Clinical Neuroscience

[Moyamoya disease with partial epilepsy and cerebral infarct in early childhood]

NEMES András1, LAZÁNYI Zoltán1, NAGY István1

MARCH 20, 1997

Clinical Neuroscience - 1997;50(03-04)

[An 8-month-old infant with initial partial epileptic seizure is described in whose case moyamoya disease and a form of infarct were established. In connection with the case the possible reasons for the pathological picture, clinical characteristics and diagnosis are detailed. Attention is called to the fact that stroke can be considered as a possible etiological factor while clarifying the etiology of a partial epileptic seizure in infancy.]


  1. Országos Idegsebészeti Tudományos Intézet, Neuroradiológiai Osztály, Budapest



Further articles in this publication

Clinical Neuroscience

[Reoperation of recurrent supratentorial lobar gliomas (astrocytoma, glioblastoma)]

ÁFRA Dénes, SIPOS László

[Hundred-nine patients with recurrent supratentorial gliomas were reoperated during the last 15 years at the National Institute of Neurosurgery, Budapest. The patients were grouped according to the WHO classification based on the first histology. Fifty patients with low grade (A 2), 30 with anaplastic astrocytoma (A 3) and 29 with glioblastoma multiforme (GM) were reoperated on. Nine patients with low grade astrocytoma were irradiated following the first operation. They survived 5 to 14 months (median value 7 months). Those 22 patients who were irradiated after the reoperation survived longer despite the malignant tranformation of their tumour. The median survival time was 24 months. Twenty-four patients with anaplastic astrocytoma received radiotherapy after the first operation in a total dose from 50 to 60 Gy. Six patients did not give their consent. The median survival after the second operation was 5.5 months. Four patients also received chemotherapy and 1 of them is still alive 4 years after the reoperation. Out of the 29 patients operated on with glioblastoma 20 were irradiated. The median survival after the reoperation was 3 months, and only a few patients lived longer than 6 months. Chemotherapy itself did not influence the survival time significantly in either group. The survival time was influenced significantly by the first histology. Radiotherapy after the reoperation prolonged the survival time significantly, especially in the group of patients with primary low grade astrocytoma. Reoperation of patients with malignant astrocytoma who were irradiated immediately after the first operation extended the survival time slightly.]

Clinical Neuroscience

[Comparative human immunohistochemical investigations of peptidergic innervation of embryonal and adult cerebral blood vessels]

BÜKI András, DÓCZI Tamás, HORVÁTH Zoltán, FÜRTÖS András

[Peptidergic fibres innervating human embryonic and adult cerebral blood vessels were screened for calcitonine gene-related peptide, neuropeptide-Y and substance-P immunopositive fibres, the mediaadventitia border displayed a network-like fibre arrange ment. There was no significant difference either in the distribution of the individual peptidergic fibres or in the age-dependent appearance of peptidergic networks. On the basis of these results it seems to be unlikely that the different clinical course of juvenile and adult subarachnoid haemorrhages could be the result of an age specific difference of peptidergic fibre distribution influencing the neurogenic mecha nisms of cerebral autoregulation.]

Clinical Neuroscience

[Statistical evaluation and pathological significance of the incidence of corpus amylaceum]


[Objective: Semiquantitative evaluation of the corpus amylaceum (polyglucosan body) was carried out by a method reported before on a 1407 autopsy - partly biopsy - material. Design: The present report contains the data of the occurrence of corpus amylaceum in different disease groups demonstrated in tables expressed in numbers and percentage evaluated by homogenity examination. Setting: The manifold neuropathological examination enabled the reaching of some implications of clinical significance too. Material and method: The material involved a great number of cases not reported before. The cases were classified according to their ages and different diseases as well as a new grading system of quantitative evaluation of the frequency of corpus amylaceum in various localizations. Main outcome measures: The most important aspects were the examination of the predilection sites, the elucidation of the development of corpus amylaceum and occurrence in different conditions and diseases. Results: The method and examination permitted drawing conclusions as follows: 1. The chronic, recurring vascular-barriere disturbances appeared significant in the formation of corpus amylaceum. - 2. There was not an expressive difference between the ischemic and hemorrhagic vascular diseases considering the number of corpus amylaceum nor did more corpus amylaceum occur in Alzheimer's disease than in aged. - 3. Greater amount of corpus amylaceum may be found in some spinal cord lesions. – 4. The formation of corpus amylaceum was not observed in the regions of astrocytic gliosis (e.g. postraumatic) or in astrocytomas. This finding needs further study. - 5. The linear correlation between age and the increased quantity of corpus amylaceum may also be proved statistically. Conclusions: It deserves particular attention that more mechanisms may play a role in the formation of corpus amylaceum such as the insufficiency of astrocytes in the utilization of glucose which may be associated with accumulation of some carbohydrate polymers and glycogen. The increased volume of cerebrospinal fluid in the predilection sites is also an important factor. The spinal cord proved to be a prominent predilection site, where the great amount of corpus amylaceum occurred in some diseases which may be explained by the increased quantity of stress proteins. The final conclusion is that the corpus amylaceum has a pathog nostic significance, but it has a tendency for higher occurrence in some conditions.]

