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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/29044904/diabetes-mellitus-and-als-time-to-bridge-the-gap-between-the-bench-and-the-bed-side
#1
Ali Jawaid, Anooshay Abid, Paul E Schulz
The article of D'Ovidio et al.(1) in the current issue of European Journal of Neurology is an important addition to the growing body of evidence supporting a disease-modifying effect of metabolic disorders in neurodegenerative diseases (NDDs). Eight years ago, we first showed a protective effect of type 2 diabetes mellitus (T2DM) in ALS. Using a very large clinical cohort (n=2,371), we found that pre-morbid T2DM was associated with a reduced risk for ALS and a 4 year delay in onset.(2) Since then, a number of studies have independently confirmed a protective effect of T2DM in ALS...
October 17, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29034555/advances-and-insights-into-neurological-practice-2016-17
#2
Anthony H V Schapira
Papers published by the European Journal of Neurology reflect the broad interest of practicing neurologists in advances in the aetiology, diagnosis and management of neurological disorders. As a general journal, the proportion of papers in the different subject areas reasonably reflects the case load of a practising neurologist. Stroke represents the largest proportion of papers published, including those on pathophysiology (1-23), acute stroke management (24-47) and the outcome of patients who have suffered stroke (48-72)...
October 16, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29029362/altered-tdp-43-dependent-splicing-in-hspb8-related-distal-hereditary-motor-neuropathy-and-myofibrillar-myopathy
#3
Andrea Cortese, Matilde Laurà, Carlo Casali, Ichizo Nishino, Yukiko K Hayashi, Stefania Magri, Franco Taroni, Cristiana Stuani, Paola Saveri, Maurizio Moggio, Michela Ripolone, A Prelle, C Pisciotta, A Sagnelli, Anna Pichiecchio, Mary M Reilly, Emanuele Buratti, Davide Pareyson
INTRODUCTION: Mutations in the small heat shock protein 22 gene (HSPB8) have been associated with Charcot-Marie-Tooth type 2L, distal hereditary motor neuropathy (dHMN) type IIa and, more recently, distal myopathy/myofibrillar myopathy (MFM) with protein aggregates and TDP-43 inclusions. OBJECTIVE: To report a novel family with HSPB8 (K141E) -related dHMN/MFM and to investigate, in a patient muscle biopsy, whether the presence of protein aggregates was paralleled by altered TDP-43 function...
October 13, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29024243/low-clinical-conversion-rate-in-clinically-isolated-syndrome-cis-patients-diagnostic-benefit-of-mcdonald-2010-criteria
#4
Sina Cathérine Rosenkranz, Barbara Kaulen, Anneke Neuhaus, Susanne Siemonsen, Sascha Köpke, Martin Daumer, Jan-Patrick Stellmann, Christoph Heesen
BACKGROUND: New diagnostic criteria of Multiple Sclerosis (MS) increase the number of patients being diagnosed with MS while a substantial part might not convert to clinically definite MS (CDMS). OBJECTIVE: Diagnostic accuracy of the McDonald 2005 and 2010 criteria for conversion to CDMS was evaluated in an unselected cohort of patients in whom a MS diagnostic workup was decided. METHODS: We analyzed clinical, MRI and CSF data in all patients who presented with symptoms suspicious for MS at the University based MS outpatient clinic between 2006 and 2010 (n=165)...
October 11, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28963765/antiepileptic-drug-related-neuropsychiatric-adverse-events-in-brain-tumor-related-epilepsy-levetiracetam-front-and-center
#5
EDITORIAL
A M Feyissa
No abstract text is available yet for this article.
September 30, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28960663/high-dose-transdermal-nicotine-in-parkinson-s-disease-patients-a-randomized-open-label-blinded-endpoint-evaluation-phase-2-study
#6
Gabriel Villafane, Claire Thiriez, Etienne Audureau, Céline Straczek, Philippe Kerschen, Florence Cormier-Dequaire, Axel Van Der Gucht, Jean-Marc Gurruchaga, Morgane Quéré-Carne, Eva Evangelista, Muriel Paul, Gilles Defer, Philippe Damier, Philippe Remy, Emmanuel Itti, Gilles Fénelon
BACKGROUND: Studies of the effects of nicotine on motor symptoms in Parkinson's disease (PD) brought out discordant results. The aim of the present study was to evaluate the efficacy and safety of high doses of transdermal nicotine on motor symptoms in PD. METHODS: Forty PD patients were randomly assigned to a treated and untreated arm in an open-label study. Treated patients received increasing doses of nicotine to reach 90 mg/day by 11 weeks. This dosage was maintained for 28 weeks (W39), and then reduced over six weeks...
