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https://www.readbyqxmd.com/read/30109707/electroclinical-features-of-epilepsy-monosomy-1p36-syndrome-and-their-implications
#1
Alberto Verrotti, Marco Greco, Gaia Varriale, Agnese Tamborino, Salvatore Savasta, Marco Carotenuto, Maurizio Elia, Francesca Operto, Lucia Margari, Vincenzo Belcastro, Angelo Selicorni, Elena Freri, Sara Matricardi, Tiziana Granata, Francesca Ragona, Giuseppe Capovilla, Alberto Spalice, Giangennaro Coppola, Pasquale Striano
OBJECTIVIES: Monosomy 1p36 syndrome is a recognized syndrome with multiple congenital anomalies; medical problems of this syndrome include developmental delay, facial dysmorphisms, hearing loss, short stature, brain anomalies, congenital heart defects. Epilepsy can be another feature but there are few data about the types of seizures and long term prognosis. The aim of this work was to analyse the electroclinical phenotype and the long-term outcome in patients with monosomy 1p36 syndrome and epilepsy...
August 14, 2018: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/30108280/ketogenic-diet-a-potentially-valuable-therapeutic-option-for-the-management-of-refractory-epilepsy-in-classical-neonatal-nonketotic-hyperglycinemia-a-case-report
#2
Maina P Kava, Annie Robertson, Lawrence Greed, Shanti Balasubramaniam
Nonketotic hyperglycinemia (NKH) is a devastating inborn error of glycine metabolism caused by deficient activity of the glycine cleavage enzyme. Classically, patients present with lethargy, hypotonia, myoclonic jerks, transient respiratory depression in the first week of life and often progress to death. Surviving infants have profound psychomotor retardation, refractory epilepsy and poor quality of life. Currently, no effective therapeutic avenues exist for severe NKH. Ketogenic diet (KD) has been trialled only in a small group of patients with neonatal NKH and early myoclonic encephalopathy, in whom significant improvements in seizure control were reported...
August 14, 2018: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/30105131/bilateral-stereotactic-lesions-and-chronic-stimulation-of-the-anterior-thalamic-nuclei-for-treatment-of-pharmacoresistant-epilepsy
#3
A R Sitnikov, Yu A Grigoryan, L P Mishnyakova
Background: The use of the anterior nucleus of thalamus (ANT) as a target for treatment of pharmacoresistant epilepsy is based on its crucial role in seizure propagation. We describe results of chronic bilateral ANT stimulation and bilateral ANT lesions in 31 patients with refractory epilepsy. Methods: ANT DBS was performed in 12 patients (group I) and bilateral stereotactic radiofrequency lesions of ANT were performed in 19 patients (group II). Targeting was based on stereotactic atlas information with correction of the final coordinates according to the location of anatomical landmarks and intraoperative microelectrode recording data...
2018: Surgical Neurology International
https://www.readbyqxmd.com/read/30103160/electrical-activity-of-the-human-amygdala-during-all-night-sleep-and-wakefulness
#4
Zeidy Muñoz-Torres, Francisco Velasco, Ana L Velasco, Yolanda Del Río-Portilla, María Corsi-Cabrera
OBJECTIVE: The aim of the present work was to characterize the dynamics of the human amygdala across the different sleep stages and during wakefulness. METHODS: Simultaneous intracranial electrical recordings of the amygdala, hippocampus, and scalp electroencephalography during spontaneous sleep polysomnography in four patients suffering from epilepsy were analyzed. RESULTS: Power spectrum of the amygdala revealed no differences between rapid eye movement (REM) and wakefulness for all frequencies except higher power at 9 Hz during wakefulness and some low Gamma frequencies...
July 29, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/30100226/cannabidiol-improves-frequency-and-severity-of-seizures-and-reduces-adverse-events-in-an-open-label-add-on-prospective-study
#5
Jerzy P Szaflarski, Elizabeth Martina Bebin, Gary Cutter, Jennifer DeWolfe, Leon S Dure, Tyler E Gaston, Pongkiat Kankirawatana, Yuliang Liu, Rani Singh, David G Standaert, Ashley E Thomas, Lawrence W Ver Hoef
The objective of this study was to characterize the changes in adverse events, seizure severity, and frequency in response to a pharmaceutical formulation of highly purified cannabidiol (CBD; Epidiolex®) in a large, prospective, single-center, open-label study. We initiated CBD in 72 children and 60 adults with treatment-resistant epilepsy (TRE) at 5 mg/kg/day and titrated it up to a maximum dosage of 50 mg/kg/day. At each visit, we monitored treatment adverse events with the adverse events profile (AEP), seizure severity using the Chalfont Seizure Severity Scale (CSSS), and seizure frequency (SF) using seizure calendars...
