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Background: Neuromodulatory applications such as vagus nerve stimulation (VNS) and responsive neurostimulation (RNS) are safe and effective strategies for medically intractable epilepsy secondary to complex partial seizures, but researchers have yet to compare their efficacies. Objective: The goal of this study is to compare VNS and RNS efficacy at reducing seizure frequency and complication rates in subjects with medically intractable epilepsy secondary to complex partial seizures. Methods: This is a retrospective chart review of 30 patients with medically intractable complex partial epilepsy, who underwent either VNS or RNS placement at a single institution between June 2012 and January 2016. There was a mean follow-up of 19 months. Seizure frequency reduction and complications were identified. Results: The median seizure frequency reduction was similar for VNS (66%) and RNS (58%). There was no major morbidity or mortality, and the frequency of minor complications was similar between VNS (15%) and RNS (18%). Conclusion: We found that VNS and RNS reduced the median seizure frequency similarly with no difference in morbidity or mortality. Further prospective studies are warranted as VNS and RNS therapy improves over time.

Resume Introduction Plusieurs travaux ont montre qu’une resection au niveau du lobe temporal (RT), pratiquee dans le cas d’une epilepsie du lobe temporal median (ELT) pharmaco-resistante, peut se traduire par un declin des performances en memoire anterograde ou memoire du passe. Cependant, la plupart de ces travaux n’ont pas utilise de criteres d’inclusion stricts pour constituer des groupes de patients homogenes. Dans la presente etude, nous avons evalue l’impact d’une RT sur les performances mnesiques en realisant un suivi neuropsychologique longitudinal de patients atteints d’ELT, patients selectionnes selon des criteres demographiques et cliniques restreints. Methode Trente patients atteints d’ELT unilaterale (17 droites et 13 gauches) ont ete evalues a 4 tests de memoire anterograde et a six tests de memoire du passe, trois mois avant la RT, ainsi que cinq mois et 12 mois apres l’operation. Resultats Suite a la RT, les performances en memoire anterograde sont ameliorees un an apres l’operation. Seuls les patients operes dans l’hemisphere droit ameliorent leur memoire autobiographique. Conclusions Une amelioration postoperatoire des capacites mnesiques a ete observee des lors que les patients presentaient des caracteristiques cliniques specifiques.

La survenue de crises chez des patients epileptiques n’est pas toujours un phenomene completement aleatoire. Parmi les facteurs favorisants, le stress et, d’une facon plus generale, le contexte emotionnel sont les circonstances le plus souvent rapportees par les patients. Nous avons plus particulierement etudie cette « vulnerabilite emotionnelle » a travers une approche clinique et cognitive. Nous avons pu montrer que bien que basee sur une appreciation subjective, cette vulnerabilite etait associee a des marqueurs de type « biais attentionnels » pour les informations emotionnelles. De plus, les patients vulnerables sont plus souvent des femmes et ont debute leur epilepsie apres un evenement emotionnel marquant.

Plusieurs des caracteristiques morphologiques et electrocliniques de l’epilepsie mesiotemporale (EMT) peuvent etre reproduites chez la souris adulte par l’injection unilaterale d’une faible dose de kainate (KA) dans l’hippocampe dorsal. Apres un etat de mal initial et une periode latente de deux semaines, les souris developpent des decharges recurrentes et des remaniements morphologiques dans l’hippocampe semblables a la sclerose de l’hippocampe. L’EMT etant souvent associee a une resistance a plusieurs medicaments anti-epileptiques, nous avons evalue dans ce travail les effets de quatre molecules (diazepam, valproate, carbamazepine et lamotrigine) sur les decharges hippocampiques recurrentes. Seul le diazepam (0,5-3 mg/kg, i.p.) supprime de facon dose-dependante les decharges hippocampiques. Le valproate (100-400 mg/kg), la carbamazepine (25-100 mg/kg) et la lamotrigine (30-90 mg/kg) ne suppriment les crises qu’a la plus forte dose. A ces doses, le trace EEG est fortement perturbe, et les animaux presentent des signes d’incapacitation motrice. Ces resultats montrent que les decharges hippocampiques chez la souris sont resistantes aux anti-epileptiques classiques et suggerent que ce modele d’EMT constitue un outil d’evaluation pharmacologique de cette forme d’epilepsie.

UMMARY The authors report an 11-year-old girl with epilepsy, poor school-performance and minor behavioural disorders. The epilepsy is characterized by complex partial seizures, sometimes progressing secondarily into generalized tonic-clonic seizures. and is poorly controlled by medical treatment. Chromosomal analysis revealed a ring chromosome 20 mosaicism. The affected patient shows signs of ring chromosome 20 syndrome, characterized in the present case by poor school-performance, behavioural disorders and epilepsy. RESUME Mosaiqire dhn chromosome 20 circiilaire ctrez une fillerie presentant des crises partielles coniplexes Les auteurs rapportent l e cas d'une fillette de 11 ans presentant une epilepsie, des resultats scolaires mediocres et des troubles comportementaus mineurs. Ľepilepsie etait caracterisee par des crises partielles complexes quelquefois secondairement generalisees en crises tonico-cloniques globales et etait mal controee par le traitement. Ľ analyse chromosomique revela une rnosaique ď un chroniosonie 20 en anneau. La fillette rnontrait les signes ď un syndrome r(2O) caracterise dans le cas present par des resultats scolaires mediocres, des troubles comportementaux et une epilepsie. ZUSAMMENFASSUNG Mosaik des Ringchromosonrs 20 bei ernem Madchen mil komplexen Partiulunfallen Die Autoren berichten uber ein 11-jahriges Madchen mit Epilepsie, schlechten Schulleistungen und leichten Verhaltensstorungen. Die Epilepsie ist charakterisiert durch komplexe Partialanfalle, die manchmal in generalisierte tonisch-klonische Anfalle ubergehen und die medikarnentos schlecht zu kontrollieren sind. Die Chromosomenanlyse ergab ein Mosaik des Ringchromosoms 20. Die Patientin hatte Zeichen des r(20) Syndroms, die sich in ihrem Fall in schlechten Schulleistungen, Verhaltensstorungen und Epilepsie auserten. RESUMEN Mosaicismo con cromosoim 20 en unillo en una niaa con crisis parciales cornplejas Los autores aportan el caso de una nina de 11 anos con epilepsia, escolaridad pobre y alteraciones menores del comportarniento. La epilepsia se caracteriza por ataques parciales complejos, en ocasiones con generalization secundaria en forma de convulsiones tonico-clonicas, y que se controlan pobremente con la medicacion. El cariotipo revelo un mosaicismo con un cromosoma 20 en anillo. El paciente muestra signos de un sindrome de r(20), caracterizado en este caso por dificuftad. escolar, alteraciones en el comportamiento y epilepsia.

