Enhanced EEG connectivity in children with febrile seizures.

BACKGROUND: Epilepsy is currently conceptualized as a disturbance of neuronal networks with altered connectivity that persists into the interictal phase. Febrile seizures are sometimes a precursor in childhood of lifelong epilepsy. We investigated whether studying functional connectivity in children with febrile seizures could help understand the mechanisms underlying their long-term seizure susceptibility. METHODS: EEG was recorded during rest and intermittent photic stimulation (IPS) in 12 FS patients, 5 siblings and 15 control children between 6 and 36 months of age. Original EEG data were transformed into source space using a multiple regional source model. Source coherence values were calculated for the interfrontal, interoccipital and occipito-frontal connections for the delta, theta, alpha, beta and gamma frequency bands. RESULTS: Our results suggest enhanced delta and theta frequency EEG source coherence in patients with FSs compared to siblings and control children, both under resting conditions and during IPS, more consistent for the theta band and the occipito-frontal connections. CONCLUSIONS: Enhanced connectivity in patients with FSs could indicate a seizure-prone state and interfere with the maturation of cerebral networks. Further prospective studies are needed to assess whether hyperconnectivity is a risk factor for epileptogenesis and neurodevelopmental disorders.

Cognitive disorders in pediatric epilepsy.

Childhood epilepsy may cause cognitive disorders and the intellectual quotient is indeed not normally distributed in epileptic children, a fair proportion of whom show an IQ in the deficient range. Some epileptic syndromes happen during vulnerability periods of brain maturation and interfere with the development of specific cognitive functions. This is the case for the Landau-Kleffner syndrome, which generally appears during speech development and affects language. Similarly, West syndrome - or infantile spasms - is an epileptogenic encephalopathy appearing during the first years of life and induces a major delay in social and oculo-motor development. Specific impairments can also be identified in partial childhood epilepsies in relation with seizure focus localization. For instance, left temporal and frontal epilepsies are frequently associated with verbal impairments. Moreover, episodic memory disorders have been described in children suffering from temporal lobe epilepsy whereas executive deficits (planning, self-control, problem solving) have been reported in frontal lobe epilepsy. In most cases, including its mildest forms, childhood epilepsy induces attention deficits, which may affect academic achievement. These observations militate in favor of individual neuropsychological assessments as well as early interventions in order to provide the child with an optimal individualized treatment program.

Reorganization of the auditory, visual and multimodal areas in early deaf individuals.

Plasticity resulting from early sensory deprivation has been investigated in both animals and humans. After sensory deprivation, brain areas that are normally associated with the lost sense are recruited to carry out functions in the remaining intact modalities. Previous studies have reported that it is almost exclusively the visual dorsal pathway which is affected by auditory deprivation. The purpose of the current study was to further investigate the possible reorganization of visual ventral stream functions in deaf individuals in both the auditory and the visual cortices. Fifteen pre-lingual profoundly deaf subjects were compared with a group of 16 hearing subjects. We used fMRI (functional magnetic resonance imaging) to explore the areas underlying the processing of two similar visual motion stimuli that however were designed to evoke different types of processing: (1) a global motion stimulus (GMS) which preferentially activates regions of the dorsal visual stream, and (2) a form-from-motion (FFM) stimulus which is known to recruit regions from both visual streams. No significant differences between deaf and hearing individuals were found in target visual and auditory areas when the motion and form components of the stimuli were isolated (contrasted with a static visual image). However, increases in activation were found in the deaf group in the superior temporal gyrus (BA 22 and 42) and in an area located at the junction of the parieto-occipital sulcus and the calcarine fissure (encompassing parts of the cuneus, precuneus and the lingual gyrus) for the GMS and FFM conditions as well as for the static image, relative to a baseline condition absent of any visual stimulation. These results suggest that the observed cross-modal recruitment of auditory areas in deaf individuals does not appear to be specialized for motion processing, but rather is present for both motion and static visual stimuli.

Children with a history of atypical febrile seizures show abnormal steady state visual evoked potential brain responses.

Atypical febrile seizures (FSs) are considered a risk factor for the onset of epilepsy in later life as well as for potential cognitive impairment. However, distinctive characteristics defining the group of children at risk for negative outcomes are not well established. In the following study, children from 6 to 59 months with a history of atypical FSs were investigated using steady state visual evoked potentials (ssVEP), a brain response known to increase with age. Abnormally, low theta and alpha ssVEP brain responses were found in children with a history of atypical FSs.

Functional reorganization of the auditory pathways following late callosotomy.

