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Results for “Epilepsy, Generalized”

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PubMed2002

Non convulsive status epilepticus after replacement of valproate with lamotrigine.

PURPOSE: Lamotrigine (LTG) and valproate (VPA) are widely used in the treatment of generalized epilepsies. Nevertheless seizure aggravation with LTG has been reported in juvenile myoclonic epilepsy and epilepsy with myoclonic absences. The aim of this study was to describe clinical features and EEG findings in patients with non convulsive status epilepticus (NCSE) after replacement of VPA with LTG. METHOD: Retrospective analysis of three identified patients in a large university hospital. RESULTS: We describe three adult patients aged 39, 50 and 75 years, who developed non-convulsive status epilepticus (NCSE) after replacement of VPA with LTG. The episodes of NCSE presented as an acute confusional state with mild myoclonus. Ictal EEG showed generalized spike-wave or polyspike wave activity. The clinical symptoms and the EEG responded promptly to IV benzodiazepines and patients remained well controlled with dose reduction of LTG (in one patient) or discontinuation of LTG (in two patients). CONCLUSION: The reported episodes of NCSE in these patients with generalized epilepsy may be either due to a possible paradoxical reaction to LTG or due to a withdrawal effect of VPA.

Adult↗

PubMedDate not supplied

Thalamic regulation of epileptic spike and wave discharges.

Idiopathic generalized epilepsy is characterized by the spontaneous occurrence on the electroencephalogram of bilateral synchronous spike and wave discharges that involve the entire cortical mantle from the very onset. A central role in cortical synchronization is played by the thalamus, and in particular by the reticularis thalami nucleus, which is regarded as the pacemaker structure for the rhythmic cortical oscillations called spindle activity. Several studies have suggested that the network mechanisms which underlie spindle generation can also account for spike and wave discharges. Experimental investigations on genetic animal models of generalized epilepsy, in which seizures occur spontaneously in the absence of any inductive agent, confirmed that the thalamo-cortical system is involved in spike and wave discharge generation. According to these studies a genetically determined dysfunction in reticularis thalami neurons may alter the electroresponsiveness of the developing thalamo cortical system and cause a persistent state of abnormal excitation.

Animals↗

PubMed2002

Mutation of GABRA1 in an autosomal dominant form of juvenile myoclonic epilepsy.

Although many genes that predispose for epilepsy in humans have been determined, those that underlie the classical syndromes of idiopathic generalized epilepsy (IGE) have yet to be identified. We report that an Ala322Asp mutation in GABRA1, encoding the alpha1 subunit of the gamma-aminobutyric acid receptor subtype A (GABA(A)), is found in affected individuals of a large French Canadian family with juvenile myoclonic epilepsy. Compared with wildtype receptors, GABA(A) receptors that contain the mutant subunit show a lesser amplitude of GABA-activated currents in vitro, indicating that seizures may result from loss of function of this inhibitory ligand-gated channel. Our results confirm that mutation of GABRA1 predisposes towards a common idiopathic generalized epilepsy syndrome in humans.

Adolescent↗

PubMed1998

West syndrome and its related epileptic syndromes.

The purpose of this project was to study the relationship between West syndrome (WS) and its related epileptic syndromes, and reconsider the nosological limits of WS. The electroclinical features of 45 patients who experienced spasms in series were investigated, as well as some features not common in patients with WS. All patients were mentally retarded. The patients were divided into three groups: Group 1 consisted of 12 patients with refractory epilepsies with onset in early infancy, group 2 consisted of 5 patients with symptomatic localization-related epilepsies associated with spasms in series, and group 3 consisted of 28 patients with generalized epilepsies who had spasms in series after age 2 years. Partial seizures were the dominant symptom throughout the clinical course and spasms in series associated with atypical hypsarrhythmia appeared transiently during infancy in a significant number of the patients in group 1. In group 2, complex partial seizures (CPS) were the main seizure type and hypsarrhythmia was not observed during the clinical course. The EEGs in group 3 patients showed diffuse slow spike-waves or multifocal epileptic discharges in all but 1 patient. The EEG of the remaining patient still showed hypsarrhythmia at age 8 years. Therefore, group 1 patients should be classified as having WS although cortical mechanisms play a critical role in the occurrence of their seizures. Group 2 patients should be considered as having a type of localization-related epilepsy even though they share a similar pathophysiological mechanism with group 1. In group 3, 1 patient whose EEG still showed hypsarrhythmia was classified as having WS. The other patients should be classified as having generalized epilepsies other than WS.

