Showing papers in "Developmental Medicine & Child Neurology in 2002"
TL;DR: The results are consistent with earlier reports and describe in greater detail the regional pattern of age-related differences in gray and white matter in normally developing children and adolescents.
Abstract: The purpose of the present study was to describe in greater anatomical detail the changes in brain structure that occur during maturation between childhood and adolescence. High-resolution MRI, tissue classification, and anatomical segmentation of cortical and subcortical regions were used in a sample of 35 normally developing children and adolescents between 7 and 16 years of age (mean age 11 years; 20 males, 15 females). Each cortical and subcortical measure was examined for age and sex effects on raw volumes and on the measures as proportions of total supratentorial cranial volume. Results indicate age-related increases in total supratentorial cranial volume and raw and proportional increases in total cerebral white matter. Gray-matter volume reductions were only observed once variance in total brain size was proportionally controlled. The change in total cerebral white-matter proportion was significantly greater than the change in total cerebral gray-matter proportion over this age range, suggesting that the relative gray-matter reduction is probably due to significant increases in white matter. Total raw cerebral CSF volume increases were also observed. Within the cerebrum, regional patterns varied depending on the tissue (or CSF) assessed. Only frontal and parietal cortices showed changes in gray matter, white matter, and CSF measures. Once the approximately 7% larger brain volume in males was controlled, only mesial temporal cortex, caudate, thalamus, and basomesial diencephalic structures showed sex effects with the females having greater relative volumes in these regions than the males. Overall, these results are consistent with earlier reports and describe in greater detail the regional pattern of age-related differences in gray and white matter in normally developing children and adolescents.
682 citations
TL;DR: Motor function and learning disability were important predictors for participation restrictions in children with CP and has the capacity to be a model to help plan interventions for specific functional goals and to ascertain the child's participation in society.
Abstract: 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.
589 citations
TL;DR: A central database was set up to include information on over 6000 children with cerebral palsy from 13 geographically defined populations in Europe, providing a powerful means of monitoring trends in birth weight‐specific rates of CP and an infrastructure for research and service planning.
Abstract: Following agreement on definitions and classification, a central database was set up to include information on over 6000 children with cerebral palsy (CP) from 13 geographically defined populations in Europe. The overall rate for the period 1980 to 1990 was 2.08/1000 live births (95% CI 2.02 to 2.14). One in five children with CP (20.2%) was found to have a severe intellectual deficit and was unable to walk. Among babies born weighing less than 1500 g, the rate of CP was more than 70 times higher compared with those weighing 2500 g or more at birth. The rate of CP rose during the 1970s, but remained constant during the late 1980s. Future analyses will include data from children born in the 1990s. This collaborative work provides a powerful means of monitoring trends in birthweight-specific rates of CP and an infrastructure for research and service planning.
586 citations
TL;DR: In this study the prevalence of specific medical disorders that might account for a shortened life expectancy were investigated and it is postulated that hypotonia is a possible contributory factor to the risk of strabismus, scoliosis, and respiratory infections.
Abstract: The medical findings from a population-based study of Prader-Willi syndrome (PWS) are discussed (in which birth incidence of PWS was estimated at 1:22,000 and death rate at over 3% per annum). In this study the prevalence of specific medical disorders that might account for a shortened life expectancy were investigated. Of all people with a possible diagnosis of PWS, only those meeting clinical criteria and/or with a confirmed genetic diagnosis were included in the study. Sixty-six individuals, 40 males and 26 females with a mean age of 19 years (range of 0 to 46 years) agreed to participate in the population-based study group. A prevalence rate of 25% for non-insulin dependent diabetes mellitus (NIDDM) was found in adults. Mean age at onset was 20 years. Those with NIDDM had a higher past maximum body weight and a greater likelihood of positive family history. Nearly 50% across the age groups reported a history of recurrent respiratory infections. High rates of fractures (29%), leg ulceration (22% in adults), sleep disorders (20%), and severe scoliosis (15% in childhood) were also reported. It is postulated that hypotonia is a possible contributory factor to the risk of strabismus, scoliosis, and respiratory infections. Other causes of morbidity, in particular the high rates of NIDDM, may be due to a failure to manage over-eating resulting in severe obesity. Early diagnosis and clear guidance to families about these risks and how they might be prevented is recommended. It is hypothesized that the high pain threshold may result in the presence of some illness not being apparent.
