Clinical Neuroscience

[Long-term monitoring of epileptics Part 2]

NOVEMBER 20, 1994

Clinical Neuroscience - 1994;47(11-12)

[VI. Technical and methodological considerations. VII. Recommended use of specific ETM systems. VIII. Minimum standard requirement for specific indications in practice. IX. Guidelines for written evidence of ETM.]

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Clinical Neuroscience

[Clinico-morphological correlation in common dementias]

PAPP I. Mátyás

[ln the late sixties the revelation that more than half of the dementia cases in elderly people were caused by Alzheimer's disease and not by „cerebral arteriosclerosis" gave dementia research a new impetus, which accelerated in the eighties and has nowadays become explosive and has supplied us with a mass of data almost beyond comprehension. Nevertheless, even though it is most intensively, studied elucidation of the etiology and pathogenesis of the Alzheimer' s disease is still not expectable in the near future. ln addition to Alzheimer's disease, new results basically changing our views, have been achieved in the field of the vascular dementia and in dementias with Parkinsonian syndrome. Frontostriatal circuits discovered recently have helped our understanding of the subcortical dementia in these diseases and in others. By means of neuroimaging techniques, several dementia syndromes - paramedian thalamic infarcts, the watershed infarcts and the so called Binswanger's disease - have turned out to be much more common than previously believed. Clinicopathological observations have verified the role of the damage to the limbic and paralimbic structures, frontostriatal circuits and association pathways in the pathomechanism of dementias. ]

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[Typical behavior models of dealing with epilepsy - the models present between patient and members of the family]

NEMESSÚRI Judit, TÖLGYES Tamás, VERES Judit, RAJNA Péter

[Four are main symptom groups described which were previously considered to be not more than an organic consequence of epilepsy. In most cases in the background of these groups a pathogenic/ pathogenetic role of typical relationship constellations between the patient and his/her relatives can be observed. These constellations are illustrated by case descriptions. It is pointed out that: 1. Epilepsy is a chronic illness in the course of which in every case certain psychodynamic events take place between the patient and his or her relatives in the immediate environment. 2. According to the present „neurological" approach and to the social expectations in the course of the patients' care the main stress is on biological events and changes. As a result intrapsychic and social-psychiatric motives are frequently manifested in the form of biological symptoms, too. 3. It follows from the above that the psychic constellation is interiorized by the patient and then he/she expresses it through somatic symptoms. At first sight these symptoms seem to be typical epileptic problems”. In actual fact, somatic „epileptologic” treatment cannot be regarded as succesful. 4. Since care is mainly based on auto- and heteroanamnestic data the phenomena discussed earlier could be highlighted only by means of the simultanious exploration of the family in question and by possible disagreement between the patient and his/her environment. 5. In order to treat the epileptic patient it is essential to consider the illness in relation to the patient and the members of the family.]

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[Characteristics of gadolinium-enhancement in ischemic stroke]

KENÉZ József, BARSI Péter, KULIN Árpád, NAGY Zoltán

[Stroke is a clinical diagnosis. In acute stroke, CT is the first examination of choice to exclude hemorrhage. In ischemic stroke, MR detects the changes earlier and more exactly, than CT. Contrast-enhanced MR imaging shows specific enhancement phenomena, viz. Intravasal high signal in the vessels of the ischemic cerebral region, meningeal enhancement, transitorial, mixed type enhancement and parenchymal enhancement. Our paper is deals with the causes and diagnostic significance of the different types of these enhancement effects, and discusses some differential diagnostic conclusions. In the near future, after installing more modern MR equipment, a more exact knowledge of the pathomechanism of stroke and, as a consequence, new and more effective therapies can be expected.]

Clinical Neuroscience

[Determination of serum C-reactive protein in the illnesses of central nervous system]

PINTÉR Erzsébet, KÁLI Gábor, VUKMIROVITS György, NIKOLOVA Radka, YOUSEF M. Gassar, GYARMATI Éva

[Serum quantitative C-reactive protein concentrations were measured in 186 patients admitted with suspected neuroinfection or symptoms of other diseases in the central nervous system. The diagnoses were purulent meningitis (102), abscessus cerebri (5), meningitis basilaris (2), meningitis cryptococcica (1), sepsis (11), encephalitis (22), encephalopathia (5), autoimmune central nervous illness/disease/disorder (22), non-inflammatory central nervous disease/ disorder (12) and tumour cerebri (4). The highest values of C-reactive protein were measured in patients suffering from abscessus cerebri (250.8 +/ 64.2 mg/l), sepsis (221 +/- 16.4 mg/l) and purulent meningitis (153.0 +/- 94.59 mg/l). Serum C-reactive protein concentrations were moderately increased in viral infections (36.8 +/- 36 mg/l). The C-reactive protein level in the normal range in patients suffering from non-inflammatory and autoimmune central nervous illnesses/diseases/ disorders (4.0 +/- 1.5 mg/l or 10.0 +/- 18.5 mg/l). During our examinations the C-reactive protein levels showed both local and septicaemic reactions. C-reactive protein is considered as a valuable laboratory parameter both in diagnosings central nervous system diseases with suspected bacterial infection and in monitoring therapeutic efficiency.]

