EPILEPSIA, Issue 2005
Ruth Ottman
Summary:, During the last decade, great progress has been made in the discovery of genes that influence risk for epilepsy. However, these gene discoveries have been in epilepsies with Mendelian modes of inheritance, which comprise only a tiny fraction of all epilepsy. Most people with epilepsy have no affected relatives, suggesting that the great majority of all epilepsies are genetically complex: multiple genes contribute to their etiology, none of which has a major effect on disease risk. Gene discovery in the genetically complex epilepsies is a formidable task. It is unclear which epilepsy phenotypes are most advantageous to study, and chromosomal localization and mutation detection are much more difficult than in Mendelian epilepsies. Association studies are very promising for the identification of complex epilepsy genes, but we are still in the earliest stages of their application in the epilepsies. Future studies should employ very large sample sizes to ensure adequate statistical power, clinical phenotyping methods of the highest quality, designs and analytic techniques that control for population stratification, and state-of-the-art molecular methods. Collaborative studies are essential to achieve these goals. [source]

Idiopathic Generalized Epilepsies: A Review and Modern Approach

EPILEPSIA, Issue 2005
Chrysostomos P. Panayiotopoulos M.D., Ph.D.
First page of article [source]

Historical Aspects of Idiopathic Generalized Epilepsies

EPILEPSIA, Issue 2005
Peter Wolf
Summary:, Early in these proceedings, the origin of the three terms in the title, "idiopathic generalized epilepsy," is discussed with respect to their significance over time, and typical misunderstandings. In the mid-20th century, a rather chaotic use of a multitude of often loosely defined terms had developed, which increasingly became an obstacle to a meaningful international discussion. The International League against Epilepsy (ILAE) took the initiative to develop an internationally accepted terminology with a classification system consisting of a classification of seizures (1981) and a classification of syndromes (1989). The Idiopathic Generalized Epilepsies are one of its four major groups emerging from a double dichotomy of generalized versus localization-related and idiopathic versus symptomatic. The inclusion of biologic aspects such as syndrome-specific ages of onset ("age-related syndromes") or syndrome-specific relations of seizure occurrence to the sleep,wake cycle ("Epilepsy with Grand Mal on Awaking") meant that the syndrome classification merged the more biological views of the German school with the more neurophysiological ones of the French. Apart from establishing a common international language concerning epilepsy, the International Classification of Epilepsies and Epileptic Syndromes became an important stimulator of research, especially concerning the idiopathic epilepsies. In particular, genetic and functional imaging investigations aim at a better understanding of these conditions. It is now understood that most idiopathic syndromes have a,sometimes complex,genetic background, but we are also becoming aware of the inappropriateness of the time-honored term "generalized" and part of our dichotomies. Both localization-related and "generalized" idiopathic epilepsies seem to share a principle of ictogenesis based on functional anatomic pathogenic networks, and we seem to move toward understanding them as functional system disorders of the brain. [source]

Nonepileptic Disorders Imitating Generalized Idiopathic Epilepsies

EPILEPSIA, Issue 2005
Natalio Fejerman
Summary:, Differential diagnosis between epileptic and nonepileptic paroxysmal disorders is fundamental not only to allow correct management of patients but also to avoid the burden of unnecessary antiepileptic medication. The focus of this chapter is limited to imitators of idiopathic generalized epilepsies (IGE) which are expressed through myoclonic, tonic,clonic, tonic, atonic, and absence seizures. Apparent losses of consciousness and drop attacks also have to be considered. Benign myoclonus of early infancy is the main nonepileptic disorder in the differential diagnosis of infantile spasms, but is not dealt with here because West syndrome is not an IGE. Hyperekplexia, metabolic disorders, hypnagogic myoclonus, and disturbed responsiveness caused by the use of drugs are listed in Table 1. Other conditions that may imitate more focal epileptic seizures are omitted. Benign neonatal sleep myoclonus, apnea and apparent life-threatening events in infants, cyanotic and pallid breath-holding spells, syncope, staring spells, psychogenic seizures, hyperventilation syndrome, and narcolepsy have been selected based on frequency or difficulties in differential diagnosis with the intention to cover the most conspicuous imitators of IGE in different ages. Table 1. Nonepileptic disorders imitating idiopathic generalized epilepsies [source]

