The natural history of epilepsy in tuberous sclerosis complex.

Abstract:

BACKGROUND:Although epilepsy affects most patients with tuberous sclerosis complex (TSC), little is known about the natural history of epilepsy in this genetic disease. METHODS:A retrospective chart review of all patients with TSC seen between January 2002 and October 2008. Charts were reviewed for a history of infantile spasms (IS), seizure other than IS, refractory epilepsy, Lennox-Gastaut syndrome (LGS), anticonvulsant medication use, ages of seizure onset, last seizure, last clinic visit, clinical seizure phenotype(s), cognitive impairment, and genetic mutation. RESULTS:Two hundred ninety-one patients were included. Among these patients, 37.8% had a history of IS; 85.2% had a history of seizure; 54.1% developed multiple seizure types, not including IS; 63.2% had seizure onset in the first year of life; and 12.1% of adults without a seizure history developed epilepsy. Of epilepsy patients, 62.5% developed refractory epilepsy and 33.5% achieved epilepsy remission; 37.5% of these patients achieved medication freedom. IS was a risk factor for refractory epilepsy (p<0.0001) and LGS (p<0.0001). History of seizure, IS, age at seizure onset, and refractory epilepsy each correlated with poor cognitive outcome (p<0.0001). Epilepsy remission correlated with better cognitive outcome (p<0.0001). TSC2 was a risk factor for IS and epilepsy; patients without an identified mutation were more likely to achieve remission. CONCLUSION:Most patients with TSC develop epilepsy and most develop multiple seizure types. Onset typically occurs in the first year of life; however, adults remain at risk. Although refractory epilepsy is common, many patients achieve seizure control. Many features of seizure history are predictive of cognitive and epilepsy outcome.

journal_name

Epilepsia

journal_title

Epilepsia

authors

Chu-Shore CJ,Major P,Camposano S,Muzykewicz D,Thiele EA

doi

10.1111/j.1528-1167.2009.02474.x

subject

Has Abstract

pub_date

2010-07-01 00:00:00

pages

1236-41

issue

7

eissn

0013-9580

issn

1528-1167

pii

EPI2474

journal_volume

51

pub_type

杂志文章
  • A case with atypical childhood occipital epilepsy "Gastaut type": an ictal migraine manifestation with a good response to intravenous diazepam.

    abstract::We report the history of a 14-year-old girl with atypical childhood occipital epilepsy "Gastaut type" whose first generalized tonic-clonic seizure was preceded by migraine without aura and followed by a status migrainosus. This status lasted for 3 days despite standard analgesic therapy. An EEG recording revealed an o...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1167.2007.01265.x

    authors: Parisi P,Kasteleijn-Nolst Trenité DG,Piccioli M,Pelliccia A,Luchetti A,Buttinelli C,Villa MP

    更新日期:2007-11-01 00:00:00

  • Surgery for intractable infantile spasms: neuroimaging perspectives.

    abstract::Twenty-three infants and children underwent cortical resection (n = 15) or hemispherectomy (n = 8) for intractable infantile spasms. Infantile spasms were present at the time of surgery in 17 of the 23 patients; in six, spasms had evolved to other seizure types during surgical evaluation. Children with a remote histor...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1993.tb00459.x

    authors: Chugani HT,Shewmon DA,Shields WD,Sankar R,Comair Y,Vinters HV,Peacock WJ

    更新日期:1993-07-01 00:00:00

  • Early onset myoclonic epilepsy and 15q26 microdeletion: observation of the first case.

    abstract::The authors report the study of a 30-month-old girl with refractory myoclonic epilepsy associated with mental retardation, growth delay, peculiar facial appearance, and minor physical anomalies. Extensive genetic studies were performed, including an array-based comparative genomic hybridization (array-CGH) that showed...

    journal_title:Epilepsia

    pub_type: 古典文章,杂志文章

    doi:10.1111/j.1528-1167.2009.02078.x

    authors: Veredice C,Bianco F,Contaldo I,Orteschi D,Stefanini MC,Battaglia D,Lettori D,Guzzetta F,Zollino M

