The metabolic pathology of dopa-responsive dystonia

Kotaro Asanuma, Yilong Ma, Chaorui Huang, Maren Carbon-Correll, Christine Edwards, Deborah Raymond, Susan B. Bressman, James R. Moeller, David Eidelberg

Research output: Contribution to journalArticlepeer-review

51 Scopus citations


We used [18F]-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.

Original languageEnglish
Pages (from-to)596-600
Number of pages5
JournalAnnals of Neurology
Issue number4
StatePublished - Apr 2005
Externally publishedYes


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