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European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies

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https://www.readbyqxmd.com/read/30317687/endovascular-stroke-treatment-does-not-preclude-high-thrombolysis-rates
#1
Sarina Feda, Omid Nikoubashman, Kolja Schürmann, Oliver Matz, Simone C Tauber, Martin Wiesmann, Jörg B Schulz, Arno Reich
BACKGROUND: In 1995 intravenous recombinant tissue plasminogen activator (IVRTPA) was the first reperfusion therapy to be approved in patients with acute ischemic stroke (AIS). The significance and impact of IVRTPA in times of modern endovascular stroke treatment (EST) was analyzed in a German academic stroke centre. METHODS: A retrospective observational cohort analysis of 1.034 patients with suspected AIS presenting at the emergency department in 2014 was performed...
October 14, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30315714/eq-5d-5l-and-sf-6d-health-utility-index-scores-in-patients-with-myasthenia-gravis
#2
Carolina Barnett, Vera Bril, Ahmed M Bayoumi
INTRODUCTION: Health utilities are a preference-based method of valuing health states that are used in health care research, such as economic evaluations. There are limited health utility valuation data for patients with Myasthenia Gravis OBJECTIVES: To describe health utilities for patients with Myasthenia Gravis and different health states, using the EQ-5D-5L and SF-6D utility instruments, and to explore clinical and demographic determinants of utilities in this population. METHODS: Patients completed the EQ-5D-5L and SF-6D...
October 13, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30315684/neurological-effects-of-gba-mutations
#3
Stephen Mullin, Derralyn Hughes, Atul Mehta, Anthony H V Schapira
The association between Gaucher disease (GD) and PD (PD)has been described for almost two decades. In the biallelic state (homozygous or compound heterozygous) mutations in the glucocerebrosidase gene (GBA) may cause GD, in which glucosylceramide, the sphingolipid substrate of the glucocerebrosidase enzyme (GCase), accumulates in visceral organs leading to a number of clinical phenotypes. In the bi-allelic or heterozygous state, GBA mutations increase the risk for PD. Mutations of the GBA allele are the most significant genetic risk factor for idiopathic PD, found in for 5-20% of idiopathic PD cases, depending on ethnicity...
October 13, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30315672/obinutuzumab-a-new-anti-cd20-antibody-and-chlorambucil-are-active-and-effective-in-anti-mag-antibody-polyneuropathy
#4
C Briani, A Visentin, A Salvalaggio, M Cacciavillani, L Trentin
BACKGROUND: Rituximab, a chimeric anti-CD20 monoclonal antibody (mAb), has been used in polyneuropathy associated with anti-MAG antibody polyneuropathy with controversial results. Herein, we report on two patients with anti-MAG antibody neuropathy and concurrent chronic lymphocytic leukemia (CLL), who dramatically responded to obinutuzumab, a novel glycoengineered humanized anti-CD20 mAb. METHODS: Patient 1 was a 82-year-old man with severe demyelinating sensory-motor neuropathy...
October 13, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30312517/central-nervous-system-involvement-in-late-onset-pompe-disease-lopd-clues-from-neuroimaging-and-neuropsychological-analysis
#5
Olimpia Musumeci, Silvia Marino, Francesca Granata, Rosa Morabito, Lilla Bonanno, Teresa Brizzi, Viviana Lo Buono, Francesco Corallo, Marcello Longo, Antonio Toscano
BACKGROUND: Late Onset Pompe Disease (LOPD) is a rare, multisystem disorder, that is well established to mainly impair skeletal muscle function. Systematic studies exploring brain functions in LOPD are lacking. Aim of this study is to detect morphological and functional brain alterations as well as neuropsychological impairment in LOPD. MATERIALS AND METHODS: We studied 21 patients (10M, 11F, mean-age 49±18,4years), with defined diagnosis of LOPD, divided in two groups: one with presymptomatic hyperCKemia with no muscle weakness and the second with Limb-Girdle Muscle Weakness (LGMW group)...
October 12, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30312502/epilepsy-and-associated-mortality-in-patients-with-multiple-sclerosis
#6
I J Chou, C F Kuo, R Tanasescu, C R Tench, C G Tiley, C S Constantinescu, W P Whitehouse
BACKGROUND: We aimed to determine the prevalence of epilepsy in patients with multiple sclerosis (MS) at diagnosis and the risk of developing epilepsy after the diagnosis of MS, and the relative risk of mortality associated with epilepsy. METHODS: We used the UK Clinical Practice Research Data-link to identify 2,526 incident MS patients and 9,980 age-, sex- and index year-matched non-MS controls from 1997 to 2006. Logistic regression was used to estimate odds ratios (ORs; 95% confidence interval [CI]) for epilepsy and Cox regression was used to estimate hazard ratios (HRs; 95% CI) for epilepsy and mortality...
