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© 2022 European Academy of Neurology, Background and purpose: Rare diseases affect up to 29 million people in the European Union, and almost 50% of them affect the nervous system or muscles. Delays in diagnosis and treatment onset and insufficient treatment choices are common. Clinical practice guidelines (CPGs) may improve the diagnosis and treatment of patients and optimize care pathways, delivering the best scientific evidence to all clinicians treating these patients. Recommendations are set for developing and reporting high-quality CPGs on rare neurological diseases (RNDs) within the European Academy of Neurology (EAN), through a consensus procedure. Methods: A group of 27 experts generated an initial list of items that were evaluated through a two-step Delphi consensus procedure and a face-to-face meeting. The final list of items was reviewed by an external review group of 58 members. Results: The consensus procedure yielded 63 final items. Items are listed according to the domains of the AGREE instruments and concern scope and purpose, stakeholder involvement, rigour of development, and applicability. Additional items consider reporting and ethical issues. Recommendations are supported by practical examples derived from published guidelines and are presented in two tables: (1) items specific to RND CPGs, and general guideline items of special importance for RNDs, or often neglected; (2) items for guideline development within the EAN. Conclusions: This guidance aims to provide solutions to the issues specific to RNDs. This consensus document, produced by many experts in various fields, is considered to serve as a starting point for further harmonization and for increasing the quality of CPGs in the field of RNDs.

BACKGROUND AND PURPOSE: In these guidelines, we aimed to develop evidence-based recommendations for the use of screening questionnaires and diagnostic tests in patients with neuropathic pain (NeP).METHODS: We systematically reviewed studies providing information on the sensitivity and specificity of screening questionnaires, and quantitative sensory testing, neurophysiology, skin biopsy, and corneal confocal microscopy. We also analysed how functional neuroimaging, peripheral nerve blocks, and genetic testing might provide useful information in diagnosing NeP.RESULTS: Of the screening questionnaires, Douleur Neuropathique en 4 Questions (DN4), I-DN4 (self-administered DN4), and Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) received a strong recommendation, and S-LANSS (self-administered LANSS) and PainDETECT weak recommendations for their use in the diagnostic pathway for patients with possible NeP. We devised a strong recommendation for the use of skin biopsy and a weak recommendation for quantitative sensory testing and nociceptive evoked potentials in the NeP diagnosis. Trigeminal reflex testing received a strong recommendation in diagnosing secondary trigeminal neuralgia. Although many studies support the usefulness of corneal confocal microscopy in diagnosing peripheral neuropathy, no study specifically investigated the diagnostic accuracy of this technique in patients with NeP. Functional neuroimaging and peripheral nerve blocks are helpful in disclosing pathophysiology and/or predicting outcomes, but current literature does not support their use for diagnosing NeP. Genetic testing may be considered at specialist centres, in selected cases.CONCLUSIONS: These recommendations provide evidence-based clinical practice guidelines for NeP diagnosis. Due to the poor-to-moderate quality of evidence identified by this review, future large-scale, well-designed, multicentre studies assessing the accuracy of diagnostic tests for NeP are needed.

(1) Background: The SARS-CoV-2 pandemic had a significant impact on the management of traumatic brain injury (TBI). We aimed to compare the clinical characteristics and outcomes of TBI patients before and during the SARS-CoV-2 pandemic.; (2) Methods: We analyzed depicted data from existing medical records on sex, age, mechanism of injury, clinical performance at admission and discharge, neuroimaging, laboratory values at admission, mortality, duration of hospitalization, and referrals after discharge from the traumatology department for all adult patients during the SARS-CoV-2 pandemic and a year before. Variables were compared using the Chi-square or t-test between both groups.; (3) Results: Most patients had mild (n = 477), followed by moderate (11) and severe (11) TBI. Mild TBI was less frequent during the SARS-CoV-2 period (n = 174 vs. n = 303). The incidence of high falls increased during the SARS-CoV-2 period (14.5% vs. 24.7%; p < 0.05) in the group with mild TBI. Patients had similar mean Glasgow Coma Scales (GCS), Glasgow Outcome Scales-Extended (GOSE), and glucose levels at admission before and during the pandemic. Serum ethanol levels were significantly lower during the SARS-CoV-2 period (1.3 ± 0.7 mmol/L vs. 0.7 ± 1.2 mmol/L; p < 0.001). At discharge, the mean GCS was significantly lower (14.7 ± 1.8 vs. 14.1 ± 0.5; p < 0.05) for patients treated during the SARS-CoV-2 period than before the SARS-CoV-2 period. There were no differences in GOSE; (4) Conclusions: our results demonstrated a significant impact of SARS-CoV-2 pandemic on the frequency, mechanism, and consequences of TBI, and may help improve care for our patients.

Parkinsonova bolezen (PB) je nevrodegenerativna bolezen možganov, ki se kaže z motoričnimi in nemotoričnimi znaki. Med motorične znake prištevamo tremor, rigidnost in bradikinezijo, med nemotoričnimi pa je najpogostejši kognitivni upad. Rezultati longitudinalnih raziskav kažejo, da pri 80 % bolnikov kognitivni upad napreduje do demence (PBD). Vpliv PBD na življenje je velik. Če kognitivni upad prepoznamo dovolj zgodaj, lahko simptome PBD olajšamo in s tem izboljšamo kakovost posameznikovega življenja. V preglednem članku se bomo dotaknili najpogostejših vzrokov za nastanek kognitivnega upada, vpliva dednosti in prikazali značilno klinično sliko. Za prepoznavo kognitivnega upada uporabljamo kognitivne teste, npr. Montrealsko lestvico ocenjevanja spoznavnih sposobnosti (MoCA), Baterijo testov frontalnih funkcij (FAB), Kratek poskus spoznavnih sposobnosti (KPSS) in test Testiraj svoj spomin (TSS). Na koncu članka bomo prikazali možnosti simptomatskega zdravljenja z zdravili, ki jih imamo na voljo, ter z zdravili in postopki, ki so še v fazi razvoja.

