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International audience; Background: Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is usually associated with a poor response to antiseizure medications. We focused on MTLE-HS patients who were seizure free on medication to: (1) determine the clinical factors associated with seizure freedom and (2) develop a machine-learning classifier to better earlier identify those patients.Methods: We performed a retrospective, multicentric study comparing 64 medically treated seizure-free MTLE-HS patients with 200 surgically treated drug-resistant MTLE-HS patients. First, we collected medical history and seizure semiology data. Then, we developed a machine-learning classifier based on clinical data.Results: Medically treated seizure-free MTLE-HS patients were seizure-free for at least 2 years, and for a median time of 7 years at last follow-up. Compared to drug-resistant MTLE-HS patients, they exhibited: an older age at epilepsy onset (22.5 vs 8.0 years, p < 0.001), a lesser rate of: febrile seizures (39.0% vs 57.5%, p = 0.035), focal aware seizures (previously referred to as aura)(56.7% vs 90.0%, p < 0.001), autonomic focal aware seizures in presence of focal aware seizure (17.6% vs 59.4%, p < 0.001), dystonic posturing of the limbs (9.8% vs 47.0%, p < 0.001), gestural (27.4% vs 94.0%, p < 0.001), oro-alimentary (32.3% vs 75.5%, p < 0.001) or verbal automatisms (12.9% vs 36.0%, p = 0.001). The classifier had a positive predictive value of 0.889, a sensitivity of 0.727, a specificity of 0.962, a negative predictive value of 0.893.Conclusions: Medically treated seizure-free MTLE-HS patients exhibit a distinct clinical profile. A classifier built with readily available clinical data can identify them accurately with excellent positive predictive value. This may help to individualize the management of MTLE-HS patients according to their expected pharmacosensitivity.

International audience; Background and purpose - A clinical risk score for sudden unexpected death in epilepsy (SUDEP) in patients with drug-resistant focal epilepsy could help improve prevention. Methods - A case-control study was conducted including (i) definite or probable SUDEP cases collected by the French National Sentinel Mortality Epilepsy Network and (ii) control patients from the French national research database of epilepsy monitoring units. Patients with drug-resistant focal epilepsy were eligible. Multiple logistic regressions were performed. After sensitivity analysis and internal validation, a simplified risk score was developed from the selected variables. Results - Sixty-two SUDEP cases and 620 controls were included. Of 21 potential predictors explored, seven were ultimately selected, including generalized seizure frequency (>1/month vs.

Objective To evaluate the efficiency of resective epilepsy surgery (RES) in patients over 50 years and determine prognostic factors. Results Over the 147 patients over 50 years (54.9 ± 3.8 years [50–69]) coming from 8 specialized French centres for epilepsy surgery, 72.1%, patients were seizure-free and 91.2% had a good outcome 12 months after RES. Seizure freedom was not associated with the age at surgery or duration of epilepsy. In multivariate analysis, seizure freedom was associated with MRI and neuropathological hippocampal sclerosis (HS) (P = 0.009 and P = 0.028 respectively), PET hypometabolism (P = 0.013), temporal epilepsy (P = 0.01). On the contrary, the need for intracranial exploration was associated with a poorer prognosis (P = 0.001). Postoperative number of antiepileptic drugs was significantly lower in the seizure-free group (P = 0.001). Neurological adverse event rate after surgery was 21.1% and 11.7% of patients had neuropsychological adverse effects overall transient. Conclusions RES is effective procedure in the elderly. Even safe it remains at higher risk of complication and population should be carefully selected. Nevertheless, age should not be considered as a limiting factor, especially when good prognostic factors are identified.

Objectives (i) to create a shortened version of the Action Research Arm Test scale, (ii) to investigate its psychometric properties compared to the original scale and (iii) to externally validate it within an independent cohort. Design Prospective longitudinal cohort study. Settings Two University Hospitals (France, Switzerland). Participants 47 patients with poststroke motor deficits of the upper limb coming from two different sites were included and divided into two cohorts (n = 22 for the construction cohort; n = 25 for the validation cohort). Main Measures We used the first cohort to build the Mini-ARAT by shortening the Action Research Arm Test scale on the basis of ceiling/floor effects and collinearity of the subscales. We studied its reliability, validity, and responsiveness and performed an external validation with the second cohort. Results The Mini-ARAT consisted of 2 subscales from the original Action Research Arm Test scale (Grip and Pinch). Internal consistency (α = 87) and inter-rater reliability (0.99, 95% CI: 0.98–0.99, p Conclusion The Mini-ARAT is a time-effective tool able to capture the dynamics of motor deficits with high reliability and consistency, providing excellent information about residual motor functions, which is critically important for clinical and research purposes.