Symptomatic Epilepsies Imitating Idiopathic Generalized Epilepsies

EPILEPSIA, Issue 2005
Hirokazu Oguni
Summary:, The diagnosis of idiopathic generalized epilepsies (IGEs) is not generally difficult if one follows the clinical and electroencephalogram (EEG) definitions of each subsyndrome that constitutes IGEs. In contrast, symptomatic epilepsies develop based on organic brain lesions and are easily diagnosed by the presence of developmental delay, neurologic abnormalities, and a characteristic seizure and EEG pattern. However, in clinical practice, it is sometimes difficult to differentiate IGEs from symptomatic epilepsies, especially when the clinical course from the onset of epilepsy is too short to exhibit typical clinical and EEG findings of either epilepsy type, or when patients with symptomatic epilepsies have atypical features that imitate the clinical characteristics of IGEs. The neurodegenerative or metabolic disorders at times start during the clinical course with epileptic seizures and later show typical neurologic abnormalities. The newly recognized metabolic disorder of glucose transporter type 1 deficiency syndrome (Glut-1 DS) may start with myoclonic seizures at an age of less than 1 year and imitate benign myoclonic epilepsy in infancy early in the clinical course. Progressive myoclonus epilepsies (PMEs) that develop at 1,4 years of age at times imitate epilepsy with myoclonic-astatic seizures with respect to the presence of astatic seizures and an epileptic encephalopathic EEG pattern. In addition, young children with focal cortical dysplasia may also have similar clinical and EEG patterns, although the latter may become localized after treatment. Approximately 15% of patients with juvenile myoclonic epilepsy (JME) are resistant to antiepileptic drugs (AEDs) and may require extensive study to make a differential diagnosis from symptomatic epilepsies. PMEs that develop during adolescence may imitate JME early in the clinical course; however, a detailed history and the differentiation between myoclonic seizures and myoclonus would help to distinguish both conditions. The diagnosis of IGEs is very demanding for patients with atypical features with regard to seizure type, EEG findings, and response to appropriate AEDs. [source]

Evidence-based Treatment of Idiopathic Generalized Epilepsies with Older Antiepileptic Drugs

EPILEPSIA, Issue 2005
Nikolas Hitiris
Summary:, Older antiepileptic drugs continue to play a major role in the treatment of the idiopathic generalized epilepsies. Comparative studies of ethosuximide and valproate have demonstrated equivalence in the treatment of childhood absence epilepsy. Valproate can be regarded as the recommended first-line treatment for juvenile myoclonic epilepsy based on case series reports. Studies in patients with generalized tonic-clonic seizures have not separated out idiopathic from secondary generalized events. Treatment for the other idiopathic generalized epilepsy syndromes lacks evidence other than a few case reports and diverse expert opinion. Further randomized controlled trials of older antiepileptic drugs are recommended to solidify the evidence-based treatment of the idiopathic generalized epilepsies. [source]

Levetiracetam in the Treatment of Idiopathic Generalized Epilepsies

EPILEPSIA, Issue 2005
Richard Grünewald
Summary:, Since its introduction into clinical practice in 1999, levetiracetam, the S enantiomer of piracetam, has rapidly found a secure place, initially in the therapy of partial onset seizures and subsequently in the treatment of idiopathic generalized epilepsies (IGE). It has many of the properties of an "ideal" antiepileptic drug, including rapid absorption, linear pharmokinetics, and sparse drug interactions. Tolerabiliy is generally excellent in both adults and children, although tiredness is a common dose-limiting adverse effect. Occasionally the drug can precipitate behavioral abnormalities, especially in patients with learning disability. There is a wide safety margin in overdose. In common with most antiepileptic drugs its mode of action remains uncertain. Levetiracetam binds to a specific site in the brain, influences intracellular calcium currents and reverses negative allosteric modulators of GABA- and glycine-gated currents in vitro. Its effectiveness has been demonstrated in animal models of epilepsy and in clinical trials of partial onset and IGE. Treatment of IGEs may be straightforward, with many patients demonstrating an excellent and robust response to valproate monotherapy. However, there remains a significant minority of patients for whom valproate is unsuitable, including those who experience unacceptable adverse effects (e.g., weight gain or hair loss) and women of childbearing age in whom the teratogenic potential of valproate is unacceptable. Therapeutic response to lamotrigine in this group is often disappointing, and many clinicians now are turning to the choice of levetiracetam. Efficacy in generalized tonic,clonic seizures and myoclonus is usually apparent and some patients experience improvement in typical absences. Experience of combinations of levetiracetam with other antiepileptic drugs is limited in IGE and the responses are largely anecdotal. In our hands, patients with refractory IGEs may respond to combinations of levetiracetam with valproate, lamotrigine, and phenobarbital, and adverse effects when they occur are usually limited to tiredness. Levetiracetam does not interact with the oral contraceptive pill, simplifying treatment in women of childbearing age. Although animal data look encouraging, questions over levetiracetam's teratogenic potential and overall safety in pregnancy will remain for many years to come. [source]

