Chiari I Malformation

Data Standards

The purpose of the Chiari I Malformation Common Data Elements (CDEs) is to facilitate research by providing a standard definition for common, previously used research variables so that they may be used in a more uniform fashion across the available literature. The listing of Common Data Element and Instrument definitions and tools is not intended to limit the consideration of any other variables in the investigation of Chiari I Malformation, but merely to standardize the use of frequently observed variables, where possible. It is recognized, for example, that other metrics, especially those involving the characterization of soft tissue and CSF volume and shape, or those involving dynamic physiological measures, are likely important to future Chiari investigation and perhaps, ultimately, its evaluation and treatment. These metrics have not yet been included in this iteration of the Chiari I Malformation CDEs because there are no current standardization methods available. It is our hope that these elements may be part of future versions of the CDEs to further advance the literature. Finally, we should be mindful that it is not the purpose of these CDEs to define Chiari I Malformation itself or to guide surgical treatment; these CDEs are a tool for research purposes.

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 Chiari I Malformation was developed in 2016. The Core and Supplemental-Highly Recommended 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:

CDEs — Classified as Core, Supplemental - Highly Recommended, Supplemental, or Exploratory
CRF Modules — logically organize CDEs for data collection
Guidelines — to provide further information about the CDEs.

An overview of all Chiari I Malformation (CM1) CDE recommendations can be found in the Chiari I Malformation (CM1) CDE Highlight Summary document. For your reference, a zip file containing all the current Chiari I Malformation (CM1) 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/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

Overview of Working Group Recommendations

Summary Documents
CRF Module/Guidance	CDEs
Chiari I Malformation CDE Overall Summary	N/A
Comorbidities Working Group Summary	N/A
Demographics Working Group Summary	N/A
Imaging, Spinal Anomaly, Stability Working Group Summary	N/A
Outcomes Working Group Summary	N/A
Presentations, Natural History, Signs and Symptoms Working Group Summary	N/A
Treatment Working Group Summary	N/A

Participant Characteristics

Social Status
CRF Module/Guidance	CDEs
Social Status	CDE Details

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

Participant History and Family History

General Health History
CRF Module/Guidance	CDEs
General Exam	CDE Details
Past History (on intake)	CDE Details
CMI - Hereditary Disorder of Connective Tissue Screening (1)	CDE Details
CMI - Hereditary Disorders of Connective Tissue Screening (2)	CDE Details

Epidemiology/Environmental History
CRF Module/Guidance	CDEs
Allergy	CDE Details
Strain - Related Headache	CDE Details

Disease/Injury Related Events

History of Disease/Injury Event
CRF Module/Guidance	CDEs
CMI - Tethered Cord Screening	CDE Details
Chiari Symptom Onset	CDE Details
CMI - Headache Score	CDE Details

Classification
CRF Module/Guidance	CDEs
Chiari List of 10	CDE Details
Chiari I Malformation Presentation Exam	CDE Details

Assessments and Examinations

Imaging Diagnostics
CRF Module/Guidance	CDEs
Chiari I Malformation Imaging	CDE Details

General and Motor
CRF Module/Guidance	CDEs
Motor Strength Testing	CDE Details
Reflex Testing	CDE Details
Spine: Range of Motion Assessment	CDE Details
Coordination	CDE Details
Imbalance	CDE Details
Modified Ashworth Scale - Limb Tone Testing	CDE Details
Nystagmus/Strabismus	CDE Details
Provocative-Other Testing	CDE Details

Physical/Neurological Examination
CRF Module/Guidance	CDEs
Neurological Exam	CDE Details
Brainstem Signs/Symptoms	CDE Details
Self - Report Testing (On Intake)	CDE Details
Sensory Symptoms	CDE Details
Beighton Score	CDE Details

Treatment/Intervention Data

Drugs
CRF Module/Guidance	CDEs
Medications (Prior and Current)	CDE Details

Surgeries and Other Procedures
CRF Module/Guidance	CDEs
Surgical Site Assessment	CDE Details

Therapies
CRF Module/Guidance	CDEs
Chiari I Malformation Treatment	CDE Details
Chiari I Malformation Treatment Outcomes	CDE Details

Outcomes and End Points

Assessing Comorbidities
CRF Module/Guidance	CDEs
Co-Morbidities	CDE Details

Emotional and Cognitive Status
CRF Module/Guidance	CDEs
Zung Depression Score	N/A

