Abstract:
:We have reported long-term clinical follow-up for two siblings with Lafora disease, a brother and sister, one of whom autopsied. Both siblings had repeated attacks of severe myoclonus, tonic and tonic-clonic convulsions, and intractable status epilepticus. The addition of orally administered zonisamide brought about striking effective seizure control for about 12-14 years in both patients, relieving not only myoclonus and generalized tonic-clonic seizures but also intractable status epilepticus.
journal_name
Epilepsy Resjournal_title
Epilepsy researchauthors
Yoshimura I,Kaneko S,Yoshimura N,Murakami Tdoi
10.1016/s0920-1211(01)00282-0subject
Has Abstractpub_date
2001-09-01 00:00:00pages
283-7issue
3eissn
0920-1211issn
1872-6844pii
S0920-1211(01)00282-0journal_volume
46pub_type
杂志文章abstract::An earlier pharmacodynamic study of the chiral antiepileptic drug stiripentol in an intravenous pentylenetetrazol-induced seizure model in the rat showed the development of a significant degree of tolerance to the anticonvulsant and neurotoxic effects following subacute treatment with the racemic compound. A more rece...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/0920-1211(94)90001-9
更新日期:1994-06-01 00:00:00
abstract:PURPOSE:To evaluate the efficacy and safety of stiripentol as add-on therapy in Japanese patients with Dravet syndrome treated with clobazam (CLB) and valproate (VPA). METHODS:In this open-label study, patients aged 1-30 years entered a 4-week baseline phase, followed by a 4-week stiripentol dose-adjustment and 12-wee...
journal_title:Epilepsy research
pub_type: 临床试验,杂志文章
doi:10.1016/j.eplepsyres.2014.02.008
更新日期:2014-05-01 00:00:00
abstract::Five oral formulations of valproic acid (VPA)/divalproex sodium are approved and commonly used in the US for treatment of epilepsy, mania/bipolar disorder and migraine prophylaxis. These formulations have unique pharmacokinetic and formulation characteristics and are designed to treat distinct patient populations. We ...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2006.11.005
更新日期:2007-03-01 00:00:00
abstract:BACKGROUND:Since intracranial electrode implantation has limited spatial sampling and carries significant risk, placement has to be effective and efficient. Structural and functional imaging of several different modalities contributes to localising the seizure onset zone (SoZ) and eloquent cortex. There is a need to su...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2013.08.002
更新日期:2013-11-01 00:00:00
abstract:RATIONALE:Measurement of local cerebral blood flow (LCBF) is routinely used to locate the areas involved in the generation and spread of seizures in epileptic patients. Since the spatial distribution and extent of ictal LCBF depends on the epileptogenic network, but also on the timing of injection of tracer, we used a ...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2006.01.008
更新日期:2006-05-01 00:00:00
abstract:PURPOSE:To assess subjective seizure anticipation in patients with focal and generalized epilepsy. METHODS:Five hundred consecutively recruited out-patients (251 male, 249 female, mean age 38.1 year) from three German tertiary epilepsy referral centres filled out questionnaires regarding subjective anticipation of sei...
journal_title:Epilepsy research
pub_type: 杂志文章,多中心研究
doi:10.1016/j.eplepsyres.2006.02.001
更新日期:2006-07-01 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
abstract::The idiopathic generalized epilepsies (IGE), for which a genetic cause is widely accepted, account for 20-30% of all epilepsies. Mapping these epilepsies is difficult, but progress in the positional cloning of idiopathic epilepsy genes responsible for monogenic forms provide emerging evidence that many idiopathic epil...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2003.09.002
更新日期:2003-10-01 00:00:00
abstract::In rats, electrographic seizure activity was recorded from both the inferior collicular cortex and the deep prepiriform after i.p. administration of 15 mumol/kg bicuculline, a dose which produced a variety of seizure behaviors. electrographic spikes recorded from the deep prepiriform cortex coincided with myoclonic je...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/0920-1211(91)90032-b
更新日期:1991-01-01 00:00:00
abstract::The motivational consequences of kindling were examined in the conditioned place preference paradigm. In a distinctive environment, rats received stimulation of the amygdala that triggered afterdischarge (AD) alone or AD and generalized seizures. After 4 conditioning trials, preference for the conditioning environment...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/0920-1211(92)90051-t
更新日期:1992-12-01 00:00:00
abstract::The potential clinical efficacy of tiagabine for control of status epilepticus was evaluated in an experimental model. Tiagabine was administered to cobalt-lesioned rats in which status epilepticus was induced by injection of homocysteine thiolactone. Tiagabine was effective in controlling status epilepticus in this m...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/0920-1211(94)90067-1
更新日期:1994-12-01 00:00:00
abstract::Jasmine flowers and leaves are used extensively in folk medicine in different parts of the world to treat a variety of diseases. However, there are very few published reports on the neuropsychiatric effects of Jasmine extracts. Hence, the objectives of the present study were to examine the effects of an alcohol extrac...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2017.02.012
更新日期:2017-03-01 00:00:00
abstract::Overexpression of multidrug efflux transporters such as P-glycoprotein (Pgp; ABCB1) or multidrug resistance proteins (MRPs; ABCC) in the blood-brain barrier has recently been suggested to explain, at least in part, pharmacoresistance in epilepsy, which affects about 30% of all patients with this common brain disorder....