Objective To highlight specific characteristics of seizure semiology and EEG features associated with different subtypes of autoimmune encephalitis (AE). Methods We systematically reviewed the seizure semiology and all the EEG recordings from patients with AE managed in a tertiary referral centre for epilepsy and a neuro-intensive care unit. Each characteristic across the different subtypes of AE was compared by post hoc analysis. Results We identified 66 patients with anti-neuronal antibody-mediated AE or Rasmussen's encephalitis (RE) experiencing seizures, which were the most frequent symptom at onset. Anti-NMDAR and anti-LGI1 AE accounted for the majority of patients; 41% and 24%, respectively. We isolated specific semiological features, such as early tonic-clonic seizures (TCS) in anti-NMDAR AE, early mesial temporal lobe seizures with emotional symptoms in anti-GAD AE, somatosensory seizures in RE, and a lower frequency of TCS in anti-LGI1 AE. EEG analysis also provided additional insights into distinguishing the subtypes based on: (1) generalized rhythmic delta activity, which was more sensitive than extreme delta brush in identifying anti-NMDAR AE among all subtypes; and (2) temporal interictal epileptiform activity and temporal seizures on EEG in anti-GAD AE. We identified a new EEG pattern consisting of temporal low-voltage and periodic spikes associated with ipsilateral hippocampal abnormalities on MRI, which could be a sign of inflammatory mesial temporal involvement. Significance Specific clinical and EEG features can be useful in guiding the diagnosis of a subtype of AE with acute symptomatic seizures, particularly before the results of anti-neuronal antibody testing are available.

Anterior fossa dural arteriovenous fistulas (AF-DAVF) usually display a cortical venous drainage and are therefore at risk for rupture. Microsurgery is traditionally considered in many centers as the first-line treatment since endovascular treatment (EVT) entails a lower cure rate and significant ophthalmic risks. The anterior interhemispheric approach (AIA), originally described by Mayfrank in 1996, seems to offer the effectiveness of microsurgery while limiting the risks related to subfrontal craniotomy. The objective of this study was to analyze the surgical outcomes of patients who underwent this surgical approach for the treatment of AF-DAVF. We hereby describe our 10 years' experience of patients treated for an AF-DAVF with this technique in our institution and retrospectively analyzed our results. In addition, we describe our operative technique and its specificities. Eleven patients with AF-DAVF were included in our study. The definitive cure of the fistula was confirmed in all cases with postoperative cerebral angiography. All patients had a good neurological outcome and no major complication occurred. Brain retractors were never used during surgery, the frontal sinus was never opened neither, and anosmia was never observed after surgery. Anterior interhemispheric approach seems to be safe and effective to treat AF-DAVF with lower risks than other surgical approaches. This technique could be more widely considered when facing such midline vascular lesion.

BACKGROUND Psychogenic non-epileptic seizure-status (PNES-status), defined by psychogenic non-epileptic seizures (PNES) over 30 min, are often misdiagnosed as status epilepticus. We aimed to describe the features of patients who experienced PNES-status, admitted to an intensive care unit (ICU). METHODS We screened the patients hospitalized in our epilepsy unit during a 4-year period, with a diagnosis of PNES-status and ICU admission. RESULTS Among 171 patients with PNES, we identified 25 patients (15%) who presented 39 episodes of PNES-status leading to ICU admission. Some 76% of the patients were women. The median age at the time of the PNES-status episode was 35 years. Half (48%) alleged a history of epilepsy, but epilepsy was confirmed in only 12%. A history of psychiatric disease was found in 68%. PNES were present in 85% of patients before PNES-status, and semiology of PNES and PNES-status was similar for 79% of the patients, including hyperkinetic movements in 95% of the episodes and suspected loss of consciousness in 87%. Benzodiazepines were administrated in 77% of the episodes, antiepileptic drugs in 87%, and antibiotherapy for a ICU-related infection in 15% of the episodes. Oral intubation was performed in 41% of the episodes. Blood tests showed normal levels of creatine phosphokinase and leucocytes in 90% and 95% of the episodes, respectively. No epileptic activity was found during per-event electroencephalography but interictal epileptic activity was found in 10% of the episodes. CONCLUSION Hyperkinetic PNES-status should always be considered as a differential diagnosis of status epilepticus, with a high risk of iatrogenic consequences.

