The tonic stretch reflex threshold in children with cerebral palsy (CP) was measured to determine its test–retest reliability and its concurrent validity as a potential measure of spasticity. Fourteen children with spastic CP aged 6 to 18 years were tested on three separate occasions for clinical spasticity and stretch reflex thresholds of affected elbow flexors. Electromyographic (EMG) recordings were obtained by surface electrodes for elbow flexors and extensors during mechanical displacements of the passive joint towards extension. Displacements were produced by a torque motor at seven velocities which randomly varied from trial to trial. EMG activity was measured in the stretched flexor muscles to determine threshold angles and velocities for each velocity of stretch. These were plotted on a velocity–angle-phase diagram and regression analysis was used to determine the static stretch reflex threshold for each participant. The measure showed good test–retest reliability for the group (ICC 0.73, p<0.001) whereas a significant correlation between the measure and the clinical spasticity scale was not found. This technique is a potential outcome variable for measuring the efficacy of treatments aimed at decreasing spasticity in children with CP.

There are two major forms of developmental motor disorders: cerebral palsy (CP) and clumsiness. CP is an umbrella term covering a group of non-progressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of development. It affects about 1 in 500 live-born children. Clumsy children nowadays are classified according to DSM-IV as having developmental coordination disorder (DCD), a term denoting children who have such poor motor coordination that it affects daily activities at home and at school, but with normal intelligence and without obvious neurological pathology. The prevalence of DCD is about 10% (6 to 13%). The understanding of the motor problems in both groups of children is hampered by the large heterogeneity within each group. In both groups children differ in the expression of their motor dysfunction, in comorbidity such as the presence of cognitive and behavioural disorders, and in aetiology. In addition, the relation between the various motor disorders and the structural abnormalities of the brain is far from simple. CP is, by definition, attributed to lesions or anomalies of the young brain, but the abnormalities of the brain cannot always be visualized with imaging techniques. Despite there being some relation between lesions of the periventricular white matter and spastic diplegia, and between unilateral lesions and spastic hemiplegia, the site of the lesion does not correlate exactly with the type of motor dysfunction. In children with DCD the connection between structural abnormalities of the brain and motor dysfunction is still more ambiguous. Recently Hadders-Algra and Touwen argued that indications for pre- and perinatal brain damage can be found only in one third of children with minor motor dysfunction. This could imply that most clumsy children do not have macroscopic anomalies of the brain, but dysfunctions at the microscopic level of the nervous system, with abnormalities in the neurotransmitter or receptor systems, for example.

Knowledge about the level of functional independence that can be expected in adulthood might support decisions on the treatment of newborn infants with spina bifida. This study determined functional independence among young adults with spina bifida and its relationships with pathological characteristics known from birth (hydrocephalus and level of lesion). Data were collected from medical records and by physical examination. Functional independence was assessed on six domains (self-care, sphincter control, transfers, locomotion, communication, and social cognition) using the Functional Independence Measure (FIM). Participants were 165 patients with spina bifida (69 males, 96 females; age range 16 to 25y, mean 20y 9mo [SD 2.9]; 117 with hydrocephalus). Patients without hydrocephalus were independent for all FIM domains except sphincter control, as were patients with hydrocephalus with a lesion level below L2. Most patients with hydrocephalus and a lesion at L2 or above were dependent as regards sphincter control (98%), locomotion (79%), and self-care (54%), and quite a few needed support in transfers (38%), social cognition (29%), and communication (15%).

Thirty-nine ambulant children (22 with hemiplegia, 17 with diplegia) with spastic cerebral palsy receiving isolated gastrocnemius muscle injection with botulinum toxin A were studied prospectively. The children had a mean age of 6 years (range 3 to 13 years). Measurement of gastrocnemius muscle length was used to estimate the dynamic component of each child's spasticity and to quantify the response. There was a strong correlation between the dynamic component of spasticity before injection and the corresponding magnitude of the response after injection. Children undergoing repeated injections showed similar correlations. A strong correlation was found between the duration of response and the dynamic component. Children with hemiplegia showed twice the duration for a given dynamic component compared with those with diplegia when injected with the same total dose per unit body weight. Long-term lengthening did not occur for the cohort, although some patients showed a response at a 12-month follow-up. By delaying shortening, the injections may have a role in delaying the need for surgery. Injections were well tolerated with few side effects.

