bluuebunny/crossref_metadata_2025_split huggingface dataset:
“Paradoxical” EEG response to propofol may differentiate post‐cardiac arrest non‐convulsive status epilepticus from diffuse irreversible cerebral anoxia
Authors: Koutroumanidis Michalis , Sakellariou Dimitris , Tsirka Vasiliki 
Published: August 2019

Current EEG criteria for the diagnosis of non‐convulsive status epilepticus in critically ill patients with repetitive generalised or focal epileptiform discharges primarily rely on a widely accepted low cut‐off frequency limit of 2.5–3 Hz for non‐evolving patterns, or on discharge evolution of frequency, location or mophology. The secondary criterion is a significant clinical or EEG improvement following acute administration of a rapidly acting antiepileptic drug, such as lorazepam.We describe a comatose patient after out‐of‐hospital cardiac arrest, in whom very slow (1‐Hz), non‐evolving generalised periodic epileptiform discharges against an almost completely depressed background would suggest substantial anoxic damage and poor neurological outcome. Yet, reloading with propofol for diagnostic purposes completely dispersed generalised periodic epileptiform discharges and revealed previously absent biological activity, raising the possibility of non‐convulsive status epilepticus that was subsequently confirmed. Brain MRI showed no significant anoxic brain damage and EEG improved, but the patient died from severe cardiopulmonary complications. These observations suggest that in rare cases, slow, non‐evolving generalised periodic epileptiform discharges may reflect non‐convulsive status epilepticus rather than diffuse irreversible cerebral anoxia, while reloading with propofol can be used as an additional secondary diagnostic criterion.
Concept of epilepsy surgery and presurgical evaluation
Authors: Rathore Chaturbhuj , Radhakrishnan Kurupath 
Published: August 2019

AbstractEpilepsy surgery is a well‐accepted treatment for drug‐resistant epilepsy. The success of the epilepsy surgery depends upon an appropriate presurgical evaluation process which should ensure the selection of suitable patients who are likely to become seizure‐free following surgery without any unacceptable deficit. The two basic goals of the presurgical evaluation are the accurate localization and delineation of the extent of the epileptogenic zone, and its complete and safe resection. The process of the presurgical evaluation requires a multimodality approach wherein each modality provides unique and complimentary information which is combined with the information provided by other modalities to generate a hypothesis with regard to the likely epileptogenic zone. The basic modalities for the presurgical evaluation are clinical history, long‐term video‐EEG recording, high‐resolution MRI, and neuropsychological evaluation. The additional modalities include functional imaging studies, electrical and magnetic source imaging, functional MRI, and intracranial monitoring. Each modality has its own limitations and the information provided by none of them is absolute. Hence, a concordance among the different modalities is the key to surgical success. The presurgical evaluation is a step‐wise process starting form the most basic and most reliable tests and progressing to more complex and invasive modalities. The number of tests required varies according to the complexity involved and may include very basic minimum investigations in a given case, to the use of all the available investigations in more complex cases. The proper selection of various investigations and their accurate interpretation at each stage is required to ensure a successful outcome. In this article, we intend to review some of these basic concepts of presurgical evaluation and epilepsy surgery, and try to provide a frame work of the presurgical evaluation process.
Efficacy and safety of lacosamide as an adjunctive therapy for refractory focal epilepsy in paediatric patients: a retrospective single‐centre study
Authors: Toupin Jean‐François , Lortie Anne , Major Philippe , Diadori Paola , Vanasse Michel , Rossignol Elsa , D'Anjou Guy , Perreault Sebastien , Larbrisseau Albert , Carmant Lionel , Birca Ala 
Published: August 2019

AbstractAim. Lacosamide is an antiepileptic drug approved for the treatment of focal epilepsy in adult patients. The aim of this observational study was to review our centre's experience with lacosamide and to characterize its effectiveness and tolerability as an adjunctive antiepileptic drug in a retrospective cohort of children with refractory focal epilepsy.Methods. We retrospectively reviewed the medical records of 22 patients who received lacosamide from November 2009 to April 2014 at the CHU Ste‐Justine, University of Montreal. Treatment responders were defined as children with a ≥50% reduction in seizure frequency compared to baseline, and this was determined three months after the initiation of treatment and at the last follow‐up visit.Results. We included 14 boys and eight girls with a mean age of 12.9 years (SD: 5.2; range: 5.2–20.7 years) at the initiation of treatment. The average length of follow‐up was 11.9 months. Patients had previously received an average of 7.5 antiepileptic drugs. The mean number of concomitant antiepileptic drugs was 2.3. The mean initial and maintenance doses were 2.9 and 8.4 mg/kg/d, respectively. Thirteen (59%) and ten (45%) patients were responders after three months of treatment and at the last follow‐up visit, respectively. One became seizure‐free. Adverse effects were reported in 11 patients and none were severe. Responders and non‐responders were identical with respect to all studied parameters except gender, with the proportion of responders being greater in girls than in boys (75% vs 29%; p=0.035).Conclusion. Our study adds evidence that lacosamide appears to be a safe and effective adjunctive therapy for children with refractory focal epilepsy.
The history of progressive myoclonus epilepsies
Authors: Genton Pierre , Striano Pasquale , Minassian Berge A. 
Published: August 2019

