Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Epilepsy, Absence”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 775 records · Page 43Linked to original sources

Influence of calcium channel inhibitors upon the anticonvulsant efficacy of common antiepileptics against pentylenetetrazol-induced convulsions in mice.

Among three calcium channel inhibitors studied, nifedipine (20 mg/kg) moderately inhibited pentylenetetrazol (115 mg/kg, s.c.)-induced convulsions, whilst diltiazem (up to 20 mg/kg) and verapamil (up to 20 mg/kg) were without effect. The combinations of nifedipine (10 and 20 mg/kg) with valproate (100 mg/kg) or phenobarbital (6.25 mg/kg) resulted in significant protection against pentylenetetrazol-induced seizures. Combined treatment of nifedipine (5-20 mg/kg) with ethosuximide (100 mg/kg) also provided a clearcut anticonvulsant action. The antiepileptic drugs alone, in the above doses, were ineffective. The combination of diltiazem (10-20 mg/kg) and ethosuximide (100 mg/kg) produced protection against pentylenetetrazol, comparable to that of ethosuximide (200 mg/kg) alone. No pharmacokinetic interactions were found in the case of ethosuximide, whilst nifedipine (10 mg/kg) increased the levels of phenobarbital and valproate in plasma. The combination of diltiazem with the remaining antiepileptics were ineffective. Verapamil (up to 20 mg/kg) was without effect upon the action of the antiepileptic drugs tested. Finally, none of the calcium channel inhibitors studied influenced the action of diazepam (0.2 mg/kg). It may be concluded that combinations of ethosuximide, with either nifedipine or diltiazem, may be promising for the treatment of absence epilepsy.

Animals↗

Verbal and visual memory impairment in children with epilepsy.

Verbal and visual memory performances were evaluated in 60 epileptic children and 60 normal control subjects with Signoret's Memory Battery scale. Eighteen patients had idiopathic generalized epilepsy and 42 had partial epilepsy, mostly of the temporal (n = 28) and frontal (n = 10) lobes. Memory scores were statistically lower in epileptics than in controls and significant differences were found within each group: (1) children with idiopathic generalized epilepsy had a slight depression of visual memory; (2) memory disorder was more severe in partial epilepsy; and (3) children with left and right temporal lobe epilepsy had marked memory deficits related to hemispheric specialization.

Adolescent↗

Effects of diazepam and two beta-carbolines on epileptic activity and on EEG and behavior in rats with absence seizures.

In the present series of experiments, effects of a full benzodiazepine receptor agonist (diazepam) are described and compared with those of a partial benzodiazepine receptor agonist (ZK 91296) and an inverse partial benzodiazepine receptor agonist (FG 7142), both compounds of the beta-carboline family. In a rat model for generalized absence epilepsy, the anticonvulsant, the hypnotic and the myorelaxant properties were investigated, as well as effects on on-going behavior and effects on the electroencephalogram (EEG). While diazepam showed all behavioral and electrophysiological changes characteristic for the benzodiazepines, the partial agonist ZK 91296 reduced seizure activity without inducing any signs of sedation, sleepiness, myorelaxation and changes in behavior or EEG spectral content. The partial inverse agonist FG 7142 aggrevated epileptic activity, with slightly enhanced immobile behavior, suggesting some anxiogenic properties. The results not only demonstrate that the multiple effects of the benzodiazepines could be separated by these compounds, but also that the anticonvulsant activity is not related to changes in spectral content of the EEG. Because of its selective activity, ZK 91296 appears to be more suitable than diazepam in reducing seizure activity. Finally, FG 7142 seems a genuine partial inverse agonist which has some, but not all, of the inverse effects of a full agonist.

Animals↗

Effects of the beta-carboline abecarnil on epileptic activity, EEG, sleep and behavior of rats.

