Clinical Neuroscience

[Editorial message]

TAJTI János, RAJNA Péter

JULY 30, 2012

Clinical Neuroscience - 2012;65(07-08)

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Clinical Neuroscience

[Diagnosis and therapy of mitochondrial diseases]

PÁL Endre

[Mitochondrial diseases are a significant part of neuromuscular diseases. Majority of them is multisystemic disorder. The diagnosis can be established in more and more cases. Beyond the routine neurological examination imaging methods (MRI and MR-spectroscopy) and electrophysiology (EMG, ENG, EEG, evoked potential tests) might be helpful in setting the diagnosis. Raised blood lactate level supports the diagnosis. Muscle biopsy demonstrates mitochondrial abnormalities in the majority of cases. The positivity of genetic tests is low, because the amount of mitochondrial DNA alterations is different in tissues. Therefore other tissue than blood (mainly muscle) is necessary for genetic tests. The other reason is that the respiratory chain is under double -mitochondrial and nuclear - genetic control, and testing the nuclear genes are available only in selected laboratories. The treatment is limited, mainly symptomatic.]

Clinical Neuroscience

[Recommendations for a brief international cognitive assessment for multiple sclerosis]

BENCSIK Krisztina

Clinical Neuroscience

[Results of intrathecal baclofen therapy on spasticity in patients with brain injury]

DÉNES Zoltán, KLAUBER András, BOTH Béla, ERÕSS Loránd

[Objectives - To evaluate the results of intrathecal baclofen (ITB) therapy on the spasticity in patients with brain injury. Method - Retrospective study in Brain Injury Rehabilitation Unit between January 2001 and December 2010. Results - During the last ten years, in our unit 13 patients were involved into ITB therapy on severe spasticity, after brain injury, while more than 100 Baclofen pumps were implantated in Hungary with Hungary with coordination of the Multidisciplinary Team. ITB therapy was indicated in severe spasticity developed after seven cases of traumatic brain injuries, five cases of strokes and one case of anoxic brain injury. The mean age of patients was 26 years (18- 52). At the time of pump implantation three patients were in vegetative state. The shortest period elapsed between the brain injury and pump implantation was three months and the longest period was nine years, mean 15 months. Baclofen pump had to be changed in six cases after six years, and was removed in three cases due to decreasing spasticity. Catheter revision was performed in two cases due to flow problem. We had no complication in association with ITB therapy. Conclusions - Intrathecal baclofen therapy seems to be an effective and safe treatment in patients with severe spasticity of cerebral origin. We suggest team (neurosurgeon and rehabilitation professionals) decision in a spasticity center before involving the patient into ITB therapy, and follow up in the rehabilitation unit. The severity of spasticity as a consequence of brain injury can change during years and it is necessery to follow it with dosage and dynamics of baclofen therapy. Baclofen pump removal is suggested if the ITB therapy is further not reasonable.]

Clinical Neuroscience

[Treatment of dystonia by deep brain stimulation: a summary of 40 cases]

DELI Gabriella, BALÁS István, KOMOLY Sámuel, DÓCZI Tamás, JANSZKY József, ILLÉS Zsolt, ASCHERMANN Zsuzsanna, TASNÁDI Emese, NAGY Ferenc, PFUND Zoltán, BÓNÉ Beáta, BOSNYÁK Edit, KULIFFAY Zsolt, SZIJJÁRTÓ Gábo

[Background - Bilateral pallidal deep brain stimulation (DBS) is an established treatment option for primary generalized and segmental dystonia. In the present study we evaluated the results of our dystonia patients treated by DBS. Methods - The surgical results of forty consecutive dystonia patients underwent DBS implantation were analyzed (age: 43.7±17.7 years; sex: 22 men; etiology: 24 primary and 16 secondary dystonia; topography: 24 generalized, 12 segmental and four hemidystonia; disease duration: 16.1±9.3 years). Severity of dystonia measured by Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and health-related quality of life measured by EQ-5D scale were obtained preoperatively and compared to the scores obtained at postoperative six months and subsequent yearly follow-ups. The average follow-up lasted 2.5 years (median, 0.5-8 years). In all cases the BFMDRS scores were re-evaluated by a rater blinded to the treatment. Treatment responsiveness was defined as an at least 25% improvement on the BFMDRS scores. Non-parametric Mann-Whitney, McNemar and Kruskal-Wallis tests were applied to test statistical significance. Results - Severity of dystonia improved from 31 to 10 points (median, 68% improvement, p<0.01) in the primary dystonia group, whereas in secondary dystonia these changes were statistically insignificant (improvement from 40 to 31.5 points, 21.2%, p>0.05). However, the health-related quality of life significantly improved in both groups (primary dystonia: 0.378 vs. 0.788 and secondary dystonia: 0.110 vs. 0.388, p<0.01). Significantly more patients in the primary dystonia group responded to DBS treatment than those in the secondary dystonia group (83.3% vs. 37.5%, p<0.01). Conclusion - Our results are in accordance with previously published international findings demonstrating that DBS is a highly effective and long-lasting treatment option for primary dystonia. DBS is considerably less efficient in secondary dystonia; however, it still has a high impact on the quality of life presumably due to its pain-relieving effect.]

