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Copyright of Clinical Neuroscience / Ideggyógyászati Szemle is the property of LifeTime Media Kft. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Hungarian Medical Journal / Orvosi Hetilap is the property of Akademiai Kiado and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Clinical Neuroscience / Ideggyógyászati Szemle is the property of LifeTime Media Kft. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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.

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.

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.

Összefoglaló. Bevezetés: A COVID-19-járvány világszerte hónapokra átalakította a járóbeteg-ellátás működését is. Magyarországon a 2020. március 11-től 2020. június 17-ig fennálló egészségügyi veszélyhelyzeti rendelkezések szabták meg az új kereteket. Célkitűzés: Az első veszélyhelyzeti periódus második felében, 2020. április 22. és 2020. május 5. között mértük fel az epilepsziaellátásban részt vevő orvosok véleményét, hogy milyen mértékben változott a betegek ellátása, és hogyan élték meg a változásokat személyesen. Módszer: Internetes kérdőíves véleményfelmérés történt, a Magyar Epilepszia Liga 2020. április 16-17-re tervezett, de a COVID-19-járvány miatt elhalasztott XV. kongresszusára regisztrált neurológusok között. Kilenc egyszeres vagy többszörös feleletválasztós kérdés és 'szabad kommentár' mezők álltak rendelkezésre. Eredmények: A megkeresett 116 neurológus közül 33-an válaszoltak (28%), összesen 30 kommentár került rögzítésre. 73%-uk szerint a változások komoly nehézséget okoztak, 15%-uk gondolta, hogy ennek súlyos következményei lesznek. Új betegek fogadása 53%-ban leállt, 25%-ban nagy nehézségekbe ütközött. A gondozott betegek problémáit 49%-ban a távvizit lehetőségeivel élve meg tudták oldani, de 24%-ban ez nem sikerült. A beteg távollétében lebonyolított vizitek 68%-a dokumentált telefonbeszélgetések formájában zajlott. Az orvosok kétharmada veszélyeztetve érezte magát, hogy elkapja a vírust, ebből 40% úgy érezte, nem kap elegendő védelmet, 6% (2 fő) kapta el a fertőzést. Következtetés: A COVID-19-járvány a leginkább az új szakvélemények kiadását érintette, de a gondozási feladatokat sem mindig lehetett megfelelően megoldani. A károkat jelentősen enyhítette az ellátószemélyzet rugalmassága. A telefonvizitek, szükség esetén, az epileptológiában pótolhatják a személyes orvos-beteg találkozásokat. A járvány visszatérésének veszélye miatt a távvizit-alkalmazások technikai fejlesztése és ezek dokumentálási kérdéseinek megoldása fontos. A járványidőszakban a személyzet védelmére nagy figyelmet kell fordítani a fertőződés elkerülése és az orvosok biztonságérzetének fokozása érdekében. Orv Hetil. 2020; 161(46): 1939-1943., Introduction: COVID-19 pandemic has transformed the operation of outpatient care worldwide for months. The new framework was set in Hungary by the health emergency regulations that existed from 11. 03. 2020 to 17. 06. 2020., Objective: In the second half of the emergency period, between 22. 04. 2020 and 05. 05. 2020, we surveyed the opinion of physicians involved in epilepsy care about the extent to which patient care had changed and how they experienced the changes in person., Method: An internet questionnaire survey was conducted among neurologists registered for the annual congress of the Hungarian Chapter of the International League Against Epilepsy. Nine single- or multiple-choice questions and 'free comment' fields were available., Results: Of 116 neurologists contacted, 33 responded (28%), and a total of 30 comments were recorded. 73% said the changes caused a serious difficulty, 15% thought it would have serious consequences. Reception of new patients was stopped in 53%, and 25% encountered great difficulties. In 49%, the problems of the cared patients could be solved using remote visits, but 24% could not solve them properly. 68% of outpatient visits took the form of documented telephone conversations. Two-thirds of doctors feared catching the virus, 40% of whom felt they were not getting enough protection. 6% caught the infection., Conclusion: The COVID-19 pandemic has mostly affected the issuance of new expert opinions, but care tasks have not always been adequately addressed. The damage was significantly mitigated by the flexibility of the care staff. Telephone visits, if necessary, can replace personal doctor-patient encounters in epileptology. The technical development of remote visit applications and their documentation issues are important. During the pandemic period, great care must be taken to protect staff in order to avoid infection and increase the sense of safety of doctors. Orv Hetil. 2020; 161(46): 1939-1943.

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.

