bluuebunny/crossref_metadata_2025_split huggingface dataset:
Early experiences with tachycardia‐triggered vagus nerve stimulation using the AspireSR stimulator
Authors: El Tahry Riëm , Hirsch Martin , Van Rijckevorsel Kenou , Santos Susana Ferrao , de Tourtchaninoff Marianne , Rooijakkers Herbert , Coenen Volker , Schulze‐Bonhage Andreas 
Published: August 2019

AbstractMany epilepsy patients treated with vagus nerve stimulation additionally use an “on‐demand” function, triggering an extra stimulation to terminate a seizure or diminish its severity. Nevertheless, a substantial number of patients are not able to actively trigger stimulations by use of a magnet, due to the absence of an aura or inability for voluntary actions in the early phase of a seizure. To address this need, a novel implantable pulse generator, the AspireSR VNS system, was developed to provide automated ictal stimulation triggered by a seizure‐detecting algorithm. We report our experience with three patients in assessing the functionality of ictal stimulation, illustrating the detection system in practice. Detection of ictal tachycardia and variable additional detections of physiological tachycardia depended on the individual seizure‐detecting algorithm settings.
Epilepsia partialis continua: aetiology, semiology and prognosis in a Spanish adult cohort
Authors: Bejr‐kasem Helena , Sala‐Padró Jacint , Toledo Manuel , Santamarina Estevo , Sarria Silvana , Gonzalez‐Cuevas Montserrat , Sueiras‐Gil María , Quintana Manolo , Salas‐Puig Xavier 
Published: August 2019

AbstractAim. To describe the semiological features in patients suffering with Epilepsia Partialis Continua (EPC), also referred as Kozhevnikov syndrome and their relationship with aetiology, duration, and prognosis, as well as recurrence during follow‐up.Methods. We analysed consecutive EPC patients diagnosed and followed in our centre over a seven‐and‐a half year period. We collected demographic and clinical data, along with neuroimaging and EEG recordings. All patients were followed for more than six months. Patients were categorised with single body area or multiple body area involvement according to the body parts affected. Recurrence was defined as a second EPC episode after one week.Results. We collected data from 27 adult patients; 70.4% were men, the mean age was 65.2 years old (range: 17–89 years), and 40.7% had previous epilepsy. EPC causes were structural in 85.1% (stroke being the most frequent; 44.4%), metabolic in 11.1%, and of unknown origin in 7.4%. A cortical lesion on neuroimaging was shown in 70.4%. Involvement of multiple body areas was reported in 55.6% of patients. The optimal cut‐off period to predict death was nine days (with a sensitivity of 62.5% and specificity of 75%; p=0.039), and this group of patients exhibited more multiple body area involvement (88.9% vs 38.9%; p=0.04). During follow‐up, patients with cortical lesions had more EPC relapses (p=0.037).Conclusions. The most frequent aetiology of EPC in our patients was stroke. Multiple body area involvement and duration were associated with mortality. Patients with cortical lesions had more EPC relapses during follow‐up.
Drug‐resistant parietal lobe epilepsy: clinical manifestations and surgery outcome
Authors: Asadollahi Marjan , Sperling Michael R. , Rabiei Amin H. , Asadi‐Pooya Ali A. 
Published: August 2019

AbstractAim. We reviewed a large surgical cohort to investigate the clinical manifestations, EEG and neuroimaging findings, and postoperative seizure outcome in patients with drug‐resistant parietal lobe epilepsy (PLE).Methods. All drug‐resistant PLE patients, who were investigated for epilepsy surgery at Jefferson Comprehensive Epilepsy Center between 1986 and 2015, were identified. Demographic data, seizure data, EEG recordings, brain MRI, pathological findings, and postsurgical seizure outcome were reviewed.Results. In total, 18 patients (11 males and seven females) were identified. Sixteen patients (88%) had tonic‐clonic seizures, 12 (66%) had focal seizures with impaired awareness, and 13 (72%) described auras. Among 15 patients who had brain MRI, 14 patients (93%) had parietal lobe lesions. Only three of 15 patients (20%) who had interictal scalp EEG recordings showed parietal interictal spikes. Of 12 patients with available ictal surface EEG recordings, only three patients (25%) had parietal ictal EEG onset. After a mean follow‐up duration of 8.6 years, 14 patients (77.7%) showed a favourable postoperative seizure outcome.Conclusion. In patients with PLE, semiology and EEG may be misleading and brain MRI is the most valuable tool to localize the epileptogenic zone. Postsurgical seizure outcome was favourable in our patients with drug‐resistant parietal lobe epilepsy.
Coregistration of multimodal imaging is associated with favourable two‐year seizure outcome after paediatric epilepsy surgery
Authors: Perry Michael Scott , Bailey Laurie , Freedman Daniel , Donahue David , Malik Saleem , Head Hayden , Keator Cynthia , Hernandez Angel 
Published: August 2019