AbstractThe history of the progressive myoclonus epilepsies (PMEs) spans more than a century. However, the recent history of PMEs begins with a consensus statement published in the wake of the Marseille PME workshop in 1989 (Marseille Consensus Group, ). This consensus helped define the various types of PME known at the time and set the agenda for a new era of genetic research which soon lead to the discovery of many PME genes. Prior to the Marseille meeting, and before the molecular era, there had been much confusion and controversy. Because investigators had but limited and biased experience with these rare disorders due to the uneven, skewed distribution of PMEs around the world, opinions and nosologies were based on local expertise which did not match well with the experiences of other researchers and clinicians. The three major areas of focus included: (1) the nature and limits of the concept of PME in varying scopes, which was greatly debated; (2) the description of discrete clinical entities by clinicians; and (3) the description of markers (pathological, biological, neurophysiological, etc.) which could lead to a precise diagnosis of a given PME type, with, in the best cases, a reliable correlation with clinical findings. In this article, we shall also examine the breakthroughs achieved in the wake of the 1989 Marseille meeting and recent history in the field, following the identification of several PME genes. As in other domains, the molecular and genetic approach has challenged some established concepts and has led to the description of new PME types. However, as may already be noted, this approach has also confirmed the existence of the major, established types of PME, which can now be considered as true diseases.
Long‐term follow‐up of two siblings with adult‐onset neuronal ceroid lipofuscinosis, Kufs type A
Authors: Özkara Çiğdem , Gündüz Ayşegül , Coşkun Tülin , Alpaslan Bengi Gül , Zeydan Burcu , Delil Şakir , Muona Mikko , Lehesjoki Anna‐Elina , Kızıltan Meral E. 
Published: August 2019

AbstractAim. Reports on the clinical presentation of adult‐onset neuronal ceroid lipofuscinoses (NCL) are scarce compared to infantile‐ and childhood‐onset forms. Here, we aimed to present detailed temporal evolution of clinical and electrophysiological features of two siblings with adult‐onset NCL and homozygous mutation in the CLN6 gene.Methods. We retrospectively analysed medical records and electrophysiological data in order to delineate evolution of clinical and electrophysiological findings. Electrophysiological studies included routine EEG and video‐EEG, as well as polymyographic analysis of myoclonus and brainstem reflex studies.Results. Both patients had seizures and cerebellar signs. Despite the slow progression of ataxia, they developed no mental deterioration, but had severe obsessive compulsive disorder and depression. EEG revealed frequent generalized spikes, polyspikes, and waves, prominent on awakening and during photic stimulation without significant change throughout the clinical course. Abnormalities concerning the blink reflex, auditory startle response, and startle response to somatosensory inputs manifested within four years. The patients underwent transient and mild improvement with valproate, whereas ataxia and seizures were dramatically ameliorated following high‐dose piracetam.Conclusions. Patients with adult‐onset NCL may present with slowly progressive ataxia, persistent photosensitivity, and seizures without dementia or extrapyramidal findings. Brainstem abnormalities become more evident with time, in line with ataxia. Piracetam is effective for both seizures and ataxia.
Stereotactic bilateral transfrontal minimal radiofrequency thermocoagulation of the amygdalohippocampal complex for bilateral medial temporal lobe epilepsy: a retrospective study of 12 patients
Authors: Zhao Quanjun , Shi Tiejun , Cui Shaojie , Wu Zhaohui , Wang Wei , Jia Yunfeng , Tian Zengmin , Wang Fuli , Yin Feng , Zhao Hulin , Xiao Xia , Wang Haiying , Cai Changlan , Luo Huimin 
Published: August 2019

