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Bilateral paramedian thalamic stroke is characterized by hypersomnia, vertical gaze palsy, amnestic alteration, and apathic state. Combined lesion of the paramedian thalamus and mesencephalon bilaterally is extremely rare. Little is known about the breathing disturbances of the particular region due to the lesion. The following describes the specific case of a woman, age 62, with bilateral paramedian thalamic and mesencephalic stroke. Initially, the patient’s complaints exhibited altered vigilance and vertical gaze palsy. Notably, following the acute phase, fluctuating hypersomnia was detected. The MRI (brain) revealed an ischemic lesion in the medial part of the mesencephalon and paramedian thalamus, bilaterally. The aim of the present study is to elucidate the involvement and characteristics of sleep-related breathing abnormalities in the clinical manifestation of the combined paramedian thalamic and mesencephalic stroke. Polysomnographic recordings were accomplished seven times with 1-week interval between the consecutive recordings, toward investigating the early changes of sleep and sleep-related breathing abnormalities. Sleep structure examination featured a decrease in N3 and REM ratio and an increase in N1 and N2 ratio with minimal improvement during the recovery period. In contrast, significant changes were found in the breathing pattern: the initial central apnea dominance was followed by obstructive apneas with a gradual decrease of the total pathological respiratory events. In addition to the structural abnormality of the sleep regulating network, sleep-disordered breathing is another possible cause of hypersomnia in patients afflicted with the present localization of the lesion.

Background: Sleep problems are among the most common non-motor symptoms of Parkinson’s disease (PD). The PD Sleep Scale 2nd version (PDSS-2) improved the original PDSS by adding more items on different aspects of sleep problems, making it a more robust tool to evaluate the severity of sleep disturbances. However, previous studies on deep brain stimulation (DBS) have not used the PDSS-2. Objective: To determine if the PDSS-2 could detect improvement reliably in sleep problems after bilateral subthalamic nucleus DBS for PD. Methods: In this prospective study, 25 consecutive patients undergoing DBS implantation were enrolled. Patients were examined twice: 1 week prior to the DBS implantation (baseline) and 12 months postoperatively. Severity of PD symptoms were assessed by the Movement Disorders Society Unified PD Rating Scale (MDS-UPDRS) and the Non-Motor Symptoms Scale (NMSS). Presence and severity of sleep disturbances were specifically measured by PDSS-2. Results: Total score of MDS-UPDRS improved from 81 (median, interquartile-range: 63–103) to 55 points (median, IQR: 46–75, p < 0.001). Health-related quality of life, measured by PDQ-39, also improved from 29 (IQR: 18–40) to 15 (IQR: 9–28) points (p = 0.002). Most domains of NMSS also improved. At baseline 13 patients reported sleep problems, but 1 year after DBS implantation only 3 did (p = 0.012). Although only 6 out of 15 items showed a significant decrease after DBS implantation, the total score of PDSS-2 decreased from 24 (IQR: 17–32) to 10 (IQR: 7–18) points (P < 0.001). Conclusions: Based on our results, PDSS-2 can detect improvements in sleep quality reliably after DBS implantation.

In the present review the recent developments in the definitions of neurocognitive disorders associated with Parkinson’s disease are summarized including the possibilities for screening and treating. For a long time, the recognition of neurocognitive disorders associated in patients with Parkinson’s disease was unsatisfactory due to the heterogeneity of definitions. The recently developed Diagnostic and Statistical Manual of Mental Disorders, 5th edition (DSM-5) introduced the definitions of mild and major neurocognitive disorders instead of mild cognitive impairment and dementia. The new DSM-5 definitions are clinically well applicable; therefore, the validation of the most frequent screening tests (Mini-Mental State Examination; Addenbrooke’s Cognitive Examination; Montreal Cognitive Assessment; Mattis Dementia Rating Scale) is warranted. Based on a Hungarian sample of 295 patients with Parkinson’s disease, the cut-off scores having the best discriminative values are highly dependent on education years (Addenbrooke’s Cognitive Examination: 0–8 years of education: 82.5 points, 9–12 years of education: 83.5 points, and ≥13 years of education: 84.5 points; Mini-Mental State Examination: 26.5–27.5–28.5 points, Montreal Cognitive Assessment: 23.5–24.5–24.5 points, Mattis Dementia Rating Scale: 138.5–139.5–139.5 points, respectively). Orv. Hetil., 2015, 156(23), 915–926.

Wide variety of the movements (from the physiologic body position changes to different pathologic events) can be seen during the sleep period. The most important types of these movements are the sleep related events (from the parasomnias to the restless leg related movements), the movement disorders and the epilepsy related events. To differentiate between these events is required special skill, which is based on appropriate characterisation of the events (for example timing, repetition, pattern), the polysomnographic and video-EEG examination and validated questionnaires (FLEP scale). The appropriate differential diagnostics and therapy must be based on the knowledge of the relationship of the sleep architecture and movement events. This review would like to provide guideline for the understanding and recognizing the nature of the sleep related movements.

BACKGROUND The recently published "EarlyStim" study demonstrated that deep brain stimulation (DBS) for the treatment of Parkinson's disease (PD) with early fluctuations is superior to the optimal pharmacological treatment in improving the quality of life and motor symptoms, and preserving sociocultural position. Our retrospective investigation aimed to evaluate if DBS therapy was able to preserve the working capabilities of our patients. METHODS We reviewed the data of 39 young (< 60 years-old) PD patients who underwent subthalamic DBS implantation at University of Pecs and had at least two years follow-up. Patients were categorized into two groups based on their working capabilities: Patients with active job ("Job+" group, n = 15) and retired patients (without active job, "Job-" group, n = 24). Severity of motor symptoms (UPDRS part 3), quality of life (EQ-5D) and presence of active job were evaluated one and two years after the operation. RESULTS As far as the severity of motor symptoms were concerned, similar (approximately 50%) improvement was achieved in both groups. However, the postoperative quality of life was significantly better in the Job+ group. Majority (12/15, 80%) of Job+ group members were able to preserve their job two years after the operation. However, only a minimal portion (1/24, 4.2%) of the Job- group members was able to return to the world of active employees (p < 0.01, McNemar test). CONCLUSION Although our retrospective study has several limitations, our results fit well with the conclusions of "EarlyStim" study. Both of them suggest that with optimal timing of DBS implantation we may preserve the working capabilities of our patients.

BACKGROUND AND PURPOSE There is a lack of data on the impact on health related quality of life of peripheral neuropathic pain in Hungary. The main aims of the study were to assess the health related quality of life of non-diabetic PeNP patients identified in general practices through screening, and to assess the relationship between condition specific pain scores and health state utilities. METHODS Non-diabetic patients aged 30 years were recruited in 10 general practices in Hungary. At first, patients filled in the PainDETECT Questionnaire (PD-Q) and those who have achieved > or =13 PD-Q score (unclear or possible neuropathic pain) were further assessed by the DN4 questionnaire. Patients with PD-Q score >18 or DN4 score 4 were considered to have PeNP and they completed the EQ-5D health questionnaire. RESULTS Among the 111 patients identified as non-diabetic PeNP patients the mean age was 62 (SD=14) years, 69% were women. Average EQ-5D score was 44% lower than the gender and age matched Hungarian norm (0.42 vs. 0.75, p