September 28, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28940973/prediction-of-cerebral-infarction-and-patient-outcome-in-aneurysmal-subarachnoid-hemorrhage-comparison-of-new-and-established-radiographic-clinical-and-combined-scores
#7
Nora F Dengler, Jenny Sommerfeld, Dominik Diesing, Peter Vajkoczy, Stefan Wolf
BACKGROUND: There are numerous grading scales to describe the severity of aneurysmal subarachnoid hemorrhage (aSAH) and to predict outcome. Historically, outcome measures are heterogenous and comparability of grading scales is therefore limited. We designed this study to compare radiographic, clinical and combined grading systems in aSAH. METHODS: Data of 423 consecutive patients with aSAH were analyzed. Modified Fisher, Barrow Neurological Institute (BNI), Hunt and Hess, World Federations of Neurosurgeons (WFNS), VASOGRADE and HAIR scores were calculated from clinical and radiographic data or the combination of both...
September 23, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28940855/long-term-effects-of-cognitive-rehabilitation-on-brain-functional-outcome-and-cognition-in-parkinson-s-disease
#8
María Díez-Cirarda, Natalia Ojeda, Javier Peña, Alberto Cabrera-Zubizarreta, Olaia Lucas-Jiménez, Juan Carlos Gómez-Esteban, Maria Ángeles Gómez-Beldarrain, Naroa Ibarretxe-Bilbao
BACKGROUND: Cognitive rehabilitation has demonstrated efficacy in producing short-term cognitive and brain changes in Parkinson's disease (PD) patients. To date, no study has assessed the long-term effects of cognitive rehabilitation using neuroimaging techniques in PD. The aim was to assess the longitudinal effects of a 3-month cognitive rehabilitation program evaluating the cognitive, behavioural and neuroimaging changes after 18 months. METHODS: Fifteen PD patients underwent a cognitive, behavioural and neuroimaging assessment at pre-treatment (T0 ), post-treatment (T1 ) and after 18-months (T2 )...
September 23, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28941002/an-assessment-of-movement-disorder-society-task-force-diagnostic-criteria-for-mild-cognitive-impairment-in-parkinson-s-disease
#9
Pınar Uysal-Cantürk, Haşmet A Hanağasi, Başar Bilgiç, Hakan Gürvit, Murat Emre
BACKGROUND: Cognitive impairment is one of the most disabling non-motor symptoms of Parkinson disease (PD). Mild cognitive impairment (MCI) constitutes a major risk for the development of Parkinson's disease Dementia in the course of the disease. A Movement Disorder Society (MDS) Task Force proposed diagnostic criteria for mild cognitive impairment in Parkinson's disease (PD-MCI), comprising two operational levels: Level I and Level II. METHOD: The objective of our study was to test the accuracy of Level I versus Level II diagnostic criteria...
September 20, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28940893/l-dopa-induced-dyskinesias-motor-fluctuations-and-health-related-quality-of-life-the-copark-survey
#10
Santiago Perez-Lloret, Laurence Negre-Pages, Philippe Damier, Arnaud Delval, Pascal Derkinderen, Alain Destée, Wassilios G Meissner, François Tison, Olivier Rascol
BACKGROUND AND OBJECTIVES: Studies assessing the correlations between L-DOPA-induced dyskinesias (LIDs) and motor fluctuations with Health-related Quality of Life (HRQoL) in Parkinson's Disease (PD) have yielded conflicting results. This study aimed at assessing the relationship between LIDs and motor fluctuations with HRQoL in PD patients, and to assess the relative contribution of their severity and duration in a large sample of PD patients. METHODS: 683 PD patients of the COPARK survey were evaluated...
