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Brain Atrophy Is Substantially Accelerated in Neurological Wilson's Disease: A Longitudinal Study

ABSTRACT Background Although brain atrophy is common in neurological Wilson's disease, longitudinal studies are lacking. Objective The objective of this study was to measure longitudinal brain atrophy rate and to relate it to the change in neurological impairment in Wilson's disease. Metho...

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Published in:Movement disorders 2022-12, Vol.37 (12), p.2446-2451
Main Authors: Smolinski, Lukasz, Ziemssen, Tjalf, Akgun, Katja, Antos, Agnieszka, Skowrońska, Marta, Kurkowska‐Jastrzębska, Iwona, Członkowska, Anna, Litwin, Tomasz
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Language:English
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Summary:ABSTRACT Background Although brain atrophy is common in neurological Wilson's disease, longitudinal studies are lacking. Objective The objective of this study was to measure longitudinal brain atrophy rate and to relate it to the change in neurological impairment in Wilson's disease. Methods We included patients with brain imaging done at diagnosis and at least 12 months later. The atrophy rate was measured as percentage change in ventricular volume, whereas the change in neurological impairment was scored on the Unified Wilson's Disease Rating Scale. Results Of 57 patients, 36 had neurological presentation, 17 had hepatic presentation, and 4 were presymptomatic. The annualized atrophy rate was significantly greater in patients with the neurological presentation than in other patients (P = 0.001). In the neurological presentation, the atrophy rate correlated with the change in impairment (rho = 0.39, P = 0.018) and was significantly greater in those with worsening after diagnosis than in those without worsening (P 
ISSN:0885-3185
1531-8257
DOI:10.1002/mds.29229