bluuebunny/crossref_metadata_2025_split huggingface dataset:
Alpha Neurofeedback Has a Positive Effect for Participants Who Are Unable to Sustain Their Alpha Activity
Authors: Biswas Ankan , Ray Supratim 
Published: August 2019

AbstractAlpha rhythm (8–13 Hz) is linked to relaxed mental state in humans. Earlier reports have shown that individuals can increase their alpha power if provided with a valid feedback, compared to controls who are provided invalid feedback. However, these results remain controversial, partly because controls may be in a different behavioral state, making it difficult to directly compare their alpha power with the valid group. We here address this issue by using an experimental paradigm in which an invalid feedback is given on a fraction of trials, such that both valid and invalid conditions can be obtained from the same participant. Using electroencephalography (EEG), we recorded alpha power from the occipital area from 24 humans (nine females) and played a feedback tone which could be valid (tone frequency proportional to alpha power), invalid (tone sequence from a previous valid trial; participants were unaware of this condition), or neutral (constant tone frequency). We found that during eyes closed-state, neurofeedback did not enhance alpha activity beyond pre-trained state within the experimental duration, probably because of saturation of alpha rhythmicity. However, for participants whose alpha power decreased over time within a trial, valid feedback helped them to sustain alpha more than invalid feedback. Further, alpha increase showed a weak negative correlation with their self-reported attentional load but was uncorrelated with relaxation levels. Our results reconcile many conflicting reports in the neurofeedback literature, and show that even under most stringent control, valid neurofeedback can help participants who are otherwise unable to sustain their alpha activity.
Malformations of cortical development and epilepsies: neuropathological findings with emphasis on focal cortical dysplasia
Authors: Blümcke Ingmar , Vinters Harry V. , Armstrong Dawna , Aronica Eleonora , Thom Maria , Spreafico Roberto 
Published: August 2019

ABSTRACTStructural brain abnormalities can be increasingly recognized in patients suffering from intractable focal epilepsies using high‐resolution imaging techniques. Epilepsy surgery has become a successful treatment option for many of these patients. A broad spectrum of malformations of cortical development (MCD) can be histopathologically identified in resective surgical brain samples. Here, we discuss neuropathological findings and available classification systems in children and adult patients. Particular emphasis will be paid to the classification system for focal cortical dysplasias (FCD), which can be histopathologically distinguished as type I and II. Also mild forms of cortical malformations (mMCD) may be present, including heterotopic neurons in white matter location. However, different cohorts of epilepsy patients may present with similar histopathological findings and clinico‐pathological correlations are not always comparable with respect to outcome prediction. We will, therefore, discuss also the difficulties to classify some FCD variants. Notwithstanding, the underlying pathomechanisms in all FCD entities need to be specified. A comprehensive approach taking all currently available data into consideration will be mandatory to further develop our current understanding of FCDs, and to continuously improve our concept for a reliable classification system.
Basic mechanisms of MCD in animal models
Authors: Battaglia Giorgio , Becker Albert J. , LoTurco Joseph , Represa Alfonso , Baraban Scott C. , Roper Steven N. , Vezzani Annamaria 
Published: August 2019

ABSTRACTEpilepsy‐associated glioneuronal malformations (malformations of cortical development [MCD]) include focal cortical dysplasias (FCD) and highly differentiated glioneuronal tumors, most frequently gangliogliomas. The neuropathological findings are variable but suggest aberrant proliferation, migration, and differentiation of neural precursor cells as essential pathogenetic elements. Recent advances in animal models for MCDs allow new insights in the molecular pathogenesis of these epilepsy‐associated lesions. Novel approaches, presented here, comprise RNA interference strategies to generate and study experimental models of subcortical band heterotopia and study functional aspects of aberrantly shaped and positioned neurons. Exciting analyses address impaired NMDA receptor expression in FCD animal models compared to human FCDs and excitatory imbalances in MCD animal models such as lissencephaly gene ablated mice as well as in utero irradiated rats. An improved understanding of relevant pathomechanisms will advance the development of targeted treatment strategies for epilepsy‐associated malformations.
Electroencephalographic changes in pyridoxine‐dependant epilepsy: new observations
Authors: Naasan Georges , Yabroudi Mohamad , Rahi Amal , Mikati Mohamad A. 
Published: August 2019

