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Nouveautés en épileptologie infantile : qu'en faire ?
How to evaluate a child following a first seizure?
Pascal Sabouraud
may correspond to undiagnosed seizures. It is important to inquire about the existence of subjective sensations preceding or initiating attacks, about postictal phenomena such as confusion or drowsiness and about the circumstances of occurence of the seizures.
Clinical examination should look for any neurological signs, cutaneous stigmata or dysmorphic features. Parents should be asked about any recent changes in behaviour or school performances.
Interictal, and eventually ictal, EEG data must be interpreted within the general neurological context. In the majority of cases, careful analysis of all the above mentionned data leads to a diagnostic hypothesis on the type(s) of seizures and, sometimes, on the epilepsy syndrome. Indications for further investigations, including MRI, will be discussed on the basis of a precise hypothesis.
Treatment with antiepileptic drugs should not usually be started at the first seizure, with the exception of some epilepsy syndromes characterized by regular seizure relapse.
From historical EEG to signal treatment: applications in childhood epilepsy
Edouard Hirsch, Philippe Derambure
of epileptic syndromes is based on electroclinical correlations. The characterization of EEG abnormalities is essential for establishing a specific syndromic diagnosis.
Discussing the historical evolution of EEG techniques we show how the recent development of numerized EEG provided the medical community with very useful and simple tools for the treatment of EEG signals. These tools are now integrated in everyday clinical practice. Several methods are presented, illustrating potential applications in childhood epilepsies.
Video-EEG monitoring in neonates: indications
Nathalie Bednarek
a more precise analysis of clinical semiology of seizures; it may help in the localization of a seizure focus; it provides more comprehensive data for the evaluation of the interictal state.
Cerebral imaging in childhood epilepsy: what's new?
Catherine Chiron, Lucie Hertz-Pannier
spatial resolution improved, so it is possible to detect subtle epileptogenic lesions, even in very young patients. By visualizing more and more accurately the sulcogyral macroanatomy, MRI permits a genetic and syndromic approach of cerebral lesions associated with epilepsy. Ictal SPECT helps localizing the epileptogenic zone and placing intracranial EEG electrodes, using strict methodology with simultaneous video-EEG and multidisciplinary interpretation of the images. Functional MRI can localize motor cortex and language networks, as well as it can lateralize language with a good correlation with WADA test, using selected activation tasks in cooperative children. Interictal SPECT and PET show the dysfunctioning areas involved in the neuropsychological and behavioural disorders associated with epilepsy. Finally, fMRI may study post-lesional and post-surgical plasticity by comparing longitudinal studies in a given patient.
New antiepileptic drugs in childhood epilepsies: indications and limits
Anna Kaminska
However, the therapeutic profile of the most recent compounds is still not completely established in children because of the high number of syndromes and the difficulty to perform controlled studies in this age range. Controlled studies are most often first performed in adults and they begin in children whereas the new drug is already approved. However, some new AEDs dramatically improved seizure control, particularly in some severe epilepsy syndromes such as West syndrome and Lennox-Gastaut syndrome.
Vigabatrin demonstrated a remarkable efficiency in infantile spasms whereas it tends to worsen myoclonic epilepsies, absence epilepsy and idiopathic partial epilepsy. Lamotrigine is efficient in absence epilepsy and symptomatic or cryptogenic generalized epilepsies such as Lennox-Gastaut syndrome and myoclonic astatic epilepsy. By contrast, lamotrigine increases the frequency of seizures in severe myoclonic epilepsy in infancy (Dravet syndrome) as well as in some cases of idiopathic partial epilepsy. Felbamate remains indicated as third line drug in refractory Lennox-Gastaut syndrome provided blood parameters are controlled. The therapeutic profile of oxcarbazepine is closed to that of carbamazepine. The efficacy of topiramate was demonstrated in partial epilepsy, but the other indications remain to be précised. Pediatric studies using gabapentin and tiagabine disclosed encouraging results in partial epilepsy. Clinical trials with stiripentol represent an example of strategy for developing a new AED in children; it recently demonstrated, in association with clobazaru, efficacy in a severe myoclonique epilepsy in infancy.
Epilepsies et difficultés d'apprentissage
Epilepsy and childhood learning disabilities
Jacques Motte
cognitive functions in children with epilepsy. Several neuropsychological studies have been carried out but only on epileptic syndromes as West syndrome and Lennox-Gastaut syndrome. It looks very important to study the neuropsychological profiles in the different types of epilepsy in childhood (generalized and partial epilepsy, idiopathic and non idiopathic). Different tools are now available for detection of cognitive deficits.
