Status epilepticus is a common neurological emergency in children admitted to hospitals in resource-poor countries where it has a high mortality and is associated with a significant morbidity. In the last 5 years, some pharmaceutical companies have announced their intention to stop manufacturing parenteral preparations of phenobarbitone and chlormethaziole, and the supply of paraldehyde has been erratic. Paraldehyde and phenobarbitone are recommended for the management of status epilepticus in the recently published Advanced Paediatric Life Support guidelines. Their withdrawal would have serious repercussions for the management of status epilepticus in resource-poor countries as these drugs are relatively cheap and easy to administer in these settings.

Skeletal health in children is made up of a number of factors including the overall pattern of the skeleton, the appropriate size and shape of individual bones, bone mass and internal architecture of both tubular and plate-like bones, and bone metabolism and turnover. Where among this group of attributes does skeletal maturity fit in?

Twenty-three low-birthweight infants (17 males, six females) diagnosed with cystic periventricular leukomalacia (PVL; median gestational age 30wks, postmenstrual age range 25 to 36wks; median birthweight 1365g, range 680 to 2010g) were evaluated and compared with 209 comparison infants (117 males, 92 females; median gestational age 33wks, postmenstrual age range 25 to 39wks; birthweight 1771g, range 670 to 2460g). There were three assessment times: 36 to 38 weeks (preterm), 40 to 42 weeks (term), and 44 to 46 weeks (post-term); the Neonatal Behavioral Assessment Scale (NBAS) was used for assessment. Outcome at 2 years was assessed on the basis of a neurological examination, magnetic resonance imaging, computerized tomography, electroencephalography, and the Mental and Psychomotor Development Indices of the Bayley Scales of Infant Development. All infants in the PVL group were found to have evidence of cerebral palsy, whereas all infants in the comparison group were normally developing. Infants with PVL performed more poorly on all elements of the NBAS examination at all three assessment times compared with the comparison group. They demonstrated poorer motor control, less responsiveness to environmental stimuli, less regulatory capacity, and more abnormal reflexes compared with the comparison group. These results suggest that LBW infants with PVL show dysfunction and/or disorganization in their neurobehavioural systems in the neonatal period. Assessment of neonatal neurobehavioural characteristics using the NBAS may assist clinicians in identifying LBW infants with PVL, and in formulating plans for the developmental care of these infants.

Abnormalities of growth and development are prevalent in children with cerebral palsy (CP). The purpose of this study was to assess skeletal maturation (‘bone age’) in this population and to identify those factors related to alterations in this aspect of development. The study group was a convenience sample of 80 participants (47 males, 33 females; age range 2y 6mo to 21y 1mo; mean age 10y 10mo, SD 4y 2mo). All had moderate to severe spastic or mixed spastic CP at Gross Motor Function Classification System level III (n=8, 10%); level IV (n=21, 26%); or level V (n=51, 64%). Skeletal maturation was assessed by the detailed Fels method of scoring hand–wrist radiographs. Each evaluation included clinical and anthropometric assessments, Tanner staging, bone density measurements, and the Children's Health Status Questionnaire. Follow-up evaluations were obtained for 41 participants, providing a total of 143 evaluations. Median interval between first and last evaluations was 24.7 months (range 11.9 to 45mo). For the study group as a whole there was no significant difference between a child's skeletal age and chronological age. However, there was a high prevalence of individual participants in whom skeletal age was advanced (7%) or delayed (10%), relative to chronological age, by more than 2 years. In multivariant analyses it was found that diminished linear growth (height), low lumbar-spine bone density, and low body fat as measured by triceps skinfolds were all independently associated with delays in skeletal maturation. Multiple aspects of skeletal growth and development, including skeletal maturation, are frequently altered in children with moderate to severe CP.

The objective of this study was to explore the behaviour, including sleep patterns, of children with neurofibromatosis type 1 (NF1). For this purpose we designed a cross-sectional descriptive survey conducted by postal enquiry with telephone follow-up. Simonds and Parraga's sleep questionnaire and the Strength and Difficulties Questionnaire were used to screen sleep patterns and behaviours respectively. Information was obtained for 64 children (39 male, mean age 10y 7mo, SD 4y 1mo, range 3 to 18y; 57% response rate). There were increased numbers of children with NF1 achieving scores in the borderline and abnormal range for ratings of peer problems (p<0.001), hyperactivity (p<0.001), emotional symptoms (p<0.001), and conduct disorder (p<0.05). Total difficulties score was also high (p<0.001) in the NF1 group. Only one group of sleep problems, parasomnias (in particular sleepwalking and sleep terrors), had a higher occurrence (p[les ]0.05) in the NF1 group than in the general population. Within the NF1 group, conduct problems (p[les ]0.05), hyperactivity (p[les ]0.01), emotional problems (p[les ]0.01), and the total difficulties score (p[les ]0.01) were all significantly higher in children with frequent sleep disturbance. We conclude that NF1 is associated with sleep and behavioural problems in a high proportion of children. Psychiatric conditions, e.g. attention-deficit–hyperactivity disorder, may be under-diagnosed on a large scale in children with NF1, and the use of simple screening tools in clinical settings may prove beneficial.

