Lege Artis Medicinae

[Sudden death of a patient with purpura - post mortem recognized eosinophilic granulomatosis with polyangiitis]

DOBREAN Noémi, HAJNAL-PAPP Rozália, TUSA Magdolna, OROJÁN Iván, CSERNI Gábor

NOVEMBER 03, 2015

Lege Artis Medicinae - 2015;25(10)

[INTRODUCTION - Systemic diseases may sometimes be challenging because physicians do not think about synthesizing the parts to a single entity. CASE REPORT - A 49-year-old asthmatic female was admitted to hospital for the investigation of her cutaneous symptoms suggestive of vasculitis associated with diffuse joint complaints. The chest X-ray raised the possibility of pneumonia or neoplastic disease. Following an episode of chest pain relieved by a non-steroidal anti-inflammatory drug, she suddenly died. Her previous history included restrictive cardiomyopathy, insufficiency of both atrioventricular valves and long dating eosinophilia. Autopsy revealed a partly granulomatous eosinophilic inflammatory process in several organs, including the heart, the lungs, the kidneys, the colon and the pituitary gland. Retrospective collection of unknown anamnestic features and symptoms made possible to unify the pieces of information and symptoms to a single entity, the Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis, EGPA). CONCLUSIONS - Bronchial asthma seldom leads to death. It can rarely be part of the Churg-Strauss syndrome, of which the manifestation may be related to the administration of leukotriene antagonists also used in the presented case. These drugs may allow the withdrawal of systemic steroid therapy which is beneficial not only in the treatment of asthma but also of the syndrome. Lowering the dose of steroids may promote the development of the full blown pattern of the latter.]

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Lege Artis Medicinae

Psychoneuroimmunology and the embodied mind

SZABÓ Attila

One of the major philosophical aspirations in contemporary consciousness research is to find a framework of explanation that could successfully address the problem of mind-body relations. Descartes is often regarded as the father of dualism in modern philosophy of mind. Phenomenology and embodiment may dissolve the problem of dualism in the waters of the experiential features of the life-world and the subject. Recent findings in psychoneuroimmunology have shown that somato-psychic mechanisms exist through which bodily stimuli are translated into neuropsychological events resulting in alterations in certain behavioral patterns. These may as well include changes in the qualitative features of the lived body (Leib) resulting in an overall change in the subjective experience. The application of modern embodiment theories in life sciences has the potential to create a novel, fruitful and heuristic approach, which may help us unveil features of the “mind-body phenomenon” that have been hidden so far. In this paper, I will try to briefly outline a possible analytical framework on the grounds of classic - Husserlian and Merleau-Pontian - phenomenology and biomedical sciences.

Lege Artis Medicinae

[The patient centered paradigm]

KAPÓCS Gábor

Lege Artis Medicinae

[Current questions on Pneumococcus infections: cardiovascular complications, cardiotoxicity and new opportunities for prevention among elderly people]

LUDWIG Endre

[The respiratory tract infections, first of all severe pneumonia are associated with severe cardiovascular adverse events the significance of which that had not been recognised so far. Among the adverse events, the development of acute coronaria syndromes, heart failure, arrhythimias are the most important ones leading to significatly higher mortality even after several years. In the development of acute coronaria syndrome , the proinflammatory activites play the cenral role leading to the instability of coronaria plaques and plaque rupture associated with formation of platelet thrombi. The constriction of coronariae, peripherial vasodilatation and hypoxia also contribute to the deleterius complications. The role of pneumococci in this process seems to be especially significant since this pathogen exhibit a unique direct cardiotoxic effect, namely directly invades the myocardium and produce cardiotoxic substances. As a consequence, impairement of cardiomyocite function and contractility and formation of microscopic lesions in which later collagen deposition and long-term cardiac scarring can be detected. These pathological processes are developed despite of antibiotic treatment, consequently, vaccination against pneumococcal infection seems to be the only method for efficacious prevention of the myocardium damage and cardiovascular adverse events. According to the results of the CAPITA study, the conjugate polisaccharide vaccine decrease the number of pneumococcal pneumonia and invasive pneumococcal infections by 45 and 75% resp. in the elderly population. The recognition of the direct and indirect role of pneumococci in the development of early and late cardiovascluar adverse events gives a new aspect of the beneficial effect of efficacious vaccination.]

Lege Artis Medicinae

[Insulin pump therapy in type 1 diabetes in the light of efficacy, safety, and quality of life]

HIDVÉGI Tibor, SZEPESI Gábor, LUKÁCS Andrea, BARKAI László

[The level of glycaemic control necessary to achieve optimal outcomes in subjects with type 1 diabetes mellitus typically requires intensified insulin therapy using multiple daily injections or continuous subcutaneous insulin infusion (CSII). For CSII, the insulins of choice are the rapid-acting insulin analogues: insulin aspart, insulin lispro and insulin glulisine. The advantages of CSII over multiple daily injections in adult and paediatric populations with type 1 diabetes include superior glycaemic control, lower insulin requirements and better health-related quality of life/patient satisfaction. An association between CSII and reduced hypoglycaemic risk is more consistent in children/adolescents than in adults. The use of CSII is widely recommended in both adult and pediatric type 1 diabetes mellitus populations, but is limited in pregnant patients. The review also discusses the result of the “A non-interventional trial to observe the effect of the use of Accu Chek Combo device on quality of life and metabolic control“(COM-1101-HU 1.3).]

