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The metabolic pathology of dopa‐responsive dystonia
Author(s) -
Asanuma Kotaro,
Ma Yilong,
Huang Chaorui,
CarbonCorrell Maren,
Edwards Christine,
Raymond Deborah,
Bressman Susan B.,
Moeller James R.,
Eidelberg David
Publication year - 2005
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.20442
Subject(s) - dystonia , basal ganglia , neuroscience , cerebellum , premotor cortex , midbrain , medicine , motor cortex , dorsum , pathology , psychology , anatomy , central nervous system , stimulation
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. Ann Neurol 2005;57:596–600