Amyotrophic Lateral Sclerosis

Data Standards

The National Institute of Neurological Disorders and Stroke (NINDS) and other Federal agencies and international organizations have the common mission of developing data standards for clinical research. Through the efforts of subject-specific working groups, topic-driven data elements have been created. The first set of Common Data Elements (CDEs) for Amyotrophic Lateral Sclerosis was developed in 2011. The Core data elements to be used by an investigator when beginning a research study in this disease/disorder are listed in the Start-up Resource Listing.

Many of the CDEs will overlap across study types, which allows for comparisons and meta-analysis across studies. Consistency of the data elements and the CDE formats is kept in order to ensure the ability to transfer critical medical information electronically from one center to another. This consistency also allows for continuity across different disease areas. The goals of the NINDS CDE initiative are to increase the efficiency and effectiveness of clinical research studies and clinical treatment, increase data quality, facilitate data sharing, and help educate new clinical investigators.

Organized by domains and sub-domains, often used in clinical studies, data standards include:

Please view additional tabs for more information about the development of these CDEs. An overview of all Amyotrophic Lateral Sclerosis (ALS) CDE recommendations can be found in the ALS CDE Highlight Summary document. For your reference, a zip file containing all the current ALS CDE template CRF modules can be downloaded below.

The outline that follows includes all the CDEs associated with the CRF modules, organized by domain and sub-domain.