286 citations
TL;DR: SDR+PT is efficacious in reducing spasticity in children with spastic diplegia and has a small positive effect on gross motor function.
Abstract: This study is a comparative analysis and meta-analysis of three randomized clinical trials. Children with spastic diplegia received either 'selective' dorsal rhizotomy (SDR) plus physiotherapy (SDR+PT) or PT without SDR (PT-only). Common outcome measures were used for spasticity (Ashworth scale) and function (Gross Motor Function Measure [GMFM]). Baseline and 9- to 12-month outcome data were pooled (n=90). At baseline, 82 children were under 8 years old and 65 had Gross Motor Function Classification System level II or III disability. Pooled Ashworth data analysis confirmed a reduction of spasticity with SDR+PT (mean change score difference -1.2; Wilcoxonp<0.001). Pooled GMFM data revealed greater functional improvement with SDR+PT (difference in change score +4.0, p=0.008). Multivariate analysis in the SDR+PT group revealed a direct relationship between percentage of dorsal root tissue transected and functional improvement. SDR+PT is efficacious in reducing spasticity in children with spastic diplegia and has a small positive effect on gross motor function.
258 citations
TL;DR: Deficiency of FMRP (fragile X mental retardation protein) appears to lead to increased neuronal excitability and susceptibility to epilepsy, but particularly seems to facilitate mechanisms leading to the BFEC pattern.
Abstract: Epilepsy is reported to occur in 10 to 20% of individuals with fragile X syndrome (FXS). A frequent seizure/EEG pattern in FXS appears to resemble that of benign focal epilepsy of childhood (BFEC, benign rolandic epilepsy). To evaluate seizure frequency and type in a Chicago FXS cohort, data regarding potential seizure history were reviewed for 136 individuals with FXS (age range 2 to 51 years: 113 males and 23 females). Seizures occurred in 15 males (13.3%) and one female (4.8%): of these, 12 had partial seizures. EEG findings were available for 35 individuals (13 of 16 with seizures and 22 of 120 without seizures) and showed an epileptiform abnormality in 10 (77%) individuals with seizures and five (23%) individuals without seizures--the most common epileptiform pattern being centrotemporal spikes. Seizures were easily controlled in 14 of the 16 individuals with seizures. Many individuals, including all with centrotemporal spikes, had remission of seizures in childhood. The most common seizure syndrome resembled BFEC and this pattern had the best prognosis for epilepsy remission. Deficiency of FMRP (fragile X mental retardation protein) appears to lead to increased neuronal excitability and susceptibility to epilepsy, but particularly seems to facilitate mechanisms leading to the BFEC pattern.
240 citations
TL;DR: It was demonstrated that stimulant medication can lead to improvement in cognitive, academic, and social problems of children with NF1 and ADHD and low doses of methylphenidate to the NF1-ADHD group obtained significantly improved TOVA scores.
Abstract: 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.
223 citations
TL;DR: Investigation of standing balance in ambulatory children and adolescents with spastic diplegic CP found that most participants with CP had normal dependence on visual feedback to maintain balance, indicating that identification of those children with impaired standing balance can delineate factors that contribute to the patient's gait disorder and help to guide treatment.
Abstract: Postural control deficits have been suggested to be a major component of gait disorders in cerebral palsy (CP). Standing balance was investigated in 23 ambulatory children and adolescents with spastic diplegic CP, ages 5 to 18 years, and compared with values of 92 children without disability, ages 5 to 18 years, while they stood on a force plate with eyes open or eyes closed. The measurements included center of pressure calculations of path length per second, average radial displacement, mean frequency of sway, and Brownian random motion measures of the short-term diffusion coefficient, and the long-term scaling exponent. In the majority of children with CP (14 of 23) all standing balance values were normal. However, approximately one-third of the children with CP (eight of 23) had abnormal values in at least two of the six center of pressure measures. Thus, mean values for path length, average radial displacement, and diffusion coefficient were higher for participants with CP compared with control individuals with eyes open and closed (p<0.05). Mean values for frequency of sway and the long-term scaling exponent were lower for participants with CP compared with control participants (p<0.05). Increased average radial displacement was the most common (nine of 23) postural control deficit. There was no increase in abnormal values with eyes closed compared with eyes open for participants with CP, indicating that most participants with CP had normal dependence on visual feedback to maintain balance. Identification of those children with impaired standing balance can delineate factors that contribute to the patient's gait disorder and help to guide treatment.
