. Complaints of pain or injury were systematically collected. Significant improvements in the half‐mile walk/run were observed, but not for secondary outcomes of strength or motor skills. The mean program attendance was 80%, and no injury was reported. Children with disabilities may improve their cardiorespiratory endurance after a group aquatic aerobic exercise program with a high adult:child ratio and specific goals to maintain training heart rates.
Behaviour in children with cerebral palsy with and without epilepsy
Authors: Carlsson Malin , Olsson Ingrid , Hagberg Gudrun , Beckung Eva
Published: June 2009

The aim of the study was to describe behavioural problems in children with cerebral palsy (CP) with and without epilepsy. The children were sampled from the Western Sweden CP register and were part of a European Union project. The Strength and Difficulties Questionnaire and questions on epilepsy were answered by one parent of each child. Medical records were reviewed. Parents of 83 children (44 males, 39 females) age range participated: 30 at Gross Motor Function Classification System levels I and II, and 53 at levels III to V; 60 had spastic age range 8 to 12 years (bilateral 42, unilateral 18) and 23 dyskinetic CP; 34 children had active epilepsy. The proportion of children with normal behaviour on the total difficulties score (TDS) of the Strength and Difficulties Questionnaire was significantly lower than normative data (57% vs 80%, p<0.001). Parents of 21 children (25%) considered their child’s behaviour to be abnormal. Children with CP and epilepsy had a significantly higher median TDS (p=0.03) than seizure‐free children. In children with aided or no walking ability, the TDS was significantly higher in those with epilepsy (p=0.04). Parents of 32 children (39%) considered their children’s behaviour to have an impact on themselves and others. We conclude that behavioural problems are common in children with CP, and even more when epilepsy is present. Parents identify these problems, and professionals need to address them.
Administration of melatonin mixed with soft food and liquids for children with neurodevelopmental difficulties
Authors: Shah T , Tse APY , Gill H , Wong ICK , Sutcliffe A , Gringras P , Appleton R , Tuleu C
Published: June 2009

It is sometimes necessary for the contents of medication capsules to be mixed with certain foods and drinks because children are not always able to swallow the capsules. The compatibility and short‐term stability (6h) of melatonin capsules mixed in a variety of liquids and foodstuffs (water, orange juice, semi‐skimmed milk, strawberry yogurt, and strawberry jam) were analyzed for degradation. Extraction of melatonin from these common administration vehicles and an analytical assay for the drug and its potential degradation products were developed and validated. The results showed good recovery of melatonin from low‐ and high‐strength capsules for all administration vehicles (between 89% minimum and 111% maximum). The drug was found to be stable in the common liquids and foods tested for up to 6 hours at room temperature (no degradation peak); hence it is unlikely to compromise the results of the Use of Melatonin in Children with Neurodevelopmental Disorders and Impaired Sleep trial.
Quantification of upper extremity function and range of motion in children with cerebral palsy
Authors: Koman L Andrew , Williams Rafael M M , Evans Peter J , Richardson Rachel , Naughton Michelle J , Passmore Leah , Smith Beth Paterson
Published: June 2009

This study evaluated the hypothesis that upper extremity function and range of motion can be quantified reliably in children with cerebral palsy (CP) in a busy clinical setting. The specific aim was to determine the inter‐ and intrarater reliability of a modified House Functional Classification (MHC) system to evaluate upper extremity function and a standardized instrument to document upper extremity range of motion (Upper Extremity Rating Scale [UERS]). Sixty‐five children with CP (43 males, 22 females, mean age 9y 2mo, SD 4y 1mo) with spasticity involving the upper extremity (quadriplegia n=22; hemiplegia n=36; diplegia n=7; Gross Motor Functional Classification System Levels I n=41, II n=6, III n=3, IV n=5, V n=10) were evaluated independently by occupational therapists and orthopedic surgeons using both instruments at several visits. Inter‐ and intrarater reliability were determined for both instruments by calculating measures of agreement (weighted kappa values and intraclass correlation coefficients [ICCs]). Interrater agreement (ICC=0.94) and intrarater agreement (ICC=0.96) on the MHC were good to excellent. Similarly, inter‐rater agreement (kappa 0.66–0.81) and intrarater agreement (kappa 0.64–0.88) on the UERS was either good or excellent. The MHC and the UERS provide standardized, reliable, reproducible, and efficient instruments that can be used by occupational therapists and orthopedic surgeons to evaluate the upper extremities of children with CP.
Influence of behaviour and risk factors on motor performance in preterm infants at age 2 to 3 years
Authors: Janssen A J W M , Nijhuis‐van der Sanden M W G , Akkermans R P , Oostendorp R A B , Kollée L A A
Published: June 2009

