Clinical Neuroscience

[Reflections on the meditations about the future of neurology]

KOPA János

MARCH 20, 2011

Clinical Neuroscience - 2011;64(03-04)

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Clinical Neuroscience

[Interdisciplinary approach of vestibular system impairment]

PONGRÁCZ Endre

[In the first part of this review the definition of vertigo/dizziness was discussed. The major difference between the two signs is the exsistence of the direction, which is specific for vertigo. Dizziness is a frequent complaint in the clinical practice. Its frequency is increasing with advance of age, to intimate the play of declining cognitive process in the pathogenesis of its. The popular health significance of vertigo is in the rowing number of the patients. The onset of the most cases with acute vertigo appears between secundums and minutes so the patients will be provided in circumstances of emergency department. First of all three form schould be take into account: neuronitis vestibularis, benign paroxysmal positional vertigo and Meniere syndrome. Without tipical periferal signs of vertigo, central cause should be searched, principally stroke (lysis possibility). The differential diagnose of the different dizzeness/vertigo forms according to the elapsed time of the onset or congenital and acquired nystagmus was created in tables. The recommendations of the therapy of acute and chronic dizziness/ vertigo syndroms are, lack of results of evidence based trials doubtful. The more often used drugs based on clinical trials are discussed as vinpocetine, betahistine and piracetam. The in vitro and in vivo data suggest that the last molecule is eligible to use both in periferal and central type of vertigo syndroms.]

Clinical Neuroscience

[Epilepsy in the elderly]

NIKL János

[The incidence of epilepsy is higher among the elderly, the most rapidly growing segment of the population, than in any other age group. New-onset seizures in elderly patients are typically symptomatic or cryptogenic partial seizures that require long-term treatment. Epilepsy in the elderly is a frequently occuring pathology, differing in etiology, clinical presentation and prognosis from those of young people. Establishing the diagnosis of epilepsy in old age can be more difficult than in younger patients due to the extensive range of differential diagnoses and a higher prevalence of concomitant disease. Beyond a certain age physiological and pathophysiological changes can affect the pharmacokinetics and pharmacodynamics of antiepileptic drugs(AEDs), increasing the risk of pharmacological interactions due to polypharmacy. Furthermore, the elderly people are sensitive to advers events of AEDs, as for example, to cognitive disturbances, osteoporosis. Several of newer AEDs have good safety and cognitive effect profiles and have no interactions with other drugs. The treatment strategies are demanding: they must take into consideration the co-morbidity, co-medication, alterations in drug metabolism, and the effects on aging body. These factors make the management of epilepsy in the elderly particulary challenging, but with appropiate pharmacological treatment most elderly people with epilepsy will remain seizurefree.]

Clinical Neuroscience

[CADASIL and other hereditary small vessel diseases of the brain - Increasingly diagnosed conditions underlying familial ischaemic stroke and dementia]

GUNDA Bence, HUGUES Chabriat, BERECZKI Dániel

[CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy) has recently gained great interest in vascular neurology as the most common heritable cause of stroke and vascular dementia in adults. This autosomal dominant small vessel disease of the brain - unlike the sporadic, hypertensive form - appears already in adult midlife in the absence of vascular risk factors with ischemic episodes and progressive dementia, its first manifestation can be migraine with aura, and is often associated with psychiatric disturbances. The magnetic resonance imaging (MRI) changes showing a characteristic pattern may precede symptoms by more than a decade. The identification of the mautation of the NOTCH 3 gene responsible for the disorder encoding a transmembrane receptor of vascular smooth muscle cells - has given great impetus on research to understand the molecular and vascular pathogenesis of the disease. The special importance of this latter lies in the fact that CADASIL provides a pure genetic model for subcortical cerebral ischemia and vascular dementia without the confounding factors of comorbidities and advanced age. Thus insights into CADASIL may help us better understand the more common sporadic forms as well. Moreover CADASIL is one of the best studied examples of secondary migraine. Currently we have far less knowledge on other forms of hereditary small vessel disease of the brain such as CARASIL, HERNS, CRV, HVR, PXE etc. Neurologists are becoming more and more familiar with CADASIL, and with the wider availability of MRI it is increasingly diagnosed. However the disorder is still probably underrecognised. This review aims to summarize our current knowledge on CADASIL with special emphasis on diagnostic and diffrential diagnostic points for the practising neurologist.]

Clinical Neuroscience

[EEG source localization using LORETA (Low Resolution Electromagnetic Tomography)]

PUSKÁS Szilvia

[Electroencephalography (EEG) has excellent temporal resolution, but the spatial resolution is poor. Different source localization methods exist to solve the so-called inverse problem, thus increasing the accuracy of spatial localization. This paper provides an overview of the history of source localization and the main categories of techniques are discussed. LORETA (Low Resolution Electromagnetic Tomography) is introduced in details: technical informations are discussed and localization properties of LORETA method are compared to other inverse solutions. Validation of the method with different imaging techniques is also discussed. This paper reviews several publications using LORETA both in healthy persons and persons with different neurological and psychiatric diseases. Finally future possible applications are discussed.]

