Huntington’s Disease

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)

Huntington's Disease Start-up Resource Listing: All Core and Supplemental–Highly Recommended CDEs recommended for Huntington's Disease study start-up

Huntington's Disease Highlight Summary: Overview of all Huntington's Disease-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
General Core	CDE Details
Demographics	CDE Details

Social Status
CRF Module/Guidance	CDEs
Social Status	CDE Details

Participant History and Family History

Epidemiology/Environmental History
CRF Module/Guidance	CDEs
Epi/Environment Guidelines for CDE Use	N/A
Food Frequency Questionnaire (FFQ)	CDE Details
Godin Leisure-Time Exercise Questionnaire	CDE Details
History of Early Exposure to Huntington's disease	CDE Details
Infection History	CDE Details
National Institutes of Health Diet History Questionnaire (DHQ)	N/A
Non-prescribed Drug Use Questionnaire	CDE Details
PD DOC Mini Environmental Risk Questionnaire for Parkinson's Disease Patients Baseline (PD DOC MERQ-PD-B)	CDE Details
Risk Factor Questionnaire (RFQ) - All	CDE Details
Risk Factor Questionnaire (RFQ) - Epidemiology of Dystonia	CDE Details
Risk Factor Questionnaire (RFQ) - Female Health History	CDE Details
Risk Factor Questionnaire (RFQ) - NSAIDS and Calcium Channel Blockers (CCB)	CDE Details
Risk Factor Questionnaire (RFQ) - Occupation and Employment Status	CDE Details
Risk Factor Questionnaire (RFQ) - Residential History	CDE Details
Risk Factor Questionnaire (RFQ) - Toxicants	CDE Details
Risk Factor Questionnaire (RFQ-U) - Height and Weight	CDE Details
Risk Factor Questionnaire (RFQ-U) - Pesticides (Home)	CDE Details
Risk Factor Questionnaire (RFQ-U) - Pesticides (Work)	CDE Details
Risk Factor Questionnaire (RFQ-U) - Smoking and Tobacco	CDE Details
The Medical Research Council Physical Activity Assessment	N/A
World Health Organization Disability Assessment Schedule (WHODAS)	N/A

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

Disease/Injury Related Events

History of Disease/Injury Event
CRF Module/Guidance	CDEs
Medical History of Huntington's Disease	CDE Details
Ohio State University TBI Identification Method Short Form	CDE Details

Assessments and Examinations

Laboratory Tests and Biospecimens/Biomarkers
CRF Module/Guidance	CDEs
Blood Collection and Processing Guidelines	N/A
CSF Collection and Processing Guidelines	N/A
DNA Elements - Information from the Laboratory	CDE Details
DNA Elements - Information from the Study Investigator	CDE Details
DNA Elements - Participant/Subject Information	CDE Details
NINDS Repository Huntington's Disease Data Elements	N/A
Other Biological Specimens Collection Guidelines	N/A
Solid Tissue Collection Guidelines	N/A
Urine Collection and Processing Guidelines	N/A

Other Clinical Data
CRF Module/Guidance	CDEs
HD Brain Processing Guidelines	N/A
Neuropathology Data Form	CDE Details
Preparation of Brains for Stereological Evaluation Guidelines	N/A

Imaging Diagnostics
CRF Module/Guidance	CDEs
Magnetic Resonance (MR) Localization Imaging	CDE Details

Outcomes and End Points

Quality of Life/Patient Reported Outcomes
CRF Module/Guidance	CDEs
HDQLIFE	N/A
EuroQoL-5 Dimension Questionnaire (EQ-5D)	CDE Details
Huntington's Disease Health Related Quality of Life (HDQoL)	N/A
Quality of Life in Neurological Disorders (Neuro-QOL)	N/A
Short Form 36-Item Health Survey (SF-36)	CDE Details

Motor Function
CRF Module/Guidance	CDEs
Huntington's Disease Dysphagia Scale	N/A
Unified Huntington's Disease Rating Scale (UHDRS)	N/A

Emotional/Behavioral
CRF Module/Guidance	CDEs
Behavior/Psychiatry Guidelines for CDE Use	N/A
Apathy Evaluation Scale	N/A
Apathy Scale (AS)	CDE Details
Cambridge Brain Repair Centre (BRC) HD Sleep Questionnaire	CDE Details
Concise Health Risk Tracking (CHRT) (CHRT-C) (CHRT-SR)	N/A
Florida Obsessive Compulsive Inventory (FOCI)	N/A
Frontal Systems Behavioral Scale (FrSBe)	CDE Details
Hospital Anxiety and Depression Scale (HADS)	N/A
Irritability Scale	N/A
Padua Inventory - Washington State University Revision	N/A
Pittsburgh Sleep Quality Index (PSQI)	CDE Details
Problem Behaviours Assessment HD - Short Version (PBA-s)	CDE Details
Scale for Outcomes in Parkinson's Disease-Sleep (SCOPA-Sleep)	CDE Details

