Journal
ANNALS OF NEUROLOGY
Volume 57, Issue 4, Pages 596-600Publisher
WILEY
DOI: 10.1002/ana.20442
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Funding
- NINDS NIH HHS [R01 NS37564, NS047668] Funding Source: Medline
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We used [F-18]-fluorodeoxyglucose and positron emission tomography to determine a discrete cerebral pattern of abnormal glucose utilization in dopa-responsive dystonia. Network analysis demonstrated that dopa-responsive dystonia is associated with a specific pattern of regional metabolic covariation, characterized by increases in the dorsal midbrain, cerebellum, and supplementary motor area, as well as reductions in motor and lateral premotor cortex and in the basal ganglia. This pattern was not expressed in mutation carriers for primary torsion dystonia. Dopa-responsive dystonia has a unique metabolic architecture that differs from other inherited forms of dystonia.
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