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Epileptic Syndromes (epileptic + syndrome)

Distribution by Scientific Domains

Medical Sciences	95%

Selected Abstracts

Prevalence of Childhood Epilepsy and Distribution of Epileptic Syndromes: A Population-based Survey in Okayama, Japan

EPILEPSIA, Issue 3 2006
Eiji Oka
Summary:,Methods: Information on patients younger than 13 years with active epilepsy was collected from medical records. Patients diagnosed with epilepsy according to clinical and EEG findings were put on the list even if those patients had had a single seizure or seizures occurring during febrile episodes. Results: In total, 2,220 cases were identified from a background population of 250,997. The prevalence rate was 8.8 per 1,000. If we exclude patients who had experienced a single seizure or seizures occurring during febrile episodes to compare our results with previous reports, the prevalence rate was 5.3 per 1,000. Of the 2,220 cases, 2,030 (91.4%) were classified into three major categories by ILAE classification. They consisted of 1,556 (76.7%) with localization-related epilepsy, 453 (22.3%) with generalized epilepsy, and 21 (1.0%) with undetermined epilepsy. Of the 2,030 cases, 309 (15.2%) were classified into epileptic syndrome categories, and 84.8% of the total were nonspecific types of epilepsy. Conclusions: The prevalence rate of childhood epilepsy was distributed from 5.3 to 8.8 per 1,000. The appearance rate of various types of epileptic syndromes was low. Most cases could not be classified into the detailed categories of the International Classification (ILAE, 1989). [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]

Specific Epileptic Syndromes Are Rare Even in Tertiary Epilepsy Centers: A Patient-oriented Approach to Epilepsy Classification

EPILEPSIA, Issue 3 2004
Christoph Kellinghaus
Summary: Purpose: To assess the practicability and reliability of a five-dimensional patient-oriented epilepsy classification and to compare it with the International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The dimensions consist of the epileptogenic zone, semiologic seizure type(s), etiology, related medical conditions, and seizure frequency. Methods: The 185 epilepsy patients (94 adults, 91 children, aged 18 years or younger) were randomly selected from the database of a tertiary epilepsy center and the general neurological department of a metropolitan hospital (28 adults). The charts were reviewed independently by two investigators and classified according to both the ILAE and the patient-oriented classification. Interrater reliability was assessed, and a final consensus among all investigators was established. Results: Only four (4%) adults and 19 (21%) children were diagnosed with a specific epilepsy syndrome of the ILAE classification. All other patients were in unspecific categories. The patient-oriented classification revealed that 64 adults and 56 children had focal epilepsy. In an additional 34 adults and 45 children, the epileptogenic zone could be localized to a certain brain region, and in 14 adults and five children, the epileptogenic zone could be lateralized. Fourteen adults and 21 children had generalized epilepsy. In 16 adults and 14 children, it remained unclear whether the epilepsy was focal or generalized. Generalized simple motor seizures were found in 66 adults and 52 children, representing the most frequent seizure type. Etiology could be determined in 40 adults and 45 children. Hippocampal sclerosis was the most frequent etiology in adults (10%), and cortical dysplasia (9%), in children. Seven adults and 31 children had at least daily seizures. Seventeen adults and 26 children had rare or no seizures at their last documented contact. The most frequent related medical conditions were psychiatric disorders and mental retardation. Interrater agreement was high (kappa values of 0.8 to 0.9) for both the patient-oriented and the ILAE classification. Conclusions: Specific epilepsy syndromes included in the current ILAE classification are rare even in a tertiary epilepsy center. Most patients are included in unspecific categories that provide only incomplete information. In contrast, all of the patients could be classified by the five-dimensional patient-oriented classification, providing all essential information for the management of the patients with a high degree of interrater reliability. [source]

A Clinical Guide to Epileptic Syndromes and their Treatment: Based on the New ILAE Diagnostic Scheme

EPILEPSIA, Issue 8 2003
Richard S. McLachlan M.D.
No abstract is available for this article. [source]

Photosensitivity in Relation to Epileptic Syndromes: A Survey from an Epilepsy Center in Japan

