This is a prospective cohort study of 237 children (130 males, 107 females) placed on the ketogenic diet for control of intractable epilepsy (mean age at starting diet 3 years 8 months; age range 2 months to 9 years 10 months); average length of follow-up was 308 days. There were 133 children on the diet at 1 year and 76 at 2 years. Height and weight measurements were converted into age- and sex-appropriate z scores. There was a rapid drop in weight z scores in the first 3 months. After this initial period, the weight z score remained constant in children who started the diet below the median weight for their age and sex, although z scores continued to decrease in children starting above the median. There was a small decrease in height z scores in the first 6 months (<0.5); however, there were larger changes by 2 years. There was no difference based on sex for either height or weight. The ketogenic diet generally provides sufficient nutrition to maintain growth within normal parameters over a defined period. Very young children grow poorly on the diet and should be followed-up carefully over long periods of use.

The aim of the study was to determine whether learning and memory are compromised in school children with recently diagnosed idiopathic and/or cryptogenic epilepsy and to study relationships between learning and memory and psychosocial and epilepsy variables. Word span and learning of locations were assessed within 48 hours after diagnosis of epilepsy and three and 12 months later, in 69 school children with epilepsy (aged 9.1 years, SD 2.7; 33 males, 36 females) and 66 classmates. Results showed that patients and controls performed similarly in registration, recall, and retention. Patients recalled slightly less than controls when probed under conditions of increased demand on working memory. Maladaptive reactions of parents and children to the onset of epilepsy and not reaching 6-months of seizure remission contributed to poor performance. Individually, those patients who required special assistance at school, under-performed occasionally in one or the other component of memory. Although the proportion of under-performers was stable over time, the children composing the group did change. It was concluded that school children with new onset idiopathic or cryptogenic epilepsy are inordinately vulnerable when processing memory tasks. The vulnerability is neither persistent nor memory-specific.

Eighteen children (13 males, five females) who had severe developmental language delay/disorder and some features of autism (although insufficient in severity and combination to meet ICD-10 diagnostic criteria for childhood autism) at preschool age (Time 1; mean age 4 years 4 months) were followed up 4 years later (Time 2; mean age 8 years 7 months). At the initial assessment the diagnostic dilemma was how much the social communication impairments and behavioural problems were secondary to the language problem and how much they constituted a genuine case of a pervasive developmental disorder. It was anticipated that at follow-up some children would continue to show social impairments but that in others social impairments would have receded as language competence improved. Follow-up assessments included the Wechsler Intelligence Scale for Children, the Clinical Evaluation of Language Fundamentals, the Children's Communication Checklist, and the Social Communication Questionnaire. At follow-up, five children had continuing language disorder and were considered to fulfil diagnostic criteria for childhood autism, four children had continuing language disorder and met criteria for atypical autism, and nine met criteria for atypical autism but had somewhat recovered language skills. Thus, even in the subgroup of children whose language ability had improved the features of autism had not dissipated. Severity of social communication impairments and repetitive behaviours at Time 1, rated retrospectively from case notes, were associated with severity of autism symptoms and pragmatic competence at Time 2. The findings are discussed in relation to the unclear boundary between autism spectrum disorders and language delay/disorder.

We evaluated gross motor function following botulinum toxin A (BTX-A) injections in the lower limbs of children with spastic cerebral palsy in a randomized clinical trial, using a cross-over design. Forty-nine children (24 males, 25 females, age range 22 to 80 months) were randomly allocated to two groups: group 1 received BTX-A and physiotherapy, and group 2 received physiotherapy alone for 6 months. At the end of this period, group 2 received BTX-A and physiotherapy and group 1 continued with physiotherapy alone. Assessment measures were the Gross Motor Function Measure (GMFM), the Vulpe Assessment Battery (VAB), joint range of movement, the Modified Ashworth Scale, and a parental questionnaire. Sustained gains in gross motor function were found in both groups of children but the only additional benefit found in group 1 was a significant increase in fine motor rating on the VAB. By contrast, parents rated the benefit of treatment highly. It is likely that assessment at 3 and 6 months post injection was too late to demonstrate peak gross motor function response and that changes in GMFM are not sustained over 6 months with a single dose. Further studies should investigate changes over shorter time periods and consider covariables such as BTX-A dosage, number of injection sites, and the role of repeated injections combined with other interventions such as casting.

Functional outcome and provision of care to 82 children (males: females 2.7:1; age range 0 to 16 years) with severe head injury were investigated. The children were admitted to the intensive care units of the Regional Neuroscience Units of the Greater Manchester and Lancashire districts of the North West Region of the UK between 1994 and 1996. A questionnaire was devised based on 12 areas of recovery and data were collected at discharge and 6 weeks, 6 months and 12 months from discharge. Data were collected during home visits and at joint assessment at 12 months with the district consultant community paediatrician (CCP). Early involvement of the CCP enhanced the provision of needs at discharge and 6 weeks after discharge, as did a period of stay in district-level care before discharge home. CCPs received formal notification of the injured child in only 32% of cases by discharge, and 54% of cases by 6 months. Sixty-five per cent of children required early educational support but structured help reached only 55% of these children by the end of the study. Integrated planning between health and education was achieved in about half of the study population. Good physical recovery was achieved by the majority of children but parents said they did not feel prepared for the degree of help which their child still required 12 months after discharge. Children who required anticonvulsants at 12 months' follow-up scored significantly lower on cognitive potential. Psychosocial family functioning deteriorated in a substantial number of families according to parental perception. Prevalence of this perception did not diminish over the study period. Aspects of caregivers' understanding and the child's language deficits, self-care skills, fine and gross motor performance, as well as family, social, and financial consequences were assessed. A dedicated and integrated approach to assessment and provision of care across the domains of hospital, education, and community is discussed.

Two male patients (a child and an adult) with congenital mirror movement were studied using functional MRI (fMRI) and transcranial magnetic stimulation (TMS). Bilateral primary sensorimotor cortices were activated during unilateral hand gripping on fMRI when the child patient was 8 years old and the adult was 37 years old. Bilateral motor evoked potentials were induced from the hand and forearm muscles after TMS of each hemisphere. Bilateral motor responses were also induced from the arm muscles in the adult patient. Bilateral motor responses had short and similar latencies. Contralateral motor responses to TMS were smaller than ipsilateral ones in the hand muscles, while contralateral responses were larger than ipsilateral ones in the arm muscles. Contralateral hand motor responses reduced in amplitude or disappeared with increasing age while in the child patient, mirror movements decreased gradually. Our results suggest that bilateral activation of the primary sensorimotor cortices during intended unilateral hand movement and bilateral motor responses to TMS account, at least in part, for the pathophysiology of congenital mirror movement. Reduction of contralateral hand motor responses may be related to the decrease in mirror movements during development.

An unusual case of cervical spinal cord tethering with diplomyelia is described. A 12-month old female presented with self-mutilation of the fingers due to sensory loss in the hands, absent reflexes, poor muscle tone, and reduced distal upper-limb movements. There was a deep skin dimple overlying the T1 spinous process. Imaging showed angulation of the lower cervical cord and an operation revealed a low cervical meningocele and a split cord malformation with tethering of one half of the cord; the cord was untethered. In this report the literature is reviewed.

There has been some controversy about whether the recessively-inherited disease infantile neuroaxonal dystrophy (IND; Seitelberger's disease) is a separate entity or a variety of pantothenatekinase-associated neurodegeneration (PKAN/Hallervorden–Spatz syndrome).