ABSTRACTObjectivePyridoxine‐dependent epilepsy (PDE) is a rare disease, of which the EEG manifestations are only partially characterised. We report our observations of EEG recordings in four patients with PDE.Materials and methodsEEG tracings from four patients fulfilling the clinical criteria for PDE were reviewed. Relative to the time of treatment with pyridoxine, EEG recordings were available before treatment in two patients (at ages four and 10 months), immediately after treatment in two patients and during long‐term follow‐up with treatment in all four patients.ResultsPre‐pyridoxine interictal EEG findings included: diffuse slowing, bilateral independent multifocal epileptiform discharges, generalized bursts of polyspike slow waves and focal or generalized sharp waves. In addition, the EEG was often asymmetrical and included: generalized semi‐rhythmic sharp and slow waves, a burst suppression pattern and continuous generalized spike and slow waves. In one patient, who was followed subsequently, a decrease in multifocal spikes and sharp waves and permanent cessation of clinical seizures, within 10 minutes of concurrent reduction of spikes in the pre‐existing generalized spike slow wave pattern, was observed immediately after pyridoxine treatment. However, despite the clinical response in this patient we observed persistent generalized burst suppression for four days, and fluctuation of the EEG with diffuse slowing on day four and transient exacerbation of discharges with continuous spike slow waves on day 22. This was followed by intermittent sharp waves at eight and 20 months, mild slowing at 31 months and normal EEG at 43 months. Long‐term EEG findings in the other three patients receiving pyridoxine ranged between normal and intermittent multifocal sharp waves.ConclusionOur data confirm previous observations and provide the following new findings: (1) the presence of burst suppression pattern after cessation of seizures can occur for up to five days after initiation of pyridoxine and should not exclude the diagnosis of PDE, (2) possible fluctuation and even transient worsening of electrographic discharges were observed for up to three weeks after initiation of pyridoxine and (3) the abnormal EEG can persist for up to 43 months before normalizing (range 1‐43 months) and in other cases in which it continues to be abnormal it may still improve after increasing the dose of pyridoxine.
Zonisamide in West syndrome: an open label study
Authors: Yum Mi‐Sun , Ko Tae‐Sung 
Published: August 2019

ABSTRACTAimsInfantile spasms are usually resistant to conventional anti‐epileptic drugs. Although adrenocorticotropic hormones or vigabatrin are regarded as standard agents for the treatment of infantile spasms, there are still limitations of their use. We determined the efficacy and tolerability of high‐dose zonisamide in patients with recent‐onset infantile spasms.MethodsSeventeen patients with infantile spasms, who were admitted to our hospital between October 2005 and November 2007, were eligible for enrolment within two months of diagnosis. Zonisamide was administered at a starting dose of 2‐8 mg/kg/day, increasing by 2‐5 mg/kg/day every three to four days until the seizures disappeared or the dose reached 30 mg/kg/day. Complete response was defined as clinical cessation of infantile spasms over 28 consecutive days and disappearance of hypsarrhythmia by EEG analysis.ResultsOf the 17 treated patients (nine who received initial monotherapy and eight add‐on therapy), five of 12 (42.0%) cryptogenic patients and two of five (40.0%) symptomatic patients showed complete disappearance of spasms. The maximum daily dose was 10‐28 mg/kg, and the effective daily dose was 10‐22 mg/kg. Mean time period before disappearance of spasms and hypsarrhythmia was eight days. Seizure recurrence was observed in three of the seven patients who showed complete disappearance of spasms. Adverse effects included irritability in four patients and poor oral intake in two patients.ConclusionAlthough further study of zonisamide is warranted, high‐dose zonisamide can be effective and safe in some infants with newly diagnosed West syndrome.
Myoclonic status epilepticus in juvenile myoclonic epilepsy
Authors: Larch Julia , Unterberger Iris , Bauer Gerhard , Reichsoellner Johannes , Kuchukhidze Giorgi , Trinka Eugen 
Published: August 2019