Cognitive
CRF Module/Guidance	CDEs
Cognitive Summary Table and Guidelines for CDE Use	N/A
Circle Tracing	N/A
Cued Movement Sequencing	N/A
Delis-Kaplan Executive Function System (D-KEFS)	N/A
Emotional Recognition	N/A
Hopkins Verbal Learning Test - Revised (HVLT-R)	CDE Details
Map Search Task	N/A
Mental Rotation	N/A
Montreal Cognitive Assessment (MoCA)	N/A
Phonemic Verbal Fluency (PVF)	N/A
Self-Paced Tapping	N/A
Simple and Two-Choice Reaction Time	N/A
Speeded Tapping Test	N/A
Spot the Change	N/A
Stroop Test	CDE Details
Symbol Digit Modalities Test (SDMT)	N/A
Timed Up and Go (TUG)	N/A
Trail Making Test	N/A
Written Verbal Fluency Test	CDE Details

Functional Outcomes
CRF Module/Guidance	CDEs
Functional Outcomes/Patient Reported Outcomes Guidelines for CDE Use	N/A
PROMIS Pain Interference Short Form	N/A
Sickness Impact Profile (SIP)	N/A

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/Guidance’ 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 Huntington’s Disease was developed in 2011.

To develop the Huntington’s disease (HD) CDEs, the Huntington’s disease CDE Working Group divided into subgroups to focus on identifying and defining data elements in the domains of:

Motor
Imaging Biomarkers
Biochemical Markers
Genetics

Epidemiology/Environment
Functional Outcomes/Patient Reported Outcomes
Behavior/ Psychiatry

Pathology
Operations
Cognitive
Scale Metrics and Statistics

Roster

The Huntington’s Disease CDE Working Group was comprised of eleven 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 HD CDE Working Group was supported by the NINDS CDE Team. The complete HD CDE Working Group roster and the rosters by Subgroup are shown below.

Complete Huntington’s Disease CDE Working Group Roster

The Working Group members served on one subgroup. The subgroups began meeting by teleconference in March 2011 and continued to hold calls every four to six weeks through fall 2011 to define the CDEs for their domains and to recommend standardized, validated instruments for HD research. The Huntington Disease CDE Oversight Committee was created in 2016.

Oversight Committee Members

Karen Anderson, MD

Georgetown University Medical Center, Washington, District of Columbia

Co-Chair
Ira Shoulson, MD

University of Rochester, Rochester, New York; Georgetown University, Washington, District of Columbia; Grey Matter Technologies LLC, Sarasota, Florida

Co-Chair
Kevin Biglan, MD, MPH

Eli Lilly and Company, Indianapolis, Indiana
Noelle Carlozzi, PhD

University of Michigan, Ann Arbor, Michigan
Blair Leavitt, MD

The University of British Columbia, BC Children's Hospital Research Institute, British Columbia, Canada
Kathleen Shannon, MD

University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin
Glenn Stebbins, PhD

Rush University, Rush Medical College, Chicago, Illinois
Julie Stout, PhD

Monash University, Melbourne, Australia

Working Group Co-Chairs

Ira Shoulson, MD

University of Rochester, Rochester, New York; Georgetown University, Washington, District of Columbia
Julie Stout, PhD

Monash University, Melbourne, Australia

Behavior/Psychiatry

Karen Anderson, MD

University of Maryland School of Medicine, Baltimore, Maryland

Chair
David Craufurd, MB, BS, MSc, FRCPsych

The University of Manchester School of Medicine, Manchester, United Kingdom
Mark Groves, MD

Beth Israel Medical Center, Phillips Ambulatory Care Center, New York, New York
Erik van Duijn, MD, PhD

Leiden University Medical Center, Leiden, Netherlands
Daniel van Kammen, MD, PhD

CHDI Foundation, Inc., New York, New York

Biochemical Markers

Blair Leavitt, MD, CM, FRCPC

The University of British Columbia, BC Children's Hospital, British Colombia, Canada

Chair
Beth Borowsky, PhD

CHDI Foundation, Inc., New York, New York
Steven Hersch, MD, PhD

Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
Ruth Luthi-Carter, PhD

Ecole Polytechnique Federale de Lausanne, Luasanne, Switzerland
Oksana Suchowersky, MD, PhD, FRCP

University of Calgary, Calgary, Canada
Sarah Tabrizi, MD, PhD, FRCP

UCL Institute of Neurology, National Hospital of Neurology and Neurosurgery, London, United Kingdom

Cognitive

Jennifer Thompson, PhD

Greater Manchester Neuroscience Centre, Salford, United Kingdom

Chair
Leigh Beglinger, PhD

University of Iowa, Carver College of Medicine, Iowa City, Iowa
Peter Como, PhD

U.S. Food and Drug Administration, Washingotn, District of Colombia
Deborah Harrington, Ph.D

University of California San Diego, Ad hoc member, San Diego, California
Sarah Queller, PhD

