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Clinical Neuroscience

SEPTEMBER 30, 2017

[The Multiple Sclerosis Registry of Szeged]

BENCSIK Krisztina, SANDI Dániel, BIERNACKI Tamás, KINCSES Zsigmond Tamás, FÜVESI Judit, FRICSKA-NAGY Zsanett, VÉCSEI László

[Multiple sclerosis (MS) is a rare disease of the central nervous system considering the total population, the prevalence in Hungary is 83.9/100.000. The first MS registry was established in Denmark in the middle of the 1950’s. This was followed by the establishment of several national, then international databases with the number of enrolled patients in the hundred-thousands. At the beginning, the primary goal of the registries were the epidemiological surveys, focusing on the number of patients, the prevalence, the incidence, the mortality and the co-morbidity. As of today, however, with the rapid advancement and development of new disease modifying therapies (DMT) with different effectiveness and adverse reactions, the therapeutic use of the registries became even more essential: the modern, up-to-date, well established registries become integral part of the DMTs’ monitorization. The Multiple Sclerosis Registry of Szeged was first established as a “paper-based” database, then, in 2012, it was upgraded to an electronic, easily contactable and useable internet-based registry. As of today, it contains the socio-demographic and clinical data of more than 600 patients; we constantly add new patients as well as keep the registry up-to-date with the refreshment of old patients’ data. Aside from the “classical” clinical data, it can be used for the recording and assessment of the MRI scans and the data on psychopathological and quality of life assessments, which are becoming more and more important in everyday MS management. The establishment of the internet-based registry incredibly helped both the monitorization of the effectiveness of DMTs, and the success of the new epidemiological and psychopathological surveys. ]

Clinical Neuroscience

SEPTEMBER 30, 2017

[Childhood sporadic type of hemiplegic migraine with arteria cerebri media hypoperfusion]

NAGY Csaba, BAJZIK Gábor, SKOBRÁK Andrea, CSORBA Eszter, LAJTAI Anikó, BALOGH Gábor, NAGY Ferenc, VAJDA Zsolt

[Hemiplegic migraine is a rare subtype of migraine that is associated with reversible motor weakness in the aura phase. This is an uncommon form of migraine usually starting in childhood. The purpose of this case report is to highlight the differential diagnostic difficulty of the first attack. We describe a case, where the fluctuating unilateral motor weakness and aphasia suggested that the patient had ischaemic stroke. Nevertheless the brain MRI and MR angiography, the measured 5-hydroxyindole acetic acid (5-HIAA) concentration changes and the spontaneously improving clinical status proved the diagnosis of hemiplegic migraine. The MRI and MR angiography was very beneficial in establishing the correct diagnosis in this case. To distinguish between the familiar and sporadic type of hemiplegic migraine further genetic tests can be carried out.]

Clinical Neuroscience

JULY 30, 2017

[Is second-line immunomodulatory treatment effective in multiple sclerosis?]

RÁCZ Lilla, BERÉNYI Ervin, BARSI Péter, BERNÁTH Dávid, CSÉPÁNY Tünde

[Purpose - Natalizumab is the first evidence based monoclonal antibody, which was launched for treatment in relapsing remitting multiple sclerosis in Hungary in 2010. Standardized follow-up is required to use it. Our aim was to evaluate the efficacy and to monitor the safety of natalizumab treatment by using an electronic database established for MS registry. Clinical activity was measured by annual relapse rates, functional status of patients measured by EDSS and MFSC. Radiological activity was evaluated by standard MRI protocol. Data, results of MS patients and side effects of natalizumab treatment were recorded in iMed software. Results - 31 patients started the natalizumab treatment after 6.5±5.8 years from the onset of MS. The efficacy of treatment was evaluated after a mean of 67 (min: 14 max: 128) infusions in December 2016. The drop-out rate was low, due to the presence of neutralising antibodies in one case, pregnancy in two cases and development of malignant disease in one case which was not related to the natalizumab treatment. The treatment was well tolerated with excellent compliance without serious side effects. The annual relapse rate reduced from a mean of 1.7 to 0.03 (p<0.000001) in the first 12 months of treatment compared to the pretreatment 12 month activity, and it stayed at low level during the whole follow up. EDSS was stable or improved with an exception of two cases. In 23 subjects (77%) lack of new/enlarging T2 lesions and lack of gadolineum-enhancing lesions on MRI were observed. 18 patients (60%) had no evidence of disease activity (NEDA-3). PASAT test improved in most of the cases. Conclusion - The natalizumab therapy was very effective in all cases including those patients who had active disease under the previous immunomodulatory treatment.]

