Purpose:? To estimate the lifetime prevalence of epilepsy in Prey Veng province (Cambodia). Methods:? Door-to-door screening was performed using a random cluster survey whereby all people >1?year of age were screened for epilepsy by using a validated and standardized questionnaire for epilepsy in tropical countries. Suspected ...
PubMed
Heterotopic grey matter is an abnormality of neuronal migration that has been reported in association with refractory epilepsy. In this study we reviewed the magnetic resonance (MR) imaging records of all patients undergoing MR scanning for evaluation of intractable epilepsy and identified sixteen patients who had grey matter ...
Recent data have reported the effectiveness of levetiracetam (LVT) on generalized seizures. Among them, it seems that LVT can be successfully used to treat absence seizures. Many antiepileptic drugs (AEDs) have been occasionally reported to paradoxically aggravate some seizures. We retrospectively identified patients with aggravation of ...
Epilepsy is a form of brain disorder caused by abnormal discharges of neurons. The most common manifestations of epilepsy are seizures which could affect visual, aural and motor abilities of a person. Absence epilepsy is a form of epilepsy common mostly in children. The most common ...
NASA Astrophysics Data System (ADS)
PurposeTo characterize patterns and determinants of normal and abnormal cognitive development in children with new onset epilepsy compared to healthy controls.MethodsLongitudinal (2-year) cognitive growth was examined in 100 children, age 8-18 years, including healthy controls (n=48) and children with new onset epilepsy (n=52). Cognitive maturation was ...
PubMed Central
Epilepsy is a complex neurological disease. Currently ~20 genetic variants are known to cause Mendelian forms of human epilepsy, leaving a vast heritability undefined with future hopes resting on candidate gene resequencing and/or large scale genome-wide association studies. Rodent models for genetically complex epilepsy have been ...
The idiopathic generalized epilepsies (IGEs), constituting approximately a quarter of all epilepsy cases, are presumed to arise primarily from genetic abnormalities. A minority of cases have been identified to be caused by mutations in a single gene, but in the vast majority, mutations in multiple genes are presumed to contribute to ...
Fifteen cases of juvenile myoclonic epilepsy (JME) were identified from one hundred and eighty consecutive patients referred to a new epilepsy clinic at St Thomas' Hospital between April 1989 and December 1990, a prevalence of 8.3%. Of these, only one was referred with a putative diagnosis of JME. Diagnosis of the other patients on ...
Absence of the corpus callosum is often associated with cognitive deficits, autism, and epilepsy. Using a genomic microarray, the authors analyzed DNA from 25 patients with radiographically confirmed callosal anomalies and identified three patients with de novo copy number changes in chromosome regions 2q37, 6qter, and 8p. Chromosomal ...
One quadrillion synapses are laid in the first two years of postnatal construction of the human brain, which are then pruned until age 10 to 500 trillion synapses composing the final network. Genetic epilepsies are the most common neurological diseases with onset during pruning, affecting 0.5% of 2�10-year-old children, and these epilepsies are often ...
Mutations in doublecortin (DCX) are associated with intractable epilepsy in humans, due to a severe disorganization of the neocortex and hippocampus known as classical lissencephaly. However, the basis of the epilepsy in lissencephaly remains unclear. To address potential functional redundancy with murin Dcx, we targeted one of the closest homologues, ...
We have studied 124 children with typical absence epilepsy. The onset of symptoms was in 12% under 4 years, in 51% between 4-8 years and in 37% above 8 years. The F:M ratio was 2:1 in children under 4 years versus 1:1 above 8 years. Absences alone occurred in 82% and absences followed or preceded by generalized ...
... or the child reached the maximal allowable or highest tolerated dose. The study�s primary outcome goal was ... segment of society, by people all over the world. Cincinnati Children�s Hospital Medical Center ( www.cincinnatichildrens.org ) ...
MedlinePLUS
... Electroencephalogram (EEG) is utilized to diagnose epilepsy by determining the presence or absence of epileptiform discharges (ED). ...
DTIC Science & Technology
GLUT-1 deficiency syndrome (GLUT-1 DS) is a disorder of cerebral glucose transport associated with early infantile epilepsy and microcephaly. We report two boys who presented with refractory absence epilepsy associated with hypoglycorrhachia, both of whom have genetically confirmed GLUT-1 DS. We propose that these children serve to ...
