Natural History Study of and Genetic Modifiers in Spinocerebellar Ataxias

NCT ID: NCT01060371

Last Updated: 2023-06-01

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

800 participants

Study Classification

OBSERVATIONAL

Study Start Date

2010-04-30

Study Completion Date

2024-05-19

Brief Summary

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Spinocerebellar ataxias (SCA) are genetic neurological diseases that cause imbalance, poor coordination, and speech difficulties. There are different kinds of SCA and this study will focus on types 1, 2,3, and 6 (SCA 1, SCA 2, SCA 3 , also known as Machado-Joseph disease and SCA 6). The diseases are rare, slowly progressive, cause increasingly severe neurological difficulties and are variable across and within genotypes. The purpose of this research study is to bring together a group of experts in the field of SCA for the purpose of learning more about the disease.

The research questions are:

1. How does your disease progress over time?
2. What are the best ways to measure the progression?
3. Do some genes, other than the gene that is abnormal in your disease, have any effect on the way the disease behaves?

This is a nationwide study and we expect that 800 patients will participate all over the USA. The participants will be in the study for an indeterminate period of time. Study visits will be done every 6 or 12 months depending on the participating site.

Detailed Description

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If you decide to participate in this study, we will collect 1 tablespoon (15 milliliters) of blood during the first/screening visit in order to extract your DNA. The sample will be sent to the research laboratory of Dr Stefan Pulst at the University of Utah for the study of genetic factors that modify the course of your disease.

As part of this study, we would like to put some of your blood in a tissue repository. Submission of your sample to the repository may give scientists valuable research material that can help them to develop new diagnostic tests, new treatments, and new ways to prevent diseases. Scientists will not use your sample, or material isolated from it, for commercial products or services. Your blood will be kept by Dr. Stefan Pulst.

Your sample will not have your name or other personal information linked to it. Your sample may be shared with researchers at the University of Utah and at other institutions. The only information we will keep with the sample is your age, what disease you have, the age at onset of your disease and the duration of the disease. The principal investigator at your site will be the only person who can link the sample to you. You can have your sample removed from the bank later by written request to your PI.

You do not have to participate in the genetic modifier study or the tissue repository to be in the remaining part of this study.

You will also be asked to complete several assessments that include questionnaires, motor function test, a neurological exam and a physical exam.

Conditions

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Spinocerebellar Ataxia Type 1 Spinocerebellar Ataxia Type 2 Spinocerebellar Ataxia Type 3 Spinocerebellar Ataxia Type 6

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Spinocerebellar Ataxia 1

If you decide to participate in this study, the following study procedures will be performed:

* blood collection for DNA testing, analysis (genetic modifier study) and banking
* Medical history
* Physical exam
* Scale for Assessment and Rating of Ataxia (SARA)
* Timed measure of your hand dexterity and walking (25 ft)
* Questionnaire about your daily living activities, your physical and mental quality of life and assessment of depression.
* Disease stage estimation by the clinician.
* Demographics and disease-related information (i.e. age, sex, race, age at disease onset, disease duration)
* Review of your medical records

All Participants

Intervention Type GENETIC

If you decide to participate in this study, we will collect 1 tablespoon (15 milliliters) of blood during the first/screening visit in order to extract your DNA.

Spinocerebellar Ataxia 2

If you decide to participate in this study, the following study procedures will be performed:

* blood collection for DNA testing, analysis (genetic modifier study) and banking
* Medical history
* Physical exam
* Scale for Assessment and Rating of Ataxia (SARA)
* Timed measure of your hand dexterity and walking (25 ft)
* Questionnaire about your daily living activities, your physical and mental quality of life and assessment of depression.
* Disease stage estimation by the clinician.
* Demographics and disease-related information (i.e. age, sex, race, age at disease onset, disease duration)
* Review of your medical records

All Participants

Intervention Type GENETIC

If you decide to participate in this study, we will collect 1 tablespoon (15 milliliters) of blood during the first/screening visit in order to extract your DNA.

