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https://www.readbyqxmd.com/read/28431772/low-glycemic-index-treatment-in-patients-with-drug-resistant-epilepsy
#1
Se Hee Kim, Hoon-Chul Kang, Eun Joo Lee, Joon Soo Lee, Heung Dong Kim
OBJECTIVE: Low glycemic index treatment (LGIT) is a newly developed dietary therapeutic option for epilepsy that is less restrictive than the ketogenic diet (KD). Our objective was to determine the efficacy and tolerability of LGIT. METHODS: From March 2014 to February 2015, 36 patients received LGIT at Severance Children's Hospital. One-year seizure outcomes and side effects were evaluated. RESULTS: A total of 36 patients were assessed. Fourteen were female...
April 18, 2017: Brain & Development
https://www.readbyqxmd.com/read/28427564/inflammation-in-epileptic-encephalopathies
#2
Oleksii Shandra, Solomon L Moshé, Aristea S Galanopoulou
West syndrome (WS) is an infantile epileptic encephalopathy that manifests with infantile spasms (IS), hypsarrhythmia (in ~60% of infants), and poor neurodevelopmental outcomes. The etiologies of WS can be structural-metabolic pathologies (~60%), genetic (12%-15%), or of unknown origin. The current treatment options include hormonal treatment (adrenocorticotropic hormone and high-dose steroids) and the GABA aminotransferase inhibitor vigabatrin, while ketogenic diet can be given as add-on treatment in refractory IS...
2017: Advances in Protein Chemistry and Structural Biology
https://www.readbyqxmd.com/read/28392943/report-from-a-survey-of-parents-regarding-the-use-of-cannabidiol-medicinal-cannabis-in-mexican-children-with-refractory-epilepsy
#3
Carlos G Aguirre-Velázquez
Structured online surveys were used to explore the experiences of the parents of children with refractory epilepsy using medicinal cannabis in Mexico during September 2016. The surveys, which were completed in full, were reviewed, and 53 cases of children aged between 9 months and 18 years were identified. Of these, 43 cases (82%) were from Mexico and 10 (18%) were from Latin American countries. Of the 43 Mexican cases, the diagnoses were as follows: 20 cases (47%) had Lennox-Gastaut syndrome (LGS); 13 cases (30%) had unspecified refractory epilepsy (URE); 8 cases (19%) had West syndrome (WS); 1 case (2%) had Doose syndrome (DS); and 1 case (2%) had Ohtahara syndrome (OS)...
2017: Neurology Research International
https://www.readbyqxmd.com/read/28386925/vagus-nerve-stimulation-surgical-technique-of-implantation-and-revision-and-related-morbidity
#4
Flavio Giordano, Anna Zicca, Carmen Barba, Renzo Guerrini, Lorenzo Genitori
Indications for vagus nerve stimulation (VNS) therapy include focal, multifocal epilepsy, drop attacks (tonic/atonic seizures), Lennox-Gastaut syndrome, tuberous sclerosis complex (TSC)-related multifocal epilepsy, and unsuccessful resective surgery. Surgical outcome is about 50-60% for seizures control, and may also improve mood, cognition, and memory. On this basis, VNS has also been proposed for the treatment of major depression and Alzheimer's' disease. The vagus nerve stimulator must be implanted with blunt technique on the left side to avoid cardiac side effects through the classic approach for anterior cervical discectomy...
April 2017: Epilepsia
https://www.readbyqxmd.com/read/28379373/genetic-and-phenotypic-heterogeneity-suggest-therapeutic-implications-in-scn2a-related-disorders
#5
Markus Wolff, Katrine M Johannesen, Ulrike B S Hedrich, Silvia Masnada, Guido Rubboli, Elena Gardella, Gaetan Lesca, Dorothée Ville, Mathieu Milh, Laurent Villard, Alexandra Afenjar, Sandra Chantot-Bastaraud, Cyril Mignot, Caroline Lardennois, Caroline Nava, Niklas Schwarz, Marion Gérard, Laurence Perrin, Diane Doummar, Stéphane Auvin, Maria J Miranda, Maja Hempel, Eva Brilstra, Nine Knoers, Nienke Verbeek, Marjan van Kempen, Kees P Braun, Grazia Mancini, Saskia Biskup, Konstanze Hörtnagel, Miriam Döcker, Thomas Bast, Tobias Loddenkemper, Lily Wong-Kisiel, Friedrich M Baumeister, Walid Fazeli, Pasquale Striano, Robertino Dilena, Elena Fontana, Federico Zara, Gerhard Kurlemann, Joerg Klepper, Jess G Thoene, Daniel H Arndt, Nicolas Deconinck, Thomas Schmitt-Mechelke, Oliver Maier, Hiltrud Muhle, Beverly Wical, Claudio Finetti, Reinhard Brückner, Joachim Pietz, Günther Golla, Dinesh Jillella, Karen M Linnet, Perrine Charles, Ute Moog, Eve Õiglane-Shlik, John F Mantovani, Kristen Park, Marie Deprez, Damien Lederer, Sandrine Mary, Emmanuel Scalais, Laila Selim, Rudy Van Coster, Lieven Lagae, Marina Nikanorova, Helle Hjalgrim, G Christoph Korenke, Marina Trivisano, Nicola Specchio, Berten Ceulemans, Thomas Dorn, Katherine L Helbig, Katia Hardies, Hannah Stamberger, Peter de Jonghe, Sarah Weckhuysen, Johannes R Lemke, Ingeborg Krägeloh-Mann, Ingo Helbig, Gerhard Kluger, Holger Lerche, Rikke S Møller
Mutations in SCN2A, a gene encoding the voltage-gated sodium channel Nav1.2, have been associated with a spectrum of epilepsies and neurodevelopmental disorders. Here, we report the phenotypes of 71 patients and review 130 previously reported patients. We found that (i) encephalopathies with infantile/childhood onset epilepsies (≥3 months of age) occur almost as often as those with an early infantile onset (<3 months), and are thus more frequent than previously reported; (ii) distinct phenotypes can be seen within the late onset group, including myoclonic-atonic epilepsy (two patients), Lennox-Gastaut not emerging from West syndrome (two patients), and focal epilepsies with an electrical status epilepticus during slow sleep-like EEG pattern (six patients); and (iii) West syndrome constitutes a common phenotype with a major recurring mutation (p...
March 4, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28346953/lennox-gastaut-syndrome-a-state-of-the-art-review
#6
Mario Mastrangelo
Lennox-Gastaut syndrome (LGS) is a severe age-dependent epileptic encephalopathy usually with onset between 1 and 8 years of age. Functional neuroimaging studies recently introduced the concept of Lennox-Gastaut as "secondary network epilepsy" resulting from dysfunctions of a complex system involving both cortical and subcortical structures (default-mode network, corticoreticular connections, and thalamus). These dysfunctions are produced by different disorders including hypoxic-ischemic encephalopathies, meningoencephalitis, cortical malformations, neurocutaneous disorders, or tumors...
March 27, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28295228/cognitive-network-reorganization-following-surgical-control-of-seizures-in-lennox-gastaut-syndrome
#7
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
#8
REVIEW
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...
April 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
#9
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
#10
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...
April 3, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28188044/cannabinoids-in-treatment-resistant-epilepsy-a-review
#11
REVIEW
Brooke K O'Connell, David Gloss, Orrin Devinsky
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 8, 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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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