Classification of the Myoclonic Epilepsies

EPILEPSIA, Issue 2003
Ilo E. Leppik
Summary: The myoclonic epilepsies are a collection of syndromes in which myoclonic seizures are a prominent feature. Proper classification of a patient's syndrome is critical for appropriate treatment and prognosis. However, classification of such syndromes is often difficult because the terminology used to describe seizures can be confusing and inconsistent. Myoclonic epilepsy syndromes can be epileptic or nonepileptic and can also be divided into inherited and acquired forms. Progressive myoclonic epilepsy (PME) syndromes are the most severe of the myoclonic epilepsies. Diagnosis of PME syndromes on clinical grounds can be difficult, but advances in genetic testing have made diagnoses more accurate. Some other benign myoclonic epilepsy syndromes also have identified gene markers, which can aid in diagnosis. To accurately classify a patient's epilepsy syndrome, clinicians should use all available clinical laboratory tools appropriately. Improved accuracy of diagnosis for patients with myoclonic epilepsies should lead to more dependable prognoses and more effective treatment. [source]

Editor's Introduction: Cabbages and Kings in the Classification of Seizures and the Epilepsies

EPILEPSIA, Issue 1 2003
Robert S. Fisher
First page of article [source]

Benign Partial Epilepsies of Adolescence: A Report of 37 New Cases

EPILEPSIA, Issue 12 2001
G. Capovilla
Summary: ,Purpose: To delineate the electroclinical features of patients with partial seizures in adolescence with a benign outcome. Methods: Patients were recruited in five different Italian epilepsy centers. Patients were selected among those with partial seizures between ages 11 and 17 years. We excluded benign childhood epilepsies, those with neurologic or mental deficits, and those with neuroradiologically documented lesions. We also excluded patients with less than 3 years' follow-up or who were still receiving antiepileptic therapy. Results: There were 37 (22 male, 15 female) patients. Seizures started at the mean age of 14.5 years (range, 11,16.11). Two main electroclinical patterns emerged: 16 of 37 patients had somatomotor seizures frequently associated with focal theta discharges involving the centroparietal regions. Ten of 37 patients showed versive seizures and interictal spiking involving the posterior regions. A third group had clinical characteristics resembling the cases described by Loiseau. All had a favorable outcome. Conclusions: This relevant multicenter study further confirms the existence of benign partial epilepsies with onset during adolescence. [source]

New Insights into the Clinical Management of Partial Epilepsies

EPILEPSIA, Issue S5 2000
Prof. Edouard Hirsch
Summary The diagnosis, treatment, and prognosis of seizure disorders depend on the correct identification of epileptic syndromes. Partial epilepsies are heterogeneous and can be divided into idiopathic, cryptogenic, and symptomatic epilepsies. The most common of the idiopathic localization-related epilepsies is benign epilepsy with rolandic or centrotemporal spikes (BECTS). Seizures remain rare and the use of antiepileptic drug (AED) treatment in all patients does not appear justified. Children who present with some of the electroclinical characteristics of BECTS may also display severe unusual neurologic, neuropsychological, or atypical symptoms. In some cases, carbamazepine has been implicated as a triggering factor. Primary reading epilepsy and idiopathic occipital lobe epilepsies with photosensitivity are examples of an overlap between idiopathic localization-related and generalized epilepsies and respond well to sodium valproate. Autosomal dominant nocturnal frontal lobe epilepsy and benign familial infantile convulsions are recently described syndromes, differing in several ways from classical idiopathic localization-related epileptic syndromes. In cryptogenic or symptomatic epilepsy, the topography of the epileptogenic zone might influence drug efficacy. An individualized approach to AED selection, tailored to each patient's needs, should be used. Resistance of seizures to antiepileptic therapy may be due to diagnostic and/or treatment error or may be the result of noncompliance. Increasing the dosage, discontinuation or replacement of a drug, or addition of a second drug is indicated in truly resistant cases. The use of more than two AEDs rarely optimizes seizure control, and in some cases reduction of treatment may improve seizure control while lessening side effects. EEG-video assessment of patients with refractory epilepsy is important. Indications for and timing of epilepsy surgery should be reconsidered. Surgical therapy should probably be used more often and earlier than it is at present. [source]

Animal Models of Limbic Epilepsies: What Can They Tell Us?