The profile of the anxiolytic beta-carboline isopropyl 6-benzyloxy-4-methoxymethyl beta-carboline-3-carboxylate (abecarnil; ZK 112 119), a partial agonist at benzodiazepine receptors, was determined in two experiments. In the first, abecarnil was given to WAG/Rij rats; these rats generate spontaneously occurring spike-wave discharges and are regarded as a model for absence epilepsy. Effects were measured on epileptic activity, together with those on the spectral content of the background electroencephalograph (EEG), as well as on ongoing behavior. In a second experiment, effects on sleep and behavior were investigated in Wistar rats. It was found that, similarly to classical benzodiazepines, abecarnil possessed a strong antiepileptic character and also changed the background EEG to more high-frequency waves and less spindle activity. It also produced more immobile behavior. Abecarnil induced only small, marginally significant increases in slow-wave sleep while reducing REM sleep as a proportion of total sleep. It also reduced the number of REM periods. These observations are consistent with the proposed partial agonist activity of abecarnil, a drug with interesting therapeutic implications.

Animals↗

Reduction of epileptic seizures through EEG biofeedback training.

Biofeedback training of the sensorimotor rhythm (SMR) was carried out in three male and three female adolescent epileptics and in two normal controls. The patients represented a cross-section of epilepsies including grand mal, myoclonic, afocal and psychomotor types. Three of the cases were mentally retarded. 12-14 Hz (SMR) activity was detected by a combination of sharp analog filtering and digital processing. The patients were provided with feedback whenever they produced 0.5 sec of 12-14 Hz activity of a specified amplitude. Additional feedback was provided for epileptiform activity slow waves or movement. Furthermore, feedback for SMR production was inhibited by digital logic circuitry when movement, slow waves or spikes were present. Seizure reduction was obtained in five of the six epileptics. Several patients showed increased percentage of SMR when feedback was provided and varying degrees of normalization in their EEG as demonstrated by fast Fourier, crossed power spectral density and coherence analyses.

Adolescent↗

Intrathalamic injections of gamma-hydroxybutyric acid increase genetic absence seizures in rats.

The effects of intrathalamic injections of gamma-hydroxybutyric acid (GHB) and of NCS 382 85, a specific antagonist of GHB receptors, were evaluated in rats with spontaneous generalized absence epilepsy. Bilateral injections of GHB (25 and 50 micrograms/side) into the mediolateral thalamus increased spontaneous spike and wave discharges (SWD) in a dose-dependent fashion. This effect was suppressed by administration of NCS 382 85 (50 micrograms/side). Bilateral injection of NCS 382 85 alone (50 and 100 micrograms/side) into the same sites had no effect on SWD duration. Injection of GHB or NCS 382 85 into the midline thalamus and the area of reticular nuclei did not modify the SWD. These data suggest that GHB receptors in the mediolateral thalamus may be involved in the control of spontaneous SWD in this rat model of petit mal epilepsy.

Animals↗

Increases in the power spectral slope of background electroencephalogram just prior to asymmetric spike and wave complexes in epileptic patients.

Moment-to-moment electroencephalogram (EEG) changes just before spike and wave complexes (SWCs) were investigated by using a non-stationary power spectral analysis in 10 epileptic patients with asymmetric SWCs. The instantaneous power spectra of background EEG were estimated for a 10-s period just before SWCs in 10 locations. The spectral shape, showing a l/f amplitude characteristic, was assessed by the negative slope (spectral slope) of a straight line on a plot of log power versus frequency. Spectral slope significantly increased toward SWCs at locations with greater SWCs. This finding suggested structural changes in EEG frequency composition just before SWCs because of an epileptogenic process.

Adolescent↗

Gamma-hydroxybutyrate promotes oscillatory activity of rat and cat thalamocortical neurons by a tonic GABAB, receptor-mediated hyperpolarization.

