bluuebunny/crossref_metadata_2025_split huggingface dataset:
Adjunctive lacosamide for focal epilepsy: an open‐label trial evaluating the impact of flexible titration and dosing on safety and seizure outcomes
Authors: Baulac Michel , Coulbaut Safia , Doty Pamela , McShea Cindy , De Backer Marc , Bartolomei Fabrice , Vlaicu Mihaela 
Published: August 2019

AbstractAims. To evaluate the safety and effectiveness of lacosamide in a real‐life setting with the use of a flexible dose titration schedule and individualised maintenance doses up to the maximum approved dose of 400 mg/day.Methods. Adults with a diagnosis of focal seizures, with or without secondary generalization, were enrolled in this open‐label Phase IV trial (NCT01235403). Lacosamide was initiated at 100 mg/day (50 mg bid) and uptitrated over a 12‐week period to 200, 300 or 400 mg/day, based on safety and seizure control. Although dose increases were to be in increments of 100 mg/day, intermediate doses were permitted at each escalation step for one week for patients known to be particularly sensitive to starting new AEDs. After receiving a stable, effective dose for three weeks, patients entered the 12‐week maintenance period. Primary outcomes were incidence of treatment‐emergent adverse events (TEAEs) and withdrawal due to TEAEs. Seizure outcomes, all secondary, were median focal seizure frequency, ≥50% reduction in focal seizure frequency, and seizure freedom.Results. One hundred patients with a mean age of 44 years were enrolled and 74 completed the trial. The incidence of TEAEs was 64.0% (n=100), with the most frequently reported (≥5% of patients) being dizziness, headache, and asthenia. Fourteen patients withdrew due to TEAEs, most frequently due to dizziness (six patients; 6.0%), vomiting (two patients; 2%), and tremor (two patients; 2%). Among patients with baseline and maintenance phase seizure data (n=75), median reduction in focal seizure frequency from baseline was 69.7% and the ≥50% responder rate was 69.3%. Among 74 patients who completed the maintenance phase, 21 (28.4%) were seizure‐free.Conclusion. Flexible lacosamide dosing in this open‐label trial was associated with a favourable tolerability and safety profile; the nature of the TEAEs was consistent with that observed in previous pivotal trials. Treatment with lacosamide was also associated with effective seizure control.
The semiology of benign focal epilepsy with affective symptoms
Authors: Nascimento Fábio A. , Sotero de Menezes Márcio A. , Simão Cristiane A. , Takeshita Bruno T. , Blattes da Rocha Samanta F. , Kowacs Pedro A. 
Published: August 2019

AbstractBenign focal epilepsy with affective symptoms (BFEAS) is a rare childhood epilepsy syndrome essentially characterized by “epileptic attacks with affective symptoms of a terrifying type”. Since the original description, approximately 50 cases have been reported. To our knowledge, however, none of the studies included video‐EEG data. Herein, we detail the electroclinical features of a neurodevelopmentally normal 9‐year‐old boy with epilepsy since the age of 2 years. His seizure semiology essentially consisted of nocturnal focal seizures featuring abrupt fear and autonomic phenomena (such as excessive sweating, repeated swallowing, and coughing), associated with impaired consciousness. These seizures were often secondary generalized, and he had multiple episodes of convulsive status epilepticus. He has been seizure‐free for the past year and a half on dual antiepileptic therapy with sulthiamine and valproate. His intellectual and social abilities are excellent (IQ of 116), although he does have difficulties particularly in language learning, and was recently diagnosed with phonological dyslexia with dysorthography. By presenting our patient's history and video‐EEG, we intend to further detail the semiology of seizures with affective symptomatology. [Published with video sequence on www.epilepticdisorders.com]
Epilepsy after cerebral infection: review of the literature and the potential for surgery
Authors: Ramantani Georgia , Holthausen Hans 
Published: August 2019

AbstractThe risk of unprovoked seizures in population‐based cohorts of cerebral infection survivors is 7–8% in developed countries, rising to considerably higher rates in resource‐poor countries. The main risk factors for epilepsy after cerebral infection, besides acute seizures, are infection‐associated brain lesions and status epilepticus during the acute phase. Despite the high prevalence of pharmacoresistant epilepsies after cerebral infections, especially in patients with MRI‐identifiable lesions, only a small minority undergoes epilepsy surgery. However, excellent surgical candidates are particularly those with a history of meningitis or encephalitis in early childhood, hippocampal sclerosis on MRI, as well as a history, seizure semiology, and EEG‐findings compatible with the diagnosis of a mesial temporal lobe epilepsy syndrome. More challenging are patients with neocortical/extratemporal lobe epilepsies post cerebral infection. Finally, patients with a severe hemispheric injury with contralateral hemiparesis are candidates for hemispherectomy/hemispherotomy. This review attempts to shed some light on this frequent cause of symptomatic focal epilepsy, with an emphasis on the chances offered by epilepsy surgery.
Focal epilepsy with paroxysmal pain due to somatic injury
Authors: Yu Tao , Liu Chang , Zhang Guojun , Qiao Liang , Ni Duanyu , Wang Yuping , Li Yongjie 
Published: August 2019