A developmentally normal adolescent boy with a history of childhood absence epilepsy presented with recurrence of pyknolepsy after a seven-year period of remission. The characteristics of his EEG showed the same 3-Hz generalised spike-wave discharge as in his previous EEG in childhood. To our knowledge, this is the first case report describing recurrence ...
Mutations in the GABRB3 have been recently associated with childhood absence epilepsy (CAE) in families from Honduras and Mexico. In this study, we aimed to determine the frequency of mutation in this gene in our cohort of families with CAE and other related idiopathic generalized epilepsy (IGE) syndromes. We screened the open reading ...
Aim: The purpose of this review was to examine the possible pathophysiological links between epilepsy, cognition, sleep macro- and microstructure, and sleep disorders to highlight the contributions and interactions of sleep and epilepsy on cognitive functioning in children with epilepsy. Method: PubMed was used as the medical database ...
ERIC Educational Resources Information Center
One quadrillion synapses are laid in the first two years of postnatal construction of the human brain, which are then pruned until age 10 to 500 trillion synapses composing the final network. Genetic epilepsies are the most common neurological diseases with onset during pruning, affecting 0.5% of 2-10-year-old children, and these epilepsies are often ...
Photosensitivity is the most common mode of seizure precipitation. It is age-related, more frequent in females, and most often found in generalised epilepsies. Little is known about its relation to individual epileptic syndromes. This study on 1062 epileptic patients who had 4007 split screen video EEG investigations revealed that the relation to generalised ...
Neocortical seizures are often poorly localized, explosive and widespread at onset, making them poorly amenable to epilepsy surgery in the absence of associated focal brain lesions. We describe, for the first time in an unselected group of patients with neocortical epilepsy, the finding that high-frequency (60--100 Hz) epileptiform ...
SummaryPurposeTo determine the long-term efficacy of anterior temporal lobectomy for medically refractory temporal lobe epilepsy in patients with nonlesional magnetic resonance imaging (MRI).MethodsWe identified a retrospective cohort of 44 patients with a nonlesional modern �seizure protocol� MRI who underwent anterior temporal lobectomy for treatment ...
BACKGROUND: The epileptic syndrome in childhood was described by The International League against Epilepsy (ILAE) for the evaluation and understanding purpose. They defined epilepsy, clinical symptoms and paraclinic manifestations, in relation with the age of onset and prognostic determinants. The etiological classification included the symptomatic group, ...
Fixation-off sensitivity is an electroencephalographic phenomenon characterized by spike-and-wave discharges that only occur when central vision and fixation are eliminated. It is especially seen in children with Panayiotopoulos-type, early-onset, benign childhood occipital epilepsy or Gastaut type, late-onset, childhood occipital epilepsy. It can also be ...
To investigate the outcome of temporal lobe epilepsy surgery and identify the variables which predict a good prognosis with respect to seizures in postoperative follow-up after two and four years. This retrospective study included 115 selected patients who underwent surgery for temporal lobe epilepsy between 1996 and 2007. In the ...
We report an unusual association between idiopathic occipital epilepsy and childhood absence epilepsy in 2 pediatric patients. At first clinical and electroencephalographic evaluation, the patients presented the peculiar signs of idiopathic occipital epilepsy Gastaut type: focal sensory visual seizures, ...
The author first reviews the definition of myoclonia as an epileptic crisis differentiated from tonic crises and infantile spasms. He reviews the prevalence and incidence found in bibliographic data, under the following headings 1. Early myoclonic encephalopathy or neonatal myoclonic encephalopathy 2. Early epileptic syndrome with bursts of suppression or Otahara's syndrome. 3. West's syndrome. 4. ...
... epilepsy - Focus on pregnancy (an evidence-based review): Teratogenesis and perinatal outcomes. Neurology. 2009;73:133. Seizure ...
Idiopathic epilepsies (IEs) are a group of disorders characterized by recurrent seizures in the absence of detectable brain lesions or metabolic abnormalities. IEs include common disorders with a complex mode of inheritance and rare Mendelian traits suggesting the occurrence of several alleles with variable penetrance. We previously described a large ...
