Status epilepticus occurring in petit mal.
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We studied a patient with idiopathic generalized epilepsy and frequent absences, using electroencephalogram-correlated functional magnetic resonance imaging. Four prolonged runs of generalized spike-wave discharge occurred during a 35-minute experiment. Time-locked activation was observed bilaterally within the thalami in conjunction with widespread but symmetrical cortical deactivation with a frontal maximum. We demonstrate the reciprocal participation of focal thalamic and widespread cortical networks during human absence seizures and suggest reductions in cortical blood flow, in response to synchronized electroencephalogram activity.
Photosensitivity is an abnormal visual sensitivity of the brain in reaction to flickering light sources or patterns and is expressed in the electroencephalogram as generalized spike-and-wave discharge and in more susceptible individuals as clinical seizures. The most common types of seizures are generalized tonic-clonic, followed by myoclonic and absence. The photosensitive epilepsies are classified as pure photosensitive, where seizures occur only with the flickering light source/pattern or during intermittent photic stimulation (IPS) in the laboratory, and epilepsy with photosensitivity, where spontaneous seizures also occur. Positive response to IPS in idiopathic epilepsy syndromes, which are included in the International Classification or are in development, is reported to range from 7.5% in juvenile absence epilepsy to 100% in pure photosensitive epilepsy. The treatment of photosensitivity and pure photosensitive epilepsy with rare seizures includes general and specific protective measures. For most patients, however, combination treatment with antiepileptic drugs is necessary. Valproic acid monotherapy has a success rate of 73-86%. Levetiracetam appears to be a new alternative therapeutic option. Clobazam, lamotrigine, ethosuximide, and topiramate also have been recommended as second-choice therapies.
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The aim of this study was to describe the clinical and neurophysiologic correlates of atypical absence seizures in children with intractable epilepsy. In a retrospective review, 19 children with videoelectroencephalographic monitoring (female n=14; male n=5) fulfilled the electroclinical criteria for this seizure type. Atypical absence seizures occurred in a spectrum of clinical conditions associated with educational disability and intractable seizures. In comparison with children with only atypical absence seizures, children with atypical absence in association with multiple seizure types were more likely to have severe educational disability (n=11 of 13; P = .01), a slower ictal frequency (n=10 of 13; P = .01), and slow background rhythms for age (n = 13 of 13; P = .03). This study illustrates the broad clinical spectrum in which atypical absence seizures are encountered. Differentiation between children with only atypical absence seizures and children with multiple seizure types can be useful with respect to potential academic ability.
Generalized absence seizures in humans are a unique type of epilepsy characterized by a synchronous, bilateral 3-Hz spike and wave discharge emanating from a cortical and thalamic network within the brain. The availability of a number of pharmacological and genetic animal models has provided us with the means with which to investigate the cellular and molecular mechanisms underlying these seizures. Over the last few years a significant amount of research in these models has focused on the role of the inhibitory GABAB receptors, which have been previously described in a number of brain areas as being responsible for a long-lasting hyperpolarization and depression in neurotransmitter release. Initial studies provided evidence that the GABAB receptor was capable of generating the low threshold calcium spike required for initiation of the burst firing, leading researchers to hypothesize that the GABAB receptors played a significant role in these seizures. Subsequent research took advantage of the new generation of GABAB antagonists that became available in the early 1990s and demonstrated that in a number of models the seizures could be abolished by the administration of one of these compounds. Further biochemical, molecular, and electrophysiological experiments have been carried out to determine the exact involvement of GABAB receptors and their mechanism of action. The current evidence and interpretations of this work are presented here.
In a controlled clinical investigation based on ten patients with simple absences and ten patients with myoclonic atonic seizures, all patients who had insufficient response to conventional antiepileptic treatment received clonazepam (Rivotril [Denmark]; Clonopin, comparable US product) combined with previous antiepileptic drugs. The effects of the combined use of clonazepam and the previous antiepileptid drugs were compared with the effects of placebo combined with the same drugs. The trial was single-blind crossover with sequential analysis. In a daily dose of usually 3 to 6 mg, depending on patient age, the antiepileptic effect of clonazepam was significantly superior to placebo and was estimated as remarkably good. Side-effects of somnolence, fatigue, drowsiness, and coordination disturbances occurred in most of the patients, but subsided spontaneously or could be controlled by slow increase or slight reduction of dosage. Mental sideeffects such as agitation, confusion, and aggressiveness were more troublesome and caused discontinuation of clonazepam in two patients.
Sodium valproate (Epilim) has been used in the management of 100 patients with previously uncontrolled epilepsy for periods up to 2 years. If all manifestations of epilepsy are considered together, 75% to 100% control of seizures was achieved in 43% of patients, 25% to 74% control in 26%, and no improvement occurred in 31% of patients. Control of 75% to 100% was achieved in 57% of patients with a spike and wave electroencephalogram (EEG) disturbance but only in 35% of those with focal abnormalities, excessive slow activity, or normal records. When the various manifestations of epilepsy were considered individually, the greatest improvement was found among the patients with the minor forms of generalized epilepsy (petit mal absences, myoclonus and atonic attacks) in whom 75% to 100% control was obtained in 67%, compared with 43% of those with major generalized seizures (grand mal) and 30% of those with temporal lobe attacks and other forms of focal epilepsy. Gastrointestinal disturbances and drowsiness were noted as side effects in the early stages of treatment, but the majority of patients tolerated the drug well and many commented on increased mental alertness while taking it. Two patients were over-stimulated and some noticed tremor or twitching as side effects. Some minor abnormalities in blood coagulation studies were noted, but these were transient and did not appear to be of clinical significance. Regular blood counts and biochemical studies have not shown any significant changes. Sodium valproate appears to be a safe and useful anticonvulsant with the advantage that it usually makes patients brighter rather than drowsier. Abnormalities of platelet function have been described in some overseas reports, so that any unexplained bruising or bleeding in a patient taking valproate is an indication for a platelet count and coagulation studies.
Data from a regional EEG laboratory allowed us to identify almost all children in Nova Scotia (population 85,000) with one or more unprovoked, afebrile seizures from 1977 through 1985. We then reviewed hospital and pediatric neurology physician charts to limit cases to those with two or more definite afebrile seizures between the ages of 1 month and 16 years. In all, 693 children developed epilepsy: typical childhood absence seizures (AS) (97), either generalized tonic-clonic (GTCs) or partial seizures either secondarily generalized or not (511), and other generalized seizure types, including infantile spasms (IS) as well as myoclonic, akinetic, tonic, and atypical AS (85). The incidence of epilepsy was 118 in 100,000 for children aged less than 1 year, 48 in 100,000 for those aged 1-5 years, 43 in 100,000 for those aged 6-10 years, and 21 in 100,000 for those aged 11-15 years. The incidence for each year of age between 1 and 10 years was remarkably constant (mean 46 in 100,000 +/- 7 SD). Comparison of the incidence rates showed significant differences for those aged less than one year as compared with all others, and for those aged greater than 10 years as compared with those aged 1-10 years. We conclude that the incidence of epilepsy is highest in the first year of life, plateaus in early childhood, and decreases markedly after age 10 years. The overall incidence of epilepsy in childhood is lower than that reported in previous studies.