Background and purpose: Neurological disorders pose a profound unmet medical need for which new solutions are urgently needed. The consideration of both biological (sex) and socio-cultural (gender) differences between men and women is necessary to identify more efficacious, safer and tailored treatments. Approaches for putting sex and gender medicine into practice have gathered momentum across Europe, but it is currently unclear to what extent they have been implemented in the field of neurology and neuroscience. Methods: We mapped current activities in research, funding and education aimed at integrating sex and gender consideration in neuroscience and neurology in Europe. We examined and analyzed data gathered from literature searches, policy documents and reports by the European Commission and national funding agencies, web-based searches, Web of Science, and searches of project databases of funding agencies. An informative/non-systematic search was performed for sections on policies and funding, education, and basic research, while a systematic literature and database review was conducted for quantitative analysis of research output and funded projects in terms of sex and gender analysis. Results: Our mapping shows that there is a growing interest in and attention given to sex and gender considerations in neurological fields, both from funding agencies and researchers. However, most activities, especially for education, are limited to the individual motivation of researchers and are not organically built within curricula and strategic research priorities. Conclusion: We recommend actions that might help increase the consideration of sex and gender specifically in the field of neuroscience and neurology.

People with epilepsy frequently have cognitive impairment. The majority of cognitive problems is influenced by a variety of interlinked factors, including the early onset of epilepsy and the frequency, intensity and duration of seizures, along with the anti-epileptic drug treatment. With a systematic review, we investigate significant factors about the cognitive impairment in epilepsy. Most cognitive problems in adult people with epilepsy include memory, attention and executive function deficits. However, which cognitive area is mainly affected highly depends on the location of epileptic activity. Moreover, modifications in signalling pathways and neuronal networks have an essential role in both the pathophysiology of epilepsy and in the mechanism responsible for cognitive impairment. Additionally, studies have shown that the use of polytherapy in the treatment of epilepsy with anti-epileptic drugs (AEDs) heightens the risk for cognitive impairment. It can be challenging to distinguish the contribution of each factor, because they are often closely intertwined.

Introduction: We examined the association between cerebrospinal fluid (CSF)-derived biomarkers of Alzheimer's disease and neuropsychiatric symptoms (NPS) in older non-demented adults. Methods: We included 784 persons (699 cognitively unimpaired, 85 with mild cognitive impairment) aged ≥ 50 years who underwent CSF amyloid beta (Aβ42), hyperphosphorylated tau 181 (p-tau), and total tau (t-tau) as well as NPS assessment using Beck Depression and Anxiety Inventories (BDI-II, BAI), and Neuropsychiatric Inventory Questionnaire (NPI-Q). Results: Lower CSF Aβ42, and higher t-tau/Aβ42 and p-tau/Aβ42 ratios were associated with BDI-II and BAI total scores, clinical depression (BDI-II ≥ 13), and clinical anxiety (BAI ≥ 10), as well as NPI-Q–assessed anxiety, apathy, and nighttime behavior. Discussion: CSF Aβ42, t-tau/Aβ42, and p-tau/Aβ42 ratios were associated with NPS in community-dwelling individuals free of dementia. If confirmed by a longitudinal cohort study, the findings have clinical relevance of taking into account the NPS status of individuals with abnormal CSF biomarkers.

Coronavirus disease 2019 (COVID‐19), a multi‐organ disease caused by severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2), continues to challenge health and care systems around the globe. The pandemic has disrupted acute neurology services and routine patient care and has impacted the clinical course in patients with chronic neurological disease. COVID‐19 appears to have exposed inequalities of societies and healthcare systems and had a disproportionate impact on already vulnerable communities. The next challenge will be to set up initiatives to stop disparities in all aspects related to COVID‐19. From the medical perspective, there is a need to consider inequalities in prevention, treatment and long‐term consequences. Some of the issues of direct relevance to neurologists are summarised. With this appraisal, the European Academy of Neurology NeuroCOVID‐19 Task Force intends to raise awareness of the potential impact of COVID‐19 on inequalities in healthcare and calls for action to prevent disparity at individual, national and supranational levels.

In this letter-to-the-editor the Task Force on Gender and Diversity issues in Neurology founded under the auspices of the European Academy of Neurology (EAN) addresses various gender issues that are arising during the COVID-19 pandemic. These issues concern different aspects, spanning from gender disparities in health care workforce to gender differences amongst patients suffering from COVID-19, risk factors, occurrence of neurological complications and (outcomes of) management. Due to the continuous flow of COVID-19 related papers this review cannot be comprehensive. We attempted to select the most relevant literature on this topic.

Immunization by means of vaccination is a global health success story, saving millions of lives every year. In this regard, the epidemiology of measles, rabies, polio, rubella, varicella, influenza and mumps infections, all of which can harm the nervous system, could be contained by global vaccination campaigns. In addition, toxoid vaccines against bacterial toxins such as tetanus and diphtheria are indispensable and effective interventions for toxin-mediated neurologic diseases.

