Mai Yamakawa1, Sally Dwyer2, Xing Song3, Jeffrey Statland1. 1. Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA. 2. The ALS Association Mid-America Chapter, Mission, Kansas, USA. 3. University of Missouri, Health Management and Informatics, School of Medicine, Columbia, Missouri, USA.
Abstract
INTRODUCTION/AIMS: The Midwest has the highest regional prevalence of self-reported amyotrophic lateral sclerosis (ALS) in the United States, but with limited epidemiological studies. We aimed to explore the characteristics of patients with ALS in the Midwest. METHODS: This was a retrospective cohort study of participants with ALS deceased between January, 2010, and September, 2020, registered with the ALS Association Mid-America Chapter. Demographics and clinical variables included gender, race/ethnicity, military status, site of onset, interventions (gastrostomy, non-invasive ventilation, tracheostomy), and visits to ALS Association-registered clinics. Disease characteristics were compared to the National ALS Registry, and survival analysis was performed followed by sample augmentation with historical data to estimate survival with hypothetical censoring. RESULTS: The database included 1447 participants with a mean age at diagnosis of 65.7 ± 11.9 y (>60 y at diagnosis: 72%). The median survival from symptom onset was 28.0 mo (95% confidence limit: 26.3, 29.7); sample augmentation increased this to 41.0 mo (38.5, 43.5). Bulbar onset disease and older age at diagnosis were associated with shorter survival. Participants not followed in ALS-Association registered clinics were more frequently male, had familial onset and tracheostomy. Veterans (N = 298) were older at diagnosis but had similar survival after adjustment for age. DISCUSSIONS: Our cohort had an older age at onset and more frequent bulbar onset than the National ALS Registry, perhaps reflecting ascertainment biases in each registry. Prospective cohort studies with more clinical and functional data are needed to better characterize ALS in Midwest, veterans, and non-clinic populations, and to optimize care.
INTRODUCTION/AIMS: The Midwest has the highest regional prevalence of self-reported amyotrophic lateral sclerosis (ALS) in the United States, but with limited epidemiological studies. We aimed to explore the characteristics of patients with ALS in the Midwest. METHODS: This was a retrospective cohort study of participants with ALS deceased between January, 2010, and September, 2020, registered with the ALS Association Mid-America Chapter. Demographics and clinical variables included gender, race/ethnicity, military status, site of onset, interventions (gastrostomy, non-invasive ventilation, tracheostomy), and visits to ALS Association-registered clinics. Disease characteristics were compared to the National ALS Registry, and survival analysis was performed followed by sample augmentation with historical data to estimate survival with hypothetical censoring. RESULTS: The database included 1447 participants with a mean age at diagnosis of 65.7 ± 11.9 y (>60 y at diagnosis: 72%). The median survival from symptom onset was 28.0 mo (95% confidence limit: 26.3, 29.7); sample augmentation increased this to 41.0 mo (38.5, 43.5). Bulbar onset disease and older age at diagnosis were associated with shorter survival. Participants not followed in ALS-Association registered clinics were more frequently male, had familial onset and tracheostomy. Veterans (N = 298) were older at diagnosis but had similar survival after adjustment for age. DISCUSSIONS: Our cohort had an older age at onset and more frequent bulbar onset than the National ALS Registry, perhaps reflecting ascertainment biases in each registry. Prospective cohort studies with more clinical and functional data are needed to better characterize ALS in Midwest, veterans, and non-clinic populations, and to optimize care.
Authors: Henk-Jan Westeneng; Thomas P A Debray; Anne E Visser; Ruben P A van Eijk; James P K Rooney; Andrea Calvo; Sarah Martin; Christopher J McDermott; Alexander G Thompson; Susana Pinto; Xenia Kobeleva; Angela Rosenbohm; Beatrice Stubendorff; Helma Sommer; Bas M Middelkoop; Annelot M Dekker; Joke J F A van Vugt; Wouter van Rheenen; Alice Vajda; Mark Heverin; Mbombe Kazoka; Hannah Hollinger; Marta Gromicho; Sonja Körner; Thomas M Ringer; Annekathrin Rödiger; Anne Gunkel; Christopher E Shaw; Annelien L Bredenoord; Michael A van Es; Philippe Corcia; Philippe Couratier; Markus Weber; Julian Grosskreutz; Albert C Ludolph; Susanne Petri; Mamede de Carvalho; Philip Van Damme; Kevin Talbot; Martin R Turner; Pamela J Shaw; Ammar Al-Chalabi; Adriano Chiò; Orla Hardiman; Karel G M Moons; Jan H Veldink; Leonard H van den Berg Journal: Lancet Neurol Date: 2018-03-26 Impact factor: 44.182