Search

Face processing relies on predictive processes driven by low spatial frequencies (LSF) that convey coarse information prior to fine information conveyed by high spatial frequencies. However, autistic individuals might have atypical predictive processes, contributing to facial processing difficulties. This may be more normalized in autistic females, who often exhibit better socio-communicational abilities than males. We hypothesized that autistic females would display a more typical coarse-to-fine processing for socio-emotional stimuli compared to autistic males. To test this hypothesis, we asked adult participants (44 autistic, 51 non-autistic) to detect fearful faces among neutral faces, filtered in two orders: from coarse-to-fine (CtF) and from fine-to-coarse (FtC). Results show lower d' values and longer reaction times for fearful detection in autism compared to non-autistic (NA) individuals, regardless of the filtering order. Both groups presented shorter P100 latency after CtF compared to FtC, and larger amplitude for N170 after FtC compared to CtF. However, autistic participants presented a reduced difference in source activity between CtF and FtC in the fusiform. There was also a more spatially spread activation pattern in autistic females compared to NA females. Finally, females had faster P100 and N170 latencies, as well as larger occipital activation for FtC sequences than males, irrespective of the group. Overall, the results do not suggest impaired predictive processes from LSF in autism despite behavioral differences in fear detection. However, they do indicate reduced brain modulation by spatial frequency in autism. In addition, the findings highlight sex differences that warrant consideration in understanding autistic females., (© 2024 The Author(s). Autism Research published by International Society for Autism Research and Wiley Periodicals LLC.)

We report on the case of an adolescent with juvenile myoclonic epilepsy exhibiting compulsory sporadic voluntary movement. These movements entailed the deliberate act of touching her forehead with her hand and were triggered by a short and indefinable cephalic sensation. Upon inquiry regarding the nature of this movement, the patient reported a sudden perception of a peculiar event localized "inside her head". In the course of a prolonged video-electroencephalographic recording, it appeared that the movements consistently followed the occurrence of diffuse Generalized Spike-and-Wave Bursts (GSWBs), lasting one to three seconds. This observation suggests that the impact of the GSWBs on the patient's stream of consciousness could be detected by the patient herself, who attributed it to an internal sensation, like a "blip on a screen". This clinical observation echoes the "blip syndrome", as described by the neurologist James W. Lance thirty years ago. These findings give some support that "blips" could be of an epileptic origin and lend weight to the notion that some patients could perceive their own EEG discharges. Moreover, this observation should be added to the perennial debate on the cognitive impact of intercritical discharges., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Author(s).)

Recent research suggests that autistic females may have superior socio-cognitive abilities compared to autistic males, potentially contributing to underdiagnosis in females. However, it remains unclear whether these differences arise from distinct neurophysiological functioning in autistic males and females. This study addresses this question by presenting 41 autistic and 48 non-autistic adults with a spatially filtered faces oddball paradigm. Analysis of event-related potentials from scalp electroencephalography reveal a neurophysiological profile in autistic females that fell between those of autistic males and non-autistic females, highlighting sex differences in autism from the initial stages of face processing. This finding underscores the urgent need to explore neurophysiological sex differences in autism and encourages efforts toward a better comprehension of compensation mechanism and a clearer definition of what is meant by camouflaging., (© 2024. The Author(s).)

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., Competing Interests: Conflict of interest The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as potential conflicts of interest., (Copyright © 2022. Published by Elsevier B.V.)