Background: Patients with minor acute ischemic stroke or transient ischemic attack are at high risk for subsequent stroke, and more potent antiplatelet therapy in the acute setting is needed. However, the potential benefit of more intense antiplatelet therapy must be assessed in relation to the risk for major bleeding. The SOCRATES trial (Acute Stroke or Transient Ischemic Attack Treated With Aspirin or Ticagrelor and Patient Outcomes) was the first trial with ticagrelor in patients with acute ischemic stroke or transient ischemic attack in which the efficacy and safety of ticagrelor were compared with those of aspirin. The main safety objective was assessment of PLATO (Platelet Inhibition and Patient Outcomes)–defined major bleeds on treatment, with special focus on intracranial hemorrhage (ICrH). Methods: An independent adjudication committee blinded to study treatment classified bleeds according to the PLATO, TIMI (Thrombolysis in Myocardial Infarction), and GUSTO (Global Use of Strategies to Open Occluded Coronary Arteries) definitions. The definitions of ICrH and major bleeding excluded cerebral microbleeds and asymptomatic hemorrhagic transformations of cerebral infarctions so that the definitions better discriminated important events in the acute stroke population. Results: A total of 13 130 of 13 199 randomized patients received at least 1 dose of study drug and were included in the safety analysis set. PLATO major bleeds occurred in 31 patients (0.5%) on ticagrelor and 38 patients (0.6%) on aspirin (hazard ratio, 0.83; 95% confidence interval, 0.52–1.34). The most common locations of major bleeds were intracranial and gastrointestinal. ICrH was reported in 12 patients (0.2%) on ticagrelor and 18 patients (0.3%) on aspirin. Thirteen of all 30 ICrHs (4 on ticagrelor and 9 on aspirin) were hemorrhagic strokes, and 4 (2 in each group) were symptomatic hemorrhagic transformations of brain infarctions. The ICrHs were spontaneous in 6 and 13, traumatic in 3 and 3, and procedural in 3 and 2 patients on ticagrelor and aspirin, respectively. In total, 9 fatal bleeds occurred on ticagrelor and 4 on aspirin. The composite of ICrH or fatal bleeding included 15 patients on ticagrelor and 18 on aspirin. Independently of bleeding classification, PLATO, TIMI, or GUSTO, the relative difference between treatments for major/severe bleeds was similar. Nonmajor bleeds were more common on ticagrelor. Conclusions: Antiplatelet therapy with ticagrelor in patients with acute ischemic stroke or transient ischemic attack showed a bleeding profile similar to that of aspirin for major bleeds. There were few ICrHs. Clinical Trial Registration: URL: http://www.clinicaltrials.gov . Unique identifier: NCT01994720.

Abnormally high deposition of iron can contribute to neurodegenerative disorders with cognitive impairment. Since previous studies investigating cognition-brain iron accumulation relationships focused on elderly people, our aim was to explore the association between iron concentration in subcortical nuclei and two types of memory performances in a healthy young population. Gender difference was found only in the globus pallidus. Our results showed that iron load characterized by R2* value on the MRI in the caudate and putamen was related to visual memory, while verbal memory was unrelated to iron concentration.

Hippocampal volume loss has been described in several pathological conditions including sleep disorders. Whether differences in normal sleep are associated with differences in hippocampal volume is unknown. Here we designed a study to assess volume of the hippocampus with regard to bedtime, wake up time and sleep duration in 90 healthy university students. To assess hippocampal volumes we applied semi-automatic user-independent magnetic resonance volumetry. We found a significant association between delayed bedtime and smaller hippocampal volumes, a non-significant tendency for smaller hippocampal volumes in the late wake up time group and significantly smaller hippocampal volumes for both short and long sleepers. These results suggest the importance of adequate sleep timing and especially bedtime in determining hippocampal volume.

Asymmetry is one of the unique and mysterious features of Parkinson's disease (PD). Motor symptoms develop unilaterally either on the left (LPD) or the right side (RPD). Incongruent data are available whether the side of onset has an impact on cognition in PD. The objective of this study is to compare the visuospatial performance of RPD and LPD patients. Seventy-one non-demented, non-depressive and right-handed patients were categorized into RBD (n = 36) and LPD (n = 35) groups. Rey-Osterrieth Complex Figure Test (ROCF) was evaluated by both the Taylor's and Loring's scoring systems. Subsequently, we also performed subgroup analyses on patients having short disease duration (≤5 years, 15 RBD and 15 LPD patients). The standard analysis of ROCF (Taylor's system) did not reveal any differences; however, the utilization of the Loring's system demonstrated that LPD patients had significantly worse visuospatial performance than the RPD subjects (3.0 vs. 2.0 points, median, p = 0.002). Correlation between the number of spatial errors and the degree of asymmetry was significant (r = -0.437, p = 0.001). However, this difference could not be observed in PD patients with short disease duration. LPD patients had worse visuospatial performance than the RPD subjects and the number of errors tightly correlated with the degree of asymmetry and long disease duration.

Cuprizone is a copper-chelating mitochondrial toxin that causes oligodendrocyte apoptosis and demyelination preferentially in the corpus callosum (CC) and the superior cerebellar peduncles, but not in the spinal cord (SC) of C57BL/6 mice. Here we aimed to determine the activities of copper-containing enzymes in correlation with the distribution of demyelination during exposure to cuprizone. The study revealed mitochondrial complex IV and superoxide dismutase activity alterations in both the pathology-affected CC and the non-affected SC. This observation raises the possibility that regionally different subcellular molecular interactions lead to the selective oligodendrocyte loss induced by the nonselective mitochondrial toxin, cuprizone.

Schizophrenic patients have Theory of Mind (ToM) deficits even during remission, but it is yet unknown whether this could be influenced. We examined the neural correlates of irony understanding in schizophrenic patients, as an indicator of ToM capacity, and evaluated how linguistic help inserted into the context phase could affect irony comprehension. Schizophrenic patients in remission and healthy controls were subjected to event-related functional MRI scanning while performing irony, 'irony with linguistic help', and control tasks. Patients understood irony significantly worse than healthy controls. The patients showed stronger brain activity in the parietal and frontal areas in the early phase of irony task, however the healthy controls exhibited higher activation in frontal, temporal and parietal regions in the latter phase of the irony task. Interestingly the linguistic help not only improved the patients' ToM performance, but it also evoked similar activation pattern to healthy controls.

Introduction: Changes in human behaviour and lifestyle over the last century have resulted in a dramatic increase in the incidence of diabetes worldwide. Neuropathy is a common and costly complication of both type 1 and type 2 diabetes. The prevalence of neuropathy is estimated to be about 8% in newly diagnosed patients and greater than 50% in patients with long-standing disease. There are two main types of diabetic neuropathies, named as sensorimotor and autonomic neuropathies. Sensorimotor neuropathy is marked by pain, paraesthesia and sensory loss, and autonomic neuropathy may contribute to myocardial infarction, malignant arrhythmia and sudden death. Methods: In this article we reviewed the pathogenesis, clinical manifestations diagnosis and treatment of diabetic neuropathies. Conclusion: Sensorimotor and autonomic neuropathies (cardiovascular, gastrointestinal and genitourinary autonomic neuropathies) are common in diabetic patients. Apart from strict glycaemic control, no further therapeutic approach exists in the prevention of this phenomenon. Intensive diabetes therapy, intensive multifactorial cardiovascular risk reduction and lifestyle intervention are recommended in patients with cardiovascular autonomic neuropathy. Gastroparesis is the most debilitating complication of gastrointestinal autonomic neuropathy and genitourinary autonomic neuropathy can cause sexual dysfunction and neurogenic bladder; these conditions are hard to manage. The symptomatic treatment of sensory symptoms includes tricyclic antidepressants, serotonin and norepinephrine reuptake inhibitors, gabapentin, pregabalin and opioids. Other treatment strategies are not so effective.