August 9, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/30099920/genetic-polymorphism-of-vkorc1-1639-in-children-with-intracranial-hemorrhage-due-to-vitamin-k-deficiency
#6
Uğur Berber, Mehmet Akif Özdemir, Ekrem Unal, Serpil Taheri, Serkan Yildiz, Keziban Korkmaz Bayramov, Yunus Güler, Hüseyin Per
Intracranial hemorrhage due to vitamin K deficiency is a serious disease that can lead to morbidity, mortality, and mental retardation. Our goal in this study is to determine the frequency of VKORC1-1639 G>A polymorphism in patients who have undergone intracranial hemorrhage due to vitamin K deficiency bleeding (VKDB). To study VKORC1-1639 G>A polymorphism, blood was drawn from patients (n = 51, age 8:0 ± 6:5 years) followed at the Pediatric Neurology and Hematology section, Faculty of Medicine, Erciyes University, between 1990 and 2016, diagnosed with VKDB as idiopathic or from patients diagnosed with intracranial hemorrhage due to secondary vitamin K deficiency and also from volunteers (n = 51, age 11 ± 4...
August 13, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/30097339/comparative-effectiveness-of-add-on-therapy-with-newer-generation-antiepileptic-drugs-in-bulgarian-patients-with-refractory-epilepsy
#7
Ekaterina Viteva, Zahari Zahariev
OBJECTIVES: The objective of this study is to perform an open, prospective study on various aspects of comparative effectiveness of newer-generation antiepileptic drugs as add-on therapy in Bulgarian patients with drug-resistant epilepsy. METHODS: The study was performed with the participation of 1259 patients with epilepsy who attended the Clinic of Neurology at the University Hospital in Plovdiv, Bulgaria for regular visits and completed diaries about seizure frequency, severity, and adverse events...
August 7, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/30096484/lithium-affects-rat-hippocampal-electrophysiology-and-epileptic-seizures-in-a-dose-dependent-manner
#8
Guohui Jiang, Tianqiang Pu, Zhimin Li, Xiaodong Zhang, Ruijiao Zhou, Xing Cao, Juming Yu, Xiaoming Wang
Lithium, a classic mood stabilizer, prevents apoptosis-dependent cellular death and has garnered considerable interest as a neuroprotective agent that is efficacious in the treatment of many neurological diseases. However, the effects of lithium in epilepsy remain controversial. We found that different doses of lithium affect epileptic seizure activity and bidirectionally modulate the susceptibility to and severity of seizures induced by pilocarpine in rats. Recently, it has been demonstrated that systematically administered lithium affects the powers of hippocampal gamma and theta oscillations in baseline electroencephalograms...
July 29, 2018: Epilepsy Research
https://www.readbyqxmd.com/read/30094170/regional-cortical-thickness-changes-accompanying-generalized-tonic-clonic-seizures
#9
Jennifer A Ogren, Raghav Tripathi, Paul M Macey, Rajesh Kumar, John M Stern, Dawn S Eliashiv, Luke A Allen, Beate Diehl, Jerome Engel, M R Sandhya Rani, Samden D Lhatoo, Ronald M Harper
Objective: Generalized tonic-clonic seizures are accompanied by cardiovascular and respiratory sequelae that threaten survival. The frequency of these seizures is a major risk factor for sudden unexpected death in epilepsy (SUDEP), a leading cause of untimely death in epilepsy. The circumstances accompanying such fatal events suggest a cardiovascular or respiratory failure induced by unknown neural processes rather than an inherent cardiac or lung deficiency. Certain cortical regions, especially the insular, cingulate, and orbitofrontal cortices, are key structures that integrate sensory input and influence diencephalic and brainstem regions regulating blood pressure, cardiac rhythm, and respiration; output from those cortical regions compromised by epilepsy-associated injury may lead to cardiorespiratory dysregulation...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/30092753/cannabidiol-for-treating-drug-resistant-epilepsy-in-children-the-new-south-wales-experience
#10
Kerrie-Anne Chen, Michelle Farrar, Michael Cardamone, Deepak Gill, Robert Smith, Christopher T Cowell, Linda Truong, John A Lawson
OBJECTIVE: To evaluate the tolerability and safety of cannabidiol for treating drug-resistant epilepsy in children, and to describe adverse events associated with such treatment. STUDY DESIGN: Prospective, open label cohort study. SETTING: Three tertiary NSW referral centres with paediatric neurology services. PARTICIPANTS: First 40 children enrolled in the NSW Compassionate Access Scheme for children with drug-resistant epilepsy and uncountable daily seizures...
