43 results on '"Klivényi, Péter"'
Search Results
2. Novel heterozygous STUB1 gene mutation causes SCA48 in a Hungarian patient
- Author
-
Szpisjak, László, primary, Salamon, András, additional, Németh, Viola L., additional, Szépfalusi, Noémi, additional, Maróti, Zoltán, additional, Kalmár, Tibor, additional, Zimmermann, Aliz, additional, Zádori, Dénes, additional, and Klivényi, Péter, additional
- Published
- 2023
- Full Text
- View/download PDF
3. Neurosyphilis vagy nem – egy differenciáldiagnosztikai kihívás esete
- Author
-
Csáti, Anett, primary, Szok, Délia, additional, Végh, Rita, additional, Jakab, Katalin, additional, Sárvári, Károly Péter, additional, Terhes, Gabriella, additional, Pásztor, Gyula, additional, Gaál, Magdolna, additional, Klivényi, Péter, additional, and Tajti, János, additional
- Published
- 2023
- Full Text
- View/download PDF
4. Clinical features of cervical dystonia patients classified by the COL-CAP concept and treated with ultrasound-guided botulinum neurotoxin
- Author
-
Szabó, Máté, primary, Do Kiem, Dániel, additional, Gárdián, Gabriella, additional, Szpisjak, László, additional, Salamon, András, additional, Klivényi, Péter, additional, and Zádori, Dénes, additional
- Published
- 2023
- Full Text
- View/download PDF
5. Alemtuzumabterápiával kezelt sclerosis multiplexes betegek követéses vizsgálata a szegedi Sclerosis Multiplex Centrumban
- Author
-
Fricska-Nagy, Zsanett, primary, Füvesi, Judit, additional, Kincses, Zsigmond Tamás, additional, Légrádi, Dóra, additional, Vécsei, László, additional, Klivényi, Péter, additional, and Bencsik, Krisztina, additional
- Published
- 2022
- Full Text
- View/download PDF
6. Ischaemiás stroke-on átesett afáziás személyek olvasási folyamatainak elemzése
- Author
-
Kis, Orsolya, primary, Steklács, János, additional, Jakab, Katalin, additional, and Klivényi, Péter, additional
- Published
- 2022
- Full Text
- View/download PDF
7. Rare complication of West Nile viral encephalitis
- Author
-
Faragó, Péter, primary, Fricska-Nagy, Zsanett, additional, Klivényi, Péter, additional, and Tajti, János, additional
- Published
- 2021
- Full Text
- View/download PDF
8. Atypical presentation of late-onset Sandhoff disease : A case report
- Author
-
Salamon, András, primary, Szpisjak, László, additional, Zádori, Dénes, additional, Lénárt, István, additional, Maróti, Zoltán, additional, Kalmár, Tibor, additional, Brierley, Charlotte M. H., additional, Deegan, Patrick B., additional, and Klivényi, Péter, additional
- Published
- 2021
- Full Text
- View/download PDF
9. A Magyar Klinikai Neurogenetikai Társaság konszenzusajánlása a felnőttkori spinalis izomatrophia (SMA) kezeléséhez
- Author
-
Boczán, Judit, primary, Klivényi, Péter, additional, Kálmán, Bernadette, additional, Széll, Márta, additional, Karcagi, Veronika, additional, Zádori, Dénes, additional, and Molnár, Mária Judit, additional
- Published
- 2021
- Full Text
- View/download PDF
10. Légzési elégtelenséggel járó CANOMAD szindróma
- Author
-
Salamon, András, primary, Dézsi, Lívia, additional, Radics, Bence, additional, Varga, Edina Tímea, additional, Hortobágyi, Tibor, additional, Tömösvári, Adrienn, additional, Vécsei, László, additional, Klivényi, Péter, additional, and Rajda, Cecília, additional
- Published
- 2020
- Full Text
- View/download PDF
11. Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT
- Author
-
Jarabin, János András, primary, Klivényi, Péter, additional, Tiszlavicz, László, additional, Molnár, Fiona Anna, additional, Gion, Katalin, additional, Földesi, Imre, additional, Kiss, József Géza, additional, Rovó, László, additional, and Bella, Zsolt, additional
- Published
- 2020
- Full Text
- View/download PDF
12. Az előrehaladott Parkinson-kór jellemzői a klinikai gyakorlatban: az OBSERVE-PD vizsgálat eredményei és a magyarországi alcsoport elemzése
- Author
-
Takáts, Annamária, primary, Aschermann, Zsuzsanna, additional, Vécsei, László, additional, Klivényi, Péter, additional, Dézsi, Lívia, additional, Zádori, Dénes, additional, Valikovics, Attila, additional, Varannai, Lajos, additional, Onuk, Koray, additional, Kinczel, Beatrix, additional, and Kovács, Norbert, additional
- Published
- 2020
- Full Text
- View/download PDF
13. Multiplex ischaemiás stroke Osler-Rendu-Weber-kórban
- Author
-
Salamon, András, primary, Faragó, Péter, additional, Németh, Viola Luca, additional, Szépfalusi, Noémi, additional, Horváth, Emese, additional, Vass, Andrea, additional, Bereczky, Zsuzsanna, additional, Tajti, János, additional, Vécsei, László, additional, Klivényi, Péter, additional, and Zádori, Dénes, additional
- Published
- 2019
- Full Text
- View/download PDF
14. Additional value of tau protein measurement in the diagnosis of Creutzfeldt-Jakob disease
- Author
-
Cseh, Edina Katalin, primary, Veres, Gábor, additional, Danics, Krisztina, additional, Szalárdy, Levente, additional, Nánási, Nikolett, additional, Klivényi, Péter, additional, Vécsei, László, additional, and Zádori, Dénes, additional
- Published
- 2019
- Full Text
- View/download PDF
15. A Parkinson-kór stádiumbeosztása és az előrehaladott stádium kezelésének néhány aktuális problémája
- Author
-
Klivényi, Péter, primary and Vécsei, László, additional
- Published
- 2018
- Full Text
- View/download PDF
16. GENETICALLY DETERMINED DISEASES ASSOCIATED WITH PATHOLOGICAL BRAIN IRON ACCUMULATION AND NEURODEGENERATION
- Author
-