    更新日期:2009-07-01 00:00:00

  • Temporal lobe epilepsy surgery and the quest for optimal extent of resection: a review.

    abstract::The efficacy of surgery to treat drug-resistant temporal lobe epilepsy (TLE) has been demonstrated in a prospective randomized trial. It remains controversial which resection method gives best results for seizure freedom and neuropsychological function. This review of 53 studies addressing extent of resection in surge...

    journal_title:Epilepsia

    pub_type: 杂志文章,评审

    doi:10.1111/j.1528-1167.2008.01604.x

    authors: Schramm J

    更新日期:2008-08-01 00:00:00

  • Febrile seizures: clinical characteristics and initial EEG.

    abstract::We examined the relationship between clinical characteristics and EEG classification in all children with febrile seizures examined at the University Pediatric Clinic, Skopje, Yugoslavia between 1982 and 1984. This is the only facility in Macedonia providing EEG or neurologic consultation for children. EEGs were class...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1992.tb02282.x

    authors: Sofijanov N,Emoto S,Kuturec M,Dukovski M,Duma F,Ellenberg JH,Hirtz DG,Nelson KB

    更新日期:1992-01-01 00:00:00

  • Low-pressure hydrocephalic state complicating hemispherectomy: a case report.

    abstract:UNLABELLED:Low-pressure hydrocephalic state (LPHS) has only recently been described as a distinct clinical entity occurring in patients with bioatrophic lesions of the brain. We report a patient in whom this syndrome developed after subtotal hemispherectomy for intractable epilepsy. METHODS:A 30-year-old man developed...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1046/j.1528-1157.2002.46101.x

    authors: Daniel RT,Lee GY,Halcrow SJ

    更新日期:2002-05-01 00:00:00

  • Vigabatrin: clinical evidence supporting rational polytherapy in management of uncontrolled seizures.

    abstract::Monotherapy is the policy for management of patients with epilepsy. With increasing knowledge of the biology of epilepsy and of the modes of action of antiepileptic drugs (AEDs), this concept must be reevaluated. When monotherapy fails to control seizures, subsequent treatment should be based on "rational pharmacology...

    journal_title:Epilepsia

    pub_type: 临床试验,杂志文章

    doi:10.1111/j.1528-1157.1994.tb02447.x

    authors: de Bittencourt PR,Mazer S,Marcourakis T,Bigarella MM,Ferreira ZS,Mumford JP

    更新日期:1994-03-01 00:00:00

  • A guinea pig model of mesial temporal lobe epilepsy following nonconvulsive status epilepticus induced by unilateral intrahippocampal injection of kainic acid.

    abstract:PURPOSE:  Models of temporal lobe epilepsy are commonly utilized to study focal epileptogenesis and ictogenesis. The criteria that define animal models representative of human mesial temporal lobe may vary in different laboratories. We describe herein a focal epilepsy model of mesial temporal (hippocampal) origin that ...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1167.2012.03669.x

    authors: Carriero G,Arcieri S,Cattalini A,Corsi L,Gnatkovsky V,de Curtis M

    更新日期:2012-11-01 00:00:00

  • Effects of valproate on acylcarnitines in children with epilepsy using ESI-MS/MS.

    abstract:PURPOSE:To determine the influence of valproate (VPA) treatment on acylcarnitines in children with epilepsy. METHODS:Determination of acylcarnitines (including free carnitine and acylcarnitines from C2 to C18) in dried blood spot specimens using tandem-mass spectrometry. Longitudinal study of changes in acylcarnitines...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1167.2006.00833.x

    authors: Werner T,Treiss I,Kohlmueller D,Mehlem P,Teich M,Longin E,Gerstner T,Koenig SA,Schulze A

    更新日期:2007-01-01 00:00:00

  • Role of the intracarotid amobarbital-pentylenetetrazol EEG test in the diagnosis and surgical treatment of patients with complex seizure problems.

