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Drug-resistant AND epilepsy

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https://www.readbyqxmd.com/read/30517966/ubtf-mutation-causes-complex-phenotype-of-neurodegeneration-and-severe-epilepsy-in-childhood
#1
Lucie Sedláčková, Petra Laššuthová, Katalin Štěrbová, Jana Haberlová, Emílie Vyhnálková, Jana Neupauerová, David Staněk, Marie Šedivá, Pavel Kršek, Pavel Seeman
INTRODUCTION:  Neurodegenerative diseases of childhood present with progressive decline in cognitive, social, and motor function and are frequently associated with seizures in different stages of the disease. Here we report a patient with severe progressive neurodegeneration with drug-resistant epilepsy of unknown etiology from the age of 2 years. METHODS AND RESULTS:  Using whole exome sequencing, we found heterozygous missense de novo variant c.628G > A (p...
December 5, 2018: Neuropediatrics
https://www.readbyqxmd.com/read/30515765/cannabis-based-products-for-pediatric-epilepsy-a-systematic-review
#2
Jesse Elliott, Deirdre DeJean, Tammy Clifford, Doug Coyle, Beth K Potter, Becky Skidmore, Christine Alexander, Alexander E Repetski, Vijay Shukla, Bláthnaid McCoy, George A Wells
OBJECTIVE: To assess the benefits and harms of cannabis-based products for pediatric epilepsy. METHODS: We identified in this living systematic review randomized controlled trials (RCTs) and nonrandomized studies (NRSs) involving children with epilepsy treated with cannabis-based products. We searched MEDLINE, Embase, PsycINFO, Cochrane Library, and gray literature (April 25, 2018). The primary outcome was seizure freedom; secondary outcomes were seizure frequency (total, ≥50% reduction), quality of life, sleep, status epilepticus, death, gastrointestinal adverse events, and visits to the emergency room...
December 4, 2018: Epilepsia
https://www.readbyqxmd.com/read/30511535/efficacy-of-rufinamide-in-childhood-refractory-epilepsy
#3
Edibe Pembegül Yıldız, Zeynep Hızlı, Gonca Bektaş, Melis Ulak-Özkan, Burak Tatlı, Nur Aydınlı, Mine Çalışkan, Meral Özmen
Yıldız EP, Hızlı Z, Bektaş G, Ulak-Özkan M, Tatlı B, Aydınlı N, Çalışkan M, Özmen M. Efficacy of rufinamide in childhood refractory epilepsy. Turk J Pediatr 2018; 60: 238-243. Rufinamide has been used as a new antiepileptic drug in the treatment of drug-resistant epilepsy, in recent years. The objective of this study was to evaluate the reliability of rufinamide and its impact on seizure frequency in patients diagnosed with drug-resistant epilepsy, where seizures could not be controlled with `classical` antiepileptic drugs...
2018: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/30508033/association-of-seizure-spread-with-surgical-failure-in-epilepsy
#4
John P Andrews, Abhijeet Gummadavelli, Pue Farooque, Jennifer Bonito, Christopher Arencibia, Hal Blumenfeld, Dennis D Spencer
Importance: Seizures recur in as many as half of patients who undergo surgery for drug-resistant temporal lobe epilepsy (TLE). Understanding why TLE is resistant to surgery in some patients may reveal insights into epileptogenic networks and direct new therapies to improve outcomes. Objective: To characterize features of surgically refractory TLE. Design, Setting, and Participants: Medical records from a comprehensive epilepsy center were retrospectively reviewed for 131 patients who received a standard anteromedial temporal resection by a single surgeon from January 1, 2000, to December 31, 2015...