The aim of this cross‐sectional study was to determine the influence of test‐taking behaviour and risk factors for delayed motor performance in 437 preterm infants (244 males, 193 females; ≤32 weeks of gestation) at the corrected age of 2 to 3 years (mean 29mo [SD 3.3]). Other mean (SD) sample demographics were: postmenstrual age 29+5 weeks (1+5), range 25+0–32+0; birthweight 1213.7g (331.7), range 468–2350; and days in the neonatal intensive care unit 21.1 (21.3), range 1–165. Children (n=23) with a severe disability were excluded. We assessed motor performance and behaviour during testing with the Motor Scale and the Behaviour Rating Scale (BRS) of the Bayley Scales of Infant Development, 2nd edition (BSID‐II). Risk factors were tested against delayed motor performance as the dependent variable in binary logistic regression analysis. Median score on the Motor Scale in terms of the BSID‐II Psychomotor Developmental Index (PDI) was 86. ‘Delayed’ motor performance was observed in 46.5% of the children tested, and behaviour was ‘not‐optimal’ in 31.4%. The Motor Scale and BRS scores were significantly correlated (rs=0.62, p<0.01). Risk factors for delayed motor performance were: neonatal convulsions (odds ratio [OR] 4.5; 95% confidence interval [CI] 1.6–12.9), low maternal educational level (OR 3.3; 95% CI 1.7–6.5), male sex (OR 2.8; 95% CI 1.8–4.3), and chronic lung disease (OR 2.1; 95% CI 1.1– 4.1). We conclude that preterm infants are at high risk of delayed motor performance and non‐optimal test‐taking behaviour.
Motor coordination difficulties and physical fitness of extremely‐low‐birthweight children
Authors: BURNS YVONNE R , DANKS MARCELLA , O’CALLAGHAN MICHAEL J , GRAY PETER H , COOPER DAVID , POULSEN LEITH , WATTER PAULINE
Published: June 2009

Motor coordination difficulties and poor fitness exist in the extremely low birthweight (ELBW) population. This study investigated the relative impact of motor coordination on the fitness of ELBW children aged 11 to 13 years. One hundred and nine children were recruited to the study: 54 ELBW participants (mean age at assessment 12y 6mo; 31 male, 23 female; mean birthweight 769g, SD 148g; mean gestational age 26.6 weeks, SD 2.1 weeks) and 55 comparison children (mean age at assessment 12y 5mo; 28 males, 27 females; at least 37 weeks’ gestation). All children completed the Movement Assessment Battery for Children (MABC), functional tests of postural stability and strength, growth measures, and tests of respiratory function. Maximal oxygen uptake (VO2max) was calculated from a 20m shuttle run test as a measure of fitness. The ELBW group had greater problems with postural stability (p=0.001) and motor coordination (p=0.001), with 70% rated as having a definite motor problem on the MABC brackets (those who scored less than the 5th centile on the MABC). The ELBW was also less fit than the comparison group (p=0.001), with 45% below the 10th centile for VO2max. There were differences between the groups for growth, strength, and particularly respiratory function. However, respiratory function did not significantly correlate with VO2max in the ELBW group. Motor coordination was the most powerful predictor of VO2max in both the ELBW (p=0.001) and the comparison groups (p=0.001).
Hyperactive stretch reflexes, co‐contraction, and muscle weakness in children with cerebral palsy
Authors: POON DORA M Y , HUI‐CHAN CHRISTINA W Y
Published: June 2009

