We know how important it is to diagnose epilepsy correctly, but how to do so is surprisingly little validated. An inadequate history and misinterpretation of the electroencephalogram (EEG) are the major reasons 4 to 26% of children and adults in European studies are misdiagnosed with epilepsy. Error rates are not available for most countries, or for more precise diagnoses of seizure types, epilepsy syndrome, and cause.

Adeli suit treatment (AST) is a pertinent and timely topic for research. Many families and clinicians are interested in the outcomes of treatment using the Adeli suit, but the rehabilitation community does not have adequate scientific support for its use as a generally accepted treatment for cerebral palsy (CP). Very little research has been completed around non-traditional treatments such as AST. The study reported in this issue of DMCN by Bar-Haim et al. compared the use of AST and traditional neurodevelopment treatment (NDT) in children with CP.

This study compared the efficacy of Adeli suit treatment (AST) with neurodevelopmental treatment (NDT) in children with cerebral palsy (CP). Twenty-four children with CP, Levels II to IV according to the Gross Motor Function Classification System (GMFCS), were matched by age and functional status and randomly assigned to the AST or NDT treatment groups. In the AST group (n=12; eight males, four females; mean age 8.3y [SD 2.0]), six children had spastic/ataxic diplegia, one triplegia and five spastic/mixed quadriplegia. In the NDT group (n=12; nine males, three females; mean age 8.1y [SD 2.2]), five children had spastic diplegia and seven had spastic/mixed quadriplegia. Both groups were treated for 4 weeks (2 hours daily, 5 days per week, 20 sessions). To compare treatments, the Gross Motor Function Measure (GMFM-66) and the mechanical efficiency index (EIHB) during stair-climbing were measured at baseline, immediately after 1 month of treatment, and 10 months after baseline. The small but significant time effects for GMFM-66 and EIHB that were noted after 1 month of both intensive physiotherapy courses were greater than expected from natural maturation of children with CP at this age. Improvements in motor skills and their retention 9 months after treatment were not significantly different between the two treatment modes. Post hoc analysis indicated a greater increase in EIHB after 1 month (p=0.16) and 10 months (p=0.004) in AST than that in NDT, predominantly in the children with higher motor function (GMFCS Levels II and III). The results suggest that AST might improve mechanical efficiency without a corresponding gain in gross motor skills, especially in children with higher levels of motor function.

The aim of this study was to describe the social experience of children with cerebral palsy (CP) in mainstream classes in Canada and compare it with that of their classmates without disability. The CP group included 25 females and 35 males (mean age 10y 5mo [SD 0.95], range 10y 4mo–10y 10mo) diagnosed as having hemiplegia (n=44) or diplegia (n=16) and classified as Level I on the Gross Motor Function Classification System (GMFCS). Fifty-seven comparison children, born at term and without any motor and/or sensory impairment, were recruited from the classes of the children with CP during a school visit (mean age 10y 3mo, [SD 1.0], range 10y–10y 6mo). They were matched to children with CP for sex, age, parents' education level, and family income. Social adjustment measures (social status, reciprocated friendships, social isolation, aggression, sociability/leadership, and verbal and/or physical victimization) were obtained by conducting a class-wide sociometric interview (n=943) in the classes of the children with CP. Findings showed that children with CP (specifically females with CP and irrespective of their type of disability) had fewer reciprocated friendships, exhibited fewer sociable/leadership behaviours, and were more isolated and victimized by their peers than their classmates without a disability. This seems to suggest that females and males with CP are perceived differently from their peers in a mainstreaming context. The discussion addresses the issue of age- and sex-related differences and provides avenues of intervention relating to personal and environmental factors that could facilitate or interfere with the social experience of children with CP in a mainstream environment.

