bluuebunny/crossref_metadata_2025 huggingface dataset:
A descriptive analysis of studies on behavioural treatment of drooling (1970–2005)
Authors: Van der Burg Jan J W , Didden Robert , Jongerius Peter H , Rotteveel Jan J
Published: June 2009

A descriptive analysis was conducted on studies on the behavioural treatment of drooling (published between 1970 and 2005). The 17 articles that met the inclusion criteria described 53 participants (mean age 14y 7mo, [SD 4y 9mo]; range 6‐28y). Sex of 87% of the participants was reported: 28 male, 18 female. For 60% of the participants the degree of learning disability was reported, varying from severe/profound (n=24, 75%), moderate (n=4, 13%), to mild (n=2, 6%), while two participants (6%) had no learning disabilities. Forty‐two participants (79%) were diagnosed with cerebral palsy. Behavioural procedures included instruction, positive and negative reinforcement, overcorrection and restitution, verbal and automatic cueing, and/or self‐management. Effective behavioural procedures are reported in children with and without learning disability and/or motor impairment. Even participants with profound learning disability may benefit from behavioural intervention. However, the evidence base in terms of number of studies in this area is limited. Fifteen studies used a single participant design; two studies implemented an experimental‐comparison group design. Some of these studies were poorly designed and methodological flaws were identified. Therefore, conclusions about efficacy of behaviour therapy for drooling and/or best practice cannot be drawn, although our analysis suggests that this approach is promising. However, future research on this topic is needed. After years of research focused on medical treatment, the option of behavioural treatment to reduce drooling should be reconsidered in relation to the medical management of this problem.
Neuromotor task training for children with developmental coordination disorder: a controlled trial
Authors: Niemeijer A S , Smits‐Engelsman B C M , Schoemaker M M
Published: June 2009

The aim of this study was to evaluate neuromotor task training (NTT), a recently developed child‐centred and task‐oriented treatment programme for children with developmental coordination disorder (DCD). A treatment and a non‐treatment control group of children with DCD were included. Children were selected if they scored below the 15th centile on the Movement Assessment Battery for Children (MABC). The children in the treatment group were recently referred for physiotherapy (n=26; 20 males, 6 females; mean age 7y 2mo [SD 1y 3mo]). The parents of the non‐treated children were concerned about their children's motor performance and responded to advertisements for free testing (n=13; 10 males, 3 females; mean age 7y 2mo [SD 2y 1mo]). Before and after nine weekly 30‐minute sessions of NTT or at least 9 weeks of no intervention, the MABC and the Test of Gross Motor Development ‐ 2 (TGMD‐2) were administered. Therapists reported per session on treatment goals and tasks trained. The results indicate that motor performance does not improve spontaneously and that NTT is effective. During the intervention period, only the treated group improved on the MABC and the TGMD‐2. Children improved most on tasks similar to those trained. In older children with poorer motor patterns, NTT's treatment success was higher. The Child Behavior Checklist subscales withdrawn, thought problems, anxious/depressed, and delinquency were determinants of effects on motor patterns.
Cerebrospinal fluid baclofen concentrations in patients undergoing continuous intrathecal baclofen therapy
Authors: Albright A Leland , Thompson Kristen , Carlos Signe , Minnigh M Beth
Published: June 2009

The aim of this study was to report concentrations of cerebrospinal fluid (CSF) baclofen in children undergoing chronic intrathecal baclofen (ITB) infusions. CSF baclofen concentrations were analyzed in 53 specimens obtained by intrathecal catheter aspiration from 43 participants (28 males, 15 females; range 3‐44y, mean 16y [SD 8y 11mo]), with functioning baclofen pumps and catheters. Daily ITB doses ranged from 70 to 1395μg per day (mean 607μg per day [SD 363], median 575). Baclofen concentration was quantified by high‐pressure liquid chromatography and confirmed by injection onto a gas chromatograph. CSF baclofen concentrations from children receiving either simple continuous or complex infusions ranged from 0.2 to 20.0μg/ml (mean 4.64μg/ml, median 3.3μg/ml). CSF baclofen concentrations from children receiving simple continuous infusions ranged from 0.5 to 12.9 (mean 4.7μg/ml, median 3.55μg/ml). There was no correlation between ITB dosage and CSF baclofen concentration. We conclude that baclofen concentration can be measured to determine if baclofen is present in CSF. However, there appears to be no correlation between the ITB dose infused and the corresponding CSF baclofen level.
Hyoscine skin patches for drooling dilate pupils and impair accommodation: spectacle correction for photophobia and blurred vision may be warranted
Authors: Saeed Manzar , Henderson Gladys , Dutton Gordon N
Published: June 2009