The aim of this study was to examine the repeatability of and relationships among spasticity, co‐contraction of agonist–antagonist, and muscle strength in children with cerebral palsy (CP). Eight children with spastic diplegic CP (five males, three females; Gross Motor Function Classification System [GMFCS] Levels I–III; mean age 10y 2mo [SD 2y 9mo], range 6–13y) and nine children in a comparison group (six males, three females; mean age 8y 10mo [SD 2y 4mo], range 6y to 12y 6mo) were assessed twice to examine repeatability of Composite Spasticity Scale, soleus stretch reflexes, electromyography (EMG) co‐contraction ratio, and torque recorded during maximal isometric voluntary contraction of ankle dorsiflexors and plantarflexors. Sixty‐one children with spastic CP, (54 diplegic, seven hemiplegic; 32 males, 29 females; GMFCS levels I–III; mean age 10y 8mo [SD 2y 9mo], range 6–15y) were then assessed to delineate possible correlations among these measures. Intraclass correlation coefficients (0.78–0.97) showed high data repeatability in both groups. Children with spastic CP demonstrated significantly larger soleus stretch reflex/M‐response areas smaller torques, but larger EMG co‐contraction ratios during both voluntary dorsiflexion and plantarflexion (all p<0.05). Children with spastic CP who had larger soleus stretch reflex/M‐response areas demonstrated larger plantarflexion co‐contraction ratio (r = 0.28), and produced smaller plantarflexion and dorsiflexion torques (r = –0.48 and –0.27 respectively). However, no correlation was noted between soleus stretch reflex and clinical spasticity. Our findings demonstrated that hyperactive soleus stretch reflex affected torque production of ankle muscles. Moreover, the severity of spasticity may not be fully described by either stretch reflex or tone measure alone.
Recurrence risk after a first remote symptomatic unprovoked seizure in childhood: a prospective study
Authors: RAMOS‐LIZANA J , AGUIRRE‐RODRÍGUEZ J , AGUILERA‐LÓPEZ P , CASSINELLO‐GARCÍA E
Published: June 2009

The aim of this study was to assess recurrence risk after a first remote symptomatic unprovoked seizure in childhood. All consecutive patients younger than 14 years with a first remote symptomatic unprovoked seizure who were seen at our hospital between 1994 and 2006 were included in the study and prospectively followed. Only two patients received antiepileptic treatment. Sixty‐three children were included, with 35 males and 28 females. Mean age at first seizure was 4 years (SD 3y 5mo). Kaplan–Meier estimate of recurrence risk was 59% (95% confidence interval [CI] 47–71), 76% (95% CI 65‐87), 85% (95% CI 76–94), and 87% (95% CI 78–96) at 6, 12, 18, and 24 months respectively. A total of 55 children out of 63 were affected by a static encephalopathy of pre‐ or perinatal origin. In this subgroup, recurrence risk at 12 and 24 months was 79% (95% CI 68–90) and 89% (95% CI 80–98). Univariable analysis using the Cox proportional hazards model showed that presence of global developmental delay/intellectual disability and Todd’s paresis were associated with a significant increase in recurrence risk. In multivariable analysis, only Todd’s paresis was significantly associated. Recurrence risk after a first remote symptomatic unprovoked seizure in childhood is much higher than what some previous studies suggests.
A systematic review of the effectiveness of aerobic exercise interventions for children with cerebral palsy: an AACPDM evidence report
Authors: Rogers Anna , Furler Barbara‐Lynne , Brinks Stephen , Darrah Johanna
Published: June 2009

The aim of this review was to assess the evidence regarding the effectiveness of aerobic training interventions for children with cerebral palsy (CP). The target population included children with CP of any severity, aged 2 to 17 years. The following databases were searched for English language studies from 1960 to 2006: MEDLINE, EMBASE, CINAHL, Pascal, Cochrane Library, CSA Neuroscience Abstracts, The Physiotherapy Evidence Database (PEDro), and Sport Discus. Search terms included ‘cerebral palsy’, ‘athetoid’, ‘ataxic’, ‘spastic diplegia’, ‘hemiplegia’, ‘quadriplegia’, ‘aerobic’, ‘exercise’, ‘training’, ‘physical activity’, ‘aquatic/water/pool therapy’, and ‘continuous exercise’. The American Academy for Cerebral Palsy and Developmental Medicine systematic review guidelines were used to format the review. One thousand, four hundred and eighty nine articles were identified and examined for the stated inclusion and exclusion criteria. Thirteen articles met the criteria for inclusion. The evidence suggests that aerobic exercise with children with CP can improve physiological outcomes, but the influence of these changes on outcomes representing activity and participation are unknown. Future research needs improved methodological rigour in order to determine a specific set of exercise guidelines and safety considerations.
Outcome tools used for ambulatory children with cerebral palsy: responsiveness and minimum clinically important differences
Authors: Oeffinger D , Bagley A , Rogers S , Gorton G , Kryscio R , Abel M , Damiano D , Barnes D , Tylkowski C
Published: June 2009

