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https://www.readbyqxmd.com/read/28295228/cognitive-network-reorganization-following-surgical-control-of-seizures-in-lennox-gastaut-syndrome
#1
Aaron E L Warren, A Simon Harvey, David F Abbott, Simon J Vogrin, Catherine Bailey, Andrew Davidson, Graeme D Jackson, John S Archer
We previously observed that adults with Lennox-Gastaut syndrome (LGS) show abnormal functional connectivity among cognitive networks, suggesting that this may contribute to impaired cognition. Herein we report network reorganization following seizure remission in a child with LGS who underwent functional magnetic resonance imaging (fMRI) before and after resection of a cortical dysplasia. Concurrent electroencephalography (EEG) was acquired during presurgical fMRI. Presurgical and postsurgical functional connectivity were compared using (1) graph theoretical analyses of small-world network organization and node-wise strength; and (2) seed-based analyses of connectivity within and between five functional networks...
March 11, 2017: Epilepsia
https://www.readbyqxmd.com/read/28284045/dosing-considerations-for-rufinamide-in-patients-with-lennox-gastaut-syndrome-phase-iii-trial-results-and-real-world-clinical-data
#2
Sanjeev Kothare, Gerhard Kluger, Rajesh Sachdeo, Betsy Williams, Omar Olhaye, Carlos Perdomo, Francesco Bibbiani
PURPOSE: Lennox-Gastaut syndrome (LGS), a rare, severe form of childhood-onset epilepsy, is difficult to control. Rufinamide is indicated for adjunctive treatment of seizures associated with LGS in adults and pediatric patients aged ≥1 year. In clinical practice, rufinamide dosing and titration may differ from the trial setting. Here, rufinamide clinical trial data are compared with real-world experience to provide insight into optimal dosing and titration strategies. METHODS: Rufinamide Phase III and open-label extension (OLE) studies were reviewed; effect of titration and dose on adverse events (AEs) and concomitant AED use were analyzed...
February 17, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28256379/outcome-of-childhood-onset-epilepsy-from-adolescence-to-adulthood-transition-issues
#3
REVIEW
R Nabbout, D M Andrade, N Bahi-Buisson, H Cross, I Desquerre, O Dulac, T Granata, E Hirsch, V Navarro, L Ouss, P L Pearl, D Schmidt, E Thiele, P R Camfield, C S Camfield
This is the second of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper addresses the outcome for some particularly challenging childhood-onset epileptic disorders with the goal of recommending the best approach to transition. We have grouped these disorders in five categories with a few examples for each. The first group includes disorders presenting in childhood that may have late- or adult-onset epilepsy (metabolic and mitochondrial disorders)...
February 27, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28249786/adrenocorticotropic-hormone-protects-learning-and-memory-function-in-epileptic-kcna1-null-mice
#4
Morris H Scantlebury, Kyoung-Chul Chun, Shun-Chieh Ma, Jong M Rho, Do Young Kim
ACTH, a member of the melanocortin family of peptides, is often used in the treatment of the developmental epileptic encephalopathy spectrum disorders including, Ohtahara, West, Lennox Gastaut and Landau-Kleffner Syndromes and electrical status epilepticus of sleep. In these disorders, although ACTH is often successful in controlling the seizures and/or inter-ictal EEG abnormalities, it is unknown whether ACTH possesses other beneficial effects independent of seizure control. We tested whether ACTH can ameliorate the intrinsic impairment of hippocampal-based learning and memory in epileptic Kcna1-null (KO) mice...
February 27, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28188044/cannabinoids-in-treatment-resistant-epilepsy-a-review
#5
REVIEW
Brooke K O'Connell, David Gloss, Orrin Devinsk
Treatment-resistant epilepsy (TRE) affects 30% of epilepsy patients and is associated with severe morbidity and increased mortality. Cannabis-based therapies have been used to treat epilepsy for millennia, but only in the last few years have we begun to collect data from adequately powered placebo-controlled, randomized trials (RCTs) with cannabidiol (CBD), a cannabis derivative. Previously, information was limited to case reports, small series, and surveys reporting on the use of CBD and diverse medical marijuana (MMJ) preparations containing: tetrahydrocannabinol (THC), CBD, and many other cannabinoids in differing combinations...