Clinical Neuroscience

[Anti-NMDA-receptor encephalitis: description of the syndrome in line with presentation of the first Hungarian patient]

HOLLÓDY Katalin, CSÁBI Györgyi, LÁNG Anikó, RÓZSAI Barnabás, KOMÁROMY Hedvig, BORS László, ILLÉS Zsolt

[In the majority of cases, anti-NMDA (N-methyl-D-aspartate) receptor encephalitis is a severe, but treatable disorder, therefore early diagnosis and adequate therapy are very important. It should be suspected in children and young women, who develop acute psychiatric symptoms and seizures. During the course of the disease severe encephalopathy, agitation, hallucinations, orofacial dyskinesias, prolonged cognitive disturbance, autonomic symptoms can be observed and akinetic mutism develops. EEG shows diffuse slowing. Brain MRI is normal or unspecific. Elevated protein, pleiocytosis and oligoclonal bands can be present in the CSF. Detection of NMDA-receptor antibodies in sera or CSF confirms diagnosis. We present the case of a 15-year old girl, who fully recovered within two months after steroid treatment and repeated plasma exchange. Ovarian teratoma has not been detected.]

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[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

BOCZÁN Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette, SZÉLL Márta, KARCAGI Veronika, ZÁDORI Dénes, MOLNÁR Mária Judit

[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]

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[Benefits of SGLT-2 inhibitors: beyond glycemic control]

BALOGH Zoltán, SIRA Lívia

[In the recent years, according to international and Hungarian guidelines, in addition to lifestyle modification, metformin is the preferred initial glucose-lowering drug for most people with type 2 diabetes, if not contraindicated. Sodium glucose co­transporter-2 inhibitors have been shown to reduce progression of chronic kidney disease, or kidney failure, as well as the risk of hospitalizations for congestive heart failure and (mainly in secondary prevention) cardiovascular death in patients with type 2 diabetes. For major adverse cardiovascular events and for the renoprotection, there seems to be no class effect. On the other hand, a class effect of sodium glucose co­transporter-2 inhibitors is evident for hospitalization for heart failure. In this review the authors summarize novel data about sodium glucose cotransporter-2 inhibitors, and about their new perspectives in the near future.]

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Capability of stroke scales to detect large vessel occlusion in acute ischemic stroke – a pilot study

TÁRKÁNYI Gábor, KARÁDI Nozomi Zsófia, CSÉCSEI Péter, BOSNYÁK Edit, FEHÉR Gergely, MOLNÁR Tihamér, SZAPÁRY László

Rapid changes of stroke management in recent years facilitate the need for accurate and easy-to-use screening methods for early detection of large vessel occlusion (LVO) in acute ischemic stroke (AIS). Our aim was to evaluate the ability of various stroke scales to discriminate an LVO in AIS. We have performed a cross-sectional, observational study based on a registry of consecutive patients with first ever AIS admitted up to 4.5 hours after symptom onset to a comprehensive stroke centre. The diagnostic capability of 14 stroke scales were investigated using receiver operating characteristic (ROC) analysis. Area under the curve (AUC) values of NIHSS, modified NIHSS, shortened NIHSS-EMS, sNIHSS-8, sNIHSS-5 and Rapid Arterial Occlusion Evaluation (RACE) scales were among the highest (>0.800 respectively). A total of 6 scales had cut-off values providing at least 80% specificity and 50% sensitivity, and 5 scales had cut-off values with at least 70% specificity and 75% sensitivity. Certain stroke scales may be suitable for discriminating an LVO in AIS. The NIHSS and modified NIHSS are primarily suitable for use in hospital settings. However, sNIHSS-EMS, sNIHSS-8, sNIHSS-5, RACE and 3-Item Stroke Scale (3I-SS) are easier to perform and interpret, hence their use may be more advantageous in the prehospital setting. Prospective (prehospital) validation of these scales could be the scope of future studies.

Clinical Neuroscience

[Transcranial direct current stimulation in neuroscientific researches – pitfalls and solutions]

PESTHY Orsolya, JANACSEK Karolina, NÉMETH Dezső

[Transcranial direct current stimulation (tDCS) is a promising brain stimulation tool which is non-invasive, easy to use and relatively cheap. Since it can change brain activity in a temporal manner, it can contribute to both clinical practice and neuroscientific research. However, the effectiveness of tDCS has been questioned considering the lack of full understanding of its mechanism of action and the seemingly contradictory results. In this review, we aim to provide a summary of potential problems and possible solutions. Our main focus is on the inter-individual differences in the effect of tDCS which can explain the noisy data, thus, controlling for them is important in order to show reliable results. This review is hoped to contribute to maximizing the potential of tDCS by helping future researchers to design replicable studies.]

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[Neurological aspects of the COVID-19 pandemic caused by the SARS-CoV-2 coronavirus]

BERECZKI Dániel, STANG Rita, BÖJTI Péter, KOVÁCS Tibor

[By the spring of 2020 the COVID-19 outbreak caused by the new SARS-CoV-2 coronavirus has become a pandemic, requiring fast and efficient reaction from societies and health care systems all over the world. Fever, coughing and dyspnea are considered the major signs of COVID-19. In addition to the involvement of the respiratory system, the infection may result in other symptoms and signs as well. Based on reports to date, neurological signs or symptoms appear in 30-50% of hospitalized COVID-19 patients, with higher incidence in those with more severe disease. Classical acute neurological syndromes have also been reported to associate with COVID-19. A drop in the volume of services for other acute diseases has been described in countries with healthcare systems focusing on COVID-19. During the COVID-19 epidemic it is also important to provide appropriate continuous care for those with chronic neurological disorders. It will be the task of the future to estimate the collateral damage caused by the COVID-19 epidemic on the outcome of other neurological disorders, and to screen for the possible late neurological complications of the SARS-CoV-2 coronavirus infection.]