The actions of gamma-hydroxybutyrate, a drug known to lead to an increase in nocturnal slow wave sleep and induce epileptic states following systemic application, on the membrane properties of thalamocortical neurons from brain slices of the rat and cat dorsal lateral geniculate nucleus were studied using sharp electrode intracellular recordings. Gamma-hydroxybutyrate applied by addition to the perfusion medium led to a concentration-dependent and reversible hyperpolarization of the membrane potential accompanied by a decrease in apparent input resistance (0.1 mM: 2.3 +/- 0.3 mV, 9.5 +/- 1.0%; 10 mM: 11.3 +/- 1.3 mV, 37.5 +/- 10.8%, respectively). In six of seven neurons the iontophoretic or bath (0.1-0.2 mM) application of low concentrations of gamma-hydroxybutyrate led to a hyperpolarization accompanied by the appearance of low-frequency (< 4 Hz) membrane potential oscillations crowned by bursts of action potentials, when the membrane potential of these neurons was initially set depolarized to the range where ongoing oscillatory activity occurred. The gamma-hydroxybutyrate-elicited hyperpolarization was reversibly antagonized by the co-application of the GABAB receptor antagonist CGP 35348 (0.4-1 mM), but was not affected by the putative gamma-hydroxybutyrate receptor antagonist NCS 382 (0.1-5 mM) or tetrodotoxin (1 microM), suggesting that gamma-hydroxybutyrate tonically activates postsynaptic GABAB receptors. The gamma-hydroxybutyrate-induced promotion of oscillatory activity and action potential burst firing of thalamocortical neurons may be one mechanism by which gamma-hydroxybutyrate leads to an increase in the deep stages of sleep and the generation of electroencephalogram and behavioural patterns typical of absence epilepsy.

Action Potentials↗

Glycine potentiation of anticonvulsant drugs in pentylenetetrazol seizures in rats.

This study evaluates the glycine potentiation of anticonvulsant drugs in subcutaneous pentylenetetrazol seizures in rats. Administered alone, glycine (30 or 40 mM/kg, PO) induced no anticonvulsant effect or neurological deficit. Coadministered with anticonvulsants, glycine significantly enhanced the anticonvulsant potency of diazepam and sodium valproate without affecting the neurological deficit induced by the anticonvulsants. Glycine did not significantly alter the anticonvulsant activity of ethosuximide or phenobarbital. These findings indicate a possible glycine-sensitive component in the mechanism of action of diazepam and sodium divalproate in subcutaneous pentylenetetrazol seizures. With the possible exception of sodium valproate, the present study provides little support for a glycine and gamma-aminobutyric acid (GABA) interaction as a mechanism of anticonvulsant activity in SC PTZ seizures. Further studies are required to determine the role of strychnine-sensitive and strychnine-insensitive glycine receptors in this experimental model of absence epilepsy.

Animals↗

Effects of the GABAB antagonist CGP 35348 on sleep-wake states, behaviour, and spike-wave discharges in old rats.

The GABAB antagonist CGP 35348 was intraperitoneally given in doses of 100, 300, and 900 mg/kg to old rats. These rats were earlier chronically provided with EEG and EMG electrodes. Sleep recordings based on visual inspection of EEG and EMG recordings were made for 3 h post injection, and spontaneous behaviour in the recording cage was additionally observed. With 100 and 300 mg/kg, the drug produced an increase in the duration of REM sleep compared to the saline-injected control group. The REM sleep latency was correspondingly reduced. Non-REM sleep and total sleep duration increased and an s-shaped dose-response relationship was found. Explorative behaviour was diminished after injections with 100 and 300 mg/kg CGP 35348. The number and duration of spike-wave discharges were reduced after all doses of CGP 35348 and during all 3 recording hours. The latter outcomes confirm the strong suppressive action of this drug on spike-wave discharges; these effects have also been reported in models of absence epilepsy. The hypnotic properties and especially the increase in REM sleep after the administration of CGP 35348 deserve attention considering the paucity of drugs which facilitate REM sleep. The discovery of drugs promoting REM sleep might have theoretical as well as clinical consequences.

Aging↗

Phenobarbital-induced Tourette-like symptoms.

A 7-year-old child with spastic cerebral palsy and a seizure disorder developed Tourette-like symptoms during therapy with phenobarbital. These symptoms occurred while phenobarbital blood levels were in the therapeutic range. Known causes of movement disorders were eliminated after evaluation. On repeat challenge with phenobarbital, the symptoms recurred. Phenobarbital should be added to the list of drugs that cause Tourette-like symptoms.

Cerebral Palsy↗

Cerebral glucose metabolism in five patients with Lennox-Gastaut syndrome.