Vascular cognitive impairment (VCI) is a common feature of vascular brain lesions. The heterogeneity of clinical presentation after a stroke makes it challenging to find. However, the Montreal Cognitive Assessment Scale (MoCA) may be suitable for such purpose. Aim of the present study was to validate the Slovenian version of MoCA for detecting cognitive impairment after ischemic stroke. Seventy patients up to three months after ischemic stroke and 69 age-matched healthy control were included. All performed the MoCA and Hachinski Ischemic Scale (HIS). Cut-off value, sensitivity, specificity and likelihood ratio of MoCA were calculated with the receiver operating characteristic curve. Mean age of healthy controls was 67.1 (SD 9.2) years, mean MoCA was 25.8 (SD 2.6) points, and mean HIS was [1.9 (SD 1.5). There were no age differences between healthy controls and patients [70.4 (SD 10.9) years]. However, patients performed significantly worse on MoCA [mean 18.7 (SD 6.3) points) and had higher HIS score (7.6 (SD 0.3) points]. Optimal cut-off values for MoCA were 24/25 points. The area under the curve was 0.86, sensitivity was 81%, and specificity was 70%. A negative correlation was found between MoCA and education and age. Our results demonstrate that an optimal cut-off for screening for VCI in the Slovenian population is 24/25 points on MoCA, which is similar to other studies performed on patients with different cultural background. The test is easy to perform and could be used in daily clinical practice.

Alzheimer's disease (AD) is the most common cause of neurodegenerative dementia where the most prominent cognitive impairment is memory loss. Screening tests are valuable tools for detecting cognitive impairment. One such test is the Alzheimer's Disease Assessment Scale–Cognitive Subscale (ADAS-Cog) which consists of 11 tasks. The main goal of our study was to validate the Slovenian version of the ADAS-Cog for detecting mild cognitive impairment (MCI) and AD patients. Our sample included 128 participants: 61 healthy controls, 32 people with MCI, and 35 with AD. All participants were evaluated with the Slovenian translation of ADAS-Cog. The optimal cut-off for the ADAS-Cog for MCI was 10/11 points. The sensitivity was 88%, specificity 85% and likelihood ratio 6. The optimal cut-off for AD was 11/12 points. Sensitivity was 100%, specificity 95% and likelihood ratio 20. The obtained cut-off values are comparable to the results from similar validation studies. We can conclude that the ADAS-Cog is a reliable and valid tool for identifying people with MCI and AD from among healthy controls in the Slovenian population.

Alzheimer’s disease (AD) pathology is partly characterized by accumulation of aberrant forms of tau protein. Here we report the results of ADAMANT, a 24-month double-blinded, parallel-arm, randomized phase 2 multicenter placebo-controlled trial of AADvac1, an active peptide vaccine designed to target pathological tau in AD (EudraCT 2015-000630-30). Eleven doses of AADvac1 were administered to patients with mild AD dementia at 40 μg per dose over the course of the trial. The primary objective was to evaluate the safety and tolerability of long-term AADvac1 treatment. The secondary objectives were to evaluate immunogenicity and efficacy of AADvac1 treatment in slowing cognitive and functional decline. A total of 196 patients were randomized 3:2 between AADvac1 and placebo. AADvac1 was safe and well tolerated (AADvac1 n = 117, placebo n = 79; serious adverse events observed in 17.1% of AADvac1-treated individuals and 24.1% of placebo-treated individuals; adverse events observed in 84.6% of AADvac1-treated individuals and 81.0% of placebo-treated individuals). The vaccine induced high levels of IgG antibodies. No significant effects were found in cognitive and functional tests on the whole study sample (Clinical Dementia Rating-Sum of the Boxes scale adjusted mean point difference −0.360 (95% CI −1.306, 0.589)), custom cognitive battery adjusted mean z-score difference of 0.0008 (95% CI −0.169, 0.172). We also present results from exploratory and post hoc analyses looking at relevant biomarkers and clinical outcomes in specific subgroups. Our results show that AADvac1 is safe and immunogenic, but larger stratified studies are needed to better evaluate its potential clinical efficacy and impact on disease biomarkers. The authors present the results of a 24-month phase 2 study of AADvac1, a tau vaccine against Alzheimer’s disease. AADvac1 was safe and induced high levels of antibodies. In the whole study sample, there were no significant changes on clinical outcomes.

OBJECTIVES Sleep-wake disorders are common in the general population and in most neurological disorders but are often poorly recognized. With the hypothesis that neurologists do not get sufficient training during their residency, the Young European Sleep Neurologist Association (YESNA) of the European Academy of Neurology (EAN) performed a survey on postgraduate sleep education. METHODS A 16-item questionnaire was developed and distributed among neurologists and residents across European countries. Questions assessed demographic, training and learning preferences in sleep disorders, as well as a self-evaluation of knowledge based on five basic multiple-choice questions (MCQs) on sleep-wake disorders. RESULTS The questionnaire was completed by 568 participants from 20 European countries. The mean age of participants was 31.9 years (SD 7.4 years) and was composed mostly of residents (73%). Three-quarters of the participants reported undergraduate training in sleep medicine, while fewer than 60% did not receive any training on sleep disorders during their residencies. Almost half of the participants (45%) did not feel prepared to treat neurological patients with sleep problems. Only one-third of the participants correctly answered at least three MCQs. Notably, 80% of participants favoured more education on sleep-wake disorders during the neurology residency. CONCLUSIONS Education and knowledge on disorders in European neurological residents is generally insufficient, despite a strong interest in the topic. The results of our study may be useful for improving the European neurology curriculum and other postgraduate educational programmes.