Clinical Neuroscience

MAY 30, 2017

The role of the insula in the parieto-frontomedial epileptic network. Clues from successful surgical treatment

BALOGH Attila, AIMEN Anwar, KELEMEN Anna, ERÕSS Loránd, FABÓ Dániel

We present a case of MRI negative SMA seizure with the seizure onset zone in the secondary leg area on the superior bank of the Sylvian fissure, localized with multiscale electro-clinical and neuroradiological examinations. The 34-year-old female patient’s intractable epilepsy started at age 14. She had diffuse pain aura in her left leg followed by tonic posturing with fully preserved consciousness suggesting parieto-fronto-medial seizure propagation. Her daily nocturnal SMA seizures became drug-resistant. Multiple 3T MRI images and neuropsychological evaluations were normal. Interictal PET detected a right parietal and insular FDG hypometabolism. The seizure onset zone and the symptomatogenic zone were localized by invasive electrophysiology. The insular deep electrode showed the propagation of ictal activity with an onset in the secondary sensory leg area through the insula to the fronto-medial surface. Eighteen spontaneous seizures, electrical cortical stimulation and cortical mapping confirmed the designated area of the resection, which was later proved macroscopically abnormal during surgery. The histological and immunohistological workup confirmed focal cortical dysplasia (IIb type). Postoperative postprocessing morphometry of the preoperative MRI study confirmed the lesion in the right inferior parietal lobe. The patient remained seizure free after surgery for more than 4 years, and medication free for the last two years. Our results concluded that the insula has a „relay” or „node” function in the parieto-opercular-fronto-medial epileptic network. The insular functional connectivity predisposed frontal propagation of the epileptic activity in the connectome of her epilepsy. The three-way insular structural connectivity has determining function on the seizure propagation.

Clinical Neuroscience

MARCH 30, 2017

Pazopanib induced unilateral posterior reversible encephalopathy syndrome

ARSLAN Beyza Muhsine, BAJRAMI Arsida, DEMIR Elif, CABALAR Murat, YAYLA Vildan

Posterior reversible encephalopathy syndrome (PRES) is a reversible clinical and neuroradiological syndrome which may appear at any age and characterized by headache, altered consciousness, seizures, and cortical blindness. The exact incidence is still unknown. The most commonly identified causes include hypertensive encephalopathy, eclampsia, and some cytotoxic drugs. Vasogenic edema related subcortical white matter lesions, hyperintense on T2A and FLAIR sequences, in a relatively symmetrical pattern especially in the occipital and parietal lobes can be detected on cranial MR imaging. These findings tend to resolve partially or completely with early diagnosis and appropriate treatment. Here in, we present a rare case of unilateral PRES developed following the treatment with pazopanib, a testicular tumor vascular endothelial growth factor (VEGF) inhibitory agent.

Clinical Neuroscience

JANUARY 20, 2017

[Magnetic resonance imaging in the course of alemtuzumab and teriflunomide therapy]

MIKE Andrea, KINCSES Zsigmond Tamás, VÉCSEI László

[Our work aimed to review the published results of magnetic resonance imaging (MRI) obtained in the course of alemtuzumab and teriflunomide therapy in multiplex sclerosis. In multiplex sclerosis MRI sensitively detects subclinical pathological processes, which do not manifest clinically in the early course of the disease, however have substantial significance from the viewpoint of the long-term disease prognosis. MRI has an increasingly important role in the early monitoring of the therapeutic efficacy. In the last 15 years several clinical trials have been conducted with alemtuzumab and teriflunomide in multiple sclerosis providing evidence about the favourable clinical effect of these drugs. MRI images were acquired in these trials as well, and the results published recently in the scientific literature. These MRI results denote the suppression of the disease activity and the neurodegenerative processes, which may imply a favourable effect on the long-term prognosis of the disease. ]

Clinical Oncology

SEPTEMBER 05, 2015

[Up-to-date treatment of head and neck cancers]

PACZONA Róbert, CSENKI Melinda, HIDEGHÉTY Katalin

[The head and neck squamous cell cancer is often detected at an advanced stage, resulting in dismal prognosis despite the relevant developments of the oncotherapy in the last decades. The introduction of new techniques, new drugs and combinations though improves the survival for certain subgroups of patients, meanwhile the organ-function preservation and side effects reduction approaches lead to improved quality of life. The preventive-supportive care prior and during the therapy (dental care, nutrition, toxicity management) and the complex rehabilitation has paramount importance. MRI and 18FDG PET-CT and the advanced methods of molecular pathology became part of the diagnostic work up. The selection of the therapy based on the tumor characteristics, HPV detection, on the patient’s condition and wishes, as well as on multi-disciplinary team decision based on the available technical options. Early stage tumors can be successfully treated either by surgery alone or by radiation therapy. For locally advanced cases concomittant chemo-radiotherapy stays in the axis of the complex management using advanced radiation technique (IGRT-adaptive- IMRT) with cisplatin (or if contraindicated with cetuximab), which could be complemented by induction chemotherapy and/or surgery. Several new approaches are currently being tested in clinical studies after establishment of cisplatine-cetuximab treatment for recurrent/metastatic tumors. In the future, detection of molecular processes and driver mutations could result in development of more effective targeted anti-tumor agents, and individual molecular tumor profi le guided therapy, including the various forms and combinations with emerging immunotherapy.]

