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ALS Registry

S Fadilah Abdul Wahid, Zhe Kang Law, Nor Azimah Ismail, Raymond Azman Ali, Nai Ming Lai
BACKGROUND: Amyotrophic lateral sclerosis (ALS), which is also known as motor neuron disease (MND) is a fatal disease associated with rapidly progressive disability, for which no definitive treatment as yet exists. Current treatment regimens largely focus on relieving symptoms to improve the quality of life of those affected. Based on data from preclinical studies, cell-based therapy is a promising treatment for ALS/MND. OBJECTIVES: To assess the effects of cell-based therapy for people with ALS/MND, compared with placebo or no additional treatment...
November 8, 2016: Cochrane Database of Systematic Reviews
Andrea Calvo, Cristina Moglia, Christian Lunetta, Kalliopi Marinou, Nicola Ticozzi, Gianluca Drago Ferrante, Carlo Scialo, Gianni Sorarù, Francesca Trojsi, Amelia Conte, Yuri M Falzone, Rosanna Tortelli, Massimo Russo, Adriano Chiò, Valeria Ada Sansone, Gabriele Mora, Vincenzo Silani, Paolo Volanti, Claudia Caponnetto, Giorgia Querin, Maria Rosaria Monsurrò, Mario Sabatelli, Nilo Riva, Giancarlo Logroscino, Sonia Messina, Nicola Fini, Jessica Mandrioli
The aim of this multicenter, retrospective study is to investigate the role of clinical characteristics and therapeutic intervention on ALS prognosis. The study included patients diagnosed from January 1, 2009 to December 31, 2013 in 13 Italian referral centers for ALS located in 10 Italian regions. Caring neurologists collected a detailed phenotypic profile and follow-up data until death into an electronic database. One center collected also data from a population-based registry for ALS. 2648 incident cases were collected...
October 24, 2016: Journal of Neurology
Karin N Wagner, Haikady Nagaraja, Dawn C Allain, Adam Quick, Stephen Kolb, Jennifer Roggenbuck
Although genetic testing for amyotrophic lateral sclerosis (ALS) is widely available, it is unknown what proportion of patients with ALS have access to genetic counseling and testing, and patient attitudes towards ALS genetic testing have not been studied. We conducted a national survey of ALS patients enrolled in the Agency for Toxic Substances and Disease Registry, which consisted of multiple choice questions and two 12 item Likert scale series assessing respondents' experience with and attitude toward genetic testing...
October 20, 2016: Journal of Genetic Counseling
Chia-Chun Tseng, Shun-Jen Chang, Wen-Chan Tsai, Tsan-Teng Ou, Cheng-Chin Wu, Wan-Yu Sung, Ming-Chia Hsieh, Jeng-Hsien Yen
OBJECTIVE: Past studies have shown common pathologic characteristics and shared immunologic features between polymyositis (PM) and amyotrophic lateral sclerosis (ALS). To explore the potential relationship between the two diseases, we performed a nationwide cohort study. METHODS: We identified all newly diagnosed PM from Taiwan's Registry of Catastrophic Illness Database between January 1, 1998 and December 31, 2011. Each PM patient was matched to at most 5 control patients from the National Health Insurance Research Database by sex, age, and entry date...
October 9, 2016: Arthritis Care & Research
Paul Talman, Thi Duong, Steve Vucic, Susan Mathers, Svetha Venkatesh, Robert Henderson, Dominic Rowe, David Schultz, Robert Edis, Merrilee Needham, Richard Macdonnell, Pamela McCombe, Carol Birks, Matthew Kiernan
OBJECTIVE: To capture the clinical patterns, timing of key milestones and survival of patients presenting with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) within Australia. METHODS: Data were prospectively collected and were timed to normal clinical assessments. An initial registration clinical report form (CRF) and subsequent ongoing assessment CRFs were submitted with a completion CRF at the time of death. DESIGN: Prospective observational cohort study...
September 30, 2016: BMJ Open
Pallavi S Mishra-Kalyani, Brent A Johnson, Jonathan D Glass, Qi Long
Clinical disease registries offer a rich collection of valuable patient information but also pose challenges that require special care and attention in statistical analyses. The goal of this paper is to propose a statistical framework that allows for estimating the effect of surgical insertion of a percutaneous endogastrostomy (PEG) tube for patients living with amyotrophic lateral sclerosis (ALS) using data from a clinical registry. Although all ALS patients are informed about PEG, only some patients agree to the procedure which, leads to the potential for selection bias...