October 12, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30308706/disease-progression-in-blepharospasm-a-5-year-longitudinal-study
#7
Gina Ferrazzano, Antonella Conte, Angelo Gigante, Giovanni Defazio, Alfredo Berardelli, Giovanni Fabbrini
BACKGROUND: Clinical manifestation of dystonic spasms in blepharospasm (BSP) patients may be heterogeneous. Whether the varying phenomenology of eyelid spasms becomes manifest sequentially during the course of the disease or aggregates in separate clusters according to different disease courses is still unclear. For this purpose, we longitudinally evaluated the clinical features in BSP patients over a 5-year period, and also tested the blink reflex recovery cycle in a subgroup of BSP patients...
October 11, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30308699/a-randomised-controlled-trial-of-a-walking-training-with-simultaneous-cognitive-demand-dual-task-in-chronic-stroke
#8
Daan Meester, Emad Al-Yahya, Andrea Dennis, Johnny Collett, Derick T Wade, Martin Ovington, Francesca Liu, Meaney Andy, Cockburn Janet, Heidi Johansen-Berg, Helen Dawes
OBJECTIVE: To evaluate the tolerability of, adherence to and efficacy of a community walking training programme with simultaneous cognitive demand (dual-task) compared to a control walking training programme without cognitive distraction. METHODS: Adult stroke survivors, at least 6 months after stroke with a visibly obvious gait abnormality or reduced two-minute walk distance were included into a 2-arm parallel randomized controlled trial of complex intervention with blinded assessments...
October 11, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30308696/early-thrombosis-prophylaxis-with-enoxaparin-is-not-associated-with-hematoma-expansion-in-patients-with-spontaneous-intracerebral-hemorrhage
#9
Bogdan Ianosi, Max Gaasch, Verena Rass, Lukas Huber, Werner Hackl, Mario Kofler, Alois Josef Schiefecker, Alberto Addis, Ronny Beer, Paul Rhomberg, Bettina Pfausler, Claudius Thomé, Elske Ammenwerth, Raimund Helbok
BACKGROUND: Early pharmacological deep vein thrombosis (DVT) prophylaxis is recommended by guidelines, but rarely started within 48 hours. We aimed to analyze the effect of early (within 48h) versus late (>48h) DVT prophylaxis on hematoma expansion (HE) and outcome in patients with spontaneous intracerebral hemorrhage (ICH). METHODS: We analyzed 134 consecutive patients admitted to a tertiary neuro-intensive care unit with diagnosed spontaneous ICH, obtained informed consent and without previous anticoagulation, a severe coagulopathy, hematoma evacuation, early withdrawal of therapy or ineligibility for DVT prophylaxis according to our institutional protocol...
October 11, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30300463/management-of-psychogenic-nonepileptic-seizures-pnes-a-multidisciplinary-approach
#10
Sara Gasparini, Ettore Beghi, Edoardo Ferlazzo, Massimiliano Beghi, Vincenzo Belcastro, Klaus P Biermann, Gabriella Bottini, Giuseppe Capovilla, Rosa A Cervellione, Vittoria Cianci, Giangennaro Coppola, Cesare M Cornaggia, Pasquale De Fazio, Salvatore De Masi, Giovambattista De Sarro, Maurizio Elia, Giuseppe Erba, Lucia Fusco, Antonio Gambardella, Valentina Gentile, Anna T Giallonardo, Renzo Guerrini, Francesca Ingravallo, Alfonso Iudice, Angelo Labate, Ersilia Lucenteforte, Adriana Magaudda, Laura Mumoli, Costanza Papagno, Giovanni B Pesce, Eugenio Pucci, Pietrantonio Ricci, Antonino Romeo, Rui Quintas, Chiara Sueri, Giovanna Vitaliti, Riccardo Zoia, Umberto Aguglia
BACKGROUND: The International League Against Epilepsy (ILAE) proposed a diagnostic scheme for psychogenic nonepileptic seizures (PNES). The debate on ethical aspects of the diagnostic procedures is ongoing, the treatment is not standardized, and management might differ according to the age groups. OBJECTIVE: To reach an expert and stakeholder consensus on PNES management. METHODS: A board comprising adult and child neurologists, neuropsychologists, psychiatrists, pharmacologists, experts in forensic medicine and bioethics as well as patients' representatives was formed...