Mutations in PCDH19, encoding protocadherin 19 on chromosome X, cause familial epilepsy and mental retardation limited to females or Dravet-like syndrome. Heterozygous females are affected while hemizygous males are spared, this unusual mode of inheritance being probably due to a mechanism called cellular interference. To extend the mutational and clinical spectra associated ...
is seeking companies interested in commercializing a unique method of identifying the neurological patterns, according to the Epilepsy Foundation of America. Epilepsy is one of the most common serious neurological neurological patterns between epileptic seizures and have discovered a method to identify biomarkers
E-print Network
, pediatric epilepsy patients *Eric D. Marsh, *Bradley Peltzer, *Merritt W. Brown III, *Courtney Wusthoff, y of sharps and spikes, interictal epi- leptiform discharges (IEDs), in guiding epilepsy surgery in children of IEEG recorded from 19 children with intractable, mostly extra- temporal lobe epilepsy. Approximately 8
SummaryIntroductionA crucial issue in the genetic analysis of idiopathic generalized epilepsy (IGE) is deciding on the phenotypes that are likely to give the greatest power to detect predisposing variants. A complex inheritance pattern and unclear nature of the genotype�phenotype correlation makes this task difficult. In the absence of much definitive ...
In a chemical mutagenesis screen, we identified the novel Scn8a8J allele of the gene encoding the neuronal voltage-gated sodium channel Nav1.6. The missense mutation V929F in this allele alters an evolutionarily conserved residue in the pore loop of domain 2 of Nav1.6. Electroencephalography (EEG) revealed ...
Absence epilepsy is a neurological disorder that causes a recurrent loss of consciousness and generalized spike-and-wave discharges on an electroencephalogram (EEG). The role of metabotropic glutamate receptors (mGluRs) and associated scaffolding proteins in absence epilepsy has been unclear to date. We ...
Two siblings from a consanguineous Egyptian marriage showed an identical phenotype of cortical lissencephaly with cerebellar hypoplasia, severe epilepsy, and mental retardation. Examination of karyotype revealed 46, t(7;12)(q22;p13)mat (7;12)(q22;p13)pat in both affected children, suggesting a homozygous reciprocal balanced translocation. Each healthy parent was a carrier of ...
BackgroundPatients with epilepsy often suffer from other important conditions. The existence of such co-morbidities is frequently not recognized and their relationship with epilepsy usually remains unexplained.Methodology/Principal FindingsWe describe three patients with common, sporadic, non-syndromic epilepsies in whom large genomic ...
Mutations in inhibitory GABAA receptor subunit genes (GABRA1, GABRB3, GABRG2 and GABRD) have been associated with genetic epilepsy syndromes including childhood absence epilepsy (CAE), juvenile myoclonic epilepsy (JME), pure febrile seizures (FS), generalized epilepsy ...
The attitudes and epilepsy-related knowledge of teachers are an important component of the educational experiences of children with epilepsy. Unfortunately however, the exploration of teacher attitudes and knowledge has been extremely limited in the United States. This article describes a survey-based research study of the attitudes and ...
Of the 50 million people with epilepsy worldwide, around 80% reside in resource-poor countries, which are ill-equipped to tackle the enormous medical, social and economic challenges posed by epilepsy. The capability to identify people with epilepsy and provide cost-effective care is compromised by widespread ...
In the present paper we consider the on-off intermittency phenomena observed in time series of spontaneous paroxysmal activity in rats with genetic absence epilepsy. The method to register and analyze the electroencephalogram with the help of continuous wavelet transform is also suggested.
Energy Citations Database
, T. and Catterall, W.A. (2006) Reduced sodium current in GABAergic interneurons in a mouse model discharges and absence epilepsy in mice Ligia A. Papale1,2,{ , Barbara Beyer3,{ , Julie M. Jones4, Lisa M-wave discharges (SWD), the hallmark of absence epilepsy, in Scn8a8J heterozygotes and in heterozygotes for two
On February 12, 2009, we commemorated the 200th anniversary of Charles Darwin's birth and the 150th anniversary of the publication of the first edition of the 'On the origin of species'. Only in the sixth edition of the Origin Darwin explicitly stated that natural selection applied to the brain as to all other organs and contemporary epilepsy research plays an interesting role ...
In patients with idiopathic generalized epilepsies (IGE), bursts of generalized spike and wave discharges (GSWD) lasting ?2 seconds are considered absence seizures. The location of the absence seizures generators in IGEs is thought to involve interplay between various components of thalamo-cortical circuits; we have recently postulated ...
Anxiety represents a major problem for people with epilepsy, and it is important to understand why it arises and how to reduce its potential debilitating and adverse effects. The aim of this study was to determine the prevalence of anxiety in a community-identified sample of people with epilepsy and to identify ...
