The sensitivity of the pendulum test to variation in spasticity in persons with spastic cerebral palsy (CP) was tested in 30 participants with CP and 10 participants without CP (controls) (mean age 13.8 years). The participants with CP were classified into three groups, with normal (mean age 15.9 years), mild/moderate (13.0 years), or severe (23.0 years) muscle tone, as assessed clinically using a modified Ashworth scale. Joint motion during the pendulum test was measured with an electrogoniometer. Muscle relaxation was confirmed using surface EMG. Outcome measures from the pendulum test were (1) number of oscillations, (2) duration of oscillations, (3) excursion of the first backward swing, and (4) relaxation index (first swing excursion/difference between the starting and resting angles). Data were assessed using one-way analysis of variance. Outcome measures 1 to 3 differed significantly between control participants and participants with CP (p<0.05). The first swing excursion was the best predictor of the degree of spasticity in persons with CP, being significantly different between all groups (p<0.05). The number of oscillations and their duration differentiated between control participants and all participants with CP (p<0.05) but not between participants with CP who had mild/moderate versus severe spasticity (p>0.05). The relaxation index was not a sensitive measure (p>0.05 between most study groups). We conclude that the pendulum test is a valid tool for assessing spasticity in persons with CP and that the first swing excursion is the most sensitive outcome measure.

To study the development of physical health and some neuromotor functions in anorexia nervosa (AN) 51 individuals (48 females, three males) with a mean AN onset of 14 years, recruited after community screening, were followed prospectively together with 51 age-, sex-, and school-matched individuals without AN (controls). About 10 years after AN onset, all individuals were examined in respect of physical health and neurodevelopment. There were no deaths. Weight and height had normalised, except in three participants with persistent AN. Significantly more participants with AN had a physical complaint/disorder, including hirsutism. This might be a long-term complication in weight restored AN. Dysdiadochokinesis occurred almost exclusively among individuals with former AN in accordance with our previous studies.

This paper examines the perceptions of mainstreaming among six young people (mean age, 15.5 years; range 13 to 18 years) with physical disabilities, their parents, and seven teaching staff of one ‘designated’ school in the United Kingdom. The study used semistructured interviews to allow the participants to talk about their experiences of mainstreaming in their own words. The results were then analysed and formed the basis for a multiperspective case study. The analysis revealed that the processes involved that exclude the child with a physical disability from mainstream school life are highlighted. They appear to be subtle and also specific to individual children and families.

To create a short screening instrument to investigate the development of ideomotor praxis representation (IPR), 357 normally developing children between the ages of 2½ and 9½ years were investigated. The IPR screening consisted of six mimed actions which had to be performed on verbal request. The greatest change in the representational style is the transition from performing the action using the body as the object to performing with an imaginary object (i.e. symbolic representation). This transition suggests a development from a concrete and egocentric level of performance to an abstract and allocentric level. Before the age of 6 years differences are evident between self-directed and externally directed gestures. The level of symbolic representation has to be dissociated from the degree of motor precision, which shows hardly any improvement after 6 years of age. With the proposed model one may interpret the level of IPR in clinical practice. IPR screening is clinically relevant from the age of 4½ years onwards.

Dichotic listening performance was studied in children who at an early age had undergone a myringotomy with insertion of ventilating tubes for persistent middle ear infections (otitis media with effusion; OME) and compared with age-equivalent children who had no history of otitis media or hearing problems. The OME group consisted of 19 children with a median age of 9 years; 15 of whom were right-handed, and 14 were boys. The comparison sample comprised 18 children with a median age was 9 years 4 months. Hand preference and sex were matched with the OME group. Both groups were tested with dichotic listening to consonant–vowel syllables and additional forced-attention tasks. The comparison sample showed a weak right-ear advantage, and age-adequate attentional modulations. The children in the OME group showed a strong right-ear advantage, but were not able to modulate the ear advantage during directed-attention tasks. Models for interpreting the result are discussed.

This cross-sectional study describes oral health problems in a sample of children with Down syndrome (DS) compared with their siblings. Data were collected using the Oral Assessment – Down Syndrome questionnaire, a validated French language evaluation to be completed by the child's carer. Parents attending a national meeting anonymously answered a questionnaire for their child with DS and for the sibling closest in age and returned their replies by post. Data concerning 204 individuals with DS (103 males, 101 females; mean age 9.6 years) and 161 of their siblings (80 males, 81 females; mean age 11.8 years) were returned. The association between DS status, age, and oral health was analysed. There was a greater frequency of oral health problems in those with DS with regard to function, clinical signs, disability, and development. The prevalence of chewing problems did not improve with age, the prevalence of bleeding gums and mouth breathing increased with age, and that of tongue protrusion reduced with age. Most parents rated the general and oral health of their children with DS highly, although worse than for their siblings.

