The placentas of 68 infants with intrauterine growth restriction (IUGR) were examined for evidence of impaired uteroplacental circulation and compared with those of 65 appropriately grown infants. Infarcts and/or accelerated villous maturation were present in the placentas in 27 (40%) of the infants with IUGR compared with seven (11%) of the infants without IUGR (P<0.001). The infants were followed-up at 4 and 12 months of age and growth parameters recorded. Medical and developmental assessments and neuromotor developmental examinations were also performed. The 23 infants in the IUGR group with placentas with evidence of impaired uteroplacental circulation were compared with the 31 infants with IUGR with normal placentas. There was no difference between the groups in growth, cognitive development, or neuromotor abnormality. It was concluded that IUGR is strongly associated with placental markers of impaired uteroplacental blood flow while it would appear that there is no association between placental pathology and growth or neurodevelopment in the first year.

Hashimoto's encephalopathy (HE) is a steroid responsive, relapsing encephalopathy associated with thyroid autoantibodies. Paediatric literature mainly consists of case reports of the disease. A questionnaire survey of 68 consultant paediatric neurologists was undertaken through the British Paediatric Neurology Association in 2002 to gather preliminary data about this condition. The response rate was 68% and a total of ten patients were identified: nine from the UK and one from Ireland. Complete clinical details were available on eight patients (seven females and one male). Age at presentation ranged from 10 to 15 years (mean 12y 7mo, SD 1y 1mo). Presenting features included seizures (n=7/8), encephalopathy (n=7/8), cognitive decline (n=4/8), behavioural problems (n=3/8), psychosis (n=2/8), myoclonus (n=1/8), and tremors (n=1/8). All had thyroid autoantibodies and four were also hypothyroid at diagnosis. One patient became hypothyroid during follow-up. Electroencephalograms (EEGs) showed high amplitude slow background rhythms in all patients, and one patient also had focal spikes. Cognitive deficits were identified in four patients and persisted in one over 2 years of follow-up. Six patients improved with steroids and two improved spontaneously. Two had relapsing courses during follow-up afer diagnosis (range 12–48 months). HE may be currently under-recognized in children and increased awareness can result in prompt diagnosis and treatment. Steroid therapy appears to be beneficial. Neuropsychological assessment is required in all cases and may guide steroid therapy. Long-term prognosis for cognition remains guarded at this time.

This paper describes the neuroimaging and neuropathological findings in five cases of severe brain damage after traumatic mid-trimester amniocentesis, all performed between 1986 and 1994. Although fetal injury after amniocentesis has been reported, reports of brain injury are infrequent. Continuous ultrasound monitoring may reduce the risk of fetal injury but follow-up ultrasound scans can be falsely reassuring. Withdrawal of blood-stained fluid, particularly if it contains tissue fragments, should alert the operator to the possibility of fetal damage. Histological examination of such tissue fragments may confirm the nature of the fetal damage. The consequences of fetal brain injury are severe, all five of our cases showed evidence of disruption of brain development compatible with mid-term injury. Obstetricians and their patients should be aware of the small but significant risk of brain damage after mid-term amniocentesis.

The clinical efficacy of lidocaine for convulsive status epilepticus in 53 convulsive episodes was examined in 37 children (17 males, 20 females). Mean age of patients receiving lidocaine was 3 years 7 months (SD 3y 5mo). Lidocaine administration achieved control of status epilepticus in 19 of 53 convulsive episodes (35.8%). Seizures ceased within 5 minutes of lidocaine administration in all 19 patients who were responsive to the drug. Regarding aetiology of status epilepticus and types of seizures, there was no statistical difference in effectiveness. Mild decrease of oxygen saturation, monitored by pulse oximetry, was observed in one patient, which improved by oxygenation using a mask. Lidocaine is a useful anticonvulsive agent; however, the response rate to lidocaine appears to be quite low, as less than half of the seizures were effectively controlled by lidocaine. Favourable properties of the drug include prompt responses, less alteration of consciousness, and fewer adverse effects, including less respiratory depression.

