Abstract:
PURPOSE:To characterize seizure types and electroencephalographic features of glucose transporter type 1 deficiency syndrome (Glut-1 DS). METHODS:Twenty children with clinical and laboratory features of Glut-1 DS were evaluated. Age at seizure diagnosis, seizure classification, and response to treatment were determined by chart review. Thirty-two continuous 24-h EEG monitoring sessions and reports of 42 routine EEG studies were assessed. RESULTS:Age at seizure diagnosis was between 4 weeks and 18 months (mean, 5 months). Seizure types were generalized tonic or clonic (14), absence (10), partial (nine), myoclonic (six), or astatic (four). During 24-h EEGs, background activity showed generalized 2.5- to 4-Hz spike-wave discharges (41%), generalized slowing or attenuation (34%), no abnormalities (34%), focal epileptiform discharges (13%), or focal slowing or attenuation (9%). No seizures were captured during 69% of the studies; the remainder had absence (19%), myoclonic (9%), or partial seizures (3%). On evaluation of routine and 24-h EEG studies, focal epileptiform discharges (24%) and slowing (11%) were more frequent in ages 0-24 months. In older children (2-8 years), generalized epileptiform discharges (37.5%) and slowing (21%) were more common. CONCLUSIONS:In all ages, a normal interictal EEG was the most common EEG finding. When abnormalities occurred, focal slowing or epileptiform discharges were more prevalent in the infant. In older children (2 years or older), a generalized 2.5- to 4-Hz spike-wave pattern emerged. Seizure types observed included, absence, myoclonic, partial, and astatic.
journal_name
Epilepsiajournal_title
Epilepsiaauthors
Leary LD,Wang D,Nordli DR Jr,Engelstad K,De Vivo DCdoi
10.1046/j.1528-1157.2003.05302.xsubject
Has Abstractpub_date
2003-05-01 00:00:00pages
701-7issue
5eissn
0013-9580issn
1528-1167pii
epi05302journal_volume
44pub_type
杂志文章相关文献
EPILEPSIA文献大全abstract:PURPOSE:To examine which variables available early in the course of childhood epilepsy are associated with a poor short-term outcome and to develop models to predict such an outcome. METHODS:We prospectively followed up 466 children with newly diagnosed epilepsy for 2 years. Variables were collected at intake and afte...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1157.1999.tb00770.x
更新日期:1999-06-01 00:00:00
abstract::A 30-year-old man with long-standing localization-related epilepsy and mental retardation had seizures that were partially controlled with valproate (VPA) 500 mg four times daily. Routine examination showed severe thrombocytopenia with mild leukopenia and chronic low-grade hemolytic anemia. Pertinent laboratory result...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1157.1994.tb02927.x
更新日期:1994-01-01 00:00:00
abstract::Scrub typhus is an emerging infection, and there is little information about status epilepticus (SE) in scrub typhus. We report the clinical spectrum and outcome of SE in scrub typhus. In a 3-year prospective hospital-based observational study, all scrub typhus patients with SE were included. Scrub typhus was diagnose...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/epi.13412
更新日期:2016-07-01 00:00:00
abstract:PURPOSE:Intracerebral vascular malformations including cavernous angiomas (CAs) and arteriovenous malformations (AVMs) are an important cause of chronic pharmacoresistant epilepsies. Little is known about the pathogenetic basis of epilepsy in patients with vascular malformations. Intracerebral deposits of iron-containi...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1167.2012.03405.x
更新日期:2012-03-01 00:00:00
abstract:PURPOSE:Some evidence suggests that levetiracetam (LEV) possesses antiepileptogenic characteristics. The purpose of this study was to investigate the time course of seizure protection by LEV compared with that of phenytoin (PHT), phenobarbital (PB), valproate (VPA), and carbamazepine (CBZ) in the spontaneously epilepti...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1167.2005.29604.x
更新日期:2005-09-01 00:00:00
abstract:PURPOSE:To investigate the self-reported quality of life (QOL) of children and adolescents with new-onset epilepsy and explore parent's perceptions of their child's QOL and the impact of epilepsy on the family. METHODS:As part of the Standard and New Antiepileptic Drug (SANAD) trial, 248 children (aged 8-15 years) wit...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1167.2011.03094.x
更新日期:2011-08-01 00:00:00
abstract:OBJECTIVE:Drebrins are crucial for synaptic function and dendritic spine development, remodeling, and maintenance. In temporal lobe epilepsy (TLE) patients, a significant hippocampal synaptic reorganization occurs, and synaptic reorganization has been associated with hippocampal hyperexcitability. This study aimed to e...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/epi.16595
更新日期:2020-08-01 00:00:00
abstract:PURPOSE:Synaptic vesicle protein 2A (SV2A), the binding site for the antiepileptic drug (AED) levetiracetam, has been shown to be involved in the control of neuronal excitability. The aim of the study was to define the expression and cell-specific distribution of SV2A in developmental focal lesions associated with medi...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1167.2008.01955.x
更新日期:2009-06-01 00:00:00
abstract::Status epilepticus (SE) is associated with significant mortality and morbidity. A reliable prognosis may help better manage medical resources and treatment strategies. We examined the role of preexisting comorbidities on the outcome of patients with SE, an aspect that has received little attention to date. We prospect...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1167.2012.03451.x
更新日期:2012-05-01 00:00:00
abstract::This chapter covers the syndromes of benign epilepsy with centrotemporal spikes (BECTS), nonlesional focal epilepsy in otherwise normal children (NLFN), and the genetic generalized epilepsies. BECTS is an epilepsy syndrome that always enters terminal remission before the general age of a planned transition of adolesce...