Bone-mineral content (BMC; g) and density (BMD; g/cm2) were measured by dual energy X-ray absorptiometry in the proximal femur, femoral neck, and total body of nutritionally adequate children (n=17; 11 girls, six boys; aged 7.6 to 13.8 years) with spastic cerebral palsy (CP). Bone-mineral-free lean tissue (BMFL; g) and fat mass (FM; g) were obtained from total body scans. Chronological and developmental age-based z scores for the children with CP were derived from a pediatric database (n=894). Children with CP had BMC z scores from –1.8 (total body) to –3.2 (femoral neck) SDs below the normative sample. Non-independent ambulators had lower z scores for total body BMD, femoral neck BMD, and BMC than independent ambulators. The BMFL z score of individuals with CP was 2 SDs below that of the reference group and higher in the independent ambulators than in the non-independent ambulators, whereas FM deviated little. These findings suggest that non-nutritional factors, such as ambulation, account for the low BMC, BMD, and BMFL tissue observed in this population.

Ankle spasticity and strength in 27 children with spastic diplegic cerebral palsy (CP) (mean age 9 years, range 3 to 18 years) and a group of 12 children without CP (comparison group) (mean age 9 years, range 5 to 18 years) were observed. To measure spasticity, a KinCom dynamometer dorsiflexed the passive ankle at five different speeds and recorded the resistive plantarflexion torques. Work values for the torque-angle data were calculated at each speed. Using this data, linear regression was used to measure spasticity. To measure strength, the dynamometer rotated the ankle from maximum dorsiflexion to maximum plantarflexion at a speed of 10°/s while the child performed a maximum plantarflexion concentric contraction. The movement was reversed to record maximum dorsiflexion. Maximum torques and work by the plantarflexors and dorsiflexors were calculated. The group with CP had significantly more spasticity in the plantarflexors and significantly less strength in the plantarflexors and dorsiflexors than the group without CP. Results provide objective information quantifying ankle spasticity and strength in children with CP.

This paper reports on a systematic review of the literature commissioned to examine the feasibility of universal screening for speech and language delay. The results, based on an examination of productivity figures, including positive predictive ability and likelihood ratio, indicate that a number of screening tests are adequate. Sensitivity was generally lower than specificity, and study quality was inversely related to both sensitivity and likelihood ratio, suggesting that it is easier to identify accurately children who do not have language and speech problems than those who do. The review concluded that there is insufficient evidence to warrant the introduction of universal screening at this stage. This paper discusses the type of data that would be needed to address this issue further and recommendations are made for alternative approaches to early identification.

The incidence of childhood autism and other autistic spectrum disorders (ASDs) in preschool children was determined for two areas of the West Midlands between 1991 and 1996. Children diagnosed before the age of 5 years and residing within the study areas at diagnosis were detected from the records of four child development centres. The incidence rate per 10000 children per year for the combined areas was 8.3 for all children with ASDs, 3.5 for classical childhood autism (CA), and 4.8 for other ASDs. Rates were similar in both areas, despite differences in social deprivation and proportions of ethnic minorities. While rates for classical CA increased by 18% per year, a much larger increase (55% per year) was seen for ‘other ASDs’, suggesting that clinicians are becoming increasingly able and/or willing to diagnose ASDs in preschool children.

Language and learning disabilities occur in almost half of individuals with oral clefts. The characteristics of these cognitive dysfunctions vary according to the cleft type, and the mechanisms underlying the relation between cleft type, cognitive dysfunction, and cleft-caused middle-ear disease are unknown. This study investigates preattentive auditory discrimination, which plays a significant role in language acquisition and usage, in infants with different cleft types. A mismatch negativity (MMN) component of brain evoked potentials, which indexes preconscious sound discrimination, and brain responses to rare sine-wave tones were recorded in 12 healthy infants and 32 infants with oral clefts at the ages of 0 and 6 months. Infants with clefts were subdivided into two categories: those with cleft lip and palate (CLP) (n=11 at birth, n=6 at the age of 6 months) and those with cleft palate only (CPO) (n=17 at birth, n=8 at the age of 6 months). At both ages, brain responses to rare sounds tended to be smaller in both cleft subgroups than in healthy peers. However, in the latency range of 300 to 500 ms, the MMN was significantly smaller in infants with CPO. In infants with CLP, the MMN was comparable to that of healthy infants. Differences in auditory discrimination between infants with CLP and CPO, as reflected by MMN, were detectable at birth and persisted into later infancy. This pattern parallels known behavioural differences between children with these cleft types. Brain responses to rare sounds, in contrast, had no differentiative power with respect to the cleft type.