August 13, 2018: Medical Journal of Australia
https://www.readbyqxmd.com/read/30090699/long-term-use-of-methylphenidate-in-a-boy-with-hypothalamic-tumor-drug-resistant-epilepsy-and-adhd
#11
Dobrinko Socanski, Nebojsa Jovic, Harald Beneventi, Anita Herigstad
•We report the successful treatment of a boy with hypothalamic tumor, gelastic seizures, drug-resistant epilepsy and ADHD•The use of methylphenidate significantly reduced symptoms of ADHD while seizure frequency remained unchanged.
2018: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/30090156/epileptic-encephalopathy-and-autism-a-complex-interplay
#12
Ashish H Chepure, Mansi P Somaiya, Alka A Subramanyam, Ravindra K Kamath
Drug-resistant epileptic encephalopathy such as Dravet syndrome presents with autistic symptoms. Three cases with autism spectrum disorder with comorbid Dravet syndrome were assessed. All the cases presented with onset of seizures before a year and with autistic features. The patients responded to a combination of antiepileptic drugs (AEDs), resulting in reduced frequency of seizures and behavioral issues. Contrary to the belief that both epilepsy and use of AEDs have adverse impact on the cognition of children with an early onset of epilepsy, we found improvement in the symptoms of our patients who presented with autism and epilepsy...
April 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30089215/relationship-between-aggravation-of-seizures-and-methylphenidate-treatment-in-subjects-with-attention-deficit-hyperactivity-disorder-and-epilepsy
#13
Jangho Park, Hae-Won Choi, Mi-Sun Yum, Tae-Sung Ko, Seung-Hyun Shon, Hyo-Won Kim
OBJECTIVES: We aimed to investigate the effectiveness and safety of methylphenidate (MPH), and especially its influence on seizures, in subjects with attention-deficit/hyperactivity disorder (ADHD) and epilepsy through a retrospective chart review of subjects treated with MPH in a clinical setting. We also evaluated factors that could affect seizure aggravation during MPH treatment. METHODS: From April 2004 to July 2011, MPH was prescribed to 105 subjects with ADHD and epilepsy...
August 8, 2018: Journal of Child and Adolescent Psychopharmacology
https://www.readbyqxmd.com/read/30083127/efficacy-retention-and-tolerability-of-brivaracetam-in-patients-with-epileptic-encephalopathies-a-multicenter-cohort-study-from-germany
#14
Laurent M Willems, Astrid Bertsche, Frank Bösebeck, Frauke Hornemann, Ilka Immisch, Karl M Klein, Susanne Knake, Rhina Kunz, Gerhard Kurlemann, Lisa Langenbruch, Gabriel Möddel, Karen Müller-Schlüter, Felix von Podewils, Philipp S Reif, Bernhard J Steinhoff, Isabel Steinig, Felix Rosenow, Susanne Schubert-Bast, Adam Strzelczyk
Objective: To evaluate the efficacy and tolerability of brivaracetam (BRV) in a severely drug refractory cohort of patients with epileptic encephalopathies (EE). Method: A multicenter, retrospective cohort study recruiting all patients treated with EE who began treatment with BRV in an enrolling epilepsy center between 2016 and 2017. Results: Forty-four patients (27 male [61%], mean age 29 years, range 6 to 62) were treated with BRV. The retention rate was 65% at 3 months, 52% at 6 months and 41% at 12 months...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/30081431/anti-epileptic-drugs-improved-serial-7s-scores-on-the-mini-mental-state-examination-in-elderly-with-cognitive-impairment-and-epileptiform-discharge-on-electroencephalography
#15
Kazumasa Shiozaki, Satoshi Kajihara
AIM: As the population ages, epilepsy among elderly people is increasing. Epileptic seizures in the elderly are mostly complex partial seizures without convulsions, so more caution is needed in the diagnosis of epilepsy. If epileptic seizures in the context of memory impairment are overlooked, epilepsy may be misdiagnosed as dementia. However, in older patients with epilepsy, there is a possibility that cognitive function may be impaired in the interictal phase. This study aims to investigate the efficacy of anti-epileptic drug (AED) treatment for patients with cognitive impairment and interictal epileptiform discharge (IED) on electroencephalography...
