Clinical Neuroscience

[Hungarian Epilepsy League - Account on the meeting of the Hungarian Epilepsy League]

SZUPERA Zoltán

JANUARY 30, 2010

Clinical Neuroscience - 2010;63(01-02)

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Clinical Neuroscience

[Frontotemporal dementia, Pick’s disease]

ANDREW Kertesz

[A significant expansion of knowledge in the last few years, especially in the molecular biology of frontotemporal dementia (FTD) is summarized. This condition, formerly known as Pick’s disease and considered rare, is estimated to be 12-15% of all dementias and 30-50% early onset ones. The clinical picture is protean, mainly a behavioural and language impairment, but the extrapyramidal syndromes of CBD and PSP also belong. These seemingly different presentations converge, as one or other areas in the brain are affected. Less than half of the cases are tauopathies, the majority has been discovered to have a TDP-43 and most recently a FUS proteinopathy, shared with ALS, opening potential opportunities for pharmacological approaches to treatment. Tau and progranulin mutations on Ch-17 and some others, point to molecular mechanisms. A glossary is provided to navigate the complex terminology.]

Clinical Neuroscience

[How does the brain create rhythms?]

SZIRMAI Imre

[Connection was found between rhythmic cortical activity and motor control. The 10 Hz μ-rhythm and the 20-30 Hz bursts represent two functional states of the somatomotor system. A correspondence of the central μ-rhythm of the motor cortex and the physiological hand tremor (8-12 Hz) is presumed. The precise tuning of the motor system can be estimated by the frequency of repetitive finger movements. In complex tapping exercise, the index finger is the most skillful, the 3rd, 4th and 5th fingers keep rhythm with less precision. It was found that the organization of mirror movements depends on the cortical representation of fingers. Mirror finger movements are more regular if the subject begins the motor action with the 5th (small) finger. Concerning cortical regulation of finger movements, it was suggested that there are two time-keeping systems in the brain; one with a sensitivity above and another with a sensitivity below the critical frequency of 3 Hz. The preferred meter which helps to maintain synchronous finger movements is the cadence of 4/4 and 8/8. We observed that the unlearned inward-outward sequential finger movement was equally impaired in nonmusician controls and patients with Parkinson-disease. In movement disorders, the ability of movement and the “clock-mechanism” are equally involved. The polyrhythmic finger movement is not our inborn ability, it has to be learned. The “timer” function, which regulates the rhythmic movement, is presumably localised in the basal ganglia or in the cerebellum. The meter of the music is built on the reciprocal values of 2 raised to the second to fifth power (1/12, 1/22, 1/23, 1/24, 1/25). The EEG frequencies that we consider important in the regulation of cons-cious motor actions are approximately in the same domain (4, 8, 16, 32, 64 Hz). During music performance, an important neural process is the coupling of distant brain areas. Concerning melody, the musical taste of Europeans is octavebased. Musical ornaments also follow the rule of the gothic construction, that is: pursuit of harmony towards the single one rising from the unification of 8-4-2 classes. Leibnitz concerned music as the unconscious mathematics of the soul. Movement-initiating effect of music is used in rehabilitation of patients with movement disorders. The meter and rhythm have superiority over the melody. It is possible that rhythmic movements can be generated also in the absence of sensory input and the central oscillators can produce “fictive motor patterns”.]

Clinical Neuroscience

[Anterior cervical fusion on the lower cervical spine: own clinical experiences]