AbstractAims. Multimodal coregistration uses multiple image datasets coregistered to an anatomical reference (i.e. MRI), allowing multiple studies to be viewed together. Commonly used in intractable epilepsy evaluation and generally accepted to improve localization of the epileptogenic zone, data showing that coregistration improves outcome is lacking. We compared seizure freedom following epilepsy surgery in paediatric patients, evaluated before and after the use of coregistration protocols at our centre, to determine whether this correlated with a change in outcome.Methods. We included paediatric epilepsy surgery patients with at least one anatomical and one functional neuroimaging study as part of their presurgical evaluation. Preoperatively designated palliative procedures and repeat surgeries were excluded. Multiple pre‐, peri‐, and postoperative variables were compared between groups with the primary outcome of seizure freedom.Results. In total, 115 were included with an average age of 10.63 years (0.12–20.7). All evaluations included video‐EEG (VEEG) and MRI. Seven (6%) had subtraction single‐photon emission CT (SPECT), 46 (40%) had positron emission tomography (PET), and 62 (54%) had both as part of their evaluation. Sixty (52%) had extratemporal epilepsy and 25 (22%) were MRI‐negative. Sixty‐eight (59%) had coregistration. Coregistered patients were less likely to undergo invasive EEG monitoring (p=0.045) and were more likely to have seizure freedom at one (p=0.034) and two years (p<0.001) post‐operatively. A logistic regression accounting for multiple covariates supported an association between the use of coregistration and favourable post‐surgical outcome.Conclusions. Coregistered imaging contributes to favourable postoperative seizure reduction compared to visual analysis of individual modalities. Imaging coregistration is associated with improved outcome, independent of other variables after surgery. Coregistered imaging may reduce the need for invasive EEG monitoring, likely due to improved confidence in presurgical localization. These findings support the use of multimodal coregistered imaging as part of the presurgical assessment in patients evaluated for surgical treatment of intractable epilepsy.
Intractable ictal vomiting: a new form of reflex epilepsy?
Authors: Sureshbabu Sachin , Nayak Dinesh , Aggarwal Vikash , Peter Sudhir , Khanna Laxmi , Mittal Gaurav 
Published: August 2019

AbstractIn this description of the clinical course of a young female with persistent and protracted vomiting along with occasional loss of consciousness and subtle motor manifestations, the differential diagnosis is debated. The epileptic origin of her symptoms was substantiated by the presence of interictal epileptiform discharges and dramatic response to valproate monotherapy. Possible lobar localizations are discussed with the support of existing literature on this rare ictal manifestation [Published with video sequence on www.epilepticdisorders.com].
A case of post‐leptospirosis autoimmune epilepsy presenting with sleep‐related hypermotor seizures
Authors: Makhija Prashant , Gopinath Siby , Kannoth Sudheeran , Radhakrishnan Kurupath 
Published: August 2019

ABSTRACTThis video‐illustrated case report concerns a 49‐year‐old woman who presented with sleep‐related hypermotor seizures. The antecedent history of leptospirosis, high frequency of new‐onset seizures, presence of an unclassified anti‐neuronal antibody, and dramatic response to steroids strongly supported post‐infectious immune‐mediated pathogenesis in our patient. To the best of our knowledge, post‐leptospirosis autoimmune epilepsy presenting as sleep‐related hypermotor seizures has not hitherto been reported. [Published with video sequence on www.epilepticdisorders.com].
Mowat‐Wilson syndrome presenting with fever‐associated seizures
Authors: Seo Se Eun , Kim Se Hee , Lee Seung Tae , Choi Jong Rak , Lee Joon Soo , Kim Heung Dong , Kang Hoon‐Chul 
Published: August 2019