Injuries at various levels of the auditory system have been shown to lead to functional reorganization of the auditory pathways. In particular, it has recently been shown that such reorganization can occur in callosal agenesis. The pattern of cortical activity following callosotomy is however still unknown, but behavioral results suggest that it could be significantly different from that observed in callosal agenesis. We aimed to confirm this hypothesis by investigating fMRI responses to complex sounds presented binaurally and monaurally in a callosotomized patient. In the binaural condition, the callosotomized subject showed patterns of auditory cortical activation that were similar to those of neurologically intact individuals. However, in both monaural conditions, the callosotomized individual showed a significant increase of the asymmetries favoring the contralateral pathways. Such patterns of cortical responses are only partially consistent with the results obtained from callosal agenesis subjects using the exact same procedure. Indeed, the latter show differences compared with normals in both binaural and monaural conditions. These findings provide neurological evidence that callosotomy could lead to distinctive functional reorganization of the human auditory pathways.

Detection of hemodynamic responses to epileptic activity using simultaneous Electro-EncephaloGraphy (EEG)/Near Infra Red Spectroscopy (NIRS) acquisitions.

Simultaneous recordings of Electro-EncephaloGraphy (EEG) with Near InfraRed Spectroscopy (NIRS) allow measuring hemodynamic changes (changes in the concentration of oxy- and deoxyhemoglobin) at the time of epileptic discharges detected on scalp EEG. Two NIRS detection methods based on the General Linear Model (GLM) respectively in the time domain and in the time-frequency domain are investigated in this study using realistic simulations of spontaneous interictal epileptic activity. We evaluated the sensitivity at different Signal to Noise Ratios (SNR), the effect of either a large or a small number of discharges and the impact of model misspecification (e.g. omission or false detection of epileptic discharges). We also explored the effect on the estimation of key parameters, which set the model order. Simulations showed that both methods become inaccurate in lower SNR conditions, leading to many false positive detections. However, the time-frequency estimator showed better performance than the time-domain one. Key parameters for each algorithm were identified and results suggest to model confounds in the GLM differently for oxy- and deoxyhemoglobin. We also demonstrated that an inaccurate marking of epileptic events has a small impact on the detection statistics whereas an inaccurate specification of the hemodynamic response function delay decreases drastically the detection abilities. Finally, we illustrated the two methods on clinical EEG/NIRS data of one patient with focal epilepsy, showing an increase of regional Cerebral Blood Volume (rCBV) spatially concordant with the presumed epileptogenic focus.

Women process multisensory emotion expressions more efficiently than men.

Despite claims in the popular press, experiments investigating whether female are more efficient than male observers at processing expression of emotions produced inconsistent findings. In the present study, participants were asked to categorize fear and disgust expressions displayed auditorily, visually, or audio-visually. Results revealed an advantage of women in all the conditions of stimulus presentation. We also observed more nonlinear probabilistic summation in the bimodal conditions in female than male observers, indicating greater neural integration of different sensory-emotional informations. These findings indicate robust differences between genders in the multisensory perception of emotion expression.

Electrophysiological study of auditory development.

Cortical auditory evoked potential (CAEP) testing, a non-invasive technique, is widely employed to study auditory brain development. The aim of this study was to investigate the development of the auditory electrophysiological signal without addressing specific abilities such as speech or music discrimination. We were interested in the temporal and spectral domains of conventional auditory evoked potentials. We analyzed cerebral responses to auditory stimulation (broadband noises) in 40 infants and children (1 month to 5 years 6 months) and 10 adults using high-density electrophysiological recording. We hypothesized that the adult auditory response has precursors that can be identified in infant and child responses. Results confirm that complex adult CAEP responses and spectral activity patterns appear after 5 years, showing decreased involvement of lower frequencies and increased involvement of higher frequencies. In addition, time-locked response to stimulus and event-related spectral pertubation across frequencies revealed alpha and beta band contributions to the CAEP of infants and toddlers before mutation to the beta and gamma band activity of the adult response. A detailed analysis of electrophysiological responses to a perceptual stimulation revealed general development patterns and developmental precursors of the adult response.

Functional reorganization of the human auditory pathways following hemispherectomy: an fMRI demonstration.

The present study investigated the functional reorganization of ipsilateral and contralateral auditory pathways in hemispherectomized subjects. Functional reorganization was assessed using functional Magnetic Resonance Imaging (fMRI) and stimulation with complex sounds presented binaurally and monaurally. For neurologically intact control subjects, results showed that binaural stimulations evoked balanced activity in both hemispheres while monaural stimulations induced strong contralateral activity and weak ipsilateral activity. The results obtained from hemispherectomized subjects were substantially different from those obtained from control subjects. Specifically, activity in the intact hemisphere showed a significant decrease in response to contralateral stimulation but, concomitantly, an increase in response to ipsilateral stimulation. The present findings suggest that a substantial functional reorganization takes place in the auditory pathways following an early hemispherectomy. The exact nature of this functional reorganization remains to be specified.