Clinical Neuroscience

[Progressive myoclonus epilepsy - A review and case histories]


[Progressive myoclonus epilepsy (PME) syndrome is a heterogeneous entity underlain by rare genetic diseases. The most frequent origins of progressive myoclonus epilepsy are: Unverricht-Lundborg disease, Lafora's disease, ceroid lipofuscinosis, MERRF (myoclonus epilepsy with ragged red fibers) and sialidosis. Action myoclonic jerks, epileptic seizures, mental deterioration, neurological impairments are the four main symptoms of PME syndrome. Nowadays in more and more cases a causal diagnosis is possible in the PME syndrome. In this paper clinical data and findings of our 10 outpatients suffering from PME were analysed retrospectively. Electrophysiological, neuroimaging and histological examinations were performed. On the basis of the findings of the 10 patients 6 suffered from Unverricht-Lundborg disease, 1 from Lafora disease and 1 from MERRF In 2 patients we were not able to find specific diagnosis. Among our patients gypsy nationality occurs in a relatively large proportion and we know of more gypsy PME cases under care of other centres. It may raise the question whether Hungarian gypsies suffer from PME more frequently. This paper - to our knowledge – is the first study dealing with a group of PME cases in Hungary.]

Clinical Neuroscience

[Recent data for the pathobiochemistry of tension-type headache]

TAJTI János, SZOK Délia, VÉCSEI László

[The most prevalent of the independent headache disorders (30%) is tension-type headache (other types: migraine 4-10%, cluster 0.04-2.4%). These patients represent 50-90% of the patients in the "Headache Outpatient Clinics". This justifies a review of the latest biochemical data aimed at elucidating the pathomechanism of the disease. According to the criteria of the International Headache Society (IHS), two main forms of tension-type headache are distinguished. Tension-type episodic headache: fewer than 15 attacks per month, but at least 10 attacks lasting from 30 minutes to 7 days. Typically, they are pressing, tight, but not throbbing. Mild to moderate pain, bilateral, around the temples or forehead, pan-like, not increasing with normal physical activity, not accompanied by photophobia or phonophobia. Chronic tension-type headache: more than 15 attacks of headache per month, lasting all day and with features similar to the episodic form, for at least 6 months. In this summary, we first present the main experimental findings and then the conclusions that can be drawn from them. ]

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Clinical Neuroscience

The applications of transcranial Doppler in ischemic stroke


Background: This overview provides a summary of the applications of transcranial Doppler (TCD) in ischemic stroke. Results: A fast-track neurovascular ultrasound protocol has been developed for detecting occlusion or stenosis. The technique is more reliable in the carotid area than in the posterior circulation. By monitoring the pulsatility index the in­crea­sed intracranial pressure can be diagnosed. TIBI score was developed for grading residual flow. TCD has been shown to accurately predict complete or any recanalization. Regarding recanalization, TCD has a sensitivity of 92%, a specificity of 88%, a positive predictive value of 96%, a negative predictive value of 78% and an overall accuracy of 91%, respectively. Sonothrombolysis seemed to be a promising application but randomized controlled trials have shown that it does not improve clinical outcome. TCD examination can detect microembolic signals (MES) which are associated with an increased risk of stroke. Micro­em­boli were detected in symptomatic and asymptomatic carotid artery stenosis and during carotid endarterectomy. The number of microemboli can be decreased by antithrombotic therapy. Contrast en­chan­ced examination and Valsalva maneuver with continuous TCD monitoring can accurately screen for right-to-left shunt.

