osteopenia is common in children, adolescents, and young adults with severe cerebral palsy (cp; spastic quadriplegia) living in residential care, and frequently results in atraumatic fractures. on clinical grounds 67 patients (34 males, 33 females) with severe cp (gross motor function classification system [gmfcs] levels iv or v) aged 5 to 25 years (median 20y) were divided into three groups with increasing likelihood of severe impairment of bone quality: (1) patients without fractures and without anticonvulsant medication (n=13); (2) patients without fractures and with anticonvulsant medication (n=45); (3) patients with fractures and with anticonvulsant medication (n=9). evaluation included measurements of quantitative ultrasound (qus) of the calcaneus, multiple serum analyses, and determination of urinary bone-resorption markers. values of the quantitative ultrasound index (qui) were significantly different (p=0.001): group 1 (median 56.9; interquartile range 43.8–75.3); group 2 (49.9; 40.0–60.0); group 3 (35.6; 30.5–38.5). when comparing values of laboratory serum and urine in the three groups, we found significant differences in values of serum bone alkaline phosphatase (p=0.001), serum parathyroid hormone (pth; p=0.002), serum albumin (p=0.020), and urinary deoxypyridinoline/creatinine ratio (p=0.004). in multiple regression analysis, no laboratory variable was found to be an independent predictor of qui. qus of the calcaneus may be a useful method to assess bone quality and fracture risk in children and young adults with severe cp living in residential care, independent of information from laboratory data.

This study assessed the impact of epilepsy on motor function in children. We aimed to analyze the effect in uncomplicated epilepsies (cryptogenic partial and idiopathic generalized epilepsy). A group of 87 children with epilepsy (47 males, 40 females; mean age 8y, standard deviation [SD] 1y 9mo, range 4y 11mo to 12y 11mo), but without learning disability or other neurological comorbid disorders, was compared with a control group of 107 children (76 males, 31 females; mean age 8y 4mo, SD 2y 2mo, range 4y 7mo to 12y 2mo). The differences in main motor skills and psychomotor speed were analyzed using the Movement ABC and computerized measures for simple reaction times and finger tapping. No significant difference in motor function was found. The overall psychomotor development of children with epilepsy is comparable to controls. However, a significant slowing of psychomotor speed in the group with epilepsy was reported. No relation with antiepileptic drug treatment was demonstrated and no difference in psychomotor speed between different types of epilepsy was found. The existence of a condition leading to excessive neuronal discharge leads to a general cortical inhibition. The slowing of psychomotor speed in children with uncomplicated epilepsy may be the behavioural presentation of this inhibition.

The aim of this study was to describe post-streptococcal movement disorders that form part of the acute rheumatic fever complex. The clinical records of patients diagnosed with Sydenham's chorea were analyzed retrospectively to investigate epidemiology, the significance of socioeconomic deprivation, clinical manifestations, treatments, outcomes, long-term morbidity, and disease evolution. Forty-two patients (21 males, 21 females) were diagnosed with Sydenham's chorea. The median presentation age was 9 years 8 months (range 3y 5mo to 13y 2mo). Nineteen patients were of indigenous African ancestry; 23 were of mixed ancestry. All patients lived in poverty and had poor access to medical care. Twelve of the total group had disabling symptoms for longer than 2 years; six of these patients developed paediatric autoimmune neuropsychiatric disorder associated with Streptococcus (Paediatric autoimmune neuropsychiatric disorder associated with Streptococcus [PANDAS]), five Tourette syndrome (TS), and one learning difficulties. Poor outcome was significantly more prevalent in patients of mixed ancestry, in those with a positive family history, previous behavioural problems, or a failure to complete 10 days of penicillin and ‘bed-rest’/hospitalization. Sydenham's chorea is one manifestation of post-streptococcal neuropsychiatric movement disorders. This study demonstrates that patients can present with one diagnosis and evolve other neuropsychiatric conditions such as TS and PANDAS. In the South African context, it is important to delineate neuropsychiatric movement disorders associated with streptococcal infections. The potential genetic susceptibility should be explored.

‘Auntie, speak to me! I'm frightened because it's dark’‘What good would that do? You can't see me’‘That doesn't matter. If anyone speaks it gets light’

Problems are painful, not only for what they are, manifest for all to see in the world beyond, but from how they are perceived within ourselves. A change in our perception of them can alter them in ways which may seem magical.

