Effectiveness of add-on stiripentol to clobazam and valproate in Japanese patients with Dravet syndrome: additional supportive evidence.


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-week fixed-dose phase. The primary efficacy endpoint was responder rate (proportion of patients with a ≥50% reduction from baseline phase in clonic or tonic-clonic seizure frequency over the last 4 weeks of fixed-dose treatment [target phase]). Safety and pharmacokinetics were also assessed. KEY FINDINGS:Of 27 patients screened in the baseline phase, 24 patients entered the dose-adjustment phase. All patients completed the study. Responder rate was 66.7% (16/24, 95% CI: 44.7-84.4%), and four patients became free from clonic or tonic-clonic seizures. The duration of clonic or tonic-clonic seizures was also significantly reduced in the target versus baseline phase. The most frequent adverse events were somnolence, anorexia, ataxia, nasopharyngitis and γ-glutamyl transpeptidase increase, all of which were of mild-to-moderate severity. Stiripentol plasma concentration in the fixed-dose phase was 4-25 μg/mL. After adding stiripentol to CLB and VPA, the minimum plasma concentrations of CLB and N-desmethyl-CLB (NCLB) increased and that of 4'-hydroxy-N-desmethyl-CLB(OH-NCLB) decreased, while those of VPA and bromide (optionally used) were not affected. SIGNIFICANCE:Add-on stiripentol to CLB and VPA was well tolerated and significantly decreased clonic or tonic-clonic seizures in patients with Dravet syndrome.


Epilepsy Res


Epilepsy research


Inoue Y,Ohtsuka Y,STP-1 Study Group.




Has Abstract


2014-05-01 00:00:00














  • Assessment of the neuropsychiatric comorbidities in Chinese children with epilepsy using the MINI-KID tool.

    abstract::This study aimed to assess neuropsychiatric comorbidities and analyze risk factors in Chinese children with epilepsy. Children with epilepsy aged between 6 and 16 years from the Children's Hospital of Fudan University were included. Children with asthma and typically developing children were matched for age and gender...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Li T,Zhou H,Li Y,Li C,Zhang Y,Zhou Y,Wang Y

    更新日期:2018-02-01 00:00:00

  • An epileptic case with mosaic ring chromosome 6 and 6q terminal deletion.

    abstract::Ring chromosomes are rare chromosome disorders that arise usually de novo. Children with ring chromosome 6 have a wide range of intellectual functioning and congenital anomalies. We report an epileptic case of a 10-year-old boy to be mild psychomotor retardation and dysmorphic traits including microcephaly, brachyceph...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Kara N,Okten G,Guneş SO,Saglam Y,Tasdemir HA,Pinarli FA

    更新日期:2008-08-01 00:00:00

  • Familial pure paroxysmal kinesigenic dyskinesia in Han population from the Chinese mainland: a new subtype?

    abstract::Paroxysmal kinesigenic dyskinesia (PKD) is a rare neurologic inherited disease with heterogeneity. Autosomal dominant (AD) is the common inherited mode. There have been two loci mapped to 16 chromosome for PKD. However, no gene responsible for it has been identified so far. We collected 6 pedigrees from Chinese mainla...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Zhou J,Li G,Chen C,Liu D,Xiao B

    更新日期:2008-08-01 00:00:00

  • Lack of laterality in the effects of right and left amygdala kindling on weight gain in female rats.

    abstract:PURPOSE:Women with epilepsy often suffer from weight gain. A similar phenomenon is seen in female rats that are kindled from the amygdala. Interestingly, it has been reported that kindling of the left amygdala causes more weight gain than kindling of the right amygdala. The present study was designed to confirm and ext...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Hum KM,Megna S,Burnham WM

    更新日期:2009-11-01 00:00:00

  • Effect of arterial blood pressure and serum glucose on brain intracellular pH, cerebral and cortical blood flow during status epilepticus in the white New Zealand rabbit.

    abstract::Generalized seizures can induce both hypertension and hyperglycemia which may aggravate preexisting cerebral or medical conditions in patients. In vivo fluorescent imaging of regional cortical blood flow and brain intracellular pH (pHi) was performed in fasted New Zealand rabbits (n = 35) in which either mean arterial...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Tomlinson FH,Anderson RE,Meyer FB

    更新日期:1993-02-01 00:00:00

  • Population pharmacokinetics of gabapentin in infants and children.

    abstract:PURPOSE:To characterize gabapentin pharmacokinetics in infants and children using a population approach and to identify important demographic and/or physiologic determinants of gabapentin disposition. METHODS:Gabapentin was administered in single doses of 10 mg/kg (N=48 healthy subjects, age 1 month-12 years) or in mu...

