A consecutive series of 53 children with myelomeningocele (mean age 7.6, range 3.2 to 11.4 years) was assessed in order to see if the children with motor paresis of the lower limbs achieved the expected level of ambulation, and if not, to identify possible causative factors. Methods used were clinical examination of orthopaedic and neurological status, information from medical reports, and documentation of orthoses use. Functional skills were documented and energy expenditure was examined. Thirty-one of 53 children had reached the expected ambulation considered possible according to their motor paresis, whereas 22 of the 53 performed worse than expected. Balance disturbances, occurrence of spasticity in knee and hip joints, and number of shunt revisions made differed significantly between the groups that achieved and did not achieve expected ambulation. Functional skills of mobility differed significantly between two muscle-function levels in children who had walking ability. Energy expenditure was higher in the non-achieving group than in the group who achieved expected ambulation in each of the muscle-function levels. Results show that children with similar muscle paresis exhibit different ambulatory function. This indicates the importance of a close analysis of other factors which may cause ambulation to deteriorate in order to predict future ambulation in children with myelomeningocele.

The aim of this study was to determine the reliability of family and professional assessment of manual ability using the Manual Ability Classification System (MACS) for children with cerebral palsy (CP) in the UK. Families who were taking part in a study measuring the activities and participation of children with CP were invited to classify their child's manual ability using the MACS. Postal surveys were conducted with the families and health professionals nominated by the families. Perfect agreement was assessed as a percentage; chance-corrected agreement was measured using Cohen's kappa (κ), and reliability was determined using the intraclass correlation coefficient (ICC). Families of 91/128 (71%) children responded to the survey (53 males, 38 females; mean age 9y 11mo [SD 1y 11mo], range 6–12y) out of whom 88 indicated a single MACS level. Seventy-two children (82%) were classified with spastic CP, 12 (14%) with dyskinesia, two (2%) with ataxia, and two (2%) were not classified. There were 21, 27, 11, 10, and 19 children who were classified by their families in Gross Motor Function Classification System Levels I to V respectively; 14, 30, 18, 13, and 13 children classified by their families in MACS levels I to V. The survey of health professionals generated 60/71 (85%) responses from physiotherapists, 55/58 (93%) responses from paediatricians, and 21/24 (88%) responses from occupational therapists. There was perfect agreement between families and professionals for more than 50% of children; the indices of chance-corrected agreement ranged from κ=0.3 to 0.5, and the reliability coefficients ranged from ICC 0.7 to 0.9. Indices of agreement and reliability between families and professionals were equivalent to those between different professionals. The MACS, therefore, offers a valid and reliable method for communicating about the manual ability of children with CP. Families and professionals may not always agree precisely on a MACS level, particularly if children's performance of manual tasks varies in different environments.

Participation and activity performance (motor and cognitive or behavioural) were examined in 148 children with cerebral palsy (CP; 87 males, 61 females; mean age 9y 8mo, SD 1y 11mo; range 6y 1mo to 13y 7mo), mainstreamed in fully inclusive (n=100) and in self-contained classes (n=48) within general schools in Israel, using the School Function Assessment. Differences in participation within these groups were analyzed in relation to the type of CP (mainly spastic hemiplegia, spastic diplegia, and spastic tetraplegia), the level of impairment according to the Gross Motor Function Classification System (GMFCS; level II 55%, level III 37%, and level IV 8%), and additional neuroimpairments. Univariate analyses of variance revealed significant differences in levels of participation and levels of activity performance between different types of CP and GMFCS levels. With regard to additional neuroimpairments, significant differences in participation were found for fully included children with speech and language impairments and those with learning disability within the self-contained group. Physical activity performance partly accounted for differences in participation between children with different types of CP and different levels of motor impairment. These findings suggest that within the mainstreamed environment, participation and activity performance increase as motor disability and/or additional neuroimpairments (speech and language impairments and learning disability) decrease.