Regional cerebral glucose metabolic rates were estimated by positron emission tomography, in parallel with electroencephalography and cranial computed tomography in 5 patients with Lennox-Gastaut syndrome. The 5 patients, 3 boys and 2 girls, ranged in age from 10-15 years. Computed tomography revealed no gross abnormalities. Each patient received 2-5 mCi of 2-(18F)-fluoro-2-deoxy-D-glucose (18F-FDG) intravenously. Averaged cerebral glucose metabolic rates were reduced in each cerebral region as compared with controls. Unilateral hypometabolism was present in 4 patients: one in the inferior frontal gyrus as well as the posterior portion of the superior temporal gyrus; one in the inferior frontal gyrus; one in the posterior portion of the superior temporal gyrus; and one demonstrated diffuse hemispheric hypometabolism including the inferior frontal and posterior portion of the superior temporal gyrus. The side of hypometabolism was the same as the epileptogenic focus on the electroencephalogram. No focal changes were demonstrated on the electroencephalogram of a patient whose positron emission tomography revealed hemispheric hypometabolism. Hypometabolism of the inferior frontal and posterior portion of the superior temporal gyrus may relate to the possible pathogenesis of Lennox-Gastaut syndrome. Positron emission tomography has the potential to reveal a latent focal or lateralized abnormality in some patients with non-localized electroencephalographic changes.

Blood Glucose↗

Lenńox-Gastaut syndrome associated with leukoencephalopathy.

We report a 13-year-old boy with Lennox-Gastaut syndrome (LGS) associated with leukoencephalopathy. He was diagnosed with unclassified acute lymphocytic leukemia at the age of 3 years. After initial chemotherapy, he received intravenous methotrexate (total dosage 1,035 mg), intrathecal methotrexate (total dosage 221 mg), and whole brain irradiation (2,400 cGy). From about the age of 8 years, he developed slurred speech, hyperactivity, and mental deterioration. Cranial CT revealed calcification of the subcortical white matter. At age 9 years, he exhibited tonic seizures and atonic seizures. EEG showed diffuse slow spike-wave discharges, which are characteristic of LGS. Although multiple antiepileptic drugs have been prescribed, the frequency of seizures remains unchanged and his mental state is becoming progressively worse.

Adolescent↗

Effects of the GABA-uptake inhibitor tiagabine on electroencephalogram, spike-wave discharges and behaviour of rats.

Effects of the anticonvulsant tiagabine in doses of 1, 3 and 10 mg/kg were investigated on electroencephalogram (EEG), spike-wave discharges and behaviour of WAG/Rij rats. These rats are considered as an animal model of generalized, non-convulsive, absence epilepsy. WAG/Rij rats spontaneously show a considerable number of spike-wave discharges in their EEG. These discharges can be facilitated by GABA agonists. The facilitatory effects of these agonists are completely opposite to their effects on convulsive seizures, which are reduced by these drugs. Tiagabine enhances the effects on the GABA system, since it acts as a GABA re-uptake inhibitor. According to expectations, tiagabine enhanced in a dose-related way both the number and mean duration of spike-wave discharges. The low dose of 1 mg/kg had almost no effects, but doses of 3 and 10 mg/kg were effective. Furthermore, tiagabine in the latter two doses increased the power in the higher beta band of the background EEG, whereas no significant changes in behavioural parameters were found. An unexpected finding was the occurrence of a second type of spike-wave discharges. These were again seen with the two higher doses of tiagabine, while 1 mg/kg had no effect. An assumption is that this second type of discharges are forerunners of genuine spike-wave discharges. In general, this experiment supports that non-convulsive epilepsy is associated with a GABA hyperfunction. It also underlines the biochemical differences of convulsive and non-convulsive animal models of epilepsy. Tiagabine, with its GABA-mimetic properties, belongs to the category of drugs effective in convulsive animal models and not in non-convulsive models of epilepsy.

Action Potentials↗

Anticonvulsant drug effects on spontaneous thalamocortical rhythms in vitro: valproic acid, clonazepam, and alpha-methyl-alpha-phenylsuccinimide.