EPILEPSIA, Issue 3 2001
Hideaki Shiraishi
Summary: ,Purpose: We examined the incidence and distribution of photosensitivity among the different age groups and different types of epilepsies and epileptic syndromes. Furthermore, we considered the influence of ethnic and geographic factors on the incidence of photoparoxysmal response (PPR) in epilepsy patients. Methods: We analyzed the responses to intermittent photic stimulation (IPS) by using a Grass PS22 or PS33 photic stimulator for in 2,187 unselected patients with epilepsy who were treated in our center. Results: The classic PPR was elicited in 37 (1.7%) patients. The mean age of these 37 patients was 17.0 years. The subpopulation of patients having PPR included 2.0% of all patients with symptomatic generalized epilepsy, 5.6% (p < 0.01) of those with idiopathic generalized epilepsy, 0.7% of those with symptomatic localization-related epilepsy, and 2.9% of those with undetermined epilepsy. PPR accounted for 17.4 % (p < 0.01) of the patients with juvenile myoclonic epilepsy, 7.6% (p < 0.01) of those with grand mal on awakening, and 6.1% (p < 0.01) of those with symptomatic occipital lobe epilepsy. The incidence of PPR increased in patients up to age 15 years, and suddenly decreased after age 20 years. Conclusion: The present study presents the first report from eastern Asia, analyzing the incidence of PPR with a restricted definition comparable to the other studies, and the rate of PPR was relatively low compared with the studies performed in the European countries. We could confirm the clear relation between age and positive PPR. [source]

A Clinical Guide to Epileptic Syndromes and their Treatment.

EUROPEAN JOURNAL OF NEUROLOGY, Issue 2 2009
Second edition (Based on the ILAE classifications, practice parameter guidelines)
No abstract is available for this article. [source]

Epilepsy and Language Development: The Continuous Spike-Waves during Slow Sleep Syndrome

EPILEPSIA, Issue 6 2007
Séverine Debiais
Summary:,Background: Continuous spike-waves during slow sleep syndrome (CSWSS) is a rare epileptic syndrome occurring in children, which is characterized by the association of epilepsy, neuropsychological disorders, and abnormal paroxysmal electroencephalographic (EEG) discharges activated by sleep. Language can be affected but, to date, language disorders and their long-term outcome have been documented only rarely. Purposes: Description of language impairment in patients with the CSWSS. Methods: We performed a detailed language testing in 10 right-handed children and adolescents with the CSWSS. Their pragmatic performance was compared to that of a control population of 36 children aged 6,10 years. Results: Patients with CSWSS had lower scores in tests measuring their lexical, morphosyntactic, and pragmatic skills compared to controls. Comprehension remains unaffected. In addition, language impairment was found to be just as severe in patients in remission as those still in an active phase. Conclusions: We found severe language impairments in lexical and syntactic skills. The language profile is different from that observed in the Landau,Kleffner syndrome. Moreover patients in remission and those in an active phase of the CSWSS have the same language impairment profiles. This confirms the poor long-term neuropsychological prognosis. Our results raise points about the relationship between epileptic activity and language development. This pilot study underscores the need to assess language, and especially pragmatic skills, and to study long-term outcome in such childhood epileptic syndromes. [source]

Transient epileptic amnesia: a new epileptic syndrome in development?

ANNALS OF NEUROLOGY, Issue 3 2010
Pasquale Striano MD
No abstract is available for this article. [source]

Complex epileptic (Foix,Chavany,Marie like) syndrome in a child with neurofibromatosis type 1 (NF1) and bilateral (opercular and paracentral) polymicrogyria