Children with physical disabilities are at increased risk of limitations to participation in everyday activities. This study describes research examining the participation of children in day-to-day formal and informal activities (excluding mandated academic schooling). Using the Children's Assessment of Participation and Enjoyment (CAPE) measure, data on participation patterns were collected from 427 children (229 males, 198 females; mean age 10y [SD 2y 4mo]; range 6-14y) with physical limitations and from their families. The primary types of physical disability in the sample included cerebral palsy, spina bifida, acquired brain injury, and musculoskeletal limitations. Findings indicate a broad range of diversity and intensity of participation, with proportionately greater involvement in informal rather than formal activities. Significant differences in participation and enjoyment were found between males and females, and for children more than 12 years of age. Children's participation was less diverse in families reporting lower income, single-parent status, and lower respondent parent education. These findings provide a foundation for an improved understanding of the participation of children with physical disabilities, which can assist families and service providers in planning activities that fit with their child's preferences and ensure active participation.

This study sought to describe change and stability in health-related quality of life (HRQL) over time in a cross-section of children with cerebral palsy (CP) using the Child Health Questionnaire (CHQ) through repeated surveys of the children's parents/caregivers. A total of 177 children with CP (98 males, 79 females); age range 3 to 18 years (mean age 8y 6mo, [SD 4y 2mo]; Gross Motor Function Classification System: Level I 40%, Level II 14%, Level III 14%, Level IV 16%, and Level V 17%) were enrolled as a convenience sample from the outpatient clinics at a tertiary-care children's hospital. The main outcome measure was HRQL as determined by the CHQ – Parent Form 50. Parents reported reduced HRQL for their children with CP relative to norms for the CHQ. Although Role – Physical functioning (reflecting how physical impairment interferes with daily activity) declined with time, all other subscales of the CHQ were stable over the course of 1 year. CHQ scores were not measurably affected by common medical interventions. Children with CP have lower CHQ scores than other children that are, on average, stable over the course of 1 year.

This study investigated whether in preterm children who had ventricular dilatation (VD) on neonatal cranial ultrasound outcome at age 8 years was influenced by the additional presence of germinal matrix haemorrhage – intraventricular haemorrhage (GMH–IVH). Six-hundred and ninety-nine preterm infants (<33wks' gestation, mean 29.6wks [SD 2.1]) with either normal cranial ultrasound (n=616; 286 females, 330 males), or with VD with (n=66; 32 females, 34 males) or without (n=17; 4 females, 13 males) GMH–IVH were enrolled in the study. At age 8 years outcome was assessed in 567 (81%) of the 699 children by neurological examination, the Test of Motor Impairment (TOMI), the test of Visuo-Motor Integration (VMI), and the Wechsler Intelligence Scales for Children. Results showed that the proportion of children with disabling impairments was higher in the group with VD and GMH–IVH. Performance on TOMI and VMI (even in those without disabling impairments) was poorer in those with VD and GMH–IVH than in children with normal scans or those with VD only. Children with VD and GMH–IVH had significantly lower performance IQ than children with normal ultrasound, whereas those with VD only were not different from those with normal scans. Results suggest the presence of subtle white matter injury that has not been identified by neonatal cranial ultrasound. Although this study did not investigate biochemical markers of haemorrhage, we hypothesize that non-protein-bound iron is likely to be a contributing factor to white matter damage in preterm infants.

A prospective controlled study with repeated measures before and after surgery examined the medical, surgical, and health outcomes of gastrostomy for children with disabilities at a tertiary paediatric referral centre in the North Thames area, UK. Anthropometric measures included weight, mid-upper-arm and head circumference. Five-day prospective food diaries were completed and data on physical health and surgical outcomes recorded. Seventy-six children participated and underwent gastrostomy (44 males, 32 females; median age 3y 4mo, range 4mo–17y 5mo), and 35/76 required an anti-reflux procedure. Categories of disability were: cerebral palsy (32/76), syndrome of chromosomal or other genetic origin (25/76), slowly progressive degenerative disease (11/76), and unconfirmed diagnosis (8/76). Most children had gross motor difficulties (99%) and were non-ambulant (83%). Oromotor problems were identified in 78% of children, 69% aspirated, and 65% were fed nasogastrically before surgery. The mean weight before surgery was –2.84 standard deviation score (SDS; SD 2.21, range –9.8 to 3.4). Two-thirds of children achieved catch-up growth postoperatively: weight-for-age (mean difference 0.51 SDS, 95% CI 0.23–0.79, p=0.001) and mid-upper arm circumference (mean difference 1.12cm, 95% confidence interval 0.50–1.75, p=0.001). Health gains included a reduction in drooling, secretions, vomiting, and constipation. Major surgical complications were found in 13/74 children. The study provides evidence that catch-up growth and health gains are possible following gastrostomy.

