Clinical Neuroscience

[31th Congress of the Hungarian Pediatric Neurological, Neurosurgical, Pediatric and Adolescent Psychiatric Society]

NAGY Andrea

JANUARY 20, 2007

Clinical Neuroscience - 2007;60(01-02)

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Clinical Neuroscience

[THE FAMILIAL INCIDENCE OF EPILEPSY IN THE GROUP OF EPILEPTIC PATIENTS EXAMINED AFTER THEIR FIRST SEIZURE - PILOT STUDY]

RÓZSAVÖLGYI Margit, RAJNA Péter

[Introduction - It is essential to identify the genetic factors of epilepsy in the every day clinical practice for several reasons. The proof of the genetically defined sub-clusters existing inside the epileptic disease group is significant in diagnoses and therapy. The risk of inheriting epilepsy could influence the patient’s family planning which has a great impact on their quality of life. The aim of the study - To analyse clinical data obtained from patients examined after their first provoked or unprovoked seizure and the observation of the recurrence of seizures. To compare the data obtained with the familial occurrence of epilepsy. Population and methods - Data was obtained from a questionnaire developed by the authors. The epileptic patients with positive familial data underwent to an analysis of their family tree. Results - Of 120 persons who were examined the prevalence of epilepsy in their family was 20.4%. This corresponds to the familial prevalence of generalised epilepsy according to the published clinical data. The recurrence of seizures was experienced by 32% of the patients with a family background affected by epilepsy. The risk of reoccurring seizures was the highest if the familial epilepsy manifested itself in the same generation (among brothers or sisters) and if we were able to register epileptiform activity on the interictal EEG. According to our clinical data the genetic set up can play a role also in the provoked first epileptic seizure. The incidence of familial epilepsy was found high (12.72%) in the presence of incidental epileptic seizures when the EEG was free of epileptiform alterations. Conclusion - 1. The genetic basis for the first epileptic seizure in the population of young adults approaches the data known in idiopathic generalised epilepsy irrespective of the fact whether it was related to the seizure provoking factors or not. 2. The risk of seizure reactivation was higher in non-provoked seizures then at the incidental epileptic symptoms. Seizure reactivation had to be taken into consideration when epileptiform patterns appeared on the patient's EEG and/or epileptic symptoms were experienced by the patient's brother or sister. The probability of reoccurring seizures was lower if the epileptic seizures manifested in parents or earlier generations.]

Clinical Neuroscience

[CLINICAL EXPERIENCE WITH LEVETIRACETAM FOR ADULTS WITH EPILEPSY]

BARCS Gábor, SZŰCS Anna

[Objective - A retrospective study to evaluate the efficacy of levetiracetam in the treatment of adult pharmacoresistant epilepsy. Method - Retrospective work up of our treatmentexperiences with 55 pharmacoresistant patients treated with levetiracetam (11 of them on monotherapy) for 6-39 months. Three treatment groups were analysed: idiopathic generalised epilepsy (9 patients); partial epilepsy (30 patients); malignant or malignated epileptic syndromes (16 patients). Result - Seven idiopathic generalised patients (77%) and 5 partial epilepsy patients (16%) became seizure free. One idiopathic generalised epileptic patient, 10 partial epilepsy patients (33%) significantly improved. Six patients (37%) from the group of malignant or malignated epileptic syndromes also significantly improved. Five of the improved idiopathic generalised epilepsy patients and 6 of the improved partial epilepsy patients received levetiracetam monotherapy. Altogether seven patients (12% of the whole population) relapsed after a 4-15 months improved period. Fifteen patients (27%) suffered side effects (mainly somnolence, headache, dizziness and irritability) improving after dose reduction of levetiracetam (generally below 2000 mg pro day). Conclusion - Levetiracetam is an effective, well tolerable, broad-spectrum drug as adjunctive treatment or monotherapy in adult patients unsuccessfully treated with other antiepileptic drugs.]

Clinical Neuroscience

[PRAMIPEXOLE THERAPY OF RESTLESS LEGS SYNDROME]

VIDA Zsuzsanna, SZAKÁCS ZOLTÁN

[The restless legs syndrome is a disorder belonging to the family of movement disorders during sleep, often remains unrecognized, although it is the second most common cause of chronic sleep deficiency and daytime sleepiness. In accordance with international guidelines, pharmacotherapy of this disorder should begin with a dopamine agonist. Owing to their efficacy and favorable safety profile, newly introduced, selective dopamine agonists have become extensively used for this purpose. This study evaluated the efficacy of one of the products in this group, pramipexole. Fifty-one patients suffering from idiopathic restless legs syndrome underwent monotherapy with pramipexole in daily doses of 0.25 to 1.0 mg. Therapeutic efficacy was evaluated using three tools, i.e. follow-up questionnaires, actigraphy, and Forced Immobilisation Test. An excellent therapeutic effect was seen in more than 80 per cent of the study population. As shown by findings of the follow-up questionnaires, pramipexole resulted in substantial improvements of both daytime and nighttime symptoms of RLS. Actigraphy monitoring demonstrated a statistically significant increase in the ratio of time spent without limb movement to the time spent in bed; furthermore, the result of the Forced Immobilisation Test also improved. It seems fair to conclude from the findings of this study that pramipexole monotherapy is an effective treatment in restless legs syndrome.]

Clinical Neuroscience

[UPDATES IN PRACTICAL NEUROLOGY - I. THE PRINCIPLES OF MODERN LEVODOPA THERAPY IN PARKINSON’S DISEASE]

KLIVÉNYI Péter, VÉCSEI László

[Despite the levodopa is used for the treatment of Parkinson’s disease for a long time, recently many questions raised about its clinical use. New issues emerged based on the clinical trials, on latest neuroimaging data and on better understanding the pathomechanism of motor complications. These observations have changed the routine clinical use of levodopa. In this review we summarize the evidences and practical implications of levodopa therapy.]

Clinical Neuroscience

[A CASE OF SCHIZENCEPHALY WITH POLYMICROGYRIA]

LEEL-ŐSSY Lóránt, SZŰCS Iván, ALMÁSI Kálmán

[A case of extensive bilateral frontotemporal schizencephaly is alleged - more extensively in the left hemisphere - which associated with polymicrogyria. The cortical anomaly was discovered only incidentally by MR examination in a 22 year-old man who suffered from headache due to a mild head trauma. Neurological examination proved to be negative. He had no complaints or symptoms a few weeks later. The developmental anomalies in corticalisation are shortly overviewed in this group together with the possible causing factors. It has been emphasized the importance of the precise intrauterine and/or postpartum differential diagnosis between schizencephaly, porencephaly and other failure in corticalisation.]

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Clinical Neuroscience

Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.

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.

Hypertension and nephrology

[About the care of patients with hyperuricaemia and gout]

[This consensus document is intended to provide guidance for the effective and efficient treatment of asymptomatic individuals with high uric acid levels and gout patients.]

Clinical Neuroscience

[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]

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.]