    abstract::The results of surgical treatment of intractable epilepsy seizures in a group of patients investigated with the intracarotid amobarbital-pentylenetetrazol EEG test were analyzed. This diagnostic test, originally designed to distinguish a mechanism of primary from that of secondary bilateral synchrony in the EEG was al...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1976.tb03377.x

    authors: Gloor P,Rasmussen T,Altuzarra A,Garretson H

    更新日期:1976-03-01 00:00:00

  • Mechanisms of video-game epilepsy.

    abstract:PURPOSE:We aimed to elucidate the mechanisms underlying video-game epilepsy by comparing the flicker- and spatial-frequency ranges over which photic and pattern stimulation elicited photoparoxysmal responses in two different populations: (a) 25 patients with a history of seizures experienced while playing video games; ...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1999.tb00903.x

    authors: Fylan F,Harding GF,Edson AS,Webb RM

    更新日期:1999-01-01 00:00:00

  • Pharmacokinetic and pharmacodynamic analysis of (2S,3S)-sec-butylpropylacetamide (SPD) in rats and pigs-A CNS-active stereoisomer of SPD.

    abstract:OBJECTIVES:To advance the development of (2S,3S)-sec-butylpropylacetamide (SPD) as a new treatment for acute repetitive seizures (ARS), by studying its pharmacokinetics (PK) in pigs and its PK-pharmacodynamic (PK-PD) correlation in rats. METHODS:Two (2S,3S)-SPD intramuscular formulations (FA and FB ) were administered...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/epi.16411

    authors: Bibi D,Bialer M

    更新日期:2020-01-01 00:00:00

  • Extracellular magnesium and anticonvulsant effects of valproate in young rat hippocampus.

    abstract::Extracellular field potential recordings were made in CA3 subfield of hippocampal slices from rats aged 11-22 days. In these experiments, we analyzed the effects induced by modifying [Mg2+] in the medium (1 or 2 mM) on (a) 4-aminopyridine (4-AP, 50 microM)-induced synchronous events (including ictal- and interictal-li...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1995.tb01016.x

    authors: Fueta Y,Siniscalchi A,Tancredi V,Avoli M

    更新日期:1995-04-01 00:00:00

  • MR spectroscopy shows reduced frontal lobe concentrations of N-acetyl aspartate in patients with juvenile myoclonic epilepsy.

    abstract:PURPOSE:Neuropsychological studies suggest frontal lobe dysfunctions in patients with juvenile myoclonic epilepsy (JME). In this study we investigated whether an underlying mechanism could be a regional neuronal damage not visible with structural magnetic resonance (MR), but detectable with magnetic resonance spectrosc...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.2000.tb00158.x

    authors: Savic I,Lekvall A,Greitz D,Helms G

    更新日期:2000-03-01 00:00:00

  • Ictal and interictal activity in partial epilepsy recorded with multichannel magnetoelectroencephalography: correlation of electroencephalography/electrocorticography, magnetic resonance imaging, single photon emission computed tomography, and positron em

    abstract::Ictal and interictal epileptic activity was recorded for the first time by multichannel magnetoencephalography (MEG) in three patients with partial epilepsy. Pre- and intra-operative localization of the epileptogenic region was compared. The interictal epileptic activity was localized at the same region of the tempora...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1992.tb02195.x

    authors: Stefan H,Schneider S,Feistel H,Pawlik G,Schüler P,Abraham-Fuchs K,Schlegel T,Neubauer U,Huk WJ

    更新日期:1992-09-01 00:00:00

  • Double pathology in Rasmussen's encephalitis: etiologic considerations.

    abstract::In a 7-year-old girl with epilepsia partialis continua (EPC) involving the left face, arm, and leg for 1 year, serial neuroimaging studies showed progressive, brain atrophy. Because medical treatment was ineffective, she underwent a large fronto-temporal surgical resection. Neuropathological examination showed loss of...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1996.tb00597.x

    authors: Yacubian EM,Rosemberg S,Marie SK,Valério RM,Jorge CL,Cukiert A

    更新日期:1996-05-01 00:00:00

  • Resective surgery for intractable focal epilepsy in patients with low IQ: predictors for seizure control and outcome with respect to seizures and neuropsychological and psychosocial functioning.