AbstractBackground. Some patients with temporal lobe epilepsy have bilateral discharges and a few have bilateral medial temporal sclerosis. Stereotactic bilateral radiofrequency thermocoagulation (RFTC) of the amygdalohippocampal complex can terminate seizures or reduce seizure severity in patients with bilateral medial temporal lobe epilepsy (BMTLE).Aim. To explore the safety and efficacy of bilateral transfrontal minimal RFTC of the amygdalohippocampal complex for the treatment of BMTLE.Methods. A total of 12 BMTLE patients were treated with bilateral transfrontal minimal RFTC of the amygdalohippocampal complex under limited coagulations. The volumes of coagulated lesions were less than 0.6 cm3 Clinical outcomes were evaluated using Engel's classification, the Liverpool Seizure Severity Scale (LSSS) 2.0, Wechsler Adult Intelligence Scale‐Revised (WAIS‐R), and Wechsler Memory Scale‐Revised (WMS‐R). Quality of life (QOL) was evaluated using the 36‐item Short Form Health Survey (SF‐36).Results. Of the 12 patients, five (42%) were assessed as Engel Class I during 12–62 months of follow‐up. LSSS scores declined sharply compared with the baseline of patients not in the seizure‐free category. Functions of memory and intelligence declined transiently without statistical significance (p>0.05) immediately after surgery, but improved significantly (p<0.05) six months later. The qualities of life improved except vitality.Conclusion. Bilateral transfrontal minimal RFTC of the amygdalohippocampal complex may terminate seizures or reduce seizure severity in patients with BMTLE. Under limited coagulations, neuropsychological function was not affected but improved along with seizure control.
Sensitive quantitative detection of somatic mosaic mutation in “double cortex” syndrome
Authors: Damiano John A. , Do Hongdo , Ozturk Ezgi , Burgess Rosemary , Kalnins Renate , Jones Nigel C. , Dobrovic Alexander , Berkovic Samuel F. , Hildebrand Michael S. 
Published: August 2019

ABSTRACTAims. Somatic mutation of the lissencephaly‐1 gene is a cause of subcortical band heterotopia (“double cortex”). The severity of the phenotype depends on the level of mutation in brain tissue. Detecting and quantifying low‐level somatic mosaic mutations is challenging. Here, we utilized droplet digital PCR, a sensitive method to detect low‐level mutation.Methods. Droplet digital PCR was used in concert with classic genotyping techniques (SNaPshot assays and pyrosequencing) to detect and characterize the tissue mosaicism of a somatic mutation (LIS1 c.190A>T; p.K64X) in a patient with posterior bilateral SBH and refractory epilepsy.Results. The high sensitivity of droplet digital PCR and the ability to target individual DNA molecules allowed us to detect the mutation at low level in the brain, despite the low quality of the DNA sample derived from formalin‐fixed paraffin‐embedded tissue. This low mutation frequency in the brain was consistent with the relatively subtle malformation resolved by magnetic resonance imaging. The presence of the mutation in other tissues from the patient permitted us to predict the timing of mutagenesis.Conclusion. This sensitive methodology will have utility for a variety of other brain malformation syndromes associated with epilepsy for which historical pathological specimens are available and specific somatic mosaic mutations are predicted.
Hemimegalencephaly with Bannayan‐Riley‐Ruvalcaba syndrome
Authors: Ghusayni Ryan , Sachdev Monisha , Gallentine William , Mikati Mohamad A , McDonald Marie T 
Published: August 2019

ABSTRACTHemimegalencephaly is known to occur in Proteus syndrome, but has not been reported, to our knowledge, in the other PTEN mutation‐related syndrome of Bannayan‐Riley‐Ruvalcaba. Here, we report a patient with Bannayan‐Riley‐Ruvalcaba syndrome who also had hemimegalencephaly and in whom the hemimegalencephaly was evident well before presentation of the characteristic manifestations of Bannayan‐Riley‐Ruvalcaba syndrome. An 11‐year‐old boy developed drug‐resistant focal seizures on the fifth day of life. MRI revealed left hemimegalencephaly. He later showed macrocephaly, developmental delay, athetotic quadriplegic cerebral palsy, and neuromuscular scoliosis. Freckling of the penis, which is characteristic of Bannayan‐Riley‐Ruvalcaba syndrome, was not present at birth but was observed at 9 years of age. Gene analysis revealed a c.510 T>G PTEN mutation. This patient and his other affected family members, his father and two siblings, were started on the tumour screening procedures recommended for patients with PTEN mutations. This case highlights the importance of early screening for PTEN mutations in cases of hemimegalencephaly not otherwise explained by another disorder, even in the absence of signs of Proteus syndrome or the full manifestations of Bannayan‐Riley Ruvalcaba syndrome.
High‐level visual manifestations of epileptic seizures originating from the medial parietal cortex
Authors: Pendekanti Shrita , Baek Sori , Kilinc Yagmur Basak , Parvizi Josef 
Published: August 2019

