Paraneoplastická limbická encefalitida je vzácné onemocnění náležící do skupiny paraneoplastických neurologických syndromů. V diagnostice hraje zásadní roli zobrazení mozku magnetickou rezonancí. Předkládáme kazuistiku 57letého muže, u něhož vyšetření magnetickou rezonancí jistě přispělo ke stanovení této diagnózy. Další významnou součástí diagnostického postupu je průkaz specifických, takzvaných antineuronálních autoprotilátek Prognóza choroby nebývá dobrá, je závislá především na možnostech suprese základního tumorózního onemocnění., Paraneoplastic limbic encephalitis is a rare disorder which belongs to a group of paraneoplastic neurologic syndromes. The diagnostic role of magnetic resonance imaging (MRI) is of utmost importance. We report a case of a 57-year-old man in whom MRI significantly contributed to establishing of proper diagnosis. Other important diagnostic test includes identification of specific, so called antineural auto antibodies. Typically, the prognosis of the disease is not good; it depends mainly on the ability to control the underlying malignant disease., Jandura J, Ryška P, Žižka J, Klzo L, Eliáš P, Štěpán P, Adamkov J., and Literatura 10
Only a few cases of pneumocystis pneumonia (PCP) in Cushing’s syndrome have been published in the literature so far. In the majority of these patients, the pneumonia occurred after reduction of the hypercortisolism with medicamentous treatment. We report two cases of PCP during conservative treatment of hypercortisolism. We describe clinical, imaging and laboratory findings in two patients and review published cases of pneumocystits pneumonia in Cushing’s syndrome. A 60-year-old woman and 20-year-old man with Cushing’s syndrome due to ectopic ACTH syndrome were treated at our department. Both developed pneumocystis pneumonia early after treatment with ketoconazole and ethomidate bromide had been introduced and the levels of cortisol rapidly decreased. PCP prophylaxis in patients with high cortisolemia should be started before treatment of hypercortisolism in current practice. Gradual lowering of plasma cortisol should also reduce the risk of infection by Pneumocystis jiroveci., Filip Gabalec, Alžběta Zavřelová, Eduard Havel, Jaroslav Cerman, jr., Jakub Radocha, Ioannis Svilias, Jan Čáp, and Literatura 10
According to our research, Uzbek individuals with Parkinson’s disease have high prevalence of 0/0 polymorphisms of GSTT1 and GSTM1 genes, as well as combinations of GSTT1 (0/0)/GSTM1/(0/0) genotypes. These mutations are associated with an earlier debut of the disease, its mixed form, and rapid rate of progression. Determination of mutations in GSTT1 and GSTM1 genes of xenobiotic detoxification in patients with Parkinson’s disease confirms multifactorial nature of this pathology and the role of the influence of various external factors in the modification of clinical signs of disease and its prognosis., Khanifa Khalimova, Malika Raimova, Rustam Matmurodov, Khurshidakhon Rasulova, Amina Burnasheva, Yekaterina Jmirko, Rustam Mukhamedov, and Literatura
Purpose: To investigate the protective action of Ceruloplasmin (Cp) on the lysis of erythrocytes in men with prostate tumors. Material and Methods: The blood erythrocytes of the patients with benign hyperplasia of prostate (BHP) and prostate adenocarcinoma (CaP) were studied. Patients at the age 60-75 with early stages of a cancer have been investigated. The control group was consisted of apparently healthy males with the compatible age. n=15 for each group. The clinical stage of the disease was diagnosed by means of rectal, histological and echographic examination of the prostate gland. Photometric methods were applied to register lysis dynamics in order to test out the protective action of Cp. Results: The nonspecific protective function of Cp preparation on BHP and CaP erythrocytes as well as on the control group erythrocytes was revealed. CaP erythrocytes have shown more sensitivity to the lysis provocative factor than BHP and the control group erythrocytes, that was presumably attributed to the structural and functional changes of the erythrocytes developed in the presence of malignant tumor., N. Kotrikadze, M. Zibzibadze, L. Ramishvili, M. Alibegashvili., T. Chigogidze, L. Managadze, and Literatura