October 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30300462/adiponectin-profile-at-baseline-is-correlated-to-progression-and-severity-of-multiple-sclerosis
#11
E Signoriello, G Lus, R Polito, S Casertano, O Scudiero, M Coletta, M L Monaco, F Rossi, E Nigro, A Daniele
BACKGROUND AND PURPOSE: Adiponectin is a relevant cytokine linking energy metabolism and immune system. After being assembled, adiponectin circulates as oligomers of different molecular weight, LMW, MMW and HMW. These latter have the most potent biological effects. Multiple Sclerosis (MS) is an autoimmune disease of human central nervous system. The aim of this study was to characterize the expression levels of both total Adiponectin and its oligomerization state in the serum from 99 MS patients at the baseline (i...
October 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30300458/the-high-burden-of-neurological-disease-in-the-older-general-population-results-from-the-canadian-longitudinal-study-on-aging
#12
Christina Wolfson, Seyed-Mohammad Fereshtehnejad, Romain Pasquet, Ron Postuma, Mark R Keezer
BACKGROUND: Our objective was to study the association between the presence of a neurological disease and the comorbidity burden, as well as healthcare utilization. METHODS: Using baseline data from the Canadian Longitudinal Study on Aging (CLSA), we examined the burden of five neurological conditions. The CLSA is a population-based study of approximately 50,000 individuals, aged 45 to 85 years at baseline. We used multivariable Poisson regression to identify correlates of comorbidity burden and healthcare utilization...
October 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30300457/multiple-sclerosis-a-review
#13
Ruth Dobson, Gavin Giovannoni
Multiple sclerosis (MS) is the commonest non-traumatic disabling disease to affect young adults. The incidence of MS is increasing worldwide, together with the socioeconomic impact of disease. The underlying cause of MS, and mechanisms behind this increase remain opaque, although complex gene-environment interactions almost certainly play a significant role. The epidemiology of MS indicates that low vitamin D, smoking, childhood obesity and infection with the Epstein-Barr virus are likely to play a role in disease development...
October 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30300455/disturbed-sleep-in-cluster-headache-is-not-the-result-of-transient-processes-associated-with-the-cluster-period
#14
Nunu Laura T Lund, Agneta Henriette Snoer, Anja Sofie Petersen, Rasmus Paulin Beske, Poul Jørgen Jennum, Rigmor Højland Jensen, Mads Christian J Barloese
BACKGROUND: Cluster headache (CH) is characterized by severe, unilateral attacks of pain and a high nocturnal attack burden. It remains unknown if perturbations of sleep are solely present during the CH bout. Therefore, we aimed to investigate differences in sleep between the bout and remission period in episodic CH (eCH) patients, secondly to compare patients in the two phases with controls. METHODS: eCH patients, aged 18-65 years, diagnosed according to the International Classification of Headache Disorders 2nd edition, were admitted for polysomnography at the Danish Center for Sleep Medicine, in bout and in remission...
October 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30298612/impact-of-the-systematic-use-of-the-gugging-swallowing-screen-in-acute-ischemic-stroke-patients
#15
Margarida Lopes, Eduardo Freitas, Marta Oliveira, Ester Dantas, Nídia Azevedo, Pedro Rodrigues, João Pinho, Carla Ferreira
BACKGROUND: Post-stroke dysphagia occurs in up to three fourths of patients and is associated with a higher risk of respiratory infections and poor outcome. Systematic screening of dysphagia in the acute stroke unit (ASU) is essential to identify patients at risk of aspiration and to provide dietary recommendations. Our study aimed to assess the impact of the systematic application of the Gugging Swallowing Screen (GUSS) in acute ischemic stroke patients. METHODS: Retrospective study of consecutive acute ischemic stroke patients admitted to an ASU in two time periods: pre-GUSS, February/2014-July/2015, when the 10mL water swallowing test was systematically administered; GUSS, August/2015-October/2016, when the GUSS test was systematically administered...