ABSTRACTWe describe the case of a patient with well‐localized focal seizures originating from the medial parietal cortex. Seizures originated from area 7m, and findings revealed clear visuospatial semiological signs that may be used clinically to help diagnose similar cases of seizures in non‐lesional patients.
Stimulation‐induced ictal vocalisation of left frontal lobe origin
Authors: Baysal‐Kirac Leyla , Hartl Elisabeth , Vollmar Christian , Ernst Katharina , Lohr Hannah , Noachtar Soheyl 
Published: August 2019

ABSTRACTPure ictal non‐speech vocalisation occurs in frontal and temporal lobe epilepsies. Electrical stimulation of supplementary motor areas is reported to evoke vocalisation in selected patients. Here, we report a patient with focal epilepsy of the left anterior insula who had isolated monotonous vocalisation elicited by electrical stimulation of the left superior frontal gyrus. Quantitative analysis of audio signals was performed and compared with a former patient with left frontal lobe epilepsy who had pure ictal vocalisation. Both patients showed a comparable reduction in frequency variation indicating a monotonous voice. [Published with video sequences onwww.epilepticdisorders.com]
Epilepsy for primary health care: a cost‐effective Latin American E‐learning initiative
Authors: Carrizosa Jaime , Braga Patricia , Albuquerque Marly , Bogacz Alicia , Burneo Jorge , Coan Ana C. , Contreras Guilca , Guilhoto Laura , Izquierdo Álvaro , Ladino Lady , Lin Katia , Manreza Maria L. , Morales Lilia , Ríos Loreto , Solarte Rodrigo , Valente Kette , Venegas Viviana , Uscátegui‐Daccarett Angélica , Yacubian Elza M. ,   on behalf of the Working Group EVELA (Epilepsy Virtual Education in Latin America), and the ILAE Education Commission
Published: August 2019

ABSTRACTAims. A lack of neurologists in Latin America forces primary health care providers to manage epilepsy. With the main goal of improving diagnostic and therapeutic management of patients with epilepsy through training of physicians in the primary health care level, the International League Against Epilepsy Education Commission (2013–2017) created a low‐cost, regional, virtual course.Methods. The course, set‐up in Moodle platform, was structured in eight modules, each lasting for a week. Teaching was based on written didactic material, videos, and interactive discussions, both in Spanish and Portuguese. Topics included epidemiology, diagnosis, classification, treatment, prognosis, social issues, and epilepsy policies. Each course was limited to 50 participants and priority was given to general practitioners. Certification was given to those approving the final examination.Results. Since 2015, five courses have been developed, involving 143 participants from 17 countries and 21 tutors. Of the participants, 61% worked in primary health care services. A total of 129 participants (90%) completed the course, and 110 submitted the final examination with an approval rate of 95%. From 85 participants completing the course evaluation, 98% would recommend the course to other colleagues, and 99% showed interest in taking other similar courses. High self‐confidence for the management of patients with epilepsy increased from 21% at baseline to 73% after the course.Conclusions. The online course on epilepsy for primary care physicians in Latin America was shown to be a cost‐effective course, with good retention and excellent approval rates. Our current challenges include periodic updating, complete self‐sustainability, and exploring different strategies to reach our target audience more effectively.
Rasmussen syndrome: an atypical presentation in ten patients
Authors: Caraballo Roberto H. , Valenzuela Gabriela Reyes , Pociecha Juan , Princich Juan P. , Gutierrez Robinson , Beltran Lucas , Lubieniecki Fabiana , Bartuluchi Marcelo 
Published: August 2019

ABSTRACTAim. The aim of this study was to analyse the electroclinical and imaging findings and outcome of patients with Rasmussen syndrome (RS) with atypical manifestations.Methods. We conducted a retrospective, descriptive study of 10 of 44 consecutive patients with RS with atypical features, followed between 1999 and 2017.Results. Six patients were boys and four were girls. The mean and median ages at onset of the seizures were 8.8 and 6.5 years, respectively (range: 4.6‐13 years). All of the patients except one had seizures. Eight patients (80%) had epilepsia partialis continua that started at a mean age of 7.5 years (range: 7–15 years). In our series, hemiparesis without seizures was the first manifestation in three patients, one of whom had dual pathology. In two patients, the first manifestation was dyskinetic movements, followed by delayed‐onset seizures associated with unilateral caudate atrophy. Two patients had a focal lesion mimicking focal cortical dysplasia as the first MRI abnormality; one of these two patients had epileptic spasms in clusters. Bilateral cerebral hemisphere involvement was observed in three patients during the course of the disease. Six of eight patients responded well to surgical treatment.Conclusions. Progressive hemiparesis alone or with delayed‐onset seizures, dyskinetic movements associated with seizures, a focal lesion mimicking focal cortical dysplasia, and bilateral brain involvement were the atypical features recognized. Our series of patients responded well to surgery. Clinical, video‐EEG, and neuroradiological follow‐up is important for early confirmation of RS in order to initiate adequate management of the condition.
IKHFA’ “DWIPROSES FONOLOGIS dan KAIDAH FONOLOGINYA”
Authors: Anam Faqihul Agus 
Published: August 2019

