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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.

Functional neurological disorders (FND), a subtype of functional disorders (FD), are a frequent motive for neurology referrals. The various presentations and the unknown physiopathology of FD have led to the multiplication of terms describing these disorders over the years.We examined the FD-related articles published from 1960 to 2020 in PubMed and PsycINFO databases. We searched for: psychogenic, somatization, somatoform, medically unexplained symptoms, hysteria, conversion disorder, dissociative, functional neurological disorder, and functional disorder. Use rates in the title, abstract, keyword, or MeSH fields were collected over successive 5-year periods. After correcting for off-topic results, we examined proportional distribution over time, term associations, and disciplinary fields (neurology and psychiatry). Term impact was estimated via H-index and number of citations.We found that none of the terms is prevailing in the recent medical literature. We observed three trends in the use rates: stability, increase, and decrease of use over time. While most of the terms were present in a stable proportion of the publications, hysteria and psychogenic lost popularity over time. We found a differential preference for terminology between disciplines. Functional neurological disorder showed the highest citation impact, yielding 10% of highly cited publications.We found a dynamic and evolving use of the different terms describing FD in the last 60 years. Despite the tendency to use the term functional in the recent highly cited publications, its low prevalence and coexistence with several other terms suggest that a precise, explanatory and non-offensive term remains yet to be found.

Abnormal movements are intrinsic to some forms of endogenous psychoses. Spontaneous dyskinesias are observed in drug-naïve first-episode patients and at-risk subjects. However, recent descriptions of spontaneous dyskinesias may actually represent the rediscovery of a more complex phenomenon, 'parakinesia' which was described and documented in extensive cinematographic recordings and long-term observations by German and French neuropsychiatrists decades before the introduction of antipsychotics. With the emergence of drug induced movement disorders, the description of parakinesia has been refined to emphasize the features enabling differential diagnosis with tardive dyskinesia. Unfortunately, parakinesia was largely neglected by mainstream psychiatry to the point of being almost absent from the English-language literature. With the renewed interest in motor phenomena intrinsic to SSD, it was timely not only to raise awareness of parakinesia, but also to propose a scientifically usable definition for this phenomenon. Therefore, we conducted a Delphi consensus exercise with clinicians familiar with the concept of parakinesia. The original concept was separated into hyperkinetic parakinesia (HPk) as dyskinetic-like expressive movements and parakinetic psychomotricity (PPM), i.e., patient's departing from the patient's normal motion style. HPk prevails on the upper part of the face and body, resembling expressive and reactive gestures that not only occur inappropriately but also appear distorted. Abnormal movements vary in intensity depending on the level of psychomotor arousal and are thus abated by antipsychotics. HPk frequently co-occurs with PPM, in which gestures and mimics lose their naturalness and become awkward, disharmonious, stiff, mannered, and bizarre. Patients are never spontaneously aware of HPk or PPM, and the movements are never experienced as self-dystonic or self-alien. HPk and PPM are highly specific to endogenous psychoses, in which they are acquired and progressive, giving them prognostic value. Their differential diagnoses and correspondences with current international concepts are discussed.

To assess the prophylactic effect of anodal tDCS of the left motor cortex in patients with resistant chronic migraine (CM) and its long-term maintenance.In a patient-assessor blinded, sham-controlled trial, 36 patients were randomized to receive anodal tDCS (active group, n = 18) or sham tDCS (sham group, n = 18). The studied population was characterized by a previous failure of at least 3 classes of preventive drugs and a mean duration of migraine history of 26 years. The tDCS procedure consisted of an induction phase of 5 consecutive daily sessions (week 1) followed by a maintenance phase of 1 weekly session during the next 4 weeks and two bimonthly sessions in the next month, for a total of 11 sessions during 2 months. Anodal tDCS was delivered at 2 mA intensity for 20 min over the left motor cortex. The primary endpoint was the reduction in the monthly number of migraine attacks from baseline to each period of follow-up (months 1, 2, 3, 5) between the active and sham groups.The monthly number of migraine attacks expressed as the percentage of reduction from baseline was significantly reduced in the active versus the sham group, from the end of first month (-21% ± 22 vs. -2% ±25, p = 0.019) to the end of follow-up (3-month post-treatment) (-32% ± 33 vs. -6% ±39, p = 0.011). At this time, the rate of responders, defined as a reduction of the monthly number of migraine attacks ≥30% from baseline, was significantly higher in the active group than in the sham group (50% vs. 14%, p = 0.043).Our results show a marked prophylactic effect of anodal tDCS of the left motor cortex in resistant CM extending several months after the stimulation period, and suggest that this neuromodulatory approach may be part of the prophylactic alternatives available for CM.