Ács, Péter, primary, Molnár, Mária Judit, additional, Klivényi, Péter, additional, and Kálmán, Bernadette, additional
- Published
- 2016
- Full Text
- View/download PDF
17. Unilateral thalamic infarction causing downward gaze palsy in a patient with uncorrected tetralogy of Fallot: a case report = A lefelé tekintés bénulását okozó egyoldali thalamusinfarktus Fallot-tetralógia nem korrigált formájával élő felnőttnél: esetismertetés
- Author
-
Annus, Ádám, primary, Bencsik, Krisztina, additional, Járdánházy, Tamás, additional, Vécsei, László, additional, and Klivényi, Péter, additional
- Published
- 2016
- Full Text
- View/download PDF
18. Ajánlás a Parkinson-kór előrehaladott stádiumának kezeléséhez
- Author
-
Aschermann, Zsuzsanna, primary, Dibó, György, additional, Klivényi, Péter, additional, Kovács, Norbert, additional, Kovács, Tibor, additional, Takáts, Annamária, additional, Tamás, Gertrúd, additional, and Varannai, Lajos, additional
- Published
- 2016
- Full Text
- View/download PDF
19. Validation of the Hungarian Unified Dyskinesia Rating Scale
- Author
-
Horváth, Krisztina, primary, Aschermann, Zsuzsanna, additional, Ács, Péter, additional, Bosnyák, Edit, additional, Deli, Gabriella, additional, Pál, Endre, additional, Késmárki, Ildikó, additional, Horváth, Réka, additional, Takács, Katalin, additional, Balázs, Éva, additional, Komoly, Sámuel, additional, Bokor, Magdolna, additional, Rigó, Eszter, additional, Lajtos, Júlia, additional, Takáts, Annamária, additional, Tóth, Adrián, additional, Klivényi, Péter, additional, Dibó, György, additional, Vécsei, László, additional, Hidas, Eszter, additional, Nagy, Ferenc, additional, Herceg, Mihály, additional, Imre, Piroska, additional, and Kovács, Norbert, additional
- Published
- 2015
- Full Text
- View/download PDF
20. [Neurosyphilis or not - a case of a differential diagnostic challenge].
- Author
-
Csáti A, Szok D, Végh R, Jakab K, Sárvári PK, Terhes G, Pásztor G, Gaál M, Klivényi P, and Tajti J
- Subjects
- Female, Humans, Adult, Diagnosis, Differential, Prevalence, Syphilis cerebrospinal fluid, Syphilis complications, Syphilis diagnosis, Neurosyphilis diagnosis, Neurosyphilis complications, Neurosyphilis drug therapy, Myelitis, Transverse
- Abstract
We report the case of a 42-year-old woman with paraparesis associated with transverse myelitis. For differential diagnostics detailed microbiological, cerebrospinal fluid (CSF) and neuroimaging examinations were performed. Syphilis was confirmed, but diagnosis of neurosyphilis was only probable based on the CSF microbiological test results. The beneficial treatment response to application of the therapeutic protocol for syphilis supported the supposed diagnosis of syphilis-associated myelitis in our case. In this case report we reviewed the differential diagnostic tools of myelopathies/myelitis.
.
Nowadays regarding to growing prevalence of syphilis worldwide physicians should face on its presence and medical consequences.- Published
- 2023
- Full Text
- View/download PDF
21. [Novel heterozygous STUB1 gene mutation causes SCA48 in a Hungarian patient].
- Author
-
Klivényi P, Szpisjak L, Salamon A, Németh VL, Szépfalusi N, Maróti Z, Kalmár T, Zimmermann A, and Zádori D
- Subjects
- Humans, Hungary, Ataxia diagnosis, Ataxia genetics, Mutation, Atrophy, Ubiquitin-Protein Ligases genetics, Dysarthria, Brain Diseases
- Abstract
Autosomal dominant cerebellar ataxias (ADCA), also known as spinocerebellar ataxias (SCA) are a group of progressive neurodegenerative diseases with remarkable clinical and genetic heterogeneity. In the last ten years 20 genes were identified in the background of SCAs. One of these genes was STUB1 (STIP1 homology and U-box containing protein 1) (chromosome 16p13, NM_005861.4) encoding a multifunctional E3 ubiquitine ligase (CHIP)1. In 2013, STUB1 was identified as a causative gene of autosomal recessive spinocerebellar ataxia 16 (SCAR16), but in 2018 Genis et al. published that heterozygous mutations of this gene can cause the autosomal dominantly inherited SCA48 as well1,2. 28 French, twelve Italian, three Belgian, two North-American, one Spanish, one Turkish, one Dutch, one German and one British SCA48 families have been reported so far2-9. Based on these publications, SCA48 is a late-onset, progressive disorder characterized by cerebellar dysfunction, cognitive impairment, psychiatric features, dysphagia, hyperreflexia, urinary tract symptoms and movement disorders including Parkinsonism, chorea, dystonia and rarely tremor. The brain MRI in all SCA48 patients demonstrated vermian and hemispheric cerebellar atrophy which was more pronounced in the posterior areas (lobules VI and VII) of the cerebellum in most of the cases2-9. Besides this, T2- weighted imaging (T2WI) hyperintensity of dentate nuclei (DN) was reported in some Italian patients10. Moreover, the most recent publication described alterations on DAT-scan imaging in some French families9. Neurophysiological examinations did not find any central or peripheral nervous system abnormalities2,3,5. Neuropathologic findings revealed definite cerebellar atrophy and cortical shrinkage with variable severity6,7. The histopathological assessment denoted Purkinje cell loss, p62-positive neuronal intranuclear inclusions in some cases and tau pathology in one patient6-7. In this paper we describe the clinical and genetic characterization of the first Hungarian SCA48 case with a novel heterozygous STUB1 gene missense mutation.
