Lege Artis Medicinae

[Regulations of patients’ rights in Hungary in the view of international directions]

MOLNÁR Angéla

SEPTEMBER 10, 2001

Lege Artis Medicinae - 2001;11(08-09)

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Lege Artis Medicinae

[Anti-atherosclerotic effects of Ca-antagonists in the light of new clinical data]

ALFÖLDI Sándor

[Based on in vitro and animal research, it has been found that calcium antagonist drugs are capable of inhibiting directly the atherosclerotic process. In spite of their advantegous antiatherosclerotic effect, the fast-release, first-generation dihydropyridines had an unfortunate effect on the number of newly developed myocardial infarctions and on cardiovascular mortality. New, controlled clinical studies have found long acting calcium antagonists to decrease significantly the progression of the atherosclerosis in the carotid artery - verified with B-mode ultrasound scans - and the appearance of cardiovascular clinical events, when either proven coronary sclerosis (PREVENT, CAPARES studies) or hypertension (INSIGHT, ELSA, VHAS) was present. If further clinical trials (CAMELOT, NORMALISE - now under way) justify these promising clinical data, then long-acting calcium channel blockers may become new and significant tools in the prevention of the progression and clinical complications of atherosclerotic disease.]

Lege Artis Medicinae

[Congress of ASH, 2001.]

ALFÖLDI Sándor

Lege Artis Medicinae

[Endothel dysfunction and hypertension]

KELEMEN Judit, BLASKÓ György

[In the past two decade numerous data has been collected about the role of endothelium in the development of several cardiovascular disorders i.e. hypertension, congestive heart failure and atherosclerosis. Endothelial cells had been thought to be passive barriers only, but it turned out that through paracrine and autocrine hormone secretion they take part in modulating and regulating the vasodilator and vasoconstrictor effects being directed to vascular smooth muscle cells. The intact endothelium prevents the adhesion of platelets and monocytes, the platelet aggregation, as well as the migration and proliferation of vascular smooth muscle cells. It has been shown that both in experimental and human hypertension the endothelial function i.e. the so-called endothel-dependent vasodilatation is damaged, being the main feature of endothelial dysfunction. In spite of extensive research it is not clear whether endothelial dysfunction is a cause or a consequence of hypertension, with exact pathomechanism being also unclear. Methods, by which this important parameter could be precisely measured are under development. Researchers also examine whether recently used antihypertensive agents could improve or eliminate endothelial dysfunction and whether this effect may offer benefit to patients in terms of morbidity and mortality. This article attempts to summarize the most up-to-date information about the endothelial dysfunction research.]

Lege Artis Medicinae

[Risk to the fetus in the light of new data]

BODA Domokos

[Extensive research efforts based on principles of evidence-based medicine have helped lately to accumulate large amount of information on the risks to the fetus during pregnancy. As a result, the standpoints concerning risks to the fetus have become more precise in many respects. A number of earlier assumptions have been proven to be exaggerated or unfounded and the range of cases at risk has been narrowed down. This paper presents the most important data on potential risks to the fetus originating from hereditary factors, infections, maternal diseases, drugs, irradiation and special gestational anomalies. The data presented highlight the necessity of identifying the fetal damage during the pregnancy unequivocally, based exclusively on evidence of probative force and with realistic appraisal of the danger of any existent fetal damage.]

