Hungarian Radiology

[What we need to know on pharmacoeconomics]

SZÁNTÓ Dezső

APRIL 20, 2004

Hungarian Radiology - 2004;78(02)

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Hungarian Radiology

[Necrotising sarcoid granulomatosis: imitator in the chest]

AGÓCS Ágnes, ZIBOTICS Hilda, GREXA Erzsébet

[INTRODUCTION - Both intra and extrapulmonary manifestation of necrotising sarcoid granulomatosis is very rare. It is characterised by variable pulmonary radiological picture, typical histology and benign clinical course CASE REPORT - A young male patient was admitted to our hospital due to a mass lesion in the right apex of the lung suspicious of tuberculosis discovered on plain chest X-ray. Antituberculotic therapy resulted no change of radiological picture. Cytological sample taken by ultrasound guided fine needle aspiration contained no tumor cells. Finally necrotising sarcoid granulomatosis was confirmed by histological examination of the mass removed by atypical surgical resection. CONCLUSION - Intrapulmonary necrotising sarcoid granulomatosis has a variable picture. It has no typical radiological pattern or localisation, therefore it is impossible to diagnose by X-ray morphology. Final diagnosis is based on histology.]

Hungarian Radiology

[RSNA 2003]

MESTER Ádám

Hungarian Radiology

[Long live May Day!]

LOMBAY Béla

Hungarian Radiology

[Opsismodysplasia A report of two cases]

AL Kaissi A, CHEHIDA FB, NESSIB N, GHACHEM MB, KOZLOWSKI K

[INTRODUCTION - Opsismodysplasia is a rare, severe, neonatal dwarfism usually associated with fatal outcome in the first few years of life. Up to, 2003 about 15 cases have been reported. CASE REPORT - We describe two brothers six and four years old with opsismodysplasia, who presented to the paediatric orthopaedic clinic with the diagnosis of short stature and genu varum deformity. CONCLUSION - Paediatric specialists should be aware, that in rare instances, with improving medical care, they may see children with severe bone dysplasias which usually do not reach age in which paediatric orthopaedic services are required.]

Hungarian Radiology

[Lajos Kósa, Zoltán Novák, Edgár Székely: X-ray atlas of the pediatric lung diseases]

LOMBAY Béla

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Clinical Neuroscience

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.

Lege Artis Medicinae

[Second game, 37th move and Fourth game 78th move]

VOKÓ Zoltán

[What has Go to do with making clinical decisions? One of the greatest intellectual challenges of bedside medicine is making decisions under uncertainty. Besides the psychological traps of traditionally intuitive and heuristic medical decision making, lack of information, scarce resources and characteristics of doctor-patient relationship contribute equally to this uncertainty. Formal, mathematical model based analysis of decisions used widely in developing clinical guidelines and in health technology assessment provides a good tool in theoretical terms to avoid pitfalls of intuitive decision making. Nevertheless it can be hardly used in individual situations and most physicians dislike it as well. This method, however, has its own limitations, especially while tailoring individual decisions, under inclusion of potential lack of input data used for calculations, or its large imprecision, and the low capability of the current mathematical models to represent the full complexity and variability of processes in complex systems. Nevertheless, clinical decision support systems can be helpful in the individual decision making of physicians if they are well integrated in the health information systems, and do not break down the physicians’ autonomy of making decisions. Classical decision support systems are knowledge based and rely on system of rules and problem specific algorithms. They are utilized widely from health administration to image processing. The current information revolution created the so-called artificial intelligence by machine learning methods, i.e. machines can learn indeed. This new generation of artificial intelligence is not based on particular system of rules but on neuronal networks teaching themselves by huge databases and general learning algorithms. This type of artificial intelligence outperforms humans already in certain fields like chess, Go, or aerial combat. Its development is full of challenges and threats, while it presents a technological breakthrough, which cannot be stopped and will transform our world. Its development and application has already started also in the healthcare. Health professionals must participate in this development to steer it into the right direction. Lee Sedol, 18-times Go world champion retired three years after his historical defeat from AlphaGo artificial intelligence, be­cause “Even if I become the No. 1, there is an entity that cannot be defeated”. It is our great luck that we do not need to compete or defeat it, we must ensure instead that it would be safe and trustworthy, and in collaboration with humans this entity would make healthcare more effective and efficient. ]

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.]

Clinical Neuroscience

Capability of stroke scales to detect large vessel occlusion in acute ischemic stroke – a pilot study

TÁRKÁNYI Gábor, KARÁDI Nozomi Zsófia, CSÉCSEI Péter, BOSNYÁK Edit, FEHÉR Gergely, MOLNÁR Tihamér, SZAPÁRY László

Rapid changes of stroke management in recent years facilitate the need for accurate and easy-to-use screening methods for early detection of large vessel occlusion (LVO) in acute ischemic stroke (AIS). Our aim was to evaluate the ability of various stroke scales to discriminate an LVO in AIS. We have performed a cross-sectional, observational study based on a registry of consecutive patients with first ever AIS admitted up to 4.5 hours after symptom onset to a comprehensive stroke centre. The diagnostic capability of 14 stroke scales were investigated using receiver operating characteristic (ROC) analysis. Area under the curve (AUC) values of NIHSS, modified NIHSS, shortened NIHSS-EMS, sNIHSS-8, sNIHSS-5 and Rapid Arterial Occlusion Evaluation (RACE) scales were among the highest (>0.800 respectively). A total of 6 scales had cut-off values providing at least 80% specificity and 50% sensitivity, and 5 scales had cut-off values with at least 70% specificity and 75% sensitivity. Certain stroke scales may be suitable for discriminating an LVO in AIS. The NIHSS and modified NIHSS are primarily suitable for use in hospital settings. However, sNIHSS-EMS, sNIHSS-8, sNIHSS-5, RACE and 3-Item Stroke Scale (3I-SS) are easier to perform and interpret, hence their use may be more advantageous in the prehospital setting. Prospective (prehospital) validation of these scales could be the scope of future studies.

Clinical Neuroscience

Chronic form of Pisa syndrome after prolonged exposure to low-dose amisulpride treatment

ERDEM Şimşek Nazan, ÖZKAYNAK Sibel Sehur

Pisa syndrome is a movement problem defined by tonic, sustained lateral flexion with a slight posterior rotation of the trunk. It seems to be a side effect of antipsychotic medicine in most cases. The clinical duration of Pisa syndrome can be acute, chronic, or recurrent. As far as we know, no reports are available in the literature on the chronic form of Pisa syndrome caused by low-dose amisulpride. A case of refractory tardive dystonia form of Pisa syndrome during treatment with stable low-dose amisulpride is presented in this report. Long-term, low-dosage amisulpride therapy may induce tardive dystonia even in patients with no other risk factors for dystonia.