    abstract:PURPOSE:To investigate possible predictive factors for seizure control in a group of children and adults with low IQs (IQ, < or =70) who underwent resective surgery for intractable focal epilepsy and to study outcome with respect to seizures and neuropsychological functioning. We also studied psychosocial outcome in th...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.0013-9580.2004.34003.x

    authors: Bjørnaes H,Stabell KE,Heminghyt E,Røste GK,Bakke SJ

    更新日期:2004-02-01 00:00:00

  • A definition and classification of status epilepticus--Report of the ILAE Task Force on Classification of Status Epilepticus.

    abstract::The Commission on Classification and Terminology and the Commission on Epidemiology of the International League Against Epilepsy (ILAE) have charged a Task Force to revise concepts, definition, and classification of status epilepticus (SE). The proposed new definition of SE is as follows: Status epilepticus is a condi...

    journal_title:Epilepsia

    pub_type: 历史文章,杂志文章,评审

    doi:10.1111/epi.13121

    authors: Trinka E,Cock H,Hesdorffer D,Rossetti AO,Scheffer IE,Shinnar S,Shorvon S,Lowenstein DH

    更新日期:2015-10-01 00:00:00

  • Methsuximide lowers lamotrigine blood levels: A pharmacokinetic antiepileptic drug interaction.

    abstract:PURPOSE:To determine whether methsuximide (MSM) affects lamotrigine (LTG) blood levels and whether any change is of clinical significance. METHODS:LTG serum levels in 16 patients taking MSM were compared with those before starting or after stopping the MSM. The 16 patients, (11 boys, five girls) were young people (mea...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.2000.tb00218.x

    authors: Besag FM,Berry DJ,Pool F

    更新日期:2000-05-01 00:00:00

  • Clinical evolution and prognosis of childhood epilepsies.

    abstract::Five-hundred twelve epileptic children were followed longitudinally for several years. The 2- and 4-year remission statuses at the latest examination were 50.6 and 44.2%, respectively. Dividing our sample into groups showing onset of seizures in infancy or at 2-3, 4-7, 8-10, or 11-14 years, the remission rates were as...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1982.tb05053.x

    authors: Sofijanov NG

    更新日期:1982-02-01 00:00:00

  • Quality of life in psychogenic nonepileptic seizures.

    abstract:PURPOSE:Psychogenic nonepileptic seizures (PNESs) are events that alter or seem to alter the neurologic function and, in their appearance, resemble epileptic seizures (ESs). In patients with ESs the psychological and medical aspects of epilepsy greatly influence the health-related quality of life (HRQOL). The relation ...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1046/j.1528-1157.2003.35302.x

    authors: Szaflarski JP,Hughes C,Szaflarski M,Ficker DM,Cahill WT,Li M,Privitera MD

    更新日期:2003-02-01 00:00:00

  • The anticonvulsant profile of rufinamide (CGP 33101) in rodent seizure models.

    abstract:PURPOSE:To evaluate the anticonvulsant profile and behavioral toxicity of rufinamide in animal seizure models compared to the established antiepileptic drugs (AEDs): phenytoin, phenobarbital, valproate, and ethosuximide, or vehicle. METHODS:In acute studies of anticonvulsant efficacy, the AEDs were administered via or...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1167.2008.01552.x

    authors: White HS,Franklin MR,Kupferberg HJ,Schmutz M,Stables JP,Wolf HH

    更新日期:2008-07-01 00:00:00

  • Double-blind, placebo-controlled evaluation of cinromide in patients with the Lennox-Gastaut Syndrome. The Group for the Evaluation of Cinromide in the Lennox-Gastaut Syndrome.

    abstract::This study evaluated the effects of cinromide in patients with Lennox-Gastaut Syndrome. No difference between cinromide and placebo was shown in terms of seizure reduction or global evaluations. This study is important, however, because it represents an effort to overcome methodologic difficulties inherent in evaluati...

