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

The purpose of this study was to identify group differences in children with attention-deficit–hyperactivity disorder and motor dysfunction (ADHD-MD) and ADHD only, and to evaluate the medication responsiveness of ADHD-MD. Sixty-three children (49 males and 14 females; mean age 9 years 10 months, SD 2 years 10 months) underwent a triple blind, placebo-controlled crossover study evaluating two dose levels of methylphenidate (0.3 mg/kg and 0.05 mg/kg, twice daily) and placebo. Forty-nine trials were completed. Nineteen were children with ADHD-MD, 44 had ADHD only. Behavior and functioning were assessed at home and at school. Treatment effects were assessed using the Abbreviated Symptom Questionnaire for Parents and Teachers. Children with ADHD-MD were more likely to have severe ADHD-combined type and other neurodevelopmental and behavioral problems. Both groups of children had a linear dose response to medication (placebo, low, high) and there was no evidence of a group by dose interaction or an overall group effect at home or school. The lack of group effect suggests that these children responded to medication like the other subgroups.

Long-term continuous intrathecal baclofen (CITB) infusion is a treatment option used to manage otherwise intractable spasticity and is delivered via an implantable pump. The purpose of this single-center multidisciplinary review was to report on the long-term safety and efficacy of CITB in the treatment of 21 children with intractable severe spasticity of cerebral origin. Nineteen recipients had spastic quadriplegia and two had spastic diplegia. Seven recipients had level IV severity on the Gross Motor Functional Classification System and 14 had level V. Median age at implantation was 12 years (range 4 to 20). Fifteen recipients were male, 6 were female. Seventeen recipients were alive at the end of the follow-up period (31 to 78 months; mean 53, SD 4). The Ashworth scale showed a substantial decrease in spasticity in the upper and lower extremities at 6 months and at the most recent follow-up. The Gross Motor Function Measure and Pediatric Evaluation of Disability Inventory showed no functional change. Most treatment goals were at least partly achieved. Caregivers reported a reduction in use of oral medication for spasticity, and improvements in comfort, function, and ease of care. Caregiver satisfaction was high. During 80 recipient-years of pump operation, 153 treatment-associated adverse events occurred: 27 of these were device-related. There were four deaths unrelated to CITB, including one from acute pancreatitis. Our findings might assist in establishing patient selection criteria and treatment goals, improving patient follow-up, and monitoring adverse events.

Eighteen children (13 males, five females) who had severe developmental language delay/disorder and some features of autism (although insufficient in severity and combination to meet ICD-10 diagnostic criteria for childhood autism) at preschool age (Time 1; mean age 4 years 4 months) were followed up 4 years later (Time 2; mean age 8 years 7 months). At the initial assessment the diagnostic dilemma was how much the social communication impairments and behavioural problems were secondary to the language problem and how much they constituted a genuine case of a pervasive developmental disorder. It was anticipated that at follow-up some children would continue to show social impairments but that in others social impairments would have receded as language competence improved. Follow-up assessments included the Wechsler Intelligence Scale for Children, the Clinical Evaluation of Language Fundamentals, the Children's Communication Checklist, and the Social Communication Questionnaire. At follow-up, five children had continuing language disorder and were considered to fulfil diagnostic criteria for childhood autism, four children had continuing language disorder and met criteria for atypical autism, and nine met criteria for atypical autism but had somewhat recovered language skills. Thus, even in the subgroup of children whose language ability had improved the features of autism had not dissipated. Severity of social communication impairments and repetitive behaviours at Time 1, rated retrospectively from case notes, were associated with severity of autism symptoms and pragmatic competence at Time 2. The findings are discussed in relation to the unclear boundary between autism spectrum disorders and language delay/disorder.

