Clinical Neuroscience

[Report on a research project from abroad (EFNS)]

ERTSEY Csaba

AUGUST 20, 2003

Clinical Neuroscience - 2003;56(07-08)

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Clinical Neuroscience

[6th National Congress of the Hungarian Stroke Society]

Clinical Neuroscience

[Imaging of dopamine transporter with 99mTc-TRODAT-SPECT in movement disorders]

KANYÓ Balázs, ÁRGYELÁN Miklós, DIBÓ György, SZAKONYI Zsolt, VÉCSEI László, FÜLÖP Ferenc, LÁNCZ Adrienn, FORGÁCS Péter, PÁVICS László

[99mTc-TRODAT-1 is a new, technetium based radiopharmaceutical that selectively binds to the dopamine transporters. The aim of the study was to evaluate the dopamine transporter status in movement disorders. Methods - In eight healthy volunteers (age range 22-58 years), 28 patients with Parkinson’s disease (age range 42-80 years), 10 patients with Parkinsonian syndrome (age range 51-79 years) and 13 patients with essential tremor (age range 43-71 years) were 99mTc-TRODAT-SPECT tests performed. The results were evaluated visually and semiquantitatively. Results - The visual assessments were concordant with those of the semiquantitative in each case. The 99mTc- TRODAT uptake of the striatum was referenced to the cerebellum, the frontal and occipital cortex. The best deviation was found in aspect of the occipital cortex. The striatum/occipital ratio was the following: healthy volunteers: 2.12±0.27; Parkinson’s disease: 1.52±0.27; Parkinsonian syndrome: 1.57±0.26; essential tremor: 2.06±0.69. The striatal dopamine transporter availability was significantly lower in subjects with Parkinson's disease or Parkinsonian syndrome compared to the control subjects. There was no difference between healthy volunteers and patients with essential tremor. Using discriminant analysis, the discriminant function had significantly different values in the group of Parkinson’s disease than in Parkinsonian syndrome: f= -3.675×caud/occipit+6.293×put/occipit -2.548. Conclusion - 99mTc-TRODAT-SPECT is able to visualise the presynaptic dopaminergic degeneration. This method itself can be useful in differential diagnosis in some type of movement disorders.]

Clinical Neuroscience

[Two cases of frontotemporal dementia]

SZABÓ Erzsébet, SZABÓ Mihály

[Frontotemporal dementias represent the third most common cause of primer degenerative dementias next to Alzheimer’s disease and Lewy body disease. Frontotemporal dementia constitutes 10-20% of all praesenilis dementias. The authors present the results of the 10 years' clinical, neuropsychological, neuropathological examinations and brain imaging with the examples of two cases. At the early stage of frontotemporal dementia changes of personality and social conduct are prominent, whereas cognitive functions are relativelly well preserved. The usual dementia tests are not sufficiently sensitive to disclose noncognitive symptoms. Clinical diagnosis as well as differentiation from functional psychiatric disorders can be difficult. Brain imaging present the frontal and the anterior temporal lobe atrophy and selective hypometabolism in these areas. The typical onset is between at the age 50 and 65 years. It is very rare under the age of 30. The symptoms of two patients started at the age of 42-44. The first diagnosis was post traumatic stress disorder. Later stereotyped behaviour, mental rigidity, hyperorality, irritability, progressive reduction of speech and vegetative dysfunctions appeared. Besides the affecting of the irresistibly worsening symptoms and the medical care requiring strength and inventiveness, the authentic informing of the relatives is also a challenge. The caregivers have special relationship with the patients and their relatives.]

Clinical Neuroscience

[Apoptosis in focal brain ischaemia]

ZÁDOR Zsolt, LACZA Zsombor, BENYÓ Zoltán, HARKÁNY Tibor, HORTOBÁGYI Tibor

[Ischaemic stroke is one of the major causes of death and disability in the developed world. It is caused by focal impairment of cerebral blood flow. The subsequent ischaemic cell death is predominantly necrotic in nature. However, a therapeutically important characteristic is the delayed apoptotic cell demise in the border zone of the primary lesion core. Apoptosis is one of the most intensively studied field of current medical and biological research. The better understanding of its mechanism may provide novel and more effective ways of therapy in a wide range of diseases including ischemic stroke. The salient neurological features of focal brain ischaemia and the morphological signs of apoptotic and necrotic cell death are summarized. The mechanism of apoptosis is discussed. It is divided into an early genetic phase of decisionmaking followed by a cellular execution phase. The characteristics of the early shift in the finely tuned balance of proand antiapoptotic genes and their protein products, which is preceded by an inbalance in intracellular ionized calcium homeostasis, energy depletion and mitochondrial dysfunction is discussed. The crucial role of caspases in apoptosis is emphasized. The three possible pathways during the execution phase is described: the intrinsic- and extrinsic caspase activation cascade and the caspase-independent intracellular signal transduction route. The molecular mechanism of neural cell membrane damage in the execution phase is discussed and some examples of altered protein synthesis also known as message-selection are given. The important role of late reperfusion in the execution phase is emphasized. The possible targets of antiapoptotic therapeutic approaches and the results of experimental studies are presented as well as the perspectives of their use in human clinical care.]