ACTA PAEDIATRICA, Issue 4 2009
Mario Mastrangelo
Abstract The association of brain malformations and symptomatic epilepsy in the setting of neurofibromatosis type 1 (NF1) is rarely reported. When it occurs, patients can present clinically with infantile spasms, focal seizures, generalized tonic clonic seizures or atypical absences. We report on a 10-year-old (molecularly proven) NF1 girl manifesting a complex epileptic syndrome resembling the Foix,Chavany,Marie spectrum (also known as opercular syndrome) associated with bilateral (opercular and paracentral lobular) polymicrogyria (PMG). Anecdotal cases of unilateral PMG in the setting of NF1 have been described in association with other-than-opercular epileptic syndromes. The typical clinical opercular syndrome consisting in mild mental retardation, epilepsy and pseudobulbar palsy is usually associated to bilateral perisylvian PMG (BPP) Conclusion: To the best of our knowledge, the complex epileptic syndrome hereby reported has not been previously recorded in the setting of NF1. In addition, the present girl manifested all the clinical features of an opercular syndrome but had an asymmetrical PMG (not a BPP). [source]

Coexistence of symptomatic focal and absence seizures: Video-EEG and EEG-fMRI evidence of overlapping but independent epileptogenic networks

EPILEPSIA, Issue 7 2009
Serge Chassagnon
Summary The distinction between typical absences and hypomotor seizures in patients having frontal lesions is difficult. In focal epilepsy, generalized-like interictal discharges can reflect either a coexistent generalized epileptic trait or a secondary bilateral synchrony. Using combined measures of the EEG and blood oxygenation level dependent (BOLD) activity, we studied a 50-year-old patient with both absence-like and symptomatic focal motor seizures. Focal activity induced activation in the lesional area and deactivation in the contralateral central cortex. Generalized spike-and-wave discharges (GSWDs) resulted also in perilesional activation, and multifocal symmetrical cortical and thalamic activations, and deactivation in associative cortical areas. Although the central cortex was involved during both types of epileptic activity, electroencephalography (EEG),functional magnetic resonance imaging (fMRI) revealed distinct neuronal networks at the time of the focal or generalized discharges, allowing a clear-cut differentiation of the generators. Whether the patient had distinct epileptic syndromes or distinct electrographic patterns from the lesional trigger remains debatable. [source]

A new animal model of infantile spasms with unprovoked persistent seizures

EPILEPSIA, Issue 2 2008
Chong L. Lee
Summary Purpose: Infantile spasms is one of the most severe epileptic syndromes of infancy and early childhood. Progress toward understanding the pathophysiology of this disorder and the development of effective therapies has been hindered by the lack of a relevant animal model. We report here the creation of such a model. Methods: The sodium channel blocker, tetrodotoxin (TTX), was chronically infused into the developing neocortex or hippocampus of infant rats by way of an osmotic minipump starting on postnatal day 10,12. Results: After a minimum of 10 days of infusion, approximately one-third of these rats began to display very brief (1,2 s) spasms, which consisted of symmetric or asymmetric flexion or extension of the trunk and sometimes involvement of one or both forelimbs. The typical ictal EEG pattern associated with the behavioral spasms consisted of an initial generalized, high amplitude, slow wave followed by an electrodecrement with superimposed fast activity. The interictal EEG revealed multifocal spikes and sharp waves, and in most animals that had spasms a hypsarrhythmic pattern was seen, at least intermittently, during NREM sleep. Like in humans, the spasms in the rat often occurred in clusters especially during sleep,wake transitions. Comparison of the ictal and interictal EEGs recorded in this model and those from humans with infantile spasms revealed that the patterns and the frequency components of both the ictal events and hypsarrhythmia were very similar. Discussion: The TTX model of infantile spasms should be of value in furthering an understanding of the pathophysiology of this seizure disorder. [source]

Epilepsy and Language Development: The Continuous Spike-Waves during Slow Sleep Syndrome