This study determined whether an auditory, tactile, visual, and vestibular intervention (ATVV) reduced the length of hospitalization of 37 preterm infants by increasing the proportion of alert behavioral states, thereby improving their feeding progression. Participants comprised 12 infants born between 23 and 26 weeks' gestation with normal head ultrasounds and 25 CNS-injured infants born between 23 and 31 weeks' gestation. Infants were randomly assigned to the control group (11 males, five females) or study group (seven males, 14 females) at 32 weeks' postconceptional age. ATVV intervention was administered to the study group for 15 minutes, twice daily, 5 days per week, from 33 weeks of age until discharge. The study group demonstrated increased alertness during the first 5 minutes of intervention, which was significantly correlated to length of stay (p<0.05). The proportion of nippled (teat) intake increased significantly faster for the study group (p=0.0001). Infants in the study group were discharged at a mean of 36.54 weeks, 1.6 weeks earlier than control infants (p< 0.05). ATVV intervention facilitated increased alertness, faster transition to complete nipple feeding, and decreased length of hospitalization.

Forty-six of 93 children with neurofibromatosis type 1(NF1) were found to satisfy the diagnostic criteria for attention-deficit–hyperactivity disorder (ADHD). Detailed comparisons were made among 20 children with NF1 and ADHD (12 males, 8 females; mean age 10.7 years, SD 2.2), 26 control children with NF1 (15 males, 11 females; mean age 11.3 years, SD 2.3), 14 control children with ADHD (7 males; mean age 9.9 years, SD 1.9), and 14 normally developing control children (7 males; mean age 11.2 years, SD 2.8). Children with NF1 and ADHD had the lowest IQ scores among the four groups. Test of Variables of Attention (TOVA) scores were poorer in the NF1–ADHD and ADHD control groups than in the two non-ADHD groups. Those with NF1 and ADHD were rated significantly poorer on the Child Behavior Checklist (CBCL) than were the NF1 control group. By administrating low doses (5 to 15 mg) of methylphenidate to the NF1–ADHD group, significantly improved TOVA scores were obtained. One-year follow-up yielded significantly improved CBCL scores. Our results show a high incidence of ADHD in NF1 and support an association between ADHD and learning and social problems in children with NF1. It was demonstrated that stimulant medication can lead to improvement in cognitive, academic, and social problems of children with NF1 and ADHD.

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

The aim of the study was to examine whether school performance was reduced in children with very-low birthweight beyond what could be explained by the deficit in intelligence-score at 4 years of age. Participants (n=333) examined at 4 years of age included three subgroups on the basis of birthweight: very-low birthweight (VLBW <1501g; n=102), low birthweight (LBW 1500–2300g; n=139), and normal birthweight (NBW [ges ]2500g; n=92). The general cognitive index index (CGI) from the McCarthy Scales of Children's Abilities was used as an intelligence measurement at this assessment. At 18 to 20 years of age a telephone interview concerning school performance was conducted. Two hundred and fifty-seven participants (53% male, 47% female) entered the analysis. A model with School Performance score after the last year of compulsory school as the outcome variable, and McCarthy GCI and birthweight group as predicting variables, was used in linear regression analyses. Twenty-four per cent of the variance was explained. Birthweight had no influence in this model. In conclusion, the study did not find that birthweight influenced school performance beyond that represented by the McCarthy GCI found at age 4 years.

The study aimed to evaluate the effect of low-intensity night-time therapeutic electrical stimulation (TES) on arm strength and function in children with intermediate type spinal muscular atrophy (SMA). The design was a randomized controlled trial with a 6-month baseline control period. Children were evaluated at baseline, 6, and 12 months. TES was applied from 6 to 12 months to the deltoid and biceps muscle, of a randomly selected arm with the opposite arm receiving a placebo stimulator. Thirteen individuals with SMA between 5 to 19 years of age were recruited into the study and eight completed the 12-month assessment. No statistically significant differences between the treatment and control arm were found at baseline, 6, and 12 months for elbow flexors, or shoulder abductors on quantitative myometry or manual muscle testing. There was no significant change in excitable muscle mass assessed by M-wave amplitudes, nor function on the Pediatric Evaluation of Disability Inventory (self-care domain). Therefore, in this study there was no evidence that TES improved strength in children with SMA.

