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Brain iron accumulation in Wilson disease: a post mortem 7 Tesla MRI - histopathological study
P Dusek1,2, E Bahn3, T Litwin4
1Institute of Neuroradiology, University Medical Center Göttingen, Göttingen, Germany.
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 correlates 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 multigradient 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 with 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.
Insights
In Wilson disease (WD), MRI hypointensity in the basal ganglia is linked to iron, not copper deposits. This finding helps clarify the cause of MRI signal changes in WD patients.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Wilson disease (WD) is a genetic disorder causing copper accumulation.
- T2/T2*-weighted (T2*w) MRI often shows basal ganglia hypointensity in WD, suggesting paramagnetic deposits.
- The exact composition of these deposits (copper vs. iron) remains unclear.
Purpose of the Study:
- To investigate the neuropathological basis of T2*w MRI hypointensity in WD.
- To determine if basal ganglia hypointensity in WD is associated with copper or iron deposition.
- To correlate MRI findings with quantitative measurements of iron and copper in brain tissue.
Main Methods:
- Utilized 7T-MRI on brain slices from WD and control cases.
- Acquired high-resolution T2*w images and reconstructed R2* parametric maps.
- Examined histopathology using Turnbull iron staining and immunohistochemistry, correlating with R2* values and metal concentrations.
Main Results:
- T2*w hypointensity in the globus pallidus and putamen correlated with iron staining.
- WD cases showed increased iron and copper in the putamen compared to controls.
- R2* values correlated significantly with iron concentration, but not copper concentration, in the basal ganglia.
Conclusions:
- T2/T2*w MRI hypointensity in the basal ganglia of Wilson disease patients is primarily due to iron deposition.
- This study clarifies the role of iron in WD-related MRI signal abnormalities.
- Findings emphasize the importance of considering iron overload in the interpretation of basal ganglia MRI in WD.
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