Clinical Neuroscience

[The role of MRI in measuring the effectivity of disease modifying treatments II]

KINCSES Zsigmond Tamás1,2, TÓTH Eszter1, FRICSKA-NAGY Zsanett1, FÜVESI Judit1, RAJDA Cecília1, BENCSIK Krisztina1, VÖRÖS Erika2, CSOMOR Angéla2, PALKÓ András2, VÉCSEI László1,3

MARCH 30, 2018

Clinical Neuroscience - 2018;71(03-04)

DOI: https://doi.org/10.18071/isz.71.0081

[The paraclinical examinations, principally the MRI have an increasing significance in the diagnosis of multiple sclerosis. However, MRI markers also have a prominent role in monitoring of the disease-course and activity, and also in the planning of possible therapeutic changes. In accordance with previously published international guidelines, in this article we propose a protocol for the monitoring the treatment efficacy in multiple sclerosis. This could be the basis of a consensus based guideline to be implemented in Hungary.]

AFFILIATIONS

  1. Szegedi Tudományegyetem, Neurológiai Klinika, Szeged
  2. Szegedi Tudományegyetem, Radiológiai Klinika, Szeged
  3. MTA-SZTE, Idegtudományi Kutatócsoport, Szeged

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Clinical Neuroscience

A rare aetiology of stroke; myxomatous aneurysm caused by atrial myxoma

ACAR Erkan, OZDEMIR Zeynep, SELCUK Hakan Hatem, ÇOBAN Eda, SOYSAL Aysun

Atrial myxoma is a rare cause of stroke. In this report we present the case of a 52-year-old female patient who went to hospital suffering from a headache. Her neurological examination was normal except for a positive Babinski sign on the left. In the superolateral of the right, a Sylvian fissure consistent with a thrombosed aneurysm was detected using computerised tomography (CT). Diffusion MRI showed an acute infarction on the right MCA area. Transthorasic Echocardiography and ECG were normal. A -16×4 mm-sized fusiform perpendicular aneurysm on the M2 segment Sylvian curve of right MCA and a -6×4 mm-sized dissecting aneurysm on P3 segment of the left posterior cerebral artery (PCA) were observed in cerebral angiography. Transesophageal echocardiography (TEE) demonsrated a large mass with a suspected size of 2×2×1.5 cm on the left atrium. The mass was resected and on the eighth day after the operation, she had a temporary vision loss and hyperintensity on the T1 sequence was interpreted as laminary necrosis suspected on Cranial MRI. In follow up, she was stable with 300mg acetylsalicylic acid treatment. The main treatment is surgical resection in stroke caused by atrial myxoma.

Clinical Neuroscience

[The role of MRI in measuring the effectivity of disease modifying treatments I]

TÓTH Eszter, BENCSIK Krisztina, VÖRÖS Erika, FRICSKA-NAGY Zsanett, FÜVESI Judit, RAJDA Cecília, CSOMOR Angéla, PALKÓ András, VÉCSEI László, KINCSES Zsigmond Tamás

[MRI has a significant role in the diagnosis of multiple sclerosis. The newer and newer treatment options of the disease make it necessary to monitor the effectiveness of the therapy. Besides the clinical signs (clinical relapses and progression), the different MRI parameters can also reflect the disease activity. In our current article we summarize those MRI markers, which best predict the long-term disability, based on the international standards.]

Clinical Neuroscience

Long-term follow-up results of concomitant chemoradiotherapy followed by adjuvant temozolomide therapy for glioblastoma multiforme patients. The importance of MRI information in survival: Single-center experience

LUKÁCS Gábor, TÓTH Zoltán, SIPOS Dávid, CSIMA Melinda, HADJIEV Janaki, BAJZIK Gábor, CSELIK Zsolt, SEMJÉN Dávid, REPA Imre, KOVÁCS Árpád