Lege Artis Medicinae

[Wegener’s granulomatosis: diagnostic questions]

CSISZÉR Eszter, SOLTÉSZ Ibolya, FÜZESI Katalin

[INTRODUCTION - Wegener’s granulomatosis is a disease of unknown origin classified as an immune vasculitis. The main pathologic feature is necrotising granulomatous small vessel vasculitis. Clinically the upper and/or lower airways are affected most often as well as the kidneys in the generalised form. The highly specific antineutrophil cytoplasmic antibodies have diagnostic significance beside the pathology and clinical symptoms. PATIENTS AND METHODS - We have studied the clinical and diagnostic characteristics of 15 cases diagnosed in our centre from the last 25 years. All patients had pulmonary manifestation. We analysed the organ involvement, clinical signs indicating the diagnosis, chest radiography, chances for biopsies of diagnostic significance and the difficulties of differential diagnostics. RESULTS - The diagnosis was made based on histological samples from the upper airways in three cases and from the lower airways in six cases (in four cases the bronchoscopic biopsies were diagnostic, in two patients thoracotomy was necessary). In six cases, clinical signs and typical organ manifestations were the basis of the diagnosis. CONCLUSION - This very rarely occurring disease which is difficult to recognise requires pulmonological examination due to the involvement of lungs. If this diagnosis comes to mind - based on findings of bilateral, multiplex, round pulmonal shadows with cavitation - the pulmonologist should search for other organ manifestations and involve respective specialists. Ideally, diagnosis is established by histopathology or by positive cytoplasmic antineutrophil cytoplasmic antibodies.]

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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.

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Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

Clinical Neuroscience

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

Evaluation of the effectiveness of transforaminal epidural steroid injection in far lateral lumbar disc herniations

EVRAN Sevket, KATAR Salim

Far lateral lumbar disc herniations (FLDH) consist approximately 0.7-12% of all lumbar disc herniations. Compared to the more common central and paramedian lumbar disc herniations, they cause more severe and persistent radicular pain due to direct compression of the nerve root and dorsal root ganglion. In patients who do not respond to conservative treatments such as medical treatment and physical therapy, and have not developed neurological deficits, it is difficult to decide on surgical treatment because of the nerve root damage and spinal instability risk due to disruption of facet joint integrity. In this study, we aimed to evaluate the effect of transforaminal epidural steroid injection (TFESI) on the improvement of both pain control and functional capacity in patients with FLDH. A total of 37 patients who had radicular pain caused by far lateral disc herniation which is visible in their lumbar magnetic resonance imaging (MRI) scan, had no neurological deficit and did not respond to conservative treatment, were included the study. TFESI was applied to patients by preganglionic approach. Pre-treatment Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI) scores of the patients were compared with the 3rd week, 3rd month and 6th month scores after the procedure. The mean initial VAS score was 8.63 ± 0.55, while it was 3.84 ± 1.66, 5.09 ± 0.85, 4.56 ± 1.66 at the 3rd week, 3rd month and 6th month controls, respectively. This decrease in the VAS score was found statistically significant (p = 0.001). ODI score with baseline mean value of 52.38 ± 6.84 was found to be 18.56 ± 4.95 at the 3rd week, 37.41 ± 14.1 at the 3rd month and 34.88 ± 14.33 at the 6th month. This downtrend of pa­tient’s ODI scores was found statistically significant (p = 0.001). This study has demonstrated that TFESI is an effective method for gaining increased functional capacity and pain control in the treatment of patients who are not suitable for surgical treatment with radicular complaints due to far lateral lumbar disc hernia.

Clinical Neuroscience

Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: A prospective study

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Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. This study comprised 30 autoimmune MG patients and 30 healthy volunteers. Autonomic tests including sympathetic skin response (SSR) and R-R interval variation analysis (RRIV) was carried out. The tests were performed two times for patients who were under acetylcholinesterase inhibitors during the current assessment. The RRIV rise during hyperventilation was better (p=0.006) and Valsalva ratio (p=0.039) was lower in control group. The SSR amplitudes were lower thereafter drug intake (p=0.030). As much as time went by after drug administration prolonged SSR latencies were obtained (p=0.043).Valsalva ratio was lower in the AchR antibody negative group (p=0.033). The findings showed that both ocular/generalized MG patients have a subclinical parasympathetic abnormality prominent in the AchR antibody negative group and pyridostigmine has a peripheral sympathetic cholinergic noncumulative effect.