Coexistence of Idiopathic Rolandic Epilepsy and CSWS in Two Families

EPILEPSIA, Issue 10 2006
Xavier De Tiège
Summary:,Purpose: To report two families combining benign childhood epilepsy with centrotemporal spikes (BCECS) and cryptogenic epilepsy with continuous spike,waves during sleep (CSWS) in first-degree relatives. Methods: Clinical, EEG, and cerebral imaging data are described. Results: Family 1: The proband was 3 years old at epilepsy onset. First seizures were convulsive, with centrotemporal spikes on EEG. At age 5 years, he had complex partial seizures, psychomotor regression, and centrotemporal CSWS. [18F]fluorodeoxyglucose (FDG) positron emission tomography (PET) showed left parietal hypermetabolism. After several antiepileptic drug (AED) trials, valproate (VPA) and ethosuximide (ESM) induced seizure remission, CSWS disappearance, and psychomotor improvement. Learning disabilities, however, persisted. Family history was remarkable for BCECS in his father. Family 2: The proband was 2 years old at epilepsy onset. First seizures were convulsive, with centrotemporal CSWS on EEG. Despite several AED trials including corticosteroids, focal negative myoclonia, atypical absences, and psychomotor regression occurred, leading to severe mental retardation. FDG-PET showed bilateral parietal hypermetabolism. Vagus nerve stimulator was implanted. Her family history was remarkable for BCECS in her father and febrile convulsions in infancy in her mother. Conclusions: These data suggest the existence of a common genetic basis between BCECS and cryptogenic epilepsies with CSWS. The higher expression in patients with CSWS could be related to other genetic or acquired factors. These data suggest that these epileptic syndromes constitute edges of a continuum. [source]

Prevalence of Childhood Epilepsy and Distribution of Epileptic Syndromes: A Population-based Survey in Okayama, Japan

Specific Epileptic Syndromes Are Rare Even in Tertiary Epilepsy Centers: A Patient-oriented Approach to Epilepsy Classification

Absence Epilepsy with Onset before Age Three Years: A Heterogeneous and Often Severe Condition

EPILEPSIA, Issue 7 2003
Yves Chaix
Summary: Purpose: The classification of epilepsies and epileptic syndromes recognizes three syndromes with typical absences [TA, i.e., childhood and juvenile absence epilepsies (CAE and JAE), and epilepsy with myoclonic absences (EMA), none of which is characterized by onset in early childhood]. Although several other forms of absence epilepsies have been described recently, none concerns infants and very young children, and little is known about the nosology and prognosis of early-onset absences. Methods: We retrospectively selected all cases with onset of absences as the only or major seizure type before age 3 years and ,2 years of follow-up among cases newly referred between 1986 and 2002. Neurospychological assessments (generally IQ measure), behavior patterns, and schooling situations were reviewed for each child. Results: We found 10 patients (7 F, 3 M). No child had sensory or motor deficits: neuroimaging was performed in nine and was normal in eight, with aspecfic findings in one. Only two could be characterized as CAE and EMA, respectively, both with seizure control and a good cognitive outcome. Among the remaining eight cases, four had a fairly homogeneous presentation with predominantly brief absences and clearly asymmetric interictal EEGs. All eight had neuropsychological and/or behavioral difficulties. Three had full seizure control, and five, persisting absences, with a follow-up ranging beetween 2 years 8 months to 9 years 4 months; only one child was older than 12 years. Conclusions: Great heterogeneity exists among absence epilepsies of early onset, which are rare conditions. Only a few patients can be categorized into well-known syndromes. The overall prognosis is poor. Early onset of absences is uncommon, and multicenter studies should help clarify the nosology and prognosis. [source]

Nonsymptomatic Generalized Epilepsy in Children Younger than Six Years: Excellent Prognosis, but Classification Should Be Reconsidered after Follow-up: The Dutch Study of Epilepsy in Childhood