Large-scale interactions among multiple brain regions manifest as bursts of activations, called neuronal avalanches, which reconfigure according to the task at hand and, hence, might constitute natural candidates to design brain-computer interfaces (BCI). To test this hypothesis, we compared source-reconstructed magneto/electroencephalography during resting-state and a motor imagery task performed within a BCI protocol. For each condition, we defined an individual avalanche transition matrix, tracking the probability that an avalanche would spread across any two regions. The edges whose transition probabilities significantly differed between conditions hinged selectively on premotor regions in all subjects, defining a topography related to the task. Furthermore, the individual differences in the transition probabilities for edges between pre/motor regions and parietal ones positively related to the individual task performance. Our results show that the patterns of propagation of large-scale perturbations are related to behavior and can be used to inform brain-computer interfaces.

Cognitive impairment is an important comorbidity of refractory temporal lobe epilepsy (TLE). We aimed to explore the impact of (i) specific lesions, such as dysembryoplastic neuroepithelial tumor (DNET), dysplasia, or hippocampal sclerosis, (ii) focus localization (medial versus lateral) and (iii) focus lateralization (right versus left) on the neuropsychological profile of refractory TLE adult patients. We examined the neuropsychological characteristics of 312 adults with refractory TLE: 100 patients without hippocampal sclerosis (HS) and 212 with HS. Scores on tests of intelligence (Global IQ, Verbal IQ and Performance IQ), working memory, episodic memory (verbal and visual learning and forgetting), executive functions and language abilities were analyzed. Three main factors influenced the neuropsychological profile of refractory TLE patients: (i) the lesion, patients with HS obtaining poorer cognitive performances than patients without HS and specifically DNET patients performing better than patients with HS, (ii) the focus side, that seems only relevant for verbal memory abilities which are affected in left but not right TLE patients and (iii) the localization of seizure focus, patients with medial TLE exhibiting lower memory performances than patients with lateral TLE. Lesion, localization and lateralization are major contributors of the cognitive impairment depicted in TLE. Hippocampal sclerosis appears as the main contributor.

The rapid urbanization of the past decades has forced numerous species to adapt to their new environments over a very restricted time scale. Previous studies suggested that individuals living in urban areas have specific characteristics as compared to those living in rural areas. In feral pigeon populations (Columba livia), individuals living in cities are more melanic than those living in less urbanized areas. In this study, we tested whether the degree of eumelanin-based coloration in pigeons reflects certain adaptations to urban areas such as boldness and tolerance to stress. Hence, we examined the relationship between eumelanin-based coloration and three variables of boldness (arrival to a new food source, arrival latency, and flight distance) and tolerance to stress (breath rate). Our results show that the eumelanin-based coloration of individuals is not correlated with the boldness variables, but tends to be positively related to breath rate in adults. Therefore, eumelanin-based coloration could reflect the capacity of adult individuals to manage stress in an urban environment, but not the boldness. The higher frequency of eumelanic pigeons observed in cities might thus be due to urban selective forces that favor individuals with a higher response to stress than bolder individuals.

La Société de réanimation de langue française et la Société française de médecine d’urgence ont décidé d’élaborer de nouvelles recommandations sur la prise en charge de l’état mal épileptique (EME) avec l’ambition de répondre le plus possible aux nombreuses questions pratiques que soulèvent les EME : diagnostic, enquête étiologique, traitement non spécifique et spécifique. Vingt-cinq experts ont analysé la littérature scientifique et formulé des recommandations selon la méthodologie GRADE. Les experts se sont accordés sur 96 recommandations. Les recommandations avec le niveau de preuve le plus fort ne concernent que l’EME tonico-clonique généralisé (EMTCG) : l’usage des benzodiazépines en première ligne (clonazépam en intraveineux direct ou midazolam en intramusculaire) est recommandé, répété 5 min après la première injection (à l’exception du midazolam) en cas de persistance clinique. En cas de persistance 5 min après cette seconde injection, il est proposé d’administrer la seconde ligne thérapeutique : valproate de sodium, (fos-)phénytoïne, phénobarbital ou lévétiracétam. La persistance avérée de convulsions 30 min après le début de l’administration du traitement de deuxième ligne signe l’EMETCG réfractaire. Il est alors proposé de recourir à un coma thérapeutique au moyen d’un agent anesthésique intraveineux de type midazolam ou propofol. Des recommandations spécifiques à l’enfant et aux autres EME sont aussi énoncées.

