Abstract:
:The characteristics of evoked potentials in patients with dentatorubral-pallidoluysian atrophy (DRPLA) were investigated. Twelve patients with DRPLA and three patients with progressive myoclonic epilepsy (PME) attributable to other causes participated in the study. In 11 out of the 12 patients, the diagnosis of DRPLA was genetically confirmed, based on a 56-75 CAG triplet repeat expansion on chromosome 12p; in the remaining patient, the diagnosis was not genetically confirmed but the patient was clinically diagnosed as having DRPLA and was within the same pedigree as one of the 11 genetically confirmed patients. Two out of the three patients with PME, who had been tested for dodecamer repeat expansion in the cystatin B gene, were genetically confirmed as having Unverricht-Lundborg disease (UL); the remaining patient was also clinically diagnosed as having UL, but the patient did not have the aforementioned genetic abnormality. Somatosensory evoked potentials (SEPs) and brainstem auditory evoked responses (BAERs) were recorded. The amplitudes of the SEPs were determined as the peak-to-peak amplitudes between P2 and N2 deflections. The results revealed that high-amplitude SEPs were not evoked in any of the DRPLA patients; on the other hand, high-amplitude SEPs were evoked in all the patients with UL. Moreover, BAERs were absent in seven out of the 12 patients with DRPLA; on the other hand, all UL patients showed BAERs in which all peaks, from I to V, were distinguishable. These results suggest differences in pathophysiology between DRPLA, which predominantly affects the brainstem and subcortical regions, and PME, characterized by cortical hyperexcitability. Thus, evoked potential measurements may be useful to differentiate DRPLA patients from those with progressive myoclonic epilepsy.
journal_name
Epilepsy Resjournal_title
Epilepsy researchauthors
Kasai K,Onuma T,Kato M,Kato T,Takeya J,Sekimoto M,Watanabe K,Minami N,Goto Y,Minabe Ydoi
10.1016/s0920-1211(99)00028-5subject
Has Abstractpub_date
1999-10-01 00:00:00pages
3-11issue
1eissn
0920-1211issn
1872-6844pii
S0920121199000285journal_volume
37pub_type
杂志文章abstract::Benzodiazepines (BDZs) are GABA(A) receptor modulators with anxiolytic, hypnotic, and anticonvulsant properties. BDZs are understood to potentiate GABA(A) receptor function by increasing channel opening frequency, in contrast to barbiturates, which increase channel open duration. However, the in vitro evidence demonst...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2009.03.007
更新日期:2009-08-01 00:00:00
abstract:PURPOSE:We studied the effect of fosphenytoin (FOS) pre-medication on the incidence and thresholds of after-discharges (ADs), seizures, and functional responses during electrical stimulation mapping (ESM). METHODS:As individualized by the attending epileptologist, FOS was given intravenously at 2 mg-phenytoin-equivale...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2018.01.017
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journal_title:Epilepsy research
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:2003-12-01 00:00:00
abstract:PURPOSE:To discern more static and lesion based from more dynamic epilepsy-driven features of depression and personality in focal symptomatic epilepsy, change in personality and mood was examined as a function of focus lateralization and seizure outcome after epilepsy surgery. METHODS:Self-reported personality and dep...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2008.07.011
更新日期:2008-12-01 00:00:00
abstract:OBJECTIVES:To evaluate the agreement between magnetoencephalography (MEG) and intracranial electroencephalography (ICEEG) results, to determine the characteristics that lead to concordance, and to assess how these factors relate to favorable epilepsy surgery outcome. MATERIALS:This retrospective study reviewed 50 pati...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2014.08.016
更新日期:2014-11-01 00:00:00
abstract::Increased excitatory amino acid transmission and decreased GABAergic inhibitory responses seem to be important mechanisms in the genesis of convulsions, where reactive oxygen species (ROS) have recently been suggested to play a critical role. Therefore, administration of antioxidants may be potentially beneficial for ...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2005.08.005
更新日期:2005-08-01 00:00:00
abstract:OBJECTIVE:Using the gamma-butyrolactone (GBL) model of absence seizures in Long-Evans rats, this study investigated if 2.5-6 Hz paroxysmal discharges (PDs) induced by GBL were synchronized among the thalamocortical system and the hippocampus, and whether inactivation of the hippocampus affected PDs. METHODS:Local fiel...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2015.12.007
更新日期:2016-02-01 00:00:00
abstract:PURPOSE:To identify clinical risk factors for Dravet syndrome (DS) in a population of children with status epilepticus (SE). MATERIAL AND METHODS:Children aged between 1 month and 16 years with at least one episode of SE were referred from 6 pediatric neurology centers in Switzerland. SE was defined as a clinical seiz...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2014.02.019
更新日期:2014-05-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.eplepsyres.2016.08.025
更新日期:2016-11-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2011.09.013
更新日期:2012-02-01 00:00:00
abstract:OBJECTIVE:This retrospective study addresses the cost-effectiveness of add-on therapy with lamotrigine in clinical practice. METHODS:Two years' observational data of 165 patients were used. Seizure frequency, adverse effects and direct medical costs were recorded for the year before and the year after the start of lam...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2005.09.007
更新日期:2005-12-01 00:00:00
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
doi:10.1016/j.eplepsyres.2011.01.010
更新日期:2011-03-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2003.08.001
更新日期:2003-09-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2020.106479
更新日期:2020-11-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2011.12.010
更新日期:2012-05-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/s0920-1211(02)00107-9
更新日期:2002-09-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/s0920-1211(97)00068-5
更新日期:1998-01-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2014.09.025
更新日期:2014-12-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2010.10.009
更新日期:2011-01-01 00:00:00
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journal_title:Epilepsy research
pub_type: 临床试验,杂志文章
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更新日期:2011-10-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/s0920-1211(00)00120-0
更新日期:2000-08-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2011.08.012
更新日期:2012-01-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章,评审
doi:10.1016/j.eplepsyres.2020.106304
更新日期:2020-05-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2011.05.017
更新日期:2011-09-01 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
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/0920-1211(92)90049-y
更新日期:1992-04-01 00:00:00
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journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2015.08.012
更新日期:2015-11-01 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:OBJECTIVE:In Rasmussen syndrome (RS), in addition to the predominant involvement of cytotoxic T cells, heterogeneous autoantibodies against neural molecules are also found, but their function has not been elucidated. We examined antibodies to N-methyl-d-aspartate (NMDA) type glutamate receptor (GluR) subunits (GluN2B &...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2015.03.004
更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:Epilepsy is among the more stigmatising diseases, leading to a negative impact on the quality of life (QoL) of people with epilepsy (PWE). Assessment of the QoL and stigma in PWE reflects the outcome of their disease, and the findings can be used to improve the management of epilepsy. To fill a gap in the li...
journal_title:Epilepsy research
pub_type: 杂志文章
doi:10.1016/j.eplepsyres.2020.106437
更新日期:2020-11-01 00:00:00