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Clinical Neuroscience

NOVEMBER 20, 2015

[A rare complication of a rare disease; stroke due to relapsing polychondritis]

KILIC COBAN Eda, XANMEMMEDOV Elimir, COLAK Melek, SOYSAL Aysun

[Relapsing polychondritis (RP) is an episodic and progressive inflammatory disease of cartilaginous structures. Its diagnosis is based primarily on clinical features such as laboratory parameters, biopsy. Neurological complications occur in 3% of the cases and are classified as an important cause of death. The cranial nerve disorders are most common but hemiplegia, ataxia, myelitis, polyneuritis, seizures, confusion, hallucination and headache can also happen. The aetiology of central nervous system involvement is still unknown. Moreover stroke has rarely reported in these patients. The diagnosis of stroke is challenging because of its rarity among these patients. Perhaps vasculitis is the common underlying mechanism. Also meningitis and encephalitis can occur during the course of RP. A 44 year-old woman was admitted with uncontemplated left hemiparesis, redness, swelling, and tenderness of the metacarpophalangeal and interphalangeal joints of the right hand and the cartilaginous portion. White blood cell count, C-reactive protein and the erythrocyte sedimentation rate were elevated. Vasculitis biomarkers were normal in our patient. Carotid and vertebral artery doppler ultrasonography, cranial and cervical MR Angiography were normal. Echocardiography showed a mild mitral valve prolapse and regurgitation. Our patient had the history of auricular polychondritis but she had not been diagnosed. Hemiparesis was her first neurological manifestation that led her to doctors for diagnosis. Our patient fulfilled the criteria of RP so no biopsy was needed. She was treated with oral prednisolone (80 mg/day) and aspirin (300 mg/day) and now she is on 10 mg prednisolone and 150 mg azathioprine. Two months later her physical and neurological symptoms returned to normal.]

Clinical Neuroscience

MARCH 30, 2016

Cerebral amyloid angiopathy related inflammation: is susceptibility weighted imaging the clue for diagnosis?

CSÉCSEI Péter, KOMOLY Sámuel, SZAPÁRY László, BARSI Péter

Background - Cerebral amyloid angiopathy-related inflammation (CAA-ri) is characterized by various neurological symptoms such as gradually developing confusion, progressive cognitive decline, seizure or headaches; T2 hyperintensities on magnetic resonance imaging (MRI); and neuropathological evidence of cerebral amyloid angiopathy (CAA) and associated vascular or perivascular inflammation. Although histological confirmation is necessary for accurate diagnosis, in case of typical clinical features and neuroimaging, the diagnosis can be established without biopsy. Case summary - We present the case of a 57-year-old man with a history of hypertension who presented to the emer¬gency department 3-week history of progressive headache and a gradually developing altered mental status. On examination, he was found to have left sided weakness and decreased pscyhomotility. Routine clinical work-up (lab investigations, CT, cerebrospinal fluid analysis) did not show obvious diagnosis, so we performed an MRI. It raised the suspicion of CAA-ri which diagnosis was verified by neuroradiological evaluation. High dose steroid treatment was initiated. The patient rapidly responded to treatment, his focal neurological signs resolved. Control MRI after 1.5 months showed multiple haemorrhagic laesions in the field of previous inflammation which posteriorly supported the previous supposed work-diagnosis. Conclusions - Although histopathology is the gold standard for the diagnosis of cerebral amyloid angiopathy, the typical clinical presentation, good response to steroids and accurate neuroradiological criteria make biopsy unnecessary to diagnose CAA-ri.

Clinical Neuroscience

JANUARY 30, 2016

Long term follow-up of lesional and non-lesional patients with electrical status epilepticus in slow wave sleep