Clinical Neuroscience

[Selective ultrastructural vulnerability in the cuprizone-induced experimental demyelination]

ÁCS Péter, KOMOLY Sámuel

[Background and purpose - It has been reported that multiple sclerosis has four different neuropathological subtypes, and two of them (type III and IV) are characterized by primary oligodendrocyte loss. However, the exact pathomechanism that lead to oligodendrocyte apoptosis in human demyelinating diseases is still elusive. The copper chelator cuprizone induces primary oligodendrocyte apoptosis and consequent demyelination in well defined areas of the mouse brain. Nevertheless, the precise subcellular events that result in oligodendrocyte cell death in the cuprizone model are still unknown. We aimed to study the ultrastructural alterations that might induce oligodendrocyte apoptosis in the cuprizone experimental demyelination model. Methods - C57BL/6 mice were given cuprizone for two, 21 and 35 days to induce demyelination to investigate early pathological events, and different stages of demyelination. In addition, mice were given cuprizone for 35 days and were allowed to recover for two or 14 days to study early and late remyelination. After the cuprizone treatment, mice were sacrificed and the corpus callosum, the superior cerebellar peduncle, the optic nerve and the sciatic nerve were studied by electron microscopy. Results - The ultrastructural analysis revealed that cuprizone induced oligodendrocyte apoptosis is accompanied by the formation of giant mitochondria in the affected cells in the corpus callosum and in the superior cerebellar peduncle. Apoptosis of the myelin producing cells was present through the whole cuprizone challenge. Severe demyelination occurred after three weeks of cuprizone administration associated with massive macrophage infiltration and astrocytosis of the demyelinated areas. Axons and neurons remained unaffected. Conclusion - The formation of giant mitochondria in myelin producing oligodendrocytes is the first pathological sign in the cuprizone experimental demyelination. Mitochondrium pathology in the cuprizone challenge might serve as a useful model to study the pathomechanism of multiple sclerosis subtypes (III and IV) characterized by primary oligodendrocyte degeneration.]

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[Health behavior of tattooed persons]

CZUPY Gábor, PONGÓ Gabriella, MIHÁLFFY Dávid, SUSÁNSZKY Éva

[INTRODUCTION - In post-modern societies tattoo has become decidedly fashionable as acceptance have replaced negative attitudes towards it. We assume that body decoration is a fashion-conscious behavior in the present Hungarian society and thus their lifestyle and risk behavior would not differ from the average of the Hungarian population; also, that there would be no gender differences in the motivations of having a tattoo nor in the messages communicated by tattoos. SAMPLE AND METHOD - Information on tattooed persons was collected on the Internet. The online questionnaire was completed by 508 persons. For processing the data we used the SPSS PC statistical software package. RESULTS - The majority of our sample was under 35 years of age. Respondents have an average of 5-7 tattoos. The most preferred body parts for tattooing are the arms and legs. The primary motivations for having a tattoo are self-expression and the need of commemorating emotions and events. There are significant differences between the characteristics of tattooing of men and women, however, these differences are not related to the social body. The eating habits of men and women are similar; the average number of meals per day is higher than three; the majority of the respondents eats healthy foods and chose their food consciously. They exercise 4-5 hours per week. The proportions of the overweight and the obese are far greater than the nationwide age averages. More than half of the respondents are active smokers. The estimated proportion of heavy drinkers is especially prominent among the tattooed women. SUMMARY - The motivational background and message communication support our assumptions that tattooing is fashion rather than a deviant behavior. The tattooed persons in our sample are health conscious regarding sports and exercise. Appearance has a great importance for them. On the other hand, above average use of legal addictive substances (alcohol) is characteristic among them.]