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Brain iron accumulation in Wilson disease: a post-mortem 7 Tesla MRI - histopathological study

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Item Type:Article
Title:Brain iron accumulation in Wilson disease: a post-mortem 7 Tesla MRI - histopathological study
Creators Name:Dusek, P. and Bahn, E. and Litwin, T. and Jabłonka-Salach, K. and Łuciuk, A. and Huelnhagen, T. and Madai, V.I. and Dieringer, M.A. and Bulska, E. and Knauth, M. and Niendorf, T. and Sobesky, J. and Paul, F. and Schneider, S.A. and Czlonkowska, A. and Brück, W. and Wegner, C. and Wuerfel, J.
Abstract:Aims: In Wilson disease (WD), T2/T2*-weighted (T2*w) MRI frequently shows hypointensity in the basal ganglia that is suggestive of paramagnetic deposits. It is currently unknown whether this hypointensity is related to copper or iron deposition. We examined the neuropathological correlate of this MRI pattern, particularly in relation to iron and copper concentrations. Methods: Brain slices from nine WD and six control cases were investigated using a 7T-MRI system. High resolution T2*w images were acquired and R2* parametric maps were reconstructed using a multi-gradient recalled echo sequence. R2* was measured in the globus pallidus (GP) and the putamen. Corresponding histopathological sections containing the lentiform nucleus were examined using Turnbull iron staining, and double staining combining Turnbull with immunohistochemistry for macrophages or astrocytes. Quantitative densitometry of the iron staining as well as copper and iron concentrations were measured in the GP and putamen and correlated to R2* values. Results: T2*w hypointensity in the GP and/or putamen was apparent in WD cases and R2* values correlated with quantitative densitometry of iron staining. In WD, iron and copper concentrations were increased in the putamen compared to controls. R2* was correlated with the iron concentration in the GP and putamen whereas no correlation was observed for the copper concentration. Patients with more pronounced pathological severity in the putamen displayed increased iron concentration, which correlated with an elevated number of iron-containing macrophages. Conclusions: T2/T2*w hypointensity observed in vivo in the basal ganglia of WD patients is related to iron rather than copper deposits.
Keywords:Brain, Iron Accumulation, Pathology, Post-Mortem 7T MRI, Wilson Disease
Source:Neuropathology and Applied Neurobiology
Page Range:514-532
Date:October 2017
Official Publication:https://doi.org/10.1111/nan.12341
PubMed:View item in PubMed

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