Background and purpose\ud \ud The recent SARS‐CoV‐2 pandemic has posed multiple challenges to the practice of clinical neurology including recognition of emerging neurological complications and management of coexistent neurological diseases. In a fast‐evolving pandemic, evidence‐based studies are lacking in many areas. This paper presents European Academy of Neurology (EAN) expert consensus statements to guide neurologists caring for patients with COVID‐19.\ud \ud \ud Methods\ud \ud A refined Delphi methodology was applied. In round 1, statements were provided by EAN scientific panels (SPs). In round 2, these statements were circulated to SP members not involved in writing them, asking for agreement/disagreement. Items with agreement >70% were retained for round 3, in which SP co‐chairs rated importance on a five‐point Likert scale. Results were graded by importance and reported as consensus statements.\ud \ud \ud Results\ud \ud In round one, 70 statements were provided by 23 SPs. In round two, 259/1061 SP member responses were received. Fifty‐nine statements obtained >70% agreement and were retained. In round three, responses were received from 55 co‐chairs of 29 SPs. Whilst general recommendations related to prevention of COVID‐19 transmission had high levels of agreement and importance, opinion was more varied concerning statements related to therapy.\ud \ud \ud Conclusion\ud \ud This is the first structured consensus statement on good clinical practice in patients with neurological disease during the COVID‐19 pandemic that provides immediate guidance for neurologists. In this fast‐evolving pandemic, a rapid response using refined Delphi methodology is possible, but guidance may be subject to change as further evidence emerges.

OBJECTIVE: The purpose of this study was to test the hypothesis that subcortical β-amyloid (Aβ) deposition was associated with elevated scores on standardized measures of depressive and anxiety symptoms when compared with cortical (Aβ) deposition in persons without dementia. METHODS: The authors performed a cross-sectional study, derived from the population-based Mayo Clinic Study of Aging, comprising participants aged ≥70 years (N=1,022; 55% males; 28% apolipoprotein E [APOE] ε4 carriers; without cognitive impairment, N=842; mild cognitive impairment; N=180). To assess Aβ deposition in cortical and subcortical (the amygdala, striatum, and thalamus) regions, participants underwent Pittsburgh Compound B positron emission tomography (PiB-PET) and completed the Beck Depression Inventory-II (BDI-II) and the Beck Anxiety Inventory (BAI). The investigators ran linear regression models to examine the association between PiB-PET standardized uptake value ratios (SUVRs) in the neocortex and subcortical regions and depressive and anxiety symptoms (BDI-II and BAI total scores). Models were adjusted for age, sex, education level, and APOE ε4 carrier status and stratified by cognitive status (without cognitive impairment, mild cognitive impairment). RESULTS: Cortical PiB-PET SUVRs were associated with depressive symptoms (β=0.57 [SE=0.13], p

Background/Aim: The Clock Drawing Test (CDT) is a valid alternative screening tool to the Mini-Mental State Examination (MMSE) and, crucially, it may be completed faster. The aim of our study was to standardize and simplify the CDT scoring system for screening in three common conditions: mild cognitive impairment (MCI), Alzheimer’s disease (AD) and mixed dementia (MD). Methods: We included 188 subjects (43 healthy volunteers, 49 patients with MCI, 54 patients with AD, and 42 patients with MD), who performed the MMSE and CDT. The CDT was evaluated using a modified 4-point scoring system. Results: The healthy subjects had the highest median values for the MMSE and CDT, followed by patients with MCI, AD and MD. The optimal cut-off for all patients and each patient group separately was 3 out of 4 points. Sensitivity was 89% for AD, 93% for MD and 83% for all patients, while specificity was 91%. The MMSE produced similar results. In comparison to the MMSE, sensitivity for MCI was significantly higher using the CDT (20 vs. 69%, respectively). Conclusion: A simple, 4-point scoring system may be used as a screening method for fast and accurate detection of cognitive impairment in patients with MCI, AD and MD.

Simple imaginary movements activate similar cortical and subcortical areas to actual movements, chiefly in the sensory-motor network. However, only a few studies also examined the imagery of more skilful movements such as reaching. Ten volunteers performed reaching movements or imagined the same movements. EEG was simultaneously recorded and analysed with sLORETA, which compared the preparation for actual and imagined reaching with respect to their baseline and between tasks. Major differences between them were found at three time intervals after target presentation, always in favour of the actual reaching condition. The first one was from 160 to 220 msec in the frontal and parietal regions. The second difference was evident from 220 to 320 msec in the premotor cortex. The third difference was evident from 320 msec, mainly in the perirolandic region. Also, the anterior and posterior cingulate cortices were widely involved, in both tasks. We suggest the existence of two separate systems which may work together during actual reaching programming. The first one involves structures such as the premotor cortex, supplementary motor area and primary motor cortex, together with the parietal and occipital cortex. This system may integrate extrinsic target coordinates with proprioceptive information from the reaching arm and pre-stored programs in the associative motor cortex. It is activated strongly and involves more cortical areas in actual than imagined reaching. The second system, common to both tasks, involves anterior and posterior cingulate cortices, with the possible role of contributing awareness and focusing the various components of the process.

