Aicardi's presentation on epilepsy at the 1998 International Child Neurology Association conference in Ljubljana, Slovenia, started dramatically by using the iceberg, with larger amounts of ice below the surface, as an analogy for epilepsy. He then talked about the phenomena associated with epilepsy - the learning and behaviour problems. Beckung and Uvebrant titled their 1997 paper on epilepsy ‘Hidden dysfunction in childhood epilepsy’. ‘Hidden’ and ‘under the surface’ maybe, but hidden from whom? I suspect young people with epilepsy and their parents have been well aware for many years of some of these associated problems. But health professionals presented with children with epilepsy have concentrated too much on the convulsive episode itself and ignored the broader spectrum.

Neuropsychological and behavioral status were examined in 57 children aged 7 to 16 years with complex partial seizures (CPS) and compared with 27 sibling control children of the same age. Epilepsy had a significant effect on both cognitive and behavioral adjustment measures. Children with CPS had significant impairment across all seven cognitive domains assessed, reflective of a profile of relatively diffuse and generalized cognitive dysfunction. Age at onset of recurrent seizures was the strongest and most consistent predictor of adequacy of cognitive functioning; earlier age at onset was associated with poorer cognitive status. Children with CPS also had more problems compared with sibling control children on measures of social and school competence and internalizing behavior problems, but not externalizing behaviors. Further, frequency of seizure activity in the past year, rather than age at seizure onset, emerged as the strongest predictor of these behavioral difficulties. These findings are discussed in the context of understanding the impact of CPS on cognition and behavioral adjustment, and identifying the contribution of various aspects of the neurodevelopmental course of CPS to these issues.

Attempts at subtyping attention-deficit–hyperactivity disorder (ADHD) along the hyperactivity dimension are considered controversial. This study addresses this issue by dividing a non-clinical sample of Brazilian children (mean age, 9.4 years; SD, 2.9), who were attending a mainstream school in the Greater Rio de Janeiro area, into four behavioral domain groups (normal [NO, N=324], hyperactive/impulsive [HI, N=17], inattentive [IA, N=48], and combined [C, N=13]) on the basis of teacher ratings on an ADHD scale. The groups did not differ in intellectual level as determined by the Human Figure Drawing test. Comparisons were made between groups along the factorial dimensions extracted from the Composite Teacher Rating Scale, academic performance and neuropsychological measures were then performed. Our data showed that IA and C children are less independent and more prone to socialization problems than NO children, and that HI and C children are less anxious and fearful than IA children. Furthermore, the groups differed in academic and neuropsychological performance. The results could be considered consistent with the hypothesis that ADD with hyperactivity (ADD/+) and ADD without hyperactivity (ADD/−) represent singular nosological entities.

To test the validity of a new computerized task to assess children's receptive vocabulary, event-related potentials (ERPs) were recorded from 56 typically developing children ranging in age from 5 to 12 years. This ERP-computerized vocabulary task does not require a child to give a verbal or motor (i.e. pointing) response. Single pictures, from an existing standardized test of receptive vocabulary, were presented on a computer screen and simultaneously named either correctly (congruent) or incorrectly (incongruent) via a computer. As predicted, the N400 amplitude was found to be significantly higher to the incongruent picture–word pair (i.e. the child knew it was an incorrect pairing) than to the congruent picture–word pair (i.e. the child knew it was a correct pairing). This effect was found for each of the four age groups (5 to 6 years, 7 to 8 years, 9 to 10 years, 11 to 12 years). This task accurately estimated current receptive vocabulary in typically developing children. Although still in the development stage, it may eventually serve as an adjunct to a thorough neurological and neurodevelopmental assessment of some children presenting with moderate to severe cerebral palsy.

