Results of surgery to correct fixed flexion contracture of the knee and improve voluntary knee extension in 39 knees in 20 children (11 females, 9 males; mean age 12 years 8 months, age range 5 to 20 years) with cerebral palsy were analyzed. All patients had neuromotor disease and 18 children had spastic diplegia or quadriplegia. All patients could initiate voluntary knee extension but lacked full passive extension. Five patients (10 knees) were free walkers with a mean motor severity index of 19 and mean fixed knee contracture of 20 degrees. Fifteen patients (29 knees) were not free walkers and 13 were wheelchair ambulators. They had a mean motor severity index of 8 and mean fixed knee contractures of 30 degrees. Surgical procedures included various combinations of hamstring lengthening and/or posterior capsulotomy to allow free passive knee extension, with or without quadriceps mechanism shortening, to enhance voluntary extension. The best results were in patients who had hamstring lengthening, posterior capsulotomy, and quadriceps mechanism shortening.

The purpose of this study was to investigate the effectiveness of Johnstone pressure splints (JPSs) on spasticity and cutaneous sensory inputs in children with spastic cerebral palsy (CP). Thirty-four children with spastic diplegic CP participated in this study. Children whose motor development levels were similar were divided into a treatment and a control group. Each group consisted of 17 participants (six females and 11 males). Mean age of the treatment group was 48.82 months (SEM 4.42), and the control group, 47.52 months (SEM 5.27). The treatment group underwent Bobath's neurodevelopmental therapy (NDT) combined with JPSs. The control group underwent NDT alone five days a week for three months. Before and after treatments, lower-extremity passive range of motion (ROM) by goniometric measurements, spasticity by Modified Ashworth Scale (MAS), and somatosensory evoked potentials (SEPs) were measured. Passive ROM showed significant improvements in both groups (p<0.01). In the treatment group, all MAS scores increased. In the control group, the difference was significant except for values of internal rotator muscles. Improvements in passive ROM in the treatment group were significantly higher than the control group except in hip abduction and external rotation (p<0.05). MAS scores of the treatment group were significantly higher than the control group (p<0.05). SEP values increased in both groups but values of the treatment group were significantly higher than the control group (p<0.05).

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.

The purpose of this study was to investigate early electroclinical manifestations and evaluate treatment responses by video-EEG in infants with newly diagnosed spasms. Spasms were recorded in 44 infants (27 males, 17 females) before adequate treatment. Mean ages at onset of spasms and at first video-EEG were 5.3 months (range 0.9 to 9 months) and 5.9 months (range 2.4 to 11.5 months) respectively. Thirteen infants had cryptogenic and 31 had symptomatic aetiology. First treatment was vigabatrin in 36 infants. All infants were followed until 12 months of age or death. Treatment response in the first months of therapy was assessed by repeated video-EEG studies in 23 infants. On the first video-EEG, 34 infants had typical symmetric motor spasms, three infants showed asymmetric or asynchronous behaviour, and seven infants had only subtle spasms. Interictal EEG showed hypsarrhythmia in 27 infants and multifocal spikes with normal or nearly normal background in 17 infants. Subtle spasms, asymmetric or asynchronous spasms, and asymmetric ictal or interictal EEG abnormalities were associated with symptomatic aetiology and poor cognitive and seizure outcome at 12 months. Serial video-EEG recordings showed a transition from motor to subtle spasms during the first 2 weeks of vigabatrin therapy in four infants and only subtle spasms in two therapy-resistant infants at 12 months. Cessation of spasms usually preceded disappearance of hypsarrhythmia or multifocal spikes, but persistence of multifocal spikes over several weeks was always associated with existing spasms. Transition of hypsarrhythmia into multifocal spikes was observed during vigabatrin therapy even in infants with intractable spasms. Initial diagnosis of infantile spasms requires video-EEG studies especially in infants with symptomatic aetiology who may show only subtle spasms. Video-EEG is the only reliable method for assessing treatment response as spasms and interictal EEG abnormalities are modified by treatment and may become subtle.