Background Classical Trigeminal neuralgia (CTN) is a severe, disabling form of painful cranial neuropathy. Even though CTN has a typical clinical picture, diagnosis it is often missed or delayed in clinical practice. This study was carried over to investigate the occurrence of diagnostic and therapeutic errors in CNT. Methods We studied 102 patients suffering from CTN recruited through a multicentric survey. All the patients were evaluated using telephone/frontal interviews conducted by headache/pain specialists using an ad hoc questionnaire. Results Patients were mostly females (F:M ratio 2.64:1); in 50% of cases age ranged between 51 and 70 years. Eighty-six percent of the patients consulted a physician at the time they experienced the first pain attacks. Specialists consulted before CTN diagnosis was finally made were: primary care physicians (43.1%), dentists (in 30.4% of cases), otorhinolaryngologists (3.9%), neurosurgeons (3.9%), neurologists or headache specialists (14.7%), others (8%). In most cases (85.3%) the final diagnosis was made by a neurologist or headache specialist, and the mean interval between the disease onset and the specialist consultation was 10.8±21.2 months. The correct diagnosis were made after 7.2±12.5 months, being the misdiagnoses at the first consultation in 42.1% of cases. Ten patients underwent erroneous dental extraction. Instrumental and laboratory investigations were carried out in 93.1% of the patients before the final diagnosis of CTN. Conclusions Our results emphasize the need to improve the neurological knowledge in order to promptly recognize the clinical picture of CTN and properly adhere to the specific treatment guidelines.

Background and purpose: Although the main clinical features of COVID-19 infection are pulmonary, several associated neurological signs, symptoms and diseases are emerging. The incidence and characteristics of neurological complications are unclear. For this reason, the European Academy of Neurology (EAN) core COVID-19 Task Force initiated a survey on neurological symptoms observed in patients with COVID-19 infection. Methods: A 17-question online survey was made available on the EAN website and distributed to EAN members and other worldwide physicians starting on 9 April 2020. Results: By 27 April 2020, proper data were collected from 2343 responders (out of 4199), of whom 82.0% were neurologists, mostly from Europe. Most responders (74.7%) consulted patients with COVID-19 mainly in emergency rooms and in COVID-19 units. The majority (67.0%) had evaluated fewer than 10 patients with neurological manifestations of COVID-19 (neuro COVID-19). The most frequently reported neurological findings were headache (61.9%), myalgia (50.4%), anosmia (49.2%), ageusia (39.8%), impaired consciousness (29.3%) and psychomotor agitation (26.7%). Encephalopathy and acute cerebrovascular disorders were reported at 21.0%. Neurological manifestations were generally interpreted as being possibly related to COVID-19; they were most commonly recognized in patients with multiple general symptoms and occurred at any time during infection. Conclusion: Neurologists are currently and actively involved in the management of neurological issues related to the COVID-19 pandemic. This survey justifies setting up a prospective registry to better capture the prevalence of patients with neuro COVID-19, neurological disease characteristics and the contribution of neurological manifestations to outcome. info:eu-repo/semantics/publishedVersion

V razvitem svetu ima 5–10 % prebivalstva nad 65 let demenco, katere pojavnost se vedno narasca. Demence zaradi možganskih žilnih bolezni – vaskularna demenca (VaD) predstavljajo dobro petino vseh vzrokov za demenco. Milejsa oblika je vaskularni kognitivni upad (VaKU). Za postavitev diagnoze VaD je pomembno, da sta upad spoznavnih sposobnosti in možgansko-žilna bolezen jasno casovno povezana ter ni popravljivih vzrokov za kognitivni upad. Pri postavitvi diagnoze uporabimo nevropsiholosko testiranje in slikovne preiskave. Glavni dejavniki tveganja za VaKU in VaD so starost, ateroskleroza, sladkorna bolezen in arterijska hipertenzija, ki sprožijo kaskado dogodkov v patogenezi kognitivne okvare. Ta je zelo raznolika in poteka z ali brez pridruženih nevroloskih simptomov. Klinicna slika je odvisna od podrocja in velikosti možganske spremembe. Pri zdravljenju VaKU in VaD je najbolj pomembna primarna preventiva. Za zdravljenje simptomov VaKU in VaD se uporabljajo enaka zdravila kot za zdravljenje simptomov Alzheimerjeve bolezni. Pomembni sta se rehabilitacija in sekundarna preventiva ponovne možganske kapi. Glavni dejavniki tveganja za VaD so starost, ateroskleroza, sladkorna bolezen in arterijska hipertenzija, ki sprožijo kaskado dogodkov v patogenezi kognitivne okvare. Ta je zelo raznolika in poteka z ali brez pridruženih nevroloskih simptomov. Klinicna slika je odvisna od podrocja in velikosti možganske lezije. Pri zdravljenju VaKU in VaD je najbolj pomembna primarna preventiva. Za zdravljenje simptomov VaKU in VaD se uporabljajo enaka zdravila kot za zdravljenje simptomov Alzheimerjeve bolezni. Pomembni sta se rehabilitacija in sekundarna preventiva ponovne možganske kapi.

