‘Normal’ comes from the latin norma, a carpenter's square, and in mediaeval times meant something at a right angle or perpendicular. A few centuries later it came to mean conforming to a standard and was used in physics to refer to the average of a set of variables. If the variable concerned, such as head size or IQ, is continuous and has a normal distribution, by convention the normal range has come to include values falling within two standard deviations either side of the mean – defining about 4.6% of the population as abnormal. By a similar approach, if the variable has a skewed distribution, such as the age of walking, those lying outside the 2nd and 98th centiles are often defined as abnormal, while the normal is equated to the average. This can also lead to misunderstandings, as, for example, in some child-rearing books when parents are told that the normal age of walking is 12 months, which can mistakenly imply that the 50% of children, who by definition are not walking at that age, are abnormal.

Professionals actively engaged in clinical trials involving patients with neurological damage must applaud researchers/clinicians who are willing to test popular clinical practices of equivocal or undetermined evidence. Kerr et al.'s study (p 870) is a major undertaking with sound scientific methodology. It is the fourth study published in DMCN in recent years that has failed to demonstrate the efficacy of neuromuscular electrical stimulation (NMES) in enhancing muscle force generation or improved functional ability of children with cerebral palsy (CP).

The study by Sullivan et al. (p 877) aimed to measure energy balance and body composition in children with severe cerebral palsy (CP) who were fed either orally or by gastrostomy tube. The authors show that feeding by gastrostomy tube, using a commercial formula, may lead to disproportionate (excess) fat deposition in these children. They note that while caloric supplementation of children with eating impairments is associated with weight gain, the nutritional composition of a diet for this group of children has yet to be determined.

A randomized placebo-controlled trial was carried out to investigate the efficacy of neuromuscular electrical stimulation (NMES) and threshold electrical stimulation (TES) in strengthening the quadriceps muscles of both legs in children with cerebral palsy (CP). Sixty children (38 males, 22 females; mean age 11y [SD 3y 6mo]; age range 5–16y) were randomized to one of the following groups: NMES (n=18), TES (n=20), or placebo (n=22). Clinical presentations were diplegia (n=55), quadriplegia (n=1), dystonia (n=1), ataxia (n=1), and non-classifiable CP (n=2). Thirty-four children walked unaided, 17 used posterior walkers, six used crutches, and the remaining three used sticks for mobility. Peak torque of the left and right quadriceps muscles, gross motor function, and impact of disability were assessed at baseline and end of treatment (16wks), and at a 6-week follow-up visit. No statistically significant difference was demonstrated between NMES or TES versus placebo for strength or function. Statistically significant differences were observed between NMES and TES versus placebo for impact of disability at the end of treatment, but only between TES and placebo at the 6-week follow-up. In conclusion, further evidence is required to show whether NMES and/or TES may be useful as an adjunct to therapy in ambulatory children with diplegia who find resistive strengthening programmes difficult.

Gastrostomy tube (GT) feeding in children with cerebral palsy (CP) is associated with significant increases in weight gain and, potentially, with overfeeding. This study aimed to measure energy balance and body composition in children with CP who were fed either orally or by GT. Forty children (27 males, 13 females; median age 8y 6mo; range 1y 4mo–18y 11mo) with spastic quadriplegic CP, of whom 22 were gastrostomy-fed and 18 orally-fed, underwent anthropometry, indirect calorimetry, and total energy expenditure determination (doubly-labelled water method). Total body water content (estimated by the 18O dilution method) was used to determine body composition. The Gross Motor Function Classification System (GMFCS) was used to determine the degree of motor impairment. GMFCS levels ranged from I to V; in the gastrostomy group 19 out of 22 were Level V and two out of 22 were Level IV. Within the orally-fed group, 11 out of 18 were Level V and four out of 18 were Level IV. Resting metabolic rate and total energy expenditure of the gastrostomy-fed children were lower but they had a significantly larger triceps skinfold thickness (p=0.01) and fat mass index (p=0.02) than the orally-fed children. Both groups had consistently higher body-fat content and lower fat-free (i.e. muscle and bone) content than the reference population of age- and sex-matched children without disabilities. This study has demonstrated the relatively low energy expenditure and high body-fat content of children with severe CP and highlighted the potential risk of overfeeding with available enteral feeds administered via GT.

