Clinical Neuroscience

[PhD thesis Asphyxia-induced brain oedema formation in newborn pigs: a particular role for histamine]

KOVÁCS József

JULY 10, 2004

Clinical Neuroscience - 2004;57(07-08)

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Clinical Neuroscience

[Myasthenia in a patient with sarcoidosis and schizophrenia (in English language)]

RÓZSA Csilla, KIS Gábor, KOMOLY Sámuel

[A 44-year-old male patient was hospitalised with paranoid schizophrenia in 1985. Depot neuroleptic treatment was started which successfully prevented further psychotic relapses for the next ten years. His myasthenia gravis started with bulbar signs in 1997 and the symptoms soon became generalized. The diagnosis of myasthenia gravis was confirmed by electromyography, by positive anticholinesterase test and by the detection of anti-acetylcholine receptor antibodies in the serum. Mediastinal CT examination showed enlarged hilar lymph nodes on the left but no thymic pathology was observed. Mediastinoscopy was performed and biopsies were obtained from the affected nodes. Histology revealed sarcoidosis. The patient suffered respiratory crisis following the thoracic intervention (in September 1998). Combined oral corticosteroid (64 mg methylprednisolone/e.o.d.) and azathioprine (150 mg/day) treatment regimen was initiated and complete remission took place in both the myasthenic symptoms and the sarcoidosis. The follow-up CT scans showed no mediastinal pathology (January 2000). During steroid treatment a transient psychotic relapse occured which was successfully managed by supplemental haloperidol medication added to his regular depot neuroleptics. The patient currently takes 150 mg/day azathioprine and receives 40 mg/month flupentixol depot im. His physical and mental status are stable and he has been completely symptome free in the last 24 months. The association of myasthenia gravis and sarcoidosis is very rare. To our best knowledge no case has been reported of a patient suffering from myasthenia gravis, sarcoidosis, and schizophrenia at the same time.]

Clinical Neuroscience

[“From brain - through mind - to society“ XIII. International Semmelweis Symposium]

Clinical Neuroscience

[Prognosis and classification of hypertensive striatocapsular haemorrhages]

HORNYÁK Csilla, KOVÁCS Tibor, PAJOR Péter, SZIRMAI Imre

[Introduction - Nontraumatic intracerebral haemorrhage accounts for 10 to 15% of all cases of stroke. Patients and method - In our study hypertensive striatocapsular haemorrhages were divided into six types on the basis of arterial territories: posterolateral, lateral, posteromedial, middle, anterior and massive (where the origin of the hemorrhage can not be defined due to the extensive damage of the striatocapsular region) type. We analysed laboratory data, clinical presentations and risk factors as alcoholism, smoking and hypertension of 111 cases. The size of the hematoma, midline shift and severity of ventricular propagation were measured on the acute CT-scan. The effect on the 30-day clinical outcome of these parameters were examined Results and conclusion - According to our results, the most important risk factor of hypertensive intracerebral haemorrhage was chronic alcoholism. Blood cholesterol, triglyceride levels and coagulation status had no effect on the prognosis, but high blood glucose levels Significantly worsen the clinical outcome. In our study, lateral striatocapsular haemorrhage was the most common while middle one was the least common type. The overall mortality is 42%, but differs by the type. The 30-day outcome significantly depends on the type of the haemorrhage, the initial level of consiousness, the size of the haematoma, the severity of ventricular propagation, the midline shift and the blood glucose levels. The clinical outcome proved to be the best in the anterior type, good in the posteromedial and lateral types. The prognosis of the massive type is poor. In our study, the classes and the mortality of the striatocapsular haemorrhages was different from the literature data. The higher mortality in our cohort could be due to the longer follow-up and the severe accompanying diseases of our patients.]

Clinical Neuroscience

[Alcohol as a risk factor for hemorrhagic stroke (in English language)]

SAJJAN Daniel, BERECZKI Dániel

[Purpose - Whereas the protective effect of mild-to-moderate alcohol consumption against ischemic stroke has been well recognized, there is conflicting evidence regarding the link between alcohol consumption and hemorrhagic strokes. The aim of the present study is to summarize the results of case-control and cohort studies published on this issue. Methods - Recent epidemiologic articles on the relationship between alcohol consumption and hemorrhagic stroke were identified by Medline searches limited to title words using the following search terms: ”alcohol AND cerebrovascular dis*”, ”alcohol AND stroke”, ”alcohol AND cerebral hemorrhage” and ”alcohol AND hemorrhagic stroke”. Results - Most case-control and cohort studies either repor-ted only on total strokes or on a combined group of hemorr-hagic strokes including intracerebral as well as subarachnoid hemorrhages. There was a consensus among reports that heavy alcohol consumption was associated with a higher risk of hemorrhagic strokes. Controversy remains regarding the effect of mild-to-moderate alcohol consumption: while some studies reported a protective effect, others found a dose-dependent linear relationship between the amount of alcohol consumed and the risk of hemorrhagic stroke. The differential effect of moderate alcohol consumption on hemorrhagic compared to ischemic strokes is mostly attributed to alcohol- and withdrawal- induced sudden elevations of blood pressure, and coagulation disorders. Conclusions - Heavy drinking should be considered as one of the risk factors for hemorrhagic stroke. In contrast to the protective effect of mild-to-moderate alcohol use against ischemic strokes, moderate drinking might result in an increased risk of hemorrhagic strokes.]