Different types of resistance to passive movement, i.e. hypertonia, were described in schizophrenia spectrum disorders (SSD) long before the introduction of antipsychotics. While these have been rediscovered in antipsychotic-naïve patients and their non-affected relatives, the existence of intrinsic hypertonia vs drug-induced parkinsonism (DIP) in treated SSD remains controversial. This integrative review seeks to develop a commonly accepted framework to specify the putative clinical phenomena, highlight conflicting issues and discuss ways to challenge each hypothesis and model through adversarial collaboration. The authors agreed on a common framework inspired from systems neuroscience. Specification of DIP, locomotor paratonia (LMP) and psychomotor paratonia (PMP) identified points of disagreement. Some viewed parkinsonian rigidity to be sufficient for diagnosing DIP, while others viewed DIP as a syndrome that should include bradykinesia. Sensitivity of DIP to anticholinergic drugs and the nature of LPM and PMP were the most debated issues. It was agreed that treated SSD should be investigated first. Clinical features of the phenomena at issue could be confirmed by torque, EMG and joint angle measures that could help in challenging the selectivity of DIP to anticholinergics. LMP was modeled as the release of the reticular formation from the control of the supplementary motor area (SMA), which could be challenged by the tonic vibration reflex or acoustic startle. PMP was modeled as the release of primary motor cortex from the control of the SMA and may be informed by subclinical echopraxia. If these challenges are not met, this would put new constraints on the models and have clinical and therapeutic implications., Competing Interests: Declaration of competing interest Authors have no conflict of interest to declare related to the current manuscript., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: The study aimed to determine the level of agreement between patients with epilepsy and their proxies when assessing psychiatric comorbidities, sleep disorders, and medication adherence using standardized questionnaires., Methods: This agreement study is an ancillary analysis of the PRERIES study, a matched case-control study exploring SUDEP risk factors. Controls aged 15 years and older, with active epilepsy or in remission for less than 5 years were recruited between 01/01/2011 and 03/31/2019. An interview was carried out by a trained psychologist on both the patient and a proxy-respondent. During these independent interviews, the following comorbidities were explored: psychiatric comorbidities using the MINI, the STAI- Y 2 and NDDI-E scales, sleep disorders with the SDQ-SA and Epworth scales and medication adherence. Level of agreement between patient and their proxy was estimated using Gwet's AC1&2., Results: Among the 107 patient-proxy dyads recruited, proxy respondents were mainly family members (65.4%) or spouses (30.8%). Exploration of present major depression showed excellent agreement at 0.81 [0.65;0.97], as well as exploration of dysthymia at 0.96 [0.61;1]. Suicidal risk evaluation had a lesser agreement at 0.77 [0.60;0.94]. Agreement on anxiety was moderate 0.5 [0.38;0.62]. For sleep disorder, SDQ-SA presented a better agreement than the Epworth questionnaire with respectively 0.73 [0.51;0.95] and 0.45 [0.26;0.63]. For medication adherence, the overall agreement rate was excellent (0.90 [0.78;1])., Conclusion: Exploration of potential risk factors through families can give valuable and relatively robust information, especially if the respondent lives with the patient, and should be retrieved, when possible, in usual clinical setting., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

The aim of the present study was to compare the analgesic effect of motor cortex stimulation using high-frequency repetitive transcranial magnetic stimulation or transcranial direct current stimulation and transcutaneous spinal direct current stimulation in patients with complex regional pain syndrome. Thirty-three patients with complex regional pain syndrome were randomized to one of the three treatment groups (repetitive transcranial magnetic stimulation, n = 11; transcranial direct current stimulation, n = 10; transcutaneous spinal direct current stimulation, n = 12) and received a series of 12 sessions of stimulation for 3 weeks (induction phase) and 11 sessions for 4 months (maintenance therapy). The primary end-point was the mean pain intensity assessed weekly with a visual numerical scale during the month prior to treatment (baseline), the 5-month stimulation period and 1 month after the treatment. The weekly visual numerical scale pain score was significantly reduced at all time points compared to baseline in the transcutaneous spinal direct current stimulation group, at the last two time points in the repetitive transcranial magnetic stimulation group (end of the 5-month stimulation period and 1 month later), but at no time point in the transcranial direct current stimulation group. A significant pain relief was observed at the end of induction phase using transcutaneous spinal direct current stimulation compared to repetitive transcranial magnetic stimulation ( P = 0.008) and to transcranial direct current stimulation ( P = 0.003). In this trial, transcutaneous spinal direct current stimulation was more efficient to relieve pain in patients with complex regional pain syndrome compared to motor cortex stimulation techniques (repetitive transcranial magnetic stimulation, transcranial direct current stimulation). This efficacy was found during the induction phase and was maintained thereafter. This study warrants further investigation to confirm the potentiality of transcutaneous spinal direct current stimulation as a therapeutic option in complex regional pain syndrome., Competing Interests: The authors report no competing interests., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Guarantors of Brain.)

