Clinical Neuroscience

[Ferenc Obál Jr.]

BENEDEK György

MAY 10, 2005

Clinical Neuroscience - 2005;58(05-06)

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Clinical Neuroscience

[Frontotemporal dementia - Part I History, prevalence, clinical forms]

GALARIOTIS Vasilis, BÓDI Nikoletta, JANKA Zoltán, KÁLMÁN János

[The authors report a comprehensive publication consisting of three parts going into the details of history, prevalence, clinical forms, differential diagnosis, genetics, molecular pathomechanism, pathology, clinical diagnosis and treatment of frontotemporal dementia (FTD). The first part of the present review deals with history, prevalence and clinical forms of FTD. The prototypical FTD with circumscribed atrophy was first described by Arnold Pick; Alois Alzheimer found the intraneural inclusions in the patients’ brain. Later it was recognised that many patients had neither the atrophy nor the cellular changes, but genetic mutations have been identified. Frontotemporal dementia is a degenerative condition with unknown etiology in the frontal and anterior temporal lobes of the brain. It is a progressive neurobehavioral syndrome characterized by early decline in social interpersonal conduct, early impairment in the regulation of personal conduct, early emotional blunting, and early loss of insight. There are no reliable epidemiological studies on the prevalence of FTD, but it is well-accepted that FTD is a common cause for dementia before the age of 65 (it constitues approximately five percent of all irreversible dementias). The nomenclature of the FTD has been confusing and continues to be. Three major clinical syndromes can be identified: 1 frontal variant FTD (dementia of frontal type) in which changes in social behavior and personality predominate, 2. in semantic dementia (progressive fluent aphasia) there is a breakdown in the conceptual database which underlies language production and comprehension, 3. in progressive nonfluent aphasia the phonologic and syntactic components of language are affected. The authors report two cases, which can point to clinical symptoms and forms, and mention the problems of the differential diagnosis and therapy.]

Clinical Neuroscience

[Pathophysiology of restless leg syndrome and periodic leg movement disorder in view of the latest research findings]

KÖVES Péter, SZAKÁCS ZOLTÁN

[Both restless leg syndrome and periodic leg movement disorder have been classified as primary sleep disorders by the International Classification of Sleep Disorders. Considering the characteristic clinical symptoms, it is supposed that their pathomechanism involves the peripheral and central stimulusprocessing mechanisms of the nervous system as well as several elements of the motor system. During the last couple of years many new elements of the pathomechanism have been discovered, in particular the dysfunction of the postsynaptic dopamin receptors related to the iron metabolism of the central nervous system, the role of opiate receptors, and the involvement of subclinical small fiber neuropathy. Many of these findings have been incorporated into the diagnostic and treatment protocols used in the management of patients with restless leg syndrome or periodic leg movement disorder. Considering the rapidly increasing number of publications on their pathomechanism and the various fields it involves, the authors found it necessary to evaluate these data and to interpret their relationships within the frame of sleep-wake regulation.]

Clinical Neuroscience

[THE SELECTIVE COX-2 INHIBITOR MELOXICAM AND SALICYLATE THERAPY]

TÓTH Károly

[Although during treatment of arthrosis with meloxicam the level of thromboxan A2 decreases, thrombocyte functions are not affected. Meloxicam in therapeutic doses doesn’t increase the risk of haemorrhage. Previously it was suspected that coadministration of salicylates with certain other non-steroid antiinflammatory drugs (NSAIDs) will suppress the effect of salicylate. Van Ryn et al have proved that this is not the case with salicylate plus meloxicam therapy. It is hypothesized that meloxicam loosely binds to the cyclooxygenase-1 (COX-1) enzyme and salicylate can easily replace it.]

Clinical Neuroscience

[Primary prevention program of the Hungarian Spine Society - Part II Controlled prospective study of short term efficacy]

SOMHEGYI Annamária, TÓTH János, MAKSZIN Imre, GARDI Zsuzsa, FESZTHAMMER Artúrné, DARABOSNÉ Tim Irma, TÓTHNÉ Steinhausz Viktória, TÓTHNÉ Szabó Klára, VARGA Péter Pál

[Introduction - The primary prevention program of the Hungarian Spine Society is set around 12 test exercises that assess performance of postural muscles and then develops their strength and flexibility. By this the scheme aims to make the biomechanically correct use of the spinal column spontaneous. Subjects and methos - The application of the scheme in the frame of physical education in a primary school was investigated in a prospective controlled study in the school-year of 2001/2002. Participating children were aged 6 to 14. The intervention group (n=200) regularly did the posture correction exercises with their teacher's direction in physical education classes, while the control group (n=213) did not. The 12 test exercises of the posture correction scheme of the Hungarian Spine Society were tested by an independent physiotherapist at the beginning and at the end of the school-year in both groups. Results were analyzed by paired and unpaired t-tests and by a chi-square test. Results - At the end of the school-year the strength and flexibility of the postural muscles of the intervention group improved significantly compared both to their own performance (p<0.01) at the beginning of the school-year and to the control group. Test results of the control group were significantly (p<0.05) worse at the end of the school-year than their own results at beginning of the school-year, and were more significantly (p<0.01) worse than the test results of intervention group. Conclusion - The results of the controlled study confirm that regular use of the preventive exercises in physical education improves the strength and flexibility of postural muscles. In order to analyze the results concerning specific muscle groups or age groups a detailed study with age- and gender-matched controls will be necessary.]