This prospective longitudinal multicenter study of ambulatory children with cerebral palsy (CP) examined changes in outcome tool score over time, tool responsiveness, and used a systematic method for defining minimum clinically important differences (MCIDs). Three hundred and eighty‐one participants with CP (Gross Motor Function Classification System [GMFCS] Levels I–III; age range 4–18y, mean age 11y [SD 4y 4mo]; 265 diplegia, 116 hemiplegia; 230 males, 151 females). At baseline and follow‐up at least 1 year later, Functional Assessment Questionnaire, Gross Motor Function Measure, Pediatric Quality of Life Inventory, Pediatric Outcomes Data Collection Instrument, Pediatric Functional Independence Measure, temporal–spatial gait parameters, and oxygen cost were collected. Adjusted standardized response means determined tool responsiveness for nonsurgical (n=292) and surgical (n=87) groups at GMFCS Levels I to III. Most scores reaching medium or large effect sizes were for GMFCS Level III. Nonsurgical group change scores were used to calculate MCID thresholds for ambulatory children with CP. These values were verified by examining participants who changed GMFCS levels. Tools measuring function were responsive when a change large enough to cause a change in GMFCS level occurred. MCID thresholds assess change in study populations over time, and serve as the basis for designing prospective intervention studies.
The role of epilepsy in early language development in a child with a congenital lesion in the right hemisphere
Authors: Mayor‐Dubois C , Maeder‐Ingvar M , Deonna T , Roulet‐Perez E
Published: June 2009

Early epilepsy is known to worsen the developmental prognosis of young children with a congenital focal brain lesion, but its direct role is often very difficult to delineate from the other variables. This requires prolonged periods of follow‐up with simultaneous serial electrophysiological and developmental assessments which are rarely obtained. We studied a male infant with a right prenatal infarct in the territory of the right middle cerebral artery resulting in a left spastic hemiparesis, and an epileptic disorder (infantile spasms with transient right hemihypsarrhythmia and focal seizures) from the age of 7 months until the age of 4 years. Pregnancy and delivery were normal. A dissociated delay of early language acquisition affecting mainly comprehension without any autistic features was documented. This delay was much more severe than usually expected in children with early focal lesions, and its evolution, with catch‐up to normal, was correlated with the active phase of the epilepsy. We postulate that the epilepsy specifically amplified a pattern of delayed language emergence, mainly affecting lexical comprehension, reported in children with early right hemisphere damage.
Twenty years after childhood‐onset symptomatic generalized epilepsy the social outcome is usually dependency or death: a population‐based study
Authors: Camfield Carol , Camfield Peter
Published: June 2009

Aims:  To document the adult social outcome for childhood onset symptomatic generalized epilepsies (SGE).

Methods:  Using the Nova Scotia population‐based epilepsy cohort we identified children who developed SGE between 1977 and 1985. Social outcome for those >18 years old was assessed in 2003–5 by semi‐structured interview with patients and/or caretakers.

Results:  Eighty children developed SGE, 12% of childhood epilepsy. Syndromes at onset were: SGE undefined 32 (37%), West 32 (42%), Lennox‐Gastaut 4, myoclonic astatic 9, other 3. Nineteen (24%) died. Fifty‐two survivors (22 women, 30 men) were 23.7 SD 3.6 years old: 46 (88%) had mental retardation; 34 (65%) continued AEDs; and 27 (51%) were unable to walk. Social outcome was good (live independently) in 7 (13%), moderate (unable to live independently but independent for most daily activities) in 15 (29%) and poor (dependent for nearly all activities) in 30 (58%). Twenty‐nine (56%) remained at home, 40% in group homes or institutions. Financial dependency was marked – complete dependence on parents/state in 90%. Eleven (20%) were socially isolated, and only 7 (13%) had significant social interaction in community activities. Health was satisfactory in 49 (94%). There were no pregnancies. Only 2 were seizure‐free, off AEDs, living independently and financially independent.