journal_title:Epilepsia
pub_type: 杂志文章,评审
doi:10.1111/epi.12706
更新日期:2014-08-01 00:00:00
abstract::We evaluated language functions in 154 patients with left hemisphere speech dominance undergoing anterior temporal lobectomy (ATL). Measures of phonemic and semantic fluency, confrontation naming, repetition, comprehension, and reading were administered before and 3 weeks postoperatively. Patients were grouped by focu...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1157.1995.tb00464.x
更新日期:1995-11-01 00:00:00
abstract::Concentrations of prostaglandins (PGs) in the central nervous system are known to increase during and after experimentally induced seizures. In the present study, concentrations of PGE-2 were determined by radioimmunoassay in lumbar cerebrospinal fluid (CSF) of 17 children shortly after a febrile convulsion. PGE-2 dat...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1157.1988.tb03724.x
更新日期:1988-05-01 00:00:00
abstract::We report 10 seizure-related thermal injuries severe enough to require hospitalization in patients with epilepsy. Eight of the ten incidents were with patients who had had seizures with impaired consciousness two or more times a month. This suggests that seizure frequency is a risk factor and implies the importance of...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1157.1992.tb02290.x
更新日期:1992-01-01 00:00:00
abstract::Epilepsy was defined conceptually in 2005 as a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures. This definition is usually practically applied as having two unprovoked seizures >24 h apart. The International League Against Epilepsy (ILAE) accepted recommendations of a t...
journal_title:Epilepsia
pub_type: 杂志文章,评审
doi:10.1111/epi.12550
更新日期:2014-04-01 00:00:00
abstract::Seizures cannot be medically controlled in approximately 40% of people with epilepsy. Although we are beginning to understand how to better treat certain seizure types, we still do not know the regulatory events that determine antiepileptic drug resistance. Proposed pathoetiologic mechanisms include altered expression...
journal_title:Epilepsia
pub_type: 杂志文章,评审
doi:10.1111/epi.12183
更新日期:2013-05-01 00:00:00
abstract::The epilepsy community increasingly recognizes the need for a modern classification system that can also be easily integrated with effective informatics tools. The 2010 reports by the United States President's Council of Advisors on Science and Technology (PCAST) identified informatics as a critical resource to improv...
journal_title:Epilepsia
pub_type: 杂志文章,评审
doi:10.1111/epi.12211
更新日期:2013-08-01 00:00:00
abstract::The Epilepsy Association of Maryland, an affiliate of the Epilepsy Foundation of America (EFA), recognized that the Maryland state regulations and laws on driving licensing restricted the rights of individuals with epilepsy. After identifying the problem, the Association determined the needed changes and planned a leg...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1157.1994.tb02494.x
更新日期:1994-05-01 00:00:00
abstract:PURPOSE:Univerricht-Lundborg disease (ULD), with its major symptom of action myoclonus, is supposed to be very rare in the Netherlands and western Europe. We hypothesized that the syndrome may be underdiagnosed in patients with myoclonus epilepsy. METHODS:Mutation analysis of the cystatin B gene was performed in 21 ca...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.0013-9580.2004.43703.x
更新日期:2004-09-01 00:00:00
abstract::Idiopathic generalized epilepsies, i.e., juvenile myoclonic epilepsy (JME), childhood absence epilepsy, and epilepsy with grand mal [generalized tonic-clonic seizures (GTCS)], are the most common genetic epilepsies. Linkage studies using Bf, HLA serologic, and DNA markers by three independent investigators, one from L...