More than 20 years ago, complex partial epilepsy of occipital-temporal origin was suggested as having been the “nervous disease” of Gustave Flaubert, one of the most famous French novelists. The aim of the present work, therefore, was to reevaluate the diagnosis of Flaubert’s “nervous disease” in the light of reemerged biographic information and letters, as well as the numerous scientific advances in epilepsy and its psychopathology in recent years. If the semiology of the reported attacks is considered, epilepsy ranks among the most probable diagnoses. In our opinion, psychopathological considerations suggest primary involvement of mesial temporal lobe structures with typical findings of ictal and interictal mood behavior.

Resume Introduction Les propagations des crises d’epilepsie partielle se font selon un schema spatio-temporel propre a chaque localisation cerebrale. Caracteriser ce schema peut aider a mieux definir cette localisation. Dans les epilepsies de la face mediale du lobe temporal, l’etude des propagations s’est focalisee sur le passage des crises vers le lobe temporal controlateral. Etat des connaissances . L’analyse de ces propagations s’est faite au mieux grâce aux enregistrements EEG intracrâniens. Les sequences de propagation des crises d’origine medio-temporale aux structures ipsi-, puis controlaterales, diversement appreciees selon les auteurs, ont conduit a incriminer plusieurs voies de passage interhemispherique possibles : les trois commissures liant les lobes temporaux (corps calleux, commissure hippocampique dorsale et commissure anterieure) et un circuit indirect passant par les lobes frontaux. D’autre part, les vitesses de propagation des crises d’origine temporo-limbique sont lentes, le delai inter-temporal moyen est d’environ 30 secondes, avec cependant une forte variabilite. Cette lenteur pourrait etre liee en partie a la rarete des connexions commissurales des structures temporo-limbiques entre elles chez l’homme. A l’inverse, les regions medio-temporales paralimbiques (rhinales et polaires) sont fortement connectees par voie commissurale et des temps de transfert inter-temporal de l’ordre de 100 ms ont ete mesures pour une decharge epileptique induite a partir du cortex perirhinal anterieur. Il existe donc des possibilites de passage extremement rapide inter-medio-temporal a partir des regions paralimbiques anterieures, passant vraisemblablement par la commissure anterieure. Perspectives . Les modalites de propagation des crises d’origine medio-temporale restent debattues. Pourtant elles conditionnent par leur extension et vitesse la semiologie electroclinique des crises et notre capacite a discerner l’origine des decharges epileptiques. Leur etude merite d’etre poursuivie. Conclusions . La conception duelle des propagations inter-medio-temporales, selon leur vitesse et lieu d’origine, s’appuie sur un faisceau d’arguments neurophysiologiques et anatomiques concordants developpes dans cette revue.

Resume Introduction La memoire du passe, contrairement a la memoire anterograde, rarement ete examinee dans l’epilepsie du lobe temporal et jamais avant et apres traitement chirurgical. Par ailleurs, les etudes de cas rapportant un deficit de la memoire des faits publics avec une preservation de la memoire autobiographique sont tres rares. Methodes Nous presentons le cas d’un patient atteint d’une epilepsie temporale pharmacoresistante en rapport avec une sclerose hippocampique gauche. Des examens neuropsychologiques complets ont ete realises avant et apres lobectomie temporale, avec un interet particulier pour la memoire du passe, evaluee exhaustivement au moyen de tests standardises, mais egalement d’epreuves adaptees au patient. Resultats Le patient presentait une memoire preservee pour les faits et episodes autobiographiques, mais des scores deficients aux tests de faits publics, dans un contexte de capacites cognitives generales normales. De plus, un oubli accelere pour du materiel verbal et non verbal etait mis en evidence. Conclusion Les resultats suggerent que le trouble de consolidation a long terme pourrait avoir contribue au deficit de la memoire des faits publics. Nous discutons les caracteristiques specifiques du traitement des faits publics en essayant d’expliquer sa plus grande vulnerabilite par rapport a la memoire autobiographique, dans le cas de l’oubli accelere.

Resume L’exerese complete d’une lesion responsable d’une epilepsie permet la disparition ou la reduction importante des crises dans 85 % des cas. Cependant, le pronostic semble moins favorable dans les epilepsies temporales lesionnelles car les structures temporo-mesiales peuvent participer a la genese des crises sans etre le siege de ces lesions. L’analyse electro-clinique et radiologique des patients peut apporter des elements d’orientation en faveur d’une telle implication mais ne semble pas toujours suffisante. Afin d’evaluer les criteres nous ayant conduits au choix de la strategie chirurgicale, nous rapportons une serie retrospective consecutive de 47 patients traites pour une epilepsie temporale pharmaco-resistante symptomatique d’une lesion temporale extra-hippocampique traites soit par lesionectomie seule (groupe 1, n = 17), soit par lesionectomie associee a une resection des structures temporo-mesiales (groupe 2, n = 30). Les cas presentant une sclerose hippocampique associee a la lesion (double pathologie) ont ete exclus de cette etude. Les resultats de cette etude montrent qu’avec un recul moyen de 72 mois, 84 % des patients du groupe 2 ont ete liberes de leur crise (grade Ia de Engel) contre seulement 47 % des patients du groupe 1. L’analyse statistique a identifie le type de chirurgie comme le facteur pronostique preponderant. En conclusion, les criteres electro-cliniques retenus pour evoquer une participation temporo-mesiale dans l’elaboration des crises sont insuffisants pour preciser la strategie chirurgicale. Compte tenu de sa valeur pronostique, l’evaluation de cette participation, parfois complexe, apparait importante a realiser. L’existence d’une double pathologie « fonctionnelle » acquise est evoquee pour expliquer ce resultat.