Hyoscine skin patches diminish salivation by their anticholinergic action. The aim of reporting this case series is to present the ophthalmic side effects in children, and to highlight the precautions to take. Five children (two males, three females; age range 8‐18y) with quadraplegic cerebral palsy (Gross Motor Function Classification System Level V) and profound intellectual impairment, wearing hyoscine skin patches to control excessive salivation, were examined. Binocular visual acuity, pupil reaction, pupil diameter, and dynamic retinoscopy were recorded before and after instilling guttae cyclopentolate 1%. The accommodative responses were compared with the expected norms. Spectacle correction was provided for refractive error and to compensate for lack of accommodation. Tinted lenses were provided for photophobia. Visual assessment was repeated 6 months later. All children had dilated pupils with a mean diameter of 7.8mm (range 7‐9mm) before instilling guttae cyclopentolate. Mean pupil constriction to bright light was only 1.8mm (range 1‐2mm). Mean resting accommodation was 0.45 dioptres (D; range 0‐1D) and no accommodative responses were detected on dynamic retinoscopy. Three children were photophobic. Visual behaviour was seen to improve after the use of appropriate spectacles. Photophobia was relieved by tinted lenses. The anticholinergic effects of hyoscine skin patches can cause photophobia and impair vision due to pupil dilatation and paralysis of accommodation. Appropriate spectacles are recommended.
Autoimmune diseases in parents of children with infantile autism: a case—control study
Authors: Mouridsen Svend Erik , Rich Bente , Isager Torben , Nedergaard Niels Jørgen
Published: June 2009

This register study compared the rates and types of autoimmune disease in the parents of 111 patients (82 males, 29 females; mean age at diagnosis 5y 5mo [SD 2y 6mo]) with infantile autism (IA) with a matched control group of parents of 330 children from the general population. All parents were screened through the nationwide Danish National Hospital Register. We inquired about 35 autoimmune diseases during an observation period of 27 years. At follow‐up the case and control mothers were identical in age (65y 7mo [SDs 9y 7mo and 9y respectively]). For case and control fathers the figures were 70 years 2 months (SD 10y 2mo) and 69 years 1 month (SD 10y 1mo) respectively. A similar proportion of case and control mothers had a diagnosis of any autoimmune disease: 10.8% versus 9.1%. For case fathers the proportion was 8.6% versus 4.6%. Two autoimmune conditions were associated with IA: ulcerative colitis in mothers (p=0.05) and type 1 diabetes in fathers (p=0.02). Additional studies are required to determine whether there is a true association between a parental history of autoimmune disease and pervasive developmental disorder in their offspring.
Reduced parietal and visual cortical activation during global processing in Williams syndrome
Authors: Mobbs Dean , Eckert Mark A , Menon Vinod , Mills Debbie , Korenberg Julie , Galaburda Albert M , Rose Fred E , Bellugi Ursula , Reiss Allan L
Published: June 2009

Several lines of investigation suggest that individuals with Williams syndrome (WS), a neurodevelopmental disorder of well‐characterized genetic etiology, have selective impairments in integrating local image elements into global configurations. We compared global processing abilities in 10 clinically and genetically diagnosed participants with WS (eight females, two males; mean age 31y 10mo [SD 9y 7mo], range 15y 5mo‐48y 4mo) with a typically developed (TD) age‐ and sex‐matched comparison group (seven females, one male; mean age 35y 2mo [SD 10y 10mo], range 24y‐54y 7mo) using functional magnetic resonance imaging (fMRI). Behavioral data showed participants with WS to be significantly less accurate (p<0.042) together with a non‐significant trend to be slower than the TD comparison group while performing the global processing task. fMRI data showed participants with WS to possess reduced activation in the visual and parietal cortices. Participants with WS also showed relatively normal activation in the ventral occipitotemporal cortex, but elevated activation in several posterior thalamic nuclei. These preliminary results largely confirm previous research findings and neural models implicating neurodevelopmental abnormalities in extended subcortical and cortical visual systems in WS, most notably dorsal‐stream pathways.
Effects of oral stimulation and oral support on non‐nutritive sucking and feeding performance in preterm infants
Authors: Boiron M , Nobrega L Da , Roux S , Henrot A , Saliba E
Published: June 2009

This study compared the effects of oral stimulation with those of oral support on non‐nutritive sucking and feeding parameters in preterm infants. Preterm infants (23 males, 20 females) born between 29 and less than 34 weeks' gestational age (GA; mean GA 31.2wks [standard error of mean{SEM} 0.39]; mean birth‐weight 1580g [SEM 120]) were allocated to one of three experimental groups: (Stimulation+support [five males, four females]; Stimulation [four males, seven females]; and Support [seven males, five females]) or a control group. Non‐nutritive sucking pressure and sucking activity were quantified in the gavage and transition periods. Oral support minimizes fluid loss, stabilizes the jaw, and organizes deglutition. The time of transition, the quantity of milk ingested per day, and the number of bottle feeds per day were recorded. Variables were analyzed by repeated‐measures analysis of variance, with birth‐weight as covariate (ANCOVA). Transition time was reduced (p<0.0001) for the Stimulation+support and Support groups. ANCOVA computed during gavage showed increased non‐nutritive sucking pressure and sucking activity (p<0.001) for the Stimulation and Stimulation+support groups. ANCOVA computed during transition revealed increases in non‐nutritive sucking pressure and daily bottle feeds (p<0.001) for the three experimental groups and in daily milk ingested (p=0.002) for the Stimulation+support and Support groups. We demonstrated that oral support is the result of both the action of chin and cheek support, and the aid to deglutition. An analysis of the organization of sucking patterns should be undertaken to provide better understanding of the mechanisms involved in oral support.
Physical and sedentary activity in adolescents with cerebral palsy
Authors: Maher Carol A , Williams Marie T , Olds Tim , Lane Alison E
Published: June 2009

