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Clinical Neuroscience

JULY 30, 2020

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.

Clinical Neuroscience

JULY 30, 2020

Extraskeletal, intradural, non-metastatic Ewing’s sarcoma. Case report

OTTÓFFY Gábor, KOMÁROMY Hedvig

Intracranial localization of Ewing’s sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing’s sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re­gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima­ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.

Clinical Neuroscience

JANUARY 30, 2016

Comparison of hospitalized acute stroke patients’ characteristics using two large central-eastern european databases

ORBÁN-KIS Károly, SZŐCS Ildikó, FEKETE Klára, MIHÁLKA László, CSIBA László, BERECZKI Dániel, SZATMÁRI Szabolcs

Objectives – Stroke is the third leading cause of death in the European region. In spite of a decreasing trend, stroke related mortality remains higher in Hungary and Romania when compared to the EU average. This might be due to higher incidence, increased severity or even less effective care. Methods – In this study we used two large, hospital based databases from Targu Mures (Romania) and Debrecen (Hungary) to compare not only the demographic characteristics of stroke patients from these countries but also the risk factors, as well as stroke severity and short term outcome. Results – The gender related distribution of patients was similar to those found in the European Survey, whereas the mean age of patients at stroke onset was similar in the two countries but lower by four years. Although the length of hospital stay was significantly different in the two countries it was still much shorter (about half) than in most reports from western European countries. The overall fatality rate in both databases, regardless of gender was comparable to averages from Europe and other countries. In both countries we found a high number of risk factors, frequently overlapping. The prevalence of risk factors (hypertension, smoking, hyperlipidaemia) was higher than those reported in other countries, which can explain the high ratio of recurring stroke. Discussion – In summary, the comparatively analyzed data from the two large databases showed several similarities, especially regarding the high number of modifiable risk factors, and as such further effort is needed regarding primary prevention.

Clinical Neuroscience

NOVEMBER 20, 2015

[The diagnostic and management challenges for posterior reversible leucoencephalopathy syndrome]

KILIC COBAN Eda, GEZ Sedat, KARA Batuhan, SOYSAL Aysun

[Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological entity characterized by epileptic seizures, headaches, altered mental status and focal neurological signs. Hypertension is the second most common condition associated with PRES. The 50-year-old-male patient with right-sided hemiparesis and speech disturbances admitted to our clinic. His blood pressure at the emergency service was 220/140 mmHg. A left putaminal hematoma was seen in his CT and MRI. In his brain MRI, FLAIR and T2 –weighted sequences showed bilateral symmetric diffuse hyperintensities in the brain stem, basal ganglia, and occipital, parietal, frontal, and temporal lobes. After the intense antihypertensive drug treatment, his blood pressure came to normal limits within a week. During his hospitalisation he had a recurrent speech disturbance lasting an hour. His electroencephalography was normal. In his repeated diffusion weighted MRI, an acute lacunary infarct was seen on right centrum semiovale. Two months later, the control MRI showed only the previous lacuner infarcts and the chronic putaminal hematoma. We presented a case developping either a cerebral hemorrhage or a lacunar infarction due to PRES. The main reason of the following complications of the disease was delayed diagnosis. Uncontrolled hypertension was guilted for the events. ]

Clinical Neuroscience

NOVEMBER 20, 2015

[A rare complication of a rare disease; stroke due to relapsing polychondritis]

KILIC COBAN Eda, XANMEMMEDOV Elimir, COLAK Melek, SOYSAL Aysun

[Relapsing polychondritis (RP) is an episodic and progressive inflammatory disease of cartilaginous structures. Its diagnosis is based primarily on clinical features such as laboratory parameters, biopsy. Neurological complications occur in 3% of the cases and are classified as an important cause of death. The cranial nerve disorders are most common but hemiplegia, ataxia, myelitis, polyneuritis, seizures, confusion, hallucination and headache can also happen. The aetiology of central nervous system involvement is still unknown. Moreover stroke has rarely reported in these patients. The diagnosis of stroke is challenging because of its rarity among these patients. Perhaps vasculitis is the common underlying mechanism. Also meningitis and encephalitis can occur during the course of RP. A 44 year-old woman was admitted with uncontemplated left hemiparesis, redness, swelling, and tenderness of the metacarpophalangeal and interphalangeal joints of the right hand and the cartilaginous portion. White blood cell count, C-reactive protein and the erythrocyte sedimentation rate were elevated. Vasculitis biomarkers were normal in our patient. Carotid and vertebral artery doppler ultrasonography, cranial and cervical MR Angiography were normal. Echocardiography showed a mild mitral valve prolapse and regurgitation. Our patient had the history of auricular polychondritis but she had not been diagnosed. Hemiparesis was her first neurological manifestation that led her to doctors for diagnosis. Our patient fulfilled the criteria of RP so no biopsy was needed. She was treated with oral prednisolone (80 mg/day) and aspirin (300 mg/day) and now she is on 10 mg prednisolone and 150 mg azathioprine. Two months later her physical and neurological symptoms returned to normal.]

