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Clinical Neuroscience

JULY 30, 2019

A case report of Morvan syndrome

AYTAC Emrah, ACAR Türkan

Morvan syndrome is a rare disease characterized by peripheral nerve hyperexcitability, encephalopathy, dys­autonomia and significant insomnia. The patient, who was included in the present study, was followed-up at our clinics for confusion, myokymia, hyperhidrosis, epileptic seizures, tachycardia, agitation, hypokalemia, and hyponatremia. The cranial MRI of the patient demonstrated hyperintensities at the T2 and FLAIR sections of the medial temporal lobe and insular lobes. Electromyography and neurotransmission examination results were concordant with peripheral nerve hyperreactivity. Contactin-associated protein-like 2 antibodies and leucine-rich glioma inactivated protein 1 antibodies were detected as positive. The patient was diagnosed with Morvan syndrome; intravenous immunoglobulin and corticosteroid treatment was started. Almost full remission was achieved. This very rare syndrome implies challenges in diagnosis and treatment; however, remission can be achieved during the follow-up. In addition, caution is needed in the long-term follow-up of these patients regarding the development of malignancies.

Clinical Neuroscience

SEPTEMBER 30, 2016

[Operation - rehabilitation - employment]

MÉSZÁROS Gabriella, FEHÉR Miklós, BORBÉLY Csaba

[In this article we would like to bring the attention to the importance of early rehabilitation, coordinated operation of different subdivisions of rehabilitation and the expanding opportunities after medical care through following a 38- year-old female patient's case who was operated 6 years ago with anaplasticus oligodendroglioma. We find it important that the experts working on the field of health care, social services or labour should be aware of the possibilities of rehabilitation of their patients from the capability assessment, through its development until their placement in integrated labour market. It is important, that even during the medical care both the client and their relatives receive proper information.]

Clinical Neuroscience

JULY 30, 2015

[LGI1 encephalitis: the first Hungarian patient]

SZŐTS Mónika, MARTON Annamária, ILLÉS Zsolt, BAJZIK Gábor, NAGY Ferenc

[In the recent years, it has been increasingly recognised that in a group of limbic encephalitis antibodies are directed against the scaffolding protein LGI1 (Leucine-rich glioma inactivated 1), which is part of the voltage gated potassium channel (VGKC) complex on neural synapses. Patients present with seizures and subacute history of neuropsychiatric symptoms, including psychosis and changes in memory, cognition, behaviour. Faciobrachial dystonic seizures can be observed, which are highly characteristic for LGI1 encephalitis. MRI shows medial temporal abnormalities in more than half of the cases. CSF evaluation is usually normal. Hyponatremia is frequently associated and may confuse the initial diagnosis. Early recognition and prompt initiation of immunotherapies are of great importance. The clinical improvements often correlate with the antibody levels. We present the case of a 64-year old man, who responded quickly to plasma exchange and major improvement was noted within few weeks.]

Clinical Neuroscience

OCTOBER 05, 2013

[Occurence and molecular pathology of high grade gliomas]

MURNYÁK Balázs, CSONKA Tamás, HEGYI Katalin, MÉHES Gábor, KLEKNER Álmos, HORTOBÁGYI Tibor

[Background - Glial tumours represent the most frequent type of primary brain cancers. Gliomas are characterized by heterogeneity that makes the diagnosis, histological classification and the choosing of correct therapy more difficult. Despite the advances in developing therapeutic strategies patients with malignant gliomas have a poor prognosis; therefore glial tumours represent one of the most important areas of cancer research. There are no detailed data on the epidemiology of gliomas in Hungary. Methods - In the first section of our publication, we analysed the histological diagnosed cases between 2007 and 2011 at the Institute of Pathology, University of Debrecen Medical and Health Science Centre. We analyzed the incidence of 214 high-grade gliomas by tumor grades, gender, age, and the anatomical localization. Results - The majority of cases were glioblastoma (182 cases), and the remaining 32 cases were anaplastic gliomas. The mean age of patients was 57 years (±16.4), and the male:female ratio was 1.1:1. The most frequent area of tumors was the frontal lobe followed by the temporal, parietal and occipital lobe. We include new findings published recently about glioma patogenesis, molecular pathways, mutant genes and chromosomal regions. We explain briefly the role of selected important genes in glioma genesis and give an update on knowledge provided by modern molecular methods, which could beneficially influence future therapy and the diagnosis of gliomas.]

