Nora Volkow, MD
Brookhaven National Laboratory (BNL), USA
To assess brain activity in adult patients with A-T. An understanding of brain metabolism or activity in A-T may lead to the identification of potential therapeutic targets for this disease.
Participants with A-T demonstrated lower brain activity or metabolism in the cerebellum as compared to control participants. This result is not unexpected, as this area of the brain is particularly vulnerable in A-T and undergoes cell loss as the disease progresses. Adult participants with A-T also demonstrated increased metabolism in an area of the brain known as the globus pallidus, which helps regulate movements. This increased activity in the globus pallidus was correlated with decreased motor performance in the participants with A-T. Further studies are needed to corroborate these new findings; however, the “over active” globus pallidus may represent a target for therapeutic intervention for A-T.
Volkow, N.D., Tomasi, D., Wang, G.J., Studentsova, Y., Margus, B., and Crawford, T.O. (2014). Brain glucose metabolism in adults with ataxia-telangiectasia and their asymptomatic relatives. Brain 137, 1753-17