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https://www.readbyqxmd.com/read/27934621/drug-resistant-epilepsy-after-treatment-for-childhood-acute-lymphocytic-leukaemia-from-focal-epilepsy-to-lennox-gastaut-syndrome
#1
Karina A González-Otárula, Blanca Mercedes Álvarez, François Dubeau
Drug-resistant epilepsy, not associated with acute brain complications or central nervous system leukaemic involvement, can develop in patients treated for acute lymphocytic leukaemia during childhood. It has been postulated that this rare complication may be due to CNS oncological treatment neurotoxicity, related to intrathecal drugs, such as methotrexate, and brain radiotherapy. We report four patients who developed drug-resistant epilepsy sometime after receiving treatment for acute lymphocytic leukaemia...
December 8, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/27857813/anesthetic-management-of-a-child-with-lennox-gastaut-syndrome-with-intractable-epilepsy-posted-for-intracranial-surgery
#2
Indu Kapoor, Girija Prasad Rath
No abstract text is available yet for this article.
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27846468/therapeutic-effects-of-the-ketogenic-diet-in-children-with-lennox-gastaut-syndrome
#3
Yunjian Zhang, Yi Wang, Yuanfeng Zhou, Linmei Zhang, Lifei Yu, Shuizhen Zhou
OBJECTIVE: The aim of this study was to evaluate the efficacy of the ketogenic diet (KD) on the clinical and electroencephalographic (EEG) features of Lennox-Gastaut syndrome (LGS) and explore the relationships between EEG changes and clinical efficacy. METHODS: We retrospectively studied 47 patients with LGS who accepted KD therapy between May 2011 and May 2015. Clinical efficacy and EEG features such as background activity, abnormal interictal epileptic discharges (IEDs) and the discharge location were evaluated prior to and at 3 and 6 months after therapy...
December 2016: Epilepsy Research
https://www.readbyqxmd.com/read/27827811/lacosamide-in-lennox-gastaut-syndrome-caution-is-still-needed
#4
REVIEW
Edoardo Ferlazzo, Sara Gasparini, Chiara Sueri, Vittoria Cianci, Umberto Aguglia
No abstract text is available yet for this article.
December 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/27806796/-dynamin-1-related-infantile-spasms-a-case-report-and-review-of-literature
#5
X L Deng, F Yin, C L Zhang, Y P Ma, F He, L W Wu, J Peng
Objective: To analyze the clinical and gene mutation characteristics of dynamin-1 (DNM1)-related infantile spasms. Method: Clinical, laboratory and genetic data of one case of DNM1-related infantile spasms diagnosed by Xiangya Hospital in September 2015 were analyzed.Through taking "Dynamin-1" "DNM1" as key words to search at CNKI, Wanfang, PubMed and OMIM to date (April 2016), the clinical characteristics of 9 reported cases of DNM1-related epileptic encephalopathy in international literature with our case were reviewed...
November 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27781027/understanding-genotypes-and-phenotypes-in-epileptic-encephalopathies
#6
REVIEW
Ingo Helbig, Abou Ahmad N Tayoun
Epileptic encephalopathies are severe often intractable seizure disorders where epileptiform abnormalities contribute to a progressive disturbance in brain function. Often, epileptic encephalopathies start in childhood and are accompanied by developmental delay and various neurological and non-neurological comorbidities. In recent years, this concept has become virtually synonymous with a group of severe childhood epilepsies including West syndrome, Lennox-Gastaut syndrome, Dravet syndrome, and several other severe childhood epilepsies for which genetic factors are increasingly recognized...
September 2016: Molecular Syndromology
https://www.readbyqxmd.com/read/27686687/spectral-characteristics-of-intracranial-electroencephalographic-activity-in-patients-with-lennox-gastaut-syndrome
#7
Dongpyo Lee, Junge Liang, Yun Jung Hur, Nam-Young Kim, Heung Dong Kim
OBJECTIVE: The purpose of the current study was to characterize the frequency profiles of epileptogenic regions, independent of visible epileptiform discharges, in intracranial EEG (iEEG) recordings of Lennox-Gastaut syndrome (LGS) patients. METHODS: We selected eight LGS patients who underwent resective surgery in the absence of definite neuroimaging findings. We calculated the absolute and relative band powers of continuous spike-free iEEG data and compared the characteristics of the resected and remaining regions...
September 26, 2016: Brain & Development
https://www.readbyqxmd.com/read/27683846/deconstructing-tolerance-with-clobazam-post-hoc-analyses-from-an-open-label-extension-study
#8
Barry E Gidal, Robert T Wechsler, Raman Sankar, Georgia D Montouris, H Steve White, James C Cloyd, Mary Clare Kane, Guangbin Peng, David M Tworek, Vivienne Shen, Jouko Isojarvi
OBJECTIVE: To evaluate potential development of tolerance to adjunctive clobazam in patients with Lennox-Gastaut syndrome. METHODS: Eligible patients enrolled in open-label extension study OV-1004, which continued until clobazam was commercially available in the United States or for a maximum of 2 years outside the United States. Enrolled patients started at 0.5 mg·kg(-1)·d(-1) clobazam, not to exceed 40 mg/d. After 48 hours, dosages could be adjusted up to 2...