Participation in regular physical activity (PA) provides health, psychological, and physiological benefits for people with and without a physical disability. This study investigated the physical and sedentary activity patterns of adolescents with cerebral palsy (CP). A cross‐sectional, descriptive, postal survey was used, consisting of the Physical Activity Questionnaire for Adolescents (PAQ‐A), self‐reported level of gross motor function (based on the Gross Motor Function Classification System [GMFCS]), and specific questions regarding weekly sedentary activities. Following piloting to determine test‐retest reliability (intraclass correlation [ICC] for PA=0.90; total weekly sedentary time=0.84) and concurrent validity (survey PA score vs pedometry, Pearson'sr=0.24; survey PA score vs accelerometry,r=‐0.21; survey weekly sedentary time vs logbook,r=0.38), the survey was mailed to all adolescents with CP in South Australia registered with Novita Children's Services (n=219). One hundred and twelve valid surveys were returned (76 males, 36 females; age range 11‐17y, mean age 13y 11mo [SD 23mo]; GMFCS Level I,n=42; Level II,n=27; Level III,n=10; Level IV,n=17; Level V,n=15; level not reported,n=1). Results were compared with recent normative age‐ and sex‐matched data sets. Key findings were that PA level of adolescents with CP was related to level of gross motor function and inversely related to age, and that adolescents with CP were less physically active than their peers without disability. Comparisons with normative data sets suggested that adolescents with CP tend to participate in less structured and lower intensity PA compared with non‐disabled adolescents, though sedentary activity patterns (TV and computer use) of adolescents with and without CP were similar.
Life satisfaction of young adults with spina bifida
Authors: Barf HA , Post M W M , Verhoef M , Jennekens‐Schinkel A , Gooskens R H J M , Prevo A J H
Published: June 2009

This study concerns life satisfaction and its determinants in Dutch young adults with spina bifida (SB). Data on life satisfaction (Life Satisfaction Questionnaire [LiSat‐9]) were related to hydrocephalus, lesion level, disabilities, and demographic variables. In total, 179 young adults with SB participated (41% male, age range 16‐25y; 79% SB aperta, 67% hydrocephalus [HC], 39% wheelchair‐dependent). Most were satisfied with their life as a whole (24% dissatisfied). No difference was found from a population reference group (28% dissatisfied). Highest proportions of dissatisfaction were found for financial situation (44%), partnership relations (49%), and sex life (55%). Least dissatisfaction was found for contact with friends (17%) and families (15%). Young adults with SB and HC were more satisfied with their financial situation and family life but were less satisfied with self‐care ability and partnership relations than those without HC and the reference group. However, except for self‐care ability, relationships between life satisfaction and having SB were weak. In conclusion, self‐care ability and partnership relations were rated least favourable and may need more attention from care providers. Overall, SB does not seem to be an important determinant of life satisfaction.
Aicardi syndrome associated with anterior cephalocele in a female infant
Authors: Melbourne‐Chambers R , Singh Minott I , Mowatt L , Johnson P , Thame M
Published: June 2009

Aicardi syndrome is a triad of abnormalities that includes total or partial agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. This syndrome was first described in 1965. A female infant with Aicardi syndrome associated with a nasoethmoidal cephalocele is described in this report. She presented with a history of unilateral nasal discharge since birth and seizures since age 1 week. She was microcephalic and there was visual impairment. A fleshy mass of the left nostril was noted. Ophthalmological evaluation revealed left exotropia, dysplastic optic discs and retina, ‘morning glory’ appearance of the left optic disc, and bilateral chorioretinal lacunae. Magnetic resonance imaging of the brain showed absence of the corpus callosum, dysmorphic changes of the lateral ventricles, a superiorly located third ventricle, heterotopic grey matter of the frontal lobes, a left nasoethmoidal cephalocele, and closed lip schizencephaly of the left frontal lobe. This female infant developed asymmetric infantile spasms at age 8 weeks. Surgical correction of the cephalocele was declined. She developed recurrent pneumonias secondary to aspiration of feeds and died at age 8 months during one of these events.
Current approaches to intervention in children with developmental coordination disorder
Authors: Sugden David
Published: June 2009

This review analyzes approaches to intervention in children with developmental coordination disorder within the framework of how children develop and learn motor skills, drawing upon maturational, cognitive, and dynamic systems models. The approaches to intervention are divided into two categories: (1) process or deficit‐oriented approaches; and (2) approaches that teach specific functional skills. These approaches are viewed alongside theoretical, empirical, and experiential standpoints, noting the differences and the fact no single approach is, as yet, fully substantiated. Principles and guidelines are drawn from the analyses that support both cognitive and dynamic models and that are set within an ecological framework.
Behavior and social competency in idiopathic and cryptogenic childhood epilepsy
Authors: Berg Anne T , Vickrey Barbara G , Testa Francine M , Levy Susan R , Shinnar Shlomo , DiMario Francis
Published: June 2009