ABSTRACTBackgroundMyoclonic status epilepticus (MSE) is rarely found in juvenile myoclonic epilepsy (JME) and its clinical features are not well described. We aimed to analyze MSE incidence, precipitating factors and clinical course by studying patients with JME from a large outpatient epilepsy clinic.MethodsWe retrospectively screened all patients with JME treated at the Department of Neurology, Medical University of Innsbruck, Austria between 1970 and 2007 for a history of MSE. We analyzed age, sex, age at seizure onset, seizure types, EEG, MRI/CT findings and response to antiepileptic drugs.ResultsSeven patients (five women, two men; median age at time of MSE 31 years; range 17‐73) with MSE out of a total of 247 patients with JME were identified. The median follow‐up time was seven years (range 0‐35), the incidence was 3.2/1,000 patient years. Median duration of epilepsy before MSE was 26 years (range 10‐58). We identified three subtypes: 1) MSE with myoclonic seizures only in two patients, 2) MSE with generalized tonic clonic seizures in three, and 3) generalized tonic clonic seizures with myoclonic absence status in two patients. All patients responded promptly to benzodiazepines. One patient had repeated episodes of MSE. Precipitating events were identified in all but one patient. Drug withdrawal was identified in four patients, one of whom had additional sleep deprivation and alcohol intake. Two patients received inappropriate treatment (carbamazepine, phenytoin).ConclusionsMSE is a rare event in JME. Precipitating factors are commonly identified and for such cases the treatment response and outcome are excellent, in contrast to other cases with unknown causes.
Unexpected gamma glutamyltransferase rise increase during levetiracetam monotherapy
Authors: Broli Marcella , Provini Federica , Naldi Ilaria , Bisulli Francesca , Sama Claudia , Baruzzi Agostino , Tinuper Paolo , Riva Roberto 
Published: August 2019

ABSTRACTLevetiracetam is an antiepileptic drug (AED) with a favourable tolerability profile with little or no effect on liver function. We describe an epileptic patient who developed a significant increase in gamma glutamyltransferase (γGT) while on levetiracetam monotherapy.
Epidural and foramen‐ovale electrodes in the diagnostic evaluation of patients considered for epilepsy surgery
Authors: Beleza Pedro , Rémi Jan , Feddersen Berend , Peraud Aurelia , Noachtar Soheyl 
Published: August 2019

ABSTRACTPurposeTo evaluate the clinical utility of epidural and foramen‐ovale recordings and associated morbidity in the pre‐surgical evaluation of epilepsy.MethodsWe retrospectively analysed 59 epilepsy patients, who underwent recordings with epidural (n = 59) and foramen‐ovale electrodes (n = 46) as part of their pre‐surgical evaluation between 1990‐1999. The epidural and foramen‐ovale evaluation was based on the results of the non‐invasive EEG‐video recordings in patients, in whom non‐invasive evaluation failed to localise seizure onset (75%, 44 patients) or where EEG, and imaging studies were discrepant (25%, 15 patients) but allowed a testable hypothesis on the seizure onset zone.ResultsMost patients (n = 57) were evaluated between 1990‐1994. Only two patients were evaluated later. The results of the epidural (n = 559) and foramen‐ovale (n = 83) electrode recordings allowed us to proceed to resective epilepsy surgery in 31% (n = 18) and to exclude further invasive evaluation in 15% (n = 9) of the patients. In 49% (n = 29) of the patients the results guided further invasive recordings using subdural and/or depth electrodes. For only three patients no additional information was gained by the electrode recordings. Temporary morbidity included local infection (epidural; n = 1) and facial pain (foramen ovale; n = 1) but no permanent complication occurred.DiscussionEpidural and foramen‐ovale electrodes have almost been abandoned in recent years, most likely because of the improvement of neuroimaging techniques such as MRI, PET and ictal SPECT. However, in selected patients, epidural electrodes and foramen‐ovale electrodes are either useful as a measure to avoid invasive evaluation or serve to guide invasive evaluation.
Paroxysmal tonic eye deviation: an atypical presentation of hypothalamic hamartoma
Authors: Olivieri Ivana , Teutonico Federica , Orcesi Simona , Papalia Grazia , Uggetti Carla , Bastianello Stefano , Balottin Umberto , Veggiotti Pierangelo 
Published: August 2019

