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Regional decrease in brain glucose metabolism in asymptomatic gene carriers of Machado-Joseph disease: a preliminary
1Department of Neurology, National Yang-Ming University School of Medicine, Taiwan, ROC.
Zhonghua Yi Xue Za Zhi = Chinese Medical Journal; Free China Ed
|April 29, 1998
Summary
Positron emission tomography (PET) with [18F]-2-fluoro-2-deoxy-D-glucose (FDG) detected decreased brain metabolism in asymptomatic Machado-Joseph disease (MJD) gene carriers. This suggests preclinical disease activity and supports PET
Area of Science:
- Neuroscience
- Molecular Biology
- Medical Imaging
Background:
- Machado-Joseph disease (MJD) is a neurodegenerative disorder with a known molecular abnormality.
- Previous studies used [18F]-2-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) to assess metabolic changes in symptomatic MJD patients.
- This study investigated metabolic changes in asymptomatic MJD gene carriers.
Purpose of the Study:
- To evaluate brain glucose metabolism in asymptomatic MJD gene carriers using FDG-PET.
- To identify potential preclinical markers of MJD activity.
Main Methods:
- FDG-PET was employed to measure glucose metabolism in the brains of three asymptomatic MJD gene carriers.
- Ten normal control subjects were included for comparison.
Main Results:
- Asymptomatic MJD gene carriers showed significantly decreased glucose utilization in the cerebellar hemisphere and occipital cortex compared to controls.
- These metabolic changes suggest preclinical MJD activity.
- PET examination alone achieved 100% diagnostic sensitivity using decreased cerebellar hemisphere metabolism as a criterion.
Conclusions:
- Regional brain metabolism measurement by PET is a noninvasive method.
- Subclinical reduction in FDG uptake in the cerebellum and occipital cortex serves as an objective marker of MJD disease activity.
- The high sensitivity of PET supports its routine use for investigating asymptomatic at-risk MJD individuals.