Between 1987 and 1998, 470 patients visited the national clinic for obstetrical brachial plexus palsies at the Karolinska Hospital, Stockholm, Sweden. This study reports on 247 children examined at 5 years of age using a special protocol for testing the sensory and motor function of the hand. The children were distributed in various groups depending on the number of injured nerves, whether they had some muscle activity in their biceps or deltoid muscles at 3 months of age, and whether any operations with nerve reconstruction had been performed. The shoulder range of movement in C5–6 palsies was significantly better in the group which had been operated on (operated group), but otherwise there were no differences between children who had been operated on (non-operated group) and those who had not. A decrease in grip strength and bimanual function in level C5–6 palsies was found, though these roots should not innervate the distal hand. Outcome was not influenced by the number of avulsions in upper-plexus palsies or whether the operation took place before or after the age of 6 months. The group with extensive lesions (C5–Th1) had the most root avulsions, showing a correlation between increased avulsions and decreased hand function. This study does not support operating on children with no activity of the biceps and deltoid muscles at 3 months of age, as other authors have concluded. Rather, it favours waiting for a late recovery.

Little is known about long-term physical sequelae, cognitive functioning, and quality of life in children who have experienced ischemic stroke. Thirty-seven patients under 16 years of age were studied; the median interval after stroke was 7 years. CT-scans were reassessed to determine the type of infarction at baseline. Occurrences of death, of new cardiovascular events, and of seizures during follow-up were recorded. Surviving patients were invited for a follow-up examination, including physical check-up, global screening of cognition, and an inventory of subjective health perception. Only two patients were lost to follow-up. During follow-up four died, nine developed seizures, eight had transient ischemic attacks, and two experienced a recurrent ischemic stroke. None of the patients had cardiac complications during follow-up. In 11 of 27, no functional impairment was found, in 15 there was a hemiparesis of varying severity, and in one a paraplegia. There was a significant shift in cognitive functioning towards lower levels, especially in children with epilepsy. Remedial teaching was frequently needed. Many of the parents perceived their child's behavior to be very changeable. Three-quarters of the children considered themselves as healthy as other children, and almost all of them as happy. The physical and functional prognosis after ischemic stroke in childhood is relatively good, particularly in children with no serious causative illness, but special education is often needed and social changes occur.

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.

spasticity, a neurological problem secondary to an upper motor neuron lesion, has a significant effect on skeletal muscle. the upper motor neuron lesions may be secondary to a cerebral vascular accident, head injury, spinal cord injury, or degenerative diseases such as multiple sclerosis, or perinatal brain injuries such as cerebral palsy. functional ability in these patients can be severely compromised but the basic mechanisms underlying these deficits are not clearly understood. in this review we evaluate the current evidence in the literature that suggests that skeletal muscle tissue itself is altered in spastic conditions. experimental studies were evaluated that included a variety of methods encompassing joint mechanics, tissue mechanics, and muscle morphology. taken together, the literature strongly supports the assertion that ‘spastic muscles’ are altered in a way that is unique among muscle plasticity models and inconsistent with simple transformation due to chronic stimulation or disuse. further studies are required to detail the intra- and extracellular modifications of skeletal muscle that occur secondary to spasticity so that novel therapeutic treatments can be developed for this impairment.