Auditory processing in a patient with a unilateral lesion of the inferior colliculus.

The role of the inferior colliculus (IC) in human auditory processing is still poorly understood. We report here the results obtained with a 12-year-old boy (FX) who suffered a very circumscribed lesion of the right IC without additional neurological damage. The child underwent an extensive battery of psychophysical hearing tests. Results revealed normal peripheral auditory functioning, bilaterally. Furthermore, masking-level differences and frequency-pattern recognition were normal for each ear. When the right ear was stimulated, behavioural tests assessing central auditory processing yielded normal results. However, when the left ear was stimulated, speech recognition in the presence of a competing ipsilateral signal and duration-pattern recognition were impaired. Similarly, performance on two dichotic speech recognition tests was poor when the target stimulus was presented in the left and the competing signal in the right ear. Finally, sound-source localization in space was deficient for speakers located on the side contralateral to the lesion. The pattern of results suggests that auditory functions such as recognition of low-redundancy speech presented monaurally, recognition of tone duration patterns, binaural separation and integration, as well as sound-source localization in space, depend on the integrity of the bilateral auditory pathways at the IC level.

Electrophysiological markers of visuocortical development.

The development of noninvasive techniques for the assessment of functional brain maturation is critical. The present study analyzed 63 babies' and children's (27 days to 5.5 years) cerebral responses to a pattern-reversal visual stimulation using high-density (128 electrodes) electrophysiological recordings. Developmental data were further compared with those of young adults (n = 16). Tremendous changes in pattern visual evoked potentials (pVEPs) morphology were observed between 7 and 24 months characterized by the emergence of negative components labeled "N70" and "N145" and the reduction of the P100 amplitude. The adult pattern of response appears from 24 months onward. Spectral density values show an increase of higher frequencies with age. Coherence values show a reduction between 3 and 23 months of age as well as a further increase toward adulthood between areas implicated in visual processing. These results are discussed in light of developmental features such as synaptogenesis, myelination, and neuronal networks refinement.

Cross-modal reorganization and speech perception in cochlear implant users.

Recent work suggests that once the auditory cortex of deaf persons has been reorganized by cross-modal plasticity, it can no longer respond to signals from a cochlear implant (CI) installed subsequently. To further examine this issue, we compared the evoked potentials involved in the processing of visual stimuli between CI users and hearing controls. The stimuli were concentric circles replaced by a different overlapping shape, inducing a shape transformation, known to activate the ventral visual pathway in human adults. All CI users had their device implanted for >1 year, but obtained different levels of auditory performance following training to establish language comprehension. Seven of the 13 patients showed good capacities for speech recognition with the CI (good performers) while the six others demonstrated poor speech recognition abilities (poor performers). The evoked potentials of all patients showed larger amplitudes, with different distributions of scalp activations between the two groups. The poor performers exhibited broader, anteriorly distributed, high P2 amplitudes over the cortex whereas the good performers showed significantly higher P2 amplitudes over visual occipital areas. These results suggest the existence of a profound cross-modal reorganization in the poor performers and an intramodal reorganization in the good performers. We interpret these data on the basis of enhanced audiovisual coupling as the key to a long-term functional improvement in speech discrimination in CI users.

The effect of visors on head and facial injury in National Hockey League players.

There has been an increase in the number of concussions sustained by players in the National Hockey League (NHL). While wearing a helmet is now required by the NHL, the face visor remains optional. It is unknown to what degree face visors influence concussion, other head injury and eye-injury rates at the professional level. Data from the 2001-2002 NHL season were examined. It was found that wearing a face visor did not significantly influence the prevalence of concussion. Visor protection did, however, minimise eye-injuries and other, non-concussion head injuries. These data suggest that, while a visor may prevent some head and eye-injuries, other measures may be necessary to reduce the number of concussions.

Interaction between the flash evoked SSVEPs and the spontaneous EEG activity in children and adults.