This study used meta-analytical techniques to explore the association between intelligence and age in children with Duchenne muscular dystrophy (DMD). The sample comprised 1224 children and young adults with DMD (mean age 12y 3mo, SD 4y; range 2y to 27y). Standardized measures including the Wechsler Intelligence Scales (WIS) and the Stanford-Binet Intelligence Scales were used to estimate intelligence. No age-related difference was noted for Full-scale and Performance intelligence quotients (IQ). However, Verbal IQ increased significantly with age. Age-related increases were noted for the WIS Information, Similarities, Arithmetic, Comprehension, Digit Span, Picture Arrangement, Block Design, and Coding subscales. These results support the notion that younger children with DMD have deficits in verbal reasoning and verbal processing. Older children with DMD, particularly those of 14 years and older, were less likely to present with these problems. Implications of these findings and possible future research directions are discussed.

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.

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.

Strength, power, and coactivation levels were measured during knee extension and flexion tasks in 20 children (13 males, 7 females) with developmental coordination disorder (DCD) and 20 age-matched control children (12 males, 8 females). Participants were classified according to results obtained on the McCarron Assessment of Neuromuscular Development, with each group including two age levels: 6 to 7 and 9 to 10 years. Tasks were performed on a Biodex dynamometer under isometric and isokinetic conditions with surface EMG recorded from the vastus lateralis and biceps femoris muscles. Significant effects of group and age were observed for the measures of peak torque (isometric and isokinetic) and power, with the decreased power in the group with DCD being more apparent at higher velocities. A lower flexor–extensor percentage was recorded for the group with DCD but only under isokinetic conditions. From a neuromuscular perspective, the group with DCD had increased levels of coactivation (RMS agonist:RMS antagonist) during the isometric flexion and isokinetic extension actions. Possible causes of the strength and power deficits observed in children with DCD are discussed.

Mirror movements in individuals with hemiplegic cerebral palsy (CP) may result from a reorganization of the central sensorimotor system. Motor performances of both hands were measured to characterize mirror activity (or mirroring) and hand functions in 22 participants (6 to 18 years) with hemiplegic CP and in 17 control participants. During a unimanual repetitive squeezing task, contractions of the active hand and fingertip forces of the opposite hand were recorded simultaneously. In the control group, slight mirror activity (or mirroring) was found that decreased with age. In participants with CP, mirror activity was 15 times stronger than in the control group, and was found at all age levels. Mirroring was more prominent in the unaffected hand of the CP group. The amount of mirror activity was not related to the degree of hemiplegia, which was assessed with measures of spasticity, strength, and dexterity. Mirror movements disturbed functional bimanual skills, although to some extent they could be suppressed by voluntary effort.

the aim of this study was to establish the rate and spectrum of psychiatric disorder among children before and after temporal lobe surgery for epilepsy. data were examined for associations between psychopathology and seizure outcome following surgery, or association between psychopathology and other variables, such as laterality of lesion, sex, cognitive level, and underlying pathology. participants were 60 children (35 males, 25 females) who had focal seizures of temporal lobe origin and who had undergone temporal lobe resection between 1992 and 1998; mean age at time of operation 10y 7mo, (sd 4y 11mo) range 7mo to 17y 11mo. mean length of follow-up was 5.1 years (sd 2.3, range 2 to 10y). twenty-eight (47%) children had undergone right temporal lobectomy. diagnosis of a psychiatric disorder was present in 50/60 (83%) children at some point, with high rates of psychiatric comorbidity. common childhood psychiatric disorders of attention-deficit–hyperactivity disorder, oppositional defiant disorder/conduct disorder, and emotional disorders were present in about 25% of children. disorders rarely seen in the general child population were over-represented: disruptive behaviour disorder – not otherwise specified (30/60 [50%]), and pervasive developmental disorder (autistic spectrum disorder; 23/60 [38%]). there was no significant relationship between pathology, sex, seizure frequency, or postoperative seizure outcome and psychiatric disorder, other than for pervasive developmental disorder. the same proportion of children had psychiatric diagnoses pre- and postoperatively (43/60 [72%] and 41/57 [72%] respectively). although mental health problems are common in children undergoing temporal lobe resection, there are few predictors of psychiatric outcome following epilepsy surgery. parents require counselling on these issues in the preoperative work-up.

Sixty-nine very-low-birthweight infants out of a population of 923 had cerebral palsy (CP) at an 18-month follow-up. Thirty-nine of these had cranial ultrasound abnormalities in the neonatal period and 30 had normal cranial ultrasounds. The distribution of subtypes of CP differed markedly between the two groups, with hemiplegia predominating in those with abnormal cranial ultrasounds and diplegia in those with normal cranial ultrasounds. Regardless of ultrasound appearance, the relative risk of CP increased approximately fourfold with a neonatal history of sepsis.

