Lege Artis Medicinae

[The 25th Congress of Colon Section of the Hungarian Society of Gastroenterology - Gyula, January 21-22. 2005.]

ÚJSZÁSZY László

APRIL 20, 2005

Lege Artis Medicinae - 2005;15(04)

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Lege Artis Medicinae

[In the focus: Allergy]

NÉKÁM Kristóf

Lege Artis Medicinae

[COMMODORE]

MATOS Lajos

Lege Artis Medicinae

[NOVEL RESULTS ON SYSTEMIC LUPUS ERYTHEMATOSUS]

KISS Emese, GERGELY Péter, SZEGEDI Gyula

[Systemic lupus erythematosus is an autoimmune disorder. It stands at the focus of medical interest: basic scientists, clinicians and innovative biotechnologists all pay attention to lupus. Authors of this article present the novel scientific results on the etiopathogenesis, clinical and laboratory characteristics of SLE. Furthermore, authors discuss diagnostic problems and the possible therapeutic modalities. One of the most important results is the characterisation and mapping of the susceptibility genes as well as the analysis of their functional features. More and more is known about the relationship between natural and adaptive immunity, about the cooperation of T and B cells. The abnormalities of intracellular biochemical processes and signal transduction pathways have been cleared. The importance of cytokine network and infective agents in the pathogenesis of SLE has largely been investigated recently. With regards to the outcome of the disease, there is growing attention paid on chronic organ damages, such as end-stage renal disease, osteoporosis and atherosclerosis - in connection with the increased life expectancy. Evidence accumulates on the importance of immune-inflammatory processes in the initiation and perpetuation of osteoporosis and atherosclerosis. There is an urgent need for validated biomarkers which can predict the susceptibility, prognosis, clinical manifestations, activity and severity of SLE. To follow and measure the effectiveness of treatment is also required. Although the principals of lupus management have not changed, novel immune modulators, biological therapy and non-medical treatments (e.g. stem-cell transplantation) have become available. Further research and clinical observations may help to find the real place of such therapeutics.]

Lege Artis Medicinae

[RARE ASSOCIATION OF HODGKIN’S LYMPHOMA, GRAVES’ DISEASE AND MYASTHENIA GRAVIS]

RESS Zsuzsa, MEKKEL Gabriella, ILLÉS Árpád

[INTRODUCTION - In some cases other diseases associate with Hodgkin’s lymphoma, when it is diagnosed or relapses. Association of Hodgkin’s lymphoma with Graves’ disease and myasthenia gravis in one patient has not yet been reported in the literature. CASE REPORT - We report on a young female patient who had suffered from Hodgkin’s lymphoma since 1996. He had received polychemotherapy and mantle field irradiation previously. After treatment, complete remission was stated in 2000. Then she was treated because of Graves’ disease. In 2001 she complained of dysarthria, dysphagia, ptosis and diplopia. Thorough examinations proved myasthenia gravis. Considering the progression plasmapheresis was administered several times with cyclophosphamide and intravenous immunglobulin, besides conservative therapy. Recently she is euthyroid state, Hodgkin’s disease is in remission and her only complaint is dysarthria. CONCLUSION - The importance of this case on one hand is the rare association of these diseases, on the other is that Graves’ disease and myasthenia gravis occurred during in the remission of Hodgkin’s disease. Causal relation is not unambiguous but the role of disturbed immunregulation caused by Hodgkin’s lymphoma or the irradiation of the neck region can also contribute to it. The pure coincidental occurrence of Hodgkin lymphoma, Graves’ disease and myasthenia gravis is highly unlikely.]

Lege Artis Medicinae

[CLINICAL EXAMINATION OF ENZYME CATALASE AND ITS MUTATIONS IN HUNGARY]

GÓTH László

[Hydrogen peroxide is formed during physiological and pathological processes in the human body. Hydrogen peroxide in high concentration and its more reactive compound (hydroxyl radical) can damage cells. Recent findings show that hydrogen peroxide in low concentration is required for many physiological pathways of cell signaling such as diabetes, platelet activation, inflammation, immune response. In human tissues the steady state concentration of hydrogen peroxide changes between 0.1 nmol/l-100 micromol/l. The main regulator of hydrogen peroxide metabolism is the enzyme catalase. This enzyme has a fast and high activity on high hydrogen peroxide concentrations and it shows a weak effect on its physiologic concentration. Acatalasemia, the inherited deficiency of enzyme catalase seems to be rather a syndrome than a simple enzyme deficiency as it was thought earlier. It is associated with changes in lipid, carbohydrate and erythrocyte metabolisms. Contrary to the the worldwide (11 countries) distribution of acatalasemia only two syndrome causing catalase mutations have been reported from Japan. Recently, four novel catalase mutations detected in one acatalasemic and 12 hypocatalasemic Hungarian families which mutations are responsible for the decreased catalase activity.]