    journal_title:Epilepsy research

    pub_type: 临床试验,杂志文章


    authors: Ouellet D,Bockbrader HN,Wesche DL,Shapiro DY,Garofalo E

    更新日期:2001-12-01 00:00:00

  • Metabolic and endocrine effects of valproic acid chronic treatment.

    abstract::Treatment of epileptic patients with valproic acid (VPA) may be associated with substantial weight changes that may increase morbidity and impair adherence to the treatment regimen. VPA-induced weight gain seems to be associated with many metabolic disturbances; the most frequent are hyperinsulinemia and insulin resis...

    journal_title:Epilepsy research

    pub_type: 杂志文章,评审


    authors: Belcastro V,D'Egidio C,Striano P,Verrotti A

    更新日期:2013-11-01 00:00:00

  • Can a generalized kindling seizure induce a reward state?

    abstract::The postictal behavioral depression (PBD), characterized by behavioral immobility and unresponsiveness to environmental stimuli, observed after a stage 5 kindling seizure is opioid dependent. Morphine injection prolongs while naloxone and naltrexone (opioid antagonists) reduce or eliminate PBD. Opioids have clear rewa...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Paredes RG,Muzzi G,Aguirre E,Romero V

    更新日期:2000-02-01 00:00:00

  • A gut feeling about the ketogenic diet in epilepsy.

    abstract::The ketogenic diet has been used to treat intractable epilepsy for many years, yet the mechanism(s) underlying its effectiveness have not been completely elucidated. However, emerging evidence indicates that the ketogenic diet may correct or otherwise alter a 'dystopic' gut microbiota that exhibits altered fecal micro...

    journal_title:Epilepsy research

    pub_type: 杂志文章,评审


    authors: Pittman QJ

    更新日期:2020-10-01 00:00:00

  • Clinical correlation and prognostic implication of periodic EEG patterns: A cohort study.

    abstract:OBJECTIVE:Despite increasing amounts of research on periodic discharges (PDs), large clinical studies regarding their prognostic value are lacking. The aim of the current study was to evaluate the clinical implications and prognostic value of PDs. METHODS:In this single-center retrospective cohort study, we included p...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Li HT,Wu T,Lin WR,Tseng WJ,Chang CW,Cheng MY,Hsieh HY,Chiang HI,Lee CH,Chang BL,Lim SN

    更新日期:2017-03-01 00:00:00

  • Inhibition of different GABA transporter systems is required to attenuate epileptiform activity in the CA3 region of the immature rat hippocampus.

    abstract::GABA transporters (GATs) are an essential element of the GABAergic system, which regulate excitability in the central nervous system and are thus used as targets for anticonvulsive therapy. However, in the immature nervous system the functions of the GABAergic system and the expression profile of GATs are distinct fro...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Sharopov S,Chen R,Sun H,Kolbaev SN,Kirischuk S,Luhmann HJ,Kilb W

    更新日期:2014-02-01 00:00:00

  • Diverse mechanisms of antiepileptic drugs in the development pipeline.

    abstract::There is a remarkable array of new chemical entities in the current antiepileptic drug (AED) development pipeline. In some cases, the compounds were synthesized in an attempt improve upon the activity of marketed AEDs. In other cases, the discovery of antiepileptic potential was largely serendipitous. Entry into the p...

    journal_title:Epilepsy research

    pub_type: 杂志文章,评审


    authors: Rogawski MA

    更新日期:2006-06-01 00:00:00

  • Co-occurrence of major depression or suicide attempt with migraine with aura and risk for unprovoked seizure.

    abstract::We hypothesized and found that the co-occurrence of migraine with aura (MA) with major depression (MD) or with suicide attempt (SA) increases the risk for developing unprovoked seizure more than these conditions alone. Number of conditions showed a linear relationship to seizure risk. This may reflect a new condition ...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Hesdorffer DC,Lúdvígsson P,Hauser WA,Olafsson E,Kjartansson O

    更新日期:2007-07-01 00:00:00

  • Association of intronic variants of the KCNAB1 gene with lateral temporal epilepsy.

    abstract::The KCNAB1 gene is a candidate susceptibility factor for lateral temporal epilepsy (LTE) because of its functional interaction with LGI1, the gene responsible for the autosomal dominant form of LTE. We investigated association between polymorphic variants across the KCNAB1 gene and LTE. The allele and genotype frequen...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Busolin G,Malacrida S,Bisulli F,Striano P,Di Bonaventura C,Egeo G,Pasini E,Cianci V,Ferlazzo E,Bianchi A,Coppola G,Elia M,Mecarelli O,Gobbi G,Casellato S,Marchini M,Binelli S,Freri E,Granata T,Posar A,Parmeggiani