Background: Minimal clinically important difference (MCID) is the smallest change in an outcome, which a patient identifies as meaningful. Although the 2 most frequently applied Parkinson's disease (PD) “quality of life” questionnaires (the PDQ-39 and PDQ-8) provide encouragingly similar results, their MCID thresholds appear to be vastly different. Our aim was to calculate the MCID estimates for both PDQ-39 and PDQ-8 Summary Indices (PDQ-39-SI and PDQ-8-SI) by the utilization of both anchor- and distribution-based techniques. Methods: Nine hundred eighty-five paired investigations of 365 patients were included. Three different techniques were used simultaneously to calculate the MCID values. Results: First, we replicated the previously published results demonstrating how both PDQ-39-SI and PDQ-8-SI provide similar values and respond in a similar way to changes. Subsequently, we calculated the MCID thresholds. The most optimal estimates for MCID thresholds for PDQ-39-SI were -4.72 and +4.22 for detecting minimal clinically important improvement and worsening. For PDQ-8-SI, these estimates were -5.94 and +4.91 points for detecting minimal clinically important improvement and worsening respectively. Conclusions: Our study is the first one that directly compared the MCID estimates for both PDQ-39-SI and PDQ-8-SI on a large pool of patients including all disease severity stages. These MICD estimates varied across PD severity.

Background: Dyskinesia is among the most troublesome symptoms of advanced Parkinson's disease (PD). The recently developed Unified Dyskinesia Rating Scale (UDysRS) can simultaneously measure several subjective and objective aspects of dyskinesia, irrespective of the other motor symptoms of PD. Despite the advantages of deep brain stimulation (DBS), previous studies on DBS have not used the UDysRS yet. Methods: In this prospective study, 71 consecutive patients undergoing DBS implantation were enrolled. Patients were examined twice: 1 week prior to the DBS implantation (baseline) and 12 months postoperatively. The severity of PD-related symptoms was assessed by the Movement Disorders Society Unified PD Rating Scale (MDS-UPDRS). The presence and severity of dyskinesia were specifically measured by the UDysRS and patient diaries. Results: At baseline, all 71 patients had dyskinesia, but 1 year after DBS implantation, 25 patients were dyskinesia-free, and an additional 19 had only mild dyskinesia. The total score on the UDysRS decreased from 38.0 ± 17.8 to 10.8 ± 13.0 (p < 0.001). Besides this, all parts of the UDysRS showed significant improvement after STN DBS treatment, and the magnitude of these changes had a large effect size. The total score of MDS-UPDRS improved from 76.5 ± 24.3 to 60.4 ± 21.4 points (p < 0.001). Conclusions: Based on our results, UDysRS can reliably detect improvements in dyskinesia after DBS implantation.

Background Levodopa/carbidopa intestinal gel therapy (LCIG) can efficiently improve several motor and non-motor symptoms of advanced Parkinson's disease (PD). The recently developed Movement Disorder Society-sponsored Unified Parkinson's Disease Rating Scale (MDS-UPDRS) improved the original UPDRS making it a more robust tool to evaluate therapeutic changes. However, previous studies have not used the MDS-UPDRS and the Unified Dyskinesia Rating Scale (UDysRS) to assess the efficacy of LCIG. Objectives Our aim was to determine if the MDS-UPDRS and UDysRS could detect improvement in the experiences of daily living following 1-year LCIG treatment. Methods In this prospective, multicenter, open-label study, 34 consecutive patients undergoing LCIG treatment were enrolled. Patients were examined twice: prior to LCIG initiation and 12 months later. Impact of PD-related symptoms and dyskinesia was assessed by the MDS-UPDRS and UDysRS. Results Non-motor Experiences of Daily Living part of MDS-UPDRS improved from 20 (median, interquartile-range, IQR:14–23) to 16 points (median, IQR:12–20, p = 0.044) and the Motor Experiences of Daily Living ameliorated from 24 (median, IQR:20–29) to 18 points (median, IQR:13–25, p = 0.025). Health-related quality of life, measured by PDQ-39, also improved from 35.4 (median, IQR:26.9–50.3) to 27.0 (median, IQR:21.3–31.4) points (p = 0.003). The total score of UDysRS decreased from 47 (median, IQR:36–54) to 34 (median, IQR:21–45) points (p = 0.003). Conclusions As far as the authors are aware of, our paper is the first to evaluate the impact of LCIG on dyskinesia by the means of UDysRS. Changes in MDS-UPDRS and UDysRS confirm that LCIG treatment can efficiently improve experiences of daily living in advanced PD.

The minimal clinically important difference is the smallest change of scores clinically meaningful to patients.We aimed to calculate these threshold values in association with the International Parkinson and Movement Disorder Society UPDRS (MDS-UPDRS) Parts I and II and to evaluate the feasibility of the composite score of Part I and II (MDS-UPDRS I+II) as an outcome.Nine hundred eighty-five paired investigations of 365 patients were reviewed, implementing three different techniques simultaneously.Based on the ordinal regression modeling, the MDS-UPDRS I+II score is an applicable outcome measure. Any improvement greater than 2.64 points or any worsening more than 2.45 points on MDS-UPDRS Part I represent a minimal, yet clinically meaningful change. In reference to Part II, the smallest changes considered clinically relevant were 3.05 and 2.51 points for improvement and deterioration, respectively. The thresholds for MDS-UPDRS I+II were 5.73 points for improvement and 4.70 points for worsening.Our minimal clinically important difference thresholds can be utilized in clinical practice in judging clinical relevance. © 2016 International Parkinson and Movement Disorder Society.

Multiple sclerosis is a chronic inflammatory, demyelinating degenerative disease of the central nervous system. Current treatments target pathological immune responses to counteract the inflammatory processes. However, these drugs do not restrain the long-term progression of clinical disability. For this reason, new therapeutic approaches and identification of novel target molecules are needed to prevent demyelination or promote repair mechanisms. Transient Receptor Potential Ankyrin 1 (TRPA1) is a nonselective cation channel with relatively high Ca

OBJECTIVE The aim of this study was to examine chronic brain white matter hyperintensities in migraine and to gain data on the characteristics of the lesions. BACKGROUND Migraine associates with a higher incidence of magnetic resonance imaging (MRI)-visible white matter signal abnormalities. Several attack-related pathomechanisms have been proposed in the lesion development, including the effect of repeated intracerebral hemodynamic changes. METHODS Supratentorial white matter hyperintensities of 17 migraine patients were investigated interictally with quantitative MRI, including quantitative single voxel spectroscopy, diffusion, and perfusion MRI at 3.0-Tesla. The findings were compared with data measured in the contralateral, normal-appearing white matter of migraineurs and in the white matter of 17 healthy subjects. RESULTS Significantly higher apparent diffusion coefficient values, prolonged T2 relaxation times, and decreased N-acetyl-aspartate and creatine/phosphocreatine concentrations were found in the white matter hyperintensities. The cerebral blood flow and blood volume values were mildly decreased inside the hyperintensities. Differences were not present between the migraine patients' normal-appearing white matter and the white matter of healthy subjects. CONCLUSIONS The MRI measurements denote tissue damage with axonal loss, low glial cell density, and an enlarged extracellular space with an increased extracellular water fraction. These radiological features might be the consequences of microvascular ischemic changes during migraine attacks.