Spinocerebellar Ataxia 3

If you decide to participate in this study, the following study procedures will be performed:

* blood collection for DNA testing, analysis (genetic modifier study) and banking
* Medical history
* Physical exam
* Scale for Assessment and Rating of Ataxia (SARA)
* Timed measure of your hand dexterity and walking (25 ft)
* Questionnaire about your daily living activities, your physical and mental quality of life and assessment of depression.
* Disease stage estimation by the clinician.
* Demographics and disease-related information (i.e. age, sex, race, age at disease onset, disease duration)
* Review of your medical records

All Participants

Intervention Type GENETIC

If you decide to participate in this study, we will collect 1 tablespoon (15 milliliters) of blood during the first/screening visit in order to extract your DNA.

Spinocerebellar Ataxia 6

If you decide to participate in this study, the following study procedures will be performed:

* blood collection for DNA testing, analysis (genetic modifier study) and banking
* Medical history
* Physical exam
* Scale for Assessment and Rating of Ataxia (SARA)
* Timed measure of your hand dexterity and walking (25 ft)
* Questionnaire about your daily living activities, your physical and mental quality of life and assessment of depression.
* Disease stage estimation by the clinician.
* Demographics and disease-related information (i.e. age, sex, race, age at disease onset, disease duration)
* Review of your medical records

All Participants

Intervention Type GENETIC

If you decide to participate in this study, we will collect 1 tablespoon (15 milliliters) of blood during the first/screening visit in order to extract your DNA.

Interventions

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All Participants

If you decide to participate in this study, we will collect 1 tablespoon (15 milliliters) of blood during the first/screening visit in order to extract your DNA.

Intervention Type GENETIC

Eligibility Criteria

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Inclusion Criteria

* Presence of symptomatic ataxic disease
* Definite molecular diagnosis of SCA 1, 2,3,or 6 either in the subject or another affected family member
* Willingness to participate in the study and ability to give informed consent.
* Age 6 years and above

Exclusion Criteria

* Known recessive, X-linked and mitochondrial ataxias
* Exclusion of SCA 1, 2, 3 and 6 by previous DNA testing,
* A lack of willingness to participate in the study
Minimum Eligible Age

6 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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National Ataxia Foundation

OTHER

Sponsor Role collaborator

University of California, Los Angeles

OTHER

Sponsor Role collaborator

University of Florida

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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S. Subramony, MD

Role: PRINCIPAL_INVESTIGATOR

University of Florida

Locations

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University of California Los Angeles

Los Angeles, California, United States

Site Status RECRUITING

University of California San Francisco

San Francisco, California, United States

Site Status RECRUITING

University of Florida

Gainesville, Florida, United States

Site Status RECRUITING

University of South Florida

Tampa, Florida, United States

Site Status ACTIVE_NOT_RECRUITING

Emory University

Atlanta, Georgia, United States

Site Status RECRUITING

University of Chicago

Chicago, Illinois, United States

Site Status ACTIVE_NOT_RECRUITING

John Hopkins University

Baltimore, Maryland, United States

Site Status RECRUITING

Harvard University

Boston, Massachusetts, United States

Site Status RECRUITING

University of Michigan

Ann Arbor, Michigan, United States

Site Status ACTIVE_NOT_RECRUITING

University of Minnesota

Minneapolis, Minnesota, United States

Site Status ACTIVE_NOT_RECRUITING

Columbia University

New York, New York, United States

Site Status RECRUITING

University of Utah

Salt Lake City, Utah, United States

Site Status ACTIVE_NOT_RECRUITING

Countries

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United States

Facility Contacts

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Brian Clemente

Role: primary

310-206-8153

Julia Glueck

Role: primary

415-502-7640

SH Subramony, MD

Role: primary

352-273-5550

Rebecca MacMurray

Role: primary

404-728-4909

Ann Fishman

Role: primary

410-502-5816

Jason MacMore

Role: primary

617-726-3216

Sheng-Han Kuo, MD

Role: primary

212-305-5558

Related Links

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http://www.ataxia.org/

National Ataxia Foundation

Other Identifiers

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505-2009

Identifier Type: OTHER

Identifier Source: secondary_id

OCR16458

Identifier Type: OTHER

Identifier Source: secondary_id

IRB201700740

Identifier Type: -

Identifier Source: org_study_id

NCT01223417

Identifier Type: -

Identifier Source: nct_alias

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