‘Cerebral palsy’ is an emotive term, which to parents often has the same impact as cancer or other dreaded diagnoses. For a child it is a label which can open doors to extra provision in schools and in the community, but which in itself may mean segregation, causing difficulties in participation. For a clinician it can be a diagnostic trap due to the failure to consider other conditions that impair motor function. For both clinicians and researchers it can be difficult to specify. Concerned about limitations of the current concept, a group of selected experts met last year to discuss the definition and classification of cerebral palsy. The preliminary results of their work are printed in this issue (p 571–576), with accompanying invited personal commentaries. We hope that it will provoke vigorous discussion.

The International Workshop on the Definition and Classification of Cerebral Palsy have proposed a fresh definition and classification of cerebral palsy (CP; p 571). In their accompanying paper they explain their reasoning behind this. Why is there the need for revision of the widely accepted and comfortably familiar definitions of Bax and Mutch? These definitions are beautifully succinct and clear but perhaps not inclusive enough, making no mention of pathogenesis or of the functional and ‘non-motor’ features of CP which are most significant when it comes to everyday life. There has been debate in the past as to whether a more aetiological definition should be adopted but we are not yet ready for this. CP remains unexplained in around 15% of children, even after good neuroimaging and metabolic investigation and we recognize that a wide range of CNS disorders can result in a similar clinical picture. Thus, a predominantly phenotypic definition and classification remains the most appropriate. The workshop clearly deliberated long and hard over every word of the revised definition producing a much more detailed description of CP that now encompasses the effect on function (activity limitation) and the comorbid features. As a result, the definition is longer and it does not ‘trip off the tongue’ in the way the old ones did.

Revisiting and updating the definition and classification of cerebral palsy (CP) is not just an academic task for the developmental paediatrician and neurologist but an exercise aimed at providing a framework for multidisciplinary management by all professionals and care providers involved in the care of children with developmental disabilities.

The occurrence of the International Workshop on Definition and Classification of Cerebral Palsy, in Bethesda, Maryland, USA, July 2004, was an encouraging sign of progress towards a collaborative attempt to reach an internationally accepted definition and classification system for cerebral palsy (CP). The definition given in this issue, apparently submitted by a small subset of workshop attendees, is disappointing in that it does little to address the four areas of imprecision encountered in previous definitions of CP, as described by Stanley et al. These four areas are:

‘Cerebral palsy (CP) describes a group of disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behaviour, and/or by a seizure disorder.’

Parents and paediatric neurologists need information on the long-term social prognosis of children with cerebral palsy (CP). No large population-based study has been performed on this topic. On 31 December 1999, to find predictors in childhood of subsequent education and employment, 819 participants with CP born between 1965 and 1978 (471 males; mean age 28y 10mo, SD 4y, range 21 to 35y) in the Danish Cerebral Palsy Registry were compared with 4406 controls without CP born between 1965 and 1978 (2546 males; mean age 28y 10mo, SD 4y, range 21 to 35y). Diagnostic subtypes of the 819 participants with CP were: 31% hemiplegia, 43% diplegia, 18% tetraplegia, and 8% other types. Level of motor impairment with respect to walking ability was: 62% able to walk without assistance, 21% with assistance, and 16% not able to walk (for 1% of study children walking ability was not known). Relevant information was obtained from Denmark's unique registries. Of the participants with CP, 33% vs 77% of controls, had education beyond lower secondary school (i.e. after age 15–16y), 29% were competitively employed (vs 82% of controls), 5% were studying, and 5% had specially created jobs. Excluding participants with CP with an estimated developmental quotient (DQ) of less than 50 or inability to walk at age 5 to 6 years, the odds ratios (multivariate analysis) for not being competitively employed were 1.9 for diplegia versus hemiplegia, 22.5 for DQ 50 to 85 versus DQ greater than 85, and 3.7 for those with epilepsy versus those without epilepsy. The severity of motor impairment among participants with CP able to walk had just a minor influence. Only half the participants with CP who had attended mainstream schooling were employed. In conclusion several childhood characteristics seemed to predict long-term social prognosis.