Lege Artis Medicinae

[The evaluation of statin adherence in patients from the village of Méhkerék]

HANYECZ Vince, DANI Győző, MÁRK László

[INTRODUCTION - One of the basic elements of cardiovascular prevention is lipid lowering therapy, the quality of which is indicated by the increasing rate of lipid target values achieved during recent years. Further improvement is needed and for this the enhancement of patient adherence to statin treatment is a possible way. PATIENTS AND METHODS - The prescription habits in 81 patients taking statins (41 atorvastatin and 40 rosuvastatin) from 392 randomly selected ones for another study were analysed from the database of general practitioner in the village called Méhkerék in Békés County, Hungary. RESULTS - The statin adherence of the patients was evaluated in two different ways: the estimation by the general practitioner and the determination of prescribed drug boxes during one year. There was no significant difference between the two methods. Similarly, no significant difference was found between the atorvastatin or rosuvastatin adherence. The reduction of total cholesterol was significantly greater in patients taking rosuvastatin (p=0.03). A significant correlation was found between the prescribed box numbers and the decrease of LDL- and total cholesterol. CONCLUSIONS - The patients’ adherence seems to be independent from the type of statin. For the evaluation of patient adherence the GP's simple estimation and determination of prescribed drug boxes during one year are equally appropriate. This suggests, that the GP would be able to select those patients whose education needs longer time. ]

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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.

Clinical Neuroscience

[The Comprehensive Aphasia Test in Hungarian]

ZAKARIÁS Lilla, RÓZSA Sándor, LUKÁCS Ágnes

[In this paper we present the Comprehensive Aphasia Test-Hungarian (CAT-H; Zakariás and Lukács, in preparation), an assessment tool newly adapted to Hungarian, currently under standardisation. The test is suitable for the assessment of an acquired language disorder, post-stroke aphasia. The aims of this paper are to present 1) the main characteristics of the test, its areas of application, and the process of the Hungarian adaptation and standardisation, 2) the first results from a sample of Hungarian people with aphasia and healthy controls. Ninety-nine people with aphasia, mostly with unilateral, left hemisphere stroke, and 19 neurologically intact control participants were administered the CAT-H. In addition, we developed a questionnaire assessing demographic and clinical information. The CAT-H consists of two parts, a Cognitive Screening Test and a Language Test. People with aphasia performed significantly worse than the control group in all language and almost all cognitive subtests of the CAT-H. Consistent with our expectations, the control group performed close to ceiling in all subtests, whereas people with aphasia exhibited great individual variability both in the language and the cognitive subtests. In addition, we found that age, time post-onset, and type of stroke were associated with cognitive and linguistic abilities measured by the CAT-H. Our results and our experiences clearly show that the CAT-H provides a comprehensive profile of a person’s impaired and intact language abilities and can be used to monitor language recovery as well as to screen for basic cognitive deficits in aphasia. We hope that the CAT-H will be a unique resource for rehabilitation professionals and aphasia researchers in aphasia assessment and diagnostics in Hungary. ]

Clinical Neuroscience

[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

Clinical Neuroscience

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

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

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.

Hypertension and nephrology

[Association between cyclothymic affective temperament and hypertension]

NEMCSIK János, BATTA Dóra, KŐRÖSI Beáta, RIHMER Zoltán

[Affective temperaments (cyclothymic, hypertymic, depressive, anxious, irritable) are stable parts of personality and after adolescent only their minor changes are detectable. Their connections with psychopathology is well-described; depressive temperament plays role in major depression, cyclothymic temperament in bipolar II disorder, while hyperthymic temperament in bipolar I disorder. Moreover, scientific data of the last decade suggest, that affective temperaments are also associated with somatic diseases. Cyclothymic temperament is supposed to have the closest connection with hypertension. The prevalence of hypertension is higher parallel with the presence of dominant cyclothymic affective temperament and in this condition the frequency of cardiovascular complications in hypertensive patients was also described to be higher. In chronic hypertensive patients cyclothymic temperament score is positively associated with systolic blood pressure and in women with the earlier development of hypertension. The background of these associations is probably based on the more prevalent presence of common risk factors (smoking, obesity, alcoholism) with more pronounced cyclothymic temperament. The scientific importance of the research of the associations of personality traits including affective temperaments with somatic disorders can help in the identification of higher risk patient subgroups.]