Clinical Neuroscience

[Intracranial propagation of invasive aspergilloma in an immuncompromised patient]

SIMON Márta, VASTAGH Ildikó, VÁRALLYAY György, TURÁNYI Eszter, SRÉTER Lídia, CZIRJÁK Sándor, BERECZKI Dániel

MAY 30, 2010

Clinical Neuroscience - 2010;63(05-06)

[Aspergillus infection of the central nervous system is a rare disease, occasionally seen among immunocompromised patients. The most frequent pathway is hematogenic dissemination. Less known is the direct propagation from the paranasal sinuses, which is usually observed in immunocompetent patients. We report a patient who developed cavernous sinus syndrome due to an invasive intracranial aspergilloma after longlasting chemo- and steroid therapy for chronic lymphoid leukemia and immunhemolytic anemia. The characteristic features seen on radiological images - brain CT and MRI - suggested the possibility of invasive aspergilloma. Postoperative histology defined the diagnosis. Our case review highlights the importance of considering the possibility of an invasive opportunistic infection of the CNS in an immunocompromised patient presenting a new neurological sign.]

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Clinical Neuroscience

[Neurology! Adieau? (Part 1)]

SZIRMAI Imre

[The neurological practice suffered considerable changes during the last twenty years. The recent therapeutic methods and the acceptance of the ideology of evidence based medicine, which is based on confidence in statistics, changed the reasoning of the neurologists. Therapy protocols intrude into the field of individual medicine, and doctors accept treatment schemes to alleviate responsibility of their decisions. In contrast with this, recent achievements in pharmacogenetics emphasize the importance of individual drug therapies. The protocol of intravenous cerebral thrombolysis does not require defining the origin of cerebral ischaemia in the acute stage, therefore, this procedure can be regarded as human experiment. According to the strict protocol thrombolysis might be indicated only in 1-8% of patients with cerebral ischaemia. According to the Cohrane database more trials are needed to clarify which patients are most likely to benefit from treatment. Because of the change in therapeutic principles transient ischaemic attack has been newly defined as “acute neurovascular syndrome”. Multiplication of neurological subspecialties has been facilitated by the development of diagnostic tools and the discovery of effective new drugs. The specialization led to narrowing of interest and competency of clinicians. Several new neurological scientific societies were founded for the representation of specific disorders. In Hungary, between 1993 and 2000 nine scientific societies were grounded within the field of clinical neurology. These societies should be thankful to the pharmaceutical industries for their existence. In some European countries in 2007 only three neurological subspecialties were accepted, which are neurophysiology, neuro-rehabilitation and childneurology. Neuro-radiology is in the hands of general radiologists, the specialization is not granted for neurologists. Because of the subspecialization the general professionalism of neurologists has diminished. Among young neurologists the propedeutic skills suffered most seriously. Subspecialisation of teachers also interferes with the practice oriented teaching of medical students and residents.]

Clinical Neuroscience

[Neurology! Adieau? (Part 2)]

SZIRMAI Imre

[Teaching of neurologists is indisposed worldwide. University tutors are engaged in teaching, research and patient-care. This triple challenge is very demanding, and results in permanent insecurity of University employees. To compensate for the insufficient clinical training, some institutes in the USA employ academic staff members exclusively for teaching. The formation of new subspecialties hinders the education and training of general neurologists. At the present four generations of medical doctors are working together in the hospitals. The two older generations educate the younger neurologists who have been brought up in the world of limitless network of sterile information. Therefore their manual skills at the bedside and knowledge regarding emergency treatment are deficient. Demographics of medical doctors changed drastically. Twice as many women are working in neurology and psychiatry than men. Integrity of neurology is threatened by: 1. Separation of the cerebrovascular diseases from general neurology. Development of "stroke units" was facilitated by the better reimbursement for treatment and the interest of the pharmaceutical companies. The healthcare politics assisted to split the neurology into two parts. The independent status of “stroke departments” will reduce the rest of clinical neurology to outpatient service. 2. The main argumentation to segregate the rare neurological diseases was that their research will provide benefit for the diseases with high prevalence. This argumentation can rather be considered territorial imperative. The separation of rare diseases interferes with the teaching of differential diagnostics during neurological training. The traditional pragmatic neurology can not be retrieved. The faculty of neurology could retain its integrity because of the improvement of diagnostic methods and the more and more effective drugs. Nevertheless, even the progression of neurological sciences induces dissociation of clinical neurology. Neurology shall suffer fragmentation if the professional authorities fail to control the separation of subspecialties, if teaching of future neurologists, including practical knowledge and skills of diagnostic decision making, is not supported.]

Clinical Neuroscience

[Image fusion guided brachytherapy of brain tumors]

JULOW Jenő

[The paper is the summary of mostly published works of the clinical results of intracavitary Yttrium-90 colloid irradiation of recurrent cystic craniopharyngiomas, and Iodine-125 interstitial irradiation of gliomas, pinealomas, brainstem tumors, recurrent meningeomas, solid craniopharyngiomas and metastases. It concisely demonstrates the usefulness of image fusion in the verification of isotope seeds and catheters, the comparison of 125 Iodine stereotactic brachytherapy and LINAC radiosurgery modalities on physical dose distribution and radiobiological efficacy, and the analysis of volumetric changes after interstitial irradiation of gliomas. Results of the immunohistochemical study deal with the role of microglia/macrophage system in the tissue response to I- 125 interstitial brachytherapy of cerebral gliomas. Due to financial reasons, gamma knife and Linac are not available to many countries and neurosurgical institutes. In the absence of the above mentioned radiosurgical methods, we have shown brachytherapy as an alternative solution in the treatment of different types of inoperable or recurrent brain tumors. The observed results may be noticable at LINAC and gamma knife irradiation too.]

Clinical Neuroscience

[Clinical studies on pramipexol retard]

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[Pramipexol retard is the newest drug for the treatment of Parkinson’s disease. The prolonged release of the agent in this preparation allows a more continuous dopaminergic stimulation than previous preparations, without reducing the agent’s already known and proven clinical efficiency. In addition, it has a more favourable adverse effect profile than previous preparations, and patient compliance can also be better as it needs to be taken only once daily. These benefits have been proven in recent clinical studies, of which the most important ones are reviewed here.]

Clinical Neuroscience

[Epidemiology of dementia in Hungary]

ÉRSEK Katalin, KÁRPÁTI Krisztián, KOVÁCS Tibor, CSILLIK Gabriella, GULÁCSI L. Ádám, GULÁCSI László

[Objective - To estimate the epidemiology and the distribution of disease severity of dementia in Hungary, using published data. To estimate the demented population of 2008 and to make a projection for 2050. Methodology - With an outlook for the international professional literature and the available Hungarian information we examine the epidemiology of dementia in Hungary by age-groups and disease severity (according to MMSE categories), then make our estimation for the entire population. Results - Based on the estimation of the number of demented people in Hungary there is a noticeable difference between the domestic and the internationally published data. According to previous Hungarian studies, the number of the demented subjects vary between 530 and 917 thousand patients. Multiplying the elderly age-group’s populations by the global prevalence data it results in 101 thousand of demented patients. Estimation by the domestic published data we remarkably overestimate the presumed value, whereas by using the global prevalence figures we underestimate. Conclusions - There is a strong need for a representative study to obtain exact figures on the prevalence of dementia in Hungary. Getting exact figures of the Hungarian prevalence of dementia it is a strong need an overall representative study. With the lack of it the health and social care systems are not able to prepare for providing the increasing number of patients.]

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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

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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

[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

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Lege Artis Medicinae

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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.]