Clinical Neuroscience

[Extrapyramidal diseases 1996]

VÉCSEI László

SEPTEMBER 20, 1996

Clinical Neuroscience - 1996;49(09-10)

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Clinical Neuroscience

[Hemodynamic adaptation of fetal brain]

JAKOBOVITS Ákos, HENDRIK Jörn

[Doppler color ultrasonography of the middle cerebral and umbilical arteries was performed on 104 fetuses born at term. A total of 254 investigations were carried out. Of the 104 fetuses studied, 52 infants had birth weights appropriate for gestational age (mean 3409.2 g) and 52 infants were small for gestational age (mean 2272.1 g). Cerebral hemodynamic adaptation was observed in growth retarded fetuses due to placenta! insufficiency. ln these cases the elevated umbilical vascular resistance evidenced the placenta! insufficiency. At the same time the decrease of the cerebral vascular velocimetry indexes indicated the improving cerebral blood supply. Only the systolic/diastolic ratio was significantly reduced in growth retarded fetuses when compared with normal controls. ln the umbilical artery the pulsatility index and systolic/diastolic ratio were raised significantly in growth retarded fetuses. The ratio of the cerebral arterial to umbilical cord artery index values proved a better indicator of the difference between growth retarded and normal controls than the index of the cerebral or umbilical artery alone. The ratios of all three index values of the growth retarded fetuses were significantly smaller than those of the normal controls (pulsatility index 1.03 versus 1. 60, resistance index 0.84 versus 1.19 and systolic/diastolic ratio 1.01 versus 2.02). The ratios of the small for date fetuses due to other, nonplacental causes were simi­ lar to the normal controls. The blood circulation disorder evokes hemodynamic adaptation in the feta! brain. The intrauterine growth restriction is a consequence of this disturbed blood supply. The cerebral circulatory adaptation failed in the small for date fetuses non associated with decreased blood supply.]

Clinical Neuroscience

[Distal Ulnar Neuropathies]

KISS Gábor, KÓMÁR József

[Compression of the distal part of the ulnar nerve is an uncommon entrapment neuropathy. Depending on the site of compression it may result in pure motor symptoms. The atrophy of the small hand muscles without sensory deficit may mimic motor neuron disease. Correct clinical examination and electrophysiology including measurement of ulnar distal motor latency to the first dorsal interosseous muscle can reveal the correct diagnosis. 46 surgically treated cases of distal ulnar neuropathy are reported. 12 patients had only muscle weakness without sensory abnormalities. 6 of them showed motor deficit restricted to the first dorsal interosseous and adductor pollicis muscles. 34 patients were followed up 31 of them improved, 3 cases should be reoperated because of recidive lipoma.]

Clinical Neuroscience

[Importance of sociological and psychological factors in pathogenesis of ischaemic stroke]

MENDE Lilla, JÓRI Birkás Adrien, FAZEKAS Gábor, FAZEKAS András

[The authors investigate sociological and psychological factors in the pathogenesis of ischaemic stroke, as a follow up of their earlier study. In the first part of the recent study patients and control subjects are compared in terms of frequency, severity, type and the temporal distribution of life events within the 5 years preceding the stroke. In the second part the importance of coping-style and psychological characteristics are studied. It is shown that the likely factors leading to stroke are: lower life events score in the more distant period, but higher life events score preceding the stroke, poor coping, greater emotional instability, trait-anxiety, hostility, and decreased job involvement. The type of life events, global Type-A behaviour, "speed and impatience", "hard driving and competitiveness” seem indifferent. On the basis of these results a successful stroke-programme should consider the complexity of somatic, sociological and psychological factors.]

Clinical Neuroscience

[Transoesophageal echocardiography after stroke]

NAGY Lajos, SÁMOCYI Marianna, TARJÁN Jenő, GARZULY Ferenc

[40 stroke patients were studied by both transoesophageal and transthoracic echocardiography. The diagnosis of stroke was based on medical history, physical examination and computerized tomographic brain scan. 39 patients underwent carotid duplex scan as well. Transoesphageal echocardiography was used to examine 40 patients of whom 19 had cardiac source of embolism. Out of these, 7 patients had definitive, whereas 12 had possible cardiac source of embolism. Transthoracic echocardiography was diagnostic only in 8 cases. Using carotid duplex scan, carotid stenosis was detected in 8 patients and sclerosis without significant stenosis in 8 others. From each of these two groups 4 patients had coexistent cardiac source of embolism as well. In the 8 patients with atrial fibrillation the left atrial thrombus and spontaneous echo contrast were more frequent than in patients with sinus rhythm. The transoesophageal echocardiography altered the management of antico agulation in 3 patients. The authors concluded that transoesophageal echocardiography is necessary in stroke patients.]

Clinical Neuroscience

[Prognosis of neuroepithelial tumours by means of cell proliferation studies]

GYÖMÖRI Éva, MÉSZÁROS István, MÉHES Gábor, DÓCZI Tamás, PAJOR László

[Cell-kinetic analysis of tumours has recently been widely used in clinical oncology for prognosis of patients treated with malignant neoplasms and for controlling the efficiency of treatment protocols. Definition of biological nature of neuroepithelial tumours was based on grading depending on the severity of cellular anaplasia. Neuroepithelial tumours can be characterized not only by the histological features but also by the DNA content and abnormalities of the cell proliferation – though the relationship between histological malignancy, proliferative activity and cellular aneuploidity was found to be rather controversial according to the literature. In this review article the clinical value of cell cycle analysis such as distribution of DNA content, DNA index; S-phase fraction; proliferation-markers [MIB 1 antibody, bromdeoxy uridin labelling index, mitotic index, definition of nucleolar organization region) are discussed on the basis of personal experience and review of the appropriate literature. Flow cytometry and examination of proliferation markers have a significant role in the definition of prognosis of patients suffering from WHO grade II and III neuroepithelial tumours. Gliomas giving rise to recidivism have a rapid cell cycle already at their first occurrence, which is characterized by raised proliferation indices, and occurrence of aneuploid cell clones. An unfavourable outcome can be prognosed in patients suffering from a WHO grade II or III glioma if the DNA index is above or below 1+0.1 if the value of the S phase fraction is above 6%, if more than 1 mitosis is found in 10 large magnification field, and if the number of cells labelled with MIB 1 antibody exceeds 3 in 1 large magnification field. The literature confirms our notion that further studies of proliferation characteristics may help in the production of a malignity score of gliomas that could support the efficiency of traditional histological grading in prognosis and control of complex therapy of these tumours.]

