Clinical characteristics of a large cohort of US participants enrolled in the National Amyotrophic Lateral Sclerosis (ALS) Registry, 2010-2015 [Supporting dataset]

Details

• Journal Article:
  Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
• Personal Author:
  Horton, Kevin ; Mehta, Paul ; Oskarsson, BjÖrn ; Raymond, Jaime
• Corporate Authors:
  United States. Agency for Toxic Substances and Disease Registry ; United States. Agency for Toxic Substances and Disease Registry. Division of Toxicology and Human Health Sciences ; Mayo Clinic ; United States. Agency for Toxic Substances and Disease Registry. Office of Innovation and Analytics
• Description:
  Background: Amyotrophic lateral sclerosis (ALS) is a progressive fatal disease with a varying range of clinical characteristics. Objective: To describe the clinical characteristics in a large cohort of ALS participants enrolled in the National ALS Registry. Methods: Data from ALS participants who completed the Registry's online clinical survey module during 2010-2015 were analyzed to determine characteristics, such as site of onset, associated symptoms, time of symptom onset to diagnosis, time of diagnosis to hospice referral, and pharmacological and non-pharmacological interventions. Results: Of the 1758 participants who completed the survey, 60.9% were male, 62.1% were 50-69 years old, and 95.5% white. Approximately, 72.0% reported initial limb weakness onset of disease, followed by bulbar (22.1%), and trunk/global onset (6.1%). Other symptoms ever experienced included cramps (56.7%), fasciculations (56.3%), and dysarthria (33.0%). The median time between an increase of muscle cramps until an ALS diagnosis was 12 months; limb onset participants had cramps longer preceding diagnosis versus those with bulbar onset. The most frequent interventions used included riluzole (48.3% currently using), wheelchairs/scooters (32.8%), and noninvasive breathing equipment (30.0%). Participants with trunk/global onset were referred to hospice almost four times earlier than others. Conclusions: These data show how ALS clinical characteristics differ widely in a large cohort of participants preceding diagnosis and reflect variations in disease onset, progression, and prognosis. Better characterization of symptom onset may assist clinicians in diagnosing ALS sooner, which could lead to earlier therapeutic interventions.
• Subjects:
  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis
  • Cohort Studies
  • Female
  • Humans
  • Male
  • Middle Aged
  More +
• Keywords:
  • Cancer
  • Computational Biology
  • Medicine
  • Neuroscience
  • Science Policy
• DOI:
  https://dx.doi.org/10.6084/m9.figshare.8187920
• Landing Page:
  https://tandf.figshare.com/articles/Clinical_characteristics_of_a_large_cohort_of_US_participants_enrolled_in_the_National_Amyotrophic_Lateral_Sclerosis_ALS_Registry_2010_2015/8187920
• Dataset Download URL:
  https://tandf.figshare.com/ndownloader/articles/8187920/versions/1
• Identifier:
  https://dx.doi.org/10.6084/m9.figshare.8187920
• Format:
  XLSX Spreadsheet file
• Modified:
  2019/07/08
• Document Type:
  Dataset
• Funding:
  CDC/National ALS Registry
• Corporate as Subject:
  National ALS Registry
• Place as Subject:
  United States
• Media Type:
  application/vnd.ms-excel
• Described By Type:
  HTML Repository Page
• License:
  https://creativecommons.org/licenses/by/4.0/
• Rights:
  Public Domain
• Described By:
  https://stacks.cdc.gov/view/cdc/126617
• CIO:
  Office of Innovation and Analytics
• Division:
  Division of Toxicology and Human Health Sciences
• Contact Point:
  BjÖrn Oskarsson
• Pages in Document:
  1 spreadsheet file
• Email:
  oskarsson.bjorn@mayo.edu
• Download URL:
  https://stacks.cdc.gov/view/cdc/126617/cdc_126617_DS1.zip
• File Type:
  [ZIP - 2.66 KB]
• Collection(s):
  CDC Public Access
• Main Document Checksum:
  urn:sha-512:74dbfcdc51ca0a267a40f7b3d43aeec2c094678e80c6e560889d1fc810fb56b423847f7ef4d1a1cffb270be5c9afab46e04ff365d45ec49b42fb845816fd783e