TCA Cycle in the Dentate in Friedreich's Ataxia

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).

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