Lege Artis Medicinae

[Impact of mometasone furoate nasal spray on the ocular symptoms of allergic rhinitis - Short presentation of a meta-analysis]

TAMÁS László

JANUARY 20, 2012

Lege Artis Medicinae - 2012;22(01)

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Lege Artis Medicinae

[Pilomatrix carcinoma with a lymph node metastasis - First Hungarian publication of a rare case]

VAJDA Adrienne, LÉVAY Bernadett

[INTRODUCTION - Pilomatrix carcinoma is a very rare malignant tumour, which derives from hair matrix cells. The male:female ratio among patients is 4:1, the mean age of patients is 45 years. This tumour type is very aggressive and grows slowly. CASE REPORT - The authors describe the case of a 37-year-old man who presented in 2004 with an approximately 2×2 cm, compact lesion tumour in the lumbar region. The tumour was excised and diagnosed as carcinoma sebaceum on the basis of histological examination. Several weeks after surgery, a protruding, semi-spherical lesion with a diameter of 2 cm appeared in the scar. The patient was referred to our hospital for excision of this lesion and for assessing the possibility of Muir-Torre syndrome. The examinations did not indicate the presence of any internal tumours. The recurrent tumour was excised with a 2 cm intact margin. Histological examination confirmed the diagnosis of pilomatrix carcinoma. In 2005, two new tumours were excised and semithick skin was transplanted in several sessions. At an oncological follow-up examination in 2009, a 13×7 mm lymph node was felt in the righ inguinal region, which was found to be a metastasis of pilomatrix carcinoma on the basis of aspiration cytology. Surgical block dissection was performed, followed by postoperative radiotherapy at a dose of 25×2 Gy. At present, the patient is symptom- free. Thoracic-abdominal CT and clinical examination performed in early June 2011 did not indicate progression of the original disease. CONCLUSION - Pilomatrix carcinoma with a lymph node metastasis has not been previously reported in Hungarian. The diagnosis was established on the basis of histological examination, which was indispensable for successful treatment.]

Lege Artis Medicinae

[Genetic background of thyroid cancers]

HALÁSZLAKI Csaba, LAKATOS Péter, KÓSA P. János, BALLA Bernadett, JÁRAY Balázs, TAKÁCS István

[Molecular genetics has become an indispensable diagnostic tool in a number of diseases. The most frequent thyroid tumours are associated with genetic alterations that might be used for diagnostic purpose in the future. Somatic mutations and rearrangements in BRAF, RAS family RET/PTC and PAX8/PPAR-gamma genes may occur in papillary and follicular thyroid carcinomas. Other mutations of the RET gene can be found in medullary carcinomas (in sporadic as well as hereditary types), whereas mutations of the genes TP53, RAS, and BRAF are associated with poorly differentiated and anaplastic carcinomas. At present, the most reliable diagnostic tool for the differential diagnosis of thyroid nodules is fine-needle aspiration cytology. However, the existing malignancy cannot be unequivocally proven in up to 10-40% of all samples. On the basis of previous results, genetic examination of fine needle aspiration samples from thyroid nodules can contribute to a more precise diagnosis and the timely removal of potentially malignant nodules.]

Lege Artis Medicinae

[How do physicians sleep and dream?]

SUSÁNSZKY Éva, SZÁNTÓ Zsuzsa

[INTRODUCTION - Satisfying sleep is especially important for physicians. Our study analyses physicians’ sleep and dream from the point of view of continuous nightand- day duty. SAMPLE AND METHOD - Questionnaires were completed by 125 physicians among whom the proportion of night shift taking and only day-time working persons was equal. The questionnaire contained the Athens Insomnia Scale and the Dream Quality Questionnaire as well as questions about demographical characteristics and work circumstances. RESULTS - Almost each doctor mentioned sleep problems, principally daytime sleepiness (78%) and sleep deprivation (70%). Long sleep latency is reported more often by women doctors; the frequency of night awakenings increases, while daytime sleepiness decreases by age. The feeling of performance-loss is more prevalent among night shift takers. Dream characteristics differ significantly neither along demographical characteristics nor by work shifts. CONCLUSION - Although sleep problems are more frequent among physicians when comparing to the Hungarian general population, the frequency of clinical level insomnia is not higher. On the other hand, physicians can recall their dreams more often (25% vs 7%) and the emotional load of their dreams influence their daytime mood more commonly.]

Lege Artis Medicinae

[Playing with Passion]

PINTÉRNÉ Ambrusz Aliz, CSIKY-MÉSZÁROS Mária

Lege Artis Medicinae

[Surrealistic Art from the Perspective of Psychoanalysis]

GYIMESI Anna

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Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.

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

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

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.