Abstract：
OBJECTIVE:Recent studies have suggested that the early introduction of a ketogenic diet (KD) could improve seizure control in myoclono-astatic epilepsy (MAE). This multicenter study sought to identify the benefits of KD use on seizure control and epilepsy and on developmental outcomes in children with resistant MAE. METHODS:Fifty children who were diagnosed with severe MAE in the French network of Reference Centers for Rare Epilepsies and who were treated with KD between 2000 and 2013 were included in this study. The seizure frequency and EEG recordings were assessed two weeks before KD introduction, 2 and 6 months after, and during the last follow-up, which also included an assessment of developmental outcome. RESULTS:Patients had a median follow up of 52 months (range 13-136) and received 4.3 antiepileptic drugs [2-9] before KD introduction. Fifty-four percent (54%) of our patients were seizure-free after 6 months of KD or more, and 86% experienced more than a 70% seizure reduction after 2 months of KD. Forty-four percent (44%) of them had a clear benefit of early KD treatment (after four AEDs failed). Early KD treatment did not result in a greater seizure reduction (p=0.055), but significantly resulted in remission (p<0.028). Fifty percent of patients with resistant MAE had normal development outcomes. Earlier KD treatment, after three AEDs failed, was correlated with a better cognitive outcome (p<0.01). SIGNIFICANCE:Early introduction of KD treatment in resistant MAE has a strong, persistent anticonvulsant effect with long-term remission and better cognitive outcomes.
journal_name
Epilepsy Resjournal_title
Epilepsy researchauthors
Stenger E,Schaeffer M,Cances C,Motte J,Auvin S,Ville D,Maurey H,Nabbout R,de Saint-Martin Adoi
10.1016/j.eplepsyres.2017.02.005subject
Has Abstractpub_date
2017-03-01 00:00:00pages
64-69eissn
0920-1211issn
1872-6844pii
S0920-1211(17)30080-3journal_volume
131pub_type
杂志文章,多中心研究abstract：:Cranial MRI has been shown to be a safe procedure in patients with a vagus nerve stimulator (VNS), but body MRI may cause overheating of the stimulator lead. Here we report a case of a patient with an implanted vagus nerve stimulator who required a cervical spinal MRI due to a rapidly progressive paraparesis. The spin...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2009.02.008
更新日期：2009-04-01 00:00:00
abstract：:In rats with electrodes implanted in the hippocampus or amygdala, the effect was studied of a single episode of low frequency stimulation (125 msec, biphasic 4 Hz, for 1 min at a current 50% of threshold for after-discharge), LFS, on the after-discharge thresholds (ADTs) during the course of hippocampal and amygdaloid...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/0920-1211(89)90029-6
更新日期：1989-05-01 00:00:00
abstract：:This prospective study examined the effects of the new antiepileptic drug (AED) gabapentin (GBP) compared to the standard AED carbamazepine (CBZ) and placebo (PLA) on eye movements, posture and finger force control in 12 healthy volunteers who received single doses of 600 mg GBP and 400 mg CBZ in a placebo-controlled,...
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(98)00015-1
更新日期：1998-06-01 00:00:00
abstract：INTRODUCTION:Recent ILAE classification defined focal cortical dysplasia (FCD) patients with accompanying epileptic lesions as a separate group. We investigated data of patients with sole FCD lesions regarding long-term seizure outcome and different characteristics of FCD type 1 and type 2 patients. METHODS:Eighty chi...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2017.08.008
更新日期：2017-10-01 00:00:00
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journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/0920-1211(93)90075-i
更新日期：1993-01-01 00:00:00
abstract：:Mutations of genes coding for ion channels cause several genetically determined human epileptic syndromes. The identification of a gene variant linked to a particular disease gives important information, but it is usually necessary to perform functional studies in order to completely disclose the pathogenic mechanisms...
journal_title：Epilepsy research
pub_type： 杂志文章,评审
doi：10.1016/j.eplepsyres.2010.08.003
更新日期：2010-11-01 00:00:00
abstract：:Infantile spasms (IS) is a developmental and epileptic encephalopathy with heterogeneous etiologies including many genetic causes. Genetic studies have identified pathogenic variants in over 30 genes as causes of IS. Many of these genetic causes are extremely rare, with only one reported incidence in an individual wit...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2019.106181
更新日期：2019-10-01 00:00:00
abstract：:Bupropion, a unique, non-nicotine smoking cessation aid and an effective antidepressant, is well known to produce seizures following overdosing in humans. However, the experimental background for the usefulness of antiepileptic drugs in the protection against bupropion-induced convulsions has not been established yet....
