Lege Artis Medicinae

[Dilemmas in the Research of Rare Diseases ]

VARGA Orsolya

APRIL 20, 2014

Lege Artis Medicinae - 2014;24(04)

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Lege Artis Medicinae

[Vaccine coverage of invasive and carried Streptococcus pneumoniae isolates in Hungary]

TÓTHPÁL Adrienn, LAUB Krisztina, KARDOS Szilvia, NAGY Károly, DOBAY Orsolya

[Vaccination against Streptococcus pneumoniae will be mandatory for infants and children from 1 July 2014 in Hungary. Since the introduction of the conjugate vaccine against Pneumococcus, the distribution of Pneumococcus serotypes has changed significantly. Surveillance data from the National Centre for Epidemiology show that certain vaccine-type pneumococcal serotypes have almost disappeared owing to the immunization program, while other serotypes have emerged. Nowadays serotype 3 and the multiresistant 19A serotype are the most frequent causes oif invasive infections. A study analysing the nasal carriage of children attending kindergartens also confirmed the decline of vaccine- serotypes parallel to vaccination coverage and the simultaneous emergence of previously very types, such as 19A. The most recent study examining nasal carriage of children attending nursery (age < 3 years) showed that the 13-valent conjugate vaccine has efficiently decreased the carriage of serotypes 3 and 19A, verifying that the immunisation program should be continued using the vaccine providing the broadest coverage against Pneumococcus.]

Lege Artis Medicinae

[Diagnostic challenges in our adolescent patient with lymphoma]

MAGYARI Ferenc, RAJNAI Hajnalka, BARNA Sándor, MILTÉNYI Zsófia, VÁRÓCZY László, CSOMOR Judit, UDVARDY Miklós, ILLÉS Árpád

[OBJECTIVES - Unclassifiable B-cell lymphoma, which shows intermediate features typical for both diffuse, large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma (HL) is a novel category of diffuse, large B-cell lymphomas (DLBCL/HL), described in the WHO classification in 2008. This rare type of lymphomas presents peculiar clinical, morphological and immunophenotypical patterns, previously called gray-zone lymphomas. CASE REPORT - In December 2011 a 17- year old boy was diagnosed with mixedcellularity subtype of classical HL on the basis of left inguinal lymph node biopsy. Staging examinations revealed a IV/BXS (abdominal bulky) stage disease with unfavourable prognosis. Because of the unusally extended disease (nodal-extranodalbulky), a histological revision was performed. After a half course of ABVD chemotherapy the patient’s symptoms disappeared and the sizes of the involved lymph nodes decreased. On the basis of the histological revision, the diagnosis was changed to DLBCL/HL, so the treatment was modified to R-CHOP-14 regimen. After 3 cycles of R-CHOP-14 a complete metabolic remission (CMR) was achieved, which was confirmed by a 18FDG-PET/CT scan. Staging examinations after further 4 cycles of RCHOP- 14 therapy showed that the patient was still in CMR, but a PET-negative large mass (7×3 centimeter) still remained visible in the abdominal region. Considering this residual tumour and the agressive subtype of lymphoma the patient was referred for an autologous haemopoietic stem cell transplantation (AHSCT). After 2 cycles of R-DHAP regimen, successful CD34- positive stem cell collection was performed in August 2012. In September 2012, a RLAM-BEAM conditioning was performed followed by AHSCT. Posttransplantation 18FDG-PET/CT scan revealed further morphological regression, no symptom of the underlying disease appeared and the patient is in complete remission for 15 months. CONCLUSIONS - This case exemplifies that in case of atypical clinical findings and unusual progress of the disease it might be worthwile to re-evaluate the case and the (histological) diagnosis, which requires a close cooperation between the clinician and the pathologist.]

Lege Artis Medicinae

[The Cultural History of the Discovery of Insulin ]

FELKAI Péter

Lege Artis Medicinae

[Fulvestrant therapy resulting long progression free survival in case of symptomatic, metastatis breast cancer]

KÓSZÓ Renáta, KAHÁN Zsuzsanna

Lege Artis Medicinae

[Neuroethics ]

SZEBIK Imre

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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

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

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEN Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

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[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.]

Clinical Neuroscience

Acute effect of sphenopalatine ganglion block with lidocaine in a patient with SUNCT

KOCATÜRK Mehtap, KOCATÜRK Özcan

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing/short-lasting unilateral neuralgiform headache with cranial autonomic features (SUNCT/SUNA) is a rare severe headache. At the time of an attack, it can hinder a patient from eating and requires acute intervention. The sphenopalatine ganglion is an extracranial parasympathetic ganglion with both sensory and autonomic fibers. Sphenopalatine ganglion block has long been used in the treatment of headache, particularly when conventional methods have failed. Here, we present a patient who was resistant to intravenous lidocaine, but responded rapidly to sphenopalatine ganglion block during an acute episode of SUNCT/SUNA.