Epilepsy as an issue for the public health community has a relatively short history in the United States. Not since the 1970s, when Congress established the Commission for the Control of Epilepsy and Its Consequences and the publication of its formal report, "Plan for Nationwide Action on Epilepsy," has significant attention been paid ...
Imaging of epilepsy patients is challenging, since epileptogenic lesions (defined as structural lesions causally related to the epilepsy syndrome) may be small and often do not change during life. Prior clinical information about the epilepsy syndrome and the semiology of the seizures is needed in order to plan the examination ...
The prevalence of idiopathic generalized epilepsies (IGEs) has been assessed as being 15-20% of all epilepsies. The seizure types in IGEs are typical absences, myoclonic jerks, and generalized tonic-clonic seizures (TCS), alone or in varying combinations and with variable severity. The seizures tend to be more frequent on awakening and ...
Scientists have now identified a genetic mutation responsible for epilepsy in dogs. Further research on dogs with this mutation might help researchers better understand how to treat or prevent seizures in humans with Lafora disease, according to the authors.
NSDL National Science Digital Library
Background electroencephalography (EEG), recorded with scalp electrodes, in children with childhood absence epilepsy (CAE) and control individuals has been analyzed. We considered 5 CAE patients, all right-handed females and aged 6-8 years. The 15 control individuals had the same characteristics of the CAE ones, but presented a normal EEG. The EEG was ...
This study aimed to examine the background electroencephalography (EEG) in children with childhood absence epilepsy, a condition whose presentation has strong developmental links. EEG hallmarks of absence seizure activity are widely accepted and there is recognition that the bulk of inter-ictal EEG in this group is normal to the naked ...
... of Epilepsy in Women | Hormones and Epilepsy | Birth Control for Women with Epilepsy | Genetics and Epilepsy | Epilepsy and Sexual Relationship | Pregnancy and ...
Although epilepsy is defined by the occurrence of spontaneous epileptic seizures, a large body of evidence indicates that epilepsy is linked to a spectrum of behavioral, psychiatric, and cognitive disorders as well as to sudden death. Explanations for these associations include the following: (1) The effects of structural lesions that may impair the ...
ObjectiveTo examine the relationship between genotype and phenotype in idiopathic generalized epilepsies (IGEs) using a novel approach that focuses on seizure type rather than syndrome.MethodsThe authors evaluated whether the genetic effects on myoclonic seizures differ from the genetic effects on absence seizures. For this purpose, they studied 34 ...
Currently, there is intense clinical research into various aspects of the medical risks relating to epilepsy, including total and cause-specific mortality, accidents and injuries in patients with epilepsy and mortality related with seizures. Seizures occurring in precarious situations and resulting in injuries are still an important concern for patients ...
Study Objectives.To describe the semiological features of NREM arousal parasomnias in detail and identify features that can be used to reliably distinguish parasomnias from nocturnal frontal lobe epilepsy (NFLE).Design.Systematic semiologial evaluation of parasomnias and NFLE seizures recorded on video-EEG monitoring.Patients.120 events (57 parasomnias, 63 ...
Absence epilepsy is a common form of idiopathic generalized epilepsy whose etiology is poorly understood due to genetic and phenotypic heterogeneity. The inbred mouse strain C3H/He exhibits spontaneous absence seizures characterized by spike and wave discharges (SWD) on the electroencephalogram concomitant with ...
Voxel-based morphometry of unilateral temporal lobe epilepsy reveals abnormalities in cerebral April 2004; accepted 5 May 2004 Voxel-based morphometric (VBM) investigations of temporal lobe epilepsy identified the expected abnormalities in hippocampus and extrahippocampal temporal lobe, as well as more
Epilepsy and migraine are chronic neurological disorders with episodic manifestations that are commonly treated in neurological practice and frequently occur together. In this review we examine similarities and contrasts between these disorders, with focus on epidemiology and classification, temporal coincidence, triggers, and mechanistically based therapeutic overlap. This ...
This study sought to present clinical and outcome data of patients with idiopathic childhood occipital epilepsy of Gastaut, to validate previously reported characteristics of this epilepsy. The study group was comprised of 12 affected children (three boys and nine girls), with a median age of onset at 10.3 years. Common ictal manifestations included ...