The aim of this study was to determine whether botulinum toxin A (BTX-A) injected into the parotid and submandibular salivary glands of children aged 6 to 16 years with cerebral palsy (CP) and relative sialorrhoea significantly decreases their drooling and improves their quality of life. Twenty children (10 males, 10 females; mean age 10y 1mo [SD 3y 8mo]; range 6y 1mo–16y 7mo) with CP, identified as having significant daily drooling (scoring at least 6 on drooling frequency/severity scales) were recruited. BTX-A (2U/kg; maximum 70U) was injected under sedation, using ultrasound guidance (1.4U/kg and 0.6U/kg divided between parotid and submandibular glands respectively). Nineteen children completed the study: 15 with spastic quadriplegia (Gross Motor Function Classification System [GMFCS] Level V); one with dystonia (GMFCS Level V); one with hemiplegia (GMFCS Level II); and two with a mixed pattern of CP (GMFCS Levels III and V). Drooling was assessed by five methods at baseline, and 4 and 12 weeks after injection. Qualitative assessment of drooling frequency and severity scores showed statistically significant reductions at 4 weeks (p<0.001) and 12 weeks (p=0.006). Qualitative assessment of quality of life scores (rated by parents and teachers separately) also significantly improved (p<0.001 and p=0.023 respectively). Quantitative assessments showed that the number of bibs/scarves changed per day was significantly reduced at 4 weeks (p<0.001). There was no side effect from the injections themselves; 89% of parents and children wished for further intrasalivary BTX-A injections in the future. We conclude that percutaneous intrasalivary BTX-A injections into the parotid and submandibular salivary glands can reduce drooling in children with CP and relative sialorrhoea, leading to an improvement in their quality of life.

The effects of adapted spoons on food spilling and movement kinematics (movement duration, dysfluency) were examined in people with tetraparetic cerebral palsy (CP). We varied the thickness of the spoon's handle (diameters of 1, 3, and 5cm), the substance with which the spoon was filled (sugar or water), and the size of the bowl into which the spoon had to be emptied (diameter 5 or 11cm). Ten adolescents with tetraparesis (five males, five females; mean age 17y 2mo [SD 1y 3mo]; range 14y 7mo–18y 6mo) and 12 healthy comparison participants (four males, eight females; mean age 25y 6mo [SD 3y 0mo]; range 20y 0mo–30y 0mo) participated. Four participants with tetraparesis could walk independently, while the other six sat unstrapped in a wheelchair. The task consisted of picking up and transporting the filled spoon and emptying it into a bowl. Movements of the hand were recorded by a three-dimensional motion tracking device. When the spoon's handle was thickened participants with CP were faster in picking up the spoon and transporting it to the bowl, in particular when the spoon was filled with water. Importantly, this faster performance did not lead to increased spilling. These results are the first systematic test of the supposed beneficial effect of adapted spoons on movement performance in people with CP.

An ongoing population-based register of cerebral palsy (CP) in the West of Ireland was established in 2002 to calculate the prevalence of CP and to monitor CP epidemiological trends in the area. Children were only included if they were at least 5 years of age; children with postneonatal CP were also included. Eighty-five children were identified, giving an overall prevalence for the period 1990 to 1999 of 1.88 per 1000 neonatal survivors (95% confidence interval 1.5–2.4). Males accounted for 68% (n=51) and females for 32% (n=24) of all cases. Among infants weighing less than 1500g at birth, the rate of CP was 39/1000 neonatal survivors compared with 1.3/1000 for infants weighing more than 2500g. The most common CP subtype was bilateral spastic CP (51%), followed by hemiplegia (32%), dyskinesia (9%), and ataxia (7%). Eighteen per cent of all children were unable to walk, 21% had a sensory impairment, and 56% had an intellectual impairment.

Our aim was to study trends in the prevalence of pervasive developmental disorders (PDD) and to quantify their association with morphogenetic anomalies and with perinatal characteristics such as gestational age, birthweight, and hospitalization in a neonatal care unit. Data from a French morbidity register of childhood disabilities with the use of consistent definitions over time within the same geographical area were analyzed. The data of a total of 454 children (312 males, 142 females) with PDD, born between 1980 and 1993 and residing in Isère county, were recorded at the age of 7 years. The overall prevalence of PDD was 22.2 out of every 10000. There was a significant increase, from 14.7 to 30.8 out of every 10 000, during the period of study. Among these children with PDD, morphogenetic anomalies were observed in 12.1% (95% confidence interval [CI] 9.3–15.5), and the hospitalization rate during the neonatal period was 22% (95% CI 17.0–27.5), which is significantly higher than the observed rates in the general population. The increase in the prevalence of PDD, the association with perinatal risk factors, and the high rate of neonatal hospitalization require further studies to investigate the reasons for and mechanisms of these developmental disorders.