ABSTRACTMowat‐Wilson syndrome (MWS) is a disorder caused by mutations or deletions of the zinc finger E‐box‐binding homeobox 2 (ZEB2) gene. Diagnosis of MWS can be challenging to neurologists, because its manifestations are diverse and the spectrum of genetic mutations are broad. Here, we describe two patients with MWS who initially showed atypical forms of fever‐triggered seizures during childhood. Both had characteristic facial features, cognitive impairment, and genito‐urinary anomalies consistent with MWS. By performing targeted next‐generation sequencing (NGS) using a gene panel for epilepsy, we were able to identify a nonsense mutation (c.1965C>A) in the ZEB2 gene of one patient and a frameshift mutation (c.2348dupC) in the other patient. Fever‐induced seizures can be presenting signs of MWS. MWS should be considered in the differential diagnosis of fever‐induced seizures, especially when the patient has distinctive facial features and multiple anomalies, including cardiac, genito‐urinary, and eye defects.
Early infancy‐onset stimulation‐induced myoclonic seizures in three siblings with inherited glycosylphosphatidylinositol (GPI) anchor deficiency
Authors: Mogami Yukiko , Suzuki Yasuhiro , Murakami Yoshiko , Ikeda Tae , Kimura Sadami , Yanagihara Keiko , Okamoto Nobuhiko , Kinoshita Taroh 
Published: August 2019

ABSTRACTInherited glycosylphosphatidylinositol anchor deficiency causes a variety of clinical symptoms, including epilepsy, however, little information is available regarding seizures as a symptom. We report three siblings with inherited glycosylphosphatidylinositol anchor deficiency with PIGL gene mutations. The phenotypes of the subjects were not consistent with CHIME syndrome or Mabry syndrome, as reported in previous studies. All shared some clinical manifestations, including transient apnoea as neonates, dysmorphic facial features, and intellectual disability. Between one week and 3 months of life, all patients developed myoclonic seizures. Myoclonic jerks were easily evoked by sudden unexpected acoustic or tactile stimuli. None showed elevation of serum alkaline phosphatase. Vitamin B6 was given to one of the three siblings, but failed to suppress seizures. The presence of early infancy‐onset stimulation‐induced myoclonic seizures combined with dysmorphic facial features should lead physicians to consider the possibility of inherited glycosylphosphatidylinositol anchor deficiency.
Long‐term seizure outcome following epilepsy surgery in the parietal lobe: a meta‐analysis
Authors: Pilipović‐Dragović Sanja , Ristić Aleksandar J. , Bukumirić Zoran , Trajković Goran , Sokić Dragoslav 
Published: August 2019

ABSTRACTAim. Due to a limited number of patients with drug‐resistant parietal lobe epilepsy in surgical series, there are insufficient data about long‐term seizure outcome following surgery restricted to the parietal lobe. We performed a meta‐analysis to asses long‐term outcomes in patients with parietal lobe epilepsy who underwent surgery confined to the parietal lobe or resection with major involvement of the parietal cortex.Methods. An English language literature search for studies on parietal lobe surgery and outcome was conducted using the MEDLINE database, followed by a manual search based on specific criteria. An inverse variance random effect meta‐analysis model was used to estimate the pooled proportion of Engel Class I. Meta‐regression models were used to examine the association between outcome and potential predictors.Results. The search yielded seven retrospective studies with a total sample size of 253 patients (mean follow‐up: 104.9±74.8 months). Following surgery, Engel Class I surgical outcome was achieved in 62.4% (95% CI: 0.492–0.755). Two independent predictors were identified for positive long‐term outcome: interictal EEG localized to the parietal region (p=0.007) and the presence of tumour (p=0.022).Conclusion. Following surgery confined to the parietal lobe or resection with major involvement of the parietal cortex, the long‐term prognosis of patients with parietal lobe epilepsy is favourable.
A child with hyperekplexia and epileptic myoclonus
Authors: Wadi Lara , Medlej Yasser , Obeid Makram 
Published: August 2019

