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PubMed1999

Are there generalised spike waves and typical absences in benign rolandic epilepsy?

Generalised 3 Hz spike wave (SW) discharges with or without absences have been described in children with benign epilepsy with centrotemporal spikes (BECTS), leading to speculations about a continuum between childhood absence epilepsy (CAE) and BECTS. We thus decided to evaluate the prevalence of absence seizures (AS) and generalised 3 Hz SW in patients with BECTS. All patients with BECTS first referred since 1986 have been identified prospectively. Their medical and electroencephalograph (EEG) records were analysed retrospectively, in the search for AS and generalised SW discharges. Over a period of 11 years, we found typical rolandic spikes in 66 newly referred patients; 64 had seizures typical for the condition (18 female, 46 male), two were asymptomatic and were not further analysed. All had routine waking EEG recordings, and 49 children (76%) had at least one sleep EEG. AS or classical generalised 3 Hz SW were never recorded from history or EEG data, respectively. However, 17 patients had some diffuse SW discharges, lasting 1-5 s, which appeared as grossly symmetrical in only seven children, with a clearly asymmetrical aspect in the others. Among these seven patients, the discharges were only seen on awakening in one, both during waking and nREM sleep stage I or II in one and only during nREM sleep stage I or II in five. They were apparently subclinical in all. We thus found neither AS nor classical 3 Hz SW discharges among 64 consecutive patients with BECTS. Brief bursts of bilateral abnormalities occur in about 25% of the cases, mostly with sleepiness. Such findings do not substantiate the existence of a continuum between CAE and BECTS.

Child↗
PubMed1992

Do idiopathic generalized epilepsies share a common susceptibility gene?

Molecular genetic research in epilepsy is most promising in the homogeneous, genetically determined forms of the disease. The phenotype-genotype strategy used makes some epileptic syndromes more suitable for such study than others. Unequivocal clinical presentation, mendelian transmission with similar clinical expression in close relatives, and sufficiently large numbers of affected families are requisites. This makes idiopathic generalized epilepsies (IGEs), particularly absence and juvenile myoclonic epilepsies (JMEs), suitable subtypes for genetic analysis. Results of family studies to date show that five IGE syndromes have a common genetic origin. Linkage studies have localized a gene defect on chromosome 6p that predisposes to a group of IGEs: JME, absence epilepsy, and generalized tonic-clonic seizures in epilepsy families with JME probands. Environmental and additional genetic factors are other areas requiring further study to elucidate the discriminant factors associated with different varieties of IGE.

Chromosomes, Human, Pair 6↗
PubMed1975

Treatment of epileptic seizures with clonazepam. A reappraisal.

The therapeutic efficacy of orally administered clonazepam has been evaluated in 32 epileptic patients, with substantial improvement in 22. The drug is active in all types of seizures, particularly in myoclonus, petit mal absences, and partial complex epilepsy; it seems the drug of choice in generalized infantile organic epilepsy, although the treatment of these patients is still unsatisfactory. In some patients, the drug seemed less effective after months of therapy. Drowsiness is the main side effect.

Administration, Oral↗
PubMed1999

A serotonin-1A receptor agonist and an N-methyl-D-aspartate receptor antagonist oppose each others effects in a genetic rat epilepsy model.

The WAG/RIJ rats exhibit spontaneously occurring spike-wave discharges (SWD) accompanied by behavioural phenomena, with characteristics similar to the human absence type epilepsy. To study the mechanisms involved in this type of epileptiform activity we investigated the effects of the serotonin-1A (5-HT1A) receptor agonist 8-hydroxy-2-(di-n-propylamino)-tetralin (8-OH-DPAT) and the N-methyl-D-aspartate (NMDA) receptor antagonist (+)-5-methyl-10,11-dihydro-5H-dibenzo [a,d]cyclohepten-5,10-imine maleate (MK-801). Intracerebroventricular (i.c.v.) injection of 8-OH-DPAT caused marked, dose dependent increase, MK-801 a decrease in the cumulative duration and number of spike-wave discharges. Pretreatment with MK-801 (10 microg/rat i.c.v.) abolished the increase caused by 8-OH-DPAT (20 microg/rat i.c.v.), but the decrease in SWD to MK-801 was counterbalanced by 8-OH-DPAT. These data provide evidence for an interaction of glutamatergic and serotonergic mechanisms in the triggering and maintenance of epileptic activity in this genetic model of absence epilepsy.