Clinical Neuroscience

[Differentiation of parkinsonian and essential tremor by the use of electrophysiological methods]

GERTRÚD Tamás, FELYÉR Dénes, MAGYAR András, PÁLVÖLGYI László, TAKÁTS Annamária, SZIRMAI Imre, KAMONDI Anita

[Objective - Tremor is one of the most common movement disorders. Different tremors are induced by central and/or peripheral oscillators. The motor cortex plays a significant role in the generation of parkinsonian tremor but its function in essential tremor is not clear. We examined the effect of motor cortex activation on parkinsonian and essential tremor during movement of the contralateral hand. Our aim was to study the role of interhemispheric motor connections in genesis of different tremors. Patients and methods - We recorded the tremor of nine Parkinson patients and seven patients suffering from essential tremor using accelerometry. After Fast Fourier-transformation of digitized tremor signal we measured the power changes at the peak frequency after flash triggered movement (FM) and self-paced movement (SPM). For control we used flash signal without movement. Results - Peak frequency of parkinsonian and essential tremor was not different. The power decrease of parkinsonian tremor was significant during flash triggered and self-paced movement compared to the effect of flash (pFlash-FM=0.0008; pFlash-SPM=0.002), changes during the different movement protocols were not different (pFM-SPM=0.33). During self-paced movement parkinsonian tremor became significantly smaller than essential tremor (p<0.05). The effect of movement was not significant on the power of essential tremor (p=0.42), probably due to high standard deviation of individual data. Conclusions - Voluntary movement of the contralateral hand decreases parkinsonian tremor suggesting that its generator can be inhibited via the activation of the motor cortex. The diverse reaction of essential tremor may reflect various connections between its generator system and the motor areas, therefore it is not a separate disease entity.]

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Clinical Neuroscience

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 role of sleep in the relational memory processes ]

CSÁBI Eszter, ZÁMBÓ Ágnes, PROKECZ Lídia

[A growing body of evidence suggests that sleep plays an essential role in the consolidation of different memory systems, but less is known about the beneficial effect of sleep on relational memory processes and the recognition of emotional facial expressions, however, it is a fundamental cognitive skill in human everyday life. Thus, the study aims to investigate the effect of timing of learning and the role of sleep in relational memory processes. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. Our results suggest that the timing of learning and sleep plays an important role in the stabilizing process of memory representation to resist against forgetting.]

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

[Diagnosis and treatment of microvascular coronary heart disease. Specialities of conditions in Hungary]

SZAUDER Ipoly

[Invasive investigations show that in two-thirds of patients the myocardial ischaemia persists without obstructive coronary disease and any other heart conditions (INOCA). The underlying cause may be microvascular dysfunction (CMD) with consecutive microvascular coronary disease (MVD) and microvascular or epicardial vasospastic angina (MVA). The modern practice of clinical cardiology while using the developed non-invasive cardiac imaging permits exact measuring of the coronary flow with its characteristic indices. All of these improve the diagnosing of CMD-induced myocardial ischemia and provide opportunity to determine primary MVD cases. Since the recognition and treatment of MVD is significantly underrep­resented in the Hungarian medical care, the primary stable microvascular angina (MVA) is described in detail below with its modern invasive and non-invasive differential diagnosis and treatment, concerning especially its frequency provoked by high blood pressure and female coronary heart diseases. There are highlighted all recommended diagnostic procedures available under domestic conditions.]

Hypertension and nephrology

[Association between cyclothymic affective temperament and hypertension]

NEMCSIK János, BATTA Dóra, KŐRÖSI Beáta, RIHMER Zoltán

[Affective temperaments (cyclothymic, hypertymic, depressive, anxious, irritable) are stable parts of personality and after adolescent only their minor changes are detectable. Their connections with psychopathology is well-described; depressive temperament plays role in major depression, cyclothymic temperament in bipolar II disorder, while hyperthymic temperament in bipolar I disorder. Moreover, scientific data of the last decade suggest, that affective temperaments are also associated with somatic diseases. Cyclothymic temperament is supposed to have the closest connection with hypertension. The prevalence of hypertension is higher parallel with the presence of dominant cyclothymic affective temperament and in this condition the frequency of cardiovascular complications in hypertensive patients was also described to be higher. In chronic hypertensive patients cyclothymic temperament score is positively associated with systolic blood pressure and in women with the earlier development of hypertension. The background of these associations is probably based on the more prevalent presence of common risk factors (smoking, obesity, alcoholism) with more pronounced cyclothymic temperament. The scientific importance of the research of the associations of personality traits including affective temperaments with somatic disorders can help in the identification of higher risk patient subgroups.]