December 3, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/30506559/briva-life-a-multicentre-retrospective-study-of-the-long-term-use-of-brivaracetam-in-clinical-practice
#5
V Villanueva, F J López-González, J A Mauri, J Rodriguez-Uranga, M Olivé-Gadea, J Montoya, J Ruiz-Giménez, J Zurita
OBJECTIVES: Evaluate long-term effectiveness and tolerability of brivaracetam in clinical practice in patients with focal epilepsy. MATERIALS AND METHODS: This was a multicentre retrospective study. Patients aged ≥16 years were started on brivaracetam from November 2016 to June 2017 and followed over 1 year. Data were obtained from medical records at 3, 6 and 12 months after treatment initiation for evaluation of safety- and seizure-related outcomes. RESULTS: 575 patients were included in analyses; most had been treated with ≥4 lifetime antiepileptic drugs...
December 1, 2018: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/30505235/the-mtor-pathway-in-treatment-of-epilepsy-a-clinical-update
#6
Jennifer L Griffith, Michael Wong
Nearly a third of patients with epilepsy have seizures refractory to current medical therapies. In the search for novel drug targets, the mTOR pathway has emerged as key in the regulation of neuronal function, growth and survival, and other cellular processes related to epileptogenesis. Hyperactivation of the mTOR pathway has been implicated in tuberous sclerosis complex and other 'mTORopathies', clinical syndromes associated with cortical developmental malformations and drug-resistant epilepsy. Recently published clinical trials of mTOR inhibitors in tuberous sclerosis complex have shown that these drugs are effective at decreasing seizure frequency...
May 2018: Future Neurology
https://www.readbyqxmd.com/read/30504559/epilepsy-surgery-for-focal-cortical-dysplasia-seizure-and-quality-of-life-qolie-89-outcomes
#7
Jitender Chaturvedi, Malla Bhaskara Rao, A Arivazhagan, Sanjib Sinha, Anita Mahadevan, M Ravindranadh Chowdary, K Raghavendra, A S Shreedhara, Nupur Pruthi, Jitender Saini, Rose Dawn Bharath, Jamuna Rajeswaran, P Satishchandra
Aim: Surgery for drug resistant epilepsy (DRE) with focal cortical dysplasia (FCD) often requires multiple non-invasive as well as invasive pre-surgical evaluations and innovative surgical strategies. There is limited data regarding surgical management of people with FCD as the underlying substrate for DRE among the low and middle-income countries (LAMIC) including India. Methodology: The presurgical evaluation, surgical strategy and outcome of 52 people who underwent resective surgery for DRE with FCD between January 2008 and December 2016 were analyzed...
November 2018: Neurology India
https://www.readbyqxmd.com/read/30503504/invasive-evaluation-in-children-seeg-vs-subdural-grids
#8
REVIEW
Delphine Taussig, Mathilde Chipaux, Martine Fohlen, Nathalie Dorison, Olivier Bekaert, Sarah Ferrand-Sorbets, Georg Dorfmüller
Designed from the 60s to the 80s for adults, and despite the development of many new techniques, invasive explorations still have indications in children with focal drug-resistant epilepsy. The main types are stereoelectroencephalography (SEEG) and subdural explorations (SDE). They provide precise information on the localization of the epileptogenic zone (EZ), its relationships with eloquent cortex, and the feasibility of performing a tailored surgical resection. Thermocoagulations, which are a diagnostic and therapeutic tool, can be performed using SEEG electrodes...
November 16, 2018: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/30502474/multilobar-resections-for-3t-mri-negative-epilepsy-worth-the-trouble
#9
Evangelos Kogias, Barbara Schmeiser, Soroush Doostkam, Armin Brandt, Thilo Hammen, Josef Zentner, Georgia Ramantani
OBJECTIVE: Multilobar resections for MRI-negative drug-resistant epilepsy warrant attention since they account for up to one-third of MRI-negative epilepsy surgery. Despite their high relevance, data is sparse, and the risk-benefit ratio is yet under debate. This study aimed to investigate the postsurgical seizure outcomes in this particularly challenging subgroup. METHODS: We retrospectively analyzed the data of four consecutive 3T MRI-negative patients with drug-resistant focal epilepsy who underwent multilobar epilepsy surgery in our institution...
