Clinical Neuroscience


FARSANG Marianna, TAKÁTS Annamária, SZIRMAI Imre, KOVÁCS Tibor

JANUARY 20, 2005

Clinical Neuroscience - 2005;58(01-02)

[Corticobasal degeneration was described in 1968 by Rebeiz, Kolodny and Richardson, who characterized the disease as a syndrome of asymmetric akinesis and rigidity, dystonia of the upper limb, apraxia, myoclonus and dementia. Atrophy of the frontal and parietal lobe, neuronal loss, gliosis and achromatic neurones (and nowadays astrocytic plaques) are the characteristic pathological features of the disease. Corticobasal degeneration is a rare or a rarely recognized disease and it is frequently misdiagnosed as Parkinson’s disease. According to the Lang’s criteria, corticobasal degeneration can be diagnosed in the presence of rigidity and one cortical symptom (apraxia, cortical sensory loss, alien hand) or in a patient with rigidity, dystonia and focal reflex myoclonus. Exclusion criteria are early dementia (as in primary degenerative dementias), early vertical gaze problems (as in progressive supranuclear palsy), resting tremor and good, sustained therapeutic response to levodopa (as in Parkinson’s disease), severe autonomic problems (as in multiple system atrophy) and any pathology on imaging studies which might explain the clinical symptoms. It should be mentioned, that recently early dementia is recognized as an initial symptom of corticobasal degeneration. The authors present a case and review the literature to call attention to this disorder.]



Further articles in this publication

Clinical Neuroscience



[In this work the authors provide evidences for a unifying concept of the syndromes of benign focal childhood epilepsies, Landau-Kleffner syndrome, and electrical status epilepticus in sleep treating them as a spectrum of disorders with a common transient, age dependent, non lesional, genetically based epileptogenic abnormality, the nature of which is still not known. The electro-clinical features of these syndromes are congruent with the different degree involvement of the perisylvian cognitive network and with the involvement of the thalamo-cortical associative system of variable degree. These epilepsies are characterized by the abundance of regional epileptiform discharges in sharp contrast with the rare and in several cases lacking seizures. The nature and severity of interictal cognitive symptoms are closely related to localization within the network and amount of epileptic interictal discharges. Spike-wave discharges are attributed to an alternation of overexcitation (spikes) and overinhibition (waves). The recurrent overinhibition represented by the wave of the discharges may interfere with the continuous depolarization of the cells of a large population of neurons, which is a requirement of the overt seizures. The overinhibition also interfere with cognitive processes which are correlated with the continuous presence of the fast (gamma) activity, binding the required cortical areas. Hence the recurrent inhibition works against the existence of the binding fast frequency activity. This is the assumed reason for the co-existence of the relative lack of overt seizures and in the same time for the frequently observed epileptogenic cognitive deficit symptoms ("cognitive epilepsies"). The time course of these syndromes overlaps with important developmental milestones. The frequent epileptic discharges alters the evolution of the perisylvian network developing late after early childhood and is very vulnerable for any interference in this imprinting time for speech and other cognitive functions. This spectrum of disorders represents a type of age linked, mild to severe ‘epileptic encephalopathy’ limited to the perisylvian network, where the cognitive impairment is underlied by epileptic discharges interfering with cognitive development.]

Clinical Neuroscience

[EXPERIMENTAL DEMYELINATION CAUSED BY PRIMARY OLIGODENDROCYTE DYSTROPHY Regional distribution of the lesions in the nervous system of mice brain]


[Background and purpose - Heterogeneity of multiple sclerosis lesions has been recently indicated: In addition to T-cell-mediated or T-cell plus antibody-mediated autoimmune mechanisms (patterns I-II) two other patterns (III-IV) were described. Patterns III-IV are characterized by primary oligodendrocyte dystrophy, reminiscent of virus- or toxin-induced demyelination rather than autoimmunity. It was described more than 30 years ago that dietary application of a copper-chelating agent called cuprizone results in primary oligodendrocyte degeneration which is followed by demyelination. The aim of the present study was to examine the regional distribution of cuprizone induced oligodendrocyte dystrophy and demyelination in the nervous system of mice. Material a methods - Demyelination was induced in male weanling Swis-Webster mice by feeding them on a diet containing 0.6% (W/W) cuprizone bis(cyclohexanone)-oxalyldihydrazone (G. F. Smith Chemical, Columbus OH) for 8 weeks. Animals were sacrificed after 3, 7, 14, 27, 35, 56 days of cuprizone administration. Samples were taken from corpus callosum, anterior commissure, optic nerve, cervical spinal cord and sciatic nerve. Samples were examined by immunohistochemistry, in situ hybridization for myelin proteins and myelin protein mRNA-s, respectively. Conventional neuropathological stainings and electron microscopy was also performed. Results - Oligodendrocyte degeneration and demyelination followed a particular standard pattern in the central nervous system. Profound myelin loss developed in the superior cerebellar peduncle, anterior comissure and corpus callosum, whereas the optic nerves, velum medullare anterior and spinal cord showed little or no demyelination. Sciatic nerves were unaffected. No infiltration by lymphocytes or blood-brain barrier damage was observed during cuprizone treatment. Conclusion - Cuprizone induced oligodendrocyte damage and demyelination follows a particular standard pattern in the central nervous system of mice. Cuprizone induced demyelination might be considered as a model for human demyelinating disorders with primary oligodendrocyte dystrophy and apoptosis.]