217 citations
TL;DR: The authors sought to determine which components of passive resistance (magnitude, rate of change, onset angle of stretch, or velocity dependence) were most related to Ashworth scores and which were related to motor function in cerebral palsy (CP).
Abstract: This study aimed to explore the limitations of the Ashworth scale for measuring spasticity. An isokinetic dynamometer to quantify resistance to passive stretch and surface EMG was used to verify if a stretch response occurred and, if so, at what joint angle. The authors sought to determine which components of passive resistance (magnitude, rate of change, onset angle of stretch, or velocity dependence) were most related to Ashworth scores and which were related to motor function in cerebral palsy (CP). Twenty-two individuals with spastic CP (11 males, 11 females; mean age 11.9 years, SD 4.3) and a comparison group of nine children without CP (four males, five females; mean age 11.3 years, SD 2.5) participated in the study. The group with CP included those with a diagnosis of spastic diplegia, hemiplegia, or quadriplegia, distributed across Gross Motor Functional Classification Levels. Procedures included: (1) clinical assessment at the knee joint, (2) functional assessments, and (3) isokinetic assessment of passive resistance torque in hamstrings and quadriceps at three velocities. EMG data were recorded simultaneously to identify stretch responses. Detecting stretch responses using the Ashworth scale compared with instrumented measures showed near complete agreement at extremes of the scale, with marked inconsistencies in mid-range values. Ashworth scores were correlated with instrumented measures, particularly for the quadriceps, with higher correlations to the rate of change in resistance (stiffness) and onset angle of stretch than to peak resistance torque. Those with greater resistance tended to have poorer function with isokinetic relations typically stronger.
212 citations
TL;DR: The effects of recreational horseback riding therapy (HBRT) on gross motor function in children with cerebral palsy were determined in a blinded study using the Gross Motor Function Measure (GMFM).
Abstract: 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.
201 citations
TL;DR: The term ‘developmental coordination disorder’ (DCD) in general refers to children with normal intelligence who have poor motor coordination without clear evidence of a neurological pathology, such as cerebral palsy (CP) or muscular dystrophy.
Abstract: In the past 40 years, children with minor developmental motor disorders have been studied by professionals from various fields: paediatricians, neurologists, psychiatrists, psychologists, and physical and occupational therapists. The differences in background of these professionals led to a plethora of terms used to refer to these conditions: e.g. minimal cerebral palsy, minimal cerebral dysfunction, developmental apraxia or dyspraxia, minimal brain dysfunction, sensory integrative dysfunction, and developmental coordination disorder. It was generally acknowledged that such a multitude of names was confusing and so, in 1994, an international consensus meeting of professionals from various fields agreed to use the term ‘developmental coordination disorder’ (DCD). DCD in general refers to children with normal intelligence who have poor motor coordination without clear evidence of a neurological pathology, such as cerebral palsy (CP) or muscular dystrophy. In other words, DCD is a ‘final common output’ term, without aetiological or pathophysiological foundation. The advantage of such an umbrella term is that it includes all children with motor problems that interfere with their daily life, i.e. children who deserve clinical attention. However, the disadvantage of the term DCD is its aspecificity. Indeed, it is becoming increasingly clear that children with DCD constitute a heterogeneous population with various types of motor dysfunction.
TL;DR: The reliability and validity of three generic instruments for measuring the health of children with cerebral palsy and to compare them with a disease-specific measure, the Gross Motor Function Measure (GMFM), varied significantly in this patient population.
Abstract: 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.
TL;DR: In this paper, the authors examined 17 children (nine males, eight females; mean age 13 years) with prenatal alcohol exposure of various durations and detected specific brain morphological alterations by means of MRI and to see if findings correlated with particular cognitive deficits.
Abstract: 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.
TL;DR: In a substantial proportion of males, the occurrence of a fracture had a significant impact on subsequent mobility, and twenty percent of ambulant males and 27% of those using orthoses lost mobility permanently as a result of the fracture.
