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Results for “Epilepsy, Generalized”

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PubMed1996

Felbamate in therapy-resistant epilepsy: an Italian experience. Felbamate Italian Study Group.

We evaluated the use of felbamate in 379 adults and children with refractory epilepsies in an open-label, compassionate clinical use setting. Prior to the termination of the program, because of reports of aplastic anemia, 351 patients had completed 2 months of treatment with felbamate at a dose of 2400-3600 mg/day for adults or 30-45 mg/kg/day for children. Of the 246 patients who had a diagnosis of therapy-refractory localization-related epilepsy with or without secondary generalization, 52% (126/246) achieved a seizure reduction of 50% or more, including 10% (25/246) who became seizure free. There was no difference in response rate between adults and children. Of the 80 patients who had a diagnosis of Lennox-Gastaut syndrome (LGS), 60% (48/80) achieved a seizure reduction of 50% or more, including 6% (5/80) who became seizure free. Of the 25 patients with a diagnosis of generalized epilepsy (other than LGS) or undetermined epilepsy whether focal or generalized, 60% (15/25) achieved a seizure reduction of 50% or more, including 12% (3/24) who became seizure free. The results of this uncontrolled study suggest that felbamate could be useful in patients with epilepsies which are refractory to other antiepileptic drugs after careful risk-benefit assessment and consideration of all circumstances involved.

Adolescent↗

PubMed2003

Genetic influences on myoclonic and absence seizures.

OBJECTIVE: To examine the relationship between genotype and phenotype in idiopathic generalized epilepsies (IGEs) using a novel approach that focuses on seizure type rather than syndrome. METHODS: The authors evaluated whether the genetic effects on myoclonic seizures differ from the genetic effects on absence seizures. For this purpose, they studied 34 families containing 2 or more members with IGEs and assessed whether the number of families concordant for seizure type exceeded that expected by chance. The authors performed a similar analysis to examine the genetic contributions to juvenile myoclonic epilepsy (JME), juvenile absence epilepsy (JAE), and childhood absence epilepsy (CAE). RESULTS: The observed number of families concordant for seizure type (myoclonic, absence, or both) was greater than expected (20 vs 7.51; p < 0.0001). The observed number of families concordant for syndrome was greater than expected when JME was compared with absence epilepsies (JAE+CAE) (17 vs 11.9; p < 0.012) but not when JAE was compared with CAE (8 vs 6.82; p = 0.516). CONCLUSIONS: These results provide evidence for distinct genetic effects on absence and myoclonic seizures, suggesting that examining the two seizure types separately would be useful in linkage studies of idiopathic generalized epilepsies. The approach presented here can also be used to discover other clinical features that could direct division of epilepsies into groups likely to share susceptibility genes.

Adolescent↗

PubMed1988

Corpus callosotomy in the treatment of secondary generalizing intractable epilepsy.

Over the past 5 years 9 patients underwent partial or complete corpus callosotomy for treatment of medically intractable epileptic seizures. A beneficial effect of the procedure was observed in 8 patients. Criteria for performance of the procedure included CT evidence of unilateral cerebral damage and non-localized epileptic foci in that hemisphere with secondary spread to the uninvolved side. The best results were obtained in atonic seizures. Long-term side effects were minor. The procedure is recommended in those patients with medically uncontrolled atonic or secondary generalized motor seizures in whom the corpus callosum is thought to mediate the spread of epileptic activity from a damaged cerebral hemisphere. Side effects are minimized by staging of the callosal section. If a partial callosotomy was beneficial, complete section can then be avoided.

Adolescent↗

PubMed1996

Circling seizures.

Twelve cases with circling seizures are presented with their clinical, electroencephalographic and radiological findings. Four patients had symptomatic partial epilepsy, five had cryptogenic partial epilepsy, and the remaining three had idiopathic generalized epilepsy. Three of the patients with symptomatic partial epilepsy had frontal lesion, and one had parito-occipital lesion. Turning direction had no lateralizing value in patients with partial epilepsy. Based on our study we conclude that circling seizures may occur in different epileptic syndromes and epilepsies. In cases with symptomatic partial epilepsy, lesions are mostly located in frontal lobes but also in parietooccipital areas.