Behaviroal and related disorders are frequently reported in association with childhood epilepsy but the reasons for this are unclear. In a long‐term prospective, community‐based study of newly‐diagnosed childhood epilepsy, behavioral assessments (Child Behavior Checklist) were performed in children 8 to 9 years after the initial diagnosis of epilepsy to determine the impact of remission and medication status on behavioral problems. Children with epilepsy were also compared with sibling controls. A total of 226 children (108 females, 118 males; mean age 13y 1mo [SD 2y 8mo], range 8‐17y) with idiopathic or cryptogenic epilepsy were included in the analyses. One hundred and twenty‐eight matched pairs were included in analyses of case‐sibling differences. Lack of remission and current medication use were associated with worse behavioral problem and competency scores. Lack of remission generally had a greater effect than medication use, except for attention problems; medication status had the more deleterious effect (p<0.001). Children with epilepsy had significantly worse behavioral problems and competency scores relative to sibling controls. Even in paris in which the patient was seizure‐free and off medication, significant case‐sibling differences persisted for most scales (p=0.05 to p=0.001). Lack of remission and continued use of antiepileptic drugs have a negative influence on behavioral problems in children with epilepsy but do not fully explain the worse scores relative to siblings. This suggests an independent effect associated with the epilepsy itself.
Seizure risk in patients with attention‐deficit‐hyperactivity disorder treated with atomoxetine
Authors: Wernicke Joachim F , Holdridge Karen Chilcott , Jin Ling , Edison Timothy , Zhang Shuyu , Bangs Mark E , Allen Albert J , Ball Susan , Dunn David
Published: June 2009

The comorbidity of seizures, epilepsy, and attention‐deficit‐hyperactivity disorder (ADHD) prompted the examination of whether atomoxetine use for ADHD is associated with an increased risk of seizures. Seizures and seizure‐related symptoms were reviewed from two independent Eli Lilly and Company databases: the atomoxetine clinical trials database and the atomoxetine postmarketing spontaneous adverse event database. Review of clinical trial data indicated that the crude incidence rates of seizure adverse events were between 0.1 and 0.2%, and were not significantly different between atomoxetine, placebo, and methylphenidate. Only 2% of the postmarketing spontaneous reports of seizure events were classified as having no clear contributing or confounding factors, and the reporting rate (8 per 100 000 patients exposed) was within the expected range of population‐based incidence. Although children with ADHD are increasingly recognized as being at an elevated risk for seizures, treatment of ADHD symptoms with atomoxetine does not appear to elevate this risk further. The shared vulnerability between ADHD and seizure activity should be taken into account when making treatment decisions for populations of children with epilepsy and children with ADHD.
Vision screening in children with developmental delay can be improved: analysis of a screening programme outside the ophthalmic clinic
Authors: Nielsen Lisbeth Sandfeld , Skov Liselotte , Jensen Hanne
Published: June 2009

The purpose of the present study was to evaluate the effectiveness of a new vision‐screening programme in detecting ophthalmic disorders in children with developmental delay (DD; IQ80) and in children with normal IQ. A group of 467 children with an IQ80 (272 males, 195 females; age range 4‐15y; mean age 10y 4mo [SD 2y 10mo]) and 123 children with an IQ>80 (85 males, 38 females; age range 3‐16y; mean age 10y 5mo [SD 3y 2mo]) had an examination that included new vision‐screening items: distance and near visual acuity and stereopsis for near objects (Lang stereo test II). A full ophthalmological examination was also conducted to determine the effectiveness of the new screening items. The previous screening programme consisted of only monocular visual acuity at distance. Sensitivity, specificty, the positive predictive value (PPV), and negative predictive value (NPV) of the tests were calculated with regard to the following ophthalmic disorders: hyperopia, myopia, astigmatism, anistometropia, amblyopia, low vision, and strabismus. Overall, the prevalence of ophthalmic disorders was 33.4% in children with DD and 11.4% in typically developing children. With the use of the new programme, the effectiveness of vision screening in both groups of children was improved. In children with DD, sensitivity increased from 49.4 to 80.1%, specificity decreased from 98.1 to 83.3%, PPV decreased from 92.8 to 70.6%, and NPV increased from 79.4 to 89.3%. In typically developing children, sensitivity improved from 50.0 to 85.7%, specificity declined from 98.2 to 87.2%, PPV decreased from 77.8 to 46.2%, and NPV increased from 93.9 to 97.9%. We conclude that the currently used vision‐screening programme can be significantly improved. The speed and simplicity of the proposed screening programme makes it suitable for use by school nurses. The improvements were most prominent in children with DD.
Surveillance of vision and ocular disorders in children with Down syndrome
Authors: Stephen Elma , Dickson Jennifer , Kindley A David , Scott Christopher C , Charleton Patricia M
Published: June 2009