Twenty healthy preterm infants (gestational age 26 to 33 weeks, postmenstrual age [PMA] 32.1 to 39.6 weeks, postnatal age [PNA] 2.0 to 11.6 weeks) were studied weekly from initiation of bottle feeding until discharge, with simultaneous digital recordings of pharyngeal and nipple (teat) pressure and nasal thermistor and thoracic strain gauge readings. The percentage of sucks aggregated into ‘runs’ (defined as [ges ] 3 sucks with [les ] 2 seconds between suck peaks) increased over time and correlated significantly with PMA (r=0.601, p<0.001). The length of the sucking-runs also correlated significantly with PMA (r=0.613, p<0.001). The stability of sucking rhythm, defined as a function of the mean/SD of the suck interval, was also directly correlated with increasing PMA (r=0.503, p=0.002), as was increasing suck rate (r=0.379, p<0.03). None of these measures was correlated with PNA. Similarly, increasing PMA, but not PNA, correlated with a higher percentage of swallows in runs (r=0.364, p<0.03). Stability of swallow rhythm did not change significantly from 32 to 40 weeks' PMA. In low-risk preterm infants, increasing PMA is correlated with a faster and more stable sucking rhythm and with increasing organization into longer suck and swallow runs. Stable swallow rhythm appears to be established earlier than suck rhythm. The fact that PMA is a better predictor than PNA of these patterns lends support to the concept that these patterns are innate rather than learned behaviors. Quantitative assessment of the stability of suck and swallow rhythms in preterm infants may allow prediction of subsequent feeding dysfunction as well as more general underlying neurological impairment. Knowledge of the normal ontogeny of the rhythms of suck and swallow may also enable us to differentiate immature (but normal) feeding patterns in preterm infants from dysmature (abnormal) patterns, allowing more appropriate intervention measures.

This study examined the evolution of individuals with cerebral palsy (CP) from childhood to adulthood. Seventy-two adults with a diagnosis of CP born between 1934 and 1980 were studied. Individuals were recruited and data comprehensively collected using case notes and through direct assessments of the majority of participants from three rehabilitation units in Bologna, Padua, and Rovigo in Italy. The main findings can be summarized as follows: contact with health and rehabilitation services was radically reduced once individuals reached adulthood; more individuals who were integrated into mainstream schools achieved and maintained literacy than those who had attended special schools; in a high number of participants, motor performance deteriorated once into adulthood. Independent walking or other forms of supported locomotion were lost in many on reaching adulthood. Of those who continued to walk, walking deteriorated in terms of distance. It was concluded that even though CP has been considered as predominantly a childhood pathological condition, the evolution of the effects of CP do not stop at 16 or 18 years of age. For this reason, the traditional child- (or infant-) oriented approach concentrating mainly or exclusively on the achievement of independent walking, may not be an ideal approach to children with CP. Instead a more independence-oriented therapeutic approach would be appropriate.

Neurological integrity in sick children is difficult to assess clinically. The aim of this study was to determine the predictive value of EEG activity recorded with a bedside EEG analysing monitor in an intensive care unit. EEG activity was monitored in 108 children (age range 2 weeks to 16 years, median 1.7 years) considered at risk for cerebral abnormalities with a cerebral function analysing monitor (CFAM). Recordings were evaluated for features of background EEG activity including mean amplitude, frequencies, and symmetry. Electrical seizure activity was quantified if present. Predictive value of the EEG features was evaluated relative to the clinical neurological outcome after one year. Asymmetrical recordings were not seen in any child with a normal outcome. Suppression of background activity was seen in 75% of the children who died. Seizures were present in 68% of children with a poor outcome. Seventeen of the 32 children (65%) who died had prolonged seizures. Absence of seizures and the presence of superimposed fast EEG activity in response to benzodiazepine infusions correlated with good outcome. A combination of two or more predictive EEG features demonstrated >90% specificity and positive predictive likelihood of poor outcome. EEG features provide information about the functional cerebral integrity of sick children. Changes in cerebral activity detected by the CFAM aid decision making by providing such information readily at the bedside in intensive care.

From 1975 to 1 January 1999, 1103 neurologically involved patients (mean age 13.2 years; 686 males, 417 females) referred with problematic drooling, or sialorrhea, were assessed at a pediatric rehabilitation center by a team consisting of an otolaryngologist, speech pathologist, and a dentist. The initial standard treatment for persistent sialorrhea (in the compliant or aware patient) is oral-motor training. A group of 522 patients with persistent significant drooling after a minimum of 6 months of oral-motor training, or profuse drooling in the presence of a low cognitive level, underwent surgery, usually when over 6 years of age. From 1978 to 1 January 1998, the operation of first choice was submandibular duct relocation (SDR), and was completed in a total of 226 patients. Midway through 1988, sublingual gland excision was also completed at the time of submandibular duct relocation (SDRSGE); 249 of these procedures have been completed to January 1st 1999. Those patients who had SDRSGE had significantly fewer complications that required additional surgery than those that had SDR only. However, the impact of surgery on the drooling as evaluated in subsets of both groups (SDR n=115, SDRSGE n=106) was statistically similar. The study of 11 children with salivary gland radionuclitide scans to determine the effect of submandibular duct surgery on gland function was inconclusive. The pattern of oral-motor function in 26 children studied after SDRSGE surgery suggested that those children with severe impairment of volitional motor function and profuse drooling tended to have a poorer outcome following surgery compared to those with milder impairments.