Adult↗

PubMed2005

[Efficacy of keppra in combined therapy in pharmacoresistant adult epilepsy patients].

There were analyzed efficacy of keppra (Levetiracetam) in combined therapy in 31 patients with pharmacoresistant partial epilepsy and in 2 cases with idiopathic generalized epilepsy. During the 4 months 48.4% experienced at least a 50% reduction in the frequency of partial-oncet seizures, 12.9% at least a 75% reduction, and 25.8% demonstrated a seizure free. The combined therapy was ineffective only in 12.9%. The authors emphasize a good tolerability of keppra. There was only one case with side-effect, that demand cancel of Levetiracetam. The results obtained reveal effectiveness of keppra using in epileptology, in particular, in pharmacoresistant epilepsy.

Adult↗

PubMed2006

Game-related seizures presenting with two types of clinical features.

We evaluated 22 patients with epileptic seizures in which the seizures were triggered by various games or game-related materials. Based on whether spontaneous seizure coexisted or not, these 22 patients were divided into two groups. Ten patients who experienced seizures exclusively while playing or watching specific games were referred to as Group I, while 12 patients that had both game-induced and spontaneous seizures were classified as Group II. The patients in Group I had a middle-age onset (39.1 years) with a male predominance (90%). The electroencephalogram (EEG) or brain magnetic resonance imaging revealed non-specific abnormalities in 60%, and the partial onset seizure was recognized in 30% of patients. Antiepileptic drugs had uncertain benefits in this group. In Group II, patients had a male predominance (67%), with onset during adolescence (16.3 years). Most of them had generalized tonic-clonic seizures, myoclonic seizures, and absences, and 42% showed epileptiform discharge on EEG. These 12 patients were categorized into idiopathic generalized epilepsies. Although photosensitivity was an important factor, higher mental activity seemed to be significant precipitants of seizures in Group II. Antiepileptic drugs were necessary and valproic acid alone or combined with clonazepam was effective in this group. The results showed that game-related seizures are not a unique and homogeneous syndrome and may consist of different mechanisms. Teenage onset, coexistent spontaneous seizure, and associated idiopathic generalized epilepsies were crucial factors in the determination of antiepileptic drug therapy. Moreover, avoiding the related games altogether may be a more productive preventive measure.

Adolescent↗

PubMed1992

Bromide treatment of pharmaco-resistant epilepsies with generalized tonic-clonic seizures: a clinical study.

In a retrospective controlled clinical study we investigated the efficacy of bromides (BR) as to the frequency of generalized tonic-clonic seizures (GTCS) as a monotherapy (n = 5) or combined with other antiepileptic drugs (AEDs) (n = 55), and compared the results statistically with those achieved with a treatment based on phenobarbitone (PB) and/or phenytoin (PHT) in a very similar group of another 60 patients. All patients treated with BR had previously proved to be pharmaco-resistant. In most of the cases the epilepsies were based on early cerebral lesions, and were partly associated with severe mental and physical handicaps. Most of the patients were between three and 14 years old. The percentage of responders was 58%, which means a reduction in the GTCS-frequency of more than 50%. The positive result was observed for 28 months on the average. 27% of all patients became free of GTCS for 18 months on the average (range, 44 days to 62 months), and 32% were improved (reduction in GTCS-frequency of more than 50% while not GTCS-free) for nearly three years on the average (range, 79 days to 110 months). Compared to PHT or PB, BR were slightly more efficient and similarly tolerable, but no statistically significant difference was seen (48% responders in the control group versus 58% responders in the BR-group). Thus, the statistical correlation with competitive therapies confirmed the good efficacy of BR against GTCS, which was previously demonstrated by clinical tests without control studies. We recommended BR in the case of every pharmaco-resistant epilepsy with GTCS as a therapeutic alternative.

Adolescent↗

PubMed2002

The use of 2-deoxy-2-[18F]fluoro-D-glucose (FDG-PET) positron emission tomography in the routine diagnosis of epilepsy.