International audience

Prophylaxis or treatment of tumor-associated seizures is adaily concern in neurosurgical practice but is often guided by the surgeon's habits rather than evidence from clinical trials, which is lacking. The present study reviews the literature on the incidence, clinical aspects and treatment of epilepsy and epileptic seizures in patients undergoing surgery for meningioma. Based on the published data, we then performed a French nationwide survey of neurosurgeons’ practices regarding perioperative management of meningioma-related epilepsy and epileptic seizures.

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.

BackgroundPathogenic KCNQ2 variants are a frequent cause of developmental and epileptic encephalopathy.MethodsWe recruited 13 adults (between 18 years and 45 years of age) with KCNQ2 encephalopathy and reviewed their clinical, EEG, neuroimaging and treatment history.ResultsWhile most patients had daily seizures at seizure onset, seizure frequency declined or remitted during childhood and adulthood. The most common seizure type was tonic seizures (early) infancy, and tonic-clonic and focal impaired awareness seizures later in life. Ten individuals (77%) were seizure-free at last follow-up. In 38% of the individuals, earlier periods of seizure freedom lasting a minimum of 2 years followed by seizure recurrence had occurred. Of the 10 seizure-free patients, 4 were receiving a single antiseizure medication (ASM, carbamazepine, lamotrigine or levetiracetam), and 2 had stopped taking ASM. Intellectual disability (ID) ranged from mild to profound, with the majority (54%) of individuals in the severe category. At last contact, six individuals (46%) remained unable to walk independently, six (46%) had limb spasticity and four (31%) tetraparesis/tetraplegia. Six (46%) remained non-verbal, 10 (77%) had autistic features/autism, 4 (31%) exhibited aggressive behaviour and 4 (31%) destructive behaviour with self-injury. Four patients had visual problems, thought to be related to prematurity in one. Sleep problems were seen in six (46%) individuals.ConclusionSeizure frequency declines over the years and most patients are seizure-free in adulthood. Longer seizure-free periods followed by seizure recurrence are common during childhood and adolescence. Most adult patients have severe ID. Motor, language and behavioural problems are an issue of continuous concern.

Background The vagus nerve has anti-inflammatory properties. We aimed to investigate vagus nerve stimulation (VNS) as a new therapeutic strategy targeting an intrinsic anti-inflammatory pathway in a pilot study in Crohn's disease patients. The main objectives addressed the questions of long-term safety, tolerability, and anti-inflammatory effects of this therapy. This study is the continuation of previous reported findings at 6 months. Methods Nine patients with moderate active disease underwent VNS. An electrode wrapped around the left cervical vagus nerve was continuously stimulated over 1 year. Clinical, biological, endoscopic parameters, cytokines (plasma, gut), and mucosal metabolites were followed-up. Key results After 1 year of VNS, five patients were in clinical remission and six in endoscopic remission. C-reactive protein (CRP) and fecal calprotectin decreased in six and five patients, respectively. Seven patients restored their vagal tone and decreased their digestive pain score. The patients' cytokinergic profile evolved toward a more "healthy profile": Interleukins 6, 23, 12, tumor necrosis factor α, and transforming growth factorβ1 were the most impacted cytokines. Correlations were observed between CRP and tumor necrosis factor α, and some gut mucosa metabolites as taurine, lactate, alanine, and beta-hydroxybutyrate. VNS was well tolerated. Conclusion & inferences Vagus nerve stimulation appears as an innovative and well-tolerated treatment in moderate Crohn's disease. After 12 months, VNS has restored a homeostatic vagal tone and reduced the inflammatory state of the patients. VNS has probably a global modulatory effect on the immune system along with gut metabolic regulations. This pilot study needs replication in a larger randomized double-blinded control study.