September 20, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28940741/hypermetabolism-appears-to-be-an-adverse-prognostic-biomarker-in-als-a-potential-for-therapeutic-intervention
#11
Steve Vucic
Hypermetabolism has been identified as a potential pathophysiological process in approximately 50-60% of amyotrophic lateral sclerosis (ALS) patients and is characterised by an increase in the resting energy expenditure (REE), a measure of total daily energy expenditure [1, 2]. The finding of hypermetabolism in ALS is unexpected given that ALS patients exhibit lower weight and free fat mass (FFM), both of which would lead to hyopmetabolism [3]. This article is protected by copyright. All rights reserved.
September 20, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28940704/hypermetabolism-is-a-deleterious-prognostic-factor-in-patients-with-amyotrophic-lateral-sclerosis
#12
Pierre Jésus, Philippe Fayemendy, Marie Nicol, Géraldine Lautrette, Huguette Sourisseau, Pierre-Marie Preux, Jean-Claude Desport, Benoit Marin, Philippe Couratier
The aim of this study was to investigate in ALS patients in order to determine their nutritional, neurological and respiratory parameters and their survival according to the metabolic level. METHODS: Nutritional assessment included resting energy expenditure (REE) measured by indirect calorimetry (hypermetabolism if REE variation [ΔREE] > 10%) and fat mass (FM) using impedancemetry. Neurological assessment included ALSFRS-R score. Survival analysis used the Kaplan-Meier method and the multivariate Cox model...
September 20, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28926165/pre-stroke-physical-activity-is-associated-with-fewer-post-stroke-complications-lower-mortality-and-a-better-long-term-outcome
#13
Chi-Pang Wen, Chung-Hsiang Liu, Jiann-Shing Jeng, Shih-Pin Hsu, Chih-Hung Chen, Li-Ming Lien, An-Chih Chen, Jiunn-Tay Lee, Ping-Kun Chen, Chih-Shan Hsu, Chang-Ming Chern, Chien-Chung Chen, Ming-Chin Hsu, Kang Lu, Han-Jung Chen, Hao-Kuang Wang, Chih-Hsin Muo, Chung Y Hsu
BACKGROUND AND PURPOSE: Physical activity is associated with a reduced incidence of first-time stroke; however, few studies have examined the effect of pre-stroke physical activity on post-stroke complications and clinical outcomes. METHODS: A total of 39,835 cases of stroke registered in the nationwide stroke registry system of Taiwan between 2006 and 2009 were analyzed according to 5 levels of severity as determined by NIHSS (National Institutes of Health Stroke Scale) score upon hospital admission...
September 19, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28926157/evaluation-of-a-palliative-and-hospice-care-telephone-hotline-for-severely-affected-multiple-sclerosis-patients-and-their-caregivers
#14
Julia Strupp, Bernadette Groebe, Andrea Knies, Michaela Mai, Raymond Voltz, Heidrun Golla
BACKGROUND: Palliative and hospice care (PHC) still highly focus on cancer patients. OBJECTIVES: To connect severely affected Multiple Sclerosis (MS) patients and caregivers to PHC, a nationwide hotline was implemented facilitating access to PHC. METHODS: The hotline was designed in cooperation with the German Multiple Sclerosis Society. Self-disclosed information given by callers was documented using case report forms supplemented by personal notes...
September 19, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28926154/brain-functional-connectome-abnormalities-in-als-are-associated-with-disability-and-cortical-hyperexcitability
#15
Nimeshan Geevasinga, Mayuresh S Korgaonkar, Parvathi Menon, Mehdi Van den Bos, Lavier Gomes, Sheryl Foster, Matthew C Kiernan, Steve Vucic
OBJECTIVE: The present study utilized a multimodality approach encompassing connectome network combined with brain volume analysis, and assessment of cortical excitability to provide novel insights into amyotrophic lateral sclerosis (ALS) pathogenesis. METHODS: Magnetic resonance images (MRI) were acquired using a 3.0 Tesla GE Signa HDx scanner, using an 8-channel head coil. MR images for the resting state scan were acquired using echo planar imaging MR sequence, acquiring 40 contiguous axial/oblique slices...
September 19, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28921834/the-role-of-pre-morbid-diabetes-on-developing-amyotrophic-lateral-sclerosis
#16
Fabrizio D'Ovidio, Angelo d'Errico, Paolo Carnà, Andrea Calvo, Giuseppe Costa, Adriano Chiò
BACKGROUND: The literature on the association between diabetes and Amyotrophic Lateral Sclerosis (ALS) counts a limited number of studies. This cohort study was developed in order to assess the role of diabetes on the risk of developing ALS. METHODS: The study population was represented by all residents in Turin (Italy) at the beginning of 1996, already present at 1991 Census, older than 14 years (n=727,977), followed up for ALS occurrence from 1998 to 2014. Presence of diabetes at baseline or during the follow-up was ascertained through two Piedmont regional sources: the Diabetes Registry and the ATC Drugs Prescriptions Archive...