Feeding problems are common in children with neuromotor impairments. For example, a community-based survey by Reilly et al. demonstrated oral motor dysfunction in more than 90% of a sample of 49 children with cerebral palsy (CP). The sample contained 21 children with diplegia, 20 with quadriplegia, and six with hemiplegia. Most had either predominantly spastic (n=20) or mixed (n=22) signs. Thirty-three children were classified as having severe to profound functional impairment according to the Standard Recording of Central Motor Deficit. Over one-third of the sample had severe oromotor impairment and was at risk of chronic undernourishment. Although oral motor impairment was observed in all types of CP, moderate to severe oromotor dysfunction was more common in children with four limbs involved. In clinical populations, 60% of children with CP were found to have swallowing problems, with evidence of chronic aspiration in 41%. In assessing children with dysphagia, two issues are, therefore, paramount: is feeding safe, and is it adequate?

Children with cerebral palsy (CP) often have speech, language, and/or communication disorders and are referred to speech and language therapy (SLT). They may also be referred to SLT services for dysphagia: treatment of which is beyond the scope of this review. Although the exact prevalence of the communication disorders associated with CP is not known, it has been estimated that approximately 20% of children with a diagnosis of CP have severe communication impairment and are classified as non-verbal. Many more will have less severe speech and communication disorders as a consequence of their motor impairment, or disorders arising from speech, language, and cognitive processing deficits associated with CP.

We report on a 13-year-old male who had acute enteroviral encephalitis causing cerebellar symptoms at the age of 10 years. Magnetic resonance imaging (MRI) showed no abnormalities. Clinically he appeared to be recovered completely after 6 months. Twenty-three months after the recovery, MRI was performed because he presented with slight lower-limb and truncal ataxia experienced as lack of foot coordination while playing football or riding a bicycle. MRI demonstrated severe cerebellar atrophy. Clinically he recovered completely in 10 days. Only sophisticated electrophysiological methods revealed cerebellar dysfunction. The case provides evidence for the plasticity of cerebellar regulatory structures involved in the coordination of fine movements. It seems that in childhood the slow, isolated disintegration of cerebellar systems can be compensated for by upper thalamic or telencephalic connections, in a similar way to a congenital deficit of the cerebellum.

The relationships between quality of life (QoL), participation, and environment are being studied within a multicentre European study of 8- to 12-year-old children with cerebral palsy (CP; Study of PARticipation of Children with cerebral palsy Living in Europe [SPARCLE], www.ncl.ac.uk/sparcle/). The international group was determined that children with CP who also had intellectual impairment (IIm) should not be excluded from the study, but the group realized that there was little information available on how to assess subjective QoL in children with IIm. This annotation reviews and discusses the literature on conceptual and methodological issues in relation to such assessment.

Exercise for children with cerebral palsy (CP) is gaining popularity among pediatric physical therapists as an intervention choice. Exercise in water appeals to children with CP because of the unique quality of buoyancy of water that reduces joint loading and impact, and decreases the negative influences of poor balance and poor postural control. In this paper, research of land-based exercise and aquatic exercise for children with CP is reviewed. Clinically relevant considerations for aquatic exercise programming for children with CP are discussed.

We report on two sisters with a childhood-onset form of predominantly axial dystonia with marked diurnal fluctuations. Onset of clinical features was at approximately 6 years of age. Associated features included marked fatigue, slight facial dysmorphism, short stature, obesity, and learning disability. Dystonia and fatigue responded to 3,4-dihydroxyphenylalanine (DOPA) therapy, with recurrence of symptoms upon withdrawal; the efficacy has been maintained over 7 years. Other symptoms were not influenced. There was no other case in the family (which included an older, healthy brother), except for non-specific fatigue without dystonia in the mother, and there was no significant family history except for obesity on the father's side. These observations are discussed in relation to the classical descriptions of Segawa syndrome, and to more recent reports of childhood onset, age-related, and transient benign paroxysmal tonic upgaze and ataxia. The combination of symptoms, their sensitivity to DOPA, and their persistence throughout childhood constitute, to our knowledge, a new clinical entity, which we propose to categorize as a DOPA-sensitive dystonia-plus syndrome.