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[History of vaccine production in Hungary ]

ÓCSAI Lajos

[This study presents the complete history of the Hungarian vaccine production, partly in association with the process of fighting vaccine-preventable infectious diseases, and underlines the fact that every government actively contributed to the age-adjusted mandatory vaccination schedule of the past 140 years. It demonstrates the various achievements from the smallpox lymph production through the launch of diphtheria serum production at Phylaxia and the establishment of the National Public Health Institute (OKI) with its vaccine production and the later institutional transformation of OKI into Humán as economic corporation to its closure. Among all OKI’s vaccine production activities, this study focuses on the production of influenza vaccines, due to its international importance in the 1960s and 1970s. The vaccine production against diphtheria tetanus and pertussis stands out from Humán’s activities, and the tetanus component of this vaccine is still used in the products of a multinational vaccine manufacturer. ]

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[Diagnosis and treatment of microvascular coronary heart disease. Specialities of conditions in Hungary]

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[Invasive investigations show that in two-thirds of patients the myocardial ischaemia persists without obstructive coronary disease and any other heart conditions (INOCA). The underlying cause may be microvascular dysfunction (CMD) with consecutive microvascular coronary disease (MVD) and microvascular or epicardial vasospastic angina (MVA). The modern practice of clinical cardiology while using the developed non-invasive cardiac imaging permits exact measuring of the coronary flow with its characteristic indices. All of these improve the diagnosing of CMD-induced myocardial ischemia and provide opportunity to determine primary MVD cases. Since the recognition and treatment of MVD is significantly underrep­resented in the Hungarian medical care, the primary stable microvascular angina (MVA) is described in detail below with its modern invasive and non-invasive differential diagnosis and treatment, concerning especially its frequency provoked by high blood pressure and female coronary heart diseases. There are highlighted all recommended diagnostic procedures available under domestic conditions.]

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[Association between cyclothymic affective temperament and hypertension]

NEMCSIK János, BATTA Dóra, KŐRÖSI Beáta, RIHMER Zoltán

[Affective temperaments (cyclothymic, hypertymic, depressive, anxious, irritable) are stable parts of personality and after adolescent only their minor changes are detectable. Their connections with psychopathology is well-described; depressive temperament plays role in major depression, cyclothymic temperament in bipolar II disorder, while hyperthymic temperament in bipolar I disorder. Moreover, scientific data of the last decade suggest, that affective temperaments are also associated with somatic diseases. Cyclothymic temperament is supposed to have the closest connection with hypertension. The prevalence of hypertension is higher parallel with the presence of dominant cyclothymic affective temperament and in this condition the frequency of cardiovascular complications in hypertensive patients was also described to be higher. In chronic hypertensive patients cyclothymic temperament score is positively associated with systolic blood pressure and in women with the earlier development of hypertension. The background of these associations is probably based on the more prevalent presence of common risk factors (smoking, obesity, alcoholism) with more pronounced cyclothymic temperament. The scientific importance of the research of the associations of personality traits including affective temperaments with somatic disorders can help in the identification of higher risk patient subgroups.]

Clinical Neuroscience

Neuroscience highlights: Main cell types underlying memory and spatial navigation

KRABOTH Zoltán, KÁLMÁN Bernadette

Interest in the hippocampal formation and its role in navigation and memory arose in the second part of the 20th century, at least in part due to the curious case of Henry G. Molaison, who underwent brain surgery for intractable epilepsy. The temporal association observed between the removal of his entorhinal cortex along with a significant part of hippocampus and the developing severe memory deficit inspired scientists to focus on these regions. The subsequent discovery of the so-called place cells in the hippocampus launched the description of many other functional cell types and neuronal networks throughout the Papez-circuit that has a key role in memory processes and spatial information coding (speed, head direction, border, grid, object-vector etc). Each of these cell types has its own unique characteristics, and together they form the so-called “Brain GPS”. The aim of this short survey is to highlight for practicing neurologists the types of cells and neuronal networks that represent the anatomical substrates and physiological correlates of pathological entities affecting the limbic system, especially in the temporal lobe. For that purpose, we survey early discoveries along with the most relevant neuroscience observations from the recent literature. By this brief survey, we highlight main cell types in the hippocampal formation, and describe their roles in spatial navigation and memory processes. In recent decades, an array of new and functionally unique neuron types has been recognized in the hippocampal formation, but likely more remain to be discovered. For a better understanding of the heterogeneous presentations of neurological disorders affecting this anatomical region, insights into the constantly evolving neuroscience behind may be helpful. The public health consequences of diseases that affect memory and spatial navigation are high, and grow as the population ages, prompting scientist to focus on further exploring this brain region.

Clinical Neuroscience

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

MOLNÁR Mária Judit, MOLNÁR Viktor, LÁSZLÓ Izabella, SZEGEDI Márta, VÁRHEGYI Vera, GROSZ Zoltán

[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.]