The objectives of this study were threefold: (1) to evaluate the accuracy of clinical evaluation compared with videofluoroscopic swallowing studies (VFSSs) in the detection of penetration and aspiration in children of age 0 to 15 years presenting with feeding and swallowing problems; (2) to assess the relationship between therapists' confidence ratings in making judgements about the presence or absence of penetration and aspiration, and the accuracy of their evaluation as confirmed by VFSSs; (3) to identify clinical predictors of penetration and aspiration during clinical evaluation of children with feeding and swallowing difficulties. We used a prospective study to evaluate the sensitivity, specificity, and positive and negative predictive values of a diagnostic clinical evaluation compared with VFSSs (criterion standard). Clinical evaluation and videofluoroscopy forms for oral motor and swallowing evaluation, which included potential indicators of aspiration, were designed for this project. Seventy-five children with feeding problems participated (33 females, 42 males; age range 0 to 14 years, mean 2 years; 62% of participants younger than 12 months). For fluids, clinical evaluation showed a sensitivity of 92% for aspiration. For solids, sensitivity for detecting aspiration was 33%. Analysis of the therapists' mean confidence ratings compared with the accuracy of their judgement demonstrated that when therapists were very sure that the child was aspirating or penetrating or not, they were correct. When the therapists were unsure, then the accuracy of prediction was not as good. Cough was the most significant predictor (p<0.05) of fluid aspiration and penetration. We conclude that clinical evaluation with experienced clinicians can detect aspiration and penetration of fluids in children of varied ages and diagnoses, but that it is not accurate with solids.

Lower respiratory tract infections in children with severe neurodisability are usually caused by aspiration of stomach contents from gastroesophageal reflux (GOR) or direct aspiration (DA) of food due to oral and pharyngeal motor problems. To determine the contributions and interactions of GOR and DA, oesophageal 24-hour pH monitoring and feeding videofluoroscopy were performed in 34 children (age range 7 months to 16 years, mean 7 years) who had severe physical and learning disabilities and who were slow feeders. Subjects were divided into three groups according to the frequency of their respiratory tract infections. Subjects in group 1 had no respiratory tract infections (N=10); five had GOR and none had DA. Subjects in group 2 had minor respiratory tract infections but had not received more than one course of antibiotics for this in the previous year (N=8); two had GOR alone, four had DA alone, and two had neither. All subjects in group 3 had recurrent respiratory tract infections (N=16); one had GOR alone, seven had DA alone, and eight had both GOR and DA. This study suggests that oral and pharyngeal motor problems are the major cause of respiratory tract infection in children with severe neurodisability. These problems lead to DA and, if GOR is present, to the aspiration of stomach contents. Those children with both DA and GOR are more likely to have severe respiratory tract infections which may lead to gastrostomy feeding (together with fundoplication). GOR without sufficient oral and pharyngeal motor problems to cause DA is less likely to cause respiratory tract infection in children with severe neurodisability.

Children with autism have a relatively shorter index finger (2D) compared with their ring finger (4D). It is often presumed that the 2D:4D ratio is associated with fetal testosterone levels and that high fetal testosterone levels could play a role in the aetiology of autism. It is unknown whether this effect is specific to autism. In this study, 2D:4D ratios of 144 males aged 6 to 14 years (mean age 9y 1mo [SD 1y 11mo]) with psychiatric disorders were compared with those of 96 males aged 6 to 13 years from the general population (mean age 9y 1mo [SD 1y 10mo]). Psychiatric disorders were divided into autism/Asperger syndrome (n=24), pervasive developmental disorder–not otherwise specified (PDD–NOS; n=26), attention-deficit–hyperactivity disorder (ADHD)/oppositional defiant disorder (ODD; n=68), and anxiety disorders (n=26). Males with autism/Asperger syndrome (p<0.05) and ADHD/ODD (p<0.05) had significantly lower (though not significantly; p=0.52) ratios than males with an anxiety disorder, and males with autism/Asperger syndrome had lower ratios than those in the comparison group. These results indicated that higher fetal testosterone levels may play a role, not only in the origin of autism, but also in the aetiology of PDD–NOS and of ADHD/ODD. Males with anxiety disorders might have been exposed to lower prenatal testosterone levels.

