The purpose of this cross-sectional descriptive study was to describe the particular types of behavioral problems, self-concept, and symptoms of depression experienced by children with both low IQ and epilepsy. Three groups of children (83 males, 81 females; mean age 11 years 10 months, SD 1 year 10 months; age range 9 to 14 years) with epilepsy were compared: (Group 1) Low IQ (<85), n=48, 25 males, 23 females; (Group 2) Middle IQ (85 to 100), n=58, 24 males, 34 females; and (Group 3) High IQ (>100), n=58, 34 males, 24 females. The Child Behavior Checklist, Piers–Harris Self-Concept Scale, and Children's Depression Inventory were used to measure behavior, self-concept, and depression respectively. Results indicated that children in the Low IQ group had the most behavioral and mental health problems. Additionally, there were IQ group-by-sex interactions, with females in the Low IQ group being at the highest risk for poor self-concept. Findings suggest that children with both epilepsy and low IQ should be carefully assessed for mental health problems in the clinical setting.

This study investigated the occurrence, nature, and severity of speech, language, and cognitive impairment in 76 children (61 males, 15 females) with isolated sagittal synostosis (ISS) aged 9 months to 15 years 7 months. There was no increased prevalence of global cognitive impairment in the group but there was a high prevalence rate of speech and/or language impairment with 28 (37%) displaying impairment of whom 20 (71%) had moderate or severe impairments that fulfilled the criteria for specific impairments. Prevalence rates were only increased for children over two years of age. Expressive language impairment occurred most frequently. Raised intracranial pressure, peri-neonatal risk factors, otitis media, or being operated were not associated with impairment. Surgery at a later age and a family history of speech and language impairment were both associated with impairments but numbers were small. The findings suggest that children with ISS are at an increased risk of developing speech and language impairment.

The ambulatory prognosis of children with cerebral palsy (CP) remains a major issue for both their parents and the professionals concerned with their management. The question most frequently asked by the parents is: Will my child walk? Several studies have examined the prognostic criteria for ambulation in children with CP.

To understand the evolution of photosensitivity and to evaluate if its disappearance is related to the response to anticonvulsant therapy, we performed a long-term study of 42 patients (17 males, 25 females; age at onset 6 years 9 months, SD 5 years 2 months, range 5 years to 12 years 1 month) who had electroencephalography (EEG) evidence of photosensitive epilepsy. Of the patients, 36 were treated with valproate (VPA) monotherapy and four received VPA in combination with other antiepileptic drugs (AEDs), which were carbamazepine and lamotrigine. Two patients were given no drugs, but treated with stimuli avoidance. All patients were investigated with EEG by using intermittent photic stimulation. The photoparoxysmal response indicated the presence of photosensitivity. At the end of follow-up, the photoparoxysmal response had disappeared in 25 patients. Thirty-three patients became seizure-free. Our study confirms that photosensitive epilepsy has a good prognosis for seizure control that is independent of the persistence or disappearance of photosensitivity.

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 general aim of this study was to examine the stiffness and damping characteristics (rate at which oscillations diminish) of limbs of a small population of patients with spastic cerebral palsy (CP) during the Leg Drop Pendulum Test while under general anesthesia and to compare them with patients without CP, both awake and under similar anesthetized conditions. To facilitate a quantitative comparison the authors defined a set of parameters that reflect the stiffness and damping of limbs with spasticity under general anesthesia. To give these parameters more meaning a similar set was constructed for children without disabilities under conditions of normal wakefulness and under anesthesia. The test population of 10 patients with spasticity ranged in age from 4 to 19 years and had a mean age of 10 years 9 months (SD 5 years 10 months); there were four females and six males. The control population consisted of eight patients without disabilities who ranged in age from 7 to 19 years with mean age of 13 years 9 months (SD 3 years 3 months); there were six females and two males. To even out the populations, two limbs from the non-affected sides of two patients with hemiplegic spasticity (both female) were used in the control group. The Leg Drop Pendulum Test was administered to 10 limbs with spastic CP and 10 control limbs under general anesthesia. The two test populations were chosen from patients who were previously scheduled to undergo surgery. Two measures compared limbs of those under anesthetic with those under alert conditions: the damped natural frequency and the damping ratio. The natural frequency is the frequency at which a pendulum oscillates as it comes to rest; the damping ratio is a measure of how quickly the oscillations diminish. In spasticity, active muscle tone disappeared under anesthesia. In both groups the underlying biomechanics of the limb varied considerably. In controls the variation seemed to be growth related.