International audience; Aim: KCNB1 encephalopathy encompasses a broad phenotypic spectrum associating intellectual disability, behavioral disturbances, and epilepsies of various severity. Using standardized parental questionnaires, we aimed to capture the heterogeneity of the adaptive and behavioral features in a series of patients with KCNB1 pathogenic variants.Methods: We included 25 patients with a KCNB1 encephalopathy, aged from 3.2 to 34.1 years (median = 10 years). Adaptive functioning was assessed in all patients using the French version of the Vineland Adaptive Behavior Scales, Second Edition (VABS-II) questionnaire. We screened global behavior with the Childhood Behavioral Check-List (CBCL, Achenbach) and autism spectrum disorder (ASD) with the Social Communication Questionnaire (SCQ). We used a cluster analysis to identify subgroups of adaptive profiles.Results: VABS-II questionnaire showed pathological adaptive behavior in all participants with a severity of adaptive deficiency ranging from mild in 8/20 to severe in 7/20. Eight out of 16 were at risk of Attention Problems at the CBCL and 13/18 were at risk of autism spectrum disorder (ASD). The adaptive behavior composite score significantly decreased with age (Spearman’s Rho=-0.72, p

Introduction: Epilepsy is one of the most common brain conditions, which affects over 70 million people worldwide, with a higher risk for people over the age of 70. In the other hand, dementia prevalence is up to 7% of individuals above the age of 65 years, and concerns 55 million people worldwide. Several studies were conducted to pursue causal relationship or bidirectional connection between those two pathologies and one of the latest systemic reviews gathering a numerous studies about this subject was published in 2017. By reviewing the most relevant articles in the literature we aimed to clarify the interconnection between epilepsy and dementia: coincidence due to the highest incidence in the elderly, or bidirectional relationship? We also focused our research on the newest studies about the possible impact of anti-epileptic drugs to improve dementia and vice versa. Methods: Due to the large numbers of articles about epilepsy and dementia, we collected twenty-three studies and reviews from 2014 to 2021 that focused on the relationship between those two pathologies, also some latest publications, especially epidemiological ones, concerning epilepsy and dementia. Results: Dementia is a heterogeneous syndrome that can be caused by numerous neurological and medical conditions. Epilepsy is more frequent in the elderly and is significantly higher in elderly patients with dementia than in elderly patients without dementia, whatever the type of dementia (AD, Lewy Body dementia, FTD,…). Numerous studies show that the high incidence of both diseases is not a coincidence of ageing and shared risk factors (i.e cerebrovascular events) and that the two diseases share common pathophysiological mechanisms. In the future, antiseizure approaches would complement and potentially enhance other strategies for treating AD, including that targeting disease protein aggregation.

Focal neuronal lipofuscinosis (FNL) is an uncommon epileptic disorder related to an excess of lipofuscin accumulation within dysmorphic-appearing neurons (DANs), whose epileptogenic mechanisms are still poorly understood. It shares some clinical and neuroimaging similarities with focal cortical dysplasia of type IIb (FCDIIb), but it represents a different pathological entity. Here, we identified two patients with FNL among a 10-year cohort of 323 patients who underwent neurosurgery for a focal pharmacoresistant epilepsy. We describe the electroclinical, metabolic and neuropathological features of both patients with FNL who benefited from a comprehensive presurgical investigation. While the previous reports showed frontal lobe localization of the lesion, FNL was identified in the temporal lobe, in one of our patients. EEG investigations in both patients showed striking focal and rich interictal activity resembling that described in FCDIIb. Besides focal intraneuronal lipofuscin accumulation, the neuropathological analysis demonstrated that somata of DANs were surrounded by a large amount of GABAergic presynaptic buttons, suggesting the involvement of interneurons in the epileptogenicity of FNL. To further explore the role of GABAergic transmission in the generation of epileptiform activity in FNL, we performed in vitro multi-electrode array recordings on the post-surgery tissue from one patient. Spontaneous interictal-like discharges (IILDs) were identified only in the restricted area displaying the highest density of lipofuscin-containing DANs, suggesting a close correlation between the density of lipofuscin-containing neurons and epileptogenicity. Moreover, IILDs were blocked by the GABAA receptor antagonist gabazine. All together, these findings showed how GABA signaling may contribute to the generation of interictal-like activity in FNL tissue.

International audience; Patients with chronic neurological diseases may have predisposing risk factors for severe COVID-19 and should be considered as priority candidates for SARS-CoV-2 vaccination. Nevertheless, the safety of RNA vaccine was evaluated in healthy volunteers or in patients with stable chronic medical conditions excluding patients with chronic neurological diseases. We report here the early tolerability of Comirnaty vaccine in 36 patients with chronic neurological diseases and demonstrate good early tolerability, better than found in healthy people in phase 3 trials.

International audience; Conditions experienced during early development can lead to profound long-lasting changes in physiology and behaviour.The extent to which such “programming” effects are transmitted to the next generation remains largely unexplored. Here, weassessed whether maternal exposure to elevated corticosterone stress hormone during early post-natal development had animpact on neophobia and antipredator behaviour in the offspring. Our data showed that maternal early-life hormonal manipulationhad no impact on offspring behavioural traits. This occurred despite the treatment associated changes to metabolism,physiology and behaviour of the study mothers up until adulthood, as previously reported.