AbstractWe present two cases with paroxysmal pain that developed after a somatic injury to the trunk. The main characteristic of the episodes was paroxysmal severe pain, mainly located in the original region of somatic injury, with ipsilateral tonic or dystonic behaviour. The clinical characteristics supported a diagnosis of focal epilepsy. Both scalp EEG and MEG findings suggested epileptic activities on the contralateral central cortex. The focal seizures had a good response to antiepileptic drugs. It is hypothesized that peripheral somatic injuries can modify cortical excitability and lead to plastic changes in the sensory/motor cortex, ultimately resulting in focal seizures. We provide additional evidence for the phenomenon that a peripheral somatic injury could induce focal epilepsy. [Published with video sequence on www.epilepticdisorders.com]
Assessing quantitative EEG spectrograms to identify non‐epileptic events
Authors: Goenka Ajay , Boro Alexis , Yozawitz Elissa 
Published: August 2019

AbstractAims. To evaluate the sensitivity and specificity of quantitative EEG (QEEG) spectrograms in order to distinguish epileptic from non‐epileptic events.Methods. Seventeen patients with paroxysmal non‐epileptic events, captured during EEG monitoring, were retrospectively assessed using QEEG spectrograms. These patients were compared to a control group of 13 consecutive patients (ages 25‐60 years) with epileptic seizures of similar semiology. Assessment of raw EEG was employed as the gold standard against which epileptic and non‐epileptic events were validated. QEEG spectrograms, available using Persyst 12 EEG system integration software, were each assessed with respect to their usefulness to distinguish epileptic from non‐epileptic seizures. The given spectrogram was interpreted as indicating a seizure if, at the time of the clinically identified event, it showed a visually significant change from baseline.Results. Eighty‐two clinically identified paroxysmal events were analysed (46 non‐epileptic and 36 epileptic). The “seizure detector trend analysis” spectrogram correctly classified 33/46 (71%) non‐epileptic events (no seizure indicated during a clinically identified event) vs. 29/36 (81%) epileptic seizures (seizure indicated during a clinically identified event) (p=0.013). Similarly, “rhythmicity spectrogram”, FFT spectrogram, “asymmetry relative spectrogram”, and integrated‐amplitude EEG spectrogram detected 28/46 (61%), 30/46 (65%), 22/46 (48%) and 27/46 (59%) non‐epileptic events vs. 27/36 (75%), 25/36 (69%), 25/36 (69%) and 27/36 (75%) epileptic events, respectively.Conclusions. High sensitivities and specificities for QEEG seizure detection analyses suggest that QEEG may have a role at the bedside to facilitate early differentiation between epileptic seizures and non‐epileptic events in order to avoid unnecessary administration of antiepileptic drugs and possible iatrogenic consequences.
Atonic seizures in children with surgically remediable epilepsy: a motor system seizure phenotype?
Authors: Scholly Julia , Bartolomei Fabrice , Valenti‐Hirsch Maria Paola , Boulay Clotilde , De Saint Martin Anne , Timofeev Alexander , Kehrli Pierre , Hirsch Edouard 
Published: August 2019