ABSTRACTHyperekplexia is a rare neurogenetic disorder characterized by startle. Accurate diagnosis of this notorious mimicker of epilepsy is important to prevent life‐threatening apnoea. We report a novel case of concomitant GLRA1‐related hyperkeplexia and myoclonic epilepsy. A toddler with daily paroxysms of head drops and falls presented with epileptic myoclonus on EEG, however, whole‐exome sequencing revealed hyperekplexia‐related GLRA1 mutation. The boy eventually developed spells induced by noise and surprise. All his spells remitted upon treatment with clonazepam. Paediatricians and paediatric neurologists should be aware of this possible mixed presentation in order to appropriately tailor medication regimens and treatment goals. [Published with video sequence on www.epilepticdisorders.com].
Neonatal tremor episodes and hyperekplexia‐like presentation at onset in a child with SCN8A developmental and epileptic encephalopathy
Authors: Pons Linda , Lesca Gaëtan , Sanlaville Damien , Chatron Nicolas , Labalme Audrey , Manel Véronique , Arzimanoglou Alexis , de Bellescize Julitta , Lion‐François Laurence 
Published: August 2019

ABSTRACTSCN8A encephalopathy is a newly defined epileptic encephalopathy caused by de novo mutations of the SCN8A gene. We report herein a four‐year‐old boy presenting with severe non‐epileptic abnormal movements, of possibly antenatal onset, progressively associated with pharmacoresistant epilepsy and regression, associated with a de novo heterozygous missense mutation of SCN8A. This case shows that paroxysmal non‐epileptic episodes of severe tremor and hyperekplexia‐like startles and a striking vegetative component can be the first early symptoms of severe SCN8A developmental and epileptic encephalopathy. Clinicians should be aware of these symptoms in order to avoid misdiagnosis and ensure early appropriate therapeutic management. [Published with video sequences on www.epilepticdisorders.com].
Identifying the educational needs of physicians in pediatric epilepsy in order to improve care: results from a needs assessment in Germany, Spain, and the United States
Authors: Murray Suzanne , Labbé Sara , Kothare Sanjeev , Málaga Ignacio , Kluger Gerhard , Ogden Patti , Lazure Patrice , Arzimanoglou Alexis 
Published: August 2019

ABSTRACTAims. The objective of this study was to gather evidence‐based data on the educational needs of neuropediatricians. A needs assessment was conducted to identify the clinical challenges of physicians when diagnosing, medically treating, and managing pediatric patients with epilepsy; which could be addressed through educational interventions.Methods. A two‐phase mixed‐methods approach was used to conduct the needs assessment in Germany, Spain, and the US. Phase 1 consisted of qualitative data collection through multiple sources: a literature review, semi‐structured interviews with clinicians and nurses working in pediatric epilepsy, and interpretation and input from faculty experts. Qualitative data were coded (NVivo) and analyzed using a thematic analysis, and findings were then used to design the second phase. Phase 2 consisted of quantitative data collection through an online survey that aimed to validate the identified challenges and underlying causes using a larger sample than in Phase 1. Data from the survey were analyzed using frequency tabulations and chi‐square tests (SPSS).Results. A total of 267 participants were included in the study. Phase 1 included 88 participants (neurologists, pediatricians, neuropediatricians, and nurses). Phase 2 included 179 participants (neurologists, pediatricians, and neuropediatricians). The main areas of challenge which emerged from the triangulated data included: the integration of guidelines into practice, identification of epilepsy and epilepsy events, integration of genetic testing into practice, integration of non‐pharmacological treatments, transition from pediatric to adult care, and involvement and engagement with caregivers. Underlying causes of these challenges are reported, along with supporting qualitative findings.Conclusions. This study identified the educational needs of physicians working in pediatric epilepsy in Germany, Spain and the USA. Increasingly, educational interventions are required to be evidence‐based. The results of this study could be used to design such interventions to support neuropediatricians who wish to specialize in pediatric epileptology, in order to manage the identified challenges.
A web‐based diagnostic reference centre for the European Reference Network “EpiCare”: recommendations of the eNeuropathology working group
Authors: Kölble Konrad , Cross J. Helen , Becker Albert , Blümcke Ingmar 
Published: August 2019

