Related Experiment Video
Updated: May 27, 2026
Treating SCA1 Mice with Water-Soluble Compounds to Non-Specifically Boost Mitochondrial Function
Published on: January 22, 2017
MR spectroscopy and atrophy in Gluten, Friedreich's and SCA6 ataxias
M Hadjivassiliou1, L I Wallis, N Hoggard
1Department of Neurology, Royal Hallamshire Hospital, Sheffield, UK. m.hadjivassiliou@sheffield.ac.uk
Background:
Previous work using proton MR spectroscopy ((1)H-MRS) of the cerebellum in the ataxias suggested that (1)H-MRS abnormalities and atrophy do not necessarily occur concurrently.
Aims:
To investigate the spectroscopic features of different types of ataxias.
Methods:
Using a clinical MR system operating at 1.5T, we performed (1)H-MRS with a single voxel placed over the right dentate nucleus in 22 patients with gluten ataxia (GA), six patients with Friedreich's ataxia (FA), six patients with spinocerebellar ataxia type 6 (SCA6) and 21 healthy volunteers. Atrophy of the vermis and hemispheres on standard MRI was rated by a neuroradiologist. Any interaction between atrophy and (1)H-MRS was analysed for the three groups of patients and controls.
Results:
Patients with GA had significant atrophy of the vermis and hemispheres as well as abnormal (1)H-MRS. Patients with SCA6 had more severe overall atrophy of the vermis and hemispheres, but relatively preserved N-acetyl-aspartate/creatine (NAA/Cr). The FA group showed significant atrophy of only the superior vermis with normal (1)H-MRS.
Conclusions:
This study suggests that (1)H-MRS of the cerebellum in patients with ataxia provides information in addition to the presence of atrophy. There are significant (1)H-MRS differences amongst different types of ataxia with interesting correlations between atrophy and NAA/Cr.
