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Clinical Neuroscience

JANUARY 30, 2014

[Dostoyevsky’s epilepsy in the light of recent neurobiological data]

TÉNYI Dalma, RAJNA Péter, JANSZKY József, HORVÁTH Zsuzsanna, TÉNYI Tamás, GYIMESI Csilla

[Background and purpose -Since the 1960s several theories have developed on the epilepsy of Fyodor Mikhailovich Dostoyevsky. Probably the most exciting and still actual question might be the subject of the „ecstatic aura”, he described in his novels based on his own experiences. During this extremely rare seizure onset the patients experience a strong sense of happiness, harmony and wholeness. The symptomatogenic zone of ecstatic seizures were considered to be of temporal lobe origin for a long time. Lately though this theory seems to be questioned based on the results of SPECT and deep brain EEG monitoring techniques in addition to the enrichment of our knowledge concerning the function of the insular cortex. Methods - Literary and scientific overview on the subject of Dostoyevsky’s epilepsy, with special concern to his ecstatic seizures. Results and conclusion - According to new electrophysiology and imaging techniques ecstatic seizures - including the seizure onset of Dostoyevsky - could rather be connected to the insular cortex.]

Lege Artis Medicinae

DECEMBER 18, 2013

[New potential perspectives of statin therapy]

CSÁSZÁR Albert

[Statins have become crucial components of the therapy of cardiovascular diseases. Beyond their cholesterol-lowering effect, statins turned out to have further beneficial effects on various vascular mechanisms. One of the best known effects is antithrombotic capacity, which is related partly to platelet function and partly to the coagulation cascade. Besides experimental observations, interventional clinical trials have also demonstrated that statins have an antithrombotic effect both in arterial and venous thrombosis. Regarding the effects of statins on dementia, previous studies with relatively small sample sizes had controversial results. Recently, two observational studies of tens of thousands of elderly patients reported that statins reduce the incidence of nonvascular dementia. Evaluation of the data revealed that statins have pleiotropic effects in this case, too. The results discussed here shed light on new benefits of statin therapy used for reducing cardiovascular mortality, namely the prevention of thrombotic events and dementia. These benefits are related to the antithrombotic and anti-inflammatoric capacity of statins.]

LAM Extra for General Practicioners

DECEMBER 17, 2013

[NEW POTENTIAL PERSPECTIVES OF STATIN THERAPY]

CSÁSZÁR Albert

[Statins have become crucial components of the therapy of cardiovascular diseases. Beyond their cholesterol-lowering effect, statins turned out to have further beneficial effects on various vascular mechanisms. One of the best known effects is antithrombotic capacity, which is related partly to platelet function and partly to the coagulation cascade. Besides experimental observations, interventional clinical trials have also demonstrated that statins have an antithrombotic effect both in arterial and venous thrombosis. Regarding the effects of statins on dementia, previous studies with relatively small sample sizes had controversial results. Recently, two observational studies of tens of thousands of elderly patients reported that statins reduce the incidence of nonvascular dementia. Evaluation of the data revealed that statins have pleiotropic effects in this case, too. The results discussed here shed light on new benefits of statin therapy used for reducing cardiovascular mortality, namely the prevention of thrombotic events and dementia. These benefits are related to the antithrombotic and anti-inflammatoric capacity of statins.]

Clinical Neuroscience

NOVEMBER 30, 2013

[Treatment possibilities in advanced Parkinson’s disease]

TAKÁTS Annamária, NAGY Helga, RADICS Péter, TÓTH Adrián, GERTRÚD Tamás

[In the course of Parkinson’s disease, advanced and late stages can be distinguished. In the advanced stage, levodopa has good effect on motor symptoms, but patient care is often hindered by levodopa-induced complications such as motor fluctuation and dyskinesias. In the late stage levodopa response becomes poor, falls, dementia and psychotic symptoms appear and patients often need hospitalization. In the advanced stage, the quality of life may be improved better by device-aided therapy than by best oral medical treatment. The alternatives are apomorhin pump, levodopa carbidopa intestinal gel with pump and deep brain stimulation. The therapy plan should be based on the principle: “the right treatment, to the right patient, in the right time”.]

Clinical Neuroscience

NOVEMBER 30, 2013

[Reversible hepatocerebral degeneration-like syndrome due to portovenous shunts]

SIBEL Güler, UFUK Utku, AHMET Tezel, ERCÜMENT Ünlü

[Ataxia and tremor are rare manifestations of hepatocerebral degeneration due to portovenous shunts. Ammonia is a neurotoxin that plays a significant role in the pathogenesis of hepatic encephalopathy. A 58-year old male patient was assessed with the complaints of gait disturbance, hand tremor, and impairment of speech. His neurological examination revealed dysarthric speech and ataxic gait. Bilateral kinetic tremor was noted, and deep tendon reflexes of the patient were hyperactive. Serum ammonia level was found to be 156.9 μg/dL. Cranial magnetic resonance (MR) imaging revealed increased signal intensity in bilateral globus pallidus on T1-weighted axial sections, and bilateral prominent hyperintense lesions in the middle cerebellar peduncles on T2-weighted axial sections. On his abdominal MR portography, multiple portohepatic venous collaterals were noted in the right and left lobes of liver parenchyma in 2D FIESTA axial MR sections. To our knowledge, we reported the first case of acquired hepatocerebral degeneration presenting with cerebral symptoms without any hepatic findings in which clinical improvement was noted, and hyperammonemia disappeared following medical treatment.]