ABSTRACTEpilepsy surgery is a valuable treatment strategy for a selected group of patients with drug‐resistant focal epilepsy. While reliable disease classification is essential for the optimal management of patients in general and crucial for the development of more personalized therapies in the future, arriving at a precise diagnosis often poses considerable difficulties due to the broad and variant‐rich spectrum of epilepsy‐associated brain lesions. Given the scarcity of European institutions diagnostically focusing on the histopathology of epilepsy surgery cases, the provision of subspecialty expertise as well as training opportunities remains logistically and financially challenging. To improve this situation, the European Reference Network's (ERN) epilepsy care program (EpiCare, http://epi-care.eu) has set out to develop a web‐based microscopy referral and teaching framework. This paper reviews the aspects of digital microscopy, data storage, and image analysis technology relevant to the practice of neuropathology. Cognizant of the European data security requirements and regulations, we propose a collaborative, diagnostic network initiative (the eNeuropathology reference centre) and delineate a roadmap for its implementation favouring open‐source, vendor‐independent browser platforms.
De novo absence status epilepticus in three paediatric patients: a new idiopathic epilepsy syndrome?
Authors: Caraballo Roberto H. , Chacón Santiago , Fasulo Lorena , Bedoya Claudio 
Published: August 2019

ABSTRACTAim. Absence status epilepticus (ASE) is a prolonged generalized absence seizure that usually lasts for hours and can even last for days. The main symptom is the altered content of consciousness while the patient may be alert and partly responsive.Methods. We describe the electroclinical features, treatment, and evolution of three paediatric patients with de novo ASE with an excellent response to valproic acid (VPA).Results. Three paediatric patients presented with non‐convulsive status epilepticus and an acute confusional state with impaired consciousness and EEG abnormalities compatible with typical ASE, associated with generalized spike‐and‐wave paroxysms at 2.5‐4 Hz, as the first epileptic manifestation at eight, three, and nine years of age, respectively. No significant personal and/or family history was reported. None of the patients had absence seizures or any other type of seizure before the occurrence of the ASE. All of them responded well to VPA and had a benign disease course. Neuroradiological imaging was normal in all patients. These three cases presented with ASE as the first manifestation of their epilepsy; none of them had any other type of seizure before the event or during their follow‐up, which was long‐term in one. All patients had an excellent response to VPA.Conclusions. Our three cases presented with generalized typical ASE as the first manifestation of their epilepsy. De novo ASE might be considered as a well‐defined idiopathic epilepsy syndrome or a variant of an idiopathic generalized epilepsy syndrome, such as a particular type or variant of childhood absence epilepsy.
Effect of bromfenac on free radical oxidation in model systems
Authors: Galimova E F , Khaibullina Z G , Enikeev D A , Bortsova Yu L , Mochalov K S , Galimova S Sh , Travnikov O Yu , Asadullina T S , Aver’yanova K S 
Published: August 2019

Aim. The study of free radical oxidation processes in an experiment on model systems using the anti-inflammatory drug bromfenac (nakwan) widely used in ophthalmology for the treatment of infectious and inflammatory diseases of the anterior chamber of the eye.
Methods. The antioxidant capacity of the drug was evaluated by chemiluminescence registration and analysis of model systems that generate reactive oxygen species and reproduce lipid peroxidation processes using the chemiluminomer CL-003. The following parameters of spontaneous and induced chemiluminescence were determined: light sum and maximum luminescence amplitude, duration of latent period, amplitude of fast and slow flash.
Results. When tested in vitro in two different model systems, a high antioxidant activity of the studied drug was established, up to complete suppression of chemiluminescence when 90 μg of bromfenac was added to the incubation medium, which characterizes the inhibition of the generation of reactive oxygen species. A significant increase in total antioxidant activity with bromfenac was also demonstrated, which is reflected by the integral parameter of chemiluminescence — light sum which decreased with the introduction of 10 μg of the drug by 1.2 times, and with 90 μg by 1.5 times. A comparative analysis of the antioxidant properties of various nonsteroidal anti-inflammatory drugs used in ophthalmic practice demonstrated a more pronounced efficacy of bromfenac compared to ketorolac, the use of which was not accompanied by statistically significant changes in chemiluminescence. A very important mechanism of the positive effect of bromfenac is the direct dependence of the action on its quantity in the reaction medium, which opens up prospects for the controlled correction of free radical phenomena and the excessive activation of lipid peroxidation in the imbalance of the pro- and antioxidant processes in biological systems.
Conclusion. It is suggested that the protective effects of the drug in various infectious-inflammatory lesions of the eye can be determined, along with previously known properties, its antioxidant activity, restriction of increased production of reactive oxygen species and oxidative stress phenomena.