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A 4-year-old boy presented with acute febrile illness with altered sensorium, decreased responsiveness to command, excessive sleepiness, headache and generalised-onset motor seizures for the past 2 days with intact bladder and bowel control. There was no history of jaundice, change in the colour of

It has been known for several decades that epilepsy and autism spectrum disorders (ASD) are related to each other. Epilepsy frequently accompanies ASD. The purpose of this study was to investigate relationship between clinical and electroencephalogram (EEG) findings in ASD patients and to identify EEG characteristics that may create a disposition to epilepsy in ASD by examining differences in clinical and EEG findings between patients diagnosed with ASD without epilepsy and ASD with epilepsy. A total of 102 patients aged 2 to 18 years and diagnosed with ASD based on Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) diagnostic criteria between January 2017 and June 2019 were included in the study. Patients were assigned into two groups: (1) ASD with epilepsy and (2) ASD without epilepsy. Clinical findings were retrieved from patients' files, and EEG findings from first EEG records in the EEG laboratory at the time of diagnosis. EEG findings were defined as central, parietal, frontal, temporal, or generalized, depending on the location of rhythmic discharges. The incidence of epilepsy in our ASD patients was 33.7% and that of febrile convulsion was 4%. Generalized motor seizures were the most common seizure type. Epileptic discharges most commonly derived from the central and frontal regions. These abnormalities, especially frontal and central rhythmic discharges, may represent a precursor for the development of epilepsy in ASD patients.

The aim of this study was to investigate ictal vocalizations associated with myoclonic (MS) and myoclonic-atonic seizures (MAS) in patients with myoclonic epilepsy in infants (MEI) and epilepsy with myoclonic-atonic seizures (EMAS, Doose syndrome), respectively. Subjects were retrospectively recruited among patients with MEI and EMAS for whom ictal video-polygraphs were recorded between 1990 and 2019. We reviewed all MS and MAS in order to estimate how often they were associated with vocalizations, and analyze the temporal relationship between vocalizations and spike-wave complexes (SWCs) and myoclonic EMG potentials based on simultaneous examination of the polygraphs and sound signals. Ictal video-polygraphs from 15 patients with MEI (2-34 MS per patient) and 26 with EMAS (2-26 MAS per patient) were examined. Ictal vocalizations were audible in two patients with MEI (11%; 3-18 MS per patient) and nine with EMAS (35%; 2-11 MAS per patient). Sounds were always non-speech and were immediately followed by head or body dropping in the case of MAS. Detailed analysis based on simultaneous and synchronous examination of video-polygraphs and sound signals in one patient with MEI and five patients with EMAS demonstrated that the onset of the ictal vocalizations corresponded to that of the myoclonic EMG potentials and negative spike components of SWC. Comparison of the length of myoclonic EMG potentials as well as the strength of drop seizures between MAS with and without vocalizations revealed that MAS with vocalizations were associated with longer myoclonic EMG potentials and stronger drop seizures than MAS without vocalizations (p

Background: Epileptic seizures were noted as one of the most overlooked manifestations in syphilis; therefore a few clinicians are concerned about the relationship between epilepsy and syphilis. Our study sought to clarify the prevalence and clinical features of epileptic seizures in patients with syphilis. Methods: We retrieved relevant articles from different databases, using the keywords “syphilis and epilepsy” and then performed statistical analysis to characterize the relationship between these diseases. Results: Forty one articles were included in this study: eight described the prevalence of syphilis and epilepsy and the remaining 33 were case reports on syphilis with epileptic seizures. The meta-analysis included 1252 patients with syphilis. The pooled estimate of proportion of prevalence (95% confidence interval) was 0.1384 (0.0955–0.2005), and the proportion and heterogeneity showed different degrees of change among three subgroups. The systematic review included 46 cases of syphilis with epileptic seizures. Thirty two (80%) patients had motor seizures, among whom 20 (62.5%) had tonic-clonic seizures. In addition, 30 (75%) patients had impaired awareness and 18 (45%) had status seizures. Twenty five (62.5%) patients were 35–55 years of age, and 77.5% of the included patients were men. Thirty seven (97.4%) patients were seizure-free after anti-syphilis treatment. Limitations: Research in this field has been conducted for a relatively short period and publication bias may exist. Furthermore, some patients with syphilis and epileptic seizures may not have received a clear diagnosis. Conclusion: The proportion of prevalence was 0.1384. Most of the included patients were 35–55 years of age and had impaired awareness and motor seizures. Many patients with syphilis and epileptic seizure showed full recovery or the development of minor neurological sequelae, and nearly all patients were seizure-free after timely anti-syphilis treatment.