Background: The placebo-controlled phase of the PreCISe study showed that glatiramer acetate delayed onset of clinically definite multiple sclerosis (CDMS) in patients with clinically isolated syndrome and brain lesions on MRI. Objective: To compare the effects of early versus delayed glatiramer acetate treatment in the open-label phase of PreCISe. Methods: Patients with a clinically isolated syndrome suggestive of MS with unifocal manifestation and ≥2 T2-weighted brain lesions were randomized to receive glatiramer acetate 20 mg/d (early-treatment, n=198) or placebo (delayed-treatment, n=211) for 36 months or until conversion to CDMS, followed by open-label glatiramer acetate treatment for two years. Results: Early glatiramer acetate treatment reduced CDMS conversion risk by 41% (hazard ratio 0.59, 95% confidence interval 0.44–0.80; p=0.0005) versus delayed-treatment, and was associated with a 972-day delay (185%) in conversion to CDMS, less brain atrophy (−28%, p=0.0209), fewer new T2 lesions/year (−42%, Conclusions: Effects of early glatiramer acetate treatment on the rate of conversion to CDMS and on MRI measures of disease activity and lesion burden support initiating glatiramer acetate treatment soon after the first clinical symptoms suggestive of MS and continuing treatment to sustain benefits.

Introduction: Among the non-motor features of Parkinson’s disease (PD), cognitive impairment is one of the most troublesome problems. Highly sensitive and specific screening instruments for detecting dementia in PD (PDD) are required in the clinical practice. Methods: In our study we evaluated the sensitivity and specificity of different neuropsychological tests (Addenbrooke’s Cognitive Examination, ACE; Frontal Assessment Battery, FAB and Mattis Dementia Rating Scale, MDRS) in 73 Parkinson’s disease patients without depression. By receiver operating characteristic curve analysis, these screening instruments were tested against the recently established clinical diagnostic criteria of PDD. Results: Best cut-off score for ACE to identify PDD was 80 points (sensitivity ¼ 74.0%, specificity ¼ 78.1%). For FAB the most optimal cut-off value was 12 points (sensitivity ¼ 66.3%, specificity ¼ 72.2%); whereas for MDRS it was 125 points (sensitivity ¼ 89.8%, specificity ¼ 98.3%). Among the examined test batteries, MDRS had the best clinicometric profile for detecting PDD. Conclusion: Although the types of applied screening instruments might differ from movement disorder clinic to clinic within a country, determination of the most specific and sensitive test for the given population remains to be an important task. Our results demonstrated that the specificity and sensitivity of MDRS was better than those of ACE, FAB and MMSE in Hungary. However, further studies with larger sample size and more uniform criteria for participation are required to determine the most suitable screening instrument for cognitive impairment.

To perform a pilot study assessing possible efficacy, safety, and optimal dosage of pramipexole in essential tremor.Twenty-nine patients with essential tremor were enrolled into this 16-week-long study. After recording baseline condition, 2 different dosages of immediate-release formulation of pramipexole were evaluated (1.05 mg/d and 2.1 mg/d in 3 identical dosages). Subsequently, immediate- and extended-release formulations were compared. The Fahn-Tolosa-Marin Tremor Rating Scale, Activities of Daily Living, the EuroQol instrument for detecting health-related outcome, and Clinical Global Impression of Improvement Scale were obtained. After completing the study, a rater blinded to the treatment reassessed the tremor rating scales based on video recordings.Twenty-four patients (82.6%) completed the study. Causes for discontinuation were adverse effects of pramipexole: intolerable nausea (n = 3), daytime sleepiness (n = 1), and anxiety (n = 1). Twenty-one patients had a score of less than 3 on the Clinical Global Impression of Improvement Scale (treatment responders, 72.4%). All the major outcome values demonstrated significant improvement. The severity of tremor was reduced by 52.0% (43.7 vs 20.8 points, Fahn-Tolosa-Marin Tremor Rating Scale), and the EuroQol instrument for detecting health-related outcome score improved from 0.69 to 0.91 (P0.01). The dose of 2.1 mg was more effective than that of 1.05 mg; however, both the immediate- and extended-release formulations were equally efficacious. After completion of the study, 15 patients (51.7% of the enrolled patients) wanted to remain on pramipexole treatment.This pilot study suggests that pramipexole may be effective in the treatment of essential tremor. However, further controlled studies are required.

Absztrakt Bevezetés: Az elhízásban számos tényező játszik szerepet, ezek között az agy megváltozott működésének is jelentőséget tulajdonítanak. A kutatások egyik fontos területe a táplálkozás és anyagcsere központi szabályozásának vizsgálata, mivel a kapcsolódó betegségek – elhízás, kóros soványság, diabetes mellitus, metabolikus szindróma – a modern társadalmak egészségügyi ellátására egyre növekvő terhet rónak. Az e megbetegedések hátterében álló patofiziológiai elváltozások, de ma még az egészséges szervezet centrális regulációs mechanizmusai sem ismertek kellő mértékben. E működések jobb megértését szolgálják a manapság egyre szélesebb körben alkalmazott funkcionális MR- (fMRI) vizsgálatok. Jelen kísérleteinkben kövér betegek és egészséges kontrollszemélyek ízingerlés kiváltotta agyi fMRI-aktivitásváltozásait tanulmányoztuk. Módszerek: A vizsgálatban 10 elhízott és 10 egészséges, hasonló kor- és nemi megoszlású önkéntes alany vett részt. Az ízingerek a következők voltak: 0,1 M nádcukor (kellemes), 0,5 mM koncentrációjú kinin-hidroklorid (kellemetlen) és vaníliaízesítésű folyékony tápszer (Nutridrink; magas kalóriatartalmú komplex íz). Az ízoldatokat PVC-csövön, 5–5 ml térfogatban juttattuk az alanyok szájüregébe. Öblítésként és semleges ingerként desztillált víz szolgált. A képalkotás során, 3T térerőn, standard Echo Planar Imaging (EPI) szekvenciát alkalmaztunk. Az adatok utófeldolgozása az FMRIB Software Library (FSL) programcsomaggal történt. Eredmények: Az ízingerlés kiváltotta agyi aktiváció jellemzően különbözött a két csoportban. A kövér betegek több kérgi és kéreg alatti struktúrában mutatkozó nagy jelintenzitásával összehasonlítva a kontrollokban kevesebb agyterület mérsékeltebb aktivációját tapasztaltuk. Következtetés: Jelen fMRI-vizsgálataink a kellemes és kellemetlen ízingerek hatására kövérek és egészségesek számos agyterületén létrejövő eltérő aktivációra világítottak rá. Eredményeink alapján a módszer segíthet az elhízás központi idegrendszeri tényezőinek pontosabb megismerésében, és hosszú távon hozzájárulhat hatékonyabb testsúlycsökkentő terápiák kifejlesztéséhez is.

