The metabolic pathology of dopa-responsive dystonia

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,...

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Bibliographic Details
Published in:Annals of neurology 2005-04, Vol.57 (4), p.596-600
Main Authors: Asanuma, Kotaro, Ma, Yilong, Huang, Chaorui, Carbon-Correll, Maren, Edwards, Christine, Raymond, Deborah, Bressman, Susan B., Moeller, James R., Eidelberg, David
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Brain - metabolism
Brain Mapping
Diseases of striated muscles. Neuromuscular diseases
Dystonia - drug therapy
Dystonia - metabolism
Dystonia - physiopathology
Female
Fluorodeoxyglucose F18
Humans
Image Processing, Computer-Assisted
Levodopa - therapeutic use
Male
Medical sciences
Nervous system (semeiology, syndromes)
Nervous system as a whole
Neurology
Parkinson Disease - metabolism
Parkinson Disease - physiopathology
Positron-Emission Tomography
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Summary: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. Ann Neurol 2005;57:596–600
ISSN:0364-5134
1531-8249
DOI:10.1002/ana.20442