The repeatability of energy-expenditure measurements were studied in five children and four adults without disabilities using the Cosmed K4 (high technology). The ability to detect change in measurements was compared between this instrument and the Physiological Cost Index (PCI; low technology). The results of repeatability (95% range) for oxygen cost were 13.1% in children and 13% in adults. In contrast, the SD of PCI was 6 to 72% of the mean in adults and wider in children (91%; 95% range). The validity of PCI as an outcome measure was questioned. In addition, 177 children with motor disability were prospectively studied using the Cosmed K4. Previous experience with the Cosmed K2 (intermediate technology) helped to develop a practical and repeatable protocol for testing children with disability using the Cosmed K4. The protocol commenced with 5 minutes of rest to achieve baseline values of heart rate and oxygen consumption, followed by 10 minutes of continuous walking at a self-selected speed on a 10-metre level oval walking track. The test concluded with 5 minutes of rest to monitor the return to baseline values. Ninety-one percent of the children with disability quickly reached a steady-state of oxygen consumption and carbon-dioxide production. The carbon-dioxide sensor in the Cosmed K4 has enabled a new group of severely involved children with cerebral palsy (9%) to be defined. These children have been termed ‘physiologically marginal ambulators’.

The aim of the present research was to investigate the impact of abnormal fetal environment on explicit memory performance. Based on animal models, it was hypothesized that infants of diabetic mothers (IDMs) experience perturbations in memory performance due to exposure to multiple neurologic risk factors including: chronic hypoxia, hyperglycemia/reactive hypoglycemia, and iron deficiency. Memory performance, as measured by the elicited/deferred imitation paradigm, was compared between 13 IDMs (seven females, six males; mean age 365 days, SD 11) and 16 typically developing children (seven females, nine males; mean age 379 days, SD 9). The IDM group was characterized by shorter gestational age (mean 38w, SD 2), greater standardized birthweight scores (mean 3797g, SD 947), and lower iron stores (mean ferritin concentration 87μg/L, SD 68) in comparison with the control group (mean gestational age: 40w, SD 1; mean birthweight: 3639g, SD 348; mean newborn ferritin concentration 140μg/L, SD 46). After statistically controlling for both gestational age and global cognitive abilities, IDMs demonstrated a deficit in the ability to recall multi-step event sequences after a delay was imposed. These findings highlight the importance of the prenatal environment on subsequent mnemonic behavior and suggest a connection between metabolic abnormalities during the prenatal period, development of memory, circuitry, and behavioral mnemonic performance.

Six boys and five girls with a mean age of 8.6 (range 3 to 13) years with foetal alcohol syndrome (FAS) were studied by MRI and single photon emission computed tomography (SPECT) to find specific areas of vulnerability. Morphological anomalies shown in six of 11 patients by MRI were situated both cortically and subcortically: cortical atrophy (N=2), dilated ventricle (N=1), corpus callosum hypoplasia (N=1), cerebellar atrophy (N=2), one of the latter with Arnold–Chiari malformation (N=1). Delayed myelination of the white matter was seen in two patients. Volumetric studies of the hippocampus showed morphological left–right asymmetry in five of eight patients. However, SPECT showed mild hypoperfusion of the left hemisphere in all 10 subjects. The negative left–right index was located especially in the left parietooccipital region, i.e. in the brain areas implicated in arithmetical and logical–grammatical functions, which are known to be affected in FAS. Normal left–right dominance was also lacking in the frontal area, i.e. the brain area affected in attention-deficit–hyperactivity disorder (ADHD). Detection of these abnormalities, although they are not unique to FAS, may be helpful in the diagnosis and any attempts at rehabilitation. Diverse morphological and functional abnormalities are more frequent than has usually been believed even in less impaired children with FAS.