Adolescent↗

PubMed1984

[Changes in paroxysmal activity, spectral characteristics of the EEG and visual evoked potentials after sleep deprivation in patients with epilepsy and syncope].

The effect of 24-hour sleep deprivation (SD) on a number of electrophysiological indices in epilepsy and syncope was studied. Specific electroencephalographic manifestations were shown to be markedly activated which is important for the differential diagnosis of epilepsy and syncope. Following SD, patients with primary generalized epilepsy exhibited an increased spectral power of the 0- and alpha-rhythms and an elevated amplitude of visual evoked potential components (beginning with the H2 component), whereas patients with secondary generalized epilepsy had an enhanced amplitude of the late components of visual evoked potentials (P4, H4). The mechanisms of the proconvulsive effect of SD are discussed.

Adult↗

PubMed2004

Efficacy and tolerability of the new antiepileptic drugs II: treatment of refractory epilepsy [RETIRED]: report of the Therapeutics and Technology Assessment Subcommittee and Quality Standards Subcommittee of the American Academy of Neurology and the American Epilepsy Society.

OBJECTIVE: To assess the evidence demonstrating efficacy, tolerability, and safety of seven new antiepileptic drugs (AEDs) (gabapentin, lamotrigine, topiramate, tiagabine, oxcarbazepine, levetiracetam, and zonisamide) in the treatment of children and adults with refractory partial and generalized epilepsies. METHODS: A 23-member committee including general neurologists, pediatric neurologists, epileptologists, and doctors in pharmacy evaluated the available evidence based on a structured literature review including MEDLINE, Current Contents, and Cochrane library for relevant articles from 1987 until March 2003. RESULTS: All of the new AEDs were found to be appropriate for adjunctive treatment of refractory partial seizures in adults. Gabapentin can be effective for the treatment of mixed seizure disorders, and gabapentin, lamotrigine, oxcarbazepine, and topiramate for the treatment of refractory partial seizures in children. Limited evidence suggests that lamotrigine and topiramate are also effective for adjunctive treatment of idiopathic generalized epilepsy in adults and children, as well as treatment of the Lennox Gastaut syndrome. CONCLUSIONS: The choice of AED depends upon seizure and/or syndrome type, patient age, concomitant medications, AED tolerability, safety, and efficacy. The results of this evidence-based assessment provide guidelines for the prescription of AEDs for patients with refractory epilepsy and identify those seizure types and syndromes where more evidence is necessary.

Acetates↗

PubMed2006

The effect of valproate on silent period and corticomotor excitability.

OBJECTIVE: To investigate, by transcranial magnetic stimulation, the effects of valproate on silent period and corticomotor excitability. METHODS: thirty patients with generalized epilepsy were studied at baseline, and re-examined 4 (S1) and 25 (S2) weeks after the administration of valproate (mean dose: 1040 +/- 284 mg). Transcranial magnetic stimulation was performed with a figure of eight coil (recording, first dorsal interosseous). Threshold was measured at 1% steps. Silent period was measured using a recently described protocol. Briefly, silent periods were elicited at 5% increments from 0 to 100% maximum stimulus intensity. At each stimulus intensity, 4 silent periods were obtained and the average value of silent period duration was used to construct a stimulus/response curve of stimulus intensity versus silent period. The resulting curves were then fitted to a Boltzman function and were statistically compared. The motor-evoked potential recruitment curve was constructed under active conditions and analyzed in a similar way. RESULTS: Valproate increased threshold from 36.5 +/- 5.99% at baseline to 41.02 +/- 7.84% at S1 (p < 0.0001, paired t-test). The maximum value of the silent period curve decreased from 257.5 +/- 3.9 ms at baseline to 230.3 +/- 3.9 ms at S1 (p < 0.0001, F-test and AIC) while the other best-fit values (V(50), slope, threshold) were not significantly affected. Regarding the motor-evoked potential recruitment curve, the maximum value decreased significantly post-drug (from 0.449 +/- 0.007 to 0.392 +/- 0.009, p < 0.01, F-test and AIC test), whereas the rest of the best-fit values remained unaffected. CONCLUSION: In patients with idiopathic generalized epilepsy, valproate increases threshold and reduces the maximum values of the silent period curve and the motor-evoked potential recruitment curve. These findings probably reflect valproate's effects on voltage-dependent Na(+) channels, as well as an activation of GABA(A) receptors.