Hypertension and nephrology

OCTOBER 20, 2013

[Therapeutic apheresis in pediatry]

TÚRI Sándor, BERECKI Csaba, HASZON Ibolya, PAPP Ferenc

[The possible mechanisms of therapeutic plasma mexchange: 1. the removal of circulatory plasma factor (anti Gbm disease, myasthenia gravis, Guillain Barré syndrome), 2. monoclonal antibody (Waldenström macroglobulinemia, myeloma protein), 3. circulatory immuncomplexes cryoglobulinaemia, myeloma protein, SLE), 4. alloantibody, 5. toxic factor, 6. replacement of a specific plasma factor, 7. a repear of the function of reticulo-endothelial system, 8. the removal of the inflammatory mediators, 9. the changes of the ratio of antigen-antibody which makes immuncomplexes more soluble, 10 stimulation of lymphocyte clones for supporting the cytotoxic therapy. Indications of emergency plasmapheresis: 1. Goodpasture syndrome with rapidly progressive glomerulonephritis and hemoptoe, 2. hyperviscosity syndrome, 3. TTP/HUS, 4. High level of factor VIII inhibitor, 5. respiratory insufficiency Guillain-Barré syndrome, 6. myasthenia gravis, 7. acute mushroom intoxication, or protein bound toxins. Further indications for plasmapheresis: 8. cryoglobulinemia, 9. other cases of rapidly progressive glomerulonephritis (when steroid+ cyclophosphamide are ineffective), 10. Wegener granulomatosis, 11. polyarteritis nodosa, 12. systemic lupus erythematosus (when steroid and cyclophosphamid therapy is not effective or associated with cerebral vasculitis, antiphospholipid syndrome combined with bleeding and thrombosis), 13. focal segmental glomerulosclerosis (resistant for therapy), 14. acute tubulointerstitial nephritis, 15. acute vascular rejection, 16. rheumatoid arthritis systemic type, 17. hypertrigliceridemia (≥25 mM), 18. thyreotoxic crisis, 19. acute necrotizing pancreatitis, 20. acute fulminant hepatitis, 21. paraquat intoxication, 22. snake bite (when antiserum is unavailable), 23. drug intoxication.]

Clinical Neuroscience

MARCH 30, 2013

[Efficacy of deep brain stimulation in our patients with Parkinson’s disease]

GERTRÚD Tamás, TAKÁTS Annamária, RADICS Péter, RÓZSA Ildikó, CSIBRI Éva, RUDAS Gábor, GOLOPENCZA Péter, ENTZ László, FABÓ Dániel, ERÕSS Loránd

[Background and purposes - In advanced Parkinson’s disease, medically refractory motor fluctuation or medically resistant tremor considerably affects quality of life. However, these symptoms can be mostly successfully treated by deep brain stimulation. We analyzed the efficacy of bilateral subthalamic stimulation in our patients with Parkinson’s disease. Methods - We assessed the clinical data of ten patients who have been treated in the Department of Neurology, Semmelweis University and have been operated in the National Institute of Neurosciences between 2008 and 2011. The Hoehn-Yahr scale score, the Unified Parkinson’s Disease Rating Scale score and the Parkinson’s Disease Questionnaire 39, as well as the dose of antiparkinson medication were documented prior to and one year after surgery. Results - Patient condition improved according to the Hoehn-Yahr scale, approximately by two stages. The dose of antiparkinson medication could be reduced by 63.4% (p=0.005) post operation. Unified Parkinson’s Disease Rating Scale scores decreased by 70.9% (p=0.005). 12 hours after medication withdrawal, execution of daily activity improved by 57.1% (p<0.01) and motor functions developed by 79.1% (p<0.01). Duration of dyskinesias decreased by 62.5% (p=0.018), duration of akinesia diminished by 87.5% (p=0.005). Quality of life rose by 41.6% (p<0.01). Neuropsychological tests detected improvement in verbal memory. Conclusion - With deep brain stimulation, the dosage of antiparkinson medication could be significantly reduced, with considerable improvements in motor function and quality of life. Although the number of patients is still low, good results have been established by careful patient selection, precise neurosurgical procedure and by appropriate programming and patient care.]