AbstractAim. Atonic seizures are common in some epileptic syndromes beginning in infancy or early childhood but they are rarely described in epilepsy with focal seizures of structural aetiology. We aimed to characterize the electroclinical features of atonic seizures in surgically remediable paediatric patients and to study the spatiotemporal organization of the underlying epileptogenic networks.Methods. We retrospectively analysed two consecutive, longitudinally evaluated and surgically treated paediatric patients presenting with atonic seizures as a manifestation of pharmacoresistant epilepsy of structural aetiology, evidenced by scalp‐ and stereotactic intracerebral video‐EEG‐recordings. A quantitative analysis of the epileptogenic zone organization was performed using the “epileptogenicity index”.Results. Long‐lasting generalized ictal atonia, occurring in infancy, was a predominant clinical feature in both patients, with some hints of focal origin present in one case. The seizure phenotype evolved at later age into subtle segmental atonia, associated with prominent positive motor signs. The epileptogenic zone was localized within the dorsolateral or mesiolateral premotor region. Its spatial organization was focal, matching the lesional cortex in one and distributed involving several lesional and non‐lesional structures in the other case. The emergence of atonic semiology temporally correlated with involvement of both lateral and mesial premotor, as well as primary motor areas.Conclusion. We hypothesize that atonic seizures may be considered as a motor system seizure phenotype in the setting of structural epilepsy. Complete removal of the epileptogenic area provided excellent seizure control.
Exceptional response to brivaracetam in a patient with refractory idiopathic generalized epilepsy and absence seizures
Authors: Grande‐Martín Alberto , Sopelana‐Garay David , Pardal‐Fernández José Manuel , Sánchez‐Honrubia Rosa María , Sánchez‐Larsen Álvaro Abelardo 
Published: August 2019

ABSTRACTBrivaracetam is currently indicated as adjunctive therapy for patients with focal‐onset seizures with or without secondary generalization. However, it has been suggested that it could provide broad‐spectrum efficacy given its similarity to levetiracetam and based on the results from preclinical studies and others of patients with generalized epilepsy. We present the case of a woman with refractory idiopathic generalized epilepsy and absence seizures with dramatic response to brivaracetam. Our report supports a consideration of treatment with this new antiepileptic drug on a case‐by‐case basis in patients with refractory generalized epilepsy, while we await further studies on this topic.
Sleep‐related hypermotor epilepsy activated by rapid eye movement sleep
Authors: Wang Xiaoli , Marcuse Lara V , Jin Lang , Zhang Ying , Zhang Wenjuan , Chen Beibei , Yuan Na , Wang Bi , Liu Yonghong 
Published: August 2019

ABSTRACTMost sleep‐related seizures occur during non‐rapid eye movement (NREM) sleep, particularly during stage changes. Sleep‐related hypermotor epilepsy (SHE) is a rare epileptic syndrome characterized by paroxysmal motor seizures, mainly arising from NREM sleep. Here, we report a patient with SHE who had seven seizures captured on video‐EEG‐polysomnography during REM sleep. Ictal semiology of this patient ranged from brief paroxysmal arousals to hypermotor seizures. On EEG‐polysomnography, the spontaneous arousals were more frequent during REM than NREM sleep, with a considerably higher arousal index in REM sleep (20/hour). While the reason for seizures during REM sleep in this patient is unclear, we speculate that the threshold and mechanisms of arousal during different sleep stages may be related to the occurrence of seizures. [Published with video sequences on www.epilepticdisorders.com].
Ictal spitting in non‐dominant temporal lobe epilepsy: an anatomo‐electrophysiological correlation
Authors: Quevedo‐Diaz Marcos , Campo Adrià Tauste , Vila‐Vidal Manel , Principe Alessandro , Ley Miguel , Rocamora Rodrigo 
Published: August 2019

ABSTRACTWe report a patient presenting drug‐resistant, non‐dominant temporal lobe epilepsy with ictal spitting and prosopometamorphopsia, both extremely rare semiologies. Second‐phase pre‐surgical monitoring was performed using SEEG due to lesion‐negative imaging and the rare semiology. The seizure onset zone was delimited to the right anterior hippocampus and the temporobasal cortex, with the propagation zone within the entorhinal cortex. Interestingly, direct electrical stimulation to the entorhinal cortex, which was reproduced in a number of trials, evoked spitting without leading to seizures or post‐discharges. After the resection of the epileptogenic zone, the patient remained seizure‐free without AEDs for a follow‐up period of five years (Engel Class 1a). The neuropathology revealed a focal cortical dysplasia type FCD‐Ia. Spectral analysis of intracranial ictal EEG (iEEG) data suggested a possible role of the basal temporal and entorhinal cortex as a necessary node in ictal spitting. [Published with video sequences onwww.epilepticdisorders.com].
Electroclinical and radiological observation of dysfunctional zones in a patient with neurosyphilis
Authors: Sakai Katsuya , Yazawa Shogo , Sugimoto Akiko , Nakao Koichi , Tsuruta Kazuhito , Ochiai Eiji , Suzuki Yukiko , Matsuhashi Masao 
Published: August 2019