Benign myoclonic epilepsy in infancy (BMEI) is a rare syndrome included among idiopathic generalized epilepsies (IGE) and syndromes with age-related onset. Recently, it has been shown that a few patients with BMEI later had other epilepsy types mainly IGE but never childhood absence epilepsy ...
Dysfunctional default mode network (DMN) has been observed in various mental disorders, including epilepsy (see review Broyd et al. [2009]: Neurosci Biobehav Rev 33:279�296). Because interictal epileptic discharges may affect DMN, resting-state fMRI was used in this study to determine DMN functional connectivity in 14 healthy controls and 12 absence ...
Dietary therapies are established as beneficial for symptomatic generalized epilepsies such as Lennox-Gastaut syndrome; however, the outcome for idiopathic generalized epilepsy has never been specifically reported. The efficacy of the ketogenic and modified Atkins diet for childhood and juvenile absence epilepsy ...
Sudden death is over 20 times more frequent in people with epilepsy than the general population. The literature on clinical risk factors is now able to define individuals at the highest risk. Despite these advances in our understanding of risk, the mechanism of sudden unexpected death in epilepsy remains elusive. While it is unlikely that a single ...
Galloway-Mowat Syndrome (GMS) is an autosomal recessively inherited condition which manifests with severe encephalopathy, featuring microcephaly, developmental delay, and early-onset intractable epilepsy. Patients typically show also renal involvement from the onset. We report two siblings with GMS presenting with early-onset, intractable epilepsy and ...
During the past decade, substantial progress has been made in delineating clinical features of the epilepsies and the basic mechanisms responsible for these disorders. Eleven human epilepsy genes have been identified and many more are now known from animal models. Candidate targets for cures are now based upon newly ...
A homozygous mutation of the CNTNAP2 gene has been associated with a syndrome of focal epilepsy, mental retardation, language regression and other neuropsychiatric problems in children of the Old Order Amish community. Here we report genomic rearrangements resulting in haploinsufficiency of the CNTNAP2 gene in association with epilepsy and schizophrenia. ...
We identified a novel homozygous 15q13.3 microdeletion in a young boy with a complex neurodevelopmental disorder characterized by severe visual impairment, hypotonia, profound intellectual disability, and refractory epilepsy. The homozygous deletion of the genes within this deleted region provides a useful insight into the pathogenesis of the observed ...
The objective of this investigation was to determine quantitatively whether experimental epilepsy is associated with a change in the pharmacodynamics of benzodiazepines in vivo. For that purpose the pharmacodynamics of midazolam were quantified by an integrated pharmacokinetic-pharmacodynamic approach in three different models of experimental epilepsy: ...
The epidemiology of lesions identified by magnetic resonance imaging (MRI), along with the use of pre-surgical evaluations and surgery in childhood-onset epilepsy patients has not previously been described. In a prospectively identified community-based cohort of children enrolled from 1993 to 1997, we examined (i) the frequency of ...
A total of 142 patients (84 per cent aged less than 20 years) with various forms of generalized epilepsy have been treated with sodium valproate alone or in combination with other drugs. The mean duration of symptoms was six years, and half the patients had daily seizures. Nine patients had typical absences, 33 had absences with ...
A child with pattern sensitive epilepsy is reported. She had both absences and generalised seizures. The latter occurred spontaneously but the former followed concentration on patterned materials. These self-induced seizures appeared to have an affective component. The EEG showed generalised atypical spike and wave discharges both in the resting state and ...
. Huntsman, M. M., Porcello, D. M., Homanics, G. E., DeLorey, T. M. & Huguenard, J. R. (1999). Reciprocal
Kindling is a model of epilepsy whereby repeated administration of brief low intensity trains of electrical stimulation come to elicit electrographic and behavioral manifestations of seizure. n the absence of overt tissue damage, an animal that has been kindled is rendered in a p...
EPA Science Inventory
Neurons of the reticular thalamus (RT) display oscillatory burst discharges that are believed to be critical for thalamocortical network oscillations related to absence epilepsy. Ca�+-dependent mechanisms underlie such oscillatory discharges. However, involvement of high-voltage activated (HVA) Ca�+ channels in this process has been discounted. We ...
Absence seizures are characterized by impairment of consciousness associated with widespread bilaterally synchronous spike-and-wave discharges (SWDs) in the electroencephalogram (EEG), which reflect highly synchronized oscillations in thalamocortical networks. Although recent pharmacological studies suggest that the basal ganglia could provide a remote control system for ...