## Abstract Our objective was to investigate thalamic neuronal dysfunction in patients with Huntington disease (HD). We performed localized single‐voxel proton magnetic resonance spectroscopy (MRS) of the thalamus in 22 HD patients and 25 healthy individuals. The mean age of patients was 48.5 years
Proton magnetic resonance spectroscopy in Huntington's disease: Evidence in favour of the glutamate excitotoxic theory?
✍ Scribed by Dr. S. D. Taylor-Robinson; R. A. Weeks; D. J. Bryant; J. Sargentoni; C. D. Marcus; A. E. Harding; D. J. Brooks
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- English
- Weight
- 927 KB
- Volume
- 11
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
Abstract
The gene responsible for Huntington's disease (HD) has been located, but its action and the pathophysiology of HD remain unclear. Glutamate excitotoxicity may contribute to the striatal neurodegeneration seen in HD. We used localised proton magnetic resonance spectroscopy (MRS) of the brain to investigate five patients with early HD, one symptom‐free gene carrier, and 14 healthy volunteers. Peak area ratios of choline‐containing compounds (Cho), glutamine and glutamate (Glx), and N‐acetyl moieties including N‐acetylaspartate (NAx), relative to creatine (Cr), were calculated. Spectra were analysed from the striatum and the occipital and the temporal cortex. The HD patients all had an elevated Glx/Cr in spectra localised to the striatum, compared with healthy controls, and one patient also had an elevated thalamic Glx/Cr. The mean Glx/Cr was unaltered in the cortical spectra of HD patients. The asymptomatic gene carrier displayed no spectral abnormalities. Our findings suggest disordered striatal glutamate metabolism and may support the theory of glutamate excitotoxicity in HD.
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