ABSTRACTWe report a 33‐year‐old Japanese man who suffered from repetitive generalized tonic‐clonic seizures which were medically intractable. Neurosyphilis was serologically diagnosed in blood and cerebrospinal fluid, and penicillin G (PcG) was consequently effective. The EEG during PcG pre‐treatment showed frequent right occipital spikes and right frontocentral slow waves, which disappeared after treatment. During pre‐treatment, positron emission tomography with 18‐fluorodeoxyglucose and Tc‐99m ethyl cysteinate dimer single‐photon emission computed tomography revealed occipital hypermetabolism and hyperperfusion (“hot” area) and fronto‐temporo‐parietal hypometabolism and hypoperfusion (“cool” area) over the right hemisphere. The spike sources of magnetoencephalography during pre‐treatment were localized to “hot” areas, and the slow activities were distributed to the fronto‐temporo‐parietal region, corresponding to “cool” areas. The inflammatory seizure focus and reversible dysfunctional zone associated with neurosyphilis were clearly delineated using these techniques.
Atypical postictal transient subcortical T2 hypointensity in a newly diagnosed diabetic patient with seizures
Authors: Paoletti Matteo , Bacila Ana , Pichiecchio Anna , Farina Lisa Maria , Rognone Elisa , Cremascoli Riccardo , Fanucchi Simona , Manni Raffaele , Bastianello Stefano 
Published: August 2019

ABSTRACTCommon postictal MRI findings include transient cortical T2 hyperintensity, restricted diffusion, and gyral and/or adjacent leptomeningeal contrast enhancement. In certain uncommon pathological conditions, other signal abnormalities can be present, suggesting a different underlying pathogenic mechanism. We report the case of a 66‐year‐old man, recently diagnosed with diabetes mellitus type 2, presenting with new‐onset visual and auditory hallucinations, “absence” seizures, and repeated peaks of hyperglycaemia without hyperketonaemia or increased serum osmolarity. EEG confirmed epileptic discharges in the right temporal region and MRI showed vast subcortical T2 hypointensity in the right temporal lobe, without any cortical hyperintensity, restricted diffusion, or contrast enhancement. Subcortical signal abnormality and EEG discharges resolved after a month of follow‐up, with a small juxtacortical gliotic focus as a sequela. Peaks in hyperglycaemia have been reported to be responsible for T2 hypointense subcortical abnormalities through a proconvulsant mechanism linked to increased ketone body concentrations. Hyperosmolarity and hyperketonaemia were not evident in this case, however, transient accumulation of free radicals that alter the intercellular space can be considered the presumable cause of this finding. In summary, it is important to consider any unusual findings on postictal MRI in order to avoid errors in interpretation.
A comparison between the 1981 and 2017 International League Against Epilepsy classification of seizure types based on an outpatient setting
Authors: Gao Hui , Sander Josemir W. , Xiao Yingfeng , Zhang Yingying , Zhou Dong 
Published: August 2019

ABSTRACTAim. To compare between the 1981 and 2017 International League Against Epilepsy (ILAE) classification of seizure types based on an outpatient setting.Methods. We retrospectively reviewed 200 patients from our outpatient epilepsy registry. Based on clinical information, their seizure types were classified according to ILAE official reports, and differences between the 1981 and 2017 classifications were compared. All unclassifiable cases based on either one or both classification systems were discussed.Results. The 200 patients had a total of 243 manifestations. Some terms in the 2017 classification clearly correspond to those of the 1981 classification, while others lack clarity and are more controversial. The three most frequently encountered seizure types based on the 2017 classification were focal to bilateral tonic‐clonic (83; 34.1%), unknown‐onset tonic‐clonic (56; 23.0%), and focal impaired awareness (52; 21.4%). Based on the 1981 classification, the three most frequently encountered seizure types were unclassified (89; 36.6%), secondary generalized tonic‐clonic (sGTCS) (83; 34.1%), and complex partial (CPS) (36; 14.8%). Seventy‐five of 89 (84.3%) unclassified cases based on the 1981 classification were classified using the 2017 classification mainly due to the addition of the “unknown origin” category and a combination of different levels of terms (level of awareness and motor/non‐motor features). In 14 cases, seizures were unclassifiable using both classification systems; eight were rare manifestations with unclear awareness or unusual bilateral movements and six were due to a lack of detailed description.Conclusion The 2017 seizure classification greatly reduces the number of unclassifiable cases. The combination of awareness level and motor/non‐motor features introduces greater flexibility and allows for detailed seizure description. Several cases, however, remain unclassified, but these are mostly due to a lack of understanding of epilepsy. The 2017 seizure classification demonstrates a steady transition from the 1981 classification with acceptable consistency and improvements.
Probable dysimmune epilepsia partialis continua manifesting as epileptic moving toes syndrome: electroclinical features of a challenging case
Authors: Brigo Francesco , Vogrig Alberto , Bratti Arianna , Tavernelli Veronica , Nardone Raffaele , Trinka Eugen 
Published: August 2019