Introduction To evaluate an updated algorithm in the detection of urinary tract infection (UTI) prior to high-dose corticosteroid treatment in acute relapses in multiple sclerosis (MS). This updated algorithm aimed to decrease the unnecessary use of antibiotics, whilst maintaining accuracy and safety. Methods Prospective cohort study of 471 consecutive patients with MS relapses in a hospital-based outpatient acute relapse clinic. 172 patients met exclusion criteria, leaving 299 patients for analysis. Patients underwent urine dipstick and were treated for UTI if 2 or more of: nitrites, leukocyte esterase and cloudy urine were positive. Patients with confirmed acute MS relapse were treated with high dose intravenous or oral methylprednisolone. Results Significant bacteriuria (>105 colony forming units/mL) was present in 33 (11%, 95% CI 8-15) patients. The algorithm sensitivity and specificity was 24% and 94% respectively; the negative predictive value was 91%. The overall accuracy of the algorithm was 87%. No adverse sequelae were identified in 25 patients who received high dose methylprednisolone in the presence of an untreated UTI. Conclusion With an improved specificity, this updated algorithm addresses previous issues concerning the unnecessary prescription of antibiotics, whilst improving accuracy and maintaining safety.

The European Association of Young Neurologists and Trainees (EAYNT) is the independent proxy for the interests of junior neurologists in Europe. The main emphasis of the advocacy activities of this legally constituted nonprofit organization lies on the improvement of education, training, and research conditions for up-and-coming neurologists. The organization is also known worldwide as the networking platform for junior neurologists and promoter of cross-cultural understanding.2

The prevalence of both Alzheimer's disease (AD) and vascular dementia (VaD) is increasing with the aging of the population. Studies from the last several years have shown that people with diabetes have an increased risk for dementia and cognitive impairment. Therefore, the authors of this consensus review tried to elaborate on the role of diabetes, especially diabetes type 2 (T2DM) in both AD and VaD. Based on the clinical and experimental work of scientists from 18 countries participating in the International Congress on Vascular Disorders and on literature search using PUBMED, it can be concluded that T2DM is a risk factor for both, AD and VaD, based on a pathology of glucose utilization. This pathology is the consequence of a disturbance of insulin-related mechanisms leading to brain insulin resistance. Although the underlying pathological mechanisms for AD and VaD are different in many aspects, the contribution of T2DM and insulin resistant brain state (IRBS) to cerebrovascular disturbances in both disorders cannot be neglected. Therefore, early diagnosis of metabolic parameters including those relevant for T2DM is required. Moreover, it is possible that therapeutic options utilized today for diabetes treatment may also have an effect on the risk for dementia. T2DM/IRBS contribute to pathological processes in AD and VaD.

Background: The Union Europeenne des Medecins Specialistes Section of Neurology (UEMS SEN)/European Board of Neurology (EBN) examination was launched in 2009 to set standards in knowledge and skills for the next generation of European neurologists. Methods: Stimulated by the low participation at the first examination, we conducted a survey amongst 76 junior colleagues to assess awareness, motivation and obstacles regarding this examination. Results: Remarkably, junior neurologists indicated great interest in the examination and motivation to spend additional preparation time (up to 6 months) with the aim to catch up with European standards and improve the care of patients. However, there are major issues, which detain our colleagues from taking the examination including the amount of the examination fee, the potential lack of language abilities and the varying usage of the recommended books for preparation. Conclusions: This survey highlights the positive attitude of junior neurologists regarding initiatives to improve clinical skills and knowledge. Moreover, this study delineated several factors, which may be considered in the future to increase participation and substantiate the significance of the examination.

At the 67th annual meeting of the American Academy of Neurology (AAN), there was a roundtable discussion with representatives from the International Working Group of Young Neurologist and Trainees–World Federation of Neurology (IWGYNT-WFN), Asia and Oceanian Association of Neurology, Association of South East Asian Nations Neurological Association, European Association of Young Neurologists and Trainees (EAYNT), and the AAN's Consortium of Neurology Residents and Fellows (AAN-CNRF). The purpose of the meeting was to provide updates on the developments affecting young neurologists throughout the world, as well as to discuss current initiatives focused on the improvement of neurology training on the international stage.

Objectives To derive a simple predictive model to guide the use of corticosteroids in patients with relapsing remitting MS suffering an acute relapse. Materials and Methods We analysed individual patient randomised controlled trial data (n=98) using a binary logistic regression model based on age, gender, baseline disability scores [physician-observed: expanded disability status scale (EDSS) and patient reported: multiple sclerosis impact scale 29 (MSIS-29)], and the time intervals between symptom onset or referral and treatment. Results Based on two a priori selected cut-off points (improvement in EDSS ≥ 0.5 and ≥ 1.0), we found that variables which predicted better response to corticosteroids after 6 weeks were younger age and lower MSIS-29 physical score at the time of relapse (model fit 71.2% - 73.1%). Conclusions This pilot study suggests two clinical variables which may predict the majority of the response to corticosteroid treatment in patients undergoing an MS relapse. The study is limited in being able to clearly distinguish factors associated with treatment response or spontaneous recovery and needs to be replicated in a larger prospective study.