NIH Resources
NIH Resources
CRF Module/Guideline CDEs
National Institutes of Health (NIH) Toolbox N/A
Patient-Reported Outcomes Measurement Information System (PROMIS) N/A
Quality of Life in Neurological Disorders (Neuro-QOL) N/A
Participant Characteristics
Demographics
CRF Module/Guideline CDEs
Demographics CDE Details
General Core CDE Details
Social Status
CRF Module/Guideline CDEs
Social Status CDE Details
Participant History and Family History
General Health History
CRF Module/Guideline CDEs
Family History CDE Details
Medical History CDE Details
Disease/Injury Related Events
Classification
CRF Module/Guideline CDEs
Symptom, Sign, Diagnosis Criteria CDE Details
Genetics CDE Details
Assessments and Examinations
Laboratory Tests and Biospecimens/Biomarkers
CRF Module/Guideline CDEs
Biomarkers Other Collection Information Guidelines N/A
Collection of Fluid: Blood Guidelines N/A
Collection of Fluid: CSF Guidelines N/A
Collection of Fluid: Plasma Guidelines N/A
Collection of Fluid: Urine Guidelines N/A
Fasting Information CDE Details
Laboratory Tests CDE Details
Resting Energy Expenditure CDE Details
Tissue Based Marker Collection CDE Details
Imaging Diagnostics
CRF Module/Guideline CDEs
Brain Perfusion Magnetic Resonance Imaging CDE Details
Diffusion Tensor Imaging (DTI) CDE Details
Functional Magnetic Resonance Imaging (fMRI) CDE Details
Magnetic Resonance Imaging (MRI) CDE Details
Magnetic Resonance Spectroscopy CDE Details
Non-Imaging Diagnostics
CRF Module/Guideline CDEs
Electrophysiology CDE Details
Physical/Neurological Examination
CRF Module/Guideline CDEs
Neuromuscular Examination CDE Details
Physical Examination CDE Details
Vital Signs and Other Body Measures
CRF Module/Guideline CDEs
Vital Signs CDE Details
Treatment/Intervention Data
Drugs
CRF Module/Guideline CDEs
Prior and Concomitant Medications CDE Details
Outcomes and End Points
Muscle Strength Testing
CRF Module/Guideline CDEs
Pinch Strength N/A
Fixed Dynamometry CDE Details
Grip Strength CDE Details
Grip Strength Fatigue CDE Details
Hand Held Dynamometry CDE Details
Manual Muscle Testing-Using the Medical Research Council Muscle Grading Scale CDE Details
Cognitive
CRF Module/Guideline CDEs
Cognitive Recommendations Guidelines for Use N/A
ALS Cognitive Behavioral Screen N/A
ALS Depression Inventory (ADI-12) CDE Details
Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire (ALS-FTD-Q) N/A
Beck Depression Inventory II (BDI-II) CDE Details
Cambridge Behavioral Inventory - Revised CDE Details
Center for Neurologic Study - Lability Scale for pseudobulbar affect (PBA) CDE Details
Edinburgh Cognitive Behavioral ALS Screen (ECAS) N/A
Emotional Lability Questionnaire: Person with MND (ELQ-MND) CDE Details
Emotional Lability Questionnaire: Relatives and Carers (ELQ-R and C) CDE Details
Frontal Behavioral Inventory (FBI) CDE Details
Frontal Behavioral Inventory - ALS Version (FBI-ALS) CDE Details
Frontal Behavioral Inventory - Modified by Heidler Gary N/A
Frontal Systems Behavioral Scale (FrSBe) CDE Details
Geriatric Depression Scale (GDS) CDE Details
Hamilton Depression Rating Scale (HDRS) CDE Details
Hospital Anxiety and Depression Scale (HADS) N/A
Motor Neuron Disease Behavioural Instrument (MiND-B) N/A
Neuropsychiatric Inventory - Clinician Version (NPI-C) N/A
Neuropsychiatric Inventory Questionnaire (NPI-Q) N/A
Penn State Screen of Frontal and Temporal Dysfunction Syndromes N/A
UCSF Screening Exam N/A
Written Verbal Fluency Test CDE Details
Functional Outcomes
CRF Module/Guideline CDEs
6 Minute Walk Test N/A
ALS Functional Rating Scale - Revised (ALSFRS-R) CDE Details
ALS Swallowing Severity Scale (ALSSS) CDE Details
Barthel Index N/A
Center for Neurologic Study - Bulbar Function Scale (CNS-BFS) CDE Details
Checklist for Individual Strength - Fatigue (CIS) CDE Details
Clinical Milestones/Events CDE Details
Death Report N/A
Dynamic Gait Index (DGI) CDE Details
Edmonton Symptom Assessment System (ESAS) CDE Details
EuroQoL-5 Dimension Questionnaire (EQ-5D) CDE Details
Fatigue Severity Scale (FSS) CDE Details
Madrid Quantitative Neuromuscular Assessment (MAQUINA) CDE Details
Modified Fatigue Impact Scale (MFIS) CDE Details
Performance-Oriented Mobility Assessment (POMA) CDE Details
Schwab and England Activities of Daily Living Scale (ADL-Scale) CDE Details
Sensory Organization Test (SOT) N/A
Timed 25-Foot Walk (T25-FW) CDE Details
Timed Up and Go (TUG) N/A
Summary of All Outcome Recommendations
CRF Module/Guideline CDEs
ALS CDE Instrument Recommendations by Outcome Domain and Classification N/A
ALS Outcomes Recommendations Guidelines for Use N/A
Quality of Life/Patient Reported Outcomes
CRF Module/Guideline CDEs
Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) N/A
EuroQoL-VAS N/A
McGill Quality of Life Questionnaire (MQOL) CDE Details
Quality of Life in Neurological Disorders (Neuro-QOL) N/A
Quality of Life in Swallowing Disorders (SWAL-QOL) CDE Details
Schedule for the Evaluation of Individual Quality of Life - Direct Weighting (SEIQoL-DW) N/A
Short Form 36-Item Health Survey (SF-36) CDE Details
Sickness Impact Profile (SIP) N/A
Pulmonary Function Testing/Respiratory Status
CRF Module/Guideline CDEs
Baseline Dyspnea Index (BDI) N/A
Borg Dyspnea Scale N/A
Myocardial Oxygen Consumption, Gas Exchange and Nocturnal Oximetry CDE Details
Pulmonary Function CDE Details
Transition Dyspnea Index (TDI) N/A
Subjective Assessments/Patient and Caregiver Reported Outcomes
CRF Module/Guideline CDEs
Caregiver Burden Scale (CGBS) CDE Details
Clinical Global Impression Scale N/A
Zarit Caregiver Burden Scale N/A
Upper Motor Neuron Signs/Neuromuscular Excitability
CRF Module/Guideline CDEs
Modified Ashworth Scale for Grading Spasticity CDE Details
NINDS Myotatic Reflex Scale N/A
Tardieu Scale CDE Details

Prospective Assessments for Suicidal Ideation and Behavior

Investigators should review the FDA’s "Guidance for Industry: Suicidal Ideation and Behavior: Prospective Assessment of Occurrence in Clinical Trials” for the most up-to-date information about suicidal ideation and behavior. One scale that FDA suggests is the Columbia Suicide Severity Rating Scale (C-SSRS) (available at http://www.cssrs.columbia.edu).

Accessibility Statement

The NINDS CDE Team has requested, and in many cases received, permission to post proprietary instruments/scales recommended by the CDE Working Groups to this site. When permission is granted, the documents are posted in the exact format they were received in order to maintain the validity of the instrument. If approval is not granted for the instrument to be reproduced on this site, with the author/copyright holder’s permission, a link is posted to an external Web site where users can get more information about the instrument. If you have difficulty accessing either the proprietary instruments/scales or the external links, please contact the NINDS Office of Clinical Research at carolina.mendoza-puccini@nih.gov.