PathogenicWe recruited 13 adults (between 18 years and 45 years of age) withWhile most patients had daily seizures at seizure onset, seizure frequency declined or remitted during childhood and adulthood. The most common seizure type was tonic seizures (early) infancy, and tonic-clonic and focal impaired awareness seizures later in life. Ten individuals (77%) were seizure-free at last follow-up. In 38% of the individuals, earlier periods of seizure freedom lasting a minimum of 2 years followed by seizure recurrence had occurred. Of the 10 seizure-free patients, 4 were receiving a single antiseizure medication (ASM, carbamazepine, lamotrigine or levetiracetam), and 2 had stopped taking ASM. Intellectual disability (ID) ranged from mild to profound, with the majority (54%) of individuals in the severe category. At last contact, six individuals (46%) remained unable to walk independently, six (46%) had limb spasticity and four (31%) tetraparesis/tetraplegia. Six (46%) remained non-verbal, 10 (77%) had autistic features/autism, 4 (31%) exhibited aggressive behaviour and 4 (31%) destructive behaviour with self-injury. Four patients had visual problems, thought to be related to prematurity in one. Sleep problems were seen in six (46%) individuals.Seizure frequency declines over the years and most patients are seizure-free in adulthood. Longer seizure-free periods followed by seizure recurrence are common during childhood and adolescence. Most adult patients have severe ID. Motor, language and behavioural problems are an issue of continuous concern.

International audience; Collecting 130 electronic medical records and diagnoses from emergency room stays of eleven patients with confirmed psychogenic nonepileptic seizure (PNES) over a 17-year period (2001-2018), 48 different diagnostic terms were retrieved. This emphasized the need for a consensual terminology encompassing not only PNES but also all functional transient (paroxysmal) events, including episodes of motor or sensory deficits, and cognitive symptoms. Rather than defining what it is not (PNES, stroke mimicks…), it would be more accurate to define what it is: a paroxysmal functional event.

International audience; We present a novel method for automatic sleep scoring based on single-channel EEG. We introduce the use of a deep convolutional neural network (CNN) on raw EEG samples for supervised learning of 5 class sleep stage prediction. The network has 14 layers, takes as input the 30-s epoch to be classified as well as two preceding epochs and one following epoch for temporal context, and requires no signal preprocessing or feature extraction phase. We train and evaluate our system using data from the Sleep Heart Health Study (SHHS), a large multi-center cohort study including expert-rated polysomnographic records. Performance metrics reach the state of the art, with accuracy of 0.87 and Cohen kappa of 0.81. The use of a large cohort with multiple expert raters guarantees good generalization. Finally, we present a method for visualizing class-wise patterns learned by the network.

Ρesume. La prevalence de la comorbidite psychiatrique au sein de la population souffrant d'une epilepsie pharmacoresistante est importante. Bien que l'impact de ces troubles psychopathologiques sur la qualite de vie ait ete souligne, peu de donnees sont disponibles concernant les modalites de leur prise en charge. Ce travail presente une revue des donnees de la litterature, englobant les travaux originaux, et les opinions d'expert, tout en soulignant l'interet de promouvoir des etudes portant specifiquement sur cette population de patients. L'accent est mis sur la nature et le role de la depression au cours de l'epilepsie partielle pharmacoresistante, et le debat autour de l'existence d'une psychopathologie specifique au sein de cette population.

Objective. The optimal duration of routine EEG (rEEG) has not been determined on a clinical basis. This study aims to determine the time required to obtain relevant information during rEEG with respect to the clinical request. Method. All rEEGs performed over 3 months in unselected patients older than 14 years in an academic hospital were analyzed retrospectively. The latency required to obtain relevant information was determined for each rEEG by 2 independent readers blinded to the clinical data. EEG final diagnoses and latencies were analyzed with respect to the main clinical requests: subacute cognitive impairment, spells, transient focal neurologic manifestation or patients referred by epileptologists. Results. From 430 rEEGs performed in the targeted period, 364 were analyzed: 92% of the pathological rEEGs were provided within the first 10 minutes of recording. Slowing background activity was diagnosed from the beginning, whereas interictal epileptiform discharges were recorded over time. Moreover, the time elapsed to demonstrate a pattern differed significantly in the clinical groups: in patients with subacute cognitive impairment, EEG abnormalities appeared within the first 10 minutes, whereas in the other groups, data could be provided over time. Conclusion. Patients with subacute cognitive impairment differed from those in the other groups significantly in the elapsed time required to obtain relevant information during rEEG, suggesting that 10-minute EEG recordings could be sufficient, arguing in favor of individualized rEEG. However, this conclusion does not apply to intensive care unit patients.