BRAIN PATHOLOGY, Issue 2 2002
Douglas A. Coulter
First page of article [source]

Baseline cognition, behavior, and motor skills in children with new-onset, idiopathic epilepsy

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 1 2010
VIKRAM V BHISE
Aim, Epilepsy is associated with difficulties in cognition and behavior in children. These problems have been attributed to genetics, ongoing seizures, psychosocial issues, underlying abnormality of the brain, and/or antiepileptic drugs. In a previous study, we found baseline cognitive differences between children with partial versus generalized and convulsive versus non-convulsive seizures. Measures in that study focused primarily on IQ scores. In the present study, we assessed baseline function with respect to new learning, attention, and memory, thus providing a more comprehensive profile than our previous study. Method, We examined 57 children (42 females, 15 males), aged 6 to 17 years (mean 10y 1mo, SD 2y 9mo), with new-onset, idiopathic epilepsy, using tests of cognitive function reflective of new learning, memory, and attention. Seizures were classified as generalized convulsive (n=5), generalized non-convulsive (n=18), or focal (n=34). Focal seizures were divided into unilateral versus bilateral independent foci, and presence versus absence of secondary generalization. Results, Attention was a particular area of weakness across all groups. The Vocabulary score of an intelligence screen was higher for the focal seizure groups (p=0.012), primarily because of a difference between the unilateral focal and the primary generalized groups (p<0.047). Children with generalized, non-convulsive seizures performed significantly worse than the focal group on a measure of short-term auditory memory (p=0.019). All groups performed poorly on a test of visual,motor speed. Interpretation, These findings suggest intrinsic abnormalities in children with new-onset, idiopathic epilepsy at baseline. [source]

Epilepsy in fragile X syndrome

DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 11 2002
Elizabeth Berry-Kravis MD PhD
Epilepsy is reported to occur in 10 to 20% of individuals with fragile X syndrome (FXS). A frequent seizure/EEG pattern in FXS appears to resemble that of benign focal epilepsy of childhood (BFEC, benign rolandic epilepsy). To evaluate seizure frequency and type in a Chicago FXS cohort, data regarding potential seizure history were reviewed for 136 individuals with FXS (age range 2 to 51 years: 113 males and 23 females). Seizures occurred in 15 males (13.3%) and one female (4.3%): of these, 12 had partial seizures. EEG findings were available for 35 individuals (13 of 16 with seizures and 22 of 120 without seizures) and showed an epileptiform abnormality in 10 (77%) individuals with seizures and five (23%) individuals without seizures - the most common epileptiform pattern being centrotemporal spikes. Seizures were easily controlled in 14 of the 16 individuals with seizures. Many individuals, including all with centrotemporal spikes, had remission of seizures in childhood. The most common seizure syndrome resembled BFEC and this pattern had the best prognosis for epilepsy remission. Deficiency of FMRP (fragile X mental retardation protein) appears to lead to increased neuronal excitability and susceptibility to epilepsy, but particularly seems to facilitate mechanisms leading to the BFEC pattern. [source]

Neuronal plasticity: implications in epilepsy progression and management

DRUG DEVELOPMENT RESEARCH, Issue 8 2007
Sherifa A. HamedArticle first published online: 12 FEB 200
Abstract Epilepsy is a common neurological disease. A growing number of research studies provide evidence regarding the progressive neuronal damage induced by prolonged seizures or status epilepticus (SE), as well as recurrent brief seizures. Importantly, seizure is only one aspect of epilepsy. However, cognitive and behavioral deficits induced by progressive seizures or antiepileptic treatment can be detrimental to individual function. The neurobiology of epilepsy is poorly understood involving complex cellular and molecular mechanisms. The brain undergoes changes in its basic structure and function, e.g., neural plasticity with an increased susceptibility in neuronal synchronization and network circuit alterations. Some of these changes are transient, while others are permanent with an involvement of both glutamatergic and ,-aminobutyric acid (GABA)ergic systems. Recent data suggest that impaired neuronal plasticity may underlie the cognitive impairment and behavioral changes associated with epilepsy. Many neurologists recognize that the prevention or suppression of seizures by the use of antiepileptic drugs (AEDs) alone is insufficient without clear predictions of disease outcome. Hence, it is important to understand the molecular mechanisms underlying epileptogenesis because this may allow the development of innovative strategies to prevent or cure this condition. In addition, this realization would have significant impact in reducing the long-term adverse consequences of the disease, including neurocognitive and behavioral adverse effects. Drug Dev Res 68:498,511, 2007. © 2008 Wiley-Liss, Inc. [source]