Clinical Neuroscience

[The representation of headache in the Hungarian medical literature]


[Background - Less than 1.2% of papers published in the first 50 volumes of the journal Clinical Neuroscience/Ideggyógyászati Szemle - the major official journal of Hungarian neurologists - focused on headache despite the fact that headache is among the most frequent complaints in neurological consultations. In the current study the authors evaluated the presence of headache as the main topic in articles of the Hungarian medical literature. Methods - They identified full publications on headache by handsearching all volumes of the journal Clinical Neuroscience/ Ideggyógyászati Szemle from 1950 till the end of 2003. Electronic searches were performed to find Hungarian papers focusing on headache using the bibliographic databases of the Hungarian National Library of Healthcare (Hungarian Medical Bibliography, HMB) and the American National Library of Medicine of the National Institutes of Health (Medline and Oldmedline). Results of handsearch and electronic searches were cross checked for the journal Clinical Neuroscience/Ideggyógyászati Szemle. Results - Of the 2618 full papers published in Clinical Neuroscience/ Ideggyógyászati Szemle headache was the main topic in 32 articles (1.2%), most of them published after 1985. The electronic search of the HMB resulted in 132 documents in 41 journals, whereas using the PubMed search engine, they identified 66 Hungarian papers on headache. Migraine was the most frequent topic of interest in papers found by all three searches. The HMB search identified all headache articles published after 1990 in Clinical Neuroscience/Ideggyógyászati Szemle which were identified by handsearch. The Oldmedline, database contained four of the seven papers identified by handsearch from 1954-1964. After 2002, the start of indexing Clinical Neuroscience/Ideggyógyászati Szemle in the Medline, the only article identified by handsearch was also found by the electronic search. Conclusion - After the lack of interest until the mid-1980-ies, headache became a frequent topic in the Hungarian medical literature. Bibliographical data of articles on headache published after 1990 can be identified by electronic searches of the Hungarian and international bibliographic databases using carefully constructed but simple search strategies. An increasing presence of Hungarian headache research was found in international journals in the last two decades.]

Clinical Neuroscience

[Moussong-Kovács Erzsébet]


Clinical Neuroscience



[There is an increasing number of peripheral nerve disorders with inflammatory and immune mechanisms involved. The precise diagnosis is of utmost importance, since these patients can be successfully treated. Unfortunately, there is no specific marker for any disease of this group. The diagnosis therefore relies on the appropriate consideration of the clinical, neurophysiological and laboratory data, which requires in-depth knowledge of these diseases. In this paper we review the diagnostic criteria and treatment strategies for the major types of chronic inflammatory polyneuropathies.]

All articles in the issue

Related contents

Lege Artis Medicinae

[The effects of angiotensin receptor blockers on the nervous system in hypertension and dementia]


[The renin-angiotensin system (RAS) is one of the most important mechanisms regarding the pathomechanism and treatment of hyprtension. The most of the elements of the RAS are found in the nervous system too. The effect of angiotensin converting enzyme inhibitors and angiotensin receptor blockers (ARBs) is based on the inhibition of the RAS. ARBs might have a special role in the central nervous system because they do not decrease the production of angiotensin but inhibit its harmful effects mediated through the AT1 receptor while allowing the stimulation of AT2 receptors with resulting pleiotrophic actions. Hypertension is the most important risk factor for stroke and has a negative effect on cognitive functions. Antihypertensive treatment has an effect on the nervous system; in addition to the consequences of the reduced blood pressure, ARBs might provide additional advantages in stroke and dementia prevention.]