Children with Down syndrome have a high prevalence of ocular disorders. The UK Down's Syndrome Medical Interest Group (DSMIG) guidelines for ophthalmic screening were locally implemented into a protocol that included neonatal eye examination by an opthalmologist and a comprehensive ophthalmological examination (cycloplegic refraction, ophthalmoscopy, and orthoptic assessement) by at least the age of 3 years, followed by preschool follow‐up as indicated. We audited retrospectively surveillance for ocular disorders before and after the DSMIG‐based guidelines were locally adopted in 1995. Results were compared for children born before and after the implementation of screening guidelines. A total of 81 children (43 females, 38 males) with Down syndrome were identified. After the DSMIG protocol, 34/36 children received a full ophthalmological examination in the neonatal period, compared with 9/27 children before 1995 (p<0.001). Neonatal screening resulted in the detection of cataracts in three infants. Mean age of first comprehensive ophthalmic screening outside the neonatal period was similar in the two groups (1y 6mo before guidelines vs 1y 9mo after), as were the proportion of children receiving preschool eye checks (27/30 before; 17/18 after). Overall, 65.7% children were screened in accordance with the guidelines, improving to 100% in recent years. At school age, 43% of the study population had significant refractive errors, with 27% having hypermetropia and astigmatism. Earlier prescription of glasses for refractive errors was seen (mean age 5y 6mo before guidelines; 3y 6mo after; p<0.001). Prevalence of other ocular disorders included strabismus (34/72, 47%), nasolacrimal duct obstruction (26/73, 35.6%), cataracts (5/64, 7.8%), and nystagmus (12/72, 16%). Establishment of the DSMIG‐based local protocol has streamlined ocular surveillance. It is anticipated that this will improve developmental and functional outcomes in Down syndrome.
Quality of life and health‐related quality of life of adolescents with cerebral palsy
Authors: Rosenbaum Peter L , Livingston Michael H , Palisano Robert J , Galuppi Barbara E , Russell Dianne J
Published: June 2009

This study assessed quality of life (QOL) and health‐related quality of life (HRQOL) of 203 adolescents with cerebral palsy (111 males, 92 females; mean age 16y [SD 1y 9mo]). Participants were classified using the Gross Motor Function Classification System (GMFCS), as Level I (n=60), Level II (n=33), Level III (n=28), Level IV (n=50), or Level V (n=32). QOL was assessed by self (66.5%) or by proxy (33.5%) with the Quality of Life Instrument for People With Developmental Disabilities, which asks about the importance and satisfaction associated with the QOL domains of Being, Belonging, and Becoming; HRQOL was captured through proxy reports with the Health Utilities Index, Mark 3 (HUI3), which characterizes health in terms of eight attributes, each having five or six ordered levels of function. GMFCS level was not a source of variation for QOL domain scores but was significantly associated with the eight HRQOL attributes and overall HUI3 utility scores (p<0.05). Some QOL domain scores varied significantly by type of respondent (self vs proxy; p<0.05). Overall HUI3 utility values were significantly but weakly correlated with QOL Instrument scores for Being (r=0.37), Belonging (r=0.17), Becoming (r=0.20), and Overall QOL (r=0.28), and thus explain up to 14% of the variance (r2). These findings suggest that although QOL and HRQOL are somewhat related conceptually, they are different constructs and need to be considered as separate dimensions of the lives of people with functional limitations.
Interobserver agreement of the Gross Motor Function Classification System in an ambulant population of children with cerebral palsy
Authors: McDowell Brona C , Kerr Claire , Parkes Jackie
Published: June 2009

Gross Motor Function Classification System (GMFCS) level was reported by three independent assessors in a population of children with cerebral palsy (CP) aged between 4 and 18 years (n=184; 112 males, 72 females; mean age 10y 10mo [SD 3y 7mo]). A software algorithm also provided a computed GMFCS level from a regional CP registry. Participants had clinical diagnoses of unilateral (n=94) and bilateral (n=84) spastic CP, ataxia (n=4), dyskinesia (n=1), and hypotonia (n=1), and could walk independently with or without the use of an aid (GMFCS Levels I‐IV). Research physiotherapist (n=184) and parent/guardian data (n=178) were collected in a research environment. Data from the child's community physiotherapist (n=143) were obtained by postal questionnaire. Results, using the kappa statistic with linear weighting (κ1w), showed good agreement between the parent/guardian and research physiotherapist (κ1w=0.75) with more moderate levels of agreement between the clinical physiotherapist and researcher (κ1w=0.64) and the clinical physiotherapist and parent/guardian (κ1w=0.57). Agreement was consistently better for older children (>2y). This study has shown that agreement with parent report increases with therapists’experience of the GMFCS and knowledge of the child at the time of grading. Substantial agreement between a computed GMFCS and an experienced therapist (κ1w=0.74) also demonstrates the potential for extrapolation of GMFCS rating from an existing CP registry, providing the latter has sufficient data on locomotor ability.
Low and high frequency repetitive transcranial magnetic stimulation for the treatment of spasticity
Authors: Valle Angela C , Dionisio Karen , Pitskel Naomi Bass , Pascual‐Leone Alvaro , Orsati Fernanda , Ferreira Merari J L , Boggio Paulo S , Lima Moises C , Rigonatti Sergio P , Fregni Felipe
Published: June 2009

The development of non‐invasive techniques of cortical stimulation, such as transcranial magnetic stimulation (TMS), has opened new potential avenues for the treatment of neuropsychiatric diseases. We hypothesized that an increase in the activity in the motor cortex by cortical stimulation would increase its inhibitory influence on spinal excitability through the corticospinal tract and, thus, reduce the hyperactivity of the gamma and alpha neurons, improving spasticity. Seventeen participants (eight males, nine females; mean age 9y 1mo [SD 3y 2mo]) with cerebral palsy and spastic quadriplegia were randomized to receive sham, active 1Hz, or active 5Hz repetitive TMS of the primary motor cortex. Stimulation was applied for 5 consecutive days (90% of motor threshold). The results showed that there was a significant reduction of spasticity after 5Hz, but not sham or 1Hz, stimulation as indexed by the degree of passive movement; however this was not evident when using the Ashworth scale, although a trend for improvement was seen for elbow movement. The safety evaluation showed that stimulation with either 1Hz or 5Hz did not result in any adverse events as compared with sham stimulation. Results of this trial provide initial evidence to support further trials exploring the use of cortical stimulation in the treatment of spasticity.
Evaluating interventions to improve gait in cerebral palsy: a meta‐analysis of spatiotemporal measures
Authors: Paul Scott M , Siegel Karen Lohmann , Malley James , Jaeger Robert J
Published: June 2009