February 7, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28186331/adult-motor-phenotype-differentiates-dravet-syndrome-from-lennox-gastaut-syndrome-and-links-scn1a-to-early-onset-parkinsonian-features
#6
Danah Aljaafari, Alfonso Fasano, Fábio A Nascimento, Anthony E Lang, Danielle M Andrade
Distinguishing adult patients with Lennox-Gastaut syndrome from those with Dravet syndrome is challenging. We have previously reported that patients with Dravet syndrome present a very peculiar motor phenotype. Here we sought to confirm that this association was not linked to the chronic use of antiepileptic drugs or the many lifetime seizures. To this aim, we studied 14 adult patients with Lennox-Gastaut syndrome and 14 adults with Dravet syndrome because both conditions share similar seizure severity. We found that antecollis and parkinsonian gait were significantly more common in the Dravet group, thus suggesting that these features are part of the Dravet syndrome adult phenotype...
March 2017: Epilepsia
https://www.readbyqxmd.com/read/28078312/biallelic-scn10a-mutations-in-neuromuscular-disease-and-epileptic-encephalopathy
#7
Marios Kambouris, Julien Thevenon, Ariane Soldatos, Allison Cox, Joshi Stephen, Tawfeg Ben-Omran, Yasser Al-Sarraj, Hala Boulos, William Bone, James C Mullikin, Alice Masurel-Paulet, Judith St-Onge, Yannis Dufford, Corrine Chantegret, Christel Thauvin-Robinet, Jamil Al-Alami, Laurence Faivre, Jean Baptiste Riviere, William A Gahl, Alexander G Bassuk, May Christine V Malicdan, Hatem El-Shanti
OBJECTIVES: Two consanguineous families, one of Sudanese ethnicity presenting progressive neuromuscular disease, severe cognitive impairment, muscle weakness, upper motor neuron lesion, anhydrosis, facial dysmorphism, and recurrent seizures and the other of Egyptian ethnicity presenting with neonatal hypotonia, bradycardia, and recurrent seizures, were evaluated for the causative gene mutation. METHODS AND RESULTS: Homozygosity mapping and whole exome sequencing (WES) identified damaging homozygous variants in SCN10A, namely c...
January 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28038390/a-two-year-retrospective-evaluation-of-perampanel-in-patients-with-highly-drug-resistant-epilepsy-and-cognitive-impairment
#8
Bernd Huber, Gaby Schmid
PURPOSE: The objective of this work was to review systematically the efficacy and tolerability of perampanel (PER) in residential patients of an epilepsy center. METHOD: We adopted an industry-independent noninterventional retrospective evaluation on the basis of the paper and electronic records complemented by personal information on the part of the treating neurologists. All patients (N=26, 15 females, mean age: 30, range 21-55years) started on PER from its introduction to the market in September 2012 until December 15th 2013 were included...
December 27, 2016: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/27994367/brainstem-dysfunction-in-patients-with-late-onset-lennox-gastaut-syndrome-voxel-based-morphometry-and-tract-based-spatial-statistics-study
#9
Kang Min Park, Yun Jung Hur, Sung Eun Kim
BACKGROUND: There have been a few reports of patients who developed Lennox-Gastaut syndrome (LGS) in the second decades of their life. OBJECTIVES: The aim of this study was to investigate electroclinical presentation in patients with late-onset LGS. In addition, we evaluated structural abnormalities of the brain, which may give some clue about the common pathogenic pathway in LGS. MATERIALS AND METHODS: We enrolled the patients with late-onset LGS...
October 2016: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/27934621/drug-resistant-epilepsy-after-treatment-for-childhood-acute-lymphocytic-leukaemia-from-focal-epilepsy-to-lennox-gastaut-syndrome
#10
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 1, 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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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...
February 2017: Brain & Development
https://www.readbyqxmd.com/read/27683846/deconstructing-tolerance-with-clobazam-post-hoc-analyses-from-an-open-label-extension-study
#17
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
#18
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
#19
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
#20
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
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