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Abstract #2616

Analysis of Iron Accumulation in MAPT- and C9orf72-associated Frontotemporal Lobar Degeneration: QSM, T2*-w MRI, and Histology

Fieke Prinse1,2, Lucia Giannini1, Marjolein Bulk1, Ernst Suidgeest2, Kyra Dijkstra3, Renee van Buuren1, Elise Dopper1, Harro Seelaar1, and Louise van der Weerd2,3
1Alzheimer center and Neurology, Erasmus Medical Center, Rotterdam, Netherlands, 2CJ Gorter for MRI, department of Radiology, Leiden University Medical Center, Leiden, Netherlands, 3Human Genetics, Leiden University Medical Center, Leiden, Netherlands

Synopsis

Keywords: Electromagnetic Tissue Properties, Ex-Vivo Applications, Genetic Diseases, Neurodegeneration, Iron

Motivation: Ex-vivo histology is the most common method to assess iron accumulation in frontotemporal lobar degeneration, but in vivo assessment is crucial to unravel disease mechanisms.

Goal(s): This study aims to assess the distribution and severity of iron accumulation post-mortem using susceptibility MRI and compare this to histological data.

Approach: We analyzed postmortem tissue of 5 MAPT-FTLD and 2 C9orf72-FTLD cases using a histological iron staining, T2*-weighted MRI, and QSM maps.

Results: We found that histology, susceptibility MRI, and QSM show good correspondence of iron distribution in FTLD brain tissue. However, sharp details are better seen on T2*-weighted MRI, and U-fibers on QSM maps.

Impact: Our study showed that susceptibility-based imaging can be used to visualize iron accumulation in FTLD; with T2*-weighted MRI and QSM showing complementary spatial information. Therefore, these two methods should be used parallel and not independent of each other.

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Keywords