Independent mobility in early childhood has been associated with the development of various cognitive and psychosocial skills. However, children with physical disabilities are not always able to move independently and may be at risk for delays in these areas. Early provision of powered mobility can offer young children an opportunity for independent mobility. Despite this, there is little information to help determine when a young child has the cognitive skills necessary to operate a powered wheelchair safely. This current research aims to identify these skills. A cognitive assessment battery and a wheelchair mobility training and assessment program were developed. Twenty-six children with physical disabilities between the ages of 20 and 36 months were evaluated on the cognitive assessment and participated in the wheelchair training and assessment program. A stepwise regression analysis was used to determine which of the cognitive skills predicted wheelchair mobility performance. The cognitive domains of spatial relations and problem solving were found to be significant and accounted for 57% of the variance in wheelchair skills. Developmental cut-off points on these scales as they relate to wheelchair skills are presented and clinical applications are discussed.

This study explored possible neural mechanisms that contribute to improvements in balance control produced by reactive balance training in children with cerebral palsy (CP). Six children with CP (four males, two females; mean age 9y 4mo), two with spastic hemiplegia (Gross Motor Function Classification System [GMFCS] level I) and four with spastic diplegia (GMFCS level II,) were given 5 days of intensive training in reactive balance control (100 perturbations per day on a moveable force platform). Surface electromyography was used to characterize changes in neuromuscular responses pretraining, immediately posttraining, and 1 month posttraining. Training in reactive balance control resulted in improvements in directional specificity of responses (a basic level of response organization) and other spatial/temporal characteristics including: (1) faster activation of muscle contraction after training, allowing children to recover stability faster; (2) emergence of a distal–proximal muscle sequence; and (3) improved ability to modulate the amplitude of muscle activity (increased amplitude of agonist and decreased amplitude of antagonist, reducing coactivation). Each child with spastic hemiplegia or diplegia showed a different combination of factors that contributed to improved performance; the level of change in neural factors depended on the severity of involvement of the child: hemiplegia vs diplegia, and level of involvement within each diagnostic category.

Pyridoxine dependency was one of the first genetically mediated seizure disorders to be recognized. However, the biochemical abnormality and gene locus are still unknown. Knowledge about the condition has come from case reports and small clinical series. However, new features are still being recognized both clinically, as well as more predictably, from the application of new imaging techniques. Early onset seizures responsive to pyridoxine exist which do not appear to be the same disorder. In some parts of the world, high-dose pyridoxine is used in the treatment of other major seizure disorders of young children such as West syndrome.

The concurrent validity of a 1 minute walk test at a child's maximum walking speed was assessed in children with bilateral spastic cerebral palsy (BSCP). The distance covered during the 1 minute walk test was compared with the children's gross motor function as assessed by the Gross Motor Function Measure (GMFM). Twenty-four male and 10 female children with CP (mean age 11y, range 4 to 16y) participated in the study. Gross Motor Function Classification System (GMFCS) levels were; level I (n=3), level II (n=17), level III (n=10), and level IV (n=4). Participants had clinical diagnoses of symmetrical diplegia (n=19), asymmetrical diplegia (n=14), and quadriplegia (n=1). Results showed a significant correlation between GMFM score and the distance covered during the 1 minute walk (r=0.92; p<0.001). There was also a significant decrease in the distance walked with increasing GMFCS level (p<0.001). We concluded that the 1 minute walk test is a valid measure for assessing functional ability in children with ambulatory BSCP. Its cost-effectiveness and user friendliness make it a potentially useful tool in the clinical setting. Further study needs to address its reliability and ability to detect change over time.

Children with cerebral palsy (CP) are functionally limited to varying degrees because of their decreased central control and coordination of their movements. The effects of growth predispose children with neurological impairments to the secondary problems of muscle contractures, bony deformities, and unusual gait abnormalities. Health care programmes aim to prevent deformities and encourage the development of functional and independent skills and abilities. Orthoses continue to have an important role in many therapeutic regimens for children with CP, however, there have been differences in designs of orthoses prescribed for the common problem of spastic equinus. This is because different treatment paradigms have evolved which advocate different designs of ankle–foot orthoses (AFOs).

