Striatal dysfunction in X‐linked dystonia‐parkinsonism is associated with disease progression

• Published in: European journal of neurology Vol. 24; no. 5; pp. 680 - 686
• Main Authors: Brüggemann, N., Rosales, R. L., Waugh, J. L., Blood, A. J., Domingo, A., Heldmann, M., Jamora, R. D., Münchau, A., Münte, T. F., Lee, L. V., Buchmann, I., Klein, C.
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01.05.2017
• Subjects: Adult; Corpus Striatum - diagnostic imaging; Corpus Striatum - metabolism; Corpus Striatum - physiopathology; Disease Progression; dopamine transporter imaging; Dystonic Disorders - diagnostic imaging; Dystonic Disorders - metabolism; Dystonic Disorders - physiopathology; DYT3 dystonia; Genetic Diseases, X-Linked - diagnostic imaging; Genetic Diseases, X-Linked - metabolism; Genetic Diseases, X-Linked - physiopathology; Humans; IBZM SPECT; Magnetic Resonance Imaging; Male; Middle Aged; neurogenetics; Tomography, Emission-Computed, Single-Photon - methods; X‐linked dystonia‐parkinsonism; X-linked dystonia-parkinsonism; IBZM SPECT; DYT3 dystonia; dopamine transporter imaging; neurogenetics

Background and purpose X‐linked dystonia‐parkinsonism (XDP) is an inherited neurodegenerative adult‐onset movement disorder associated with striatal atrophy. As the dopaminergic system has not yet been systemically studied in this basal ganglia model disease, it is unclear whether nigrostriatal dysf...