The objective was to determine the effects of low-dose, high-concentration, dual localized botulinum toxin A (BTX-A) injections on upper limb movement quality and function. Study design was an evaluator-blinded, randomized, controlled trial. Forty-two children (31 males, 11 females; range 2–8y, mean 4y [SD 1.6]) with hemiplegic cerebral palsy (Gross Motor Function Classification System level I) participated. All received occupational therapy. The treatment group (n=21) received one injection series (mean muscles injected 6 [SD 1.05]; total dose 82–220 units, mean 139 [SD 37.48]; dilution 100 units/0.5ml). Primary outcome of Quality of Upper Extremity Skills Test (QUEST) at 6 months was not significant (p=0.318). Secondary outcomes were average treatment effects at 1, 3, and 6 months, which favoured the treatment group: QUEST (p<0.001); Canadian Occupational Performance Measure (performance, p=0.002; satisfaction p=0.007); parent Goal Attainment Scaling (GAS; p=0.001), therapist GAS (p<0.001); Pediatric Evaluation of Disability Inventory (PEDI) functional skills (p=0.030); Ashworth (p<0.001). PEDI caregiver assistance was not significant (p=0.140). Therapy alone is effective, but at 1 and 3 months movement quality is better where BTX-A is also used. Moreover, function is better at 1, 3, and 6 months, suggesting BTX-A enhances therapy outcomes beyond the pharmacological effect. One- and 3-month Ashworth and QUEST scores suggest precise needle placement accuracy.

Review of the definition of cerebral palsy (CP) has focused on disorders of movement and posture development that cause activity limitations. Recent research provides new insights into CP, showing that activity limitation in individuals with hemiplegic CP is not exclusively caused by disorders related to movement execution, but is also related to the planning of movements. In this review article, converging evidence is presented suggesting that, in addition to movement execution impairments, impairments in movement planning may also limit the performance of activities of daily living. Evidence from studies of fingertip-force planning and anticipatory planning in object manipulation tasks is discussed.

The aim of this study was to assess the effect of early provision ([les ] 8 years) of a powered wheelchair (PWC) in children with tetraplegia. Twenty-nine children (15 males, 14 females; mean age 6 years 3 months, age range 3 to 8 years) with spastic or dystonic tetraplegia were studied. All participants had severe motor impairment. Treatment outcomes were investigated in several dimensions of disablement: Impairment, Functional Limitation/Activity, Disability/Participation. It was found that the level of independence improved significantly after PWC provision, while motor impairment, IQ, and quality of life did not. The majority of children (21 of 27) reached a level of driving competence which allowed them to move around with or without minimal (i.e. verbal) adult support. Achievement of this competence was not statistically related to IQ or motor impairment but correlated to the time spent in the PWC. The majority of parents (21 of 25) were not in favour of the PWC when the study started but after PWC provision, 23 of 25 parents expressed positive feelings about it. Reactions of the majority of children (23 of 25) were positive from the beginning of the study and did not change over time. The authors concluded that PWCs can aid independence and socialization and the majority of children can achieve a good-enough driving competence, even those with severe learning disability or motor deficit. PWCs should not be viewed as a last resort but as a means of providing efficient self-locomotion in children with a severe motor deficit.

Previous studies of grasping and object manipulation in children with cerebral palsy (CP) have suggested a dichotomy in the ability to use anticipatory control (planning) of the fingertip force output, depending on the type of sensory information (tactile or proprioceptive) on which it is based. The present study further explores this issue by testing the ability of 15 children with hemiplegic CP aged between 8 and 14 years to scale the fingertip force output in advance during the lifting of small objects whose weight and surface texture are varied. The results indicate that children with hemiplegia can use anticipatory control based on both the weight and texture of the object, but require a greater number of trials than age-matched children without CP (control children) before they can do so. We suggest that the initial lack of anticipatory control results from an indistinct internal representation of the object's physical properties due to disturbed sensory mechanisms, which may have direct implications for therapeutic intervention.

We present a prospective study at school age of neuropsychiatric and neurodevelopmental outcome of language delay suspected at child health screening around 30 months of age. In a community sample, 25 children (21 males, 4 females) screening positive and 80 children (38 males, 42 females) screening negative for speech and language problems at age 30 months were examined in detail for language disorders at age 6 years. The screen-positive children were then followed for another year and underwent in-depth neuropsychiatric examination by assessors blind to the results of previous testing. Detailed follow-up results at age 7 years were available for 21 children. Thirteen of these 21 children (62%) had a major neuropsychiatric diagnosis (autism, atypical autism, Asperger's syndrome, attention-deficit–hyperactivity disorder [ADHD]), or combinations of these. Two further children (10%) had borderline IQ with no other major diagnosis. We conclude that children in the general population who screen positive for speech and language problems before age 3 years appear to be at very high risk of autism spectrum disorders or ADHD, or both, at 7 years of age. Remaining language problems at age 6 years strongly predict the presence of neuropsychiatric or neurodevelopmental disorders at age 7 years.