Anterior fossa dural arteriovenous fistulas (AF-DAVF) usually display a cortical venous drainage and are therefore at risk for rupture. Microsurgery is traditionally considered in many centers as the first-line treatment since endovascular treatment (EVT) entails a lower cure rate and significant ophthalmic risks. The anterior interhemispheric approach (AIA), originally described by Mayfrank in 1996, seems to offer the effectiveness of microsurgery while limiting the risks related to subfrontal craniotomy. The objective of this study was to analyze the surgical outcomes of patients who underwent this surgical approach for the treatment of AF-DAVF. We hereby describe our 10 years' experience of patients treated for an AF-DAVF with this technique in our institution and retrospectively analyzed our results. In addition, we describe our operative technique and its specificities. Eleven patients with AF-DAVF were included in our study. The definitive cure of the fistula was confirmed in all cases with postoperative cerebral angiography. All patients had a good neurological outcome and no major complication occurred. Brain retractors were never used during surgery, the frontal sinus was never opened neither, and anosmia was never observed after surgery. Anterior interhemispheric approach seems to be safe and effective to treat AF-DAVF with lower risks than other surgical approaches. This technique could be more widely considered when facing such midline vascular lesion.

International audience; Objective: Lateral temporal lobe epilepsies (LTLE) are poorly characterized heterogeneous epilepsies. As the lateral temporal lobe supports distinct functions, we hypothesized that neuropsychological profiles could differ according to the localization of the seizure focus within the lateral temporal lobe. Methods: We retrospectively examined the neuropsychological characteristics of 74 consecutive patients with refractory LTLE assessed in the context of a presurgical investigation at the Pitié-Salpêtrière Hospital in Paris between 1998 and 2018. Precise localization of the epileptic focus was correlated with scores on tests of intelligence (Global, Verbal and Performance IQ), working memory, episodic memory (verbal and visual learning and forgetting), executive functions and language abilities. Results: : We demonstrated an impact of the localization of the epileptic focus within the lateral temporal lobe with worse learning and/or executive performances depicted in the infero-basal and pure pole LTLE groups and greater language difficulties in the posterior LTLE group, Antiepileptic drugs had a greater effect than parameters related to the epilepsy itself as the lesion or the disease duration, and finally as in medial TLE, the age, the education, and the sex influenced some cognitive performances. Conclusion: Our findings show that the lateral temporal neocortex is also part of the neural substrate for memory processing and executive functions and suggest that this involvement could be related to functions devoted to specific subregions of the temporal lobe (i.e temporal pole, inferior and basal regions) that support language and semantic processing.

International audience; Historically, the anterior part of the temporal lobe was labelled as a unique structure named Brain Area 38 by Brodmann or Temporopolar Area TG by Von Economo, but its functions were unknown at that time. Later on, a few studies proposed to divide the temporal pole in several different subparts, based on distinct cytoarchitectural structure or connectivity patterns, while a still growing number of studies have associated the temporal pole with many cognitive functions. In this review, we provide an overview of the temporal pole anatomical and histological structure and its various functions. We performed a literature review of articles published prior to September 30, 2020 that included 112 articles. The temporal pole has thereby been associated with several high-level cognitive processes: visual processing for complex objects and face recognition, autobiographic memory, naming and word-object labelling, semantic processing in all modalities, and socio-emotional processing, as demonstrated in healthy subjects and in patients with neurological or psychiatric diseases, especially in the field of neurodegenerative disorders. A good knowledge of those functions and the symptoms associated with temporal pole lesions or dysfunctions is helpful to identify these diseases, whose diagnosis may otherwise be difficult.

International audience; Epilepsy is one of the most common chronic disorders affecting women of childbearing age. Unfortunately, many women with epilepsy (WWE) still report not receiving key information about pregnancy. They obviously need information about epilepsy and pregnancy prior to conception with a particular emphasis on effective birth control (i.e. contraception), necessity to plan pregnancy, antiepileptic drugs optimization, and folate supplementation. The risks associated with use of antiepileptic drugs during pregnancy have to be balanced against fetal and maternal risks associated with uncontrolled seizures. This report reviews evidence-based counseling and management strategies concerning maternal and fetal risks associated with seizures, teratogenic risks associated with antiepileptic drug exposure with a special emphasis on developmental and behavioural outcomes of children exposed to intra utero antiepileptic drugs.

