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Abstract #2791

Longitudinal monitoring of the cerebral iron load in de novo neurological Wilson disease

Monika Dezortova1, Petr Dusek2,3, Artem Lescinskij1,2, Julio Acosta-Cabronero4,5, Radan Bruha6, and Milan Hajek1

1MR-Unit, Dept. Diagnostic and Interventional Radiology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic, 2Dept. Radiology, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic, 3Dept. Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic, 4German Center for Neurodegenerative Diseases, Magdeburg, Germany, 5Wellcome Centre for Human Neuroimaging, UCL Institute of Neurology, University College London, London, United Kingdom, 64th Dept. Internal Medicine, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic

We report a long-term study of three de novo diagnosed Wilson disease patients with neurological form who repeatedly underwent magnetic resonance imaging and neurological examinations for 2 years after treatment initiation. The quantitative measurement of susceptibility revealed higher values in basal ganglia and thalamus compared to controls which correspond to higher iron accumulation.

Degree of iron load reflected the clinical severity of neurological impairment. Thus, we can suppose that the increase of the brain iron concentration can be a marker of suboptimal response to anti-copper therapy and unfavorable outcome.

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