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Evidence for disturbances of copper metabolism in dystonia

From the image towards a new concept

From the Department of Neurology (Dr. Becker), Saarland University/Homburg, Department of Neurology (Drs. Berg and Naumann), University of Würzburg, Germany; and Human Genetics (Dr. Francis), Welcome Trust Center for Human Genetics, Oxford, UK.

Address correspondence and reprint requests to Dr. Georg Becker, Department of Neurology, Saarland University, 66421 Homburg, Germany; e-mail: georg.becker{at}med-rz.uni-saarland.de

The pathogenesis of idiopathic adult onset dystonia (ID) is still unclear. Although neuropathologic studies did not reveal consistent abnormalities, electrophysiologic and neuroimaging findings point toward a disinhibition and overactivity of the frontal motor cortical areas caused by an altered basal ganglia outflow. The lentiform nuclei are assumed to play a major role in this scenario. Recent neurochemical analysis of brain tissue stimulated by transcranial ultrasound studies demonstrated an increased copper content of the lentiform nuclei in patients with ID. The shift of brain copper level may substantially influence neuronal activity causing a reduced inhibitory output from the lentiform nuclei to the motor cortex. The reason for the presumably altered copper metabolism is not clear, but preliminary findings suggest that reduced levels of the Menkes protein, a membrane ATPase exporting copper out of the cells, may be implicated. Disturbances of brain copper metabolism may explain various phenomena of ID; however, it needs to be determined whether these observations represent the basic pathogenetic mechanism of ID or reflect another as yet unidentified pathologic process.

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