We compared the effect of sex and attention-deficit–hyperactivity disorder (ADHD) subtyping in groups of females and males. One hundred and one females with ADHD (mean age 10y 4mo [SD 2y 8mo]; range 5y–18y) were classified according to subtype by Diagnostic and Statistical Manual of Mental Disorders (4th edn) criteria (inattentive [ADHD-I]; combined [ADHD-C]) and balanced by subtype to 101 males (mean age 10y 5mo [SD 2y 9mo]; range 5y 4mo–17y 6mo). All children underwent IQ and reading assessment, and 109 underwent the continuous performance task (Test Of Variables of Attention [TOVA]). Parents completed the Conners' Abbreviated Rating Scale (ABRS), the Child Behavior Checklist (CBCL), learning disability questionnaires, and reported use and efficacy of methylphenidate. Teachers completed the Swanson, Kotkin, Agler, M-Flynn, and Pelham (SKAMP) rating scale. Sex differences were found only on the CBCL; females were more impaired on the attention (p<0.001) and somatization (p=0.028) subscales but not for IQ, other questionnaires, TOVA scores, methylphenidate treatment, or demographics. Females with ADHD-C, but not males, had significantly higher T-scores than females with ADHD-I on social, attention, delinquent, and aggressive behaviours. Regardless of sex, children with ADHD-C had higher scores on all CBCL subscales (p=0.047), ABRS (p<0.001), and SKAMP (p=0.03) than children with ADHD-I. The results support the supposition that ADHD in females is the same disorder as in males. ADHD subtyping was the important determinant of ADHD core symptoms; females with ADHD were found to have significant risk of psychopathology.

We describe a quantitative and comparative review of a selection of European birthweight standards for gestational age for singletons, to enable appropriate choices to be made for clinical and research use. Differences between median values at term across standards in 10 regions and misclassification of ‘small for gestational age’ (SGA), were studied. Sex and parity differences, exclusion criteria, and methods of construction were considered. There was wide variation between countries in exclusion criteria, methods of calculating standards, and median birthweight at term. The lightest standards (e.g. France's medians are 255g lower than Norway's medians) were associated with fewer exclusion criteria. Up to 20% of the population used in the construction of the Scottish standard would be classified as SGA using the Norwegian standard. Substantial misclassification of SGA is possible. Assumptions about variation used in the construction of some standards were not justified. It is not possible to conclude that there are real differences in birthweight standards between European countries. Country-based standards control for some population features but add misclassification due to the differing ways in which standards are derived. Standards should be chosen to reflect clinical or research need. If standards stratified by sex or parity are not available, adjustments should be made. In multinational studies, comparisons should be made between results using both a common standard and country-based standards.

This study reports on a novel methodology using the V-scope to quantify elbow and shoulder movement in young children with obstetric brachial plexus palsy (OBPP), and the intra- and interreliability of this method. The V-scope, a portable, inexpensive movement analysis system, was configured in an L-shape, with two transmitting towers placed on the floor and one 1.35m off the ground. These towers received ultrasonic pulses from buttons that were placed over standardized landmarks of the child's trunk, chest, and upper limb. Two physiotherapists (a paediatric and a generalist) facilitated the maximum range of active elbow flexion/extension and shoulder abduction/flexion in 30 children with OBPP (18 females, 12 males; age range 6mo–4y 7mo; mean age 2y 6mo [SD 1y 2mo]). Assessments were conducted on two occasions, one week apart. The V-scope was found to be feasible to use by a specialist and a generalist physiotherapist, demonstrating moderate to high reliability coefficients, small measurement errors, and lack of missing data. The pediatric physiotherapist was more reliable in measuring elbow and shoulder movement compared with the generalist physiotherapist, which suggests that the same experienced, pediatric physiotherapist should assess elbow and shoulder movement across all occasions of testing.