September 19, 2016: Scientific Reports
Ola Nakken, Jonas Christoffer Lindstrøm, Ole-Bjørn Tysnes, Trygve Holmøy
Recent studies suggest that the incidence and mortality of amyotrophic lateral sclerosis (ALS) are increasing. Changing environmental factors could influence disease risk differently throughout life span, and also between genders, birth cohorts, and seasons of birth. We aimed at describing long-term ALS mortality trends in Norway between 1951 and 2014 using age-period-cohort analysis. The Norwegian Cause of Death Registry provided ALS mortality data that were age- and sex-adjusted through direct standardization...
December 2016: Journal of Neurology
Caitlin S Jackson-Tarlton, Timothy J Benstead, Steve Doucette
Amyotrophic lateral sclerosis (ALS) is associated with nutritional deficits. Gastrostomy tubes are often inserted in patients with ALS to supplement or replace oral intake. The aim of this study was to better understand the practices of gastrostomy tube insertion in patients with ALS. Pre-collected de-identified data were obtained from the Canadian Neuromuscular Disease Registry (CNDR). Feeding tube status was compared with markers of dysphagia, respiratory compromise, and weight status in both univariate and multivariate analysis by employing odds ratios...
August 18, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Paul Mehta, Wendy Kaye, Leah Bryan, Theodore Larson, Timothy Copeland, Jennifer Wu, Oleg Muravov, Kevin Horton
PROBLEM/CONDITION: Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a progressive and fatal neuromuscular disease for which no cure or viable treatment has been identified. ALS, like most noncommunicable diseases, is not a nationally notifiable disease in the United States. The prevalence of ALS in the United States during 2010-2011 was estimated to be 3.9 cases per 100,000 persons in the general population. Updated prevalence estimates are needed to help monitor disease status, better understand etiology, and identify risk factors for ALS...
2016: MMWR. Surveillance Summaries: Morbidity and Mortality Weekly Report. Surveillance Summaries
Wouter van Rheenen, Aleksey Shatunov, Annelot M Dekker, Russell L McLaughlin, Frank P Diekstra, Sara L Pulit, Rick A A van der Spek, Urmo Võsa, Simone de Jong, Matthew R Robinson, Jian Yang, Isabella Fogh, Perry Tc van Doormaal, Gijs H P Tazelaar, Max Koppers, Anna M Blokhuis, William Sproviero, Ashley R Jones, Kevin P Kenna, Kristel R van Eijk, Oliver Harschnitz, Raymond D Schellevis, William J Brands, Jelena Medic, Androniki Menelaou, Alice Vajda, Nicola Ticozzi, Kuang Lin, Boris Rogelj, Katarina Vrabec, Metka Ravnik-Glavač, Blaž Koritnik, Janez Zidar, Lea Leonardis, Leja Dolenc Grošelj, Stéphanie Millecamps, François Salachas, Vincent Meininger, Mamede de Carvalho, Susana Pinto, Jesus S Mora, Ricardo Rojas-García, Meraida Polak, Siddharthan Chandran, Shuna Colville, Robert Swingler, Karen E Morrison, Pamela J Shaw, John Hardy, Richard W Orrell, Alan Pittman, Katie Sidle, Pietro Fratta, Andrea Malaspina, Simon Topp, Susanne Petri, Susanne Abdulla, Carsten Drepper, Michael Sendtner, Thomas Meyer, Roel A Ophoff, Kim A Staats, Martina Wiedau-Pazos, Catherine Lomen-Hoerth, Vivianna M Van Deerlin, John Q Trojanowski, Lauren Elman, Leo McCluskey, A Nazli Basak, Ceren Tunca, Hamid Hamzeiy, Yesim Parman, Thomas Meitinger, Peter Lichtner, Milena Radivojkov-Blagojevic, Christian R Andres, Cindy Maurel, Gilbert Bensimon, Bernhard Landwehrmeyer, Alexis Brice, Christine A M Payan, Safaa Saker-Delye, Alexandra Dürr, Nicholas W Wood, Lukas Tittmann, Wolfgang Lieb, Andre Franke, Marcella Rietschel, Sven Cichon, Markus M Nöthen, Philippe Amouyel, Christophe Tzourio, Jean-François Dartigues, Andre G Uitterlinden, Fernando Rivadeneira, Karol Estrada, Albert Hofman, Charles Curtis, Hylke M Blauw, Anneke J van der Kooi, Marianne de Visser, An Goris, Markus Weber, Christopher E Shaw, Bradley N Smith, Orietta Pansarasa, Cristina Cereda, Roberto Del Bo, Giacomo P Comi, Sandra D'Alfonso, Cinzia Bertolin, Gianni Sorarù, Letizia Mazzini, Viviana Pensato, Cinzia Gellera, Cinzia Tiloca, Antonia Ratti, Andrea Calvo, Cristina Moglia, Maura Brunetti, Simona Arcuti, Rosa Capozzo, Chiara Zecca, Christian Lunetta, Silvana Penco, Nilo Riva, Alessandro