HEGYI Márta, SIEGLER Zsuzsa, FOGARASI András, BARSI Péter, HALÁSZ Péter

Objectives – A retrospective study has been done at the Bethesda Children’s Hospital Epilepsy Center with those patients whose EEG records fulfilled in one or more records the criteria of electrical status epilepticus in slow wave sleep (ESES) pattern, occupying at least 75% of NREM sleep with bilateral discharges, and had detailed disease history and long term follow-up data, between 2000 and 2012. Patients and methods – Thirty-three patients (mean 11.1±4.2 years of age) were studied by 171 sleep EEG records. Sleep was recorded after sleep deprivation or during spontaneous sleep at least for one hour length of NREM. From the 492 EEGs, 171 sleep records were performed (average five/patient). Average follow-up time was 7.5 years. Eighty-two ESES records have been analyzed in 15 non-lesional and 18 lesional (11 with dysgenetic and seven with perinatal - asphyxic or vascular origin) patients. Variability of seizure types, seizure frequency and frequency of status epilepticus was higher in the lesional group. Impairment of the cognitive functions was moderate and partial in the non-lesional, while severely damaged in the lesional group. Results – EEG records of 29 patients showed unihemispherial spike fields with a perpendicular axis (in anterior, medial and posterior variants) to the Sylvian fissure, regardless their lesional or non-lesional origin. Only three (1one non-lesional and two lesional) patients had bilateral synchronous spike-wave discharges with bilateral symmetric frontocentral spike fields. The individual discharges of the sleep EEG pattern were very similar to the awake interictal records except their extension in time and field, their increased number, amplitude, and continuity of them and furthermore in the increased trans-hemispheral propagation and their synchronity. Conclusions – Assumed circuits involved in the pathomechanism of discharges during NREM sleep in ESES are discussed based on our findings.

Clinical Neuroscience

MARCH 30, 2016

The electrophysiological changes after 1 hz RTMS in ALS patients. A pilot study

MAJOR Zsigmond Zoltán, VACARAS Vitalie, MARIS Emilia, CRISAN Ioana, FLOREA Bogdan, MAJOR Andrea Kinga, MURESANU Fior Dafin

Motor neuron diseases are disabling poor prognostic conditions, with no successful treatment. Repetitive transcranial magnetic stimulation might offer a temporary functional improvement. Objective - We intended to evaluate the extent of the functional improvement using electrophysiological and clinical tests. Methods - Patients with motor neuron disease (amyotrophic lateral sclerosis) were included. Muscle strength and respiratory function assessment represents the clinical approach, and central motor conduction time, motor unit number estimation, blink reflex and H-reflex stands for electrophysiology. Two tests were performed using the whole battery prior and after low frequency repetitive transcranial magnetic stimulation, using 1 Hz stimulation frequency for five consecutive days, 20 minutes daily, at 80% of the individual resting motor threshold. Results - Central motor conduction time, muscle strength and pulmonary function showed no statistically significant differences, but a tendency towards improvement. Motor unit number estimation, blink reflex and H-reflex showed a significantly better outcome after the five day repetitive transcranial magnetic stimulation treatment. Conclusion - Low frequency repetitive transcranial magnetic stimulation influences beneficially electrophysiological parameters in amyotrophic lateral sclerosis, but with little clinical impact; further studies are needed to validate the extent of the effect.

Clinical Neuroscience

JANUARY 30, 2016

[The propagation and semiology of focal epileptic seizures. case presentations to the insula. theoretical considerations]

BALOGH Attila, BALOGH Attila ifj.

[Study objectives – The developing of diagnostical examinations in epileptology provides new challenges in seizure semiology. On the analysis of seizures it is important to examine the mechanisms of their propagation. The brain connectivity (based on the neuroimaging), the shadowing of the movement of excessive neuronal activity (based on computerized EEG and MEG methods), the cognition of the physiological and pathological brain networks are the foot- stone of the epileptic seizure propagation. The investigators prove, by means of case demonstrations of the role of the network nodes and the role of the epileptic hubs in the seizure symptomatology. Methods – The preoperative, intra and postoperative data are analised of three insular and one parietal epileptic patients in point of view of their seizure symptomes. Complex neuroimaging, noninvasive and invasive electrophysiology, intensive long-term video-EEG monitoring, computerized EEG analysis, fuctional mapping, intraoperative corticography were used. The etiology were confirmed with hystology. Results – It is observed that on seizure semiology our patients plays the insula a double role. In some cases, it is the focus of insular seizures with their symptoms difficult to identify. However, in the majority of cases and as a consequence of its rich neural connections, the insula has a peculiar property in the evolution of the symptomatogenic features of seizures. This observations are developing new relationships between the mechanism of seizure propagation and its semiological consequences. Conclusions – On epileptological point of view there are brain structures which has peculiar role in the „designe” of propagation of the epileptic excitement. The numerous new methods in neuroimaging and neurophysiology allowed the connectomical examination of the epileptic networks. The role of the epileptic diathesis is approachable with the metholdology of the brain connectivity. Theoretically the node of the epileptic network consist of the potential pathes where the localised excessive excitement can propagete. The route where the actual seizure can go adhead is determined by the actual edpileptic propensity of the above mentioned potential pathes.]