Current experimental methods for the study of reaching in the MRI environment do not exactly mimic actual reaching, due to constrains in movement which are imposed by the MRI machine itself. We tested a haptic robot (HR) as such a tool. Positive results would also be promising for combined use of fMRI and EEG to study reaching. Twenty right-handed subjects performed reaching tasks with their right hand with and without the HR. Reaction time, movement time (MT), accuracy, event-related potentials (ERPs) and event-related desynchronisation/synchronisation (ERD/ERS) were studied. Reaction times and accuracies did not differ significantly between the two tasks, while the MT was significantly longer in HR reaching (959 vs. 447 ms). We identified two positive and two negative ERP peaks across all leads in both tasks. The latencies of the P1 and N2 peaks were significantly longer in HR reaching, while there were no significant differences in the P3 and N4 latencies. ERD/ERS topographies were similar between tasks and similar to other reaching studies. Main difference was in ERS rebound which was observed only in actual reaching. Probable reason was significantly larger MT. We found that reaching with the HR engages similar neural structures as in actual reaching. Although there are some constrains, its use may be superior to other techniques used for reaching studies in the MRI environment, where freedom of movement is limited.

To evaluate a dipstick algorithm for urinary tract colonization, prior to high-dose corticosteroid treatment in acute relapses of multiple sclerosis (MS). Methods: Prospective cohort study of 267 consecutive patients with MS relapses requiring corticosteroid treatment in a hospital-based, ambulatory, acute MS relapse clinic. A total of 18 participants met the exclusion criteria, leaving 249 for analysis. Main outcome measures were urinary dipstick sensitivity, specificity, positive predictive value, negative predictive value and safety of antibiotic co-treatment with high-dose corticosteroids. Results: Significant bacteriuria (≥10 5 colonies ml) rate in this population was 11% (95% CI, 7.1-14.9). Specificity and sensitivity of positive leucocyte esterase or nitrite were 78% and 65%. Negative predictive value of urine dipstick was 96%. No clinical adverse events occurred in the 3% (95% CI, 0.9-5.1) of patients with a false-negative dipstick. Eighteen per cent of patients were unnecessarily treated with antibiotics for 48 h. Conclusion: Urinary dipstick testing allows for rapid and safe management of patients suffering from an acute MS relapse. The algorithm is conservative, and future work is needed to reduce the false-positive rate. Click here to view the accompanying paper in this issue. © 2012 The Author(s) European Journal of Neurology © 2012 EFNS.

Objective We compared the temporal dynamics of sensory-motor network during the preparation of actual and imagined reaching and tried to separate ideational-to-motor from pure ideational part of motor program. Methods Ten volunteers reached or imagined reaching with right arm. EEG and reaction time were recorded. Event related potentials were analysed with sLORETA ( http://www.uzh.ch/keyinst/loreta ), comparing preparation of both tasks with respect to their baseline and between them. Results Reaction time for actual reaching was 360 ± 59 ms. There were three key differences between tasks with stronger activity during actual reaching. The first was from 160 ms to 220 ms after target presentation in frontal and parietal regions, the second from 220 ms to 320 ms, in premotor cortex and the third from 320 ms to the reaching onset, mainly in the peri-rolandic region. These brain regions can be crucial for real reaching; processing arm-target integration and muscle activation. Anterior and posterior cingulate cortex were also involved most likely in awareness and controlling of the process. Conclusions and key message Our results suggest the existence of two systems: cortical/integrational and subcortical/controlling system. Observed differences in the first system may be important in separating the ideational from the motor part of the program.

For more than two decades Mini-Mental State Examination (MMSE) has been adapted to the Slovenian language as 'Kratek preizkus spoznavnih sposobnosti' (KPSS). In this study, we evaluated the influences of age and education on the KPSS score, looking for the cut-off point with the optimal ratio of sensitivity (SE) and specificity (SP) to support the use of the KPSS as a screening tool. During the years 2000-03 we examined 258 Slovenian volunteers. Volunteers were divided in two groups based on clinical criteria. A total of 189 were healthy, aged from 45 to 96 years, 69 were demented patients aged from 46 to 91 years, of both sexes, all different levels of education and different degrees of dementia. Median value, SE, SP, positive predictive power and negative predictive power were calculated at cut-off points 23/24, 24/25, 25/26 and 26/27. Younger age and higher education (at least 10 years of education) were each associated with higher KPSS scores. The Slovenian modification of the MMSE demonstrates an optimal cut-off score at 25/26 points for screening dementia in the Slovenian population, due to the best SP (75%)/SE (73%) ratio. The cut-off level 26/27 is recommended for screening highly educated persons.