Preterm infants are at increased risk for cognitive disorders, including impairments in recognition memory. This study evaluated the effects of extreme prematurity on the neural pathway for auditory recognition memory using event-related potentials (ERPs), a neurophysiological technique widely used in cognitive neuroscience. ERPs were recorded at term postmenstrual age in 35 preterm infants born at less than 32 weeks' gestation (22 males, 13 females; mean birthweight ([BW] 1154g, SD 374g) with normal brain ultrasounds, compared with 40 healthy, term newborns (1 to 3 days of age; 20 males, 20 females; BW 3672g, SD 420g). Because infants must be able to detect and discriminate sounds before recognizing them, two paradigms were used to assess these functions. The first evaluated the detection and discrimination of speech sounds. The second tested recognition of the mother's voice compared with a stranger's. Results showed significantly different patterns of speech sound discrimination in preterm infants compared with term infants. No evidence of maternal voice recognition was elicited from the preterm infants. No specific patterns of auditory detection or discrimination were associated with patterns of recognition memory, suggesting that the function of multiple neural pathways may have been altered in this group of preterm infants. These results provide a functional corroboration of magnetic resonance imaging studies showing effects of prematurity on early brain development, even among preterm infants with normal cranial ultrasonography.

Unexplained crying in infants aged 1 to 3 months is a common concern for Western parents and health services. This study examined the hypothesis that the crying is due to high infant responsivity, and provides evidence about the types of stimulation that trigger crying in infants who present with high responsivity. The sample included 93 eight-day-old infants from a community sample (47 females, 46 males; mean birthweight 3457g; mean gestation 39.4 weeks; mean Apgar scores 8.28 at 1 minute and 9.59 at 5 minutes). Infants were tested for their response to two standard, mildly challenging, procedures: a neurobehavioural test involving undressing, putting down, and handling, and the Guthrie test, involving a painful heel prick to obtain a blood sample. The infants' crying over 24 hours was recorded in parental diaries. Newborn infants who exhibited high responsivity during the neurobehavioural assessment cried, rather than fussed, the most at home. High responsivity during the neurobehavioural assessment also predicted those infants who cried a lot and met a definition of ‘colic’ at home. The findings support the responsivity hypothesis and show that infants with high responsivity are upset by undressing, putting down, and sustained handling. Explanations for this and implications for the management of infant crying and colic are discussed.

Drooling is one of the more common associated dysfunctions in children with cerebral palsy (CP) and is perhaps the most problematic socially. In 1990, at the urging of the United Cerebral Palsy Research and Education Foundation, North American experts from a variety of related fields met in Charlottesville, Virginia, to address in a systematic way the subject of drooling. Based on the outcome of that gathering, known as the Consortium on Drooling, a review article was published in this journal, three instructional courses and one symposium were subsequently presented at AACPDM annual meetings, a number of local conferences and seminars have been generated, and several research projects have been undertaken. Thus, there has been considerable activity and some progress in the past 10 years but most of the fundamental issues still remain. The purpose of this annotation is to summarize briefly the new knowledge that has accrued, offer some personal insights based on experiences of the past decade and suggest a practical clinical approach to drooling management. For a systematic overview of salivary physiology, drooling, and the approach to diagnosis and treatment, the reader is referred to the 1992 review article.