PAZDERNYIK Szilárd, SÁNDOR László, ELEK Péter, BARZÓ Pál

[Both acute and chronic instability of the cervical spine can be succesfully treated by anterior crevical fusion. The main goal is to create a spondylodesis through which the instable motion segments are fixed in the position defined by the surgeon. The spondylodesis is realised by the bone healing of the intervertebral space. The consolidation itself is facilitated by the operative stabilisation of the segments involved, and also by the implantation/transplantation of the osteoproductive/ osteoinductive materials. The sooner consolidation is achieved, the more likely it is to be able to avoid the material dependent complications and/or that of dislocation. So as to support this theory a retrospective clinical/radiological study was performed. During this the length and the safety of the consolidation was measured by applying various anterior cervical plating systems. A total of 485 patients having cervical injuries or degenerative disc disease were treated by anterior cervical plating. For bone transplantation partly pure autolog spongious partly autolog cortico-spongious morsalised bone chips, furthermore autolog tricortical bone block were applied. A standard protocoll was used for data collection, evaluation and also follow-up. The patients treated with plate systems were divided into 3 groups: Group 1: Non-locked H-plate system with autogeneous cancellous bone (155 trauma patients, for a total of 210 cervical motion segments, 1.35 segments/patients). Group 2: Non-locked H-plate system with tricortical autograft (167 patients, for a total of 290 cervical motion segments, 1.73 segments/patients). Group 3: Locked cervical plate system with tricortical autograft (73 patients, for a total of 110 cervical motion segments, 1.5 segments/patients). Patients treated with standalone cage belong to group 4. These cages were filled with autogenous cortico-spongiosus bone chips (90 patients, for a total of 90 cervical motion segments, 1.0 segments/patients). Evaluations included postoperative clinical, X-ray and CT examination, and follow-ups at 6, 16, 52, and 104 weeks. We established three grades, and classified the degree of bony fusion between the graft and vertebra: not-yet-fused, fused or non-union. When evaluating the results the following statements/observations were made: a) There is a fast and safe consolidation in the case of those patients that underwent dinamic disc osteosynthesis (p=0.00001). b) Whereas performing fixation with non-locked or locked screw plate systems and strutgrafted with tricortical autograft created prolonged healing requiring months and developed non-unions more often (non-locked screw-plate system versus locked screwplate system) (p>0.05). c) Using locked screw-plate fixation systems non-union rate in our study was 21%, suggesting that this form of fixation has only a limited use. d) In our study complete consolidation without pseudoarthrosis was achieved by using standalone cages filled with autolog cortico- spongiosus bone chips, but bony healing was delayed due to cage coating and the substitution of pure autogenous spongiosa for cortico-spongiosus bone chips. It is recommended to treat acute/chronic instability of the cervical spine both by using non-fixed plate system with autolog cancellosus bone and by standalone cage filled with cortico-spongiosus bone chips as well. It is worth keeping in mind that by applying this lattest an extra surgery to harvest the graft will be avoided.]

Clinical Neuroscience

[Extracellular matrix of intracerebral tumors with different invasion activity]

KLEKNER Álmos, VARGA Imre, BOGNÁR László, HUTÓCZKI Gábor, KENYERES Annamária, TÓTH Judit, HANZÉLY Zoltán, SCHOLTZ Beáta

[Objectives - Ineffective surgical and radiotherapy of glioblastoma is mainly due to its intensive infiltrating behavior. Contrarily, brain metastases of anaplastic carcinomas are well-circumscribed intracerebral lesions that can be easily exstirpated in most cases. The molecules of the extracellular matrix (ECM) play a pivotal role in the peritumoral infiltration. In this study the mRNA expression of the ECM components was investigated in two types of intracerebral malignoma with different invasion activity. Our aim was to identify the ECM molecules that are responsible for the different intensity of peritumoral infiltration of tumors from different origin. Methods - The mRNA expression of twenty-three ECM molecules was determined by quantitative reverse transcriptase polymerase chain reaction. Four pieces of glioblastoma and four pieces of intracerebral lung adenocarcinoma metastasis from neurosurgical operation were investigated. Immunohistochemical investigations were performed in case of five molecules. Results - The mRNA expression of nine molecules (brevican, neurocan, neuroglycan-C, syndecan-1,2,4, tenascin-C, versican and matrix-metalloproteinase-[MMP]2) differed significantly by comparison of the two tumor types. By immunohistochemistry, neurocan, syndecan, versican and MMP-2 showed alteration in staining intensity according to the mRNA expression, while MMP-9 showed higher staining intensity in the metastatic tumor. Conclusions - The identified molecules can play an important role in the different infiltration activity of tumors from different origin. Thus these ECM-components could serve as targets for anti-invasion therapy in the future.]

Clinical Neuroscience

[Posterior cortical atrophy (Benson-syndrome)]

RÓZSA Anikó, SZILVÁSSY Ildikó, KOVÁCS Krisztina, BOÓR Krisztina, GÁCS Gyula

[We present the characteristics of posterior cortical atrophy - a very rare cortical dementia - in a 69 year old woman’s case. Our patient’s symptoms began with a visual problem which was initially explained by ophthalmological disorder. After neurological exam visual agnosia was diagnosed apart from other cognitive disorder (alexia without agraphia, acalculia, prosopagnosia, constructional disorder, clock-time recognition disorder, dressing apraxia, visuospatial disorientation). The brain MRI showed bilateral asymmetric parieto-occipital atrophy which is characteristic of posterior cortical atrophy.]

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Clinical Neuroscience

[The Comprehensive Aphasia Test in Hungarian]