October 25, 2016: Neurology
https://www.readbyqxmd.com/read/27665735/the-molecular-and-phenotypic-spectrum-of-iqsec2-related-epilepsy
#9
Ayelet Zerem, Kazuhiro Haginoya, Dorit Lev, Lubov Blumkin, Sara Kivity, Ilan Linder, Cheryl Shoubridge, Elizabeth Emma Palmer, Michael Field, Jackie Boyle, David Chitayat, William D Gaillard, Eric H Kossoff, Marjolaine Willems, David Geneviève, Frederic Tran-Mau-Them, Orna Epstein, Eli Heyman, Sarah Dugan, Alice Masurel-Paulet, Ame'lie Piton, Tjitske Kleefstra, Rolph Pfundt, Ryo Sato, Andreas Tzschach, Naomichi Matsumoto, Hirotomo Saitsu, Esther Leshinsky-Silver, Tally Lerman-Sagie
OBJECTIVE: IQSEC2 is an X-linked gene associated with intellectual disability (ID) and epilepsy. Herein we characterize the epilepsy/epileptic encephalopathy of patients with IQSEC2 pathogenic variants. METHODS: Forty-eight patients with IQSEC2 variants were identified worldwide through Medline search. Two patients were recruited from our early onset epileptic encephalopathy cohort and one patient from personal communication. The 18 patients who have epilepsy in addition to ID are the subject of this study...
November 2016: Epilepsia
https://www.readbyqxmd.com/read/27635610/-effectiveness-and-safety-of-rufinamide-at-treatment-of-epilepsy-with-complications-and-drug-resistant-epilepsy-according-to-meta-analysis-data
#10
P V Mazin, I V Sheshunov, Yu V Kislitsyn, N K Mazina
AIM: Meta-analysis was undertaken to evaluate rufinamide safety and effectiveness in numerous heterogeneous groups of patients with severe and drug resistant epileptic disorders. MATERIAL AND METHODS: There were 164 relevant articles available via medico-clinical periodic databases, but only 15 have been chosen suitable for meta-analysis. All together 1847 participants were included into common massive, with Lennox-Gastaut syndrome (LGS) and similar encephalopathy syndromes, and with drug-resistant partial epileptic forms...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/27629372/resective-surgery-combined-with-corpus-callosotomy-for-children-with-non-focal-lesional-lennox-gastaut-syndrome
#11
Ping Ding, Shuli Liang, Shaohui Zhang, Junchen Zhang, Xiaohong Hu, Xiaoman Yu
BACKGROUND: The first prospective trial for resective surgery combined with corpus callosotomy (CCT) was performed to investigate the outcomes of the combined surgery in children with Lennox-Gastaut syndrome (LGS) without focal lesion on brain MRI. METHODS: This study enrolled 68 children with LGS and without focal lesion on brain MRI, of which 25 received medicine (medicine group) and 43 underwent surgery (surgery group), including 20 with exclusively resective surgery (exclusively resection subgroup) and 23 with resective surgery combined with CCT (combined CCT subgroup)...
November 2016: Acta Neurochirurgica
https://www.readbyqxmd.com/read/27557111/thirty-years-of-orphan-drug-legislation-and-the-development-of-drugs-to-treat-rare-seizure-conditions-a-cross-sectional-analysis
#12
Jan Henje Döring, Anette Lampert, Georg F Hoffmann, Markus Ries
BACKGROUND: Epilepsy is a serious chronic health condition with a high morbidity impairing the life of patients and afflicted families. Many epileptic conditions, especially those affecting children, are rare disorders generating an urgent medical need for more efficacious therapy options. Therefore, we assessed the output of the US and European orphan drug legislations. METHODS: Quantitative analysis of the FDA and EMA databases for orphan drug designations according to STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) criteria...