Clinical Neuroscience

JANUARY 30, 2016

Facial virus inoculations infect vestibular and auditory neurons in rats

HELFFERICH Frigyes, LOURMET Guillaume, SZABÓ Rebeka Éva, BOLDOGKŐI Zsolt, PALKOVITS Miklós

Background and purpose – There is growing evidence for the viral origin of the Bell’s facial palsy, vestibular neuritis and sudden sensorineural hearing loss, however their exact pathophysiology is still unknown. We investigated the possibility of brainstem infections following peripheral viral inoculations in rats. Methods – Pseudorabies virus, a commonly used neurotropic viral retrograde tracer was injected into the nasolabial region of rats. Five and 6 days after injections, infected brainstem nuclei were demonstrated by immunohistochemical techniques. Results – Infected neurons were found in the motor facial, the medial vestibular, and the sensory trigeminal nuclei, as well as in the medial nucleus of the trapezoid body. Conclusion – Pseudorabies virus infects auditory and vestibular sensory neurons in the brainstem through facial inoculation. The possible routes of infections: 1. trans-synaptic spread constituted by facio-vestibular anastomoses: primarily infected motor facial neuron infects neurons in the medial vestibular nucleus, 2. via trigeminal sensory nerves: the sensory trigeminal complex innervated by GABAergic medial vestibular neurons, and 3. one bisynaptical route: infected facial motoneurons may receive indirect input from the medial vestibular nucleus and the trapezoid body via connecting neurons in the sensory trigeminal complex. We may assume that latent infections of these areas may precede the infections of the peripheral organs and the reactivation of the virus exerts the symptoms.

Clinical Neuroscience

NOVEMBER 20, 2015

[Earlier and more efficiently: the role of deep brain stimulation for parkinson’s disease preserving the working capabilities]

DELI Gabriella, BALÁS István, KOMOLY Sámuel, DÓCZI Tamás, JANSZKY József, ASCHERMANN Zsuzsanna, NAGY Ferenc, BOSNYÁK Edit, KOVÁCS Norbert

[Background – The recently published “EarlyStim” study demonstrated that deep brain stimulation (DBS) for the treatment of Parkinson’s disease (PD) with early fluctuations is superior to the optimal pharmacological treatment in improving the quality of life and motor symptoms, and preserving sociocultural position. Our retrospective investigation aimed to evaluate if DBS therapy was able to preserve the working capabilities of our patients. Methods – We reviewed the data of 39 young (<60 years-old) PD patients who underwent subthalamic DBS implantation at University of Pécs and had at least two years follow-up. Patients were categorized into two groups based on their working capabilities: Patients with active job (“Job+” group, n=15) and retired patients (without active job, “Job-” group, n=24). Severity of motor symptoms (UPDRS part 3), quality of life (EQ-5D) and presence of active job were evaluated one and two years after the operation. Results – As far as the severity of motor symptoms were concerned, similar (approximately 50%) improvement was achieved in both groups. However, the postoperative quality of life was significantly better in the Job+ group. Majority (12/15, 80%) of Job+ group members were able to preserve their job two years after the operation. However, only a minimal portion (1/24, 4.2%) of the Job- group members was able to return to the world of active employees (p<0.01, McNemar test). Conclusion – Although our retrospective study has several limitations, our results fit well with the conclusions of “EarlyStim” study. Both of them suggest that with optimal timing of DBS implantation we may preserve the working capabilities of our patients.]

Clinical Neuroscience

JANUARY 30, 2016

[A visual based proto-consciousness model of human thinking]

SZŐKE Henrik, HEGYI Gabriella, CSÁSZÁR Noémi, VAS József Pál, KAPÓCS Gábor, BÓKKON István

[Background and objectives – Here we present our results of many years of research on the visual (pictorial) representation model expanded with some new ideas in a simplified form. Our goal is to make available our new pictorial model for a broader scientific community and to point to its possible importance in the future. Method – Own scientific publications, selective literature analysis and preliminary experiments. Results – Our several scientific publications and preliminary experiments were presented outlining our new molecular visual representation model as brain might be able to generate internal images by regulated biophotons in early V1 retinotopic visual regions. We also proposed that some of symptoms and characteristics of autism and savantism may suggest that visual (pictorial) thinking might be a possible cognitive model in the case of healthy people as well. Our model can present a uniform molecular basis for many visual related phenomena. Conclusions – It is possible that a so-called visual proto-consciousness might be developed in evolution, which is directly related to the retinotopic visual areas, and which has a different cognitive ability from verbal abilities. If our model can be exactly proved it presents a common molecular basis for various visual phenomena such as visual perception and imagination, phosphenes ect. and might open new ways in several fields of science such as visual prosthesis for the blind, artificial intelligence, visual neuroscience, cognitive and autism research.]