- Published
- 2023
- Full Text
- View/download PDF
22. [Follow up examination of multiple sclerosis patients treated with alemtuzumab in Multiple Sclerosis Centre, Szeged].
- Author
-
Füvesi J, Kincses TZ, Légrádi D, Vécsei L, Klivényi P, Bencsik K, and Fricska-Nagy Z
- Subjects
- Humans, Alemtuzumab adverse effects, Follow-Up Studies, Recurrence, Multiple Sclerosis diagnostic imaging, Multiple Sclerosis drug therapy, Multiple Sclerosis, Relapsing-Remitting diagnostic imaging, Multiple Sclerosis, Relapsing-Remitting drug therapy
- Abstract
Background and Purpose: Therapeutic strategy of relapse-remitting multiple sclerosis has changed significantly during the past decade. While earlier escalating therapy was widely applied, recently, in case of high disease activity, induction therapy has become available., Methods: In our study, we processed the data of our alemtuzumab treated patients from our register. Alte-ra-tions in relapse rate and MRI activity due to the treatment were determined. These data were compared in patients treated with alemtuzumab as an escalating and as an induction therapy. We noted the observed side effects., Results: The 49 patients observed in the study had undergone two cycles of alemtuzumab therapy. The drug was applied as an induction therapy in 9 cases. Average relapse rate during the two years was 1.4±1.0, which decreased to 0.1±0.3 in the two years following the therapy. The average EDSS before therapy was 2.7±2.2 in the induction therapy group and 2.7±1.7 in the escalating therapy group. After the treatment, this score decreased to 1.6±0.6 and 2.5±1.8 in the induction and the escalating therapy group, respectively. We found clinical and MRI progression in case of 4 patients. As regards to side effects, cytokine release was detected in 51.0% of the patients following the first cycle, and 24.5% of the patients following the second cycle of the therapy. Infection related to the therapy was observed in 28.6%, while autoimmune thyreoiditis was diagnosed in 18.3% of the patients., Conclusion: The tight follow-up of the treated patients and the precise documentation of the register enable the comparison of results yielded by placebo controlled clinical studies with daily practice, as well as to gain information on the benefits of induction therapy as a paradigm shift in treating MS patients.
- Published
- 2022
- Full Text
- View/download PDF
23. [Investigation of reading abilities of ischemic stroke patients with aphasia].
- Author
-
Kis O, Steklács J, Jakab K, and Klivényi P
- Subjects
- Humans, Ischemic Stroke, Stroke complications, Aphasia etiology, Language Disorders, Dyslexia
- Abstract
Background and Purpose: Reading is a part of the language processes; a strong interaction can be found between them so the damage of the one has a strong impact on the other. It is worth to put emphasis on the exploration of reading disorders which occur with aphasia to have a better outcome of the rehabilitation process. The aim of our study is to explore the main characteristics of aqcuired reading disorders to have a more specialized and individualized language therapy., Methods: 19 ischemic stroke patients with aphasia took part in our study. All participants were right-handed with a lesion of left arteria cerebri media infarct. Due to the Hungarian version of Western Aphasia Battery 10 mild and 9 moderate participated. Reading abilities were investigated with our reading battery which consisted four main tasks: grapheme-phoneme correspondence, reading words, lexical access and reading comprehension. Tobii X120 device was used for recording and analyzing patients' eye-movements., Results: Significant positive correlations were found between the four subscales of Western Aphasia Battery and some part of the reading tasks. Eye-movements were analyzed, especially fixation count and total fixation duration. The severity of language disorder had a strong impact on fixation count and fixation duration. The more serious the language disorder was the more eye movements were detected., Conclusion: Our data support the idea that the severity of aphasia had a strong impact on reading processes and eye-movements. Eye-tracking device can help to have a deeper insight in the background brain mechanisms during reading. Our results contribute to have a more accurate diagnostic process to have a more specialized language therapy with better outcome.
- Published
- 2022
- Full Text
- View/download PDF
24. [Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients].
- Author
-
Boczán J, Klivényi P, Kálmán B, Széll M, Karcagi V, Zádori D, and Molnár JM
- Subjects
- Adult, Child, Consensus, Humans, Hungary, Prospective Studies, Retrospective Studies, Muscular Atrophy, Spinal genetics, Muscular Atrophy, Spinal therapy
- Abstract
Background and Purpose: Background - Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose - The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients., Methods: Methods - Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients - We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III., Results: In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA., Conclusion: This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.
- Published
- 2021
- Full Text
- View/download PDF
25. [Advanced Parkinson's disease characteristics in clinical practice: Results from the OBSERVE-PD study and sub-analysis of the Hungarian data].