The timed ‘Up & Go’ test (TUG) is a test of basic or functional mobility in adults which has rarely been used in children. Functional mobility was defined for this study as an individual's ability to manoeuvre his or her body capably and independently to accomplish everyday tasks. Reliability and validity of TUG scores were examined in 176 children without physical disabilities (94 males, 82 females; mean age 5y 9mo [SD 1y 8mo]; range 3 to 9y) and in 41 young people with physical disabilities due to cerebral palsy or spina bifida (20 males, 21 females; mean age 8y 11mo [SD 4y 3mo], range 3 to 19y). Mean TUG score for children without physical disability was 5.9s (SD 1.3). Reliability of the TUG test was high, with intraclass correlation coefficients (ICC) of 0.89 within session, and 0.83 for test–retest reliability. Mean score of the group aged 3 to 5 years was significantly higher (6.7s SD 1.2) than that of the older group (5.1s, SD 0.8; p=0.001). Scores in the younger group reduced significantly over a 5-month follow-up period (p=0.001), indicating that the TUG was responsive to change. Within-session reliability of the TUG in young people with disabilities was very high (ICC=0.99). There were significant differences in TUG scores between children classified at levels I, II, and III of the Gross Motor Function Classification System (p=0.001). TUG scores showed a moderate negative correlation with scores on the Standing and Walking dimensions of the Gross Motor Function Measure (n=22, rho=–0.52, p=0.012). There was no significant difference in TUG scores between typically developing male and female children. The TUG can be used reliably in children as young as 3 years using the protocol described in this paper. It is a meaningful, quick, and practical objective measure of functional mobility. With further investigation, the TUG is potentially useful as a screening test, an outcome measure in intervention studies for young people with disabilities, a measure of disability, and as a measure of change in functional mobility over time.

The aim of the present research was to investigate the impact of abnormal fetal environment on explicit memory performance. Based on animal models, it was hypothesized that infants of diabetic mothers (IDMs) experience perturbations in memory performance due to exposure to multiple neurologic risk factors including: chronic hypoxia, hyperglycemia/reactive hypoglycemia, and iron deficiency. Memory performance, as measured by the elicited/deferred imitation paradigm, was compared between 13 IDMs (seven females, six males; mean age 365 days, SD 11) and 16 typically developing children (seven females, nine males; mean age 379 days, SD 9). The IDM group was characterized by shorter gestational age (mean 38w, SD 2), greater standardized birthweight scores (mean 3797g, SD 947), and lower iron stores (mean ferritin concentration 87μg/L, SD 68) in comparison with the control group (mean gestational age: 40w, SD 1; mean birthweight: 3639g, SD 348; mean newborn ferritin concentration 140μg/L, SD 46). After statistically controlling for both gestational age and global cognitive abilities, IDMs demonstrated a deficit in the ability to recall multi-step event sequences after a delay was imposed. These findings highlight the importance of the prenatal environment on subsequent mnemonic behavior and suggest a connection between metabolic abnormalities during the prenatal period, development of memory, circuitry, and behavioral mnemonic performance.

A longitudinal comparison was made between development of verbal and visuo-spatial short-term memory and vocabulary in children with Down syndrome (DS), children with specific language impairment (SLI), and typically developing children as a control group. Participants were 12 children with DS (6 males, 6 females; mean chronological age 9y 9mo [SD 2.8mo], range 8y 6mo to 11y 4mo); nine children with SLI (4 males, 5 females; mean chronological age 3y 9mo [SD 4.8mo], range 3y 3mo to 4y 5mo); and 12 typically developing children (5 males, 7 females; mean chronological age 4y 4mo [SD 3.9mo], range 3y 3mo to 4y 3mo). Participants were matched on mental age (mean mental age 4y 3mo). All participants completed verbal short-term memory, visuo-spatial short-term memory, and expressive and receptive vocabulary tasks on three occasions over 1 year. Similarities were seen in the clinical groups for verbal short-term memory. There was some evidence of difficulty in visuo-spatial short-term memory in the children with SLI relative to the other groups, but all three groups showed overlap in visuo-spatial short-term memory performance. At the final time-point vocabulary performance in the clinical groups was similar; the typically developing children showed higher vocabulary abilities than both clinical groups.