We present a prospective study at school age of neuropsychiatric and neurodevelopmental outcome of language delay suspected at child health screening around 30 months of age. In a community sample, 25 children (21 males, 4 females) screening positive and 80 children (38 males, 42 females) screening negative for speech and language problems at age 30 months were examined in detail for language disorders at age 6 years. The screen-positive children were then followed for another year and underwent in-depth neuropsychiatric examination by assessors blind to the results of previous testing. Detailed follow-up results at age 7 years were available for 21 children. Thirteen of these 21 children (62%) had a major neuropsychiatric diagnosis (autism, atypical autism, Asperger's syndrome, attention-deficit–hyperactivity disorder [ADHD]), or combinations of these. Two further children (10%) had borderline IQ with no other major diagnosis. We conclude that children in the general population who screen positive for speech and language problems before age 3 years appear to be at very high risk of autism spectrum disorders or ADHD, or both, at 7 years of age. Remaining language problems at age 6 years strongly predict the presence of neuropsychiatric or neurodevelopmental disorders at age 7 years.

Relationships between early motor development and language and reading skills were studied in 154 children, of whom 75 had familial risk of dyslexia (37 females, 38 males; at-risk group) and 79 constituted a control group (32 females, 47 males). Motor development was assessed by a structured parental questionnaire during the child's first year of life. Vocabulary and inflectional morphology skills were used as early indicators of language skills at 3 years 6 months and 5 years or 5 years 6 months of age, and reading speed was used as a later indicator of reading skills at 7 years of age. The same subgroups as in our earlier study (in which the cluster analysis was described) were used in this study. The three subgroups of the control group were ‘fast motor development’, ‘slow fine motor development’, and ‘slow gross motor development’, and the two subgroups of the at-risk group were ‘slow motor development’ and ‘fast motor development’. A significant difference was found between the development of expressive language skills. Children with familial risk of dyslexia and slow motor development had a smaller vocabulary with poorer inflectional skills than the other children. They were also slower in their reading speed at the end of the first grade at the age of 7 years. Two different associations are discussed, namely the connection between early motor development and language development, and the connection between early motor development and reading speed.

The reliability of visual interpretation of electroencephalograms (EEG) is of great importance in assessing the value of this diagnostic tool. We prospectively obtained 50 standard EEGs and 61 EEGs after partial sleep deprivation from 93 children (56 males, 37 females) with a mean age of 6 years 10 months (SE 5mo; range 4mo–15y 7mo) with one or more newly diagnosed, unprovoked seizures. Two clinical neurophysiologists independently classified the background pattern and the presence of epileptiform discharges or focal non-epileptiform abnormalities of each EEG. The agreement was substantial for the interpretation of the EEG as normal or abnormal (kappa 0.66), almost perfect for the presence of epileptiform discharges (kappa 0.83), substantial for the occurrence of an abnormal background pattern (kappa 0.73), and moderate for the presence of focal non-epileptiform discharges (kappa 0.54). In conclusion, the reliability of the visual interpretation of EEGs in children is almost perfect as regards the presence of epileptiform abnormalities, and moderate to substantial for the presence of other abnormalities.

Evaluation of the cognitive repercussions of an epileptic disorder and its treatment are important issues in clinical follow-up. This especially holds true for temporal lobe epilepsy (TLE) where resective surgery can be a valid treatment option. However, in patients where TLE coexists with another neurocognitive disorder, questions can arise about the precise nature of the neuropsychological deficits. The aim of the present study was to evaluate memory impairments, found in a male aged 12 years who had the dual pathology of fragile X syndrome and refractory TLE. Memory functions of this child were compared with those of a male aged 11 years 7 months with fragile X syndrome matched for intellectual functioning as indicated by highly comparable verbal (5y 5mo vs 5y 9mo) and non-verbal (7y 2mo vs 6y 1mo) cognitive age equivalents. Performance on each neuropsychological measure was evaluated twice, separately with normative data based on chronological age and on verbal or non-verbal cognitive level. A specific, distinguishable profile of task performance could be found only when controlling for general level of cognitive functioning. This made it possible to accurately evaluate neuropsychological abilities before and 6 months after anterior temporal lobe resection even in this male with a complex neurological pathology.

