One-hundred and fifty-seven vertically infected HIV-1 positive infants (85 males, 72 females) underwent longitudinal assessment to determine whether early neurodevelopmental markers are useful predictors of mortality in those infants who survive to at least 4 months of age. Survival analysis methods were used to estimate time to death for quartiles of 4-month scores (baseline) on the Bayley Scales of Infant Development (BSID). Cox proportional hazards progression was used to estimate relative hazard (RH, 95% CI) of death for BSID scores and potential confounders. Thirty infants with BSID scores at 4 months of age died during follow-up. Survival analysis revealed greater mortality rates in infants with BSID (Mental Developmental Index and Psychomotor Developmental Index) scores in the lower quartile(p=0.004, p=0.036). Unadjusted univariate analyses revealed increased mortality associated with baseline CD4+ 29%, gestational age <37 weeks, smaller head circumference, advanced HIV and higher plasma viral load. BSID scores independently predicted mortality after adjusting for treatment, clinical category, gestational age, plasma viral load and CD4+ percentage.

Hemispatial neglect is commonly observed in adults following right-hemisphere brain lesions. Given the potential for reorganization in the developing brain, spatial neglect may not be apparent following early unilateral damage. This study aimed to determine whether infants who experienced pre- or perinatal focal brain lesions showed evidence of spatial neglect. Study participants were 33 infants/toddlers (22 males, 11 females; age range 6 to 48 months); 27 preschool children (14 males, 13 females; age range 28 to 75 months) with either left hemisphere (LH) or right hemisphere (RH) damage of pre- or perinatal onset (total 60), and 36 control individuals (15 females, 21 males; age range 28 to 75 months). Participants were assessed using two object-removal preference tasks. Control children showed no lateralized preference for object removal. Those with LH or RH damage preferentially removed objects from the side of the board ipsilateral to the lesion first and the contralateral side last. These results suggest that spatial neglect may be found in young children even after very early unilateral brain damage. Further, in contrast to the adult pattern, contralateral neglect is present for up to 6 years after either LH or RH damage. This pattern suggests that there may be a different distribution of attention to space in the developing brain as compared with the mature brain. The persistence of spatial neglect suggests that there are some limitations on plasticity in the developing human brain.

Cervical accelerometry with digital signal processing (DSP) can identify signals that are consistently associated with swallowing during feeding of infants. It is shown that these signals, called initial discrete sounds (IDSs), become more uniform with advancing postmenstrual age (PMA) in healthy preterm infants. The objectives of this study were to determine if cervical accelerometry and DSP at a higher sample rate than previously used (22kHz versus 16kHz), in conjunction with a DSP software package that allows manipulation of the sound files, would improve the accuracy of the previously developed variance index (VI) method of analyzing accelerometric information. The modified VI method was then used to measure developmental differences in the IDS morphology of infants with and without bronchopulmonary dysplasia (BPD). VIs were calculated for 24 feeding studies of infants of between 32 and 39 weeks PMA: 12 studies on healthy preterm infants (n=10: three males, seven females; mean gestational age [GA] 28.6 weeks, SD 0.4; mean birthweight [BW] 1080g, SD 82; PMA mean 35.2 weeks, SD 0.6) and 12 studies on infants with BPD (n=7: five males, two females; GA 27.1 weeks, SD 0.4; BW 911g, SD 71; PMA 36.2 weeks, SD 0.6). There was a significant inverse correlation between VI and PMA for the healthy preterm group (r=0.66, m=–2.31/week, p=0.02). There was no significant correlation between VI and PMA for the BPD cohort. The VI of infants with BPD was significantly different from that of infants without BPD (p<0.007, multiple regression analysis, interaction PMA×Group). Additionally, using our modified VI technique, 100% of swallows were found to have the expected IDS signals.