Abstract: The objective of this study was to determine the prevalence, circumstances, and outcome of fractures in males with Duchenne muscular dystrophy (DMD) attending neuromuscular clinics. Three hundred and seventy-eight males (median age 12 years, range 1 to 25 years) attending four neuromuscular centres were studied by case-note review supplemented by GP letter or by interview at the time of clinic attendance. Seventy-nine (20.9%) of these patients had experienced fractures. Forty-one percent of fractures were in patients aged 8 to 11 years and 48% in independently ambulant patients. Falling was the most common mechanism of fracture. Upper-limb fractures were most common in males using knee-ankle-foot orthoses (65%) while lower-limb fractures predominated in independently mobile and wheelchair dependent males (54% and 68% respectively). Twenty percent of ambulant males and 27% of those using orthoses lost mobility permanently as a result of the fracture. In a substantial proportion of males, the occurrence of a fracture had a significant impact on subsequent mobility.
TL;DR: Parents’systematic accounts of the health status of 408 school-aged children with cerebral palsy are reported, as are relations between severity of functional motor impairment and eight functional health status domains, and degree of functional limitation as described by the Gross Motor Function Classification System.
Abstract: 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.
TL;DR: The purpose of this annotation is to summarize existing research on strength testing and training, primarily focusing on CP, and address the following clinical questions: Can strength be measured reliably and in a valid way in cerebral palsy?
Abstract: Cerebral palsy (CP) is a collection of disorders characterized by an insult to the developing brain that produces a physical disability as the primary or distinguishing feature. The spastic form of CP is most common and in those patients, additional clinical signs may include muscle shortening, diminished selective control, and weakness. The recognition of weakness as a component of CP has been longstanding as evidenced by the names given to this disorder and its subtypes. ‘Cerebral palsy’ means weakness originating from the brain, and the use of the suffixes ‘plegia’ or ‘paresis’ also indicate that weakness is a prominent feature. More than 50 years ago, Phelps contended that resisted exercise ‘to develop strength or skill in a weakened muscle or an impaired muscle group’ was an integral part of treatment in CP. (p 59) Since that time, physical educators have also advocated strengthening. Yet for years, conventional clinical wisdom in physical therapy argued against the use of strength testing and training in children with CP and, indeed, in all persons with CNS disorders. The rationale for this exclusion appears to be multifaceted. First, therapists were discouraged by the relatively meager functional responses to strength training in patients with spasticity compared with those with polio. Clinicians also feared that strong near maximal effort would exacerbate spasticity and muscle tightness in those who were already ‘stiffer’ than normal. Many also attested that impaired selective control in CP essentially prohibited performance of strengthening activities. Consequently, this approach was discarded in favor of a more direct focus on the brain. Only recently has strength testing and training experienced a resurgence in habilitation and rehabilitation programs for this population and for other spastic motor disorders. However, hesitation and even resistance to their incorporation are still encountered despite the lack of evidence to suggest that strengthening is detrimental in the presence of spasticity and accumulating evidence to support this type of exercise. The purpose of this annotation is to summarize existing research on strength testing and training, primarily focusing on CP and address the following clinical questions: (1) Can strength be measured reliably and in a valid way in cerebral palsy? (2) Is weakness a significant impairment in CP? (3) Is strength training effective in increasing force production and improving motor function and disability in CP? (4) Is strength training safe in the presence of spasticity and for children and adolescents, regardless of health status, who have an immature musculoskeletal system?
TL;DR: Gains were made in motor function and self care following a course of Bobath therapy in children with cerebral palsy, and a repeated measures design demonstrates that in this population, gains were made.
Abstract: This study aimed to evaluate functional effects of Bobath therapy in children with cerebral palsy (CP). Fifteen children with a diagnosis of CP were recruited (9 males, 6 females; mean age 7 years 4 months, SD 2 years 8 months; age range 2 to 12 years). Types of motor disorder were as follows: spastic quadriplegia (n=9); spastic diplegia (n=4); athetoid quadriplegia (n=1), and ataxia (n=1). Participants were distributed across the following Gross Motor Function Classification levels: level I, n=1; level II, n=4; level III, n=5; level IV, n=4; and level V, n=1. Children awaiting orthopaedic intervention were excluded. A repeated measures design was used with participants tested with the Gross Motor Function Measure (GMFM) and Pediatric Evaluation of Disability Inventory (PEDI) at 6-weekly intervals (baseline, before and after Bobath therapy, and follow-up). As the data were of ordinal type, non-parametric statistics were used, i.e. Wilcoxon's test. Participants showed a significant improvement in scores in the following areas following Bobath therapy compared with the periods before and after Bobath therapy: GMFM total score (p=0.009); GMFM goal total (p=0.001); PEDI self care skills (p=0.036); and PEDI caregiver assistance total score (p=0.012). This demonstrates that in this population, gains were made in motor function and self care following a course of Bobath therapy.