    更新日期:2011-03-01 00:00:00

  • Juvenile myoclonic epilepsy: A system disorder of the brain.

    abstract::The prevailing understanding of generalized epilepsy is shaped by the traditional definition that "the responsible neuronal discharge takes place, if not throughout the entire grey matter, then at least in the greater part of it and simultaneously on both sides". This view is no longer tenable since concurrent finding...

    journal_title:Epilepsy research

    pub_type: 杂志文章,评审


    authors: Wolf P,Yacubian EM,Avanzini G,Sander T,Schmitz B,Wandschneider B,Koepp M

    更新日期:2015-08-01 00:00:00

  • Chronic treatment with diazepam or the inverse benzodiazepine receptor agonist FG 7142 causes differential changes in the effects of GABA receptor stimulation.

    abstract::Daily treatment of mice with diazepam leads to the development of tolerance to the anxiolytic and anticonvulsant effect of the benzodiazepine, while daily treatment with the proconvulsant benzodiazepine receptor inverse agonist FG 7142 produces sensitization to its effects in that seizures develop (chemical kindling)....

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Löscher W,Stephens DN

    更新日期:1988-07-01 00:00:00

  • Increased CPA6 promoter methylation in focal epilepsy and in febrile seizures.

    abstract::Focal epilepsy (FE) is one of the most common forms of adult epilepsy and is usually regarded as a multifactorial disorder. Febrile seizures (FS) often appear during childhood in a subtype of FE patients, i.e. with temporal lobe epilepsy (TLE) and hippocampal sclerosis (HS). FS are the most common human convulsive eve...

    journal_title:Epilepsy research

    pub_type: 杂志文章,多中心研究


    authors: Belhedi N,Perroud N,Karege F,Vessaz M,Malafosse A,Salzmann A

    更新日期:2014-01-01 00:00:00

  • TRPV1 mediates the anticonvulsant effects of acetaminophen in mice.

    abstract:OBJECTIVE:Acetaminophen is one of the most commonly used analgesic and antipyretic drugs. It has been reported that acetaminophen has anticonvulsant effects in several animal models of seizure. An active metabolite of acetaminophen, AM404, inhibits the uptake of the endocannabinoid anandamide. However, the mechanism of...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Suemaru K,Yoshikawa M,Aso H,Watanabe M

    更新日期:2018-09-01 00:00:00

  • Triheptanoin in acute mouse seizure models.

    abstract::Triheptanoin, the triglyceride of heptanoate, is used to treat certain hereditary metabolic diseases in USA because of its anaplerotic potential. In two chronic mouse seizure models this clear tasteless oil was found to be reproducibly anticonvulsant. Here we investigated the effects of triheptanoin feeding in C3H and...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Thomas NK,Willis S,Sweetman L,Borges K

    更新日期:2012-05-01 00:00:00

  • A static magnetic field modulates severity of audiogenic seizures and anticonvulsant effects of phenytoin in DBA/2 mice.

    abstract:RATIONALE:In a search for potential supplements or alternatives to the pharmacological treatment of epilepsy, we examined the effects of static magnetic fields on audiogenic seizures of DBA/2 mice. METHODS:Two strains of DBA/2 mice were subjected to auditory stimulation that resulted sequentially in wild running, loss...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: McLean MJ,Engström S,Holcomb RR,Sanchez D

    更新日期:2003-06-01 00:00:00

  • Are some idiopathic epilepsies disorders of ion channels?: A working hypothesis.

    abstract::Epilepsy is a common neurological disease and encompasses a variety of disorders with paroxysms. Although there is a genetic component in the pathogenesis of epilepsy, the molecular mechanisms of this syndrome remains poorly understood. Linkage analysis and positional cloning have not been sufficient tools for determi...

    journal_title:Epilepsy research

    pub_type: 杂志文章,评审


    authors: Hirose S,Okada M,Kaneko S,Mitsudome A

    更新日期:2000-10-01 00:00:00

  • Neurodevelopmental and epilepsy outcome in children aged one to five years with infantile spasms--a North Indian cohort.

    abstract:PURPOSE:The present study was planned as there is paucity of outcome data of children with infantile spasms, from India where profile of patients is different from the western world. Moreover, most previous studies have either not used strict inclusion criteria or standardized psychometric tests for developmental outco...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Sehgal R,Gulati S,Sapra S,Tripathi M,Kabra M,Pandey RM

    更新日期:2014-03-01 00:00:00

  • Visual seizures in children.