Neuropsychology and academic achievement of epileptic children: executive-functions tests
Patrice Gillet, Dominique Sauvage, Catherine Billard
a usefull assessment tool enabling identification of the structural disturbances that will affect the learning process. For school-age children, assessment can be made in regard to the DSM IV criteria of specific learning disabilities, and within a neuropsychological framework that pays a particular attention to the executive functions.
Cognitive consequences of Rolandic Epilepsy
Anne de Saint-Martin, Caroline Seegmuller, Roberta Carcangiu, Catherine Kleitz, Edouard Hirsch, Christian Marescaux, Marie-Noëlle Metz-Lutz
syndrome in 1959 by Beaussart and Nayrac was a great progress for childhood epileptology: the existence of a focal epilepsy with a good prognosis and no underlying brain lesion, so-called "benign" epilepsy, was recognized. Since the first descriptions, numerous neuropsychological studies were performed showing variable results. All the studies agree with the fact that children with rolandic epilepsy keep a normal global intellectual efficiency and a good long-term outcome. Nevertheless, some children may suffer transiently during the active phase of the epilepsy from oromotor dysfunction, neuropsychological deficits, or attention deficits with learning disorders. The analysis of cognitive and neurophysiological correlations evidenced a significant correlation between the epileptic focus localization and few specific dysfunctions. We evidenced mainly a significant effect of the persistence of a prolonged slow focus and a strong activation of night EEG spike and waves on cognitive decline and attention disorders. These abnormalities are mainly observed during severe or atypical evolutions of rolandic epilepsy. Preliminary longitudinal studies show that these cognitive deficits are transient. Thus, the presence of an active epileptic focus, without underlying brain lesion, could interfere with normal maturation of cognitive function.
Are idiopathic generalized epilepsies of childhood really benign?
Bernard Echenne, Renée Cheminal, Agathe Roubertie, François Rivier
school difficulties observed in children with idiopathic generalized epilepsies, especially absence epilepsies, myoclonic epilepsies considered as non-severe, and generalized epilepsies with tonico-clonic seizures.
In a controlled study concerning typical absence epilepsy, we have shown a lack of high or moderately elevated level of non-verbal and total IQ scores on Wechsler's tests (WISC III); we also observed a significant impairment of memory, a lengthening of school realisations, concerning motor skills and alertness and also hyperactivity and/or attention disorders, concerning 35 to 50% of cases. Most of these children present with school difficulties, of variable degree.
These results show that the term "benign" is not appropriate in many cases of so-called benign idiopathic generalized epilepsies.
Influence of EEG abnormality localisations on cognitive functions in childhood epilepsies
Isabelle Jambaqué
mental retardation. Neuropsychological evaluation may provide useful information on the nature and severity of these deficits which interfere with school achievement. Advances in medical knowledge have shown that childhood epilepsy should not be considered as a single disorder but encompasses a whole range of different conditions that exhibit specific clinical, EEG and outcome characteristics. It is known becoming apparent that these various clinical entities have different cognitive expressions that yet need to be specified according to the age of onset and the location of seizure focus. A few neuropsychological studies have demonstrated that both ictal and interictal EEG epileptiform discharges can lead to cognitive deterioration. There is now evidence that the topographic impact of epilepsy needs to be considered in epileptic children to optimize their cognitive evaluation and guide their neuropsychological rehabilitation.
Language and learning disorders in epilepsy with continuous spike-waves during slow sleep
Catherine Billard-Daudu
reducing EEG paroxysmal activity should be considered. This applies particularly to Landau-Kleffner syndrome and to the syndrome of continuous spike-waves during slow sleep. In developmental language disorders, EEG paroxysmal activity is present in almost 30% of the cases. Paroxysmal abnormalities are usually less frequent than what is observed in epilepsy with continuous spike-waves during slow sleep. Pathogenesis remains unknown and the relationship between EEG evolution and language improvement is not as clear as in Landau-Kleffner syndrome.
Cognitive impairment in childhood epilepsy: the role of antiepileptic drugs
Sylvie Nguyen The Tich, Yann Péréon
to identify, especially in severe epilepsy. Nevertheless, the possible negative effect of treatment on cognitive performance is a constant preoccupation for parents.
Despite numerous clinical investigations this remains questionable. Methodological difficulties could in part explain contradictory results obtained for a given drug.
The most recent data suggest that antiepileptic drugs used in monotherapy (with the exception of phenobarbital) do not have clear effect on cognitive and behavioral development in children. This does not exclude individual sensibility observed in clinical practice. Identifying clinical risk factors for significant cognitive adverse effects may help prescription. For the new antiepileptic drugs few additional studies are needed to define cognitive and behavioral profiles.
Psychopathology in children with epilepsy. Specific information about epilepsy
Marie-Odile Livet
be considered as a major task by the treating physician. Quality of the information provided by the physician and listening to the parents and the child are important factors that will influence the follow-up.