2016: PloS One
https://www.readbyqxmd.com/read/27544474/emerging-antiepileptic-drugs-for-severe-pediatric-epilepsies
#13
Basanagoud Mudigoudar, Sarah Weatherspoon, James W Wheless
The medical management of the epilepsy syndromes of early childhood (eg, infantile spasms, Dravet syndrome, and Lennox-Gastaut syndrome) is challenging; and requires careful evaluation, classification, and treatment. Pharmacologic therapy continues to be the mainstay of management for these children, and as such it is important for the clinician to be familiar with the role of new antiepileptic drugs. This article reports the clinical trial data and personal experience in treating the severe epilepsies of childhood with the recently Food and Drug Administration-approved new antiepileptic drugs (vigabatrin, rufinamide, perampanel, and clobazam) and those in clinical trials (cannabidiol, stiripentol, and fenfluramine)...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544470/the-expanding-clinical-spectrum-of-genetic-pediatric-epileptic-encephalopathies
#14
Rolla Shbarou, Mohamad A Mikati
Pediatric epileptic encephalopathies represent a clinically challenging and often devastating group of disorders that affect children at different stages of infancy and childhood. With the advances in genetic testing and neuroimaging, the etiologies of these epileptic syndromes are now better defined. The various encephalopathies that are reviewed in this article include the following: early infantile epileptic encephalopathy or Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West syndrome, severe myoclonic epilepsy in infancy (Dravet syndrome), Landau-Kleffner syndrome, Lennox-Gastaut syndrome, and epileptic encephalopathy with continuous spike-and-wave during sleep...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27544466/pediatric-epileptic-encephalopathies-pathophysiology-and-animal-models
#15
REVIEW
Li-Rong Shao, Carl E Stafstrom
Epileptic encephalopathies are syndromes in which seizures or interictal epileptiform activity contribute to or exacerbate brain function, beyond that caused by the underlying pathology. These severe epilepsies begin early in life, are associated with poor lifelong outcome, and are resistant to most treatments. Therefore, they represent an immense challenge for families and the medical care system. Furthermore, the pathogenic mechanisms underlying the epileptic encephalopathies are poorly understood, hampering attempts to devise novel treatments...
May 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/27513709/spectrum-of-epilepsy-and-electroencephalogram-patterns-in-idic-15-syndrome
#16
Agatino Battaglia, Laura Bernardini, Isabella Torrente, Antonio Novelli, Gloria Scarselli
Previous reports summarized the seizure types occurring in patients with idic(15) syndrome. To better define this issue, we retrospectively analyzed the evolution of electroencephalogram findings and seizures in 35 patients with confirmed idic(15). Epilepsy occurred in 28 patients (80%), with a median age of onset of 3 years 3 months. The initial seizures were infantile spasms associated with a hypsarrhythmic electroencephalogram (nine patients), focal/generalized tonic (seven patients), or atypical absences (eight patients)...
October 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27513375/low-dose-verapamil-as-an-adjunct-therapy-for-medically-refractory-epilepsy-an-open-label-pilot-study
#17
Jaishree Narayanan, Robert Frech, Shaun Walters, Vimal Patel, Roberta Frigerio, Demetrius M Maraganore
Previous studies using verapamil as an adjunct therapy to anti-seizure medications have used doses ranging from 120 to 240mg per day. However, despite showing promising results, there was an increased incidence of side effects. The aim of this study is to assess the efficacy and tolerability of low dose verapamil (20mg p.o. tid) as adjunct therapy to patient's anti-seizure medications irrespective of the type or etiology of the epilepsy. In an open-label pilot study we enrolled 20 adult patients with history of epilepsy who continued to have a minimum of 2 seizures a month despite being on or having tried maximum tolerated doses of 3 or more standard antiepileptic drugs under the supervision of an epileptologist...
October 2016: Epilepsy Research
https://www.readbyqxmd.com/read/27475280/ketogenic-diet-treatment-for-pediatric-super-refractory-status-epilepticus
#18
Brian Appavu, Lisa Vanatta, John Condie, John F Kerrigan, Randa Jarrar
PURPOSE: We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm. METHOD: A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015. RESULTS: Ten children with super-refractory status epilepticus, ages 2-16 years, were identified...
October 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27466475/surgical-treatment-of-pediatric-focal-cortical-dysplasia-clinical-spectrum-and-surgical-outcome
#19
Hye Eun Kwon, Soyong Eom, Hoon-Chul Kang, Joon Soo Lee, Se Hoon Kim, Dong Seok Kim, Heung Dong Kim
OBJECTIVE: To analyze the clinical presentation and outcomes of surgically treated focal cortical dysplasia (FCD) in children. METHODS: We reviewed 75 cases of confirmed FCD by pathology after resective surgery. We used the pathologic classification proposed by the International League Against Epilepsy and included clinical profile and seizure and neurodevelopmental outcomes in analyses. RESULTS: There were 11 cases of FCD type I, 34 of type IIa, 19 of type IIb, and 11 of type III...
August 30, 2016: Neurology
https://www.readbyqxmd.com/read/27434073/clinical-outcome-of-patients-with-deep-brain-stimulation-of-the-centromedian-thalamic-nucleus-for-refractory-epilepsy-and-location-of-the-active-contacts
#20
Byung-Chul Son, Young Min Shon, Jin-Gyu Choi, Jiyeon Kim, Sang-Woo Ha, Sung-Hoon Kim, Si-Hoon Lee
OBJECTIVES: To investigate the clinical outcome of patients treated with chronic deep brain stimulation (DBS) of the centromedian nucleus (CM) for refractory epilepsy and to determine the location of active contacts. METHODS: The outcome of CM stimulation was evaluated as percent seizure reduction compared to the baseline 3 months. To establish the location of active contacts, 27 leads were studied in 14 patients with refractory epilepsy. An analysis was conducted to reveal whether any coordinates of the center of the active contacts predicted percent seizure reduction...
2016: Stereotactic and Functional Neurosurgery
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