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International audience; The transfer of adolescents from paediatric care to adult health facilities is often difficult for the patients and their families and can lead to a breakdown in medical follow-up and therefore serious complications. Existing recommendations for the successful transition of patients with chronic disorders do not specifically address patients with handicap. Preparations for the transfer must be made well in advance. They must aim to achieve the autonomisation of adolescents by making them responsible and providing them with the knowledge that will enable them to manage their care themselves, the know-how to react appropriately if there is any change in their condition, and to move comfortably within the adult health system. This requires the active participation of the patient, his or her family and the paediatric and adult care teams. It involves multidisciplinary management plus the production and maintenance of an educational therapy programme. Finally, the identification of doctors trained in handicap, relevant sub-specialists and even expert patients could enable improvements in the continuum of complete and appropriate care for these patients within adult medicine.

Tuberous sclerosis (TSC) epilepsy includes infantile spasms and focal seizures before the age of 2 years, whereas focal seizures are predominant over 2 years and generalized seizures may occasionally be part of Lennox-Gastaut syndrome. The better and earlier the seizure control, the better the child's subsequent cognitive and behavioral prognosis. As for epilepsy of other causes, therapeutic options depend on the type of seizure/epilepsy, age and drug resistance, but there are significant specificities for TSC. (1) As first-line treatment, vigabatrin is unanimously recommended for infantile spasms and focal seizures before 2 years and is also widely used for seizures over 2 years, as are levetiracetam and carbamazepine. (2) If seizures persist (about 40% of children and adolescents), cannabidiol and everolimus, an inhibitor of the mTOR pathway, have recently been approved as adjunctive therapy to the arsenal of antiseizure medications authorized for this age group and to the ketogenic diet. (3) Surgery is an essential treatment option in cases of drug resistance and should be discussed as soon as two treatments have failed. Presurgical investigations and operating techniques have recently progressed spectacularly, for example laser thermocoagulation with stereotactic location. A particularity of TSC is the possibility of sequential interventions on several epileptogenic tubers. (4) Finally, the innovative principle of initiating "pre-seizure" treatment with vigabatrin from the first months of life has just proven effective on the subsequent development of epilepsy in TSC. © 2022 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.

The objective of this study was to investigate the adaptive functioning of adults who had a slight to moderate intellectual deficiency, in regard of age and intellectual quotient (IQ). Cognitive and adaptive functioning were evaluated using the WAIS and VINELAND scales in 16 adults who accepted to participate in this study. We found a correlation between global IQ and each domain of the adaptive score, mostly communication skills. We also found that there was an age effect on socialization skills. Most skills were learned during infancy and adolescence, especially communication skills, which are highly stable at different ages and highly correlated with IQ. However, some abilities are still acquired in adulthood, mostly socialization skills, especially in persons with the lowest IQ. These data are of particular interest for people caring for disabled adults.

Introduction L‘hemispherotomie verticale para sagittale decrite par O. Delalande est un geste neurochirurgical de deconnection hemispherique. Elle est indiquee dans le traitement des epilepsies refractaires dont la zone epileptogene est largement etendue sur un hemisphere. Nous rapportons notre experience des dix dernieres annees. Materiel et methodes Entre 2008 et 2018, 11 patients (6 filles et 5 garcons) ont beneficie d’une hemispherotomie verticale para sagittale pour une epilepsie refractaire au traitement medicamenteux bien conduit dans le service de Neurochirurgie pediatrique de Marseille. Tous les patients ont beneficie d’un bilan pre operatoire complet (EEG, Video EEG, Pet Scan, IRM, Bilan neuropsychologique) et ont ete discute en reunion multidisciplinaire de chirurgie de l’epilepsie pediatrique. Tous les enfants ont beneficie de la technique de Delalande. Ils ont ete revus en consultation a 1 mois, 6 mois puis de facon annuelle. Tous ont beneficie d’un examen anatomopathologique sur le cortex reseque. Resultats Sur les 11 enfants, 4 presentaient une encephalite de Rasmussen (36,4 %), 4 autres souffraient d’une hemimegalencephalie (36,4 %), 2 presentaient une dysplasie corticale etendue (18,2 %) enfin un enfant presentait une epilepsie secondaire a un accident vasculaire cerebrale ischemique. Tous les patients ont eu une remission complete postoperatoire immediate des manifestations epileptiques, dont 8 (72,8 %) restent stables pendant le suivi. Pour les autres 3 patients (27,3 %), on a note une re-apparition des crises a distance durant le suivi (18–36 mois postoperatoire). Un patient a ete repris au bloc operatoire pour une deconnexion incomplete, avec une remission totale des crises apres le geste complementaire. Les complications postoperatoires sont centrees sur l’hemiparesie controlaterale, presente chez 100 % des enfants. le deficit predomine a distance sur le membre superieur. Nous n’avons pas enregistre d’hydrocephalies postoperatoires. Deux patients (18,2 %) ont presente une collection sous-durale homolaterale, traitee par une derivation sous-duro-peritoneale, avec une bonne evolution. Ces HSD nous ont conduits a modifier notre technique operatoire. Conclusion L’hemispherotomie verticale para sagittale est un traitement efficace des epilepsies refractaires hemispheriques. Elle permet un controle complet des crises chez la majorite des enfants et une qualite de vie optimale. La recidive de crises a distance doit faire envisager une deconnexion incomplete et envisager un second temps chirurgical. Les HSD peuvent etre eviter par une modification simple de la technique.