Overview

To develop the Amyotrophic Lateral Sclerosis (ALS) CDEs, the ALS CDE Working Group divided into subgroups to focus on identifying and defining data elements in the domains of:

  • Biomarkers and Imaging
  • Clinical Research/ Quality of Life
  • Cognitive/ Frontotemporal Dementia
  • Genetics

History and Acknowledgements

The National Institute of Neurological Disorders and Stroke (NINDS) has formed an Oversight Committee to direct the updating and further development of Amyotrophic Lateral Sclerosis CDEs. The Amyotrophic Lateral Sclerosis CDE Committee is supported by the NINDS CDE Team.

Previously, the Amyotrophic Lateral Sclerosis CDE Committee was currently comprised of four different subgroups. Chairs were appointed to lead the overall Working Group and the individual subgroups. The Working Group members actively developed the CDEs for their specific subgroups and also had an opportunity to review and comment on the recommendations of the other subgroups.

The Working Group members served on either one or two subgroups. The subgroups began meeting by teleconference in November 2010 and continued to hold calls every four to six weeks through spring 2011 to define the CDEs for their domains and to recommend standardized, validated instruments for ALS research. The Amyotrophic Lateral Sclerosis CDE Oversight Committee was created in 2013.

Complete Amyotrophic Lateral Sclerosis CDE Roster

Oversight Committee Members

  • Melanie Leitner, PhD
    Prize4Life, Inc, Berkeley, California
    Chair
  • Christin Bermudez Andrews, PhD
    University of Miami, Miami, Florida
  • James D. Berry, MD, MPH
    Massachusetts General Hospital, Boston, Massachusetts
  • Lucie Bruijn, PhD, MBA
    Dementia Discovery Fund, Boston, Massachusetts
  • Adriano Chio, MD
    University of Torino, AOU San Giovanni Battista, Turin, Italy
  • Valerie Cwik, MD
    Muscular Dystrophy Association, Tucson, Arizona
  • Murray Grossman, MD. EdD
    University of Pennsylvania, Philadelphia, Pennsylvania
  • Amanda Haidet-Phillips, PhD
    AveXis, Bannockburn, Illinois
  • Omar Jawdat, MD
    University of Kansas Medical Center, Kansas City, Kansas
  • Edward Kasarskis, MD, PhD
    University of Kentucky College of Medicine, Veterans Affairs Medical Center, Lexington, Kentucky
  • Prof. Dr. Med. Albert Ludolph
    Universit?ts- und Rehabilitationskliniken Ulm, Ulm, Germany
  • Alex Sherman, MS
    Neurological Clinical Research Institute, Massachusetts General Hospital, Boston, Massachusetts
  • Bryan Traynor, MB, MD, PhD, MMSc, MRCPI
    National Institute on Aging (NIA), National Institutes of Health (NIH), Bethesda, Maryland
  • Martin Turner, PhD
    Oxford University, Oxford, United Kingdom
  • Susan Woolley-Levine, PhD
    Sutter Pacific Medical Center, San Francisco, California
  • Jennifer Murphy, PhD
    University of California, San Francisco, San Francisco, California (Ad Hoc Member)

Working Group Co-Chairs

  • Lucie Bruijn, PhD
    ALS Association, Washington, District of Columbia
  • James Caress, MD
    Wake Forest University, Winston-Salem, North Carolina

Biomarkers/ Imaging

  • Robert Bowser, PhD
    Barrow Neurological Institute, Phoenix, Arizona
    Chair
  • Michael Benatar, MS, DPhil
    University of Miami School of Medicine, Miami, Florida
  • Jonathan Glass, MD
    Emory University School of Medicine, Atlanta, Georgia
  • Varan Govind, PhD
    University of Miami School of Medicine, Miami, Florida
  • Vincent Meininger, MD, PhD
    F?d?ration des Maladies du Syst?me Nerveux, H?pital de la Salp?tri?re, Paris, France
  • Hiroshi Mitsumoto, MD
    Columbia University Motor Neuron Center, New York, New York
  • Jeremy Shefner, MD, PhD
    Upstate Medical University, Syracuse, New York
  • Alexander Sherman, MD
    Massachusetts General Hospital, Boston, Massachussetts
  • Martin Turner, PhD
    Oxford University, Oxford, United Kingdom

Clinical Research/ Quality of Life (QOL)

