Abstract：
PURPOSE:We investigated long term prognosis of Lennox-Gastaut syndrome (LGS) with active application of recent advanced treatment modalities such as ketogenic diet (KD) or epilepsy surgery (ES). METHODS:We retrospectively evaluated 68 patients with LGS, aged 18-35 years. We assessed seizure outcomes for a range of therapeutic modalities. Evolution of seizure types, EEG characteristics, cognition, ambulation, social outcomes and other clinical data were also evaluated. RESULTS:For a mean follow-up duration of 19.3 years (range 8.3-32.5 years), finally sixteen patients (23.5%) were seizure-free. Of the 68 patients, 26 (38.2%) were treated solely with AEDs and six became seizure-free. The KD was administered to 19 patients, five patients maintained a seizure free state during the KD but only one patient was able to maintain a seizure free state by continuing on a modified Atkins diet. Focal resective surgery was performed in 15 patients, ten out of the 15 patients had Engel class I outcomes, but only three patients maintained seizure freedom to adults. CC was performed in 17 patients, and VNS was performed in 14 patients. These palliative procedures were also effective but had limitations to obtain and maintain seizure freedom to adults. Characteristic EEG features of diffuse slow spike-wave and generalized paroxysmal fast activity ceased in half of the patients. In cognitive terms, 94.7% of patients exhibited moderate to profound mental retardation. Only 39.7% of patients had intact independent daily living skills, and 25.4% could not walk, even with support. CONCLUSIONS:KD and epilepsy surgery were effective for seizure control, but they did not necessarily lead to the maintenance of a seizure-free state. LGS remains a form of intractable epilepsy despite the application of recent advanced treatment modalities.
journal_name
Epilepsy Resjournal_title
Epilepsy researchauthors
Kim HJ,Kim HD,Lee JS,Heo K,Kim DS,Kang HCdoi
10.1016/j.eplepsyres.2014.11.004subject
Has Abstractpub_date
2015-02-01 00:00:00pages
10-9eissn
0920-1211issn
1872-6844pii
S0920-1211(14)00318-0journal_volume
110pub_type
杂志文章abstract：:The subjects were 106 SMIDS with epilepsy. They were classified into four epileptic syndromes: (1) SE-MISF (34.0%), (2) SGE (25.5%), (3) SLRE (20.7%), and (4) epileptic discharge-free patients (EDFP) (19.8%). Clinical electroencephalographic studies elucidated the following result: (1) The seizure disappearance rate w...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2007.09.006
更新日期：2007-12-01 00:00:00
abstract：OBJECTIVE:The purpose of this study was to investigate regional homogeneity (ReHo) in children with new-onset drug-naïve Benign Epilepsy with Centrotemporal Spikes (BECTS), chronic BECTS and healthy controls (HC) using the Regional Homogeneity (ReHo) method applied to resting state fMRI data. METHODS:Resting state fMR...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2015.06.017
更新日期：2015-10-01 00:00:00
abstract：:Catamenial epilepsy is a multifaceted neuroendocrine condition in which seizures are clustered around specific points in the menstrual cycle, most often around perimenstrual or periovulatory period. Generally, a twofold or greater increase in seizure frequency during a particular phase of the menstrual cycle could be ...
journal_title：Epilepsy research
pub_type： 杂志文章,评审
doi：10.1016/j.eplepsyres.2009.02.017
更新日期：2009-07-01 00:00:00
abstract：:Epilepsy affects about 1 % of the world population. Mesial temporal lobe epilepsy (mTLE) presents with seizures initiated in hippocampus and is the most frequent form of epilepsy. About 30 % of individuals with mTLE do not respond to conventional medications maintaining seizures and consequently new lesions on a daily...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2020.106427
更新日期：2020-10-01 00:00:00
abstract：:Although accumulating evidence indicates that the immunomodulatory medication thalidomide exerts anticonvulsant properties, the mechanisms underlying such effects of thalidomide are still unknown. Our previous preclinical study suggested that nitric oxide (NO) signaling may be involved in the anticonvulsant effects of...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2020.106362
更新日期：2020-08-01 00:00:00
abstract：OBJECTIVE:To study the different epilepsy syndromes that included epileptic spasms (ES) in clusters without hypsarrhythmia (WoH). METHODS:Between 2/1990 and 7/2013, we registered 48 patients with the electroclinical diagnostic criteria of ES in clusters WoH. RESULTS:We recognized two subgroups. In the first subgroup ...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2016.03.006
更新日期：2016-07-01 00:00:00
abstract：PURPOSE:We sought to systematically review and perform a meta-analysis of the magnetic resonance spectroscopy (MRS) findings regarding juvenile myoclonic epilepsy (JME). METHODS:We searched for studies in the PubMed, Web of Science, and Embase electronic databases. Two authors collected articles and extracted data ind...