This study investigated the immediate effects of stimulant medication (methylphenidate) on cognitive attention processes in children with attention-deficit–hyperactivity disorder (ADHD). Thirteen males and two females (mean age 9y 5mo, SD 18.3mo) with a diagnosis of ADHD and who were to be prescribed methylphenidate were assessed twice on one day with the Test of Everyday Attention for Children, a neuropsychological battery designed to tap different aspects of cognitive attention. Between assessments, the children were administered methylphenidate (10mg). Each child had at least average intelligence (IQ 80 or over, as measured by the Wechsler Intelligence Scale for Children – III UK) and was on no other medication. A group of 16 children, who were matched for age, sex, and intelligence, also performed the cognitive tests twice on the same day to control for practice effects of testing. At the first assessment, children with ADHD demonstrated significant impairments in several aspects of cognitive attention in comparison with the control group, particularly sustained attention. After administration of methylphenidate for the children with ADHD, they showed significant improvements in their performance on measures of cognitive attention compared to controls. The immediate effects of methylphenidate and the significance of measuring cognitive aspects of attention as well as behavioural measures are discussed.

The aim of this pilot study was to evaluate functional training for children with cerebral palsy (CP). The outcomes examined were goal-achievement, gross motor function, performance of everyday activities, caregiver assistance, parents' perception of family-centredness, and preschool assistants' feeling of competence in the care of the children. The outcome measures used were goal attainment scaling, Gross Motor Function Measure, Pediatric Evaluation of Disability Inventory (functional skills and caregiver assistance scales), Measure of Processes of Care, and a questionnaire. The intervention was carried out in the context of daily life settings, i.e. in an ecological framework. Fourteen children (three females, 11 males; age range 1y 6mo to 6y) with diplegia and tetraplegia, classified at Gross Motor Function Classification System levels II–V, participated in the five-month intervention. Frequency of training towards a specific goal varied between twice a day and 23 times a day, depending on the type of goal. Seventy-seven per cent of goals were fully attained. Gross motor function and performance of daily activities, including social function, improved significantly. A decrease in caregiver assistance was noted in relation to the children's mobility. Parents' perception of family-centredness improved in all domains. Furthermore, the children's preschool assistants felt more competent in their care of the children.

We report a female infant noticed by her mother to have reduced movements of her right wrist and left foot at birth. This female presented to the Accident and Emergency Department of Queen Elizabeth Hospital, London, UK, aged 6 weeks, with significant weakness of her right wrist and left foot. Her clinical history also revealed sudden diminution in fetal movements 2 weeks before delivery, consistent with neuropathology in utero and neuromuscular deficit in the immediate postnatal period. An initial examination revealed generalized hypotonia, areflexia, and paucity of movements. Electrophysiological studies suggested demyelinating polyneuropathy; sural nerve biopsy confirmed a diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). With the results and the pre- and postnatal clinical history, we believe this to be the first reported individual with congenital CIDP confirmed in the neonatal period. We describe the treatment and outcome up to the age of six years.

In the UK, guidelines on the management of childhood epilepsy have just been published by the National Institute for Clinical Excellence (NICE). These, it is claimed, are evidence based. One definition of evidence-based medicine (EBM) is the conscientious, explicit, and judicious use of current best evidence in making decisions about the care of individual patients. Surely, no one could question that this is a good thing and that it should be the cornerstone of our clinical management of patients with epilepsy. The NICE guidelines contain almost 200 recommendations. In addition, NICE has previously published guidelines on the use of the newer antiepileptic drugs (AEDs) in children and the Cochrane database of systematic reviews includes many on the topic of epilepsy. If there is so much evidence around on which to base our clinical practice, why does it seem that most practitioners involved in the clinical care of children with epilepsy base most of their practice on case reports, anecdotes, and personal experience?

The aim of this study was to evaluate the clinical features and frequency of autistic disorder or Asperger syndrome (AS; according to Diagnostic and Statistical Manual of Mental Disorders, 4th edition [DSM-IV] criteria) in children exposed to anticonvulsant medication in utero. During a 20-year study period, 626 children were born in Aberdeen to mothers taking antiepileptic drugs (AEDs). The study examined long-term effects of prenatal exposure to AEDs in 260 children (122 males, 138 females). Of these, 26 (16 males) were reported by parents to have social or behavioural difficulties. Eleven children (6 males, 5 females) fulfilled the DSM-IV criteria for autistic disorder and one (female) fulfilled the DSM-IV criteria for AS. These children comprised 4.6% of the exposed children studied, and 1.9% of all exposed children born during the study period. Mean age of these children at diagnosis was 5 years 4 months (SD 2y 11mo) and 9 years 10 months (SD 3y 10mo) at the time of this study. Other children from the group of 26 had difficulties in areas of speech and language development and social communication but did not meet the criteria for an autism spectrum disorder (ASD). Sodium valproate was the drug most commonly associated with autistic disorder, five of 56 (8.9%) of the study children exposed to sodium valproate alone had either autistic disorder or AS. It was concluded that prenatal exposure to anticonvulsant medication is a risk factor for the development of an ASD. Fetal anticonvulsant syndrome associated autistic disorder is characterized by an even sex ratio, absence of regression or skill loss, and language delay in the absence of global delay.

