Clinical Neuroscience

[The operation of the neurological institutes in 1967 ]

SIMEK Zsófia1

AUGUST 01, 1969

Clinical Neuroscience - 1969;22(08)

[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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  1. Országos Ideg- és Elmegyógyintézet Szervezési Módszertani Osztály

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Clinical Neuroscience

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

HALÁSZ Péter, MOLNÁR Gyula, HIDASI József

[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 ]

PANETH Gábor

[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.]

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[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.]

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[Essential familial myoclonus]

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[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. ]

Clinical Neuroscience

[Electrophysiological changes after local electron irradiation of the brain]

PÁSZTOR András, TOMKA Imre, SARKADI Ádám

[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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Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

Evaluation of the effectiveness of transforaminal epidural steroid injection in far lateral lumbar disc herniations

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Clinical Neuroscience

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Clinical Neuroscience

[What happens to vertiginous population after emission from the Emergency Department?]

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[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.