EPILEPSIA, Issue 7 2002
C. M. Middeldorp
Summary: ,Purpose: To assess the prognosis and the accuracy of the epilepsy classification in young children with nonsymptomatic generalized epilepsy. Methods: Of the cohort of the Dutch Study of Epilepsy in Childhood (n = 466), all children younger than 6 years with a diagnosis of idiopathic (IGE) or cryptogenic (CGE) generalized epilepsy either at intake (n = 108) and/or after 2 years of follow-up (n = 102) were included. The number of reclassifications after 2 years was determined, and the reasons for reclassification were analyzed. All children receiving a diagnosis of IGE or CGE at 2 years were followed up for 5 years to study their outcome in terms of terminal remission (TR). Data on their level of intellectual functioning were collected at the start of this analysis. Results: The epilepsy syndrome was reclassified in 17 children. In 14 of them, the seizure type also was reclassified, and in three, the course of the epilepsy determined the new epilepsy type. Two other children had a reclassification of their seizure types without a change of the epilepsy type. Many children were categorized as having IGE not otherwise specified. In all probability, this is a heterogeneous group, containing patients with various epilepsy syndromes, with generalized tonic,clonic seizures as a common hallmark. Of the 102 children with IGE or CGE at 2 years of follow-up, 75% had a TR of >6 months after 2 years, and 85% a TR of ,1 year after 5 years. Conclusions: In a fair proportion of children with nonsymptomatic generalized epilepsy in this age group, it is not possible to classify firmly the epilepsy and/or the seizures immediately after the intake. Instead, they are reclassified during the course of the disease. This and the apparent heterogeneity of the category IGE not otherwise specified point to inherent drawbacks of the current International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The prognosis of IGE at this young age is generally excellent. [source]

Ictal EEG Patterns in Band Heterotopia

EPILEPSIA, Issue 4 2002
Arthur C. Grant
Summary: Band heterotopia (BH) or "double cortex" syndrome is a neuronal migration disorder resulting in a diffuse band of subcortical grey matter and variable abnormality of the overlying cortex. Patients with BH have a spectrum of psychomotor delay and seizures. Associated epileptic syndromes and interictal EEG findings have been described, but ictal EEG patterns are lacking. Methods: We describe the clinical, interictal, and ictal EEG findings in two girls with BH and intractable seizures. Results: Ictal EEG patterns correlated well with clinical seizure types, and did not have features unique to BH. Similarly, seizure behaviors and interictal EEG findings were typical of those seen in symptomatic generalized epilepsies. Conclusions: Despite evidence implicating the ectopic grey matter in seizure discharges, we conclude that seizure semiology and associated ictal EEG patterns in BH are no different from those seen in other causes of symptomatic generalized epilepsies. [source]

Photosensitivity in Relation to Epileptic Syndromes: A Survey from an Epilepsy Center in Japan

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]

Enhanced synaptic excitation,inhibition ratio in hippocampal interneurons of rats with temporal lobe epilepsy

EUROPEAN JOURNAL OF NEUROSCIENCE, Issue 2 2007
F. Stief
Abstract A common feature of all epileptic syndromes is the repetitive occurrence of pathological patterns of synchronous neuronal activity, usually combined with increased neuronal discharge rates. Inhibitory interneurons of the hippocampal formation control both neuronal synchronization as well as the global level of activity and are therefore of crucial importance for epilepsy. Recent evidence suggests that changes in synaptic inhibition during temporal lobe epilepsy are rather specific, resulting from selective death or alteration of interneurons in specific hippocampal layers. Hence, epilepsy-induced changes have to be analysed separately for different types of interneurons. Here, we focused on GABAergic neurons located at the border between stratum radiatum and stratum lacunosum-moleculare of hippocampal area CA1 (SRL interneurons), which are included in feedforward inhibitory circuits. In chronically epileptic rats at 6,8 months after pilocarpine-induced status epilepticus, frequencies of spontaneous and miniature inhibitory postsynaptic currents were reduced, yielding an almost three-fold increase in excitation,inhibition ratio. Consistently, action potential frequency of SRL interneurons was about two-fold enhanced. Morphological alterations of the interneurons indicate that these functional changes were accompanied by remodelling of the local network, probably resulting in a loss of functional inhibitory synapses without conceivable cell death. Our data indicate a strong increase in activity of interneurons in dendritic layers of the chronically epileptic CA1 region. This alteration may enhance feedforward inhibition and rhythmogenesis and , together with specific changes in other interneurons , contribute to seizure susceptibility and pathological synchronization. [source]

Complex epileptic (Foix,Chavany,Marie like) syndrome in a child with neurofibromatosis type 1 (NF1) and bilateral (opercular and paracentral) polymicrogyria