In this study parents' systematic accounts of the health status of 408 school-aged children with cerebral palsy (CP) are reported (221 males, 187 females; mean age 8 years 5 months, SD 1 year 11 months; range 5 to 13 years), as are relations between severity of functional motor impairment and eight functional health status domains. Data were collected as part of a longitudinal study of the motor development of a population-based, stratified, random sample of children with CP from across Ontario, Canada. The Gross Motor Function Classification System (GMFCS) was used to classify severity of CP and functional health status was described with the eight-level Health Utilities Index – Mark 3. Rates of functional limitations in Mobility, Dexterity, Speech, and Vision were statistically significantly associated with GMFCS levels (all p<0.01), with correlation values (tau-b) of 0.82, 0.58, 0.46, and 0.36, respectively. Functional limitations in hearing (tau-b=0.16; p=0.04) and cognition (tau-b=0.27; p<0.01) were both statistically significantly associated with GMFCS levels, though correlations were low. Neither emotion (tau-b=0.03; p=0.24) nor pain (tau-b=0.07; p=0.37) was associated with degree of functional limitation as described by the GMFCS. Clinical and epidemiological implications of findings are discussed.

We evaluated gross motor function following botulinum toxin A (BTX-A) injections in the lower limbs of children with spastic cerebral palsy in a randomized clinical trial, using a cross-over design. Forty-nine children (24 males, 25 females, age range 22 to 80 months) were randomly allocated to two groups: group 1 received BTX-A and physiotherapy, and group 2 received physiotherapy alone for 6 months. At the end of this period, group 2 received BTX-A and physiotherapy and group 1 continued with physiotherapy alone. Assessment measures were the Gross Motor Function Measure (GMFM), the Vulpe Assessment Battery (VAB), joint range of movement, the Modified Ashworth Scale, and a parental questionnaire. Sustained gains in gross motor function were found in both groups of children but the only additional benefit found in group 1 was a significant increase in fine motor rating on the VAB. By contrast, parents rated the benefit of treatment highly. It is likely that assessment at 3 and 6 months post injection was too late to demonstrate peak gross motor function response and that changes in GMFM are not sustained over 6 months with a single dose. Further studies should investigate changes over shorter time periods and consider covariables such as BTX-A dosage, number of injection sites, and the role of repeated injections combined with other interventions such as casting.

This study evaluated the efficacy and safety of three doses of botulinum toxin A (BTX-A; Dysport®) in 125 patients (mean age 5.2 years, SD 2; 54% male)with dynamic equinus spasticity during walking. Participants were randomized to receive Dysport (10, 20, or 30 units/kg) or placebo to the gastrocnemius muscle of both legs. Muscle length was calculated from electrogoniometric measurements and the change in the dynamic component of gastrocnemius shortening at four weeks was prospectively identified as the primary outcome measure. All treatment groups showed statistically significant decreases in dynamic component compared with placebo at 4 weeks. Mean improvement in dynamic component was most pronounced in the 20 units/kg group, being equivalent to an increase in dorsiflexion with the knee extended at 19°, and was still present at 16 weeks. The safety profile of the toxin appears satisfactory.

