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. ]


  1. Balassagyarmati Városi Tanács Kórház Női Ideg- és Elmeosztály



Further articles in this publication

Clinical Neuroscience

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


[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.]

Clinical Neuroscience

[Mental hygiene (mental health protection) some questions on the organisation of ]


[We can only talk about modern standards in the psychiatric sector of health care for the population if we set our approach and organisation at the level of mental hygiene. This is a major challenge, partly because of a lack of vision, decades of neglect and serious cadre problems, and partly because of the proliferation of problems in this area. In this overview study, we will discuss in general terms the problems, facts and ideas that the mental hygiene doctor working in this field encounters, and more specifically what is being achieved in Csepel under relatively favourable conditions.]

Clinical Neuroscience

[Distribution of "external" cholinesterase activity in the brainstem and telencephalon of the domestic rabbit based on histochemical studies]

PAPP Mátyás

[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.]

Clinical Neuroscience

[The operation of the neurological institutes in 1967 ]

SIMEK Zsófia

[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.]

Clinical Neuroscience

[Electrophysiological changes after local electron irradiation of the brain]


[The somatosensory area of the cat brain was irradiated with electron beams from a Van de Graaf generator. The electroencephalographic changes following irradiation were studied using chronic epidural and deep electrodes. 1. Within the 50-210 Krad radiation range, the higher the radiant intensity applied, the sooner the irritative phase occurred and the more pronounced the activation of the curves. 2. 3. The excitatory stage was followed by a depressive EEG phase lasting one to two days, characterized by intact occipital alpha activity in addition to local permanent flattening. 4. In 210 Krad dozis, the lesions were irreversible. The EEG picture appeared to be normalized for doses of 50-150 Krad, but electrical activity in the deep structures remained reduced throughout. The animals lost sensation and tactile function and none of them regained it during the 2-month survival period. 5. The reduced electrical activity in the ventrobasal structures of the thalamus was associated with retrograde cell degeneration through thalamo-cortical connections.]

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[The role of sleep in the relational memory processes ]

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[A growing body of evidence suggests that sleep plays an essential role in the consolidation of different memory systems, but less is known about the beneficial effect of sleep on relational memory processes and the recognition of emotional facial expressions, however, it is a fundamental cognitive skill in human everyday life. Thus, the study aims to investigate the effect of timing of learning and the role of sleep in relational memory processes. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. Our results suggest that the timing of learning and sleep plays an important role in the stabilizing process of memory representation to resist against forgetting.]

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[Purpose: Establishing a balance between theoretical and practical training is essential in the training of college nursing students. The aim was to assess the students’ experiences of clinical practice and the problems they indicate in the component, which plays an important role in preparing the training for professional competencies. The study sample consisted of 3rd and 4th grade students (N=96) participating in nursing training at the Faculty of Health Sciences of Semmelweis University. The method of data collection was based on the completion of a quantitative online questionnaire, which also included qualitative elements in the form of three open-ended questions. Among the statements measured on the Likert scale, the balance of theory-practice, the role of participants in education, and the time spent on practical tasks received lower average ranks from both grades. Logistic regression is suitable for estimating the probability of a positive/negative impression of a practice as a combined effect of several explanatory variables. Compared to the results of a previous (2009) study, the problems with field clinical training remain unresolved.]

Clinical Neuroscience

Neuroscience highlights: The mirror inside our brain

KRABÓTH Zoltán, KÁLMÁN Bernadette

Over the second half of the 19th century, numerous theories arose concerning mechanisms involved in understanding of action, imitative learning, language development and theory of mind. These explorations gained new momentum with the discovery of the so called “mirror neurons”. Rizzolatti’s work inspired large groups of scientists seeking explanation in a new and hitherto unexplored area of how we perceive and understand the actions and intentions of others, how we learn through imitation to help our own survival, and what mechanisms have helped us to develop a unique human trait, language. Numerous studies have addressed these questions over the years, gathering information about mirror neurons themselves, their subtypes, the different brain areas involved in the mirror neuron system, their role in the above mentioned mechanisms, and the varying consequences of their dysfunction in human life. In this short review, we summarize the most important theories and discoveries that argue for the existence of the mirror neuron system, and its essential function in normal human life or some pathological conditions.

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[The importance of patient reported outcome measures in Pompe disease]

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[In recent decades it has become increasingly important to involve patients in their diagnostic and treatment process to improve treatment outcomes and optimize compliance. By their involvement, patients can become active participants in therapeutic developments and their observations can be utilized in determining the unmet needs and priorities in clinical research. This is especially true in rare diseases such as Pompe disease. Pompe disease is a genetically determined lysosomal storage disease featuring severe limb-girdle and axial muscle weakness accompanied with respiratory insufficiency, in which enzyme replacement therapy (ERT) now has been available for 15 years. In our present study, patient reported outcome measures (PROMs) for individuals affected with Pompe disease were developed which included questionnaires assessing general quality of life (EuroQoL, EQ-5D, SF36), daily activities and motor performance (Fatigue Severity Score, R-PAct-Scale, Rotterdam and Bartel disability scale). Data were collected for three subsequent years. The PROM questionnaires were a good complement to the physician-recorded condition assessment, and on certain aspects only PROMs provided information (e.g. fatigue in excess of patients’ objective muscle weakness; deteriorating social activities despite stagnant physical abilities; significant individual differences in certain domains). The psychological effects of disease burden were also reflected in PROMs. In addition to medical examination and certain endpoints monitored by physicians, patient perspectives need to be taken into account when assessing the effectiveness of new, innovative treatments. With involvement of patients, information can be obtained that might remain uncovered during regular medical visits, although it is essential in determining the directions and priorities of clinical research. For all orphan medicines we emphasize to include patients in a compulsory manner to obtain general and disease-specific multidimensional outcome measures and use them as a quality indicator to monitor treatment effectiveness.]