Adolescent↗

PubMed2006

Role of valproate across the ages. Treatment of epilepsy in children.

In June 2005 a team of experts participated in a workshop with the objective of reaching agreement on the place of valproate use in the treatment of paediatric epilepsy patients. A general "consensus of the meeting" was that the initiation of antiepileptic drug (AED) treatment should be based on a seizure-syndromic approach in children. Participants of the meeting also agreed that valproate is currently the AED with the broadest spectrum across all types of seizures and syndromes. Its superiority has been shown over almost 40 years of clinical experience. The best results are seen in idiopathic generalized epilepsy with or without photosensitivity, idiopathic focal and symptomatic generalized tonic-clonic seizures (GTCS). Evidence supports the use of valproate, ethosuximide and lamotrigine in absence epilepsies and the use of carbamazepine, lamotrigine, oxcarbazepine, phenytoin, topiramate, valproate and phenobarbital for primary GTCS. For new AEDs trials have been undertaken to define their therapeutic role but studies comparing their role to 'old' broad-spectrum drugs in specific syndromes are missing. Experts concluded that intravenous (i.v.) valproate is a useful agent in the treatment of non-convulsive status epilepticus (SE). There is an easy transition to oral treatment following i.v. valproate use. The discussion also concluded that, despite the lack of studies, valproate is an interesting, underutilized alternative in convulsive SE but more controlled studies are needed. The side effects of valproate use are well documented. Its effect on cognition and behaviour is more favourable than many of the other AEDs which is an important consideration in children. Overall, the clinical consensus of the meeting was that valproate's well established therapeutic properties far outweigh the negative side effects. Contraindication or withdrawal should be assessed individually.

Anticonvulsants↗

PubMed2006

Genes and loci involved in febrile seizures and related epilepsy syndromes.

Epilepsy is a paroxysmal disorder with a cumulative incidence of about 3%. About 13% of patients with epilepsy have a history of febrile seizures (FS). Generalized epilepsy with FS plus (GEFS+) is a familial epilepsy syndrome in which patients can have classic FS, FS that persist beyond the age of 5 years (i.e., FS+), and/or epilepsy. Both genetic and environmental factors have been shown to contribute to the pathogenesis of FS and GEFS+. During the past 10 years, molecular genetic studies have contributed a great deal to the identification of genetic factors involved in FS and GEFS+. In this study we aimed to provide a comprehensive review of currently known genes for FS and GEFS+, and the methods and approaches used to identify them. We also discuss the knowledge we currently have and hypotheses regarding the effect of the mutations on their respective protein functions.

Epilepsy, Absence↗

PubMed2005

Obsessionality, obsessive-compulsive disorder, and temporal lobe epilepsy.

We evaluated the prevalence of obsessive-compulsive disorder (OCD) in patients with temporal lobe epilepsy (TLE) and we investigated the hypothesis that obsessionality may represent a trait in TLE. Eighty-two consecutive patients with epilepsy, 62 with TLE and 20 with idiopathic generalized epilepsy (IGE), and 82 matched healthy controls were evaluated using the SCID-IP, Y-BOCS, MMPI-2 (specifically the Psychasthenia and Obsessiveness scales), BDI, and STAI Y1 and Y2. Nine of the TLE patients, none of the IGE patients, and one of the controls had a diagnosis of OCD. Psychasthenia and Obsessiveness scores were significantly higher in the TLE than in the IGE and control groups. Patients with TLE and OCD differed significantly with respect to history of depression when compared with patients with TLE without OCD, whereas there were no differences in age at onset and duration of epilepsy, seizure pattern and frequency, MRI features, laterality of the EEG focus, antiepileptic drug therapy and combinations, and BDI scores.