- Author
-
Takáts A, Aschermann Z, Vécsei L, Klivényi P, Dézsi L, Zádori D, Valikovics A, Varannai L, Onuk K, Kinczel B, and Kovács N
- Subjects
- Cross-Sectional Studies, Humans, Hungary epidemiology, Movement Disorders diagnosis, Movement Disorders epidemiology, Parkinson Disease epidemiology, Parkinson Disease psychology, Severity of Illness Index, Surveys and Questionnaires, Movement Disorders psychology, Parkinson Disease classification, Parkinson Disease diagnosis, Quality of Life psychology
- Abstract
Background and Purpose: The majority of patients with advanced Parkinson's disease are treated at specialized movement disorder centers. Currently, there is no clear consensus on how to define the stages of Parkinson's disease; the proportion of Parkinson's patients with advanced Parkinson's disease, the referral process, and the clinical features used to characterize advanced Parkinson's disease are not well delineated. The primary objective of this observational study was to evaluate the proportion of Parkinson's patients identified as advanced patients according to physician's judgment in all participating movement disorder centers across the study. Here we evaluate the Hungarian subset of the participating patients., Methods: The study was conducted in a cross-sectional, non-interventional, multi-country, multi-center format in 18 countries. Data were collected during a single patient visit. Current Parkinson's disease status was assessed with Unified Parkinson's Disease Rating Scale (UPDRS) parts II, III, IV, and V (modified Hoehn and Yahr staging). Non-motor symptoms were assessed using the PD Non-motor Symptoms Scale (NMSS); quality of life was assessed with the PD 8-item Quality-of-Life Questionnaire (PDQ-8). Parkinson's disease was classified as advanced versus non-advanced based on physician assessment and on questions developed by the Delphi method., Results: Overall, 2627 patients with Parkinson's disease from 126 sites were documented. In Hungary, 100 patients with Parkinson's disease were documented in four movement disorder centers, and, according to the physician assessment, 50% of these patients had advanced Parkinson's disease. Their mean scores showed significantly higher impairment in those with, versus without advanced Parkinson's disease: UPDRS II (14.1 vs. 9.2), UPDRS IV Q32 (1.1 vs. 0.0) and Q39 (1.1 vs. 0.5), UPDRS V (2.8 vs. 2.0) and PDQ-8 (29.1 vs. 18.9)., Conclusion: Physicians in Hungarian movement disorder centers assessed that half of the Parkinson's patients had advanced disease, with worse motor and non-motor symptom severity and worse QoL than those without advanced Parkinson's disease. Despite being classified as eligible for invasive/device-aided treatment, that treatment had not been initiated in 25% of these patients.
- Published
- 2020
- Full Text
- View/download PDF
26. CANOMAD syndrome with respiratory failure.
- Author
-
Salamon A, Dézsi L, Radics B, Varga ET, Hortobágyi T, Tömösvári A, Vécsei L, Klivényi P, and Rajda C
- Subjects
- Fatal Outcome, Humans, Male, Middle Aged, Polyneuropathies, Quality of Life, Anemia, Hemolytic, Autoimmune, Ataxia, Ophthalmoplegia, Respiratory Insufficiency
- Abstract
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.
- Published
- 2020
- Full Text
- View/download PDF
27. [Multiple ischemic stroke in Osler-Rendu-Weber disease].
- Author
-
Salamon A, Faragó P, Németh VL, Szépfalusi N, Horváth E, Vass A, Bereczky Z, Tajti J, Vécsei L, Klivényi P, and Zádori D
- Subjects
- Aged, Child, Female, Humans, Telangiectasia, Hereditary Hemorrhagic genetics, Brain Ischemia diagnosis, Pulmonary Artery abnormalities, Pulmonary Veins abnormalities, Stroke diagnosis, Telangiectasia, Hereditary Hemorrhagic complications
- Abstract
Hereditary hemorrhagic teleangiectasia (HHT, Osler-Rendu-Weber disease) is an autosomal dominantly inherited disorder caused by the mutation of several possible genes and characterized by malformations of the arteriovenous system in multiple organs. The clinical diagnosis is based on the Curaçao criteria ((1) spontaneous, recurrent epistaxis; (2) teleangiectasias in characteristic sites (lips, oral cavity, nose, fingers); (3) visceral lesions (gastrointestinal, pulmonary, cerebral, spinal); (4) affected first degree relative). The aim of this study is to present the first genetically confirmed Hungarian case of hereditary hemorrhagic teleangiectasia with multiple ischemic strokes. Our 70-year-old woman has been suffering from severe epistaxis since her childhood and presented gastrointestinal bleeding during her adulthood as well. The characteristic skin lesions developed in the 5th decade of life. She was admitted to our department with loss of consciousness and fluctuating speech and swallowing problems. MRI of the brain supplemented with angiography revealed multiple arteriovenous malformations and multiple subacute ischemic lesions. The EEG demonstrated slowing of electric activity in the left frontal lobe. The neuropsychological assessment showed deficits in anterograde memory and executive functions. The diagnostic work-up for other characteristic alterations identified an arteriovenous malformation in the left lung. The genetic analysis demonstrated a heterozygous mutation in the 7th exon of the ENG gene at position 834 resulting in a thymine duplication and an early stop codon by a frame shift. The present case is largely similar to those already described in literature and draws the attention to the importance of multidisciplinary collaboration in the care of HHT patients.
- Published
- 2019
- Full Text
- View/download PDF
28. [The treatment of advanced Parkinson's disease].
