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

doi:10.1002/ana.20442

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 journal › Article › peer-review

56 Scopus citations

Abstract

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 language	English
Pages (from-to)	596-600
Number of pages	5
Journal	Annals of Neurology
Volume	57
Issue number	4
DOIs	https://doi.org/10.1002/ana.20442
State	Published - Apr 2005
Externally published	Yes

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AU - Ma, Yilong

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AU - Edwards, Christine

AU - Raymond, Deborah

AU - Bressman, Susan B.

AU - Moeller, James R.

AU - Eidelberg, David

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AB - 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.

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The metabolic pathology of dopa-responsive dystonia

Abstract

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