Clinical Neuroscience

[Positron emission tomography in presurgical localization of epileptic foci]

JUHÁSZ Csaba

[The success of cortical resection for intractable epilepsy of neocortical origin is highly dependent on the accurate presurgical delineation of the regions responsible for generating seizures. In addition to EEG and structural imaging studies, functional neuroimaging such as positron emission tomography (PET) can assist lateralization and localization of epileptogenic cortical areas. In the presented studies, objectively delineated focal PET abnormalities have been analyzed in patients (mostly children) with intractable epilepsy, using two different tracers: 2-deoxy-2-[18F]fluoro-D-glucose (FDG), that measures regional brain glucose metabolism, and [11C]flumazenil (FMZ), that binds to GABAA receptors. The PET abnormalities were correlated with scalp and intracranial EEG findings, structural brain abnormalities, as well as surgical outcome data. In patients with extratemporal foci and no lesion on MRI, FMZ PET was more sensitive than FDG PET for identification of the seizure onset zone defined by intracranial EEG monitoring. In contrast, seizures commonly originated from the border of hypometabolic cortex detected by FDG PET suggesting that such areas are most likely epileptogenic, and should be addressed if subdural EEG is applied to delineate epileptic cortex. In patients with cortical lesions, perilesional cortex with decreased FMZ binding was significantly smaller than corresponding areas of glucose hypometabolism, and correlated well with spiking cortex. Extent of perilesional hypometabolism, on the other hand, showed a correlation with the life-time number of seizures suggesting a seizurerelated progression of brain dysfunction. FMZ PET proved to be also very sensitive for detection of dual pathology (coexistence of an epileptogenic cortical lesion and hippocampal sclerosis). This has a major clinical importance since resection of both the cortical lesion and the atrophic hippocampus is required to achieve optimal surgical results. Finally, the author demonstrated that in patients with neocortical epilepsy, FDG PET abnormalities correctly regionalize the epileptogenic area, but their size is not related to the extent of epileptogenic tissue to be removed. In contrast, complete resection of cortex with decreased FMZ binding predicts good surgical outcome suggesting that application of FMZ PET can improve surgical results in selected patients with intractable epilepsy of neocortical origin.]

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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

Autonomic nervous system may be affected after carpal tunnel syndrome surgery: A possible mechanism for persistence of symptoms after surgery

ONDER Burcu, KELES Yavuz Betul

After carpal tunnel surgery, some patients report complaints such as edema, pain, and numbness. Purpose – The aim of this study was to evaluate autonomic nervous system function in patients with a history of carpal tunnel surgery using sympathetic skin response (SSR). Thirty three patients (55 ±10 years old) with a history of unilateral operation for carpal tunnel syndrome were included in the study. The SSR test was performed for both hands. Both upper extremities median and ulnar nerve conduction results were recorded. A reduced amplitude (p=0.006) and delayed latency (p<0.0001) were detected in the SSR test on the operated side compared to contralateral side. There was no correlation between SSR and carpal tunnel syndrome severity. Although complex regional pain syndrome does not develop in patients after carpal tunnel surgery, some of the complaints may be caused by effects on the autonomic nervous system.

Clinical Neuroscience

Extraskeletal, intradural, non-metastatic Ewing’s sarcoma. Case report

OTTÓFFY Gábor, KOMÁROMY Hedvig

Intracranial localization of Ewing’s sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing’s sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re­gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima­ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.

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.]

Clinical Neuroscience

Valproic acid associated pleuropericardial effusion: case report

DEMIR Figen Ulku

Introduction - Valproic acid is an effective antiepileptic and mood stabilizer used in the treatment of many neurological and psychiatric disorders. Although there are frequently seen side effects, effusions between layers of pleural and pericardial membranes are rare to be seen. Case - Pleuropericardial effusion was detected in a 23 years old woman who was under valproic acid treatment because of epileptic seizure. After 1 year of valproic acid treatment, patient complained of dyspnea. As all the researches intended on etiology were usual, valproic acid has been thought to be responsible for the matter. Control examination after 1.5 months regarding the end of treatment revealed complete recovery of pleuropericardial effusion. Discussion - Pleural and pericardial effusions are rarely seen complications related to the use of valproic acid. It must also be kept in mind that valproic acid causes a potential for such side effects which can be blamed etiologically when the other possibilities for patients are excluded.