A number of interventions to improve gait in individuals with cerebral palsy (CP) have been reported in the literature. The aim of this study was to perform a meta‐analysis of these studies to determine the overall efficacy of these interventions. Effect sizes (Hedge's g) for spatiotemporal measures of gait (velocity, cadence, stride length) pre‐ and postintervention were analyzed. Sixty‐three studies were included, and the overall effect size was statistically significant for both fixed effects and random models. Types of interventions were grouped into spasticity treatments, orthopedic (bony and soft tissue) surgery, lower extremity orthoses, or ‘other’. When the data were analyzed in subgroups by type of intervention, each intervention had a statistically significant effect size with the exception of the ‘other’. More importantly, the present study indicates the need to address participant inclusion criteria and power analysis more adequately in future research studies of interventions to improve gait in CP.
Goal attainment scaling in paediatric rehabilitation: a critical review of the literature
Authors: Steenbeek Duco , Ketelaar Marjolijn , Galama Krys , Gorter Jan Willem
Published: June 2009

The aim of the study was to review the psychometric properties and use of goal attainment scaling (GAS) in paediatric rehabilitation research. We performed a critical literature review searching: (1) all studies whose main focus was to assess the psychometric properties of GAS in paediatric rehabilitation; and (2) all effect studies in paediatric rehabilitation that used GAS as one of the outcome measures. Three articles in the first group and six in the second group met the inclusion criteria. None of the studies had investigated the content reliability of the scales. Interrater reliability had been investigated in one study and had been found to be good. Only one trial had assessed the content validity of the developed scales, which was found to be acceptable. Comparisons showed that GAS, because of its idiosyncratic nature, measures different constructs from those measured by some related instruments. Low concurrent validity was found. All included studies reported good sensitivity to change. We conclude that the literature supports promising qualities of GAS in paediatric rehabilitation. GAS is a responsive method for individual goal setting and for treatment evaluation. However, current knowledge about its reliability when used with children is insufficient. There is a need for further development of GAS and its application for children of different ages and disabilities, across therapists of different disciplines.
A qualitative study of the health‐related quality of life of disabled children
Authors: Young Bridget , Rice Helen , Dixon‐Woods Mary , Colver Allan F , Parkinson Kathryn N
Published: June 2009

This qualitative study investigated what disabled children thought most important in their lives and examined how well their priorities are represented in KIDSCREEN, a generic health‐related quality of life (HRQoL) instrument. Participants were a subgroup of families who had previously taken part in a study of quality of life and participation in children with cerebral palsy (CP) using KIDSCREEN. This subgroup was sampled purposively, using children's scores on KIDSCREEN and demographic characteristics. Twenty‐eight children (15 males, 13 females; age range 8y 3mo‐13y 5mo) and 35 parents were interviewed. Ten children were at Gross Motor Function Classification System Level I, 15 were at Levels II or III, and three were at Levels IV or V. Eleven children had unilateral spastic CP, 16 had bilateral spastic C P, and one child had dyskinetic C P. Interviews were audio‐recorded and transcribed verbatim. The analysis was based on the constant comparative method and focused largely on the children's data, though the parent data were drawn upon to illuminate the children's data. Four overarching areas of HRQoL were identified: social relationships; home and school environment; self and body; and recreational activities and resources. These generally mapped well to the dimensions and items in KIDSCREEN. The precedence children gave to environmental, social, interpersonal, health, and functional concerns corresponded well with the balance of these items in KIDSCREEN. However, children had some specific priority areas that were not represented in KIDSCREEN. These included: relationships with family members other than parents; inclusion and fairness; home life and neighbourhood; pain and discomfort; environmental accommodation of needs; and recreational resources other than finances and time. We recommend that further consideration be given to inclusion of these areas in the assessment of HRQoL of disabled children.
Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele
Authors: Bruinings A L , Van Den Berg‐Emons H J G , Buffart L M , Van Der Heijden‐Maessen H C M , Roebroeck M E , Stam H J
Published: June 2009