- Author
-
Klivényi P and Vécsei L
- Subjects
- Humans, Severity of Illness Index, Disease Management, Disease Progression, Parkinson Disease therapy
- Abstract
The treatment of Parkinson's disease depends on the symptoms of the patients and obviously the stage of the disease. Several different approaches can be found in the literature. Based on the published data, in this review we try to summarize the different approaches to the disease stages and theirs' clinical relevance. Actually, one of the most important issue is the recognition of advanced stage and therefore we reviewed the device-aided therapies.
- Published
- 2018
- Full Text
- View/download PDF
29. [Recommendation for treatment options in advanced Parkinson's disease].
- Author
-
Aschermann Z, Dibó G, Klivényi P, Kovács N, Kovács T, Takáts A, Tamás G, and Varannai L
- Subjects
- Humans, Parkinson Disease therapy
- Abstract
The treatment of advanced Parkinson's disease is challenging for both physicians and caregivers. The device-aided therapies need expertise and dedicated hospital centers. In this summary we have concluded the available data and recommendation for the treatment options in advanced Parkinson's disease and adopt them to the daily care in Hungary.
- Published
- 2016
- Full Text
- View/download PDF
30. The first identified Central-Eastern European patient with genetically confirmed dentatorubral-pallidoluysian atrophy.
- Author
-
Zádori D, Tánczos T, Jakab K, Vécsei L, and Klivényi P
- Subjects
- Diagnosis, Differential, Electroencephalography, Female, Heterozygote, Humans, Hungary, Magnetic Resonance Imaging, Middle Aged, Myoclonic Epilepsies, Progressive pathology, Myoclonic Epilepsies, Progressive psychology, Brain pathology, Executive Function, Myoclonic Epilepsies, Progressive diagnosis, Myoclonic Epilepsies, Progressive genetics, Nerve Tissue Proteins genetics, Trinucleotide Repeat Expansion
- Abstract
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.
- Published
- 2015
31. [Experience with levodopa/carbidopa intestinal gel in the treatment of advanced Parkinson's disease in Hungary].
- Author
-
Nagy H, Takáts A, Tóth A, Bereczki D, Klivényi P, Dézsi L, Dibó G, Vécsei L, Kovács N, Aschermann Z, Komoly S, Varannai L, Zemlényi G, and Valikovics A
- Subjects
- Aged, Aged, 80 and over, Antiparkinson Agents adverse effects, Carbidopa adverse effects, Drug Therapy, Combination, Dyskinesia, Drug-Induced etiology, Female, Gels, Humans, Hungary, Levodopa adverse effects, Male, Middle Aged, Patient Selection, Quality of Life, Retrospective Studies, Time Factors, Treatment Outcome, Antiparkinson Agents administration & dosage, Carbidopa administration & dosage, Dyskinesia, Drug-Induced prevention & control, Intestines, Levodopa administration & dosage, Parkinson Disease drug therapy
- Abstract
In the advanced Parkison's disease (PD) the late complications of levodopa therapy have to be considered: motor and/or non-motor fluctuations with or without disturbing dyskinesias. The non-motor fluctuations often influence the quality of life (QoL) in a much more negative way compared with the motor symptoms. In the treatment of advanced PD there are several device-aided methods - deep brain stimulation, apomorphine pump, levodopa/carbidopa intestinal gel (LCIG) - to improve the symptoms, the QoL, sometimes even in an individual, tailored custom form. The LCIG therapy was introduced in Hungary in 2011. Here we summarize the data of our patients: we have tested almost 60 patients and in 43 cases we have started this treatment. We analyze the duration of illness, levodopa therapy, motor and non-motor fluctuation of patients and present our experiences with the test phase and the chronic LCIG therapy via PEG/PEJ implantation. We paid attention to the surgery and device - depending side effects. Our experiences are similar to the international data. In patients selection ,,the right treatment, to the right patient, in the right time" is of importance.
- Published
- 2014
32. [VALIDATION OF THE HUNGARIAN MDS-UPDRS: WHY DO WE NEED A NEW PARKINSON SCALE?].
- Author
-
Horvath K, Aschermann Zsuzsanna, Acs P, Bosnyák E, Deli G, Pál E, Késmárki I, Horváth Réka, Takács K, Komoly S, Bokor M, Rigó E, Lajtos J, Klivényi P, Dibó G, Vécsei L, Takáts A, Tóth A, Imre P, Nagy F, Herceg M, Hidasi E, and Kovács N
- Subjects
- Antiparkinson Agents adverse effects, Cognition, Factor Analysis, Statistical, Humans, Hungary, Language, Levodopa adverse effects, Movement Disorders etiology, Observer Variation, Parkinson Disease complications, Parkinson Disease drug therapy, Reproducibility of Results, Severity of Illness Index, Translations, Tremor etiology, Antiparkinson Agents administration & dosage, Levodopa administration & dosage, Parkinson Disease physiopathology, Parkinson Disease psychology, Surveys and Questionnaires standards
- Abstract
Background: The Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) has been published in 2008 as the successor of the original UPDRS. The MDS-UPDRS organizing team developed guidelines for the development of official non-English translations consisting of four steps: translation/back-translation, cognitive pretesting, large field testing, and clinimetric analysis. The aim of this paper was to introduce the new MDS-UPDRS and its validation process into Hungarian., Methods: Two independent groups of neurologists translated the text of the MDS-UPDRS into Hungarian and subsequently back-translated into English. After the review of the back-translated English version by the MDS-UPDRS translation administration team, cognitive pretesting was conducted with ten patients. Based on the results of the initial cognitive pretesting, another round was conducted. For the large field testing phase, the Hungarian official working draft version of MDS-UPDRS was tested with 357 patients with Parkinson's disease (PD). Confirmatory factor analyses (CFA) determined whether the factor structure for the English-language MDS-UPDRS could be confirmed in data collected using the Hungarian Official Draft Version. To become an official translation, the Comparative Fit Index (CFI) had to be ≥ 0.90 compared to the English-language version., Results: For all four parts of the Hungarian MDS-UPDRS, the CFI was ≥ 0.94., Conclusion: The overall factor structure of the Hungarian version was consistent with that of the English version based on the high CFIs for all the four parts of the MDS-UPDRS in the CFA; therefore, this version was designated as the "OFFICIAL GUNGARIAN VERSION OF THE MDS-UPDRS'.