The Mayo-Portland Adaptability Inventory (MPAI; designed to be administered by clinicians) is a popular measure of disability following head injury in adults. Its acceptability, validity, and reliability were assessed for use with children. There were 335 children and adolescents (215 males, 120 females) aged between 1 and 19 years at injury (median age 9y 8mo [SD 5y]) in our sample. The test was acceptable to respondents, rapidly and easily administered, and required only small modifications. It demonstrated validity against client and parent reports of major symptoms. It demonstrated test–retest reliability within the limitations of our data and excellent interrater accord. Consequently, the MPAI is recommended for paediatric use for evaluating rehabilitation needs and therapy outcome.

To increase awareness of sudden and unexpected death in Lesch-Nyhan disease (LND) and to explore its potential causes, we report the anteceding clinical features and laboratory evaluations of five males with LND who ultimately experienced sudden and unexpected death, along with three additional males who suffered serious respiratory events during life. The ages of patients ranged from 2 to 45 years. The cause of sudden death in LND appears to have a respiratory rather than a cardiogenic basis. All cases cannot be linked readily with a single respiratory process. Instead, different respiratory processes appear to operate in different cases. These may include aspiration, laryngospasm, central apnea, cyanotic breath-holding spells, and high cervical spine damage. Better recognition of these processes will help to guide appropriate workup and management that could include chest imaging, endoscopy of the airways, polysomnography, electroencephalogram, and brain and/or spine imaging.

Hurler syndrome is the most severe form of mucopolysaccharidosis type I. There is a paucity of literature reporting the gross motor abilities of children with untreated Hurler syndrome. The purpose of this case series is to describe the gross motor abilities of one male and three female children (mean age 11.4mo [SD 3.1]; range 9.5–16mo) diagnosed with Hurler syndrome. The children were assessed using the Peabody Developmental Motor Scales, 2nd edition. Gross motor delays were present in all four children at the time of assessment, and were most evident in locomotor abilities for three of the children. All four children had range of motion limitations at multiple joints. This case series provides evidence for early gross motor delays in this population, as well as evidence for specific gross motor abilities of children with untreated Hurler syndrome. It is recommended that children diagnosed with Hurler syndrome be referred to physical therapy services upon diagnosis and that physical therapists be part of the interdisciplinary team involved in the care of children with Hurler syndrome.

Constraint-induced (CI) movement therapy is a physical intervention that has been receiving increasing attention in pediatric rehabilitation. So far, the evidence suggests that practice associated with CI therapy may improve impaired unimanual hand function in some children with hemiplegic cerebral palsy (CP). However, CI therapy has several important limitations. Most importantly, children with hemiplegia have impairments in bimanual coordination beyond their unilateral impairments. Thus, an intervention approach to increase functional independence during activities of daily living by using both hands in cooperation is needed. Here we briefly review the etiology of hemiplegic CP, describe studies of pediatric CI therapy efficacy in relation to the etiology, discuss the conceptual and practical limitations of CI therapy for this population, and describe bimanual coordination impairments in children with hemiplegia. Finally, we introduce a new intervention for children with hemiplegia, hand–arm bimanual intensive training (HABIT), to address the limitations of CI therapy and to improve bimanual coordination. HABIT retains the two major elements of pediatric CI therapy (intensive structured practice and child-friendliness). The proposed methodology demonstrates that extensive targeted practice can be provided in a child-friendly manner without using a physical restraint, although the efficacy of such an approach remains to be determined.

A systematic search was performed of all articles from January 1995 to December 2005 published in peer-reviewed journals on children with developmental coordination disorder (DCD). Criteria were applied to ensure that articles included DCD so 319 articles were included in the analysis. Since the publication of a systematic search conducted in 1994, the number of publications in this field has greatly increased across countries and disciplines. The term DCD was used in 52.7% of the articles. Other terms were used less frequently: clumsy children (7.2%), developmental dyspraxia (3.5%), handwriting problems (3.1%), hand–eye coordination problems (2.8%), sensory integration dysfunction (2.5%), deficits in attention, motor control, and perception (2.5%), minor neurological dysfunction (2.2%), and several other scattered terms (23.5%). The data indicated that progress has been made in the usage of the term DCD, but a standardized approach has not yet been achieved. Without consistent use of the term DCD, there is limited communication of research results internationally, slowed progress in understanding the condition, and limited development of intervention and management programmes for children with DCD.