Introduction - Glioblastoma multiforme (GBM) is the most common malignant primary anomaly of central nervous system. The GBM infiltrates the nearly sturctures from the initial tumor and its metastatic attribution is well known. The aim of our single-centered retrospective study was to introduce the importance of postoperative medical imaging confirmation of total tumor resection for patient with GBM combined concomitant and adjuvant chemoradiotherapy on a 10 year long patient follow up. Methods - From January 2006 to April 2015 we registered 59 patients with newly diagnosed GBM at the University of Kaposvár Health Center Institute of Diagnostic Imaging and Radiation Oncology. The histological diagnosis was confirmed by a proficient neuropathologist (World Health Organisation WHO; grade IV astrocytoma). According to histological status if the ECOG performance status of patients allowed it the mutidisciplinary oncoteam recommended adjuvant chemoradiotherapy all features strictly by Stupp protocol. (60 Gy dose on the gross tumor volume and 2-3 cm margin for the clinical target volume with parallel 75 mg/m2 TMZ. Four weeks after monotherapial phase patients had to recieve 6 cycles of TMZ first cycle with 150 mg/m2 up to 200 mg/m2). The irradiation was carried out by a conformal three dimensional planning system. Results - 59 patients with the median age of 63 (range 17-84) year. Our sample counted 34 male patients and 25 woman patients. 14 patients underwent gross total tumor resection while, 39 patients underwent partial resection and the rest from our sample 6 patients passed through biopsy. Statistical analysis showed a lengthier survival among males than females, with a median survival of 13 months for males and females, the OS of 26.209 for males, meanwhile 15.625 for females. However, the difference is not considerable (log-rank p=0.203). Our study found that the estimated survival of patients at least 50 years old is significantly shorter at a median survival of 12 months (log rank p=0.027) than that of patients below 50 years of age at a median survival of 23 months. The longest estimated median survival was calculated with patients of ECOG '0' condition (16 months). However, no significant difference was found in the estimated survival of patients of different ECOG conditions (log-rank p=0.146). Based on the extent of surgery, complete resection resulted in the longest average survival of 36.4 months, followed by 21.5 months among patients with biopsy, and 15.8 months among patients with partial resection. Different surgical procedures, however, did not result in significant differences in survival (log-rank p=0.059). The overal survival of patients who had complete resection confirmed by MRI compared with the overal survival of patients with residual tumor confirmed by MRI as well we can estimate that there is significant difference between these two groups (p=0,004). Conclusion - Despite complex and intense treatment, recurrence is inevitable and causes relatively rapid death. In our analysis complete resection, as defined from the neurosurgeon’s report and postoperative MRI, resulted in an independently significant improvement in OS. Our results are the evidences that the treatment of patients with glioblastoma multiforme in Hungary is at least on the same level as any other developed European countries.

Clinical Neuroscience

[Schizotypal traits and verbal creativity]

FEJES Nikolett Éva, RÓZSA Sándor, MUST Anita

[Introduction - The relationship of schizophrenia and its milder, subclinical forms, with creativity has been in the centre of theoretical interest for decades, however, the systematic research of the topic only prevailed more recently. Purpose - Here we aimed to examine the connection between different schizotypal and non-schizotypal personality traits and verbal creativity in a nonclinical sample. We also investigated the correspondence of two schizotypy inventories, the Oxford-Liverpool Inventory of Feelings and Experiences and a special character configuration of the Temperament and Character Inventory associated with schizotypy. Method - 57 healthy adults (14/43 m/f, mean age 21.51±1.43 years) - took part in the experiment. All participants received a detailed information sheet and gave informed consent prior to participation. Participants completed the Oxford-Liverpool Inventory and the Temperament and Character Inventory to measure both schizotypal and non-schizotypal personality traits. Torrance Test of Creative Thinking was used to measure verbal creativity. Associations between reported measures were examined with correlational and regression analyses. Results and conclusion - Out of the specific Temperament and Character configuration associated with schizotypy (low self-directedness, cooperativeness and high self- transcendence), we only found low self-directedness to be correlated significantly with Oxford-Liverpool schizotypy rates (Self-directedness-schizotypy: r=-.730, p<.01). There was no significant connection between schizotypal traits and verbal creativity. In our sample, the Self-directedness and Reward-dependence character and temperament subscales predicted significantly the verbal creativity level (Self-directedness: b=.330, p=.015; Reward - dependence: b=-.260; p=.049). Based on our results, besides schizotypal traits, other personality measures might be considered in relation to verbal creativity, providing further details to the empirical investigation of creativity. We found low self-directedness to be correlated with Oxford-Liverpool schizotypy rates, however, the sample size was not large enough to test the concurrent validity of the two inventories. Future studies might consider to extend the study sample, preferably to both clinical and non-clinical populations.]

Clinical Neuroscience

A case with angiographic demonstration of isolated anterior spinal artery occlusion

DOGAN Burcu Vasfiye, KARA Batuhan, SELCUK Hatem Hakan, DIRICAN Ceyhan Ayten, KOKSAL Ayhan

Anterior spinal artery syndrome (ASAS) is a rare syndrome which occurs due to thrombosis of anterior spinal artery (ASA) which supplies anterior two thirds of the spinal cord. A 27-year-old female patient was admitted to emergency clinic with sudden onset neck pain, sensory loss and weakness in proximal upper extremities which occurred at rest. Thrombophilia assessment tests were negative. Echocardiography was normal. Serum viral markers were negative. In cerebrospinal fluid (CSF) examination, cell count and biochemistry was normal, oligoclonal band was negative, viral markers for herpes simplex virus (HSV) type-1 and type-2, Brucella, Borrellia, Treponema pallidum, Tuberculosis were negative. Diffusion restriction which reveals acute ischemia was detected in Diffusion weighted MRI. Digital subtraction angiography (DSA) was performed. Medical treatment was 300mg/day acetilsalycilic acid. Patient was discharged from neurology clinics to receive rehabilitation against spasticity.