This study aimed to determine whether neonatal feeding performance can predict the neurodevelopmental outcome of infants at 18 months of age. We measured the expression and sucking pressures of 65 infants (32 males and 33 females, mean gestational age 37.8 weeks [SD 0.5]; range 35.1 to 42.7 weeks and mean birthweight 2722g [SD 92]) with feeding problems and assessed their neurodevelopmental outcome at 18 months of age. Their diagnoses varied from mild asphyxia and transient tachypnea to Chiari malformation. A neurological examination was performed at 40 to 42 weeks postmenstrual age by means of an Amiel-Tison examination. Feeding performance at 1 and 2 weeks after initiation of oral feeding was divided into four classes: class 1, no suction and weak expression; class 2, arrhythmic alternation of expression/suction and weak pressures; class 3, rhythmic alternation, but weak pressures; and class 4, rhythmic alternation with normal pressures. Neurodevelopmental outcome was evaluated with the Bayley Scales of Infant Development–II and was divided into four categories: severe disability, moderate delay, minor delay, and normal. We examined the brain ultrasound on the day of feeding assessment, and compared the prognostic value of ultrasound and feeding performance. There was a significant correlation between feeding assessment and neurodevelopmental outcome at 18 months (p<0.001). Improvements of feeding pattern at the second evaluation resulted in better neurodevelopmental outcome. The sensitivity and specificity of feeding assessment were higher than those of ultrasound assessment. Neonatal feeding performance is, therefore, of prognostic value in detecting future developmental problems.

Children with bilateral cerebral palsy (CP) born during 1989 to 1992 (n=346) to a geographically defined population were ascertained and followed up to age 5 years. The aims of the study were to monitor hip development by serial X-rays; to record gross locomotor development, aspects of physical management, and any hip-related orthotics or surgery; to learn more of the natural history of hip development in this condition; and to provide guidelines for a surveillance protocol for those clinically managing children with bilateral CP. Children were X-rayed at 18, 24, 30, 48, and 60 months. X-rays were taken in a standardized position and measured to record migration percentage, acetabular index, Sharp's angle, and Smith's diaphyseal ratios. Relationships between the measures were investigated and a natural history of the children's hip development suggested. At as early as 18 months, migration percentages were significantly greater than in the normally developing population, although no measured CP factor could be identified to be the cause of this early change. Two possible factors affecting early hip development are described, neither of which is clinically apparent: the first separates the bilateral CP population as a whole from the normal population; but there is an additional presdisposing factor affecting some children only which in the presence of the motor disorder, has a significant influence on early hip development and subsequent dysplasia. The study confirms that, when measured correctly, migration percentage is the best guide to hip surveillance and the need for treatment. It is suggested that all children with bilateral CP should be X-rayed in a standardized position at 30 months (corrected for gestational age).

The quality of general movements (GMs) was assessed repeatedly during the first postnatal months in a mixed group of 52 children at either low or high risk for neurodevelopmental disorders. In addition, all children were reexamined at 4 to 9 years. The follow-up assessment consisted of a neurological examination and an evaluation of behaviour by means of parental questionnaires. The quality of GMs changed frequently, to stabilize in the final phase. The final GM phase is that of the so-called fidgety GMs which occurs between 2 and 4 months postterm. The quality of the fidgety GMs predicted outcome very well. Definitely abnormal GMs were associated with a high risk for the development of cerebral palsy, whereas mildly abnormal GMs were associated with the development of minor neurological dysfunction, attention-deficit–hyperactivity disorder, and aggressive behaviour.