TL;DR: The ketogenic diet generally provides sufficient nutrition to maintain growth within normal parameters over a defined period and should be followed-up carefully over long periods of use.
Abstract: This is a prospective cohort study of 237 children (130 males, 107 females) placed on the ketogenic diet for control of intractable epilepsy (mean age at starting diet 3 years 8 months; age range 2 months to 9 years 10 months); average length of follow-up was 308 days. There were 133 children on the diet at 1 year and 76 at 2 years. Height and weight measurements were converted into age- and sex-appropriate z scores. There was a rapid drop in weight z scores in the first 3 months. After this initial period, the weight z score remained constant in children who started the diet below the median weight for their age and sex, although z scores continued to decrease in children starting above the median. There was a small decrease in height z scores in the first 6 months (<0.5); however, there were larger changes by 2 years. There was no difference based on sex for either height or weight. The ketogenic diet generally provides sufficient nutrition to maintain growth within normal parameters over a defined period. Very young children grow poorly on the diet and should be followed-up carefully over long periods of use.
TL;DR: Assessment of feeding difficulties in children with disabilities confirmed the significant impact of neurological impairment in children on body growth and nutritional status becoming worse in those with a greater degree of motor impairment.
Abstract: Poor nutritional status and growth failure are common in children with cerebral palsy (CP). The aim of this study was to assess, within a subgroup of a large and clearly defined population of children with disabilities, the impact of feeding difficulties on (1) the quality (micronutrient intake) and quantity (macronutrient intake) of their diet and (2) their growth. One hundred children with disabilities (40 females, 60 males; mean age 9 years, SD 2 years 5 months; range 4 years 6 months to 13 years 7 months) underwent a detailed dietetic analysis and a comprehensive anthropometric assessment. Diagnostic categories of disability were: CP (n=90); global developmental delay (n=3); Marfan syndrome (n=1); intractable epilepsy (n=2); agenesis of the corpus callosum (n=2); methyl malonic aciduria (n=1); and congenital rubella (n=1). Neurological impairment was classified according to difficulty with mobility which was graded as mild (little or no difficulty walking), moderate (difficulty walking but does not need aids or a helper), and severe (needs aids and/or a helper or cannot walk). Results confirmed the significant impact of neurological impairment in children on body growth and nutritional status becoming worse in those with a greater degree of motor impairment. The major nutritional deficit was in energy intake, with only one fifth reportedly regularly achieving over 100% estimated average requirement (EAR), whilst micronutrient intake was less markedly impaired and protein intake was normal in this group (96% above EAR). Many children with neurological impairment would benefit from individual nutritional assessment and management as part of their overall care.
TL;DR: Benign paroxysmal torticollis of infancy is a disorder characterized by recurrent episodes of head tilt secondary to cervical dystonia, which may be regarded as a migraine aura equivalent.
Abstract: Benign paroxysmal torticollis of infancy (BPTI) is a disorder characterized by recurrent episodes of head tilt secondary to cervical dystonia. Attacks are often accompanied by vomiting, pallor, and ataxia, settling spontaneously within hours or days. Episodes begin within the first 12 months of life and resolve by 5 years. We report four patients with BPTI. Symptoms started from 3 months of age, with head tilting lasting between 10 minutes and 2 months; the shorter episodes were followed by vomiting, apathy, and unsteadiness. Head tilt became less prominent after infancy, replaced by vertigo and eventually by migraine headaches. Two patients came from a kindred with familial hemiplegic migraine linked to CACNA1A mutation. BPTI may be regarded as a migraine aura equivalent. The syndrome poses interesting questions regarding varying phenotypic expression of calcium channelopathies at different stages of development.
TL;DR: The safety profile of the toxin appears satisfactory and 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.
Abstract: 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 degrees, and was still present at 16 weeks. The safety profile of the toxin appears satisfactory.