Clinical Neuroscience

[The first identified Central-Eastern European patient with genetically confirmed dentatorubral-pallidoluysian atrophy]


[Aims - Dentatorubral-pallidoluysian atrophy (DRPLA) is an inherited neurodegenerative disorder characterized by a trinucleotide repeat expansion. The disease mainly occurs amongst the Japanese and is extremely rare in the European population. The characteristic clinical symptoms are cerebellar ataxia, dementia, choreoathetoid movements, epileptic seizures and myoclonus. The aim of this study is to present the first genetically confirmed Hungarian case of DRPLA. Case report - The middle-aged female patient developed the characteristic clinical symptoms except myoclonus over her late thirties with positive family history. The major finding in the skull magnetic resonance imaging was the atrophy of infratentorial brain structures with the consequential dilation of related cerebrospinal fluid spaces. A detailed neuropsychological examination was also performed and it revealed moderate cognitive dysfunctions, mild depression and anxiety. As underlying conditions, Huntington’s disease and common spinocerebellar ataxia forms all came into consideration, but all the result of the respective genetic tests were negative. However, the test for mutation in the ATN1 gene revealed pathological heterozygous CAG repeat expansion. Conclusion - This case study serves as the first description of genetically confirmed DRPLA in the Central-Eastern region of Europe, the clinical features of which seems to be very similar to the previously reported cases.]

Lege Artis Medicinae

[Development of the diagnostic criteria of Alzheimer’s disease]


[Alzheimer’s disease is a new endemic of the 21st century, which is going to become the biggest health and social problem of the ageing societies in the next few decades. Significant discoveries have been made by structural and functional imaging and biochemical (especially CSF) and genetic tests regarding the diagnosis of Alzheimer’s disease. The modern diagnostic results were recently incorporated into the diagnostic criteria. This review summarises these diagnostic results.]

Lege Artis Medicinae

[Deep brain stimulation: a breakthrought in the treatment of movement disorders]

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

[Over the last 20 years, it became clear that deep brain stimulation is a breakthrough in the treatment of drug-resistant movement disorders. Stimulation acts by functional inhibition of certain pathologically hyperactive nuclei. Of advantage is that the patient himself can change stimulation parameters depending on his actual status, thus reaching optimal quality of life. This option has been available for years in Hungary, as well. It is a safe, effective and cost-effective alternative in the symptomatic management of drug-refractory Parkinson’s disease, essential tremor, and primary dystonia. Before surgery, a comprehensive investigation including clinical diagnosis, severity, surgery contraindications, and expected benefit has to be performed. Based on the results of international multicenter studies, bilateral subthalamic nucleus stimulation may improve - besides symptoms, such as tremor, rigidity, bradykinesia and levodopa-induced side effects - also quality of life in Parkinson’s disease. In essential tremor, stimulation of the ventral intermediate nucleus of the thalamus is capable of spectacularly decreasing tremor. For primary dystonias, stimulation of the pallidum can improve drug-resistant symptoms and quality of life, and it substantially reduces social dependency and the amount of nursing required. In childhood dystonias, surgery may be performed at an age of as low as seven years.]

Clinical Neuroscience

[The application of RBANS (Repeatable Battery for the Assessment of Neuropsychological Status) in neurocognitive testing of patients suffering from schizophrenia and dementia]

JUHÁSZ Levente Zsolt, KEMÉNY Katalin, LINKA Emese, SÁNTHA Judit, BARTKÓ György

[Introduction - The purpose of our study was to find out whether the Hungarian adaptation of the RBANS (Repeatable Battery for the Assessment of Neuropsychological Status), a brief neurocognitive screening test, is appropriate for the differentation of healthy and non-healthy subject groups, or for the detection of differences between the cognitive performance of patient groups. Patients and method - The test battery was administrated to 38 healthy subjects, 69 schizophrenic patients, and 18 patients suffering from dementia (10 probable Alzheimer-type and eight vascular dementia). Results - There was a significant decrease of performance in all patient groups compared to the healthy group. In the schizophrenic group, the test indicated a deterioration of functioning in all cognitive areas. The patient group with Alzheimer-type dementia performed only slightly better than the schizophrenic group, because the fall of performance was not significant only one of the cognitive areas (in the visuo-spatial tasks) when compared to the healthy group. There was no difference between the performance of patients with vascular dementia and that of healthy subjects in direct memory, verbal and visuo-spatial tasks. The test results indicated an even deterioration of cognitive areas in patients with Alzheimer-type dementia. As for the vascular dementia group, the most vulnerable area proved to be that of attention, while their verbal functions were relatively spared. The deterioration in other cognitive functions shown by schizophrenic subjects was more moderate, but still significant. A comparison of the RBANS scores of the schizophrenic patients in our study and the result of an American study was also carried out. The global indeces showed no difference; only the pattern of the sub-scales was a little different. Conclusion - The Hungarian version of the RBANS seems appropriate for the differentiation of healthy and deteriorated cognitive performance in a Hungarian patient population.]