Clinical Neuroscience

[COMBINED USE OF PATTERN ELECTRORETINOGRAPHY AND PATTERN VISUAL EVOKED POTENTIALS IN THE NEUROOPHTHALMOLOGICAL DIAGNOSIS]

BENEDEK Krisztina, PÁLFFY Andrea, BENCSIK Krisztina, FEJES Imre, RAJDA Cecília, TUBOLY Gábor, LISZLI Péter

JANUARY 22, 2008

Clinical Neuroscience - 2008;61(01-02)

[Objective - To survey the role of pattern electroretinography (PERG) and pattern visual evoked potentials (VEPs) in the process of clinical evaluation of neuro-ophthalmological cases. The study is illustrated with the electrophysiological findings of 231 patients sent to our laboratory in 2005 because of the suspicion of damage in the optic pathway. Methods - The RETIport program of the ROLAND equipment (Wiesbaden - Germany) was used to record PERGs and VEPs. The recordings were done according to the recommendations of the International Society for Clinical Electrophysiology of Vision. Results - The combined application of PERG and VEP provided useful data on the retino-cortical conduction time and hence demyelinisation. The comparison of the N35/P50 and P50/N90 amplitudes of the PERG recordings greatly promoted the diagnostic procedure. Conclusions - The combined application of PERG and VEP methods is recommended in the assessment of optic nerve function of neuro-ophthalmological patients.]

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Clinical Neuroscience

[Functional magnetic resonance imaging in neurology (in English language)]

AUER Tibor, SCHWARCZ Attila, HORVÁTH Réka A., BARSI Péter, JANSZKY József

[The present contribution discusses the clinical use of functional MRI (fMRI) and its role in the most common neurological diseases. FMRI was found a reliable and reproducible examination tool resulting in a wide distribution of fMRI methods in presurgical evaluation of epilepsy in determining the relationship of eloquent areas and the epileptic focus. Preliminary data suggest that fMRI using memory paradigms can predict the postoperative memory decline in epilepsy surgery by determining whether a reorganization of memory functions took place. Speech-activated fMRI became the most used tool in determining hemispheric dominance. Visual and senso-motor cortex can also be routinely investigated by fMRI which helps in decision on epilepsy surgery. FMRI combined with EEG is a new diagnostic tool in epilepsy and sleep disorders. FMRI can identify the penumbra after stroke and can provide an additional information on metabolic state of the threatened brain tissue. FMRI has a predictive role in poststroke recovery. In relapsing-remitting MS an adaptive reorganization can be demonstrated by fMRI affecting the visual, motor, and memory systems, despite preserved functional performance. Much more extensive reorganization can be demonstrated in secondary progressive MS. These findings suggest that the different stages of MS are related to different stages of the reorganization and MS becomes progressive when there is no more reserve capacity in the brain for reorganization. FMRI offers the capability of detecting early functional hemodynamic alterations in Alzheimer’s disease before morphological changes. FMRI can be a valuable tool to test and monitor treatment efficacy in AD. FMRI can also provide information about the mechanisms of different therapeutic approaches in Parkinson disorder including drug treatment and deep brain stimulation.]

Clinical Neuroscience

[Complex regional pain syndrome]

EMBEY-ISZTIN Dezső

[Complex regional pain syndrome is one of the frequently topics to be discussed during International Congresses. Three books have been published recently by the IASP Press (1994., 2001., 2005.) The aim of my publications is to draw the attention to this syndrome frequently unrecognized, and to summarize an interesting case report.]

Clinical Neuroscience

[THE USE OF STALEVO IN HUNGARY. AN OBSERVATIONAL STUDY]

KLIVÉNYI Péter, VÉCSEI László

[The triple combination of levodopa, DDCI and entacapone (Stalevo) is used to treat motor complication in Parkinsonian patients with fluctuation. An observational investigation has been conducted in Hungary to study the effects of Stalevo on the “wearing off” phenomenon and on the quality of life in patients, who are to be treated with. The introduction of Stalevo to the treatment resulted in no changes in the number of patients taking selegiline, amantadine and dopamin agonists, while the number of patients taking anticholinergic drugs were slightly increased. This treatment significantly decreased the average Hoehn-Yahr stadium, as well as the non-motor symptoms, without any remarkable side effect. Stalevo also improved the quality of life, detected by the EQ-5D questionare and the visual analoge scale.]