The possibility that so-called anti-epileptic drugs (AEDs) may aggravate epilepsy must always be borne in mind by the clinician. Many reports of such aggravation of seizures have been published. Most such reports are anecdotal and speculative, and suggest that many such reactions are idiosyncratic. However, for some there is a sufficient body of evidence to suggest that some AEDs used in certain epilepsies may consistently cause worsening of seizures. Seizure aggravation may include increase in the frequency or severity of existing seizures, emergence of new types of seizure, or the occurrence of status epilepticus. The pathophysiology of seizure aggravation is poorly understood including non-specific effects such as those associated with sedation, drug-induced encephalopathy, and paradoxical or inverse pharmacodynamic effects. For some epilepsies the choice of AEDs may be inappropriate, and although the mechanism of seizure aggravation is not clear, its occurrence may be fairly predictable. This is best documented for the use of carbamazepine in idiopathic generalized and myoclonic epilepsies. Most other AEDs have been reported occasionally to cause seizure aggravation. The lowest risk of seizure aggravation appears to be with valproate. Risk factors for worsening of seizures are epileptic encephalopathy, polytherapy, high frequency of seizures, and cognitive impairment. Advances in pharmacogenomics may in the future enable such adverse effects to be predicted for individual patients. Meanwhile, a systematic approach to reporting AED-induced seizure aggravation should be developed.

This study sought to determine whether neonatal cranial ultrasound abnormalities are related to motor and cognitive outcomes in low-birthweight (LBW, <2000 g) children without major motor or cognitive disability. The relation of neonatal cranial ultrasound abnormalities to motor performance and general cognitive ability at ages 2, 6, and 9 years was examined in a prospectively followed regional cohort of LBW children, excluding those with major disability, and controlling for other risk factors. Compared with children without ultrasound abnormalities, children with abnormalities indicative of ischemic white-matter injury had poorer motor performance at all three ages and, at age 2, lower general cognitive ability. The latter finding was not seen when motor performance was also controlled for. It was concluded that in LBW children without major motor or cognitive disability, ischemic white-matter injury indicated by neonatal cranial ultrasound abnormalities adversely affected motor performance at ages 2, 6, and 9 years, but not general cognitive ability.

The present investigation examines the relation between various clinical measures and the performance of a functional precision grip–lift task. Fifteen children with hemiplegic cerebral palsy (CP), aged 8 to 14 years, and 15 age-matched control children grasped and lifted an object whose surface texture was varied while their fingertip forces were recorded. The force coordination was compared with tactile sensibility, grip strength, manual dexterity, and spasticity using correlational and regression analyses. The findings highlight the importance of tactile sensibility in this task. However, the manner in which sensibility was related varied for the sensory adaptation of fingertip forces, the anticipatory scaling of the force increase, and the smooth transitions between the temporal phases comprising the grip–lift task. The findings also indicate that spasticity affects some measures of the task, but not others, suggesting that the relation between spasticity and motor performance may not be absolute. The results further suggest that the impairments in grasping in children with hemiplegic CP are largely but not exclusively due to disturbed sensory mechanisms which may have direct implications for therapeutic intervention.

Children with epilepsy often experience poor social and educational outcomes. This study aimed to determine the timing of services with respect to the onset of seizures. It also aimed to identify the aspects of childhood epilepsy (type of epilepsy, etiology, seizure control, and treatment) that are associated with the use of special education services. As part of a prospective community-based study, 613 children were recruited when first diagnosed with epilepsy. Mean age at first seizure was 5 years 11 months (SD 4, range 1mo to 15y 8mo). Parents were interviewed 5 years after children were first diagnosed with epilepsy (n=542; 276 [51%] males). Children's mean age at time of interview was 11 years 10 months (SD 4y 1mo, range 5y 8mo to 21y 8mo). Etiology was classified as idiopathic (n=181, 33.4%), cryptogenic (n=261, 48.2%), and remote symptomatic (n=100, 18.5%). Service use was reported in 315 (58%) children. Compared with neurologically intact children (i.e. cryptogenic and idiopathic etiology; n=415, 77%), children with a remote symptomatic etiology and/or an epileptic encephalopathy (n=127, 23%) received services more frequently (88% vs 49%, p<0.001). In the former group, services were initiated for 66 (15%) children before their first seizure; according to age at onset, services were initiated before the first seizures in 12/164 (7.3%) if <5 years, 34/171 (19.9%) if 5–9 years, and 20/80 (25%) if >10 years. A large proportion of children with epilepsy, even if neurologically otherwise normal, receive special education services. Initiation of services often precedes onset of seizures even in neurologically intact children. This suggests that behavioral and cognitive abnormalities may predate the onset of epilepsy and are not necessarily the direct consequences of epilepsy.