TL;DR: Twenty-seven MRI examinations from 17 children with bilateral lesions of the basal ganglia and thalamus were reevaluated, and correlated with the type of cerebral palsy as well as motor and cognitive impairment and type of CP.
Abstract: Twenty-seven MRI examinations from 17 children (7 females, 10 males) with bilateral lesions of the basal ganglia and thalamus, presenting over a period of 8 years, were reevaluated, and correlated with the type of cerebral palsy (CP) as well as motor and cognitive impairment. Children were between 1 year 6 months and 17 years old at last examination (mean 5 years 9 months). Brain damage had occurred as a consequence of birth asphyxia in nine patients and of neonatal shock in four patients. No adverse event could be identified in four children. In these, late prenatal compromise is assumed, as extensive screening (including MR spectroscopy in two patients) did not yield an underlying metabolic disorder. Three different degrees of MRI lesion patterns could be defined: a mild pattern (involvement of nucleus lentiformis and ventro-lateral thalamus only; n=7), an intermediate pattern (involvement of nucleus lentiformis, ventro-lateral thalamus, and pericentral region; n=3), and a severe pattern (involvement of nucleus lentiformis, entire thalamus, pericentral region, and hippocampus; n=7). This grading of MRI findings correlated significantly with the severity of both cognitive and motor impairment and type of CP. Normal cognitive development and mild motor delay was only seen with the mild pattern. All children developed CP: purely dyskinetic CP was only seen with the mild pattern, whereas the dyskinetic-spastic or spastic CP types could be seen in all three lesion patterns, with dyskinetic-spastic CP more related to the moderate, and purely spastic CP more related to the severe pattern.
TL;DR: It is concluded that VLBW children, in the absence of these identified risk factors, have normal cognitive development.
Abstract: In a population-based follow-up study (the Stockholm Neonatal Project), 182 children with a birthweight of 1500 g or less (very-low birthweight: VLBW) and a control group of 125 children born healthy at term were examined with the Wechsler Preschool and Primary Scale of Intelligence-Revised (WPPSI-R) and a neuropsychological test battery (Nepsy) at 5 1/2 years of age. The WPPSI-R results of the VLBW children fell well within the normal range: WPPSI-R full-scale IQ 95.7, verbal subscale IQ 99.9, and performance subscale IQ 91.6. Nevertheless, the control group had significantly better results than the VLBW group which could be attributed to the greater variability of the VLBW group, with a larger proportion falling in the lower area of the IQ distribution, especially on the performance subscale. Likewise, the control group displayed better executive function (Nepsy). Paternal education was equal in the two groups and was the single most important predictor of IQ, possibly acting as a protective factor. The need for glasses or lenses was inversely associated with all IQ measures and severe retinopathy of prematurity (ROP) had the most negative impact on full-scale and performance IQ. These two IQ measures were also negatively associated with intrauterine growth retardation late in pregnancy. We conclude that VLBW children, in the absence of these identified risk factors, have normal cognitive development.
TL;DR: ATVV intervention facilitated increased alertness, faster transition to complete nipple feeding, and decreased length of hospitalization of 37 preterm infants.
Abstract: 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.
TL;DR: Results showed that four treatments per week over a 4-week period were well tolerated when separated by rest periods and the intermittent program led to improvements in motor function that were maintained over the rest periods.
Abstract: The aims of the study were: (1) to determine the feasibility of a rehabilitation program combining intensive therapy periods (4 times/week for 4 weeks) with periods without therapy (8 weeks) over a 6-month period in severely impaired children with cerebral palsy (CP); and (2) to measure changes in gross motor function after intensive therapy periods (immediate effects) and rest periods (retention). A convenient sample included five children (two females, three males; mean age 22.6 months [SD 9.9]) with severe forms of CP with impairment of four limbs and trunk (GMFCS levels IV and V). A multiple-baseline design was used. Changes in motor performance were assessed by a blind evaluator using the Gross Motor Function Measure. Visual and statistical analyses followed. Level of compliance during intensive therapy was 93.1%. Children received a mean of 30 treatments over the 24 weeks of the experimental phase compared with the 48 treatments they would have received routinely. Increases in GMFM scores (mean 9.2%; range 3 to 15%) were significant in three children (p<0.05) and all participants maintained their motor performance during the two 8-week rest periods. Results showed that four treatments per week over a 4-week period were well tolerated when separated by rest periods. The intermittent program led to improvements in motor function that were maintained over the rest periods. Results underline the need to reconsider the organization of physical rehabilitation programs. A regime that is intensive enough without being tiring and one that provides practice conditions for consolidating motor skills learned during the intensive therapy period may best optimize motor training.