Hypertension and nephrology

DECEMBER 30, 2012

[Acute heart failure and acute renal injury: pathophysiology and management of cardiorenal syndrome type 1]

LÁSZLÓ Ágnes, ÁCS Tamás, JÁRAI Zoltán

[The functional connection between heart and kidney is well known. Several types of this relationship have been recently characterized as cardiorenal syndromes. The relevance of this relationship in clinical practice is supported by the fact, that the consequences of the primary dysfunction are profoundly influenced by the magnitude and the treatment possibilities of the secondary dysfunction. Moreover, the administered therapy for heart failure can deteriorate renal hemodinamics, or side effects of the treatment can lead to the worsening of the clinical picture. Loop diuretics decrease venous congestion, but also induce neurohormonal activation and a decrease in glomerular filtration rate. The body of positive evidence with the use of mineralocorticoid receptor antagonists in acute settings is limited. Inotropic agents on the one hand improve hemodinamics, on the other hand increase the danger of arrhythmia and mortality (levosimendan seems to be an exception). Aquaretics decrease symptoms without influencing mortality. The natriuretic peptide neseritid improved clinical symptoms, but did not improve endpoints in clinical trials. Vasodilators improve hemodinamics, but their usefulness is limited because of their profound hypotensive effect. The effectiveness and benefits of ultrafiltration has to be tested in more clinical trials. Because of such treatment difficulties the management of these patients is a complex task that needs the involvement of intensive therapeutic specialists, nephrologists and cardiologists. This review focuses on the pathophysiology and possible management of the patients with acute heart failure with acute kidney injury, called type 1 cardiorenal syndrome from the cardiologist’s point of view.]

Lege Artis Medicinae

SEPTEMBER 20, 2012

[Systemic diseases in pseudoexfoliation syndrome]

HOLLÓ Gábor

[Pseudoexfoliation syndrome is a condition associated with the production and accumulation of a pathological protein (pseudoexfoliation material). Originally, the syndrome was recognised on the basis of its intraocular symptoms and had been considered to be an isolated eye disease for a long time. However, some 20 years ago it became clear that in pseudoexfoliation syndrome pseudoexfoliation material is present all over in the body. In the past decade, vascular dysfunction associated with this syndrome has been also recognised. Recent studies have shown that pseudoexfoliation syndrome is caused by genetic alterations affecting a lysil oxidaselike (LOXL) protein, LOXL1. LOXL1 has an important role in the synthesis of extracellular material. To our current knowledge, pseudoexfoliation syndrome is a systemic elastosis associated with oxidative stress. Its complications are in part ocular (development of nuclear cataract, zonular damage and development of pseudoexfoliative glaucoma) and in part systemic (dysfunction of capillaries, muscular and elastic arteries, impairment of baroreflex function, increase of arterial rigidity, development of aorta aneurism, recurrent venous occlusions and neurosensory hearing loss). Despite the recognition of the above complications, currently it is not possible to set guidelines of a potential cardiovascular screening for patients with pseudoexfoliation syndrome, since the frequency and significance of systemic complications vary across different populations.]

Clinical Neuroscience

JULY 30, 2012

[Treatment of dystonia by deep brain stimulation: a summary of 40 cases]

DELI Gabriella, BALÁS István, KOMOLY Sámuel, DÓCZI Tamás, JANSZKY József, ILLÉS Zsolt, ASCHERMANN Zsuzsanna, TASNÁDI Emese, NAGY Ferenc, PFUND Zoltán, BÓNÉ Beáta, BOSNYÁK Edit, KULIFFAY Zsolt, SZIJJÁRTÓ Gábo

[Background - Bilateral pallidal deep brain stimulation (DBS) is an established treatment option for primary generalized and segmental dystonia. In the present study we evaluated the results of our dystonia patients treated by DBS. Methods - The surgical results of forty consecutive dystonia patients underwent DBS implantation were analyzed (age: 43.7±17.7 years; sex: 22 men; etiology: 24 primary and 16 secondary dystonia; topography: 24 generalized, 12 segmental and four hemidystonia; disease duration: 16.1±9.3 years). Severity of dystonia measured by Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and health-related quality of life measured by EQ-5D scale were obtained preoperatively and compared to the scores obtained at postoperative six months and subsequent yearly follow-ups. The average follow-up lasted 2.5 years (median, 0.5-8 years). In all cases the BFMDRS scores were re-evaluated by a rater blinded to the treatment. Treatment responsiveness was defined as an at least 25% improvement on the BFMDRS scores. Non-parametric Mann-Whitney, McNemar and Kruskal-Wallis tests were applied to test statistical significance. Results - Severity of dystonia improved from 31 to 10 points (median, 68% improvement, p<0.01) in the primary dystonia group, whereas in secondary dystonia these changes were statistically insignificant (improvement from 40 to 31.5 points, 21.2%, p>0.05). However, the health-related quality of life significantly improved in both groups (primary dystonia: 0.378 vs. 0.788 and secondary dystonia: 0.110 vs. 0.388, p<0.01). Significantly more patients in the primary dystonia group responded to DBS treatment than those in the secondary dystonia group (83.3% vs. 37.5%, p<0.01). Conclusion - Our results are in accordance with previously published international findings demonstrating that DBS is a highly effective and long-lasting treatment option for primary dystonia. DBS is considerably less efficient in secondary dystonia; however, it still has a high impact on the quality of life presumably due to its pain-relieving effect.]