Clinical Neuroscience

[A case history and diagnostical rewiev of primary cerebral angiitis]

CSÉPÁNY Tünde, KOLLÁR József, SIKULA Judit, MOLNÁR Mária, CSIBA László

[The authors present a case history of primary cerebral angiitis with four years of follow-up. The early diagnosis was based on typical clinical symptoms, brain MRI, intracerebral MRA and histology of sural nerve biopsy. Electroneurography suggested peripheral involvement, although the patient did not have clinical signs of peripheral neuropathy. Glucocorticoid and immunosuppressive treatment resulted in remission. The diagnostic difficulties of primary cerebral vasculitis are also summarized in the discussion.]

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VÉCSEI László

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[In memoriam Prof. Dr. Ferenc Garzuly (1937-2021)]

ILLÉS Zsolt, HAHN Katalin, KÁLMÁN Bernadette

[Ferenc Garzuly passed away after a long and productive life at the age of 84. He worked for almost 60 years at the Markusovszky University Teaching Hospital, where primarily led the laboratory of neuropathology and the department of neurology, but transferred to the department of pathology after his retirement. By authoring several books on rare diseases, he greatly enriched the case-based tea­ching approach in medicine. He described the Hun­garian type of transthyretin mutation causing the familial me­nin­go­cerebrovascular amyloidosis phenotype. The presentation of a special phenotype of Fabry disease associated with megadolichobasilar anomaly and a novel mutation in the alpha-galactosidase-A gene is also associated with his name. ]

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[Kidney transplantation in Hungary, 2010]

LANGER Róbert, TORONYI Éva

[Hungarian kidney transplantation has been established with three milestone operations. In 1902 Emerich Ullmann showed the technical feasibility of renal transplantation on dogs, and later the living donor transplant of András Németh in 1962 and the program starting operation of Ferenc Perner in 1973 already meant the real possibility for Hungarian patients. More than 5000 kidney transplantations were done since, and the operations are now made at the four university medical schools centers. In 2009 248 renal transplantations were done in our country (Budapest: 148, Szeged: 51, Pécs: 39, Debrecen: 34), from which 24 were living donor and nine combined kidney-pancreas cases. Despite the worsening financing situation in the health care system the numbers of transplantations are stable within a 15 year period, but this means a marked decrease in international comparison. In our country, the ratio of living donation is low, there is no paired donation, incompatible transplantation, the problems of hypersensitive patients are unresolved, and there is no old-for-old program. The solution to all of these problems could be joining to Eurotransplant, which is the definite wish of the transplant society based on the positive Slovenian and Croatian examples.]

Clinical Neuroscience

[Ferenc Garzuly: In labyrinth of diagnostics: studies for doctors and medical students]

HORVÁTH Boldizsár

Hungarian Radiology

[The role of static MR-urography in diagnostics of urinary obstruction]

KIS Zsuzsanna, FAZEKAS Péter, KULCSÁR Dániel, KÖTELES Márta, KOVÁCS Annamária, MAGYAR Klára

[INTRODUCTION - The idea and methodology of MRurography has just crystallized recently due to the development of technology. The traditional MRU technology means the strongly T2 weighted sequence, suitable for depicting stationary liquid spaces. Its use is independent of the functional status of the kidneys thus it is suitable for depicting dilatated ureters in case of nonfunctioning kidneys, too. Authors's aim was to define the role of sMRU in the diagnostics of obstructive urinary diseases. PATIENTS AND METHODS - 60 sMRU examinations were performed on 59 patients using a 1.5 T Siemens Symphony MR scanner in the CT-MR Laboratory of the Markhot Ferenc County Hospital between May 1, 2003 and October 31, 2005. The sMRU was performed with 2D T2 TSE sequence with angiographic character. In each case, the examinations were completed with conventional sequences in multiple planes. The role of gadolinium enhanced T1-weighted MR urography in the same diseases was not studied. In 7 cases, low-dose thin slice CT examination was performed to reveal the precise cause of obstruction. The examinations were preceded by abdominal ultrasound or intravenous urography (IVU). RESULTS - Out of the 60 sMRU examinations uretery dilatation were observed in 50 cases. In the background of obstruction, stone could be detected in 13 patients, dilatation was observed in 4 patients and MR did not indicate stone. In 7 cases with known neoplastic disease, associated urinary obstruction could be detected. In the background of obstruction primary neoplasm was found in 10 patients unknown prior the MR examination. Other benign obstruction occurred in 13 cases. Obstruction was not proved in 10 patients. In these cases the examination was justified by uncertain ultrasound findings together with abnormal renal function parameters. Follow up sMRU was performed in one patient. False diagnosis was established in two patients, the cause of dilatation was not found in one patient. CONCLUSIONS - The sMRU examination can provide more precise detection of the causes of severe urinary obstructions. It helps to define the level of obstruction in case of known malignant tumors. The grade of urinary dilatation could be also evaluated. The sMRU examination in conjunction with conventional sequences in multiple planes made possible to set up a correct therapy plan.]

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