    journal_title:Epilepsia

    pub_type: 临床试验,杂志文章

    doi:10.1111/j.1528-1157.1989.tb05321.x

    authors:

    更新日期:1989-07-01 00:00:00

  • Nonepileptic seizures in children.

    abstract:PURPOSE:To determine if the clinical characteristics of nonepileptic seizures (NES) are different in children younger than 13 years age as compared to adolescents. METHODS:Retrospective review of medical records and video-EEGs (VEEG) of all patients with NES confirmed on VEEG monitoring was performed. RESULTS:Sixty-e...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1167.2007.01200.x

    authors: Patel H,Scott E,Dunn D,Garg B

    更新日期:2007-11-01 00:00:00

  • Zonisamide in epilepsy: a pilot study.

    abstract::We compared zonisamide monotherapy (12 weeks) to carbamazepine monotherapy (12 weeks) after phenytoin baseline monotherapy (8 weeks) in an open crossover pilot study of eight adults with uncontrolled partial seizures. Zonisamide had definite antiepileptic activity in five subjects. In two of these, response to zonisam...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1985.tb05408.x

    authors: Wilensky AJ,Friel PN,Ojemann LM,Dodrill CB,McCormick KB,Levy RH

    更新日期:1985-05-01 00:00:00

  • Toxocara infection and epilepsy in children: a case-control study.

    abstract::A case-control study of 305 children was done to evaluate the relationship between toxocariasis and seizures and risk factors associated with toxocariasis transmission and seizure onset. A significant association existed between seropositivity for anti-Toxocara canis and seizures (p less than 0.05); the correlation wa...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1990.tb05356.x

    authors: Arpino C,Gattinara GC,Piergili D,Curatolo P

    更新日期:1990-01-01 00:00:00

  • Regional expression of multidrug resistance genes in genetically epilepsy-prone rat brain after a single audiogenic seizure.

    abstract:PURPOSE:The multidrug resistance (mdr) gene family encodes the drug transport macromolecule P-glycoprotein (P-gp), which contributes to the functionality of the blood-brain barrier. Recent evidence suggests that P-gp-mediated drug extrusion may play a facilitatory role in refractory epilepsy. We investigated the region...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1046/j.1528-1157.2002.156702.x

    authors: Kwan P,Sills GJ,Butler E,Gant TW,Meldrum BS,Brodie MJ

    更新日期:2002-11-01 00:00:00

  • Monotherapy or polytherapy for epilepsy revisited: a quantitative assessment.

    abstract::Some investigators argue that treating epilepsy with several antiepileptic drugs (AEDs) simultaneously (polytherapy) may give rise to more adverse effects than monotherapy, but this argument lacks supporting quantitative data. To reexamine this issue, we recruited a cohort of patients from the outpatients of the Speci...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1995.tb00484.x

    authors: Lammers MW,Hekster YA,Keyser A,Meinardi H,Renier WO,van Lier H

    更新日期:1995-05-01 00:00:00

  • Effects of hippocampal afterdischarges on Purkinje cell activity.

    abstract::Hippocampal afterdischarges can induce increased Purkinje cell activity that characteristically shows a pronounced acceleration during the period of the afterdischarges. Increased Purkinje cell activity may continue for many seconds after termination of the hippocampal activity. Typical frequency histograms are presen...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1157.1975.tb06053.x

    authors: Mitra J,Snider RS

    更新日期:1975-06-01 00:00:00

  • Focal cortical dysplasia type IIb: completeness of cortical, not subcortical, resection is necessary for seizure freedom.

    abstract:PURPOSE:Focal cortical dysplasia type IIb (FCD IIb) lesions are highly epileptogenic and frequently cause pharmacoresistant epilepsy. Complete surgical resection leads to seizure freedom in most cases. However, the term "complete" resection is controversial with regard to the necessity of performing resections of the s...

    journal_title:Epilepsia

    pub_type: 杂志文章

    doi:10.1111/j.1528-1167.2011.03158.x

    authors: Wagner J,Urbach H,Niehusmann P,von Lehe M,Elger CE,Wellmer J

    更新日期:2011-08-01 00:00:00