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

To quantify parameters of rhythmic suckle feeding in healthy term infants and to assess developmental changes during the first month of life, we recorded pharyngeal and nipple pressure in 16 infants at 1 to 4 days of age and again at 1 month. Over the first month of life in term infants, sucks and swallows become more rapid and increasingly organized into runs. Suck rate increased from 55/minute in the immediate postnatal period to 70/minute by the end of the first month (p<0.001). The percentage of sucks in runs of [ges ]3 increased from 72.7% (SD 12.8) to 87.9% (SD 9.1; p=0.001). Average length of suck runs also increased over the first month. Swallow rate increased slightly by the end of the first month, from about 46 to 50/minute (p=0.019), as did percentage of swallows in runs (76.8%, SD 14.9 versus 54.6%, SD 19.2; p=0.002). Efficiency of feeding, as measured by volume of nutrient per suck (0.17, SD 0.08 versus 0.30, SD 0.11cc/suck; p=0.008) and per swallow (0.23, SD 0.11 versus 0.44, SD 0.19 cc/swallow; p=0.002), almost doubled over the first month. The rhythmic stability of swallow–swallow, suck–suck, and suck–swallow dyadic interval, quantified using the coefficient of variation of the interval, was similar at the two age points, indicating that rhythmic stability of suck and swallow, individually and interactively, appears to be established by term. Percentage of sucks and swallows in 1:1 ratios (dyads), decreased from 78.8% (SD 20.1) shortly after birth to 57.5% (SD 25.8) at 1 month of age (p=0.002), demonstrating that the predominant 1:1 ratio of suck to swallow is more variable at 1 month, with the addition of ratios of 2:1, 3:1, and so on, and suggesting that infants gain the ability to adjust feeding patterns to improve efficiency. Knowledge of normal development in term infants provides a gold standard against which rhythmic patterns in preterm and other high-risk infants can be measured, and may allow earlier identification of infants at risk of neurodevelopmental delay and feeding disorders.

This study sought to examine the reliability and validity of three generic instruments for measuring the health of children with cerebral palsy (CP) and to compare them with a disease-specific measure, the Gross Motor Function Measure (GMFM). The Pediatric Evaluation and Disability Inventory (PEDI), the Pediatric Outcomes Data Collection Instrument (PODCI), and the Child Health Questionnaire (CHQ) were completed by the primary caregivers of 115 young children with spastic CP. The GMFM was administered to the children. The mean age of the sample was 5 years 8 months (range 3:1 to 10:4) and consisted of more males (58%) than females. The PEDI scales demonstrated higher internal consistency than the PODCI and CHQ scales. In comparison with the GMFM, the PODCI transfer and mobility scale (relative validity, 62%) and the PEDI mobility scale (relative validity, 53%) detected the most significant health differences between children with hemiplegia, diplegia, and quadriplegia. The PEDI social function scale detected the largest differences in cognitive function between children with an IQ of less than 70 compared with those with an IQ of 70 or greater. The reliability and validity of these different instruments varied significantly in this patient population.

This study examined 17 children (nine males, eight females; mean age 13 years) with prenatal alcohol exposure of various durations. The aim of the study was to detect specific brain morphological alterations by means of MRI and to see if findings correlated with particular cognitive deficits. Of the 17 children, five had been exposed to heavy maternal consumption of alcohol (over 10 drinks/week) during the first trimester only; four had been exposed during the first and second trimester; and eight had been exposed throughout pregnancy. Five children had alcohol related neurobehavioural disorder, seven were diagnosed as having foetal alcohol effects and five were diagnosed as having foetal alcohol syndrome. Hypoplasia of the vermis was observed in 10 children and malformed posterior vermis in one additional child. Five children had hypoplastic cerebellar hemispheres. Hypoplasia of the corpus callosum was observed in two children. Small hippocampi were observed in three children and wide cortical sulci in six. No specific structural anomaly correlated with a particular neuropsychological deficit. In this study, deviations in the development of the vermis was the most sensitive morphological indicator of the effects of prenatal alcohol exposure. It was seen in every diagnostic group including children who had been exposed during only the first trimester of pregnancy.

In a representative series of 176 children with cerebral palsy (CP), aged 5 to 8 years, associations were studied between additional neuroimpairments, activity limitations, and participation restrictions in the domains of mobility, education, and social relations as proposed in the International Classification of Functioning Disability and Health (ICF). Learning disability occurred in 40%, epilepsy in 35%, visual impairment in 20%, and infantile hydrocephalus in 9% of the children. Additional neuroimpairments were most frequently seen in children with tetraplegia and dystonic CP and in those with antecedents of brain malformations or severe perinatal compromise. Activity limitations were studied with the Gross Motor Function Classification System (GMFCS) and a system for grading bimanual fine motor function (BFMF) was developed. There was a strong correlation of 0.74 between the GMFCS and BFMF (p<0.001). Learning disability, activity limitations, and participation restrictions were all clinically strongly associated with each other (p<0.001). Restriction in mobility was best predicted by the GMFCS, learning disability, and the BFMF; in education by learning disability and the GFMCS; and in social relations by learning disability, the GMFCS, and BFMF. Motor function and learning disability were important predictors for participation restrictions in children with CP. The ICF has the capacity to be a model to help plan interventions for specific functional goals and to ascertain the child's participation in society.