Resume L’electroencephalogramme (EEG) de sommeil est determinant dans l’exploration de certaines epilepsies en pediatrie, en raison de la mauvaise qualite du trace de l’EEG de veille ou de l’effet specifique du sommeil sur les anomalies de l’EEG. Cependant, l’endormissement et le maintien de l’etat endormi lors de la sieste peuvent etre difficiles a obtenir malgre la mise en place de recommandations de principe ; il est possible alors de recourir a une prise medicamenteuse. Comme les benzodiazepines, l’hydroxyzine ou l’hydrate de chloral peuvent influencer la macrostructure du sommeil et donc affecter l’interpretation de l’examen ou necessiter une surveillance particuliere, certaines unites de neurophysiologie clinique recourent a la melatonine en liberation immediate. Cette neurohormone physiologique permet d’induire le sommeil et a ete experimentee avec succes en premedication de differentes explorations complementaires necessitant une immobilite prolongee. Cet article passe en revue les arguments, tires de la litterature et bases sur notre experience, demontrant que les donnees d’efficacite et de tolerance de l’utilisation de la melatonine dans l’obtention du sommeil au cours de l’EEG de l’enfant semblent confirmer l’interet de cette utilisation. Toutefois, aucune etude randomisee versus placebo n’est actuellement disponible.

Myoclonus is a sudden brief (20–250 ms) contraction (positive myoclonus), or a brief and sudden cessation of tonic muscle (negative myoclonus) inducing a simple jerky movement of body part. Myoclonus could have different origins in almost every part of the nervous system, from the cortex to the peripheral nerve, sharing a large panel of etiologies. It is regarded as the paradigmatic movement disorder causing jerks, although not the sole. This paper aims to depict the clinical and neurophysiological characteristics of myoclonus. It shows how neurophysiological investigations including surface polymyography and methods exploring cortical excitability, namely conventional EEG, EEG – jerk-locked back-averaging, somatosensory evoked potentials and C-reflex studies are required to define the generator of myoclonus in the central nervous system and clearly classify myoclonus as cortical, corticothalamic, subcortical – resulting from lesions or dysfunctions of basal ganglia/reticular system – or spinal. This paper also enlightens other movement disorders that may mimic myoclonus appearances, including psychogenic jerks, simple motor tics, spasms and startle syndromes. Finally, it raises few unresolved questions regarding the propriospinal myoclonus or peripheral myoclonus entities, the role of the cerebellum in myoclonic diseases and the relationship between cortical and epileptic myoclonus.

Background Pitolisant (BF2.649) is a nonimidazole histamine 3 receptor antagonist. In previous animal studies, it has been shown that pitolisant might be helpful in chronic seizure treatment of both partial and generalized epilepsies. The present study is a multicenter, national, pragmatic, noncomparative, open-label, exploratory phase II trial. It is the first reported study of the clinical effects of pitolisant in human epilepsy. Objective The goal of this trial was to explore the antiepileptic effect of 3 different doses of pitolisant (20, 30, and 40 mg once daily) in patients presenting partial seizure onset despite therapy with adequate dose of 1 to 3 appropriate antiepileptic drugs. Methods The study has been conducted in 6 study sites in France between 2005 and 2006. The primary end point was the proportion of responders having a seizure rate decrease by at least 50%. A larger clinical trial could be started according to the results. An interim analysis was planned in the protocol to decide if the study should be continued or not according to the efficacy and safety results. Descriptive statistics were used for the analysis. Results An initial goal of 40 patients included had been planned; 23 were finally included. Pitolisant was well tolerated and achieved a clinical response in one third of patients after 3 months of treatment. Conclusions Despite encouraging data, there is no evidence for the efficacy of the drug for the regimen that was used, but no firm conclusions can be drawn because the number of included subject was small and the study was not placebo controlled.

L’EEG standard de 20 minutes fait partie integrante de la prise en charge des patients suspects d’epilepsie ou ayant ete diagnostique mais sa rentabilite varie en fonction des situations cliniques. En revanche, le recours aux EEG de longue duree permet d’augmenter la sensibilite de l’examen, au prix de contraintes plus importantes. Ainsi, plusieurs modalites sont disponibles avec avantages et inconvenients : EEG de sieste, holter-EEG sans video, monitoring-video au lit du malade, courte hospitalisation en unite de VEEG. Toutefois, augmenter la sensibilite (detecter des anomalies EEG significatives) peut se faire au detriment de la specificite (detecter des anomalies sans rapport avec la situation clinique), comme nous allons l’illustrer dans cet atelier, en particulier dans certaines populations cibles. Adapter les indications aux questions cliniques, savoir quand se contenter d’un resultat EEG standard normal et quand il faut, au contraire, insister pour recueillir les anomalies a l’aide d’examens prolonges, seront les principes qui guideront les animateurs au cours de cet atelier, destine a tout neurologue exercant ou en formation, realisant ou indiquant des EEG. Les supports utilises dans l’atelier seront constitues de cas illustratifs choisis dans les bases des services des animateurs.