Although ankle–foot orthoses (AFOs) are frequently prescribed to correct skeletal malalignment in children with spastic diplegia, their effect on standing balance abilities has not been documented. This study investigated balance differences related to the presence of pathology and orthotic conditions during conditions of unexpected stance perturbation by comparing four children aged between 3½ and 15 years with spastic cerebral palsy and four control children matched for years of independent walking experience. Electromyographic and kinematic data were collected and compared between groups and in three orthotic conditions (no AFOs, solid AFOs, dynamic AFOs). Results revealed that balance responses of children with spasticity were characterized by: increased coactivation of muscles as opposed to distal to proximal recruitment, decreased presence of upright posture in stance, increased use of ‘on-toes’ strategies, and different sway characteristics compared with the typically developing children. In both groups of children, the use of solid AFOs during perturbed stance resulted in: decreased activation of gastrocnemius muscles, disorganized muscle-response patterns, decreased use of ankle strategies, and increased joint angular velocities at the knee compared with conditions without AFOs or with dynamic AFOs. These preliminary results support the use of dynamic AFOs to correct skeletal malalignment in children with spastic diplegia.

The aim of this study was to compare the linear growth of children with cerebral palsy (CP) with that of children without CP. The segmental lengths (humerus, ulna, femur, tibia, and spine), recumbent length, body weight, and bone age of 62 children with CP (age range 2.25 to 14 years, mean 7.13 years) were measured and compared with 68 children without CP (age range 1.50 to 12.67 years, mean 6.73 years). The results show that bone-age delay is common in children with CP (68% with a delay of more than 1 year). The linear growth of children with CP is similar to that of children without CP when bone age is used instead of chronological age. There is a strong correlation between segmental lengths and body height. Hence, this study favours using segmental lengths for estimating height in children with CP and using the comparison group as a reference for this purpose. Nutritional and non-nutritional factors on bone-age delay are discussed.

The development of postural adjustments during reaching movements was longitudinally studied in seven infants with cerebral palsy (CP) between 4 and 18 months of age. Five infants developed spastic hemiplegia, one spastic tetraplegia, and one spastic tetraplegia with athetosis. Each assessment consisted of a simultaneous recording of video data and surface EMGs of arm, neck, trunk, and leg muscles during reaching in various lying and sitting positions. The basic organization of postural adjustments of the children developing spastic CP was intact. Their main problem was a deficient capacity to modulate the postural adjustments to task-specific constraints – a deficit which was attributed to a combination of an impaired motor coordination and deficits in sensory integration. The child with spastic-dyskinetic CP showed distinct abnormalities in the basic organization of postural adjustments.

A 5-year old girl with cerebral palsy (CP), preterm birth, postnatal aortic thrombus, and cerebellar venous infarction who is homozygous for the thrombophilic factor-V Leiden (fVL) mutation is reported. The role of hereditary thrombophilic disorders in the development of perinatal vascular lesions such as aortic thrombi, renal-vein thrombosis, venous-sinus thrombosis, and cerebral infarction is unknown. This case report brings into question a potential association between fVL, perinatal vascular lesions, perinatal stroke, and CP.

Inherited and acquired thrombophilic disorders have recently been associated with cerebral palsy (CP) and complications in pregnancy. Thrombophilic disorders are not diseases per se, but are recognized risk factors for a variety of diseases across all ages, including atherosclerosis, cerebral infarction, inflammatory bowel disease, Legg–Perthes disease, deep venous thromboses, and preeclampsia. In healthy individuals, the balance between procoagulant and anticoagulant mechanisms is exquisitely regulated, protecting against both excessive bleeding and excessive clotting (thrombophilia). This annotation is intended to be a broad overview of thrombophilia, with special attention to the factor-V Leiden (fVL) mutation, an autosomal dominant point mutation resulting in resistance to activated protein C (APC). This thrombophilic disorder has wide clinical implications for the management of carrier women and their offspring, as illustrated by the accompanying case report.

Episodic dyscontrol syndrome may affect both children and adults. Those who treat children with neurological disorders are likely to see patients referred for investigation of outbursts of aggressive behaviour, especially if episodes are possibly of an epileptic nature. This may occasionally be the case, but in some children it seems that their threshold to such behaviour is abnormally low, and this may also result from defects in the biochemistry or structure of the brain. There is nothing calculated about this behaviour, and it cannot be explained by justifiable anger, such as the result of frustration. Boys are affected more frequently than girls, and the syndrome is more often seen among urban than rural populations. Such outbursts can occur in isolation, but also after a series of minor episodes such as the crying of a younger sibling for nights on end. The outbursts may be very brief or last several hours.