The purpose of the present study was to determine the long-term outcome of 76 children (40 females and 36 males) diagnosed and treated with modern antituberculosis drugs. The median age of the children on admission was 29.5 months and on follow-up 9 years. Antituberculosis therapy consisted of daily isoniazid (20mg/kg), rifampicin (20mg/kg), ethionamide (20mg/kg), and pyrazinamide (40mg/kg) for 6 months. Twenty-three children received daily prednisone (2–4mg/kg) for the first month of treatment. Raised intracranial pressure was actively monitored and treated. Patients with non-communicating hydrocephalus received ventriculo-peritoneal shunts shortly after admission while communicating hydrocephalus was treated with oral acetazolamide (100mg/kg/day) and furosemide (1mg/kg/day) in 3–4 divided doses. Communicating hydrocephalus that did not respond to this regimen within the first month of treatment also underwent ventriculo-peritoneal shunting. Only 20% of children were functionally completely normal at follow-up. Main areas of functional deficit were cognitive impairment (80%), poor scholastic progress (43%), and emotional disturbance (40%). Twenty-five per cent of children had evidence of motor impairment, but all could walk and only 5 of 76 children (6% of total) were unable to run. One child was blind but no child had sensori-neural deafness. It was concluded that these disabilities in children from mainly deprived socioeconomic backgrounds have serious implications for their future social, academic, and career prospects. A high index of suspicion of TBM in high tuberculosis incidence communities will help prevent the morbidity documented in this study.

The aim of this study was to examine the correlation between EEG, visual, and brain MRI findings in 19 term infants with perinatal brain lesions. All 19 had their visual acuity and visual fields assessed and had an EEG and a brain MRI performed at 1 year of age. Four of the five infants with normal optic radiations and occipital cortices on MRI had normal vision. Involvement of optic radiations and occipital cortices was only associated with visual abnormalities in eight of 14 infants. The correlation between visual abnormalities and EEG findings was stronger. All infants with a completely normal EEG from the posterior regions had normal vision and all those with an EEG non-reactive to eye closure had visual abnormalities, irrespective of MRI data. A reactive EEG with other abnormal features (such as spikes, rapid or slow activities) was accompanied by abnormal vision in five of eight participants. Results suggest that there is a better correlation between visual function and EEG activity than between visual function and involvement of the classical visual areas of the occipital cortex and optic radiations on brain MRI at 1 year of age.

Children with hemiplegic cerebral palsy (CP) commonly exhibit tactile sensory deficiencies in their hands in addition to their motor problems. The aim of this study was to compare and evaluate the usefulness of some common tests of tactile sensibility for use with children with hemiplegic CP. Twenty-five children with hemiplegia aged between 5 and 18 years, and 19 control individuals participated. All children were examined with Semmes–Weinstein monofilaments, two-point discrimination (2PD), stereognosis of familiar objects, stereognosis of forms, and functional sensibility. Dexterity, spasticity, and bimanual task performance were also assessed. Results from the different sensory tests deviated greatly. We found three tests to be useful: 2PD of 3mm spacing, which was the most sensitive test, stereognosis of familiar objects, and functional sensibility assessed through the Pick-up test (comparing performance with and without the influence of vision). Stereognosis of forms and threshold values of touch (Semmes–Weinstein monofilaments) are seemingly less useful tests for children with CNS impairments. Deficient sensibility was strongly related to dexterity. Aspects concerning the testing methodology are discussed.