Clinical Neuroscience

[FREQUENCY OF DIFFERENT FORMS OF DEMENTIA IN THE DEPARTMENT OF NEUROPATHOLOGY OF THE HUNGARIAN NATIONAL INSTITUTE OF PSYCHIATRY AND NEUROLOGY DURING A 3-YEAR PERIOD]

KOVÁCS Gábor Géza, KŐVÁRI Viktor, NAGY Zoltán

[Background - Dementia is an increasing problem of the society. The underlying cause of dementia may be difficult to diagnose during life. Only neuropathological examination gives definite diagnosis. Differences in the reported frequency may be related to factors such as the age or gender of subjects with dementia. Materials and methods - In our neuropathology-based study we examined 156 consecutive subjects clinically diagnosed with dementia during a 3-year period. Using histopathological criteria we calculated the frequencies of various disorders causing dementia. We studied the effect of age and gender on these frequencies. Results - Alzheimer’s disease was the most frequent pathologic finding (57.7%) followed by vascular dementia (43%); diffuse Lewy body disease (15.4%); argyrophilic grain dementia (12.1%), various forms of frontotemporal dementia (5.7%); and other (4.5%). The latter comprise prion disease, alcoholic encephalopathy, and hippocampal sclerosis. Mixed pathology was common: concomitant Alzheimer’s disease was present in 41.6% of diffuse Lewy body disease cases and in 49.2% of vascular dementia patients. Pure disease forms are rare: Alzheimer’s disease: 26.3%, vascular dementia: 17.3%, diffuse Lewy body disease: 5.1%, argyrophilic grain dementia: 2.5%. Females were overrepresented among those with Alzheimer’s disease with age at death above 75 years (p <0.02), while males were overrepresented in patients below 75 years with vascular dementia (p <0.05). Conclusions - Our study indicates that the frequency of neurodegenerative dementias is high in the examined patients, but vascular pathology frequently influences the clinical course.]

Clinical Neuroscience

[MANAGEMENT AFTER DBS IMPLANTATION]

KOVÁCS Norbert, BALÁS István, JANSZKY József, ASCHERMANN Zsuzsanna, NAGY Ferenc, DÓCZI Tamás, KOMOLY Sámuel

[Deep brain stimulation is a widely used technique for the treatment of movement disorders. This method is a breakthrough in treatment of drug-resistant idiopathic Parkinson’s disease, essential tremor and dystonia. The aim of the present paper is to give an inside overview of the postoperative management like fine tuning of the stimulation and balancing the antiparkinsonian medication. We also discuss the advantage of the use of the (Access Therapy) patient controller. After reviewing the stimulation-related side-effects and their management, the contraindicated medical procedures are discussed.]

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[New chapter in visual evoked potential studies - Clinical application of the multifocal vep method ]

JANÁKY Márta, PÁLFFY Andrea, BENEDEK Krisztina, BENEDEK György

[The multifocal visual evoked potentials are the evoked responses over the visual cortex in response to the stimulation of circumscribed small areas in the central 30 degree region of the retina. The recording of multifocal visual evoked potentials was made possible by the computer algorhythm elaborated by Sutter in 1991. Multifocal electroretinograpy, developed upon the same theoretical principles, is already an routine clinical examination method for the topographic analysis of functional damages in the central part of the retina and for the differential diagnostics in neuro-ophthalmology. The multifocal visual evoked potential, however, has not been introduced into the clinical practice, although it displays the function of ganglion cells in a given region of the retina in a more detailed way than the sensitivity threshold in the perimetry. This examination makes the objective verification of defects possible in the visual pathway, too. In our department the recording of multifocal visual evoked potentials was started in 2002. In this paper we present the basics of this method and also deal with the problems concerning its application and its status in the history of visual evoked potentials.]

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Cases of inborn errors of metabolism diagnosed in children with autism

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Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

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Clinical Neuroscience

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SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

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