Clinical Neuroscience

[Essential familial myoclonus]

ZONDA Tamás1, SZABÓ Endre1

AUGUST 01, 1969

Clinical Neuroscience - 1969;22(08)

[Authors describe the literature on the pathology of essentialis familiaris myoclonus and the evolution of the concept of the pathology. They share their own observed cases within a family. They discuss diagnostic difficulties and differential diagnosis of the syndrome. They describe the hereditary history of their family. ]

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  1. Balassagyarmati Városi Tanács Kórház Női Ideg- és Elmeosztály

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[Study of the antiepileptic effect of Seduxen (Chronic Diazepam treatment)]

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[The most striking feature of the clinical and EEG effects of Sx is the initial strong antiepileptic effect, followed by a return of seizures as the EEG deteriorates. Similar experiences were reported by Trolle (1965), who, after a pause in the treatment, observed a return to the initial good effect on renewed administration. The findings of our EEG studies suggest not simply a loss of effect due to chronic use, but that after a while the effect of Sx is reversed and now no longer improves but worsens the epileptic mechanism in the direction of the grand mal mechanism (repetitive spikes). The hypersensitivity to the pharmacological denervation induced by the drug is most likely to underlie such a drug effect. However, further studies on this point are needed. In our experience, in the PM. varians epilepsy mechanism, Sx is very effective in preventing spike-wave paroxysms and the corresponding clinical seizures. However, in all cases of repetitive rapid discharges in sleep, it should be used with caution, as the susceptibility to such discharges is increased by Sx. The best clinical effect was obtained in patients with temporal epilepsy resistant to other medications, despite the fact that EEG foci were not completely suppressed by Sx. Probably just as we have seen with Valium, it abolishes generalisational potentials. The effect of Sx on petit mal epilepsy in 2 patients is of course not established, but the initial good effect seen in other epilepsy types was not seen in both patients and the spike-wave mechanism in both cases shifted towards a grand mal mechanism. Compared with foreign Valium, qualitatively quite similar effects were observed in 6 cases. In some patients, the antiepileptic effect of per os Sx was stronger than that of injected Valium, probably because of the more consistent and sustained blood levels. It can therefore be concluded that in drug-resistant severe epileptic patients, Sx given for about 4 weeks has proven to be a very effective antiepileptic adjuvant. We consider it probable - and this would coincide with the hypothesis of the development of denervation hypersensitivity - that by taking drug breaks, the long-term treatment with S adverse effect on the patient will be prevented. Nevertheless, the indications for diazepam are mainly in the treatment of frequent seizures resistant to other drugs, cumulative seizures or status epilepticus, in the form of i.V. shock therapy.]

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[According to Gerebtzoff, using the modified Koelle method, it can be shown in the rabbit CNS that the cell clusters with "external" cholinesterase activity are roughly identical to the termination sites of the cholinesterase-containing systems (ascending cholinergic reticular system, cholinergic limbic system) described in Shute and Lewis in the rat. Some bundles of fibres belonging to these cholinesterase-containing systems can be observed without pathological evidence.]

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[1. The tasks set for the I.I.s can only be achieved if in the coming years local administrations make it possible to have a psychiatrist (in care) for every 40-50 000 inhabitants. Debrecen and Budapest are currently the closest to this, but in most counties (especially those without a mental health bed) it is a major problem to achieve this. To achieve this plan would require about 250 psychiatrists working in the care network. At the beginning of 1968 (including hospital psychiatrists) the number of psychiatrists was 210. Given the need to increase the number of psychiatric beds in the same period, training of specialists is essential. Temporary neurologists working in outpatient clinics, who currently treat neurotic patients for a large part of their practice, could be called in to help. The total number of neurologists at the beginning of 1968 was 292. 2. there is a need to draw up a code of organisation and operation which will enable the psychiatrists working in the I.I. under the present framework to see their tasks clearly and to interpret them uniformly in the care of both the urban and the rural population. 3. To address common professional and other problems in the form of training, methodological letters and other written material. 4. The need to implement close functional cooperation between the I.I.s and hospital departments. 5. Starting from the existing neurosurgeons, prepare for the complex tasks of the future by providing adequate space and specialists.]

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