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Clinical Neuroscience

JULY 30, 2020

[Advanced Parkinson’s disease characteristics in clinical practice: Results from the OBSERVE-PD study and sub-analysis of the Hungarian data]

TAKÁTS Annamária, ASCHERMANN Zsuzsanna, VÉCSEI László, KLIVÉNYI Péter, DÉZSI Lívia, ZÁDORI Dénes, VALIKOVICS Attila, VARANNAI Lajos, ONUK Koray, KINCZEL Beatrix, KOVÁCS Norbert

[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. 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. 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). 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.]

Clinical Neuroscience

JULY 30, 2020

Extraskeletal, intradural, non-metastatic Ewing’s sarcoma. Case report

OTTÓFFY Gábor, KOMÁROMY Hedvig

Intracranial localization of Ewing’s sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing’s sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re­gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima­ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.

Clinical Oncology

DECEMBER 10, 2018

[Treatment of head and neck cancer]

KATONA Csilla, LANDHERR László

[Head and neck cancers cause worldwide a signifi cant problem in health care systems. Despite great advances in therapeutic modalities its prognosis has not changed in the past few decades. It is mainly caused by classical risk factors, like alcohol consumption and smoking, but in a signifi cant number of oropharyngeal cancers HPV infection plays a major role, which is associated with a new patient group characterized by a much better prognosis and therapeutic response. In the diagnostic phase staging examinations (CT scan, MRI, FDG-PET) are also involved which are necessary to multidisciplinary decision making. It can be largely infl uenced by the patient’s preference. The therapy is based on multimodality approach; surgery, radiotherapy, chemoirradiation, chemotherapy and the combination of these are used in early or locally advanced tumours. Targeted agents like EGFR inhibitors are partly used in the recurrent/metastatic setting or in combination with radiotherapy. Immun checkpoint inhibitors are new therapeutic options for pretreated, recurrent/metastatic patients and their role is under investigation in earlier therapeutic lines. Several clinical trials aim treatment desintensifi cation strategies in HPV positive tumours. Molecular genetic tests try to defi ne subgroups of patients to plan individualized treatment. Regarding the signifi cant functional and aesthetic damage of both disease and treatment, supportive care and rehabilitation are of great importance.]

Clinical Neuroscience

NOVEMBER 30, 2019

Vestibular evoked myogenic potential responses in Parkinson’s disease

CICEKLI Esen, TITIZ Pinar Ayse, TITIZ Ali, OZTEKIN Nese, MUJDECI Banu

Background - Our objectives were to determine the differences in the vestibular evoked myogenic potential (VEMP) responses in patients diagnosed with early staged idiopathic Parkinson’s disease (PD) compared to the normal population and evaluate the vestibular system disorder causing balance-posture disorders. Second aim of this study was to investigate caloric test responses particularly in early staged PD compared to normal popu­lation. Material and methods - Thirty patients (14 females and 16 males; mean age, 60.6 ± 13.1 years) diagnosed with idiopathic PD and 28 healthy subjects (20 males and 8 females; mean age, 59.1 ± 6.4 years) were included. The patient and control groups were subdivided according to their age, gender and the patient group was subdivided according to onset time of the Parkinson symptoms, Hoehn-Yahr staging. The subgroups were compared for VEMP and caloric test responses. Results - There were no significant differences between the study and control groups for right and left VEMP measurements. Patients over 60 years and under 60 years did not show significant differences in terms of right and left mean VEMP measurements. However, P1 amplitude was significantly lower in patients over 60 years old (P = .004). Gender, disease duration, BERG balance scale and Hoehn-Yahr stage had no effect on the VEMP amplitudes. There was no significant correlation with the side of Parkinsonian symptoms to the side of canal paresis (P = .566) and the side on which no VEMP response was obtained in caloric test. Conclusion - VEMP responses were not different between PD and healthy subjects. VEMP P1 amplitude was decreased with age in PD group. Canal paresis and symptoms side were not statistically correlated in caloric test.

Clinical Neuroscience

NOVEMBER 30, 2018

[The treatment of advanced Parkinson’s disease]

KLIVÉNYI Péter, VÉCSEI László

[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. ]

Clinical Oncology

FEBRUARY 10, 2018

[Treatment of hepatocellular carcinoma - an update]

DEMETER Gyula, VÉGH Éva

[Last time we have described about the modern treatment of hepatocellular carcinoma (HCC) in „Klinikai Onkológia” in 2014 (1) and a detailed guideline regarding epidemiology, treatment according to BCLC staging system has been published as well in a special edition in this year (2). Here, we discuss mainly the fi rst- and second line systemic treatment of HCC according to our experience and the new results of clinical trials. 203 patients were treated in our Department between 2010 and 2016. These results have been presented already on the MKOT conference in 2016. In this year we have started second line systemic therapy with regorafenib in 9 cases.]