International audience; In altricial species, parents brood their chicks constantly before leaving them unattended sometimes for extendedperiods when they become thermally independent. During this second phase, there is sometimes important interindividualdifferences in parental attendance and the fitness costs and benefits of parental strategies have previouslybeen extensively investigated. However, the impact of parental presence on offspring behaviors andstress physiology has been overlooked. Here, we examined the influence of parental presence on offspringhormonal and behavioral stress sensitivities in snow petrel chicks. We demonstrated for the first time in a wildbird species that attended chicks had lower stress-induced corticosterone levels and a lower probability to showdefensive behavior compared to the alone chicks. This reduced stress sensitivity is certainly explained by thewell-known link between corticosterone and nutritional status, and by the recent delivery of meals to theattended chicks and the improvement of their nutritional status. It may also be explained by the parental protectionagainst predators or inclement weather, or/and by the psychosocial comfort of parental presence for theoffspring. Overall, these results suggest that the presence of a parent in the nest reduces offspring stress sensitivityin wild birds. Further studies would now be required to disentangle the impact of nutritional status andparental presence on stress sensitivity and to better understand the potential impact of parental presence andcirculating corticosterone levels on growth and cognitive development in wild birds.

Objective: We aimed to delineate the phenotypic spectrum and long-term outcome of individuals with KCNB1 encephalopathy. Methods: We collected genetic, clinical, electroencephalographic, and imaging data of individuals with KCNB1 pathogenic variants recruited through an international collaboration, with the support of the family association “KCNB1 France.” Patients were classified as having developmental and epileptic encephalopathy (DEE) or developmental encephalopathy (DE). In addition, we reviewed published cases and provided the longterm outcome in patients older than 12 years from our series and from literature. Results: Our series included 36 patients (21 males, median age = 10 years, range = 1.6 months-34 years). Twenty patients (56%) had DEE with infantile onset seizures (seizure onset = 10 months, range = 10 days-3.5 years), whereas 16 (33%) had DE with late onset epilepsy in 10 (seizure onset = 5 years, range = 18 months-25 years) and without epilepsy in six. Cognitive impairment was more severe in individuals with DEE compared to those with DE. Analysis of 73 individuals with KCNB1 pathogenic variants (36 from our series and 37 published individuals in nine reports) showed developmental delay in all with severe to profound intellectual disability in 67% (n = 41/61) and autistic features in 56% (n = 32/57). Long-term outcome in 22 individuals older than 12 years (14 in our series and eight published individuals) showed poor cognitive, psychiatric, and behavioral outcome. Epilepsy course was variable. Missense variants were associated with more frequent and more severe epilepsy compared to truncating variants. Significance: Our study describes the phenotypic spectrum of KCNB1 encephalopathy, which varies from severe DEE to DE with or without epilepsy. Although cognitive impairment is worse in patients with DEE, long-term outcome is poor for most and missense variants are associated with more severe epilepsy outcome. Further understanding of disease mechanisms should facilitate the development of targeted therapies, much needed to improve the neurodevelopmental prognosis.

PURPOSE:Psychological health at work for care providers is an important issue, because they are directly involved in quality of patient care. Managerial and organizational determinants have been found to be indicators of psychological health at work. The main objective of this study was to explore the relationships between the psychological health at work of pediatric oncology care workers with managerial and organizational determinants and with quality of care.MATERIALS AND METHODS:We performed regression analysis between psychological health at work (quality of work life [QWL], job satisfaction, and so on), managerial determinants (transformational leadership, perceived autonomy support), organizational determinants (organizational support, organizational justice, and participatory approach), and perceived quality of care.RESULTS:Participants were 510 health care professionals working in French pediatric oncology centers. No significant differences in the psychological health at work of the participants were found based on age, sex, length of employment, or professional discipline. In simple regression, significant associations were found between psychological health at work with all managerial and organizational determinants. In multiple regression, a significant link was found between QWL and perceived organizational support (β = .21; P < .001), organizational justice (β = .20, P < .001), and overall participatory approach (β = .10; P < .02). Job satisfaction was also related to perceived organizational support (β = .16; P < .01). Finally, perceived quality of care was linked to QWL (β = .15; P < .01) and job satisfaction (β = .30; P < .001).CONCLUSION:These results emphasize the importance of the role of managers and the organization in psychological health at work of health care providers and also in the quality of patient care

Temporal pole epilepsy (TPE) is a poorly known and difficult to individualize subtype of temporal lobe epilepsy. Consequently, in drug-resistant TPE, there is still a debate on the need for a large surgical removal of the temporal pole and mesial temporal structures or a limited resection of the temporal pole. We reviewed all patients who underwent presurgical evaluation for drug-resistant epilepsy over a 17-year period, and report here 19 patients with proven drug-resistant temporal pole epilepsy who underwent a selective temporal pole resection with respect to mesial structures. Most (15) TPE patients exhibited seizures resembling mesiotemporal seizures, whereas the others exhibited nocturnal hyperkinetic seizures or an association of both seizure types. MRI revealed a temporal pole lesion in 58% of patients. Long-term postoperative outcome after a conservative surgery was excellent: 63% of patients were seizure-free (International League Against Epilepsy [ILAE] 1) at 1-year postsurgery and 78% at 5 years. These results show that TPE has no specific electroclinical features but is a distinct type of temporal lobe epilepsy allowing a conservative surgery. Respecting the mesiotemporal structures is a valid surgical approach for drug-resistant temporal pole epilepsy.