A male and female with juvenile metachromatic leukodystrophy (MLD) with unusual manifestations are presented, each involving a novel arylsulfatase A gene mutation. One patient demonstrated acute intermittent encephalopathic episodes for 1 year after having received the diagnosis of MLD at the age of 6 years. The other patient presented at the age of 5 years with acute hemiparesis, which was diagnosed as acute disseminated encephalomyelitis and resolved in 3 weeks. After 2 years of remission he started to show progressive neurological deterioration. The episodic manifestations in both patients were associated with acute, resolving cerebral lesions on magnetic resonance imaging accompanying or preceding the classical demyelinating lesions of MLD. The diagnosis of MLD was based on arylsulfatase A enzyme activity levels and genetic analysis, and after the exclusion of neurological conditions such as encephalitis, vasculopathy, or mitochondrial disorders. The pathogenesis of this previously undescribed finding in MLD is unknown but might be related to a susceptibility of myelin to acute damage.

The aim of this paper is to evaluate correlative magnetic resonance imaging (MRI) and histological parameters of development of cortical afferents during pathfinding and target selection in transient fetal cerebral laminas in human fetuses and preterm infants. The transient fetal subplate zone, situated between the fetal white matter (i.e. intermediate zone) and the cortical plate, is the crucial laminar compartment for development of thalamocortical and corticocortical afferents. The prolonged coexistence of transient (endogenously active) and permanent (sensory-driven) circuitry within the transient fetal zones is a salient feature of the fetal and preterm cortex; this transient circuitry is the substrate of cerebral functions in preterm infants. Another transient aspect of organization of developing fibre pathways is the abundance of extracellular matrix and guidance molecules in periventricular crossroads of projection and corticocortical pathways. Both the subplate zone and periventricular crossroads are visible on MRI in vivo and in vitro. Hypoxic-ischaemic lesions of periventricular crossroads are the substrate for motor, sensory, and cognitive deficits after focal periventricular leukomalacia (PVL). Lesions of distal portions of the white matter and the subplate zone are the substrate for diffuse PVL. The neuronal elements in transient fetal zones form a developmental potential for plasticity after perinatal cerebral lesions.

‘Stability of the Bayley II Scales of Infant Development in a sample of low-risk and high-risk infants’ Harris et al. DMCN Vol 47: 820–823

We would like to correct an error that was printed in the above mentioned article:

p 821: The sentence should have read: ‘The initial assessment took place during the first year of life (between 2.5 and 12.5mo of age…’ and not ‘The initial assessment took place during the first year of life (between 2y 6mo and 12y 6mo of age…’

We sincerely apologize for this error.

The possibility that so-called anti-epileptic drugs (AEDs) may aggravate epilepsy must always be borne in mind by the clinician. Many reports of such aggravation of seizures have been published. Most such reports are anecdotal and speculative, and suggest that many such reactions are idiosyncratic. However, for some there is a sufficient body of evidence to suggest that some AEDs used in certain epilepsies may consistently cause worsening of seizures. Seizure aggravation may include increase in the frequency or severity of existing seizures, emergence of new types of seizure, or the occurrence of status epilepticus. The pathophysiology of seizure aggravation is poorly understood including non-specific effects such as those associated with sedation, drug-induced encephalopathy, and paradoxical or inverse pharmacodynamic effects. For some epilepsies the choice of AEDs may be inappropriate, and although the mechanism of seizure aggravation is not clear, its occurrence may be fairly predictable. This is best documented for the use of carbamazepine in idiopathic generalized and myoclonic epilepsies. Most other AEDs have been reported occasionally to cause seizure aggravation. The lowest risk of seizure aggravation appears to be with valproate. Risk factors for worsening of seizures are epileptic encephalopathy, polytherapy, high frequency of seizures, and cognitive impairment. Advances in pharmacogenomics may in the future enable such adverse effects to be predicted for individual patients. Meanwhile, a systematic approach to reporting AED-induced seizure aggravation should be developed.