Conclusion:  The social outcome of children with SGE 20 years after diagnosis is usually disappointing. One quarter die and nearly all survivors have mental retardation and are highly dependent.
Motor stereotypies in children with autism and other developmental disorders
Authors: GOLDMAN SYLVIE , WANG CUILING , SALGADO MIRAN W , GREENE PAUL E , KIM MIMI , RAPIN ISABELLE
Published: June 2009

The purpose of the study was to count and characterize the range of stereotypies – repetitive rhythmical, apparently purposeless movements – in developmentally impaired children with and without autism, and to determine whether some types are more prevalent and diagnostically useful in children with autism. We described each motor stereotypy recorded during 15 minutes of archived videos of standardized play sessions in 277 children (209 males, 68 females; mean age 4y 6mo [SD 1y 5mo], range 2y 11mo–8y 1mo), 129 with autistic disorder (DSM‐III‐R), and 148 cognitively‐matched non‐autistic developmentally disordered (NADD) comparison children divided into developmental language disorder and non‐autism, low IQ (NALIQ) sub‐groups. The parts of the body involved and characteristics of all stereotypies were scored blind to diagnosis. More children with autism had stereotypies than the NADD comparison children. Autism and, to a lesser degree, nonverbal IQ (NVIQ) <80, especially in females contributed independently to the occurrence, number, and variety of stereotypies, with non‐autistic children without cognitive impairment having the least number of stereotypies and children with autism and low NVIQ the most. Autism contributed independently to gait and hand/finger stereotypies and NVIQ <80 to head/trunk stereotypies. Atypical gazing at fingers and objects was rare but virtually limited to autism. Stereotypies are environmentally modulated movement disorders, some highly suggestive, but not pathognomonic, of autism. Their underlying brain basis and genetic correlates need investigation.
Bacterial endocarditis in a child presenting with acute arterial ischemic stroke: should thrombolytic therapy be absolutely contraindicated?
Authors: TAN MARILYN , ARMSTRONG DEREK , BIRKEN CATHERINE , BITNUN ARI , CALDARONE CHRISTOPHER A , COX PETER , KAHR WALTER , MACGREGOR DAUNE , ASKALAN RAND
Published: June 2009

Thrombolysis is considered to be contraindicated in acute ischemic stroke secondary to infective endocarditis (IE). We report a 12‐year‐old female who presented with acute dense right hemiparesis and aphasia. Cranial magnetic resonance imaging and angiography showed multiple diffusion‐restricted lesions in the left hemisphere and absence of flow in the left internal carotid artery. She was treated with intra‐arterial tissue plasminogen activator within 6 hours of her presentation. Subsequently she was diagnosed with pneumococcal endocarditis and underwent debridement of vegetations and patch repair of the mitral valve. The patient did not have hemorrhagic complications following thrombolytic therapy or surgery. Pathological analysis of the mitral valve vegetations revealed mostly fibrin thrombus. Follow‐up imaging showed complete recanalization of the left internal carotid artery, and the patient had a remarkable neurological recovery. This is the first case report of successful intra‐arterial thrombolytic therapy in childhood IE‐related stroke. We believe that thrombolytic therapy contributed to a favorable outcome in our patient and may be safe in selected patients with childhood IE‐related acute ischemic stroke.
Long‐term effects of botulinum toxin A in children with cerebral palsy
Authors: TEDROFF KRISTINA , GRANATH FREDRIK , FORSSBERG HANS , HAGLUND‐AKERLIND YVONNE
Published: June 2009

The long‐term effects of botulinum toxin A (BoNT‐A) treatment in children with cerebral palsy (CP) are still elusive. We studied a prospective clinical cohort of 94 children with different subtypes (50% spastic diplegic CP, 22% hemiplegic CP, 25% tetraplegic CP, 3% dyskinetic CP), sex (55% male, 45% female), severity according to Gross Motor Function Classification System (29% Level I, 15% Level II, 16% Level III, 17% Level IV, 23% Level V), and age (median 5y 4mo, range 11mo–17y 8mo). The longest follow‐up time was 3 years 7 months (median 1y 6mo) and included a maximum of eight injections per muscle (median two injections to a specific muscle). Outcome measurements were muscle tone (Modified Ashworth Scale) and joint range of motion (ROM). Assessments were made at a minimum before and 3 months after each injection. Ninety‐five per cent confidence intervals for differences from baseline were used to identify significant changes. BoNT‐A injections induced reduction of long‐term spasticity in all muscle‐groups examined: the gastrocnemius, hamstring, and adductor muscles. The reduction in tone was most distinct in the gastrocnemius muscle, and each repeated injection produced an immediate reduction in muscle tone. However, improvement in ROM was brief and measured only after the first injections, whereupon the ROM declined. Thus, the results suggest that BoNT‐A can be effective in reducing muscle tone over a longer period, but not in preventing development of contractures in spastic muscles. The dissociation between the effects on muscle tone and ROM indicates that development of contractures is not coupled to increased muscle tone only, but might be caused by other mechanisms.
Systematic reviews of psychosocial interventions for autism: an umbrella review
Authors: SEIDA JENNIFER KREBS , OSPINA MARIA B , KARKHANEH MOHAMMAD , HARTLING LISA , SMITH VERONICA , CLARK BRENDA
Published: June 2009

