Clinical Neuroscience

[Long-term monitoring of epileptics]

SEPTEMBER 20, 1994

Clinical Neuroscience - 1994;47(09-10)

[Introduction, Indications for ETM, Qualifications and responsibilities of ETM staff, ETM instruments and processes, Instrumentation and workflows for long-term monitoring of behavioral and EEG symptoms of epilepsy,]

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Clinical Neuroscience

[Evaluation of gliomas by means of multi-model techniques]

BORBÉLY Katalin

[Gliomas constitute more than 50% of brain tumours. Primary malignant forms recur within 1/2 to 1 year after surgery, and even totally removed benign forms may recur. 50% of recurrent astrocytomas are more malignant than the original tumour. The time elapsing until recurrence strongly depends on the degree of malignity and the surgical removal. However, the age of the patient also plays an important role. Survival of the patient after the establishment of diagnosis also depends on the therapy. Effective treatment requires a knowledge of the degree of malignity as well as differentiation between recurrent tumour and radiation necrosis. CT and MRI scans offer high sensitivity, but poor specificity. Evaluation of tumour metabolism by means of 18F-fluoro-deoxy-glucose positron emission tomography (PET-FDG) helps to determine the degree of malignity of the gliomas, and recurrent tumour can safely be differentiated from necrosis following radiation therapy.]

Clinical Neuroscience

[Symptomps, localization of cerebral lesions and aetiology of "letter-by-letter" reading]

SÉRA László, MÁRKUS Atilla, BERNÁTH László

[The symptoms, localization of cerebral lesions and aetiology were analysed literally data of 78 patients suffering from pure alexia (letter-by-letter reading). During the 100 years since Déjerine's first case study on the issue was been published our knowledge on the clinical and pathomorphological characteristics of pure alexia has become more exact. On the one hand, clinical symptomatology has narrowed, eg. right homonymous hemianopsia is no regarded as a compulsory concomintant symptom on the other hand, the locus of damage underlying the symptoms may be at various areas of the brain (eg. subangular), not only at the occipital regions. The role of the posterior trajectory system in the reading process emphasized. In addition however, taking into account recent neuropsychological findings, the importance of other areas of the brain is presumable. In accordance with cognitive psychological research of the reading process it is concluded that it is essential the is for our understanding of the whole reading process that all of these factors are taken into consideration.]

Clinical Neuroscience

[Transoral and posterior fixation for inveterated fracture of odontoid process - Case report]

VERES Róbert, LAKATOS István, KENÉZ József, PENTELÉNYI Tamás

[Combined operative treatment is reported of a fixed malpositioned type III. (according to Anderson-D'Alonso) oblique anterior odontoid process fracture. Due to the ventral and dorsal compression of the spinal canal a part of the dens and a part of the C.li body were transorally removed, and a part of the arch of the atlas also removed by a dorsal approach. The position was fixed by means of a combined method of a transoral Harms-plate and a posterior C.I.-C.II. fransarticular screwing according to Magerl. With this method were achiered proper decompression and stabile C.1.-C.II. arthrodesis.]

Clinical Neuroscience

[Cerebral aspergillosis]

ILLÉS Zsolt, GARZULY Ferenc, BRITTIG Ferenc, PERENYEI Miklós

[Four cases of cerebral aspergillosis are reported. Brain abscesses developed in a patient with chronic alcoholism and pyogenic urogenital process, and in a baby, treated for aplastic anaemia. Haemorrhagic necroses were found in the other two cases, one of them suffered from agressive hepatitis, the other had lymphoid leukaemia. Pulmonary alterations were observed as part of generalised aspergillosis in all the cases. Early diagnosis of pulmonary processes should be emphasized as the disease can hardly be influenced when neurologic symptoms appear.]

Clinical Neuroscience

[The effect of the cue-controlled modification of the level of vigilance on the intentional inhibition of seizure in patients with partial epilepsy]

SZUPERA Zoltán, RUDISCH Tibor, BONCZ István

[The cue-controlled modification of the level of vigilance, as one of the methods of self-control appeared to be a practicable therapeutic intervention for the intentional inhibition of epileptic seizures in some special cases of epilepsy. The authors worked out a variation of the above self-control technique, in which the aura imagined in hypnosis was associated with the change in vigilance in patients suffering from partial epilepsy, in order to enable them to try to inhibit the epileptic fits with this associated modification of the level of alertness during auras. The authors report two cases of intractable partial epileptic patients, in which the patients attained the application of this self-control method. The first patient carried out intentional seizure inhibition in 73 cases over one year, reducing the frequency of the fits from the previous 115 to 77. In the second case, the patient was able to decrease considerably even the number of epileptic auras by learning and applying the technique, consequently the number of auras decreased to 7 compared with the 38 fits observed in the previous 8 months, further he was able to terminate the attack in 6 cases out of auras. The authors think, that their method might be useful for a certain group of patients suffering from partial epilepsy to inhibit epileptic attacks, and would mean a new possibility in the management of intractable cases.]

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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.

Clinical Neuroscience

[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. ]

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.

Lege Artis Medicinae

[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.]

Lege Artis Medicinae

[Current issues in the peripartum management of diabetic women from the perspective of an internist-diabetologist]

KERÉNYI Zsuzsanna

[In pregnancy complicated with diabetes, treatment of hyperglycaemia is of fundamental importance during delivery in order to improve the outcome parameters of both the mother and the neonate. This is particularly important in the case of mothers with type 1 diabetes and of all mothers who require insulin treatment during their pregnancy. The use of antenatal steroids for women at risk of pre-term birth further complicates the treatment of hyperglycaemia in the period immediately before delivery and requires the appropriate change of insulin therapy. The requirement of nil per os in the delivery period necessitates proper fluid, glucose and insulin treatment in the pre-delivery hours. After surgical delivery the patients may also need infusion treatment until the first meal. As there is no unified guideline for the peripartum management of diabetes, the author re­views the international literature on the internal medicine issues concerning the peripartum treatment of pregnant women with diabetes. This study reviews the characteristics of insulin treatment of women with various types of diabetes before, du­ring and di­rect­ly after delivery. It presents a dosing schedule for women who needed an antenatal steroid treatment in the period before delivery due to premature birth for the purpose of lung maturation. The study also addresses the application and programming of pe­ripartum blood glucose tests, continuous interstitial glucose monitoring (CGM) and insulin pump treatment (CSII).]