TL;DR: The findings support the literature, which states that individuals with spastic diplegic CP are more involved distally compared with proximally in the lower extremities, and conflict with the literature which contains several assumptions that a spastic muscle is a strong muscle and that spasticity causes weakness in the opposing muscle group.
Abstract: The relation between spasticity and strength in individuals with cerebral palsy (CP) has not been extensively researched. Knee and ankle spasticity and strength were quantified in a retrospective analysis of 60 individuals with spastic diplegic CP (mean age 12 years, range 3 to 38) and a group of 50 individuals without disabilities (WD group; mean age 12 years, range 4 to 36). Spasticity was measured using a KinCom dynamometer that stretched the passive knee flexors or ankle plantarflexors at different speeds and recorded the amount of resistive torques. For the strength tests, the participant performed a maximum contraction of the knee flexors/extensors and ankle plantarflexors/dorsiflexors throughout their range of motion at a speed of 10°/s on the dynamometer. Pearson's correlation coefficient was used to determine if a relation existed between spasticity and strength within the same muscle group and in opposing muscle groups at the knee and ankle joints. A t-test was performed to determine if greater spasticity and less strength existed at the ankles compared with the knees in those with CP. Results show that there was no relation between spasticity and strength either within the same muscle group or at opposing muscle groups at the knee and ankle joints in persons with CP. Individuals with spastic diplegic CP were more involved (greater spasticity, less strength) distally at the ankles compared with the knees. The findings conflict with the literature, which contains several assumptions, one of which is that a spastic muscle is a strong muscle and that spasticity causes weakness in the opposing muscle group. We found no relation between spasticity and strength in individuals with CP. Our findings support the literature, which states that individuals with spastic diplegic CP are more involved distally compared with proximally in the lower extremities.
TL;DR: The present data suggest that children with SLI show a specific deficit in automatic discrimination of CV syllables differing in place of articulation, whereas the processing of simple tone differences seems to be unimpaired.
Abstract: It is well known from behavioural experiments that children with specific language impairment (SLI) have difficulties discriminating consonant-vowel (CV) syllables such as /ba/, /da/, and /ga/. Mismatch negativity (MMN) is an auditory event-related potential component that represents the outcome of an automatic comparison process. It could, therefore, be a promising tool for assessing central auditory processing deficits for speech and non-speech stimuli in children with SLI. MMN is typically evoked by occasionally occurring 'deviant' stimuli in a sequence of identical 'standard' sounds. In this study MMN was elicited using simple tone stimuli, which differed in frequency (1000 versus 1200 Hz) and duration (175 versus 100 ms) and to digitized CV syllables which differed in place of articulation (/ba/, /da/, and /ga/) in children with expressive and receptive SLI and healthy control children (n=21 in each group, 46 males and 17 females; age range 5 to 10 years). Mean MMN amplitudes between groups were compared. Additionally, the behavioural discrimination performance was assessed. Children with SLI had attenuated MMN amplitudes to speech stimuli, but there was no significant difference between the two diagnostic subgroups. MMN to tone stimuli did not differ between the groups. Children with SLI made more errors in the discrimination task, but discrimination scores did not correlate with MMN amplitudes. The present data suggest that children with SLI show a specific deficit in automatic discrimination of CV syllables differing in place of articulation, whereas the processing of simple tone differences seems to be unimpaired.
TL;DR: It is concluded that TIMP scores significantly predict AIMs PR 6 to 12 months later, but the TIMP at 3 months of age has the greatest degree of validity for predicting motor performance on the AIMS at 12 months and can be used clinically to identify infants likely to benefit from intervention.