Aim  A wide range of psychosocial interventions for the treatment of individuals with autism‐spectrum disorders (ASDs) have been evaluated in systematic reviews. We conducted an umbrella review of systematic reviews of the effectiveness of psychosocial interventions for ASD.

Method  Comprehensive searches were conducted in 25 bibliographic databases, relevant journals and reference lists up to May 2007. Studies included were systematic reviews on any psychosocial intervention for individuals with ASDs. Two reviewers independently assessed study relevance and quality.

Results  Thirty systematic reviews were included. The majority of reviews evaluated interventions based on behavioural theory (n=9) or communication‐focused (n=7) therapies. Positive intervention outcomes were reported in the majority of the reviews. Methodological quality of the reviews was generally poor.

Interpretation  The reviews reported positive outcomes for many of the interventions, suggesting that some form of treatment is favourable over no treatment. However, there is little evidence for the relative effectiveness of these treatment options. Many of the systematic reviews had methodological weaknesses that make them vulnerable to bias. There is a need for further systematic reviews that adhere to strict scientific methods and for primary studies that make direct comparisons between different treatment options.
The development of emotion and empathy skills after childhood brain injury
Authors: TONKS JAMES , SLATER ALAN , FRAMPTON IAN , WALL SARAH E , YATES PHIL , WILLIAMS W HUW
Published: June 2009

Lasting socio‐emotional behaviour difficulties are common among children who have suffered brain injuries. A proportion of difficulties may be attributed to impaired cognitive and/or executive skills after injury. A recent and rapidly accruing body of literature indicates that deficits in recognizing and responding to the emotions of others are also common. Little is known about the development of these skills after brain injury. In this paper we summarize emotion‐processing systems, and review the development of these systems across the span of childhood and adolescence. We describe critical phases in the development of emotion recognition skills and the potential for delayed effects after brain injury in earlier childhood. We argue that it is important to identify the specific nature of deficits in reading and responding to emotions after brain injury, so that assessments and early intervention strategies can be devised.
Effects of bisphosphonates in children with osteogenesis imperfecta: an AACPDM systematic review
Authors: CASTILLO HEIDI , SAMSON‐FANG LISA ,   ON BEHALF OF THE AMERICAN ACADEMY FOR CEREBRAL PALSY AND DEVELOPMENTAL MEDICINE TREATMENT OUTCOMES COMMITTEE REVIEW PANEL
Published: June 2009

This systematic review of the effects of bisphosphonate treatment in children with osteogenesis imperfecta was conducted using the American Academy for Cerebral Palsy and Developmental Medicine methodology for developing systematic reviews of treatment interventions (Revision 1.1) 2004. Despite a large body of published literature, there have been only eight studies with a sufficiently high level of internal validity to be truly informative. These studies confirm improvement in bone density. Many, but not all studies, demonstrate reduction in fracture rate and enhanced growth. There has been extremely limited evaluation of broader treatment impacts such as deformity, need for orthopedic surgery, pain, functioning, or quality of life. Short‐term side effects were minimal. Which medication and dosing regimen is optimal and how long patients should be treated are unclear. This body of evidence would be strengthened by a larger controlled trial, because many studies lacked adequate power to evaluate stated outcomes. These studies do not address the impacts of bisphosphonates in children with milder forms of osteogenesis imperfecta and severe forms that are not due to mutations in the type I pro‐collagen gene (e.g. types VII and VIII). Additional research is needed into treatment of infants. More studies evaluating medication choices, optimal dosing, duration of treatment, post‐treatment impacts, and long‐term side effects are necessary.
A randomized controlled trial of the impact of therapeutic horse riding on the quality of life, health, and function of children with cerebral palsy
Authors: DAVIS E , DAVIES B , WOLFE R , RAADSVELD R , HEINE B , THOMASON P , DOBSON FIONA , GRAHAM H K
Published: June 2009