PURPOSE: Morphologic abnormalities of the hippocampal formations (MAHF) are more frequently observed in magnetic resonance imaging (MRI). We wished to specify the types of disorders associated with these malformations based on a retrospective case series by studying the language of the children presenting these abnormalities. PATIENTS AND METHODS: From the data of all the MRIs taken in the neuroradiology ward of our center over 16 months in patients under 18 years of age, we retrospectively selected the children with an MAHF, isolated or associated with other malformations. The MAHFs were defined and described according to criteria of shape or orientation defects of the hippocampal formations. We studied the files of the patients with isolated MAHF again. Those whose clinical presentation was compatible with language assessment were tested in a prospective approach. RESULTS: Out of 2208 MRIs from 1 January 2007 to 30 April 2008, 96 (4.3%) showed an MAHF, including 61 (64%) boys and 35 (36%) girls, aged from 2 months to 17 years. Eighty-two (85%) had associated abnormalities, mainly including cerebral atrophy, corpus callosum agenesis or defect, and abnormal ventricular frontal horns. Fourteen (15%) had an isolated MAHF: 2 on the left hemisphere, 2 on the right hemisphere, and 10 on both. Of these 14, 9 were compatible with language assessment. From the test results, we divided these children into 2 groups, depending on the type and severity of the impairment. Four had very serious language disorders as part of mental retardation or autistic disorders; 4 others had language disorders predominantly in expression and phonology, with weak to pathological visual memory. This study showed no potential relation between the lateralization of MAHF and language disorders, nor between the existence of epilepsy and the severity of the language disorders. Of these 14 children, 9 had behavior and autism spectrum disorders and 7 were epileptic. CONCLUSION: Even though language disorders are often part of a larger deficiency presentation, the results we obtained suggest that isolated MAHFs are not only causes of amnestic disorders, but they could also directly underlie language disorders, particularly in expression.

Introduction Une crise d’epilepsie est un signe frequent inaugural d’un accident vasculaire cerebral chez l’enfant. Vingt-cinq pour cent de ces enfants vont presenter une epilepsie. La frequence de l’epilepsie refractaire varie de 0 a 6O % dans la litterature. L’hemispherotomie fonctionnelle est actuellement le « gold standard » lors de la prise en charge neurochirurgicale. Nous rapportons pour la premiere fois notre experience de deconnexion sus-insulaire guidee par SEEG des aires « centrales » chez ces enfants. Materiels et methode Suivi prospectif. 2 enfants de 12 et 13 ans. Epilepsie refractaire evoluant depuis 6 ans pour l’un et 5 ans pour l’autre suite a un AVC ischemique sylvien etendu, de cause inconnue malgre un bilan exhaustif. Un bilan complet pre chirurgical a ete realise associant, EEG, video–EEG, champ visuel, Pet TDM, IRM morphologique et fonctionnelle ainsi qu’un bilan neuropsychologique. La frequence des crises est variable, pluri quotidiennes chez la fille et pluri-hebdomadaires pour le garcon. Les chutes sont frequentes avec presence de traumatismes violents retrouves dans l’historique. Une SEEG sans cadre controlee par la neuronavigation a ete realisee chez les 2 enfants dans l’idee de realiser une deconnexion fonctionnelle epargnant les regions non epileptogenes. Resultats Pour ces 2 enfants, la SEEG a permis de visualiser plusieurs crises habituelles. La zone ZE a pu etre delimitee par correlations electro-cliniques et confirmee par le calcul de l’index d’epileptogenicite sur les differentes electrodes impliquees. La ZE etait a chaque fois centree par le foyer ischemique et entourait le sillon central. Nous avons implante 11 electrodes chez le garcon et 12 chez la fille. Il n’y avait aucun argument pour une ZE temporale ou occipitale associee. Le bilan neuropsychologique restait dans la normale faible. Une deconnexion sus insulaire de la ZE a ete realisee. Les 2 enfants sont classes actuellement Engel 1 avec un suivi moyen de 11 mois. L’hemiplegie post-operatoire a recupere en 3 semaines. Les 2 enfants ont ameliore leur autonomie et ont repris une scolarisation normale avec AVS. La diminution des traitements oraux a ete debutee. Conclusion La deconnexion sus insulaire guidee par SEEG de la ZE permet une deconnexion respectant au mieux les regions saines et les grands faisceaux de substance blanche. Elle nous parait representer une veritable alternative fonctionnelle a l’hemispherotomie chez ces enfants. La preservation des structures occipitales et temporales doit ameliorer la qualite de vie de ces enfants et probablement leurs resultats scolaires. L’etude SEEG est un imperatif dans la prise de decision. Nous rapportons les 2 premiers cas de deconnexion sus insulaire de la ZE chez des enfants presentant une epilepsie refractaire apres AVC ischemique sylvien large. Ces resultats doivent etre confirmes a l’avenir.