    abstract::Twenty-one children (11 boys, 10 girls) suffering from visual seizures were studied. The visual seizures manifested as loss of vision, flickering light, blurring or figurative hallucinations. Ictal EEGs during visual seizures were recorded in 4 cases; the ictal discharges all originated in the occipital area. Visual s...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Aso K,Watanabe K,Negoro T,Takaesu E,Furune A,Takahashi I,Yamamoto N,Nomura K

    更新日期:1987-07-01 00:00:00

  • Classification of benign infantile afebrile seizures.

    abstract:PURPOSE:The aim of this study is to classify infantile cases with benign seizures into known epileptic syndromes, thereby facilitating discussion of clinical factors that could play an important role in diagnosis. SUBJECTS:Fifty-seven patients with afebrile seizures fulfilling all of the following criteria were enroll...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Tanabe T,Hara K,Kashiwagi M,Tamai H

    更新日期:2006-08-01 00:00:00

  • The interactions between anticonvulsants and non-vitamin K antagonist oral anticoagulant agents: A systematic review.

    abstract::Use of non-vitamin K antagonist oral anticoagulants (NOACs), including dabigatran etexilate, rivaroxaban, apixaban, edoxaban or betrixaban provides a safe and convenient alternative to the traditional anticoagulation with vitamin K antagonists or heparin derivatives. Many patients receiving long-term seizure prophylax...

    journal_title:Epilepsy research

    pub_type: 杂志文章,评审


    authors: Taha M,Li W,Schmidt CM,Gonzalez-Castellon M,Taraschenko O

    更新日期:2020-05-01 00:00:00

  • Gabapentin bioavailability: effect of dose and frequency of administration in adult patients with epilepsy.

    abstract:UNLABELLED:Gabapentin (GBP) is a non-metabolized antiepileptic drug that is eliminated by renal excretion and displays saturable, dose dependent absorption. The recommended dosing schedule for GBP is t.i.d. At large daily doses, oral bioavailability (F) may be improved by giving the daily dose more frequently. OBJECTI...

    journal_title:Epilepsy research

    pub_type: 临床试验,杂志文章


    authors: Gidal BE,DeCerce J,Bockbrader HN,Gonzalez J,Kruger S,Pitterle ME,Rutecki P,Ramsay RE

    更新日期:1998-07-01 00:00:00

  • Zonisamide for add-on treatment of refractory partial epilepsy: a European double-blind trial.

    abstract::The new antiepileptic drug zonisamide was evaluated in a European multicenter parallel-group double-blind trial as add-on treatment for 139 patients with refractory partial epilepsy. During treatment with zonisamide complex partial seizures decreased by 27.7% compared to placebo (P < 0.05) and the median rate dropped ...

    journal_title:Epilepsy research

    pub_type: 临床试验,杂志文章,随机对照试验


    authors: Schmidt D,Jacob R,Loiseau P,Deisenhammer E,Klinger D,Despland A,Egli M,Bauer G,Stenzel E,Blankenhorn V

    更新日期:1993-05-01 00:00:00

  • Comparison of PET measurements of cerebral blood flow and glucose metabolism for the localization of human epileptic foci.

    abstract::We compared the relative sensitivity of two interictal PET techniques, bolus injection of [15O] labeled water for estimation of cerebral blood flow (H2(15)O CBF-PET), and 18F 2-deoxyglucose (18FDG-PET) for cerebral glucose metabolism (CMRglc), and T2-weighted magnetic resonance imaging, in 28 patients with medically i...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Leiderman DB,Balish M,Sato S,Kufta C,Reeves P,Gaillard WD,Theodore WH

    更新日期:1992-11-01 00:00:00

  • Treatment of paediatric epilepsy in Germany: antiepileptic drug utilisation in children and adolescents with a focus on new antiepileptic drugs.

    abstract:BACKGROUND:Treatment of paediatric epilepsy has advanced with the development of new antiepileptic drugs (AEDs). There are few data for Germany to which extent new AEDs are used in the treatment of childhood epilepsy. AIMS:To characterise utilisation of antiepileptics in children and adolescents with a specific emphas...

    journal_title:Epilepsy research

    pub_type: 杂志文章


    authors: Dörks M,Langner I,Timmer A,Garbe E

    更新日期:2013-01-01 00:00:00

  • Grey matter anomalies in drug-naïve childhood absence epilepsy: A voxel-based morphometry study with MRI at 3.0T.

    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


    authors: Wang G,Dai ZY,Song W,Wang S,Shi H,Pan P,Chen F,Xu Y,Zhong J

    更新日期:2016-08-01 00:00:00