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.]

AFFILIATIONS

  1. Országos Idegsebészeti Tudományos Intézet, Neuroradiológiai Osztály, Budapest

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[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.]

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

LEEL-ŐSSY Lóránt

[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.]

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[Progressive myoclonus epilepsy - A review and case histories]

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[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.]

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[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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[The new coronavirus, SARS-CoV-2, which causes the COVID-19 disease can lead to severe acute respiratory distress syndrome (ARDS). It poses a serious challenge to the health care system, especially intensive care. Neurological patients, usually of advanced age and with a myriad of comorbidities, are at particular risk through the impact of the new coronavirus on their condition and nutritional capacity. Stroke is a leader in morbidity and mortality data, with a focus on dysphagia and its complications due to COVID-19 disease and acute cerebrovascular accident. In the acute phase of stroke, 30-50% of patients suffer from dysphagia, which still shows a prevalence of 10% six months later. Dysphagia results in decreased or insufficient fluid and nutrient uptake, supp­lemented by inactivity, leading to malnutrition and sarcopenia, which worsens overall condition, outcome, and rehabilitation efficiency. Screening and early detection of swallowing disorders is a fundamental issue in order to develop a personalized and timely-initiated nutritional therapy strategy. Nutritional therapy plays a key role in frequent intensive care due to COVID-19 disease, where it increases the chances of recovery and reduces the length of stay in the intensive care unit and mortality. This is especially true in critically ill patients requiring prolonged ventilation. In COVID-19 diagnosed patients, screening for dysphagia, bedside assessment, and instrumental examination, followed by swallowing rehabilitation, are of paramount importance. Stroke can also be a complication of the COVID-19 infection. Care for cerebrovascular patients has also adapted to the pandemic, “triazination” has become systemic, and dysphagia screening for stroke patients and nutritional therapy adapted to it have also shed new light. ]

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[The efficacy of intravenous thrombolysis (IVT) is moderate in the proximal vascular segments of intracranial arteries, as opposed to mecha­nical thrombectomy (MT). In the management of acute ischemic stroke (AIS) caused by large vessel occlusions (LVO), IVT prior to MT is highly recommended based on the latest guidelines, but the necessity of IVT has been questioned by the latest studies of the past years. The aim of our study was to investigate and compare the efficacy and safety of direct mechanical thrombectomy (dMT) and combined therapy (CT) for patients who suffered an AIS with LVO and were treated in our department. We investigated patients with AIS caused by LVO who were admitted up to 4.5 hours after symptom onset and underwent MT in our department between November 2017 and August 2019. Patients’ data were collected in our stroke register. Patients enrolled in our study were divided into two groups depending on whether dMT or CT was used. Our primary outcome was the 30- and 90- day functional outcome measured by modified Rankin Scale (mRS). Mortality at 30- and 90- day, successful recanalization rates, and symptomatic intracranial hemorrhage were considered as secondary outcomes. A total of 142 patients (age: 68.3 ± 12.6 years, 53.5% female) were enrolled in our study, including 81 (57.0%) dMT cases, and 61 (43.0%) patients who received CT. The vascular risk factors and comorbidities were significantly higher in the dMT-treated group. At day 30, the rate of favorable functional outcomes was 34.7% in dMT vs. 43.6% among those who received CT (p = 0.307), by day 90 this ratio changed to 40.8% vs. 46.3% (p = 0.542). Mortality rates at day 30 were 22.2% and 23.6% (p = 0.851), and at day 90 33.8% and 25.9% (p = 0.343). The rate of effective recanalization was 94.2% for dMT-treated patients and 98.0% for CT-treated patients (p = 0.318). Symptomatic intracranial hemorrhage was detected in 2.5% of dMT-treated patients and 3.4% of CT-treated group (p = 0.757). Our results suggest that CT is associated with a moderately better outcome compared to dMT. IVT prior to MT did not increase the risk of symptomatic intracranial hemorrhages.]

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[Systemic thrombolysis and endovascular intervention in postpartum stroke]

BERECZKI Dániel Jr., NÉMETH Beatrix, MAY Zsolt, SZAKÁCS ZOLTÁN, GUBUCZ István, SZIKORA István, SZILÁGYI Géza

[Introduction - There are no previously published cases about intravenously applied recombinant tissue plasminogen activator in acute ischemic stroke during puerperium. Case presentation - We report a 40-year-old woman with postpartum acute ischemic stroke caused by multiple cervical artery dissections treated by systemic thrombolysis and endovascular intervention. Discussion - There are only limited data regarding thrombolytic treatment in acute stroke during pregnancy and puerperium. Current acute stroke treatment guidelines - while considering pregnancy as a relative exclusion criterion - do not deal with the postpartum state. Conclusion - As the condition is rare, randomized controlled trials are not feasible, therefore further reports on similar cases could eventually help us suggest guidelines or at least propose recommendations for the acute thrombolytic treatment of strokes occurring in pregnancy and puerperium.]