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[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

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[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

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[Myelination disturbance in a patient with hyperuricemia and hyperserotoninemia combined with 18q deletion syndrome]

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[We previously reported a male patient with an 18q21.3 deletion, hyperuricemia and typical symptoms of the Lesch- Nyhan syndrome who lacked hypoxanthine-guanine-phosphoribosyl- transferase (HGPRT) deficiency. The patient developed progressive peripheral neuropathy in additon to his profound mental retardation and self-injurious behavior. At the age of 23 years MR imaging revealed globally delayed myelination with relative sparing of the corpus callosum and frontal lobes. They were focal hyperintensities suggestive of gliosis. Multimodality evoked potentials found evidence of impaired central and peripheral conduction. Single photon emission computed tomographic (SPECT) imaging demonstrated left frontal hyperperfusion and under it a temporoparietal hypoperfusion.]

Clinical Neuroscience

[The role of MRI in measuring the effectivity of disease modifying treatments I]

TÓTH Eszter, BENCSIK Krisztina, VÖRÖS Erika, FRICSKA-NAGY Zsanett, FÜVESI Judit, RAJDA Cecília, CSOMOR Angéla, PALKÓ András, VÉCSEI László, KINCSES Zsigmond Tamás

[MRI has a significant role in the diagnosis of multiple sclerosis. The newer and newer treatment options of the disease make it necessary to monitor the effectiveness of the therapy. Besides the clinical signs (clinical relapses and progression), the different MRI parameters can also reflect the disease activity. In our current article we summarize those MRI markers, which best predict the long-term disability, based on the international standards.]

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[Osteonercosis of the femoral head during pregnancy]

KISS-POLAUF Marianna, TAKÁCS János, TASI Róbert, RÁKÓCZI István

[Osteonecrosis of the femoral head associated with pregnancy is a rare condition. Approximately 40-50 cases have been reported, the first one by Pfeifer in 1957. Avascular osteonecrosis is usually caused by factors that impair the bone’s blood supply (intraosseous arterious or venous occlusion, venous stasis, hypertension in the bone marrow). Owing to the cautious use of radiological imaging techniques during pregnancy and the limited experience with this condition, the correct diagnosis is usually made only retrospectively. Thus, total hip replacement is required in most cases. In the third trimester, MRI examination is safe to perform, and in some cases a simple X-ray should be also considered, as timely avoidance of weight-bearing and other therapeutic interventions might help to prevent the arthroplasty. Here, we would like to present a case observed and treated by us, and to overview the options that could facilitate making the correct diagnosis and finding the appropriate therapeutic program.]

Clinical Neuroscience

[Teriflunomide: new oral immunmodulant drug in therapy of multiple sclerosis]

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[Multiple sclerosis (MS) is the autoimmune, demyelinating, neurodegenerative disorder of the central nervous system (CNS). There are nine drugs available in Hungary reimbursed by the National Health Insurance Fund of Hungary (OEP) to reduce the activity of the disease, from which seven can be used as first line therapies. We have approximately 20 years of experience with the interferon b-1a/1b and glatiramer-acetate products. Though in case of approximately 30% of the patients using one of the first line drugs, the disease remains active, that we call break-through disease. The reasons for break-through disease could be the insufficient adherence and compliance, the appearance of neutralizing antibodies or the high activity of the disease. One of the oral immunomodulating drugs for MS, teriflunomide, was registered in Europe in 2013. Because of the anti-proliferative and anti-inflammatory effect of teriflunomide, it can be used for the reduction of the disease activity in the relapsing-remitting course of MS. The effect of teriflunomide was proved in one Phase II. and four Phase III. (TEMSO, TOWER, TENERE, TOPIC) studies. Teriflunomide 14 mg once daily was able to demonstrate in two consecutive placebo-controlled phase 3 clinical trials that significantly reduces the relapse rate (31.5% and 36.3%) and in both studies significantly reduces the sustained disability progression (29.8% and 31.5%) moreover delays the appearance of the clinically definitive MS in patients with clinically isolated syndrome (CIS). According to the TENERE study there were no significant differences observed between teriflunomide 14 mg and IFNb-1a s.c. in time to failure and annualized relapse rate but the treatment satisfaction domains of global satisfaction, side-effects and convenience were significantly improved with teriflunomide compared with s.c. IFNb-1a. ]