PURPOSE: Positron emission tomography with 2-deoxy fluoroglucose positron emission tomography (18-FDG-PET) is widely used in the pre-surgical evaluation of subjects with epilepsy, but little is known of its usefulness in a non-surgical population. PROCEDURES: We analyzed the sensitivity of PET as a diagnostic tool in a large unselected population of epilepsy subjects. Pre-surgical and non-surgical portions of this population were individually assessed as well. The relationship of PET abnormalities to other neurodiagnostic tests was examined. Statistical assessment relied primarily on contingency tables (chi-square tests), with ANOVA or non-parametric assessment used as necessary. RESULTS: While PET was more likely to identify areas of decreased metabolism in the surgical population than in the non-surgical populations, it nevertheless found a significant number of abnormalities in the total population and in the non-surgical group alone. Even in groups in which the clinical diagnosis was unknown, abnormalities were found 40% of the time. PET was useful as an exclusionary diagnostic tool for non-epileptic seizures (NES) and primary generalized epilepsies (PGE) with sensitivity, specificity, and accuracy > 90%. The PET was somewhat more sensitive than magnetic resonance imaging (MRI) in finding abnormalities in the total population, but was less sensitive than electroencephalography (EEG). CONCLUSION: PET may be a useful diagnostic tool in the general epilepsy population even when a definitive clinical diagnosis is not suggested by other modalities.

Journal Article↗

PubMed2006

Excitability of the human epileptic cortex after chronic valproate: a reappraisal.

We explored the action of chronic valproic acid (VPA) on the human epileptic cortex by means of transcranial magnetic stimulation (TMS). TMS is an emerging biomarker for neurotropic drugs. We had 15 drug-naive patients with different epileptic syndromes. Interictally, we measured several TMS indexes of cortical excitability before commencing VPA and 3 months later. At that time, all patients were clinical responders to the drug, whose plasma levels were in the "therapeutic range". We then compared the two conditions, while 18 healthy subjects, of whom 12 were retested at a similar delay, acted as controls. In the pooled patients, the baseline resting motor threshold to TMS was similar to that of controls, but it increased significantly (P < 0.05) after VPA. Intracortical facilitation, another index of cortical excitability, was abnormally enhanced at baseline but decreased significantly after VPA (P < 0.05). On splitting patients according to their diagnosis, the threshold increase was significant (P < 0.05) among partial, but not generalized epilepsies. The reverse was true for changes in intracortical facilitation. TMS phenomena had no linear relation to VPA serum levels. Based on the known pharmacology of TMS effects, VPA reduced the intrinsic membrane excitability of motor cortical neurons, possibly through changes in Na+ channel activity. Then, VPA corrected a transmitter-mediated interneuronal hyper-excitability of the primary motor cortex. The former effect was best seen in partial, and the latter in generalized epilepsy patients.

Adolescent↗

PubMed2000

Fenfluramine-Phentermine (Fen-Phen) and Seizures: Evidence for an Association.

Fenfluramine-phentermine combination therapy ("fen-phen") became a popular treatment for obesity in the 1990s. Although this treatment causes cardiac toxicity, use of these medications has not previously been associated with seizures. We report five cases with apparent association between use of fenfluramine-phentermine and occurrence of seizures. Three patients with a history of childhood-onset idiopathic generalized epilepsy in remission experienced a recrudescence of seizures following treatment with fenfluramine-phentermine. Two patients presented with new-onset seizures in midlife following use of fenfluramine-phentermine, and seizures persisted following discontinuation of this therapy. One of these patients restarted fenfluramine-phentermine months later, and experienced recurrent seizures. The nature of the association between fenfluramine-phentermine and seizures is uncertain from this preliminary report. There may be a specific association with idiopathic generalized epilepsies, which appeared to be overrepresented in this case series. An effect of fen-phen on seizure threshold appears most likely; however, an epileptogenic effect cannot be excluded.

Journal Article↗

PubMed1985

Alterations in transient visual-evoked potentials induced by clonazepam and sodium valproate.

The transient visual-evoked potentials (VEP), recorded in patients suffering from generalized epilepsy, can be characterized by a duplication of the negative peak with a latency of 70-100 ms, a strong increase in amplitude of the negative peak between 80 and 130 ms and the development of a high amplitude slow negative wave following the primary complex. In this study we have investigated to which degree these changes are influenced by the intravenous administration of clonazepam and sodium valproate. Therefore, VEPs were registered in 30 patients, suffering from generalized epilepsy, just before and immediately after the intravenous injection of 0.4-0.8 mg clonazepam and further at 15, 30 and 16 min thereafter. The same procedure was applied on 10 patients treated with an intravenous injection of 400 mg sodium valproate. In both groups we found more or less a reduction in number and degree of the mentioned VEP changes. The improvement was associated with a strong decrease in amplitude of the VEP components in the group treated with clonazepam but was not seen in the sodium valproate group.