Queller Consulting, Saint Petersburg, Florida
Megan Smith, Ph.D

The University of Iowa, Ad hoc member, Iowa City, Iowa
Glenn Stebbins, PhD

Rush University, Rush Medical College, Chicago, Illinois
Julie Stout, PhD

Monash University, Melbourne, Australia

Epidemiology/Environment

Caroline Tanner, MD, PhD

The Parkinson's Institute, Sunnyvale, California

Chair
Monica Busse, PhD

Cardiff University, Cardiff, United Kingdom
Elan Louis, MD, MSc

Columbia University, Mailman School of Public Health, New York, New York
Karen Marder, MD, MPH

Columbia University, College of Physicians and Surgeons, New York, New York
Brad Racette, MD

Washington University School of Medicine, St Louis, Missouri

Functional Outcomes/Patient Reported Outcomes

Jody Corey-Bloom, MD, PhD

University of California San Diego, San Diego, California

Chair
Noelle Carlozzi, PhD

The Center for Rehabilitation Outcomes and Assessment Research, University of Michigan, Ann Arbor, Michigan
Aileen Ho, PhD

University of Reading, Earley Gate, Reading, United Kingdom
Bernhard Landwehrmeyer, MD, PhD

University of Ulm, Ulm, Germany
Jane Paulsen, PhD

University of Iowa, Carver College of Medicine, Iowa City, Iowa
Lori Quinn, PT, EdD

Cardiff University, Cardiff, United Kingdom
David Tulsky, Ph.D.

University of Michigan Medical School, Ann Arbor, Michigan

Genetics

Tatiana Foroud, PhD

Indiana University School of Medicine, Indianapolis, Indiana

Chair
James Gusella, PhD

Massachusetts General Hospital, Center for Human Genetic Research, Boston, Massachusetts
Lesley Jones, PhD

Cardiff University School of Medicine, Cardiff, United Kingdom

Imaging Biomarkers

Elizabeth Aylward, PhD

Seattle Children's Research Institute, Seattle, Washington

Chair
Andrew Feigin, MD

New York University School of Medicine, New York, New York
Nellie Georgiou-Karistianis, PhD

Monash University, Melbourne, Australia
Diana Rosas, MD

Massachusetts General Hospital, Boston, Massachusetts
Christopher Ross, MD, PhD

The Johns Hopkins University, Baltimore, Maryland
Rachel Scahill, MA, PhD

UCL Institute of Neurology, London, United Kingdom

Motor

Kevin Biglan, MD

University of Rochester School of Medicine and Dentistry, Rochester, New York

Chair
Joanne Fielding, PhD

Monash Medical Centre, Clayton, Australia
Joseph Jankovic, MD

Baylor College of Medicine, Houston, Texas
Ralf Reilmann, MD

University Clinic Muenster, Munster, Germany
Kathleen Shannon, MD

University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin

Operations

Elise Kayson, MS, RNC, ANP

University of Rochester, Rochester, New York

Chair
Lisa deBlieck, MPA, CCRC

University of Rochester, Rochseter, New York
Terry Tempkin, RNC, MSN, ANP

University of California Davis Medical Center, Sacremento, California

Pathology

Jean Paul Vonsattel, PhD

Children's Hospital of New York-Presbyterian, New York, New York

Chair
Marian DiFiglia, MD, PhD

Massachusetts General Hospital, Boston, Massachusetts
Carol Moskowitz, MS, APRN-C, CNRN

Columbia University HD Center, New York, New York
Raymund Roos, MD

Leiden University Medical Center, Leiden, Netherlands
Clifford Saper, MD, PhD

Harvard Medical School and Beth Israel Deaconess Medical Center, Boston, Massachusetts
Leslie Thompson, PhD

University of California, Irvine, Irvine, California

Scale Metrics and Statistics

Doug Langbehn, MD, PhD

University of Iowa, Carver College of Medicine, Iowa City, Iowa

Chair
Peggy Auinger, M.S.

University of Rochester, Rochseter, New York
Kenneth Evans, PhD

Ontario Cancer Biomarker Network, Toronto, Canada
Michael McDermott, PhD

University of Rochester Medical Center, Rochester, New York
John Warner, PhD

CHDI Foundation, Inc., New York, New York

NINDS CDE Team

Codrin Lungu, MD

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
Daniel Miller, PhD

National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland
Debra Babcock, MD, PhD (Until 2017)

National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland
Joanne Odenkirchen, MPH (Until 2016)

NINDS CDE Project Officer, National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland
Beth-Anne Sieber, PhD (Until 2017)

National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland
Joy Esterlitz, MS (Until 2020)

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

The Emmes Company, LLC, Rockville, Maryland
Kristen Joseph, MA (Until 2019)

The Emmes Company, LLC, Rockville, Maryland
Muniza Sheikh, MS, MBA

The Emmes Company, LLC, Rockville, Maryland
Sherita Ala'i Hansen, MS (Until 2018)

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

A publication related to the HD CDE initiative is in development. The citation for this publication will be included on this site as soon as it is available.

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.

Huntington's Disease CDEs Revision History