Background:Clobazam is a benzodiazepine with known antiepileptic action; however, it is not considered first line therapy in the treatment of epilepsy. The objective of this study was to evaluate the efficacy of clobazam as add-on therapy in adults with temporal lobe epilepsy associated with MRI evidence of hippocampal sclerosis (HS).Method:This is a retrospective study, conducted at our epilepsy clinic which evaluated clobazam as add-on therapy in patients with temporal lobe epilepsy and MRI signs of HS. Clobazam was prescribed based on the minimum effective dose up to the maximum tolerated dose.Results:Seventy-eight patients met the inclusion criteria (51 women), ages ranging from 16 to 76 years old (mean=42.2). Dosage of clobazam ranged from 5 to 60 mg/day (mean=22.6 mg/day). Clobazam was used from one month to eight years (mean=29 months). Sixteen (20.5%) patients were seizure-free, 20 (25.5%) had more than 75% improvement in seizure control, eight (10%) had more than 50% and 20 (26%) were non responders to clobazam. In 14 (18%) we could not determine seizure frequency during follow-up. The improvement in seizure control lasted for more than one year in 30 (68%) patients.Conclusion:Our data suggest that clobazam should be considered as add-on therapy in the treatment of patients with temporal lobe epilepsy associated with MRI signs of HS.

Introduction/Objectif: On peut observer des anomalies diffuses du lobe temporal a l'IRM de patients atteints d'epilepsie du lobe temporal mesial (ELTM). Le but de cette etude etait d'effectuer des analyses qualitatives et quantitatives des structures du lobe temporal chez des patients atteints d'ELTM familiale (ELTMF) et non familiale (ELMT). Methodes: Deux groupes de patients ont ete identifies: 67 patients atteints d'ELMTF, dont 14 avaient des crises refractaires au traitement, et 30 patients atteints d'ELMT non familiale refractaire, Nous avons effectue des analyses qualitatives de l'IRM (avec reconstruction multidimensionnelle) et du volume de l'hippocampe et des lobes temporaux anterieurs chez tous les patients et chez un groupe de temoins normaux comprenant 23 individus. Nous avons utilise le test du c2 et l'ANOVA comme analyses statistiques. Resultats: Nous avons identifie des anomalies du lobe temporal anterieur par analyse visuelle chez seulement 4 % des patients atteints d'ELTMF et une atrophie du lobe temporal anterieur par analyse volumetrique chez 19 % de ces patients. Dans le groupe de patients atteints d'ELTM non familiale, nous avons observe des anomalies du lobe temporal anterieur par analyse visuelle chez 17 % des patients et une atrophie du lobe temporal anterieur chez 13 %. Une atrophie de l'hippocampe etait presente chez 90 % des patients atteints d'ELTMF et chez 83 % des patients atteints de la forme non familiale. Aucun signe de dysplasie corticale n'a ete observe, Conclusion: Une atrophie du lobe temporal anterieur et d'autres anomalies ne touchant pas la partie mesiale des lobes temporaux etaient rares tant chez les cas familiaux que chez les cas non familiaux. Malgre son etiologie genetique, l'atrophie de l'hippocampe n'est pas associee a des anomalies de structures en dehors des regions temporales mesiales dans l'ELTMF.

Background:The lateralizing value of the motor automatisms is generally doubted in most patients with temporal lobe epilepsy. However, subgroup analysis of the seizures of temporal lobe origin suggests a role for motor automatisms in discriminating seizures of neocortical versus mesial temporal lobe origin.Methods:Video-EEG of a patient with well-defined neocortical temporal lobe epilepsy was reviewed to assess the localizing value of motor automatisms.Results:We report a patient with left upper extremity motor automatisms and clonic movements of the proximal left lower extremity with altered awareness as the sole manifestations of right temporal neocortical seizures.Conclusion:Early onset unilateral motor automatisms without dystonic posturing can localize the seizure origin to the contralateral temporal lobe neocortex.

Nuclear magnetic resonance spectroscopy (1H-MRS) is a non-invasive method in detecting abnormal spectra of various brain metabolites containing N-acetylaspartate (NAA), Choline (Cho), Creatine (Cr), gamma-Aminobutyric acid (GABA) and Glutamate. Technical processing of the MR-systems, improved automated shimming methods and further development of special shim coils increase the magnetic field homogeneity and lead to a better spectral quality and spectral resolution. The handling of the systems becomes more user-friendly and is more likely to be used in routine diagnostics. The 1H-MRS has become a diagnostic tool for assessing a number of diseases of the central nervous system mainly including epilepsies and brain tumours. The role of 1H-MRS in the assessment of epilepsies will probably increase in future. In the following article, the principles of 1H-MRS and an overview of it in the evaluation and treatment of epilepsies with special regard to temporal lobe epilepsies (TLE) has been illustrated.

Background: Temporal lobectomy in adults is an accepted form of treatment for patients with intractable complex partial seizures. There have been few long-term studies of children undergoing temporal lobectomy for epilepsy. Methods: We reviewed the pediatric cases of temporal lobectomy for intractable epilepsy performed by the Comprehensive Epilepsy Program at the University of Alberta Hospitals between 1988 and 2000. All patients had preoperative and postoperative clinical evaluations, seizure charts, drug levels, EEG, CT/MRI, long-term video EEG monitoring and neuropsychological testing. The patients were reassessed at 6 weeks, 6 months and 1 year postoperatively, then yearly. The duration of follow up was 1–10 years (mean 5 years). Results: Forty-two patients were studied (25 males and 17 females). Age at surgery ranged from 18 months to 16 years. The interictal EEG was abnormal in 38 of the 42 patients. Twenty-two patients had focal epileptic discharge and 1 had generalized epileptic discharge. Focal slowing was seen in 9 patients and diffuse slowing in 5 patients. CT scan was abnormal in 17 of 39 patients and normal in 22 of 39. MRI was abnormal in 34 of 42 patients and normal in 8 of 42. Pathology included brain tumors in 14 patients, mesial temporal sclerosis in 8, focal cortical dysplasia in 4, tuberous sclerosis in 4, dual pathology in 4, porencephalic cyst in 1 and normal pathology or gliosis in 6. Thirty-three of 42 patients (78%) were seizure-free following surgery and an additional 5 (12%) had a decrease in seizure frequency. Three patients had complications, but there were no deaths. Conclusion: Temporal lobectomy is a safe and effective treatment for children with intractable complex partial seizures. Seventy-eight percent of patients are seizure-free following the surgery and there are few complications. MRI is superior to CT scan for detection of temporal lobe pathology yet failed to detect abnormalities in some patients. The most common pathologies found were brain tumors, mesial temporal sclerosis and developmental lesions. In addition to seizure control, many patients experienced improvement in cognitive and psychosocial function following surgery.