Clinical Neuroscience

[Systemic thrombolysis and endovascular intervention in postpartum stroke]


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Clinical Neuroscience

Comparison of hospitalized acute stroke patients’ characteristics using two large central-eastern european databases

ORBÁN-KIS Károly, SZŐCS Ildikó, FEKETE Klára, MIHÁLKA László, CSIBA László, BERECZKI Dániel, SZATMÁRI Szabolcs

Objectives – Stroke is the third leading cause of death in the European region. In spite of a decreasing trend, stroke related mortality remains higher in Hungary and Romania when compared to the EU average. This might be due to higher incidence, increased severity or even less effective care. Methods – In this study we used two large, hospital based databases from Targu Mures (Romania) and Debrecen (Hungary) to compare not only the demographic characteristics of stroke patients from these countries but also the risk factors, as well as stroke severity and short term outcome. Results – The gender related distribution of patients was similar to those found in the European Survey, whereas the mean age of patients at stroke onset was similar in the two countries but lower by four years. Although the length of hospital stay was significantly different in the two countries it was still much shorter (about half) than in most reports from western European countries. The overall fatality rate in both databases, regardless of gender was comparable to averages from Europe and other countries. In both countries we found a high number of risk factors, frequently overlapping. The prevalence of risk factors (hypertension, smoking, hyperlipidaemia) was higher than those reported in other countries, which can explain the high ratio of recurring stroke. Discussion – In summary, the comparatively analyzed data from the two large databases showed several similarities, especially regarding the high number of modifiable risk factors, and as such further effort is needed regarding primary prevention.

Hypertension and nephrology

[Cerebrovascular diseases in patients with chronic kidney disease]

KISS István, NAGY Judit

[The reason of the unfavourable life expectancy of patients with chronic kidney disease (CKD) is not only the development of end-stage renal failure but the frequent appearance of cardiovascular diseases (CVD). Chronic kidney damage itself is a cardiovascular risk state and the occurrence of CVD/associated diseases is significantly higher in chronic kidney failure. Beside risk stratification and valid treatment of CVD (hypertension, diabetes mellitus, ischemic heart disease e.g.) we and the international nephrological community have left the cerebrovascular diseases of CKD patients out of consideration. However, up to 50% of patients suffering a stroke will die immediately, only 10% of stroke survivors can continue his/her profession, but the others will be permanently disabled. High blood pressure is a strong predictor of stroke and of other CVD in most of the patients. In stroke risk reduction it is particularly important to reach the target blood pressure values. The main object of the “Live under 140/90 mmHg” programme of the Hungarian Society of Hypertension is to familiarize with target blood pressure itself and how to reach target blood pressure. In 2010, prevention, early diagnosis and management of stroke are the most important challenges of this programme (The Brain Control Programme). We think it is advisable to prepare and publish a clinical practice guideline in collaboration with stroke societies which is similar to the guidelines of international societies and of the Hungarian Society of Stroke but specific for CKD patients. This guideline would help to give a uniform, up-to-date treatment for the cerebrovascular diseases of CKD patients.]

Clinical Neuroscience

Utilization of acute vascular imaging and neurointervention for acute ischaemic stroke patients in 20 Hungarian stroke centers

POZSEGOVITS Krisztián, SZABÓ Géza, SZUPERA Zoltán, NAGY Péter, NÉMETH László, KONDÁKOR István, TUSA Csaba, BERENTE László, SALACZ Pál, VÉCSEI László, SAS Katalin, SEMJÉN Judit, NIKL János, SZAPÁRY László, KAKUK Anikó, RÓZSA Csilla, HORVÁTH Melinda, IMRE Piroska, KÖVES Ágnes, BALOGH István, MOLNÁR Sándor, FOLYOVICH András, AL-MUHANNA Nadim, BÉRES-MOLNÁR Katalin Anna, HAHN Katalin, KRISTÓF Piroska, SZÁSZ Attila Sándor, SZŰCS Anna, BERECZKI Dániel

Background - Acute mortality rate of stroke in Hungary is significantly higher than in Western Europe, which is likely to be partially attributable to suboptimal treatment. Subjects and methods - We examined the use of acute vascular imaging and mechanical thrombectomy for acute ischaemic stroke patients. We collected data on 20 consecutive patients from Hungarian stroke centres before 31st August 2016. Results - Out of the reported 410 patients, 166 (40.4%) underwent CT angiography and 44 (10.7%) had mechanical thrombectomy. Conclusion - Only about 1/3 of acute ischaemic stroke patients eligible for thrombectomy actually had it. The underlying reasons include long onset-to-door time, low utilization of acute vessel imaging and a limited neuro­intervention capacity needing improvement.