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Clinical Neuroscience

Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.

Clinical Neuroscience

[Advanced Parkinson’s disease characteristics in clinical practice: Results from the OBSERVE-PD study and sub-analysis of the Hungarian data]

TAKÁTS Annamária, ASCHERMANN Zsuzsanna, VÉCSEI László, KLIVÉNYI Péter, DÉZSI Lívia, ZÁDORI Dénes, VALIKOVICS Attila, VARANNAI Lajos, ONUK Koray, KINCZEL Beatrix, KOVÁCS Norbert

[The majority of patients with advanced Parkinson’s disease are treated at specialized movement disorder centers. Currently, there is no clear consensus on how to define the stages of Parkinson’s disease; the proportion of Parkinson’s patients with advanced Parkinson’s disease, the referral process, and the clinical features used to characterize advanced Parkinson’s disease are not well delineated. The primary objective of this observational study was to evaluate the proportion of Parkinson’s patients identified as advanced patients according to physician’s judgment in all participating movement disorder centers across the study. Here we evaluate the Hungarian subset of the participating patients. The study was conducted in a cross-sectional, non-interventional, multi-country, multi-center format in 18 countries. Data were collected during a single patient visit. Current Parkinson’s disease status was assessed with Unified Parkinson’s Disease Rating Scale (UPDRS) parts II, III, IV, and V (modified Hoehn and Yahr staging). Non-motor symptoms were assessed using the PD Non-motor Symptoms Scale (NMSS); quality of life was assessed with the PD 8-item Quality-of-Life Questionnaire (PDQ-8). Parkinson’s disease was classified as advanced versus non-advanced based on physician assessment and on questions developed by the Delphi method. Overall, 2627 patients with Parkinson’s disease from 126 sites were documented. In Hungary, 100 patients with Parkinson’s disease were documented in four movement disorder centers, and, according to the physician assessment, 50% of these patients had advanced Parkinson’s disease. Their mean scores showed significantly higher impairment in those with, versus without advanced Parkinson’s disease: UPDRS II (14.1 vs. 9.2), UPDRS IV Q32 (1.1 vs. 0.0) and Q39 (1.1 vs. 0.5), UPDRS V (2.8 vs. 2.0) and PDQ-8 (29.1 vs. 18.9). Physicians in Hungarian movement disorder centers assessed that half of the Parkinson’s patients had advanced disease, with worse motor and non-motor symptom severity and worse QoL than those without advanced Parkinson’s disease. Despite being classified as eligible for invasive/device-aided treatment, that treatment had not been initiated in 25% of these patients.]

Clinical Neuroscience

Evaluation of anxiety, depression and marital relationships in patients with migraine

DEMIR Fıgen Ulku, BOZKURT Oya

Aim - The aim of this study was to evaluate the frequency and characteristics of attacks in patients with migraine, to determine the effects of anxiety or depressive symptoms, and to evaluate the marital relationships of patients with migraine. Method - Thirty patients who were admitted to the neurology outpatient clinic of our hospital between July 2018 and October 2018 and were diagnosed with migraine according to the 2013 International Headache Society (IHS) diagnostic criteria were included in this cross-sectional study. Age, sex, headache frequency and severity, depressive traits, marital satisfaction and anxiety status were examined. We used the Beck Depression Inventory (BDI), State-Trait Anxiety Inventory (STAI), Maudsley Marital Questionnaire (MMQ) and Visual Analogue Scale (VAS) for measuring relevant parameters. Results - The mean severity of migraine pain according to VAS scale was 6.93 ± 1.41 and the mean number of migraine attacks was 4.50 ± 4.24. The mean BDI score of the patients was 12.66 ± 8.98, the mean MMQ-M score was 19.80 ± 12.52, the mean MMQ-S score was 13.20 ± 9.53, the mean STAI-state score was 39.93 ± 10.87 and the mean STAI-trait score was 45.73 ± 8.96. No significant correlation was found between age, number of migraine attacks, migraine duration, migraine headache intensity, and BDI, STAI and MMQ scores (p>0.05). But there was a positive correlation between MMQ-S and scores obtained from the BDI and STAI-state scales (p<0.05). Conclusion - In this study more than half of the migraine patients had mild, moderate or severe depression. A positive correlation was found between sexual dissatisfaction and scale scores of depression and anxiety.