Lege Artis Medicinae

APRIL 18, 2020

[What is worth to know about COVID-19 for (not only) a cardiologist]

HEPP Tamás, CSÉKE Balázs, BENCZÚR Béla

[SARS-CoV-2 virus infection sprang from Wuhan the capital of the Chinese Hubei province, at the end of 2019 and caused a worldwide pandemic with 1.5 million confirmed cases and claimed almost 100 000 victims until the beginning of April, 2020. First analyses of Chinese COVID-patients confirmed that diabetes, hypertension, and cardiovascular diseases were highly prevalent among SARS-CoV2 infected patients, and might be associated with poor outcome. As previously shown for SARS-CoV-1, SARS-CoV-2 similarly utilizes ACE2 as receptor for viral alveolar cell entry. A suspicion has arisen that the widely used ACE-inhibitor/ARB therapy could be potentially harmful for patients suffering from COVID-19 infection as these agents upregulate the ACE2-expressions. From the other point RAAS-blockade might be beneficial due to fact that ACE2 counters the deleterious effects of Angiotensin II. Authors provide a comprehensive over­view of the most recent literature and summarize the link between COVID-19 and car­diovascular disease. It is important to em­phasize that there are no available hu­man evidences confirming if the RAAS-in­hi­bitor therapy were harmful or helpful in pa­tients suffering from COVID-19.]

Clinical Neuroscience

MARCH 30, 2020

[New applications of conventional EEG analysis ]

CLEMENS Béla, PUSKÁS Szilvia

[Neurophysiological research suggests that the so-called “standard” EEG analysis has been confronted with new diagnostic challenges. The findings mainly concern the occurrence, the neurophysiological and clinical significance of epileptiform EEG discharges in several neurological and psychiatric disorders. In addition to well-known interictal and ictal discharges, a growing number of recently recognized epileptiform phenomena have been described. The first reports suggested that they might be relevant for the comprehensive description of epileptic dysfunction and might contribute to diagnosis and treatment as well. However, considerable improvement of present-day “standard” EEG technique is necessary to give an appropriate answer to most challenges. Reliable registration and quantitative assessment of well-known epileptiform transients require extended electrode coverage of the head (high-density EEG) and long-term recordings including waking and sleep states to estimate frequency and dyna­mics of targeted activities. Computer-based automatic event detection is preferable to spare time and cost of the evaluation. The authors review recent progress concerning epidemiology, neurophysiology and clinical impact of well-known epileptiform transients and candidate epileptiform activities in neurological and psychiatric conditions. However, recent results need confirmation in large patient populations; therefore, research should not be restricted to a few central laboratories.]

Clinical Neuroscience

MARCH 30, 2020

CANOMAD syndrome with respiratory failure

SALAMON András, DÉZSI Lívia, RADICS Bence, VARGA Tímea Edina, HORTOBÁGYI Tibor, TÖMÖSVÁRI Adrienn, VÉCSEI László, KLIVÉNYI Péter, RAJDA Cecília

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

Lege Artis Medicinae

JANUARY 20, 2020

[End of the line? Addenda to the health and social care career of psychiatric patients living in Hungary’s asylums]

KAPÓCS Gábor, BACSÁK Dániel

[The authors are focusing on a special type of long term psychiatric care taking place in Hungary outside of the conventional mental health care system, by introducing some institutional aspects of the not well known world of so called social homes for psychiatric patients (asylums). After reviewing several caracteristics of institutional development of psychiatric care in Hun­gary based on selected Hungarian and in­ternational historical sources, the main struc­tural data of present Hungarian institutional capacities of psychiatric health and social care services are shown. Finally, the authors based on own personal experiences describe several functional ascpects of the largest existing asylum in EU, a so­cial home for long term care of psychiatric pa­tients. By the beginning of the 20th century, Hungarian psychiatric institutions were operating on an infrastructure of three large mental hospitals standing alone and several psychiatric wards incorporated into hospitals. Nevertheless, at the very first session of the Psychiatrists’ Conference held in 1900 many professionals gave warning: mental institutions were overcrowded and the quality of care provided in psychiatric hospital wards, many of which located in the countryside of Hungary, in most cases was far from what would have been professionally acceptable. The solution was seen in the building of new independent mental hospitals and the introduction of a family nursing institution already established in Western Europe; only the latter measure was implemented in the first half of the 20th century but with great success. However, as a result of the socio-political-economic-ideological turn following the Second World War, the institution of family nursing was dismantled while different types of psychiatric care facilities were developed, such as institutionalised hospital and outpatient care. In the meantime, a new type of institution emerged in the 1950s: the social home for psychiatric pa­tients, which provided care for approximately the same number of chronic psychiatric patients nationwide as the number of functioning hospital beds for acute psychiatric patients. This have not changed significantly since, while so­cial homes for psychiatric patients are perhaps less visible to the professional and lay public nowadays, altough their operational conditions are deteriorating of late years. Data show, that for historical reasons the current sys­tem of inpatient psychiatric care is proportionately arranged between health care and social care institutions; each covering one third. Further research is needed to fully explore and understand the current challenges that the system of psychiatric care social- and health care institu­tions are facing. An in-depth analysis would significantly contribute to the comprehensive improvement of the quality of services and the quality of lives of patients, their relatives and the health- and social care professionals who support them. ]

Clinical Oncology

APRIL 10, 2019

[Current views on the male breast cancer]

BAKI Márta

[Breast cancer in men is a rare disease, and accounts for only 1% of all diagnosed breast cancers. Hungarian incidence by available data much higher. The greatest risk factor of male breast cancer the elevated estrogen concentration in the body. Genetic disorders, as a Klinefelter syndrome and estrogen exposures and other metabolic changes might cause the male breast cancer. Symptom duration is longer than female population and the male breast cancers diagnosed in older ages and advanced stages. Frequency of BRCA2 mutation is probably 10% among male patients. The most common type is invasive ductal carcinoma with estrogen and progesterone receptor positivity. Diagnostic, surgical, radiation procedures and chemotherapy probably same as female breast cancer. The guidelines recommend as in adjuvant and curative setting the tamoxifen and other selective estrogen receptor modulators treatment. By large nation based registry the survival rate is different from male and female breast cancers. New biomarkers, genetic changes are under investigation to understand munch better the male breast cancer.]