Microalbuminuria, assessed by measuring immunoreactive albumin, is common in patients with cerebrovascular disease and is associated with increased risk of stroke. Total urinary albumin (t-uAlb) comprises both immunoreactive albumin (ir-uAlb) and nonimmunoreactive albumin (nir-uAlb). We hypothesized that t-uAlb is a more sensitive indicator of microalbuminuria than ir-uAlb, and that measurement of t-uAlb will increase the prevalence of microalbuminuria in ischemic stroke patients compared with measurement of ir-uAlb and will show a stronger correlation with the severity of stroke and oxidative stress. In urine samples from 98 patients with ischemic stroke, the albumin-to-creatinine ratios t-uAlb/uCreat and ir-uAlb/uCreat were measured by high-performance liquid chromatography (HPLC) and immunoturbidimetry (IT), and the nir-uAlb/uCreat ratio was calculated. Urinary ortho-tyrosine (o-Tyr/uCreat), an indicator of oxidative stress, was measured by HPLC. The severity of stroke was scored based on the National Institutes of Health Stroke Scale (NIHSS). The prevalence of microalbuminuria detected by HPLC was significantly higher than that detetcted by IT (66.3 vs 36.7%). Although all forms of albumin showed significant correlation with stroke severity (t-uAlb: r = 0.24, P.05 ir-uAlb: r = 0.25, P.05 nir-uAlb: r = 0.29, P.05), only nir-uAlb was found to be an independent predictor of stroke severity (B = 0.20, β = 0.35, P.05). In addition, t-uAlb/uCreat and nir-uAlb/uCreat had a significant correlation with o-Tyr/uCreat (r = 0.336, P.05 and r = 0.358, P.05 respectively), whereas ir-uAlb/uCreat did not (r = 0.22, P.05). Our data suggest that in acute ischemic stroke patients, t-uAlb is a more sensitive indicator of microalbuminuria than the presently used ir-uAlb. Future studies should aim to elucidate the underlying mechanisms for the relationship among urinary albumins and cerebrovascular diseases and the role of urinary albumins in risk stratification for stroke.

There are contradictory results on lateralisation and localisation of rhythm processing. Our aim was to test whether there is a hemispheric dissociation of metric and non-metric rhythm processing. We created a non-metric rhythm stimulus without a sense of metre and we measured brain activities during passive rhythm perception. A total of 11 healthy, right-handed, native female Hungarian speakers aged 21.3 ± 1.1 were investigated by functional magnetic resonance imaging (fMRI) using a 3T MR scanner. The experimental acoustic stimulus consisted of comprehensive sentences transformed to Morse code, which represent a non-metric rhythm with irregular perceptual accent structure. Activations were found in the right hemisphere, in the posterior parts of the right-sided superior and middle temporal gyri and temporal pole as well as in the orbital part of the right inferior frontal gyrus. Additional activation appeared in the left-sided superior temporal region. Our study suggests that non-metric rhythm with irregular perceptual accents structure is confined to the right hemisphere. Furthermore, a right-lateralised fronto-temporal network extracts the continuously altering temporal structure of the non-metric rhythm.

Caffeine is the most often used psychoactive substance. Caffeine may influence neuroplasticity in animals. We investigated the relationship between caffeine intake (coffee consumption) and brain morphology. Forty-five healthy, non-smoking women aged 19-30 were included in the present study. We used semi-automatic user-independent MR volumetry and voxel-based morphometry. We investigated the relationship between caffeine intake (coffee consumption) and the volumes of the cortical brain structures where caffeine is supposed to act. We found that high-level and low-level caffeine intake was associated with a larger hippocampus compared to moderate-level caffeine intake. The other brain structures showed no association with coffee consumption or caffeine intake. The U-shape association between caffeine concentration and its effect has already been described in some experimental studies. To our knowledge this is one of the very first studies, which tries to find an association between brain morphology and coffee consumption or caffeine intake in humans using MR imaging.

Deep brain stimulation of the subthalamic nuclei (STN) is a well established treatment in advanced Parkinson's disease (PD). Based on the clinical efficacy and elicited side-effects, both unipolar and bipolar stimulation modes may be applied. Bipolar stimulation usually produces a more focused and therefore thinner area of tissue activated during stimulation than unipolar stimulation does. The primary aim of our clinical study was to quantify the different clinical efficacy between these two stimulation modes. Twenty-one patients with PD previously underwent bilateral STN DBS implantation were involved in the study. Approximately three years after the implantation, we evaluated rigidity, tremor and bradykinesia according to the Unified Parkinson's disease Rating Scale in a practically off condition. Keeping the cathode of the chronic stimulation setting constant, the amplitude of stimulation was changed between 0 and 3.6 V by 0.2 V steps. Subsequently, the improvements in rigidity, tremor and bradykinesia were compared between unipolar and bipolar modes using 60 μs pulse-width and 130 Hz frequency. Within the examined amplitude range, unipolar stimulation usually had a significantly higher efficacy than bipolar stimulation; however, also with a higher rate of side-effects (19% vs. 0%). Depending on the evaluated parkinsonian symptoms, the efficacy of uni- and bipolar stimulation was different. To achieve the same level of improvement during bipolar stimulation, approximately 0.4-0.5 V higher amplitude was required than in unipolar mode. However in some cases, the efficacy of bipolar stimulation was unable the reach that of unipolar stimulation within the examined amplitude range.

Background/Aims: We investigated adaptive reorganization in Parkinson’s disease (PD) by fMRI using a passive movement task and compared the brain activation patterns of 10 patients with left- versus right-sided dominant symptoms. Five healthy controls were also investigated with the same settings. Methods: We grouped patients according to the predominant side of symptoms; thus, a right-sided dominant and a left-sided dominant group was formed. The paradigm consisted of a 4-finger passive movement task, which altered with resting states. For each subject, this examination was performed twice: on the left and on the right hand separately. Results: In healthy controls, motor-related areas contralateral to the moving fingers showed activation on fMRI. Concerning PD patients, motor-related areas of the ipsilateral hemisphere – including the primary motor cortex, supplementary motor area, and basal ganglia – seemed to be involved in the motor reorganization in PD. However, we could only demonstrate this reorganization in patients with right-sided dominant symptoms. Conclusions: We suggest that the human brain in PD tries to compensate for the failure of the basal ganglia motor loop by employing alternative (ipsilateral) motor pathways, indicating that a complex reorganization can also take place in disorders like PD which affect the whole motor-related network.

A neurológusszakorvos-hiány egyre nyilvánvalóbb problémává válik, nemcsak a kis kórházakban, hanem a súlyponti intézményekben és az egyetemi klinikákon is. A jelenlegi felmérés célja a neurológus szakorvosok jelenlegi számának áttekintése, valamint a következő évtizedben várható szakmai utánpótlás becslése. Működési engedéllyel 2010 elején Magyarországon 1310 neurológus rendelkezik. Az Országos Egészségbiztosítási Pénztár számára neurológiai tevékenységet 2009 során legalább 1 alkalommal 948 szakorvos jelentett. A rutin-betegellátásban ténylegesen elsősorban neurológiai területen dolgozók száma 750 körüli. A neurológusok életkori megoszlására jellemző a fiatal korosztály jelentős hiánya. Az ország 9 megyéjében legfeljebb egy 35 év alatti neurológus dolgozik. A 2000–2009 közötti tízéves időszakban, éves átlagban 22 új szakorvos lépett a rendszerbe. Ez a szám kevés a külföldre távozó és a betegellátásból egyéb okból kilépő neurológus szakorvosok pótlására. A 40–60 év közötti neurológusok száma a jelenlegi kormegoszlás alapján 2020-ra akkor is a jelenlegi érték kétharmada alá csökken, ha teljesen megszűnne a külföldi munkavállalás. Ha a külföldre vándorlás a jelenlegi szinten marad, és az utánpótlás mértéke nem nő, 2020-ra várhatóan 300 szakorvosra marad az ország neurológiai betegellátása. Mivel ez a szám a feladat ellátásához elégtelen, és a tendencia egyértelműen előre látható, az egészségügyi kormányzatnak sürgősen lépnie kell a jövő neurológus szakorvosi ellátásának biztosítása érdekében.