OBJECTIVE: To evaluate the interaction between the steady-state visual evoked potentials (SSVEPs) recorded during the intermittent photic stimulation (IPS) and the spontaneous EEG activities both in children and adults. METHODS: EEG was recorded during the rest and under 5, 7.5, 10 and 12.5 Hz IPS in 41 children between 3 and 16 years and 10 adults. We distinguished between the spontaneous resting EEG spectra, SSVEPs (1st harmonic) and undriven (ongoing) EEG spectra recorded during the IPS. RESULTS: We show that IPS influences spontaneous EEG activity by specifically suppressing or desynchronizing individual posterior dominant resting EEG frequencies (DF) in both children and adults. Further, this highly significant and consistent suppressing effect positively correlates with the SSVEPs amplitude. CONCLUSIONS: Our data suggest that the desynchronization of the spontaneous EEG activity under IPS and the SSVEPs are related to each other. SIGNIFICANCE: These relationships could be interesting to study in pathological conditions where the neural synchronization and the responses to IPS have been shown to be affected, such as epilepsy and schizophrenia.

Impact of childhood epilepsy on reading and phonological processing abilities.

Although children with epilepsy tend to exhibit more reading difficulties than their classmates, no systematic studies have investigated the relationship between these difficulties and epilepsy. As functional neuroimaging studies have implicated both temporal and frontal lobes in the phonological aspect of reading [K.R. Pugh, B.A. Shaywitz, S.E. Shaywitz, et al. Brain 1996;119:1221-38], seizure activity originating in either region could interfere with phonological processing, whereas generalized seizures would not disturb this function as much. To explore this hypothesis, we compared the metaphonological skills of school-aged children with either temporal lobe epilepsy (TLE), frontal lobe epilepsy (FLE), or generalized absence seizures (ABS) with those of healthy controls. While the reading ability of all epileptic children was close to 2 years behind expectations, children with TLE did not differ from the controls on phonological tasks. In contrast, children with FLE exhibited significant deficits, whereas children with ABS showed difficulties restricted to phonemic segmentation. The results suggest that FLE and, to a lesser extent, generalized seizures may interfere with phonological processing, whereas TLE may affect other aspects of reading.

Blind subjects process auditory spectral cues more efficiently than sighted individuals.

The goal of the present study was to investigate how monaural sound localization on the horizontal plane in blind humans is affected by manipulating spectral cues. As reported in a previous study (Lessard et al. 1998), blind subjects are able to calibrate their auditory space despite their congenital lack of vision. Moreover, the performance level of half of the blind subjects was superior to that of sighted subjects under monaural listening conditions. Here, we first tested ten blind subjects and five controls in free-field (1) binaural and (2) monaural sound localization tasks. Results showed that, contrary to controls and half the blind subjects, five of the blind listeners were able to localize the sounds with one ear blocked. The blind subjects who showed good monaural localization performances were then re-tested in three additional monaural tasks, but we manipulated their ability to use spectral cues to carry out their discrimination. These subjects thus localized these same sounds: (3) with acoustical paste on the pinna, (4) with high-pass sounds and unobstructed pinna and (5) with low-pass sounds and unobstructed pinna. A significant increase in localization errors was observed when their ability to use spectral cues was altered. We conclude that one of the reasons why some blind subjects show supra-normal performances might be that they more effectively utilize auditory spectral cues.

Unitary attention in callosal agenesis.

The interhemispheric organisation of two specific components of attention was investigated in three patients affected by partial or complete agenesis of the corpus callosum. A visuospatial component of attention was explored using a visual search paradigm in which target and distractors were displayed either unilaterally within a single visual hemifield, or bilaterally across both visual hemifields in light of prior work indicating that split-brain patients were twice as fast to scan bilateral displays compared to unilateral displays. A central component of attention was explored using a psychological refractory period (PRP) paradigm in which two visual stimuli were presented laterally at various stimulus onset asynchronies (SOAs), with each stimulus associated with a different speeded two-alternative choice task. The stimulus-response compatibility in the second task was systematically manipulated in this paradigm, in light of prior work indicating that split-brain patients exhibited a close-to-normal PRP effect (i.e., slowing of the second response as SOA is decreased), with, however, abnormally decreasing effects of the manipulation of the response mapping on the second task speed as SOA was decreased. The present results showed that, although generally slower than normals in carrying out the two tasks, the performance of each of the three acallosal patients was formally equivalent to the performance of a matched control group of normal individuals. In the visual search task, the search rate of the acallosal patients was the same for unilateral and bilateral displays. Furthermore, in the PRP task, there was more mutual interference between the lateralised tasks for the acallosal patients than that evidenced in the performance of the matched control group. It is concluded that the visuospatial component and the central component of attention in agenesis of the corpus callosum are interhemispherically integrated systems.

[Neuropsychological profile of intractable partial epilepsies]. / Le bilan d'une épilepsie partielle pharmaco-résistante: explorations neuropsychologiques.