Clinical Neuroscience

[Chronic recurrent transverse myelitis or multiple sclerosis]

UNGUREÁN Aurélia, PÁLFI Sándor, DIBÓ György, VÉCSEI László

[The simultaneous appearance of multiple sclerosis and transverse myelitis is a known phenomenon. Apart from the appearance of spinal symptoms, other neurological signs of the brain involvement can urually be found, MRI shows disseminated foci in the brain. The positive evoked responses, the immunological abnormalities of the CSF, and the oligoclonality together prove the presence of multiple sclerosis. In these cases the symptoms of transverse myelitis may precede other signs of multiple sclerosis, or appear separately as a relapse. Recurrent transverse myelitis as an independent entity with negative MRI and CSF immunology, is an exciting topic concerning the etiologic factors and therapeutical considerations. Tvolging from the literature, the appearance of transverse myelitis as an independent entity, is rather rare. Details are given of a female patient with recurrent spinal cord signs, negative MRI and CSF immunology. During her ten-year follow-up no, symptoms of multiple sclerosis have developed. Further studies are needed to clarify the etiological factors, pathomechanism and therapeutic considerations of this relatively new and probably independent clinical entity.]

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Fluoxetine use is associated with improved survival of patients with COVID-19 pneumonia: A retrospective case-control study

NÉMETH Klára Zsófia, SZÛCS Anna , VITRAI József , JUHÁSZ Dóra , NÉMETH Pál János , HOLLÓ András

We aimed to investigate the association between fluoxetine use and the survival of hospitalised coronavirus disease (COVID-19) pneumonia patients. This retrospective case-control study used data extracted from the medical records of adult patients hospitalised with moderate or severe COVID-19 pneumonia at the Uzsoki Teaching Hospital of the Semmelweis University in Budapest, Hungary between 17 March and 22 April 2021. As a part of standard medical treatment, patients received anti-COVID-19 therapies as favipiravir, remdesivir, baricitinib or a combination of these drugs; and 110 of them received 20 mg fluoxetine capsules once daily as an adjuvant medication. Multivariable logistic regression was used to evaluate the association between fluoxetine use and mortality. For excluding a fluoxetine-selection bias potentially influencing our results, we compared baseline prognostic markers in the two groups treated versus not treated with fluoxetine. Out of the 269 participants, 205 (76.2%) survived and 64 (23.8%) died between days 2 and 28 after hospitalisation. Greater age (OR [95% CI] 1.08 [1.05–1.11], p<0.001), radiographic severity based on chest X-ray (OR [95% CI] 2.03 [1.27–3.25], p=0.003) and higher score of shortened National Early Warning Score (sNEWS) (OR [95% CI] 1.20 [1.01-1.43], p=0.04) were associated with higher mortality. Fluoxetine use was associated with an important (70%) decrease of mortality (OR [95% CI] 0.33 [0.16–0.68], p=0.002) compared to the non-fluoxetine group. Age, gender, LDH, CRP, and D-dimer levels, sNEWS, Chest X-ray score did not show statistical difference between the fluoxetine and non-fluoxetine groups supporting the reliability of our finding. Provisional to confirmation in randomised controlled studies, fluoxetine may be a potent treatment increasing the survival for COVID-19 pneumonia.

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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

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

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[The connection between the socioeconomic status and stroke in Budapest]

VASTAGH Ildikó, SZŐCS Ildikó, OBERFRANK Ferenc, AJTAY András, BERECZKI Dániel

[The well-known gap bet­ween stroke mortality of Eastern and Western Euro­pean countries may reflect the effect of socioeconomic diffe­rences. Such a gap may be present between neighborhoods of different wealth within one city. We set forth to compare age distribution, incidence, case fatality, mortality, and risk factor profile of stroke patients of the poorest (District 8) and wealthiest (District 12) districts of Budapest. We synthesize the results of our former comparative epidemiological investigations focusing on the association of socioeconomic background and features of stroke in two districts of the capital city of Hungary. The “Budapest District 8–12 project” pointed out the younger age of stroke patients of the poorer district, and established that the prevalence of smoking, alcohol-consumption, and untreated hypertension is also higher in District 8. The “Six Years in Two Districts” project involving 4779 patients with a 10-year follow-up revealed higher incidence, case fatality and mortality of stroke in the less wealthy district. The younger patients of the poorer region show higher risk-factor prevalence, die younger and their fatality grows faster during long-term follow-up. The higher prevalence of risk factors and the higher fatality of the younger age groups in the socioeconomically deprived district reflect the higher vulnerability of the population in District 8. The missing link between poverty and stroke outcome seems to be lifestyle risk-factors and lack of adherence to primary preventive efforts. Public health campaigns on stroke prevention should focus on the young generation of socioeconomi­cally deprived neighborhoods. ]

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[Risk of nonsteroidal antiinflammatory drugs. Focus on aceclofenac]

FARSANG Csaba

[Nonsteroidal antiinflammatory drugs (NSAIDs) are among the most frequently used pharmaceuticals. Nevertheless, a number of studies emphasized that NSAIDs were damaging not only the gastrointestinal (GI), but also the cardiovascular (CV) system, could increase the blood pressure, the frequency of coronary events (angina, myocardial infarction) and stroke incidence, as well as they might deterio­rate renal functions. The National Institute for Health and Care Excellence (NICE) did not find evidence that administering NSAIDs could increase the risk of developing COVID-19 or worsened the condition of COVID-19 patients. However, unwanted effects of specific drugs differ substantially in their occurrence and seriousness as well. It seemed to be for a long time that the NSAIDs provoked higher GI-risk was closely related to the COX1/COX2 selectivity, like the cardiovascular (CV) risk to the COX2/COX1 selectivity, however, the recent data did not prove it clearly. Based on the available literature while pondering the gastrointestinal and cardiovascular adverse events, among all NSAIDs the aceclofenac profile seemed to be the most favourable.]

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.