- Published
- 2014
33. [Clinical studies with levodopa/carbidopa intestinal gel].
- Author
-
Klivényi P and Vécsei L
- Subjects
- Antiparkinson Agents adverse effects, Carbidopa adverse effects, Clinical Trials as Topic, Cognition drug effects, Confounding Factors, Epidemiologic, Dopamine Agents adverse effects, Gels, Humans, Levodopa adverse effects, Peripheral Nervous System Diseases chemically induced, Quality of Life, Research Design, Sample Size, Sleep drug effects, Antiparkinson Agents administration & dosage, Carbidopa administration & dosage, Dopamine Agents administration & dosage, Drug Delivery Systems methods, Intestines, Levodopa administration & dosage, Parkinson Disease drug therapy
- Abstract
The motor complications in advanced Parkinson's disease may interfere with the quality of life. To overcome this disability, a jejunal levodopa pump was developed. In this review we have summarized the published clinical data with this method.
- Published
- 2014
34. Assessment of the role of multidrug resistance-associated proteins in MPTP neurotoxicity in mice.
- Author
-
Plangár I, Zádori D, Szalárdy L, Vécsei L, and Klivényi P
- Subjects
- 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine, Allopurinol pharmacology, Animals, Brain metabolism, Chromatography, High Pressure Liquid, Corpus Striatum drug effects, Dopamine Agents administration & dosage, Drug Administration Schedule, Flavanones pharmacology, Infusions, Parenteral, Male, Mice, Mice, Inbred C57BL, Multidrug Resistance-Associated Protein 2, Neurotoxins, Oxidative Stress, Parkinson Disease etiology, Silymarin pharmacology, Sulfinpyrazone pharmacology, Uric Acid metabolism, 3,4-Dihydroxyphenylacetic Acid metabolism, Corpus Striatum metabolism, Dopamine metabolism, Dopamine Agents pharmacology, Homovanillic Acid metabolism, Multidrug Resistance-Associated Proteins metabolism, Parkinson Disease drug therapy, Parkinson Disease metabolism
- Abstract
Goals: The available scientific data indicate that the pathomechanism of Parkinson's disease (PD) involves the accumulation of endogenous and exogenous toxic substances. The disruption of the proper functioning of certain transporters in the blood-brain barrier and in the blood-cerebrospinal fluid barrier in PD would accompany to that accumulation. Although there is an emerging role of the dysfunction of multidrug resistance-associated proteins (MRPs), members of ATP-b nding cassette (ABC) transporter superfamily, in neurodegenerative disorders, there is only a few available data as regards PD. So the aim of our study was the assessment of the role of certain MRPs (1 ,2, 4 and 5) in neurotoxicity induced by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, Methods: Following the intraperitoneal administration of silymarin (with MRP1, 2, 4 and 5 inhibitory effects), naringenin (with MRP1, 2 and 4 stimulatory effects), sulfinpyrazone (with MRP1, 4 and 5 inhibitory and MRP2 stimulatory effects) and allopurinol (with MRP4 stimulatory effect in doses of 100 mg/kg, 100 mg/kg, 100 mg/kg and 60 mg/kg, respectively, for one week before and after the administration of MPTP in C57B/6 mice in acute dosing regimen the striatal concentrations of dopamine, 3,4-dihydroxyphenylacetic acid and homovanillic acid has been measured using high-performance liquid chromatography., Results: Although the results of these experiments showed that neither of these substances exerted significant influence on MPTP-induced striatal depletion of dopamine and its metabolites, naringenin exerted a slight prevention of dopamine decrease, while allopurinol considerably enhanced the MPTP-induced lethality in mice. The explanation of these findings would be that the stimulation of MRP1- and MRP2-mediated transport of glutathione conjugates of toxic substances may have slight beneficial effects, while stimulation of MRP4-mediated efflux of brain urate, which has an important antioxidant potency, may worsen the effects of oxidative stress.
- Published
- 2013
35. Diffusion MRI measured white matter microstructure as a biomarker of neurodegeneration in preclinical Huntington's disease.