Postictal syndrome (PIS) encompasses the clinical, biological, electroencephalographic (EEG) and magnetic resonance imaging (MRI) signs that follow the termination of a seizure. These signs occur as soon as the epileptic discharge ends, but might remain for a substantially long period of time, making them amenable to clinical observation. As a direct consequence, neurologists and intensivists are more frequently attending patients with PIS than during their seizure. Moreover, careful PIS documentation may help physicians to diagnose epileptic seizure from other non-epileptic disorders. Careful analysis of PIS could also be helpful to better characterize the seizure (seizure subtypes, and to some extent, the localization and/or lateralization of the seizure). This article aims to review the main clinical, biological, EEG and MRI components of PIS, discuss differential diagnoses and propose a general clinical attitude, based on the acronym "WAITTT": W for "Watch", to monitor and investigate PIS in order to provide relevant information on seizure, AIT for "Avoid Inappropriate Treatment", to underscore the risk carrying out unnecessary drug injections and intensive care procedures in the setting of a self-limited symptomatology, and TT for "Take Time", to keep in mind that time remains the clinician's best ally for treating patients with PIS.

International audience; This study aims at examining the precise temporal dynamics of the emotional facial decoding as it unfolds in the brain, according to the emotions displayed. To characterize this processing as it occurs in ecological settings, we focused on unconstrained visual explorations of natural emotional faces (i.e., free eye movements). The General Linear Model (GLM; Smith and Kutas, 2015a,b; Kristensen et al., 2017a) enables such a depiction. It allows deconvolving adjacent overlapping responses of the eye fixation-related potentials (EFRPs) elicited by the subsequent fixations and the event-related potentials (ERPs) elicited at the stimuli onset. Nineteen participants were displayed with spontaneous static facial expressions of emotions (Neutral, Disgust, Surprise, and Happiness) from the DynEmo database (Tcherkassof et al., 2013). Behavioral results on participants’ eye movements show that the usual diagnostic features in emotional decoding (eyes for negative facial displays and mouth for positive ones) are consistent with the literature. The impact of emotional category on both the ERPs and the EFRPs elicited by the free exploration of the emotional faces is observed upon the temporal dynamics of the emotional facial expression processing. Regarding the ERP at stimulus onset, there is a significant emotion-dependent modulation of the P2–P3 complex and LPP components’ amplitude at the left frontal site for the ERPs computed by averaging. Yet, the GLM reveals the impact of subsequent fixations on the ERPs time-locked on stimulus onset. Results are also in line with the valence hypothesis. The observed differences between the two estimation methods (Average vs. GLM) suggest the predominance of the right hemisphere at the stimulus onset and the implication of the left hemisphere in the processing of the information encoded by subsequent fixations. Concerning the first EFRP, the Lambda response and the P2 component are modulated by the emotion of surprise compared to the neutral emotion, suggesting an impact of high-level factors, in parieto-occipital sites. Moreover, no difference is observed on the second and subsequent EFRP. Taken together, the results stress the significant gain obtained in analyzing the EFRPs using the GLM method and pave the way toward efficient ecological emotional dynamic stimuli analyses.

International audience; Predicting the outcome of a comatose or poorly responsive patient is a major issue for intensive care unit teams, in order to give the most accurate information to the family and to choose the best therapeutic option. However, determining the level of cortical activity in patients with disorders of consciousness is a real challenge. Reliable criteria are required to help clinicians in the decision-making process, especially in the acute phase of coma. In this paper, we propose recommendations for recording and interpreting electroencephalography and evoked potentials in comatose patients based on the literature and the clinical experience of a group of neurophysiologists trained in the management of comatose patients. We propose methodological guidelines and discuss prognostic value of each test as well as the limitations concerning recording and interpretation. Recommendations for the strategy and timing of neurophysiological assessments are also proposed according to various clinical situations.