The aim of the study: This pilot study assessed the ability of a video/audio-based seizure monitoring system to evaluate (I) baseline frequency and severity of nocturnal seizures with motor features in patients with drug-resistant epilepsy (DRE) and (II) the individual effect of brivaracetam (BRV) treatment on number, duration and movement intensity of these seizure types. Algorithmic feature analysis was developed for assessment of qualitative changes in movement intensity measurements within seizure types before and after BRV intervention. Materials and methods: Night-time motor seizures of recruited patients were recorded in two separate four-week monitoring periods. The first period defined a prescreening phase (n = 13 patients) to establish a baseline, and the second period defined the intervention phase (n = 9 patients), with BRV initiated during the second week of the second monitoring period. All recorded nights were analyzed by an expert video reviewer, and all unequivocal seizures were classified by an epileptologist. Seizure frequencies using both seizure diaries and video monitoring were compared. The effect of BRV on both seizure duration and movement intensity was assessed by numerical comparison of visual features calculated from motion characteristics of the video, as well as spectral features from the recorded audio. The statistical significance of changes in seizure duration and intensity before and after the intervention were investigated by Wilcoxon rank-sum test and visual inspection of Kernel density estimation. Results: 8 patients marked seizures in their seizure diaries during the prescreening phase. During the three-week follow-up, three patients achieved > 50% seizure decrease, four patients did not respond to treatment, and two patients experienced worsening of seizures. Five patients were able to document 40–70% of their seizures compared to the video/audio monitoring system. According to the signal feature analysis the intervention decreased movement intensity with clear clinical significance in three patients, whereas statistically significant differences in features appeared in 8 out of 9 patients. Conclusions: The novel video/audio monitoring system improved the evaluation of treatment effect compared to the seizure diaries and succeeded in providing a comparative intra-patient assessment of the movement intensity and duration of the recorded seizures. publishedVersion

Seizures are uncommon with posterior circulation strokes. They are more often associated with anterior circulation strokes, with only a limited number of cases of status epilepticus reported to be related to brain stem ischemia. The literature includes case reports of generalized tonic-clonic seizures and associated status epilepticus as an initial presentation of acute basilar artery thrombosis. However, there are only rare cases reporting focal motor seizure as status epilepticus in the setting of acute basilar artery thrombosis, an important clinical presentation that should prompt evaluation for acute brain stem ischemia.

Purpose Accurate characterization and quantification of seizure types are critical for optimal pharmacotherapy in epilepsy patients. Technological advances have made it possible to continuously monitor physiological signals within or outside the hospital setting. This study tested the utility of single-channel surface-electromyography (sEMG) for characterization of motor epileptic seizure semiology. Methods Seventy-one subjects were prospectively enrolled where vEEG and sEMG were simultaneously recorded. Three epileptologists independently identified and classified seizure events with upper-extremity (UE) motor activity by reviewing vEEG, serving as a clinical standard. Surface EMG recorded during the events identified by the clinical standard were evaluated using automated classification methods and expert review by a second group of three independent epileptologists (blinded to the vEEG data). Surface EMG classification categories included: tonic-clonic (TC), tonic only, clonic only, or other motor seizures. Both automated and expert review of sEMG was compared to the clinical standard. Results Twenty subjects experienced 47 motor seizures. Automated sEMG event classification methods accurately classified 72 % (95 % CI [0.57, 0.84]) of events (15/18 TC seizures, 5/9 tonic seizures, 1/3 clonic seizures, and 13/17 other seizures). Three independent reviewers’ majority-rule analysis of sEMG correctly classified 81 % (95 % CI [0.67, 0.91]) of events (16/18 TC seizures, 8/9 tonic seizures, 1/3 clonic seizures, and 13/17 other manifestations). Conclusions Continuous monitoring of sEMG data provides an objective measure to evaluate motor seizure activity. Using sEMG from a wearable monitor recorded from the biceps, automated and expert review may be used to characterize the semiology of events with UE motor activity, particularly TC and tonic seizures.