Muscle function often becomes progressively more compromised in children with spastic cerebral palsy, leading to reduced mobility. This study aimed to examine the role that muscle connective tissue plays in this process. Severity of spasticity as determined by a range of clinical measures was assessed in 26 children (14 males 12 females; age range 4 to 17 years) with either diplegic or quadriplegic cerebral palsy (CP). Muscle biopsies from the vastus laeralis muscle were obtained for biomedical and histological analysis during orthopaedic surgery as part of the child's ongoing care. Total collagen was quantified by hydroxyproline determination. Two clinical measures of severity, Modified Ashworth Scale and Balance, were shown to have a highly significant correlation with collagen content, and Ambulatory Status, Clonus, and Selective Muscle Control all showed positive trends. Collagen I accumulated in spastic muscle's endomysium which appeared to be thickened, and fibrotic regions with sparse muscle fibres were evident in more severe cases. This suggests that collagen may be involved in increases in muscle stiffness observed in spasticity. Once developed, these changes are essentially irreversible and we suggest that future treatments should consider including prevention of muscle fibrosis.

In orthopaedic clinical practice hip pain is the main complaint of adults with cerebral palsy (CP). The aim of this descriptive study was to specify prevalence of hip pain and to propose methods of care other than surgery. The study was a cross sectional multicentre one based in the Rhône-Alpes region. Patients over 15 years of age, with CP who were non-ambulatory were included. Two hundred and thirty-four patients were questioned. Mean age of the patients was 27 years 10 months, median 26 years 1 month, with 59.3% males and 40.7% females. Patients were questioned about pain with precise information about the circumstances of pain, tolerance, and treatment. Pain was judged to be intolerable if it prevented usual activities. Prevalence of hip pain was 47.2%. Pain was judged to be tolerable in 35.6% of the 234 patients, i.e. in 75.5% of patients with hip pain. There were three types of pain: provoked pain, pain linked to position, and spontaneous pain. Medical treatment was given to only 13.6% of patients with hip pain. The first treatment for hip pain is to avoid circumstances where pain occurs; medical treatment is reserved for when daily life cannot be adapted sufficiently to prevent pain. Medical treatment must be appropriate with doses of adequate strength before proposing surgery.

To understand how the ‘caregiving context’ could affect responses to procedural pain, the authors sought to determine whether (1) the combined effects of sweet taste and holding (caregiving contact) were greater than the effects of either alone, (2) any combined effects were additive or interactive, and (3) the interventions had similar effects on behavioral (crying and facial activity) and physiological (heart rate, vagal tone) responses to the heel-stick procedure in newborn infants in a randomized two-factorial intervention trial. Eighty-five normally developing newborn infants were studied with a mean gestational age of 39.4 weeks on the 2nd or 3rd day of life. Infants were randomized in blocks of eight to receive (1) no holding and water taste (control participants), (2) no holding and sucrose taste (sucrose group), (3) holding and water taste (holding group), or (4) holding and sucrose taste (holding and sucrose group). Crying was reduced significantly by taste and holding, and the interventions combined additively. Facial activity was only significantly reduced by holding. For physiological measures, the interventions interacted with each other and preintervention levels to reduce heart rate and lower vagal tone more during the procedure in infants in whom heart rate and vagal tone were higher before intervention. Consequently, sweet taste and holding interventions combined in complex ways when acting on different behavioral and physiological response systems to modify stressful pain experiences. The results suggest that providing a caregiving context when painful procedures are performed may be a simple and practical method of reducing pain experience in infants, and that no one measure captures these effects.

