Five children (three males, two females; four right-, one left-handed; age range 6 to 14 years) who developed aphasia after gross-total excision of left predominantly thalamic tumors are reported. Three patients had Broca aphasia, one had mixed transcortical aphasia, and one patient had conduction aphasia. In the months after surgery three children improved while receiving radiation and/or chemotherapy, although none recovered completely. Two patients with malignant tumors developed worsening aphasia when the tumor recurred and later died. Two patients (of three tested) had visuospatial difficulties in addition to language deficits. Attention and executive functioning were affected in three of three patients tested. Memory, verbal and/or visual functioning, were affected in four of four patients tested. Both patients who were tested showed transient right hemineglect. Two (of two tested) were probably apraxic. The wide range of deficits in these children highlights the importance of the thalamus and other subcortical structures in developing cognition.

To document the characteristics of early posttraumatic seizures (EPTS) in non-accidental head injury (NAHI), and examine their relation with outcome, a retrospective study was carried out. All children with NAHI admitted to the Royal Hospital for Sick Children, Edinburgh since 1981 were identified. The characteristics of EPTS, EEG, and outcome were noted. Forty-four cases were identified. The average age of children at presentation was 5.9 months. Thirty-two of these children had EPTS. The median length of follow-up was 3.0 years. The mortality rate was six in 44 (14%). The neurodevelopmental outcome correlated significantly with the presence and severity of EPTS (Tau = 0.317, p=0.017). Of survivors, 22% developed late posttraumatic epilepsy, the outcome in those with epilepsy was significantly worse than those without (p<0.0001). It was concluded that the severity of the primary brain injury dictates the severity of the EPTS and neurodevelopmental status at follow-up.

The study combined prospective neuropsychological and EEG results of 22 children presenting with typical benign partial epilepsy with rolandic spikes (19 participants) and occipital spikes (three participants). The aims were to assess the types of cognitive problems which may be encountered in this population, to evaluate the course of cognitive and learning capacities during the active phase of epilepsy, and to see if there was a correlation with paroxysmal activity on the EEG. Average age at entry in the study was 8.4 years and each child was seen 2 to 4 times over a period of 1 to 3 years. EEGs showed persistent spike foci in most cases that worsened in three cases, but there were no continuous spike–waves during sleep. No child had persistent stagnation, marked fluctuations, or a regression in cognitive abilities. Of 22 children, 21 had average IQ (>80). Eight children had school difficulties requiring special adjustment. No single cognitive profile was identified. Four children had delayed language development and eight children had transient weak scores in one isolated domain (verbal, visuospatial, memory) which improved or normalized during the course of the study with concomitant EEG improvement or normalization. In two of the three children with aggravation of the paroxysmal EEG activity, clinical changes were documented. A proportion of children with typical benign partial epilepsy with rolandic spikes showed mild, varied, and transient cognitive difficulties during the course of their epilepsy and in most cases this probably had a direct relation with the paroxysmal EEG activity.

This study investigated the association between family relationships and compliance in the treatment of childhood epilepsy. It was a prospective study of 21 families with a child who had epilepsy attending Central Middlesex Hospital, London. There were 13 boys, eight girls, with a mean age of 12.0 years (SD 2.9) at initial assessment. Mothers were interviewed for assessment of expressed emotion as a measure of parent–child relationships. Assessment of the mothers' adjustment using the General Health Questionnaire (Goldberg 1978), and psychological adjustment of the children using the Rutter Scales (Rutter et al. 1970a), were completed by mothers and teachers. Reassessment was 3 to 4 years after initial contact, including paediatric case-note review to assess clinic attendance and overall treatment compliance. Significantly more of the group who had a good level of compliance had recovered from epilepsy at follow-up. Good treatment compliance was found to be associated with less maternal hostility and criticism. Children and mothers in the good compliance group had fewer psychiatric symptoms. Poor treatment compliance and the associated psychological disturbances suggest assertive paediatric and psychosocial intervention may be needed for some children with epilepsy.

To obtain a preliminary validation of the Non-Communicating Children's Pain Checklist for individuals with inability to communicate verbally, 32 caregivers of individuals with cognitive impairments aged 3 to 44 years retrospectively completed the Non-Communicating Children's Pain Checklist and rated item usefulness (0 to 10). In the second part of the study 33 caregivers completed the checklist after two painful (e.g. burns, falls, surgery), one distressful (e.g. unwanted grooming, feared animal/noise), and one calm event (e.g. watching television). Checklist scores did not correlate with the age, sex, or physical limitations of individuals with inability to communicate verbally. In the first part of the study Cronbach's alpha was 0.66; all mean usefulness ratings exceeded 5 out of 10. In the second part of the study after four items were removed, Cronbach's alpha was 0.79. Checklist scores during pain correlated with numerical ratings of pain intensity, did not differ between the two pain events, and differed significantly from calm scores. Differences in Checklist scores during pain and distress were found for two subscales. The checklist exhibits internal consistency, and preliminary evidence suggests it can detect pain and is reliable over time.

The non-invasive Exeter Dysphagia Assessment Technique (EDAT) was evaluated as a method of assessing the aetiology of dysphagia in children with cerebral palsy (CP). Data have been collected from a group of 20 typically developing children (nine girls, 11 boys; age range 7 to 14 years) for comparison with 125 dysphagic children with CP (81 boys, 44 girls; age range 1 to 18 years). The swallowing mechanism has been separated into physiological phases: anticipatory, delivery, oral transit, and oral–pharyngeal. Normal or abnormal function in each phase was recorded and the common causes of any impaired phase were considered, starting with generalized possibilities before focusing on specific parts of swallowing physiology. Data from 125 dysphagic children with CP show marked differences from the data for the typically developing children. Interpreting individual results was valuable in assisting the assessment team to formulate management strategies; two examples are presented. The technique appears to provide a cost effective, non-invasive, and valuable clinical tool.

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.

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.

The objective of this and other systematic reviews by the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) is to provide the biomedical research and clinical practice communities with current knowledge about evidence supporting various interventions used in the management of developmental disabilities. “Systematic, evidence-based reviews are innovative in their comprehensive review of the literature, use of standard methods of presenting data, and special emphasis on the validity of research methods.” (p.470) AACPDM reviews summarize what is known and not yet known, to identify the gaps in our information about treatment outcomes and to gauge the strength of the internal validity of that evidence.