The aim of this study was to assess the energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele (MMC) compared with peers without a disability. Eighteen participants with MMC aged between 16 and 30 years (13 males, five females; mean age 21y 4mo [SD 4y 8mo]) and 18 age‐ and sex‐matched non‐disabled participants performed several standardized activities. Energy cost was assessed by oxygen uptake expressed per unit time (all activities) and per metre (walking and wheelchair use at preferred speed). Physical strain was calculated by dividing energy cost by aerobic capacity. For all activities no difference was found in energy cost per unit time between ambulatory participants with MMC and comparison participants. Energy cost per metre walking at preferred speed in participants with MMC was 0.26ml/kg/m (SD 0.08), and in comparison participants was 0.20ml/kg/m (SD 0.03); p=0.08. Non‐ambulatory participants with MMC had lower energy cost (per unit time and per metre) during wheelchair use than comparison participants during walking (p<0.05). For most activities, physical strain was 1.4 to 2 times higher in participants with MMC than in comparison participants (p<0.05). In conclusion, energy cost per unit time of daily activities was not increased in participants with MMC. However, energy cost per metre during walking at preferred speed and physical strain were higher than in peers without disability.
Working memory, processing speed, and set‐shifting in children with developmental coordination disorder and attention‐deficit–hyperactivity disorder
Authors: Piek Jan P , Dyck Murray J , Francis Mona , Conwell Alistair
Published: June 2009

It has been suggested that the high levels of comorbidity between attention‐deficit‐hyperactivity disorder (ADHD) and developmental coordination disorder (DCD) may be attributed to a common underlying neurocognitive mechanism. This study assessed whether children with DCD and ADHD share deficits on tasks measuring working memory, set‐shifting, and processing speed. A total of 195 children aged between 6 years 6 months and 14 years 1 month (mean 10y 4mo [SD 2y 2mo]) were included in this study. A control group (59 males, 79 females), a DCD group (12 males, six females), an ADHD‐predominantly inattentive group (16 males, four females), and an ADHD‐combined group (15 males, four females), were tested on three executive functioning tasks. Children with DCD were significantly slower on all tasks, supporting past evidence of a timing deficit in these children. With few exceptions, children with ADHD did not perform more poorly than control children. These findings demonstrate the importance of identifying children with motor deficits when examining tasks involving a timing component.
Developmental outcome in five children with Hurler syndrome after stem cell transplantation: a pilot study
Authors: Lücke Thomas , Das Anibh M , Hartmann Hans , Sykora Karl‐Walter , Donnerstag Frank , Schmid‐Ott Gerhard , Grigull Lorenz
Published: June 2009

Hurler syndrome (mucopolysaccharidosis type 1H; MPS1H) is a lysosomal storage disease caused by a deficiency of α‐L‐iduronidase activity. The natural course of this neurodegenerative disease inevitably leads to premature death within the first 10 years of life. Enzyme replacement therapy is effective in correcting the enzymatic deficiency of organs other than the central nervous system. Hematopoietic stem cell transplantation (SCT) is the only treatment known to prevent psychomotor deterioration. However, the classical transplantation protocols resulted in a high incidence of graft failure and regimen‐related toxicity. Recently, we published a well‐tolerated, fludarabine‐based, radiation‐free conditioning regimen for SCT in patients with Hurler syndrome. Here we report the developmental outcome (assessed by the Denver Developmental Screening Test before and yearly after SCT) of four females and one male with MPS1H (mean age at last follow‐up 71mo, range 42‐87mo) treated in accordance with this strategy. Mean age at SCT was 25 months (range 10‐36mo). All children were engrafted and in ambulatory care. They all showed psychomotor development without neurodegeneration. In all patients, after SCT a regression of intracranial lesions could be seen that paralleled the psychomotor improvements. SCT led to a relative reduction of head circumference in all cases.
Secondary abnormalities of neurotransmitters in infants with neurological disorders
Authors: García‐Cazorla A , Serrano M , Pérez‐Dueñas B , González V , Ormazábal A , Pineda M , Fernández‐álvarez E , Campistol J M D , Artuch R M D
Published: June 2009

Neurotransmitters are essential in young children for differentiation and neuronal growth of the developing nervous system. We aimed to identify possible factors related to secondary neurotransmitter abnormalities in pediatric patients with neurological disorders. We analyzed cerebrospinal fluid (CSF) and biogenic amine metabolites in 56 infants (33 males, 23 females; mean age 5.8mo [SD 4.1mo] range 1d–1y) with neurological disorders whose aetiology was initially unknown. Patients were classified into three clinical phenotypes: epileptic encephalopathy, severe motor impairment, and non‐specific manifestations. All patients showed normal results for screening of inborn errors of metabolism. We report clinical, neuroimaging, and follow‐up data. Among the patients studied, 10 had low homovanillic acid (HVA) levels and in four patients, 5‐hydroxyindoleacetic acid (5‐HIAA) was also reduced. Patients with neonatal onset had significantly lower levels of HVA than a comparison group. HVA deficiency was also associated with severe motor impairment and the final diagnosis related to neurodegenerative disorders. 5‐HIAA values tended to be decreased in patients with brain cortical atrophy. The possibility of treating patients with L‐Dopa and 5‐hydroxytryptophan, in order to improve their neurological function and maturation, may be considered.
Functional gait comparison between children with myelomeningocele: shunt versus no shunt
Authors: Battibugli Simone , Gryfakis Nicholas , Dias Luciano , Kelp‐Lenane Claudia , Figlioli Stephanie , Fitzgerald Elizabeth , Hroma Nicole , Seshadri Roopa , Sullivan Christine
Published: June 2009