A Mac Keith Multidisciplinary Meeting formulated the following consensus statement concerning postural management for children with cerebral palsy (CP) based on evidence from clinical experience and scientific literature:

Definition: A postural management programme is a planned approach encompassing all activities and interventions which impact on an individual's posture and function. Programmes are tailored specifically for each child and may include special seating, night-time support, standing supports, active exercise, orthotics, surgical interventions, and individual therapy sessions.

We have considered multiple subpial transection (MST) as a treatment option for Landau–Kleffner syndrome (LKS) for the past 6 years. The effect of this technique on language and cognitive ability, behaviour, seizures, and EEG abnormalities is analysed here. Five children (4 males, 1 female; aged 5.5 to 10 years) underwent MST with sufficiently detailed pre- and postoperative data for analysis. Behaviour and seizure frequency improved dramatically after surgery in all children. Improvement in language also occurred in all children, although none improved to an age-appropriate level. All five had electrical status epilepticus in sleep (ESES) before surgery, which was eliminated by the procedure. One child has had an extension of his MST due to the recurrence of ESES and accompanying clinical deterioration with good effect. An attempt is made to set the effect of MST against the natural history of the condition. MST is an important treatment modality in LKS, although the timing of this intervention and its effect on final language outcome remains to be defined.

There has been little exploration of major biologic regulators of cerebral development in autism. We measured insulin-like growth factors (IGF) -1 and -2 from cerebrospinal fluid (CSF) by radio immunoassay in 25 children with autism (median age 5y 5mo; range 1y 11mo–15y 10mo; 20 males, 5 females), and in 16 age-matched comparison children without disability (median age 7y 4mo; range 1y 1mo–15y 2mo; eight males, eight females). IGF-1 and -2 concentrations were further correlated with age of patients and head size. CSF IGF-1 concentration was significantly lower in patients with autism than in the comparison group. The CSF concentrations of children with autism under 5 years of age were significantly lower than their age-matched comparisons. The head circumferences correlated with CSF IGF-1 in children with autism but no such correlation was found in the comparison group. There was no difference between the two groups in CSF IGF-2 concentrations. No patients with autism had macrocephaly. We conclude that low concentrations of CSF IGF-1 at an early age might be linked with the pathogenesis in autism because IGF-1 is important for the survival of Purkinje cells of the cerebellum. The head growth might be explained by the actions of IGF-1 and -2 reflected in CSF concentrations.

Event-related functional magnetic resonance imaging (fMRI) was used to investigate the hypothesis that males who were born very preterm may show differences in relative strength of blood oxygen level dependent (BOLD) signals in selective brain areas during performance of a simple response inhibition task compared with term-born controls. Participants were eight males (mean gestational age at birth 28wks, [SD 2]; mean age at testing 16y, [SD 1] and 14 controls matched for sex, age (mean age 17y, [SD 1]), and IQ. A ‘go-no-go’ task was used to assess response selection and motor response inhibition in response to a visual stimulus. When the ‘no-go’ condition was contrasted with an attentional control condition, preterm participants showed reduced BOLD signal response bilaterally in the cerebellum, right caudate nucleus, and thalamus, and prefrontal areas including left inferior prefrontal and left anterior cingulate gyri. They also showed increased response mainly in temporal regions. These results suggest that despite good task performance, individuals who were born very preterm exhibit different BOLD signal responses in selective brain areas compared with controls which may underline the use of alternative strategies when challenged with motor response inhibition processing.

This study aims to evaluate the use of the United States National Center for Health Statistics (NCHS) weight-for-height centiles (WHC) in screening children with cerebral palsy (CP) for depleted body fat and to identify an alternate screening method. Growth data from 276 children aged from 3 to 12 years with CP were analyzed retrospectively. Height or a proxy for height, mid-upper arm circumference, weight, and skinfold thicknesses were recorded. Mid-upper arm fat area was calculated for each participant. The sensitivities and specificities of WHC and a number of alternative anthropometric screening methods for identifying participants with severely depleted fat stores were determined. WHC <10th centile failed to identify 45% of children with severely depleted fat stores. Triceps skinfold thickness <10th centile identified 96% of malnourished children. WHC standards lack adequate sensitivity for identification of severely depleted fat stores in children with CP. Use of triceps skinfold thickness, using cut-off value of <10th centile for age and sex, is recommended to screen for suboptimal fat stores in children with CP.