ABSTRACTEpilepsia partialis continua (EPC) is a rare form of focal status epilepticus. We describe a 22‐year‐old woman with EPC manifesting with isolated toe movements, prevalent over the left side and initially misdiagnosed as psychogenic, clinically almost indistinguishable from those observed in “painful legs and moving toes syndrome”. The continuous involuntary movements with EMG correlates of twitches lasting <100 ms, the sharp waves over fronto‐central regions on EEG, and the marked asymmetry in somatosensory evoked potentials with higher cortical amplitude over the right side following peripheral stimulation over the left foot confirmed the epileptic nature of the symptoms, leading to the diagnosis of EPC. The toe movements were markedly reduced following steroid therapy, whereas the infusion of immunoglobulins caused aseptic meningitis. Despite an extensive diagnostic work‐up (including a search for antibodies for paraneoplastic and autoimmune encephalitis), an ultimate aetiological diagnosis was not reached, although the dramatic response to corticosteroids strongly supported an underlying dysimmune pathophysiology. Diagnosing EPC can be challenging, especially if movements are confined to a very small body region or strongly resemble movements encountered in other conditions. EEG‐EMG monitoring should be performed in patients with continuous involuntary muscular jerks in order not to overlook a diagnosis of EPC. [Published with video sequences onwww.epilepticdisorders.com].
Genetic literacy series: genetic epilepsy with febrile seizures plus
Authors: Myers Kenneth A. , Scheffer Ingrid E. , Berkovic Samuel F. ,   on behalf of the ILAE Genetics Commission
Published: August 2019

ABSTRACTGenetic epilepsy with febrile seizures plus (GEFS+) is a familial epilepsy syndrome in which affected individuals within a family typically have a variety of epilepsy phenotypes, varying from simple febrile seizures and febrile seizures plus with a good outcome to severe epileptic encephalopathies. Here, we review the spectrum of epilepsy phenotypes, the genetic architecture of GEFS+, and the implicated genes. Using an illustrative clinical case study, we describe important steps in managing patients with GEFS+: making the diagnosis of GEFS+, appropriate genetic testing, and counselling.
Inadequate benzodiazepine dosing may result in progression to refractory and non‐convulsive status epilepticus
Authors: Rao Shishir Keekana , Mahulikar Advait , Ibrahim Mohammad , Shah Aashit , Seraji‐Bozorgzad Navid , Mohamed Wazim 
Published: August 2019

ABSTRACTAims. Status epilepticus (SE) is defined as ongoing seizures lasting longer than five minutes or multiple seizures without recovery. Benzodiazepines (BZDs) are first‐line agents for the management of SE. Our objective was to evaluate BZD dosing in SE patients and its effects on clinical/electrographic outcomes.Methods. A retrospective analysis was conducted from a prospective database of SE patients admitted to a university‐based neurocritical care unit. The initial presentation and progression to refractory SE (RSE) and non‐convulsive SE (NCSE) with coma was evaluated. Outcome measures included length of stay (LOS), rates of intubation, ventilator‐dependent days, and Glasgow outcome scale (GOS). The lorazepam equivalent (LE) dosage of BZDs administered was calculated and we analysed variations in progression if 4 mg or more of LE (adequate BZDs) was administered.Results. Among 100 patients, the median dose of LE was 3 mg (IQR: 2–5 mg). Only 31% of patients received adequate BZDs. Only 18.9% of patients with NCSE without coma received adequate BZDs (p=0.04). Among patients progressing to RSE, 75.4% had not received adequate BZDs (p=0.04) and among patients developing NCSE with coma, 80.6% did not receive adequate BZDs (p=0.07). Escalating doses of BZDs were associated with a decrease in cumulative incidences of RSE (correlation coefficient r=‐0.6; p=0.04) and NCSE with coma (correlation coefficient r=‐0.7; p=0.003). Outcome measures were not influenced by BZD dosing.Conclusion. The majority of our patients were not adequately dosed with BZDs. Inadequate BZD dosing progressed to RSE and had a tendency to lead to NCSE with coma. Our study demonstrates the need to develop a hospital‐wide protocol to guide first responders in the management of SE.