ABSTRACTHypothalamic hamartoma is a rare developmental nonneoplastic malformation, often characterised by early onset gelastic seizures and later progressive cognitive and behavioural deterioration. In this case study, we have examined a child who presented with an atypical onset of benign paroxysmal gaze deviation between two to three months of age. The patient subsequently developed gelastic seizures at age 13. Based on the observation that hypothalamic hamartomas do not involve any functional region involved in eye motility, we speculate that both gaze deviation and gelastic seizures are a manifestation of the epileptogenic nature of the hypothalamic hamartoma. [Published with video sequences]
Detection of microchromosomal aberrations in refractory epilepsy: a pilot study
Authors: McMahon Jacinta M. , Scheffer Ingrid E. , Nicholl Jillian K. , Waters Wendy , Eyre Helen , Hinton Lyn , Nelson Paul , Yu Sui , Dibbens Leanne M. , Berkovic Samuel F. , Mulley John C. 
Published: August 2019

ABSTRACTSeizures often occur in patients with microchromosomal aberrations responsible for moderate to severe intellectual disability. We hypothesised that epilepsy alone could be caused by microdeletions or microduplications, which might also relate to epilepsy refractory to medication. Chromosomes from 20 subjects with epilepsy and repeated failure of antiepileptic medication were examined using molecular methods. Firstly, the 41 subtelomeric regions were scanned using fluorescence in situ hybridization and multiplex ligation‐dependent probe amplification. Secondly, a genome‐wide scan was carried out using oligonucleotide‐array comparative genome hybridisation on two platforms: Nimblegen and Agilent. Two aberrations (2/20) were identified: a recurrent microdeletion at 15q13.3 previously characterised in patients with seizures that generally respond to medication, and a novel 1.15 Mb microchromosomal duplication at 10q21.2 also present in the unaffected mother. We conclude that gene content of microchromosomal aberrations is not a major cause of refractory seizures, but that microchromosomal anomalies are found in an appreciable fraction of such cases.
Treatment of malignant migrating partial epilepsy of infancy with rufinamide: report of five cases
Authors: Vendrame Martina , Poduri Annapurna , Loddenkemper Tobias , Kluger Gerhard , Coppola Giangennaro , Kothare Sanjeev V. 
Published: August 2019

ABSTRACTThe syndrome of malignant migrating partial seizures of infancy (MMPEI) is characterized by early onset of multiple seizures types, highly pharmaco‐resistant seizures, and overall poor prognosis. In this study, we investigated retrospectively the efficacy of rufinamide (RUF) in five infants with MMPEI, by examining seizure type and frequency, EEG features and antiepileptic drug use. The data demonstrate that two of the five patients showed good efficacy and tolerability for RUF with a >50% reduction in seizure frequency. The use of RUF for the treatment of MMPEI may therefore provide some hope for such infants and should be further validated.[Published with video sequences]
Facio‐mandibular myoclonus: a rare cause of nocturnal tongue biting
Authors: Seneviratne Udaya 
Published: August 2019

ABSTRACTFacio‐mandibular myoclonus is a parasomnia characterized by forceful myoclonus of masticatory muscles in sleep. This condition typically presents with recurrent nocturnal tongue biting, which can be misdiagnosed for nocturnal seizures. The use of surface electromyographic channels over the facial muscles during video‐EEG monitoring is helpful in confirming the diagnosis based on a typical burst pattern. This case report highlights difficulties in the diagnosis of facio‐mandibular myoclonus and useful features which differentiate it from similar conditions.
Electroclinical features of benign infantile seizures with mild gastroenteritis
Authors: Saadeldin Imad Yassin 
Published: August 2019

ABSTRACTThe purpose of this study was to analyze the electroclinical features of patients with benign infantile seizures with mild gastroenteritis and demonstrate the benign nature of this entity. From 30 patients who were included in the study, five were excluded (two with developmental delay, one with microcephaly and two lost during follow‐up). Twenty‐five patients who fulfilled the diagnostic criteria for benign infantile seizures with mild gastroenteritis were assessed and followed at the Pediatric Department, Armed Forces Hospital, Southern Region, Khamis Mushayt, Saudi Arabia, between January 2004 and January 2009. The median age at presentation was 10.4 months. Of the infants, 14 were females and 11 were males. Seizures were focal tonic or clonic in eight (32%) patients, focal with secondary generalisation in three (12%), generalised tonic‐clonic in nine (36%) and consisted of staring only with no motor components in five (20%). Interictal electroencephalograms and brain imaging were normal for all patients. No patient required treatment with antiepileptic drugs. All the patients were found to have normal psychomotor development and neurological examination after a follow‐up period of between 15 and 56 months. The limitations of this study are the relatively small sample size, relatively short study period and the fact that the study was conducted in a tertiary referral hospital. The prevalence of this entity may be more common at the level of primary health centres. Increasing the awareness of clinicians regarding the existence of this syndrome and its benign nature in children will limit unnecessary investigations.[Published with video sequences]
Rufinamide from clinical trials to clinical practice in the United States and Europe
Authors: Resnick Trevor , Arzimanoglou Alexis , Brown Lawrence W. , Flamini Robert , Kerr Michael , Kluger Gerhard , Kothare Sanjeev , Philip Sunny , Harrison Miranda , Narurkar Milind 
Published: August 2019