Reliability of patterns of hippocampal sclerosis as predictors of postsurgical outcome

EPILEPSIA, Issue 9 2010
Maria Thom
Summary Purpose:, Around one-third of patients undergoing temporal lobe surgery for the treatment of intractable temporal lobe epilepsy with hippocampal sclerosis (HS) fail to become seizure-free. Identifying reliable predictors of poor surgical outcome would be helpful in management. Atypical patterns of HS may be associated with poorer outcomes. Our aim was to identify atypical HS cases from a large surgical series and to correlate pathology with clinical and outcome data. Methods:, Quantitative neuropathologic evaluation on 165 hippocampal surgical specimens and 21 control hippocampi was carried out on NeuN-stained sections. Neuronal densities (NDs) were measured in CA4, CA3, CA2, and CA1 subfields. The severity of granule cell dispersion (GCD) was assessed. Results:, Comparison with control ND values identified the following patterns based on the severity and distribution of neuronal loss: classical HS (CHS; n = 60) and total HS (THS; n = 39). Atypical patterns were present in 30% of cases, including end-folium sclerosis (EFS; n = 5), CA1 predominant pattern (CA1p; n = 9), and indeterminate HS (IHS, n = 35). No HS was noted in 17 cases. Poorest outcomes were noted for no-HS, and CA1p groups with 33,44% International League Against Epilepsy (ILAE) class I at up to 2 years follow-up compared to 69% for CHS (p < 0.05). GCD associated with HS type (p < 0.01), but not with outcome. Conclusions:, These findings support the identification and delineation of atypical patterns of HS using quantitative methods. Atypical patterns may represent distinct clinicopathologic subtypes and may have predictive value following epilepsy surgery. [source]

Epilepsy with dual pathology: Surgical treatment of cortical dysplasia accompanied by hippocampal sclerosis

EPILEPSIA, Issue 8 2010
Dong W. Kim
Summary Purpose:, The presence of two or more epileptogenic pathologies in patients with epilepsy is often observed, and the coexistence of focal cortical dysplasia (FCD) with hippocampal sclerosis (HS) is one of the most frequent clinical presentations. Although surgical resection has been an important treatment for patients with refractory epilepsy associated with FCD, there are few studies on the surgical treatment of FCD accompanied by HS, and treatment by resection of both neocortical dysplastic tissue and hippocampus is still controversial. Methods:, We retrospectively recruited epilepsy patients who had undergone surgical treatment for refractory epilepsy with the pathologic diagnosis of FCD and the radiologic evidence of HS. We evaluated the prognostic roles of clinical factors, various diagnostic modalities, surgical procedures, and the severity of pathology. Results:, A total of 40 patients were included, and only 35.0% of patients became seizure free. Complete resection of the epileptogenic area (p = 0.02), and the presence of dysmorphic neurons or balloon cells on histopathology (p = 0.01) were associated with favorable surgical outcomes. Patients who underwent hippocampal resection were more likely to have a favorable surgical outcome (p = 0.02). Conclusions:, We show that patients with complete resection of epileptogenic area, the presence of dysmorphic neurons or balloon cells on histopathology, or resection of hippocampus have a higher chance of a favorable surgical outcome. We believe that this observation is useful in planning of surgical procedures and predicting the prognoses of individual patients with FCD patients accompanied by HS. [source]

Course and outcome of childhood epilepsy: A 15-year follow-up of the Dutch Study of Epilepsy in Childhood