Twenty-one individuals (19 females, two males) with teenage- onset anorexia nervosa (AN), 19 of whom were weight restored, were assessed using single-photon emission computed tomography (SPECT) 7 years after onset of AN, at a mean age of 22 years. For comparison we recruited a younger group without neuropsychiatric disorder (mean age 9:8 years; five females, four males) who underwent SPECT at follow-up after an operation for coarctation of the aorta or because of lymphatic leukaemia. Ethical considerations precluded the study of regional cerebral blood flow (rCBF) in participants with completely normal development. The group with AN showed marked hypoperfusion of temporal, parietal, occipital, and orbitofrontal lobes compared to the contrast group. rCBF was not correlated to body mass index in any of the groups. Results suggest that, even long after re-feeding has occurred, AN may be associated with moderate to severe cerebral blood flow hypoperfusion in the temporoparietal (or temporoparietooccipital) region and in the orbitofrontal region. A limitation of the study is that the young contrast group in this study could be expected to have a higher global rCBF than the group with AN. However, this should not significantly affect the relative values used in this study.

Although assessment of the quality of movement in children with cerebral palsy (CP) is difficult, the development of the Gross Motor Performance Measure (GMPM) has facilitated this process. In order to determine the interobserver reliability of the GMPM, 36 children with spastic neuromuscular disorders (mean age 7 years, range 4 to 15 years) were evaluated using four of the five dimensions of the GMPM. Percent Agreement, Intraclass Correlations, and Kappas were calculated by both dimension and attribute to determine reliability. In addition, reliability measures were evaluated over time to determine whether reliability improved with continual use of the GMPM. Overall, interobserver reliability was in the ‘fair to good’ category regardless of the reliability measure used in the analysis. Reliability scores improved over time with a greater number of individual item scores moving from the ‘fair to good’ category to the ‘excellent’ category. Results from this study indicate that it is possible to assess reliably the quality of movement in children with CP.

Acquired epileptic aphasia (AEA, or Landau–Kleffner syndrome) is a unique condition in which children can lose oral language (OL) comprehension and expression for a prolonged period. These children can benefit from visual forms of language, mainly sign language (SL), but the quality of SL has never been analyzed. The case is reported here of a boy with AEA who lost speech comprehension and expression from 3 years 6 months to 7 years and was educated in SL from the age of 6 years. His SL was evaluated at the age of 13 years and 6 months and compared with a control child with congenital sensorineural deafness. It was found that: (1) our patient achieved the same proficiency in SL as the control child with deafness; (2) SL learning did not compete with, but perhaps even hastened, the recovery of OL. Intact ability to learn a new linguistic code such as SL suggests that higher-order language areas were preserved and received input from a separate visual route, as shown by neuropsychological and functional imaging research in deaf and hearing signers.

To assess time costs of caring for children with severe disabilities in the community compared to caring for children without disabilities, a diary- and questionnaire-based study was carried out. Sixteen complete data sets were obtained from families with children who have disabilities (mean age 8.7 years) and 31 complete data sets from families with normally developing children (mean age 4.9 years). Diagnoses in the study group included cerebral palsy, autism, Sanfillipo syndrome, lissencephaly, and osteogenesis imperfecta. Items of personal care per waking hour were significantly greater in children with disabilities than non-disabled children (p<0.001). In the study group, there was no correlation (r=–0.12) between age and frequency of care whereas a significant correlation was observed between degree of disability as measured by the Functional Independence Measure for children (WeeFIM) and frequency of care items (r=0.89). Twelve of the 16 mothers in the study group were not in paid employment. Twelve had little or no extended family support. Benefits awarded did not correlate with the degree of disability as measured by the WeeFIM (r=–0.11). Care needs of children with severe disabilities are significantly greater than those of non-disabled children and do not decrease with advancing age. Mothers of children with disabilities are unable to work outside the home because of these care needs. This brings the family income, even when benefits are included, to a level that is less than peer families with non-disabled children. A Functional Disability Score may help to achieve more appropriate allocation of state resources.

Boys with Asperger syndrome (n=20), attention-deficit–hyperactivity disorder (n=20), and reading and writing disorder (n=20) were followed up and retested on several neuropsychological measures 1 to 2 years after initial assessments. Wechsler Intelligence Scale for Children (WISC-III) Full Scale, Verbal, and Performance IQ scores remained stable for all diagnostic groups. Kaufman factors and ‘fluid’ and ‘crystallized’ abilities were also stable measures. Subtest stability over time, was slightly more variable. There was a tendency for the group with Asperger syndrome to deteriorate over time with respect to logical reasoning abilities. Measures of executive function/attention (‘go-no-go’ and ‘conflict’ tests) showed good test–retest stability in all diagnostic groups. This is the first study of its kind.