Objectives Eliciting a generalized seizure is essential to electroconvulsive therapy (ECT), but there is still a need to understand how patient and session variables interact to generate a seizure of adequate quality. Here, we investigate factors associated with motor seizure length as a measure of quality in a large database of patients who underwent ECT. Methods This is a retrospective cohort including data from all adult inpatients who underwent ECT at a university hospital in Brazil from 2009 to 2015. We used linear mixed models to investigate the effects of patient, session, and medication on seizure length. Results Session information was available for 387 patients, a total of 3544 sessions and 4167 individual stimulations. Multiple stimulations were necessary in 12.4% of sessions. Median seizure length was 30 seconds. Seizure length was directly correlated with stimulus dosage and inversely correlated with the session number, patient age, prescription of anticonvulsants in the day before and β-blockers during the session, and the thiopental dose. Use of benzodiazepines was not associated with a shorter seizure duration, irrespective of dose. Conclusions We demonstrate here how motor seizure length evolves during a course of ECT. With a large number of sessions, we are able to integrate a host of factors in a prediction model. Seizure quality was influenced by a number of the studied factors, many of which are potentially modifiable and could be assessed before initiating and handled during treatment.

PURPOSE: This study aims to characterize the natural history of generalized motor seizures through longitudinal stratification of patient-reported clinical seizures into high, medium and low rates of generalized motor seizures (also known as generalized tonic-clonic seizures or GTCs). METHODS: From 2007–2018, 1.4 million seizures were recorded by 12,402 SeizureTracker.com users that met inclusion/exclusion criteria. The number of GTCs per year since the first seizure diary entry was calculated for each user and categorized as: Low (0 GTCs/year), Medium (1–2 GTCs/year), or High (≥ 3 GTCs/year) GTC rates. RESULTS: Kaplan-Meier survival curves for the time until exiting the initial category were computed. There was a global difference between risk groups (p

Subacute sclerosing panencephalitis (SSPE) is a degenerative disease of the brain caused by a persistent measles virus infection occurring mostly in childhood or early adolescence. The spectrum of epileptic phenomena associated with SSPE is wide, varying from partial or generalized tonic-clonic seizures and atypical absences to myoclonic-atonic attacks. Tonic seizures are very rare in SSPE.Herein, we discuss a case of 25 years old male that presented with adult-onset SSPE with tonic seizures accompanying myoclonic seizures.Patient was treated with clonazepam 5 mg/day and an isoprinosine regimen with a dose of 70 mg/kg/day. This is the fourth case of SSPE presenting with myoclonic and tonic seizures and the first case of SSPE with myoclonic and tonic seizures reported in an adult-onset case in the English literature.Adult-onset SSPE with tonic seizures is rare and may be confusing, thus, it is important to recognize the presence of this type of tonic motor seizures in SSPE patients.

Представяме клиничен случай на 70-годишна жена, постъпила по спешност в Клиника по неврология със заплашващ епилептичен статус от прости моторни пристъпи в дясна лицева половина и дясна ръка. Проведената компютърна томография на глава визуализира калцирала екстрааксиална окръглена формация вляво, задно фронтално, вероятно менингиом. При постъпването и по време на симптоматичните пристъпи се регистрираха динамични промени в ЕКГ с негативиране на Т-вълните прекордиално, както и покачване на серумния тропонин в динамика. Ехокардиографски се установи хипокинезия, обхващаша средните сегменти на лявата камера. Проведе се инвазивно изследване, при което не се установиха данни за коронарна болест. От вентрикулографията е с данни за хипокинезия на средни сегменти и се измери ФИ 47%. Проведе се комплексна терапия с валпроат, дексаметазон, манитол и клоназепам, леветирацетам, ацетизал, статин и бета-блокер. След овладяване на епилептичните пристъпи и уточняване на сърдечно-съдовия статус, пациентката беше насочена за неврохирургично лечение на туморната формация. We present a clinical case of a 70-year-old woman who was urgently admitted to the Department of Neurology with threatening status epilepticus from simple motor seizures in the right half of the face and right hand. Computed tomography of the head visualized a calcified extra axial round formation in the left posterofrontal, possibly a meningioma. On admission and during the symptomatic attacks, dynamic ECG changes were recorded with negative T waves precordially, as well as a dynamic increase in serum troponin. Echocardiography revealed hypokinesia involving the middle segments of the left ventricle. Coronary angiography was performed with no evidence of coronary disease. From the ventriculography hypokinesia of middle segments was found and measured EF 47%. Complex therapy with valproate, dexamethasone, mannitol and clonazepam, levetiracetam, acetizal, a statin and a beta-blocker was initiated. After controlling the epileptic seizures and diagnostic work-up of the cardiovascular status, the patient was referred for neurosurgical treatment of the tumor formation.

