The metabolic pathology of dopa-responsive dystonia

被引:41
作者
Asanuma, K
Ma, YL
Huang, CR
Carbon-Correll, M
Edwards, C
Raymond, D
Bressman, SB
Moeller, JR
Eidelberg, D
机构
[1] N Shore Long Isl Jewish Hlth Syst, Med Res Inst, Ctr Neurosci, Manhasset, NY 11030 USA
[2] N Shore Univ Hosp, Dept Neurol, New York, NY USA
[3] NYU, Sch Med, New York, NY USA
[4] Beth Israel Deaconess Med Ctr, Philips Ambulatory Care Ctr, Dept Neurol, New York, NY 10003 USA
[5] Columbia Univ, Columbia Univ Coll Phys & Surg, Dept Psychiat, New York, NY USA
关键词
D O I
10.1002/ana.20442
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
We used [F-18]-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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页码:596 / 600
页数:5
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