Visual–perceptual abilities were assessed in 5-year-old children with the following neonatal neurological conditions: born preterm with normal ultrasound scan (NL, n=17); born preterm with ultrasound diagnosis of intraventricular haemorrhage (IVH, n=17); born preterm with ultrasound diagnosis of periventricular leukomalacia (PVL, n=12); born term with hypoxic–ischaemic encephalopathy (HIE, n=11). Visual–perceptual ability was evaluated with the L94: eight visual–perceptual tasks designed to evaluate different aspects of visual perception at the preschool level in children with multiple disabilities. Impairment was established in comparison to the performance age obtained on non-verbal intelligence subtests, instead of chronological age. Frequency of L94 impairment was highest in children with PVL, while children with IVH did not differ from the NL control group. Impairment rates were increased also in children with transient periventricular echodensities, and in children with HIE. Impairments were only moderately related to the delay of visual acuity maturation in infancy.

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.

This study aimed to determine whether sodium valproate (VPA) improves cognitive performance and behaviour in children with learning and behavioural problems associated with electrographic epileptiform discharges but without clinical seizures. A randomized, double-blind, single-crossover trial was carried out with VPA or placebo on eight participants with different learning and behaviour problems. Participants also underwent neuropsychological testing under video EEG and the parent and teacher Behaviour Check List (CBCL; Achenbach 1991a, b) during each treatment phase. Clinically none of the children improved on VPA. On formal testing children were more distractable, had increased delay in response time, and showed lower memory scores while on VPA. In addition, parents reported higher internalizing scores on the CBCL while children were on VPA. Our data do not support the use of VPA in similar patients.

There were two purposes for this study: to determine the within-subject changes of visual evoked potentials (VEPs) over time, and to evaluate the usefulness of baseline VEP testing in the early diagnosis of increased intracranial pressure (ICP). Twenty children with hydrocephalus between the ages of 4 and 18 years (18 males, 2 females) were evaluated monthly for up to four months. Flash-evoked VEP tests were done in a standardized manner using a Cadwell Quantum 84 evoked potential stimulation and recording instrument. One participant had abnormal VEPs found during a previous episode of shunt malfunction. For all of the other VEP tests, the latency of the N2 response was in the range of adult normal values. For 15 participants with multiple evaluations there were non-significant differences in N2 latency from one session to the next. This study's results provide evidence that within-subject VEP measurements in children with hydrocephalus appear to be relatively stable over limited periods. However, considerable differences were found when comparing VEPs between participants. Further research may provide additional evidence that periodic, routine VEP testing is an effective way to monitor individuals who have shunted hydrocephalus in order to detect increased ICP before the appearance of obvious symptoms.

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.

Obstetric brachial plexus palsy (OBPP) is caused by traction to the brachial plexus during labour. In the majority of cases delivery of the upper shoulder is blocked by the mother's pubic symphysis (shoulder dystocia). If additional traction is applied to the child's head, the angle between the neck and the shoulder is forcefully widened, overstretching the ipsilateral brachial plexus. The resulting traction injury may vary from neurapraxia or axonotmesis to neurotmesis and avulsion of rootlets from the spinal cord. Recently, the exact origin of OBPP was again a matter of debate. It was suggested that intrauterine maladaptation, not nerve traction, causes the plexopathy. The incidence of OBPP varies from 1.6 to 2.9 per 1000 births in prospective studies. The upper brachial plexus is most commonly affected, resulting in paresis of the shoulder and biceps muscles, as first described by Erb and Duchenne. Hand function is additionally impaired in approximately 15% of patients; isolated injury to the lower plexus (Déjèrine-Klumpke's type) is rare.

This study examined the association between infant developmental milestones and educational level at 31 years of age in the Northern Finland 1966 Birth Cohort (n=12 058). Developmental data (age at standing, walking, speaking, and measures of bowel and bladder control) were gathered from children's welfare centres. Information on type of schooling at 14 years of age was reported by children and parents. School achievement at 16 years of age and educational level at 31 years were obtained from national registers. Those who reached infant developmental milestones sooner in their first year of life had significantly better (p<0.05) mean scores in teacher ratings at 16 years, and at 31 years they were more likely to have achieved a better educational level than slower developers. The adjusted odds ratios for individuals who developed more slowly to remain at a basic educational level (7 to 16y) ranged significantly from 1.1 to 1.3. The possibility of advancing from secondary to tertiary level was 1.4 times greater in faster developers than in slow developers. In conclusion, those who develop faster during their first year of life tend to attain higher levels of education in adolescence and adulthood.