Many children and young adults with cerebral palsy (CP) have diminished bone mineral density (BMD) and a propensity to fracture with minimal trauma. The aim of this study was to identify variables which are routinely assessed as part of standard clinical care and that might be used to identify those individuals with CP who are most likely to have low BMD. One hundred and seven participants (ages 2 years 1 month to 21 years 1 month; mean age 10 years 11 months, SD 4 years 2 months) with moderate to severe spastic CP were assessed in detail. This included gathering clinical data, taking anthropometric measures of growth and nutrition, as well as dual energy X-ray absorptiometry measures of BMD. Seventeen participants were ambulatory with assistance (Gross Motor Function Classification System [GMFCS] level III), and 90 were capable of little or no ambulation even with assistance (26 GMFCS level IV and 64 GMFCS level V). Weight z score proved to be the best predictor of BMD z score. Declining BMD z scores also correlated with increasing age and greater severity of involvement. It can be predicted, with reasonable reliability, that a 10-year-old non-ambulatory child with quadriplegic CP and a ‘typical’ weight z score of –2 will have a BMD z score that is at best –2. Prior fractures, use of anticonvulsants, and feeding difficulties further reduce predicted BMD.

The aim of this study was to examine measures of health-related quality of life (HRQL) in children with cerebral palsy (CP) by comparing scores of a generic HRQL measure, the Child Health Questionnaire (CHQ); a disease-specific HRQL measure for children with CP, the Caregiver Questionnaire (CQ); and a pediatric functional measure, the Wee-Functional Independence Measure (WeeFIM®). Participants included 30 caregivers of children with CP. The caregivers' children were a mean age of 8 years 6 months (17 females, 13 males). The ethnic origin of the children was 18 African-American, 8 white, 3 Hispanic, and 1 Middle Eastern. Significant correlations were found between the CQ and WeeFIM total and subscale scores (r=0.388 to 0.641). There was no correlation between the CHQ and CQ total summary scores, but significant correlations were found between the CHQ subscales related to parent time and family cohesion and the CQ total and subscale scores (r=0.386 to 0.481). The lack of correlation between the CHQ and WeeFIM indicates HRQL and function are different constructs that cannot be inferred from each other. The fair relationship found between the CQ and WeeFIM suggests that the constructs measured in these two assessments overlap. The lack of correlation between the total summary scores of the CHQ and CQ suggests the CQ may be a more specific measure of HRQL for this population that reflects the impact of the child's condition on the caregiver.

Individual rhythms of suck, swallow, and respiration are disrupted in preterm infants with bronchopulmonary dysplasia (BPD). Integration of respiration into suck–swallow efforts is critical for establishing coordinated suckle feeding. This study quantitatively assessed the coordination of respiration and swallow in infants with and without BPD. Thirty-four preterm infants of 26 to 33 weeks' gestational age were included: 14 participants with BPD (eight males, six females)and 20 comparison participants without BDP (10 males, 10 females). Participants were studied at postmenstrual age 32 to 40 weeks and postnatal age 2 to 12 weeks using digital recordings of pharyngeal pressure, nasal thermistor flow, and thoraco-abdominal plethysmography. The coefficients of variation (COV; standard deviation/mean) of the swallow–breath (SW–BR) and breath–breath (BR–BR) intervals during swallow runs, the percentage of ‘apneic swallows’ (runs of [ges ]3 swallows without interposed breaths), and phase relationships of respiration and swallow were used to quantify rhythmic coordination and integration of respiration into feeding episodes. Apneic swallows were significantly increased after 35 weeks in infants with BPD (mean 13.4% [SE 2.4]) compared with non-BDP infants (6.7% [SE 1.8]; p<0.05), as were SW–BR phase relationships involving apnea. The BPD cohort also had significantly higher SW–BR COV and BR–BR COV than non-BPD infants, indicating less rhythmic coordination of swallowing and respiration during feeding. Results emphasize the need for frequent rests and closer monitoring when feeding infants with respiratory compromise. Quantitative assessment of the underlying rhythms involved in feeding may be predictive of longer-term feeding and neurological problems.