New onset motor seizures, especially generalized convulsive seizures, are common but frightening to patients and bystanders, and typically result in evaluation in the emergency department (ED). Emergency physicians are challenged to confirm whether the event precipitating the visit was indeed a seizure rather than a mimic such as stroke, syncope, non-epileptic spell, or migraine. When a true seizure is suspected, the next step is to identify a precipitating cause.

Purpose: Focal seizures constitute the most common seizure type and are associated with poor control. One of the major difficulties in detecting focal onset with wearable devices seizures is related to their phenomenological complexity. We aimed at capturing focal onset seizures with motor manifestations with a multimodal wearable device to identify the digital semiology and the evolution pattern of ictal manifestations. Methods: Participants were asked to wear a multimodal wearable device (IMEC) aimed at seizure detection while admitted to an epilepsy monitoring unit. Seizures were labelled by a neurologist and start and offset time were noted. The signals captured by the device during the seizure window were plotted and a visual inspection was performed for focal motor seizures with impaired awareness and for focal motor aware seizures. Results: Fifty-three seizures from twelve patients with focal seizures with motor manifestations recorded with the device were visually inspected. Overall, a common pattern presented across focal motor seizures with impaired awareness and it was characterized by early cardiac manifestations followed by motor phenomena and final EDA response. Motor seizures with retained awareness appeared to be characterized by brief motor events not associated with major autonomic manifestations Conclusion: an overall common digital phenotype and time-evolution pattern was demonstrated for focal motor seizures with impaired awareness. The identification of the evolution pattern could more precisely inform the development of highly preforming algorithms opening the possibility to a more precise, and potentially customizable way to optimize focal seizure detection.

Focal Nonmotor Versus Motor Seizures: The Impact on Diagnostic Delay in Focal Epilepsy Pellinen J, Tafuro E, Yang A, et al. Epilepsia. 2020;61(12):2643-2652. doi:10.1111/epi.16707Objective:To test the hypothesis that people with focal epilepsy experience diagnostic delays that may be associated with preventable morbidity, particularly when seizures have only nonmotor symptoms, we compared time to diagnosis, injuries, and motor vehicle accidents (MVAs) in people with focal nonmotor versus focal seizures with motor involvement at epilepsy onset.Methods:This retrospective study analyzed the enrollment data from the Human Epilepsy Project, which enrolled participants between 2012 and 2017 across 34 sites in the United States, Canada, Europe, and Australia, within 4 months of treatment for focal epilepsy. A total of 447 participants were grouped by initial seizure semiology (focal nonmotor or focal with motor involvement) to compare time to diagnosis and prediagnostic injuries including MVAs.Results:Demographic characteristics were similar between groups. There were 246 (55%) participants with nonmotor seizures and 201 (45%) participants with motor seizures at epilepsy onset. Median time to diagnosis from first seizure was 10 times longer in patients with nonmotor seizures compared to motor seizures at onset (P < .001). The number and severity of injuries were similar between groups. However, 82.6% of MVAs occurred in patients with undiagnosed nonmotor seizures.Significance:This study identifies reasons for delayed diagnosis and consequences of delay in patients with new-onset focal epilepsy, highlighting a treatment gap that is particularly significant in patients who experience nonmotor seizures at epilepsy onset.