Clinical Neuroscience

NOVEMBER 30, 2016

Unilateral thalamic infarction causing downward gaze palsy in a patient with uncorrected tetralogy of fallot: a case report

ANNUS Ádám, BENCSIK Krisztina, JÁRDÁNHÁZY Tamás, VÉCSEI László, KLIVÉNYI Péter

Introduction - Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD). Adults with surgically uncorrected forms of this condition are extremely rare, since operation is recommended in childhood to prevent cyanosis. Cyanotic CHD increases the risk of thromboembolic events. An endothelial dysfunction caused by chronic hypoxia and shear stress due to rheological alterations with a platelet dysfunction appear to be the explanation behind this finding. Paramedian thalamic infarction causing vertical gaze palsy without midbrain involvement is an infrequent finding. We report here a rare case of a patient with untreated TOF, who suffered a left-sided unilateral thalamic infarction presenting as downward gaze palsy and diplopia. Case presentation - A 44-year-old women complained of sudden onset diplopia and vertigo. Neurological examination revealed a downward gaze palsy with other symptoms related to a vertebrobasilar territory circulatory disturbance. The MRI scan revealed an acute infarction, 8 mm in diameter in the left medial thalamic region without midbrain involvement. Discussion - Adults with uncorrected forms of TOF are extremely uncommon, and descriptions of stroke in these patients are therefore rarities. We set out to give a concise survey of the literature regarding TOF patients with stroke. Conclusion - We present a rare case of unilateral thalamic infarction causing downward gaze palsy in an adult patient with uncorrected TOF. Cyanotic CHD is regarded as one of the risk factors of stroke. Besides other pathologic conditions, ischaemic stroke at an early age should raise the suspicion of a cardioembolic origin and, in rare cases, might result from cyanotic CHD.

Clinical Neuroscience

JULY 30, 2016

Gray matter atrophy in presymptomatic Huntington’s patients

KIRÁLY András, KINCSES Zsigmond Tamás, SZABÓ Nikoletta, TÓTH Eszter, CSETE Gergő, FARAGÓ Péter, VÉCSEI László

Background - Huntington’s disease is a progressive disease in which neurodegeneration is on-going from the early presymptomatic phase. Development of sensitive biomarkers in this presymptomatic stage that are able to monitor the disease progression and test the efficacy of putative neuroprotective treatments are essential. Methods - Seven presymptomatic Huntington mutation carriers and ten age-matched healthy controls were recruited. Six of the patients participated in a 24 months longitudinal study having MRI scans 12 and 24 months after the baseline measurements. High resolution T1 weighted images were carried out and voxel based morphometry was used to analyse the data. Apart of group differences, correlation of CAG repeat number with focal cortical thickness and with global gray matter volume was calculated. Results - Focal cortical atrophy was found bilaterally in the superior temporal sulcus and in the left middle frontal gyrus in presymptomatic Huntington patients in whom no sign of cognitive or motor deterioration was detected. Global gray matter atrophy (p<0.048) and decreased total brain volume was found. The number of CAG triplets showed no correlation with the focal gray matter atrophy and total brain volume. Strong correlation between the CAG repeat number and global gray matter volume was found (p<0.016). Conclusion - Cortical atrophy is apparent in the early, presymptomatic stage of the disease. With further validation in large patient sample atrophy measure could be biomarker of disease progression and putatively of neurodegeneration.

Clinical Neuroscience

JULY 30, 2016

Burning mouth syndrome: Evaluation of clinical and laboratory findings

HALAC Gulistan, TEKTURK Pinar, EROGLU Saliha, CIKRIKCIOGLU Ali Mehmet, CIMENDUR Ozlem, KILIC Elif, ASIL Talip

Background and purpose - Burning mouth syndrome is a chronic and persistent painful condition characterized by burning sensation in the oral mucosa. We investigated the etiological factors of patients presented with the history of burning in the mouth who admitted our outpatient clinics over the 8-years period and who had no underlying identifiable local factors. We also tried to determine their demographic and clinical characteristics. Our aim was to investigate the association between burning mouth and psychiatric disorders such as depression and anxiety, chronic diseases like diabetes mellitus (DM) and other laboratory studies in patients complaining of solely burning in the mouth. Methods - The study included patients with the history of burning in mouth who presented in our outpatient clinic between 2005 and 2012. They were evaluated by a neurologist, a psychiatrist, an internist, and a dentist. Complete blood counts, biochemical analysis and cranial magnetic resonance imaging (MRI) were performed for all patients. Results - A total of 26 (22 (84%) females, 4 (15%) males; mean age 55.9 years) patients were enrolled in this study. Five (19.2%) of the patients had depression, 2 (7.7%) had anxiety disorder, 2 (7.7%) had diabetes mellitus, 8 (30%) had B12 vitamin deficiency, 3 (11.5%) had decreased ferritin levels in blood, and 1 (3.8%) had folic acid deficiency. Cranial MRI of all patients were normal. Nine patients (34.6%) had no etiological causes. Conclusion - A multidisciplinary approach in the management of burning mouth and establishment of common criteria for the diagnosis would provide insight into the underlying pathophysiological mechanism.