Epilepsy is one of the most common neurological disorders, affecting approximately 50 million people worldwide. Despite a dramatic increase in treatment options over the past 30 years, it still ranks fourth in the world's disease burden. There are now close to 30 antiepileptic drugs (AEDs), with more than two thirds introduced to the market after carbamazepine (CBZ) and one third after its derivative, oxcarbazepine (OXC). Following the introduction of these newer AEDs, the role of CBZ and OXC in the therapeutic armamentarium for seizure control and effective epilepsy management needs to be reviewed. The main guidelines list both CBZ and OXC as first-line options or second-line alternatives for the treatment of focal-onset epilepsy and primary generalized tonic-clonic seizures. While evidence suggests that overall AEDs have similar efficacy, some newer AEDs may be better tolerated than CBZ. In line with this, there have been changes in treatment patterns, with many variations across different countries. However, CBZ remains among the two or three most prescribed drugs for focal epilepsy in many countries, and is widely used across Europe, Africa, South America, and Asia, where it represents a good compromise between cost, availability, and effectiveness. OXC is among the first-choice options for the initial treatment of focal-onset seizures in several countries, including the US and China, where the oral suspension is commonly prescribed. This review provides guidance on the optimal use of these two drugs in clinical practice, including in children, the elderly, and in pregnancy.

La Société de réanimation de langue française et la Société française de médecine d’urgence ont décidé d’élaborer denouvelles recommandations sur la prise en charge de l’état de mal épileptique (EME) avec l’ambition de répondre leplus possible aux nombreuses questions pratiques que soulèvent les EME : diagnostic, enquête étiologique, traitementnon spécifique et spécifique. Vingt‑cinq experts ont analysé la littérature scientifique et formulé des recommandationsselon la méthodologie GRADE. Les experts se sont accordés sur 96 recommandations. Les recommandations avecle niveau de preuve le plus fort ne concernent que l’EME tonicoclonique généralisé (EMETCG) : l’usage des benzodia‑zépines en première ligne (clonazépam en intraveineux [IV] direct ou midazolam en intramusculaire) est recommandé,répété cinq minutes après la première injection (à l’exception du midazolam) en cas de persistance clinique. En casde persistance cinq minutes après cette seconde injection, il est proposé d’administrer la deuxième ligne thérapeu‑tique : valproate de sodium, (fos‑)phénytoïne, phénobarbital ou lévétiracétam. La persistance avérée de convulsions30 minutes après le début de l’administration du traitement de deuxième ligne signe l’EMETCG réfractaire. Il est alorsproposé de recourir à un coma thérapeutique au moyen d’un agent anesthésique intraveineux de type midazolam oupropofol. Des recommandations spécifiques à l’enfant et aux autres EME sont aussi énoncées.

OBJECTIVE: To report 3 patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who developed generalized myoclonus. METHODS: Patient data were obtained from medical records from the University Hospital “12 de Octubre,” Madrid, Spain. RESULTS: Three patients (2 men and 1 woman, aged 63–88 years) presented with mild hypersomnia and generalized myoclonus following the onset of the so-called inflammatory phase of coronavirus disease 2019 (COVID-19). All of them had presented previously with anosmia. Myoclonus was generalized with both positive and negative jerks, predominantly involving the facial, trapezius, sternocleidomastoid, and upper extremities muscles. These myoclonic jerks occurred spontaneously and were extremely sensitive to multisensory stimuli (auditive and tactile) or voluntary movements, with an exaggerated startle response. Other causes of myoclonus were ruled out, and none of the patients had undergone respiratory arrest or significant prolonged hypoxia. All of them improved, at least partially, with immunotherapy. CONCLUSIONS: Our 3 cases highlight the occurrence of myoclonus during the COVID-19 pandemic as a post- or para-infectious immune-mediated disorder. However, we cannot rule out that SARS-CoV-2 may spread transneuronally to first- and second-order structures connected with the olfactory bulb. Further investigation is required to clarify the full clinical spectrum of neurologic symptoms and optimal treatment.