Objective Essential oils (EOs) like eucalyptus and camphor have pro-convulsant properties. These EOs are present in many over- the- counter balms and oils. The effect of exposure to these EOs and occurrence of seizure is not systematically studied. The aim of this study was to evaluate the relationship between essential oils and the first episode of seizure and breakthrough seizures in known epileptic patients. Methods This was a multi-center prospective study, conducted in four hospitals over four years. Every person presenting with the first episode of seizure or breakthrough seizure was asked about exposure to EOs, mode of exposure, time to onset of a seizure in relationship to exposure, duration of seizure, type of seizure, and antiepileptic drug therapy. Results During the four-year period there were 55 patients with essential oil-related seizure (EORS). 22(40 %) had essential oil-induced seizures (EOIS) and 33(60 %) had essential oil-provoked seizures (EOPS). The female: male ratio was 1:1.1, the age of the patients ranged from 8 months to 77 years. In the EOIS group, 95 % had generalized tonic-clonic seizures and 5% had focal impaired awareness seizures. In the EOPS group, 42.4 % had focal impaired awareness seizures, 27.3 % generalized tonic-clonic seizures, 15 % focal to bilateral tonic-clonic seizures, and 15 % focal aware motor seizures. EOs implicated were preparations containing eucalyptus and camphor. Conclusion Exposure to essential oils of eucalyptus and camphor is an under-recognized cause of the first and breakthrough seizure. Identifying the true causative factor will prevent unnecessary antiepileptic drug therapy and future recurrence.

We have developed a healthcare mobile application, for human activity recognition, monitoring of well-being and detection of individuals going towards a health hazard based on the data collected from sensors embedded in mobile phones and wearables. The data from sensors are processed within the mobile application to detect and classify different Activities of Daily Living. The developed framework is used to collect data in an unconstraint environment from individuals suffering from neurological disorders. The data is further tested using signal processing and machine learning algorithms. Results of in-app processing and classification are stored in a dedicated mobile database for later reference and analysis. This paper shows that statistical and Machine Learning methods can also be used within a mobile application for classification of ADLs. MyNeuroHealth has been designed in accordance with the scale of the prevalence of neurological disorders among the general population of developing countries and has become more relevant in COVID-19 pandemic as it offers real-time nonintrusive monitoring. Results show that MyNeuroHealth can detect and classify Motor Seizures and falls with an accuracy of 99%. The app is also able to detect if a patient had stumbled or fallen due to any reason and notifies caregiver accordingly.

Super-refractory status epilepticus (SRSE) represents a neurological emergency that is characterized by a lack of response to the third line of antiepileptic treatment, including intravenous general anesthetics. It is a medical challenge with high morbidity and mortality. Electroconvulsive therapy (ECT) has been recommended as a nonpharmacologic option of treatment after other alternatives are unsuccessful. Its effect on the cessation of SRSE has been minimally investigated. The objective of this article is to analyze the effect of ECT on SRSE. For this purpose, a multidisciplinary team created a protocol based on clinical guidelines similar to those described previously by Ray et al. (2017). ECT was applied to six patients with SRSE after the failure of antiepileptic treatment and pharmacologic coma.The objective of each ECT session was to elicit a motor seizure for at least 20 s. SRSE was resolved in all patients after several days of treatment, including ECT as a therapy, without relevant adverse effects. Thus, ECT is an effective and feasible option in the treatment of SRSE, and its place in the algorithm in treatment should be studied due to the uncommon adverse effects and the noninvasive character of the therapy.

Seizure detection devices can improve epilepsy care, but wearables are not always tolerated. We previously demonstrated good performance of a real‐time video‐based algorithm for detection of nocturnal convulsive seizures in adults with learning disabilities. The algorithm calculates the relative frequency content based on the group velocity reconstruction from video‐sequence optical flow. We aim to validate the video algorithm on nocturnal motor seizures in a pediatric population. We retrospectively analyzed the algorithm performance on a database including 1661 full recorded nights of 22 children (age = 3‐17 years) with refractory epilepsy at home or in a residential care setting. The algorithm detected 118 of 125 convulsions (median sensitivity per participant = 100%, overall sensitivity = 94%, 95% confidence interval = 61%‐100%) and identified all 135 hyperkinetic seizures. Most children had no false alarms; 81 false alarms occurred in six children (median false alarm rate [FAR] per participant per night = 0 [range = 0‐0.47], overall FAR = 0.05 per night). Most false alarms (62%) were behavior‐related (eg, awake and playing in bed). Our noncontact detection algorithm reliably detects nocturnal epileptic events with only a limited number of false alarms and is suitable for real‐time use.

