Lege Artis Medicinae

[The Adventures of Haydn’s Skull]

GERLINGER Imre

JANUARY 27, 2009

Lege Artis Medicinae - 2009;19(01)

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[Saving Lives at the Cost of the Physician’s Life?]

BARANYAI Zsolt

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[The Truths of August the Dumb – Clown Doctors]

GYIMESI Ágnes

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[Health and Anthropology – Foucault’s Reading of Kant]

KELEMEN Gábor

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[The lost humanness]

FRENKL Róbert

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[Importance of statin therapy in stroke prevention]

KÁPOSZTA Zoltán, RÁCZ Klára

[Stroke is the third leading cause of death and a leading cause of major adult disability in developed countries. The annual incidence of hospitalized stroke varies between 400-500 per 100 000 inhabitants every year in Hungary. In the past decade, cholesterol lowering with 3- hydroxy-3-methylglutaryl-coenzyme A (HMGCoA) reductase inhibitors (statins) has proved to reduce risk of stroke in patients with and without coronary disease (CAD). In patients with CAD, statin therapy reduces the risk of first stroke by 25% to 35% versus placebo and, moreover, intensive statin therapy to LDL-C targets below 2.6 mmol/L (100 mg/dL) appears to reduce the risk further. More recently it has also been shown that intensive statin therapy can reduce risk of recurrent stroke in nondiabetic as well as diabetic patients with recent stroke or transient ischaemic attack but no CAD. The overall reduction of stroke and TIA was 23%. Evidence from retrospective studies suggests that in addition to risk reduction statin pretreatment may improve stroke outcome. It may due to their pleiotropic effects that include improvement of endothelium function, anti-inflammatory, antithrombotic, and immunomodulatory effects. As statins have both an excellent safety profile and simple administration, physicians should consider using statins, at dosages shown to have efficacy in clinical trials, in all patients whose cardiovascular risk profile puts them at high risk of stroke.]

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Clinical Neuroscience

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.

Hungarian Immunology

[Ocular myositis]

KISS Emese, FACSKÓ Andrea, DÉVÉNYI Katalin, DANKÓ Katalin, ZEHER Margit

[INTRODUCTION - Dermato-/polymyositis is an autoimmune disorder, which belongs to the idiopathic inflammatory myopaties. It involves skeletal muscles in form of weakness and inflammatory infiltrates. Characteristic skin lesions are present in dermatomyositis. Other organs may also be affected mainly in the presence of myositis specific autoantibodies. The inflammation usually involves the proximal muscles of extremities. CASE REPORT - In the present work we report the case of a 52-year-old woman. In the previous history the removal of rectal adenocarcinoma was remarkable in 1994. After that she received chemotherapy. She complied for severe headache and pain in the right eye in 2000 October, therefore a skull CT was performed, indicating thickening of rectus medalis muscle within orbital cavity. There was an enhancement of contrast material in the muscle. Glaucoma was excluded. Neurologist suspected the presence of myositis and indicated 0.5 mg/kg corticosteroid therapy. Soon after the left eye became painful, but due to the corticosteroid treatment both eyes became painless. A control orbital CT was completely negative in 2000 November. Immunology consultancy revealed a mild proximal muscle atrophy in both lower extremities, but CPK and LDH enzyme levels were normal, EMG was characteristic for mild chronic nerve lesion. The biopsy, taken from the involved proximal muscle of lower extremity, did not show inflammatory infiltration. Complete screening for cancer was negative. Thyroid gland disease could be excluded. Immune laboratory data were negative, autoantibodies, including anti-Jo1, could not be detected. Based on the results a rare disease, ocular myositis was diagnosed. Considering the clinical improvement, the withdrawal of corticosteroid therapy was offered. Stringent immunology and oncology follow-up is required. CONCLUSION - In relation to our case report, we discuss clinical symptoms of orbital myositis, diagnostic procedures to identify the disease and also differential diagnostic considerations.]

Clinical Neuroscience

[Atlas of Microsurgery of the Lateral Skull Base]

VERES Róbert és munkatársa

Clinical Neuroscience

[The first identified Central-Eastern European patient with genetically confirmed dentatorubral-pallidoluysian atrophy]

ZÁDORI Dénes, TÁNCZOS Tímea, JAKAB Katalin, VÉCSEI László, KLIVÉNYI Péter

[Aims - Dentatorubral-pallidoluysian atrophy (DRPLA) is an inherited neurodegenerative disorder characterized by a trinucleotide repeat expansion. The disease mainly occurs amongst the Japanese and is extremely rare in the European population. The characteristic clinical symptoms are cerebellar ataxia, dementia, choreoathetoid movements, epileptic seizures and myoclonus. The aim of this study is to present the first genetically confirmed Hungarian case of DRPLA. Case report - The middle-aged female patient developed the characteristic clinical symptoms except myoclonus over her late thirties with positive family history. The major finding in the skull magnetic resonance imaging was the atrophy of infratentorial brain structures with the consequential dilation of related cerebrospinal fluid spaces. A detailed neuropsychological examination was also performed and it revealed moderate cognitive dysfunctions, mild depression and anxiety. As underlying conditions, Huntington’s disease and common spinocerebellar ataxia forms all came into consideration, but all the result of the respective genetic tests were negative. However, the test for mutation in the ATN1 gene revealed pathological heterozygous CAG repeat expansion. Conclusion - This case study serves as the first description of genetically confirmed DRPLA in the Central-Eastern region of Europe, the clinical features of which seems to be very similar to the previously reported cases.]

Clinical Neuroscience

[INITIAL STAGES OF ANTHROPOLOGY IN HUNGARY]

PÁSZTOR Emil

[In the second half of the 19th century anthropological researches started everywhere in the world. Cranioscopy formed an important part of the biological anthropology. József Lenhossék (1818-1888) worked also on this subject and on the basis of one of his researches in 1875 he became the founder of the anthropology in Hungary. On 76 skulls of several collections and on 265 heads together with his coworkers he performed 50 measurements on each skulls and heads and calculated the important ratios (skull-indexes). He determined the skull-indexes of the Hungarian people. These indexes are valid also today.]