Hungarian Radiology

[„Brain-circling”]

LOMBAY Béla

FEBRUARY 20, 2004

Hungarian Radiology - 2004;78(01)

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Further articles in this publication

Hungarian Radiology

[Conference of the Senior and Junior Club of the Society of Hungarian Radiologists]

MAKULA Éva

Hungarian Radiology

[Cornell Seminar]

VÁRKONYI Ildikó

Hungarian Radiology

[Recovery of multiple brain abscesses caused by Serratia marcescens in newborn age]

FEJES Melinda, BORBÁS Éva, PAPP Attila, SZÉKHELYI Zsuzsanna, SZŰTS Ágnes

[INTRODUCTION - Multiple brain abscesses caused by Serratia marcescens is a rare disease in newborn infants. The paper describes the development, case history and treatment of the disease. Radiological features of Gram negative bacterial meningitis and brain abscesses are also discussed. PATIENT AND METHODS - A newborn baby boy presented polycythaemia and fever was admitted to the hospital in the first days of his life. On the 11th day after admission convulsions occured and because of suspected intracranial abnormality or meningitis cranial CT was performed. On CT scans multiple abscesses were revealed and surgical therapy including drainage and ventricle shunt was done. During the treatment he had epileptic seisures frequently, but he became symptome free after the introduction of complex antiepileptic therapy. Now the two year old boy is in a good physical condition but he has mild motoromental deficience. CONCLUSION - Radiological imaging plays an important role in the diagnosis and the follow up of brain abscesses and also in the evaluation of its complications.]

Hungarian Radiology

[Future planning of radiology before joining the EU]

BARANYAI Tibor

Hungarian Radiology

[Theoretical basis of tumor staging The significance of imaging in oncological diseases]

GŐDÉNY Mária

[The general goal for staging patients with any cancer is to determine the overal extent of disease prior to choice of therapy. It has been well documented that biological behavior of any cancer and, therefore, the prognosis of the patient is strongly linked to the extent of the tumor, the presence or absence of lymphatic dissemination as well as systemic metastases. The staging systems have undergone a number of modifications. Classifications of TNM system identifies the depth of tumor invasion (T), the status of regional lymph nodes (N) and the distant metastases. These three parameters are then incorporated into the final clinical stage. There are further modifications which influence the patient survival, e.g. biological, genetical, hystological factors, tumor grade. All cancer cells show dysregulation of cell cycle controll. As the cancer proliferates and the tumor reaches approximately 1-2 mm in diameter further growth recquires the development of new blood vessels (neo-angiogenesis). Intensity of tumor growth has a prognostic influence to the patient's life and depends on the tumor doubling time, which classifies tumors into slow, intermadiate and rapid growing types. The diagnostic impact of imaging is based on the ability of a technique to detect and evaluate the cancer accurately. The very high accuracy and reproducibility of cross-sectional imaging, paticularly computed tomography (CT) and magnetic resonance imaging (MRI) make these methods extremely important in the detection, staging and in the evaluation of the tumors. The revolutionary advances in detection and treatment of malignant disease have led to an increasing role of the radiologist as a member of the multidisciplinary cancer team.]

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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.

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

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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.

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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

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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.

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