International audience; Brain-computer interfaces (BCIs) have been largely developed to allow communication, control, and neurofeedback in human beings. Despite their great potential, BCIs perform inconsistently across individuals and the neural processes that enable humans to achieve good control remain poorly understood. To address this question, we performed simultaneous high-density electroencephalographic (EEG) and magnetoencephalographic (MEG) recordings in a motor imagery-based BCI training involving a group of healthy subjects. After reconstructing the signals at the cortical level, we showed that the reinforcement of motor-related activity during the BCI skill acquisition is paralleled by a progressive disconnection of associative areas which were not directly targeted during the experiments. Notably, these network connectivity changes reflected growing automaticity associated with BCI performance and predicted future learning rate. Altogether, our findings provide new insights into the large-scale cortical organizational mechanisms underlying BCI learning, which have implications for the improvement of this technology in a broad range of real-life applications.

Older adults with epilepsy frequently report changes in memory function, with a concern about degenerative disease. However, the precise limit between memory changes related to the effects of longstanding chronic epilepsy and physiological aging on memory abilities is very difficult to specify. Knowledge about characteristics of physiological cognitive aging, its influence on memory test results, and its interactions with clinical variables related to epileptic disease are therefore essential to interpret memory scores. This paper aims at presenting an overview of the different factors that might modulate the developmental trajectory of episodic memory in chronic temporal lobe epilepsy. For this purpose, the effects of age on memory in healthy and epileptic persons will be first reviewed with a special focus on episodic memory functions in chronic temporal lobe epilepsy. Finally, scientific evidence supporting or not the presence of an accelerated memory decline in patients with longstanding epilepsy will be presented by discussing the interests and the limits of the existing literature. Identifying the best outcome measures to assess episodic memory and understanding the risk factors that might precipitate memory decline are key knowledge to improve the clinical care and quality of life of persons with chronic temporal lobe epilepsy.

Although most children with acute lymphoblastic leukemia (ALL) receive fractionated total body irradiation (FTBI) as myeloablative conditioning (MAC) for allogeneic hematopoietic stem cell transplantation (allo-HSCT), it is an important matter of debate if chemotherapy can effectively replace FTBI. To compare outcomes after FTBI versus chemotherapy-based conditioning (CC), we performed a retrospective EBMT registry study. Children aged 2–18 years after MAC for first allo-HSCT of bone marrow (BM) or peripheral blood stem cells (PBSC) from matched-related (MRD) or unrelated donors (UD) in first (CR1) or second remission (CR2) between 2000 and 2012 were included. Propensity score weighting was used to control pretreatment imbalances of the observed variables. 3.054 patients were analyzed. CR1 (1.498): median follow-up (FU) after FTBI (1.285) and CC (213) was 6.8 and 6.1 years. Survivals were not significantly different. CR2 (1.556): median FU after FTBI (1.345) and CC (211) was 6.2 years. Outcomes after FTBI were superior as compared with CC with regard to overall survival (OS), leukemia-free survival (LFS), relapse incidence (RI), and nonrelapse mortality (NRM). However, we must emphasize the preliminary character of the results of this retrospective “real-world-practice” study. These findings will be prospectively assessed in the ALL SCTped 2012 FORUM trial.

Cystic fibrosis, a rare, hereditary and chronic disease, affects the psychic functioning of children and their families. Since it is incurable, constant adjustments are essential to make sense of the inevitable evolution of the disease. The psychologists support the families and the patients so that all live as well as possible with cystic fibrosis.

International audience; Developmental and epileptic encephalopathies (DEE) refer to a heterogeneous group of devastating neurodevelopmental disorders. Variants in KCNB1 have been recently reported in patients with early-onset DEE. KCNB1 encodes the alpha subunit of the delayed-rectifier voltage-dependent potassium channel Kv 2.1. We review the 37 previously reported patients carrying 29 distinct KCNB1 variants and significantly expand the mutational spectrum describing 18 novel variants from 27 unreported patients. Most variants occur de novo and mainly consist of missense variants located on the voltage sensor and the pore domain of Kv 2.1. We also report the first inherited variant (p.Arg583*). KCNB1-related encephalopathies encompass a wide spectrum of neurodevelopmental disorders with predominant language difficulties and behavioral impairment. Eighty-five percent of patients developed epilepsies with variable syndromes and prognosis. Truncating variants in the C-terminal domain are associated with a less severe epileptic phenotype. Overall, this report provides an up-to-date review of the mutational and clinical spectrum of KCNB1, strengthening its place as a causal gene in DEEs and emphasizing the need for further functional studies to unravel the underlying mechanisms.