This study aimed to identify the sexual and reproductive health knowledge, attitudes, and behaviour of young people, aged between 14 and 23 years, with spina bifida who had attended the Spina Bifida Clinic at a tertiary centre in Melbourne, Australia within the past 10 years. Subjects participated in a structured interview and their parents completed a written questionnaire. Fifty-one (55%) of 93 eligible young people and 69 of 97 parents (71%) participated. Most young people were satisfied with the amount of general sex education they had received. However, 95% stated they had inadequate knowledge about sexual and reproductive health relating to spina bifida and 59% of parents considered they had inadequate knowledge. Thirty-nine percent of young people and 30% of parents had discussed sexuality issues with a doctor. However, 93% of young people and 100% of parents said that they would definitely talk about these issues if the discussions were initiated by their doctor. A significant degree of sexual intimacy was reported, with 60% reporting an intimate relationship, and 25% (10 females, three males) reporting sexual intercourse. Thirty-seven percent of females had experienced unwanted sexual attention, and 30% reported unwanted sexual touching. This study suggests that health professionals should pay greater attention to sexual and reproductive health issues in young people with spina bifida.

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?

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.

Autism has been described in association with a variety of medical and genetic conditions. We previously reported on a patient whose clinical phenotype was compatible with both fetal valproate syndrome (FVS) and autism. Here we present five additional patients with FVS and autism. In all five of our patients, there was evidence of cognitive deficits, manifestations of autism, and typical phenotypic characteristics of FVS. The association between this known teratogen and autism has both clinical and research implications.

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.

This report describes trends, predictors, and causes of mortality in persons with cerebral palsy (CP) using individuals identified by the Western Australian Cerebral Palsy Register and born between 1958 and 1994. Two thousand and fourteen people were identified (1154 males, 860 females), of whom 225 had died by 1 June 1997. Using date-of-death data, crude and standardized mortality rates were estimated and predictors of mortality sought using survival analysis stratified by decade of birth, description of impairments, and demographic and perinatal variables. For those born after 1967, the cause of death profile was examined over time. Mortality exceeded 1% per annum in the first 5 years and declined to age 15 years after which it remained steady at about 0.35% for the next 20 years. The strongest single predictor was intellectual disability, but all forms of disability contributed to decreased life expectancy. Half of those with IQ/DQ score <20 survived to adulthood, increasing to 76% with IQ/DQ score 20-34, and exceeding 92% for higher scores. Severe motor impairment primarily increased the risk of early mortality. Despite there being 72 persons aged from 25 to 41 years with severe motor impairment in our data set, none had died after the age of 25 years. Infants born after more than 32 weeks' gestation were at significantly higher risk of mortality than very preterm infants, accounted for by their higher rates of intellectual disability. No improvements in survival of persons with CP were seen over the study period despite advances in medical care, improved community awareness, and the increasing proportion of very preterm births among people with CP. This may be the result of improved neonatal care enabling the survival of infants with increasingly severe disabilities.

