Hungarian Radiology

[REVIEW OF THE LITERATURE]

GYENES György, WENINGER Csaba

FEBRUARY 15, 2005

Hungarian Radiology - 2005;79(01)

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Hungarian Radiology

[2005, International Year of Physics]

LOMBAY Béla

Hungarian Radiology

[Gastrointestinal stromal tumors]

BAHÉRY Mária

[Gastrointestinal stromal tumors are the most common mesenchymal neoplasm of the gastrointestinal tract. Gastrointestinal stromal tumors are characterized by remarkable variability in their differentiation potential. They are defined by their expression of KIT (CD117), a tyrosine kinase growth factor receptor. The expression of KIT is important to distinguish gastrointestinal stromal tumors with immunohistochemically method from other mesenchymal neoplasms such as leiomyomas, leiomyosarcomas, leiomyoblastomas and schwannomas. Pathologically proved gastrointestinal stromal tumors are appropriate KIT-inhibitor therapy. Gastrointestinal stromal tumors arise with variable frequency throughout the gastrointestinal tract, they most frequently occur in the stomach (40-70%), followed by the small intestine (20-30%), colorectum (5- 15%) and esophagus (<5%). The most gastrointestinal stromal tumors arise within the muscularis propria, they most commonly have an exophytic growth pattern and manifest as dominant extraluminal masses. Radiologic features of gastrointestinal stromal tumors vary depending of tumor size and organ of origin. They are characteristically well circumscribed, sharply defined and have hemorrhage, necrosis or cyst formation. The radiologic features of gastrointestinal stromal tumors are often distinct from those of epithelial tumors. There are no specific radiologic features to separate gastrointestinal stromal tumors from other mesenchymal tumors, yet.]

Hungarian Radiology

[Esophageal diverticula in mixed connective tissue disease]

SZÁNTÓ Dezső, SZŰCS Gabriella, DITRÓI Edit

[INTRODUCTION - The functional and morphological changes of oesophagus occur in two third of mixed connective tissue disease patients according to the literature. CASE REPORT - We report three cases of 27, 39 and 48 year old women suffering of lateral pharyngoesophageal, epibronchial and epiphrenic diverticula associated with connective tissue disease. Diverticula had an average diameter of 3.8 cm (maximal diameters: 7.2-8 cm). The esophageal pouches produced dysphagia, dystonia, motility disorders, food stagnation and vomiting, retrosternal burning sensation and tachyarrythmia after 5-16 month's latency period. In one patient pneumoesophagus also evolved. The high serum enzyme levels and proximal electromyogram proved the presence of polymyositis. CONCLUSION - The localization, number and the size of esophageal diverticula are determined by the interstitial myopathy.]

Hungarian Radiology

[Basics and methods of radiostereometric analysis in orthopaedic practice]

ILLYÉS Árpád, KISS Rita M., KÖLLŐ Katalin, KISS Jenő

[Photogrametry is a discipline processing geometric information of images according to the general image concept. Radiostereometric analysis (Roentgen stereophotogrametric analysis) is the most dynamically developing area of the orthopaedic photogrametry. The aim of the authors was to summarize the different methods of radiostereometric analysis in use. Two basically different methods are used: marker-based and model-based radiostereometric analysis. The authors summarize and compare the steps, the advantages and the disadvantages of either method. Radiostereometric analysis is because of its high precision appropriate as a tool in scientific research and diagnostic in orthopaedics, such as detecting the 3D micromotion of different components of implants, the wear of polyethylene inserts or determining anterior-posterior and rotational movements of the knee. Analyzing the reproducibility and accuracy of the reconstruction methods one can establish that the accuracy of analytical methods in use is behind the accuracy of digital automated software. The precision of the model-based radiostereometry can never achieve the accuracy of the marker based radiostereometry because of the inaccuracy of the initial input model of implants, although the one of the method of reversed engineering - one of model-based radiostereometric analysis - can approach the accuracy of marker-based radiostereometry.]

Hungarian Radiology

[Solitary fibrous tumor of the chest]

AGÓCS Ágnes, KISHINDI KISS Katalin, PENCS Mónika, TÓTH Tivadar

[INTRODUCTION - Solitary fibrous tumor is a rare, in most of the cases benign tumor of mesenchymal origin arising from the pleura. CASE REPORT - Significantly raised right hemidiaphragm was noted on the chest X-ray of an elderly female patient with good general condition. A large solid mass lesion was seen above the normally positioned right hemidiaphragm on ultrasound and chest CT examinations. The mass was removed by surgical resection and a solitary fibrous tumor was confirmed by histological and immunhistochemical examinations. CONCLUSION - Solitary fibrosus tumor is a rare and histologically benign tumor. Occasionally it may enlarge rapidly and transform into malignant variant after several years. Therefore complete surgical resection and long term follow up is needed in all cases.]

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FARSANG Csaba

[Nonsteroidal antiinflammatory drugs (NSAIDs) are among the most frequently used pharmaceuticals. Nevertheless, a number of studies emphasized that NSAIDs were damaging not only the gastrointestinal (GI), but also the cardiovascular (CV) system, could increase the blood pressure, the frequency of coronary events (angina, myocardial infarction) and stroke incidence, as well as they might deterio­rate renal functions. The National Institute for Health and Care Excellence (NICE) did not find evidence that administering NSAIDs could increase the risk of developing COVID-19 or worsened the condition of COVID-19 patients. However, unwanted effects of specific drugs differ substantially in their occurrence and seriousness as well. It seemed to be for a long time that the NSAIDs provoked higher GI-risk was closely related to the COX1/COX2 selectivity, like the cardiovascular (CV) risk to the COX2/COX1 selectivity, however, the recent data did not prove it clearly. Based on the available literature while pondering the gastrointestinal and cardiovascular adverse events, among all NSAIDs the aceclofenac profile seemed to be the most favourable.]

Clinical Neuroscience

Neuroscience highlights: The mirror inside our brain

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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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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

[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

BOCZÁN Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette, SZÉLL Márta, KARCAGI Veronika, ZÁDORI Dénes, MOLNÁR Mária Judit

[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]

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Possible genotype-phenotype correlations in Niemann-Pick type C patients and miglustat treatment

ÇAKAR Emel Nafiye, ÖNAL Hasan

Niemann-Pick type C is a rare lysosomal storage disease caused by impaired intracellular cholesterol transport. The autosomal recessive disease is caused by mutations in NPC1 or NPC2 genes. Clinical-laboratory features, genotype-phenotype correlation and miglustat treatment response of our patients diagnosed with early infantile Niemann-Pick type C were evaluated. In this article, four Niemann-Pick type C patients diagnosed in the early infantile period are presented. Common features of our patients were hepatomegaly, splenomegaly, cholestasis and retardation in motor development. Patients 1 and 2 are twins, with homozygous mutation c.2776G>A p.(Ala926Thr) in NPC1 gene and severe lung involvement. Lung involvement, which is mostly associated with NPC2 gene mutation in the literature, was severe in our patients and they died early. In patients 3 and 4, there were respectively c.2972del p.(Gln991Argfs*6) mutation in NPC1 gene and c.133C>T p.(Gln45*) homozygous mutation in NPC2 gene. In these two patients, improvement in neurological findings were observed with treatment of miglustat. In our twin patients, severe lung involvement was observed. Two of our four early infantile Niemann-Pick type C patients exhibited neurological gains with miglustat treatment.