Background: One of the possible pathomechanisms of sudden death in epilepsy (SUDEP) is a postictal dysregulation of autonomic nervous system. We performed a heart rate variability (HRV) analysis of the periictal state to analyze whether a cardiac autonomic disturbance exists after an epileptic seizure. Methods: We included 31 periictal video–EEG–ECG recordings of 31 patients with epilepsy who had consecutively undergone pre-surgical evaluation. Nine generalized tonic-clonic (GTCS), 15 complex partial, and seven simple motor seizures were included. HRV was evaluated by analyzing 5-min-long ECG epochs, sampling from baseline, direct preictal, early-postictal (

Az osszefoglalo kozlemenyben a szerzők attekintest nyujtanak a neuromyelitis optica (NMO) epidemiologiajarol, klinikai jellegzetessegeiről. A kozelmultban leirt anti-aquaporin-4 (AQP-4) antitest a betegseget az ellenanyag-medialt csatornabetegsegek koze sorolja, es kimutatasa a diagnosztika fontos elemet kepezi. A szerzők hangsulyozzak a betegseg multidiszciplinaris jelleget: az NMO spektrumbetegsegkent definialt relapszalo latoideg-gyulladas (RION) az NMO-hoz hasonlo terapiat igenyelhet es szemeszeti diagnozisa alapvető, mig a szisztemas autoimmun korkepekhez tarsulo myelitisek es az NMO-val gyakran asszocialodo szisztemas autoimmun betegsegek a klinikai immunologusok es reumatologusok figyelmet igenylik. Alapvető a sclerosis multiplextől valo elkulonites az elterő terapias protokollok miatt. A kriteriumrendszer valtozasa, az NMO-spektrum-betegsegek definialasa, az NMO-specifikus IgG (anti-AQP-4) azonositasa, valamint megbizhato diagnosztikus tesztek a betegseg korai diagnozisat es igy adekvat terapiat e...

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is characterized by severe and frequent daily pain attacks causing transient physical disability for the patients during the headache period. Currently there is no option for abortive treatment of the attacks, mainly due to the short-lived nature and frequency of the repeated headaches, while highly efficacious therapy is also unavailable for short-term prevention. We report rapidly suppressed headache attacks with orally administered methylprednisolone in eight headache periods of three patients with idiopathic, episodic SUNCT syndrome. The remission was maintained until the period was over in all cases. Although the mechanism of methylprednisolone action is unclear, it is probably based on the anti-inflammatory effects of the drug.

This review summarizes protein biomarkers in mild and severe traumatic brain injury in adults and children and presents a strategy for conducting rationally designed clinical studies on biomarkers in head trauma.We performed an electronic search of the National Library of Medicine's MEDLINE and Biomedical Library of University of Pennsylvania database in March 2008 using a search heading of traumatic head injury and protein biomarkers. The search was focused especially on protein degradation products (spectrin breakdown product, c-tau, amyloid-beta(1-42)) in the last 10 years, but recent data on "classical" markers (S-100B, neuron-specific enolase, etc.) were also examined.We identified 85 articles focusing on clinical use of biomarkers; 58 articles were prospective cohort studies with injury and/or outcome assessment.We conclude that only S-100B in severe traumatic brain injury has consistently demonstrated the ability to predict injury and outcome in adults. The number of studies with protein degradation products is insufficient especially in the pediatric care. Cohort studies with well-defined end points and further neuroproteomic search for biomarkers in mild injury should be triggered. After critically reviewing the study designs, we found that large homogenous patient populations, consistent injury, and outcome measures prospectively determined cutoff values, and a combined use of different predictors should be considered in future studies.

Background and purpose: We studied the long-term crisis-preventing effect of combined prednisolone-azathioprine (PR-AZA) treatment in myasthenia gravis (MG). Methods: Case–control study with a median follow-up of 64 months in the treated group, and 80 months in the non-treated group. Sixty-nine patients with episodes of myasthenic crisis (MC) were treated and followed prospectively in 1990–2004. Twenty-seven patients had MC between 1990 and 1996, and were not treated with immunosuppressants on long-term. Long-term PR-AZA treatment was introduced in another 42 patients, who developed MC in 1997–2004. The difference in the frequency of repeated MCs between the treated and the non-treated group during long-term follow-up was investigated. As secondary end-points, we analyzed the number of admittances to the ICU; the number of mechanical ventilation episodes; the duration of ICU treatment; the characteristics of the applied treatment; and the functional outcome of the patients 1 month after the onset of the crisis. Results: Recurrent MCs occurred in 74% of the non-treated and 19% of the treated group (P

Hemorheological factors play an important role in the pathomechanism of ischemic cerebrovascular disorders. Abnormal rheological conditions in patients with chronic cerebrovascular disease predispose for recurrent strokes. Vinpocetine (VP), a synthetic ethyl esther of apovincamine, has successfully been used in the treatment of cerebrovascular diseases, in part because of its favourable rheological effects.The study investigates the hemorheological changes in 40 patients in the chronic stage of ischemic cardiovascular disease after administration of vinpocetine. All patients received a high dose of intravenous VP in doses gradually increased to l mg/kg/day. In addition, 20 patients (mean age: 61+/-8 years) received 30 mg VP orally for 3 months. The other 20 patients (mean age: 59+/-6 years), who received placebo tablets, served as controls. Hemorheological parameters (hematocrit, plasma fibrinogen, whole blood viscosity, red blood cell aggregation and deformability) were evaluated at 1 and 3 months.The high-dose parenteral VP significantly decreased red blood cell aggregation, plasma and whole blood viscosity (p0.05) compared to the initial values. In patients with additional oral treatment, plasma and whole blood viscosities were significantly lower compared to the placebo patients at 3 months (p0.05).Our results confirmed the beneficial rheological effects of high-dose parenteral VP (partially caused by hemodilution) observed previously, and also warrant its long-term oral admission to maintain the beneficial rheological changes.

A carotisstent beültetése a szignifikáns carotis stenosis kezelésének egyik lehetséges módszere. A beavatkozás során észlelhető kóros rheologiai viszonyok, fokozódó thrombocytaaggregáció és szabadgyök-képződés a stenttrombózis és a korai restenosis rizikóját növelhetik. Célkitűzés: Jelen tanulmányunk célja az volt, hogy megvizsgáljuk, hogyan változnak a haemorheologiai viszonyok, a thrombocytaaggregáció és az oxidatív stressz carotis stenosis endovascularis kezelése során és az azt követő napokban. Módszer: 18 (12 férfi, 6 nő, 66 ± 9 év) szignifikáns carotis stenosis miatt endovascularis úton kezelt beteg vizsgálatát végeztük el. Értékeltük a haemorheologiai paraméterek, valamint a thrombocytaaggregáció alakulását. Az oxidatív stressz jellemzésére a katalázaktivitást mértük. A méréseket közvetlenül a beavatkozás előtt, illetve után, majd 1, 2, 5 nappal és 1 hónappal később végeztük el. A beavatkozás előtt betegeink telítő dózisú (300 mg) clopidogrelkezelésben részesültek. Eredmények: A hematokrit, a plazmafibrinogén-koncentráció (Pfc), a teljesvér- (Tvv) és plazmaviszkozitás (Pv) a stentelést követően azonnal szignifikánsan csökkent ( p < 0,001). A beavatkozás 5. napjára a Tvv, a Pfc, a Pv, a vörösvértest és a thrombocytaaggregáció szignifikánsan ( p < 0,0001) fokozódott a stentelés után mért értékekhez képest. Az 1 hónapos kontrollnál ugyanezen paraméterek, kivéve a Tvv, szignifikáns csökkenést mutattak az 5. napi értékekhez képest. A katalázaktivitás beavatkozás utáni napon mért értéke az 1 hónapos kontrollra szignifikánsan emelkedett. Konklúzió: A haemorheologiai paraméterek és a thrombocytaaggregáció a carotisstent-beültetés kapcsán specifikus változást mutatnak. A vér áramlási viszonyainak kóros megváltozása és a thrombocyták fokozódó aktivációja a beavatkozás első hetében a legkifejezettebbek, a stent thromboticus elzáródását indukálhatják. A beavatkozás kapcsán indukálódó oxidatív stressz mértéke 1 hónap után csökken szignifikánsan.