8-Hydroxy-2-(di-n-propylamino)tetralin↗
PubMed1999

Tiagabine-induced absence status in idiopathic generalized epilepsy.

Several medications such as baclofen, amitriptyline and even antiepileptic drugs such as carbamazepine or vigabatrin are known to induce absence status epilepticus in patients with generalized epilepsies. Tiagabine (TGB) is effective in patients with focal epilepsies. However, TGB has also been reported to induce non-convulsive status epilepticus in several patients with focal epilepsies and in one patient with juvenile myoclonic epilepsy. In animal models of generalized epilepsy, TGB induces absence status with 3-5 Hz spike-wave complexes. We describe a 32-year-old patient with absence epilepsy and primary generalized tonic-clonic seizures since 11 years of age, who developed her first absence status epilepticus while treated with 45 mg of TGB daily. Administration of lorazepam and immediate reduction in TGB dosage was followed by complete clinical and electroencephalographic remission. This case demonstrates that TGB can induce typical absence status epilepticus in a patient with primary generalized epilepsy.

Adult↗
PubMed1993

Absence seizures associated with panic attacks initially misdiagnosed as temporal lobe epilepsy: the importance of prolonged EEG monitoring in diagnosis.

While temporal lobe epilepsy is often considered in the differential diagnosis of patients with anxiety or panic disorders, other types of epilepsy can confound the presentation or treatment of adults with panic disorders. The cases of two patients are presented who were initially thought to have temporal lobe epilepsy producing panic attacks, but who were subsequently found to have primary generalized seizures. The clinical implications are discussed.

Adult↗
PubMed2001

[Occurrence of epilepsy, its types and course during the first decade of life in progeny of mothers with epilepsy--prospective study].

On the basis of the prospective study, concerning 100 children of mothers with epilepsy, observed in the first decade, it was established that epilepsy appeared more often in this period than it was reported in the retrospective studies. The frequency of epilepsy amounted 7%. The pregnant-perinatal negative factors in mothers whose children suffered from epilepsy, weren't essentially larger than in other mothers with epilepsy. The occurrence of epilepsy in mothers till 10 year's of age increases the risk of early appearance of epilepsy in offspring essentially (p < 0.05). The epilepsy with absence seizures in mothers is related to the increased number of children with epilepsy in the first decade significantly more often than the epilepsy only with generalized tonic-clonic seizures (p < 0.012). Among the children with epilepsy, there were cases with the same type as in mothers epilepsy (absence), and with other generalized idiopathic epileptic syndromes (West syndrome, Dose syndrome, epilepsy with tonic-clinic seizures). The course of epilepsy in offspring of mothers with epilepsy was typical for the relevant epileptic syndroms appearing in childhood.

Age of Onset↗
PubMed1999

Epilepsy with myoclonic absences with early onset: a follow-up study.

We studied six children (four girls and two boys) suffering from cryptogenic myoclonic absence seizures with early onset. The age at onset of the seizures ranged between 6 and 27.8 months (mean age +/- SD: 18.5+/-12.4 months). The neurologic evaluation was normal in all patients at the first hospital admission. After the diagnosis, we followed up all children for at least 5 years. At the end of follow-up, two of these patients (a girl and a boy) showed severe mental retardation, a high number (from one to three per day) of seizures, and persistent pathologic electroencephalograms. The other patients showed normal electroencephalograms: all of them were seizure free and without mental retardation. The two patients with mental retardation have been treated with polytherapy. In all other children we used valproate alone successfully. Our data suggest that myoclonic absence seizures with early onset can have a good long-term prognosis. Valproate is a useful anticonvulsant drug in these patients. Mental retardation is present only in patients with poor seizure control.

Anticonvulsants↗
PubMed1997

Lamotrigine in absence and primary generalized epilepsies.