Cíl. Pseudomyxom peritonea je velmi vzácné semimaligní onemocnění. Záměrem našeho sdělení je pojednání o typickém nálezu, možnostech léčby a komplikacích. Metoda. Nedávné případy z našeho pracoviště ilustrují patognomické znaky nemoci v CT obrazu, obvyklý klinický nález, průběh onemocnění i svízelnost jeho léčby. Ve dvou našich kazuistikách je příčinou pseudomyxomu peritonea mucinózní cystadenom appendixu, v jednom appendikální low grade cystadenokarcinom, v posledním případě mucinózní cystadenokarcinom neznámého původu. U prvního pacienta se navíc objevila raritní komplikace - metastatická ložiska intrathorakálně. Závěr. Pseudomyxom peritonea je klinický syndrom, který je způsoben excesivní produkcí gelatinózního ascitu buňkami mucinózního cystadenomu či cystadenokarcinomu různého původu, nejčastěji se však jedná o tumory appendixu. Typický morfologický nález v zobrazovacích metodách určuje jejich významné místo v diagnostice, stagingu a monitorování výsledků léčby pseudomyxomu., Aim. Pseudomyxoma peritonei is an uncommon semimalignous disease. The aim of our statement is the discussion about the typical finding, the possibilities of treatment and complications. Method. The recent findings from our workplace illustrate pathological signs of the disease on CT-scan, usual clinical finding, the progression of the disease and the arduous of its treatment. In two of our cases is the cause of pseudomyxoma peritonei mucinous cystadenoma of the appendix, in one low grade mucinous cystadenocarcinoma and in the last case the mucinuos cystadenoma of unknown origin. There has appeared by the first patient a rare complication - intrathoracic implants lesions. Conclusion. Pseudomyxoma peritonei is a clinical syndrome that is caused by the excessive production of gelatinous ascites cells mucinous cystadenoma or cystadeno-karcinoma of different origin, but mostly these are the cases of appendix tumors. The typical morphological found in the imaginig methods determines their important place in the diagnostics, staging and the monitoring of the results of the treatment of pseudomyxoma., Žáčik M, Kacvinská T, Pernická B, Pospíšková M, Palíšek J., and Literatura 12
Stiff-person syndrome (SPS) is a rare disorder characterized by muscle stiffness and painful spasms. Misdiagnosis may occur due to the fact that the clinical picture of SPS is often atypical. The main pathophysiologic mechanism underlying the development of SPS is insufficient inhibition at the cortical and spinal levels. There is good evidence for a primary autoimmune etiology. A 61-year-old man was admitted to a neurological department due to muscle hypertonia with episodic attacks of painful spasms predominantly affecting axial muscles. The symptoms developed shortly after tickborne meningoencephalitis. Electromyography (EMG) revealed signs of continuous motor unit activity. Antibodies against glutamate decarboxylase (anti-GAD) were highly elevated. We present a case of a man who developed clinically severe anti-GAD positive SPS, provoked by tick-borne encephalitis. After therapeutic plasma exchange (TPE) a rapid, temporary improvement of the clinical and neurophysiological findings was noted. Only after being placed on long-term immunosuppression did the patient achieve stable recovery. This case supports the importance of EMG findings and demonstrates the effect of TPE as well as the need for chronic immunosuppression in severe cases of SPS., Edvard Ehler, Jan Latta, Petra Mandysová, Jana Havlasová, Milan Mrklovský, and Literatura 22