EPILEPSIA, Issue 7 2010
Ada Geerts
Summary Purpose:, To study the course and outcome of childhood-onset epilepsy during 15-year follow-up (FU). Methods:, We extended FU in 413 of 494 children with new-onset epilepsy recruited in a previously described prospective hospital-based study by questionnaire. Results:, Mean FU was 14.8 years (range 11.6,17.5 years). Five-year terminal remission (TR) was reached by 71% of the cohort. Course during FU was favorable in 50%, improving in 29%, and poor or deteriorating in 16%. Mean duration of seizure activity was 6.0 years (range 0,21.5 years), strongly depending on etiology and epilepsy type. Duration was <1 year in 25% of the cohort and exceeded 12 years in another 25%. Antiepileptic drugs (AEDs) were used by 86% during a mean of 7.4 years: one-third had their last seizure within 1 year of treatment, and one-third continued treatment at the end, although some had a 5-year TR. At last contact, 9% of the cohort was intractable. In multivariate analysis, predictors were nonidiopathic etiology, febrile seizures, no 3-month remission, and early intractability. Eighteen patients died; 17 had remote symptomatic etiology. Standardized mortality ratio for remote symptomatic etiology was 31.6 [95% confidence interval (CI) 18.4,50.6], versus 0.8 [95% CI 0.02,4.2] for idiopathic/cryptogenic etiology. Discussion:, In most children with newly diagnosed epilepsy, the long-term prognosis of epilepsy is favorable, and in particular, patients with idiopathic etiology will eventually reach remission. In contrast, epilepsy remains active in ,30% and becomes intractable in ,10%. AEDs probably do not influence epilepsy course; they merely suppress seizures. Mortality is significantly higher only in those with remote symptomatic etiology. [source]

Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005,2009

EPILEPSIA, Issue 4 2010
Anne T. Berg
Summary The International League Against Epilepsy (ILAE) Commission on Classification and Terminology has revised concepts, terminology, and approaches for classifying seizures and forms of epilepsy. Generalized and focal are redefined for seizures as occurring in and rapidly engaging bilaterally distributed networks (generalized) and within networks limited to one hemisphere and either discretely localized or more widely distributed (focal). Classification of generalized seizures is simplified. No natural classification for focal seizures exists; focal seizures should be described according to their manifestations (e.g., dyscognitive, focal motor). The concepts of generalized and focal do not apply to electroclinical syndromes. Genetic, structural,metabolic, and unknown represent modified concepts to replace idiopathic, symptomatic, and cryptogenic. Not all epilepsies are recognized as electroclinical syndromes. Organization of forms of epilepsy is first by specificity: electroclinical syndromes, nonsyndromic epilepsies with structural,metabolic causes, and epilepsies of unknown cause. Further organization within these divisions can be accomplished in a flexible manner depending on purpose. Natural classes (e.g., specific underlying cause, age at onset, associated seizure type), or pragmatic groupings (e.g., epileptic encephalopathies, self-limited electroclinical syndromes) may serve as the basis for organizing knowledge about recognized forms of epilepsy and facilitate identification of new forms. [source]

High seizure frequency prior to antiepileptic treatment is a predictor of pharmacoresistant epilepsy in a rat model of temporal lobe epilepsy

EPILEPSIA, Issue 1 2010
Wolfgang Löscher
Summary Purpose:, Progress in the management of patients with medically intractable epilepsy is impeded because we do not fully understand why pharmacoresistance happens and how it can be predicted. The presence of multiple seizures prior to medical treatment has been suggested as a potential predictor of poor outcome. In the present study, we used an animal model of temporal lobe epilepsy to investigate whether pharmacoresistant rats differ in seizure frequency from pharmacoresponsive animals. Methods:, Epilepsy with spontaneous recurrent seizures (SRS) was induced by status epilepticus. Frequency of SRS was determined by video/EEG (electroencephalography) monitoring in a total of 33 epileptic rats before onset of treatment with phenobarbital (PB). Results:, Thirteen (39%) rats did not respond to treatment with PB. Before treatment with PB, average seizure frequency in PB nonresponders was significantly higher than seizure frequency in responders, which, however, was due to six nonresponders that exhibited > 3 seizures per day. Such high seizure frequency was not observed in responders, demonstrating that high seizure frequency predicts pharmacoresistance in this model, but does not occur in all nonresponders. Discussion:, The data from this study are in line with clinical experience that the frequency of seizures in the early phase of epilepsy is a dominant risk factor that predicts refractoriness. However, resistance to treatment also occurred in rats that did not differ in seizure frequency from responders, indicating that disease severity alone is not sufficient to explain antiepileptic drug (AED) resistance. These data provide further evidence that epilepsy models are useful in the search for predictors and mechanisms of pharmacoresistance. [source]