Background:Patients with stroke are more susceptible to infections, suggesting possible deficiencies of early immune responses, particularly of leucocytes.Aims:To serially examine leucocyte antisedimentation rate (LAR), a simple test to detect activation of leucocytes, and correlate it with S100β, procalcitonin and outcome in patients with acute ischaemic events.Methods:Venous blood samples were taken from 61 healthy volunteers and 49 patients with acute ischaemic events (acute ischaemic stroke (AIS), n = 38; transient ischaemic attack (TIA), n = 11) within 6 hours, at 24 and 72 hours after onset of symptoms.Results:LAR was significantly higher in acute ischaemic events compared to controls within 6 hours after onset of stroke regardless of post-stroke infections. In addition, the increase of LAR was delayed and attenuated in TIA in contrast to AIS. A deficiency in early increase of LAR was associated with post-stroke infections and a poor outcome, measured by the Glasgow Outcome Scale in AIS. There was a positive correlation between LAR and S100β at 72 hours after the onset of ischaemic stroke. Increased levels of S100β at 24 and 72 hours after stroke were associated with poor outcome.Conclusions:An early activation of leucocytes indicated by an increase of LAR is characteristic of acute ischaemic cerebrovascular events. A delayed and ameliorated leucocyte activation represented by LAR is characteristic of TIA in contrast to stroke. Deficient early activation predisposes to post-stroke infections related to poor outcome. In addition, the extent of tissue injury correlates with the magnitude of innate immune responses.

We aimed to determine the functional localisation of right-left discrimination (RLD) by functional MRI (fMRI). In this study, 16 male volunteers were examined. There were three task sessions: one active and two baseline tasks. During the baseline tasks participants were instructed to show numbers with their fingers. The first baseline task was performed with the right hand, the second one with the left hand. During the active (RLD) task participants were also instructed to show numbers. The difference between baseline and active tasks was that during the active task the hand with which the participant should perform the instruction was assigned randomly. Thus, participants were unaware which hand should be used before the instruction command. During RLD, activations occurred in the right-sided frontal, precuneus, postcentral, angular, lingual, and superior temporal gyri. Activations also appeared in the left-sided temporal gyri and precuneus. Of the activations, 76.7% appeared in the right hemisphere, 23.3% in the left hemisphere. Conclusively, we found that RLD is mainly related to the right hemisphere, and requires activation of the parieto-temporo-occipital junction and the visual system including cuneus, precuneus, and gyrus lingualis.

Cerebrovascularis betegekben gyakoriak a haemorrheologiai és haemostaseologiai paraméterek kóros eltérései. A statinok az ischaemiás stroke prevenciójában hatékonyan alkalmazható szerek, kedvező hatásuk hátterében a lipidszint csökkentése mellett egyéb tényezők is igazolhatók. Célok: Tanulmányukban a szerzők áttekintik az atorvastatin stroke-prevencióval, haemorrheologiai és haemostaseologiai hatásaival kapcsolatos irodalmi adatokat, hivatkozva egy korábbi vizsgálatuk eredményeire, mely kis dózisú atorvastatinkezelés rövid távú haemorrheologiai, valamint endothel-diszfunkcióra és a thrombocytaaggregációra gyakorolt effektusait vizsgálta. Módszerek: 27 krónikus, hyperlipidaemiás agyérbetegnél (átlagéletkor: 61 ± 8 év) meghatározták a szérumlipidszinteket, a haemorrheologiai paramétereket (haematocrit, plazmafibrinogén-koncentráció, plazma- és teljesvér-viszkozitás, vörösvértest-aggregáció és -deformabilitás), a thrombocytaaggregációt kiinduláskor, majd 1 és 3 hónappal napi 10 mg atorvastatinkezelést követően. Az endothel-diszfunkciót jelző von Willebrand-faktor aktivitását a kezelés előtt, majd 1 hónap múlva mérték meg. Eredmények: A plazmakoleszterin-szint átlagos csökkenése 1, illetve 3 hónap múlva egyaránt 28% volt ( p < 0,001), az LDL-koleszterin-szint 40%-kal, illetve 38%-kal ( p < 0,001) csökkent a kiindulási értékhez képest. Az atorvastatinkezelés a teljesvér-viszkozitást a 3. hónap végére, míg a vvt-deformabilitást már az első hónap végére szignifikánsan csökkentette ( p < 0,05). A kollagén indukálta thrombocytaaggregáció a kiindulási értékhez képest szignifikánsan csökkent ( p < 0,001) változatlan antiaggregációs kezelés mellett. Szintén szignifikánsan javult a von Willebrand-faktor-aktivitás már 1 hónapos kezelést követően ( p < 0,05). Következtetések: Mind az irodalmi, mind a szerzők saját eredményei az atorvastatin komplex, kedvező hatására utalnak. A lipidcsökkentő effektus mellett a kis dózisban és rövid ideig alkalmazott atorvastatin javítja a haemorrheologiai paramétereket, a thrombocytaaggregációt és az endothel-diszfunkciót.

Oligodendroglial cells differ in their ultrastructural appearance depending on their myelin producing and maintaining activity. To better understand the relationship between light and electron microscopic features of myelination, myelin formation in the corpus callosum was studied in young postnatal mice. Immunostaining for myelin basic protein (MBP), which has an important role in myelin compaction, was compared with conventional Luxol Fast Blue myelin staining and with electron microscopic images of unlabeled tissue. MBP-immunostaining labeled a few oligodendroglial cells at postnatal day (P)3, and a few axons at P7 in the corpus callosum, below the fronto-parietal somatosensory cortex. By P10 there were more myelinated axons below the somatosensory cortex and the first MBP-immunoreaction appeared in the cingulum: labeling appeared even later in the remaining areas of corpus callosum. Electron microscopy revealed numerous medium oligodendroglial cells at P7 in the corpus callosum, below the somatosensory cortex with the first sign of myelination at P10. By P14, there were numerous myelin sheaths with loosely built structure, and the number of myelin sheaths increased continuously thereafter. However, even as late as P28, the presence of both thick, compact and thin, loosely structured myelin sheaths in the same section suggested ongoing myelination. With Luxol Fast Blue myelin staining was first observed in the corpus callosum relatively late, at P14. Areal differences in myelination of the corpus callosum, seen with MBP-immunohistochemistry, indicate that myelin formation follows cortical maturation rather than the rostro-caudal developmental growth of the corpus callosum. Myelination of the afferent and efferent fibers within the cortical areas seems to follow the inside-out maturational pattern of cortical neurons, with the first myelinated axons always appearing in layers V-VI. In addition to the known neuronal and astroglial factors that regulate myelin formation by oligodendroglial cells, we suggest that these cells and their myelin covering may also influence axonal maturation. Light microscopic data obtained with MBP-immunohistochemistry correlates well with electron microscopic observations but not with Luxol Fast Blue staining which reveals myelinated axons only relatively late in development. Therefore, both MBP-immunostaining and electron microscopy are useful, alone or in combination, for the detection of myelination, demyelination as well as remyelination processes in animal models and also in humans.