Patients with Sturge-Weber syndrome often present with seizures during the first year of life. Currently, only patients with clinically significant seizures who do not respond to medical treatment are candidates for early epileptic surgery. However, a delay of surgical treatment may result in cognitive deterioration. We studied the correlation between parameters and outcome of seizures to re-examine the criteria for early epilepsy surgery. We performed a retrospective chart review combined with telephone interviews of parents of all Israeli infants with unilateral Sturge-Weber syndrome and early onset seizures, and we examined whether age of seizure onset and seizure intensity were correlated with cognitive level and the degree of hemiparesis at follow-up. We recruited a total of 15 patients with unilateral Sturge-Weber syndrome and early onset seizures, five of whom underwent epilepsy surgery. The mean follow-up period of all the patients was 15 years: six patients had normal intelligence, four had borderline cognitive level, three had mild mental retardation and two had moderate mental retardation. Eight of the ten non-operated patients still experience seizures at follow-up. Cognitive delay was significantly correlated with seizure intensity in the early period, but not with the age of seizures onset, the degree of hemiparesis, or the presence of ongoing seizures. We conclude that high seizure intensity in young patients with Sturge-Weber syndrome is a prognostic marker for mental deterioration.

Atraumatic fractures are often seen in children and adolescents with cerebral palsy (CP) and epilepsy in full-time care. Increased bone fragility was postulated to be due to osteopenia resulting from a combination of factors including immobilization and antiepileptic treatment. The aim of this study was to determine the effect of vitamin D and calcium substitution on bone mineral density (BMD) in a group of children with CP in full-time care. Twenty children with the most severe form of CP (spastic quadriplegia) who had been treated with antiepileptic drugs for a relatively long period of time were included in the study. Physical examination and laboratory analyses excluded other possible causes of osteopenia. BMD was measured by dual X-ray absorptiometry. Thirteen patients were treated for 9 months with 1,25-dihydroxy-cholecalciferol vitamin D (0.25 mcg daily) and with calcium (500 mg daily). Seven control children were used for observation only. BMD greatly increased in the treated group, while children with CP in full-time care who did not receive vitamin D and calcium substitution continued to lose their bone mass. It can be concluded that the addition of vitamin D and calcium increases BMD in children with the most severe form of CP, who are receiving antiepileptic drugs.

Postoperative pain in children with spastic cerebral palsy (CP) is often attributed to muscle spasm and is difficult to manage using opiates and benzodiazepines. Adductor-release surgery to treat or prevent hip dislocation in children with spastic CP is frequently performed and is often accompanied by severe postoperative pain and spasm. A double-blinded, randomized, placebo-controlled clinical trial of 16 patients (mean age 4.7 years) with a mainly spastic type of CP (either diplegic or quadriplegic in distribution) was used to test the hypothesis that a significant proportion of postoperative pain is secondary to muscle spasm and, therefore, might be reduced by a preoperative chemodenervation of the target surgical muscle by intramuscular injection of botulinum toxin A (BTX/A). Compared with the placebo, BTX/A was found to be associated with a reduction in mean pain scores of 74% (P<0.003), a reduction in mean analgesic requirements of approximately 50% (P<0.005), and a reduction in mean length of hospital admission of 33% (P<0.003). It was concluded that an important component of postoperative pain in the patient population is due to muscle spasm and this can be managed effectively by preoperative injection with BTX/A. These findings may have implications for the management of pain secondary to muscle spasm in other clinical settings.

The aim was to investigate the performance of children with attention-deficit–hyperactivity disorder (ADHD) in tasks involving motor–memory representations. A special grip object recorded forces generated by the fingertips during a precision grip–lift task. Common objects were lifted from a linear scale. Twenty-five boys with ADHD were evaluated and grouped according to the presence (ADHD+) or absence (ADHD) of movement dysfunction using the Movement Assessment Battery for Children (Henderson and Sugden 1992). Mean group ages were 11.4 years(range 9.0 to 11.0 years) and 11.7 years (9.0 to 15.6 years), respectively. They were compared to a control group of 25 age-matched boys, mean group age 11.8 years (range 9.0 to 13.0 years). Variability of motor performance was predominant in the ADHD+ group. Several of these participants presented a higher grip-force output during the gripping movement. They also had difficulties in adapting the motor output to target different weights, suggesting deficient anticipatory parameter control based on memory representations. The results suggest that in some children motor problems are due to detrimental neural control functions rather than core symptoms of ADHD.