Adolescent↗

PubMed1981

[Frequency and semeiological evolution of epileptic seizures occurring between 40 and 65 years of age (author's transl)].

458 patients who had their first epileptic fit between the ages of 40 and 65 are classified into 5 groups according to the seizure symptomatology: when the symptomatology remains unchanged, the frequency of fits is less than 1 per month in 84% of generalized fits and more than 1 per month in 52% of focal fits. When the symptomatology changes, the frequency of fits is less than 1 per month when the inaugural fit is generalized (23 out of 27) and more than 1 per month when the inaugural fit is focal (15 out of 21). Modifications of the symptomatology correlate positively with a prevalence of tumor aetiology. Primary generalized epilepsies are rare (4.4%). Non-specific secondary generalized epilepsies (including vascular, metabolic or toxic encephalopathies) account for 24.4% of the cases. Focal epilepsies are the most common form (40.7%), elementary fits being four times more frequent than complex fits.

Adult↗

PubMed2004

Therapy-resistant epilepsy in children: with the view of the long-term outcome.

Of 362 children with therapy-resistant epilepsy, 119 patients had generalized tonic seizures, which were the most common seizure type in patients with generalized epilepsy. The long-term observation of the patients with tonic seizures revealed that their seizures were completely controlled in 27 patients for more than 5 years. The effective antiepileptic drugs were VPA (10 patients), PHT (5), CLB (4), ZNS (2), VPA + PHT (4), ZNS + CLB (1) and VPA + AZA (1). For obtaining improvement of tonic seizures, another strong factor is reducing the number of antiepileptic drugs, such as the average number of drugs, which was 4.7 to 2.4 in these 27 patients.

Anticonvulsants↗

PubMedDate not supplied

Treatment of intractable childhood epilepsy with high-dose valproate.

Forty-six children with refractory epilepsy (12 with symptomatic generalized epilepsy, 14 with symptomatic partial epilepsy, and 20 with undetermined epilepsy) were treated by high-dose (serum level above 100 micrograms/ml) valproate (VPA) therapy. Monotherapy was used with 34 patients and two drugs with 12. Serum VPA concentrations ranged from 105.1 to 198.4 micrograms/ml. Assessment of initial response to treatment, after the serum level had reached the appropriate level, showed seizures to be completely controlled in 15 (32.6%) of 46 patients and improved in 12 (26.1%) (50% or more). Follow-up of more than 6 months after the time of initial response showed control of seizures in 14 (30.4%) and improvement in 11 (23.9%). The initial effect on EEG was the disappearance of epileptic discharges in 3 (6.5%) of 46 patients and marked improvement in 15 (32.6%). Follow-up revealed the disappearance of epileptic discharges in 7 (15.2%) and marked improvement in 9 patients (19.6%). High-dose VPA therapy was especially effective for West syndrome and for epilepsy with continuous spike-waves during slow-wave sleep. Control of atypical absences and myoclonic seizures was relatively good. Hypofibrinogenemia and thrombocytopenia were sometimes encountered but these side effects were reversible with reduction of dosage.

Adolescent↗

PubMed2006

Prevalence of childhood epilepsy and distribution of epileptic syndromes: a population-based survey in Okayama, Japan.

PURPOSE: A population-based survey childhood epilepsy was undertaken in Okayama Prefecture, Japan, to determine the prevalence rate and the distribution of epilepsies and epileptic syndromes according to the International Classification (ILAE, 1989). 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).

Age Factors↗

PubMed1989

The localization-related epilepsies: some problems with subclassification.

One of the nosological problems of childhood epilepsies is the evolutionary change of foci with age. Benign childhood epilepsy with centro-temporal spike may evolve to show EEG features of childhood epilepsy with occipital paroxysms and vice versa. West syndrome is generally accepted as generalized epilepsy but is often preceded or followed by partial seizures and may have both partial seizures and tonic spasms in a single attack. Among epilepsies with both generalized and focal seizures, severe myoclonic epilepsy in infancy is more linked to generalized epilepsy whereas epilepsy with continuous spike-waves during slow wave sleep is more linked to localization-related epilepsy. Any type of partial epilepsy may develop nonconvulsive generalized seizures transiently with generalization of paroxysmal discharges during the clinical course. Moreover, nonconvulsive generalized seizures may occur even in localization-related epilepsy with spike-wave index during NREM sleep in less than 85%.