The International League Against Epilepsy at the threshold of its second century: Challenges and opportunities

EPILEPSIA, Issue 12 2009
Solomon L. Moshé
First page of article [source]

Antiepileptic drugs in children in developing countries: Research and treatment guideline needs

EPILEPSIA, Issue 11 2009
Mina Farkhondeh
Summary Epilepsy is the most common neurologic disorder in childhood. Effective interventions are available for treatment; however, the treatment gap in children is more than 80% in many developing countries. An important reason for this huge treatment gap is limited access to antiepileptic drugs (AEDs). This article discusses the reasons for such a treatment gap, and possible ways forward in improving care of children with epilepsy worldwide. [source]

Ring chromosome 20 syndrome: A link between epilepsy onset and neuropsychological impairment in three children

EPILEPSIA, Issue 11 2009
Aglaia Vignoli
Summary Purpose:, Ring chromosome 20 [r(20)] syndrome is a well-defined chromosomal disorder characterized by epilepsy, mild-to-moderate mental retardation, and lack of recognizable dysmorphic features. Epilepsy is often the most important clinical manifestation of the syndrome, even if its appearance is not constantly precocious. Seizures are frequently drug resistant. Methods:, We describe three children with [r(20)] syndrome in whom the onset of epilepsy (age at onset range: 4 years and 6 months to 9 years and 4 months) determined a kind of epileptic status (age at onset range: 6 years and 10 months to 9 years and 8 months) with dramatic neuropsychological deterioration. This epileptic status lasted for several months because of refractoriness to most antiepileptic drugs (AEDs), but it was treated successfully with a combination of valproate and lamotrigine in two children. Results:, As soon as seizures stopped, the children showed prompt recovery with partial restoration of the neuropsychological impairment. Conclusion:, This clinical picture can be described as abrupt epileptic encephalopathy. [source]

Assessment and surgical outcomes for mild type I and severe type II cortical dysplasia: A critical review and the UCLA experience

EPILEPSIA, Issue 6 2009
Jason T. Lerner
Summary Recent findings on the clinical, electroencephalography (EEG), neuroimaging, and surgical outcomes are reviewed comparing patients with Palmini type I (mild) and type II (severe) cortical dysplasia. Resources include peer-reviewed studies on surgically treated patients and a subanalysis of the 2004 International League Against Epilepsy (ILAE) Survey of Pediatric Epilepsy Surgery. These sources were supplemented with data from University of California, Los Angeles (UCLA). Cortical dysplasia is the most frequent histopathologic substrate in children, and the second most common etiology in adult epilepsy surgery patients. Cortical dysplasia patients present with seizures at an earlier age than other surgically treated etiologies, and 33,50% have nonlocalized scalp EEG and normal magnetic resonance imaging (MRI) scans. 2-(18F)Fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) is positive in 75,90% of cases. After complete resection, 80% of patients are seizure free compared with 20% with incomplete resections. Compared with type I, patients with type II cortical dysplasia present at younger ages, have higher seizure frequencies, and are extratemporal. Type I dysplasia is found more often in adult patients in the temporal lobe and is often MRI negative. These findings identify characteristics of patients with mild and severe cortical dysplasia that define surgically treated epilepsy syndromes. The authors discuss future challenges to identifying and treating medically refractory epilepsy patients with cortical dysplasia. [source]

The concept of the epilepsy syndrome: How useful is it in clinical practice?

EPILEPSIA, Issue 2009
Ettore Beghi
Summary An epilepsy syndrome is a disorder characterized by a cluster of symptoms and signs customarily occurring in combination. A syndromic approach to the epilepsies would be of practical value for diagnostic, prognostic, and therapeutic purposes. However, despite considerable efforts by leaders in the field of epileptology and the improved knowledge of the clinical, genetic, imaging, and biologic aspects of epilepsy, there are no measurable objective criteria for recognizing seizure types and epilepsy syndromes as separate diagnostic entities with well-defined prognostic and therapeutic aspects. The lack of pragmatic, evidence-based instruments to devise a syndromic classification useful for clinical practice can be explained by the evolving concept of epilepsy syndrome, its dynamic characteristics, the poor prognostic predictivity, and the extremely complex genetic and pathophysiologic mechanisms underlying the epileptic phenomena. In addition, the results of the published reports on epilepsy syndromes are mostly biased by flaws in the study population, design, and statistical analysis. The Classification Core Group of the International League Against Epilepsy (ILAE), which is working on a new classification of the epilepsies, stated that the process of syndrome identification requires that an evidence-based approach be applied to the published literature and future studies. [source]