Abstract: The Test of Infant Motor Performance (TIMP) is a test of functional movement in infants from 32 weeks' post-conceptional age to 4 months postterm. The purpose of this study was to assess in 96 infants (44 females, 52 males) with varying risk, the relation between measures on the TIMP at 7, 30, 60, and 90 days after term age and percentile ranks (PR) on the Alberta Infant Motor Scale (AIMS). Correlation between scores on the TIMP and the AIMS was highest for TIMP tests at 90 days and AIMS testing at 6 months (r=0.67, p=0.0001), but all comparisons were statistically significant except those between the TIMP at 7 days and AIMS PR at 9 months. In a multiple regression analysis combining a perinatal risk score and 7-day TIMP measures to predict 12-month AIMS PR, risk, but not TIMP, predicted outcome (21% of variance explained). At older ages TIMP measures made increasing contributions to prediction of 12-month AIMS PR (30% of variance explained by 90-day TIMP). The best TIMP score to maximize specificity and correctly identify 84% of the infants above versus below the 10th PR at 6 months was a cut-off point of 1 SD below the mean. The same cut-off point correctly identified 88% of the infants at 12 months. A cut-off of -0.5 SD, however, maximized sensitivity at 92%. A negative test result, i.e. score above -0.5 SD at 3 months, carried only a 2% probability of a poor 12-month outcome. We conclude that TIMP scores significantly predict AIMS PR 6 to 12 months later, but the TIMP at 3 months of age has the greatest degree of validity for predicting motor performance on the AIMS at 12 months and can be used clinically to identify infants likely to benefit from intervention.
TL;DR: The hypothesis that children with autistic spectrum disorders often have macrocephalus is tested, and that those without comorbid learning disability are most frequently affected, is found to be correct.
Abstract: This study was undertaken to test the hypothesis that children with autistic spectrum disorders often have macrocephalus, and that those without comorbid learning disability are most frequently affected. Fifty consecutive children with Asperger syndrome (45 males, five females; mean age 9 years, range 1 year 6 months to 16 years) without indications of underlying medical disorders were matched for birth year and sex with 50 children (45 males, five females; mean age 6 years 4 months, range 1 year 4 months to 13 years 11 months) who met criteria for autistic disorder (a lower-functioning disorder within the autism spectrum) and with 50 children (45 males, five females; mean age 8 years 4 months, range 1 year 6 months to 15 years 5 months) who met criteria for attention-deficit-hyperactivity disorder. Birth and neuropsychiatric follow-up records were examined and data relating to occipitofrontal circumference, weight, and height were detailed. The group with Asperger syndrome included a subset of individuals with macrocephalus recorded both at birth and at follow-up after the first year of life. Another subgroup developed macrocephalus during early childhood. Autistic spectrum disorders include a subgroup with macrocephalus characterized by a relatively high level of functioning and a clinical presentation most often consistent with a diagnosis of Asperger syndrome.
TL;DR: Findings for a community-based sample show many similarities with studies of clinically referred samples suggesting that teacher-completed ratings of DSM-IV symptoms may be a useful methodology for investigating the phenomenology of tic disorders.
Abstract: This study examined comorbid psychiatric symptoms in a large, community-based sample of children and adolescents. The study sample comprised a total of 3006 school children: 413 preschool (3 to 5 years; 237 males, 176 females; mean age 4 years 2 months, SD 8 months), 1520 elementary school (5 to 12 years; 787 males, 733 females; mean age 8 years 2 months, SD 1 year 11 months), and 1073 secondary school children (12 to 18 years; 573 males, 500 females; mean age 14 years 4 months, SD 1 year 10 months), all of whom were attending regular education programs. Children were evaluated with a teacher-completed DSM-IV-referenced rating scale. The sample was divided into four groups: attention-deficit-hyperactivity disorder with tics (ADHD+tics), ADHD without tics (ADHD), tics without ADHD (T), and a comparison group i.e. neither ADHD nor tics (Non). The percentage of children with tic behaviors varied with age: preschool children (22.3%), elementary school children (7.8%), and adolescents (3.4%). Tic behaviors were more common in males than females, regardless of comorbid ADHD symptoms. For many psychiatric symptoms, screening prevalence rates were highest for the ADHD groups (ADHD+tics>ADHD>T>Non). However, obsessive-compulsive and simple and social phobia symptoms were more common in the groups with tic behavior. Findings for a community-based sample show many similarities with studies of clinically referred samples suggesting that teacher-completed ratings of DSM-IV symptoms may be a useful methodology for investigating the phenomenology of tic disorders.