Padovani, Massimiliano Filosto, Bernard Muller, Robbert Jan Stuit, Ian Blair, Katharine Zhang, Emily P McCann, Jennifer A Fifita, Garth A Nicholson, Dominic B Rowe, Roger Pamphlett, Matthew C Kiernan, Julian Grosskreutz, Otto W Witte, Thomas Ringer, Tino Prell, Beatrice Stubendorff, Ingo Kurth, Christian A Hübner, P Nigel Leigh, Federico Casale, Adriano Chio, Ettore Beghi, Elisabetta Pupillo, Rosanna Tortelli, Giancarlo Logroscino, John Powell, Albert C Ludolph, Jochen H Weishaupt, Wim Robberecht, Philip Van Damme, Lude Franke, Tune H Pers, Robert H Brown, Jonathan D Glass, John E Landers, Orla Hardiman, Peter M Andersen, Philippe Corcia, Patrick Vourc'h, Vincenzo Silani, Naomi R Wray, Peter M Visscher, Paul I W de Bakker, Michael A van Es, R Jeroen Pasterkamp, Cathryn M Lewis, Gerome Breen, Ammar Al-Chalabi, Leonard H van den Berg, Jan H Veldink
To elucidate the genetic architecture of amyotrophic lateral sclerosis (ALS) and find associated loci, we assembled a custom imputation reference panel from whole-genome-sequenced patients with ALS and matched controls (n = 1,861). Through imputation and mixed-model association analysis in 12,577 cases and 23,475 controls, combined with 2,579 cases and 2,767 controls in an independent replication cohort, we fine-mapped a new risk locus on chromosome 21 and identified C21orf2 as a gene associated with ALS risk...
September 2016: Nature Genetics
Jonathan A Knibb, Noa Keren, Anna Kulka, P Nigel Leigh, Sarah Martin, Christopher E Shaw, Miho Tsuda, Ammar Al-Chalabi
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a progressive and usually fatal neurodegenerative disease. Survival from diagnosis varies considerably. Several prognostic factors are known, including site of onset (bulbar or limb), age at symptom onset, delay from onset to diagnosis and the use of riluzole and non-invasive ventilation (NIV). Clinicians and patients would benefit from a practical way of using these factors to provide an individualised prognosis. METHODS: 575 consecutive patients with incident ALS from a population-based registry in South-East England register for ALS (SEALS) were studied...
July 4, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
D Ferraro, D Consonni, N Fini, A Fasano, C Del Giovane, J Mandrioli
BACKGROUND AND PURPOSE: To compare two recently developed staging systems for amyotrophic lateral sclerosis (ALS) [King's College and Milano-Torino staging (MITOS) systems] in an incident, population-based cohort of patients with ALS. METHODS: Since 2009, a prospective registry has been recording all incident cases of ALS in the Emilia Romagna region in Italy. For each patient, detailed clinical information, including the ALS functional rating scale score, is collected at each follow-up...
September 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Summer B Gibson, Diana Abbott, James M Farnham, Khanh K Thai, Hailey McLean, Karla P Figueroa, Mark B Bromberg, Stefan M Pulst, Lisa Cannon-Albright
OBJECTIVE: To estimate the risks for cancer (overall and site-specific) in an amyotrophic lateral sclerosis (ALS) cohort. METHODS: In this observational longitudinal study, ALS and cancer cases were identified in a computerized Utah genealogy database (Utah Population Database) linked to a statewide cancer registry and death certificates. Hazard ratios (HRs) were estimated as the ratio of observed to expected number of cancers. Site-specific rates for cancer were estimated within the Utah Population Database; sex, birth year (5-year range), and birth state (Utah or not) cohorts were used to estimate the expected number of cancers among ALS cases...
July 19, 2016: Neurology
Leah Bryan, Wendy Kaye, Vinicius Antao, Paul Mehta, Oleg Muravov, D Kevin Horton
BACKGROUND: The National ALS Registry is made up of two components to capture amyotrophic lateral sclerosis (ALS) cases: national administrative databases (Medicare, Medicaid, Veterans Health Administration and Veterans Benefits Administration) and self-identified cases captured by the Registry's web portal. This study describes self-reported characteristics of U.S. adults with ALS using the data collected by the National ALS Registry web portal risk factor surveys only from October 19, 2010 through December 31, 2013...
