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Abstract #2329

Brain iron accumulation in Wilson disease measured by QSM and MR relaxometry

Monika Dezortova1, Vit Herynek1, Julio Acosta-Cabronero2, Lenka Kotackova3, Daniela Zahorakova4, Simon Daniel Robinson5, Filip Jiru1, Radan Bruha6, Zdenek Marecek7, Milan Hajek1, and Petr Dusek8

1MR-Unit, Dept Diagnostic and Interventional Radiology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic, 2German Center for Neurodegenerative Diseases, Magdeburg, Germany, 3Institute of Clinical Biochemistry and Laboratory Diagnostics, Charles University in Prague, 1st Faculty of Medicine and General University Hospital, Prague, Czech Republic, 4Dept Pediatrics and Adolescent Medicine, Charles University in Prague, 1st Faculty of Medicine and General University Hospital, Prague, Czech Republic, 5Dept Biomedical Imaging and Image guided Therapy, Medical University of Vienna, Vienna, Austria, 64th Dept Internal Medicine, Charles University in Prague, 1st Faculty of Medicine and General University Hospital, Prague, Czech Republic, 7KlinMed, Prague, Czech Republic, 8Dept Neurology and Center of Clinical Neuroscience, Charles University in Prague, 1st Faculty of Medicine and General University Hospital, Prague, Czech Republic

Relaxometry and quantitative susceptibility mapping were used in patients with neurologic symptoms of Wilson disease (WD) that leads to copper metabolism disturbances and its gradual accumulation in liver and brain. These quantitative MR techniques revealed decreased T2 relaxation times in the basal ganglia, higher susceptibility in the deep gray matter nuclei and no T1 changes. It indicates presence of insoluble para- or superparamagnetic compounds, presumably in a form of hemosiderin. These deposits appear to be not related to the ceruloplasmin oxidase activity nor with the severity of neurological symptoms.

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