journal_title：Epilepsy research
pub_type： 杂志文章,meta分析,评审
doi：10.1016/j.eplepsyres.2016.01.004
更新日期：2016-03-01 00:00:00
abstract：:It has been established that febrile seizures and its extended syndromes like generalized epilepsy with febrile seizures (FS) plus (GEFS+) and Dravet syndrome have been associated with mutations especially in SCN1A and GABRG2 genes. In patients, the onset of FS is likely due to the combined effect of temperature and i...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2017.04.020
更新日期：2017-08-01 00:00:00
abstract：PURPOSE:We performed a retrospective study to investigate seizure, EEG, social and cognitive outcome in adult LGS subjects. METHODS:We retrospectively evaluated 27 LGS patients aged 40-59 years. We assessed in particular the evolution of different seizure types and EEG findings, as well as cognitive and social outcome...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2010.01.012
更新日期：2010-05-01 00:00:00
abstract：:Pregabalin (PGB) is a new antiepileptic drug (AED) which is a structural, non-functional analogue of gamma-aminobutyric acid. It acts at presynaptic calcium channels to modulate neurotransmitter release in the CNS. While the efficacy and tolerability of PGB have been demonstrated in several randomised controlled trial...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2009.08.001
更新日期：2009-12-01 00:00:00
abstract：OBJECTIVES:Perampanel, a selective, noncompetitive AMPA receptor antagonist, is indicated as adjunctive therapy for the treatment of partial seizures with or without secondarily generalized seizures and primary generalized tonic-clonic seizures in patients with epilepsy aged 12years and older. In vitro studies and Phas...
journal_title：Epilepsy research
pub_type： 杂志文章,随机对照试验
doi：10.1016/j.eplepsyres.2017.04.018
更新日期：2017-08-01 00:00:00
abstract：:There are many difficulties involved with the treatment of epilepsy, and these problems have driven the search for new agents to control epileptic seizures. Calcitonin is a peptide hormone that has been well studied and shown to have a positive effect on neuropathic and chronic pain. The mechanism by which calcitonin ...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2014.01.012
更新日期：2014-03-01 00:00:00
abstract：:Two hundred and thirty-five patients suffering from newly diagnosed epilepsy were randomly allocated to treatment with either oxcarbazepine or carbamazepine in a double-blind multi-centre study. After a titration phase (between 4 and 8 weeks), the optimal individual dose of trial medication was determined and treatmen...
journal_title：Epilepsy research
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/0920-1211(89)90070-3
更新日期：1989-01-01 00:00:00
abstract：INTRODUCTION:Prior research has shown that people with epilepsy are at risk for a poorer health-related quality of life (HRQOL). However, patients differ greatly in how well they adjust to their epilepsy. To better understand these differences, the present study examined the role of personality. More specifically, we e...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2019.106251
更新日期：2020-01-01 00:00:00
abstract：BACKGROUND:Valproate is used widely for the treatment of epilepsy but has been associated with hyperandrogenism, hyperinsulinemia, and dyslipidemia. The mechanism for these associations is unknown, but they have been hypothesized to be secondary to valproate-associated weight gain. This study was conducted to test the ...
journal_title：Epilepsy research
pub_type： 杂志文章,多中心研究
doi：10.1016/s0920-1211(03)00085-8
更新日期：2003-05-01 00:00:00
abstract：:This paper reports the first systematic review of uncommon causes of status epilepticus reported in the literature between 1990 and 2008. Uncommon causes are defined as those not listed in the main epidemiological studies of status epilepticus. 181 causes were identified. These were easily categorised into 5 specific ...
journal_title：Epilepsy research
pub_type： 杂志文章,评审
doi：10.1016/j.eplepsyres.2010.07.015
更新日期：2010-10-01 00:00:00
abstract：INTRODUCTION:Rolandic epilepsy (RE) is an idiopathic focal childhood epilepsy with a well-established neuropsychological profile of language impairment. The aim of this study is to provide a functional correlate that links rolandic (sensorimotor) pathology to language problems using functional MRI. MATERIALS AND METHO...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2013.10.008
更新日期：2013-12-01 00:00:00
abstract：:This paper evaluates the patient-specific seizure prediction performance of pre-ictal changes in bivariate-synchrony between pairs of intracranial electroencephalographic (iEEG) signals within 15min of a seizure in patients with pharmacoresistant focal epilepsy. Prediction horizons under 15min reduce the durations of ...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2010.07.014
更新日期：2010-10-01 00:00:00
abstract：:Since magnetic resonance imaging (MRI) technique is constantly evolving with higher field strength scanners, the question arises whether images from different field strength scanners can be used interchangeably for scientific and clinical purposes. We address this issue in a study group of patients with temporal lobe ...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2008.06.012
更新日期：2008-11-01 00:00:00
abstract：:A brief review of kindling is provided, which highlights some important points of historical interest often overlooked by researchers. These points include the fact that the original rating scale of convulsive seizures presented by Racine 'EEG Clin. Neurophysiol 32 (1972) 281'. was based on amygdala kindling, and may ...
