Epilepsy in childhood.
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Of thirty-five patients with various types of epilepsy treated with sodium valproate, 15 achieved complete seizure control on that drug alone, 12 other patients benefited and eight failed to improve on the drug. Excellent results were more likely in those with petit mal epilepsy and in those whose epilepsy was controlled with other drugs at the expense of side effects. Three patients were unable to tolerate valproate, but in general few patients experienced side effects and several patients felt much better on valproate than on their previous drugs. A twice daily dosage regime was satisfactory. Plasma valproate levels at the final dose covered a wide range, 0.21 - 1.2mmol/l (34 to 190 microgram/ml) and did not correlate with response, lack of response or side effects.
Epilepsy is a common disorder affecting approximately one in every two hundred people, from all walks of life, and presenting in addition to the seizure disorder itself, varying social, psychological and economic problems. A simple classification of epilepsy is described, and the concept of 'seizure threshold' introduced. Having diagnosed epilepsy, the need for investigation to separate the symptomatic epilepsies from idiopathic epilepsy is stressed. Accurate diagnosis of seizure type ensures that optimal therapy can be offered. The various drugs used to control epilepsy are discussed, and comments on the general management of the patient are offered.
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INTRODUCTION: The ring-shaped chromosome 20 (r20) syndrome is an infrequent chromosopathy which is associated with epileptic seizures, behaviour disorders and mental retardation. It results from the fusion of the two arms of the chromosome with deletion of the telomeric portions. CLINICAL CASE: We present a case of r20, review published cases and describe the clinical and neurophysiological characteristics. CONCLUSIONS: The r20 syndrome is the third type of epilepsy known to be of genetic basis related to chromosome 20. It has clinical and neurophysiological characteristics which give it a distinctive character and are easily identified. The fact that on locus 20q13 (telomeric portion of the long arm of chromosome 20) two genes related to epileptic channelopathies (CHRNA4 and KCNQ2) have been described, suggest the hypothesis that the subjacent deletion in cases of r20 syndrome affect one of these genes and explains the epileptogenicity. We consider this hypothesis and the possibility that r20 syndrome may be an epileptic channelopathy.
The experience of a neurosurgical unit devoted to the surgical therapy of the epilepsies is only based on very peculiar patients. However, relationship between a given epilepsy and associated psychiatric disorders are more easily understood because of more extensive explorations. Bilateral temporal epilepsies involving the limbic system on the one hand, bilateral frontal epilepsies on the other one, and P.M. status which may be paralleled, make these patients more susceptible to acute mental confusions, to acute thymic disorders, to delirious attacks. Direct relations between chronic psychosis and severe epilepsies are much more uncertain.
In the light of three case histories, other personal observations and the literature, the clinical and electroencephalographical differences between absences in the limited sense and continuous LENNOX petit-mal state are described and the problems of the latter discussed. As a rule, petit-mal state is diagnosed as such in young people or adults, and practically never before the 10th year of life. In about two thirds of cases, its clinical symptomatology consists of a twilight condition lasting some hours to a few days, coupled with inertia and apathy. The remaining third of the patients usually experience milder disturbances, e.g. in the form of concentration difficulties, tiredness, and (more rarely) severe forms including lethargy. The EEG correlate of a petit-mal state is made up of continuous bilaterally synchronous, frontally marked (less frequently with exclusively frontal localization), usually irregular spike waves or poly-spike waves, which frequently occur in only rudimentary forms and register a frequency of 2 1/2-4 c/sec. For the treatment of petit-mal state, benzodiazepines and in particular clonazepam (Rivotril) (1-2 mg i.v.) are recommended. During the interval condition the same therapy as with an absence epilepsy, e.g. succinimides or dipropylacetate (Depakine) is administered. Anti-grand-mal remedies, especially hydantoins, may trigger petit-mal status.
INTRODUCTION: The limbic system, is associate to emotional behavior. Partial epileptic seizures are the expression of alterations of the temporal lobe, hippocampus and amygdala. In nonconvulsive episodes, autonomic alterations, visceral dysfunction and abnormal emotional states illustrate epileptic symptomatology. Fear responses, with possible connections to amygdala are presents in partial epileptic seizures. CLINICAL CASE: We present five patients with symptomatology mainly in emotional expression. Four had a structural lesion: one, multiple phacomatosis, two, tumors of the amygdala region (ganglioglioma) and one, a pineal cyst. The fifth child lacking a structural lesion, showed spike waves in the temporal lobe. CONCLUSION: It is essential to search for structural or functional alterations in patients with paroxysmal fear reactions, as probable partial crisis with psychoaffective manifestation.
An outline is given of the incidence and aetiology of childhood convulsions. Aspects of diagnosis and drug therapy are discussed.
Epilepsy is a common disorder affecting approximately one in every two hundred people, from all walks of life, and presenting in addition to the seizure disorder itself, varying social, psychological and economic problems. A simple classification of epilepsy is described, and the concept of 'seizure threshold' introduced. Having diagnosed epilepsy, the need for investigation to separate the symptomatic epilepsies from idiopathic epilepsy is stressed. Accurate diagnosis of seizure type ensures that optimal therapy can be offered. The various drugs used to control epilepsy are discussed, and comments on the general management of the patient are offered.
Typological behavior reactions of WAG/Rij rats were studied from the standpoint of divergent modulatory integration hypothesis. This rat strain has a genetically determined dominant dysfunction of the benzodiazepine system of the thalamic nuclei. This disorder provokes an epileptiform disease such as absence epilepsy. It was suggested that the dysfunction of this system would result in a modification of the modulatory systems, which support the motivation states of escape and avoidance reactions as well as of the modulatory systems, which form the emotional states. Modifications of these states are the background of typological behavioral features of WAG/Rij rats. It was shown that WAG/Rij have the lower threshold of the development of haloperidol catalepsy, higher levels of fear and depression. On the first day of training in a shuttle box, WAG/Rij rats demonstrated better avoidance performance than Wistar rats. On the second and 28th days, the amnestic effect of the epileptiform disease was observed. The amnestic effect was also observed after passive avoidance conditioning. The results are discussed in terms of the modulatory integratin hypothesis.
A short classification and brief clinical description of epileptic seizures are presented. The pharmacokinetic principles underlying modern drug treatment of epileptic seizures are discussed and the drug(s) of choice for different seizure types are listed according to their clinical efficacy. The current view of using single-drug therapy rather than polypharmacy, whenever possible, is emphasized.
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Lethargic mutant mice carry a mutation in the CCHB4 gene, which encodes the beta4 subunit of voltage-regulated calcium channels. These mutants have been shown to display a complex neurobehavioral phenotype that includes EEG discharges suggestive of absence epilepsy, chronic ataxia, and hypoactivity. The current studies demonstrate a fourth element of their phenotype, consisting of transient attacks of severe dyskinetic motor behavior. These attacks can be triggered by specific environmental and chemical influences, particularly those that stimulate locomotor activity. Behavioral and EEG analyses indicate that the attacks do not reflect motor epilepsy, but instead resemble a paroxysmal dyskinesia. The lethargic mutants provide additional evidence that calcium channelopathies can produce paroxysmal dyskinesias and provide a novel model for studying this unusual movement disorder.
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