- Author
-
Kincses ZT, Szabó N, Tóth E, Zádori D, Faragó P, Németh D, Janacsek K, Babos M, Klivényi P, and Vécsei L
- Subjects
- Adult, Anisotropy, Biomarkers, Case-Control Studies, Corpus Callosum ultrastructure, Female, Humans, Huntington Disease pathology, Male, Middle Aged, Brain ultrastructure, Diffusion Magnetic Resonance Imaging, Huntington Disease diagnosis, Nerve Fibers, Myelinated ultrastructure
- Abstract
Background: Huntington's disease is a progressive neurodegenerative disease, genetically determined by CAG trinucleotide expansions in the IT15 gene. The onset of the symptoms is related to the number of CAG triplets. Because the patients are asymptomatic in the early phase of the disease, in vivo biomarkers are needed to follow up the reurodegeneration and to test putative neuroprotective approaches. One such promising biomarker is the diffusion MRI measured microstructural alteration of the white matter., Methods: Seven presymtomatic, mutation carriers and ten age-matched healthy controls were included in the study. Diffusion parameters were compared between groups and correlated with measures describing neurodegeneration. In order to reduce the possible misregistration bias due to atrophy the analysis was restricted to the core of each fibre bundles as defined by maximal fractional anisotropy (Tract-Based Spatial Statistics)., Results: Decreased fractional anisotropy, along with increased mean, parallel and perpendicular diffusivity was found in white matter tracts, mainly in the corpus callosum. An inverse correlation was detected between the fractional anisotropy and neurodegeneration score (derived from the number of CAG triplets and the patient age) from the areas of the left precentral gyrus, frontal lobe, corpus callosum and the capsula extrema. Altered diffusion parameters are promising biomarkers of the neurodegeneration in Huntington's disease.
- Published
- 2013
36. [The absence of the common LRRK2 G2019S mutation in 120 young onset Hungarian Parkinon's disease patients].
- Author
-
Balicza P, Bereznai B, Takáts A, Klivényi P, Dibó G, Hidasi E, Balogh I, and Molnár MJ
- Subjects
- Adult, Age of Onset, Aged, Female, Gene Frequency, Genetic Predisposition to Disease, Genetic Testing, Glycine, Humans, Hungary epidemiology, Leucine-Rich Repeat Serine-Threonine Protein Kinase-2, Male, Middle Aged, Serine, Parkinson Disease epidemiology, Parkinson Disease genetics, Point Mutation, Protein Serine-Threonine Kinases genetics
- Abstract
Parkinson's disease is a promising target of applying personalized medicine. For this purpose it is crucial to reveal the genetic and environmental factors, which contribute to the disease, also to collect epidemiologic data and to preserve the patients samples and data in a proper biobank. In our investigation we examined the prevalence of the most frequent Parkinson's disease causing LRRK2 G2019S mutation in a Hungarian Parkinson-patient group. From 120 patients, we haven't detected this substitution in anyone. Our investigation suggest that the mutation LRRK2 G2019S may be a rare cause of Parkinson disease in the Hungarian population.
- Published
- 2012
37. [Paroxysmal kinesigenic dyskinesia].
- Author
-
Vadász D, Klivényi P, and Vécsei L
- Subjects
- Anticonvulsants therapeutic use, Anxiety etiology, Carbamazepine therapeutic use, Humans, Male, Quality of Life, Social Isolation, Young Adult, Chorea classification, Chorea diagnosis, Chorea drug therapy, Chorea genetics, Chorea psychology
- Abstract
Paroxysmal kinesigenic dyskinesia (PKD) is a rare neurological disease and the diagnosis is based on case history and clinical features. Despite of simply diagnostic criteria, the recognition of the disease is sometimes delayed. The involuntary movements in PKD lead to anxiety, social isolation, trauma and worsens the quality of life. To establish the diagnosis many other paroxysmal syndromes have to be excluded. The disease responds to antiepileptic therapy well. The genetic background of the familiar cases is not known. Here we present a 19 year-old patient with PKD and review the current literature. Our patient's events were triggered by sudden movements and last several seconds. His physical and neurological examinations were normal and responded well to carbamazepine therapy.
- Published
- 2011
38. [Clinical studies on pramipexol retard].
- Author
-
Klivényi P and Vécsei L
- Subjects
- Aged, Antiparkinson Agents administration & dosage, Antiparkinson Agents adverse effects, Benzothiazoles administration & dosage, Benzothiazoles adverse effects, Clinical Trials, Phase III as Topic, Delayed-Action Preparations, Dose-Response Relationship, Drug, Double-Blind Method, Drug Administration Schedule, Female, Humans, Male, Middle Aged, Multicenter Studies as Topic, Pramipexole, Treatment Outcome, Antiparkinson Agents therapeutic use, Benzothiazoles therapeutic use, Parkinson Disease drug therapy
- Abstract
Pramipexol retard is the newest drug for the treatment of Parkinson's disease. The prolonged release of the agent in this preparation allows a more continuous dopaminergic stimulation than previous preparations, without reducing the agent's already known and proven clinical efficiency. In addition, it has a more favourable adverse effect profile than previous preparations, and patient compliance can also be better as it needs to be taken only once daily. These benefits have been proven in recent clinical studies, of which the most important ones are reviewed here.
- Published
- 2010
39. [Long term experience with Stalevo in Szeged, Hungary].
- Author
-
Klivényi P and Vécsei L
- Subjects
- Aged, Antiparkinson Agents administration & dosage, Antiparkinson Agents adverse effects, Drug Combinations, Drug Resistance, Dyskinesia, Drug-Induced etiology, Female, Humans, Male, Middle Aged, Pain Measurement, Quality of Life, Severity of Illness Index, Time Factors, Treatment Outcome, Antiparkinson Agents therapeutic use, Carbidopa therapeutic use, Catechols therapeutic use, Dyskinesia, Drug-Induced prevention & control, Levodopa therapeutic use, Parkinson Disease drug therapy
- Abstract
The triple combination of levodopa, DDCI and entacapone (Stalevo) is used to treat motor complication in patients with Parkinson's disease. In this study we summarized the clinical data of our patients treated with Stalevo for the longest period. We can concluded, that after switching to Stalevo due to wearing off, the average levodopa doses were lower then before and the motor complications were milder. After 3 years of Stalevo therapy the levodopa doses were increased but still did not reach the average doses before introducing Stalevo. After switching the patients' general well-being was improved as indicated by the visual analogue scale. In summary, the Stalevo treatment is safe and effective for long run and improves the patients' quality of life.