Clinical Neuroscience

Investigation of risk factors, topographic location and stroke mechanisms of unilateral isolated and posterior cerebral ARTERY thalamic infarcts

GÖKCAL Elif, SENGUL Yildizhan, USLU Ilgen Ferda

Aim - In this study, we aimed to examine the risk factors, topographic features and stroke mechanisms of acute ischemic unilateral infarcts of thalamus. Methods - Patient with isolated thalamic infarct and those with posterior cerebral artery (PCA) infarction who were admitted to our hospital between January 2014 and January 2017 with acute unilateral thalamic infarction (TI) were included in this study (isolated thalamic infarction/ isolated TI; thalamic and posterior cerebral artery infarction/PCA+TI). Demographic characteristics and vascular risk factors of the patients were determined. Thalamic infarct areas were recorded topographically as anterior, posteromedial, ventrolateral, posterolateral, more than one area, and variant areas. Stroke mechanism was determined according to the criteria of „Trial of Org 10172 in Acute Stroke Treatment” (TOAST). Patients with isolated TI and PCA TI were compared according to risk factors, stroke mechanism and infarct topography. Results - Forty-three patients with a mean age of 63.3 ± 14.5 years were included in the study. Twenty-eight patients (60.1%) were found to have isolated TI and the remaining 15 patients (34.9%) had PCA+TI. 32.1% of patients with isolated TI had sensory symptoms on presentation, and 60% of patients with PCA-TI had sensorimotor symptoms. The mean age, the mean score on National Institutes of Health Stroke Scale (NIHSS) and the mean frequency of atrial fibrillation were higher in PCA+TI patients than in isolated-TI patients (p: 0.04, p: 0.004, p: 0.02 respectively). 32.6% of the patients had ventrolateral, 30.2% had posteromedial involvement. Ventrolateral topography was seen in 46.7% of the PCA+TI patients, while posteromedial topography was seen in 39.3% of the isolated-TI patients. 53.6% of the isolated-TI had small vessel disease etiology, while 40% of the PCA+TI had cardioembolic etiology, and the other 40% had large artery atherosclerosis. Conclusion - Our study showed that the most ommon stroke mechanism in patients with thalamic infarction is the small vessel disease. Isolated TI and PCA+TI patients differ in terms of etiologic mechanism and infarct topography. Variant territorial involvement and multiple area involvements can be quite common in thalamic infarcts.

Clinical Neuroscience

[Decisional collisions between evidence and experience based medicine in care of people with epilepsy]

RAJNA Péter

[Background – Based on the literature and his long-term clinical practice the author stresses the main collisions of evidence and experience based medicine in the care of people with epilepsy. Purpose – To see, what are the professional decisions of high responsibility in the epilepsy-care, in whose the relevant clinical research is still lacking or does not give a satisfactory basis. Methods – Following the structure of the Hungarian Guideline the author points the critical situations and decisions. He explains also the causes of the dilemmas: the lack or uncertainty of evidences or the difficulty of scientific investigation of the situation. Results – There are some priorities of experience based medicine in the following areas: definition of epilepsy, classification of seizures, etiology – including genetic background –, role of precipitating and provoking factors. These are able to influence the complex diagnosis. In the pharmacotherapy the choice of the first drug and the optimal algorithm as well as the tasks during the care are also depends on personal experiences sometimes contradictory to the official recommendations. Same can occur in the choice of the non-pharmacological treatments and rehabilitation. Discussion and conclusion – Personal professional experiences (and interests of patients) must be obligatory accessories of evidence based attitude, but for achieving the optimal results, in some situations they replace the official recommendations. Therefore it is very important that the problematic patients do meet experts having necessary experiences and also professional responsibility to help in these decisions. ]