November 28, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/30501919/mirth-and-laughter-induced-by-electrical-stimulation-of-the-posterior-insula
#10
Hao Yan, Chang Liu, Tao Yu, Kaijia Yu, CuiPing Xu, Xueyuan Wang, Duanyu Ni, Yongjie Li
Laughter induced in the insula is uncommon. In this case-report we present a description of a patient with drug-resistant focal epilepsy for whom laughter with mirth was elicited when the posterior insula (PI) was stimulated by intracranial electrodes. We attempted to analyze the progression of laughter in the insula and suggest a possible pathway for the expression of laughter.
November 27, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/30497921/efficacy-and-tolerability-of-olive-oil-based-ketogenic-diet-in-children-with-drug-resistant-epilepsy-a-single-center-experience-from-turkey
#11
Orkide Guzel, Utku Uysal, Nur Arslan
PURPOSE: Ketogenic diet (KD) is an effective non-pharmacological treatment for drug-resistant epilepsy. The aim of this study was to investigate the efficacy, tolerability and complications of olive oil-based KD in epileptic children. METHOD: In this single-center, prospective study, patients were followed up at 1, 3, 6 and 12 months after KD initiation. Initially, blood ketone levels were measured daily, and as needed thereafter to maintain the levels between 4 and 5 mmol/L...
November 20, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/30497199/efficacy-of-limited-hippocampal-radiofrequency-thermocoagulation-for-mesial-temporal-lobe-epilepsy
#12
(no author information available yet)
OBJECTIVERadiofrequency thermocoagulation (RFTC), which has been developed for drug-resistant epilepsy patients, involves less brain tissue loss due to surgery, fewer surgical adverse effects, and generally good seizure control. This study demonstrates the effectiveness of RFTC performed at limited hippocampal locations.METHODSDaily seizure diaries were prospectively maintained for at least 6 months by 9 patients (ages 30-59 years) with drug-resistant mesial temporal lobe epilepsy (MTLE) before treatment with RFTC...
October 1, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/30497135/endoscope-assisted-with-robotic-guidance-and-using-a-hybrid-technique-interhemispheric-transcallosal-hemispherotomy-a-comparative-study-with-open-hemispherotomy-to-evaluate-efficacy-complications-and-outcome
#13
(no author information available yet)
OBJECTIVEEndoscope-assisted hemispherotomy (EH) has emerged as a good alternative option for hemispheric pathologies with drug-resistant epilepsy.METHODSThis was a prospective observational study. Parameters measured included primary outcome measures (frequency, severity of seizures) and secondary outcomes (cognition, behavior, and quality of life). Blood loss, operating time, complications, and hospital stay were also taken into account. A comparison was made between the open hemispherotomy (OH) and endoscopic techniques performed by the senior author...
November 1, 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/30485839/comprehensive-circular-rna-profiling-reveals-the-regulatory-role-of-the-circrna-0067835-mir-155-pathway-in-temporal-lobe-epilepsy
#14
Guo-Hua Gong, Feng-Mao An, Yu Wang, Ming Bian, Di Wang, Cheng-Xi Wei
BACKGROUND/AIMS: Temporal lobe epilepsy (TLE) is the most common form of adult localization-related epilepsy that is accompanied by progressive etiopathology and high incidences of drug resistance. Circular RNAs (circRNAs) play important roles in fine-tuning gene expression, however, the expression profile and clinical significance of circRNAs in TLE remains unknown. METHODS: Circular RNA microarray was conducted to identify TLE-related circRNAs. CCK8 assays and flow cytometric assays were conducted to clarify the role of circRNA in TLE in vitro...
November 28, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/30485578/genotype-phenotype-correlations-in-focal-malformations-of-cortical-development-a-pathway-to-integrated-pathological-diagnosis-in-epilepsy-surgery
#15
REVIEW
Barbora Benova, Thomas S Jacques
Malformations of cortical development (MCD) comprise a broad spectrum of developmental brain abnormalities. Patients presenting with MCDs often suffer from drug resistant focal epilepsy, and some become candidates for epilepsy surgery. Their likelihood of achieving freedom from seizures, however, remains uncertain, and depends in a major part on the underlying pathology. Tissue samples obtained in epilepsy surgery form the basis of definite histopathological diagnosis; however, new molecular-genetic methods have not yet been implemented in diagnostic process for MCD cases...
