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Ann Neurol. 2005 Apr;57(4):596-600.

The metabolic pathology of dopa-responsive dystonia.

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  • 1Center for Neurosciences, Institute for Medical Research, North Shore-Long Island Jewish Health System, Manhasset, New York, NY, USA.


We used [(18)F]-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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