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2003.12.007
更新日期:2004-02-01 00:00:00
abstract:BACKGROUND:Little is known, so far, about the cerebral structural abnormalities in drug-naïve patients with childhood absence epilepsy (CAE). We aimed to investigate regional grey matter (GM) volume differences using voxel-based morphometry (VBM) in patients and closely matched healthy control subjects. METHODS:Twenty...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2016.05.003
更新日期:2016-08-01 00:00:00
abstract::Temporal lobe epilepsy (TLE) has been modeled in mice using pilocarpine induction, with variable results depending on specific strains. To allow efficient xenotransplantation for the purpose of optimizing potential cell-based therapy of human TLE, we have determined the optimal dosing strategy to produce spontaneous r...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2014.10.016
更新日期:2015-01-01 00:00:00
abstract:PURPOSE:Epilepsy is a diverse neurological condition with extreme genetic and phenotypic heterogeneity. The introduction of next-generation sequencing into the clinical laboratory has made it possible to investigate hundreds of associated genes simultaneously for a patient, even in the absence of a clearly defined synd...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2018.05.004
更新日期:2018-08-01 00:00:00
abstract::The anticonvulsant activities of a noncompetitive (GYKI 52466) and a competitive (NBQX) AMPA/kainate antagonist were compared in the maximal electroshock (MES) seizure test and various chemoconvulsant models. Both antagonists were protective in the MES and pentylenetetrazol tests. GYKI 52466 was also protective agains...
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
abstract:BACKGROUND:High frequency oscillations (HFOs) have provided a new insight in understanding ictogenesis and seizure localization. In absence seizures, HFOs were predominantly localized in the medial prefrontal cortex (MPFC) in MEG studies. METHODS:We studied HFOs (80-250 Hz) in scalp EEGs in patients with absence epile...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2015.06.008
更新日期:2015-09-01 00:00:00
abstract:PURPOSE:Indoleamine 2,3-dioxygenase (IDO) is a cytokine-inducible enzyme that participates in tryptophan (trp) and serotonin metabolism with an ability to modulate neuroinflammation. Several recent studies have shown associations between cytokines and epilepsy. In this study we investigated whether activation of IDO is...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2011.02.003
更新日期:2011-05-01 00:00:00
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::Freeze-lesion-induced focal cortical dysplasia in rats closely resembles human microgyria, a neuronal migration disorder associated with drug-resistant epilepsy. Alterations in expression of N-methyl-D-aspartate receptors (NMDARs) containing NR2B subunits have been suggested to play a role in the hyperexcitability see...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2006.07.013
更新日期:2006-12-01 00:00:00
abstract::The actions of adenosine in modulating amygdala kindling were examined using the stable adenosine analog 5'-N'-ethylcarboxamidoadenosine (NECA) and caffeine, an adenosine antagonist. Systemically administered NECA was found to significantly reduce the rate of postictal spiking and to significantly increase the duratio...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/0920-1211(90)90070-c
更新日期:1990-08-01 00:00:00
abstract:OBJECTIVE:To validate the Chinese version of the Generalized Anxiety Disorder-7 (GAD-7) in Chinese people with epilepsy (PWE). METHODS:A consecutive cohort of PWE from the West China Hospital was recruited. Each patient received a psychiatric evaluation comprising the Mini International Neuropsychiatric Interview (MIN...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2015.11.019
更新日期:2016-02-01 00:00:00
abstract::Multiple subpial transection (MST) is an effective surgical therapy for patients with intractable seizures whose epileptogenic lesions lie in the cortex and are unresectable. Morrell developed this procedure and reported clinical results obtained using it. However, only the disappearance of epileptiform discharges aft...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/0920-1211(95)00003-s
更新日期:1995-05-01 00:00:00
abstract:BACKGROUND:Many new antiepileptic drugs (AEDs) have become available in recent years. Investigations of prescription patterns and exposure of AEDs to different patient groups are important regarding drug safety aspects. The aim of this study was to investigate the use of AEDs in epilepsy, with focus on exposure of AEDs...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2011.02.012
更新日期:2011-06-01 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
abstract::Mutations of the neuronal voltage-gated sodium channel alpha subunit type II (SCN2A) cause various epileptic syndromes, but have never been reported in association with acute encephalopathy. To validate the involvement of SCN2A mutations in acute encephalopathy, we screened 25 patients and found a novel missense mutat...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2012.04.016
更新日期:2012-11-01 00:00:00
abstract:PURPOSE:Epilepsy is associated with an increased mortality. This study estimates the effects of co-morbid disorders on short-term and long-term mortality and presents cause-specific mortality for children and young adults with epilepsy. METHODS:We established a population-based cohort of 1,855,946 children born in Den...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2015.05.001
更新日期:2015-08-01 00:00:00
abstract:PURPOSE:The aim of this study was to investigate by a prospective, self-controlled method, whether treatment with carbamazepine (CBZ) and sodium valproate (VPA) monotherapy may alter serum lipoprotein (a) [Lp(a)] concentrations in epileptic children. METHODS:Serum Lp(a) concentrations have been determined in 18 epilep...
journal_title:Epilepsy research
pub_type: 临床试验,杂志文章
doi:10.1016/j.eplepsyres.2006.05.002
更新日期:2006-08-01 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