Most neonatal seizures are occasional seizures and not true epilepsy. This study investigates seizure types of true neonatal epilepsies and their evolution with development. Seventy-five children with epilepsies of onset within 1 month of life, who were examined between 1970 and 1995, and whose seizure types could be confirmed with ictal EEG recordings, were studied. The patients were followed up for a minimum of 3 years and the evolution of epileptic syndromes was investigated. Sixty-three (84%) of 75 patients had partial seizures, while nine had generalized seizures, and only three had both generalized and partial seizures. Twenty-three of 24 neonates with benign familial or non-familial neonatal convulsions presented with partial seizures; these syndromes should not necessarily be categorized into generalized epilepsy as they are in the present International Classification. Age-dependent changes were a common feature of symptomatic neonatal epilepsies. Eighteen (41%) of 44 patients with symptomatic epilepsies of neonatal onset developed West syndrome in infancy. Fifteen (83%) of these 18 patients presented with symptomatic localization-related epilepsy in the neonatal period. In seven of these 15 patients, West syndrome was followed by localization-related epilepsy. Symptomatic localization-related epilepsy with transient West syndrome in infancy is another type of age-dependent epileptic syndrome.

Childhood craniopharyngiomas are histologically benign tumours arising from remnants of Rathke's pouch in the hypothalamic–pituitary region. The two common treatment approaches are primary total resection or limited resection followed by radiotherapy. To study the outcome after a primary surgical approach, we followed 25 consecutive patients (10 females, 15 males) under 16 years of age who were treated in a single institution with a management policy of radical tumour excision (mean age at diagnosis 9 years 2 months, SD 4 years 3 months; range 2 years 9 months to 15 years 11 months). Mean follow-up after primary surgery was 11 years 3 months (SD 7 years 7 months). Tumour control, and neurological, endocrine, and hypothalamic complications and their impact on health-related quality of life were assessed (medical follow-up, semi-structured interview, and questionnaires). Results of tumour control were generally good, however, local failure was observed in 6 of 25 patients, and severe late-treatment complications decreased quality of life for many long-time survivors. Endocrine deficiency occurred in 24/25, visual complications in 16/24, neurological complications in 8/24, obesity in 14/23, increased daytime sleepiness in 6/21, and significant school problems in 10/20. Patients with craniopharyngioma rated their health-related quality of life as considerably lower than healthy controls; the domains of social and emotional functioning were particularly affected. Parents' ratings were considerably lower than those of the patients. Poor functional outcome was associated with large tumours infiltrating or displacing the hypothalamus, the occurrence of hydrocephalus, and young age at diagnosis, but also with multiple operations due to tumour recurrence. Alternative treatment strategies should be considered, especially in very young patients with large tumours.

Paroxysmal kinesigenic choreoathetosis (PKC) is a rare movement disorder, characterized by recurrent, brief involuntary dystonic attacks that are provoked by sudden movements. Pathophysiology is uncertain, but a channelopathy is discussed. Treatment recommendations favour antiepileptic drugs (AEDs) acting on voltage-gated neuronal ion channels. This report summarizes the history of three children (6, 8, and 10 years of age) with idiopathic PKC successfully treated with low doses of lamotrigine, an AED acting primarily via neuronal voltage-sensitive sodium channels.

Foetal alcohol syndrome (FAS) was first described in 1973 by Jones and colleagues. Since then, results of epidemiological studies and clinical experience have shown that exposure of the foetus to high maternal alcohol consumption has a range of consequences. It can result in both major and minor malformations, permanent impairment of growth with a disproportionately low weight-to-height ratio and reduced head growth, and CNS dysfunction ranging from learning disability to specific learning disorders – all characteristics that can, however, have other causes. Effects within all domains can range from mild to severe. Effects seen in a child and the diagnosis reached depend on the duration of exposure to substantial maternal alcohol consumption during pregnancy, the range and timing of peak blood-alcohol levels, the nutritional state of the mother, and individual susceptibility.

