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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
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...
September 28, 2016: Neurology
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...
September 26, 2016: Epilepsia
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
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
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
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
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
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
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
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
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
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
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
Yun Jung Hur, Heung Dong Kim
OBJECTIVE: Callosotomy can reveal hidden primary epileptogenic areas in Lennox-Gastaut syndrome (LGS). We studied the significance of causal connectivity for identifying hidden epileptogenic areas in preoperative electroencephalography (EEG) and for making a decision regarding resective surgery. METHODS: We enrolled 18 LGS patients who underwent corpus callosotomy. Eight patients with unilateral epileptogenicity on post-callosotomy EEG underwent resective surgery (group A)...
August 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Won-Du Chang, Ho-Seung Cha, Chany Lee, Hoon-Chul Kang, Chang-Hwan Im
Ictal epileptiform discharges (EDs) are characteristic signal patterns of scalp electroencephalogram (EEG) or intracranial EEG (iEEG) recorded from patients with epilepsy, which assist with the diagnosis and characterization of various types of epilepsy. The EEG signal, however, is often recorded from patients with epilepsy for a long period of time, and thus detection and identification of EDs have been a burden on medical doctors. This paper proposes a new method for automatic identification of two types of EDs, repeated sharp-waves (sharps), and runs of sharp-and-slow-waves (SSWs), which helps to pinpoint epileptogenic foci in secondary generalized epilepsy such as Lennox-Gastaut syndrome (LGS)...
2016: Computational and Mathematical Methods in Medicine
Samuel Asinof, Connie Mahaffey, Barbara Beyer, Wayne N Frankel, Rebecca Boumil
Dynamin 1 is a large neuron-specific GTPase involved in the endocytosis and recycling of pre-synaptic membranes and synaptic vesicles. Mutations in the gene encoding dynamin 1 (DNM1) underlie two epileptic encephalopathy syndromes, Lennox-Gastaut Syndrome and Infantile Spasms. Mice homozygous for the Dnm1 "fitful" mutation, a non-synonymous coding variant in an alternatively spliced exon of Dnm1 (exon 10a; isoform designation: Dnm1a(Ftfl)) have an epileptic encephalopathy-like disorder including lethal early onset seizures, locomotor and neurosensory deficits...
November 2016: Neurobiology of Disease
Ricardo Zavala-Yoe, Ricardo A Ramirez-Mendoza, Luz M Cordero
Doose and Lennox-Gastaut (syndromes) are rare generalized electroclinical affections of early infancy of variable prognosis which manifest with very diverse kinds of seizures. Very frequently, these types of epilepsy become drug resistant and finding reliable treatment results is very difficult. As a result of this, fighting against these syndromes becomes a long term (or endless) event for the little patient, the neurologist and the parents. A lot of Electroencephalographic (EEG) records are so accumulated during the child's life in order to monitor evolution and correlate it with medications...
June 2016: Journal of Integrative Neuroscience
Leslie A Rudzinski, Naymeé J Vélez-Ruiz, Evan R Gedzelman, Elizabeth A Mauricio, Jerry J Shih, Ioannis Karakis
Ezogabine, clobazam, and perampanel are among the newest antiseizure drugs approved by the Food and Drug Administration between 2011 and 2012. Ezogabine and perampanel are approved for adjunctive treatment of partial epilepsy. Perampanel is also approved for adjunctive treatment of primary generalized tonic-clonic seizures. Ezogabine and perampanel have novel mechanisms of action. Ezogabine binds to voltage-gated potassium channels and increases the M-current thereby causing membrane hyperpolarization. Perampanel is a selective, non-competitive 2-amino-3-(3-hydroxy-5-methyl-isoxazol-4-yl)propanoic acid receptor antagonist, which reduces neuronal excitation...
August 2016: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
Sheridan M Hoy
Once-daily oral topiramate extended release (USL255; hereafter referred to as topiramate XR) [QUDEXY(®) XR] is approved in the USA for use as initial monotherapy and adjunctive therapy in patients aged ≥2 years with partial-onset seizures (POS) or primary generalized tonic-clonic seizures and as adjunctive therapy in patients aged ≥2 years with seizures associated with Lennox-Gastaut syndrome. Compared with twice-daily topiramate immediate release at the same total daily dose, topiramate XR provided bioequivalent exposure, an extended absorption rate (permitting convenient once-daily dosing) and more constant therapeutic plasma concentrations (potentially minimizing topiramate-associated adverse events)...
June 2016: CNS Drugs
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