Advances in our understanding of the molecular biology of human epileptogenesis and secondary hyperexcitability have resulted in part from the development and characterization of animal models of mesial temporal lobe epilepsy, trauma-induced epilepsy, and age-specific models of febrile seizures and neonatal hypoxia-induced seizures. Each of these models displays similarities to and differences from their human counterparts and will undoubtedly prove useful for evaluating novel therapies aimed at preventing the development, or halting the progression, of epilepsy or insult-induced secondary hyperexcitability. This article briefly reviews those models that most closely parallel human mesial temporal lobe epilepsy, trauma-induced epilepsy, febrile seizures, and hypoxia-induced seizures. Particular attention is paid to the underlying pathology, the presence or absence of a latent period, and development of spontaneous seizures.

Summary: Purpose: To compare the localizing yield of sphenoidal electrodes placed under fluoroscopic guidance (SEs) and anterior temporal electrodes (ATEs) in ictal recordings from a group of patients with seizure disorders of anterior temporal origin. Methods: We compared ictal recordings of 156 seizures obtained with SEs and ATEs from 40 consecutive patients with seizures of anterior temporal origin. Four electroencephalographers reviewed ictal recordings independently and blind to the patients' identity, presurgical data, and inclusion of ATEs or SEs. Outcome variables included (a) number of correctly localized seizures with SE and ATE recordings by at least three raters; (b) number of ictal foci in which all seizures were localized only with SEs; and (c) number of seizures in which SEs identified the ictal onset ≥5 s earlier than ATEs. Results: Interrater agreement among the four raters was significantly greater with SE than with ATE recordings (p < 0.0001). The number of seizures correctly localized was significantly greater with SEs (n = 144) than with ATEs (n = 99; p < 0.0001). All the seizures [n = 36 (23%)] originating from 14 ictal foci (29%) in 11 patients (27.5%) were localized only with SEs. Finally, the ictal onset was detected at SEs ≥5 s earlier than at ATEs in 67 (43%) seizures originating from 33 (69%) foci in 30 (75%) patients. Conclusions: SEs improve interrater agreement in the localization of seizures of anterior temporal origin, and in about one fourth of patients, SEs add ictal data not identified by ATEs.

To study the influence of interictal spikes on 18F-fluorodeoxyglucose positron emission tomography (FDG-PET), EEG monitoring was performed during PET scanning in 21 patients with temporal lobe epilepsy. Asymmetry indices were calculated in the polar, mesial, anterior-lateral, mid-lateral and posterior-lateral temporal region of interests of FDG-PET (PET-AI). 70.7% of spikes were recorded with their maximum at the anterior temporal region (F7, F8, FT9, FT10), 29.3% at mid-temporal (T7, T8), and none at posterior temporal region (P7, P8). Regardless of the side of epileptic focus, right-left difference of the total spikes had a significant negative correlation with right/left PET-AIs of the anterior-lateral temporal region (Spearman’s ρ = –0.565, p = 0.009), polar (ρ = –0.500, p = 0.021) and whole temporal region (ρ = –0.480, p = 0.028). FDG-PET hypometabolism may reflect not only a permanent functional deficit but also a transient regional cerebral dysfunction related to the occurrence of interictal spikes.

Objectives: Firstly, to study the pathology at surgery in children undergoing temporal lobectomy for intractable partial epilepsy. Secondly, to compare neuroimaging techniques (CT, MRI) in the preoperative detection of pathology. Lastly, to examine the surgical outcome in children. Methods: Forty-two pediatric patients undergoing temporal lobectomy for intractable epilepsy at the Comprehensive Epilepsy Program at the University of Alberta Hospital between the years 1988–1998 were studied. Patients had extensive preoperative investigations including CT and MRI. The pathology at surgery was reviewed and compared to preoperative neuroimaging. Charts were reviewed to determine surgical outcome. Results: Brain tumors were the most common pathology, found in 13/42 patients. Mesial temporal sclerosis (MTS) was found in 8 patients and dual pathology in an additional 5. Focal cortical dysplasia (FCD) was seen in 4 patients, 1 patient had a porencephalic cyst and 4 patients had tubers of tuberous sclerosis. Seven patients had no specific pathology detected. MRI was clearly more sensitive than CT in the detection of pathology. MRI was abnormal in 27/42 cases (64%), while CT scan was found to be abnormal in only 12/39 (31%). Surgical outcome was excellent, with 34/42 patients (80%) having an Engel class I outcome. One patient had significant improvement with an Engel class II outcome, 3 (7%) had little improvement (Engel class III) and 4 (10%) were unchanged (Engel class IV). Three patients (7%) had surgical complications. Conclusions: A wide variety of developmental pathology is seen following temporal lobectomy for intractable epilepsy of childhood. Brain tumors, FCD and MTS are common. MRI is superior to CT in the detection of pathology, which may be subtle in children. Surgical outcome is excellent, with most children being seizure free and few complications being seen.

Resume L'analyse quantifiee de l'EEG a ete utilisee chez neuf patients epileptiques temporaux droitiers inclus dans une procedure prechirurgicale pour tenter de predire la localisation des centres du langage. Ce travail avait pour but de valider un protocole d'etude des variations de la puissance spectrale de l'alpha dans un paradigme d'activation precedemment developpe chez des sujets sains droitiers : la diminution significative de la puissance spectrale du rythme alpha sur l'hemisphere gauche au cours d'une tâche d'ecriture de la main droite par rapport aux valeurs de repos, et/ou la diminution de la puissance spectrale du rythme alpha sur l'hemisphere droit au cours d'une tâche de reconnaissance de formes de la main gauche par rapport aux valeurs de repos, etaient considerees comme un marqueur electrophysiologique de la specialisation hemispherique gauche pour le langage. Dans ce travail, la localisation du langage obtenue par l'etude des variations spectrales chez les patients etait comparee aux resultats du test de Wada considere comme la technique de reference. Parmi les neuf sujets, huit avaient l'hemisphere gauche dominant pour le langage (pour un hemisphere droit) au test de Wada. Six patients avaient une diminution significative de la puissance de l'alpha en accord avec les resultats du test de Wada ; pour les trois patients restant (incluant le patient dont l'hemisphere droit etait dominant pour le langage), les variations spectrales etaient non significatives. La diminution de la puissance spectrale du rythme alpha sur l'hemisphere actif au cours d'un paradigme d'activation cerebrale semble statistiquement liee a la specialisation hemispherique gauche des centres du langage. Cependant, pour un patient epileptique donne, l'etude des seules variations spectrales ne permet pas de definir de maniere cliniquement pertinente la lateralisation des centres du langage.