WCN 2013 No: 2781 Topic: 36 — Other Topic Do young European neurologists know enough to comfortably treat patients with neurotrauma? M. Rakusa, S. Gak, E. Ehler, Y. Alekseenko, J. Opara, D. Muresanu, P. Vos. Department of Neurology, University Medical Centre Maribor, Maribor, Slovenia; Department of Neurology, Sechenov Moscow State Medical University, Moscow, Russia; Department of Neurology, Pardubice Regional Hospital, Pardubice, Czech Republic; Department of Neurology and Neurosurgery, Vitebsk Medical University, Vitebsk, Belarus; ‘Repty’ Rehab Centre, Tarnowskie Gory, Poland; Department of Neurosciences, University of Medicine and Pharmacy ‘Iuliu Hatieganu’, Cluj-Napoca, Romania; Department of Neurology, Slingeland Hospital, Doetinchem, The Netherlands Background: Traumatic brain injury (TBI) and spinal cord injury (SCI) are taking together total (in)direct medical costs among the diseases with the highest annual costs (US-40 billion dollars). Despite the clinical and societal impact of TBI, young neurologists lack the opportunities for formal training in this topic. Objective:Wewanted to evaluatemotivation, knowledge and obstacles in treating patientswith neurotrauma and how to improve the training. Patients and methods: Sixty neurologists were included (33 women) from 10 central and eastern European countries. Survey consisted of fifteen questions testing both knowledge (nine multiple choice questions) and exploring opinions. Results: The mean age of the participants was 30.8 ± 5.6 years. The majority (60%) were resident or consultant (up to 5 years of postacademic experience). Theyhad at least one patientwith traumatic brain, spine or peripheral nerve injury per week in their daily practice (45%). The majority (63%) didn't have any formal training and 82% was certain that the diagnosis and treatment of traumatic brain injured patients would significantly improve, if neurologists become more involved. In practical test no one achieved 0 or 9 points (mean 4.2 ± 1.6).Almost three-quarters (72%) of participants think they don't have enough knowledge to comfortably treat their patients and 98% of them would like to get more practical training mostly in form of teaching sessions (37%) or focused workshops (30%). Conclusions: Our results clearly indicate that young European neurologists need and wish to improve their knowledge in neurotraumatology. Since there is little formal training, teaching/ special sessions/courses at European congresses may be first step to improve education in this field. doi:10.1016/j.jns.2013.07.2441 Abstract — WCN 2013 No: 3003 Topic: 36 — Other Topic Sleep disturbances in systemic lupus erythematosus WCN 2013 No: 3003 Topic: 36 — Other Topic Sleep disturbances in systemic lupus erythematosus C. Falup-Pecurariu, L. Duca, M. Moarcas. Department of Neurology, County Emergency University Hospital, Romania; Faculty of Medicine, Transylvania University, Romania; Department of Immunology, County Emergency University Hospital, Brasov, Romania Knowledge about sleep complaints in systemic lupus erythematosus (SLE) is limited. The aim of this study is to characterize sleep complaints in patients with systemic lupus erythematosus. Method: Prospective study on 42 consecutive patients with SLE (95, 23% women, the mean age 53, 43 ± 22, 69 years). There was a direct interview with a standardized questionnaire which contained demographic characteristics and validated scales. Main outcome measures included: Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), EQ-5D, Fatigue Severity Scale (FSS), Lupus QoL questionnaire, Hospital Anxiety and Depression Scale. The Pittsburgh Sleep Quality Index (PSQI) was used to quantify sleep quality. Poorer sleep quality was correlated with higher PSQI scores. Results: Mean PSQI scores were significantly higher in patients with higher Lupus QoL scores, depression, and fatigue. PSQI had significant correlations with worse QoL (p b 0.01). Patients reported difficulties falling asleep (73.8%), reduced subjective sleep quality (88. 09%), and disturbed sleep (90. 47%). Conclusions: Sleep disturbances are frequently encountered in SLE patients and are significantly linked to quality of life and depression. A better management of sleep alterations could improve these symptoms. doi:10.1016/j.jns.2013.07.2442 Abstract — WCN 2013 No: 3014 Topic: 36 — Other Topic Quality of sleep in medical students WCN 2013 No: 3014 Topic: 36 — Other Topic Quality of sleep in medical students A. Ergin, A. Erdogan, A.I. Bozkurt, N. Ergin. Pamukkale University, Turkey; Denizli Goverment Hospital, Denizli, Turkey Objective: To determine the sleep quality and related factors of the 1st, 4th and 6th year medical students in Pamukkale University, Denizli, Turkey. Methods: This study is a cross-sectional survey. It was carried out after obtaining an IRB permission. The study group included in the 1st, 4th and 6th year medical students. There were 113 (88.9%) participants from the 1st, 62 (73.8%) from the 4th, and 46 (93.8%) from the 6th year in the study. The questionnaire consists of two parts. The first part had questions on the demographic characteristics of the participants. The Family Welfare Scale was used to determine the socio-economic levels. The second part was consisted of the Pittsburgh Sleep Quality Index (PSQI). 1 On behalf of the Scientist Panel on Neurotraumatology, European Federation of Neurological Societies (EFNS) and The European Association of Young Neurologists and Trainees (EAYNT). Abstracts / Journal of the Neurological Sciences 333 (2013) e679–e727 e707

The fine structure of muscle capillaries was analyzed morphometrically in 5 normal controls (41 capillaries), 5 cases of scleroderma (23 capillaries), 4 cases of polymyositis (30 capillaries), 6 cases of dermatomyositis (40 capillaries) and 5 cases of systemic lupus erythematosus (SLE) (50 capillaries). In all 4 connective tissue disorders there was hypertrophy of the endothelial cells and the pericytes. The mean capillary area was also significantly increased in scleroderma, dermatomyositis and SLE but not in polymyositis. In all 4 diseases the number of pinocytotic vesicles per unit endothelial cell area was significantly reduced while the mitochondrial and endoplasmic reticulum fractions of the endothelial area were significantly increased. Replication of the basal lamina around muscle capillaries was observed in 24–74% of the capillaries in inflammatory muscle diseases and in none of the control capillaries. Similar abnormalities of the basement membrane can also occur in other myopathies and in neurogenic atrophies. It is postulated that in the inflammatory myopathies the change is indicative of repeated cycles of capillary degeneration and regeneration and is a primary event rather than being the consequence of muscle fiber necrosis. Endothelial microtubular inclusions were observed in 2 of 6 cases of dermatomyositis and in 2 of 5 cases of SLE. While the inclusions appear to be highly specific for these diseases, their nature and pathogenetic significance remains undetermined.