Although lamotrigine has been approved in the United States as adjunctive therapy for partial seizures in patients older than 12 years, there is increasing evidence that it is just as effective, if not more effective, in the treatment of generalized seizures. A large number of open-label studies and some single-blind data, all using lamotrigine as add-on therapy in patients with previously refractory generalized seizures, are available. Controlled studies, some on newly diagnosed, previously untreated patients with generalized seizures are ongoing. Investigations have demonstrated that patients with the following generalized seizure types improve with lamotrigine add-on therapy: Typical and atypical absence, atonic, generalized tonic-clonic, myoclonic, and clonic seizures. Response rates, defined as the percentage of patients with better than 50% reduction in seizure frequency, have been, depending on seizure type, in the range of 30% to 56%, with 0 to 33% of the patients becoming seizure free. The best responses have been noted in typical and atypical absences, and atonic seizures. Children and adults appear to have comparable responses. In addition, add-on studies in patients with specific, previously refractory, epilepsy syndromes have demonstrated that the best improvement in seizure control occurs in patients with petit mal epilepsy, "other symptomatic" generalized epilepsies, and in Lennox-Gastaut syndrome, followed by patients with other myoclonic epilepsies, myoclonic absence and West syndrome. Many previously refractory patients are able to achieve lamotrigine monotherapy. However, patients with nonprogressive myoclonic epilepsy have little, if any, response. Early data from ambulatory encephalographic (EEG) recordings in patients with previously refractory absence seizures, and from controlled studies on patients with newly diagnosed typical absence seizures, appear to confirm the efficacy of lamotrigine in those patients. Controlled studies are ongoing in patients with absence seizures, in patients with generalized tonic-clonic seizures, and in patients with Lennox-Gastaut syndrome. Dosing in generalized seizures is similar to that for partial seizures. Because of the shorter half-life of lamotrigine in children, as compared to adults, higher (mg/kg) doses are often needed in young patients. We conclude that lamotrigine is a promising drug for absence and primary generalized seizures in both children and adults.

Adolescent↗
PubMed1983

A clinical and epidemiological study of adult patients with epilepsy.

The clinical and epidemiological data of 1054 patients with epilepsy between the ages of 16 and 66 years are described. 70% of the patients were treated in neurological departments, 20% by their general practitioners, while 8% received no treatment. The age-specific prevalence was 430 per 100 000 inhabitants, the same for men and women. Hereditary epilepsies (pyknoleptic absences, juvenile myoclonic epilepsy, epilepsy with awakening grand mal) comprised 16% of all the epilepsies; epilepsies with clinical primary generalized grand mal seizures as the only seizure type 32%, epilepsies with complex partial seizures 25% and epilepsies with simple partial seizures 18%. 8% could not be classified.

Adolescent↗
PubMed2001

Ictal single photon emission computed tomography in absence seizures: apparent implication of different neuronal mechanisms.

Absence seizures represent a complex group of epilepsy, characterized by lapse of consciousness with staring. Bilateral, synchronous, and symmetric bursts of 3-Hz spike-and-wave discharges are observed on the electroencephalogram, whereas interictal background activity is normal. This kind of epilepsy has to be differentiated from other generalized epilepsies such as juvenile absence epilepsy and juvenile myoclonic epilepsy. Moreover, absence seizures, together with generalized spike-and-wave discharges, may coexist with other types of epilepsy such as frontal lobe epilepsy, temporal lobe epilepsy, benign epilepsy with centrotemporal spikes, and childhood epilepsy with occipital paroxysms. We have carried out ictal single photon emission computed tomography (SPECT) in 10 patients with clinical evidence of absence seizures with the aim to better understand and to distinguish this kind of seizure as primarily or secondarily generalized to a specific area and to obtain more information on the neuronal mechanisms involved in the different types of seizures, usually not identifiable at the first appearance. During the long follow-up period (9 months to 14 years), 7 of the 10 examined patients underwent interictal SPECT when they became seizure free. Our data permitted, in two patients, the diagnosis of childhood absence seizures; in three patients, they suggested the possibility of later appearance of other seizure types, on the basis of focal hyperperfusion indicating a possible focal firing. In three of the examined patients, the diagnosis of idiopathic localization-related epilepsies mimicking childhood absence seizures could be performed. In the last two patients, the hypothesis of a coexistence of absences with partial and generalized seizures was considered. From our results, it can be presumed that ictal SPECT findings may contribute to the physiopathologic classification of the different types of epilepsies. Moreover, anticonvulsant treatment more appropriate to the different forms of seizures can be used.

Adolescent↗