October 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30298572/anti-inflammatory-disease-modifying-treatment-and-disability-progression-in-primary-progressive-multiple-sclerosis-a-cohort-study
#16
Johannes Lorscheider, Jens Kuhle, Guillermo Izquierdo, Alessandra Lugaresi, Eva Havrdova, Dana Horakova, Raymond Hupperts, Pierre Duquette, Marc Girard, Alexandre Prat, François Grand'Maison, Pierre Grammond, Patrizia Sola, Diana Ferraro, Maria Trojano, Cristina Ramo-Tello, Jeanette Lechner-Scott, Eugenio Pucci, Claudio Solaro, Mark Slee, Vincent Van Pesch, José Luis Sánchez Menoyo, Anneke van der Walt, Helmut Butzkueven, Ludwig Kappos, Tomas Kalincik
BACKGROUND: Treatment options in primary progressive multiple sclerosis (PPMS) are scarce and, with the exception of ocrelizumab, anti-inflammatory agents failed to show efficacy in ameliorating disability progression. OBJECTIVE: To investigate a potential effect of anti-inflammatory disease modifying treatment on disability outcomes in PPMS. METHODS: Using MSBase, a large, international, observational database, we identified patients with PPMS who were either never treated or treated with a disease modifying agent...
October 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30298540/prognostic-significance-of-genetic-biomarkers-in-idh-wild-type-lower-grade-glioma-the-need-to-further-stratify-this-tumor-enity-a-meta-analysis
#17
Huy Gia Vuong, Thao T K Tran, Hanh T T Ngo, Thong Quang Pham, Tadao Nakazawa, Kar-Ming Fung, Lewis Hassell, Ryohei Katoh, Tetsuo Kondo
BACKGROUND: The clinical outcomes of IDH-wild type (IDH-wt) lower-grade glioma (LGG) have been the subject of debate for some time. In this meta-analysis, we aimed to assess the prognostic values of several known genetic markers (eg, TERT promoter mutation, H3F3A mutation, CDKN2A loss) in this tumor group. METHODS: Four electronic databases including PubMed, Scopus, Web of Science and Virtual Health Library were searched for relevant articles. Pooled hazard ratio (HR) and corresponding 95% confidence interval (CI) for overall survival (OS) were calculated using random effect model weighted by inverse variance method...
October 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30295399/natural-history-of-a-cohort-of-abcd1-variants-female-carriers
#18
Tommaso Schirinzi, Gessica Vasco, Chiara Aiello, Cristiano Rizzo, Andrea Sancesario, Alberto Romano, Martina Favetta, Maurizio Petrarca, Laura Paone, Enrico Castelli, Enrico Silvio Bertini, Marco Cappa
BACKGROUND: The therapeutic scenario of X-linked adrenoleukodystrophy (X-ALD) is rapidly changing. Whereas the disease is well characterized in males, the condition remains to be fully clarified in women carrying ABCD1 variants. Specifically, data on clinical progression are needed, in order to recommend any appropriate management. Objective of this study is to outline the natural history of a cohort of untreated ABCD1 heterozygous female carries. METHODS: Longitudinal data from a single-center population of 60 carriers were retrospectively reviewed...
October 8, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30295375/postural-instability-during-attacks-of-migraine-without-aura
#19
Evangelos Anagnostou, Stathis Gerakoulis, Panagiota Voskou, Evangelia Kararizou
BACKGROUND AND PURPOSE: Migraine has long been associated with unsteadiness and dizziness but postural control has not been studied in the ictal state. Here, we measured the stability of upright stance during migraine attacks. METHODS: Static balance was assessed prospectively in migraine patients (n=30) during quiet stance for 40 s on a posturographic force platform. Recordings were performed both ictally and in the pain-free interval. Subjects were assessed under 4 different conditions yielding different visual and proprioceptive feedback environments...
October 8, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30266037/a-cost-utility-analysis-of-decompressive-hemicraniectomy-vs-medical-treatment-in-the-management-of-space-occupying-brain-oedema-post-middle-cerebral-artery-infarction
#20
Anudeep Bhattacharyya, Anas Tahir, Abhinaya Chandrashekar, Shivani Vasisht, Luke Stinson, Joyce Omatseye
BACKGROUND AND PURPOSE: Data from randomly controlled trials has indicated that a decompressive hemicraniectomy is more clinically effective than medical treatment in the management of space occupying brain oedema post middle cerebral artery infarction. This economic evaluation compares the impact of the two options in the UK. No recent study has conducted an economic evaluation on this topic for the UK. METHOD: A cost utility analysis over a time period of one year was used, measuring benefits in terms of Quality Adjusted Life Years and costs in pound sterling, discounted to 2015 prices...
September 28, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
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