Background: 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., Methods: 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., Results: 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., Conclusion: 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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

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. <1/year: adjusted odds ratio [AOR] 2.6, 95% confidence interval [CI] 1.25-5.41), nocturnal or sleep-related seizures (AOR 4.49, 95% CI 2.68-7.53), current or past depression (AOR 2.0, 95% CI 1.19-3.34) or the ability to alert someone of an oncoming seizure (AOR 0.57, 95% CI 0.33-0.98). After internal validation, a clinically usable score ranging from -1 to 8 was developed, with high discrimination capabilities (area under the receiver operating curve 0.85, 95% CI 0.80-0.90). The threshold of 3 has good sensitivity (82.3%, 95% CI 72.7-91.8), whilst keeping a good specificity (82.7%, 95% CI 79.8-85.7)., Conclusions: These results outline the importance of generalized and nocturnal seizures on the occurrence of SUDEP, and show a protective role in the ability to alert someone of an oncoming seizure. The SUDEP-CARE score is promising and will need external validation. Further work, including paraclinical explorations, could improve this risk score., (© 2022 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Migralepsy is a nosographical entity depicting a clinical event whose occurrence seems rather exceptional in view of the comorbidity observed between epilepsy and migraine. Defined more precisely as a migraine aura-triggered epileptic seizure (within the time limit of one hour), it is susceptible to numerous diagnoses by excess, undoubtedly stimulated by the elegance of the term diagnosis coined by Lennox and Lennox in 1960. This review points to the main criticisms, which were given to it, but also to the international recognition brought by the International Classification of Headache Disorders (ICHD 3). In fact, there are undoubtedly clinical cases falling under the strict definition of migralepsy, cases which are rare but relevant for understanding the pathophysiology of the two colliding events: migraine aura and epileptic seizure involving the occipital lobe., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Background: Pathogenic KCNQ2 variants are a frequent cause of developmental and epileptic encephalopathy., Methods: We recruited 13 adults (between 18 years and 45 years of age) with KCNQ2 encephalopathy and reviewed their clinical, EEG, neuroimaging and treatment history., Results: While 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., Conclusion: 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., Competing Interests: Competing interests: EC is a consultant to Xenon Pharmaceuticals and Knopp Biosciences; his participation in this work has been reviewed and approved by Baylor College of Medicine in accordance with institutional conflict of interest policies. MKK serves on speakers bureaus for Greenwich, Novartis and Lundbeck, and on an advisory board for Stealth Biotherapeutics. PS received fees from Ultragenyx, Zogenyx, Biomarin, PTC pharmaceuticals, GW pharma, Neuraxpharma and research grants from GW pharma, PTC Pharmaceuticals, ENECTA SV, Kolfarma, and has been investigator for clinical trials for Ultragenyx and Zogenix. SW received speaker and consultancy fees from UCB, Xenon, Zogenix, Lundbeck and Biocodex., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Visual processing is thought to function in a coarse-to-fine manner. Low spatial frequencies (LSF), conveying coarse information, would be processed early to generate predictions. These LSF-based predictions would facilitate the further integration of high spatial frequencies (HSF), conveying fine details. The predictive role of LSF might be crucial in automatic face processing, where high performance could be explained by an accurate selection of clues in early processing. In the present study, we used a visual Mismatch Negativity (vMMN) paradigm by presenting an unfiltered face as standard stimulus, and the same face filtered in LSF or HSF as deviant, to investigate the predictive role of LSF vs. HSF during automatic face processing. If LSF are critical for predictions, we hypothesize that LSF deviants would elicit less prediction error (i.e., reduced mismatch responses) than HSF deviants. Results show that both LSF and HSF deviants elicited a mismatch response compared with their equivalent in an equiprobable sequence. However, in line with our hypothesis, LSF deviants evoke significantly reduced mismatch responses compared to HSF deviants, particularly at later stages. The difference in mismatch between HSF and LSF conditions involves posterior areas and right fusiform gyrus. Overall, our findings suggest a predictive role of LSF during automatic face processing and a critical involvement of HSF in the fusiform during the conscious detection of changes in faces., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Lacroix, Harquel, Mermillod, Vercueil, Alleysson, Dutheil, Kovarski and Gomot.)

Objective: 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., Methods: 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., Results: 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)., Conclusion: 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., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Competing Interests: Competing interests: None declared.

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., (© 2020 John Wiley & Sons Ltd.)