Myoclonus-dystonia (DYT11) is a rare, autosomal dominant hereditary disorder clinically characterized by myoclonus and/or dystonia. The disease is most commonly caused by the mutations of the SGCE gene. Causative therapy is not available currently. Regarding symptomatic treatment, zonisamide, insulin therapy, carbamazepine and zolpidem may be utilized. If these drugs are not effective, bilateral globus pallidus internus deep brain stimulation may come into consideration. The aim of this study is to demonstrate the efficacy of zonisamide treatment in a Hungarian patient with genetically proven myoclonus-dystonia. Our 25-year-old female patient has had jerky, lightning-like movements since her childhood, mainly localized to her right upper limb. In addition, muscle cramps associated with writing and walking were also present. The symptoms were reduced by alcohol consumption. Brain MRI did not show any abnormality. Neurophysiological studies raised the possibility of subcortical myoclonus. After detailed phenotyping, genetic testing was performed, yielding the diagnosis of myoclonus-dystonia. A heterozygous mutation in the 6th exon of the SGCE gene at the position 709, resulting in an early stop codon (c.709C> T, p.Arg237*) was demonstrated. After considering the risk-benefit ratio, we decided to start zonisamide treatment. The dose was titrated gradually to 300 mg/d over 6 weeks. Myoclonus- and dystonia-specific tests demonstrated significant improvement compared to the pre-treatment status. The aim of this case report is to draw attention to this rare condition, its treatment and the importance of collaboration between medical subspecialties. Orv Hetil. 2019; 160(34): 1353-1357.

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.

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.

The rare, genetically determined group of diseases characterized by pathological accumulation of iron in the central nervous system and progressive, typically movement disorder's symptoms are called NBIA (neurodegeneration with brain iron accumulation). By the rapid development of molecular genetics, it has become apparent that different mutations in numerous genes can lead to pathological cerebral iron accumulation. Simultaneously, it has also been recognized that the age of onset, the symptoms and the prognosis of NBIA disorders are much more diverse than it was previously perceived. To our knowledge, a review article on the most recent clinical data of NBIA has not been published in Hungarian. In the first part of this publication, we survey the general clinical characteristics and the diagnostic algorithm of NBIA diseases and address some considerations for differential diagnostics. In the second part of this review, the particular NBIA disorders are presented in details. The purpose of this article is to provide a clinical overview that may be useful for neurologists, pediatricians and any other medical practitioners interested in this field.

Objectives: Different methods of open or minimally invasive thymectomies have been recommended for the treatment of myasthenia gravis (MG). We compared the results of standard transsternal thymectomy (TS) and two different types of minimally invasive thymectomies [video-assisted thoracoscopic extended thymectomy (VATET) and classic Video-Assisted Thoracoscopic Surgery (cVATS)] performed at the same department., Methods: During three different time periods 71 patients (60 female and 11 male; mean age 31 [range, 14-84] years) underwent thymectomy for MG. Twenty-three underwent standard transsternal thymectomy (January 1995 - September 2004), 22 VATET (September 2004 - August 2009), and 26 cVATS (September 2009 - December 2011) thymectomy for the right side. Operative data, MG- and surgery-related postoperative morbidity and early improvement of MG during the initial 1-year follow-up period were compared among the three methods., Results: There were no perioperative deaths during the study period. Operative time was 112, 211, and 116 minutes (p = 0.001) in the TS, VATET and cVATS, respectively, and the length of hospital stay was 8.9, 5.6, and 4.0 (p = 0.001) days. Postoperative MG-related neurological morbidity affected 21.7%, 18.2%, and 7.7% (p = 0.365) of the patients and the surgery-related morbidity rate was 4.3%, 13.7%, and 0% (p = 0.118) in the TS, VATET and cVATS groups, respectively. Symptom improvement rates were 91.3%, 94.7%, and 87.5% (p = 0.712), and complete remission rates were 13%, 10.5%, and 11.5% (p = 0.917) after TS, VATET and cVATS thymectomies, respectively., Conclusions: In terms of operative time and hospital stay the best results were found after cVATS. The use of a less invasive surgical intervention resulted in less surgical-, and MG related neurological complications. The improvement of MG symptoms was excellent and results were similar after different types of thymectomies.

Background: The Unified Dyskinesia Rating Scale (UDysRS) was published in 2008. It was designed to be simultaneous valid, reliable and sensitive to therapeutic changes. The Movement Disorder Society 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 UDysRS and its validation process into Hungarian., Methods: After the translation of UDysRS into Hungarian and back-translated into English, it was reviewed by the UDysRS translation administration team. Subsequent cognitive pretesting was conducted with ten patients. For the large field testing phase, the Hungarian official working draft version of UDysRS was tested with 256 patients with Parkinson's disease having dyskinesia. Confirmatory factor analyses (CFA) determined whether the factor structure for the valid Spanish UDysRS 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 Spanish-language version., Results: For the Hungarian UDysRS the CFI was 0.98., Conclusion: The overall factor structure of the Hungarian version was consistent with that of the Spanish version based on the high CFIs for the UDysRS in the CFA; therefore, this version was designated as the Official Hungarian Version Of The UDysRS.

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.

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'.

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.

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.

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.

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.

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.

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.

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.

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.

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.