Assessment of the quality of general movements (GMs) in early infancy is a powerful instrument to predict cerebral palsy (CP). The aim of the present study is to explore the value of GM assessment in predicting minor neurological dysfunction (MND) at 9 to 12 years of age. Two groups of infants were studied prospectively: 28 low-risk full-term infants (11 females, 17 males) and 24 high-risk infants, mostly born preterm (<37 weeks; 11 females, 13 males). In each group the quality of GMs (normal or abnormal) was assessed during two developmental periods: the age at which ‘writhing’ GMs occur (36 weeks' postmenstrual age to 7 weeks' postterm) and the age at which ‘fidgety’ GMs occur (8 to 17 weeks' postterm). Eight of 24 high-risk infants were diagnosed as having CP at 4 to 9 years of age. The remaining 44 children were followed-up at 9 to 12 years. In children without CP, quality of GMs at ‘fidgety age’ was related to neurological condition (normal, simple MND, complex MND) at follow-up (rho=0.46, p<0.01). Abnormal GMs at ‘fidgety-GM age’ showed a specific relationship to the development of coordination problems (χ2=6.1, p=0.01) and fine manipulative disability (Fisher, p<0.05) at 9 to 12 years. This finding supports the notion that the quality of GMs may provide information on the integrity of complex supraspinal circuitries.

This review aimed to synthesize current research evidence to determine the effectiveness of conservative interventions for infants with positional plagiocephaly. A systematic review was conducted, where papers were sourced from 13 library and internet databases. Research was included if published in English between 1983 and 2003. Level of evidence and quality of each paper was assessed to determine studies' magnitude of inherent bias. Results were synthesized in a narrative format and were considered with respect to homogeneity of participants, response rate, and outcome measures. Sixteen papers met inclusion criteria: 12 were case series and four were comparative studies. The methodological quality of the studies was moderate to poor, thus their results should be interpreted with caution. A consistent finding was that counterpositioning±physiotherapy or helmet therapy may reduce skull deformity; however, it was not possible to draw conclusions regarding the relative effectiveness of these interventions. Further investigation is required to compare the effect of helmet therapy with counterpositioning alone or when combined with physiotherapy. First, there is a need to develop an outcome measurement battery which incorporates psychometrically-sound measures from the perspectives of clinicians and patients.

Long-term outcomes of the ketogenic diet in the treatment of epilepsy have not previously been reported. A retrospective chart review of children treated with the ketogenic diet for more than 6 years at the Johns Hopkins Hospital was performed. The response was documented at clinic visits and by telephone contacts; laboratory studies were obtained approximately every 6 to 12 months. Satisfaction and tolerability were assessed by means of a brief parental telephone questionnaire. In all, 28 patients (15 males, 13 females), currently aged 7 to 23 years, were identified. The median baseline seizure frequency per week at diet onset was 630 (range 1–1400). Diet duration ranged from 6 to 12 years; 19 remain on the diet currently. After 6 years or more, 24 children experienced a more than 90% decrease in seizures, and 22 parents reported satisfaction with the diet's efficacy. Ten children were at less than the 10th centile for height at diet initiation; this number increased to 23 at the most recent follow-up (p=0.001). Kidney stones occurred in seven children and skeletal fractures in six. After 6 years or more the mean cholesterol level was 201mg/dl, high-density lipoprotein was 54mg/dl, low-density lipoprotein was 129mg/dl, and triglycerides were 97mg/dl. Efficacy and overall tolerability for children are maintained after prolonged use of the ketogenic diet. However, side effects, such as slowed growth, kidney stones, and fractures, should be monitored closely.

This study investigated the relation between sensory visual problems and the severity of visuospatial difficulties in a large group of young children with Williams' syndrome (WS). A questionnaire describing visual and associated problems was completed by the families of 108 children with WS and detailed follow-up assessments were conducted, including visual, spatial, motor, visuocognitive, and linguistic tests of 73 of these children (mean age 7 years 3 months; 40 males, 73 females). Children with WS showed a much higher incidence of common paediatric sensory vision problems (strabismus, visual acuity loss, amblyopia, reduced stereopsis) than normally developing children. It was found that delays with respect to age normative values increased with age on all tests. No significant correlation was found between the presence of a visual deficit and the severity of the visuospatial problems, suggesting that the difficulties children with WS have in understanding spatial arrangements are not simply a result of their earlier sensory visual problems. Results confirm the dissociation between visuospatial and language abilities in children with WS, and support the neurobiological model of a split between ventral and dorsal stream processing of visual information with a generalized deficit in dorsal stream processing in young children with WS.

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.