This research aims to describe phonological processes (PP) of ikhfa, as object of Qur’anic Phonology, and the phonological rule of ikhfa by Generative Phonology approach. In addition, this aims to prove the duality of PP ikhfa too. The data are al-Hashmi’s data (2004) that is wrtitten in his theses. The goal of aplicated this model of data is keeping validity of data. The result shows that PP of ikhfa’ is the duality of PP, namely consonant asimilation and vocalic nasalitation. The PP rule of PP ikhfa’ is [n] à/n/ [α ant] [β cor]/_ [α ant]. It means that arabic nasal sound [n] can be pronounced as phonem /n/ with [+ ant] [+ cor] property of distingtive feature if tha nasal sound [n] located at sound environment with [+ ant] property of distinctive feature.

Key words: Phonological Processes (PP), Ikhfa’, Quranic
Phonology, Generative Phonology, Distinctive Feature
The Development of Analytic Team Collaborative Learning Model Based on Brain-Based Learning (BBL) for Junior High School Science Learning in Agroecosystem Areas
Authors: Mertha Yolanda Leony Agustia , Mudakir Imam , Prihatin Jekti 
Published: August 2019

Abstract
Students in the agroecosystem area are generally less active because the teacher does not utilize the potential of the surrounding agroecosystem. This can be overcome by applying Brain-Based Learning (BBL) approach combined with AT collaborative learning model. This study aimed to produce Analytic Team learning model based on Brain-Based Leaning (AT-BBL) that is valid, practical, and effective, and also improves the students' critical thinking skills and learning outcomes. This study used R&D with 4D development model which only carried out three stages, namely define, design, and development without using the disseminate stage. The average validation is 86.98 (highly valid). The average percentage of teacher responses is 92.94%, and the average percentage of student responses is 91.22% which is very practical.

Keywords: Agroecosystem, BBL approach, Analytic Team Learning Model, Collaborative Learning
PENGARUH HASIL PERKEBUNAN KEMIRI TERHADAP KEHIDUPAN SOSIAL EKONOMI MASYARAKAT DESA BENTENG TADO KECAMATAN LEMBOR SE LATAN KABUPATEN MANGGARAI BARAT NUSA TENGGARA TIMUR
Authors: Chandra Kamilus Adi , Jayanti Mustika Arif 
Published: August 2019

Masyarakat Desa Benteng Tado pada umumnya memiliki banyak perkebunan kemiri. Kemiri merupakan tanaman pohon asli indo-malaysia yang bijinya di manfaatkan sebagai sumber minyak dan rempah-rempah dan sangat berguna bagi masyarakat dunia. Tujuan dari penelitian ini adalah untuk mengetahui apa pengaruh hasil perkebunan kemiri terhadap kehidupan sosial ekonomi masyarakat Desa Benteng Tado Kecamatan Lembor Selatan Kabupaten Manggarai Barat Propinsi Nusa Tenggara Timur.Metode yang digunakan dalam penelitian ini yaitu metode korelasional. Data hasil penelitian dianalisis secara statistik dengan teknik analisis data menggunakan pengukuran koefisien pearson produck moment dengan menggunakan program bantu SPSS 20 for windows.Berdasarkan hasil uji statistik pearson product moment correlations, diketahui nilai koefisien korelasi antara hasil perkebunan kemiri dengan kehidupan sosial ekonomi sebesar =0,937 dengan signifikansi =0,000. Nilai sig.=0,000<0,05, sehingga hipotesis yang diajukan diterima. Hal ini menunjukan bahwa ada pengaruh antara hasil perkebunan kemiri dengan kehiduan sosial ekonomi masyarakat Desa Benteng Tado Kecamatan Lembor Selatan Kabupaten Manggarai Barat Nusa Tenggara Timur