Clinical Neuroscience

OCTOBER 05, 2013

[Occurence and molecular pathology of low grade gliomas]

MURNYÁK Balázs, CSONKA Tamás, KLEKNER Álmos, HORTOBÁGYI Tibor

[Background - The WHO grade I. and II. low-grade gliomas represent nearly the 15% of all primary brain tumors. These tumours contain clinically, hisologically and molecularly distinct tumor types. According to their histologic characteristic, grade II glial tumours are the diffuse astrocytoma, oligodendroglioma and oligoastrocytoma subgroups; the ependymal tumors are not included in this study. Methods - In our publication, we analysed the histological diagnosed glioma cases between 2007 and 2011 at our institution. Results - Low-grade gliomas were diagnosed in 127 cases (62 male / 65 female), and the mean ages were 39 years (±20.3). More than half of the cancers were localizated in the frontal lobe, and the second most frequent area was the temporal lobe. Finally, we comlete our report with an overview of major molecular pathways in low-grade gliomas.]

Clinical Neuroscience

SEPTEMBER 30, 2012

[Role of the intraoperative electrical brain stimulation in conserving the speech and language function in neurosurgical operations of awake patients]

ERÕSS Loránd, FEKETE Gábor, ENTZ László, FABÓ Dániel, BORBÉLY Csaba, KOZÁK Lajos Rudolf, ANDREJKOVICS Mónika, CZIRJÁK Sándor, FEDORCSÁK Imre, NOVÁK László, BOGNÁR László

[Aim of the study - To summarize the results gained with awake craniotomies, which were performed in either low grade glioma patients or epilepsy surgical patients whose tumor or epileptogenic zone, was in the vicinity of eloquent, mostly language, cortices. Patient selection and methods - In our retrospective study we selected 16 patients who were operated awake between 1999-2011 at the Neurosurgical Department of MÁV Kórház Budapest, or at the National Institute of Neurosciences in Budapest, or at the Neurosurgical Department of the University of Debrecen in Debrecen. In the presurgical evaluation if it was possible we performed functional magnetic resonance imaging, tractography and detailed neuropsychological testing. At the National Institute of Neurosciences all patients were operated with the aid of MR guided neuronavigation. Results - Anesthesia was carried out without complications in all of the 16 cases. Monitoring of sleep deepness has significantly contributed to the safety of anesthesia during the superficial anesthezied states of the operation. The intraoperative neuropsychological tasks used for testing language were sensitive enough to judge the little disturbances in speech during stimulation. Stimulation evoked seizures could be adequately managed during surgery and did not influence the outcome of the procedures. The use of neuronavigation helped significantly by planning the optimal place for the craniotomy and by intraoperative orientation. Conclusions - Awake craniotomies require well practiced surgical teams, which requires the cooperation of neuro-anesthesiologits, neurosurgeons, neuropsychologist and electrophysiologists. It has two goals, first to reduce the time of surgery to minimize surgical complications, secondly the detailed intraoperative mapping of cognitive and motor functions to avoid any neurological deficit. The intraoperative anatomical data provided by the neuronavigation and the functional data provided by awake intraoperative stimulation of the patient together serve the safety of the patient which is essential in the neurologically minimal invasive neurosurgical approach of the 21st century.]

Clinical Neuroscience

JANUARY 30, 2012

[The clinical relevance of 1p19q codeletion of oligodendrogliomas at the Department of Neurosurgery in Debrecen]

KLEKNER Álmos, FEKETE Gábor, RENCSI Márta, MÉHES Gábor, SZABÓ Péter, BOGNÁR László

[Object - To determine the clinical relevance of 1p19q codeletion in case of patients treated between 2006 and 2008 with oligodendroglial tumor at the Department of Neurosurgery, University of Debrecen. Questions - Beside the traditional morphological investigations, methods of rapidly developing molecular pathology are also available for routine diagnostic procedures. Numerous studies confirm that the codeletion of the 1p and 19q alleles has a clinical relevance regarding the sensitivity for chemotherapy. In this study the connection between the 1p19q codeletion and clinical parameters was tested to evaluate the prognostic role of this genetic alteration in neurosurgical patients. Methods, clinical data - In the present study experiences about the clinical relevance of 1p19 codeletion is summarized. Between 2006 and 2008, 28 patients with brain tumor containing oligodendroglial component was tested for 1p19q codeletion. The result of the analysis was compared with clinical data such as tumor localization, extent of resection, histological grade, presence of astrocyte component, time of first recurrence, age and gender. Furthermore, the potency of 1p19q codeletion as a prognostic factor for chemosensitivity by analyzing the data of patients who underwent different treatment protocols was also evaluated. Results - Our results suggest that 1p19q codeletion can be valued as a positive prognostic factor, which is concordant with the results available in the literature. We also found positive correlation with oligodendroglial component, recurrence free survival of grade III tumors, sensitivity to chemoand radiotherapy, and inverse correlation with histological grade and age was detected. Conclusion - Though the 1p19q codeletion is currently not the part of the routine patient management, based on our study we found it appropriate for clinical use as a prognostic factor, and its predictive role in establishing oncotherapy can be also discussed.]

