Caroline Seegmuller

EEG criteria predictive of complicated evolution in idiopathic rolandic epilepsy

Background: Although so-called “benign” epilepsy with centrotemporal spikes (BECTS) always has an excellent prognosis with regard to seizure remission, behavioral problems and cognitive dysfunctions may sometimes develop in its course. To search for clinical or EEG markers allowing early detection of patients prone to such complications, the authors conducted a prospective study in a cohort of unselected patients with BECTS. Methods: In 35 children with BECTS, academic, familial, neurologic, neuropsychological, and wake and sleep EEG evaluations were repeated every 6 to 12 months from the beginning of the seizure disorder up to complete recovery. Results: In 25 of 35 patients (72%), behavioral and intellectual functioning remained unimpaired. In 10 of 35 patients (28%), educational performance and familial maladjustment occurred. These sociofamilial problems were correlated with impulsivity, learning difficulties, attention disorders, and minor (7/35 cases, 20%) or serious (3/35 cases, 8%) auditory–verbal or visual–spatial deficits. Worsening phases started 2 to 36 months after onset and persisted for 9 to 39 months. Occurrence of atypical evolutions was significantly correlated with five qualitative and one quantitative interictal EEG pattern: intermittent slow-wave focus, multiple asynchronous spike-wave foci, long spike-wave clusters, generalized 3-c/s “absence-like” spike-wave discharges, conjunction of interictal paroxysms with negative or positive myoclonia, and abundance of interictal abnormalities during wakefulness and sleep. Clinical deterioration was not linked with seizure characteristics or treatment. Conclusion: Different combinations of at least three of six distinctive interictal EEG patterns and their long-lasting (≥6-month) persistence seem to be the hallmarks of patients with BECTS at risk for neuropsychological impairments.

Landau–Kleffner syndrome: sleep EEG characteristics at onset

OBJECTIVES Landau-Kleffner syndrome (LKS) and benign epilepsy with centro-temporal spikes (BECTS) are two forms of non-lesional age-related focal epilepsies. LKS is a severe disease, affecting language abilities, attention and behavior, and evolving to acquired global aphasia. As LKS is usually readily responsive to an adequate pharmacological management, an early diagnosis of children at risk for this syndrome is essential. BECTS is characterized by the absence of neurological or neuropsychological deficits throughout the course of epilepsy. However, children initially presenting some clinical and EEG features suggesting BECTS may develop severe cognitive impairments during the course of epilepsy. These cases raise the question of whether LKS and BECTS delineate fundamentally different conditions, or represent subclasses of a broad continuum. METHODS We compared sleep EEG characteristics of 7 children with typical LKS to those of 6 children with classical BECTS. RESULTS Morphology, topography, organization, and abundance of interictal abnormalities during sleep differentiated these two syndromes from epilepsy onset, before the occurrence of aphasic deficits in LKS. The specific sleep EEG patterns possibly predictive of LKS were (1) unilateral slow wave foci, (2) bilateral independent spike-and-wave discharges, and (3) major activation of spike-and-wave discharges during sleep, exceeding 40% (40-90%) of the first sleep cycle and 30% (30-80%) of the following cycles. CONCLUSIONS These data support the hypothesis that during LKS evolution, language networks involved in the spread of abundant idiopathic interictal abnormalities (and mainly slow waves) may be progressively inhibited and become unable to carry out their normal physiological role.

Intelligence quotient improves after antiepileptic drug withdrawal following pediatric epilepsy surgery.

Antiepileptic drugs (AEDs) have cognitive side effects that, particularly in children, may affect intellectual functioning. With the TimeToStop (TTS) study, we showed that timing of AED withdrawal does not majorly influence long‐term seizure outcomes. We now aimed to evaluate the effect of AED withdrawal on postoperative intelligence quotient (IQ), and change in IQ (delta IQ) following pediatric epilepsy surgery.

Long-term outcome after cognitive and behavioral regression in nonlesional epilepsy with continuous spike-waves during slow-wave sleep.

Purpose:  To present the long‐term follow‐up of 10 adolescents and young adults with documented cognitive and behavioral regression as children due to nonlesional focal, mainly frontal, epilepsy with continuous spike‐waves during slow wave sleep (CSWS).