Identification of the responsible mutant genes and of the functional consequences of the mutations in experimental preparations have begun to shed light on mechanisms underlying a rare form of partial epilepsy in humans, autosomal dominant nocturnal frontal lobe epilepsy. Likewise, study of the mechanisms of nongenetic models of a common form of human epilepsy, complex partial epilepsy of temporal lobe origin, has established the hippocampal dentate granule cells as a functional barrier to invasion of epileptiform activity into hippocampus in normal brain; this barrier is defective in an epileptic brain. Potential mechanisms by which the 'barrier function' might become flawed, such as mossy fiber sprouting, are discussed.

We compared the cognitive functions and interictal cerebral glucose metabolism of 11 patients with mesial temporal lobe epilepsy (MTLE) with frequent seizures to those of 10 patients with MTLE with rare seizures; the groups were matched for age, sex, education, IQ, and focus side. The frequent-seizure group had more set-shifting impairment that correlated with glucose hypometabolism in the prefrontal cortices. Our results suggest that frequent seizures in MTLE are associated with hypofunction of the prefrontal cortex.

Anterior temporal resection may effectively relieve therapy-resistant seizures in children and give social, psychological and economic benefits. Epidemiological and prognostic data demonstrate that this is an under-utilised procedure: although an estimated 677 Ontario children per year would be candidates for anterior temporal resection, only 18 were performed in a 12 month period.

Contributions made by John Hughlings Jackson (1835-1911) to today's epileptology are unparalleled, and what he did has still not been reevaluated. We divide his contributions into three themes: (a) He began his research with the seizures later named jacksonian seizures. Through his research, Jackson established a revolutionary definition quite similar to our contemporary definition of epilepsy. The first step was made to the neurosurgical treatment for epilepsy through his research. (b) He elucidated the mechanism of postepileptic psychosis by his theory of evolution-dissolution and positive-negative symptoms, later called jacksonism. (c) His encounter with his patient, Dr. Z, helped him to establish his idea of dreamy state, eventually leading to the concept of temporal lobe epilepsy.

The majority of medically refractory partial epilepsies arise in the frontal and temporal lobes and represent a major challenge to the neurologist. The phenomenology of seizures from these regions is diverse and fascinating, at times lying on the border of many medical specialties. An appreciation of the wealth of potential phenomena can lead to more accurate diagnosis and treatment.

Resume Chez des patients souffrant d'epilepsie partielle grave, pharmacoresistante, explores en videostereo-EEG, la definition de la «zone epileptogene repose principalement sur l'enregistrement de crises spontanees, mais aussi de crises induites par la stimulation electrique (SE) intracerebrale. Seules les SE par «trains (SET, 50 Hz) sont couramment employees dans ce but; les SE par «chocs (SEC, 1 Hz) sont essentiellement utilisees pour le reperage des regions fonctionnelles, surtout motrices. Au cours d'une etude prospective menee chez dix patients ayant une epilepsie temporale, nous avons pu montrer que la SEC s'averait susceptible d'induire frequemment des crises, surtout dans la portion anterieure de la corne d'Ammon. Meme si son efficacite parait moindre que celle de la SET, elle permet en revanche, dans la majorite des cas, de reproduire les manifestations subjectives critiques de facon isolee, d'apprecier l'organisation des decharges au cours meme de la stimulation, et ne donne qu'exceptionnellement des reponses «inattendues («faux positifs?).

