[Juvenile myoclonic epilepsy].
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Relationship between amplitude and interval of healthy and epileptic children's alpha activity was investigated. A directly proportional, though non-linear (mostly exponential) correlation was found, i. e. the higher the waves, the wider their are, and vice versa, the lower, the narrower. This is a well-known feature of clinical electroencephalography, representing a good organization of EEG elements and, in terms of physiology, suggesting good "co-operation" of the recruitment process excitation and inhibition. Low interval values are matched by equally low amplitude values while inhibition is adequate. At higher interval values, amplitude is seen rising rapidly, inhibition is inadequate resulting in "paroxysmal" alpha activity which is occasionally found assuming the nature of spike and slow wave activity. The appearance of such principal changes in the recruitment process is abrupt, but a certain part of the changes often fails to manifest itself in the EEG curve. Epileptic children's alpha activity has a relatively higher amplitude, slower rhythm and a more regular regulation of amplitudes and intervals as well as that of their ratio. This is in line with the tendency toward hypersynchrony of EEG elements in epileptics. Epileptic spike and wave activity has an opposite sense of regulation as to the relationship of spike amplitudes and intervals, the principle being: the higher the spikes, the shorter the intervals between them. Apart from frequency, amplitude and angular velocity, that is another substantial difference between alpha activity physiological synchronization in vigilance and pathological spike hypersynchronization in petit mal seizure. Changes in vigilance seem to be in agreement with this phenomenon. The nature of recruitment process regulation (given in type of function) appears to be very stable, often remaining unchanged even under pathological conditions. Thus e. g. aplha wave recruitment during vigilance and spike recruitment during seizure are linear in either case. But while in alpha activity the amplitude-interval ratio is directly proportional, in spikes the proportionality is inverse. Alpha activity appears to be accompanied by inhibitory processes, spikes by excitatory processes. The number of active neurones and their synchronization in the course of spike-wave episodes is higher than during alpha activity. For that reason, in the course of epileptic recruitment process, expedient temporal-spatial integration of neuronal activity decreases to be superceded by simplified hypersynchronous reverberation with lower analytical-synthetic ability and lower reflectivity.
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The clinical trial of Dipropylacetat (Convulex) demonstrates the advantages of a well organized multicenter-study. In rather short time reliable information concerning indication, compatibility and dosage can be received. The results of this project are similar to other observations dealing with DPA: Good therapeutical effect in primary generalized epilepsies independent of the seizure-type. Astonishing results influencing Lennox-Syndrome. In cases not treated before, Convulex shows at least the same effect than the "standard anticonvulsants" but less side effects, especially sedation. In those untreated "new-comers" therapeutical effect comes earlier than in cases of premedication. 20 to 50 mg/kg/day can be recommended in childhood. In rare cases the dosage was increased up to 120 mg/kg/day without problems.
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Between 3700 and 3900 children are admitted annually to this children's hospital. During the past four years a total of 455 children with convulsions were admitted for investigation. 255 of these cases were calcified as epilepsy, 202 as febrile convulsions and 28 as neonatal convulsions. A quarter of the epileptic children showed fits of the petit mal type. There were only 5 cases of infantile propulsive petit mal. High frequency of fits and, in particular, status epilepticus, were very rarely seen in the present study. A case of self-induced photosensitive epilepsy, later combined with psychomotor epilepsy, is referred to in detail. Of 149 children with grand mal epilepsy, 36 were classed as idiopathic, and 88 as symptomatic cases. 37 of the symptomatic cases showed focal epilepsy with generalisation. It was concluded from the clinical course and the EEG that the combined effects of familial predisposition towards fits and exogenous cerebral lesions were operative factors in 14 patients. Children with febrile, convulsions possessed an EEG suggestive of a familial predisposition towards fits in slightly more than 50% of the cases. No cause could be found for the seizures in 5 infants with neonatal convulsions, but the mother of one of these infants was an epileptic herself, undergoing treatment with anticonvulsive drugs in high dosage and a withdrawal syndrome was suspected in this particular case. 17 infants with neonatal convulsions were symptomless on leaving hospital and remained so during the first year of life. Of the remaining cases, 10 showed neurological disturbances and one died. There was only one case in which neonatal convulsions progressed directly to epilepsy. The peak incidence of the first appearance of fits occurred during the first year of life (136 children), with 18% of the entire case material presenting within the first 6 months. 108 children presented with fits for the first time during the second year of life. Thereafter, the tendency towards the development of fits for the first time in life declined with increasing age.
A study was carried out after i.v. administration of 200 mg/Kg of taurine on 37 epileptic patients in whom frequency and duration of seizures was considerable. Twenty-two subjects were given taurine for 15 consecutive days and then, once a week for a period of 6 weeks. In 5 control cases, the daily dosage wascontinued up to the 30th day; in 5 more, up to the 45th day and finally, in 5 up to the 60th day. Out of 37 patients 15 had temporal lobe epilepsy, 10 were cases of Lennox-Gastaut syndrome, 5 had generalised epilepsy, either convulsive or non-convulsive, 4 had H.H.E. syndrome and three brothers had myoclonic familial progressive epilepsy. In each group the results were similar and may be summarised as follows: I) both interictal activity and electroclinical seizures were reduced by about 30% within the first 10 days of administration; II) between the 30th and 45th day the ictal and interictal activity returned to its initial values; III) after this period, on the 60th day, values were similar to the initial ones; IV) as far as interictal activity is concerned, the same effect was found during nocturnal sleep; V) no significant changes were noticed in nocturnal sleep cycles and stages; VI) there was no apparent evidence that any phase of sleep might facilitate any improvement. Improvement was observed in about 50% of the cases (21) both physically and psychologically. The physical improvement was noticed above all in the appearence of the skin while the psychological improvement was mostly related to both attention and memory. Any pathological aspect of personality did not appear to be modified.
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Though epilepsy is recognised as a major medical and social problem in Singapore, there is no epidemiological survey to realise the size of the problem. We studied this problem from data collected from hospitals, school health clinics and from military statistics. The prevalence varied in the three sample populations studied. The most accurate data for prevalence study were obtained in army recruits. A life-time prevalence of 3.8 per 1000 was noted. The prevalence variation with age, sex and races is discussed. The mortality from epilepsy was 0.5 per 100,000. The types of epilepsies and the treatment patterns are discussed.
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