September 18, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28921817/diagnostic-criteria-for-adult-onset-leukoencephalopathy-with-axonal-spheroids-and-pigmented-glia-due-to-csf1r-mutation
#17
Takuya Konno, Kunihiro Yoshida, Ikuko Mizuta, Toshiki Mizuno, Toshitaka Kawarai, Masayoshi Tada, Hiroaki Nozaki, Shu-Ichi Ikeda, Osamu Onodera, Zbigniew K Wszolek, Takeshi Ikeuchi
BACKGROUND: To establish and validate diagnostic criteria for adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) due to colony stimulating factor 1 receptor (CSF1R) mutation. METHODS: We developed diagnostic criteria for ALSP based on a recent analysis of the clinical characteristics of ALSP. These criteria provide "probable" and "possible" designations for patients who do not have a genetic diagnosis. To verify its sensitivity and specificity, we retrospectively applied our criteria to 83 ALSP cases who had CSF1R mutations (24 of these were analyzed at our institutions, and the others were identified from the literature), 53 cases who had CSF1R mutation-negative leukoencephalopathies, and 32 cases who had cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with NOTCH3 mutations...
September 18, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28906581/is-there-a-benefit-of-mechanical-thrombectomy-in-patients-with-large-stroke-dwi-aspects-%C3%A2-5
#18
P-F Manceau, S Soize, M Gawlitza, G Fabre, S Bakchine, C Durot, I Serre, G-E Metaxas, L Pierot
BACKGROUND AND PURPOSE: Whether to withhold mechanical thrombectomy when the diffusion-weighted imaging (DWI) lesion exceeds a given volume is undetermined. Our aim was to identify markers that will help to select patients with large DWI lesions [DWI-Alberta Stroke Program Early Computed Tomography Score (DWI-ASPECTS) ≤ 5] that may benefit from thrombectomy. METHODS: From May 2010 to November 2016, 82 acute ischaemic stroke patients with DWI-ASPECTS ≤5 (43 men, 64...
September 14, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28906578/ultra-early-hematoma-growth-in-antithrombotic-pretreated-patients-with-intracerebral-hemorrhage
#19
Jaume Roquer, Rosa María Vivanco-Hidalgo, Jaume Capellades, Angel Ois, Elisa Cuadrado-Godia, Eva Giralt-Steinhauer, Carolina Soriano-Tárraga, Marina Mola-Caminal, Maria Serra-Martínez, Carla Avellaneda-Gómez, Jordi Jiménez-Conde, Ana Rodríguez-Campello
BACKGROUND: Patients with acute intracerebral hemorrhage (ICH) pretreated with antithrombotic drugs may have increased early hematoma growth, which would increase mortality risk. We analyzed the effect of antiplatelet (AP) and vitamin-K antagonist (VKA) pretreatment on ultra-early hematoma growth (uHG), and its relationship with mortality in patients with acute supratentorial ICH. METHODS: This is an observational retrospective study of a prospective register of 197 ICH patients with first computed tomography (CT) scan taken less than 6 hours from ICH symptoms onset...
September 14, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28906575/prevalence-correlates-and-impact-of-pain-and-cramps-in-anti-mag-neuropathy-a-multicentre-european-study
#20
Yusuf A Rajabally, Emilien Delmont, Fu Liong Hiew, Anne-Catherine Aubé-Nathier, Aude-Marie Grapperon, Julien Cassereau, Shahram Attarian
BACKGROUND: The frequency of pain and cramps is uncertain in anti-MAG (Anti-myelin associated glycoprotein antibody) neuropathy. Whether these symptoms may affect function/quality of life is unknown. METHODS: We performed a cross-sectional study of the prevalence, correlates and impact of pain, pain subtypes and cramps, their severity, frequency and anatomical distribution, in 55 clinically stable patients with anti-MAG neuropathy. RESULTS: Pain of any type was reported by 80% of subjects...
September 14, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
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