ABSTRACTRufinamide is a triazole derivative structurally unrelated to other antiepileptic drugs that is indicated for the adjunctive treatment of seizures associated with Lennox‐Gastaut syndrome (LGS) in patients aged ≥4 years. Originally granted orphan drug status, marketing authorisation was obtained on the basis of a randomised, double‐blind, placebo‐controlled trial conducted in 138 LGS patients. An open‐label extension study subsequently demonstrated that rufinamide's efficacy and tolerability were maintained over the longer term (median duration of treatment, 432 days). Recently published reports from Europe and the United States have described the use of adjunctive rufinamide to treat LGS in clinical practice. These data complement the clinical trial results, by providing information on the efficacy and tolerability of rufinamide when used on an individualised basis in real‐world practice, under less tightly restricted conditions in terms of patient population and dosing strategies. A comparison of the data reveals that a “lower and slower” dosing strategy tends to be adopted in clinical practice, in comparison with the clinical trial, which does not appear to compromise efficacy, but may provide improvements in tolerability. Individual case reports provide additional valuable information on how rufinamide is being used to treat different seizure types associated with LGS. Since clinical experience with rufinamide is currently at an early stage, there are still unanswered questions relating to its use, and it is likely that its place in the adjunctive treatment of LGS will evolve as further data emerge.
Complex partial status epilepticus: an unusual presentation
Authors: Praharaj Samir Kumar , Sarkhel Sujit , Paul Imon , Tripathi Anshuman , Katshu Mohammad Zia‐ul‐Haq , Sinha Vinod Kumar 
Published: August 2019

ABSTRACTNonconvulsive status epilepticus (NCSE) is not uncommon, especially in the elderly population. It can occasionally present as confusion, personality change, coma, subtle motor activity and psychosis, thus causing diagnostic difficulties. We report an unusual case of NCSE which presented as psychosis‐like episodes at regular intervals. The confirmation of diagnosis was aided by serial electroencephalography.
Fronto‐polar epilepsy masquerading as juvenile myoclonic epilepsy
Authors: Keezer Mark R. , Saint‐Hilaire Jean‐Marc , Nguyen Dang Khoa 
Published: August 2019

ABSTRACTA woman diagnosed with juvenile myoclonic epilepsy for over 30 years presented with stereotyped episodes of abnormal sensations in both arms. Continuous video‐EEG monitoring for 14 days and MRI brain revealed that the patient's somatosensory events with associated postictal aphasia, as well as her myoclonic and generalised tonic‐clonic seizures, were likely due to a symptomatic left fronto‐polar epilepsy. Our case emphasizes the need for clinicians to consider fronto‐polar epilepsy as a potential cause of myoclonic seizures, particularly when associated with other semiologic features suggestive of frontal lobe epilepsy. [Published with video sequences]
Epileptic seizure induced by fennel essential oil
Authors: Skalli Souad , Bencheikh Rachida Soulaymani 
Published: August 2019

ABSTRACTAn epileptic seizure is reported in a 38‐year‐old woman, known to be an epileptic patient. Although she was under antiepileptic treatment and had well‐controlled epilepsy, she developed a typical generalised tonic‐clonic seizure and remained unconscious for 45 minutes following ingestion of a number of cakes containing an unknown quantity of fennel essential oil. Involuntary diarrhoea accompanied her epileptic seizure. This reported case recalls the fact that fennel essential oil can induce seizures and that this oil should probably be avoided by patients with epilepsy. Labelling of products with fennel essential oil should refer to the risk of seizures, particularly for patients with epilepsy. An awareness programme should involve all stakeholders affected by this issue.