Electroencephalography allows the functional analysis of electrical brain cortical activity and is the gold standard for analyzing electrophysiological processes involved in epilepsy but also in several other dysfunctions of the central nervous system. Morphological imaging yields complementary data, yet it cannot replace the essential functional analysis tool that is EEG. Furthermore, EEG has the great advantage of being non-invasive, easy to perform and allows control tests when follow-up is necessary, even at the patient's bedside. Faced with the advances in knowledge, techniques and indications, the Societe de Neurophysiologie Clinique de Langue Francaise (SNCLF) and the Ligue Francaise Contre l'Epilepsie (LFCE) found it necessary to provide an update on EEG recommendations. This article will review the methodology applied to this work, refine the various topics detailed in the following chapters. It will go over the summary of recommendations for each of these chapters and underline proposals for writing an EEG report. Some questions could not be answered by the review of the literature; in those cases, an expert advice was given by the working and reading groups in addition to the guidelines

Objectives In the context of the first clinical trial of vagus nerve stimulation (VNS) in Crohn’s disease (CD), our main objective was to quantify the acute and chronic effects of VNS on brain activity in CD patients. Methods We measured the electroencephalogram (EEG) in 9CD patients under VNS at 10 Hz just before VNS initiation, after 6 weeks and after 12 months of chronic VNS. Results Acute VNS induced increased spectral power in delta and theta bands on frontal, temporal and occipital electrodes. The main significant modulation was the 12 months’ chronic effect of VNS which consisted mainly in a decreased power in the alpha frequency band which was correlated with the normalization of bowel mucosal inflammation, anxiety state and vagal tone. Conclusions In addition to the activation of vagal efferent fibers that regulate the autonomic nervous system, our data suggest that chronic VNS has a regulatory action via afferent vagal fibers on anxio-depressive symptomatology associated to CD, which could be directly highlighted by the modulation of EEG alpha power known to be associated to depressed states. Significance This is the first report of the central effects of VNS in CD patients.

Pallidal deep brain stimulation (globus pallidus internus (GPi) DBS) is the best therapeutic option for disabling isolated idiopathic (IID) and inherited (INH) dystonia. Acquired dystonia (AD) may also benefit from GPi DBS. Efficacy and safety in the long-term remained to be established.To retrospectively assess long-term clinical outcomes and safety in dystonic patients who underwent GPi DBS.Patients were videotaped and assessed preoperatively and postoperatively (1-year and at last available follow-up) using the Burke-Fahn-Marsden Dystonia Rating Scale (motor score (BFMDRS-M); disability score (BFMDRS-D)).Sixty-one patients were included (follow-up 7.9±5.9 years; range 1-20.7). In IID and INH (n=37), the BFMDRS-M improved at first (20.4±24.5; p0.00001) and last (22.2±18.2; p0.001) follow-ups compared with preoperatively (50.5±28.0). In AD (n=19), the BFMDRS-M ameliorated at 1-year (40.8±26.5; p0.02) and late follow-ups (44.3±24.3; p0.04) compared with preoperatively (52.8±24.2). In INH dystonia with other neurological features (n=4) there was no motor benefit. In IID and INH, the BFMDRS-D improved at 1-year (9.5±7.5; p0.0002) and late follow-ups (10.4±7.8; p0.016) compared with preoperatively (13.3±6.9). In AD, the BFMDRS-D reduced at 1-year (12.0±8.1; p0.01) and late follow-ups (12.7 ±6.1; p=0.2) compared with preoperatively (14.35±5.7). Most adverse events were hardware related.GPi DBS is an effective and safe treatment in most patients with dystonia.

Simple febrile seizures last for less than 10 minutes and resolve spontaneously, in the context of a febrile illness, without focal features or recurrence during the subsequent 24 hours. We report the case of fortuitous video-EEG recording of a FS, clinically classified as “simple”, which demonstrated a focal, temporal onset. This clinical finding is in agreement with animal model studies demonstrating focal onset. [Published with video sequences]

Resume La levitation du membre superieur est une elevation involontaire du membre superieur progressivement amene dans une position anti-gravifique. Elle a ete decrite dans un grand nombre d’affections degeneratives, dont la paralysie supranucleaire progressive, la maladie de Creutzfeldt-Jakob, l’atrophie cortico-basale et la maladie de Huntington, mais egalement dans le contexte d’accidents vasculaires cerebraux de topographie variable. L’analyse des cas publies dans la litterature montre une grande heterogeneite clinique qui suggere que le terme est aujourd’hui utilise pour recouvrir des phenomenes cliniques dont la physiopathologie peut etre differente. En se basant sur une serie de cas personnels, nous proposons de distinguer quatre types de levitation du membre superieur, renvoyant a des physiopathologies et des cadres etiologiques differents. Nous distinguerons la levitation dystonique, la levitation ataxique, la levitation capricieuse et la levitation syncinetique, a partir de considerations cliniques, physiopathologiques et etiologiques.