The aim of this study was to compare functional gait differences between patients with myelomeningocele (MM) who have a ventriculoperitoneal shunt (VPS) with those who do not. Our analyses were adjusted for confounding by age, lesion level, orthotic use, and assistive device use. The Functional Mobility Scale (FMS) was used to compare the shunted group (n=98; 60 males, 38 females; mean age 10y 2mo [SD 3y 11mo]; 73 sacral/19 low lumber/six high lumbar lesion level) with the non‐shunted group (n=63; 32 males, 31 females; mean age 9y 11mo [SD 3y 11mo]; 45 sacral/12 low lumber/six high lumbar lesion level). Participants with a shunt had lower FMS 500 and FMS 50 scores compared with participants without a shunt; hence the participants without a shunt were more independent in their ambulation at medium and longer distances. For a subset of participants who underwent a three‐dimensional gait analysis, we also collected temporal–spatial gait parameters (velocity, cadence, and stride length). Our results show that participants with MM and no shunt who underwent gait analysis(11 males, 10 females; mean age 9y 6mo [SD 4y]; 15 sacral/6 low lumber/0 high lumbar lesion level) tend to walk at a significantly greater velocity and stride length as compared with those with a shunt (33 males, 18 females; mean age 10y [SD 4y]; 38 sacral/13 low lumber/zero high lumbar lesion level). These data allow the treatment team to present more specific information regarding functional ambulatory expectations to patients with MM and their families.
A repeated course of constraint‐induced movement therapy results in further improvement
Authors: Charles Jeanne R , Gordon Andrew M
Published: June 2009

A recent therapeutic intervention, constraint‐induced movement therapy (CIMT), has been shown to improve movement efficiency and quality of movement in the involved hand of children with hemiplegic cerebral palsy (CP). In the present study, we investigate the long‐term effects of CIMT and the effect of a second course on involved limb function using an ABABA design. Eight children with mild to moderate hemiplegic CP (six males, two females; mean age 8y 7mo [SD 2y 6mo]; range 5–11y), who had received a CI therapy intervention 12 months before this study, participated in a second intervention. In both interventions, the children wore a sling on their non‐involved upper extremity for 6 hours per day during 10 out of 12 consecutive days and were engaged in play and functional activities that provided structured practice using the involved upper extremity. The results indicated initial improvements in movement efficiency, as measured by the Jebsen–Taylor Test of Hand Function, the Speed and Dexterity subtest (no. 8) of the Bruininks–Oseretsky Test of Motor Proficiency, and caregivers' perceptions of amount of use and quality of movement of the involved limb, were retained 12 months after the first intervention. The second intervention resulted in further improvement on these measures. Results indicate that intensive practice associated with CIMT may be retained long term, and that continued improvements may occur after a second intervention dose.
West syndrome in an infant with vitamin B12deficiency in the absence of macrocytic anaemia
Authors: Erol Ilknur , Alehan Füsun , Gümüs Ayten
Published: June 2009

Vitamin B12deficiency in infants often produces haematological and neurological deficits, including macrocytic anaemia, neurodevelopmental delay or regression, irritability, weakness, hypotonia, ataxia, apathy, tremor, and seizures. The diagnosis of vitamin B12deficiency can be difficult when the typical macrocytic anaemia is absent. We report the case of a 10‐month‐old female diagnosed with West syndrome associated with vitamin B12deficiency but without macrocytic anaemia caused by nutritional inadequacy in the mother. The patient's motor skills and cognitive development were normal until she was 9 months old, when she began to exhibit a series of sudden flexions of the head, trunk, arms, and legs. She was exclusively breast‐fed and had received no vitamin supplementation. Results of electroencephalography (EEG) indicated modified hypsarrhythmia and the patient was diagnosed as having West syndrome. Synthetic adrenocorticotropic hormone was administered and although her spasms had resolved, the patient remained apathic and could not sit without assistance. EEG results indicated generalized slow activity. After she was diagnosed as having vitamin B12deficiency, parenteral treatment with vitamin B12was initiated. Her symptoms resolved and EEG was completely normal. When she was 20 months old she exhibited an age‐appropriate developmental and neurological profile. To our knowledge, this is the first report of West syndrome as a presenting symptom of vitamin B12deficiency.
Distressed neighborhoods and child disability rates: analyses of 157 000 school‐age children
Authors: Msall Michael E , Avery Roger C , Msall Emily R , Hogan Dennis P
Published: June 2009

The aim of this study was to assess rates of childhood disability as indicated by functional limitation of motor, sensory, or self‐care skills in children living in severely‐distressed neighborhoods. For a neighborhood in the US Census Track to be considered severely distressed, three of the following four characteristics need to be present: >27% of children live in poverty, >23% high school drop‐out rate, >34% male unemployment rate, and >37% of households headed by females alone. In the 2000 US Census, 157 000 children between ages 5 and 15 years resided in the State of Rhode Island. Severely‐distressed neighborhoods were found in 12.6% of Rhode Island Census Tracks. These areas accounted for 14.5% of the school‐age population, 25% of children with motor disabilities, 29% of children with self‐care disabilities, and 14% of children with sensory disabilities. For each increasing level of neighborhood distress, rates of child disability increased. Child disability rates in moderately distressed neighborhoods were 3.7%, compared with 1.1% in advantaged neighborhoods. Children in distressed neighborhoods had disproportionately high rates of disability, especially in motor and self‐care functioning. Comprehensive interventions aimed at children in distressed neighborhoods are crucial to reduce health disparities for vulnerable children.