Clinical Neuroscience

[Gliosarcoma of the pineal region with cerebellar metastasis: case illustration]

BANCZEROWSKI Péter, VAJDA János, BÁLINT Katalin, SIPOS László

JANUARY 30, 2012

Clinical Neuroscience - 2012;65(01-02)

[A very rare case of gliosarcoma of the pineal region with cerebellar metastasis is presented. A few cases of glioblastoma and fibrosarcoma have already been published however there was no reported case with gliosarcoma at the pineal region even with cerebellar metastases.]

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Clinical Neuroscience

[LETTER OF THE EDITOR IN CHIEF]

RAJNA Péter, TAJTI János

Clinical Neuroscience

[Depression in neuropsychiatric diseases]

HIDASI Zoltán, SALACZ Pál, CSIBRI Éva

[Depression is frequently observed together with neurological disorders. Moreover this connection is bidirectional in the case of several neurological disorders, as depression can be either a comorbide syndrome or also a risk factor of them. Neurobiological background of depression involves neuroanatomical structures, their interconnected networks, disturbances of neurotransmitters, neurohormonal, neuroimmunological and neurotrophic changes, genetic background. Disfunction of these systems also plays a role in the pathogenesis of comorbid depression of neurological disorders. Interactions and clinical aspects of biological factors involved in the pathogenesis of depression in dementias, Parkinson’s disease, cerebrovascular disorders and epilepsy are discussed further. Depression as a result of neurobiological factors responsible for both neurological and psychiatric consequencies of these disorders, are often atypical as a clinical manifestation, however chracteristic for the particular neurological disorder. Evaluation of the biological backgound and clinical features of depression in neurological disorders makes the complex neuropsychiatric approach of these disorders possible.]

Clinical Neuroscience

[The clinical relevance of 1p19q codeletion of oligodendrogliomas at the Department of Neurosurgery in Debrecen]

KLEKNER Álmos, FEKETE Gábor, RENCSI Márta, MÉHES Gábor, SZABÓ Péter, BOGNÁR László

[Object - To determine the clinical relevance of 1p19q codeletion in case of patients treated between 2006 and 2008 with oligodendroglial tumor at the Department of Neurosurgery, University of Debrecen. Questions - Beside the traditional morphological investigations, methods of rapidly developing molecular pathology are also available for routine diagnostic procedures. Numerous studies confirm that the codeletion of the 1p and 19q alleles has a clinical relevance regarding the sensitivity for chemotherapy. In this study the connection between the 1p19q codeletion and clinical parameters was tested to evaluate the prognostic role of this genetic alteration in neurosurgical patients. Methods, clinical data - In the present study experiences about the clinical relevance of 1p19 codeletion is summarized. Between 2006 and 2008, 28 patients with brain tumor containing oligodendroglial component was tested for 1p19q codeletion. The result of the analysis was compared with clinical data such as tumor localization, extent of resection, histological grade, presence of astrocyte component, time of first recurrence, age and gender. Furthermore, the potency of 1p19q codeletion as a prognostic factor for chemosensitivity by analyzing the data of patients who underwent different treatment protocols was also evaluated. Results - Our results suggest that 1p19q codeletion can be valued as a positive prognostic factor, which is concordant with the results available in the literature. We also found positive correlation with oligodendroglial component, recurrence free survival of grade III tumors, sensitivity to chemoand radiotherapy, and inverse correlation with histological grade and age was detected. Conclusion - Though the 1p19q codeletion is currently not the part of the routine patient management, based on our study we found it appropriate for clinical use as a prognostic factor, and its predictive role in establishing oncotherapy can be also discussed.]

Clinical Neuroscience

[The validation of the hungarian version of the Dimensional Yale-Brown Obsessive-Compulsive Scale]

HARSÁNYI András, CSIGÓ Katalin, RAJKAI Csaba, DÖME László, DEMETER Gyula, RACSMÁNY Mihály

[Background - The Obsessive-Compulsive disorder (OCD) has a complex phenotype, which can be summarized by using a few consistent and temporally stable symptom dimensions. The dimensional approach derived from the systematic factor analytic studies of OCD symptoms. In 2006, a new psychometric scale was created by M.C. Rosario-Campos and her colleague, the Dimensional Yale- Brown Obsessive-Compulsive Scale (DY-BOCS). This scale measures the presence and severity of obsessive-compulsive (OC) symptoms within six distinct dimensions. The Hungarian translation of the test and preliminary results were published in 2009. Purpose - The objective of this recent study was two folded: on one hand, our goal was to validate the Hungarian version of the DY-BOCS on a larger sample size. On the other hand, we wanted to publish our results gained by the Hungarian version of the test in English. Methods - We assessed 30 Hungarian patients diagnosed with OCD by DSM-IV. Reliability and validity of the expert and of the self-report were estimated. Results - Self-report and expert ratings were highly correlated. The global DY-BOCS score was well correlated with the total Yale-Brown Obsessive-Compulsive Scale score. The internal validity of the symptom dimensions and the global severity score were high. Divergent validity was also good. Conclusion - These results indicate that the Hungarian version of the DY-BOCS is a reliable and valid clinical tool.]