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2005.01.006
更新日期：2005-03-01 00:00:00
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journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2019.106156
更新日期：2019-09-01 00:00:00
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journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/s0920-1211(01)00309-6
更新日期：2002-02-01 00:00:00
abstract：:We have surveyed the rates at which clinicians in the U.K. and Eire withdraw antiepileptic drugs (AEDs) in patients with active epilepsy. Practice differed widely and there was a lack of consensus regarding the rates employed. Inpatient reductions were 2-4 times faster than outpatient reductions. AED reductions tended...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/0920-1211(87)90060-x
更新日期：1987-11-01 00:00:00
abstract：:Multiple forms of the ketogenic diet (KD) have been successfully used to treat drug-resistant epilepsy, however its mainstream use as a first-line therapy is still limited. Further investigation into its clinical efficacy as well as the molecular basis of activity is likely to assist in the reversal of any resistance ...
journal_title：Epilepsy research
pub_type： 杂志文章,评审
doi：10.1016/j.eplepsyres.2011.04.017
更新日期：2012-07-01 00:00:00
abstract：:Seizure forecasting would improve the quality of life of patients with refractory epilepsy. Although early findings were optimistic, no single feature has been found capable of individually characterizing brain dynamics during transition to seizure. Cross-frequency phase amplitude coupling has been recently proposed a...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2017.11.009
更新日期：2018-01-01 00:00:00
abstract：OBJECTIVE:This was the first proof of principle clinical trial assessing the efficacy and safety of rufinamide as adjunctive therapy in epileptic patients. The pharmacokinetic (PK) profile of rufinamide was also determined. METHODS:Fifty patients with diagnoses of partial or primary generalized tonic-clonic seizures w...
journal_title：Epilepsy research
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：10.1016/s0920-1211(00)00185-6
更新日期：2001-02-01 00:00:00
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journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2006.06.015
更新日期：2006-10-01 00:00:00
abstract：:Generalized epilepsy with febrile seizures plus (GEFS+) comprises a group of clinically and genetically heterogeneous epilepsy syndrome. Here, we provide the first report of clinical presentation and mutational analysis of SCN1A gene in 36 Malaysian GEFS+ patients. Mutational analysis of SCN1A gene revealed twenty sev...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2012.08.004
更新日期：2012-12-01 00:00:00
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journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/0920-1211(93)90054-b
更新日期：1993-07-01 00:00:00
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journal_title：Epilepsy research
pub_type： 杂志文章,多中心研究,随机对照试验
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更新日期：2019-07-01 00:00:00
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journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/0920-1211(89)90001-6
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journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/0920-1211(93)90061-b
更新日期：1993-07-01 00:00:00
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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：:Mutations in voltage-gated sodium channel genes cause several types of human epilepsies. Often, individuals with the same sodium channel mutation exhibit diverse phenotypes. This suggests that factors beyond the primary mutation influence disease severity, including genetic modifiers. Mouse epilepsy models with voltag...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2015.11.016
更新日期：2016-01-01 00:00:00
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journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/0920-1211(95)00049-6
更新日期：1995-11-01 00:00:00
abstract：BACKGROUND:People with epilepsy may experience an altered pattern of mortality due to cancer. We determined cancer mortality in two distinct populations with epilepsy: (i) an institutionalised cohort with severe epilepsy (SEC) and (ii) another cohort with presumed milder epilepsy (MEC). METHODS:Mortality data from 197...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2008.11.001
更新日期：2009-02-01 00:00:00
abstract：OBJECTIVES:to determine how the duration of SE, the EEG findings during/after SE, the depth of coma at presentation and age impact on the prognosis of convulsive status epilepticus indepedent of aetiology and to analyse the outcome of status epilepticus with respect to mortality and morbidity (the latter measured in te...
journal_title：Epilepsy research
pub_type： 杂志文章,评审
doi：10.1016/j.eplepsyres.2010.09.003
更新日期：2011-01-01 00:00:00
abstract：:The rat lateral fluid-percussion injury (FPI) model has been used extensively to study post-traumatic epilepsy (PTE). Epidemiological studies have reported that the risk of PTE is higher after more severe injury. Adult, male Wistar rats subjected to different atmospheric pressures of injury during FPI showed great var...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2018.09.005
更新日期：2018-11-01 00:00:00
abstract：PURPOSE:Studies of adults who underwent temporal lobectomy for intractable temporal lobe epilepsy (TLE) demonstrated declining seizure free rates over time. Using seizure and social parameters, we followed patients who had temporal lobe surgery (TLS) in childhood to determine long-term outcomes. METHODS:We identified ...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2008.07.012
更新日期：2008-12-01 00:00:00
abstract：:Simultaneous electroencephalography-transcranial magnetic stimulation (EEG-TMS) investigates cortical reactivity to external perturbations. TMS evoked potentials (TEPs) have been described in normals during sleep and wake but not after sleep deprivation or in pathologically enhanced excitability, i.e., epilepsy. The a...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2011.05.015
更新日期：2011-09-01 00:00:00
abstract：:The purpose of this study was to determine the influence of levetiracetam on the steady-state serum concentrations of other commonly used antiepileptic drugs (AEDs). Serum AED concentrations were measured at baseline and after adjunctive therapy with levetiracetam (1000-4000 mg/day) or placebo in four phase III trials...
journal_title：Epilepsy research
pub_type： 临床试验,杂志文章,meta分析,随机对照试验
doi：10.1016/j.eplepsyres.2005.01.005
更新日期：2005-03-01 00:00:00
abstract：:Lithium is still the mainstay in the treatment of affective disorders as a mood stabilizer. Lithium also shows some anticonvulsant properties. While the underlying mechanisms of action of lithium are not yet exactly understood, we used a model of clonic seizure induced by pentylenetetrazole (PTZ) in male NMRI mice to ...
journal_title：Epilepsy research
pub_type： 杂志文章
doi：10.1016/j.eplepsyres.2010.02.001
更新日期：2010-05-01 00:00:00
