Lege Artis Medicinae

[Variation pleases - but to whom?]

FORGÁCS Iván

JULY 20, 2006

Lege Artis Medicinae - 2006;16(07)

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Lege Artis Medicinae

[Compounds – Plants...]

GRÉTSY Zsombor

Lege Artis Medicinae

[WEGENER’S GRANULOMATOSIS PRESENTING AS MASTOIDITIS: A DIAGNOSTIC CHALLENGE]

NAGY Pál, SZABÓ László, DOMJÁN Gyula, GADÓ Klára, BALOGH Károly

[INTRODUCTION - Wegener's granulomatosis has an uncertain pathomechanism, but is probably autoimmune in origin. In typical cases the mucosa of the nose, paranasal sinuses and of the lower respiratory tract, as well as the lungs and the kidneys are affected. Patients present with sinusitis, recurrent pneumonia or renal disease associated with microhaematuria, pyuria or azotaemia. Fever, polyarthralgia or polyarthritis may also occur. The underlying pathologic changes are necrotizing vasculitis, granulomas and parenchymal necrosis. The diagnosis is based on a combination of the clinical picture, microscopic findings and immunofluorescent demonstration of cANCA. CASE REPORT - A 27-year-old woman presented with symptoms of unilateral mastoiditis. In the following 7 months she underwent 7 operations in 4 hospitals for a locally progressive, destructive process of uncertain etiology showing a septic course. The clinical picture was not specific, the cANCA test was not definitive, and the histologic findings were initially interpreted as nonspecific inflammation. Repeated biopsies, multiple reviews of the microscopic specimens, consultations, differential diagnostic considerations, and, finally, the success of the treatment with corticosteroids and cyclophosphamide led to the diagnosis of Wegener’s granulomatosis. Currently the patient has been in remission for 32 months. CONCLUSION - The definitive diagnosis of Wegener’s granulomatosis, particularly of its localized or limited form, may be problematic despite well-defined diagnostic criteria. Setting up the correct diagnosis may take months or years. In case of unusual respiratory or otological symptoms, and in view of ineffective medical or surgical treatment, Wegener’s granulomatosis has to be considered. An atypical clinical picture, inconclusive histologic, radiologic and laboratory findings warrant the need for close collaboration of various specialists. This is particularly important since state-of-the-art therapy of Wegener’s granulomatosis promises a favourable prognosis.]

Lege Artis Medicinae

[Interdisciplinary forum of MOTESZ - Budapest, 23-25th March, 2006.]

KRAMER Imre

Lege Artis Medicinae

[Odour]

SZIGETI Zoltán

Lege Artis Medicinae

[CONTROVERSIAL ISSUES IN THE TREATMENT OF OPERABLE CERVICAL CANCER]

PETE Imre

[Wertheim hysterectomy as a baseline surgical treatment for patients with FIGO stage 1a2-2a cervical cancer was introduced in 1989 at the National Institute of Oncology, Budapest, Hungary. From then until 2002 when the results were first evaluated 308 such operations were performed. The average 5- and 10-year survival rates were 80% and 75%, respectively. Although these results are comparable to literature data, several issues concerning this group of patients remain to be solved. The participation rate in the Pap test screening programme covered by the national health insurance is only 30%. There is no national database on the current treatment of these patients that could help identify the necessary steps to be taken in order to improve the results. The number of radical hysterectomies performed a year in Hungary, or their outcome, is not known. The treatment scheme varies among the institutes and so does surgical experience. There is no consent regarding treatment policy, preoperative irradiation or chemotherapy, surgical technique, or postoperative management. The improvement of the participation rate in the screening programme, the use of prognostic factors to determine the most appropriate treatment, the role of minimally invasive and fertility-preserving surgery, the preservation of ovaries and the use of neoadjuvant chemotherapy are some of the further issues that need to be discussed. The first step should be data acquisition from all institutes in the country in order to develop and apply uniform treatment guidelines.]

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[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.

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

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.