ZAKARIÁS Lilla, RÓZSA Sándor, LUKÁCS Ágnes

[In this paper we present the Comprehensive Aphasia Test-Hungarian (CAT-H; Zakariás and Lukács, in preparation), an assessment tool newly adapted to Hungarian, currently under standardisation. The test is suitable for the assessment of an acquired language disorder, post-stroke aphasia. The aims of this paper are to present 1) the main characteristics of the test, its areas of application, and the process of the Hungarian adaptation and standardisation, 2) the first results from a sample of Hungarian people with aphasia and healthy controls. Ninety-nine people with aphasia, mostly with unilateral, left hemisphere stroke, and 19 neurologically intact control participants were administered the CAT-H. In addition, we developed a questionnaire assessing demographic and clinical information. The CAT-H consists of two parts, a Cognitive Screening Test and a Language Test. People with aphasia performed significantly worse than the control group in all language and almost all cognitive subtests of the CAT-H. Consistent with our expectations, the control group performed close to ceiling in all subtests, whereas people with aphasia exhibited great individual variability both in the language and the cognitive subtests. In addition, we found that age, time post-onset, and type of stroke were associated with cognitive and linguistic abilities measured by the CAT-H. Our results and our experiences clearly show that the CAT-H provides a comprehensive profile of a person’s impaired and intact language abilities and can be used to monitor language recovery as well as to screen for basic cognitive deficits in aphasia. We hope that the CAT-H will be a unique resource for rehabilitation professionals and aphasia researchers in aphasia assessment and diagnostics in Hungary. ]

Lege Artis Medicinae

[Thiazide- or thiazide-like diuretics should be used in the treatment of patients with hypertension? Particularities of the situation in Hungary]

VÁLYI Péter

[Diuretics have remained the cornerstone of the antihypertensive treatment since their widespreading in the 1960s. According to the 2018 ESC/ESH Guidelines for the management of arterial hypertension, in the absence of evidence from direct comparator trials and recognizing that many of the approved single-pill combinations are based on hydrochlorothiazide, this drug and thiazide-like indapamide can be considered suitable antihypertensive agents. In the 2018 Hungarian guidelines indapamide is named as the most efficacious diuretic in the treatment of patients with hypertension. The aim of the publication is redefining thiazide- and thiazide-like diuretic use in the treatment of hypertensive patients, with particular attention to presently available hydrochlorothia­zide and indapamide, and their combination drugs in Hungary.]

Lege Artis Medicinae

[Focus on Lege Artis Medicinae (LAM)]

VASAS Lívia, GEGES József

[Three decades ago, LAM was launched with the goal of providing scientific information about medicine and its frontiers. From the very beginning, LAM has also concerned a special subject area while connecting medicine with the world of art. In the palette of medical articles, it remained a special feature to this day. The analysis of the history of LAM to date was performed using internationally accepted publication guidelines and scientific databases as a pledge of objectivity. We examined the practice of LAM if it meets the main criteria, the professional expectations of our days, when publishing contents of the traditional printed edition and its electronic version. We explored the visibility of articles in the largest bibliographic and scientific metric databases, and reviewed the LAM's place among the Hun­ga­rian professional journals. Our results show that in recent years LAM has gained international reputation des­pite publishing in Hungarian spoken by a few people. This is due to articles with foreign co-authors as well as references to LAM in articles written exclusively by foreign researchers. The journal is of course full readable in the Hungarian bibliographic databases, and its popularity is among the leading ones. The great virtue of the journal is the wide spectrum of the authors' affiliation, with which they cover almost completely the Hungarian health care institutional sys­tem. The special feature of its columns is enhanced by the publication of writings on art, which may increase Hungarian and foreign interest like that of medical articles.]

Lege Artis Medicinae

[LAM 30: 1990–2020. Facing the mirror: Three decades of LAM, the Hungarian medicine and health care system]

KAPÓCS Gábor

Clinical Neuroscience

Neuroscience highlights: Main cell types underlying memory and spatial navigation

KRABOTH Zoltán, KÁLMÁN Bernadette

Interest in the hippocampal formation and its role in navigation and memory arose in the second part of the 20th century, at least in part due to the curious case of Henry G. Molaison, who underwent brain surgery for intractable epilepsy. The temporal association observed between the removal of his entorhinal cortex along with a significant part of hippocampus and the developing severe memory deficit inspired scientists to focus on these regions. The subsequent discovery of the so-called place cells in the hippocampus launched the description of many other functional cell types and neuronal networks throughout the Papez-circuit that has a key role in memory processes and spatial information coding (speed, head direction, border, grid, object-vector etc). Each of these cell types has its own unique characteristics, and together they form the so-called “Brain GPS”. The aim of this short survey is to highlight for practicing neurologists the types of cells and neuronal networks that represent the anatomical substrates and physiological correlates of pathological entities affecting the limbic system, especially in the temporal lobe. For that purpose, we survey early discoveries along with the most relevant neuroscience observations from the recent literature. By this brief survey, we highlight main cell types in the hippocampal formation, and describe their roles in spatial navigation and memory processes. In recent decades, an array of new and functionally unique neuron types has been recognized in the hippocampal formation, but likely more remain to be discovered. For a better understanding of the heterogeneous presentations of neurological disorders affecting this anatomical region, insights into the constantly evolving neuroscience behind may be helpful. The public health consequences of diseases that affect memory and spatial navigation are high, and grow as the population ages, prompting scientist to focus on further exploring this brain region.