Clinical Neuroscience

[Mutation analysis of alpha-galactosidase A gene in Hungarian Fabry patients]

LÁSZLÓ Aranka, TÖRÖK László, RAFFAI Sarolta, TÖRÖK Éva, SALLAY Éva, ENDREFFY Emőke, MORVAI László, AMSTEL Ploos Van JK

[Aim was to detect the mutations of alpha-galactosidase A gene in two Hungarian Fabry patients. Methods - Mutation analysis was performed by polymerase chain reaction (PCR) sequencing of the seven exons and adjacent introns of the alpha-galactosidase A gene. Results - Case 1. (19 y. male patient) Mutation analysis was done for alpha-galactosidase gene, a missence mutation has been identified in the 5th exon, the aspartic acid at codon 266 has been substituted by a tyrosine (notation D266Y) due to a G-T transversion at position 10287 of the alpha GAL-A gene. Case 2. (28 y. male Fabry patient) The GAL-A mutation has been proven to be R220X mutation in exon 5 of the alpha-galactosidase A gene.]

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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

[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

[The connection between the socioeconomic status and stroke in Budapest]

VASTAGH Ildikó, SZŐCS Ildikó, OBERFRANK Ferenc, AJTAY András, BERECZKI Dániel

[The well-known gap bet­ween stroke mortality of Eastern and Western Euro­pean countries may reflect the effect of socioeconomic diffe­rences. Such a gap may be present between neighborhoods of different wealth within one city. We set forth to compare age distribution, incidence, case fatality, mortality, and risk factor profile of stroke patients of the poorest (District 8) and wealthiest (District 12) districts of Budapest. We synthesize the results of our former comparative epidemiological investigations focusing on the association of socioeconomic background and features of stroke in two districts of the capital city of Hungary. The “Budapest District 8–12 project” pointed out the younger age of stroke patients of the poorer district, and established that the prevalence of smoking, alcohol-consumption, and untreated hypertension is also higher in District 8. The “Six Years in Two Districts” project involving 4779 patients with a 10-year follow-up revealed higher incidence, case fatality and mortality of stroke in the less wealthy district. The younger patients of the poorer region show higher risk-factor prevalence, die younger and their fatality grows faster during long-term follow-up. The higher prevalence of risk factors and the higher fatality of the younger age groups in the socioeconomically deprived district reflect the higher vulnerability of the population in District 8. The missing link between poverty and stroke outcome seems to be lifestyle risk-factors and lack of adherence to primary preventive efforts. Public health campaigns on stroke prevention should focus on the young generation of socioeconomi­cally deprived neighborhoods. ]

Clinical Neuroscience

Neuroscience highlights: Main cell types underlying memory and spatial navigation

KRABOTH Zoltán, KÁLMÁN Bernadette

Interest in the hippocampal formation and its role in navigation and memory arose in the second part of the 20th century, at least in part due to the curious case of Henry G. Molaison, who underwent brain surgery for intractable epilepsy. The temporal association observed between the removal of his entorhinal cortex along with a significant part of hippocampus and the developing severe memory deficit inspired scientists to focus on these regions. The subsequent discovery of the so-called place cells in the hippocampus launched the description of many other functional cell types and neuronal networks throughout the Papez-circuit that has a key role in memory processes and spatial information coding (speed, head direction, border, grid, object-vector etc). Each of these cell types has its own unique characteristics, and together they form the so-called “Brain GPS”. The aim of this short survey is to highlight for practicing neurologists the types of cells and neuronal networks that represent the anatomical substrates and physiological correlates of pathological entities affecting the limbic system, especially in the temporal lobe. For that purpose, we survey early discoveries along with the most relevant neuroscience observations from the recent literature. By this brief survey, we highlight main cell types in the hippocampal formation, and describe their roles in spatial navigation and memory processes. In recent decades, an array of new and functionally unique neuron types has been recognized in the hippocampal formation, but likely more remain to be discovered. For a better understanding of the heterogeneous presentations of neurological disorders affecting this anatomical region, insights into the constantly evolving neuroscience behind may be helpful. The public health consequences of diseases that affect memory and spatial navigation are high, and grow as the population ages, prompting scientist to focus on further exploring this brain region.

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.