Adult↗

PubMed2003

Oxcarbazepine in the treatment of childhood epilepsy.

In this study, oxcarbazepine was began as monotherapy to evaluate the efficacy and safety of the drug. Forty-two patients (19 females, 23 males) with partial or generalized epilepsy more than 4 years of age were included (mean age, 11.9 +/- 3.4 years). The mean age at epilepsy onset 8.9 +/- 4 years. Complete blood count, liver function tests, electrolytes, lipid levels, electrocardiography, electroencephalography, and magnetic resonance imaging were performed in all patients. Oxcarbazepine dose was begun at 10 mg/kg/day twice daily and increased to 30 mg/kg/day at the end of the second week. Patients with inadequate seizure control even with the dose of 45 mg/kg/day or intolerable side effects were excluded. Intolerable headache and leukopenia led to discontinuation of the drug in two patients. At the sixth month, 35 of the patients (87.5%) were seizure free (91.7% of the generalized epilepsy patients and 81.2% of the partial epilepsy patients). The most frequent tolerable side effect was drowsiness in 12 patients. As a result, we found oxcarbazepine safe and effective in children with either generalized or partial epilepsy.

Adolescent↗

PubMed1997

Prevalence of childhood epilepsy in Kaunas, Lithuania.

The prevalence of epilepsy in children aged 0-15 years of Kaunas city, Lithuania, was evaluated on 1 January 1995. Multiple sources for case identification were used, i.e. medical records at the university hospital, regional outpatient clinics and consultation centres, institutions, schools and kindergartens for the handicapped. Active epilepsy was defined as two or more unprovoked epileptic seizures with at least one seizure occurring within the previous 5 years, regardless of the antiepileptic drug treatment. Prevalence was found to be 4.25 (3.42, if age-standardized) in 1000. The highest rate was found in the 10-14 years age group. The male/female ratio was 1.29. No possible causes could be determined in 60.3% of cases. Congenital causes were diagnosed in 18.8% of cases, perinatal causes in 15.3%, traumatic causes in 2.6% and neuroinfectious causes in 2.4%. Classification of epilepsies and epileptic syndromes [Commission on Classification and Prognosis of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989; 30:389-399] revealed that 50% of cases were localization-related epilepsies, 29.9% were generalized epilepsies, 15.9% were undetermined whether partial or generalized and 4.2% were unclassifiable. Rates for idiopathic, symptomatic and cryptogenic cases are presented.

Adolescent↗

PubMed2004

Efficacy and tolerability of the new antiepileptic drugs, II: Treatment of refractory epilepsy: report of the TTA and QSS Subcommittees of the American Academy of Neurology and the American Epilepsy Society.

PURPOSE: To assess the evidence demonstrating efficacy, tolerability, and safety of seven new antiepileptic drugs (AEDs) [gabapentin (GBP), lamotrigine (LTG), topiramate (TPM), tiagabine (TGB), oxcarbazepine (OXC), levetiracetam (LEV), and zonisamide (ZNS)] in the treatment of children and adults with refractory partial and generalized epilepsies. METHODS: A 23-member committee, including general neurologists, pediatric neurologists, epileptologists, and doctors in pharmacy, evaluated the available evidence based on a structured literature review including MEDLINE, Current Contents, and Cochrane Library for relevant articles from 1987 to March 2003. RESULTS: All of the new AEDs were found to be appropriate for adjunctive treatment of refractory partial seizures in adults. GBP can be effective for the treatment of mixed seizure disorders, and GBP, LTG, OXC, and TPM for the treatment of refractory partial seizures in children. Limited evidence suggests that LTG and TPM also are effective for adjunctive treatment of idiopathic generalized epilepsy in adults and children, as well as treatment of the Lennox-Gastaut syndrome. CONCLUSIONS: The choice of AED depends on seizure and/or syndrome type, patient age, concomitant medications, and AED tolerability, safety, and efficacy. The results of this evidence-based assessment provide guidelines for the prescription of AEDs for patients with refractory epilepsy and identify those seizure types and syndromes for which more evidence is necessary.

Acetates↗