This randomized controlled trial examined whether therapeutic horse riding has a clinically significant impact on the physical function, health and quality of life (QoL) of children with cerebral palsy (CP). Ninety‐nine children aged 4 to 12 years with no prior horse riding experience and various levels of impairment (Gross Motor Function Classification System Levels I−III) were randomized to intervention (10wks therapeutic programme; 26 males, 24 females; mean age 7y 8mo [SD 2y 5mo] or control (usual activities, 27 males, 22 females; mean age 8y 2mo [SD 2y 6mo]). Pre‐ and post‐measures were completed by 72 families (35 intervention and 37 control). Children’s gross motor function (Gross Motor Function Measure [GMFM]), health status (Child Health Questionnaire [CHQ]), and QoL (CP QoL‐Child, KIDSCREEN) were assessed by parents and QoL was assessed by children before and after the 10‐week study period. On analysis of covariance, there was no statistically significant difference in GMFM, CP QoL‐Child (parent report and child self‐report), and CHQ scores (except family cohesion) between the intervention and control group after the 10‐week study period, but there was weak evidence of a difference for KIDSCREEN (parent report). This study suggests that therapeutic horse riding does not have a clinically significant impact on children with CP. However, a smaller effect cannot be ruled out and the absence of evidence might be explained by a lack of sensitivity of the instruments since the QoL and health measures have not yet been demonstrated to be sensitive to change for children with CP.
Manganese induces sustained Ser40 phosphorylation and activation of tyrosine hydroxylase in PC12 cells
Authors: Posser Thaís , Franco Jeferson L. , Bobrovskaya Larisa , Leal Rodrigo B. , Dickson Phillip W. , Dunkley Peter R.
Published: June 2009

Manganese (Mn2+) is an essential metal involved in normal functioning of a range of physiological processes. However, occupational overexposure to Mn2+ causes neurotoxicity. The dopaminergic system is a particular target for Mn2+ neurotoxicity. Tyrosine hydroxylase (TH) is the rate limiting enzyme for dopamine synthesis and is regulated acutely by phosphorylation at Ser40 and chronically by protein synthesis. In this study we used pheochromocytoma 12 cells to investigate the effects of Mn2+ exposure on the phosphorylation and activity of TH. Mn2+ treatment for 24 h caused a sustained increase in Ser40 phosphorylation and TH activity at a concentration of 100 μM, without altering the level of TH protein or PC12 cell viability. Inhibition of protein kinase A and protein kinase C and protein kinases known to be involved in sustained phosphorylation of TH in response to other stimuli did not block the effects of Mn2+ on Ser40 phosphorylation. A substantial increase in H2O2 production occurred in response to 100 μM Mn2+. The antioxidant TroloxTM completely inhibited H2O2 production but did not block TH phosphorylation at Ser40, indicating that oxidative stress was not involved. Sustained TH phosphorylation at Ser40 and the consequent activation of TH both occurred at low concentrations of Mn2+ and this provides a potential new mechanism for Mn2+‐induced neuronal action that does not involve H2O2‐mediated cell death.
Acute – an ambiguous concept in healthcare
Authors: Gabrielsen Elisabeth , Lindström Unni Å. , Nåden Dagfinn
Published: June 2009

The aim of this study was to explore the concept acute semantically. The concept is being used both in clinical and theoretical contexts, without questioning whether a clear meaning of the concept has been established. The analysis has been applied according to the semantic analysis strategy of the philosopher (and pedagogue) Koort (1975). First, the results show that the concept of acute has had an unclear meaning and perception in the Norwegian language. Second, the synonyms indicate two different contents. The experiences and events that occur acute might be experienced as ‘sharp’ and ‘intense’. The synonyms point also at time, such as quick, rapid and swift. Third, it reveals that the shortlived experience as synonym does have a very little binding to the concept acute. The occurence that began suddenly is not meant to subside the same way as it began.
New observations on the splenius capitis and rectus capitis ventralis muscles of the Common Swift Apus apus (Apodidae)
Authors: BRAUSE CRISTETA , GASSE HAGEN , MAYR GERALD
Published: June 2009