Other Non-Motor
CRF Module/Guidance	CDEs
Assessment of Shunt Function	CDE Details

Functional Outcomes
CRF Module/Guidance	CDEs
Functional Status Karnofsky Score	CDE Details
Functional Status Lansky Score	CDE Details
Nurick (Ambulatory Function) Grade	N/A
McCormick Scale	N/A
ASIA Motor Scale	N/A
ASIA Sensory Scale	N/A
Functional Independence Measure	N/A
Functional Independence Measure for Children (WeeFIM)	N/A
Chicago Chiari Outcome Scale	N/A
Modified Japanese Orthopaedic Association Score	N/A

Pain
CRF Module/Guidance	CDEs
McGill Pain Questionnaire (MPQ)	N/A
Brief Pain Inventory (Short Form)	CDE Details
Numeric Rating Scale ? Neck Pain	CDE Details

Quality of Life/Patient Reported Outcomes
CRF Module/Guidance	CDEs
Chiari Symptom Profile	N/A
EuroQoL-5 Dimension Questionnaire-Youth (EQ-5DY)	N/A
Headache Disability Index	N/A
Health Transition Index	N/A
Meaningful Improvement	N/A
Neck Disability Index (NDI)	CDE Details
Patient Reported Outcomes Measurement Information System (PROMIS) ? Pediatric	N/A
Patient-Reported Outcomes Measurement Information System (PROMIS)	N/A
Quality of Life in Neurological Disorders (Neuro-QOL) - Pediatric Functional Health	N/A
Quality of Life in Neurological Disorders (Neuro-QOL)	N/A
Short Form 36-Item Health Survey (SF-36)	CDE Details
Short Form 12- Item Health Survey (SF-12)	N/A
The North American Spine Society Satisfaction Questionnaire	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 http://www.cssrs.columbia.edu).

Accessibility Statement

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 Chiari I Malformation CDE Working Group was comprised of six 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 Chiari I Malformation CDE Working Group was supported by the NINDS CDE Team.

Roster

Complete Chiari I Malformation CDE Roster

Oversight Committee Members

Allison Ashley-Koch, PhD

Duke University, Durham, North Carolina
Ulrich Batzdorf, MD

University of California, Los Angeles, Los Angeles, California

Chair
Ari Blitz, MD

Johns Hopkins Hospital, Baltimore, Maryland
Paolo Bolognese, MD

Chiari Neurosurgical Center at NSPC, Lake Success, New York
John Heiss, MD

Surgical Neurology Branch, NINDS, NIH, Bethesda, Maryland
Petra Klinge, MD, PhD

Warren Alpert Medical School of Brown University, Providence, Rhode Island
Roger Kula, MD

The Chiari Institute, Great Neck, New York
David Limbrick, MD, PhD

Washington University School of Medicine in St. Louis, St. Louis, Missouri
Brandon Rocque, MD

Children's of Alabama - Neurosurgery, Birmingham, Alabama

Core Demographic Database, Epidemiology

Cormac Maher, MD

University of Michigan, Ann Arbor, Michigan

Co-Chair
Brandon Rocque, MD

Children's of Alabama, Birmingham, Alabama

Co-Chair
Tim George, MD

Pediatric Neurosurgery Center of Central Texas, Austin, Texas

Presentation, Natural History, Signs Symptoms

Ulrich Batzdorf, MD

University of California, Los Angeles, Los Angeles, California

Co-Chair
Roger Kula, MD

The Chiari Institute, Great Neck, New York

Co-Chair
Gavin Britz, MD

Houston Methodist Hospital, Houston, Texas
Graham Flint, MD

Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom
Petra Klinge, MD, PhD

Rhode Island Hospital, Hasbro Children's Hospital, Providence, Rhode Island
Yong Liu, MD

Tsinghua University YuQuan Hospital, Beijing, China
Jeffrey Wisoff, MD

NYU Pediatric Neurosurgery Associates, New York, New York

Co-morbidities, Risk Factors, Genetics

Clair Francomano, MD

Greater Baltimore Medical Center, Towson, Maryland

Co-Chair
Allison Ashley-Koch, PhD

Duke University Medical Center, Durham, North Carolina

Co-Chair
Deven Atnoor, PhD

Data Analytics, Lexington, Massachusetts
Andrew Brodbelt, PhD

The Walton Centre, Fazakerley Liverpool, United Kingdom
Miroslav Gjurasin, MD, PhD

