Hungarian Radiology

[REVIEW OF THE LITERATURE]

GYENES György

MARCH 20, 2006

Hungarian Radiology - 2006;80(01-02)

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Hungarian Radiology

[Summaries of the lectures on the 20th Sopron Ultrasound Days]

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[Diagnostic pitfalls and artifacts of multislice CT]

BARANYAI Tibor

[There is a spectacular development in diagnostic radiology in the last one and a half decades. State-of-the-art US, CT and MR appliances and the dynamic software developments has improved diagnostic safety by order of magnitude, which resulted in the reduction of possible errors and misinterpretations. The advent of MSCT resulted in shorter scanning times, the submillimeter collimation and the subsecond scan time improves the spatial resolution of the image, the motion artifacts are reduced and the evaluation of the parenchymal organs improves. However, the new technology of MSCT raises new questions. Due to faster data collection the acquisition time decreases, that is why the tracing of the contrast material must be accurately timed. The high contrast material density that appears suddenly in pulsing vessels makes a disturbing effect on its environment, thus making way to erroneous interpretation. The performance of a secondary reconstruction (2D and 3D reconstructions) may diminish the possibility of diagnostic pitfalls and artifacts. Reconstruction increments made from appropriately overlapping thin slices are required for good image quality and spatial resolution, otherwise the image quality is deteriorating, some vessels might “disappear”, they are not depicted. We are struggling with several problems using MIP CTangiography. The proper elimination of the bones, the improper selection of VOI (volume of interest) might lead to false positive result, and the assessment of small vessels might become impossible. The differentiation of soft plaque and vessel thrombus can also be a problem, and the hard plaque may imitate a constriction. The knowledge of breath and pulsating motion artifacts, beam-hardening artifacts and flow-related artifacts is essential. Differentiating difficulties during virtual endoscopy, the partial volumen effect, the interpretation of various post-operative conditions, the disturbing effects of implants may cause diagnostic and differential diagnostic problems. The author gives a summary of possible errors, misinterpretations and artifacts that may occur with the application of MSCT even if examination protocols are followed.]

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[Board meeting of the Hungarian College of Radiology]

PALKÓ András, FORRAI Gábor

Hungarian Radiology

[Oftex: “Doctors’ Continuous Postgraduate Education Electronic Index”]

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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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[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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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.