Summary: In 8 patients in whom it was uncertain whether they had occipital or temporal lobe (TL) epilepsy, clinical, scalp EEG, and radiologic features were correlated with the sites of seizure onset as determined by depth EEG. The 8 patients were selected from >40 with occipital epilepsy because they had (a) an aura considered to be of occipital lobe (OL) origin, (b) an occipital interictal epileptic focus, (c) an OL lesion, or (d) a combination of all of these. Scalp EEG and clinical patterns suggested temporal involvement in all, however. Extracranial EEG recordings were often misleading, showing multilobar interictal epileptic abnormalities, and seizure onset was of poor localizing value and did not clarify the problem sufficiently. Intracranial EEG recordings showed that seizure onset could be ordered along an Occipitotemporal gradient. Consistent OL seizure onset was observed in patients who had only elementary visual auras. Those who had inconsistent aura or no aura, suggesting OL origin, had onset of most attacks in the TL. All patients had a seizure spread pattern suggesting early TL involvement. To prevent visual field defect, surgical approaches included temporal resection when temporal seizure origin or spread was demonstrated; although occasionally this produced excellent results, it was of limited benefit in most patients, even when some seizures were proven to originate in TL structures. In patients with malignant epilepsy and in those with an occipital lesion, occipital resection should be considered. ReSUMe Chez 8 patients, pour lesquels le diagnostic du siege de l'epilepsie n'avait pas tranche entre une origine occipitale ou temporale, les donnees cliniques, EEG de surface et radiologiques, ont ete correlees avec le site de debut des crises determine par electrodes profondes. Ces patients ont ete selectionnes parmi une serie de 40 patients presentant une epilepsie occipitale, en raison: (1) d'une aura consideree comme d'origine occipitale, (2) d'un foyer epileptique intercritique occipital, (3) d'une lesion du lobe occipital, (4) d'une combinaison de ces caracteristiques. Cependant, les phenomenes cliniques et les donnees de l'EEG de surface suggeraient une implication du lobe temporal chez tous ces patients. Les enregistrements EEG extra-crâniens etaient souvent trompeurs, ils mettaient en evidence des anomalies epileptiques intercritiques multi-lobaires, et le debut des crises etait peu Iocalise, ne clarifiant pas le probleme de facon suffisante. Les enregistrements EEG intracrsniens ont montre que le debut des crises pouvait etre aligne sur un gradiant occipito-temporal. Un debut de crises occipitales fiables a ete constate chez les patients qui n'avaient que des auras visuelles elementaires. Ceux qui avaient une aura variable ou qui n'avaient pas d'aura en faveur d'une origine occipitale presentaient des crises commencant le plus souvent au niveau du lobe temporal. Tous les patients presentaient un type de diffusion des crises qui etait en faveur d'un interessement prCcoce du lobe temporal. Afin d'eviter un deficit du champ visuel, l'approche chirurgicale a inch une resection temporale lorsque l'origine temporale des crises ou l'extension temporale a pu etre demontree. Bien que les resultats aient parfois ete excellents, cette attitude n'a apporte qu'un benefice limite pour la majorite des patients, meme si l'origine de certaines crises pouvait etre rapportee aux structures du lobe temporal. Chez les patitents presentant une eepilepsie maligne, specialement chez ceux avec une lesion occipitale, il faudrait envisager une resection occipitale. RESUMEN Segun las regiones de comienzo de los ataques detectado con electrodos profundos, las caracteristicas radiologicas y del EEG de cuero cabelludo fueron correlacionadas en ocho pacientes en los que existian dudas con respecto al diagnostico de epilepsia occipital o del lobulo temporal. Los casos se solicitaron de una serie de mas de 40 enfermos con epilepsia occipital porque tenian i) un aura considerada de origen occipital, ii) un foco epileptico occipital interictal, iii) una lesion en el lobulo occipital o iv) una combinacion de todos los datos. Sin embargo, los patrones clinicos y del EEG de cuero cabelludo sugerian una afectacion temporal en todos los casos. Los registros extracraneales del EEG fueron frecuentemente confusos mostrando anomalias epilepticas interictales multilobares y el comienzo de las descargas fue de escaso valor de localizacion y no aclararon suficientemente el problema. Registros intracraneales del EEG mostraron que el comienzo de los ataques podian ser ordenados a lo largo del gradiente occipito-temporal. Se observo un comienzo consistente en el 1ohulo occipital en pacientes que solo tenian auras occipitales elementales. En los que no tenian aura que sugiriese un origen en el 1ohulo occipital la mayor parte de los ataques tenian un cornienzo en el lobulo temporal. Todos los enfermos tenian un patron de propagacion de los ataques que sugeria una afectacion precoz del 1obulo temporal. Para evitar el defecto del campo visual las tecnicas quirurgicas incluyeron una reseccion del lobulo temporal donde el origen de los ataques era temporal, o se demostro una propagacion a pesar de que esta tecnica produjo, ocasionalmente, excelentes resultados produjo un beneficio limitado en la mayoria de los casos incluso cuando alguno de los ataques se originaban en estructuras del lobulo temporal. En los enfermos con epilepsia maligna, especialmente los que tenian una lesion occipital, debe de considerarse una reseccion del citado lobulo. ZUSAMMENFASSUNG Bei 8 Patienten mit Unsicherheit, ob sie eine occipitale oder eine Temporallappenepilepsie hatten, wurden die klinischen, die Scalp-EEG und die radiologischen Befunde rnit den Orten der Anfallsentstehung nach Tiefen-EEG-Ableitung korreliert. Sie wurden aus uber 40 Patienten rnit occipitaler Epilepsie ausgewahlt, da sie (1) eine Aura mit Entstehung im Occipitallappen, (2) einen occipitalen, interiktalen epileptischen Fokus, (3) eine Occipitallappenlasion oder (4) eine Kombination dieser Symptome hatten. Scalp-EEG und klinisches Anfallsmuster legten eine Temporallappenbeteiligung bei allen Patienten nahe. Die extrakraniellen EEG-Ableitungen fuhrten oft zu einer Fehldiagnose, da sie multilobulare interiktale epileptische Abnorrnitaten zeigten und einen Anfallsbeginn rnit schlechtem lokalisatorischem Wert, so daβ diese Frage nicht ausreichend geklart wurde. Die intrakraniellen EEG-Ableitungen zeigten, daβ der Anfallsbeginn entlang einem occipitotemporalen Gradienten angeordnet werden konnte. Ein konsistenter Beginn des Anfalls im Occipitallappen wurde bei Patienten geschen, die nur elementare visuelle Auren hatten. Patienten mit inkonsistenten oder fehlenden Auren und vermutetem Occipitallappenbeginn zeigten den Beginn der meisten Anfalle im Temporallappen. Alle Patienten zeigten ein Anfallsausbreitungsmuster, das eine fruhe Temporallappenbeteiligung nahelegte. Um visuelle Ausfalle zu verhindern, schloβ die chirurgische Entfernung eine Temporallappenresektion rnit ein, wenn ein temporaler Anfallsbeginn oder eine Temporallappenausbreitung gezeigt wurde; obwohl dies gelegentlich exzellente Resultate lieferte, war dieses Vorgehen bei der Mehrzahl von beschranktem Wert, auch wenn einzelne Anfalle nachweislich in Temporallappenstrukturen entstanden. Bei Patienten rnit einer malignen Epilepsie, Speziell rnit einer Occipitallappenlasion, sollte eine occipitale Resektion erwogen werden.

L'electroencephalographie (EEG) est un outil important pour le diagnostic, la lateralisation et la localisation de l'epilepsie temporale (ET). Dans cet article, nous revoyons les caracteristiques electroencephalographiques de l'epilepsie temporale. Plusieurs electrodes « non-standard » peuvent etre necessaires pour une evaluation plus poussee de la localisation EEG. L'enregistrement EEG ictal est une composante importante des protocoles preoperatoires pour des motifs chirurgicaux. Differents rythmes ictaux ont ete decrits, dont l'attenuation du rythme de fond, le phenomene depart-arret-depart, une activite lateralisee irreguliere a 2-5 Hz et des ondes sinusoidales a 5-10 Hz ou des decharges epileptiformes repetitives. L'EEG postictal peut egalement fournir de l'information precieuse pour la lateralisation. L'activite delta postictale peut etre lateralisee chez 60% des patients atteints d'ET et concorde avec le cote du debut de la crise chez la plupart des patients. Quand les patients sont evalues en vue d'une resection chirurgicale, des enregistrements EEG effractifs peuvent s'averer necessaires. Le succes de la chirurgie repose sur la localisation exacte du debut des crises chez ces patients.