This preliminary study compared the daily living skills of children with and without attention-deficit–hyperactivity disorder (ADHD), and the influence of a social skills training group on these skills. Twenty-seven children with ADHD (2 females, 25 males; age range 5 to 8y, mean 6y 6mo, SD 10mo), and 24 children without ADHD (8 females, 16 males; age range 5 to 8y, mean 6y 11mo, SD 10mo) performed the Assessment of Motor and Process Skills (AMPS). Fourteen of the children with ADHD used medication daily. Nine of the total group with ADHD were randomly selected to attend group treatment which focused on social skills training, through meaningful occupations (e.g. art, games, cooking). Children were evaluated at the beginning of group treatment and after 10 sessions. Ten children without ADHD were evaluated at similar intervals. Children with ADHD initially achieved significantly lower scores on the AMPS in all process skills (p<0.001) and in the coordination motor subtest (p<0.005) than children without ADHD. Children with ADHD significantly improved from the first to the second evaluation and no longer differed from the children without ADHD after treatment (p<0.008). The results emphasize the need for a focus upon occupation in assessment and treatment of children with ADHD.

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 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.

Hashimoto's encephalopathy (HE) is a steroid responsive, relapsing encephalopathy associated with thyroid autoantibodies. Paediatric literature mainly consists of case reports of the disease. A questionnaire survey of 68 consultant paediatric neurologists was undertaken through the British Paediatric Neurology Association in 2002 to gather preliminary data about this condition. The response rate was 68% and a total of ten patients were identified: nine from the UK and one from Ireland. Complete clinical details were available on eight patients (seven females and one male). Age at presentation ranged from 10 to 15 years (mean 12y 7mo, SD 1y 1mo). Presenting features included seizures (n=7/8), encephalopathy (n=7/8), cognitive decline (n=4/8), behavioural problems (n=3/8), psychosis (n=2/8), myoclonus (n=1/8), and tremors (n=1/8). All had thyroid autoantibodies and four were also hypothyroid at diagnosis. One patient became hypothyroid during follow-up. Electroencephalograms (EEGs) showed high amplitude slow background rhythms in all patients, and one patient also had focal spikes. Cognitive deficits were identified in four patients and persisted in one over 2 years of follow-up. Six patients improved with steroids and two improved spontaneously. Two had relapsing courses during follow-up afer diagnosis (range 12–48 months). HE may be currently under-recognized in children and increased awareness can result in prompt diagnosis and treatment. Steroid therapy appears to be beneficial. Neuropsychological assessment is required in all cases and may guide steroid therapy. Long-term prognosis for cognition remains guarded at this time.

In two males, 11 and 12 years of age, referred for attention-deficit–hyperactivity disorder (ADHD), 47,XYY syndrome was diagnosed. A team that included a neuropsychologist, a physiotherapist, and a physician examined them. Stature (patients were above 97.5% height for age), muscle consistency, and tremor indicated chromosome analysis. Psychological tests results did not fully fit the ADHD diagnosis. On the basis of our clinical observation we felt that stimulant medication was indicated. Administration of methylphenidate led to improved motor and cognitive functions as well as social adaptation in both patients. We suggest that this treatment might well be considered in clinically similar patients with XYY sex chromosomes; we further suggest that learning problems in such individuals may be related to ADHD.

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.

Because of the availability of new knowledge about the neurobiology of developmental brain injury, information that epidemiology and modern brain imaging is providing, the availability of more precise measuring instruments of patient performance, and the increase in studies evaluating the efficacy of therapy for the consequences of injury, the need for reconsideration of the definition and classification of cerebral palsy (CP) has become evident. Pertinent material was reviewed at an international symposium participated in by selected leaders in the preclinical and clinical sciences. Suggestions were made about the content of a revised definition and classification of CP that would meet the needs of clinicians, investigators, and health officials, and provide a common language for improved communication. With leadership and direction from an Executive Committee, panels utilized this information and have generated a revised Definition and Classification of Cerebral Palsy. The Executive Committee presents this revision and welcomes substantive comments about it.