Clinical Neuroscience

[European Association of Young Neurologists and Trainees]

VARGA Edina Tímea, SZTRIHA László, RÓNA-VÖRÖS Krisztina

NOVEMBER 30, 2010

Clinical Neuroscience - 2010;63(11-12)

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Clinical Neuroscience

[The concept of epileptic networks. Part 2.]

HALÁSZ Péter

[In this paper we investigate evidences supporting the network concept of epilepsies from different approaches. Firstly the functions of cortical networks in which most of the epileptic networks are embedded, are treated. Then the tentative characteristics of an epileptic network are enumerated and the conversion mechanisms from physiological to epileptic networks are analyzed. Later the role of neuronal oscillations in epileptic networks and aspects of epilepsies provoked by sensory and cognitive tasks is studied. Lastly new fMRI data in mapping BOLD networks underlying spike and seizure discharges are used as arguments in favour of the epileptic network hypothesis. In a second part the well-known epilepsies related, or probably related to physiological networks are shown. Finally consequences of the network approach for creating a new unified epilepsy classification are discussed.]

Clinical Neuroscience

[Account on the scientific meeting of Környey Society in 2010. Part 2.]

[Account on the scientific meeting of Környey Society in 2010. Part 2. 2010;63(11-12)]

Clinical Neuroscience

[Special concerns in medical care of severely demented patients suffering from Alzheimer disease]

RAJNA Péter

[While the expected life-time increases dementias will show a pandemic nature. Author analyses the special medical and social concerns of severe states of Alzheimer disease. Having introduced the epidemiologic, diagnostic data and forms of palliatíve therapies he concentrates on the hitherto unsolved problems of patients and caregivers. He belongs to the experts identifying the Alzheimer diseases as a forced aging process. Accepted the theory of L. Hayflick he evaluates the aging process in frame of an evolution model. He states that for aged human beings having already performed the task of reproduction further conditions of living circumstances and even that of death are no more programmed. This hypothesis may have a very negative content at the first sense. But the author rather considers the uncertainty of aging a positive message. He establishes that the occurrence of degenerative processes connecting the aging must be limited by preventive interventions far more efficient than the present possibilities. Concerning these steps we are still on the level of recommendations for preserving our general health and data of relevant science are quite contradictory. Although criteria of “healthy", “qualified” or “assumable” aging are getting defined, we still do not know exactly how we should live optimally and what should we do for this in different epochs of our life. Somatic and psychological parameters are lacking which would be able to signalize the actual level of the aging process in a given person. We, physicians, also do not have exact opinion on the level of severity of dementias at which the patient already lost his her features of personality and individuality on an irreversible way. We have no idea on the phase when the ratio of joy and suffer falls below the value of 1.0 because we still have no tool for its measure. The author pointed the necessity of acceptance of a new preventive attitude and application of new methods in the medical care of the forced or pathological aging, instead of the present approaches based on the “wait and treat” attitude. Because of the intensive increase of the patient population the identification and care of patients can be efficient with a much more involvement of the basic medical network. Financial background can be improved by new theoretical bases of criteria of the palliative treatments. But the direct professional programs can be planned after solution of the existing moral and social dilemmas.]

Clinical Neuroscience

[Evaluation of general well-being in adults with therapy resistant partial epilepsy taking oxcarbazepine]

BALOGH Attila, SZENTESI Annamária, JÁNOSI István

[Aims - An open label, national, multicenter, observational study had been conducted to evaluate of psychological general well-being in adults with therapy resistant partial epilepsy taking oxcarbazepine in Hungary. The possibility was examined to differentiate the improvement of the psychic well-being caused by the better status of the seizure disorder or caused by the psychopharmacological effect of the oxcarbazepine (in the next: OXC). Methods - 528 patients were asked to fill the Hungarian validated version of Psychological General Well-Being Schedule (PGWB), English version created by Harold J. Dupuy in 1984, at the baseline visit and after 12- week long duration OXC therapy regim. Finally the data of 332 patients could be evaluated per protocol, biostatistical analysis was carried out. Results - Based on data of 332 patients (per protocol population) the OXC treatment significantly (p<0.0001) improved all items of PGWB in clinically well- controlled (responder) epileptic patients and improved affective markers of patients with uncontrolled epilepsy also. In case of patients suffering from uncontrolled epilepsy the increase of psychical wellbeing items score influenced by biological effects were higher than those influenced by external effects. Conclusions - It can be stated that the adopted PGWB test battery is reliable quantitative method to examine the psychological well-being of patients suffering from partial epilepsy. It was observed that OXC has significant psychopharmalogical effect besides the well-known anticonvulsive property among large Hungarian population of partial epileptic patiens. In case of OXC therapy the positive psychic effect of the successful anticonvulsive treatment and the stabilization property on the affectivity of epileptic patients significantly separates. The OXC has no indication for the treatment of psychiatric conditions.]