We have previously reported that significant hyperopia at 9 months predicts mild deficits on visuocognitive and visuomotor measures between 2 years and 5 years 6 months. Here we compare the motor skills of children who had been hyperopic in infancy (hyperopic group) with those who had been emmetropic (control group), using the Movement Assessment Battery for Children (Movement ABC). Children were tested at 3 years 6 months (hyperopic group: 47 males, 63 females, mean age 3y 7mo, SD1.6mo; control group: 61 males, 70 females, mean age 3y 7mo, SD1.2mo) and at 5 years 6 months (hyperopic group: 43 males, 56 females, mean age 5y 4mo, SD1.7mo; control group: 51 males, 62 females, mean age 5y 3mo, SD1.6mo). The hyperopic group performed significantly worse at both ages, overall and on at least one test from each category of motor skill (manual dexterity, balance, and ball skills). Distributions of scores showed that these differences were not due to poor performance by a minority but to a widespread mild deficit in the hyperopic group. This study also provides the first normative data on the Movement ABC for children below 4 years of age, and shows that it provides a useful measure of motor development at this young age.

The aim of this study was to propose a classification system for childhood arterial ischaemic stroke (AIS). Subtypes from the Trial of Org 10172 in Acute Stroke Therapy (TOAST) classification, previously shown to be applicable to children, were retained in the proposed Paediatric Stroke Classification (PSC). Additional important paediatric AIS aetiologies were identified from a literature review. Preliminary validation was performed by three raters who categorized clinical vignettes from 135 patients (66 male; median age 6.3y, range 0.1 to 16y). Eight aetiological subtypes were identified and defined, as follows: (1) sickle cell disease; (2) cardioembolic; (3) moyamoya syndrome; (4) cervical arterial dissection; (5) steno-occlusive cerebral arteriopathy; (6) other determined aetiology; (7) multiple probable/possible aetiologies; and (8) undetermined aetiology. There was very good agreement between the raters about categorization of the vignettes. Causes of disagreement were identified and final categories and definitions were modified accordingly. We conclude that the PSC enables the categorization of children with AIS into aetiological subtypes relevant to this age group. This will be useful in multicentre studies of natural history and treatment but will require further independent validation.

This study used meta-analytical techniques to explore the association between intelligence and age in children with Duchenne muscular dystrophy (DMD). The sample comprised 1224 children and young adults with DMD (mean age 12y 3mo, SD 4y; range 2y to 27y). Standardized measures including the Wechsler Intelligence Scales (WIS) and the Stanford-Binet Intelligence Scales were used to estimate intelligence. No age-related difference was noted for Full-scale and Performance intelligence quotients (IQ). However, Verbal IQ increased significantly with age. Age-related increases were noted for the WIS Information, Similarities, Arithmetic, Comprehension, Digit Span, Picture Arrangement, Block Design, and Coding subscales. These results support the notion that younger children with DMD have deficits in verbal reasoning and verbal processing. Older children with DMD, particularly those of 14 years and older, were less likely to present with these problems. Implications of these findings and possible future research directions are discussed.

The aim of this study was to evaluate the effects of a modified version of constraint-induced (CI) movement therapy on bimanual hand-use in children with hemiplegic cerebral palsy (CP; age range 18mo to 4y) and to make a comparison with conventional paediatric treatment. Twenty-one children (13 females, eight males) completed the CI therapy programme and 20 children (12 males, eight females) served as a control group. Children in the CI therapy group were expected to wear a restraint glove for 2 hours each day over a period of 2 months. The training was based on principles of motor learning used in play and in motivational settings. To evaluate the effect of treatment, the Assisting Hand Assessment (AHA) was used. Assessments took place on three occasions: at onset, after 2 months, and 6 months after the first assessment. A significant interaction was found between group and AHA measure (ANOVA, F2,74=5.64, p=0.005). The children who received CI therapy improved their ability to use their hemiplegic hand significantly more than the children in the control group after 2 months, i.e. after treatment. Effect size was high after treatment and remained medium at 6 months. As the treatment was tailored to each child's capacity and interests, little frustration was experienced by the children.

Preterm birth can affect many neurological functions and is known to affect vision when damage to the visual cortex or optic radiations occurs. However, it is not known whether extreme preterm birth affects visual development, either favorably or unfavorably. Additional time in an extrauterine environment could conceivably allow the acceleration of visual abilities as a result of additional experience. Although recent studies suggest that even apparently healthy preterm infants may suffer some degree of loss of neurological function. In this paper we describe what is known about visual development in preterm infants, and we make the case that additional studies are needed to clarify the impact of preterm birth on vision. Because the visual system now lends itself to quantitative studies of function, it could offer researchers and clinicians a method of detecting subtle effects of preterm birth on neurological development and function.

The relationships between quality of life (QoL), participation, and environment are being studied within a multicentre European study of 8- to 12-year-old children with cerebral palsy (CP; Study of PARticipation of Children with cerebral palsy Living in Europe [SPARCLE], www.ncl.ac.uk/sparcle/). The international group was determined that children with CP who also had intellectual impairment (IIm) should not be excluded from the study, but the group realized that there was little information available on how to assess subjective QoL in children with IIm. This annotation reviews and discusses the literature on conceptual and methodological issues in relation to such assessment.