Amyotrophic Lateral Sclerosis

Data Standards

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

• CDEs — Classified as Core, Supplemental–Highly Recommended, Supplemental, or Exploratory

• CRF Modules — Template forms that logically organize CDEs for data collection

• Guidance Documents — Provide further information about the CDEs

• Instrument Notice of Copyright (NOC) Documents — Include pertinent information on recommended instruments (Instrument Notice of Copyright Information)

Amyotrophic Lateral Sclerosis Start-up Resource Listing: All Core and Supplemental–Highly Recommended CDEs recommended for Amyotrophic Lateral Sclerosis study start-up

Amyotrophic Lateral Sclerosis Highlight Summary: Overview of all Amyotrophic Lateral Sclerosis-specific CDE recommendations as they appear on the website

Click Expand All to view the CDEs associated with the CRF modules, organized by domain and subdomain.

NIH Resources

NIH Resources
CRF Module/Guidance	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/Guidance	CDEs
Demographics	CDE Details
General Core	CDE Details

Social Status
CRF Module/Guidance	CDEs
Social Status	CDE Details

Participant History and Family History

General Health History
CRF Module/Guidance	CDEs
Family History	CDE Details
Medical History	CDE Details

Disease/Injury Related Events

Classification
CRF Module/Guidance	CDEs
Symptom, Sign, Diagnosis Criteria	CDE Details
Genetics	CDE Details

Assessments and Examinations

Laboratory Tests and Biospecimens/Biomarkers
CRF Module/Guidance	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/Guidance	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/Guidance	CDEs
Electrophysiology	CDE Details

Physical/Neurological Examination
CRF Module/Guidance	CDEs
Neuromuscular Examination	CDE Details
Physical Examination	CDE Details

Vital Signs and Other Body Measures
CRF Module/Guidance	CDEs
Vital Signs	CDE Details

Treatment/Intervention Data

Drugs
CRF Module/Guidance	CDEs
Prior and Concomitant Medications	CDE Details

Outcomes and End Points

Muscle Strength Testing
CRF Module/Guidance	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/Guidance	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/Guidance	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/Guidance	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/Guidance	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/Guidance	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/Guidance	CDEs
Caregiver Burden Scale (CGBS)	CDE Details
Clinical Global Impression Scale	N/A
Zarit Burden Interview (ZBI)	N/A

Upper Motor Neuron Signs/Neuromuscular Excitability
CRF Module/Guidance	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 https://cssrs.columbia.edu/).

Instrument Notice of Copyright Information

The NINDS CDE Team does not post proprietary instruments/scales recommended by the CDE Working Groups on this website. This includes, but is not limited to, copyrighted or trademarked instruments/scales. Information about recommended instruments can be found in the Notice of Copyright (NOC) documents under ‘CRF Module/Guideline’ on each disorder’s data standards page. For any questions regarding these instruments/scales please contact the corresponding owner/author. The NINDS CDE Team is not responsible for the availability or content of these external sites, nor does the NINDS CDE Team endorse, warrant or guarantee the products, services or information described or offered at these other internet sites.

Overview

The first set of Common Data Elements (CDEs) for Amyotrophic Lateral Sclerosis was developed in 2011.

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

Roster

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

Universitats - 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

Federation des Maladies du Systeme Nerveux, Hopital de la Salpetriere, 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

NINDS CDE Program Officer, National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland(NIH), Bethesda, MD, USA
Sherita Ala'i Hansen, MS (Until 2018)

The Emmes Company, LLC, Rockville, Maryland
Robin Feldman, BS, MBA

The Emmes Company, LLC, Rockville, Maryland
Aria Lans, MPH

The Emmes Company, LLC, Rockville, Maryland
Amanda Suller, BS (Until 2015)

The Emmes Company, LLC, Rockville, Maryland
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

Publications

Publications related to the ALS CDE Effort

Sherman AV, Gubitz AK, Al-Chalabi A, Bedlack R, Berry J, Conwit R, Harris BT, Horton DK, Kaufmann P, Leitner ML, Miller R, Shefner J, Vonsattel JP, Mitsumoto H.2013. Infrastructure resources for clinical research in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2013 May; 14 Suppl 1:53-61. PubMed PMID: 23678880.

Presentations related to other CDE Effort

February 2010 – Frontotemporal Dementias Common Data Element Workshop, February 2-3, 2010, , Miami, Florida. The National Institute of Neurological Disorders and Stroke (NINDS) Common Data Elements (CDE). Authors: Margaret Sutherland (NINDS)

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