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Definition de criteres pour le choix des candidats a la chirurgie de l'epilepsie temporale. L'echec du traitement medical est l'une des indications absolues d'envisager la chirurgie. C'est un concept relatif qui doit etre individualise. Il est facile de determiner quand les crises d'un patient sont parfaitement controlees. Cependant, la persistance des crises n'est pas necessairement une mesure de l'absence de reponse au traitement ou de l'invalidite. La decision de recourir a la chirurgie devra etre basee sur les facteurs suivants: la certitude du diagnostic, des crises qui sont frequentes et invalidantes et des crises chez des patients qui sont refractaires a une medication antiepileptique optimale quand au choix et a la posologie.

To identify children with complex partial seizures with increased risk for suboptimal seizure control with 1 medication, a computerized database containing all patients seen in the context of a single pediatric neurology practice was reviewed for patients with complex partial seizures. Participants included in analysis were then divided into groups; a group in whom seizure control was attained with a single medication (group 1) and a group for whom 2 or more medications were required for seizure control (group 2). Status epilepticus, developmental disabilities, and the presence of coexisting other seizures/types were also significantly different, with a higher predominance in group 2 children. Patients with status epilepticus, coexistent seizure types, and developmental disabilities should be identified and more carefully followed, with a lower threshold for starting these children on a combination of antiepileptic drugs adhered to.

Summary Familial mesial temporal lobe epilepsy (FMTLE) was first described as a benign syndrome with prominent psychic and autonomic seizures and no association with hippocampal sclerosis (HS) or febrile seizures (FS). Better definition of the syndrome allowed identification of more heterogeneous phenotypes with mild to severe epileptic disorders, and a variable association with HS and FS. The genetics of these conditions is largely unknown and the hope for the future is that the identification of FMTLE genes will lead to more appropriate approaches for the diagnosis and treatment of TLE.

SUMMARY: Panic disorder is a psychiatric diagnosis whose main feature is paroxysmal attacks of anxiety that strike suddenly without apparent provocation. Physicians explain the attacks as an ictal phenomenon in patients with known seizures because of their similarities to complex partial seizures. We report eight patients with seizures and panic disorder. Recognition of a second diagnostic entity resulted in a beneficial change in treatment in six of the eight. We did not find an increased incidence of panic disorder in our seizure clinic population as compared with the genera] population. RESUME L'attaque de panique est un diagnostic psychiatrique caracterise surtout par la survenue soudaine, sans provocation apparente, d'une crise d'anxiete Il existe des similarites avec les crises partielles complexes et, chez les patients presentant des crises documentees, on peut etre tente d'expliquer ces attaques comme un phenomene critique. Les auteurs rapportent 8 patients presentant a la fois des crises epileptiques et des attaques de panique. La reconnaissance d'un second diagnostic a permis d'obtenir une amelioration du traitement chez six patients sur huit. Les auteurs n'ont pas constate d'incidence augmentee des attaques de panique chez les patients frequentant une consultation epileptologique, par comparaison avec la population generale. RESUMEN Los trastornos de panico representan un diagnostico psiquiatrico cuyo principal componente consiste en ataques paroxisticos de ansiedad que aparecen repentinamente sin ningun tipo de provocacion. Teniendo en cuenta su similaridad con ataques parciales complejos puede producirse la tendencia de entender estos ataques como fenomenos ictales en pacientes que tienen ya ataques epilepticos. Los autores presentan ocho enfermos con ataques y trastornos de panico. El reconocimiento de un segundo diagnostico condujo a un cambio beneficioso en el tratamiento de seis de los ocho enfermos. Los autores no han encontrado un incremento de la incidencia de los trastornos de panico en las clinicas de ataques comporandolos con la poblacion general. ZUSAMMENFASSUNG Panik-Attacken stellen eine psychiatrische Diagnose dar, deren Hauptmerkmal paroxysmale Angstzustande sind, die plotzlich ohne sichtbare Provokation auftreten. Wegen ihrer Ahnlichkeit mit komplexen Patialanfalien, konnen diese Zustande leicht als iktale Phanomene des Anfalls-Patienten gedeutet werden. Wir berichten uber 8 Patienten mit Anfallen und mit Panik-Attacken. Die Entdeckung der zweiten Diagnose hatte einen gunstigen Einfluβ auf die Behandlung bei 6 von 8 Patienten zur Folge. Gegenuber der Gesamtbevolkerung fanden wir keine erhohte Inzidenz der Panik-Attacken bei unseren epileptischen Patienten.

Sialidosis type I has been described in several ethnic groups but to the best of our knowledge has not been reported in Indian families. We report on the clinical, biochemical and electrophysiological features in three siblings born to parents of South Indian origin. The diagnosis was missed for two years as they were labelled as cases of Ramsay-Hunt Syndrome.

The diagnostic utility of different noninvasive electrode placements for deriving the electroencephalogram and detecting interictal epileptiform discharges was compared. Anterior temporal and nasopharyngeal electrodes in combination with routine scalp electrodes detected over 97% of the spikes, whereas recording from only standard electrode placements detected 58%. Minisphenoidal and surface sphenoidal electrodes were generally not helpful. In some circumstances, however, the use of surface sphenoidal electrodes provided important confirmatory information. In no case did the minisphenoidal electrodes provide unique information, and their use seems unjustified, although the inclusion of other nonstandard electrodes in the recording montage is important to increase the yield.

The EEG of 45 patients with complex partial epilepsy was recorded from standard and supplementary inferior temporal electrode sites for 2 or more days via cable telemetry onto video (VHS) tape (22-25 channels, common reference). Epochs with "temporal spikes" were read into a topographic EEG device where individual spikes were visually identified and averaged in sums of 8-32. Analysis of spike voltage topography revealed two distinct patterns - dipolar, Type 1 and non-dipolar, Type 2. One or the other spike type predominated in all but two patients. Application of source modeling techniques (3 shells, single dipole, 6 parameters) to the spike topography data revealed that both spike types had similar equivalent dipoles in terms of location and orientation, except for vector elevation. However, calculated dipoles for Type 1 spikes were more stable over the course of the spike peak. Correlations with clinical data and intracranial EEG suggest that Type 1 spikes originate in mesial temporal structures, while Type 2 spikes arise from temporal or frontal neocortex. Spike voltage topography and equivalent dipole localization appear to be useful in the presurgical evaluation of patients with focal epilepsy.