Super refractory status epilepticus ( SRSE ) is the status epilepticus in which the seizures continue for 24 hour or more after anesthetic medications to control and reappear when the patient is weaned from the anesthetic agent. Characteristic seizures are distinguished, by the presence and/or absence of motor seizure, into convulsive and non-convulsive status epilepticus (NCSE). An aggressive therapy is often needed after diagnosis is confirmed. Continuous monitoring during therapy is very important. The management for SRSE includes pharmacological and non-pharmacological treatment. Clinical and electrographic seizure suppression is the treatment goal. After that we need to find and treat the cause of seizures. Clinical judgement by the clinician is needed to identify the risks of excessive suppression of the nervous system, so as the morbidity and mortality is reduced for those patients who can survive but often have difficult and prolonged recovery. Received: 24 Oct 2018Reviewed: 28 Oct 2018Accepted: 6 Nov 2018 Citation: Kurniawan D, Machin A, Hidayati HB, Islamiyah WR. Management of super refractory status epilepticus. Anaesth Pain & Intensiv Care 2018;22 Suppl 1:S67-S72

In this proof-of-concept investigation, we demonstrate a marker-free video-based method to detect nocturnal motor seizures across a spectrum of motor seizure types, in a nighttime setting with a single adult female with refractory epilepsy. In doing so, we further explore the intermediate biosignals, visually mapping seizure “fingerprints” to seizure types. The method is designed to be flexible enough to generalize to unseen data, and shows promising performance characteristics for low-cost seizure detection and classification. The dataset contained recordings from 27 recorded nights. Seizure events were observed in 22 of these nights, with 36 unequivocally confirmed seizures. Each seizure was classified by an expert epileptologist according to both the ILAE 2017 standard and the Lüders semiological classification guidelines, yielding 5 of the ILAE-recognized seizure types and 7 distinct seizure semiologies. Evaluation was based on inference of motion, oscillation, and sound signals extracted from the recordings. The model architecture consisted of two feature extraction and event determination layers and one thresholding layer, establishing a simple framework for multimodal seizure analysis. Training of the optimal parameters for was done by randomly resampling the event hits for each signal, and choosing a threshold that kept an expected 90% sensitivity for the sample distribution. With the cut-off values selected, statistical performance was calculated for two target seizure groups: those containing a clonic component, and those containing a tonic component. When tuned to 90% sensitivity, the system achieved a very low false discovery rate of 0.038/hour when targeting seizures with a clonic component, and a clinically-relevant rate of 1.02/hour when targeting seizures with a tonic component. These results indicate a sensitive method for detecting various nocturnal motor seizure types, and a high potential to differentiate motor seizures based on their video and audio signal characteristics. Paired with the low cost of this technique, both cost savings and improved quality of care might be achieved through further development and commercialization of this method.

Remodeled Cortical Inhibition Prevents Motor Seizures in Generalized Epilepsy Jiang X, Lupien-Meilleur A, Tazerart S, Lachance M, Samarova E, Araya R, Lacaille JC, Rossignol E. Ann Neurol. 2018 Sep;84(3):436-451.Objective: Deletions of CACNA1A, encoding the α1 subunit of CaV 2.1 channels, cause epilepsy with ataxia in humans. Whereas the deletion of Cacna1a in γ-aminobutyric acidergic (GABAergic) interneurons (INs) derived from the medial ganglionic eminence (MGE) impairs cortical inhibition and causes generalized seizures in Nkx2.1Cre;Cacna1ac/c mice, the targeted deletion of Cacna1a in somatostatin-expressing INs (SOM-INs), a subset of MGE-derived INs, does not result in seizures, indicating a crucial role of parvalbumin-expressing (PV) INs. Here, we identify the cellular and network consequences of Cacna1a deletion specifically in PV-INs. Methods: We generated PVCre;Cacna1ac/c mutant mice carrying a conditional Cacna1a deletion in PV neurons and evaluated the cortical cellular and network outcomes of this mutation by combining immunohistochemical assays, in vitro electrophysiology, 2-photon imaging, and in vivo video-electroencephalographic recordings. Results: PVCre;Cacna1ac/c mice display reduced cortical perisomatic inhibition and frequent absences, but only rare motor seizures. Compared to Nkx2.1Cre;Cacna1ac/c mice, PVCre;Cacna1ac/c mice have a net increase in cortical inhibition, with a gain of dendritic inhibition through sprouting of SOM-IN axons, largely preventing motor seizures. This beneficial compensatory remodeling of cortical GABAergic innervation is mechanistic target of rapamycin complex 1 (mTORC1)-dependent, and its inhibition with rapamycin leads to a striking increase in motor seizures. Furthermore, we show that a direct chemogenic activation of cortical SOM-INs prevents motor seizures in a model of kainate-induced seizures. Interpretation: Our findings provide novel evidence suggesting that the remodeling of cortical inhibition, with an mTOR-dependent gain of dendritic inhibition, determines the seizure phenotype in generalized epilepsy and that mTOR inhibition can be detrimental in epilepsies not primarily due to mTOR hyperactivation.