Objective To test if mitochondrial (mt)DNA deletions accumulate in brains of patients with multiple sclerosis (MS). Background Previous studies demonstrated an accumulation of oxidative damage to mtDNA and decreased activity of mitochondrial enzymes in lesions of MS, where activated immune cells produce increased amounts of reactive oxygen species and nitric oxide. The unknown link between oxidative damage and decreased activity of mitochondrial enzymes may be the accumulation of deletions in mtDNA molecules. mtDNA deletions in the brain have been associated with neurodegeneration and aging. Methods mtDNA deletions were quantified by using real-time PCR in laser-dissected, COX-positive and COX-negative single neuronal and glial cells from frozen postmortem brain tissue specimens including normal appearing gray (NAGM) and white matter (NAWM) regions and chronic active plaques of MS patients, and gray matter (GM) and white matter (WM) regions of age-matched controls. Three patients with advance Alzheimer’s and Parkinson’s diseases were included as positive controls. The proportion of deleted mtDNA molecules was correlated with pathology and age. Results We detected no pathology-related accumulation of mtDNA deletions when comparisons were made among NAGM, NAWM, and plaque of MS brains, or between NAGM–GM and NAWM–WM of patients and age-matched controls. However, an accumulation of mtDNA deletions was noted in non-neurological controls beyond 60 years of age and in patients with Alzheimer’s and Parkinson’s diseases. As expected, the rate of mtDNA deletions was higher in COX− than in COX+ cells. Conclusion While aging and neurodegeneration in PD and AD are associated with accumulation of COX− cells and mtDNA deletions, the pathology of MS is not.

A method which demonstrates myelinated axons in the central and peripheral nervous systems will be described. Paraffin, frozen or semi-thin epoxy-embedded sections were immunostained first with a monoclonal antibody raised against a 200 kilo-Dalton neurofilament protein and then counter-stained with Luxol fast blue.

The importance of the sleep related breathing disorders (obstructive sleep apnea syndrome, central sleep apnea, and Cheyne-Stokes breathing) in the pathophysiology crebro- and cardiovascular disorders is well known. The relationship of sleep related breathing abnormalities and epilepsy is also important but underestimated in the daily practice. The relation is bidirectional. The breathing abnormalities in sleep may play important role in generating epileptic seizure, but the adverse effect of seizure and antiepileptic therapy (generation of apneas and hypopneas) may worsen the seizure control. The effect of new therapies (vagal nerve and deep brain stimulation) on the sleep architecture and sleep disordered breathing must be examined and discussed. Here we present a brief case of epileptic patient with deep brain stimulation therapy on sleep as well. The examination of the sleep related breathing abnormalities in epilepsy patient may help improve the effectiveness of antiepileptic therapy.

The recently published "EarlyStim" study demonstrated that deep brain stimulation (DBS) for the treatment of Parkinson's disease (PD) with early fluctuations is superior to the optimal pharmacological treatment in improving the quality of life and motor symptoms, and preserving sociocultural position. Our retrospective investigation aimed to evaluate if DBS therapy was able to preserve the working capabilities of our patients.We reviewed the data of 39 young (60 years-old) PD patients who underwent subthalamic DBS implantation at University of Pécs and had at least two years follow-up. Patients were categorized into two groups based on their working capabilities: Patients with active job ("Job+" group, n = 15) and retired patients (without active job, "Job-" group, n = 24). Severity of motor symptoms (UPDRS part 3), quality of life (EQ-5D) and presence of active job were evaluated one and two years after the operation.As far as the severity of motor symptoms were concerned, similar (approximately 50%) improvement was achieved in both groups. However, the postoperative quality of life was significantly better in the Job+ group. Majority (12/15, 80%) of Job+ group members were able to preserve their job two years after the operation. However, only a minimal portion (1/24, 4.2%) of the Job- group members was able to return to the world of active employees (p0.01, McNemar test).Although our retrospective study has several limitations, our results fit well with the conclusions of "EarlyStim" study. Both of them suggest that with optimal timing of DBS implantation we may preserve the working capabilities of our patients.

Background The common symptoms of multiple sclerosis are fatigue, depression, cognitive dysfunction, pain and sexual dysfunction, which influence the health-related quality of life of the patients. Objective We aimed to determine the correlations between the health-related quality of life, the level of disability, fatigue and depression in glatiramer acetate-treated patients with multiple sclerosis in Hungary. Methods The Hungarian versions of the Multiple Sclerosis Quality of Life-54, Fatigue Impact Scale and Beck Depression Inventory questionnaires were completed by 428 relapsing-remitting multiple sclerosis patients treated with glatiramer acetate from 19 Hungarian centers. Results The prevalence of fatigue was found to be 62.4%. The prevalence of depression was lower (13.4%) than that described in previous studies (36–54%) among patients with multiple sclerosis. Significant differences in the health-related quality of life were found between fatigued and non-fatigued patients. The level of disability, fatigue, depression and the duration of the disease correlated significantly with the quality of life. However, linear regression analysis indicated that the quality of life was predicted by the level of disability, depression, social and cognitive fatigue, but not by physical fatigue. Conclusions Decreasing the disease activity in multiple sclerosis with immunomodulatory therapy, together with improvements of the diagnostics and treatment of the accompanying depression and fatigue are of high priority to improve the health-related quality of life of patients with multiple sclerosis.

Sleep problems are one of the most common non-motor symptoms of Parkinson’s disease (PD). The Parkinson’s disease Sleep Scale 2nd version (PDSS-2) was published in 2011 showing satisfactory clinimetric results. We performed an independent testing of the scale adding further information on its clinimetric properties. In this nationwide study 537 PD patients were enrolled. Besides PDSS-2, we assessed Patient’s Global Impression-Severity (PGI) scale on sleep disturbances, Non-motor Symptoms Scale and MDS-UPDRS. Following the Classical Theory of Tests we performed descriptive data analysis, factor analysis, reliability, validity and precision measurements. Subsequently, we evaluated cut-off value for detecting clinically meaningful sleep problems based on receiver operating characteristics analysis. Based on the PGI scale, 161 patients (30.0 %) did not reported any sleep problems. Factor analysis revealed almost the same factor structure described by the original PDSS-2 validation study. Cronbach’s alpha was 0.863 and all item had good item-total correlation. PDSS-2 demonstrated high convergent validity with Non-Motor Symptoms Scale and Clinical Global Impression-Severity and non-motor part of MDS-UPDRS, and divergent validity with age, gender, education level, disease-duration and Hoehn–Yahr Stages. Presence of sleep problems was identified by scores >10.5 points on PDSS-2 (sensitivity 85.3 %, specificity 60.8 %, diagnostic accuracy 78.1 %); whereas scores >19.5 points indicated marked sleep-related problems (specificity 68.5 %, sensitivity 78.0 %, diagnostic accuracy 74.3 %). Independent and cross-cultural validation of patient reported outcomes is essential to confirm or reject the findings obtained by the developers of the scale. Our results demonstrate that fundamental clinimetric properties of the PDSS-2 are satisfactory.