Twenty-five children (21 males, 4 females; mean age 4.8 years) with attention-deficit-hyperactivity disorder (ADHD) participated in this study in addition to 25 typically developing children (21 males, 4 females; mean age 4.9 years). Parental ratings of the preschool children and preschool child self-ratings were examined within the framework of three domains: behavioral disturbance, social competence, and familial environment. Compared to their typically developing peers, preschool children classified as having ADHD were rated by their parents as significantly more aggressive, more demanding of parental time, less socially skilled, less adaptable to change in routine, and as exhibiting more non-compliance. In contrast to these parental ratings, preschool children with ADHD perceived themselves as equally competent, and as socially accepted as their peers. Parents of preschool children with ADHD rated themselves as less competent parents, and as experiencing a restricted parenting role. Although parenting a preschool child with ADHD was viewed as stressful, the parents did not rate general family functioning to be adversely affected.

Haemophagocytic lymphohistiocytosis (HLH) is characterized anatomically by an infiltration of multiple tissues with lymphocytes and haemophagocytic histiocytes. First symptoms are usually hepatosplenomegaly, pancytopenia, and intractable fever. Up to 73% of those with HLH develop CNS involvement during the disease course. The peculiarity of the two patients presented here, a 20-month-old Italian female and a 4-year-old Moroccan female, is that the initial presenting neurological symptoms mimicked an encephalitis, anticipating the typical systemic symptoms by 1 and 4 months. They developed progressive encephalopathy accompanied by status epilepticus, one child developed a secondary hydrocephalus. In both children it was not possible to detect an underlying infection or malignant disease and there were no other cases in the family that suggested a familial form of HLH. Diagnosis and initiation of treatment was delayed because of the initial encephalopathic clinical picture and the late onset of the typical systemic features. As early diagnosis allows better therapeutical approaches, haemophagocytic lymphohistiocytosis should be considered in children with persistent or progressive findings of encephalopathy, especially in the absence of identification of a plausible pathogen.

This study investigated the adaptive gait pattern in obstacle clearance in 12 normally developing (ND) children (six males, six females; mean age 10y 2mo, SD 10mo) and 12 children who had spastic diplegic cerebral palsy (CP) and who were independent ambulators (10 males, two females; mean age 13y 5mo, SD 2y 7mo). Children in both groups had 8 to 12 years of walking experience. They walked up to and crossed over obstacles of a height of 0%, 10%, and 20% of their leg length. Kinematic aspects of the three-dimensional leg movements were captured with the Vicon system and analyzed with one-way analysis of variance with repeated measure. Both groups increased foot clearance for higher obstacles. Children with CP were slower in approach and crossing speed, and used a wider base of support than the ND children when the obstacle height was increased. Results suggest that motor problems in obstacle clearance in children with CP lie in the implicit process of motor control, i.e. torque dynamics, not the explicit process, i.e. movement-match with the environmental constraints.

Two children who presented with symptomatic metabolic acidosis after being put on topiramate (TPM) are reported. The first patient was an 11-year-old male with refractory complex partial epilepsy who was put on TPM for 13 months. He developed hyperventilation 1 week after increasing the dose to 300mg/day. Arterial blood gas revealed hyperchloraemic metabolic acidosis with partial respiratory compensation: pH 7.36, PCO2 27.2 mmHg, bicarbonate 14.9 mEq/L, base excess –8.9 mmol/L. Hyperventilation and acidosis resolved after administration of sodium bicarbonate and reduction of the dose of TPM. The second patient was a female who developed increasing irritability at age 16 months and 21 months, each time associated with introduction of TPM and resolved promptly upon withdrawal of the drug. Venous blood gas taken during the second episode revealed pH 7.34, PCO2 37.4 mmHg, bicarbonate 20.4 mEq/L, base excess –4.2 mmol/L. The predominant mechanism of TPM-induced hyperventilation involves inhibition of carbonic anhydrase at the proximal renal tubule, resulting in impaired proximal bicarbonate reabsorption. The occurrence of hyperpnoea or mental status change in any patient who is on TPM should prompt an urgent blood gas sampling, with correction of the acid-base disturbances accordingly.