Adolescent↗

PubMed2005

Levetiracetam in pediatrics.

Levetiracetam is a new antiepileptic drug whose efficacy and tolerability are already well known in adults. Few studies are available in children. This review, based on the international literature, aims to identify and make known the possible indications for levetiracetam in childhood. Most studies suggest that levetiracetam is effective against partial and generalized epilepsy. In resistant partial epilepsy, the percentage of responders reaches 64%, with 8 to 23% seizure free. Levetiracetam is used to treat symptomatic and idiopathic epilepsies. The drug has also proven effective against photosensitivity and epileptic and nonepileptic myoclonus. The most frequent side effects involve the behavioral sphere and manifest mostly in patients with a history of behavioral problems. In some patients, levetiracetam increases the number of seizures, but this adverse reaction can be partially avoided with slow titration. Doses for children should be 130 to 140% of those advised for adults. Levetiracetam seems to have a broad spectrum of action and is, on the whole, well tolerated. Its efficacy against generalized epilepsy is particularly promising in childhood.

Adult↗

PubMed1997

Menstrual cycle and ovary alterations in women with epilepsy on antiepileptic therapy.

Impaired reproductive function is thought to frequently affect women with epilepsy, mainly when seizures originate in the temporal lobe. In this study, we evaluated menstrual cycle features and assessed ovulation by determining luteal progesterone (Pg) levels in 101 consecutive women with epilepsy (36 with idiopathic generalized epilepsy -IGE; 65 with partial epilepsy -PE), aged between 16 and 50 years, treated with various antiepileptic drugs (AED). PE originated in the temporal lobe (TLE) in 40 subjects, in the frontal lobe in 13, in the parietal lobe in 2, while the origin of focal seizures remained undetermined in 10 patients. In all patients, menstrual and reproductive history, body mass index, hair distribution and hormonal pattern were assessed. Suprapubic ovary ultrasound (US) examination was carried out in 83 patients (28 with IGE, 55 with PE). Three patients with IGE and one with PE were amenorrheic. Oligomenorrhea occurred in 16 patients, polymenorrhea in 2. Changes in menstrual cyclicity were independent from epilepsy type (19.4% in IGE; 23.1% in PE) and from origin of focal discharges (22.5% of patients with TLE; 20.0% with origin in other brain areas). Luteal Pg levels remained below 2 ng/ml in 30 patients independently of epilepsy type. Corpus luteum dysfunction was combined with hyperandrogenism in 15 of these patients. In the other cases different alterations of hypothalamus-pituitary-ovary axis were observed. Valproic acid blunted luteal Pg surge more frequently than other AED. Polycystic ovaries (PCO) were observed in 14 (16.9%) patients (21.0% with IGE: 14.5% with PE). These prevalences are not higher than those reported in the general population. Among PE patients, PCO was found in 1 case with undetermined focal origin and in 7 TLE cases, who also had ovary volume significantly larger than patients with seizures originating from the frontal or parietal lobe. Epileptic women exhibited an increased occurrence of multifollicular ovaries (MFO) found in 12 cases (14.4% vs 5% in the general population). However, no defined hormonal or clinical pictures were associated with this US alteration in most patients. These findings reappraise the impact of ovary alterations in women mainly affected by mild to moderate epilepsy, on differing AED regimens, with the exception of more frequent ovulatory dysfunction and PCO occurrence in patients taking VPA.

Adolescent↗

PubMed1983

[Early diagnosis of Lafora disease. Significance of paroxysmal visual manifestations and contribution of skin biopsy].