The neuropsychological assessment is an essential part of the clinical work-up of patients suffering from pharmaco-resistant epilepsy. The aim of the present article is to give an overview of the assessment with regard to the functions tested, the techniques employed and the factors influencing the interpretation of the results. In the first part of the article, the role of the neuropsychological evaluation is discussed in relation to other methods of clinical investigation, such as EEG, brain imaging techniques and sodium amytal exploration, all of which intend to identify the localization (region and laterality) of the epileptogenic focus. This is particularly important for patients slated for epilepsy surgery. In addition, the neuropsychological exam aims to determine the extent of the deficits in various cognitive and sensori-motor domains by taking into account the structural and/or functional reorganization that may have taken place and the compensatory mechanisms that are available to the patient. In this context, the effect of various factors influencing the mental and emotional functioning of the patient, such as the age at onset and duration of the epilepsy, the presence of a structural lesion, the type and number of anticonvulsant drugs, the personality and the adaptability of the patient must be considered. In children, the effect of recurrent seizures may be more devastating since these risk to interfere with normal brain development. On the other hand, the greater plasticity of the immature brain provides a larger window in which compensation can take place. Evidence suggests that attentional processes are affected in all types of epilepsy. Furthermore, the antiepileptic medication may have a deleterious effect on the cognitive and emotional status of the patient, particularly in patients with pharmaco-resistant epilepsy who are usually taking a combination of anticonvulsant drugs. The second part of the article presents localization-specific neuropsychological impairments and the tests most suited to reveal these deficits. Thus, in temporal lobe epilepsy, memory functions are more affected than in other types of partial epilepsy, whereas intellectual functions are usually preserved. However, recent studies cast some doubt on the prevailing view that left-hemisphere foci are associated with verbal memory deficits only and that right-hemisphere foci solely affect visuo-spatial memory. The dichotomy appears to be more subtle, especially in patients with a long-standing seizure history. Frontal foci produce deficits in motor coordination, attention, working memory and executive functions. These deficits appear to be more pronounced in children than in adults. Few studies have explored the neuropsychological consequences of parieto-occipital foci given that this type of epilepsy is relatively rare. The neuropsychological profile of the patient should guide healthcare providers to implement the necessary remedial measures to facilitate the patient's social integration and to improve his quality of life.

Impact of temporal lobe epilepsy on phonological processing and reading: a case study of identical twins.

In order to evaluate the possible consequences of temporal lobe epilepsy on reading acquisition, we first compared the reading skills and phonological awareness abilities in a set of 13-year-old identical twins, one of whom is affected by temporal lobe epilepsy (LB). We then compared their performances to those of an age- and IQ-matched control group. Both siblings have an intellectual quotient above average as well as normal memory and linguistic abilities. Results showed that the reading age of LB (assessed by the Lefabvrais French reading test) was more than two years behind expectations whereas that of her sister was above average. Further, in contrast to her sister and healthy control subjects, LB exhibited specific deficits in elaborate metaphonological awareness abilities (non-word repetition, rhyme production, phonemic segmentation and syllabic inversion). These could be linked to temporal lobe dysfunction, thus confirming the important role of the temporal lobes in reading acquisition.

Hyperbaric oxygen for children with cerebral palsy: a randomised multicentre trial. HBO-CP Research Group.

BACKGROUND: The use of hyperbaric oxygen for children with cerebral palsy has spread worldwide, despite little scientific evidence of efficacy. We did a randomised trial to assess the efficacy and side-effects of this form of therapy in children with cerebral palsy. METHODS: 111 children with cerebral palsy aged 3-12 years were randomly assigned hyperbaric oxygen (n=57) or slightly pressurised room air (n=54). All children received 40 treatments over 2 months. Hyperbaric oxygen treatment was 1 h in 100% oxygen at 1.75 atmospheres absolute (ATA); children on slightly pressurised air received air at 1.3 ATA (the lowest pressure at which pressure can be felt, thereby ensuring the maintenance of masking). The main outcome measure was gross motor function. Secondary outcomes included performance in activities of daily living, attention, working memory, and speech. FINDINGS: For all outcomes, both groups improved over the course of the study, but without any difference between the two treatments. The score on the global gross motor function measure increased by 3.0% in the children on slightly pressurised air and 2.9% in those on hyperbaric oxygen. The mean difference between treatments was -0.40 (95% CI -1.69 to 0.90, p=0.544). Other changes were seen in speech, attention, memory, and functional skills. Ear problems occurred in 27 children treated by hyperbaric oxygen and in 15 treated with hyperbaric air (p=0.004). INTERPRETATION: In this study, hyperbaric oxygen did not improve the condition of children with cerebral palsy compared with slightly pressurised air. The improvement seen in both groups for all dimensions tested deserves further consideration.