In swifts and hummingbirds (Apodiformes), the splenius capitis muscle displays a characteristic modification, the ‘cruciform origin’. The muscle pairs arise from the second vertebra by several slips which criss‐cross and interdigitate with each other, before inserting into the skull. In the course of a study on the neck muscles of the Common Swift, we paid special attention to the arrangement of these slips of the splenius capitis muscle, and noted a previously unrecognized individual modification of this muscle. In addition, we observed an incipient cruciform origin of the deep portion (slip) of the rectus capitis ventralis muscle that has not been noticed for swifts or any other avian taxon before. The development and function of these modifications of the splenius capitis and rectus capitis ventralis muscle are discussed.
CHARGE CONTRAST IMAGING OF EXCEPTIONALLY‐PRESERVED FOSSILS
Authors: KEARNS STUART L. , ORR PATRICK J.
Published: June 2009

Abstract:  Charge contrast images are a variant of secondary electron images acquired by operating a variable pressure scanning electron microscope in low vacuum mode; i.e. a gas is present in the specimen chamber. Spatial variation in the amount of charge that accumulates on the surface of the specimen is expressed as differences in greyscale tone; areas that are charging less are darker in tone. The precise mechanisms by which charge contrast images are generated are not known fully. Various different properties of a mineral may create a charge contrast; electrical conductivity is known to be one potentially important variable. As carbon is highly conductive but typical host lithologies (carbonates, silicates) less so or dielectric, the technique is potentially very suitable for imaging organically preserved fossils such as those from the Solite and Jehol biotas. It can also be applied to Burgess Shale fossils: complex films comprising ‘aluminosilicates’ with or without carbonaceous remains. Charge contrast images reveal anatomical detail not visible using either optical or other scanning electron microscope‐based imaging methods.
Medicaid Managed Care and Health Care Access for Adult Beneficiaries with Disabilities
Authors: Burns Marguerite E.
Published: June 2009

Objective. To evaluate the impact of Medicaid managed care organizations (MCO) on health care access for adults with disabilities (AWDs).

Data Sources. Mandatory and voluntary enrollment data for AWDs in Medicaid MCOs in each county were merged with the Medical Expenditure Panel Survey and the Area Resource File for 1996–2004.

Study Design. I use logit regression and two evaluation perspectives to compare access and preventive care for AWDs in Medicaid MCOs with FFS. From the state's perspective, I compare AWDs in counties with mandatory, voluntary, and no MCOs. From the enrollee's perspective, I compare AWDs who must enroll in an MCO or FFS to those who may choose between them.

Principal Findings. Mandatory MCO enrollees are 24.9 percent more likely to wait >30 minutes to see a provider, 32 percent more likely to report a problem accessing a specialist, and 10 percent less likely to receive a flu shot within the past year. These differences persist from the state evaluation perspective.

Conclusions. States should not expect a dramatic change in health care access when they implement Medicaid MCOs to deliver care to the adult disabled population. However, continued attention to specialty care access is warranted for mandatory MCO enrollees.
Comparative life history and fecundity of Helicoverpa armigera (Hubner) (Lepidoptera: Noctuidae) on different soybean varieties
Authors: NASERI Bahram , FATHIPOUR Yaghoub , MOHARRAMIPOUR Saeid , HOSSEININAVEH Vahid
Published: June 2009

The effect of different soybean varieties (Glycine max 356, M4, M7, M9, Clark, Sahar, JK, BP, Williams, L17, Zane, Gorgan3 and DPX) on the life history and fecundity of the gram pod borer Helicoverpa armigera (Hubner) (synonym Heliothis armigera (Hubner), also known as the American or African bollworm) was determined at 25 ± 1°C, 65 ± 5% r.h. and a light : dark cycle of 16:8 h. The larval period ranged 17.30 to 26.20 days on M7 and L17, respectively. The longest development time was on L17, BP and Sahar (42.71, 40.29 and 39.20 days, respectively) and the shortest was on M7, M9, Williams and Clark (34.21, 36.06, 36.60 and 36.82 days, respectively). The development index of overall immature stages varied from 1.39 to 2.41, with the minimum on L17 and BP and the maximum on M7. The highest daily fecundity and total fecundity (118.92 and 582.70 eggs, respectively) and the lowest (37.88 and 177.10 eggs, respectively) were observed on DPX and 356, respectively. Cluster analysis of the biological parameters of H. armigera on different soybean varieties demonstrated that BP, Sahar and L17 were partially resistant to H. armigera. Knowledge of the extent of susceptibility or resistance of crop varieties and biology of a pest on that crop are fundamental components of integrated pest management (IPM) programs for many crops. Such information can help to detect and monitor pest infestation, variety selection and crop breeding.