A 5-year-old boy with focal cortical dysplasia was referred to our hospital because of epileptic seizures. He showed mild weakness of the left hand without sensory disturbance. Brain MRI revealed extensive cortical dysplasia with pachygyria and microgyria around the right central sulcus. On EEG examination, interictal spikes were noted over the right fronto/centro/parietal region. A 37-channel magnetometer revealed that the sources of the spikes were in a small, restricted region of the normal frontal lobe adjacent to the dysplastic brain. Somatosensory evoked magnetic fields indicated that the location of the current source of N20 was in the same area. Our patient shows a unique case of plasticity and reorganization of the somatosensory function due to cortical dysplasia.

Eighty-two women who were consuming alcohol while pregnant attended a special clinic at the University Central Hospital, Helsinki with the aim of reducing heavy drinking during pregnancy. The children born to these women were followed up regularly. During their preschool years the children were assessed to have fetal alcohol syndrome, fetal alcohol effects, alcohol-related neurodevelopmental disorder, pre- and/or postnatal growth retardation, or they were assessed to have normal cognitive and somatic growth. Of the original children, 70 of 82 could be traced at the age of 12 years. Through semistructured interview and contact with the health and social care authorities, information was gathered about schooling, family structure, whether help had been sought for behavioural difficulties and major adverse events in the family. The longer the intrauterine alcohol exposure and the more severe the diagnosis related to prenatal alcohol exposure, the more often the children required special education, were temporarily or permanently taken into care, and had behavioural problems. There is a considerable need for prolonged multidisciplinary follow-up and support of all children whose mothers have not been able to reduce drinking in early pregnancy, whether or not cognitive disturbances are evident in early childhood.

Foix–Chavany–Marie syndrome (FCMS) is a distinct clinical picture of suprabulbar (pseudobulbar) palsy due to bilateral anterior opercular lesions. Symptoms include anarthria/severe dysarthria and loss of voluntary muscular functions of the face and tongue, and problems with mastication and swallowing with preservation of reflex and autonomic functions. FCMS may be congenital or acquired as well as persistent or intermittent. The aetiology is heterogeneous; vascular events in adulthood, nearly exclusively affecting adults who experience multiple subsequent strokes; CNS infections; bilateral dysgenesis of the perisylvian region; and epileptic disorders. Of the six cases reported here, three children had FCMS as the result of meningoencephalitis, two children had FCMS due to a congenital bilateral perisylvian syndrome, and one child had intermittent FCMS due to an atypical benign partial epilepsy with partial status epilepticus. The congenital dysgenetic type of FCMS and its functional epileptogenic variant share clinical and EEG features suggesting a common pathogenesis. Consequently, an increased vulnerability of the perisylvian region to adverse events in utero is discussed. In honour of Worster-Drought, who described the clinical entity in children 40 years ago, the term Worster-Drought syndrome is proposed for this unique disorder in children.

3-phosphoglycerate dehydrogenase deficiency is a severe but treatable disorder of serine synthesis, first described in 1996 (Jaeken et al. 1996a). The patient presented with West syndrome, severe psychomotor delay, failure to thrive, microcephaly, atypical ocular movements, and pyramidal signs. Treatment with oral L-serine abolished seizures and improved psychomotor development, hyperexcitability, head growth, cortical and subcortical hypotrophy, and hypomyelination of the brain on MRI scans. 3-phosphoglyce-rate dehydrogenase deficiency is a treatable congential error that probably leads to West syndrome.

Three ambulant males with multicore myopathy, a rare congenital myopathy, are reported with nocturnal hypoventilation progressing to respiratory failure at the age of 9, 13, and 21 years. Deterioration in these individuals occurred over several months without any precipitating event. Patients had clinical evidence of nocturnal hypoventilation with hypoxaemia and hypercapnia. Forced vital capacity was significantly reduced (20 to 43% of predicted level). These parameters improved on institution of overnight ventilation using a BiPAP pressure support ventilator with face mask or nasal pillows with O2 saturation maintained above 90% overnight and an increase in forced vital capacity by as much as 100% (0.3 to 0.6 litres). This was matched by a symptomatic and functional improvement. Also present in these patients and not previously reported is the association of multicore myopathy with paraspinal contractures which produce a characteristic scoliosis described as a ‘side-sliding’ spine. This may be improved by spinal bracing or surgery.