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., Competing Interests: Declaration of competing interest None declared under financial, general, and institutional competing interests., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: The aim of this study was to evaluate neurologists' reliability in recognizing retrospectively a diagnosis of psychogenic status and status epilepticus (SE) based solely on clinical semiology, as reported in medical charts., Methods: This is a retrospective analysis of medical records of patients with suspected SE, diagnosed with psychogenic status and SE, proven by video-electroencephalography (EEG) monitoring, over a two-year period, from January 1st 2012 to December 31st 2013. Eight additional patients outside this time frame were included in this series because they had video-EEG proven psychogenic status, and they met all the inclusion criteria. The group with SE was divided into symptomatic SE (SSE) if a precipitating factor was identified, and undetermined SE (USE) if none were identified. Twenty-two neurologists from the CHU de Grenoble-Alpes were asked to fill out a survey where they were asked to score, for each patient, their agreement, using Likert scales, for the respective diagnoses of psychogenic status and SE. Their opinions were based on a provided written sheet summarizing the clinical description of the event and patients' clinical context. Neurologists were blinded to video-EEG monitoring results and final diagnosis. The level of agreement, disagreement, and the homogeneity of neurologist's responses according to the final diagnosis were then calculated. Finally, clinical data, as provided in the event's clinical description and context, considered as highly relevant by neurologists to establish an accurate diagnosis were gathered., Results: Eighteen neurologists completed the survey for 48 patients, including 11 diagnosed with psychogenic status and 37 with SE (30 with SSE and 7 with USE). For patients diagnosed with SE, the presence of a precipitating factor increased the likelihood and the homogeneity among neurologists of a diagnosis of SE (77%), with a specificity (Sp) of 96% and a positive predictive value of 95%. The lack of a precipitating factor significantly decreased the diagnosis likelihood of SE (55%) with a predictive value of 82%. For patients diagnosed with psychogenic status, most of neurologists agreed with the diagnosis of psychogenic status (69%) with a predictive value of 82%, although heterogeneity in the diagnosis was found. According to neurologists participating in this study, most significant terms, found in the medical charts, helping to distinguish SE from psychogenic status were "stereotypical movements", "limb myoclonus", "epilepsy", and "vigilance alteration". To differentiate psychogenic status from SE, most relevant terms used by neurologists were "resistance to eyes opening", "anarchic movements", "prolonged motor manifestations", "limb tremor" and "opisthotonus". However, analysis of the distribution of the terms among the different groups (SSE, USE, and psychogenic status) showed no significant difference., Significance: This study is in line with previous literature highlighting the difficulty in retrospectively differentiating SE from psychogenic status based on clinical events description recorded in the medical chart., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Peripherally induced movement disorders (PIMDs) represent a rare and debated complication of peripheral trauma., Phenomenology Shown: We report a case of task-specific "lipstick" jerky dystonic tremor as a consequence of traumatic shoulder injury, successfully treated with EMG-guided botulinum toxin injections., Educational Value: This case expands the phenotypic spectrum of PIMDs, with a visual example of a task-specific dystonic tremor after peripheral trauma, and the efficacy of EMG-guided botulinum toxin treatment in the setting of posttraumatic dystonic tremor., Competing Interests: Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial, or not-for-profit sectors. Conflicts of Interest: The authors report no conflicts of interest. Ethics Statement: This study was performed in accordance with the ethical standards detailed in the Declaration of Helsinki. All patients that appear on video have provided written informed consent; authorization for the videotaping and for publication of the videotape was provided., (© 2019 Cavallieri et al.)

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., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

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.

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.

We investigated how two different reading tasks, namely reading to memorize [Read & Memorize (RM)] and reading to decide whether a text was relevant to a given topic [Read & Decide (RD)], modulated both eye movements (EM) and brain activity. To this end, we set up an ecological paradigm using the eye fixation-related potentials (EFRP) technique, in which participants freely moved their eyes to process short paragraphs, while their electroencephalography (EEG) activity was recorded in synchronization with their EM. A general linear model was used to estimate at best EFRP, taking account of the overlap between adjacent potentials, and more precisely with the potential elicited at text onset, as well as saccadic potentials. Our results showed that EM patterns were top-down modulated by different task demands. More interestingly, in both tasks, we observed slow-wave potentials that gradually increased across the first eye fixations. These slow waves were larger in the RD task than in the RM task, specifically over the left hemisphere. These results suggest that the decision-making process during reading in the RD task engendered a greater memory load in working memory than that generated in a classic reading task. The significance of these findings is discussed in the light of recent theories and models of working memory processing.