Notes on the origins of Epilepsia and the International League Against Epilepsy

EPILEPSIA, Issue 3 2009
Simon D. Shorvon
Summary The recent discovery of archival material has shed interesting light on the origins of Epilepsia and also the International League Against Epilepsy (ILAE). The idea of an international journal devoted to epilepsy seems first to have arisen from talks between Dr. L. J. J. Muskens and Dr. W. Aldren Turner in 1905. A protracted series of subsequent letters between Muskens and a Haarlem publisher show how the idea slowly took shape. The committee of patronage, editorial board, and editorial assistants was probably first approached at the First International Congress of Psychiatry, Neurology, Psychology, and Nursing of the Insane, held in Amsterdam in 1907. At this meeting, the concept of an international organization to fight epilepsy (to become the ILAE) was also first proposed in public, again by Muskens. The concept of the ILAE was clearly modeled on another international organization,the International Commission for the Study of the Causes of Mental Diseases and Their Prophylaxis. This Commission had been first publicly proposed in 1906 by Ludwig Frank, at the Second International Congress for the Care and Treatment of the Insane. The proposed Commission and ILAE shared many features, aims, and personnel. Despite an auspicious start, the International Commission was prevented by personal and political differences from ever actually coming into being. However, the first issue of Epilepsia appeared in March 1909 and the ILAE was inaugurated in August 1909; and both have flourished and celebrate their centenaries this year. [source]

Epilepsy and respiratory chain defects in children with mitochondrial encephalopathies

EPILEPSIA, Issue 11 2008
Divya Khurana
First page of article [source]

Measures of adherence to epilepsy treatment: Review of present practices and recommendations for future directions

EPILEPSIA, Issue 7 2008
Angelia M. Paschal
Summary Epilepsy is one of the most common neurological disorders worldwide, and the majority of people with epilepsy who live in developed countries manage their condition with antiseizure medication. Surprisingly, therefore, the literature on epilepsy does not document a comprehensive investigation of patient adherence to medication treatment. This paper reviews existing literature on direct and indirect measures of adherence. Based on this review, areas in need for further research have been identified, including improvement of self-report instruments, consideration of cultural factors, attention to patient literacy or numeracy levels, and inclusion of patient-guided measures. While no single method of determining adherence has proved effective, combining direct and indirect measures in a patient-guided, culturally competent atmosphere may increase adherence to treatment, improving health outcomes for this population. [source]

Psychiatric Comorbidity in Epilepsy: A Population-Based Analysis

EPILEPSIA, Issue 12 2007
Jose F. Tellez-Zenteno
Summary Purpose: The estimated prevalence of mental health disorders in those with epilepsy in the general population varies owing to differences in study methods and heterogeneity of epilepsy syndromes. We assessed the population-based prevalence of various psychiatric conditions associated with epilepsy using a large Canadian national population health survey. Methods: The Canadian Community Health Survey (CCHS 1.2) was used to explore numerous aspects of mental health in persons with epilepsy in the community compared with those without epilepsy. The CCHS includes administration of the World Mental Health Composite International Diagnostic Interview to a sample of 36,984 subjects. Age-specific prevalence of mental health conditions in epilepsy was assessed using logistic regression. Results: The prevalence of epilepsy was 0.6%. Individuals with epilepsy were more likely than individuals without epilepsy to report lifetime anxiety disorders or suicidal thoughts with odds ratio of 2.4 (95% CI = 1.5,3.8) and 2.2 (1.4,3.3), respectively. In the crude analysis, the odds of lifetime major depression or panic disorder/agoraphobia were not greater in those with epilepsy than those without epilepsy, but the association with lifetime major depression became significant after adjustment for covariates. Conclusions: In the community, epilepsy is associated with an increased prevalence of mental health disorders compared with the general population. Epilepsy is also associated with a higher prevalence of suicidal ideation. Understanding the psychiatric correlates of epilepsy is important to adequately manage this patient population. [source]