Clinical Neuroscience

NOVEMBER 20, 2015

[Novel strategy in the radiotherapy of metastatic brain tumors: simultaneous whole brain radiotherapy and integrated stereotactic radiosurgery]

KALINCSÁK Judit, LÁSZLÓ Zoltán, SEBESTYÉN Zsolt, KOVÁCS Péter, HORVÁTH Zsolt, DÓCZI Tamás, MAGNEL László

[Background and purpose – Treatment of central nervous system (CNS) tumors has always played an important role in development of radiotherapy techniques. Precise patient immobilisation, non-coplanar field arrangement, conformal treatment, arc therapy, radiosurgery, application of image fusion to radiation planning or re-irradiation were first introduced into clinical routine in the treatment of brain tumors. Methods – A modern multifunctional radiation instrument, Novalis TX has been installed at the University of Pécs two years ago. New methods, such as real time 3D image guided therapy, dynamic arc therapy and ultra-conformity offer further progress in treatment of CNS tumors. Whole brain irradiation and simultaneous fractionated stereotactic radiosurgery or integrated boost seem to be an optimal method in the treatment of not only soliter or oligo, but even a higher number (4-9) and not typically radiosensitive brain metastases. The new treatment strategy is illustrated by presentation of four case histories. Results – Treatment protocol was completed in all cases. Treatment period of 1.5 to 3 weeks, and treatment time of only a few minutes were not stressful for the patients. A quite remarkable clinical improvement as to general condition of the patients was experienced in three cases. Follow-up images confirmed either remission or a stable disease. Conclusions – Simultaneous whole brain radiotherapy and integrated stereotactic radiosurgery is a reproducible, safe method that offers an effective irradiation with delivery of definitive dosage even in cases with radio-insensitive brain metastasis.]

Clinical Neuroscience

MARCH 30, 2016

[Computed tomographic examination of cranial lesions, a paleoradiological approach]

ZÁDORI Péter, BAJZIK Gábor, BÍRÓ Gergely, LELOVICS Zsuzsanna, BALASSA Tímea, BERNERT Zsolt, ÉVINGER Sándor, HAJDU Tamás, MARCSIK Antónia, MOLNÁR Erika, ŐSZ Brigitta, PÁLFI György, WOLFF Katalin, REPA Imre

[Background and purpose - Introducing the multidisciplinary paleoradiology research at the Institute of Diagnostic Imaging and Radiation Oncology of the Kaposvár University, highlighting the cases with potential central nervous system involvement - from the scanning methods to the 3D printing - in order to draw attention to the historical background and clinical aspects of certain pathological conditions. Methods - The authors developed the examination protocols for three different CT scanners. Among the examined archaeological remains cranial lesions were identified in 26 cases, from which 4 cases with potential central nervous system involvement are demonstrated. The scanning parameters and the advantages of secondary image reconstructions (multiplanar reconstruction, maximum intensity projection, three-dimensional volume rendering technique) are presented with the cases. Results - The authors demonstrate a case with destructive skull lesions due to syphilis from the 15th century AD, a condition rarely seen or even unknown nowadays in the modern world. With the CT images of the skull base fracture from the Iron Age, signs of healing could be verified. Using the CT images a non-invasive approach is presented in the case of the craniofacial osteosarcoma in order to visualize the local status and the direct intracranial propagation. Advantages of the 3D VRT reconstructions are shown in the case of unilateral coronal suture synostosis. Conclusion - Paleoradiological CT examinations serve as a non-invasive, non-destructive tool for studying archaeological remains and artifacts. The special applications provided by the imaging modality contribute to the conventional paleopathological investigations. Keywords: paleoradiology, computed tomography, 3D reconstruction, skull lesions, paleopathology]