Clinical Neuroscience

[Importance of hydrocephalus following severe brain injury during postacute rehabilitation]

DÉNES Zoltán, LANTOS Ágnes, SZÉL István, THOMKA Magdolna, VASS Mátyás, BARSI Péter

[Objectives - We report our experiences with hydrocephalus in early rehabilitation over a seven-year period. Method - Retrospective study in Brain Injury Rehabilitation Unit of the National Institute for Medical Rehabilitation, between 1 January 2002 and 31 December 2008. Results - At our institute in the last seven years, we treated 83 patients with secondary and six patients with primary hydrocephalus. The majority of hydrocephalus was of post traumatic origin (52) and remaining 23 following stroke (SAH, AVM, ICH) or brain operation (tumour - seven, and one cranioplasty), and all these patients had undergone ventricular shunt implantation. Mean age of patients was 36 (14-80) years. Hydrocephalus was diagnosed in our rehabilitation unit in 20 of 83 cases and the other patients were shunted before transfer to our unit. The median time point of shunting was 70 (range: 20-270) days after trauma, brain surgery or stroke. Post-operative complications were seen in 12 of 89 patients: six infections and six shunt failure and revision was necessary in 14%. In PTH cases, the post-operative improvement was seen in 40 of 52 patients being shunted and corresponded to FIM scores. At the other 31 cases, with non-traumatic origin, only two patients remain unchanged. Conclusions - Hydrocephalus is considered to be a frequent and important complication after severe brain damage. The incidence of hydrocephalus treated with shunt implantation in our neuro-rehabilitation unit was 4.4%. The postoperative improvement was 77%. Posttraumatic hydrocephalus concerns 5.2% of patients with severe TBI during last seven years in our institution. Diagnosis of posttraumatic hydrocephalus was established in 24%, and complication after shunt implantation (14%) was also recognized in the post-acute rehabilitation unit. It is strongly recommended for the team working at such type of units to obtain clinical practice. Teamwork, good cooperation between acute and postacutecare is necessary for successful rehabilitation of these patients.]

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Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

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

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

[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

Evaluation of the effectiveness of transforaminal epidural steroid injection in far lateral lumbar disc herniations

EVRAN Sevket, KATAR Salim

Far lateral lumbar disc herniations (FLDH) consist approximately 0.7-12% of all lumbar disc herniations. Compared to the more common central and paramedian lumbar disc herniations, they cause more severe and persistent radicular pain due to direct compression of the nerve root and dorsal root ganglion. In patients who do not respond to conservative treatments such as medical treatment and physical therapy, and have not developed neurological deficits, it is difficult to decide on surgical treatment because of the nerve root damage and spinal instability risk due to disruption of facet joint integrity. In this study, we aimed to evaluate the effect of transforaminal epidural steroid injection (TFESI) on the improvement of both pain control and functional capacity in patients with FLDH. A total of 37 patients who had radicular pain caused by far lateral disc herniation which is visible in their lumbar magnetic resonance imaging (MRI) scan, had no neurological deficit and did not respond to conservative treatment, were included the study. TFESI was applied to patients by preganglionic approach. Pre-treatment Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI) scores of the patients were compared with the 3rd week, 3rd month and 6th month scores after the procedure. The mean initial VAS score was 8.63 ± 0.55, while it was 3.84 ± 1.66, 5.09 ± 0.85, 4.56 ± 1.66 at the 3rd week, 3rd month and 6th month controls, respectively. This decrease in the VAS score was found statistically significant (p = 0.001). ODI score with baseline mean value of 52.38 ± 6.84 was found to be 18.56 ± 4.95 at the 3rd week, 37.41 ± 14.1 at the 3rd month and 34.88 ± 14.33 at the 6th month. This downtrend of pa­tient’s ODI scores was found statistically significant (p = 0.001). This study has demonstrated that TFESI is an effective method for gaining increased functional capacity and pain control in the treatment of patients who are not suitable for surgical treatment with radicular complaints due to far lateral lumbar disc hernia.