November 28, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30482884/title
#16
REVIEW
T L Poon, C Ht Lui, I Chan, D Yw Siu
Epilepsy is defined as drug-resistant after failure of two adequate trials of appropriately chosen and administered antiepileptic drugs. Approximately 30% of patients with epilepsy have drug-resistant epilepsy. Reasons for treatment failure include failure to recognise epilepsy syndrome, poor drug compliance, and lifestyle factors. Patients with drug-resistant epilepsy should be encouraged to have early referral to a tertiary epilepsy centre for presurgical evaluation. Comprehensive neurophysiology, structural neuroimaging, neuropsychological, and psychiatric assessments are regarded as essential for determining suitability for epilepsy surgery...
November 27, 2018: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
https://www.readbyqxmd.com/read/30477743/epilepsy-surgery-for-children-with-low-grade-epilepsy-associated-tumors-factors-associated-with-seizure-recurrence-and-cognitive-function
#17
Ara Ko, Se Hee Kim, Se Hoon Kim, Eun Kyung Park, Kyu-Won Shim, Hoon-Chul Kang, Dong-Seok Kim, Heung Dong Kim, Joon Soo Lee
OBJECTIVE: Low-grade epilepsy-associated tumors (LEATs) are associated with childhood seizures that are typically drug-resistant, necessitating surgical interventions. In this study, we aimed to investigate the efficacy of surgical intervention in children with LEATs and to identify factors associated with seizure and cognitive outcomes. METHODS: We reviewed 58 children less than 18 years of age who underwent epilepsy surgery due to histopathologically confirmed LEATs and had a minimum postoperative follow-up duration of 24 months...
October 24, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30472655/fifteen-minute-consultation-when-medicines-don-t-work-the-child-with-poorly-controlled-seizures
#18
Pooja Harijan, Arnab Kumar Seal, Manish Prasad, William P Whitehouse
Drug-resistant epilepsy (DRE) occurs in 20%-30% of children with epilepsy with significant impact on their quality of life. Management of this group of children has greatly improved in the recent years with streamlining of epilepsy surgery services and associated quaternary multimodal evaluation. This article provides a review of DRE in children and management based on recent evidence and published opinion. We have also presented an algorithmic approach to the child with possible DRE.
November 24, 2018: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/30472578/single-stage-epilepsy-surgery-in-children-and-adolescents-with-focal-cortical-dysplasia-type-ii-prognostic-value-of-the-intraoperative-electrocorticogram
#19
Gudrun Gröppel, Christian Dorfer, Sharon Samueli, Anastasia Dressler, Angelika Mühlebner, Daniela Prayer, Thomas Czech, Martha Feucht
OBJECTIVE: To evaluate prospectively the informative/prognostic value of epileptic discharges in the post-resection ECoGs of children with drug-resistant epilepsies and Focal Cortical Dysplasia type II (FCD-II). METHODS: Included were consecutive patients with focal epilepsies and suspected FCD-II who were planned for single-stage epilepsy surgery based on non-invasive presurgical evaluation results. Intraoperative ECoGs were recorded using a 32-channel system with strip- and/or grid-electrodes...
November 14, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/30470404/diffuse-low-grade-glioma-related-epilepsy
#20
REVIEW
Johan Pallud, Guy M McKhann
The World Health Organization classifies diffuse low-grade gliomas (DLGGs) are highly epileptogenic primary brain tumors; epileptic seizures occur in more than 90% of cases. Epileptic seizures and drug resistance progress during the course of DLGGs. The glioma-related epileptogenic mechanisms are multifactorial; epileptogenic foci lie within the infiltrated peritumoral neocortex. A short seizure duration before surgery and a large extent of resection are the main predictors of postoperative seizure control in DLGGs...
January 2019: Neurosurgery Clinics of North America
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