2016: PloS One
Tracy L Peters, John D Beard, David M Umbach, Kelli Allen, Jean Keller, Daniela Mariosa, Dale P Sandler, Silke Schmidt, Fang Fang, Weimin Ye, Freya Kamel
Some trace metals may increase risk of amyotrophic lateral sclerosis (ALS), whereas others may be beneficial. Our goal was to examine associations of ALS with blood levels of selenium (Se), zinc (Zn), copper (Cu), and manganese (Mn). We conducted a case-control study of 163 neurologist confirmed patients from the National Registry of Veterans with ALS and 229 frequency-matched veteran controls. We measured metal levels in blood using inductively coupled plasma mass spectrometry and estimated odds ratios (ORs) and 95% confidence intervals (CIs) for associations between ALS and a doubling of metal levels using unconditional logistic regression, adjusting for age, gender, and race/ethnicity...
May 2016: Neurotoxicology
Christopher C D Evans, Ashley Petersen, Eric N Meier, Jason E Buick, Martin Schreiber, Delores Kannas, Michael A Austin
BACKGROUND: Traumatic arrests have historically had poor survival rates. Identifying salvageable patients and ideal management is challenging. We aimed to (1) describe the management and outcomes of prehospital traumatic arrests; (2) determine regional variation in survival; and (3) identify Advanced Life Support (ALS) procedures associated with survival. METHODS: This was a secondary analysis of cases from the Resuscitation Outcomes Consortium Epistry-Trauma and Prospective Observational Prehospital and Hospital Registry for Trauma (PROPHET) registries...
August 2016: Journal of Trauma and Acute Care Surgery
John D Beard, Lawrence S Engel, David B Richardson, Marilie D Gammon, Coleen Baird, David M Umbach, Kelli D Allen, Catherine L Stanwyck, Jean Keller, Dale P Sandler, Silke Schmidt, Freya Kamel
BACKGROUND: Factors underlying a possible excess of amyotrophic lateral sclerosis (ALS) among military veterans remain unidentified. Limitations of previous studies on this topic include reliance on ALS mortality as a surrogate for ALS incidence, low statistical power, and sparse information on military-related factors. OBJECTIVES: We evaluated associations between military-related factors and ALS using data from a case-control study of U.S. military veterans. METHODS: From 2005 to 2010, we identified medical record-confirmed ALS cases via the National Registry of Veterans with ALS and controls via the Veterans Benefits Administration's Beneficiary Identification and Records Locator System database...
May 2016: Environment International
Carlotta Franchi, Elisa Bianchi, Elisabetta Pupillo, Marco Poloni, Alessandro Nobili, Ida Fortino, Angela Bortolotti, Luca Merlino, Ettore Beghi
OBJECTIVE: To investigate the association between angiotensin converting enzyme inhibitors (ACEIs), angiotensin II receptors blockers (ARBs) and motor neuron disease (MND). METHODS: This is a population-based nested case-control study. Data were obtained from a population registry and the administrative database of the Lombardy Region (Northern Italy) from 2000 through 2010. Included were 1,200 patients with newly diagnosed MND/ALS and 120,000 controls, randomly selected from the same population and matched for gender, age and area of residence...
July 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Helen E Stephens, Erik Lehman, Divisha Raheja, Chengwu Yang, Susan Walsh, Zachary Simmons
Complex interactions between pain, depression, and anxiety impact quality of life in patients with ALS. Psychological approaches to pain control may be useful. This study explored the role of self-efficacy in mitigating pain. Individuals registered with the Agency for Toxic Substances and Disease Registry National ALS Registry and who experienced pain were invited to participate in an online survey. Subjects completed the Brief Pain Inventory-Short Form, Hospital Anxiety and Depression Scale, and Chronic Pain Self-Efficacy Scale...
2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Susanna B Park, Steve Vucic, Benjamin C Cheah, Cindy S-Y Lin, Adrienne Kirby, Kristy P Mann, Margie C Zoing, Jennica Winhammar, Matthew C Kiernan
BACKGROUND: Abnormalities in membrane excitability and Na(+) channel function are characteristic of amyotrophic lateral sclerosis (ALS). We aimed to examine the neuroprotective potential, safety and tolerability of the Na(+) channel blocker and membrane stabiliser flecainide in ALS. METHODS: A double-blind, placebo-controlled, randomised clinical trial of flecainide (200 mg/day) for 32-weeks with a 12-week lead-in phase was conducted in participants with probable or definite ALS recruited from multiple Australian centres (ANZCT Registry number ACTRN12608000338369)...
December 2015: EBioMedicine
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