In patients with focal epilepsy, focal neurological dysfunction can occur due to status epilepticus and also as a post-ictal phenomenon. Bulbar dysfunction as evident by drooling, dysarthria, swallowing difficulties, and palatal–glossalpharyngeal weakness has been reported in conjunction with epilepsy. This is non-progressive and is correlated in its severity with the frequency of seizures. Accompanying EEG discharges are often localized to rolandic areas that cortically represent oral movements and salivation. We report a 6-year-old male and a 6½-year-old female with progressive bulbar dysfunction resulting from epilepsy. Ictal EEGs in patient 1 did not confirm a diagnosis of epilepsy. With no evidence of a cortical or brainstem focus from EEG or MRI, it is very difficult to explain the mechanism of bulbar dysfunction. The complete restoration of bulbar function after treatment with antiepileptic drugs demonstrates the need to consider epilepsy in similar clinical situations.

The aim of this study was to evaluate anaerobic muscle performance in children after long-term recovery from Guillain-Barré syndrome (GBS). Participants were recruited from all children and adolescents with GBS admitted to The Hospital for Sick Children, University of Toronto, Canada, between 1988 and 1995. We assessed 17 children (nine males, eight females; mean age at GBS diagnosis 7 years 11 months, SD 4 years 2 months; mean age at testing 14 years 1 month) more than two years after acute GBS. Participants completed the Wingate anaerobic test to evaluate mean and peak muscle power. For mean arm muscle power (mean 47.5% [SD 17.4%] of normal), only two participants were in the normal range. For mean leg muscle power (mean 83% [SD 28.3%] of normal), 15 participants were in the normal range. For arm peak power (mean 92.6% [SD 20.7%] of normal), 12 participants were in the normal range. For leg peak power (mean 116.3% of normal, SD 15.8%), all participants were in the normal range. Detailed manual muscle testing with the modified Medical Research Council scale was done on 34 muscle groups. Data were pooled to give a mean arm and leg muscle strength score (9.7, SD 0.30 and 9.4, SD 0.50 respectively). Pearson's correlations of muscle endurance with arm and leg strength were not significant. Children with GBS have excellent long-term recovery of peak muscle power. Muscle endurance, measured by mean muscle power, was normal in the legs but markedly low in the arms. Pediatric rehabilitation programs after GBS should specifically target endurance of arm muscles.

Patients with congenital varicella syndrome (CVS) typically present with clinical symptoms consisting of skin lesions, neurological defects, eye diseases, and/or limb hypoplasia. In rare cases, isolated manifestations in the brain or eye have been reported. The varicella-zoster virus (VZV), as the causative agent of CVS, could only be detected in a few infants with CVS. In addition, there is little in the literature on antiviral treatment of infants born with signs of CVS. We report a case of CVS in a male infant who presented with generalized clonic cerebral seizures at age 4 months. An endogenous intracerebral viral reactivation following intrauterine VZV infection was assumed. After the diagnosis was confirmed virologically, acyclovir was administered intravenously for 10 days and afterwards orally for 3 weeks. This antiviral treatment was aimed at preventing progression of the disease. We concluded from this case that infants with intrauterine VZV infection can suffer intracerebral VZV reactivations that require antiviral treatment.

Autism is a neurodevelopmental disorder with strong genetic influences. Clinical experience and limited empirical evidence support the view that autism may be associated with aberrant interhemispheric information transfer. This empirical controlled study examined whether, at neuropsychological testing, children with autism showed problems with interhemispheric information transfer. The study included auditory, visual, and motor measures covering information transfer within, as well as across, modalities. Thirty children (24 males, 6 females; mean age 12 years 8 months, SD 2 years 8 months; range 9 years 5 months to 17 years 5 months) without learning disability but with autism spectrum disorders were compared with 30 children from a mainstream school matched for age, sex, and IQ>75. Children with autism spectrum disorder performed significantly worse than the comparison group on most of the tests (p=0.02 for auditory perception and attention, p=0.005 for visual perception, p=0.0001 for motor control, p=0.04 for tactile perception). Results support the notion that aberrant interhemispheric transfer may be involved in the pathogenesis or clinical course of autism. The findings were not accounted for by lower IQ in the group with autism.