Children's Hospital Zagreb, Zagreb, Croatia
Rodney Grahame, MD

Hospital of St John and St Elizabeth, London, United Kingdom
Alfons Macaya, MD

Vall d'Hebron University Hospital, Barcelona, Spain
Juan Sahuquillo, MD

Vall d'Hebron University Hospital, Barcelona, Spain
Eric Trumble, MD

Florida Hospital Medical Group, Orlando, Florida
Roy Weller, MD, PhD

University of Southampton School of Medicine, Southampton, United Kingdom

Spinal Anomaly, Stability/ Imaging

Ed Benzel, MD

Cleveland Clinic Center for Spine Health, Cleveland, Ohio

Co-Chair
Douglas Brockmeyer, MD, FAAP

Primary Children's Hospital, Salt Lake City, Utah

Co-Chair
Harold Rekate, MD

The Chiari Institute, Great Neck, New York

Co-Chair
Brandon Rocque, MD

Children's of Alabama, Birmingham, Alabama

Co-Chair
Noam Alperin, PhD

University of Miami Miller School of Medicine, Miami, Florida
Olivier Baledent, PhD

The Amiens-Picardie University Hospital, Amiens, France
Barth Green, MD

University of Miami, Miami, Florida
Jeffrey Greenfield, MD, PhD

Weill Cornell Brain and Spine Center, New York, New York
Victor Haughton, MD

University of Wisconsin School of Medicine, Madison, Wisconsin
John Heiss, MD

National Institute of Health Neurosurgery, Bethesda, Maryland
Fraser Henderson, Sr, MD

Metropolitan Neurosurgery Group, Chevy Chase, Maryland
Jamie Jacobs, BS, MAT

Intrinsic Medical Imaging, Boston, Massachusetts
Bryn Martin, PhD

University of Idaho, Moscow, Idaho
Arnold Menezes, MD

University of Iowa Hospital & Clinic, Iowa City, Iowa
Misao Nishikawa, MD, PhD

Moriguchi-Ikuno Memorial Hospital, Osaka, Japan
John Oshinski, PhD

Emory University School of Medicine, Atlanta, Georgia
Marcus Stoodley, MD

Macquarie University Hospital, Sydney, Australia

Treatment

Paolo Bolognese, MD

Chiari Neurosurgical Center at NSPC, Lake Success, New York

Co-Chair
David Limbrick, MD, PhD

St. Louis Children's Hospital, St. Louis, Missouri

Co-Chair
Palma Ciaramitaro, MD

Centro Regionale Esperto Siringomielia-S. Chiari S.C. di Neurochirurgia, Torino, Italy
Jorg Klekamp, MD

Christliches Krankenhaus Quakenbruck, Quakenbruck, Germany
W. Jerry Oakes, MD

Children's of Alabama, Birmingham, Alabama
Gerald Rodts, MD

Emory Healthcare, Atlanta, Georgia
Marshall Wisoff, BS

ConMed Orthopedics, Largo, Florida

Outcomes

John Heiss, MD

National Institute of Health Neurosurgery, Bethesda, Maryland

Co-Chair
Mark Luciano, MD, PhD

The Johns Hopkins Hospital, Baltimore, Maryland

Co-Chair
Simon Archibald, PhD

Integra LifeSciences, New York, New York
Richard Ellenbogen, MD

Seattle Children's Hospital, Seattle, Washington
Bermans Iskandar, MD

University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin
Richard Kuntz, MD

Medtronic Inc., Minneapolis, Minnesota
Fabrice Parker, MD

Le Centre de Reference - Syringomyelie, Le Kremlin Bicetre Cedex, France

CSF Staff Representatives

Kaitlyn Esposito

Chiari & Syringomyelia Foundation, Staten Island, New York
Paul Farrell

Chiari & Syringomyelia Foundation, New York, New York
Andrea Grosz

Chiari & Syringomyelia Foundation, Forest Hills, New York
Dorothy Poppe

Chiari & Syringomyelia Foundation, Staten Island, New York
Cathy Poznik

Chiari & Syringomyelia Foundation, Twinsburg, Ohio

NINDS/NIH

M. Carolina Mendoza-Puccini, MD

NINDS CDE Program Officer, National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland
Robert Riddle, PhD

National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, Maryland

NINDS CDE Team

Sherita Ala'i, MS (Until 2018)

The Emmes Company, LLC, Rockville, Maryland
Joy Esterlitz, MS (Until 2020)

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

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

The Emmes Company, LLC, 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.

Chiari I Malformation CDE Revision History