Mediterranean spotted fever (MSF) in Bulgaria shows increasing severity, especially in patients over sixty years. As T lymphocytes are the primary effector cells in MSF and are altered by the ageing process we aimed to specify the changes in their numbers in elderly patients. We performed a clinicoepidemiological study, haematological and biochemical analysis of 132 patients of 60+ and 30 patients between 19 and 57 years with MSF. We investigated the cell immunity of 20 patients of 60+ using immunofluocytometry. The control group consisted of 10 younger patients and 10 healthy individuals of 60+. MSF showed more unfavourable course in the elderly.We found T-lymphocytes depletion in all patients with MSF. Activated T lymphocytes were increased in elderly patients with MSF. We found significant differences between the number of activated T lymphocytes in elderly patients and healthy elderly persons. The observed T-lymphocytes depletion in MSF corresponded to the disease severity. The increase in the activated T lymphocytes in patients over 60 years contributes to an adequate immune response., Margarita Gospodinova, and Literatura
Cieľ: V práci sme sa zamerali na vedomosti pacientov s astmou v oblastiach self-manažmentu tohto ochorenia a najmä na vplyv faktoru dĺžky trvania tohto ochorenia na úroveň vedomostí o self-manažmente. Zároveň sme zisťovali vzťah medzi úrovňou vedomostí o self-manažmente ochorenia a kontrolou astmy. Metodika: V tejto prierezovej štúdii bolo do súboru zaradených 297 respondentov. Na testovanie kontroly astmy a úrovne vedomostí pacientov sme použili valídne nástroje a to Dotazník vedomostí o self-manažmente astmy pre dospelých (Measuring asthma self-management knowledge in adults) a Test kontroly astmy (Astma Control Test ACTTM). Na štatistické spracovanie dát boli využité: Pearsonov Chi-kvadrat test nezávislosti a Spearmanov korelačný koeficient. Výsledky: Až 220 z 297 respondentov (74,07 %) preukázalo nedostatočné vedomosti, a to konkrétne v doméne Vedomosti o liečbe astmy. V oblasti Manažment bronchiálnej astmy malo nedostatočné vedomosti 191 respondentov (64,31 %). Početnosť správnych odpovedí zároveň naznačuje, že úroveň vedomostí v doménach self-manažmentu astmy, významných pre kontrolu ochorenia, bola vyššia v skupine pacientov diagnostikovaných v priemere jeden rok ako v skupine, ktorá trpí ochorením dlhšie (6-10 rokov), i keď rozdiel nie je štatisticky významný. Záver: Nedostatky vo vedomostiach astmatikov, vyššia frekvencia chýb v oblastiach self-manažmentu, vrátane self-monitoringu a horšia kontrola astmy pri ochorení diagnostikovanom viac ako 6 rokov naznačujú, že je potrebné v klinickej praxi venovať čas a pozornosť reedukácii, opakovanému posúdeniu vedomostí a praktických zručností (aplikácia metód self-manažmentu, inhalačnej techniky) v skupine už skôr diagnostikovaných astmatikov., Aim: This project is aimed to find out the patient knowledge with asthma in the area of self-management of this disease and particularly the influence of the length of the disease on the knowledge level about self-management. At the same time we tried to find out the relation between the knowledge level of the asthma self-management and control of asthma disease. Methods: The total number of 297 adult asthma patients were included in the cross sectional study. The valid instruments Asthma Control Test and Measuring asthma self management knowledge in adults were used for testing asthma control and the degree of patient knowledge in self management of asthma. The Chi-squared test and the Spearman´s rank correlation coefficient were used for statistical processing of data. Results: A total of 220 out of 279 respondents (74%) showed poor knowledge of selfmanagement of asthma, particularly in “Knowledge of asthma treatment” domain and 191 patients in “Management of bronchial asthma” domain (64%). Patients who had been diagnosed one year ago had better level of knowledge in selfmanagement of asthma than those who had been treated for asthma longer than six years (6-10 years). This difference was not significant, but it was related to those domains which has significant influence on asthma control (domains 2-5). Conclusion: Poor knowledge of asthma patients, higher frequency of errors in self management and worse control of asthma in patients with asthma longer than six years imply the need for better and repeated education, check of patient knowledge and skills for self-management, use of peak flow meter included in this group of patients., Michaela Dingová, Petronela Osacká, and Literatura