Lege Artis Medicinae

[Elevation of IgG antibodies against tissue transglutaminase as a diagnostic tool for coeliac disease in selective IgA deficiency]

KORPONAY-SZABÓ Ilma, DAHLBOM I, LAURILA K, KOSKINEN S, WOOLEY N, PARTANEN J, KOVÁCS JB, MÄKI M, HANSSON T

JUNE 22, 2004

Lege Artis Medicinae - 2004;14(06)

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Lege Artis Medicinae

[INCIDENCE RATES OF CHILDHOOD TYPE 1 DIABETES WITHIN EUROPE AND HUNGARY BASED ON EURODIAB DATA]

GYÜRÜS Éva, SOLTÉSZ Gyula

[Type 1 diabetes is generally believed to be be the result of an immune destruction of pancreatic ßcells in genetically susceptible individuals exposed to environmental risk factors. To study the epidemiology of childhood-onset type 1 diabetes mellitus in Europe, the EURODIAB collaborative group established in 1988 prospective geographicallydefined registers of new cases diagnosed under 15 years of age. The 10-year-old study shows a greater than 10-fold range in incidence rate of childhood diabetes in Europe. The standardised average annual incidence rate during the period 1989-1998 ranged from 3,6 cases per 100 000 per year in Macedonia to 43,9 cases per 100 000 per year in Finland. Combined data from all centres indicates that the annual rate of increase in incidence was 3,2% but in some central and eastern European countries it was higher. The age-group-specific rates of increase were 5% for children aged 0-4 years, 3,7% for 5-9 years, and 2,1% for 10-14 years, which shows that the highest rates of increase occurred in the youngest age group. The Hungarian Childhood Diabetes Registry has collected the data of all newly diagnosed children with type 1 diabetes aged 0-14 years since 1st January 1978. The standardised incidence rate during the period 1978-2002 was 8,6 cases per 100000 per year, the lowest in the youngest (0-4 yr), highest in the10-14-year-old-children. There was a linear increasing trend in incidence with the average rate of annual increase of 5,1%. Comparing our incidence rate with other European countries Hungary belongs to the medium-risk countries with similar age- and sex-specific incidence rates. The results of the EURODIAB study confirm a very wide range of incidence rates of childhood type 1 diabetes within Europe and show that the increase in incidence varies from country to country. Such variation seems to be unlikely to be explained by genetic differences, since Europeans (except some small populations) are more homogeneous compared with other populations of other continents. The rapid increase in incidence may be explained by changes in environmental factors.]

Lege Artis Medicinae

[CHARM-Alternative ]

MATOS Lajos

Lege Artis Medicinae

[A CASE OF PRIMARY HEPATIC AMYLOIDOSIS PRESENTING WITH GASTRIC BLEEDING]

STOGICZA Ágnes, GRABER Hedvig, SKALICZKI József, NÁDOR Katalin, MAGYAR Tamás

[INTRODUCTION - Amyloidosis - at an early stage - has no typical clinical findings, but severe weight loss, hepatomegaly and elevated serum alkaline phosphatase level can be clues to the diagnosis. CASE REPORT - We report a 66-year-old woman presenting at our unit with massive gastrointestinal bleeding. Gastroscopy was performed and a haemorragic ulcer was found. Before admission she had lost 20 kg-s in 6 months. The patient had hepatomegaly and markedly elevated serum alkaline phosphatase level. These findings suggested the presence of malignancy, but ultrasound, CT and MR examinations did not support this hypothesis. After liver biopsy the diagnosis of amyloidosis was proven. She had rapid downhill clinical course of gastric bleeding from an ulcer resulting in death. CONCLUSIONS - So far, no specific treatment exists for amyloidosis, but there have been promising results reported about liver transplantation and autologous stem-cell transplantation.]

Lege Artis Medicinae

[MEDICAL ANTHROPOLOGICAL APPROACH TO CHILDREN'S SURMISES ON SMOKING]

BAK Judit, PIKÓ Bettina

[INTRODUCTION - The aim of our study was to explore school children'’s surmises on smoking. The following questions were in the focus of our analysis: what concepts children have about smoking and smoking-related diseases before adolescence? Have they already tried smoking? METHODS - The study was conducted among 3rd, 4th and 5th year school children (N=128) in two towns of Békés County, namely in Békés and Köröstarcsa. The sample consisted of 57% males and 43% females. Regarding sampling we followed international studies with similar aims where samples of 9-11-year-old average children were thought to be ideal for such study purpose using the draw-and-write technique. RESULTS - Most respondents from the study have not tried smoking yet. On the other hand, there are great number of adults who smoke in children’s environments, in many times, both parents do. Despite these facts, children’s attitudes toward smoking is rather negative. Children'’ s opinions reflect many negative aspects of smoking: the health-damaging effect, the financial aspects, the negative effects for social and physical environment. CONCLUSIONS - Similar to previous international studies, children of our sample possess correct and comprehensive knowledge of the smoking-related health problems. Findings of our study provides a support to the need of a smoking prevention program for children in the age of their negative opinions of smoking and well before the peer group effect is getting significant.]

Lege Artis Medicinae

[On teaching, with personal overtones]

BODA Domokos

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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.

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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

[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 role of sleep in the relational memory processes ]

CSÁBI Eszter, ZÁMBÓ Ágnes, PROKECZ Lídia

[A growing body of evidence suggests that sleep plays an essential role in the consolidation of different memory systems, but less is known about the beneficial effect of sleep on relational memory processes and the recognition of emotional facial expressions, however, it is a fundamental cognitive skill in human everyday life. Thus, the study aims to investigate the effect of timing of learning and the role of sleep in relational memory processes. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. Our results suggest that the timing of learning and sleep plays an important role in the stabilizing process of memory representation to resist against forgetting.]

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.