journal_title:Epilepsia
pub_type: 杂志文章,评审
doi:10.1111/j.1528-1157.1990.tb05855.x
更新日期:1990-01-01 00:00:00
abstract:PURPOSE:To study the neurophysiologic characteristics of epileptic myoclonus in patients with Lennox-Gastaut syndrome (LGS) and myoclonic astatic epilepsy (MAE). METHODS:Three patients with symptomatic LGS (mean age, 15 years +/- 4) and three with cryptogenic MAE (mean age, 9 years +/- 3) were studied. Temporal relati...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1046/j.1528-1157.2002.30301.x
更新日期:2002-06-01 00:00:00
abstract::The International League Against Epilepsy (ILAE) presents a revised operational classification of seizure types. The purpose of such a revision is to recognize that some seizure types can have either a focal or generalized onset, to allow classification when the onset is unobserved, to include some missing seizure typ...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/epi.13670
更新日期:2017-04-01 00:00:00
abstract::Polypharmacy is a widely employed treatment strategy in epilepsy, particularly for individuals with poorly controlled seizures. Drug combinations should be carefully considered to minimize the potential for unfavorable interactions. Older-generation antiepileptic drugs (AEDs) are well known for their pharmacokinetic i...
journal_title:Epilepsia
pub_type: 杂志文章,评审
doi:10.1111/j.1528-1167.2007.00983.x
更新日期:2007-03-01 00:00:00
abstract::There is no doubt that structural morphological brain lesions and malformations in epilepsy represent major etiological factors for the cognitive impairments seen in this disease. The role of epileptic activity and seizures for cognition and cognitive development, however, is less easily determined. Epileptic dysfunct...
journal_title:Epilepsia
pub_type: 杂志文章,评审
doi:10.1111/j.1528-1167.2007.01360.x
更新日期:2007-01-01 00:00:00
abstract:PURPOSE:Studies using magnetic resonance imaging have shown that reduced hippocampal volume is associated with a history of febrile seizures, the duration of epilepsy, and the number of generalized tonic-clonic seizures. It is uncertain whether these factors have the same influence on functional as on structural measur...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.0013-9580.2004.51803.x
更新日期:2004-03-01 00:00:00
abstract::Since the clinical data have been equivocal in regard to the effects of clonazepam (CZP) in focal-motor seizures, an alumina gel monkey model was used to evaluate quantitatively its efficacy with respect to this seizure category. The insolubility of CZP and its short biological half-life in monkey necessitated its eva...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1157.1979.tb04852.x
更新日期:1979-12-01 00:00:00
abstract::The emergency treatment of seizures is an important practical issue, especially the treatment of generalized convulsive status epilepticus (GCSE). Benzodiazepines or older standard antiepileptic drugs (phenobarbital, phenytoin) have typically been used as initial intravenous treatment of GCSE. As new parenteral antiep...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1167.2008.01929.x
更新日期:2008-12-01 00:00:00
abstract::We examined auto-correlation and interval distribution characteristics of neuronal discharge from patients with complex partial seizures. The objective was to compare the interictal firing patterns of neurons in mesial temporal structures ipsilateral to the site of seizure onset with firing patterns of neurons in homo...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/j.1528-1157.1996.tb00001.x
更新日期:1996-02-01 00:00:00
abstract::As new antiepileptic drugs (AEDs) become available, physicians will define their appropriate use in particular patient populations. For women, the issues include gender-specific efficacy and tolerability, including the impact of the AED on reproductive health. Women with epilepsy who are treated with established AEDs ...
journal_title:Epilepsia
pub_type: 杂志文章,评审
doi:10.1111/j.1528-1157.1996.tb06037.x
更新日期:1996-01-01 00:00:00
abstract:OBJECTIVE:A large number of studies have highlighted the important role of the gut microbiota in the pathophysiology of neurological disorders, suggesting that its manipulation might serve as a treatment strategy. We hypothesized that the gut microbiota participates in absence seizure development and maintenance in the...
journal_title:Epilepsia
pub_type: 杂志文章
doi:10.1111/epi.16813
更新日期:2021-01-11 00:00:00
abstract:PURPOSE:Polymicrogyria (PMG) is an epileptogenic malformation of cortical development. We describe the clinical epilepsy and imaging features of a large cohort with PMG-related epilepsy. METHODS:Participants were recruited through the Epilepsy Phenome/Genome Project, a multicenter collaborative effort to collect detai...
journal_title:Epilepsia
pub_type: 杂志文章,多中心研究
doi:10.1111/epi.12238
更新日期:2013-08-01 00:00:00