Complex motor behaviors differing from typical automatisms were found in 12 of 502 patients with temporal lobe epilepsy. Movements involved proximal limb segments (6) or body axis (6) and were often preceded by auras and followed by automatisms. Seven of 12 patients are seizure free after surgery. The other 5 patients declined surgery.

While voxel-based 3-D MRI analysis methods as well as assessment of subtracted ictal versus interictal perfusion studies (SISCOM) have proven their potential in the detection of lesions in focal epilepsy, a combined approach has not yet been reported. The present study investigates if individual automated voxel-based 3-D MRI analyses combined with SISCOM studies contribute to an enhanced detection of mesiotemporal epileptogenic foci. Seven consecutive patients with refractory complex partial epilepsy were prospectively evaluated by SISCOM and voxel-based 3-D MRI analysis. The functional perfusion maps and voxel-based statistical maps were coregistered in 3-D space. In five patients with temporal lobe epilepsy (TLE), the area of ictal hyperperfusion and corresponding structural abnormalities detected by 3-D MRI analysis were identified within the same temporal lobe. In two patients, additional structural and functional abnormalities were detected beyond the mesial temporal lobe. Five patients with TLE underwent epileptic surgery with favourable postoperative outcome (Engel class Ia and Ib) after 3-5 years of follow-up, while two patients remained on conservative treatment. In summary, multimodal assessment of structural abnormalities by voxel-based analysis and SISCOM may contribute to advanced observer-independent preoperative assessment of seizure origin.

Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-27T11:21:12Z No. of bitstreams: 0Bitstream added on 2014-05-27T14:44:04Z : No. of bitstreams: 1 2-s2.0-10944270571.pdf: 58041 bytes, checksum: 1cea34506b451376f9fc90111263e486 (MD5) Made available in DSpace on 2014-05-27T11:21:12Z (GMT). No. of bitstreams: 0 Previous issue date: 2004-12-01 Objective: To compare the performance of patients with complex partial epilepsy with the normal controls in the subtests of an instrument used to assess intelligence function. Method: Fifty epileptic patients, whose ages ranged from 19 to 49 years and 20 normal controls without any neuropsychiatric disorders. The Wechsler-Bellevue adult intelligence test was applied in groups, epileptic patients and control subjects. This test is composed of several subtests that assess specific cognitive functions. A statistical analysis was performed using non-parametric tests. Results: All the Wechsler-Bellevue subtests revealed that the intelligence functions of the patients were significantly inferior to that of the controls (p

When considering surgery in children, it is important to think about the potential effect on psychiatric and behavioral outcome. A growing body of evidence suggests that the type of epilepsy (benign or catastrophic) and the age of the patient affect developmental outcome. In younger children with catastrophic epilepsy, the primary goal of therapy is not only to control seizures, but also to offer the child the opportunity for the best possible development. In older children with partial seizures, the therapeutic goal is to control seizures, which may allow a more normal life and improve behavior. Epilepsy surgery generally appears to have a positive effect on behavior and development in many younger children with catastrophic epilepsy. Psychiatric and behavioral outcomes in older children with complex partial epilepsy are less clear; many patients improve, but there does appear to be a small risk of developing new psychiatric or behavioral disorders postoperatively.

A 32-year-old man with mental retardation and uncontrolled complex partial epilepsy receiving carbamazepine (CBZ) and divalproex sodium (VPA), developed frequent episodes of forced upward gaze after increase in the daily VPA dosage. CBZ dosage was decreased, with prompt resolution of symptoms. The upward gaze problem recurred several months later. CBZ dosage was decreased further with subsequent resolution of symptoms. Therefore, the oculogyric crisis (OGC) appeared to be induced by CBZ.

Peripheral distal neuropathy associated with hypoglycemia secondary to insulinoma is quite rare. So far, less than 40 cases have been reported in literature. In this report, we describe a 50-year-old patient with insulinoma-polineuropathy and neuropsychiatric symptoms, interpreted as temporal lobe epilepsy, over the preceding 7 years. Due to the variability of the clinical presentation, diagnostic mistakes are frequent, and diagnosis of insulinoma is often delayed. Thus, the hypoglycemic nature of neuropathy can be lately recognized.

A study of concentrated attention patterns in epileptic patients was conducted with the objectives: characterization of the patients' epileptic condition; assessment of the concentrated attention levels in epileptic and nonepileptic individuals; comparison of the attention levels of the two groups. An evaluation was performed of 50 adult outpatients with complex partial seizures and 20 non-epileptic individuals (comparative group) at the Neuroepilepsy Ambulatory Unit, State University of Campinas SP, Brazil. METHOD: characterization of seizure types, frequency and duration; concentrated attention assessment (Concentrated Attention Test - Toulouse-Piéron); comparison of the epileptic with non-epileptic individuals. RESULTS: A statistically significant difference was observed between the groups with regard to Correct Response, Wrong Response and No Response. A difference was observed in relation to Time, but it was statistically insignificant. The epileptic patients presented inferior cognitive performance in relation to concentrated attention when compared with the non-epileptic individuals, findings compatible with the clinical complaints. Estudou-se o padrão da atenção concentrada em pacientes epilépticos, com o objetivo de se caracterizar a condição epiléptica, avaliar os níveis de atenção concentrada em epilépticos e em sujeitos não-epilépticos e comparar esses níveis entre os grupos. Foram avaliados 50 pacientes adultos com crises parciais complexas em atendimento no Ambulatório de Neuroepilepsia da Universidade Estadual de Campinas SP, Brasil. Também foram estudados 20 sujeitos sem epilepsia, para efeito comparativo. MÉTODO: caracterização tipológica, freqüência e duração das crises; avaliação da atenção concentrada (Teste de Atenção Concentrada - Toulouse-Piéron); e comparação dos resultados dos pacientes dos pacientes com os sujeitos não-epilépticos. RESULTADOS: Houve diferença estatisticamente significativa entre ambos os grupos nos itens Acertos e Erros + Ausências. No item Tempo houve diferença, mas sem significância estatística. Os pacientes apresentaram desempenhos cognitivos inferiores nos padrões de atenção concentrada em comparação com os não-epilépticos, resultados esses compatíveis com as queixas clínicas.