- Published
- 2009
40. [Neuroprotection in Parkinson's disease and other neurodegenerative disorders: preclinical and clinical findings].
- Author
-
Rákóczi K, Klivényi P, and Vécsei L
- Subjects
- Animals, Creatine pharmacology, Creatine therapeutic use, Dopamine Agonists pharmacology, Dopamine Agonists therapeutic use, Glatiramer Acetate, Humans, Indans pharmacology, Indans therapeutic use, Kynurenic Acid pharmacology, Kynurenic Acid therapeutic use, Micronutrients pharmacology, Micronutrients therapeutic use, Oxidative Stress drug effects, Peptides pharmacology, Peptides therapeutic use, Selegiline pharmacology, Selegiline therapeutic use, Tocopherols pharmacology, Tocopherols therapeutic use, Ubiquinone pharmacology, Ubiquinone therapeutic use, Nerve Degeneration drug therapy, Neurodegenerative Diseases drug therapy, Neuroprotective Agents pharmacology, Neuroprotective Agents therapeutic use, Parkinson Disease drug therapy
- Abstract
The authors summarized the evidence supporting neuroprotection based on the data available in the literature. In vivo and in vitro studies have indicated that many compounds can decrease neurodegeneration, excitotoxicity, oxidative stress, protein aggregation, disturbance of Ca2+ homeostasis and compensate the energy impairment. Selegiline, rasagiline, dopamine agonists and other molecules (ubiquinone, kynurenic acid, tocopherol, creatine, glatiramer acetate) exert neuroprotective effects in preclinical studies. Much less clinical data are available regarding neuroprotection in different neurological disorders. In this review, such preclinical and clinical evidences are summarized.
- Published
- 2009
41. [The use of Stalevo in Hungary for patients with Parkinson disease and its effect on the quality of life].
- Author
-
Klivényi P and Vécsei L
- Subjects
- Adult, Aged, Amantadine administration & dosage, Antiparkinson Agents administration & dosage, Carbidopa administration & dosage, Catechols administration & dosage, Cholinergic Antagonists administration & dosage, Dopamine Agonists administration & dosage, Drug Combinations, Female, Humans, Hungary, Levodopa administration & dosage, Male, Middle Aged, Selegiline administration & dosage, Surveys and Questionnaires, Time Factors, Treatment Outcome, Antiparkinson Agents therapeutic use, Carbidopa therapeutic use, Catechols therapeutic use, Levodopa therapeutic use, Parkinson Disease drug therapy, Quality of Life
- Abstract
The triple combination of levodopa, DDCI and entacapone (Stalevo) is used to treat motor complication in Parkinsonian patients with fluctuation. An observational investigation has been conducted in Hungary to study the effects of Stalevo on the "wearing off" phenomenon and on the quality of life in patients, who are to be treated with. The introduction of Stalevo to the treatment resulted in no changes in the number of patients taking selegiline, amantadine and dopamine agonists, while the number of patients taking anticholinergic drugs were slightly increased. This treatment significantly decreased the average Hoehn-Yahr stadium, as well as the non-motor symptoms, without any remarkable side effect. Stalevo also improved the quality of life, detected by the EQ-5D questionnaire and the visual analogue scale.
- Published
- 2008
42. [Updates in practical neurology--I. The principles of modern levodopa therapy in Parkinson's disease].
- Author
-
Klivényi P and Vécsei L
- Subjects
- Antiparkinson Agents adverse effects, Dopamine Agents adverse effects, Evidence-Based Medicine, Humans, Levodopa adverse effects, Parkinson Disease metabolism, Antiparkinson Agents therapeutic use, Dopamine metabolism, Dopamine Agents therapeutic use, Levodopa therapeutic use, Parkinson Disease drug therapy
- Abstract
Despite the levodopa is used for the treatment of Parkinson's disease for a long time, recently many questions raised about its clinical use. New issues emerged based on the clinical trials, on latest neuroimaging data and on better understanding the pathomechanism of motor complications. These observations have changed the routine clinical use of levodopa. In this review we summarize the evidences and practical implications of levodopa therapy.
- Published
- 2007
43. [Clinical features and diagnosis of spinocerebellar ataxia].
- Author
-
Klivényi P, Horváth Z, and Vécsei L
- Subjects
- Ataxin-7, Ataxins, Chromosome Disorders genetics, Diagnosis, Differential, Humans, Machado-Joseph Disease diagnosis, Prevalence, Proteins genetics, RNA, Long Noncoding, RNA, Untranslated, Spinocerebellar Ataxias physiopathology, Chromosome Disorders diagnosis, Genes, Dominant, Nerve Tissue Proteins genetics, Spinocerebellar Ataxias diagnosis, Spinocerebellar Ataxias genetics
- Abstract
In the past decade, a great progress has been made in understanding genetic basis of the spinocerebellar ataxia. Based upon the genotypes, more then 20 subgroups of autosomal dominant spinocerebellar ataxia have been identified with different gene mutations. Neither the pathomechanism nor the function of these genes is fully understood. In these disorders the main clinical sign is ataxia. Other symptoms may be present as well, but no specific clinical feature is known for differentiating subgroups. Specific diagnosis can be made by genetic tests. In this review we summarize the clinical features and genetic backgrounds of the most common spinocerebellar ataxias.
- Published
- 2004
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.