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Published on: September 26, 2016
Proton MR spectroscopic imaging in ataxia-telangiectasia
D D M Lin1, T O Crawford, H M Lederman
1Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA. ddmlin@jhmi.edu
Neuropediatrics
|December 21, 2006
Summary
Proton magnetic resonance spectroscopy reveals significant metabolite loss in the cerebellum of Ataxia-telangiectasia (A-T) patients, correlating with neurodegeneration. No basal ganglia abnormalities were found, despite symptoms suggesting extrapyramidal dysfunction.
Area of Science:
- Neuroscience
- Biochemistry
- Medical Imaging
Background:
- Ataxia-telangiectasia (A-T) is a rare, inherited neurodegenerative disorder characterized by progressive cerebellar degeneration and movement abnormalities.
- Neuroimaging studies often show posterior fossa atrophy in A-T patients.
- The underlying biochemical changes in specific brain regions are not fully understood.
Purpose of the Study:
- To investigate regional metabolite levels in the posterior fossa and basal ganglia of A-T patients using proton magnetic resonance spectroscopic imaging.
- To test the hypothesis that metabolite abnormalities reflect neurodegenerative processes in these areas.
- To correlate spectroscopic findings with clinical and morphological imaging data.
Main Methods:
- Proton magnetic resonance spectroscopic imaging was performed on 8 A-T patients and 8 age-matched controls.
- Levels of N-acetyl aspartate (NAA), choline (Cho), and creatine (Cr) were quantified.
- Metabolite levels were compared between groups in the cerebellum, brainstem, and basal ganglia.
Main Results:
- A-T patients showed a profound loss of NAA, Cr, and Cho in the cerebellar vermis compared to controls (p < 0.01 for NAA).
- A trend towards decreased metabolites was observed in the cerebellar hemispheres of A-T patients.
- No significant metabolite abnormalities were detected in the basal ganglia of A-T patients.
Conclusions:
- Proton MR spectroscopic findings in A-T are consistent with posterior fossa atrophy observed in morphologic neuroimaging.
- The absence of basal ganglia metabolite alterations suggests that extrapyramidal symptoms in A-T are not directly linked to biochemical changes in this region.
- MR spectroscopy is a valuable tool for assessing neurodegeneration in Ataxia-telangiectasia.