Clinical Neuroscience

MAY 30, 2018

Assessment of mental health of carers according to patient stage of idiopathic Parkinson’s disease

YAZAR Olgun Hülya, YAZAR Tamer, DEMIR Yancar Esra, CANKAYA Soner, ENGINYURT Özgür

Purpose - In this study the aim was to collect data to assess the mental health of carers for patients with diagnosis of idiopathic Parkinson’s disease (IPD) according to disease stage and to examine precautions to reduce the patient and disease load on carers. Method - The study included 144 patients with staging according to modified Hoehn and Yahr criteria and 144 patient relatives who provided care support for patients every day, for some or all of the day, and who were over the age of 18 years and accepted participation in the research. Our prospective and cross-sectional study performed detailed neurological examination of patients, and after completing the ‘Personal Information Form’ with the interviewer every patient, with idiopathic Parkinson’s disease (IPD) according to ‘UK Brain Bank’ diagnostic criteria, had the ‘Unified Parkinson’s Disease Rating Scale (UPDRS)’ and ‘Modified Hoehn and Yahr scale (HYS)’ applied. Carers first completed the ‘Personal Information Form’ and then had the ‘Short Symptom Inventory (SSI)’ applied. Results - As the stage of disease increased, the points for all sub-scales of the Short Symptom Inventory increased. Conclusion - With the parallel increase in disease scores and UPDRS stage scores, the points obtained by carers on the SSI sub-scales increased. This data shows that with progressing disease stage, the load on the carer increases and mental health begins to be disrupted.

Clinical Oncology

MAY 10, 2015

[Invasive endoscopy in oncology]

MADÁCSY László

[Recent advances in interventional gastrointestinal endoscopy have led to a large variety of new diagnostic and minimally invasive endoscopic surgical procedures in oncological patients. Endoscopic ultrasound with the possibility of fi ne needle aspiration is currently one of the most accurate imaging technology for adequate staging of gastrointestinal cancers including oesophageal, gastric, rectal and pancreatic cancer. Endoscopic mucosal resection and endoscopic submucosal dissection offers a minimal invasive endoscopic treatment modality as an alternative for laparoscopic surgery for patients with early intramucosal neoplasias, fl at adenomas and laterally spreading tumors of the oesophagus, stomach, duodenum and colorectum. Self-expandable metal stents are now readily available for endoscopic palliation of different type of malignant gastrointestinal obstructions including oesophageal, duodenal, colonic and biliary stenosis. These recent developments of interventional gastrointestinal endoscopy lead to more precise and accurate tumor staging and more effective oncological therapy for patients with gastrointestinal cancers.]

Clinical Oncology

SEPTEMBER 10, 2014

[Treatment of hepatocellular carcinoma - today]

VÉGH Éva, DEMETER Gyula, BODOKY György

[The hepatocellular carcinoma (HCC) is one of the main causes of cancer-related death globally, and at the same time, it is the main event leading to death in cirrhotic patients. In the etiology of HCC, the non-alcoholic liver disease may be an important cause besides the viral cirrhosis. The HCC’s staging systems (Child-Pugh scores, Cancer of the Liver Italian Programme/CLIP, Barcelona Clinic Liver Cancer/ BCLC) play an important role in predicting the prognosis and determining the appropriate therapy. In Europe, the treatment strategy is based on the BCLC staging system. Screening of cirrhotic patient is also important because curative therapy is available only for the early-stage HCC. Several therapeutic options exist in the intermediate stage disease; among them the radiofrequency ablation (RFA), the transarterial chemoembolization (TACE) and the percutan ethanol injection (PEI) are most important. For the advanced disease, the only approved systemic therapy is sorafenib, which has been well-tolerated and yielded a substantially relative improvement in overall survival. For patient in end-stage disease with impaired liver function or a poor performance status, only supportive care is recommended.]

Lege Artis Medicinae

APRIL 20, 2014

[Diagnostic challenges in our adolescent patient with lymphoma]

MAGYARI Ferenc, RAJNAI Hajnalka, BARNA Sándor, MILTÉNYI Zsófia, VÁRÓCZY László, CSOMOR Judit, UDVARDY Miklós, ILLÉS Árpád

[OBJECTIVES - Unclassifiable B-cell lymphoma, which shows intermediate features typical for both diffuse, large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma (HL) is a novel category of diffuse, large B-cell lymphomas (DLBCL/HL), described in the WHO classification in 2008. This rare type of lymphomas presents peculiar clinical, morphological and immunophenotypical patterns, previously called gray-zone lymphomas. CASE REPORT - In December 2011 a 17- year old boy was diagnosed with mixedcellularity subtype of classical HL on the basis of left inguinal lymph node biopsy. Staging examinations revealed a IV/BXS (abdominal bulky) stage disease with unfavourable prognosis. Because of the unusally extended disease (nodal-extranodalbulky), a histological revision was performed. After a half course of ABVD chemotherapy the patient’s symptoms disappeared and the sizes of the involved lymph nodes decreased. On the basis of the histological revision, the diagnosis was changed to DLBCL/HL, so the treatment was modified to R-CHOP-14 regimen. After 3 cycles of R-CHOP-14 a complete metabolic remission (CMR) was achieved, which was confirmed by a 18FDG-PET/CT scan. Staging examinations after further 4 cycles of RCHOP- 14 therapy showed that the patient was still in CMR, but a PET-negative large mass (7×3 centimeter) still remained visible in the abdominal region. Considering this residual tumour and the agressive subtype of lymphoma the patient was referred for an autologous haemopoietic stem cell transplantation (AHSCT). After 2 cycles of R-DHAP regimen, successful CD34- positive stem cell collection was performed in August 2012. In September 2012, a RLAM-BEAM conditioning was performed followed by AHSCT. Posttransplantation 18FDG-PET/CT scan revealed further morphological regression, no symptom of the underlying disease appeared and the patient is in complete remission for 15 months. CONCLUSIONS - This case exemplifies that in case of atypical clinical findings and unusual progress of the disease it might be worthwile to re-evaluate the case and the (histological) diagnosis, which requires a close cooperation between the clinician and the pathologist.]