The purpose of this study was to compare the cumulative efficacy (three treatment sessions) of botulinum toxin A (BTX-A) alone, casting alone, and the combination of BTX-A and casting in the management of dynamic equinus in ambulatory children with spastic cerebral palsy (CP). Thirty-nine children with spastic CP (mean age 5y 10mo, range 3 to 9y) were enrolled in the study. A multicenter, randomized, double blind, placebo-controlled prospective study was used. Children were randomly assigned to one of three treatment groups: BTX-A only (B), placebo injection plus casting (C), or BTX-A plus casting (B+C). The dosage for the BTX-A injections was 4U/kg per extremity. Assessments were performed at baseline, 3, 6, 7.5, and 12 months with a total of three treatments administered after the evaluations at baseline, 3, and 6 months. Primary outcome measures were ankle kinematics, velocity, and stride length. Secondary outcome measures were ankle spasticity, strength, range of motion, and ankle kinetics. Group B made no significant change in any variable at any time. Groups C and B+C demonstrated significant improvements in ankle kinematics, spasticity, passive range of motion, and dorsiflexor strength. Results of this 1-year study indicate that BTX-A alone provided no improvement in the parameters measured in this study, while casting and BTX-A/casting were effective in the short- and long-term management of dynamic equinus in children with spastic CP.

Children with brain injuries that are apparently similar in etiology, severity, and clinical management have striking differences in outcomes. There is increasing evidence that genetic factors may influence brain injury severity and outcome. Apolipoprotein E (apoE) is a lipid transport protein abundantly present in cells in the brain. In addition to transporting lipids, apoE has other functions necessary for cell maintenance and repair. The gene for apoE exists in three alleles (ε2, ε3, and ε4). Strittmatter and colleagues found APOE ε4 to be an important predisposing factor for the development of Alzheimer's disease. Subsequently, the ε4 allele was shown to convey vulnerability to worse outcome after traumatic brain injury and other neurological insults in adults.

Studying the increase in the rate of oxygen uptake (V[dot above]O2) during the transition from rest to exercise (V[dot above]O2 kinetics) provides important information about aerobic function during a metabolic state that mimics habitual activity far more closely than traditional steady-state exercise. The time constant of V[dot above]O2 (τ V[dot above]O2) provides a method of quantifying the V[dot above]O2 kinetic response in the non-steady state. It can also be used to quantify the O2 deficit, which provides a means of assessing the ‘strain’ on non-aerobic energy sources at exercise onset. Children with spastic cerebral palsy (CP) do not tolerate exercise well, and report early fatigue. Precise causalities are not known, although investigating V[dot above]O2 kinetics in this population has the potential to enhance our understanding of the underlying mechanisms. In addition, there are an increasing number of practitioners in the field of paediatric CP who use V[dot above]O2 measurements to evaluate the efficacy of interventions and rehabilitation regimens. Measuring V[dot above]O2 kinetics can complement the traditional cardiorespiratory outcome measures, providing much useful information about the combined function of the respiratory, circulatory, and metabolic systems. Importantly, this can be achieved by using submaximal exercise intensities, reducing the stress placed on participants. To our knowledge there are no published data describing the V[dot above]O2 kinetic responses of children with CP. This review seeks (1) to summarize the main findings from V[dot above]O2 kinetics research involving non-impaired adults and children so that measuring V[dot above]O2 kinetics in children with CP can be considered within an appropriate framework, and (2) to address methodological issues directly related to measuring kinetics in children with CP.

Throughout the centuries, in Western culture, scientists, philosophers, and artists have been fascinated by the working of the mind. They have attempted to translate this into visual models that are able to provide tangible references to otherwise abstract concepts. These metaphors are deeply rooted in culture and their significance encompasses ideas which shape and characterize different epochs. With a consideration of visual models of the mind developed in Western thought during the 16th and 18th centuries, this article aims to provide an overview of some of the most significant metaphors developed to illustrate the working of the mind and highlight their broader cultural significance. Generally, models used to describe the brain, and consequently to conceptualize the mind, match the most recent technical achievements or important cultural developments. They illustrate the processes through which material reality and the self are perceived and represented. This article shows how these models betray broader cultural concerns and testify to the zeitgeist of an epoch.