SummaryObjective Automated seizure detection and alarming could improve quality of life of and potentially prevent sudden, unexpected death in patients with severe epilepsy. As currently available systems focus on tonic-clonic seizures, we want to detect a broader range of seizure types including tonic, hypermotor and clusters of seizures. Methods In this multi-centre, prospective cohort study, non-EEG signals heart rate and accelerometry were measured during the night in patients undergoing a diagnostic video-EEG examination. Based on clinical video-EEG data seizures were classified and categorized as clinically urgent or not. Seizures included for analysis were tonic, tonic-clonic, hypermotor and clusters of short myoclonic/tonic seizures. Features reflecting physiological changes in heart rate and movement were extracted. Detection algorithms were developed based on stepwise fulfillment of conditions during increases in either feature. A training set was used for development of algorithms and an independent test set for assessing performance. Results 95 patients were included, but due to sensor failures, data from only 43 (out of whom 23 patients had 86 seizures, representing 402 hours of data) could be used for analysis. The algorithms yield acceptable sensitivities, especially for clinically urgent seizures (sensitivity 71%-87%), but come with high false alarm rates (2.3-5.7 per night, positive predictive value 25-43%). There was a large variation in number of false alarms per patient. Significance It seems feasible to develop a detector with a high sensitivity but false-alarm rates are too high for use in clinical practice. For further optimization, personalization of algorithms may be necessary. This article is protected by copyright. All rights reserved.

International audience; – We report a 37-year-old, right-handed patient with drug-resistant focal epilepsy whose seizures were characterized by explosive hyperkinetic behaviour. Video-SEEG revealed bifocal organization of epilepsy with two distinct cortical origins of seizures: the right temporal pole and left temporal lateral and perisylvian cortex. Irrespective of the cortical pattern of seizure onset, the hyperkinetic semiology was extremely similar. This supports a major role for " final common pathway " subcortical circuits in the genesis of the hyperkinetic semiology in this patient.

This companion paper to the introduction of the International League Against Epilepsy (ILAE) 2017 classification of seizure types provides guidance on how to employ the classification. Illustration of the classification is enacted by tables, a glossary of relevant terms, mapping of old to new terms, suggested abbreviations, and examples. Basic and extended versions of the classification are available, depending on the desired degree of detail. Key signs and symptoms of seizures (semiology) are used as a basis for categories of seizures that are focal or generalized from onset or with unknown onset. Any focal seizure can further be optionally characterized by whether awareness is retained or impaired. Impaired awareness during any segment of the seizure renders it a focal impaired awareness seizure. Focal seizures are further optionally characterized by motor onset signs and symptoms: atonic, automatisms, clonic, epileptic spasms, or hyperkinetic, myoclonic, or tonic activity. Nonmotor-onset seizures can manifest as autonomic, behavior arrest, cognitive, emotional, or sensory dysfunction. The earliest prominent manifestation defines the seizure type, which might then progress to other signs and symptoms. Focal seizures can become bilateral tonic-clonic. Generalized seizures engage bilateral networks from onset. Generalized motor seizure characteristics comprise atonic, clonic, epileptic spasms, myoclonic, myoclonic-atonic, myoclonic-tonic-clonic, tonic, or tonic-clonic. Nonmotor (absence) seizures are typical or atypical, or seizures that present prominent myoclonic activity or eyelid myoclonia. Seizures of unknown onset may have features that can still be classified as motor, nonmotor, tonic-clonic, epileptic spasms, or behavior arrest. This "users' manual" for the ILAE 2017 seizure classification will assist the adoption of the new system.