Childhood absence epilepsy (CAE) is one of the most frequent epilepsies in infancy. The first-line recommended therapy for CAE is based on the prescription of the narrow-spectrum ethosuximide and the broad-spectrum valproic acid, which have similar efficacy in the first 12 months. Nevertheless, some antiepileptic drugs (AEDs) may worsen seizure duration and type in this syndrome. In line with this, we have encountered a case of identical twins with CAE and early exposure to different antiseizure drugs leading to divergent outcomes. From this, we hypothesized that the first AED to treat CAE may determine the long-term prognosis, especially in the developing brain, and that some situations leading to drug resistance may be explained by use of an inappropriate first AED. Therefore, we investigated this hypothesis by using a genetic mouse model of absence epilepsy (BS/Orl). Mice received a first appropriate or inappropriate AED followed by the same appropriate AED. Our data demonstrate that an inappropriate first AED has a negative impact on the long-term efficacy of a second appropriate AED. This work supports the necessity to effectively diagnose epileptic syndromes prior to medication use, particularly in children, in order to prevent the deleterious effects of an inappropriate initial AED., (Wiley Periodicals, Inc. © 2018 International League Against Epilepsy.)

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., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Importance: Ataxia with oculomotor apraxia type 1 (AOA1) is an autosomal recessive cerebellar ataxia due to mutations in the aprataxin gene (APTX) that is characterized by early-onset cerebellar ataxia, oculomotor apraxia, axonal motor neuropathy, and eventual decrease of albumin serum levels., Objectives: To improve the clinical, biomarker, and molecular delineation of AOA1 and provide genotype-phenotype correlations., Design, Setting, and Participants: This retrospective analysis included the clinical, biological (especially regarding biomarkers of the disease), electrophysiologic, imaging, and molecular data of all patients consecutively diagnosed with AOA1 in a single genetics laboratory from January 1, 2002, through December 31, 2014. Data were analyzed from January 1, 2015, through January 31, 2016., Main Outcomes and Measures: The clinical, biological, and molecular spectrum of AOA1 and genotype-phenotype correlations., Results: The diagnosis of AOA1 was confirmed in 80 patients (46 men [58%] and 34 women [42%]; mean [SD] age at onset, 7.7 [7.4] years) from 51 families, including 57 new (with 8 new mutations) and 23 previously described patients. Elevated levels of α-fetoprotein (AFP) were found in 33 patients (41%); hypoalbuminemia, in 50 (63%). Median AFP level was higher in patients with AOA1 (6.0 ng/mL; range, 1.1-17.0 ng/mL) than in patients without ataxia (3.4 ng/mL; range, 0.8-17.2 ng/mL; P < .01). Decreased albumin levels (ρ = -0.532) and elevated AFP levels (ρ = 0.637) were correlated with disease duration. The p.Trp279* mutation, initially reported as restricted to the Portuguese founder haplotype, was discovered in 53 patients with AOA1 (66%) with broad white racial origins. Oculomotor apraxia was found in 49 patients (61%); polyneuropathy, in 74 (93%); and cerebellar atrophy, in 78 (98%). Oculomotor apraxia correlated with the severity of ataxia and mutation type, being more frequent with deletion or truncating mutations (83%) than with presence of at least 1 missense variant (17%; P < .01). Mean (SD) age at onset was higher for patients with at least 1 missense mutation (17.7 [11.4] vs 5.2 [2.6] years; P < .001)., Conclusions and Relevance: The AFP level, slightly elevated in a substantial fraction of patients, may constitute a new biomarker for AOA1. Oculomotor apraxia may be an optional finding in AOA1 and correlates with more severe disease. The p.Trp279* mutation is the most frequent APTX mutation in the white population. APTX missense mutations may be associated with a milder phenotype.

Background: 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., Objective: To retrospectively assess long-term clinical outcomes and safety in dystonic patients who underwent GPi DBS., Methods: 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))., Results: 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; p<0.00001) and last (22.2±18.2; p<0.001) follow-ups compared with preoperatively (50.5±28.0). In AD (n=19), the BFMDRS-M ameliorated at 1-year (40.8±26.5; p<0.02) and late follow-ups (44.3±24.3; p<0.04) 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; p<0.0002) and late follow-ups (10.4±7.8; p<0.016) compared with preoperatively (13.3±6.9). In AD, the BFMDRS-D reduced at 1-year (12.0±8.1; p<0.01) and late follow-ups (12.7 ±6.1; p=0.2) compared with preoperatively (14.35±5.7). Most adverse events were hardware related., Conclusions: GPi DBS is an effective and safe treatment in most patients with dystonia., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

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., (Copyright © 2016. Published by Elsevier Masson SAS.)