Spontaneous thalamocortical epileptiform activity was elicited in rodent thalamocortical slices by a medium containing no added Mg2+. Multiple varieties of activity were generated in these slices, including simple thalamocortical burst complex (sTBC) activity that resembled the spike-wave discharges of generalized absence epilepsy, and complex thalamocortical burst complex (cTBC) activity that resembled generalized tonic-clonic seizure discharges. In a further pharmacological characterization of this activity, the effects of the broad-spectrum anticonvulsants valproic acid, alpha-methyl-alpha-phenylsuccinimide (the active metabolite of methsuximide) and clonazepam were studied. All three drugs were found to be effective in controlling both sTBC and cTBC activity when applied in clinically relevant concentration ranges. The effectiveness of valproic acid against spontaneous rhythms in vitro was not due to augmentation of GABAergic inhibition. No effect of valproic acid on GABA-activated chloride currents was evident in patch-clamp recordings of acutely isolated thalamic or cortical neurons. The equivalent general clinical and experimental spectrum of action of broadly effective anticonvulsants provided an additional correlation between the clinical efficacy of anticonvulsant drugs and their effects against epileptiform discharges in rodent thalamocortical slices. This further validates spontaneous generalized low-Mg2+ thalamocortical activity as a potentially valuable in vitro model of the primary generalized epilepsies, in which the cellular mechanisms underlying generation and control of these seizure discharges can be studied.

Action Potentials↗

Juvenile myoclonic epilepsy of Janz: clinical observations in 60 patients.

We studied 60 patients with juvenile myoclonic epilepsy (JME). There was a high positive family history for epilepsy (33.3%). Age at onset of epilepsy ranged from 4 to 18 years with an average of 13.9 years. 88.3% of patients were seizure-free. The most effective drug was valproate. In eight patients drug withdrawal was attempted but all patients relapsed during a follow-up period of 1 year. Video-EEG studies were performed in eight newly diagnosed patients; myoclonic jerks were recorded in five patients.

Adolescent↗

Thelarche variant in a girl with Angelman syndrome.

A case of Angelman syndrome (AS) with thelarche variant in a 4.5-year-old girl is presented. Clinical suspicion of AS was raised at the age of 15 months when she presented with mental retardation and epilepsy, absence of speech, ataxic gait with jerky movements, hyperactivity and paroxysmal episodes of laughter. Moreover, she had facial dysmorphic features such as microbrachycephaly, mid-facial hypoplasia, macrostomia and prominent mandible. Dinucleotide repeat polymorphism (DNRP) analysis, identified absence of maternal alleles at D15S543, D15S113 and GABRB3 loci, findings consistent with AS. Studies on CYP19 locus (outside the 15q11-13 region) revealed the presence of two different alleles, thus excluding the possibility of paternal isodisomy of chromosome 15 in this patient. Breast development at the age of 4.5 years, accompanied by accelerated growth velocity and bone age suggested the diagnosis of variant thelarche. This is the second case of AS with sexual precocity reported and whether this combination is a coincidence or not remains to be clarified.

Alleles↗

Stargazin mutation impairs cerebellar synaptogenesis, synaptic maturation and synaptic protein distribution.

Stargazin mutation results in absence epilepsy and cerebellar ataxia in stargazer (stg) mice. We have previously discovered defects of AMPA receptor function, failure of BDNF expression and immature morphology specifically in the cerebellar cortex of stg mice. To further characterize the nature of synaptic abnormalities, we examined the ultrastructure of cerebellar granule cell output synapses and measured the expression levels of several synaptic proteins in different brain regions of stg mutant. Electron microscopic examination revealed a number of immature features in the molecular layer of the mutant cerebellar cortex, including the presence of desmosoid plaques, concentric profiles of parallel fibers, smaller presynaptic terminal and fewer synaptic vesicles. Quantitative measurement showed a significantly lower number of synapses and smaller area of presynaptic terminals in adult stg cerebellum when compared with age-matched wildtype. Immunoblotting analysis of the SNARE proteins revealed selective reduction of the levels of synaptobrevin and synaptophysin in synaptosomes from stg cerebellum. The expression levels of synapsins were not altered in stg cerebellum, but showed a significant upregulation in stg cerebral cortex and hippocampus. Our results suggest that, despite the relatively normal gross morphology of cerebellum, stargazin mutation results in abnormal ultrastructure of cerebellar synapses, and stargazin-induced regional failure of BDNF expression may be responsible for abnormal SNARE protein distribution and partially attributes to the defects in the synaptic ultrastructure.

Animals↗