  • Merit Cudkowicz, MD, MSc
    Massachusetts General Hospital, Boston, Massachusetts
    Chair
  • Stanley Appel, MD
    Houston Methodist Hospital, Houston, Texas
  • Richard Barohn, MD
    University of Kansas Medical Center, Kansas City, Kansas
  • Michael Benatar, MS, DPhil
    University of Miami School of Medicine, Miami, Florida
  • Frank Bennett, PhD
    Ionis Pharmaceuticals, Carlsbad, California
  • Kathie Bishop, PhD
    Tioga Pharmaceuticals, San Diego, California
  • Benjamin Rix Brooks, MD
    Carolinas Healthcare System, Charlotte, North Carolina
  • Jesse Cedarbaum, MD
    Bristol-Myers Squibb, New York City, New York
  • Valerie Cwik, MD
    Muscular Dystrophy Association, Tucson, Arizona
  • Julaine Florence, PT, MHS, DPT
    Washington University School of Medicine, St. Louis, Missouri
  • Orla Hardiman, MD
    Trinity College Institute of Neuroscience, Dublin, Ireland
  • Laura Herbelin, CCRP
    University of Kansas Medical Center, Kansas City, Kansas
  • Edward Kasarskis, MD, PhD
    University of Kentucky College of Medicine, Lexington, Kentucky
  • Wendy King, PT
    Ohio State University, Columbus, Ohio
  • Zachary Simmons, MD
    Penn State Hershey Medical Center, Hershey, Pennsylvania
  • Richard Smith, MD
    University of California San Diego, Center for Neurological Study, San Diego, California
  • Eric Sorensen, MD
    Mayo Clinical Rochester, Great Lakes ALS, Rochester, Minnesotta
  • Hong Yu, MPH
    Massachusetts General Hospital, Boston, Massachusetts

Cognitive/ Frontotemporal Dementia (FTD)

  • Catherine Lomen-Hoerth, MD, PhD
    University of California San Francisco, San Francisco, California
    Co-Chair
  • Zachary Simmons, MD
    Penn State Hershey Medical Center, Hershey, Pennsylvania
    Co-Chair
  • Sharon Abrahams, PhD
    University of Edinburgh, Edinburgh, United Kingdom
  • Richard Buchsbaum
    Columbia University Mailman School of Public Health, New York City, New York
  • Lora Clawson, MSN, CRNP
    Johns Hopkins Medicine, Baltimore, Maryland
  • Laura Goldstein, PhD
    King's College London, London, United Kingdom
  • Murray Grossman, MD, EdD
    University of Pennsylvania, Philadelphia, Pennsylvania
  • Dan Moore, PhD
    Univeristy of California San Francisco, California Pacific Medical Center, San Francisco, California
  • Jennifer Murphy, PhD
    University of Califonia San Francisco, San Francisco, California
  • Seamus Thompson, PhD
    Columbia University Mailman School of Public Health, New York, New York
  • Susan Woolley, PhD
    Forbes Norris ALS Research Center, San Francisco, California

Genetics

  • Bryan Traynor, MB, MD, PhD, MMSc, MRCPI
    National Institute on Aging (NIA), National Institutes of Health (NIH), Bethesda, Maryland
    Chair
  • Robert Brown, MD
    University of Massachusetts Medical School, Worcester, Massachusetts
  • Adriano Chio, MD
    University of Torino, AOU San Giovanni Battista, Torino, Italy
  • Vivian Drory, MD
    Tel-Aviv Sourasky Medical Center, Tel Aviv, Israel
  • Karen Morrison, PhD
    University of Birmingham, Birmingham, United Kingdom
  • Rosa Rademakers, PhD
    Mayo Clinic College of Medicine, Rochester, Minnesotta
  • Kevin Talbot, MD, DPhil
    Oxford University, Oxford, United Kingdom
  • Leonard van den Berg, MD, PhD
    University Medical Center Urecht, Utrecht, Netherlands

NINDS CDE Team

  • Robin Conwit, MD
    National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland
  • Amelie Gubitz, PhD
    National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland
  • M. Carolina Mendoza-Puccini, MD
    Scientific Program Specialist, National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland(NIH), Bethesda, MD, USA
  • Sherita Ala'i Hansen, MS
    The Emmes Corporation
  • Robin Feldman, BS, MBA
    The Emmes Corporation
  • Aria Lans, MPH
    The Emmes Corporation
  • Amanda Suller, BS
    The Emmes Corporation
  • Lisa Hunegs, MSW, MPH
    KAI Research, Inc. (An Altarum Company), Rockville, Maryland
  • Kristy Miller, MPH
    KAI Research, Inc. (An Altarum Company), Rockville, Maryland
  • Megan Schmidt
    KAI Research, Inc. (An Altarum Company), Rockville, Maryland

Updates

Please see attached revision history document. Please contact NINDSCDE@emmes.com if you require further information or have any questions about the revision history.

Amyotrophic Lateral Sclerosis CDE Revision History