TCA Cycle in the Dentate in Friedreich's Ataxia

NCT ID: NCT03122925

Last Updated: 2023-03-27

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

COMPLETED

Total Enrollment

20 participants

Study Classification

OBSERVATIONAL

Study Start Date

2017-08-29

Study Completion Date

2023-01-11

Brief Summary

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OBJECTIVE: To measure the tricarboxylic acid (TCA) cycle rate in the dentate nucleus in a group of control subjects and subjects with Friedreich's Ataxia (FRDA).

HYPOTHESIS: The TCA cycle rate will be lower in FRDA subjects than in controls APPROACH: The investigators will infuse carbon-13 (13C) labeled glucose and measure the rate of 13C label incorporation from glucose to glutamate in the brain using in vivo magnetic resonance spectroscopy.

Detailed Description

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The investigators will measure the TCA cycle rate in the dentate nucleus in a group of FRDA patients and in a group of age-matched healthy controls using 13C MRS in vivo together with systemic i.v. infusion of 13C-labeled glucose.

The investigators aim to obtain adequate data in 16 subjects grouped as follows:

* n=4 pilot subjects (healthy subjects) for testing and optimization of the experimental setup.
* n=6 healthy controls
* n=6 FRDA patients

In other to reach this goal, the investigators plan to recruit up to 20 subjects, in case some subjects withdraw before completion of the MR scan, or in case the measured data are not adequate (e.g. technical problem with the scanner).

Conditions

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Friedreich Ataxia

Study Design

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Observational Model Type

CASE_CONTROL

Study Time Perspective

PROSPECTIVE

Study Groups

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Control

Healthy subjects

MR

Intervention Type DEVICE

MR

Friedreich's Ataxia

Diagnosed for Friedreich's Ataxia

MR

Intervention Type DEVICE

MR

Interventions

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MR

MR

Intervention Type DEVICE

Eligibility Criteria

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Inclusion Criteria

* 18 years of age or older
* able to consent for themselves
* considered healthy (controls) OR be diagnosed with FRDA.

Exclusion Criteria

* All subjects:
* Participants who cannot have an MRI, as determined by the CMRR safety screening form (e.g. metal implant)
* Pregnancy
* Clautrophobia
* Diabetes
* Clinically significant cardiac disease

Control subjects:

\- Neurological disease
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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University of Minnesota

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Pierre-Gilles Henry, PhD

Role: PRINCIPAL_INVESTIGATOR

University of Minnesota

Locations

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CMRR

Minneapolis, Minnesota, United States

Site Status

Countries

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United States

Other Identifiers

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RAD-2017-25500

Identifier Type: -

Identifier Source: org_study_id

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