Lege Artis Medicinae

[An Interview with Internist János Iványi on the 60th Anniversary of his Graduation ]

NEMESÁNSZKY Elemér

FEBRUARY 20, 2009

Lege Artis Medicinae - 2009;19(02)

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[Asymptomatic hepatic giant haemangioma]

KOVÁCS GÁBOR, NYIKOS Orsolya, GERVAIN Judit

[INTRODUCTION - Haemangioma is the most common type of benign hepatic tumors. Haemangiomas are usually asymptomatic, except for those that exceed five centimeters in size and are called “giant” haemangiomas. Malignant transformation has not been noted and therefore only regular follow-up is recommended for smaller haemangiomas. Surgical intervention becomes necessary only if symptoms or complications occur. Nevertheless, atypical presentation can cause a significant differential diagnostic problem. CASE REPORT - The authors report the case of a 51-year old woman with a focal hepatic abnormality diagnosed during a routine abdominal ultrasound examination. Results of the subsequent computed tomography scan suggested a multiple hepatocellular carcinoma based on the morphological appearance. Following oncology consultation, she was admitted to our department for liver biopsy. Histology did not reveal any malignancy. This result together with the lack of symptoms and complaints and the normal laboratory test results shifted the likely diagnosis towards a benign haemangioma. Blood-pool scintigraphy confirmed this diagnosis. CONCLUSIONS - Haemangiomas are benign hepatic tumors. They are often diagnosed accidentally, by routine abdominal ultrasound examination. If an abdominal ultrasound raises suspicion of haemangioma, abdominal MRI scan or blood-pool scintigraphy is recommended to be performed in order to exclude malignancy for lesion sizes of <2 cm or >2 cm, respectively. The reported case is considered important because of the differential diagnostic problems the large size and the atypical presentation of the given haemangioma imposed. Ultrasound-guided liver biopsy via fine-needle aspiration has previously been contraindicated. This invasive procedure can be performed in patients with focal hepatic pathology if diagnosis cannot be established by non-invasive tests, especially if malignancy is suspected. It can be performed conditional on the localization of the tumor, the patient’s general condition, the laboratory results, and if there is a therapeutic consequence of the biopsy results.]

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[Treatment of acute pancreatitis, with special regard to pharmaceutical therapy]

DÖBRÖNTE Zoltán

[Treatment of acute pancreatitis is mainly supportive, including the correction of any factors causing or sustaining the disease process, efforts to limit complications, as well as treatment of complications. Pharmaceutical efforts to influence the pathophysiological events with protease inhibitors or by influencing the release of the pro-inflammatory cytokine cascade did not prove to be effective, so there is no known effective and specific drug therapy for clinical use. Adequate pain control is an important component of pharmaceutical management, and - although yet controversial - early antibiotic prophylaxis and effective antimicrobial treatment of the inflammatory complications (infected necrosis or fluid collection, SIRS, sepsis) have probably a determining role in the outcome of severe necrotizing pancreatitis. Carbapenems proved to be the most potent antibiotics. For the prevention of the not infrequent fungal superinfection in acute pancreatitis, early administration of fluconasole can also decrease mortality. Surgery is indicated in the first stage of infected necrosis and infected pancreatic and peripancreatic fluid collections. In certain patients with a high operative risk, endoscopic or percutaneous drainage with lavage can also be worth trying. Optimal conditions for the treatment of severe necrotizing pancreatitis, as well as adequate management of multiple organ failure can only be warranted at an intensive care unit. In the chemoprevention of pancreatitis complicating endoscopic retrograde cholangiopancreatography (ERCP), non-steroidal anti-inflammatory drugs promise a new therapeutic option. There are insufficient data about the beneficial effects of the protease inhibitor ulinastatin, and results with nitroglycerin are contradictory.]

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[Misunderstanding]

JÓJÁRT György

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[Deception of patients with scientifically not proven methods]

Lege Artis Medicinae

[Labiolingual lesions - important symptoms of a rare endocrine syndrome]

SALLAI Ágnes, HOSSZÚ ÉVA, GERGICS Péter, RÁCZ Károly, FEKETE György

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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

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEN Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

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To handle the HaNDL syndrome through a case: The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis

ÇOBAN Eda, TEKER Ruken Serap, SERİNDAĞ Helin, SAKALLI Nazan, SOYSAL Aysun

The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a rare entity. This disease has been related to migrainous headaches. It is a benign, self-limited disorder, which is characterized by fluctuating neurological symptoms and cerebrospinal fluid lymphocytosis. We describe a case of a 47 years old man with acute onset of headache and aphasia. Cerebrospinal fluid analysis revealed a lymphocytic pleocytosis (25 cells/μl, 100% lymphocytes). Electroencephalogram showed moderate slow rhythm in the left hemisphere, with temporoparietal predominance, and without epileptiform activity. His blood tests as well as magnetic resonance imaging (MRI) results were normal. With the diagnosis of HaNDL syndrome the patient was accepted in the Department of Neurology and discharged with full recovery.

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Isolated hypoglossal nerve palsy due to a jugular foramen schwannoma

ÖZTOP-CAKMAK Özgür, VANLI-YAVUZ Ebru, AYGÜN Serhat, BASTAN Birgül, EGEMEN Emrah, SOLAROGLU Ihsan, GURSOY-OZDEMIR Yesemin

Introduction – Although the involvement of the hypoglossal nerve together with other cranial nerves is common in several pathological conditions of the brain, particularly the brainstem, isolated hypoglossal nerve palsy is a rare condition and a diagnostic challenge. Case presentation – The presented patient arrived to the hospital with a history of slurred speech and an uncomfortable sensation on his tongue. Neurological examination showed left-sided hemiatrophy of the tongue with fasciculations and deviation towards the left side during protrusion. Based on the clinical and MRI findings, a diagnosis of hypoglossal nerve schwannoma was made. Discussion – Hypoglossal nerve palsy may arise from multiple causes such as trauma, infections, neoplasms, and endocrine, autoimmune and vascular pathologies. In our case, the isolated involvement of the hypoglossal nerve was at the skull base segment, where the damage to the hypoglossal nerve may occur mostly due to metastasis, nasopharyngeal carcinomas, nerve sheath tumors and glomus tumors. Conclusion – Because of the complexity of the region’s anatomy, the patient diagnosed with hypoglossal nerve schwannoma was referred for gamma knife radiosurgery.

Lege Artis Medicinae

[Vigilance for Drug Safety: the SCOPE Project – An Interview with Mick Foy, Pharmacovigilance Working Group Leader of the British Medicines Authority (MHRA) ]

CZIGLÉNYI Boglárka