Clinical Neuroscience

FEBRUARY 10, 2004

[Brachytherapy of brain stem tumours]

JULOW Jenő, VIOLA Árpád, MAJOR Tibor, VALÁLIK István, SÁGI Sarolta, MANGEL László, KOVÁCS Rita Beáta, REPA Imre, BAJZIK Gábor, NÉMETH György

[Introductions - The optimal therapy of brain stem tumours of different histopathology determines the expected length of survival. Authors report 125Iodine interstitial irradiation of brain stem tumours with stereotactic brachytherapy. Case reports - Two patients having brain stem tumours were suffering from glioma or from metastases of a carcinoma. In Case 1 the tumour volume was 1.98 cm3 at the time of planning interstitial irradiation. The control MRI examination performed at 42 months post-op showed a postirradiation cyst size of 5.73 cm3 indicating 65.5% shrinkage. In Case 2 the shrinkage was more apparent as the tumour vo-lume measured on the control MRI at 8 moths post-op was only 0.16 cm3 indicating 97.4% shrinkage of the 6.05 cm3 target volume at the time of brachytherapy with the metastasis practically disappearing. Quick access to histopathological results of the stereotactic intraoperative biopsy made it possible to carry out the 125Iodine stereotactic brachytherapy immediately after the biopsy, resulting in less inconvience for patients of a second possible intervention. The control MRI scans show significant shrinkage of tumours in both patients. Conclusion - The procedure can be performed as a biopsy. The CT and image fusion guided 125Iodine stereotactic brachytherapy can be well planned dosimetrically and is surgically precise.]

Clinical Neuroscience

MARCH 30, 2008

[DYNAMICS OF THE TRIPLE RING, VOLUMETRICAL ANALYSIS, PREDICTIONS FOR TREATMENT PLANNING]

KOLUMBÁN Zsuzsa, VIOLA Árpád, MAJOR Tibor, BAJZIK Gábor, JULOW Jenő

[Objective - The aim of this study is to reveal the volumetrical changes in tumor necrosis, reactive zone and edema following low-dose rate I-125 interstitial irradiation of 20 inoperable (partially irresecable, partially inoperable) lowgrade gliomas. Methods - The volumes of the three regions on imagefused control CT/MRI images were measured for a 24- month period with 36 occasions. The delivered dose on the tumor surface (GTV) was 50-60 Gy. Dose planning and image fusion were performed with the BrainLab Target 1.19 software, mathematical and statistical computations were carried out with the Matlab Numeric Computation and Visualization software. The control images with the „triple ring” were fused with the planning images, and the isodose curves were adjusted to them. Results - Relative volumes normalized to volume of the reference dose were calculated and plotted in the time domain. The mean values of volumes were determined from the patients' measured data then a polynom was fitted to the mean values using the polynomial curve fitting method. The accuracy of our results were verified by statistical tools. Conclusions - The new polynomial prediction approach using image fusion analysis of the volume of tumor necrosis, reactive ring and edema caused by interstitial irradiation as a function of time provides valuable information for 1. selecting the best patient’s treatment option, 2. following up patient’s condition and 3. planning reirradiation or reoperation if necessary.]

Clinical Neuroscience

FEBRUARY 20, 2002

[Chemotherapy of recurrent supratentorial malignant gliomas (Phase II study)]

ÁFRA Dénes, SIPOS László, VITANOVICS Dusan

[At the Hungarian National Institute of Neurosurgery 73 recurrent supratentorial malignant tumours were treated by chemotherapy during the last ten years. Chemotherapy was applied after postoperative radiotherapy but in some cases following reoperation only. All cases were clinically and by CT or MRI verified recurrences. Forty-three patients received BCNU-DBD (dibromodulcitol) treatment (23 anaplastic astrocytoma - AA, and 20 glioblastoma multiforme - GM): day 1. BCNU 150 mg/sq.m. in iv. infusion, day 2. dibromdulcitol 1000 mg/sq orally was given. This course was repeated every six weeks, altogether 2-8 times. Sixteen patients with AA responded with complete or partial regression but only 6 did with GM. Median survival was 14 and 7 months, the difference proved to be significant, p=0.0091. PCV combination (procarbazine, CCNU, vincristine) was applied to 16 patients with AA and 14 cases with recurrent oligodendroglioma (O). Treatment started with vincristine 1.5 mg/sq. m. iv. (2.0 mg maximum), the next day CCNU 100 mg/sq.m. was given, followed by procarbazine 60 mg/sq.m. on days 8-22. and finished by the same dose of vincristine on day 30. The course was repeated after one month, mostly six times. Six patients with AA did not respond; in cases of oligodendroglioma all but one responded with complete or partial improvement. It is remarkable that no significant difference was found between the survivals of BCNU-DBD or PCV treated AA patients. Chemotherapy of supratentorial malignant glioma recurrences with nitroso-ureas and their combination proved to be efficacious. It also seems, that in recurrent cases lower grade gliomas show better response rate than glioblastomas.]