The early diagnosis of Lafora's disease is often difficult, from primary generalized epilepsy and other progressive myoclonic epilepsies. The authors emphasize the diagnostic interest of paroxysmal visual manifestations, reported in only 25 per cent of the cases in the literature, and present in 17 of their 31 personal cases. These visual manifestations can be elementary or complex. The elementary ones appear often early, before other symptoms of the disease. Two demonstrative cases are reported. In one of them an occipital seizure was recorded, demonstrating the epileptic critical nature of these elementary visual manifestations. They have a high diagnostic value since they do not occur neither in primary generalized epilepsy (Grand Mal type), nor in the other progressive myoclonic epilepsies. In the 2 reported cases, skin biopsy, as proposed by Carpenter and Karpati, demonstrated the specific storage in the sweat ducts and glands. The interest of this skin biopsy is underlined, its positivity seeming both constant and specific in Lafora's disease.

Adolescent↗

PubMed1996

[A clinico-genealogical study of a family with juvenile myoclonic epilepsy].

Juvenile myoclonic epilepsy (JME) is idiopathic generalized epilepsy of teenagers characterized by massive myoclonic fits. It is supposed to be of hereditary nature with location of the gene on the short arm of the 6th chromosome. A family is described where two sibs suffered from JME attacks while their uncle had isolated generalized convulsive seizures. The autosomal recessive pattern of JME inheritance was suggested. JME gene may be linked to the genes which determine photosensitivity and fast generalized spike or polyspike EEG activity. High frequency of neuroendocrine disturbances in JME patients is emphasized.

Adolescent↗

PubMed1991

Crossover phenomena in epileptic syndromes of childhood.

Benign Rolandic epilepsy (BRE) and primary generalized epilepsy (PGE) are found at about the same period of childhood. Crossover from PGE to BRE and vice versa may occur; two short case reports provide further evidence for this development. Hence, transitions from a primarily generalized to a focal (partial) epileptic seizure disorder and vice versa is a possibility. Both PGE and BRE are based upon states of hyperexcitability, i.e. dysfunctional rather than structural abnormalities that are capable of changing their spatial distribution.

Child↗

PubMed2003

The lack of association between febrile convulsions and polymorphisms in SCN1A.

Febrile convulsions (FCs) represent the majority of childhood seizures, and patients have a genetic predisposition to their development. The genetic susceptibility to FCs seems to involve multiple genes in most instances. Recent studies provided evidence that mutations in SCN1A represent the most frequent cause of generalized epilepsy with febrile seizures plus an autosomal-dominant epilepsy syndrome. SCN1A mutations alter channel inactivation, resulting in persistent inward sodium current. It is not known if polymorphisms in those genes involved in familial epilepsies also contribute to the pathogenesis of FCs. By performing an association study, we used single nucleotide polymorphisms to investigate the distribution of genotypes of SCN1A in patients with FCs. A total of 104 Taiwanese children with FCs and 83 normal control subjects were included in the study. Polymerase chain reaction was used to identify the A/G polymorphism of the SCN1A gene. The results showed that genotypes and allelic frequencies for the SCN1A gene polymorphisms in both groups were not significantly different. These data suggest that the SCN1A gene might not be one of the susceptibility factors for FCs. Pure FCs and febrile convulsions associated with idiopathic generalized epilepsy may not share a common genetic etiology.

Alleles↗

PubMed2001

Polymorphism analysis of JRK/JH8, the human homologue of mouse jerky, and description of a rare mutation in a case of CAE evolving to JME.

Disruption of the function of the mouse jerky gene by transgene insertion causes generalized recurrent seizures reminiscent of human idiopathic generalized epilepsy (IGE). A human homologue, JRK/JH8, has been cloned, which maps to 8q24, a chromosomal region associated with several forms of IGE. JRK/JH8 is, therefore, a candidate locus for at least some forms of IGE. We report corrected cDNA sequences and extended open reading frames for the mouse jerky and human JRK/JH8 genes, which add 48 amino acids to the N-terminus of the Jerky protein and which extends the region of homology with the N-terminal DNA-binding domain of the centromere-binding protein, CENP-B. Systematic sequencing of the coding region of the extended JRK/JH8 gene identified single nucleotide polymorphisms that define three haplotypes, which were used for association studies in patients with idiopathic generalized epilepsy. We report one subject with childhood absence epilepsy (CAE) that evolved to juvenile myoclonic epilepsy (JME) that has a unique de novo mutation that results in a non-conservative amino acid change at a potential protein glycosylation site. Familial analysis supports a causal role for this mutation in the disease.

Alleles↗