Epileptic encephalopathy with continuous spike‐waves during sleep: The need for transition from childhood to adulthood medical care appears to be related to etiology

Epileptic encephalopathy with continuous diffuse spike‐waves during slow‐wave sleep (ECSWS) presents clinically with infrequent nocturnal focal seizures, atypical absences related to secondary bilateral synchrony, negative myoclonia, and atonic and rare generalized tonic–clonic seizures. The unique electroencephalography (EEG) pattern found in ECSWS consists of continuous, diffuse, bilateral spike‐waves during slow‐wave sleep. Despite the eventual disappearance of clinical seizures and EEG abnormalities by adolescence, the prognosis is guarded in most cases because of neuropsychological and behavioral deficits. ECSWS has a heterogeneous etiology (genetic, structural, and unknown). Because epilepsy and electroencephalography (EEG) abnormalities in epileptic encephalopathy with continuous diffuse spike‐waves during slow‐wave sleep (ECSWS) are self‐limited and age related, the need for ongoing medical care and transition to adult care might be questioned. For adolescents in whom etiology remains unknown (possibly genetic) and who experience the disappearance of seizures and EEG abnormalities, there is rarely need for long‐term neurologic follow‐up, because often a relatively normal cognitive and social evolution follows. However, the majority of patients with structural and possibly “genetic syndromic” etiologies will have persistent cognitive deficits and will need suitable socioeducative care. Therefore, the transition process in ECSWS will depend mainly on etiology and its related features (epileptic active phase duration, and cognitive and behavioral evolution) and revolve around neuropsychological and social support rather than medical and pharmacologic follow‐up.

Épilepsies de l’enfant et fonctions cognitives

L’epilepsie touche chaque annee en France 4 000 enfants de moins de 10 ans. Or, l’enfance est une periode critique pour le developpement cognitif et psychoaffectif. La physiopathologie des troubles d’apprentissage chez l’enfant avec epilepsie est complexe et multifactorielle mais le role specifi que de certains facteurs cognitifs est maintenant bien connu. C’est pour repondre a ces besoins specifi ques d’evaluation et de prise en charge que le SESSAD l’Essor a ete cree. Il est compose d’une equipe pluridisciplinaire (directrice, pedopsychiatre, neuropediatre, psychologue, neuropsychologue, orthophonistes, psychomotricienne, educateurs specialises) permettant de prendre en compte les differents facteurs (neurologiques, cognitifs ou psychoaffectifs). Son objectif est de permettre l’integration scolaire et sociale des enfants avec epilepsie âges de 3 a 16 ans, en travaillant etroitement avec la famille et l’ecole. Les prises en charge peuvent etre individuelles ou en groupe et avoir lieu au SESSAD, a l’ecole, ou a domicile. Apres trois annees de fonctionnement, il apparait clairement que les troubles associes a l’epilepsie, en particulier cognitifs, restent encore mal connus du milieu scolaire et mal pris en charge. La creation d’un centre ressource specialise pour l’epilepsie qui pourrait informer les familles, former les professionnels, et proposer une evaluation specifi que de chaque situation permettrait de repondre en partie aux besoins et de constituer un reseau de soins adapte, au plus proche du domicile de l’enfant.La scolarite est le prealable a une insertion socioprofessionnelle reussie. Le risque de diffi cultes scolaires pour un enfant epileptique est important. Il sera fonction des caracteristiques propres a l’epilepsie, du retentissement psychologique et des capacites d’adaptation du systeme scolaire a ses troubles cognitifs specifiques. La possibilite d’une orientation vers un etablissement specialise doit etre abordee par le medecin sans attendre. L’institut de reeducation de Toul Ar C’hoat est le seul college en France specialise dans l’accueil de jeunes epileptiques en souffrance scolaire. Les jeunes ont une epilepsie de gravite variable. Meme lorsque l’epilepsie parait stabilisee ou peu active mais elle peut etre responsable d’un flechissement cognitif durable. L’objectif de la prise en charge multidisciplinaire en internat sera une remise en confiance et une reprise des acquisitions afin de permettre une reinsertion en milieu ordinaire apres en moyenne deux annees de sejour. Lorsque l’epilepsie est plus severe, la prise en charge s’attachera a permettre une rescolarisation afin de preparer un projet de formation adaptee aux contraintes de la maladie. Cette structure existe depuis 1959 et repond a un besoin encore trop souvent sous-estime.Les epilepsies de l’enfant sont decrites comme souvent responsables de troubles cognitifs et de diffi cultes d’apprentissage scolaire. Cependant, leur repercussion sur le fonctionnement cognitif et comportemental depend tres largement du type de crise (generalisee ou partielle) et de la cause de l’epilepsie. A titre d’exemple, nous traitons ici separement deux grandes categories d’epilepsie : les epilepsies idiopathiques, focales ou generalisees, survenant independamment de toute lesion cerebrale, le facteur etiologique principal etant represente par une predisposition genetique, reelle ou presumee ; l’epilepsie temporale, forme probablement la mieux etudiee parmi les epilepsies partielles non idiopathiques, resultat d’une lesion cerebrale identifi able (symptomatique) ou fortement suspectee (probablement symptomatique ou crytpogenique). Devant l’apparition de signes evocateurs d’un decalage des apprentissages, un depistage precoce des eventuels defi cits s’impose pour mettre rapidement en place les mesures adaptees aux troubles specifi ques identifi es.