Eighteen adolescents who had survived Reye syndrome (RS) in early childhood were assessed on cognitive, emotional, and behavioural variables in a second follow-up study tracking this group. Siblings were used as controls. The entire group with RS had survived with no obvious neurological damage at the first follow-up study. Indeed, current findings suggested that long-term cognitive, emotional, and behavioural functioning was comparable to siblings in approximately half of the group with RS. However, two factors were associated with a less favourable outcome. Cognitive, emotional, and behavioural functioning were significantly poorer in the subgroup of survivors whose illness had occurred in the first year of life. In addition, loss of consciousness, although the association with poor outcome was not as noticeable, was also associated with relative deficits on some scales of cognitive ability. Many of these deficits had not been obvious at the first follow-up and the importance of neurodevelopmental factors are considered. Finally, the implications of these findings for research and interventions in RS and other such encephalopathies are discussed.

This study investigated the effect of functional electrical stimulation (FES), applied during walking, on the gait of children with spastic cerebral palsy (CP). Eight children (five males, three females; mean age 13y 2mo, SD 2y 2m; range 8y 11mo to 17y 6mo) diagnosed with diplegic (n=6) or hemiplegic (n=2) spastic CP completed the study. All participants were ambulant. Core FES strategies based on common CP gait deviations were developed and tailored for each child. FES strategies for each child were evaluated in two separate test sessions. Effects of FES on gait were monitored with three-dimensional motion analysis. Within each test session each child's gait was assessed when walking without FES (phase A) and with FES (phase B). An A–B–A–B test sequence was employed allowing the effects of the withdrawal and reinstatement of FES to be assessed. All children performed 10 consecutive walks in each phase. Replication of this sequence on a separate day allowed the repeatability of the intervention to be evaluated. Outcome measures, including summary variables of kinematic data, temporal–spatial variables, and mode of initial contact, were predefined for each child and targets for clinical significance were set for these outcome measures. Comparisons were performed between these targets and the actual outcomes. Consistent clinically significant improvements were recorded for three children: one child showed some improvement that was statistically significant but not clinically significant. Results for one child were mixed. There was no change in the remaining three children. Gait analysis proved to be a useful tool in both developing and determining the effectiveness of FES strategies.

A cohort of 48 children with Noonan syndrome, with a mean age of 9 years 10 months (SD 3y 7mo; 28 males, 20 females), was recruited from a national study. Children were assessed using the Wechsler Intelligence Scales and Test of Motor Impairment–Revised (TOMI-R). The Piers–Harris Self-evaluation Questionnaire was also completed in a subgroup of age-appropriate children. Children's parents and teachers completed the Rutter A and B scales to assess of the degree of behavioural and emotional problems respectively. Mean Full-scale IQ score was 84, and one-quarter of the participants had learning disabilities. Verbal IQ tended to be slightly lower than Performance IQ. About half of the group showed evidence of mild to moderate impairment, confirming the impression of ‘clumsiness/developmental coordination disorder’ on the TOMI-R. Level of self-esteem, as determined by the Piers–Harris Questionnaire, was comparable to that of a standardized population. This research has identified some characteristic psychological features in Noonan syndrome. However, a specific behavioural phenotype could not be identified.

Lesch–Nyhan syndrome (LNS) is an X-linked recessive disorder resulting from a deficiency of the metabolic enzyme hypozanthine–guanine phosphoribosyltransferase (HPRT). This syndrome presents with abnormal metabolic and neurological manifestations including hyperuricemia, mental retardation, spastic cerebral palsy (CP), dystonia, and self-mutilation. The mechanism behind the severe self-mutilating behavior exhibited by patients with LNS is unknown and remains one of the greatest obstacles in providing care to these patients. This report describes a 10-year-old male child with confirmed LNS who was treated for self-mutilation of his hands, tongue, and lips with repeated botulinum toxin A (BTX-A) injections into the bilateral masseters. Our findings suggest that treatment with BTX-A affects both the central and peripheral nervous systems, resulting in reduced self-abusive behavior in this patient.