August 6, 2018: Psychogeriatrics: the Official Journal of the Japanese Psychogeriatric Society
https://www.readbyqxmd.com/read/30078774/under-reporting-of-sudden-unexpected-death-in-epilepsy
#16
Sarah Chen, Golsa Joodi, Orrin Devinsky, Murrium I Sadaf, Irion W Pursell, Ross J Simpson
The identification and characterization of sudden unexpected deaths in epilepsy (SUDEP) may be improved, helping to optimize prevention and intervention. We set out to assess the frequency and demographic and clinical characteristics of SUDEP cases in a sudden death cohort. All out-of-hospital deaths were investigated from March 1, 2013 to February 28, 2015 in Wake County, NC, attended by the Emergency Medical Services. Cases were screened and adjudicated by three physicians to identify sudden death cases from any cause among free-living adults, aged 18-64...
July 31, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/30078771/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
#17
Suzanne Murray, Sara Labbé, Sanjeev Kothare, Ignacio Málaga, Gerhard Kluger, Patti Ogden, Patrice Lazure, Alexis Arzimanoglou
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. 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...
August 3, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/30077870/visually-validated-semi-automatic-high-frequency-oscillation-detection-aides-the-delineation-of-epileptogenic-regions-during-intra-operative-electrocorticography
#18
Shennan Aibel Weiss, Brent Berry, Inna Chervoneva, Zachary Waldman, Jonathan Guba, Mark Bower, Michal Kucewicz, Benjamin Brinkmann, Vaclav Kremen, Fatemeh Khadjevand, Yogatheesan Varatharajah, Hari Guragain, Ashwini Sharan, Chengyuan Wu, Richard Staba, Jerome Engel, Michael Sperling, Gregory Worrell
OBJECTIVE: To test the utility of a novel semi-automated method for detecting, validating, and quantifying high-frequency oscillations (HFOs): ripples (80-200 Hz) and fast ripples (200-600 Hz) in intra-operative electrocorticography (ECoG) recordings. METHODS: Sixteen adult patients with temporal lobe epilepsy (TLE) had intra-operative ECoG recordings at the time of resection. The computer-annotated ECoG recordings were visually inspected and false positive detections were removed...
July 20, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/30075883/risk-factors-of-cognitive-impairment-in-pediatric-epilepsy-patients-with-focal-cortical-dysplasia
#19
Nobusuke Kimura, Yukitoshi Takahashi, Hideo Shigematsu, Katsumi Imai, Hiroko Ikeda, Hideyuki Ootani, Rumiko Takayama, Yukiko Mogami, Noriko Kimura, Koichi Baba, Kazumi Matsuda, Takayasu Tottori, Naotaka Usui, Satohiko Kondou, Yushi Inoue
OBJECTIVE: The purpose of this study was to identify the risk factors of cognitive impairment in pediatric epilepsy patients with focal cortical dysplasia (FCD). METHODS: 77 patients with histopathologically confirmed FCD were studied. The statistical relationship between cognition levels and clinical factors at presurgical evaluation was analyzed. Cognitive function was evaluated by development quotient or intelligence quotient (DQ-IQ). RESULTS: Ages at seizure onset were younger than 15 years (mean ± SD; 5...
July 31, 2018: Brain & Development
https://www.readbyqxmd.com/read/30075540/a-retrospective-study-of-transcutaneous-vagus-nerve-stimulation-for-poststroke-epilepsy
#20
Guang-Fu Song, Hao-Yan Wang, Cheng-Ji Wu, Xin Li, Fu-Yi Yang
BACKGROUND: This study assessed the effect transcutaneous vagus nerve stimulation (TVNS) for poststroke epilepsy (PSE). METHODS: Fifty-two patients with PSE were included in this study. Twenty-seven patients received TVNS, 30 minutes each session, once daily, twice weekly for a total of 4 weeks; and were assigned to the treatment group. Twenty-five patients were at waiting list and were assigned to the control group. The primary outcome included weekly seizure frequency...
August 2018: Medicine (Baltimore)
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