journal_title：Epilepsy research
pub_type： 杂志文章,评审
doi：10.1016/s0920-1211(02)00071-2
更新日期：2002-06-01 00:00:00
abstract：:To assess the efficacy of CM40907--a new antiepileptic drug--in suppressing inter-ictal spikes, 6 patients with severe intractable epilepsy and frequent spikes in their inter-ictal EEGs were entered in a double-blind placebo-controlled trial. The results show that CM40907 is efficacious in reducing spike counts in the...
journal_title：Epilepsy research
pub_type： 临床试验,杂志文章
doi：10.1016/0920-1211(90)90046-x
更新日期：1990-04-01 00:00:00
abstract：:This study was carried out to evaluate Synthetic Aperture Magnetometry-kurtosis (SAM(g(2))), a spatially filtered source localization technique in magnetoencephalography (MEG), for identification of epileptogenic areas of focal cortical dysplasia (FCD). Three children with FCD were investigated to localize the ictal o...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2008.06.006
更新日期：2008-10-01 00:00:00
abstract：:The antidepressant drug fluoxetine (FLX) has been shown to exert antiepileptic effects in several animal models, but mixed preclinical findings and occasional reports of proconvulsant effects have led to hesitation towards its use in epileptic people. Despite being developed as a selective serotonin reuptake inhibitor...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2012.03.019
更新日期：2012-08-01 00:00:00
abstract：:The Fourth Eilat Conference on New Antiepileptic Drugs (AEDs) was held at the Royal Beach Hotel, Eilat, Israel, from 6th to 10th September 1998. Epileptologists and scientists from 20 countries attended the conference, which was held to discuss a number of issues in drug development, including outcome assessment in ep...
journal_title：Epilepsy research
pub_type：
doi：10.1016/s0920-1211(98)00108-9
更新日期：1999-03-01 00:00:00
abstract：INTRODUCTION:The anticonvulsant effects of melatonin (MT) have been demonstrated in several different experimental seizure models. Thus, the present study aimed to determine the anticonvulsant efficacy of MT, the optimum time for its administration prior to the induction of a seizure, and its effective dose in a rat mo...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2015.11.004
更新日期：2015-12-01 00:00:00
abstract：SUMMARY:Metabotropic glutamate receptors (mGluR) may represent a perspective target for anticonvulsant therapy but spectrum of their anticonvulsant effects is not sufficiently known. Our study was aimed at comparison of anticonvulsant actions of antagonists of mGluR1 and mGluR5 subtypes in immature rats. Seven-, 12-, 1...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2008.11.004
更新日期：2009-02-01 00:00:00
abstract：:Weak relationships have been reported between subjective complaints of memory deficits and memory performance in neuropsychological tests in patients with temporal lobe epilepsy (TLE). This study investigated whether the individual exposure to cognitive demands in daily life contributes to cancel out the performance-c...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/s0920-1211(98)00072-2
更新日期：1998-11-01 00:00:00
abstract：OBJECTIVE:To determine the relative bioavailability and tolerability of a topiramate (TPM) suspension after rectal administration. DESIGN/METHOD:Seven healthy men and five healthy non-pregnant women were enrolled. A 100 or 200 mg tablet of TPM was given orally and a 200 mg dose was given rectally in a randomized, open...
journal_title：Epilepsy research
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/s0920-1211(03)00083-4
更新日期：2003-05-01 00:00:00
abstract：:Zonisamide (Zonegran), a novel antiepileptic drug (AED) approved recently in Europe as adjunctive therapy for refractory partial seizures in adults, has been used extensively in Japan and the United States. A substantial body of clinical experience has accumulated over a 14-year period, allowing the properties and pha...
journal_title：Epilepsy research
pub_type： 杂志文章,评审
doi：10.1016/j.eplepsyres.2005.11.004
更新日期：2006-02-01 00:00:00
abstract：:The 5-hydroxytryptamine-1A (5-HT1A) receptors are known to be involved in the inhibition of seizures in epilepsy. Moreover, studies propose a role for the 5-HT1A receptor in memory function; it is believed that the higher density of this receptor in the hippocampus plays an important role in its regulation. Positron e...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2014.09.013
更新日期：2014-12-01 00:00:00