The relationship of preterm birth to health-related quality of life (HRQoL) was examined for children aged 1 to 4 years. Three gestational age groups with a NICU history were selected, <32 weeks (n=65), 32 to 36 weeks (n=41), [ges ]37 weeks (n=54), and a reference group from the open population (n=50). The main instrument was the TNO-AZL Preschool Quality Of Life (TAPQOL) questionnaire, which was completed by the parents. In addition, other outcome measures obtained from parents or neonatologists were investigated. Children born <32 weeks had significantly lower HRQoL than the reference group in the scales for lungs, stomach, eating disorders, motor functioning, communication, and anxiety. Parental feelings towards the child were related to the child's HRQoL. We found differences between the neonatologists' and parents' perceptions of the children's situation, which can have clinical consequences (e.g. different opinions about what needed treatment). Neonatal intensive care after birth has HRQoL implications for all children, particularly in children born at <32 weeks of gestation.

It has recently been suggested that lycra garments are helpful for children with cerebral palsy (CP). Twelve children, with athetosis, ataxia, and spasticity, were fitted with lycra garments (Kendall-Camp UK Ltd). Scores on the Paediatric Evaluation of Disability Inventory (PEDI) scales were determined before and after wearing the garment for at least 6 hours a day for 6 weeks. Five children with motor problems representative of the whole group were investigated during a reach-and-grasp task by kinematic motion analysis; reflective markers were used with and without the garment. Carers were given a questionnaire concerning the practicalities of using the garments. All 12 children made improvements in at least one of the functional scales of the PEDI, and scores for the whole group showed significant gains (Wilcoxon χ2 test, self-help p<0.01; mobility p<0.5; social p<0.1). These changes were usually slight, although noticed by carers. Six children made gains of at least one scale of the caregiver assistance scores, two of the children showed losses (due to difficulties removing the garment for toileting), and four showed no change. Motion analysis indicated that (1) two children with athetosis had improved proximal stability in sitting and in smoothness of arm movements, (2) one child with ataxia had improved in proximal and distal stability, and (3) two children with spasticity had more jerky movements, although one improved in proximal stability. All children had problems in wearing the garments, including problems with toileting and incontinence of urine; the parents of only one child wanted to continue using it. Results suggest that the functional benefit of lycra garments for children with CP is mainly due to improvements in proximal stability but this should be weighed against the inconvenience and loss of independence.

Development of children aged 5 to 12 years born to mothers with heroin dependency raised at home or adopted was studied in comparison with: (1) children with environmental deprivation alone (i.e. low parental socioeconomic status [SES] and evidence of neglect), (2) children born to fathers with heroin dependency fathers, and (3) control individuals of average SES. One hundred and sixty children (84 males and 76 females; average age at examination 8 years) were evaluated between 1998 and 1999. All were attending mainstream schools. All participants were examined by a paediatrician and a psychologist using standard neurological and psychological age-appropriate tests, as well as tests and questionnaires to assess learning ability and attention span. The Conners and Achenbach questionnaires and the Pollack Taper test were used to assess possible presence of attention-deficit–hyperactivity disorder (ADHD). Mothers were assessed for ADHD using Wender's questionnaire. Children born to parents with heroin dependency raised at home and those of low SES exhibited intellectual impairment both on verbal and performance skills. They also had impaired reading and arithmetic skills. Children born to mothers with heroin dependency but who were adopted at a young age had normal intellectual and learning abilities, except for some reduced function on the performance Wechsler Intelligence Scale for Children–Revised. We found a high rate of ADHD among all children born to parents with heroin dependency, including those adopted, as well as in children with low parental SES. The highest rate of ADHD was in children born to mothers with heroin dependency raised at home, being twice that observed in the other groups. Mothers of these groups of children also had a high rate of ADHD.

The aim of this study was to evaluate the effect of therapeutic electrical stimulation (TES) applied to antagonists of spastic leg muscles on gross motor function in children with spastic diplegic cerebral palsy. Twelve children between 5 and 12 years of age completed a 24-month crossover study in which six were randomly assigned to receive TES for the first 12 months and the remaining six for the last 12 months. Physiotherapy and a home training program were not altered. All were evaluated blindly in terms of tests of motor function and video recordings at the start and at 12 and 24 months. At the end of the study parents/carers gave a subjective assessment of the effect of TES. No significant effect of TES on motor or ambulatory function was found on the blinded evaluation, but parents of 11 of the 12 children stated that TES had a significant effect. We conclude that it is unlikely that TES has a significant effect on motor and ambulatory function in spastic diplegia.